Introduction Hoffman’s syndrome is an extremely rare, potentially treatable, form of myopathy requiring high clinical suspicion for diagnosis with routine measurement of serum TSH. Presenting with increased muscular mass (pseudohypertrophy), proximal muscle weakness, stiffness, and cramping it may be complicated by renal failure and ATN. Calf muscles are typically affected in Hoffman’s; however, patient presented postpartum with proximal upper limb involvement. Case Presentation Caucasian 23 year old female presented with fatigue, generalized weakness, and severe muscle aches only bilaterally in upper extremities with low grade fever and numbness in arms and legs for 5 weeks, preceded by upper respiratory infection treated with Levaquin with no improvement. Her past medical history is significant for asthma and seasonal allergies. She had a normal vaginal delivery and gave birth to a full term healthy baby 4 months prior to this presentation. She had no symptoms of diplopia, ptosis, difficulty chewing, dysphagia, urinary or bowel function changes. Examination showed a well nourished, average height, normocephalic, with BMI of 31, no periorbital swelling, normal thyroid, cardiac, and respiratory exam. Musculoskeletal exam showed bilateral upper arm pseudohypertrophy and severe weakness with 2+ strength, with normal straight leg raise test. Labs showed TSH markedly elevated at 603.02, T3 uptake 21.6, total T4 0.5, and T7 extremely low at 0.1. CPK was 1,800. Liver function tests were nonspecific for transaminitis with SGOT 63 and SGPT 1529, creatinine and BUN were normal. Antinuclear antibody tested negative. Acetylcholine receptor binding antibody was less than 0.8 nmol per liter. Thyroid peroxidase autoantibodies > 1,000 IU per ml. Erythrocyte sedimentation rate was 9. Thyroid sonogram showed mildly enlarged heterogenous thyroid gland with no nodules, approximately 3.8x1.0x1.0cm on the right and 4.0x1.0x1.0cm on the left. Electromyography demonstrated trace fibrillations with increased muscle discharge, paramyotonic pattern, and delayed relaxation phase. By exclusion, diagnosis of severe hypothyroidism with neuromuscular dysfunction was made. Patient started 50mcg levothyroxine with profound improvement. Discussion There are 4 subtypes of myopathy associated with hypothyroidism: Hoffmann’s syndrome, myasthenic syndrome, atrophic form, and Kocher-Debre-Semelaigne syndrome (pediatric form of myopathy). The pathophysiology is explained by reduced glycolysis and oxidative phosphorylation within the muscle tissue, with decrease in ATP phosphorylation beyond a critical limit, generating a marked release in CPK. Lab findings include CK, TSH, FT3, FT4, FT7, TgAb, and TPOAb (determine the autoimmune links). EMG and electroneurography assess the degree of peripheral nerve impairment. Muscle MRI may differentiate hypothyroid myopathy from muscular dystrophy Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.
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