bluuebunny/crossref_metadata_2025 huggingface dataset:
Cell‐mediated immunity in multiple sclerosis as determined by sensitivity of different lymphocyte populations to various brain tissue antigens
Authors: Brinkman C. J. J. , Nillesen W. M. , Hommes O. R. , Lamers K. J. B. , De Pauw B. E. J. , Delmotte P.
Published: January 2005

Peripheral blood lymphocytes from patients with multiple sclerosis (MS) or other neurological diseases and from healthy individuals were separated by density gradient sedimentation into several subfractions. Individual cell populations were cultured in the presence of several human brain tissue antigens. In comparison to controls, mononuclear cells with a density of ≤ 1.077 gm/cm3 from MS patients displayed a significantly increased sensitivity after incubation with purified human myelin basic protein (MBP) but not with other brain tissue antigens. In particular, the lymphocytes of patients suffering from MS for more than four years reacted positively with MBP, suggesting that the reaction was time dependent. No difference between MS patients and controls in sensitivity to any brain tissue antigen could be detected with cells of lower density (i.e., 1.073 to 1.069 gm/cm3 or ≤ 1.069 gm/cm3). Comparable lymphocyte activity was found to antigens isolated from both MS and control brain tissue. These results suggest that patients with chronic progressive MS have a secondary immune activity to MBP.
Dopaminergic deficiency and delayed visual evoked potentials in humans
Authors: Bodis‐Wollner Ivan , Yahr Melvin D. , Mylin Leland , Thornton John
Published: January 2005

Abnormal delay in visual evoked potential (VEP) could be normalized in 9 of 14 previously untreated parkinsonian patients under the influence of levodopa/carbidopa therapy. In 6 of 11 previously untreated schizophrenic patients the VEP latency became abnormal following therapy with agents known to cause dopaminergic blockade. Besides defects in retrobulbar conduction secondary to demyelination, synaptic dysfunction due to neurotransmitter deficiency or receptor blockade must also be considered as a mechanism underlying VEP delays in humans.
Early proliferative changes in astrocytes in postischemic noninfarcted rat brain
Authors: Petito C. K. , Babiak T.
Published: January 2005

Transient cerebral ischemia in rats was produced by permanent occlusion of the vertebral arteries and 30‐minute occlusion of the common carotid arteries. This model produces ischemic necrosis of neurons in the corpus striatum, cerebral cortex, and hippocampus; infarcts, with necrosis of neuropil, astrocytes, and blood vessels, are rare. Changes in striatal astrocytes at 40 minutes and 3 hours of reperfusion were evaluated by electron microscopy, and quantitative estimates of increases in cytoplasmic and mitochondrial area were performed.

In areas of corpus striatum with moderate ischemic cell change, the percentage of astrocytic nuclei increased from 10.79% in controls to 17.76% at 40 minutes after ischemia (p < 0.01) and 19.86% at 3 hours (p < 0.01). Astrocytic cytoplasm was expanded and contained increased numbers of mitochondria, many of which were pleomorphic and had dilated intracristal spaces and condensed matrix. Rough endoplasmic reticulum was increased. Total mitochondrial area and number of mitochondrial profiles rose significantly in the astrocytic perikarya and foot processes at 3 hours postischemia. The greater number of astrocytes, the increases in mitochondria and rough endoplasmic reticulum and the configurational changes in the mitochondria suggest increased metabolic activity of astrocytes in postischemic, noninfarcted brain.
Chronic mycotic meningitis with spinal involvement (arachnoiditis): A report of five cases
Authors: Stein Sherman C. , Corrado Michael L. , Friedlander Marvin , Farmer Peter
Published: January 2005

Five patients developed mycotic spinal arachnoiditis‐meningitis causing signs and symptoms of spinal cord neoplasm. Four had cryptococcal infection, the fifth had aspergillosis. In three patients, diagnosis was made at surgery; all three developed acute fungal meningitis postoperatively and two died. The diagnosis was made nonsurgically in two patients and was followed by medical cure.

These five and twelve other reported patients with mycotic spinal arachnoiditis shared features that suggested the diagnosis. In contrast to most patients with spinal tumors, those reported here tended to be young (mean age, 32 years), to lack evidence for a primary tumor, and to have a fluctuating history of spinal symptoms for several months. Frequent associated findings were recent pregnancy; the abuse of alcohol, narcotis, or both; and the presence of headache and fever. Plain roentgenogrms of the spine were normal. No single finding was diagnostic, but the combination of several would be rare with spinal tumor.
Seizures induced by thinking
Authors: Wilkins Arnold J. , Zifkin Benjamin , Andermann Frederick , McGovern Enda
Published: January 2005

A patient with generalized convulsions noted that seizures were reliably precipitated by mental arithmetic. The interictal electroencephalogram revealed only a mild, diffuse, nonspecific disturbance, but bursts of generalized epileptiform activity with no obvious clinical expression accompanied efforts at mental arithmetic with a significantly high incidence. Tasks involving multiplication, division, and manipulation of spatial information were significantly associated with discharges, but few, if any, discharges appeared when addition and subtraction tasks of equivalent difficulty were performed. Tasks involving the retention of numerical information in short‐term memory (e.g., immediate repetition of a series of 8 digits) were never associated with paroxysmal EEG activity. It is argued that generalized epilepsy of this kind may be related to focal dysfunction in a manner analogous to the involvement of the occipital lobe in cases of pattern‐sensitive epilepsy.
Long‐term management of respiratory failure in amyotrophic lateral sclerosis
Authors: Sivak Edward D. , Gipson W. Terry , Hanson Maurice R.
Published: January 2005

Present‐day technology has made the in‐home care of patients requiring prolonged mechanical ventilation increasingly common. When this technology is applied to the care of patients with amyotrophic lateral sclerosis, physiological, psychological, and ethical issues must be adequately addressed. Assessment of medical and social factors in six patients, four of whom are still living, indicates that in‐hospital as well as follow‐up evaluation should be carried out in an effort to anticipate the long‐range problems, challenges, and limitations of home care ventilation. Because of increasing availability and simplicity of portable respiratory support devices, the implications of long‐term ventilatory support for some patients with amyotrophic lateral sclerosis and similar illnesses will become more commonly considered in planning long‐term home care.
Dopamine and homovanillic acid concentrations in striatal and limbic regions of human brain
Authors: Walsh Francis X. , Stevens Thomas J. , Langlais Philip J. , Bird Edward D.
Published: January 2005

In an attempt to further define the dopaminergic nature of the limbic nucleus accumbens from the morphologically similar striatal caudate and putamen, the levels of dopamine (DA), homovanillic acid (HVA), and HVA/DA ratios, an index of dopamine turnover, were measured in these three structures of human brain. The levels of dopamine in the accumbens (2.49 ng/mg), caudate (2.39 ng/mg), and putamen (3.00 ng/mg) were similar. The homovanillic acid concentration in the accumbens (7.44 ng/mg) and putamen (6.54 ng/mg) were comparable, while its concentration was considerably lower in the caudatae (3.61 ng/mg). The most striking difference between the limbic accumbens and the striatum was observed in the HVA/DA ratio. This index of turnover was significantly higher in the accumbens (3.64) when compared to the caudate (1.80), and was 59% higher than that found in the putamen (2.53). The data provide evidence for differences in dopamine activity in the mesolimbic versus the nigrostriatal pathways.
Dexamethasone and local cerebral glucose utilization in freeze‐traumatized rat brain
Authors: Pappius H. M.
Published: January 2005

Local cerebral glucose utilization (LCGU) was studied using the 14C‐deoxyglucose method in dexamethasonetreated rats with focal cortical freezing lesions. Widespread depression of LCGU, which developed with time after the lesion in untreated animals, was significantly diminished by dexamethasone (0.25 mg/kg/day) started either 6 to 18 hours before or 4 or 24 hours after the lesion. The effect of dexamethasone was most striking in cortical areas of the traumatized hemisphere, where the depression was most profound in untreated animals. Thus, three days after the lesion, average LCGU in these regions was 47% and 72% of normal in untreated and pretreated rats, respectively. Dexamethasone also affected LCGU bilaterally in subcortical structures and in white matter. The results suggest that dexamethasone modified the widespread depression in functional state of the rat brain that developed in response to injury. Since the spatial distribution and time course of the observed changes in LCGU did not parallel those of cerebral edema, these effects of dexamethasone do not appear to be mediated by effects on the edematous process.
Decreased serum 24, 25‐dihydroxyvitamin D concentration during long‐term anticonvulsant therapy in adult epileptics
Authors: Zerwekh Joseph E. , Homan Richard , Tindall Richard , Pak Charles Y. C.
Published: January 2005

Serum concentrations of 25‐hydroxyvitamin D (25‐OHD), 24, 25‐dihydroxyvitamin D [24, 25‐(OH)2D], and 1α, 25‐dihydroxyvitamin D [1, 25‐(OH)2D] were measured in 30 ambulatory adult epileptic patients during long‐term anticovulsant treatment with phenytoin, phenobarbital, or carbamazepine. For the entire group, serum 24, 25‐(OH)2D was decreased (p < 0.0005) as compared to normal subjects to a mean value of 0.7 ± 0.1 (SEM) ng/ml. However, serum 1, 25‐(OH)2D was increased at 50 ± 7 pg/ml (p < 0.025). Serum 25‐OHD declined insignificantly to 19 ± 3 ng/ml. All three drugs caused a significant reduction of serum 24, 25‐(OH)2D concentrations. A significant decrease in serum 25‐OHD was observed only for the phenobarbital‐treated patients. Serum 1, 25‐(OH)2D was high in patients receiving phenytoin or carbamazepine but not in those taking phenobarbital.

The finding suggest that while various anticonvulsant drugs appear to exert different effects on vitamin D metabolism, a universal finding is diminished serum 24, 25‐(OH)2D. The results support the notion that 24, 25‐(OH)2D deficiency may play an important role in the pathogenesis of anticonvulsant‐induced osteomalacia.
Alcohol and Parkinson disease
Authors: Lang A. E. , Marsden C. D. , Obeso J. A. , Parkes J. D.
Published: January 2005

A survey of the drinking habits of 125 patients with idiopathic Parkinson disease showed no difference from a control population in the amount of alcohol consumed and only mild effects of alcohol on parkinsonian symptoms in a small percentage (16%) of these patients. A case of parkinsonism provoked by alcohol withdrawl is also reported, and the complex effects of alcohol on function of the basal ganglia are briefly reviewed.
Neurofibrillary axonal pathology in aluminum intoxication
Authors: Troncoso Juan C. , Price Donald L. , Griffin John W. , Parhad Irma M.
Published: January 2005

Aluminum chloride injected into the cisterna magna of 3‐to 4‐week‐old rabbits is known to produce neurofibrillary changes in the perikarya and dendrites of neurons of the spinal cord and brainstem. This report calls attention to the early appearance and prominence of neurofibrillary swellings in proximal axons of affected neurons. One and 2 days after intoxication, large axonal swellings containing maloriented neurofilaments were present in the ventral horns. Over the next days and weeks, neurofilaments accumulated in the neuronal perikarya and dendrites. Two months after injection, abnormalities in the cell bodies were less apparent but occasional giant axonal swellings persisted in the ventral horn. This sequence of pathological changes, reflecting disorganization of the neuronal cytoskeleton, may result from an increase in synthesis of neurofilament polypeptides, and impairment in integration of these constituents into the neuronal cytoskeleton, or a defect in the intracellular transport of neurofilaments.
Peripheral nervous system complications of coronary artery bypass graft surgery
Authors: Lederman Richard J. , Breuer Anthony C. , Hanson Maurice R. , Furlan Anthony J. , Loop Floyd D. , Cosgrove Delos M. , Estafanous F. George , Greenstreet Richard L.
Published: January 2005

Among 421 patients undergoing coronary artery bypass graft surgery, 55 (13%) developed 63 new peripheral nervous system (PNS) complications postoperatively. Most common was a brachial radiculoplexopathy, which occurred in 23 patients. Of these, 21 involved lower trunk or medial cord fibers. In 17 there was a correlation between the site of jugular vein cannulation and the side affected, suggesting that needle trauma played a role. Stretching from chest wall retraction may have caused some cases. Other deficits included 13 saphenous, 8 common peroneal, and 5 ulnar mononeuropathies. Six patients had persistent singultus, suggesting phrenic nerve involvement. Unilateral vocal cord paralysis was found in 5. An isolated partial Horner syndrome and a facial neuropathy were also identified. Males were more likely to develop PNS complications. Hypothermia during surgery was associated with increased risk. Most PNS deficits were transient, and lasting disability was rare.
Primitive reflexes and cognitive function in the elderly
Authors: Koller William C. , Glatt Sander , Wilson Robert S. , Fox Jacob H.
Published: January 2005

Primitive reflexes were studied in patients with presumed Alzheimer disease and in age‐matched nondemented controls. A snout reflex occurred with equal frequency (54%) in the two groups. An abnormal glabellar reflex was elicited more often in the demented patients (23%) than the controls (8%). The presence of a snout reflex was directly correlated with increasing age; the glabellar reflex showed a similar but nonsignificant trend. Neither reflex finding correlated with the presence of apparent cerebral atrophy by CT scan or the results of psychometric tests. The finding of primitive reflexes has little clinical value in the evaluation of the elderly.
Local cerebral glucose utilization in the newborn macaque monkey
Authors: Kennedy C. , Sakurada O. , Shinohara M. , Miyaoka M.
Published: January 2005

The [14C]dexyglucose method for quantitative determination of local cerebral glucose utilization was employed in newborn macaque monkeys. Values ranged from a high of 180 μmol/100 gm/min in the inferior colliculus to a low of 14 μmol/100 gm/min in the centrum ovale, a wider range than had been found in this laboratory for pubescent monkeys. Relatively low values in white matter overlapped with the generally higher values in gray matter. Rates for structures in the lower neuraxis were equal to or exceeded those reported for the mature animal, while those above the midbrain were generally lower. The auditory system was unique in having rates equal to, or exceeding, its mature levels in all parts of the pathway including the cortex. In the visual system, subcortical structures were at their mature levels while rates in the cortical areas were variably low; nevertheless, the striate cortex had differential rates marking the various laminae. It was possible to demonstrate the metabolic counterpart of the ocular dominance columns in two newborn animals that had had one eye occluded during the experiment. Rates for white matter were above their mature levels, with the differences being greatest in the brainstem and cerebellum. A high correlation between glucose utilization and local blood flow measured with [14C]antipyrine (r = 0.92) was found.
Dopaminergic dysfunction in tourette syndrome
Authors: Singer Harvey S. , Butler Ian J. , Tune Larry E. , Seifert William E. , Coyle Joseph T.
Published: January 2005

A prospective clinical and biochemical study on the effects of treatment with haloperidol has been performed in seven patients with Tourette syndrome. Pretteatment cerebrospinal fluid levels of homovanillic acid (CSF HV A) were significantly reduced in all patients, whereas 5‐hydroxyindoleacetic acid was reduced in only two. With haloperidol treatment, symptoms decreased in all cases (21 to 88%) and clinical improvement was associated withan increased level of CSF HV A, often returning to the normal range. Optimal therapeutic response was found with serum levels of haloperidol between 1 and 4 ng/ml; however, disturbing side effects also occurred within this range. These results support the hypothesis that Tourette syndrome may result form a supersensitivity of dopaminergic receptors.
Locked‐in syndrome in multiple sclerosis with sparing of the ventral portion of the pons
Authors: Forti Alberto , Ambrosetto Giovanni , Amore Mario , De Maria Roberto , Michelucci Roberto , Omicini Ebe , Rizzuto Nicola , Fenzi F. , Tassinari Carlo Alberto
Published: January 2005

“Locked‐in” syndrome was observed in a 42‐year‐old woman with multiple sclerosis. Autopsy revealed extensive demyelination in both cerebral peduncles, the periaqueductal region, and the internal capsule, but the ventral portion of the pons was spared.
Inflammatory facioscapulohumeral muscular dystrophy and coats syndrome
Authors: Wulff John D. , Lin James T. , Kepes John J.
Published: January 2005

We report a case of facioscapulohumeral muscular dystrophy and Coats syndrome. This association has been reported only once before. Three muscle biopsies from our patient over 13 years showed changes of a slow inflammatory myopathy. Corticosteroid treatment did not improve his clinical status. We postulate that our case may be a variant of facioscapulohumeral muscular dystrophy.
Neurobiological correlates of learning disabilities
Authors: Golden Gerald S.
Published: January 2005

Most investigator now agree on the existence of abnormalities of brain function in children with learning disabilities with or without hyperactivity. However, attempts to define the neurobiological substrates have been hindered by many factors. On the clinical level, learning disability is not a monolithic entity but an operational term used to define a number of specific conditions. The only common feature is difficulty with school performance in the face of normal intelligence. Until homogeneous subgroups can be defined, there is no good reason to suspect a single, discrete biological marker.

Neuropathological studies are limited to 2 cases. Attempts to visualize the brain by computerized tomography have provided limited but inconsistent evidence of variations from normal hemispheric widths and asymmetries. Neurophysiological studies have succeeded in separating learning disabled children from normal controls by the application of discriminant analysis and other statistical techniques. Computer‐assisted analysis of brain bioelectrical activity has sharpened these tools. Unfortunately, the techniques have not been able to narrow the focus to single, discrete cerebral areas; rather, they have shown dysfunction in widely separated but probably interconnected regions.

The only neurochemical studies available have been performed on hyperactive childŕen rather than on patients with uncomplicated learning disabilities. A hypothesis that the underlying pathophysiology involves dopamine depletion has the most support and provides an explanation for some of the therapeutic effects of psychostimulant drugs. Problems are the focus on motor aspects of hyperactivity rather than the attention deficit and heavy dependence on the use of clinical rating scales. The biochemical analyses and conclusions would be more powerful if better psychophysiological markers could be developed, including such factors as attentiveness, distractability, vigilance, attention span, and reaction time.

The results of physical and neurological examinations as well as of clinical genetic studies also point toward a biological basis for learning disabilities. These clinical tools help to sharpen our focus but still provide insufficient specificity. Productive search will require close collaboration between neuropsychologists and investigators using the quantitative tools of neuroanatomy, neurophysiology, and neurochemistry.
Imparied biotin status in anticonvulsant therapy
Authors: Krause Klaus‐Henning , Berlit Peter , Bonjour Jean‐Pierre
Published: January 2005

In 264 epileptics undergoing long‐term therapy with anticonvulsants, significantly reduced plasma biotin levels were found compared with a normal control group: 74% of the epileptics had biotin levels for those treated with sodium valproate were higher than for those treated with phenytion, primidone, or carbamazepine. The observed reduction in biotin levels might be a factor influencing the efficacy of these three antionvulsants.
The origin of fasciculations
Authors: Roth G.
Published: January 2005

A collision method in a single axon was used to determine the origin of 100 fasciculations in various lower motor neuron lesions. For 80% of the fasciculations, it was situated on the distal extremity of the axon regardless of the type of lesion, its duration, or the severity of the denervation. For several reasons, this proportion is underestimated.

The fasciculations of distal origin were sometimes associated with double discharges, the mechanism of production and clinical significance of which probably differ.
Evoked potential abnormalities in the various inherited ataxias
Authors: Nuwer Marc R. , Perlman Susan L. , Packwood James W. , Kark R. A. Pieter
Published: January 2005

Visual (VEP), brainstem auditory (BAEP), and somatosensory (SEP) evoked potential tests were performed in 45 patients representing ten types of inherited disorders in which ataxia was the most prominent symptom. Comparable VEP abnormalities were present among all types of patients. Normal BAEP tests were recorded in most patients except those with olivopontocerebellar atrophy. SEP results were often more severely abnormal in patients with Friedreich's ataxia. The observations emphasize the similarity in expression of different metabolic‐degenerative disorders. When these tests are used clinically, certain features of evoked potentials (especially left‐right symmetry) are typical of the inherited ataxias as a group. Few distinguishing features differentiate the individual disorders.
Blood flow changes in arteriovenous malformation during behavioral activation
Authors: Deutsch Georg
Published: January 2005

Striking task‐dependent fluctuations were observed in the cerebral blood flow pattern of a patient with a left posterior hemispheric arteriovenous malformation (AVM). Two‐dimensional measures of regional cerebral flwo in the resting state, using the xenon 133 inhalation technique, revealed a region of high flow coincident with the AVM seen on the patient's arteriograms. In subsequent studies, the AVM stood out as a region of high blood flow during a relaxed state, while it approached normal levels of flow when there was attentional demand. These observations suggest that focal regulatory mechanisms exist at the AVM or else that very substantial redistributions of blood flow are taking place which the flow rate in the AVM reflects only passively. Patients considered for embolic treatment of an AVM would benefit from an assessment of behavioral influences on flow in the AVM.
Bromocriptine and lisuride in Parkinson disease
Authors: Lieberman Abraham N. , Gopinathan Govindan , Neophytides Andreas , Leibowitz Morton , Walker Russell , Hiesiger Emil
Published: January 2005

Lisuride was compared with bromocriptine in 25 parkinsonian patients in whom the response to levodopa had diminished; 19 had “wearing off,” “on‐off” phenomena, or both. At the time bromocriptine was added to levodopa, the mean age of the patients was 62.7 years and mean disease duration was 8.9 years. Disability decreased by 34% in the on period and by 20% in the off period, and the number of hours the patients were on increased from 9.6 to 12.8. All these changes were significant (p ≤ 0.01 to 0.05). Bromocriptine, however, had to be discontinued in 11 patients because of adverse effects. In the remaining 14 patients, bromocriptine was eventually discontinued because of decreased efficacy. Mean dose of bromocriptine was 55 mg (range, 20 to 100 mg).

At the time lisuride was added to levodopa the patients were older (65.4 years), had had the disease longer (11.4 years), and were more disabled. Nonetheless, disability decreased in the on period by 33% and in the off period by 17%, and the number of hours the patients were on increased from 3.9 to 8.9. All these changes were significant (p ≤ 0.01 to 0.05). The mean dose of lisuride was 2.8 mg (range, 0.6 to 5.0 mg). Lisuride was discontinued in 8 patients because of adverse effects.

Both bromocriptine and lisuride are useful in managing patients with advanced Parkinson disease whose response to levodopa has diminished. While it is presently not possible to state which of the drugs is more effective, ultimately their usage will probably be determined by their relative cost.
Relapsing herpes simplex encephalitis following antiviral therapy
Authors: Davis Larry E. , McLaren Laroy C.
Published: January 2005

Although treatment for herpes simplex virus (HSV) encephalitis with antiviral agents has improved survival, occasional patients experience unexplained clinical exacerbations. This report presents evidence that some relapses may occur from recurrent viral encephalitis.

An adult male developed the classic symptoms of HSV encephalitis. The cerebrospinal fluid (CSF), electroencephalogram, and isotope brain scan suggested a localized encephalitis involving the left temporal lobe. The pateint was treated for 10 days with high doses of cytosine arabinoside instead of the currently recommended adenine arabinoside. Progression of encephalitis stopped, and clinical recovery occurred. The HSV antibody titer increased eightfold. Fifty‐four days after the initial encephalitis, the patient relapsed with a subacute progressive encephalitis involving the same brain area. The CSF demonstrated oligoclonal bands, elevated immunoglobulin G levels (100 mg/dl), and a high HSV antibody titer (1:8,192 by indirect hemagglutination test). From a left temporal lobe biopsy taken 74 days after onset of the initial encephalitis, herpes simplex virus type 1 was isolated. Without renewed antiviral drug therapy, the patient slowly recovered.
Case of downbeat nystagmus influenced by otolith stimulation
Authors: Chambers B. R. , Ell J. J. , Gresty M. A.
Published: January 2005

A patient with downbeat nystagmus and familial ataxia is described. The nystagmus was induced by static tilt away from normal upright posture, by linear acceleration of the head, and by convergence. It is inferred that the nystagmus was modulated by otolith‐specific stimuli and not by stimulation of the canals. These findings demonstrate the role of otolith function in generation of eye movements in the vertical plane and support proposed interrelationships between otolith and vergence mechanisms. The nystagmus and associated oscillopsia were partially suppressed by treatment with clonazepam.
Basal forebrain neurons in the dementia of Parkinson disease
Authors: Whitehouse Peter J. , Hedreen John C. , White Charles L. , Price Donald L.
Published: January 2005

Demented patients with Parkinson disease share certain neuropathological and neurochemical features with patients suffering from Alzheimer disease. Recently, loss of cholinergic neurons in the basal forebrain, particularly the nucleus basalis of Meynert, has been implicated in the pathophysiology of Alzheimer disease. The present investigation of 12 patients with Parkinson disease demonstrates that the demented patients with this disease also show a selective loss of cells in the nucleus basalis of Meynert, thus providing an important link between the dementias of Alzheimer disease and Parkinson disease.
Chronic experimental allergic encephalomyelitis produced by bovine proteolipid apoprotein: Immunological studies in rabbits
Authors: Cambi Franca , Lees Marjorie B. , Williams Rosemarie M. , Macklin Wendy B.
Published: January 2005

A chronic experimental allergic encephalomyelitis (EAE) has been produced in rabbits sensitized with bovine white matter proteolipid apoprotein. Eleven of 12 animals developed clinical disease one to six months after immunization with a single dose of the apoprotein. The clinical course was characterized by posterior ataxia, flaccid paralysis progressing to spastic paralysis, and incontinence. Spontaneous relapses and remissions were observed in 3 rabbits. Histologically, acute and chronic encephalomyelitis accompanied by primary demyelination were observed. Serumantibody production, assayed by both an enzyme‐linked immunosorbent assay and an electroblot procedure, did not correlate with either the clinical course or the histopathological findings. Delayed hypersensitivity to proteolipid apoprotein was observed in all rabbits prior to the onset of clinical signs. The data suggest that lymphocytes specifically sensitized to the proteolipid may be involved in the pathogenesis of the demyelination in chronic EAE.
Cerebral emboli of paradoxical origin
Authors: Jones H. Royden , Caplan Louis R. , Come Patricia C. , Swinton Neil W. , Breslin Donald J.
Published: January 2005

A diagnosis of paradoxical cerebral embolus (PCE) was made in five patients aged 31 to 62 years who sustained eight cerebral ischemic events. No patient had evidence of primary carotid system or left heart disease. A probe‐patent foramen ovale was the presumed mechanism in four patients, and an unsuspected congenital atrial septal defect was found in the fifth patient. Clinically apparent pulmonary emboli or venous thrombosis preceded the cerebral event in only one instance.

Review of the literature reveals a high mortality with PCE. However, careful clinical search for this lesion may be rewarding: four of our five patients survived. One should consider PCE in any patient with cerebral embolus in whom there is no demonstrable left‐sided circulatory source. This principle applies particularly if there is concomitant venous thrombosis, pulmonary embolism, or enhanced potential for venous thrombosis due to, for example, morbid obesity, use of hormonal birth control pills, prolonged bed rest (especially postoperatively), or systemic carcinoma.
Friedreich ataxia and low pyruvate carboxylase activity in liver and fibroblasts
Authors: Dijkstra U. J. , Willems J. L. , Joosten E. M. G. , Gabreëls F. J. M.
Published: January 2005

Biochemical studies in liver, muscle, and cultured fibroblasts were carried out in seven patients with Friedreich ataxia. Lowered activity of pyruvate carboxylase was shown in liver and cultured fibroblasts in all instances.
Normal vitamin E status in spinal muscular atrophy
Authors: Sokol Ronald J. , Iannaccone Susan T.
Published: January 2005

In an attempt to confirm prior reports of vitamin E deficiency in Werdnig‐Hoffmann disease, we assessed vitamin E nutritional status in 11 children and 6 adults with spinal muscular atrophy (SMA). Serum vitamin E levels (10.6 ± 3.3 m̈g/ml for those less than 12 years old, 13.2 ± 4.5 for those older than 12) and the ratio of serum vitamin E to total serum lipids were normal in all patients. Vitamin E nutritional status appears to be normal in SMA, and vitamin E therapy is not indicated.
Incidence and treatment of peripheral venous thrombosis in patients with glioma
Authors: Ruff Robert L. , Posner Jerome B.
Published: January 2005

The incidence, prevention, and treatment of peripheral venous thrombosis were studied retrospectively in 381 patients with malignant glioma. Of 264 patients who did not receive antithrombotic prophylaxis, 97 (36.7%) developed clinical phlebitis confirmed by venography. Sixty‐six cases occurred within 6 weeks of craniotomy. By contrast, only 12 (10%) of 117 patients who received intermittent pneumatic pressure to the calves during cranictomy developed phlebitis (4 patients within 6 weeks of the surgery). Of the 109 patients with venous thrombosis, 103 were treated with anticoagulants. Of the 6 patients treated conservatively, 3 died of pulmonary emboli. Intracranial hemorrhage occurred in 1.9% of the patients taking anticoagulants and in 2.2% of those who did not develop phlebitis. We conclude that patients with malignant gliomas have a high risk of developing peripheral venous thrombosis; that antithrombotic therapy reduces the incidence of thrombosis following craniotomy; and that, in patients who develop phlebitis, anticoagulation reduces the risk of pulmonary emboli without increasing the risk of intracranial hemorrhage.
β‐Galactosidase–neuraminidase deficiency (galactosialidosis): Clinical, pathological, and enzymatic studies in a postmortem case
Authors: Sakuraba Hitoshi , Suzuki Yoshiyuki , Akagi Masao , Sakai Masao , Amano Naoji
Published: January 2005

Three male siblings in a Japanese family were affected with β‐galactosidase–neuraminidase deficiency (galactosialidosis). One patient died at 45 years of age, and postmortem liver and brain tissues were studied enzymatically. The residual activity of neuraminidase was relatively high in these tissues. Neuraminidase activity did not change in the tissues after repeated freezing and thawing of the homogenates, whereas this enzyme in control tissues lost 30 to 60% of its activity. There was a profound deficiency of β‐galactosidase in the tissues of the patient. Michaelis constant, pH profiles, and cryostability were identical for the patient and control patients. Cathepsin B was moderately increased in activity in the patient's tissues.
Segmental demyelination and remyelination in lumbar spinal root of patients dying with diabetes mellitus
Authors: Ohnishi Akio , Harada Masayoshi , Tateishi Jun , Ogata Jun , Kawanami Sachiko
Published: January 2005

Morphometric evaluations of histopathological changes in postmortem materials from three patients with diabetes mellitus with neuropathy, nephropathy, and retinopathy were made on the sural nerve, lumbar spinal roots, lumbar dorsal root ganglion (2 cases), and fasciculus gracilis. In all three patients there was a marked decrese in densities of both the large and the small myelinated fibers in the sural nerves. In the lumbar spinal roots, segmental demyelination and remyelination with or without decrease in the number of myelinated fibers per root was the main finding in both dorsal and ventral roots, being more common in the dorsal roots. At the third cervical segment of the the fasciculus gracilis, the myelinated fiber density was slightly decreased in one patient and moderately decreased in the other two. The number of cell bodies of the fifth dorsal root ganglion was within normal limits in the two patient where this was examined, although the median diameters approximated the lower limit found in controls. The potential presence of segmental demyelination and remyelination in dorsal and ventral spinal roots should be considered especially when assessing electromyographic changes and nerve conduction in diabetic patients.
Computed tomography in acute disseminated encephalomyelitis
Authors: Lukes Steven A. , Norman David
Published: January 2005

Computed tomographic (CT) scans were obtained for eleven patients with acute disseminated encephalomyelitis (ADE). Four patients had normal CT scans despite repeated examination. Abnormalities were found in seven patients and included cortical‐enhancing lesions, low‐density lesions in the deep white matter and basal ganglia, and edema of the brainstem. These findings support the hypothesis that both vascular injury and demyelination are involved in the pathogenesis of ADE. A delay between the onset of clinical signs and the appearance of lesions on CT scan was common. Clinical improvement was accompanied by improvement in CT abnormalities. There was a limited correlation between the clinical course and the anatomical distribution and type of abnormality seen on CT scan.
Downbeat nystagmus with a pseudocycloid waveform: Improvement with base‐out prisms
Authors: Lavin P. J. M. , Traccis S. , Dell'Osso L. F. , Abel L. A. , Ellenberger C.
Published: January 2005

Downbeat nystagmus in primary position and oscillopsia resulted from nutritional deficiency during prolonged intravenous therapy of a patient with hyperemesis gravidarum. Wide bandwidth infrared oculography demonstrated a pseudocycloid nystagmus waveform with an increasing‐velocity exponential slow phase. Because the oscillopsia decreased and the nystagmus was damped with convergence, visual acuity improved with the addition of base‐out prisms to each spectacle lens.
Superior oblique myokymia
Authors: Rosenberg Michael L. , Glaser Joel S.
Published: January 2005

Superior oblique myokymia is an unusual eye movement phenomenon generally considered to be a uniocular rotary microtremor easily treated with carbamazepine. Eleven new cases are presented, one with a unique combination of motility problems, emphasizing that the disorder may have varying clinical manifestations. Long‐term follow‐up of the patients revealed a natural history of recurrent spontaneous remissions and relapses. Treatment with carbamazepine was of short‐term benefit in most patients but failed to affect the long‐term course of the disease. No patient developed other evidence of neurological disease. The condition seems to be a benign, permanent condition limited in its manifestations to the superior oblique muscle.
Lithium interferes with reserpine‐induced dopamine depletion
Authors: Reches Avinoam , Hassan Mohamed N. , Jackson Vernice , Fahn Stanley
Published: January 2005

Long‐term lithium treatment attenuated the hypokinetic effect of reserpine in a patient with tardive dyskinesia. In rats, prophylactic lithium administration inhibits reserpine‐induced dopamine depletion in the brain. These data indicate that lithium may limit the therapeutic efficacy of reserpine in tardive dyskinesia.
Delayed onset of post–rabies vaccination encephalitis
Authors: Gamboa E. T. , Cowen D. , Eggers A. , Cogan P. , Ganti R. , Brust J. C. M.
Published: January 2005

A 31‐year‐old veterinarian developed seizures, left hemiparesis, loss of memory, and behavioral disorders 5 months after intensive antirabies vaccination, the longest incubation period yet recorded. Computed tomographic scan revealed a right frontal contrastenhanced mass that extended to the left frontal lobe through the corpus callosum. Brain biopsy showed foci of primary demyelination largely confined to the white matter. The lesions were characteristic of the demyelinating encephalomyelitis that follows treatment with certain vaccines against rabies. The hemiplegia improved, but seizures, memory impairment, and abnormal behavior persisted.
Saccadic intrusions in internuclear ophthalmoplegia
Authors: Herishanu Yuval O. , Sharpe James A.
Published: January 2005

Saccadic intrusions disrupted forward fixation in four patients with bilateral internuclear ophthalmoplegia (INO). Three had multiple sclerosis and one had brainstem infarction. Sporadic bursts of monocular abducting saccades were recorded in either eye. Each saccade was followed by a slower return to midposition. The presence of the INO prevented conjugate saccades of the fellow eye. The adducting phases were faster than convergence eye movements or the glissadic smooth eye movements that sometimes follow saccades. Dynamic overshoot of the abducting saccadic phase may contribute to the relatively rapid adducting phase. The saccadic intrusions of INO are recognized clinically as monocular horizontal oscillation; low‐amplitude intrusions are evident only during funduscopy. This distinctive dyskinesia is considered a sign of involvement extrinsic to the medial longitudinal fasciculi in patients with INO.
Brainstem glioma: Clinical manifestations of meningeal gliomatosis
Authors: Packer Roger J. , Allen Jeffrey , Nielsen Surl , Petito Carol , Deck Michael , Jereb Berta
Published: January 2005

Brainstem gliomas of children are variably malignant tumors that rarely have been reported to produce subarachnoid dissemination. Nevertheless, during a two‐year period, 5 of 15 such patients treated by us developed symptoms of leptomeningeal metastases. The diagnosis of an anaplastic astrocytoma with meningeal gliomatosis was confirmed postmortem in all 5. In 3 children, meningeal symptoms preceded other signs of posterior fossa recurrence. Symptoms of meningeal gliomatosis included local or radiating back pain (5 patients), segmental weakness (3), paresthesia (2), and incontinence (2). Myelography, performed in 4 patients, was the most useful diagnostic technique, disclosing multiple intradural filling defects or a high degree of block in 3 patients. Although the cerebrospinal fluid was abnormal in all 4 examined patients, in only 1 were malignant cells detected. Prolonged survival, which appears to predispose to dissemination of adult malignant gliomas, was not an apparent factor in our patients.
Congenic autoimmune murine models of central nervous system diseas in connective tissue disorders
Authors: Alexander Elaine L , Murphy Edwin D. , Roths John B. , E Garrett E.
Published: January 2005

Congenic mice of the MRL/Mp strain spontaneously develop an autoimmune connective tissue disease that shares immunological and histopathological features with systemic lupus erythematosus, rheumatoid arthrithritis, and Sjögren's syndrome. The autoimmune disorder in these mice is accelerated markedly by the recessive gene lpr. By 6 month of age, MRL/Mp‐lpr/lpr mice developed prominent mononuclear cell infiltrates restricted to the choriod plexus and meninges, whereas congeneric MRL/Mp‐+/+ mice (which lack the lpr gene) showed delayed but widespread inflammatory infiltrates involving cerebral vessels and meninges, with sparing of the choroid plexus. These distinctive patterns of cerebral inflammation, which are comparable in many respects to those seen in human connective tissue disease, provide some of the first animal models of relevant central nervous system histopathological processes associated with underlying connective tissue disease.
Alzheimer's disease: Choline acetyltransferase activity in brain tissue from clinical and pathological subgroups
Authors: Bird T. D. , Stranahan S. , Sumi S. M. , Raskind M.
Published: January 2005

Choline acetyltransferase activity was measured postmortem in five brain regions to determine if such activity provided biochemical support for clinical and pathological subgrouping of Alzheimer's disease. Sixteen patients with Alzheimer's disease were divided into groups based on age at onset, severity of neuropathological changes, history of myoclonus, family history of dementia, cerebellar amyloid plaques, and congophilic angiopathy. Thirty‐two age‐matched normal control subjects and 17 neurological control patients with Huntington's disease were also studied. Patients with early‐ onset and late‐onset Alzheimer's disease did not differ in the clinical duration of their disease. Choline acetyltransferase activity was significantly lower in patients with early‐onset Alzheimer's disease than in age‐matched control subjects in frontal cortex, temporal cortex, hippocampus, and cerebellum. In contrast, choline acetyltransferase activity in patients with late‐onset Alzheimer's disease was significantly lower than in age‐matched control subjects only in hippocampus. There was a tendency for choline acetyltransferase activity to be lower in cortex from patients with early‐onset Alzheimer's disease compared with cortex from the late‐onset group, and this difference was significant in temporal cortex. Choline acetyltransferase activity was also measured in the substantia innominata from 9 patients with Alzheimer's disease and 5 age‐matched control subjects. Subjects with early‐onset Alzheimer's disease had significantly lower choline acetyltransferase activity in substantia innominata than did control subjects. Patients with Alzheimer's disease and a history of myoclonus had significantly lower choline acetyltransferase activity than did affected patients without myoclonus. Multivariate regression analysis showed myoclonus to be the single best predictor of low brain choline acetyltransferase activity. These results provide further evidence for clinical, pathological, and biochemical heterogeneity in Alzheimer's disease.
Recent advances in understanding brain capillary function
Authors: Goldstein Gary W. , Betz A. Lorris
Published: January 2005

The endothelial cells in brain capillaries form a blood‐brain barrier which limits and controls the movements of solutes between blood and brain. These cells contain continuous tight junctions and exhibit a low rate of pinocytosis, resulting in formation of a permeability barrier to macromolecules and many polar compounds. However, brain capillary endothelial cells also contain specialized transport systems that facilitate blood‐to‐brain transfer of some solutes and actively pump other solutes from brain to blood. Several investigators have developed methods to isolate microvessels from brain or to grow brain capillary endothelial cells in tissue culture. This review summarizes progress made with these model systems and discusses their usefulness in increasing our knowledge of brain capillary function.
Brain microvessel endothelial cells in tissue culture: A model for study of blood‐brain barrier permeability
Authors: Bowman Phillip D. , Ennis Steven R. , Rarey Kyle E. , Lorris Betz A. , Goldstein Gary W.
Published: January 2005

Endothelial cells were prepared from bovine brain microvessels and grown in tissue culture. They contained factor VIII/von Willebrand antigen, the most specific marker available for determination of the endothelial origin of cells in culture. The cultured cells formed complex tight junctions and contained few pinocytotic vessels. These properties are responsible for formation of the blood‐brain barrier in vivo. When monolayers of the endothelial cells were exposed briefly to a calcium‐free solution or treated with 1.6 M arabinose, distinctive morphological changes occurred in the intercellular contacts. In either case, a normal structure was reestablished following return to control medium. To assess the effect of these treatments on transcellular permeability, we measured the movement of sucrose labeled with carbon 14 across a monolayer of endothelial cells cultured on a collagen‐coated nylon mesh. Removal of external calcium increased the rate of sucrose movement by 120%; the arabinose treatment increased transcellular flux by 40%.
{18F}fluorodeoxyglucose positron emission tomography in refractory complex partial seizures
Authors: Theodore William H. , Newmark Michael E. , Sato Susumu , Brooks Rodney , Patronas Nicholas , de la Paz Robert , Dichiro Giovanni , Kessler Robert M. , Margolin Richard , Manning Ronald G. , Channing Michael , Porter Roger J.
Published: January 2005

Positron emission tomography with simultaneous electroencephalographic monitoring was performed with {18F}fluorodeoxyglucose in 20 patients with complex partial seizures who had normal computed tomographic scans. Seven patients had only unilateral epileptiform discharges on the electroencephalogram, 3 had predominantly unilateral discharges, and 10 had nonlocalized epileptiform abnormalities. Positron emission tomography showed a hypometabolic lesion in 16 of the 20 patients. Pathological changes in the hypometabolic region were found in postoperative specimens in 4 of 5 patients studied. Positron emission tomography was unaffected by the seizure frequency, state of alertness, or number of spike discharges during the scan. There was a tendency for patients to have higher overall metabolic rates when taking less medication. Seizures occurring during {18F}fluorodeoxyglucose uptake in 3 patients produced a hypermetabolic area at the interictal hypometabolic focus. Positron emission tomography sometimes showed more widespread hypometabolism than suspected on the basis of the scalp‐recorded electroencephalogram. The frontal lobe showed a greater degree of hypometabolism than the temporal lobe in 3 patients. Focal lesions may be identified by positron emission tomography even if the electroencephalographic abnormality is not well localized.