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AB0213 Rare complications of paget’s disease
  1. C Resende,
  2. W Castelão,
  3. H Canhão,
  4. JE Fonseca,
  5. JC Romeu,
  6. JA Pereira Silva,
  7. M Viana Queiroz
  1. Rheumatology Unit, Santa Maria Hospital, Lisbon, Portugal

Abstract

Background Paget Disease?s (PD) myelopathy and chondrosarcoma are rare entities. The spinal cord compromise is due to the direct compression of the growing bone and also to spine cord ischaemia. Chondrosarcoma is a rare malign degenerescence of the bone disease.

Results Case 1: A 74 years old man with the previous diagnosis of polyostotic PD was admitted to our inpatient Unit with lower limbs paraparesis that began 2 months earlier. He had a spastic gait, lower limbs paresis 4/5, increased patellar and Achilles tendon reflexes (more prominent on the left), extension plantar reflexes, T6/T7 sensory level without sphincteric dysfunction. Both alkaline phosphatase and hydroxyprolinuria were high. Thoracic spine CT scan and MRI showed myelopathy at T5, T6 and T7 due to spine PD compression. Therapy consisted of pamidronate 180 mg (2 infusions of pamidronate 90 mg, 2 weeks apart) and 200 UI i.d. of inhaled calcitonin. After therapy a clear clinical and laboratorial improvement was observed. After 1 year the gait and neurological observation were normal.

Case 2: A 76 years old woman with PD involving the thoracic spine and iliac bone, was admitted to our Unit with lower limbs paraparesis, nocturnal back pain and right gluteal pain irradiating to the posterior aspect of the thigh with 4 months duration. Neurological evaluation showed paraparesis grade 1–2/5 on the right lower limb and grade 4/5 on the left, decreased right patella reflex and sensory level T12-L1. A large stony mass was palpable in the right iliac fossae and in the gluteal area. Bone turnover markers were high. Spine CT scan and MRI showed myelopathy due to PD vertebrae in T12-L1. Thoracic and abdominal CT scan revealed a bone tumour of the right iliac and pulmonary metastases. Histology of the tumour revealed a chondrosarcoma. She started calcitonin 200 UI i.d. and intravenous dexamethasone 5 mg t.i.d. with neurological improvement. She suffered an episode of sudden death 1 month later at home.

Conclusion In cases of PD with spinal cord symptoms, PD myelopathy should be the first clinical suspicion. Prompt diagnosis and therapy should be instituted to prevent irreversible neurological damage. Chondrosarcoma is an agressive tumour, non responsive to standard therapies for bone malignancies.

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