bluuebunny/crossref_metadata_2025 huggingface dataset:
Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis
Authors: Bradley Walter G. , Good Paul , Rasool Chaudri G. , Adelman Lester S.
Published: January 2005

Phrenic nerves of 11 patients with amyotrophic lateral sclerosis studied postmortem contained only 33% of the normal number of large myelinated fibers (9 controls; p < 0.001). In the phrenic nerves of these patients, there were 18% fewer large myelinated fibers in the distal segment than in the proximal segment (p < 0.025). The ratio of axonal circumference to myelin lamellae in large myelinated fibers in the distal segment was 34% greater than that in control fibers (p < 0.002). The proportion of acute axonal degeneration was the same at all levels (48.0 ± 13.7%). Sural nerves of 21 patients with amyotrophic lateral sclerosis had more acute axonal degeneration and 30% fewer myelinated fibers (p < 0.05) than controls; evidence of degeneration also extended to unmyelinated fibers. The amount of axonal transport of acetylcholinesterase in 9 sural nerves determined in vitro was reduced by 24% (p < 0.05) and the apparent transport rate was reduced by 44% (p < 0.01) compared with 4 controls. These findings show that in amyotrophic lateral sclerosis a small degree of dying‐back change and of distal axonal atrophy is superimposed on the degeneration of motor neuron cell bodies, and that the disease effects spread beyond the motor neurons.
Persistent vegetative state with high cerebral blood flow following profound hypoglycemia
Authors: Agardh C.‐D. , Rosén I. , Ryding E.
Published: January 2005

A persistent vegetative state (severe dementia) developed in a 30‐year‐old man following hypoglycemic coma. Despite the poor clinical outcome, sensory evoked response recovered between 6 and 34 months after the insult. The cerebral blood flow level at rest after 34 months was slightly above the normal range. This finding contrasts with the low cerebral blood flow regularly reported in patients who are comatose or stuporous following severe brain hypoxia‐ischemia.
Antibody responses to measles virus and canine distemper virus in multiple sclerosis
Authors: Krakowka Steven , Miele James A. , Mathes Lawrence E. , Metzler Alfred E. , Vet Med
Published: January 2005

Age‐matched serum and cerebrospinal fluid from 20 multiple sclerosis patients and 20 control patients with other neurological diseases were examined for antibodies to radiolabeled measles virus and canine distemper virus using an immunoprecipitation polyacrylamide gel technique. No evidence for reactivity to unique canine distemper virus‐virion polypeptides in the multiple sclerosis group was obtained. Competitive binding experiments with cerebrospinal fluid using labeled and unlabeled viral antigens failed to detect preferences in binding of antibodies for canine distemper virus versus measles virus. Cerebrospinal fluid samples tested for antiviral immunoglobulin M activity using both measles and canine distemper viruses showed no activity. The results of this study failed to implicate canine distemper virus directly as a cause of multiple sclerosis.
Autosomal dominant motor system degeneration in a black family
Authors: Cooper Joanna A. , Nakada Tsutomu , Knight Robert T. , Friedland Robert P.
Published: January 2005

Autosomal dominant motor system degeneration has been described primarily in Portuguese families from the Azorean Islands. The symptoms include various combinations of ataxia, pyramidal and extrapyramidal signs, appendicular dystonias, tics, ophthalmoplegias, and peripheral neuropathies with amyotrophy. One non Portuguese, non‐Azorean black family has been described previously; this report describes a second such family affected by autosomal dominant motor system degeneration.
In vivo disturbance of the oxidative metabolism of glucose in human cerebral gliomas
Authors: Rhodes Christopher G. , Wise Richard J. S. , Gibbs Jeremy M. , Frackowiak Richard S. J. , Hatazawa Jun , Palmer Anthony J. , Thomas David G. T. , Jones Terry
Published: January 2005

Abnormalities in the oxidative metabolism of glucose in human cerebral gliomas have been studied in seven patients using positron emission tomography. Measurements of regional cerebral blood flow and oxygen consumption were obtained using the oxygen‐15 steady‐state inhalation technique. Values of regional cerebral glucose consumption were obtained using fluorine 18–labeled 2‐fluoro‐2‐deoxy‐D‐glucose and a simplification of the method of Sokoloff. Functional values were obtained for regions of tumor and brain tissue in the middle cerebral artery territory of the contralateral cortex. Values of regional glucose consumption were calculated for both regions using a value of the lumped constant quoted for normal brain tissue (0.42).

Tumor regional cerebral blood flow was comparable to that in the contralateral cortex, whereas regional cerebral oxygen consumption was depressed. This depression resulted in low tumor values of the fractional oxygen extraction ratio (0.21 + 0.07), indicating that oxygen supply exceeded the metabolic demand. In contrast, tumor regional cerebral glucose consumption was not depressed and regional glucose extraction ratios were similar for tumor and brain tissue. The metabolic uncoupling between regional oxygen consumption and regional glucose consumption (CMRO2/CMRGlu = 0.24 ± 0.07 ml of oxygen per milligram of glucose) is indicative of increased aerobic glycolysis.
Disturbance of oxidative metabolism of glucose in recent human cerebral infarcts
Authors: Wise Richard J. S. , Rhodes Christopher G. , Gibbs Jeremy M. , Hatazawa Jun , Palmer Tony , Frackowiak Richard S. J. , Jones Terry
Published: January 2005

Eight patients with recent cerebral hemispheric infarction were studied with positron emission tomography and the oxygen‐15 steady‐state inhalation and [18F]deoxyglucose techniques to obtain values of regional cerebral blood flow, oxygen consumption, and glucose metabolism. The Sokoloff equation, used to calculate glucose metabolism, was simplified to exclude the exponential terms containing the rate constants. A value of the lumped constant quoted for normal brain (0.42) was used for infarcted regions and contralateral hemisphere. Mean regional cerebral blood flow, oxygen consumption, and glucose metabolism were all significantly depressed within the infarcts compared with the mirror regions in the contralateral cerebral hemisphere. The mean fractional extraction of oxygen was low, indicating an adequate supply of oxygen for residual oxidative metabolism. Regional oxygen consumption and glucose metabolism were significantly correlated within the infarcts, but with a relationship of 2 moles of oxygen per mole of glucose—one‐third that in the contralateral hemisphere and in normal brain. Although these results suggest that the metabolizing tissue of a recent cerebral infarct utilizes aerobic glycolysis, caution about the validity of this pathophysiological observation is dictated by limitations in current positron emission tomographic tracer methodology.
Hemiparesis apparently due to central pontine myelinolysis following hyponatremia
Authors: Marra Thomas R.
Published: January 2005

A 63‐year‐old woman was in an acute confusional state accompanied by rapidly progressive left hemiparesis, dysphagia, and dysarthria two weeks after an episode of diuretic‐induced hyponatremia. High‐resolution computed tomographic scanning disclosed a circumferential hypodense pontine lesion compatible with the diagnosis of central pontine myelinolysis. Although the patient improved clinically within six weeks, the computed tomographic abnormalities remained unchanged.
High blood glucose level on hospital admission and poor neurological recovery after cardiac arrest
Authors: Longstreth W. T. , Inui Thomas S.
Published: January 2005

To evaluate the relationship between blood glucose and neurological recovery after cardiac arrest, we retrospectively reviewed our experience with 430 consecutive patients resuscitated after out‐of‐hospital cardiac arrest. All these patients had received variable amounts of intravenous 5% glucose solutions before admission. Awakening patients, whose following commands or having comprehensible speech, were classified as to whether they suffered persistent neurological deficits. The mean blood glucose level on hospital admission was higher in 154 patients who never awakened than in 276 who did awaken (mean, 341 versus 262 mg per 100 milliliters; p <0.0005). Among the 276 who awakened, 90 patients with persistent neurological deficits had higher mean glucose levels on admission than did 186 without deficits (286 versus 251 mg per 100 milliliters; p < 0.02). These significant differences persisted after excluding all patients whose glucose levels were higher than 500 mg per 100 milliliters and after controlling for potentially confounding variables using multiple regression analysis.
Dysautonomia in Guillain‐Barré syndrome with dorsal root ganglioneuropathy, wallerian degeneration, and fatal myocarditis
Authors: Hodson Andrew K. , Hurwitz Barrie J. , Albrecht Renata
Published: January 2005

An acute Guillain‐Barré syndrome presenting as dysautonomia is described in a 12‐year‐old boy. The patient died of intractable cardiac arrhythmias and cardiac failure. A severe myocarditis with destruction of dorsal root ganglion cells and wallerian degeneration of dorsal roots and peripheral nerves was apparent postmortem. Segmental demyelination and inflammatory cellular infiltrations were not present at these sites.
Response of C‐6 glioma to isoproterenol and papaverine in vivo depends on β‐adrenergic receptor density
Authors: Chelmicka‐Schorr Ewa , Sportiello Michael G. , Atweh Samir F. , Arnason Barry G. W.
Published: January 2005

Rat C‐6 glioma serves as an experimental model for human glioma. C‐6 glioma cells carried to high culture passages in medium containing 10% fetal bovine serum when injected in vivo are unresponsive to treatment with the β‐adrenergic agonist isoproterenol and the phosphodiesterase inhibitor papaverine. When C‐6 glioma cells are kept in culture in serum‐containing medium, β‐adrenergic receptor density falls and, concomitantly, ability to accumulate cyclic adenosine monophosphate in response to stimulation with catecholamines declines. Responsiveness to treatment in vivo with a β‐adrenergic agonist was restored when C‐6 glioma cells were cultured in serum‐free defined medium prior to systemic injection into rats. Culturing of C‐6 glioma cells in serum‐free medium significantly increases the number of β‐ adrenergic receptors when compared with C‐6 glioma cells grown in serum‐containing medium.
Brain trace elements in Pick's disease
Authors: Ehmann W. D. , Alauddin M. , Hossain T. I. M. , Markesbery W. R.
Published: January 2005

The trace element content of the brain of two patients with Pick's disease examined postmortem was studied using instrumental neutron activation analysis. Results showed significant increases in chlorine, iron, manganese, sodium, and phosphorus and significant decreases in chromium, cesium, rubidium, and selenium and in the mean freeze‐dried to wet‐weight ratio for patients with Pick's disease compared with control patients. Brain zinc content was not elevated in the two patients, a finding that fails to support the hypothesis that elevated zinc levels play a role in the pathogenesis of Pick's disease.
Creutzfeldt‐Jakob disease: A case of 16 years' duration
Authors: Cutler Neal R. , Brown Paul W. , Narayan Tina , Parisi Joseph E. , Janotta Frank , Baron Henry
Published: January 2005

A 46‐year‐old man with Creutzfeldt‐Jakob disease confirmed postmortem had a 16‐year course of very slowly progressing incoordination and mental deterioration, suggesting Alzheimer's disease. The disease course transformed abruptly into a 7‐week terminal phase of florid Creutzfeldt‐Jakob disease. Dementing illnesses of unknown cause were present in the patient's paternal lineage.
Central nervous system catecholamine metabolism in Korsakoff's psychosis
Authors: Martin Peter R. , Weingartner Herbert , Gordon Edna K. , Burns R. Stanley , Linnoila Markku , Kopin Irwin J. , Ebert Michael H.
Published: January 2005

We attempted to replicate previous reports of impaired central noradrenergic activity in patients with Korsakoff's psychosis. No differences were demonstrated in lumbar cerebrospinal fluid concentrations of norepinephrine or its major metabolite, 3‐methoxy‐4‐hydroxypheny glycol (MHPG), or in the contribution of plasma to cerebrospinal fluid free MHPG in 6 patients with clinically well characterized Korsakoff's psychosis and 8 age‐matched healthy, normal volunteers. Likewise, cerebrospinal fluid concentrations of the major metabolites of dopamine (homovanillic acid) and serotonin (5‐hydroxyindoleacetic acid) were the same in patients and controls.
Pulmonary lymphatic flow alterations during intracranial hypertension in sheep
Authors: Simon R. P. , Bayne L. L.
Published: January 2005

The role of intracranial hypertension in the genesis of neurogenic pulmonary edema was studied in 25 sheep; cardiopulmonary hemodynamics (aortic, pulmonary arterial, and left atrial pressures and cardiac output) and fluid and protein movement across the pulmonary capillary bed (efferent pulmonary lymph flow and lymph/plasma protein ratio) were monitored. Only when intracranial pressure was raised to equal the baseline mean systemic pressure (75 to 120 Torr) did we observe the expected Cushing response of increased aortic pressure, or any alteration in pulmonary hemodynamics or fluid movement. When pulmonary changes did occur, they included an increase in pulmonary arterial pressure of between 5 and 15 Torr without any notable rise in left atrial pressure, and a sustained doubling of the pulmonary lymph flow with no dilution of the lymph/plasma protein ratio. In 3 additional animals cerebral ischemia alone produced an elevation in systemic pressure (74 Torr over baseline) without change in pulmonary arterial pressure, left atrial pressure, or pulmonary lymph flow. Thus, intracranial hypertension and ischemia both affect systemic pressure, but only the elevated intracranial pressure is followed by changes in the pulmonary circuit. We suggest that these changes in pulmonary vascular pressure, independent of changes in left atrial pressure, produce increased pulmonary transcapillary fluid flux that may result in neurogenic pulmonary edema.
A quantitative model for the in vivo assessment of drug binding sites with positron emission tomography
Authors: Mintun Mark A. , Raichle Marcus E. , Kilbourn Michael R. , Wooten G. Frederick , Welch Michael J.
Published: January 2005

We propose an in vivo method for use with positron emission tomography (PET) that results in a quantitative characterization of neuroleptic binding sites using radiolabeled spiperone. The data are analyzed using a mathematical model that describes transport, nonspecific binding, in the brain. The model demonstrates that the receptor quantities Bmax (i.e., the number of binding sites) and KD−1 (i.e., the binding affinity) are not separably ascertainable with tracer methodology in human subjects. We have, therefore, introduced a new term, the binding potential, equivalent to the product BmaxKD−1, which reflects the capacity of a given tissue, or region of a tissue, for ligand‐binding site interaction. The procedure for obtaining these measurements is illustrated with data from sequential PET scans of baboons after intravenous injection of carrier‐added [18F]spiperone. From these data we estimate the brain tissue nonspecific binding of spiperone to be in the range of 94.2 to 95.3%, and the regional brain spiperone permeability (measured as the permeability–surface area product) to be in the range of 0.025 to 0.036 cm3/(s·ml). The binding potential of the striatum ranged from 17.4 to 21.6; these in vivo estimates compare favourably to in vitro values in the literature. To our knowledge this represents the first direct evidence that PET can be used to characterize quantitatively, locally and in vivo, drug binding sites in brain. The ability to make such measurements with PET should permit the detailed investigation of diseases thought to result from disorders of receptor function.
Elevated neutral protease activity in myelin from brains of patients with multiple sclerosis
Authors: Sato Shuzo , Quarles Richard H. , Brady Roscoe O. , Tourtellotte Wallace W.
Published: January 2005

Incubation of human myelin at neutral PH resulted in the proteolytic conversion of the myelin‐associated glycoprotein to a lower molecular weight derivative (dMAG) and the degradation of basic protein. The formation of dMAG occured much more rapidly than the degradation of basic protein. The formation of dMAG and the degradation of basic protein both occured significantly more rapidly in myelin preparations purified from brains of patients with multiple sclerosis than in preparations from control brain. The results suggest that this neutral protease associated with myelin may function in the pathogenesis of demyelinating diseases such as multiple sclerosis.
Long‐term corticosteriod treatment of myasthenia gravis: Report of 116 Patients
Authors: Pascuzzi Robert M. , Coslett H. Branch , Johns T. R.
Published: January 2005

One hundred sixteen patients, aged 8 to 82 years, with myasthenia gravis were treated with prednisone, 6o to 80 mg daily, until the onset of improvement, followed by lower‐dose alternate‐day therapy of several years' duration. Of all patients, 80.2%achived wither remission(27.6%) or marked improvement (52.6%). Moderate improvement occurred in 14.7%, and 5.2% showed no improvement. Increasing age correlated with a favorable outcome, but sex, duration of illness prior to treatment, severity and distribution of weaskness at the time of onset of treatment, and presence of thymoma were not factors in the response to therapy.
Chromatin structure in dementia
Authors: McLachlan D. R. Crapper , Lewis P. N. , Lukiw W. J. , Sima A. , Bergeron C. , Boni U.
Published: January 2005

Nuclei extracted from neocortex of patients with Alzheimer's disease and treated with micrococcal nuclease release a population of dinucleosomes that contain an increase in the linker histones H1° and H1ºº. Five other degenerative brain diseases that clinically resemble Alzheimer's disease do not result in these changes, although Pick's disease is associated with an increase in H1 on dinucleosomes. Histones from nuclei of patients with Alzheimer's disease are also more resistant to salt‐induced release from chromatin than are those from age‐matched control subjects. These results support the hypothesis that an alteration in chromatin structure is a marker for Alzheimer's disease.
Effects of hypoxia‐ischemia on monoamine metabolism in the immature brain
Authors: Silverstein Faye , Johnston Michael V.
Published: January 2005

We measured acute changes in monoamine metabolites in corpus striatum of immature rat pups exposed to hypoxiaischemia, hypoxia alone, or total global ischemia. Carotid ligations and two hours of 8% oxygen environment in 7‐day‐old pups led to asymmetrical turning behavior, a 70% decrease in endogenous striatal dopamine levels, and a 125% increase in homovanillic acid (HVA) concentrations on the side of ligation. In contrast, hypoxia alone and total global ischemia alone were not associated with HVA level elevation. Elevation of HVA level with hypoxia‐ischemia showed a threshold effect between 1 and 1.5 hours, and this time course paralleled that for production of gross morphological changes in rats raised to maturity. The data suggest that dopamine release from striatal nerve terminals is associated with events causing brain injury during perinatal hypoxia‐ischemia. Tissue HVA in the animal model appears to be a quantitative marker for the effects of the insult on a population of nerve terminals.
Cerebrospinal fluid vasopressin and increased intracranial pressure
Authors: Sørensen Per Soelberg , Gjerris Flemming , Hammer Mogens
Published: January 2005

Cerebrospinal fluid and plasma vasopressin were measured in patients with cerebral disorders associated with varying levels of elevated intracranial pressure. The mean cerebrospinal fluid vasopressin concentration was significantly increased in patients with pseudotumor cerebri (2.0 ± 0.2 [SEM] pg/ml), intracranial tumor (2.3 ± 0.4 pg/ml), and intracranial hemorrhage (1.9 ± 0.3 pg/ml) compared with control patients (1.2 ± 0.1 pg/ml). A significant relationship was found between intracranial pressure and the cerebrospinal fluid vasopressin concentration within all groups of patients and in the whole sample as well (r = 0.79; < 0.001). In the groups of patients with intracranial tumor, hydrocephalus, and intracranial hemorrhage, some individuals showed plasma vasopressin concentratioons inappropriate to the corresponding plasma osmolality, but no relationship was found between intracranial pressure and plasma vasopressin concentration. It is suggested that increased intracranial pressure is a stimulus to centrally released vaso‐Pressin. The clinical importrance of increased cerebrospinal fluid vasopressin concentrations is still not known.
The functional anatomy of frontal lobe neglect in the monkey: Behavioral and quantitative 2‐deoxyglucose studies
Authors: Deuel Ruthmary K. , Collins Robert C.
Published: January 2005

A syndrome of hemisensory neglect follows damage to frontal association areas in monkeys and humans. To study the syndrome we removed frontal association cortex from the right hemisphere of macaque monkeys. After operation, behavioral testing showed conditional deficits in visual, somatosensory, and motor responses contralateral to the lesion. Brains of animals were studied with [14C]2‐deoxyglucose autoradiography to evaluate functional neuroanatomical changes. Ipsilateral striatal and selected thalamic and midbrain nuclei exhibited depression of glucose utilization (10 to 60%). No consistent glucose utilization changes appeared in cortex or in primary motor or sensory pathways. Brains from unoperated control animals did not exhibit these changes, nor did brains from operated animals with behavioral recovery from neglect. We conclude that the symptoms of frontal lobe neglect in the monkey are the result of dysfunction within a widely distributed system of subcortical centers. The distribution of the dysfunction provides an explanation for some of the clinical features of neglect.
Ultrastructural, neurological, and glycosaminoglycan abnormalities in Lowe's syndrome
Authors: Wisniewski Krystyna E. , Kieras Fred J. , French Joseph H. , Houck George E. , Ramos Peter L.
Published: January 2005

The oculocerebrorenal syndrome (OCRS), Lowe's syndrome, is an X‐linked, recessive disease characterized by mental retardation, congenital corneal abnormalities and cataracts, growth failure, rickets, osseous abnormalities, renal dysfunction with periodic acidosis, hypotonia, and areflexia. Ultrastructural studies of skin biopsy specimens in three individuals with the disorder (aged 17, 9, and 8 years) revealed cytoplasmic, membrane‐bound, electron‐lucent vacuoles and some electron‐dense membranous inclusion bodies in fibroblasts and Schwann cells, as well as axonal degeneration and vascular changes. Computed tomographic scans evidenced brain atrophy. Urinary excretion of glycosaminoglycans (GAG) was four to five times greater than in normal controls. The predominant urinary GAG was a low‐sulfated chondroitin‐4‐sulfate; chondroitin‐6‐sulfate and heparan sulfate excretion levels were normal. A tenfold increase in urinary GAG excretion was found in one patient with oculocerebrorenal syndrome during periods of behavioral agitation. These findings suggest that the clinical stigmata of oculocerebrorenal syndrome may be related to a defect in GAG metabolism.
Startle epilepsy: A clinical study
Authors: Sàenz‐Lope E. , Herranz F. J. , Masdeu J. C.
Published: January 2005

Twelve patients with perinatal anoxic encephalopathy, 1 with encephalitis, and 1 with Down's syndrome had startle epilepsy. Hemiparesis, startle‐induced seizures involving the hemiparetic side, focal electroencephalographic abnormalities, and unilateral lesions evident on computed tomographic scan were found in 6 patients, all of whom responded favorably to carbamazepine. The remaining 8 patients had severe intellectual impairment, bilateral motor deficits, generalized startle‐induced seizures, diffuse and lasting electroencephalographic abnormalities, and widespread cerebral lesions evident on computed tomographic scan. Of these, 2 patients with Lennox‐Gastaut syndrome responded to clonazepam, 4 others responded to valproic acid, and 2 others, with predominantly focal hemispheric lesions, improved on a regimen of carbamazepine.
Low T8 antigen density on lymphocytes in active multiple sclerosis
Authors: Reder Anthony T. , Antel Jack P. , Oger Joël J.‐F. , McFarland Thomas A. , Rosenkoetter Maureen , Arnason Barry G. W.
Published: January 2005

The median flurescence intensity of peripheral blood T8+ (T‐suppressor/cytotoxic) cells in patients with active (relapsing or progressive) or stable multiple sclerosis (MS) and in control subjects was determined with a fluorescence‐activated cell sorter using an indirect immunofluorescence assay. Median fluorescence intensity was singnificantly lower in patients with active MS than in controls. No differences in median fluorescence intensity were found between patients with stable MS and controls. Analysis of T8+ cells that had been modulated in vitro with OKT8 indicated that the resultant reduction in the median T8 antigen density per cell resulted in fewer T8+ cells being enumerated when the fluorescence‐activated cell sorter was used than when a fluorescence microscope was used. Reduced median T8 antigen density on cells as found in active MS may contribute to the apparent underrepresentation of circulating T‐suppressor cells in such patients and may be associated with reduced suppressor cell function.
Creutzfeldt‐Jakob disease of long duration: Clinicopathological characteristics, transmissibility, and differential diagnosis
Authors: Brown Paul , Rodgers‐Johnson Pamela , Cathala Françoise , Gibbs Clarence J. , Gajdusek D. Carleton
Published: January 2005

Five to 10% of patients with Creutzfeldt‐Jakob disease have a clinical course that extends for 2 years or more. In the present series 33 (9%) of 357 histopathologically verified cases, including 15 (7%) of 225 transmitted cases, fell into this long‐duration category, the longest transmitted case having had a 13‐year duration. As a group these cases were characterized by a higher familial representation (30%), a younger age at onset (average, 48 years), and lower frequencies of myoclonus (79%) and periodic electroencephalographic activity (45%) than occur in series of unselected cases. The clinical course most often showed a long, very slowly progressive terminal stage with both mental and physical deterioration. Although transmission of disease by primate inoculation periods and durations of illness in the long‐duration cases (64%) than in cases of shorter duration (88%), incubation periods and duration of illness in the inoculated animals bore no relation to the duration of illness in the patients. Clinical differentiation from other chronic dementing processes, particularly Alzheimer's disease, can prove impossible; however, the correct pathologica diagnosis was readily evident by light microscopic examination, and spongiosis was at least as prominent as gliosis in all but 4(15%) of the 27 patients studied postmortem, the same frequency found in case series not selected for length of illness.
Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A distinctive clinical syndrome
Authors: Pavlakis Steven G. , Phillips Peter C. , DiMauro Salvatore , De Vivo Darryl C. , Rowland Lewis P.
Published: January 2005

We report on two patients who have a mitochondrial myopathy, encephalopathy, lactic acidosis, and recurrent cerebral insults that resemble strokes (MELAS). These two and nine other reported patients share the following features: ragged red fibers evident on muscle biopsy, normal early development, short stature, seizures, and hemiparesis, hemianopia, or cortical blindness. Lactic acidemia is a common finding. We believe that MELAS represents a distinctive syndrome and that it can be differentiated from two other clinical disorders that also are associated with mitochondrial myopathy and cerebral disease: Kearns‐Sayre syndrome and the myoclonus epilepsy ragged red fiber syndrome. Existing information suggests that MELAS is transmitted by maternal inheritance. The ragged red fibers suggest an abnormality of the electron transport system, but the precise biochemical disorders in these three clinical syndromes remain to be elucidated.
Peripheral beta‐adrenergic blockade treatment of parkinsonian tremor
Authors: Foster Norman L. , Newman Richard P. , Lewitt Peter A. , Gillespie Marjorie M. , Larsen T. Andreo , Chase Thomas N.
Published: January 2005

The effect of nadolol, a peripherally acting beta‐adrenergic blocker, on resting, postural, and intention tremor was examined in 8 patients with idiopathic Parkinson's disease whose motor symptoms, other than tremor, were well controlled with conventional medications. In a double‐blind, placebo‐controlled, crossover design, patients received 80 to 320 mg of nadolol for six weeks while continuing their previous therapeutic regimen. Accelerometer readings showed a 34% reduction (p < 0.025) in tremor distance, but no change in tremor frequency, during nadolol therapy. Maximum benefit was achieved with a dose of 240 mg, when resting tremor improved 54%, postural tremor 32%, and intention tremor 54%. Physician ratings and patient reports supported the accelerometer results. Nadolol appears to be a safe, effective adjunct to current dopaminergic and anticholinergic therapy for severe tremor in Parkinson's disease.
Diagnostic importance of immunological markers in lymphoma involving the central nervous system
Authors: Ezrin‐Waters Cheryl , Klein Michel , Deck John , Lang Anthony E.
Published: January 2005

In an elderly woman, lymphoma involved the central nervous system and unusual nonlymphoreticular sites. Cerebrospinal fluid cell surface marker studies revealed a clone of malignant B cells. Studies of peripheral blood demonstrated a systemic disturbance of immune regulation similar to Epstein‐Barr virus infections and the acquired immunodeficiency syndrome.
Alexander's law: Its behavior and origin in the human vestibulo‐ocular reflex
Authors: Robinson David A. , Zee David S. , Hain Timothy C. , Holmes Allistair , Rosenberg Lisa F.
Published: January 2005

Alexander's law refers to the phenomenon in which the spontaneous nystagmus of a patient with a vestibular lesion is more intense when the patient looks in the quick‐phase than in the slow‐phase direction. Alexander's law was investigated in normal subjects as well as patients with vestibular lesions. During sinusoidal rotations of normal subjects, there was no trace of this phenomenon when subjects looked 25 degrees left and right in the dark. The phenomenon of Alexander's law is therefore created centrally and is not due to any mechanical properties of the orbit.

During rotation at constant velocity in the dark, normal subjects did weakly demonstrate Alexander's law because of a mild gaze‐evoked nystagmus present in normal subjects in the dark. Gaze‐evoked nystagmus from a cerebellar lesion involved a pronounced demonstration of Alexander's law during rotatory nystagmus. In patients with a vestibular lesion and a mild spontaneous nystagmus in the dark that obeyed Alexander's law, the nystagmus reversed upon far gaze in the slow‐phase direction. We propose that in patients with a vestibular lesion, the phenomenon of Alexander's law is created by the sum of vestibular nystagmus and an abnormally large gaze‐evoked nystagmus that is consequent to the vestibular lesion. This conclusion has a number of neurological implications concerning the ways in which the nervous system attempts to compensate for vestibular lesions.
Autosomal recessive distal muscular dystrophy: A comparative study with distal myopathy with rimmed vacoule formation
Authors: Nonaka Ikuya , Sunohara Nobuhiko , Satoyoshi Eijiro , Teresawa Kenjiro , Yonemoto Kyozo
Published: January 2005

To clarify the clinical and morphological characteristics of distal muscular dystrophy, clinical and pathological material from 4 affected persons was compared with similar studies in 4 patients with distal myopathy with rimmed vacuole formation. Although these two forms of autosomal recessive distal myopathy with onset in young adulthood were highly similar in their clinical symptoms, histochemical and electron microscopic findings of muscles subjected to biopsy were quite different. The muscle abnormalities in distal muscular dystrophy were almost the same as those in Duchenne muscular dystrophy, showing massive fiber necrosis followed by active fiber regeneration. In contrast, distal myopathy with rimmed vacuole formation showed a progressive muscle fiber atrophy and loss, rimmed vacuoles in the sarcoplasm, and no apparent fiber necrosis or regeneration.
Crystalline encephalopathy: Cerebral immunoprotein deposits and isolated angiitis
Authors: Pezeshkpour G. , Stuart Thomas D. , Estridge M. N.
Published: January 2005

Brain biopsy specimens from a 38‐year‐old woman with adversive seizures and bifrontal mass lesions evident on computed tomographic scans showed extracellular and intracellular deposits of crystallized protiens. These were morphologically identical to the immunoglobylin crystals seen in reactive or neoplastic plasma cells and by peroxidase‐antiperoxidase methods were found to contrain polyclonal immunoglobulins. In addition, severe angiitis of the intracerebral blood vessels was present.
Leptomeningeal metastases: Reduction in regional cerebral blood flow and cognitive impairment
Authors: Siegal Tali , Mildworf Bracha , Stein Daniel , Melamed Eldad
Published: January 2005

Twenty‐six patients with recently diagnosed leptomingeal metastases underwent multiple diagnostic procedures in order to assess possible cerebral parenchymal involvement. Pretreatment studies included detailed neurological examination, computed tomographic scan, electroencephalgram, neuropsychological assessement, psychiatric evaluation, and measurement of regional cerebral blood flow (rCBF) using the xenon 133 inhalation technique. At diagnosis 50% of the patients had symptoms and signs of cerebral dysfunction. Computed tomographic scanning and electroencephalography detected abnormalities in 42% and 58%, respectively. Scoring was abnormally low on at least three neuropsychological tests in 93% of the patients. The mean rCBF was reduced in 88% of patients by 16.1 ± 2.5% (mean ± SEM; range, 8 to 45%) compared with age‐matched controls (p < 0.001).rCBF was reduced and cognition was impaired in patients with or without overt cerebral manifestations. Neuropsychological testing and rCBF measurement may be helpful in early detection of cerebral dysfunction in patients with leptomeningeal metastases.
Intracranial pressure, conductance to cerebrospinal fluid outflow, and cerebral blood flow in patients with benign intracranial hypertension (pseudotumor cerebri)
Authors: Gjerris F. , Sørensen P. Soelberg , Vorstrup S. , Paulson O. B.
Published: January 2005

Intracranial pressure, conductance to cerebrospinal fluid outflow, and cerebral blood flow were investigated in 14 patients with benign intracranial hypertension (pseudotumor cerebri). Intracranial pressure was increased in 9 patients (20 to 30 mm Hg), borderline in 4 patients (15 to 18 mm Hg), and normal in 1 patient (8 mm Hg). Six patients had plateau waves, and all had B waves in more than 50% of the monitored time. Conductance to cerebrospinal fluid outflow, measured by a lumbo‐lumbar perfusion method, was significantly reduced: 0.042 ml·mm Hg−1·min−1 (± 0.004 {SEM}; normal more than 0.080 ml·mm Hg−1·min−1). Cerebral blood flow was measured by xenon 133 inhalation and single photon emission computer tomography. Mean hemispheric flow was normal in all cases, averaging 59 ± 9 ml·100 gm−1·min−1. Only 2 patients showed focal low‐flow areas. Thus, a disturbance of cerebrospinal fluid circulation seems to be of pathogenetic significance in benign intracranial hypertension.
The role of plasma lipids in carotid bifurcation atherosclerosis
Authors: Ford C. Stephen , Crouse John R. , Howard George , Toole James F. , Ball Marshall R. , Frye Janice
Published: January 2005

The severity of coronary artery atherosclerosis correlates with increased plasma concentrations of total cholesterol (TC), low‐density lipoprotein cholesterol (LDL‐C), and triglycerides and with decreased plasma concentrations of high‐density lipoprotein cholestrol (HDL‐C). The role of plasma lipoproteins in the pathogenesis of cerebral atherosclerosis, however, is less clear. Several investigators report that lipoprotein abnormlities correlate inversely with the incidence of cerebral infarction. We analyzed risk factors for carotid bifurcation atheroscerosis in 121 consecutive patients who underwent cerebral angiography. This analysis revealed a significant positive correlation between the plasma TC/HDL‐C ratio and the extent of carotid bifurcation atherosclerosis (p = 0.01). The extent of atherosclerosis correlated inversely with plasma concentrations of HDL‐C (p = 0.02). Triglyceride concentration correlated positively with the extent of atherosclerosis with marginal significance (p = 0.07). LDL‐C and TC concentrations did not correlate with bifurcation atherosclerosis (p > 0.1). Only 4% of the variation in the extent of bifurcation atherosclerosis was explicable on the basis of plasma lipoprotien concentrations.
Mesulergine (CU32‐085) in the treatment of Parkinson's disease
Authors: Pfeifer Ronald F. , Wilken Kathleen , Glaeske Carolyn
Published: January 2005

Twenty patients with Parkinson's disease were treated with the 8‐α‐ergoline derivative mesulergine. Participants were divided into two groups, and over a nine‐week period, mesulergine dosage was increased to amaximum of either 10 or 20 mg daily. During this time levodopa‐carbidopa (LD‐CD) dosage was reduced and treatement was discontinued if possible. The dosage of LD‐CD was reduced 75% in the 10 mg group and 74% in the 20 mg group. Patients in the low dose group maintained in each of the cardinal signs of parkinsonism. Adverse effects were generally mild and infrequent. Many adverse effects induced by LD‐CD diminished or resolved. Our results suggest that mesulergine can be valuable in the management of Parkinson's disease, particularly in those individuals experiencing dose‐limiting adverse effects from LD‐CD.
Central neurogenic hyperventilation in a conscious patient with primary cerebral lymphoma
Authors: Bateman D. E. , Gibson G. J. , Hudgson P. , Tomlison B. E.
Published: January 2005

The clinical and neuropathological findings of a patient with central neurogenic hyperventilation are described. The patient had extensive primary central nervous system lymphoma but no lesions below the level of the superior colliculus. The possible mechanism of central neurogenic hyperventilation is discussed with reference to the present case and those previously described.
Early‐onset Alzheimer's disease: An analysis of CT findings
Authors: Drayer Burton P. , Heyman Albert , Wilkinson William , Barrett Lynn , Weinberg Tina
Published: January 2005

Brain computed tomographic scan of 60 patients with early‐onset Alzheimer's disease (mean age, 60.7 years) were compared awith those of age‐ and sex‐matched control subjects. Computed tomographic analysis included standard ventricular measurements as well as subjectice ratings of ventriculat sulcal size. These indiceswere correlated with the results of a battery of neuropsychological tests and electroencephalographic findings.

Linear measurements of ventricular size were significantly greater in the patients with Alzheimer's disease than in the age‐matched control group (p<0.0005). Using subjective appraisal of ventricular and sulcal size, the neuroradiologist noted anmormalities significantly more often in patients than in controls (p<0.0005). Linear measurements of ventricular size correlated significantly (p<0.05) with the severity of aphasia and dementia and the presence of electroemcephalographic abnormalities. There was, however, no correlation between the subjective judgement of cortical atrophy and degree impairment as measured byneuropsychological tests. The fidings in this study demonstrate the usefulness of computed tomographic imaging Alzheimer's disease of early onset.
Late‐onset Wilson's disease with neurological involvement in the absence of Kayser‐Fleischer rings
Authors: Ross M. Elizabeth , Jacobson Ira M. , Dienstag Jules L. , Martin Joseph B.
Published: January 2005

We report a patient with Wilson's disease whose case is unusual in two respects. First, the patient was 58 years of age when the diagnosis was made based on urinary, serum, and hepatic copper studies, as well as liver histological investigation. Second, despite the patient's neurological impairment, Kyser‐Fleischer rings had not developed. We conclude that in patients with chronic, degenerative neurological disease, particularly when associated with hepatic dysfunction, the diagnosis of Wilson's disease should be considered regardless of age at onset or the absence of Kyser‐Fleischer rings on slit lamp examination.
Facioscapulohumeral dystrophy presenting in infancy with facial diplegia and sensorineural deafness
Authors: Korf Bruce R. , Bresnan Michael J , Shapiro Frederic , Sotrel Anna , Abroms Isreal F.
Published: January 2005

Six patients are described in whom facial diplegia occured in the first year of life, with subsequent development of facioscapulohumeral dystrophy. All had severe proggressive disability prior to adolescence. Facial involvement did not include extraucular muscles. All six patients had a sensorineural hearing loss. Evidence of a mildly affected parent was found in three families. Progressive and severe facioscapulohumeral dystrophy accompanied by facial diplegia and sensorineural hearing loss may represent a separate genetic form of facioscapulohumeral dystrophy.
The influence of preexiting oscillations on the binoculars optokinetic response
Authors: Abadi R. V. , Dickinson C. M.
Published: January 2005

Full‐field optokinetic were examined in patients with congenital nystagmus. If the stimulus moved in the same plane as the congenital nystagmus, a gaze‐modulated response was recorded for both idiopathic and albino observers. When the congenital nystagmus was almost totally suppressed, neither a gaze‐evoked nor a sawtooth response was see. For stimulus movements in a plane orthogonal to the congenital nystagmus, normal sawtooth optokinetic responses were exhinited by both groups. Experimental evidence supports the hypothesis that the optokinetic response in the meridian of the spontaneous oscillation has been adapted.
Balo's concentric sclerosis: New observations on lesion development
Authors: Moore G. R. Wayne , Neumann Paul E. , Suzuki Kinuko , Lijtmaer Hugo N. , Traugott Ute , Raine Cedric S.
Published: January 2005

A 54‐year‐old woman with a four‐month history of progressive neurological illness was found at postmortem examination to have lesions of Balo's concentric sclerosis. Balo lesions were found in several areas scattered widely throughout the central nervous system, including the spinal cord, a previously unreported location, and were studied histological and ultrastructural methods. Balo lesions consisted bands of intact myelin alternating with zones of demyelination. These lesions were centered on a perivascular cuff of inflammatory cells. The center of the lesion was the oldest area with the concentric rings of demyelination decreasing in age with increasing distance from the center. The bands of intact myelin comprised mainly remyelinated fibers, were similiar to those seen at the edges of chronic active multiple sclerosis plaques, and may have represented the repaired margins of preceding episodes. The occurrence of small foci of acute demyelination centered on perivascular cuffs and other changes typical of both acute and chronic active multiple sclerosis may indicate that the lesion of Balo's concentric sclerosis represents an intermediate stage in the development of an established multiple sclerosis lesion.
Decreased sympathetic neuronal uptake in idiopathic orthostatic hypotension
Authors: Polinsky Ronald J. , Goldstein David S. , Brown Robert T. , Keiser Harry R. , Kopin Irwin J.
Published: January 2005

The disappearance rates from plasma of intravenously administered levo‐norepinephrine (1‐NE), dextro‐norepinephrine (d‐NE), and isoproterenol (ISO) were measured in normal subjects and in patients with either multiple‐system atrophy (MSA) or idiopathic orthostatic hypotension (IOH). The two isomers, 1‐NE and d‐NE, were removed at similar rates in all groups. In normal subjects, the d and 1 isomers of norepinephrine were cleared more rapidly than ISO. In patients with IOH, the initial rates of disappearance of the NE isomers from plasma were slower than normal and similar to the rate for ISO disappearance. Plasma NE levels, NE clearance, and the apparent release rate of NE into plasma from sympathetic neurons were significantly lower in patients with IOH than in normal subjects. Only the apparent NE secretion rate was related to the baseline plasma NE level. Sympathetic neuronal dysfunction in IOH is attended by a reduction in the clearance of NE. The very low plasma NE levels, in association with the striking reduction in NE clearance, suggest that in IOH there is a marked decrease in NE release. NE clearance and apparent NE secretion rate are normal in MSA, consistent with a central nervous system dysfunction in regulating the sympathetic nervous system. Neuronal uptake of NE in humans does not appear to be stereoselective.
Hip flexion–abduction to elicit asterixis in unresponsive patients
Authors: Noda Shosaku , Ito Hiroaki , Umezaki Hirotoshi , Minato Seiichiro
Published: January 2005

Asterixis at the hip joints can be elicited by a simple diagnostic maneuver in which the hips are passively flexed and abducted about 60 to 90 degrees between the thighs. The test evoked asterixis in 8 of 10 patients who were stuporous or semicomatose because of hepatic encephalopathy. The asterixis seems to be provoked by the involuntary contraction of hip adductors against gravity.
The clinical spectrum of necrotizing angiopathy of the peripheral nervous system
Authors: Kissel John T. , Slivka Andrew P. , Warmolts John R. , Mendell Jerry R.
Published: January 2005

The peripheral neuropathy seen with necrotizing angiopathy is said to begin classically as a mononeuritis multiplex, usually associated with polyarteritis nodosa, rheumatoid arthritis, or systemic lupus erythematosus. Our experience, however, suggests that a large number of these patients do not have a well‐defined collagen vascular disease or the typical clinical pattern. In 350 consecutive nerve biopsies (sural or superficial radial), 16 patients showed a necrotizing angiopathy in the epineurial blood vessels. Six of these 16 patients had a distal symmetrical sensorimotor poly‐neuropathy. The remaining 10 had a mononeuritis multiplex, although in 8 overlapping nerve involvement somewhat obscured the picture of mononeuritis. In 12 patients, no specific underlying collagen vascular disease could be diagnosed by accepted criteria despite extensive clinical, radiological, and serological evaluations. The peripheral neuropathy was the only objective evidence of vasculitis in 7 of these 12 patients. Our findings suggest that patients with a peripheral neuropathy secondary to necrotizing angiopathy often do not have a definable collagen vascular disease. In fact, peripheral neuropathy may be the sole manifestation of vasculitis. Furthermore, the neuropathy was found to be a distal symmetrical sensorimotor neuropathy in a higher proportion of cases than has been documented previously.