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Strengthening of the proximal muscles in Charcot-Marie-Tooth disease

Letters to the Editor

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Several reports1-3 have suggested that the proximal muscles in

Charcot-Marie-Tooth (CMT) disease may be strengthened by resistance exercise.

Any conclusion from such studies cannot be extrapolated to distal muscles, which

are selectively involved by CMT disease.4 More important, however, in our large

CMT1 and CMT2 population, most patients' proximal muscles are strong enough and

so do not need strengthening.

Several of our patients with apparent gluteus maximus weakness, assessed by

manual muscle testing, were found to have one of the following problems: (1)

foot inversion causing hip flexion-intrarotation and knee flexion, as protective

mechanisms aimed at a reduction of foot inversion; (2) plantarflexor failure in

patients with residual dorsiflexion range of motion or in patients wearing shoes

with excessive heels (which caused knee and hip flexion); (3) plantarflexor

contracture or equinus deformity compensated by hip flexion and pelvis

antiversion; and (4) quadriceps muscle weakening with the consequent need to

keep the trunk bent and to use 1 or 2 canes.

These patients promptly recovered normal strength in the gluteus maximus after

their distal joints had been stabilized by use of proper footwear or an orthotic

device and after postural training to promote fully erect stance.5 This result

indicates that gluteus maximus weakness did not result from the neuropathy

itself; rather, it resulted from an inability to contract fully a muscle that

participated in an unused pattern of movement and from resistance caused by hip

flexors in contracture.

Our experience suggests that classical strengthening exercises for the glutei

muscles may not be helpful, particularly when the distal alterations causing

abnormal posture in stance are not first corrected.

doi:10.1053/apmr.2001.24085

References

1. Lindeman E, Leffers P, Spaans F, Drukker J, Kerckhoffs M, Koke A. Strength

training in patients with myotonic dystrophy and hereditary motor and sensitive

neuropathy: a randomized clinical trial. Arch Phys Med Rehabil 1995;76:612-20.

MEDLINE

2. Lindeman E, Leffers P, Reulen J, Spaans F, Drukker J. Progressive

resistance training in neuromuscular patients. Effects on force and surface EMG.

J Electromyogr Kinesiol 1999;9:379-84. MEDLINE

3. Lindeman E, Leffers P, Reulen J, Spaans F, Drukker J. Surface EMG of

proximal leg muscles in neuromuscular patients and in healthy controls.

Relations to force and fatigue. J Electromyogr Kinesiol 1999;9:299-307. MEDLINE

4. Dyck PJ, Chance PF, Lebo RV, Carney JA. Hereditary motor and sensory

neuropathies. In: Dyck PJ, JW, Low PA, Poduslo JF, editors. Peripheral

neuropathy. 3rd ed. Philadelphia: WB Saunders; 1993. p 1094-136.

5. Vinci P, Perelli SL. Malattia di Charcot-Marie-Tooth: aspetti

clinico-riabilitativi. Neurol News 1999;2:3-31.

Paolo Vinci, MD

Specialized Rehabilitation Hospital L. Spolverini

Ariccia (Rome), Italy

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