bluuebunny/crossref_metadata_2025_split huggingface dataset:
Primitive reflexes and cognitive function in the elderly
Authors: Koller William C. , Glatt Sander , Wilson Robert S. , Fox Jacob H. 
Published: January 2005

AbstractPrimitive reflexes were studied in patients with presumed Alzheimer disease and in age‐matched nondemented controls. A snout reflex occurred with equal frequency (54%) in the two groups. An abnormal glabellar reflex was elicited more often in the demented patients (23%) than the controls (8%). The presence of a snout reflex was directly correlated with increasing age; the glabellar reflex showed a similar but nonsignificant trend. Neither reflex finding correlated with the presence of apparent cerebral atrophy by CT scan or the results of psychometric tests. The finding of primitive reflexes has little clinical value in the evaluation of the elderly.
Local cerebral glucose utilization in the newborn macaque monkey
Authors: Kennedy C. , Sakurada O. , Shinohara M. , Miyaoka M. 
Published: January 2005

AbstractThe [14C]dexyglucose method for quantitative determination of local cerebral glucose utilization was employed in newborn macaque monkeys. Values ranged from a high of 180 μmol/100 gm/min in the inferior colliculus to a low of 14 μmol/100 gm/min in the centrum ovale, a wider range than had been found in this laboratory for pubescent monkeys. Relatively low values in white matter overlapped with the generally higher values in gray matter. Rates for structures in the lower neuraxis were equal to or exceeded those reported for the mature animal, while those above the midbrain were generally lower. The auditory system was unique in having rates equal to, or exceeding, its mature levels in all parts of the pathway including the cortex. In the visual system, subcortical structures were at their mature levels while rates in the cortical areas were variably low; nevertheless, the striate cortex had differential rates marking the various laminae. It was possible to demonstrate the metabolic counterpart of the ocular dominance columns in two newborn animals that had had one eye occluded during the experiment. Rates for white matter were above their mature levels, with the differences being greatest in the brainstem and cerebellum. A high correlation between glucose utilization and local blood flow measured with [14C]antipyrine (r = 0.92) was found.
Dopaminergic dysfunction in tourette syndrome
Authors: Singer Harvey S. , Butler Ian J. , Tune Larry E. , Seifert William E. , Coyle Joseph T. 
Published: January 2005

AbstractA prospective clinical and biochemical study on the effects of treatment with haloperidol has been performed in seven patients with Tourette syndrome. Pretteatment cerebrospinal fluid levels of homovanillic acid (CSF HV A) were significantly reduced in all patients, whereas 5‐hydroxyindoleacetic acid was reduced in only two. With haloperidol treatment, symptoms decreased in all cases (21 to 88%) and clinical improvement was associated withan increased level of CSF HV A, often returning to the normal range. Optimal therapeutic response was found with serum levels of haloperidol between 1 and 4 ng/ml; however, disturbing side effects also occurred within this range. These results support the hypothesis that Tourette syndrome may result form a supersensitivity of dopaminergic receptors.
Locked‐in syndrome in multiple sclerosis with sparing of the ventral portion of the pons
Authors: Forti Alberto , Ambrosetto Giovanni , Amore Mario , De Maria Roberto , Michelucci Roberto , Omicini Ebe , Rizzuto Nicola , Fenzi F. , Tassinari Carlo Alberto 
Published: January 2005

Abstract“Locked‐in” syndrome was observed in a 42‐year‐old woman with multiple sclerosis. Autopsy revealed extensive demyelination in both cerebral peduncles, the periaqueductal region, and the internal capsule, but the ventral portion of the pons was spared.
Inflammatory facioscapulohumeral muscular dystrophy and coats syndrome
Authors: Wulff John D. , Lin James T. , Kepes John J. 
Published: January 2005

AbstractWe report a case of facioscapulohumeral muscular dystrophy and Coats syndrome. This association has been reported only once before. Three muscle biopsies from our patient over 13 years showed changes of a slow inflammatory myopathy. Corticosteroid treatment did not improve his clinical status. We postulate that our case may be a variant of facioscapulohumeral muscular dystrophy.
Neurobiological correlates of learning disabilities
Authors: Golden Gerald S. 
Published: January 2005

AbstractMost investigator now agree on the existence of abnormalities of brain function in children with learning disabilities with or without hyperactivity. However, attempts to define the neurobiological substrates have been hindered by many factors. On the clinical level, learning disability is not a monolithic entity but an operational term used to define a number of specific conditions. The only common feature is difficulty with school performance in the face of normal intelligence. Until homogeneous subgroups can be defined, there is no good reason to suspect a single, discrete biological marker.Neuropathological studies are limited to 2 cases. Attempts to visualize the brain by computerized tomography have provided limited but inconsistent evidence of variations from normal hemispheric widths and asymmetries. Neurophysiological studies have succeeded in separating learning disabled children from normal controls by the application of discriminant analysis and other statistical techniques. Computer‐assisted analysis of brain bioelectrical activity has sharpened these tools. Unfortunately, the techniques have not been able to narrow the focus to single, discrete cerebral areas; rather, they have shown dysfunction in widely separated but probably interconnected regions.The only neurochemical studies available have been performed on hyperactive childŕen rather than on patients with uncomplicated learning disabilities. A hypothesis that the underlying pathophysiology involves dopamine depletion has the most support and provides an explanation for some of the therapeutic effects of psychostimulant drugs. Problems are the focus on motor aspects of hyperactivity rather than the attention deficit and heavy dependence on the use of clinical rating scales. The biochemical analyses and conclusions would be more powerful if better psychophysiological markers could be developed, including such factors as attentiveness, distractability, vigilance, attention span, and reaction time.The results of physical and neurological examinations as well as of clinical genetic studies also point toward a biological basis for learning disabilities. These clinical tools help to sharpen our focus but still provide insufficient specificity. Productive search will require close collaboration between neuropsychologists and investigators using the quantitative tools of neuroanatomy, neurophysiology, and neurochemistry.
Imparied biotin status in anticonvulsant therapy
Authors: Krause Klaus‐Henning , Berlit Peter , Bonjour Jean‐Pierre 
Published: January 2005

AbstractIn 264 epileptics undergoing long‐term therapy with anticonvulsants, significantly reduced plasma biotin levels were found compared with a normal control group: 74% of the epileptics had biotin levels for those treated with sodium valproate were higher than for those treated with phenytion, primidone, or carbamazepine. The observed reduction in biotin levels might be a factor influencing the efficacy of these three antionvulsants.
The origin of fasciculations
Authors: Roth G. 
Published: January 2005

AbstractA collision method in a single axon was used to determine the origin of 100 fasciculations in various lower motor neuron lesions. For 80% of the fasciculations, it was situated on the distal extremity of the axon regardless of the type of lesion, its duration, or the severity of the denervation. For several reasons, this proportion is underestimated.The fasciculations of distal origin were sometimes associated with double discharges, the mechanism of production and clinical significance of which probably differ.
Evoked potential abnormalities in the various inherited ataxias
Authors: Nuwer Marc R. , Perlman Susan L. , Packwood James W. , Kark R. A. Pieter 
Published: January 2005

AbstractVisual (VEP), brainstem auditory (BAEP), and somatosensory (SEP) evoked potential tests were performed in 45 patients representing ten types of inherited disorders in which ataxia was the most prominent symptom. Comparable VEP abnormalities were present among all types of patients. Normal BAEP tests were recorded in most patients except those with olivopontocerebellar atrophy. SEP results were often more severely abnormal in patients with Friedreich's ataxia. The observations emphasize the similarity in expression of different metabolic‐degenerative disorders. When these tests are used clinically, certain features of evoked potentials (especially left‐right symmetry) are typical of the inherited ataxias as a group. Few distinguishing features differentiate the individual disorders.
Blood flow changes in arteriovenous malformation during behavioral activation
Authors: Deutsch Georg 
Published: January 2005

AbstractStriking task‐dependent fluctuations were observed in the cerebral blood flow pattern of a patient with a left posterior hemispheric arteriovenous malformation (AVM). Two‐dimensional measures of regional cerebral flwo in the resting state, using the xenon 133 inhalation technique, revealed a region of high flow coincident with the AVM seen on the patient's arteriograms. In subsequent studies, the AVM stood out as a region of high blood flow during a relaxed state, while it approached normal levels of flow when there was attentional demand. These observations suggest that focal regulatory mechanisms exist at the AVM or else that very substantial redistributions of blood flow are taking place which the flow rate in the AVM reflects only passively. Patients considered for embolic treatment of an AVM would benefit from an assessment of behavioral influences on flow in the AVM.
Bromocriptine and lisuride in Parkinson disease
Authors: Lieberman Abraham N. , Gopinathan Govindan , Neophytides Andreas , Leibowitz Morton , Walker Russell , Hiesiger Emil 
Published: January 2005

AbstractLisuride was compared with bromocriptine in 25 parkinsonian patients in whom the response to levodopa had diminished; 19 had “wearing off,” “on‐off” phenomena, or both. At the time bromocriptine was added to levodopa, the mean age of the patients was 62.7 years and mean disease duration was 8.9 years. Disability decreased by 34% in the on period and by 20% in the off period, and the number of hours the patients were on increased from 9.6 to 12.8. All these changes were significant (p ≤ 0.01 to 0.05). Bromocriptine, however, had to be discontinued in 11 patients because of adverse effects. In the remaining 14 patients, bromocriptine was eventually discontinued because of decreased efficacy. Mean dose of bromocriptine was 55 mg (range, 20 to 100 mg).At the time lisuride was added to levodopa the patients were older (65.4 years), had had the disease longer (11.4 years), and were more disabled. Nonetheless, disability decreased in the on period by 33% and in the off period by 17%, and the number of hours the patients were on increased from 3.9 to 8.9. All these changes were significant (p ≤ 0.01 to 0.05). The mean dose of lisuride was 2.8 mg (range, 0.6 to 5.0 mg). Lisuride was discontinued in 8 patients because of adverse effects.Both bromocriptine and lisuride are useful in managing patients with advanced Parkinson disease whose response to levodopa has diminished. While it is presently not possible to state which of the drugs is more effective, ultimately their usage will probably be determined by their relative cost.
Relapsing herpes simplex encephalitis following antiviral therapy
Authors: Davis Larry E. , McLaren Laroy C. 
Published: January 2005

AbstractAlthough treatment for herpes simplex virus (HSV) encephalitis with antiviral agents has improved survival, occasional patients experience unexplained clinical exacerbations. This report presents evidence that some relapses may occur from recurrent viral encephalitis.An adult male developed the classic symptoms of HSV encephalitis. The cerebrospinal fluid (CSF), electroencephalogram, and isotope brain scan suggested a localized encephalitis involving the left temporal lobe. The pateint was treated for 10 days with high doses of cytosine arabinoside instead of the currently recommended adenine arabinoside. Progression of encephalitis stopped, and clinical recovery occurred. The HSV antibody titer increased eightfold. Fifty‐four days after the initial encephalitis, the patient relapsed with a subacute progressive encephalitis involving the same brain area. The CSF demonstrated oligoclonal bands, elevated immunoglobulin G levels (100 mg/dl), and a high HSV antibody titer (1:8,192 by indirect hemagglutination test). From a left temporal lobe biopsy taken 74 days after onset of the initial encephalitis, herpes simplex virus type 1 was isolated. Without renewed antiviral drug therapy, the patient slowly recovered.
Case of downbeat nystagmus influenced by otolith stimulation
Authors: Chambers B. R. , Ell J. J. , Gresty M. A. 
Published: January 2005

AbstractA patient with downbeat nystagmus and familial ataxia is described. The nystagmus was induced by static tilt away from normal upright posture, by linear acceleration of the head, and by convergence. It is inferred that the nystagmus was modulated by otolith‐specific stimuli and not by stimulation of the canals. These findings demonstrate the role of otolith function in generation of eye movements in the vertical plane and support proposed interrelationships between otolith and vergence mechanisms. The nystagmus and associated oscillopsia were partially suppressed by treatment with clonazepam.
Basal forebrain neurons in the dementia of Parkinson disease
Authors: Whitehouse Peter J. , Hedreen John C. , White Charles L. , Price Donald L. 
Published: January 2005

AbstractDemented patients with Parkinson disease share certain neuropathological and neurochemical features with patients suffering from Alzheimer disease. Recently, loss of cholinergic neurons in the basal forebrain, particularly the nucleus basalis of Meynert, has been implicated in the pathophysiology of Alzheimer disease. The present investigation of 12 patients with Parkinson disease demonstrates that the demented patients with this disease also show a selective loss of cells in the nucleus basalis of Meynert, thus providing an important link between the dementias of Alzheimer disease and Parkinson disease.
Chronic experimental allergic encephalomyelitis produced by bovine proteolipid apoprotein: Immunological studies in rabbits
Authors: Cambi Franca , Lees Marjorie B. , Williams Rosemarie M. , Macklin Wendy B. 
Published: January 2005

AbstractA chronic experimental allergic encephalomyelitis (EAE) has been produced in rabbits sensitized with bovine white matter proteolipid apoprotein. Eleven of 12 animals developed clinical disease one to six months after immunization with a single dose of the apoprotein. The clinical course was characterized by posterior ataxia, flaccid paralysis progressing to spastic paralysis, and incontinence. Spontaneous relapses and remissions were observed in 3 rabbits. Histologically, acute and chronic encephalomyelitis accompanied by primary demyelination were observed. Serumantibody production, assayed by both an enzyme‐linked immunosorbent assay and an electroblot procedure, did not correlate with either the clinical course or the histopathological findings. Delayed hypersensitivity to proteolipid apoprotein was observed in all rabbits prior to the onset of clinical signs. The data suggest that lymphocytes specifically sensitized to the proteolipid may be involved in the pathogenesis of the demyelination in chronic EAE.
Cerebral emboli of paradoxical origin
Authors: Jones H. Royden , Caplan Louis R. , Come Patricia C. , Swinton Neil W. , Breslin Donald J. 
Published: January 2005

AbstractA diagnosis of paradoxical cerebral embolus (PCE) was made in five patients aged 31 to 62 years who sustained eight cerebral ischemic events. No patient had evidence of primary carotid system or left heart disease. A probe‐patent foramen ovale was the presumed mechanism in four patients, and an unsuspected congenital atrial septal defect was found in the fifth patient. Clinically apparent pulmonary emboli or venous thrombosis preceded the cerebral event in only one instance.Review of the literature reveals a high mortality with PCE. However, careful clinical search for this lesion may be rewarding: four of our five patients survived. One should consider PCE in any patient with cerebral embolus in whom there is no demonstrable left‐sided circulatory source. This principle applies particularly if there is concomitant venous thrombosis, pulmonary embolism, or enhanced potential for venous thrombosis due to, for example, morbid obesity, use of hormonal birth control pills, prolonged bed rest (especially postoperatively), or systemic carcinoma.
Friedreich ataxia and low pyruvate carboxylase activity in liver and fibroblasts
Authors: Dijkstra U. J. , Willems J. L. , Joosten E. M. G. , Gabreëls F. J. M. 
Published: January 2005

AbstractBiochemical studies in liver, muscle, and cultured fibroblasts were carried out in seven patients with Friedreich ataxia. Lowered activity of pyruvate carboxylase was shown in liver and cultured fibroblasts in all instances.
Normal vitamin E status in spinal muscular atrophy
Authors: Sokol Ronald J. , Iannaccone Susan T. 
Published: January 2005

AbstractIn an attempt to confirm prior reports of vitamin E deficiency in Werdnig‐Hoffmann disease, we assessed vitamin E nutritional status in 11 children and 6 adults with spinal muscular atrophy (SMA). Serum vitamin E levels (10.6 ± 3.3 m̈g/ml for those less than 12 years old, 13.2 ± 4.5 for those older than 12) and the ratio of serum vitamin E to total serum lipids were normal in all patients. Vitamin E nutritional status appears to be normal in SMA, and vitamin E therapy is not indicated.
Incidence and treatment of peripheral venous thrombosis in patients with glioma
Authors: Ruff Robert L. , Posner Jerome B. 
Published: January 2005

AbstractThe incidence, prevention, and treatment of peripheral venous thrombosis were studied retrospectively in 381 patients with malignant glioma. Of 264 patients who did not receive antithrombotic prophylaxis, 97 (36.7%) developed clinical phlebitis confirmed by venography. Sixty‐six cases occurred within 6 weeks of craniotomy. By contrast, only 12 (10%) of 117 patients who received intermittent pneumatic pressure to the calves during cranictomy developed phlebitis (4 patients within 6 weeks of the surgery). Of the 109 patients with venous thrombosis, 103 were treated with anticoagulants. Of the 6 patients treated conservatively, 3 died of pulmonary emboli. Intracranial hemorrhage occurred in 1.9% of the patients taking anticoagulants and in 2.2% of those who did not develop phlebitis. We conclude that patients with malignant gliomas have a high risk of developing peripheral venous thrombosis; that antithrombotic therapy reduces the incidence of thrombosis following craniotomy; and that, in patients who develop phlebitis, anticoagulation reduces the risk of pulmonary emboli without increasing the risk of intracranial hemorrhage.
β‐Galactosidase–neuraminidase deficiency (galactosialidosis): Clinical, pathological, and enzymatic studies in a postmortem case
Authors: Sakuraba Hitoshi , Suzuki Yoshiyuki , Akagi Masao , Sakai Masao , Amano Naoji 
Published: January 2005

AbstractThree male siblings in a Japanese family were affected with β‐galactosidase–neuraminidase deficiency (galactosialidosis). One patient died at 45 years of age, and postmortem liver and brain tissues were studied enzymatically. The residual activity of neuraminidase was relatively high in these tissues. Neuraminidase activity did not change in the tissues after repeated freezing and thawing of the homogenates, whereas this enzyme in control tissues lost 30 to 60% of its activity. There was a profound deficiency of β‐galactosidase in the tissues of the patient. Michaelis constant, pH profiles, and cryostability were identical for the patient and control patients. Cathepsin B was moderately increased in activity in the patient's tissues.
Segmental demyelination and remyelination in lumbar spinal root of patients dying with diabetes mellitus
Authors: Ohnishi Akio , Harada Masayoshi , Tateishi Jun , Ogata Jun , Kawanami Sachiko 
Published: January 2005

AbstractMorphometric evaluations of histopathological changes in postmortem materials from three patients with diabetes mellitus with neuropathy, nephropathy, and retinopathy were made on the sural nerve, lumbar spinal roots, lumbar dorsal root ganglion (2 cases), and fasciculus gracilis. In all three patients there was a marked decrese in densities of both the large and the small myelinated fibers in the sural nerves. In the lumbar spinal roots, segmental demyelination and remyelination with or without decrease in the number of myelinated fibers per root was the main finding in both dorsal and ventral roots, being more common in the dorsal roots. At the third cervical segment of the the fasciculus gracilis, the myelinated fiber density was slightly decreased in one patient and moderately decreased in the other two. The number of cell bodies of the fifth dorsal root ganglion was within normal limits in the two patient where this was examined, although the median diameters approximated the lower limit found in controls. The potential presence of segmental demyelination and remyelination in dorsal and ventral spinal roots should be considered especially when assessing electromyographic changes and nerve conduction in diabetic patients.
Computed tomography in acute disseminated encephalomyelitis
Authors: Lukes Steven A. , Norman David 
Published: January 2005

AbstractComputed tomographic (CT) scans were obtained for eleven patients with acute disseminated encephalomyelitis (ADE). Four patients had normal CT scans despite repeated examination. Abnormalities were found in seven patients and included cortical‐enhancing lesions, low‐density lesions in the deep white matter and basal ganglia, and edema of the brainstem. These findings support the hypothesis that both vascular injury and demyelination are involved in the pathogenesis of ADE. A delay between the onset of clinical signs and the appearance of lesions on CT scan was common. Clinical improvement was accompanied by improvement in CT abnormalities. There was a limited correlation between the clinical course and the anatomical distribution and type of abnormality seen on CT scan.
Downbeat nystagmus with a pseudocycloid waveform: Improvement with base‐out prisms
Authors: Lavin P. J. M. , Traccis S. , Dell'Osso L. F. , Abel L. A. , Ellenberger C. 
Published: January 2005

AbstractDownbeat nystagmus in primary position and oscillopsia resulted from nutritional deficiency during prolonged intravenous therapy of a patient with hyperemesis gravidarum. Wide bandwidth infrared oculography demonstrated a pseudocycloid nystagmus waveform with an increasing‐velocity exponential slow phase. Because the oscillopsia decreased and the nystagmus was damped with convergence, visual acuity improved with the addition of base‐out prisms to each spectacle lens.