Hepatic myelopathy (HM) is a rare complication of chronic liver diseases usually associated with a portosystemic shunt, causing a progressive spastic parapare sis, and is likely to be overlooked. Thirteen patients wit...Hepatic myelopathy (HM) is a rare complication of chronic liver diseases usually associated with a portosystemic shunt, causing a progressive spastic parapare sis, and is likely to be overlooked. Thirteen patients with liver cir-rhosis associated with surgical or spontaneous portosystemic shunts were studied to determine the frequency and gravity of HM. Six patients exhibited clearcut 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.展开更多
Objective: Nicotinic acetylcholine receptors have close interactions with the dopaminergic system and play critical roles in cognitive function. The purpose of this study was to compare these receptors between living ...Objective: Nicotinic acetylcholine receptors have close interactions with the dopaminergic system and play critical roles in cognitive function. The purpose of this study was to compare these receptors between living PD patients and healthy subjects. Methods: Nicotinic acetylcholine receptors were imaged in 10 nondemented Parkinson’s disease patients and 15 age-matched healthy subjects using a single-photon emission computed tomography ligand [123I] 5- iodo- 3- [2(S)- 2- azetidinylmethoxy]pyridine. Using an arterial input function, we measured the total distribution volume (V; specific plus nondisplaceable), as well as the delivery (K1). Results: Parkinson’s disease showed a widespread significant decrease (approximately 10% ) of V in both cortical and subcortical regions without a significant change in K1. Interpretation: These results indicate the importance of extending the study to demented patients.展开更多
A patient presented with a painful, pulsating nodosity with marked tenderness on the right side of her neck. Cervical magnetic resonance imaging (MRI) exhibited pathological signal changes in the perivascular tissue o...A patient presented with a painful, pulsating nodosity with marked tenderness on the right side of her neck. Cervical magnetic resonance imaging (MRI) exhibited pathological signal changes in the perivascular tissue of the carotid bifurcation area and around the external carotid artery. Similarly, Doppler sonography revealed an echo-poor wall change with outward bulging and slight narrowing of the affected vessel lumen. The clinical symptoms and our findings led to the diagnosis of carotidynia. We proposed that cervical MRI and Doppler sonography could be used for diagnosing carotidynia.展开更多
Objective: To assess the linguistic abilities of a boy having Landau-Kleffner Syndrome, and relate the focal disturbance of brain activity due to epilepsy to the cognitive and linguistic deficits. Methods: Several kin...Objective: To assess the linguistic abilities of a boy having Landau-Kleffner Syndrome, and relate the focal disturbance of brain activity due to epilepsy to the cognitive and linguistic deficits. Methods: Several kinds of assessments were carried out, including epileptic source analysis using electronic source localization methods and PET, neuropsychological assessment of cognitive functions, and assessment of speech perception skills (discrimination of phonetic and stress cues) using ERPs. Results: The source of epileptic activity was localized in the left superior temporal lobe. The neuropsychological assessment showed dissociation between verbal and nonverbal functions, and the performance in former was bellow the normal range. ERPs obtained to the processing of phonetic and stress speech cues indicated that the two cues were processed asymmetrically: the mismatch negativity component (MMN) was obtained for the phoneme difference, but not for the stress pattern difference. Conclusions: Our data converged as it showed that the patient presented a selective impairment of the language system, and the verbal working memory system appeared to be especially defective. It is suggested that the language deficit is at least partly due to the focal disturbance of those neural networks that underlie the functioning of the working memory system. Significance: LKS is a childhood language disorder that might serve as a model in studying what happens to the language system if, in the course of developme nt, the essential neural circuits are severely disturbed.展开更多
The authors describe the clinical and diagnostic characteristics of four patients with metastatic CNS disease presenting in de novo nonconvulsive status epilepticus (NCSE). Treatment with anticonvulsants resulted in r...The authors describe the clinical and diagnostic characteristics of four patients with metastatic CNS disease presenting in de novo nonconvulsive status epilepticus (NCSE). Treatment with anticonvulsants resulted in resolution of NCSE in two patients and a brief improvement in mental status in the other two patients. NCSE should be considered in the differential diagnosis of acute mental status change in patients with metastatic CNS disease.展开更多
Objective: To investigate the hypothesis that risk factors may be differently related to severity of acute coronary syndromes(ACS), with ST elevation used as a marker of severe ACS. Design: Cross sectional study of pa...Objective: To investigate the hypothesis that risk factors may be differently related to severity of acute coronary syndromes(ACS), with ST elevation used as a marker of severe ACS. Design: Cross sectional study of patients with ACS. Setting: 103 hospitals in 25 countries in Europe and the Mediterranean basin. Patients: 10 253 patients with a discharge diagnosis of ACS in the Euro heart survey of ACS.Main outcome measures: Presenting with ST elevation ACS. Results: Patients with ACS who were smokers had an increased risk to present with ST elevation(age adjusted odds ratio(OR) 1.84, 95%confidence interval(CI) 1.67 to 2.02). Hypertension(OR 0.65, 95%CI 0.60 to 0.70) and high body mass index(BMI)(p for trend 0.0005) were associated with less ST elevation ACS. Diabetes mellitus was also associated with less ST elevation, but only among men. Prior disease(infarction, chronic angina, revascularisation) and treatment with aspirin, βblockers, or statins before admission were also associated with less ST elevation. After adjustment for age, sex, prior disease, and prior medication, smoking was still significantly associated with increased risk of ST elevation(OR 1.53, 95%CI 1.38 to 1.69), whereas hypertension was associated with reduced risk(OR 0.75, 95%CI 0.69 to 0.82). Obesity(BMI>30 kg/m2 versus< 25 kg/m2)was independently associated with less risk of presenting with ST elevation among women, but not among men. Conclusion: Among patients with ACS, presenting with ST elevation is strongly associated with smoking, whereas hypertension and high BMI(in women) are associated with less ST elevation, independently of prior disease and medication.展开更多
文摘Hepatic myelopathy (HM) is a rare complication of chronic liver diseases usually associated with a portosystemic shunt, causing a progressive spastic parapare sis, and is likely to be overlooked. Thirteen patients with liver cir-rhosis associated with surgical or spontaneous portosystemic shunts were studied to determine the frequency and gravity of HM. Six patients exhibited clearcut 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.
文摘Objective: Nicotinic acetylcholine receptors have close interactions with the dopaminergic system and play critical roles in cognitive function. The purpose of this study was to compare these receptors between living PD patients and healthy subjects. Methods: Nicotinic acetylcholine receptors were imaged in 10 nondemented Parkinson’s disease patients and 15 age-matched healthy subjects using a single-photon emission computed tomography ligand [123I] 5- iodo- 3- [2(S)- 2- azetidinylmethoxy]pyridine. Using an arterial input function, we measured the total distribution volume (V; specific plus nondisplaceable), as well as the delivery (K1). Results: Parkinson’s disease showed a widespread significant decrease (approximately 10% ) of V in both cortical and subcortical regions without a significant change in K1. Interpretation: These results indicate the importance of extending the study to demented patients.
文摘A patient presented with a painful, pulsating nodosity with marked tenderness on the right side of her neck. Cervical magnetic resonance imaging (MRI) exhibited pathological signal changes in the perivascular tissue of the carotid bifurcation area and around the external carotid artery. Similarly, Doppler sonography revealed an echo-poor wall change with outward bulging and slight narrowing of the affected vessel lumen. The clinical symptoms and our findings led to the diagnosis of carotidynia. We proposed that cervical MRI and Doppler sonography could be used for diagnosing carotidynia.
文摘Objective: To assess the linguistic abilities of a boy having Landau-Kleffner Syndrome, and relate the focal disturbance of brain activity due to epilepsy to the cognitive and linguistic deficits. Methods: Several kinds of assessments were carried out, including epileptic source analysis using electronic source localization methods and PET, neuropsychological assessment of cognitive functions, and assessment of speech perception skills (discrimination of phonetic and stress cues) using ERPs. Results: The source of epileptic activity was localized in the left superior temporal lobe. The neuropsychological assessment showed dissociation between verbal and nonverbal functions, and the performance in former was bellow the normal range. ERPs obtained to the processing of phonetic and stress speech cues indicated that the two cues were processed asymmetrically: the mismatch negativity component (MMN) was obtained for the phoneme difference, but not for the stress pattern difference. Conclusions: Our data converged as it showed that the patient presented a selective impairment of the language system, and the verbal working memory system appeared to be especially defective. It is suggested that the language deficit is at least partly due to the focal disturbance of those neural networks that underlie the functioning of the working memory system. Significance: LKS is a childhood language disorder that might serve as a model in studying what happens to the language system if, in the course of developme nt, the essential neural circuits are severely disturbed.
文摘The authors describe the clinical and diagnostic characteristics of four patients with metastatic CNS disease presenting in de novo nonconvulsive status epilepticus (NCSE). Treatment with anticonvulsants resulted in resolution of NCSE in two patients and a brief improvement in mental status in the other two patients. NCSE should be considered in the differential diagnosis of acute mental status change in patients with metastatic CNS disease.
文摘Objective: To investigate the hypothesis that risk factors may be differently related to severity of acute coronary syndromes(ACS), with ST elevation used as a marker of severe ACS. Design: Cross sectional study of patients with ACS. Setting: 103 hospitals in 25 countries in Europe and the Mediterranean basin. Patients: 10 253 patients with a discharge diagnosis of ACS in the Euro heart survey of ACS.Main outcome measures: Presenting with ST elevation ACS. Results: Patients with ACS who were smokers had an increased risk to present with ST elevation(age adjusted odds ratio(OR) 1.84, 95%confidence interval(CI) 1.67 to 2.02). Hypertension(OR 0.65, 95%CI 0.60 to 0.70) and high body mass index(BMI)(p for trend 0.0005) were associated with less ST elevation ACS. Diabetes mellitus was also associated with less ST elevation, but only among men. Prior disease(infarction, chronic angina, revascularisation) and treatment with aspirin, βblockers, or statins before admission were also associated with less ST elevation. After adjustment for age, sex, prior disease, and prior medication, smoking was still significantly associated with increased risk of ST elevation(OR 1.53, 95%CI 1.38 to 1.69), whereas hypertension was associated with reduced risk(OR 0.75, 95%CI 0.69 to 0.82). Obesity(BMI>30 kg/m2 versus< 25 kg/m2)was independently associated with less risk of presenting with ST elevation among women, but not among men. Conclusion: Among patients with ACS, presenting with ST elevation is strongly associated with smoking, whereas hypertension and high BMI(in women) are associated with less ST elevation, independently of prior disease and medication.
