Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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March 21, 2016
Subacute Sclerosing Panencephalitis
- Background:
- Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disease caused by persistent infection of the measles virus. The most important risk factor for SSPE is contracting measles in the first 2 years of life.
- It affects children and young adults.
- Clinical Presentation:
- Initially, the patient presents with mild cognitive deterioration characterized by poor school performance and abnormal behavior associated with myoclonic jerks.
- It is followed by memory loss, seizures, neurologic deterioration, and coma.
- Often, there is a past medical history of measles several years before the onset of the disease (7–14 years prior).
- Key Diagnostic Features:
- EEG showing periodic activity (Rademecker complex)
- Elevated anti-measles antibody in the CSF and high IgG levels in CSF
- MRI demonstrates nonenhancing white matter signal abnormalities that are usually bilaterally symmetric, progressing to diffuse atrophy.
- DTI detects early white matter abnormalities that may have significant therapeutic implication, even in the presence of normal findings on conventional imaging, in patients with SSPE.
- Differential Diagnosis:
- Metachromatic leukodystrophy
- Krabbe disease
- Mitochondrial encephalopathies
- X-linked adrenoleukodystrophy
- Treatment:
- There is currently no definitive treatment.
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