Syndrome de Bing-Neel révélateur d’une maladie de Waldenström : étude d’un cas et revue de la littérature

Introduction

The most frequent neurological complication of Waldenström's macroglobulinemia is IgM-mediated polyneuropathy. Direct tumor cell infiltration of the nervous system is very rare and better known as the “Bing and Neel syndrome”. This syndrome relates usually to a meningeal or meningo-myelo-cerebral tumor infiltration.

Observation

A 54-year-old man developed a terminal cauda equina syndrome over several years. MRI disclosed lumbar roots infiltration and lumbar puncture the presence of lymphocytic meningitis with intrathecal synthesis of monoclonal IgM identical to that found in the blood. The bone biopsy revealed a lymphoplasmocytic infiltration consistent with the diagnosis of Waldenström's macroglobulinemia. The final diagnosis was meningeal and lumbar roots infiltration revealing Waldenström's macroglobulinemia. Partial remission was obtained after polychemotherapy with CHOP, rituximab and methotrexate. At the end of the treatment, the patient improved his bladder's control and was able to walk with a stick.

Discussion

We reviewed the 35 cases of “Bing and Neel syndrome” we have identified by a PubMed research. The present case report is original by the initial neurological presentation of the disease three years before diagnosis and the successful use of rituximab in the polychemotherapy regimen.     

Mélanome malin leptoméningé primitif : une étiologie rare de pachy- et leptoméningite

Introduction

Leptomeningitis and pachymeningitis are known to occur consecutive to many causes.

Observation

We report the case of a 24-year-old woman with symptoms of raised intracranial pressure and repeated switching transient hemiparesis. The magnetic resonance imaging (MRI) showed a pachyleptomeningitis. Search for a cause was negative. The pathology examination of meningeal tissue revealed a malignant melanoma, without any sign of cutaneous melanoma, leading to the diagnosis of primary leptomeningeal malignant melanoma.

Conclusion

A meningeal biopsy can enable the rare diagnosis of primary leptomeningeal malignant melanoma in a patient with unexplained pachyleptomeningitis.     

Maladie de Parkinson : une maladie à forte composante environnementale ?

Parkinson's disease (PD) is the most common cause of the parkinsonian syndromes and the most frequent neurodegenerative disease after Alzheimer's disease. Only a minority of PD cases is due to a single factor, in particular a genetic mutation. In the large majority of cases, it is considered that PD is a complex or multifactorial disease that results from the effect of multiple risk or protective factors, either genetic or environmental, and, possibly, from their interaction. Epidemiological studies, through a variety of approaches, have brought important evidence in favour of the contribution of environmental factors to the etiology of PD. In this review, we will present current evidence by focusing on specific illustrative examples.     

L’épreuve d’anticipation, une étude de cas

In the field of psychology, very few studies deal with anticipation, the latter being defined as a paradoxical conduct. This paper describes a specific instrument elaborated by Mr Berta. The examined person is asked to imagine what he would wish to become in other life. Thus, he describes this imagery life that is located between two poles; one is rejected, the other ardently desired. Usually, this double attitude is illustrated by imagery. The negative pole (i.e. what the person is fighting) is actually what he does not want to become but as he struggles he actually develops in himself this unwanted, negative pole. The writers provide a clinical vignette, a woman called Mrs B. hospitalized for a depressive state consecutive to her husband’s death. Her protocole shows that she still is strong enough to integrate the bereavement. This way of organizing the psyche between the two poles thus having recourse to deep ethic values provides with innovating psychotherapeutic strategies.     

Le traitement par apomorphine en perfusion continue sous-cutanée dans la maladie de Parkinson : analyse rétrospective d’une série de 81 patients

Background

Continuous subcutaneous infusion of apomorphine (CAI) has shown efficacy in the treatment of motor fluctuations but its place in the therapeutic arsenal remains poorly defined in terms of indication, acceptability and long-term tolerance. Indeed, few studies have been carried out with a follow-up greater than 12 months. The main objective was to assess the quality of life of Parkinson's disease (PD) patients treated with CAI. We also evaluate the effectiveness on the motor fluctuations, the long-term tolerance of this treatment with its causes of discontinuation and the treatment regimens used.

Methods

We conducted a retrospective study of 81 PD patients treated with CAI between April 2003 and June 2012. Data were collected from medical records. A repeated measures analysis of variance by the linear mixed model was used (significance level: 5%).

Results

In August 2012, 27/81 patients were still treated with CAI with a mean duration of 28 months, 46/81 discontinued CAI (9 precociously), and 8 were lost to view. We didn’t show improvement in the quality of life nor efficacy of CAI on the UPDRS IV score (P = 0.54) and dyskinesia score (P = 0.95). The CGI score patient also reflects this result with a majority response suggesting no significant change with CAI. We observed relative good cognitive and psychiatric tolerance. Adverse events were frequent but often benign. The average (± SD) rate of apomorphine was 3.15 ± 1.71 mg/h and the oral dopaminergic treatment was decreased by 37.8%.

Discussion

The results are consistent with the literature except for the lack of efficiency on motor fluctuations which may be due to the use of too small doses of apomorphine. This seems to be a leading cause of discontinuation of CAI, especially when it is associated with side effects or important constraints. For better efficiency on motor fluctuations, we recommend the use of apomorphine at higher doses to obtain an optimal continuous dopaminergic stimulation.     

Régression de sténoses intracrâniennes vertébrobasilaires symptomatiques sous corticothérapie au cours d’une maladie de Horton

Introduction

Involvement of intracranial arteries in giant cell arteritis is a rare condition but often carries a fatal prognosis. Corticosteroids seem to be insufficient to avoid ischemic cerebral complications, and could even promote the occurrence of stroke. We report the case of a patient with giant cell arteritis who experienced recurrent cerebellar stroke caused by intracranial vertebrobasilar stenoses with a favorable outcome following treatment.

Case report

A 77-year-old woman presented with a 3-month history of impaired general condition. She had new-onset headaches, jaw claudication and transient vertigo, especially when she woke-up. The brain MRI showed a recent cerebellar infarction. One week later, she was hospitalized for a clinical deterioration related to a recurrent cerebellar stroke caused by intracranial vertebro-basilar stenoses. Giant cell arteritis was confirmed on the temporal artery biopsy. A treatment with high-dose oral corticosteroids was begun associated with an intensive antiplatelet therapy. The clinical outcome was favorable with rapid improvement of gait imbalance together with a complete radiological regression of the intracranial stenoses.

Conclusion

Ischemic stroke in giant cell intracranial arteritis is a severe condition without a well-defined treatment. Corticosteroid therapy improves intracranial stenoses caused by vasculitis but should be initially associated with an intensive antithrombotic therapy to avoid early recurrence of cerebral infarcts.     

Comparaison de la qualité de vie de patients atteints d’atrophie multisystématisée et de maladie de Parkinson

Purpose

Quality of life (QoL) in multiple system atrophy (MSA) is thought to be poorer than in Parkinson's disease (PD), primarily because of motor impairment, autonomic dysfunction and depression. The aim of the study was to investigate QoL in 10 patients with probable MSA (parkinsonian subtype) compared with 10 PD patients matched for motor disability on UPDRS III motor score.

Methods

All patients were ambulatory and non-demented. Mean durations of disease in MSA and PD patients were respectively 3.6 and 9.0 years. QoL was assessed using the SF-36 health-related questionnaire and a life satisfaction visual analogue scale. Patients were also evaluated for cognitive function (Mattis Dementia Rating Scale [Mattis DRS], Wisconsin Card Sorting Test [WCST], Stroop, Fluencies), depression (Beck Depression Inventory-II [BDI-II]), apathy (Modified Apathy Evaluation Scale) and were screened for non-motor symptoms (NMS Quest).

Results

The only difference in QoL between MSA and PD patients matched for motor disability was that the SF-36 vitality subscore was more impaired in MSA and negatively correlated with interference index on Stroop word colour testing. Depression and non-motor symptoms were associated with poorer QoL in both groups. Among MSA patients, cognitive impairment (Stroop interference index) and apathy also had a negative impact.

Conclusion

There was no major difference in QoL between MSA and PD patients matched for motor disability with a disease duration about 5 years longer. The SF-36 vitality subscore was more impaired in MSA and associated with interference sensitivity.     

Un tremblement d’attitude révélant une maladie de Huntington

Introduction

Initial manifestations of Huntington's disease (HD) are varied and chorea is not always the first motor symptom.

Case report

We report the case of a 44-year-old woman, with a family history of HD, who presented isolated head and upper limbs tremor for 4 years. Genetic testing confirmed the diagnosis of HD and no cause of secondary postural tremor was found. Propanolol was introduced with success.

Conclusion

This kind of presentation is unusual and has mostly been reported in the juvenile form of HD.     

La maladie de Parkinson idiopathique : une maladie métabolique ?

Parkinson's disease is a neurodegenerative disorder clinically characterized by motor impairments (tremor, bradykinesia, rigidity and postural instability) associated or not with non-motor complications (cognitive disorders, dysautonomia). Most of patients loose weight during evolution of their disease. Dysregulations of hypothalamus, which is considered as the regulatory center of satiety and energy metabolism, could play a major role in this phenomenon. Deep brain stimulation of the subthalamic nucleus (NST) is an effective method to treat patients with advanced Parkinson's disease providing marked improvement of motor impairments. This chirurgical procedure also induces a rapid and strong body weight gain and sometimes obesity. This post-operative weight gain, which exceeds largely weight lost recorded in non-operated patient, could be responsible of metabolic disorders (such as diabetes) and cardiovascular diseases. This review describes body weight variations generated by Parkinson’ disease and deep brain stimulation of the NST, and focuses on metabolic disorders capable to explain them. Finally, this review emphasizes on the importance of an adequate nutritional follow up care for parkinsonian patient.     

Profils de personnalité et fonctionnement émotionnel dans les sports extrêmes : à propos d’une étude exploratoire chez des BASE-jumpers

This study examines relations between emotional factors (anxiety and depression), normal and pathological personality, and risk-taking behavior in 11 BASE-jumpers comparing to a control group (n = 11).

Method

All the subjects have been evaluated with self-report questionnaires measuring their emotional states before and after the jump, their involvement in risk-taking behavior, sensation seeking, personality disorders, anxious and depressive symptomatology. Results show a significant effect of BASE-jump practice on emotional state. They have higher scores on the thrill and adventure seeking subscale, they show more drug consumption and more accidents than control group. We found also clinical elements of pathological personality solely from cluster B of the DSM-IV-TR classification among BASE-jumpers. Emotional factors are correlated with borderline personality in this same group.

Discussion

We analyzed those results depending on emotional and behavioral dysregulation, and their specifics involvement in borderline personality, risk-taking behaviors, and also addiction. Sensation seeking could be especially reinforced on the base of a borderline personality with the possibility of a build-up of risk which may put individuals addict to it.     

Le suicide dans la maladie de Huntington : état actuel des connaissances

Objectives

In 1872, George Huntington was the first to describe a genetic disease combining three types of disorder symptoms viz: motor, cognitive - evolving to cortical dementia and psychiatric. The purpose of this paper is to provide a selective review of the major issues and findings concerning suicide in Huntington's disease. The aim was to understand why patients with Huntington's disease have a higher suicide rate than those suffering from other neuro-developmental diseases.

Materials and methods

The present review is based on a MEDLINE survey of the relevant literature. The terms used in the search were: “Suicide”, “predictive genetic testing”, “suicidal risk factors” and “suicide attempt” all in combination with “Huntington's disease”. All abstracts were read and potentially relevant articles were examined in full. Various other important cross-references were included.

Results

Most of the authors found that suicidal occurrence in Huntington patients was four times higher than that in the general population. No specific individual risk factor was found except a lack of offspring and of psychological support. This review also considered the patient's attitude towards predictive genetic testing and its results. Reactions varied from psychological relief to paradoxical suicidal resurgence.

Conclusion

This paper emphasizes the need of appropriate psychiatric care for Huntington's patients in order to try to prevent suicidal behaviour.     

Les troubles anxiodépressifs dans la maladie de Parkinson : une revue de la littérature

For a long time, depression and anxiety have been neglected in Parkinson’s disease in favour of motor impairments. Since the 70’s and the beginning of the dopatherapy, the psychopathological perturbations are more and more considered, including by the neurologists. The aim of this study is to review the main studies related to depression and anxiety in Parkinson’s disease. Four main topics are tackled in this review: The prevalence, the etiology and the semiology of the depression as well as the anxiety disorders; the assessment tools and methodological problems for assessing these troubles. Beside these main subjects, we adress some interesting questions, which concern patients as well as clinicians, like the relationship between the depression and/or anxiety and the motor impairments, the disease duration and the type of the Parkinson’s disease. We tackle also the question of the relationship between the depression and/or anxiety and the cognitive troubles and we focus about the main types of cognitive impairment found in Parkinson’s disease with depression and anxiety. We review some studies, which found a link between psychic disorders and depression. It is now obvious that the deep brain stimulation has become an efficient treatment of motor symptoms. In this review, we focus on the effect of the deep brain stimulation on depression. We also discuss the different treatment available for depression and anxiety in Parkinson’s disease. Because of their frequency and their impact on the quality of life, it is particularly important to give a specific attention to these troubles in Parkinson’s disease.