bluuebunny/crossref_metadata_2025 huggingface dataset:
Pergolide in late‐stage Parkinson disease
Authors: Lang A. E. , Quinn N. , Brincat S. , Marsden C. D. , Parkes J. D.
Published: January 2005

Twenty‐six patients with late‐stage Parkinson disease were given 0.4 to 15 mg of pergolide mesylate daily in addition to, or as replacement for, levodopa or bromocriptine therapy. Despite treatment with individually determined optimum doses of levodopa, bromocriptine, and anticholinergics, they had shown response failure or fluctuating response. Forty percent (11 patients) were unable to tolerate pergolide. Nausea and vomiting, somnolence, and psychiatric disturbances were the most frequent side effects. Eleven of the remaining patients improved on pergolide, 2 were unchanged, and 2 were slightly worse. Among the patients who benefited, pergolide improved dose‐related response fluctuations more than non‐dose‐related fluctuations, with a reduction in number and duration of “off” periods and improvement in quality of sleep and early morning akinesia but little change in freezing episodes. Despite treatment failure in many cases, pergolide is at present the best available drug for specific latestage management problems.
A defect in catecholamine metabolism in kinky‐hair disease
Authors: Grover Warren D. , Henkin Robert I. , Schwartz Michael , Brodsky Nancy , Hobdell Elizabeth , Stolk Jon M.
Published: January 2005

The measurement of blood and cerebrospinal fluid levels of copper, dopamine β‐hydroxylase, dopamine, norepinephrine, and epinephrine in a patient with kinky‐hair disease indicated a block in the conversion of dopamine to norepinephrine. The decreased activity of dopamine β‐hydroxylase was not corrected by increasing serum copper levels. A second patient with kinky‐hair disease had similar clinical and biochemical findings on initial examination. With oral supplemental copper therapy, the second patient attained levels within the control range for blood and cerebrospinal fluid copper, dopamine β‐hydroxylase, dopamine, and norepinephrine, demonstrating a state of genetic heterogeneity for this syndrome.
Ross river virus‐induced demyelination: I. Pathogenesis and histopathology
Authors: Seay Alan R. , Wolinsky Jerry S.
Published: January 2005

Ross River virus (strain T48) infection in mice causes and encephalomyelitis characterized by focal, primary demyelination in the cerebellum, brainstem, and spinal cord. Maximal serum and brain content of virus occurs on days 2 and 4, respectively. Virus is not detectable in serum after day 3 or in brain after day 9. Histopathological lesions are present by day 2 and consist of perivascular macrophage and polymorphonulcear leukocyte infiltration, focal necrosis in the internal granule cell layer, and myelin disruption. Mononuclear cell infiltrates are present by day 5. Foci of demyelination in the presence of preserved axons become more widespred by day 8, and early partial remyeliantion occurs by day 13. Immunosuppression reduces the mononuclear dell infiltration but does not alter the demyelination. Althogh the mechanism of Ross River virus‐induced demyelination is not known, these findings suggest that it is not imnune mediated.
Pseudoepileptic seizures: Methods and video analysis to aid diagnosis
Authors: Luther J. Scott , McNamara James O. , Carwile Sandra , Miller Patrica , Hope Victor
Published: January 2005

The purposes of this study were to obtain a detailed description of the clinical features of pseudoepileptic (PE) seizures, to try to reproduce these events in the laboratory during a single recording, and to establish a framework useful to the clinician for evaluating patients whose behavior suggests physiological seizures, but about which doubt remains. We analyzed 37 episodes of PE seizures recorded in 30 patients during a single three‐hour video/EEG recording. The PE seizures occurred spontaneously or were induced by sequential activation procedures. The historical information together with behavioral observations show that the PE seizures mimicked primary generalized seizures in 15 episodes, elementary partial seizures with secondary generalization in 21 episodes, and complex partial seizures in a single episode. Our data underscore the usefulness of the video/EEG recording method and suggest activation techniques which, combined with detailed historical information, aid in establishing the diagnosis of PE seizures.
Progression of carotid disease after endarterectomy: A Doppler ultrasound study
Authors: Norrving Bo , Nilsson Bengt , Olsson Jan‐Edvin
Published: January 2005

The clinical prognosis and evolution of carotid lesions after unilateral endarterectomy were determined in 64 patients examined 1 to 13 years after surgery (mean observation period, 6 years). Surgery mainly was confined to symptomatic patients with a stenosis only on the appropriate side. Average annual stroke rate was 1.6% on the operated and 0.8% on the nonoperated side. Direct Doppler examination at follow‐up revealed a recurrent stenosis (≥50%) or occlusion in 36% of the operated carotid arteries, not significantly different from the proportion of progressive carotid lesions on the nonoperated side (27%). In total, 43.8% of the patients had developed new lesions in one or both carotid arteries, as compared to previous angiographic findings. About 30% of the progressive lesions were associated with symptoms of transient ischemic attacks or stroke, as opposed to 5.5% of vessels without progression of lesions (p < 0.001). The incidence of recurrent stenosis on the operated side is considerably higher than that previously reported for symptomatic recurrent stenosis, but may represent the natural course of carotid disease in this population, in which carotid surgery thus should not be regarded as definitive treatment.
Preoperative CT diagnosis of mesial temporal sclerosis for surgical treatment of epilepsy
Authors: Wyler Allen R. , Bolender Nicole‐Fr.
Published: January 2005

A computerized tomographic (CT) scanning technique has been devised by which the anatomy of the mesial temporal lobe can be examined in epileptics who are being considered for temporal lobectomy. Among 25 patients examined with this technique, 17 have had the anatomy of their mesial temporal lobe verified at craniotomy, showing close correlation between surgical findings and the high‐resolution cerebrospinal fluid–enhanced CT scans. Atrophy was present in 4 patients, whereas chronic herniation of the mesial temporal structures over the free edge of the tentorium was present in 12; in 1 patient the structures were normal. The correlation of anatomical lesion with an electroencephalographically (EEG) defined epileptogenic focus will improve the outcome of surgery for epilepsy. This CT technique aids in identifying the epileptogenic temporal lobe for patients in whom the focus is ambiguous by EEG criteria alone.
Comparison between fascicular and whole sural nerve biopsy
Authors: Pollock Martin , Nukada Hitoshi , Taylor Peter , Donaldson Ivan , Carroll Grant
Published: January 2005

Sural nerve regeneration and sensation were evaluated in 16 subjects five or more years after fascicular or whole nerve biopsy. Conduction studies demonstrated successful nerve regeneration in all subjects who had whole nerve biopsy. Postbiopsy interrogation revealed no long‐term pain or paresthesias but a high incidence of tactile‐induced dysesthesias. No significant difference was seen when areas of sural sensory loss were compared in fascicular and whole nerve biopsy groups. We conclude that whole nerve biopsy should be recommended in preference to fascicular biopsy since it is simpler, has greater diagnostic potential, and allows for a more complete morphological evaluation.
Decreased pyruvate dehydrogenase complex activity in Huntington and Alzheimer brain
Authors: Sorbi Sandro , Bird Edward D. , Blass John P.
Published: January 2005

The activity of the pyruvate dehydrogenase complex (PDHC) was reduced in affected areas of brain from patients with Huntington disease (caudate, putamen) and Alzheimer disease (frontal cortex) where choline acetyltransferase (CAT) activity was low. PDHC was also deficient in an area (Huntington hippocampus) where CAT was not significantly reduced. The activity of fumarase, an inner mitochondrial marker, was normal in all areas examined. The activities of PDHC and CAT correlated well in caudate, putamen, and amygdala but not in hippocampus or frontal cortex. Both total activity and activation of PDHC were below normal in fibroblasts from 4 patients with C‐21 trisomy Down syndrome, who are at very high risk to develop Alzheimer disease. However, no abnormality of PDHC was detected in Huntington or Alzheimer fibroblasts. Deficiency of PDHC may play a role in the pathophysiology of Huntington and Alzheimer diseases, although it does not appear to be a primary defect. Loss of tissue oxidative capacity may relate to the reduction in cerebral metabolic rate and blood flow which are characteristic of many dementing illnesses.
Prenatal symmetrical thalamic degeneration with flexion spasticity at birth
Authors: Parisi J. E. , Collins G. H. , Kim R. C. , Crosley C. J.
Published: January 2005

The unusual occurrence of spasticity at birth with symmetrical thalamic damage was found in a male infant delivered at 36 weeks' gestation following an episode of traumatically induced premature labor at 32 weeks. The infant was found to be spastic in flexion with increased stretch reflexes, depressed primitive reflexes, and moderate flexion contractures. Computerized tomographic scans showed bilateral nonenhancing thalamic densities. Neuropathologically, the lateral thalamic nuclei and the red nucleus showed neuron loss, astrocytosis, and, as confirmed by electron microprobe analysis, calcified neurons. The striatum was uninvolved. These findings closely resemble those reported as “symmetrical thalamic degeneration in infancy” and are strongly reminiscent of the pattern of thalamic involvement frequently seen in status marmoratus. It would appear that there is a period during perinatal life in which the lateral thalamus can be rendered vulnerable to hypoxic‐ischemic injury, and that the thirty‐second week of gestation must be included within this period.
Pupil‐sparing oculomotor palsies with internal carotid—posterior communicating artery aneurysms
Authors: Kissel John T. , Burde Ronald M. , Klingele Terence G. , Zeiger H. Evan
Published: January 2005

The charts of 84 patients admitted to the Neurosurgery Service of the Washington University Medical Center between January, 1960, and July, 1981, with aneurysms at or near the junction of the internal carotid and posterior communicating arteries (ICA‐PoCA) were reviewed. Special emphasis was placed on the pupillary size and reactivity of 51 patients with oculomotor nerve involvement. Seven initially had normal pupils, representing 8% of the total group and 14% of those presenting with oculomotor palsies. In 4 of these patients pupillary involvement developed within 5 days, and in I pupillary involvement developed in 4 months. Pupillary sparing appears to be more common than previously appreciated in patients with ICA‐PoCA aneurysms and oculomotor nerve involvement. Patients with acute somatic oculomotor paresis should be observed closely for at least one week for the development of pupillary involvement. Arteriography may be indicated more frequently than previously recommended.
Electrophysiology of duchenne dystrophy myotubes in tissue culture
Authors: Rothman Steven M. , Bischoff Richard
Published: January 2005

Single myotubes from cultures of 6 patients with Duchenne muscular dystrophy and 6 patients whose biopsy specimens lacked histological abnormality were studied with intracellular physiological recording. Average resting membrane potentials were 47.8 ± 2.3 mV and 47.0 ± 1.5 mV for the control and Duchenne cultures, respectively. Action potentials elicited at a standard resting potential of −80 mV were 97.3 ± 4.0 mV and 98.6 ± 8.2 mV for the two groups. Maximum rate of rise of action potentials was 154.2 ± 25.2 V/sec for control and 143.1 ± 37.1 V/sec for Duchenne myotubes. Action potentials mediated by an influx of calcium ions were seen only when the myotubes from both groups were bathed in high concentrations of calcium and 4‐aminopyridine (20 mM and 1 mM, respectively). Thus, the plasma membrane of Duchenne dystrophy myotubes does not have active electrical properties that differ from those in controls. Previous reports of low resting membrane potentials in biopsies studied acutely may reflect secondary changes in degenerating fibers.
Myoclonic ocular jerks
Authors: Selhorst John B. , Ochs Alfred L. , Selbst Richard G.
Published: January 2005

A 39‐year‐old woman with a familial trait for myoclonic jerks of the neck presented with spontaneous 5‐ to 40‐degree single jerks of the eyes, usually upward, each producing a brief interruption in fixation. Eye movement recordings showed the myoclonic ocular jerks to be saccades with variable intersaccadic intervals. Treatment with 6.0 mg per day of clonazepam reduced her symptoms.
Studies of human and monkey “Epileptic” neocortex in the in vitro slice preparation
Authors: Schwartzkroin Philip A. , Turner Dennis A. , Knowles W. Douglas , Wyler Allen R.
Published: January 2005

The in vitro slice technique was used to study neuronal activity in human cortical tissue removed during neurosurgical Procedures for intractable epilepsy and in monkey neocortex rendered epileptogenic by injection of alumina gel. In both cases, biopsies were guided by electrocorticographic signs of epileptiform activity. Intracellular recordings were made from 167 neurons in human tissue and from 73 neurons in monkey tissue samples. There was little spontaneous activity in these biopsies and no indication of spontaneous cellular bursting. Stimulation at the pial surface or in white matter evoked synaptically driven activity that was primarily excitatory. Graded bursts of activity could be elicited from some cells, but no all‐or‐none paroxysmal depolarization shifts were recorded. Inhibitory postsynaptic potentials were relatively rare. Intracellular injections of dye in a small number of neurons revealed no obvious differences between bursting and nonbursting neurons. These in vitro studies of chronic epileptic cortex have thus far provided few clear insights into the basic mechanisms of epilepsy.
Abnormal unloading reflex in a patient with infarction of the medial lemniscus
Authors: Angel Ronald W. , Goldstein Mark
Published: January 2005

The unloading reflex was measured in the intrinsic muscles of the hand. By removing various fractions of the load on the muscles, we determined the size of the electromyographic response as a function of the change in the external force. This technique was applied to both hands of a patient with a pontine lesion who had impaired position sense in the left hand. The response to fractional unloading was significantly smaller in the left hand, an asymmetry not found in control subjects. The results suggest (1) that the unloading reflex is mediated, at least in part, by suprasegmental pathways, and (2) that the fractional unloading technique can reveal abnormalities not found by conventional methods.
Oral physostigmine and lecithin improve memory in Alzheimer disease
Authors: Thal L. J. , Fuld P. A. , Masur D. M. , Sharpless N. S.
Published: January 2005

Eight patients with early Alzheimer disease were treated with gradually increasing multiple daily doses of oral physostigmine and supplemental lecithin. Six individuals showed improvement in total recall and retrieva storage (LTR), with a decrease in intrusions (a measure of inaccurate recall). The optimal individual dose was either 2.0 or 2.5 mg of physostigmine for each responding patient. Results of this open trial were subsequently replicated during a double‐blind crossover trial comparing physostigmine treatment to placebo. All six patients again demonstrated improvement in total recall and LTR, with a decrease in intrusions. The decrease in intrusions was strongly correlated with increasing inhibition of cholinesterase activity in cerebrospinal fluid, suggesting that the degree of improvement in the patient's memory was related to the amount of physostigmine that reached the brain. Other improvement and metabolites in cerebrospinal fluid were unaffected by the physostigmine therapy, suggesting a specific effect of physostigmine on the cholinergic system. The results suggest that small oral doses of physostigmin combined with lecithin ingestion have therapeutic benefit for some patients with Alzheimer disease.
Myasthenia gravis in children: Long‐term follow‐up
Authors: Rodriguez Moses , Gomez Manuel R. , Howard Frank M. , Taylor William F.
Published: January 2005

We report observations made on 149 patients with juvenile myasthenia gravis studied from onset of disease for as long as 40 years. Median follow‐up was 17 years; minimum was 4 years. Eight other patients with congenital myasthenia gravis were studied separately. Of the juvenile myasthenic patients, 85 (57%) underwent thymectomy because of disease severity. In juvenile myasthenia gravis, a spontaneous remission rate of 22.4 per 1,000 person‐year was observed, regardless of disease duration. A remission rate of 260 per 1,000 person‐years was seen during the first year after thymectomy, with a rate of 95 per 1,000 person‐years during the next 2 years. Early surgery, presence of bulbar symptoms, absence of ocular signs or generalized symptoms, onset of symptoms between ages 12 and 16, and presence of other immune disease were associated with increased postoperative remission rates. Epilepsy (4 patients) and neoplasia (7 patients) were the most frequent associated nonimmune disorders; rheumatoid arthritis (5 patients) and neoplasia diabetes mellitus (3 patients), asthma (3 patients), and thyroid disease (3 patients) were the most frequent associated immune diseases.
Roles of echocardiography and arrhythmia monitoring in the evaluation of patient with suspected systemic embolism
Authors: Come Patricia C. , Riley Marilyn F. , Bivas Natalie K.
Published: January 2005

Patients with suspected systemic embolism were evaluated for possible cardiac origin of emboli using echocardiography (280 patients) and prolonged arrhythmia (Holter) monitoring (150 patients). Echocardiography demonstrated cardiac abnormalities that might predispose to emboli in 47% of patients with and 14% of patients without clinically evident cardiovascular disease. Lesions that might be directly responsible for emboli, including thrombi, myxomas, and vegetations, were identified in only 11 patients (4%), all of whom had clinically evident disease. Patients less than 45 years of age with clinically evident cardiovascular disease were especially likely to have a potential source of embolism identified.

Holter monitoring demonstrated atrial fibrillation in 15 patients. All 15, however, had known atrial fibrillation in the past or had atrial fibrillation detected on admission electrocardiogram, or both.

The findings suggest that it is not cost effective to perform echocardiography and arrhythmia monitoring on all patients with suspected systemic emboli. Because echocardiographic identification of a likely responsible lesion was limited to those with clinically evident cardiovascular disease, a policy of performing this test only on such patients would have eliminated 38% of the echocardiographic studies in this series.
Computed tomographic scan correlates of auditory comprehension deficits in aphasia: A prospective recovery study
Authors: Selnes Ola A. , Knopman David S. , Niccum Nancy , Rubens Alan B. , Larson David
Published: January 2005

Lesion localization and volume as assessed on computed tomography were analyzed to determine their relationships to auditory comprehension deficits following left hemisphere ischemic stroke in 39 patients. Auditory comprehension was initially tested approximately four weeks after onset, and then monthly for five months, using the Token Test, a series of commands of increasing complexity. Computed tomographic scans obtained five months after onset were used for lesion localization and volume determination. Lesions in the posterior superior temporal and infrasyvian suprmarginal regions were strongly associated with poor outcome. Patients with initially preserved auditory comprehension tended to have lesions that spared these regions, whereas patients with persistent deficits had lesions that included these regions. Six of 8 patients who initially had severe deficits in auditory comprehension but recovered completely lacked lesions in these regions. Lesion volume except when very large or very small was not closely associated with outcome.
Age dependent susceptibility to reovirus type 3 encephalitis: Role of viral and host factors
Authors: Tardieu Marc , Powers M. Linda , Weiner Howard L.
Published: January 2005

Reovirus type 3 inoculated intracerebrally or subcutaneously into newborn mice induced an acute necrotizing and uniformly fatal encephalitis. Subsequently, between the eighth and tenth day of age, reovirus type 3 infection changed to a nonlethal infection. This pattern of virulence was directly correlated with the ability of the virus to replicate in the brain and histologically was accompanied by a gradual diminution in the intensity and extent of encephalitis such that histological abnormalities were absent in the brains of virus‐injected adult animals. Some animals injected at ages 10 to 18 days developed brainstem and diencephalic lesions, indicating a nonuniform resistance of neurons to viral infection with aging. Neither a changing immune response pattern nor a modification or loss of viral receptors appears to explain either the susceptibility of newborn or the resistance of adult animals to reovirus type 3 encephalitis. Splenic mononuclear cells from mice younger (but not older) than 7 days also permitted reovirus type 3 replication in vitro. Thus, the age dependent resistance to reovirus type 3 encephalitis appears related to an intrinsic resistance to viral replication by neuronal cells, a resistance that may occur simultaneously in nonneuronal cell populations.
A sibship with neuroaxonal dystrophy and renal tubular acidosis: A new syndrome?
Authors: Maccario Micheline , Mena Hernando , Weir Michael R. , Matson Michael D. , Reimann Bernhard E. F.
Published: January 2005

The clinical and pathological features of three siblings from nonconsanguineous parents are described. Two of the children died at 61/2 and 7 months of age of a central nervous system disorder characterized by severe behavioral retardation, hypotonia, and pyramidal tract signs. The third child, now 8 years old, has global mental retardation, inability to talk, marked tremors, and gait disturbances. All children exhibited inability to concentrate or acidity the urine, with growth delay partially corrected in the living child by alkali therapy. Computed tomographic scans revealed hyperdense lesions in the thalamus of one of the siblings and more striking hyperdensity of the basal ganglia in the eldest child.

Findings at postmortem examination in two children, and by renal and sural nerve biopsy in the third, include swollen axons in the internal capsule and peripheral nerves, and neurons with iron‐staining deposits, gliosis, and macrophages containing lipofuscin pigments in the brain. The renal findings include material positive for periodic acid–Schiff and hyperdense granular deposits in renal tubules.
Tetrahydrocannabinol for tremor in multiple sclerosis
Authors: Clifford David B.
Published: January 2005

Based on one patient's enthusiastic report, eight patients with multiple sclerosis, seriously disabled with tremor and ataxia, were given oral tetrahydrocannabinol. Two demonstrated improved motor coordination.
Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease
Authors: Salazar Andres M. , Masters Colin L. , Gajdusek D. Carleton , Gibbs Clarence J.
Published: January 2005

A review of over 2,000 cases of Creutzfeldt‐Jakob disease and related disorders in the literature and our own files yielded 231 cases of dementia with early lower motor neuron signs. The clinical‐pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt‐Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.
Multiple malignant astrocytomas in a patient with spontaneous progressive multifocal leukoencephalopathy
Authors: Sima Anders A. F. , Finkelstein Sydney D. , McLachlan Donald R.
Published: January 2005

A 67‐year‐old man with a four‐year history of horizontal and vertical nystagmus and progressive cerebellar dysfunction later developed profound dementia and died of bronchopneumonia. Neuropathological examination revealed numerous active and inactive progressive multifocal leukoencephalopathy (PML) lesions in the cerebrum and cerebellum. Active lesions showed marked perivascular lymphocytic cuffing. Papovaviruses were identified ultrastructurally in both oligodendrocytes and astrocytes. Multiple malignant astrocytomas were documented, originating from PML lesions. No viruses could be demonstrated in neoplastic astrocytes. An immune‐deficient state was not revealed clinically or at postmortem examination.
Gerstmann‐Sträussler‐Scheinker's disease
Authors: Kuzuhara Shigeki , Kanazawa Ichiro , Sasaki Hidenao , Nakanishi Takao , Shimamura Kayako
Published: January 2005

Findings are reported in three members of a Japanese family with a chronic familial disease characterized by signs of marked cerebellar dysfunction, mild pyramidal and extrapyramidal dysfunction, and loss or decrease of the kness and ankle jerks. Although the clinical features suggested olivopontocerebellar atrophy, postmortem study of one patient with obvious dementia revealed massive multiform plaques of Kuru type as well as multicentric, senile, and primitive types throughout the central nervous system, most prominent in the cerebellar and cerebral cortics and caudate nucleus. There was degeneration of the spinocerebellar and pyramidal tracts, posterior columns, superior cerebellar peduncles, cerebellar cortex, dentate nucleus, and vestibular nuclei as well as gliosis of the inferior colliculus and cerebellar foliar white matter. There were no cerebral spongiform changes, although slight spongy alteration without glial reaction was present. The clinical and neuropathological characteristics were consistent with those reported as Gerstmann‐Sträussler‐Scheinker's disease in an Austrian family.
Functional recovery of cortical neurons as related to degree and duration of ischemia
Authors: Heiss W.‐D. , Rosner G.
Published: January 2005

Imultaneous recordings of spontaneous single cell activity and local cerebral blood flow were obtained from 72 cortical neurons and adjacent brain in 54 cats before, during, and after ischemia induced by reversible occulsion of the middle cerebral artery. In most cells spontaneous electrical activity ceased at flow values of about 0.18 ml/gmm/in (range, 0.06 to 0.22 ml/gm/min). No signal was obtained from 28 neurons during reperfusion following ischemia of varying degree and duration. Overall, neurons exposed to a residual flow of 0.14 ml/gm/min or less for more than 45 minutes had a poorer prognosis compared to any other combination of degree and duration of ischemia. A discriminant curve was estimated to define the border line between recovering and nonrecovering cells. Regions showing irreversible neuronal failure contained selective neuronal necrosis or areas of infarction by histological examination. Reperfusion restored neuronal function in 44 cells. In this group of neurons, there was a joint interaction of duration of ischemia, ischemic residual flow, and recovery time: cells exposed to moderate ischemia (0.09 to 0.22 ml/gm/min) for up to 20 minutes recovered rapidly; most neurons subjected either to extreme ischemia (less than 0.09 ml/gm/min) of short duration (less than 20 minutes) or to moderate ischemia (0.09 to 0.22 ml/gm/min) for longer periods (20 to 141 minutes) required from 19 to 50 minutes for recovery. A few resistant neurons tolerated less than 0.09 ml/gm/min for more than 20 minutes.

Cell survival and functional recovery following ischemia depend not only on separate effects of residual flow and duration of ischemia but on their combined influence. Selective vulnerability forms the natural basis of scattered cell survival.
Tissue acid‐base balance and oxygen metabolism in human cerebral infarction studied with positron emission tomography
Authors: Syrota A. , Castaing M. , Rougemont D. , Berridge M. , Baron J. C. , Bousser M. G. , Pocidalo J. J.
Published: January 2005

Nine patients who had suffered strokes were examined between 10 and 34 days after onset using positron emission tomography. DMO labeled with carbon 11 was used to evaluate brain acid‐base balance, and the oxygen‐15 inhalation technique was used to measure regional cerebral blood flow, the oxygen extraction fraction, and cerebral metabolic rate for oxygen. {11C}DMO concentration and oxygen metabolism variables were measured in the infarcted area and in the symmetrical region in the contralateral cerebral hemisphere. {11C}DMO concentration was found to be unchanged or slightly increased in five cases and markedly increased in four cases. The apparent increase in tissue pH can be explained by the presence of a large extracellular fluid space with a pH nearly identical to that of brain plasma, or by an increase in intracellular pH, or by both phenomena. The change in {11C}DMO concentration in the infarcted area relative to that in the normal tissue was independent of the change in blood flow. Cerebral metabolic rate for oxygen was decreased in all cases. The increase in {11C}DMO concentration in the infarcted area was linearly correlated with the decrease in the oxygen extraction fraction in the same region; that is, it was correlated with the occurrence of perfusion in excess of metabolic demand. The overabundant local perfusion could play a role in the decreased H+ content.
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: Report of 35 cases
Authors: Hagberg Bengt , Aicardi Jean , Dias Karin , Ramos Ovidio
Published: January 2005

Thirty‐five patients, exclusively girls, from three countries had a uniform and striking progressive encephalopathy. After normal general and psychomotor development up to the age of 7 to 18 months, developmental stagnation occurred, followed by rapid deterioration of higher brain functions. Within one‐and‐a‐half years this deterioration led to severe dementia, autism, loss of purposeful use of the hands, jerky truncal ataxia, and acquired microcephaly. The destructive stage was followed by apparent stability lasting through decades. Additional insidious neurological abnormalities supervened, mainly spastic parapareses, vasomotor disturbances of the lower limbs, and epilepsy. Prior extensive laboratory investigations have not revealed the cause. The condition is similar to a virtually overlooked syndrome described by Rett in the German literature. The exclusive involvement of females, correlated with findings in family data analyses, suggests a dominant mutation on one X chromosome that results in affected girls and nonviable male hemizygous conceptuses.
Antibodies to cerebellar Purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma
Authors: Greenlee John E. , Brashear H. Robert
Published: January 2005

Sera from 2 patients with ovarian carcinoma and paraneoplastic cerebellar degeneration confirmed postmortem were reacted with frozen sections of human cerebellum and stained using indirect immunofluorescence methods. Both sera produced bright cytoplasmic staining of Purkinje cells and of neurons within deep cerebellar nuclei. Titration of these sera to end point revealed staining at final dilutions of 1:640 and 1:2, 560, respectively. Neither of these sera reacted with sections of human cerebrum, basal ganglia, spinal cord, peripheral nerve, lung, liver, kidney, or ovary. Staining of Purkinje cells was not obtained with sera from 34 normal, healthy controls, 5 patients with oat cell carcinoma of the lung, 6 patients with inflammatory central nervous system disorders, or 12 of 14 neurologically normal patients with ovarian carcinoma. Sera from 2 neurologically normal patients with ovarian carcinoma, however, produced staining of Purkinje cells and deep nuclei similar to that obtained with sera from patients with paraneoplastic cerebellar degeneration. The present study documents the presence of antibodies to Purkinje cells in patients with ovarian carcinoma and cerebellar degeneration and demonstrates that development of these antibodies may antedate the onset of clinically evident cerebellar degeneration.
Cauda equina syndrome secondary to long‐standing ankylosing spondylitis
Authors: Bartleson J. D. , Cohen M. D. , Harrington T. M. , Goldstein N. P. , Ginsburg W. W.
Published: January 2005

Fourteen patients with cauda equina syndrome secondary to long‐standing ankylosing spondylitis are described. The roughly symmetrical neurological deficits were very slowly progressive and began long after the onset of the spondylitis, usually well after the rheumatological symptoms had stopped. Eventually every patient had cutaneous sensory loss in the fifth lumbar and sacral dermatomes. All patients developed urinary sphincter disturbances of a lower motor neuron type. There was prominent loss of rectal sphincter tone, and all but 2 patients had bowel complaints, including incontinence and severe constipation. Seven patients had mild to moderate weakness in the lumbosacral myotomes. Seven patients had pain in the rectum or lower limbs. Electromyographic abnormalities were consistent with multiple lumbosacral radiculopathies. Myelography and computed tomographic scanning of the lumbosacral spine showed characteristic enlargement of the caudal sac and dorsal arachnoid diverticula that had eroded the laminae and spinous processes. Recognition of this syndrome, coupled with computed tomographic scanning of the lower sponal canal, allows one to omit myelography, a procedure that is difficult because of the associated spine abnormalities. Surgical intervention should be avoided.
A Progressive familial encephalopathy in infancy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis
Authors: Aicardi J. , Goutières F.
Published: January 2005

Eight infants developed a progressive disorder of the central nervous system with bilateral spasticity and dystonia, acquired microcephaly, and a rapid course toward profound deterioration and death. All the patients had abnormal cerebrospinal fluid with mild but persistent lymphocytosis. Computed tomography showed various combinations of bilateral symmetrical calcifications in the basal ganglia, progressive brain atrophy, and deep white matter hypodensities, the first two being present in all families but not in every individual patient. The disorder is familial and probably genetic in origin, although some features, especially the pleocytosis, may erroneously suggest an inflammatory condition.
Biochemical studies of pyridoxal and pyridoxal phosphate status and therapeutic trial of pyridoxine in patients with carpal tunnel syndrome
Authors: Smith Gillian P. , Rudge P. J. , Peters T. J.
Published: January 2005

A number of recent studies report response of patients with carpal tunnel syndrome to pyridoxine treatment. Neurological and biochemical studies were therefore performed on six patients both before and after treatment with pyridoxine for at least 9 weeks. Free pyridoxal, pyridoxal phosphate, and total pyridoxal were assayed in plasma and neutrophils. The pyridoxal status was also estimated by assaying red cell aspartate aminotransferase. No evidence was obtained to suggest that these patients were deficient in either pyridoxal or pyridoxal phosphate. Although four of the patients claimed some partial symptomatic relief, there was no consistent improvement in clinical findings or neurophysiological measurements following pyridoxine treatment.
Huntington's disease: Survival of large striatal neurons in the rigid variant
Authors: Bugiani Orso , Tabaton Massimo , Cammarata Sergio
Published: January 2005

A morphometric method was employed to investigate the relationship of rigidity and hyperkinesia to the degree of striatal and nigral nerve cell loss in one patient with the rigid variant of Huntington's disease and four patients with hyperkinetic chorea. Both striatal neuron populations, small and medium‐sized neurons and large neurons, were affected in the patients with hyperkinetic chorea, whereas the large neurons were preserved in the patient with the rigid variant. The substantia nigra was slightly involved in each patient. The findings suggest that the rigid variant of Huntington's disease may reflect the unbalanced activity of residual, mostly large, striatal neurons, inhibiting the substantia nigra.
Effect of baclofen on sleep‐related periodic leg movements
Authors: Guilleminault Christian , Flagg Wayne
Published: January 2005

Five patients with nocturnal myoclonus (periodic leg movements during sleep), mean age 59.6 years, were monitored lygraphically for fifteen successive nights. Using a double‐blind drug study design with placebo at baseline, we inestigated the effect of baclofen on these patients. All patients had the repetitive sleep‐related abnormal movements dring both the baseline nights and those on which baclofen had been administered. The number of movements varied during the four baseline nights, but the movements induced sleep fragmentation, i.e., very short electroencepha graphic changes. Baclofen increased the number of movements but decreased their amplitude during non–rapid eye movevement (REM) sleep and shortened the interval between movements. Its effect on sleep was dose related: as dosages Ceased, delta sleep progressively increased and REM sleep decreased. Sleep fragmentation resulting from muscle aches decreased, as indicated by the diminution in alpha electroencephalographic arousals and K complexes. Baclo‐dosages of 20 mg and 40 mg were the most efficacious.
Neglect in children
Authors: Ferro José M. , Martins Isabel Pavão , Távora Luis
Published: January 2005

Three children developed neglect following right‐hemispheric lesions. In the first, computed tomography (CT) demonstrated a striatoinsular infarction. In the second, CT revealed a large meningioma compressing the right hemisphere, and in the third, CT revealed two intracerbrall hemorrhages in the posterior parietal and ocupital lobes. Neglect in these children produced a clinical picture comparable to that occurring in adults, but there was a rapid and complete recovery. The paucity of reports of neglect in children may be a function of the rapid recovery, the phenomenon being missed if not looked for in the acute atage. We conclude that, at least for children aged aged 6 years or more, the right hemisphere is dominant for directed selective attention.
Hypothalamic mass and gigantism in neurofibromatosis: Treatment with bromocriptine
Authors: Duchowny Michael S. , Katz Raananah , Bejar Rafael L.
Published: January 2005

A child with neurofibromatosis exhinbited gigantism and acromegaly in association with a hypothalamic mass lesion. Bromocriptine, 5 mg daily, reduced somatic growth rate and restored biochemical mohesostasis but had no effect on tumor grwoth. Radiation therapy arrested tumor enlargement and stabilized deeriorating visual function.
Parkinson's disease: Neuron loss in the nucleus basalis without concomitant Alzheimer's disease
Authors: Nakano Imaharu , Hirano Asao
Published: January 2005

A representative region of the nucleus basalis of Meynert was investigated in 11 patients with idiopathic Parkinson's disease and compared with the identical region in 13 age‐matched control subjects. Simultaneously, the cerebral cortex and the nucleus basalis in the patients with Parkinson's disease were examined for senile plaques and Alzheimer's neurofibrillary tangles. The nucleus basalis was significantly depleted of its large neurons in Parkinson's disease (p < 0.001 versus controls; Student t test), but in the majority of cases the neuron loss was not associated with Alzheimer's disease.
Axolemma is a mitogen for human Schwann cells
Authors: Sobue Gen , Brown Mark J. , Kim Seung U. , Pleasure David
Published: January 2005

The mechanisms responsible for the induction of Schwann cell proliferation in peripheral nerves undergoing wallerian degeneration and segmental demyelination are not understood. To determine whether contact with axolemma stimulates mitosis of human Schwann cells, cultured Schwann cells from spinal roots obtained postmortem and from sural nerve biopsy specimens were incubated with axolemmal fractions prepared from human spinal cord or from adult rat central nervous system. Schwann cell proliferation was estimated by autoradiographic assay of tritiated thymidine incorportion. Schwann cell labeling indices after exposure to human or rat axolemmal fractions ranged from 26.7 to 59.9%; labeling indices of Schwann cells cultured without axolemmal fraction were 9.8 to 22.4%. The stimulation index, or ratio of Schwann cell labeling index with axolemmal fraction to that without axolemmal fraction, ranged from 1.97 to 3.40. This study demonstrates that both human and rat axolemma are capable of stimulating human Schwann cell replication in vitro.
Reversible visual evoked potential abnormalities in vitamin E deficiency
Authors: Messenheimer John A. , Greenwood R. S. , Tennison M. B. , Brickley J. J. , Ball C. J.
Published: January 2005

A patient with cystic fibrosis and cirrhosis developed a progressive neurological syndrome associated with ataxia, proximal weakness, and ophthalmoplegia. Profound deficiencies of vitamins A, D, and E were present. Visual acuity and results of retinal funduscopy were normal. The pattern reversal visual evoked potential was initially abnormal (P100 latency, 136 and 130 ms from left and right eyes, respectively) but became normal (less than 3 standard deviations from mean control P100 latency) over a two‐month period when vitamin E was administered. This case documents a potentially reversible visual evoked potential abnormality in a visually asymptomatic patient with vitamin E deficiency.
Monoclonal antibody analysis of mononuclear cells in myopathies. I: Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells
Authors: Arahata Kiichi , Engel Andrew G.
Published: January 2005

In 76 muscle specimens (normal controls, 9; Duchenne dystrophy, 11; scleroderma, 11; dermatomyositis, 13; polymyositis, 15; inclusion body myositis, 17), mononuclear cells were analyzed at perivascular, perimysial, and endomysial sites of accumulation. Monoclonal antibodies reactive for B cells, T cell subsets, killer (K) or natural killer (NK) cells, and the Ia antigen were used for cell typing. Macrophages were identified by the acid phosphatase reaction. Few extravascular mononuclear cells occurred in normal muscle. In all inflammatory myopathies, a mixed exudate of T cells, B cells, and macrophages was present. Mature K/NK cells were rare in all diseases. In dermatomyositis, polymyositis, and inclusion body myositis, there was a positive gradient for T cells, T8+ cells, and activated T cells and a negative gradient for B cells and T4+ cells between perivascular and endomysial sites. In scleroderma the predominant perimysial exudate consisted mostly of T cells and macrophages. The percentage of B cells at all sites, and the T4+/T cell ratio in the endomysium, were significantly higher in dermatomyositis than in the other diseases. In polymyositis and inclusion body myositis. The endomysial exudate contained a large number of T cells, T8+ cells, and activated T cells but only sparse B cells. T cells accompanied by macrophages focally surrounded and invaded nonnecrotic fibers in polymyositis and inclusion body myositis. Rare fibers in Duchenne dystrophy and a very few fibers in dermatomyositis and scleroderma were similarly affected. We infer that (1) T‐B, T‐T, and T‐macrophage cooperativities are likely to exist in muscle in different myopathies; (2) T cell‐mediated fiber injury plays a role in polymyositis and inclusion body myositis; (3) T cell–mediated fiber injury can also occur in inherited diseases, such as Duchenne dystrophy; and (4) a local humoral response may occur in muscle in dermatomyositis and possibly in polymyositis and inclusion body myositis.
Decreased axon caliber and neurofilaments in hereditary motor and sensory neuropathy, Type I
Authors: Nukada Hitoshi , Dyck Peter James
Published: January 2005

The axons of large‐ and intermediate‐diameter myelinated fibers of sural nerves of patients with hereditary motor and sensory neuropathy, type I (HMSN‐I), were previously found to be attenuated relative to their myelin spiral length. We inferred that axonal atrophy might account for secondary segmental demyelination and remyelination. To assess whether the observed axonal atrophy could be explained by a decrease in neurofilaments, we have evaluated the number of neurofilaments, microtubules, and other axon organelles in sural nerves of patients with HMSN‐I. Whereas the density per square micrometer of neurofilaments or microtubules in diseased nerves was not significantly different from that in control specimens, the number of neurofilamets per axon as related to myelin spiral length was significantly less for intermediate and large myelinated fibers in HMSN‐I nerves. The regression lines for the number of microtubules per axon on myelin spiral lengths, were also less steep in HMSN‐I, but the difference did not reach statistical significance. These results indicate that the number of neurofilaments is proportional to axon diameter but significantly below that expected considering myelin spiral length. Decreased neurofilament synthesis, assembly, or transport may underlie the axonal atrophy in HMSN‐I.