bluuebunny/crossref_metadata_2025_split huggingface dataset:
Quantitative sudomotor axon reflex test in normal and neuropathic subjects
Authors: Low P. A. , Caskey P. E. , Tuck R. R. , Fealey R. D. , Dyck P. J. 
Published: January 2005

AbstractWe have quantified postganglionic sweat output in human subjects resulting from axon reflex stimulation using acetylcholine electrophoresis. Dehumidified nitrogen of controlled temperature and flow rate was passed through an acrylic plastic chamber placed over a defined area of skin. Sweat droplets were evaporated; humidity change was sensed by a narrow‐range humidity sensor housed in a temperature‐controlled compartment and was plotted on a chart recorder. The time integral (area under the curve) was continuously integrated and converted to absolute units using a derived equation. Because stimulation and recording were simultaneous, an accurate determination of the latency of the sweat response was also possible.Quantitative sudomotor axon reflex tests were performed on the left forearm and foot of 33 female and 29 male normal subjects aged 11 to 69 years. Acetylcholine, 10%, was electrophoresed for 5 mA‐minutes in the forearm and 10 mA‐minutes in the foot, and recording was continued for an additional 5 minutes. The mean sweat output in males was 2.7 and 3.0 times that in females in forearm and foot, respectively (p < 0.0001). Studies in selected autonomic neuropathies confirm that quantitative sudomotor axon reflex tests will detect postganglionic sudomotor abnormalities sensitively and reproducibly.
Hereditary form of sustained muscle activity of peripheral nerve origin causing generalized myokymia and muscle stiffness
Authors: Auger Raymond G. , Daube Jasper R. , Gomez Manuel R. , Lambert Edward H. 
Published: January 2005

AbstractSix patients in two unrelated families had a hereditary form of sustained muscle activity of peripheral nerve origin. Although varying in severity among affected family members, the disease was manifested clinically as generalized myokymia and muscle stiffness. Motor and sensory nerve conduction velocities and results of nerve biopsy were normal. Repetitive after‐discharges followed each motor nerve stimulus. Needle electrode examination demonstrated spontaneous recurrent bursts of motor unit activity. The burst activity of a motor unit occurred rhythmically (0.5 to 4 bursts per second) and independently of the activity of other units. Within a burst, the firing rate was high (150 to 300 Hz). The clinical and electromyographic abnormalities improved considerably after treatment with carbamazepine or phenytoin.
Calcium and vitamin D metabolism in guamanian chamorros with amyotrophic lateral sclerosis and parkinsonism–dementia
Authors: Yanagihara Richard , Garruto Ralph M. , Gajdusek D. Carleton , Tomita Akio , Uchikawa Takashi , Konagaya Yoko , Chen Kwang‐Ming , Sobue Itsuro , Plato Chris C. , Gibbs Clarence J. 
Published: January 2005

AbstractWe evaluated 16 Guamanian Chamorros with amyotrophic lateral sclerosis and 33 patients with parkinsonism‐dementia for disturbances of calcium and vitamin D metabolism. The serum immunoreactive parathyroid hormone level was mildly elevated in 6 patients with amyotrophic lateral sclerosis and in 5 patients with parkinsonism‐dementia. There were significant positive correlations between serum immunoreactive parathyroid levels and duration of illness in male patients with motor neuron disease, but not in female patients or in patients with parkinsonism‐dementia. Intestinal absorption of calcium, as assessed by serum and urinary activity of calcium 47 following oral administration, was decreased in 2 patients with amyotrophic lateral sclerosis and in 4 patients with parkinsonism‐dementia, all of whom had low levels of serum 1,25‐dihydroxyvitamin D. Reduction in cortical bone mass were striking in patients with motor neuron disease. A significant negative correlation was found between the percentage of cortical area of the second metacarpal bone and muscle atrophy and weakness, and significant positive correlations were found between degree of immobility and ratio of urinary hydroxproline to creatinine in patients with amyotrophic lateral sclerosis and parkinsonism‐dementia. In general abnormalities in calcium metabolism were subtle. Thus, if the demonstrated deposition of metals, particularly calcium and aluminum, in central nervous system tissues of Guamanians with these two conditions is a cause of the diseases and of the early appearance of neurofibrillary tangles in neurons, the accumulation has apparently occurred long before onset of symptoms, and detectable abnormalities of calcium and vitamin D metabolism may already have been corrected.
The lymphoproliferative response to measles virus in twins with multiple sclerosis
Authors: Greebstein Jeffrey I. , McFarland Henery F. , Mingioli Elizabeth S. , McFarlin Dale E. 
Published: January 2005

AbstractThe cellular immune response to measles virus, as measured by lymphocyte proliferation in normal individuals, is considerably lower than that to mumps or vaccinia viruses, and stable multiple sclerosis patients do not differ significantly from the norm. The response to these viruses was studied in 28 twin sets both concordant and discordant for multiple sclerosis. Normal responses to mumps and vaccinia viruses occurred throughout. Seven affected twins manifested a persistently elevated response to measles virus, whereas the unaffected twins had a (normal) low response. The differences were unrelated to differences in T cell subsets, unusual kinetics of the response, or differential susceptibility of lymphocytes to the effects of measles virus infection in vitro. The specificity of the response resides in an E+ subpopulation, and the addition of low‐responder E+ cells to high‐responder E+ cells failed to identify an active low‐responder suppressor population. These findings suggest the presence of clonally expanded measles‐specific T cell populations in the high responders with multiple sclerosis.
Levels of adenylate deaminase, adenylate kinase, and creatine kinase in frozen human muscle biopsy specimens relative to type 1/type 2 fiber distribution: Evidence for a carrier state of myoadenylate deaminase deficiency
Authors: Fishbein William N. , Davis John I. , Armbrustmacher Vernon W. , Griffin Joe L. , Foster Walter D. 
Published: January 2005

AbstractMyoadenylate deaminase deficiency is believed to refelct a genettic deficiency of skeletal muscle, but it pattern of inheritance has not been established. We examined, histochemically and by quantitative biochemical assay, muscle biopsy specimens from 3 putative carriers of this disorder. Adenylate kinease and creatine kinease were assayed in parallel with adenylate deaminease in order to establish enzyme activity ratios and the variation of each enzyme with fiber‐type distribution. Control tissue consisted of 34 biopsy specimens without notable abnormalities from 30 patients, and included 4 specimen pairs with disaparate fiber‐type contributions. By linear regression analysis, adenylate deam inase level averaged 2.8‐fold higher, and adenylate kinase 4.5‐fold higher, in type 2 than in type 1 fibers, whereas creatine kinase level did not differ. The slopes of the regression lines resulting from analysis of the four specimen pairs from infividual patients agreed well with the overll regression line in each plot. The 3 putative carriers had adenylate deaminease levels 2.5 to 5.7 times lower than the mean control value for thier fiber‐type distribution, but at least 20 times higher than thier enzyme‐deficient kinfolk. This finding indicates that a carrier state does exist, and that the deficencny state reflects an autosomal recessive inheritance pattern. Three additional biopsy specimens were excluded from evaluation when preliminary analysis showed elevated adenylate kinease/adenylate deaminase ratios that were outliers at the 1% level. This result suggests a carrier incidence of 10% in the muscle biopsy specimen population, which would markedly bias population estimates if undetected.
Hypometric saccades and low‐gain pursuit resulting from a thalamic hemorrhage
Authors: Brigell Mitchell , Babikian Viken , Goodwin James A. 
Published: January 2005

AbstractSaccadic and pursuit eye movements were measured in a patient with a right thalamic hemorrhage using an infrared reflection technique. Saccades were hypometric when directed away from the side of the lesion, and pursuit was low in gain, with many interposed saccades, when directed toward the side of the lesion. This eye movement pattern is similar to that observed in hemidecorticate patients. We postulate that the ocular abnormalities observed in our patients were secondary to an interruption of the efferent pathways from the right cerebral areas mediating saccadic and smoothpursuit eye movements.
Microangiopathy of vasa nervorum in dysglobulinemic neuropathy
Authors: Powell Henry C. , Rodriguez Moses , Hughes Richard A. C. 
Published: January 2005

AbstractThickening of vessel walls resulting from endothelial proliferation was observed in the vasa nervorum of eleven patients with peripheral neuropathy associated with dysglobulinemia. Electron microscopy showed endothelial proliferation accompanied by abnormal accumulation of masses of intracytoplasmic filaments in each case. Of the eleven patients with dysglobulinemia, nine had monoclonal gammopathy and two were found to have polyclonal elevation of gamma globulin levels. Symptoms of neuropathy characteristically preceded detection of serum protein abnormalities by months to years. Nerve fiber lesions involved primarily the axon in four cases; segmental demyelination was the principal abnormality in the other seven. Both abnormalities were present to some degree in all eleven patients. Biphasic myelinopathy with uniform separation of myelin lamellae attributable to globulin deposition was observed in four cases.The microvascular changes included endothelial cytoplasmic enlargement, virtually obliterating the vessel lumen in many instances, with thickening of pericytes, in which intracytoplasmic filaments were prominent and pinocytotic vesicles numerous. No extracellular filaments were noted, and amyloid stains were negative. Possible effects of these microvascular changes include ischemia resulting from severe vascular luminal narrowing, and altered vascular permeability. Severe loss of axons in this group of neuropathies may be the result of ischemia, whereas altered vascular permeability may admit globulin into the endoneurium, where it may directly affect the myelin sheath and precipitate demyelination.
Mechanisms of recovery from aphasia: Evidence from serial xenon 133 cerebral blood flow studies
Authors: Knopman David S. , Rubens Alan B. , Selnes Ola A. , Klassen Arthur C. , Meyer Maurice W. 
Published: January 2005

AbstractIn 21 patients who suffered aphasia resulting from left hemisphere ischemic infarction, the xenon 133 inhalation cerebral blood flow techique was used to measure cerebral blood flow within 3 months and 5 to 12 months after stroke. In addition to baseline measurements, cerebral blood flow measurements were also carried out while the patients were performing purposeful listening. In patients with incomplete recovery of comprehension and left posterior temporal–inferior parietal lesions, greater cerebral blood flow occurred with listening in the right inferior frontal region in the late studies than in the early studies. In patients with nearly complete recovery of comprehension and without left posterior temporal–inferior parietal lesions, early listening studies showed diffuse right hemisphere increases in cerebral blood flow. Later listening studies in this latter patient group showed greater cerebral blood flow in the left posterior temporal–inferior parietal region. The study provides evidence for participation of the right hemisphere is language comprehension in recovering aphasics, and for later return of function in left hemisphere regions that may have been functionally impaired early during recovery.
Value of computed tomographic scan in the evaluation of adult patients after their first seizure
Authors: Ramirez‐Lassepas Manuel , Morillo Luis R. , Gumnit Robert J. , Cipolle Robert J. 
Published: January 2005

AbstractThe role of the computed tomographic (CT) scan in evaluating adults after their first seizure(s) was determined by reviewing the hospital records of 148 patients studied within 30 days of their ictus. Patients with alcohol or drug intoxication or withdrawal or with known brain tumor, craniotomy, or open skull fracture were excluded. Type of seizure; neurological, CT, and electroencephalographic findings; final diagnosis; and hospital outcome were cross correlated.The cause of seizure was established in 71 patients (48%); a structural lesion was identified by CT in 55 (37%), and 16 (11%) had metabolic seizures. CT findings agreed with the results of neurological examination 82% of the time. Structural lesions (including three tumors) were found by CT in 14 patients (15%) with nonfocal findings and in 12 (22%) of those with generalized electroencephalographic abnormalities.Our findings are similar to those reported in adult epileptics and lead us to conclude that the CT scan is a valuable aid in establishing the cause of seizures and in disclosing early lesions.
Tissue culture demyelination by normal human serum
Authors: Silberberg Donald H. , Manning Margaret C. , Schreiber Alan D. 
Published: January 2005

AbstractSerum from all of 20 normal individuals induced some degree of demyelination when applied to well‐myelinated mouse cerebellum cultures. An intact complement sequence through C5 is required. Demyelinating activity was heat labile at 56°C for 30 minutes but was not destroyed at 50°C for 30 minutes (which inhibits properdin factor B and alternate complement pathway activation, but not the classic complement pathway). Sera from patients with agammaglobulinemia, C4 deficiency, or C6 deficiency all induced demyelination. Our results suggest that tissue culture demyelination results from nonimmunoglobulin activation of the alternate complement pathway and is not limited to sera from patients with neurological disease.
Azathioprine in the treatment of myasthenia gravis
Authors: Witte Arnold S. , Cornblath David R. , Parry Gareth J. , Lisak Robert P. , Schatz Norman J. 
Published: January 2005

AbstractTwenty‐four patients with myasthenia gravis were treated with azathioprine. Eighteen of the patients tolerated the drug. Six discontinued azathioprine therapy because of toxicity. Of the 18 patients, 15 (83%) improved while receiving azathioprine; in 8 (44%) improvement was felt to result solely from azathioprine. Initial response was seen after 4 to 10 months of treatment, with a mean of 6.4 months. Patients continued to improve for up to 24 months, with the mean time of peak improvement being 14 months. Relapse occurred within one year in all 6 patients in whom azathioprine administration was discontinued. Azathioprine is a reasonable alternative to corticosteroids in selected myasthenic patients requiring immunosuppression.
Augmented anti—acetylcholine receptor response following long‐term penicillamine administration
Authors: Bever Christopher T. , Dretchen Kenneth L. , Blake George J. , Chang Hai W. , Penn Audrey S. , Asofsky Richard 
Published: January 2005

AbstractBecause of the association of D‐penicillamine (DP) therapy with myasthenia gravis, we have studied long‐term DP treatment in five inbred strains of mice with doses comparable to those used in patients with rheumatoid arthritis. No clinical weakness or anti—acetylcholine receptor (AChR) antibody developed with up to 6 months of treatment, but augmented responses did occur to challenge with purified AChR in adjuvant. Anti‐AChR antibody titers in C57BL/6 and C3H/He mice were significantly higher after challenge with AChR in DP‐treated than in control mice. Augmented anti‐AChR titers were not seen in strain A mice, but after 6 months of DP treatment increased susceptibility developed to the induction of experimental autoimmune myasthnia gravis. Nine weeks after challenge with purified AChR, 10 of 11 mice developed clinical weakness, leading to death in 6. Results of edrophonium testing were positive in 5 of 6 mice, and electrophysiological abnormalities were demonstrated in 3 of the surviving mice.Long‐term DP treatment is associated with augmented anti‐AChR antibody responses in C3H/He and C57BL/6 mice, and increased susceptibility to experimental autoimmune myasthenia gravis in strain A mice.
Coronary artery disease in patients with cerebrovascular disease: A prospective study
Authors: Rokey Roxann , Rolak Loren A. , Harati Yadollah , Kutka N. , Verani Mario S. 
Published: January 2005

AbstractCoronary artery disease is the cause of death in most patients who have transient ischemic attacks or stroke. Evaluation for this condition is not routinely performed in such patients, and no prospective studies have been reported. We prospectively examined 50 consecutive patients with transient ischemic attacks or mild stroke to determine the prevalence and importance of coronary artery disease. All patients were examined by a cardiologist and underwent both exercise thallium‐201 scintigraphy and exercise radionuclide ventriculography. Sixteen patients were suspected to have coronary artery disease on the basis of clinical evaluation. In 15 of these the disease was confirmed by the nuclear scans. The remaining 34 patients had no clinical evidence of heart disease, yet 14 had abnormal cardiac scans. Twenty of 22 patients with abnormal scans who underwent cardiac catheterization had significant coronary artery disease or a cardiomyopathy. The discovery of heart disease altered clinical management in 13 patients. Overall, 29 of 50 patients had significant coronary artery disease, compared with a 7% prevelence of the condition in other patients of similar age at the same institution.
Clinical features of carbamyl phosphate synthetase‐I deficiency in an adult
Authors: Call Gregory , Seay Alan R. , Sherry Richard , Qureshi Ijaz A. 
Published: January 2005

AbstractCarbamyl phosphate synthetase‐I (CPS‐I) catalyzes the first reaction required for the conversion of ammonia to urea through the urea cycle. Severe CPS‐I deficiency causes marked hyperammonemia with encephalopathy in infancy and usually results in death within the first few months of life. We describe a 33‐year‐old woman whose CPS‐I activity is less than 5% of normal. She has had mild, intermittent symptoms throughout life but has never experienced severe encephalopathy. Although mildly retarded, she has no major neurological deficits. Therapy with a low‐protein diet, lactulose, and sodium benzoate has prevented recurrence of hyperammonemia and symptoms. Cranial computed tomographic scans demonstrate prominent lucency of cerebral white matter, and cerebral evoked potential recordings indicate slowed central conduction. These findings suggest that the metabolic disturbances in this patient may have adversely affected central myelin formation or maintenance. This woman represents, to our knowledge, the oldest reported patient with CPS‐I deficiency, and the case illustrates the need to consider urea cycle disorders in the differential diagnosis of intermittent neurological symptoms regardless of the patient's age.
Cerebral amyloid angiopathy causing intracranial hemorrhage
Authors: Kalyan‐Raman Uma P. , Kalyan‐Raman Krishna 
Published: January 2005

AbstractTen cases of sporadic cerebral amyloid angiopathy (CAA) were seen as intracranial hemorrhage. CAA was seen as a stroke or catastrophic intracranial hemorrhage in all cases. CAA complicated by intracranial hemorrhage carried a high mortality; 9 of 10 patients died in spite of aggressive medical or surgical treatment. Senile dementia of Alzheimer's type was an associated finding in only 1 patient. Computed tomographic scanning in 4 patients initially was not diagnostic for hemorrhage. Lambda chains and amyloid P protein were demonstrated in the areas of amyloid angiopathy immunocytochemically. The pathogenesis of hemorrhage in CAA could be either increased fragility of amyloid‐laden blood vessels or rupture of microaneurysms. Surgical treatment of intracranial hemorrhage in CAA should therefore be undertaken with caution.
Hexacarbon neuropathy: Cell body changes are early, dynamic, and specific
Authors: Sterman Arnold B. 
Published: January 2005

AbstractTo study the evolution of cell body alterations during toxic neuropathy we exposed rats to the prototype neurotoxin 2,5‐hexanedione and examined perikarya of lumbar dorsal root ganglia with electron microscopy and stereology at three stages of neuropathy. Compared to unintoxicated controls, neurons from rats with incipient (four weeks) and intermediate (six to seven weeks) neuropathy showed dispersion of Nissl substance and significant decreases (p < 0.001) in the volume fractions of Nissl bodies, but not of mitochondria or Golgi apparatus. However, at advanced (twelve to fourteen weeks) stages the volume fraction of Nissl bodies had increased and no longer differed from that of control; distinct chromatolysis‐like changes also became prominent. To evaluate the specificity of this remodeling we compared current morphometric results to data from rats exposed to acrylamide monomer and found significant differences (p < 0.001) in the volume fractions of Nissl bodies and mitochondria. We conclude: (1) in axonopathy, cell body remodeling occurs early and advances as a dynamic, evolving process, and (2) distinct differences in the patterns of cell body changes can distinguish the neuropathies studied, implying distinct cell body functions.
Principles and pitfalls of nerve conduction studies
Authors: Kimura Jun 
Published: January 2005

AbstractThis report reviews the fundamental principles and the changing concepts of nerve stimulation techniques, and discusses the proper application of these techniques in the differential diagnosis of peripheral nerve disorders. Nerve conduction studies help delineate the extent and distribution of the neural lesion and distinguish two major categories of peripheral nerve disease: demyelination and axonal degeneration. Although the method is based on simple principles, pitfalls abound in practice.Variability in nerve conduction measurement may result from temperature change, variations among nerve segments, and the effects of age. Other sources of error include excessive spread of stimulation current, anomalous innervation, temporal dispersion, and inaccuracy of surface measurement. Unlike a bipolar dervation, which selectively records near‐field potentials, a referential recording may give rise to stationary far‐field peaks from a moving source. Overlooking this possibility can lead to an incorrect interpretation of findings.Conventional nerve conduction studies deal primarily with measurements of the distal nerve segments in an extremity. More recent techniques are applicable to less accessible anatomical regions, as illustrated by elicitation of the blink reflex, F wave, and H reflex, and the use of the inching technique. Other methods used to assess special aspects of nerve conduction include the ischemic test and studies of slow‐conducting fibers.