Foot drop and plantar flexion failure determine different gait strategies in Charcot-Marie-Tooth patients.
ABSTRACT To describe the temporal, kinetic, kinematic, electromyographic and energetic aspects of gait in Charcot-Marie-Tooth patients with foot drop and plantar flexion failure.
A sample of 21 patients fulfilling clinical, electrodiagnostic and genetic criteria for Charcot-Marie-Tooth disease were evaluated by computerized gait analysis system and compared to a group of matched healthy subjects. Patients were classified as having isolate foot drop (group 1) and association of foot drop and plantar flexion failure (group 2).
While it was impossible to detect a reliable gait pattern when the group of patients was considered as a whole and compared to healthy subjects, we observed two distinctive gait patterns when patients were subdivided as group 1 or 2. Group 1 showed a gait pattern with some characteristics of the "steppage pattern". The complex motor strategy adopted by this group leads to reduce the swing velocity and to preserve the step length in spite of a high energy consumption. Group 2 displayed a "clumsy pattern" characterized by very slow gait with reduced step length, a broader support area and great reduction in the cadence. This group of patients is characterized by a low energy consumption and greater energy recovery, due above all to the primary deficit and the various compensatory mechanisms.
Such between-group differences in gait pattern can be related to both primary motor deficits and secondary compensatory mechanisms. Foot drop and plantar flexion failure affect the overall gait strategy in Charcot-Marie-Tooth patients.
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ABSTRACT: Observations have been made on a series of 228 patients with hereditary motor and sensory neuropathy, comprising 120 index cases and 108 affected relatives. These could be separated into genetically distinct type I and type II categories depending upon whether motor nerve conduction velocity in the median nerve was below or above 38 m s-1. These disorders constitute separate genetic subgroups within the clinical spectrum of 'peroneal muscular atrophy'. Type I cases were more numerous. Most were of autosomal dominant inheritance, but a proportion were sporadic. Four families with probable autosomal recessive inheritance were identified; these displayed significantly slower motor conduction velocity. There was a positive correlation between motor conduction velocity in the propositi and that in their relatives in the total type I group which persisted after the autosomal recessive cases had been extracted, indicating further genetic heterogeneity amongst the autosomal dominant families. No X-linked recessive families were encountered. Type I cases had a peak age of onset of symptoms during the first decade of life. In comparison with the type II cases, they displayed a greater tendency to show weakness of the hands, upper limb tremor and ataxia, generalized tendon areflexia and more extensive distal sensory loss, sometimes with acrodystrophic changes. Foot and spinal deformities were more frequent, probably due to the earlier age of onset. Nerve thickening was confined to the type I cases. The onset of symptoms was most often in the second decade in the type II cases, but in some it was delayed, even as late as the seventh decade. Most cases were again of autosomal dominant inheritance, but two probable autosomal recessive families were detected, as well as sporadic cases. Upper limb tremor also occurred in this form but was considerably less common. In both types, males tended to be more severely affected, and asymptomatic affected family members ('formes frustes') were more commonly female.Brain 07/1980; 103(2):259-80. · 9.92 Impact Factor
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ABSTRACT: Fifty-eight patients with severe, progressive multiple sclerosis were prospectively randomized to one of three treatments: 20 received intravenous ACTH, 20 received high-dose intravenous cyclophosphamide plus ACTH, and 18 were placed on a regimen consisting of plasma exchange, low-dose oral cyclophosphamide, and ACTH. The three groups were similar in age, sex, duration and type of disease, and degree of disability. Before treatment and six months and one year after treatment, a disability-status score, ambulation index, and functional-status score were determined, and a quantitative neurologic examination was performed. In the ACTH group, the number of patients stabilized or improved was 8 of 20 at six months and 4 of 20 at one year; in the cyclophosphamide-ACTH group, 18 of 20 at six months and 16 of 20 at one year; and in the plasma exchange group, 11 of 18 at six months and 9 of 18 at one year. High-dose cyclophosphamide plus ACTH was most effective in halting progression of the disease at both 6 and 12 months (at 12 months, cyclophosphamide-ACTH vs. ACTH, P = 0.0004; cyclophosphamide-ACTH vs. plasma exchange, P = 0.087). Thus, progressive multiple sclerosis may be stabilized by short-term, intensive immunosuppression with cyclophosphamide plus ACTH.New England Journal of Medicine 02/1983; 308(4):173-80. · 51.66 Impact Factor
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ABSTRACT: To prospectively determine the frequency and circumstances of falls in older persons with peripheral neuropathy and to identify gait characteristics on smooth and irregular surfaces associated with falls in this same population. This was a descriptive and observational study of a prospective group cohort. Spatial and temporal gait measures on smooth and irregular surfaces, as well as basic demographic and clinical data, were obtained in 20 older persons with peripheral neuropathy. Falls and fall-related injuries were then prospectively determined for 1 yr. Thirteen of 20 (65%) subjects fell, and 6 of 20 (30%) subjects sustained a fall-related injury during the year of observation. Of the 76 reported falls, 69 (90.8%) were associated with a surface abnormality (irregular or slick). Gait measures on the smooth surface did not distinguish between fall groups. On the irregular surface, however, step-time variability tended to be higher for those subjects who fell than for those who did not (89 +/- 29 vs. 64 +/- 26 msecs, respectively; P = 0.077) and for those who were injured from a fall compared with those who were not injured (101 +/- 21 vs. 71 +/- 29 msecs, respectively; P = 0.038). Older patients with peripheral neuropathy have a high rate of falls, and these falls are often associated with walking on irregular surfaces. Gait analysis on an irregular surface may be superior to that on a smooth surface for detecting fall risk in this patient population.American Journal of Physical Medicine & Rehabilitation 03/2007; 86(2):125-32. · 1.73 Impact Factor