[Dermo-neuro-hypsarrhythmia. Association of a congenital dermatosis and hypsarthymia. 9 cases (author's transl)].
These 9 cases confirm that tuberose sclerosis, von Recklinghausen's neurofibromatosis, incontinentia pigmenti, linear warty sebaceous naevus and alopecic naevus resulting in a woolly naevus of the scalp may be complicated by flexion spasms with hypsarhythmia. Early diagnosis of hypsarhythmia makes possible specific treatment with ACTH or hydrocortisone.
D000015:Abnormalities, Multiple; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007223:Infant; D008297:Male; D009207:Myoclonus / Q000188:drug therapy / Q000209:etiology; D009456:Neurofibromatosis 1 / Q000150:complications*; D009506:Nevus / Q000150:complications*; D009508:Nevus, Pigmented / Q000150:complications; D010859:Pigmentation Disorders / Q000150:complications; D012535:Scalp; D012878:Skin Neoplasms / Q000150:complications*; D013036:Spasms, Infantile / Q000150:complications* / Q000188:drug therapy; D014402:Tuberous Sclerosis / Q000150:complications*
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone
Dermo-neuro-hypsarythmie. Association d'une dermatose congénitale et d'une hypsarythmie, A propos de 9 cas