bluuebunny/crossref_metadata_2025_split huggingface dataset:
Chronic leigh disease: A genetic and biochemical study
Authors: Plaitakis Andreas , Whetsell William O. , Cooper Jack R. , Yahr Melvin D. 
Published: January 2005

AbstractThe large family of a 21‐year‐old man who died of Leigh disease was investigated for evidence of neurological abnormalities and presence of the adenosine triphosphate‐thiamine diphosphate phosphoryltransferase inhibitor factor. Of 217 persons (seven generations) included in the pedigree, 68 were examined neurologically and biochemically. Fourteen (20%), 5 of whom had abnormal neurological findings, were found to excrete the inhibitor factor. Clinical manifestations varied from severe neurological affliction to subtle deficits. A chronic relapsing course was frequently encountered, with exacerbations occurring in association with apparent metabolic stress. Parental consanguinity was identified in the propositus as well as in other family members with neurological abnormalities. Males and females were affected, and no vertical transmission of the trait was found. These multigenerational data suggest that Leigh disease in adults is inherited in an autosomal recessive manner and has variable degrees of expression with a wide spectrum of neurological manifestations.
Hereditary spinal arachnoid cysts, distichiasis, and lymphedema
Authors: Schwartz James F. , O'Brien Mark S. , Hoffman James C. 
Published: January 2005

AbstractFamilial congenital spinal arachnoid cysts causing progressive paraplegia are reported in two adolescent siblings as part of a hereditary syndrome. The other features of this unusual dominantly inherited disorder include double rows of eyelashes, partial ectropion of the lower eyelids, and acquired late‐onset lymphedema of the lower extremities. The siblings' mother, who had roentgenographic evidence of a similar intraspinal lesion, was free of neurological symptoms but had the other characteristic features. Surgical treatment of the arachnoid cysts improves the progressive paraplegia.
The acute sensory neuronopathy syndrome: A distinct clinical entity
Authors: Sterman Arnold B. , Schaumburg Herbert H. , Asbury Arthur K. 
Published: January 2005

AbstractFour to twelve days following initial antibiotic treatment for a febrile illness, three adults suddenly experienced numbness and pain over the face and entire body. Each had received a penicillin or a semisynthetic derivative, and two patients also received other antibiotics. Signs appeared rapidly and included profound sensory ataxia, areflexia, and widespread sensory loss, primarily of large fiber modalities (proprioceptive sensibility). Slowed or absent sensory conduction was found. There was no weakness, and electrical study of muscle and motor nerve conduction was normal in all. The cerebrospinal fluid was acellular, and protein levels were elevated to 126 and 175 mg/dl in two cases and were normal in the other. Presently, all have a severe, static, residual sensory deficit. During follow‐up of five years, no evidence of neoplastic disease or immunological disorder has appeared.Because of the rapid onset, widespread and pure sensory involvement, and poor recovery, the lesion is most likely confined to the dorsal root and gasserian ganglia (sensory neuronopathy). This pattern resembles that of the experimental lesions induced by doxorubicin and pyridoxine. It appears likely that either the previously administered antibiotics or the illness for which they were administered were of pathogenetic importance. We designate this previously unrecognized disorder the acute sensory neuronopathy syndrome and suggest that it represents a distinct, readily identifiable clinical entity.
Reversed Optokinetic Nystagmus (OKN): Mechanism and Clinical Significance
Authors: Halmagyi G. M. , Gresty M. A. , Leech J. 
Published: January 2005

AbstractWe studied reversed optokinetic nystagmus (OKN) in 31 patients and found that it is actually the patient's own gaze‐modulated spontaneous nystagmus shifted to the primary position of gaze by optokinetic stimulation. Two of our 31 patients were known to have congenital nystagmus. Most of the 29 others were adults who presented with neurological symptoms and nystagmus and were therefore suspected of having posterior fossa lesions. However, their nystagmographic findings and benign natural history suggest that they also had congenital nystagmus, which was unrecognized until they developed incidental neurological symptoms. These cases support the view that reversed OKN is pathognomonic of a benign, probably congenital, nystagmus.
Cerebral microvessel 2‐deoxy‐D‐glucose uptake during ischemia‐induced seizures
Authors: Nell Janet H. , Welch K. M. A. 
Published: January 2005

AbstractMicrovessels were isolated by albumin flotation and glass bead filtration from cerebral cortices of gerbils decapitated during petylenetetrazol (PTZ)‐induced seizures and ischemia‐induced seizures. The uptake of 2‐deoxy‐D‐glucose (2‐DG) into these microvessels was studied in vitro. This uptake was increased during PTZ‐induced seizures, in keeping with mechanisms whereby glucose transport into brain is increased to keep pace with the augmented tissue glycolysis proveked by enhanced neuronal activity. Uptake of 2‐DG was decreased in microvessels isolated from animals that were decapitated while undergoing a seizure during ischemia and 90 to 120 minutes after ischemia. In contrast to results obtained when seizures are due to other causes, these results suggest that brain glucose transport is decreeased during a seizure if the microvasculature has been previously subjected to ischemia. The results are discussed in relation to clinical stroke and Todd's postepileptic paralysis.
Memory enhancement in Korsakoff's psychosis by clonidine: Further evidence for a noradrenergic deficit
Authors: McEntee William J. , Mair Robert G. 
Published: January 2005

AbstractThree drugs, d‐amphetamine, clonidine, and methysergide, which presumably enhance central noradrenergic activity by different pharmacological mechanisms, were administered to eight patients with the Korsakoff syndrome in a two‐week subacute, double‐blind, counterbalanced experiment to study the effects of these agents on memory function as measured by a neuropsychological test battery. Of the drugs tested, only clonidine, a putative alphanoradrenergic agonist, was associated with significant improvement in memory. The data are consistent with the hypothesis that damage to ascending norepinephrine‐containing neurons in the brainstem and diencephalon may be the basis for amnesia in Korsakoff's psychosis.
Normal human sleep: Regional cerebral hemodynamics
Authors: Sakai Fumihiko , Meyer John Stirling , Karacan Ismet , Derman Sabri , Yamamoto Masahiro 
Published: January 2005

AbstractMeasurements of regional cerebral blood flow (rCBF) by the xenon 133 inhalation method along with polygraph recordings were made serially during relaxed wakefulness and different stages of nocturnal sleep in 18 righthanded normal volunteers. During stage I‐II sleep the fast flow (Fg) values declined significantly, more in the brainstem‐cerebellar (BSC) regions than in hemispheric regions. During stage III‐IV sleep, Fg further declined diffusely in both hemispheric (‐28%) and BSC (‐29%) regions. Duringg awakening from stage IV sleep to alpha‐frequency wakefulness, BSC flow values increased more than hemispheric flow values. During REM sleep, regional Fg values increased diffusely in both hemispheric (+41%) and BSC (+47%) regions compared with wakefulness. There was a significant inverse correlation between the increase in end‐tidal partial pressure for carbon dioxide and the reduction in bihemispheric Fg during sleep. Cerebral vasomotor responsiveness to carbon dioxide is decreased during both REM and non‐REM sleep.
Characterization of clinical CSF dynamics and neural axis compliance using the pressure‐volume index: I. The normal pressure‐volume index
Authors: Shapiro K. , Marmarou A. , Shulman K. 
Published: January 2005

AbstractThe pressure‐volume index (PVI) technique was used to measure neural axis compliance, cerebrospinal fluid (CSF) formation (If), and CSF absorption (Ro) in 23 children and 7 adults, all free from intracranial masses, who were undergoing diagnostic or therapeutic procedures. Using bolus manipulation of CSF, If was 0.36 ± 0.08 ml/min and Ro was 2.8 ± 0.8 mm Hg/ml/min in both adults and children. PVI, as a measure of neural axis compliance or volume buffering capacity, was 25.9 ± 3.7 ml for the adults. Measured PVI in the children varied from 8.2 to 30.1 ml but correlated well (r = 0.93) with predicted PVI based on estimates of intracranial and spinal volumes obtained from external measurements. This study provides normal reference data in humans for assessing CSF hydrodynamics and neural axis compliance using the PVI technique in pathological settings.
Masticatory spasm in facial hemiatrophy
Authors: Kaufman Michael D. 
Published: January 2005

AbstractSpontaneous spasms began in the left masseter muscle six years following the onset of facial hemiatrophy in a young woman. A dental procedure had preceded the original facial wasting by four weeks. The electromyographic findings were similar to those described in hemifacial spasm and consisted of brief bursts of one to four morphologically different potentials, each firing at rates of up to 200 Hz. The findings demonstrate that spontaneous activity due to nerve injury can arise in muscles innervated by the trigeminal nerve.
Age effect on dopamine and serotonin metabolite levels in cerebrospinal fluid
Authors: Seifert William E. , Foxx Jerry L. , Butler Ian J. 
Published: January 2005

AbstractHomovanillic acid (HVA), 5‐hydroxyindoleacetic acid (5‐HIAA), and 3‐methoxy‐4‐hydroxyphenylethylene glycol (MHPG) were measured in lumbar cerebrospinal fluid from a group of patients ranging in age from 1 week to 45 years. Quantitation of these biogenic amine metabolites was achieved using a gas chromatographic/mass spectrometric technique. The subjects had various specific disorders of the nervous system, though patients with movement disorders and biochemical defects known to affect the neurotransmitter systems examined in this study were specifically avoided.The results indicated a strong inverse correlation in children between CSF HVA and 5‐HIAA concentrations and age. The decline in these metabolites with age appeared to be exponential. No significant age effect was observed for MHPG. The results indicate the importance of comparing CSF metabolite levels in children with values in age‐matched controls.
Benzodiazepine receptor interactions may be involved in the neurotoxicity of various penicillin derivatives
Authors: Antoniadis Adam , Müller Walter E. , Wollert Uwe 
Published: January 2005

AbstractThe interaction of seven penicillin derivatives with specific [3H]flunitrazepam binding to benzodiazepine receptors was investigated. The affinities of the penicillins for benzodiazepine receptor seemed to depend on the lipophilia of the derivatives. The concentrations of the penicillins which inhibit specific [3H]flunitrazepam binding are consistent with penicillin levels found in the central nervous system of patients developing penicillin‐induced convulsions. The results suggest that penicillins inhibit GABAergic transmission not only at the GABA receptor, but also at the benzodiazepine receptor, which is thought to be part of a neuronal system facilitating GABAergic transmission. Both mechanisms may account at least in part for the convulsive and neurotoxic properties of penicillins.
Cerebellar softening
Authors: Scotti Giuseppe , Spinnler Hans , Sterzi Roberto , Vallar Giuseppe 
Published: January 2005

AbstractTwenty‐one adults with acute cerebellar softening confirmed by computerized tomography (CT) were divided into two groups according to clinical course, CT findings, and outcome. In the first group, cosisting of 6 patients, the condition ran a progressive courses, with deterioration of consciousness, signs of brainstem compression, and the appearance of hydrocephalus on CT scan. Three patients underwent surgery successfully, 2 died postoperatively, and 1 recovered spontaneously. In the second group, comprising 15 patients, the course was benign: cerebellar symptoms and signs improved without surgery, and only discrete cerebellar signs remained a month after onset.
Basal lamina formation at the site of spinal cord transection
Authors: Feringa Earl R. , Kowalski Timothy F. , Lee Vahlsing H. 
Published: January 2005

AbstractThe pia‐glial basal lamina (BL) at the site of spinal cord injury could be an important physical impediment to central nervous system regeneration. We used an epithelial BL‐specific immunohistochemical stain to determine the location of the pia‐glial BL after spinal cord transection.Small segments of BL were found at the margin of the lesion 5 days after transection. After 10 days, longer and more numerous segments were seen. At 20 days, the entire transected end of the spinal cord was capped by a layer of BL.
Muscle metals in motor neuron disease
Authors: Pierce‐Ruhland Richard , Patten Bernard M. 
Published: January 2005

AbstractTo investigate the role of metallic elements in motor neuron disease (MND), we used a photon‐excited, energy‐dispersive x‐ray analytical system to measure the metal content of muscle biopsies from 21 patients with MND and compared the results with those obtained from biopsies from neurologically diseased control patients matched for age, sex, and muscle biopsied.By t test analysis, the MND patients did not differ significantly from controls in the average muscle biopsy concentrations of lead, mercury, arsenic, manganese, aluminum, or any of the other ten elements measured. Subgroup analysis showed that women with MND had less barium (p < 0.05) and iron (p < 0.01) in their muscles than controls, a finding probably an artifact of the multiple statistical analyses done on the data. Metallic content of muscle, for unknown reasons, tends to decrease with age in MND, but increases with age in controls.The evidence indicates that the muscle content of heavy metals in patients with MND does not differ from that in matched controls and that in MND, muscle does not store toxic metals for retrograde transport to anterior horn cells, as has been postulated by others.
Viral and bacterial antibody responses in multiple sclerosis
Authors: Vartdal Frode , Vandvik Bodvar , Norrby Erling 
Published: January 2005

AbstractAn imprint electroimmunofixation method (IEIF) was used to characterize antibodies to eight viral antigens (measles, mumps, rubella, herpes simplex type 1, varicella‐zoster, vaccinia, cytomegalovirus, adenovirus) and four bacterial antigens (β‐hemolytic streptococcus, Hemophilus influenzae type B, Escherichia coli, enterococcus) in serum and cerebrospinal fluid (CSF) of 12 patients with multiple sclerosis (MS). Twelve patients matched for age and sex served as controls. Evidence for intrathecal synthesis of oligoclonal antibodies to one or more antigens was found in all 12 MS patients and in 1 of the controls. In the MS group, antibodies to viruses with neurotropic properties were more frequently associated with local synthesis than antibodies to other viruses and bacteria. The types and number of locally synthesized antibodies showed no correlation with disease duration and severity. The antibodies were not associated with oligoclonal CSF IgG and appear to account for only a minor fraction of the locally synthesized CSF IgG in MS.
Section of the pituitary stalk in the rhesus monkey: Morphological and endocrine observations
Authors: Lobo Antunes J. , Louis Kenneth M. , Huang Sidney , Zimmerman Earl , Carmel Peter W. , Ferin Michel 
Published: January 2005

AbstractThe effect of sectioning the pituitary stalk on the hypothalamic magnocellular system of the rhesus monkey was studied by specific immunocytochemical techniques. Regeneration of the proximal stump with reconstitution of a “new” neurohypophysis occurred as early as three weeks after operation, illustrating the remarkable regenerative ability of the system. The functional capacity of the newly formed neurovascular units was also tested, and the results indicate that they not only resume basal secretion of posterior pituitary hormones, but are also able to increase their output in response to a variety of challenges.
An autosomal dominant syndrome of hemiplegic migraine, nystagmus, and tremor
Authors: Zifkin Benjamin , Andermann Eva , Andermann Frederick , Kirkham Trevor 
Published: January 2005

AbstractA mother and son suffer from hemiplegic migraine with onset in childhood. Both have nystagmus which has not changed for many years, but the date of onset is uncertain. They have an asymmetrical tremor, clinically indistinguishable from essential tremor. Neuroophthalmological examination revealed inability to produce, smooth pursuit, gaze‐paretic nystagmus, rebound nystagmus, failure of fixation suppression of the vestibuloocular reflex both horizontally and vertically, and, low gain of the optokinetic system. These abnormalities, confirmed by electrooculography, are commonly seen in disease of the cerebellum and brainstem. Treatment with propranolol and pizotyline lessened the number of episodes of hemiplegia and improved the tremor.Hemiplegic migraine has been reported in association with nystagmus, retinal degeneration, deafness, and ataxia in varying combinations in three other families with autosomal dominant inheritance. These associated neurological manifestations likely represent system degenerations rather than the effect of repeated ischemia imputable to the migraine itself.The syndrome of hemiplegic migraine, tremor, and ocular smooth pursuit system disorder seen in this family appears to be inherited as a single autosomal dominant trait, although more than one autosomal dominant gene may be involved.
Epileptic patterns of local cerebral metabolism and perfusion in humans determined by emission computed tomography of 18FDG and 13NH3
Authors: Kuhl David E. , Engel Jerome , Phelps Michael E. , Selin Carl 
Published: January 2005

AbstractSeventeen patients with partial epilepsy had electroencephalographic (EEG) monitoring concurrent with cerebral positron emission computed tomography (PECT) after 18F‐fluorodeoxyglucose (18FDG) and 13N‐ammonia (13NH3) were given intravenously as indicators of local cerebral glucose utilization (LCMRglc) and relative perfusion, respectively. In 12 of 15 patients who had unilateral or focal electrical abnormalities, interictal 18FDG scan patterns clearly showed localized regions of decreased (14 to 58%) LCMRglc that correlated anatomically with the eventual EEG localization. These hypometabolic zones appeared normal on x‐ray computed tomography in all but 3 patients and were unchanged on 18FDG scans repeated on different days. In 5 of 6 patients who underwent anterior temporal lobectomy the interictal 18FDG scan correctly detected the pathologically confirmed lesion as a hypometabolic zone, and removal of the lesion site resulted in marked clinical improvement. In contrast, the ictal 18FDG scan patterns clearly showed foci of increased (82 to 130%) LCMRglc that correlated temporally and anatomically with ictal EEG spike foci and were within the zones of interictal hypometabolism (three studies in 2 patients). 13NH3 distributions paralleled 18FDG increases and decreases in abnormal zones, but 13NH3 differences were of lesser magnitude. When the relationship of 13NH3 uptake to local blood flow found in dog brain was applied as a correction to the patients' 13NH3 scan data, local alterations in perfusion and glucose utilization were usually matched in both the interictal and the ictal state.We conclude that the interictal 18FDG‐PECT scan is useful in aiding localization of the dysfunctional cerebral zone most likely to be responsible for seizures in patients being considered for anterior temporal lobectomy. With further development, emission computed tomography may help in better categorizing the various forms of the disorder and in elucidating the basic mechanisms of epilepsy in humans.
Epidural spinal cord compression from metastatic tumor: Results with a new treatment protocol
Authors: Greenberg Harry S. , Kim Jae‐Ho , Posner Jerome B. 
Published: January 2005

AbstractEighty‐three patients with epidural spinal cord compression from metastatic cancer were treated with high‐dose adrenocorticosteroids and a new radiation fractionation protocol. Only those patients were included who had complete or almost complete block on myelography and who had not received prior radiation therapy to the area of compression. Patients were given 100 mg of dexamethasone intravenously at the time of diagnosis and 500 rads of radiation on each of the first three days. After a four‐day rest, radiation therapy was continued at 300 rads to a total dose of 3,000 rads.The effects of this new protocol on the patient's motor abilities did not differ from those of previous protocols, namely, 47 of 83 patients (57%) were ambulatory after treatment, with no responses in patients totally paraplegic before treatment. However, early administration of high doses of dexamethasone substantially ameliorated pain in the majority of patients, with relief often coming within hours after the drug was given. On the basis of these data, we recommend high doses of adrenocorticosteroids combined with radiation therapy for acute treatment of spinal cord compression. The optimum fractionation schedule for radiation therapy is not established.
Transient focal cerebral ischemia as a presenting manifestation of unruptured cerebral aneurysms
Authors: Fisher Marc , Davidson Robin I. , Marcus Elliott M. 
Published: January 2005

AbstractFew reports have described an association between cerebral transient ischemic attacks (TIAs) and unruptured cerebral aneurysms. This study presents seven patients with TIA who had aneurysms in a vascular distribution appropriate to their clinical symptoms. In three patients, angiographic evidence of embolization was present distal to the aneurysm without another apparent cardiac or extracranial arterial source for the emboli. The most reasonable pathogenesis for TIA in a patient with an associated saccular aneurysm would be thrombosis of the aneurysmal sac with subsequent embolization. However, subarachnoid blood can cause permanent focal intracranial narrowing, and this appeared to be a factor in at least one patient. The results imply that patients with symptoms of TIA should have their intracranial arterial circulation visualized as part of the diagnostic evaluation.
Demyelinating radiculopathy in the kearns‐sayre syndrome: A clinicopathological study
Authors: Groothuis Dennis R. , Schulman Sidney , Wollman Robert , Frey James , Vick Nicholas A. 
Published: January 2005

AbstractIn the few previously autopsied patients with the Kearns‐Sayre form of progressive ophthalmoplegia, the most prominent abnormalities have been in muscle, with less conspicuous changes in the central nervous system, primarily in the brainstem. Similar findings were present in the case reported here, but in addition there was severe demyelination in the initial few millimeters of the cranial and spinal motor roots distal to the glial‐Schwann cell junction. Milder demyelination was observed in the dorsal spinal and afferent cranial nerve roots, including the eighth nerve, but deafness was due to virtually total destruction of the organ of Corti. Our observations suggest that a radiculopathy may be a feature in some cases of Kearns‐Sayre syndrome.