Abstract
Huntington's disease (HD) occurs worldwide with prevalence varying from 0.1 to 10/100,000 depending of the ethnic origin. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Clinical and genetics data of 21 consecutive patients recruited from 2009 to 2014 from the outpatient clinic of six medical centers were analyzed. Statistical analysis was performed using descriptive statistics. Twenty one patients from 17 families were diagnosed positive for the IT15 gene CAG expansion. Clinical symptoms were predominantly motor (19/21). Twelve patients had psychiatric and behavioral disorders, and 11 patients had cognitive disorders essentially of memory impairment. Analysis of genetic results showed that 5 patients had reduced penetrant (RP) alleles and 16 had fully penetrant (FP) alleles. The mean CAG repeat length in patients with RP alleles was 38.4 ± 0.54, and 45.37 ± 8.30 in FP alleles. The age of onset and the size of the CAG repeat length showed significant inverse correlation (p <0.001, r = -0.754). Clinical and genetic data of Moroccan patients are similar to those of Caucasian populations previously reported in the literature.
Highlights
Huntington’s disease (HD) is a rare neurodegenerative genetic disease, characterized by generalized chorea, dementia, and psychiatric disorders
Patients and methods During the period 2009 to June 2014, 21 Huntington's disease (HD) patients belonging to 17 unrelated families were recruited consecutively from the outpatient clinic of six medical centers in the province of Rabat-Salé (Morocco); the majority of them were seen in the Department of Neurology of Hospital of Spécialités
Statistical analysis was conducted using descriptive statistics, the relationship between the repeat length and the age at onset was assessed with the Bravais-Pearson correlation coefficient and statistical significance level was set at p < 0.05
Summary
Huntington’s disease (HD) is a rare neurodegenerative genetic disease, characterized by generalized chorea, dementia, and psychiatric disorders. Up to date there is no publication in the literature reporting patients from North African origin. This present study is the first report of clinical and genetic data characteristics of Huntington patients in Morocco. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Conclusion: Clinical and genetic data of Moroccan patients are similar to those of Caucasian populations previously reported in the literature.
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