X-linked spinocerebellar ataxia type 4 https://rarediseases.info.nih.gov/diseases/9980/spinocerebellar-ataxia-x-linked-type-4 https://www.malacards.org/card/spinocerebellar_ataxia_x_linked_4 icd11.foundation:1033689736 SCTID:719818007 MEDGEN:337122 spinocerebellar ataxia X-linked type 4 MONDO:0010534 Spinocerebellar ataxia, X-linked, type 4 is characterized by ataxia, pyramidal tract signs and adult-onset dementia. It has been described in three generations of one large family. The disease manifests during early childhood with delayed walking and tremor. The pyramidal signs appear progressively and by adulthood memory problems and dementia gradually become apparent. Transmission is X-linked but the causative gene has not yet been identified. The disease is usually fatal during the sixth decade of life. OMIM:301840 ataxia-dementia syndrome, X-linked X-linked ataxia-dementia syndrome Orphanet:85292 spinocerebellar ataxia, X-linked type 4 ataxia-dementia syndrome X-linked MESH:C537316 Scax4 GARD:0009980 DOID:0111832 UMLS:C1844933 spinocerebellar ataxia, X-linked 4 SCAX4 X-linked cerebellar ataxia X-linked syndromic intellectual disability