Minimal clinically important differences in functional motor scores in adults with spinal muscular atrophy.

BACKGROUND AND PURPOSE: In patients with spinal muscular atrophy (SMA), functional disease scores are frequently used to evaluate the course of the disease and the efficacy of treatment. The aim of the present study was to propose minimal clinically important difference (MCID) values for motor scores in order to estimate the degree of change within a functional score that can be considered clinically meaningful. METHODS: To estimate the MCID, distribution-based approaches were used. For each assessment [Revised Upper Limb Module (RULM), Hammersmith Functional Motor Scale Expanded (HFMSE) and 6-min walk test (6MWT)] and subgroup (SMA type 2, SMA type 3, ambulatory and non-ambulatory), the following MCID values based on a cohort of 51 adults with SMA were calculated: standard error of measurement (SEm), one-half of standard deviation (1/2 SD) and one-third of standard deviation (1/3 SD) of patients' baseline scores. RESULTS: For the overall cohort, the SEm, 1/2 SD and 1/3 SD MCID values were 2.9, 6.4 and 4.3 for the RULM and 4.3, 10.6 and 7.0 for the HFMSE, respectively. Subgroup analysis led to generally lower standard deviations and consecutively lower MCID values due to the significantly different motor functions of the groups. The respective MCID values for the 6MWT were 55.5 m, 71.1 m and 47.8 m. CONCLUSIONS: Our data provide MCID values for functional motor scores commonly used in adults with SMA in order to distinguish statistical effects from 'real' changes. A complementary systematic consensus process could help to further adjust the MCID values we propose.

Technical evolutions of the French multipurpose instruments for Cognitive Neurosciences.

Since the first French flight in space in 1982, the CNES has developed a wide range of instruments, especially in the field of Neurosciences. The design of these instruments has considerably evolved from rather simple equipment up to much more sophisticated tools that are being specially tailored for these missions. Four major phases can be identified: -a simple adaptation of an echographe leading to the first neurosciences experiments (the ARAGATZ'88 mission), -the ILLUSIONS and VIMINAL instruments used during the ANTARES'92 and ALTAIR'93 missions, -the COGNILAB instrument developed for the CASSIOPEE'96 mission, to be re-used in 1997 and in 1999, -a preliminary design of the 1999 mission payload, including virtual reality concepts, in a modular design to adapt to the European COF. Aside from the evolution of scientific requirements, the experience gained during the flights led to progressive improvements in the different technical parts, including visual system, body restraint systems, accessories, such as a force feedback joystick, computer and software, etc. This paper describes the technical evolutions in the CNES Neurosciences program.

Counterimmunoelectrophoresis for the diagnosis of bovine mycoplasmosis.

Counterimmunoelectrophoresis (CIE) procedure has been established for mycoplasma identification from cultures, and the specificity and the sensitivity of this technique were evaluated. Preparations of milk samples and clinical and necropsy specimens were used for the technique. From 171 milk samples, 80 (46.8%) were positive by CIE (73 samples with Mycoplasma bovis and 7 with Mycoplasma arginini) and 77 (45%) were positive by biochemical and growth inhibition tests (70 samples with Mycoplasma bovis and 7 with Mycoplasma arginini). From 36 mycoplasma culture-positive clinical and necropsy specimens, CIE identified 5 different mycoplasma species and from 3 mycoplasma culture-positive specimens, the technique identified coinfection by 2 mycoplasma species. The CIE is a reliable, specific, sensitive, and rapid technique for the diagnosis of mycoplasmosis in the bovine.

[Micrometric in situ marking apparatus for an object on a microscope grid. Application of observation of serial sections]. / Dispositif de repérage micrométrique in situ d'un objet sur une grille de microscope. Application à l'observation des coupes sériées.

A device for the precise localization (better than 0.5 micron) of an object on a grid has been developed and connected to the translation system of an electron microscope. Applied to biological thin sections, this device enables to easily find and to observe a selected microcrystal projection obtained by the grid sectioning technique. Moreover, low dose observations on ribbons of ultrathin sections can be made because the information is repeated in the successive sections of the ribbon: With the device the position of the selected object is determined at high dose in one section and low dose measurement is made on the following section.

[Diagnosis of septic abortion in dairy cows]. / Diagnostic des avortements infectieux chez les bovins laitiers.

During a two year period, March 1977 to April 1979, a total of 92 bovine abortions were studied. The cause of abortion was determined in 34.8% of the cases examined. Opportunistic bacteria, the most commonly diagnosed cause of abortion, accounted for 31.2% of the cases. Leptospirosis was associated with 28.1% of the abortions, infectious bovine rhinotracheitis and fungi in respectively 15.7%, bovine viral diarrhea in 6.2%. A congenital abnormality accounted for one case (3.1%). In 23 cases (25%), there was no definitive diagnosis, in spite of evidence of experience with pathogen or suggestive findings of pathology, but insufficient evidence to warrant diagnosis. No findings were recorded in 35.8% of the (possibly noninfectious) cases and in only four cases (4.4%), specimens were unsatisfactory for examination.