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Introduction Dans la MPI, les troubles cognitifs sont discrets et presents des l’apparition des troubles moteurs, voire meme les precedent. Les fonctions executives sont les plus concernees. Objectifs Determiner la prevalence et le choix des tests cognitifs dans la maladie de Parkinson. Evaluer la relation entre la severite des troubles cognitifs, l’etat moteur, l’âge de debut et la duree d’evolution de la MPI. Patients et methodes Etude prospective realisee au service de neurologie du CHU de Constantine durant le mois d’octobre 2015. Onze patients inclus : 4 femmes et 7 hommes parkinsoniens evoluant depuis 6 ans en moyenne. Les echelles suivantes ont ete utilisees : MMSE, MMP, MATTIS, MoCA, BREF, test d’Isaac, fluence verbale lexicale et categorielle, 5 mots de Dubois, test de l’Horloge, TMT-A et B, et le MDS-UPDRS et le 4IADL. Resultats Le MATTIS le MMSE n’etaient touches que chez 2 patients. Dans le BREF, l’inhibition etait alteree chez 7 patients et la fluence verbale lexicale chez 10, le MoCA est altere chez 10 patients, le MMP chez 7, l’IADL chez 2, les 5 mots de Dubois chez 5, le test de l’horloge chez 9, le TMT A chez 4, TMT B chez 3. Discussion Il n’y a pas de correlation entre le MMSE, le MATTIS et les troubles cognitifs legers. Le MoCA, la fluence verbale lexicale et le test de l’horloge sont les 3 tests les plus touches. Le debut tardif de la maladie concorde avec la severite des troubles cognitifs. Le MoCA et le MDS-UPDRS evoluent parallelement a la duree d’evolution de la maladie. Conclusion Les troubles cognitifs dans la maladie de Parkinson sont frequents, leur recherche doit faire partie du bilan neurologique de base. Le MoCA s’avere le plus specifique.

Introduction Pathologie chronique, la maladie de Parkinson impose des prises regulieres et nombreuses de traitements. Une mauvaise observance peut entrainer des fluctuations d’effet. Objectifs Etudier de maniere standardisee l’observance therapeutique chez des patients parkinsoniens au cours du mois precedant une hospitalisation. Patients et methodes Trente-sept patients presentant une maladie de Parkinson depuis plus de 5 ans ont complete successivement un auto-questionnaire sur differents facteurs d’observance therapeutique mesuree par le nombre d’oublis et l’automedication : duree d’evolution, sexe, âge, polymedication, nombre de prises, connaissance du traitement, troubles cognitifs, aidant, pilulier-alarme, vecu de l’oubli de traitement et contrainte ressentie. Resultats Cinquante-quatre pour cent des patients oublient des prises mais cela reste peu frequent (2,5 fois par mois). Ces oublis ne sont correles avec aucune des variables etudiees. Les patients interroges prennent en moyenne 3 molecules differentes en 6 prises par jour. Seuls 3 des 37 patients presentent des oublis significatifs. Il s’agit d’hommes, neurostimules, sans trouble cognitif, bien informes sur les traitements et leurs potentiels effets secondaires. Discussion L’adherence des patients au traitement est satisfaisante. Les oublis sont sporadiques et la bonne comprehension des traitements leur permet d’adapter les prises selon leur heure de lever, leurs activites quotidiennes et leurs symptomes. Ces resultats semblent peu en accord avec les donnees de la litterature (Daley et al., 2012). Dans notre population, seul un impact de la neurostimulation semble pouvoir expliquer une moins bonne observance. Conclusion Globalement, cette etude originale et standardisee revele une relative bonne compliance au traitement. Ces resultats preliminaires meriteraient d’etre confirmes sur une plus grande population.

Introduction Des symptomes non moteurs fluctuants au cours de la maladie de Parkinson (MP) sont frequents mais sous-estimes. Objectifs A travers cette etude, nous avons identifie les differents types de fluctuations non motrices (FNM) et leurs caracteristiques evolutives. Patients et methodes C’est une etude descriptive ayant inclus des patients suivis a la consultation externe de neurologie du CHU Fattouma Bourguiba de Monastir pour maladie de Parkinson idiopathique. Nous avons recueilli a travers une fiche les donnees epidemiologiques, cliniques, therapeutiques et evolutives de la maladie. Les signes non moteurs ont ete recherches en passant le questionnaire WOQ 19 (Wearing Off Questionnaire 19) des fluctuations non motrices de la MP. Resultats Quatre-vingt-seize patients etaient inclus ayant : âge moyen de 68,2 ans et un sex-ratio de 1,6. La majorite avaient une forme tremblante. Au total, 71,9 % avaient des fluctuations non motrices. L’anxiete constituait la FNM la plus frequente (23 %). Les troubles de la pensee et la douleur etaient rapportes chez 17 %. Nous avons note des correlations significatives entre le nombre de symptomes non moteurs (SNM) et l’âge des malades, entre la presence de FNM et la forme a predominance tremblante. Discussion Les FNM sont des manifestations frequentes au cours de la MP. Les plus decrites sont les fluctuations cognitive-psychiques, dans notre serie, l’anxiete etait le signe le plus frequent. L’âge avance, la severite des signes moteurs sont des facteurs de risque d’apparition des FNM. Nous avons trouve une relation significative entre la forme tremblante et les FNM ; a notre connaissance, cette relation n’a jamais ete decrite. Conclusion Les fluctuations non motrices (FNM) sont considerees actuellement comme des manifestations frequentes au cours de l’evolution de la maladie de Parkinson. Elles sont genantes et necessitent ainsi un depistage et une prise en charge precoce.

It has become increasingly evident over the past years that the pathological process in Parkinson's disease extends well beyond the substantia nigra and involves non-dopaminergic neurotransmitter systems that mediate motor and non-motor symptoms. In this article, both the pathophysiology and the pharmacological management of these non-dopaminergic symptoms are discussed.

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.

Gait disorders and axial symptoms are the main therapeutic challenges in advanced Parkinson's disease (PD). Gait disorders in PD are characterized by spatial and temporal dysfunction. Gait hypokinesia is the first to appear and is responsible for the decrease in velocity. A good sensitivity to the levodopa is well established. Morris et al. [Morris ME, Iansek R, Matyas TA, Summers JJ. Ability to modulate walking cadence remains intact in Parkinson's disease. J Neurol Neurosurg Psychiatry 1994a;57(12):1532-4; Morris ME, Iansek R, Matyas TA, Summers JJ. The pathogenesis of gait hypokinesia in Parkinson's disease. Brain 1994b;117(Pt. 5):1169-81; Morris ME, Iansek R, Matyas TA, Summers JJ. Stride length regulation in Parkinson's disease. Brain 1996;119:551-68] demonstrated that the ability to modulate walking cadence remains intact in PD, and could correspond to a compensatory mechanism. More advanced disease stages of the disease are characterized by abnormal temporal parameters (such as stride length variability, stride time variability and cadence elevation) which are unresponsive to levodopa therapy and may be correlated with the occurrence of falls and freezing of gait (FOG). Lastly, postural instability also results in falls and is poorly responsive to levodopa. A link between gait impairment and frontal disorders has recently been suggested. After a few years of evolution, paradoxical episodic phenomena are described: festination ("hastening gait" with rapid small, short steps) and FOG (involuntary and sudden cessation of gait). Both symptoms are often incapacitating for PD patients, because of their resultant loss of independence and their poor response to levodopa therapy. Kinematical studies of FOG revealed a decrease in velocity, stride length and an exponential increase in cadence, prior to a FOG episode. New approaches (functional MRI, wavelets...) should offer new perspectives concerning these disabling symptoms.

In the last years, several experimental biotherapies have been developed to treat Parkinson's disease. Initially, fetal dopaminergic transplants were proposed. Although a proof of concept and encouraging results have been provided, limitations of this treatment emerged over the years and the failure of controlled trials have conducted to a pause in the development of strategies based on fetal cells. Alternative approaches such as the use of retinal pigmented cells recently provided disappointing results in patients and much hope has now been reported on other sources of dopaminergic neurons such as those originating from stem cells. This strategy is however not yet ready for clinical trials in patients. Eventually, gene therapy is a new original experimental technique which has elicited several trials in the last few years some of them being promising.

The common perception that Parkinson's disease patients tend to be depressed, anxious, apathetic and harm-avoiding has currently been challenged by the recognition that they can also exhibit a hedonistic, novelty-seeking personality. Thus, Parkinson's disease patients may indulge in their passions in an irresponsible and disinhibited manner, and engage in repetitive, compulsive behaviors that may be harmful and destructive to their social or professional lives. The dopamine dysregulation syndrome includes hypersexuality, pathological gambling, and compulsive shopping; it is associated with addiction to dopaminergic medication. However, not all behavioral changes are necessarily accompanied by a dopaminergic addiction. After antiparkinson treatment is initiated, patients enter a 'honeymoon period' during which changes in mood and behavior reflect a return to the patients' premorbid personality. The increased motivation and higher level of activity in professional as well as leisure activities are considered positive changes by both the patients and their relatives. With prolonged and increased dopaminergic treatment, these positive behavioral changes can become excessive and evolve into nocturnal hyperactivity and stereotyped, repetitive and time consuming behaviors which ultimately disorganize the patient's everyday routine and herald behavioral addictions. These drug-induced behavioral changes are under-appreciated by neurologists and under-reported by the patients who neither complain about the behaviors nor understand the relationship between motivated behavior and dopaminergic medication. For these reasons, we propose a new scale for the assessment of behavior and mood to quantify and track changes related to Parkinson's disease, to dopaminergic medication, and to non-motor fluctuations. This scale is based on the concept of hypo- and hyperdopaminergic mood and behavior. The scale consists of 18 items addressing non-motor symptoms, grouped in four parts: general psychological evaluation, apathy, non-motor fluctuations and hyperdopaminergic behaviors. The rating in five points (0-4 from absent to severe) is carried out during a semi-structured interview. Open-ended questions introduce each item, allowing patients to express themselves as freely as possible. Close-ended questions permit the rating of severity and intensity. This new instrument can be used by psychologists, psychiatrists or neurologists familiar with Parkinson's disease. Designed to detect changes in mood and behavior of Parkinson's disease patients resulting either from the disease or its treatment, this tool can be used in conjunction with the neurocognitive evaluation, to help tailor the treatment of motor and non-motor symptoms to each individual's needs.

Resume Introduction La camptocormie, definie comme une inclinaison majeure du tronc sur le bassin cedant en decubitus, est reconnue comme une complication rare mais classique de la maladie de Parkinson. Observation Nous rapportons le cas d’un patient âge de 49 ans qui developpa progressivement un syndrome parkinsonien akineto-rigide et tremulant, et une camptocormie. L’imagerie par resonance magnetique des muscles dorso-lombaires, l’electromyogramme et la biopsie musculaire confirmerent l’existence d’une myosite focale paraspinale lateralisee a gauche. Discussion Plusieurs cas publies recemment permettent de decrire un tableau clinique coherent dont ce sujet est representatif : patients de sexe masculin, plutot jeunes, arrives au stade des fluctuations motrices, traites par agoniste et L-Dopa, installant en 1 an un tableau severe, tres invalidant et non modifie par les traitements anti-parkinsoniens. Conclusion Des etudes systematiques seront necessaires pour expliciter le lien qui peut unir ces differents processus pathogeniques a priori si eloignes.

Resume Les dysfonctions sexuelles chez les patients parkinsoniens sont caracterisees par une diminution des performances sexuelles ; cependant, des troubles du comportement sexuel, en particulier l’hypersexualite, sont frequemment decrits. Les comportements sexuels compulsifs ou des paraphilies peuvent aussi exister. Les facteurs de risque les plus souvent rencontres sont l’existence d’un traitement agoniste dopaminergique et/ou des antecedents de comportement compulsif sexuel. Les troubles du comportement sexuel ont ete decrits avec tous les agonistes dopaminergiques, parfois avec la L-DOPA, mais aussi avec la selegiline. Ces modifications comportementales peuvent egalement se presenter apres stimulation des noyaux de la base. Certains cas d’hypersexualite ou certaines paraphilies actives ont parfois des consequences penales. Par honte et pudeur, les patients ou leur famille parlent difficilement de ces problemes ; un interrogatoire precis sur la sexualite des patients doit donc faire partie de l’anamnese et des informations claires sur les consequences sexuelles doivent etre fournies au patient ainsi qu’a sa famille. Des modifications therapeutiques sont necessaires avec diminution ou changement du traitement antiparkinsonien, instauration d’un traitement neuroleptique atypique, voire meme antiandrogenique.

REM sleep behavior disorders (RBD) are vigorous, complex movements corresponding to enacted dreams. They may disturb sleep, and injure the patients or their bed partner. RBD are frequently associated with neurodegenerative diseases, especially synucleopathies. They can precede parkinsonism or dementia by five to 10 years. These presymptomatic RBDs are frequently associated with EEG slowing, reduced olfaction, mild visuospatial cognitive impairment, and decreased dopamine transport in functional brain imaging. In Parkinson's disease, 15 to 60% patients speak, laugh, shout, kick, punch or fight invisible enemies during REM sleep. In contrast to sleepwalkers, patients with RBD rarely stand up or walk, but can fall out of bed. Sleep monitoring indicates an imperfect abolition of muscle tone during REM sleep in these patients. RBD are probably caused by nondopaminergic pontine lesions in the REM sleep atonia system. This condition may also expose patients with Parkinson's disease to a higher risk of daytime and nighttime hallucinations, and to more frequent cognitive impairment. Interestingly, several spouses reported that they observed a sharp contrast between the slow, limited movements, and poorly intelligible, low voice of their affected spouse when awake, and the fast, vigorous movements with loud voice that the very same patient exhibited during enacted dreams. We recently demonstrated that parkinsonism indeed disappears during RBD, in a large study combining the interview of 100 couples and the sleep and video monitoring of 50 patients. The mechanism of this improvement is now being explored.

Dopamine replacement therapy in Parkinson's disease ameliorates motor symptoms. However, it has recently been recognized that a small subgroup of patients suffer motor and behavioral disturbances attributable to taking quantities of medication well beyond the dose required to treat their motor disabilities. Dopamine dysregulation syndrome can be regarded as a pattern of compulsive medication use leading to disabling motor and behavioral features. The major theories of psychostimulant addiction may help explain some of the phenomena seen in the dopamine dysregulation syndrome. In contrast to the predictable pattern of severe degeneration of ventrolateral nigral dopaminergic cells, there is a smaller and more variable loss of dopamine neurons within the ventral tegmental areas. Sensitization of ventral striatal networks to antiparkinsonian therapy and appetitive behaviors may be analogous to the neuroplastic changes in the dorsal striatum thought to contribute to the motor complications of treatment such as dyskinesias. This syndrome greatly affects patients, their families and society. Treatment is difficult; deep brain stimulation of the subthalamic nucleus may therefore prove useful in some cases.

Nonmotor fluctuations (NMF) in Parkinson's disease are nonmotor symptoms that occur in coincidence with motor fluctuations or independently. Long under-assessed, NMF are now recognized as frequent and sometimes involving a greater degree of disability than motor fluctuations. They can be classified in three categories: dysautonomic, cognitive/psychiatric and sensory/pain. Recognition of these nonmotor fluctuations as part of Parkinson's disease has important implications. Some symptoms such as dyspnea, chest pain, or abdominal pains can mimic cardiac or gastrointestinal emergencies. The underlying pathogenic mechanisms of NMF are not well known. The dopaminergic system is probably involved via modulation of other systems (serotoninergic, adrenergic) since NMF usually respond to dopaminergic treatment. Subthalamic nucleus deep brain stimulation alleviates NMF-- particularly sensory, dysautonomic and cognitive fluctuations--while psychic fluctuations respond less consistently to this treatment. The development of new instruments that enable a comprehensive and precocious assessment of NMF is important for optimized management of advanced Parkinson's disease.

Introduction Les signes non moteurs de la maladie de Parkinson sont responsables d’un declin de la qualite de vie et d’un handicap considerable. Cependant, ces manifestations restent souvent mal reconnues et insuffisamment traites. Objectifs L’etude vise a evaluer la prevalence des signes non moteurs chez les sujets suivis pour une maladie de Parkinson et a proposer un algorithme de prise en charge adapte. Methodes Etude prospective menee au service de neurologie du CHU de Fes, incluant 30 patients suivis pour une maladie de Parkinson. L’etude repose sur un questionnaire detaillant les differents symptomes non moteurs de la maladie ainsi qu’un examen clinique comportant l’UPDRS, la recherche d’une hypotension orthostatique, l’evaluation de la douleur, l’utilisation du questionnaire abrege de Beck pour la depression, le MMSE et les inventaires d’apathie et d’anxiete. Resultats Les signes sensoriels sont representes par l’hyposmie (33%) et la douleur (87 %). Les troubles dysautonomiques sont domines par l’hypersudation (80%) et les troubles uro-genitaux (73 %). L’hypersalivation est presente dans 23 % des cas et l’hypotension orthostatique dans 45 %. Les troubles du comportement du sommeil paradoxal sont rapportes dans 67 % des cas. Ils sont associes a un ou plusieurs troubles du sommeil dans 85 %. La depression est frequente (90 %). La psychose et la demence sont retrouvees respectivement chez 10 % des cas. Discussion Les signes non moteurs de la maladie de Parkinson peuvent preceder de plusieurs annees le debut des signes moteurs. Ils sont dus a l’evolution de la synucleinopathie au niveau encephalique. Des echelles d’evaluation simplifiees sont validees pour le depistage de ces signes. La prise en charge de ces symptomes repose sur des mesures d’hygiene de vie associees a un ajustement du traitement dopaminergique et un traitement medicamenteux specifique Conclusion L’amelioration de la prise en charge des patients parkinsoniens passe par l’integration de la recherche des signes non moteurs au cours de la consultation en raison de leur impact sur la qualite de vie.