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At a glance

It is a syndrome consisting of alopecia or hypotrichosis, oligophrenia, microcephaly, seizures, and mental retardation.

Synonyms

Alopecia-Epilepsy-Oligophrenia Syndrome; Alopecia-Mental Retardation-Epilepsy-Microcephaly Syndrome; Epilepsy-Oligophrenia Syndrome of Moynahan.

History

This medical condition was first described in 1962 by E. J. Moynahan, a British dermatologist practicing in London, England.

Classification

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

Incidence

The incidence remains unknown.

Genetic inheritance

Familial with parental consanguinity; autosomal recessive trait.

Pathophysiology

Unknown.

Diagnosis

Microcephaly, alopecia or hypertrichosis, and epilepsy (grand mal seizures).

Clinical aspects

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.

Anesthetic considerations

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.

Pharmacological implications

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.

References

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Baraitser  M, Carter  CO, Brett  EM: A new alopecia/mental retardation syndrome. J Med Genet 20:64, 1983.  [PubMed: 6842537]
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Moynahan  EJ: Familial congenital alopecia, epilepsy, mental retardation with unusual electroencephalogram. Proc R Soc Med 55:411, 1962.  [PubMed: 14476762]
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Perniola  T, Krajewska  G, Carnevale  F,  et al: Congenital alopecia, psychomotor retardation, convulsions in two sibs of a consanguineous marriage. J Inherit Metab Dis 3:49, 1980.  [PubMed: 6777601]

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