ABSTRACT
Through the observation of seven cases we reported the epidemiological, clinical, paraclinical, evolutionary and therapeutical aspects of this disease. Epidemiologically, the sex ratio is of 0,75. The medium age is of six years and two months. Factors in favor of a hereditary origin are the consanguinity found in 28,6% of cases and the presence of a similar case in the family observed in 42,3%. Clinically, the quasi constantly neurolgical signs are ataxia [100%], dysarthria [71, 4%], areflexia of endon reflex in both lowner limbs [57,14], deep sensation troubles are less frequent [28,6%]. Vestibular affection with form of horizontal nystagmus is found in 14,3% of cases. The dysmorphic syndrome is in the form of claw feet found in [57,14%] and of dorsal scoliosis in [14,3%]. Cardiac affection in the form of cardiopathy is noted in two magnetic resonance imagery in one patient. The electromyography is disturbed in 28.6% of cases. The genetic study done to two cases [28,6%]. Paraclinically, we found cerebellar atrophy in 57,14% which was confirmed by cases showed an expansion of the triplet GAA at the level of the chromosome nine. Improvement under symptomatic treatment based on muscular and functional physiotherapy was progressively slow for all our patients