Search PubMed⌕ Search

Biomedical subjects

I Steiner

Publications and source records attributed to I Steiner.

At least 19 recordsLinked to original sources
PubMed2006

Abnormal musical pacemaker in a patient with musical hallucinations.

Music creation requires a highly orchestrated temporal pattern. The study of a patient with repetitive musical hallucinations enabled us to examine temporal pacemakers in music production. Here, we show that the pattern of faster silent production of a chosen tune compared with its production aloud was reversed when the patient produced the hallucinatory tune. This observation might suggest the utilization of a pacemaker(s), which functions differentially during the disease.

Adult↗
PubMed2005

Frontotemporal dementia with ubiquitinated neuronal inclusions and visuospatial impairment.

Dementia with ubiquitinated neuronal inclusions has been described only with frontotemporal dementia (FTD). The authors report a patient with progressive FTD accompanied by prominent impairments in visuospatial cognitive functions. Pathology was characterized by ubiquitin-positive intranuclear and cytoplasmic neuronal inclusions. Cortical pathology was widespread and posteriorly accentuated but spared the hippocampal dentate gyrus.

Agnosia↗
PubMed2005

Can a herpes simplex virus type 1 neuroinvasive score be correlated to other risk factors in Alzheimer's disease?

Herpes simplex virus type 1 (HSV-1) is latent in the nervous system of most humans. Ball [Can J Neurol Sci 9 (1982) 303] first suggested the hypothesis that HSV-1 could be involved in the pathogenesis of Alzheimer's Disease (AD) by noting that regions of the brain particularly and earliest affected in AD were the same as those most damaged during HSV encephalitis. Data from Itzhaki's research suggests that HSV-1 in the brain and the carriage of an apolipoprotein E allele 4 (ApoE e4) together confer risk for AD [J Pathol 97 (2002) 395], [Mol Chem Neuropathol 28 (1996) 135], [Alzheimer's Rep 1 (1998) 173], [Biochem Soc Trans 26 (1998) 273]. Of the two other studies based on Itzhaki's findings, one showed similar results [Lancet 349 (1997) 1102], and the other showed a similar trend [Lancet 351 (1998) 1330], [Lancet 352 (1998) 1312]. To further examine the role of HSV-1 in the etiology of AD, we have formulated a Neuroinvasive Score that quantifies the presence and viral load of HSV-1 in eight brain regions. These regions are: entorhinal cortex, hippocampus, pons, cerebellum, and neocortex (temporal, parietal, occipital, and frontal). We hypothesize that the Neuroinvasive Score that encompasses the presence, amount, and extent of HSV-1 spreading (neuroinvasiveness), will correlate with the genetic risk factor, ApoE e4, in the assessment of autopsy samples from AD patients. If the neuroinvasive score can be directly correlated to the different stages of AD (mild, moderate, severe), this will strengthen the hypothesis that HSV-1 is involved in AD and that ApoE e4 also confers risk for the development and progression of AD.

Alzheimer Disease↗
PubMed2005

Viral encephalitis: a review of diagnostic methods and guidelines for management.

Viral encephalitis is a medical emergency. The spectrum of brain involvement and the prognosis are dependent mainly on the specific pathogen and the immunological state of the host. Although specific therapy is limited to only several viral agents, correct immediate diagnosis and introduction of symptomatic and specific therapy has a dramatic influence upon survival and reduces the extent of permanent brain injury in survivors. We searched MEDLINE (National Library of Medicine) for relevant literature from 1966 to May 2004. Review articles and book chapters were also included. Recommendations are based on this literature based on our judgment of the relevance of the references to the subject. Recommendations were reached by consensus. Where there was lack of evidence but consensus was clear we have stated our opinion as good practice points. Diagnosis should be based on medical history, examination followed by analysis of cerebrospinal fluid for protein and glucose contents, cellular analysis and identification of the pathogen by polymerase chain reaction (PCR) amplification (recommendation level A) and serology (recommendation level B). Neuroimaging, preferably by magnetic resonance imaging, is an essential aspect of evaluation (recommendation level B). Lumbar puncture can follow neuroimaging when immediately available, but if this cannot be obtained at the shortest span of time it should be delayed only in the presence of strict contraindications. Brain biopsy should be reserved only for unusual and diagnostically difficult cases. All encephalitis cases must be hospitalized with an access to intensive care units. Supportive therapy is an important basis of management. Specific, evidence-based, anti-viral therapy, acyclovir, is available for herpes encephalitis (recommendation level A). Acyclovir might also be effective for varicella-zoster virus encephalitis, gancyclovir and foscarnet for cytomegalovirus encephalitis and pleconaril for enterovirus encephalitis (IV class of evidence). Corticosteroids as an adjunct treatment for acute viral encephalitis are not generally considered to be effective and their use is controversial. Surgical decompression is indicated for impending uncal herniation or increased intracranial pressure refractory to medical management.

Central Nervous System↗
PubMed2005

[Acute aortic syndromes].

The term acute aortic syndrome comprises in addition to classic dissection also two recently described entities--penetrating ulcer and intramural haematoma. Forty-two necropsy cases of acute aortic syndrome were encountered during the last six years--41 dissections and one penetrating ulcer; the intramural haematoma was not seen. According to the DeBakey classification, there were 26 dissections of type I, 8 of type II, 1 of type IIIA, and 4 of type IIIB; in 2 cases the dissection was confined to the abdominal aorta. The dissection had a chronic character in four cases. Aortic rupture was found in 24 cases (59%), most frequently (19x) into the pericardial cavity. The aortic dissection continued into aortic branches in 25 patients (61%), causing stenosis/obstruction of coronary arteries in 7, of branches of the arch in 20, of abdominal arteries in 12, and of renal arteries in 17 patients, respectively. Histologically, there were degenerative lesions of the Erdheim type in the media of 10 aortas (from 21 completely examined). As possible risk factors for aortic dissection there appeared hypertension in 32 patients, anuloaortic ectasia in 11, saccular aneurysm ot the abdominal aorta in 6, family history of dissection in 2, Marfan syndrome in 2, prolaps of the mitral valve in 2, and bicuspid aortic valve in 2 patients, respectively. The aortic dissection was iatrogenic in 7 patients, presenting as a complication of a cardiosurgical or invasive cardiological procedure.

Acute Disease↗
PubMed2005

[A hitherto unknown priority of Jan Ev. Purkyne--myocardial sleeves of the pulmonary veins. Contribution to the pathogenesis of atrial fibrillation].

According to recent electrophysiological discoveries, atrial fibrillation is triggered by ectopic beats which in most cases originate in the pulmonary veins, in extensions of left atrial myocardium upon these veins, so called myocardial sleeves. In the relevant literature it is stated that this anatomical structure was first described by Ferdinand Raeuschel in 1836 in the publication "De arteriarum et venarum structura". This publication appeared as Raeuschel's dissertation thesis at the faculty of medicine in Breslau (now Polish Wroclaw) at the department of physiology under the leadership of Jan Ev. Purkyne. In the introduction, the author thanks his tutor for introducing him into the problematic and for the continuous help, stating that all new discoveries in the dissertation should be ascribed to Purkyne. Thus, the description of myocardial sleeves of pulmonary veins appears as a yet further priority of the great Czech scientist.

Atrial Fibrillation↗
PubMed2005

[Pulmonary veins and atrial fibrillation: a pathological study of 100 hearts].

Pathogenesis of atrial fibrillation (AF), the most common sustained heart arrhythmia, is not yet fully elucidated. Recent electrophysiological studies have shown that in most patients with AF the arrhythmia is triggered by ectopic beats originating from extensions of left atrial myocardium over the pulmonary veins (PVs), so called myocardial sleeves (MSPV). A total of 100 hearts (393 PVs) obtained at autopsy were prospectively studied - 50 from patients with chronic AF (average age 76.9 +/- 7.3 yrs.) and a control group of 50 with a sinus rhythm (aver. age 71.7 +/- 9.5 yrs.). This is a largest study published on this topic so far. It appeared that MSPV frequently harbour pathological lesions, particularly senile atrial amyloid, and scarring. These two pathological changes were evaluated semiquantitatively on a grade 0-3 basis in individual PVs, comparing the results in the AF vs. the control group. Amyloidosis of MSPV was found in 68 % of all hearts and in 55 % of all sleeves. The deposits were most marked in the right superior PV. Amyloidosis was more frequent and more severe in MSPV of patients with AF (58.5 %; average grade 0.89) than of those without AF (51.7 %; aver. grade 0.76); the differences, however, lack statistical significance. Scarring of MSPV was present in all 349 sleeves, more markedly in the left inferior, left superior, and right superior PVs. It was significantly more severe in patients with AF compared to those without the arrhythmia. By an injection metod, we have shown that MSPV are supplied by coronary arteries. However, the degree of scarring of the sleeves did not correlate with the degree of coronary atherosclerosis. We suggest that genesis of the scarring is not postnecrotic but degenerative, due to diffuse hypoxia of the sleeve myocardium. To conclude, amyloidosis and particularly scarring of MSPV appear generally in the elderly population as an arrhythmogenic substrate for AF.

Aged↗
PubMed2004

Rasmussen encephalitis with active inflammation and delayed seizures onset.

Severe focal epilepsy is regarded as a clinical hallmark of Rasmussen encephalitis (RE). The authors report two children with progressive hemiparesis, contralateral hemispheric atrophy, and pathologic features characteristic for RE. At histologic diagnosis and over several months, neither patient experienced seizures. The report enlarges the clinical spectrum of RE and suggests that seizures are not an obligatory presenting symptom of the disorder.

Age of Onset↗
PubMed2003

[Carcinoid heart disease].

Demonstrated are eight cases of carcinoid heart disease (7 females, 1 male; average age 63.4 y.). The primary tumor was localized in the ileum in 5, and in the cecal area in 3 patients, with liver metastases in each case. The characteristic heart lesion appears as an endocardial plaque composed of loose intercellular matrix with scattered smooth muscle cells, covered by an endothelial layer. The plaques are apposed on an otherwise normal valvular, or mural endocardium of the right heart. The resulting combined valvular lesions show a predominance o tricuspid incompetence, or of pulmonary stenosis. In one case with a widely patent foramen ovale there was involvement of left-heart valves in addition. Local effect of serotonin appears to play the main role in pathogenesis of the endocardial plaques.

Adult↗
PubMed2003

[Pulmonary embolism--temporal aspects].

The post-mortem incidence of pulmonary embolism (PE) in the years 1960-2000 was retrospectively reviewed. In five reference years 1960, 1970, 1980, 1990 and 2000 there was a total of 582 necropsy patients with PE encountered in 3420 autopsies of adults (> 18 y.)--an incidence of 17.0%. The incidence had a steadily declining trend--from 19.2% in 1960 to 14.2% in the year 2000. The incidence was higher in females (20.4%) than in males (14.4%). We have also followed the seasonal variation of PE incidence; this was the highest in autumn (September-November) 18.6%, and the lowest in spring (March-May) 14.3%. The average age of patients who died with PE increased from 63.7 y. in 1960 to 67.8 y. in 2000; it was higher in females (66.9 y.) than in males (64.2 y.). Our results confirm those of most pathological studies that the post-mortem incidence of PE is declining.

Aged↗
PubMed2002

Mitochondrial cardiomyopathy--case report.

A male infant developed progressive neuromuscular disease, hypertrophic cardiomyopathy and brain atrophy since the birth. Increased level of lactate with increased lactate/pyruvate ratio suggested a disturbance in the mitochondrial energy metabolism. The activities of respiratory chain complexes III, IV and II + III, of pyruvate dehydrogenase complex and of citrate synthase in isolated muscle mitochondria were low in comparison with controls, with parallel decrease in the content of protein amount of respiratory chain complexes III and IV. No large scale deletions of mitochondrial DNA (mtDNA) and mtDNA point mutations A3243G, A8344G or T8993G indicating syndromes MELAS, MERRF or NARP were detected. The boy died at the age of 7 weeks. The autopsy revealed typical changes of mitochondrial cardiomyopathy-marked myocardial hypertrophy with muscle pallor, histological finding of diffuse fine granularity of the cytoplasm in the perinuclear regions, and ultrastructural findings of mitochondrial hyperplasia, enlargement (megamitochondria) and abnormal shape.

Adult↗