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1.
Summary Large intracytoplasmic inclusions arising from the endoplasmic reticulum and nuclear envelope were found in the muscle fibers of a 32-year-old individual with a life-long, chronic and progressive sensory-motor neuropathy. The morphological features of the inclusions were similar to that occasionally reported in the striated muscles in several unrelated conditions as well as to hyaline or colloid inclusions commonly seen in motor neurons of the brain stem and spinal cord. The chemical nature of the inclusions is not known. Their occurrence in the muscle fibers is probably secondary to chronic denervation.  相似文献   

2.
Summary Tyrosine hydroxylase (TH) contents in the caudate nucleus, putamen, and substantia nigra from control and parkinsonism brains were measured for the first time by a sandwich enzyme immunoassay. Both the TH protein content and TH activity (Vmax) were decreased in parallel in the parkinsonian brains as compared with those of the control brains. In contrast, TH homospecific activity (activity per enzyme protein) was significantly increased in the parkinsonian brains. The results indicate that the decrease of TH activity in parkinsonian brains is due to the decrease of TH protein content as a result of cell death. The increase in the homospecific activity of residual TH in parkinsonian brain suggests such molecular changes in TH molecules as result in a compensatory increase in TH activity.  相似文献   

3.
Summary A 46-year-old female, with an 11 year history of malignant lymphoreticular disease, developed a neurological illness clinically manifested by a focal mass lesion in the left frontal lobe. In biopsied tissue, immunofluorescence study revealed the presence of JC antigen in the glial cells. Histologically, the lesion was characteristic of PML consisting of focal necrosis in the subcortical white matter, numerous fat laden macrophages and marked hypertrophy of oligodendrocytes and astrocytes. By electron microscopy, hypertrophic astrocytes contained intranuclear viral particles consistent with papova virions and aggregates of intracytoplasmic viral particles consisting of a single to several virions tightly surrounded by a single membrane. The membrane appeared to have been derived from that of the cellular vesicles. Fusion of the virus-associated membrane to the astroglial plasmalemma occurred when the virions appeared to shift towards extracellular space. The virioncontaining astrocytes showed cytoplasmic fibrillar hypertrophy similar to the characteristic gigantic astroglias of PML. This fact would provide an additional evidence that these gigantic cells, although lacking identifiable viral structures, were the result of anaplastic transformation by JC virus. Many virus-bearing sstroglias were noted to be in the early stage of cellular necrosis, or edematous degeneration. This further indicates that the JC virus is capable of inducing both lytic and abortive astroglial infections. Many oligodendroglias were hypertrophic due to the presence of intranuclear viral particles and markedly increased numbers of microtubules and free ribosomes in the cytoplasm. Membrane-bound intracytoplasmic viral particles were also noted in the oligodendroglias. Some fat laden macrophages contained large intracytoplasmic viral bodies, presumably orginating from phagocytized virus-bearing cells.This paper was presented in part at the 33rd Annual Meeting of Electron Microscopy Society of America, Las Vegas, August, 1975 (Abstract by Preskorn et al., 1975).Supported in part by Veterans Administration Research Project, U. S. A. No. 9242-01.  相似文献   

4.
The terms multicystic encephalomalacia and subcortical leucomalacia (SCL) are used to describe the presence of areas of necrosis that develop into cystic lesions inside the brain. These lesions are generally due to severe asphyxia and/or hypotension. The designation SCL can also be used to describe the extent and the seat of the lesion in the brain or to distinguish subcortical lesions from periventricular and/or combined ones. In this study we give an account of our experience with eight newborns admitted to our neonatal intensive care unit, who presented clinical-neurological alterations and encephalomalacic lesions whose presence was documented by ultrasonography.  相似文献   

5.
Summary The use of case histories in examining the premorbid personality of affectively ill patients is especially useful in the case of patients with a predominantly manic course of the disorder, because this kind of affective illness is very rare. The concept of the manic type of premorbid personality is described in detail and contrasted with the concept of the melancholic type often found in patients with a purely depressive course of the illness.  相似文献   

6.
Zusammenfassung An einer Stichprobe von 99 herzoperierten Patienten wurde zur Erfassung, Beschreibung und Klassifikation früh-postoperativer psychischer Störungen aus dem psychopathologischen Befundbogen des AMP/AMDP-Systems eine Kurzform (HRPD) entwickelt. Weitgehend mit Hilfe einer klassischen Itemanalyse wurde eine Auswahl von 36 Symptomen vorgenommen.Eine Faktorenanalyse dieser Kurzform ergab 8 Merkmalsfaktoren bzw. Syndromskalen: Desorientierung, Konzentrations-/Denkstörungen, paranoid-halluzinatorische Symptomatik, Angstsymptomatik, gehemmt-depressive Symptomatik, Hostilität, Kontrollverlust sowie Selbstaufgabe.Clusteranalytisch wurden 6 psychopathologisch verschiedene Patientengruppen gefunden: unauffällig, fast unauffällig, leichte psychoorganische Symptomatik mit Affektstörungen, schwere psychoorganische Symptomatik mit Kontrollverlust, Hostilität mit paranoid-halluzinatorischer und psychoorganischer Symptomatik, sowie delirante Symptomatik.Ein Vergleich der Ergebnisse der HRPD mit Syndrombeschreibungen anderer AMP/AMDP-Untersucher ergab zum Teil gute Übereinstimmungen, zum Ted aber auch deutliche Unterschiede, die am ehesten durch die besondere Situation herzoperierter Patienten bedingt sein dürfte.Vorform in englischer Sprache: HRPD = Hamburg Rating Scale for Psychic Disturbances  相似文献   

7.
Histomorphological and histochemical variability was studied in muscle specimens from 30 patients with congenital muscular dystrophy (CMD). We found involvement of the central nervous system in 8 patients (Fukuyama CMD, F-CMD), involvement of the brain and the eyes in 5 patients (muscle, eye and brain disease, MEB-D) and hypodense white matter on the CT scans of 2 patients with (sub)normal intelligence (occidentaltype cerebromuscular dystrophy, O-CMD). No morphological hallmarks were found to differentiate these subgroups. Only fat cell infiltration was found to be increased with increasing age in pure CMD (pure-CMD). The morphological data did not appear to be correlated with the clinical severity or type of dystrophy (pure-CMD, F-CMD, MEB-D and O-CMD). Immunohistochemistry with dystrophin, vimentin and desmin antibodies in 14 patients (6 pure-CMD, 5 F-CMD, 2 MEB-D and 1 O-CMD) showed a normal expression pattern.This investigation is part of the research program Disorders of the Neuromuscular System of the University of Nijmegen  相似文献   

8.
Summary It was the purpose of the present study to quantify the expected motor deficit in parkinsonian patients with the computer assisted Motor Performance Test Series (MPS), version 05.87 by Schuhfried (1987) and to examine which of the motor test variables found correlate at a significance level of p<0.01 with items of motor examination recorded at neurological examination and activities of daily living of the Unified Parkinson's Disease Rating Scale (UPDRS), version 3.0.38 patients with idiopathic Parkinson's disease (PD) stages I–IV according to Hoehn and Yahr, aged 41 to 73 years were studied. The study design, i.e. initial rating by the physician followed immediately by testing of motor function with MPS was strictly adhered to in each patient.Physician's rating of rigor and the scores of the semiquantitative tests (finger taps, hand movements and alternating movements) as expression of hypokinesia and the activities of daily living correlated with the 3 factors of the Motor Performance Test Series at a highly significant level independent of disease stage. Tremor is only partly and never significantly reflected in the motor data measured. Stages I–II and II–IV (Hoehn and Yahr) differ significantly in the representative data of the Motor Performance Test Series.The results of the study support the assumption that MPS is a valid instrument for quantitative measurement of the motor deficit in parkinsonian patients, but that only some subtests are pathognomonic.  相似文献   

9.
Summary Focal mucoid degeneration was found in a N. suralis biopsy of a 8 year old child, diagnosed clinically and electrophysiologically as progressive muscular atrophy Charcot-Marie-Tooth.  相似文献   

10.
Zusammenfassung In der vorliegenden Arbeit untersuchten wir die konsiliarisch-psychiatrische Versorgung eines großen Allgemeinkrankenhauses in Verbindung mit der Aufnahme auf eine Kriseninterventionsstation.Besonders interessierten uns Zusammenhänge zwischen der Häufigkeit des Auftretens von Suizidalität, psychiatrischer Krise und Übernahme von konsiliarisch-psychiatrisch untersuchten Patienten. Von insgesamt 6004 Patienten des Allgemeinkrankenhauses wurden in einem Zeitraum von 2 Monaten 227 (130 Frauen, 97 Männer) psychiatrisch untersucht. Am häufigsten wurden psychogene Reaktionen und Anpassungsstörungen (ICD-9: 308.0–309.9) mit 36.6%, am zweithäufigsten Suchterkrankungen (ICD-9: 303, 305.0, 304.0–304.9, 305.1–305.9) mit 26.8% diagnostiziert. In 90 Fällen lag eine psychiatrische Krise vor. Das Durchschnittsalter dieser Patienten war niedriger als das der Übrigen. Bei 91 Patienten fand man ein suizidales Syndrom. Es wurden 23 Patienten innerhalb und 7 nach Ablauf von 24 Std auf die Kriseninterventionsstation (KIS) übernommen.Mit Hilfe der Konfigurationsfrequenzanalyse (KFA) fanden wir 3 signifikante Typen: (1) Krisen-Patienten (N = 18), bei denen sowohl Suizidalitat, Krise und Ubernahme auf die Kriseninterventionsstation innerhalb von 24 Std zusammentraf; (2) typische Nicht-Krisen-Patienten (N = 111), bei denen keine der 3 Variablen bejaht wurde; (3) eine Gruppe (N = 48), die trotz des Vorliegens eines suizidalen Syndroms und einer aktuellen Krise nicht auf die KIS übernommen wurden.Bei der Gruppe von Patienten (N = 23), die eine angebotene Aufnahme auf die Kriseninterventionsstation ablehnten, bestätigte sich durch eine Nachuntersuchung nach Ablauf eines Jahres unsere Hypothese eines hohen Risikos.Die vorliegende Arbeit ist Herrn Prof. Dr. med. Wilhelm Feuerlein zum 65. Geburtstag gewidmet.  相似文献   

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