Hypothyroidism Presenting as Hoffmans Syndrome  A Case Report

Ankur Jain,Ishu Singh,Jasbir Kumar,N.s Neki ,Tamil Mani

doi:10.3329/jom.v16i2.25440

Abstract

Hoffman syndrome is characterized by pseudohypertrophy of muscles, muscles weakness & stiffness complicating hypothyroidism. We describe the disorder in a 45 years old female admitted with complaints of myalgia, proximal muscle weakness & calf muscle hypertrophy since 11 months. Thyroid function tests, marked elevation of muscle enzyme, electromyogram & muscle biopsy established the diagnosis of thyroid myopathy with Hoffmans syndrome. Therapy with levothyroxine resulted in marked clinical & biochemical improvements.J MEDICINE July 2015; 16 (2) : 112-114

Highlights

Hoffman’s syndrome was first described by Hoffman in 1897 in an adult who developed muscle stiffness and difficulty in relaxation of muscles after thyroidectomy.[1]. It is characterized by the presence of hypothyroidism with muscle stiffness & pseudohypertrophy
The muscle biopsy taken from hypertrophied calf muscles showed muscle fibre hypertrophy, increased nuclei, mucoid deposits at places with increased interfibreground substance conforming the diagnosis of hypothyroid myopathy with Hoffman’s syndrome
The presence of cores correlated with the severity of hypothyroidism, muscle hypertrophy, cramps & duration of hypothyroid state.[12]

Summary

Introduction

Hoffman’s syndrome was first described by Hoffman in 1897 in an adult who developed muscle stiffness and difficulty in relaxation of muscles after thyroidectomy.[1]. Whereas muscle hypertrophy with weakness & slowness of movement in cretinous children is known as Kocher Debre Semelaigne Syndrome.[2, 3] the two conditions tend to merge into each other & may even occur at different times in the same patient.[4] In India only few cases have been reported so far 2,5,6 & to the best of our knowledge, the present case is the first case reported from this part of the country. There was no history of dyspnoea, chest pain, jaundice, oliguria, constipation, cold intolerance, prolonged fever, hematuria, drug intake, hypertension or diabetes mellitus Her developmental history & milestones were consistent with age. The muscle biopsy taken from hypertrophied calf muscles showed muscle fibre hypertrophy, increased nuclei, mucoid deposits at places with increased interfibreground substance conforming the diagnosis of hypothyroid myopathy with Hoffman’s syndrome. The patient showed significant improvement in the symptoms within 4 weeks.

Discussion

Conclusion