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Midline essential tremor (Mid-ET) is a distinctive group of essential tremor (ET) in which tremor affects the neck, jaw, tongue, and/or voice. For long, it has been considered as an ultimate stage of the disease and a marker of its severity. However, recent studies pointed its complexity in terms of non-motor presentation. Thus, we aimed to investigate the non-motor signs (NMS) in Mid-ET.We conducted a cross-sectional study in a tertiary neurology referral center including ET patients classified into two groups based on the presence or not of midline tremor (Mid-ET vs. No-Mid-ET). We assessed NMS using the non-motor severity scale (NMSS), a large battery of cognitive tests, clinical and electrophysiological study of the autonomic nervous system along with the evaluation of sleep disturbances.A total of 163 patients were included: Mid-ET (n = 79) and No-Mid-ET (n = 84) matched in gender and age of onset. Mid-ET patients had higher proportion of late-onset ET ( 60 years old, p = 0.002) and more extrapyramidal signs (p = 0.005). For NMS, Mid-ET was marked with cognitive dysfunction (p = 0.008). The hallmarks of the neuropsychiatric profile of Mid-ET were executive dysfunction (p = 0.004), attention problems (p 0.000), episodic memory impairment (p = 0.003), and greater depression (p = 0.010). The presence of RBD was a trait of Mid-ET (p = 0.039). In both Mid-ET and No-Mid-ET phenotypes, clinical and neurophysiological dysautonomia correlated with cognitive dysfunction.Mid-ET patients had greater cognitive dysfunction, depression, RBD, higher proportion of late-onset ET, and more extrapyramidal signs. Taken all together, these findings could provide a redesigned insight into the underlying physiopathology of Mid-ET indicative of a greater cerebellar dysfunction.

Few studies have reported abnormal ocular movements in cases of amyotrophic lateral sclerosis (ALS) and their link with other disease features. Our study aimed to describe and analyse eye movement abnormalities in ALS patients. Specifically, we set out to investigate the correlation between non-motor signs and oculomotor impairment in order to understand the pathogenesis of the disease. All ALS patients seen from 2018 to 2020 in the department of Neurology of Razi hospital underwent the recording of saccadic eye movements. Results were compared with healthy controls. Sixty-two patients were included. Altered saccadic eye movements (72.6%) correlated with tongue atrophy and bladder dysfunction. The most common finding was altered smooth pursuit (56.5%), which showed correlation with bladder dysfunction and altered frontal assessment battery (FAB) scores. Prolonged latencies of horizontal saccades (34%) correlated with sensory and extrapyramidal signs. Our study is the first to examine the characteristics of eye movements in a large African cohort of ALS patients and to show correlations with extra-motor clinical signs. Our findings showed extra-motor cortex dysfunction in ALS with greater frequency of eye movement abnormalities in comparison with previous studies. Altered horizontal pursuit, the core abnormality, confirmed the extension of the neurodegenerative process to the frontal and prefrontal cortices. Prolonged horizontal saccade latencies reflect mainly the involvement of the parietal eye field. Anti-saccadic abnormalities were the least common finding and showed, paradoxically, no link with executive dysfunction.

Unverricht-Lundborg disease (ULD), also called progressive myoclonic epilepsy type 1, is usually characterized by the presence of ataxia associated with myoclonus and epileptic seizures without progressive cognitive deficit, presenting during late childhood and early adolescence. Currently, there is a growing body of evidence for atypical presentations of the disease with a milder phenotype or without the full symptomatology. We describe a case report of a late-onset phenotype with progressive myoclonus-ataxia syndrome accompanied by initial recurrent falls, resulting in specific phobia and agoraphobia starting at the age of 50 years old. The examination revealed multifocal myoclonus with cerebellar ataxia and electroencephalogram showed generalized polyspikes and spike-wave discharges. Electromyogram revealed positive myoclonus of 60-ms duration in the face and the presence of C reflex. A genetic study confirmed the diagnosis of ULD in the patient and other additional family members, presenting a wide range of intra-familial variability. We discuss the challenging differential diagnosis for such a misleading presentation and its possible underlying pathophysiological mechanisms. Our case report may contribute to broadening the age and clinical boundaries for this disease and emphasizes the intra-familial age and symptom variability. Based on a suggestive family history, the diagnosis of ULD should be considered in this context, even in older patients.

Background and ObjectivesPatients with pediatric-onset multiple sclerosis (POMS) typically experience higher levels of inflammation with more frequent relapses and reach irreversible disability at a younger age than adult-onset patients. There have been few randomized placebo-controlled clinical trials of multiple sclerosis (MS) disease-modifying therapies (DMTs) in patients with POMS, and most available data are based on observational studies of off-label use of DMTs approved for adults. We assessed the effectiveness of natalizumab compared with fingolimod using injectable platform therapies as a reference in pediatric patients in the global MSBase registry.MethodsThis retrospective study included patients with POMS who initiated treatment with an injectable DMT, natalizumab, or fingolimod between January 1, 2006, and May 3, 2021 (N=1218). The primary outcome was the time to first relapse from index therapy initiation. Secondary study outcomes included annualized relapse rate; proportions of relapse-free patients at 1, 2, and 5 years post baseline; time to treatment discontinuation; and times to 24-week confirmed disability worsening and confirmed disability improvement.ResultsPatients treated with fingolimod had a significantly lower risk of relapse than patients treated with injectable DMT (hazard ratio [HR], 0.49; 95% confidence interval [CI], 0.29–0.83;P=0.008). After adjustment for prior DMT experience in the unmatched sample, patients treated with natalizumab had a significantly lower risk of relapse than patients treated either with injectable DMT (HR, 0.15; 95% CI, 0.07–0.31;PP=0.049). The adjusted secondary study outcomes were generally consistent with the primary outcome or with previous observations. The findings in the inverse probability treatment weighting–adjusted patient populations were confirmed in multiple sensitivity analyses.DiscussionOur results suggest that natalizumab and fingolimod have broadly equivalent therapeutic efficacies in patients with POMS, consistent with previous studies of natalizumab and fingolimod in adult-onset patients and POMS. However, analyses of relapse outcomes suggest natalizumab is superior to fingolimod in the control of relapses in this population with high rates of new inflammatory activity.Classification of EvidenceThis study provides Class III evidence that natalizumab may provide better disease control than fingolimod in patients with POMS.

Summary Peduncular hallucinosis (PH) is a rare neurological disorder, characterized by visual hallucinations, and often associated with sleep and oculomotor disturbances. It is typically due to thalamic and brainstem lesions. We report here the case of a 69-year-old patient, with personal history of depression, diabetes and hypertension, presenting with complex auditory and visual hallucinations associated with insomnia but without oculomotor abnormalities. Brain MRI revealed isolated bilateral symmetric paramedian upper pontine ischemic lesions, which are likely to be the cause of this PH. This case could expand the etiological spectrum of PH, adding bipontine infarction to the list of lesions responsible for this rare condition.

BackgroundOver the decades, several natural history studies on patients with primary (PPMS) or secondary progressive multiple sclerosis (SPMS) were reported from international registries. In PPMS, a consistent heterogeneity on long-term disability trajectories was demonstrated. The aim of this study was to identify subgroups of patients with SPMS with similar longitudinal trajectories of disability over time.MethodsAll patients with MS collected within Big MS registries who received an SPMS diagnosis from physicians (cohort 1) or satisfied the Lorscheider criteria (cohort 2) were considered. Longitudinal Expanded Disability Status Scale (EDSS) scores were modelled by a latent class growth analysis (LCGA), using a non-linear function of time from the first EDSS visit in the range 3–4.ResultsA total of 3613 patients with SPMS were included in the cohort 1. LCGA detected three different subgroups of patients with a mild (n=1297; 35.9%), a moderate (n=1936; 53.6%) and a severe (n=380; 10.5%) disability trajectory. Median time to EDSS 6 was 12.1, 5.0 and 1.7 years, for the three groups, respectively; the probability to reach EDSS 6 at 8 years was 14.4%, 78.4% and 98.3%, respectively. Similar results were found among 7613 patients satisfying the Lorscheider criteria.ConclusionsContrary to previous interpretations, patients with SPMS progress at greatly different rates. Our identification of distinct trajectories can guide better patient selection in future phase 3 SPMS clinical trials. Additionally, distinct trajectories could reflect heterogeneous pathological mechanisms of progression.

We describe a Tunisian family carrier of the same rare mutation iniTARDBP/ibut developing different neurodegenerative disease with heterogenous features. We explored the possible genetic modifiers leading to the observed intrafamilial phenotypic variability. Genetic analysis identifiediTARDBP/ip.G294A mutation among4 members. Additionally, the ALS case was muted iniGBA/i. While the three cases of AD were carriers ofiPRKN/iandiGBA/imutations. Finally, the FTD-parkinsonism patient was mutated foriLRRK2/ip.G2019S that might increase his susceptibility to develop Parkinsonism spectrum. Genetic variants ofiTARDBP/imay influence the clinical manifestation in ALS case.

Multiple sclerosis (MS) is a chronic inflammatory disease leading to multifocal neuronal demyelination and axonal damage in the central nervous system (CNS). MS symptoms vary widely but typically do not include headaches. A large spectrum of headaches manifestations was reported as comorbidities in MS and results in additional disability. Migraine, tension-type headache and cluster headache are the most frequently reported primary headache syndromes in patients with MS (pwMS). Secondary causes of headache should be excluded (cerebral vein thrombosis, CNS or systemic infection, cervical and/or cranial trauma, headaches associated with psychiatric disorders, medication overuse headache, etc.) in this particular population. A careful medical history and general and neurological examinations and sometimes further investigations may be needed to rule out secondary headache syndromes. In pwMS, the headache could be an adverse effect of the disease-modifying therapies or a complication of pain medication overuse prescribed to relieve other causes of pain related to MS (neuropathic pain, mechanical pain, pain associated with spasticity, etc.). Migraine-type headache occurs in pwMS more frequently than in the general population. It can precede the disease onset, be associated with relapses, or appear during the MS course. A predominance of brainstem inflammatory lesions is described on magnetic resonance imaging (MRI) in MS patients with migraine. The relationship between both conditions remains unclear. Migraine and MS occur in the same demographic groups with similar background factors, including gender, hormonal status, and psychological features (anxiety, depression, stress). An early diagnosis and adequate treatment of migraine in MS patients are important to improve their quality of life. In this review, we focus on the relationship between MS and Migraine, discuss the differential diagnoses of migraine in pwMS, and describe its management in this particular context.

Background A wide range of neurological manifestations has been described in COVID-19. Methods In this nationwide retrospective observational study, patients in Tunisia diagnosed with COVID-19 between the 2nd of March and the 16th of May 2020 were contacted by telephone. We collected demographic and clinical data and specified characteristics and evolution of main neurological symptoms. Results Of 1034 confirmed COVID-19 patients, 646 were included (mean age 42.17 years old) and 466 (72.1%) had neurological symptoms. Neurological symptoms were isolated 22.7% (n = 106). Headache was the most frequent neurological symptom (n = 279, 41.1%): mainly frontotemporal (n = 143, 51.1%) and mild or moderate (n = 165, 59.1%). When associated with fever (n = 143, 51.3%), headache was more likely to be severe and present at onset. Recovery was reported in 83.2%. Smell and taste impairment were found in 37.9% (n = 245) and 36.8% (n = 238) respectively. Among them, 65.3% (156/239) were anosmic and 63.2% (146/231) were ageusic. A complete improvement was found in 72.1% (174/240) of smell impairment and in 76.8% (179/233) of taste impairment. Myalgia (n = 241, 37.3%) and sleep disturbances (n = 241, 37.3%) were also frequent. Imported cases had more neurological symptoms (p = 0.001). In 14.5%, neurological symptoms preceded the respiratory signs (RS). RS were associated with more frequent (p = 0.006) and numerous (p < 0.001) neurological symptoms. Conclusions Neurological symptoms in COVID-19 are frequent, can be isolated and present at onset. A total recovery is the most recorded outcome. RS are predictive of neurological symptoms. Studies in to virus and host genetics should be considered to understand the different phenotypes.

Resume La sclerose en plaques est une maladie inflammatoire chronique du systeme nerveux central d’etiologie auto-immune. Son phenotype et son evolution peuvent differer d’un patient a l’autre. L’epidemiologie et la presentation de la sclerose en plaques ont connu des modifications remarquables au cours des dernieres decennies notamment dans les pays du Maghreb. Une augmentation de la prevalence et de l’incidence surtout chez la femme a ete notee. Une severite particuliere de la maladie a ete decrite chez les patients d’origine maghrebine par rapport aux Caucasiens. Dans des etudes recentes, la progression du handicap, comparee entre les patients vivant en Afrique du Nord, les immigrants nord-africains et les Caucasiens, etait plus severe chez les patients d’origine nord-africaine. En outre, la severite de la maladie chez les Nord-Africains vivant en France, en particulier chez ceux de la deuxieme generation, a ete demontree. En effet, l’evolution de la maladie serait due a une interaction complexe entre les facteurs genetiques et environnementaux. Au cours des dix dernieres annees, les chercheurs ont valide de nombreux facteurs genetiques et expositions environnementales qui augmentent la susceptibilite a la sclerose en plaques. L’allele HLA-DRB1 * 15/01 a ete associe a un risque plus important de developper la maladie. A la predisposition genetique, s’ajoutent les facteurs environnementaux. L’infection par le virus de l’Epstein-Barr, le tabagisme, et l’alimentation etaient associes au phenotype de la maladie. Ainsi, Son heterogeneite clinique pourrait resulter d’interactions gene-environnement. Des etudes supplementaires sont necessaires pour evaluer l’effet des facteurs environnementaux et genetiques sur la sclerose en plaques aux pays du Maghreb, afin d’etablir les mecanismes en cause et etudier les strategies preventives sous nos latitudes.

Painful ophthalmoplegia is a common presenting symptom in neuro-ophthalmology emergencies. We report an unusual case of a recurrent painful ophthalmoplegia due to a third nerve schwannoma mimicking « ophthalmoplegic migraine ». A 18 year-old girl had presented 4 episodes of left eye painful ophthalmoplegia respectively in 8, 13, 16 and 17 years old. One year after the last episode, neurological examination was normal. Brain MRI focused on the oculomotor nerve showed an enhancing nodular lesion suggesting a third nerve schwannoma. Thus, recurrent painful ophthalmoplegia revealing oculomotor nerve schwannoma, as described in our case, is exceptional. To our knowledge, only thirteen cases have been reported in the literature. Third nerve schwannoma is a rare cranial nerve tumor, typically revealed by progressive palsy of the oculomotor nerve. Recurrent painful ophthalmoplegia with persistent headache and enhancement in brain imaging should suggest tumoral lesions.

Aims The aims of this study were to analyze retinal and choroidal changes on optical coherence tomography (OCT) and OCT-Angiography (OCT-A) in Alzheimer's disease (AD) patients and compare them to other forms of major dementia. We also aimed to analyze the correlation between clinical severity of global cognitive deficiency assessed by the mini-mental state exam (MMSE) score and OCT/OCT-A parameters. Methods Retrospective cross-sectional evaluative study of AD, and age-and gender-matched patients with other dementias. Fundus examination, OCT and OCT-A were compared. Results Ninety-one eyes of AD patients and 53 eyes of patients with other dementias were included. Retinal deposits were found in 6.59% of AD cases. OCT highlighted the presence of hyperreflective deposits and localized areas of outer retina and ellipsoid zone disruption, respectively in 20.87% and 15.38% of AD cases. Hyperreflective foci were noted within inner retinal layers in 4.39% of AD cases. Quantitative analysis revealed a thicker nasal retinal nerve fiber layer (p = 0.001) and ganglion cell complex in superior (p = 0.011) and temporal quadrants (p = 0.009) in eyes of AD patients, compared to other dementias. OCT-A showed a significantly higher fractal dimension of both superficial and deep capillary plexus (p = 0.005), with lower choriocapillaris density (p = 0.003) in AD patients. Conclusions Structural OCT could highlight the presence of hyperreflective deposits in AD, probably reflecting beta-amyloid deposits, associated to outer retinal disruptions. Quantitative OCT analysis showed structural differences between AD patients and other dementias, and combined OCT-A could identify microvascular changes in AD patients representing new potential differential diagnosis criteria.

The clinical spectrum of stiff-person syndrome (SPS) encompasses a wide range of signs including psychiatric symptoms (PS).Our objective was to provide an overview of the spectrum of PS in SPS through a systematic literature search and 2 illustrative case reports.We reported 2 anti-glutamic acid decarboxylase-positive SPS cases that presented with phobic disorder, and we performed a systematic review by following the 2020 Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Articles published in PubMed, MEDLINE on Ovid, Embase, and via a manual search before October 20, 2020, were selected by 2 independent reviewers. Original studies, case reports, editorials, commentaries, and letters to the editor reporting cases of SPS with PS were all included. Conference abstracts, reviews and book chapters, unavailable articles, and those not reporting SPS cases or PS were excluded. Quantitative summary data were calculated.In addition to our 2 cases, we identified 237 cases of SPS with PS from 74 additional included publications totaling 239 patients. Anxiety (56%) and depression (45%) were the most common PS in SPS. Mean diagnostic delay was 4.7 years. Among the 3 SPS phenotypes, the classic form was predominant (77%), followed by stiff-limb syndrome (13%) and progressive encephalomyelitis with rigidity and myoclonus (10%). The most frequent etiology of SPS with PS was autoimmune (90%), followed by cryptogenic (7%) and paraneoplastic forms (7%). These patients were mainly treated with immune-mediated therapies and GABAergic drugs.Our review revealed that the most common PS of SPS are anxiety and depression occurring mostly in autoimmune and classic forms, allowing a clearer understanding of this entity, which may lead to earlier diagnosis and better outcome.

Atypical Parkinsonian syndromes (APS) encompass a spectrum of neurodegenerative diseases including dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal syndrome (CBS). The effects of the Apolipoprotein E (APOE) gene on APS clinical features are controversial and understudied in several populations. We aimed to explore the influence of APOE genotype on clinical features in an APS Tunisian cohort.We included clinically diagnosed APS patients genotyped for APOE, and analyzed the clinical and APOE genotype associations.A total of 328 APS patients were included, comprising 184 DLB, 58 PSP, 49 MSA, and 37 CBS. Significant differences in initial Mini-Mental State Examination and Frontal Assessment Battery scores according to APOE genotypes (P=0.05 and 0.0048) were found. Executive dysfunction (P=0.026) disorientation (P=0.025), and hallucinations (P0.001) were more pronounced among APOE-ɛ4 carriers particularly in DLB. Memory disorders were also correlated to APOE-ɛ4 allele (P=0.048) and were more frequent among DLB and PSP carriers. Depression was associated to APOE-ε4 (P=0.042), more markedly in APOE-ε4-CBS and MSA carriers.Our findings suggested a role of APOE-ε4 in defining a more altered cognitive phenotype with variable degrees across subgroups in APS patients, especially in DLB carriers. This effect mainly concerned executive, memory and orientation functions as well as hallucinations.

Background. Amyotrophic lateral sclerosis (ALS) is a fatal disease whose diagnosis and early management can improve survival. The most used diagnostic criteria are the revised El Escorial criteria (rEEC) and Awaji criteria (AC). The comparison of their sensitivities showed contradictory results. Our study aimed to compare the sensitivities of these two criteria in the diagnosis of definite ALS, at first visit, in a Tunisian hospital cohort. Materials and Methods. This was a retrospective study including 173 patients diagnosed with ALS at the Department of Neurology of the Razi Hospital between January 2003 and April 2018.After studying the clinical features of the disease in our study population,each patient was categorized according to the rEEC and AC based on data collected in his medical record during his first visit to our department. Then, we compared the sensitivities of these two criteria in the diagnosis of definite ALS. Results. Our Tunisian cohort was characterized by a slower disease progression. The sensitivity of the AC (69.4%) was significantly higher than that of the rEEC (40.5%) ( p p p = 0.063). It was impossible to categorize seventeen patients by the two criteria. Conclusion. Our study demonstrated that patients in AC are more sensitive than rEEC in the early diagnosis of ALS in our Tunisian cohort. However, this superiority is gradually reduced during the evolution of the disease.

Introduction Data on epidemiology of atypical parkinsonian syndromes (APS) in North African countries are limited. Our objective was to study the epidemiological features of APS in a Tunisian population. Methods We conducted a 17‐year retrospective cross‐sectional descriptive study in the Department of Neurology at Razi University Hospital. We included all patients responding to consensus diagnosis criteria of APS. We recorded demographic and clinical data. Group differences were assessed with a post hoc ANOVA with a Bonferroni error correction. Results We included 464 APS patients. Hospital prevalence of APS among all parkinsonism cases was 20.6%. Mean annual increase of incidence defined as newly diagnosed APS cases per year reached 38.8%/year. APS were divided into 4 etiological subgroups: dementia with Lewy bodies (DLB; 56.7%); progressive supranuclear palsy(PSP; 16.2%); multiple system atrophy (MSA; 14.6%); and finally corticobasal syndrome (CBS; 12.5%). Sex‐ratio was 1.2. This male predominance was found in all subgroups except MSA (p = .013). Mean age at onset was 68.5 years, most belated in DLB (69.7 years; p 70 years) in 50.7% of patients and was significantly associated with DLB subgroup (p = .013). Inaugural parkinsonism was associated with CBS and MSA (p = .0497), and gait disorders at disease onset were associated with PSP and MSA (p = .0062). Cognitive and mood disorders were more marked in DLB and most preserved in MSA. Consanguinity was more marked in CBS (p = .037), and family history of dementia and psychiatric diseases was more common in DLB. Thirty‐seven families with similar cases of APS were identified. Conclusions This is the largest African epidemiological study on APS. In our population, APS were frequent and dominated by DLB. The age of onset of parkinsonism was the most decisive feature for differential diagnosis., In this paper, we described the largest cohort of atypical parkinsonian syndromes (APS) ever reported in both sub‐Saharan and North African countries comprising 464 Tunisian patients with APS. In our population, APS were frequent and dominated by DLB. The age of onset of parkinsonian syndrome was the most decisive feature for differential diagnosis.

Genetic generalized epilepsies (GGE) (childhood absence epilepsy (CAE), juvenile myoclonic epilepsy (JME) and epilepsy with generalized tonic-clonic seizures (GTCS)) are mainly determined by genetic factors. Since few mutations were identified in rare families with autosomal dominant GGE, a polygenic inheritance was suspected in most patients. Recent studies on large American or European cohorts of sporadic cases showed that susceptibility genes were numerous although their variants were rare, making their identification difficult. Here, we reported clinical and genetic characteristics of 30 Tunisian GGE families, including 71 GGE patients. The phenotype was close to that in sporadic cases. Nineteen pedigrees had a homogeneous type of GGE (JME-CAE-CGTS), and 11 combined these epileptic syndromes. Rare non-synonymous variants were selected in probands using a targeted panel of 30 candidate genes and their segregation was determined in families. Molecular studies incriminated different genes, mainly CACNA1H and MAST4. The segregation of at least two variants in different genes in some pedigrees was compatible with the hypothesis of an oligogenic inheritance, which was in accordance with the relatively low frequency of consanguineous probands. Since at least 2 susceptibility genes were likely shared by different populations, genetic factors involved in the majority of Tunisian GGE families remain to be discovered. Their identification should be easier in families with a homogeneous type of GGE, in which an intra-familial genetic homogeneity could be suspected.

Introduction L’atteinte des voies visuelles represente 30 % des formes de debut de la Sclerose en plaques (SEP). L’etude des potentiels evoques visuels (PEV) constitue un biomarqueur interessent diagnostique et therapeutique. Objectifs Etudier l’apport des PEV dans l’evaluation de l’efficacite du traitement de deuxieme ligne de la SEP. Patients et methodes Il s’agissait d’une etude prospective menee au service de neurologie de l’Hopital Habib Bourguiba de Sfax et de l’hopital Razi de Tunis. Nous avons inclus des patients suivis pour une SEP refractaire a un traitement par interferons. Ils ont ete explores par des PEV avant et 6 mois apres l’escalade therapeutique. Nous avons calcule le SUM score a chaque moment. Une aggravation correspondait a une augmentation ≥ 1 et une amelioration a une reduction ≥ 1. Resultats Nous avons inclus 49 patients (âge moyen = 34,07 ans ; sexe ratio (F/H) = 2 :1. Les PEV etaient alteres chez 75,6 % de nos patients initialement et 71 % apres escalade therapeutique. Une amelioration du SUM score a ete observee dans 38,7 % chez les patients sous Natalizumab versus 16 % chez ceux sous Fingolimod (p = 0,037). Une diminution de la latence de l’onde P100 a ete significative avant et apres Natalizumab (p Discussion Peu d’etudes se sont interessees a l’interet des PEV dans l’evaluation de l’efficacite therapeutique des medicaments de la SEP. A l’instar des Resultats de l’etude de Philips et al, nous avons retrouve une superiorite du Natalizumab dans la reparation de la conduction nerveuse au niveau des voies visuelles au cours de la SEP. Ceci suggere un pouvoir remyelinisant de celui-ci. Conclusion L’evaluation de l’effet des medicaments de la SEP sur les voies visuelles, via les PEV, constitue un biomarqueur valide et reproductible.

Introduction Le tremblement est decrit chez 26 a 58 % des patients atteints de sclerose en plaques (SEP). Bien qu’invalidant, il n’est pas pris en compte dans l’evaluation fonctionnelle des patients. Objectifs Etudier les caracteristiques du tremblement au cours de la SEP et son rapport avec les lesions radiologiques et evaluer son retentissement fonctionnel et psycho-social. Patients et methodes Nous avons mene une etude transversale incluant des patients suivis pour SEP au service de Neurologie de l’hopital Razi entre avril et juin 2019. Nous avons recherche un tremblement et etudie ses caracteristiques et son retentissement. Nous avons utilise les echelles : Brief ataxia rating scale (BARS), Activities of daily living (ADL), Assessment of tremor-related handicap questionnaire (TRH) et Fahn-Tolosa-Marin Tremor Rating Scale (FTMTRS). Nous avons complete par une etude neurophysiologique du tremblement. Resultats Nous avons inclus 45 patients. Un tremblement etait retrouve chez 18 patients : postural (n = 16), cinetique (n = 5) et de repos (n = 3). Il etait significativement associe a la forme remittente et a une duree d’evolution inferieure a 5 ans. Un retentissement fonctionnel et psychosocial a ete objective, via les scores utilises, independamment de son type, de sa duree d’evolution et de sa severite. Les lesions bulbaires etaient significativement liees au tremblement (p = 0,019). Discussion Nos resultats ont demontre un retentissement du tremblement sur la qualite de vie des patients SEP, independant de la forme de la SEP, de sa duree d’evolution et de sa severite. L’association significative du tremblement aux lesions bulbaires, retrouvee dans notre etude, conforterait l’hypothese impliquant les lesions du tronc cerebral et les circuits cerebello-thalamiques dans la genese de celui-ci. Conclusion Les resultats de notre etude incitent a rechercher regulierement le tremblement au cours de la SEP et a l’integrer dans des echelles de mesure du handicap.

Introduction La maladie de Parkinson (MP) est une maladie heterogene. Les facteurs de progression sur le plan cognitif et moteur sont sujets d’investigation afin de determiner ses differents phenotypes cliniques evolutifs. Objectifs Etudier les facteurs predictifs du declin cognitif et moteur au cours de la progression de la MP. Patients et methodes Nous avons mene une etude transversale au service de Neurologie de l’hopital Razi incluant des patients suivis pour MP. Pour evaluer la progression, nous avons utilise le score Unified Parkinson disease rating scale III (UPDRS-III) et l’echelle Hoehn et Yahr (HY) pour l’estimation du declin moteur ; le Mini-examen de l’etat mental (MMSE) pour l’evaluation de l’efficience cognitive globale et la Batterie rapide d’efficience frontale (BREF) pour l’evaluation des fonctions executives. Resultats Nous avons inclus 150 patients. Le taux de declin cognitif annuel du MMSE (0,48 point) etait correle a un âge de debut plus jeune et a la presence de dysautonomie clinique et electrophysiologique. Le taux annuel de declin moteur de l’UPDRS-III (1,56 points) etait correle a un âge de debut precoce, la presence de troubles du comportement en sommeil paradoxal (RBD) et la presence de dysautonomie. Le risque de chute etait correle a la dysfonction executive et l’hypotension orthostatique. Discussion Comme dans la litterature, le taux annuel du declin etait correle a la presence de dysautonomie. Ceci pourrait etre explique par l’hypotension orthostatique et son effet deletere sur la perfusion cerebrale. Sur le plan moteur, la dysautonomie ainsi que la dysfonction frontale etaient responsable d’un risque plus significatif de chute. Les RBD sont correles a une progression plus rapide. Conclusion La presence de dysautonomie et de RBD etait associee a un phenotype evolutif plus severe de la maladie. Identifier les facteurs de risque modifiables de cette progression permettrait la ralentir.