Objective: We present the clinical profile, imaging, treatment trends and outcomes of a cohort SSPE patients who presented to our Institute with movement disorders.
Background: Subacute sclerosing panencephalitis (SSPE) is an progressive neurological disease with eencephalitic syndrome that develops several years following as secondary to a measles infection and is associated with widespread demyelination. It is characterized by a rapidly progressive clinical course of cognitive decline and movement disorders in young children and adolescents.
Method: This is a prospective cohort study of consecutive patients who presented to the Neurology OPD in a tertiary care Institute in Northern part of India and seen by the first author.
Results: We had 23 patients who presented over a three year period. We found myoclonus(80%) and dystonia(70%) to be the commonest movement disorders associated with the condition. 78% of patients had more than one type of movement disorders. Though the movement disorders are the common presenting manifestation, imaging abnormalities of the basal ganglia are very rare on MR imaging, and we found only one patient with putaminal hyperintensities. MR imaging was normal in two patients. All but one patient had cognitive decline at presentation, hinting that patients with normal cognition can also have SSPE. Upto 40% patients improved atleast one Jabbour grade with intrathecal interferon therapy – a finding that offers hope to patients and families of those with SSPE.
Conclusion: SSPE is associated with multiple hyperkinetic movement disorders in 78% of patients and basal ganglia abnormalities are very rare on imaging. Since several patients showed improvement with intrathecal interferons, this treatment should be made easily accessible by doing an Ommaya reservoir implantation, as this could improve adherence to intrathecal interferons compared to repeated lumbar punctures.
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To cite this abstract in AMA style:
A. Elavarasi, D. Dash, M. Tripathi, R. Singh, D. Vibha, A. Garg. Spectrum of Movement disorders, Imaging findings in SSPE and response to therapy – A prospective cohort study [abstract]. Mov Disord. 2024; 39 (suppl 1). https://www.mdsabstracts.org/abstract/spectrum-of-movement-disorders-imaging-findings-in-sspe-and-response-to-therapy-a-prospective-cohort-study/. Accessed November 21, 2024.« Back to 2024 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/spectrum-of-movement-disorders-imaging-findings-in-sspe-and-response-to-therapy-a-prospective-cohort-study/