REPOST: Patients With Hereditary Ataxia Show Improvement With CoQ10

[Guest guest]

Patients With Hereditary Ataxia Show Improvement With CoQ10 (Ubiquinone)

http://www.pslgroup.com/dg/1f79da.htm

The abstract of the Neurology article referred to above is below:

Neurology

2001 Apr 10;56(7):849-55

Familial cerebellar ataxia with muscle coenzyme Q10 deficiency.

Musumeci O, Naini A, Slonim AE, Skavin N, Hadjigeorgiou GL, Krawiecki N,

Weissman BM, Tsao CY, Mendell JR, Shanske S, De Vivo DC, Hirano M, DiMauro

S.

Department of Neurology, Columbia University College of Physicians and

Surgeons, New York, NY, USA.

OBJECTIVE: To describe a clinical syndrome of cerebellar ataxia associated

with muscle coenzyme Q10 (CoQ10) deficiency. BACKGROUND: Muscle CoQ10

deficiency has been reported only in a few patients with a mitochondrial

encephalomyopathy characterized by 1) recurrent myoglobinuria; 2) brain

involvement (seizures, ataxia, mental retardation), and 3) ragged-red fibers

and lipid storage in the muscle biopsy. METHODS: Having found decreased

CoQ10 levels in muscle from a patient with unclassified familial cerebellar

ataxia, the authors measured CoQ10 in muscle biopsies from other patients in

whom cerebellar ataxia could not be attributed to known genetic causes.

RESULTS: The authors found muscle CoQ10 deficiency (26 to 35% of normal) in

six patients with cerebellar ataxia, pyramidal signs, and seizures. All six

patients responded to CoQ10 supplementation; strength increased, ataxia

improved, and seizures became less frequent. CONCLUSIONS: Primary CoQ10

deficiency is a potentially important cause of familial ataxia and should be

considered in the differential diagnosis of this condition because CoQ10

administration seems to improve the clinical picture.

PMID: 11294920

[Guest guest]

Patients With Hereditary Ataxia Show Improvement With CoQ10 (Ubiquinone)

http://www.pslgroup.com/dg/1f79da.htm

The abstract of the Neurology article referred to above is below:

Neurology

2001 Apr 10;56(7):849-55

Familial cerebellar ataxia with muscle coenzyme Q10 deficiency.

Musumeci O, Naini A, Slonim AE, Skavin N, Hadjigeorgiou GL, Krawiecki N,

Weissman BM, Tsao CY, Mendell JR, Shanske S, De Vivo DC, Hirano M, DiMauro

S.

Department of Neurology, Columbia University College of Physicians and

Surgeons, New York, NY, USA.

OBJECTIVE: To describe a clinical syndrome of cerebellar ataxia associated

with muscle coenzyme Q10 (CoQ10) deficiency. BACKGROUND: Muscle CoQ10

deficiency has been reported only in a few patients with a mitochondrial

encephalomyopathy characterized by 1) recurrent myoglobinuria; 2) brain

involvement (seizures, ataxia, mental retardation), and 3) ragged-red fibers

and lipid storage in the muscle biopsy. METHODS: Having found decreased

CoQ10 levels in muscle from a patient with unclassified familial cerebellar

ataxia, the authors measured CoQ10 in muscle biopsies from other patients in

whom cerebellar ataxia could not be attributed to known genetic causes.

RESULTS: The authors found muscle CoQ10 deficiency (26 to 35% of normal) in

six patients with cerebellar ataxia, pyramidal signs, and seizures. All six

patients responded to CoQ10 supplementation; strength increased, ataxia

improved, and seizures became less frequent. CONCLUSIONS: Primary CoQ10

deficiency is a potentially important cause of familial ataxia and should be

considered in the differential diagnosis of this condition because CoQ10

administration seems to improve the clinical picture.

PMID: 11294920