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Objective: The aim of this study was to describe the clinical, biological and radiological characteristics of patients with syphilitic vasculitis, and to assess the outcome after treatment., Methodology: A retrospective review was carried out based on the records of patients with ischemic stroke, and reactive CSF TPHA and VDRL results. None of these patients showed symptoms of any other diseases or had received high doses of penicillin., Results: A total of 53 patients with stroke met the diagnostic criteria for syphilitic arteritis. Their average age was 41±12 years. Nine patients had a history of genital ulcer (17%), and the median duration of illness after presenting a chancre was 8 [range: 1-14] years. A prodromal syndrome was seen in 27 patients (50.9%) and included changes in mental status in 14 patients (26.4%), seizures in 10 cases (18.9%), headache in eight (15.1%) and memory loss in seven (13.2%). Neurological events included focal motor deficits in 29 cases (54.7%), ataxia in 11 (20.8%) and movement disorders in 15 (28.3%). HIV serology was performed in 31 patients and proved negative in every case. Disease evolution was generally favorable: 12 patients (22.6%) were autonomous at the time of hospital discharge; 29 (54.7%) had partially recovered; and only seven (13.2%) still had signs of severe sequelae., Conclusion: A diagnosis of syphilitic stroke should be suspected in young patients as a manifestation of syphilis, and tests for neurosyphilis should be routine in neurology departments to make a prompt diagnosis, thereby preventing psychological sequelae., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Growing evidence suggests that hypovitaminosis D contributes to the pathogenesis of multiple sclerosis (MS)., Objective: This study aimed to evaluate whether vitamin D levels are associated with having MS and some of its characteristics in the Moroccan population., Methods: Using liquid chromatography-tandem mass spectrometry, the 25(OH)D 3 metabolite was measured to quantify vitamin D serum levels (DSLs) in 113 patients with MS and 146 healthy controls matched for gender and age. DSLs were then compared between patients and controls, with correlations sought between DSLs and gender, age at onset, disease duration, MS type, degree of disability (EDSS score) and disease severity (MSSS) in patients., Results: Hypovitaminosis D (DSL<30ng/mL) was observed in 97.3% of MS patients and in 98.6% of controls. Although the mean DSL was slightly lower in patients (11.69±6.97ng/mL) than in controls (12.98±6.58ng/mL), there was no significant association between DSL and MS status (P=0.131). Similarly, among patients, no apparent association was found between DSL and MS type (P=0.214), EDSS score (P=0.076) or MSSS (P=0.772)., Conclusion: Our study suggests that DSL is not associated with having MS nor with MS type, degree of disability or disease severity in the Moroccan population. On the other hand, DSL was lower in women and decreased with age., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

This review exposes recent advances on the role of vitamin D, cholecalciferol, a secosteroid, in the central nervous system. In humans, vitamin D arises from cutaneous transformation of 7-dehydrocholesterol under the effect of UVB exposure or from food intake. Vitamin D has an immunomodulatory role through its anti-inflammatory and anti-autoimmune actions. In the nervous system, vitamin D is involved in the regulation of calcium-mediated neuronal excitotoxicity, in the reduction of oxidative stress, and in the induction of synaptic structural proteins, neurotrophic factors and deficient neurotransmitters. Reduced exposure to sunlight and low food intake can lead to vitamin D deficiency. Increasing evidence highlights the impact of vitamin D deficiency as a favoring factor in various central or peripheral neurological diseases, especially multiple sclerosis and several neurodegenerative diseases, such as amyotrophic lateral sclerosis, Parkinson's disease and Alzheimer's disease. Recently, several clinical trials on vitamin D supplementation stressed the role of vitamin D as a protective and/or prognostic factor in the onset and progress of such neurological conditions., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system characterized by focal inflammatory infiltrates, demyelinating lesions and axonal injury. The purpose of the study was to evaluate the retinal nerve fiber layer (RNFL) thickness by optical coherence tomography (OCT) in Moroccan patients with MS and to assess the relationship between RNFL thickness and disease duration, Expanded Disability Status Scale (EDSS) score, visual acuity and automated visual field indices., Materials and Methods: Thirty-one patients with definite MS and thirty-one disease-free controls were enrolled in the study. After neurologic consultation, ophthalmologic examination including visual acuity, automated visual field testing and OCT were performed., Results: Significant differences between both groups were observed in OCT parameters (total, temporal and macular ganglion cell layer) with lower thickness in the MS group. In patients without a history of optic neuritis, there were statistically significant inverse correlations between total RNFL thickness and disease duration, neurologic disability evaluated by the EDSS, logMAR visual acuity and automated visual field indices., Conclusions: OCT seems to be a reproducible test to detect axonal loss of ganglion cells in MS. Further and larger longitudinal prospective studies would be valuable to assess the evolution over time of the RNFL measurements in Moroccan MS patients., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Objective: To report the characteristics of the most frequent tremors in a population of Moroccan patients., Background: Tremor is the most common movement disorder. It implies a wide variety of disorders with Parkinson's disease and essential tremor being the most frequent., Methods: A retrospective study of 148 patients with tremor referred to our movement disorders outpatient clinic was performed. Clinical features and treatment regimens were analyzed. Patients with parkinsonian tremor were excluded., Results: We included 62 patients with non-parkinsonian tremor. The etiologies were as follows: essential tremor (54.8%), dystonic tremor (19.4%), tremor associated with dystonia (14.5%), enhanced physiological tremor (3.2%), cerebellar tremor (3.2%), psychogenic tremor (3.2%) and Holmes' tremor (1.6%). The characteristics of essential tremor patients were analyzed. Female patients accounted for 67.6% of patients. Mean age at the onset of tremor was 52.2 ± 16.4 years. Family history of tremor was reported in 17.6% of cases. Tremor affected the arms (94.1%), head (52.9%), voice (35.3%) and legs (8.8%). Tremor was bilateral in 87.5% but was asymmetrical in 50% of patients. Patients had postural tremor (76.5%), kinetic tremor (79.4%) and rest tremor (associated in 11.8%). Treatment relied on propranolol (88.3%), primidone (14.7%), gabapentin (14.7%), clonazepam (14.7%), alprazolam (11.8%), topiramate (5.9%) and, in one patient, radiosurgery., Conclusions: Essential tremor was the predominant diagnosis, confirming its high prevalence. There was a predominance of female patients and a peak of age at onset in the fifth and sixth decades. Asymmetry of the disease was noted in half of patients., (Copyright © 2013. Published by Elsevier Masson SAS.)

Lung carcinomas have a high propensity to metastasize to the liver, adrenal, bone or brain. However, certain atypical intracranial sites may occur. We report two cases of adenocarcinoma of the lung discovered by peculiar brain metastases. The first patient presented with an orbital apex syndrome due to a metastasis to the cavernous sinus with intraorbital extension. The second patient presented with headache and visual loss due to metastasis to the pituitary. Intrasellar and parasellar metastases are rare sequelae of pulmonary neoplasms. Etiological diagnosis is based on imaging and sampling or biopsy. They are often associated with a poor prognosis. The differential diagnosis of an intracranial process in the area of the sella should include metastasis even in the absence of a known primary cancer., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction: Multiple system atrophy (MSA) is a sporadic and rapidly progressive neurodegenerative disorder of poor prognosis, characterised clinically by any combination of parkinsonian, autonomic, cerebellar, or pyramidal signs. We report our experience in movement disorders consultation concerning the clinical presentation and the course of MSA in Moroccan patients., Methods: A retrospective review of the medical records of 17 patients with diagnosis of MSA seen in our outpatient clinic from January 2007 to December 2010., Results: In our 17 patients, 76.5% were men and the mean age of onset was 52±9 years. MSA-P was the major clinical phenotype (82.4%). Eleven patients (64.7%) were classified as having probable MSA and six patients (35.3%) as possible MSA. Dysautonomic features were detected in all patients; urinary symptoms were found in 76.5% of cases and orthostatic hypotension in 64.7%. Treatment regimen included l-Dopa with a mean daily dose of 621.4±346.8mg/day and symptomatic treatment of dysautonomia. The mean duration of disease evolution was of 4.7±1.9 years., Discussion: Our results show a male predominance and an early age of disease onset. MSA-P was the predominant subtype. Our results are similar to the European MSA series., Conclusion: Multicentre studies are needed to better characterise MSA in Morocco given the rarity of this disease., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction: Quality of life assessment of multiple sclerosis patients in Morocco appears essential, considering the early age of onset and the significant functional, psychological and social impact of disease symptoms and disorders. Our goal was to translate the MSQOL-54 into Arabic followed by cross-cultural adaptation, and validation of the translated version., Methods: Patients with multiple sclerosis were recruited over a period of 7 months, from February to August 2007. Sociodemographic data and a detailed clinical description (neurological examination, EDSS, MMS) were collected before administration of the questionnaire. The MSQOL-54 was translated and test validation techniques were applied: back translation, revision by a committee of experts, study of acceptability, multivariate analysis, reliability and clinical validity., Results: Seventy-eight patients were included. There were 23 men and 55 women of various ages, various stages of disability (EDSS from 1 to 8.5) and various clinical forms. Evaluation of the 78 completed questionnaires revealed a satisfactory acceptability considering the limited number of missing answers and the average time to complete the test (24 minutes). The reliability and the multimultivariate analysis were excellent (coefficient of Cronbach ≥0.7 with a percentage of convergence and divergence validity around 100%). A very significant relationship was found between physical scores and EDSS., Conclusion: Measuring the quality of life is an approach that confirmed its usefulness through this study. We propose a valid Arabic version of MSQOL-54., (Copyright © 2012. Published by Elsevier Masson SAS.)

Introduction: Neurological manifestations in Behçet's disease represent between 4 to 49% of systemic manifestations and remain, in the long term, the leading cause of morbidity and mortality., Methods: Retrospective series of 40 severe Neurobehçet cases fulfilling the International Study Group criteria for Behçet's disease were consecutively recruited over a period from June 2004 to December 2010. All patients had clinical and ophthalmologic examinations; they underwent laboratory and imaging investigations. They received corticosteroids and cyclophosphamide as initial bolus of 600 mg/m(2) of BSA in the 1st, 2nd, 4th, 6th and 8th day followed by a bolus of 600 mg/m(2) BSA every 2 months for 2 years. Antithrombotic therapy was given to patients with cerebral deep venous thrombosis. Patient follow-up and tolerance to treatment were analyzed., Results: The average age at diagnosis was 34±13 years, with a sex-ratio of 1.78. The clinical presentation was dominated by the meningoencephalitis in 48.8% of cases, cerebral deep venous thrombosis in 43.6% of cases and myelopathy in 7.7% of cases. The 40 patients receiving cyclophosphamide bolus, despite two aggravated cases, evolved positively with clinical improvement and good tolerance., Conclusion: The demographic and clinical aspects of our series are similar to those reported in the literature. In contrast to previously reported cases of a poor prognosis in severe neurobehçet's disease, our study suggests that immediate and aggressive treatment by cyclophosphamide may ameliorate the prognosis. However, a multicenter study is needed to confirm the possible efficacy of cyclophosphamide and further assess the long-term tolerance., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Strokes are the main neurological manifestation of antiphospholipid syndrome. Other clinical presentations are possible and may mimic classic symptoms of multiple sclerosis (MS). A 46-year-old woman, with a history of two miscarriages, presented four subacute neurological episodes (optic neuritis, right facial paralysis, paraparesis of the thigh, and right brachial monoparesis). Using McDonald criteria, the diagnosis of multiple sclerosis was retained. Because of the occurrence of thrombocytopenia during a final relapse, we reconsidered the diagnosis of MS. Search for antiphospholipid antibodies was positive. All clinical manifestations and complementary tests were compatible with the diagnosis of antiphospholipid syndrome associated with multiple sclerosis. Given the great similarity of clinical, radiological and biological findings in the two diseases, non-thrombotic neurological manifestations of antiphospholipid syndrome can be difficult to distinguish from MS associated with antiphospholipid syndrome., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Psychotic symptoms are a rare but well-known complication of epilepsy. The prevalence is estimated between 4 and 9%., Patient: We report a case of a 40-year-old patient, unrecognized epileptic, who presented an acute psychotic syndrome which seemed to be of functional origin, the EEG performed during the episode, and the cerebral CT scan being normal. Nevertheless, the clinical presentation, especially the sudden ending of delusions, led to further investigations. Careful history taking and repeated EEG recordings allowed the diagnosis of partial epilepsy that had begun 17 years earlier and symptomatic of a dysembryoplastic tumour of the left hippocampus revealed by MRI., Discussion and Conclusion: Search for an epileptic origin of an acute psychotic syndrome must always be undertaken by systematic EEG. The possibility of a symptomatic temporal tumor must not be overlooked., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Introduction: Central nervous system involvement is rare in Wegener granulomatosis. Stroke is the most common event suggestive of the disease., Comment: A 35-year-old woman, who was followed for rhinitis and mild asthma, described gradual decline of visual acuity in the right eye over two months, persistent nasal obstruction and fronto-orbital headache since a few weeks. She presented left hemiparesis due to a stroke, associated with exophthalmos and deficits of the optic nerve and abducens of the right eye. The otolaryngological examination found signs of crusty rhinitis and right nasal stenosis. The diagnosis of Wegener's granulomatosis was established on the basis of the clinical findings, radiological aspects and the presence of ANCA. The patient was treated by antiplatelet agents and high-dose corticosteroids associated with immunosuppressive drugs including cyclophosphamide in a monthly bolus., Discussion: This case illustrates two of the three pathogenic mechanisms that may account for central nervous system involvement in Wegener granulomatosis: vasculitis, extension by contiguity of granulomatous tissue from the nasal cavity or sinuses, and in situ formation of a granuloma into the brain parenchyma or meninges., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Introduction: Neurological manifestations of systemic lupus erythematosus are frequent and polymorphic. In 40% of cases, lupus can be revealed by neurological symptoms. Cerebral nervous system complications predominate and can be a negative factor for prognosis. Peripheral features are rare and various and can compromise functional prognosis, sometimes with fatal outcome., Case Report: We report the case of a 30-year-old woman who presented a cerebral venous thrombosis of the superior longitudinal sinus. Outcome was favorable with antibiotics and anticoagulants. Four months later, she developed an acute polyradiculoneuritis associated with an inflammatory syndrome and positive tests for antinuclear antibody and antinuclear anti-DNA. The diagnosis of neurolupus was retained on the basis of four criteria of the American college of Rheumatology. The patient was given steroid therapy associated with a course of intravenous immunoglobulin. She has fully recovered her deficit., Conclusion: Cerebral venous thrombosis and acute polyradiculonévrites are rare events in systemic lupus erythematosus. Early diagnosis and management are crucial., (2009 Elsevier Masson SAS. All rights reserved.)

Introduction: Horton's disease is the most common vasculitis of elder people. Several neurological complications are reported, but pachymeningitis is exceptional., Observation: A 71-year-old patient who presented headache, hyperesthesia of the scalp, weight loss with a biological inflammatory syndrome and meningeal thickening on MRI. The diagnosis of pachymeningitis related to Horton's disease was retained. The patient was treated by corticosteroids with a good clinical, biological and radiological course after 22 months., Conclusion: Horton's disease is a potential diagnosis in elderly persons with pachymeningitis and inflammatory syndrome., (Copyright 2009. Published by Elsevier Masson SAS.)

Introduction: Cyclophosphamide (CPM) is used empirically in the treatment of progressive multiple sclerosis. The purpose of this study was to evaluate its efficacy in naive patients with progressive multiple sclerosis., Patients and Methods: Thirty-one patients were retrospectively included (17 secondary multiple sclerosis, 14 primary progressive multiple sclerosis), and received infusions of CPM (600mg/m(2)) every eight weeks, for 18 months. Evaluation was based on time to EDSS progress., Results: Among patients with secondary progressive multiple sclerosis, 94% were stable or improved at six months, 77% at 12 months, and 75% at 18 months. The same result was reported in 69.2% of primary progressive multiple sclerosis at six months, 64% at 12 months, and 55.5% at 18 months. We found no difference between the two groups., Conclusion: Our results suggest that the CPM is efficient in progressive multiple sclerosis; however, its place must be reconsidered before establishing an international consensus for treatment; open label studies are needed to validate the present findings.

Introduction: Neurological manifestations of celiac disease are rare and polymorphic. Similar to lesions of the digestive tract, the standard treatment includes steroids., Case Report: A 41-year-old woman, followed up for celiac disease resistant to gluten-free diet, developed rapidly spastic paraparesis, cerebellar syndrome, horizontal diplopia and decline of visual acuity. The diagnosis of neurological complications of celiac disease was established and the patient was treated with methylprednisolone, followed by oral prednisone. For 9 years, the patient's neurological status remained stabilized with a prednisone dose at 20mg per day. The patient relapsed when progressive reduction of prednisone dose was attempted; neurological and gastrointestinal signs worsening at 15mg per day; increasing the dose to 30mg improved the clinical status., Discussion: The mechanism of onset of neurological disease remains unknown. Immunological, nutritional, toxic or metabolic factors could be involved. The positive response to corticosteroids observed in this patient suggest an immunological mechanism.

Primary Sjogren's syndrome is an autoimmune exocrinopathy with various extraglandular complications. Central neurological manifestations were described in 20 percent of cases. Cerebellar syndrome is uncommon in Sjogren's syndrome, but can occur after diagnosis, or rarely as an inaugural sign of the disease. We report the case of a 54-year-old man who presented a cerebellar syndrome with xerostomy and xerophthalmos. The diagnosis of primary Sjorgren's syndrome was confirmed on the accessory salivary gland biopsy. The patient received oral and intravenous corticosteroid therapy associated with cyclophosphamide monthly. Outcome was good.

Background: Pseudoainhum is a rare disease characterised by gradual fibrous constriction of the fingers and/or toes eventually resulting in their amputation. In this article, we report the first case seen in Morocco, highly unusual in terms of its severity., Case Report: A 46-year-old woman with no toxic habits was hospitalised for spontaneous amputation of the fingers and toes. This condition began when the patient was 12 years old with the appearance of a circular constriction band at the base of the fifth toe, eventually resulting in its loss. The patient gradually lost all her other toes and fingers except for the first joint of her left index finger. There was no family history of any similar condition. Clinical examination also revealed perforating plantar disease in two of the stumps and peripheral neuropathy in all four limbs, comprising predominantly axonal disease responsive to electromyogram. Amputation of the index finger was completed and histological examination of the removed section showed nothing unusual, with no signs of diabetes., Discussion: The peculiarity of our case resides in the exceptional severity of these amputations and their association with isolated polyneuropathy. Pseudoainhum has been described chiefly in patients with congenital keratoderma, certain systemic diseases, diabetes and alcohol dependence. A number of etiopathogenic hypotheses have been suggested: traumatic, infectious, vascular, neurological and genetic processes.

Introduction: Benign intracranial hypertension (BIH) or pseudotumor cerebri is diagnosed on the basis of Dandy's criteria. BIH creates an emergency situation because of the risk of lost vision. In this work, we studied retrospectively a series of 10 cases of BIH all meeting Dandy's criteria. Our objective was to assess the benefit of the corticosteroid-acetazolamide combination on clinical course, especially on papiledema., Methods: Eighty-four patients were hospitalized at the neurology department (Hopital des Specialites, Rabat) over a period of 14 years (1988-2001). They were divided into three groups: forty cases of cerebral thromophlebitis, 10 cases of BIH. In the remaining 34 cases, the investigations were insufficient, so that Dandy's criteria could not be verified. We studied only the 10 cases presenting with a diagnosis of BIH diagnosis complying with Dandy's criteria. The patients underwent a physical examination, cerebral magnetic resonance imaging (MRI) if possible or CT scan with conventional angiography, and CSF examination with pressure measurement. We analyzed age, sex-ratio, clinical aspects and the outcome after treatment. The major criterion of outcome was the regression of papilledema., Results: There were 9 women and 1 man. The mean age was 24.6 8.4 years. Behcet's disease was noted in 3/10 patients. The clinical features were those described in the literature. Patients were treated by corticosteroids combined with acetazolamide and CSF depletion in all cases. CSF derivation was performed in only 1 patient. Definitive blindness was noted in 2 patients at admission. A favorable course was noted in 8/10 cases, with regression of papilledema within approximately 1 month., Discussion: We suggest that the corticosteroid-acetazolamide combination can have a beneficial effect on papilledema in BIH. However, these results should be confirmed by a prospective, randomized, double blind controlled study.

Clinical and electrophysiologic data concerning the postanoxic action myoclonus syndrome were described by Lance and Adams in 1963. A patient presented myoclonus involving all parts of the body after laryngospasm. The myoclonus was worsened by emotion and voluntary activity and was clearly attenuated by sleep. Spectacular improvement was observed within one week after valproate and piracetam administration. Clinicians should be aware of this syndrome in order to propose appropriate treatment and avoid delay in the therapeutic decision.

Lafora disease is a progressive myoclonic epilepsy, Clinically defined by the association of myoclonic, epileptic fits and dementia. We report a case with an atypical Lafora disease, marked by delayed onset at 25 years of age, prolonged course, associated with secondary cognitive impairment and myoclonic features.

Hydatid cysts of the brain are very rare. Exceptionally, signs and symptoms are primarily those of acute cerebral ischaemia. Two cases of acute cerebral ischaemia are reported in a 21 year old and 40 year old women. A computed tomographic scan revealed a middle cerebral artery (MCA) infarct and an abrupt cutoff of the MCA at cerebral angiography. A few months later, a CT scan showed cysts in the territory of the infarct. Hydatid cysts were also found in multiple viscera, particularly in the heart. These two observations and some cases reported in the literature suggested that the myocardial cyst may have ruptured into the ventricular cavity, resulting in widespread intravascular dissemination of embryo and causing an acute cerebral infarction. The interest of these cases lies in the rarity of an acute cerebral ischaemia due to hydatid cyst embolism, and in the early diagnosis of cardiac cysts in young patients.

We studied 5 cases of syphilitic lateral amyotrophic sclerosis. The diagnosis was based on the presence of a lymphocytic reaction in the CSF and positive VDRL and TPHA reactions in both blood and CSF. Clinically, the disease affected the arms in 3 cases and produced paraplegia in 2 cases. The gradual extension of amyotrophy over several months, the diffusion of electromyographic abnormalities and the finding of spinal cord atrophy at myelography and CT suggested a subacute ischemic mechanism with meningo-myelic arteritis involving the anterior horns. After treatment with penicillin G in high doses, the outcome was constantly favourable, with improvement of motor deficit in 4 cases and stabilisation in 1 case in a 5 to 13 years' follow-up.