ABSTRACT:The neurological manifestations of hypothyroidism are very unusual to see as initial symptoms and they usually occur late in the course of disease. Hoffmann syndrome is a rare form of hypothyroid myopathy in adults characterized by presence of muscle weakness, stiffness and pseudohypertrophy. We present a case of 27 years old male presenting with features of proximal muscle weakness of both upper and lower limbs with hypertrophy of calf muscles. His TSH and CPK were markedly raised and EMG showed myopathic disorder. We report this case because of its rarity.

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Extramedullary hematopoiesis (EMH) is a well-recognized process in which the body attempts to maintain erythrogenesis in response to an alteration in the normal production of red blood cells. It is observed in hemoglobinopathies, myeloproliferative disorders, neoplasm's involving the bone marrow and other conditions. Commonly observed areas of EMH include the paraspinal regions of the thorax, liver and spleen, but it has been reported in other locations, including the adrenal gland, bowel, dura mater and breast. Our patient presented with E beta-thalassemia along with hypogonadism who was later diagnosed of having EMH by imaging studies.

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