TL;DR: Before and after fatiguing, the instrumented spine was significantly stiffer than the intact spine in flexion, extension, and right and left lateral bending but not in axial rotation; there were no significant differences between the constructs with or without graft-to-plate fixation before or after fatigue.
Abstract: Summary:
After L1 corpectomy in T11–L3 human cadaveric spine, anterior thoracolumbar instrumentation with strut grafting restores spinal stability. T12–L2 angular rotation was measured in response to moments of 0.0, 1.5, 3.0, 4.5, and 6.0 Nm in flexion, extension, lateral bending, and axial rotation, respectively. The spines were tested: 1) intact; 2) after partial L1 corpectomy, grafting, and instrumentation (Profile plate, DePuy-AcroMed, Raynham, MA), with the wooden dowel graft screwed to the plate; 3) without graft screw fixation; and 4) after flexion–extension cyclic fatiguing for 5000 cycles at a load of ±3.0 Nm. Before and after fatiguing, the instrumented spine was significantly (p ≤ 0.05) stiffer than the intact spine in flexion, extension, and right and left lateral bending but not in axial rotation. There were no significant differences between the constructs with or without graft-to-plate fixation before or after fatigue. The instrumented spines were more rigid in bending away from the implant than bending toward the implant. Anterior spinal instrumentation with the Profile implant augments stiffness in the sagittal and coronal planes but not in the axial plane. Although graft-to-plate fixation may prevent graft migration into the canal, it does not contribute to spinal rigidity.
TL;DR: A male patient with rigid spine muscular dystrophy caused by newly identified compound heterozygote mutations of the selenoprotein N gene is presented and this disease is discussed as a possible differential diagnosis for early-onset reduced spine mobility.
Abstract: Early spinal rigidity is a nonspecific feature reported in diseases such as neuromuscular and central movement disorders. We present a male patient with rigid spine muscular dystrophy caused by newly identified compound heterozygote mutations of the selenoprotein N gene and discuss this disease as a possible differential diagnosis for early-onset reduced spine mobility. Rigid spine muscular dystrophy is a rare myopathy presenting in childhood with a typical combination of stable or slowly progressive mild to moderate muscle weakness, limitation in flexion of the spine, and progressive restrictive ventilatory disorder. The clinical features of our patient include early-onset rigidity of his spine, scoliosis, mild muscular weakness predominantly of neck and trunk flexors, and restrictive ventilatory disorder. Biopsy of the biceps muscle revealed nonspecific myopathic changes, and molecular analysis confirmed the diagnosis of rigid spine muscular dystrophy. Thus, neuromuscular diseases such as muscular dystrophy must be considered in all patients presenting with early spinal rigidity, and genetic determination is a possible way to determine the diagnosis.
TL;DR: This case report illustrates the importance of obtaining a detailed medical history when investigating deaths, including nonfatal conditions, such as AS, and shows the value of CT in the evaluation of the mechanism and manner of death.
Abstract: Ankylosing spondylitis (AS) is a chronic rheumatic disease that causes spinal rigidity with an increased risk of spinal fractures. We present a case report where a middle-aged man, in apparent good health, died following a fall from his bike. Postmortem computed tomography (CT) showed several fractures in the cervical and thoracic spine, with displacement into the spinal canal as well as spinal changes consistent with AS. The cause of death was determined to be upper spinal cord injury caused by cervical spinal fractures that were facilitated by spinal rigidity from AS. Further investigation into the medical records revealed that the decedent had previously been treated for AS. This case report illustrates the importance of obtaining a detailed medical history when investigating deaths, including nonfatal conditions, such as AS. Furthermore, it shows the value of CT in the evaluation of the mechanism and manner of death.
TL;DR: This patient is similar to those recently reported as having stiffness and spasms of the legs due to a possible chronic spinal interneuronitis and provides further evidence that this kind of movement disorder may be caused by spinal cord pathology.
Abstract: A patient with a 2(1/2)-year history of painful spasms and rigidity of both lower limbs is described. Symptoms began after an episode of acute myelitis. The spasms--which were spontaneous and stimulus sensitive and occurred on voluntary action--involved the repetitive grouped discharge of motor units. Continuous motor unit activity was present at rest in the muscles of both legs, and cutaneomuscular reflexes were abnormal. This patient is similar to those recently reported as having stiffness and spasms of the legs due to a possible chronic spinal interneuronitis and provides further evidence that this kind of movement disorder may be caused by spinal cord pathology.