| Aicardi syndrome accompanied by auditory disturbance and multiple brain tumors. | |
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MedLine Citation:
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PMID: 1667249 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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 remarkable hypotonia with psychomotor retardation. Abnormal ophthalmological findings included chorioretinopathy with pale and round-shaped peripapillary lacunae, and there was modified hypsarrhythmia 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. |
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Authors:
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K Hamano; T Matsubara; S Shibata; C Hirano; Z Ito; Y Ase; J Kusakari; H Takita |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Brain & development Volume: 13 ISSN: 0387-7604 ISO Abbreviation: Brain Dev. Publication Date: 1991 Nov |
Date Detail:
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Created Date: 1992-05-26 Completed Date: 1992-05-26 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7909235 Medline TA: Brain Dev Country: JAPAN |
Other Details:
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Languages: eng Pagination: 438-41 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, University of Tsukuba, Ibaraki, Japan. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Acoustic Impedance Tests Adrenocorticotropic Hormone / pharmacology Audiometry Brain Neoplasms / congenital*, pathology Corpus Callosum / abnormalities*, pathology Female Hearing Disorders / congenital*, pathology Humans Infant Magnetic Resonance Imaging Reflex / physiology Syndrome |
| Chemical | |
Reg. No./Substance:
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9002-60-2/Adrenocorticotropic Hormone |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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