[Psychological and cognitive interventions in end-of-life support of patients with amyotrophic lateral sclerosis. A review.] / Prise en charge psychologique et cognitive au cours de l'accompagnement de fin de vie de patients atteints de sclérose latérale amyotrophique. Une revue systématique.

Amyotrophic lateral sclerosis (ALS) is an incurable disease characterized by muscle atrophy leading to complete paralysis. Once diagnosed, the average life expectancy is three to five years. In this context, palliative and end-of-life care are essential, as well as the development of cognitive and/or psychological therapies to improve the quality of life of patients. In this context, we conducted a review of the pertinent literature about psychological and cognitive interventions in end-of-life support for ALS patients. We identified 504 references out of which only four studies met our inclusion criteria. Two studies focused on dignity therapy, one study on the delay between the diagnosis and the start of psychological care in a specialized centre, and one case-report on psychological therapy combined with a computer-assisted communication system. The results of these studies, although very limited, suggest that psychological interventions may improve the management and quality of life of end-of-life ALS patients. Further studies should investigate the impact of psychological support adapted to ALS, using, for example, computer-assisted communication allowing to implement these interventions in a larger number of patients and over the long term.

La sclérose latérale amyotrophique (SLA) est une maladie neurogénérative qui se caractérise notamment par une amyotrophie progressive évoluant jusqu'à la paralysie complète du patient dont l'espérance de vie est, en moyenne, de trois à cinq ans. Les soins palliatifs et le développement de thérapies pour améliorer la qualité de vie des patients sont essentiels. Dans ce cadre, nous avons réalisé une revue de la littérature portant sur les interventions psychologiques et cognitives dans la prise en charge des patients atteins de SLA en fin de vie. Nous avons identifié 504 références dont quatre rapportant des études qui répondaient aux critères d'inclusion. Deux études portaient sur la thérapie de la dignité, une sur la rapidité d'une prise en charge psychologique dans un centre spécialisé et un rapport de cas concernait une prise en charge psychologique combinée à un système de communication assistée par ordinateur. Les résultats de ces quatre études, bien que limités, suggèrent que les interventions psychologiques pourraient améliorer la qualité de vie des patients en fin de vie. De nouvelles recherches devraient être menées pour investiguer l'impact d'une prise en charge psychologique adaptée à la SLA en utilisant, par exemple, une communication assistée afin d'implémenter ces interventions sur un plus grand nombre de patients et sur le long terme.

[An illustrative case of the POEMS syndrome]. / Cas clinique. Un cas illustratif de POEMS syndrome.

POEMS syndrome is a rare and invalidating entity characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and dermatoses. The diagnosis of this condition is often late and challenging due to the heterogeneity of clinical forms. The light chains secreted by the clonal plasmocytes cause overproduction of VEGF (Vascular Endothelial Growth Factor) responsible for the appearance of the clinical manifestations of POEMS. The diagnostic approach is based on different clinical and biological criteria. Patients with a solitary plasmacytoma are candidates for radiotherapy treatment. Patients with diffuse bone involvement or bone marrow infiltration are best treated by systemic drugs. The response to treatment may take several months before clinical and biological improvement. Early diagnosis and dedicated management limit the clinico-functional impact of POEMS.

Le POEMS syndrome est une entité rare et invalidante caractérisée par une polyneuropathie, une organomégalie, une endocrinopathie, une gammapathie monoclonale et des atteintes dermatologiques. Le diagnostic de cette infection est souvent tardif et représente un véritable défi au vu de l'hétérogénéité des formes cliniques. Les chaînes légères sécrétées par les plasmocytes clonaux entraînent une surproduction de VEGF (Vascular Endothelial Growth Factor) responsable de la plupart des manifestations cliniques du POEMS. La démarche diagnostique repose, en pratique, sur des critères cliniques dont les principaux sont la polyneuropathie et la gammapathie monoclonale. Le bilan d'extension reprend le dosage du VEGF, l'électrophorèse et l'mmunofixation des protéines sériques. Un bilan radiologique permet d'objectiver des lésions osseuses ostéosclérotiques ou des adénopathies et l'électromyogramme la polyneuropathie. Les patients qui souffrent d'un plasmocytome en l'absence d'une infiltration médullaire de plasmocytes clonaux sont des candidats au traitement par radiothérapie. Les patients avec une atteinte osseuse diffuse ou une localisation médullaire recevront un traitement systémique. La réponse au traitement peut prendre plusieurs mois avant une amélioration clinique et biologique. Un diagnostic précoce et une prise en charge spécifique limitent l'impact clinico-fonctionnel du POEMS.

[Loss of balance, gait disturbance and falls in an elderly woman]. / LA VIGNETTE DIAGNOSTIQUE DE L'ÉTUDIANT. Troubles de l'équilibre, de la marche et chutes chez une patiente âgée.

We report the diagnostic workup of a 75-year old woman seen in the outpatient clinic and complaining of a very common symptom that is walking difficulty and falls. We stress the complexity of the clinical examination and of the pathophysiology of equilibrium and gait disorders. They are numerous aetiologies that are very often associated in elderly patients. The therapeutic approach is necessarily multidisciplinary, both medical and paramedical. In the particular case of the patient illustrated in this vignette, the diagnosis was myofibrillar myopathy, a rare and recently described muscle disease. This is the occasion to stress that for many rare disorders, it is the disease, but not the signs or symptoms, which is rare.

[Multifocal motor neuropathy: a retrospective study of sensory nerve conduction velocities in long-term follow-up of 21 patients]. / Neuropathie motrice multifocale: existe-t-il une altération de la conduction sensitive au long cours? Une étude rétrospective chez 21 patients.

INTRODUCTION: Multifocal motor neuropathy is a well described condition characterized by slowly progressive, predominantly distal, asymmetric limb weakness and wasting, predominantly in the arms within an anatomical distribution of individual motor nerves, with minimal or no sensory involvement. METHOD: The aim of this retrospective study was to look for a significant reduction of the amplitude of sensory potentials in a cohort of 21 patients with defined multifocal motor neuropathy according to the Workshop Report criteria [Workshop Report, 2001. 79th ENMC International Workshop. Multifocal motor neuropathy 14-15 April 2000, Hilversum. The Netherlands. Muscle Nerve 11, 309-314], within a follow-up of at least 3 years. RESULT: Thirteen patients (62%) (Group 1) had a reduction of the amplitude of at least one sensory potential, of whom four patients had abnormalities of two or more sensory potentials, while eight patients (Group 2) had no abnormality. No significant differences were found for gender, age at onset, number of involved motor nerves, CSF protein count, presence/absence of anti-GM1 serum antibodies and response to IgIV between the two groups. CONCLUSION: This study underlines the difficulty in defining criteria for multifocal motor neuropathies capable of distinguishing them from other chronic acquired demyelinating polyneuropathies, and mainly from multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, also called Lewis-Sumner's syndrome.

[Therapeutic armamentarium in neurology: the birth of a new era]. / L'arsenal thérapeutique en neurologie: une nouvelle ère voit le jour.

The field of neurology was long infamous for a lack of therapeutic options. How many of you have once thought: "Neurologists don't cure the disease, they admire it". But those days have passed into history, and the field is now vibrant with new treatments and hope even for patients with the worst neurodegenerative diseases. We summarized in the present review the latest major advances in therapeutic principles and practice for some of the most frequent chronic neurological disorders such as headaches, epilepsy, multiple sclerosis, dementias, Parkinson's disease, sleep/wake disturbances and peripheral neuropathies. We cannot cure or prevent, but we can now halt or control symptoms and disease progression to provide physical and psychological relief, and a better quality of life for patients who suffer from these otherwise devastating neurological conditions.

Guidelines for the management of epilepsy in the elderly.

Seizures starting in patients over 60 years old are frequent. Diagnosis is sometimes difficult and frequently under- or overrated. Cerebrovascular disorders are the main cause of a first seizure. Because of more frequent comorbidities, physiologic changes, and a higher sensitivity to drugs, treatment has some specificity in elderly people. The aim of this paper is to present the result of a consensus meeting held in October 2004 by a Belgian French-speaking group of epileptologists and to propose guidelines for the management and the treatment of epilepsy in elderly people.

[Neuroleptic malignant syndrome and general paralysis: a clinical case]. / Le cas clinique du mois. Syndrome malin des neuroleptiques et paralysie générale.

Described in 1960 by Jean Delay in relation to the use of haloperidol, neuroleptic malignant syndrome remains relatively rare, and poorly known by the medical profession. The emergence of the atypical antipsychotic agents and preventive measures which have become general in recent years in hospital departments using dopamine receptor antagonists has not altered the prognosis, which remains potentially fatal in approximately a quarter of cases. This article proposes a descriptive summary of this syndrome in terms of clinical and biological diagnostics as well as of evolution, epidemiology, differential diagnosis and treatment. It describes the case of a patient affected by general paralysis having developed a neuroleptic malignant syndrome, thus the hypothesis what the neuroleptic malignant syndrome preferentially arises in subjects having underlying organic or metabolic problems and provides food for thought regarding the main medical and psychiatric overlaps, the use of dopaminergic agents and the behaviour to be adopted when dealing with a patient presenting with inaugural psychiatric symptomatology.

[Diabetic peripheral neuropathy]. / La neuropathie diabétique périphérique.

Peripheral neuropathy is a very frequent consequence of diabetes mellitus. Its clinical expression is quite variable. A specific therapy is sometimes necessary. Early diagnosis of diabetic neuropathy is a cornerstone of patient's follow-up. Differential diagnosis of diabetic neuropathy is sometimes difficult from another type of neuropathy or a focal, even systemic, disease. It is mandatory to know how a diabetic neuropathy may express. Pathophysiological mechanisms involved in diabetic neuropathy are complex and interrelated. Hyperglycaemia alone, even mild or moderate, vascular disorders and dysimmune factors may be combined to induce axonal injury. Glycaemic control is the cornerstone of effective treatment for neuropathy associated with diabetes. Specific pain control and therapies of autonomic disturbances are regularly required.

[Transient ischemic attacks: a new definition]. / Les accidents ischémiques transitoires: une nouvelle définition.

According to its initial definition, which dates back more than 50 years, a transient ischemic attack (TIA) is a sudden focal neurologic deficit lasting for less than 24 hours, of presumed vascular origin, and confined to an area of the brain or eye perfused by a specific artery. Recent data on the pathophysiology of cerebral ischemia and the progress made by the imaging techniques have led an American TIA Working Group to propose a new definition which states that: "A transient ischemic attack is a brief episode of neurologic dysfunction, caused by local brain or retinal ischemia, with clinical symptoms typically lasting less than one hour, and without evidence of cerebral infarction". The advantages and limitations of this new definition, the need for an emergency medical care in the presence of a TIA, the clinical signs associated with this condition, the diagnostic work up, and the differential diagnosis are briefly discussed. A clinical example illustrates the difficulties that can be encountered in a case of TIA.