bluuebunny/crossref_metadata_2025_split huggingface dataset:
The ocular motor defects in progressive supranuclear palsy
Authors: Troost B. Todd , Daroff Robert B. 
Published: December 2004

AbstractThe results of quantitative infrared horizontal eye movement recordings in 8 patients with progressive supranuclear palsy are presented. Some of the patients had total paralysis of vertical movements, but none had completely lost the ability to perform horizontal eye movements.All patients had a defect in ocular fixation previously undescribed in this condition: the universal presence of square‐wave jerks. Analysis of refixation sacades demonstrated hypometria, slow velocity/amplitude relationships, and profound prolongation of duration. The pursuit abnormality, characterized clinically by “cogwheel” eye movements, represented the inability to match eye velocity to target velocity. The ratio of peak eye velocity to peak target velocity (pursuit gain) was 0.2 to 0.5. Defects in the vestibuloocular reflex included inability to increase the gain of the reflex (ratio of peak eye velocity to head velocity) during viewing of a visible, stationary target and failure to suppress the reflex when viewing a target rotating with the head.
WAIS performance in brain‐damaged left‐ and right‐handers
Authors: Todd John , Satz Paul 
Published: December 2004

AbstractWAIS performance was compared in 35 pairs of left‐ and right‐handed subjects who had been individually matched for lateralization, cause of brain damage, age, education, and gender. WAIS results were similar in the two groups as well as in two subgroups of subjects with unilateral brain damage. No differences between the unilateral groups were found to support the hypothesis advanced by Levy and Nagylaki in 1972 that left‐handers have inferior visual‐spatial abilities.
Phenytoin‐Related cerebellar degeneration without seizures
Authors: Rapport Richard L. , Shaw Cheng‐Mei 
Published: December 2004

AbstractCerebellar changes have been reported in relationship to epilepsy alone as well as to phenytoin therapy for the control of seizures. The cliniconeuropathological correlation between these changes and epilepsy or the anticonvulsant is usually complicated by the presence of both variables. Experimental evidence suggests that phenytoin alone may be sufficient to cause cerebellar changes following intoxication. We report a case of cerebellar degeneration in a patient treated with isoniazid and prophylactically treated with phenytoin who never had a seizure.
Persistent cerebellar ataxia after exposure to toluene
Authors: Boor John William , Hurtig Howard I. 
Published: December 2004

AbstractAn optometrist and a glue sniffer exposed independently to toluene vapors developed cerebellar, pyramidal, and cognitive dysfunction. All deficits cleared completely in the former but persisted in the latter. The findings confirm a previous report of a toluene‐induced neurological syndrome that is exclusively central, sparing peripheral nerves.
Congenital fiber type disproportion in identical twins
Authors: Curless Richard G. , Nelson Marilyn B. 
Published: December 2004

AbstractEighteen‐month‐old identical twins with the muscle histological characteristics of congenital fiber type disproportion are reported. The fiber typing system of pH‐dependent adenosine triphosphatase was used to analyze the size and percentage of type 1, 2A, and 2B fibers in muscle tissue obtained by needle biopsy. Both twins had significantly larger type 2 than type 1 fibers, with 2B fibers representing the largest type. One patient also had type 1 predominance at the expense of a reduction in 2B (2B deficiency). The probands as well as other family members had transient delayed motor development, macrocephaly, and normal intelligence. A biopsy obtained from 1 of these family members failed to demonstrate a similar histological abnormality. The disorder is nonprogressive in this family, as evidenced by minimal disability in the older members and gradual improvement in the probands.
Emotional sequelae of head injury
Authors: Dikmen Sureyya , Reitan Ralph M. 
Published: December 2004

AbstractThe emotional after‐effects of head injury in relation to acuteness versus chronicity of the injury and neuropsychological deficits were studied. The results indicate that there is a decline in emotional distress over time after the injury and show that patients with significant initial and residual neuropsychological deficits experience greater emotional distress than those with negligible difficulties.
Rabies: Recent advances in pathogenesis and control
Authors: Miller Aaron , Nathanson Neal 
Published: December 2004

AbstractCurrent knowledge of rabies is reviewed, with emphasis on recent developments in virology, immunology, pathogenesis, treatment, and prophylaxis. Although only a few cases of human rabies occur annually in the United States, the infection is enzootic in wildlife, and an estimated 30,000 possible exposures requiring treatment occur each year.Rabies belongs to the family rhabdovirus, and its molecular anatomy and biochemistry of replication have been described in some detail. There have been advances in measurement of antirabies antibodies, and techniques for measuring cellular immune response have recently been developed. Early stages of infection are more fully understood, with a hypothesis to explain peripheral sequestration of virus during prolonged incubation periods. Rabies was once thought to be uniformly fatal, but a few patients have survived with aggressive supportive measures. A newly developed vaccine (soon to be licensed in the United States) has been shown highly potent for preexposure immunization and promises to be very effective for postexposure prophylaxis. Current Public Health Service recommendations for postexposure treatment are summarized. Recent research has suggested a novel approach to control of wildlife rabies through oral immunization.
Calcification of axons in experimental spinal cord trauma
Authors: Balentine J. Douglas , Spector Myron 
Published: December 2004

AbstractSelective discrete intraaxoplasmic deposits of hydroxyapatite crystallites were observed in adult male rats subjected to experimental trauma to the lumbosacral spinal cord. Although previously unreported in spinal cord trauma, the presence of these deposits in minimally altered axons and during the early posttrauma period suggests that such selective calcification may be of more than secondary significance. In view of the current emphasis on cytotoxicity of calcium, especially in relation to axonal degeneration, an evaluation of the role of calcium in augmenting traumatic spinal cord necrosis is warranted.
Chronic neurogenic quadriceps amyotrophy
Authors: Furukawa Tetsuo , Akagami Noriko , Maruyama Shoichi 
Published: December 2004

AbstractTwo patients with chronic neurogenic quadriceps amyotrophy are reported. A 61‐year‐old woman had symmetrical wasting and weakness of the quadriceps femoris of eight years' duration. A biopsy revealed neuropathic changes, and electromyograms showed neurogenic as well as myopathic patterns in the quadriceps and other muscles. A 29‐year‐old woman had long‐standing wasting limited to the quadriceps and hip muscles. EMG and biopsy were compatible with neurogenic atrophy. She had a brother suffereing from a more widespread and severe form of the disease. These cases suggest that chronic neurogenic quadriceps amyotrophy is a forme fruste of Kugelberg‐Welander disease.
Visual evoked potentials and postmortem findings in a case of cortical blindness
Authors: Spehlmann Rainer , Gross Robert A. , Ho Sam U. , Leestma Jan E. , Norcross Karyl A. 
Published: December 2004

AbstractA patient with cortical blindness due to extensive bilateral posterior cerebral infarcts showed occipital visual evoked potentials to flash stimulation on repeated testing. These responses were probably mediated by extrageniculocalcarine connections between the optic nerve and the secondary visual cortex of the occipital convexity.
Neuropathological changes in cerebellar biopsies of epileptic patients
Authors: Salcman Michael , Defendini Richard , Correll James , Gilman Sid 
Published: December 2004

AbstractIn 5 patients with pharmacologically intractable epilepsy, biopsy of the cerebellar cortex at the time of surface electrode implantation for therapeutic stimulation demonstrated marked degeneration of the Purkinje cell layer in every case. Two of the patients had never experienced a generalized convulsion. Comparison of these biopsy findings with the autopsy findings in two other groups of patients with a seizure disorder of different duration and severity and with the findings in other reported series suggests that permanent neuronal damage in epilepsy is related to the cumulative effects of the frequency and chronicity of the disorder. The pathogenesis remains unclear. The histopathological changes are identical to those seen in generalized cerebral hypoxia, but the observations in our series support the view that in epilepsy the pathogenesis may not be hypoxic.
Epidural spinal cord compression from metastatic tumor: Diagnosis and treatment
Authors: Gilbert Robert W. , Kim Jae‐Ho , Posner Jerome B. 
Published: December 2004

AbstractThe clinical findings in 130 consecutive cases of spinal cord compression by metastatic extradural tumors were analyzed. These 130 patients were combined with a previous survey of 105 patients to compare the effectiveness of radiation therapy (RT) alone with that of surgical decompression followed by RT. Ambulation after treatment was considered a successful outcome. The most common primary tumors producing spinal cord compression were (in order) breast, lung, prostate, and kidney. In 68% of these tumors the thoracic region was involved. Pain was the primary symptom in 96% of the patients, while motor or sensory deficits (or both) were found in 82% of them. Therapy consisted of surgery and RT in 65 patients and RT alone in 170 patients. There were no differences in outcome between those treated by surgery combined with RT and those managed by RT alone. Patients with radiosensitive tumors and those ambulatory at the onset of treatment benefited whether treated by surgery or by RT. Seventy‐five percent of living patients who improved from treatment remained ambulatory at 6 months, and approximately 50% of living patients were ambulatory at 1 year. We conclude that RT without decompressive laminectomy is as effective as decompressive laminectomy in treating epidural spinal cord compression from systemic cancer.
Creatine kinase BB isoenzyme levels by radioimmunoassay in patients with neurological disease
Authors: Bell Rodney D. , Rosenberg Roger N. , Ting Ruby , Mukherjee Amal , Stone Marvin J. , Willerson James T. 
Published: December 2004

AbstractCreatine kinase BB (CK‐BB) isoenzyme was measured by radioimmunoassay in serum and in cerebrospinal fluid from 61 patients with various neurological disorders. Statistically significant elevations of CK‐BB isoenzyme in the serum were observed in patients with acute cerebrovascular accidents and in those with seizures and a prolonged alteration in level of consciousness. Statistically significant elevations of CK‐BB isoenzyme in the cerebrospinal fluid were also found in patients who had suffered acute cerebrovascular accidents. Some patients with central nervous system infections, acute demyelinating disease, certain drug overdoses, head trauma, and complex migraine also had elevations of serum and CSF CK‐BB isoenzyme. In 2 patients with elevations of CK‐BB isoenzyme, the elevations in serum occurred later than those detected in the cerebrospinal fluid.If CK‐BB elevations determined by radioimmunoassay can be demonstrated to be quantitatively related to the extent of brain damage, then these determinations in conjunction with experimental animal models and newer radiological techniques should allow evaluation, in an objective and precise manner, of measures designed to decrease that damage.
McArdle disease: The mystery of reappearing phosphorylase activity in muscle culture—A fetal isoenzyme
Authors: Dimauro Salvatore , Arnold Susan , Miranda Armand , Rowland Lewis P. 
Published: December 2004

AbstractTo understand the apparently paradoxical appearance of phosphorylase in muscle cultured from patients with McArdle disease, the enzyme in muscle culture was studied immunologically and electrophoretically. Antibody against normal human muscle phosphorylase completely inhibited the enzyme activity of adult muscle, but it had no effect on phosphorylase activity of muscle cultures from normal individuals or patients with McArdle disease. Also, amounts of antibody that would completely inhibit phosphorylase in mature muscle left about 30% of the activity in muscle obtained from human fetus at four months' gestation.Acrylamide‐disc and slab‐gel electrophoresis showed a single band of phosphorylase activity in adult muscle and two bands in fetal muscle. This suggested that at four months' gestation, both fetal and mature forms are present but that only the mature isoenzyme is inhibited by the antibody. The enzyme from cultured muscle gave only a single band, with the electrophoretic mobility of the fetal isoenzyme. These data suggest that phosphorylase activity in muscle cultured from patients with McArdle disease is due to a fetal isoenzyme whose genetic control is different from that of the mature enzyme.
Primary cerebral venous thrombosis in young adults: The diverse manifestations of an underrecognized disease
Authors: Averback P. 
Published: December 2004

AbstractPrimary cerebral venous thrombosis has a wide clinical and pathological spectrum, which has not been recognized. Seven cases of aseptic cerebral venous thrombosis (5 fatal) are discussed, with pathological study and literature review. Five patients ranged in age from 22 to 31 years. In 3 cases no discernible cause for thrombosis was found.Novel features of these cases include: primary bilateral internal cerebral venous thrombosis; isolated thrombocytopenia; occurrence in the context of (1) ulcerative colitis plus oral contraceptive use, (2) a twin transfusion syndrome, and (3) presenting syndrome five years after mastectomy for carcinoma. Diagnosis and treatment are discussed, and it is suggested that a focal coagulopathy may be responsible for this underdiagnosed condition.
Normal CAT scans in craniopharyngioma
Authors: Volpe Bruce T. , Foley Kathleen M. , Howieson John 
Published: December 2004

AbstractThree patients with symptoms of headache and vision loss had normal routine CAT scans, with and without injection of contrast medium, as their initial diagnostic procedure. Subsequent pneumoencephalography revealed large suprasellar masses, which at operation were found to be partially cystic craniopharyngiomas. The findings emphasize the existence of craniopharyngiomas that are isodense with brain and may not be detected on routine CAT scan. When a patient's clinical presentation suggests chiasmal or sellar abnormalities, the pneumoencephalogram remains the most accurate diagnostic procedure.
What hope for repair of the brain?
Authors: Raisman Geoffrey 
Published: December 2004

AbstractThe consequences of axonal injuries in the adult nervous system depend on the location of the axons: complete anatomical and functional repair can occur in the peripheral but not the central nervous sytem. A quantitative electron microscopical comparison of the reactions to axonal injuries in a peripheral site (the superior cervical sympathetic ganglion) and in a central site (the septal nuclei) in adult rats shows that in both cases the denervated postsynaptic sites are reinnervated by new formation of synapses. However, in the ganglion it is the originally severed axons which form the new synapses (regeneration), whereas in the septum the new synapses arise from local, undamaged axons and the cut axons do not reestablish their original contacts. Failure of regeneration in the central site is probably due to the fact that the growing axon sprouts are incapable of finding a pathway to the denervated tissues. Since the observations indicate that the neuropil of the central nervous system is capable of highly efficient synapse formation after injury, true repair of central nervous sytem damage might be possible if methods could be devised for dealing with the “pathway” problem.
Carotid and vertebral‐basilar transient ischemic attacks: Effect of anticoagulants, hypertension, and cardiac disorders on survival and stroke occurrence— A population study
Authors: Whisnant Jack P. , Niall E. F. Cartlidge , Elveback Lila R. 
Published: December 2004

AbstractA population of 199 patients from Rochester, MN, was followed from the time of their first carotid or vertebralbasilar transient ischemic attack(TIA). Patients treated with anticoagulants had no significant difference in survival from untreated patients. Among patients with carotid TIA who received anticoagulants, the net probability of stroke was slightly but not significantly lower than in untreated patients. The difference favoring treated patients with vertebral‐basilar TIA was significant starting at three months.The rate of intracranial hemorrhage was higher among all patients receiving anticoagulant treatment than among untreated patients and was significantly higher among those 55 to 74 years old. Almost all the hemorrhages occurred after a year or more of anticoagulant treatment and in patients more than 65 years old.Patients with high diastolic blood pressure had a significantly higher net probability of stroke than did patients with lower blood pressure and those receiving antihypertensive drugs. By implication, treatment of hypertension was effective in preventing stroke in patients with TIA.Linear discriminant analysis and actuarial analysis indicated that diastolic blood pressure and anticoagulant therapy were the only factors that influenced stroke occurrence. There was no suggestion that previous myocardial infarction, angina pectoris, valvular heart disease, cardiac arrhythmia, or congestive heart failure–individually or in combination–influenced the occurrence of stroke or survival.
Dopamine binding following prolonged haloperidol pretreament
Authors: Hitri Ana , Weiner William J. , Borison Richard L. , Diamond Bruce I. , Nausieda Paul A. , Klawans Harold L. 
Published: December 2004

AbstractThe number of binding sites for tritiated dopamine as well as well as the affinity of 3H‐dopamine for dopamine receptors was studied in rats chronically pretreated with the neuroleptic haloperidol. The rats were given haloperidol for 14 or 21 days and were killed on day 21. It was found that 14 days of haloperidol pretreatment followed by drug withdrawal resulted in a 67% increase in the number of 3H‐dopamine binding sites in the striatum and a ninefold increase in the affinity constant in the striatum. The same pretreatment regimen had no effect on either the number of receptor sites or the affinity constant in the nucleus accumbens. These results are consistent with the hypothesis that chronic neuroleptic pretreatment results in receptor site hypersensitivity in the striatum, which may be a major factor in the production of tardive dyskinesia.
The effects of single‐dose alternate‐day prednisone therapy on the immunological status of patients with neuromuscular diseases
Authors: Cook J. D. , Trotter J. L. , Engel W. K. , Sciabbarrasi J. S. 
Published: December 2004

AbstractSeveral immunological variables were examined in patients receiving high‐single‐dose, alternate‐day prednisone therapy for neuromuscular diseases. Dose‐dependent leukocytosis, lymphopenia, and monocytopenia occurred which were maximal 6 hours after prednisone administration but returned to control levels by the 24‐hour point. The lymphopenia involved T‐cells, B‐cells, and null cells, with the T‐cells most affected. Plasma cortisol levels and lymphocyte transformation in response to mitogens were also transiently and reversibly suppressed. There was a persistent decrease in serum IgG. Lymphocyte transformation was also suppressed when normal lymphocytes were incubated with treated patient sera or when treated patient lymphocytes were incubated in autologous pretreatment sera. The suppression factor was not removed from the lymphocytes by extensive washing. Patients whose disease responded to the high‐single‐dose, alternate‐day prednisone regimen were indistinguishable from nonresponders by the immunological responses measured.
Congenital sensory neuropathy with selective loss of small myelinated fibers
Authors: Low P. A. , Burke W. J. , McLeod J. G. 
Published: December 2004

AbstractThe results of clinical, electrophysiological, autonomic, and histopathological studies in a 6‐year‐old patient with congenital sensory neuropathy are presented. Selective loss of pain perception affecting the extremities was noted from infancy. Nerve conduction studies were normal. Small myelinated fibers were selectively reduced in the sural nerve, and unmyelinated fibers were normal.
Anomalies of motor development in hyperactive boys
Authors: Denckla Martha Bridge , Rudel Rita G. 
Published: December 2004

AbstractForty‐eight boys who scored high on rating scales for the “hyperactive syndrome” but who went without traditional neurological signs of learning disabilities were compared with 50 control boys on coordination tests. Discriminant function scores for speed, rhythm, and overflow correctly classified 89% of the boys as those with “hyperactive” versus “normal” behavioral histories. Thus, neurological examination of “hyperactive” boys does reveal developmentally immature coordination.
A double‐blind comparison of levodopa, madopa, and sinemet in Parkinson disease
Authors: Diamond Shirley G. , Markham Charles H. , Treciokas Leo J. 
Published: December 2004

AbstractA sixteen‐week study examined the effect of Madopa and Sinemet on patients with Parkinson disease suffering nausea or vomiting as side‐effects of levodopa therapy and compared the efficacy of the three preparations in controlling the symptoms of Parkinson disease. Following a control period on levodopa, 20 patients underwent four consecutive four‐week regimens as follows: (1) double‐blind, in which a randomized half received levodopa and half received Madopa; (2) single‐blind, in which all received Madopa; (3) double‐blind, in which a re‐randomized half received Madopa and half Sinemet; and (4) single‐blind, in which all received Sinemet. Levodopa administration via Sinemet and Madopa was held to a fixed 20% of prior levodopa dosage.Almost all patients showed great reduction in nausea and vomiting with both Madopa and Sinemet. Seventy percent of the patients showed improvement in disability compared to their levodopa baseline levels. Group means showed no difference between the improvement seen on Madopa and that seen on Sinemet. However, examination of individual responses showed that the majority of patients fared distinctly better on either Sinemet or Madopa.
Clinical variation in 2 related children with neuronopathic Gaucher disease
Authors: Grover Warren D. , Tucker Samuel H. , Wenger David A. 
Published: December 2004

AbstractThe clinical features in 2 second cousins with neuronopathic Gaucher disease include slowly progressive ataxia, spasticity, myoclonus, and seizures with relative preservation of intellectual function. Organomegaly was noted only in Patient 1. Both patients had diffuse slowing with paroxysmal features in electroencephalograms and a deficiency of β‐glucosidase activity in leukocytes and skin fibroblast cultures. The parents of Patient 1 and the related father of Patient 2 had levels of β‐glucosidase activity consistent with the carrier state for Gaucher disease. The value of β‐glucosidase activity in the mother of Patient 2 suggests a different mutation, the result being a defective enzyme component not detectable by measuring total activity.