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Leukoencephalopathy with brain stem and spinal cord involvement-high lactate syndrome

ORPHA:137898· ICD-10 E88.8

Definition

This disease is characterised by progressive cerebellar ataxia with pyramidal and spinal cord dysfunction, associated with distinctive MRI anomalies and increased lactate in the abnormal white matter.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Adolescent, Adult, Antenatal, Childhood, Infancy, Neonatal