Biomedical subjects
Publications and source records attributed to J Kulisevsky.
Myelopathy is a rare complication of chronic liver disease associated with extensive portal-systemic shunt of blood. Its main clinical feature is a spastic paraparesis without sensory or sphincteric impairment. We describe 3 patients who presented hepatic myelopathy following the performance of a portacaval shunt.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
In cirrhotic patients, even in a stable nonencephalopathic state, MRI may show cerebral atrophy and increased signal in globus pallidus on T1-weighted sequences. We investigated the relationship between cerebral atrophy and increased pallidal signal and the clinical status of 30 cirrhotic patients. We found a weak association between the two MRI findings. There were different patterns of clinical variables related to the imaging findings. Performance on motor tasks involving speed correlated with the pallidal signal and plasma ammonia levels but not with atrophy. Test results for memory and frontal-premotor function were associated with brain atrophy but not with the pallidal signal or with ammonia.
An 81-year-old woman had the sudden onset of left-sided ballismus and an accompanying behavioral change characterized by elation, distractibility, inflated self-esteem, and insomnia, suggesting secondary mania. An MRI revealed a small ischemic infarction of the right thalamus.
Patients with cirrhosis show increased signal intensity in the globus pallidus on T1-weighted magnetic resonance imaging of the brain. This abnormal appearance of the basal ganglia has been related to the severity of liver failure and to the presence of portal-systemic shunting, although its cause and clinical significance remain unknown. We prospectively assessed the metabolic, neurological and neuropsychological statuses of 30 stable cirrhotic patients and correlated these clinical variables with computed measurements of globus pallidus signals. Some metabolic variables denoting disease severity appeared to be significantly related to image changes, although the strongest association was found with plasma ammonia levels. After adjustment for ammonia level, on multiple regression analysis, the other variables were not significant. Furthermore, pallidal changes were associated with specific neurological symptoms and neurological functions, symptoms and functions that also had a significant correlation with ammonia levels. Our findings suggest that globus pallidus signal abnormality could arise as a marker of brain impairment related to hyperammonemia.
Explore the source record for details and available documents.
Two patients are presented with muscle spasms in an amputation stump. Neither patient experienced neuropathic pain nor phantom sensations, though phantom sensory phenomena, severe pain, and lack of response to treatment is characteristic of reported cases. One patient, a 75 year old man, has had myoclonic activity of the stump for more than two years, and the other, a 79 year old woman, recovered spontaneously after three months and is symptom free after a one year follow up. We emphasise the lack of association with pain and the need to consider spontaneous improvement when therapy is evaluated.
Primary Meige syndrome is a form of cranial dystonia of unknown cause. Only three postmortem studies have been reported, and the results of these studies have not been consistent. We have examined the brain of a 72-year-old man with typical primary Meige syndrome and found mild to moderate cell loss in the zona compacta of the substantia nigra, locus ceruleus, midbrain tectum, and dentate nucleus of the cerebellum. Also frequent Lewy bodies were present in pigmented nuclei of the brainstem. No abnormalities were detected elsewhere. These pathological findings support the notion that brainstem pathology is important in the pathophysiology of cranial dystonia.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
UNLABELLED: Dementia with Lewy bodies (DLB) is the second most common cause of degenerative dementia after Alzheimer's disease (AD). At present, pre-mortem diagnosis of DLB can only be made clinically using the International Consensus Criteria. However, an accurate differential diagnosis between these diseases could improve the therapeutic handling of patients with DLB, due to their supersensitivity to neuroleptic treatment and the difficult treatment of their psychotic symptoms. OBJECTIVE: To assess the utility of cardiac MIBG imaging as diagnostic study for DLB, to help in the differential diagnosis with AD. MATERIAL AND METHODS: Cardiac MIBG imaging was performed in 11 patients with clinical criteria of probable DLB (7 males, mean age 77 years [range 62-89 years], mean MMSE 17 [range 11-28], and in 9 patients with clinical criteria of probable AD (3 males, mean age 79 years [range 61-87 years], mean MMSE 17 [range 4-25]). Planar anterior images of the thorax were acquired at 15 minutes. (early study) and 4 hours (late study) after tracer injection. Myocardial MIBG activity was quantified by means of a heart-to-mediastinum ratio (HMR). A HMR > 1.8 was considered normal. RESULTS: Respect AD patients, patients with DLB showed decreased HMR in the early study (1.34 +/- 0.27 [range 1.03-1.98] vs. 1.84 +/- 0.22 [range 1.53-2.15], p<0.001) and in the late study (1.22 +/- 0.23 [range 0.95-1.75] vs. 1.73 +/- 0.08 [range 1.59-1.89], p<0.0001). CONCLUSIONS: Cardiac MIBG imaging could be a useful tool for differential diagnosis between DLB and AD.
Involuntary movements are unusual during or after acute stroke, and alien hand sign has not been reported after single parietal infarction. We report on a woman who presented with involuntary movements and a sense of alienness of her left upper limb following a right parietal infarction. This combination, that can meet the criteria for the 'alien hand syndrome', is rarely seen after acute lesions sparing the corpus callosum and the mesial frontal areas.
Entacapone (Comtan) is a potent, selective inhibitor of peripheral catechol-O-methyltransferase (COMT) with therapeutic potential as an adjuvant to levodopa therapy in patients with Parkinson's disease. Entacapone decreases peripheral conversion of levodopa to 3-O-methyldopa increasing central extracellular levodopa and consequently striatal dopamine concentrations. At doses of 200 mg 2 to 10 times daily coadministered with levodopa/carbidopa or levodopa/benserazide entacapone may increase the duration of clinical response both after the first single dose and after repeated dosing in patients with end-of-dose fluctuations. At this dosage, it has a time to peak-plasma concentration of 1.2 hours and an elimination half life of 3.4 hours. In two multicentric, long-term (approximately 6 month), randomized and placebo-controlled studies, the duration of 'on' time was increased and the duration of 'off time' was decreased in patients who received adjunctive entacapone therapy. Moreover, patients randomized to entacapone reduced their levodopa requirements. In these and other phase III studies, entacapone was generally well tolerated, with few reported adverse events, mainly dyskinesias and gastrointestinal disorders. The dyskinesias were generally well controlled by decreasing the mean daily levodopa dose. Entacapone appears as a clinically significant and beneficial adjunct to levodopa therapy in Parkinson's disease patients with end-of-dose fluctuations.
Motor fluctuations are a common problem in the long-term treatment of Parkinson's disease (PD). Entacapone (Comtan) is a potent, peripherally acting, reversible and selective inhibitor of catechol-O-methyltransferase (COMT). Used as an adjuvant to levodopa therapy, entacapone slows the elimination of levodopa by decreasing peripheral conversion to 3-O-methyldopa, increasing central extracellular levodopa and striatal dopamine concentrations. Coadministered with levodopa/carbidopa or levodopa/benserazide, at doses of 200 mg 2 to 10 times daily in patients with end-of-dose fluctuations, entacapone may increase the duration of clinical response, both after the first single dose and after repeated dosing. At this dosage, it has a time to peak plasma concentration of 1.2 h and an elimination half life of 3.4 h. In two multicentric, long-term (24 weeks), parallel, randomized and placebo-controlled studies, entacapone increased the duration of 'on' time (by approximately 1 hour daily) and decreased the duration of 'off' time with a concomitant reduction in the mean daily levodopa dose. In these and other phase III studies, entacapone was generally well tolerated, with most adverse effects being dyskinesias and gastrointestinal disorders. Increased dyskinesia were generally controlled by reducing levodopa doses. Entacapone appears to be a useful adjunct in extending the benefit of each levodopa dose in PD patients with end-of-dose fluctuations.