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HSVE IIH ICL Pallavi to talk TCH DSA PCA sign Hypothermia Jog to talk PML PowerPoint PPT Presentation


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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

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HSVE IIH ICL Pallavi to talk TCH DSA PCA sign Hypothermia Jog to talk PML

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Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

HSVE

IIH

ICL

Pallavi to talk

TCH

DSA

PCA sign

Hypothermia

Jog to talk

PML


Case 1

Case 1


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Metabolic lab: WNL

  • WBC Counts: 9500

  • HIV: -ve


Mri brain

MRI brain


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 3rd day

    • No fever

    • Single SG seizure

    • More drowsy (GCS 7/15)

    • Left hemiparesis

    • At night

      • Right pupil dilated

    • Intubated


Ct scan brain

CT scan brain


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 7th day

    • Unconscious (GCS 4/15)

    • On ventilator

    • Right III nerve palsy

    • Left pupil also became dilated

    • Dense left hemiparesis


Repeat ct scan brain

Repeat CT scan brain


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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

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?


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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

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

Case 2


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 21/F

  • Headache

    • Bilateral

    • Throbbing

    • Increasing severity

  • Occasional vomiting


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • On examination

    • Conscious/oriented

    • Bilateral papilloedema

    • No other deficit

    • No neck stiffness


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Routine lab: normal

  • CSF

    • Opening pressure40 cm

    • Proteins34

    • Sugar76(BSL 122)

    • Cells2 (100% Lympho)


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Management

    • Drained 30 cc CSF

    • Low salt diet

    • Acetazolamide 1000 mg/d

    • Weight loss 3 kg

  • Improved gradually

  • At present

    • No symptoms

    • No papilloedema


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Lateral sinus stenosis


Iih and 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

    • Duralvenous fistulas

    • Venous sinus compression

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


Controversy

Controversy

  • Whetherthis abnormality is cause or consequence of increasedintracranial pressure?

  • Cause:

    • Stenoses→ Obstruction to venous outflow → ↑ intracranial venous pressure proximalto the stenosis → reduction in CSF absorption via the arachnoid granulations → ↑ CSF pressure

    • In this setting, a pressure gradient across the stenosis canbe measured

    • Reconstruction of the venous lumen with endovascularstents would be effective in lowering elevated CSF pressure


Controversy1

Controversy

  • Whetherthis abnormality is cause or consequence of increasedintracranial pressure?

  • Consequence:

    • ↑ intracranial CSF pressure → secondary narrowingof sinus lumen by compression

    • It can be reversed bylumbar puncture or shunt surgery procedures


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • The role of lateral sinus stenosis remains to be evaluated

  • There are studies in favor of both hypotheses


Cause

Cause

  • Endovascular treatment of idiopathic intracranial hypertension

    • Neurology 2008; 70: 641-647

  • Conclusion:

    • Importance of venous sinus disease in etiologyof IIH is underestimated

    • Patients with IIH in whom avenous sinus stenosis is demonstrated by MRV should be evaluated with direct retrograde cerebral venographyand manometry

    • In patients with venous sinus stenosis who do not respond to medical treatment, endovascular stentplacement seems to be an interesting option


Consequence

Consequence

  • Transverse sinus stenoses persist after normalization of the CSF pressure in IIH

    • Neurology 2005; 65: 1090-1093

  • Conclusion:

    • Transverse sinus stenoses, as revealed byMR venography, persist in patients with idiopathic intracranialhypertension after normalization of CSF pressure, suggestingthe lack of a direct relationship between the caliber of sinusand CSF pressure


Repeat mri

Repeat MRI


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

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

    • IIH

    • MS


Case 3

Case 3


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • On examination:

    • Conscious; oriented

    • Fundi: normal

    • Neck stiffness

    • No other deficit

  • CT scan brain:

    • Normal


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Investigations:

    • CSF:

      • Proteins 176

      • Sugar45 (BSL 109)

      • Cells30 (100% L)

    • Hemogram

    • HIV: -ve

    • Metabolic lab: normal


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • His headache gradually reduced

  • Required CSF drainage twice

  • HIV was repeated by ELISA: -ve

  • CD4+ count: 68

  • DNA quantative PCR for HIV: -ve


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Improved subsequently

  • Discharged on

    • Fluconazole

    • TMP/SMX

    • AKT


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Repeat CD4+ count after 2 months: 212

  • Now presented with

    • Fever

    • Weight loss

    • Lymphadenopathy


Idiopathic cd4 lymphocytopenia icl

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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Dr Pallavi Bhargav


Case 4

Case 4


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • 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


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • Next day again had similar headache while taking hot water bath

    • Lasted for 1 hour

  • Readmitted

    • No deficit

  • MR-angio was done


Mr angio

MR-angio


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • When seen

    • Comfortable

    • No deficit

  • Investigations

    • Metabolic lab: normal

    • Counts: normal

    • CSF

      • No xanthochromia

      • Protein83

      • Sugar98

      • Cells 15 (100% L)


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

  • What is the diagnosis?

    • Thunderclap headache

      • To be investigated for cause

  • Any further investigations?

    • DSA

  • Treatment options?

    • Received indomethacin on SOS basis


Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

DSA


Repeat mr angio

Repeat MR-angio


Thunderclap headache tch

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


  • Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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)


    Comparison of mras 9 12 and 13 121

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


    9 12 2010

    9/12/2010


    12 12 2010

    12/12/2010


    Reversible cerebral vasoconstriction syndrome rcv

    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


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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)


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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

    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%


  • Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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

    Case 5


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 65/M

    • Acute right hemiparesis

    • Clinically

      • Right hemianopia

      • Right hemiparesis

      • Right hypoaesthesia


    Hyperdense pca sign

    Hyperdense PCA sign

    • HPCA sign

      • Stroke 2006; 37: 399

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

    • This sign may notonly be helpful in the early diagnosis of PCA infarction butmight also act as a prognostic marker in acute PCA territoryischemic stroke


    Case 6

    Case 6


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • Young lady

    • H/O electric shock

    • Became unconsciousness

    • When came to casualty had cardiorespiratory arrest

      • Monitor showed asystole

    • Resuscitated

    • Shifted to ICU


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • 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

    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


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • Problems

      • Technically difficult

      • Ideal induction technique

        • Internal vs. external

      • Target temperature

      • Duration

      • Re-warming rate

    • Complications

      • Shivering

      • Arrhythmias


    Hypothermia technique

    Hypothermia: technique

    • Dr. Sameer Jog


    Case 7

    Case 7


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    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


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • CSF

      • Protein55

      • Sugar67

      • Cells4 (100% L)

    • Treatment

      • Continued on ART

      • Physiotherapy

    • Continued worsening

      • Bed ridden


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • Diagnosis?

      • PML


    Hsve iih icl pallavi to talk tch dsa pca sign hypothermia jog to talk pml

    • He underwent follow up MRI after 1 year

      • CD4+ count at this stage was 107


    Hot cross bun appearance

    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


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