bluuebunny/crossref_metadata_2025 huggingface dataset:
In vivo phosphorus nuclear magnetic resonance spectroscopy in status epilepticus
Authors: Petroff Ognen A. C. , Prichard James W. , Behar Kevin L. , Alger Jeffrey R. , Shulman Robert G.
Published: January 2005

Bicuculline‐induced status epilepticus was studied in paralyzed rabbits ventilated with an oxygen and nitrous oxide mixture. An Oxford Instruments TMR 32–200 spectrometer was used to record phosphorus 31 nuclear magnetic resonance spectra of the in vivo brain. An array of conventional physiological variables including the electroencephalogram was simultaneously recorded. Several features were consistently observed during status epilepticus: (1) Phosphocreatine levels fell to about two‐thirds of their control values and remained at that level despite a gradual decline in seizure activity: (2) intracellular pH declined and then remained constant, whereas seizure discharges declined; (3) adenosine triphosphate levels remained constant at their control values. These new, lower levels of brain phosphocreatine and intracellular pH were largely unaffected by increases in seizure activity brought about by elevation of blood pressure from levels too low to support adequate cerebral perfusion, by waning of anticonvulsant drug effect, or by repeated doses of bicuculline.
Peripheral nerve function and metabolic control in diabetes mellitus
Authors: Troni Walter , Carta Quirico , Cantello Roberto , Caselle Maria Teresa , Rainero Innocenzo
Published: January 2005

Measurement of conduction velocity along the H reflex arc was used to study sensorimotor peripheral nerve function in diabetic patients during short‐ and long‐term improvement of hyperglycemia. In ten type I diabetics a slight (p < 0.05) conduction increase occurred after 6 hours of normal glycemia induced by an artificial endocrine pancreas. Similar but more prominent improvement occurred in twelve type I diabetics treated with continuous subcutaneous insulin injection for 6 months. The results suggest that nerve conduction slowing in diabetic patients stems partly from reversible, nonstructural abnormalities and partly from more slowly reversible morphological or chemical changes in peripheral nerve.
Changes in the human vestibulo‐ocular reflex after loss of peripheral sensitivity
Authors: Baloh R. W. , Honrubia V. , Yee R. D. , Hess K.
Published: January 2005

Quantitative rotational testing was used to study changes in the vestibulo‐ocular reflex of patients with unilateral and bilateral peripheral vestibular lesions. Compared with normal subjects, the patients exhibited a characteristic pattern of decreased gain and increased phase lead at low frequencies of sinusoidal stimulation and decreased time constants on impulsive stimulation. By contrast, gain and phase measurements on high‐frequency–low‐amplitude sinusoidal stimulation were often normal. In the patients with bilateral lesions, the results of caloric testing correlated with the results of low‐frequency rotational testing but not with the results of high‐frequency testing. There are two main clinical implications of these findings. First, patients with absent response to caloric stimulation (unilateral or bilateral) may have a normal response to high‐frequency sinusoidal rotation (i.e., the frequencies that constitute most natural head movements). This probably explains why such patients do not report oscillopsia. Second, low‐frequency sinusoidal rotational testing and caloric testing are more sensitive than high‐frequency sinusoidal or impulsive rotational testing for detecting early loss of vestibular sensitivity due, for example, to ototoxic drugs.
Cerebellar atrophy following phenytoin intoxication
Authors: Lindvall Olle , Nilsson Bengt
Published: January 2005

Cerebellar degeneration has been demonstrated in several patients receiving phenytoin therapy. In most cases it has been unclear, however, whether the degeneration was caused by the drug per se or by other mechanisms known to lead to cerebellar damage. We describe a patient who developed a marked cerebellar atrophy, demonstrated on computed tomographic scan, following an episode of acute, severe phenytoin intoxication. The patient received phenytoin prophylactically for 21/2 months after an uncomplicated subarachnoid hemorrhage and was in good health when the treatment was started. He has never had seizures, and no other possible cause of chronic cerebellar changes is known. The initially severe clinical signs of cerebellar dysfunction have subsided slowly. We conclude that phenytoin can directly cause cerebellar degeneration.
Age‐related differences in brain electrical activity of healthy subjects
Authors: Duffy Frank H. , Albert Marilyn S. , McAnulty Gloria , Garvey Arthur J.
Published: January 2005

A combined neurophysiological (electroencephalographic [EEG] and sensory evoked potential) and neuropsychological investigation was performed on 63 healthy men ranging in age from 30 to 80 years. Although alpha frequency diminished slightly with age, neither amplitude nor frequency demonstrated a high age correlation. Alpha blocking, in contrast, did correlate with age, in the direction of reduced alpha reactivity. EEG background activity underwent significant age‐correlated change, consisting of reductions in slow activity and augmentation of fast activity i.e., EEG desynchronization. Previously reported age‐related EEG slowing may be related to the presence of disease in the populations studied. Topographic analysis revealed that the greatest change occurred in the temporal lobes. More change was noted either early or late in the age span, suggesting that aging is a nonlinear process. More features were derived from the right hemisphere than from the left, suggesting that the aging process is not completely symmetrical.
Effect of aging on the accuracy of visually guided saccadic eye movement
Authors: Warabi Tateo , Kase Manabu , Kato Takamasa
Published: January 2005

Changes in oculomotor behaviors with aging were studied in normal young and elderly subjects. Saccadic eye movments induced by presentation of a visual target were analyzed. Elderly subjects commonly showed an elongation of the time to locate the target, accompanied by an increase in reaction times (mean increase, 100 ms) and a decrease in saccadic velocities. The decrease in the velocity was particularly notable when a large‐amplitude saccade was executed. In spite of the slowed motor responses, most elderly subjects preserved the function necessary to execute a correct saccade toward the visual target. The saccadic slowing was accompanied by an increase in saccade duration. Although a longer time was necessary for elderly subjects to locate the target, the accuracy of the initial saccades was not different from that of young subjects. One group of elderly subjects showed extremely long reaction times. These subjects, displaying no abnormal neurological symptoms, were not able to locate the visual target with initial saccades. They had to execute multistep saccades typically seen in patients with degenerative neurological diseases.
Conjugate eye deviation after acute hemispheric stroke: Delayed recovery after previous contralateral frontal lobe damage
Authors: Steiner Israel , Melamed Eldad
Published: January 2005

In 42 patients with acute unilateral ischemic or hemorrhagic hemispheric stroke, conjugate eye deviation toward the lesioned side was usually of brief duration. It subsided within 48 hours in 24 patients (57%) and lasted no longer than 5 days in 38 patients (90%). Early disappearance of conjugate eye deviation was an isolated phenomenon and preceded any improvement in the other focal neurological deficits, which persisted in their initial severity. In 6 additional patients with stroke, conjugate eye deviation was remarkably prolonged, lasting from 13 to more than 43 days. In all 6 there was evidence for preexisting damage to the contralateral frontal region. Our study suggests that early recovery of conjugate eye deviation in patients with acute hemispheric stroke may be mediated by the contralateral unaffected frontal eye field.
Neurological complications of bone marrow transplantation in childhood
Authors: Wiznitzer Max , Packer Roger J. , August Charles S. , Burkey Pa Edith D.
Published: January 2005

Bone marrow transplantation, used in the treatment of cancer, aplastic anemia, and metabolic diseases, involves the use of potentially neurotoxic agents to suppress immunity and eradicate malignancy. Fifty‐seven patients with a median age of 11 years (age range, 6 months to 24 years) underwent bone marrow transplantation at the Children's Hospital of Philadelphia. Fifty‐nine percent developed neurological abnormalities. Twenty‐six patients (46%) had central nervous system (CNS) dysfunction, including infection (8), cerebrovascular accident (5), CNS leukemia (7), metabolic encephalopathy (5), and paraparesis with CNS toxoplasmosis (1). Neuropsychological dysfunction was present in 4 of 5 longterm survivors who were tested. Fourteen of 19 patients (74%) on whom postmortem examination was performed were found to have CNS abnormalities, including cerebral atrophy (10), focal cerebral injury (6), leukemia (5), and infection (3). Fourteen patients (24%) had peripheral nervous system dysfunction. CNS dysfunction was more common in patients with lymphoreticular malignancies. Cerebrovascular accidents (in patients with lymphoreticular malignancies) and infections (in our general population and in patients with lymphoreticular malignancies) occurred more often in our patients than in patients with similar illnesses who did not undergo bone marrow transplantation. The combination of prior treatment and preparative therapy for bone marrow transplantation predisposes patients to neurological and neuropsychological sequelae.
Hippocampal dendritic abnormalities in a rat model of phenylketonuria
Authors: Lacey Daniel J.
Published: January 2005

An increased concentration of dendritic spines was present in one region of the hippocampus in rats made hyperphenylalaninemic (phenylketonuria model). It is uncertain whether this finding resulted from an actual hyperplasia or a failure of maturational spine reduction. The result is unusual in that Golgi studies of most mental retardation syndromes and relevant animal models have shown loss of dendritic spines.
Phenytoin interacts with calcium channels in brain membrances
Authors: Greenberg David A. , Cooper Edward C. , Carpenter Celia L.
Published: January 2005

Phenytoin at concentrations of between 30 and 300 μM inhibited binding of the calcium antagonist {3H} nitrendipine to voltage‐dependent calcium channels in brain membranes. Other anticonvulsants (phenobarbital, carbamazepine, valproic acid, and clonazepam) failed to inhibit binding or did so only at concentration much higher than occur clinically. Calcium channel blockade may be important in the clinical actions of phenytoin, including certain of the adverse effects of the drug.
The role of myelin P2 protein in the production of experimental allergic neuritis
Authors: Rostami Abdolmohammad , Brown Mark J. , Lisak Robert P. , Summer Austin J. , Zweiman Burton , Pleasure David E.
Published: January 2005

Myelin P2 protein has been proposed as the primary antigen in whole myelin‐induced experimental allergic neuritis (EAN). We investigated the neuritogenic properties of P2 by sensitizing Lewis rats with complete Freund's adjuvant (CFA) containing P2, P2 plus phosphatidyl serine, or whole myelin containing an equivalent amount of P2. Animals were examined using a battery of clinical, electrophysiological, immunological, and morphological methods. Myelinimmunized rats developed the characteristic features of EAN. P2‐sensitized rats developed a similar but much less intense disorder. When rats were sensitized with P2 in the presence of phosphatidyl serine, however, they developed radiculoneuropathy that was indistinguishable from myelin‐induced EAN. Inoculation with phosphatidyl serine plus complete Freund's adjuvant or complete Freund's adjuvant alone had no detectable effect on peripheral nerves. These studies demonstrate that sensitization of rats with a single myelin antigen, P2 protein, is sufficient to induce the clinical, electrophysiological, and neuropathological features of EAN.
The aging human brain
Authors: Creasey Helen , Rapoport Stanley I.
Published: January 2005

Postmortem and computed tomographic studies demonstrate many anatomical, morphological, and neurochemical differences between brains of old and young human beings. The variability of the results is great, however, and brains of some old subjects have characteristics of brains of younger controls. Furthermore, important aspects of brain functional activity are not reduced in the elderly. These include resting cerebral oxidative metabolism and “crestallized” intelligence as represented by verbal subtests on the Wechsler Adult Intelligence Scale. In the absence of superimposed disease (which frequently limits aging studies), overall functions can be maintained at high and effective levels because of the plasticity and redundancy capabilities of the human brain.
IgG production within the central nervous system: A critical review of proposed formulae
Authors: Lefvert Ann Kari , Link Hans
Published: January 2005

Demonstration of intrathecal IgG production is employed in the diagnosis of various neurological disorders. This pathological IgG fraction in cerebrospinal fluid (CSF) can be visualized directly as oligoclonal bands by electrophoresis or isoelectric focusing or can be calculated as “excess” or “synthesized” IgG according to different formulae. A comparison of the results obtained with isoelectric focusing and with five formulae showed that even though three of the formulae discriminated well between a reference population and patients with multiple sclerosis, all five gave wrong and misleading results in the presence of blood‐brain barrier damage, as defined by an abnormally raised CSF/serum albumin ratio. A mathematical and statistical evaluation of the different formulae showed only those based on covariance between CSF/serum IgG and CSF/serum albumin to be valid, and these only when values of CSF/serum albumin were normal. Among the five formulae the IgG index (equal to CSF/serum IgG:CSF/serum albumin) is unique in having a comparitively small and constant maximal relative error resulting from the variation coefficients of the IgG and albumin assays. In the case of blood‐brain barrier damage, there exists currently no valid procedure to calculate intrathecally produced IgG; in such instances sensitive electrophoretic or isoelectric focusing methods demonstrating oligoclonal IgG bands are most appropriate to demonstrate intrathecal IgG production.
Cerebrospinal fluid acetylcholinesterase activity in senile dementia of the Alzheimer type
Authors: Tune Larry , Gucker Stephen , Folstein Marshal , Oshida Leslie , Coyle Joseph T.
Published: January 2005

Acetylcholinesterase (AChE) activity was measured in cerebrospinal fluid (CSF) samples from 36 individuals, including 12 persons with Alzheimer's disease, 12 normal controls, and 12 patients with other dementias. AChE activity also was measured in 47 normal subjects whose ages ranged from 20 to 84 to evaluate the effect of age on AChE activity than in age‐matched controls and patients with other dementia syndromes. No correlation was found between duration of illness, age, or severity of illness (as measured by the Mini‐Mental State Examination score) and CSF AChE activity in Alzheimer's disease. AChE increased significantly over the age range of 20 to 84. CSF AChE activity may prove to be a useful diagnostic test to confirm the clinical diagnosis of moderate to severe Alzheimer's disease.
In vivo estimation of muscle protein synthesis in myotonic dystrophy
Authors: Halliday David , Ford G. C. , Edwards Richard H. T. , Rennie Michael J. , Griggs Robert C.
Published: January 2005

The rate of muscle protein synthesis in patients with myotonic dystronic has been studies, and result correlated with total muscle mass. Whole body and skeletal muscle protein synthesis were estimated by stable isotope methodology with a primed, continuous infusion of 1‐[13C]leucine with measurement of incorporation of [13C] leucine into muscle into muscle protein in biospy samples. Whole body leucine flux, protein synthesis, and protein breakdown were only slightly depressed, but muscle protein synthesis was markedly decreased, in myotonic dystrophy. This depression of muscle protein synthesis in myotonic dystrophy correlates with previous observations of impaired insulin‐induced muscle uptake of amino acids and support the suggestion that muscle wasting in this disease is the consequence of defective anabolism in muscle.
Atypical presentations of pyridoxine‐dependent seizures: A treatable cause of intractable epilepsy in infants
Authors: Goutières Françoise , Aicardi Jean
Published: January 2005

We repport on 3 patients with atypical pyridoxine‐dependent seizures. Each had either late onset of convulsions(2 cases)or seizure‐free intervals of up to several months' duration in the absence of pyridoxine supplementation. The findings, taken together with those in 9 previously reported cases, indicate that a trial of pyridoxine shoul be performed in all seizure disorders with onset before 18 months of age, regardless of type.
Local cerebral metabolic rate for glucose during petit mal absences
Authors: Engel Jerome , Lubens Perry , Kuhl David E. , Phelps Michael E.
Published: January 2005

Four patients with primary generalized or true petit mal epilepsy were studied with positron emission tomography using f'{18} fluorodeoxyglucose (FDG). FDG studies were carried out during 10 minutes of hyperventilation before and again after medical control of spontaneous absences. Before seizures were controlled all 4 patients demonstrated frequent bilaterally synchronous three‐per‐second spike‐and‐wave discharges associated with altered consciousness. After spontaneous seizures were controlled, hyperventilation produced only electroencephalographic slowing without clinical symptoms in 3; the fourth patient had absences less frequently. Patterns of local cerebral metabolic rate for glucose (CMRGlc) were normal and identical for ictal and interictal scans; there was, however, a 2.5‐ to 3.5‐fold diffuse ictal increase in global CMRGlc evident when ictal studies were compared with hyperventilation control studies in which no seizures occurred. The CMRGlc was similar in the two scans obtained from the patient who had absences during both studies. No anatomical substrate of petit mal epilepsy was identified. The CMRGlc in these patients during petit mal absences was higher than that recorded in other patients during partial or generalized convulsive seizures. This difference may reflect the fact that petit mal absences are not associated with postictal depression.
Assessment of vestibulo‐ocular reflexes in congenital nystagmus
Authors: Gresty Michael A. , Barratt Hilary J. , Page Nicholas G. R. , Ell Jonathan J.
Published: January 2005

The vestibulo‐ocular reflex and its suppression by fixation of a target rotating with the subject were tested in 18 subjects with congenital nystagmus using steps of constant velocity rotation and sinusoidal stimuli swept in frequency between 0.01 and 1.0 Hz. Responses to stopping stimuli were abnormal in waveform and of short duration in most subjects tested. This pattern was attributed to masking of the response by spontaneous eye movements and to adaptation. In contrast, during both oscillation in the dark and attempted suppression of the vestibulo‐ocular reflex, all subjects had nystagmus that was modulated with the stimulus during all frequencies of stimulation. The phase relationship of the nystagmus to the motion stimulus was the same as in normal subjects. Estimates of gain of the vestibulo‐ocular reflex response were not meaningful because of contamination of the vestibular response by the congenital nystagmus waveforms. Modulation of amplitude and reversal of nystagmus in phase with the vestibular stimulus at all frequencies of oscillation were shown most clearly during attempted suppression of the vestibulo‐ocular reflex. This finding is clinically useful because it establishes suppression as a test of the presence of vestibular function in congenital nystagmus.
Hyponatremia and cerebral infarction in patients with ruptured intracranial aneurysms: Is fluid restriction harmful?
Authors: Wijdicks E. F. M. , Vermeulen M. , Hijdra A. , van Gijn J.
Published: January 2005

We studied retrospectively the relationship between hyponatremia and cerebral infarction in 134 consecutive patients with aneurysmal subarachnoid hemorrhage. In 44 patients sodium levels fell below 135 mmol/L on at least two consecutive days between the second and the tenth day after the hemorrhage. Twenty‐five of these patients fulfilled the criteria for the syndrome of inappropriate secretion of antidiuretic hormane. Cerebral infarction developed in 27 of the 44 patients with hyponatremia and in 19 of the 90 patients with normal serum sodium levels (p < 0.001). Cerebral infarctions were more often fatal in patients with hyponatremia (p < 0.01).

Twenty‐six of the 44 patients had been treated with fluid restriction to correct the serum sodium levels, and infarctions developed in 21. Fluid restriction to correct hyponatremia appears to be potentially dangerous in patients with aneurysmal subarachnoid hemorrhage.
Computed tomographic analysis of brain morphometrics in 30 healthy men, aged 21 to 81 years
Authors: Schwartz Michael , Creasey Helen , Grady Cheryl L. , DeLeo James M. , Frederickson Hal A. , Cutler Neal R. , Rapoport Stanley I.
Published: January 2005

Computed transverse axial tomography (CT) was employed to examine brain morphometrics in 30 healthy men, aged 21 to 81 years. Seven consecutive CT slices 30 to 80 mm above the inferior orbitomeatal line were analyzed. CT numbers in grey and white matter regions were not correlated significantly with age (p<0.05), nor were right/left ratios for derived parameters. The volume of gray matter was correlated negatively with age (p>0.05), and the volume of cerebrospinal fluid correlated positively with age, in the seven slices. The volumes of the lateral and third ventricles were elevated in the elderly, and volumes of the thalamus and tenticular nucleus were reduced. The results demonstrate that brain atrophy, evidenced by a loss of grey matter and by dilatation of cerebrospinal fluid spaces, occurs in the healthy elderly, and provide baseline CT‐derived brain morphometric data for healthy men in relation to age.
The second sensory area in humans: Evoked potential and electrical stimulation studies
Authors: Lüders H. , Lesser R. P. , Dinner D. S. , Hahn J. F. , Salanga V. , Morris H. H.
Published: January 2005

A patient with intractable seizures originating from a right frontal focus was evaluated for surgical treatment. This evaluation was carried out using a chronically implanted array of 96 stainless steel electrodes 1 cm apart and covering the perirolandic and frontal areas. Somatosensory evoked potentials and electrical stimulation of the subdural electrodes localized the primary sensory hand area. Evoked potentials of identical waveform but of lower amplitude and 2.4 ms longer latency were recorded in the inferior frontal gyrus immediately anterior to the face area of the motor strip. Electrical stimulation of that area elicited: (1) a “paralyzing” feeling in the left arm and face; (2) inhibition of rapid alternating movements of left fingers, left hand, and tongue; (3) inability to maintain a strong voluntary muscle contraction of the left hand or tongue; and (4) speech arrest. This appears to be the first report of a secondary sensory area in humans demonstrated by both electrical stimulation and evoked potential studies. Electrical stimulation showed that the secondary sensory area overlapped an area of complex motor control, suggesting that the secondary sensory area provides direct sensory feedback information for appropriate motor integration.
Myophosphorylase deficiency impairs muscle oxidative metabolism
Authors: Haller Ronald G. , Lewis Steven F. , Cook J. D. , Blomqvist C. G.
Published: January 2005

We studied oxidative metabolism during bicycle exercise in 4 patients with myophosphorylase deficiency. Maximal oxygen uptake (VO2max) was low (14.0 ± 1.4ml·kg−1· min−1, mean ± SE) compared with that in normal subjects (37.7 ± 1.9; n = 12) and patients with myalgia (24.9 ± 1.8; n = 10). Carbohydrate oxidation, as estimated by the respiratory exchange ratio (R), was low relative to workload (max R, mean ± SE: McArdle's disease, 0.96 ± 0.02; normal subject, 1.13 ± 0.02; myalgia, 1.09 ± 0.02). Intravenous glucose administration increased maximal oxygen uptake about 20% in those with McArdle's disease, but both VO2max and R remained lower than in control subjects. These findings suggest that the capacity for dynamic exercise in McArdle's disease is limited by the availability of oxidative substrate, and indicate that blood glucose is unable to substitute fully for muscle glycogen as an oxidative fuel. We also found that exercise cardiac output (Q) was excessive relative to oxygen uptake in affected patients (δQ̇/δV̇O2, mean ± SE: McArdle's disease, 11.6 ± 1.7; normal subjects, 48 ± 0.2; myalgia, 5.6 ± 0.2). This hyperkinetic circulation in exercise may serve to increase the delivery of blood‐brone oxidative substrate to working muscle.
Myasthenia gravis: Reactivation of clinical disease and of automimmune factors after discontinuation of long‐term azathioprine
Authors: Hohlfeld Reinhard , Toyka Klaus V. , Besinger Uwe A. , Gerhold Beate , Heininger Kurt
Published: January 2005

In 15 patients with myasthenia gravis who were in stable clinical remission while receiving azathioprine, we monitored disease severity and serial autoantibody titers before and after discontinuation of azathioprine. Cellular immunoreactivity against tuberculin (PPD) and agianst Torpedo acetylcholine receptor (AChR) was measured serially in 11 patients. Eight of 15 patients (53%) had a clinical relapse after 3 to 11 months, necessitating the reinstitution of immunosuppressive treatment in 6 patients. Seven patients have remained clinically stable during an observation period of 20 to 40 months. Anti‐AChR autoantibody titers correlated closely with the clinical course in the majority of patients, and rese markedly in 7 of the 8 patients who relapsed. Cellular stimulation indices correlated less closely with the clinical severity. Only in 3 patients did the clinical score, antibody titer, and cellular stimulation index rise concurrently. In 4 patients who had high cellular stimulation indices after the discontinuation of azathioprine, it was possible to isolate AChR‐reactive inducer/helper T lymphocytes.
Axonal attenuation and secondary segmental demyelination in myeloma neuropathies
Authors: Ohi Takakazu , Kyle Robert A. , Dyck Peter James
Published: January 2005

The sural nerves of 5 patients with osteosclerotic myeloma and polyneuropathy, of 3 patients with multiple myeloma and polyneuropathy, and of 6 healthy subjects were studied by neuropathological, morphomemmtric, and teased‐fiber approaches to assess cellular (Schwann cell or axon) vulnerability and to explore the mechanism of segmental demyelination. As compared with controls, the nerves of both types of myeloma neuropathy demonstrated a statistically significant and marked loss of myelinated fibers and increased frequencies of axonal degeneration among teased fibers at statistically significant levels. The peaks of diameter histograms of myelinated fibers of osteosclerotic myeloma/polyneuropathy nerves were displaced to smaller diameter categories, suggesting fiber atrophy. Segmental demyelination and remyelination was clustered, as found in secondary demyelination. Large‐and intermediate‐diameter myelinated fibers of osteosclerotic myeloma/polyneuropathyl nerves had significantly smaller axon calibers relative to myelin spiral length seen in electron micrographs. The loss of myelinated fiber axons, the shift of the peaks of diameter histograms to smaller sizes, the lack of noticeable increased numbers of demyelinated axons, the clustered distribution of segmental demyelination, and the axonal attenuation suggest a special axonal or neuronal vulnerabilityl and appear to provide an explanation for the observed segmental demyelination. Whether axonal attenuation has a perikaryaeal or proximal genesis now needs to be determined.
γ‐Vinyl GABA: A double‐blind placebo‐controlled trial in partial epilepsy
Authors: Gram Lennart , Klosterskov Peter , Dam Mogens
Published: January 2005

The antiepileptic effect of γ‐vinyl γ‐aminobutyric acid (GABA), an irreversible GABA‐transaminase inhibitor, was investigated in an add‐on, placebo‐controlled, double‐blind, cross‐over, fixed‐dose trial. Twenty‐one patients suffering from difficult to control complex partial seizures participated; 18 patients completed the trial. Serum levels of concomitant antiepileptic drugs were kept constant throughout the trial. Three patients (17%) reduction in seizure frequency and in 8 (44%) the seizures were reduced by at least 50%. Two patients developed a modereat and 1 patient a marked increase in seizure frequency during treatment with γ‐vinyl GABA. Except for 2 patients, who had to discontinue the trial becuase of adverse effects of γ‐vinyl GABA, the participants were unable to discriminate between treatment regimens with regard to side effefts γ‐vinyl GABA seems to be a promosing new antiepileptic drug, and the first one to present convincing evidence of a GABAergic mechanism of action.
Anatomical basis for acquired fluent aphasia in children
Authors: VanDongen Hugo R. , Loonen M. Christa B. , VanDongen Krijn J.
Published: January 2005

Three girls aged 9 to 11 years developed fluent aphasia associated with acute brain lesions. As localized by computed tomography, the abnormalities in all three resided in the posterior part of the left hemisphere, encroaching upon Wernicke's area.
Association between essential tremor and Parkinson's disease
Authors: Geraghty John J. , Jankovic Joseph , Zetusky Walter J.
Published: January 2005

To examine a possinle relationship of essential tremor to Parkinson's disease (PD), 130 patients with essential tremor were studied. Twenty‐five patients had both essential tremor and PD. These 25 patients were matched for age and duration of PD symptoms with 25 patients who had idiopathic PD. Bradykinesia, postural instability, and gait difficulty were more severe in the patients with idiopathic PD. Degree of tremor, rigidity, and fumctional disability did not differ in the two groups. The prevalence of PD in the population with essential tremor was 24 times greater than expected. This study suggests that some patients with essential tremor have genetically increased risk for PD.
Progressive multifocal leukoencephalopathy: Clinical and radiographic features
Authors: Krupp Lauren B. , Lipton Richard B. , Swerdlow Michael L. , Leeds Norman E. , Llena Jose
Published: January 2005

Between April 1982 and March 1984 7 pathologically confirmed cases of progressive multifocal leukoencephalopathy (PML) were diagnosed at our institution. Only 1 case had been seen in the preceding twenty years. Four patients had acquired immunodeficiency syndrome (AIDS). The others had chronic lymphocytic leukemia, Hodgkin's lymphoma and systemic lupus erythematosus. All patients presented with progressive neurological deficits. In most, the initial computed tomographic (CT) scan was disproportionately less abnormal than the clinical findings. In 5 patients the first CT scan revealed hypodensities of the cerebral white matter which lacked mass effect and did not enhance with contrast agent. The lesions were observed to enlarge progressively on CT scans but often lagged behind the rate ofcllinical evolution. Although 3 patients were treated with cytosine arabinoside, none improved. PML had similiar clinical, radiographic, and pathological features in the AIDS and non‐AIDs patients. Of 79 AIDS paatients cared for at our institution between December 1979 and December 1983, 3.8% had PML. PML should be suspected in AIDS patients in the presence of the characteristic CT features, especially when CT‐clinical dissociation occurs.
Reversible, late‐onset disulfiram‐induced neuropathy and encephalopathy
Authors: Borrett D. , Ashby P. , Bilbao J. , Carlen P.
Published: January 2005

Disulfiram toxicity is a well‐recognized cause of peripheral neuropathy and encephalopathy, usually developing within a few months of the start of therapy. We describe a patient who insidiously developed a peripheral neuropathy and encephalopathy after thirty years of disulfiram ingestion. Both complications partially resolved after the medication was stopped.
Infantile cerebellar atrophy
Authors: Furman J. M. , Baloh R. W. , Chugani H. , Waluch V. , Bradley W. G.
Published: January 2005

We descrine a family whose menbers have a dominantly inherited, early‐onset, nonprogressive syndrome that includes spontaneous upbeating nystagmus and mild gait ataxia. Magnetic resonance imaging showed localized atrophy of cerebellar cermis. Several families described in the literature resemble our family but differ in mode of inheritance, age at onset, rate of progression or clinical findings. We believe this family represents a unique type of inherited early‐onste atrophy of the cerebellar vermis.
Brain neurotransmitter abnormalities in neuronal intranuclear inclusion body disorder
Authors: Kish S. J. , Gilbert J. J. , Chang L. J. , Mirchandani L. , Shannak K. , Hornykiewicz O.
Published: January 2005

Biochemical markers for the major neurotransmitter systems measured in the brain of a patients who died with neuronal intranuclear inclusion disease. A severe nigrostriatal dopamine deficiency constitutes the basis for the parkinsonian features in the patient. A marked loss of brain norepinephrine and serotonin was found in basal ganglia and hypothalamus. Brain amino acid (γ‐aminobutyric acid and glutamate) and cholinergic (choline acetyltransferase activity) systems were either normal or less severely affected.
Hypoglycemia: Causes, neurological manifestations, and outcome
Authors: Malouf Renée , Brust John C. M.
Published: January 2005

During a 12‐month prospective study there were 125 visits to the Harlem Hospital Emergency Room for symptomatic hypoglycemia. Sixty‐five patients had obtundation, stupor, or coma; 38 had confusion or bizarre behavior; 10 were dizzy or tremulous; 9 had had seizures; and 3 had suffered sudden hemiparesis. Diabetes mellitus, alcoholism, and sepsis, alone or in combination, accounted for 90% of predisposing conditions; others included fasting, terminal cancer, gastroenteritis, insulin abuse, and myxedema. Average blood glucose level were lower among comatose than among obtunded patients, but overlap was considerable, and overall there was little correlation among cause, blood glucose levels, and symptoms. Although mortality was 11%, only one death was attributable to hypoglycemia per se, and only four survivors had focal neurological residua.
Basal ganglia glucose utilization after recent precentral ablation in the monkey
Authors: Dauth George W. , Gilman Sid , Frey Kirk A , Penney John B.
Published: January 2005

In the macaque monkey, unilateral ablation of areas 4 and 6 of Brodmann result initially in a signficant decrease of glucose metabolic activity in the ipsilateral caudate nucleus, putamen, globus pallidus, substantia nigra, and subthalamic nucleus. The contralateral hemisphere shows nonsignificant but consistently decreased activity in the caudate nucleus, putamen, and globus pallidus. Cerebral blood flow is decreased in the same pattern as the glucose metabolic activity. The change in glucose metabolic activity result from loss of neurons known to project directly from the cerebral cortex to the basal ganglia and also from indirect effect(diaschisis) in basal ganglia structures that do not receive connections from the cerebral cortex.
Nuerological finding in triosephosphate isomerase deficiency
Authors: Poll‐The Bwee Tien , Aicardi Jean , Girot Robert , Rosa R.
Published: January 2005

Two siblings with hemolytic anemia caused by triosephosphate isomerase deficiency developed a progressive neurological syndrome featuring dystonic movements, tremor, pyramidal tract signs, and evidence of spinal motor neuron involvement. Intelligence was unaffected. The findings in these patients and in 14 previously published cases indicate that neurological manifestations are an integral part of the disorder and suggest that specific structures in the basal ganglia, brainstem, and spinal cord bear the brunt of the pathological process, which does not affect the cerebral cortex.
Progressive encephalopathy in children with acquired immue deficiency syndrome
Authors: Epstien Leon G. , Sharer Leroy R. , Joshi Vijay V. , Fojas Myrna M. , Koenigsberger M. Richard , Oleske James M.
Published: January 2005

We are reporting the cases of four children with a diagnosis of acquired immune deficiency syndrome (AIDS) who demonstrated a progressive encephalopathy manifested by loss of motor milestones or intellectual abilities, and weakness with pyramidal tract signs. Two patients were ataxic. One child was cortically blind and suffered myoclonic jerks before his death. Two of the patients had isolated seizures at some time during the disease course. Two patients developed secondary microcephaly and all four patients showed cortical atrophy on computed tomographic scanning. Three of the patients died. Postmortem neuropathological findings included gross cortical atorphy, microglial nodules, and intranuclear inclusions, as are described in the subacute encephalitis reported in adult AIDS patients. We propose that the possible pathogenetic mechanisms of the encephalopathy include opportunistic or persistent viral infection of the brain.
Action of Lambert‐Eaton myasthenic syndrome IgG at mouse motor nerve terminals
Authors: Prior C. , Lang B. , Wray D. , Newsom‐Davis J.
Published: January 2005

We have studied the electrophsiological effects of IgG obtained from four patients with Lambert‐Eaton myasthenic syndrome (LEMS) (two with small cell carcinoma), using the mouse passive transfer model. Mice received LEMS or control IgG or plasma, 10 to 60 mg daily. Microeletrode intracellular recordings were from diaphragm muscle. LEMS IgG and plasma decreased end‐plate potential quantal content similarly, confirming IgG as the active factor. LEMS IgG was equally effective C5‐deficient mice, indicating that late complement components are not required. The time course of decline and recovery of quantal content closely followed that of the human IgG in the mouse serum, with time to half‐maximal effect of about 1.5 days in each case. Binding/dissociation of IgG or down/up regulation of the antigenic determinants, possibly Ca2+ channels, has a half‐life of between 2 and 36 hours. The results confirm our concepts that IgG antibody to nerve terminal determinants underlies the disorder of transmitter release in LEMS.
Leukoencephalopathy in diffuse hemorrhagic cerebral amyloid angiopathy
Authors: Gray F. , Dubas F. , Roullet E. , Escourolle R.
Published: January 2005

We have studied 12 patients with diffuse hemorrhagic cerebral amyloid angiopathy clinically and at postmortem examination. The brains in 8 patients had diffuse bilateral loss of myelin in the hemispheric white matter sparing the U fibers, corpus callosum, and internal capsules. The periventricular areas were predominantly affected. Microscopic examination of the white matter showed an association with subacute or chronic edematous lesions: spongiosis, swollen oligodendroglia, widening of the perivascular spaces with edema fluid or siderophages, hyalinization of the blood vessel walls, incomplete myelin loss, and astrocytic gliosis. Three of 8 autopsied patients had undergone computed tomographic examination, which showed bilateral hypodensity of the hemispheric white matter. The brains of 4 patients with illnesses of shorter duration showed only discrete but similar lesions in the centrum semiovale. These white matter changes are similar to those observed in Binswanger's subcortical encephalopathy. We suggest that a common mechanism of hypoperfusion of the distal white matter causes the leukoencephalopathy.
Metachromatic leukodystrophy manifesting as a schizophrenic disorder: Computed tomographic correlation
Authors: Finelli Pasquale F.
Published: January 2005

A 32‐year‐old woman with a 12‐year history of schizophrenia demonstrated symmetrical bifrontal and biparietal periventricular hypodensities on computed tomographic scan. Sural nerve biopsy and urine and leukocyte enzyme assay confirmed the diagnosis of metachromatic leukodystrophy. The computed tomographic correlate in an adult with metachromatic leukodystrophy in whom the psychiatric manifestations were the predominant clinical feature is described.
Immunofluorescence study of patients with neuropathy and IgM M proteins
Authors: Takatsu Masami , Hays Arthur P. , Latov Norman , Abrams Gary M. , Nemni Raffaello , Sherman William H. , Nobile‐Orazio Eduardo , Saito Toyokazu , Freddo Lorenza
Published: January 2005

Immunofluorescence histochemistry was used to study the pathogenesis of polyneuropathy in patients with an IgM M protein. Seventeen patients had an M protein that reacted with myelin‐associated glycoprotein (MAG), and their serum immunostained myelin sheaths of normal peripheral nerve of humans and certain other species. The staining was specific for the M protein idiotype and was abolished by prior absorption of serum with MAG. The sural nerve biopsy specimens from these 17 patients had pathological features of primary demyelination and deposits of IgM on the myelin sheaths. Sural nerve specimens of 2 patients with an M protein reactive with chondroitin sulfate showed axonal degeneration and diffuse deposits of IgM in the endoneurium. Serum of one of these patients immunostained connective tissue; the staining was specific for the M protein idiotype and was blocked by absorption of the serum with chondroitin sulfate. The antigenic specificity of the IgM M protein in another 9 patients with neuropathy is not known; however, sural nerve specimens obtained from some of the patients showed axonal degeneration and endoneurial deposits of IgM, and the serum IgM immunostained axons in some instances. The findings suggest that IgM M proteins may cause the neuropathy and that more than one autoantigen is involved.
Repetitive involuntary movement associated with episodic cerebral ischemia
Authors: Yanagihara Takehiko , Piepgras David G. , Klass Donald W.
Published: January 2005

Clinical characteristics of 12 patients with carotid occlusive disease who experienced episodes of repetitive involuntary movement were analyzed and correlated with laboratory findings. The majority of patients had rhythmic involuntary movements of one or both extremities on one side, which were characteristically precipitated by standing, walking, or hyperextension of the neck and were promptly alleviated by assuming a sitting or supine position. Six patients had a history of a prior transient ischemic attack or stroke. Electroencephalograms contained no epileptiform activity at rest or during sleep in any patient or during the involuntary movements in 2 patients. Cerebral angiography revealed either occlusion or high‐grade stenosis of the internal or common carotid artery on the side opposite the involuntary movement in all patients. We conclude that the repetitive involuntary movements result from transient hemodynamic ischemic episodes rather than partial (focal) epilepsy.
Isolated angiitis of the central nervous system
Authors: Craven Robert S. , French John K.
Published: January 2005

Angiitis confined to the central nervous system is a rare; disorder in which inflammation and consequent irregular narrowings of medium‐sized cerebral arteries are prominent. Clinical and radiological features in 3 patients with this condition are described. All patients responded to either steroid or steroid plus azathioprine therapy.
Self‐abatement of complex partial seizures
Authors: Prichard Paul B. , Holmstrom Valerie L. , Giacinto Joseph
Published: January 2005

Seven of 71 patients with complex partial seizures claimed they were able to abate their seizures, while none of 18 with simple partial seizures were able to do so. Self‐abatement exercises included highly stereotyped cognitive and physical components unique to the individual. Those who could abate their seizures had attained higher educational status, better social and vocational adjustment, and better psychological adjustment than did the control group of patients with epilepsy. The self‐abatement group was also more likely to have right hemispheric electroencephalographic abnormalities. Characterization of the self‐abatement group may be relevant to the selection of candidates for behavioral therapy for epilepsy.
Status epilepticus in well–oxygenated rats causes neuronal necrosis
Authors: Nevander Gunilla , Ingvar Martin , Auer Roland , Siesjö Bo K.
Published: January 2005

Neuronal necrosis in the brain resulting from status epilepticus of 15 to 120 minutes duration in ventilated and well‐oxygenated rats was assessed. Seizures were induced by inhalation of the convulsant gas flurothyl, and terminated by withdrawal of flurothyl and a single injection of thiopental. The animals were allowed to recover for one week, and neuronal damage was assessed by cell counts following subserial sectioning of the brain and microscopical examination of the sections. Infarction of the pars reticulata of the substantia nigra occurred in 5 of the 6 animals with seizure duration of 30 minutes, and in all animals with longer seizure durations. There also was a common affectation of the central parts of the globus pallidus. The pars compacta of the substantia nigra was never affected. After 45 to 120 minutes of seizures, moderate neuronal necrosis was observed in the neocortex (layers 3 and 4), and after 60 to 120 minutes was seen in amygdaloid and thalamic nuclei, as well as in CA4 and CA1 hippocampal pyramidal cells. Notably, CA3 neurons were not damaged nor were dentate granule cells affected. After 120 minutes of seizures, damage regularly affected the neocortex and the ventral–posterior nuclei of the thalamus. A conspicuous feature was the localization of neuronal necrosis at sites close to the ventricles.
Clinical history, brain metabolism, and neuropsychological function in Alzheimer's disease
Authors: Cutler Neal R. , Haxby James V. , Duara Ranjan , Grady Cheryl L. , Kay Arthur D. , Kessler Robert M. , Sundaram Magesh , Rapoport Stanley I.
Published: January 2005

Data concerning 7 patients with a diagnosis of presumptive Alzheimer's disease (mean age, 65.6 years) are presented in detail in relation to the patients' regional cerebral metabolic rates for glucose. Rates were measured by positron emission tomography with fluorine 18–labeled fluoro‐2‐deoxy‐D‐glucose under conditions of reduced visual and auditory stimulation. A relationship was found between severity of dementia and brain metabolism. In patients with mild to moderate Alzheimer's disease, memory and intellectual deficits were evident without major reductions in absolute metabolic rates, while ratios of regional to whole brain metabolism revealed reductions in regions of the parietal lobes. In the late, severe form of the disease, brain metabolic rates were consistently and significantly reduced. The findings suggest that memory and intellectual deficits are reflected in reductions of brain metabolism in some brain regions in mild to moderate forms of Alzheimer's disease and that, in the late, severe form of the disease, reductions occur consistently throughout the brain.