Odontoleukodystrophy
Odontoleukodystrophy
Definition
A leukodystrophy characterized by progressive ataxia beginning during infancy, a pyramidal syndrome and dental agenesis. The syndrome has been described in four children born to consanguineous parents. The mode of transmission is autosomal recessive.
Also known as dentoleukoencephalopathy, leukodystrophy with oligodontia — per MONDO