Hoffman's syndrome: pseudohypertrophic myopathy as initial manifestation of hypothyroidism. Case report

Abstract

-The frequency of myopathy in hypothyroidism ranges from 30 to 80%. The major symptoms related are weakness, muscular cramps and myalgia. The pseudohyperthrophic form is called Hoffman's syndrome. The electrophysiological study reveals myopathy, neuropathy or mixed pattern. Laboratorial investigation generally shows increased levels of muscle enzymes and low serum thyroid hormones, with thyrotrophic-stimulating hormone (TSH) elevated. The treatment consists in hormone replacement and the prognosis is good in most of the cases. We report an adult male who developed muscular cramps, myalgia, weakness, pseudohyperthrophy, associated with facial edema and alteration of his voice. The muscle enzymes were increased and T4 was undetectable with a raised level of TSH. The myopathy was the initial manifestation of hypothyroidism in this case. Resumo -A frequência de miopatia no hipotireoidismo varia de 30% a 80%. Os sintomas relacionados ao acometimento muscular são fraqueza, cãimbras e mialgias. A forma pseudo-hipertrófica é denominada síndrome de Hoffman. O estudo eletrofisiológico pode revelar padrão miopático, neuropático ou misto. A investigação laboratorial em geral mostra aumento das enzimas musculares e redução dos níveis de hormônio tireoidiano com TSH elevado. O tratamento consiste na reposição oral de hormônio e o prognóstico é bom na maioria dos casos. Relatamos o caso de um adulto que apresentou cãimbras, mialgia, fraqueza com pseudohipertrofia muscular associados a edema facial e alteração da voz. As enzimas musculares estavam elevadas e o nível de T4 foi indetectável com aumento de TSH. A miopatia foi manifestação inicial de hipotireoidismo neste caso.

Hoffmann’s Syndrome: A Case Report

“…7 Patients present with muscle cramps, muscle stiffness, weakness, hyporeflexia and delayed deep tendon reflexes. 8 Muscle enlargement (pseudohypertrophy) is very rare and its etiology remains controversial. 9 Calf muscles (gastrocnemius) are commonly involved.…”

“…The EMG findings compatible with myogenic pattern are diminished duration, and amplitude of motor unit action potentials (MUAP). 8 Hoffmann syndrome carries favorable prognosis once hormone replacement is instituted and most of the symptoms regress slowly with time, including the muscle enlargement. The decline of the muscle enzyme levels occurs slowly, varying from weeks, months or even years with the treatment.…”

“…The decline of the muscle enzyme levels occurs slowly, varying from weeks, months or even years with the treatment. 8,11 Electrophysiological findings may persist in some patients despite improvement of symptomatology and muscle enzymes. 8 …”

“…8,11 Electrophysiological findings may persist in some patients despite improvement of symptomatology and muscle enzymes. 8 …”

Hoffmann's Syndrome associated with Autoimmune Hypothyroidism: a Case Report

“…Myopathy syndromes associated with hypothyroidism are Hoffmann's Syndrome, KocherDebre-Semelaigne Syndrome, atrophic form and myasthenic syndrome [1]. Hoffmann's Syndrome which is one of these is a rare disorder in adults, first described by Hoffmann in 1897.…”