HSVE IIH ICL

Pallavi to talk

TCH DSA

PCA sign Hypothermia

Jog to talk

PML

Case 1

42/F History since 3 days

Fever Headache Confusion

No seizures, rash On examination

Drowsy, confused (GCS 10/15) Fundi normal No other deficit No neck stiffness

Metabolic lab: WNL WBC Counts: 9500 HIV: -ve

MRI brain

CSF: Proteins 110 Sugar 65 (BSL 135) Cells 26 (95% lympho) HSV PCR sent

Started on I/V acyclovir 600 mg 8 hourly Neurostatus same on day 2

3rd day No fever Single SG seizure More drowsy (GCS 7/15) Left hemiparesis At night

Right pupil dilated Intubated

CT scan brain

In view of large area of damage with mass effect Underwent decompression craniotomy Biopsy take from temporal lobe showed F/O

encephalitis

Next 3 days (Day 4-6) No significant change On ventilator Drowsy (GCS 5-6/15) Developed right III nerve palsy Occasional focal and SG seizures

7th day Unconscious (GCS 4/15) On ventilator Right III nerve palsy Left pupil also became dilated Dense left hemiparesis

Repeat CT scan brain

Further course Continued on I/V acyclovir for 3 weeks Gradually improved Weaned off ventilator Became alert Left hemiparesis improved No seizures

Present condition Oriented; independent Right ptosis is persistent; though eye movements

and pupillary size are normal

Discussion

Decompression craniotomy in HSVE Useful option in cases with mass effect and poor

response to acyclovir and anti-oedema measurs Some reports suggest that in addition partial

resection of temporal lobe is of benefit additional reduction of infectious material can be achieved Child’s Nerv Syst 1999; 15: 84–86

Malignant HSVE?

Surprisingly few cases of decompression have been described in literature 2 cases

Surg. Neurol. 2002; 57 (1): 20

Review of literature: Total 13 cases of infectious encephalitis requiring

decompression 6 had HSVE

J Neurosurg. 2008; 108 (1): 174

What is new in HSV encephalitis? Long Term Treatment of Herpes Simplex

Encephalitis With Valacyclovir Ongoing trial

The purpose of the study is to determine if treatment with oral valacyclovir 2 gm TDS for 90 days is both effective and safe after completing i/v acyclovir treatment and if it can increase survival with or without mild impairment of the brain and mental functions

Case 2

21/F Headache

Bilateral Throbbing Increasing severity

Occasional vomiting

On examination Conscious/oriented Bilateral papilloedema No other deficit No neck stiffness

Routine lab: normal CSF

Opening pressure 40 cm Proteins 34 Sugar 76 (BSL 122) Cells 2 (100% Lympho)

Management Drained 30 cc CSF Low salt diet Acetazolamide 1000 mg/d Weight loss 3 kg

Improved gradually At present

No symptoms No papilloedema

Lateral sinus stenosis

IIH and lateral sinus stenosis

By definition IIH is idiopathic Venous disorders can cause rise in intracranial

pressure and present with syndrome like IIH Venous sinus thrombosis Dural venous fistulas Venous sinus compression

In many patients with IIH, neuroimaging shows narrowing of the transverse sinuses

Controversy

Whether this abnormality is cause or consequence of increased intracranial pressure?

Cause: Stenoses → Obstruction to venous outflow → ↑

intracranial venous pressure proximal to the stenosis → reduction in CSF absorption via the arachnoid granulations → ↑ CSF pressure

In this setting, a pressure gradient across the stenosis can be measured

Reconstruction of the venous lumen with endovascular

stents would be effective in lowering elevated CSF pressure

Controversy

Whether this abnormality is cause or consequence of increased intracranial pressure?

Consequence: ↑ intracranial CSF pressure → secondary narrowing

of sinus lumen by compression It can be reversed by lumbar puncture or shunt

surgery procedures

The role of lateral sinus stenosis remains to be evaluated

There are studies in favor of both hypotheses

Cause

Endovascular treatment of idiopathic intracranial hypertension Neurology 2008; 70: 641-647

Conclusion: Importance of venous sinus disease in etiology of

IIH is underestimated Patients with IIH in whom a venous sinus stenosis is

demonstrated by MRV should be evaluated with direct retrograde cerebral venography and manometry

In patients with venous sinus stenosis who do not respond to medical treatment, endovascular stent

placement seems to be an interesting option

Consequence

Transverse sinus stenoses persist after normalization of the CSF pressure in IIH Neurology 2005; 65: 1090-1093

Conclusion: Transverse sinus stenoses, as revealed by MR

venography, persist in patients with idiopathic intracranial hypertension after normalization of CSF pressure, suggesting the lack of a direct relationship between the caliber of sinus and CSF pressure

Repeat MRI

Venous channels are becoming more important and controversial with association with more and more neurological diseases IIH MS

Case 3

Middle aged male H/O pleural effusion 6 months ago

Treated with AKT On INH and Rifa at present

No respiratory symptoms CXR: normal

Presented with 14 days history of Headache Vomiting

On examination: Conscious; oriented Fundi: normal Neck stiffness No other deficit

CT scan brain: Normal

Investigations: CSF:

Proteins 176 Sugar 45 (BSL 109) Cells 30 (100% L)

Hemogram HIV: -ve Metabolic lab: normal

Started on 4 drugs AKT with steroids after CSF report

Other CSF reports were pending

Next day CSF India ink +ve CSF PCR for TB -ve

Started on i/v amphotericine B

His headache gradually reduced Required CSF drainage twice

HIV was repeated by ELISA: -ve

CD4+ count: 68

DNA quantative PCR for HIV: -ve

Improved subsequently Discharged on

Fluconazole TMP/SMX AKT

Repeat CD4+ count after 2 months: 212

Now presented with Fever Weight loss Lymphadenopathy

Idiopathic CD4 lymphocytopenia (ICL) CD4+T cells <300 or a CD4+ cell count <20%

of total T cell on two occasions No evidence of infection on HIV testing Absence of any defined immunodeficiency or

therapy associated with depressed levels of CD4+ T cells

Dr Pallavi Bhargav

Case 4

40 years old male Presented with sudden onset severe headache

Started while taking hot water bath Over vertex and occipital region Associated with nausea No loss of consciousness

No past H/O similar headache, trauma, fever C/O DM on OHAs

Came to hospital in 1 hour Headache was already subsiding then No neurological deficit No neck stiffness

Admitted Received NSAID Non-contrast CT scan brain: normal No headache in next 36 hours Discharged

Next day again had similar headache while taking hot water bath Lasted for 1 hour

Readmitted No deficit

MR-angio was done

MR-angio

When seen Comfortable No deficit

Investigations Metabolic lab: normal Counts: normal CSF

No xanthochromia Protein 83 Sugar 98 Cells 15 (100% L)

What is the diagnosis? Thunderclap headache

To be investigated for cause Any further investigations?

DSA Treatment options?

Received indomethacin on SOS basis

DSA

Repeat MR-angio

Thunderclap headache (TCH)

IHS 2 Diagnostic criteria:A. Severe head pain fulfilling criteria B and C B. Both of the following characteristics:

Sudden onset, reaching maximum intensity in <1 minute

Lasting from 1 hour to 10 days C. Does not recur regularly over subsequent weeks or

months D. Not attributed to another disorder (in case of primary)

Notes: Headache may recur within the first week after onset In case of primary, normal CSF and normal brain imaging

are required

Causes of secondary TCH: SAH Sentinel hemorrhage Intracerebral haemorrhage Venous sinus thrombosis Arterial dissection (intra- and extracranial) CNS angiitis Reversible cerebral vasoconstriction syndromes Pituitary apoplexy Colloid cyst of the third ventricle CSF hypotension Acute sinusitis

Comparison of MRAs (9/12 and 13/12)

Comparison of MRAs (9/12 and 13/12)

9/12/2010

12/12/2010

Reversible cerebral vasoconstriction syndrome (RCV) Relatively newer name Previous names

Benign angiopathy of the central nervous system Migrainous angiitis Post-partum angiopathy Call-Fleming syndrome

Stroke 1988; 19: 1159-1170

Clinical features Thunderclap headache

Tend to recur for few weeks Focal deficits

Strokes Bleeds Posterior reversible leucoencephalopathy

Seizures Predisposing factors in 60% patients

Pregnancy and puerperium Exposure to drugs

Diagnosis Angiography (CTA / MRA / DSA) demonstrated

multifocal or segmental narrowing Improvement in vasoconstricton in 12 weeks

No CT or CSF evidence of SAH Normal or near normal CSF Appropriate clinical history (thunderclap headache)

Differential diagnosis Posterior reversible leucoencephalopathy syndrome CNS vasculitis

Treatment No large studies Nimodepine or verapamil Short course of steroids (mostly in earlier reports) Intra-arterial therapy in severe cases

Underdiagnosed 83 patients with TCH

Neurology 2006; 67: 2164-9 56 patients had thunderclap headache of unknown

etiology When these patients underwent MRA, 39% were

found to have reversible cerebral vasoconstriction In cases of thunderclap headache, if CT and CSF

are normal, a noninvasive angiography should be done

67 patients with RCVS Brain 2007; 130 (12): 3091-31

21% of patients who ultimately demonstrated vasoconstriction initially had normal angiographic studies

In other words, there was a lag between the onset of symptoms and the presence of vasoconstriction

This suggests that cerebral vasoconstriction may begin in smaller distal vessels that extend beyond the resolution of MRA before involving larger proximal cerebral blood vessels

Bath induced thunderclap headache

Cephalalgia 2008; 28: 524-530 21 patients Bathing was the initial trigger for thunderclap

headaches in 9 (43%) 15 (71%) had other non-bath-related attacks 18 (86%) reported that the headache occurred

immediately when water was sprayed over their body, with warm water (52%) as the most common

13 (62%) had RCV on imaging Nimodipine was effective in stopping further

attacks in 84%

In case of thunderclap headache, if CT and CSF are normal, angio should be done in all to look for not only aneurysm but also for RCV

Case 5

65/M Acute right hemiparesis

Clinically Right hemianopia Right hemiparesis Right hypoaesthesia

Hyperdense PCA sign

HPCA sign Stroke 2006; 37: 399

Detected in >1/3 of all patients with PCA infarct, suiting incidence of hyperdense MCA

This sign may not only be helpful in the early diagnosis of PCA infarction but might also act as a prognostic marker in acute PCA territory

ischemic stroke

Case 6

Young lady H/O electric shock Became unconsciousness When came to casualty had cardiorespiratory

arrest Monitor showed asystole

Resuscitated Shifted to ICU

On examination On ventilator and intropic support Unconscious (GCS 3/15) Pupils 3 mm NRL Doll eye movement absent Corneal reflexes absent

Diagnosis: Hypoxic brain injury secondary to cardio-respiratory

arrest

Treatment?

Therapeutic hypothermia

Decreases cerebral metabolic demand Clinical trials and meta-analysis showed

improved outcome with hypothermia Resuscitation 2007; 73: 29-39 NEJM 2002; 346(22): 1756

16-23% improved outcome Cool up to 33°C (32-34°C) for 12-24 hours To be started within min to hours after arrest

Problems Technically difficult Ideal induction technique

Internal vs. external Target temperature Duration Re-warming rate

Complications Shivering Arrhythmias

Hypothermia: technique

Dr. Sameer Jog

Case 7

Middle aged male

Immunocompramised CD4+ count 55 On ART

Presented with 2 months H/O Asymmetric ataxia (R>L) Dysarthria No pyramidal signs/dementia/bladder involvement

CSF Protein 55 Sugar 67 Cells 4 (100% L)

Treatment Continued on ART Physiotherapy

Continued worsening Bed ridden

Diagnosis? PML

He underwent follow up MRI after 1 year CD4+ count at this stage was 107

Hot cross bun appearance

The sign is due to Selective loss of myelinated transverse

pontocerebellar fibers and neurons in pontine raphe Preservation of pontine tegmentum and

corticospinal tracts Has been described in

MSA-c Parkinsonism due to vasculitis SCA 2 SCA 3 vCJD

Has not been descried in PML till date