Hereditary Motor and Sensory Neuropathy, Types I and II

Carter, G. T.; Abresch, Richard T.; Fowler, William M.; Johnson, Ernest H.; Kilmer, David D.; McDonald, Craig

doi:10.1097/00002060-199509001-00008

Cited by 95 publications

(51 citation statements)

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“…Our results do not support the overwork weakness hypothesis in patients with CMT disease. In contrast, our results are similar to those of Carter et al (8), who presented equal grip and pinch strength in both hands in 53 patients with CMT disease. A number of differences between our study and that of Vinci et al (9) should be noted.…”

Section: Discussionsupporting

confidence: 91%

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The hypothesis of overwork weakness in Charcot-Marie-Tooth: A critical evaluation

Pomeren¹,

Selles²,

Ginneken³

et al. 2009

J Rehabil Med

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Objective: It has been reported that the non-dominant hand of patients with Charcot-Marie-Tooth disease is stronger than the dominant hand as a result of overwork weakness. The objective of this study was to determine if this hypothesis could be verified in our population. Design: Survey. Subjects: Twenty-eight patients with Charcot-Marie-Tooth disease type I or II from a rehabilitation department of a university hospital in the Netherlands. Methods: The strength of 3 intrinsic muscle groups of the dominant and non-dominant hand were determined using the Medical Research Council scale and the Rotterdam Intrinsic Hand Myometer. Furthermore, grip strength, pinch and key grip strength were measured. Results: We found no differences in muscle strength for the dominant and non-dominant hand, except for a stronger key grip strength of the dominant hand in patients with CharcotMarie-Tooth disease type II. Conclusion: In our population, the dominant hand of patients with Charcot-Marie-Tooth disease type I and II was equally strong as the non-dominant hand, suggesting that there is no presence of overwork weakness in the dominant hand in our group of patients. This implies that patients with Charcot-Marie-Tooth disease do not have to limit the use of their hands in daily life in order to prevent muscle strength loss.

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Section: Discussionsupporting

confidence: 91%

“…This finding of Vinci et al (1) contrasts with that of Carter et al (8) who found that strength was equal in both hands in 53 patients with CMT disease, and is also in contrast with findings in healthy subjects (9).…”

Section: Introductionmentioning

confidence: 73%

The hypothesis of overwork weakness in Charcot-Marie-Tooth: A critical evaluation

Pomeren¹,

Selles²,

Ginneken³

et al. 2009

J Rehabil Med

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“…For example, the grip strength measured in 86 patients with CMT disease 2 was approximately 170 and 280 N (women and men) compared with 176 and 293 N in the current study. In another study on 20 patients with CMT disease, 10 a mean grip force was found of 227 N in women and men combined.…”

contrasting

confidence: 53%

Dynamometry of intrinsic hand muscles in patients with Charcot–Marie–Tooth disease

Selles¹,

Ginneken²,

Schreuders³

et al. 2006

Neurology

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Abstract-Background: Several problems are associated with manual muscle testing and dynamometry in the hands of patients with Charcot-Marie-Tooth (CMT) disease. Objective: To evaluate the efficacy of the Rotterdam Intrinsic Hand Myometer (RIHM) to directly measure intrinsic hand muscle strength in CMT disease. Methods: We measured hand muscle strength and hand function in 41 patients with CMT disease. Results: RIHM measurement of intrinsic strength had excellent reliability. We found overlapping RIHM strength values in Medical Research Council grades 3 to 5. High grip and pinch strength could be found in patients with severe intrinsic muscle weakness. RIHM measurements were more strongly correlated with fine motor skills of the hand than grip and pinch strength. Conclusions: The Rotterdam Intrinsic Hand Myometer is a reliable instrument to measure intrinsic hand muscles strength in patients with CharcotMarie-Tooth disease, providing more detailed information than manual muscle testing and a more direct assessment of intrinsic muscle loss than grip and pinch dynamometers.

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“…CMT Tip 1A'da ayak bileği kassal zayıflığının, tüm bu günlük yaşam becerilerinin kazanılmasına engel olabileceği ve ayak deformitesinin, ağrı ve yürümede zorluğa sebep olabileceği bildirilmiştir (26)(27)(28)(29)(30) 6DYT süresince gruplar arasında adım uzunlukları açısından anlamlı fark olduğu, distal grupta proksimal gruba göre daha yüksek değerde adım uzunluğu belirlenmiştir. Bu durumun distal tutulumlu gruptaki çocukların yaşlarının daha büyük ve boylarının da daha uzun olması gibi fiziksel özellikleri arasındaki farktan kaynaklandığı düşünülmektedir.…”

Section: Discussionunclassified

Farkli Nöromusküler Hastaliklarda Ayak Bi̇leği̇ Li̇mi̇tasyonunun Yürüme Beceri̇si̇ Üzeri̇ne Etki̇si̇

Kaya¹,

Alemdaroğlu²,

Yılmaz³

et al. 2014

Turk J Physiother Rehabil

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Amaç: Çalışmanın amacı farklı bölgesel kas zayıflığına sahip iki grupta (proksimal ve distal) ayak bileği limitasyonunun, yürüme becerisi üzerine olan etkisini araştırmak ve karşılaştırmak idi.Yöntem: Çalışmaya Brooke Alt Ekstremite Fonksiyonel Sınıflandırması'na göre devre 1-3, primer olarak proksimal kas tutulumu olan 20 çocuk (yaş ortalaması:9.05±3.1 yıl), ve distal kas tutulumu olan (yaş ortalaması:12.95±3.3 yıl) diğer 20 çocuk olmak üzere, toplam 40 çocuk dahil edildi. Çocukların demografik bilgileri kaydedildi. Ayak bileği eklem limitasyonu gonyometre ile ölçüldü. Yürüme kapasiteleri, 6 dakika yürüme testi (6DYT) ile değerlendirildi. 6 dakikadaki yürüme mesafesi, attıkları tur, adım sayısı, adım uzunlukları ve kadansları kaydedildi. Sonuçlar:Çalışmada ayak bileği dorsifleksiyon limitasyonu ve 6 dakikada yürüdükleri toplam mesafe açısından gruplar arasında fark bulunmadı (p>0.05). 6DYT'nin diğer performans parametreleri açısından, test sırasındaki adım uzunluklarında distal kas tutulumlu grup lehine istatistiksel olarak anlamlı fark bulundu (p<0.01). Proksimal tutulumlu grupta 6 dakika süre-since yürünen toplam mesafe ve sağ ve sol ayak bileği limitasyon sonuçları arasında; sağ (r= -0.579, p= 0.01) ve sol (r=-0.445, p= 0.05) negatif yönde, orta kuvvette, istatistiksel olarak anlamlı ilişki bulundu (p<0.05).Tartışma: Çalışmanın sonuçları NMH'ye sahip bireylerde, özellikle de proksimal kas zayıflığı-nın hakim olduğu musküler distrofili çocuklarda hastalıklarının erken dönemlerinden itibaren ayak bileği eklem hareket açıklığının yürüme performansı üzerindeki önemini ortaya koymuş-tur.Anahtar Kelimeler: Nöromusküler hastalıklar; ayak eklemleri; yürüme RESEARCH ARTICLE EFFECT OF ANKLE JOINT LIMITATION ON WALKING ABILITY IN DIFFERENT NEUROMUSCULAR DISEASES ABSTRACTPurpose: In this study, we aimed to investigate the impact of the ankle limitation on the walking ability among two groups that have different spatial distribution of the weakness. Methods:In total, 40 children at the levels 1-3 of the Brooke Lower Extremity Scale are included in this study. 20 of them have been diagnosed distal muscle weakness (mean ages: 12.9±3.3 year), and the other 20 have been proximal muscle weakness (mean ages: 9±3.1). The demographic information were recorded. Ankle joint limitation were measured with goniometer. Their ambulatory capacities were evaluated with the 6 minutes walk test (6MWT). Total walking distances in 6 minutes, strolls that taken, step counts, stride lengths, and cadences were saved.Results: There was not any significant difference between the groups with respect to the limitations in ankle joints' movement and 6 minute total walking distance (p > 0.05). For the other 6MWT walking parametres, there was a statistically significant difference on the stride lengths in the test in favour of (to the good) the group with distal muscle weakness (p< 0.01). There was a negative correlation between the right (r=-0.579, p=0.01) and left (r=-0.445, p= 0.05) ankle dorsiflexion limitations and 6MWT results in the group with ...

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Hereditary Motor and Sensory Neuropathy, Types I and II

Cited by 95 publications

References 0 publications

The hypothesis of overwork weakness in Charcot-Marie-Tooth: A critical evaluation

The hypothesis of overwork weakness in Charcot-Marie-Tooth: A critical evaluation

Dynamometry of intrinsic hand muscles in patients with Charcot–Marie–Tooth disease

Farkli Nöromusküler Hastaliklarda Ayak Bi̇leği̇ Li̇mi̇tasyonunun Yürüme Beceri̇si̇ Üzeri̇ne Etki̇si̇

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