Subacute sclerosing pan encephalitis (SSPE) also known as Dawson Disease, Dawson encephalitis, and measles encephalitis is a rare and chronic form of progressive brain inflammation caused by a persistent infection with measles virus. In this presentaion i will a case a sspe and give u some information regarding daignosis and treatment

1. Dr. Amit Vatkar
MBBS, DCH, DNB Pediatrics
Fellow in Pediatric Neurology, Mumbai
Trained in Neurophysiology & Epilepsy, USA
Contact No. : +91-8767844488
Email: vatkaramit@yahoo.com
SUBACUTE
SCLEROSING
PANENECPHALITISSUBACUTE SCLEROSING
PANENECPHALITIS

2. 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

3. • 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.

4. • 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

5. 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

6. - 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 irritation
Other systems: normal
CNS EXAMINATION

8. PROVISONAL DIAGNOSIS
Myoclonic jerks for evaluation probably SSPE

9. 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

10. 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

11. SLOW WAVE PERIODIC BURST

13. EEG :
Periodic complexes with sharp wave &
spike discharges seen.
S/O SSPE.

14. FINAL DIAGNOSIS
SSPE
SUBACUTE SCLEROSING
PANENCEPHALITIS

15. 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

16. 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.

17. 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

18. STAGES OF SSPE
Stages Parts of brain involved
I
II
III
IV
Cortex
Cortex + subcortical white matter
Deep nuclear structures
Entire CNS

19. JABBOUR’ S Clinical stages of SSPE (1969)
Stage 1: subtle features
• Behavioural changes
• Decreased scholastic performance
• Irritability
• Hyperactivity
• Negativism
• Psychosis

20. 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

21. 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

22. STAGE 4
• Mutism
• Generalised hypotonia
• Loss of cortical function
• Random eye movements
• Vegetative stage

23. 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

24. 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.

25. 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.

26. 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.

27. TO CONCLUDE
PREVENTION IS THE BEST
TREATMENT.

28. 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 disorders
2. Developmental Disorders including delayed speech, motor milestones, and coordination issues
3. Autism and other Behavioural disorders, including attention-deficit/hyperactivity disorder (ADHD),
school failure and sleep problems
4. Movement Disorders,
5. Cerebral palsy, muscular dystrophy, and nerve muscle disorders
6. Headaches, including migraines

29. Dr. Amit Vatkar
Pediatric Neurologist, Navi Mumbai
MBBS, DNB
Email: vatkaramit@yahoo.com
Contact No.: +91-8767844488
Visit us at: http://pediatricneurology.in/
THANK YOU !