A Long-term Clinical and Electroencephalographic Study of a Case of Aicardi Syndrome with the Transition from Infantile Spasma to Lennox Syndrome

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  • 点頭てんかんからＬｅｎｎｏｘ症候群への変容を伴ったＡｉｃａｒｄｉ症候群の１例　　その臨床的，脳波学的経時的変化を中心として
  • その臨床的, 脳波学的経時的変化を中心として

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Abstract

This paper reported the result of a long-term clinical and electroencephalographic study of a case of Aicardi syndrome involving a 6-year- old girl. She started having flexor spasms at 5 months of age; the case was diagnosed as infantile spasms, and she received anticonvulsants. At 9 months of age, flexor spasms recurred, and she was treated with ACTH. The seizures was cotrolled for almost 4 years thereafter, but at 4 years and 10 months of age tonic seizures and astatic seizures, chinical manifestations of Lennox syndrome, were noted and these seizures are still intractable.<BR>All patients so far reported with Aicardi syndrome have had severe psychomotor retardation. On the other hand our case did not have psychomotor retardation until 2 years of age; she was able to walk alone at 1 year 1 month of age and speak a few words at 1 year 4 months of age.<BR>Nineteen EEG records taken during the 6 years were studied. At the onset, the records showed hypsarhythmia, but not the, suppression-burst pattern. At 8 months of age, after about 3 months on the anticonvulsants, hypsarhythmia diminished, and sporadic multifocal spikes appeared. ACTH therapy improved the EEG findings for a time. However, multifocal spikes reappeared soon and gradually increased in frequency. At 6 years 7 months of age, the EEGs showed slow spike & wave complexes and rapid rhythms which sometimes revealed complete asynchrony between right and left hemispheres. We think that this findings is a characteristic electroencephalographic pattern of Aicardi syndrome with Lennox syndrome.

Journal

  • NO TO HATATSU

    NO TO HATATSU 14 (6), 579-585, 1982

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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