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We report the case of a 10-year-old boy with neuro-Behçet's disease. Spinal cord MRI showed signal abnormalities in the cervical and thoracic cord, but cerebral CT and MRI revealed no abnormality.
X-linked hypophosphataemic rickets is a familial form of Vitamin D resistant rickets in which gross bony and ligamentous changes may occur. Two patients showing severe spinal disease with evidence of spinal cord compression requiring neurosurgical intervention are reported. The management of such lesions may be problematic as cord compression may be found at several levels at presentation, and further difficulties develop after neurosurgical treatment.
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Authors analyse the group of 22 operated patients admitted to the Spinal Centre of the Traumatological Hospital Brno for instable spinal fracture with the transversal lesion of the spinal cord, in whom the acutely affected abdomen and acute surgical disease manifested. The anamnestic and clinic examination of the abdomen is not reliable in patients with the transversal lesion of spinal cord for valid laws of the spinal cord. Authors evaluate the contribution of new diagnostic imaging techniques and endoscopic methods to determinate the early indication for surgical treatment.
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Prior to the advent of MR imaging, the internal architecture of the spinal cord could not be directly imaged. The solution of many technical problems (e.g., respiratory motion, cardiac and CSF pulsation, inadequate spatial resolution) has provided the opportunity for an increasingly refined analysis of intramedullary lesions. This article begins with a brief review of the results of high-resolution MR imaging studies of the cadaveric spinal cord. The article then focuses on MR imaging in the diagnosis of intramedullary diseases that involve specific neural pathways or vascular territories. Lesions are categorized as degenerative, inflammatory, traumatic, or ischemic. These diseases generally have distinctive clinical findings that reflect dysfunction of particular ascending sensory tracts or descending motor tracts. The corresponding abnormalities on MR images reflect the pathologic changes that occur in the affected neural pathways. Knowledge of the appearance of these diseases on MR images allows the formation of a narrow differential diagnosis and, in many cases, the confident exclusion of neoplasm as the cause of myelopathy.
One hundred and eighty-one patients attending a dermatology clinic were studied. Twenty-two (12%) were B27 positive. Twenty had peripheral psoriatic arthritis; 3 of these showed sacroiliitis (1 B27 positive, 2 B27 negative). Only one of the other 161 patients had sacroiliitis and he was B27 positive. Subsequently we examined 54 consecutive patients with psoriatic arthritis: 51 had peripheral arthritis (6 with sacroiliitis) and 3 exclusively axial involvement (2 sacroiliitis, one syndesmophytes with normal sacroiliac joints). All patients were pooled together and divided in 4 groups: B27 positive and negative, with or without peripheral arthritis. HLA-B27 and/or peripheral arthritis were associated with an increase in axial involvement. Patients lacking both B27 and peripheral arthritis did not have sacroiliitis and only in one case showed evidence of spinal disease (0.7%). Half of the patients with peripheral arthritis and spinal involvement were B27 positive. All 3 B27 positive patients without peripheral arthritis and with spinal disease were men and they all had bilateral sacroiliitis, indistinguishable from idiopathic ankylosing spondylitis (AS). HLA-B27 and peripheral arthritis appeared to act as separate factors that increased the risk of spinal arthritis in patients with psoriasis. The effect of B27 on psoriasis appeared to be detected in 2 different ways: as a coincidental factor increasing the risk of idiopathic AS (as for the general population) or as one of the multiple HLA associations that increase the risk of psoriatic arthritis; in this latter case the spinal involvement could occur as another manifestation of the clinical course of the disease.
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Two unrelated patients with hereditary spherocytosis developed idiopathic spinal cord dysfunction. This coincidence, combined with similar individual case reports in the older European literature, suggests that abnormalities may exist in constituents common to red cell membranes and nerve tissue. A similar conclusion has been proposed to explain red cell membrane abnormalities in some of the muscular dystrophies.
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