bluuebunny/crossref_metadata_2025 huggingface dataset:
Brain death: I. Angiographic correlation with the radioisotopic bolus technique for evaluation of critical deficit of cerebral blood flow
Authors: Korein Julius , Braunstein Philip , George Ajax , Wichter Melvin , Kricheff Irvin , Lieberman Abraham , Pearson John
Published: December 2004

An innocuous intravenous portable radioisotopic test using technetium 99m pertechnetate was employed to demonstrate the deficit of cerebral blood flow associated with brain death. The results are compared to those of bilateral carotid and vertebral angiography in 20 patients. Absence of a bolus tracing from the head in the presence of a control tracing of a bolus from the femoral artery in two successive studies one hour apart reliably correlated with the clinical and electroencephalographic findings signifying cerebal death in comatose, apneic patients. Angiography indicated absence of intracranial circulation in 10 patients. Stasis filling or retrograde emptying of arterial vessels (or both) occurred in 7 patients. There was no evidence of venous filling in any of these 17 patients; all of them had either an absent head bolus or an “intermediate tracing.” Results indicate that either form of tracing represents a critical decrease of cerebral blood flow. Two other patients had evidence of severely impaired abnormal posterior fossa circulation without angiographic evidence of cerebral circulation; both of these patients had an absent head bolus. An additional patient had an unusually small head bolus, and angiography revealed extravasation of radiopaque material but no evidence of intracranial circulation. We conclude that the bolus technique is a helpful adjunct in diagnosing brain death.
Biochemical correlates of illness and recovery in Reye's syndrome
Authors: Trauner Doris , Sweetman Lawrence , Holm Janette , Kulovich Stanko , Nyhan William L.
Published: December 2004

Eight patients with Reye's syndrome were followed through the course of their disease with serial measurements of serum concentrations of ammonia, short‐chain organic acids, and amino acids. Correlations were made between clinical status and biochemical alterations. Elevated short‐chain fatty acids, hyperammonemia, and hyperaminoacidemia were found in all patients studied. Clinical improvement appeared to correlate most closely with clearance of short‐chain fatty acids from the serum. These observations suggest that the clinical symptoms are at least in part related to organic acidemia and that treatment directed toward the rapid clearance of these compounds from the system is reasonable.
Effect of tranexamic acid on rebleeding after subarachnoid hemorrhage: A double‐blind controlled clinical trial
Authors: van Rossum J. , Wintzen A. R. , Endtz L. J. , Schoen J. H. R. , de Jonge H.
Published: December 2004

In a double‐blind controlled clinical trial on 51 patients with subarachnoid hemorrhage, tranexamic acid, 4 gm per day for ten consecutive days, did not favorably affect the outcome. Neither mortality nor rebleeding rates were improved after a follow‐up of three months.
Chronic hydrocephalus associated with short stature and growth hormone deficiency
Authors: Hier Daniel B. , Wiehl Albert C.
Published: December 2004

A 28‐year‐old man with chronic hydrocephalus due to aqueductal stenosis was found to have a subnormal response of growth hormone to arginine infusion. It is postulated that compression of the pituitary by the enlarged third ventricle produced a deficiency of growth hormone and short stature.
Therapeutic trials in muscular dystrophy: II. Ethylene and its derivatives
Authors: Enomoto Akira , Bradley Walter G.
Published: December 2004

The plant growth stimulants ethylene dinitramine (EDNA) and ethylene have been reported to increase muscle regeneration. Such chemicals might therefore be of therapeutic benefit in the human muscular dystrphies. This report describes a series of experiments to reinvestigate the effects of EDNA, ethylene, and another plant‐stimulating ethylene derivative, 2‐chloroethylenephosphoric acid (Ethephon). EDNA and Ethephon administered two days after wounding both produced a significant increase of muscle protein synthesis seven days after the injury of normal muscle, though the increases were only 35 and 22%, respectively. The results suggested that early suppression of regeneration might explain the later increases. Ethylene administered in an identical way to that previously reported failed to increase muscle protein synthesis in murine muscular dystrophy or in wounded normal muscle. These studies suggest that treatment of human muscular dystrophies with ethylene derivatives is unlikely to prove of value.
Huntington's chorea: Selective depletion of activity of angiotensin converting enzyme in the corpus striatum
Authors: Arregui Alberto , Bennett James P. , Bird Edward D. , Yamamura Henry I. , Iversen Leslie L. , Snyder Solomon H.
Published: December 2004

The activity of angiotensin converting enzyme, which transforms the relatively inactive decapeptide angiotensin I to the active octapeptide angiotensin II by removal of an L‐histidyl‐L‐leucine residue, has been assayed in numerous regions of the calf brain and of the brains of humans with Huntington's chorea and controls. In calf brain there are pronounced regional variations in enzyme activity, with highest activity in the globus pallidus and area postrema. In human brain, enzyme activity is highest in the corpus striatum, with similar levels in the caudate, putamen, and globus pallidus. Converting enzyme activity is reduced by 83 to 92% in the globus pallidus in Huntington's chorea. The caudate and putamen of choreic patients display 62 to 69% reductions in enzyme activity. Converting enzyme activity in two cerebral cortical regions from choreic brains is not significantly different from control.
Inhibitors of GABA metabolism: Implications for Huntington's disease
Authors: Schwarcz Robert , Bennett James P. , Coyle Joseph T.
Published: December 2004

Stereotaxic injection of kainic acid into rat striatum produces neuronal degeneration and neurochemical alterations resembling Huntington's disease (HD). Since correction of the deficiency in γ‐aminobutyric acid (GABA) in HD may be of therapeutic value, the efficacy of drugs that inhibit the GABA‐degrading enzyme (GABA‐T) in elevating the levels of GABA has been examined in the animal model for HD. High doses of di‐N‐propylacetic acid only partially correct the GABA deficiency in the striatum, whereas low doses of γ‐acetylenic GABA, a specific irreversible inhibitor of GABA‐T, completely restore the levels of GABA in striatum and substantia nigra.
Regeneration of corticospinal axons in the rat
Authors: Feringa Earl R. , Shuer Lawrence M. , Lee Vahlsing H. , Davis Sam W.
Published: December 2004

In the rat, a few long descending motor tracts capable of carrying an impulse and causing a propagated impulse in the ipsilateral sciatic nerve will regenerate after complete spinal cord transection. In this experiment such regeneration was found in both treated and control animals.

Orthograde axonal transport of tritiated proline injected into the motor cortex labels only the corticospinal tracts in the rat spinal cord. Scintillation counts of measured lengths of spinal cord can be used as a measure of the number of labeled corticospinal axons.

Comparison of radioactivity per unit length of measured cord segments taken from above and below the site of a previous spinal cord transection can give a reliable estimate of the number of labeled axons that regenerated and crossed the site of injury. Using this test we have demonstrated that some corticospinal axons had regenerated six months after spinlal cord transection in control animals, animals made tolerant to degenerating spinal cord antigens, and animals treated with cyclophosphamide. A group treated with a single 75 mg per kilogram dose of cyclophosphamide 24 hours after spinal cord transection showed the best evidence of corticospinal tract regeneration.
Epileptogenic mineralization: Pathological variants with good prognosis
Authors: Averback P.
Published: December 2004

Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic “brain stone.” Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy.
Inferior branch palsy of the oculomotor nerve
Authors: Susac John O. , Hoyt William F.
Published: December 2004

Three patients with inferior branch palsies of the oculomotor nerve are described. Two were under 10 years of age and the third was 30 years old at the onset. All 3 presented with painless diplopia. The onset was sudden in 2 patients and progressive in the third. The palsy cleared within a short time in the 2 patients with sudden onset, and a possible viral origin was considered. In the third patient, with progressive palsy, there was no change eight years later.
δ‐Aminolevulinic acid: Influences on synaptic GABA receptor binding may explain CNS symptoms of porphyria
Authors: Müller Walter E. , Snyder Solomon H.
Published: December 2004

Symptoms of acute porphyria have been attributed to effects of δ‐aminolevulinic acid (ALA). We report that ALA selectively competes for the binding of tritiated γ‐aminobutyric acid ([3H]GABA) associated with synaptic GABA receptors in central nervous system membranes. Concentrations of ALA that inhibit GABA receptor binding are consistent with levels of ALA thought to exist in the central nervous system of porphyric patients. Some of the symptoms of acute porphyria resemble those elicited by muscimol, a potent GABA agonist drug. Barbiturates, which exacerbate porphyric symptoms, are potent facilitators of the synaptic actions of GABA. The results suggest that some symptoms of acute porphyria might be attributable to a mimicking by ALA of GABA at its central nervous system receptor sites.
Perinatal risk factors in children with serious motor and mental handicaps
Authors: Nelson Karin B. , Broman Sarah H.
Published: December 2004

Fifty children with marked neurological abnormality manifested by moderate or severe motor disability and severe mental retardation were compared with a large control population with respect to prospectively ascertained perinatal characteristics. None of 60 prenatal factors distinguished the affected group from controls. In labor and delivery, lowest fetal heart rate in the second stage of labor, arrested progress of labor, and use of midforceps discriminated between the two groups. Neonatal characteristics of children who were later severely handicapped differed from controls, particularly with respect to difficulty in initiating and maintaining respiration, intracranial hemorrhage, neonatal seizures, low birth weight and small head circumference, lowest hemoglobin or hematocrit, and overall neurological status.

Multivariate analysis, including factors from all epochs, indicated that intracranial hemorrhage and neonatal seizures were the strongest independent discriminators between the neurologically impaired children and controls.
Neurological complications of infantile osteopetrosis
Authors: Lehman Ralph A. W. , Reeves Jerry D. , Wilson W. Bruce , Wesenberg Richard L.
Published: December 2004

Seven cases of infantile osteopetrosis are presented. Five of these were available for detailed clinical examination and 2 for retrospective review, including autopsy slides. Neurological deficits in these patients are reviewed. Involvement of the central nervous system parenchyma was suggested by observations of delayed development, ocular abnormalities, and reflex changes as well as radiographic and autopsy findings. Cerebral atrophy was present in several of our patients as well as some reported in the literature and may account for the ventricular enlargement found in many of these patients. Though hydrocephalus may be present, it is unclear that this is frequent or that it can occur without antecedent intracranial hemorrhage. The large head size is not accounted for by calvarial thickening or by hydrocephalus. Despite our patients' small stature, pituitary function appeared to be normal. Surgical decompression may stabilize cranial nerve function, particularly when the optic nerves are involved.
“Disappearing” spinal cord compression: Oncolytic effect of glucocorticoids (and other chemotherapeutic agents) on epidural metastases
Authors: Posner Jerome B. , Howieson John , Cvitkovic Esteban
Published: December 2004

Four patients suffering spinal cord compression resulting from epidural metastases were treated with adrenocorticosteroid hormones. For 2 patients, prompt relief of symptoms followed glucocorticoid therapy alone and was associated with marked shrinkage or disappearance of the metastasis, a direct oncolytic effect of the steroids. For the other 2 patients, glucocorticoids combined with other chemotherapeutic agents caused disappearance of the extradural tumor. For 1 patient, failure to recognize the oncolytic effects of the chemotherapy led to an unnecessary surgical procedure, and for a second patient an unnecessary operation was narrowly averted. Thus, for certain patients, glucocorticoids may occasionally have a marked oncolytic effect on epidural metastatic tumors.
X‐Linked muscular dystrophy
Authors: Furukawa Tetsuo , Peter James B.
Published: December 2004

X‐linked muscular dystrophy has been separated into two types that are generally considered to be distinct entities. We have investigated three families with X‐linked muscular dystrophy who demonstrate remarkable intrafamilial variability. In one family 2 brothers with a benign type had a maternal uncle who was affected with the Duchenne type. In another family, 4 members had the benign type but a fifth was much more severely affected than in the classic Duchenne dystrophy; therefore the presence of an “aggressive form” is proposed. A third family also had both benign and severe types. A search of the literature revealed families in which the severe and benign types coexisted.

The genetic determinants of this heterogeneity are not yet known. Clinical similarities between bening and severe types of X‐linked muscular dystrophy and the presence of families with both types suggest that the two are intimately related.
“Release hallucinations” as the major symptom of posterior cerebral artery occlusion: A report of 2 cases
Authors: Brust John C. M. , Behrens Myles M.
Published: December 2004

Visual hallucinations were the presenting symptoms in 2 patients with probable infarcts in the territory of a posterior cerebral artery. They occurred in areas of paracentral scotomas, right in Patient 1 and left in Patient 2. In Patient 1 they were formed, prolonged, and not apparently related to past experience. In Patient 2 they were at first paroxysmal and unformed, with more prolonged metamorphopsia; later there appeared to be palinoptic formed images, possibly postictal in nature. Such hallucinations appear to be of a “release” type, and may be more common than is generally appreciated in patients with posterior cerebral artery occlusion.
Congenital arachnoid cyst with unusual clinical, radiological, and pathological findings
Authors: Rosich‐Pla Antonio , Smith Bernard H. , Sil Ranajit
Published: December 2004

Choroid plexus–like structures were found in the wall of a congenital frontotemporal arachnoid cyst in a 14‐year‐old girl who presented with temporal lobe seizures since the age of 6. The temporal cortex beneath the cyst was maldeveloped. The clinical, radiological, and pathological aspects of congenital arachnoid cysts are reviewed.
Paroxysmal choreoathetosis following head injury
Authors: Robin Joseph J.
Published: December 2004

A 33‐year‐old man developed paroxysmal choreoathetosis following a severe head injury. Phenobarbital therapy was promptly effective. The family history as well as physical examinations and laboratory studies were unremarkable.
The influence of route of delivery and hyaline membranes on the risk of neonatal intracranial hemorrhages
Authors: Leviton Alan , Gilles Floyd , Strassfeld Ruth
Published: December 2004

The relationship of route of delivery and hyaline membranes to eight sites of intracranial hemorrhage was evaluated in 513 autopsied newborn infants. Those delivered by cesarian section appeared to be at less risk for most hemorrhages compared with newborns delivered vaginally. However, the reductions in risk were small and did not achieve nominal levels of statistical significance. Thus, “birth trauma” probably contributes little to the risk of most neonatal intracranial hemorrhages. Newborns who developed hyaline membrances appeared to be at greater risk for most hemorrhages than those who did not, particularly if the infant had been delivered by cesarian section. This was especially marked for subarachnoid and germinal matrix hemorrhage.
Idiopathic regressing arteriopathy
Authors: Mokri Bahram , Houser O. Wayne , Sundt Thoralf M.
Published: December 2004

Three patients had nonatheromatous occlusive cervicocephalic arterial disease which on follow‐up showed resolution of the occlusive or stenosing abnormalities. The patients' relatively young age (middle thirties to middle forties) and severe unilateral headache or focal head, neck, or mastoid pain were prominent common clinical features. The dominant arteriographic features were intimal irregularities, multiple vessel involvement, tendency to arterial dissection and aneurysm formation, sparing of intracranial arteries, and tendency to partial or complete resolution of the abnormalities and regression toward normal. We have called this presumed entity “idiopathic regressing arteriopathy” pending appropriate histopathological studies.
Lower motor neuron disease with spinocerebellar degeneration
Authors: Page Robert W. , Moskowitz Roland W. , Nash Robert E. , Roessmann Uros
Published: December 2004

A patient with polymyositis responded initially to steroid therapy. A muscle biopsy disclosed features of primary myopathy and group atrophy. The patient became refractory to therapy and died with relentlessly progressive weakness. The autopsy disclosed lower motor neuron involvement and degeneration of the spinocerebellar tracts. There was loss of Purkinje cells, which may have occurred secondary to an anoxic episode prior to death. The case is unique because of the limited involvement of the lower motor and spinocerebellar systems.
Supraclavicular and carotid bruits in hemodialysis patients
Authors: Messert Bernard , Marra Thomas R. , Zerofsky Ronald A.
Published: December 2004

Auscultation of the neck vessels in patients receiving chronic hemodialysis reveals a high incidence of subclavian and carotid bruits as well as the expected precordial hemic murmurs. These bruits are associated with the high‐output hyperdynamic circulatory state. In older patients the finding could be misinterpreted as evidence of extracranial cerebrovascular disease, but clinical considerations should obviate unnecessary neuroradiological diagnostic procedures.
Temporal lobe oligodendroglioma developing from infancy into adulthood
Authors: Pelc Sabine , Brihaye Jean , Périer Olivier , Heimann Rodolphe , Balériaux Danièle
Published: December 2004

Clinical, radiological, surgical, and histological features are reported for a patient with a temporal lobe oligodendroglioma that had developed in infancy and was diagnosed and partially resected at age 10. The patient is presently 24 years old and still well. To the best of our knowledge this is the first report of an oligodendroglioma with probable onset at 5 months of life and satisfactory course over 24 years.
The pathophysiology of focal epilepsy: Neurophysiological considerations
Authors: Pedley Timothy A.
Published: December 2004

The study of epilepsy serves to emphasize the importance of integrating basic research with clinical data. In this selective review, the results of recent experiments using intracellular recording techniques, ion‐specific microelectrodes, and a methodology suitable for studying mammalian cortex in vitro are discussed in terms of cellular phenomena which underlie clinical observations made in epileptic patients. This information has begun to clarify: (1) the changes in neuronal behavior associated with interictal and ictal events; (2) alterations in local ionic microenvironment which occur during focal epileptogenesis; (3) intrinsic control mechanisms which serve to restrict seizure spread; (4) neuronal characteristics which account for the differences in seizure patterns seen in infants and adults; and (5) the possible long‐term consequences of recurrent local neuronal hyperexcitability.
Valproic acid in epilepsy: Clinical and pharmacological effects
Authors: Mattson Richard H. , Cramer Joyce A. , Williamson Peter D. , Novelly Robert A.
Published: December 2004

The antiepileptic drug valproic acid was studied in an open clinical trial as adjunct medication for 23 patients with uncontrolled seizures of a generalized or partial type. Two‐thirds of the patients experienced reduction in seizure frequency ranging from 25 to 100%. Extensive testing revealed no evidence of serious systemic toxicity due to the drug. Minor side effects (e.g., nausea, vomiting, or sedation) were usually transient.

Sodium valproate syrup and valproic acid in capsules gave equivalent mean low (23.3 μg/ml) and maximum (42.5 μg/ml) serum concentrations. The drug had a relatively short half‐life of 8.7 hours, necessitating administration in divided daily doses.

During initiation of valproate therapy there was evidence of a decline in total serum phenytoin concentration (16.5 to 10.2 μg/ml; p < 0.001) while the percentage of free phenytoin increased (10.9 to 20%). The quantity of unbound phenytoin was relatively stable throughout. This observation was interpreted as a drug interaction: valproic acid competed with phenytoin for access to plasma protein binding sites.
The spectrum of primary blastomycotic meningitis: A review of central nervous system blastomycosis
Authors: Gonyea Edward F.
Published: December 2004

Three cases of meningitis with initial and exclusive neurological involvement prompted a review of the clinical, cerebrospinal fluid, and pathological findings in an additional 78 patients with central nervous system blastomycosis. The first patient of the 3 had progressive cerebellar dysfunction as the result of chronic basilar meningitis. The second had a C8‐T1 radiculopathy without other evidence of superior sulcus syndrome, and subsequent acute fatal meningitis. The third had aseptic, benign, self‐limited meningitis followed by clinically obvios systemic blastomycosis.

Diagnosis is difficult, and it is likely that other cases have been presumptively treated for tuberculous meningitis. A more aggressive approach to diagnosis is proposed that takes into account the condition of the patient, the likelihood of dissemination at necropsy, and the frequent meningeal infections that are negative on culture of lumbar CSF.
Behavioral toxicity of anesthetic gases
Authors: Synder Bruce D. , Thomas Robert S. , Gyorky Zsuzsanna
Published: December 2004

Operating room personnel were studied for evidence of acute reversible or chronic cumulative cognitive dysfunction as measured by psychometric testing. In comparison with matched controls, no acute reversible deficits were noted in a relatively unpolluted operating room environment. There were only 2 marginally significant test differences between the control and operating room groups; however, control subjects did better than operating room personnel on 16 of 18 tests at both the beginning and end of the work week. Analyses of the deficit patterns and correlations with years of operating room exposure only weakly support the hypothesis that the test differences are due to toxic exposure.
Familial multiple sclerosis: Clinical, histocompatibility, and viral serological studies
Authors: Eldridge Roswell , McFarland Henry , Sever John , Sadowsky Doris , Krebs Helen
Published: December 2004

Evaluation of presumed “multiple sclerosis families” and comparison with recently reported families has led us to the following observations: (1) Seven of our original fourteen presumptive multiple sclerosis families had to be eliminated after personal clinical evaluation of family members failed to confirm the diagnosis in a second close relative. (2) No segregation of HLA type was noted between affected and unaffected individuals in our seven bona fide multiple sclerosis families, and no consistent segregation was noted in the twenty‐eight families reported elsewhere. This supports other genetic evidence that there is not a single, major gene mapping in the HLA complex which predisposes to multiple sclerosis. (3) The DW 2 antigen was increased in frequency among affected members of our families, and the A3 B7 haplotype was more frequent among affected members of other families reported. But unaffected members also tended to have an increased frequency of these same antigens. (4) No relationship was noted between HLA type and antimeasles antibody titer within our families.
Bilateral oculomotor paralysis due to neurosyphilis
Authors: Jordan Kenneth , Marino Joseph , Damast Melvyn
Published: December 2004

Bilateral third nerve paresis attributed to neurosyphilis has not been documented in the absence of associated neurological deficits. We describe a patient with isolated bilateral third nerve paresis, positive serological findings in blood and cerebrospinal fluid, CSF pleocytosis, and increased protein content. Resolution of these abnormalities occurred when the patient was treated with penicillin.
Levodopa in Parkinson disease: A long‐term appraisal of mortality
Authors: Joseph Clifford , Chassan Jacob B. , Koch Maria‐Luise
Published: December 2004

Mortality rates for a group of patients in a collaborative multicenter study of levodopa (Larodopa) in the treatment of parkinsonism were compared with corresponding mortality rates in the general population adjusted for age and sex distribution in accordance with that of the parkinsonian patients under study. The death rate, conservatively adjusted for dropouts among parkinsonian patients, is estimated to be approximately 33% greater than that for the general population. This is in contrast to an earlier study of Hoehn and Yahr, prior to the advent of levodopa treatment, where in the mortality rate for parkinsonian patients was shown to be in excess of that in the general population by a factor of 2.9 or nearly 200%.
Four‐year treatment of patients with parkinsonism using amantadine alone or with levodopa
Authors: Timberlake William H. , Vance Michael A.
Published: December 2004

Half of 94 parkinsonian patients improved on amantadine therapy during acute double‐blind trials. In a four‐year follow‐up, amantadine given alone or added to a stable dose of levodopa had its greatest effect in the first month and helped few patients after six months. Levodopa either alone or added to a stable dose of amantadine had a beneficial effect lasting three years or more.

The side‐effects of edema and livido reticularis occurred twice as often in women. Confusion and hallucinations appeared sooner on a regimen of 300 mg of amantadine a day, but the ultimate incidence was the same on 200 mg a day. Withdrawal effects from amantadine are no less frequent or serious than from other antiparkinson medications and are not evidence that amantadine is still helping the patient. Considering the years of exposure, the morbidity and mortality do not indicate any risks peculiar to amantadine. Our mortality in all groups combined was 2.4 times that of the age‐ and sex‐matched United States population.
Ischemia‐induced seizures and cortical monoamine levels
Authors: Welch K. M. A. , Wang T.‐P. F. , Chabi Eva
Published: December 2004

Seizure activity as a component of the ischemic process possibly responsible for monoamine changes described in the gerbil stroke model was the subject of this study. Abnormal motor activity suggestive of seizures developed one to three hours after unilateral ligation of the common carotid artery in approximately 50% of gerbils that exhibited signs of stroke. Reduction of cortical levels of dopamine and norepinephrine was observed only when seizures occurred in association with stroke. The levels of 5‐hydroxytryptamine were reduced bilaterally in animals with and without signs of stroke and were reduced further in animals with stroke plus seizures.

Further study is needed to establish whether the catecholamine changes associated with ischemia‐induced seizures are primary and causative or secondary to seizure activity itself. In the ischemic brain, 5‐hydroxytryptamine metabolism appears disordered independent of seizure activity. Seizure activity must be taken into account when the mechanisms of disordered monoamine metabolism are being examined in the gerbil stroke model.
Regulation of intracranial pressure in rat, dog, and man
Authors: Mann J. Douglas , Butler Albert B. , Rosenthal Jaime E. , Maffeo Charles J. , Johnson Richard N. , Bass Norman H.
Published: December 2004

Intracranial resistance and compliance were assessed in the cerebrospinal fluid system of rat, dog, and man by means of low‐volume, short‐duration infusions of artificial CSF into the subarachnoid space. A family of pressure/time response curves was obtained for each species: at low flow rates, steady‐state pressure elevations were associated with marked neurological dysfunction. From these curves a mathematical model was developed which describes transport and pressure‐dependent valvelike characteristics of the CSF outflow resistance. This resistance. This resistance shows a progressive phylogenetic change in rat, dog, and man which increasingly enhances the species' abilities to vent fluid from the intracranial cavity into the venous circulation. Arachnoid villi appear to be the major structures modulating sustained, nonlethal elevations of intracranial pressure.
Amanita mushroom poisoning with recovery from coma: A case report
Authors: Teutsch Carol , Brennan Robert W.
Published: December 2004

The incidence of Amanita mushroom poisoning seems to be increasing in the United States. Its neurological aspects contribute importantly to morbidity and mortality. In reported series, mortality has ranged from 50 to 90%, and survival following the appearance of coma was exceptional. The cause of nervous system involvement was uncertain. In the patient presented here, clinical and electroencephalographic observations were consistent with severe hepatic encephalopathy and correlated closely with liver function abnormalities. Despite the development of coma, full recovery followed the use of thioctic acid, an experimental therapeutic agent.
Chronic paroxysmal hemicrania: A disabling headache syndrome responding to indomethacin
Authors: Price Richard W. , Posner Jerome B.
Published: December 2004

A 57‐year‐old man experienced daily episodes of unilateral headache accompanied by homolateral partial Horner syndrome and rhinorrhea for more than 40 years. Treatment with indomethacin eliminated these headaches. The patient's illness is best classified as “chronic paroxysmal hemicrania” as described by Sjaastad and Dale.
Visual dysfunctions in optic tract lesions
Authors: Bender Morris B. , Bodis‐Wollner Ivan
Published: December 2004

Characteristic visual symptoms and signs in 12 patients with neoplasms or aneurysms involving the optic tract are summarized. Blurred vision was the most common initial manifestation. Optic atrophy became apparent in 7 of the 12 patients. Most patients had uniocular central scotomas with reduced visual acuity, and 2 had homonymous scotomas. Field defects were frequently incomplete and incongruous, combining central scotomas with elements of homonymous and bitemporal amblyopias. Seven patients had endocrine disturbances and memory deficits in addition to their visual symptoms.
Studies of fragmented sarcoplasmic reticulum from human skeletal muscle
Authors: Scarpa A. , Dimauro S. , Bonilla E. , Schotland D. L.
Published: December 2004

Sarcoplasmic reticulum (SR) vesicles were isolated from muscle biopsies of 4 normal volunteers, a patient with McArdle disease (before and during contracture), and a patient with normokalemic periodic paralysis. Fractions were analyzed for purity by electron microscopy and biochemical analysis of specific marker enzymes. Adenosine triphosphate (ATP)‐dependent calcium ion uptake was measured kinetically by the absorbence changes of murexide, a metallochromic indicator of ionized Ca++ concentrations, in the absence of oxalate or other calcium‐complexing anions. In these experiments, time resolution of the Ca++ transport rate was limited by the manual mixing of reagents, which occurred in 1 to 3 seconds. In 1 case the “true” initial velocity of Ca++ uptake was measured by rapid mixing of ATP in a stopped‐flow apparatus and by following the change in absorbence of murexide in a storage oscilloscope. In SR from normal human muscle the ATP‐dependent Ca++ uptake was 3.5 nmoles per second per milligram of protein for the first 5 seconds after ATP mixing. Fast kinetic experiments showed that Ca++ uptake proceeded linearly for the first 500 msec at a rate of 9 nmoles per second per milligram of protein (at 25°C) and then progressively declined to reach steady‐state levels in 40 to 50 seconds. No abnormality of Ca++ transport was found in SR vesicles from the patient with McArdle disease and the patient with normokalemic periodic paralysis.
The action of phenytoin on a composite electrical‐chemical synapse in the lamprey spinal cord
Authors: Selzer Michael E.
Published: December 2004

The effect of phenytoin (PTN), 20 μg per milliliter, was tested on the composite electrical‐chemical synapse between pairs of giant interneurons in the isolated spinal cords of river lampreys (Ichthiomyzon unicuspis). The main observations were that: (1) PTN reversibly reduced the chemical component of the excitatory postsynaptic potential by up to 70%; (2) PTN greatly reduced or eliminated posttetanic potentiation (PTP) of the chemical component; (3) PTN produced little or no decrease in the size of the electrical component; and (4) PTN did not cause blockage of the electrical component during high‐frequency stimulation of the presynaptic neuron. Since the electrical component reflects the presynaptic spike, the suppression of PTP cannot be explained by blockage of this spike during the tetanus. These four observations are consistent with a current hypothesis concerning the anticonvulsant effect of PTN—that the drug inhibits calcium entry into stimulated presynaptic terminals. This would block PTP by reducing the accumulation of calcium in the terminal during repetitive stimulation.
Ventricular volume in man computed from CAT scans
Authors: Penn Richard D. , Belanger Michael G. , Yasnoff William A.
Published: December 2004

A new interactive computer system has been developed to measure ventricular volume from CAT scans. Testing this system on phantoms demonstrated an accuracy to within 16%. Then a series of scans of patients with obstructive hydrocephalus was analyzed using both traditional linear measures and the computer method. None of the traditional measures were directly proportional to the calculated volume. The area measure of ventricular volume correlated well with the computer‐generated values. Clinical studies which attempt to quantitate ventricular volume should use a computerized or planigraphic measure.
A disorder of biogenic amines in dihydropteridine reductase deficiency
Authors: Butler Ian J. , Koslow Stephen H. , Krumholz Allan , Holtzman Neil A. , Kaufman Seymour
Published: December 2004

A severe deficiency of dihydropteridine reductase (DHPR) in liver, brain, and cultured skin fibroblasts was demonstrated in a child with hyperphenylalaninemia and an atypical form of phenylketonuria. DHPR is required for regeneration of the cofactor, tetrahydrobiopterin. The cofactor is essential in hydroxylation of aromatic amino acid precursors in the biosynthesis of neurotransmitters, serotonin, dopamine, and norepinephrine. In gray tissue at brain biopsy, dopamine was low at 3 ng per gram of tissue, serotonin was barely detected, and norepinephrine appeared high at 1600 ng per gram. In cerebrospinal fluid, homovanillic acid (HVA) was low normal at 33 ng/ml, 5‐hydroxyindoleacetic acid (5‐HIAA) was low at 4.2 ng/ml, and after a high dose of oral probenecid there was impaired accumulation of HVA to 128 ng/ml and 5‐HIAA to 22.4 ng/ml. When the patient was 22 months of age, treatment with hydroxylated aromatic amino acid precursors was initiated, and after three months HVA and 5‐HIAA levels were increased in CSF. The apparent restoration of biogenic amines in brain appears to have delayed the rate of neurological deterioration. DHPR activity in cultured skin fibroblasts of children with persistent hyperphenylalaninemia should permit early diagnosis and early treatment of this disorder.
The cherry‐red spot‐myoclonus syndrome
Authors: Rapin Isabelle , Goldfischer Sidney , Katzman Robert , Engel Jerome , O'Brien John S.
Published: December 2004

Three young women, 2 of them sisters, were found to have cherry‐red spots at the macula when they were children. In 1 patient the spots faded before she was 20 years old. In all 3, incapacitating myoclonus and insidious visual loss developed in adolescence. Their intellect is normal and they have no gargoyle‐like features. A variety of lysosomal inclusions were noted in cortical neurons in a biopsy specimen taken from 1 patient in childhood. Liver biopsy fifteen years later revealed mucopolysaccharide‐like inclusions in Kupffer cells and hepatocytes. Lipofuscin bodies were abundant in neurons and hepatocytes. The patients excrete sialic acid‐containing oligosaccharides not present in normal urine, suggesting a defect in degradation of glycoproteins. The specific enzymatic defect in these patients appears to be a deficiency of lysosomal neuraminidase.
Predictors of mortality in presenile and senile dementia
Authors: Kaszniak Alfred W. , Fox Jacob , Gandell David L. , Garron David C. , Huckman Michael S. , Ramsey Ruth G.
Published: December 2004

Forty‐seven hospitalized patients with a diagnosis of presenile or senile dementia and without focal neurological disease or major systemic illness were given complete neurological, neuroradiological, and neuropsychological examinations. Mortality at one year after hospitalization was determined, 12 patients being lost to follow‐up. Of the remaining 35 patients, 19 were alive and 16 had died. These two groups did not differ in age, education, length of dementia history, sex, race, or degree of cerebral atrophy (by computerized tomography). Significant differences were found in degree of electroencephalographic abnormality and in 8 of 14 cognitive measures, the largest single difference being on a test of expressive language. Discriminant function analysis made with the cognitive measures as dependent variables yielded a correct prediction (classification analysis) of mortality in all but 1 case (97% accuracy). These results suggest that (1) degree of functional brain impairment rather than degree of cerebral atrophy may be the more important influence on mortality in dementia patients without focal lesions, (2) short‐term survival may be accurately predicted in such patients by tests of cognitive functioning, and (3) expressive language deficit in such patients may indicate a particularly poor prognosis for survival.
Perhexiline neuropathy: A clinicopathological study
Authors: Said Gérard
Published: December 2004

Five patients developed a mild to severe polyneuropathy while under treatment with perhexiline maleate, a drug used in long‐term treatment for angina pectoris. Recovery took place within a few months after drug withdrawal.

We performed qualitative and quantitative light and electron microscopical studies, including teased fiber preparations, in different patients; 16 to 90% of the fibers showed segmental demyelination, an unusual feature in drug‐induced neuropathies, and 3 to 20% were undergoing wallerian degeneration. Severe loss of myelinated axons was noted in all 5 patients. In these patients clinical symptoms occurred only when a great number of fibers had already been lost and most of the surviving fibers showed demylination.
Changing patterns of childhood aphasia
Authors: Woods Bryan T. , Teuber Hans‐Lukas
Published: December 2004

Acquired aphasia in children has been generally characterized as nonfluent, transient, and frequently due to right hemisphere lesions. We studied 65 children with unilateral hemispheric brain lesions occurring after speech acquisition any time from the second through the fourteenth year. Of 34 patients with a left hemisphere lesion, 25 had an initial aphasic speech disturbance, while of 31 patients with a right hemisphere lesion, only 4 (including 2 lefthanders) showed any initial aphasia. All those who became aphasic before the age of 8 years eventually regained speech, but recovery time required ranged from less than a month to more than two years. One 5‐year‐old boy who recovered had initial jargon aphasia. Our review of the literature indicated that the conflict between our results and the traditional claim of frequent aphasia with right hemisphere lesions was only apparent; the great majority of crossed aphasias are concentrated in reports written before antibiotics were used, and many cases were associated with systemic bacterial infections.
Monoligated Palladium Species as Catalysts in Cross‐Coupling Reactions
Authors: Christmann Ute , Vilar Ramón
Published: December 2004

Palladium‐mediated cross‐coupling reactions are attractive organometallic transformations for the generation of CC, CN, CO, and CS bonds. Despite being widely employed in small‐scale syntheses, cross‐coupling reactions have not found important industrial applications because until recently, only reactive aryl bromides and iodides could be used as substrates. These substrates are generally more expensive and less widely available than their chloride counterparts. Over the past few years, new catalytic systems with the ability to activate unreactive and sterically hindered aryl chlorides have been developed. The new catalysts are based on palladium complexes that contain electron‐rich and bulky phosphine or carbene ligands. The enhanced reactivity observed with these new systems has been attributed to the formation of unsaturated and reactive [PdL] species which can readily undergo oxidative addition reactions with ArX to yield [Pd(Ar)X(L)].
Cover Picture: An Unprecedented Bridging Phenoxyl Radical in Dicopper(II) Complexes: Evidence for an S=3/2 Spin State (Angew. Chem. Int. Ed. 3/2005)
Authors: Michel Fabien , Torelli Stéphane , Thomas Fabrice , Duboc Carole , Philouze Christian , Belle Catherine , Hamman Sylvain , Saint‐Aman Eric , Pierre Jean‐Louis
Published: December 2004

Metal–radical interactions are at the heart of many biocatalysts that contain transition metals. The cover picture shows the structure of a dicopper(II) complex in which a phenoxyl radical bridges the metal centers. The three spins are ferromagnetically coupled, as the black arrows indicate. In their Communication on page 438 ff., F. Thomas and co‐workers describe the synthesis, structure, and properties of this type of complex. The stability and reactivity are dictated by the nuclearity of the complex and the nature of the coordinating solvent.
An analysis of optical rotatory dispersion curves of synthetic polypeptides
Authors: Yamaoka K. K.
Published: December 2004

A two‐term Drude equation was proposed to he substituted for the Moffitt‐Yang equation as an empirical expression in order to analyze and regenerate observed optical rotatory dispersion (ORD) curves of synthetic polypeptides and proteins. A convenient graphical method was introduced to evaluate four parameters involved in the present expression. The method was demonstrated in detail with an observed ORD curve of sodium poly‐α, L‐glutamate (NaPLG) at pH 4.35. A number of ORD curves of poly‐γ‐benzyl‐L‐glutamate (PBLG) in both helix and random‐coil were analyzed. For all ORD curves examined, the critical wavelengths, λ0 and λ1, were found to be 193 and 226 mμ. Aromatic solvents wore found to constitute a group distinct from other helix‐forming solvents. A far ultraviolet ORD curve of NaPLG was also analyzed with use of Natanson's equation which contains two damping factors. It was concluded that for an exact description of the ORD curve of helical polypeptides a third term, probably negative, should be taken under consideration. An illustrative application of the present method to the data of the helix‐coil transition of NaPLG shed much light on the relation between the four parameters and its molecular conformation as a function of pH.
Electrophoresis of DNA. III. The effect of several univalent electrolytes on the mobility of DNA
Authors: Ross Philip D. , Scruggs Robert L.
Published: December 2004

The electrophoretic mobility of DNA in the presence of tetramethylammonium and alkali metal ion chlorides has been studied as a function of ionic strength. Each cation exhibits a characteristic behavior in accord with the idea that the order of inter‐action with DNA is Li+ > Na+ > K+ > TMA+. The application of theories of the electrophoresis of polyelectrolytes is discussed, leading to an attempt to calculate the fractional charge per DNA phosphorus from the mobility data. Over the range 0.05–0.4M a constant and unique value of the DNA charge is obtained in the presence of each cation. Values of the zeta potential and of the friction factor per monomer unit are also calculated.
The exclusion of large solutes by cartilage proteinpolysaccharide
Authors: Gerber Bernard R. , Schubert Maxwell
Published: December 2004

The dialysis technique of Ogston and Phelps, using a Millipore filter as a membrane to measure the effect of a nondialyzable macromolecule on the distribution of dialyzable macromolecules, has been used to measure the effect of a compound of protein and polysaccharide (PP‐L), isolated from cartilage, on the distribution of some dialyzable macromolecules. PP‐L (1%) in buffer acts as if part of its solution volume were unavailable as follows: 55% for serum albumin, 64% for chondroitin sulfate made by alkaline degradation of PP‐L, and 98% for chondroitin sulfate made by trypsin degradation of PP‐L. The use of different buffers, variation of pH from 5 to 10, variation of temperature from 5 to 42°C., variation of salt concentration from 0.15 to 2N, and variation in ethanol concentration from 0 to 30% had no effect on the distribution of albumin, which was always excluded to about the same extent from the PP‐L solution. This effect could account for the virtual absence of serum proteins and of free chondroitin sulfate from cartilage. At low salt concentration (0.10–0.02N) and at 5°C, the distribution was reversed and albumin behaved as if strongly bound by PP‐L. At low‐levels of albumin this binding of PP‐L increased linearly with albumin concentration. This binding was partly reversed by raising the temperature or by adding urea, and completely reversed by the addition of salt. Polystyrene sulfonate, a nondiffusible polyanion, had an effect similar to that of PP‐L in excluding chondroitin sulfate from its solutions. It showed a very strong binding effect for albumin which persisted even in the presence of salt as high as 2N. A quantitative comparison has been made between the excluded volume effect of the soluble macromolecules PP‐L and hyaluronate and the effect of insoluble Sephadex particles to exclude large molecules from their interiors. The technique of Ogston and Phelps is thus valuable‐ not only for measuring the exclusion of diffusible macromolecules from part of the solution volume of nondiffusible macromolecules, but also for measuring the existence and extent of binding of diffusible macromolecules to nondiffusible macromolecules.