Cases reported "Nervous System Diseases"

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1/33. Embolization of arteriovenous malformations with Onyx: clinicopathological experience in 23 patients.

    OBJECTIVE: To report our experience in treatment of arteriovenous malformations (AVMs) using a new liquid embolic agent, Onyx (Micro therapeutics, Inc., Irvine, CA). methods: Between January 1998 and May 1999, 23 patients (8 men and 15 women) were treated. The patients' average age was 40 years, with seizure being the most common presenting symptom (39%). The average Spetzler-Martin grade on presentation was 3. The average AVM volume before embolization was 14.5 cm3. RESULTS: We observed an average 63% reduction in AVM volume after 129 arterial feeders were embolized. There were four adverse events. Two patients experienced ischemia because of inadvertent occlusion of an arterial feeder. One of these patients made a full recovery, but the other patient had a permanent deficit. Two other patients experienced transient neurological deficits that resolved within 1 week of embolization. Permanent morbidity was thus 4% (1 of 23 patients). There were no deaths. Twelve patients underwent subsequent radiosurgery, and 11 patients had surgery that resulted in complete resection of their AVMs. Histopathological examinations showed mild acute inflammation in specimens resected 1 day after embolization. Chronic inflammatory changes were observed in specimens resected more than 4 days after embolization. In two patients, angionecrosis of the embolized vessels was noted. No evidence of parenchymal hemorrhage was observed in these patients, and vessel wall integrity was maintained as well. CONCLUSION: Onyx is a new nonadhesive liquid embolic agent that has been used to treat 23 patients at our institution with good results. Its nonadhesive nature and ease of use make it a promising agent in the future treatment of AVMs.
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2/33. Neurological deficit after surgery for spinal deformity--the value of normal intra operative wake-up tests in large sagitta curves. A report of two cases from an institution.

    Neurological deficit after surgery for spinal deformity is a rare but devastating complication. Factors that have been associated with a post surgical deficit are congenital curves, large curves, kyphotic deformities, anterior and posterior surgery, ligation of multiple anterior vessels and hypotension; controlled or otherwise. Intra operative wake up tests have been used alone or in combination with spinal cord monitoring: to detect evolving neurological deficit in spinal deformity surgery. Despite these checks, major neurological deficits still occur post surgery. This is a report of two cases with normal intra operative wake up tests, but developed neurological deficit twelve hours after the end of surgery.
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3/33. The neurological masquerade of intravascular lymphomatosis.

    BACKGROUND: Intravascular lymphomatosis (IVL) is an uncommon systemic disease characterized by occlusion of small vessels by malignant lymphomatous cells. central nervous system involvement usually presents as subacute encephalopathy, dementia, seizures, or multifocal cerebrovascular events. OBJECTIVE: To increase awareness about IVL, an uncommon cause of neurological disease. DESIGN: This is a retrospective case series of 8 pathologically proved cases of IVL with neurological disease. patients were part of a pathological series collected between April 1962 and October 1998 at indiana University School of medicine and the Armed Forces Institute of pathology, washington, DC. SETTING: Neurological and neuropathological examinations were performed at tertiary referral hospitals. patients: Eleven patients were diagnosed pathologically as having IVL, but 3 were not included in this evaluation because of a lack of appropriate clinical information. Of the final sample (n = 8), there were 4 men and 4 women (mean /- SD age, 62.9 /- 9.9 years). RESULTS: All 8 patients had focal neurological deficits, 7 had encephalopathy or dementia, 5 had epileptic seizures, and 2 had myelopathy. death occurred at a mean of 7.7 months (range, 1-24 months) after the onset of symptoms. All patients had elevated cerebrospinal fluid protein levels, 4 had pleocytosis, and 2 had an elevated IgG level in their cerebrospinal fluid. Of the 4 patients who underwent a brain biopsy, 1 was diagnosed as having IVL before death. CONCLUSIONS: Intravascular lymphomatosis is an uncommon disease with a myriad of potential neurological manifestations. diagnosis requires a high index of suspicion and a pathological examination. If diagnosed early, aggressive chemotherapy is potentially curative, although the overall prognosis remains dismal.
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4/33. Neuro-ophthalmic sarcoidosis.

    sarcoidosis is a multisystem disorder in which ocular involvement occurs in about one-quarter and neurosarcoidosis in 7 per cent of patients. When the retina is involved, the reported incidence of central nervous system sarcoidosis is 37 per cent. The patient described had a transient papular eruption of the legs, bilateral hilar lymphadenopathy, polyarthralgia with knee effusions, and bilateral facial and peripheral neuropathy. Ocular involvement was characterized by anterior uveitis (in the initial stages), vitreous flare, bilateral disc oedema, macular oedema, streak haemorrhages, peripheral periphlebitis, nerve fibre bundle defects, and candle-wax spots. fluorescein angiography showed no fluorescence of the candle-wax spots nor of the adjacent vessels. However, there was hyperfluorescence of two retinal lesions. This patient had unilateral internal ophthalmoplegia, only three cases of which have been reported in the literature. Her health was restored by heavy, prolonged corticosteroid therapy. Her family history revealed that an uncle died of sarcoidosis complicated by cryptococcal meningitis. The literature on retinopathy in sarcoidosis is reviewed and the lesions noted in the posterior segment are listed.
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5/33. Human T-cell lymphotropic virus type 1-associated retinal vasculitis in children.

    PURPOSE: To describe predominant retinal vasculitis in children carrying human T-cell lymphotropic virus type 1 (HTLV-1). methods: The authors examined clinical records of patients with HTLV-1-associated uveitis between 1987 and 2001 in Kagoshima University Hospital and reviewed cases of extensive, smoldering retinal vasculitis. RESULTS: Three previously healthy teenagers noted mild visual symptoms and presented with extensive sheathing of retinal vessels, complicated by mild anterior segment inflammation. The retinal vascular disease responded poorly to systemic corticosteroids, had a smoldering course with persistent sheathing of retinal vessels, and eventually resulted in diffuse chorioretinal degeneration. Results of laboratory studies were unremarkable except for the presence of serum antibodies to HTLV-1. One patient developed HTLV-1-associated myelopathy 11 years after the onset of ocular disease. CONCLUSIONS: The retinal vasculitis differed from the retinal vascular changes commonly seen in HTLV-1-associated uveitis. The authors suggest a clinical disease HTLV-1-associated retinal vasculitis that affects young HTLV-1 carriers, characterized by smoldering retinal vasculitis with ultimate retinal degeneration.
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6/33. Neurological deficit from a purely vascular etiology after unilateral vessel ligation during anterior thoracolumbar fusion of the spine.

    STUDY DESIGN: Comprehensive analysis of patient records who underwent anterior approach to the thoracolumbar spine at a single institution. OBJECTIVES: To report on neurologic deficit from a purely vascular injury to the spinal cord occurring after unilateral anterior thoracolumbar spinal surgery that did not involve additional correction or other etiologies. SUMMARY OF BACKGROUND DATA: The largest study in the literature regarding the risks of neurovascular deficit during anterior exposure of the thoracolumbar spine reports that there exists no risk with unilateral ligature of the segmental arteries. methods: The records and operative notes of 265 consecutive patients were reviewed. All adult neurologically intact patients, average age 40.2 years (range 18-85 years) who have had surgery between 1985 and 2002 that involved anterior approach to the thoracic spine were included. Segmental arteries were ligated midbody, away from the foramen and the aorta. Seventy-two percent of the approaches were left-sided. An average of 5.1 unilateral segmental artery ligations were performed per procedure. RESULTS: Two patients out of 265 had major neurologic deficit after anterior thoracolumbar approach. Both patients had staged procedures: posterior spinal fusion then anterior spinal fusion to the thoracolumbar spine. One deficit occurred immediately after surgery and the other occurred 24 hours after surgery. No additional corrective maneuvers were performed; neither patient was hypotensive, nor did they experience blood loss anemia and their postoperative computed tomography myelogram study was normal. CONCLUSION: Neurologic deficit after anterior exposure to the thoracolumbar spine occurred in 0.75% of patients in this study exclusively from unilateral left-sided ligation of the T10-T12 segmental vessels. Both patients had common risks of prior kyphosis correction, revision surgery and left-sided approach.
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7/33. Cord monitoring changes and segmental vessel ligation in the "at risk" cord during anterior spinal deformity surgery.

    STUDY DESIGN: Retrospective analysis of all cases of anterior spinal deformity surgery that had intraoperative spinal cord monitoring (somatosensory-evoked potentials, SSEPs). OBJECTIVES: The prime purpose of this study was to determine the incidence of significant SSEP changes in patients undergoing anterior spinal deformity surgery. A secondary objective was to ascertain if patients with "cords at risk" were more likely to produce significant intraoperative SSEP changes and what proportion of these changes resulted in postoperative neurological deficit. SUMMARY OF BACKGROUND DATA: There is anecdotal evidence to suggest that patients with intraspinal abnormalities are at greater risk of postoperative neurological deficit after spinal deformity surgery. To date, there have been no studies detailing this risk and how it relates to the type of surgery performed. This is a question of increasing relevance with the modern trend towards more anterior scoliosis correction and instrumentation. Recent reports have suggested a low incidence of neurological complication with anterior deformity procedures. There is controversy as to whether SSEP monitoring is required for these anterior procedures and whether soft clamping of segmental vessels before their division is a necessary precaution. METHOD: This study is a chart review of all patients who had an anterior deformity operation between 1990 and 2001. Those patients who had a complete data set (preoperative magnetic resonance imaging scan, patient and procedural documentation, and adequate intraoperative SSEP traces) were included in this study. A significant SSEP change was correlated with the type of procedure performed, whether that patient had a "cord at risk" (CAR) and the degree of postoperative neurological deficit if present. RESULTS: During the study period, 871 patients underwent elective anterior spinal deformity surgery. Ninety five (11%) patients had intraspinal abnormalities on magnetic resonance imaging. From this group, 27 (3%) were termed CAR. Twenty six (3%) patients had significant change in the intraoperative SSEP monitoring. Seventeen (2% total) occurred in the CAR group and nine (1% of total) in the normal cord group. There were five patients (0.6%) with significant postoperative neurological deficits, four (0.5%) in the CAR group, and one (0.1%) in the normal cord group. These patients had also demonstrated changes in their SSEPs. The sensitivity of SSEP monitoring for the whole series was 100%, specificity 97.5%, the positive predictive value was 19% and the negative predictive value was 100%. The CAR group was significantly more likely to have significant SSEP changes during any operation and was more likely to have postoperative paresis. CONCLUSION: patients with identified cords at risk should undergo spinal cord monitoring (SSEP) if they undergo anterior spinal deformity surgery. Soft clamping of segmental vessels is indicated with cord monitoring to prevent the risk of postoperative neurological sequelae.
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8/33. The neurological implications of fibromuscular dysplasia.

    fibromuscular dysplasia is a noninflammatory segmental disease of the arteries, of unknown origin. While the renal arteries are most commonly affected, other larger vessels, including the carotid and vertebrals, may be involved. fibromuscular dysplasia has been implicated as a cause of stroke in adults and (on rare occasions) in children. The classic angiographic presentation is that of a string of beads. The condition frequently requires surgical or interventional radiological treatment. These approaches most commonly lead to long-term neurological improvement. Five cases with typical angiographic appearance are presented.
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9/33. Diffuse-type gastric cancer in a young adult presenting with neurological symptoms and liver rupture.

    A 27-yr-old man presented with neurological symptoms and subsequent painful liver enlargement. Cranial computerized tomographic scans and gastroscopy were negative. Ultrasound examination revealed nonhomogeneous echo pattern of the enlarged liver; a guided biopsy specimen suggested hepatoma. The patient died of hemorrhagic shock 5 days after liver biopsy and 4 wk after the initial presentation. autopsy revealed diffuse-type gastric carcinoma as the primary lesion, liver ruptures at sites distant from the biopsy, thrombosis of the sagittal sinus, and widespread permeation of blood and lymphatic vessels with anaplastic carcinoma cells. The incidence of gastric cancer in young adults from the Cancer Registry of slovenia is presented for the period from 1979 to 1987. Our case corroborates the importance of considering this malignancy early in the evaluation of young symptomatic patients.
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10/33. Histopathological findings in the central and peripheral nervous systems in neuroborreliosis. A report of three cases.

    Very little is known about tissue changes and pathophysiological mechanisms in Garin-Boujadoux-Bannwarth syndrome. We report histopathological findings in the central and peripheral nervous systems of three typical cases. In the acute stage of the disease mononuclear perivascular infiltrations with mainly T-helper cells were the prominent finding, whereas after treatment there was no vessel involvement. The fibre changes in the peripheral nervous system consisted of axonal degeneration. No borrelia burgdorferi-specific antigen could be detected by immunohistochemical methods. Thus vasculitis might be one of the primary pathophysiological mechanisms for the involvement of the nervous system.
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