Encephalopathie epileptique infantile precoce d’etiologie anoxo-ischemique au Chu Campus de Lome (Togo)

L’Encéphalopathie Epileptique Infantile Précoce (EEIP) ou syndrome d’Ohtahara, qui est une forme rare d’encéphalopathie néonatale, présente des caractéristiques électro-cliniques, thérapeutiques et évolutives particulières. Ses étiologies, essentiellement structurelles, sont plus malformatives qu’anoxo-ischémiques pour la majorité des cas décrits dans la littérature. Nous rapportons les deux premiers cas d’EEIP d’étiologie anoxo-ischémique, confirmés par la tomodensitométrie cérébrale et suivis dans le service de neurologie depuis l’âge de 2 et 5 mois, respectivement. Les patients avaient des antécédents de souffrance cérébrale néonatale et, jusqu’à l’âge d’un an, présentaient une évolution clinique sévère marquée par la persistancede spasmes toniques en extension (plus de 20 crises par jour) et un retard de développement psychomoteur, malgré la prise de phénobarbital, de carbamazépine ou de valproate de sodium et de méthylprednisone. A l’âge de huit mois et un an, l’électroencéphalogramme de sommeil était toujours de type suppression-burst, tandis que celui de veille se transformait en hypsarythmie (syndrome de West).Mots-clés: Syndrome d’0htahara ; Encéphalopathie épileptique infantile précoce ; Syndromes épileptiques ; Anoxie cérébrale ; Lomé   Early infantile epileptic encephalopathy with anoxo-ischemic etiology at University Hospital Campus of Lome (Togo)Early Infantile Epileptic Encephalopathy (EIEE) or Ohtahara syndrome is a rare form of neonatal encephalopathy. It presents particular electro-clinical, therapeutic and outcome features. Its etiologies are essentially structural and more malformative than anoxo-ischemic for most of the cases which are described in literature. We report the first two cases of EIEE with anoxo-ischemic etiology, which were confirmed by CT-scan and followed up in the department of neurology since 2 and 5 months old, respectively. Patients had a neonatal cerebral pain history and, up to one-year-old, presented a severe outcome which was characterized by the persistence of tonic spasms in extension (more than 20 seizures per day) and psychomotor development delay, despite taking phenobarbital, carbamazepine or sodium valproate and methylprednisone. At eight months and one year old, the electroencephalogram of sleep presented showed suppression-burst pattern, while that of awakening was transformed into hypsarrhythmia (West’s syndrome). Key words: Ohtahara Syndrome; Early Infantile Epileptic Encephalopathy; Epileptic Syndromes; Cerebral Anoxia; Lomé

Case report: an area postrema syndrome revealing a neuromyelitis optica spectrum disorder associated with central nervous system tuberculosis in a young Togolese (black African) woman

Abstract Background Area postrema syndrome (APS) is considered to be one of the most specific clinical presentations of neuromyelitis optica spectrum disorders (NMOSDs). In sub-Saharan Africa, NMOSDs and even more so those revealed by an APS, are rarely reported. However, studies among mixed populations have shown that NMOSDs disproportionately affect black people with relatively more frequent encephalic involvement. We report a case of APS revealing an NMOSD associated with central nervous system (CNS) tuberculosis in a young Togolese woman residing in Togo (West Africa). Case presentation A 28-year-old Togolese woman was admitted for left hemibody sensory problems with ataxia. These problems were observed while the patient was hospitalized for a few days in the hepato-gastroenterology department for persistent vomiting, abdominal pain and hiccups lasting for about a month. The examination confirmed left hemibody ataxia with nystagmus when looking to the left, pronounced left osteotendinous reflexes, and left hemibody hypoesthesia up to the base of the neck. Encephalic magnetic resonance imaging (MRI) showed a hypersignal lesion in the bulbar more lateralized on the left in the fluid-attenuated inversion recovery sequence, not enhanced after a gadolinium injection. Biological assessment showed the presence of Mycobacterium tuberculosis deoxyribonucleic acid in the cerebrospinal fluid and a sedimentation rate of 120 mm in the 1st hour. The result of the anti-AQP4 antibody test was positive. Two months from the onset of digestive problems with Lhermitte’s sign and hand and foot contracture access without vesico-sphincter problems were established. Cervical medullary MRI showed an additional intramedullary hypersignal lesion in the T2 sequence at the C2 level, not enhanced after a gadolinium injection. A second course of intravenous corticosteroids was administered, and anti-tuberculosis treatment was continued. The outcome was favorable. After 8 months of anti-tuberculosis treatment, the patient started immunosuppressive therapy (azathioprine 50 mg twice daily) to limit the risk of recurrence of NMOSD. Conclusion The recognition of an APS is an additional challenge for the diagnosis of NMOSDs, especially in countries with limited resources. CNS tuberculosis must be tested when faced with an NMOSD because it seems to be a major cause

Deficits cognitifs chez les enfants et adolescents atteints de drepanocytose (Genotype SS) a Brazzaville

Introduction : La drépanocytose impacte négativement le neurodéveloppement de l’enfant. Objectifs : Déterminer la fréquence des déficits cognitifs chez les enfants et adolescents drépanocytaires homozygotes à Brazzaville ; identifier les facteurs associés. Méthodes : Il s’est agi d’une étude transversale analytique. Elle a porté sur les enfants drépanocytaires homozygotes âgés de 6 à 16 ans. Elle a été réalisée au Centre National de Référence de la Drépanocytose de Brazzaville, de mars à septembre 2019. Les domaines neurocognitifs ont été évalués par l’échelle d’intelligence de Wechsler pour enfants, 5e édition. Les variables d’étude ont été sociodémographiques et cliniques. Résultats : Sur 130 enfants drépanocytaires homozygotes reçus en consultation de routine dans le cadre de leur suivi, 83 (63,84%) présentaient des déficits cognitifs. Parmi eux, 43 (51,80 %) étaient des garçons et 40 (48,20%), des filles. L’âge moyen était de 10,93 ± 2,83 ans. Les patients présentaient des déficits de langage, des habilités visuo-spatiales et des fonctions attentionnelles et exécutives. Les accidents vasculaires cérébraux, l’anémie sévère et l’âge avaient un impact sur ces déficits cognitifs. La mémoire de travail n’était pas altérée. L’intelligence générale était conservée. Conclusion : Dans notre milieu, les déficits cognitifs sont fréquents chez les enfants et adolescents drépanocytaires homozygotes. Les facteurs associés à l’altération des fonctions neurocognitives sont similaires à ceux rapportés dans la littérature. De ce fait, il est important d’inclure une évaluation neurocognitive systématique dans le suivi de ces enfants et de les accompagner par des méthodes pédagogiques adaptées ou des programmes de réhabilitation cognitive.   English title: Cognitive deficits in children and adolescents with sickle cell disease (Genotype SS) in Brazzaville Introduction: Sickle cell disease has an negative impact on neurodevelopment in child. Objectives: To determine the frequency of cognitive deficits in children and adolescents with sickle cell disease (genotype SS) in  Brazzaville; to identify the associated factors. Methods: An analytical prospective study was conducted at the National Center of Reference of Sickle Cell Disease in Brazzaville, from March to September 2019. It included 6-16-year-old children with homozygous sickle cell disease (genotype SS). Neurocognitive domains were evaluated by means of the Wechsler Intelligence Scale for Children, Fifth Version. Analyzed parameters were sociodemographic and clinical. Results: Among the 130 children with homozygous sickle cell disease presenting for a routine clinic visit for follow-up, 83 (63.84%) had cognitive deficits. Of these, there are 43 (51.80 %) boys and 40 (48.20%) girls. Mean age was 10.93 ± 2.83 years. Deficits of language, visuo-spatial functions, attention and executive functions were detected in patients. Strokes, severe anemia and age had an impact on these cognitive deficits. Working memory was not impaired. General intelligence was preserved. Conclusion: In our setting, cognitive deficits are very frequent in children and adolescents with homozygous sickle cell disease. The factors associated with neurocognitive impairment in our patients get closer to those reported in literature. It is important to include a systematic neurocognitive assessment in the follow-up of these children and to support them with adapted teaching methods or cognitive rehabilitation programs. &nbsp