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Abstract
Sixty-one members of a large Spanish kindred with autosomal dominant limb-girdle muscular dystrophy (LGMD), spanning 5 generations, were examined at the Hospital Vail d’Hebron, Barcelona and other centers.
Highlights
Sixty-one members of a large Spanish kindred with autosomal dominant limb-girdle muscular dystrophy (LGMD), spanning 5 generations, were examined at the Hospital Vail d'Hebron, Barcelona and other centers
Characteristic proximal muscle weakness involving pelvic and shoulder girdle muscles was found in 32 patients (15 men, 17 women) between the ages of 7 and 66 years
Weakness extended to distal muscles late in the course in adult cases, but generalized weakness occurred at initial presentation in severely affected juvenile cases
Summary
Motor system excitability was measured in 16 children with ADHD, 16 with chronic tic disorder or Tourette's disorder (TD), 16 with comorbid ADHD and TD, and 16 healthy control children, in a study at the University of Gottingen, Germany. Sixty-one members of a large Spanish kindred with autosomal dominant limb-girdle muscular dystrophy (LGMD), spanning 5 generations, were examined at the Hospital Vail d'Hebron, Barcelona and other centers. Characteristic proximal muscle weakness involving pelvic and shoulder girdle muscles was found in 32 patients (15 men, 17 women) between the ages of 7 and 66 years (mean 34 years). Age at onset ranged from less than 1 to 58 years (mean 16 years).
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