Subacute sclerosing panencephalitis (SSPE) is a late complication of childhood measles. It is characterized by a progressive decline in cognitive and motor functions, seizures, and eventually death. Although a combination of intrathecal interferon alpha (IFN-α) and daily oral isoprinosine has been reported to have a good outcome, there is no cure for this condition. We present a case of a 16-year-old male with SSPE who presented with progressive weakness, frequent loss of postural control, multiple episodes of generalized tonic-clonic seizures, and urinary incontinence. On exploration of his history, he had measles at the age of two months. Investigation showed increased serum measles antibody titer, high amplitude spikes in electroencephalogram (EEG), and high fluid-attenuated inversion recovery (FLAIR) signals on MRI of the brain consistent with probable SSPE. He was managed symptomatically until his condition got worse and he eventually passed away.
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The measles virus matrix F50S mutation from a lethal case of subacute sclerosing panencephalitis promotes receptor-independent neuronal spread.
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Mayo Clinic Graduate School of Biomedical Sciences, Mayo Clinic, Rochester, Minnesota, USA.
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Ann Indian Acad Neurol
November 2024
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Article Synopsis
- SSPE is a rare, progressive disorder caused by a lingering mutant measles virus in the brain, with unclear treatment strategies among neurologists.
- A survey of 298 Indian neurologists revealed insights into their management practices, showing varied preferences for therapies such as Isoprinosine and valproate.
- There is a significant gap in follow-up care for SSPE patients and an urgent need for clearer treatment guidelines to improve patient outcomes.
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Tremor Other Hyperkinet Mov (N Y)
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Department of Neurology, King George's Medical University, Lucknow, India.
Background: Subacute Sclerosing Panencephalitis (SSPE) is a fatal disorder marked by gradual cognitive and motor deterioration, leading to death typically within 1-3 years.
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Subacute sclerosing panencephalitis (SSPE) is a devastating fatal condition caused by mutated measles virus. It predominantly affects children of younger age and invariably leads to mortality. Though reported rarely, the disease continues to cause significant morbidity in children.
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Department of Molecular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
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