bluuebunny/crossref_metadata_2025 huggingface dataset:
The AIDS dementia complex: II. Neuropathology
Authors: Navia Bradford A. , Cho Eun‐Sook , Petito Carol K. , Price Richard W.
Published: January 2005

In order to define the histopathological substrate of the dementia that frequently complicates the acquired immune deficiency syndrome (AIDS), we analyzed the neuropathological findings in 70 autopsied adult AIDS patients, 46 of whom had suffered clinically overt dementia. Less than 10% of the brains were histologically normal. Abnormalities were found predominantly in the white matter and in subcortical structures, with relative sparing of the cortex. Their frequency and severity generally correlated well with the degree and duration of clinical dementia. Most commonly noted was diffuse pallor in the white matter, which in the pathologically milder cases was accompanied by scanty perivascular infiltrates of lymphocytes and brown‐pigmented macrophages, and in the most advanced cases by clusters of foamy macrophages and multinucleated cells associated with multifocal rarefaction of the white matter. However, in nearly one third of the demented cases the histopathological findings were remarkably bland in relation to the severity of clinical dysfunction. In addition, similar mild changes were noted in over one half of the nondemented patients, consistent with subclinical involvement. Vacuolar myelopathy was found in 23 patients and was generally more common and severe in patients with advanced brain pathology. Evidence of cytomegalovirus (CMV) infection was noted in nearly one quarter of the brains and was associated with a relative abundance of microglial nodules, but correlated neither with the major subcortical neuropathology nor with the clinical dementia, indicating that CMV infection likely represented a second, superimposed process. This study establishes the AIDS dementia complex as a distinct clinical and pathological entity and, together with accumulating virological evidence, suggests that it is caused by direct LAV/HTLV‐III brain infection.
Treatment of mitochondrial myopathy due to complex III deficiency with vitamins K3 and C: A 31P‐NMR follow‐up study
Authors: Argov Zohar , Bank William J. , Maris John , Eleff Scott , Kennaway Nancy G. , Olson Robert E. , Chance Britton
Published: January 2005

A patient with mitochondrial myopathy due to complex III deficiency who was treated with vitamin K3 (menadiol sodium diphosphate, 40 mg daily) and vitamin C showed clinical improvement. A 1‐year study with phosphorus 31 nuclear magnetic resonance (31P‐NMR) monitoring has shown that (1) clinical and metabolic improvement was maintained by this therapy; (2) increasing the dose of vitamin K3 to 80 mg daily improved the bioenergetic state of the patient's muscles at rest; (3) postexercise recovery was less responsive to the increased dose; and (4) a higher dose of vitamin K3 (80 mg/day) did not produce side effects. The differential therapeutic effects of vitamin K3 at rest and during exercise recovery are probably due to the differential kinetics of each metabolic state. Monitoring muscle bioenergetics with 31P‐NMR is valuable in documenting therapeutic improvements in mitochondrial myopathies.
Multidisciplinary prediction of seizure relief from cortical resection surgery
Authors: Dodrill Carl B. , Wilkus Robert J. , Ojemann George A. , Ward Arthur A. , Wyler Allen R. , van Belle Gerald , Tamas Laszlo
Published: January 2005

Preoperative variables from a full range of medical specialties were used to predict degree of seizure relief from cortical resection surgery as treatment for epilepsy in 100 patients. General, seizure history, electroencephalographic (EEG), radiological, surgical, and psychological/neuropsychological data were considered. The patients were divided into one large predictive group (n = 75) and a smaller independent cross‐validation sample (n = 25). Eight predictive variables emerged: (1) single EEG focus; (2) anterior‐midtemporal lobe discharges; (3) discharges only from the side of surgery; (4) rate of occurrence of discharges in surgical area; (5) Wechsler Adult Intelligence Scale Digit Symbol subtest; (6) Marching Test, preferred hand, time; (7) Minnesota Multiphasic Personality Inventory (MMPI) Hysteria scale score; and (8) MMPI Paranoia scale score. By use of multivariate procedures, increased predictability of surgical outcome was obtained not only with the predictive group but with the independent cross‐validation sample. The results demonstrate that predictions of seizure relief from epilepsy surgery can be made with 80% accuracy using multiple, rather than single, predictors.
Magnetic resonance imaging in intractable partial epilepsy: Correlative studies
Authors: Sperling Michael R. , Wilson Gabriel , Engel Jerome , Babb Thomas L. , Phelps Michael , Bradley William
Published: January 2005

A study was performed evaluating magnetic resonance imaging (MRI) in 35 patients with intractable complex partial seizures in whom computed tomographic (CT) scans showed no focal abnormalities. These results were correlated with positron emission tomography evaluation (PET), the electroencephalographic ictal onset, and findings during pathological examination. Seven patients had structural lesions that were epileptogenic, detected by MRI; the lesions were tuberous sclerosis, astrocytomas, or hamartomas. Three of these 7 patients underwent PET scanning, which was normal in all. Of 18 patients with mesial temporal sclerosis, 10 were shown by PET to have temporal lobe hypometabolism, though all 18 had normal MRI findings. The results indicate that MRI contributes information to that provided by CT and PET, by detecting nonsclerotic epileptogenic lesions of the temporal lobe.
Effects of ACTH on seizure susceptibility in the developing Brain
Authors: Holmes Gregory L. , Weber Donald A.
Published: January 2005

Adrenocorticotropic hormone (ACTH) has been frequently used as an anticonvulsant drug in some childhood seizure disorders. Despite its widespread use, few studies have evaluated the effects of ACTH on seizure susceptibility in the developing animal or the long‐term consequences of ACTH treatment on brain development. In this study, ACTH was given either for 2 days or 14 days prior to kindling in 15‐, 22‐, and 30‐day‐old rats. Morphological changes in the brain were studied using routine light microscopy and dendrite branch counting following Golgi staining. Both acute and chronic ACTH treatment inhibited the rate of kindling in all three age groups. There were no differences in brain morphology between the controls and the ACTH‐treated rats killed shortly after kindling. Rats treated with ACTH and killed as adults, however, had significantly more dendrite branches than did controls. In the immature brain, ACTH treatment reduces seizure susceptibility and has no long‐term deleterious effects on neuronal growth.
Amyloid plaques in Creutzfeldt‐Jakob disease stain with prion protein antibodies
Authors: Kitamoto Tetsuyuki , Tateishi Jun , Tashima Takatoshi , Takeshita Iwao , Barry Ronald A. , Dearmond Stephen J. , Prusiner Stanley B.
Published: January 2005

Amyloid plaques are found in the brains of some patients with Creutzfeldt‐Jakob disease (CJD) and all patients with a related transmissible disorder, Gerstmann‐Sträussler syndrome (GSS). In scrapie, a prion disease of animals, amyloid plaques have been shown to be composed of prion proteins (PrP), which form filaments of relatively uniform diameter. We report here that antisera raised against hamster scrapie PrP specifically stain amyloid plaques in the brains of both humans and rodents with CJD as well as a human subject with GSS. Earlier studies showed that these antibodies react with both rodent and human CJD PrP. The immunostained congophilic amyloid plaques in rodent brains measured 10 to 30 μm in diameter and exhibited a Maltese cross appearance. Limited proteolysis enchanced immunostaining of amyloid plaques in human brain sections from patients with CJD or GSS. Presumably proteolysis increases the exposure of those epitopes shared by human and rodent PrP. The differences in immunoreactivity between rodent and human amyloid plaques are consistent with other findings showing that cellular genes, not infectious purified prions, encode PrP.
Prognosis of dementia in normal‐pressure hydrocephalus after a shunt operation
Authors: Thomsen A. M. , Børgesen S. E. , Bruhn P. , Gjerris F.
Published: January 2005

Forty patients who had normal‐pressure hydrocephalus were examined neuropsychologically before and 12 months after a ventriculoatrial shunt operation. Comparison of the preoperative and postoperative test results showed that cognitive functions improved in 16 patients, were unchanged in 19, and deteriorated in 5. Moreover, we found that the outcome of the operation depended on patient selection criteria. When three or more of the following preoperative signs were present (known cause, short history, low cerebrospinal fluid outflow, small sulci and/or periventricular hypodensity indicated by computed tomography), an improvement in cognitive function was seen in 80% of the patients after a shunt operation.
Mollaret's meningitis: A case with increased circulating natural killer cells
Authors: Goldstein Rose , Guberman Alan , Izaguirre Carlos A. , Karsh Jacob
Published: January 2005

A patient with longstanding Mollaret's meningitis had an increased number of circulating natural killer cells during an acute attack, as evidenced by 73% large granular lymphocytes and 80% Leu‐11a–positive peripheral mononuclear cells. Numbers of large granular lymphocytes and Leu‐11a–positive cells returned to normal by three months after the attack. Similarity of the morphological features of the Mollaret's cell to those of large granular lymphocytes is noted.
The value of intraoperative EEG monitoring during carotid endarterectomy
Authors: Cho I. , Smullens S. N. , Streletz L. J. , Fariello R. G.
Published: January 2005

Since controversy continues concerning the value of routine electroencephalographic (EEG) monitoring and selective carotid bypass shunting, we reviewed the neurological morbidity and mortality following elective carotid endarterectomy in 172 patients with symptomatic carotid occlusive disease. The use of EEG monitoring and selective shunting was associated with a reduction in the frequency of carotid shunts (49 to 12%) and a decline in combined major neurological morbidity and mortality (2.3 to 1.1%). We conclude that the routine use of intraoperative EEG monitoring is a valuable procedure, particularly in patients at high risk for perioperative neurological complications.
Early detection and treatment of spinal epidural metastases: The role of myelography
Authors: Rodichok Lawrence D. , Ruckdeschel John C. , Harper Gregory R. , Cooper Gregory , Prevosti Louis , Fernando Leonides , Baxter Donald H.
Published: January 2005

Spinal epidural metastases were detected in 75 of 140 cancer patients with back pain who were evaluated prospectively by clinical criteria, spine roentgenography, and bone scan. Fifty‐five of the 75 patients with epidural metastases had no evidence of myelopathy when diagnosed. Of the patients diagnosed and treated while still ambulatory, more than 90% remained so. Myelograms were performed in 127 patients to diagnose the 75 with epidural disease. To try to reduce the number of myelograms needed, we attempted to design radiotheraphy ports based on clinical symptoms and the plain spine films alone. A port could not be designed for 64 of the 127 patients, either because of diffuse vertebral metastases or a normal plain roentgenogram. A port could be designed for 63 patients, and all epidural disease would have been encompassed in 50 of the 54 patients who had spinal epidural metastases (93%). Most patients with cancer and back pain require myelography for accurate treatment planning. There are, however, situations in which treatment can be determined based on symptoms and plain films alone, with a low risk of missing epidural cancer.
Sympathetic neuronal destruction in macaque monkeys by guanethidine and guanacline
Authors: Palmatier Margaret A. , Schmidt Robert E. , Plurad Santiago B. , Johnson Eugene M.
Published: January 2005

To determine whether the peripheral sympathetic neurons of subhuman primates are destroyed by guanacline treatment, we treated Macaca fasicularis with 2 or 20 mg/kg of guanethidine, guanacline, or the saturated analog of guanacline (SAG) 5 times per week for 4 or 12 weeks. All monkeys given 20 mg/kg of guanethidine, guanacline, or SAG showed a marked loss of neurons in the ganglia of the peripheral sympathetic nervous system. Treatment of macaques with 2 mg/kg of the guanidinium compounds resulted in patches of small‐cell infiltrate, slight neuronal loss, and degenerative alterations in the sympathetic ganglia. Neuronal alterations in sympathetic ganglia of all treated monkeys were accompanied by a prominent heterogeneous infiltrate of mononuclear cells arranged primarily in a perivascular distribution and extending into the ganglionic neuropil. Peripheral sensory ganglia were unaffected. These histological findings are similar to those described in the guanethidine‐induced immune‐mediated sympathectomy, which has been extensively studied in the rat.
Parasagittal infarction in neonatal asphyxia
Authors: Pasternak Joseph F.
Published: January 2005

A patient is described who suffered a bilateral hemorhagic infarction in the parasagittal cerebrum following neonatal asphyxia. This case confirms experimental results that show the parasagittal cerebrum is especially vulnerable to ischemic damage in newborns.
Effect of generalized spike‐and‐wave discharge on glucose metabolism measured by positron emission tomography
Authors: Ochs Rachel F. , Gloor Pierre , Tyler Jane L. , Wolfson Tina , Worsley Keith , Andermann Frederick , Diksic Mirko , Meyer Ernst , Evans Allan
Published: January 2005

Positron emission tomography was used to study the cerebral metabolic rate for glucose (CMRGlc) in 7 adult patients with generalized spike‐and‐wave activity in the electroencephalogram. No consistent changes were seen in the CMRGlc. There was a slight trent toward an increased CMRGlc in 2 patients with primary generalized epilepsy, while in the 5 other patients with minor deviations from this condition or with secondary generalized epilepsy, the CMRGlc was unaffected by spike‐and‐wave activity or was below the normal range. Neither the amount of spike‐and‐wave activity in the EEG nor the presence of clinically evident absence seizures appeared to influence the CMRGlc. Therefore, we conclude that neuronal activity underlying spike‐and‐wave discharge does not seem to require increased glucose utilization.
Infarction of the conus medullaris
Authors: Anderson N. E. , Willoughby E. W.
Published: January 2005

We describe 5 patients who presented with an acute cauda equina syndrome, which we believe was due to infarction of the conus medullaris. In 3 patients, the onset was spontaneous, and in 2 patients it was secondary to temporary occlusion of the distal aorta during medical manipulation. Pain in the buttocks and posterior thighs was a prominent initial symptom in the 3 patients with unprovoked attacks. The main clinical features were profound impairment of bowel and bladder function and of perianal and perineal sensation (S3 to S5 segments). There was sensory and motor impairment in the legs of variable extent, most marked in the S1 and S2 segments, but extending as high as L4 in 2 patients. In 1 patient, ischemic changes in the conus medullaris were confirmed post mortem. Ischemia confined to the caudal tip of the spinal cord is rare, and an underlying anomaly of the pattern of arterial supply is a likely predisposing factor. Four patients had partial return of function over a period of weeks.
Somatostatin augments the spread of limbic seizures from the hippocampus
Authors: Perlin Jonathan B. , Lothman Eric W. , Geary William A.
Published: January 2005

The role of the neuropeptide somatostatin in limbic seizures was studied using electrical stimulation of the hippocampus in kindled rats. Cysteamine, an agent which selectively and reversibly depletes brain somatostatin stores, had a biphasic action. An early proconvulsant effect was seen within a few hours, consisting of prolonged electrographic seizures in the hippocampus and more severe behavioral convulsions. A later anticonvulsant effect, maximal at 1 to 2 days and dissipating within a week, was manifested by less intense behavioral convulsions without change in the duration of electrical seizure activity. Both effects were dose‐dependent. No change in afterdischarge thresholds was detected at any time after the administration of cysteamine. Intraventricular administration of somatostatin to animals with behavioral seizures attenuated by cysteamine treatment restored the responses to precysteamine levels. We conclude that somatostatin facilitates the spread of seizures over limbic circuits from a region of focal seizure initiation.
Phosphofructokinase activity in the brain in Alzheimer's disease
Authors: Sims Neil R. , Blass John P. , Murphy Cathleen , Bowen David M. , Neary David
Published: January 2005

The activity of phosphofructokinase, a key regulatory enzyme of glycolysis, was determined in neurosurgical samples of neocortex obtained from 6 patients with primary degenerative dementia, including 4 with Alzheimer's disease. No consistent reductions were seen compared with samples from nondemented controls. These results contrast with the greatly reduced activity reported previously for postmortem brain specimens from patients with Alzheimer's disease.
Progressive cytochrome c oxidase deficiency in a case of earns‐sayre syndrome: Morphological, immunological, and biochemical studies in muscle biopsies and autopsy tissues
Authors: Bresolin Nereo , Moggio Maurizio , Bet Luciano , Gallanti Andrea , Prelle Alessandro , Nobile‐Orazio Eduardo , Adobbati Laura , Ferrante Claudio , Pellegrini Giulio , Scarlato Guglielmo
Published: January 2005

We report biochemical, immunological, and morphological findings in a patient with fatal Kearns‐Sayre syndrome. Histochemical and biochemical findings from muscle biopsy specimens obtained 7 years aprt documented the disease's evolution from a mild mitochondrial disorder affecting a small proportion of muscle fibers to a severe disorder affecting a large proportion of muscle fibers. Cytochrome c oxidase activity in muscle declined profoundly as the disease progressed, although the level of enzyme protein was normal, as shown by immunochemical techniques. Other organs were severely affected by the disease. Examination of postmortem tissue showed spongiosis in the frontal cortex, diffuse loss of Purkinje cells in the cerebellum, liver steatosis, and heart fibrosis with mitochondrial abnormalities. Cytochrome c oxidase activity was only slightly reduced in these organs.
The role of scanpaths in facial recognition and learning
Authors: Rizzo Matthew , Hurtig Richard , Damasio Antonio R.
Published: January 2005

We carried out a comprehensive assessment of eye movements in 2 subjects with impaired facial learning and recognition to determine if the defect might be associated with abnormal scanning of faces. Standard electrooculogram showed that fixation, pursuit, saccades, and scanning of salient features of scenes and faces were normal, indicating that the defect was not due to abnormal scanning. However, study of the transitional properties of scanning revealed that the scanpaths of personally meaningful familiar faces, whether or not they were consciously recognized, were less predictable than those of other faces. This result indicates the existence of an internal and relatively specific schema for familiar faces sufficient to alter the “predictable” scanpath used for nonfamiliar faces. This internal schema is accessed even when conscious recognition fails, i.e., when other pertinent memories related to the possessor of the face are not evoked. The meaning of this finding is comparable to that obtained with autonomic studies of covert recognition in prosopagnosia and suggests that properties of scanning can be used as an index of higher neural processing.
Magnetic resonance imaging in temporal lobe epilepsy: Pathological correlations
Authors: Kuzniecky Ruben , de la Sayette Vincent , Ethier Roméo , Melanson Denis , Andermann Frederick , Berkovic Samuel , Robitaille Yves , Olivier André , Peters Terence , Feindel William
Published: January 2005

A retrospective single‐blind study assessing the value of magnetic resonance imaging (MRI) in 48 patients treated surgically for temporal lobe epilepsy was carried out. The imaging findings were correlated with the surgical findings in all cases. Abnormal MRI signals were detected in 34 of 48 (71%) epileptic patients and in 3 of 48 (6.2%) normal or disease control subjects. Twelve patients had structural foreign‐tissue lesions, all detected by MRI. Of 14 patients with severe gliosis of the neocortex and/or mesial temporal structures, 11 had abnormal MRI scans. In patients with mild or moderate gliosis of mesial temporal structures, 6 of 12 had abnormal MRI scans. These results indicate that MRI is a sensitive technique for localizing foreign‐tissue lesions, mesial temporal sclerosis, and gliosis in patients with intractable temporal lobe seizures.
Cytochrome c oxidase deficiency in leigh syndrome
Authors: DiMauro Salvatore , Servidei Serenella , Zeviani Massimo , DiRocco Maja , DeVivo Darryl C. , DiDonato Stefano , Uziel Graziella , Berry Kenneth , Hoganson George , Johnsen Stanley D. , Johnson Peter C.
Published: January 2005

We studied 6 mitochondrial enzymes in crude extracts and isolated mitochondria from 5 children with pathologically proven subacute necrotizing encephalomyelopathy (Leigh syndrome). Samples were taken from brain (5 patients), skeletal muscle (4 patients), liver (4 patients), kidney (4 patients), heart (1 patient), and cultured fibroblasts (3 patients). An isolated defect of cytochrome c oxidase (COX) activity was found in brain (Decemberrease of activity to 15 to 39% of the normal mean), muscle (9 to 20%), kidney (1 to 67%), and in the 1 available heart (4%) from a patient with cardiopathy. COX activity was also Decemberreased in liver of 3 patients (2 to 13% of normal) and in cultured fibroblasts of 2 patients (18 and 27%), but it was normal in both liver and fibroblasts from 1 patient. Immunotitration using polyclonal antibodies against human heart COX showed essentially normal amounts of cross‐reacting enzyme protein in various tissues from different patients. Electrophoresis of COX immunoprecipitated from brain mitochondrial extracts showed normal patterns of COX subunits in 2 patients. This study confirms the theory that COX deficiency is an important cause of Leigh syndrome.
Effects of hypothermia on brainstem auditory evoked potentials in humans
Authors: Markand Omkar N. , Lee Byung I. , Warren Carroll , Stoelting Robert K. , King Robert D. , Brown John W. , Mahomed Yousuf
Published: January 2005

Ten adult patients who underwent open heart surgery under induced hypothermia had brainstem auditory evoked potentials (BAEPs) recorded at 1°‐ to 2°C‐steps as body temperature was lowered from 36°C to 20°C to determine temperature‐dependent changes. Hypothermia produced increased latencies of BAEP waves I, III, and V; the prolongation was more severe for the later components with the result that interpeak latencies I–III, III–V, and I–V were also prolonged. The temperature‐latency relationship was nonlinear and best expressed by exponential curve. The latencies of waves I, III, V and the interpeak latency I–V increased roughly 7% for each 1°C drop; they doubled at a temperature around 26°C. The amplitude of the BAEP components had a quasiparabolic relationship to temperature; the amplitude rose with hypothermia to 28° or 27°C, but Decemberreased lineraly with further cooling. All BAEP components were present at temperatures above 23°C and absent below 20°C. With rewarming, the changes reversed and BAEPs returned to initial prehypothermia status.
Tau antisera recognize neurofibrillary tangles in a range of neurodegenerative disorders
Authors: Joachim Catharine L. , Morris James H. , Kosik Kenneth S. , Selkoe Dennis J.
Published: January 2005

Neurofibrillary tangles occur in a number of apparently distinct neurodegenerative diseases and in normal aging of the human brain. Antibodies raised against Alzheimer's disease paired helical filaments immunolabel the tangles seen in all other tangle‐associated disorders examined to date. The neuronal microtubule‐associated protein, tau, has recently been identified as an antigenic component of neurofibrillary tangels and senile plaque neurites in Alzheimer's disease. Three different polyclonal antibodies with strong tau immunoreactivity are examined in this study. These antibodies were found to immunostain tangles in normal aged brain and in brains affected by a range of neurodegenerative disorders, including Down's syndrome, Alzheimer's disease plus Parkinson's disease, progressive supranuclear palsy, and the parkinsonism‐dementia complex of Guam, as well as Pick bodies in Pick's disease. The findings further illustrate the relative nonspecificity of neurofibrillary lesions in neurodegenerative disorders.
Progressive aphasia without dementia: Two cases with focal spongiform degeneration
Authors: Kirshner Howard S. , Tanridag Oguz , Thurman Lawrence , Whetsell William O.
Published: January 2005

Two patients with the syndrome of progressive aphasia without evidence of generalized dementia underwent postmortem neuropathological examinations. In both patients, characteristic changes of Alzheimer's disease, Pick's disease, or Creutzfeldt—Jakob disease were absent. Both patients showed a focal spongiform change involving primarily layer 2 of the left inferior frontal gyrus (and temporal cortex in Patient 1) and a mild astrocytosis in layer 2 and deeper cortical layers. This focal, spongiform cortical degeneration in patients with progressive aphasia does not appear to duplicate any known central nervous system degenerative disease.
Dysmyelination in transgenic mice containing JC virus early region
Authors: Trapp Bruce D. , Small Judy A. , Pulley Michael , Khoury George , Scangos George A.
Published: January 2005

JC virus (JCV) causes the chronic human demyelinating disease progressive multifocal leukoencephalopathy. Because of host range restrictions, experimental models of JCV‐induced demyelination have not been available. The restricted tropism of JCV infectivity has recently been overcome by the production of transgenic mice that contain the early region of JCV in all cells. This portion of the DNA encodes JCV T‐antigens. These mice display a dysmyelinating phenotype, the severity of which is related to the level of JCV early region expression in brain. With the use of immunocytochemistry and in situ hybridization, we characterized morphologically myelin‐specific and JCV gene expression in a severely affected strain of these mice. Our results suggest that expression of JCV T‐antigens occurs predominantly in oligodendrocytes and is the primary cause of dysmyelination. Affected oligodendrocytes do not myelinate axons properly. However, they express myelin‐specific genes and display some of the morphological phenotypes of early stages of myelination. A decreased ratio between levels of transcriptional and translational products of genes encoding the major structural proteins of central nervous system myelin was apparent. These results suggest that JCV T‐antigens arrest the maturation of oligodendrocytes and inhibit the production of myelin. These results also demonstrate that JCV transgenic mice are a good model for investigating mechanisms of JCV‐induced demyelinating lesions in progressive multifocal leukoencephalopathy.
Multifocal cytomegalovirus encephalitis in AIDS
Authors: Masdeu J. C. , Small C. B. , Weiss L. , Elkin C. M. , Llena J. , Mesa‐Tejada R.
Published: January 2005

A 43‐year‐old man with the acquired immunodeficiency syndrome had clinical evidence of multifocal disease of the brain, but computed tomography was negative. Magnetic resonance imaging revealed multifocal lesions, histologically proven to be caused by cytomegalovirus. Therapy with 9{2‐hydroxy‐1‐(hydroxymethyl) ethoxymethyl} guanine (BW B759U) resulted in stabilization of the patient's clinical disease and radiographic improvement of the lesions.
Brain infarction in sickle cell anemia: Magnetic resonance imaging correlates
Authors: Pavlakis Steven G. , Bello Jacqueline , Prohovnik Isak , Sutton Millicent , Ince Carol , Mohr Jay P. , Piomelli Sergio , Hilal Sadek , De Vivo Darryl C.
Published: January 2005

Brain infarction is a well‐known but poorly understood complication of sickle cell disease. Seventy‐three sickle cell disease patients underwent neurological examinations and high‐field, heavily T2‐weighted axial cranial magnetic resonance image (MRI) scanning. Eighteen of the 73 had a history of stroke, defined as an acute, focal neurological sign lasting longer than 1 hour; in the event of a convulsive onset, an MRI abnormality as a correlate was necessary. Sixteen of the 18 stroke patients demonstrated focal MRI abnormalities consistent with arterial borderzone infarctions. Fifty‐five of the 73 patients had no history of stroke. Six of the 55 (11%) had focal MRI abnormalities suggesting previous subclinical stroke. Five of these lesions were in borderzone regions. The distinguishing feature in 21 of the 22 patients with MRI abnormalities was the predilection for lesions in the high cortical convexity, in the general regions of arterial borderzones between the major cerebral arteries and adjacent deep white matter. The pattern of MRI lesions suggests two pathogenetic mechanisms: (1) proximal large‐vessel disease with inadequate cerebral perfusion (distal field insufficiency syndrome) and (2) distal small‐vessel disease (sludging syndrome).
Spectral power of human limbic evoked potentials: Relationship to seizure onset
Authors: Meador K. J. , Loring D. W. , King D. W. , Gallagher B. B. , Rogers O. L. , Smith J. R. , Flanigin H. F.
Published: January 2005

Limbic evoked potentials were recorded from intracerebral electrodes bilaterally implanted in the hippocampi of 16 epileptic patients undergoing evaluation for surgical resection of a seizure focus. Spectral analysis of the limbic evoked potentials revealed consistently lower spectral power of both low‐band (1–12.6 Hz) and high‐band (13–20 Hz) components recorded from the hippocampus that was associated with the electrographically confirmed seizure focus as compared to the contralateral side. Limbic evoked potential recordings appear to be useful in the determination of a focal functional deficit and thus in identifying the site of epileptogenic pathology. Further, the results support the hypothesis of a local generator for limbic evoked potentials and demonstrate that limbic evoked potentials in humans include not only low‐frequency, high‐amplitude electric field changes, but also a high‐frequency component.
Focal cortical myoclonus and rolandic cortical dysplasia: Clarification by magnetic resonance imaging
Authors: Kuzniecky Ruben , Berkovic Samuel , Andermann Frederick , Melanson Denis , Olivier André , Robitaille Yves
Published: January 2005

Focal cortical myoclonus is rare. Obvious causes include tumor or atrophy involving the motor strip, but in some cases no cause is apparent. We present 4 patients who started to have focal myoclonus in childhood. All had focal motor seizures as well, and one had recurrent focal motor status epilepticus. All 4 had a mild progressive hemiparesis. Electrographic investigations showed focal epileptic discharges in the contralateral rolandic areas. Radiological studies were unrevealing, but magnetic resonance showed rolandic lesions in 3 patients. At surgery, abnormally wide gyri were found in the distribution demonstrated by magnetic resonance. The pathological substrate was focal cortical dysplasia. All patients have improved considerably following surgery. These findings suggest that focal myoclonus may be due to a rolandic neuronal migration disorder. Visualization of these lesions by magnetic resonance permits development of a surgical strategy leading to optimal treatment of these medically intractable epileptic disorders.
Successful chemotherapy for recurrent malignant oligodendroglioma
Authors: Cairncross J. Gregory , Macdonald David R.
Published: January 2005

Eight consecutive patients with recurrent malignant oligodendroglioma were treated with systemic chemotherapy. Six patients received a combination of procarbazine, lomustine (CCNU), and vincristine; 1 received carmustine (BCNU), and 1 diaziquone. All responded by clinical and computed tomographic scan criteria. One patient had a complete response for 78 weeks, and 7 patients had unequivocal partial responses lasting 30+ to 68+ weeks. Two partial responders had complete control of systemic metastases. Malignant oligodendroglioma is a uniquely chemosensitive glial tumor.
Seizures following myelography with iopamidol
Authors: Levey Allan I. , Weiss Howard , Yu Robin , Wang Henry , Krumholz Allan
Published: January 2005

Iopamidol, a water‐soluble contrast medium, has been rarely associated with seizures. We describe 3 patients (from a series of 785) who had generalized tonic‐clonic seizures after iopamidol myelography. Two of the patients underwent lumbar and one cervical myelography. There was a history of seizures in 2 patients, who convulsed was high, ranging from 3,000 to 4,500 mg iodine. Despite the reported low incidence of complications with iopamidol, seizures may occur, especially in patients with previous seizures and also in those receiving higher doses of iopamidol.
Serum antibodies to gangliosides in guillain‐barré syndrome
Authors: Ilyas A. A. , Willison H. J. , Quarles R. H. , Jungalwala F. B. , Cornblath D. R. , Trapp B. D. , Griffin D. E. , Griffin J. W. , McKhann G. M.
Published: January 2005

To determine whether antibodies to acidic glycolipids of nervous tissue are present in patients with Guillain‐Barré syndrome (GBS), sera from patients with GBS and appropriate control subjects were tested by a thin‐layer chromatogram overlay technique. Chromatograms on which the whole ganglioside fractions from peripheral nerve and brain had been separated were overlaid with appropriate dilutions of the patients' sera (1:100 or greater), and antibody binding was revealed with a radiolabeled or peroxidase‐labeled second antibody. Antibodies to ganglioside antigens were detected in 5 of 26 patients with GBS. IgG antibodies in 1 patient reacted strongly with LM1 (sialosyl Para‐globoside), the major ganglioside of human peripheral nervous system myelin, and its hexaose analog (sialosyl lactosaminyl paragloboside), a minor ganglioside of human peripheral nervous system myelin. The antibody titer in this patient fell 8‐fold over 6 weeks coincident with clinical improvement. IgG from 2 other patients with GBS reacted with GD1b ganglioside, and the antibody titers in these patients also decreased substantially with clinical improvement. IgM antibodies in the sera from 2 other patients reacted with GD1a and GT1b gangliosides, which have a shared terminal carbohydrate sequence. Antibodies to gangliosides were not detected in the sera from 19 patients with other neurological diseases or from 10 normal subjects, and the frequency with which antiganglioside antibodies occurred in the patients with GBS was significantly greater than that in the combined control subjects (p < 0.01). The results demonstrate relatively high levels of antibodies to gangliosides in some GBS patients.
Fluctuations of CD4+ T‐cell subsets in remitting‐relapsing multiple sclerosis
Authors: Rose Lynn Massman , Ginsberg Arthur H. , Rothstein Ted L. , Ledbetter Jeffrey A. , Clark Edward A.
Published: January 2005

Patients with multiple sclerosis (MS) frequently have selective depletion of the CD45R+CD4+ T‐cell subset during active phases of disease. To study the relationship between changes in this subset and the onset of objective clinical exacerbations of disease, a longitudinal study was undertaken. Two CD4+ T‐cell subsets and two CD8+ T‐cell subsets were monitored by two‐color immunofluorescence using a fluorescence‐activated cell sorter. These subsets of peripheral blood lymphocytes were monitored monthly for one year in a group of 9 patients with remitting‐relapsing MS and in 11 healthy age‐matched control subjects. Significant changes in the ratio of two CD4+ T‐cell subsets (CD45R−/ CD45R+) were detected in 7 of 9 patients with MS, but not in any of the control subjects. Of those 7 persons, 4 suffered major clinical relapses substantiated by alterations in the neurological examination. The other 3 suffered minor relapses with subjective clinical abnormalities. All 7 had increased CD4+ T‐cell subset ratios (%CD4+CD45R−/%CD4+CD45R+) within the month that new symptoms were reported. Most such increases resulted from a simultaneous depletion in the number of CD45R+CD4+ T cells and an increase in the number of CD45R−CD4+ T cells. One patient suffered a major relapse with no change in the ratio of CD4+ subsets but had a depletion of all CD4+ T cells. There were no consistent changes in any of the other subsets measured. These results indicate that a subgroup of patients with MS have abnormal fluctuations of two CD4+ T‐cell subsets, which may correlate with increased disease activity.
Age effects on properties of motor unit action potentials: ADEMG analysis
Authors: Howard Januarye E. , McGill Kevin C. , Dorfman Leslie J.
Published: January 2005

We have measured the configurational and firing properties of 13,206 motor unit action potentials (MUAPs) from the brachial biceps, brachial triceps, and anterior tibial muscles in 10 young (20–40 years), 10 middle‐aged (40–60 years), and 10 elderly (60–80 years) normal individuals, using an automatic method for Decemberomposition of the electromyo‐graphic (EMG) interference pattern (ADEMG). Recordings were made during stable isometric contractions at threshold, 10%, and 30% of maximum voluntary contraction using standard concentric needle electrodes. At supra‐threshold forces, an average of 5.9 simultaneously active MUAPs were identified at each recording site. Mean amplitudes, durations, and numbers of turns all increased linearly with age in both low‐threshold and high‐threshold MUAPs (p < 0.01), suggesting an ongoing process of progressive denervation and compensatory reinnervation. Mean MUAP firing rates Decemberreased with age (p = 0.01) when force was measured proportionately, but not when measured absolutely. In a subgroup of 12 age‐matched gender pairs, men had larger mean MUAP amplitudes, rise rates, and numbers of turns (p < 0.05), probably reflecting larger muscle fiber diameters. These findings amplify previous observations from traditional analysis of lowest‐threshold single MUAPs; establish a base of normative adult data for ADEMG; and further validate the clinical applicability of rapid, automatic EMG Decemberomposition.
Brain acetylcholinesterase after acute parathion poisoning: A comparative quantitative histochemical analysis post mortem
Authors: Finkelstein Y. , Wolff M. , Biegon A.
Published: January 2005

The postmortem distribution of acetylcholinesterase (AChE) inhibition was studied in the brains of 2 victims of lethal parathion intoxication and 2 control brains matched for age and sex. AChE activity in discrete brain regions was studied by quantitative histochemistry of 40‐μm‐thick sagittal or coronal cryostat sections from the 4 brains. Inhibition of human brain AChE by parathion is regionally selective. The biggest Decemberreases (60–85%) were observed in the cerebellum, some thalamic nuclei, and the cortex. Only a moderate Decemberrease (10–30%) was observed in the substanda nigra and basal ganglia, and no effect was seen in white matter. Detailed knowledge of the brain regions affected bf parathion poisoning may explain some of the clinical manifestations of organophosphate poisoning.
Torsional nystagmus in the lateral medullary syndrome
Authors: Morrow Marchk J. , Sharpe James A.
Published: January 2005

Torsional nystagmus was recorded in 3 patients with the lateral medullary syndrome. Magnetic search coil oculog‐raphy demonstrated slow phases of nystagmus of increasing, Decemberreasing, and constant velocity. Neural integration of torsional eye velocity commands to position commands is impaired by lateral medullary infarction. Torsional pulsion of saccades, consisting of torsional fast eye movements induced during saccades downward or away from the side of infarction, was recorded in 2 patients. All patients had skew deviation with hypotropia on the side of brainstem damage. The torsional nystagmus beat away from the side of infarction in each patient, but in one it alternated direction as the eyes drifted about a neutral position of torsion. We attribute the torsional nystagmus to an imbalance of central projections from the anterior and posterior semicircular canals and the otolith receptors that mediate ocular counterroll.
Brain neurotransmitters in glycine encephalopathy
Authors: Kish Stephen J. , Dixon Lori M. , Burnham W. McLntyre , Perry Thomas L. , Becker Lawrence , Cheng Jerome , Chang Li‐Jan , Rebbetoy Michelle
Published: January 2005

We measured neurotransmitter Marchkers in autopsied brain of infants with glycine encephalopathy (GE). Because patients with GE develop intractable seizures, special attention was devoted to those neurotransmitter systems implicated in human epilepsy. Mean levels of glycine in the frontal cortex of GE patients were three times higher than control values. No abnormalities were observed for concentrations of gamma‐aminobutyrici acid (and related receptors), other major neurotransmitter amino compounds, or activities of cholineacetyl‐transferase and aspartate aminotransferase. Mean acetylcholinesterase activity was significantly elevated by 46%. As experimental data suggest, glycine Marchkedly potentiates the action of the excitatory neurotransmitter glutamic acid. To the extent that the brain seizures in patients with GE can be explained by this mechanism, pharmacotherapy with excitatory amino acid antagonists may represent a new approach to the treatment of GE.
Efficacy of adjuvant chemotherapy for patients with poor‐risk medulloblastoma: A preliminary report
Authors: Packer Roger J. , Siegel Kathy R. , Sutton Leslie N. , Evans Audrey E. , D'angio Giulio , Rorke Lucy B. , Bunin Greta R. , Schut Luis
Published: January 2005

Recent studies have demonstrated that preoperative and postoperative factors can broadly stratify patients with medulloblastoma/primitive neuroectodermal tumors (MB/PNET) into risk groups. For children with factors that suggest poor outcome after treatment with surgery and radiotherapy, the addition of chemotherapy can improve survival. Since 1983, 26 children with poor‐risk posterior fossa MB/PNET have been treated at our institution with craniospinal radiation therapy and adjuvant chemotherapy. Chemotherapy consisted of vincristine during radiotherapy and eight 6‐week cycles of vincristine, cis‐platinum, and l‐(2‐chloroethyl)‐3‐cyclohexyl‐1‐nitrosourea (CCNU). Twenty‐five of 26 patients (96%) who have been entered on this protocol remain alive and free of disease at a median of 24 months from diagnosis (range 6 to 50 months). Twenty patients have completed all therapy and are at a median of 32 months from initial diagnosis with no evidence of disease. These patients were compared to a group of children with similar prognostic features treated at our institution between 1975 and 1983. Actuarial disease‐free survival was statistically significantly better for protocol patients than for historical control subjects (p < 0.002). This difference was most Marchked in patients who had received radiation therapy alone (p < 0.0003). Actuarial 2‐year disease‐free survival was 96% for patients on protocol as compared to 59% for historical control patients who had been treated with radiotherapy alone. The chemotherapy given in this protocol was well tolerated. The results of this study, although preliminary, suggest that adjuvant chemotherapy is at least transiently effective in improving the rate of disease‐free survival for children with poor‐risk MB/PNET.
Lambert‐eaton myasthenic syndrome IgG depletes presynaptic membrane active zone particles by antigenic modulation
Authors: Nagel Alexandre , Engel Andrew G. , Lang Bethan , Newsom‐Davis John , Fukuoka Tadahiro
Published: January 2005

The Lambert‐Eaton myasthenic syndrome (LEMS) is an autoimmune disease that can be transmitted from human to mouse with immunoglobulin G (IgG). Electrophysiological studies indicate that LEMS IgG acts on presynaptic voltage‐sensitive calcium channels, probably reducing their number, and freeze‐fracture electron microscopy demonstrates that LEMS IgG has an effect on the presynaptic active zone paticles, which represent putative voltage‐sensitive calcium channels. The active zone particles, normally arranged in double parallel rows, move closer together, form clusters, and are reduced in number. The morphological data suggest modulation of the active zone particles cross‐linked by LEMS IgG. If this were the case, then only divalent LEMS IgG and F(ab')2 should alter the deployment of active zone particles and moNovemberalent Fab should be without effect. To test this hypothesis, mouse diaphragms were exposed to control and LEMS IgG and IgG fragments in organ culture for 24 hours and then studied by quantitative freeze‐fracture electron microscopy. Divalent LEMS IgG and F(ab')2 aggregated and depleted the active zone particles, whereas moNovemberalent Fab had no effect. The findings reconfirm that the active zone particles are targets of LEMS IgG and are direct evidence for modulation of the particles by LEMS IgG. The findings are in harmony with parallel electrophysiological studies of the effects of LEMS IgG fragments on transmitter release in the same diaphragm muscles (Lang et al, J Physiol 1987;390:173P).
Biochemical analysis of caudate nucleus biopsy samples from parkinsonian patients
Authors: Goldstein Menek , Lieberman Abraham N. , Helmer Elizabeth , Koslow Max , Ransohoff Joseptemberh , Elsworth John D. , Roth Robert H. , Deutch Ariel Y.
Published: January 2005

Biochemical analyses of caudate nucleus biopsy samples from patients with Parkinson's disease undergoing autologous adrenal transplantation were performed. Activity of the dopamine biosynthetic enzyme tyrosine hydroxylase, and concentrations of dopamine and its priMarchy metabolite homovanillic acid were significantly greater than anticipated on the basis of previously published postmortem values. These data suggest that postmortem changes in various biochemical parameters of dopamine function are more rapid than has been generally appreciated. Further analysis of striatal biopsy samples may reveal predictive relationships between striatal indices of dopamine function and therapeutic response to adrenal transplantation.
Ergot alkaloids block neurogenic extravasation in dura mater: Proposed action in vascular headaches
Authors: Saito Kiyoshi , Marchkowitz Stephen , Moskowitz Michael A.
Published: January 2005

Although the ergot alkaloids (ergots) are useful drugs for the acute treatment of migraine headaches, their mechanism of action remains obscure. When administered to rats in clinically relevant doses, ergots blocked the development of neurogenic plasma extravasation in dura mater. Plasma extravasation was induced by depolarization of perivascular axons following capsaicin injection or unilateral electrical stimulation of the trigeminal nerve. The ergot action could not be accounted for by vasoconstriction alone because neurogenic plasma leakage was not blocked by angiotensin or phenylephrine. Furthermore, ergots did not block plasma extravasation induced by administering sensory neuropeptides that mediate enhanced permeability. We propose that the therapeutic effects of ergots in vascular headaches may result from peripheral blockade of small fiber (C or A‐delta)‐dependent neurogenic inflammation within the dura mater.