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. The disease is odontoleukodystrophy (Monarch Disease Ontology identifier MONDO_0019177). Also known as: dentoleukoencephalopathy, leukodystrophy with oligodontia.