Alopecia-Epilepsy-Oligophrenia Syndrome Of Moynahan

In the family reported by Moynahan (1962), 2 brothers were affected. The alopecia consisted of a delay in the growth of hair. The father of the boys had been bald until age 2 and a maternal aunt until age 4. Mosavy (1975) observed 4 affected sibs and Pfeiffer and Volklein (1982) reported affected brother and sister. Wessel et al. (1987) reported 3 sibs with alopecia, seizures, and mental retardation. In a family derived from Bangladesh, van Haeringen et al. (1990) described a father and 3 children (of 7) with microcephaly, sparse hair, mild to moderate mental retardation, and, in the 2 affected boys, generalized seizures. The affected father was related to his wife, so this may be an instance of pseudodominance of a recessive disorder. It is difficult to distinguish this disorder from that described in entry 203650.