bluuebunny/crossref_metadata_2025 huggingface dataset:
Cortical activation pattern during saccadic eye movements in humans: Localization by focal cerebral blood flow increases
Authors: Melamed Eldad , Larsen Bo
Published: January 2005

Regional cerebral blood flow (rCBF) was measured in human subjects during saccadic eye movements by a 254‐channel dynamic gamma camera. Focal rCBF increases were repeatedly observed in an area within the middle precentral and premotor regions which corresponds to the frontal eye field in humans. Our findings suggest that this region is localized between the “face” and “hand” areas in the precentral gyrus and extends anteriorly out of the primary motor strip into the adjacent premotor zone. In addition, saccades were associated with focal blood flow increases in regions corresponding to the frontal supplementary motor area and to the posterior temporooccipital visual association cortex. Similar changes in patterns of rCBF occurred during contralateral and ipsilateral horizontal saccades and also during vertical saccades, and did not differ between the right and left hemispheres. Focal rCBF increases were observed in the frontal eye field during several additional test procedures including ocular fixation and visual perception of a nonmoving target, auditory stimulation with closed eyes, and reading. It is presumed that these focal flow increases reflect increased localized neuronal activity and metabolic rate and therefore permit visualization of the cortical activation pattern associated with saccadic eye movements in humans.
Emery‐dreifuss muscular dystrophy
Authors: Rowland L. P. , Fetell M. , Olarte M. , Hays A. , Singh N. , Wanat F. E.
Published: January 2005

A man had weakness of humeroperoneal distribution associated with limited range of motion of the cervical spine and elbows. At age 25 he developed permanent atrial paralysis, and a cardiac pacemaker was inserted. Although this case was sporadic, most others have been transmitted as an X‐linked recessive trait. Mixed patterns in electromyography and muscle histology have caused nosological confusion, but the unique clinical signs seem to define a distinct form of muscular dystrophy, warranting the designation “Emery‐Dreifuss” type.
Oral phenytoin loading in adults: Rapid achievement of therapeutic plasma levels
Authors: Record Kenneth E. , Rapp Robert P. , Young A. Byron , Kostenbauder Harry B.
Published: January 2005

Oral loading doses of phenytoin were estimated from a simplified pharmacokinetic model. Twenty patients were administered a calculated average dose of 19.1 mg per kilogram of phenytoin divided into two to four increments given over 3 to 21 hours (10.55 ± 4.74, mean ± SD). Plasma samples drawn 2 to 22 hours (10.75 ± 4.32, mean ± SD) after completion of the loading dose resulted in therapeutic levels ranging from 8.1 to 18.0 μg per milliliter (11.42–11.37 ± 2.41, mean ± SD). Thus, this regimen in which no increment of the loading dose exceeds 600 mg is sufficient to achieve and maintain therapeutic plasma concentrations 18 to 24 hours after initiation of the loading dose.
Musical hallucinations in a deaf elderly patient
Authors: Miller Timothy C. , Crosby T. W.
Published: January 2005

A remarkably observant 89‐year‐old woman experienced the abrupt onset of musical hallucinations on the background of longstanding progressive hearing loss and in the absence of psychosis or major dementia. These hallucinations were nearly constant, well‐formed perceptions of religious hymns, and their content could be altered by several mechanisms. The patient also occasionally heard voices recapitulating childhood experiences. One can speculate that the syndrome relates to sensory deprivation.
Brain electrical activity mapping (BEAM): A method for extending the clinical utility of EEG and evoked potential data
Authors: Duffy Frank H. , Burchfiel James L. , Lombroso Cesare T.
Published: January 2005

The difficulties inherent in extracting clinically useful information by visual inspection alone from the massive amounts of data contained in multichannel polygraphic recordings have placed limits on the accuracy and range of utility of electroencephalography and evoked potentials. A method for condensing and summarizing the spatiotemporal information contained in recordings from multiple scalp electrodes is described. Data dimensionality is reduced and visibility increased by computer‐controlled topographic mapping and display of data as color television images. Examples are given in which such brain electrical activity mapping (BEAM) (1) localizes tumors in patients with normal or nondiagnostic EEGs, (2) adds additional information to that visible on computerized axial tomography, and (3) demonstrates electrophysiological abnormalities in patients with functional lesions but normal CT scans. A sensitivity to the functional component of a neurological lesion suggests that BEAM may provide complementary information to the anatomical definition provided by the CT scan.
No barbiturate protection in a dog model of complete cerebral ischemia
Authors: Steen Petter A. , Milde James H. , Michenfelder John D.
Published: January 2005

Complete global ischemia was produced in 39 dogs by temporary ligation of the aorta. Prior to the ischemic episode, pentobarbital (30 to 45 mg per kilogram of body weight) was administered to 19 of these dogs. The neurological effects of cerebral ischemia episodes lasting 8, 9, or 10 minutes were compared in dogs treated with pentobarbital and those not treated. At 48 hours following the ischemic episode most of the dogs made ischemic for 8 minutes were normal, whereas most animals made ischemic for 10 minutes were dead or comatose. The 9‐minute ischemic period resulted in a relatively even distribution of normal and damaged dogs. There were no differences between treated and untreated dogs. Cerebral blood flow, cerebral metabolic rate for oxygen, and various cerebral metabolites were measured in dogs surviving 48 hours. Again, there were no differences between treated and untreated dogs. We conclude that barbiturates provide no protection in this model of complete global ischemia. This conclusion supports the hypothesis that the likely mechanism of barbiturate protection in models of incomplete ischemia or hypoxia is based on cerebral metabolic depression; such a mechanism would not be expected to be effective in complete global ischemia.
Diffuse cerebral ischemia in the cat: III. Neuropathological sequelae of severe ischemia
Authors: Ginsberg Myron D. , Graham David I. , Welsh Frank A. , Budd William W.
Published: January 2005

The neuropathological consequences of severe diffuse cerebral ischemia were investigated in an animal model in which postischemic alterations of regional brain blood flow and energy metabolism had been previously characterized. Pentobarbital‐aneshetized cats received either 15 or 30 minutes of ischemia produced by basilar artery and bilateral carotid artery occlusions plus mild hypotension; this was followed by 60 to 90 minutes of normotensive recirculation. The brains were perfusion‐fixed for light microscopy. Both insult durations resulted in unequivocal ischemic cell change affecting neurons of the cerebral neocortex, striatum, thalamus, and hippocampus and portions of the rostral brainstem. Animals with 30 minutes of prior ischemia differed from those with 15 minutes of ischemia in showing a more apparent regional accentuation of ischemic change in the parasagittal cortical gyri—the sites of previously documented focal postischemic heterogeneities of blood flow and metabolism. In other respects, however, the overall distribution and spectrum of severity of the ischemic alterations were similar for the two insult durations. These data support the view that significant permanent neuronal injury may result from a period of cerebral ischemia as brief as 15 minutes.
Regeneration of the magnocellular system of the rhesus monkey following hypothalamic lesions
Authors: Antunes J. Lobo , Carmel Peter W. , Zimmerman Earl A. , Ferin M.
Published: January 2005

The hypothalamic magnocellular system of the rhesus monkey was studied with specific immunocytochemical techniques in animals that had undergone hypothalamic lesions. The results indicate that this system maintains a regenerative capacity even when its tracts are interrupted within the hypothalamus. New neurohemal units are reconstituted from newly formed vessels within the scar as well as from preexistent blood vessels, such as perforating and pial arterioles, and the vessels of the pars tuberalis of the pituitary gland, which normally do not contain neurosecretory terminals.
The aicardi syndrome: Report of 4 cases and review of the literature
Authors: Bertoni John M. , von Loh Siegrun , Allen Richard J.
Published: January 2005

The findings in 4 cases of Aicardi syndrome (female infants with mental retardation, seizures, characteristic eye lesions, agenesis of the corpus callosum, vertebral anomalies, and abnormal but nondiagnostic electroencephalographic patterns) are compared to 39 previously reported cases. Available information suggests tha the syndrome results from an unknown intrauterine insult occurring no later than the first trimester. No familial cases have been reported, and the cause remains unknown.
Homozygous expression of a dominant gene for charcot‐marie‐tooth neuropathy
Authors: Killian James M. , Kloepfer H. Warner
Published: January 2005

A kindred of 68 French Acadians who were heterozygous for a dominant gene of Charcot‐Marie‐Tooth disease associated with peripheral nerve hypertrophy are described. Marriage between 2 heterozygotes resulted in 2 homozygous offspring. Clinical features of the homozygotes were similar to the classic description of Dejerine‐Sottas disease. Laboratory studies in this family revealed no chemical, metabolic, or chromosomal abnormalities in either the homozygotes or the heterozygotes.
Pupillary dysfunction in myasthenia gravis
Authors: Lepore Frederick E. , Sanborn George E. , Slevin John T.
Published: January 2005

The constriction‐dilation cycles of pupils exposed to a stationary, discrete slit‐lamp beam were significantly prolonged in 25 myasthenic patients (1,060.4 ± 45.8 msec) undergoing therapy with steroids, anticholinesterases, or both, compared to normal controls (801.9 ± 8.6 msec) or subjects receiving steroids for nonneurological disease (860.9 ± 14.9 msec). The duration of myasthenia correlated with the slowing of the cycle time. Myasthenia gravis may affect ectodermally derived smooth muscle or the autonomic neuromuscular junction or both, and not be restricted to the well‐demonstrated alterations of neuromuscular junction in striated muscle of mesodermal origin. Alternatively, prolonged pupillary cycles could be attributed to dysfunction of central pathways of the pupillary light reflex.
Biogenic amine metabolism in tourette syndrome
Authors: Butler Ian J. , Koslow Stephen H. , Seifert William E. , Caprioli Richard M. , Singer Harvey S.
Published: January 2005

Biogenic amine metabolism in the central nervous system of 9 children with Tourette syndrome was evaluated by quantitation of their metabolites in cerebrospinal fluid by a gas chromatographic/mass spectrometric method. Homovanillic acid (HVA), 5‐hydroxyindoleacetic acid (5‐HIAA), and 3‐methoxy‐4‐hydroxyphenylethylene glycol (MHPG) were measured in CSF before and after oral administration of probenecid. Dopamine metabolism appeared defective, as both baseline and accumulated levels of HVA after probenecid were decreased. Serotonin metabolism also appeared defective in some patients with low baseline and low accumulated levels of 5‐HIAA after probenecid. Taken together with other clinical features of this disease, the results suggest an underlying disorder of dopamine and serotonin metabolism in Tourette syndrome.
The cubital tunnel syndrome: Diagnosis and precise localization
Authors: Miller Robert G.
Published: January 2005

The cubital tunnel syndrome is a subgroup of ulnar neuropathies arising at the elbow, with nerve entrapment under the aponeurosis connecting the two heads of the flexor carpi ulnaris muscle. To separate this condition more clearly from tardy ulnar palsy, the clinical and electrophysiological features of 9 patients are presented, 6 of whom had the syndrome bilaterally. There was no history of trauma and no clinical or roentgenographic evidence of joint deformity in any of the patients.

In 9 of the 15 ulnar nerves, abnormal conduction was localized to the level of the cubital tunnel (1.5 to 3.5 cm distal to the medial epicondyle). The findings were confirmed intraoperatively in 7 patients and corresponded to a tight band compressing the ulnar nerve and causing narrowing at the cubital tunnel with swelling proximally. This syndrome represents a common and distinct subgroup of ulnar neuropathies at the elbow.
Ophthalmoplegia and bulbar palsy in variant form of maple syrup urine disease
Authors: Chhabria Shakuntala , Tomasi Lawrence G. , Wong Paul W. K.
Published: January 2005

This is the third reported case of a neonate with ophthalmoplegia in whom the diagnosis of maple syrup urine disease (MSUD) was delayed into infancy. In this case, the demonstration of significant residual branched‐chain ketoacid decarboxylase activities suggests that this atypical presentation may be characteristic of the MSUD variant form.
Lidase treatment of spinal cord transected rats
Authors: Kowalski Timothy F. , Lee Vahlsing H. , Feringa Earl R.
Published: January 2005

Russian investigators have recently reported clinical recovery of enzyme treated, spinal cord transected rats. Using the exact protocols outlined by Matinian and Andreasian, we repeated a portion of their experiment using a Lidase preparation manufactured in the USSR. Animals were evaluated for return of bladder function, clinical evidence of hind limb motor function, cortical evoked response after sciatic nerve stimulation, and axonal transport of cortically injected tritiated proline by regenerated corticospinal axons.

The only difference between treated and control animals was that the mean total body weight of the treated animals increased more than that of controls (p < 0.05). No animal walked, had return of voluntary motor activity, showed cortical evoked response, or had evidence for transport of tritiated proline over regenerated corticospinal axons.
Immunoglobulin class and light chain type of oligoclonal bands in CSF in multiple sclerosis determined by agarose gel electrophoresis and immunofixation
Authors: Link Hans , Assunta Laurenzi Maria
Published: January 2005

Agarose gel electrophoresis and immunofixation of CSF and serum from 39 patients with multiple sclerosis (MS) revealed oligoclonal IgG in the CSF in all cases and oligoclonal IgA and IgM in 1 patient each. IgG kappa bands only were found in 10 patients, while no patient had IgG lambda bands alone. IgG kappa bands predominated in 20 patients and IgG lambda bands in 5, while 4 patients had the same number of IgG kappa and IgG lambda bands. Twenty‐seven patients also displayed IgG bands with kappa and lambda present simultaneously. Bands of free lambda chains were found in 7 patients, while free kappa chain bands were not seen. One or 2 faint IgG bands in 4 patients constituted the only serum abnormality.

In 4 additional MS patients selected on the basis of normal findings on agarose gel electrophoresis of the CSF, immunofixation did not reveal oligoclonal lg, while isoelectric focusing showed bands in 1.

Immunofixation is recommended for proving the presence of oligoclonal Ig in CSF and for characterizing oligoclonal Ig into classes and types of light chains.
Bilateral rhinocerebral phycomycosis
Authors: Kasper Lloyd H. , Bernat James L. , Nordgren Richard E. , Reeves Alexander G.
Published: January 2005

A 57‐year‐old diabetic woman developed bilateral rhinoorbitocerebral phycomycosis. Successful treatment was accomplished by correction of the ketoacidosis, surgical debridement, and amphotericin B therapy. Rhinoorbitocerebral phycomycosis is a fulminant and frequently fatal disease most often seen in debilitated hosts. This survival suggests that aggressive therapy is indicated in patients with extensive fungal involvement.
Acute generalized polyneuropathy accompanying lithium poisoning
Authors: Brust John C. M. , Hammer Jeffrey S. , Challenor Yasoma , Healton Edward B. , Lesser Ronald P.
Published: January 2005

A woman with typical symptoms of lithium toxicity had, in addition, severe generalized sensorimotor peripheral neuropathy, Which cleared completely as she recovered. Electrodiagnostic studies suggested axonal lesions; autopsy ten months latter revealed no residual abnormalities. Previous studies have shown that lithium can affect peripheral nerve function, but this is the first reported case of peripheral neuropathy in association with lithium toxicity.
Paroxysmal choreoathetosis associated with thyrotoxicosis
Authors: Fischbeck Kenneth H. , Layzer Robert B.
Published: January 2005

Acquired paroxysmal choreoathetosis can be associated with a variety of structural and metabolic disorders. A patient is presented who had paroxysmal choreoathetosis associated with thyrotoxicosis and who responded to treatment of the underlying thyroid disease.
Dorsal thalamic lesion in a noted case of human memory dysfunction
Authors: Squire Larry R. , Moore Robert Y.
Published: January 2005

The extensively studies patient N. A. has had a severe verbal memory deficit since 1960, when he sustained a stab wound to the brain with a miniature fencing foil. His amnesia occurs in the absence of any other know cognitive defect. Recent CT scans have localized a lesion in the left dorsal thalamus of this patient in a position corresponding to the dorsomedial nucleus; there is no radiographic evidence of other damage in the diencephalon or cerebral cortex. The dorsomedial thalamus may be critical in the neuropathology of diencephalic amnesia and, in humans, may be required for normal functions.
Familial fatal parkinsonism with alveolar hypoventilation and mental depression
Authors: Purdy Allan , Hahn Angelika , Barnett Henry J. M. , Bratty Paul , Ahmad Dildar , Lloyd Kenneth G. , McGeer Edith G. , Perry Thomas L.
Published: January 2005

The clinical, pathological, and neurochemical characteristics of a newly recognized inherited neurological disorder are reported. Lethargy and mental depression are early symptoms, followed by mild parkinsonism and progressive weight loss. Failure of automatic respiratory control develops and may result in sudden death. Advanced degeneration of the substantia nigra, cell loss and gliosis of the basal ganglia, and focal gliosis in the medulla are seen on pathological study. Degeneration of the nigrostriatal dopaminergic system is evidenced by low levels of tyrosine hydroxylase, dopamine, homovanillic acid, and L‐dopa decarboxylase in postmortem brain samples. Taurine concentrations in fasting plasma and CSF are somewhat depressed; brain contents of taurine are within normal limits.
Editors Report
Authors: Plum Fred
Published: January 2005

No Abastract.
Central core disease and malignant hyperthermia syndrome
Authors: Frank John P. , Harati Yadollah , Butler Ian J. , Nelson Thomas E. , Scott Charles I.
Published: January 2005

In a detailed investigation of a family with musculoskeletal abnormalities obsesrved in four generations, the probad and his mother were found to have central cores and multicores on histochemical and electron microscopic studies of biopsied muscle. A male sibling experienced a malignant hyperthermic reaction during hand surgery, and although similar reactions had not been observed in other family members, unexplained ventricular dysrhythmias did occur in the proband during surgery. The proband and his mother were subsequently shown to be susceptible to malignant hyperthermia by abnormal in vitro muscle contractures in the presence of halothane or caffeine.

Physicians and dentists caring for patients with central core disease should be aware of its possible association with malignant hyperthermia. Complete evaluation pf patients with central core disease should include in vitro muscle contracture studies for malignant hyperthermia since many of these patients require surgery for musculo‐skeletal defects.
The carcinomatous neuromyopathy of oat cell lung cancer
Authors: Hawley Rollin J. , Cohen Martin H. , Saini Nirmal , Armbrustmacher Vernon W.
Published: January 2005

A prospective controlled clinical‐neurophysiological‐pathological study of 71 patients with oat cell carcinoma of the lung revealed no increased incidence of peripheral neuropathy at the initial stages of illness. All patients developed neuropathy by the time they had lost 15% of their body weight, but the neuropathy was less severe than in 20 age‐matched alcoholic patients with an equal degree of weight loss. The weight loss and peripheral neuropathy progressed with atrophy of type II (adenosine triphosphatase‐positive) muscle fibers out of proportion to the patient's loss of body weight. By 40% body weight loss, all the patients had moderate symmetrical peripheral neuropathy, 6 had proximal brachial or lumbosacral plexus metastases, and 9 had distal pressure palsies. Mononeuritis multiplex developed in only 1 patient, who had diabetes mellitus. Two patients developed Eaton‐Lambert syndrome, which resolved in 1 when chemotherapy controlled the systemic tumor, with no protein in the tumor postmortem which could produce the characteristic electromyographic findings of the syndrome.
Extramedullary hematopoiesis and spinal cord compression complicating polycythemia rubra vera
Authors: Rice G. P. A. , Assis L. J. P. , Barr R. M. , Ebers G. C.
Published: January 2005

A 68‐year‐old man withpolycythemia rubra vera developed gradually progressive paraparesis followed by an acute, painless paraplegia. Extradural thoracic spinal cord compression was found to be secondary to extramedullary hematopoiesis (EH). The neurological complications of EH are reviewed.
Management of hypoventilation in motor neuron disease presenting with respiratory insufficiency
Authors: Sivak Edward D. , Streib Erich W.
Published: January 2005

A 57‐year‐old man presented with a three‐month history of somnolence, hypoventilation, cor pulmonale, and episodes of apnea during sleep. Neurological studies revealed motor neuron disease, mainly confined to the muscles of respiration, that was causing respiratory insufficiency in the supine position. Nighttime treatment of respiratory insufficiency has permitted independent activity during wakefulness.
Local cerebral glucose utilization in newborn and pubescent monkeys during focal motor seizures
Authors: Kato Motohiro , Malamut Barbara L. , Caveness William F. , Hosokawa Shnichi , Wakisaka Shnichiro , O'Neill Raymond R.
Published: January 2005

The [14C]deoxyglucose method was used to determine the rate of local cerebral glucose utilization (LCGU) in newborn and pubescent monkeys during focal motor seizures induced by injecting penicillin into the face‐hand area of the right motro cortex. Seizures were studied in 3 newborn and 6 pubescent monkeys, and 3 newborn and 4 pubescent monkeys were used as controls. In controls, the pattern of glucose utiliztion within structures of the sensorimotor system was quite different at the two age levels; newborns showed far less activity, especially in the neocortex and striatum. In the monkeys with seizures, the unilateral increase in LCGU relative tot he controls was greater in newborn than in pubescent monkeys except in the cerebral and cereballar cortices. The increased glucose utilization in cortical and subcortical structures of the newborn was ipsilateral to the dischargin lesion and lacked the well‐defined pattern seen in the pubescetn mmonkeys. In general, newborn brain was capable of supporting a focal motro seizure but lacked the precise clinical and electrographic expressions or efficient energy metabolism that accopany maturation of the brain at puberty.
Propagation of focal motor seizures in the pubescent monkey
Authors: Caveness William F. , Kato Mothohiro , Malamut Barbara L. , Hosokawa Shnichi , Wakisaka Shinichiro , O'Neill Raymond R.
Published: January 2005

The rate of local cerebral glucose utilization was determined for quantification of energy metabolism in macrostructures of the sensorimotor system during propagation of focal motor seizures in 24‐month‐old monkeys. The rate was measured in 4 control animals and in 4 monkeys each with seizures limited to the contralateral face, seizures of the contralateral face and upper extremity, and bilateral expression of seizures. Glucose utilization increased significantly, primarily unilaterally, with propagation. The increase was greatest in the sensory and motor cerebral cortices, putamen, and globus pallidus, was somewhat less in sensory and motor thalamic relay nuclei, and was least in the cerebellar cortex.
Spinal evoked potential in the monkey
Authors: Feldman Martin H. , Cracco Roger Q. , Farmer Peter , Mount Fred
Published: January 2005

Computer‐averaged evoked potential responses (EPs) to stimulation of the sciatic nerve and cervical spinal cord were recorded from the dura and skin over the cauda equina and spinal cord in seven monkeys, three with chronic spinal cord lesions.

Sciatic EPs consisted of predominantly negative triphasic propagated potentials recorded at all spinal levels and greatest in amplitude over the cauda equina and caudal spinal cord. The conduction velocity of this EP was faster over the cauda equina and rostral spinal cord than over caudal cord segments. Triphasic potentials were succeeded by small negative potentials over the cauda equina and larger negative potentials over the lumbar enlargement. Sciatic EPs over the upper lumbar and thoracic cord were more sensitive to asphyxia than the initial triphasic potentials recorded over cauda equina and caudal cord but resisted changes from increasing the rate of stimulation up to 100 per second. Propagated thoracic EPs were preceded by nonpropagated potentials. The longer latency negative potentials occurring locally over the cauda equina and lower lumbar enlargement were abolished at levels of asphyxia and were attenuated at rates of stimulation that did not affect the preceding triphasic potentials.

Following complete spinal cord transection, nonpropagated sciatic EPs were recorded in leads rostral to the section. In preparations with chronic partial cord hemisection involving dorsal and lateral quadrants, ipsilateral sciatic EPs had increased latency, reduced amplitude, and poor definition in the vicinity of and rostral to the lesion.

Direct cervical cord stimulation elicited caudally propagated potentials which were followed by large, broad potentials over the lumbar enlargement.
Optokinetic nystagmus and parietal lobe lesions
Authors: Baloh Robert W. , Yee Robert D. , Honrubia Vicente
Published: January 2005

In an attempt to define better the mechanism of impaired optokinetic nystagmus (OKN) caused by parietal lobe lesions, we recorded the eye movements of two patients. One had a slowly enlarging parietal glioma, and the other, an infarction involving the parietal and occipital lobes. In both patients, ipsilateral foveal pursuit and full‐field pursuit of a surrounding optokinetic drum were impaired while voluntary and involuntary saccades (fast components) were normal. Foveal pursuit was impaired more than full‐field pursuit, suggesting the existence of two separate pathways by which visual signals for OKN slow phases reach the oculomotor centers in the brainstem. We conclude that the ipsilateral slow phase OKN deficit seen in out patients resulted from damage to the foveal pursuit pathway that runs from the occipitoparietal association area to the ipsilateral brainstem horizontal gaze center.
Computerized densitometry and color coding of [14C] deoxyglucose autoradiographs
Authors: Goochee Charles , Rasband Wayne , Sokoloff Louis
Published: January 2005

A computerized image processing system has been developed for quantitative analyses of autoradiographs obtained with the [14C] deoxyglucose method. The system is composed of standard, commercially available components and includes a scanning microdensitometer, computer, image memory and display system, and monochrome and color monitors. The associated computer programs are written in PASCAL. Autoradiographs are automatically scanned, and the optical density of each spot is digitized at a maximum resolution of 65,536 readings per 6.4 X 6.4 mm area and stored in memory. Images can be reconstructed from the data in memory, displayed on the monitors, and utilized for microdensitometric analyses or manipulated for image enhancement, enlargement, or weighted averaging of selected regions. The digitized data can also be utilized to solve the operational equation of the [14C] deoxyglucose method, and color‐coded images of autoradiographs can be reconstructed so that each color represents a narrow range of the rate of glucose utilization. By means of this system, it is possible to generate quantitative metabolic maps that display the distribution of actual rates of local glucose utilization throughout the entire central nervous system in regions as small as 100 μm or less.
Dyslexia: Automated diagnosis by computerized classification of brain electrical activity
Authors: Duffy Frank H. , Denckla Martha B. , Bartels Peter H. , Sandini Giulio , Kiessling Louise S.
Published: January 2005

We describe a method for the diagnosis of dyslexia based upon a study of electronecephalographic and evoked potential data recoreded from 13 normal and 11 dyslexic boys. Measuremkents were made from topographic maps of brain electrical activity recorded during resting and activated testing conditions. Using a statistically based technique, we developed rules for classification that successfully diagnosed 80 to 90% of subjects not used in the initial rule development. The nature of the most useful measurements suggests that aberrant neurophysiology in dyslexia involves both hemispheres and is present at rest as well as during complex testing. The method has promise for future diagnosis and research.
Sleep apnea, narcolepsy, and dreaming: Regional cerebral hemodynamics
Authors: Meyer John Stirling , Sakai Fumihiko , Karacan Ismet , Derman Sabri , Yamamoto Masahiro
Published: January 2005

Regional cerebral blood flow after inhalation of xenon 133 as well as polysomnography were recorded during daytime sleep and the awake state in patients with narcolepsy and sleep apnea. Brainstem‐cerebellar (BSC) gray matter blood flow (Fg) values in the awake state were r educed below normal (p < 0.05) in both narcolepsy and sleep apnea; in sleep apnea, bihemispheric Fg values were also reduced in the awake state. After sleep onset, Fg paradoxically increased in narcolepsy but decreased further in sleep apnea. Maximal regional Fg changes occurred in BSC regions in both groups of patients. Oral administration of methylphenidate hydrochloride (Ritalin) increased resting Fg values in awake narcoleptics, particularly in BSC regions, but attenuated Fg increases during sleep onset. Regional Fg values during visual dreaming or hypnagogic hallucinations in narcoleptics were maximally increased in right parietooccipital regions.

In narcoleptics, impaired control of sleep‐wake and REM mechanisms is attenuated by methylphenidate. In patients with sleep apnea, brainstem functional activity is low in the awake state but becomes critically reduced during sleep, culminating in apnea‐stimulated arousal followed by repetitive cycles as sleep recurs.
Children's language disorders: Recent research advances
Authors: Ludlow Christy L.
Published: January 2005

Between 3 and 8% of preschool children in Britain and the United States are delayed in their language development by more than a year below the normal range. Evidence suggests that such children are Particularly impaired in acquiring syntactic and morphological rules, although well‐defined syndromes of impaired language development have not yet been determined. The effects of preschool impairments in language development on subsequent school achievement are significant: 60% of these children are in special classes for the learning impaired at 9 years of age. Auditory processing deficits have been demonstrated in language‐impaired children, but the contributions of such deficits to language development difficulties have yet to be determined. While autistic children have severe cognitive disorders and visual and auditory perceptual disturbances, the difficulties of nonautistic languageimpaired children seem confined more to language expression and comprehension. Recent neuropsychological evidence concerning brain organization for language behavior suggests that these children have deficiencies in language function which normally depend on left hemisphere functioning.
Mechanisms of increased brain glucose and glycogen after hydrocortisone: Possible clinical significance
Authors: Thurston Jean Holowach , Hauhart Richard E. , Dirgo John A. , Jones Elizabeth M.
Published: January 2005

We reported previously that chronic administration of hydrocortisone to normal developing mice increases the brain glucose content and cerebral energy reserve. The present report concerns possible mechanisms of this action. Increases in brain glucose (and glycogen) levels were not due to reduction of cerebral metabolic rate, and the effect of hydrocortisone in facilitating transport of hexose from blood to brain was not impressive. Chronic hydrocortisone treatment induced increases in the activities of brain glycerophosphate dehydrogenase and pyruvate carboxylase in vivo; there was no effect on eleven other enzymes of brain glucose and glycogen metabolism. In normal nursing mice, hydrocortisone produced consistent elevations in plasma β‐hydroxybutyrate (and glycerol) levels. Brain β‐hydroxybutyrate levels were also increased. Therefore, the brain glucose concentration may be elevated in these animals because of the availability of an increased supply of ketone bodies as alternative substrates for cerebral oxidative metabolism and biosynthesis. Ketonemia, elevated cerebral glucose and β‐hydroxybutyrate concentrations, and increased glycerophosphate dehydrogenase activity in brain suggest possible explanations for the beneficial action of adrenocorticotropic hormone and glucocorticoids in the treatment of infantile myoclonic epilepsy and other neurological disorders.
Radation therapy for brain metastases
Authors: Cairncross J. Gregory , Kim Jae‐Ho , Posner Jerome B.
Published: January 2005

We treated 183 patients who had brain metastases with whole‐brain radiation therapy (RT) and adrenocortico‐ steroids utilizing a new high‐dose radiation protocol. Treatment produced neurological improvement in 135 patients (74%); there was no change in 37 (20%), and deterioration occurred i n l l (6%). Of those patients who improved, two‐thirds maintained neurological improvement for the remainder of their lives or for at least nine months, and one‐third relapsed. One‐third of those who relapsed improved again with steroids or further RT. Clinical improvement paralleled tumor regression on CT scan. Despite the clinical response, median survival was only twelve weeks; 24% lived six months and 8% lived one year. Death resulted from progressive brain disease in 16% of the patients. Two‐thirds of the patients died of advancing systemic disease in the setting of stable, improved neurological function. These data suggest that the majority of patients with brain metastases benefit from RT and that systemic cancer, not central nervous system disease, limits the length of life.
Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts
Authors: Moser Hugo W. , Moser Ann B. , Kawamura Nariko , Murphy Jerome , Suzuki Kunihiko , Schaumburg Herbert , Kishimoto Yasuo
Published: January 2005

Because postmortem brain and adrenal tissue from patients with adrenoleukodystrophy (ALD) or adrenomyeloneuropathy (AMN) have been shown to contain abnormally large amounts of very long chain fatty acids (C24 through C30), we searched for such an abnormality in cultured skin fibroblasts. Total lipid extracts of cultured fibroblasts were hydrolyzed, their fatty acid composition was derermined by gas‐liquid chromatography, and the ratio of C26 to C22 fatty acids was calculated.

In 29 control cell lines this ratio was 0.064 ± 0.019. In 5 patients with autopsy‐proved ALD the ratio was 0.778 ± 0.139; in 6 patients with clinical features typical of ALD it was 0.764 ± 0.092; in 2 patients with autopsy‐proved AMN, 0.890 ± 0.02; and in 2 patients with clinical features typical of AMN, 0.560 ± 0.079. Abnormal ratios were observed in 4 of 5 ALD heterozygotes. In 3 patients in whom the diagnosis of ALD was suspected, an abnormal ratio (0.860) was observed in 1 and normal ratios (0.06 and 0.074) in the 2 others.
Valproic acid and ethosuximide interaction
Authors: Mattson Richard H. , Cramer Joyce A.
Published: January 2005

The addition of valproic acid to ethosuximide in treatment to prevent seizures caused increased serum concentration of ethosuximide in four of five patients observed. Ethosuximide levels increased from 73 to 112 m̈g/ml (53% higher), with concomitant toxicity. Both were reversed by reduction of the ethosuximide dose from 27.4 to 20.4 mg/kg. Serum concentrations of these two drugs should be monitored closely when they are given together.
Interaction of multiple sclerosis serum with liposomes containing ganglioside GM1
Authors: Mullin Brian R. , Montanaro Anthony J. , Reid J. D. , Nishimura Robert N.
Published: January 2005

Multiple sclerosis serum contains a factor or factors capable of releasing sequestered [14C]glucose from liposomes containing GM1, the major ganglioside present in human central nervous system myelin. Although some control sera can also release large quantities of liposomal glucose, multiple sclerosis and control sera differ in the mechanism of this release. Liposomal damage by multiple sclerosis serum does not appear to be complement mediated, but shares certain releasing characteristics of cholera toxin.
Antiserum‐mediated demyelination: Relationship between remyelination and functional recovery
Authors: Saida Kyoko , Sumner Austin J. , Saida Takahiko , Brown Mark J. , Silberberg Donald H.
Published: January 2005

A focal demyelinative lesion of peripheral nerve was produced by intraneural injection of either antiserum from rabbits with experimental allergic neuritis or experimental allergic encephalomyelitis or antiserum to galactocerebroside. We studied the relationship between clinical and electrophysiological recovery from this lesion and the morphological pattern of remyelination. Foot muscles on the injected side weakened within an hour of injection and remained paralyzed for 7 days; strength gradually returned to normal by 16 days after injection. Electrophysiological conduction block, apparent within a few hours of injection, persisted for about 7 days. At 8 days we detected dispersed, very low amplitude muscle action potentials with long latency. Morphologically, demyelinated axons were surrounded by Schwann cells at 7 days after injection, but compacted myelin was not present. After 8 days, remyelinating axons became surrounded by thickening compacted myelin. The time of onset of remyelination and the rate of remyelination up to 14 days following the injection were independent of axon size. The onset of clinical and electrophysiological recovery from the lesion corresponded to the appearance of 2 to 8 myelin lamellae around each remyelinating axon. At 37 days after injection, when conduction velocities had returned to preinjection values, myelin thickness of remyelinating fibers had increased to approximately one‐third that of control nerves.
Effects of stroke on local cerebral metabolism and perfusion: Mapping by emission computed tomography of 18FDG and 13NH3
Authors: Kuhl David E. , Phelps Michael E. , Kowell Arthur P. , Metter E. Jeffrey , Selin Carl , Winter James
Published: January 2005

By means of emission computed tomography (ECT), we used 18F‐fluorodeoxyglucose (18FDG) and 13N‐ammonia (13NH3) as indicators of abnormalities in local cerebral glucose utilization (LCMRglc) and relative perfusion, respectively. The ECAT positron tomograph was used to scan normal control subjects and 10 stroke patients at various times during recovery. In normal subjects, mean CMRglc was 5.28 ± 0.76 mg per 100 gm tissue per minute (mean ± SD; N = 8). In patients with stroke, mean CMRglc in the contralateral hemisphere was moderately decreased during the first week, profoundly depressed in irreversible coma, and normal after clinical recovery.

Quantification was restricted by incomplete understanding of tracer behavior in diseased brain, but relative local distributions of 18FDG and 13NH3 trapping qualitatively reflected the increases and decreases as well as coupling and uncoupling expected for local alterations in glucose utilization and perfusion in stroke. Early after cerebrovascular occlusion there was a greater decrease in local trapping of 13NH3 than 18FDG within the infarct, probably because of increased anaerobic glycolysis. Otherwise, 18FDG was a more sensitive indicator of cerebral dysfunction than was 13NH3. Hypometabolism, due to deactivation or minimal damage, was demonstrated with the 18FDG scan in deep structures and broad zones of cerebral cortex that appeared normal on x‐ray computed tomography and technetium 99m pertechnetate scans.

In its present state of development, the 18FDG ECT method should aid in defining the location and extent of altered brain in studies of disordered function after stroke. With improved knowledge of tracer behavior in diseased brain, the method has promise for mapping the response to therapeutic intervention and increasing our understanding of how the human brain responds to stroke.
Blindness following tentorial herniation
Authors: Keane James R.
Published: January 2005

In seven patients, permanent bilateral visual loss was the chief neurological residual following relief of tentorial herniation. The causes of herniation were unilateral subdural hematoma in three patients and bilateral subdural hematomas, bilateral subdural empyemas, traumatic intracerebral hematoma, and postoperative infarction in one patient each. Three patients, including two with occipital infarction documented by computed tomographic scan, developed optic atrophy, indicating that both pregeniculate visual pathway damage and posterior cerebral artery compression occurred.

Increasing availability of computed tomographic scanning should help eliminate this rare cause of blindness by facilitating earlier diagnosis of supratentorial masses.
Multiple sclerosis in a migrant population: 2. Half‐orientals immigrating in childhood
Authors: Kurtzke John F. , Bui‐Quoc‐Huong
Published: January 2005

We studied offspring of Vietnamese mothers and French fathers who had been born in Vietnam and brought to France to be raised. All but 72 of 3,451 such individuals completed background and neurological questionnaires. Those with positive answers on the latter were examined neurologically. The median year of birth for the 3,379 respondents was 1948, the median year of arrival in France was 1957, and they had resided in France a median of 18 years before interview in 1975. Twenty‐five persons had neurological symptoms, and 6 others reported a history of seizures alone. A total of 16 reported seizures: 7 with infantile febrile fits and 9 with afebrile seizure disorders.

Three cases of exacerbating‐remitting multiple sclerosis (MS) were discovered as well as 3 persons in whom MS was suspected: 1 with cervical myelopathy, 1 with progressive paraparesis, and 1 with bilateral optic neuropathy. The 3 MS cases provided a crude prevalence rate (or 18‐year cumulative risk) of 89 per 100,000. Their age‐specific prevalence rate was 169 per 100,000 population aged 20 through 29 years. Each measure was similar to such rates for Denmark and had lower 95% confidence limits that clearly exceeded expectations for Vietnam. The results provide further evidence that the risk of MS can be increased by migration in childhood from low‐ to high‐risk regions.
Diminished epidermal growth factor binding by neurofibromatosis fibroblasts
Authors: Zelkowitz Marvin , Stambouly Joseph
Published: January 2005

Neurofibromatosis (NF) is an autosomal dominant disease characterized by growth abnormalities of epithelial, mesothelial, and endothelial elements. We recently reported abnormal growth and morphology of NF fibroblasts in tissue culture. Because epidermal growth factor (EGF) is known to stimulate the growth of fibroblasts in tissue culture, we studied the binding of commercial iodine 125‐labeled EGF to age‐ and passage‐matched confluent NF (N = 6) and normal (N = 4) fibroblasts. Fibroblasts were maintained at 37°C for 2, 30, 60, 120, and 240 minutes in a medium in which the cells grow slowly (Dulbecco's Eagle medium) and one in which they grow normally (Ham's F‐12 medium). Binding assays were done in both serum‐free media according to accepted procedures. The EGF binding did not differ in the two media, and pooled data are presented. These data demonstrate no significant differences in the early binding of EGF to normal and NF fibroblasts (4,682 ± 1,092 versus 3,441 ± 826 cpm/106 cells; 20,000 cpm/ng; p > 0.15 at 30 minutes). At one hour, however, differences suggestive of abnormal EGF binding become apparent (12,495 ± 1,989 versus 3,172 ± 853 cpm/106 cells; 20,000 cpm/ng; p < 0.0025). We conclude that there may be a membrane defect in NF which is reflected by diminished EGF binding.
Torsion dystonia in israel
Authors: Korczyn Amos D. , Kahana Esther , Zilber Nelly , Streifler Max , Carasso Raphael , Alter Milton
Published: January 2005

A country‐wide search for idiopathic torsion dystonia (ITD) in Israel between 1969 and 1975 revealed 42 patients (41 Jewish and 1 Druze Arab). Prevalence of ITD per million population, age‐adjusted to the United States population in 1970, was 10.8 in the total Jewish population (22.0 among Jews of European extraction contrasted with 1.5 among Jews with Afro‐Asian forebears). Among Europeans, the highest prevalence was among Jews from Eastern Europe.

The average age‐adjusted annual incidence rates per million population were 0.43 in the total Jewish population, 0.98 in the Europeans, and 0.11 in the Afro‐Asians. Among the 40 patients for whom familial data were available, the majority of cases (26) were sporadic. The other 14 belonged to four unrelated European families, all of Russian‐Polish origin. The pattern of inheritance in these four families fits an autosomal dominant model with incomplete penetrance.
Periodic movements in sleep (nocturnal myoclonus): Relation to sleep disorders
Authors: Coleman Phd Richard M. , Pollak Charles P. , Weitzman Elliot D.
Published: January 2005

Periodic movements in sleep (PMS) are stereotyped, repetitive, nonepileptiform movements of the lower extremities. A total of 409 sleep disorder patients were studied with all‐night polysomnogram recording, and 53 (13%) had PMS. Such movements occurred in a wide variety of sleep‐wake disorders in addition to insomnia. The prevalence and magnitude of PMS were not statistically greater in patients with insomniac disorders than in those with syndromes of excessive daytime sleepiness or other sleep‐wake disorders. The results suggest that although PMS is responsible for disturbed sleep in relatively few patients, chronic sleep‐wake disturbance is associated with PMS and may lead to the development of these movements.