bluuebunny/crossref_metadata_2025_split huggingface dataset:
Multifocal varicella‐zoster virus leukoencephalitis temporally remote from herpes zoster
Authors: Horten Bruce , Price Richard W. , Jimenez Daisy 
Published: January 2005

AbstractTwo patients with cancer, one with Hodkin's disease and the other with a granulosa cell tumor of the ovary, developed a progressive, eventually fatal infection of the central nervous system exhibiting multifocal symptoms and signs. Pathologically, gross abnormalities of the brain resembled those in progressive multifocal leukoencephalopathy (PML), with discrete and confluent plaque‐like lesions concentrated in the white matter, particularly along the gray‐white junction. Microscopically, pathological changes differed distinctly from those associated with PML; in addition to confluent foci of white matter injury characterized by early demyelination and subsequent necrosis, prominent Cowdry type A eosinophilic intranuclear inclusions were noted in oligodendrocytes, astrocytes, and neurons. By electron microscopy, intranuclear spherical particles consistent in size and appearance with herpesvirus nucleocapsids were found within the lesions. Immunoperoxidase studies detected varicella‐zoster virus (VZV) antigens in infected cells, implicating this virus as the responsible agent despite a lapse of many months between the cutaneous herpes zoster and onset of cerebral symptoms in both patients.
Galvanic vestibular tests in the assessment of coma and brain death
Authors: Toglia Joseph U. , Adam Rasheed U. , Stewart George 
Published: January 2005

AbstractGalvanic stimulation over the mastoid process elicits oculovestibular responses that provide a measure of brainstem function. The equipment is simple and is available in most neurodiagnostic laboratories. This type of test provides an advantage over caloric testing in the presence of external and middle ear disease and basal skull fracture and can be a useful adjunct in the evaluation of coma and brain death.
Usefulness of electrophysiological studies in the diagnosis of lumbosacral root disease
Authors: Tonzola Richard F. , Ackil Albert A. , Shahani Bhagwan T. , Young Robert R. 
Published: January 2005

AbstractClinical, electrophysiological, and myelographic findings were correlated in 57 patients with the clinical diagnosis of lumbosacral root disease. Conventional motor and sensory (including sural nerve) conduction studies were normal in all patients. Electromyography, late response studies in different muscles of the lower extremity, the myelogram, or combinations of these tests were abnormal in 44 patients (77%). Of 36 patients (63%) with abnormal myelograms, 14 had normal electrophysiological studies. Twenty‐nine (51%) had an abnormal electrophysiological finding; although 8 patients in this group had a normal myelogram, 2 had an abnormal discogram and 1 an abnormal epidurogram. Electrophysiological or myelographic findings, in some cases both, correlated well with clinical signs and symptoms in 41 patients (72%). H‐reflex and F response studies, when abnormal, helped in localizing a lesion in the appropriate root distribution.
Focal hyperemia followed by spreading oligemia and impaired activation of rcbf in classic migraine
Authors: Olesen Jes , Larsen Bo , Lauritzen Martin 
Published: January 2005

AbstractRegional cerebral blood flow (rCBF) was measured in 254 areas of a hemisphere with the xenon 133 intraarterial injection method. Six cases of classic migraine were followed from the normal state into the prodromal phase, and in 3 cases further into the headache phase. One patient with common migraine was similarly followed during his only classic attack. The attacks were initiated by focal hyperemia in 3 patients. During prodromes all patients displayed occipitoparietal rCBF reduction (oligemia), but in only 1 case did the reduction approach critical values. Oligemia gradually spread anteriorly in the course of 15 to 45 minutes. In 4 patients a global oligemia was observed. In 4 patients severe headache was present concomitantly with oligemia and with no sign of hyperemia or nonhomogeneous brain perfusion. The normal rCBF increase during cortical activity (hand movement, speech, and similar activities) was impaired in 6 patients. The results indicate that the vasospastic model of the migraine attack is too simplistic.
Sustained upgaze in coma
Authors: Keane James R. 
Published: January 2005

AbstractComa with sustained upward gaze deviation followed cardiac arrest in 15 patients and prolonged systemic hypotension in 2 patients. Neuropathological examinations confirmed the expected diffuse cerebral and cerebellar damage with relative sparing of the brainstem. No focal lesions were found in the upper midbrain or pretectum. Six patients developed slow, downward‐beating nystagmus as the forced upward gaze resolved. Four of 6 patients with posthypoxic facial myoclonic jerks demonstrated synchronous upward gaze movements. Forced upgaze in coma usually is the result of severe hypoxic encephalopathy. While anatomical localization is obscure, this sign does not indicate a structural lesion in the pretectum or midbrain and may result from damage to cerebellar pathways.
Autologous lymphocyte proliferation in multiple sclerosis and the effect of intravenous ACTH
Authors: Birnbaum Gray , Kotilinek Linda 
Published: January 2005

AbstractThe proliferative reponse of peripheral blood T cells to autologous non–T cells, a reaction called the autologous mixed lympbocyte reaction (MLR), was significantly increased in 17 patients with active multiple sclerosis (MS) compared to age‐ and sex‐matched individuals with other neurological diseases (OND). Following a 10‐day course of intravenous adrenocorticotropic hormone (ACTH) therapy the value were reduced to control levels. No differences were noted between MS patients and controls in their response to alloantigens. The increased autologous MLR in patients with active MS appeared to result from an increased stimulatory capacity of non–T cells rather than from an intrinsically greater T cell proliferative potential. ACTH appeared to induce a change in the populations of circulating non–T cells such that these cells had a decreased stimulatory capacity in both autologous and allogeneic MLR. The disease in stimulatory MLR was, however, significantly greater than the decrease in allogeneic stimulatory capacity, suggesting a functional decrease of specific non–T cellenriched subpopulations. No significant changes in the numbers of myeloperoxidase‐positive (MP+) cells were noted in the blood of MS patients before and after ACTH therapy. Since the autologous MLR results in generation of cells that regulate immune responsiveness, the changes noted provide additional evidence for abnormal immune regulation in MS.
Chronic GM1 gangliosidosis presenting as dystonia: II. Biochemical studies
Authors: Kobayashi Takuro , Suzuki Kunihiko 
Published: January 2005

AbstractA patient with chronic GM1 gangliosidosis was studies enzymatically and biochemically. Leukocyte acid β‐galactosidase activity was severely deficient. In brain and liver, the 4‐methylumbelliferyl β‐galactosidase with acidic pH optimum and lactosylceramidase II were deficient while other hydrolases were present in normal amounts, including sialidase determined with N‐acetylneuramin‐lactose and fetuin as substrates. Nautral β‐galatosidase in liver was incerased up to fourfold over the control. Corresponding to the pathological findings, GM1 ganglioside sialic acid was increased in the basal ganglia to 57% of the total (normal, 12 to 16%), accounting for the rise in total ganglioside to 180% of normal in this region. Only slight to moderate elevations in the proportion of GM1 ganglioside were noted in the cerebral cortex and white matter, without major increase in total ganglioside. Elevated asislo GM1 ganglioside was also confined to the basal ganglia. There was no increase in hepatic glycoproteins or in keratan sulfate‐like materials. This is the only known patient with chronic GM1 gangliosidosis in whom abnormal accumulation of GM1 ganglioside has been demonstrated in affected tissue and sialidase deficiency has been excluded as the primary genetic defect.
Progressive neurological disease associated with chronic cytomegalovirus infection
Authors: Bray Patrick F. , Bale James F. , Anderson Robert E. , Kern Earl R. 
Published: January 2005

AbstractCytomegalovirus (CMV), the most common infectious cause of nervous system damage in the fetus and newborn infant, generally has been thought to exert its teratogenic effect during early pregnancy, and it has been assumed that clinical or postmortem abnormalities reflect static insults incurred earlier. Over the past two years we have identified two infants who presented with various clinical signs suggesting CMV infection. Serial CT head scans showed mild hydrocephalus progressing to extensive loss of brain substance and hydrocephalus in one and a normal scan going on to polycystic encephalomalacia in the other. Both infants had urine or leukocyte cultures that were repeatedly positive for CMV. These data suggest that CMV can cause progressive central nervous system damage after birth. The findings may account for some unexplained brain malformations and for a portion of the neurodegenerative disorders occurring in babies who appear normal at birth.
Myasthenia induced by monoclonal anti‐acetylcholine receptor antibodies: Clinical and electrophysiological aspects
Authors: Burres Steven A. , Crayton John W. , Gomez Christopher M. , Richman David P. 
Published: January 2005

AbstractTo investigate individual components of the humoral immune response directed against the acetylcholine receptor in experimental myasthenia, we injected monoclonal antireceptor antibody into normal rats. These antibodies were produced by cloned hybridoma lines formed by fusion of immune spleen cells with the myeloma cell line P3‐X63‐Ag8. Antibodies from two of seven clones induced acute experimental myasthenia, manifested by clinical weakness, decremental electromyographic response to repetitive nerve stimulation, and diminished mean amplitudes of miniature end‐plate potentials (mepps): 0.55 ± 0.02 mv (SEM) versus 0.71 ± 0.02 mv for controls (p < 0.001). In clinically affected animals, mepp amplitudes from the diaphragm correlated with decremental responses in the gastrocnemius muscle (r = −0.91, p < 0.01). The results suggest that binding of a single antibody species to a single determinant on the acetylcholine receptor molecule is sufficient to induce acute experimental myasthenia gravis.
Isoniazid‐induced elevation of CSF GABA levels and effects on chorea in huntington's disease
Authors: Manyam Bala V. , Katz Leonard , Hare Theodore A. , Kaniefski Kathryn , Tremblay Robert D. 
Published: January 2005

AbstractA randomized, double‐blind, crossover, placebo‐controlled clinical trial of oral isoniazid was undertaken in eight men with known Huntington's disease. Six completed the trial. Overall chorea scores indicated some amelioration, but clinical improvement was noticed in only two patients and was mild. Side effects included anorexia and elevation of liver enzyme levels. Cerebrospinal fluid (CSF) and plasma γ‐aminobutyric acid (GABA) concentrations were measured simultaneously. Mean CSF GABA increased threefold following treatment with isoniaid (414 ± 52 SEM pmol/ml) compared to placebo (120 ± 11 pmol/ml). No significant changes occurred in plasma GABA levels between the placebo and drug treatment phases. Reversal of central GABA deficiency appears not to correct extrapyramidal symptoms in Huntington's disease.
Iris pigmentation (melanin) in idiopathic dystonic syndromes including torticollis
Authors: Korein Julius 
Published: January 2005

AbstractIris pigmentation was evaluated in 153 Caucasian patients with torticollis and other focal, segmental, or generalized dystonias of unknown cause. Since these disorders are rare in non‐Caucasians, it was hypothesized that a relationship might exist between decreased melanin metabolism, reflected by iris pigmentation, and a genetic predisposition to these disorders of voluntary movement. Patients were separated into two groups on the basis of iris pigmentation. Compared to control groups, there was a statistically significant reversal in the ratio of patients with light eyes versus dark eyes. Further comparisons were made in two groups of patients with drug‐induced nonparkinsonian dyskinesias; no significant correlation was found between their dyskinetic syndrome and iris pigmentation. Data concerning race, ethnic origin, and other disorders of the basal ganglia support the hypothesis that there may be genetic predilection in individuals whose metabolic pathways produce less systemic melanin.
CSF changes during acute meningoencephalitis in mice caused by encephalomyocarditis virus
Authors: Griffin Diane E. 
Published: January 2005

AbstractEncephalomyelitis was induced in mice by intravenous inoculation with encephalomyocarditis virus. Examination of the cerebrospinal fluid revealed marked pleocytosis and increased amounts of protein, including immunoglobulins. These proteins entered the cerebrospinal fluid by transfer from serum during the period of central nervous system infection.
Multiple sclerosis: Capping of surface immunoglobulin G on macrophages engaged in myelin breakdown
Authors: Prineas J. W. , Graham J. S. 
Published: January 2005

AbstractMacrophages were examined for immunoglobulin G (IgG) and albumin in actively demyelinating lesions in two patients with multiple sclerosis (MS) using the peroxidase‐antiperoxidase immunocytochemical technique. In both cases, macrophages were present that stained for cytoplasmic or surface IgG or both. In one case, in which the tissue was rapidly fixed in chilled fixative, macrophages located among myelinated nerve fibers at plaque margins, but not elsewhere in the plaque, revealed surface IgG in the form of caps restricted to one or both poles of the cell. These caps were absent in sections stained for albumin. Because capping implies the presence of a multivalent ligand close to the cell surface and because cap formation was observed only in macrophages contacting myelin sheaths, we suggest that antimyelin antibody cytophilic for macrophages may be present in the central nervous system in MS, and that immune ligand‐mediated phagocytosis may play a role in myelin breakdown in the disease. This study provides the first direct evidence that IgG participates locally in myelin breakdown in MS.
Congenital hemihypertrophy: Oncogenic potential of the hypertrophic side
Authors: Furukawa Tetsuo , Shinohara Tamiko 
Published: January 2005

AbstractCongenital hemihypertrophy is a rare condition, occasionally associated with neoplasms on the affected side of the body. Investigation of three cases disclosed increased growth rate of cultured skin keratinocytes and fibroblasts and high skin temperature on the hypertrophic side. These observations suggest the oncogenic potential of the affected side in the condition.
Vitamin E deficiency in Werdnig‐Hoffmann disease
Authors: Shapira Yehuda , Amit Rami , Rachmilewitz Eliezer 
Published: January 2005

AbstractVitamin E levels were measured in the plasma of infants and children with various neuromuscular disorders. Seven of 8 infants with Werdnig‐Hoffmann disease (WHD) had a singificantly lower plasma vitmin E level (p < 0.01) than age‐matched normal controls, children with congenital myopathies, or children with muscular dystrophy. Vitamin E deficiency in WHD is not caused by malabsorption. A therapeutic trial of vtamin course of the disease. Vitamin E deficiency may play a role in the pathologenesis of WHD.
The incidence and prevalence of reported multiple sclerosis
Authors: Baum Herbert M. , Rothschild Beth B. 
Published: January 2005

AbstractA national survey, sponsored by the National Institute of Neurological and Communicative Disorders and Stroke, to determine the incidence, prevalence, and economic impact of multiple sclerosis has just been completed. These data are the first report of the results. Based on the data gathered, it is estimated that on Januaryuary 1, 1976, there were a reported 123,000 multiple sclerosis patients in the conterminous United States (a rate of 58 per 100,000). The annual incidence for the period 1970–1975 was estimated to be 8,800 (a rate of 4.2 per 100,000). The pattern of the disease being more common among females, whites, persons aged 30 to 50 years, and individuals living above the 37th parallel was also demonstrated. In addition to demographic characteristics, selected disease characteristics of the incidence and prevalence populations were also examined.
Peripheral nerve unmyelinated axons following lumbar sympathectomy
Authors: Chad D. , Shoukimas G. M. , Bradley W. G. , Bosch E. P. , Shahani B. T. , Young R. R. 
Published: January 2005

AbstractOne year after bilateral lumbar sympathectomy for Raynaud's syndrome, a young woman underwent fascicular biopsy of the sural nerve to help elucidate the cause of lower limb pain. The morphological features, density, and diameter spectrum of unmyelinated fibers were normal. The sympathetic nervous system appears to contribute few, if any, axons to the total population of unmyelinated axons in this cutaneous nerve.
Resetting of tremor by mechanical perturbations: A comparison of essential tremor and parkinsonian tremor
Authors: Lee Robert G. , Stein Richard B. 
Published: January 2005

AbstractWith the use of a computer‐controlled torque motor, experiments were carried out on 11 patients with essential tremor and 13 with parkinsonian tremor to determine the effect of mechanical displacements at the wrist joint on the established pattern of tremor. Analysis of the timing of tremor bursts in electromyographic recordings before and following the stimuli revealed that the phase of essential tremor could be readily reset by external perturbations. In the majority of the parkinsonian patients the same type of stimuli had very little effect on the pattern of tremor. Differences between predicted and actual times of occurrence of tremor bursts following the stimuli were used to calculate a normalized resetting index, with 0 representing no resetting and 1, complete resetting. For the patients with essential tremor the mean resetting index was 0.64 ± 0.14 (SD); for the parkinsonian patients the values were 0.16 ± 0.19. The results suggest that oscillations within spinal reflex loops play a major role in the generation of essential tremor. Reflex mechanisms are less important in parkinsonian tremor, which is more likely dependent on oscillations originating in the central nervous system.
Dependence of the achilles tendon reflex on the excitability of spinal reflex pathways
Authors: Burke David , McKeon Brian , Skuse Nevell F. 
Published: January 2005

AbstractMuscle afferent activity from the triceps surae was recorded during experimentally induced alterations in amplitude of the Achilles tendon jerk. No changes in the neural afferent response to tendon percussion or in the background level of neural activity occurred when the reflex response was altered by discomfort, distraction, changes in attention, or changes in the rate of tendon percussion. Reinforcement of the Achilles tendon jerk by forceful contraction of the forearm muscles did not alter the relationship between intensity of the tendon tap and amplitude of the evoked neural afferent volley. Nevertheless, such maneuvers lowered the reflex threshold and raised reflex sensitivity so that a smaller afferent volley was required to produce a tendon jerk, and an increase in the afferent volley produced a disproportionately greater increase in reflex electromyographic activity than would have occurred at rest. Reinforcement maneuvers potentiated the H‐reflex but did not alter the electrically induced afferent volley or the background level of neural activity. It is concluded that these changes in reflex responsiveness occurred through intrinsic spinal mechanisms independent of the fusimotor system.
Myoclonus and ocular oscillations induced by L‐tryptophan
Authors: Baloh Robert W. , Dietz John , Spooner Joseph W. 
Published: January 2005

AbstractA patient with chronic manic‐depressive illness developed generalized myoclonus and spontaneous ocular oscillations after a single 2 gm dose of L‐tryptophan. She had been pretreated with both a tricyclic antidepressant and a monoamine oxidase inhibitor. The involuntary movements gradually disappeared within 24 hours after the drugs were discontinued. Electrooculographic recording 7 hours after onset of the abnormal eye movements revealed square‐wave jerks and hypometric voluntary saccades. Pursuit as well as optokinetic and vestibular slow phases were normal except for superimposition of the square‐wave jerks. Repeat recording 24 hours later was entirely normal.
Local cerebral glucose metabolism in newborn dogs: Effects of hypoxia and halothane anesthesia
Authors: Duffy T. E. , Cavazzuti M. , Cruz N. F. , Sokoloff L. 
Published: January 2005

AbstractLocal cerebral glucose utilization (LCGU) was measured in 36 neuroanatomical structures of normal awake, halothane‐anesthetized, and hypoxic newborn puppies by the autoradiographic 2‐[14C]deoxyglucose method. In normal animals, LCGU was highest in the vestibular nucleus and in other gray matter nuclei of the brainstem and declined in a caudal‐to‐rostral progression through the neuraxis (i.e., LCGU of cerebellum > thalamus ≅ caudateputamen > cerebral cortex). Lowest rates of glucose metabolism were detected in white matter structures. Halothane anesthesia (1.5% inspired) caused few changes in local glucose metabolism, the most notable being decreased LCGU among structures of the auditory system (cochlear nucleus, lateral lemniscus, inferior colliculus) and increased LCGU in the interpeduncular nucleus. Acute systemic hypoxia (arterial oxygen tension of approximately 12 mm Hg) produced markedly heterogeneous effects on local glucose metabolism: LCGU was increased in some gray matter structures, decreased in the thalamus, and substantially increased in the subcortical white matter and corpus callosum. In puppies whose brains were frozen in situ after 55 minutes of hypoxia, the concentration of lactate was increased ten‐ to elevenfold in cortical gray and subcortical white matter, but the concentrations of glucose, adenosine triphosphate, and phosphocreatine declined to a greater extent in the white matter. The results suggest that during hypoxia the high rate of glycolysis in white matter exceeded substrate supply so that glucose availability became the limiting factor for local energy production. Such a mechanism may contribute to the white matter injury that often develops following hypoxic‐ischemic insults in the perinatal period.
Nevoid basal cell carcinoma syndrome and epilepsy
Authors: Murphy Martin J. , Tenser Richard B. 
Published: January 2005

AbstractFour patients affected with the nevoid basal cell carcinoma syndrome and recurrent seizures are described and the pertinent literature is reviewed. Three of the patients had multiple basal cell carcinomas and cutaneous nevi, dentigerous cysts, and skeletal anomalies characteristic of the syndrome. The seizures had their onset in childhood and were generalized tonic‐clonic in three patients and both tonic‐clonic and complex partial in the fourth. Focal neurological deficits were found in only one patient. Pneumoencephalography in two patients and head CT scan in a third were negative. Electroencephalography showed focal spike discharges in one patient, bilateral anterior paroxysmal slowing in another, and nonspecific shifting temporal theta transients in the other two cases. Psychometric testing yielded full‐scale scores ranging from 39 to 84. In addition to mental retardation, congenital communicating hydrocephalus, and medulloblastoma, seizures should be included among the neurological manifestations of the nevoid basal cell carcinoma syndrome.