AICARDI SYNDROME ACCOMPANIED BY AUDITORY DISTURBANCE AND MULTIPLE BRAIN-TUMORS
AICARDI SYNDROME ACCOMPANIED BY AUDITORY DISTURBANCE AND MULTIPLE BRAIN-TUMORS
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DOI：
10.1016/s0387-7604(12)80044-1
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发表时间：
1991-11-01
影响因子：
1.7
通讯作者：
TAKITA, H
中科院分区：
文献类型：
--
作者：
HAMANO, K;MATSUBARA, T;TAKITA, H
We described a 5-month-old girl with Aicardi syndrome accompanied by auditory disturbance and multiple brain tumors. She was admitted to our hospital because she suffered from intractable flexor spasms. Physical examination revealed craniofacial asymmetry, left auricular deformity, scoliosis, and and remarkable hypotonia with psychomotor retardation. Abnormal ophthalmological findings included chorioretinopathy with pale and round-shaped peripapillary lacunae, and there was modified hypsarrythmia in her EEG. MRI revealed multiple brain tumors in the 3rd and the lateral ventricles which are considered to be choroid plexus papilloma with agenesis of the corpus callosum. ACTH therapy was administered because of the intractable seizures. After ACTH therapy, the thresholds of waves I and V were much improved. The interpeak latency of waves I-V of the left ear and the peak latency of wave I of the right ear had been lengthened. Acoustic reflex with contralateral stimulation showed no response in the left ear. These findings indicate that the auditory system is also involved in the Aicardi syndrome and that ACTH is effective for its dysfunction.
