bluuebunny/crossref_metadata_2025_split huggingface dataset:
Transient visual obscurations with elevated optic discs
Authors: Sadun Alfredo A. , Currie John N. , Lessell Simmons 
Published: January 2005

AbstractThe pathogenesis of the transient obscurations of vision that occur with papilledema is a subject of speculation and debate. We present four examples of transient obscurations of vision that were clinically indistinguishable from those of papilledema; they occurred in patients with elevated optic discs from causes not related to increased intracranial pressure. The underlying mechanism for visual obscurations in all of these patients appears to be transient ischemia of the optic nerve head consequent to increased tissue pressure. Axonal swelling, intraneural masses, and increased influx of interstitial fluid may all contribute to increases in tissue pressure in the optic nerve head. The consequent reduction in perfusion pressure renders the small, low‐pressure vessels that supply the optic nerve head vulnerable to compromise. Brief fluctuations in intracranial or systemic blood pressure may then result in transient loss of function in the eyes. We postulate that such mechanisms may apply in all cases of optic disc elevation, including papilledema.
More intense focal seizure types after callosal section: The role of inhibition
Authors: Spencer Susan S. , Spencer Dennis D. , Glaser Gilbert H. , Williamson Peter D. , Mattson Richard H. 
Published: January 2005

AbstractCorpus callosum section reduces the incidence of generalized seizures associated with loss of conciousness. Minimal data are available, however, regarding the types of seizures that may be present following surgery. Five of 17 patients in our clinic who underwent partial or complete corpus callosum section developed more intense and sometimes newly patterned (but not necessarily more frequent) focal seizures postoperatively. These patients were not predictably different from the other 12 in terms of age at onset of seizures or at surgery, cause of seizures, extent of callosum sectioned, radiographically demonstrable structural lesions, or the types of preoperative seizures. All 5 patients, however, had bilateral independent frontal electroencephalographic foci. No patients without these electroencephalographic findings developed more intense focal seizures after surgery. The findings are consistent with the results of experimental animal studies and argue for a suppressive influence of the contralateral hemisphere on some types of seizures.
Ocular palsy preceding chronic relapsing polyneuropathy by several weeks
Authors: Donaghy Michael , Earl C. J. 
Published: January 2005

AbstractThree patients exhibited an unusual variant of chronic relapsing polyneuritis in which extroacular muscle palsies developed 19 days, 20 days, and 31/2 months, respectively, before the neuropathy affected the limbs. The peripheral Neuropathy evolved over 7 to 12 weeks, lasted 15 months to 13 years, and relapsed in every case. Each patients had dysphagia, areflexia, sensory loss, and weakness of all four limbs, which in two was asymmetrical. Slowed motor nerve conduction and prolonged F wave latencies were present, and the cerebrospinal fluid protien level was elevated without increased cell count. No patient recovered fully despite treatment with immunosuppressant drugs.
Hypophysectomy mitigates skeletal muscle fiber damage in hamster dystrophy
Authors: Karpati G. , Jacob P. , Carpenter S. , Prescott S. 
Published: January 2005

AbstractAblation of the pituitary gland by a rediofrequency lesion markedly retarded the musculoskeletal growth of young dystrophic hamsters. The prevalence of centronucleared muscle fibers, which is a reliable cumulative index of the microscopic patholgical expression of dystrophy, was drastically reduced in quadriceps muscles of 35‐ and 45‐day‐old hypophysectomized dystrophic hamsters, campared with sham‐operated controls. Mitigation of skeletal muscle fiber damage by musculoskeletal growth retardation may also occur in human dystrophy.
Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome
Authors: Wisniewski K. E. , Wisniewski H. M. , Wen G. Y. 
Published: January 2005

AbstractOne hundred brains of patients with Down's syndrome(DS) who died in insstitutions for chronic care were exemined for clinicopathological correlation of Alzheimer's disease. Fifty‐one were below and 49 were above age 30 years at death. Tissues from the right, prefrontal, and hippocampal cortices were processed for microscopy using H & E and Bodian‐periodic acid‐schiff impregnation. M orphometric evluations of plaques and tangles were carried out Plaques or plaques and tangles were found in the brains of 56 patients with DS, 7 below age 30 and 49 above the age of 30. The brains of the patients with DS who also had clinical dementia had more than twenty plaques or plaques and tangles per 1.5 × 106 μm2 of cortex. The numbers of plaques and tangles found in the brains of the patients with DS above the age of 30 greatly increased with age but varied from brain to brain. These observations suggest a coreelation among dementia, the density of plaques and tangles, and age. All 100 brains studied showed early arrest of brain growth and brain atrophy, a condition that may have been due to prenatal arrest of neurogenesis mainly in the granular cell layers, prenatal and postnatal arrest of synaptogenesis, and early aging. Plaques and tangles developed twenty to thirty years earlier and dementia was clinically detected at least three times more frequently (20 to 30%) in DS than it is known to occur in the non‐DS population.
Alterations in regional brain concentrations of neurotensin and bombesin in Parkinson's disease
Authors: Bissette Garth , Nemeroff Charles B. , Decker Michael W. , Kizer John S. , Agid Yves , Javoy‐Agid France 
Published: January 2005

AbstractFrozen samples of postmortem human brain tissue from patients with Parkinson's disease(n = 25) and control patients who died without neurological disease (n = 25) were assayed for neurotensin and bombesin by specific radioimmunoassay. Twelve brain regions were examined: substantia nigra, ventral tegmental area, periaqueductal gray matter, caudate nucleus, putamen, globus pallidus, amygdala, hippocampus, nucleus accumbens, frontal cortex, cingulate cortex, and entorhinal cortex. In patients with Parkinson's disease, the concentration of neurotensin was significantly reduced in the hippocampus. The concentration of neither peptide was significantly altered in the substantia nigra or ventral tegmental area, two regions known to exhibit reductions in other neurotransmitter substances.
The relationship between levels of cerebrospinal fluid myelin basis protein and IgG measurements in patients with multiple sclerosis
Authors: Warren Kenneth G. , Catz Ingrid 
Published: January 2005

AbstractCerebrospinal fluid (CSF) myelin basic protein (MBP) levels, CSF/serum, albumin ratio (CSF/S alb), and 4 CSF IgG measurements‐absolute CSF IgG level (CSF IgG), CSF IgG/albumin ratio, the Tibbling‐Link IgG index, and the daily rate of intrathecal IgG synthesis‐were measured in patients with multiple sclerosis and control subjects. In four clinical subgroups of patients, including 22 with polysymptomatic exacerbations, 22 with monosymptomatic exacerbations, 41 with chronic progressiv disease and 21 in remission, there was no correlation between CSF MBP and either CSF/S alb or the CSF IgG measurements. This finding was also observed in longitudinal studies of patients. CSF MBP lavel, as determined in a cross‐sectional stydy of 325 patients with multiple sclerosis, are an exellent indicator of disease activity.
Atypical multiple sclerosis with concentric of lamellar demyelinated lesions: Two Japanese patients studied post mortem
Authors: Itoyama Yasuto , Tateishi Jun , Kuroiwa Yoshigoro 
Published: January 2005

AbstractIn two Japanese patients with atypical multiple sclerosis studied post mortem, there was a concentric or lamellar pattern of demyelinated lesions. This was detected in the thoracic spinal cord of one patient and in the optic chiasm of the other. In addition, lesions more typical of multiple sclerosis were present. Clinically, both cases ran courses intermediate between Baló's disease and multiple sclerosis. The patients had a relatively rapid progression of disease and had severe visual disability or paraplegia associated with faver, headache, and pleocytosis. The formation of a concentric or lamellar pattern of the lesions in cases of demyelinating diseases may relate to the brief duration or to the acuteness or severity of the disease process.
Induction of reproducible brain infarction by photochemically initiated thrombosis
Authors: Watson Brant D. , Dietrich W. Dalton , Busto Raul , Wachtel Mitchell S. , Ginsberg Myron D. 
Published: January 2005

AbstractWe have used a photochemical reaction in vivo to induce reprodcible thrombosis leading to cerebral infarction in rats. After the intravenous injection of rose bengal, a potent photosensitzing dye, an ischemic lesion was formed by irradiating the left parietal convexity of the exposed skull for 20 minutes with green light(560nm) from a filtered xenon arc lamp. Animals were allowed to surivive form 30 minutes to 15 days after irradiation. Early microscopic alerations within the irradiated zone included the formation of thrombotic plugs and adjacent red blood cell stasis within pial and parenchymal vessels. Scanning electron microscopy revealed frequent platelet aggregates adhering to the vascular endothelium, often resulting in vascular occlusion. Carbon‐black brain perfusion demonstrated that occlusion of vascular channels progressed after irradiation and was complete within 4 hours. Histopathological examination at 1, 5, and 15 days revealed that the associated infarct evolved reproducibly through several characteristic stages, including a phase of massive macrophage infiltration. Although cerbral infarction in this model is initiated by thrombosis of small bolld vessels, the fact that the main pathological features of stroke are consistently reproduced should permit its use in assessing treatment regimens, Further, the capability of producing infarction in preselected cortiacal regions may facilitate the study of behavioral, functional, and structural consequences of acute and chronic stroke.
Tissue culture studies of neurofibromatosis: Effects of axolemmal fragments and cyclic adenosine 3′,5′‐monophosphate analogues on proliferation of schwann‐like and fibroblast‐like neurofibroma cells
Authors: Sobue Gen , Sonnenfeld Kenneth , Rubenstein Allen E. , Pleasure David 
Published: January 2005

AbstractSix dermal neurofibromas obtained from 5 patients with neurofibromatosis were dissociated and the cells were plated on polylysine‐coated glass. Two principal cell types were observed in the cultures: elongated and bipolar Schwann‐like cells (SLCs), and polymorphic flattened fibroblast‐like cells (FLCs). Indirect immunofluorescence demonstrated that SLCs expressed surface laminin but not surface fibronectin; FLCs expressed surface fibronectin but were only weakly positive for surface laminin. Tritiated thymidine autoradiography demonstrated that cultured SLCs proliferated slowly (labeling index, 0.7 to 4.0%), whereas FLCs divided more rapidl%(labeling index, 7.5 to 26.4%). Axolemmal fragments prepared from human or rat central nervous system specimens adhered to SLCs derived from each of the 6 neurofibromas, but not to FLCs. Axolemmal fragments induced a marked proliferative response of SLCs from 2 of the 6 neurofibromas but had no effect on proliferation of SLCs from the other 4 neurofibromas or FLCs from any of the 6 neurofibromas. In one patient from whom 2 neurofibromas were obtained, SLCs from one neurofibroma responded to axolemmal fragments, while SLCs from the other did not. Treatment of the cultures with 0.1 mM cyclic adenosine 3′,5′‐monophosphate (cAMP) analogue, 8‐bromo cAMP, caused marked inhibition of proliferation of both SLCs and FLCs derived from all 6 neurofibromas. The same concentration of another cAMP analogue, dibutyryl cAMP, inhibited proliferation of SLCs but not of FLCs.
Hypoglycemia masquerading as cerebrovascular disease (hypoglycemic hemiplegia)
Authors: Wallis William E. , Donaldson Ivan , Scott Russell S. , Wilson John 
Published: January 2005

AbstractHypoglycemia produced hemiplegia with right‐sided predilection in 16 patients initially suspected of having suffered a stroke. Fifteen patients had no demonstrable brain disease, and the hemiplegia cleared rapidly once the hypoglycemia was corrected. Invasive investigations such as carotid arteriography are not required in most patients. The features of hypoglycemia hemiplegia suggest that a selective neuronal vulnerability and not underlying focal brain disease is responsible in most cases.
Neurological complications in infants and children with acquired immune deficiency syndrome
Authors: Belman Anita Lesgold , Ultmann Monica H. , Horoupian Dikran , Novick Brian , Spiro Alfred J. , Rubinstein Arye , Kurtzberg Diane , Cone‐Wesson Barbara 
Published: January 2005

AbstractNeurological complications occurred in 6 children, aged 6 months to 5 years, with acquired immune deficiency syndrome who were followed for 14 months. The most frequent manifestations included encephalopathies, acquired microcephaly, and pyramidal tract signs. Computed tomographic examinations showed variable degrees of cortical atrophy with ventricular dilatation and calcification. Electrophysiological abnormalities were demonstrated. Two children had documented central nervous system infections. Neurological deterioration resulted in dementia in 3 children. Cognitive impairment and developmental delays were evident in the other 3. Postmortem examination of the 3 children who died showed subacute cytomegalovirus encephalitis in 1; nonspecific hemispheric white matter changes, calcific vasopathy of the basal ganglia, and striking bilateral corticospinal tract degeneration in the second; and extensive calcific vasopathy of the basal ganglia and frontal centrum semiovale, and bilateral attenuation of the frontopontine and corticospinal tracts in the third.
Diabetic autonomic dysfunction, blood pressure, and sleep
Authors: Guilleminault Christian , Mondini Susanna , Hayes Boyd 
Published: January 2005

AbstractWe measured serially the blood pressure of 7 normal controls and 11 Type I insulin‐dependent and 6 Type II non‐insulin‐dependent diabetic patients during sleep. All subjects underwent 2 or 3 nights of monitoring during which blood pressure was measured with an arteriosonde, a Doppler‐type system with a cuff that automatically inflates every 15 minutes. All 7 normal controls, 3 of 11 Type I, and all 6 Type II diabetic patients had normal results from bedside tests of autonomic function. The Type I patients had abnormal systolic blood pressure measurements throughout the night. Unlike normal controls and Type II diabetics, their blood pressure did not rise progressively in the early morning hours. During rapid eye movement (REM) sleep, their blood pressure measurements were significantly higher and lower than values measured during non‐REM sleep, in sharp contrast to those in the other two groups, whose REM sleep–related blood pressure values were slightly above the preceding non‐REM sleep measurements.
Joseph's disease: Clinical and pathological studies in a Japanese family
Authors: Yuasa Tatsuhiko , Ohama Eisaku , Harayama Hiromi , Yamada Mitsunori , Kawase Yasuhiro , Wakabayashi Masatoshi , Atsumi Tetsushi , Miyatake Tadashi 
Published: January 2005

AbstractJoseph's disease is a hereditary ataxia found among descendants of Portuguese from the Azores Islands. We describe the clinical and pathological features of 4 members of a Japanese family who were diagnosed as having Joseph's disease. The illness began with cerebellar ataxia between the ages of 18 and 45 years. Nystagmus, dysarthria, and pyramidal signs were early manifestations. External ophthalmoplegia, dystonia and/or athetotic movements, and muscular atrophy appeared in the late stages. Neuropathological findings in one patient revealed degeneration of the dentatorubral and pallidoluysian systems, substantia nigra, pontocerebellar system, Clarke's column and spinocerebellar tracts, and anterior horn cells, as well as the cranial nuclei in the brainstem. Neurons in the inferior olivary nuclei, Purkinje's and granule cells, the cerebral cortex, thalamus, and striatum were spared. Involvement of the dentatorubral and pallidoluysian systems seems to be a characteristic feature of this disease in Japan.
Normal myelination of regenerating peripheral nerve sprouts despite circulating antibodies to galactocerebroside in rabbits
Authors: Stoll Guido , Reiners Karlheinz , Schwendemann Günther , Heininger Kurt , Toyka Klaus V. 
Published: January 2005

AbstractRabbits were immunized with galactocerebroside and a crush lesion was created in the tibial nerve before the onset of experimental allergic neuritis. Normal regeneration and myelination of distal peripheral nerve sprouts occurred and was identical to that of controls, although circulating antigalactocerebroside antibodies were present and nerve roots showed typical signs of beginning experimental allergic neuritis.
Abnormal platelet glutamate dehydrogenase activity and activation in dominant and nondominant olivopontocerebellar atrophy
Authors: Sorbi Sandro , Tonini Stefano , Giannini Emiliana , Piacentini Silvia , Marini Paolo , Amaducci Luigi 
Published: January 2005

AbstractGlutamate dehydrogenase (GDH) activity and its allosteric modulation by purine nucleotides were studied in platelet preparations from 4 patients with a nondominant form of adult‐onset olivopontocerebellar atrophy (OPCA) and in affected and nonaffected members of two families with a dominant form of OPCA. A partial deficiency of GDH activity (40 to 50% of control values) was present in 3 patients with nondominant OPCA and in 2 patients, father and son, with a dominant form of OPCA. Platelet GDH from these patients and controls was regularly inactivated by 2 mM guanosine‐5′‐triphosphate (GTP) and simulated one‐ to twofold by 2 mM adenosine‐5′‐diphosphate (ADP). In the presence of 0.2% Triton X‐100, the activating effect of ADP was enhanced four‐ to sixfold. The partial deficiency in maximum catalytic activity observed in these patients persisted under all conditions used for enzyme assay. In affected members, but not in one unaffected member of another family with a dominant type of OPCA, GDH activity was in the control range but was not activated by ADP in either the presence or absence of Triton. These results suggest that there may be at least two possible alterations of GDH in patients with OPCA: one which decreases the maximum catalytic activity and one which impairs the regulatory properties of the enzyme. Furthermore, this study suggests that platelet GDH determination in patients with OPCA may provide a simple and useful tool to classify these disorders and to understand the basic pathophysiological mechanisms involved.
Blink‐induced saccadic oscillations
Authors: Hain T. C. , Zee D. S. , Mordes M. 
Published: January 2005

AbstractA patient with a neurodegenerative disease had abnormal saccades only when he blinked. These saccades were hypermetric and were followed immediately, without any intersaccadic interval, by a large, oppositely directed saccade (dynamic overshoot). To explain these findings, we hypothesize that a blink‐related neural signal can modulate the activity of pause cells that normally inhibit saccadic burst neurons during fixation. In pathological circumstances, abnormal function of pause cells could lead to large‐amplitude saccadic oscillations. In normal subjects, blinks could induce short bursts of low‐amplitude flutter.
Benign versive or circling epilepsy with bilateral 3‐cps spike‐and‐wave discharges in late childhood
Authors: Gastaut Henri , Aguglia Umberto , Tinuper Paolo 
Published: January 2005

AbstractTwenty‐eight of 920 patients seen between the ages of 8 and 20 years for a first seizure manifested versive or circling events associated with bilateral rhythmic 3‐cps spike‐and‐wave discharges. In 21 (75%), the seizures appeared between the ages of 10 and 14 years. Paroxysmal adversion or gyration was always toward the same side in a given patient. “Break of contact” during the ictus was found in 70% of the seizures, and secondary generalization occurred in half. Ictal electroencephalographic recordings failed to show a focal hemispheric origin. Additional features were: absence of prior major diseases; normal results of neurological and neuroradiological examinations; excellent response to anticonvulsant treatment with sodium valproate and/or phenobarbital; frequent family history of epileptic seizures (25% of patients); and association with generalized seizures (57.2% of patients). We conclude that versive or circling epilepsy with bilateral rhythmic 3‐cps spike‐and‐wave discharges represents a benign form of primary generalized epilepsy in late childhood.
What determines the latency and amplitude of stationary peaks in far‐field recordings?
Authors: Kimura Jun , Kimura Akio , Ishida Tetsuro , Kudo Yutaka , Suzuki Shoji , Machida Masafumi , Matsuoka Hiroo , Yamada Thoru 
Published: January 2005

AbstractIn 20 radial nerves from 10 healthy persons, a referential derivation from the tip of the first or second digit registered two biphasic stationary peaks, PI‐NI and PII‐NII, following stimulation of the nerve in the forearm. These two peaks occurred slightly before the arrival of the propagating impulse at the wrist and at the base of the digit, respectively. With stepwise reduction of stimulation from a maximal to a threshold intensity, the far‐field potential decreased in amplitude linearly with the near‐field potential recorded at the junction of the volume conductor. Abduction of the first digit or flexion of the second and third digits altered the waveform and, to a lesser degree, the latency of the farfield peaks. However, these changes appeared in an inconsistent manner, which we were unable to characterize. We conclude that in far‐field recording a stationary peak results at the border of the adjoining volume conductors in proportion to the magnitude of the propagating axonal volley approaching the boundary.