Mélissa Tir

Exhaustive, one-year follow-up of subthalamic nucleus deep brain stimulation in a large, single-center cohort of parkinsonian patients.

OBJECTIVE To prospectively assess the impact of subthalamic nucleus (STN) deep brain stimulation (DBS) at 12 months after surgery in a series of 100 consecutive patients treated in a single center. The primary objective was to describe the clinical outcome in terms of efficacy and tolerance in STN-DBS patients. A secondary objective was to discuss presurgery clinical characteristics a posteriori as a function of outcome. METHODS One hundred and three consecutive patients with severe Parkinsons disease received bilateral STN-DBS in our clinic between May 1998 and March 2003. Clinical assessment was performed before and 12 months after surgery and was based on the Unified Parkinsons Disease Rating Scale, Parts II, III, and IV A; the Schwab and England Scale; and cognitive evaluation. Patient-rated overall improvement was also evaluated. RESULTS Twelve months after surgery, the Unified Parkinsons Disease Rating Scale Part III score decreased by 43%, the Unified Parkinsons Disease Rating Scale Part II score (activities of daily living) fell by 34%, and the severity of dyskinesia-related disability decreased by 61%. The main surgical complications after STN-DBS were as follows: infection (n = 7), intracerebral hematoma (n = 5), electrode fracture (n = 4), and incorrect lead placement (n = 8). We observed cognitive decline and depression in 7.7 and 18% of the patients, respectively. The mean patient-rated overall improvement score was 70.7%. CONCLUSION The efficacy and safety of STN-DBS in our centers large cohort of Parkinsonian patients are generally similar to the results obtained by other groups, albeit at the lower limit of the range of reported values. In contrast to efficacy, the occurrence of adverse events cannot be predicted. Younger patients with Parkinsons disease (i.e., those younger than 60 yr) often show an excellent response to levodopa. However, in view of our data on overall patient satisfaction and the occurrence of adverse events, we suggest that older patients (but not those older than 70 yr) and less dopa-sensitive patients (but not those with a response <50%) should still be offered the option of STN-DBS.

Motor‐related circuit dysfunction in MSA‐P: Usefulness of combined whole‐brain imaging analysis

The aim of this study was to evaluate in vivo changes in the brains macro‐ and microstructure (notably in the motor system) in the parkinsonian variant of multiple system atrophy (MSA‐P) and in Parkinsons disease (PD) and to characterize the cerebral anatomical differences between the two conditions. We used a combination of voxel‐based morphometry (VBM) and whole‐brain, voxel‐based diffusion tensor imaging analysis (VB‐DTI). Forty‐seven right‐handed subjects (14 MSA‐P patients, 19 PD patients, and 14 controls) were evaluated using VBM and VB‐DTI in an analysis of covariance (ANCOVA) with a significance threshold set to P < 0.005. In MSA‐P patients, VBM analysis revealed a lower density of grey matter (GM) in a motor‐related circuit (especially in the left primary motor cortex, PMC), relative to PD patients, and in the left supplementary motor area (SMA), relative to controls). Diffusion tensor imaging analysis revealed lower fractional anisotropy (FA) values in the left PMC and the right cerebellum in MSA‐P patients, compared with controls. Using a volumetric diffusion technique, our study revealed selective tissue degeneration in motor circuits, regardless of the volume loss detected in VBM and in agreement with pathology reports and clinical motor characteristics. Our findings suggest that MSA‐P is characterized by both macro‐ and microstructural changes in the sensorimotor circuit.

Global Hypoactivity and Apathy

Global hypoactivity contrasting with apparently unaffected cognitive abilities was initially reported in cases of frontal damage and is now recognized as a leading behavioral feature of many cerebral diseases. Of the various terms used to refer to this behavioral change, “apathy” is now the most widely used.

Huntington's Disease Revealed by Familial Cervical Dystonia

Mrs. M (a 45-year-old woman) had progressively developed isolated cervical dystonia (CD) over a 12-month period. She had no history of long-term treatment with medications in general or antipsychotics in particular. With the exception of CD, the results of a neurological examination (including psychiatric and cognitive status) were completely normal. Mrs. M did not report a family history of neurological disorders at the time of our initial interview but had said that she was no longer in touch with family members as a result of a dispute, raising the possibility that her knowledge of her family’s medical history might have been inaccurate or biased. However, Mrs. M was then lost to follow-up. Five years later, she consulted for memory problems (although CD was still the predominant clinical sign). A neuropsychological assessment revealed mild cognitive impairment (memory impairment, constructional apraxia, and cognitive dysexecutive syndrome), and a clinical assessment evidenced mild, generalized chorea. In the time since her first consultation, Mrs. M had re-established a relationship with her family and now reported a family history of movement disorders (suggesting the inheritance of an autosomal-dominant condition) (Fig. 1). Taken as a whole, these data prompted us to diagnose Huntington’s disease (HD), which was subsequently

D - 14 Effets de la stimulation électrique du NST sur les complications dopa-induites dans la maladie de Parkinson

Introduction La stimulation du noyau sous-thalamique (NST) semble reduire les fluctuations et les dyskinesies dopa-induites dans la maladie de Parkinson, mais cet effet a ete beaucoup moins etudie que les autres symptomes de la maladie. Objectifs Evaluer precisement l’efficacite a long terme de la stimulation du NST sur les complications motrices dopa-induites. Methodes 36 patients furent evalues avant la chirurgie et 1 et 5 ans apres, avec les echelles du CAPSIT-PD pour la dystonie off drug et les dyskinesies on drug, stimulateur eteint ou allume, avant et apres l’administration de L-dopa dispersible. les echelles UPDRS II, IV et de Schwab et England (SE) furent aussi utilisees. Resultats L’UPDRS IV s’ameliora de 62 % a 1 an et 68 % a 5 ans, l’UPDRS IVA de 72 % a 1 an et 71 % a 5 ans. Les dyskinesies on drug s’ameliorerent a 1 an de 61 % on stim et 60 % off stim, a 5 ans de 85 % on stim et 77 % off stim. Les dystonies off drug s’ameliorerent a 1 an de 45 % on stim et 12 % off stim, a 5 ans de 66 % on stim et 35 % off stim. Pour l’UPDRS II et la SE, il y eut une amelioration significative a 1 et 5 ans. A 5 ans, 1 patient/3 avait le meme score on et off drug a l’UPDRS II (aucun a 1 an), et a la SE (16 % a 1 an). Discussion Il y a un effet positif de la stimulation du NST sur les complications liees a la L-dopa qui persiste et meme augmente a long terme. Les scores de dyskinesie sont ameliores a la fois de maniere chronique et lors d’un test d’administration aigue de L-dopa. De plus cet effet persiste lorsque le stimulateur est eteint. Conclusion La diminution des doses de L-dopa apres stimulation peut expliquer en partie l’amelioration des complications motrices, mais nos resultats sont aussi en faveur du renversement des mecanismes entrainant ces complications.