a case of tolosa hunt syndrome
TRANSCRIPT
An Interesting Case
Dr PannerA Unit
Presenter Dr Ramesh BabuM
Brief History
bull A 53yrs old female from Westbengal Diabetic -
10yrs
bull Co Headache - Lt periorbital - 4 months
bull Parasthesia over left frontal area
bull Ho loss of Weight ~5kg in last 4 months
bull Evening rise of temparature - (99-100f) on ampoff
bull Headache UL Lt periorbital spreads to frontal amp
rest of the cranium started on 1022017 at 930pm
at bedtime which was sudden in onset very severe
unbearable throbbing type continuous lasts for 2-3
days and episodic in nature not subsiding with
painkillers no pain with eye movements
bull No HO nausea vomitings giddiness double vision
LOC seizures sweating lacrimation flushing
redness of the face and the eye photophobia
phonophobia
bull No ho similar episodes in the past
bull with the above complaints approached to local
physician(20217) - treated with NSAIDS and antidepressants
asked for MRI- showed left 1cm mass lesion at the base of the
skull and suggested to see a Neurosurgeon
bull He referred to Neurologist INSkolkata (1317)- they asked
complete tuberculosis work - all reports against TB except
mantoux test - 18mm and started on ATT and tapering dose of
steroids over 1 month (8317) referred to NIMHANS for CSF
evaluation
bull In NIMHANS (15317)- LP - dry tap and suggested to continue
on ATT Patient couldnt tolerate ATT hence she stopped ATT
bull On 20317 in INS they repeated CSF analysis
which was normal
bull On 31317 she was started on antibiotic course
(T Septran and Cipro) for 5 days NSAIDS with
steroids Patient improved her symptoms by
90
bull Presently she had mild periorbital pain
bull On Examination
bull Patient conscious well oriented
bull No Neurological deficits
bull Opthalmology- Unremarkable No ocular or
cranial bruits Fundus - normal
bull Other systems - normal
Investigationsbull CBC- Normal limits
bull ESR- 34mmhr
bull CRP- negative
bull FBS- 152mgdl
bull HbA1c- 66
bull Mantoux test- strong + 18mm
bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)
protein- 75mgdl Grams stain Indian ink - negative
bull CSF XPERT MTBRIF- Negative
bull S ACE levels- normal
bull Chest xray- normal
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Brief History
bull A 53yrs old female from Westbengal Diabetic -
10yrs
bull Co Headache - Lt periorbital - 4 months
bull Parasthesia over left frontal area
bull Ho loss of Weight ~5kg in last 4 months
bull Evening rise of temparature - (99-100f) on ampoff
bull Headache UL Lt periorbital spreads to frontal amp
rest of the cranium started on 1022017 at 930pm
at bedtime which was sudden in onset very severe
unbearable throbbing type continuous lasts for 2-3
days and episodic in nature not subsiding with
painkillers no pain with eye movements
bull No HO nausea vomitings giddiness double vision
LOC seizures sweating lacrimation flushing
redness of the face and the eye photophobia
phonophobia
bull No ho similar episodes in the past
bull with the above complaints approached to local
physician(20217) - treated with NSAIDS and antidepressants
asked for MRI- showed left 1cm mass lesion at the base of the
skull and suggested to see a Neurosurgeon
bull He referred to Neurologist INSkolkata (1317)- they asked
complete tuberculosis work - all reports against TB except
mantoux test - 18mm and started on ATT and tapering dose of
steroids over 1 month (8317) referred to NIMHANS for CSF
evaluation
bull In NIMHANS (15317)- LP - dry tap and suggested to continue
on ATT Patient couldnt tolerate ATT hence she stopped ATT
bull On 20317 in INS they repeated CSF analysis
which was normal
bull On 31317 she was started on antibiotic course
(T Septran and Cipro) for 5 days NSAIDS with
steroids Patient improved her symptoms by
90
bull Presently she had mild periorbital pain
bull On Examination
bull Patient conscious well oriented
bull No Neurological deficits
bull Opthalmology- Unremarkable No ocular or
cranial bruits Fundus - normal
bull Other systems - normal
Investigationsbull CBC- Normal limits
bull ESR- 34mmhr
bull CRP- negative
bull FBS- 152mgdl
bull HbA1c- 66
bull Mantoux test- strong + 18mm
bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)
protein- 75mgdl Grams stain Indian ink - negative
bull CSF XPERT MTBRIF- Negative
bull S ACE levels- normal
bull Chest xray- normal
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bull Headache UL Lt periorbital spreads to frontal amp
rest of the cranium started on 1022017 at 930pm
at bedtime which was sudden in onset very severe
unbearable throbbing type continuous lasts for 2-3
days and episodic in nature not subsiding with
painkillers no pain with eye movements
bull No HO nausea vomitings giddiness double vision
LOC seizures sweating lacrimation flushing
redness of the face and the eye photophobia
phonophobia
bull No ho similar episodes in the past
bull with the above complaints approached to local
physician(20217) - treated with NSAIDS and antidepressants
asked for MRI- showed left 1cm mass lesion at the base of the
skull and suggested to see a Neurosurgeon
bull He referred to Neurologist INSkolkata (1317)- they asked
complete tuberculosis work - all reports against TB except
mantoux test - 18mm and started on ATT and tapering dose of
steroids over 1 month (8317) referred to NIMHANS for CSF
evaluation
bull In NIMHANS (15317)- LP - dry tap and suggested to continue
on ATT Patient couldnt tolerate ATT hence she stopped ATT
bull On 20317 in INS they repeated CSF analysis
which was normal
bull On 31317 she was started on antibiotic course
(T Septran and Cipro) for 5 days NSAIDS with
steroids Patient improved her symptoms by
90
bull Presently she had mild periorbital pain
bull On Examination
bull Patient conscious well oriented
bull No Neurological deficits
bull Opthalmology- Unremarkable No ocular or
cranial bruits Fundus - normal
bull Other systems - normal
Investigationsbull CBC- Normal limits
bull ESR- 34mmhr
bull CRP- negative
bull FBS- 152mgdl
bull HbA1c- 66
bull Mantoux test- strong + 18mm
bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)
protein- 75mgdl Grams stain Indian ink - negative
bull CSF XPERT MTBRIF- Negative
bull S ACE levels- normal
bull Chest xray- normal
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bull with the above complaints approached to local
physician(20217) - treated with NSAIDS and antidepressants
asked for MRI- showed left 1cm mass lesion at the base of the
skull and suggested to see a Neurosurgeon
bull He referred to Neurologist INSkolkata (1317)- they asked
complete tuberculosis work - all reports against TB except
mantoux test - 18mm and started on ATT and tapering dose of
steroids over 1 month (8317) referred to NIMHANS for CSF
evaluation
bull In NIMHANS (15317)- LP - dry tap and suggested to continue
on ATT Patient couldnt tolerate ATT hence she stopped ATT
bull On 20317 in INS they repeated CSF analysis
which was normal
bull On 31317 she was started on antibiotic course
(T Septran and Cipro) for 5 days NSAIDS with
steroids Patient improved her symptoms by
90
bull Presently she had mild periorbital pain
bull On Examination
bull Patient conscious well oriented
bull No Neurological deficits
bull Opthalmology- Unremarkable No ocular or
cranial bruits Fundus - normal
bull Other systems - normal
Investigationsbull CBC- Normal limits
bull ESR- 34mmhr
bull CRP- negative
bull FBS- 152mgdl
bull HbA1c- 66
bull Mantoux test- strong + 18mm
bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)
protein- 75mgdl Grams stain Indian ink - negative
bull CSF XPERT MTBRIF- Negative
bull S ACE levels- normal
bull Chest xray- normal
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bull On 20317 in INS they repeated CSF analysis
which was normal
bull On 31317 she was started on antibiotic course
(T Septran and Cipro) for 5 days NSAIDS with
steroids Patient improved her symptoms by
90
bull Presently she had mild periorbital pain
bull On Examination
bull Patient conscious well oriented
bull No Neurological deficits
bull Opthalmology- Unremarkable No ocular or
cranial bruits Fundus - normal
bull Other systems - normal
Investigationsbull CBC- Normal limits
bull ESR- 34mmhr
bull CRP- negative
bull FBS- 152mgdl
bull HbA1c- 66
bull Mantoux test- strong + 18mm
bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)
protein- 75mgdl Grams stain Indian ink - negative
bull CSF XPERT MTBRIF- Negative
bull S ACE levels- normal
bull Chest xray- normal
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bull On Examination
bull Patient conscious well oriented
bull No Neurological deficits
bull Opthalmology- Unremarkable No ocular or
cranial bruits Fundus - normal
bull Other systems - normal
Investigationsbull CBC- Normal limits
bull ESR- 34mmhr
bull CRP- negative
bull FBS- 152mgdl
bull HbA1c- 66
bull Mantoux test- strong + 18mm
bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)
protein- 75mgdl Grams stain Indian ink - negative
bull CSF XPERT MTBRIF- Negative
bull S ACE levels- normal
bull Chest xray- normal
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Investigationsbull CBC- Normal limits
bull ESR- 34mmhr
bull CRP- negative
bull FBS- 152mgdl
bull HbA1c- 66
bull Mantoux test- strong + 18mm
bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)
protein- 75mgdl Grams stain Indian ink - negative
bull CSF XPERT MTBRIF- Negative
bull S ACE levels- normal
bull Chest xray- normal
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
MRI
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
MRI
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
MRI Report
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Summary
bull A 53 yrs old lady with acute retroorbital headache and
Ltfrontal parasthesia of 4 month duration not
associated eye movements without any associated
symptoms of migraine raised ICT features autonomic
symptoms with constitutional symptomswith no
neurological deficits with normal investigations except
positive Mantoux test amp MRI - showing contrast
enhancing lesion of cavernous sinus inflammatory
peudotumor with good response to short antibiotic
course and to steroids the likely differentials will be
Cavernous sinus syndrome
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Discussion
bull Causes for retroorbital pain - orbital superior
orbital fissure cavernous sinus or intracranial
infiltrative neoplastic or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam
bull In some patients no etiology for the pain
syndrome is idiopathic eye pain eye strain or
atypical facial pain
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Cavernous Sinus
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Cavernous sinus mass
has a wide differential including
bull Meningioma
bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
bull Infection non - infectious inflammatory
bull Schwannoma -trigeminal schwannoma is the most common
bull Cavernous haemangioma
bull Lymphoma
bull Metastatic disease (ie perineural spread of tumour through neural foramina)
bull Aneurysm
bull Haemangiopericytoma
bull Neurosarcoidosis rarely involves the cavernous sinus
bull Base of skull tumour
chondrosarcoma
osteosarcoma
chordoma (usually midline)
bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bull Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome
bull Tumors may be primary or may arise from either
local spread or as metastases
bull Cavernous sinus syndrome is defined by its
resultant signs and symptoms ophthalmoplegia
chemosis proptosis Horner syndrome or
trigeminal sensory loss Infectious or
noninfectious inflammatory vascular traumatic
and neoplastic processes are the principal
causes
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Cavernous sinus lesions are characterized by the following signs
bull Unilateral and isolated third fourth or sixth cranial nerve palsy
bull Combination patterns of ophthalmoplegia
bull Painful ophthalmoplegia
bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
bull Ocular and cranial bruits
bull Conjunctival congestion arterialization of conjunctival veins
bull Ocular hypertension
bull Optic disc edema or pallor retinal hemorrhages
bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
andor decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
bull Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bullThe Tolosa-Hunt syndrome was first described in 1954 in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery
bullThe criteria to diagnose the syndrome as follows
bull1 - acute retro-orbital pain
bull2 - alterations on the third fourth sixth or first branch of the fifth cranial nerve and less
commonly involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid
bull3 - symptoms persisting for days or weeks
bull4 - spontaneous pain remission
bull5 - recurrent episodes
bull6 - prompt response to steroidsPathology
bullThe constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells along with thickening of dura mater within the cavernous sinus
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bull The exact cause of Tolosa-Hunt syndrome is unknown
one theory is an abnormal autoimmune response
linked with an inflammation
bull In some cases inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation)
bull Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered ldquodiagnosticrdquo of Tolosa-Hunt
syndrome
bull Steroids are generally tapered over weeks to months
in some cases prolonged therapy may be necessary
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Post Treatment
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Repeat MRI Report
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
Treatmentbull The three most common treatments of cavernous sinus tumors are
bull 1Observation
bull 2Non-surgical radiation therapy (SRS) and
bull 3Microsurgical resection (corrective surgery)
bull Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time
bull Regular scans should be performed with careful monitoring for change
bull If tests indicate the tumor has spread SRS is an option and can provide temporary
relief of symptoms
bull Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled such as when the optic nerves have become
compressed
bull
bull Thank You
bull Thank You