Basilar artery fenestration and ischemic stroke: An unclear causal relationship.

Basilar artery fenestration is the second most commonly observed fenestration of the cerebral arteries. In addition to our case, we reviewed the clinical, imaging findings, treatment, and prognosis of 9 other reported cases. Patients' mean age was 45.1 years. Half of them had cardiovascular risk factors. Mean time to diagnosis was 9.4 days. The main symptoms were right hemiparesis and dysarthria. Basilar artery fenestration was found in all patients, as well as ours, in addition to a thrombus, found in 2 cases. One patient was treated by IV thrombolysis and thrombectomy. In other cases, antiplatelet drugs or anticoagulants were used. A favorable outcome was observed in most cases with one reported death.

Impulse control disorders in Parkinson disease: A cross-sectional study in Morocco.

Impulse control disorders (ICDs) in Parkinson's disease (PD) comprise a class of psycho-behavioral disorders often associated with dopamine agonist treatment. The aim of our study was to determine the prevalence of ICDs in a group of Moroccan PD patients and to bring forward some specific aspects in our population. One hundred twenty-five PD patients, without memory impairment and treated for at least six months, were studied. They were questioned about ICDs using the QUIP-RS, and simultaneously evaluated on the motor symptoms and their treatment. Our sample was then divided into two groups: ICDs (+) and ICDs (-) groups. ICDs were identified in 28% of patients: pathological gambling in 3.2%, compulsive sexual behavior in 7.2%, pathological buying in 9.6%, eating behavior disorder in 7.2%, punding-hobbyism in 11.1%. At least two ICDs were found in 14% of patients and dopamine dysregulation syndrome in 10.4%. We also noticed another kind of "ICDs-mimics" specific to our own social context such as "excessive charity" in 18.4%, or excessive reading of the Qur'an in 9.6%. These aspects were not included in the calculation of ICDs prevalence. The ICDs (+) group was younger than the ICDs (-) group (P=0.042) and ICDs were more frequent in men (P=0.031). Dopamine agonist equivalent daily dose (DAED) was significantly higher (P=0.01) in the ICDs (+) group. There are no differences between classes of dopamine agonist used. Young age, male gender and DAED are risk factors for the occurrence of ICDs in Moroccan PD patients, as already described in the DOMINION cohort, but the prevalence found in our study was higher. We highlighted some specific ICDs-mimics in our Arab-Muslim population.

[Severe punding in Parkinson's disease]. / Punding sévère au cours d'une maladie de Parkinson.

BACKGROUND: Punding is a stereotypical behavior characterized by an intense fascination with repetitive handling and examining of mechanical devices or arranging common objects. This condition, which is different from both obsessive-compulsive disorder and mania, is still underestimated in patients with Parkinson's disease and may have deleterious social consequences on patients and their families. CASE REPORT: We report the case of severe punding in a 23-year-old parkinsonian woman, who presented, a few days following a rise in the dose of pergolide up to 2,5 mg/(d), frequent and daily unusual repetitive behavior, characterized by ceaseless sewing, disassembly and reassembly of phones, and coloring of drawings. These behaviors were associated with a common peak of dose dyskinesia and were responsible for a considerable reduction in duration of sleep with negative impact on the quality of life of her parents. These symptoms significantly improved immediately after switching pergolide to an equivalent dose of ropinirole (12 mg/(d). DISCUSSION: Punding has only recently come to the attention of physicians through the first report in a parkinsonian patient, triggered by dopaminergic replacement therapy. The phenomenon was thought to be related to excessive dopaminergic stimulation of the limbic and associative pathways. The current mainstay of treatment is the reduction in the dose of dopaminergic medication or changing the presumed responsible drug, often a dopaminergic agonist. In this article, the authors review the epidemiology, pathophysiology and management of this curious phenomenon.

[Pediatric multiple sclerosis: is it different from the adult form?]. / La SEP de l'enfant: est-elle différente de celle de l'adulte ?

INTRODUCTION: Multiple sclerosis (MS) is not uncommon in children. The aim of this study was to compare early onset MS (EOMS) with adult onset MS (AOMS). METHODS: A retrospective study including MS cases between 1997 and 2010. EOMS was defined by age at MS onset<18years. Data were collected using the EDMUS database (European Database of Multiple Sclerosis) including: sex, age at onset, disease duration, EDSS, score after relapse. The MSSS and the Progression Index were calculated. Patients with disease duration less than one year were excluded. MS symptoms at onset and at further relapses were also noted. These parameters were compared between the EOMS and the AOMS groups. RESULTS: Two hundred fifty-nine cases were included including 31 EOMS (11.96%). The mean follow-up was 96months. The relapsing-remittent form was significantly more frequent in the pediatric group (94% vs 79%). Mean EDSS and MSSS scores and the percentage of fast progressors (MSSS>5) were lower in the EOMS group. Analysis of neurological symptoms at the first MS attack and further neurological events showed a lower frequency of gait disturbances, motor symptoms and bladder symptoms in the EOMS group compared with the AOMS group. The 10-year mean EDSS score was 1.9 for EOMS and 4.1 for AOMS, after 25years it was 4.5, and 7.27 respectively. CONCLUSION: This study highlights the relative frequency of EOMS in our MS population. However, different severity scores showed less disability progression in EOMS patients compared with AOMS patient; irreversible disability was reached at an early age.

[Striatopallidodentate calcinosis, hypoparathyroidism and neurological features: a case series study]. / Calcinose striatopallidodentelée, hypoparathyroïdie et atteinte neurologique: étude de série.

INTRODUCTION: The respective roles of hypocalcemia and intracerebral calcifications in the occurrence of various neurological manifestations in hypoparathyroidism is not entirely clear. Nevertheless, therapeutic and prognostic implications are important. OBJECTIVES: We analyze the neurological clinical aspects observed in hypoparathyroidism and correlate them to the biological calcium abnormality and radiological CT scan findings. We also compare these results with data reported in the idiopathic form of striatopallidodentate calcinosis. PATIENTS: The neurological clinical, CT scan findings and outcome have been retrospectively studied in patients recruited during 13 years (2000-2012) for neurological features associated with hypoparathyroidism or pseudohypoparathyroidism. RESULTS: Twelve patients with primary hypoparathyroidism (n=5), secondary to thyroidectomy (n=4) and pseudohypoparathyroidism (n=3) were studied. The sex-ratio was 1 and mean age was 39 years. All patients had a tetany, 60% had epilepsy, associated in one patient with "benign" intracranial hypertension; 50% had behavioral changes. Response to calcium therapy was excellent for all these events. Moderate cognitive deficit was noted in three patients (25%), parkinsonism in two patients and hyperkinetic movement disorders in one other. These events were not responsive to calcium therapy and were more common in cases of extensive brain calcifications and in patients who had pseudohypoparathroidism. COMMENTS: This study suggests that, in patients with hypoparathyroidism, epilepsy and psychiatric disorders are induced by hypocalcemia and reversible after its correction. Cognitive and extrapyramidal impairment seem to be related to the progressive extension of intracerebral calcification, particularly in patients with a late diagnosis. In patients with pseudohypoparathyroidism, this finding is different because of the contribution of other factors, specific to this disease.

[Posterior reversible encephalopathy syndrome and eclampsia: a descriptive study of 13 cases in Morocco]. / Syndrome d'encéphalopathie postérieure réversible et éclampsie: étude descriptive de 13 cas au Maroc.

INTRODUCTION: The occurrence of posterior reversible encephalopathy in eclampsia is a rare but known event. We propose to describe the clinical and radiological features and the outcome. METHODS: A retrospective study was conducted from January 2005 to April 2010 including all cases of posterior reversible encephalopathy syndrome (PRES) occurring on eclampsia in patients hospitalized in the obstetrical intensive care unit, University Hospital of Casablanca. RESULTS: Thirteen cases of PRES on eclampsia were collected, the average age was 29 years (18-42). Systolic pressure and diastolic blood pressure at admission were higher than 150 mmHg and 100 respectively in 10 cases. The signs found were: a regressive blindness in five patients and focal signs in four. The complications were thrombocytopenia in 10 patients, abnormal liver function in eight, Hellp syndrome in nine, and acute renal failure in two. The brain regions most commonly affected were the parietal and occipital areas (13 patients), followed by temporal regions, frontal, and basal ganglia (eight patients each). Five patients required assisted ventilation (AV) over 24 hours. Death complicated the outcome in four of our patients, but no deaths were directly attributable to PRES itself, and all four patients had Hellp syndrome and required AV greater than 48 hours. In the other patients, total regression of neurological signs was noted. CONCLUSION: This study emphasizes the severity of the Posterior 'reversible' encephalopathy syndrome on eclampsia.

Methylenehydrofolate reductase (C677T) polymorphism and large artery ischemic stroke subtypes.

BACKGROUND: The role for the methylenetetrahydrofolate reductase C677T gene variants in the risk of ischemic stroke is controversial. METHOD: This first case-control study including 91 cases affected by ischemic stroke and 182 controls matched for age, sex, and same area was conducted in Casablanca, Morocco. Allele and genotype frequency were characterized by using PCR followed by HinfI enzymatic digestion. RESULTS: We found no statistic association of T allele carriers genetic factors with stroke; odds ratio, 1.1; 95% confidence interval (CI), 0.59-2.04, P = 0.303. The results shown significant association of T allele carriers genetic factors with atherothrombotic subtype stroke (n = 42); odds ratio, 2.1; 95% CI: 1.17-3.8; P = 0.012, and adjusted odds ratio of 6.5; 95% CI: 1.86-23.1, P = 0.003, for TT genotype variant compared with CC wild genotype. CONCLUSION: We suggested that MTHFR C677T variant may be a determinant of atherothrombotic event of ischemic stroke in Morocco.

Devic-like syndrome in the course of pulmonary tuberculosis.

INTRODUCTION: Optic neuromyelitis or Devic's syndrome is a very rare disease affecting the optic tracts and the spinal cord. Its association with evolving pulmonary tuberculosis has been reported in a handful of case reports. CASE REPORT: The authors report two cases of Devic's syndrome associated pulmonary tuberculosis (48 and 43 years old men). The First patient was experiencing evolving pulmonary tuberculosis. The two patients were admitted for bilateral blindness followed by paraplegia and sphincter disturbance. Clinical examination and investigations excluded direct tuberculous involvement of the nervous system or a reaction to antituberculous therapy and Devic's syndrome was diagnosed, based on Wingerchurk's criteria. Following treatment with corticosteroids and antituberculous chemotherapy, we noted partial recovery of motor symptoms and sphincter control but the patients remained completely blind. CONCLUSIONS: Throughout this case report, the authors emphasize the rarity of this association and discuss the pathophysiological mechanism, which appears to be an immune dysfunction triggered by mycobacterium infection.

[Methylprednisolone pulse in treatment of childhood chronic inflammatory demyelinating polyneuropathy]. / Place des bolus de méthylprednisolone dans le traitement des polyradiculonévrites chroniques de l'enfant.

Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is rare and treatment is based primarily on intravenous immunoglobulins or oral corticosteroids. Boluses of methylprednisolone (MP) are a possible alternative. We report 3 cases of CIDP in children with good outcome after MP pulse therapy. One male (7 years of age) and 2 females (4 and 5 years of age) presented with recurring episodes of functional impotence of both lower limbs and walking impairment, partially reversible without treatment. Clinical and electrophysiological data and the analysis of the cerebrospinal fluid were compatible with CIDP. MP pulses were administered: the total number of pulses varied from 5 to 8, very satisfactory progression on the clinical and electrophysiological pattern was noted, without recurrence in the 3 cases. Childhood CIDP presents clinical, electrophysiological outcome, and prognostic particularities, recurring readily, and the outcome is good. Boluses of MP are an alternative for treatment of these neuropathies in childhood.

[Primary Sjögren's syndrome and neuromyelitis optica]. / Syndrome de Goujerot-Sjögren primitif et neuromyélite optique de Devic.

Neuromyelitis optica (NMO) is characterized by the association of optic neuritis and myelitis without any other neurological signs. In 30 % of the cases, NMO is associated with a systemic disorders. We report two cases of NMO associated with primary Sjögren's syndrome that was diagnosed during an acute flare of NMO. The relationship between NMO and Sjögren's syndrome has not been clearly identified. This association likely reflects the coexistence of two autoimmune disorders. The prognosis of NMO appears to be more severe in patients having coexisting Sjögren's syndrome, which renders useful to search for this association in patients with neuromyelitis optica.