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Non-pharmacological complementary interventions, particularly mind-body practices, are of growing importance in the management of Parkinson's disease (PD). Among these, mindfulness meditation seems particularly effective, especially on anxiety and depression symptoms. However, current knowledge on mindfulness standardized programs in PD is still limited, particularly in France. Aiming at improving this knowledge we designed the M-PARK study in two phases. Phase 1 consisted in a French national survey to explore expectations, needs and initiatives for mindfulness meditation for PD patients. Phase 2 was a clinical trial with objectives to assess feasibility, acceptability and effects of a mindfulness (MBSR) program proposed to PD patients.

Il existe un intérêt croissant en neurologie pour les approches complémentaires basées sur la pleine conscience ayant démontré leur efficacité dans les maladies chroniques, la dépression ou l’anxiété. Nous proposons dans cet article d’en faire une revue pratique. Du point de vue neurobiologique, il a pu être observé une relation entre la pratique de la méditation, des améliorations fonctionnelles et des changements d’activité et/ou de structure dans des régions engagées dans les processus attentionnels, de régulation émotionnelle ou de conscience de soi. Malgré certaines limites méthodologiques, un nombre important d’études et de méta-analyses suggèrent une utilité de ces interventions en neurologie. Elles doivent au mieux, être proposées sous la forme de programmes structurés et encadrés par des professionnels de santé qualifiés avec une expérience de la pathologie ciblée. Une consultation préprogramme permet d’explorer des contre-indications que sont principalement les troubles psychiques sévères, aigus ou non stabilisés qui peuvent exposer à de rares effets secondaires. De manière générale, ces interventions sont sûres et efficaces sur la composante de détresse émotionnelle et psychique associées aux maladies neurologiques. Il existe cependant, certains effets plus spécifiques. Nous les passerons en revue concernant : les douleurs neuropathiques, les migraines et céphalées, la maladie d’Alzheimer, la maladie de Parkinson, la sclérose latérale amyotrophique, la sclérose en plaques, l’épilepsie, le sommeil et l’accident vasculaire cérébral. En conclusion, bien qu’ayant démontré leurs bienfaits en particulier dans la détresse psychologique ces pratiques nécessitent d’être encadrées et doivent faire l’objet de recherches constantes d’acceptabilité, d’efficacité et de coût en neurologie.

One of the objectives of the French expert centers for Parkinson's disease (NS-Park) network was to determine a consensus procedure for assessing cognitive function in patients with Parkinson's. This article presents this procedure and briefly describes the selected tests.

The association between the pattern of cortical thickness (CT) and executive dysfunction (ED) in mild cognitive impairment (MCI) and subjective cognitive complaints (SCC) is still poorly understood. We aimed to investigate the association between CT and ED in a large French cohort (MEMENTO) of 2323 participants with MCI or SCC.

L’atrofia multisistemica (AMS) è una malattia neurodegenerativa dell’adulto, di evoluzione progressiva e di prognosi grave. I criteri diagnostici sono essenzialmente clinici e permettono la diagnosi di AMS con due gradi di certezza: «possibile» e «probabile»; il carattere «certo» è, dal canto suo, apportato dall’esame anatomopatologico. Questa diagnosi si basa sulla combinazione variabile di quattro segni clinici: una sindrome parkinsoniana (P), una sindrome cerebellare (C), una disautonomia e una sindrome piramidale. La presenza di una disautonomia è indispensabile per il carattere «probabile» o «possibile» dell’AMS e, in funzione della prevalenza della sindrome parkinsoniana o della sindrome cerebellare, si definiscono due forme di AMS: l’AMS di tipo P o l’AMS di tipo C. Alcuni esami complementari consentono di apportare degli argomenti per la diagnosi, come la presenza di anomalie suggestive di AMS alla risonanza magnetica (RM) cerebrale o la presenza di una denervazione dopaminergica al Dat-Scan. La diagnosi di certezza si basa sull’esame anatomopatologico post-mortem, che mostra in particolare una degenerazione olivo-ponto-cerebellare e della via nigrostriatale, associata ad abbondanti inclusioni oligodendrogliali intracitoplasmatiche contenenti dell’alfa-sinucleina. Le risorse terapeutiche sono limitate, fatta eccezione per il trattamento sintomatico della disautonomia. È necessario un accompagnamento da parte di un’equipe pluridisciplinare per migliorare le condizioni di vita del paziente e della sua famiglia.

Multiple system atrophy (MSA) is an atypical parkinsonian syndrome including cerebellar impairment and poor response to levodopa. We assessed right hand motor activation in patients with MSA before and after an acute levodopa challenge in comparison with patients with PD and healthy volunteers (HVs).

The term “neuroacanthocytosis” is normally used to refer to autosomal recessive chorea–acanthocytosis and X-linked McLeod syndrome, but there are other movement disorders in which erythrocyte acanthocytosis may also be seen, such as Huntington disease-like 2 and pantothenate kinase-associated neurodegeneration. Disorders of serum lipoproteins such as Bassen–Kornzweig disease form a distinct group of neuroacanthocytosis syndromes in which ataxia is observed, but movement disorders are not seen. Genetic testing has enabled us to distinguish between these disorders, even when there are considerable similarities between phenotypes. Improved detection is important for accurate genetic counseling, for monitoring for complications, and, it is hoped, for implementing causal treatments, once these become available. As in other neurodegenerative conditions, animal models are a promising strategy for the development of such therapies.

To evaluate the prevalence, characteristics, and treatment of restless legs syndrome (RLS) in France.

Triple A (3A) syndrome, a rare autosomal recessive disorder, is characterized by adrenocorticotropic hormone–resistant adrenal insufficiency, achalasia of the cardia, alacrima, and variable autonomic and neurologic dysfunction. The gene responsible, AAAS, recently has been identified. We describe the neurologic phenotype of the first adult case of 3A syndrome presenting bulbospinal amyotrophy as the prominent sign in association with a homozygous nonsense mutation identified in the AAASgene.

Leg tremor during standing is a rare feature in idiopathic Parkinson's disease (PD). Tremor during standing usually has a low frequency (range, 4–6 Hz), similar to PD rest tremor frequency, and is improved by levodopa. We describe three cases of fast orthostatic tremor (FoT) of legs and trunk mimicking primary orthostatic tremor (OT) in patients treated with levodopa for PD. Asymmetrical akinetorigid syndrome was accompanied by a rest tremor in two cases. We obtained electrophysiological parameters by electromyographic (EMG) polygraphic recording after 16 hours withdrawal of antiparkinsonian treatment and at the maximal effect of levodopa in order to investigate the effect of dopaminergic stimulation upon such cases of orthostatic tremor in PD. Electrophysiological parameters of orthostatic tremor, especially frequency (range 14–18 Hz), were similar to that seen in POT. Severity of tremor was independent of seriousness and duration of PD. Levodopa had no effect either on the handicap due to OT or on the amplitude and frequency of the EMG OT activity. In contrast, mild improvement of OT was obtained with benzodiazepines in two cases and parkinsonian syndrome was levodopa‐sensitive. These findings suggest that FoT in PD would not be directly controlled by the dopaminergic system. However, increased rhythmicities in basal ganglia or in cerebello‐thalamic loops at the rapid frequencies range seen in PD could favor the emergence of a primary orthostatic tremor‐like tremor in PD patients. © 2001 Movement Disorder Society.

Among the movement disorders associated with acanthocytosis, McLeod syndrome (McKusick 314850) is the one that is best characterized on the molecular level. Its defining feature is low reactivity of Kell erythrocyte antigens. This is due to absence of membrane protein KX that forms a complex with the Kell protein. KX is coded for by the XKgene on the X‐chromosome. We present six males (aged 29 to 60 years), with proven XKmutations, to discuss the chorea associated with McLeod syndrome. The movement disorder commonly develops in the fifth decade and is progressive. It affects the limbs, the trunk and the face.

Article abstract-We examined the frequency of dementia and depressive symptomatology in 60 Parkinson's disease (PD) patients, identified in a large representative sample of the population, aged 65 years and older, living at home or in institutions in Gironde, France. Dementia, diagnosed according to DSM-III-R criteria, was present in 17.6%, and depressive symptomatology, assessed by the Center for Epidemiologic Studies-Depression Scale, was present in 32.7%. The frequency of dementia in PD increased strongly with age and was higher in institutionalized PD patients than in those living at home. PD was significantly associated with dementia for individuals living at home (odds ratio = 8.2, adjusted for age and symptoms of depression).

The characterization and localization of non-catecholaminergic cells producing dopamine after L-Dopa load have been investigated in the normal rat brain by a direct immunohistochemical labelling of amines using specific antibodies. The detection of dopamine-containing non-catecholaminergic cells has been achieved in rats given a commonly used mixture of L-Dopa plus peripheral decarboxylase inhibitor, and compared to controls. Results indicate that serotoninergic neurons tend toward a switch of their metabolism into dopamine production after L-Dopa load in a dose-dependent manner. In addition small non-aminergic cells, identified as aromatic amino-acid decarboxylase-containing cells, were observed to produce dopamine after exogenous L-Dopa load. Possible implications of such results concerning the mode of action of L-Dopa in the brain are discussed.

The aim of this work was to study l-DOPA-containing neuronal structures of the rat posterior and dorsal hypothalamus by means of immunohistochemistry using antiserum against glutaraldehyde conjugated l-DOPA. Aspects and distribution of l-DOPA immunoreaction among cells of the supramammillary nucleus and the A11, A13c and A13 cell groups are described and compared to dopamine immunoreactivity, mainly through a double colored labelling procedure employing a color modification of the DAB reaction by metallic ions. Differences between l-DOPA and dopamine stainings within cell groups as the presence of cells with predominant or exclusive l-DOPA coloration are tentatively explained under the light of previous findings using immunohistochemistry of catecholamines synthesizing enzymes and catecholamines histofluorescence.

We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa‐induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the latter. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.

Abstract: Pain is a recognized feature of idiopathic Parkinson’s disease (IPD) but has never been studied in multiple system atrophy (MSA), the commonest cause of atypical parkinsonism. We retrospectively analysed histories of pain in 100 consecutive cases of clinically probable MSA. Details were obtained from the medical records of 100 patients with MSA, comprising 82 with the striatonigral degeneration (SND) type and 18 with the olivopontocerebellar atrophy (OPCA) type of MSA. Pain was reported in 47% of the MSA patients. It was classified as rheumatic in 64% of MSA patients reporting pain, sensory in 28%, dystonic in 21%, and levodopa-related in 16%, mostly related to off-period or diphasic dystonias. There was a mixed pain syndrome in 19% of these patients. Pain was significantly more commonly reported by females (P=0.02), and by patients with levodopa-induced dyskinesias (P=0.02). No other clinical feature differentiated MSA patients who reported pain from those who did not. The mean delay between disease onset and onset of pain was 2.9 years, but pain was reported at the time of, or before, disease onset in about 30% of patients. The overall prevalence of pain in MSA was similar to that reported in IPD, but the distribution of pain categories was different.

OBJECTIVES: To evaluate the incidence, types, determinants, and consequences of sleep disorders in patients with multiple system atrophy (MSA) and determine whether their characteristics are similar to those of patients with Parkinson's disease (PD). METHODS: Information about sleep disorders was collected using a standardised questionnaire in an unselected group of 57 patients with MSA and in 62 patients with PD matched as a group for age, sex distribution, and disease duration. RESULTS: Seventy percent of patients with MSA complained of sleep disorders compared with 51% of patients with PD (p=0.03). The most commonly reported sleep disorders were sleep fragmentation (52.5%), vocalisation (60%), REM sleep behaviour disorder (47.5%), and nocturnal stridor (19%). Except for sleep fragmentation, the incidence of these disorders was significantly higher than in PD. Sleep problems tended to be associated with more severe motor symptoms, longer disease duration, depression, and longer duration of levodopa treatment. Half of patients with MSA with sleep disorders complained of daytime somnolence compared with 30% of patients with PD. Daytime somnolence was significantly associated with disease severity in MSA. CONCLUSION: This study shows that sleep disorders are more common in patients with MSA than in those with PD after the same duration of the disease, reflecting the more diffuse underlying pathological process in MSA.

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A 56-year-old woman with coeliac disease developed myoclonus of cortical origin and palatal myoclonus with lesions of subcortical white matter on magnetic resonance imaging. Myoclonus can thus be a prominent feature of coeliac disease encephalopathy. A slight vitamin E deficiency was found but does not satisfactorily explain this myoclonic encephalopathy, which remains of unknown origin.

Article abstract-A systematic review of the neurologic literature identified 433 cases of pathologically proven multiple system atrophy over a 100-year period. Earlier case reports included patients younger in age with more frequent cerebellar involvement. Mean age of onset was 54.2 years (range 31 to 78) and survival was 6.2 years (range 0.5 to 24). Survival analysis showed a secular trend from a median duration of 4.9 years for publications between 1887 and 1970 to 6.8 years between 1991 and 1994. Older age of onset was associated with shorter survival; the hazard ratio for patients with onset after 60 years was 1.8 (95% CI 1.4 to 2.3) compared with patients between 31 and 49 years. Cerebellar features were associated with marginally increased survival (6.1 years versus 5.4 years; p = 0.04). There were no differences in survival according to gender, parkinsonian, or pyramidal features or whether the patient was classified as striatonigral degeneration or olivopontocerebellar atrophy type. These results demonstrate the poor prognosis for patients with multiple system atrophy but may be biased toward the worst cases. Future research needs to recruit more representative samples.