bluuebunny/crossref_metadata_2025 huggingface dataset:
Über Unabhängige Bestandteile und univariante Systeme
Authors: Wegscheider Rudolf
Published: January 2005

Eine eindeutige und widerspruchsfreie Wahl der Zahl der unabhängigen Bestandteile ist nur unter Berücksichtigung der bei der Ableitung der Phasenregel gemachten Voraussetzungen möglich, wie dies in der von mir gegebenen Definition der unabhängigen Bestandteile geschehen ist. Demgemäß ist das Verhalten von Systemen gegebener Gesamtzusammensetzung innerhalb eines passend abgegrenzten Temperatur‐ und Druckbereichs zugrunde zu legen. Am besten geht man von der analytischen Zusammensetzung der beim Gleichgewicht möglichen Phasen aus. Hierbei tritt die Notwendigkeit auf, in demselben System bei verschiedenen Versuchsbedingugen die Zahl der unabhängigen Bestandteile verschieden zu wählen, was bezüglich der Temperatur schon von GIBBS geschehen ist. Bei einer anderen möglichen Darstellung durch eine größere Zahl von Komponenten unter Hinzufügung einschränkender Bedingungen muß zwischen Bedingungen unterschieden werden, die aus der Natur des Systems mit Notwendigkeit hervorgehen, und solchen, welche dem System willkürlich auferlegt werden. Bei den Bedingungen der ersten Art ist die Wahl einer geringeren Zahl von unabhängigen Bestandteilen und die Wahl einer größeren Zahl von Komponenten mit Nebenbedingungen sachlich identisch. Bei der Darstellung durch solche Zusatzbedingungen stößt man aber auf die Schwierigkeit, daß dieselbe Zusatzbedingung in einen Teilbereich des Systems auf die Zahl der Freiheiten von Einfluß sein kann und in einem anderen nicht. Nicht aus der Naur des Systems hervorgehende Zusatzbedingungen beeinflussen die Zahl der unabhängigen Bestandteile nicht. Die univarianten Systeme im Sinn der Phasenregel decken sich nicht mit den Systemen, welche Volumänderungen ohne Änderung des Drucks gestatten. Die letztere Erscheinung tritt fast immer bei in‐ und univarianten Systemen auf, kann aber auch bei Systemen mit mehr Freiheiten für singuläre Werte der inneren Veränderlichen vorkommen.
Beitrag zur Natur des Basenaustausches
Authors: Biesalski Ernst
Published: January 2005

Es wird das Gebiet des bekannten Basenaustausches als Grenzgebiet aufgestellt und charakterisiert.

Es wird z. T. experimentell nachgewiesen, daß auch negativ geladene Substrate von nicht zeolithischem Charakter ihre „Elektrolytreste”︁ auf dem Wege von 1. austauschen.
Die Katalyse des Hydroxylamins. III
Authors: Kurtenacker A. , Werner Felicitas
Published: January 2005

In Fortsetzung früherer Versuche wurde die Einwirkung von Vanadium‐(4)‐, Molybdän‐(5)‐, Kupfer‐(1)‐ und Eisen‐(2)‐Salz auf Hydroxylamin unter stufenweiser Änderung der Säure‐ bzw. Laugekonzentration untersucht. Im allgemeinen bestätigen die Versuche die Folgerungen, die sich aus dem Verhalten der höheren Oxydationsstufen der oben genannten Metalle gegenüber Hydroxylamin ergeben. Im einzelnen verlaufen die Reaktionen zwischen den hier untersuchten Salzen und Hydroxylamin wie folgt:

Vanadylsulfat reduziert Hydroxylamin in stark alkalischer Lösung quantitativ zu Ammoniak. Beim Übergang in die neutrale Lösung entstehen neben Ammoniak in steigender Menge gasförmige Oxydationsprodukte (Stickoxydul und Stickstoff) und in schwach saurer Lösung stellt sich der Vorgang als eine durch Vanadylsalz katalysierte Zersetzung des Hydroxylamins in Ammoniak einerseits und Stickoxydul bzw, Stickstoff andererseits dar. In stark saurer Lösung hört die Einwirkung auf

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Benign myoclonus of early infancy
Authors: Lombroso C. T. , Fejerman Natalio
Published: January 2005

Sixteen infants, aged 3 to 81/2 months, were studied close to the onset of a myoclonic syndrome akin to that normally called infantile spasms with hypsarrhythmia. None were found to have the clinical and laboratory characteristics common to the syndrome, however: none showed regression or arrest in their neurological/developmental status, nor did their EEG ever develop a hypsarrhythmic or other abnormal pattern. All were followed for a minimum of 18 months to a maximum of nine years. In all patients the myoclonus stopped early, in none persisting after the age of 2 years, and no other seizure phenomena have so far evolved in any of these infants. This favorable outcome occurred in those who were treated as well as those who were not. The self‐limited course of this condition is emphasized within the context of the more unusual myoclonic syndromes of early and late infancy.
The effect of isoprinosine in subacute sclerosing panencephalitis (SSPE)
Authors: Streletz L. J. , Cracco J.
Published: January 2005

Although no therapeutic agent has proved to be of benefit in subacute sclerosing panencephalitis (SSPE), the use of Isoprinosine has been associated with clinical improvement in several preliminary reports. Two additional patients with SSPE are presented who had repeated serological and electroencephalographic studies during therapy with Isoprinosine. Their clinical courses are discussed in view of the laboratory findings and the natural history of the disease.
Niemann‐pick disease type D: Lipid analyses and studies on sphingomyelinases
Authors: Rao Barbara Gillis , Spence Matthew W.
Published: January 2005

Lipids and sphingomyelinase activity were studied in spleen, liver, and brain tissues of a 13‐year‐old boy with Niemann‐Pick disease type D (NPD‐D). The greatest lipid changes occurred in spleen; cholesterol, cholesterol esters, total phospholipids, sphingomyelin, and bis‐(monoacylglyceryl)phosphate were increased above normal range. In liver, striking increases were observed in cholesterol and bis‐(monoacylglyceryl)phosphate. Minor changes in neutral and acidic glycolipid patterns occurred in liver, spleen, and brain. Sphingomyelinase activity (optimal at pH 5.0) was elevated above mean control levels in liver and spleen, but not in brain, kidney, or leukocytes. Enzyme properties were generally normal. Activity of NPD‐D liver crude homogenate, but not that of normal liver homogenates, was inhibited at high protein concentrations. Activity levels of a second sphingomyelinase, optimal at pH 7.4, in NPD‐D brain were apparently normal. These findings are generally consistent with the classification of NPD‐D as a sphingomyelin lipidosis.
Leigh's disease in an adult with evidence of “inhibitor factor” in family members
Authors: Whetsell William O. , Plaitakis Andreas
Published: January 2005

A 21‐year‐old man with a longstanding history of impaired visual acuity, strabismus, broad‐based gait, and below‐average intellectual capacity developed respiratory difficulties and intermittent generalized weakness at age 19. He subsequently showed signs of massive brainstem dysfunction and died. Postmortem examination demonstrated changes compatible with subacute necrotizing encephalomyelopathy of Leigh involving portions of the diencephalon, midbrain, pons, and medulla as well as portions of the spinal cord and optic nerves. Evaluation of family members for presence of the urinary inhibitor factor for thiamine diphosphate phosphoryl transferase revealed abnormal levels in a brother, a maternal uncle, and the maternal grandfather of the patient.
Peripheral nerve ischemia: Part 2. Accumulation of organelles
Authors: Korthals Jan K. , Korthals Maria A. , Wisniewski Henryk M.
Published: January 2005

Following simultaneous ligation of the aorta and femoral artery in the cat, organelles accumulated in distal portions of the sciatic nerve and in lower portions of the tibial and personeal nerves. The accumulations were located in the proximal and distal ends of the nerve infarction and delineated the borders of the necrotic area.

Topographical analysis of the necrosis and organelle accumulations following ligation of these main arteries showed the larger nerve fascicles to be more sensitive to ischemia than the smaller ones. The center of a nerve fascicle was more sensitive than the periphery. The organelles accumulated in ischemic nerves during the first hours after arterial ligation, presumably as a result of lack of energy for fast axoplasmic transport. Later accumulations reflected mechanical block of axoplasmic transport due to focal necrosis of the nerve.

The content of organelle accumulations was similar to that found in lesions resulting from transection except for the presence of 60 to 70 A filaments at the distal end of the infarction.
Carnitine deficiency: Acute postpartum crisis
Authors: Angelini Corrado , Govoni Edmondo , Bragaglia Mercedes M. , Vergani Ludovica
Published: January 2005

A 16‐year‐old girl, previously in good health, developed progressive generalized muscle weakness following her first parturition. The neck and proximal limb muscles were especially weak and painful. Carnitine (4‐trimethylamino‐3‐hydroxybutyrate) was markedly decreased in muscle, plasma, and urine. Dietary carnitine supplementation, 2.0 gm daily, was followed by clinical improvement and decreased lipid droplets in muscle biopsy. Together with previously reported cases, 2 fatal, the patient's illness emphasizes the risk of pregnancy in women with carnitine deficiency.
Retroperitoneal neuroblastoma widely metastatic to the central nervous system
Authors: Dresler Steven , Harvey Danny G. , Levisohn Paul M.
Published: January 2005

A girl with congenital extraadrenal neuroblastoma died at 3 months of age with multiple parenchymal metastases in the brain and spinal cord. The bones of the skull, cranial dura, venous sinuses, and orbits were not involved. Autopsy findings suggest that the tumor penetrated the spinal meninges and disseminated through the cerebrospinal fluid. It is possible that hematogenous metastasis occurred as well. The intracerebral metastases were not identified on a CT scan performed several hours before the child's death.
Enzyme treatment of spinal cord transected rats
Authors: Feringa Earl R. , Kowalski Timothy F. , Vahlsing H. Lee , Frye Roy A.
Published: January 2005

Russian investigators have recently reported clinical recovery of enzyme treated, spinal cord transected rats. Using the exact protocols of Matinian and Andreasian's two most successful treatment regimens, we repeated their experiments using United States produced pure hyaluronidase and trypsin or trypsin and elastase. Animals were evaluated for return of bladder function, clinical evidence of hind limb motor function, cortical evoked response after sciatic nerve stimulation, and axonal transport of cortically injected tritiated proline by regenerated corticospinal axons.

None of the treated animals differed from control animals treated only with the enzyme vehicle. No animals walked, had return of voluntary motor activity, showed cortical evoked response, or had evidence for transport of tritiated proline over regenerated corticospinal axons.
Nerve lipid abnormalities in human diabetic neuropathy: A correlative study
Authors: Brown Mark J. , Iwamori Masao , Kishimoto Yasuo , Rapoport Basil , Moser Hugo W. , Asbury Arthur K.
Published: January 2005

To study endoneurial lipid composition in human diabetic neuropathy, we biopsied sural nerves from 3 middleaged men with adult‐onset diabetes mellitus. Magnitude of electrophysiological abnormalities and myelinated fiber loss paralleled the clinical severity of neuropathy in all cases. Cholesterol ester concentration was elevated to about 800% of normal in diabetic nerves. Reduction in total endoneurial lipid concentration correlated best with decrease in myelin volume as calculated from measured fiber diameters. Cholesterol, cerebroside, and most phospholipids were reduced in keeping with the severity of fiber loss in each nerve. The phosphatidylinositol‐phosphatidylserine fraction was most reduced in the least affected nerves. Cerebroside nonhydroxy fatty acids in diabetic nerves were of shorter chain length and more saturated than normal. It is not yet clear whether the abnormalities of phosphatidylinositol‐phosphatidylserine and cerebroside fatty acids are of pathogenetic importance or whether these changes may be the nonspecific consequence of axonal degeneration.
Transfer factor is ineffective in amyotrophic lateral sclerosis
Authors: Olarte Marcelo R. , Gersten Joanne C. , Zabriskie John , Rowland Lewis P.
Published: January 2005

Sixty‐six patients with amyotrophic lateral sclerosis (ALS) participated in a double‐blind, placebo‐controlled study of transfer factor. A structured rating score of neurological function was recorded after each monthly administration of transfer factor or placebo. Statistical analysis of mean scores for all patients and for those with specific clinical abnormalities showed no significant difference between the two groups. There was no evidence of therapeutic value for transfer factor. During the observation period of nearly one year, more than two‐thirds of the deterioration occurred in the first six months. Meaningful clinical trials of other potential therapeutic agents could probably be conducted within six months in double‐blind crossover trials.
Hypernatremic hemorrhagic encephalopathy
Authors: Young Richard S. K. , Truax Bradley T.
Published: January 2005

A 12‐year‐old juvenile diabetic was inadvertently given 500 ml of hypertonic saline intravenously. He developed hypernatremia, hyperosmolality, metabolic acidosis, and hyperglycemia. Seizures and stupor ensued, followed by coma and death. Computerized cranial tomography revealed numerous small subcortical hemorrhages that were verified postmortem.
Neonatal myasthenia gravis in the infant of a myasthenic mother in remission
Authors: Elias Stanton B. , Butler Ian , Appel Stanley H.
Published: January 2005

A 26‐year‐old woman was in spontaneous clinical remission from myasthenia gravis (MG) for six months, yet gave birth to a full‐term infant with typical neonatal MG. It is believed that transplacental transfer of anti‐acetylcholine (ACh) receptor antibodies is responsible for neonatal MG; therefore, neonatal MG represents an in vivo assay of the pathogenic potential of anti‐ACh receptor antibodies in 2 human individuals. Anti‐ACh receptor antibodies were present in both mother and infant (titers 12.3 × 10−9 and 4.4 × 10−9 moles per liter, respectively) at the time of birth, and both mother's and infant's sera accelerated the degradation of ACh receptors in myotube cultures. This case suggests that “host factors” unique to the individual appear to modify or even determine whether the presence of anti‐ACh receptor antibodies will result in clinical myasthenia.
Cerebral embolism from septal fibromuscular dysplasia of the common carotid artery
Authors: So Elson L. , Toole James F. , Moody Dixon M. , Challa Venkata R.
Published: January 2005

A rare type of fibromuscular dysplasia of the common carotid artery, in the form of a septum, was observed in a patient suffering from recurrent cerebral infarctions. Angiography demonstrated growth fo the lesion and documented the presence of an intraluminal thrombus attached to it. Comparison with reported cases of the more common “string of beads” lesion of fibromuscular dysplasia suggests that the risk for development of ischemic neurological problems is higher for this type of fibromuscular lesion. Surgical correction is recommended, especially for symptomatic patients.
Cellular immune response to measles, mumps, and vaccinia viruses in multiple sclerosis
Authors: McFarland Henry F. , McFarlin Dale E.
Published: January 2005

The cell‐mediated immune response to measles, mumps, and vaccinia viruses was studied in MS patients, normal controls, and neurological disease controls using a lymphocyte proliferation assay. A small but significant difference was found in the response to measles between the MS and normal control groups but not between the MS and neurological disease control groups. In each of the groups, the response of both peripheral blood leukocytes and purified T‐cells to measles was significantly less than the response to mumps or vaccinia. The lower response to measles was not due to the presence of blocking factors or to lack of antigenicity of the measles virus preparation used in the assay. These findings suggest that differences exist in the normal immune response between these viruses. Precise quantitation of the immunological response to viruses in MS and other disease states will depend on identification of the various functionally reactive cell populations.
Eating epilepsy associated with a deep forebrain glioma
Authors: Robertson W. C. , Fariello R. G.
Published: January 2005

Seizures associated with deglutition are rare, and previously reported cases of so‐called eating epilepsy have not identified specific electrophysiological or anatomical foci. We evaluated a 14‐year‐old with a deep hemispheric astrocytoma in whom focal sezures were consistently triggered by eating. Electroencephalograms were consistent with a deeply located seizure focus. Eating epilepsy should be grouped with reflex epilepsies that may originate with the diencephalon.
Huntington's disease: Abnormality of lymphocyte capping
Authors: Noronha Avertano B. C. , Roos Raymond P. , Antel Jack P. , Arnason Barry G. W.
Published: January 2005

Concanavalin A‐induced lymphocyte capping was studied in 30 patients with Huntington's disease (HD) and 39 controls. A significant decrease in lymphocyte capping was seen in the 24 patients with HD less than 60 years of age compared to 25 age‐matched controls (p < 0.01); above age 60, differences between patients with HD and controls were not significant. These findings lend support to previous observations that generalized membrane abnormalities are present in patients with HD. Studies of HD lymphocyte capping may permit a detailed analysis of membrane function in cells that are readily obtained from living patients.
Subarachnoid spread of ependymoma: Diagnosis and evaluation of therapy by computerized tomography
Authors: Geehr Robert B. , Dohrmann George J. , Dohrmann George J. , Dohrmann George J. , Kier E. Leon , Wagner Franklin C. , Kapp Daniel S.
Published: January 2005

The potential of ependymomas to spread throughout the intracranial subarachnoid space is well documented, though the incidence is disputed. This relates in part to the time required for such metastases to produce symptoms. The diagnosis of such spread, however, is important for therapeutic management. This report demonstrates the effectiveness of computerized tomography in diagnosing spread of ependy moma throughout the basal cisterns and cerebral fissures.
Barognosis in a patient with hemiaraxia
Authors: Angel Ronald W.
Published: January 2005

Barognosis was tested in a patient who had a lesion of the right cerebellar hemisphere. While blindfolded, he used each hand to estimate the mass of given test loads, alternating hands between trials. he moved each load sinusoidally, comparing it with the previous one. The period of oscillation was found to be a linear function of the mass. With eah load, the period of oscillation was longer when he used the ataxic hand. On 23 of 59 comparisons between loads, the patient reported incorrectly. Ninety‐one percent of the errors were in the direction of loads feeling heavier when the ataxic hand was used.

To explain these results, it is propsed that (1) the perceived magnitude of a load depends on the corollary discharge, and (2) cerebellar damage may reduce the accuracy of the corollary discharge.
Medullablastoma presenting as neck tumors in 2 infants
Authors: Gordon McComb J. , Davis Richard L. , Isaacs Hart , Landing Benjamin H.
Published: January 2005

Extension of neuroepithelial neoplasms outside the neuraxis, though uncommon, is well known and usually follows surgical intervention. More unusual is the brain tumor that appears beyond the bounds of the central nervous system prior to operative manipulation. Two infants presented with soft tissue masses in the neck that proved to be medulloblastomas. Autopsy examination in both infants showed primary medulloblastoma of the cerebellar vermis with extensive spread through the subarachnoid spaces. The neck masses may have been direct extension from the central nervous system tumors, though this was not proved in either case.
The effect of alpha‐adrenergic innervation on caudate blood flow
Authors: Levy Lewis , Wicke J. D.
Published: January 2005

A thermal diffusion probe, with cannulas for intracerebral microinfusion of drugs and an electrode to monitor electroencephalographic (EEG) activity, was used to examine the local effect of vasoactive amines in a 4 to 5 mm sphere of caudate nucleus in cats. The results demonstrated that it is possible to alter local cerebral blood flow (CBF) without causing any change in systemic blood pressure or heart rate, or in CBF and the EEG in the opposite caudate. One‐microliter intracerebral injections, containing varying amounts of phenylephrine, increased local CBF in proportion to dose. The effect was blocked by intracerebral infusion of phentolamine. However, local alpha‐adrenergic blockade did not inhibit vascular responses to blood pressure elevation or to metabolic influences on local blood flow.
Comparison of antigenic sources for acetylcholine receptor antibody assays in myasthenia gravis
Authors: McAdams Mildred W. , Roses Allen D.
Published: January 2005

Antibody titers to the acetylcholine receptor (AChR) from patients with myasthenia gravis, in identical serum samples, were directly compared using denervated rat, human, and baboon muscle as the source of AChR antigen for radioimmunoassay (RIA). Calculations were standardized by using binding isotherms for each antigen source and calculating the percentage of AChR sites labeled with [125I]α‐bungarotoxin at the concentration used in the RIA. In patients with high AChR antibody titers, the antibody concentration when human muscle was used as the antigen source measured up to tenfold higher than that with denervated rat muscle. In patients who had low antibody titers with human muscle antigen, assays using rat denervated muscle AChR frequently failed to demonstrate diagnostically abnormal titers. The data explain differences among several reported series in the percentages of patients with myasthenia gravis who had elevated serum antibody concentrations. AChR antibody concentrations with baboon muscle as the antigen source were comparable to those in which human muscle was used.
Failure of intravenous and intrathecal cytarabine to modify central nervous system IgG synthesis in multiple sclerosis
Authors: Tourtellotte Wallace W. , Potvin Alfred R. , Mendez Mario , Baumhefner Robert W. , Potvin Janet H. , Ma Booe I. , Syndulko Karl
Published: January 2005

To study the nature of the multiple sclerosis (MS) central nervous system (CNS) immune reaction, cytarabine (ara‐C) was administered intravenously to three patients and intrathecally to another seven. Although intravenous administration severely suppressed circulating leukocytes derived from the bone marrow, there was no change in de novo CNS IgG synthesis (rate or presence of CSF IgG oligoclones). Therefore, the MS CNS immune reaction can persist in the presence of severe granulocytopenia and severe monocytopenia of one to two weeks' duration. In four patients who were given ara‐C intrathecally, cytotoxic levels (>10 μg/ml) were present for at least 24 hours. A brief elevation in CSF albumin indicated transient damage to the blood‐brain barrier. Leukopenia did not result in the seven patients treated intrathecally, and de novo CNS IgG synthesis did not change or was transiently increased. Assuming that cytotoxic levels in CSF diffused to the CNS sites responsible for synthesizing IgG, results indicate that CNS IgG synthesis does not depend on in situ rapid turnover of immune cells. None of the ten patients demonstrated neurological improvement or persistent adverse effects.
Cerebrospinal fluid biochemical markers of central nervous system metastases
Authors: Clifford Schold S. , Wasserstrom William R. , Fleisher Martin , Schwartz Morton K. , Posner Jerome B.
Published: January 2005

β‐Glucuronidase and carcinoembryonic antigen (CEA) were measured in the cerebrospinal fluid of patients with cancer. Both substances were found to reliably detect the presence of leptomeningeal infiltration by carcinoma. Neither substance was reliable in the detection of leptomeningeal infiltration by lymphoma or of metastases to the brain parenchyma or spinal epidural space. β‐Glucuronidase was moderately elevated in chronic infectious meningitis, whereas CEA was not. Both markers approached control levels with favorable treatment of the leptomeningeal metastases, reflecting the effectiveness of treatment. Both β‐glucuronidase and CEA hold promise as indicators of early metastatic involvement of the leptomeninges by carcinoma.
Intensive evaluation of referred unclassified neuropathies yields improved diagnosis
Authors: Dyck Peter James , Oviatt Karen F. , Lambert Edward H.
Published: January 2005

Intensive evaluation of 205 cases of undiagnosed neuropathy in a center with special approaches and facilities permittede classification of 76% of the patients. Inherited disorders accounted for 42% of the series, 21% of the patients were shown to have inflammatory‐demyelinating polyradiculoneuropathy, and 13% had neuropathies assocciated with other disorders. A considerable improvement in diagnosis was possible from evaluation of the kin of the patients with undiagnosed neuropathy. Analysis of the frequency and type of various sensory symptoms alos was helpful in distinguishing between acquired and inherited neuropathies.
Familial hemiplegic migraine: EEG and CT scan study of two cases
Authors: Gastaut J. L. , Yermenos E. , Bonnefoy M. , Cros D.
Published: January 2005

The cases of a woman and her son with a long history (24 and 9 years, respectively) of hemiplegic migraine are presented. The mother demonstrated exclusively right‐sided paralysis; in the son, paralysis occurred on each side. Electroencephalograms recorded during the attacks were characterized by pseudoperiodic slow sharp waves over the hemisphere contralateral to the hemiplegia. Between attacks the neurological examination and EEG were normal. CT scans performed during and between attacks showed no abnormalities.
Ammonia metabolism in Reye syndrome and the effect of citrulline
Authors: Delong G. Robert , Glick Thomas H.
Published: January 2005

Ammonia metabolism in Reye syndrome was studied by quantitative analysis of the time course of hyperammonemia and the urinary excretion of ammonia, urea, and total nitrogen. These measures were then utilized to assess the effect of citrulline administration in 8 patients compared to results in 22 patients managed without citrulline. Two indices of the severity and duration of hyperammonemia correlated strongly with mortality: the half‐time for decline of hyperammonemia and the area under the hyperammonemia curve (an index of the total burden of ammonia presented to the brain). These results suggest that the total amount of ammonia delivered to brain may be important to the pathogenesis of encephalopathy. Citrulline‐treated patients had more severe disease at admission and greater abnormalities in indices of nitrogen and ammonia metabolism, though the latter did not reach significance. The urine ammonia/urea nitrogen excretion ratio, an index of the efficiency of ammonia conversion to urea, normalized more rapidly in the citrulline‐treated group, evidence that citrulline may have improved urea cycle function. Overall mortality did not differ in the two groups. The deaths of 2 citrulline‐treated patients in this small group were attributable to factors unrelated to treatment, however, so the possible effect of citrulline on mortality was not definitively tested. No indication was found that citrulline was harmful, nor that it increased ammonia levels.
Evidence for autosomal recessive inheritance in systemic carnitine deficiency
Authors: Di Donato S. , Rimoldi M. , Cornelio F. , Bottacchi E. , Giunta A.
Published: January 2005

Identical twin girls died at age 3 years from acute episodes of metabolic encephalopathy reminiscent of Reye syndrome. Biochemical studies in one of the twins demonstrated low levels of carnitine in muscle, liver, and plasma. Carnitine content was low in the liver and plasma of both parents, implying autosomal recessive transmission.
Childhood multiple sclerosis: Clinical features and demonstration of changes in T cell subsets with disease activity
Authors: Hauser Stephen L. , Bresnan Michael J. , Reinherz Ellis L. , Weiner Howard L.
Published: January 2005

Using monoclonal antibodies that identify T cell subsets in human beings (T4 = inducer cell; T5 = suppressor/cytotoxic cell), we have previously shown that most patients with multiple sclerosis (MS) have decreased T5 cells and an elevated T4: T5 ratio during periods of disease activity. We have recently studied three children with MS beginning at 4 years 11 months, 10 1/2 years, and 2 years 11 months of age. Clinical symptoms included a relapsing brainstem syndrome in the first, Devic syndrome and recurrent optic neuritis in the second, and multiple attacks with accumulating neurological deficits in the third. Two of these children represent the youngest cases of MS yet reported. In each child, circulating T5 cells were reduced or absent during an acute exacerbation of disease; following stabilization, T5 cells returned and the T4: T5 ratio became normal. No changes in T cell subsets were seen in a group of age‐matched healthy children or in children suffering from other neurological diseases. Our finding that young children with active MS have a pattern of T cell abnormalities identical to that of adult MS patients suggests that the same pathophysiological process which causes MS in adults occurs in young children as well.
A new autoradiographic approach for imaging forebrain dopamine distribution
Authors: Wooten G. Frederick , Horne Malcolm K.
Published: January 2005

Regional distribution of carbon 14 activity in the forebrain was studied autoradiographically following systemic administration of tracer doses of L‐[14C]‐dopa to rats pretreated with carbidopa. Five minutes after the injection, regional forebrain distribution of carbon 14 varied with regional cerebral blood flow. At 45 minutes, 2 hours, and 4 hours after administration of 14C‐dopa, however, carbon 14 activity had accumulated selectively in forebrain regions rich in dopamine (e.g., the striatum, olfactory tubercle, and nucleus accumbens). A unilateral lesion of the substantia nigra markedly attenuated the accumulation of carbon 14 in the ipsilateral striatum, while reserpine pretreatment reduced by about 50% the accumulation of carbon 14 in the striatum bilaterally. These results outline a new autoradiographic method for mapping forebrain dopamine systems and suggest an approach to imaging brain dopaminergic neurons in humans by positron emission tomography.
Recurrent herpes simplex encephalitis: Recovery of virus after Ara‐A treatment
Authors: Dix Richard D. , Baringer J. Richard , Panitch Hillel S. , Rosenberg Sidney H. , Hagedorn Joel , Whaley Jay
Published: January 2005

A 54‐year‐old man developed clinical findings consistent with herpes simplex virus (HSV) encephalitis. These signs included an abrupt onset of focal central nervous system disease, cerebrospinal fluid pleocytosis, localized electroen cephalographic abnormalities, and a computerized tomographic scan showing right temporal lobe involvement. Treatment with adenine arabinoside (Ara‐A) resulted in improvement. Two months later he again became confused, and a left hemiparesis developed. Although biopsy revealed extensive necrosis and inflammatory response, HSV antigens and herpesvirus particles were not detected. Culture of biopsy tissue yielded HSV type 1 only after 18 days. A second course of Ara‐A was administered but the patient failed to improve and died four months later. Extensive inflammatory necrosis of both temporal lobes involving gray and white matter was found. Cultures were negative for HSV. The recovery of virus from our patient during the second encephalitic episode should raise concerns regarding the efficacy of Ara‐A treatment and the role of the virus in recurrent disease. In addition, the importance of maintaining biopsy tissue in culture for prolonged periods is emphasized.
Variable waveforms in downbeat nystagmus imply short‐term gain changes
Authors: Abel L. A. , Traccis S. , Dell'Osso L. F. , Ansevin C. F.
Published: January 2005

A patient with downbeat nystagmus subsequent to ankylosing spondylitis was studied. His nystagmus was found to exhibit both increasing‐ and decreasing‐velocity exponential slow phases as well as the linear form more often reported. Alternation between waveforms sometimes occurred on a beat‐to‐beat or even intrabeat basis. Possible expalnations for all three waveforms are presented in terms of short‐term gain changes in cerebellar compensation for leaky brainstem neural integrators. A computer model was developed and its results are discussed.
Changes in regional cerebral blood flow during the course of classic migraine attacks
Authors: Lauritzen Martin , Olsen Tom Skyhøj , Lassen Niels A. , Paulson Olaf B.
Published: January 2005

Regional cerebral blood flow (rCBF) following carotid arteriography was studied in thirteen patients with classic migraine. Using the 133xenon intraarterial injection method, rCBF was measured in 254 areas in one hemisphere. Nine patients developed a characteristic attack following arteriography and were examined by a series of rCBF studies, spaced by intervals of 5 to 10 minutes. A wave of reduced blood flow originating in the posterior part of the brain and progressing anteriorly was observed in eight of the nine patients. The oligemia advanced at a speed of 2 mm per minute over the hemisphere, progressing anteriorly but not crossing the rolandic or sylvian sulcus. Typically, the spreading oligemia reached the primary sensorimotor area after symptoms from that area had begun and persisted there long after the focal symptoms had disappeared. The observed time course suggests that the focal symptoms are not secondary to the oligemia. We suggest that focal symptoms and blood flow changes may be secondary to spreading depression of Leao.
Parental age as a risk factor in Alzheimer's disease
Authors: Corkin Suzanne , Growdon John H. , Rasmussen Shelley L.
Published: January 2005

Because of the associations between Alzheimer's disease and Down's syndrome, advanced maternal and paternal age, which are risk factors for Down's syndrome, have been proposed as risk factors for Alzheimer's disease. Parental age data were obtained from a group of patients with Alzheimer's disease and compared with comparable data for a group of nondemented subjects matched for age and socioeconomic level. As a way of detecting a familial causative factor in Alzheimer's disease, life‐span data were also collected for parents of patients and control subjects. The results indicated that patients with Alzheimer's disease and nondemented control subjects did not differ in the mean ages of their parents at the time of the patients' or subjects' births, nor did parental age correlate significantly with age at onset of disease. Moreover, the parents of patients with Alzheimer's disease did not differ in mean age at death from the parents of nondemented control subjects.
Outcome in neonates with convulsions treated in an intensive care unit
Authors: Bergman Ira , Painter Michael J. , Hirsch Robert P. , Crumrine Patricia K. , David Ronald
Published: January 2005

Neurological and developmental outcome was assessed in 131 survivors of neonatal seizures aged 1 to 5 years who had been treated in a single intensive care unit from 1976 to 1979. Half the children had been born at less than 37 weeks' gestational age, and 28% at 31 weeks or less. Fifty‐one children were normal on examination, 17 had minor abnormalities, 25 had moderate disabilities, 30 had severe disabilities, 6 had died because of profound neurological deficits, and 2 could not be located. Recurrent nonfebrile seizures had developed in 26 children. Most children with motor handicaps or visual loss were intellectually retarded, but 10 of 15 children with bilateral hearing loss were intellectually normal. Of 77 children whose seizures were caused by a hypoxic‐ischemic insult, 41 developed moderate or severe disabilities. As determined by multivariate analysis, significant neonatal predictors of poor outcome in this group included seizures with late onset, tonic seizures, and seizures lasting for many days. Although seizure freqency and neonatal mortality associated with seizures were greatest in very premature infants, the outcome in premature infants who survived was not significantly different from that of term infants.
Hepatitis a virus immunoglobulin m antibody in acute neurological disease
Authors: Bosch Vivian V. , Dowling Peter C. , Cook Stuart D.
Published: January 2005

Sera from 167 patients with Guillain‐Barré syndrome, 224 patients with other neurological diseases, and 10 normal subjects were tested for hepatitis A virus– specific immunoglobulin M antibodies. Two patients with classic Guillain‐Barré syndrome and 1 patient with acute myeloradiculopathy had high titers of virusspecific IgM antibody. All three patients had jaundice and laboratory evidence of liver involvement before the onset of the neurological disease. None of the other sera from patients with Guillain‐Barré syndrome or control subjects were positive for hepatitis A virus.
Lack of clinical efficacy of chronic oral physostigmine in Alzheimer's disease
Authors: Jotkowitz Seymour
Published: January 2005

Ten patients with mild to severe Alzheimer's disease were treated for up to 10 months with 10 to 15 mg oral physostigmine daily or with placebo, according to a single blind, multiple crossover protocol. No clinically meaningful improvement occurred with physostigmine treatment.
Occupational adjustment following neurosurgical treatment of epilepsy
Authors: Augustine Elizabeth A. , Novelly Robert A. , Mattson Richard H. , Glaser Gilbert H. , Williamson Peter D. , Spencer Dennis D. , Spencer Susan S.
Published: January 2005

Occupational adjustment was investigated in a group of 32 adult epileptic patients followed for 1 to 10 years after cortical resection for intractable seizues. An employment status rating was assigned according to the following scale: (1) employed (working 75 to 100% of full time); (2) underemployed (25 to 74%); and (3) unemployed (0 to 24%). The number of patients employed increased from 14 to 23, whereas the number of underemployed decreased from 8 to 0. The unemployed group showed little change. An improvement or maintenance of employment status was correlated directly with improved postoperative seizure control. Conversely, poor occupational adjustment was often associated with fair or poor seizure control following operation. Unemployment was also related to the presence of preoperative psychiatric disorders, a history of past unemployment, and cognitive disturbances affecting nondominant hemisphere function. Although improvement in seizure control is important, this investigation suggests it is only one of many influences on the occupational adjustment of patients following surgical treatment of epilepsy.
Neurodevelopmental outcome of survivors with posthemorrhagic hydrocephalus following grade II neonatal intraventricular hemorrhage
Authors: Krishnamoorthy K. S. , Kuehnle K. J. , Todres I. D. , Delong G. R.
Published: January 2005

This report describes the neurodevelopmental outcome in 12 children who developed posthemorrhagic hydrocephalus following Grade II intraventricular hemorrhage. Grade II intraventricular hemorrhage is defined as the partial filling of both lateral ventricles by intraventricular blood, documented by computed tomographic brain scan. Characeristics of the study group included a mean birth weight of 1.8 kg and a mean gestation of 32.3 weeks. Subjects were followed for three to seven years (mean, four and one‐half years). Neurological evalutions were conducted prospectively. Bayley's Scales of Infant Development and McCarthy's Scales of Children's Abilities were used for the psychometric evalutions. All children were ambulatory. Only 1 had moderate spastic diplegia. Nine children (75%) had IQ scores within the normal range. The results of this prospective analysis suggest a favorable outcome for infants surviving with Grade II intraventricular hemorrhage, even with the complication of posthemorrhagic hydrocephalus.
Outcome of neonatal intraventricular hemorrhage with periventricular echodense lesions
Authors: McMenamin Joseph B. , Shcackelford Gray D. , Volpe Joseph J.
Published: January 2005

The incidence of periventricular‐intraventricular hemorrhage (PV‐IVH) in a group of 460 pretern infants with birth weight less than 2,250 gm, studies by cranial ultrasonography, was 39%. Sixty‐four (36%) of the infants with periventricular‐intravenmtricular hemorrhage had, in addition, perventricular intraparenchymal echodensity (IPE) evident on ultrasound scan. Thirty‐three of the 64 infants had large IPE, and 31 had small IPE. Large IPE consisted of globular echodensity, most often onj the side of maximum intraventricular hemorrhage, extending from the external angle of the laterakl ventricle into major portions of the white mater of the frontal and parietal lobes; small IPE, often bilateral, consisted of linear echodensity extending for a few millimeters from the external ange of the lateral ventricle into the periventricular white matter. The outcome for ingfants with large and a mall IPE differed markedly. Mortality was greatest(94%) for infants with large IPE and birth weight less thasn 1,000gm. All survivors with large IPE, regardless of birth weight, had modrate to severe neurological deficits evident on follow‐up. In contrast, infants with small IPE and birth weight, had moderate to severe neurological deficts evident on follow‐up. In contrast, infants with small IPE and birth weight less thasn 1,000 gm had a mortality of38%. Moreover, 70% of all survivors with small IPE were free of neurological deficits on follow‐up. The difference in outsome appeared to relate in largest part to the severity of the parenchymal involvement. Thjese data have major significance for decisions concerning management of infants with periventricular‐hemorrhage and intraparenchymal involvement.
Painful lumbosacral plexopathy with elevated erythrocyte sedimentation rate: A treatable inflammatory syndrome
Authors: Bradley Walter G. , Chad David , Verghese Jacob P. , Liu Hsiu‐Chih , Good Paul , Gabbai Alberto A. , Adelman Lester S.
Published: January 2005

Six patients had a syndrome of painful lumbosacral plexopathy and elevated erythrocyte sedimentation rate. Sural nerve biopsy in each case showed axonal degeneration and epineurial arterioles surrounded by mononuclear inflammatory cells. Differential fascicular involvement suggested an ischemic cause in three nerves, but no patient had a necrotizing vasculitis. None of the six patients had vasculitis or cancer. Three of the six were diabetic and were initially thought to have diabetic plexopathy, but deterioration continued despite control of the diabetes. These six patients appeared to have an ischemic neuropathy with an immunological basis. Five were treated with immunosuppressant drugs, and in four the plexopathy improved or was arrested. The pathogenesis is unclear, but postmortem findings in one case suggest that the syndrome does not stem from an underlying vasculitis.
Abnormal neuromuscular transmission in an infantile myasthenic syndrome
Authors: Albers James W. , Faulkner John A. , Dorovini‐Zis Katerina , Barald Kate F. , Must Rachel E. , Ball Richard D.
Published: January 2005

A term infant required intubation for respiratory depression. Examination revealed hypotonia and areflexia with intact extraocular movements. Electrodiagnostic studies demonstrated defective neuromuscular transmission characterized by borderline low motor evoked amplitudes, profound decremental responses at all stimulation rates, and moderate facilitation (50 to 740%) 15 seconds after 5 seconds of 50 Hz stimulation. Repetitive muscle action potential responses were not recorded following stimulation of nerves by single shocks. Sensory evoked responses and needle electromyographic findings were normal, as were acetylcholine receptor antibody levels. Results of muscle histochemical analyses, including acetylcholinesterase stains, were normal. End‐plate histometric analyses demonstrated only a slight reduction in mean synaptic vesicle diameter compared with that in an adult control subject. In vitro muscle contractile properties, stimulating the muscle directly, were normal. Anticholinesterase medications were ineffective. Guanidine produced clinical deterioration. The amplitude of motor evoked responses progressively declined, whereas the percentage of decrement and amount of post‐tetanic facilitation increased. Although the nature of the transmission defect was not identified, the data are consistent with abnormal acetylcholine resynthesis, mobilization, or storage without abnormality of release or receptors.
Midbrain paresis of horizontal gaze
Authors: Zackon David H. , Sharpe James A.
Published: January 2005

Unilateral paramedian involvement of the midbrain tegmentum causes monocular paralysis of adduction in the ipsilateral eye, paresis of contralateral saccades in the opposite eye, and conjugate paresis of ipsilateral smooth pursuit. The adduction paralysis can be nuclear, or internuclear from a lesion in the medial longitudinal fasciculus. This distinctive midbrain syndrome of horizontal gaze paresis is exemplified by means of quantitative infrared oculographic, radiological, and neuropathological correlation in two patients with predominantly paramedian midbrain tumors involving the mesencephalic reticular formation and the oculomotor nucleus. Binocular paralysis of elevation provided evidence that one human oculomotor nucleus contains axons to both superior rectus muscles, as does the simian oculomotor nucleus. The midbrain tectum was spared. These pathophysiological correlations indicate that the mesencephalic reticular formation contains pathways that control contralateral saccades and ipsilateral smooth pursuit.
Unique aspects of human newborn cerebral metabolism evaluated with phosphorus nuclear magnetic resonance spectroscopy
Authors: Younkin Donald P. , Delivoria‐Papadopoulos Maria , Leonard Joe C. , Harihara V. Subramanian , Eleff Scott , Leigh PhD John S. , Chance Britton
Published: January 2005

In vivo phosphorus nuclear magnetic resonance spectroscopy (31P NMR) was used to evaluate the pattern of phosphate compounds in seven newborn babies (mean gestational age, 32 weeks; birth weight, 1,430 gm; age, 37 days) with a history of perinatal asphyxia. Spectra were collected in a 1.9 Tesla superconductive magnet with surface coil techniques. The spectra had characteristic peaks for phosphorylated monoesters (PME), inorganic phosphate (Pi), phosphodiesters (PD), phosphocreatine (PCr), and ATP. In contrast to cortical spectra from mature animals, these newborn infant 31P NMR spectra were dominated by a large PME peak and had small PCr, Pi, and PD peaks. Intracellular pH, as measured from the chemical shift of the Pi peak relative to the PCr peak, was 7.1 ± 0.1 (SD). We studied one infant postmortem, and a large PME peak was present in his spectrum. The presence of PME 3 hours after death strongly suggests that it is not a sugar phosphate. In NMR spectroscopy, compounds are identified by their chemical shift relative to a known standard (PCr); the chemical shift of the PME peak was 6.5 ppm, suggesting that it is a mixture of phosphoryl ethanolamine and phosphoryl choline. The PCr/Pi ratio (1.3 ± 0.7) and the PCr/ATP ratio (0.7 ± 0.4) were lower in these babies than in mature animals (greater than 2 and greater than 1.4, respectively); the PME/PD ratio (1.2 ± 0.6), however, was much greater in the infants (mature animals, less than 0.2). These findings suggest that there are unique aspects of human newborn cerebral metabolites and bioenergetic reserve.