[Medico-economic assessment of the Pontoise Hospital stroke unit]. / Évaluation médico-économique de l'unité neurovasculaire du centre hospitalier de Pontoise.

INTRODUCTION: Annually, approximately 120,000 people in France have a stroke. Various controlled studies have pointed out the benefits of treatment in a stroke unit (SU). The objective of this study was to evaluate, from a medical point of view, the economic impact of the Pontoise Hospital SU. PATIENTS AND METHODS: Based on the national cost study (NCS [étude nationale des coûts: ENC]) we analyzed data of five diagnosis related groups (DRG) which have a principle diagnosis in relation with stroke. This work was limited to strokes and transient ischemic events in adults and excluded sub-arachnoid hemorrhage. Medical and economic parameters were collected over the period from January to October 2006 and compared with those of the same period in 2005, that is to say before the opening of the SU. RESULTS: Three hundred and twenty-three hospital stays occurred between January 1st and October 31st, 2006 and 216 during the same time period before the opening of the SU, an increase of approximately 50% of all stroke-related admissions in our hospital. The number of stays carried out in the neurology unit increased by 29%. There was no significant difference between the two periods regarding age (median 69 versus 70 years) and sex- ratio. Average length of stay (ALS) was the same (9 days). There were no significant differences concerning the death rate (5.6% versus 6.2%) and that of discharge to home (44.6% versus 44.4%). The cost by stay in 2006 was 3534 euros [median; min 664-max 57,542] versus 3541 euros in 2005 [681-35,149] (p=0.57). Analysis by DRG highlighted an increase in the cost for serious strokes, cerebral infarctions and hemorrhages. For transitory ischemic events, the cost and the ALS decreased. CONCLUSION: After the opening of the SU, there was an increase in the activity without an increase in the total cost. This could be related in part to the limited means allocated to the stroke unit at its opening (in particular medical staff). The NCS can be used to evaluate the activity of a stroke unit. This work could be completed on a larger number of units or in several units of different size.

[Multiple system atrophy: prognostic factors in the "MSA-Aquitaine" cohort]. / Atrophie multisystématisée: survie et facteurs pronostiques dans la cohorte "MSA-Aquitaine".

INTRODUCTION: Multiple system atrophy (MSA) is a common cause of atypical parkinsonism, of poor prognosis. MSA is associated with short survival but data stemming from clinical or pathological studies are sparse and contrasted. Factors predicting survival in MSA are not fully established. We investigated the survival and prognostic factors of MSA in the cohort "MSA-Aquitaine". METHODS: This was a retrospective study of an unselected cohort of patients included throughout Aquitaine based on the Consensus Conference statement concerning MSA diagnostic criteria, with prospective follow-up on mortality. All patients received a standard clinical examination and disease history was collected through medical records and interviews of patients. Survival was ascertained by telephonic calls. RESULTS: From 1 November 1998 to 1 April 2002, we diagnosed 86 patients (43 men and 43 women) with "probable" or "possible" MSA. Median survival from study inclusion was 2.4 years and was 10.2 years from clinical onset, very similar to the other series. Low age at study, diabetes, dysphagia, Hoehn and Yahr stage 5 can predict shorter survival in patients with MSA. CONCLUSION: We confirm that the prognosis for MSA patients is poor and that some factors may predict shorter survival.

The European Multiple System Atrophy-Study Group (EMSA-SG).

Introduction. The European Multiple System Atrophy-Study Group (EMSA-SG) is an academic network comprising 23 centers across Europe and Israel that has constituted itself already in January 1999. This international forum of established experts under the guidance of the University Hospital of Innsbruck as coordinating center is supported by the 5th framework program of the European Union since March 2001 (QLK6-CT-2000-00661). Objectives. Primary goals of the network include (1) a central Registry for European multiple system atrophy (MSA) patients, (2) a decentralized DNA Bank, (3) the development and validation of the novel Unified MSA Rating Scale (UMSARS), (4) the conduction of a Natural History Study (NHS), and (5) the planning or implementation of interventional therapeutic trials. Methods. The EMSA-SG Registry is a computerized data bank localized at the coordinating centre in Innsbruck collecting diagnostic and therapeutic data of MSA patients. Blood samples of patients and controls are recruited into the DNA Bank. The UMSARS is a novel specific rating instrument that has been developed and validated by the EMSA-SG. The NHS comprises assessments of basic anthropometric data as well as a range of scales including the UMSARS, Unified Parkinson's Disease Rating Scale (UPDRS), measures of global disability, Red Flag list, MMSE (Mini Mental State Examination), quality of live measures, i.e. EuroQoL 5D (EQ-5D) and Medical Outcome Study Short Form (SF-36) as well as the Beck Depression Inventory (BDI). In a subgroup of patients dysautonomic features are recorded in detail using the Queen Square Cardiovascular Autonomic Function Test Battery, the Composite Autonomic Symptom Scale (COMPASS) and measurements of residual urinary volume. Most of these measures are repeated at 6-monthly follow up visits for a total study period of 24 months. Surrogate markers of the disease progression are identified by the EMSA-SG using magnetic resonance and diffusion weighted imaging (MRI and DWI, respectively). Results. 412 patients have been recruited into the Registry so far. Probable MSA-P was the most common diagnosis (49% of cases). 507 patients donated DNA for research. 131 patients have been recruited into the NHS. There was a rapid deterioration of the motor disorder (in particular akinesia) by 26.1% of the UMSARS II, and - to a lesser degree - of activities of daily living by 16.8% of the UMSARS I in relation to the respective baseline scores. Motor progression was associated with low motor or global disability as well as low akinesia or cerebellar subscores at baseline. Mental function did not deteriorate during this short follow up period. Conclusion. For the first time, prospective data concerning disease progression are available. Such data about the natural history and prognosis of MSA as well as surrogate markers of disease process allow planning and implementation of multi-centre phase II/III neuroprotective intervention trials within the next years more effectively. Indeed, a trial on growth hormone in MSA has just been completed, and another on minocycline will be completed by the end of this year.

[Stroke-like presentation of cerebral lymphoma]. / Lymphome cérébral primitif à présentation pseudovasculaire.

INTRODUCTION: The clinical presentation of primary cerebral lymphoma can take on many forms. CASE REPORT: We report the case of a patient who experienced recurrent neurological events mimicking stroke with normal brain MRI. A late performed MRI showed a mesencephalic lesion. A biopsy was obtained and led to the diagnosis of primary B cell lymphoma. CONCLUSION: This observation illustrates the diagnostic challenge of this rare disorder with a poor prognosis.

[Guillain-Barré syndrome secondary to cranial surgery: direct or fortuitous relationship?]. / Syndrome de Guillain-Barré secondaire à une craniectomie: association fortuite ou relation directe?

The Guillain-Barré syndrome, an acute polyradiculonevritis (PRN), usually appears after a post-infectious immuno-allergic reaction. We report a case of acute PRN following cranial surgery. Post-surgical Guillain-barré syndrome is rarely described. We reviewed the pathophysiological mechanisms possibly involved in this disease that have to be evoked in patients with abnormal post-surgical neurological symptoms.

Epidemiology of multiple system atrophy: a prevalence and pilot risk factor study in Aquitaine, France.

We investigated the prevalence of multiple system atrophy (MSA) in Gironde, France, through a network of 120 public and private specialists and assessed the relationship between some environmental factors and MSA in a case-control study involving 50 MSA patients, 50 Parkinson's disease (PD) patients and 50 healthy controls. The occupational exposure to pesticides was evaluated through a job-exposure matrix. On prevalence day (November 1, 1998), the crude prevalence of MSA in Gironde was 1.94/100,000 inhabitants. We found no significant relationship between occupational exposure to pesticides and MSA. PD patients were significantly less frequently ever-smokers than controls and the same tendency was observed for MSA patients. We also described the clinical features that heralded the disease among this nonselected population.

[Progressive supranuclear palsy: a clinical, natural history and disability study]. / Paralysie supranucléaire progressive: étude clinique, histoire naturelle et progression du handicap.

We studied the clinical features, the natural history and disability in 47 progressive supranuclear palsy patients and brain imaging aspects by routinely performed MRI in a subgroup of 25. Unexplained falls together with atypical parkinsonism (symmetric, levodopa unresponsive without resting tremor) are good clinical pointers of the early diagnosis, since they occurred within the first year. Cognitive slowness and unspecific visual complains are also early symptoms, while usual cardinal signs such as supranuclear palsy are more delayed. Blepharospasm and eyelid opening apraxia as well as deep sighs are also quite characteristic clinical features (1/3 of cases). Cardinal signs (falls, pseudobulbar signs, supranuclear gaze palsy) worsened rapidly (20 to 30 months) towards a major disability. In the 20 patients deceased during follow-up, the mean survival time was about 5 years. The MRI study showed typical cortical fronto-temporo-parietal atrophy, mesencephalic and quadrigeminal plate atrophy with third ventricle dilatation. In conclusion, unexplained falls associated with atypical parkinsonism are contributive for the early clinical diagnosis. Non specific visual complains could be useful pointers in the absence of supranuclear ophthalmoplegia. MRI contributes to the clinical diagnosis even in the first 3 years of the disease course.

Routine MRI for the differential diagnosis of Parkinson's disease, MSA, PSP, and CBD.

We assessed the usefulness of routine MRI for the differential diagnosis of Parkinson's disease (PD) with "atypical" parkinsonian syndromes in everyday clinical practice. We studied routinely performed MRI in PD (n = 32), multiple system atrophy (MSA, n = 28), progressive supranuclear palsy (PSP, n = 30), and corticobasal degeneration (CBD, n = 26). From a preliminary analysis of 26 items, 4 independent investigators rated 11 easily recognizable MRI pointers organized as a simple scoring system. The frequency, severity and inter-rater agreement were determined. The total severity score was subdivided into "cortical", "putaminal", "midbrain", and "pontocerebellar" scores. The frequency of putaminal involvement (100%) and vermian cerebellar atrophy (45%) was significantly higher in MSA, but that of cortical atrophy (50%), midbrain atrophy and 3(rd) ventricle enlargement (75%) was higher in PSP and CBD. The median total score fairly differentiated "atypical" parkinsonian syndromes from PD (positive predictive value-PPV-90%). However, the median total score was unable to differentiate atypical parkinsonian syndromes each other. The "cortical" score distinguished CBD and PSP from MSA with a fair PPV (>90%). The PPV of the "putaminal" score was high (70%) for the differential diagnosis of MSA with PSP and CBD. The "midbrain" score was significantly higher in PSP and CBD compared to MSA. These results are in accordance with the underlying pathology found in these disorders and demonstrate that a simple MRI scoring procedure may help the neurologist to differentiate primary causes of parkinsonism in everyday practice.

Sleep disorders and their determinants in multiple system atrophy.

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.