[Clinical and genetic analysis of 188 families with spinocerebellar degeneration. Friedreich's disease and P. Marie's hereditary ataxias]. / Analyse clinique et génétique de 188 familles d'hérédo-dégénérescence spino-cérébelleuse. Maladies de Friedreich et hérédo-ataxies de P. Marie.

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.

[Epidemiologic aspects of cerebrovascular accidents in Tunisia]. / Aspects épidémiologiques des accidents vasculaires cérébraux en Tunisie.

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).

Prevalence of epilepsy in Kelibia, Tunisia.

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.

Prevalence study of neurologic disorders in Kelibia (Tunisia).

A full-scale survey, in Kelibia, Tunisia, screening 34,874 persons started on July 1, 1985. The accuracy of this survey was evaluated by a second survey using a randomized sample of 1,673 subjects (control survey). Better selection and training of the interviewers during the control survey led to a higher positive predictive value with no modification in prevalence ratios of neurologic disorders. The control survey helped to validate the full-scale survey data which were then used to establish the prevalence ratios of major neurologic disorders in Kelibia. Prevalence ratios, age-adjusted to the WHO population, were compared to those of studies using similar methodology. Migraine prevalence ratios in Nigeria, Ecuador, and Kelibia were equivalent. Epilepsy and Parkinson's disease prevalence ratios were close to those of other similar studies. The stroke prevalence ratio was low, compared to other studies, but was not the lowest. It seems that in Kelibia, stroke does not constitute a public health problem as it does in the USA or urban China. The large full-scale survey, in Kelibia, provided estimates of prevalence ratios for stroke, epilepsy, migraine and other common neurologic disorders for comparisons with other countries. However, definitions of neurologic disorders and diagnostic criteria differ from one study to another making difficult the comparison of results between different countries. Had the WHO protocol developed well-defined criteria and a standardized neurologic examining tool, more accurate comparisons could have been made.