bluuebunny/crossref_metadata_2025 huggingface dataset:
Neonatal‐onset adrenoleukodystrophy in a girl
Authors: Haas Joel E. , Johnson Edward Stidworthy , Farrell Donald L.
Published: January 2005

A 4‐year 11‐month‐old girl developed cerebral degeneration with onset in the neonatal period. Postmortem examination showed gross, microscopic, ultrastructural, and biochemical changes identical to those associated with adrenoleukodystrophy (ALD), a sex‐linked disorder of boys beginning in the first decade of life. Cytoplasmic inclusions ultrastructurally identical to those in brains and adrenal glands of ALD patients were present not only in this girl's adrenal glands and brain but also in reticuloendothelial cells of the liver, lymph node, spleen, thymus, and hepatic lysosomes. Thin‐layer and gas‐liquid chromatographic analysis of cerebral tissues demonstrated abnormal long‐chain fatty acids in the cholesterol ester fraction, identical to those present in affected tissues of males with ALD. The documentation of abnormal long‐chain fatty acids in cerebral tissues of a female patient supports the concept that infantile‐onset ALD is a clinically and pathologically distinctive entity characterized by prominent visceral storage abnormalities and occurrence in both sexes. These findings also suggest that ALD and related entities are either phenotypic variants of a specific enzyme deficiency or separate diseases resulting from different mutations in a common metabolic pathway.
Mood, vegetative disturbance, and dexamethasone suppression test after stroke
Authors: Finklestein Seth , Benowitz Larry I. , Baldessarini Ross J. , Arana George W. , Levine David , Woo Elaine , Bear David , Moya Kenneth , Stoll Andrew L.
Published: January 2005

Assessments of mood disturbance and “vegetative” (appetite or sleep) disturbance as well as a single‐dose dexamethasone suppression test (DST) were carried out in 25 randomly selected stroke patients and in 13 nonstroke control patients hosptalized in a rehabilitation center. Prevalence rates of moderate‐to‐sever depression of mood and vegetative disturbance were significantly higher in stroke patients than controls (48% and 52% versus 0% and 8%, respectively), as was the prevalence of abnormal DST results (52% versus 8%). Abnormal DST results were associated with the occurrence of moderate to severe mood, appetite, and sleep disturbances among all patients. In 2 stroke patients, repeated DST results paralleled the clinical course. The DST may be useful as an adjunct to the diagnosis and in monitoring the progress of the common and potentially reversible mood and vegetative disturbances occurring after stroke.
Effect of folic acid and vitamin B12 deficiencies on 5‐hydroxyindoleacetic acid in human cerebrspinal fluid
Authors: Botez M. I. , Young Simon N. , Bachevalier Jocelyne , Gauthier Serge
Published: January 2005

Indoles were measured in cerebrospinal fluid (CSF) from control patients, from patients suffering from folate deficiency, and from patients with vitamin B12 deficiency. The folate‐deficient patients were classified according to whether they exhibited a neuropsychiatric syndrome, consisting of organic mental changes, polyneuropathy, and depression, which responded to folate administration. CSF 5‐hydroxyindoleacetic acid was low in the vitamin B12‐deficient patients and in those folate‐deficient patients whose neuropsychiatric signs were responsive to folate administration, but was normal in the folate‐deficient patients whose symptoms were not related to folate deficiency. CSF 5‐hydroxyindoleacetic acid returned to normal with folate treatment in the patients exhibiting folate‐responsive neuropsychiatric signs. The data indicate a close association between folate‐responsive neuropsychiatric symptoms and change in 5‐hydroxytryptamine metabolism in the central nervous system.
Intravascular malignant histiocytosis mimicking central nervous system vasculitis: An immunopathological diagnostic approach
Authors: Krieger C. , Robitaille Y. , Jothy S. , Elleker G.
Published: January 2005

A 53‐year‐old man presented with a lumbosacral polyradiculoneuropathy and developed fluctuating encephalopathy suggestive of multifocal small vessel disease. Postmortem examination demonstrated multifocal vascular occlusions by undifferentiated cells confined to the intravascular space. Extravascular spread was found only in spleen and liver. The presence of lysozyme and absence of factor VIII in the cytoplasm of the malignant cells confirmed their histiocytic nature. This patient had an unusual intravascular form of malignant histiocytosis that must be included in the differential diagnosis of multifocal vascular disease.
Superior sagittal sinus thrombosis associated with androgen therapy for hypoplastic anemia
Authors: Shiozawa Zenji , Yamada Hideo , Mabuchi Chiyuki , Hotta Tomomitsu , Saito Masaru , Sobue Itsuro , Hunag Yun Peng
Published: January 2005

Of 27 patients with hypoplastic anemia treated between 1971 and 1974 with male hormone and protein‐assimilating hormone, 3 developed superior sagittal sinus thrombosis (SSST). The clinical symptoms and signs and angiographic findings of SSST were characteristic enough to allow an early diagnosis. Signs related to SSST were seizures, hemiplegia, facial palsy, stupor, and coma, with the most important prodrome and consistent subjuective complaint being headache. Following discontinuation of the hormone therapy, neurological signs and symptoms related to SSST gradually subsided. In all cases, the hematological picture improved with discontinuation of the hormone therapies. It appears that administration of male hormone or protein‐assimilating hormone can be associated with the development of SSST. If neurological symptoms and signs of SSST appear, administration of the hormones should be discontinued.
Extracranial vascular changes and the source of pain in migraine headache
Authors: Drummond Peter D. , Lance James W.
Published: January 2005

The extracranial circulation of 66 migrainous patients was assessed during unilateral headache by recording the pulse amplitude of the superficial temporal artery and its main frontal branch, by facial thermography, and by changes in the intensity of headache when temporal or carotid arteries were compressed.

Amplitude of pulsation of the superficial temporal artery did not differ between headache and headache‐free sides but the amplitude of its frontal branch was increased on the headache side, specifically in a subgroup of patients whose headache was relieved by compressing the superficial temporal artery (“extracranial vascular” group). Facial thermograms demonstrated significant differences in heat loss from the temples and orbits between migrainous patients and controls, frontotemporal changes being more conspicuous in the extracranial vascular group. It was concluded that dilatation of the superficial temporal artery and its branches contributes substantially to migraine headache in only a minority of patients.
Pupil sparing in oculomotor palsy: A brief review
Authors: Nadeau Stephen E. , Trobe Jonathan D.
Published: January 2005

The clinical dictum that pupil sparing in oculomotor nerve palsy predicts an extraaxial ischemic lesion while pupil involvement predicts an extraaxial compressive lesion has some important exceptions. Two case reports and a review of the literature disclose that pupil sparing occurs in a small proportion of intraaxial and compressve subarachnoid oculomotor nerve lesions and in a large proportion of compressive cavernous sinus oculomotor nerve lesions. Careful examination is also necessary to separate examples of apparent pupil sparing in cases of aberrant regeneration and in some instances of cavernous sinus compression.
Central nervous system excitatory effects of meperidine in cancer patients
Authors: Kaiko Robert F. , Foley Kathleen M. , Grabinski Patricia Y. , Heidrich George , Rogers Ada G. , Inturrisi Charles E. , Reidenberg Marcus M.
Published: January 2005

The analgesic meperidine has been reported to produce signs of central nervous system excitation in human beings. To determine the relationship between signs and symptoms of central nervous system excitation and plasma levels of meperidine and normeperidine, we studied 67 patients receiving meperidine for the relief of postoperative or chronic pain. In 48 patients, excitatory effects ranging from mild nervousness to tremors, twitches, multifocal myoclonus, and seizures were directly correlated with accumulation of normeperidine in plasma. Evidence of compromised renal function occurred in only 14 of the 48 symptomatic patients, suggesting that renal dysfunction may contribute to but is not the sole factor in the accumulation of normeperidine or its relation to adverse neurological signs. In a second study we surveyed mood alterations in 47 patients receiving meperidine and 29 receiving other narcotic analgesics for postoperative pain. The repeated administration of meperidine was associated with adverse alterations in various elements of mood (e.g., apprehension, sadness, restlessness).
Central pontine myelinolysis following rapid correction of hyponatremia
Authors: Laureno Robert
Published: January 2005

Central pontine and extrapontine myelinolysis was experimentally produced in dogs by the rapid correction of severe, sustained, vasopressin‐induced hyponatremia. Hyponatremia alone or slowly corrected hyponatremia did not produce the disease. Affected dogs showed rigid quadriparesis. The central pons, lateral aspects of the thalamus and adjacent internal capsules, deep layers of cerebral cortex and subjacent white matter, cerebellum, and other regions were symmetrically involved. Myelin and oligodendroglia were affected out of proportion to axons and neurons. Thus, the clinical features, the distribution of the lesions, and their histological features closely resemble the human disease. These experiments document an electrolyte manipulation that can cause permanent neuropathological lesions. Taken with the available clinical data on human patients, the experimental results indicate that human myelinolysis may be due to a rapid increase in serum sodium from previously low levels, and that rapid normalization of severe, sustained hyponatremia should therefore be avoided.
Computerized tomography as a diagnostic aid in acute hemorrhagic leukoencephalitis
Authors: Rothstein Ted L. , Shaw Cheng‐Mei
Published: January 2005

Computerized tomography (CT) in a pathologically proven case of acute hemorrhagic leukoencephalitis (AHL) showed a mass effect and increased absorption coefficient in the right hemisphere within 18 hours of the onset of neurological symptoms. The changes corresponded to the site of white matter edema, necrosis, and petechial hemorrhages demonstrated postmortem. The early changes on CT reflect the hyperacute nature of AHL and differ from those of herpes simplex encephalitis.
Intracerebral hemorrhage and vasculitis related to ephedrine abuse
Authors: Wooten Marvin R. , Khangure Makhan S. , Murphy Martin J.
Published: January 2005

Intracerebral hemorrhage occurred in a 20‐year‐old man following self‐administration of ephedrine. Changes seen on cerebral angiography were typical of vasculitis, and immune complex deposition was found in a skin biopsy. This combination has not previously been reported with ephedrine abuse although it is well known with amphetamine abuse.
Progressive multifocal leukoencephalopathy: A burnt‐out case
Authors: Price Richard W. , Nielsen Surl , Horten Bruce , Rubino Michael , Padgett Billie , Walker Duard
Published: January 2005

A patient with Hodgkin's disease developed progressive multifocal leukoencephalopathy (PML), documented by brain biopsy to be associated with JC virus infection. His disease progressed over several months, resulting in severe neurological deficit, but then stabilized with little or no further clinical progression during the remaining year of his life. Histopathological evaluation of the brain at autopsy supported the clinical impression that brain infection was arrested. Whereas the brain biopsy exhibited the histological features of active PML including giant bizarre astrocytes, at postmortem examination brain lesions appeared inactive, with regression of astrocytic changes and elimination of oligodendroglial inclusions. Similarly, JC virus antigen, present in the brain biopsy, was not detected in the autopsied brain. This case provides further evidence that PML is not invariably fatal and that clinical and cytological remission can occur.
Somatostatin is increased in the basal ganglia in Huntington disease
Authors: Aronin Neil , Cooper Paul E. , Lorenz Linda J. , Bird Edward D. , Sagar Stephen M. , Leeman Susan E. , Martin Joseph B.
Published: January 2005

Huntington disease (HD) is an autosomal dominant hereditary disorder characterized by premature cell death, predominantly in the neostriatum. Decreased concentrations of several neurotransmitters and neuropeptides have been reported in the basal ganglia in Huntington disease. We now report that concentrations of radioimmunoassayable somatostatin are increased in extracts of the caudate (mean ± standard error of the mean, ng/gm net weight; 247± 24 versus 85 ± 11), putamen (275 ± 48 versus 74 ± 11), external globus pallidus (100 ± 10 versus 27 ±6), and internal globus pallidus (108± 21 versus 21 ± 8) in the disease. The concentrations of immunoreactive substance P measured in the same extracts were markedly reduced in caudate (mean ± standard error of the mean, pmol/gm wet wight; 25 ± 3 versus 109 ± 20), putamen (28 ± 7 versus 88 ± 28), external globus pallidus (39 ± 9 versus 196 ± 62), and internal globus pallidus (60 ± 17 versus 263 ± 39), as well as in both subdivisions of the substantia nigra. Gel permeation chromatography and high‐performance liquid chromatography showed radioimmunoassayable somatostatin to include peptides with physicochemical properties of the tetradecapeptide somatostatin and larger substances, including somatostatin‐28‐like material. A single peak of immunoreactive substance P corresponding to synthetic substance P was found by high performance liquid chromatography.

These results suggest that immunoassayable somatostatin‐containing neuronal elements in the neostriatum and globus pallidus in Huntington disease are affected differentially by the disease process from neurons that contain immunoreactive substance P.
Facial recognition and brain asymmetries: Clues to underlying mechanisms
Authors: Gazzaniga Michael S. , Smylie Charlotte S.
Published: January 2005

A series of similar faces was presented to either the left or right visual field of three adults with brains surgically split along the corpus callosum. The left hemisphere displayed a marked and persistent deficit in performing a match‐to‐sample task, whereas the right hemisphere performed the task well. Additional test results suggest that the superiority is not specific to faces and is also not caused by specialized differences in sensory processes, but rather is related to differences in each hemisphere's ability to encode stimuli that cannot be adequately differentiated with a verbal description.
Induction of brain edema following intracerebral injection of arachidonic acid
Authors: Chan Pak Hoo , Fishman Robert A. , Caronna John , Schmidley James W. , Prioleau George , Lee Janie
Published: January 2005

The effects of polyunsaturated fatty acids on brain edema formation have been studied in rats. Intracerebral injection of polyunsaturated fatty acids (PUFAs), including linolenic acid (18:3) and arachidonic acid (20:4), caused significant increases in cerebral water and sodium content concomitant with decreases in potassium content and Na+‐ and K+‐ dependent adenosine triphosphatase activity. There was gross and microscopic evidence of edema. Saturated fatty acids and monounsaturated fatty acid were not effective in inducing brain edema. The {125I}‐bovine serum albumin spaces increased twofold and threefold at 24 hours with 18:3 and 20:4, respectively, indicating vasogenic edema with increased permeability of brain endothelial cells. Staining of the brain was observed five minutes after injection of Evans blue dye followed by arachidonic acid perfusion. A major decrease in brain potassium content was evidence of concurrent cellular (cytotoxic) edema as well. The induction of brain edema by arachidonic acid was dose dependent and maximal between 24 and 48 hours after perfusion. Dexamethasone (10 mg/kg) was effective in ameliorating the brain edema, whereas a cyclooxygenase inhibitor, indomethacin (10 mg/kg), was not. These data indicate that arachidonic acid and other PUFAs have the ability to induce vasogenic and cellular brain edema and further support the hypothesis that the degradation of phospholipids and accumulation of PUFAs, particularly arachidonic acid, initiate the development of brain edema in various disease states.
Endocrine function following complex partial seizures
Authors: Pritchard P. B. , Wannamaker B. B. , Sagel J. , Nair R. , Devillier C
Published: January 2005

Previous studies have demonstrated hyperprolactinemia following generalized tonic‐clonic seizures and after electroconvulsive therapy. We found transient hyperprolactinemia following complex partial seizures but little change in serum gonadotropins, thyroid‐stimulating hormone, growth hormone, or cortisol. Serum prolactin was invariably normal interictally. Postictal elevation of serum prolactin may represent a biochemical marker of complex partial seizures, and it offers a potential pathogenic mechanism for the sexual dysfunction that often complicates temporal lobe epilepsy.
Plasma concentrations of sodium valproate: Their clinical value
Authors: Turnbull D. M. , Rawlins M. D. , Weightman D. , Chadwick D. W.
Published: January 2005

Plasma valproate concentrations were monitored prospectively in 54 previously untreated adult patients with epilepsy. Dose and plasma concentration were highly correlated. Adverse effects were common in association with plasma levels above 100 μg/ml. In patients suffering tonic‐clonic seizures without focal symptoms, no seizures occurred when plasma levels were higher than 50 μg/ml. In patients with partial seizures, it was difficult to define a lower limit to a therapeutic range.
Cytoskeletal disorganization induced by local application of β,β′‐iminodipropionitrile and 2,5‐hexanedione
Authors: Griffin John W. , Fahnestock Kenneth E. , Price Donald L. , Cork Linda C.
Published: January 2005

β,β′‐Iminodipropionitrile and 2,5‐hexanedione are neurotoxins that produce neurofilamentous axonal swellings. The swellings produced experimentally with these agents are similar in structure but different in distribution. Neither the relationships between these agents nor the mechanisms of action are known. In this study local effects on nerve fibers were compared following injection of β′‐iminodipropionitrile and 2,5‐hexanedione beneath the perineurium of rat sciatic nerves. Soon after injection, 2,5‐hexanedione reproduced the distinctive cytoskeletal disorganization previously described with β′‐iminodipropionitrile: microtubules collected into a central channel, with neurofilaments segregated in a surrounding subaxolemmal ring. Later, the β′‐iminodipropionitrileinjected nerves developed local neurofilament accumulations, reproducing the neurofilamentous axonal swellings characteristic of systemic intoxication with these agents. The results indicate that both these agents have direct local effects on the axonal cytoskeleton and probably are similar in mechanism of action. Both these agents appear to segregate neurofilaments from the rest of the axonal cytoskeleton. This segregation may prevent the normal proximal‐to‐distal transport of neurofilaments, resulting in the formation of neurofilamentous axonal swellings.
Regional blood flow in ethylnitrosourea‐induced brain tumors
Authors: Blasberg Ronald G. , Kobayashi Tomowo , Horowitz Marc , M Jerry M. , Groothuis Dennis , Molnar Peter , D Joseph D.
Published: January 2005

Regional blood flow was measured in experimental brain tumors using iodoantipyrine labeled with carbon 14 and quantitative autoradiography. A total of fifteen oligodendrogliomas, sixteen mixed gliomas, one astrocytoma, one ependymoma, and three malignant schwannomas were studied in 9 rats. The mean tumor blood flows for all glioma classifications were similar, averaging 45 ± 3 (standard error of the mean) ml · hg−1 · min−1. Flow was fairly uniform within individual oligodendrogliomas and there was no apparent correlation between blood flow and tumor size of location. The mixed gliomas were larger than the oligodendrogliomas and had a wider range of blood flow. Small focal areas of necrosis were observed in 7 mixed gliomas, and low flows were usually measured in these regions; these flows were not always the lowest regional values measured within the mixed gliomas or total group of tumors, however. Small tumor regions with increased vascularity, frequently with endothelial cell proliferation, were observed in oligodendrogliomas and to a greater extent in mixed gliomas; these regions were correlated with small elevations in blood flow (10 to 15 ml · hg−1 · min−1) in comparison with surrounding tumor tissue. Brain adjacent to tumor usually had higher blood flows than that in tumor periphery. Hemispheric differences in blood flow related to the site of primary tumor growth were not observed.
Neurotoxicity of chemotherapeutic agents after blood‐brain barrier modification: Neuropathological studies
Authors: Neuwelt Edward A. , Glasberg Mark , Frenkel Eugene , Barnett Peggy
Published: January 2005

We examined in 47 dogs the effects of 5‐fluorouracil, Adriamycin (doxorubicin hydrochloride), cis‐diamminedichloroplatinum cyclophosphamide, and bleomycin given in association with osmotic blood‐brain barrier modification. The dose of drug ranged from 100% to as little as 5 to 10% of the conventional systemic dosage. Serial neurological observation and subsequent postmortem neuropathological evaluation at times varying from 2.5 hours to 52 days after drug administration showed that cis‐platinum and Adriamycin were highly neurotoxic, as evidenced by neurological deficits and pathological changes in the central nervous system parenchyma; 5‐fluorouracil and bleomycin had much less, but consequential neurotoxicity; and cyclophosphamide was not associated with substantial toxicity. Intracarotid cis‐platinum, unlike the other drugs, damaged the blood‐brain barrier and resulted in marked neurotoxicity in the absence of osmotic blood‐brain barrier opening. The neural lesions produced by these agents were not specific but were manifested as foci of hemorrhagic necrosis and edema. In addition, secondary brainstem hemorrhage was observed in animals that developed transtentorial herniation. On the basis of these studies, of five drugs studied at a wide range of doses, only cyclophosphamide appears to be safe enough to evaluate in clinical trials that utilize blood‐brain barrier modification to enhance drug delivery. These studies also suggest that the lack of neurotoxicity associated with the usual administration of most chemotherapeutic agents probably stems from limited entry of drug into the brain through an intact blood‐brain barrier.
Zinc in muliple sclerosis
Authors: Dore‐Duffy Paula , Catalanotto Frank , Donaldson James O. , Ostrom Karin M. , Testa Marcia A.
Published: January 2005

Zinc profiles were examined in 68 patients with multiple sclerosis, 62 normal volunteers, and 13 patients with other neurological diseases. Plasma zinc levels were slightly increased in patients with multiple sclerosis and significantly increased in those with other neurological impairments (p <0.01), compared with control subjects. Albumin‐bound as well as protein‐bound zinc levels were normal in all groups tested. The α2 macroglobulin‐bound zinc level was significantly lower (p < 0.01) in patients with multiple sclerosis than in control subjects. Erythrocyte‐bound zinc levels were significantly increased (p < 0.05) in patients with multiple sclerosis when compared with control subjects. Erythrocyte‐bound zinc was normal in patients with other neurological impairments. Because erythrocyte‐bound zinc levels are relatively independent of daily fluctuations in dietary zinc intake, an increase in these values may suggest alterations in the control mechanisms governing zinc compartmentalization in patients with multiple sclerosis.
Prognosis of ocular myasthenia
Authors: Bever Christopher T. , Aquino Abdias V. , Penn Audrey S. , Lovelace Robert E. , Rowland Lewis P.
Published: January 2005

A retrospective study of 108 patients with myasthenia gravis who had solely ocular symptoms and signs at onset was carried out to identify factors influencing prognosis. Increasing duration of pure ocular myasthenia was associated with a decreasing risk of late generalized symptoms; only 9 (15%) of the observed generalizations occurred after more than 2 years of solely ocular symptoms. Increasing age at onset was associated with greater risk of respiratory crisis or death caused by myasthenia, whereas patients younger at onset had a greater chance of a benign outcome. Neither systemic curare tests nor responses to repetitive nerve stimulation had prognostic value.
Chronic subdural hematoma with transient neurological deficits: A review of 15 cases
Authors: Moster Mark L. , Johnston David E. , Reinmuth Oscar M.
Published: January 2005

Fifteen patients with chronic subdural hematomas had transient neurological deficits. The important clinical features were aphasia in 9 patients, absence of headache in 8, and head injury in 8. Computed tomographic scan correctly identified all 15 cases of chronic subdural hematoma and should be considered mandatory before initiating anticoagulation in patients with transient ischemic attacks.
Diffuse bone marrow metastasis by glioblastoma: Premortem diagnosis by peroxidase‐antiperoxidase staining for glial fibrillary acidic protein
Authors: Yung W. K. A. , Tepper S. J. , Young D. F.
Published: January 2005

Extraneural metastases from malignant glioma and glioblastoma are believed to be rare. The most common sites of metastases are lung, lymph nodes, bone, and liver. We recently encountered two patients with glioblastoma multiforme who presented with pain and thrombocytopenia caused by diffuse metastasis to bone marrow. A premortem diagnosis was established in the first patient with the aid of peroxidase‐antiperoxidase staining of the bone marrow biopsy specimen for glial fibrillary acidic protein, a glial‐specific marker. In the second patient glial fibrillary acidic protein staining confirmed the glial nature of the primary brain tumor as well as the metastatic tumor in bone marrow. The first patient also had metastatic nodules on the pleural surface and on the fifth rib. All three metastatic foci had similar cellular morphology, suggesting selection of a population of tumor cells with extraneural metastatic potential.
Electrophysiological studies in Tourette's syndrome
Authors: Krumholz Allan , Singer Harvey S. , Niedermeyer Ernest , Burnite Rose , Harris Kenneth
Published: January 2005

The value of standard electrophysiological studies using electroencephalography and evoked responses was evaluated in patients with Tourette's syndrome. Sixteen‐channel electroencephalograms were obtained in 40 patients (36 males,4 females) awake and asleep, and evoked responses were obtained in a subgroup of 17 patients. Evoked response variables evaluated included latencies and amplitudes of visual evoked responses, brainstem auditory evoked responses, and somatosensory evoked responses to median and peroneal nerve stimulation. Only 5 of the 40 patients (12.5%) demonstrated electroencephalographic abnormalities, which included central spikes, generalized and paroxysmal slow activity, and slowing of the normal basic frequency. Evoked response studies demonstrated no consistent differences between the patients with Tourette's syndrome and age‐ and sex‐matched controls. The data demonstrate no notable diagnostic or therapeutic value for routine electroencephalographic or evoked response studies in Tourette's syndrome.
Thin axons relative to myelin spiral length in hereditary motor and sensory neuropathy, type I
Authors: Nukada Hitoshi , Dyck Peter James , Karnes Jeannine L.
Published: January 2005

The relationship of axonal to myelin area in semithin transverse sections of myelinated fibers obtained from sural nerves at the ankle level was morphometrically assessed using computer imaging. Ten patients with hereditary motor and sensory neuropathy, type I and 41 control subjects were examined. In large‐ and intermediate‐diameter myelinated fibers of diseased nerves, axons were significantly attenuated relative to the amount of myelin. Using electron micrographs, a similar finding was obtained when axon area was regressed on myelin spiral length. The altered relationship was found to be greater with more severe fiber loss. These data, plus other evidence, indicate that in this disorder there is a progressive atrophy of axons, usually most severe in distal aspects of lumbosacral neurons and associated with secondary segmental demyelination and remyelination and hypertrophic neuropathy, preceding distal axonal loss. Because the process may begin in utero or in infancy, not only atrophy but also maldevelopment of axons may be involved.
Diabetic neuropathy
Authors: Brown Mark J. , Asbury Arthur K.
Published: January 2005

Peripheral nerve disorders are important late complications of diabetes mellitus. Polyneuropathy, which may involve varying proportions of sensory, motor, and autonomic fibers, is considered the consequence of metabolic derangements that result from chronic hyperglycemia. Symmetrical proximal motor neuropathy (“diabetic amyotrophy”) also may have a metabolic basis. Mononeuropathies in diabetes may have an ischemic or compressive cause. Advances have been made in understanding the biochemical basis for diabetic polyneuropathy. The treatment of symptomatic diabetic neuropathy should be directed toward long‐term normalization of blood glucose until more specific therapies become available.
Extraction of clinical information from electroencephalographic background activity: The combined use of brain electrical activity mapping and intravenous sodium thiopental
Authors: Duffy Frank H. , Jensen Frances , Erba Giuseppe , Burchfiel James L. , Lombroso Cesare T.
Published: January 2005

Traditional visual inspection of electroencephalographic (EEG) tracings and computer‐assisted topographic mapping were compared in thier abilities to detect and locate supratentorial lesions following intravenous sodium thiopental administration. Of the 13 subjects, 8 had atrophic and 5 had mass lesions, all defined by computed tomographic scan and 11 confirmed at operation. EEGs made before and after thiopental administration were evaluated separately from topographic maps of statistical difference between EEGs at multiple frequency ranges amde before and after thiopental injection. Topographic mapping of statistical difference accurately detected all thirteen lesions, whereas EEG detected eight. In addition to demonstrating reduced beta production overlying structural abnormalities, topographic mapping revealed regionally augmented beta, especially over irritative lesions. Moreover, localization was possible with the topographic method after the first thiopental injection, whereas a second injection was required for EEG localization by visual inspection. Changes in slow (delta) activity were also useful in delineation of atrophic lesions, in which delta was usually augmented but occasionally regionally reduced. Multielectrode studies with topographic mapping appear essential in delineating cerebral abnormalities, because both slow and fast activities may be increased or reduced over such areas. The relative response of EEG background activity to thiopental at different frequencies may assist lesion characterization as well as localization.
Selective memory improvement and impairment in temporal lobectomy for epilepsy
Authors: Novelly Robert A. , Augustine Elizabeth A. , Mattson Richard H. , Glaser Gilbert H. , Williamson Peter D. , Spencer Dennis D. , Spencer Susan S.
Published: January 2005

Reexamination of 23 patients 1 year after elective anterior temporal lobectomy for intractable complex partial (psychomotor) seizures showed a reduction in certain memory functions combined with an improvement in others. Both improvement and impairment in selective memory functions were related to the degree of postoperative seizure reduction as well as to the side of the remaining temporal lobe. The results imply that uncontrolled seizures interfered with the memory functions of the temporal lobe contralateral to the epileptogenic focus.
Encephalopathy following arteriography: A possible toxic effect of contrast agents
Authors: Haley E. Clarke
Published: January 2005

Three elderly men being evaluated for cerebrovascular disease developed acute confusional states following teriography with approximately 50 ml of meglumine iothalamate. Two patients became stuporous. All covered in several days, and no specific cause was found for the disorder. In the absence of other demonstrated abnormalities, we suggest that in each patient the cephalopathy may have been a toxic reaction to the contrast agent.
Human alpha‐fetoprotein–rich fraction inhibits galactocerebroside antibody–mediated lysis of oligodendrocyte in vitro
Authors: Lubetzki‐Korn Isabelle , Hirayama Mikio , Silberberg Donald H. , Eccleston P. Ann , Schreiber Alan D. , Pleasure David , Brenner Talma , Abramsky Oded
Published: January 2005

Polyclonal rabbit antiserum to galactocerebroside (anti‐GalC) produces titer‐dependent lysis of cultured Percollisolated bovine and rat oligodendrocytes. In this study anti‐GalC produced complement‐dependent lysis of 76% of the bovine cells and 65% of the rat cells maintained for 3 to 6 days in vitro. With the concomitant addition of human umbilical cord serum fractions containing fetal alpha‐fetoprotein (AFP), lysis was decreased to 31% and 39%, respectively. Control antisera (anti–complete Freund's adjuvant/albumin) showed a cytotoxicity index of 15% for bovine cells and 23% for rat cells. Neither albumin, nor normal human serum, nor any of several pregnancy‐associated hormones reduced the lysis produced by anti‐GalC. AFP‐rich fraction reduced oligodendrocyte lysis when mixed with anti‐GalC or complement, but not when first incubated with the cells. Similar findings were obtained when AFP was assayed in specific oligodendrocyte radioimmunoassays utilizing anti‐GalC antibody. Our experiments indicate that AFP activity may result from its binding to anti‐GalC antibody; it is possible that the Fc portion of the antibody is involved. These data provide in vitro evidence of a possible immunosuppressive role of AFP in the central nervous system.
Baclofen in the treatment of trigeminal neuralgia: Double‐blind study and long‐term follow‐up
Authors: Fromm Gerhard H. , Terrence Christopher F. , Chattha Amrik S.
Published: January 2005

A double‐blind crossover study of the effects of baclofen was conducted on 10 patients with typical trigeminal neuralgia. Baclofen significantly decreased the number of painful paroxysms in 7 of the 10 patients. An open trial in another 50 patients with trigeminal neuralgia refractory to or unable to tolerate carbamazepine showed that 37 (74%) were relieved of their attacks by baclofen, either alone (12 patients) or in combination with previously ineffective doses of carbamazepine or phenytoin (25). On long‐term follow‐up of one to five years (mean, 3.0 years), 18 of the 60 patients (30%) continued pain free while receiving baclofen; 10 (17%) went into remission after 3 to 6 months; 13 (22%) became refractory to baclofen after 1 to 18 months; and 2 (3%) elected operation despite a good response to baclofen. The results indicate that baclofen is a useful drug in the treatment of trigeminal neuralgia.
Medullasin activity in granulocytes of patients with multiple sclerosis
Authors: Aoki Yosuke , Miyatake Tadashi , Shimizu Natsue , Yoshida Mitsuo
Published: January 2005

Medullasin activity in mature granulocytes was measured in the blood of 22 patients with multiple sclerosis (definite type, 16; probable type, 6). The activity was elevated in every patient in relapse; it decreased to nearly normal levels at the beginning of improvement and further decreased to normal levels with remission. Serial determinations of the level of medullasin activity in 3 patients revealed that activity increased several days before the onset of acute excerbation. Medullasin activity level in mature granulocytes obtained from patients with neurological diseases other than multiple sclerosis was largely within the normal range, except in 2 patients with spinocerebellar degeneration. Measurement of medullasin activity in mature granulocytes may become useful in both diagnosis and evaluation of multiple sclerosis.
Juvenile progressive dystonia: A new phenotype of GM2 Gangliosidosis
Authors: Meek David , Wolfe Leonhard S. , Andermann Eva , Andermann Frederick
Published: January 2005

A 10‐year‐old boy developed progressive dystonia and dementia. His symptoms had begun at age 2½ years, and he had been unable to walk by 8 years. At age 10 he was severely dystonic, unable to use his hands to feed himself, and almost anarthric. He had dysphagia and urinary incontinence, and functioned at a 4‐year‐old level of mental development. The mean percentages of β‐hexosaminidase A measured in serum, leukocytes, and fibroblasts by the heat denaturation method, each on three separate assays, were 5.9,9.8, and 13.0%, respectively. These values are higher than in Tay‐Sachs disease but are similar to levels seen in late‐onset or adult cases of GM2 gangliosidosis. This patient appears to represent a new phenotype of juvenile GM2 gangliosidosis having dystonia as the dominant symptom.
Patterns of local cerebral glucose utilization determined in Parkinson's disease by the [18F]fluorodeoxyglucose method
Authors: Kuhl David E. , Metter E. Jeffrey , Riege Walter H.
Published: January 2005

[18F]Fluorodeoxyglucose scans were performed on 9 patients with Parkinson's disease and 14 normal subjects. Five patients were restudied after an interval of 3 to 4 years. We found no selective metabolic change in striatum, where dopamine deficit is known to be greatest, in affected patients; cerebral glucose metabolism was reduced uniformly throughout the parkinsonian brain (average 18% decrease). With increased severity of bradykinesia and the development of mild to moderate dementia, global brain metabolism in Parkinson's disease decreased further. In one moderately demented patient with Parkinson's disease, severe parietal cortex hypometabolism was found, similar to that seen in Alzheimer's disease. In contrast, mildly to moderately demented patients with Huntington's disease have marked caudate hypometabolism, but cerebral glucose metabolism is normal elsewhere. It appears that in addition to the wellknown neurotransmitter loss in the nigrostriatal system, there is an abnormal metabolic process involving neurons throughout the parkinsonian brain.
Computed tomography of the brain in subacute sclerosing panencephalitis
Authors: Krawiecki Nicolas S. , Dyken Paul R. , El Gammal Taher , Durant Robert H. , Swift Andrea
Published: January 2005

Twenty‐four computed tomographic scans of 12 patients with confirmed subacute sclerosing panencephalitis were studied using standardized techiques of radiological assessment. Abnormalities encountered were of four types–s(1) lateral ventricular dilatation, (2) cerebral cortical atrophy and sylvian fissure widening, (3) low parenchymal attenuation, and (4) brainstem atrophy and cerebellar atrophy‐and of varying degrees. The abnormalities correlated best with the stage and duration of disease, but not necessarily well with the patient's mental state. The fewest radiological abnormalities were encountered in the acute or early stages, whereas more signs of parenchymal disturbances in the form of low attenuations emerged during intermediate periods. Chronic periods were accompanied by atrophic changes in the form of cortical atrophy, ventricular dilatation, and brainstem cerebellar atrophy.
Acquired immune deficiency syndrome and multiple tract degeneration in a homosexual man
Authors: Horoupian D. S. , Pick P. , Spigland I. , Smith P. , Portenoy R. , Katzman R. , Cho S.
Published: January 2005

A 35‐year‐old homosexual man with acquired immune deficiency syndrome developed progressive dementia and spastic quadriparesis over a two‐month period. Severe symmetrical, bilateral degeneration of the corticospinal and frontopontine fibers and cerebellar white matter were found postmortem. Several glial nodules in the brainstem and cerebellum suggested “smoldering” brainstem encephalitis. The possible association of the adenovirus that was isolated from the patient's cerebrospinal fluid to these obscure changes in the central nervous system is discussed.
Focal cerebral ischemia in the rat: Topography of hemodynamic and histopathological changes
Authors: Tyson George W. , Teasdale Graham M. , Graham David I. , McCulloch James
Published: January 2005

We studied local cerebral blood flow, as measured by autoradiography with digital image processing and by tissue morphology, in six rats 4 hours after occlusion of the proximal middle cerebral artery. A consistent, three‐dimensional pattern of graded reductions in local cerebral blood flow involved the affected hemisphere, with a densely ischemic zone (local cerebral blood flow less than 3 ml/100 gm/min) in the doesolateral caudate putamen and the adjacent frontoparietal cortex. In the frontoparietal cortex, the normal laminar pattern of local cerebral blood flow was disrupted, and there was a transcortical gradient in flow, with pronounced ischemia in deeper layers and relatively preserved superficial flow. Comparisons of autoradiographic finding with histopathological abnormalities in adjacent forzen sections showed that the region of ischemic damage corresponded closely with the area of greatest reduction in blood flow. Although around this region local cerebral blood flow increased centrifugally, a striking finding was that flow density changed abruptly (a tenfold variation in flow within a 1 to 2 mm interval) at the edge of the pathological lesion. Penumbral conditions may therefore exist in only a very narrow zone 4 hours after onset of focal ischemia. After occlusion of a major cerebral artery, the pattern of local cerebral blood flow changes appears to depend on interactions among vascular architecture, reductions in perfusion pressure, alterations in metabolic demands, and variations in local vascular resistance.
The ontogeny of seizures induced by leucine‐enkephalin and β‐endorphin
Authors: Snead O. Carter , Stephens Helen
Published: January 2005

Rats ranging in postnatal age from 6 hours to 28 days were implanted with cortical and depth electrodes as well as an indwelling cannula in the lateral ventricle. We then administered varying amounts of the opiate peptides leucine‐enkephalin and β‐endorphin intracerebroventricularly with continous electroencephalographic monitoring.

Leucine‐enkephalin produced electrical seizure activity in rats as young as 2 days. β‐Endorphin administration was associated with seizures at the fifth postnatal day, with a high incidence of apnea resulting in death in animals as young as 6 hours. An adult seizure response to β‐endorphin and leucine‐enkephalin was seen at 15 and 28 days of age, respectively. Naloxone blocked the seizure produced by these opiate peptides in all age groups. The data indicate that the opiate peptides are potent epileptogenic compounds in developing brain, that seizures induced by leucine enkephalin differ from those caused by β‐endorphin, and that petit mal–like seizure activity can be an adult response in the rodent.
Acquired perinatal leukoencephalopathy
Authors: Leviton Alan , Gilles Floyd H.
Published: January 2005

Between 36 and 44 weeks after conception, telencephalic white matter in the newborn appears to be particularly vulnerable to insults that result in morphological disturbances. Available evidence indicates that this disorder (or group of disorders), named acquired perinatal leukoencephalopathy, reflects a decrease in blood flow through the distal vessels supplying paraventricular white matter and/or a metabolic disturbance produced by, or in response to, an infectious agent. Major advances in our understanding of the causes, mechanisms, and sequelae of acquired perinatal leukoencephalopathy may be made in the next decade because of improvement in brain imaging techniques, improved survival of high‐risk babies, and relatively routine follow‐up of these high‐risk babies.
Congenital hydrocephalus and eye abnormalities with severe developmental brain defects: Warburg's syndrome
Authors: Bordarier C. , Aicardi J. , Goutieres F.
Published: January 2005

Five patients are reported with Warburg's syndrome, characterized by: (1) congenital hydrocephalus, (2) severe neonatal neurological dysfunction, (3) abnormalities of the anterior and posterior chambers of the eyes, (4) absence of known cause, and (5) severe developmental abnormalities of cortical gyration and architectonics. Fourteen similar published cases are reviewed. The syndrome can be diagnosed during life on the conjunction of the first four features listed. Evidence is adduced that this syndrome is a genetically determined condition with an autosomal recessive mode of inheritance and with a 25% recurrence risk for offspring of the parents of an affected infant.
Iron uptake by mammalian cortical neurons
Authors: Swaiman Kenneth F. , Machen Valynda L.
Published: January 2005

Although iron accumulates in the brain in a number of pathological conditions, including Hallervorden‐Spatz syndrome, Parkinson's disease, and neurosyphilis, studies of brain iron metabolism have been performed only rarely. Neuronal‐enriched cultures were prepared from fetal mouse brain. After 18 days the cells were exposed to radiolabeled iron. Total iron uptake and incorporation into ferritin were rapid and linear over four hours. The addition of either methylamine or ammonium chloride, both known blockers of transferrin‐iron release through their lysosomotropic properties, inhibited total iron uptake. Methylamine also inhibited the rate of ferritin‐iron incorporation, most likely by interfering with transferrin‐iron release. The data suggest that neuronal iron transport, much like that in other mammalian tissues, is transferrin mediated and that blockers of transferrin‐iron release may be of value in conditions in which there is brain iron overload.
The neurobiology of vascular head pain
Authors: Moskowitz Michael A.
Published: January 2005

Nervous connections between the trigeminal ganglia and cerebral blood vessels have recently been identified in experimental animals and have been termed the trigeminovascular system. Existence of this system in humans is inferential. Trigeminovascular neurons and their peripheral unmyelinated nerve fibers contain the neurotransmitter peptide substance P. Most newly synthesized substance P is transported from ganglion cell bodies to afferent nerve fibers, where depolarization‐induced release of neurotransmitter into the wall of the cerebral blood vessel occurs. Substance P dilates pial arteries, increases vascular permeability, and activates cells that participate in the inflammatory response. The relationship of trigeminovascular fibers to the pathogenesis of vascular head pain sheds light on possible mechanisms of migraine and other central nervous system conditions associated with headache and inflammation.