X-linked spinocerebellar ataxia type 4 (MONDO_0010534) can be described as follows. 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. Also known as: SCAX4, X-linked ataxia-dementia syndrome, spinocerebellar ataxia, X-linked type 4.