1/281. The rontgenographic findings in the acute neuronopathic form of Niemann-Pick disease.The rontgenographic changes are described in two patients with the acute neuronopathic form of Niemann-Pick disease. These consist of metaphyseal splaying, osteoporosis and the quadrate appearance of the lumbar vertebrae with relatively long pedicles. The parenchymatous involvement is manifested by interstitial lung changes, enlargement of liver, spleen and kidney and distended intestinal loops with an abnormal mucosal pattern associated with prolonged transit time of the contrast material. The differential diagnosis of the above changes is discussed.- - - - - - - - - - ranking = 1keywords = disc (Clic here for more details about this article) |
2/281. Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors.BACKGROUND: Antiamphiphysin antibodies react with a 128-kd protein found in synaptic vesicles.They were first described in patients with paraneoplastic stiff-man syndrome and breast cancer, but studies suggest that they can also occur in patients with other tumors and neurological disorders. OBJECTIVE: To determine if antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors. patients AND methods: Of 2800 serum samples tested by routine immunohistochemical procedures on sections of paraformaldehyde-fixed rat brain for the detection of autoantibodies associated with paraneoplastic neurological syndromes, 5 were selected because of labeling suggestive of antiamphiphysin antibodies and subsequently confirmed by the results of Western blot analysis using recombinant amphiphysin protein. Controls consisted of 40 patients with various nonparaneoplastic neurological diseases; 101 patients with cancer but without paraneoplastic neurological syndrome; 9 patients with small cell lung cancer, anti-Hu antibodies, and paraneoplastic neurological syndrome; 3 patients with M2-type antimitochondrial antibodies but no neurological disorder; and 30 normal subjects. RESULTS: Of the 5 patients with antiamphiphysin antibodies, patient 1 had sensory neuronopathy, encephalomyelitis, and breast cancer; patient 2 had limbic encephalitis, and small cell lung cancer was detected in the mediastinum after 24 months of follow-up; patient 3 had encephalomyelitis and ovarian carcinoma; and patients 4 and 5 had lambert-eaton myasthenic syndrome and small cell lung cancer (patient 4 subsequently developed cerebellar degeneration). None of the 5 had stiffness. Two patients (Nos. 2 and 4) had antimitochondrial antibodies. The two patients (Nos. 4 and 5) with lambert-eaton myasthenic syndrome had antibodies directed against the voltage-gated calcium channel, and patient 2 subsequently developed anti-Hu antibodies. In the controls, antiamphiphysin antibodies were detected by Western blot analysis in 3 of 8 patients with anti-Hu antibodies, but in none of the other groups. CONCLUSIONS: These data indicate that antiamphiphysin antibodies are not specific for one type of tumor or one neurological syndrome and can be associated with other neural and nonneural antibodies. The simultaneous association of several antibodies in some patients suggests multimodal autoantibody production.- - - - - - - - - - ranking = 44.553919837494keywords = degeneration (Clic here for more details about this article) |
3/281. Parenchymatous cerebral neurocysticercosis in a quadriplegic patient.OBJECTIVE: To present and discuss a case of cerebral neurocysticercosis in a quadriplegic patient. DESIGN: Case report of a case of neurocysticercosis in a high level spinal cord injury (SCI) patient who developed episodes of autonomic dysreflexia and orthostatic hypotension associated with transient neurologic deficits and seizures. SETTING: spinal cord Unit of the University Hospital of Geneva, switzerland. SUBJECT: Single patient case report. MAIN OUTCOME MEASURE: Clinical and radiological magnetic resonance imaging follow-up of the patient between July 1995 and October 1997. RESULTS: Treatment of cysticercosis with praziquantel relieved the patient from autonomic dysreflexia, symptomatic orthostatic hypotension, transitory neurological deficits and seizures. CONCLUSION: diagnosis of neurocysticercosis in a quadriplegic patient might be difficult because of frequent overlaps with some usual symptoms occurring in high level SCI, mostly autonomic dysreflexia and orthostatic hypotension. neurocysticercosis should be kept in mind when a SCI patient living in, or coming from endemic zones presents with new neurological abnormalities and seizures. magnetic resonance imaging appears to be more sensitive than computerised tomography to confirm the diagnosis of active cysticercosis. Treatment with praziquantel associated with cimetidine to increase the drug bioavailability and prednisone to reduce the inflammatory reaction gives good results.- - - - - - - - - - ranking = 1keywords = disc (Clic here for more details about this article) |
4/281. Prolonged severe 5-fluorouracil-associated neurotoxicity in a patient with dihydropyrimidine dehydrogenase deficiency.5-fluorouracil (5-FU) is an analogue of pyrimidine nucleosides that is widely used in the treatment of head and neck, breast, ovarian, and colon cancer. stomatitis, diarrhea, dermatitis, and myelosuppression are the main toxicities of 5-FU. A less frequent side effect that is becoming more recognized is neurologic toxicity. Dihydropyrimidine dehydrogenase (DPD) is the rate-limiting enzyme in the catabolism of 5-FU. DPD deficiency follows an autosomal recessive pattern of inheritance, and its prevalence is estimated to be 3%. Cancer patients who are receiving 5-FU treatment and are DPD deficient can develop severe side effects. The neurologic toxicity can vary from being mild to severe and prolonged. We describe the side effects of 5-FU in a colon cancer patient who suffered severe mucositis, desquamating dermatitis, prolonged myelosuppression, and neurologic toxicity that required admission to the intensive care unit. The patient remained hospitalized for 3 months. Recovery from the side effects was complete 4 months after the last 5-FU treatment. Subsequent testing revealed that this patient has an extremely low level of DPD activity (0.015 nmol/min/mg protein; mean, 0.189 nmol/min/mg protein). Because neurologic toxicity is becoming more recognized and DPD affects the catabolism of 5-FU, we discuss management issues and the use of new DPD inhibitors. We also discuss whether screening for DPD deficiency is warranted to identify patients at risk for severe toxicities from 5-FU treatment.- - - - - - - - - - ranking = 2keywords = disc (Clic here for more details about this article) |
5/281. iliac artery pseudoaneurysm following renal transplantation presenting as lumbosacral plexopathy.A renal transplant patient developed chronic and progressive back and lower extremity pain followed by foot weakness. The correct diagnosis of lumbosacral plexopathy was made after electromyography and nerve conduction studies and the etiology of radiculopathy due to nerve root compression was excluded. This prompted further investigations that led to the discovery of a large internal iliac artery pseudoaneurysm. We emphasize the use of electrodiagnostic studies to investigate patients with back and limb pain for correctly localizing responsible pathology. In this case a potentially lethal situation was correctly identified in a transplant patient.- - - - - - - - - - ranking = 1keywords = disc (Clic here for more details about this article) |
6/281. Abnormal liver function tests following inadvertent inhalation of volatile hydrocarbons.The use of aerosols containing volatile hydrocarbons in conditions of poor ventilation can result in accidental overexposure which can cause central nervous system effects and hepatic injury. We present a case in which inadvertent usage of an adhesive spray used to make greeting cards resulted in vague neurological symptoms and abnormal liver function tests both of which fully resolved on discontinuation.- - - - - - - - - - ranking = 1keywords = disc (Clic here for more details about this article) |
7/281. Antiphospholipid antibody syndrome in a patient with neurosarcoidosis.The case of a 56-year-old woman who developed neurosarcoidosis and was discovered to have inferior vena cava and lower extremity thromboses is described. She was found to have anticardiolipin antibodies. This newly described association of antiphospholipid antibody syndrome with sarcoidosis is discussed and the relevant literature reviewed.- - - - - - - - - - ranking = 2keywords = disc (Clic here for more details about this article) |
8/281. Neurologic compromise after an isolated laminar fracture of the cervical spine.STUDY DESIGN: Report of a rare fracture of the cervical spine. OBJECTIVES: To illustrate the importance of the cervical spinolaminar line in the diagnosis of this unusual injury and to comment on appropriate investigations, management, and outcome. SUMMARY OF BACKGROUND DATA: Laminar fractures of the cervical spine are uncommon and are often missed. They usually occur after a hyperextension injury. It is unusual for these injuries to cause neurologic compromise. The injury reported here differs in that it was a result of direct trauma to the posterior aspect of the neck, and there was a significant neurologic deficit. methods: The clinical findings, roentgenographic appearance, treatment, complications, and follow-up assessment are presented and discussed. RESULTS: Initial neurologic examination revealed a right hemiparesis. Radiographs showed disruption of the spinolaminar line at C5 and a computed tomography scan revealed a fracture of the lamina of C5 with spinal canal encroachment. Management included high-dose corticosteroid administration and a posterior spinal decompression. The patient's initial postoperative course was complicated by acute pulmonary edema, which responded well to intravenous furosemide and ventilation. Follow-up assessment showed significant neurologic improvement. CONCLUSIONS: The satisfactory outcome in the case of this rare injury was the result of a prompt, accurate diagnosis and appropriate management.- - - - - - - - - - ranking = 1keywords = disc (Clic here for more details about this article) |
9/281. Civilian gunshot wounds to the head with brain stem localization. A case report.The authors present a case of a patient wounded to the head and back by civilian firearm projectiles. The case peculiarity is that only one bullet reached the brain stem level causing significant neurological deficits. The final clinical picture is comparable to the "caudal pontine tegmentum syndrome". The authors describe both the bullet path and the intracranial localization taking into account ballistic details. The problems associated with prognosis, diagnosis, and treatment for gunshot wounds are discussed. In addition, the authors explain the main intracranial lesions and their mechanisms, the role of investigation, and the protocol of medical and surgical treatment. Lastly, a systematic approach for treating these types of gunshot wounds is outlined.- - - - - - - - - - ranking = 1keywords = disc (Clic here for more details about this article) |
10/281. Delayed focal neurological deficit secondary to a cervico-thoracic spinal cord epidural haematoma.This is a report of an elderly woman who developed focal neurological deficit in association with a cervico-thoracic spinal epidural haematoma. Symptoms developed several days after the initial injury and subsequently resolved without surgical intervention. The unusual features of this presentation are discussed.- - - - - - - - - - ranking = 1keywords = disc (Clic here for more details about this article) |
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