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Adult onset of subacute sclerosing panencephalitis: a case report
1Istituto di Neurologia, Università Cattolica, Roma.
Abstract:
A new case of subacute sclerosing panencephalitis with onset in adult life is reported. Clinical picture was characterized by a maculopathy, followed two years after by behavioural disturbances, psychomotor impairment, pyramidal signs and left-side myelonic jerks synchronously with the typical periodic R-complex in the EEG. CT-scan and MRI showed a wide demyelinative lesion in the right temporo-occipital area of the brain. Elevated antibody titers to measles virus in serum and CSF were present. Death occurred within 6 months while in coma. The neuropathologic findings confirmed the diagnosis of SSPE revealing widespread inflammatory lesions in the grey and white matter areas of demyelinization more evident in the right temporo-occipital regions and several Cowdry type A inclusions in glial cells and neurons.
Insights
This report details a rare adult-onset subacute sclerosing panencephalitis (SSPE) case. Neuropathology confirmed widespread inflammation and demyelination, linked to elevated measles virus antibodies.
Area of Science:
- Neurology
- Neuroimmunology
- Pathology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
- Typically affects children, with adult-onset cases being exceptionally uncommon.
- Caused by persistent measles virus infection leading to chronic brain inflammation.
Observation:
- A unique case of adult-onset SSPE presented with maculopathy.
- Clinical progression included behavioral changes, psychomotor decline, pyramidal signs, and myoclonic jerks.
- Electroencephalogram (EEG) showed periodic R-complex, while CT and MRI revealed demyelination in the right temporo-occipital lobe.
Findings:
- Elevated serum and cerebrospinal fluid (CSF) antibody titers to measles virus were detected.
- Neuropathologic examination confirmed SSPE with widespread grey and white matter lesions and Cowdry type A inclusions.
- Demyelination was pronounced in the right temporo-occipital regions.
Implications:
- Highlights the possibility of adult-onset SSPE, expanding the known clinical spectrum.
- Emphasizes the importance of considering SSPE in adults with unexplained progressive neurological deficits.
- Reinforces the link between measles virus and SSPE pathogenesis, even in late-onset presentations.