Endocrine Abstracts

Introduction: Hoffman’s syndrome is a rare form of hypothyroid myopathy characterised with muscle hypertrophy, stiffness and wakefulness.

Case report: A 43-years-old woman admitted to our hospital with a complaint of fatigue and progressive muscular weakness with cramps and myalgia started 3 months before.Thyroid gland examination was normal. On neurological examination she had proximal and lower limb muscle weakness(3/4) and hyporeflexia. Her calf muscles was hypertrophic.Laboratorial investigation reveals increased serum levels of muscular enzymes, dislipidemia severe hypothroidism. Electromyography (EMG) muscles revealed low amplitude and short duration motor unit action potentials (MUAPs) with early recruitment suggestive of a myopathic disorder. The patient was diagnosed of having severe hypothyroidism with Hoffman syndrome. L-thyroxine treatment was started (100 μg per day) and dose was elevated 150 μg per day after 2 weeks later. After 1 month therapy her hypothyroid symptoms reduced, pseudohypertrophy of the calf muscles regressed, muscle enzymes were reduced.

Discussion: The characteristic features of Hoffman’s syndrome include localised or generalised hypertrophy of muscles in addition to muscle weakness, stiffness, cramps and pain as compared to the classic symptoms of hypothyroidism.The CPK is the best biochemical marker of myopaties. Mild to moderate elevation of serum CPK level is seen in 70–90% patients with hypothyroidism indicative of muscle involvement but does not correlate with the severity of weakness. The clinical presentation and biochemical features might make it a bit difficult for the physician to differentiate it from polymyositis or muscle dystrophies. Hoffman Syndrome has good prognosis if diagnosed earlier and treated appropriately.This case report shows that Hoffman syndrome, though a rare presentation of hypothyroidism has a good prognosis with timely diagnosis and appropriate management. In the differential diagnosis of myopathy with pseudohypertrophy, Hoffmann’s syndrome should be considered. It is an infrequent cause of myopathy, with good prognosis.