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J Chin Med Assoc November 2008 • Vol 71 • No 11 583

Introduction

Trigeminal autonomic cephalalgia (TAC) is a primary headache syndrome that is characterized by severe

short-lasting unilateral headaches with ipsilateral cra-

nial autonomic symptoms. Based on the International

Classification of Headache Disorders, 2nd edition(ICHD-2),1 this group includes cluster headache, par-

oxysmal hemicrania, and short-lasting unilateral neu-

ralgiform headache with conjunctival injection andtearing. The pathophysiology is still poorly understood.

The hypothalamus and trigeminovascular system have

been implicated.2

Several reports have described the association of

TAC-like headache with intracranial lesions. Pituitary

adenomas were the most common lesions.3 Other le-

sions, such as arteriovenous malformation of temporal

and frontal lobes, brain tumor in posterior fossa, ver-

tebral artery aneurysm, and orbitosphenoidal asper-gillosis, were also reported.3 Most of the lesions were

located around the sella region or posterior cranial

fossa. Rarely, parenchymal lesions and sphenoid sinusinfection were also noted.

Cutaneous allodynia is common during migraine

attacks. It is related to sensitization of neurons in thetrigeminal nucleus caudalis and/or thalamus.4 The as-

sociation between allodynia and TAC such as cluster

headache is disputed,5 and the location responsible

for allodynia in TAC is not clear.In this report, we present a patient with multiple

sclerosis, whose clinical features resembled TAC with

allodynia. To the best of our knowledge, this is the firstcase report of spinal trigeminal nucleus as the under-

lying anatomic correlation.

C AS E R EP OR T

Symptomatic Trigeminal Autonomic Cephalalgia

Associated with Allodynia in a Patient with

Multiple Sclerosis

Fang-Chun Liu, Jong-Ling Fuh, Shuu-Jiun Wang*

Neurological Institute, Taipei Veterans General Hospital and National Yang-Ming University School of

Medicine, Taipei, Taiwan, R.O.C.

A patient with symptomatic trigeminal autonomic cephalalgia (TAC) provides a chance to understand the pathophysiology

and anatomic correlates of TAC. A 28-year-old woman experienced intermittent sharp and excruciating pain over her right

temporal, ear and neck regions for 3 days. The headaches lasted 10–20 minutes each, occurred 1–2 times a day, and

were accompanied by prominent ipsilateral lacrimation and conjunctival injection. The patient had hiccups, 4-limb numb-

ness and impaired visual acuity in both eyes. She had also had 3 episodes of left-side optic neuritis in the past half year.

Neurologic examination showed brushing allodynia over the right face and scalp during the headache attacks. The visual

acuity of her right eye was 6/60 and that of the left eye was 1/60. Brain magnetic resonance imaging showed non-

enhancing lesions on the right lateral tegmentum of the lower pons where the spinal trigeminal nucleus is located and the

floor of the 4th ventricle. The patient was diagnosed as having multiple sclerosis with symptomatic TAC. Her headaches,

autonomic signs and allodynia subsided 3 days after pulse therapy and gabapentin treatment were given. We suggest

that the spinal trigeminal nucleus lesion was responsible for the symptomatology of TAC and cutaneous allodynia in our

patient. [ J Chin Med Assoc 2008;71(11):583–586]

Key Words: allodynia, multiple sclerosis, trigeminal autonomic cephalalgia

© 2008 Elsevier. All rights reserved.

*Correspondence to: Dr Shuu-Jiun Wang, Neurological Institute, Taipei Veterans General Hospital,

201, Section 2, Shih-Pai Road, Taipei 112, Taiwan, R.O.C.

E-mail: [email protected] ● Received: March 31, 2008 ● Accepted: June 2, 2008



Case Report

A 28-year-old woman presented with an excruciating

pain that was initially localized to the right ear and then

spread to her right temporal and neck regions. Thepain began 3 days before admission. The headaches

lasted for approximately 10–20 minutes each and oc-curred 1–2 times a day. It was accompanied by markedipsilateral lacrimation, conjunctival injection, nausea

and vomiting, but not rhinorrhea, nasal stuffiness or

ptosis. In addition, the patient found that touching

the right side of her face and scalp would induce severepain during the headache attacks, which did not hap-

pen when the headache subsided. TAC was tentatively

diagnosed. In addition to the headaches, she also com-plained of persistent hiccup, 4-limb numbness and

impaired visual acuity.

The patient had had 3 recurrent episodes of optic

neuritis of her left eye in the past half year. The visualacuity of the left eye during the last attack was only

counting fingers but recovered to 6/20 2 weeks later.

The magnetic resonance imaging (MRI) finding of theleft optic nerve at that time (Figure 1) was compatible

with optic neuritis, but no brain parenchymal abnormal-

ities were noted. Cerebrospinal fluid studies includingIgG index were normal.

Neurologic examination showed impaired pinprick

sensation over distal limbs. Other sensory modalities,

such as light touch, position and vibration were nor-mal. The visual acuity of the right eye deteriorated

from 6/6 to 6/60, and that of the left from 6/60 to1/60. Fundoscopy showed temporal pale on the leftoptic disc. No focal weakness or impairment of other

cranial nerves was detected. Tendon reflexes were normal

and bilaterally symmetrical. Brush-evoked allodynia,

tested by repetitively applying a 4 ×4-inch gauze pad,

was found on the patient’s right face (trigeminal nerve

1st and 2nd branches’ distribution) and scalp during

the headache attacks.Brain MRI showed non-enhancing T1 isointense

and T2 high signal lesions on the right lateral tegmen-

tum of the lower pons, bilateral optic nerves and thefloor of the 4th ventricle (Figure 2A). These lesions had

not been seen on the brain MRI done 2 months previ-

ously. There were no hemisphere white matter lesions.

Cerebrospinal fluid studies including oligoclonal bandand IgG index were normal. Autoimmune markers such

as ANA, ds-DNA, SS-A and SS-B were negative. Based

on the clinical, neuroimaging and laboratory findings,the diagnosis of definite multiple sclerosis was made.

The headaches, autonomic symptoms and allodynia

subsided gradually within 3 days after steroid pulsetherapy (1,000mg/day methylprednisolone) and ga-

bapentin were given. Of note, her hiccups were alsocontrolled when the dosage of gabapentin reached

800 mg/day. Gabapentin was tapered 1 month later.However, the hiccups and nausea recurred 1 week

after gabapentin withdrawal. Therefore, gabapentin

was reinstituted, which relieved the hiccups immedi-ately. As of the last visit 9 months after this attack, no

further symptoms had occurred. Follow-up brain MRI

4 months later disclosed that the lesion had markedly resolved (Figure 2B).

Discussion

Our patient presented with paroxysmal unilateral head-

aches and ipsilateral autonomic symptoms, which werecompatible with TAC. Brain MRI showed a lesion

J Chin Med Assoc • November 2008 • Vol 71 • No 11584

F.C. Liu, et al

A B

Figure 1. (A) Axial T2-weighted magnetic resonance imaging shows abnormal high signals at the left optic nerve. (B) Axial T1-weighted

imaging with gadolinium shows patchy enhancement in the segment just anterior to the optic canal and the prechiasmatic segment of

the left side optic nerve.



involving the right spinal trigeminal nucleus. Since

this lesion was only shown during this attack but not

2 months previously, we considered it to be responsible

for both her TAC and cutaneous allodynia.Only 3 patients with multiple sclerosis presenting

with TAC have been reported before. The first case was a 42-year-old man with headache lasting for 2–3

hours, which was initially localized to the right orbit, and

then spread to the homolateral maxillary and temporalregions.6 Ipsilateral tearing, rhinorrhea and eye redden-

ing were found. His lesions were around the lateral

ventricles and root entry zone of the right trigeminal

nerve. The second case was a 53-year-old man withstabbing pain, shooting from his left upper lip to above

and medial to his left eye for 15 seconds, followed by

a piercing pain over the left cheek for 20 seconds.7

Profuse tearing and eye congestion were also noted.

No abnormality was found on imaging studies. The

third case was a 42-year-old man with right temporalstabbing pain that lasted 5–30 seconds.8 Ipsilateral tear-

ing and conjunctival injection were found. The lesions

were on the anterior pons, right cerebral peduncle and

medulla. The lesions of our patient and the first andprobably the third cases described above all involved

the trigeminal system. In contrast to previous cases,

only our patient presented with cutaneous allodynia.

Functional imaging has confirmed an activation of

the hypothalamic grey matter in induced or spontaneous

cluster headaches.2,9 Sympathetic nerves surrounding

carotid arteries10 and the trigeminal systems11 mightalso be possible locations for autonomic symptoms.

In our and other multiple sclerosis cases,6,8 the involve-ment of the trigeminal system but not the hypothalamus

on brain MRI suggested that a trigeminal nerve system

lesion itself could present as TAC. Furthermore, allo-dynia has been linked to the sensitization of the 2nd-

order neurons in the spinal trigeminal nucleus and/or

3rd-order neurons in the thalamus in migraine patients.4

It is not known whether this is also true in TAC patients.Nevertheless, the ipsilateral spinal trigeminal nucleus

lesion in our patient supports this speculation.

Gabapentin has been reported to be effective inthe treatment of trigeminal neuralgia in patients with

multiple sclerosis.12 Both headaches and allodynia sub-

sided after pulse therapy and gabapentin treatment inour patient. In addition to pulse therapy, we could

not exclude the possibility that gabapentin might play

a role in relieving both TAC and allodynia in our

patient.In conclusion, our patient is the first reported case

presenting with both symptomatic TAC and allody-

nia. This case demonstrated that the spinal trigeminal

J Chin Med Assoc • November 2008 • Vol 71 • No 11 585

Symptomatic TAC with allodynia

A B

CST

ML

VII Nu.

VI Nu.

STT

STN

C

Figure 2. (A) Axial magnetic resonance fluid-attenuated inversion-

recovery imaging (MRI FLAIR) shows a hyperintense lesion in the

right lateral tegmentum of the lower pons. (B) The lesion is under

resolution markedly on MRI FLAIR 4 months later. (C) The lesion

was suspected as spinal trigeminal nucleus (STN). STT = spinal

trigeminal tract; CST = corticospinal tract; ML =medial lamiscus.



nucleus itself could activate both the trigemino-

autonomic reflex and allodynia pathway.

Acknowledgments

This study was supported, in part, by a grant from Taipei Veterans General Hospital (V96C1-041), Taipei,

Taiwan.

References

1. The International Classification of Headache Disorders: 2nd

edition. Cephalalgia 2004;24(Suppl):9–160.2. Goadsby PJ. Pathophysiology of cluster headache: a trigeminal

autonomic cephalgia. Lancet Neurol 2002;1:251–7.3. Favier I, van Vliet JA, Roon KI, Witteveen RJ, Verschuuren JJ,

Ferrari MD, Haan J. Trigeminal autonomic cephalgias due to

structural lesions: a review of 31 cases. Arch Neurol 2007;64:25–31.

4. Burstein R, Yarnitsky D, Goor-Aryeh I, Ransil BJ, Bajwa ZH. An association between migraine and cutaneous allodynia. Ann Neurol 2000;47:614–24.

5. Ladda J, Straube A, Forderreuther S, Krause P, Eggert T.Quantitative sensory testing in cluster headache: increasedsensory thresholds. Cephalalgia 2006;26:1043–50.

6. Leandri M, Cruccu G, Gottlieb A. Cluster headache-like painin multiple sclerosis. Cephalalgia 1999;19:732–4.

7. Davey R, Al-Din A. Secondary trigeminal autonomic cephalgiaassociated with multiple sclerosis. Cephalalgia 2004;24:605–7.

8. Vilisaar J, Constantinescu CS. SUNCT in multiple sclerosis.Cephalalgia 2006;26:891–3.

9. May A. Cluster headache: pathogenesis, diagnosis, and manage-ment. Lancet 2005;366:843–55.

10. Hardebo JE. How cluster headache is explained as an intra-cavernous inflammatory process lesioning sympathetic fibers.Headache 1994;34:125–31.

11. Frese A, Evers S, May A. Autonomic activation in experimentaltrigeminal pain. Cephalalgia 2003;23:67–8.

12. Khan OA. Gabapentin relieves trigeminal neuralgia in multiplesclerosis patients. Neurology 1998;51:611–4.

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