bluuebunny/crossref_metadata_2025_split huggingface dataset:
Combined short‐term immunotherapy for experimental autoimmune myasthenia gravis
Authors: Pestronk Alan , Drachman Daniel B. , Teoh Robert , Adams Robert N. 
Published: January 2005

AbstractA therapeutic strategy was designed to eliminate the humoral immune response to acetylcholine receptor (AChR) in ongoing experimental autoimmune myasthenia gravis (EAMG). Rats with EAMG were treated with a protocol consisting of three components: (1) A single high dose of cyclophosphamide (200 mg/kg) was used to produce a rapid and sustained fall in the anti‐AChR antibody levels by preferential destruction of antibody‐producing B‐lymphocytes. “Memory” lymphocytes were not eliminated by cyclophosphamide. (2) Irradiation (600 rads) was used to eliminate the “memory” cells. It eliminated the anamnestic response to a challenge with the antigen AChR. (3) Bone marrow transplantation was used to repopulate the hematopoietic system after the otherwise lethal dose of cyclophosphamide. We used bone marrow from syngeneic rats with active EAMG to simulate an autologous transplant.Rats with EAMG treated with this combined protocol showed a prompt and sustained fall in the anti‐AChR antibody levels and had no anamnestic response to a challenge with AChR. Thus, an affected animal's own marrow could be stored and used later for repopulation after cyclophosphamide‐irradiation treatment.This treatment eliminates the animals' ongoing immune responses and reconstitutes the immune system in its original state. The success of this approach suggests that, if their safety could be established, similar “curative” strategies might be developed for the treatment of patients with severe antibody‐mediated autoimmune disorders, such as myasthenia gravis.
Tetrahydropterin therapy for hyperphenylalaninemia caused by defective synthesis of tetrahydrobiopterin
Authors: Kaufman Seymour , Kapatos Gregory , Rizzo William B. , Schulman Joseph D. , Tamarkin Lawrence , van Loon Glen R. 
Published: January 2005

AbstractA patient with hyperphenylalaninemia caused by a defect in the synthesis of tetrahydrobiopterin was treated with 6‐methyltetrahydropterin. This synthetic analog of the naturally occurring hydroxylation cofactor tetrahydrobiopterin, when given orally at a daily dose of 20 mg per kilogram of body weight increased depressed plasma and cerebrospinal fluid levels of norepinephrine. At a daily dose of 8 mg/kg, this pterin increased depressed cerebrospinal fluid levels of the biogenic amine metabolites dihydroxyphenylacetic acid, homovanillic acid, and 5‐hydroxyindoleacetic acid. At these doses of 6‐methyltetrahydropterin, there was an improvement of the patient's neurological symptoms, including a pronounced decrease in eye rolling and drooling and a marked increase in muscle strength, coordination, and physical activity.
Intelligence in epilepsy: A prospective study in children
Authors: Bourgeois Blaise F. D. , Prensky Arthur L. , Palkes Helen S. , Talent Barbara K. , Busch Suzanne G. 
Published: January 2005

AbstractA prospective study tested the stability of the IQ in children with seizure disorders. Seventy‐two children with epilepsy underwent psychological evaluations within two weeks of initial diagnosis and yearly thereafter for an average of 4 years. Forty‐five of the patients also had a nonepileptic sib evaluated in parallel. The mean IQ for all the children with epilepsy was 99.7 (±20.2, standard deviation) at the time of the initial test, not significantly different from the siblings. This score did not change appreciably with time. Eight of the 72 epileptic patients (11.1%), however, had a persistent decrease in IQ of 10 points or more. These patients had a higher incidence of drug levels in the toxic range (p <0.001), their epilepsy was more difficult to control (p <0.005), and their seizures began at an earlier age (p <0.05). Discriminant analysis revealed that the number of drugs to which the patient became toxic and the age at seizure onset were the two best predictors of ultimate IQ. These two predictors correctly classified 71% of all patients as to whether their IQ would drop by 10 or more points during the test period, remain within 10 points of the initial test score, or increase by more than 10 points. Total number of seizures and seizure control were less good predictors, according to this method of analysis. The findings suggest that, in younger children in particular, total seizure control should not be achieved at the price of repeated episodes of drug toxicity.
Senile dementia of the Alzheimer type
Authors: Terry Robert D. , Katzman Robert Katzman 
Published: January 2005

AbstractThe prevalence of severe dementia in the United States is about 1.3 million cases, of which at least 50 to 60% are of the Alzheimer type. Severe dementia of the Alzheimer type is found rarely in a clearly dominant pattern, although often one or more relatives are affected. Down's syndrome in adults is often associated with Alzheimer changes. The diagnosis is a clinicopathological one; there is a considerable error rate in the clinical diagnosis early in the course of the disease, especially in regard to dementia in depression. The differential diagnosis involves a great many disorders, including multi‐infarct dementia, tumors, subdural hematomas, and others. Physiological aspects of Alzheimer's disease include a diffusely slow electroencephalogram, reduced cerebral blood flow, and particular patterns noted on positron emission tomographic scanning. The latter technique has also demonstrated that oxygen extraction is normal in Alzheimer's disease, thus excluding ischemia from possible pathogenetic factors. Morphological changes, that is, the presence of plaques and tangles, are widely distributed in neocortex, paleocortex, and many deep gray areas down through the pontine tegmentum, but largely exclude the basal ganglia, thalamus, and substantia nigra. Numerous plaques without neocortical tangles are found in many demented persons older than 75 years. A severe loss of large neocortical neurons is characteristic of the disease. The chemical nature of the paired helical filaments that make up the neurofibrillary tangle has not yet been ascertained. Neurons are markedly deficient in the basal forebrain nuclei, and this deficiency may account for the severe diminution of choline acetyltransferase and acetylcholine in the neocortex and palecortex. Muscarinic cholinergic receptors are present in normal amounts. Norepinephrine is reduced in some cases, and somatostatin in most. Substance P is low in severe cases. The etiology of the disorder is unknown and the role of aluminum is disputed. Management of patients with Alzheimer's disease is difficult, and neuroleptics are to be used with great caution because of their side effects. Substrate therapy has not been effective; physostigmine improves memory but is not suitable for general use. Trophic factors, gangliosides, and aluminum chelation are being investigated for use in pharmacological intervention.
Thymectomy‐induced decrease in Tγ cells and OKT8+ cells in multiple sclerosis
Authors: Trotter John L. , Gebel Howard M. , Ferguson Thomas B. , Garvey William F. , Rodey Glen E. 
Published: January 2005

AbstractStudies were performed on lymphocytes from patients with multiple sclerosis before and after thymectomy (group I) and before and after thymectomy and azathioprine therapy (group II), and from matched control patients with multiple sclerosis, control patients with other neurological diseases, and healthy control subjects. Following treatment the percentage of Tγ cells in both group I and group II patients decreased from above the mean normal control levels to below this level; OKT8 + cell numbers in group I became lower than in any of the control groups; the percentage of concanavalin A‐‐induced suppressor activity, which was initially normal, fell in group II and suggestively but not significantly in group I; and total blood lymphocytes in group I decreased from normal to below control levels. Other tests showed no significant changes with therapy. No significant difference in changes in clinical status were observed after one year in the treated patients compared with matched controls.
Spinal somatosensory evoked potentials in juvenile diabetes
Authors: Cracco Joan , Castells Saladore , Mark Erna 
Published: January 2005

AbstractSpinal somatosensory Evoked potentials to stimulation of the peroneal nerves in the popliteal fossa were recorded from 46 insulin‐dependent neurologically normal patients with juvenile diabetes. Conduction velocities of these potentials were determined over proximal peroneal nerve, cauda equina, and spinal cord and were compared with those obtained from 46 age‐matched control subjects.Mean values for overall spinal conduction velocity (L3–C7 spines) and conduction velocity over rostral spinal cord (T6–C7 spines) and peroneal nerve–cauda equina (stimulus to L3 spine) were lower in the diabetic group (p < 0.001). Peripheral nerve conduction velocity alone was slow in 5 patients, and spinal conduction velocity was slow in 8; in 2 patients both peripheal and spinal velocities were slow. This study suggests that in addition to impairment of peripheral nerve function, patients with juvenile diabetes without clinical evidence of neurological involvement can have a defect in spinal afferent transmission.
Carrier detection of sialidosis with partial β‐galactosidase deficiency by the assay of lysosomal sialidase in lymphocytes
Authors: Tsuji Shoji , Yamada Takamichi , Ariga Toshio , Toyoshima Itaru , Yamaguchi Haruyasu , Kitahara Yoshisuke , Miyatake Tadashi , Yamakawa Tamio 
Published: January 2005

AbstractLysosomal and plasma membrane sialidase activities in lymphocytes were studied in four patients with sialidosis with partial β‐galactosidase deficiency, four obligate heterozygotes, and three siblings of a patient. Lysosomal sialidase activity in homozygotes was absent, and that in heterozygotes was significantly decreased to 70% of control level. The results indicate that carriers can be detected by the assay of lysosomal sialidase activity of lymphocytes.
Mirror movement: Application of movement‐related cortical potentials
Authors: Shibasaki Hiroshi , Nagae Kazuhisa 
Published: January 2005

AbstractIn a patient withy Kallmann's syndrome (hypogonadotropic hypogonadism and anosmia) manifesting mirror movement, cortical potential associated with unilateral and bilateral simulataneous voluntary middle finger extensions were studied. Premovement negative slope, which has been shown to reflect preparatory excitation of motor cortex corresponding to the voluntary movement, was recorded bilaterally in this patient in spite of intended unilateral hand movement. It is suggested that mirror movement in this particular patient is generated by unintended excitation of the opposite motor contex.
Neurofilament subunit–related proteins in neuronal intranuclear inclusions
Authors: Palo Jorma , Haltia Matti , Carpenter Stirling , Karpati George , Mushynski Walter 
Published: January 2005

AbstractWell‐preserved nuclei were isolated from the brain of a patient with neuronal intranuclear inclusion disease (NIID). The inclusions in situ stained for proteins and, by immunohistochemical techniques, for the 200K and 68K components of the neurofilament protein triplet but were negative for the 145K neurofilament protein. At the electron microscopic level the inclusions were seen to consist of straight, randomly arranged tubular filaments 8.5 to 9.5 nm in diameter. They were resistant to extraction with hot buffer containing sodium dodecyl sulfate (SDS) and 2‐mercaptoethanol, indicating that the proteins of NIID inclusions are probably cross‐linked by covalent bonds other than disulfide. This feature is also exhibited by the paired helical filaments occurring in brain in Alzheimer's disease. No major differences were found in the polypeptide composition of nuclei in NIID and control nuclei resolved by SDS‐polyacrylamide gel electrophoresis. The inclusions isolated from SDS‐extracted nuclei were shown by immunohistochemical techniques to stain for all three neurofilament subunits, indicating that 145K‐related antigenic sites were uncovered during the extraction procedure.
Autosomal dominant inheritance of hereditary canine spinal muscular atrophy
Authors: Sack George H. , Cork Linda C. , Morris Jeanette M. , Griffin John W. , Price Donald L. 
Published: January 2005

AbstractHereditary canine spinal muscular atrophy (HCSMA) is a motor neuron disease in Brittany spiniels. Three phenotypes are recognized (accelerated, intermediate, and chronic) and are distinguished on the basis of rate of progression and age at onset. Breeding studies within a kindered of more than 125 dogs (Brittany spaniel and beagle‐Brittany outcrosses) have established an autosomal dominant inheritance for HCSMA. Pups homozygous for the trait have accelerated disease, whereas heterozygous dogs have intermediate or chronic disease. The reason for the two phenotypes in heterozygotes is under study. HCSMA provides a unique opportunity to study the genetic and pathophysiological mechanisms of a motor neuron disease, and findings may have broad relevance to investigations of autosomal dominant degenerative disorders of the central nervous system.
Nonhemorrhagic venous infarction of the spinal cord
Authors: Kim R. C. , Smith H. R. , Henbest M. L. , Choi B. H. 
Published: January 2005

AbstractA 71‐year‐old man experienced gradually progressive leg weakness, urinary retention, and mild loss of sensation in dermatomes T8 through T12 bilaterally. After 5 to 6 weeks of illness, he developed flaccid paraplegia and sensory loss below T8. He died 16 weeks after onset of neurological symptoms. Neuropathologically, there was widespread, subtotal necrosis of the spinal cord, largely of nonhemorrhagic character, from T8 downward. Dorsal and anterior median spinal veins were occluded by a partially organized thrombus. Comparison of this case with 19 previously recorded examples of venous infarction of the spinal cord (8 hemorrhagic, 7 nonhemorrhagic, and 4 embolic) suggests major differences in clinical presentation, rate of progression, and length of survival among the three groups.
Exacerbation of myasthenia gravis after removal of a thymoma having a membrane phenotype of suppressor t cells
Authors: Kuroda Yasuo , Oda Ken‐Ichiro , Neshige Ryuji , Shibasaki Hiroshi 
Published: January 2005

AbstractWe describe a case of myasthenia gravis associated with a thymoma having a membrane phenotype of suppressor T cells, as defined by monoclonal antibodies. Thymectomy resulted in exacerbation of the disease, a drastic increase in serum acetylcholine receptor‐antibody titer, and a decrease of the circulating suppressor T cell population. The findings indicate that the etiopathogenic relationship between the thymus and myasthenia gravis is variable and complex.
Lymphomatoid granulomatosis with isolated involvement of the brain
Authors: Schmidt Brain J. , Meagher‐Villemure Kathleen , Carpio Jaime Del 
Published: January 2005

AbstractA patient with biopsy‐proven lymhomatoid granulomatosis of the brain as the sole manifestation of the disease is described. During 14 months of follow‐up since surgical excision of the cerebral mass, no evidence of recurrent brain involvement or of extracranial lesions has been found. This case is unusual in the confinement of the disease to the central nervous system and in the favorable outcome following surgical treatment alone.
Clinical and genetic variations in the syndrome of adult GM2 gangliodosis resulting from hexosaminidase a deficiency
Authors: Argov Z. , Navon R. 
Published: January 2005

AbstractSix patients from three families developed adult GM2 gangliosidosis resulting from severe β‐hexosaminidase A deficiency. The clinical picture varied between and within families and included spinocerebellar, various motor neuron, and cerebellar connection syndromes. Psychosis appeared in each family. Involvement of three generations was recorded in one family. The phenotype of adult GM2 gangliosidosis is variable and cannot form a basis for genetic classification. Detailed hexosaminidase determinations in eight patients from five families revealed all patients to have minor quantities of hexosaminidase A (about 15% of normal), with marked increase in hexosaminidase I isozyme.
Gas within intracranial abscess cavities: An indication for surgical excision
Authors: Young Ronald F. , Frazee John 
Published: January 2005

AbstractFive patients were treated in whom gas within an intracranial abscess cavity was identified by plain roentgenogram, computed tomographic scan, or both. Management by aspiration in three patients was unsuccessful. Total excision of the abscess cavity was eventually required in all five patients, and a persistent extracranial communication was identified and closed in each. One patient died secondary to transtentorial herniation and severe brainstem injury; the other four recovered fully. Although certain anaerobic organisms may produce gas in the absence of a communication to the outside of the body, such production is uncommon. Total surgical excision is recommended for gas‐containing abscesses because it allows removal of the mass lesion and identification and closure of possible persistent extracorporal communication.
Nystagmus in motor neuron disease: Clinicopathological study of two cases
Authors: Kushner Michael J. , Parrish Margaret , Burke Alan , Behrens Myles , Hays Arthur P. , Frame Boy , Rowland Lewis P. 
Published: January 2005

AbstractTwo patients with amyotrophic lateral sclerosis proved postmortem had nystagmus in addition to typical clinical signs of motor neuron disease. The first patient had gaze‐evoked rotatory nystagmus that was followed by horizontal nystagmus in the primary position with supranuclear paresis of horizontal gaze and upgaze. The second patient had rotatory nystagmus that was evoked by lateral gaze, with normal range of eye movements. Nystagmus is so rare in motor neuron disease that these observations may imply another disease, but postmortem examination did not provide any other explanation. These two cases add to the increasing evidence that motor neuron disease comprises a heterogeneous group of disorders.
Human leukocyte interferon administration to patients with symptomatic and suspected rabies
Authors: Merigan Thomas C. , Baer George M. , Winkler William G. , Bernard Kenneth W. , Gibert Claude G. , Chany Charles , Veronesi Ricardo ,   The Collaborative Group
Published: January 2005

AbstractBody fluids and brain tissue from rabid human patients have demonstrated only low titers of interferon. Therefore, pharmacokinetic studies of systemically administered and locally injected leukocyte interferon were performed in 2 North American patients with suspected rabies who showed no clinically important side effects of this therapy. Similar therapy was given to 5 patients with symptomatic rabies in Europe and America. Although no prolongation of the clinical course was seen in 3 patients given high‐dose intraventricular and systemic therapy, treatment was not intiated until between 8 and 14 days after symptoms were seen. The intraventricular dosage regimen produced cerebrospinal fluid levels that appeared to fall progressively over the 24 hours after injection and demonstrated good but somewhat delayed distribution into the lumbar sac. Titers produced by this therapy were 30‐ to 10,000‐fold higher than those normally observed in this infection, however. In the patients treated at the highest dosage, a diminished and delayed antirabies neutralizing antibody titer was observed, probably a result of the administration of the exogenous interferon.
Nonconvulsive status epilepticus following metrizamide myelography
Authors: Pritchard Paul B. , O'Neal David B. 
Published: January 2005

AbstractEpileptic seizures are a known complication of metrizamide myelography. To our knowledge, this is the first report of a case of nonconvulsive status epilepticus of the absence type following metrizamide myelography. There was symptomatic and electroencephalographic improvement after intravenous administratration of antiepileptic drugs, and there was no neurological residual. Nonconvulsive status epilepticus should be considered when impairment of consciousness supervenes after radiographic procedures using metrizamide.
Progressive cerebral ischemia antedates cerebrovascular symptoms by two years
Authors: Meyer John Stirling , Rogers Robert L. , Mortel Karl F. 
Published: January 2005

AbstractProspective longitudinal cerebral blood flow values were serially plotted over a four‐year interval against the course of cerebral ischemia before, during, and after onset of clinical symptoms. Of 161 normal subjects (mean age, 62 years), 86 were risk free and 75 had hypertension, heart disease, diabetes mellitus, and/or hyperlipidemia. Twenty‐one subjects developed cerebrovascular symptoms during the prospective trial. Mean hemispheric cerebral blood flow values were significantly lower for at risk than for risk‐free subjects. Symptomatic subjects showed lower values than those in either of the two asymptomatic groups at every session. Statistical analysis of cerebral blood flow values for symptomatic patients compared one and two years prior to onset of symptoms, at the onset of symptoms, and 1 year later showed reductions compared to asymptomatic risk‐factored subjects tested in a similar prospective manner. Measurable declines in cerebral perfusion accompany development and progression of aortocerebral atherosclerosis prior to clinical appearance of signs and symptoms of cerebrovascular disease. If confirmed, these observations should permit the institution of preventive medical and/or surgical interventive measures and an evaluation of their outcome.
Dystonia in 61‐year‐old identical twins: Observations over 45 years
Authors: Eldridge Roswell , Ince Susan E. , Chernow Bart , Milstien Sheldon , Lake C. Raymond 
Published: January 2005

AbstractWe examined 61‐year‐old identical twin women of Jewish extraction with a probable autosomal recessive form of torsion dystonia. The dystonia in each was relatively mild and discovered only because a young relative developed dystonia. The twins were said to be discordant for dystonia, but personal evaluation led to the diagnosis of dystonia in both. Their slow course, with prolonged spontaneous remission in one twin, is in contrast to that described in most published reports. Although similar in mode of onset and initial course, the twins were dissimilar in age at onset, influence of pregnancy, diurnal variation in symptoms, need for medication, later course, and degree of disability at age 61. Normal plasma levels of norepinephrine and dopamine‐beta‐hydroxylase are consistent with autosomal recessive hereditary torsion dystonia.The importance of personal evaluation of key family members in establishing the correct genetic basis for a heterogeneous group of disorders, such as the hereditary dystonias, is stressed.
Computed tomography in the diagnosis of Wernicke's encephalopathy: A radiological‐neuropathological correlation
Authors: Mensing J. W. A. , Hoogland P. H. , Slooff J. L. 
Published: January 2005

AbstractA case of Wernicke's encephalopathy with pathological verification is reported. Results of computed tomography scanning performed one day after admission of the patient, a male alcoholic, strongly suggested Wernicke's encephalopathy and were in agreement with the subsequent postmortem findings.