leukodystrophy with oligodontiatremor-ataxia-central hypomyelination syndromehypomyelination-cerebellar atrophy-hypoplasia of the corpus callosum syndromeTACH syndromedentoleukoencephalopathyHLD7leukoencephalopathy-ataxia-hypodontia-hypomyelination syndromeataxia-delayed dentition-hypomyelination syndrome; odontoleukodystrophy
obo:hasOBONamespace
disease_ontology
n2:IAO_0000115
A hypomyelinating leukodystrophy characterized by autosomal recessive inheritance of hildhood onset of progressive motor decline manifest as spasticity, ataxia, tremor, and cerebellar signs, as well as mild cognitive regression that has_material_basis_in homozygous or compound heterozygous mutation in the POLR3A gene on chromosome 10q22.