Gangliosidosis

Definition

Gangliosidosis is a lysosomal storage disease characterized by disorders of ganglioside metabolism and progressive neurological disorders.

Symptoms

  • Neurological decline
  • Macrocephaly
  • Cherry red macula
  • Hypotonia

Reasons

HEXA, GLB1 gene mutations.

Diagnosis

Enzyme activity, genetic testing.

Treatment

Symptomatic treatment.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

In case of status epilepticus, emergency services should be consulted.

This page is for general information purposes only.