Thoracic myelopathy complicating acute meningococcal meningitis: MRI findings

Spinal cord dysfunction is a rare complication of Neisseria meningitidis (meningococcal) meningitis. We report a 17-year-old patient who had a 3-day history of fever, headache and vomiting, agitation, and unresponsiveness. Cerebrospinal fluid showed a marked polymorphonuclear pleocytosis. Latex particle agglutination was positive for meningococci. The patient was given intravenous antibiotics and intravenous dexamethasone. Over the next 4 days, he developed weakness of the lower extremities, with areflexia and extensor plantar responses. MRI revealed contiguous hyperintensities on T2-weighted images involving the thoracic spinal cord from T4 to T9 and 4 brain abscesses. Five months later, he recovered brain function, but the paraparesis remained. This case illustrates that myelopathy may complicate acute meningococcal meningitis, possibly due to a vasculitis, stroke, autoimmune myelopathy, or direct infection of the spinal cord. Patients with myelopathy associated with acute meningitis should receive spinal MRI. In addition, meningitis should be considered in patients presenting with acute myelopathy.     

Transverse myelopathy in systemic lupus erythematosus. Report of three cases and review of the literature.

Three cases of transverse myelopathy associated with systemic lupus erythematosus were reported, and 23 similar cases previously reported were reviewed. A diagnosis of systemic lupus erythematosus was made in only 60% before the onset of transverse myelopathy. The time of onset of myelopathy was randomly distributed during the disease. The most common presenting neurologic symptom was numbness, or weakness of the legs, or both. A unique association between the acute stage of transverse myelopathy and marked reduction of cerebrospinal fluid glucose concentration was observed. Thirteen patients died, 9 had permanent neurologic deficits, and only 4 recovered nearly normal function. Eight patients had ischemic necrosis of the spinal cord at postmortem examination, and vascular lesions were found in the spinal cord of 3 additional patients. The value of steroid treatment was uncertain. Patients who were started on steroid therapy within 24 hours of the onset of myelopathy may have benefited.     

Human immunodeficiency virus type 1 in spinal cords of acquired immunodeficiency syndrome patients with myelopathy: expression and replication in macrophages.

Spinal cord disease is common in patients infected with human immunodeficiency virus type 1 (HIV-1), and a characteristic vacuolar myelopathy is present at autopsy in approximately one-fourth of acquired immunodeficiency syndrome patients. Pathologic examination of the spinal cord shows vacuolation of white matter and infiltration by macrophages, a process distinct from HIV-1 encephalopathy. To determine the presence and localization of HIV-1 RNA in the spinal cords of acquired immunodeficiency syndrome patients with vacuolar myelopathy, we used the technique of combined in situ hybridization and immunohistochemical staining on the same slide. Spinal cord tissue sections were stained with markers for macrophages, endothelial cells, oligodendroglia, astrocytes, and myelin and then hybridized in situ with HIV-1-specific RNA probes. Combined in situ hybridization and immunohistochemical staining on three spinal cords showed HIV-1 expression in mononuclear and multinucleated macrophages localized mainly to areas of myelopathy in spinal cord white matter. Immunohistochemical staining and electron microscopy showed myelin within macrophages and electron microscopy revealed HIV-1 budding from macrophages. These data suggest a role for HIV-1-infected macrophages locally in the pathogenesis of vacuolar myelopathy and add to the body of evidence that these cells play a role systemically in the development of HIV-1-related disease.     

Magnetic resonance imaging in the evaluation of patients with rheumatoid arthritis and subluxations of the cervical spine

We used magnetic resonance imaging (MRI) to examine 21 patients with rheumatoid arthritis and vertebral subluxations of the cervical spine, in whom neurologic symptoms and signs indicated spinal cord compression. Based on neurologic signs, the patients were assigned to 1 of 3 classes: class I, no objective signs of cervical myelopathy (9 patients); class II, only 1 objective sign of cervical myelopathy (4 patients); or class III, 2 or more objective signs of cervical myelopathy (8 patients). Atlantoaxial subluxation (20 patients) and subluxations below C2 (6 patients) were detected equally well by MRI and radiography. MRI revealed physical distortion of the spinal cord in all class III patients with compressive myelopathy. This distortion was found less frequently in class II and class I patients (3 patients), and the difference was statistically significant (P less than 0.005, class III versus class I and class II). No correlation was found between the vertebral dislocation (measured in millimeters) on plain radiographs and the presence of cord distortion on MRI. Myelography in class III patients showed that passage of contrast medium was blocked at the same level as the cord distortion seen on MRI. These findings suggest that MRI can serve as a useful, noninvasive procedure in the diagnosis and management of rheumatoid arthritis patients in whom compressive cervical myelopathy is suspected.     

Computed tomography of paraspinal musculature in ankylosing spondylitis

Computed tomography (CT) was used to delineate the paraspinal musculature in 14 patients with ankylosing spondylitis (AS). Abnormal atrophy of the erector spinae muscles and multifidi was demonstrated in all 8 patients with total bony ankylosis of the spine, but was not present in those with isolated syndesmophyte formation, vertebral squaring alone or sacroiliac joint ankylosis with normal spinal radiographs. There was a significant positive correlation between a CT score of paravertebral muscle wasting and clinical parameters of disease duration and restriction of spinal mobility. Wasting and asymmetry of the psoas muscles was seen in 3 patients with unilateral hip joint involvement. These findings suggest that a relationship exists between decreased or absent spinal movement and atrophy of the paraspinal musculature in AS.     

Clinical features of intradural spinal cord metastases in primary lung cancer]

We retrospectively investigated the clinical features of 9 consecutive cases of intradural spinal cord metastasis from primary lung cancer treated at our hospital between April 1999 and March 2002. During those three years, spinal cord metastasis was diagnosed in seven of 49 (14.3%) cases with small cell carcinoma and only two of 284 (0.7%) cases with non-small cell carcinoma. Eight of the 9 cases had concomitant brain metastasis that preceded spinal cord metastasis and had received brain irradiation. The other patient without brain metastasis had also received prophylactic cranial irradiation. The interval from brain irradiation to the diagnosis of spinal cord metastasis ranged from 116 to 708 days (median 183 days). The most common initial symptom was muscle weakness of the lower extremities in five cases. Seven of the patients rapidly developed transverse myelopathy within two weeks. Magnetic resonance imaging (MRI) with contrast enhancement demonstrated intramedullary tumors in seven cases and intradural extramedullary tumors in two cases. Spinal cord metastasis was often multifocal, and in each case lumbar enlargement was commonly involved. Radiation therapy with or without concurrent chemotherapy produced both neurologic improvement and a tumor response shown on MRI in 4 patients. Heightened awareness of the increasing incidence and background risk factors of this unusual complication could lead to earlier diagnosis and more effective treatment for neurologic palliation.     

Adalimumab significantly reduces both spinal and sacroiliac joint inflammation in patients with ankylosing spondylitis: a multicenter, randomized, double-blind, placebo-controlled study

OBJECTIVE: To compare the efficacy of adalimumab versus placebo in reducing spinal and sacroiliac (SI) joint inflammation, by magnetic resonance imaging (MRI) in patients with active ankylosing spondylitis (AS). METHODS: This was a randomized, multicenter, double-blind, placebo-controlled study. Patients (n = 82) received 40 mg adalimumab or placebo every other week during an initial 24-week double-blind period. MRIs of both the spine and SI joints were obtained at baseline, week 12, and week 52. Spinal and SI joint inflammation were measured using the Spondyloarthritis Research Consortium of Canada (SPARCC) MRI index. RESULTS: The spine SPARCC score in placebo-treated patients increased by a mean of 9.4% from baseline, compared with a mean decrease of 53.6% in adalimumab-treated patients (P < 0.001); the SI joint SPARCC score decreased by a mean of 12.7% from baseline in placebo-treated patients and by 52.9% in adalimumab-treated patients (P = 0.017). The response in adalimumab-treated patients was maintained at week 52. Placebo-treated patients were switched to open-label adalimumab treatment at week 24 and experienced similar reductions in spinal and SI joint inflammation by week 52. Similar large reductions in the spine and SI joint SPARCC scores were noted, even in patients who failed to meet the ASsessment in Ankylosing Spondylitis (International Working Group) criteria (nonresponders) at 12 weeks. In adalimumab-treated patients, a reduced C-reactive protein concentration at week 12 was significantly associated with improvement in the spine SPARCC score (P = 0.018). CONCLUSION: Adalimumab significantly reduced both spinal and SI joint inflammation in patients with active AS after 12 weeks of treatment, and these improvements were maintained for up to 52 weeks.     

Abnormalities of the sacroiliac joints in diffuse idiopathic skeletal hyperostosis: demonstration by computed tomography

Eight patients with classical spinal radiographic features of diffuse idiopathic skeletal hyperostosis (DISH) had pelvic radiographs which suggested sacroiliac joint abnormalities. No patient had clinical features of ankylosing spondylitis. Computed tomography of the sacroiliac joints revealed several abnormalities including asymmetric intraarticular partial fusion, osteophytes with or without bridging, and vacuum phenomenon. Sacroiliac joint disease can complicate DISH.     

Spinal muscular atrophy

Spinal muscular atrophy is an autosomal recessive neurodegenerative disease characterised by degeneration of spinal cord motor neurons, atrophy of skeletal muscles, and generalised weakness. It is caused by homozygous disruption of the survival motor neuron 1 (SMN1) gene by deletion, conversion, or mutation. Although no medical treatment is available, investigations have elucidated possible mechanisms underlying the molecular pathogenesis of the disease. Treatment strategies have been developed to use the unique genomic structure of the SMN1 gene region. Several candidate treatment agents have been identified and are in various stages of development. These and other advances in medical technology have changed the standard of care for patients with spinal muscular atrophy. In this Seminar, we provide a comprehensive review that integrates clinical manifestations, molecular pathogenesis, diagnostic strategy, therapeutic development, and evidence from clinical trials.     

MRI morphometric characterisation of the paediatric cervical spine and spinal cord in children with MPS IVA (Morquio-Brailsford syndrome)

Nearly all children with MPS IVA develop skeletal deformities affecting the spine. At the atlanto-axial spine, odontoid hypoplasia occurs. GAG deposition around the dens, leads to peri-odontoid infiltration. Transverse/alar ligament incompetence causes instability. Atlanto-axial instability is associated with cord compression and myelopathy, leading to major morbidity and mortality. Intervention is often required. Does the presence of widened bullet shaped vertebra in platyspondily encroach on the spinal canal and cause spinal stenosis in MPS IVA? So far, there have been no standardised morphometric measurements of the paediatric MPS IVA cervical spine to evaluate whether there is pre-existing spinal stenosis predisposing to compressive myelopathy or whether this is purely an acquired process secondary to instability and compression. This study provides the first radiological quantitative analysis of the cervical spine and spinal cord in a series of affected children. MRI morphometry indicates that the MPS IVA spine is narrower at C1–2 level giving an inverted funnel shape. There is no evidence of a reduction in the Torg ratio (canal-body ratio) in the cervical spine. The spinal canal does not exceed 11 mm at any level, significantly smaller than normal historical cohorts (14 mm). The sagittal diameter and axial surface area of both spinal canal and cord are reduced. C1–2 level cord compression was evident in the canal-cord ratio but the Torg ratio was not predictive of cord compression. In MPS IVA the reduction in the space available for the cord (SAC) is multifactorial rather than due to congenital spinal stenosis.     

Clinical characterization of neuroschistosomiasis due to Schistosoma mansoni and its treatment

The involvement of the central nervous system (CNS) by Schistosoma mansoni may or may not cause clinical manifestations. When symptomatic, neuroschistosomiasis mansoni (NSM) is one of the most severe presentations of this infection. The neurological manifestations are due to numerous granulomas grouped in confined areas of the spinal cord or the brain. Considering the symptomatic form, myelopathy is far more frequent than the cerebral disease. Spinal cord NSM presents as a low cord syndrome of acute/subacute progression usually associated with involvement of the cauda esquina roots. Lower limbs pain, weakness and sensory disturbance, and autonomic dysfunctions, particularly bladder dysfunction, are often present. Cerebrospinal fluid (CSF) examination generally shows an inflammatory pattern with or without eosinophils and/or IgG against schistosomal antigens. Magnetic resonance imaging (MRI) demonstrates signs of inflammatory myelopathy. Cerebral NSM presents as a slow-expanding intracranial tumor-like lesion. Its clinical manifestations are variable and depend on the increased intracranial pressure and on the site of the lesion. The diagnosis of spinal cord NSM is based on clinical evidence whereas the cerebral disease is usually diagnosed by biopsy of the nervous tissue. There is no consensus on the treatment of NSM. We discuss the literature data on this topic, and suggest a therapeutic approach based on our experience with 69 spinal cord NSM patients who have been followed up by a long period of time. Outcome is largely dependent on early treatment, particularly in the medullar disorder, and is better in cerebral NSM.     

Computed tomography in the diagnosis of early ankylosing spondylitis

Computed tomography (CT) was compared with plain radiography and quantitative sacroiliac (SI) scintigraphy in 28 patients with early ankylosing spondylitis (AS) of less than or equal to 10 years duration. Compared with conventional radiography, CT improved delineation of the SI joints and revealed more abnormalities and higher grades of sacroiliitis; this was significant in patients with early AS of less than or equal to 3 years duration. Quantitative sacroiliac scintigraphy showed higher SI joint: sacrum ratios of radioisotope uptake in patients with AS compared with controls. However, its diagnostic usefulness was limited by the frequency of inconsistent results and the lack of specificity. CT examination of the SI joints may be a useful adjunct in the diagnosis of early AS.     

Neck pain in the elderly: a management review. Part II

Rheumatoid arthritis and metastatic cancer occur commonly in the elderly, and may cause neck pain. Rheumatoid arthritis may produce cervical radiculopathy and myelopathy resulting from vertebral body subluxation, although radiological manifestations of subluxation are much more common than neurological dysfunction. Cervical spinal cord compression is a neurological emergency and may produce cervical radiculopathy as well as myelopathy. Careful neurological and radiological assessments are required to minimize pain and preserve neurological function in elderly patients suffering from neck pain complicating rheumatoid arthritis or cervical spinal metastasis.     

Simultaneous occurrence of diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis in the same patient.

Diffuse idiopathic skeletal hyperostosis (DISH) frequently gives rise to some diagnostic confusion, as it may radiologically mimic ankylosing spondylitis (AS). A patient with features of DISH and AS is described and the literature is reviewed. The diagnostic value of sacroiliac computerized tomography is emphasized. The role of spinal mobility in the appearance of the enthesiophytes is discussed since our patient, who underwent a segmental fusion, presented different radiological features in the mobile segment and the fused region.     

Tomography-guided palisade sacroiliac joint radiofrequency neurotomy versus celecoxib for ankylosing spondylitis: a open-label,randomized, and controlled trial

Sacroiliac joint (SIJ) pain is a common symptom in ankylosing spondylitis (AS). Palisade sacroiliac joint radiofrequency neurotomy (PSRN) is a novel treatment for the SIJ pain. In the current clinical trial, we treated AS patients with significant SIJ pain using PSRN under computed tomography guidance and compared the results with the celecoxib treatment. The current study included 155 AS patients. Patients were randomly assigned to receive PSRN or celecoxib treatment (400 mg/day for 24 weeks). The primary endpoint was global pain intensity in visual analog scale, at week 12. Secondary endpoints included pain intensity at week 24, disease activity, functional and mobility capacities, and adverse events at week 24. In comparison with the baseline collected immediately prior to the interventions, global pain intensity was significantly lower at both 12 and 24 weeks after the treatment in both arms. Pain reduction was more robust in the PSRN arm (by more than 1.9 and 2.2 cm at 12 and 24 weeks in comparison with the celecoxib arm, P < 0.0001 for both). The PSRN was also more effective in improving physical function and spinal mobility (P < 0.05 vs. celecoxib for both). Gastrointestional irritation was more frequent in the celecoxib arm than in the PSRN arm (P < 0.05). No severe complications were noted in either arm. PSRN is both efficacious and safe in managing SIJ pain in patients with AS.     

Spondyloarthritis at the crossroads of imaging, pathology, and structural damage in the era of biologics

Ankylosing spondylitis (AS) is characterized by two key pathologic findings: sacroiliac joint and spinal inflammation and new bone formation with possible bone fusion, usually in the axial skeleton. Thus, structural damage in AS must be viewed differently than that in rheumatoid arthritis. Tumor necrosis factor blockers effectively inhibit inflammation, as shown by signs and symptoms, function, C-reactive protein, and MRI, and will probably prevent erosive structural damage. However, the ossification of already-damaged bone cannot be influenced by tumor necrosis factor blockade, because these drugs do not inhibit osteoblasts. It remains to be seen whether additional targeting of new bone formation is clinically meaningful in advanced AS. The most important action to prevent structural damage is probably an early diagnosis and effective anti-inflammatory treatment of AS.     

Imaging in ankylosing spondylitis

The introduction of symptomatically highly effective anti-tumour necrosis factor alpha therapies for ankylosing spondylitis (AS) has generated interest in the use of imaging to evaluate the potential structure-modifying properties of these agents. Several approaches have been developed to score the plain radiographic abnormalities in AS. Of these, the modified Stoke AS Spinal Score is the most responsive to change, although responsiveness is limited and requires a minimum of 2 years before significant change becomes apparent in patients on standard therapies. Magnetic resonance imaging (MRI) is the most sensitive imaging abnormality, and the advent of fat-suppression imaging allows detection of bone marrow inflammation in the sacroiliac joints as one of the earliest abnormalities in AS. Limited studies have shown that spinal inflammation can be scored reliably using either a system that evaluates the entire spine or a system that limits evaluation to only the most severely affected spinal segments. Both methods also demonstrate excellent responsiveness. The prognostic significance of acute changes on MRI remains unclear. Reliable approaches to the evaluation of chronic changes are yet to be developed. MRI represents a major advance in the diagnostic evaluation of AS.     

Spinal and sacroiliac involvement in SAPHO syndrome: A single center study of a cohort of 354 patients

ObjectiveSAPHO syndrome is a highly heterogeneous disease with distinct treatment response. We report the largest cohort of SAPHO syndrome and explore its clinical classification with special interest in spinal and sacroiliac involvement.MethodsA total of 354 patients with SAPHO syndrome were recruited in Peking Union Medical College Hospital. The demographic, clinical and imaging data were collected at baseline. Spinal and sacroiliac involvement was determined by the co-existence of related symptoms and imaging evidence of lesions in the spine or sacroiliac joints on either bone scintigraphy, CT or MRI.ResultsA total of 197 (55.6%) patients were identified to have spinal or sacroiliac involvement. Compared to those without spinal or sacroiliac lesions, these patients were significantly older at onset (38 ± 12 vs 35 ± 10 years old, p = 0.019) but had comparable duration of disease. Therapeutically, patients with spinal or sacroiliac involvement had been treated more aggressively with more frequently prescribed NSAIDs, glucocorticoids, DMARDs, TNF-α inhibitors, and bisphosphonates (all p ≤ 0.001). Nonetheless, greater disease activity was observed for these patients at baseline, supported by both inflammatory markers (ESR and hs-CRP) and visual analog scale (VAS) for pain (all p < 0.001).ConclusionsSAPHO patients with spinal or sacroiliac involvement are older at onset and have greater disease activity despite of more aggressive treatments compared to those without. Stratified management is in urgent need for this rare disease.     

Protective effect of D-pinitol on the experimental spinal cord injury in rats

Metabolic Brain Disease - Spinal cord injury (SCI) is the major cause of the spinal damage affecting motor and sensory function. Thus, the present study was conducted to investigate the effect of...     

Magnetic resonance imaging for the study of cervical myelopathy in rheumatoid arthritis

Cervical myelopathy is found fairly often with rheumatoid arthritis. It is one of the worst complications of the disease and can lead to tetraplegia or even to sudden death. However, when we consider the high incidence of involvement of the cervical spine in rheumatoid arthritis, the number of cases of cervical myelopathy, even of slight degree, is not very high. We have used magnetic resonance to identify the condition of the cervical structures, especially the nerve structures, in 15 patients with rheumatoid arthritis, with involvement of the cervical articulations but without neurological symptoms. We found anterior compression of the spinal cord caused by the odontoid process of the epistropheus in 13 cases. One case had lateral deviation of the spinal cord and another had compression of a vertebral artery. In another the lumen of the nasopharynx was decreased and one had posterior compression of the spinal cord by the posterior arch of the atlas. Magnetic resonance also makes it possible to detect a rheumatoid pannus on the affected articulations. We conclude that magnetic resonance is at present a useful instrument for evaluation of the presence of cervical myelopathy in rheumatoid arthritis patients, to prevent more serious complications.