Lipids and Lipoproteins Abnormalities in Fridreich s Ataxia

Objectives: Study serum lipoprotein levels in patients with Friedreich s ataxia and its relation with the development of cardiac abnormalities.Patients and Methods: The study included 18 patients with clinical manifestations of Friedreich s ataxia and 19 healthy subjects for control. Serum total cholesterol, HDL cholesterol, LDL cholesterol, triglycerides, apolipoprotein A, apolipoprotein B and phospholipids were determined for patients and control group. ECG was done for every patient. Most of the patients were males (83%) while 17% were females.Results: The study showed that the total serum cholesterol, HDL and LDL cholesterol were significantly raised in the group B (sporadic cases) while they remained within the control level in the group A (familial cases). Serum triglycerides were significantly raised in both groups of the patients. Serum Apoprotein A and B were significantly decreased in a (familial) while in group B (sporadic), Apoprotein A was significantly decreased and Apoprotein B was not significantly altered. Serum phospholipids did not show significant changes in any of the two groups. ECG examination showed evidence of myocardial ischemia in the form of low voltage QRS, depression of ST segment, flattening or inversion of T wave in pericardial and limb leads. The changes were found in 80% of the patients of the familial group (A) and in 37% of the patients of the sporadic group (B).Conclusion: The total serum cholesterol, HDL and LDL cholesterol were significantly raised in the group B (sporadic cases) while they remained within the control level in the group A (familial cases). Serum triglycerides were significantly raised in both groups of the patients. Serum Apoprotein A and B were significantly decreased in a (familial) while in group B (sporadic), Apoprotein A was significantly decreased and Apoprotein B was not significantly altered. Serum phospholipids did not show significant changes in any of the two groups. ECG examination showed evidence of myocardial ischemia in the form of low voltage QRS, depression of ST segment, flattening or inversion of T wave in pericardial and limb leads. The changes were found in 80% of the patients of the familial group (A) and in 37% of the patients of the sporadic group.