bluuebunny/crossref_metadata_2025 huggingface dataset:
Das Zustandsdiagramm Calcium/Quecksilber
Authors: Eilert A.
Published: January 2005

Die thermische Analyse ergibt im Einklang mit entsprechenden mikrophotographischen Aufnahmen bzw. ergänzt durch diese, daß aus Ca‐Amalgamen mit mehr als 2,15 Gew.‐% Ca primär die Kristallart CaHg3, sich ausscheidet. Diese reagiert bei 265° mit einer Schmelze von 2,15% Ca‐Gehalt unter Bildung der Kristallart CaHg5. Die Reaktion verläuft vollständig. Die Kristallart CaHg5 wird auch durch direkte Analyse gutausgebildeter einzelner Kristalle bestätigt.

Bei + 84° reagieren die CaHg5‐Kristalle mit einer Schmelze von 0,8% Ca‐Gehalt. Es entsteht hierdurch die Kristallart CaHg10. Diese Reaktion verläuft äußerst langsam und bei gewöhnlicher Abkühlung nicht vollständig.
Über die Ausdehnung bei der Erstarrung von eisenhaltigem Aluminium
Authors: Masing G. , Dahl O.
Published: January 2005

Es wird gezeigt, daß die Ausdehnung der Eisen‐Aluminium‐legierungen bei der Erstarrung auf eine Gasentbindung zurückzuführen ist. Die Zusammensetzung der hierbei entstehenden Warzen und ihre Bildungsbedingungen entsprechen dem Zustandsdiagramm der Eisen‐Aluminiumlegierungen; das Al‐reiche Eutektikum liegt bei etwa 2,2%, Fe.
Über zwei Beispiele rückläufiger Umwandlungskurven in anisotropen binären Systemen
Authors: Wever Franz
Published: January 2005

Die ermittelten thermischen Befunde über den Verlauf der A3‐ und A4‐Umwandlung in den Zweistoffsystemen Eisen‐Silicium und Eisen‐Zinn legen eine Abgrenzung des Zustandsfeldes der γ‐Phase durch einen geschlossenen Kurvenzug in hohem Maße nahe. Dabei sind jedoch zwei Möglichkeiten grundsätzlich zu unterscheiden: auf der Grenzlinie kann ein nonvarianter Tripelpunkt angenommen werden, in dem nebeneinander der Δ‐, der γ‐und der α‐Mischkristall im Gleichgewicht sind; das γ‐Feld kann andererseits auch im Sinne der TAMMANN' schen Entwicklung durch einen stetigen Kurvenzug ohne Tripelpunkt abgeschlossen werden. Die erstere Annahme würde in Verbindung mit den Feststellungen WESTGRENS über die strukturelle Gleichheit von Δ‐und α‐Phase des reinen Eisens auf eine polymorphe Umwandlung zwischen Δ‐und α‐Mischkristall ohne gleichzeitige Änderung der Kristallstruktur führen; sie steht damit im Gegensatz zu den gewohnten Vorstellungen über das Wesen des Polymorphismus.
Zur Analyse der Gemische von Brom‐Sauerstoff‐Wasserstoffverbindungen
Authors: Pollak Friedrich
Published: January 2005

Die vollständige Analyse von Gemischen der Bromsauerstoffsäuren mit ihren Salzen, Bromid und elementarem Brom erfordert die Bestimmung von vier Ionenarten, nämlich des Bromations, Hypobromitions, Bromidions und Wasserstoffions. Die Analyse erfaßt diese Ionen nicht nur, soweit sie als solche schon vorliegen (aktuelle Ionen), sondern auch die welche bei der Verschiebung bestehender Gleichgewichte durch die Reagentien erst entstehen (potentielle Ionen).
Studien über höhere Silberoxyde. II
Authors: Jirsa Franz
Published: January 2005

Die Bestimmung des Sauerstoffs im Oxyde Ag2O2 war bis jetzt nur durch die genaue gasometrische Methode möglich. Es wurde eine rasche und genaue Titrationsmethode mit Kaliumjodid gefunden, welche die Ergebnisse auf 0,01% genau angibt. Gleichzeitig wurde die Reaktion des Ag2O2 mit Hydrazin studiert und gefunden, daß beide Stoffe glatt nach dem Schema

reagieren. Durch dieses Schema ist die Möglichkeit einer Bestimmung des Totalsauerstoffs in Ag2O2 entweder durch die acidimetrische Methode oder durch eine volumetrische Methode gegeben. Die Zersetzung mit Hydrazin verläuft im Vergleiche zu der Auflösung durch Säure bereits auf kaltem Wege.
Dichten und Dampfdrucke von wäßrigen Ammoniaklösungen und von flüssigem Stickstofftetroxyd für das Temperatur‐gebiet 0° bis 60°
Authors: Mittasch A. , Kuss E. , Schlueter H.
Published: January 2005

Es wurde eine Methode zur gleichzeitigen Bestimmung von Dichten und Dampfdrucken, auch für höhere Drucke, ausgearbeitet und die Einrichtung von 2 Arten von Tensionspyknometern beschrieben, eines Tensionspyknometers mit Hg‐Manometer und eines solchen mit Glasfedermanometer.
Über Echte und ψ‐Haloidsalze
Authors: Hantzsch A. , Carlsohn H.
Published: January 2005

1. In Anlehnung an frühere Untersuchungen an Säuren und Salzen gibt es auch bei den Halogeniden echte und ψ‐Salze.
Elektrizitätsleitung in Schwermetallsulfiden
Authors: Tubandt C. , Haedicke Max
Published: January 2005

Es wurde für eine Reihe von Schwermetallsulfiden, deren Verhalten es ungewiß erscheinen ließ, ob man es bei ihnen mit rein metallischen oder, wenigstens teilweise, elektrolytischen Leitern zu tun hat, der Beweis erbracht, dsß es sich unzweifelhaft um rein metallische Leiter handelt. Dies gilt besonders für das Antimontrisulfid, Molybdändisulfid, Zinnsulfid und Bleisulfid, es gilt aber auch für das durchsichtige, bei niederer Temperatur isolierende Cadmiumsulfid. Rotes Antimontrisulfid und Arsensulfid bleiben in reinem Zustande bis zum Umwandlungs‐ bzw. Schmelzpunkt Isolatoren. Schwefelsilber und Kupfersulfür sind unter den Schwermetallsulfiden die einzigen elektrolytischen bzw. (in der β‐Modifikation) gemischten Leiter.
Über den Nachweis des Thoriums in den Glühfäden fertiger Vakuumlampen
Authors: Selényi P.
Published: January 2005

Die Elektronenemission des Glühfadens einer jeden Vakuumlampe läßt sich leicht mit Hilfe einer äußeren Anode (Stanniolhülle um den Kolben), die man durch ein Strommeßinstrument mit dem positiven Lampenpol verbindet, messen.

Der Elektronenstrom, der eine Stärke von mehreren Milliampere erreichen kann, fließt dann durch das infolge Erwärmung leitend gewordene Kolbenglas und bringt die äquivalente Menge Natrium in die Lampe hinein.

Wird die Lampe in diesem Zustande mit 30–40% ihrer Nennspannung gebrannt, so zeigt sich der bezeichnende Unterschied zwischen thorhaltigem und nicht thorhaltigem Draht: erster emittiert noch stark, der letztere kaum merkbar.

In der oben beschriebenen Anordnung kann eine jede Vakuumlampe als Gleichrichter verwendet werden.
Über Gleichgewichte zwischen geschmolzenen Metallen und Salzen
Authors: Tubandt C. , Münzing Ernst
Published: January 2005

Es wurden die Gleichgewichte (Pb, Ag, PbCl2, AgCl) und (Cu, Ag, CuCl, AgCl) im Schmelzfluß untersucht.

Das Gleichgewicht (Pb, Ag, PbCl2, Ag Cl) ist von beiden Seiten her erreichbar; es ist merklich abhängig von der Temperatur; Zusatz von Natriumchlorid oder Kaliumchlorid führt keine merkliche Verschiebung des Gleichgewichts herbei.

Beide Gleichgewichte sind durch die von R. Lorenz im Anschluß an die van der Waals‐Van Laar'sche Theorie der Dampfspannungen entwickelte Massenwirkungsformel für kondensierte System durch alle Konzentrationen gut darstellbar.
Gyral development of the human brain
Authors: Chi Je G , Dooling Elizabeth C , Gilles Floyd H.
Published: January 2005

To determine the development of the convolutional pattern of the cerebral hemispheres, we have examined multiple gross photographs of 507 brains and serial sections of 207 brains from infants of 10 to 44 weeks' gestational age. The sequential developmental changes of the individual fissures, sulci, and gyri of the cerebral hemispheres throughout the gestational period are tabulated. Schematic drawings made from enlarged photographs show the major changes associated with each month of the second and third trimesters.

The brains of twins between 19 and 32 weeks' gestational age show a delay of approximately two to three weeks in the development of convolutional markings compared with the brains of nontwins. There is no significant difference between male and female brains.

We found left‐right asymmetries of the transverse temporal gyri, sylvian fissures, and planum temporale. In general, the right cerebral hemisphere shows gyral complexity earlier than the left. Our findings allow speculation about the significance of left‐right asymmetry of the brain as it affects speech and language development.
Neurogenic muscle atrophy and osteomalacia in adult fanconi syndrome
Authors: Mallette L. E. , Patten B. M.
Published: January 2005

A 41‐year‐old mechanic in whom severe proximal muscle weakness and atrophy, hyperactive tendon reflexes, and marked bone pain developed was found to have hypophosphatemic osteomalacia from idiopathic adult‐onset Fanconi syndrome. Concentrations of calcium, 25‐hydroxyvitamin D, and parathyroid hormone in venous blood were normal. A muscle biopsy specimen studied histochemically showed evidence of neurogenic atrophy without myopathic changes. Specifically, there were small groups of small angular type I and type II fibers that appeared excessively dark with oxidative enzyme stains. Administration of neutral phosphate by mouth restored muscle strength nearly to normal within four months.
Chronic relapsing experimental allergic encephalomyelitis: An experimental model of multiple sclerosis
Authors: Wiśniewski H. M. , Keith A. B.
Published: January 2005

A chronic relapsing form of experimental allergic encephalomyelitis was induced by single sensitization of immature strain 13 and Hartley guinea pigs. Clinically, the disease was characterized by remissions and relapses. Recent and old demyelinating plaques, which are the two characteristic features of multiple sclerosis lesions, were also present. The model described opens new avenues for the study of immunological mechanisms governing remissions and exacerbations in multiple sclerosis and offers new opportunities for evaluating the efficacy of drugs for its treatment.
The complications of high‐dose corticosteroid therapy in neurosurgical patients: A prospective study
Authors: Marshall Lawrence F. , King Joseph , Langfitt Thomas W.
Published: January 2005

We studied prospectively the complications of high‐dose corticosteroid administration in 121 consecutive neurosurgical patients. Dexamethasone was begun in a daily dosage of 16 mg in 36 patients, 40 mg in 43 patients, and 80 mg in 42 patients, with an antacid, and was appropriately tapered.

There were three major complications (incidence, 2.4%), with 1 death definitely attributable to corticosteroid therapy (0.8%). Two of the major complications occurred in patients with previous or concurrent upper gastrointestinal disease. Nineteen patients who were maintained on long‐term (mean 206 – 56 days) daily doses of 40 mg or more for the treatment of malignant brain tumors remained free of complications.

There was no evidence that increased intracranial pressure, diminished level of consciousness, or higher dosage schedules increased the risk. High‐dose dexamethasone therapy is safe in neurosurgical patients. One must use caution, however, when the history suggests gastrointestinal erosive disease.
Anterior interosseous nerve syndrome following cutdown catheterization
Authors: Finelli Pasquale F.
Published: January 2005

Traumatic neuropathy following intravenous catheterization by means of a cutdown at the antecubital fossa resulted in inability to flex the tip of the index finger and thumb. The impaired pincer grasp that results causes considerable handicap when the dominant hand is involved.
Regional cerebral blood flow pattern in subcortical propagation of focal seizures in newborn monkeys
Authors: Hosokawa Shinichi , Yamashita Yoriaki , Ueno Hideo , Caveness William F.
Published: January 2005

The propagation of penicillin‐induced focal seizures from the face‐hand area of the right precentral gyrus was determined in 15 newborn monkeys by the carbon 14 antipyrine method for regional cerebral blood flow (rCBF). With the electroencephalographic spikes confined to the right hemisphere, the rCBF increase remained in the right sensorimotor cortex. With EEG spikes on the right and concomitant muscular contractions in the left face, the rCBF incrase extended to the right putamen, globus pallidus, and, in 1 instance, the substantia nigra. With EEG spikes appearing bilaterally but the clinical expression remaining in the left face, the rCBF increase extended more fully into the right substantia nigra, VL, VPL, VPM of the thalamus, pontine nucleus, and the left simple and paramedian lobules of the cerebellu. With bilateral EEG spikes and the muscular contractions progressing from the face to the left hand, the rCBF increase extended into the right caudate, subthalamic nucleus, red nucleus, CM of the thalamus, and the left cerebellar nuclei (n. dentatus, n. interpositus).
Whipple's disease with predominantly neuroophthalmic manifestations
Authors: Finelli Pasquale F. , McEntee William J. , Lessell Simmons , Morgan Thomas F. , Copetto James
Published: January 2005

Supranuclear paralysis of gaze was a prominent manifestation of Whipple's disease in 2 patients with dementia, 1 of whom also had bilateral uveitis. Because of the absence of gastrointestinal problems in these patients, correct diagnosis was delayed. Whipple's disease is a treatable disorder and should be considered in patients with otherwise unexplained supranuclear gaze paralysis and uveitis.
Glucocorticoid preservation of motor nerve function during early degeneration
Authors: Hall Edward D. , Baker Thomas , Riker Walter F.
Published: January 2005

In cats, an intensive short‐term triamcinolone regimen prior to section of the soleus motor nerve significantly reduced the excitability loss that otherwise would have occurred over a 48‐hour period in its axons and terminals. The triamcinolone effect was manifested chiefly by striking preservation and enhancement of the capacity of soleus motoneurons to generate posttetanic repetition (PTR). This triamcinolone effect was also reflected by the greatly augmented posttetanic potentiation (PTP) obligatory to the neurogenic PTR. These principal effects were supported by an improvement in the capacity of the 48‐hour denervated system to maintain repetitive transmission and to recover from high‐frequency nerve stimulation. Overall, these results indicate that glucocorticoids can preserve excitability in degenerating motoneurons by a direct action. These findings provide a new basis for understanding clinical improvements that may be seen with intensive glucocorticoid therapy in certain neurological disorders.
Depression of synaptic transmission by diphenylhydantoin
Authors: Yaari Yoel , Pincus Jonathan H. , Argov Zohar
Published: January 2005

Diphenylhydantoin (phenytoin, DPH) depresses synaptic transmission at the frog neuromuscular synapse by presynaptic and postsynaptic mechanisms. In normal Ringer's solution the amplitude of the neurally evoked end‐plate potentials and their quantal content are reduced. Somewhat paradoxically, miniature end‐plate potential (mepp) frequency is increased by the drug. These effects could result if DPH blocked both calcium transport at the axonal membrane and intracellular calcium sequestration. Mepp amplitude is reduced, and DPH also induces nerve conduction block at high rates of stimulation. The relevance of these effects to the anticonvulsive activity of DPH is discussed.
Alpha‐pattern coma: 24 cases with 9 survivors
Authors: Grindal Alan B. , Suter Cary , Martinez A. Julio
Published: January 2005

Alpha‐pattern coma denotes the association of a comatose state with an electroencephalogram consisting predominately of alpha‐frequency activity. Over the past three years we have studied 24 such cases: 14 following cardiopulmonary arrest, 6 following respiratory arrest, and 3 following brainstem infarction. Of the 9 patients who survived, only 1 had a significant neurological deficit. It was found that: (1) alpha‐pattern coma can result from a variety of neurological insults; (2) a significant proportion of patients survive, often with little or no deficit: (3) clinical evidence of intact brainstem function implies a favorable outcome; (4) the electroencephalogram was similar in survivors and nonsurvivors and did not differentiate diffuse cerebral dysfunction from focal brainstem disease; and (5) neuropathological studies demonstrated diffuse cortical as well as brainstem changes.
False diagnosis of orbital apex tumor by CAT scan in thyroid eye disease
Authors: Susac John O. , Martins Albert N. , Robinson Bernard , Corrigan Domenic F.
Published: January 2005

Computerized transaxial tomography is now the procedure of choice in diagnosing exophthalmos. Caution must be exercised, however, in interpreting the results. The patient reported here had a swollen inferior rectus muscle that simulated an orbital apex tumor on CAT scan.
Isolated myopathic involvement of the diaphragmatic musculature in a neonate
Authors: Bergen Barry J. , Sangalang Virgilio E. , Aterman Kurt
Published: January 2005

A myopathy restricted to the diaphragm was found in an infant who died at 3 months of age of respiratory failure due immobility of the diaphragm. Grossly, the diaphragm was thin and translucent. On microscopical examination few muscle bundles were seen. Most muscle fibers were small, and numerous necrotic fibers with large sarcolemmal nuclei, focal cytoplasmic basophilia, and hyaline or homogeneous eosinophilic degeneration in the center of the fibers were seen.
Ultrastructural lesions of muscle and immunofluorescent deposits in vessels in Reye's syndrome: A preliminary report of serial muscle biopsies
Authors: Hanson P. A. , Urizar R. E.
Published: January 2005

Thirteen sequential percutaneous skeletal muscle biopsy specimens from 4 patients with Reye's syndrome were studied ultrastructurally and by direct immunofluorescence. Prominent generalized intermyofibrillar edema, mitochondrial disruption, and swelling of vascular endothelium were demonstrated in 2 patients. In all 4 patients there were granular deposits of immunoglobulins G and M in intramuscular vessels, presumably representing antigen‐antibody complexes. We postulate that these changes may be pathogenetically important in this syndrome and suggest that muscle biopsy tissue be utilized for further investigation of Reye's syndrome.
Giant axonal neuropathy: A childhood disorder of microfilaments
Authors: Koch Thomas , Schultz Paul , Williams Roger , Lampert Peter
Published: January 2005

A sural nerve biopsy was performed on an 8‐year‐old boy with a chronic, slowly progressive polyneuropathy. Light and electron microscopy as well as teased nerve‐fiber preparations demonstrated numerous giant axons filled with closely packed neurofilaments. Both myelinated and unmyelinated fibers were involved. Segmental demyelination, remyelination, and onion‐bulb formation by multiple Schwann cell processes were observed, suggesting recurrent Schwann cell dysfunction. Abundant aggregates of cytoplasmic microfilaments occurred in Schwann cells, endothelial cells, perineurial cells, endoneurial fibroblasts, and endomysial fibroblasts. These findings support the proposal that giant axonal neuropathy is a generalized disorder of cytoplasmic microfilaments and that segmental demyelination occurs concomitantly with axonal and Schwann cell disease. The pathogenesis of this rare disorder is discussed with reference to experimental toxic neuropathies.
An animal model for the production of intracranial pressure plateau waves
Authors: Wald Alvin , Hochwald Gerald M.
Published: January 2005

This communication describes a reproducible model for the generation of intracranial pressure plateau waves by obstructing the spinal cord central canal of cats with experimentally induced hydrocephalus.
Cerebral blood flow and metabolism in chronically hyperammonemic rats: Effect of an acute ammonia challenge
Authors: Gjedde Albert , Lockwood Alan H. , Duffy Thomas E. , Plum Fred
Published: January 2005

The effects of chronic hyperammonemia on cerebral metabolism were studied in rats four and eight weeks after the construction of a portacaval shunt. Compared to sham‐operated controls, shunted animals had increased arterial concentrations of ammonia and glutamine and decreased glutamate. Cerebral blood flow, measured by xenon 133 washout in animals lightly anesthetized with nitrous oxide, increased from a control of 91 ± 5 (mean ± SEM) to 139 ± 20 ml per 100 gm tissue per minute after shunting for eight weeks; however, the cerebral metabolic rate for oxygen was not different from control four or eight weeks after the shunting procedure. Following intraperitoneal administration of a small ammonium acetate load (2.6 mmol/kg), eight‐week portacaval animals consistently underwent a fall in cerebral blood flow and cerebral oxygen consumption and developed high‐voltage slow waves in the electroencephalogram. Glutamine was produced by the brains of all groups of animals; the cerebral metabolic rate for glutamine was greater than control in eight‐week portacaval rats, the only animals to show a net uptake of ammonia, along with nonspecific effects of chronic portal‐systemic shunting, may lead to uncoupling of cerebral blood flow and oxidative metabolism.
Benign cerebellar hemorrhages
Authors: Heiman Terry D. , Satya‐Murti Saty
Published: January 2005

Two patients with cerebellar hemorrhage and a benign outcome stress the potential for spontaneous resolution and clinical recovery in some cases of this disorder. In each patient, resolution of the hemorrhage was noted on the computed tomographic scan. Computed tomography may detect a cerebellar hemorrhage even though on clinical grounds a brainstem localization is favored.
Prolonged neonatal myasthenia gravis: Electrophysiological studies
Authors: Branch Clinton E. , Swift Thomas R. , Dyken Paul R.
Published: January 2005

A female infant with neonatal myasthenia gravis remained weak for an excessively long period compared to the usual situation in this disease process. At 71 days of age, electrophysiological studies revealed evidence of a defect in neuromuscular transmission that improved with intravenous Tensilon therapy. Repeat study at 183 days of age was normal. This case represents an unusually long time for an affected infant to have neonatal myasthenia gravis. Electrophysiological studies are of value in the diagnosis and management of patients with neonatal myasthenia gravis.
Papillophlebitis: Benign retinopathy resembling papilledema or papillitis
Authors: Ellenberger Carl , Messner Kenneth H.
Published: January 2005

Three young adult patients are described to illustrate an uncommon syndrome, papillophlebitis. This entity, characterized by mild but protracted monocular visual impairment, edema of the optic disc, retinal venous engorgement, and perivenous retinal hemorrhages, resembles atherosclerotic occlusion of the central retinal vein in older patients. In younger individuals it is benign and self limited, but may be incorrectly diagnosed as optic neuritis or papilledema. These alternatives can usually be excluded if tests of visual function do not indicate an optic nerve conduction defect and if the clinical presentation and examination of the opposite ocular fundus do not disclose evidence of increased intracranial pressure. Because resolution usually occurs spontaneously, immediate neuroroenotenographic tests may be inappropriate. Effective treatment has not been established; local instillation of corticosteroids may hasten recovery.
Sagittal sinus thrombosis as a complication of regional enteritis
Authors: Sigsbee Bruce , Rottenberg David A.
Published: January 2005

A young woman with regional enteritis and a history of recurrent thrombophlebitis and pulmonary embolism developed sagittal sinus thrombosis during an acute exacerbation of her illness. Repeated attempts to document a hypercoagulable state were unsuccessful.
Late components of motor unit potentials in a patient with myoglobinuria
Authors: Pickett Jackson B.
Published: January 2005

A 45‐year‐old woman had severe weakness, myoglobinuria, and elevated muscle enzyme levels in the serum after a flulike illness. Electromyography some four weeks later showed motor unit potentials with late components; these remained present on subsequent electromyographic examinations performed at approximately monthly intervals for the following year. The persistence of motor unit potentials with late components is taken to imply that the late components were caused either by sprouts that failed to undergo myelination or, more likely, by ectopically innervated muscle fibers.
Treatment of subarachnoid hemorrhage from ruptured intracranial aneurysm with tranexamic acid: A double‐blind clinical trial
Authors: Chandra Benjamin
Published: January 2005

A double‐blind clinical trial of tranexamic acid was carried out on 39 patients with fresh subarachnoid hemorrhage from a ruptured aneurysm. Twenty patients received tranexamic acid, 6 gm daily for 14 to 21 days, while 19 patients received conventional therapy of bedrest and dexamethasone when cerebral edema developed, plus isotonic saline. Rebleeding and mortality were reduced by one‐fourth and one‐fifth, respectively (p < 0.001). No side‐effects were observed. Tranexamic acid is valuable in the treatment of subarachnoid hemorrhage caused by ruptured intracranial aneurysms.
Determination of immunoglobulin content of CSF based on light chain characteristics
Authors: Eickhoff K. , Heipertz R.
Published: January 2005

The immunoglobulin light chain (types kappa and lambda) content of normal cerebrospinal fluid is similar to that of normal serum. In inflammatory diseases of the central nervous system a shift in the k/1 ratio in comparison to serum values, usually a relative increase of Igk, can be observed. This increase of the k/1 ratio, though not specific for any disease, is most commonly found in multiple sclerosis. There is no correlation between the IgG content and the k/1 ratio. The methods described here measure bound and free light chains simultaneously. The calculations show that free light chains are present in both normal and inflammatory CSF and that they appear to be of polyclonal origin. The detection of light chain abnormalities in CSF can be taken as an indicator of endogenous immunoglobulin production in the CNS and is of significance for the diagnosis of inflammatory CNS processes, especially when other signs of endogenous immunoglobulin production are absent.
Kearns‐Sayre Syndrome with hypoparathyroidism
Authors: Horwitz Samuel J. , Roessmann Uros
Published: January 2005

The history, clinical findings, and postmortem exainination of a patient with Kearns‐Sayre syndrome are presented. In addition to the usual symptoms, the patient also had hypoparathyroidism. There is increasing evidence that the syndrome is frequently associated with involvement of the endocrine system. Central nervous system involvement consists of a spongy encephalopathy.
Anatomical correlates of electrical and behavioral events related to amygdaloid kindling
Authors: Engel Jerome , Wolfson Leslie , Brown Lucy
Published: January 2005

Anatomical structures demonstrating increased glucose uptake during the various stages of amygdaloid kindling in rats were identified by the 14C‐2‐deoxyglucose (DG) autoradiographic technique. Partial (stages 1 and 2) seizures were correlated with increased DG uptake in the ipsilateral amygdala and its direct projection fields. The appearance of generalized motor (stages 3, 4, and 5) seizures was accompanied by less limbic involvement and recruitment of a bilateral system including substantia nigra, specific and nonspecific thalamic nuclei, globus pallidus, and neocortex. Increased hippocampal DG uptake was correlated with prolonged amygdaloid afterdischarge duration but not with the behavioral seizure stage. This study does not reveal which of these structures are responsible for the observed behavioral and electrical events and which are activated by them. It does suggest, however, that three discrete anatomical systems underlie the generation of partial seizures, generalized motor seizures, and local afterdischarge.
Further characterization of bound measles‐specific IgG eluted from SSPE brains
Authors: Mehta P. D. , Kane A. , Thormar H.
Published: January 2005

Tissue residues from 3 SSPE brains extracted at neutral pH were thoroughly washed with phosphate‐buffered saline and further extracted at acidic pH. The low‐pH extracts had significant measles antibody titers, and their measles‐specific IgG content ranged from 40 to 60% of total IgG. Agarose‐gel electrophoretic analysis of the extracts showed oligoclonal IgG bands, most of which disappeared after suitable absorption with measles virus, demonstrating the presence of restricted measles antibody population. Ouchterlony analysis showed a reaction of identity between bound IgG and IgG extracted at neutral pH. Thus IgG extracted at low and neutral pHs is remarkably similar.
Cytoplasmic filaments in intracerebral cortical vessels
Authors: Nag Sukriti , Robertson David M. , Dinsdale Henry B.
Published: January 2005

Actin filaments measuring 5 nm in diameter are present in the endothelium of systemic vessels and presumably have a contractile function that may be related to vascular permeability. Little attention has been directed to the presence of similar cytoplasmic filaments in the endothelium of cerebral vessels. This study was undertaken to determine whether filaments are present in the endothelial cells of intracerebral cortical vessels of rats and humans. Ultrastructural studies revealed two populations of endothelial cytoplasmic filaments 5 nm and 10 nm in diameter, respectively. The 5‐nm filaments were abundant in the endothelium of penetrating cortical arterioles, being grouped near the cytoplasmic margins in proximity to cell junctions and along the abluminal side of the endothelial cells. The filaments were scant in capillaries and venules.
Transaqueous diffusion of acetylcholine to denervated iris sphincter muscle: A mechanism for the tonic pupil syndrome (adie syndrome)
Authors: Wirtschafter Jonathan D. , Volk Charles R. , Sawchuk Ronald J.
Published: January 2005

The accepted hypothesis for the pathophysiology of tonic pupil syndrome (Adie syndrome) was rexamined in light of recent developments concerning deneravation supersensitivity of cholinergically innervated smooth muscle. Kinetic analysis suggests that enzymatic hydrolysis is unimportant relative to convective diffusion in the turnover of acetylcholine in the aqueous humor. We postulate that the greater response to near stimuli than to light stimuli and the delay in iris sphincter contraction and relaxation can be explained by release of acetylcholine from the neuromuscular junction of the ciliary muscle followed by transaqueous diffusion to receptor sites on denervated, supersensitive iris sphincter muscle.
Pseudoxanthoma elasticum: A review of neurological complications
Authors: Iqbal Aleem , Alter Milton , Lee Seungho H.
Published: January 2005

A case of pseudoxanthoma elasticum with multisystem involvement is described. Neurological complications, as reported in the literature, are reviewed. These include cerebrovascular insufficiency, multiple lacunar infarcts, aneurysms, subarachnoid and intracerebral hemorrhages, progressive intellectual deterioration, and psychic and mental disturbance which may be due to cortical atrophy. Seizures occur more frequently than in the general population. Hypertension and alteration of cerebral vessels are the two basic pathophysiological mechanisms responsible for the neurological complications of this disease.
The morquio syndrome: Neuropathologyxs and biochemistry
Authors: Koto Atsuo , Horwitz Allen L. , Suzuki Kinuko , Tiffany Carol W. , Suzuki Kunihiko
Published: January 2005

The activity of N‐acetyl galactosamine‐6‐sulfate sulfatase was studied for the first time in the liver and brain of a patient with a clinically typical case of Morquio syndrome with keratosulfaturia. As has been demonstrated in the fibroblasts of patients with this syndrome, this enzymatic activity was markedly decreased in both organs.

Neuropathological examination revealed moderately swollen neurons containing PAS‐positive, coarse globular inclusions in the cerebral cortex, Ammon's horn, basal ganglia, and thalamic nuclei. Ultrastructurally, the inclusions consisted of stacked, straight or loose, wavy membranes of various lengths, often associated with pale or moderately electron dense homogeneous “lipid droplets.” These ultrastructural features of the inclusions were closely similar to the granulomembranous bodies of Hurler syndrome and the inclusions described in type B of the Sanfilippo syndrome. Unlike those mucopolysaccharidoses, however, no abnormalities were found in the gangliosides in the brain of the patient with Morquio syndrome.
Nemaline (rod) myopathy: Thexs need for histochemical evaluation of affected families
Authors: Bender Adam N. , Willner Judith P.
Published: January 2005

Histochemical changes in the mother of a patient with nemaline myopathy were used to identify her as the gene carrier even though rod‐bodies were not present in her muscle biopsy and she was not weak. The patient and her mother both had marked type I fiber predominance with large groups of type I fibers present. Histochemical changes known to occur in nemaline myopathy include smallness and predominance of type I fibers. Such changes support the concept that this disease may result from subtle defects in innervation since fiber types are determined by innervation. Although this disease is thought to be transmitted by autosomal dominant mode, lack of male‐to‐male transmission and a predominance of female cases in the literature suggest that this may be (1) an X‐linked dominant, (2) a sex‐influenced autosomal dominant, or (3) an autosomal dominant which is semilethal in males. The family described here is the first in which a presumably affected parent showed only the histochemical change without rod‐bodies, thus emphasizing the importance of histochemical evaluation of relatives' biopsies for genetic counseling.
Increased sodium plus potassium adenosine triphosphatase activity in erythrocyte membranes in Huntington's disease
Authors: Butterfield D. A. , Oeswein J. Q. , Prunty M. E. , Hisle K. C. , Markesbery W. R.
Published: January 2005

Dopa‐decarboxylase, acetylcholinesterase, sodium plus potassium stimulated adenosine triphosphatase (Na+ + K+ − ATPase), and membrane‐bound protein kinase were compared in the erythrocytes of patients with Huntington's disease and normal controls. All these enzymes also exist in the basal ganglia. The Na+ + K+‐ATPase level was elevated (p < 0.05) in Huntington's disease, while no significant changes were observed in the other enzymes. This finding is consistent with the concept that Huntington's disease is associated with a general membrane abnormality.
Cerebral hemodynamics and metabolism in pseudotumor cerebri
Authors: Raichle M. E. , Grubb R. L. , Phelps M. E. , Gado M. H. , Caronna J. J.
Published: January 2005

A comprehensive analysis of cerebral hemodynamics and metabolism was carried out in 14 patients with pseudotumor cerebri. Tracer techniques were employed to measure cerebral blood flow (CBF) and vascular reactivity to acute changes in arterial carbon dioxide tension and blood pressure, cerebral blood volume (CBV), and the cerebral metabolic rate for oxygen and glucose. There was a small reduction (p < 0.01) in CBF (44 ± 7 ml/100 gm/min; normal, 54 ± 9) with normal vascular reactivity; an increase (p < 0.005) in CBV (4.8 ± 0.8 ml/100 gm; normal, 3.6 ± 0.5), and normal cerebral metabolism. We conclude that an abnormality of the cerebral microvasculature is responsible for an elevation in CBV, but the intracranial hypertension can be explained only by tissue swelling due to an increase in water content. The relationship between the vascular abnormality and the tissue swelling remains to be defined.
Sparing of the onufrowicz nucleus in sacral anterior horn lesions
Authors: Iwata Makoto , Hirano Asao
Published: January 2005

Sacral anterior horns were examined with special reference to the Onufrowicz nucleus in 3 patients with Werdnig‐Hoffman disease, 2 with healed anterior poliomyelitis, and 1 who had undergone amputation of both legs above the knee. None of these patients showed symptoms of either urethral or anal sphincter disturbances. The Onufrowicz nucleus of the second sacral anterior horn appeared normal in all the patients examined, though the lateral portion of that horn and the medial nuclear group of the third sacral segment were frequently involved. These findings support the view that the Onufrowicz nucleus may be innervating the external sphincter muscle of the urethra and the anus.
Weakness in malignancy: Evidence for a remote effect of tumor on distal axons
Authors: Barron Stephen A. , Heffner Reid R.
Published: January 2005

Malignant tumors are known to have a “remote” or nonmetastatic effect on the central and peripheral nervous systems. Eight patients were seen with proximal muscle weakness in association with bronchogenic carcinoma (5), carcinoma of breast (2), and leukemia (1). Electomyography demonstrated small polyphasic motor unit potentials. Muscle biopsy, however, showed evidence of denervation. Electron microscopy revealed morphological abnormalities in the intramuscular segments of axons. This combination of EMG and biopsy findings is presented as evidence for a remote effect of tumor on the intramuscular distal axons.
Huntington'S disease: Treatment with muscimol, A GABA‐mimetic drug
Authors: Shoulson Ira , Goldblatt David , Charlton Maurice , Joynt Robert J.
Published: January 2005

Muscimol, a γ‐aminobutyric acid (GABA) analogue that exerts potent and specific agonist effects on GABA receptors, was administered orally to 10 patients with Huntington's disease. In this double‐blind study, muscimol treatment did not result in improvement in these patients' motor or cognitive functions. However, muscimol adminsitration did ameliorate chorea in the most severely hyperkinetic patient, and it was associated with the appearance of dystonic features, electroencephalographic changes, and behavioral alterations in some patients. These latter observations support a functional relationship between GABA‐ergic activity and the genesis of both dystonia and EEG abnormalities in humans. The therapeutic failure of muscimol indicates that the GABA disturbance in Huntington's disease does not alone account for the clinical features of this disorder.

This investigation was funded by a grant from the Hereditary Disease Foundation, Beverly Hills, CA. The University of Rochester Clinical Research Center is supported by U.S. Public Health Service Research Grant RR‐00044 from the Division of Research Facilities and Resources, National Institutes of Health. Muscimol was provided by Smith, Kline, & French Laboratories and formulated by the Strong Memorial Hospital Pharmacy.
Local cerebral glucose utilization in the normal conscious macaque monkey
Authors: Kennedy C. , Sakurada O. , Shinohara M. , Jehle J. , Sokoloff L.
Published: January 2005

The [14C] deoxyglucose method for quantitative determination of local cerebral glucose utilization was extended to the macaque monkey. The necessary constants required by its operational equation were evaluated. The lumped constant, measured in 7 normal conscious monkeys, was found to equal 0.344 (SEM, ± 0.036). The rate constants were also estimated and found to be very similar to those obtained previously in the rat.

With these essential constants evaluated, the method was applied to normal conscious monkeys. Local cerebral glucose utilization was found to vary markedly throughout the brain but to fall within two distributions, a higher one in gray matter and a lower one in white matter. In general, the values fell in a range to be expected from previous measurements of average energy metabolism in the brain as a whole. The values were considerably lower than those observed previously in the conscious rat.

Marked heterogeneity of rates of glucose utilization were observed in a number of anatomical structures. In some cases the patterns of heterogeneity were consistent with known histological cytoarchitecture; in others the heterogeneity did not conform with known cytoarchitectural features but corresponded to patterns previously demonstrated by electrophysiological techniques. Many of the regions of the cerebral cortex showed columnar patterns of distribution of higher and lower rates of glucose utilization. These may be a metabolic reflection of the columnar organization of function within the cerebral cortex.
Fatal long‐term sequela following radiation “cure” for ependymoma
Authors: Bachman David S. , Ostrow Peter T.
Published: January 2005

There are no previous reports of glioblastoma occurring following central nervous system irradiation. This report describes a young girl with an ependymoma at age 13 months who was treated by radiotherapy following surgical removal. At age 6 years she presented with a new lesion in the same location. Histologically there was no evidence of recurrent ependymoma. Instead, the pathological picture suggested glioblastoma or a severe radiation‐induced encephalopathy. In either case, it seems likely that the changes were radiation induced.
Friedreich'S Ataxia: I. Normal pyruvate dehydrogenase complex activity in platelets
Authors: Stumpf David A. , Parks Janice K.
Published: January 2005

Seven patients with Friedreich's ataxia had normal activities of the pyruvate dehydrogenase complex and its components pyruvate dehydrogenase (E1) and lipoamide dehydrogenase (E3) in platelets.
Chiasmal syndrome in sphenoid sinus mucocele
Authors: Goodwin James A. , Glaser Joel S.
Published: January 2005

Two patients with sphenoethmoid mucoceles developed visual field defects consistent with optic chiasm dysfunction. The presence of chiasmal field defects associated with the clinical syndrome of compressive optic neuropaty should not rule out the diagnosis of sphenoethmoid mucocele since definitive treatment can be carried out via transnasal drainage.
Bihemispheric decreases of regional cerebral blood flow in dementia: Correlation with age‐matched normal controls
Authors: Lavy Sylvan , Melamed Eldad , Bentin Shlomo , Cooper Gerald , Rinot Yosef
Published: January 2005

Regional cerebral blood flow (rCBF) was measured by the xenon 133 inhalation method in 20 patients with senile and presenile dementia, and values were compared with flow data obtained in a series of 44 age‐matched normal controls. The rCBF was reduced in patients with both presenile and senile dementia, and the degree of decrease exceeded the age‐related rCBF decline in normal subjects. Similar rCBF reductions were observed in the left and right cerebral hemispheres and in general were homogeneously distributed in the various areas. The rCBF reductions in normal aging and in dementia may be associated with similar underlying cerebral morphological changes. Our findings suggest that the greater rCBF decreases in dementia compared with the values in age‐matched controls may quantitatively reflect more severe pathological alterations in the brains of patients with intellectual deterioration.
Muscle carnitine palmityltransferase deficiency: A case with enzyme deficiency in cultured fibroblasts
Authors: Didonato S. , Cornelio F. , Pacini L. , Peluchetti D. , Rimoldi M. , Spreafico S.
Published: January 2005

A further case of carnitine palmityltransferase (CPT) deficiency in a young man is described, the defect being documented by direct enzyme assays of muscle biopsies. The finding of markedly reduced enzyme activity in the patient's cultured fibroblasts supports the concept that CPT deficiency is a systemic rather than an exclusively muscular condition.
Meningoencephalitis following enucleation for cryptococcal endopthalmitis
Authors: Birkmann Lewiston W. , Bennett Donald R.
Published: January 2005

A man developed meningoencephalitis 9 months after enucleation of his right eye for proved cryptococcal endophthalmiits secondary to a penetrating injury. The presumed portal of entry of Cryptococcus neoformans into the central nervous system was along the optic nerve subarachnoid sheath.
“Glial bundles” in the spinal cord late after paralytic anterior poliomyelitis
Authors: Iwata Makoto , Hirano Asao
Published: January 2005

“Glial bundles” in the proximal portion of the anterior spinal roots, identical to those in Werdnig‐Hoffmann disease, were observed in 3 patients many years after attacks of acute paralytic anterior poliomyelitis. These structures should therefore be considered a nonspecific astrocytic reaction secondary to the destruction of motor neurons and their axons rather than a malformation specific for Werdnig‐Hoffmann disease.
An explanation for the localization of herpes simplex encephalitis?
Authors: Davis Larry E. , Johnson Richard T.
Published: January 2005

Herpes simplex virus encephalitis tends to localize to the temporal and orbitofrontal lobes, giving characteristic clinical and pathological findings. Since the virus remains latent in the trigeminal ganglia, we postulate that this localization may be explained by reactivated virus spreading along the fifth nerve fibers, which innervate basal meninges of the anterior and middle fossae.
Comparative cranial CT enhancement in a primate model of cerebral infarction
Authors: Drayer Burton P. , Dujovny Manuel , Wolfson Sidney K. , Boehnke Manfred , Cook Eugene E. , Rosenbaum Arthur E.
Published: January 2005

The value of various enhancement techniques as opposed to nonenhanced CT scanning was compared in 15 baboons with cerebral infarction secondary to embolization of the left middle cerebral artery. The most prominent CT findings in infarction included an area of low absorption in the opercula–basal ganglia–centrum semiovale region and generalized lateral ventricular enlargement. Intravenous enhancement of the low‐density region occurred in 25% of the animals and often obscured the preenhancement abnormality. However, a rapid bolus injection of contrast material followed by immediate consecutive CT scans (computed angiotomography) permitted prominent visualization of early‐shunting veins. Delayed scanning following intrathecal enhancement better defined small infarctions that did not exhibit the usual cerebral blush. The CT imaging of inhaled xenon provides a new technique for evaluating subtle abnormalities in cerebral perfusion, even when the routine CT scan shows no abnormality.
Delayed pentobarbital administration limits ischemic brain damage in gerbils
Authors: Levy David E. , Brierley James B.
Published: January 2005

The capacity of delayed barbiturate administration to limit brain damage after unilateral cerebral ischemia was examined histologically in gerbils. The right common carotid artery was occluded in 50 animals under brief (3‐minute) halothane anesthesia; 18 animals (36%) developed motor abnormalities consistent with stroke. The arterial clasps were removed after 1 hour and the abnormal animals were divided into treatment and placebo groups. Treated gerbils received sodium pentobarbital (70 mg/kg) intraperitoneally 1 hour after clasp removal and a smaller dose (50 mg/kg) 2 hours later; these animals lost corneal reflexes but retained spontaneous respiration and were kept normothermic. Animals in the placebo group received equivalent volumes of normal saline. Except for the period of anesthesia, both groups had similar postischemic motor behavior.

Neuropathological examination of animals killed by perfusion‐fixation after 24 hours revealed fewer pentobarbital‐treated animals with shif of midline structures and with ipsilateral ischemic damage (including infarction). Compared with the placebo group, there was less extensive neuronal ischemic cell change in five regions of the ipsilateral cerebral hemispheres of the pentobarbital‐treated animals (p < 0.05). The results suggest that barbiturates administered as long as 1 hour after the end of an ischemic insult can still limit brain damage.