bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Progressive familial encephalopathy in infancy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis
Authors: Aicardi J. , Goutières F. 
Published: January 2005

AbstractEight infants developed a progressive disorder of the central nervous system with bilateral spasticity and dystonia, acquired microcephaly, and a rapid course toward profound deterioration and death. All the patients had abnormal cerebrospinal fluid with mild but persistent lymphocytosis. Computed tomography showed various combinations of bilateral symmetrical calcifications in the basal ganglia, progressive brain atrophy, and deep white matter hypodensities, the first two being present in all families but not in every individual patient. The disorder is familial and probably genetic in origin, although some features, especially the pleocytosis, may erroneously suggest an inflammatory condition.
Biochemical studies of pyridoxal and pyridoxal phosphate status and therapeutic trial of pyridoxine in patients with carpal tunnel syndrome
Authors: Smith Gillian P. , Rudge P. J. , Peters T. J. 
Published: January 2005

AbstractA number of recent studies report response of patients with carpal tunnel syndrome to pyridoxine treatment. Neurological and biochemical studies were therefore performed on six patients both before and after treatment with pyridoxine for at least 9 weeks. Free pyridoxal, pyridoxal phosphate, and total pyridoxal were assayed in plasma and neutrophils. The pyridoxal status was also estimated by assaying red cell aspartate aminotransferase. No evidence was obtained to suggest that these patients were deficient in either pyridoxal or pyridoxal phosphate. Although four of the patients claimed some partial symptomatic relief, there was no consistent improvement in clinical findings or neurophysiological measurements following pyridoxine treatment.
Huntington's disease: Survival of large striatal neurons in the rigid variant
Authors: Bugiani Orso , Tabaton Massimo , Cammarata Sergio 
Published: January 2005

AbstractA morphometric method was employed to investigate the relationship of rigidity and hyperkinesia to the degree of striatal and nigral nerve cell loss in one patient with the rigid variant of Huntington's disease and four patients with hyperkinetic chorea. Both striatal neuron populations, small and medium‐sized neurons and large neurons, were affected in the patients with hyperkinetic chorea, whereas the large neurons were preserved in the patient with the rigid variant. The substantia nigra was slightly involved in each patient. The findings suggest that the rigid variant of Huntington's disease may reflect the unbalanced activity of residual, mostly large, striatal neurons, inhibiting the substantia nigra.
Effect of baclofen on sleep‐related periodic leg movements
Authors: Guilleminault Christian , Flagg Wayne 
Published: January 2005

AbstractFive patients with nocturnal myoclonus (periodic leg movements during sleep), mean age 59.6 years, were monitored lygraphically for fifteen successive nights. Using a double‐blind drug study design with placebo at baseline, we inestigated the effect of baclofen on these patients. All patients had the repetitive sleep‐related abnormal movements dring both the baseline nights and those on which baclofen had been administered. The number of movements varied during the four baseline nights, but the movements induced sleep fragmentation, i.e., very short electroencepha graphic changes. Baclofen increased the number of movements but decreased their amplitude during non–rapid eye movevement (REM) sleep and shortened the interval between movements. Its effect on sleep was dose related: as dosages Ceased, delta sleep progressively increased and REM sleep decreased. Sleep fragmentation resulting from muscle aches decreased, as indicated by the diminution in alpha electroencephalographic arousals and K complexes. Baclo‐dosages of 20 mg and 40 mg were the most efficacious.
Neglect in children
Authors: Ferro José M. , Martins Isabel Pavão , Távora Luis 
Published: January 2005

AbstractThree children developed neglect following right‐hemispheric lesions. In the first, computed tomography (CT) demonstrated a striatoinsular infarction. In the second, CT revealed a large meningioma compressing the right hemisphere, and in the third, CT revealed two intracerbrall hemorrhages in the posterior parietal and ocupital lobes. Neglect in these children produced a clinical picture comparable to that occurring in adults, but there was a rapid and complete recovery. The paucity of reports of neglect in children may be a function of the rapid recovery, the phenomenon being missed if not looked for in the acute atage. We conclude that, at least for children aged aged 6 years or more, the right hemisphere is dominant for directed selective attention.
Hypothalamic mass and gigantism in neurofibromatosis: Treatment with bromocriptine
Authors: Duchowny Michael S. , Katz Raananah , Bejar Rafael L. 
Published: January 2005

AbstractA child with neurofibromatosis exhinbited gigantism and acromegaly in association with a hypothalamic mass lesion. Bromocriptine, 5 mg daily, reduced somatic growth rate and restored biochemical mohesostasis but had no effect on tumor grwoth. Radiation therapy arrested tumor enlargement and stabilized deeriorating visual function.
Parkinson's disease: Neuron loss in the nucleus basalis without concomitant Alzheimer's disease
Authors: Nakano Imaharu , Hirano Asao 
Published: January 2005

AbstractA representative region of the nucleus basalis of Meynert was investigated in 11 patients with idiopathic Parkinson's disease and compared with the identical region in 13 age‐matched control subjects. Simultaneously, the cerebral cortex and the nucleus basalis in the patients with Parkinson's disease were examined for senile plaques and Alzheimer's neurofibrillary tangles. The nucleus basalis was significantly depleted of its large neurons in Parkinson's disease (p < 0.001 versus controls; Student t test), but in the majority of cases the neuron loss was not associated with Alzheimer's disease.
Axolemma is a mitogen for human Schwann cells
Authors: Sobue Gen , Brown Mark J. , Kim Seung U. , Pleasure David 
Published: January 2005

AbstractThe mechanisms responsible for the induction of Schwann cell proliferation in peripheral nerves undergoing wallerian degeneration and segmental demyelination are not understood. To determine whether contact with axolemma stimulates mitosis of human Schwann cells, cultured Schwann cells from spinal roots obtained postmortem and from sural nerve biopsy specimens were incubated with axolemmal fractions prepared from human spinal cord or from adult rat central nervous system. Schwann cell proliferation was estimated by autoradiographic assay of tritiated thymidine incorportion. Schwann cell labeling indices after exposure to human or rat axolemmal fractions ranged from 26.7 to 59.9%; labeling indices of Schwann cells cultured without axolemmal fraction were 9.8 to 22.4%. The stimulation index, or ratio of Schwann cell labeling index with axolemmal fraction to that without axolemmal fraction, ranged from 1.97 to 3.40. This study demonstrates that both human and rat axolemma are capable of stimulating human Schwann cell replication in vitro.
Reversible visual evoked potential abnormalities in vitamin E deficiency
Authors: Messenheimer John A. , Greenwood R. S. , Tennison M. B. , Brickley J. J. , Ball C. J. 
Published: January 2005

AbstractA patient with cystic fibrosis and cirrhosis developed a progressive neurological syndrome associated with ataxia, proximal weakness, and ophthalmoplegia. Profound deficiencies of vitamins A, D, and E were present. Visual acuity and results of retinal funduscopy were normal. The pattern reversal visual evoked potential was initially abnormal (P100 latency, 136 and 130 ms from left and right eyes, respectively) but became normal (less than 3 standard deviations from mean control P100 latency) over a two‐month period when vitamin E was administered. This case documents a potentially reversible visual evoked potential abnormality in a visually asymptomatic patient with vitamin E deficiency.
Monoclonal antibody analysis of mononuclear cells in myopathies. I: Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells
Authors: Arahata Kiichi , Engel Andrew G. 
Published: January 2005

AbstractIn 76 muscle specimens (normal controls, 9; Duchenne dystrophy, 11; scleroderma, 11; dermatomyositis, 13; polymyositis, 15; inclusion body myositis, 17), mononuclear cells were analyzed at perivascular, perimysial, and endomysial sites of accumulation. Monoclonal antibodies reactive for B cells, T cell subsets, killer (K) or natural killer (NK) cells, and the Ia antigen were used for cell typing. Macrophages were identified by the acid phosphatase reaction. Few extravascular mononuclear cells occurred in normal muscle. In all inflammatory myopathies, a mixed exudate of T cells, B cells, and macrophages was present. Mature K/NK cells were rare in all diseases. In dermatomyositis, polymyositis, and inclusion body myositis, there was a positive gradient for T cells, T8+ cells, and activated T cells and a negative gradient for B cells and T4+ cells between perivascular and endomysial sites. In scleroderma the predominant perimysial exudate consisted mostly of T cells and macrophages. The percentage of B cells at all sites, and the T4+/T cell ratio in the endomysium, were significantly higher in dermatomyositis than in the other diseases. In polymyositis and inclusion body myositis. The endomysial exudate contained a large number of T cells, T8+ cells, and activated T cells but only sparse B cells. T cells accompanied by macrophages focally surrounded and invaded nonnecrotic fibers in polymyositis and inclusion body myositis. Rare fibers in Duchenne dystrophy and a very few fibers in dermatomyositis and scleroderma were similarly affected. We infer that (1) T‐B, T‐T, and T‐macrophage cooperativities are likely to exist in muscle in different myopathies; (2) T cell‐mediated fiber injury plays a role in polymyositis and inclusion body myositis; (3) T cell–mediated fiber injury can also occur in inherited diseases, such as Duchenne dystrophy; and (4) a local humoral response may occur in muscle in dermatomyositis and possibly in polymyositis and inclusion body myositis.
Decreased axon caliber and neurofilaments in hereditary motor and sensory neuropathy, Type I
Authors: Nukada Hitoshi , Dyck Peter James 
Published: January 2005

AbstractThe axons of large‐ and intermediate‐diameter myelinated fibers of sural nerves of patients with hereditary motor and sensory neuropathy, type I (HMSN‐I), were previously found to be attenuated relative to their myelin spiral length. We inferred that axonal atrophy might account for secondary segmental demyelination and remyelination. To assess whether the observed axonal atrophy could be explained by a decrease in neurofilaments, we have evaluated the number of neurofilaments, microtubules, and other axon organelles in sural nerves of patients with HMSN‐I. Whereas the density per square micrometer of neurofilaments or microtubules in diseased nerves was not significantly different from that in control specimens, the number of neurofilamets per axon as related to myelin spiral length was significantly less for intermediate and large myelinated fibers in HMSN‐I nerves. The regression lines for the number of microtubules per axon on myelin spiral lengths, were also less steep in HMSN‐I, but the difference did not reach statistical significance. These results indicate that the number of neurofilaments is proportional to axon diameter but significantly below that expected considering myelin spiral length. Decreased neurofilament synthesis, assembly, or transport may underlie the axonal atrophy in HMSN‐I.
Encephalitis and aseptic meningitis, Olmsted County, Minnesota, 1950–1981: I. Epidemiology
Authors: Beghi Ettore , Nicolosi Alfredo , Kurland Leonard T. , Mulder Donald W. , Hauser W. Allen , Shuster Lynne 
Published: January 2005

AbstractAll cases fulfilling stated criteria for encephalitis and aspectic meningitis in Olmsted County, Minnesota, for the period 1950 through 1981 were identified. This is, to our knowledge, the first such incidence and trend study in a delineated population, providing rates per 100,000 person‐years of 7.4 for encephalitis (189 cases) and 10.9 for aseptic menigitis (283 cases). These are about twelve and six times higher, respectively, than the rates reported by the Centers for Disease Control. The rates have been stable over successive 5‐ or 10‐year periods except for a recent increase in aseptic meningitis. Both conditions were more common in the summer months, in childhood, and among males. Viral identification using conventional laboratory tests has improved with time; in the period 1970 through 1981, virus type was specified in about one‐fourth of the cases. The most common agents identified were California and mumps viruses in encephalitis, and entero and mumps viruses in aseptic meningitis. Antecedent and/or concurrent infections were noted in 42 and 35% of encephalitis and aseptic meningitis cases, respectively. No case due to mumps, measles, or rubella viruses has occurred since 1972, reflecting the impact of immunizations. Recovery was reported at the end of the acute phase in 95% of patients with aseptic meningitis, and there were no deaths. Seventy‐eight percent of encephalitis patients recovered completely; the case fatality rate was 3.8%. Of the encephalitis cases, 2% were diagnosed initially postmortem.
Neurofilament breakdown products in degenerating rat and human peripheral nerves
Authors: Trojanowski J. Q. , Lee Virginia M.‐Y. , Schalaepfer W. W. 
Published: January 2005

AbstractThe fate of neurofilament (NF) proteins was examined in sections of nerves from rats that survived for 3 to 90 days following nerve transection, using anti‐NF monoclonal antibodies and immunohistochemical techniques. The same methods were also applied to twenty‐three human nerve biopsy specimens. Granular debris with NF immunoreactivity was observed in the rat nerves for up to 21 days after transection. Similar granular debris with NF protein immunoreactivity was observed in nerve biopsy speciments from patients with axonal degeneration. This change was most evident in specimens that demonstrated abundant ovoids in teased fiber preparations and in a patient with a recent traumatic nerve transection. Similar granular debris was not observed in normal rat or human nerves. We conclude that degenerating NF proteins have a distinctive granular appearance and that NF immunoreactivity can be demonstrated in NF breakdown products with immunohistochemical techniques for prolonged periods of time in rat and human nerves undergoing axonal degeneration. Immunohistochemical studies with anti‐NF monoclonal antibodies offer a novel approach to the evaluation of human nerve biopsy specimens.
Classic and generalized variants of Pick's disease: A clinicopathological, ultrastructural, and immunocytochemical comparative study
Authors: Munoz‐Garcia D. , Ludwin S. K. 
Published: January 2005

AbstractSix sporadic cases of dementia with lobar atrophy and neuronal cytoplasmic inclusions (Pick's disease) could be separated into two groups on the basis of the involvement of subcortical structures, the distribution and the histochemical, immunochemical, and ultrastructural characteristics of the inclusions, and possibly the age at onset. The first group (classic) was characterized by predominantly cortical atrophy and the presence in the hippocampus and neocortex of argyrophilic cytoplasmic inclusion bodies that reacted with a monoclonal antibody against neurofilament proteins and antitubulin antisera. Ultrastructurally the bodies were composed of straight fibrils of variable diameter, averaging 15 nm, and long‐period constricted fibrils. The second group (generalized) showed subcortical as well as cortical atrophy. Cortical and subcortical cytoplasmic inclusions contained RNA and stained poorly with silver and antibodies against neurofilaments and microtubules. Ultrastructurally the straight fibrils composing the bodies were coated with granular material, presumed to be derived from ribosomes. The generalized cases occurred in younger patients than did the classic cases in this series.
The risk of intracerebral hemorrhage during oral anticoagulant treatment: A population study
Authors: Wintzen A. R. , de Jonge H. , Loeliger E. A. , Bots G. T. A. M. 
Published: January 2005

AbstractIn a retrospective study of 166 patients, all admitted to the University Hospital, Leiden, The Netherlands, between January 1, 1970 and December 31, 1979, we estimated the relative risk of intracerebral hemorrhage from oral anticoagulant therapy. The risk was more than ten times higher for patients over 50 years of age than for similarly aged untreated individuals in the general population. Within this age group the risk was influenced by neither age nor sex. Hypertension, present in 80% of the patients, was the most important predisposing condition; the risk of bleeding rose with increasing intensity of anticoagulation. There was no substantial difference in clinical condition at onset, rate of Progression, mortality, or degree of recovery between patients with anticoagulant‐associated hemorrhage and those with spontaneous intracranial hemorrhage.
Familial occurrence of amyotrophic lateral sclerosis, parkinsonism, and dementia
Authors: Schmitt Horst P. , Emser Wendelin , Heimes Christian 
Published: January 2005

AbstractWe report here on a 59‐year‐old man from southwest Germany who died after a 14‐year course of an illness characterized by progressive dementia, parkinsonism, and amyotrophic lateral sclerosis. Postmortem examination revealed Alzheimer's neurofibrillary tangles in the substantia nigra, innominata, locus ceruleus, parahippocampal gyrus, and less frequently in the hippocampus and the cerebral cortex. Investigation of the patient's pedigree back to the 17th century revealed nine additional family members who had exhibited signs of amyotrophic lateral sclerosis or parkinsonism‐dementia or both. The pedigree suggests that a recessive trait with genetic epistasis is responsible for the disorder.
Multiple sclerosis immune complexes: An analysis of component antigens and antibodies
Authors: Coyle P. K. , Procyk‐Dougherty Zora 
Published: January 2005

AbstractSerum immune complexes were isolated from 21 patients with multiple sclerosis and 23 control subjects, who were normal or had other neurological diseases, and the probed for neurotropic viral and brain‐reactive components. Multiple sclerosis complexes contained antibody to herpes simplex (13 cases), measles (8 cases), cytomegalovirus (5 cases), and rubella virus (5 cases). In some complexes, herpes simplex or cytomegalovirus antigen was detected along with antibody. Myelin basic protein or antibody to myelin basic protein was found in the complexes of 7 patients with multiple sclerosis and 2 patients with other neurological diseases. Serum complexes containing antibody reactive with galactocerebroside and ganglioside were present in 12 patients with multiple sclerosis and 3 with other neurological diseases. Over 60% of the multiple sclerosis group had IgM and IgA serum complexes, including 5 patients with very high IgA complex levels. This study suggests that patients with multiple sclerosis have ongoing systemic virus production with resultant immune complex formation. In addition, they often have serum complexes that contain brain‐reactive components.
Muscle fiber degeneration and necrosis in muscular dystrophy and other muscle diseases: Cytochemical and immunocytochemical data
Authors: Cornelio F. , Dones I. 
Published: January 2005

AbstractSerial consecutive frozen sections from 164 muscle biopsy specimens were studied cytochemically. We localized calcium and albumin as endogenous markers of extracellular fluid penetration and C3 and C9 complement components as markers of muscle fiber necrosis. In both Duchenne dystrophy and congenital muscular dystrophy, a significant percentage of fibers were overloaded with calcium and penetrated by albumin. Most of these fibers appeared opaque with trichrome stain. C3 and C9 complement appeared only in necrotic fibers, which invariably were also penetrated by albumin. These observations support previous findings that muscle fiber necrosis is linked to massive inflow of extracellular fluid and complement activation. In addition, in both Duchenne and congenital dystrophies, numerous nonnecrotic fibers are penetrated by calcium‐rich extracellular fluid.
Human brain glucose utilization and cognitive function in relation to age
Authors: Duara R. , Grady PhD C. , Haxby PhD J. , Ingvar D. , Sokoloff L. , Margolin R. A. , Manning PhD R. G. , Cutler N. R. , Rapoport S. I. 
Published: January 2005

AbstractBrain oxidative metabolism was examined with positron emission tomography and {18F}2‐deoxy‐D‐glucose in 40 healthy men aged 21 to 83 years, under conditions of reduced visual and auditory stimulation. The mean cerebral metabolic rate for glucose (CMRglc) equaled 4.6 to 4.7 mg. 100 gm−1· min−1 and did not correlate significantly with age (p > 0.05). Regional cerebral metabolic rates for glucose (rCMRglc) and Q ratios (rCMRglc/CMRglc), which had lower coefficients of variation than did rCMRglc, also did not correlate with age. Hyperfrontality of cerebral metabolism was absent at all ages. Age decrements were demonstrated in the error score on the Benton Revised Visual Retention Test and in the Performance Subtest scaled score of the Wechsler Adult Intelligence Scale (WAIS) but not in the Verbal Subtest scaled score of the WAIS. The cognitive test scores did not correlate with brain metabolic rates. The results indicate that brain oxidative metabolism, when measured under resting conditions with reduced sensory input, is not reduced in relation to age in healthy men. Furthermore, no significant relations between intelligence and resting cerebral metabolism are evident.
Antispasticity drugs: Mechanisms of action
Authors: Davidoff Robert A. 
Published: January 2005

AbstractSeveral different drugs are now used, or are potentially useful, to treat patients with spasticity. Although these compounds vary in their actions on spinal neurons and reflex arcs, it is possible to formulate reasonable hypotheses regarding their modes of action. The benzodiazepines bind to specific benzodiazepine receptors linked to classic γ‐aminobutyric acid (GABA) receptors located on the terminals of primary afferent fibers. This binding results in an increased affinity of the GABA receptor for the amino acid, an augmented flux of chloride ions across the terminal membrane, and an increase in the amount of presynaptic inhibition. Baclofen activates GABAB receptors putatively located on the same terminals. Activation of these receptors retards the influx of calcium ions into the terminals, thereby reducing the evoked release of excitatory amino acids and possibly other transmitters. Progabide and its metabolites act on both classic and GABAB receptors. Glycine works on specific inhibitory receptors located on spinal interneurons and motoneurons. The phenothiazines act on the brainstem to alter the function of fusimotor fibers. Phenytoin and carbamazepine reduce the afferent output of muscle spindles. Dantrolene diminishes the activation of the contractile process in muscle fibers by reducing the release of calcium ions from the sarcoplasmic reticulum. This review summarizes the data supporting these concepts.