bluuebunny/crossref_metadata_2025 huggingface dataset:
Hand wasting in spondylotic high cord compression: An electromyographic study
Authors: Stark Richard J. , Kennard Christopher , Swash Michael
Published: January 2005

Conventional and single‐fiber electromyographic studies in two patients with a wasted hand associated with spondylotic narrowing of the spinal canal at C3‐4 and C5‐6 revealed changes that were most severe in the hand muscles (C8, T1) and moENrately severe in the extensor digitorum communis (C7), but relatively spared the C5 and C6 myotomes. This gradation of abnormality within the cervical cord is consistent with stagnant hypoxia secondary to venous congestion.
Measurement of early spontaneous recovery from aphasia with stroke
Authors: Hartman Jacqueline
Published: January 2005

The amount of spontaneous change in aphasia was quantified in 44 right‐handed patients who were aphasic following a first and unihemispheric stroke. All were initially tested less than 14 days after onset and were reevaluated at a mean of 30 days. The Porch Index of Communicative Ability was administered for objective and precise measurement. Forty‐one of the patients Demonstrated significant improvement, a finding which must be taken into account in evaluating any claims for effectiveness of speech therapy.
Pain II: New approaches to management
Authors: Fields Howard L.
Published: January 2005

There is an intrinsic analgesia system in the brain with both endogenous opioid and biogenic amine links. This system provides a basis for understanding how different therapeutic approaches relieve pain. The present review critically evaluates the analgesic efficacy of the following therapies: placebo administration, transcutaneous electrical nerve stimulation, acupuncture, intrathecal and epidural opiate injection, electrical stimulation of the brain, and psychotropic drugs.
Myoclonic seizures following chlorambucil overdose
Authors: Byrne Thomas N. , Moseley Thomas A. E. , Finer Michael A.
Published: January 2005

A 3½‐year‐old child developed myoclonic seizures following a chlorambucil overdose. Serial electroencephalograms showed paroxysmal discharges that continued several days beyond clinical seizure activity. The use of the electroencephalogram when chlorambucil toxicity is suspected is discussed.
Glycogen debrancher deficiency is reproduced in muscle culture
Authors: Miranda Armand F. , DiMauro Salvatore , Antler Adriane , Rowland Lewis P. , Stern Lawrence Z.
Published: January 2005

Muscle cultured from two adults with debrancher deficiency myopathy showed abnormal glycogen deposits by electron microscopy. Glycogen debranching activity was markedly decreased, but phosphorylase activity was normal. Lack of glycogen debraching acstivity in muscle cultures from debrancher‐deficient patients contrasts with the presence of a fetal isoenzyme of phosphorylase in muscle cultured from patients with McArdle disease and suggests that the genetic control of the debraching enzyme does not change during muscle development.
Spinal cord infarction secondary to intervertebral disc embolism
Authors: Srigley John R. , Lambert Colin D. , Bilbao Juan M. , Pritzker Kenneth P. H.
Published: January 2005

A 45‐year‐old man acutely developed high spinal cord infarction. At autopsy a clinically occult fracture of a vertebral body was demonstrated. Emboli consisting of intervertebral disc material were found in marrow vessels and in the anterior spinal artery in the area of the infarct.
Evidence for a “dying‐back” gliopathy in demyelinating disease
Authors: Johnson Edward S. , Ludwin Samuel K.
Published: January 2005

Recurent demyelination was produced in mice by Cuprizone administration. During the second course of Cuprizone, the animals showed greater resistance to the toxin and demyelination occurred slowly and was complete only after prolonged periods. The earliest changes in oligodendrocytes occurred in the most distal processes, the inner cytoplasmic tongues, which showed degenerative changes 3 to 4 weeks before degeneration of the oligodendrocyte cell bodies or demyelination occurred. The results show for the first time that in demyelinating disease, a “dying‐back” process similar to that described in axonal disease can affect the oligodendrocyte.
Leukocyte and bacterial interrelationships in experimental meningitis
Authors: Giampaolo Casimiro , Scheld Michael , Boyd James , Savory John , Sande Merle , Wills Michael
Published: January 2005

Eighty‐one rabbits were inoculated with known concentrations of type III pneumococci by cisternal puncture and then started on antibiotic therapy the following day. Aliquots of cerebrospinal fluid were sampled at regular intervals both before and after therapy and then analyzed for bacterial titer and leukocyte count. These data were used to examine the interrelationships of inoculum size, leukocyte count, and bacterial titer to each other and their effects both univariately and multivariately on outcome. Inoculum size was the most important single variable affecting outcome, followed in order by the pretherapeutic bacterial titer and leukocyte count. Higher leukocyte counts early in the course of the disease prior to therapy were associated with a favorable outcome. Continued leukocyte elevation after therapy was associated with a poor prognosis. No correlations were found between inoculum size and subsequent bacterial titers; however, the bacterial titer immediately prior to therapy correlated negatively with the level of the early white cell response. The best prognostic information was obtained from the multivariate analysis of inoculum size and pretherapeutic white cell response.
Asterixis and encephalopathy following metrizamide myelography: Investigations into possible mechanisms and review of the literature
Authors: Bertoni John M. , Schwartzman Robert Jay , van Horn Gage , Partin James
Published: January 2005

Marked asterixis occurred in two patients following metrizamide myelography. One also suffered generalized seizures and the other had severe stuttering speech for seven days. The spectrum of toxic manifestations of metrizamide is reviewed with emphasis on the unusual lethargy and other depressive effects seen with this relatively safe agent. The hypothesis that metrizamide exerts a ouabain‐like effect on the cortical surface was tested. Metrizamide in concentrations as high as 20 mM had no inhibitory effect on rat cerebral K+‐para‐nitrophenylphosphatase, a partial reaction of (Na1 + K1)‐adenosine triphosphatase. Because metrizamide is a 2‐deoxyglucose analogue, a competitive inhibition of hexokinase at the first step in glycolysis was also postulated. Metrizamide was found to competitively inhibit commercial (microbial) hexokinase. The Michaelis constant for glucose rises from 0.13 to 0.25 to 0.33 to 0.91 mM in the presence of 0, 0.4, 1.0, and 2.0 mM metrizamide, respectively. Since the concentration of metrizamide over the cerebral cortex after routine myelography may be approximately 50 mM compared with a glucose concentration of only 3.6 mM (65 mg/dl), it is postulated that impaired brain glucose metabolism may be responsible for some of the toxic effects of metrizamide.
Epilepsy complicated by sleep apnea
Authors: Wyler Allen R. , Weymuller Ernest A.
Published: January 2005

A patient with a medically intractable seizure disorder was found to suffer sleep apnea as well. Treating the sleep apnea with permanent tracheostomy improved his generalized seizures. Although this combination of neurological disorders is probably rare, it should be considered if a suggestive history is obtained.
Evoked responses in vitamin B12 deficiency
Authors: Krumholz Allan , Weiss Howard D. , Goldstein Phillip J. , Harris Kenneth C.
Published: January 2005

Abnormalities of visual, brainstem auditory, and somatosensory evoked responses were demonstrated in two of seven individuals with vitamin B12 deficiency. The evoked response delays correlated directly with the degree of neurological dysfunction. Abnormalities were present in sensory systems without clinical evidence of involvement and were similar to those found in individuals with multiple sclerosis.
Preservation of the phrenic motoneurons in werdnig‐hoffmann disease
Authors: Kuzuhara Shegeki , Chou Samuel M.
Published: January 2005

A neuropathological study on spinal cords at the third, fourth, and fifth cervical levels in 4 patients with Werdnig‐Hoffmann disease revealed marked, generalized loss of motoneurons except for a cluster of large or medium‐sized ones in the most medial portion of the anteromedial cell group. These well‐preserved neurons were regarded as the phrenic motoneurons because diaphragmatic movement was the only muscle activity that remained until the last stage of illness. Furthermore, in its location and its cytoarchitectonic and neuronal characteristics, this neuronal cluster coincides with the phrenic nucleus as previously localized in the spinal cords of patients with phrenicotomy.
Quantification of de novo central nervous system igg measles antibody synthesis in sspe
Authors: Tourtellotte Wallace W. , Ma Booe I. , Brandes D. B. , Walsh M. J. , Potvin Alfred R.
Published: January 2005

De novo central nervous system (CNS) IgG measles antibody synthesis rate was determined in two patients with subacute sclerosing panencephalitis (SSPE). Cerebrospinal fluid and sera were incubated with different concentrations of measles virus to determine maximum absorption of IgG. The difference in IgG concentration before and after incubation was considered the amount of measles‐specinc IgG antibody. Various tests were done to assess the immunological specificity of the absorption test. Using our empirical formula, the de novo CNS IgG measles antibody synthesis rate for one patient with SSPE was 57 mg/day, or 59% of the total CNS IgG synthesis rate; for the other it was 145 mg/day, or 82%. Daily determination of CNS measles antibody by empirical formula provides a quantification of humoral immunity in SSPE that corrects for both passage of measles antibody across a damaged blood‐brain barrier and the effect of an elevated blood antibody level.
Adult‐onset acid maltase deficiency presenting as diaphragmatic paralysis
Authors: Sivak Edward D. , Salanga Virgilio D. , Wilbourn Asa J. , Mitsumoto Hiroshi , Golish Joseph
Published: January 2005

A 51‐year‐old man presented with a six‐year history of gradually progressive exertional dyspnea. His complaint was attributed to increasing age, but evaluation finally led to a diagnosis of diaphragmatic paralysis. Neurological examination disclosed only minimal evidence of weakness of limb muscles. Electromyographic studies suggested a diagnosis of acid maltase deficiency, which was confirmed by electron microscopic and biochemical studies of muscle biopsy sections. Exercise stress testing was necessary to define the limits of the patient's exercise tolerance.
Confrontation visual field techniques in the detection of anterior visual pathway lesions
Authors: Trobe Jonathan D. , Acosta Paulo C. , Krischer Jeffrey P. , Trick Gary L.
Published: January 2005

The accuracy of a variety of finger and color confrontation tests in identifying chiasmal and optic nerve visual field defects was assessed in patients whose field defects had been established beforehand by a conventional achromatic kinetic technique on the Goldmann perimeter. Kinetic and static finger confrontation methods identified an average of 42% of the 28 chiasmal hemianopic defects. False negatives included eyes with hemianopias complete to the largest (V4e) Goldmann isopter. False positives (average, 15%) occurred in eyes containing nerve fiber bundle defects with borders that fell near the vertical fixational meridian.

Kinetic and static color confrontation techniques were 78.6% sensitive to hemianopias. Accuracy did not differ significantly whether the red target was presented kinetically or statically against the tangent screen, projected on the Autoplot screen, or held in the examiner's hand without attention to background. False positives (average, 23%) were slightly greater than with finger confrontation methods and occurred not only in eyes with nerve fiber bundle defects but also in eyes with no defects in reference visual fields.

Finger confrontation identified 11% or fewer of optic nerve field defects, while some color techniques detected as many as 33 1/3%. There were no false positives.
18FDG positron emission computed tomography in a study of aphasia
Authors: Metter E. Jeffrey , Wasterlain Claude G. , Kuhl David E. , Hanson Wayne R. , Phelps Michael E.
Published: January 2005

Positron emission computed tomography (PECT) using 18F‐2‐fluoro‐2‐deoxy‐D‐glucose (FDG) was used to investigate the correlations between clinical status, anatomy (as described by CT), and metabolism in five patients with stable aphasia resulting from ischemic cerebral infarction. Local cerebral metabolic activity was diminished in an area larger than the area of infarction demonstrated by CT. In one patient, FDG PECT revealed a metabolic lesion that probably caused the aphasic syndrome and was not apparent by CT. The data suggest that reliance on CT in delineating the extent of the brain lesion in aphasia or other neuropsychological defects can be misleading; FDG PECT may provide important additional information. Two patients with similar metabolic lesions had very different clinical syndromes, showing that even when currently available methods are combined, major gaps remain in clinicoanatomical correlations in aphasia.
Humoral immunity in guamanians with amyotrophic lateral sclerosis and parkinsonism‐dementia
Authors: Hoffman Paul M. , Robbins Deanna S. , Oldstone M. B. A. , Gibbs C. J. , Gajdusek D. Carleton
Published: January 2005

Among Guamanian natives, serum IgA and IgG levels were found to be higher than normal in amyotrophic lateral sclerosis (ALS); serum IgA was higher and IgM lower than normal in parkinsonism‐dementia (PD). IgA levels increased with age in ALS, PD, and normal subjects; IgG increased with age in ALS and IgM decreased with age in PD. Serum immunoglobulin (Ig) levels did not correlate with the duration of either disease. Immunodeficient ALS and PD patients had higher IgM and lower IgA levels than the other ALS and PD patients.

Neither differences in viral antibody titers nor the presence of autoantibodies or circulating immune complexes could account for the variations in serum Ig levels between patients and controls. We conclude that differences in serum Ig levels in ALS and PD patients are probably due to repeated infections and abnormal immunoregulation accompanying immunodeficiency during the course of ALS and PD, rather than to a specific antiviral or autoimmune response.
Effect of L‐glutamine and isoniazid on torticollis and segmental dystonia
Authors: Korein Juulius , Lieberman Abraham , Kupersmith Mark , Levidow Lucie
Published: January 2005

Fourteen patients with spasmodic torticollis and other segmental dystonic syndromes, who were refractory to previous forms of therapy, were selected for treatment with drugs intended to elevate brain γ‐aminobutyric acid (GABA) levels. These patients were simultaneously given diazepam, isoniazid, pyridoxine, and large doses of L‐glutamine. Involuntary spasmodic activity improved to varying degrees in 7 patients; in 2 the dyskinesia became worse. Transient alteration of renal or hepatic function occurred in 6 patients and mild euphoria unrelated to neurological improvement in 8. Two patients are still being treated. Deficiency of GABA may be a factor in some patients with these disorders.
Amyotrophic lateral sclerosis and parkinsonism‐dementia among Filipino migrants to Guam
Authors: Garruto Ralph M. , Gajdusek D. Carleton , Chen Kwang‐Ming
Published: January 2005

Amyotrophic lateral sclerosis (ALS) has occurred in 9 Filipino migrants to Guam 1 to 29 years after their arrival and parkinsonism‐dementia (PD) in 2 migrants 13 and 26 years after arrival. Seven additional Filipino patients developed more classic Parkinson disease (P) 5 to 24 years after their migration to Guam. Furthermore, 10 part‐Filipino patients who were born on Guam of Filipino and Chamorro parentage developed ALS, while 6 such part‐Filipino patients developed PD. An estimate of the average annual crude mortality rate for ALS among Filipino migrants was approximately six times that of the continental United States, yet half that currently observed among Chamorros living on Guam. A majority of all migrant patients were born in northwestern Luzon in the Philippine Islands, indicating the desirability of an intensive field epidemiological investigation in that area.
Monoaminergic mechanisms and experimental cataplexy
Authors: Foutz Arthur S. , Delashaw John B. , Guilleminault Christian , Dement William C.
Published: January 2005

The effects of pharmacological alteration of the monoamine systems were investigated in a canine model of narcolepsy. Cataplexy was quantified in eight severely affected dogs by means of the food‐elicited cataplexy test. The specific norepinephrine (NE) uptake blocker nisoxetine, and (to a much lesser extent) the specific serotonin (5‐HT) uptake blocker fluoxetine, significantly suppressed cataplexy, as did the tricy'lic antidepressants protriptyline, amitriptyline, and chlorimipramine. Thus, experimental cataplexy is suppressed more by inhibition of the uptake of NE than of 5‐HT. Methylphenidate, the α‐adrenoreceptor blocker clonidine, and the dopamine receptor blocker pimozide also suppressed cataplexy in dogs. The β‐adrenergic blocker propranolol, the fatty acid γ‐hydroxybutyrate, and the monoamine oxidase inhibitors clorgyline and pargyline had little or no effect. With one exception (pimozide). all the drugs that suppressed cataplexy are known to be potent suppressors of REM sleep. The suppression of cataplexy induced by nisoxetine or protriptyline was reversed by the anticholinesterase physostigmine, further supporting a postulated aminergic‐cholinergic interaction in the mechanisms for cataplexy.
Regional activities of metabolic enzymes and glutamate decarboxylase in human brain
Authors: Maker Howard S. , Weiss Cipora , Weissbarth Sulamith , Silides Demetra J. , Whetsell William
Published: January 2005

Interpretation of biochemical measurements in the human brain after death is complicated by a variety of premortem, perimortem, and postmortem factors. The activity of glutamic acid decarboxylase (GAD) in particular has been found to vary considerably among human brains. In contrast to neurotransmitter‐associated enzymes, metabolic enzymes are present in all brain cells and should not be specifically lost by patterned neuronal cell loss such as that which occurs in Parkinson disease. We compared the activity of GAD to that of the metabolic enzymes creatine kinase (CK), adenylate kinase, hexokinase, β‐glucuronidase, and malate, lactate, glucose‐6‐phosphate, and isocitrate dehydrogenases in 24 regions of six human brains. Of the metabolic enzymes, only CK showed a 5‐fold variation approaching that of GAD. Like GAD, CK activity was stable postmortem, but its activity was apparently inversely related to the severity and duration of the preterminal illness. CK may be a useful marker of agonal deterioration.
Virus‐induced electrotonic coupling: Hypothesis on the mechanism of periodic EEG discharges in Creutzfeldt‐Jakob disease
Authors: Traub Roger D. , Pedley Timothy A.
Published: January 2005

Experimental evidence and computer modeling indicate that periodic synchronous cellular depolarizing bursts (interictal spikes) arise when the balance between recurrent inhibition and local excitatory coupling is altered. Such a mechanism may explain the generalized periodic sharp waves that characterize the electroencephalogram of many patients with Creutzfeldt‐Jakob disease. In Creutzfeldt‐Jakob disease, fusions of neuronal processes, particularly dendrites, may lead to abnormal electrotonic coupling between cells, providing the basis for powerful excitatory interaction whereby large neuronal aggregates burst in near synchrony. Cortical synchronous discharges would give rise to sharp waves in the electroencephalogram, whereas similar discharges in brainstem, spinal cord, or elsewhere could lead to myoclonic jerks.
Humoral immunity in myasthenia gravis: Biochemical characterization of acquired antireceptor antibodies and clinical correlations
Authors: Tindall Richard S. A.
Published: January 2005

The titer and characteristics of antiacetylcholine receptor antibody (A ChR‐Ab) were investigated in 184 patients with myasthenia gravis. Mean A ChR‐Ab titers of each clinical grade increased with the severity of the disease. A ChR‐Ab was always of an IgG class. IgM (5 of 92) and IgA (2 of 48) class AChR‐Ab were detected, but only concurrently with IgG and in low concentrations. IgG subclass 3 was not prominent. In 3 patients with A ChR‐Ab titers in the normal range, blockade of bungarotoxin binding to receptor could still be demonstrated. A ChR‐Ab from 6 patients was heterogeneous in affinity for receptor, reactivity with receptor from human ocular and gastrocnemius muscle, and blockade of toxin binding. A ChR‐Ab was oligoclonal in 4 of 6 patients, as shown by concurrent production of A ChR‐Ab IgG of both κ and λ types. Amniotic fluid and fetal cord serum did not interfere with antibody‐receptor interaction. Variation in the pattern of weakness among patients was a function of both the heterogeneity of A ChR antibodies and the antigenic uniqueness of receptor complexes from different human muscles.
The origin of spontaneous electrical activity at the end‐plate zone
Authors: Brown William F. , Varkey George P.
Published: January 2005

Two types of spontaneous electrical activity are present at the end‐plate zone: low‐voltage negative potentials that correspond to miniature end‐plate potentials, and larger voltage negative‐positive potentials. The electrogenic origin of the latter has been uncertain. The origin of these larger potentials was investigated in the rat phrenic nerve diaphragm preparation and in human gastrocnemius muscle just prior to intubation during administration of preoperative anesthesia. In the hemidiaphragm the larger voltage negative‐positive potentials, were rarely triggered by intracellular or tungsten microelectrodes. The negative‐positive potentials, however, were clearly triggered by contact of the concentric needle electrode with muscle hemidiaphragm at the end‐plate region. The potentials were abolished by curare. Likewise, the equivalent potentials observed at the human gastrocnemius end‐plate zone were blocked by neuromuscular blocking agents. Therefore, these positive‐negative discharges represent postsynaptic muscle fiber action potentials and not nerve fiber activity. They were probably presynaptically activated by mechanical irritation of the motor axon terminal and preterminal branches.
Congenital hypomyelination neuropathy: Glial bundles in cranial and spinal nerve roots
Authors: Towfighi Javad
Published: January 2005

Autopsy examination of a 31/4‐year‐old child with a severe congenital hypomyelination neuropathy showed the anterior spinal nerve roots and motor cranial nerves to be almost devoid of myelin in their subarachnoid course. The posterior spinal nerve roots and peripheral nerves were less severely affected. Onion bulb formation was minimal and was present only in the sural nerve. There was extensive glial overgrowth in cranial nerves and spinal nerve roots adjacent to the brainstem and spinal cord. The extent and severity of glial overgrowth were similar to that described in Werdnig‐Hoffmann disease and morphologically appeared as glial bundles. These glial bundles are most likely secondary to chronic myelin and axonal damage.
Juvenile spinal muscular atrophy: A new hexosaminidase deficiency phenotype
Authors: Johnson William G. , Wigger H. Joachim , Karp Herbert R. , Glaubiger Lawrence M. , Rowland Lewis P.
Published: January 2005

A 24‐year‐old Ashkenazi Jewish man was evaluated for a nine‐year history of progressive leg weakness with fasciculations. Electromyography, nerve conduction velocities, muscle biopsy, and serum creatine kinase were consistent with anterior horn cell disease. On rectal biopsy, ganglion cells were filled with membranous cytoplasmic bodies and an unusual submucosal layer of periodic acid‐Schiff positive histiocytes filled with granules was seen. Hexosaminidase A in serum and leukocytes was severely decreased in the patient and partially decreased in the parents and a brother. A paternal relative had classic infantile Tay‐Sachs disease.

Juvenile spinal muscular atrophy in this patient, closely resembling the Kugelberg‐Welander phenotype, resulted from an α‐locus hexosaminidase deficiency disorder, possibly a genetic compound of HEXα2 and a milder hexosaminidase α‐locus allele. Other cases of hexosaminidase deficiency have included anterior horn cell disease as part of a more complex disorder, but this is the first case, to our knowledge, of a hexosaminidase deficiency disorder presenting as spinal muscular atrophy.
Interaction of myasthenic immunoglobulins and cholinergic agonists on acetylcholine receptors of rat myotubes
Authors: Ashizawa Tetsuo , Elias Stanton B. , Appel Stanley H.
Published: January 2005

Acetylcholine receptor (AChR) antibodies from patients with myasthenia gravis (MG) impair neuromuscular transmission by altering the number of AChRs at the skeletal muscle motor endplate. Cholinergic agonists similarly impair transmission by altering the number and affinity of AChRs. The frequent clinical resistance to anticholinesterase medication in patients with MG has raised the question of possible synergistic effects of these agents on regulation of AChRs and neuromuscular transmission. To investigate this question, rat myotube cultures were incubated with MG globulin and carbamylcholine (CMC) for 20 hours. The number of AChRs was assayed by 125I‐α‐bungarotoxin binding. Incubation of cultures with both MG globulins and CMC consistently produced greater reductions of AChRs than incubation with either substance alone. Cholinergic antagonists blocked the CMC effect but not the MG globulin effect. A muscarinic antagonist had no effect. The effects of short‐term incubation with these substances on the affinity of AChR were assessed by the rate of 125I‐α‐bungarotoxin‐AChR complex formation. CMC desensitized AChRs, but MG globulins did not alter the affinity of AChRs. Results with this in vitro model suggest that chronic anticholinesterase therapy in the presence of MG AChR antibodies may aggravate failure of neuromuscular transmission in MG.
Nerve stimulation test in murine experimental autoimmune myasthenia gravis
Authors: Pachner Andrew R. , Kantor Fred S.
Published: January 2005

Use of the mouse model of myasthenia gravis (murine EAMG), ideally suited for immunological study, has been hampered by the relatively mild character of the disease and by the extended time and effort required for inducing severe disease. Electromyographic measurement of the compound action potential after repetitive stimuli, the nerve stimulation test, was evaluated for its ability to diagnose neuromuscular transmission defects in mice immunized with Torpedo acetylcholine receptor. With the combination of provocative maneuvers and raising of the body temperature, EAMG could be diagnosed in nearly all immunized animals a few weeks after immunization, whereas clinical evaluation of muscle weakness was positive in less than half of immunized mice months after the first immunization. Thus, EMG provides a means of objective evaluation of the disease and attempts at its experimental modification.
In vivo demyelinating activity of sera from patients with Guillain‐Barré syndrome
Authors: Saida Takahiko , Saida Kyoko , Lisak Robert P. , Brown Mark J. , Silberberg Donald H. , Asbury Arthur K.
Published: January 2005

The in vivo demyelinating capacity of sera from 27 patients with Guillain‐Barré syndrome (GBS) and 47 other individuals was studied by intraneural injection into rat sciatic nerves. The morphological features of the nerves in cross section taken just proximal to the site of needle insertion was assessed 48 hours after injection and the extent of demyelination was quantitated. All 27 GBS serum samples were obtained in the first three weeks of clinical disease. Of these, 11 (41%) produced demyelination. Demyelinative activity of GBS sera correlated only with severity of clinical disease (p<0.01). The extent of demyelination after intraneural injection of human sera was less intense on average than that produced by sera from animals with experimental allergic neuritis. Three of 40 (7.5%) sera obtained from normal subjects and patients with other neurological diseases also caused in vivo demyelination, although the activity was weaker and occurred less often than with GBS serum.
Low CSF GABA in parkinsonian patients who respond poorly to therapy or suffer from the “on‐off” phenomenon
Authors: Teychenné Paul F. , Ziegler Michael G. , Lake C. Raymond , Enna S. J.
Published: January 2005

The γ‐aminobutyric acid concentration in cerebrospinal fluid was measured by radioreceptor assay in 23 parkinsonian patients (“good responders” and “poor responders” to drug therapy, “on‐off” state) and 11 age‐matched control subjects. Mean concentrations were similar but significantly lower in the poor responders and the “on‐off” state than in the good responders and control subjects. Patients who show good response to drug therapy appear to have a normal level of GABAergic activity that is not altered by drug therapy.
Selective fiber vulnerability in acute ischemic neuropathy
Authors: Parry Gareth J. , Brown Mark J.
Published: January 2005

To study the relationship between fiber size and vulnerability to ischemia, we produced partial infarction of the proximal posterior tibial nerve of Wistar rats by injecting arachidonic acid into the ipsilateral femoral artery. In the resulting lesion degenerating fibers were concentrated in the center of the nerve. Distal to the infarct, surviving myelinated and unmyelinated fibers were evenly distributed throughout both fascicles. Smaller myelinated fibers (<6μm in diameter) were more severely depleted than larger ones (>6μm). Unmyelinated fibers were reduced in number in proportion to or to a greater extent than myelinated fibers. These findings demonstrate that large myelinated fibers are relatively less vulnerable to peripheral nerve ischemia than smaller fibers, and do not support the contention that unmyelinated fibers are inherently resistant to acute ischemia.
Rapid improvement of myasthenia gravis after plasma exchange
Authors: Nielsen Viggo Kamp , Paulson Olaf B. , Rosenkvist Jørgen , Holsøe Eva , Lefvert Ann Kari
Published: January 2005

Neuromuscular transmission and the staircase phenomenon in the adductor pollicis muscle were studied in six patients with myasthenia gravis before and 24 hours after single plasma exchanges given in short series over one to four weeks. An improvement in neuromuscular transmission was observed in all patients but one within 24 hours, suggesting that an immunological block of receptor sites is reversible or that acetylcholine receptors are rapidly resynthesized. In addition to a neuromuscular transmission defect, two of the patients also showed evidence of impaired excitation‐contraction coupling with a negative staircase phenomenon and reduced posttetanic twitch potentiation. This condition became normal after plasma exchange, which may indicate that a reversible immunological impairment of the sarcoplasmic reticulum was present. Improvement lasted up to several months. The anti‐acetylocholine receptor antibody titers were lowered in response to single plasma exchanges, but actual titer levels showed poor correlation with clinical and electrophysiological changes.
Monoclonal antibody analysis of blood T‐cell subsets in myasthenia gravis
Authors: Skolinik Paul R. , Lisak Robert P. , Zweiman Burton
Published: January 2005

Analysis of peripheral blood T‐cell subsets and B‐cells in patients with myasthenia gravis was performed using monoclonal antibodies and antibody against surface immunoglobulins (SIg) in an immunofluorescent technique. We found a modest but significant decrease in percentages of OKT3‐ and OKT8‐positive cells (thought to represent total T‐cells and T‐suppressor/cytotoxic cells, respectively) in myasthenics as a group. The percentage of OKT3‐positive cells was significantly decreased in patients with late‐onset disease (>35 years old), while the percentage of OKT8‐positive cells was significantly reduced in those with early‐onset myasthenia (<35 years old). Both thymectomized and nonthymectomized patients exhibited a decreased percentages of SIg‐positive cells in myasthenics and controls. Our results suggest that only modest imbalances of circulating immunoregulatory lymphocytes occur in myasthenia gravis; however, it is conceivable that the small differences observed in this study may reflect pathogenetically important reductions in a functionally distinct lymphocyte subpopulation.
Influence of azathioprine (Imuran) on in vitro immune function in multiple sclerosis
Authors: Oger Joel J.‐F. , Antel Jack P. , Kuo Han Hwa , Arnason Barry G. W.
Published: January 2005

In vitro immune function was assessed in patients with multiple sclerosis (MS) who were receiving Imuran therapy, in untreated MS patients, and in controls. In untreated stable MS patients, concanavalin A (Con A)‐driven mitogenic reactivity (T effector function) and Con A‐induced suppressor activity were modestly reduced compared to controls; pokeweed mitogen‐induced immunoglobulin G (IgG) secretion was increased. Untreated patients with active MS demonstrated high levels of IgG secretion and marked decreases in suppressor activity. In Imuran‐treated patients, Con A mitogenic responses and suppressor activity were comparable to those observed in untreated stable patients, and IgG secretion was reduced. The results in the treated patients likely reflect a direct effect of Imuran on B cell function rather than an indirect effect mediated via suppressor cells.
Cerebrospinal fluid acid‐base balance in newborns
Authors: Hermansen Marcus C. , Ellison Patricia H.
Published: January 2005

In adults, cerebrospinal fluid (CSF) acid‐base balance is an important determinant of cerebral blood flow, ventilatory drive, and state of consciousness. Sixteen lumbar punctures were performed on newborns (mean gestational age, 33.8 weeks; range, 26 to 44 weeks). The mean CSF pH was 7.366 units, 0.036 less than the mean capillary blood pH (NS). The mean CSF arterial carbon dioxide tension (Pco2) was 40.3 mm Hg, 6.7 greater than the mean blood Pco2 (p < 0.01). The mean bicarbonate ion concentration in CSF was 21.8 mEq/L, 1.2 greater than that in blood (NS). Acid‐base determinations in CSF of neonates with intraventricular hemorrhage did not differ significantly from those of neonates with otherwise normal CSF. These acid‐base values are similar to those found in adults and did not vary with gestational age, suggesting that mechanisms to establish this acid‐base gradient are developed by 26 weeks' gestation.
Measurement of the scrapie agent using an incubation time interval assay
Authors: Prusiner Stanley B. , Cochran S. Patricia , Groth Darlene F. , Downey Deborah E. , Bowman Karen A. , Martinez Hugo M.
Published: January 2005

The titer of the scrapie agent was determined by measurements of time intervals from inoculation to onset of illness and from inoculation to death. Both intervals were found to be inversely proportional to the size of the dose injected intracerebrally into random‐bred weanling Syrian hamsters. The logarithms of the time intervals minus a time factor were linear functions of the logarithm of the inoculum size. The time factors were determined by regression analysis in order to maximize these linear relationships. An equation relating the titer of the inoculum to the dilution of the sample and the length of the time intervals was developed. This equation facilitates the use of a computerized data base. Validation of these relationships was provided by comparing samples for which the agent was measured both by end‐point titration and by time interval assay. Agreement between the two methods was generally within ±0.5 log10 median infective dose units. No differences between the molecular properties of the agents from hamster and murine sources were observed using primarily the incubation time interval method with the former and end‐point titration with the latter. The advantages of this new approach based on time interval measurements are considerable with respect to time and resources.
The electroencephalogram in Tourette syndrome
Authors: Bergen Donna , Tanner Caroline M. , Wilson Robert
Published: January 2005

The electroencephalogram (EEG) was studied in 38 patients with Tourette syndrome. Psychometric tests and neurological evaluation identified patients with signs of additional central nervous system dysfunction. Thirteen patients (34%) had some EEG abnormality. In contrast to findings by other investigators, epileptiform activity was uncommon (only 2 out of 38). Most of the patients with EEG abnormalities either had other objective signs of neurological dysfunction or were taking haloperidol, a drug known to disturb the EEG.
A benign motor neuron disorder: Delayed cramps and fasciculation after poliomyelitis or myelitis
Authors: Fetell Michael R. , Smallberg Gerald , Lewis Linda D. , Lovelace Robert E. , Hays Arthur P. , Rowland Lewis P.
Published: January 2005

Two patients had a delayed syndrome of benign fasciulation after complete recovery from paralytic poliomyelitis and a third had the syndrome after an attack of purely motor myelitis. Myalgia was prominent in all three, and two had frequent cramps. Denervation hypertrophy occurred in two. The patients were observed for at least three years, and no new weakness was seen. These cases suggest that some chronic diseases of motor neurons may be benign.
Cognition in Parkinson disease: An event‐related potential perspective
Authors: Hansch Edward C. , Syndulko Karl , Cohen Stanley N. , Goldberg Zev I. , Potvin Alfred R. , Tourtellotte Wallace W.
Published: January 2005

Auditory event‐related potentials (ERP) elicited in a target detection stimulus paradigm and pattern‐shift visual ERPs were studied in 20 male patients with idiopathic Parkinson disease (PD) and 20 age‐matched normal controls. Patients showed significantly increased latencies for both the P200 and P300 components of the auditory ERP. Patients and controls showed no significant differences in latency of the visual ERP but patients showed significantly decreased amplitude. Only one of five neuropsychological measures, the Symbol Digit Modalities test (SDMT), showed a significant negative correlation with P300 latency. The significant association between the two measures that showed impairments in the PD patients (P300 latency and SDMT scores) suggested that these measures reflect a common, disrupted aspect of cognitive function in PD.
Concanavalin a binding of the cell surface of Duchenne muscle in vitro
Authors: Heiman‐Patterson Terry D. , Bonilla Eduardo , Schotland Donald L.
Published: January 2005

Ultrastructural examination of concanavalin A (Con A) binding sites in cultured skeletal muscle from six patients with Duchenne muscular dystrophy (DMD) revealed no abnormalities at the cell surface when compared to normal cultured skeletal muscle. Reaction product at the cell surface appeared regular and continuous in both normal and dystrophic cultured muscle cells. These findings indicate that the abnormalities of Con A surface binding found in biopsied muscle of DMD are not present in aneurally cultured dystrophic muscle cells.
Entrapment of the suprascapular nerve at the spinoglenoid notch
Authors: Aiello I. , Serra G. , Traina G. C. , Tugnoli V.
Published: January 2005

A 58‐Year‐old fencing instructor developed selective denervation of the infraspinatus muscle. It was found to be caused by entrapment of the suprascapular nerve at the spinoglenoid notch due to a hypertrophied inferior transverse scapular ligament.
Electrophysiological and clinical correlation in myasthenia gravis
Authors: Oh Shin J. , Eslami Nasrollah , Nishihira Takeo , Sarala P. K. , Kuba Tadashi , Elmore Robert S. , Sunwoo Il Nam , Ro Young Il
Published: January 2005

Repetitive nerve stimulation testing of the ulnar nerve was systematically performed on 21 normal controls and 120 patients with myasthenia (MG). Diagnostic sensitivity increased from 0% in MG in remission and 17.2% in ocular MG to 100% in severe generalized MG. Six types of responses were observed in MG and could be classified into two distinct patterns based upon disease severity: (1) in mild MG, an abnormal decremental response at low rate of stimulation, normal response at high rate of stimulation, and prominent posttetanic facilitation and exhaustion phenomena; and (2) in severe MG, abnormal decremental responses at low as well as high stimulation rates and less common posttetanic facilitation and rare posttetanic exhaustion phenomena. This difference is most likely due to the severity of the neuromuscular block in MG.
Facioscapulohumeral dystrophy associated with hearing loss and coats syndrome
Authors: Taylor Donald A. , Carroll James E. , Smith Morton E. , Johnson Mary O. , Johnston Glen P. , Brooke Michael H.
Published: January 2005

Two unrelated cases of infantile facioscapulohumeral dystrophy (FSHD) with Coats syndrome of the retina and sensorineural hearing loss are described. Children with otherwise unexplained visual or hearing deficit should be examined for somatic muscle weakness, which may present only as facial weakness. Early recognition of Coats syndrome in children with FSHD may lead to preservation of vision with appropriate therapy.
Voluntary alteration of visual evoked potentials
Authors: Bumgartner James , Epstein Charles M.
Published: January 2005

Fifteen normal adults had pattern‐shift visual evoked potentials (PSVEPs) using monocular checkerboard pattern reversal. Subjects were instructed to concentrate on the pattern in the first set of trals and then to avoid perceiving it in the second set. Direct, continuous visual observation ensured that the eye remained open and fixed on the pattern. Nonetheless, a third of the subjects could spontaneously alter or obliterate the PSVEP using several maneuvers, including meditation, daydreaming, and convergence. Several could produce shifts in apparent PSVEP latency. With explicit instruction, most subjects could learn to alter their PSVEP by maneuvers that are inapparent even to an observant technician.
Diffuse axonal injury due to nonmissile head injury in humans: An analysis of 45 cases
Authors: Adams J. Hume , Graham D. I. , Murray Lilian S. , Scott Grace
Published: January 2005

Forty‐five cases of diffuse axonal injury (DAI) brought about by nonmissile head injury in humans are analyzed and compared with 132 cases of fatal head injury without DAI. All cases were subjected to a comprehensive neuropathological study. In the patients with DAI a statistically significant lower incidence of lucid interval, fracture of the skull, cerebral contusions, intracranial hematoma, and evidence of high intracranial pressure were found, with a higher incidence of head injury due to road traffic accident. Brain swelling and hypoxic brain damage were not statistically different in the two groups. The features of DAI in humans are compared with the DAI that has been produced in subhuman primates by pure inertial loading brought about by angular acceleration of the head. The available evidence indicates that DAI in human beings occurs at the time of head injury and is not due to complicating factors such as hypoxia, brain swelling, or raised intracranial pressure.
Epidemiological surveillance of amyotrophic lateral sclerosis and parkinsonism‐dementia in the commonwealth of the Northern Mariana Islands
Authors: Yanagihara R. T. , Garruto R. M. , Gajdusek D. C.
Published: January 2005

Amyotrophic lateral sclerosis (ALS) and parkinsonism‐dementia (PD), two fatal neurological diseases of unknown cause, occur in high incidence among the Chamorro people of Guam, the largest and southernmost island within the Mariana archipelago. To reassess and extend our present epidemiological knowledge of these degenerative diseases in this focal geographical region, a systematic search for both disorders was conducted on the remaining inhabited islands of Rota, Tinian, Saipan, and the four remote islands of Anatahan, Alamagan, Pagan, and Agrihan within the Marianas chain. One case of ALS (on Saipan), 2 cases of PD (on Rota and Saipan), and 6 cases of parkinsonism without dementia (2 on Rota, 3 on Saipan, 1 on Tinian) were encountered among the approximately 17,000 inhabitants. No cases of either ALS, PD, or parkinsonism were found in the four remote Northern Islands. An additional 22 cases of ALS and 8 cases of PD were identified from reports of previous case‐finding surveys, hospital records, and death certificates. Among Chamorros born on Rota, the average annual age‐adjusted mortality rates of ALS per 100,000 population were 37.7 for the 15‐year period 1956 to 1970 and 22.5 for the past decade, 1971 to 1980. Among Chamorros born on Saipan, the average annual mortality rates were 7.2 and 3.2 per 100,000, respectively, for the two periods. The mortality rates of PD were also significantly lower on Saipan than on Rota. In general, the age‐adjusted mortality rates of ALS and PD on Rota were similar to those currently observed on Guam. Since the origins and current genotypic composition of Chamorros on all the Mariana Islands are indistinguishable, the strikingly lower mortality rates of ALS and PD on Saipan suggest that environmental factors are far more important than genetic factors in the pathogenesis of these diseases.
Effect of a single oral dose of propranolol on essential tremor: A double‐blind controlled study
Authors: Calzetti S. , Findley L. J. , Gresty M. A. , Perucca E. , Richens A.
Published: January 2005

The effect of a single oral dose of propranolol (120 mg) on essential tremor was investigated in a double‐blind, placebocontrolled study in 26 patients. Hand tremor was recorded by means of accelerometers, and its frequency and amplitude calculated by using spectrum analysis. Recordings were made before and 1 1/2 hours after drug or placebo administration. Pretreatment tremor ranged from 4.2 to 9.6 Hz (median, 6.9 Hz) in frequency and from 0.002 to 1.33 cm (median, 0.014 cm) in amplitude. Neither propranolol nor placebo affected the frequency of the underlying tremor. The amplitude of tremor was reduced by 43 ± 11% (SEM) after propranolol (p < 0.01) and by 12 ± 8% after placebo (NS). The reduction observed after propranolol was significantly greater than that observed after placebo. Thre tremor response after propranolol correlated negatively with baseline frequency and positively with pretreatment amplitude, duration of tremor, and age of the patient. No significant relationships could be found between tremor response, serum propranolol levels, and degree of cardiac beta blockade as assessed by the inhibition of standing tachycardia. There was a clear tendency for patients with small tremor amplitude (less than 0.006 cm hand displacement) to show the least satisfactory response to propranolol. These results indicate that a single oral dose of propranolol is effective in producing a rapid and marked reduction of essential tremor. Measurement of pretreatment amplitude and frequency might by useful in predicting the therapeutic outcome in these patients.
No effect from double‐blind trial of physostigmine and lecithin in Alzheimer disease
Authors: Wettstein Albert
Published: January 2005

Eight patients with clinically diagnosed mild to severe Alzheimer disease were treated during two six‐week periods by a double‐blind, single‐crossover protocol with placebo or a combination of 18 gm of lecithin and 3 to 10 mg of oral physostigmine daily. No improvement in behavior, recent memory, or other neuro psychological functions occurred.