Stroke and syphilis: A retrospective study of 53 patients.

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.

Vitamin D deficiency and its role in neurological conditions: A review.

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.

A clinical study of non-parkinsonian tremor in Moroccan patients.

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.

Profile of multiple system atrophy in Moroccan patients attending a movement disorders outpatient clinic in Rabat university hospital.

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.

[Atypical metastatic sites for adenocarcinoma of the lung]. / Localisations métastatiques inhabituelles d'adénocarcinome pulmonaire.

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.

[Multiple sclerosis associated with antiphospholipid syndrome: diagnostic and therapeutic difficulties]. / Sclérose en plaques associée à un syndrome des antiphospholipides : difficultés diagnostiques et thérapeutiques.

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.

Neurological manifestations of Behçet's disease: evaluation of 40 patients treated by cyclophosphamide.

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.

Motor activation in multiple system atrophy and Parkinson disease: a PET study.

BACKGROUND: Multiple system atrophy (MSA) is an atypical parkinsonian syndrome including cerebellar impairment and poor response to levodopa. We assessed right hand motor activation in patients with MSA before and after an acute levodopa challenge in comparison with patients with PD and healthy volunteers (HVs). METHODS: Eighteen patients with MSA, 8 patients with PD, and 10 age-matched HVs were included. Regional cerebral blood flow measurements with H(2)(15)O PET were performed at rest and during a right hand movement. Statistical parametric mapping was used to analyze motor vs rest in OFF and ON conditions and the effect of levodopa on motor activation. RESULTS: Before levodopa, patients with MSA activated most known cerebral motor areas. Compared with HVs, patients with MSA exhibited less bilateral cerebellar activation and greater left superior parietal activation. They also had less bilateral cerebellar and greater supplementary motor and left superior parietal activation than patients with PD. Conversely, patients with PD had greater activation than HVs in the right cerebellum and less in the supplementary motor cortex. After levodopa, patients with MSA exhibited reduced activation in anterior cingulate, whereas patients with PD had greater activation in the right cerebellum. CONCLUSION: Patients with MSA and patients with PD recruited different motor networks. Patients with PD preferentially activated cerebellar pathways, possibly to compensate for basal ganglia dysfunction. This was not observed in patients with MSA, probably because of cerebellar dysfunction; other frontoparietal cortical areas were recruited.

Cortical tremor (FCMTE: familial cortical myoclonic tremor with epilepsy).

For 15 years, 50 Japanese and European families with cortical myoclonic tremor and epilepsy were reported in the literature under various names. More recently, the acronym familial cortical myoclonic tremor with epilepsy (FCMTE) has been proposed for this new clinical entity based on both clinical and electrophysiological criteria: irregular postural myoclonic tremor of the distal limbs, familial history of epilepsy, autosomal dominant inheritance, and a rather benign outcome. The diagnosis is confirmed by electrophysiological features favoring cortical reflex myoclonus (enhanced C reflex at rest, giant somatosensory evoked potentials (SEPs), premyoclonus cortical spikes detected by the jerk-locked back-averaging method), and a good response to antiepileptic drugs. The genetic analysis of these families shows heterogeneity with a linkage to chromosome 8q24 for Japanese families, a linkage to chromosome 2p for Italian families, the exclusion of 8q24 locus for a Spanish family, and the exclusion of both loci for a Dutch family. The similarities of this syndrome with the group of myoclonic epilepsy suggest an abnormality of a gene encoding ion channels.

[Epidemiological and clinical aspects of neurosyphilis in Morocco]. / Aspects épidémiologiques et cliniques de la neurosyphilis au Maroc.

Neurosyphilis accounts for 56%-70% of all visceral syphilis and is a complication in 5%-10% of cases of untreated syphilis. The aim of this study was to evaluate the epidemiological aspects and clinical presentations of neurosyphilis in Morocco through a series of 201 patients attending the Centre for Neurological Services at the university hospital in Rabat between 1986 and 1997. The mean age of the patients was 41.26 (SD 9.23) years (range: 17-70 years); the majority (91%) were male. The incidence of neurosyphilis in Morocco is high. From 31 cases per year in 1985, it has fallen since 1990 to reach 10 cases in 1997. Among the different clinical presentations recorded, chronic meningoencepahalitis was the commonest, followed by meningovasculitis, tabes dorsalis and optic atrophy.

[Epidemiological and clinical aspects of neurosyphilis in Morocco]

Neurosyphilis accounts for 56%-70% of all visceral syphilis and is a complication in 5%-10% of cases of untreated syphilis. The aim of this study was to evaluate the epidemiological aspects and clinical presentations of neurosyphilis in Morocco through a series of 201 patients attending the Centre for Neurological Services at the university hospital in Rabat between 1986 and 1997. The mean age of the patients was 41.26 [SD 9.23] years [range: 17-70 years]; the majority [91%] were male. The incidence of neurosyphilis in Morocco is high. From 31 cases per year in 1985, it has fallen since 1990 to reach 10 cases in 1997. Among the different clinical presentations recorded, chronic meningoencepahalitis was the commonest, followed by meningovasculitis, tabes dorsalis and optic atrophy

[Clinical and therapeutic aspects of benign intracranial hypertension]. / Hypertension intracrânienne bénigne. Aspects cliniques et thérapeutiques.

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.

[Favorable course of Lance-Adams postanoxic action myoclonus]. / Syndrome de Lance et Adams d'évolution favorable.

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.

[Descending necrotizing mediastinitis: a diagnosis not to miss]. / La cellulite cervico-médiastinale nécrosante: un diagnostic à ne pas méconnaître.

Descending necrotizing mediastinitis is a severe disease which occurs after a mild otorhinolaryngologic or dental infection. The diagnosis must be established rapidly with the help of clinical and computed tomography of the neck and chest data. Treatment is based on antibiotics, surgery and hyperbaric oxygen. The outcome is poor with high mortality. We report a case of septicemia complicating descending necrotizing mediastinitis after dental infection.