It is a syndrome consisting of alopecia or hypotrichosis, oligophrenia, microcephaly, seizures, and mental retardation.
Alopecia-Epilepsy-Oligophrenia Syndrome; Alopecia-Mental Retardation-Epilepsy-Microcephaly Syndrome; Epilepsy-Oligophrenia Syndrome of Moynahan.
This medical condition was first described in 1962 by E. J. Moynahan, a British dermatologist practicing in London, England.
Three types of this disorder are suggested:
Type I: Total alopecia and mental retardation, the main features, in association with microcephaly
Type II: Subtotal alopecia and mental retardation with or without epilepsy
Type III: Subtotal alopecia and psychomotor retardation with microcephaly and epilepsy
The incidence remains unknown.
Familial with parental consanguinity; autosomal recessive trait.
Microcephaly, alopecia or hypertrichosis, and epilepsy (grand mal seizures).
Sparse or absent scalp hair, microcephaly, seizures, short stature; hypogonadism with late puberty. Poor musculature. Sometimes sensorineural deafness.
Precautions before anesthesia
No literature on anesthesia in this condition. However, full neurologic history and examination, epileptic history, especially of control and medications, and associated anomalies resulting from microcephaly must be reviewed.
Epileptic control is essential; usual medications should be maintained until the morning of surgery wherever possible and appropriate nontriggering anesthetic agents used. Psychomotor retardation with poor speech skills may lead to an uncooperative patient.
Note potential for interaction with antiepileptic drugs. Chronic phenytoin administration increases nondepolarizing neuromuscular blocker requirements, produces gingival hyperplasia and bleeding, and may cause hepatic dysfunction. Ketamine, enflurane, and methohexital are relatively contraindicated. High concentration (>5-6%) of sevoflurane at induction might trigger epilepsy.
EM: A new alopecia/mental retardation syndrome. J Med Genet
EJ: Familial congenital alopecia, epilepsy, mental retardation with unusual electroencephalogram. Proc R Soc Med
et al: Congenital alopecia, psychomotor retardation, convulsions in two sibs of a consanguineous marriage. J Inherit Metab Dis