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Published on: July 4, 2007
Adult-Onset Subacute Sclerosing Panencephalitis Presenting With Subacute Cognitive Deficits
Dennis Yeow1,2,3, Nicola McKern3,4, Vincent Oxenham3,4
1Neurodegenerative Service, Prince of Wales Hospital and Neuroscience Research Australia, Sydney, New South Wales, Australia.
Abstract:
We describe the case of a 41-year-old man diagnosed with adult-onset subacute sclerosing panencephalitis (SSPE). The patient presented with subacute progressive cognitive deficits and a neuropsychological profile indicating predominant frontoparietal dysfunction. MRI showed only mild parietal-predominant cerebral atrophy. The patient later developed periodic myoclonic jerks with time-locked periodic slow wave complexes on electroencephalography. Evidence of intrathecal synthesis of anti-measles IgG was identified in the cerebrospinal fluid. The patient satisfied modified Dyken diagnostic criteria and was diagnosed with SSPE. A frontoparietal pattern of deficits on neuropsychological assessment may be an early clue to the diagnosis of adult-onset SSPE.
Insights
Subacute sclerosing panencephalitis (SSPE) in adults can present with cognitive decline and frontoparietal dysfunction. Early neuropsychological assessment may aid in diagnosing this rare measles complication.
Area of Science:
- Neurology
- Neuroimmunology
- Viral Encephalitis
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
- Typically associated with measles infection in childhood, adult-onset SSPE is exceptionally uncommon.
- Early diagnosis is challenging due to nonspecific initial symptoms.
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