Dr. Amit VatkarMBBS, DCH, DNB Pediatrics

Fellow in Pediatric Neurology, MumbaiTrained in Neurophysiology & Epilepsy, USA

Contact No. : +91-8767844488Email: vatkaramit@yahoo.com

SUBACUTE SCLEROSING

PANENECPHALITISSUBACUTE SCLEROSING PANENECPHALITIS

10 year old male child Siva Normal child till 1year back,• Had poor school performance for past 1 year

• H/O difficulty in walking with frequent falls for past 1 month

• c/o sudden jerky movements involving both upper limbs & lower limbs & bedridden for 7 days.

• H/O jerks decreased in frequency during sleep.

• Received treatment for above with anticonvulsants & referred here.

HISTORY

• No H/O fever / vomiting / altered sensorium.

• No H/O dog bite/ recent vaccination.

• No H/O visual or hearing impairment.

• Not able to eat because of jerks.

• He obeys & carried out simple commands & talks few words.

• No H/O bowel & bladder disturbances.

• No previous H/O measles/ exanthematous illness.

• Immunisation H/O details not known.• No H/o prior developmental delay.• No H/O similar illness in the family.

19 yr 10 yr

child conscious

afebrile no neurocutaneous markers

vitals: Anthropometry PR : 100 / min Wt : 25 Kg/32.5 Kg RR : 20/ min Ht : 130/132 cm BP : 100/70 mmHg HC : 50 cm

EXAMINATION

- posture : lying in bed flexed at thigh & knee- Sudden jerky movements of both UL & LL- speaks few words, respond to simple commands- no cranial nerve palsy or dysfunction

- motor system: tone increased in all 4 limbs Power > 3 in all 4 limbs DTR increased in all 4 limbs plantar B/L extensor

- able to perceive pain - cerebellar system & gait could not be elicited

No signs of meningeal irritationOther systems: normal

CNS EXAMINATION

PROVISONAL DIAGNOSIS

Myoclonic jerks for evaluation probably SSPE

DIFFERENTIAL DIAGNOSIS

• Juvenile myoclonic epilepsy( Janz syndrome)– Onset between 12- 16 yrs, on awakening, yrs later with GTCS, EEG- 4 –

6/sec irregular spike & wave pattern enhanced by photic stimulation

• Lafora disease– 10 – 18 yrs with GTCS, with mental deterioration with myoclonic jerks

ultimately with cerebellar and extrapyramidal signs, EEG- polyspike wave discharges in occipital region

• MERRF– Myoclonic epilepsy, mitochondrial myopathy & cerebellar signs,

associated with optic atrophy, SNHL, dementia

INVESTIGATION

• Hb : 8.4 g• TC : 10,800/ mm3• DC : P 69 L 28 E 3

• Blood sugar : 80 mg/dl• Blood urea : 46 mg/dl• Sr creatinine : 1.6 mg/dl

• CT brain : normal

SLOW WAVE PERIODIC BURST

EEG :

Periodic complexes with sharp wave & spike discharges seen.

S/O SSPE.

FINAL DIAGNOSIS

SSPESUBACUTE SCLEROSING

PANENCEPHALITIS

SSPE

• Most common form of chronic encephalitis.

• Incidence: 1 in 1,000,000

• First described by Dawson in 1934 as “Inclusion body encephalitis”

• Van Bogaert named it as SSPE.

• M: F 2:1

• Hispanic origin

SSPE

• Measles at an early age (2- 4 yr ) favors development of SSPE.

• persistent infection - altered measles virus that is harbored intracellularly in CNS & altered host response to virus.

• Interval :7 – 10 yr

• Insidious onset of neurological symptoms – juvenile period.

• Leading to an inexorable neurodegenerative process & death within 1 – 3 yr of onset.

PATHOLOGY

• Inflammation , necrosis and gliosis of brain• Microscopy : - perivascular infiltration - Neuronophagia - Proliferation of microglia & astrocytes - Coudry type A and B inclusion bodies• Cytokines are detected in brain lesions

STAGES OF SSPE

Stages Parts of brain involved

I II III IV

Cortex Cortex + subcortical white matter Deep nuclear structures Entire CNS

JABBOUR’ S Clinical stages of SSPE (1969)

Stage 1: subtle features

• Behavioural changes• Decreased scholastic performance• Irritability • Hyperactivity • Negativism • Psychosis

STAGE 2

• Myoclonic jerks• Choreo-athetosis• Tremor • Ballismus – dystonic posture• Ataxia • Spasticity

• Ocular changes: 50% of patients choreoretinitis, reduced visual acuity, optic atrophy,

papilledema, uveitis, retrobulbar neuritis

STAGE 3

• Increased spasticity • Myoclonic jerks may decrease or disappear• Decorticate or decerebrate posture• Noisy breathing• Autonomic disturbances• May die of hyperthermia , cvs collapse,

hypothalamic disturbances

STAGE 4

• Mutism• Generalised hypotonia• Loss of cortical function• Random eye movements• Vegetative stage

DIAGNOSTIC CRITERIA

• Compatible clinical course & atleast one of the following 1) measles antibody detected in CSF 2) characteristic EEG 3) typical histological findings and / or isolation of virus/ viral

antigen in brain tissue

• Elevated measles Ab titres using complement fixation technique.

CSF > 1:8 dilution serum > 1:24 dilution

CT & MRI non-specific for SSPE

• CT : - variable cortical atrophy and ventricular

dilatation - focal and multi focal whitematter

abnormalities.

• MRI : hypodense in T1 weighted images in white

matter.

EEG

• Described by Radermecker and Cobb and Hill.

• Normal in early stages.

• High amplitude 300-1500 microvolts repetitive polyphasic sharp and slow wave complexes of 0.5 – 2 sec in duration , that occur every 4-15 seconds and are syncronous with myoclonic jerks.

• Terminal stages – disorganisation of background.

TREATMENT

• Primarily supportive.• Amanitide : anti-RNA agent ,prolongs life.• Isoprinosine (inosiplex ) - purine derivative - beneficial drug for reducing morbidity &

mortality - 100 mg/kg/day• Plasmapheresis : not beneficial• Others :interferon α2b , pyroncopolymer , 5-bromo-2

deoxyuridine.

TO CONCLUDE

PREVENTION IS THE BEST

TREATMENT.

About Dr. Amit Vatkar

Dr. Amit Vatkar is a Pediatric Neurologist from Mumbai, India. He has completed his fellowship in Pediatric Neurology with specialising in Epilepsy surgery workup from Hinduja hospital under the guidance of Vrajesh Udani, top neurologist in India. He has also been trained in Epilepsy & neurophysiology at Case Western Reserve University at Cleveland under the guidance of Dr. Hans Luders.

He specialises in Clinical Neurophysiology (EEG, EMG and NCV). He also provides portable EEG services in Mumbai.

Currently, He is supporting many schools for children with special needs. He is attached to major hospitals in Mumbai where he consults pediatric neurological cases. His areas of expertise are

1. Epilepsy, Seizure disorders2. Developmental Disorders including delayed speech, motor milestones, and coordination issues3. Autism and other Behavioural disorders, including attention-deficit/hyperactivity disorder (ADHD), school failure and sleep problems4. Movement Disorders, 5. Cerebral palsy, muscular dystrophy, and nerve muscle disorders 6. Headaches, including migraines

Dr. Amit VatkarPediatric Neurologist, Navi Mumbai

MBBS, DNB

Email: vatkaramit@yahoo.comContact No.: +91-8767844488

Visit us at: http://pediatricneurology.in/

THANK YOU !