bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurological involvement in hemolytic‐uremic syndrome
Authors: Sheth Kumudchandra J. , Swick Herbert M. , Haworth Nancy 
Published: January 2005

AbstractOf 44 children with hemolytic‐uremic syndrome seen at Milwaukee Children's Hospital, 15 (34%) had neurological involvement. This group contained 8 boys and 7 girls, with a mean age of 3 1/4 years. Twelve patients had seizures within 48 hours of admission. Seizures were associated with hypertension, fever, hyponatremia, or hypocalcemia. Other neurological symptoms included altered consciousness, behavioral changes, diplopia, and dizziness. Hemiparesis (4 patients), eye involvement (7 patients), decerebrate posturing (2 patients), and ataxia (1 patient) were present on physical examination. Cerebrospinal fluid examination showed increased protein in 4 of 11 patients. Electroencephalograms were abnormal in all 9 patients tested. Computed tomographic and radionuclide scans showed evidence of vascular abnormalities in 4 of the 14 patients studied. Complete neurological recovery occurred in only 6 of the 15 children, while the remaining 6 demonstrated residual hemiparesis, seizures, and cortical visual defect. In those children with neurological involvement, there was a higher incidence of residual hypertension (49% versus 11%), chronic renal damage (40% versus 3.5%), and death (28% versus 0%), suggesting that central nervous system involvement indicates severe hemolytic‐uremic syndrome.
Periodic leg movements of sleep (nocturnal myoclonus): An electrophysiological study
Authors: Wechsler Lawrence R. , Stakes John W. , Shahani Bhaghwan T. , Busis Neil A. 
Published: January 2005

AbstractSix patients with periodic leg movements of sleep (PLMS) were studied electrophysiologically. All patients had normal results on routine nerve conduction studies and electromyography. In 5 patients the blink reflex consisted of three components, and in 1 patient it consisted of four components, with two components being normal. The second component of their blink reflex did not habituate. Other abnormalities included long‐latency responses in the extremities in 2 patients, release of H‐reflexes from flexor hallucis brevis muscle in 2 patients, and an Hmax/Mmax ratio of 98% in 2 patients. One patient's median nerve somatosensory‐evoked response had a markedly enlarged P22 wave. These electrophysiological abnormalities suggest a disorder of the central nervous system producing increased excitability of segmental reflexes. To produce increased excitability of both brainstem and spinal cord reflexes, the disorder must be operative at the pontine level or rostral to it. Since long‐latency components of blink reflexes occurred in all our patients, this may be a helpful diagnostic test when PLMS is suspected.
Signs of focal posterior cerebral abnormality in early subacute sclerosing panencephalitis
Authors: Begeer J. H. , Haaxma R. , Snoek J. W. , Boonstra S. , le Coultre R. 
Published: January 2005

AbstractIn 16 cases of subacute sclerosing panencephalitis (SSPE), praxis and visual spatial capacities were more impaired early in the disease than were language functions. Together with the electroencephalographic findings, this suggests that the early lesions in SSPE are more pronounced in the parietooccipital area than in the classic language areas. Other reports also support such a localization. Detection of the disease in its early phase when dressing apraxia and visual impairment predominate is important in conducting clinical trials of different therapeutic agents.
Cerebellar infarction: Comparison of computed tomography and magnetic resonance imaging
Authors: Simmons Zachary , Biller José , Adams Harold P. , Dunn Val , Jacoby Charles G. 
Published: January 2005

AbstractWe correlated clinical, computed tomographic (CT), and magnetic resonance imaging (MRI) findings in 14 patients with cerebellar infarctions. Before MRI, the diagnosis of cerebellar infarction was made in only 7 patients on the basis of clinical and CT evidence. Cerebellar infarction was bilateral in 3 patients and was associated with brainstem infarction in 6. Infarction occurred in the territory of the posterior inferior cerebellar artery (PICA) in 12 patients. The territory of the superior cerebellar artery (SCA) was involved in 1 patient, and 1 infarction encompassed the watershed between the PICA and the SCA. In patients with infarction of the PICA territory, the medial and intermediate hemispheric segments were most frequently involved. Involvement of the lateral hemispheric segment was infrequent and was independent of brainstem involvement. Because of its fine demonstration of anatomical detail, its lack of bony artifact, and its ability to visualize infarctions readily within the first 24 hours, MRI is an excellent method for demonstrating cerebellar infarction.
Intrathecal interferon in subacute sclerosing panencephalitis
Authors: Huttenlocher Peter R. , Picchietti Daniel L. , Roos Raymond P. , Cashman Neil R. , Horowitz Bernard , Horowitz Marilyn S. 
Published: January 2005

AbstractFive patients with clinically advanced subacute sclerosing panencephalitis (SSPE) were given human leukocyte interferon (IFN) by the lumbar route, 1 million IU every other day for a total of 30 days. Intrathecal IFN produced a meningeal inflammatory reaction in all patients and was associated with transient hemiparesis in 1. It persisted in the cerebrospinal fluid at measurable levels for 48 hours after a single injection. Although improvement was temporally related to intrathecal IFN in 1 patient, it is not clear whether this was induced by IFN or a spontaneous remission. A randomized controlled trial would be necessary to evaluate IFN critically as a therapy for SSPE.
Effect of dexamethasone on blood‐brain barrier in the normal mouse
Authors: Hedley‐Whyte E. Tessa , Hsu Dora W. 
Published: January 2005

AbstractDexamethasone ameliorates cerebral edema, but its effect on normal blood‐brain barrier is unknown, as is the site of action. Sixteen normal mice were given dexamethasone, 3 mg/kg intramuscularly or 2 mg/kg intravenously. One to two hours later 10 mg of horseradish peroxidase (HRP) in 0.1 ml of saline was administered intravenously and allowed to circulate for 15 minutes. Brain slices were examined by light and electron microscopy. The number of HRP‐permeable arteriolar segments per brain was less in dexamethasone‐treated than in control mice (p < 0.001). In addition, the number of small HRP‐filled endothelial vesicles of capillaries and of HRP‐permeable and ‐impermeable arterial segments was less in dexamethasone‐treated mice than that for similar vessels in control mice (p < 0.005, p < 0.05, and p < 0.01, respectively). The total number of small vesicles and the number of larger HRP‐filled vesicles in arterioles was the same in treated and control mice. Dexamethasone therefore decreased the normal permeability of cerebral blood vessels to HRP. This decrease was accompanied by a decrease in the number of HRP‐containing small endothelial vesicles. These data suggest that dexamethasone influences cerebral edema by decreasing the permeability of the cerebral vasculature for macromolecules.
Fiber loss is primary and multifocal in sural nerves in diabetic polyneuropathy
Authors: Dyck Peter James , Lais Alfred , Karnes Jeannine L. , O'Brien Peter , Rizza Robert 
Published: January 2005

AbstractPathological, morphometric, and teased fiber sutides of sural nerve from 36 diabetic patients with (n = 32) and without (n = 4) neuropathy and from 47 healthy subjects provide evidence that in diabetic polyneuropathy: (1) fiber loss is primary; (2) demyelination and remyelination with or without onion bulb formation are secondary; (3) remaining fibers, on average, have the same ratio of small to large fibers as in healthy individuals, but with a greatly increased variability; and (4) the spatial distribution of fiber loss is both diffuse and multifocal. Criteria developed during the study of experimental modles of ischemic neuropathy were exployed to assess whether ischemic nerve damage had occured in diabetic polyneuropathy. We conclude that there is increasing evidence that microvascular pathological abnormality and ischemia may be involved in the pathogenesis of human diabetic polyneuropathy. Cases with selective loss of small or large afferent fibers are probably extremes of a normal distribution and not different disorders.
Axonal transport in the motor neurons of rats with neuropathy induced by p‐bromophenylacetylurea
Authors: Nagata Hiroshi , Brimijoin Stephen 
Published: January 2005

AbstractAxonal transport was studied in sciatic motor neurons of rats with neuropathy induced by p‐bromophenylacetylurea (BPAU) in dimethylsulfoxide solution. Control rats were treated with the vehicle alone. To label rapidly transported proteins, the rats received an injection of 35S‐methionine into the ventral horn of the spinal cord at the L1 vertebral level. Radiolabeled protein was collected at ligatures applied on the sciatic nerve at intervals thereafter. In animals with severe motor weakness owing to treatment with BPAU, 400 mg/kg, there was evidence of increased delivery of labeled protein into the axon during the early period after isotope injection, but reduced delivery later. A dose‐dependent decrease in the amount of labeled protein recirculated by retrograde axonal transport was also noted. A significant reduction in the amoutn of protein transported retrogradely was also detected during the latent subclinical phase of the neuropathy. The velocity of rapid anterograde transport, examined in unligated sciatic nerves, was unaffected by BPAU treatment. However, the lag time between precursor injection and the onset of transport was shorter in BPAU‐treated rats than in controls. This effect was not explainable on the basis of fluctuations in core body temperature. The results are consistent with the view that disturbances of rapid anterograde and retrograde transport play a role in the peripheral neurotoxicity of BPAU. Attention is directed to the possibility that the transport disturbances and the subsequent neuropathy are related to alterations in the processing of rapidly transported membrane‐limited organelles in the nerve cell bodies.
The human anti–myelin‐associated glycoprotein IgM system
Authors: Murray Norman , Page Nicole , Steck Andreas J. 
Published: January 2005

AbstractTwo mouse monoclonal antiidiotypic antibodies that react with human monoclonal IgM antibodies with specificity for myelin‐associated glycoprotein (MAG) have been used to study the immunological specificity of the reported cross‐reactions involving the anti‐MAG IgM. Both of the antiidiotypic antibodies are shown to react with the combining site of their respective idiotypic IgM and to inhibit the reaction between IgM and MAG. Using these antiidiotypic antibodies as “surrogate” antigen, we have demonstrated immune cross‐reactivity between MAG, a human peripheral nerve glycolipid, and a low‐molecular‐weight protein of human peripheral nerve myelin. In addition, we have used the two antiidiotypic antibodies to conduct a search for evidence of shared idiotypy among 34 different neuropathy‐associated paraproteins. Our results provide no evidence for a neuropathy‐associated idiotype, suggesting a degree of polymorphism in the human anti‐MAG IgM system.
Antimigraine drug interactions with 5‐hydroxytryptamine1A receptors
Authors: Hiner Bradley C. , Roth Heidi L. , Peroutka Stephen J. 
Published: January 2005

AbstractThe interactions of four antimigraine drugs with 5‐hydroxytryptamine1A and beta‐adrenergic receptors were analyzed in rat brain membranes. Methysergide, cyprohepatadine, (−)propranolol, and pizotifen display similar affinities (IC50 values, 83 to 280 nM) for 5‐hydroxytryptamine1A receptors labeled by [3H]8‐hydroxy‐N,N‐dipropyl‐2‐aminotetralin. Cyproheptadine, pizotifen, and methysergide are essentially inactive at beta‐adrenergic receptors labeled by [3H]dihydroal‐prenolol (IC50 values, 8,700 to 87,000 nM). By contrast, (−)propranolol is an extremely potent beta‐adrenergic agent (IC50, 3 nM). These data indicate that the 5‐hydroxytryptamine1A receptor is a common site of action for (−)propranolol and other antimigraine agents.
The AIDS dementia complex: I. Clinical features
Authors: Navia Bradford A. , Jordan Barry D. , Price Richard W. 
Published: January 2005

AbstractOf 70 autopsied patients with the acquired immune deficiency syndrome (AIDS), 46 suffered progressive dementia that was frequently accompanied by motor and behavioral dysfunction. Impaired memory and concentration with psychomotor slowing represented the most common early presentation of this disorder, but in nearly one half of the patients either motor or behavioral changes predominated. Early motor deficits commonly included ataxia, leg weakness, tremor, and loss of fine‐motor coordination, while behavioral disturbances were manifested most commonly as apathy or withdrawal, but occasionally as a frank organic psychosis. The course of the disease was steadily progressive in most patients, and at times was punctuated by an abrupt acceleration. However, in 20% of patients a more protracted indolent course was observed. In the most advanced stage of this disease, patients exhibited a stereotyped picture of severe dementia, mutism, incontinence, paraplegia, and in some cases, myoclonus. The high incidence and unique clinical presentation of this AIDS dementia complex is consistent with the emerging concept that this complication is due to direct brain infection by the retrovirus that causes AIDS.
Chronic idiopathic ataxic neuropathy
Authors: Dalakas Marinos C. 
Published: January 2005

AbstractFifteen patients with chronic sensory ataxia caused by a large‐fiber sensory neuropathy were studied and followed up for a period of 17.4 years (range, 4 to 41). When first seen, they had distal paresthesias and sensory ataxia of slow onset and progression, areflexia, normal strength, and a profound loss of proprioceptive and kinesthetic sensation extending up to the most proximal joints. Needle electromyogram and motor‐nerve conduction velocity findings were normal in most of the patients and sensory potentials were absent in all. Nerve biopsy showed severe loss of the large myelinated fibers. Nine patients had a serum monoclonal or polyclonal gammopathy (3 with IgMκ, 1 with IgAκ, and 5 with a polyclonal increase of IgG, IgA, or IgM), and 8 had elevated cerebrospinal fluid γgM globulin levels in spite of low normal total cerebrospinal fluid protein levels. No circulating antibodies to ganglionic neurons were found. Therapy with immunosuppressants or plasmapheresis was unsuccessful. All patients are disabled and their conditions have continued to worsen without signs of malignancy or systemic illness during a mean follow‐up period of 17.4 years.Chronic idiopathic ataxic neuropathy is a proprioceptive neuropathy, clinically indistinguishable from the one associated with carcinoma or pyridoxine abuse due to involvement of the dorsal root ganglia, and could represent a distinct form of an indolent, slowly progressive sensory neuronopathy (ganglionopathy). Although immunopathological mechanisms may play a role, especially in patients with an associated paraproteinemia, the resistance of such patients to therapy, the progressive course, and the resemblance of this disorder to other toxic neuronopathies associated with pyridoxine abuse or doxorubicin administration suggest a possible toxic etiopathogenesis.
N‐acetyl‐β‐hexosaminidase β locus defect and juvenile motor neuron disease: A case study
Authors: Cashman Neil R. , Antel Jack P. , Hancock Larry W. , Dawson Glyn , Horwitz Allen L. , Johnson William G. , Huttenlocher Peter R. , Wollmann Robert L. 
Published: January 2005

AbstractA patient with partial deficiency of N‐acetyl‐ß‐hexosaminidase (Hex) developed a progressive motor neuron syndrome beginning at age 7, characterized by dysarthria, muscle wasting, fasciculations, and pyramidal tract dysfunction. Minor clinical features have included tremor and late distal sensory abnormalities. Rectal biopsy at age 24 demonstrated membranous cytoplasmic bodies in submucosal ganglion cells. Biochemical evaluation revealed nearly absent Hex B activity in serum, leukocytes, and fibroblasts, with partial Hex A activity in serum and leukocytes, and low normal Hex A activity in fibroblasts. Motor neuron disease can be a presentation of a Hex ß locus defect, in addition to the previously recognized Hex α locus defects.
Immunohistochemical analysis of the cellular infiltrate in multiple sclerosis lesions
Authors: Hauser Stephen L. , Bhan Atul K. , Gilles Floyd , Kemp Maurice , Kerr Claire , Weiner Howard L. 
Published: January 2005

AbstractImmunohistochemical staining of 16 brains post mortem from patients with progressive multiple sclerosis and of two biopsy specimens from patients with acute demyelinating disease was performed using a panel of monoclonal antibodies reactive with T cells and T‐cell subsets, B cells, and Ia (HLA‐DR) antigens. Lymphocytic perivascular cuffs were most prominent at the edge of active plaques and were occasionally seen in areas with no evidence of demyelination or macrophage infiltration. Perivascular cuffs consisted predominantly of T cells and Ia+ cells, with many T8+ cells and variable numbers of T4+ cells. T8/T4 ratios in cuffs varied between 1:1 and 50:1. In normal‐appearing white matter, cuffs were sparse and were predominantly T8+. The distribution of T cells in the parenchyma resembled that seen in perivascular cuffs, namely, predominantly T8+ cells and variable numbers of T4+ cells. Many Ia+ cells were present in active lesions, and the majority of these cells appeared, by histological criteria, to be macrophages. Tissue macrophages were also stained lightly by the anti‐T4 antibody. No brain had more T4+ than T8+ cells, determined using both T4 and Leu3a monoclonal antibodies. B1+ cells were rare. These results suggest that the cellular infiltrate in multiple sclerosis consists predominantly of T cells and macrophages and that there is an overrepresentation of T8+ cells compared with T4+ cells.
Increased leukotriene C4 and vasogenic edema surrounding brain tumors in humans
Authors: Black Keith L. , Hoff Julian T. , McGillicuddy John E. , Gebarski Stephen S. 
Published: January 2005

AbstractLeukotrines are pharmacologically active compounds that promote vascular permeability. In this study we sought to determine whether tissue leukotriene–like immunoreactivity was increased in intracranial tumors associated with peritumoral edema. In 20 patients undergoing craniotomy tissue specimens were immediately frozen after removal and tissue leukotriene C4 levels were determined by radioimmunoassay. An index of peritumoral edema was estimated from preoperative contrast‐enhanced computed tomographic scans. There was a significant correlation between brain edema and tissue leukotriene levels (p < 0.003). Metastatic tumors (n = 8) had the highest leukotriene C4 level at 13.8 ± 8.5 pg/mg tissue (mean ± SE) and the highest index of edema 5.7 ± 1.8. The mean leukotriene C4 level in the gliomas (n + 5) ws 6.2 ± 2.3 pg/mg tissue and the edema index was 2.1 ± 0.6. There was no edema and no neoplasma in he temporal lobes removed for seizure (n + 2), and their level of leukotriene C4 was 0.4 ± 0.1 pg/mg tissue. The formation of leukotriene C4 is stimulated by intracranial tumors. Leukotrienes increase blood–brain barrier permeability and may be important in the formation of vasogenic edema surrounding tumors.
Alzheimer'S disease: Monoamines and spiperone binding reduced in nucleus basalis
Authors: Sparks D. Larry , Markesbery William R. , Slevin John T. 
Published: January 2005

AbstractLevels of dopamine and serotonin, and of their precursors and metabolites, were measured in the nucleus basalis of Meynert (nbM) of patients with Alzheimer's disease and control patients. [3H]Spiperone binding to membrane homogenates of the nbM, indicative of both dopaminergic and serotonergic receptors, was also measured. The nbM from patients with Alzheimer's disease showed significant decreases in both spiperone binding and in the levels of dopamine and serotonin but no decrease in either tyrosine or tryptophan levels. These data indicate a loss of both dopaminergic and serotonergic synapses in the nbM of patients with Alzheimer's disease.
Elevated γ‐aminobutyric acid level in striatal but not extrastriatal brain regions in Parkinson's disease: Correlation with striatal dopamine loss
Authors: Kish Stephen J. , Rajput Ali , Gilbert Joseph , Rozdilsky Bohdan , Chang Li‐Jan , Shannak Kathleen , Hornykiewicz Oleh 
Published: January 2005

AbstractWe measured the concentration of γ‐aminobutyric acid (GABA), glutamic acid, and o‐phosphoethanolamine in autopsied brain of 9 patients who died with idiopathic Parkinson's disease and 10 control subjects. In the control striatum GABA showed an uneven rostrocaudal distribution pattern with rostral subdivisions containing about 40 to 50% higher levels. When compared with controls, GABA concentrations in Parkinson's disease striatum were generally elevated. The GABA elevation was most pronounced in the caudal subdivision of the putamen; this striatal subdivision also showed the most severe dopamine loss. We observed in the caudal putamen a significant negative correlation between the (elevated) GABA and (reduced) dopamine levels (the latter expressed as the sum of dopamine plus 3‐methoxytyramine). Milder nonsignificant elevations of GABA levels were observed in intermediate and rostral putamen followed by the caudate head subdivisions. GABA levels were normal in all extrastriatal brain areas examined. Striatal glutamic acid levels were markedly elevated in 3 of the 9 patients with Parkinson's disease. We suggest that the altered GABA metabolism in the striatum, especially the putamen, is consequent to the nigrostriatal deficiency in this disorder. This secondary change in striatal GABA function is likely to contribute to the basal ganglia dysfunction produced by the striatal dopamine loss and thus may be related to certain aspects of parkinsonian symptomatology.
Myopathy caused by a deficiency of Ca2+‐adenosine triphosphatase in sarcoplasmic reticulum (Brody's disease)
Authors: Karpati George , Charuk Jeffrey , Carpenter Stirling , Jablecki Charles , Holland Paul 
Published: January 2005

AbstractFour male patients from two families were first seen with impaired skeletal muscle relaxation that rapidly worsened during exercise. Muscle biopsies from 2 patients were examined by appropriate biochemical and microscopic immunocytochemical techniques. The adenosine triphosphate (ATP)–dependent Ca2+ transport rate was extremely low in a particulate membrane fraction of skeletal muscle, and there was also a marked reduction of the concentration of 100‐kD phosphoprotein, corresponding to Ca2+‐ATPase of sarcoplasmic reticulum, in muscle microsomes. The concentration of immunoreactive Ca2+‐ATPase of sarcoplasmic reticulum was markedly reduced on immunoblots. Evaluation by microscopic immunocytochemical techniques, using one polyclonal and two monoclonal antibodies against sarcoplasmic reticulum Ca2+ transport protein, revealed that the severe reduction of immunoreactive Ca2+‐ATPase was limited to the histochemical type 2 fibers. The deficiency of the Ca2+ transport protein in the sarcoplasmic reticulum of type 2 fibers, which may be the primary expression of a presumed gene defect, can explain the impaired muscle relaxation of the patients. This disease appears to be a clinically, electromyographically, and biochemically distinct metabolic myopathy.