L'incidence annuelle du syndrome de Guillain-Barre est de 1,5/100000 habitants La mortalite actuelle est estimee a environ 5 % d'apres des essais therapeutiques recents, bien conduits Dix pour cent des malades gardent des sequelles motrices tres invalidantes un an apres le debut des premiers signes neurologiques La prise en charge de ces malades necessite des equipes entrainees, multidisciplinaires, pouvant pratiquer l'ensemble des therapeutiques specifiques La corticotherapie per os'ou par voie intraveineuse est inefficace Les echanges plasmatiques sont le premier traitement dont l'efficacite a ete demontree par rapport a un groupe controle Les indications sont maintenant mieux connues Les formes benignes (marche possible) beneficient de 2 echanges plasmatiques; 2 echanges supplementaires sont realises en cas d'aggravation Dans les formes intermediaires (marche impossible) et les formes severes (recours a la ventilation mecanique), 4 echanges plasmatiques sont conseilles Il n'est pas utile d'augmenter leur nombre dans les formes severes ou en cas d'absence d'amelioration De fortes doses d'immunoglobulines donnees par voie intraveineuse (lq IV) [0,4 g/kg/j pendant 5 jours] sont aussi efficaces que les echanges plasmatiques dans les formes intermediaires et severes Dans ces formes, le choix entre Ig IV et echanges plasmatiques depend des contre-indications respectives de ces traitements et de leur faisabilite Les travaux en cours ont comme objectif de mieux preciser les indications respectives des echanges plasmatiques et des lq IV dans des formes de gravite differente, leur morbidite comparee, la dose optimale des lq IV

[Guillain-Barré syndrome].

Evidence-based guidelines on the therapeutic use of transcranial direct current stimulation (tDCS)

Cognitive impairment in patients with Parkinson's disease is gaining increased clinical significance owing to the relative success of therapeutic approaches to the motor symptoms of this disorder. Early investigations contributed to the concept of subcortical dementia associated with bradyphrenia and cognitive rigidity. For cognition in parkinsonian disorders, this notion developed into the concept of mild cognitive impairment and fronto-executive dysfunction in particular, driven mainly by dopaminergic dysmodulation and manifesting as deficits in flexibility, planning, working memory, and reinforcement learning. However, patients with Parkinson's disease could also develop a syndrome of dementia that might depend on non-dopaminergic, cholinergic cortical dysfunction. Recent findings, supplemented by advances in neuroimaging and genetic research, reveal substantial heterogeneity in the range of cognitive deficits in patients with Parkinson's disease. Remediation and management prospects for these cognitive deficits are based on neuropharmacological and cognitive rehabilitation approaches.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(10)70212-X.pdf

Neuropsychological and clinical heterogeneity of cognitive impairment and dementia in patients with Parkinson's disease

Synaptic dysfunction is a key pathophysiological hallmark in several neurodegenerative disorders. In this Review, Lu and colleagues consider a 'synaptic repair'-based therapy for neurodegenerative diseases that targets pathophysiology rather than pathogenesis and discuss BDNF as a potential synaptic repair molecule.

BDNF-based synaptic repair as a disease-modifying strategy for neurodegenerative diseases

Evidence that transcranial direct current stimulation (tDCS) generates little-to-no reliable neurophysiologic effect beyond MEP amplitude modulation in healthy human subjects: A systematic review

Does Transcranial Magnetic Stimulation (TMS) primarily target premotor or motor cortex in precentral gyrus

Aim of this study is to identify factors contributing the occurrence of neck lateral shift (LS) in patients with cervical dystonia (CD). A retrospective analysis focused on the treatment with botulinum toxin (BTX) was conducted on 38 consecutive idiopathic CD patients comparing subjects with and without LS. The main result was the evidence of a significantly higher BTX inter-side dose difference in patients with LS suggesting that this uncommon phenotype may be an artifact of chronic therapy with BTX.

The occurrence of lateral shift in cervical dystonia.

Background: Plasticity of the primary motor cortex (M1) has a critical role in motor control and learning. The cerebellum facilitates these functions using sensory feedback. Objective: We investigated how cerebellar degeneration influences the plasticity of the M1 by using PAS (paired associative stimulation) technique. PAS involves repeated pairs of electrical stimuli to the median nerve and transcranial magnetic stimulation (TMS) of the motor cortex. If the interval between peripheral and TMS stimulation is around 21e25 ms, corticospinal excitability is increased via a long term potentiation (LTP)-like effect within M1. Our aims were: (i) to explore the presence of a timespecifi ci nfluence of cerebellar degeneration on human associative plasticity; (ii) to evaluate the role played by somatosensory pathway on cerebellar modulation of sensory-motor plasticity. Methods: We studied 10 patients with pure cerebellar atrophy and 10 age-matched healthy subjects. Motor-evoked-potentials amplitudes, short-afferent inhibition (SAI), motor thresholds, I/O curves, somatosensory-evoked-potential (SEP) were measured before, just after and 30 min after PAS at ISIs (interstimulus intervals) of 21.5 and 25 ms. Results: Cerebellar patients show a selective lack of LTP-like effect induced by PAS25 ms, but not at 21.5 ms. SAI was overall not truly modulated by PAS but clearly differed between cerebellar patients and healthy subjects for ISIs around 25 ms (þ6 ms and þ8 ms) (P < 0.01). SEPs showed the amplitude of P25 wave was markedly reduced in patients with a more severe clinical and radiological impairment of cerebellum. Conclusions: Cerebellar patients have an altered capability of cerebellar filtering or processing of timespecific incoming sensory volleys, influencing the plasticity of M1.

The Effect of Cerebellar Degeneration on Human

In Charcot-Marie-Tooth type 1A (CMT1A) patients, daily life is mainly influenced by mobility and ambulation dysfunctions. The aim of our work was to evaluate the perception of disturbances that mostly impact on daily life in CMT1A patients and its difference on the basis of age, gender, disability, and quality of life. Forty-one CMT1A patients underwent neurological assessment focused on establishing clinical disability through the Charcot-Marie-Tooth Neuropathy Score (CMTNS) and quality of life through the Short Form-36 (SF-36) questionnaire. We identified from CMT disturbances 5 categories [weakness in lower limbs (WLL), weakness in upper limbs (WUL), skeletal deformities (SD), sensory symptoms (SS), balance (B)] and patients classified the categories from the highest to the lowest impact on daily life (1: highest; 5: lowest). Ranking of the 5 categories, in the overall sample and in the different subgroups (dividing by gender, median of age and disease duration, CMTNS, domains of SF-36), was obtained and differences among subgroups were assessed using a bootstrap approach. Rank analysis showed that WLL was the most important disturbance on daily life whereas WUL had the lowest impact. In the older CMT1A group, the most important disturbance on daily life was B that was also the most relevant disturbance in patients with a greater disability. SD influenced daily life in younger patients. SS had less impact on daily life, with the exception of patients with a milder disability. Our findings demonstrated that the perception of disturbances that mostly impact on CMT1A patients’ daily life changes over the lifetime and with degree of disability.

/pdf/the-impact-of-symptoms-on-daily-life-as-perceived-by-he3x06uk75.pdf

Raffaele Dubbioso

Papers

Does Transcranial Magnetic Stimulation (TMS) primarily target premotor or motor cortex in precentral gyrus

The occurrence of lateral shift in cervical dystonia.

The Effect of Cerebellar Degeneration on Human

The impact of symptoms on daily life as perceived by patients with Charcot-Marie-Tooth type 1A disease

Thermosensitive hereditary neuropathy with liability to pressure palsy.