THERAPEUTIC CHALLENGES OF HUNTINGTON'S DISEASE IN GUINEA: Study of HD confirmed cases in a country with limited resources.
GUELNGAR Carlos Othon1, Mendi Julianne Muthinja2, Mie Rizig3, Abass Fode Cisse 1, Amara cisse1
1Department of Neurology, National Ignace Deen Hospital, University of Conakry, Conakry, Guinea, 2Huntington disease Africa, P. box. 46092-00100, Huntington disease Africa, 3Neurology, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK
Objective:
To highlight the difficulties of the management of Huntington's chorea in a context of under-medicalization.
Background:
In a tropical environment, the biomolecular diagnostic certainty of an etiology of Huntington's chorea is difficult to establish because of the absence of a molecular biology laboratory and the therapeutic approach constitutes a major problem in a country with limited resources.
Design/Methods:
This was a study of mHtt positive cases from the the African Research Consortium on Huntington’s disease (ARCH) for a period of 4 years (2018-2022), in the Neurology Department of the University Hospital of Conakry. We included all patients, presenting neurological or psychiatric manifestations, family history, in relation to the criteria established by the UHDRS. Therapeutic options were chosen according to the availability of certain molecules in our context.
Results:
Haloperidol, Tetrabenazine, Brain active Denk ® and Gamalate-B6 ® have significantly improved the motor disorders and intellectual performance of our patients. Curiously, one case of death due to decubitus complications during hospitalization was reported.
Conclusions:
This study, the first of its kind in Guinea, shows the difficulties of managing HD in a resource-limited environment. The lack of biomolecular laboratories and the inaccessibility of tetrabenazine due to its high cost were the subject of a plea to the Ministry of Public Health.
Key words: Huntington's disease, motor disorders, therapeutics, Guinea.