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Abstract Details

A Potential Cure in Adult Onset Subacute Sclerosing Panencephalitis
General Neurology
P6 - Poster Session 6 (12:00 PM-1:00 PM)
6-004

Subacute sclerosing panencephalitis (SSPE), a slow viral infection of central nervous system caused by mutated measles virus, is an insidiously progressive disorder invariably leads to fatality within 1-3 years after onset. Various medications such as isoprinosine, interferons and ribavirin have been tried unsuccessfully till date.

 A case of adult onset SSPE who improved after treatment and is presently in remission is reported.
Case Report
A 21-year lady presented with recurrent myoclonic jerks for 6 months, recurrent generalized tonic clonic seizures and one episode of status epilepticus surviving  cardiorespiratory arrest. She also had psychosis for a year controlled on aripripazole, cognitive decline, slow gait and required 2-person support. She was cachexic, irritable, had generalized lead-pipe rigidity and bradykinesia. She had quasiperiodic negative myoclonus every 10-14 seconds, with frequent major jerks causing her to fall . MRI brain showed mild diffuse cerebral and cerebellar atrophy and EEG showed long duration long interval periodic complexes. ELISA for IgG for measles was positive and a diagnosis of SSPE was made. Being refractory to multiple antiepileptic drugs and unable to afford medications generally recommended for SSPE,  she was given a trial of levamisole with gradual escalation. After no initial change, a subtle decrease in frequency of major jerks was noted after 2 months. By 3-6 months, major jerks had reduced and periodic myoclonus progressively became infrequent. By 20 months, there was rare myoclonus in 8-10 hours and these completely subsided by 21 months. At 2 years after starting levamisole, she was able to walk unsupported and talk more fluently. EEG at this time showed normal background with no epileptiform activity. 

Levamisole, an immunopotentiating agent, generally has poor effect in autoimmune disorders and cancer. While no drug has yielded satisfactory results in SSPE till date, this case demonstrates the curative potential of levamisole in adult onset SSPE.

Authors/Disclosures
Samhita Panda, MD, DM (Department of Neurology, AIIMS, Jodhpur)
PRESENTER
Dr. Panda has nothing to disclose.