Dermatomyosite avec atteinte cardiaque : A propos d’un cas observe au Senegal

Introduction : La dermatomyosite est une myopathie inflammatoire chronique caractérisée essentiellement par une faiblesse musculaire associée à des lésions cutanées caractéristiques.Cas clinique : Nous rapportons l‘observation d‘une patiente de 20 ans qui a présenté 6 mois auparavant une faiblesse musculaire d‘installation progressive associée à des troubles de la déglutition. L‘examen clinique retrouvait un déficit moteur des 4 membres à 1/5 en proximal et 3/5 en distal avec des lésions dermatologiques érythématosquameuses, et une tachyarythmie à l‘auscultation cardiaque. L‘électroneuromyogramme a montré des potentiels d‘unités motrices polyphasiques, d‘amplitude et de durée limitée, avec des vitesses de  conduction nerveuse motrice et sensitive normales. L‘écho-Doppler cardiaque a montré une fraction d‘éjection systolique à 40%. Le dosage des enzymes musculaires révélait une élévation des CPK à 1200 UI/L, LDH à 1284 UI/L et des aldolases à 35 UI/L. Le diagnostic de dermatomyosite associée à une atteinte cardiaque a été  retenu. La patiente a été traitée par corticothérapie avec une bonne évolution clinique.Conclusion : Notre observation souligne l‘intérêt de rechercher systématiquement une atteinte cardiaque chez les patients présentant un tableau de dermatomyosite. English title: Introduction : Dermatomyositis associated with cardiac involvement: A case observed in Senegal Background: Dermatomyositis is a chronic inflammatory myopathy characterized by muscle weakness associated with characteristic skin lesions.Case report: We report the case of a 20-year-old woman who presented 6 months before muscle weakness and swallowing disorders. Clinical examination showed a motor deficit of four limbs (1/5 proximal and 3/5 distal)  with dermatological lesions and tachyarrhythmia in cardiac auscultation. Electromyography showed multiphase motor unit potentials, with decreased amplitude and duration, and normal motor and sensory nerve   conduction velocities. Echocardiography showed a reduced left ventricular ejection of 40%. The dosage of the muscle enzymes revealed a high rate of Creatine Phosphokinase at 1200 IU/L, LDH at 1284 UI/L and aldolase at 35 UI/L. Diagnosis of dermatomyositis associated with cardiac involvement was retained. The patient was   treated with steroids with a good clinical response.Conclusion: Our observation underlines the value of systematically looking at cardiac involvement in patients with dermatomyositis

Traitement de la neuromyélite optique de Devic durant de la grossesse

La neuromyélite optique de Devic est une pathologie inflammatoire démyélinisante du système nerveux central qui affecte électivement la moelle spinale, le nerf optique et les régions cérébrales à haute expression d’antigènes aquaporine 4. Il s’agit d’une pathologie auto-immune sévère due à des auto-anticorps dirigés contre l’aquaporine 4, à taux de morbidité et de mortalité élevé. Contrairement à d’autres pathologies inflammatoires notamment la sclérose en plaques ou polyarthrite rhumatoïde, la grossesse n’exerce aucune influence sur l’activité de la neuromyélite optique d’où la nécessité d’instaurer un traitement de fond durant toute la grossesse. La corticothérapie représente le traitement de premier choix de la neuromyélite optique durant la grossesse. D’autres traitements peuvent également être utilisés notamment le rituximab, certains immunosuppresseurs, les immunoglobulines. Le traitement par immunosuppresseurs ou rituximab est proposé lorsque la corticothérapie au long cours est contre-indiquée ou en cas d’inefficacité à celle-ci ou encore lorsque les effets secondaires sont intolérables. Les immunoglobulines sont administrées en cas de poussées sévères de la neuromyélite optique qui ne répondent pas aux bolus de methylprednisolone. Les immunoglobulines peuvent également être poursuivies seules à la dose 0,4g/kg/j toutes les 6 à 8 semaines jusqu’à l’accouchement. La plasmaphérèse est également une bonne alternative aux bolus de methylprednisolone lorsque les poussées sont très sévères.The Pan African Medical Journal 2016;2

Hurler–Scheie syndrome in Niger: a case series

Abstract Background Hurler–Scheie syndrome is an intermediate form of mucopolysaccharidosis type I which is a rare lysosomal storage disorder caused by the deficiency or complete absence of enzyme alpha-L-iduronidase activity. We report the first documented cases of Hurler–Scheie syndrome observed in Niger in a Touareg family. Case presentation We studied the case of two 12-year-old twin Touareg boys and their 10-year-old Touareg sister whose parents are first-degree cousins, and there was no history of similar cases in their previous generations. The diagnosis of Hurler–Scheie syndrome was considered in these patients on the basis of clinical and radiological arguments, with the highlighting of a deficiency of enzyme alpha-L-iduronidase in serum and leukocytes. The twins had presented the first symptoms at the age of 24 months and the diagnosis of Hurler–Scheie syndrome was made at the age of 12 years. In their younger sister, the first symptoms were observed at the age of 3 years and the diagnosis was made at the age of 10 years. The three probands were born after a normal full-term pregnancy and a spontaneous vaginal delivery according to their parents. Their birth weight, height, and head circumference were within normal limits according to their parents. The three probands were brought in for consultation for stunted growth, joint stiffness with gait disorders, deformities of the thoracolumbar spine, recurrent otitis media, decreased hearing, increased abdominal volume, snoring during sleep, and facial dysmorphism. Conclusions Even in countries with limited access to diagnostic means, a good knowledge of the clinical manifestations of the disease can help to guide the diagnosis of mucopolysaccharidosis type I

Clinical Profile of Parkinson's Disease: Experience of Niger

Background: Parkinson's disease (PD) is a chronic neurodegenerative pathology with unknown etiology. It is characterized clinically by the classic triad that associated tremors, bradykinesia, and rigidity. In Niger, there are no data on PD. Aims: We aimed to provide the demographic and clinical profile of PD in patients from Niger to create a database on PD in Niger. Patients and Methods: We conducted a retrospective study at the Neurology Outpatient Clinic of the Hôpital National de Niamey (HNN, Niger) over a period of 4.42 years from February 2009 to July 2013 collecting all cases of PD. The demographic and clinical features of all patients were collected and analyzed. Results: During the period of the study, 1695 patients consulted at the Neurology Outpatient Clinic of the HNN, among which 76 patients (4.48%) had secondary parkinsonism and 25 patients (1.47%) had features compatible with PD. Only patients with PD were included in this study. The mean age at onset of symptoms was 58 years (range: 42–74 years). The male sex was predominant (60%) with a sex ratio of 1.5. The mean time interval from the onset of symptoms to diagnosis of PD was 1.8 years (range: 1–5 years). The tremor was the most common symptom (84%). Bradykinesia represented 64% of the symptoms and rigidity 20%. At the time of the diagnosis of PD, 8 patients (32%) were in Stage I of the classification of Hoehn and Yahr, 16 patients (64%) in Stage II, and 1 patient (4%) in Stage III. The levodopa/carbidopa combination was the most used antiparkinsonian drug in our patients (88%). The mean time of follow-up of the patients was 2.5 years (range: 1–4.42 years). During the course of the disease, 9 patients (36%) were in Stage II of the classification of Hoehn and Yahr, 13 patients (52%) in Stage III, and 3 patients (12%) in Stage IV. Conclusion: Our study provides demographic and clinical data of PD in patients from Niger and shows that the hospital frequency of this disease is low (1.47%). The demographic and clinical features of our patients are similar to those of the patients of the prior studies reported in sub-Saharan Africa