bluuebunny/crossref_metadata_2025 huggingface dataset:
Cerebellar degeneration caused by high‐dose cytosine arabinoside: A clinicopathological study
Authors: Winkelman Marc D. , Hines John D.
Published: January 2005

Twenty‐four patients with leukemia or lymphoma refractory to conventional chemotherapy were given a course of systemic, high‐dose cytosine arabinoside (3 gm/m2 every 12 hours for twelve doses). Four patients developed cerebellar degeneration during treatment. Ataxia of gait and limb movements, dysarthria, and nystagmus appeared five to seven days after the first dose, worsened over the next two to three days, and then remained stable for two to six days. Incomplete improvement occurred over the following one to two weeks. Postmortem examination disclosed loss of Purkinje cells in the depths of cortical sulci with relative preservation of those at the crests of folia and those in the most posterior inferior portions of the cerebellum. Other patients developed a mild, reversible cerebellar syndrome over the same time course as that of the irreversible disorder. Manifestations ranged from nystagmus alone to dysarthria and unsteadiness of gait without limb ataxia. We conclude that cytosine arabinoside at this dosage causes a cerebellar degeneration with characteristic clinical and pathological features. Among the present patients with refractory hematological malignancies, such degeneration occurred with an incidence of 16.7%, more than twice that reported in previous series.
Herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: Diagnosis and management approaches
Authors: Hilt Dana C. , Buchholz David , Krumholz Allan , Weiss Howard , Wolinsky Jerry S.
Published: January 2005

Four patients with herpes zoster ophthalmicus and delayed contralateral hemiparesis are described, and their findings are compared with those in patients previously reported in the English language literature. The current patients evidenced multifocal ipsilateral cerebral angiitis by angiography and multifocal infarcts in the distribution of the ipsilateral middle cerebral artery by computed tomographic scanning. Cerebrospinal fluid showed mononuclear pleocytosis, positive oligoclonal bands, and an elevated immunoglobulin G index. Two patients were treated with corticosteroids and acyclovir, and 1 with corticosteroids alone, all without apparent response. Necrotizing angiitis ipsilateral to the herpes zoster ophthalmicus was demonstrated postmortem in 1 patient with multifocal cerebral infarction and progressive leukoencephalopathy. Neither herpes varicella zoster immunocytochemical reactivity nor viral inclusions were seen. The leukoencephalopathy associated with herpes varicella zoster either may be caused by cerebral angiitis or, as previously reported, may be a temporally remote manifestation of persistent herpes varicella zoster infection. The cerebral angiitis associated with herpes varicella zoster is histologically similar to granulomatous angiitis, and both may be related to herpes varicella zoster infection of the cerebral vasculature.
Immunoreactive myelin proteolipid protein—like activity in cerebrospinal fluid and serum of neurologically impaired patients
Authors: Trotter John L. , Wegescheide Cindy L. , Garvey William F.
Published: January 2005

Two hundred seventy‐eight samples of cerebrospinal fluid and 273 samples of sera from a variety of neurological patients were examined by radioimmunoassay for myelin proteolipid protein–like reactivity as an index of structural damage to myelin. Groups of patients with active central nervous system damage could be distinguished easily, although there was a small percentage of false positive reactions. The reactivity was most marked in the demyelinative, encephalitic, and stroke categories. A radioimmunoassay for proteolipid protein of proper sensitivity and specificity may prove to be a useful objective clinical measure of the presence and degree of disease activity in multiple sclerosis and other diseases.
External ophthalmoplegia, alpha and spindle coma in imipramine overdose: Case report and review of the literature
Authors: Pulst Stefan‐M. , Lombroso C. T.
Published: January 2005

A 13‐year‐old boy with imipramine overdose developed seizures, respiratory arrest, and coma. Abnormalities of oculovestibular reflexes, electroencephalograms, and brainstem auditory evoked potentials were monitored in relation to measurements of drug levels. An alpha‐coma electroencephalographic pattern evolved into one evidencing spindle coma and eventually into a normal pattern. Prolonged brainstem auditory evoked potentials also normalized as coma and oculocephalic reflex abnormalities resolved. In spite of the history that suggested hypoxic damage, the absence of reflex eye movements in a comatose patient and the presence of alphaand spindle‐coma electroencephalographic patterns, even with prolonged brainstem auditory evoked potentials, are not reliable prognostic indicators in tricyclic drug overdose.
Cerebral vasospasm after subarachnoid hemorrhage: An update
Authors: Heros Roberto C. , Zervas Nicholas T. , Varsos Vassilios
Published: January 2005

Symptomatic vasospasm, or delayed cerebral ischemia associated with arteriographic evidence of arterial constriction, is currently the most important cause of morbidity after acute subarachnoid hemorrhage. The development of vasospasm is directly correlated with the presence of thick blood clots in the basal subarachnoid cisterns, which can be detected by an early computed tomographic scan. Symptomatic vasospasm usually develops between 4 and 12 days after subarachnoid hemorrhage. The onset is gradual, occurring over hours or days. There is typically a gradual deterioration of the level of consciousness, accompanied by focal neurological deficits that are determined by the arterial territories involved. Hyponatremia frequently occurs and may exacerbate the symptoms. The patients are usually volume depleted, and therefore many authorities now treat them with replenishment and expansion of their intravascular volume with colloid and blood. Volume expansion, together with elevation of the systemic blood pressure and reduction of the intracranial pressure when elevated, constitute the only currently available effective therapy for symptomatic vasospasm. The cause of vasospasm remains obscure. Mechanisms of smooth muscle cell contraction and relaxation and experimental efforts to elucidate the nature of vasospasm are reviewed.
Gerstmann‐Sträussler‐Scheinker disease with coincidental familial onset
Authors: Hudson Arthur J. , Farrell Michael A. , Kalnins Renate , Kaufmann John C. E.
Published: January 2005

A family with Gerstmann‐Sträussler‐Scheinker disease had coincidental clinical onset in three members of two generations, a phenomenon suggesting a common source of a transmissible agent. A regular dietary supplement in this family was home‐bred rabbit. The clinical picture, although generally similar to that in previous accounts, included the unusual findings of visual loss (one patient) and sensory loss (one patient), and dementia was not apparent until late in the illness in two patients. Pathological examination of a cerebellar cortical biopsy specimen from one patient and postmortem tissue from two patients revealed multicentric amyloid plaques located in cerebral and cerebellar cortex, basal ganglia, and white matter with degeneration of corticospinal, dorsal spinocerebellar, dentatorubral, and geniculocalcarine tracts and dorsal columns. Spongiform change was focal and confined to the superficial cerebral cortical layers.
Coma and thyrotoxicosis
Authors: Newcomer John , Haire William , Hartman Charles R.
Published: January 2005

A patient with Graves' disease was in coma with evidence of corticospinal tract disease. Treatment with standard pharmacological measures and plasmapheresis resulted in the reduction of total thyroxine from 28.8 μg/ dl to 10.0 μg/dl and of triiodothyronine from 332 ng/dl to 150 ng/dl. These laboratory findings were associated with clinical stabilization, improvement of mental status, and resolution of the findings of corticospinal tract disease. These data support previous contentions relating reversible corticospinal tract disease to thyrotoxicosis.
Collateral reinnervation of sweat glands
Authors: Kennedy William R. , Sakuta Manabu
Published: January 2005

The collateral reinnervation of mouse sweat glands has been studied by a method that allows serial evaluation of the course of reinnervation in intact animals. The method is based on the finding that the activation of secretion from newly denervated sweat glands by pilocarpine or nerve stimulation is completely absent seven days after nerve section but returns with reinnervation. These characteristics allowed serial detection of footpad sweat glands newly reinnervated by collateral sprouting of the remaining intact saphenous nerve after section of the sciatic nerve. The number of saphenous innervated glands increased five‐to sevenfold and the total saphenous sweat territory was greatly expanded across the volar surface of the hind paw. There was less enlargement when the sciatic nerve was allowed to regenerate and participate in reinnervation of sweat glands. None of the reinnervated glands were dually innervated by saphenous and sciatic nerves, as is the case in normal glands. When the saphenous nerve was sectioned after saphenous collateral sprouting was complete and after sciatic regeneration had innervated an apparent maximal number of glands, the sciatic nerve reacted by advancing farther into the formerly enlarged saphenous territory and re‐reinnervated many of the glands. Collateral sprouting by sudomotor axons is more abundant and more widely dispersed than reported for larger nerve fibers to skeletal muscle and to low‐threshold mechanoreceptors, It more closely resembles sprouting of nociceptive axons.
Comparison of cognitive ability, personality profile, and school success in epileptic children with pure right versus left temporal lobe eeg foci
Authors: Camfield Peter R. , Gates Robert , Ronen Gabriel , Camfield Carol , Ferguson Anne , Macdonald G. Wayne
Published: January 2005

By reviewing electroencephalographic reports compiled over five years and checking clinical details, we identified and studied 27 children with apparently pure left or pure right temporal lobe epilepsy. The children underwent neurological examination and neuropsychological testing (Halstead‐Reitan Battery, Wechsler Intelligence Scale for Children [WISC] and Wide Range Achievement Test), and parents completed the Personality Inventory for Children (PIC). Of 15 boys and 12 girls with a mean age of 12.7 years, 14 had right and 13 left temporal lobe epilepsy. Seizure control was excellent in 17. Five (2 with left, 3 with right temporal lobe epilepsy) had received professional emotional help, 6 (3 with left, 3 with right focus) received special help at school, and 5 (3 with left, 2 with right focus) had failed a grade in school. No significant left–right differences were seen in WISC full scale, verbal, or performance IQ scores, neuropsychological test battery findings, or clinical scale scores on the PIC. Most of the children with unilateral temporal lobe epilepsy had no measurable cognitive or emotional difficulties. Cognitive, personality, and school problems were equally distributed between left and right temporal lobe epilepsy groups. When the two groups were combined, however, 10 patients (5 with left, 5 with right focus) were identified by the PIC as maladjusted, and as a group showed significantly lower neuropsychological test functioning than did the normally adjusted children.
Age at onset of seizures in young children
Authors: Ellenberg Jonas H. , Hirtz Deborah G. , Nelson Karin B.
Published: January 2005

Age at onset of seizures in young children and its relationship to factors such as prior neurological status and neurological outcome were examined. Of 52,360 children, 39,270 of whom were followed for the full 7 years, a total of 2,635 experienced one or more seizures between birth and 7 years of age. The incidence of nonfebrile convulsions was highest in the first year of life, especially in the first month. Children with neonatal seizures who later developed nonfebrile seizures did so early, two‐thirds by 6 months and three‐quarters by 1 year of age. Children with neurological or developmental abnormality assessed in the first year of life did not have their first seizure earlier than children without abnormality. Neurological abnormality in the first year of life before any seizure, and the presence of minor motor seizures, were associated with an increased rate of mental retardation and cerebral palsy at age 7, but early age at onset appeared to have little prognostic value regarding intellectual function, cerebral palsy, and epilepsy.
Deafness in Cockayne's syndrome: Morphological, morphometric, and quantitative study of the auditory pathway
Authors: Gandolfi A. , Horoupian D. , Rapin I. , Deteresa R. , Hyams V.
Published: January 2005

The auditory pathway of a 17‐year‐old deaf patient with Cockayne's syndrome was examined histologically. The cochlea showed marked atrophy of the spiral ganglion and attenuation of the cochlear division of the eighth cranial nerve. By means of the Computer Image Analyzer, the total number of neurons in the ventral cochlear nucleus was found to be reduced from 30,440 to 18,821. The mean diameter of the neurons in the ventral cochlear nucleus, medial dorsal olivary nucleus, and inferior colliculus was smaller than in a control patient, whereas in the medial geniculate nucleus and anterior transverse gyrus of Heschl, the neuronal size approximated the norm. The changes in the first three auditory relay nuclei were considered to represent transsynaptic atrophy caused by degeneration of the spiral ganglion and, possibly, the cochlear neuroepithelium. This histological report verifies that deafness in Cockayne's syndrome is largely sensorineural and that degeneration of spiral ganglion in humans can lead to a chain of trans‐synaptic degeneration in the ventral cochlear nucleus, medial dorsal olivary nucleus, and inferior colliculus.
Bilateral carotid artery occlusion resulting from giant cell arteritis
Authors: Howard George F. , Ho Sam U. , Kim Kwang S. , Wallach Jami
Published: January 2005

A 65‐year‐old woman with subacute global deterioration of neurological function had bilateral occlusion of the internal carotid arteries demonstrated by angiography. Postmortem examination showed isolated giant cell arteritis involving the carotid siphon bilaterally.
Pure hemidystonia with basal ganglion abnormalities on positron emission tomography
Authors: Perlmutter Joel S. , Raichle Marcus E.
Published: January 2005

We present a patient with hemidystonia and an abnormality of the contralateral basal ganglion seen only with positron emission tomography. A 50‐year‐old sinistral man suffered minor trauma to the right side of his head and neck. Within 20 minutes he developed paroxysmal intermittent dystonic posturing of his right face, forearm, hand, and foot, with weaker contractions of the left foot, lasting several seconds and recurring every few minutes. Neurological findings between spells were normal. The following were also normal: electrolyte, calcium, magnesium, and arterial blood gas levels, and findings of drug screen, cerebrospinal fluid examination, electroencephalography with nasopharyngeal leads, computed tomographic scanning (initially and four weeks later), and cerebral angiography. Positron emission tomographic scanning revealed abnormalities in the left basal ganglion region, including decreased oxygen metabolism, decreased oxygen extraction, increased blood volume, and increased blood flow.
A serial study of peripheral blood T lymphocyte subsets in relapsing–remitting multiple sclerosis
Authors: Kastrukoff Lorne F. , Paty Donald W.
Published: January 2005

Twelve patients with relapsing‐remitting multiple sclerosis and 3 control subjects were evaluated weekly for six months. Peripheral blood lymphocyte subsets were enumerated using monoclonal antibodies and analysis with a duallaser fluorescence‐activated cell sorter. Results were correlated with clinically assessed disease activity. Fluctuations occurred in the number of T lymphocytes (Leu 1+) and cells with the helper/inducer phenotype (Leu 3a/OKT4+) in both patients and controls. Fluctuations in the number of cells with the suppressor/cytotoxic (Ts/c) cell phenotype (OKT5, OKT8, Leu 2a+) also occurred. Variations in the relative numbers of cells labeled with all three Ts/c monoclonals did occur, but with OKT5 generally labeling fewer cells than OKT8 or anti–Leu 2a. When findings were correlated with disease activity, OKT5 was a more sensitive guage of disease activity than either OKT8 or anti–Leu 2a. When peripheral blood lymphocyte subsets were correlated with disease activity, the following patterns were observed: a reduction in the number of Ts/c cells without evidence of clinical disease (four episodes), the development of new symptoms suggestive of an acute relapse but without a reduction in the number of Ts/c cells (two episodes), and a reduction in the number of Ts/c cells associated with acute relapse (two episodes). In the two patients with acute relapse and a reduction in the number of Ts/c cells, clinical disease preceded a reduction in the number of Ts/c cells in 1 patient, whereas the two events occured simultaneously in the other patient. Taken together, these limited results indicate that in some cases a reduction in the number of Ts/c cells may be the result rather than the cause of disease activity and may represent an apiphenomenon.
Effect of high–dose intravenous steriod administration on contrast‐enhancing computed tomographic scan lesions in multiple sclerosis
Authors: Troiano Raymond , Hafstein Marino , Ruderman Marvin , Dowling Peter , Cook Stuart
Published: January 2005

Six consecutive patients with multiple sclerosis and lesions contrast enhancing on computed tomographic scan were treated with high‐dose intravenous infusions of methylprednisolone. Double‐dose delayed computed tomographic scans were repeated at varying intervals during corticosteriod treatment. Contrast enhancement of sclerotic plaques was reduced or eliminated within as little as 8 hours after the first infusion. Rapid associated partial clinical improvement was noted in four patients with recent exacerbations of disease activity. Similar attenuation of contrastenhancing plaques and clinical improvement were less predictable following oral corticosteriod therapy in conventional dosage. These studies suggest that the dose, route of administration, and duration of corticosteriod therapy in multiple sclerosis may partially determine the effect of treatment.
Neuronal intranuclear inclusion disease in identical twins
Authors: Haltia Matti , Somer Hannu , Palo Jorma , Johnson William G.
Published: January 2005

A pair of female identical twins exhibited slurred speech, nystagmus, and oculogyral spasms starting at age 11. The patients then had episodic rage, extrapyramidal and lower motor neuron abnormalities, and grand mal seizures, but retained largely normal intelligence, until death at age 21. Severe loss of nigral and craniospinal motor neurons was noted postmortem. Round, eosinophilic, autofluorescent inclusion bodies, 3 to 10 μm in diameter, were observed in the nuclei of mmilanost nerve cell types of the central and peripheral nervous systems and retina. Ultrastructurally the inclusions appeared as masses of filaments without a limiting membrane, the constituent filaments having a diameter of 8.5 to 9.5 nm. Histochemical results suggested the presence of proteins with a high content of tryptophan. Four similar cases have been reported previously under various designations. We propose the name neuronal intranuclear inclusion disease for the disorder.
Measles encephalomyelitis: Lack of evidence of viral invasion of the central nervous system and quantitative study of the nature of demyelination
Authors: Gendelman Howard E. , Wolinsky Jerry S. , Johnson Richard T. , Pressman Norman J. , Pezeshkpour Gholam H. , Boisset Gerardo F.
Published: January 2005

Measles encephalomyelitis appears to be an immune‐mediated parainfectious disorder, but it is unclear whether viral invasion of brain is an obligate step in its development. Immunocytochemical methods were used to search for virus antigen in formalin‐fixed, paraffin‐embedded central nervous system (CNS) tissues from 10 patients with measles encephalomyelitis and 12 patients who had died of measles without CNS involvement. All the CNS tissues studied were viral antigen negative. Similarly fixed CNS tissues from all of 6 patients with subacute sclerosing panencephalitis were viral antigen positive and served as controls. The pattern of perivenular demyelination was also determined in 4 cases of measles encephalomyelitis using antibodies to myelin associated glycoprotein and myelin basic protein and a Luxol fast blue stain. Areas of demyelination in serial sections were quantitated, and no morphometrical differences were found among tissues stained with the three stains.

The data suggest the lack of virus replication in the CNS during encephalomyelitis or fatal measles without CNS symptoms. The pattern of loss of myelin associated glycoprotein and myelin basic protein in regions of perivenular demyelination resembles that reported in experimental allergic encephalomyelitis. This pattern of demyelination has been proposed to result from a primary attack on the myelin sheath rather than from direct involvement of the oligodendroglial cell.
Opioid‐induced epileptogenic phenomena: Anatomical, behavioral, and electroencephalographic features
Authors: Chugani Harry T. , Ackermann Robert F. , Chugani Diane C. , Engel Jerome
Published: January 2005

Recent animal studies have indicated a possible role of opioids in epilepsy. Intraventricular opioid administration induces a prolonged nonconvulsive stuporous state characterized by epileptiform electroencephalographic patterns, and reversed by naloxone. In high doses, naloxone itself causes generalized clonic convulsions. We compared opioid‐induced and naloxone‐induced epileptogenic phenomena using quantitative 2‐deoxyglucose autoradiography in order to define the anatomical structures involved in these two different seizure types. When opioid‐induced seizures occurred, limbic structures were preferentially activated, but when naloxone‐induced clonic convulsions occurred, pyramidal and extrapyramidal motor areas and some limbic structures were activated. Based on the present experiments and currently available evidence, we speculate that opioid‐mediated epileptogenic phenomena are similar to those occurring during the postictal state of a fully kindled seizure, whereas naloxone‐induced epileptogenic phenomena are similar to the ictal state. Therefore, simple pharmacological manipulation of endogenous opioid systems may allow selective study of ictal and postictal phenomena.
Rebound nystagmus in olivocerebellar atrophy: A clinicopathological correlation
Authors: Bondar Roberta L. , Sharpe James A. , Lewis Anthony J.
Published: January 2005

Rebound nystagmus was recorded in a patient with cerebellar dysfunction. Neuropathological examination showed degeneration of the inferior olivary nuclei and the cerebellar cortex. The basis pontis and the brainstem tegmentum were spared. Rebound nystagmus is attributed to a smooth eye movement bias that is generated within intact vestibuloocular pathways to oppose gaze‐evoked centripetal drift of the eyes. The source of the rebound bias was not visual, because rebound nystagmus normally occurs in darkness, smooth pursuit was pursuit was paretic, and the olivocerebellar visual relay pathway was degenerated. An extraretinal, efference copy signal of the eye position error must detect the ocular drift and generate the rebound bias. The neuropathological findings indicate that rebound nystagmus can be a sign of involvement confined to the olivocerebellar circuit and that nonvisual signals of eye position are monitored independently of this cerebellar system.
“Locked‐in” state following Reye's syndrome
Authors: Kotagal Suresh , Rolfe Ursula , Schwarz Kathleen B. , Escober Wilfredo
Published: January 2005

A 12‐year‐old boy with Reye's syndrome had an initial course complicated by increased intracranial pressure and systemic hypotension. He subsequently developed brainstem damage producing permanent apnea and a “locked‐in” state. Nevertheless, the normal architecture of sleep was retained, as was a high degree of intelligence.
Suprasegmentally induced motor unit activity in paralyzed muscles of patients with established spinal cord injury
Authors: Dimitrijevic Milan R. , Dimitrijevic Meta M. , Faganel Janez , Sherwood Arthur M.
Published: January 2005

In an attempt to demonstrate the presence of functional descending fibers in patients with clinically apparent functional spinal cord transection, we examined electromyographically recorded paralyzed leg muscle responses to the Jendrassik and other reinforcement maneuvers. Two patterns were observed: a low‐amplitude, short onset time reinforcement maneuver response (RMR) restricted to one to three muscle groups (RMR1), and a larger‐amplitude response with a longer onset time that occurred bilaterally in essentially all of the recorded muscles (RMR2). The responses imply preserved descending facilitory influence on isolated populations of motor units (RMR1) or on segmental interneuron pools (RMR2). Such findings indicate the presence of functioning fibers traversing the injured portion of the spinal cord in patients diagnosed as having a complete lesion. In such cases, it is possible for patients to initiate subclinical motor unit activity or suprasegmentally induced gross movement through reinforcement maneuvers, but not to control the amplitude or duration of the response.
Cerebral blood flow and regional potassium distribution during focal ischemia of gerbil brain
Authors: Mies G. , Kloiber O. , Drewes L. R. , Hossmann K.‐A.
Published: January 2005

In 8 gerbils (Meriones unguiculatus) focal cerebral ischemia was produced by occlusion of the left common carotid artery and the opposite external carotid artery. After two hours blood flow was measured with iodoantipyrine labeled with carbon 14, and evaluated by means of quantitative autoradiography. Thereafter the same brain sections were stained for regional potassium by means of a histochemical technique. Changes in tissue potassium content were assessed by measuring the differences in optical densities in homotopic brain regions of the stained sections. The correlation between blood flow and tissue potassium level revealed that below a flow threshold of about 0.23 ml/gm/min, a definite potassium loss from the tissue was observed. The combination of autoradiographic methods with a technique for measuring the regional distribution of potassium may be useful in providing additional information on the occurrence of disturbed electrolyte homeostasis after the onset of focal ischemia.
Cranial nuclear magnetic resonance imaging in head trauma
Authors: Gandy Samuel E. , Snow Robert B. , Zimmerman Robert D. , Deck Michael D. F.
Published: January 2005

Nuclear magnetic resonance imaging revealed focal intracranial lesions consisting of extraaxial hematomas or nonhemorrhagic contusions in 3 patients with severe neurological disability following head trauma. In all 3, standard skull radiography and computed tomography had been unremarkable.
Neuroepidemiology
Authors: Kurtzke John F.
Published: January 2005

Epidemiology is the study of the natural history of disease, which includes its frequency, severity, and course and the identification of “risk factors” that influence these aspects. Neuroepidemiology is that branch of epidemiology dealing with disorders that affect the nervous system. Frequency of disease is best measured by population‐based rates, which are ratios of the number of cases to the population at risk, expressed as cases per unit of population. Incidence and mortality rates refer, respectively, to new cases of, and deaths caused by, a disease per unit of time and population. Prevalence rate is the cross‐sectional count of cases of a disease present at a given time per unit of population. Comparison of rates often requires consideration of age‐specific or age‐adjusted rates rather than crude rates, all ages. Course of illness (survival, complications, recurrence) can be measured by life‐table methods. The annual incidence for disease and injury of the nervous system is about 2.5% (2,500 per 100,000 population) and the prevalence is about 9.5%. Excluding all traumatic, pain, headache, alcohol, psychiatric, and special‐sense disorders, neurological diseases have an annual incidence and prevalence rate, respectively, of 1.1 and 3.6% of the population.
Epidemiology of parkinsonism: Incidence, classification, and mortality
Authors: Rajput A. H. , Offord Kenneth P. , Beard C. Mary , Kurland Leonard T.
Published: January 2005

An epidemiological study of parkinsonism over a 13‐year period (1967 through 1979) is presented, updating previous reports on incidence and trend in the population of Rochester, Minnesota. The overall average annual incidence of parkinsonism per 100,000 population was 20.5, adjusted to the 1970 total United States population, which is virtually unchanged from previous observations. Incidences calculated for each calendar year (1967 through 1979) revealed no remarkable change following the 1976 swine flu vaccination program. There was no sex difference and the peak incidence occurred between ages 75 and 84 years. Idiopathic Parkinson's disease was the most common variant (86%), followed by drug‐induced parkinsonism (7%). There were no new cases of postencephalitic parkinsonism diagnosed during the study period. Relative frequency of other types of Parkinson's disease as identified by practicing neurologists is presented. For each case two age‐ and sex‐matched controls were selected from the Rochester population. The survival rates in the controls were comparable to the general population of the west north central region of the United States. The mortalities in the patients were significantly higher (p= 0.001) than the controls and were unchanged from previous rates reported from the same community. In the 69 (50%) patients treated with levodopa, the mortality was comparable to that in controls. The favorable outcome in these cases is attributed to bias resulting from selection of healthier patients for treatment.
Dementia and motor neuron disease: Morphometric, biochemical, and Golgi studies
Authors: Horoupian D. S. , Thal L. , Katzman R. , Terry R. D. , Davies P. , Hirano A. , DeTeresa R. , Fuld P. A. , Petito C. , Blass J. , Ellis J. M.
Published: January 2005

In three patients dementia without neurofibrillary tangles or Pick bodies antedated amyotrophy by several years. The motor neuron disorder in two patients was characterized by terminal bulbar symptoms; in one it was similar to classic amyotrophic lateral sclerosis. In two patients, quantitative studies of selected regions of the cortex using a computerized image analyzer disclosed, as in patients with senile dementia of Alzheimer type, a marked reduction in the number of neurons, especially those larger than 90 μ2. The findings differed from those in Alzheimer dementia, however, in that the cells in the substantia innominata were not reduced and the levels of choline acetyltransferase and somatostatin‐like immunoreactivity, determined in one patient, were within normal limits. A variable degree of sponginess of the upper layers of the cortex was attributed to attrition of pyramidal cell dendrites, observed in the one patient in whom Golgi study was successful. Because of severe degeneration of the substantia nigra in all three, the disease in these patients may represent a subset of motor neuron disease or a multisystem atrophy.
Cerebral physiological and metabolic effects of hyperventilation in the neonatal dog
Authors: Young Richard S. K. , Yagel Susan K.
Published: January 2005

To clarify the changes that occur during marked hypocarbia in the neonate, we measured brain blood flow and metabolite levels after 90 minutes of hyperventilation in neonatal dogs. Brain blood flow decreased significantly in diencephalon, brainstem, and spinal cord but not in cerebral cortex or white matter. There was no substantial change in the electroencephalogram. Lactate concentrations, both in telencephalon and in superior sagittal sinus blood, increased significantly, although there was no alteration in levels of ATP or phosphocreatine. Marked hypocarbia in the neonatal dog produces an elevated brain lactate level that may be related to changes in glycolytic rate rather than to tissue ischemia or hypoxia.
Physiological responses to focal cerebral ischemia in humans
Authors: Powers William J. , Grubb Robert L. , Raichle Marcus E.
Published: January 2005

Positron emission tomography (PET) was used to investigate the regional hemodynamic and metabolic changes that accompany focal reductions in cerebral blood flow to ischemic but uninfarcted regions of the brain. Studies were performed on 7 patients chosen from a larger group of subjects with transient ischemic attacks and normal computed tomographic findings, specifically because their PET studies showed decreased blood flow to the symptomatic hemisphere rather than symmetrical flow to the two hemispheres. In regions with decreased blood flow, the cerebral metabolic rate for oxygen was also decreased, but to a lesser degree. Cerebral blood volume, vascular mean transit time, and fractional extraction of oxygen from blood were all increased. These findings indicate that cerebral oxygen metabolism was maintained in the face of decreased blood flow by local compensatory mechanisms that included dilation of intraparenchymal blood vessels and increased transfer of oxygen from blood to tissue. Such knowledge of the physiological characteristics of ischemic, uninfarcted brain is important if PET is to be used clinically to differentiate reversible cerebral ischemia from irreversible infarction.
Fine structure of cortical tubers in tuberous sclerosis: A Golgi study
Authors: Huttenlocher Peter R. , Heydemann Peter T.
Published: January 2005

The fine structure of cerebral cortex, including cortical tubers, was studied in 3 patients with tuberous sclerosis. Tubers were found to consist of two predominant cell populations, astroglia and small multipolar (stellate) neurons. Both cell types tended to form aggregates within tubers, with glia more prominent in the subpial region. The stellate neurons of tubers had beaded or varicose dendrites with few dendritic spines. The findings suggest that neurons within tubers are an aberrant primitive cell type that fails to express the pyramidal cell shape and dendritic morphology that is characteristic of normal human neocortex. Cortex intervening between tubers had basically normal dendritic morphology. However, quantitative study showed a decrease in the length of dendritic branches of pyramidal neurons, as also observed in several other conditions manifested by mental retardation.
Galactosylceramide lipidosis: Novel presentation as a slowly progressive spinocerebellar degeneration
Authors: Thomas P. K. , Halpern J.‐P. , King R. H. M. , Patrick D.
Published: January 2005

A 34‐year‐old woman was initially seen because of a progressive neurological disorder suggestive of a spinocerebellar degeneration. This condition had a late infantile onset and was unassociated with visual impairment or dementia. Nerve conduction velocity was severely reduced. A left hemiparesis later developed. A computed tomographic scan revealed multiple periventricular hypodense lesions, suggestive of a leukodystrophy. Sural nerve biopsy demonstrated changes of a chronic demyelinating neuropathy, with inclusions typical of Krabbe's disease. This diagnosis was confirmed by the finding of reduced leukocyte galactocerebrosidase activity.
Cortical abnormalities in Alzheimer's disease
Authors: Foster Norman L. , Chase Thomas N. , Mansi Luigi , Brooks Rodney , Fedio Paul , Patronas Nicholas J. , Di Chiro Giovanni
Published: January 2005

Regional cerebral glucose metabolism, an index of neuronal activity, was compared in 20 patients with Alzheimer's disease and 8 age‐matched normal volunteers by positron emission tomography following {18F}2‐fluoro‐2‐deoxy‐D‐glucose administration. Overall cortical glucose utilization in the Alzheimer's group was 10 to 49% below that of control individuals. The posterior parietal cortex and contiguous portions of posterior temporal and anterior occipital lobes were most severely affected; frontal cortex was relatively spared. This pattern of cortical involvement is consistent with the major clinical features of Alzheimer's disease. Comparison of patients with early and more advanced dementia suggested that a substantial decline in glucose metabolism occurs before cognitive impairment becomes evident; once the patient is symptomatic, however, small additional metabolic decrements are associated with a marked deterioration in intellectual function.
The induction and reversibility of cerebral acidosis in thiamine deficiency
Authors: Hakim A. M.
Published: January 2005

Regional cerebral pH was determined autoradiographically using carbon 14‐labeled dimethyloxazolidinedione in normal rats, following various durations of thiamine deficiency and replenishment with thiamine when the clinical sequelae of the deficiency appeared. In our model the clinical sequelae of thiamine deficiency (opisthotonus) appeared on the average on day 18. Regional cerebral pH on day 12 was comparable to that in controls and ranged between 7.02 ± 0.03 and 7.09 ± 0.03 (mean ± SEM) in gray matter structures. On day 14 the pH in the inferior colliculus was 6.85 ± 0.08 and relative acidosis also appeared in thalamic structures. At opisthotonus the pH was 6.48 ± 0.17 in the mamillary body, 6.43 ± 0.14 in the vestibular nucleus, and 6.36 ± 0.14 in the medial dorsal nucleus of the thalamus (p < 0.01). One dose of thiamine replenishment at this stage transiently raised the pH in the inferior colliculus to 7.25 ± 0.19 and in the medial dorsal nucleus to 7.20 ± 0.13 (p < 0.01). Cerebral regions showing significant acidosis during thiamine deficiency coincided largely with those known to be histologically vulnerable and those previously reported to show a focal rise in local cerebral glucose utilization between days 11 and 14 of thiamine deficiency. This focal acidosis shown to occur in thiamine deficiency may be one mechanism contributing to the selective histological vulnerability in this model.
In vivo measurement of brain tumor pH using [11C]DMO and positron emission tomography
Authors: Rottenberg D. A. , Ginos J. Z. , Kearfott K. G. , Junck L. , Dhawan V. , Jarden J. O.
Published: January 2005

In vivo measurements of regional brain tissue/tumor pH (rpH) have been accomplished in 9 patients with primary or metastatic brain tumors using[11C]dimethyloxazolidinedione ([11C]DMO) and positron emission tomography. Tumor rpH values ranged from 6.88 to 7.26, whereas gray matter and white matter rpH values ranged from 6.74 to 7.09 and from 6.77 to 7.03, respectively. Our result, which are consistent with reported [14C]DMO autoradiographic measurements of brain and tumor pH, suggest that the pH microenvironment of brain tumors is not more “acidic” than that of normal gray or white matter.
Electrophysiological analysis of the mode of action of muscle relaxants in spasticity
Authors: Delwaide P. J.
Published: January 2005

Variations in four electrophysiological tests (H/M, T/M, vibratory inhibition, and recovery curve of Hoffmann's reflex following stimulation at the ankle) were studied following a single administration of four myorelaxant drugs: diazepam (10 mg intramuscularly), baclofen (20 mg intramuscularly), tizanidine (4 mg orally), and idrocilamide (60 mg intramuscularly). Fifty‐one spastic patients, divided into four groups, were tested. All four drugs reduced the H/M and T/M ratios very slightly. Only diazepam and tizanidine reinforced vibratory inhibition. Diazepam and tizanidine did not modify the abnormal recovery curves, however, whereas baclofen and idrocilamide did. Reinforcement of vibratory inhibition suggests an increase in presynaptic inhibition mediated by gamma‐aminobutyric acid; changes in recovery curves are likely due to modifications of interneuronal reactivity. Matching myorelaxants to the predominant pathophysiological abnormality detected by electrophysiological exploration may lead to better treatment of spasticity.
Hemispheric lateralization of spatial contrast sensitivity
Authors: Kobayashi Shotai , Mukuno Kazuo , Ishikawa Satoshi , Tasaki Yoshiaki
Published: January 2005

Visuospatial contrast sensitivity was determined by the Arden grating chart in 23 patients with cerebral infarctions involving the primary visual cortex or visual association cortex. Subjects were classified into three groups according to their lesions: I, 6 patients with unilateral medical occipital or occipitotemporal lesions; II, 6 patients with left lateral parieto‐occipital lesions; and III, 11 patients with right lateral parieto‐occipital lesions.

Contrast sensitivity was markedly reduced in Group III, especially in patients having hemispatial agnosia. Group I patients with hemispatial agnosia showed almost normal contrast sensitivity. Spatial contrast sensitivity appears to be more affected when the lesions has an influence on the nondominant lateral parieto‐occipital cortex.
Tetrabenazine induces acute dystonic reactions
Authors: Burke Robert E. , Reches Avinoam , Traub Michael M. , Ilson Joyce , Swash Michael , Fahn Stanley
Published: January 2005

Four patients suffered acute dystonic reactions casued by tetrabenzine. Because dystonic reactions have previously been reported only after dopamine receptor blockade and not with dopamine deplection, it is likely that the ability of tetrabenazine to induce acute dystonia is due to its dopamine receptor blocking properties. Because tertrabenazine can induce acute dystonia even when combined with α‐methyl‐p‐tyrosine, presynaptic dopamine stores may not be necessary for these reactions to occur.
Polyneuropathy and IgM monoclonal gammopathy: Studies on the pathogenetic role of anti‐myelin‐associated glycoprotein antibody
Authors: Mendell J. R. , Sahenk Z. , Whitaker J. N. , Trapp B. D. , Yates A. J. , Griggs R. C. , Qurarles R. H.
Published: January 2005

Attentionl has recently been directed toward patients having a polyneuropathy IgM anti‐Myelin‐associated glycoprotein (anti‐MAG) antibody. The possibility of a pathogenetic role for the anti‐MAG antibody in the evolution of the polyneuropathy and in the development of central nervous system signs, including tremor and ataxia, remains unresolved. In 5 patients with this syndrome whose clinical courses were followed closely, in 1 of whom a complete postmortem examination of the nervous system was performed, we made the folowing obsercvations: (1) the anti‐MAG antibody did not localize to the compact layer of the myelin sheath in affected nerves, but did realize to areas of myelin splitting; (2) anti‐MAG antibody present in the sural nerve of an affected individual for 7 years was not associated with progressive pathology; (3) anti‐MAG antibody was not deposited in the central nervous system of an affected individual, although the antibody did bind to these same tissues in vitro; (4) deposition of anti‐MAG antibody observed at postmortem examination did not correlate with the degree of pathological change; and (5) study of the peripheral nervous system favored a primary axonal neuropathy with secondary demyelination.
Regional cerebral blood flow decreases during hyperglycemia
Authors: Duckrow Robert B. , Beard Daniel C. , Brennan Robert W.
Published: January 2005

The presence of hyperglycemia before brain ischemia increases stroke‐related morbidity and mortality in experimantal animals and humans. However, little is known of the effect of hyperglycemia on regional cerebral blood flow (rCBF). Acute hyperglycemia was induced in awake but restrained rats by intraperitoneal injection of 50% D‐glucose. Regional flow was determined using [14C]iodoantipyrine and quantitative autoradiography. Elevation of plasma glucose from 11 to 39 mM was associated with a 24% reduction in rCBF when compared with controls that received normal saline. Intraperitoneal D‐mannitol produced an elevation of plasma osmolality equivalent to that observed with glucose. However, rCBF was only reduced by 10%. Hyperglycemia appears to produce a global decrease in rCBF in awake rats that cannot be completely explained by the attendant increase in plasma osmolality. If a similar influence is present during brain ischemia, hyperglycemia could extend areas of critical flow limitation.
Eye movements in patients with absent voluntary horizontal gaze
Authors: Baloh R. W. , Furman J. , Yee R. D.
Published: January 2005

Despite the complete absence of horizontal saccades, two patients with pontine gliomas had horizontal reflex eye movements within a range of ±20 degrees. The gain (peak eye velocity/peak stimulus velocity) and pahse of the vestibulo‐ocular reflex were normal, but the optokinetic gain was decreased. The latency, accuracy, and peak velocity of vertical saccades were normal. Apparently the voluntary gaze centers in the pontine reticular formation are not crucial for generating horizontal or vertical saccadic eye movements.
Stimulus rate determines regional brain blood flow in striate cortex
Authors: Fox Peter T. , Raichle Marcus E.
Published: January 2005

Intravenous bolus administration of oxygen 15‐labeled water and positron emission tomography were used to measure changes in brain blood flow induced by two modes of photic stimuli over a wide range of repetition rates. These stimuli (patterned‐flash and reversingd checkerboard) were chosen in order to determine whether stimulus luminacne or stimulus frequency was reponsible for previously observed increased in blood flow in the striate cortex during photic stimulation. The response curves of blood flow change as a function of stimuls rate were nearlyl suggest that elementary stimulus variables, such as repetition rate, can have a major effect on local cerebral responses, as measured with positron emission tomography and other radiotracer methods.
Decreased morbidity from acute bacterial spinal epidural abscesses using computed tomography and nonsurgical treatment in selected patients
Authors: Leys D. , Lesoin F. , Viaud C. , Pasquier F. , Rousseaux M. , Jomin M. , Petit H.
Published: January 2005

We describe 5 patients with spinal epidural abscesses in whom computed tomographic scanning confirmed diagnosis with the use myelography. One patient required urgent surgery because of rapidly deteriorating neurological status, but the other 4 were treated nonsurgically. The medical treatment of these patients and 9 others described in the literature consisted of antibiotics administered parenterally for minimum of 8 weeks, followed by oral anitibiotic therapy. Early diagnosis with computed tomographic scanning and a benign neurological state at the state at the onset of treatment were associated with good results.
The cherry‐red spot in Tay‐Sachs and other storage diseases
Authors: Kivlin Jane D. , Sanborn George E. , Myers Garth G.
Published: January 2005

The disappearance of the cherry‐red spot in an otherwize typical patient with Tay‐Sachs disease is described and illustrated. This clinical onservation has rarely ben made, but it is consistent with the pathological findings of loss of retinal ganglion cells. Disappearance of the cherry‐red spot has also been observed in GM1 generalized gangliosidosis, the sialidoses known as the cherry‐red spot‐myoclonus syndrome and the Goldberg syndrome, and Niemann‐Pick, type C disease. Thus the absence of a cherry‐red spot in the neurologically impaired child or adult does not exclude these diagnoses.