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Muscle cramp in pediatric Charcot-Marie-Tooth disease type 1A: prevalence and predictors
Fiona Blyton1, Monique M Ryan, Robert A Ouvrier
1Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Westmead, Australia.
Insights
Calf cramps are common in children with Charcot-Marie-Tooth disease type 1A (CMT1A), increasing with age. This study found that older age and hand tremor are independent predictors of calf cramp in these children.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Charcot-Marie-Tooth disease type 1A (CMT1A) is a progressive hereditary neuropathy.
- Calf cramps are a frequently reported symptom in individuals with CMT1A, impacting quality of life.
- Understanding the factors associated with calf cramps in pediatric CMT1A is crucial for management.
Purpose of the Study:
- To identify clinical and neurophysiological correlates of calf cramp in children diagnosed with CMT1A.
- To determine independent predictors of calf cramp in this pediatric population.
Main Methods:
- A cohort of 81 Australian children (aged 2-16 years) with CMT1A participated in the study.
- Comprehensive assessments included measures of strength, range of motion, balance, agility, endurance, gait, and neurophysiology.
- Post hoc logistic regression analyses were employed to identify independent predictors of calf cramp.
Main Results:
- Calf cramp was reported by 32% of the children.
- Calf cramp was significantly associated with older age, hand tremor, stronger foot muscle strength, and better performance on functional tests.
- Logistic regression identified increasing age (OR 1.32) and hand tremor (OR 3.81) as independent predictors of calf cramp.
Conclusions:
- Calf cramps are prevalent in children with CMT1A and their frequency increases with age.
- A novel association between calf cramp and hand tremor in pediatric CMT1A was identified.
- Further research into shared mechanisms and risk factors for cramp and tremor is warranted to improve understanding and management of CMT1A complications.
Objectives:
To identify correlates of calf cramp in children with Charcot-Marie-Tooth disease type 1A (CMT1A).
Methods:
Throughout Australia, 81 children aged 2-16 years with CMT1A were recruited. Measures of strength, ankle range, foot posture, balance, agility, endurance, gait, and neurophysiology were collected. Post hoc logistic regression analyses were performed to identify independent predictors of calf cramp.
Results:
Of the 81 children, 26 (32%) reported calf cramp, and 1 child each reported toe, quadriceps, or arm cramp. Calf cramp was associated (p < 0.05) with older age; the presence of hand tremor; stronger foot inversion, eversion, dorsiflexion, and plantarflexion; and better performance in long-jump and 9-hole peg tests. Logistic regression analysis revealed only increasing age (odds ratio [OR] 1.32, 95% confidence interval [CI] 1.11-1.58; p = 0.002) and the presence of hand tremor (OR 3.81, 95% CI 1.18-12.56; p = 0.028) as independent predictors of calf cramp.
Conclusion:
Calf cramps are common in children with CMT1A and worsen with age. This study revealed a previously unrecognized link between cramp and hand tremor in children with CMT1A. Further investigation of proposed mechanisms and risk factors common to both cramp and tremor will contribute to our understanding of these common complications of CMT1A.
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