Second-dose intravenous thrombolysis with tenecteplase in alteplase-resistant medium-vessel-occlusion strokes: A retrospective and comparative study.

INTRODUCTION: In intracranial medium-vessel occlusions (MeVOs), intravenous thrombolysis (IVT) shows inconsistent effectiveness and endovascular interventions remains unproven. We evaluated a new therapeutic strategy based on a second IVT using tenecteplase for MeVOs without early recanalization post-alteplase. PATIENTS AND METHODS: This retrospective, comparative study included consecutively low bleeding risk MeVO patients treated with alteplase 0.9 mg/kg at two stroke centers. One center used a conventional single-IVT approach; the other applied a dual-IVT strategy, incorporating a 1-h post-alteplase MRI and additional tenecteplase, 0.25 mg/kg, if occlusion persisted. Primary outcomes were 24-h successful recanalization for efficacy and symptomatic intracranial hemorrhage (sICH) for safety. Secondary outcomes included 3-month excellent outcomes (modified Rankin Scale score of 0-1). Comparisons were conducted in the overall cohort and a propensity score-matched subgroup. RESULTS: Among 146 patients in the dual-IVT group, 103 failed to achieve recanalization at 1 h and of these 96 met all eligible criteria and received additional tenecteplase. Successful recanalization at 24 h was higher in the 146 dual-IVT cohort patients than in the 148 single-IVT cohort patients (84% vs 61%, p < 0.0001), with similar sICH rate (3 vs 2, p = 0.68). Dual-IVT strategy was an independent predictor of 24-h successful recanalization (OR, 2.7 [95% CI, 1.52-4.88]; p < 0.001). Dual-IVT cohort patients achieved higher rates of excellent outcome (69% vs 44%, p < 0.0001). Propensity score matching analyses supported all these associations. CONCLUSION: In this retrospective study, a dual-IVT strategy in selected MeVO patients was associated with higher odds of 24-h recanalization, with no safety concerns. However, potential center-level confounding and biases seriously limit these findings' interpretation. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05809921.

Transient recurrent episodes of abducens nerve palsy and cheiro-oral syndrome in a sub-occlusive carotid bulb atherosclerotic stenosis.

We report the case of a male in his 50s with a history of smoking admitted to our hospital for three transient recurrent episodes of less than 60 min of cheiro-oral paresthesias and binocular horizontal diplopia with convergent strabismus. On admission, his neurological examination was normal. Cerebral magnetic resonance imaging showed no cerebral lesion. Computed tomography angiography showed a sub-occlusive right carotid bulb atherosclerotic stenosis, the absence of abnormality of the subclavian arteries and the origin of the vertebral arteries, and no stenosis of the basilar artery or posterior cerebral arteries. Routine blood tests were normal with glycated hemoglobin of 6.5%. The patient underwent right carotid endarterectomy. One year after carotid endarterectomy, the patient has had no other cerebrovascular events.

Detection of moderate to severe middle cerebral artery atherosclerotic stenosis in stroke patients: Transcranial color-coded duplex sonography versus computed tomography angiography.

Background: Intracranial atherosclerotic stenosis is a common cause of ischemic cerebrovascular events and is associated with a high risk of stroke recurrence. This study aimed to assess the diagnostic accuracy of transcranial color-coded duplex sonography for moderate-to-severe middle cerebral artery stenosis in stroke patients. Methods: A retrospective analysis was carried out, including 31 patients aged ⩾18 years hospitalized for ischemic cerebrovascular event in whom middle cerebral artery stenosis ⩾30% was identified on computed tomography angiography. Transcranial color-coded duplex sonography findings were compared to the degree of stenosis blindly identified on the computed tomography angiography used as the reference method. Results: Overall, 27 patients had M1 stenosis and the other 4 had M2 stenosis. To detect M2 stenosis ⩾ 50% and ⩾ 70%, stenotic to pre-stenotic ratio ⩾ 2 and ⩾ 3 had a sensitivity of 100%, respectively. To detect M1 stenosis ⩾ 70%, peak systolic velocity ⩾ 300 cm/s had a sensitivity of 53.8% and specificity of 85.7% with area under the receiver-operating characteristic curve of 0.753 (95% confidence interval: 0.568-0.938; p = 0.026), and stenotic to pre-stenotic ratio ⩾ 3 had a sensitivity of 84.6% and a specificity of 78.6% (area under the curve = 0.854; 95% confidence interval: 0.707-1; p = 0.002). Middle cerebral artery/anterior cerebral artery velocity ratio < 0.7 had a sensitivity of 57.1% and specificity of 90% to detect dampened pre-stenotic flow in middle cerebral artery secondary to downstream M1 stenosis ⩾ 70% (area under the curve = 0.800; 95% confidence interval: 0.584-1; p = 0.040). Conclusion: This study showed that stenotic to pre-stenotic ratio ⩾ 3 was more sensitive than peak systolic velocity ⩾ 300 cm/s to screen M1 stenosis ⩾ 70%. Middle cerebral artery/anterior cerebral artery ratio < 0.7 was a good indirect sign to detect dampened pre-stenotic flow due to M1 stenosis ⩾ 70%.

Assessment of knowledge, attitudes, and practices of occupational risks and diseases among healthcare providers of the Regional Hospital Center of Dosso, Niger.

Background: In Niger, a low-income country, occupational medicine is little known and practiced, particularly in the health sector, where healthcare workers are exposed to numerous occupational risks. This study aimed to assess knowledge, attitudes, and practices about occupational risks and diseases among healthcare providers working at the Hospital of Dosso (Niger) to determine their knowledge, attitudes, and practices level about occupational risks and diseases. Methods: A descriptive and quantitative census-based study was conducted among the 105 healthcare providers practicing at the hospital of Dosso between 1 April and 31 May 2023, using a self-administered questionnaire in the French language comprising a sociodemographic part and a knowledge, attitudes, and practices part about occupational risks and diseases. Knowledge, attitudes, and practices scores were calculated and Mann-Whitney U or Kruskal-Wallis tests were used to assess their association with sociodemographic characteristics. Results: In total, 83 healthcare providers agreed to participate in the study, with a participation rate of 79%. A total of 28% of participants had received training on occupational risks and diseases. The knowledge score about occupational risks and diseases showed that 55.4% of participants had a knowledge level varying from medium to high. The score of attitudes and practices showed that 68.7% of responders had an attitudes and practices level varying from medium to high about occupational risks and diseases. According to the composite score of knowledge, attitudes, and practices, 53% of participants had a knowledge, attitudes, and practices level between medium and high. A significant association was found between the composite score of knowledge, attitudes, and practices and the workstation occupied, mainly medical imaging service (p = 0.006). Conclusion: The study showed that 53% of responders had an overall level of knowledge, attitudes, and practices about occupational risks and diseases between medium and high. Among their sociodemographic characteristics, only the workstation occupied (mainly medical imaging service) was statistically associated with the composite score of knowledge, attitudes, and practices. Considering these findings, the administrators of the Hospital of Dosso must recruit an occupational medicine specialist to provide information and prevention visits on occupational risks and diseases to healthcare providers working in this institution.

Epilepsy in Niger: An overview of the current situation.

A major public health concern in the world, particularly in sub-Saharan African countries, epilepsy is poorly studied in Niger, and its prevalence in the general population is unknown. Only two hospital-based studies have been published in Niger, reporting an estimated mean hospital prevalence of epilepsy of 24.2% (95% CI: 23.2-25.2), mainly affecting patients younger than 18 years (54.6%, 95% CI: 52.2-57.0), and males (59.8%, 95% CI: 57.4-62.2). To date in Niger, people living with epilepsy (PWE) are usually cared for by non-physician healthcare workers (mainly nurse technicians in mental health), general medical doctors (non-specialist physician), and non-neurologist physicians. In routine practice, these health workers make the diagnosis of epilepsy essentially based on the clinic, i.e., the repetition of epileptic seizures over time (mainly generalized tonic-clonic seizures) because most of them practiced in health centers or district hospitals or regional hospital centers in which electroencephalogram and neuroradiological explorations are of limited access or unavailable as well as a referral neurologist. Only 10 neurologists are currently practicing in Niger for a total population estimated at 21466800 inhabitants by the World Health Organization in 2018. In 41.8% (95% CI: 39.4-43.2) of the cases, the etiology was unknown with neuroradiological explorations and laboratory tests not performed in most cases. Genetic etiologies are the most frequent etiologies (40.8; 95% CI: 38.4-43.2), followed by Structural etiologies (11.7; 95% CI: 10.1-13.3) particularly hypoxic-ischemic brain injury, and infectious etiologies (6.8%; 95% CI: 5.6-8.0), especially cerebral malaria. PWEs are the target of social stigma and discrimination due to erroneous cultural and religious beliefs about epilepsy of the people from Niger. The present review is an update on the different aspects of epilepsy in Niger.

Outcomes after Thymectomy in Patients with Thymomatous Myasthenia Gravis.

Objectives This article describes the clinical outcomes after thymectomy in patients with thymomatous myasthenia gravis (T-MG) managed in the department of thoracic surgery of Hassan II University Hospital of Fez, Fez, Morocco. Materials and Methods We performed a retrospective analysis of medical records of 16 patients with T-MG between January 2009 and January 2017. Results There were 11 women and 5 men with a median age of 40 years at the thymectomy time and a median time of onset of symptoms to thymectomy of 12 months. At the preoperative evaluation (Myasthenia Gravis Foundation of America [MGFA] clinical classification), 7 patients were class II, 7 class III, and 2 class IV. Nine patients were in Masaoka stage I, and the remaining 7 patients stage II. We recorded one case of postoperative myasthenic crisis. At 3 years of follow-up after thymectomy, 6 patients had complete stable remission and the other 10 patients improved. Of these patients with clinical improvement, 6 patients were in MGFA class I and the remaining 4 patients class II. Conclusion The present study shows the beneficial effect of thymectomy in patients with T-MG. Postoperative clinical outcomes seem to be better when the preoperative severity of myasthenic symptoms is mild (MGFA class II).

Floating aortic arch thrombus involving the left common carotid artery complicated with ischemic stroke associated with cocaine use.

BACKGROUND: Floating aortic thrombi (FLOAT) are rare, with very few cases attributed to cocaine use. We report a new case of FLOAT involving the left common carotid artery due to cocaine use, for the first time, complicated with acute ischemic stroke. METHODS: We present in detail our case report, and then, a literature search in PubMed and Scopus was performed up to March 20, 2022, to review the reported cases of aortic thrombus associated with cocaine use. RESULTS: A 39-year-old man with a history of smoking and daily cocaine use was admitted to our stroke unit for acute left-hemispheric symptoms due to carotid-Sylvian occlusion. CT angiography of the supra-aortic trunks showed FLOAT involving the left common carotid artery. The thrombus was removed successfully by endovascular thrombectomy with recanalization of carotid-Sylvian occlusion. Our literature search yielded seven reported cases of aortic thrombus due to cocaine use revealed by lower limb ischemia (3 patients), renal infarction (1 patient), abdominal pain (1 patient), bowel ischemia (1 patient), and lower limb ischemia with renal infarction (1 patient). CONCLUSION: Aortic thrombus should be suspected in patients without overt cardiovascular risk factors but with a recent history of cocaine use who presents with acute ischemic stroke.

Unusual neurologic manifestations of Vogt-Koyanagi-Harada disease: a systematic literature review.

BACKGROUND AND PURPOSE: The usual neurologic manifestations of Vogt-Koyanagi-Harada (VKH) disease include aseptic meningitis and headaches. We performed the present study to review all unusual neurologic manifestations reported in VKH disease to summarize them. METHODS: A literature search was performed in the English language on Scopus and Medline via PubMed from 1946 to July 31, 2021, by using the following terms: "Vogt Koyanagi Harada disease" OR "VKH disease" AND "Brain" OR "Spinal cord" OR "CNS" OR "Central nervous system" OR "Neurologic" OR "Peripheral nervous system" OR "Polyneuropathies. Our inclusion criteria were unusual neurologic manifestations of VKH disease. RESULTS: Our literature search yielded 417 total articles (PubMed = 334, Scopus = 83) from which 32 studies comprising 43 patients (22 men and 21 women, of which 62.8% were younger than 50 years) were included in this systematic literature review. Regarding the study design, all studies were case reports and published between 1981 and 2021. CNS involvement was the most reported (93%) in VKH disease. Peripheral nervous system involvement represents 7% of cases. The cerebral lesions were parenchymal inflammatory lesions in the white matter or posterior fossa with or no contrast enhancement (16.3%), leptomeningitis (9.3%), pachymeningitis (7%), meningoencephalitis (2.3%), ischemic stroke (4.6%), hemorrhagic stroke (2.3%), transient ischemic attack (2.3%), and hydrocephalus (2.3%). The optic nerve lesions were anterior ischemic optic neuropathy (20.9%) and optic neuritis (9.3%). Concerning spinal cord lesion, it was mainly myelitis (14%). CONCLUSION: This systematic literature review provides a summary of the different unusual neurologic manifestations reported in VKH disease.

Clinical and etiological profile of epilepsy at the regional hospital center of Tahoua (Niger): A 4-year retrospective study.

OBJECTIVE: We aimed to evaluate epilepsy management at the regional hospital center (RHC) of Tahoua (Niger) to determine the intrahospital deficiencies to optimize to improve the management of people with epilepsy. METHODS: A descriptive retrospective study was carried out at the Psychiatric Unit of the RHC of Tahoua between January 1, 2016 and December 31, 2019. We collected from the registers of consultation all patients followed by nurse technicians in mental health for epilepsy whose diagnosis was made by nonspecialist physicians or internists. The study included patients with epilepsy who had a medical follow-up at least 6 months. RESULTS: Of the 2022 patients seen during the period of the study, 246 patients were consulted for epilepsy with a hospital frequency of 12.2%. The mean age was 22.38 years, with a slight predominance of men (57.7%). Only generalized tonic-clonic seizures (95.1%) and focal-aware seizures (4.9%) were reported. The main etiologies were cerebral malaria (18.7%), bacterial meningitis (8.1%), and head trauma (4.9%). In 60.2% of cases, the etiology was undetermined, but the etiological diagnosis investigation was incomplete (CT scan not done). Only the first-generation antiepileptic drugs were used. Only 2.8% of the patients had drug-resistant epilepsy, and 97.2% of the patients had controlled seizures. CONCLUSION: The study shows a predominance of infectious causes, particularly cerebral malaria. We found a high proportion of epilepsies with unknown etiologies with incomplete workup. The RHC of Tahoua should facilitate access to the CT scan for people with epilepsy to improve etiological diagnosis investigation.

Isolated cerebellar nodulus infarction: Two case reports and literature review.

BACKGROUND: Isolated cerebellar nodulus infarction (ICNI) is rare and has great clinical similarity with acute peripheral vestibulopathy (APV), from which it is difficult to distinguish. We report two cases of ICNI followed by a literature review to identify the discriminant clinical elements that differentiate ICNI from APV. METHODS: We describe in detail our 2 cases. Besides, a literature search in Medline via PubMed and Scopus was performed up to May 17, 2020. Clinical characteristics, mainly of well-described cases, were extracted and analyzed. RESULTS: Our search yielded 43 total publications, among which 13 were selected, including 23 patients. Spontaneous or positional rotatory vertigo with unidirectional spontaneous horizontal nystagmus, associated with the postural imbalance and unilateral lateropulsion or fall on Romberg's test, was the most common clinical picture. According to our literature review, the discriminant clinical elements which differentiate ICNI from APV were direction-changing gaze-evoked nystagmus, bilateral lateropulsion or fall on Romberg's test, and normal horizontal head impulse test. Our two patients reported a positional fleeting abnormal visual perception of spatial orientation of objects. We proposed this symptom as a discriminant clinical element. CONCLUSION: The ICNI constitutes a difficult differential diagnosis of APV. Through our two patients reported here, we proposed a supplementary discriminant symptom helpful for the clinical diagnosis.

Teachers' Knowledge About Epilepsy and Their Attitudes Toward Students with Epilepsy: A Cross-Sectional Survey in the City of Tahoua (Niger).

PURPOSE: In this survey, first, we aimed to investigate the views held about epilepsy of primary and secondary school teachers in the city of Tahoua (Niger, a Sub-Saharan African country), and secondarily, how their experience and knowledge about epilepsy influence their attitudes towards school children with epilepsy. MATERIALS AND METHODS: A cross-sectional survey was carried out between November and December 2019. We used a self-administered questionnaire survey in French-language comprising sociodemographic part, and knowledge and attitudes about epilepsy part. Composite scores of knowledge and attitudes were calculated, and statistical analyses were performed to assess their association with sociodemographic characteristics. RESULTS: Two hundred eighty-four (284) school teachers had agreed to answer the questionnaire survey, among which 25% had already witnessed an epileptic seizure in the classroom or the schoolyard. Epilepsy is considered as a chronic brain disease and psychiatric illness in 31% and 32.4% of cases, respectively. In 41.9% of cases, epilepsy is considered as a contagious disease. Significantly, we found that the composite score of knowledge and attitudes was lower in younger school teachers (p = 0.007) and those with fewer years of teaching experience (p = 0.048). High school teachers had better score composite than those of primary school and college (p = 0.072). Regarding the composite score of attitudes, we did not find an association with sociodemographic characteristics. CONCLUSION: Teachers' attitudes were not associated with their level of school taught, years of teaching experience, and their religion. It is necessary to integrate educational training courses about epilepsy into the curriculum of teachers' training.

Knowledge, Attitudes, and Practices with Respect to Epilepsy among Nurses in the City of Niamey, Niger.

Objective The aim of this study is to evaluate knowledge, attitudes, and practices about epilepsy among nurses in the city of Niamey, Niger, to identify existing gaps in their knowledge concerning epilepsy to formulate recommendations to improve the quality of care for people with epilepsy. Materials and Methods We conducted a descriptive cross-sectional survey on epilepsy knowledge among nurses in the city of Niamey, using a self-administered questionnaire including questions related to knowledge about epilepsy. Results A total of 98 nurses (29 men and 69 women) were included in the survey. The mean age of the participants was of 32.88 ± 10.87 years (range: 17-58 years). The sample consisted of 10.2% of student nurses, 64.3% of college patent, and 25.5% of bachelor nurses. Only 22.4% of participants reported having heard about epilepsy during their training programs. Among the respondents, 43.9% of them had already attended at least one epileptic seizure. Epilepsy is considered as a psychiatric illness and contagious disease by 11.2 and 19.4% of respondents, respectively. The main transmission routes reported were physical contact with an epileptic person (14.3%) and contact with saliva (4.1%). Epilepsy was considered as an incurable disease by 5.1% of respondents. When someone has an epileptic seizure, 10.2% of respondents think that physical contact with this person should be avoided to prevent from contamination. Only 6.1% of respondents had appropriate attitudes when someone has an epileptic seizure such as lying the patient on his side, clearing the airway if necessary, and diazepam injection. Conclusion Considering that people with epilepsy are largely cared out by nurses in Niger, the results of the study justify the need to train nurses with respect to epilepsy to improve their knowledge about epilepsy and the care of people with epilepsy.

Knowledge, attitudes, and practices with respect to epilepsy among primary and secondary school teachers in the city of Niamey, Niger.

OBJECTIVE: We aimed firstly to evaluate knowledge, attitudes, and practices about epilepsy among primary and secondary school teachers in the city of Niamey and secondly to formulate targeted sensitization messages for these teachers. MATERIALS AND METHODS: A descriptive cross-sectional survey was carried out among primary and secondary school teachers in the city of Niamey, capital of Niger, using a self-administered questionnaire including questions related to knowledge, attitudes, and practices about epilepsy. RESULTS: One hundred and forty-five (145) teachers aged 27-69 (mean age: 39.57 ± 8.304 years) had answered the questionnaire (52 men and 93 women). In 62.1% of cases, respondents had a bachelor degree in education signifying a high level of education. Sixty-one respondents (42.1%) considered epilepsy as a brain disease, whereas it was considered as a psychiatric illness by 15.9% of respondents and as an impurity by 38.9%. Sixteen respondents (11%) considered it as a hereditary disease. Epilepsy was considered as a contagious disease by 46.2% of teachers, and the main transmission routes reported were contact with places of crisis (26.2%), physical contact with an epileptic person (16.6%), and contact with saliva (6.9%). It was considered as an incurable disease by 6.9% of teachers. Of the 115 respondents (79.3%) who considered epilepsy as a treatable condition, 46 of them believe that epilepsy is treated by traditional medicine. When someone has an epileptic seizure, 28.3% of respondents think that physical contact with him should be avoided and that the places of the crisis should be avoided. CONCLUSION: Considering that epilepsy is common in school-age, the study results suggest the need to train teachers with respect to epilepsy to change misconceptions about epilepsy and to promote positive attitudes toward epileptic people to avoid the rejection of these people by the society responsible for problems of social integration.

Clinical and Etiological Characteristics of Epilepsy in the Elderly: A Hospital-Based Study from a Tertiary Care Referral Center of Niamey, Niger.

Objectives The aim of this study is to evaluate the management of epilepsy in the elderly at a tertiary referral center in Niger to obtain a comprehensive understanding to determine the intrahospital deficiencies to improve and to make recommendations in terms to improve the management of epilepsy in the elderly in Niger. Materials and Methods We conducted a retrospective study at the Neurology Outpatient Clinic of the National Hospital of Niamey (Niger) over a period of 5 years from May 2013 to May 2018, collecting all cases of patients aged 60 years or over diagnosed with epilepsy by neurologists. From the registers of consultation, we collected and analyzed for each patient the demographic, clinical, etiological, and therapeutic data, as well as the outcomes during follow-up visits. Results Of the 4,576 patients of all ages seen during the period of our study, we included 62 patients aged 60 years or over diagnosed with epilepsy with a hospital frequency of 1.35%. The mean age of patients was 65.82 ± 5.72 years (range: 60 and 83 years) with a predominance of the male sex (sex ratio at 1.6). Patients aged 60 to 64 years were the most represented (43.5%). Generalized tonic-clonic seizures were the most frequent (41.9%), followed by focal to bilateral tonic-clonic seizures (25.8%). All patients underwent electroencephalogram. Only 30 patients (48.4%) underwent brain imaging, and mainly brain computed tomography scan. The etiologies included poststroke epilepsy (25.8%), brain tumors (3.2%), cerebral toxoplasmosis (3.2%), and cerebral meningioma (1.6%). We found 41 cases (66.1%) of epilepsy without definite etiology and with an incomplete workup. Carbamazepine and phenobarbital were the only two antiepileptic drugs (AEDs) used. Conclusion The present study shows limited access to newer generation AEDs and diagnostic tests of epilepsy in Niger. Considerable efforts should be made to facilitate for people living with epilepsy the accessibility to diagnostic tests and the newer generation AEDs to improve the quality of epilepsy management in Niger.

Clinical and etiological characteristics of epilepsy in people from Niger: a hospital-based study from a tertiary care referral center of Niamey, Niger.

OBJECTIVES: Epilepsy constitutes a major public health concern in the world particularly in developing countries, especially in sub-Saharan African countries. We designed this study to evaluate epilepsy management at a tertiary referral center in Niger to obtain a comprehensive understanding to determine the intrahospital deficiencies to improve and to make recommendations in terms to improve epilepsy management in Niger. METHODS: We conducted a retrospective study at the Neurology Outpatient Clinic of the National Hospital of Niamey (Niger) between May 2013 and May 2018 (5 years), collecting all cases of patients diagnosed with epilepsy by the neurologists. From the registers of consultation, we collected for each patient the demographic, clinical, etiological, and therapeutic data, as well as the outcomes during follow-up visits. RESULTS: Of the 4576 patients seen during the period of the study, 1350 patients consulted for epilepsy with a hospital frequency of 29.5%. The mean age of the patients was 18.55 ± 17.15 years (range: 3 months to 83 years) with a predominance of the male sex (sex ratio at 1.5). Patients younger than 20 years were the most represented (61.6%). All patients underwent EEG. Only 463 patients (35.2%) underwent brain imaging. Generalized tonic-clonic seizures were the most frequent (50%) followed by typical absences seizures (11.8%). Cerebrovascular disease, central nervous system infections, and head injuries were the main etiologies. First-generation AEDs were the most prescribed (99%). The proportion of patients with drug-resistance was 9.6%. SIGNIFICANCE: Our study shows limited access to newer generation AEDs and diagnostic tests of epilepsy in Niger. Considerable efforts should be made to facilitate for people living with epilepsy the accessibility to diagnostic tests and newer generation AEDs in order to improve the quality of epilepsy management in Niger.

Hurler-Scheie syndrome in Niger: a case series.

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.

Hemiconvulsion-hemiplegia-epilepsy syndrome in Niger: A retrospective case series.

OBJECTIVE: To report the demographic, clinical and paraclinical characteristics as well as the outcomes during follow-up visits of HHE syndrome in Niger. PATIENTS AND METHODS: It was a retrospective study conducted at the Neurology Outpatient Clinic of the National Hospital of Niamey (Niger) between May 2003 and May 2018. RESULTS: During the period of study, 882 patients with epilepsy aged 20 years or less were seen in consultation among whom we collected 22 cases of HHE syndrome with a hospital prevalence of 2.5%. They were 14 men and 8 women (sex ratio = 1.75) with a mean age of 3.76 years (range: 1 and 20 years). At the time of diagnosis, 81.8% of patients were aged under 6 years. The antecedent of febrile convulsions in childhood was found in only 31.8% of cases. The mean age of onset of hemiplegia was 1.9 years (range: 9 months and 5 years). The mean age of onset of epileptic seizures was 2.94 years (range: 1 and 8 years). The mean interval between the onset of hemiplegia and onset of epileptic seizures was 9 months (range: 1 month and 4 years). 31.8% of patients had a delay of acquisitions at the time of diagnosis. 95.5% of patients had drug-resistant epilepsy. CONCLUSION: HHE syndrome is still seen in the countries of Sub-Saharan Africa. The high morbidity and mortality in children with HHE syndrome highlight the need to improve emergency care for febrile clonic seizures in childhood and the early and adequate management of infectious diseases in the child.

Limbic encephalitis: Experience of a moroccan center.

OBJECTIVES: Histologically defined as an inflammation-degeneration of limbic structures, limbic encephalitis (LE) is a rare disease and often difficult to diagnose particularly in institutions with limited access to laboratory tests such as antineuronal antibodies or HSV-PCR, and functional imaging. We aimed to describe the demographic, clinical, paraclinical, and etiological features of LE, as well as its medium-term prognosis in Moroccan patients. MATERIALS AND METHODS: We collected retrospectively all patients diagnosed with LE in the Department of Neurology of the University Hospital Hassan II of Fez (Morocco) between September 2008 and December 2016. We analyzed their demographic features, clinical manifestations, magnetic resonance imaging and laboratory findings, etiologies, and medium-term prognoses. RESULTS: We included 22 men and 9 women aged 14-76 years (mean age: 45.8 years). In 64.5% of cases, the onset of symptoms was acute. The clinical manifestations included generalized status epilepticus (16.1%), confusional syndrome (29%), epileptic seizures (38.7%), psychiatric disorders (48.4%), and memory disorders (45.2%). The nonlimbic symptoms were nuchal stiffness (22.6%), headaches (9.7%), fever (61.3%), vesicular rash (3.2%), and language disorders (6.5%). The different etiologies found were herpes simplex virus (6.5%), syphilis (16.1%), tuberculosis (3.2%), varicella (3.2%), paraneoplastic autoimmune LE (22.6%), anti-NMDA-R LE (6.5%), and sarcoidosis (3.2%). We found 12 cases (38.7%) of LE without definite etiology and with an incomplete diagnostic workup. The medium-term clinical course includes a complete remission in 45.2% of cases and partial remission in 45.1% of cases. The different sequelae were temporal lobe epilepsy (9.7%), anterograde amnesia (16.1%), and severe cognitive impairment (19.4%). The mortality rate was 9.7% (3 patients). CONCLUSION: Our study shows a wide diversity of etiologies of LE in Morocco with essentially an acute mode of onset of symptoms.

Thrombolysis Alert in Hassan II University Teaching Hospital of Fez (Morocco): A Prospective Study of 2 Years.

BACKGROUND: Thrombolysis alert (TA) is a procedure triggered every time a patient consults for sudden focal neurological deficit within 4.5 hours. OBJECTIVE: We aimed to determine firstly the etiological profile of TA and secondly to evaluate the delays in the management not only of thrombolyzed patients but also of nonthrombolyzed patients to determine the intrahospital delays to optimize. METHODS: Patients aged over 18 years who consulted for sudden focal neurological deficit within 4.5 hours for whom a TA has been triggered were included. Patients admitted within 4.5 hours for which a TA has not been triggered were not included. Patients with sudden focal neurological deficit who consulted more than 4.5 hours, but for whom TA has been triggered, were also included. RESULTS: We included 313 patients. The average onset-to-door time was 125.59 ± 62.78 minutes with an average National Institutes of Health Stroke Scale scores of 11.29 ± 5.98. The average door-to-imaging time was 28.36 ± 20.62 minutes. Ischemic stroke (IS) was the most common cause (70.3%), followed by hemorrhagic stroke (11.8%). Other nonstroke causes (stroke mimics) represented 17.9% of cases. They were seizures (46.4%), conversion disorders (26.8%), hypoglycemia (10.7%), brain tumors (10.7%), chronic subdural hematoma (1.8%), carbon monoxide intoxication (1.8%), and cavernoma (1.8%). Forty-six patients had been thrombolyzed. The average door-to-needle time was 90.89 ± 34.48 minutes. After 3 months, 52.1% of thrombolyzed patients were autonomous (modified Rankin scale between 0 and 2). Two patients had died (4.3%), all in the first week after the IS. CONCLUSION: Our study shows that efforts need to be made at the extra-hospital and intra-hospital level to improve delays to increase the proportion of the thrombolyzed patients.