bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acute thallium poisoning: Toxicological and morphological studies of the nervous system
Authors: Davis Larry E. , Standefer James C. , Kornfeld Marchio , Abercrombie David M. , Butler Cooley 
Published: January 2005

AbstractNine days following ingestion of 5 to 10 gm of thallium nitrate, a young man died with severe cranial and peripheral neuropathy, anuria, and heart failure. Ultrastructural examination of nerves obtained on days 7 and 9 demonstrated axonal degeneration with secondary myelin loss. Axons were swollen and contained distended mitochondria and vacuoles. Thallium levels in more than twenty organs and body fluids ranged from below 1.0 to 178 μg/gm; concentrations in twenty areas of the nervous system ranged from 29 to 140 μg/gm. The highest brain levels of thallium were found in gray matter. In the thalamus, 87% of the thallium was present in cell sap. Tissue concentrations of thallium did not parallel those reported for potassium, suggesting that thallium distribution differs from potassium distribution in human beings.
Alzheimer disease: Evidence for selective loss of cholinergic neurons in the nucleus basalis
Authors: Whitehouse Peter J. , Price Donald L. , Clark Arthur W. , Coyle Joseph T. , DeLong Mahlon R. 
Published: January 2005

AbstractThe nucleus basalis of Meynert provides diffuse cholinergic input to the neocortex. When compared with an age‐ and sex‐matched control, the nucleus basalis from a patient with Alzheimer disease demonstrated substantial reduction of neurons. Loss of this neuronal population may represent an anatomical correlate of the well‐documented cholinergic derangement in Alzheimer disease.
Voluntary and visual control of the vestibuloocular reflex after cerebral hemidecortication
Authors: Sharpe James A. , Lo Alex W. 
Published: January 2005

AbstractHorizontal vestibuloocular reflex (VOR) function was studied in five patients after cerebral hemidecortication. In darkness, VOR gain contralateral to the decorticate hemisphere was slightly higher than ipsilateral gain. Voluntary modulation of the reflex by attempted fixation of imaginary targets in darkness increased this VOR asymmetry. Voluntary cancellation of the ipsilateral VOR was better than cancellation of the contralateral VOR. Voluntary enhancement of the contralateral VOR was better than enhancement of the ipsilateral VOR. Visual control of the reflex while viewing foveal targets further increased the VOR asymmetry. Defective visual modulation corresponded to paresis of ipsilateral smooth pursuit. Abnormal voluntary responses in darkness indicate that cerebral control of the reflex can be independent of the pursuit system. The hemispheres may use a corollary discharge of eye position in the orbit and a head velocity signal to direct smooth eye movements toward the perceived location of objects. One hemisphere regulates ipsilateral smooth eye movements that achieve voluntary and visual control of the VOR.
Emery‐dreifuss humeroperoneal muscular dystorphy: An X‐linked myopathy with unusual contractures and bradycardia
Authors: Hopkins Linton C. , Jackson Joseph A. , Elsas Louis J. 
Published: January 2005

AbstractClinical, electromyographic, and muscle biopsy findings in the two largest known families with emery‐Dreifuss humieroperoneal muscular dystorphy indicate that this is an X‐linked recessive muscle disease with stereotyped clinical manifestations but with variable pathological and electromyographic characteristics. Elbow contractures, involvement of humeral muscles, Hyporeflexia, and abnorman electorcardiograms are present in our patients. The disorder is associated with a potentially lethal cardiac arrhythmia that should be managed by pacemaker insertion. The skeletal muscle disease is slowly progressive and is usually not life threatening. Muscle biopsy commonly shows type I fiver atrophy. Electromyogrphy usually indicates myopathy, though the classic findings of myopathy may not be present in every muscle.
The lateral, third, and fourth ventricle choroid plexus of the dog: A structural and ultrastructural study
Authors: Gomez Daniel G. , Potts D. Gordon 
Published: January 2005

AbstractThe morphology of the choroid plexuses of the lateral, third, and fourth ventricles in dogs was studied by histological and ultrastructural techniques after in situ fixation. Most portions of the plexus in the lateral ventricle showed a parallel arrangement of capillaries, producing a fine corrugation of the overlying layer of epithelial cells. Relatively large amounts of connective tissue separated the capillaries from the epithelium. The fourth ventricle choroid plexus showed short capillary loops projecting into the ventricle and more intimately covered by epithelium. Smaller amounts of connective tissue separated the capillaries from the epithelium. The choroid plexus of the third ventricle showed characteristics seen in the plexuses of both the lateral and fourth ventricles. The total surface of the choroid plexuses in the dog averaged 10.7 cm2, of which 55% was fourth ventricle choroid plexus.
A family affected with intestinal polyposis and gliomas
Authors: Todd David W. , Christoferson Lee A. , Leech Richard W. , Rudolf Lou 
Published: January 2005

AbstractThe third affected family with Turcot syndrome is presented and the problems associated with the occurrence of familial aggregates of cancer are discussed. Turcot syndrome represents the unique and discrete occurrence of polyposis coli with glioblastoma multiforme, medulloblastoma, or both.
The effect of stimulus orientation on the visual evoked potential in multiple sclerosis
Authors: Camisa John , Mylin Leland H. , Bodis‐Wollner Ivan 
Published: January 2005

AbstractVisual evoked potential (VEP) latency was measured in 89 patients with multiple sclerosis (MS) using both a checkerboard stimulus and an alternating vertical grating. Thirty‐seven patients had abnormal VEP latencies to the check stimulus, but 63 were classified as abnormal when the grating stimulus was employed. In an additional 22 MS patients, each eye was tested with a checkerboard stimulus, a vertical grating, and a horizontal grating to determine if edge orientation of the stimuli was responsible for the difference between grating and check diagnostic yields. The resulting diagnostic differences could be explained by taking into account the sinusoidal components of these stimuli. After equating gratings and checks for the fundamental Fourier component, each eye of 15 of the MS patients was retested with gratings in three orientations: vertical, horizontal, and oblique. Nine of the 15 patients had an orientation‐dependent abnormality of VEP latency. The results suggest that MS causes an orientational imbalance in the human visual system and that VEP testing with stimuli in more than one orientation can increase the diagnostic yield in MS patients.
Systemic and pulmonary vascular pressures during generalized seizures in sheep
Authors: Bayne L. L. , Simon R. P. 
Published: January 2005

AbstractThe systemic vascular response to seizures in known but information on the hemodynamics of the pulmonary circulation is lacking, clouding an understanding of the pathophysiology of postical pulmonary edema. We investigated this problem using electrically induced single seizures and bicuculline‐induced status epilecticus in halothaneanesthetized sheep. Aortic, pulmonary arterial, and left atrial pressures were recorded throughout the seizure activity, and the responses of the systemic and pulmonary pressures were compared. All monitored pressures rose precipitously with the onset of seizures. In all types of seizure the pulmonary pressure elevation was brief, lasting only one‐third as long as did the systemic. Pulmonary microvascular pressures rose well above the level of plasma oncotic pressure. Pulmonary vascular pressures were elevated in proportion to the duration of the seizure stimulus, while the systemic response did not so vary. Cervical spinal cord transection abolished all systemic and pulmonary vascular response to seizures, demonstrating ultimate neural mediation.
Isolation of herpes simplex virus type 1 in recurrent (Mollaret) meningitis
Authors: Steel J. Griffith , Dix Richard D. , Baringer J. Richard 
Published: January 2005

AbstractHerpes simplex virus type 1 (HSV‐1) was isolated from the cerebrospinal fluid of a patient with benign recurrent aseptic meningitis (Mollaret meningitis). HSV‐1 may be one of several causes for this rare syndrome. The isolation of HSV‐1 from our patient serves to broaden the range of herpesvirus‐associated neurological disease.
Anterior decompression of the spine for metastatic epidural cord compression: A promising avenue of therapy?
Authors: Siegal Tali , Siegal Tzony , Robin Gordon , Lubetzki‐Korn Isabelle , Fuks Zvi 
Published: January 2005

AbstractMost metastatic epidural tumors arise in a vertebral body and invade the anterior epidural space. Therefore, it is logical to decompress the spine anteriorly and not by traditional laminectomy. Surgical decompression is indicated if relapse occurs after radiotherapy and further radiation cannot be administered, if there is neurological deterioration during radiotherapy, and when histological diagnosis of the primary tumor is lacking. This pilot study consists of eleven consecutive anterior decompressions of the spine performed in nine patients. In seven instances other treatment modalities had been exhausted, and in four patients a tissue diagnosis was lacking. Before operation eight of the patients were nonambulatory, four of them paraplegic. Following decompression all but one patient became ambulatory. At operation the main bulk of the compressing tumor was found anterior or anterolateral to the cord. Spine stabilization was done when stability was a problem. Wound infection in one patient was the only postoperative complication. The encouraging outcome of our management prompts us to suggest that anterior decompression of the spine should be considered more often in metastatic compression of the cord and cauda equina.
Sylvian aqueduct syndrome caused by unilateral midbrain lesion
Authors: Auerbach Sanford H. , De Piero Theslee J. , Romanul Flaviu 
Published: January 2005

AbstractA patient developed a syndrome of head tilt, limited upgaze, and convergence‐retractory nystagmus. Pathological examination revealed a single 0.6 cm diameter metastatic tumor nodule in the right tegmentum of the midbrain extending from the posterior commissure to a plane through the lower end of the lateral geniculate body. The unique features are the lesion's small size and the fact that it was unilateral.
Association between rise in serum sodium and central pontine myelinolysis
Authors: Norenberg Michael D. , Leslie Kevin O. , Robertson Andrew S. 
Published: January 2005

AbstractTwelve hyponatremic patients with central pontine myeliolysis (CPM) showed a rise in serum sodium levels 3 to 10 days (mean, 6) prior to the development of CPM. The increase exceeded 20 mEq/L within 1 to 3 days and was then sustained for an additional 3 to 5 days. In addition, 11 of the 12 CPM patients achieved a sodium value of 147 mEq/L or greater during the period of sodium elevation. The rise in sodium frequently coincided with administration of saline solutions, diuretics, steroids, and lactulose. In contrast, 9 hyponatremic patients who did not have CPM showed sodium increases that were significantly less of slower (or both) following treatment of hyponatremia. Our findings suggest that CPM may be caused by a too rapid or excessive rise in serum sodium from a hyponatremic baseline.
Primary cerebellopontine progressive multifocal leukoencephalopathy diagnosed premortem by cerebellar biopsy
Authors: Jones H. Royden , Hedley‐Whyte E. Tessa , Freidberg Stephen R. , Kelleher Joseph E. , Krolikowski John 
Published: January 2005

AbstractA subacute progressive cerebellar brainstem syndrome developed in a patient with systemic lupus erythematosus in remission. Cerebellar biopsy documented the diagnosis of progressive multifocal leukoencephalopathy (PML). Data from this patient and 10 others in the literature emphasize the need to consider this diagnosis when ataxia develops in any patient with underlying malignancy, chronic infection, or other disease that involves immunological incompetence. Although the ataxic form of PML is not of nosological relevance, early diagnosis may eventually hav therapeutic importance.
Glial outgrowth along spinal nerve roots in amyotrophic lateral sclerosis
Authors: Ghatak Nitya R. , Nochlin David 
Published: January 2005

AbstractFormation of glial bundles in the proximal portion of the ventral nerve roots is described in a 51‐year‐old patient with the sporadic form of amyotrophic lateral sclerosis (ALS). Although the bundles were relatively fewer, they were identical in morphology and distribution to those consistently found in Werdnig‐Hoffmann disease (WHD). The occurrence of glial bundles in ALS, albeit rare, indicates that this phenomenon is not a unique feature of WHD. Similar changes have been observed in several other unrelated conditions, always in association with degeneration of neurons or axons. Thus, outgrowth of astrocytes in the form of glial bundles should be considered a special type of astrocytic reaction at the interface of the central and peripheral nervous systems.
Inborn errors of metabolism
Authors: Kolodny Edwin H. , Cable William J. L. 
Published: January 2005

AbstractInborn errors of metabolism often cause neurological dysfunction. These disorders are most common in childhood, but adult‐onset forms with a different clinical presentation are encountered, examples being Pompe disease, Tay‐Sachs disease, metachromatic leukodystrophy, Gaucher disease, and Maroteaux‐Lamy disease. In the evaluation of a patient with a possible inborn error of metabolism, simple screening tests may aid in the diagnosis and provide direction for more comprehensive laboratory analysis. In most cases, diagnosis can be established without a brain biopsy through biochemical variants of inherited metabolic disorders are being recognized through wider application of screening tests, improved specificity of laboratory analysis, cell complementation experiments, and the identification of enzyme activator factors. Accurate diagnosis is important for medical management, determining prognosis, and genetic counseling.
Presidential address: Thirty years of progress and problems in clinical neurology
Authors: Rowland Lewis P. 
Published: January 2005

AbstractResearch has transformed clinical neurology in the past thirty years, but we are faced with a paradox. Advances in basic sciences and better training for clinical investigators have accelerated the practical application of new knowledge. Now, just when neurological sciences are moving so rapidly, economic and other problems have threatened clinical investigation. It will take ingenuity and steadfast effort to continue research that is directly related to neurological diseases.
Neuroaxonal dystrophy in young adults: A clinicopathological study of two unrelated cases
Authors: Williamson Keith , Sima Anders, A. F. , Curry Bernadette , Ludwin Samuel K. 
Published: January 2005

AbstractThe clinical and pathological features of two unrelated sporadic cases of neuroaxonal dystrophy occurring in young adults are described. Initial clinical manifestations in both patients were those of primary psychiatric disorders. They subsequently developed extrapyramidal symptoms, dementia, cerebellar ataxia, and corticospinal dysfunction, but neither demonstrated myoclonic seizures. In one patient the diagnosis was made before death by brain biopsy. On pathological examination both patients showed a generalized distribution of spheroids within the central nervous system and, in one, in the peripheral nerves. In both patients, Lewy bodies were demonstrated in the pigmented brainstem nuclei. In addition, one patient showed ultrastructurally verified Lewy bodies in the cerebral cortex. Although these two cases could be considered examples of juvenile neuroaxonal dystrophy, we prefer to regard them as transitional forms in a disease spectrum with classic infantile neuroaxonal dystrophy and Hallervorden‐Spatz disease at the two extremes.
Regional cerebral blood flow and glucose metabolism following transient forebrain ischemia
Authors: Pulsinelli William A. , Levy David E. , Duffy Thomas E. 
Published: January 2005

AbstractProgressive brain damage after transient cerebral ischemia may be related to changes in postischemic cerebral blood flow and metabolism. Regional cerebral blood flow (rCBF) and cerebral glucose utilization (rCGU) were measured in adult rats prior to, during (only rCBF), and serially after transient forebrain ischemia. Animals were subjected to 30 minutes of forebrain ischemia by occluding both common carotid arteries 24 hours after cauterizing the vertebral arteries. Regional CBF was measured by the indicator‐fractionation technique using 4‐iodo‐[14C]‐antipyrine. Regional CGU was measured by the 2‐[14C]deoxyglucose method. The results were correlated with the distribution and progression of ischemic neuronal damage in animals subjected to an identical ischemic insult. Cerebral blood flow to forebrain after 30 minutes of moderate to severe ischemia ( < 10% control CBF) was characterized by 5 to 15 minutes of hyperemia; rCBF then fell below normal and remained low for as long as 24 hours. Postischemic glucose utilization in the forebrain, except in the hippocampus, was depressed below control values at 1 hour and either remained low (neocortex, striatum) or gradually rose to normal (white matter) by 48 hours. In the hippocampus, glucose utilization equaled the control value at 1 hour and fell below control between 24 and 48 hours. The appearance of moderate to severe morphological damage in striatum and hippocampus coincided with a late rise of rCBF above normal and with a fall of rCGU; the late depression of rCGU was usually preceded by a period during which metabolism was increased relative to adjacent tissue. Further refinement of these studies may help identify salvageable brain after ischemia and define ways to manipulate CBF and metabolism in the treatment of stroke.
Periodic paralysis and the sodium‐potassium pump
Authors: Layzer Robert B. 
Published: January 2005

AbstractAnalysis of the pathophysiology of hypokalemic paralysis, as it occurs in barium poisoning, chronic potassium deficiency, and thyrotoxicosis, suggests that these disorders may have a similar mechanism. An increased ratio of muscle sodium permeability to potassium permeability reduces the ionic diffusion potential, while the resting membrane potential is sustained by an increase of Na‐K pump electrogenesis. The result is that potassium entry (the sum of active and passive influx) exceeds potassium efflux; this causes a large shift of extracellular potassium into muscle until the Na‐K pump turns off, leading to depolarization and paralysis. The primary defect in familial hypokalemic periodic paralysis, as in the example of barium poisoning, may be a marked reduction of muscle permeability to potassium.
Encephalomyeloneuropathy with ganglionitis of the myenteric plexuses in the absence of cancer
Authors: Horoupian Dikran S. , Kim Yehum 
Published: January 2005

AbstractA 55‐year‐old woman presented with rapidly progressive brainstem dysfunction which led to death within a month. She also had constipation for three weeks, and barium enema showed ileus. Subacute encephalomyelitis predominantly involving the medulla and pons correlated with the patient's initial symptoms. In addition, ganglionitis of the myenteric plexuses explained the constipation and ileus. Ganglioradiculoneuropathy was another finding. The presence of abundant neuronophagia in the brainstem, dorsal root ganglia, and myenteric plexuses‐raised the speculation that a putative virus, toxic agent, or immune reaction possessed special affinity for neurons and ganglion cells. The neuropathological findings were similar to paraneoplastic changes, but no neoplasm was found.
A variant form of metachromatic leukodystrophy without arylsulfatase deficiency
Authors: Hahn Angelika F. , Gordon Bruce A. , Feleki Vera , Hinton George G. , Gilbert Joseph J. 
Published: January 2005

AbstractThe clinical, pathological, and biochemical findings in a young woman with a new variant of metachromatic leukodystropy (MLD) are reported. The patient showed slow early development and deteriorated further during her first two decades. Nerve conductions were slow, and a sural nerve biopsy showed features of a sulfatide lipidosis. Urinary sulfatide excretion was comparable to that of patients with classic MLD, yet in vitro activity of arylsulfatase A and B and cerebroside sulfatase activity were normal. Skin fibroblasts cultured in medium supplemented with 3H‐labeled sulfatide showed accumulation of labeled sulfatide in large amounts, implying a defect in sulfatide hydrolysis in vivo in spite of intact enzyme activity in vitro.
The role of the limbic system in experiential phenomena of temporal lobe epilepsy
Authors: Gloor Pierre , Olivier André , Quesney Luis F. , Andermann Frederick , Horowitz Sandra 
Published: January 2005

AbstractExperiential phenomena occurring in spontaneous seizures or evoked by brain stimulation were reported by 18 of 29 patients with medically intractable temporal lobe epilepsy who were investigated with chronic, stereotaxically implanted intracerebral electrodes. The phenomena mainly consisted of perceptual (visual or auditory) hallucinations or illusions, memory flashbacks, illusions of familiarity, forced thinking, or emotions. Experiential phenomena did not occur unless a seizure discharge or electrical stimulation involved limbic structures. For such phenomena to occur, seizure discharge or electrical stimulation did not have to implicate temporal neocortex. This was true even for perceptual experiential phenomena. Many experiential responses elicited by electrical stimulation, particularly when applied to the amygdala, were not associated with electrical afterdischarge. Limbic activation by seizure discharge or electrical stimulation may add an affective dimension to perceptual and mnemonic data processed by the temporal neocortex, which may be required for endowing them with experiential immediacy.
Treatment of neuroectodermal brain tumors
Authors: Shapiro William R. 
Published: January 2005

AbstractProspective clinical trials support the view that malignant neuroectodermal tumors should be treated vigorously using a multimodal approach that includes surgical resection, high‐dose radiation therapy, and prolonged maintenance chemotherapy. Less malignant astrocytomas and oligodendrogliomas should be treated by resection and irradiation. Laboratory research on neuroectodermal tumors has advanced rapidly, providing new data on tumor cell biology and the pharmacology of chemotherapy that directly influence the therapeutic options.