Mujer con artralgias y aumento de tamaño de miembros superiores

Abstract

We present a 32-year-old woman with a history of hypothyroidism, on replacement therapy, menstruating and with no children. She had no family history of osteoarticular disease. She told us that she had been diagnosed with muscular dystrophy when she was four years old in her country of origin. She had no medical notes or history for this, stating that this diagnosis had been given because she had been late in starting to walk and that no muscular biopsy or specific study had been carried out. She was consulting due to mechanical arthralgia of lengthy evolution, with no joint swelling, no morning stiffness; the patient said her upper limbs had enlarged over the years and the diameter of her thighs and legs had also increased. Originally she had gone to our hospital’s Neurology Department, where analysis gave normal CPK, an electromyogram showed no evidence of myopathy and an MRI of the pelvic girdle did not reveal myositis; consequently, she was sent to our department for study. The examination highlighted the absence of synovitis, generally limited passive and active joint distances, normal muscular strength and an increase in the upper limb length. Her walk also drew attention with a certain oscillation of the pelvis, exaggerated lumbar lordosis and latero-lateral sway. The tendon reflexes were normal and there was no deformity in the toes or fingers. The simple radiological study carried out showed cortical thickening and sclerosis of the shafts of tubular bone. There was irregular and heterogeneous osteosclerosis, mottled areas of radiolucency with greater endosteal than periosteal involvement and narrowing of the spinal canals (Figure). The epiphyses were respected radiologically. Densitometry of femoral neck had a Z-score of +6.2.