CoA synthase protein associated neurodegeneration

CoA synthase protein associated neurodegeneration (CoPAN) is a type of neurodegeneration with brain iron accumulation (NBIA).

Patients typically present with a variety of symptoms including spastic-dystonic paraparesis, Parkinsonism, cognitive impairment, obsessive-compulsive disorder.

Coenzyme A synthesis (CoASY) is a cofactor involved in enzymatic reactions. CoASY mutation leads to brain iron accumulation. 

MRI and CT

"Eye of the tiger sign" on T2 MRI (like PKAN) associated with bilateral basal ganglia calcifications.

 

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Article Information

rID: 50279
Section: Pathology
Synonyms or Alternate Spellings:

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