Descriptive analysis of the French NS-Park registry: Towards a nation-wide Parkinson's disease cohort?

Introduction: Parkinson's disease (PD) is the second most common neurodegenerative disorder after Alzheimer's. The French clinical research network for PD (NS-Park) has created a national patient registry to i)report medical activity of Parkinson Expert Centers (PECs) to the Ministry of Health, ii)facilitate PD patients pre-screening for clinical trials, iii) provide a source for pharmaco-epidemiology studies.

Objective: Assess the French Parkinsonian population at a nation-wide level and discover new clinical characteristics.

Factors associated with spousal burden in Parkinson's disease.

Parkinson's disease is characterized by motor and non-motor symptoms, which can lead to progressive disability that, in turn, can lead to a burden on caregivers. Thus, the objective of this study was to determine correlations between intensity of disease burden and characteristics of patients and their spouses. The study included 38couples (patients and spouses) living at home with no severe comorbidities.

[Is nocturnal polyuria more frequent among patients with Parkinson's disease?].

Objective: Nocturia is a frequent complaint in the population of idiopathic Parkinson's disease patients (IPD). The consequences of nocturia in the IPD population are at high importance as these patients have motor problems and therefore a risk of nocturnal fall. The aim of the study was to determine the mechanism of nocturia in patients with MPI, by determining the prevalence of nocturnal polyuria (NP) in this population.

Parkinson's patient runs an ultra marathon: a case report.

Studies show that physical activity involving prolonged endurance may benefit patients with Parkinson's disease by promoting the secretion and/or availability and use of dopamine. We report the case of a Parkinson's patient who took part in an ultra-marathon to show that extreme physical activity is possible and can facilitate medical treatment with a possible positive effect on brain structures. We report the case of a 48-year-old man in the initial stages of Parkinson's disease who took part in a 100-km run.

[Impact of drug-free care in posterior cortical atrophy: Preliminary experience with a psycho-educative program].

Introduction: Criteria for the diagnosis of posterior cortical atrophy (PCA) are well established, but little is known about the impact of drug-free care for patients and caregivers.

Methods: We designed an adapted and specific psycho-educative program for four patients and their caregivers who participated in six sessions, one every 2 months.

Results: Patients and caregivers improved their knowledge about the PCA syndrome; level of anxiety was slightly reduced among caregivers.

Early onset Parkinsonism associated with an intronic SOD1 mutation.

We report on a patient belonging to a large family with autosomal-dominant amyotrophic lateral sclerosis, who developed asymmetrical akineto-rigid symptoms at 33 years of age. He had no signs of lower motor neuron disease after four years of follow-up. All seven ALS patients from this family harboured a mutation located in the fourth intron of the SOD1 gene.

Intraoperative use of the Medtronic O-arm for deep brain stimulation procedures.

The purpose of this study was to analyze the feasibility and utility of 3D imaging to help lead positioning during a deep brain stimulation (DBS) procedure. A bilateral subthalamic DBS procedure was conducted in 2 patients for idiopathic Parkinson's disease. Subthalamic nucleus targeting was based on preoperative stereotactic MRI.

[Comparison of quality of life in multiple system atrophy and Parkinson's disease].

Purpose: Quality of life (QoL) in multiple system atrophy (MSA) is thought to be poorer than in Parkinson's disease (PD), primarily because of motor impairment, autonomic dysfunction and depression. The aim of the study was to investigate QoL in 10 patients with probable MSA (parkinsonian subtype) compared with 10 PD patients matched for motor disability on UPDRS III motor score.

Methods: All patients were ambulatory and non-demented.

Use of the Visual Object and Space Perception (VOSP) test battery in two cases of posterior cortical atrophy.

Two patients presenting with predominantly dorsal posterior cortical atrophy were evaluated using the Visual Object and Space Perception (VOSP) test. The objective was to determine whether the VOSP was useful to discriminate damage to the ventral and the dorsal visual pathways. Both patients failed almost all the VOSP subtests, and the battery did not permit confirmation of the integrity of the ventral pathway.

Gait and balance disorders in Parkinson's disease: impaired active braking of the fall of centre of gravity.

Gait and balance disorders are common in Parkinson's disease (PD), but its pathophysiology is still poorly understood. Step length, antero-posterior, and vertical velocities of the center of gravity (CG) during gait initiation were analyzed in 32 controls and 32 PD patients, with and without levodopa, using a force platform. Brain volumes and mesencephalic surface area were measured in PD patients.

[Exogenous risk factors in sporadic ALS: a review of the literature].

The latest reviews of the literature devoted to the epidemiology of ALS all agree that exogenic risk factors play a role in sporadic ALS. Nevertheless, there is no convincing evidence demonstrating in a reproducible manner an association between an environmental risk factor and ALS. This discordance is mainly explained by methodological skews.

[Announcement of amyotrophic lateral sclerosis diagnosis].

Breaking the news of amyotrophic lateral sclerosis (ALS) is considered as a daunting task in most cases and is not a standardizable procedure. However, proven techniques exist to reduce the trauma to the patient. Announcing ALS falls upen the neurologist who must respect the ethical principle of the patient's independence.

Control of vertical components of gait during initiation of walking in normal adults and patients with progressive supranuclear palsy.

Gait and balance disorders are common in patients with parkinsonian syndromes, but the pathophysiology of these symptoms is still poorly understood. This study examined the initiation of gait in patients with progressive supranuclear palsy (PSP, n=10), characterized by the presence of severe postural instability, and controls (n=43). We used a force plate to measure the step length, and the antero-posterior and vertical velocities of the centre of gravity (CG) during gait initiation, in natural and fast gait conditions.

Hypermetabolism in ALS: correlations with clinical and paraclinical parameters.

Despite a reduction in fat-free mass (FFM), a hypermetabolism has been reported with an average of 10% in amyotrophic lateral sclerosis (ALS) patients as compared with a healthy population. The objectives of this study were to confirm the level of hypermetabolism determined by using indirect calorimetry in 168 patients with a probable or a definite ALS and to study correlations with survival. Consecutive evaluations of resting energy expenditure (REE) were performed from diagnosis to the proximity of death in 44 ALS patients.

Subthalamic stimulation in Parkinson disease: behavior and social adaptation.

Background: Bilateral subthalamic high-frequency stimulation significantly improves motor functions in patients with advanced forms of Parkinson disease (PD). This favorable effect contrasts with a growing number of reports that the treatment may result in psychiatric complications.

Objective: To analyze the presence of behavioral disorders and social maladjustment in PD patients successfully treated with continuous bilateral subthalamic stimulation.

[Analysis of reasons for the late diagnosis of amyotrophic lateral sclerosis].

Introduction: The mean diagnostic delay of amyotrophic lateral sclerosis (ALS) is greater than one year. Its causes are multiple, related to the affection, the patient, or medical practices.

Methods: An investigation was carried out in 77 consecutive patients, to describe their medical course since the date of the first symptoms until the diagnosis.

[Functional rating scales for amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis (ALS) is a progressive degeneration of the peripheral and central motor neurons. The principal consequence is a loss of motor functions. Evaluation of motor deficit implies an assessment of the resulting deficiency or incapacity and final disability.

Spirometer-dependence of vital capacity in ALS: validation of a portable device in 52 patients.

Since the evaluation of vital capacity (VC) needs to be carried out every three months in patients with amyotrophic lateral sclerosis (ALS), a portable spirometer would be of value in clinical practice. Over the follow-up of 52 ALS patients, we compared the values of slow vital capacity measured by two spirometers: a reference flow-metered spirometer based on a Hans-Rudolph pneumotachograph and a portable Venturi spirometer. The objectives were to analyse the overall concordance of the measurements from the two devices and determine a discordance cut-off.

[Cranial neuritis with enhancement on post-contrast MRI due to Lyme disease].

We report a case of Lyme disease presenting as an oculomotor nerve palsy and meningitis. T1-weighted postcontrast magnetic resonance images showed marked enhancement of the right third, fifth and sixth nerves. This case illustrates neuroradiological features of cranial nerves during neuroborreliosis.

Diagnostic value of nerve biopsy for atypical chronic inflammatory demyelinating polyneuropathy: evaluation of eight cases.

The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) relies primarily on clinical and electrophysiologic examination, but the nerve biopsy findings may be supportive, especially in atypical cases. In order to define the usefulness of nerve biopsy in this disease, we retrospectively studied 44 consecutive patients whom we classified as having CIDP on pathological grounds. We found that 8 of these 44 patients had pathological findings indicative of CIDP but did not meet any of the usually accepted electrophysiological criteria for its diagnosis.