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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Postmortem examination of 21 patients showed a vacuolar myelopathy resembling that associated with the acquired immunodeficiency syndrome. Underlying diseases included six cases of leukemia or lymphoma, five of carcinoma, three of systemic lupus erythematosus, two of chronic lung disease, and one each of cadaveric renal transplant, cirrhosis, diabetes, hemophagocytic syndrome, and viral encephalitis. Fourteen patients were on long-term steroid therapy and 10 of these also had immunosuppressive chemotherapy. No patient had the acquired immunodeficiency syndrome, although one received blood transfusions in 1978. Signs and symptoms consistent with myelopathy included paraparesis in seven patients, ataxia in one, and bilateral extensor plantar reflexes in one. Microscopic examination showed vacuolation in spinal cord white matter primarily located in posterior and lateral columns. Lipid-laden macrophages and axonal changes were proportional to the severity of the vacuolation, which was severe in five patients, moderate in 10, and mild in six. Eight patients had coexistent viral diseases elsewhere in the central nervous system, but viral-associated antigens or genomic material was not found in regions of vacuolated spinal cord white matter. Although the etiology of these myelopathies is unknown, their association with immune suppression and coexistent viral infection of the central nervous system suggests that an opportunistic viral infection may be important.
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PMID:Idiopathic myelopathies with white matter vacuolation in non-acquired immunodeficiency syndrome patients. 186 65

Portal-systemic shunts, either spontaneous or artificial, are occasionally complicated by the development of spastic paraparesis. We report on 2 young patients who developed this complication following splenorenal shunts which were made for the treatment of oesophageal varices associated with cirrhosis of the liver and portal hypertension.
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PMID:Shunt myelopathy. A report of 2 cases. 710 Oct 74

From January 1991 to May 1994, we have operated on 15 cases of Type B aortic dissection. In 10 of these patients, thoracoabdominal repair was performed. According to Crawford's classification, 2 patients fell into Type I, 6 patients into Type II, and 2 patients into Type III. The aneurysms were exposed through a left thoracotomy extending into the retroperitoneum with the hemidiaphragm divided circumferentially. The operations were performed under femoro-femoral partial cardiopulmonary bypass. In 6 of these cases selective perfusion of the visceral branches was used. The celiac axis was reconstructed in 10 patients, superior mesenteric artery in 9, right renal artery in 7, left renal artery in 6. Abdominal vessels were reconstructed by the "inclusion" technique described by Crawford in 2 patients, by "beveling" the distal prosthetic end in 6 and by the "interposition" technique in 4 patients. Vessels arising from the false lumen were reconstructed by the "interposition" technique. To prevent paraplegia, the evoked spinal cord potentials by direct stimulation of the cord (ESPs-dsc) were monitored perioperatively and the aneurysms were repaired sequentially in segments. In all patients except 2 with Crawford type III aneurysms, spinal cord ischemia was detected by ESPs-dsc. In 7 of these patients, 2 to 8 pairs of intercostal/lumbar arteries (I/L aa.) that arose from the "responsible" aortic segment were reconstructed. Reconstruction techniques included the "inclusion" technique in 2 patients, the "beveling" technique in 1, the "interposition" technique in 1 and the "on lay grafting" technique in 3 patients. One hospital death occurred in a patient who had chronic renal insufficiency and liver cirrhosis preoperatively. Spinal cord injury occurred in 5 patients, including 4 paraparesis and 1 delayed-onset paraplegia. In 2 of these patients, responsible I/L aa., were not reconstructed correctly despite ESPs changes, and injury might have been prevented if reconstruction of the "responsible" arteries had been performed. Thoracoabdominal repair for chronic Type B aortic dissection could be performed safely with an acceptable mortality rate. Spinal cord injury remains an unsolved problem.
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PMID:Operative results of thoracoabdominal repair for chronic type B aortic dissection. 920 Nov 25

The authors attempted to describe the clinical manifestations of portal-systemic myelopathy (PSM) after transjugular intrahepatic portosystemic shunt (TIPS) creation. PSM was developed in four of 212 (1.89%) patients who underwent TIPS procedures in our hospital. Three men and one woman, ranging in age from 41 to 56 years, with a history of posthepatitis cirrhosis and recurrent bleeding from gastroesophageal varices had intrahepatic shunts created with 10-mm-diameter Wallstents. Shunt patency was confirmed by color Doppler ultrasonography (US) in each patient after TIPS creation. Progressive spastic paraparesis involving the lower extremities occurred between 5 weeks and 5 months after TIPS creation in the four patients. Neurologic examination showed evidence of spasticity in all cases, with ankle clonus, extensor plantar responses, and lower extremity hyperreflexia. All sensory modalities remained intact. Cytologic examination of cerebrospinal fluid from each patient was normal. There was no evidence of spinal cord compression on the imaging studies. PSM is a rare syndrome that includes spastic paraparesis with intact sensation. Initially noted in patients who have undergone surgical placement of a portacaval shunt, it also may occur after TIPS creation.
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PMID:Portal-systemic myelopathy after transjugular intrahepatic portosystemic shunt creation: report of four cases. 1143 45

Hepatic myelopathy is a rare complication of cirrhosis, usually associated with surgical or spontaneous porto-systemic shunts. Its pathophysiology is unknown. It is characterized by a motor involvement of the lower limbs without clinical sensory abnormality, leading to spastic paraparesis. These neurological features are related to a symmetric loss of myelin in the lateral corticospinal tracts. Usefulness of liver transplantation in this setting is not yet determined. We describe here the case of a 29-year-old male who presented with progressive spastic paraparesis of the lower limbs 3 years after a spleno-renal shunt.
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PMID:Hepatic myelopathy after splenorenal shunting: report of one case and review of the literature. 1147 43

Hepatic myelopathy (HM) is a rare complication of chronic liver diseases usually associated with a portosystemic shunt, causing a progressive spastic paraparesis, and is likely to be overlooked. Thirteen patients with liver cirrhosis associated with surgical or spontaneous portosystemic shunts were studied to determine the frequency and gravity of HM. Six patients exhibited clear-cut signs of spinal cord involvement and four of them exhibited varying degrees of disability. Neurological examination did not reveal any abnormalities in the other patients. Motor evoked potentials (MEPs) were measured in all patients; in five of them the examinations were done before and after orthotopic liver transplantation (OLT). The patients with clinical signs of spinal cord involvement exhibited severe neurophysiological abnormalities, whereas milder but unequivocal MEP abnormalities were found in four of the seven patients with normal clinical examination. The clinical and neurophysiological features of patients with slight MEP abnormalities improved after OLT, whereas the patients with a more advanced stage of disease (severe MEPs abnormalities) did not. Our findings indicate that MEP studies may disclose an impairment of the corticospinal pathways even before HM is clinically manifest and provide evidence that early diagnosis of HM and subsequent immediate liver transplantation have to be recommended.
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PMID:Corticospinal involvement in patients with a portosystemic shunt due to liver cirrhosis: a MEP study. 1604 11

The clinical presentation of acute liver failure and hepatic encephalopathy (HE) in patients with cirrhosis differs significantly. The most serious neurological complication of acute liver failure is the development of devastating brain oedema. Therefore, intracranial pressure monitoring is urgently needed in these patients. Brain oedema is amplified by hypoglycemia, hypoxia and seizures, which are also frequent complications of acute liver failure. Therefore, these parameters must also be monitored. In contrast to acute liver failure in which cerebral dysfunction progresses rapidly, cognitive decline may be clinically undetectable for a long time in cirrhotic patients, until clinically overt symptoms such as psychomotor slowing, disorientation, confusion, extrapyramidal and cerebellar symptoms or a decrease in consciousness occur. Clinically, overt HE is preceded by minimal alterations of cerebral function that can only be detected by neuropsychological or neurophysiological measures, but which nevertheless interfere with the patient's daily living. Rapidly progressing spastic paraparesis (hepatic myelopathy) is a rare complication of cirrhosis. In contrast to HE, it does not respond to blood ammonia lowering therapies but must be considered as an indication for urgent liver transplantation. Cognitive dysfunction has recently been detected in hepatitis C virus (HCV)-infected patients with normal liver function. The patients presented with severe fatigue, cognitive dysfunction and mood disorders. Alterations in brain metabolites, as detected by magnetic resonance spectroscopy, indicated central nervous system alteration in these patients. In contrast to patients with HE, HCV-infected patients did not show motor symptoms or deficits in visual perception, but considerable deficits in attention and concentration ability.
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PMID:Neurological and neuropsychiatric syndromes associated with liver disease. 1625 35

Portosystemic encephalopathy (PSE) is a well-known, common complication of portal hypertension. It is thought to be caused by nitrogenous substances such as ammonia, which are normally cleared from the blood stream by the liver. In cirrhosis and other hepatic disorders with portosystemic shunting (PSS)-- either surgical portosystemic anastomoses (PSA) or spontaneous PSS-- the collateral vessels bypass the liver allowing the accumulation of toxic, ammoniacal substances in the blood and tissues. PSE is characterized by encephalopathy; portosystemic myelopathy (PSM) is characterized by paresis of the extremities, Babinski signs and muscle spasticity in patients with cirrhosis and/or PSS. Usually only the lower extremities are involved. This report presents the first case of this syndrome observed 5 years after a transjugular intrahepatic portosystemic shunt. The 31 year old man with chronic Hepatitis B developed complete spastic paraparesis within 4 weeks after onset of clinical/neurological symptoms, accompanied by an episode of severe hepatic encephalopathy. The transcortical magnetic stimulation showed normal motoric stimulation times to the abductor digiti minimi muscles but no stimulation to the tibialis muscles was seen. Lumbar stimulation to the tibialis muscles, however, was normal. This indicates loss of motor neurons in the spinal cord, a characteristic finding in patients with portosystemic myelopathy. We performed a search of the literature for all reported cases of cirrhosis and/or PSS that developed PSM. However, the intervals between the construction of a shunt and the diagnosis of portosystemic myelopathy were shorter in total portacaval shunts (median 16 months) than in partial, non-portacaval shunts (median 60 months, p < 0.01). This suggests that not only the shunt itself but also the shunted volume contributes to the development of the syndrome Sixty-one patients with PSM have been reported in the literature since 1944. PSE had developed before PSM in almost all cases. PSM occurred from 1 month to 10 years after the creation of portacaval anastomoses (PCA) or splenorenal shunts (SRS) or in cirrhotic patients without shunts. No one type of liver disease or type of shunt appears to predispose to PSM. The mechanisms of PSE and PSM are thought to be similar and of nitrogenous origin, but their pathogenesis remains unknown. Lathyrism, a toxic syndrome with similar symptoms and signs, is caused by the ingestion of a legume, Lathyrus sativa, which contains beta-N-oxalo-L amino-L-alanine (BOAA). This animal model with or without BOAA appears to offer a reliable way of studying PSM experimentally.
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PMID:Portosystemic myelopathy: spastic paraparesis after portosystemic shunting. 1663 7

A 19-year-old gentleman presented with slowly progressive spastic paraparesis, 2 years after the therapeutic lienorenal shunt for portal hypertension secondary to cirrhosis and portal vein occlusion. After 2 years of initial evaluation, the motor functions had not worsened further. He did not have any obvious clinical or EEG features of hepatic encephalopathy. Other causes for myelopathy were ruled out. Contribution of portal vein occlusion to portosystemic shunting has not been reported previously in patients with 'hepatic myelopathy.' This uncommon complication needs to be considered in patients with shunt surgery for relieving portal hypertension.
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PMID:Hepatic myelopathy: a rare complication following extrahepatic portal vein occlusion and lienorenal shunt. 1693 95

Hepatic myelopathy (HM) is a rarely reported disorder characterized by progressive spastic paraparesis due to impaired corticospinal tract function in the setting of cirrhosis or portosystemic shunting. HM has not to date been recognized as a Model for End-Stage Liver Disease (MELD) exception for transplantation. Outcomes for a small number of patients from Europe and Asia who have undergone liver transplantation (LT) for HM suggest a potential neurological benefit, especially with earlier transplantation. We report the first use of MELD exception points for the condition of HM to enable early LT resulting in the reversal of marked spastic paraparesis. Our patient, whose myelopathy had markedly progressed without further hepatic decompensation, underwent LT 14 months after the diagnosis of HM with an adjusted MELD score of 30, which was granted as a United Network for Organ Sharing exception. After LT, there was significant neurological improvement as the patient progressed from wheelchair dependency to full ambulation. We reviewed the literature of other HM patients who had undergone LT. With our patient, there were in all 15 reported cases of LT in individuals with HM. LT can lead to a marked improvement in HM, particularly in the earlier clinical stages of the disorder. Early LT can be accomplished, as in our case, by the submission of an appeal for a MELD upgrade.
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PMID:Use of model for end-stage liver disease exception points for early liver transplantation and successful reversal of hepatic myelopathy with a review of the literature. 2103 52


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