Non-compressive myelopathy: clinical and radiological study.
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Fifty seven patients (42 males and 15 females) with non-compressive myelopathy were studied from 1997 to 1999. Acute transverse myelitis (ATM) was the commonest (31) followed by Vit B12 deficiency myelopathy (8), primary progressive multiple sclerosis (5), hereditary spastic paraplegia (3), tropical spastic paraplegia (2), subacute necrotising myelitis (1), radiation myelitis (1), syphilitic myelitis (1) and herpes zoster myelitis (1). 4 cases remained unclassified. In the ATM group, mean age was 30.35 years, antecedent event was observed in 41.9% case, 25 cases had symmetrical involvement and most of the cases had severe deficit at onset. CSF study carried out in 23 patients of ATM revealed rise in proteins (mean 147.95mg%, range 20-1200 mg/dL) and pleocytosis (mean 20.78/cumm, range 0-200 mm3). Oligoclonal band (OCB) was present in 28% of cases of ATM. The most common abnormality detected was a multisegment hyperintense lesion on T2W images, that occupied the central area on cross section. In 6 patients hyperintense signal was eccentric in location. MRI was normal in 4 cases of ATM. Thus ATM is the leading cause of non-compressive myelopathy. Clinical features combined with MRI findings are helpful in defining the cause of ATM. (+info)
Endovascular repair of descending thoracic aortic aneurysms: an early experience with intermediate-term follow-up.
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PURPOSE: The purpose of this study was to report an initial experience with the endovascular repair of descending thoracic aortic aneurysm. Complications and intermediate-term morphologic changes were identified with the intent of altering patient selection and device design. METHODS: Endografts were placed into 25 patients at high-risk for conventional surgical repair over a 3(1/2)-year period. Devices were customized on the basis of preoperative imaging information. Follow-up computed tomography scans were obtained at 1, 3, 6, and 12 months and yearly thereafter. Additional interventions occurred in the setting of endoleaks, migration, and aneurysm growth. RESULTS: The overall 30-day mortality rate was 20% (12.5% for elective cases; 33% for emergent cases). There were 3 conversions to open repair. Neurologic deficits developed in 3 patients; 1 insult resulted in permanent paraplegia. Neurologic deficits were associated with longer endografts (P =.019). Three endoleaks required treatment, and 1 fatal rupture of the thoracic aneurysm treated occurred 6 months after the initial repair. Migrations were detected in 4 patients. The maximal aneurysm size decreased yearly by 9.15% (P =.01) or by 13.5% (P =.0005) if patients with endoleaks (n = 3 patients) were excluded. Both the proximal and distal neck dilated slightly over the course of follow-up (P =.019 and P =.001, respectively). The length of the proximal neck was a significant predictor of the risk for endoleakage (P =.02). CONCLUSION: The treatment of descending thoracic aortic aneurysms with an endovascular approach is feasible and may, in some patients, offer the best means of therapy. Early complications were primarily related to device design and patient selection. All aneurysms without endoleaks decreased in size after treatment. Late complications were associated with changing aneurysm morphologic features and device migration. The morphologic changes remain somewhat unpredictable; however, alterations in device design may result in improved fixation and more durable aneurysm exclusion. (+info)
Delayed onset of ascending paralysis after thoracic aortic stent graft deployment.
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Delayed spinal cord ischemia after thoracic aortic aneurysm repair is an infrequent but devastating complication. The use of stent grafts to exclude aortic aneurysms is thought to decrease the incidence of the neurologic deficit because there is no period of significant aortic occlusion. We report a case of paraplegia that progressed to quadriplegia occurring 48 hours after the apparently successful deployment of a thoracic aortic stent graft. (+info)
Unusual consequences of heroin overdose: rhabdomyolysis, acute renal failure, paraplegia and hypercalcaemia.
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A 29-yr-old man, known to be a heroin addict, was found at home totally unrousable, bent on his hips in the lotus position. On admission, he required frequent ventricular defibrillation, external pacing and infusion of calcium. A diagnosis of rhabdomyolysis caused by heroin and cocaine overdose was made. He developed paraplegia below T12, acute renal failure, acute compartment syndrome in one leg and a coagulation defect. Despite a fasciotomy, a through-knee amputation of the leg was required. Haemodialysis was required for 26 days, and this period was complicated by increased serum calcium concentrations, which was treated with disodium pamindrate. Calcium deposits were palpable in the muscles and could be seen in vessels on limb x-rays. After 34 days, he was eventually discharged to a general surgical ward and subsequently into the community. (+info)
Effects of autonomic disruption and inactivity on venous vascular function.
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The effects of autonomic disruption and inactivity were studied on the venous vascular system. Forty-eight subjects, 24 with spinal cord injury (SCI) and 12 sedentary and 12 active able-bodied controls, participated in this study. Peripheral autonomic data were obtained to estimate sympathetic vasomotor control [low-frequency component of systolic blood pressure (LF(SBP))]. Vascular parameters were determined using strain-gauge venous occlusion plethysmography: venous capacitance (VC), venous emptying rate (VER), and total venous outflow (VO(t)). An additional vascular parameter was calculated: venous compliance [(VC/occlusion pressure) x 100]. VC and VO(t) were significantly different (SCI < sedentary < active). VER adjusted for VC was not different for any group comparison, whereas venous compliance was significantly lower in the SCI group than in the able-bodied groups and in the sedentary group compared with the active group. Regression analysis for the total group revealed a significant relationship between LF(SBP) and venous compliance (r = 0.64, P < 0.0001). After controlling for LF(SBP) through analysis of covariance, we found that mean differences for all venous vascular parameters did not change from unadjusted mean values. Our findings suggest that in subjects with SCI, the loss of sympathetic vasomotor tone contributes more than inactivity to reductions in venous vascular function. Heightened VC, VO(t), vasomotor tone, and venous compliance in the active group compared with the sedentary group imply that regular endurance training contributes to optimal venous vascular function and peripheral autonomic integrity. (+info)
A new locus for autosomal dominant pure spastic paraplegia, on chromosome 2q24-q34.
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Hereditary spastic paraplegia (HSP) comprises a group of clinically and genetically heterogeneous disorders causing progressive spasticity and weakness of the lower limbs. We report a large family of French descent with autosomal dominant pure HSP. We excluded genetic linkage to the known loci causing HSP and performed a genomewide search. We found evidence for linkage of the disorder to polymorphic markers on chromosome 2q24-q34: a maximum LOD score of 3. 03 was obtained for marker D2S2318. By comparison with families having linkage to the major locus of pure autosomal dominant HSP (SPG4 on chromosome 2p), there were significantly more patients without Babinski signs, with increased reflexes in the upper limbs, and with severe functional handicaps. (+info)
A locus for autosomal dominant "pure" hereditary spastic paraplegia maps to chromosome 19q13.
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Genetic loci for autosomal dominant pure hereditary spastic paraplegia (ADPHSP) have been mapped to chromosomes 2p, 8q, 12q, 14q, and 15q. We undertook a genomewide linkage screen of a large family with ADPHSP, for which linkage at all previously identified ADPHSP loci was excluded. Analysis of markers on chromosome 19q gave a peak pairwise LOD score of 3.72 at D19S420, allowing assignment of a novel ADPHSP locus (which we have termed "SPG12") to this region. Haplotype construction and analysis of recombination events narrowed the SPG12 locus to a 16.1-cM region between markers D19S868 and D19S902. (+info)
Spectrum of SPG4 mutations in autosomal dominant spastic paraplegia.
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Autosomal dominant hereditary spastic paraplegia (AD-HSP) is a group of genetically heterogeneous neurodegenerative disorders characterized by pro- gressive spasticity of the lower limbs. Five AD-HSP loci have been mapped to chromosomes 14q, 2p, 15q, 8q and 12q. The SPG4 locus at 2p21-p22 has been shown to account for approximately 40% of all AD-HSP families. SPG4 encoding spastin, a putative nuclear AAA protein, has recently been identified. Here, sequence analysis of the 17 exons of SPG4 in 87 unrelated AD-HSP patients has resulted in the detection of 34 novel mutations. These SPG4 mutations are scattered along the coding region of the gene and include all types of DNA modification including missense (28%), nonsense (15%) and splice site point (26.5%) mutations as well as deletions (23%) and insertions (7.5%). The clinical analysis of the 238 mutation carriers revealed a high proportion of both asymptomatic carriers (14/238) and patients unaware of symptoms (45/238), and permitted the redefinition of this frequent form of AD-HSP. (+info)