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The value of serial auditory brainstem response in patients with subacute sclerosing panencephalitis
1Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Ichikawa, Chiba, Japan. ingaki@ncnp-k.go.jp
Abstract:
A total of 98 serial auditory brainstem responses from 17 patients (11 boys and 6 girls) with subacute sclerosing panencephalitis were compared with their clinical course and stages. These patients were exposed to measles early in life (at 1.8 +/- 1.4 years old) with the average onset of the disease at 8.8 +/- 0.7 years of age. The main abnormalities of their auditory brainstem responses were a prolongation of waves I, III, and V, and of the I to V interpeak interval. Wave V thresholds were also elevated. These effects on the auditory brainstem responses started 1 to 2 years after onset of neurologic signs. I to V interpeak latency became prolonged with the progress of clinical stages, especially in Jabbour's clinically advanced stage IV. In two patients with an acute progressive type of subacute sclerosing panencephalitis, very rapid deterioration with a distorted wave pattern of later components was observed. These findings suggest rostrocaudal progression in the central nervous system of both long-term and fulminant cases.
Insights
Subacute sclerosing panencephalitis (SSPE) affects auditory brainstem responses, showing prolonged latencies and elevated thresholds. These changes correlate with disease progression, suggesting central nervous system involvement.
Area of Science:
- Neurology
- Audiology
- Pediatrics
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
- Measles infection in early childhood is a known precursor to SSPE.
- Auditory brainstem responses (ABRs) are crucial for assessing auditory pathway function.
Observation:
- Serial ABRs were analyzed in 17 patients with SSPE.
- Patients experienced measles early and developed SSPE symptoms later.
- ABR abnormalities included prolonged wave latencies (I, III, V) and interpeak intervals (I-V).
Findings:
- Elevated wave V thresholds were observed in SSPE patients.
- ABR changes appeared 1-2 years post-neurological onset.
- Interpeak latency (I-V) worsened with advanced clinical stages, particularly Stage IV.
- Rapid deterioration and distorted ABR patterns were noted in acute SSPE cases.
Implications:
- ABR findings suggest a rostrocaudal progression of SSPE in the central nervous system.
- ABRs can serve as a biomarker for SSPE progression and severity.
- Understanding ABR changes aids in monitoring disease course and potential therapeutic interventions.