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[Adult-onset subacute sclerosing panencephalitis: clinicopathological findings]
J González de la Aleja1, I J Posada, J M Sepúlveda-Sánchez
1Servicio de Neurología, Hospital Universitario 12 de Octubre, 28045 Madrid, España. jesus_goal@yahoo.es
Introduction:
Subacute sclerosing panencephalitis is a disease affecting the central nervous system that is produced by persistent infection by a defective measles virus. This disease is very infrequent and its incidence has gone down even further in western countries since the introduction of generalised measles vaccinations. Onset of the disease is usually during infancy or adolescence. Reports of cases beginning during adulthood are scarce.
Case Report:
We describe the case of a 30-year-old female with a slowly progressive subacute clinical picture consisting in behavioural disorders, with defrontalisation, cortico-subcortical cognitive impairment, long tract signs and visual disorders, which led the patient into a vegetative state. Four years after the onset of symptoms the patient died. The different electroencephalogram recordings performed did not show any periodic activity and magnetic resonance imaging of the head revealed cerebral atrophy with hyperintense lesions in T2 sequences in white matter. The histological study of the brain showed a chronic inflammatory infiltration with neuronal loss and demyelination, as well as intranuclear inclusions and neurofibrillary degeneration.
Conclusions:
The appearance of subacute sclerosing panencephalitis in adulthood is exceptional. Diagnosis requires a high degree of clinical suspicion, above all in the absence of typical symptoms, such as myoclonias or periodic complexes in EEG recordings.
Insights
Subacute sclerosing panencephalitis (SSPE) is rare in adults. This case highlights the need for high clinical suspicion in diagnosing SSPE, especially when typical symptoms are absent.
Area of Science:
- Neurology
- Virology
- Pathology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disease caused by persistent measles virus infection.
- Widespread measles vaccination has significantly reduced SSPE incidence, making adult-onset cases exceptionally infrequent.
- SSPE typically manifests in infancy or adolescence, with adult onset being a rare clinical presentation.
Observation:
- A 30-year-old female presented with a 4-year subacute decline, including behavioral changes, cognitive impairment, neurological deficits, and visual disturbances, progressing to a vegetative state.
- Electroencephalogram (EEG) recordings lacked characteristic periodic activity, and brain MRI revealed cerebral atrophy and white matter lesions.
- Histopathological examination confirmed chronic inflammation, neuronal loss, demyelination, and characteristic viral inclusions.
Findings:
- The case underscores the atypical presentation of SSPE in adulthood, deviating from classic EEG and clinical signs.
- Diagnostic challenges arise in adult SSPE due to the absence of myoclonias or periodic EEG complexes.
- Neuropathological findings were crucial for confirming the diagnosis in this rare adult-onset case.
Implications:
- Adult-onset SSPE requires heightened clinical suspicion, particularly when neurological symptoms are progressive and unexplained.
- The study emphasizes the importance of considering SSPE in the differential diagnosis of adult-onset cognitive and neurological decline, even without typical EEG findings.
- This case contributes to understanding the varied clinical spectrum of SSPE and reinforces the diagnostic value of neuropathology.
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