bluuebunny/crossref_metadata_2025 huggingface dataset:
Comparison of amaurosis fugax and transient cerebral ischemia: A prospective clinical and arteriographic study
Authors: Hurwitz Barrie J. , Heyman Albert , Wilkinson William E. , Haynes Carol S. , Udey Carol M.
Published: January 2005

We compared the clinical associations, arteriographic findings, and long‐term outcome of 93 patients with amaurosis fugax and 212 patients with focal cerebral ischemia (transient ischemic attacks [TIAs]). The group of patients with cerebral TIAs included a significantly larger proportion of blacks and had a higher prevalence of hypertension than the group with amaurosis. Operable atherosclerotic lesions of the carotid arteries were more often associated with amaurosis (66%) than with cerebral TIAs (51%). The seven‐year cumulative rate of cerebral infarction, however, was less in patients with amaurosis (14%) than in those with cerebral TIAs (27%; p < 0.02). This difference in outcome persisted after adjustment for race, hypertension, and type of therapy. There were no significant differences, however, in the cumulative rates either of recurrent TIAs or of myocardial infarction or sudden death in the two groups of patients.
The concept of subcortical and cortical dementia: Another look
Authors: Whitehouse Peter J.
Published: January 2005

The subcortical dementias such as progressive supranuclear palsy, Huntington's disease, and Parkinson's disease are said to be characterized by the presence of slowed mentation, apathy, and the absence of aphasia, agnosia, and apraxia, symptoms that are claimed to be more common in cortical dementias such as Alzheimer's disease. Conceptual problems (such as vagueness of terms and difficulties with symptom definition) and methodological problems (such as improper matching of comparison groups and inadequate assessment techniques) found in currently available studies require a reappraisal of this classification of dementias into cortical and subcortical forms. A review of recent clinical, neuropathological, and neurochemical studies offers little support for this classification system, although adequate systematic studies have not been performed.
Slowly progressive aphasia without generalized dementia: Studies with positron emission tomography
Authors: Chawluk John B. , Mesulam M.‐Marsel , Hurtig Howard , Kushner Michael , Weintraub Sandra , Saykin Andrew , Rubin Nan , Alavi Abass , Reivich Martin
Published: January 2005

Slowly progressive aphasia without generalized dementia is a degenerative syndrome selectively affecting dominant hemisphere language areas. We report changes in regional glucose metabolism measured by positron emission tomography in two patients with this condition. Striking abnormalities of glucose utilization in the left cerebral cortex were demonstrated in both patients. The findings of other neurodiagnostic studies were relatively unremarkable. The first patient had a 3‐year history of progressive anomia and impaired auditory verbal recall. An electroencephalogram was normal, and computed tomography showed mild left perisylvian atrophy. Positron emission tomography revealed profound hypometabolism in the left temporal regions. The second patient also had a 3‐year history of progressive anomia. Electroencephalography, computed tomography, and magnetic resonance imaging scans were normal. Positron emission tomography showed a major reduction in left parietal glucose utilization, with a lesser decrement in left temporal metabolism. Neither patient demonstrated significant contralateral or global abnormalities such as those reported in positron emission tomographic studies of Alzheimer's disease with or without focal clinical features. These observations support the concept of adult‐onset progressive aphasia without dementia as a clinical syndrome distinct from Alzheimer's disease.
Suppressor T cells in myasthenia gravis and antibodies to acetylcholine receptor
Authors: Lisak Robert P. , Laramore Cynthia , Levinson Arnold I. , Zweiman Burton , Moskovitz Anne R.
Published: January 2005

We cultured blood mononuclear cells of patients with myasthenia gravis both with and without removal of T8 suppressor cells. The levels of synthesized antibodies to acetylcholine receptor and IgG as well as the frequency of immunoglobulin‐secreting cells were all higher in pokeweed mitogen–stimulated cultures depleted of T8+ cells. Thus, autologous suppressor cells within the T8+ population exert some regulatory control over anti‐acetylcholine receptor–producing cells in patients with myasthenia gravis.
Ecto‐5′nucleotidase activity in B and T lymphocytes and monocytes of patients with multiple sclerosis
Authors: Armstrong Marilyn A. , Haire Margaret
Published: January 2005

Ecto‐5′ cucleotidase activity in the plasma membrane of B and T lymphocytes and cultred and noncultured monocytes from patients with multiple sclerosis and from age‐ and sex‐matched control subjects was determined by radioisotopic assay. The activity on lymphocytes and noncultured monocytes was normal, but the activity on cultured monocytes was found to be higher than normal in 70% of patients with active disease and lower than normal in 85% of patients with chronic inactive disease. Imunoflurescence staining with monoclonal antibodies, carried out in parallel with each assay, did not show any phenptypic differences between each cell population and its control.
Systematic regional differences in the cholinergic innervation of the primate cerebral cortex: Distribution of enzyme activities and some behavioral implications
Authors: Mesulam M.‐Marsel , Volicer Ladislav , Marquis Judith K. , Mufson Elliott J. , Green Robert C.
Published: January 2005

Choline acetyltransferase and acetylcholinesterase enzymatic activities were measured in 33 cytoarchitectonic subregions of the cerebral cortex in two rhesus monkeys. As expected, the hippocampus and amygdala were rich in these enzymes. In addition, the paralimbic (mesocortical) regions of the brain (e.g., parahippocampal, insular, caudal orbitofrontal, and temporopolar areas) also contained high levels of both enzymes. In contrast, the concentration of these cholinergic markers was the lowest within all frontal and temporoparietal association areas. As a group, the primary sensory and motor regions contained an intermediate level of choline acetyltransferase activity. Both cholinergic markers also showed a gradual increase from the isocortical toward the more primitive periallocortical subsectors of paralimbic areas. These anatomical patterns have potential implications for the role of cholinergic pathways in the memory process and in the pathogenesis of Alzheimer's disease.
Progressive encephalitis three months after resolution of cutaneous zoster a patient with AIDS
Authors: Ryder John W. , Croen Kenneth , Kleinschmidt‐Demasters B. K. , Ostrove Jeffrey M. , Straus Stephen E. , Cohn David L.
Published: January 2005

A 37‐year‐old homosexual man with the acquired immune deficiency syndrome (AIDS) developed progressive, ultimately fatal, neurological deficits 12 weeks after a course of cutaneous zoster. Premortem radiological procedures and cerebrospnal fluid analyses were nondiagnostic. At postmortem examination, several opportunistic infections associated with AIDS were recognized. Throughout the brain, necrotic and demyelinative lesions were present, suggestive of progressive multifocal leukoencephalopathy. However, light microscopical examination showed numerous Cowdry type A intranuclear inclusions in astrocytes, oligodendrocytes, and neurons near the periphery of the lesions. Herpes zoster encephalomyelitis was diagnosed and confirmed by electron microscopy, peroxidase–antiperoxidase staining, and by Southern blot analysis of DNA extracted from brain tissue. This case provides insight into the pathogenesis of zoster‐associated encephalomyelitis and suggests another agent to be considered in the differential diagnosis of encephalopathy in patients with AIDS and other disorders of immunological impairment.
Urinary sediment dolichols in the diagnosis of neuronal ceroid‐lipofuscinosis
Authors: Wolfe Leonhard S. , Palo Jorma , Santavuori Pirkko , Andermann Frederick , Andermann Eva , Jacob Jacob C. , Kolodny Edwin
Published: January 2005

Long‐chain polyisoprenol alcohol (dolichols) levels are significantly increased in the urinary sediment of patients with infantile, late‐infantile, and juvenile forms of neuronal ceroid‐lipofuscinosis (NCL). The values in obligate heterozy‐gotes for these diseases are similar to those in patients with other neurological diseases and in healthy controls. Antioxidant treatment of patients with juvenile NCL has no effect on dolichol values. The rate of false‐negative results is 13.9% in infantile, 7.5% in late‐infantile, and 15.0% in juvenile NCL. False‐positive results were found in 8.2 to 14.3% of patients with other neurological diseases and in 15.4% of healthy controls. The test is of considerable value in the diagnosis of NCL and in decisions on whether to perform a biopsy. It is not useful in the screening of random samples, however.
Neuroepidemiology. Part II: Assessment of therapeutic trials
Authors: Kurtzke John F.
Published: January 2005

The gold standard for therapeutic trials is the randomized, double‐blind, placebo‐controlled study design. Lack of blindness and placebo makes tenuous the attribution of results to the specific agent; lack of randomization between concurrent and comparable groups makes it impossible. The chi‐square test of the null hypothesis of no difference between treatment groups is the best method of assessment, as no overall rating system used in neurology is a true numerical scale but at best a rank‐order scale. If quantitation of results is desired, a nonparametric rank‐order test is necessary. Results over time can be assessed with a logrank (life‐table) test for single events as end point, and by chi‐square or rank‐order tests for multiple events among individual patients.
Human autonomy and the frontal lobes. Part I: Imitation and utilization behavior: A neuropsychological study of 75 patients
Authors: Lhermitte F. , Pillon B. , Serdaru M.
Published: January 2005

A type of pathological behavior, imitation behavior (IB), is newly described. In this behavior patients imitate the examiner's gestures, although not instructed to do so. Patients explain that they thought they had to imitate the examiner. IB is the first stage of utilization behavior (UB). Neuropsychological examination of 40 patients with IB, of 35 with UB, and of 50 disease controls demonstrates the existence of a frontal syndrome and two determining features of such behavior: dependence on (1) the social and (2) the physical environments. Loss of intellectual control was also required for the occurrence of such behavior. UB and/or IB were present in 96% of the 29 patients with focal lesions of the frontal lobes. Computed tomographic scans in 26 of these patients showed involvement of the inferior half of the anterior part of one or both frontal lobes. IB and UB are interpreted as release of parietal lobe activities, resulting from impairment of frontal lobe inhibition.
Recovery of somatosensory evoked potential amplitude after movement
Authors: Angel R. W. , Weinrich M. , Rodnitzky R.
Published: January 2005

Sensory transmission is know to be impaired during movement of the stimulated body part. The goal of this study was to determine whether sensory gating persists for a measurable time after completion of a voluntary movement. Tactile stimuli were applied to the right index fingertip at intervals ranging from 63 to 1,000 msec after the completion of rapid thumb movement. Somatosensory evoked potentials (SEPs), recorded over the left cerebral hemisphere, were found to be reduced in size for at least 500 msec after the cessation of movement. The prolonged attenuation of SEPs after movement appears to depend on a central process that persists for a measurable time after the movement has ended.
Cerebrospinal fluid polyamines: Biochemical markers of malignant childhood brain tumors
Authors: Phillips Peter C. , Kremzner Leon T. , De Vivo Darryl C.
Published: January 2005

The clinical value of cerebrospinal fluid (CSF) polyamine determinations in childhood medulloblastoma has been suggested. We performed 72 CSF polyamine determinations in 35 children with primary brain tumors. Spermine values were normal and spermidine values were inconsistently elevated. CSF putrescine values, however, were consistently elevated in patients with histologically malignant brain tumors: medulloblastoma, ependymoma, pineal germ cell tumors, primitive neuroectodermal tumors, and brainstem gliomas. Children with supratentorial astrocytomas had normal CSF polyamine values. CSF putrescine values were closely correlated with clinical state, with the highest concentrations identified in patients with widely disseminated recurrent disease. We found CSF putrescine to be a sensitive indicator of active disease in childhood malignant brain tumors. Further investigation is warranted into the predictive value of CSF polyamines in determining tumor relapse before clinical or other diagnostic studies reveal recurrent disease.
Phosphorylation of intact erythrocytes in human muscular dystrophy
Authors: Johnson Robert M. , Nigro Michael
Published: January 2005

The uptake of exogenous 32Pi into the membrane proteins of intact erythrocytes was measured in 8 patients with Duchenne muscular dystrophy. No abnormalities were noted after autoradiographic analysis. This contrasts with earlier results obtained when isolated membranes were phosphorylated with γ‐[32P]ATP, and suggests a possible reinterpretation of those experiments.
Parkinson's disease in monozygotic twins
Authors: Jankovic Joseph , Reches Avi
Published: January 2005

Recent studies of twins have demonstrated an unexpectedly low concordance of Parkinson's disease in monozygotic twins. Only two monozygotic twin pairs concordant for it have been reported. However, both pairs were atypical because of an early age at onset and other unusual features. We studied a monozygotic twin pair concordant for typical Parkinson's disease. The brothers have lived apart for forty years. The onset of tremor occurred three months apart, at age 71. The progression of the symptoms has been identical. Although one of the twins is more severely affected, both have typical manifestations of Parkinson's disease that respond well to dopaminergic medication. The occurrence of Parkinson's disease in these monozygotic twins suggests that genetic susceptibility is important in the tremordominant variety of Parkinson's disease.
Antiidiotypic antibody to reovirus binds to neurons and protects from viral infection
Authors: Dichter Marc A. , Weiner Howard L. , Fields Bernard N. , Mitchell Gail , Noseworthy John , Gaulton Glen , Greene Mark
Published: January 2005

A syngeneic monoclonal antiidiotype directed against the idiotype of an antireovirus type 3 hemagglutinin demonstrates several of the biological actions of the original viral hemagglutinin and binds to rat and murine cortical neurons grown in dissociated cell culture. Receptor‐bearing neurons appear within 24 hours of plating in cultures from mouse or rat cortex taken on embryonic day 15; these neurons are demonstrable for the duration of the culture life span (4 to 8 weeks). When cortical cultures are incubated with antiidiotype before or during exposure to reovirus, the antiidiotype protects neurons from type 3 infection without inhibiting infection of nonneuronal cells with either type 3 or type 1. Thus an antibody directed against a viral receptor can prevent infection of receptor‐bearing cells without directly neutralizing the virus.
Remyelination follows antibody‐induced central nervous system demyelination
Authors: Sergott Robert C. , Brown Mark J. , Silberberg Donald H.
Published: January 2005

Remyelination after anti‐galactocerebroside‐induced demyelination in the guinea pig optic nerve was evident 17 days after the onset of demyelination. The tempo of remyelination was uniform for large‐ and small‐diameter axons and progressed, with compaction and thickening of myelin sheaths. Demyelinated axons were found interspersed among remyelinated fibers and were consistently separated from oligodendrocytes by astrocytic processes. These findings indicate that central nervous system remyelination may occur after in vivo antibody‐induced demyelination, but that new myelin formation may be limited by mechanical interference from astrocytic processes.
Inhibition of L‐{18F}fluorodopa uptake into human brain by amino acids demonstrated by positron emission tomography
Authors: Leenders K. L. , Poewe W. H. , Palmer A. J. , Brenton D. P. , Frackowiak R. S. J.
Published: January 2005

The brain uptake of L‐{18F}fluorodopa was measured by positron emission tomography in a healthy male volunteer both under fasting conditions and during intravenous amino acid loading. A significant reduction of tracer uptake into the brain was demonstrated with amino acid loading. This finding represents the first direct evidence for competition between L‐dopa and other amino acids for uptake across the blood–brain barrier obtained in vivo in a human subject. It underlines the possible importance of interference by dietary amino acids with the therapeutic actions of L‐dopa in Parkinson's disease.
Cerebral granulomatous angiitis associated with isolation of human T‐lymphotropic virus type III from the central nervous system
Authors: Yankner Bruce A. , Skolnik Paul R. , Shoukimas Gregory M. , Gabuzda Dana H. , Sobel Raymond A. , Ho David D.
Published: January 2005

A 42‐year‐old homosexual man without evidence of immune deficiency developed cerebral granulomatous angiitis in association with the isolation of human T‐lymphotropic virus type III (HTLV‐III) from brain tissue and cerebrospinal fluid. This syndrome may be an additional neurological sequela of HTLV‐III infection.
Absence of antibody to HTLV I and III in Sera of Canadian patients with multiple sclerosis and chronic myelopathy
Authors: Rice G. P. A. , Armstrong H. A. , Bulman D. E. , Paty D. W. , Ebers G. C.
Published: January 2005

Recently, elevated titers of antibody to HTLV I have been demonstrated in patients with tropical spastic paraparesis and multiple sclerosis. We evaluated the possible role of human retroviruses HTLV I and III in Canadian patients with multiple sclerosis and chronic idiopathic myelopathy. Using sensitive enzyme immunoassays, we were unable to find antibody to either HTLV I or III in 201 patients with multiple sclerosis, 29 patients with chronic myelopathy, 51 patients with other neurological disorders, or 29 normal subjects. These data do not support a role for these viruses in the cause of sporadic multiple sclerosis and chronic myelopathy in a regionally based Canadian population, but do not exclude a role for other antigenically distinct retroviruses.
A rapidly progressive autosomal dominant scapulohumeral form of spinal muscular atrophy
Authors: Jansen P. H. P. , Joosten E. M. G. , Jaspar H. H. J. , Vingerhoets H. M.
Published: January 2005

Three patients from a large pedigree are described who had autosomal dominant spinal muscular atrophy that became manifest between the end of the fourth and the sixth decade. The disease progressed rapidly without evidence of corticospinal tract dysfunction, and within 3 years the patients died from respiratory failure.
Prolactin in partial epilepsy: An indicator of limbic seizures
Authors: Sperling Michael R. , Pritchard Paul B. , Engel Jerome , Daniel Charlene , Sagel Julius
Published: January 2005

A study was performed to evaluate changes in serum prolactin levels after simple and complex partial seizures, and to identify which specific anatomical structures must be involved in seizures for postictal elevation of prolactin levels to occur. Seventy‐eight seizures were studied in patients with electrodes implanted bilaterally into amygdala, hippocampus, hippocampal gyrus, and frontal sites. All 38 complex partial seizures had bilateral limbic ictal discharges, and each was followed by a significant increase in prolactin concentration (mean peak, 50.8 ng/ml; range, 16.0 to 150.0 ng/ml). Eight of 10 simple partial seizures with unilateral high‐frequency regional limbic discharges were followed by prolactin elevation (mean peak, 28.2 ng/ml; range, 13.4 to 44 ng/ml). Thirty simple partial seizures with other ictal limbic discharges or without limbic discharges were not followed by an elevated prolactin level. The data indicate that serum prolactin levels always rise after complex partial seizures involving the temporal lobes, and rise after certain simple partial seizures involving limbic structures. Thus, measurement of the prolactin level can help identify which simple partial seizures involve mesial temporal lobe structures. Limbic structures serve to trigger prolactin release, which may depend upon spread of the seizure to subcortical structures.
Electrophysiological correlates of a paroxysmal movement disorder
Authors: Toro Camilo , Torres Fernando
Published: January 2005

Averaged electroencephalographic activity related to rhythmic jerking movements was recorded in a patient with a complex neurological symptomatology. The diagnosis of a functional disorder was strongly suggested by his clinical course, inconsistent findings from physical examination, and negative workup. His abnormal movements were associated with the same electrophysiological correlates as normal willed motion, suggesting that averaged movement potentials may provide useful information in the study of patients with suspected functional movement disorders.
Acute paraparesis secondary to Arnold‐Chiari type I malformation and neck hyperflexion
Authors: Vlcek Brien W. , Ito Brain
Published: January 2005

A 2‐year‐old child experienced clinical manifestations of Arnold‐Chiari Type I malformation, rare in early childhood, after a mild hyperflexion injury of the neck that resulted in acute paraparesis. Recovery occrred after decompressive laminectomy.
Anticonvulsants, folate levels, and pregnancy outcome: A prospective study
Authors: Dansky Linda V. , Andermann Eva , Rosenblatt David , Sherwin Allan L. , Andermann Frederick
Published: January 2005

Folate levels in serum and red cells, as determined by a microbiological assay using Lactobacillus casei, and plasma anticonvulsant concentrations were monitored concurrently in nonpregnant (50 subjects) and pregnant (49 pregnancies in 46 subjects) epileptic women. Twenty‐three (46%) nonpregnant women had subnormal serum folate levels and 4 nonpregnant women (8%) showed subnormal red cell folate levels. In pregnant women not taking folate supplements, the incidence of folate deficiency increased as the pregnancy advanced. Pregnant women taking folate supplements achieved normal or supranormal blood folate concentrations. In both nonpregnant and pregnant women, serum and red cell folate levels were inversely correlated with plasma concentrations of phenytoin and of phenobarbital, and with the number of anticonvulsants. In 49 pregnancies, there were 10 abnormal outcomes (20.4%): 4 spontaneous abortions (8.2%) and 6 children with major congenital malformations (12.2%). Blood folate levels were significantly lower in pregnancies with an abnormal outcome than in those with a normal outcome. The results suggest a dose‐response relationship among anticonvulsants, folate, and adverse pregnancy outcome.
Increased monomeric immunoglobulin A levels in tears from multiple sclerosis patients
Authors: Coyle P. K. , Sibony Patrick A. , Johnson Cynthia
Published: January 2005

Mucosal immunity and the secretory antibody are known to be important defense mechanisms against many common viruses. Tears were selected as a representative mucosal fluid, and immunoglobulin A (IgA) was examined by enzyme‐linked immunosorbent assay and immunoblot. Tears from 38 normal controls and patients with systemic or ocular diseases contained almost exclusively polymeric IgA. In contrast, almost 75% of 23 patients with multiple sclerosis had detectable monomeric IgA in addition to polymeric IgA. The functional importance of this alteration in the molecular form of secretory IgA remains to be determined.
Alzheimer's disease cells exhibit defective repair of alkylating agent—induced DNA damage
Authors: Robison Steven H. , Munzer J. Scott , Mrcp Rup Tandan , Bradley Walter G.
Published: January 2005

The most common cause of senile and presenile dementia is Alzheimer's disease, a disorder with an undetermined cause. A number of studies have indicated that neurons from patients with Alzheimer's disease have decreased ribonucleic acid levels and reduced protein synthesis. Recent studies using lymphoblasts from patients with Alzheimer's disease have indicated that these cells are more sensitive to deoxyribonucleic acid (DNA)–alkylating agents. We have used cell survival, unscheduled DNA synthesis, and alkaline elution to assess the capacity for DNA repair in skin fibroblasts from normal control subjects, control subjects with central nervous system disease, and patients with Alzheimer's disease. Our results indicate that the Alzheimer's disease cells, unlike normal cells, fail to repair methyl‐methane sulfonate–induced DNA damage. Both normal and Alzheimer's disease cells are able to ameliorate the effects of ultraviolet light. These results indicate that a specific pathway for DNA repair is affected in Alzheimer's disease. The repair defect may be related to the cause of the disease or may be the cause of the disease.
Heterogeneity in myasthenia gravis: HLA phenotypes and autoantibody responses in ocular and generalized types
Authors: Kida Kaichi , Hayashi Masatoshi , Yamada Itaru , Matsuda Hiroshi , Yoshinaga Junji , Takami Shunsai , Yashiki Shinji , Sonoda Shunro
Published: January 2005

HLA phenotypes and autoantibody responses were studied in 71 Japanese patients with myasthenia gravis. HLA‐A2, Bw61, and DRw9 were associated with ocular myasthenia gravis (corrected p [CP] < 0.05 relative risk [RR] = 2.88; CP < 0.02, RR = 3.60; and CP < 0.001, RR = 4.63, respectively) and HLA‐DRw8 was associated with generalized myasthenia gravis (CP < 0.001, RR = 5.40). Neither HLA‐B8 nor DR3 was found in Japanese patients. The titer of antiacetylcholine receptor antibody (AChR Ab) and the incidence of autoantibodies other than AChR Ab were higher in patients with generalized myasthenia gravis than in those with the ocular type (2.77 ± 0.62 versus 0.17 ± 0.03 pmol/ml, p < 0.001; and 60.6 versus 29.0%, p < 0.02, respectively). Patients with a high titer of AChR Ab or with autoantibodies had an increased frequency of HLA‐DRw8 (CP < 0.02, RR = 4.61, and CP < 0.005, RR = 4.53, respectively); whereas patients with a low titer of AChR Ab or without autoantibodies had an increased frequency of HLA‐DRw9 (CP < 0.001, RR = 8.26, and CP < 0.005, RR = 4.08, respectively). These findings suggest that ocular and generalized myasthenia gravis might have different immunogenetic backgrounds.
Clonidine and Gilles de la Tourette's syndrome: Double‐blind study using objective rating methods
Authors: Goetz Christopher G. , Tanner Caroline M. , Wilson Robert S. , Susan V. Carroll , Como Peter G. , Shannon Kathleen M.
Published: January 2005

Clonidine has been suggested to be effective in Gilles de la Tourette's syndrome (GTS), but no double‐blind study has ever evaluated its effects using objective measures. Thirty patients with GTS completed a 6‐month placebo‐controlled crossover study of the effectiveness of clonidine. Videotapes were obtained at each 3‐week visit and were evaluated randomly at the end of the study for distribution, frequency, and severity of motor and vocal tics. Quantifiable psychometric examinations were performed as well. The use of clonidine did not significantly (p < 0.05) reduce motor tics, vocalizations, or behavior. The effect of a low dose (0.0075 mg/kg/day) was no different from that of a high dose (0.015 mg/kg/day); children's responses were no different from adults'; and those also receiving neuroleptic agents showed the same lack of efficacy as seen in patients on no other medication. Dosing schedule did not affect the objective ratings; scores from clonidine given twice a day were equivalent to those for three times a day.
Neocortical cell counts in normal human adult aging
Authors: Terry Robert D. , DeTeresa Richard , Hansen Lawrence A.
Published: January 2005

Fifty‐one brains from clinically and neuropathologically normal individuals ranging in age from 24 to 100 years were studied to determine what changes occur in neocortical neuroectodermal cell populations as a function of normal aging. Twenty‐m̈‐thick sections from the midfrontal, superior temporal, and inferior parietal areas were examined with an image‐analysis apparatus with combined manual and automatic editing capacity. Neuroectodermal cells were counted, measured, and assigned to one of ten categories, which were later summarized in three: large neurons (<90 m̈2), small neurons (41 to 90 m̈2), and glia (5 to 40 m̈2). Determinations were also made of brain weight, cortical thickness, neuronal density, neuron‐‐glia ratio, and percentage of cell area. The results showed statistically significant age‐related decrements in the following values: brain weight, cortical thickness in the midfrontal and superior temporal areas, large neurons in all three areas, and the neuron‐‐glia ratio in the midfrontal and inferior parietal areas. The total number of neurons, neuronal density, and percentage of cell area were all unchanged. Increasing with age were the number of small neurons in the midfrontal cortex and glia in the midfrontal and superior temporal areas. The following conclusions were drawn: (1) Aging affects the frontal and temporal lobes more than the parietal; the salient change is shrinkage of large neurons with consequently increasing numbers of small neurons; (2) constant neuronal density coupled with diminished cortical volume (decreased brain weight and cortical thinning) indicate that there is some neuronal loss with age, but it is of much lesser magnitude than previously supposed; and (3) the number of glia increases with age.
The magnetic field of complex partial seizures agrees with intracranial localizations
Authors: Sutherling William W. , Crandall Paul H. , Engel Jerome , Darcey Terrance M. , Cahan Leslie D. , Barth Daniel S.
Published: January 2005

The magnetoencephalogram (MEG) was recorded during 63 complex partial seizures in 4 patients. The MEG showed large biomagnetic signals occurring at the same time as discharges recorded from scalp electroencephalogram (EEG). These MEG signals had the same morphology and frequency as the discharges from the EEG. The location of the seizure focus was verified by depth electrode recordings in 2 patients and by lesions shown on computed tomographic scan and magnetic resonance imaging in the other 2. In each patient, MEG localization estimates were consistent with the location of the seizure focus shown by other methods. When seizures were recorded repeatedly and mapped with a single‐channel magnetometer placed at different scalp locations in a single patient, the MEG localization agreed with the electrographic seizure focus localized from depth electrodes. In the maps, the MEG resolved an ambiguity in the scalp EEG and therefore increased the confidence of localization. MEG recordings of seizures may help localize epileptic foci noninvasively.
No sex‐related differences in human corpus callosum based on magnetic resonance imagery
Authors: Oppenheim Jeffrey S. , Lee Benjamin C. P. , Nass Ruth , Gazzaniga Michael S.
Published: January 2005

Recent studies have reported sex‐related differences in the morphology of the human corpus callosum in cadaver brains. To further investigate these reports, sagittal magnetic resonance image scans from 40 male and 40 female subjects were used to compare callosal morphology. Relative callosal measurements were calculated by morphometric analysis. Significant sex‐related differences were not found for callosal areas, maximal callosal width, or callosal curvature. These results indicate the need for further study before claims of sex‐related differences can be accepted.
Cholinergic‐dependent cognitive deficits in Parkinson's disease
Authors: Dubois Bruno , Danzé François , Pillon Bernard , Cusimano Guliana , Lhermitte François , Agid Yves
Published: January 2005

In a double‐blind cross‐over study, the effects of a subthreshold dose of scopolamine (0.25 mg) on memory were compared in 32 control subjects and 32 parkinsonian patients who were without any sign of intellectual and mnemic impairment. Although the scores of the controls in the memory test battery showed no deterioration after the administration of scopolamine, the same dose resulted in significantly reduced memory performance in parkinsonian patients in two tests which involved the recognition of meaningless drawings. The selective vulnerability of parkinsonian subjects without cognitive impairment to a subthreshold dose of scopolamine suggests the existence of an underlying alteration of central cholinergic transmission. The neuropsychological findings in our study agree with postmortem biochemical data, which showed decreased cortical choline acetyltransferase activity in all parkinsonian patients, suggesting the existence of neuronal compensation in parkinsonian patients who are without cognitive impairment.
Mononeuritis multiplex in Waldenström's macroglobulinemia
Authors: Lamarca Josep , Casquero Pilar , Pou Adolf
Published: January 2005

We describe a patient with mononeuritis multiplex who had cranial nerve involvement in association with Waldenström's macroglobulinemia. Sural nerve biopsy showed abundant endoneurial granulofibrillary deposits related to microangiopathic changes. These changes were distributed in a multifocal pattern among the fascicles. Immunohistochemistry demonstrated the IgM nature of the deposit located in the endoneurial interstitium.
Phenylethanolamine N‐methyltransferase activity is decreased in Alzheimer's disease brains
Authors: Burke William J. , Chung Hyung D. , Nakra B. R. S. , Grossberg George T. , Joh Tong H.
Published: January 2005

Phenylethanolamine N‐methyltransferase (PNMT) is the rate‐limiting enzyme involved in the synthesis of epinephrine and a specific marker for epinephrine neurons. We have previously described the stability of this enzyme in a variety of pre‐ and postmortem conditions in human brain autopsy specimens and demonstrated its presence in areas of the human brain that are associated with memory and attention. We now report on 5 patients with Alzheimer's disease (AD) who had a Decemberrease in PNMT activity of 37 to 48% in areas of the brain affected by the disease but not in the cerebellum, an area of the brain unaffected by the disease. The degree of Decemberrease in PNMT activity in the hippocampus correlated significantly with the degree of dementia. We have provided direct immunochemical evidence that the Decemberrease in PNMT activity in AD is due to the loss of this specific enzyme protein.
Carbamazepine and cardiac conduction disturbances
Authors: Benassi Enrico , Bo Gian‐Paolo , Cocito Leonardo , Maffini Maura , Loeb Carlo
Published: January 2005

Carbamazepine‐induced cardiac conduction disturbance is reported in 2 patients suffering from epilepsy. The cardiac defects disappeared in both patients after carbamazepine was discontinued, and reappeared in 1 patient after treatment was resumed.
A rating scale for amyotrophic lateral sclerosis: Description and preliminary experience
Authors: Appel V. , Stewart S. S. , Smith G. , Appel S. H.
Published: January 2005

A rating scale has been developed to provide a quantitative estimate of clinical status and disease progression in amyotrophic lateral sclerosis (ALS). This scale includes assessment of swallowing, speech, and respiratory function, and both strength and function of upper and lower extremity musculature. The evaluation is relatively simple to perform and yields reproducible data for both a total ALS score and a score for each group of functions tested. A score of 30 points is normal; 164 points indicates maximal dysfunction. The total ALS score increased in a linear fashion in each of 74 patients followed for at least one year. Among patients, the rate of disease progression varied twenty‐fold, with a continuous distribution from the slowest to most rapid course. Thirty‐four percent of patients exhibited a rapid change of greater than 48 points in the year, predicting progression to a terminal stage in less than two years; 19% of patients exhibited a slow change of less than 13 points in one year, predicting progression to a terminal stage over at least five years. This ALS scoring system should permit more accurate assessment of drug efficacy in clinical trials ánd correlation of rates of progression with clinical variables.
The natural history of Tourette syndrome: A follow‐up study
Authors: Erenberg Gerald , Cruse Robert P. , David Rothner A.
Published: January 2005

Initial reports described Tourette syndrome as a lifelong disorder. Since then, others have noted that some patients experience remissions during late adolescence. To examine this issue, we sent questionnaires to 99 patients with Tourette syndrome who were 15 to 25 years old. The majority of the 58 respondents indicated that they had fewer tics as they reached late adolescence or young adulthood. Although most reported associated behaviour or learning problems, the majority felt they were coping well. The long‐term outcome in many patients with Tourette syndrome may be more optimistic than previously reported.
Familial cases of HTLV‐I—associated myelopathy
Authors: Miyai Ichiro , Saida Takahiko , Fujita Masashi , Kitahara Yoshisuke , Hirono Nobutsugu
Published: January 2005

We studied two familial cases of human T‐lymphotropic virus type I (HTLV‐I)—associated myelopathy from the Kii Peninsula, an area of endemic adult T‐cell leukemia‐lymphoma (ATLL) in Japan. Incidence of familial clustering of HTLV‐I—associated myelopathy was about 20%. Type C retrovirus was isolated from cultured cerebrospinal fluid and peripheral blood lymphocytes in both cases. Modes of transmission seem to be similar to those described in ATLL, although there are no reports of both HTLV‐I—associated myelopathy and ATLL occurring in the same family. We suggest three possibilities: (1) that the virus associated with HTLV‐I—associated myelopathy is different from the virus causing ATLL, although they seem to be morphologically and immunologically similar; (2) that HTLV‐I—associated myelopathy may be determined by the ATLL‐causing virus plus a specific genetic background; and (3) some combination of factors 1 and 2.
Marked loss of cerebral galactolipids in Pick's disease
Authors: Scicutella Angela , Davies Peter
Published: January 2005

Studies with two monoclonal antibodies reactive with galactolipids suggest that there is a marked loss of these compounds in the brains of patients with Pick's disease. Quantitative assays, immunocytochemical studies, and thinlayer chromatography confirm that both galactocerebrosides and sulfatides are selectively lost in this condition. This finding appears to be specific for Pick's disease, distinguishing it from Alzheimer's disease and other dementing disorders.
Soluble IL‐2 receptors in acute and subacute encephalitis
Authors: Boutin B. , Matsuguchi L. , Lebon P. , Ponsot G. , Artbuis M. , Nelson D.‐L.
Published: January 2005

Elevated levels of soluble interleukin‐2 receptors were present in the serum from patients with acute primary and postinfectious encephalitis and subacute sclerosing panencephalitis. In addition, soluble interleukin‐2 receptors were detected in the cerebrospinal fluid from patients with acute primary encephalitis. Their presence in the cerebrospinal fluid was not explained by damage to the blood—brain barrier and our data attest to their local origin. This suggests that it may be possible in certain neurological diseases to detect cerebral Tlymphocyte activation through a specific marker in the cerebrospinal fluid.
Anatomy of cholinesterase inhibition in Alzheimer's disease: Effect of physostigmine and tetrahydroaminoacridine on plaques and tangles
Authors: Mesulam M.‐Marsel , Geula Changiz , Asuncion Morán M.
Published: January 2005

The histochemical distribution of cholinesterases in the cerebral cortex and their response to cholinesterase inhibitors such as physostigmine and tetrahydroaminoacridine (THA) were investigated in brains from patients with Alzheimer's disease and control subjects. In the temporal neocortex of the control subjects, most of the cholinesterase activity was located within axons and cell bodies belonging to cholinergic pathways. In keeping with their well‐known cholinomimetic effects, physostigmine and THA effectively inhibited this cholinesterase activity. Cholinesterase‐containing normal axons (and in some cases cells) were severely depleted in the cerebral cortex of patients with Alzheimer's disease. Although the cerebral cortex of these patients continued to display abundant cholinesterase activity, the location of this enzyme was largely shifted to the neuritic plaques and neurofibrillary tangles. In fact, the majority of these pathological structures demonstrated intense acetylcholinesterase and butyrylcholinesterase activities. Physostigmine and THA were potent inhibitors of these plaque‐ and tangle‐bound cholinesterases as well. In patients with Alzheimer's disease, cholinesterase inhibitors would therefore appear to have a major and widespread effect directly upon the enzymatic activity of plaques and tangles. Consequently, the clinical effects of anticholinesterases in Alzheimer's disease may be based on mechanisms that are different from those that apply to the normal brain.
The metabolic pathology of the AIDS dementia complex
Authors: Rottenberg D. A. , Moeller J. R. , Strother S. C. , Sidtis J. J. , Navia B. A. , Dhawan V. , Ginos J. Z. , Price R. W.
Published: January 2005

A progressive dementing illness, the AIDS dementia complex (ADC) is the most frequent neurological complication of the acquired immunodeficiency syndrome. Characteristic alterations in regional cerebral metabolic rate for glucose (rCMRGlc), associated with the presence or progression of ADC, were demonstrated by {18F} fluorodeoxy glucose/positron emission tomography in 9 of 12 patients with ADC compared with 18 normal volunteer subjects. In these 9 patients, two distinct patterns of regional metabolic activity were highly correlated with intersubject gray matter rCMRGlc variation and with disease severity as assessed by neuropsychological testing. Relative subcortical (thalamus and basal ganglia) hypermetabolism was characteristic of early ADC, and disease progression was accompanied by cortical and subcortical gray matter hypometabolism.
Efficiency of plasma exchange in Guillain‐Barré syndrome: Role of replacement fluids
Authors:   French Cooperative Group on Plasma Exchange in Guillain‐Barrk Syndrome
Published: January 2005

The goals of this multicenter controlled trial were: (1) to study the short‐term effect of plasma exchange in the Guillain‐Barré syndrome when applied alone within 17 days of onset of the disease; and (2) to compare two replacement fluids, diluted albumin and fresh frozen plasma (FFP) with regard to efficacy and morbidity. Two hundred twenty patients were included, 111 in the control group, 109 in the plasma exchange group, 57 of whom were assigned to receive albumin and 52 to receive fresh frozen plasma. Treatment consisted of four plasma exchanges of two plasma volumes each, initiated on the day of randomization and repeated on alternate days. Significant short‐term benefits appeared in the group that received plasma exchange as demonstrated by the reduction in the proportion of patients who required assisted ventilation after randomization, and the decrease in time before beginning weaning from ventilator, time to onset of motor recovery, and time to walk with and without assistance. No statistically significant difference was found between the group that received albumin and the group that received fresh frozen plasma. Incidents during exchanges and complications related to the plasma exchange were more frequent in patients who received fresh frozen plasma. Plasma exchange is beneficial in Guillain‐Barré syndrome when it is carried out early in the course of the disease. Given its risks and the lack of its clear superiority over albumin, however, we recommend that fresh frozen plasma be abandoned in the treatment of Guillain‐Barré syndrome.
Ultralow vestibuloocular reflex time constants
Authors: Baloh R. W. , Beykirch K. , Tauchi P. , Yee R. D. , Honrubia V.
Published: January 2005

We report detailed oculomotor studies in 3 patients with central nervous system lesions and markedly decreased time constants (< 2 seconds) of the vestibuloocular reflex (VOR). In 1 patient with Chiari type I malformation, serial measurements over 3 years documented a progressive decrease in the duration of postrotatory nystagmus (100 deg/sec steps, acceleration 140 deg/sec2) until finally there was no sustained nystagmus. At this time, the patient had no response to caloric stimulation or to sinusoidal rotation below 0.2 Hz but normal gain (peak slow‐phase eye velocity/peak chair velocity) above 0.4 Hz (phase lead increased). Gaze holding, saccades, smooth pursuit, and optokinetic nystagmus were normal, but optokinetic‐after‐nystagmus disappeared. The other 2 patients (combined brainstemcerebellar atrophy) had impaired gaze holding, abnormal smooth pursuit and optokinetic nystagmus, and absent optokinetic‐after‐nystagmus. VOR gain to step and high‐frequency sinusoidal stimuli was increased. The neural mechanism that normally prolongs the VOR time constant may have reduced it in our patients.
Increases in both cerebral glucose utilization and blood flow during execution of a somatosensory task
Authors: Ginsberg Myron D. , Chang Jen Y. , Kelley Roger E. , Yoshii Fumihito , Barker Warren W. , Ingenito Gary , Boothe Thomas E.
Published: January 2005

To investigate local metabolic and hemodynamic interrelationships during functional activation of the brain, paired studies of local cerebral glucose utilization (lCMRGlc) and blood flow (lCBF) were carried out in 10 normal subjects (9 right‐handed, 1 ambidextrous) at rest and during a unilateral discriminative somatosensory/motor task—palpation and sorting of mah‐jongg tiles by engraved design. The extent of activation was assessed on the basis of percentage difference images following normalization to compensate for global shifts. The somatosensory stimulus elevated lCMRGlc by 16.9 ± 3.5% (mean ± standard deviation) and lCBF by 26.5 ± 5.1% in the contralateral sensorimotor cortical focus; smaller increments were noted in the homologous ipsilateral site. The increments of lCMRGlc and lCBF correlated poorly with one another in individual subjects. Stimulation of the right hand resulted in significantly higher contralateral lCMRGlc activation (19.6%) than did stimulation of the left hand (14.1%) (p < 0.005), whereas the lCBF response was independent of the hand stimulated. Our results indicate that both glycolytic metabolism and blood flow increase locally with the execution of an active sensorimotor task and suggest that both measures may serve as reliable markers of functional activation of the normal brain.
Alternating skew on lateral gaze (Bilateral abducting hypertropia)
Authors: Moster Mark L. , Schatz Norman J. , Savino Peter J. , Benes Susan , Bosley Thomas M. , Sergott Robert C.
Published: January 2005

We report thirty‐three patients with alternating skew deviation on lateral gaze. The right eye was hypertropic in right gaze, and the left eye was hypertropic in left gaze. Most patients had associated downbeat nystagmus and ataxia and were diagnosed as having lesions of the cerebellar pathways or the cervicomedullary junction. This contrasts with a previous report in which alternating skew was seen mainly in lesions of the midbrain pretectum.