European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
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Alström syndrome (AS) is a rare autosomal recessive genetic disorder with multisystemic involvement characterised by early blindness, hearing loss, obesity, insulin resistance, diabetes mellitus, dilated cardiomyopathy, and progressive hepatic and renal dysfunction. The clinical features, time of onset and severity can vary greatly among different patients. Many of the phenotypes are often not present in infancy but develop throughout childhood and adolescence. Recessively inherited mutations in ALMS1 gene are considered to be responsible for the causation of AS. Musculoskeletal manifestations including scoliosis and kyphosis have been previously described. ⋯ Here, we present a patient with AS who presented with cervical myelopathy due to extensive flowing ossification of the anterior and posterior longitudinal ligaments of the cervical spine resulting in cervical spinal cord compression. The presence of an auto-fused spine in an acceptable sagittal alignment, in the background of a constellation of medical comorbidities, which necessitated a less morbid surgical approach, favored a posterior cervical laminectomy decompression in this patient. Postoperatively, the patient showed significant neurological recovery with improved function. Follow-up MRI showed substantial enlargement of the spinal canal with improved space available for the spinal cord. The rarity of the syndrome, cervical myelopathy due to ossified posterior longitudinal ligament as a disease phenotype and the treatment considerations for performing a posterior cervical decompression have been discussed in this Grand Rounds' case presentation.
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We describe cases presenting with progressive thoracic myelopathy after lumbopelvic fusion attributed to proximal junctional vertebral compression fracture (PJF) followed by spinal calcium pyrophosphate dehydrate (CPPD) crystal deposition. ⋯ The combination of mechanical stress plus PJF and CPPD crystal deposition followed by a foreign body reaction to the deposited crystals caused myelopathy. Patients with radiographic evidence of PJF, especially UIV collapse, after lumbopelvic fusion should be followed carefully for the emergence of myelopathy.
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Literature has described a risk for subsequent vertical subluxation (VS) and subaxial subluxation (SAS) following atlantoaxial subluxation in rheumatoid patients; however, the interaction of each subluxation and the radiographic findings for atlantoaxial fixation has not been described. The purpose of this study was to evaluate the effects of two different posterior atlantoaxial screw fixation on the development of subluxation in patients with rheumatoid atlantoaxial subluxation. ⋯ Atlantoaxial posterior screw fixation may be an appropriate prophylactic intervention for VS and SAS if the atlantoaxial joint develops bony fusion following physiological alignment. Compared to TA, SR provided optimal atlantoaxial angle and prevented lower adjacent segment degeneration, thereby reducing SAS.
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To investigate relevant change on the Neck Pain and Disability Scale (NPAD) and Neck Disability Index (NDI) and which questionnaire is the most responsive in patients with non-specific chronic neck pain (CNP). ⋯ Relevant change on both NPAD and NDI assessed with MDC and MIC resulted in different cut-offs and consequently with different amounts of certainty that the patient is improved. Responsiveness of NPAD and NDI was similar.
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To investigate the effect of omega-3 fatty-acid supplements (n-3FA) on bleeding during posterior spinal arthrodesis. ⋯ The n-3FA use did not contribute to higher perioperative blood loss during spinal arthrodesis.