bluuebunny/crossref_metadata_2025_split huggingface dataset:
Thalamic degeneration in X‐chromosome–linked copper malabsorption
Authors: Iwata Makoto , Hirano Asao , French Joseph H. 
Published: January 2005

AbstractThalamic degeneration was present in 5 autopsied cases of X‐chromosome–linked copper malabsorption (X‐cLCM), Menkes' kinky hair disease. Among the thalamic nuclei, those in the formatio paraventricularis, intralamellaris, and extralamellaris were spared. The nuclei projecting to the granular cortices had severe neuronal depopulation. The thalamic nuclei that send axons to the agranular cortices were less often and less severely involved. The thalamic afferent system was intact except for degeneration of the red nucleus. Cerebral cortical lesions varied from case to case and usually were less marked than thalamic neuronal changes.
Hyperexcitability of motor and sensory neurons in neuromyotonia
Authors: Lance James W. , Burke David , Pollard John 
Published: January 2005

AbstractTwo members of a family with a neuropathy resembling Charcot‐Marie‐Tooth disease were unable to relax their muscles after voluntary contraction. Muscle spasm often outlasted voluntary contraction by 30 seconds or more before subsiding into myokymia and fasciculations. The posture of the hand during muscle spasm resembled that of tetany, and Trousseau's and Chvostek's signs were present although no abnormality of calcium or magnesium metabolism could be demonstrated. Muscle spasms ceased during medication with carbamazepine, 600 mg daily.Nerve stimulation, electromyography, and regional neuromuscular blockade with curare indicated that the condition originated in peripheral nerve, while microneurographic recordings showed that sensory as well as motor fibers were hyperexcitable. Sural nerve biopsy revealed axonal degeneration involving myelinated and unmyelinated fibers. It was concluded that the neural hyperexcitability is caused by a membrane defect resulting in a low threshold for excitation throughout the length of the axon.
Chronic lead intoxication: Effects on developing optic nerve
Authors: Tennekoon G. , Aitchison C. S. , Frangia J. , Price D. L. , Goldberg A. M. 
Published: January 2005

AbstractThe effects of chronic lead intoxication were studied by examining the optic nerves of mice given lead‐containing mother's milk from the day 1 to day 21 of life. Biochemical assays for myelin basic protein, 2′,3′‐cyclic nucleotide phosphodiesterase, and cerebroside sulfotransferase showed that the total amount of myelin produced by the lead‐exposed animals was decreased. Lead exposure did not alter the number of oligdendroglia or the relationship between axons and myelin sheaths. The hypomyelination was paralleled by a reduction in size of optic axons in the lead‐exposed animals. The data suggest that chronic exposure to lead in developing mice results in a primary effect on neurons and that hypomyelination is secondary to reduction in axon size.
Transmission of chronic spongiform encephalopathy with kuru plaques from humans to small rodents
Authors: Tateishi Jun , Ohta Michiya , Koga Makoto , Sato Yuji , Kuroiwa Yoshigoro 
Published: January 2005

AbstractAn unusual case of spongiform encephalopathy was transmitted directly from a human to rats and mice. After serial passages, incubation periods were shortened to about six months in rats and four months in mice. Clinical symptoms were similar in rats and mice, including ruffled fur, arched back, bradykinesia, and hind limb paralysis. Pathologically, a spongy state, proliferation of astrocytes, and neuronal changes were observed. Electron microscopic observation of the parietal cortex of rats and mice disclosed many membrane‐bound vacuoles in the neuropil, predominantly in dendrites. In the pons of mice, intramyelinic vacuoles and accumulation of extracellular fluid were prominent. The clinicopathological symptoms of the affected animals resembled those in other experimental spongiform encephalopathies, especially scrapie.
The natural history of peripheral neuropathy in primary systemic amyloidosis
Authors: Kelly John J. , Kyle Robert A. , O'Brien Peter C. , Dyck Peter J. 
Published: January 2005

AbstractThe records of 31 patients with primary systemic amyloidosis and peripheral neuropathy seen during a 17‐year period were analyzed to define the natural history of the neuropathy. Patients tended to be older men with a painful, distal, symmetrical sensorimotor neuropathy and prominent autonomic features. Loss of pain and temperature sensation was frequently more striking than loss of mechanoreception. Renal, cardiac, hematological, and gastrointestinal dysfunction often overshadowed the neuropathy. Clinical, neurophysiological, and histopathological studies pointed to axonal degeneration with predominant but not exclusive involvement of small myelinated and unmyelinated fibers. The neuropathy was progressive in all patients with or without treatment, but death was typically due to supervening medical complications.
Periodic lateralized epileptiform discharges in infants and children
Authors: PeBenito Rhandy , Cracco Joan B. 
Published: January 2005

AbstractClinical and electroencephalographic data on 7 infants and children with periodic lateralized epileptiform discharges (PLEDS) in their electroencephalograms were reviewed. The waveform, periodicity, and transitory nature of these discharges were similar to those reported in adults. In children, as in adults, PLEDs reflect severe underlying brain dysfunction which is often associated with metabolic and structural abnormalities. In adults, PLEDs are usually associated with altered states of consciousness and with acute unilateral cerebral lesions; in children, however, they often occur with little or no alteration in consciousness and with chronic, diffuse lesions of the central nervous system.
The effect of metrizamide on the electroencephalogram: A prospective study in 61 patients
Authors: Ropper Allan H. , Chiappa Keith H. , Young Robert R. 
Published: January 2005

AbstractA prospective study was made of changes in the electroencephalogram after the use of metrizamide for myelography and posterior fossa cisternography in 61 patients (62 studies). The EEG changed in 21 patients (34%); 3 of these had had previously abnormal records, and the abnormalities were accentuated on the second recording. Eight developed paroxysmal activity, including spike and wave in 1. EEG changes appeared 24 to 48 hours after the use of metrizamide in 6 patients whose intervening EEGs were normal. The percentage of records that changed tended to increase as metrizamide was brought to more rostral levels. Symptoms following the use of metrizamide were reviewed in 58 patients. Although there were no seizures in the present series, seizures did occur with subsequent use of metrizamide in 3 other patients at our institution.Previous reports may have underestimated the frequency of EEG changes after metrizamide since these may require 24 to 48 hours to develop. The appearance of such activity suggests that the use of metrizamide in patients with known seizure disorders, the anatomical level to which metrizamide is brought during radiography, removal of contrast medium, and further investigation of premedication with anticonvulsants all require careful consideration when use of metrizamide is being contemplated.
Search for viral particles and virus‐specific products in idiopathic Parkinson disease brain material
Authors: Schwartz Jerome , Elizan Teresita S. 
Published: January 2005

AbstractVirological studies were performed on brain material from 9 patients with idiopathic Parkinson disease and 3 matched controls. Electron microscopy and indirect immunofluorescent study were carried out directly on autopsy material from substantia nigra, caudate nucleus, and hypothalamus and on primary tissue culture explants of these brain areas grown alone or in cocultivation with virus‐susceptible indicator cell lines. All studies were negative for the presence of viral particles, viral inclusions, or cellular changes suggestive of virus infection. All materials were negative for antigens of herpes simplex type 1, influenza A/NWS, and measles (Edmonston strain) viruses. Within the limits of these techniques, there is no evidence at this time that viruses are an important etiological agent in idiopathic Parkinson disease.
Seizures and related epileptiform activity in hippocampus transplanted to the anterior chamber of the eye: Modulation by cholinergic and adrenergic input
Authors: Freedman Robert , Taylor David A. , Seiger Ake , Olson Lars , Hoffer Barry J. 
Published: January 2005

AbstractTransplants of rat hippocampus into the anterior chamber of the eye of a host animal were used to assess the effects of cholinergic and adrenergic neuronal inputs on the generation and duration of seizure activity. Cholinomimetics initiated both seizures and hypersynchronous neuronal activity in the transplants. Cyclic guanosine monophosphate (GMP) derivatives and isobutyl methylxanthine elicited similar changes. Reflex activation of the cholinergic parasympathetic input to the iris and transplant by illumination of the ipsilateral retina also induced seizures or increased the rate of penicillin‐induced interictal spike discharge. Application of β‐adrenergic agonists inhibited interictal spikes and paroxysmal depolarizing shifts induced by penicillin. Fluorescence histochemical studies showed that host sympathetic adrenergic fibers derived from the ground plexus of the iris invaded the transplant to form fine varicose nerve terminals. Activation of these adrenergic afferents to the transplant diminished both the amplitude and frequency of penicillin‐induced epileptiform activity. Epileptiform activity in hippocampal occular transplants is strongly modulated by cholinergic and adrenergic neuronal inputs, with the former exerting a facilatory influence and the latter, an inhibitory effect.
Cockayne syndrome: Unusual neuropathological findings and review of the literature
Authors: Soffer Dov , Grotsky Harold W. , Rapin Isabelle , Suzuki Kinuko 
Published: January 2005

AbstractTwo siblings with Cockayne syndrome (CS) are described and the literature on the subject is briefly reviewed. Of particular interest were the unusual neuropathological findings in 1 of the patients. These included microcephaly, white matter atrophy with patchy loss of myelinated fibers, calcifications of the basal ganglia, occasional ferrugination of cerebral and cerebellar neurons, and severe cerebellar degeneration. Findings not previously reported in CS were proliferation of extremely bizarre astrocytes, neurofibrillary tangles, and pigmentation of the globus pallidus. We conclude that brain involvement in CS is a result of primary degeneration in the central nervous system rather than being secondary to angiopathy or normal pressure hydrocephalus, as previously suggested.
Bacteroides meningitis successfully treated with metronidazole
Authors: Peterson Donald I. , Voorhees Elwood G. , Elder Harvey A. 
Published: January 2005

AbstractBacteroides meningitis without associated cerebral abscess occurred in an elderly man who had a long history of chronic otitis media. Anaerobic cultures of the cerebrospinal fluid revealed bacteroides organisms. Sensitivity testing showed them to be resistant to penicillin and chloramphenicol but sensitive to metronidazole. Appropriate therapy with this agent eradicated the causative organisms and produced rapid clinical improvement.
Language deficits after apparent clinical recovery from childhood aphasia
Authors: Woods Bryan T. , Carey Susan 
Published: January 2005

AbstractTwenty‐seven children with childhood injury to the left hemisphere were tested for language function and compared with appropriate controls. Eleven children had incurred their lesions before the age of 1 year, 16 afterward. The group with perinatal injury to the left hemisphere did not show a specific aphasic deficit even though they were mildly cognitively imparired. The group of children with later injury to the left hemisphere showed aphasic deficits if the orginal injury had caused a language defect; otherwise the left hemisphere injury was not associated with specific disturbances in language function. The average age at time of lesion in those children who had recovered from aphasia was 4.7 years. We conclude that even when childhood aphasia results from a unilateral nonprogressive lesion, recovery of language is less complete than has been generally supposed.
Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (leigh disease)
Authors: Devivo Darryl C. , Haymond Morey W. , Obert Kathleen A. , Nelson James S. , Pagliara Anthony S. 
Published: January 2005

AbstractAutopsy examination confirmed the diagnosis of subacute necrotizing encephalomyelopathy (SNE) in a 7‐monthold male infant who underwent several metabolic studies before death. Intermittent lactic acidemia and fumaric aciduria, an extreme hyperglycemic response to an intravenous bolus of alanine, and an elevated total body flux rate of glucose (58.4 μmoles · kg−1 · min−1) suggested a disturbance in the oxidative decarboxylation of pyruvate. Enzymological studies of postmortem samples revealed low nonactivated pyruvate dehydrogenase activity in liver (19.4%) and brain (53.8%). The lowest brain pyruvate dehydrogenase activities were noted in the midbrain and pontine regions. Supramaximal activation of the hepatic pyruvate dehydrogenase complex (135% of control values) occurred in vitro. Spontaneous reactivation following in vitro inactivation of the complex with adenosine triphosphate was significantly less (p < 0.02) in the patient's samples compared to controls. The biochemical defect was not apparent in fibroblasts. These enzymological observations point to an in vivo defect in the activation mechanism of the pyruvate dehydrogenase complex as the biochemical disturbance in SNE. The findings suggest that dichloroacetate may be beneficial in treating SNE.
Cerebral infarcts with arterial occlusion in neonates
Authors: Barmada M. A. , Moossy J. , Shuman R. M. 
Published: January 2005

AbstractAmong 592 infants examined at autopsy during a four‐year period, 32 (5.4%) had cerebral infarcts. Excluded were cases of traumatic hemorrhages and softening, periventricular leukomalacia, venous lesions, and any mass, including encephaloceles, with arterial distortion and infarction. Histological abnormalities were similar to those of infarcts in adults. Relatively advanced histopathological changes in some infants living only a few hours indicated that some infactions may have occurred in utero. The most common cause of arterial occlusion was embolization, with sepsis and disseminated intravascular coagulation playing a major role. The brains of term neonates were more frequently involved than those of premature infants. Multiple small infarcts occurred more often in premature infants. In most cases autonomic dysfunction with prolonged apnea, episodic seizures, and metabolic acidosis were the major associated clinical features, rather than focal neurological deficits. Similar cerebral infarcts in infants who survive with less severe systemic complications may lead to porecephaly, hemiplegia, mental and motor retardation, and recurrent seizures.
Acute intoxication with sodium valproate
Authors: Chadwick D. W. , Cumming W. J. K. , Livingstone I. , Cartlidge N. E. F. 
Published: January 2005

AbstractTwo epileptic patients developed an acute toxic encephalopathy consisting of altered behavior, deteriorating seizure control, and confusion while taking sodium valproate alone. Serum valproate levels were greater than 100 μg/ml in both. Toxic symptoms resolved when the dose of valproate was reduced, with a consequent fall in serum concentration of the drug.
Immunoreactive myelin basic protein in the cerebrospinal fluid in neurological disorders
Authors: Whitaker John N. , Lisak Robert P. , Bashir Rifaat M. , Fitch Olive H. , Seyer Jerome M. , Krance Robert , Lawrence Jesse A. , Ch'ien Lawrence T. , O'Sullivan Patrick 
Published: January 2005

AbstractCerebrospinal fluid from 582 persons was analyzed by a double‐antibody radioimmunoassay for the presence of material cross‐reactive with peptide 43‐88 of human myelin basic protein (BP). In a group of 104 patients with multiple sclerosis (MS), 23 of 33 individuals clinically judged to have had an exacerbation within two weeks prior to the time CSF was obtained had detectable material ranging from 2 to 200 ng/ml. In the remaining 71 MS patients who either were stable or had had an exacerbation more than two weeks before, only 1 patient had a marginally elevated level of immunoreactive material. CSF from 53 persons with cerebrovascular disease was studied, and 13 of 29 with recent infarctions had values of 2 to 540 ng/ml. The degree of elevation in strokes generally paralleled the predicted volume of the lesions, but the amounts detected did not correlate quite so closely temporally with onset as they did with the periods of active disease in MS. Of the remaining 425 patients, 29 had immunoreactive material of 2 to 400 ng/ml in their CSF. Most of these patients with detectable material had acute diseases known to affect the myelin sheath. Eight of 10 persons with acute disseminated encephalomyelitis had no detectable material. The presence in CSF of material cross‐reactive with BP peptide 43‐88 does not have diagnostic specificit for MS but can be used as a means for determining recent myelin injury. The type of BP peptide formed and mechanisms for clearance of BP and BP peptides may be important in determining the biological consequences following release of this potentially immunogenic material from the central nervous system.
Undecussated innervation to the sternocleidomastoid muscle: A reinstatement
Authors: Balagura Saul , Katz Russel G. 
Published: January 2005

AbstractClinical evidence is presented to support the contention that the sternocleidomastoid muscle is innervated primarily by undecussated fibers from the ipsilateral hemisphere. Stroke patients often show contraleteral hemiparesis accompanied by weakness in head turning to the side of the paresis.
Rheumatoid disease with encephalopathy
Authors: Kim R. C. 
Published: January 2005

AbstractA 63‐year‐old woman developed progressive rheumatoid disease complicated by a confusional state as well as persistent cerebrospinal fluid pleocytosis and hypoglycorrhachia. Neuropathological examination revealed extensive rheumatoid lesions in the cranial dura, falx, and choroid plexus. The choroid plexus has not been affected in any of the 16 previously reported cases of pathologically verified rheumatoid disease of the central nervous system. The findings in this patient support the observations of other regarding the role of the choroid plexus in development of CNS manifestations in systemic immune complex disease.
Prolonged relief of tic douloureux from partial root destruction is associated with localized analgesia
Authors: Ecker Arthur 
Published: January 2005

AbstractFifty‐eight patients who had been relieved of tic douloureux (TD) for at least 3 years after partial destruction of the trigeminal sensory root by injection of minimal amounts of alcohol were reexamined 31/2 to 21 years after treatment. Of 41 patients still in remission at a median of 10 years after treatment, 32 had analgesia; i.e., sensation of pain from pinprick was absent in all 52 originally neuralgic areas. The other 9 patients in remission had some pain on pinprick in their neuralgic areas. In contrast, of 17 patients with recurrence of TD 31/2 to 11 years (median, 6 years) after treatment, at least 15 had demonstrable pain on pinprick in the neuralgic areas. It is concluded that continuing absence of pain on pinprick in the affected facial mucocutaneous area (or areas) is associated with prolonged relief of tic douloureux.
Isoprinosine treatment in 18 patients with subacute sclerosing panencephalitis: A controlled study
Authors: Haddad Fuad Sami , Risk Winthrop S. 
Published: January 2005

AbstractIsoprinosine (inosiplex), a proposed antiviral agent, was given to 18 patients with subacute sclerosing panencephalitis (SSPE). No great improvement occurred in any patient soon after treatment, but 2 patients did improve substantially several months after discontinuing Isoprinosine. Spontaneous improvement also occurred in 4 out of 96 controls. Because of the variable natural history of SSPE, differences in clinical improvement between treated patients and controls were not significant. Changes in serial measles antibody titers did not correlate with therapy.
Cerebellar calcification on computerized tomography
Authors: Koller William C. , Klawans Md Harold L. 
Published: January 2005

AbstractCerebellar calcification on CT scan was observed in five patients over a two‐year period. It was located bilaterally and symmetrically in the dentate nucleus in all 5 patients and in the cerebellar vermis in one. Calcifications of the basal ganglia and cerebral cortex were associated in two cases each. Skull radiography did not reveal the cerebellar calcifications, and serum calcium levels were normal in all patients. None had symptoms or signs of cerebellar dysfunction, and they had a variety of different clinical diagnoses. Cerebellar calcification may be a form of benign intracerebral calcification.
Disseminated vasculomyelinopathy: An immune complex disease
Authors: Reik Louis 
Published: January 2005

AbstractThe numerous nervous system abnormalities which follow antecedent infections and immunizations appear to share a common pathogenesis involving the immune system. Pathologically, a small vessel vasculopathy involving arterioles and capillaries as well as venules in both gray and white matter is the earliest and most consistent change. Perivascular demyelination appears to develop subsequently. Delayed hypersensitivity to myelin basic protein may not adequately account for these changes.Humoral immunity may be involved instead. I postulate that antigen‐antibody complexes, formed following the introduction of foreign antigen by infection or inoculation, cause vascular injury with secondary damage to myelin. There is considerable evidence that circulating immune complexes are present in some postinfectious nervous system disorders, as are associated systemic features which suggest immune complex disease. Similar clinical and pathological nervous system changes occur in a variety of disorders in which circulating immune complexes are thought to cause vascular injury.
Brainstem abscess and the syndrome of acute tegmental encephalitis
Authors: Harvey Frederik H. , Carlow Thomas J. 
Published: January 2005

AbstractA 63‐year‐old man developed the acute onset and rapid progression of signs of lower brainstem dysfunction accompanied by a sterile cerebrospinal fluid containing moderate pleocytosis. Autopsy examination disclosed diffuse acute bacterial inflammation of the brainstem tegmentum. The findings in 55 additional cases of brainstem bacterial infections are reviewed and discussed with respect to differential diagnosis and potential treatment.
PMNs in normal spinal fluid examined by the cytocentrifuge technique
Authors: Simon Roger P. , Koerper Marion A. 
Published: January 2005

AbstractThe presence in unconcentrated CSF specimens of even a single polymorphonuclear (PMN) cel has been regarded as diagnostic of central nervous system (CNS) disease. In 18 of 50 consecutive cytocentrifuged specimens obtained from patients without evidence of CNS disease, however, we found 1 to 6 PMN cells. The observation of occasional PMN cels in concentrated CSF specimens does not appear to be diagnostic of a CNS abnormality.