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1.
We studied three patients from two kinships, affected by early onset hereditary motor and sensory neuropathy with probable autosomal recessive inheritance (HMSN type III). Morphological studies of sural nerve biopsies revealed an abnormal myelin proliferation. Two adult patients with long-term follow up, lost ability to walk at 28 and 22 years and showed severe involvement of the cranial nerves. Our observations suggest that hypermyelination neuropathy with early onset is a progressive disease with poor long-term prognosis. In one kinship the occurrence of the disease in two sibs of both sexes but not in parents, is consistent with an autosomal recessive inheritance. Familial cases of hypermyelination neuropathy have not been described in previous reports. Morphological aspects of this condition are compared with other forms of hypermyelination neuropathy.Supported by Telethon-Italy for the project: Chronic inflammatory polyradiculoneuropathy: electrophysiological and immunopathological studies  相似文献   

2.
Summary Uptake of DL-Lysine-H3 into light and dark cells of the rat inferior olivary nucleus was studied by radioautographiy. Uptake in light cells was higher than in dark cells and appears to be possibly related to the greater frequency of relationship with glial satellites. Dysthyroidal states were also observed to increase uptake of radioactivity which was prominently observed in hypothyroid rats. Since dark cells appear to take up less lysine than normal cells and are less frequently closely associated with glial satellites it is suggested these cells may represent a less physiologically active unit than the light cells rather than being simply artifactually produced by the procedures employed.
Zusammenfassung Die Aufnahme von DL-Lysin-H3 in hellen und dunklen Zellen im unteren Olivenkern der Ratte wurde mit Autoradiographie beobachtet. Die Aufnahme war höher in den hellen Zellen als in den dunklen, was möglicherweise mit einer größeren Häufigkeit von Glia-Satellit-Verbindungen in Zusammenhang gebracht werden könnte. Veränderungen im hormonalen Zustand der Schilddrüse konnten auch dazu beitragen, die Aufnahme von Radioaktivität zu erhöhen, was besonders bei Ratten mit niedriger Schilddrüsenaktivität hervortrat. Da dunkle Zellen offenbar weniger Lysin aufnehmen als normale Zellen, und sie seltener mit Glia-Satelliten verbunden sind, wird angenommen, daß diese Zellen möglicherweise eine weniger aktive physiologische Einheit darstellen als die hellen Zellen und daß es sich nicht einfach um ein Kunstprodukt handelt.


This investigation was supported in part by a PHS research grant (NB-456802) from the division of Neurological Disease and Blindness and in part by Contract NONR 4018(01), (NR 101-592), between the Office of Naval Research, Department of the Navy, and the Research Foundation of the State University of New York.  相似文献   

3.
Zusammenfassung Bei 30 Patienten mit Neuropathien unterschiedlichen Schweregrades (subklinisch, leicht, mittelschwer und schwer) wurden am N. ulnaris neben den üblichen neurophysiologischen Parametern [distale Latenz, maximale motorische und gemischte Nervenleitgeschwindigkeit (Nlg.)] die Refraktärperioden (Rp.) (absolute Rp. und relative Rp.-Amplitude und -Latenz) und die unteren Grenzfrequenzen (u. F.) (u. F.-Amplitude und -Latenz) bestimmt.Beim Vergleich mit einem Normalkollektiv (n=31, s. Lowitzsch u. Hopf, (1972a)) war die Nlg. nur in 37% der Fälle pathologisch verlangsamt, während die relative Rp.-Latenz in ca. 80% und die u. F.-Latenz in ca. 60% pathologisch verändert waren.In zwei Stichproben (13 Normalfälle und 13 Polyneuropathien) mit einer normalen gemischten Nlg. von 51,0–63,5 m/sec unterschieden sich die Mittelwerte für die distale Latenz sowie die motorische und gemischte Nlg. statistisch nur auf dem 1%-Niveau, für die relative Rp.-Latenz und die u. F.-Latenz hingegen auf dem 0,5-Niveau.Die Bestimmung der Refraktärperioden, insbesondere der rel. Rp. L., sowie der unteren Grenzfrequenz (u. F. L.), stellt eine im Vergleich mit den üblichen neurophysiologischen Verfahren (Nlg.-Bestimmung) wesentlich empfindlichere Untersuchungsmethode zur Erfassung auch geringer (subklinischer) Funktionsstörungen des peripheren Nervensystems dar.Die unterschiedliche Beeinflussung der Refraktärperioden und der Grenzfrequenzen durch die Art des zugrundeliegenden pathologischen Prozesses (axonale Degeneration — segmentale Demyelinisierung — Mischtyp) wird an Hand der in 9 Fällen nervenbioptisch (N. suralis) gewonnenen Befunde diskutiert.
Refractory periods and frequent impulse conduction in mixed N. ulnaris of man in polyneuropathies
Summary Some electrophysiological parameters were studied in the ulnar nerve of 30 patients suffering from neuropathy of various origin and severity.Absolute and relative refractory periods and lower limiting frequencies were measured and compared to the usual parameters (distal motor latency, conduction velocity of motor fibres, and the mixed nerve action potential).The conduction velocity was indicative of the diseased function in 37% whereas the relative refractory period (latency) was abnormal in nearly 80% and the lower limiting frequency (latency) in about 60%.Two samples taken at random, each of them consisting of 13 patients with normal conduction velocities between 51.0 and 63.5 m/sec showed differences only at the 1% level (p<0.01) as far as the mean values of the distal latency and the maximum conduction velocity were concerned. The difference between the mean values of the relative refractory period (latency) and of the lower limiting frequency (latency), however, was highly significant (p<0.0005). Thus, in our experience, the relative refractory period (latency) and the lower limiting frequency (latency) are more sensitive indicators of mild functional disturbances of peripheral nerves than the maximum conduction velocity.
Die Untersuchungen wurden in dankenswerter Weise von der Deutschen Forschungsgemeinschaft unterstützt.  相似文献   

4.
Summary Seven cases of meningiomas with pseudopsammoma bodies have previously been described in the literature. Two additional cases are presented. Electron microscopy of the cells surrounding the pseudopsammoma bodies reveals an ultrastructure different from that of the meningotheliomatous cells. It is concluded that meningotheliomatous meningiomas with pseudopsammoma bodies are mixed tumours, including a non-meningotheliomatous component, the origin and significance of which is uncertain.  相似文献   

5.
Summary A study of primary (VEPs) and cognitive (ERPs) visual evoked potentials was carried out in a group of non-demented Afro-American Parkinson's disease (PD) patients. Current studies suggest that differences exist in the clinical manifestations of PD in Caucasian and non-Caucasian populations. Two horizontal sinusoidal gratings differing in spatial frequency, i.e., 1 and 4 cycles per degree (cpd), were presented in an oddball paradigm to 17 patients with PD and 17 age-matched control subjects. While the 1 cpd stimulus, is not expected to reveal retinal dopaminergic deficency, but only visuocognitive deficits, the 4 cpd may give direct information of both retinal and cognitive visual deficits. We measured the latencies and amplitudes of N70, P100 and P300 components, and derived the normalized measures of P300-N70 latency difference (Central Processing Time-CPT70), the P300-P100 latency difference (CPT100) and the P300 amplitude responses normalized to either N70 and P100 amplitude (Amplitude Ratios AR70 and AR100). Our results do show that cognitive electrophysiological deficits in younger PD patients exist in non-Caucasians, perhaps to an even greater degree than in Caucasians, and confirm that absolute and normalized ERP amplitude and latency abnormalities are a distinguishing feature of younger PD patients from controls. In particular P300 measures are abnormal for 1 cpd pattern. A negative correlation exists between P300 amplitude and the motor score. By comparing the results for 1 and 4 cpd stimuli it can be concluded that primary and cognitive visual abnormalities are independently affected in PD, implying that visuo-cognitive abnormalities are not passively determined by retinal dopaminergic deficiency.  相似文献   

6.
Re-build-up phenomenon, induced by hyperventilation, is a characteristic finding on electroencephalogram (EEG) in children with moyamoya disease, and suggests close correlation with cerebral ischemic attack. Its mechanism, however, remains obscure. In this study, we examined the relationship between the cerebral hemodynamics and this phenomenon before and after vascular reconstructive surgery. Regional cerebral blood flow and its reactivity to acetazolamide were analyzed using the xenon-133 inhalation method and single photon emission computed tomography in six children with moyamoya disease. These results were compared with the EEG. Regional vasoreactivity to acetazolamide was significantly lower in re-build-up-positive regions than in re-build-up-negative regions. Postoperative studies revealed a significant improvement of reactivity in the regions where the re-build-up phenomenon disappeared after surgery. These results suggest that the re-build-up phenomenon represents a focal reduction of the cerebral perfusion reserve in moyamoya disease.  相似文献   

7.
Summary A unique model of DA system regulation is presented, in which tonic steady-state DA levels in the ECF act to down-regulate the response of the system to pulsatile DA released by DA cell action potential generation. This type of regulation is similar in many respects to the phenomenon proposed to mediate the action of norepinephrine on target neurons; i.e., an increase in the signal-to-noise ratio as measured by postsynaptic cell firing (Freedman et al., 1977; Woodward et al., 1979). However, in this model the signal and the noise are neurochemical rather than electrophysiological. Furthermore, the noise (tonic DA in the ECF) actually down-regulates the signal (phasic DA release) directly, and thereby provides a signal of its own that affects the system over a longer time-course. Therefore, the difference between signal and noise may also depend on the time frame under which such determinations are made.  相似文献   

8.
Summary Electron microscopic observations were made of early remyelination after segmental demyelination in experimental allergic neuritis (EAN) and experimental diphtheritic neuropathy. In EAN 71% of axons which had been demyelinated were invaginated each within a single Schwann cell; remyelination associated with this cellular relationship was in accordance with the spiral myelin concept, 29% of demyelinated axons in EAN were initially enveloped each by several Schwann cell processes and the associated mechanism of remyelination was by tunication resulting in a transient irregular distribution of myelin lamellae around the axon circumference. In diphtheritic neuropathy regeneration more closely paralleled development in ontogeny. 88% of demyelinated axons were invaginated each within one Schwann cell, only 12% of axons were each enveloped by more than one Schwann cell process. Early remyelination by tunication was not observed in diphtheritic neuropathy. Additional loosely associated Schwann cell processes lying within the old Schwann cell basement membrane occurred frequently in both experimental conditions. Schmidt-Lanterman clefts, redundant myelin, and desmosomes were observed in the sheaths of incipient remyelination.  相似文献   

9.
Summary. Immunoreactivities of amyloid peptide(1–42) (A42-IR) and total protein (TTIR) were measured in lumbar cerebrospinal fluid of 48 patients (12 patients in each group) with normal pressure hydrocephalus (NPH), vascular dementia (VD), Alzheimers disease (AD), Parkinsons disease without dementia (PD) and 24 controls (CON) using sensitive and specific enzyme immunoassays. TTIR in NPH was not significantly changed compared with VD, PD and CON, while NPH-A42-IR was significantly decreased compared with PD and CON. In AD, significant increases of TTIR and significant decreases of A42-IR were found. Using a TTIR by A42 plot, all NPH, PD, and CON samples were within the non-AD plot region. 92% of AD and VD samples were within the AD and non-AD area, respectively. We conclude that combined measurement of A42-IR and TTIR contributes to the differential diagnosis of NPH vs. AD and of AD vs. VD, respectively.  相似文献   

10.
Mothers of 182 preschool nursery school children rated their own parenting responses on a Parent's Report questionnaire. At the same time the mothers responded to the Behavior Style Questionnaire (BSQ) from which scores were determined for nine categories of temperament. On the basis of category scores the children were grouped into one of five temperament clusters i.e. easy, difficult, slow to warm up, high intermediate, low intermediate. The children's membership in BSQ clusters was independent of sex, age, birth order, and mothers employment status but there was a significantly higher ratio of easy children from higher socioeconomic classes I and II. Mothers of children grouped in either the difficult or slow to warmup clusters were more likely to use guilt inducing and temper-detachment parenting styles than mothers of children grouped in the easy cluster.  相似文献   

11.
Five diagnostic systems designed to differentiate infantile autism and early childhood schizophrenia were compared by deriving scores on 44 children referred consecutively to the same clinical center. While the autistic scales devised by Rimland, Polan and Spencer, Lotter, and the British Working Party correlated significantly, the degree of correspondence (35%) indicated that several children obtained high autistic scores in one system but low scores in another. The BWP's term schizophrenia has more correspondence with the term autism used by others than with Rimland's schizophrenia. In the DeMyer-Churchill categorical system (early schizophrenia, primary autism, secondary autism, and non-psychotic subnormal), primary autism most resembles Rimland's concept of infantile autism as measured by his E-1 version. All other systems differentiate psychotic from non-psychotic children but do not distinguish any of the psychotic subgroups.This study was supported in part by Public Health Service Grant No. MH05154 and also by LaRue D. Carter Memorial Hospital, State of Indiana, Indianapolis, Ind.The authors wish to thank Dr. Bernard Rimland for providing his scoring key.  相似文献   

12.
Summary Immunochemical analyses revealed that a monclonal antibody Am-3 recognized amyloid precursor protein (APP) in senile plaques extracted from Alzheimer's brain, but did not recognize amyloid protein. Immunohistochemically, however, the staining pattern of Am-3 in frozen section of Alzheimer's brain was almost the same with that of rabbit polyclonal antibody to amyloid peptide which could recognize both amyloid protein and APP. In other words, APP was present in senile plaques of various types, cerebrovascular amyloid and granular deposits. The granular deposits were 5–10 m in size and laminarily distributed in the 1st, 3rd and 4th layers of cerebral cortex. They were especially abundant in 1st and 4th layers where senile plaques were usually fewer in number. Although the distribution in the cerebral cortex was different between the senile plaques and the granular deposits, the number of the granular deposits was well correlated with that of senile plaques. The granular deposits were negative in Congo-red birefringence, but contained amyloid protein as well as APP fragment judging from positive staining by both Am-3 and polyclonal antibody to synthetic amyloid peptide. Thus, they could be regarded as pre-amyloid.  相似文献   

13.
Summary In order to cut expenditure on mental health care in Brazil, the national authority responsible for the financing of health care imposed in 1977 the following two restrictions on reimbursement: a) the costs for inpatient treatment under the diagnoses neurosis and alcoholism would be paid only for a maximum length of hospital stay of 30 days and b) the costs for inpatient treatment under the diagnoses oligophrenias and epilepsies would no longer be paid. We studied the effects of these administrative measures upon the yearly frequencies of diagnoses and the mean lengths of hospital stay in 27377 treatment episodes between 1975 and 1982. We found significant decreases in both variables for the above mentioned diagnoses after 1977. At the same time the frequencies of the diagnoses alcoholic psychoses, psychoses associated with other cerebral conditions and other psychoses increased significantly. These findings can probably not be attributed to true changes in morbidity, but are rather the results of changes in diagnostic habits in response to administratively imposed austerity measures.  相似文献   

14.
Summary This study is an attempt to determine the creatine kinase B (CK-B) subunit levels in neurogenic atrophies. A group of 69 patients was studied and the results were compared with those in a group of 32 patients with muscle disease. The results showed that the CK-B levels are considerably higher in patients with amyotrophic lateral sclerosis (P<0.001) and peroneal muscular atrophy (P<0.001). Further studies in the various subgroups of neurogenic atrophies showed that, regardless of the nosological entity, the CK-B activity is considerably higher: (1) in the widespread as opposed to limited forms (P<0.001); (2) in the chronic than in the acute neurogenic atrophies (P<0.001); and (3) in the active as opposed to residual forms (P<0.02). It is suggested that the increase of CK-B in neurogenic atrophies is a strong indication of an active regeneration process in the denervated muscle.  相似文献   

15.
Summary. Higher plasma homocysteine concentrations can influence genomic DNA methylation in peripheral blood cells. In the present controlled study we observed a significant increase (10%) of genomic DNA methylation in patients with alcoholism (t=–3.16, df=158, p=0.002) which was significantly associated with their elevated homocysteine levels (multiple linear regression, p<0.001). Since methylation of DNA is an important epigenetic factor in regulation of gene expression these findings may have important implications for a possible subsequent derangement of epigenetic control these patients.D.B. and B.L. contributed equally to this work  相似文献   

16.
This study compared parent-reported competencies and behavioral/emotional problems in demographically-matched samples of Greek and American children, ages 6–11. Parents of 356 children of each nationality completed the Child Behavior Checklist (CBCL). Competence scores were higher for American children, except on Academic Competence, where scores were higher for Greek children. Greek scores were significantly higher than American scores on the Withdrawn, Anxious/Depressed, Attention Problems, Delinquent Behavior, Aggressive Behavior, Internalizing, Externalizing, and Total Problem scales. On the Anxious/Depressed syndrome, nationality accounted for 14% of the variance. There were few main effects for sex and age and fewer interactions. The higher problem scores in the Greek sample were partly due to the tendency of Greek parents to use extreme item scores. When items were scored present v. absent, Greek scores were higher only on Withdrawn, Anxious/Depressed, Internalizing, and Total Problems, while American scores were higher on Somatic Complaints and Thought Problems. Nationality differences in rates of referral for mental health services and sample differences in exclusion criteria for prior mental health services may have contributed to differences in problem scores. Results are compared to findings from other cross-cultural studies.
Zusammenfassung Diese Studie vergleicht die Fähigkeiten und Verhaltens- bzw. emotionalen Auffälligkeiten in demographisch parallelisierten Stichproben von griechischen und amerikanischen Kindern im Alter von 6-11 Jahren. Die Eltern von 356 Kindern der beiden Nationalitäten füllten die Child Behavior Checklist (CBCL) aus. Die Kompetenzwerte waren bei den amerikanischen Kindern mit Ausnahme der akademischen Fähigkeiten höher. Die griechischen Werte waren signifikant höher als die amerikanischen im Hinblick auf die Skalen Zurückhaltung, ängstlich/depressiv, Aufmerksamkeitsstörungen, delinquentes Verhalten, aggressives Verhalten, Internalisation, Externalisation und Gesamtauffälligkeiten. Im Hinblick auf das ängstlich/depressive Syndrom war die Nationalität für 14% der Varianz verantwortlich. Nur vereinzelt wurden Haupteffekte im Hinblick auf Geschlecht und Alter und eine geringere Anzahl Interaktionen festgestellt. Die höheren Problemwerte in der griechischen Stichprobe waren teilweise durch die Tendenz der griechischen Eltern bedingt, extreme Werte anzukreuzen. Bei den Items, die als vorhanden bzw. nicht vorhanden gewertet werden mußten, waren die griechischen Werte nur bei den Skalen Zurückhaltung, ängstlich/depressiv, Internalisation und Gesamtprobleme höher, während amerikanische Werte für die Skalen körperliche Beschwerden und Denkstörungen höher lagen. Nationalitätsunterschiede der Zuweisungsraten zu psychosozialen Diensten und Stichprobenunterschieden im Hinblick auf die Ausschlußkriterien für vorangegangene Inanspruchnahme psychosozialer Dienste könnten zu den unterschiedlichen Problemwerten beigetragen haben. Die Ergebnisse werden verglichen mit anderen transkulturellen Studien.

Résumé Cette étude a comparé les compétences et les problèmes de comportement et affectifs rapportés par les parents d'un échantillon d'enfants grecs et américains âgés de 6 à 11 ans appariés démographiquement. Les parents de 356 enfants de chaque nationalité ont rempli la Child Behavior Checklist (CBCL). Les scores de compétence étaient plus élevés pour les enfants américains excepté pour la compétence scolaire pour laquelle les scores étaient plus élevés chez les enfants grecs. Les scores grecs étaient significativement plus hauts que les scores américains quant au retrait, l'anxiété-dépression, les problèmes d'attention, le comportement délinquant, le comportement agressif, les échelles d'internalisation, d'externalisation et de problèmes totaux. Pour le syndrome anxiodépressif, la nationalité intervenait pour une variance de 14%. Il y avait peu d'effets principaux en ce qui concerne le sexe et l'âge. Les scores de problèmes les plus élevés dans les échantillons grecs étaient en partie dûs à la tendance des parents grecs à utiliser des items extrêmes. Quand les items étaient cotés présents versus absents, les scores grecs étaient plus élevés seulement en ce qui concerne le retrait, l'anxiété-dépression, l'internalisation et les problèmes totaux tandis que les scores américains étaient plus hauts en ce qui concerne les plaintes somatiques et les problèmes de pensée. Les différences de nationalité en ce qui concerne les taux de référence à des services de santé mentale et les différences des échantillons dans les critères d'exclusion pour l'utilisation antérieure de services de santé mentale, peuvent avoir contribué aux différences dans les scores de problèmes. Les résultats sont comparés aux autres études transculturelles.
  相似文献   

17.
This paper concerns the relationship between authority structures and two problems reported in the literature as common to milieu or therapeutic community wards. Psychiatric wards with rational-legal and charismatic authority structures are found more likely to experience mood and morale swings on the part of patients and staff and to spend excessive time and energy changing ward rules.  相似文献   

18.
Summary The density of the cytoplasm and axoplasm of the anterior horn cell in rats was determined by X-ray microradiography. The average density of the cytoplasm of more than 400 cells from control rats was 0.31 g/3, while that of over 600 cells from rats fed IDPN (- iminodipropionitrile) was 0.43 g/3.Hyperactivity developed during the first 5 weeks and was associated with a gradual increase in cytoplasmic density to 0.51 g/3.At 6 weeks there was a drop in density to 0.36 g/3 which coincided with the appearance of axonal balloons having a density of 0.17 g/3.During the 7–12th week on the diet, the cytoplasmic density showed a gradual increase to 0.59 g/3 and the balloons to 0.29 g/3.The volume of the nerve cells remained fairly constant. The density increases were discussed in relation to hypertrophy, dystrophy, and hyperactivity.
Zusammenfassung Die Dichte des Cytoplasmas und Axoplasmas der Vorderhornzellen von Ratten wurde durch Röntgenmikroradiographie bestimmt. Die mittlere Dichte des Cytoplasmas von mehr als 400 Zellen der Kontrollratten war 0,31 g/3, während die mittlere Dichte von mehr als 600 Zellen der Ratten, die mit IDPN (- iminodipropionitrile) gefüttert waren, 0,43 g/3 war.Hyperaktivität entwickelte sich während der ersten 5 Wochen und war mit einer progressiven Zunahme der Cytoplasmadichte bis auf 0,51 g/3 verbunden.Nach 6 Wochen sank die Dichte auf 0,36 g/3. Diese Tatsache traf mit dem Auftreten der Axonauftreibungen zusammen, die eine Dichte von 0,17 g/3 hatten.Nach 7–12 Wochen zeigte die Cytoplasmadichte eine progressive Zunahme auf 0,59 g/3 und die der Auftreibungen eine Zunahme auf 0,29 g/3.Das Volumen der Nervenzellen blieb ziemlich konstant.Die möglichen Zusammenhänge zwischen Zunahme der Dichte, Hypertrophie, Dystrophie und Hyperaktivität werden dargestellt.


Supported by U. S. Public Health Grant NB 1305.  相似文献   

19.
A great deal of discussion and research has gone into defining and clarifying the role of case manager (CM) for persons with severe mental illness. This three state survey examines the philosophy and activities of practicing CMs in an attempt to identify current styles of case management. A cluster analysis based on CM rankings of five CM functions suggested four styles of case management: supportive social worker, individual therapist, therapist broker, and community advocate. Overall, CMs rated supportive interventions as most important and formal psychotherapy as relatively unimportant. CM style was related to CM activity (i.e., distribution of effort). Differences between states are noted and implications for future research are discussed.  相似文献   

20.
Summary This paper reports the conclusions drawn from an intensive study of the relationships existing between the thinking processes of neurotic patients and their emotional experiences. The data of this study are not suitable to tabulation and statistical analysis, and the conclusions only are presented. It is suggested that proof of the conclusions be sought by other investigators applying the principles outlined to individual patients as a test of validity. No other practical method of assembling proof seems available. To apply a principle, for instance, one might search to find what imagined danger a given patient is fantasying at the moment he is experiencing fear. If one assumes that such an idea must exist, and dismisses all protestations to the contrary, it is surprising how easily and frequently the idea giving rise to fear can be uncovered. If one assumes, on the other hand, that a patient can, indeed, be unaware of what he is afraid of, then the investigator may never find out what the imagined danger is.The accompanying schematic diagram illustrates the classification scheme proposed.  相似文献   

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