Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients.

BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system inflammatory disease. OBJECTIVE: To describe the disease course of CLIPPERS. DESIGN: A nationwide study was implemented to collect clinical, magnetic resonance imaging, cerebrospinal fluid, and brain biopsy specimen characteristics of patients with CLIPPERS. SETTING: Academic research. PATIENTS: Twelve patients with CLIPPERS. MAIN OUTCOME MEASURES: The therapeutic management of CLIPPERS was evaluated. RESULTS: Among 12 patients, 42 relapses were analyzed. Relapses lasted a mean duration of 2.5 months, manifested frequent cerebellar ataxia and diplopia, and were associated with a mean Expanded Disability Status Scale (EDSS) score of 4. Besides typical findings of CLIPPERS, magnetic resonance imaging showed brainstem mass effect in 5 patients, extensive myelitis in 3 patients, and closed ring enhancement in 1 patient. Inconstant oligoclonal bands were found on cerebrospinal fluid investigation in 4 patients, with an increased T-cell ratio of CD4 to CD8. Among 7 available brain biopsy specimens, staining was positive for perivascular CD4 T lymphocytes in 5 samples. Thirty-eight of 42 relapses were treated with pulse corticosteroid therapy, which led to improvement, with a mean residual EDSS score of 1.9 (range, 0-7). In 1 patient with untreated relapses, scores on the EDSS progressively increased to a score of 10 at death. Among 5 patients without long-term corticosteroid therapy, the mean annualized relapse rate was 0.5 (range, 0.25-2.8). Among 7 patients taking oral corticosteroids, no relapses occurred in those whose daily dose was 20 mg or higher. No progressive course of CLIPPERS was observed. Four patients with a final EDSS score of 4 or higher had experienced previous severe relapses (EDSS score, ≥5) and brainstem and spinal cord atrophy. CONCLUSIONS: CLIPPERS is a relapsing-remitting disorder without progressive forms. Long-term disability is correlated with the severity of previous relapses. Further studies are needed to confirm that prolonged corticosteroid therapy prevents further relapses.journal article2012 Julimporte

Conséquences motrices et cognitives de la stimulation bilatérale des noyaux sous-thalamiques chez les patients parkinsoniens opérés au CHU de Rouen

Objectifs: Les objectifs de ce travail prospectif réalisé sur la période de janvier 2004 à février 2009 au CHU de Rouen étaient : d'évaluer les conséquences motrices et cognitives de la stimulation bilatérale des NST chez les patients parkinsoniens 6 mois après l'intervention et de recenser les complications chirurgicales, post-opératoires et psychiatriques de la stimulation bilatérale des NST chez ces mêmes patients. Patients et méthodes. Les conséquences motrices de la stimulation bilatérale des NST étaient évaluée en comparant les scores UPDRS II, III et IV pré et post-opératoires à 6 mois. Le pourcentage d'amélioration des performances motrices était calculé à partir du score UPDRS off dopa pré-opératoire. L'évaluation neuro-psychologique pré et post opératoire comprenait un MMS, une échelle de Mattis, une MADRS, des épreuves de fluence verbale, une épreuve de Stroop, un test de Wisconsin, un test de Grober et Buschke, un Serial ordering et un test de mémoire des chiffres. Le suivi régulier des patients permettait de recueillir les complications post-opératoires. Résultats. Soixante deux patients ont bénéficié d'une stimulation cérébrale profonde dans le cadre d'une maladie de Parkinson dans notre centre. Quarante neuf (35 hommes, 14 femmes) ont eu une évaluation pré et post-opératoire à 6 mois. L'évaluation motrice réalisée 6 mois après l'implantation des NST révèle une amélioration significative du handicap moteur (UPDRS III off) pré-opératoire de 63% sous stimulation seule (on stimulation off dopa) et de 81% sous stimulation et traitement médicamenteux (on stimulation on dopa) (p< 0.001). Il existe également une réduction significative des complications motrices liées au traitement dopaminergique, respectivement de 62% et 72% pour la sévérité des fluctuations et des dyskinésies induites par la dopathérapie ainsi que d'une réduction significative du traitement dopaminergique (exprimé en équivalent-dopa) de 54% (p< 0.001). L'évaluation cognitive comparative montre la préservation des scores obtenus aux échelles cognitives globales. En revanche, on retrouve une altération significative des fonctions exécutives. Trois patients ont présenté un hématome intracérébral per-opératoire, un patient a présenté une infection du matériel implanté. Les complications psychiatriques post-opératoires sont marquées par une tentative d'autolyse, 7 syndromes dépressifs et 8 épisodes de manie et/ou hypomanie résolutifs. Conclusion. Nos résultats confirment les données de la littérature et la place incontournable de la stimulation bilatérale du NST dans la prise en charge des formes avancées de la maladie de parkinson.ROUEN-BU Médecine-Pharmacie (765402102) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Clavicle fracture mimicking dropped-head syndrome in a patient with multiple system atrophy

International audienceDropped-head syndrome is characterized by an abnormal ante-fixed posture of the neck, usually observed in patients with neurodegenerative disorders such as multiple system atrophy (MSA) [1]. We report an unusual cause of acute antero- and latero-collis in a patient with MSA

Clavicle fracture mimicking dropped-head syndrome in a patient with multiple system atrophy

International audienceDropped-head syndrome is characterized by an abnormal ante-fixed posture of the neck, usually observed in patients with neurodegenerative disorders such as multiple system atrophy (MSA) [1]. We report an unusual cause of acute antero- and latero-collis in a patient with MSA

Accuracy of the robot‐assisted procedure in deep brain stimulation

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Subthalamic stimulation: toward a simplification of the electrophysiological procedure.

International audienceThe aim of the present study was to assess the consequences of a simplification of the electrophysiological procedure on the post-operative clinical outcome after subthalamic nucleus implantation in Parkinson disease

Alleviation of off-period dystonia in Parkinson disease by a microlesion following subthlamic implantation

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allevation of off-period dystonia in parkinson disease by a microlesion following subthalamic implantation

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Does early verbal fluency decline after STN implantation predict long-term cognitive outcome after STN-DBS in Parkinson's disease?

International audienceBackgrounds: An early and transient verbal fluency (VF) decline and impairment in frontal executive function, suggesting a cognitive microlesion effect may influence the cognitive repercussions related to subthalamic nucleus deep brain stimulation (STN-DBS.)Methods: Neuropsychological tests including semantic and phonemic verbal fluency were administered both before surgery (baseline), the third day after surgery (T3), at six months (T180), and at an endpoint multiple years after surgery (Tyears). Results: Twenty-four patients (mean age, 63.5 ± 9.5 years; mean disease duration, 12 ± 5.8 years) were included. Both semantic and phonemic VF decreased significantly in the acute post-operative period (44.4 ± 28.2 and 34.3 ± 33.4, respectively) and remained low at 6 months compared to pre-operative levels (decrease of 3.4 ± 47.8% and 10.8 ± 32.1%) (P < 0.05). Regression analysis showed phonemic VF to be an independent factor of decreased phonemic VF at six months. Age was the only independent predictive factor for incident Parkinson's disease dementia (PDD) (F (4,19) = 3.4, P <0.03).Conclusion: An acute post-operative decline in phonemic VF can be predictive of a long-term phonemic VF deficit. The severity of this cognitive lesion effect does not predict the development of dementia which appears to be disease-related