ICD-11 classes
08 Diseases of the nervous system
Movement disorders
8A03 — Ataxic disorders
8A03.1 — Hereditary ataxia
8A03.13 — Ataxia due to abetalipoproteinemia

ICD-11 8A03.13 — Ataxia due to abetalipoproteinemia

Ataxia in the setting of abetalipoproteinemia, a rare autosomal recessive disorder caused by a mutation of the MTP gene coding for microsomal triglyceride transfer protein which impairs the ability to produce very low density lipoprotein. All patients have fat malabsorption, acanthocytosis, hypocholesterolemia, and absent apolipoprotein B.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.