The effect of piracetam on ataxia: clinical observations in a group of autosomal dominant cerebellar ataxia patients.

OBJECTIVES: Autosomal dominant cerebellar ataxias are clinically and genetically heterogeneous neurodegenerative disorders. There is no known treatment to prevent neuronal cell death in these disorders. Current treatment is purely symptomatic; ataxia is one of the most disabling symptoms and represents the main therapeutic challenge. A previous case report suggesting benefit from administration of high dose piracetam inspired the present study of the efficacy of this agent in patients with cerebellar ataxia. Piracetam is a low molecular weight derivative of gamma-aminobutyric acid. Although little is known of its mode of action, its efficacy has been documented in a wide range of clinical indications, such as cognitive disorders, dementia, vertigo and dyslexia, as well as cortical myoclonus. The present report investigated the role of high dose piracetam in patients with cerebellar ataxia. METHODS: Eight patients with autosomal dominant cerebellar ataxia were given intravenous piracetam 60 g/day by a structured protocol for 14 days. The baseline and end-of-the study evaluations were based on the International Cooperative Ataxia Rating Scale. RESULTS: Statistical analysis demonstrated a significant improvement in the patients' total score (P = 0.018) and a subscale analysis showed statistical significance for only the posture and gait disturbances item (P = 0.018). CONCLUSION: This study is providing good clinical observation in favour of high dose piracetam infusion to reduce the disability of the patients by improving their gait ataxia.

Myasthenia gravis with thymoma and autoimmune haemolytic anaemia. A case report.

Myasthenia gravis is an autoimmune disorder that affects the neuromuscular junction and leads to weakness of the skeletal muscles. Associated autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis and pernicious anaemia are present in approximately 5% of the myasthenic patients. This report presents a 64-year-old man with autoimmune haemolytic anemia associated with myasthenia gravis and thymoma. The patient developed a severe Coomb's positive autoimmune haemolytic anaemia, which was resistant to treatment with large doses of prednisone. Haemolytic anaemia entered remission one month following thymectomy, and the patient has maintained a normal haemoglobin and a negative Coomb's test without the need for steroid or immunosuppressive therapy. In conclusion, thymectomy may induce a striking improvement of therapyresistant autoimmune haemolytic anemia in patients with MG and thymoma, but in terms of remission, a long follow-up is needed as autoimmune diseases can show spontaneous fluctuations.