Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study.

OBJECTIVE: We describe and compare the sociodemographic and clinical features, treatments, and prognoses and survival times of patients with amyotrophic lateral sclerosis (ALS) in Africa. METHODOLOGY: We conducted a multicentre, hospital-based cohort study in Africa. Patients with ALS diagnosed in the neurology departments of participating hospitals from 2005 to 2017 were included. Subgroup analysis was performed by subcontinent. Survival analyses were conducted using the Cox proportional hazards model. RESULTS: Nine centres from eight African countries participated. A total of 185 patients with ALS were included: 114 from Northern Africa, 41 from Western Africa and 30 from Southern Africa. A male predominance (male to female ratio 2.9) was evident. The median age at onset was 53.0 years (IQR 44.5-64.0 years). The onset was bulbar in 22.7%. Only 47 patients (26.3%) received riluzole, mainly in Northern and Western Africa. The median survival from the time of diagnosis was 14.0 months (95% CI 10.7 to 17.2 months). The median survival was longer in Northern Africa (19.0 months, 95% CI 10.8 to 27.2 months) than in Western (4.0 months, 95% CI 0.8 to 7.1 months) and Southern (11.0 months, 95% CI 5.6 to 16.4 months) Africa (Breslow test, p<0.0001). Both subcontinental location and riluzole treatment independently affected survival. CONCLUSION: More African patients with ALS were male and younger and exhibited a lower proportion of bulbar onset compared with patients with ALS from Western nations. Survival was consistent with that in Western registers but far shorter than what would be expected for young patients with ALS. The research improves our understanding of the disease in Africa.

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.

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.

Neuromyelitis Optica Spectrum Disorders in Black African: Experience of Togo (2015-2020).

Introduction Neuromyelitis optica spectrum disorders (NMOSD) would disproportionately affect blacks within mixed populations. However, they are rarely reported in black African. The objective of this work was to report the experience of Togo, a West African country in terms of NMOSD. Methods This is a series of six cases diagnosed between 2015 and 2020 in the only three neurology departments in Togo. The diagnosis of NMOSD was made according to the criteria of the International Panel for NMO Diagnosis (2015) and the patients had a minimum clinical follow-up of 6 months after the diagnosis. The search for anti-aquaporin 4 (AQP4) antibodies was performed by immunofluorescence on transfected cells. Results The mean age was 25.33 years and the sex ratio female/male was 5/1. The average time between the first attack and the diagnosis was 122.83 days. Clinically, there was isolated medullary involvement (2/6), simultaneous opticomedullary involvement (3/6), and area postrema syndrome (1/6). Five patients were anti-AQP4 positive. All six patients had extensive longitudinal myelitis. At 6 months of follow-up, there was one case of death and one case of blindness. Conclusion The rarity of NMOSD cases in Togo could be linked to an underestimation. To better characterize the NMOSDs of the black African population, multicenter and multidisciplinary studies are necessary.

Clinical management and disease-modifying treatment for amyotrophic lateral sclerosis in African hospital centers: the TROPALS study.

Objective: To assess the availability of health workers and medications for clinical management of amyotrophic lateral sclerosis (ALS) in African hospital centers. Availability and affordability analyses of disease-modifying treatments were performed. Methods: A multicenter observational study involving African hospitals was conducted. A standard questionnaire was developed based on the European Federation of the Neurological Societies (EFNS) guidelines. We collected data on multidisciplinary care and availability of medicines. The availability and affordability were evaluated according to the WHO guidelines. Results: Nine hospital centers from eight African countries participated. We observed a low degree of implementation of multidisciplinary care in ALS management. Riluzole was only available in centers from South Africa, Senegal, Tunisia, and Togo. This treatment was unaffordable and the adjusted price was highly variable among countries. The cost of riluzole was partly or fully covered by patients, which implies a substantial economic burden. Conclusion: Our findings strengthen the need to promote multidisciplinary care in the clinical management of ALS in Africa. Disease-modifying medication should be both available and affordable. Local and international collaboration is needed to improve ALS health care access in Africa.

[Cost of stroke in Lomé (Togo).] / Coût des accidents vasculaires cérébraux à Lomé (Togo).

The financial crisis that affected the healthcare systems of most developing countries in the 1980s, the ensuing need to control hospital costs, the partial disengagement of States, and the resort to policies based on cost recovery -- all these led to the restructuring of hospital systems in Africa, in accordance with the Bamako initiative, adopted by the WHO regional committee in September 1987. This restructuring required populations to pay much of the cost of their health care. In practice, however, the major obstacle to this policy of cost recovery remains poverty. Twenty years after the adoption of this initiative, we sought to evaluate the cost of hospitalisation for cerebral stroke in Togo, where there is no national health insurance programme, and to propose strategies to improve its management. This prospective study was conducted in the neurology department of the University Hospital of Lomé over a period of 12 months, from 1 January to 31 December 2005 and included 412 consecutive patients with a confirmed diagnosis according to WHO criteria and cerebral computed tomography (CT) results. This department has 30 beds in rooms categorized according to their cost to the patients: EUR 27.30 for a superior single room, EUR 18.20 for the first category standard room, EUR 13.7 euros for the second, and EUR 8.20 for the third. Patients or their family could choose their room category. Of the 412 patients included in our study, 248 (60.2%) had an ischaemic stroke (IS) and 164 (39.8%) a haemorrhagic stroke (HS). The average length of stay was 17.4 +/- 10.4 days (range: 3 to 41 days), 10.17 days (range: 3 to 24) for IS and 26.7 (range: 13 to 41) for HS. In all, 124 (30%) patients produced insurance certificates, and 288 (70%) paid directly; among the latter 152 (36.9%) patients paid their own expenses, while relatives paid for 65 (63.10%). Housewives accounted for 136 (33%) patients, 96 (23%) retired and 180 (20.4%) civil servants. No one chose the superior quality private room; 256 (62%) patients used category 3 rooms, 68 (27.2%) of them housewives and 44 (17.2%) retired. The total cost averaged EUR 679.6 +/- 297.90, almost 19 times higher than the minimum monthly salary of civil servants in Togo (EUR 36.30). The total for IS was EUR 428.80 +/- 188.9 and for HS, EUR 935.6 +/- 36.50. The average person in Togo spends EUR 3.99 per person per year on health, while a stroke patient hospitalized in Lome spends an average of 170 times more in only 17.4 days. Accordingly, most of the Togolese cannot access specialized neurology care for a stroke. Drug expenses accounted for the highest portion of the cost, in part because patients are obliged to buy retail pharmaceutical products that could have been provided to them at the hospital. Use of generic drugs could reduce this cost. Length of stay and tests could be reduced by setting time limits for procedures and setting up rehabilitation facilities.

[The epilepsy treatment gap in six primary care centres in Togo (2007-2009)]. / Déficits de traitement et épilepsie dans six unités de soins périphériques du Togo de 2007 à 2009.

Epilepsy, the most common serious neurological condition, is one of the most widespread non-transmissible diseases in the world. In developing countries, about 90% of those with epilepsy do not receive appropriate treatment; this treatment gap, very high compared with other chronic diseases, helps to explain the marginalisation and poor living conditions of these people. Reducing this treatment gap and the burden that epilepsy represents is a difficult task and the obstacles are numerous. The cultural attitudes, the absence of priority for this disease, the weak health infrastructure and the insufficient supply of anti-epileptics are just some of the factors that prevent adequate treatment. The extent of this problem led WHO and the International League against Epilepsy to launch an international campaign in June 1997 to bring epilepsy "out from the shadows". We sought to evaluate a strategy of community-based care for epilepsy in the six pilot districts. This strategy consisted in reducing the treatment gap in six local primary care units (PCUs) and then spreading the programme to surrounding PCUs, the entire district and then the entire region. This prospective evaluation study, which took place from May 2008 to July 2009, applied many strategies. WHO/AFRO made available funding of USD 3500 a year. A training meeting was held for PCU staff and community health agents, and numerous meetings from May 2007 through March 2008 aimed to increase awareness and motivation. The National Program for Mental Health (NPMH) ensured the availability of a permanent supply of anti-epileptics. Monitoring with supervision of activities and evaluation were conducted during and at the end of the process by the members of the Lomé Hospital neurology team and the management team of every district. Community-based management of 816 people with epilepsy over a period of 15 months was assessed internally. The planned strategies were conducted. The sex ratio (M/W) was 1.10. Treatment adhesion ranged from 96% to 99%. Mortality was 9%. The treatment gap in the PCUs, which varied from 98% to 94% in May 2008 fell by July 2009, ranging from only 40% to 25%. The "good practice" of accepting and treating patients with epilepsy in these areas where traditional culture excludes them from the community demanded the local acceptance of responsibility -- both medical and psychosocial. The reduction in epilepsy attacks and the integration of 2 or 3 patients in a community sufficed to bring other people with epilepsy out from the shadows. These successes show that in developing countries, it is possible to improve the health of different populations when these projects are integrated into primary health care. Positive results, and specifically a treatment gap below 50%, were obtained in all six PCUs. These results, acquired after months of activity, contributed to decrease the stigmatisation of epilepsy. Maintaining this reduction in the treatment gap requires continuation of the struggle against epilepsy and permanent improvement of primary health care. The often unplanned moves of staff and the reluctance of district and regional health managers to allocate resources to the project to perpetuate the programme constitute major difficulties. It appears urgent to adopt an active policy for providing care of patients with epilepsy in Africa in order to increase their lifespan.

Hémangiome vertébral neuro-agressif de révélation tardive: cas cliniqueA clinical case of late-onset aggressive vertebral hemangioma with neurological signs.

Résumé Dans la majorité des cas asymptomatiques, les hémangiomes vertébraux peuvent être, dans de rares cas, symptomatiques avec des manifestations cliniques purement neurologiques. S´ils sont fréquemment observés chez un sujet adulte jeune, ils peuvent exceptionnellement être observés chez un sujet âgé. Nous rapportons un cas d´hémangiome vertébral neuro-agressif de révélation tardive traité par une chirurgie décompressive, une sclérothérapie, une cimentoplastie et suivi d´une évolution favorable. English abstract In the majority of asymptomatic cases, vertebral hemangiomas can be, in rare cases, symptomatic with purely neurological clinical manifestations. They commonly occur in young adults, exceptionally in elderly subjects. We here report a case of late onset aggressive vertebral hemangioma with neurological signs treated with decompressive surgery, sclerotherapy and cementoplasty, with favorable outcome.

Carotid Dissection and Isolated Paralysis of Ipsilateral Half Tongue: Clinical Cases.

OBJECTIVE: We report two cases of carotid dissection revealed by isolated paralysis of the ipsilateral half tongue. Observations. First patient, 52 years old, with no particular medical or surgical history, presented with isolated paralysis of the left half tongue preceded by two weeks of moderate-intensity cervicalgia and having been the subject to cervical manipulation. MRI revealed dissection of the left internal carotid artery in its prepetrous portion. The evolution after 6 weeks of platelet aggregating inhibitors treatment was favorable. The second patient, 74 years old, with no particular medical or chirurgical history, presented with a sudden onset of paralysis of the left half tongue preceded by unusual headaches associated with neck pain. Brain MRI showed aneurysmal ectasia of the left internal carotid with parietal irregularity suggestive of carotid dissection. The evolution after four weeks of treatment with anticoagulant was favorable. CONCLUSION: Carotid dissection revealed by isolated paralysis of the half tongue is rare. It is generally of good prognosis. However, in paralysis of half tongue, it must be urgently sought and treated to reduce the risk of a transient or constituted ischemic accident.

Rasmussen's Epileptogenic Encephalitis in a Tropical Country.

BACKGROUND: Encephalitis of Rasmussen is an inflammatory hemiencephalopathy of unknown etiology. It is a cause of drug-resistant epilepsy. AIM: To report two cases of Rasmussen's encephalitis (RE) in a low-income setting. CLINICAL OBSERVATION: The cases concerned were that of an 8-year-old boy and a 4-year-old girl. The illness began with daily several seizures at the age of 28 months in the boy and 23 months for the girl. Epileptic seizures were generalized in the elder one and focal in the younger. The elder presented right hemiplegia with severe cognitive impairment. In the younger child, the expression of the language was disturbed, associated with right hemiparesis at 4/5. The electroencephalography recording showed background theta asymmetric rhythm associated with discharges of periodic lateralized epileptiform discharges (PLEDs) into the left hemisphere in the two cases. Brain imaging showed left hemisphere atrophy. The seizures had decreased in intensity after association of several anticonvulsant molecules over a period of 3-6 months. The diagnosis of RE was based on clinical, paraclinical, therapeutic, and evolution arguments. CONCLUSION: There was a delay to establish the diagnosis. Further studies are needed to evaluate rehabilitation capacities in children with RE before brain maturation.

Febrile seizures in one-five aged infants in tropical practice: Frequency, etiology and outcome of hospitalization.

BACKGROUND: Convulsive seizures are the common neurological emergencies in developing regions. OBJECTIVES: The aim was to determine the prevalence, causes and outcome of seizures in childhood. PATIENTS AND METHODS: Participants were children aged 1-5 years old, admitted consecutively with a history of febrile convulsions or were presented seizures with fever during hospitalization, in two pediatric university hospitals. The prospective study covered a period from January to December 2013. At admission, emergency care and resuscitation procedures were provided according to the national guidelines. The history included the number and a parental description of seizures. Children with epilepsy, any central nervous system infections and other disease were excluded. RESULTS: We have recorded 3647 children. Among them, 308 (8.4%) infants had presented with febrile seizures including 174 males and 134 females admitted to both pediatric hospitals (Tokoin University Teaching Hospitals: 206/3070, Campus University Teaching Hospitals: 102/577). Infants from 1 to 3 years age were the most common affected and constituted 65.9% of all patients. The months of September, December and January had recorded the high frequency of admission due to seizures. Regarding the seizures type, generalized tonic-clonic seizures were predominant (46.4%) followed by tonic seizures (17.2%) and status epilepticus in 9%. The etiologies were marked by falciparum malaria (52.3%), and other infections in 47.7%. At discharge, we have noted 11% (34/308) with neurodevelopmental disabilities, 6.7% of epilepsy and 9.7% (30/308) of death. CONCLUSION: The febrile seizure in child younger 5 years is an indicator of severe malaria in tropical nations. The campaign for "roll back malaria" must continue in developing countries to avoid long-term gross neurological deficits.

Delayed early primary visual pathway development in premature infants: high density electrophysiological evidence.

In the past decades, multiple studies have been interested in developmental patterns of the visual system in healthy infants. During the first year of life, differential maturational changes have been observed between the Magnocellular (P) and the Parvocellular (P) visual pathways. However, few studies investigated P and M system development in infants born prematurely. The aim of the present study was to characterize P and M system maturational differences between healthy preterm and fullterm infants through a critical period of visual maturation: the first year of life. Using a cross-sectional design, high-density electroencephalogram (EEG) was recorded in 31 healthy preterms and 41 fullterm infants of 3, 6, or 12 months (corrected age for premature babies). Three visual stimulations varying in contrast and spatial frequency were presented to stimulate preferentially the M pathway, the P pathway, or both systems simultaneously during EEG recordings. Results from early visual evoked potentials in response to the stimulation that activates simultaneously both systems revealed longer N1 latencies and smaller P1 amplitudes in preterm infants compared to fullterms. Moreover, preterms showed longer N1 and P1 latencies in response to stimuli assessing the M pathway at 3 months. No differences between preterms and fullterms were found when using the preferential P system stimulation. In order to identify the cerebral generator of each visual response, distributed source analyses were computed in 12-month-old infants using LORETA. Source analysis demonstrated an activation of the parietal dorsal region in fullterm infants, in response to the preferential M pathway, which was not seen in the preterms. Overall, these findings suggest that the Magnocellular pathway development is affected in premature infants. Although our VEP results suggest that premature children overcome, at least partially, the visual developmental delay with time, source analyses reveal abnormal brain activation of the Magnocellular pathway at 12 months of age.