Extensive myelitis associated with anti-NMDA receptor antibodies.

International audienceBACKGROUND: Encephalitis with anti-N-methyl-D-aspartate receptor antibodies (anti-NMDAR-Ab) is a rapid-onset encephalitis including psychosis, seizures, various movement disorders and autonomic system disturbances. CASE PRESENTATION: We report a very unusual case of extensive myelitis associated with anti-NMDAR-Ab. MRI also revealed a hyperintense T2 lesion, non-suggestive of MS, which progressively extended, associated with periventricular gadolinium enhancement visualized on brain MRI. Ophthalmological evaluation showed subclinical right optic neuritis. The absence of anti-AQP4 antibody argued against neuromyelitis optica spectrum disorder. A slight psychomotor slowing prompted us to search for various causes of autoimmune encephalitis. Anti-NMDAR-Ab was found in cerebrospinal fluid. CONCLUSION: In patients with extensive myelitis who are seronegative for anti-AQP4 antibodies, and after other classical causes have been excluded, the hypothesis of atypical anti-NMDAR-Ab encephalitis should also be considered

Ventilatory impairment in Parkinson's disease : from symptom to objective disturbance

La maladie de Parkinson (MP) est la deuxième maladie neurodégénérative la plus fréquente. Parmi les nombreux signes cliniques rapportés par les patients et observés par les médecins, les manifestations respiratoires sont encore très peu étudiées.Premièrement, la dyspnée, signe fonctionnel invalidant et altérant la qualité de vie, semble fréquente dans la MP mais sa prévalence et ses caractéristiques (dimension perceptive et réponse émotionnelle notamment) doivent être précisées. L'objectif de l'étude DYSPARK était de mieux définir le profil des patients dyspnéiques, le retentissement de la plainte respiratoire et de corréler ses caractéristiques avec des éléments cliniques de la MP afin de mieux appréhender sa physiopathologie.Deuxièmement, les anomalies ventilatoires objectives (explorations fonctionnelles respiratoires - EFR) sont encore mal connues dans la MP, de même que leur évolution. Une altération des volumes pulmonaires ou une atteinte de la musculature respiratoire pourraient avoir un retentissement sur le cours évolutif de la maladie. L'objectif de l'analyse d'une sous-population de la cohorte PRODIGY-PARK était de déterminer de façon prospective, sur 5 ans, le cours évolutif des données en EFR et leur impact pronostique potentiel.Parkinson’s disease (PD) is the second most common neurodegenerative disease. Among the numerous signs reported by the patients and observed by the physicians, respiratory manifestations are one the least explored.Firstly, dyspnea, debilitating symptom that can impair the quality of life, seems to be frequent in PD, but its prevalence and its clinical characteristics (perceptive aspect and emotional response) need to be determined. The objective of the DYSPARK project was to define the clinical profile of dyspneic PD patients, the consequence of the shortness of breath and to correlate its clinical features with the motor and non-motor aspects of the disease.Secondly, objective ventilatory abnormalities (pulmonary function testings – PFT) and the change over time are not well defined in PD. A diminution of lung volumes or impaired respiratory muscles could influence the outcome of the disease. The aim of the analysis of a group of patients from the PRODIGY-PARK cohort was to prospectively assess (5 years follow-up) the PFT data and their possible prognostic impact

Atteinte ventilatoire dans la maladie de Parkinson : du symptôme à l’atteinte objective

Parkinson’s disease (PD) is the second most common neurodegenerative disease. Among the numerous signs reported by the patients and observed by the physicians, respiratory manifestations are one the least explored.Firstly, dyspnea, debilitating symptom that can impair the quality of life, seems to be frequent in PD, but its prevalence and its clinical characteristics (perceptive aspect and emotional response) need to be determined. The objective of the DYSPARK project was to define the clinical profile of dyspneic PD patients, the consequence of the shortness of breath and to correlate its clinical features with the motor and non-motor aspects of the disease.Secondly, objective ventilatory abnormalities (pulmonary function testings – PFT) and the change over time are not well defined in PD. A diminution of lung volumes or impaired respiratory muscles could influence the outcome of the disease. The aim of the analysis of a group of patients from the PRODIGY-PARK cohort was to prospectively assess (5 years follow-up) the PFT data and their possible prognostic impact.La maladie de Parkinson (MP) est la deuxième maladie neurodégénérative la plus fréquente. Parmi les nombreux signes cliniques rapportés par les patients et observés par les médecins, les manifestations respiratoires sont encore très peu étudiées.Premièrement, la dyspnée, signe fonctionnel invalidant et altérant la qualité de vie, semble fréquente dans la MP mais sa prévalence et ses caractéristiques (dimension perceptive et réponse émotionnelle notamment) doivent être précisées. L'objectif de l'étude DYSPARK était de mieux définir le profil des patients dyspnéiques, le retentissement de la plainte respiratoire et de corréler ses caractéristiques avec des éléments cliniques de la MP afin de mieux appréhender sa physiopathologie.Deuxièmement, les anomalies ventilatoires objectives (explorations fonctionnelles respiratoires - EFR) sont encore mal connues dans la MP, de même que leur évolution. Une altération des volumes pulmonaires ou une atteinte de la musculature respiratoire pourraient avoir un retentissement sur le cours évolutif de la maladie. L'objectif de l'analyse d'une sous-population de la cohorte PRODIGY-PARK était de déterminer de façon prospective, sur 5 ans, le cours évolutif des données en EFR et leur impact pronostique potentiel

What can we learn from fMRI capture of visual hallucinations in Parkinson’s disease?

Background: With disease progression, patients with Parkinson’s disease (PD) may havechronic visual hallucinations (VH). The mechanisms behind this invalidating non-motorsymptom remain largely unknown, namely because it is extremely difficult to capturehallucination events. This study aimed to describe the patterns of brain functional changeswhen VH occur in PD patients.Methods: Nine PD patients were enrolled because of their frequent and chronic VH (>10/day). Patients with severe cognitive decline (MMSE<18) were excluded. Patients werescanned during ON/OFF hallucinatory states and resting-state functional imaging (rs-fMRI)was performed. Data were analyzed in reference to the two-step method, which consists in:(i) a data-driven analysis of per-hallucinatory fMRI data, and (ii) selection of the componentsof interest based on a post-fMRI interview.Results: The phenomenology of VH ranged from visual spots to distorting faces. First, at theindividual level, several VH-related components of interest were identified and integrated ina second-level analysis. Using a random-effects self-organizing-group ICA, we evidencedincreased connectivity in visual networks concomitant to VH, encompassing V2, V3 and thefusiform gyri bilaterally. Interestingly, the stability of the default-mode network (DMN) wasfound positively correlated with VH severity (spearman’s rho = 0.77, p = 0.05).Conclusion: By using a method that does not need online self-report, we showed that VH inPD patients were associated with functional changes in associative visual cortices, possiblylinked with strengthened stability of resting-state networks

Early occurrence of inspiratory muscle weakness in Parkinson’s disease

<div><p>Introduction</p><p>In Parkinson’s disease (PD), respiratory insufficiency (including functional and muscle disorders) can impact dysarthria and swallowing. Most studies of this topic have been performed retrospectively in populations of patients with advanced PD. The objective of the present study was to characterize lung function (under off-drug conditions) in early-stage PD patients at baseline and then again two years later.</p><p>Methods</p><p>Forty-one early-stage PD patients (mean ± SD age: 61.7 ± 7.7; mean ± SD disease duration: 1.9 ± 1.7 years) were prospectively enrolled and compared with 36 age-matched healthy controls. Neurological evaluations and pulmonary function testing were performed in the off-drug condition at the inclusion visit and then two years later.</p><p>Results</p><p>Pulmonary function testing did not reveal any restrictive or obstructive disorders; at baseline, inspiratory muscle weakness was the only abnormality observed in the PD group (in 53.7% of the patients, vs. 25% in controls; p = 0.0105). The PD patients had a lower mean maximal inspiratory mouth pressure than controls and a lower sniff nasal inspiratory pressure. Two years after the initiation of chronic treatment with antiparkinsonian medications, the maximal inspiratory mouth pressure and the sniff nasal inspiratory pressure tended to be higher. Lastly, overall motor outcomes were not significantly worse in patients with inspiratory muscle weakness than in patients without inspiratory muscle weakness.</p><p>Conclusion</p><p>Inspiratory muscle weakness seems to be common in patients with early-stage PD, and was seen to be stable over a two-year period. Additional long-term follow-up studies are required to specify the impact of this new feature of PD.</p></div

Dealing with the diagnosis of Parkinson's disease and its implications for couple functioning in the early stage: An interpretative phenomenological analysis.

BackgroundFor couples facing Parkinson's disease, marital relationships are significantly impacted, even at the early stages of the disease. However, very few studies have explicitly explored the functioning of the couple and how both partners deal with Parkinson's disease. The aim of this study was to explore the experiences and strategies of couples facing Parkinson's disease in the early stage using dyadic interpretative phenomenological analysis.MethodsFifteen couples agreed to participate in the study. Semistructured interviews were conducted with each partner separately regarding his or her individual experience with Parkinson's disease, the couple's history, the impact of the diagnosis on the functioning of the couple, and his or her projections for the future.ResultsThree higher-order themes emerged from the analyses. The first theme, "Being tested by the diagnosis", highlights 4 dyadic configurations according to the individual's and the couple's capacity for adjustment following the diagnosis: "noncongruent", "collapsed", "relieved" and "avoidant". The second theme, "Talking about everything except the disease", underlines that communication about the disease is often avoided both within the couple and with relatives to protect the persons with Parkinson's disease or respect their wishes. The third theme, "Supporting each other", describes the different levels of harmony between the two partners in the management of daily life and symptoms and their relational impacts.ConclusionThese results allow us to better understand the experiences of both partners and to highlight the importance of promoting better acceptance of the diagnosis by persons with Parkinson's disease to allow better communication between partners and with relatives. Such support prevents disease-specific distress and facilitates better adjustment in the later stages of the disease