[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.

[Postherpetic abdominal pseudohernia]. / L'image du mois. Pseudo-hernie abdominale post-zostérienne.

Postherpetic neuralgia is the most feared complication of herpes zoster (HZ). The incidence increases with age. Nevertheless, around 5 % of HZ patients will experience a motor deficiency known as segmental zoster paresis (SZP) according to the affected dermatome. Abdominal pseudohernia is an exceptional clinical presentation of SZP, involving the T6-L1 dermatomas. The recognition of this rare complication of HZ prevents unnecessary explorations and interventions. There is no specific treatment but in general the evolution is favorable and spontaneous healing is observed within several months.

La névralgie post-zostérienne est la complication la plus redoutée du zona, et son incidence augmente avec l'âge. Néanmoins, environ 5 % des patients peuvent également présenter une atteinte motrice appelée parésie segmentaire zostérienne (PSZ) et dont la clinique dépend du dermatome affecté. La pseudohernie abdominale est une forme exceptionnelle de PSZ, dépendant des dermatomes T6-L1. Reconnaître cette complication rare permet d'éviter des explorations et des interventions inutiles. Il n'y a pas de traitement spécifique et, en règle générale, l'évolution est spontanément favorable en quelques mois.

[F-waves]. / L'onde F dans tous ses états.

F-waves result from the discharge of the motoneurons following their antidromic activation. The F-wave appears, as an indirect (the F-wave latency decreases when the stimulation site moves away from the muscular detection) and late response (occurring after the M response). In practice, the most useful parameter is the F-wave minimal latency, provided that at least seven distinct F-waves are evoked. When the analysis is relative either to the controlateral side, or to a former examination, this parameter is one of most sensitive in electroneuromyography. F-wave evocation implies conduction along the entire peripheral nervous system, and particularly its proximal part, which is not investigated by nervous trunks conduction velocity studies. Thus, F wave study is the most useful in plexopathies and polyradiculonevritis. In the early phase of Guillain-Barré syndrome, their absence may be the unique sign indicative of proximal conduction blocks.

Novel SACS mutation in a Belgian family with sacsin-related ataxia.

The authors describe the four patients in the first known Belgian family with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). A novel homozygous missense mutation, NM_014363.3: c.3491T>A in exon 9, of the SACS gene was identified in the present family, which results in an original amino acid of methionine to lysine substitution at amino acid residue 1164 (p.M1164K). Although the cardinal clinical features, i.e., spastic ataxia with peripheral neuropathy, in our patients were similar to those in Quebec patients, our patients exhibited some atypical clinical features, e.g., teenage-onset and absence of retinal hypermyelination. The present family is from Wallonia, and there could be shared ethnicity with the families of Charlevoix-Saguenay.

[How I explore. . . vasculitis of the peripheral nervous system]. / Comment j'explore. . . une vascularite du système nerveux périphérique.

Vasculitic neuropathies require early diagnosis and therapy since their prognosis is potentially severe. The following features are usually suggestive of a vasculitic neuropathy: painful multiple mononeuropathy with an acute/subacute course, signs and symptoms of multisystem involvement (arthritis, asthma, renal and skin involvement), biological evidence of an inflammatory syndrome. In one third of patients however, vasculitic neuropathies present as a polyneuropathy. For other patients, peripheral neuropathy is the presentation mode of a necrotizing vasculitis, in the absence of biological inflammatory syndrome. A combined muscle and nerve biopsy, will usually demonstrate the vasculitic process. Vasculitic neuropathies often have a favourable prognosis when immunosuppressive therapy (corticosteroids and/or cyclophosphamide) is initiated at an early stage.

[Advantages and limitations of the motor unit number estimation techniques]. / Analyse critique des techniques d'estimation du nombre d'unités motrices.

It is now 30 years since the first motor unit number estimation (MUNE) technique was introduced by Allan McComas as a way of providing an objective, sensitive and reproducible means of measuring the number of motor axons in living human muscle or muscle group. MUNE techniques have substantially evolved over the past decade and have been applied, with increasing frequency, to the study of age effects on motoneurone population and muscle denervating disorders such as amyotrophic lateral sclerosis (ALS), spinal muscular atrophy, poliomyelitis and different types of inherited and acquired peripheral neuropathies. In the future, one of the most important topics involving MUNE, will probably be its use in monitoring the progress of ALS patients undergoing experimental drug trials. However, among incremental, multiple point stimulation, spike-triggered averaging, F-wave analysis and statistical methods, there is no consensus about the best MUNE method. There is only a general feeling that some techniques are more valid than others. For this reason, in the present review, brief descriptions of the distinct MUNE methods are presented. In the second part of the paper, advantages and limitations (alternation, sampling errors, temporal registration etc...) of the most commonly employed procedures are considered.

[Symmetry and temporal variability of neurography]. / Symétrie et reproductibilité temporelle des données neurographiques.

UNLABELLED: The aims of the present study are to document side-to-side differences and temporal variability, between two trials (T1 and T2 at a time interval of 3 months) of nerve conduction measurements collected from 30 healthy subjects (mean age 22 +/- 2 years). METHODS: The protocol at T1 consisted of motor nerve conduction studies of median, ulnar, peroneal and tibial nerves bilaterally, with measurement of (a) motor response size (amplitude and area); (b) terminal latency; (c) minimal, mean and maximal F-wave latency; (d) motor conduction velocity; and (e) F-wave occurrence. T1 also involved sensory nerve conduction studies of median, ulnar, radial, lateral and medial cutaneous, sural and superficial peroneal nerves bilaterally, with measurement of sensory potential size (amplitude and area) and computation of sensory conduction velocity. The protocol at T2 consisted of identical measurements from the dominant side. RESULTS AND CONCLUSION: There was a negative relationship between the variability of parameters evaluating nervous conduction and the length of the nerve segment under study. Thus, the smallest side-to-side and temporal variabilities are measured for minimal F-wave latencies (on average 2-3%). The limits of symmetry and temporal variability are particularly useful for diagnosis of unilateral peripheral neuropathy or neurophysiological follow-up of patients with neuropathy, when the variability of the parameter under study is weak and when there is a high correlation between values recorded on the left and on the right or at T1 and T2. This was the case for motor response size of tibial and ulnar nerves, sensory potential size of radial nerve and minimal F-wave latencies from each studied motor nerve.

Changes in motor unit numbers in patients with ALS: a longitudinal study using the adapted multiple point stimulation method.

METHOD: The adapted multiple point stimulation (AMPS) method for calculating motor unit numbers (MUNE) was applied in 12 patients with amyotrophic lateral sclerosis (ALS) before riluzole therapy (T(0)) and again after 4, 8 and 12 months of treatment. RESULTS: Paired Student's t-test indicated a significant decrease of thenar MUNE and compound muscle action potential (CMAP) size at each 4-monthly interval, while average surface motor unit potential (SMUP) size did not change significantly over time. The rate of motor unit (MU) loss at month 4 was more than 20% in six patients (group 1) and less than 20% in six other patients (group 2). Comparison of groups 1 and 2 by Mann-Whitney U-testing indicated that percent changes in thenar MUNE and CMAP size compared to baseline were significantly different at months 4, 8 and 12, while no difference between the two groups was found for average SMUP size variations. In the group with a slow rate of MU loss, CMAP size remained stable, while in the group with a rapid rate of MU loss, there was a dramatic reduction in size of the CMAP. A positive correlation was found between percent change in thenar MUNE at T(4) and at T(12) (P < 0.001). CONCLUSION: AMPS is a useful technique to document MUNE, SMUP size and CMAP size changes over time in patients with ALS.