Debre-Semelaigne Syndrome

Hypothyroid myopathy occurs in about 30% of patients with hypothyroidism irrespective of its cause. Muscle pain, cramps, and stiffness may be seen, and are often exacerbated by cold weather. Pseudomyotonic features of delayed muscle contraction and relaxation are common. Myoedema (the mounding phenomenon) is due to the painless, electrically silent contracture produced on direct percussion. Muscle biopsy often shows a predominance of type 1 (slow-twitch) fibers, again analogous to that seen in experimental hypothyroidism (Figure 22). Muscle hypertrophy with weakness and slowness of movement occurs in the Debre-Semelaigne syndrome seen in severely hypothyroid children, and Hoffman s syndrome is a similar condition seen in adults with hypothyroidism, but is also accompanied by painful spasms. 

Rhabdomyolysis is also uncommon and is often exacerbated by concomitant medications and other underlying conditions, such as polymyositis (Madariaga et al., 2002). The prevalence is unknown, but is less common than TTX. It can also cause muscle stiffness and pseudohypertrophy, also know as Hoffman syndrome and Kocher-Debre-Semelaigne syndrome, respectively (Mastropasqua et al., 2003). A significant number of cases have mild elevated CK level without... 

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See also in sourсe #XX -- [ ]

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