Neuromyelitis Optica Spectrum Disorder Presenting Radiologically like Spinal Astrocytoma

Neuromyelitis optica spectrum disorder (NMOSD) is an infrequent antibody-mediated disorder of the central nervous system. The diagnosis is made in the setting of specific clinical syndromes and a positive aquaporin‑4 antibody titer. Intramedullary spinal cord tumors may clinically and radiologically mimic NMOSD, necessitating careful differentiation, especially in individuals who meet the epidemiological profile of NMOSD. We report two cases of NMOSD in young female patients who had a neuroradiological diagnosis of spinal cord astrocytoma and were initially planned for spinal cord surgery. We aim to highlight the similarities between NMOSD and spinal cord tumors using these two clinical cases

Pattern, Risk Factors, and Outcome of Acute Stroke in a Nigerian University Teaching Hospital: A 1‑Year Review

Background: This study aimed to document the pattern, risk factors, in‑hospital outcomes, and stroke mortality in a hospital over oneyear. Materials and Methods: Acute stroke patients admitted at the Lagos State University Teaching Hospital between October 2019 andSeptember 2020 had their records reviewed. Information including age, sex, risk factors, stroke type, access to neuroimaging, and the in‑hospital outcome was extracted and analyzed. Results: A total of 230 patient records were included in this study. The proportion of intracerebral hemorrhage (ICH) was 44.8%, while  ischemic stroke was 52.2%. Only 9.1% of ischemic stroke cases had an onset‑to‑arrival time of fewer than nine hours, with just three thrombolytic therapy given. The 30‑day mortality was 28.7%, lesser among younger patients and patients managed in the stroke unit. Conclusion: The hospital incidence of ICH was close to that of ischemic stroke. Mortality data confirms the importance of management in a stroke unit. Keywords: Nigeria, outcome, pattern, risk factors, strok

Machado-Joseph disease in a Nigerian family: mutational origin and review of the literature

Machado-Joseph disease (MJD) has been described in Africans, but no cases have been reported from Nigeria. Current MJD global distribution results from both the ancestral populations-of-origin and the founder effects of mutations, some as a consequence of the Portuguese sea travels in the 15th to 16th century. Two main ancestral haplotypes have been identified: the Machado lineage, which is more recent, predominant in families of Portuguese extraction, and the Joseph lineage, which is much older and worldwide spread, postulated to have an Asian origin. We report a Nigerian family with MJD from Calabar, once settled by Portuguese slave traders, and assessed its mutational origin. The proband was a 33-year-old man with progressive unsteady gait, weakness of all limbs, dysphagia, dysarthria, urinary frequency and diaphoresis. He had end-of-gaze nystagmus, spastic quadriparesis and atrophic small muscles of the hand. He showed fibrillation potentials on EMG, and nerve conduction studies suggested a central axonopathy without demyelination. This family bears the Joseph haplotype, which has a founder effect in the island of Flores, in the Azores (and their descendants in North-America), but is also the most common in non-Portuguese populations worldwide, with an estimated mutation age of around 7000 years.SM is the recipient of a scholarship (SFRH/BPD/77969/2011) from the Portuguese Foundation for Science and Technology (FCT); IPATIMUP is partially supported by FCT. This work was co-financed by the European Social Fund (Human Potential Thematic Operational Programme)

“Myasthenia Gravis and Anesthesia – A Review of the Literature” (English)

Myasthenia gravis (MG) is an immunological disorder characterized by damaged acetylcholine receptors (AchR) due to antibody, which not only blocks the receptor site but also causes degenerations of the receptors.. It is closely associated with disorders of similar pathogenesis, such as pernicious anaemia, thyrotoxicosis, SLE, hypothyroidism, rheumatoid like arthritis, red cell aplasia and polymyositis. The disease is characterized by skeletal muscle weakness and fatigability upon sustained effort and commonly affects the ocular, bulbar and limb muscles. Anticholinesterases, immunosuppressive drugs, plasmaphresis, and thymectomy are the four strategies in the treatment of myasthenia. The thymus plays a critical role in immuno surveillance and has been incriminated in its pathogenesis. There is therefore a general consensus that adults with myasthenia gravis should have thymectomy. The anaesthetic management of the myaesthenic patient must be individualized to the severity of the disease and the type of surgery. Such patients may have an abnormal response to muscle relaxants, a restricted respiratory capacity with inability to cough and clear the increased secretions resulting from anticholinesterase therapy or have a co-existing cardiomyopathy. It may be safe and advantageous to discontinue anticholinesterase therapy just before surgery in order to minimize their vagotonic side effects. It is also safe to decrease the dose of depolarizing and non-depolarising blockers required to maintain muscle relaxation during surgery. Furthermore, MG patients are at increased risk of developing post-operative respiratory failure and it is likely that they would require ventilatory support after surgery. Post-operative care should be done in the intensive care unit with tracheal suction and monitoring of respiratory function as well as chest physiotherapy. Despite the anaesthetic, intra- and post-operative problems envisaged in patients with myasthenia gravis, cases with mild generalized MG could have uneventful operations if necessary precautions are taken. The course of MG is extremely variable, although, spontaneous remission is common, relapse is the rule. Résumé La myasthénie gravis (MG) est liée à un dysfonctionnement du système immunitaire. (Il) Elle se caractérise par une anomalie des récepteurs de l´acétylcholine (AchR) causée par des anticorps avec pour conséquence non seulement un blocage du site des récepteurs mais également leur dégénerescence. Elle peut être associée à des affections de pathogénie identique telles que l´anémie pernicieuse, la thyréotoxicose, l´hypothyroïde ou encore la polyarthrite rhumatoïde, l´aplasie médullaire et la polymyosite. La maladie se caractérise par une faiblesse des muscles squelettiques et une fatigabilité après un effort soutenu. Elle atteint généralement les muscles oculaires extrinsèques, les muscles pharyngo-laryngés et de la face ainsi que les muscles des membres. Il existe quatre types de traitement contre la myasthénie : les anticholinesthérasiques, les immunodépresseurs, les échanges plasmatiques et la thymectomie. Le thymus qui joue un rôle décisif dans l´immuno-régulation et est impliquée dans la pathogénie . Ainsi, l´on s´accorde à penser que les adultes atteints de myasthénie gravis devraient avoir recours à une thymectomie. La prise en charge anesthésique du malade myasthénique doit être individualisée selon la gravité de la maladie et le procédé chirurgical. En effet, ces patients peuvent avoir une réaction négative aux relaxants musculaires, souffrir d´une insuffisance respiratoire ou être dans l´impossibilité de tousser et d\'expectorer les sécrétions importantes apparues après un traitement anticholinestérasique ou en rapport avec une myocardiopathie coexistante. Il est prudent d´interrompre le traitement anticholinesthérasique juste avant l´intervention afin de réduire les effets vagotoniques secondaires. Il est également conseillé de réduire la dose de curarisants polarisants et non - polarisants nécessaires au maintien du relâchement musculaire au cours de l´intervention. En outre, les malades myasthéniques sont exposés à des risques respiratoires importants et peuvent développer des défaillances en post-opératoire nécessitant ainsi assistance respiratoire. Des soins post-opératoires devront être apportés dans le service de soins intensifs : aspiration trachéale, monitoring des fonctions respiratoires et kinésithérapie respiratoire. En dépit des problèmes anesthésiques per-opératoires et post-opératoires pouvant survenir chez les patients atteints de myasthénie gravis, la forme légère généralisée peut être opérée sans complication à condition de prendre les précautions nécessaires. L´évolution de la maladie est extrêmement variée marquée par des poussées et bien qu´une rémission soit courante, il faut néanmoins s´attendre à des rechutes. (Af. J. of Neurological Sciences: 2003 22(2)

Risk and Impact of Inter-ictal Depression on Quality of life of Nigerian Women with Epilepsy of Childbearing Age.

Background: Depression, the most common psychiatry comorbidity in patients with epilepsy is common among women, especially those of reproductive age. Although it could be said that the female gender is a risk factor for depression, the risk of inter-ictal depression and its impact on the quality of life (QoL) of women with epilepsy (WWE) has not been sufficiently studied among Nigerians. Materials and Methods: In this cross-sectional case–control study, data were collected from 70 women (29 WWE and 41 women without epilepsy) within the age range ≥18 and ≤55 between July 2010 and March 2011. A questionnaire that includes items-related to demographic information, becks depression inventory-II (BDI-II) and QoL inventory in epilepsy-31 was used for data collection. Result: WWE had a significantly higher BDI-II score (P = 0.001). The frequency of depression was 37.9% in WWE and 4.9% among controls. (Odds ratio 11.9). WWE had poorer QoL score than the control (P ≤ 0.001). Depressed WWE had poorer total QoL (P = 0.007) as well as poorer emotional well-being ( Conclusion: Depression is prevalent in this sample of Nigerian WWE of childbearing age with significant impact on their total QoL, emotional well-being and social function. Regular screening for depression among this population of PWE is imperative. Rational drug management as well as non-pharmacological treatment of depressed WWE is emphasized

Machado–Joseph disease in a Nigerian family: mutational origin and review of the literature

Machado–Joseph disease (MJD) has been described in Africans, but no cases have been reported from Nigeria. Current MJD global distribution results from both the ancestral populations-of-origin and the founder effects of mutations, some as a consequence of the Portuguese sea travels in the 15th to 16th century. Two main ancestral haplotypes have been identified: the Machado lineage, which is more recent, predominant in families of Portuguese extraction, and the Joseph lineage, which is much older and worldwide spread, postulated to have an Asian origin. We report a Nigerian family with MJD from Calabar, once settled by Portuguese slave traders, and assessed its mutational origin. The proband was a 33-year-old man with progressive unsteady gait, weakness of all limbs, dysphagia, dysarthria, urinary frequency and diaphoresis. He had end-of-gaze nystagmus, spastic quadriparesis and atrophic small muscles of the hand. He showed fibrillation potentials on EMG, and nerve conduction studies suggested a central axonopathy without demyelination. This family bears the Joseph haplotype, which has a founder effect in the island of Flores, in the Azores (and their descendants in North-America), but is also the most common in non-Portuguese populations worldwide, with an estimated mutation age of around 7000 years

Accuracy of the Siriraj Stroke Score in Differentiating Cerebral Haemorrhage and Infarction in African Nigerians (English)

Background Scoring systems based on discriminant analysis technique and multivariate logistic regression have been developed to distinguish cerebral haemorrhage (CH) from cerebral infarction (CI). This distinction is necessary in the acute management of stroke patients. Objectif The Siriraj stroke score (SSS) was evaluated in Siriraj hospital, Bangkok, and the Western Infirmary, Glasgow, and needs to be validated in an African setting. Methodology The computerised tomography (CT) brain scans of all patients referred with clinical diagnosis of stroke at the University College Hospital (UCH), Ibadan, and RADMED diagnostic centre, Lagos were retrieved and reviewed as well as the case notes of the patients at the referral hospitals. The SSS was computed and the stroke subtype classified. This was compared with the CTscan diagnosis using the latter as the gold standard. Data analysis was performed with Epi-info software and by standard statistical methods. Results Ninety-six patients had complete clinical records and CT scan features consistent with stroke. Of these, 52 had cerebral infarction (CI) and 44 had cerebral haemorrhage (CH). SSS had sensitivity of 50% for CH and 58% for CI with an accuracy of 54.2%. Conclusion In this study, the SSS was not sufficiently sensitive to differentiate between CH and CI in Africans. A prospective study with larger sample size and modification of the discriminant variables is suggested. For now, while efforts must continue to find a simple clinical scoring system to differentiate between CH and CI, we contend that CTscan should remain the investigative technique of choice and should be made affordable and available. Keywords: Africa, cerebral haemorrhage, cerebral infarction, Nigeria, stroke, CT-scan, Accident vasculaire cérébral hemorragique, Accident vasculaire cérébral ischémique, A frique, Nigeria, tomodensitométrie Résumé  Introduction Dans le but d\'agir avec efficacité dans la prise en charge thérapeutique des accidents vasculaires cérébraux un score discriminant à l\'aide de variables multiples a été élaboré afin de distinguer les accidents vasculaires hémorragiques (AVCH) des accidents vasculaires ischémiques (AVCI). Objective Le Siriraj stroke score (SSS) a été évalué au Siriraj hospital, Bangkok et au Western infirmary, Glasgow. Il importait de le valider dans le cadre africain. Méthodologie Les dossiers des malades hospitalisés au Centre Hospitalier Universitaire d\'Ibadan et au centre diagnostic RADMED à Lagos ont été étudiés rétrospectvement de même que les CT-scan. Les données scannographiques ont été comparées au SSS. Les informations ont été analysées grâce au logiciel Epi-info et à l\'aide de différentes méthodes stastistiques. Résultats 96 patitents ont été retenus. 52 malades avaient un infarcissement cérébral et 44 une hémorragie cérébrale. Le SSS avait une sensiblité de 50% pour les AVC hémorragiques et 58% pour les AVCI avec une spécificité de 54,2%. Conclusion Cette étude permet de conclure en l\'absence de spécificité du SSS dans la distinction des AVCH et AVCI chez les Africains. Une étude prospective est préconisée en modifiant certaines variables. Actuellement le CT-scan reste la technique de choix pour différencier les AVCI des AVCH. Af J Neuro Sci: 2002 20(1

A review of NeuroAiDTM II (MLC901) development in Alzheimer’s disease treatment: promises of a multimodal pathway

Background: Alzheimer’s disease (AD) is a clinical and economic burden on society. Without new treatment, the impact of AD on society could triple by 2050. Aim: After a brief overview of treatments and challenges of new drug developments for AD, we reviewed the preclinical and clinical development program of NeuroAiD (MLC901, MLC601). Method: A literature search was conducted by using different web sources. The initial screening was based on keywords contained in the subtitles of each corresponding paragraphs of this article. We sorted the reviewed publications by relevance and publication date selecting 74 references out of the 319 initially shortlisted for review. Review: Since 1998, only symptomatic drugs were marketed. Intensive research has continued, aimed at delaying the onset of the disease and/or slowing its progression. However, the predictive value of delaying the onset of AD remains debated. Since 2003, aducanumab is the first new treatment approved and registered by US-FDA as an amyloid beta-directed antibody indicated for the treatment of Alzheimer’s disease under post-marketing conditions. Traditional medicines (TM) have shown interesting results, but many of TM clinical studies leave much to be desired from a methodological point of view. Among TM, NeuroAiD (MLC901/601), a botanical-derived combination, acts in a multimodal pathway combining neuroprotective and neuroregenerative properties. It has demonstrated sustained symptomatic benefits, slowing the disease progression in AD with a good safety profile. Discussion/Conclusions: The discovery of treatments preventing or slowing down the disease progression, are necessary to get reliable diagnostic tools to confirm AD diagnosis, and follow its evolution and long-term therapy. A growing consensus is emerging on the need for a multi-factorial approach to the treatment and the development of suitable AD drug combinations. Such an approach has been that of TM for a long time. This is the case for NeuroAiD, that it may be integrated safely either after symptomatic treatments have failed or on top of symptomatic treatments

Machado–Joseph disease in a Nigerian family: mutational origin and review of the literature

Machado–Joseph disease (MJD) has been described in Africans, but no cases have been reported from Nigeria. Current MJD global distribution results from both the ancestral populations-of-origin and the founder effects of mutations, some as a consequence of the Portuguese sea travels in the 15th to 16th century. Two main ancestral haplotypes have been identified: the Machado lineage, which is more recent, predominant in families of Portuguese extraction, and the Joseph lineage, which is much older and worldwide spread, postulated to have an Asian origin. We report a Nigerian family with MJD from Calabar, once settled by Portuguese slave traders, and assessed its mutational origin. The proband was a 33-year-old man with progressive unsteady gait, weakness of all limbs, dysphagia, dysarthria, urinary frequency and diaphoresis. He had end-of-gaze nystagmus, spastic quadriparesis and atrophic small muscles of the hand. He showed fibrillation potentials on EMG, and nerve conduction studies suggested a central axonopathy without demyelination. This family bears the Joseph haplotype, which has a founder effect in the island of Flores, in the Azores (and their descendants in North-America), but is also the most common in non-Portuguese populations worldwide, with an estimated mutation age of around 7000 years