Natural history of Charcot–Marie-Tooth 2: 2-year follow-up of muscle strength, walking ability and quality of life

Charcot–Marie-Tooth (CMT) disease is the most frequent inherited neuropathy, no therapies are available at the moment but clinical trials are ongoing. For that reason it is very important to know the natural history of the disease. We report the results of the natural history of clinical features an...

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Bibliographic Details
Published in:Neurological sciences 2010-04, Vol.31 (2), p.175-178
Main Authors: Padua, Luca, Pareyson, D., Aprile, I., Cavallaro, T., Quattrone, D. A., Rizzuto, N., Vita, G., Tonali, P., Schenone, A.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Charcot-Marie-Tooth Disease - physiopathology
Charcot-Marie-Tooth Disease - psychology
Disability Evaluation
Disease Progression
Female
Follow-Up Studies
Humans
Male
Medicine
Medicine & Public Health
Middle Aged
Muscle Strength
Muscle, Skeletal - physiopathology
Neurologic Examination
Neurology
Neuroradiology
Neurosciences
Neurosurgery
Original Article
Psychiatry
Quality of Life
Walking
Young Adult
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Summary:Charcot–Marie-Tooth (CMT) disease is the most frequent inherited neuropathy, no therapies are available at the moment but clinical trials are ongoing. For that reason it is very important to know the natural history of the disease. We report the results of the natural history of clinical features and quality of life (QoL) in patients with CMT2. Twenty patients were enrolled. At recruitment and at follow-up (2 years), all patients underwent neurological evaluation, QoL and disability assessments. The study-end evaluation took place 20–28 months after the baseline evaluation. During the 2-year follow-up period, CMT2 patients showed a mild reduction of strength of distal muscles of upper limbs and proximal muscles of lower limbs, a worsening sensory function and a mild increase in walking disability. However, there was no relevant worsening of QoL, except for a mild deterioration of one mental health domain.
ISSN:1590-1874
1590-3478
DOI:10.1007/s10072-009-0202-z