Category: Myoclonus
Objective: To report unusual manifestations Subacute Sclerosing Panencephalitis.
Background: Subacute Sclerosing Panencephalitis (SSPE) is a slow viral infection caused by measles virus resulting in progressive inflammation and gliosis of the neuroparenchyma. Common presentations of SSPE are cognitive impairment, stereotyped myoclonus, seizures, and characteristic periodic discharges in the electroencephalogram. However unusual presentations of SSPE often leads to delay in diagnosis, which in turn leads to delay in treatment initiation. Hence it is important to know about unusual presentations of SSPE.We report three cases with varied clinical manifestations of SSPE.
Method: This is a series of three cases of SSPE with atypical presentations.
Results: Case 1: 14-year-old boy presented with features of recurrent spontaneous falls without any loss of consciousness since 2 years, commonly backwards associated with bending of trunk without any cognitive disturbance. On examination had features of slow myoclonic jerks involving paraspinal muscles.
Case 2: 27-year-old male patient presented to us with features of personality and behavior change since 3 years with features of jerky movements involving both hands since 2 years. At the time of presentation, patient was in akinetic rigid state with intermittent slow myoclonus involving both upper limbs.
Case 3: 11-year-old boy presented with features of jerky movement involing left upper limb, which was present throughout the day since 6 months and was absent in sleep. There was no cognitive decline or personality change. On examination, he had slow myoclonic jerks of left upper limb.
None of the above patients were vaccinated for measles vaccine before. CSF for measles IgG antibodies were significantly elevated (>1:625), EEG in all above patients showed long interval high amplitude periodic discharges suggesting SSPE.
Conclusion: In conclusion, in countries where SSPE is still encountered in large numbers, one should be cautious about unusual manifestations of the common disease. This also highlights the importance of vaccination, especially in developing countries like India
To cite this abstract in AMA style:
G. Harikrishna, K. Mala, SK. Kodapala. Atypical Manifestations of Subacute Sclerosing Panencephalitis (SSPE): A Case Series from India [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/atypical-manifestations-of-subacute-sclerosing-panencephalitis-sspe-a-case-series-from-india/. Accessed November 21, 2024.« Back to 2023 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/atypical-manifestations-of-subacute-sclerosing-panencephalitis-sspe-a-case-series-from-india/