bluuebunny/crossref_metadata_2025_split huggingface dataset:
A unifying hypothesis for the cause of amyotrophic lateral sclerosis, parkinsonism, and alzheimer disease
Authors: Appel Stanley H. 
Published: January 2005

AbstractThe causes of amyotrophic lateral sclerosis, Parkinson disease, and Alzheimer disease are unknown. Furthermore, treatment for two of these conditions is almost totally lacking. The thesis is presented that each of these disorders is due to lack of a disorder‐specific neurotrophic hormone. The hormone would be elaborated or stored in the target of the affected neurons. It would be released by the postsynaptic cell and then exert its effect in a retrograde fashion after being taken up by the presynaptic terminal. In the lower motor neuron syndromes of amyotrophic lateral sclerosis, failure of muscle cells to release the appropriate motor neuron syndromes of amyotrophic lateral sclerosis, failure of muscle cells to release the appropriate motor neurotrophic hormone would result in impaired function of anterior horn cells. In Parkinson disease, the neurotrophic failure would be characterized by inability of striatal cells to provide the required dopamine neurotrophic hormone with resulting impairment of substantia nigra cells. In Alzheimer disease, the abnormalities would lie in failure of the hippocampus and cortical cells to supply the relevant cholinergic neurotrophic hormone with resulting impairment of medial septal and nucleus basalis neurons. Central nervous system tissue culture provides a convenient system in which to assay these neurotrophic hormones and should permit a test of the hypothesis.
Immunological studies in senile dementia of the alzheimer type: Evidence for enhanced suppressor cell activity
Authors: Miller Aaron E. , Neighbour P. Andrew , Katzman Robert , Aronson Miriam , Lipkowitz Rochelle 
Published: January 2005

AbstractTo examine immunological variables in senile dementia of the Alzheimer type (SDAT), we compared SDAT patients with elderly and young controls with regard to concanavalin A (Con A)–induced suppression, lymphocyte proliferation, interferon production, and serum immunoglobulin levels. The results showed exaggerated Con A suppression and reduced lymphocyte proliferation in SDAT patients compared to elderly control subjects. Interferon production after stimulation by Con A and immunoglobulin levels did not differ between SDAT and elderly control subjects. The results suggest imparied immunoregulatory mechanisms in patients with SDAT that may be related to the cause or course of SDAT. Alternatively, these immunological changes may be secondary to pathological changes in the central nervous system.
Evidence for induction of localized amyloid deposits and neuritic plaques by an infectious agent
Authors: Wisniewski Henryk M. , Moretz Roger C. , Lossinsky Albert S. 
Published: January 2005

AbstractMice inoculated intracerebrally with mouse brain homogenate infected with a particular strain of scrapie agent developed amyloid and neuritic plaques and amyloid tubercles along the injection track, solid wall‐like infiltrations at the injection site, or both, Our observations implicate an infectious agent in the localized formation of the amyloid and the neuritic plaque—the leading pathological change found in normal elderly people and animals and in patients with senile dementia of the Alzheimer type. Further, these studies indicate that microglia appear to play a central role in the pathogenesis of amyloid formation and could be involved in the processing, production, and assembly of the fibers.
Slowly alternating skew deviation: Description of a pretectal syndrome in three patients
Authors: Corbett James J. , Schatz Norman J. , Shults W. Thomas , Behrens Myles , Berry Richard G. 
Published: January 2005

AbstractThree patients who had slowly alternating skew deviation are described: each had elements of the Sylvian aqueduct syndrome. This combination of signs supports a pretectal location for lesions associated with alternating skew movements. Postmortem examination of a patient who died of chronic herpes simplex encephalitis showed extensive demyelination and periaqueductal spongiform degeneration; there was preservation of the oculomotor and trochlear nuclei, the medial longitudinal fasciculus, vestibular nuclei, and the interstitial nucleus of Cajal bilaterally. The slowly alternating dysconjugate vertical movements bear a resemblance to both see‐saw nystagmus and the ocular tilt response.
The irrelevance of fusimotor activity to the achilles tendon jerk of relaxed humans
Authors: Burke David , McKeon Brian , Skuse Nevell F. 
Published: January 2005

AbstractIn two normal subjects the sciatic nerve was blocked completely using concentrated lidocaine. The muscle afferent and reflex electromyographic responses to reproducible percussion of the Achilles tendon were recorded while the blocks developed. The intensity of percussion was sufficient to produce an Achilles tendon jerk in one subject when at rest and in the other during reinforcement. The block did not alter the muscle afferent response to tendon percussion in either subject. It is concluded that background fusimotor activity is not a prerequisite for the tendon jerk and that, during complete relaxation, there may be no significant fusimotor drive directed to the triceps surae. The varying ease with which tendon jerks can be elicited in different normal subjects or in different muscles of the same subject appears to be related not to fusimotor activity but to differences in the “central excitability state”.
Cerebellar degeneration in the rat following rapid correction of hyponatremia
Authors: Kleinschmidt‐DeMasters B. K. , Norenberg Michael D. 
Published: January 2005

AbstractDegenerative lesions in the superior vermis of the cerebellum were produced in 8 of 14 rats that were first made hyponatremic for three days with vasopressin and water and then given hypertonic saline. Within the superior vermis, lesions were predominantly localized to the crests of the folia. These lesions were characterized by demyelination of folial white matter and necrosis of granule cells at the junction with the white matter. In severe degeneration, the entire width of the granule cell layer was involved and Purkinje cell necrosis was found as well. Hyponatremia alone or administration of hypertonic saline to normonatremic rats did not result in lesions. Because of the topographical and histopathological similarity of these lesions to those of alcoholic cerebellar degeneration, our findings raise the possibility of a contribution of electrolyte‐induced injury to the pathogenesis of alcoholic cerebellar degeneration.
Congenital cholinergic nervous system dysfunction in identical twins
Authors: Kien C. L. , Herrmann Jurgen , Hogan Walter 
Published: January 2005

AbstractWe report identical male twins with secretory diarrhea, craniofacial abnormalities often found in G syndrome, hypospadias, and abnormalities of the cholinergic nervous system including achalasia, decreased tearing, corneal hypesthesia, positive Mecholyl test (eye and esophagus), and decreased sweating. The children also lacked fungiform papillae but were clinically distinct from patients with either the Riley‐Day syndrome or acquired dysfunction of the autonomic nervous system. Assessment of autonomic nervous system function should be considered in infants with recurrent diarrhea or swallowing disorders or in children with morphological characteristics suggesting G syndrome.
Binaural voltage summation of brainstem auditory evoked potentials: An adjunct to the diagnostic criteria for multiple sclerosis
Authors: Prasher D. K. , Sainz M. , Gibson W. P. R. 
Published: January 2005

AbstractBrainstem auditory evoked potential (BAEP) amplitude is modified according to whether or not the stimulus is applied monaurally or binaurally. In normal subjects, wave V amplitude increases by an average of 68.7% upon changing stimulation from monaural to binaural. From earlier studies there is evidence that brainstem potential amplitude is reduced in patients with multiple sclerosis (MS) but none to suggest that binaural stimulation results in increased amplitude. This study evaluated the extent of binaural summation of BAEPs in patients with MS.In a large majority of patients with MS who have no hearing deficit, BAEPs showed no increase in wave V amplitude on binaural stimulation. This finding is in contrast to the normal group and thus has diagnostic importance. Measurements of binaural summation therefore might usefully be applied to the clinical assessment of disease progression, or lack of it, in individual patients.
Taurine and hyperexcitable human muscle: Effects of taurine on potassium‐induced hyperexcitability of dystrophic myotonic and normal muscles
Authors: Durelli Luca , Mutani Roberto , Fassio Franco , Satta Andrea , Bartoli Ettore 
Published: January 2005

AbstractProgressively increasing concentrations of potassium chloride in Evans blue saline were administered to patients affected with myotonic dystrophy and to healthy volunteers before and after parenteral treatment with taurine. Excitability changes of thenar eminence muscles were related to the venous potassium and chloride concentrations. The actual electrolyte concentrations were compared to those to be expected if no infused electrolytes had been transported into cells. The expected concentrations were calculated by means of Evans blue dilution. This method permitted quantification of changes of muscle excitability in terms of the potassium chloride concentration capable of disturbing the electrical activity of the studied muscles. The method also provided an indirect evaluation of electrolyte movements across muscle membrane in vivo in humans. Dystrophic myotonic muscles appeared highly sensitive to extracellular potassium and, unlike normal muscles, were unable to accumulate potassium chloride when its external concentration had been raised. Administration of taurine decreased the potassium‐induced muscle hyperexcitability and favored electrolyte accumulation in dystrophic myotonic muscles. The stabilizing effect of taurine is discussed in relation to its ability to increase intracellular potassium concentration, membrane conductance, or both.
Carbamazepine and hematological monitoring
Authors: Hart Robert G. , Easton J. Donald 
Published: January 2005

AbstractClinically important hematological toxicity is uncommon with carbamazepine (CBZ). Only 20 patients with CBZ‐associated aplastic anemia have been reported since 1964. Leukopenia and thrombocytopenia occur in about 2% of patients. Based on the available data on hematological complications of CBZ, we question the justification for expensive, excessive laboratory monitoring.
Biochemical findings in a case of parkinsonism secondary to brain tumor
Authors: De Yýbenes J. García , Gervas J. J. , Iglesias J. , Mena M. A. , Del Rio R. Martín. , Somoza E. 
Published: January 2005

AbstractNeuropathological and biochemical studies were performed in a patient with craniopharyngioma and a long history of parkinsonism. Morphological examination revealed atrophy of the substantia nigra without Lewy bodies. Both the caudate and putamen nuclei showed moderate loss of large cells with glial proliferation. There was a notable decrease in dopamine and its metabolites and a reduction in norepinphrine content in both the caudate and putamen nuclei. Less consistent changes were found in serotonin and amino acid levels. Dopamine receptors, evaluated as 3H‐spiperone binding sites, were unchanged in the putamen but decreased to 30% of control values in the caudate. The findings suggest that parkinsonism was caused by the brain tumor, which damaged both the presynaptic dopaminergic nigrostriatal neurons and the postsynaptic dopamine recptors.
Acute conduction block associated with experimental antiserum‐mediated demyelination of peripheral nerve
Authors: Sumner Austin J. , Saida Kyoko , Saida Takahiko , Silberberg Donald H. , Asbury Arthur K. 
Published: January 2005

AbstractIntraneural injection of antisera from rabbits with high antigalactocerebroside antibody levels into rat sciatic nerve produced acute nerve conduction block. This was first apparent in some motor axons between 30 and 60 minutes after injection and progressed to completion within 2 to 4 hours. Concurrent morphological evidence of demyelination was present, but structural changes at the time of onset of block were mild and were restricted to the myelin and Schwann cell, particularly at the paranodal areas and Schmidt‐Lanterman clefts. It is suggested that paranodal lesions could account for the observed conduction block.
Macroglobulinemia with peripheral neuropathy simulating motor neuron disease
Authors: Rowland Lewis P. , Defendini Richard , Sherman William , Hirano Asao , Olarte M. R. , Latov Norman , Lovelace Robert E. , Inoue Kiyoharu , Osserman Elliot F. 
Published: January 2005

AbstractA 48‐year‐old man with an IgM plasma cell dyscrasia died after 14 months of symptoms and signs typical of motor neuron disease, including widespread fasciculation and normal sensation. Two laboratory results were atypical: cerebrospinal fluid protein content of 132 mg/dl and slow motor nerve conduction. At autopsy, no loss or atrophy of anterior horn neurons was found; instead, degeneration of ventral and dorsal roots and retrograde changes of chromatolysis in motor neurons implied peripheral neuropathy. Most reported cases of neuropathy associated with plasma cell dyscrasias have been sensorimotor or purely sensory, but there have been 14 previous cases of motor disorders.
Plasmapheresis and immunosuppressive drug therapy in the Eaton‐Lambert syndrome
Authors: Dau Peter C. , Denys Eric H. 
Published: January 2005

AbstractFive patients with Eaton‐Lambert syndrome (ELS) without associated malignancy were first treated by plasmapheresis alone, and subsequently by plasmapheresis combined with prednisone and azathioprine therapy. Three of the five were also treated by the immunosuppressive drugs alone. Although all three therapeutic regimens resulted in some clinical and electromyographic (EMG) improvement, the greatest improvement was seen during plasmapheresis combined with prednisone and azathioprine. The initial evoked compound muscle action potential (CMAP) increased during treatment in all patients, while facilitation of the initial CMAP by tetanic stimulation decreased. Overall, the EMG decrement elicited at 2 Hz lessened in four of the five patients. Serious complications probably attributable to guanidine toxicity developed in three patients: two had renal failure and one had gastrointestinal and urinary tract bleeding. Our results suggest that immunosuppressive therapy may have a place in the management of ELS and that circulating factors such as autoantibody may participate in its pathogenesis.
Incomplete manifestations of myotonic dystrophy in a large kinship in labrador
Authors: Pryse‐Phillips William , Johnson Gordon J. , Larsen Bodil 
Published: January 2005

AbstractIn a large kinship affected by myotonic dystrophy (MyD), 133 subjects, including 21 spouses, were examined independently by a neurologist and an ophthalmologist to determine the earliest identifying evidence of the disease. Assessment included tonometry, slit‐lamp examination, facial measurements, and electromyography (EMG). Thirty‐two subjects, all of whom had EMG characteristics of myotonia, were definitely affected. Low intraocular tensions were a more consistent finding than lens opacities; endocrine abnormalities were absent.Twenty‐seven subjects had one to five features but lacked clinical or electrographic evidence of myotonia; they were designated as having a “partial syndrome.” Signs most commonly found included weakness of the upper face, brachial hyporeflexia, low intraocular tensions, and the presence of sparse colored specks at the posterior poles of the lenses. All but 2 subjects (siblings) with the partial syndrome had a parent with either MyD or the partial syndrome. Linkage studies indicated that 7 subjects should not be carrying the gene, while others could be regarded as having a minor expression of MyD. In most persons with the partial syndrome, we could not predict whether typical MyD would develop later. Transmission of the disease may depend upon more than a single autosomal dominant gene, and nongenetic influences may also be important.
Intensive monitoring of interictal psychosis in epilepsy
Authors: Ramani Venkat , Gumnit Robert J. 
Published: January 2005

AbstractTen epileptic patients developed interictal psychosis while being treated in hospital for seizure control. They were subjected to intensive behavioral, video‐electroencephalographic, and serum anticonvulsant monitoring for an average of 7.1 weeks in a specialized epilepsy unit. In 9 patients, the interictal psychosis was indistinguishable from acute schizophrenia. Only 5 of these patients had complex partial seizures; the other 4 showed evidence of generalized epilepsies. Thus a “unique” association between schizophreniform psychosis and complex partial seizures, noted by previous authors, could not be confirmed. Only 1 patient showed normalization of the electroencephalogram during psychosis and an inverse relationship between psychosis and seizure frequency. In most cases the emergence of psychosis could not be explained. Interictal psychosis in epilepsy appears to be a spectrum of disorders that may be multifactorially determined.
Recurrent paraplegia with total recovery from spontaneous spinal epidural hematoma
Authors: Hernandez D. , Vinuela F. , Feasby R. T. E. 
Published: January 2005

AbstractA 51‐year‐old man had three episodes of complete paraplegia and anesthesia, each lasting 30 to 120 minutes, over the course of five hours. Myelography and computerized tomographic scanning showed an epidural hematoma at T11–T12 for which no cause was found. Complete spontaneous recovery occurred.
Central neurogenic hyperventilation in an awake patient with brainstem astrocytoma
Authors: Rodriguez Moses , Baele Philippe L. , Marsh H. Michael , Okazaki Haruo 
Published: January 2005

AbstractA patient had the rare combination of central neurogenic hyperventilation (Pa of 9 torr) and a normal level of consciousness for eight days. Morphine attenuated but never corrected the hyperventilation. Experimental effects of hypocapnia, which decreases both cerebral blood flow and metabolism in humans, are at odds with the normal mentation initially seen in this patient despite her marked and persistent hypocapnia. Death occurred after progressive brainstem dysfunction. Pathological study showed a well‐differentiated astrocytoma involving primarily the medulla and pons, with scattered tumor foci throughout the entire neuraxis. Possible mechanisms for central neurogenic hyperventilation are discussed briefly in relation to the pathological findings and the observed response to morphine.
Biopsy‐proved Alzheimer disease presenting as a right parietal lobe syndrome
Authors: Crystal Howard A. , Horoupian Dikran S. , Katzman Robert , Jotkowitz Seymour 
Published: January 2005

AbstractA progressive right parietal lobe disorder charcterized by astereognosis and pseudoathetosis developed in a 57‐year‐old woman. Two years later, the patient began to show signs of intellectual deterioration. Biopsy of the right frontal lobe revealed numerous plaques and tangles. The patient's right parietal lobe dysfunction was probably the first manifestation of Alzhemer disease. On rare occasions, Alzhemier disease can present as a focal syndrome with findings referable to a localized area of maximal involvement.
Catencholamine neuron systems in brain
Authors: Moore Robert Y. 
Published: January 2005

AbstractThe organization of catecholamine neuron systems in brain systems in brain is described. Dopamine neuron systems form components of larger, more general neuron systems and are organized into conventional topographic projectoins. Norepinephirne neuron systems innervate widespread areas of the neuroxais and appear to function in the regulation of other neuronal systems.
Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children
Authors: Goutières F. , Aicardi J. 
Published: January 2005

AbstractThree children ranging in age from 1 to 41/4 years suffered an acute illness with disturbance of consciousness followed by motor rigidity, loss of spontaneous movements and language, axial hypotonia, and a stereotyped response to any kind of stimulus. In two cases, images on computerized tomography were consistent with necrosis of the lenticular and caudate nuclei. The acute onset was followed by later improvement and stabilization. One child died, probably not as a direct consequence of the neurological disorder; autopsy showed bilateral necrosis of the putamina.We suggest that three patients, together with two previously reported cases, constitute a clinically and radiologically recognizable subgroup among the heterogeneous disorders that produce bilateral striatal necrosis in children. The cause of the syndrome is unknown.
Cerebral metabolism and atrophy in huntington's disease determined by 18FDG and computed tomographic scan
Authors: Kuhl David E. , Phelps Michael E. , Markham Charles H. , Metter E. Jeffrey , Riege Walter H. , Winter James 
Published: January 2005

AbstractPatterns of local cerebral glucose utilization were measured with positron emission computed tomography using the 18F‐fluorodeoxyglucose method in 13 patients with Huntington's disease (HD), 15 subjects at risk for HD, and 40 normal control subjects. These data were compared with computed tomographic measures of cerebral atrophy, with age, and with duration and severity of symptoms. The results indicate that in HD there is a characteristic decrease in glucose utilization in the caudate and putamen and that this local hypometabolism appears early and precedes bulk tissue loss. In contrast to patients with senile dementia, in these HD patients glucose utilization typically was normal throughout the rest of the brain, regardless of the severity of symptoms and despite apparent shrinkage of brain tissue. Our results suggest the possibility that the caudate may be hypometabolic in some asymptomatic subjects who are potential carriers of the autosomal dominant gene for HD.
Correlation of isotopic cisternographic patterns in multiple sclerosis with CSF IgG values
Authors: Bartolini S. , Inzitari D. , Castagnoli A. , Amaducci L. 
Published: January 2005

AbstractThirty‐eight patients with multiple sclerosis (MS) were examined with isotopic cisternography (IC) in order to study cerebrospinal fluid (CSF) dynamics. Cisternography was also performed in 15 patients with amyotrophic lateral sclerosis and in 14 with senile dementia of the Alzheimer type as controls. IC pattern of “mixed” type was found in 18 MS patients and all those with Alzheimer senile dementia examined, while the IC examination did not show abnormality in any of 15 patients with amyotrophic lateral sclerosis. In MS patients with amyotrophic lateral sclerosis. In MS Patients, the abnormal IC picture proved to be significantly correlated with the CSFIgG values as calculated by Link's and Tourtelotte's formulas. The abnormal IC in MS may be due to altered CSF reabsorption or increased transependymal flow, or it may be related to the abnormal concentration of IgG.