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A very rare inherited disorder characterized by association of alopecia and seizures.

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Alopecia Epilepsy Pyorrhea Mental Subnormality Syndrome.

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Autosomal dominant.

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Association of alopecia, mental subnormality, and psychomotor epilepsy.

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Alopecia is congenital, permanent, and universal. Periodontal disease is present in all patients. Pigmented nevi can be observed.

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Evaluate epilepsy treatment efficacy (EEG, history). Direct laryngoscopy should be performed carefully to prevent periodontal bleeding. Epilepsy medications should be given the day of surgery. Consider anesthetic drug interaction with epileptic treatment.

Shokeir MHK: Universal permanent alopecia, psychomotor epilepsy, pyorrhea and mental subnormality. Clin Genet 11:13, 1977.  [PubMed: 830443]

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