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.
Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare autosomal recessive disorder characterized by the congenital absence of horizontal gaze, progressive scoliosis, and failure of the corticospinal and somatosensory axon tracts to decussate in the medulla. HGPPS is caused by mutations of the ROBO3 gene, which encodes a protein that shares homology with the roundabout family of transmembrane receptors that are important in axon guidance and neuronal migration. To date, over 15 mutations have been found in consanguineous families of Greek, Italian, Turkish, Pakistani, Saudi Arabian, and Indian descent. To detail clinical, cerebral magnetic resonance imaging (MRI) and genetic findings of ten HGPPS patients from four unrelated Tunisian families. Four unrelated consanguineous Tunisian families with a total of ten patients suffering from horizontal gaze palsy with progressive scoliosis. Genetic linkage analysis and direct sequencing of the ROBO3 gene. All patients shared similar clinical gaze movement abnormalities and variable degrees of scoliosis. Four distinct homozygous mutations were identified. This study extends the molecular spectrum of the ROBO3 gene and the geographic origin of patients with ROBO3 gene mutations, and underlines the homogeneity of the motor ocular syndrome whatever type of mutation is encountered.
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