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.

Glyceraldehyde-3-phosphate dehydrogenase (GAPDH) and Alzheimer's disease.

Glyceraldehyde-3-phosphate dehydrogenase (GAPDH) is a ubiquitous enzyme that catalyzes the sixth step of glycolysis and thus, serves to break down glucose for energy production. Beyond the traditional aerobic metabolism of glucose, recent studies have highlighted additional roles played by GAPDH in non-metabolic processes, such as control of gene expression and redox post-translational modifications. Neuroproteomics have revealed high affinity interactions between GAPDH and Alzheimer's disease-associated proteins, including the ß-amyloid, ß-amyloid precursor protein and tau. This neuronal protein interaction may lead to impairment of the GAPDH glycolytic function in Alzheimer's disease and may be a forerunner of its participation in apoptosis. The present review examines the crucial implication of GAPDH in neurodegenerative processes and clarifies its role in apoptotic cell death.

[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.

[Paroxysmal dystonia and multiple sclerosis]. / Dystonie paroxystique et sclérose en plaques.

INTRODUCTION: Movement disorders are uncommon in multiple sclerosis, except for tremor. Patients rarely have paroxysmal dystonia (or tonic spasm), which can be the presenting manifestation of the disease. OBSERVATIONS: Two videotaped observations are presented. The first patient was a 27-year-old woman, treated for relapsing-remitting multiple sclerosis, who presented daily several short (<1minute) paroxysms of right hemibody dystonia. Brain MRI revealed several areas of cerebral demyelination, including the posterior limb of the left internal capsule with gadolinium enhancement. These events disappeared 7 days after corticosteroid infusion. The second patient was a 62-year-old man who presented brief episodes (<1minute) of daily painful left hemibody dystonia. Three months later, similar paroxysms affecting the right hemibody including the face occurred. At times, the two hemibodies were affected simultaneously. The brain MRI showed multiple areas of white matter hyperintensity, including two symmetrical areas in the posterior limb of the internal capsules. Multiple sclerosis was diagnosed on clinical, MRI and biological data. Four days after starting corticosteroids, these paroxysmal phenomena disappeared totally. CONCLUSION: Dystonia is an under-recognized aspect of paroxysmal events during multiple sclerosis. It might involve ephaptic transmission among abnormal demyelinated neurons; this ectopic excitation can arise at variable levels of the corticospinal tract, but the analysis of reported cases and those described in this study shows that impairment of the posterior limb of the internal capsule seems to be a prevalent topography. Inflammation is likely to play a role because steroids often improve these phenomena. In this article, we review the clinical aspects, pathophysiology and outcome of paroxysmal dystonia in multiple sclerosis.

[Clinical presentation of Moroccan cases with Alzheimer's disease]. / Présentation clinique de cas marocains atteints de la maladie d'Alzheimer.

INTRODUCTION: The diagnostic approach for Alzheimer's disease is based on the presence of cerebral atrophy combined with the score of the mini-examination of the mental state. In this context, this study was conducted to assess the correlation between imaging and neuropsychological testing for cases of early-onset and late-onset Alzheimer's disease. AIM OF THE STUDY: Analysis of the clinical and paraclinical aspects of Moroccan cases with Alzheimer's disease. METHODS: Seventeen sporadic cases and 8 family cases were seen at the memory clinic of the Neurology Department of the University of Casablanca Ibn Rochd Hospital. A family history was obtained through a clinical interview of the patient and a yes or no self-reporting questionnaire from the guardian or other family member. The disease was considered familial if at least one additional first degree relative suffered from early-onset AD-type dementia. All patients underwent standard somatic neurological examination, cognitive function assessment, brain imaging and laboratory tests. Written consent was obtained from the patients and their guardians prior to the study. RESULTS: In our study of 25 individuals, the observed mean age of AD patients was 64.52 ± 9.30 and we observed a slight female predominance (56% versus 44%). In addition, we found a prevalence of AD of approximately 20%, increasing with age, in the population below 60 years of age. Approximately half of our patients (48%) had a score lower than 10 and were affected by severe insanity, while 28% were affected by moderate severe insanity and 24% were light to moderately insane. Twenty-five patients underwent neuroimaging, 18 of whom were assessed by MRI, while 7 were assessed by CT. All patients had hippocampal atrophy, which progressed to affect others brain regions. The blood tests showed no abnormalities in the 25 enrolled AD cases. DISCUSSION: Age is undoubtedly the main risk factor for AD; this is also the true for our cases where advanced age was responsible for the exponential increase of the disease's frequency; it reached a peak in the age group of 60-69 years. The AD diagnosis approach is based on the presence of cerebral atrophy combined with the score of the mini-examination of the mental state (MMSE). In our study, in addition to the MMSE, depending on the level of education, the clinician used other tests that do not necessarily require a level of education such as the BEC96, visual short-term or digital memory assessment, work memory assessment, language assessment test (DO80) and apraxia. Neuropsychological examination of the cases with a score of less than 10 showed severe cognitive impairment. The cases presented memory and language impairments, aphasia, visual spatial disorientation, decreased autonomy, executive dysfunction and praxis deficits, all major causes of severe dementia. Neuroimaging revealed hippocampal and cortical atrophy. Correlated with the other studies that aimed to establish links between brain alterations and neuropsychological disorders, we can conclude that a higher level of atrophy reflects a decrease in neuropsychological performance.

[Genetic aspects of Alzheimer's disease (Review)]. / Les aspects génétiques de la maladie d'Alzheimer (Revue).

Alzheimer's disease is a degenerative brain disorder, which concerns memory, cognition and behavior pattern. Its etiology is unknown, it is characterized by typical histological lesions: senile plaques and neuro-fibrillary tangles. Alzheimer's disease is a multifactorial pathology, characterized by interactions between genetic and environmental factors. Genetic factors concern first of all the exceptional monogenic forms, characterized by early onset (<60 years), autosomal dominant forms. Mutations of the genes coding for amyloid-ß precursor protein or preselinins 1 and 2 are involved. The much more frequent sporadic forms also have genetic factors, the best studied being the apolipoprotein E4 coding allele and some more recent genotypes which will be mentioned. No causal, only symptomatic treatments are available.

[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.

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.

[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.

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.

Chorea-ballismus in acute non-ketotic hyperglycaemia.

Diabetic patients during hyperglycaemic crises may present a rare syndrome characterised by a typical triad: unilateral involuntary movements (hemichoreahemiballism), radiological contralateral striatal abnormality, and rapid resolution of symptoms after glycae - mic correction. This study reports a series of patients showing less usual aspects and also discusses the pathophysiology of this clinical-radiological syndrome. We included in this study four patients presenting choreic or ballic involuntary movements and in whom aetiological assessment revealed frank non-ketotic hyperglycaemia, without other abnormalities that could explain the movement disorder. All the patients underwent CT or MR brain imaging. The typical triad was present in only one case. Less classical aspects were more frequently found: movement disorders revealed diabetes in two patients and one patient had generalised chorea and strictly normal neuroimaging. Correction of blood glucose was not sufficient to improve symptoms in two cases. In one, abnormal movements persisted despite treatment with tetrabenazine. The clinical, radiological and outcome spectrum of the syndrome of chorea-ballismus induced by non-ketotic hyperglycaemia is heterogeneous and not restricted to a typical triad.

[Fifteen cases of food-borne botulism in Morocco: significant diagnostic contribution of electrodiagnosis]. / Le botulisme alimentaire au Maroc, à propos de 15cas. Valeur diagnostique de l'électrophysiologie.

INTRODUCTION: Botulism is a rare but serious disease, which affects the peripheral autonomous nervous system, potentially with a fatal outcome. In Morocco, botulism is exceptional. PATIENTS AND METHOD: This was a prospective analysis of the epidemiological, clinical, neurophysiological and toxicological features of 15 cases of food-borne botulism identified among a series of 45 highly suspect cases collected in Morocco during an epidemic in August1999. RESULTS: The 15 patients (eight females, seven males) included in the protocol were aged 3 to 49 years (average 18.8 years). One-third of the cases occurred in a familial context. The clinical presentation was typical and complete in nine cases; respiratory failure was noted in four patients. Botulinum toxins were found in nine cases. Outcome was less than favorable, with total recovery in seven cases, persistence of motor sequelae in three and death in five. Electrophysiological investigations showed an incrementing response at high frequency in 73.3%, a decrement at lower frequency in 60% and low motor amplitudes in 93%. These findings constituted a very sensitive and specific triad for botulism diagnosis. DISCUSSION/CONCLUSION: These findings illustrate the gravity of botulism and the important diagnostic value of neurophysiological results, especially incrementation, which can provide a very pertinent diagnostic contribution, especially in seronegative patients.

[Painful legs and moving toes syndrome associated with lumbar radiculopathy]. / Syndrome de jambe douloureuse et orteils instables associé à une radiculopathie lombaire.

INTRODUCTION: Painful legs and moving toes (PLMT) is a rare syndrome characterized by spontaneous neuropathic pain in the lower limbs associated with peculiar involuntary movements of the toes. It has been associated with a variety of peripheral and central nervous system diseases. Pathophysiology is unclear and treatment approaches remain largely empirical. CLINICAL CASE: We report a case of a 42-year-old women with typical presentation of PLMT syndrome, associated with lumbar (L5) disc prolapse. Oxcarbazepine gave a partial improvement. CONCLUSION: Clinical presentations and etiological aspects of the PLMT syndrome are described and pathophysiological mechanisms and therapeutic possibilities discussed.

[l-arginine efficiency in MELAS syndrome. A case report]. / La l-arginine, une thérapeutique symptomatique efficace au cours d'un syndrome MELAS.

INTRODUCTION: Mitochondrial encephalomyopathy lactic acidosis and stoke-like episodes (MELAS) is a rare neurodegenerative disease caused by mutations of mitochondrial DNA. CASE REPORT: We report the case of a 12-year-old child with MELAS syndrome who presented with recurrent migraine-like headache and sudden blindness suggesting stroke-like episodes. Furthermore, he developed progressive muscular impairment with bilateral hearing loss. Serum lactate and pyruvate levels were elevated and the muscle biopsy showed an aspect of red-ragged fibers with Gomori trichrome. Brain imaging showed calcifications of basal ganglia on the CT scan and a parieto-occipital high signal on diffusion-weighted MRI. A genetic analysis was not performed but the presence of hearing loss in the patient's mother was suggestive of maternal transmission. Stroke-like episodes in the form of migraine-like headache and blindness were the patient's major complaint and did not improve despite analgesic drugs. After oral administration of l-arginine at the dose of 0.4mg/kg per day, stroke-like symptoms totally and rapidly disappeared. DISCUSSION: The efficiency of l-arginine in stroke-like episodes was initially reported then confirmed in a controlled study. The pathophysiology of stoke-like episodes and the mechanisms underlying the action of l-arginine are discussed.

[Celiac disease and ischemic stroke]. / Maladie coeliaque et accidents vasculaires cérébraux ischémiques.

INTRODUCTION: Neurological manifestations of celiac disease are various. An association with ischemic stroke is not common and has not been well documented. We report two cases. OBSERVATIONS: The first patient had experienced several transient ischemic strokes in the past 2 years and then had an acute ischemic stroke involving the territory of the right posterior cerebral artery. Investigations revealed celiac disease with no other recognizable etiology. The clinical course was marked by persistent visual aftereffects, but no new vascular event. The second patient had been followed since 1998 for celiac disease confirmed by pathology and serology tests. She was on a gluten-free diet. The patient had an ischemic stroke involving the territory of the left middle cerebral artery. Apart from a positive serology for celiac disease and iron deficiency anemia, the etiological work-up was negative. DISCUSSION: The mechanisms of vascular involvement in celiac disease are controversial. The most widely incriminated factor is autoimmune central nervous system vasculitis, in which tissue transglutaminase, the main auto-antigen contributing to maintaining the integrity of endothelium tissue, plays a major role. Other mechanisms are still debated, mainly vitamin deficiency. CONCLUSION: Being a potentially treatable cause of ischemic stroke, celiac disease must be considered as a potential etiology of stroke of unknown cause, particularly in young patients, and even without gastrointestinal manifestations.

[Cobalamin deficiency: neurological aspects in 27 cases]. / Carences en cobalamine: aspects neurologiques chez 27 patients.

INTRODUCTION: Combined medullar sclerosis, together with peripheral sensory neuropathies, is the most common neurological manifestation observed in cobalamin deficiency. Biermer's disease is the predominant cause. Other clinical and etiological aspects are nevertheless frequent, although underestimated. METHODS: This retrospective study included patients with neurological symptoms and cobalamin (B12 vitamin) deficiency confirmed by laboratory tests collected over a period of 11 years. RESULTS: Twenty-seven cases were analyzed. Mean age was 47 years and there were 11 women and 16 men. Distribution of the neurological syndromes was: combined medullar sclerosis in 18 patients (67%), sensory neuropathies in 30% of cases and sensory-motor neuropathies in 15%. One patient had fronto-subcortical dementia with good improvement after vitamin replacement. In addition, autonomic dysfunction was noted in six patients (orthostatic symptomatic hypotension and/or urinary dysfunction and/or erectile failure). Dysautonomia revealed cobalamin deficiency in three patients with a good and fast response to the cobalamin therapy in all cases. Biermer's disease was diagnosed in 17 patients (63%) and a likely syndrome of nondissociation of cobalamin in two patients. One patient had Crohn's disease and no etiology was found in seven patients. In five patients (19%), nitrous oxide undoubtedly induced decompensation of latent cobalamin deficiency; four after a general anesthesia and one by chronic professional exposure. Outcome was very good in 46% of patients after vitamin replacement, particularly if treatment was started rapidly. DISCUSSION: The findings in this series highlight the frequency of autonomic dysfunction sometimes revealing cobalamin deficiency with a fast and good response to vitamin replacement and the frequency of neurological disorders following decompensation triggered by general anesthesia using nitrous oxide in patients with latent cobalamin deficiency.

[Sturge--Weber-Klippel--Trenaunay syndrome (case report)]. / Syndrome de Sturge-Weber--Klippel-Trenaunay (une observation).

INTRODUCTION: Sturge-Weber syndrome is characterized by capillary malformations in the face, associated with leptomeningeal and choroidal venous malformations. Klippel-Trenaunay syndrome consists of the triad: capillary malformation of one leg, ipsilateral hypertrophy and varicose veins. OBSERVATION: We report the case of a 23-year-old male patient who presented a complex congenital neurocutaneous syndrome with vascular malformations involving the brain, face and limbs and associated with epilepsia and leptomeningeal calcifications. The patient fulfilled the diagnostic criteria of both vascular phacomatosis syndromes. DISCUSSION AND CONCLUSION: Similar descriptions of dual or overlapping syndromes have been published. Many show that there is no clear distinction between Klippel-Trenaunay syndrome and Sturge-Weber syndrome. There is a clinical and biological overlap. The complexity of the disease phenotypes shows that a classification based on an eponymous category does not enable resolution of the nosological problems. Some authors suggest that these vascular malformations are best described in anatomical/histological or functional terms. We report a new observation that illustrates these difficulties.