Early onset ataxias in Tunisia. Intrafamilial heterogeneity.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to N Attia-Romdhane.
Explore the source record for details and available documents.
Based on the hereditary ataxias concepts and a large field survey, the authors analyzed 392 cases of spino-cerebellar degeneration belonging to 188 families. Two main clinical groups were identified: 227 cases of Friedreich ataxia and 74 cases of cerebellar hereditary ataxia of P. Marie type. The association in the same patient of peroneal atrophy of Charcot Marie type with Friedreich ataxia (17 cases) or P. Marie cerebellar hereditary ataxia (13 definite cases and 13 probable) was the most striking finding. "Forme fruste", incomplete form or complex form of Friedreich ataxia were present in some families while in some others there was spastic paraplegia or pure Charcot Marie Tooth disease. This clinical heterogeneity in families of spino-cerebellar degeneration is discussed.
We report the results of 3 epidemiological studies of stroke in Tunisia. In Tunis urban population, the crude annual incidence rate of stroke has been estimated at 0.54/1,000 and the prevalence rate at 6 to 14/1,000. The incidence rate adjusted to population at risk (greater than or equal to 45 years old), is around 1.92/1,000. A door-to-door survey conducted in Kelibia, according to a WHO protocol, showed a prevalence rate of 7.2/1,000 when adjusted to population at risk. CT diagnosed infarction and haemorrhage and excluded non-stroke cases (10 p. 100). Cerebral haemorrhage was more frequent than in other published studies (28 p. 100).
A door-to-door survey was made in Kelibia, Tunisia to determine the prevalence of major neurologic disorders, including epilepsy. The survey was made according to a World Health Organization (WHO) protocol (1981). All individuals responding positively to the screening tool were examined by a neurologic team using well-defined diagnostic criteria. One hundred forty-one individuals, alive on prevalence day (July 1, 1985), were identified as having active epilepsy, giving a crude prevalence ratio of 4.04 per 1,000 and an age-adjusted (on WHO population) prevalence ratio of 3.64 per 1,000. Prevalence ratios increase with age (in children and young adults with the highest prevalence ratio at approximately 20 years) and decrease after 40 years. The most frequently identified type was generalized convulsive seizures (93%). The most frequently associated conditions were cerebral palsy and mental retardation.
Explore the source record for details and available documents.