Kocher-Debre-Semelaigne Syndrome (KDSS) and Hoffmann’s syndrome are forms of hypothyroid myopathy that overlap.
Synonym: cretinism-muscular hypertrophy
Features in common with Hoffmann’s syndrome:
(1) clinical features of moderate to severe hypothyroidism (in KDSS this may be congenital)
(2) myopathy with pseudohypertrophy (giving the patient an athletic appearance)
(3) laboratory features of hypothyroidism and myopathy
Features distinguishing Kocher-Debre-Semelaigne Syndrome from Hoffmann’s syndrome:
(1) pediatric age group
(2) males affected more than females
(3) short stature
(4) variable orofacial manifestations (dull facies, protruding tongue and dental defects)
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Specialty: Surgery, orthopedic
