bluuebunny/crossref_metadata_2025 huggingface dataset:
Brain electrical activity in patients with presenile and senile dementia of the Alzheimer type
Authors: Duffy Frank H. , Albert Marilyn S. , McAnulty Gloria
Published: January 2005

Neurophysiological and behavioral data obtained from 9 patients with presenile dementia and 10 with senile dementia of the Alzheimer type were compared with similar data from 25 age‐ and sex‐equivalent controls. Compared with the healthy controls, both patient groups demonstrated increased background electroencephalographic slowing with a reduction in fast activity (synchronization). Topographic analyses of data from electroencephalographic and evoked potential studie indicate that areas of maximal group differences between the presenile patients and their controls include the right posterior temporal and, to a lesser extent, left midtemporal to anterior temporal areas, whereas the maximal differences between the senile patient group and their controls involve the midfrontal and anterior frontal lobes, bilaterally. Moreover, right‐sided numerical features derived from topographic maps proved most useful in differentiating the presenile patients and their age‐matched controls, whereas bilateral features were more useful in separating senile patients from their controls. These topographic dissimilarities between patient groups suggest that an age‐disease interaction exists between patients with presenile and senile dementia of the Alzheimer type. Correlational analyses between neuropsychological test scores and neurophysiological features indicate that increased slowing and decreased fast activity were associated with poorer test performance.
Hereditary dysphasic dementia and the Pick‐Alzheimer spectrum
Authors: Morris John C. , Cole Monroe , Banker Betty Q. , Wright David
Published: January 2005

Hereditary dysphasic dementia is described in terms of its clinicopathological, ultrastructural, and transmissibility characteristics. Its mode of inheritance is autosomal dominant, and its clinical manifestations of progressive dementia and severe dysphasic disturbances are expressed in late adulthood. Complete neuropathological examination of four patients reveals findings typical for Pick's disease (asymmetrical focal cerebral atrophy), Alzheimer's disease (profuse neuritic plaques), and paralysis agitans (neuronal depigmentation, depletion, and Lewy body formation in substantia nigra) in addition to a striking but nonspecific spongiform degeneration of superficial cortical layers. This unique combination of gross morphological and histopathological features qualifies hereditary dysphasic dementia as a distinct entity, but its precise relationship to the well‐recognized adult cortical dementias has been difficult to establish by conventional classification methods. This disorder and other unusual dementing illnesses may be best considered as part of a Pick‐Alzheimer spectrum of cortical neuronal degenerations.
Perinatal cerebral infarction
Authors: Ment Laura R. , Duncan Charles C. , Ehrenkranz Richard A.
Published: January 2005

The diagnosis of perinatal cerebral infarction, although frequently suggested clinically, has been made most commonly at postmortem examination; few infants surviving stroke are reported in the literature. We evaluated 18 infants with perinatal cerebral infarction in a recent twelve‐month interval. Seven were preterm neonates, 6 of whom had experienced neonatal intraventricular hemorrhage. Three full‐term infants were thought to have experienced cerebral infarction in utero and had evidence of well‐defined strokes on computed tomographic scans performed shortly after birth. Eight infants developed stroke at term. The most common cause of cerebral infarction in our series was perinatal asphyxia. Fourteen of the infants were seen with neonatal seizures. Fourteen of the 18 have died or are faring poorly at 4 to 12 months of age (corrected).
Infection of neuronal cell cultures with reovirus mimics in vitro patterns of neurotropism
Authors: Dichter Marc A. , Weiner Howard L.
Published: January 2005

Primary neuronal cell cultures of rat fetal cerebral cortex serve as in vitro models for the study of a variety of neuronal membrane receptors. Such studies have focused primarily on receptors for neurotransmitters and drugs. In the present series of experiments, we have employed this model to study the in vitro pattern of infection with reovirus types 1 and 3, two well‐characterized neurotropic viruses that show specificity for neurons (type 3) or ependymal cells (type 1) in vivo and whose specificity has been linked with surface receptors on somatic cells. We have found that in primary neural cell culture, reovirus type 3 maintained its specificity by infecting neurons whereas reovirus type 1 did not infect neurons. Both serotypes infected astrocytes in the cultures, type 1 to a greater extent than type 3. In addition, reovirus type 3 bound to the surface of neurons whereas type 1 did not. Using recombinant viral clones, the in vitro tropism and the neuronal binding were shown to be properties of the viral hemagglutinin, a small outer capsid viral protein, as is the case with the neurotropism in vivo. It is postulated that the neurotropism of reovirus type 3 is related to the interaction of the viral hemagglutinin with an as yet undefined structure on the neuronal surface.
The basis of intra‐blood‐brain‐barrier IgG synthesis
Authors: Tourtellotte Wallace W. , Staugaitis Susan M. , Walsh Michael J. , Shapshak Paul , Baumhefner Robert W. , Potvin Alfred R. , Syndulko Karl
Published: January 2005

We hold that the intra‐blood‐brain‐barrier (BBB) IgG synthesis (SYN) rate can be quantitated reliable and validly. Although several formulas distinguish patients with sclerosis from normal controls equally well, only the SYN rate formula has been validated in humans using isotopic tradcer techniques. Our formula for the IgG SYN rate is reducible to Reiber's formula; therefore, both can be used to quantify the IgG SYN rate in a manner not possible using the IgG index. Although our SYN rate formula has been validated for a modest range of BBB abnormalities (cerebrospinal fluid/serum albumin ratios), there is evidence to suggest that it may be used even in patients having severe BBB damage. We question the acceptance of unique cerebrospinal fluid IgG bands as indisputable evidence of intra‐BBB IgG SYN in the presence of a modest to severely damaged BBB. Finally, the utility of quantitation and detection of intra‐BBB IgG SYN by standardized methods in a group of asymptomatic, normal individuals compared with a group of patients with clinical definite multiple sclerosis is presented.
Clinical and experimental neurotoxicity of 2‐t‐butylazo‐2‐hydroxy‐5‐methylhexane
Authors: Spencer Peter S. , Beaubernard Christan M. , Bischoff‐Fenton Monica C. , Kurt Thomas L.
Published: January 2005

Seven workers developed weight loss, sensorimotor neuropathy, visual dysfunction, impaired mentation, and emotional lability shortly after the introduction of a new synthetic catalyst in a plant manufacturing reinfoced bathtubs. The newly introduced catalyst, 2‐t‐butylazo‐2‐hydroxy‐5‐methylhexane, is shown to produce a similar syndrome in rats. Axonal degenration of optic nerves, ascending and descending spinal tracts, and peripheral nerves appears to underlie this syndrome.
High‐resolution computed tomography in multiple sclerosis
Authors: Barrett Lynn , Drayer Burton , Shin Chelsu
Published: January 2005

Eighty‐five patients were classified as having definite (n = 34), probable (n = 18), or possible (n = 33) multiple sclerosis using the criterial of Poser. Each Patient had an enhanced computed tomographic examination, and most had cerebrospinal fluid and evoked response studies at the same time. Abnormalities including focal decreased brain density, abnormal enhancement, and cerebrospinal fluid space enlargement were found in 62% of patients (85% of those with definite, 39% of those with probable, and 52% of those with possible multiple sclerosis), and abnormal enhancing areas were demonstrated in 29% (44% of those with definite, 17% of those with probable, and 21% of those with possible disease). In the subgroup of patients with definite multiple sclerosis and recent clinical exacerbation (within the prior 8 weeks), abnormal enhancement was present in 89%. There was a strong correlation between clinical exacerbation and abnormal contrast enchancement. Cerebrospinal fluid studies (IgG, white blood cell count, total protein) had no correlation with exacerbation or abnormal enhancement. Maps of low‐density and enhancing areas were similar to those previously described in postmortem studies. Computed tomography thus provides an in vivo, objective, and anatomically specific map of the brain parenchyma and the integrity of the blood‐brain barrier that is useful in research studies evaluating the treatment of multiple sclerosis. It is also useful in patients in whom the diagnosis of multiple sclerosis is suspected but not certain on the basis of clinical and laboratory evaluation.
The nucleus basalis of Meynert in neurological disease: A quantitative morphological study
Authors: Rogers John D. , Brogan Donna , Mirra Suzanne S.
Published: January 2005

The recent finding of neuronal degeneration in the nucleus basalis of Meynert (nbM) in Alzheimer's disease suggests a possible role of this nucleus and the cholinergic system in dementing illness. We assessed neuronal population and density in two anatomical levels of the nbM in 38 coded brains from patients with a broad variety of neurological disorders. We found the most striking nbM degeneration in Alzheimer's and Parkinson's diseases. An intermediate loss of neurons was noted in the transmissible dementia Creutzfeldt‐Jakob disease and in progressive supranuclear palsy. The usually nondementing diseases amyotrophic lateral sclerosis and multiple sclerosis showed cell counts comparable to those in a control group of nondemented patients with cerebrovascular disease. Characteristic neuropathological changes of Alzheimer's, Parkinson's, and Creutzfeldt‐Jakob diseases, progressive supranuclear palsy, and subacute sclerosing panencephalitis were also found in the nbM. Most previous studies of the nbM have been based on small numbers of cases compared with controls. In our large series we used consistent methodology and a multiple‐comparison statstical procedure to avoid overreporting of statistical significance. Our comparison of the nbM not only confirms the marked degeneration of the nbM in Alzheimer's disease, but also places such degeneration into perspective in a spectrum of dementing and nondementing neurological diseases. Larger case studies, using appropriate statistical techniques for multiple group comparisons, are needed to establish the significance of nbM degeneration in neurological disease.
Positron emmission tomography in the asphyxiated term newborn: Parasagittal impairment of cerebral blood flow
Authors: Volpe Joseph J. , Herscovitch Peter , Perlman Jeffrey M. , Kreusser Katherine L. , Raichle Marcus E.
Published: January 2005

Hypoxic‐ischemic encephalopathy secondary to perinatal asphyxia in the term newborn is the most common recognized cause of the subsequent motor deficits often grouped under the rubric “cerebral palsy.” In order to provide insight into the basic nature and pathogenesis of the brain injury in such infants, we studied regional cerebral blood flow (CBF) by positron emission tomography (PET) in 17 asphyxiated term infants during the acute period of illness. A consistent and apparently unifying abnormality was observed, namely, a relative decrease in CBF to parasagittal regions, generally symmetrical and more marked posteriorly than anteriorly. Thus, parasagittal values for CBF were generally 25 to 50% lower than those for the sylvian cortex; in the normal or near normal infant, parasagittal values are only approxmately 10% lower than those for the sylvian cortex. (Additional normal findings for regional CBF were 50% higher flows to the cerebral cortex than to the cerebral white matter and flows to the basal ganglia and thalamus at least as high as those to the cerebral cortex.) That the relative deficit in CBF to parasagittal regions reflects tissue injury was indicated by the close topographic correlation on technetium brain scans in 3 patients of increased tissue uptake of radionuclide and the CBF abnormality. Moreover, the single patient studied at postmortem examination exhibited parasagittal ischemic cerebral injury that correlated well with the PET abnormaility of regional CBF.

The topography of the PET abnormality, i.e., the cerebrovascular watershed regions, suggests that the brain injury is basically ischemic and that the pathogenesis relates to impaired cerebral perfusion, perhaps secondary to systemic hypotension occurring in association with the perinatal asphyxia. Experimental data support this formulation.
Atypical Alzheimer's disease with spastic paresis and ataxia
Authors: Aikawa Hisashi , Suzuki Kinuko , Iwasaki Yuzo , Iizuka Reiji
Published: January 2005

An unsusal case of Alzheimer's disease (AD) is reproted. The patient was a 33‐year old Japanese housewife who had progressive dementia, severe spasticity, and mild ataxial for six years. Postmortem emamination revealed severe changes of AD and degeneration of the corticspinal tracts, as well as neuritic plaques and plaquelike degeneration in the cerebellum. This appears to be the twelfth reported case of AD with spasticity and ataxia.
Electroencephalographic recording from the temporal lobes: A comparison of ear, anterior temporal, and nasopharyngeal electrodes
Authors: Sperling Michael R. , Engel Jerome
Published: January 2005

A prospective study was performed to evaluate the usefulness of ear, anterior temporal, and nasopharyngeal electrodes for recognizing temporal lobe epileptic electroencephalographic (EFG) foci. One handred and three EFGs were performed on patients suspected of having epilepsy who had a previous normal record. Nasopharyngeal electrodes were simultaneously sompared with ear and anterior temporal electrodes to determine their relative abilities to detect pathological epileptiform transients from the temporal region. Epeliptic discharges were detected in 22% of the recordings. Pathological discharges seen in nasopharyngeal electrode derivations invariably were seen in ear and anterior temporal electrode derivations using carefully designed montages, and EEG diagnosis was not altered by use of nasopharyngeal electrode. We conclude that EEG recording with ear and anterior temporal electrodes is sufficient to detect pathological temporal epileptiform transients, and that the use of nasopharyngeal electrodes as a routine procedure offers no added benefit.
Ganglioside patterns in amyotrophic lateral sclerosis brain regions
Authors: Rapport Maurice M. , Donnenfeld Hyman , Brunner William , Hungund Basalingappa , Bartfeld Harry
Published: January 2005

In a search for evidence of biochemical disorders in regions of postmortem brain other than the motor cortex in amyotrophic lateral sclerosis (ALS), ganglioside patterns were also examined in the frontal, temporal, and parahippocampal gyrus cortex. In 21 ALS brains studied (20 sporadic, 1 familial), abnormal patterns were found in the frontal cortex (81%), temporal cortex (75%), motor cortex (70%), and parahippocampal gyrus cortex (71%). Patterns were established by measuring the percentage distribution of 12 ganglioside species. Two abnormal patterns were detected. One was based on low proportions of GD1b, GT1b, and GQ1b associated with high proportions of GM2 and GD3 (GM1, GD1a, GD2, and GT1a values were normal). The second abnormality was the appearance of Gx. Neither abnormality was seen in the 13 non‐ALS control brains. The first, and predominant, abnormality was found in the frontal cortex in 14 brains, and the second was observed in 13 brains; 10 brains showed both abnormalities. These findings thus constitute evidence that the disease process in ALS extends beyond the motor cortex and involves neurons in several brain areas.
Localization of lead in rat peripheral nerve by electron microscopy
Authors: Windebank Anthony J. , Dyck Peter James
Published: January 2005

Lead intoxication in rats reliably produces segmental demyelination. Following a single intravenous injection of radioactive lead, localization of tracer was observed sequentially by quantitative electron microscopical autoradiog‐raphy. The animals injected had been on a lead‐containing diet for 70 days; as a result, the blood‐nerve barrier was broken down and demyelination was proceeding. Six hours after a single dose, the lead was localized to the endoneurial space of the peroneal nerve, and 72 hours later, to the myelin membrane. Lead may exert a direct effect on the membrane and alter its stability both by altering the lipid content of the membrane and by directly interfering with the lamellar structure.
Heterogeneity of human skeletal muscle tropomyosin
Authors: Giometti Carol S. , Danon Moris J.
Published: January 2005

Six polypeptides resolved by two‐dimensional electrophoresis of homogenates from human skeletal muscle have been identified as tropomyosin by electrophoretic and immunochemical methods. The 6 proteins are consistently present in approximately the same abundance in normal biceps, deltoid, gastrocnemius, and quadriceps muscle. Analysis of samples from individuals with Becker's dystrophy, Duchenne dystrophy, limb girdle dystrophy, polymyositis, myopathy related to vitamin E deficiency, type II fiber deficiency, and from an infant with indistinct fiber type differentiation, however, showed quantitative variations in the tropomyosin pattern. Muscle with histochemically demonstrated type II fiber deficiency lacked two of the normal tropomyosin proteins and the type II myosin light chains. Muscles lacking type I myosin light chains were deficient in a different pair of tropomyosin proteins. The results suggest that normal human skeletal muscle contains one major type of tropomyosin protein (β‐tropomyosin) common to both fast and slow fibers, together with two other major proteins (α‐tropomyosin and α′‐tropomyosin), one of which is specific to fast fibers and the other to slow fibers. Preliminary data from the reaction of muscle homogenates with alkaline phosphatase indicate that 3 of the 6 tropomyosin polypeptides resolved by two‐dimensional electrophoresis are phosphorylated forms of the α‐, α′‐, and β‐tropomyosins.
Cardiac dysfunction during status epilepticus in the neonatal pig
Authors: Young Richard S. K. , Fripp Raymond R. , Yagel Susan K. , Werner John C. , McGrath Glenn , Schuler H. Gregg
Published: January 2005

Cardiovascular complications frequently occur during status epilepticus. To determine the changes in systemic and pulmonary arterial pressure, cardiops output, and left ventricular contractility during seizures, 1‐week‐old pigs were intubated, paralyzed, mechanicall
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entilated, and catheterized with a Swan‐Ganz catheter. Seizures were induced with intravenous bicuculline. Eariy changes consisted of significant systemic and pulmonary arterial hypertension. After 2 hours of seizures, the animals developed progressive systemic hypotension and decreased cardiac output. M‐mode echocardiography disclosed a decrease in left ventricular contractility. Cardiac tissue frozen in situ showed a significant increase in lactate and reductions in glucose, triglyceride, and adenosine triphosphate levels. Prolonged seizures in the neonatal pig result in cardiac dysfunction, which may play a role in the development of epileptic brain damage.
Azathioprine and steroids are not more effective in decreasing multiple sclerosis intra–blood‐brain‐barrier IgG synthesis than steroids alone
Authors: Staugaitis Susan M. , Shapshak Paul , Myers Lawrence W. , Ellison George W. , Tourtellotte Wallace W. , Lee Mike
Published: January 2005

Intra–blood‐brain‐barrier IgG synthesis rates and oligoclonal IgG banding patterns were examined in 9 patients with multiple sclerosis who were treated with azathioprine and steroids for 2 to 4.5 years. The IgG synthesis rates of 5 patients were significantly decreased from the pretreatment mean values 1 month after treatment, and their synthesis rates remained at the decreased levels throughout treatment. However, among the remaining 4 patients, the rates exceeded the pretreatment means. This continuous suppressive effect of the combined azathioprine and steroids upon the IgG synthesis rate was similar to that of steroids, suggesting that azathioprine and steroids in combination were not more effective in reducing intra–blood‐brain‐barrier IgG synthesis than steroids alone. Oligoclonal IgG patterns in all cerebrospinal fluid samples were not significantly altered during the study.
Serial immunological studies in multiple sclerosis patients treated systemically with human alpha interferon
Authors: Panitch Hillel S. , Francis Gordon S. , Hooper Carol J. , Merigan Thomas C. , Johnson Kenneth P.
Published: January 2005

A battery of immunological functions was studied over a 2‐year period in conjunction with a placebo‐controlled trial of natural human alpha interferon in patients with multiple sclerosis. IgG synthesis was increased both systemically and intrathecally by administration of interferon; however, there were only minor changes in cerebrospinal fluid oligoclonal bands. Levels of helper and suppressor T lymphocytes fluctuated independently of clinical exacerbations, although mean helper/suppressor ratios were higher in multiple sclerosis patients than in controls and increased further during interferon treatment. Cerebrospinal fluid myelin basic protein and antibodies to basic protein were not affected by exacerbations or by interferon administration. Circulating IgG antibodies induced by interferon treatment appeared to be directed at a non‐interferon contaminant of the preparation. None of the assays was a consistent indicator of disease activity or of clinical response to interferon.
Immunological complications in multiple sclerosis patients receiving interferon
Authors: Rice G. P. A. , Woelfel E. L. , Talbot P. J. , Braheny S. L. , Sipe J. C. , Knobler R. L. , Merigan T. C. , Oldstone M. B. A.
Published: January 2005

In a prospective, placebo‐controlled study designed to test the efficacy of human alpha interferon (1FN) in the treatment of multiple sclerosis, we monitored several immunological functions. Interferon was shown to have many effects on the immune system, including activation of natural killer cells in vivo and elevation of serum immunoglobulin and cerebrospinal fluid IgG ratios. Furthermore, all patients who received IFN developed antibody titers to a protein contaminant (molecular weight, 27 kilodaltons) in the IFN preparation. This antibody was associated with an elevation in serum concentrations of Clq‐ and Raji‐binding immune complexes in 6 of 12 patients. Some of these 12 patients developed symptoms suggestive of immune complex disease. IFN had pronounced effects on the immune system of these patients.
A derivative of myelin‐associated glycoprotein in cerebrospinal fluid of normal subjects and patients with neurological disease
Authors: Yanagisawa Katsuhiko , Quarles Richard H. , Johnson David , Brady Roscoe O. , Whitaker John N.
Published: January 2005

A procedure for quantitating the myelin‐associated glycoproiein (MAG) in cerebrospinal fluid (CSF) was developed. The procedure involved immunoprecipitation with rabbit polyclonal anti‐MAG antiserum followed by immune staining of electroblots with the monoclonal antibody HNK‐1. This method could detect as little as 2 ng of MAG per ml of CSF and could accurately measure levels of 5 ng/ml or greater. No intact MAG was detected in any of the CSF samples examined, but all samples contained the 90K dalton proteolytic derivative of MAG called dMAG. Samples from normal volunteers and patients with demyelinating diseases contained levels of dMAG ranging from 2 to 13 ng/ml. There was no correlation of dMAG levels with levels of myelin basic protein or with active demyelinating disease. The relatively high levels of dMAG, even in control CSF samples, probably resulted from normal physiological turnover of MAG and could possibly obscure any additional increments of MAG or dMAG release that might occur as a result of demyelination.
Neurogenic flare responses following topical application of capsaicin in humans
Authors: Helme R. D. , McKernan S.
Published: January 2005

The flare response to noxious stimulation of the skin is mediated by polymodal nociceptors of C fiber primary afferent nerves. Topical application of capsaicin initiates a flare response and burning pain. In this study, the variability of capsaicin‐induced flare and pain was assessed in 220 subjects. The major factors in flare response are body site and age; more severe reactions occur in more proximal sites and in younger subjects. Larger flares were shown to be associated with greater pain. Variability is probably due to differences in the structure and reactivity of the neurovascular unit as proposed in the Lewis model of the axon reflex. It may prove possible to assess polymodal nociceptor function using topical capsaicin in disease states that affect the peripheral terminals of primary afferent nerves.
The effect of carbidopa on the pharmacokinetics of intravenously administered levodopa: The mechanism of action in the treatment of parkinsonism
Authors: Nutt John G. , Woodward William R. , Anderson John L.
Published: January 2005

The pharmacokinetics of short and long intravenous infusions of levodopa with and without concurrent oral administration of carbidopa was studied in 9 parkinsonian patients. Carbidopa reduced by 50% both the infusion rate required to produce a clinical response and the time required for plasma clearance of levodopa. Using this value for clearance, it is estimated that carbidopa doubles the bioavailability of orally administered levodopa. Carbidopa did not alter the therapeutically effective plasma concentration of levodopa, suggesting that carbidopa does not modify the so‐called enzymatic blood‐brain barrier. The decline of the plasma levodopa concentration was biphasic; carbidopa modestly increased half‐lives of both phases. The apparent volume of distribution was not significantly altered. Carbidopa did not change the duration of the clinical response after the discontinuation of short infusions. From these observations we infer that the therapeutic effects of carbidopa can be attributed to doubling the bioavailability of orally administered levodopa and halving its plasma clearance.
Neuropathy associated with experimental Chagas' disease
Authors: Said Gérard , Joskowicz Mireille , Barreira Amilton Antunes , Eisen Harvey
Published: January 2005

Mice were acutely and chronically infected with Trypanosoma cruzi and then examined histologically for the presence of lesions in the peripheral nervous system. In acutely infected animals, small lymphocytic and macrophagic infiltrates were found in the nerves in association with intracellular parasites. Little or no nerve damage was present at this stage. In chronically infected animals, large perivascular granulomatous infiltrates were found in association with multifocal, predominantly demyelinative lesions of neighboring nerve fibers. Similar inflammatory infiltrates were present in muscles and were frequently associated with vasculitis and destruction of muscle fibers. Our pathological findings and the negative results produced by intraneural injections of sera from chronically infected animals and the positive results following injections of small numbers of live trypanosomes suggest that the demyelination is not due to circulating serum factors such as antibodies cross‐reacting with peripheral myelin. Delayed‐type hypersensitivity may be induced by the presence of the parasites, a notion supported by the development of granulomas in naive mice injected intravenously with helper T cells from chronically infected animals.
Thyrotropin‐releasing hormone enhances motor neuron–evoked contractions of cultured human muscle
Authors: Askanas Valerie , Engel W. King , Kobayashi Takayoshi
Published: January 2005

Thyrotropin‐releasing hormone (TRH), 1 to 2.7 mM, introduced to the medium of monolayer‐cultured human muscle innervated by cocultured fetal rat spinal cord with dorsal root ganglia, increased muscle fiber contractions 4.9 times (p < 0.005). Doses higher than 5 mM, after increasing contractions for a few seconds, caused complete inhibition of contraction (autorefractoriness). Neither aneurally cultured human muscle nor innervated cultured human muscle blocked by 1 mM d‐tubocurarine was excited by TRH. Thus, in this system TRH appeared to excite muscle contractions through direct or indirect action on motor neurons.
Nocturnal plasma prolactin rise in patients with complex partial seizures
Authors: Molaie Majid , Culebras Antonio , Miller Myron
Published: January 2005

We measured plasma prolactin and cortisol concentrations in 12 epileptic men with complex partial seizures during all‐night polygraphic recording, with continuous video monitoring and sequential blood sampling at 30‐minute intervals. There was a significant but moderate elevation of all‐night mean plasma prolactin concentration (p < 0.001), but not of cortisol, in epileptics when compared with normal subjects studied in a similar fashion. The data obtained in this environmentally controlled study of males with complex partial seizure disorder indicate a disruption of prolactin regulatory mechanisms resulting in a modest elevation of plasma concentrations independent of ictal electrographic discharges.
Anticerebellar antibodies in serum and cerebrospinal fluid of a patient with oat cell carcinoma of the lung and paraneoplastic cerebellar degeneration
Authors: Greenlee John E. , Lipton Howard L.
Published: January 2005

A 56‐year‐old man was seen with subacute cerebellar degeneration and was found to have oat cell carcinoma of the lung. Antibodies to cerebellar Purkinje cells and granule cells were detected in both serum and cerebrospinal fluid (CSF), and intrathecal antibody synthesis was suggested by serum CSF antibody ratios, CSF IgG index, and CSF IgG synthesis rate. The patient's condition improved slightly with plasmapheresis, corticosteroids, and therapy of his underlying tumor, but he continued to exhibit a severe cerebellar deficit until his death more than one year later. Paraneoplastic cerebellar degeneration may be accompanied by synthesis of anticerebellar antibodies, both systemically and within the central nervous system.
Monoclonal antibody analysis of mononuclear cells in myopathies. III: Immunoelectron microscopy aspects of cell‐mediated muscle fiber injury
Authors: Arahata Kiichi , Engel Andrew G.
Published: January 2005

We have previously obtained light microscopical immunocytochemical evidence for cell‐mediated muscle fiber injury and destruction in polymyositis and inclusion body myositis [1, 2]. To evaluate further interactions of the different cell phenotypes with each other and with the muscle fibers, the T8, T4, and Leu 7 markers in 7 cases of polymyositis and in 9 cases of inclusion body myositis were localized by immunoelectron microscopy. In the early stages of the cell‐mediated process, T8+ cells and macrophages are apposed against, and/or send spikelike processes into, nonnecrotic muscle fibers. Leu‐7+ cells penetrate fibers infrequently, and T4+ cells do not penetrate muscle fibers. Subsequently, an increasing number of T8+ cells and macrophages traverse the basal lamina; focally replace, displace, or compress the fiber; and spikes from these cells honeycomb the adjacent muscle fiber regions. The macrophages contain only few heterophagic vacuoles and therefore act in a cytotoxic rather than a phagocytic capacity. The integrity of the muscle fiber surface membrane facing the invading cells is maintained, but the possibility also exists that the membrane is damaged and rapidly repaired, or that the damage cannot be detected by electron microscopy. Nearby fiber regions often show either degenerative or regenerative changes. Ultimately, segments of the entire muscle fiber are replaced by the invading cells.
A new syndrome of anosmia, ichthyosis, hypogonadism, and various neurological manifestations with deficiency of steroid sulfatase and arylsulfatase C
Authors: Sunohara Nobuhiko , Sakuragawa Norio , Satoyoshi Eijiro , Tanae Ayako , Shapiro Larry J.
Published: January 2005

We describe a family consisting of 3 affected men with congenital ichthyosis, anosmia, hypogonadism, nystagmus with decreased visual acuity, strabismus, hypopigmentation of the iris, and mirror movements of the hands and feet. Two of them had limitation of ocular movement and unilateral renal agenesis or hypoplasia. The condition appears to be inherited as an X‐linked recessive trait. Clinical, pathological, and biochemical evaluations were compatible with a diagnosis of X‐linked ichthyosis. Steroid sulfatase and arylsulfatase C activities in leukocytes and fibroblasts were markedly diminished in the affected patients. Their hypogonadism was due to decreased luteninizing hormone–releasing hormone secretion (hypogonadotropic). Hyposecretion of antidiuretic hormone was also recognized. Chromosome analysis of leukocytes and skin fibroblasts revealed a normal 46,XY male karyotype in all of the patients.
Topical therapy for oropharyngeal symptoms of myasthenia gravis
Authors: Dooley J. M. , Goulden K. J. , Gatein J. G. , Gibson E. J. , Brown B. St. J.
Published: January 2005

Inhibitors of acetylcholinesterase are commonly used in the treatment of myasthenia gravis. We studied a patient with mainly bulbar myasthenia gravis who did not tolerate oral pyridostigmine despite a clear clinical response. Treatment with nebulized pyridostigmine reduced her symptoms without systemic side effects. This route of therapy may benefit other patients.
Status epilepticus: Divergence of sympathetic activity and cardiovascular response
Authors: Benowitz Neal L. , Simon Roger P. , Copeland James R.
Published: January 2005

To study changes in plasma catecholamine concentrations and their relationship to changes in systemic and pulmonary vascular pressures, we induced status epilepticus by intravenous administration of bicuculline in 5 anesthetized, paralyzed, ventilated adult sheep. Status epilepticus was accompanied by marked elevations in plasma epinephrine and norepinephrine levels (390‐fold and 163‐fold, respectively) and by marked elevations in systemic and pulmonary vascular pressures. Vascular pressure returned to normal in 60 minutes, despite plasma catecholamine levels that remained elevated for the 3‐hour duration of the study. Mild metabolic acidosis and hyperkalemia were also noted. We discuss the implications of these findings with regard to the management of hypotension during status epilepticus.
Accumulation of glycogen in sural nerve axons in adult‐onset type III glycogenosis
Authors: Ugawa Yoshikazu , Inoue Kiyoharu , Takemura Tamiko , Iwamasa Teruo
Published: January 2005

A 42‐year‐old man with adult‐onset type III glycogenosis (Cori's disease) developed a gradually progressive polyneuropathy with markedly reduced activity of muscle amylo‐1,6‐glucosidase and glycogen accumulation within all elements of biopsied sural nerve, including axons, as shown by ultrastructural assessment.
Long‐term follow‐up of early dopa treatment in Parkinson's disease
Authors: Markham Charles H. , Diamond Shirley G.
Published: January 2005

A group of 19 patients with Parkinson's disease, who began dopa therapy 1 to 3 years after onset of symptoms, were followed for 12 years. They were evaluated every 3 to 4 months on the UCLA disability scale and the Hoehn and Yahr functional classification scale. This present group was compared to two other historical groups reported on earlier [8], one that began treatment with dopa 4 to 6 years after onset of symptoms and the other that began dopa treatment 7 to 9 years after onset of symptoms. Results on both scales showed that the mean disability scores of the three groups did not differ significantly when duration of disease was matched, even though duration of dopa therapy among the groups varied from 1 to 12 years. The highly significant differences between the groups prior to dopa treatment continued throughout the study. These longitudinal data support the conclusion that the worsening over time is the result of progression of Parkinson's disease and not a result of the duration of dopa treatment. Early treatment improves the beginning years of the disease and has no adverse effect on later years.

Twelve years after beginning dopa therapy, 32% of the present group had died at a mean age of 76.0, in contrast to 50% (mean age, 74.7) and 57% (mean age, 71.5) in the groups who began dopa increasingly later in the course of their disease. Incidence of dementia increased with disease duration regardless of treatment duration. In the present group, 3 of 19 patients showed severe cyclinical fluctuations, but that complication as well as persistent chroreoathetosis and the rarely seen true on‐off effect also could not be related to duration of dopa treatment.
Oligoclonal immunoglobulin bands in cerebrospinal fluid of a patient with lymphocytic choriomeningitis
Authors: Jamieson Dara G. , Mehta Pankaj D. , Lavi Ehud
Published: January 2005

We describe a patient with meningitis caused by lymphocytic choriomeningitis virus. Oligoclonal IgG bands were found in the patient's cerebrospinal fluid during acute and convalescent stages of the illness, and results of liver function tests were abnormal. Acute attacks of lymphocytic choriomeningitis virus infection in humans can be added to the list of diseases associated with cerebrospinal fluid oligoclonal IgG.
Pathogenesis of diabetic neuropathy
Authors: Johnson Peter C. , Doll Susan C. , Cromey Douglas W.
Published: January 2005

Samples of lumbosacral trunk, posterior tibial nerve, and sural nerve obtained at autopsy from diabetic and nondiabetic patients without mononeuropathy multiplex were evaluated using 1‐μ‐thick epoxy sections and teased nerve fiber preparations. Focal fascicular lesions characterized by reduced density of myelinated axons within fascicles were found predominantly in the specimens from diabetics, mainly in the posterior tibial nerve and lumbosacral trunk. In severe examples, the perineurium and even surrounding epineurium were damaged, stamping the lesions as ischemic. In addition, identical lesions were found in biopsies of nerves of nondiabetics with vascultis. Density of myelinated fibers at the three sites demonstrated a proximal–distal graded loss that was significantly greater in the diabetic samples. The loss from the lumbosacral trunk to the posterior tibial nerve was correlated with the density of focal lesions in the lumbosacral trunk in the diabetic (p = 0.025), indicating that distal fiber loss was partly due to the focal lesions. Teased nerve fiber abnormalities were common only in sural nerves of diabetics, suggesting that they are secondary. We conclude that beyond the possible metabolic abnormalities involved in the genesis of diabetic polyneuropathy, focal fascicular lesions, likely due to diabetic microangiopathy, are also important in the development of diabetic neuropathy.
Magnetic resonance imaging in subacute necrotizing encephalomyelopathy (Leigh's disease)
Authors: Koch Thomas K. , Yee Melvin H. C. , Hutchinson Harrol T. , Berg Bruce O.
Published: January 2005

Magnetic resonance imaging (MRI) offers a sensitive alternative to computed tomography (CT) for lesion localization. Patients with subacute necrotizing encephalomyelopathy (SNE) may be diagnosed by finding focal lesions on CT that correspond to sites of anatomical involvement. We report serial CT and MRI scanning findings in a patient with clinical, radiographic, and laboratory evidence of SNE. MRI was more sensitive in detecting lesions involving the basal ganglia, brainstem, and cortex. We believe MRI is a valuable and sensitive means to establish an antemortem diagnosis of SNE.
Encephalitis caused by Coxiella burnetii
Authors: Brooks Robert G. , Licitra Carmelo M. , Peacock Marius G.
Published: January 2005

Acute infection with Coxiella burnetii usually results in a self‐limited illness, but it can occasionally cause chronic endocarditis or hepatitis. Headache is a common presenting symptom of acute infection with this agent, but specific neurological abnormalities are rare. WE report the case of a patient with acute Q fever that caused frank encephalitis. We also review the literature on central nervous system disease attributable to C. burnetii.
Preferential loss of large lumbar primary sensory neurons in carcinomatous sensory neuropathy
Authors: Ohnishi Akio , Ogawa Michiko
Published: January 2005

Morphometric studies at autopsy of a patient with sensory neuropathy associated with small‐cell lung carcinoma showed preferential loss of large‐diameter sensory nerve cell bodies (fifth lumbar dorsal root ganglion), marked decrease of large myelinated fibers in the dorsal root and sural nerve, and almost total loss of myelinated fibers in the fasciculus gracilis.
Combined 1H and 31P nuclear magnetic resonance spectroscopic studies of bicuculline‐induced seizures in vivo
Authors: Petroff Ognen A. C. , Prichard James W. , Ogino Takashi , Avison Malcolm , Alger Jeffry R. , Shulman Robert G.
Published: January 2005

Using a 1.89‐Tesla spectrometer, 1H and 31P nuclear magnetic resonance spectra were acquired from the brains of paralyzed rabbits ventilated with 30% oxygen in nitrous oxide. Intracellular pH and changes in lactate concentration in the cerebrum were monitored by nuclear magnetic resonance methods during and after bicuculline‐induced seizures, together with the electroencephalogram, heart rate, and arterial blood pressure. During seizures lasting more than an hour, cerebral intracellular pH became acidic, the cerebral lactate level rose rapidly, and both changes persisted as long as 2 hours without signs of recovery. After less prolonged seizures, lactate elevations were no less persistent, despite nearly complete recovery of intracellular pH and the electroencephalogram.
Parkinson's disease: Nigral receptor changes support peptidergic role in nigrostriatal modulation
Authors: Uhl George R. , Hackney Gail O. , Torchia Mary , Stranov Victoria , Tourtellotte Wallace W. , Whitehouse Peter J. , Tran Vinh , Strittmatter Steven
Published: January 2005

Autoradiographic studies reveal densities of binding to somatostatin, neurotensin, μ‐opiate, and benzodiazepine receptors in substantia nigra specimens from neurologically normal human brains. Binding to nigral angiotensin converting enzyme is also dense, whereas more modest densities of κ‐opiate, dopamine, and serotonin receptors are noted. In nigral specimens taken from patients with idiopathic Parkinson's disease, substantial reductions in somatostatin, neurotensin, μ‐opiate and κ‐opiate receptors contrast with more modest reductions in dopamine and benzodiazepine I receptor subtypes. Angiotensin converting enzyme, serotonin, and benzodiazepine II binding are virtually unaltered. These results underscore the likelihood of strong peptidergic influences on normal and pathologically altered human nigrostriatal circuitry.
Selective saccadic palsy caused by pontine lesions: Clinical, physiological, and pathological correlations
Authors: Hanson Maurice R. , Hamid Mohamed A. , Tomsak Robert L. , Chou Samuel S. , Leigh R. John
Published: January 2005

Two patients suffered a selective deficit of voluntary saccades and quick phases of nystagmus after hypoxic–ischemic insults during open‐heart surgery. All voluntary saccades, in both horizontal and vertical planes, were slow, and quick phases of vestibular and optokinetic nystagmus were absent. Smooth pursuit, the vestibuloocular reflex, the ability to hold steady eccentric gaze, and vergence eye movements were all preserved. Pathological studies in 1 patient confirmed neuronal necrosis and gliosis, consistent with ischemic lesions involving the median and paramedian pontine reticular formation and median basis pontis but sparing the rostral mesencephalon and rostral interstitial nucleus of the medial longitudinal fasciculus. These findings, taken with data from experimental studies, support the hypothesis that each functionally defined class of horizontal eye movements is controlled by a separate neural substrate that projects independently to the abducens nuclei. In addition, these data suggest that the rostral interstitial nucleus of the medial longitudinal fasciculus is dependent on inputs from the paramedian pontine reticular formation for the programming of normal vertical saccades.
Familial myoclonic dementia masquerading as Creutzfeldt‐Jakob disease
Authors: Little Brian W. , Brown Paul W. , Rodgers‐Johnson Pamela , Perl Daniel P. , Gajdusek D. Carleton
Published: January 2005

We describe a kindred with 7 confirmed and 2 probable cases of subacute dementia accompanied by myoclonus. The inheritance pattern is consistent with autosomal dominance and shows anticipation. The pathological changes involve marked gliosis with neuronal loss of the dorsomedial and midline thalamic nuclei, with lesser involvement of the anterior, lateral, and posterior thalamic nuclei. Medullary olivary hypertrophy is prominent. Spongiform change is minimal or absent. Attempted disease transmission to primates from 3 affected family members has been unsuccessful; Western immunoblot tests have likewise failed to detect the presence of specifically reactive 27–30 kD Mr proteins in brain tissue from 2 of these patients. We conclude that this family has a rare inherited neurological systems degeneration with associated thalamic dementia, the clinical course of which is very similar to Creutzfeldt‐Jakob disease.
Peripheral neuropathy associated with mitochondrial myopathy
Authors: Yiannikas C. , McLeod J. G. , Pollard J. D. , Baverstock J.
Published: January 2005

Twenty patients with mitochondrial myopathy were investigated for the presence of peripheral neuropathy. There were clinical features of a mild sensorimotor neuropathy in 5 patients (25%) and nerve conduction studies were abnormal in 10 patients (50%). Electrophysiological studies of the whole group showed significant impairment of motor and sensory conduction, compared with controls. Sural nerve biopsy and morphometric studies were performed on 4 patients with clinical neuropathy. There was a reduction in density of myelinated fibers and electron microscope features of axonal degeneration affecting myelinated and unmyelinated fibers. Abnormal mitochondria containing paracrystalline inclusions were seen in the Schwann Cell cytoplasm of two nerves.
Immunoglobulin abnormalities in cerebrospinal fluid and blood over the course of lymphocytic meningoradiculitis (Bannwarth's syndrome)
Authors: Henriksson Annemarie , Link Hans , Cruz Mabel , Stiernstedt Göran
Published: January 2005

The conditions of 5 patients with untreated lymphocytic meningoradiculitis (Bannwarth's syndrome, probably equivalent to Lyme disease) with serologically confirmed infection resulting from Borrelia spirochetes were followed with repetitive lumbar punctures up to 221 days after the onset of symptoms. Using a protein A plaque assay, high numbers of IgG‐, IgM‐, and IgA‐producing cells were found in the cerebrospinal fluid (CSF), whereas there were mostly normal numbers of immunoglobulin‐producing cells in peripheral blood. A markedly increased CSF IgM index and an elevated IgG index were observed in all patients during the early phase, reflecting production of these immunoglobulins within the central nervous system. All patients had oligoclonal IgG bands in the CSF that persisted during follow‐up; in the 2 patients tested, the bands contained Borrelia antibodies. Most serum immunoglobulin concentrations were normal and in only 1 patient was it possible to detect in serum some of the oligoclonal IgG bands present in CSF. Declining numbers of CSF cells producing immunoglobulin and decreasing immunoglobulin index values were observed during follow‐up, but 3 patients had an elevated CSF IgM index in the presence of normal IgG and IgA indices when examined during the later phases of disease. An intense and prolonged IgM response within the central nervous system seems to be a characteristic of the disease.