bluuebunny/crossref_metadata_2025_split huggingface dataset:
Electrophysiology of duchenne dystrophy myotubes in tissue culture
Authors: Rothman Steven M. , Bischoff Richard 
Published: January 2005

AbstractSingle myotubes from cultures of 6 patients with Duchenne muscular dystrophy and 6 patients whose biopsy specimens lacked histological abnormality were studied with intracellular physiological recording. Average resting membrane potentials were 47.8 ± 2.3 mV and 47.0 ± 1.5 mV for the control and Duchenne cultures, respectively. Action potentials elicited at a standard resting potential of −80 mV were 97.3 ± 4.0 mV and 98.6 ± 8.2 mV for the two groups. Maximum rate of rise of action potentials was 154.2 ± 25.2 V/sec for control and 143.1 ± 37.1 V/sec for Duchenne myotubes. Action potentials mediated by an influx of calcium ions were seen only when the myotubes from both groups were bathed in high concentrations of calcium and 4‐aminopyridine (20 mM and 1 mM, respectively). Thus, the plasma membrane of Duchenne dystrophy myotubes does not have active electrical properties that differ from those in controls. Previous reports of low resting membrane potentials in biopsies studied acutely may reflect secondary changes in degenerating fibers.
Myoclonic ocular jerks
Authors: Selhorst John B. , Ochs Alfred L. , Selbst Richard G. 
Published: January 2005

AbstractA 39‐year‐old woman with a familial trait for myoclonic jerks of the neck presented with spontaneous 5‐ to 40‐degree single jerks of the eyes, usually upward, each producing a brief interruption in fixation. Eye movement recordings showed the myoclonic ocular jerks to be saccades with variable intersaccadic intervals. Treatment with 6.0 mg per day of clonazepam reduced her symptoms.
Studies of human and monkey “Epileptic” neocortex in the in vitro slice preparation
Authors: Schwartzkroin Philip A. , Turner Dennis A. , Knowles W. Douglas , Wyler Allen R. 
Published: January 2005

AbstractThe in vitro slice technique was used to study neuronal activity in human cortical tissue removed during neurosurgical Procedures for intractable epilepsy and in monkey neocortex rendered epileptogenic by injection of alumina gel. In both cases, biopsies were guided by electrocorticographic signs of epileptiform activity. Intracellular recordings were made from 167 neurons in human tissue and from 73 neurons in monkey tissue samples. There was little spontaneous activity in these biopsies and no indication of spontaneous cellular bursting. Stimulation at the pial surface or in white matter evoked synaptically driven activity that was primarily excitatory. Graded bursts of activity could be elicited from some cells, but no all‐or‐none paroxysmal depolarization shifts were recorded. Inhibitory postsynaptic potentials were relatively rare. Intracellular injections of dye in a small number of neurons revealed no obvious differences between bursting and nonbursting neurons. These in vitro studies of chronic epileptic cortex have thus far provided few clear insights into the basic mechanisms of epilepsy.
Abnormal unloading reflex in a patient with infarction of the medial lemniscus
Authors: Angel Ronald W. , Goldstein Mark 
Published: January 2005

AbstractThe unloading reflex was measured in the intrinsic muscles of the hand. By removing various fractions of the load on the muscles, we determined the size of the electromyographic response as a function of the change in the external force. This technique was applied to both hands of a patient with a pontine lesion who had impaired position sense in the left hand. The response to fractional unloading was significantly smaller in the left hand, an asymmetry not found in control subjects. The results suggest (1) that the unloading reflex is mediated, at least in part, by suprasegmental pathways, and (2) that the fractional unloading technique can reveal abnormalities not found by conventional methods.
Oral physostigmine and lecithin improve memory in Alzheimer disease
Authors: Thal L. J. , Fuld P. A. , Masur D. M. , Sharpless N. S. 
Published: January 2005

AbstractEight patients with early Alzheimer disease were treated with gradually increasing multiple daily doses of oral physostigmine and supplemental lecithin. Six individuals showed improvement in total recall and retrieva storage (LTR), with a decrease in intrusions (a measure of inaccurate recall). The optimal individual dose was either 2.0 or 2.5 mg of physostigmine for each responding patient. Results of this open trial were subsequently replicated during a double‐blind crossover trial comparing physostigmine treatment to placebo. All six patients again demonstrated improvement in total recall and LTR, with a decrease in intrusions. The decrease in intrusions was strongly correlated with increasing inhibition of cholinesterase activity in cerebrospinal fluid, suggesting that the degree of improvement in the patient's memory was related to the amount of physostigmine that reached the brain. Other improvement and metabolites in cerebrospinal fluid were unaffected by the physostigmine therapy, suggesting a specific effect of physostigmine on the cholinergic system. The results suggest that small oral doses of physostigmin combined with lecithin ingestion have therapeutic benefit for some patients with Alzheimer disease.
Myasthenia gravis in children: Long‐term follow‐up
Authors: Rodriguez Moses , Gomez Manuel R. , Howard Frank M. , Taylor William F. 
Published: January 2005

AbstractWe report observations made on 149 patients with juvenile myasthenia gravis studied from onset of disease for as long as 40 years. Median follow‐up was 17 years; minimum was 4 years. Eight other patients with congenital myasthenia gravis were studied separately. Of the juvenile myasthenic patients, 85 (57%) underwent thymectomy because of disease severity. In juvenile myasthenia gravis, a spontaneous remission rate of 22.4 per 1,000 person‐year was observed, regardless of disease duration. A remission rate of 260 per 1,000 person‐years was seen during the first year after thymectomy, with a rate of 95 per 1,000 person‐years during the next 2 years. Early surgery, presence of bulbar symptoms, absence of ocular signs or generalized symptoms, onset of symptoms between ages 12 and 16, and presence of other immune disease were associated with increased postoperative remission rates. Epilepsy (4 patients) and neoplasia (7 patients) were the most frequent associated nonimmune disorders; rheumatoid arthritis (5 patients) and neoplasia diabetes mellitus (3 patients), asthma (3 patients), and thyroid disease (3 patients) were the most frequent associated immune diseases.
Roles of echocardiography and arrhythmia monitoring in the evaluation of patient with suspected systemic embolism
Authors: Come Patricia C. , Riley Marilyn F. , Bivas Natalie K. 
Published: January 2005

AbstractPatients with suspected systemic embolism were evaluated for possible cardiac origin of emboli using echocardiography (280 patients) and prolonged arrhythmia (Holter) monitoring (150 patients). Echocardiography demonstrated cardiac abnormalities that might predispose to emboli in 47% of patients with and 14% of patients without clinically evident cardiovascular disease. Lesions that might be directly responsible for emboli, including thrombi, myxomas, and vegetations, were identified in only 11 patients (4%), all of whom had clinically evident disease. Patients less than 45 years of age with clinically evident cardiovascular disease were especially likely to have a potential source of embolism identified.Holter monitoring demonstrated atrial fibrillation in 15 patients. All 15, however, had known atrial fibrillation in the past or had atrial fibrillation detected on admission electrocardiogram, or both.The findings suggest that it is not cost effective to perform echocardiography and arrhythmia monitoring on all patients with suspected systemic emboli. Because echocardiographic identification of a likely responsible lesion was limited to those with clinically evident cardiovascular disease, a policy of performing this test only on such patients would have eliminated 38% of the echocardiographic studies in this series.
Computed tomographic scan correlates of auditory comprehension deficits in aphasia: A prospective recovery study
Authors: Selnes Ola A. , Knopman David S. , Niccum Nancy , Rubens Alan B. , Larson David 
Published: January 2005

AbstractLesion localization and volume as assessed on computed tomography were analyzed to determine their relationships to auditory comprehension deficits following left hemisphere ischemic stroke in 39 patients. Auditory comprehension was initially tested approximately four weeks after onset, and then monthly for five months, using the Token Test, a series of commands of increasing complexity. Computed tomographic scans obtained five months after onset were used for lesion localization and volume determination. Lesions in the posterior superior temporal and infrasyvian suprmarginal regions were strongly associated with poor outcome. Patients with initially preserved auditory comprehension tended to have lesions that spared these regions, whereas patients with persistent deficits had lesions that included these regions. Six of 8 patients who initially had severe deficits in auditory comprehension but recovered completely lacked lesions in these regions. Lesion volume except when very large or very small was not closely associated with outcome.
Age dependent susceptibility to reovirus type 3 encephalitis: Role of viral and host factors
Authors: Tardieu Marc , Powers M. Linda , Weiner Howard L. 
Published: January 2005

AbstractReovirus type 3 inoculated intracerebrally or subcutaneously into newborn mice induced an acute necrotizing and uniformly fatal encephalitis. Subsequently, between the eighth and tenth day of age, reovirus type 3 infection changed to a nonlethal infection. This pattern of virulence was directly correlated with the ability of the virus to replicate in the brain and histologically was accompanied by a gradual diminution in the intensity and extent of encephalitis such that histological abnormalities were absent in the brains of virus‐injected adult animals. Some animals injected at ages 10 to 18 days developed brainstem and diencephalic lesions, indicating a nonuniform resistance of neurons to viral infection with aging. Neither a changing immune response pattern nor a modification or loss of viral receptors appears to explain either the susceptibility of newborn or the resistance of adult animals to reovirus type 3 encephalitis. Splenic mononuclear cells from mice younger (but not older) than 7 days also permitted reovirus type 3 replication in vitro. Thus, the age dependent resistance to reovirus type 3 encephalitis appears related to an intrinsic resistance to viral replication by neuronal cells, a resistance that may occur simultaneously in nonneuronal cell populations.
A sibship with neuroaxonal dystrophy and renal tubular acidosis: A new syndrome?
Authors: Maccario Micheline , Mena Hernando , Weir Michael R. , Matson Michael D. , Reimann Bernhard E. F. 
Published: January 2005

AbstractThe clinical and pathological features of three siblings from nonconsanguineous parents are described. Two of the children died at 61/2 and 7 months of age of a central nervous system disorder characterized by severe behavioral retardation, hypotonia, and pyramidal tract signs. The third child, now 8 years old, has global mental retardation, inability to talk, marked tremors, and gait disturbances. All children exhibited inability to concentrate or acidity the urine, with growth delay partially corrected in the living child by alkali therapy. Computed tomographic scans revealed hyperdense lesions in the thalamus of one of the siblings and more striking hyperdensity of the basal ganglia in the eldest child.Findings at postmortem examination in two children, and by renal and sural nerve biopsy in the third, include swollen axons in the internal capsule and peripheral nerves, and neurons with iron‐staining deposits, gliosis, and macrophages containing lipofuscin pigments in the brain. The renal findings include material positive for periodic acid–Schiff and hyperdense granular deposits in renal tubules.
Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease
Authors: Salazar Andres M. , Masters Colin L. , Gajdusek D. Carleton , Gibbs Clarence J. 
Published: January 2005

AbstractA review of over 2,000 cases of Creutzfeldt‐Jakob disease and related disorders in the literature and our own files yielded 231 cases of dementia with early lower motor neuron signs. The clinical‐pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt‐Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.
Multiple malignant astrocytomas in a patient with spontaneous progressive multifocal leukoencephalopathy
Authors: Sima Anders A. F. , Finkelstein Sydney D. , McLachlan Donald R. 
Published: January 2005

AbstractA 67‐year‐old man with a four‐year history of horizontal and vertical nystagmus and progressive cerebellar dysfunction later developed profound dementia and died of bronchopneumonia. Neuropathological examination revealed numerous active and inactive progressive multifocal leukoencephalopathy (PML) lesions in the cerebrum and cerebellum. Active lesions showed marked perivascular lymphocytic cuffing. Papovaviruses were identified ultrastructurally in both oligodendrocytes and astrocytes. Multiple malignant astrocytomas were documented, originating from PML lesions. No viruses could be demonstrated in neoplastic astrocytes. An immune‐deficient state was not revealed clinically or at postmortem examination.
Gerstmann‐Sträussler‐Scheinker's disease
Authors: Kuzuhara Shigeki , Kanazawa Ichiro , Sasaki Hidenao , Nakanishi Takao , Shimamura Kayako 
Published: January 2005

AbstractFindings are reported in three members of a Japanese family with a chronic familial disease characterized by signs of marked cerebellar dysfunction, mild pyramidal and extrapyramidal dysfunction, and loss or decrease of the kness and ankle jerks. Although the clinical features suggested olivopontocerebellar atrophy, postmortem study of one patient with obvious dementia revealed massive multiform plaques of Kuru type as well as multicentric, senile, and primitive types throughout the central nervous system, most prominent in the cerebellar and cerebral cortics and caudate nucleus. There was degeneration of the spinocerebellar and pyramidal tracts, posterior columns, superior cerebellar peduncles, cerebellar cortex, dentate nucleus, and vestibular nuclei as well as gliosis of the inferior colliculus and cerebellar foliar white matter. There were no cerebral spongiform changes, although slight spongy alteration without glial reaction was present. The clinical and neuropathological characteristics were consistent with those reported as Gerstmann‐Sträussler‐Scheinker's disease in an Austrian family.
Functional recovery of cortical neurons as related to degree and duration of ischemia
Authors: Heiss W.‐D. , Rosner G. 
Published: January 2005

AbstractImultaneous recordings of spontaneous single cell activity and local cerebral blood flow were obtained from 72 cortical neurons and adjacent brain in 54 cats before, during, and after ischemia induced by reversible occulsion of the middle cerebral artery. In most cells spontaneous electrical activity ceased at flow values of about 0.18 ml/gmm/in (range, 0.06 to 0.22 ml/gm/min). No signal was obtained from 28 neurons during reperfusion following ischemia of varying degree and duration. Overall, neurons exposed to a residual flow of 0.14 ml/gm/min or less for more than 45 minutes had a poorer prognosis compared to any other combination of degree and duration of ischemia. A discriminant curve was estimated to define the border line between recovering and nonrecovering cells. Regions showing irreversible neuronal failure contained selective neuronal necrosis or areas of infarction by histological examination. Reperfusion restored neuronal function in 44 cells. In this group of neurons, there was a joint interaction of duration of ischemia, ischemic residual flow, and recovery time: cells exposed to moderate ischemia (0.09 to 0.22 ml/gm/min) for up to 20 minutes recovered rapidly; most neurons subjected either to extreme ischemia (less than 0.09 ml/gm/min) of short duration (less than 20 minutes) or to moderate ischemia (0.09 to 0.22 ml/gm/min) for longer periods (20 to 141 minutes) required from 19 to 50 minutes for recovery. A few resistant neurons tolerated less than 0.09 ml/gm/min for more than 20 minutes.Cell survival and functional recovery following ischemia depend not only on separate effects of residual flow and duration of ischemia but on their combined influence. Selective vulnerability forms the natural basis of scattered cell survival.
Tissue acid‐base balance and oxygen metabolism in human cerebral infarction studied with positron emission tomography
Authors: Syrota A. , Castaing M. , Rougemont D. , Berridge M. , Baron J. C. , Bousser M. G. , Pocidalo J. J. 
Published: January 2005

AbstractNine patients who had suffered strokes were examined between 10 and 34 days after onset using positron emission tomography. DMO labeled with carbon 11 was used to evaluate brain acid‐base balance, and the oxygen‐15 inhalation technique was used to measure regional cerebral blood flow, the oxygen extraction fraction, and cerebral metabolic rate for oxygen. {11C}DMO concentration and oxygen metabolism variables were measured in the infarcted area and in the symmetrical region in the contralateral cerebral hemisphere. {11C}DMO concentration was found to be unchanged or slightly increased in five cases and markedly increased in four cases. The apparent increase in tissue pH can be explained by the presence of a large extracellular fluid space with a pH nearly identical to that of brain plasma, or by an increase in intracellular pH, or by both phenomena. The change in {11C}DMO concentration in the infarcted area relative to that in the normal tissue was independent of the change in blood flow. Cerebral metabolic rate for oxygen was decreased in all cases. The increase in {11C}DMO concentration in the infarcted area was linearly correlated with the decrease in the oxygen extraction fraction in the same region; that is, it was correlated with the occurrence of perfusion in excess of metabolic demand. The overabundant local perfusion could play a role in the decreased H+ content.
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: Report of 35 cases
Authors: Hagberg Bengt , Aicardi Jean , Dias Karin , Ramos Ovidio 
Published: January 2005

AbstractThirty‐five patients, exclusively girls, from three countries had a uniform and striking progressive encephalopathy. After normal general and psychomotor development up to the age of 7 to 18 months, developmental stagnation occurred, followed by rapid deterioration of higher brain functions. Within one‐and‐a‐half years this deterioration led to severe dementia, autism, loss of purposeful use of the hands, jerky truncal ataxia, and acquired microcephaly. The destructive stage was followed by apparent stability lasting through decades. Additional insidious neurological abnormalities supervened, mainly spastic parapareses, vasomotor disturbances of the lower limbs, and epilepsy. Prior extensive laboratory investigations have not revealed the cause. The condition is similar to a virtually overlooked syndrome described by Rett in the German literature. The exclusive involvement of females, correlated with findings in family data analyses, suggests a dominant mutation on one X chromosome that results in affected girls and nonviable male hemizygous conceptuses.
Antibodies to cerebellar Purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma
Authors: Greenlee John E. , Brashear H. Robert 
Published: January 2005

AbstractSera from 2 patients with ovarian carcinoma and paraneoplastic cerebellar degeneration confirmed postmortem were reacted with frozen sections of human cerebellum and stained using indirect immunofluorescence methods. Both sera produced bright cytoplasmic staining of Purkinje cells and of neurons within deep cerebellar nuclei. Titration of these sera to end point revealed staining at final dilutions of 1:640 and 1:2, 560, respectively. Neither of these sera reacted with sections of human cerebrum, basal ganglia, spinal cord, peripheral nerve, lung, liver, kidney, or ovary. Staining of Purkinje cells was not obtained with sera from 34 normal, healthy controls, 5 patients with oat cell carcinoma of the lung, 6 patients with inflammatory central nervous system disorders, or 12 of 14 neurologically normal patients with ovarian carcinoma. Sera from 2 neurologically normal patients with ovarian carcinoma, however, produced staining of Purkinje cells and deep nuclei similar to that obtained with sera from patients with paraneoplastic cerebellar degeneration. The present study documents the presence of antibodies to Purkinje cells in patients with ovarian carcinoma and cerebellar degeneration and demonstrates that development of these antibodies may antedate the onset of clinically evident cerebellar degeneration.
Cauda equina syndrome secondary to long‐standing ankylosing spondylitis
Authors: Bartleson J. D. , Cohen M. D. , Harrington T. M. , Goldstein N. P. , Ginsburg W. W. 
Published: January 2005

AbstractFourteen patients with cauda equina syndrome secondary to long‐standing ankylosing spondylitis are described. The roughly symmetrical neurological deficits were very slowly progressive and began long after the onset of the spondylitis, usually well after the rheumatological symptoms had stopped. Eventually every patient had cutaneous sensory loss in the fifth lumbar and sacral dermatomes. All patients developed urinary sphincter disturbances of a lower motor neuron type. There was prominent loss of rectal sphincter tone, and all but 2 patients had bowel complaints, including incontinence and severe constipation. Seven patients had mild to moderate weakness in the lumbosacral myotomes. Seven patients had pain in the rectum or lower limbs. Electromyographic abnormalities were consistent with multiple lumbosacral radiculopathies. Myelography and computed tomographic scanning of the lumbosacral spine showed characteristic enlargement of the caudal sac and dorsal arachnoid diverticula that had eroded the laminae and spinous processes. Recognition of this syndrome, coupled with computed tomographic scanning of the lower sponal canal, allows one to omit myelography, a procedure that is difficult because of the associated spine abnormalities. Surgical intervention should be avoided.