Muscular and/or neurological symptomatology described in untreated hypothyroidism: pseudo-herculean muscle hypertrophy (limbs, tongue) with hypotonia, with pseudomyotonia (delayed muscle release after contraction), myokymia and psychomotor slowing. CPK level is elevated.

Muscular pseudohypertrophy is probably secondary to metabolic disturbances due to thyroid hormone deficiency: accumulation of glycogen, increase in fibrous tissue.  

Anesthetic implications: 

check thyroid hormone levels, classic precautions in case of hypothyroidism (see this term); sometimes macroglossia.

References : 

-        Shaw C, Shaw P.
Kocher-Debre-Semelaigne syndrome: hypothyroid muscular pseudohypertrophy- a rare report of two cases.
Case Reports in Endocrinology 2012 : 153143, doi:10.1155/2012/153143

-        Razi SM, Gupta AK, Gupta DC, Gutch M, Gupta KK, Usman SI.
Van Wyk-Grumbach syndrome with Kocher-Debré-Sémélaigne Syndrome: case report of a rare association.
Eur Thyroid J 2017;6:47–51

Updated: May 2024