bluuebunny/crossref_metadata_2025_split huggingface dataset:
Free amino acid levels in amyotrophic lateral sclerosis
Authors: Patten Bernard M. , Harati Yadollah , Acosta Luis , Jung Shin‐Siung , Felmus Mark T. 
Published: January 2005

AbstractTo evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in serum levels of tyrosine, total aromatic AA, and total basic AA. ALS patients also had statistically significant elevations in cerebrospinal fluid of total basic AA, lysine, essential AA, and leucine. The severity of ALS correlated inversely with acidic AA (glutamate and aspartate) and O‐phosphoserine in cerebrospinal fluid. Activity of ALS correlated directly with serum aspartate and cerebrospinal fluid alanine.We conclude that subtle abnormalities of AA levels are present in ALS and that these are not due to age, sex, or disability. The pattern of distribution of AA levels differs from that in hepatic or renal disease and suggests defective membrane transport or poor cellular utilization of basic and essential AA in the central nervous system.
Effect of prednisolone on motor end‐plate fine structure: A morphometric study in hamsters
Authors: Chokroverty S. , Reyes M. G. , Chokroverty M. , Kaplan R. 
Published: January 2005

AbstractThe fine structure of quadriceps motor end‐plates in hamsters was analyzed quantitatively one, two, four, seven, and thirty‐two weeks following intraperitoneal injections of prednisolone. Except for transient increases in postsynaptic length and membrane profile concentration after prednisolone administration at dosages of 4 mg per kilogram of body weight for one week and 2 mg per kilogram for four weeks, mean values for various measurable profiles in the presynaptic and postsynaptic regions showed no significant differences between control and treated animals.
Juvenile neuroaxonal dystrophy: Clinical, electrophysiological, and neuropathological features
Authors: Dorfman Leslie J. , Pedley Timothy A. , Tharp Barry R. , Scheithauer Bernd W. 
Published: January 2005

AbstractWe describe 2 brothers with progressive myoclonus epilepsy that began in the second decade and was associated with cerebellar ataxia and intellectual deterioration. Electroencephalographic and cerebral evoked potential studies showed findings associated with myoclonus epilepsy. Neuropathological examination of 1 of the brothers, who died at age 23 years, revealed widespread changes of neuroaxonal dystrophy without pigment deposition in the basal ganglia. We propose the term juvenile neuroaxonal dystrophy (JNAD) to distinguish this condition on clinical grounds from infantile neuroaxonal dystrophy on the one hand, and on clinical and pathological grounds from Hallervorden‐Spatz disease on the other hand. JNAD, while exceedingly rare, must be considered in the differential diagnosis of the progressive myoclonus epilepsies.
Attenuated dynamic responses of primary endings of muscle spindles: A basis for depressed tendon responses in acrylamide neuropathy
Authors: Lowndes Herbert E. , Baker Thomas , Michelson Leslie P. , Vincent‐Ablazey Martha 
Published: January 2005

AbstractDepressed or lost tendon reflexes commonly observed in patients with peripheral neuropathies may result partly from attenuation of the dynamic discharge from the primary endings of muscle spindles. This possibility was investigated in cats with an experimental neuropathy induced with acrylamide (30 mg/kg/day intramuscularly). Achilles tendon reflexes and the dynamic discharge from primary muscle spindles were evaluated after five or ten injections of acrylamide. After five injections the animals were moderately impaired neurologically, the Achilles tendon reflex was difficult to elicit in 2 of 5 animals, and the dynamic responses of primary endings of soleus muscle spindles to stretch were depressed. Following ten injections of acrylamide the cats were severely impaired neurologically; tendon responses were either absent or difficult to elicit, and the dynamic responses of their muscle spindles to stretch were reduced by 50% (p < 0.01). Additionally, the spindles responded to stretch with only 20 to 30% the normal number of afferent impulses. These data suggest that lost tendon responses in acrylamide neuropathy result in part from inadequate activation of motoneurons by spindle afferent discharge.
Transient cerebral dysfunction following chemotherapy for osteogenic sarcoma
Authors: Allen Jeffrey C. , Rosen Gerald 
Published: January 2005

AbstractAn unusual neurological syndrome occurred in 4 of 158 patients treated for osteogenic sarcoma with combination chemotherapy. There was an abrupt onset of focal cerebral deficits approximately ten days after chemotherapy with vincristine and high‐dose methotrexate plus citrovorum factor rescue. The syndrome was short lived and always occurred early in the course of treatment. Prolonged neurological deficits remained in 2 patients. When similar chemotherapy was reinstituted in the 4 patients, no further neurological complications ensued. Possible causes include a leukoencephalopathy related to methotrexate or an embolic cerebral vasculopathy related to necrotic tumor microemboli emanating from the lungs.
Huntington disease associated with Alzheimer disease
Authors: McIntosh Gerald C. , Jameson H. Douglas , Markesbery William R. 
Published: January 2005

AbstractThe clinicopathological study of a case of Huntington disease with early severe dementia is presented. The pathological findings were those of Huntington disease (atrophy with neuronal loss and astrocytosis in the neostriatum) and Alzheimer disease (neuronal loss with numerous senile plaques and neurofibrillary tangles in the neocortex). Ultrastructural study of neurofibrillary tangles showed that they were composed of twisted tubules similar to those found in Alzheimer disease.
Ultrastructure and biochemistry of sympathetic ganglia in idiopathic orthostatic hypotension
Authors: Petito Carol K. , Black Ira B. 
Published: January 2005

AbstractThe role of transsynaptic dysfunction in the pathogenesis of idiopathic orthostatic hypotension (IOH, or idiopathic autonomic insufficiency) was examined microsocopically and biochemically in autopsy specimens. Light microscopy of the sympathetic ganglia showed abnormalities in all 4 IOH patients, including focal phagocytosis of neurons, increased numbers of satellite cells, and perivascular lymphocytic infiltrates. Electron microscopy revealed proliferation and hypertrophy of satellite cells and abnormalities in the unmyelinated axons. In constrast, the spinal cord intermediolateral columns, containing the presynaptic neurons, were unremarkable in 1 patient, exhibited only mild gliosis in another, and showed neuron loss and fibrillary gliosis in 2 patients.Postsynaptic dopamine‐β‐hydroxylase (DBH) activity was decreased at least fourfold (p < 0.02) in sympathetic ganglia of patients with IOH, while tyrosine hydroxylase (T‐OH) was normal. Ganglion choline acetyltransferase (ChAc) activity, an index of presynaptic function and integrity, was normal in the IOH group.A number of our observations suggest that presynaptic disease is not an absolute requirement for adrenergic abnormalities in IOH. The intermediolateral columns of the spinal cord were histologically normal in 2 of the patients with IOH, and ultrastructural abnormalities in sympathetic ganglia were consistent with primary adrenergic degeneration. In addition, presynaptic ChAc activity was normal in IOH ganglia, whereas postynaptic DBH activity was depressed. Finally, postsynaptic T‐OH activity, which is regulated by transsynaptic mechanisms, was normal in IOH ganglia.
Computed tomography in late‐infantile metachromatic leukodystrophy
Authors: Buonanno Ferdinando S. , Ball Marshall R. , Laster D. Wayne , Moody Dixon M. , McLean William T. 
Published: January 2005

AbstractComputed tomography of 2 patients with late‐infantile metachromatic leukodystrophy (MLD) demonstrated generalized white matter lucency and moderate ventricular enlargement. CAT scan findings in other white matter diseases likely to be confused clinically with MLD are sufficiently dissimilar to allow differentiation.
The prognosis of acute and subacute transverse myelopathy based on early signs and symptoms
Authors: Ropper Allan H. , Poskanzer David C. 
Published: January 2005

AbstractFifty‐two patients with acute and subacute transverse myelopathy (TM) were evaluated at the Massachusetts General Hospital between 1955 and 1975 and followed for 1 to 23 years (average, 5). Nineteen had symptoms of a recent acute infectious illness, 3 had cancer, and 1 had undergone a recent operation. There were four types of initial symptom. Twenty‐four patients had paresthesias at the onset of the illness, 18 had pain, usually interscapular, 7 had leg weakness, and 3 had urine retention. Prognosis depended on the nature of the onset of TM. An acute catastrophic onset was generally associated with back pain and led to a poor outcome in 7 and a good outcome in only 1 of 11 patients. A subacute progressive onset over several days to four weeks, generally with ascending paresthesias or leg weakness, was associated with a good outcome in 15 and fair outcome in 17 of 37 patients. Preceding febrile illness, treatment with corticosteroids, and the nature of CSF abnormalities had no effect on outcome. Multiple sclerosis evolved in 7 patients during the follow‐up period. Because of the frequency with which mass lesions were missed, the need for myelography in the diagnosis of TM is emphasized.The distinguishing clinical characteristics of TM provide guidelines for diagnosis and prognosis.
Pharmacological studies of the pupils in familial amyloid polyneuropathy
Authors: Okajima Toru , Nagata Jinro , Hatamoto Kazuko , Kinoshita Yoshimi , Takaba Yoshio , Tokuomi Haruhiko 
Published: January 2005

AbstractPharmacological studies of the pupils of 7 patients with familial amyloid polyneuropathy (FAP) were performed in order to demonstrate the site of the lesion. Supersensitivity to 1.25% epinephrine was observed frequently, but not to 2.5% methacholine. The lesions of the sympathetic nervous system, judging from the epinephrine and tyramine tests, were regarded as preganglionic in 2 patients and postganglionic in 4. Among 3 patients reexamined six months later, 1 had developed signs of sympathetic postganglionic disturbance and another showed a peripheral parasympathetic disturbance associated with progress of a sympathetic preganglionic disturbance. From these results it was suggested that in FAP, sympathetic abnormalities involve the pupils more often than parasympathetic disturbances, and pharmacological studies can detect the autonomic dysfunction before it becomes clinically apparent.
Peripheral nerve conduction in patients with a cervical rib and band
Authors: Gilliatt R. W. , Willison R. G. , Dietz V. , Williams I. R. 
Published: January 2005

AbstractIn 14 patients with wasting of the hand due to a cervical rib and band, motor and sensory conduction studies on the peripheral parts of the median and ulnar nerves were helpful in establishing the correct diagnosis. The median nerve findings excluded carpal tunnel syndrome even when the clinical pattern of wasting in the hand suggested this diagnosis. Preservation of conduction velocity in the ulnar nerve excluded ulnar entrapment at the elbow; the reduced amplitude of the ulnar sensory action potentials (SAPs) indicated that the lesion was distal to the dorsal root ganglia. In 3 patients with ulnar SAP amplitudes that were low but not clearly abnormal, the level of the lesion was confirmed by a reduced response to intradermal injection of histamine on the inner side of the forearm.
The cerebrospinal fluid in visna, a slow viral disease of sheep
Authors: Griffin Diane E. , Narayan Opendra , Bukowski Jack F. , Adams Robert J. , Cohen Steven R. 
Published: January 2005

AbstractThe temporal development of the CSF abnormalities was examined in visna, a chronic neurological disease caused by a persistent viral infection of sheep. Two types of changes were observed. During the first 30 to 40 days there was an intense mononuclear pleocytosis associated with high protein and proportionately elevated IgG. Later, there was a persistent slight pleocytosis associated with mildly elevated protein which had an increased proportion of IgG and antivisna antibody. These later cellular and protein changes are similar to those found in a number of chronic neurological diseases of man.
Relationship of lymphocyte invasion and survival of brain tumor patients
Authors: Brooks W. H. , Markesbery W. R. , Gupta G. D. , Roszman T. L. 
Published: January 2005

AbstractThe length of survival of 149 patients harboring primary brain tumors was retrospectively correlated with the presence and location of lymphocytic infiltration. Mononuclear invasion of malignant gliomas confined to the perivascular spaces was the only histological finding that correlated significantly with survival. Patients with malignant gliomas containing perivascular infiltration lived up to four months longer than those with no lymphocyte infiltration. The survival of patients with brain tumors who had lymphocyte invasion associated with areas of hemorrhage and necrosis beyond and separate from perivascular spaces was the same as for those with no lymphocyte involvement.
Acetylcholine receptor in myasthenia gravis: Increased affinity for α‐bungarotoxin
Authors: Elias Stanton B. , Appel Stanley H. 
Published: January 2005

AbstractStudies of the binding of 125I‐labeled α‐bungarotoxin to myasthenic motor end‐plates have been interpreted as showing a decrease in the number of acetylcholine (ACh) receptors at these end‐plates. Equilibrium binding studies of 125I‐tagged α‐bungarotoxin to detergent‐extracted ACh receptors from normal and myasthenic intercostal muscle were carried out to determine whether the reduced toxin binding previously reported could be due to a reduced affinity of myasthenic receptors for α‐bungarotoxin rather than to a decreased number of receptors. Our results show increased rather than decreased affinity of myasthenic receptors for α‐bungarotoxin and also suggest that the number of ACh receptors is indeed reduced. The presence of a change in binding affinity, in addition to the reduced number of ACh receptors, suggests the presence of membrane changes that may contribute to the pathogenesis of myasthenia gravis.
Proteolytic enzyme activity of blood leukocytes and cerebrospinal fluid in multiple sclerosis
Authors: Cuzner M. Louise , Davison Alan N. , Rudge Peter 
Published: January 2005

AbstractUpon stimulation by immune complexes, the polymorphonuclear (PMN) blood cell secretes lysosomal hydrolases, including neutral proteinase, which is concentrated in the PMN cell. Neutral and acid proteinase activity were increased and decreased, respectively, in the circulating white cells of patients with multiple sclerosis during an exacerbation of the disease, but there was no correlation with serum immune complex levels. Neutral proteolytic activity in the cellular fraction of the cerebrospinal fluid was also found to be elevated in acute multiple sclerosis, as monitored by digestion of myelin basic protein.
Rapid improvement in nerve conduction velocity following renal transplantation
Authors: Oh Shin J. , Clements Rex S. , Lee Young W. , Diethelm Arnold G. 
Published: January 2005

AbstractIn 12 patients with chronic renal failure who received kidney transplants from either cadavers (6) or related living donors (6), rapid improvement in median sensory and motor nerve conduction velocities (NCVs) was observed within a few days after transplantation. The postoperative improvement in median sensory NCV was found to bear a statistically significant negative correlation with creatinine and myo‐inositol concentrations. We suggest that metabolic phenomena are responsible for the rapid improvement in median sensory NCV following renal transplantation. The close relationship between myo‐inositol and the median sensory NCV following transplantation suggests that elevated plasma myo‐inositol concentrations may be related to nerve conduction abnormalities in uremic polyneuropathy.
Systemic lupus erythematosus, myasthenia gravis, and ehlers‐danlos syndrome
Authors: Branch C. E. , Swift T. R. 
Published: January 2005

AbstractClinical, electrophysiological, and laboratory data are reported on a 21‐year‐old black woman with systemic lupus erythematosus, myasthenia gravis, and Ehlers‐Danlos syndrome. The first two diseases are related by similar pathogenetic mechanisms. To our knowledge, this is the first patient reported with this unusual combination of rare diseases.
The long‐term prognosis in untreated cerebral aneurysms: II. Late morbidity and mortality
Authors: Winn H. Richard , Richardson Alan E. , O'Brien William , Jane John A. 
Published: January 2005

AbstractThree hundred sixty‐four patients who suffered a subarachnoid hemorrhage from an aneurysm at either the anterior (ACA) or posterior (PCA) communicating artery location and who were not surgically treated have been followed for up to 21 years in order to study the late morbidity and mortality. For patients surviving six months: (1) little improvement in morbidity was noted during the many years of subsequent follow‐up; (2) in general, ACA patients fared better than PCA patients; (3) the neurological state on original admission was strongly correlated with the degree of morbidity; (4) other factors measured at time of original hemorrhage which adversely affected morbidity included the level of blood pressure or the presence of clot or spasm; (5) if deaths from late hemorrhage are excluded, cardiovascular causes account for the majority of subsequent deaths; and (6) the risk of dying from all causes in increased when compared to a population matched by age and sex.
Experimental allergic encephalomyelitis in cobra venom factor—treated and C4‐deficient guinea pigs
Authors: Morariu Mircea A. , Dalmasso Agustin P. 
Published: January 2005

AbstractExperimental allergic encephalomyelitis (EAE) was studied in guinea pigs with an inherited deficiency of the fourth component of complement (C4) and in guinea pigs injected with cobra venom factor to deplete the third component and late‐acting components of complement. EAE was elicited by immunization with homologous spinal cord or purified basic protein. Administration of cobra factor after the injection of encephalitogenic emulsion delayed the onset and reduced the intensity of the clinical manifestations of EAE. In addition, cobra factor markedly reduced mortality during the sixty days of observation. However, pathological changes of perivascular infiltration and demyelination were similar in cobra factor–treated and untreated animals. Clinical signs of EAE and mortality in C4‐deficient guinea pigs were no different from those in normocomplementemic controls. Thus, although activation of the classic complement pathway does not appear to be involved in the production of EAE in guinea pigs, our results suggest a possible role of the alternative complement pathway in the pathogenesis of EAE.
Endogenous pain control mechanisms: Review and hypothesis
Authors: Basbaum Allan I. , Fields Howard L. 
Published: January 2005

AbstractThe anatomy, physiology, and pharmacology of an intrinsic neural network that monitors and modulates the activity of pain‐transmitting neurons is reviewed. This system can be activated by opiate administration or by electrical stimulation of discrete brainstem sites. Evidence is presented that its pain‐suppressing action is mediated in part by endogenous opiatelike compounds (endorphins).This pain suppression system is organized at three levels of the neuraxis: midbrain, medulla, and spinal cord. Activation of neurons in the midbrain periaqueductal gray matter (by electrical stimulation, opiates, and possibly psychological factors) excites neurons of the rostral medulla, some of which contain serotonin. The medullary neurons, in turn, project to and specifically inhibit the firing of trigeminal and spinal pain‐transmission neurons. As part of a negative feedback loop, the output of the pain transmission neurons, i.e., pain itself, is an important factor in activating the pain‐suppression system. A neural model which incorporates the experimental findings is proposed, and the clinical implications of the model are discussed.
The role of judges in life/death decisions for the neurologically impaired
Authors: Beresford H. Richard 
Published: January 2005

AbstractThe Massachusetts supreme court has recently ruled that decisions about withholding care from hopelessly ill, legally incapacitated patients must be made by judges. It clearly rejected the view that families and attending physicians should be empowered to make such decisions. In this respect, the ruling contrasts with that of the Quinlan case and highlights the issue of whether judges or physicians and families are better able to make medically and morally sound decisions respecting this class of patients.
Downbeat nystagmus due to anticonvulsant toxicity
Authors: Alpert Jack N. 
Published: January 2005

AbstractIsolated downbeat nystagmus was observed in 2 patients on multiple anticonvulsant regimens. The nystagmus disappeared when phenytoin dosage was reduced. Electrooculographic analysis revealed impaired downward tracking, supporting the concept of “pursuit” nystagmus.
Impaired refractory periods of peripheral sensory nerves in multiple sclerosis
Authors: Hopf Hanns C. , Eysholdt Magrit 
Published: January 2005

AbstractMaximum conduction velocity and relative refractory period (RRP) of median nerve sensory fibers were studied in 36 patients diagnosed as having multiple sclerosis (MS) and in 31 controls. Maximum conduction velocity did not differ in the two groups, but the RRP was significantly prolonged in MS patients. Increased RRP is observed mainly when peripheral nerve myelin is abnormal or damaged. Our findings support the assumption that peripheral nerve myelinated fibers are usually involved in MS.
Clinical and electroencephalographic correlates of the multiple independent spike foci pattern in children
Authors: Blume W. T. 
Published: January 2005

AbstractThe multiple independent spike foci (MISF) pattern on electroencephalograms occurred in 4.2% of 1,500 recordings obtained from patients between the ages of 6 months and 15 years. Among the 63 patients with MISF, 53 (84%) had a seizure disorder and 48 (76%) had generalized motor seizures. Half the study group had daily seizures and half had more than one type of attack. Sixty‐eight percent of MISF patients were intellectually subnormal. The intellectual status varied according to age at seizure onset, findings on clinical neurological examination, incidence of spikes in the recording, number of spike foci, and presence of excessive delta activity.
Actin filaments in human skin fibroblasts are similar in normal persons and patients with Huntington's disease
Authors: Geary Laurane E. , Lazarides Elias , Goetz Ingeburg , Roberts Eugene 
Published: January 2005

AbstractActin filaments in skin fibroblasts from patients with Huntington's disease (HD) were examined using immunofluorescent methods. Actin filaments were seen along the axis of cell elongation (stress or sheath filaments) as well as in areas of membrane ruffling (lattice filaments). In some cases, filaments appeared to radiate from foci within the cell. Bundles of these filaments radiated in various directions at different depths within a cell. These structures are similar, in cells from both normal individuals and HD patients, to actin filaments observed in other cell types. The higher‐than‐normal confluent densities achieved in culture by fibroblasts from patients with Huntington's chorea do not involve alterations in the ultrastructure of actin filaments.