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The Value of Serial Auditory Brainstem Response in Patients With Subacute Sclerosing Panencephalitis

Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry Chiba, inagaki{at}ncnp-k.go.jp

Makiko Kaga, MD

Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry Chiba

Kenji Nihei, MD

Division of Child Neurology, National Children's Hospital Tokyo

Haruko Naitoh, MD

Division of Child Neurology, National Children's Hospital Tokyo

Shuji Takayama, MD

Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry Tokyo, Japan

Kenji Sugai, MD

Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry Tokyo, Japan

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. (J Child Neurol 1999;14:422-427).

Journal of Child Neurology, Vol. 14, No. 7, 422-427 (1999)
DOI: 10.1177/088307389901400703


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