1/134. central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection.PURPOSE: central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. patients AND methods: CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly, and abnormal high titers of anti-Epstein-Barr virus (EBV) antibodies. At 8 years of age, he had a splenectomy because of progressive disease. RESULTS: After 27 months of clinical remission, muscle weakness and paresthesia developed. magnetic resonance imaging of his brain showed spotty T2 prolongation in left parietal, bilateral frontal, and temporal white matter with meningeal enhancement. brain biopsy revealed the cerebral infiltration of CD3 , CD4 , CD8-, CD45RO , CD56-, and EBV-encoded rna 1 cells. CONCLUSIONS: The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/134. Chronic axonal sensory and autonomic polyneuropathy without motor involvement: a new 'chronic inflammatory neuropathy?'.We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow-up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the 'chronic inflammatory neuropathies' are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
3/134. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
4/134. optic atrophy and chronic acquired polyneuropathy.Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, multifocal disorder usually defined as limited to the peripheral nervous system. Multifocal motor neuropathy, an acquired demyelinating neuropathy with conduction block affecting motor neurons only, may be a pathogenically distinct syndrome or a predominantly motor variant of chronic inflammatory demyelinating polyneuropathy. central nervous system demyelination including optic neuropathy has been reported uncommonly previously in these entities. We report two cases and review the literature on the possible association of optic neuropathy and chronic acquired polyneuropathy.- - - - - - - - - - ranking = 0.4keywords = nervous system (Clic here for more details about this article) |
5/134. Long term treatment of biliary stricture due to chronic pancreatitis with a metallic stent.The exact role of endoprostheses in the management of chronic pancreatitis-associated biliary strictures has not yet been clearly established. We report an unusual case of a patient with this condition who was treated for an unexpectedly long term with a self-expanding metallic endoprosthesis. There has only been one previous report of the use of metallic stents in this situation. It appears that metallic endoprostheses may have a role to play in the management of selected patients who have chronic pancreatitis-associated bile duct stricture.- - - - - - - - - - ranking = 0.35110868187415keywords = metal (Clic here for more details about this article) |
6/134. Spinal epidural abscess complicating chronic epidural analgesia in 11 cancer patients: clinical findings and magnetic resonance imaging.We reviewed the records of all patients who had received an epidural catheter for management of chronic cancer pain in a 3-year period (1993-1996). patients with nervous system infections were identified, and pertinent clinical, radiological (magnetic resonance imaging), and bacteriological data were analyzed. We identified 11 patients who developed spinal epidural abscess (SEA). All of these had back pain; radicular signs occurred in seven patients and spinal cord compression in two patients. magnetic resonance imaging revealed SEA in all 11 patients. SEA was iso- to hypointense on T1-weighted images and hyperintense on T2-weighted images relative to spinal cord. After gadolinium administration seven lesions showed characteristic rim enhancement while three showed minimal enhancement. No signs of diskitis or osteomyelitis were present, and the abscess was always localized to the posterior epidural space. Cultures were positive in all cases and revealed staphylococcus epidermidis in eight and S. aureus in three. All patients were treated with intravenous antibiotics, and four had an additional decompressive laminectomy. Two patients died within 1 week of diagnosis from overwhelming septicemia despite apparently adequate antibiotic treatment. Within 4 weeks after diagnosis of SEA two patients died from widely metastatic disease, although infection may have contributed. One patient developed septicemia while receiving appropriate antibiotics and underwent emergency laminectomy. The neurological deficits recovered in all patients who survived the acute infectious episode. We conclude that patients with chronic epidural catheters for cancer pain require prompt neurological evaluation and magnetic resonance imaging when SEA is suspected. Early evaluation and treatment may lead to full recovery.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
7/134. The value of serial auditory brainstem response in patients with subacute sclerosing panencephalitis.A total of 98 serial auditory brainstem responses from 17 patients (11 boys and 6 girls) with subacute sclerosing panencephalitis were compared with their clinical course and stages. These patients were exposed to measles early in life (at 1.8 /- 1.4 years old) with the average onset of the disease at 8.8 /- 0.7 years of age. The main abnormalities of their auditory brainstem responses were a prolongation of waves I, III, and V, and of the I to V interpeak interval. Wave V thresholds were also elevated. These effects on the auditory brainstem responses started 1 to 2 years after onset of neurologic signs. I to V interpeak latency became prolonged with the progress of clinical stages, especially in Jabbour's clinically advanced stage IV. In two patients with an acute progressive type of subacute sclerosing panencephalitis, very rapid deterioration with a distorted wave pattern of later components was observed. These findings suggest rostrocaudal progression in the central nervous system of both long-term and fulminant cases.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
8/134. headache in ehlers-danlos syndrome.OBJECTIVE: ehlers-danlos syndrome (EDS) is a complex hereditary connective tissue disorder with neurologic manifestations that include cerebrovascular disorders and chronic pain. The clinical data collected on 18 patients with EDS and chronic headaches is reported. PROCEDURE: Clinical history, neurologic examination, computerized tomography of the head, magnetic resonance imaging (MRI) of the brain, and electroencephalogram (EEG). Headaches were classified according to the International headache Society and the patients were followed by the author for a minimum of 2 years. FINDINGS: Four patients had migraine with aura, four had migraine without aura, four had tension headaches, four had a combination of migraine and tension headaches, and two had post-traumatic headaches. Nine patients exhibited blepharoclonus but none had history of seizures and their EEGs were normal, ruling out eye closure epilepsy. Although one patient had a small right frontal angioma, a second had Arnold Chiari malformation type I, and a third had an old stroke, headaches did not clinically correlate with their central nervous system (CNS) lesions. CONCLUSION: Chronic recurrent headaches may constitute the neurologic presentation of EDS in the absence of structural, congenital, or acquired CNS lesions that correlate with their symptoms. Individuals with EDS may be prone to migraine due to an inherent disorder of cerebrovascular reactivity or cortical excitability. Additional studies are needed to elucidate the pathogenesis of headaches in EDS.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
9/134. Hemosuccus pancreaticus--a rare cause of gastrointestinal bleeding: diagnosis and interventional radiological therapy.hemorrhage from the pancreatic duct, i.e. hemosuccus pancreaticus (HP), is a rare cause of gastrointestinal bleeding. Pancreatic hemosuccus is usually due to the rupture of an aneurysm of a visceral artery, most likely the splenic artery, in chronic pancreatitis. Other causes of HP are rare. We present a case of HP in a female patient with no history but with positive findings of chronic calcifying pancreatitis upon ultrasonographic investigation, computed tomography scan, and endoscopic retrograde cholangiopancreatography. With detectable fresh blood in the descending duodenum, angiography of the celiac artery revealed an aneurysm of the splenic artery as the suspected cause of intermittent bleeding from the pancreatic duct. The treatment is traditionally surgical or by interventional radiological means. This is the first case described in the literature in which interventional radiological therapy involved implantation of an uncoated metal Palmaz stent in the splenic artery. In the follow-up of 18 months no relapse of HP was observed.- - - - - - - - - - ranking = 0.050158383124879keywords = metal (Clic here for more details about this article) |
10/134. Treatment of chronic pain by spinal cord stimulation.chronic pain after injury of the nervous system is difficult to treat. This report describes our experience in the treatment of chronic pain by spinal cord stimulation (SCS) in four patients (three men and one woman, with ages ranging from 61 to 73 yr). One patient had chronic intractable pain due to a spinal cord injury, one had post-herpetic neuralgia, one had failed back surgery syndrome, and one had brachial plexus and spinal cord injuries. A permanent spinal cord stimulator was implanted in the patient with spinal cord injuries. In the other three patients, a permanent spinal cord stimulator was implanted after a successful trial stimulation with temporarily implanted electrodes. After 19 to 25 months (mean, 21 mo) of follow-up, three patients had satisfactory improvement of pain and one patient had temporary pain relief, but pain recurred two months after implantation. One patient had recurrence of pain after migration of an electrode, and the pain-relieving effects of SCS returned after repositioning the electrode. According to this preliminary experience, SCS is effective for pain reduction in selected patients with chronic pain. Long-term follow-up is mandatory to evaluate the benefits and complications of SCS for relief of chronic pain.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
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