bluuebunny/crossref_metadata_2025 huggingface dataset:
Potentiometrische Verfolgung der Reduktion von Permanganat durch Ferroion und Jodion
Authors: Müller Erich , Möllering Heinrich
Published: January 2005

Aus dem Verlauf der Spannungskurven, die bei der potentio‐metrischen Verfolgung der Reduktion von Permanganat mit Ferro‐ und Jodion erhalten werden, konnten verschiedene Schlüsse über deren Verlauf gezogen werden, die auch für die Maßanalyse von Wichtigkeit sind.
Über die Natur des martensitischen Gefügebildes
Authors: Sauerwald F. , Jackwirth G.
Published: January 2005

Nach der Methode der Schliffglühung im Vakuum und neutralen Salzen und folgender Abschreckung wurde festgestellt, daß mit normaler Ätzung hervorgerufene Martensitstruktur immer nach dem ursprünglichen γ‐Korn orientiert ist, daß immer in 1, 2 oder 3 Systemen auftretende Gleitlinien und Verwerfungserscheinungen auf dem nicht weiter behandelten Schliff auftreten, die in ihrer Orientierung ebenfalls durch das γ‐Korn gegeben sind.
Über ein hochmolekulares Zwischenprodukt der Zersetzung von [Pt en2]J2 durch Säuren
Authors: Schleicher A. , Schmitz W.
Published: January 2005

Durch Zusatz von verdünnten Säuren zu Lösungen von [Pt en2]J2 wird zunächst die Bildung eines schwerlöslichen Hydratationsproduktes desselben von der Zusammensetzung: [Pt2 en4J · 12 H2O]J · 5 H2O veranlaßt. Gleichzeitig ausgeschiedenes Jod führt dann erst zur Zersetzung zu PtJ2 und zur Bildung von Äthylendiaminsulfat. Starke Säuren bewirken dieses sofort.
Die Grundlagen der titrimetrischen Schnellbestimmung großer Mengen Mangans mit Hilfe von Phosphormonopersäure
Authors: Heczko Theodor
Published: January 2005

Durch Einwirkung von Phosphormonopersäure in saurer Lösung auf Mangansalze entsteht eine gelöste, relativ haltbare Komplexverbindung des dreiwertigen Mangans, an deren Aufbau kondensierte Phosphorsäuren beteiligt sind.
Herstellung und Eigenschaften von Nitriden
Authors: Friederich Ernst , Sittig Lieselotte
Published: January 2005

Es wurde gezeigt, daß man aus Oxydkohlegemisch im Stickstoffstrom bei etwa 1200° die bekannten Nitride der Elemente Titan, Vanadin, Bor, Silicium und Lanthan von der Zusammensetzung TiN, VN, BN, Si3N4 und LaN in reinem Zustande erhalten kann.
Über die Verseifungsgeschwindigkeit von Ionenestern
Authors: Brönsted J. N. , Delbanco Agnes
Published: January 2005

Die beiden Reaktionsstufen bei der Verseifung der Ester zweibasischer Säuren folgen unter sich verschiedenen Reaktionsgesetzen.

Die Verseifung der Ionenester ist ein exponentiell salzempfindlicher Vorgang mit primärer, positiver Salzwirkung.

Die Verseifung des Nitrourethanions kann durch die die Reaktion begleitende Stickoxydulentwicklung verfolgt werden. Die Messung der Reaktion ergab zufriedenstellende Übereinstimmung mit der neueren Theorie der Reaktionsgeschwindigkeit.
Untersuchungen über die Löslichkeit des Magnesiumhydroxyds. II. Die Löslichkeitsprodukte und die Dissoziationskonstante der Magnesiumhydroxyde
Authors: Gjaldbaek J. K.
Published: January 2005

Die Löslichkeitsprodukte der Magnesiumhydroxyde wurden auf Grund von Versuchen berechnet, die eine elektrometrische Bestimmung der Hydroxylionenaktivität in mit Magnesiumhydroxyd gesättigten Magnesiumchloridlösungen umfassen und ergaben sich bei 18° für stabiles und labiles Magnesiumhydroxyd als 10−10,95 bzw. 10−9,2.
Herstellung und Eigenschaften hochschmelzender niederer Oxyde
Authors: Friederich Ernst , Sittig Lieselotte
Published: January 2005

Es wurde eine Reihe niederer Oxyde hergestellt und deren Eigenschaften, und zwar Farbe, Härte, Schmelzpunkt und elektrische Leitfähigkeit untersucht.
Die Geschwindigkeit der Reaktion 2NO + Cl2→2NOCl im Kondensat
Authors: Trautz Max , Gerwig Wilhelm
Published: January 2005

1. Die Löslichkeit von NO in flüssigem NOCl und einigen anderen Flüssigkeiten wurde gemessen.
Über die Halogenide des Iridiums
Authors: Krauss F. , Gerlach H.
Published: January 2005

1. Ein Weg für die Darstellung der Iridium‐4‐halogenide ist noch nicht gefunden.
Über Salze und Komplexverbindungen des vierwertigen Vanadiums
Authors: Rosenheim Arthur , yü Mong Hsin
Published: January 2005

1. Es wurde gezeigt, daß die Alkalivanadylsulfate sich in siedender Schwefelsäure nach der Gleichung

zersetzen und es wurde demgemäß einerseits kristallisierte Vanadinschwefelsäure der Zusammensetzung:

andererseits eine Reihe schön kristallisierter, in Wasser und verdünnten Säuren ganz unlöslicher Doppelsulfate des dreiwertigen Vanadiums der Zusammensetzung

erhalten. Die hier beschriebenen Verbindungen unterscheiden sich in ihren Eigenschaften von den bisher bekannten Sulfaten des fünfwertigen und Doppelsulfaten des dreiwertigen Vanadiums.
Über den Verlauf der elektrochemischen Reduktion fester Elektroden
Authors: Fischbeck Kurt
Published: January 2005

1. Es wurde ein Coulometer beschrieben, das es gestattet, 1.10−3 Coulomb durch unmittelbare Ablesung zu messen.
Untersuchungen über Ozon
Authors: v. Wartenberg Hans , v. Podjaski Georg
Published: January 2005

Man kann das Ozon in solchen Verdünnungen, wie sie zu Lüftungszwecken benutzt werden, unter Durchsaugen von 1–21 Luft in 5–15 Min. durch 2–3 ccm KJ‐Lösung vollständig absorbieren und mit 5–10% Genauigkeit titrimetrisch bestimmen mit einem leicht zusammenstellbaren Apparat.
Beiträge zur Kenntnis des Systems Eisen‐Zinn
Authors: Wever Franz , Reinecken Walter
Published: January 2005

In Zusammenfassung der Gesamtheit der vorstehend entwickelten Beobachtungen ergeben sich somit als Kennzeichen für das binäre System Eisen‐Zinn ein Eisen‐Zinnmischkristall mit einer Sättigungsgrenze von 18%, eine Verbindung Fe3Sn mit 41,9% Sn, die bei 890° unter Bildung einer zweiten Verbindung FeSn2 mit 81% Sn zerfällt; letztere erfährt bei 780 755 und 49°C polymorphe Umwandlungen.
Die Anwendung von flüssigem Amalgam in der volumetrischen Analyse. VI. Bestimmung von Chromsäure, Ferrieisen und Ferricyanid mittels Titanosulfat
Authors: Someya Kin'ichi
Published: January 2005

Ez wurde Titanosulfat durch Amalgamverfahren bereitet und bei der Titration von Chromsäure ein Gemisch von Chromsäure und Ferrieisen und von Ferricyanid angewandt, wobei man Diphenylamin als Indicator benutzte.

Das Diphenylamin erwies sich auch im Falle von Stannochloridtitration des Ferricyanids anwendbar.
Die Bildung der Gallerten von Vanadiumpentoxyd und anderen anorganischen Stoffen
Authors: Ghosh S. , Chakravarti D. N. , Dhar N. R.
Published: January 2005

Feste durchsichtige Gallerten von Vanadiumpentoxyd lassen sich leicht herstellen durch Dialyse eines Sols von Vanadiumpentoxyd.

Die Koagulation eines nicht dialysierten konzentrierten Sols von Vanadiumpentoxyd durch Elektrolyte führt zur Bildung einer Gallerte.
Peptisation und Bildung von komplexen Ionen
Authors: Dhar N. R. , Ghosh S.
Published: January 2005

1. Die Versuchsergebnisse zeigen, daß der Bildung von komplexen Ionen aus einer wenig löslichen Substanz (besonders in frisch gefällter Form) die Bildung eines Kolloides vorausgeht.
Ein Verfahren zur Herstellung großer Kristalle
Authors: Kyropoulos S.
Published: January 2005

Es wird ein Verfahren beschrieben zur Herstellung großer, einheitlicher Kristalle aus der Schmelze. Es ist durch Konstanthaltung der Temperatur der Schmelze und einseitigen Wärmeabfluß durch einen Tauchkühler charakterisiert, durch dessen zweckmäßige Handhabung stets die Bildung von Einkristallen erzwungen werden kann.
Zur Kenntnis des Gleichgewichts bei der Reduktion von Chromsesquioxyd und Urandioxyd mittels Kohlenstoffs, sowie bei der Einwirkung von Stickstoff auf Urancarbid
Authors: Heusler Otto
Published: January 2005

1. Es wurde gezeigt, daß es möglich ist, im außengekühlten, innenbeheizten, elektrischen Vakuumofen chemische Gleichgewichte zwischen festen und gasförmigen Phasen bei Temperaturen zu messen, deren Höhe die Verwendung eines abgeschlossenen, von außen beheizten Reaktionsraumes nicht mehr gestattet. Die Untersuchung derartiger Gleichgewichte beschränkt sich auf solche Systeme, deren gasförmige Reaktionsteilnehmer permanente Gase und deren feste Phasen bei den anzuwendenden Temperaturen nicht merklich flüchtig sind.
Über eine Methode zur Bestimmung des Polymerisationsgrades des Dampfes im Siedepunkt
Authors: Kirejew Valentin
Published: January 2005

Die Vergleichung der Werte des Trouton'schen Koeffizienten, die nach den Verdampfungswärmedaten bestimmt sind, mit den aus den Dampfdruckdaten berechneten, gibt ein Mittel an die Hand, den Polymerisationsgrad des gesättigten Dampfes im Siedepunkte zu bestimmen.

Ein gleiches leistet auch die Vergleichung der Werte der ebullioskopischen Konstanten, die experimentell bestimmt wurden, mit den aus Dampfdruckdaten berechneten.

Beide Wege geben übereinstimmende Resultate, die für die Essigsäure auch mit dem aus der Dampfdichte berechneten Polymerisationsgrad übereinstimmen.
Die Hydrolyse von Zirkoniumchromat
Authors: Krishnamurti Panchapakisa , Dey Biman Behari
Published: January 2005

1. Der Niederschlag von Zirkoniumchromat aus konzentrierten Zirkoniumsalzlösungen mit einem großen Überschuß von Kaliumbichromat scheint zusammengesetzt nach der Formel:

.
Zur Frage der Stabilität des Eisencarbides bei höherem Druck
Authors: Scheil Erich
Published: January 2005

Es wird aus den Messungen von F. SAUERWALD auf die Existenz eines Gleichgewichtsdruckes zwischen den Eutektiken γ Fe und Graphit einerseits und γ Fe‐Fe3C andererseits geschlossen und hierfür das p t x Diagramm besprochen.
Modes of social support: The social support behaviors (SS‐B) scale
Authors: Vaux Alan , Riedel Sharon , Stewart Doreen
Published: January 2005

Conclusion

The SS‐B appears to be a promising measure of five modes of available supportive behavior: emotional support, socializing, practical assistance, financial assistance, and advice/guidance. The measure was designed to assess supportive behavior available from family and from friends. For specific purposes the measure might be modified to tap enacted supportive behavior, as was done in Study 4, or might focus on different sources (cf. Tardy, 1985). Further evidence of the divergence of mode‐specific scales would be valuable and might be demonstrated by comparing samples with mode‐specific deficits, by comparing support received for different problem types (in a more sophisticated fashion than was done here), and by showing differentiated associations between modes of support and various outcome variables. The reliable and valid assessment of specific modes of supportive behavior is an important agenda item in social support research: Such a measure would allow more elaborate theories to be tested, and will undoubtedly prove essential in a complete understanding of social support processes.
Lumping or splitting? “ophthalmoplegia‐plus” or kearns‐sayre syndrome?
Authors: Berenberg R. A. , Pellock J. M. , DiMauro S. , Schotlan D. L. , Bonilla E. , Eastwood A. , Hays A. , Vicale C. T. , Behrens M. , Chutorian A. , Rowland L. P.
Published: January 2005

Five new cases and 30 others from the literature were characterized by the clinical triad of progressive external ophthalmoplegia, atypical pigmentary degeneration of the retina, and heart block. The syndrome is sporadic, not hereditary, and begins by the age of 20 years. In many cases there is other evidence of widespread neurological disorder, implicating specific areas: cerebellum; auditory and vestibular systems; skeletal muscle; and, less often, intellectual function or corticospinal tracts. Short stature and delayed sexual maturation are also frequent. The CSF protein content is almost always greater than 100 mg/dl. Spongioform encephalopathy was found in 4 postmortem examinations. In all cases so examined, muscle mitochondria were abnormal.

The constancy of these manifestations suggests that the disorder is a true entity, not an artificial subclass of the large group of disorders associated with ophthalmoplegia. Several characteristics suggest that the disorder of neuronal and muscle metabolism is induced by persistent viral infection.

The sequence of manifestations may vary, and cardiopathy in particular may be delayed for many years. Patients with ophthalmoplegia, pigmentary degeneration of the retina, and abnormal CSF protein content are likely to develp the full syndrome. This was illustrated by observation of 2 previously reported cases and 1 new one. Because the cardiopathy may be fatal, a pacemaker should be used if indications are appropriate.
Two biochemical types of duchenne dystrophy: Sarcoplasmic reticulum membrane proteins
Authors: Samaha Frederick J. , Congedo Carol Z.
Published: January 2005

The sarcoplasmic reticulum membrane proteins were studied in 10 patients with Duchenne dystrophy by acrylamide gel electrophoresis. Two distinctly abnormal patterns in the distribution of six proteins were identified. Although the patients could be separated biochemically into two groups, no such distinction could be made from the clinical or clinical laboratory findings. The syndrome of Duchenne dystrophy may include at least two biochemically distinct diseases.
Hypertensive putaminal hemorrhage
Authors: Hier Daniel B. , Davis Kenneth R. , Richardson E. P. , Mohr J. P.
Published: January 2005

Twenty‐four consecutive cases of putaminal hemorrhage diagnosed by the technique of computerized axial tomography are reported. The anatomy of the hemorrhages as visualized by CT scanning showed excellent correlation with the pathological findings in 3 autopsied patients. The size of the hemorrhage, as measured from the scan, was correlated with the occurrence and severity of several features of the clinical examination and with the clinical outcome. The relatively low overall mortality of 37% reflected the inclusion in this series of many small, nonfatal hemorrhages that would likely have escaped diagnosis before computerized axial tomography became available.
Activation of periodic complexes of subacute sclerosing panencephalitis by sleep
Authors: Westmoreland Barbara F. , Gomez Manuel R. , Blume Warren T.
Published: January 2005

Three patients with clinically proved subacute sclerosing panencephalitis (SSPE) had electroencephalograms in which periodic stereotyped complexes characteristic of SSPE were apparent during sleep but were not observed during the initial awake recordings. The first patient had periodic complexes only during sleep. The second and third patients had complexes that were inconstant and sporadic during the awake recordings but regular and periodic during sleep. Although sleep can have a variable effect on the electroencephalographic pattern in SSPE, it may facilitate elicitation of typical periodic complexes in questionable cases of SSPE.
Cerebral infarction complicating subclavian vein catheterization
Authors: Hurwitz Barrie J. , Posner Jerome B.
Published: January 2005

Cerebral infarction has not previously been reported as a complication of attempted subclavian vein catheterization for parenteral nutrition. Two patients developed clinical and pathological evidence of embolic infarction in the left carotid and left vertebral distributions, respectively, when an artery was inadvertently punctured during attempts to catheterize the left subclavian vein. These two complications occurred in more than 1,000 successful subclavian vein catheterizations.
The adult form of acid maltase (α‐1,4‐glucosidase) deficiency
Authors: Karpatid George , Carpenter Stirling , Eisen Andrew , Aubé Michel , DiMauro Salvatore
Published: January 2005

A 31‐year‐old man who had had slowly progressive proximal limb and trunk muscle wasting and weakness for seven years presented with pneumonia related to weak respiratory muscles. Histochemical and electron microscopical study of biopsies from deltoid and quadriceps muscles showed lysosomal storage of glycogen as well as ENnervation and reinnervation, implying primary disease both of muscle and of lower motoneurons. Electromyographic activity suggested a denervating process. Activity of acid λ‐1,4‐glucoside (acid maltase) was severely ENcreased in muscle and urine. Neutral maltase activity and glycogen content were normal in muscle. The findings in this patient suggest that in the adult from of acid maltase deficiency a significant neurogenic component may contribute to muscle wasting and weakness, presumably because of involvement of spinal motoneurons.
Viruslike particles in amyotrophic lateral sclerosis: Electron microscopical study of a case
Authors: Peña Carlos E.
Published: January 2005

Autopsy of a 57‐year‐old woman dying from amyotrophic lateral sclerosis (ALS) revealed the characteristic pathological changes of the disease. Ultrastructurally, the main finding was the presence of tubular viruslike particles within the cisterns of the rough endoplasmic reticulum in the neurons of the precentral gyrus and anterior horns of the spinal cord. These particles had a mean diameter of 80 to 85 nm and were composed of a central core and a multilayered wrapping; their morphology was consistent with a rhabdovirus. It is believed that the exclusive location of particles in neurons and the presence of fine structural alterations in these cells, consistent with a cytopathogenetic effect, may indicate a relation between the particle and ALS.
Fibromuscular dysplasia: Multiple “spontaneous” dissecting aneurysms of the major cervical arteries
Authors: Ringel Steven P. , Harrison Stephen H. , Norenberg Michael D. , Austin James H.
Published: January 2005

A patient presented clinically with acute bilateral carotid artery thrombosis and at autopsy had fresh dissecting aneurysms of all four major cervical arteries. The underlying vascular disorder was fibromuscular dysplasia. It is suggested that relatively minor injury to vessels already pathologically involved contributed to the multiple dissecting aneurysms in this patient.
The eeg spike: Signal of information transmission?
Authors: Stevens Janice R.
Published: January 2005

Enhanced neuronal excitability or coherence, leading to a rapid‐bursting mode of unit discharge or to synchronous polarization, underlies the electroencephalographic spike. The propensity for EEG spike activity to occur in relation to events that require absolute command of neural circuitry suggests that the EEG spike represents a mode of discharge of underlying neural elements which is employed for communication of imperative messages. Normal when confined to certain axial nuclei of the brain during sleep or during photic or sexual excitation, compensatory in response to deafferentation or hypoxia, this powerful transmission mode must have certain advantages to have persisted throughout evolutionary history.
Cerebral necrosis following radiotherapy of extracranial neoplasms
Authors: Rottenberg David A. , Chernik Norman L. , Michael D. F. Deck , Ellis Frank , Posner Jerome B.
Published: January 2005

We have examined 6 patients with delayed cerebral necrosis following irradiation of extracranial neoplasms. Four of the 6 patients received 1,760 rets (or less) tumor dose. The initial symptoms attributable to radiation necrosis appeared 4 to 31 months after irradiation and were those of a focal supratentorial mass. Cerebral angiography delineated an avascular frontal or temporal lesion in all 6 patients; in 1 case a magnification study revealed narrowing, irregularity, and occlusion of small cortical vessels. Four of our 6 patients underwent craniotomy with partial or complete surgical extirpation of necrotic brain tissue. Two operated patients are alive and without disabling neurological symptoms 30 and 25 months, respectively, after the operation. The characteristic neuropathological features of delayed radiation necrosis of brain suggest that vascular injury rather than neuronal or glial damage is of primary pathogenetic significance.
Nonketotic hyperglycinemia: Report of a case and review of the clinical, chemical, and pathological changes
Authors: Slager Ursula T. , Berggren Robert L. , Marubayashi Stanley
Published: January 2005

A female infant with neonatal hypotonia and lethargy was found to have nonketotic hyperglycinemia. She died at the age of 5 days. Autopsy revealed slightly retarded myelination and severe spongy change in the well‐myelinated areas of the brain. Analysis of this and the other 26 reported cases suggests that patients with nonketotic hyperglycinemia develop severe mental retardation, not seen in ketotic hyperglycinemia. Elevated glycine levels in the brain and cerebrospinal fluid appear to differentiate these two forms of hyperglycinemia better than the presence of ketosis or leukopenia, and high glycine levels apparently occur in the same areas as the spongy change. While both forms show defective glycine cleavage in the liver, defective glycine cleavage in the brain has been reported only in nonketotic hyperglycinemia.
Huntington's disease: Clinical and chemical effects of choline administration
Authors: Growdon John H. , Cohen Edith L. , Wurtman Richard J.
Published: January 2005

Choline (Ch) administration sequentially increases blood Ch, brain Ch, and brain acetylcholine (ACh) levels in the rat and might be useful in Huntington's disease, a brain disorder thought to be associated with deficient cholinergic tone. Ten patients with Huntington's disease took Ch orally (8 to 20 gm per day) after the extent of their disabilities had been documented by clinical examination, movies, and combined electromyogram‐accelerometer recordings. Choline levels in blood and cerebrospinal fluid increased markedly during treatment with Ch, affirming that oral Ch administration increases the amount of ACh precursor delivered to the brain. Although some of the patients exhibited transient improvement in speech, balance, and gait, treatment with Ch failed to bring about consistent or lasting improvement in any of the subjects.
Adenine arabinoside in the treatment of progressive multifocal leukoencephalopathy: Use of virus‐containing cells in the urine to assess response to therapy
Authors: Rand Kenneth H. , Johnson Kenneth P. , Rubinstein Lucien J. , Wolinsky Jerry S. , Penney John B. , Walker Duard L. , Padgett Billie L. , Merigan Thomas C.
Published: January 2005

Two patients with biopsy‐proved progressive multifocal leukoencephalopathy (PML) were treated with near‐maximal doses of adenine arabinoside (Ara‐A), 18.6 and 20 mg per kilogram of body weight per day for 14 days. In both patients, clinical progression of the disease was correlated with an increase in the size of low‐density lesions seen by computerized tomography. One of the patients was observed to excrete abnormal epithelial cells into the urine. These cells contained papovaviruslike particles, and JC virus was cultured from the urine sediment. The relative number of these abnormal cells declined during Ara‐A treatment. Both patients died shortly after the conclusion of therapy without a change in the progression of the central nervous system disease. Systemic administration of Ara‐A did not offer significant clinical benefit in the treatment of these 2 advanced cases of PML.
Comparison of ribosomal subunit proteins from normal human and Huntington's disease skin fibroblasts
Authors: Prashad Nagindra , Rosenberg Roger N.
Published: January 2005

The ribosomal subunit proteins from skin fibroblasts of normal persons and patients with Huntington's disease (HD) were isolated by KCI‐puromycin and by EDTA treatments and were compared by sodium dodecyl sulfate polyacrylamide gel electrophoresis. The data indicate that the ribosomal proteins are identical in control and HD fibroblasts.
Propranolol for the control of belligerent behavior following acute brain damage
Authors: Elliott Frank A.
Published: January 2005

The belligerence of 7 patients who had suffered an acute brain insult was effectively controlled by propranolol in doses of 60 to 320 mg per day. Of the 7 patients, 3 were treated in the acute stage after a stroke, a severe closed head injury, and a gunshot wound of the brain, respectively. A chronic postconcussion syndrome associated with chronic irritability was present in 2, and 2 were not chronically irritable but suffered from intermittent attacks of explosive rage in response to minor irritations. In all instances the belligerent behavior was controlled without inducing general sedation.
Free amino acid levels in amyotrophic lateral sclerosis
Authors: Patten Bernard M. , Harati Yadollah , Acosta Luis , Jung Shin‐Siung , Felmus Mark T.
Published: January 2005

To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in serum levels of tyrosine, total aromatic AA, and total basic AA. ALS patients also had statistically significant elevations in cerebrospinal fluid of total basic AA, lysine, essential AA, and leucine. The severity of ALS correlated inversely with acidic AA (glutamate and aspartate) and O‐phosphoserine in cerebrospinal fluid. Activity of ALS correlated directly with serum aspartate and cerebrospinal fluid alanine.

We conclude that subtle abnormalities of AA levels are present in ALS and that these are not due to age, sex, or disability. The pattern of distribution of AA levels differs from that in hepatic or renal disease and suggests defective membrane transport or poor cellular utilization of basic and essential AA in the central nervous system.
Effect of prednisolone on motor end‐plate fine structure: A morphometric study in hamsters
Authors: Chokroverty S. , Reyes M. G. , Chokroverty M. , Kaplan R.
Published: January 2005

The fine structure of quadriceps motor end‐plates in hamsters was analyzed quantitatively one, two, four, seven, and thirty‐two weeks following intraperitoneal injections of prednisolone. Except for transient increases in postsynaptic length and membrane profile concentration after prednisolone administration at dosages of 4 mg per kilogram of body weight for one week and 2 mg per kilogram for four weeks, mean values for various measurable profiles in the presynaptic and postsynaptic regions showed no significant differences between control and treated animals.
Juvenile neuroaxonal dystrophy: Clinical, electrophysiological, and neuropathological features
Authors: Dorfman Leslie J. , Pedley Timothy A. , Tharp Barry R. , Scheithauer Bernd W.
Published: January 2005

We describe 2 brothers with progressive myoclonus epilepsy that began in the second decade and was associated with cerebellar ataxia and intellectual deterioration. Electroencephalographic and cerebral evoked potential studies showed findings associated with myoclonus epilepsy. Neuropathological examination of 1 of the brothers, who died at age 23 years, revealed widespread changes of neuroaxonal dystrophy without pigment deposition in the basal ganglia. We propose the term juvenile neuroaxonal dystrophy (JNAD) to distinguish this condition on clinical grounds from infantile neuroaxonal dystrophy on the one hand, and on clinical and pathological grounds from Hallervorden‐Spatz disease on the other hand. JNAD, while exceedingly rare, must be considered in the differential diagnosis of the progressive myoclonus epilepsies.
Attenuated dynamic responses of primary endings of muscle spindles: A basis for depressed tendon responses in acrylamide neuropathy
Authors: Lowndes Herbert E. , Baker Thomas , Michelson Leslie P. , Vincent‐Ablazey Martha
Published: January 2005

Depressed or lost tendon reflexes commonly observed in patients with peripheral neuropathies may result partly from attenuation of the dynamic discharge from the primary endings of muscle spindles. This possibility was investigated in cats with an experimental neuropathy induced with acrylamide (30 mg/kg/day intramuscularly). Achilles tendon reflexes and the dynamic discharge from primary muscle spindles were evaluated after five or ten injections of acrylamide. After five injections the animals were moderately impaired neurologically, the Achilles tendon reflex was difficult to elicit in 2 of 5 animals, and the dynamic responses of primary endings of soleus muscle spindles to stretch were depressed. Following ten injections of acrylamide the cats were severely impaired neurologically; tendon responses were either absent or difficult to elicit, and the dynamic responses of their muscle spindles to stretch were reduced by 50% (p < 0.01). Additionally, the spindles responded to stretch with only 20 to 30% the normal number of afferent impulses. These data suggest that lost tendon responses in acrylamide neuropathy result in part from inadequate activation of motoneurons by spindle afferent discharge.
Transient cerebral dysfunction following chemotherapy for osteogenic sarcoma
Authors: Allen Jeffrey C. , Rosen Gerald
Published: January 2005

An unusual neurological syndrome occurred in 4 of 158 patients treated for osteogenic sarcoma with combination chemotherapy. There was an abrupt onset of focal cerebral deficits approximately ten days after chemotherapy with vincristine and high‐dose methotrexate plus citrovorum factor rescue. The syndrome was short lived and always occurred early in the course of treatment. Prolonged neurological deficits remained in 2 patients. When similar chemotherapy was reinstituted in the 4 patients, no further neurological complications ensued. Possible causes include a leukoencephalopathy related to methotrexate or an embolic cerebral vasculopathy related to necrotic tumor microemboli emanating from the lungs.
Huntington disease associated with Alzheimer disease
Authors: McIntosh Gerald C. , Jameson H. Douglas , Markesbery William R.
Published: January 2005

The clinicopathological study of a case of Huntington disease with early severe dementia is presented. The pathological findings were those of Huntington disease (atrophy with neuronal loss and astrocytosis in the neostriatum) and Alzheimer disease (neuronal loss with numerous senile plaques and neurofibrillary tangles in the neocortex). Ultrastructural study of neurofibrillary tangles showed that they were composed of twisted tubules similar to those found in Alzheimer disease.
Ultrastructure and biochemistry of sympathetic ganglia in idiopathic orthostatic hypotension
Authors: Petito Carol K. , Black Ira B.
Published: January 2005

The role of transsynaptic dysfunction in the pathogenesis of idiopathic orthostatic hypotension (IOH, or idiopathic autonomic insufficiency) was examined microsocopically and biochemically in autopsy specimens. Light microscopy of the sympathetic ganglia showed abnormalities in all 4 IOH patients, including focal phagocytosis of neurons, increased numbers of satellite cells, and perivascular lymphocytic infiltrates. Electron microscopy revealed proliferation and hypertrophy of satellite cells and abnormalities in the unmyelinated axons. In constrast, the spinal cord intermediolateral columns, containing the presynaptic neurons, were unremarkable in 1 patient, exhibited only mild gliosis in another, and showed neuron loss and fibrillary gliosis in 2 patients.

Postsynaptic dopamine‐β‐hydroxylase (DBH) activity was decreased at least fourfold (p < 0.02) in sympathetic ganglia of patients with IOH, while tyrosine hydroxylase (T‐OH) was normal. Ganglion choline acetyltransferase (ChAc) activity, an index of presynaptic function and integrity, was normal in the IOH group.

A number of our observations suggest that presynaptic disease is not an absolute requirement for adrenergic abnormalities in IOH. The intermediolateral columns of the spinal cord were histologically normal in 2 of the patients with IOH, and ultrastructural abnormalities in sympathetic ganglia were consistent with primary adrenergic degeneration. In addition, presynaptic ChAc activity was normal in IOH ganglia, whereas postynaptic DBH activity was depressed. Finally, postsynaptic T‐OH activity, which is regulated by transsynaptic mechanisms, was normal in IOH ganglia.
Computed tomography in late‐infantile metachromatic leukodystrophy
Authors: Buonanno Ferdinando S. , Ball Marshall R. , Laster D. Wayne , Moody Dixon M. , McLean William T.
Published: January 2005

Computed tomography of 2 patients with late‐infantile metachromatic leukodystrophy (MLD) demonstrated generalized white matter lucency and moderate ventricular enlargement. CAT scan findings in other white matter diseases likely to be confused clinically with MLD are sufficiently dissimilar to allow differentiation.
The prognosis of acute and subacute transverse myelopathy based on early signs and symptoms
Authors: Ropper Allan H. , Poskanzer David C.
Published: January 2005

Fifty‐two patients with acute and subacute transverse myelopathy (TM) were evaluated at the Massachusetts General Hospital between 1955 and 1975 and followed for 1 to 23 years (average, 5). Nineteen had symptoms of a recent acute infectious illness, 3 had cancer, and 1 had undergone a recent operation. There were four types of initial symptom. Twenty‐four patients had paresthesias at the onset of the illness, 18 had pain, usually interscapular, 7 had leg weakness, and 3 had urine retention. Prognosis depended on the nature of the onset of TM. An acute catastrophic onset was generally associated with back pain and led to a poor outcome in 7 and a good outcome in only 1 of 11 patients. A subacute progressive onset over several days to four weeks, generally with ascending paresthesias or leg weakness, was associated with a good outcome in 15 and fair outcome in 17 of 37 patients. Preceding febrile illness, treatment with corticosteroids, and the nature of CSF abnormalities had no effect on outcome. Multiple sclerosis evolved in 7 patients during the follow‐up period. Because of the frequency with which mass lesions were missed, the need for myelography in the diagnosis of TM is emphasized.

The distinguishing clinical characteristics of TM provide guidelines for diagnosis and prognosis.
Pharmacological studies of the pupils in familial amyloid polyneuropathy
Authors: Okajima Toru , Nagata Jinro , Hatamoto Kazuko , Kinoshita Yoshimi , Takaba Yoshio , Tokuomi Haruhiko
Published: January 2005

Pharmacological studies of the pupils of 7 patients with familial amyloid polyneuropathy (FAP) were performed in order to demonstrate the site of the lesion. Supersensitivity to 1.25% epinephrine was observed frequently, but not to 2.5% methacholine. The lesions of the sympathetic nervous system, judging from the epinephrine and tyramine tests, were regarded as preganglionic in 2 patients and postganglionic in 4. Among 3 patients reexamined six months later, 1 had developed signs of sympathetic postganglionic disturbance and another showed a peripheral parasympathetic disturbance associated with progress of a sympathetic preganglionic disturbance. From these results it was suggested that in FAP, sympathetic abnormalities involve the pupils more often than parasympathetic disturbances, and pharmacological studies can detect the autonomic dysfunction before it becomes clinically apparent.
Angiotensin converting enzyme in kainic acid–injected striata
Authors: Singh E. A. , McGeer E. G.
Published: January 2005

The activity of angiotensin converting enzyme is decreased in kainic acid–injected neostriata of rats, providing another biochemical similarity between this animal model and Huntington's chorea.
Peripheral nerve conduction in patients with a cervical rib and band
Authors: Gilliatt R. W. , Willison R. G. , Dietz V. , Williams I. R.
Published: January 2005

In 14 patients with wasting of the hand due to a cervical rib and band, motor and sensory conduction studies on the peripheral parts of the median and ulnar nerves were helpful in establishing the correct diagnosis. The median nerve findings excluded carpal tunnel syndrome even when the clinical pattern of wasting in the hand suggested this diagnosis. Preservation of conduction velocity in the ulnar nerve excluded ulnar entrapment at the elbow; the reduced amplitude of the ulnar sensory action potentials (SAPs) indicated that the lesion was distal to the dorsal root ganglia. In 3 patients with ulnar SAP amplitudes that were low but not clearly abnormal, the level of the lesion was confirmed by a reduced response to intradermal injection of histamine on the inner side of the forearm.
The cerebrospinal fluid in visna, a slow viral disease of sheep
Authors: Griffin Diane E. , Narayan Opendra , Bukowski Jack F. , Adams Robert J. , Cohen Steven R.
Published: January 2005

The temporal development of the CSF abnormalities was examined in visna, a chronic neurological disease caused by a persistent viral infection of sheep. Two types of changes were observed. During the first 30 to 40 days there was an intense mononuclear pleocytosis associated with high protein and proportionately elevated IgG. Later, there was a persistent slight pleocytosis associated with mildly elevated protein which had an increased proportion of IgG and antivisna antibody. These later cellular and protein changes are similar to those found in a number of chronic neurological diseases of man.
Relationship of lymphocyte invasion and survival of brain tumor patients
Authors: Brooks W. H. , Markesbery W. R. , Gupta G. D. , Roszman T. L.
Published: January 2005

The length of survival of 149 patients harboring primary brain tumors was retrospectively correlated with the presence and location of lymphocytic infiltration. Mononuclear invasion of malignant gliomas confined to the perivascular spaces was the only histological finding that correlated significantly with survival. Patients with malignant gliomas containing perivascular infiltration lived up to four months longer than those with no lymphocyte infiltration. The survival of patients with brain tumors who had lymphocyte invasion associated with areas of hemorrhage and necrosis beyond and separate from perivascular spaces was the same as for those with no lymphocyte involvement.
Acetylcholine receptor in myasthenia gravis: Increased affinity for α‐bungarotoxin
Authors: Elias Stanton B. , Appel Stanley H.
Published: January 2005

Studies of the binding of 125I‐labeled α‐bungarotoxin to myasthenic motor end‐plates have been interpreted as showing a decrease in the number of acetylcholine (ACh) receptors at these end‐plates. Equilibrium binding studies of 125I‐tagged α‐bungarotoxin to detergent‐extracted ACh receptors from normal and myasthenic intercostal muscle were carried out to determine whether the reduced toxin binding previously reported could be due to a reduced affinity of myasthenic receptors for α‐bungarotoxin rather than to a decreased number of receptors. Our results show increased rather than decreased affinity of myasthenic receptors for α‐bungarotoxin and also suggest that the number of ACh receptors is indeed reduced. The presence of a change in binding affinity, in addition to the reduced number of ACh receptors, suggests the presence of membrane changes that may contribute to the pathogenesis of myasthenia gravis.
Proteolytic enzyme activity of blood leukocytes and cerebrospinal fluid in multiple sclerosis
Authors: Cuzner M. Louise , Davison Alan N. , Rudge Peter
Published: January 2005

Upon stimulation by immune complexes, the polymorphonuclear (PMN) blood cell secretes lysosomal hydrolases, including neutral proteinase, which is concentrated in the PMN cell. Neutral and acid proteinase activity were increased and decreased, respectively, in the circulating white cells of patients with multiple sclerosis during an exacerbation of the disease, but there was no correlation with serum immune complex levels. Neutral proteolytic activity in the cellular fraction of the cerebrospinal fluid was also found to be elevated in acute multiple sclerosis, as monitored by digestion of myelin basic protein.
Rapid improvement in nerve conduction velocity following renal transplantation
Authors: Oh Shin J. , Clements Rex S. , Lee Young W. , Diethelm Arnold G.
Published: January 2005

In 12 patients with chronic renal failure who received kidney transplants from either cadavers (6) or related living donors (6), rapid improvement in median sensory and motor nerve conduction velocities (NCVs) was observed within a few days after transplantation. The postoperative improvement in median sensory NCV was found to bear a statistically significant negative correlation with creatinine and myo‐inositol concentrations. We suggest that metabolic phenomena are responsible for the rapid improvement in median sensory NCV following renal transplantation. The close relationship between myo‐inositol and the median sensory NCV following transplantation suggests that elevated plasma myo‐inositol concentrations may be related to nerve conduction abnormalities in uremic polyneuropathy.
Systemic lupus erythematosus, myasthenia gravis, and ehlers‐danlos syndrome
Authors: Branch C. E. , Swift T. R.
Published: January 2005

Clinical, electrophysiological, and laboratory data are reported on a 21‐year‐old black woman with systemic lupus erythematosus, myasthenia gravis, and Ehlers‐Danlos syndrome. The first two diseases are related by similar pathogenetic mechanisms. To our knowledge, this is the first patient reported with this unusual combination of rare diseases.
The long‐term prognosis in untreated cerebral aneurysms: II. Late morbidity and mortality
Authors: Winn H. Richard , Richardson Alan E. , O'Brien William , Jane John A.
Published: January 2005

Three hundred sixty‐four patients who suffered a subarachnoid hemorrhage from an aneurysm at either the anterior (ACA) or posterior (PCA) communicating artery location and who were not surgically treated have been followed for up to 21 years in order to study the late morbidity and mortality. For patients surviving six months: (1) little improvement in morbidity was noted during the many years of subsequent follow‐up; (2) in general, ACA patients fared better than PCA patients; (3) the neurological state on original admission was strongly correlated with the degree of morbidity; (4) other factors measured at time of original hemorrhage which adversely affected morbidity included the level of blood pressure or the presence of clot or spasm; (5) if deaths from late hemorrhage are excluded, cardiovascular causes account for the majority of subsequent deaths; and (6) the risk of dying from all causes in increased when compared to a population matched by age and sex.
Experimental allergic encephalomyelitis in cobra venom factor—treated and C4‐deficient guinea pigs
Authors: Morariu Mircea A. , Dalmasso Agustin P.
Published: January 2005

Experimental allergic encephalomyelitis (EAE) was studied in guinea pigs with an inherited deficiency of the fourth component of complement (C4) and in guinea pigs injected with cobra venom factor to deplete the third component and late‐acting components of complement. EAE was elicited by immunization with homologous spinal cord or purified basic protein. Administration of cobra factor after the injection of encephalitogenic emulsion delayed the onset and reduced the intensity of the clinical manifestations of EAE. In addition, cobra factor markedly reduced mortality during the sixty days of observation. However, pathological changes of perivascular infiltration and demyelination were similar in cobra factor–treated and untreated animals. Clinical signs of EAE and mortality in C4‐deficient guinea pigs were no different from those in normocomplementemic controls. Thus, although activation of the classic complement pathway does not appear to be involved in the production of EAE in guinea pigs, our results suggest a possible role of the alternative complement pathway in the pathogenesis of EAE.
Endogenous pain control mechanisms: Review and hypothesis
Authors: Basbaum Allan I. , Fields Howard L.
Published: January 2005

The anatomy, physiology, and pharmacology of an intrinsic neural network that monitors and modulates the activity of pain‐transmitting neurons is reviewed. This system can be activated by opiate administration or by electrical stimulation of discrete brainstem sites. Evidence is presented that its pain‐suppressing action is mediated in part by endogenous opiatelike compounds (endorphins).

This pain suppression system is organized at three levels of the neuraxis: midbrain, medulla, and spinal cord. Activation of neurons in the midbrain periaqueductal gray matter (by electrical stimulation, opiates, and possibly psychological factors) excites neurons of the rostral medulla, some of which contain serotonin. The medullary neurons, in turn, project to and specifically inhibit the firing of trigeminal and spinal pain‐transmission neurons. As part of a negative feedback loop, the output of the pain transmission neurons, i.e., pain itself, is an important factor in activating the pain‐suppression system. A neural model which incorporates the experimental findings is proposed, and the clinical implications of the model are discussed.
The role of judges in life/death decisions for the neurologically impaired
Authors: Beresford H. Richard
Published: January 2005

The Massachusetts supreme court has recently ruled that decisions about withholding care from hopelessly ill, legally incapacitated patients must be made by judges. It clearly rejected the view that families and attending physicians should be empowered to make such decisions. In this respect, the ruling contrasts with that of the Quinlan case and highlights the issue of whether judges or physicians and families are better able to make medically and morally sound decisions respecting this class of patients.
Downbeat nystagmus due to anticonvulsant toxicity
Authors: Alpert Jack N.
Published: January 2005

Isolated downbeat nystagmus was observed in 2 patients on multiple anticonvulsant regimens. The nystagmus disappeared when phenytoin dosage was reduced. Electrooculographic analysis revealed impaired downward tracking, supporting the concept of “pursuit” nystagmus.
Impaired refractory periods of peripheral sensory nerves in multiple sclerosis
Authors: Hopf Hanns C. , Eysholdt Magrit
Published: January 2005

Maximum conduction velocity and relative refractory period (RRP) of median nerve sensory fibers were studied in 36 patients diagnosed as having multiple sclerosis (MS) and in 31 controls. Maximum conduction velocity did not differ in the two groups, but the RRP was significantly prolonged in MS patients. Increased RRP is observed mainly when peripheral nerve myelin is abnormal or damaged. Our findings support the assumption that peripheral nerve myelinated fibers are usually involved in MS.
Clinical and electroencephalographic correlates of the multiple independent spike foci pattern in children
Authors: Blume W. T.
Published: January 2005

The multiple independent spike foci (MISF) pattern on electroencephalograms occurred in 4.2% of 1,500 recordings obtained from patients between the ages of 6 months and 15 years. Among the 63 patients with MISF, 53 (84%) had a seizure disorder and 48 (76%) had generalized motor seizures. Half the study group had daily seizures and half had more than one type of attack. Sixty‐eight percent of MISF patients were intellectually subnormal. The intellectual status varied according to age at seizure onset, findings on clinical neurological examination, incidence of spikes in the recording, number of spike foci, and presence of excessive delta activity.
Actin filaments in human skin fibroblasts are similar in normal persons and patients with Huntington's disease
Authors: Geary Laurane E. , Lazarides Elias , Goetz Ingeburg , Roberts Eugene
Published: January 2005

Actin filaments in skin fibroblasts from patients with Huntington's disease (HD) were examined using immunofluorescent methods. Actin filaments were seen along the axis of cell elongation (stress or sheath filaments) as well as in areas of membrane ruffling (lattice filaments). In some cases, filaments appeared to radiate from foci within the cell. Bundles of these filaments radiated in various directions at different depths within a cell. These structures are similar, in cells from both normal individuals and HD patients, to actin filaments observed in other cell types. The higher‐than‐normal confluent densities achieved in culture by fibroblasts from patients with Huntington's chorea do not involve alterations in the ultrastructure of actin filaments.