bluuebunny/crossref_metadata_2025 huggingface dataset:
Hereditary spinal arachnoid cysts, distichiasis, and lymphedema
Authors: Schwartz James F. , O'Brien Mark S. , Hoffman James C.
Published: January 2005

Familial 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

Four 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

We 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

Microvessels 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

Three 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

Measurements 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

The 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

Spontaneous 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

Homovanillic 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

The 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

Twenty‐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

The 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

To 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

An 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

The 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

A 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.
Presymptomatic and early detection in Huntington's disease
Authors: Klawans Harold L. , Goetz Christopher G. , Perlik Stuart
Published: January 2005

This review summarizes research techniques that have been applied to the early diagnosis and presymptomatic detection of Huntington's disease. Presently, no one test definitively discriminates between nonaffected persons and carriers.
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

Seventeen 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

Eighty‐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

Few 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

In 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.
The use and abuse of the cerebrospinal fluid IgG profile in the adult: A practical evaluation
Authors: Hershey Linda A. , Trotter John L.
Published: January 2005

Cerebrospinal fluid (CSF) samples from 150 patients with multiple sclerosis (MS) and 190 patients with other neurological disorders were examined with a battery of tests (the IgG profile) to detect quantitative and qualitative abnormalities in CSF IgG. The CSF IgG profile consisted of an IgG/albumin (IgG/alb) ratio, an IgG index, and examination by agarose gel electrophoresis (AGE) and isoelectric focusing (IF) to detect oligoclonal bands. The tests were compared for diagnostic accuracy, including false‐positive results.

The IgG/alb ratio was less reliable in confirming the diagnosis of MS, but no significant difference in accuracy was found among the other three methods. IF tended to identify more possible and probable MS cases than did AGE but gave a higher rate of false‐positives. The IgG index and IgG synthesis rates showed no significant difference in their ability to identify MS patients. Steroid administration decreased the incidence of abnormal IgG/alb ratios and IgG indices, but not abnormal oligoclonal bands. Central nervous system (CNS) infections or immunological diseases involving the CNS produced a 28 to 40% incidence of abnormalities in the CSF. Neither the patient's age, sex, duration of illness, activity of disease, nor longitudinal course correlated with the CSF findings. A few (1%) control neurological patients had all components in the CSF IgG profile abnormal. For most routine clinical purposes the IgG index and AGE are sufficient for confirmation of diagnosis, and the IgG index was the best single test in our series.
Aluminum chloride induced neurofibrillary changes in the developing rabbit: A chronic animal model
Authors: Wisniewski Henryk M. , Sturman John A. , Shek Judy W.
Published: January 2005

Aluminum chloride injected into the brains of developing rabbits produced profound neurofibrillary changes in neurons of spinal cord and cerebrum similar to those produced in adult rabbits, along with a variety of clinical symptoms with the exception of seizures. Approximately half of the rabbits survived for more than three weeks, and many survived for several months. Many rabbits with large numbers of neurofibrillary changes had no clinical signs and symptoms. The dynamics and topography of the neurofibrillary changes induced by aluminum chloride are described over a period of several months. Many neurofibrillary tangles were seen in neurons of spinal cord and cerebrum up to 60 days after injection of aluminum chloride. There was no obvious correlation between the degree of neuroftbrillary changes and the severity of the clinical signs and symptoms. Animals examined at 85 and 100 days after injection of aluminum chloride had fewer neuronbrillary tangles or none at all, and apparently they had recovered from the neurofibrillary changes. This chronic animal model will allow better investigations of the biochemistry and pathology of neuronbrillary changes, and it will enable behavioral studies to be performed in animals with neuronbrillary changes.
Defective immunoreguktion in multiple sclerosis
Authors: Goust Jean M. , Hoffman Paul M. , Pryjma Juliusz , Hogan Edward L , Hugh Fudenberg H.
Published: January 2005

Imbalances in T cell subpopulations have been reported in multiple sclerosis (MS). In the present study of 31 MS patients, the percentage of T cells with Fc receptors for IgG (TG) was found to be increased in patients with chronic progressive diseare, and another T cell subset binding to the Raji B lymphoid cell line was decreased. An inverse correlation (r = −0.675; >95% confidence limit) was found between these two subsets, suggesting that they vary inversely in MS. The mitogenic responses of MS mononuclear cells, isolated T cells, and recombined T and non‐T cells to the lectins phytohemagglutimn and pokeweed mitogen (PWM) did not differ from those of normal cells. However, more immunoglobulin (Ig)‐producing cells were generated in a PWM‐driven system with cells from MS patients than with cells from age‐matched controls (p < 0.05). Autologous recombination of separated T and non‐T cells did not significantly modify these results. T cells from MS patients added to B cells from normal controls exerted an effect that was related to their percentage of TG cells; that is, values above 15% were associated with a suppression of Ig production, whereas for TG values below 12%, a helper effect or no modification was observed. These results suggest that changes in T cell subsets in MS are related to changes in functional ability to modulate Ig production by normal B cells. However, MS B cells partly escape regulation by their own T cells, suggesting an associated B cell hyperactivity.
Serum gangliosides in cerebral astrocytoma
Authors: Lo Hsiao S. , Hogan Edward L. , Koontz Daniel A. , Dennis Traylor T.
Published: January 2005

The serum concentration and composition of gangliosides were examined in 80 humans including 10 normal subjects. A significant increase was found in the total gangliosides of serum in 7 patients with cerebral astrocytomas. There was also an increased percentage of serum gangliosides with simpler structure, particularly GM3. The serum of patients with other intracranial tumors, including pituitary adenomas, ependymoma, teratoma, and metastases, did not show an increase in total ganglioside; however the pattern of simplification was found in these and in a few patients with extracranial tumors as well. The findings suggest that astrocytoma tumors shed sialoglycolipids into the circulation, and their assay may be useful in monitoring oncological therapy.
Neuroendocrinology and brain peptides
Authors: Cooper Paul E. , Martin Joseph B.
Published: January 2005

To date about thirty peptides—low‐molecular‐weight, single‐chain amino acid compounds‐‐are known to be distributed widely in the central nervous system within selective neuronal pathways. These findings, combined with a large body of neuropharmacological, behavioral, and electrophysiological data, open new horizons in neurobiology, force a reexamination of old and accepted hypotheses, and hold important implications for the clinician.

There is evidence that substance P. and the opioid peptides play a major role in the pain pathway, particularly at the level of the spinal cord. Available evidence also implicates vasoactive intestinal polypeptide in the control of cerebral circulation, cholecystokinin in the regulation of appetite, and vasopressin and adrenocorticotropic hormone in memory. Many questions, however, remain. For most peptides there is little information on mechanisms of biosynthesis, release, interaction with receptors, and termination of biological effect. Another important question is the interaction of peptides with other neurotransmitters. The evidence that both “classic” neurotransmitters and peptides can be found in the same neuron necessitates reformulation of Dale's “one neuron, one neurotransmitter” hypothesis. It may be that a single cell, while containing different classes of neurotransmitter, will contain only one member of any particular class.

It is not too early to speculate on the role of the numerous and diverse peptides in neuronal tissue and on the implications of peptide abnormalities in a variety of neurological diseases. The answers to these and other questions pose a fascinating challenge to neurobiologist and clinician alike.
Cerebellar dyssynergia in humans—A quantitative analysis
Authors: Miller Robert G. , Freund Hans‐J.
Published: January 2005

Patients with cerebellar lesions and limb ataxia performed two types of continuous tracking tasks involving flexion and extension of the index finger. In both tasks, patients were provided cutaneous and proprioceptive cues, but visual feedback was given in the first task (visual tracking) and not in the second (arbitrarily termed proprioceptive tracking). Raw records and Fourier‐analyzed power spectra were compared with results in normal controls. Harmonic distortion was determined for each task.

In all patients, as well as normal subjects, tracking performance was markedly improved and harmonic distortion substantially reduced during proprioceptive tracking. This surprising finding may result from a much shorter feedback loop for proprioceptive stimuli compared to visual stimuli. The tracking records, power spectra analysis, and determination of harmonic distortion provide both qualitative and quantitative data in patients with dyssynergia.
Remyelination following viral‐induced demyelination: Ferric ion—ferrocyanide staining of nodes of ranvier within the CNS
Authors: Weiner L. P. , Waxman S. G. , Stohlman S. A. , Kwan A.
Published: January 2005

Ferric ion‐ferrocyanide (Fe‐FeCN) staining was used to stain nodes of Ranvier in remyelinating central nervous system (CNS) axons following viral‐induced demyelination. As at normal nodes, Fe‐FeCN staining was observed on the cytoplasmic surface of the nodal axolemma of remyelinated fibers. These fibers were identified on the basis of inappropriately short internode lengths and thin myelin sheaths. Thus, newly formed nodes along remyelinated CNS axons recapitulate at least one normal nodal membrane property.
Plasma exchange in myasthenia gravis: Electrophysiological studies
Authors: Campbell William W. , Leshner Robert T. , Swift Thomas R.
Published: January 2005

Electrophysiological recordings of evoked muscle action potentials during repetitive stimulation were performed in four female patients undergoing plasmapheresis for myasthenia gravis. Studies were done immediately before and after each exchange and were repeated daily during a series of exchanges and at intervals thereafter. Electrophysiological improvement was seen but was delayed for at least seven days from the start of plasmapheresis therapy. No sudden and substantial improvements were encountered, though anti‐receptor antibody titers dropped precipitously. A reversible neuromuscular blocking effect of anti‐receptor antibody could not be demonstrated in these patients.
Isolated fear in complex partial status epilepticus
Authors: McLachlan R. S. , Blume W. T.
Published: January 2005

As part of her seizure disorder, a young woman with temporal lobe epilepsy had prolonged attacks of isolated fear associated electrographically with right temporal lobe status epilepticus. The attacks stopped after right temporal lobectomy. Fear by itself, a rare manifestation of complex partial seizures, probably occurs when the abnormal discharges are confined to the anterior temporal region.
The influence of immaturity on hypoxic‐ischemic brain damage in the rat
Authors: Rice John E. , Vannucci Robert C. , Brierley James B.
Published: January 2005

Brain damage in the Levine preparation (unilateral common carotid artery ligation with hypoxia) consists of ischemic neuronal alterations in the ipsilateral forebrain. As the model has been restricted to adult animals, unilateral common carotid artery ligation was carried out in 7‐day‐postnatal rats. Four to 8 hours later the 25 pups were exposed to 8% oxygen at 37°C for 3.5 hours. Controls consisted of littermates subjected to carotid ligation without subsequent hypoxia, hypoxia without prior ligation, and neither ligation nor hypoxia. After hypoxia the animals were returned to their dams and appeared normal for up to 50 hours. All pups were then killed by perfusion‐fixation.

Moderate to severe ischemic neuronal changes were seen in the ipsilateral cerebral cortex, striatum, and hippocampus in at least 90% of the animals and included infarction in 56% of the brains. Cortical damage was occasionally laminar but more often occurred in columns at right angles to the pial surface. Unlike adult animals, there was necrosis of white matter, greater ipsilaterally, originating in and spreading from myelinogenic foci. The evolution of ischemic cell change and the associated gliomesodermal reaction was more rapid than in the adult. In 22 additional pups subjected to carotid artery ligation and hypoxia, brains were analyzed for water content. Significant increases (0.6 to 3.3%) in water content of the ipsilateral hemispheres occurred in 11 of 22 brains (50%).

Unilateral ischemia combined with hypoxia in developing rats therefore results in neuronal destruction in the same brain regions as in adult animals, but also causes necrosis of white matter. The incidence of increased water content was similar to that of overt infarction. Thus, as previously shown in the adult, brain edema is a consequence rather than a cause of major ischemic damage in the immature animal.
Periodic synchronous discharge and myoclonus in Creutzfeldt‐Jakob disease: Diagnostic application of jerk‐locked averaging method
Authors: Shibasaki Hiroshi , Motomura Satoru , Yamashita Yoriaki , Shii Hiroaki , Kuroiwa Yoshigoro
Published: January 2005

The technique of jerk‐locked averaging was applied to two patients with pathologically proved Creutzfeldt‐Jakob disease. Patient 1, whose brain contained numerous kuru plaques and severe cerebral white matter degeneration, did not show any periodic synchronous discharges in spite of frequently repeated EEG recordings. The technique of jerk‐locked averaging, however, disclosed a negative sharp wave over the contralateral central area in close temporal association with myoclonus of an upper extremity. Patient 2 had classic periodic synchronous discharges on the EEG, and jerk‐locked averaging delineated a fixed relationship in time and space between the periodic discharge and the myoclonus.
Antidiuretic hormone levels in stroke patients
Authors: Joynt Robert J. , Feibel John H. , Sladek Celia M.
Published: January 2005

Serum osmolality and antidiuretic hormone (ADH) levels were determined for 17 patients with cerebral infarction, 4 with subarachnoid hemorrhage, and 12 controls. The ADH levels were elevated significantly in the stroke patients. Hyponatremia was not observed. Stroke patients are at risk for developing electrolyte disturbances; thus, fluid intake and electrolyte levels should be closely observed.
Depth electroencephalography in selection of refractory epilepsy for surgery
Authors: Spencer Susan Soloway
Published: January 2005

Depth electroencephalography (EEG) is sometimes used to evaluate medically refractory epileptic patients for surgical treatment. Surgical excision of well‐defined epileptogenic foci has been shown repeatedly to cause a substantial reduction of seizure frequency in 60 to 80% of these patients; however, because surgical success is no better at centers that employ depth EEG in patient evaluation, the procedure remains controversial. Review of the available literature shows that depth EEG results reported to date, when compared to scalp EEG results in 178 patients, could have enabled selection of 36% more patients for surgery by defining otherwise unidentifiable single epileptogenic foci. Furthermore, depth EEG could have prevented surgery in another 18% by demonstrating different or additional epileptogenic foci in patients otherwise thought to have a single discharging focus amenable to resection. Thus depth EEG had the potential to alter the surgical decision in more than 50% of patients reported. Centers that employ depth EEG may evaluate a different population of patients, which could account for their lack of increased surgical success.
Correlation of criteria used for localizing epileptic foci in patients considered for surgical therapy of epilepsy
Authors: Engel Jerome , Rausch Rebecca , Lieb Jeffrey P. , Kuhl David E. , Crandall Paul H.
Published: January 2005

Criteria for anterior temporal lobectomy, performed on seven patients with partial complex seizures, were derived from a battery of fourteen presurgical tests. Seven tests were routine studies aimed at identifying a focus of epileptic excitability, while seven were designed to reveal areas of focal functional deficit. Conflicting information was frequently obtained from the tests of epileptic excitability, suggesting that it is probably inaccurate to view patients with partial complex seizures as having a single epileptogenic focus. Presurgical evaluation must therefore be aimed at identifying the focus most responsible for the patient's habitual seizures. Tests of focal functional deficit provided useful nonconflicting confirmatory information in each of the seven patients studied. The most reliable information was obtained from depth electrode implantation, and this procedure should be considered essential except when all evidence of surface‐recorded epileptic excitability, including ictal onset, and evidence of focal functional deficit agree.
Abnormal regulation of in vitro IgG production in multiple sclerosis
Authors: Kelley Robert E. , Ellison George W. , Myers Lawrence W. , Goymerac Vincent , Larrick Susan B. , Kelley Carol C.
Published: January 2005

A pokeweed mitogen‐stimulated IgG synthesis assay was used to evaluate T suppressor cell function in peripheral blood lymphocytes (PBL) obtained form multiple sclerosis (MS) patients. Autochthonous cultures of fractionated and recombined MS B and T cells exhibited less suppression of IgG synthesis than similar cultures of PBL from control subjects. No difference occurred in the levels of suppression among MS patients grouped according to disease states. When allogenic cultures of MS B and normal T cells or normal B and MS T cells were compared to autochthonous cultures of control cells, no differences were observed. The results suggest that the lower levels of suppression observed in autologous MS cell cultures are not a result of T suppressor cell dysfunction alone.
GABA receptors are increased in brains of alcoholics
Authors: Tran Vinh T. , Snyder Solomon H. , Major Leslie F. , Hawley Rollin J.
Published: January 2005

γ‐Aminobutyric acid (GABA) receptor binding was increased in postmortem brain samples of chronic alcoholic patients compared to control patients. Numbers of binding sites were augmented in alcoholic brain, with no change in affinity. Muscarinic cholinergic and benzodiazepine receptors did not differ between controls and alcoholic brains, while a modest reduction in β‐adrenergic receptors may have been related to postmortem receptor changes. The results suggest that GABA ergic mechanisms might play a role in chronic alcoholism.
Alterations in creatine kinase in fresh muscle and cell cultures in duchenne dystrophy
Authors: Ionasescu R. , Kaeding L. , Feld R. , Witte D. , Cancilla P. , Stern L. Z.
Published: January 2005

Creatine kinase (CK) (total and isoenzymes) was measured in cultures obtained by dissociation and subsequent plating of cells from biopsied quadriceps muscle of 10 patients with Duchenne muscular dystrophy (DMD) and 20 controls. The total cellular CK and CK‐MM reached highest values around 21 days in both DMD and control cultures, suggesting that DMD cultures do not show delayed myoblast fusion. There was a significant decrease of total cellular CK in DMD cultures at all stages, but the maximum differences were noted for the peak values. The CK isoenzyme pattern in DMD cultures demonstrated a higher percentage of CK‐BB and CK‐MB and a lower percentage of CK‐MM than was observed in cultures from the controls. Addition of cytosine arabinoside after myoblast fusion to muscle cell cultures did not induce significant changes of CK isoenzyme pattern. There was no difference in the CK levels in culture medium from controls and DMD patients. The alterations of cellular CK were similar in fresh muscle and cell cultures from DMD patients.
Nerve cell fusion in a case of subacute sclerosing panencephalitis
Authors: Paula‐Barbosa M. M. , Cruz Celso
Published: January 2005

Evidence indicating cell membrane fusion among cells and their processes where subacute sclerosing panencephalitis (SSPE) virus is present is shown in an ultrastructural study of a brain biopsy. Virus budding from plasma membrane was not seen. These rare findings reinforce the hypothesis of cell‐to‐cell transmission as the most likely mechanism of SSPE virus infection. The presence of fused membranes between cell processes and cell soma probably explains the rapid spread of this virus throughout the nervous system.
Cerebral blood flow following normavolemic hemodilution in patients with high hematocrit
Authors: Henriksen Leif , Paulson Olaf B. , Smith Ronald J.
Published: January 2005

The effects on cerebral hemodynamics of venisection and a 4% albumin‐saline infusion were studied in six patients withhigh hematocrit (mean, 51.5%). Cerebral blood flow (CBF) was measured using the xenon 133 intracarotid injection method. Blood gases were measured in arterial and jugular venous blood. Rapid two‐stage hemodilution, which lowered mean hematocrit by 9 and 13%, resulted in CBF increases of 19 and 23%, respectively. Jugular venous partial pressure of oxygen and oxygen delivery capacity (CBF × arterial oxygen content) did not change significantly from baseline. The cerebral metabolic rate for oxygen increased slightly following stage 1 hemodilution but returned to baseline value following stage 2. The study lends no support to the concept that patients whose hematocrit is at the high end of the normal range have genealized cerebral hypoxia.
Cerebral hemodynamics and metabolism in postoperative cerebral vasospasm and treatment with hypertensive therapy
Authors: Montgomery Erwin B. , Grubb Robert L. , Raichle Marcus E.
Published: January 2005

A 24‐year‐old woman developed subarachnoid hemorrhage from an aneurysm at the bifurcation of the right internal carotid artery. Following successful clipping of the aneurysm she developed a left hemiplegia associated with focal cerebral vasospasm, which markedly improved when systemic blood flow and oxygen utilization were significantly depressed in both cerebral hemispheres, while blood volume was significantly elevated only on the side with vasospasm. Oxygen extraction was significantly elevated in both hemispheres, indicating a generalized impairment in oxygen delivery to the brain.
Permanent axotomy, a model of axonal atrophy and secondary segmental demyelination and remyelination
Authors: Dyck Peter James , Lais Alfred C. , Karnes Jeannine L. , Sparks Margaret , Hunder Heidi , Low Phillip A. , Windebank Anthony J.
Published: January 2005

Permanent axotomy in cats produced by hind limb ablation results in sequential pathological alterations of myelinated fibers of the proximal nerve stump. The changes are like those previously described for human uremic neuropathy and such system atrophies as Friedreich's ataxia; axonal atrophy → myelin wrinkling → nodal lengthening and internodal demyelination → remyelination.
The origins of short‐latency somatosensory evoked potentials in humans
Authors: Mauguière F. , Courjon J.
Published: January 2005

Short‐latency somatosensory evoked potentials (SEPs) to median nerve stimulation were studied in three patients with well‐localized lesions of the lemniscal pathways. In two patients who had focal thalamic vascular lesions, the early scalp‐recorded P15 far‐field potential was unaltered while later SEP components were absent. In the third patient, operated on for astrocytoma of the dorsal medulla and right dorsal funiculus, the P15 potential was absent but the earlier negative potentials recorded from the neck persisted whichever side was stimulated. The data suggest a subthalamic origin for the scalp‐recorded P15 far‐field potential.
Penicillin‐ and barium‐induced epileptiform bursting in hippocampal neurons: Actions on Ca++ and K+ potentials
Authors: Hotson John R. , Prince David A.
Published: January 2005

Both barium (Ba++) and penicillin produce spontaneous epileptiform burst generation in hippocampal neurons in vitro. Recent investigations suggest that Ba++ acts by both adding to a calcium (Ca++)‐mediated depolarization and reducing potassium (K+) conductance. In contrast, it has been proposed that pencillin produces burst generation by attenuating inhibitory postsynaptic potentials. However, some evidence suggests that penicillin may also directly alter intrinsic membrane properties. We therefore compared the actions of penicillin and Ba++ on three intrinsic Ca++‐ or K+‐mediated membrane events, namely, Ca++ spikes, Ca++‐dependent anomalous rectification, and K+‐dependent afterhyperpolarization. Ba++ augmented the Ca++ potentials and attenuated the K+‐dependent afterhyperpolarization: penicillin had no demonstrable effect on these events. Ba++ produced rhythmical burst firing and oscillations of the membrane potentials, while penicillin caused sporadic burst generation followed by a longlasting afterhyperpolarization. Synchronized, orthodromically evoked burst firing occurred after exposure to penicillin but not to Ba++.

Ba++ and penicillin are prototypes of agents which induce epileptogenesis in mammalian cortical neurons by two different but probably interrelated mechanisms. Ba++ causes burst generation by disrupting a delicate balance between depolarizing Ca++ potentials and repolarizing, hyperpolarizing K+ potentials. Pencillin does not affect Ca++‐ or K+‐mediated membrane events; other data suggest that it produces burst generation in hippocampal pyramidal neurons by attenuating γ‐aminobutyric acid‐mediated synaptic inhibition, which in turn ordinarily limits intrinsic bursting.