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Indian Pediatr 2013;50: 1067-1068 |
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Subacute Sclerosing Panencephalitis With Tics
as First Symptom
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Pranab Kumar Dey and Tunisha Bhattacharya
Department of Paediatrics, Medical College,
Kolkata, India.
Email: [email protected]
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A 10-year-old boy presented with complex tics
involving shoulder and facial muscles since five months. Tics were
insidious in onset, gradually progressive, changing in type and
location, suppressed with effort. He suffered from measles at two years
of age. Examination revealed stereotypic repetitive movements and
bradykinesia. The initial provisional diagnosis was Tic disorder of
childhood. After two weeks he developed spontaneous periodic generalised
myoclonus followed by ataxia, progressive slurring of speech and
decreased speech volume. Investigations showed normal complete hemogram,
ESR, ASO titre, anti-nuclear factor, liver function test, serum
electrolytes, copper, cerruloplasmin, parathyroid hormone and lactic
acid levels. Nerve conduction studies, electromyography, axillary skin
and muscle biopsy reports were normal. Computed tomography scan and
magnetic resonance imaging (MRI) of the brain were normal. However,
electro-encephalography showed periodic generalised bursts of
high-amplitude and slow wave complexes recurring at intervals of 6-8
seconds. Cerebrospinal fluid study revealed IgG measles 376.11 U/L
(dilution 1:4) and serum IgG measles 329.09 U/L (dilution 1:404) by
ELISA. Serum anti-measles antibody titre (5.22RFV) were elevated above
normal range (>0.7RFV) by ELFA. Blood and CSF serology for Herpes
simplex, Toxoplasma and Cytomegalovirus were all negative (both IgM and
IgG). He was treated with Isoprinosine (100mg/kg/day) but therapy with
interferon remained unaffordable. Sodium valproate and clonazepam were
added for control of myoclonus. Improvement in seizure control and tics
were noted after six months of continuous follow up. Repeat MRI showed
focal areas of hyperintesities in cortex and subcortical white matter of
both frontal and adjacent high parietal region in T 2
weighted FSE and FLAIR image after two years of
follow up.
Myoclonus is brief, involuntary twitching of
a muscle or group of muscles, may be mistaken as tics and has been
described with SSPE [1]. Tics are characterized by abrupt, repetitive
movements, commonly preceded by premonitory sensation of an urge and can
be suppressed with effort [2]. Tourette syndrome is most frequent cause
of tics, others are insults to the brain; particularly the basal
ganglia, infection, stroke, head trauma, certain toxins, drugs and
various sporadic, genetic, neurodegenerative disorders [2].
Differential diagnosis of childhood cognitive deterioration and movement
disorders like Wilsons disease, childhood systemic lupus erythematosus,
hypoparathyroidism, Hallervorden Spatz disease and progressive myoclonic
epilepsy were excluded due to lack of clinical, laboratory and imaging
findings.
Previously reported atypical features of SSPE include
isolated psychiatric manifestations, poorly controlled seizures, stroke
like onset, hemiparesis, acute encephalopathy, cerebellar ataxia, visual
disturbances, symptoms suggestive of intracranial space occupying lesion
and parkinsonism like features [3-5]. While this association may be
coincidental, but the possibility of tics as a result of insult to the
brain due to SSPE should be kept in mind.
Acknowledgment: Dr Kaberi Basu, Professor,
Department of Pediatrics for helping to diagnose and manage the case.
References
1. Bonthius DJ, Stanek N, Grose C. Subacute
sclerosing panencephalitis, a measles complication, in an
internationally adopted child. Emerging Infectious Dis. 2000;6:377-81.
2. Mejia NI, Jankovic J. Secondary tics and
tourettism. Rev Bras Psiquiatr. 2005;27:11-7.
3. Sarkar N, Gulati S, Dar L, Broor S, Kalra V.
Diagnostic dilemmas in fulminant subacutesclerosingpanencephalitis
(SSPE). Indian J Pediatr. 2004;71:365-7.
4. Dimova P, Bojinova V. Sub acute
sclerosinpanen-cephalitis with atypical onset: clinical computed
tomography and magnetic resonance imaging correlations. J Child Neurol.
2000;15:258-60.
5. Misra A, Roy A, Das Kr. S. Parkinsonian presentation of SSPE: a
report of two cases. Neurology Asia. 2008;13:
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