Objective: We aimed to study the frequency of movement Disorders other than myoclonus seen in patients with subacute sclerosing panencephalitis (SSPE).

Background: SSPE is a progressive neurological disease caused by persistent defective measles virus in the CNS.¹ It is invariably fatal. Patients usually have a clinical course characterized by progressive cognitive decline, seizures, myoclonus, ataxia, visual disturbance, and later vegetative state, eventually leading to death. Movement disorders are rarely part of the clinical features of patients with SSPE. Here we report a retrospective analysis of the frequency of movement disorders observed in our twenty-three (23) cases of SSPE.

Method: We retrospectively reviewed our video database of SSPE from our “Parkinson disease and movement disorder clinic”. We staged each patient according to Jabbour staging.²

Results: The mean age of our patients was 10.4 ± 4.066 years (range 3.5 -22 years). There were 14 males and 9 females. Of the 23 patients apart from myoclonus movement disorders seen was dystonia in 13 patients, tremor in 9 patients, Parkinsonism and stereotypy in 1 patient each. Out of the 13 patients with dystonia, 7 patients had multifocal, 3 patients had focal and the other 3 patients had generalized dystonia. Most of the focal dystonia patients had pre-dominantly upper limb involvement. Patients with multifocal dystonia had limb and oromandibular involvement. The tremor was the second common movement disorder seen in 9 patients (dystonic= 3, and postural tremors in the limbs=6 patients in which 4 patients also had rest component). One patient with dystonic tremors in limbs had camptocormia also.

Conclusion: Our study concludes that movement disorders are important clinical findings of SSPE in the form of dystonia, tremor, parkinsonism, stereotypy. It narrows down the list of differentials when a child presents with rapid cognitive decline along with movement disorders.

References: 1. Garg RK. Subacute sclerosing panencephalitis. J Neurol. 2008;255(12):1861–1871. 2. Jabbour J, Duenas D, Modlin J. SSPE-clinical staging, course, and frequency. Arch Neurol. 1975;32(7):493–494.

« Back to MDS Virtual Congress 2021

MDS Abstracts - https://www.mdsabstracts.org/abstract/movement-disorders-in-subacute-sclerosing-panencephalitis-patients/