Serial MR imaging, diffusion tensor imaging, and MR spectroscopic findings in a child with progressive encephalopathy, edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome.
نویسندگان
چکیده
PEHO syndrome is a rare symptom complex of severe progressive encephalopathy, edema, hypsarrhythmia, and optic atrophy. Disease onset is in early infancy. The current case report presents and discusses serial conventional MR imaging findings and serial functional studies including diffusion tensor imaging and quantitative MR spectroscopy findings in a 6-year-old child with PEHO.
منابع مشابه
PEHO syndrome (progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy): neuroradiologic findings.
PURPOSE To investigate the radiologic characteristics of the clinical progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO) symptom complex. This complex is nonspecific, but within this syndrome, a subgroup with a defined neuropathologic phenotype and apparently autosomal recessive inheritance exists. METHODS Brain CT or MR studies were performed on 21 patients with ...
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Progressive encephalopathy with oedema, hypsarrhythmia and optic atrophy (PEHO) syndrome is a rare Mendelian phenotype comprising severe retardation, early onset epileptic seizures, optic nerve/cerebellar atrophy, pedal oedema, and early death. Atypical cases are often known as PEHO-like, and there is an overlap with 'early infantile epileptic encephalopathy'. PEHO is considered to be recessive...
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The PEHO syndrome (progressive encephalopathy with brain, facial and peripheral edema, hypsarrhythmia and optic atrophy) is a very rare autosomal recessive disorder with progressive encephalopathy. For diagnosis and therapy, many patients may require deep sedation or even general anesthesia. There are no previous reports on the perioperative management in PEHO-patients. Distinct obstacles have ...
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عنوان ژورنال:
- AJNR. American journal of neuroradiology
دوره 27 7 شماره
صفحات -
تاریخ انتشار 2006