bluuebunny/crossref_metadata_2025 huggingface dataset:
Long‐term neurological implications of somnolence syndrome in children with acute lymphocytic leukemia
Authors: Ch'ien Lawrence T. , Aur Rhomes J. A. , Stagner Shirley , Cavallo Katie , Wood Adynel , Goff John , Pitner Samuel , Omar Hustu H. , Seifert M. J. , Simone Joseph V.
Published: January 2005

A longitudinal study of 49 children with acute lymphocytic leukemia (ALL) assessed the long‐term effects of central nervous system (CNS) prophylaxis on brain function. From 10 to 12 electroencephalograms (EEGs) were done before and at intervals during and after 30 months of treatment that included 2,400 rads of CNS irradiation plus intrathecal methotrexate therapy. None of the children had CNS leukemia, and all remained in first complete remissions. All 49 had abnormally slow EEG background frequencies during the four‐year study, and 29 (60%) developed somnolence syndrome six to eight weeks after CNS prophylaxis. During this syndrome, EEG background frequencies decreased more than 3 standard deviations below the expected mean values for normal children. Thereafter, 7 of the 29 began to show signs of learning disabilities and 7 developed recurrent seizures. Of the 20 children who did not have the syndrome, none showed later evidence of CNS dysfunction. Somnolence may be an early indicator of long‐term neurological sequelae after cranial irradiation.
The dissociation of cerebral blood flow, metabolism, and function in the early stages of developing cerebral infarction
Authors: Kogure K. , Busto R. , Schwartzman R. J. , Scheinberg P.
Published: January 2005

Temporal and site correlation of local cerebral blood flow (l‐CBF), tissue redox state, energy metabolism, tissue pH, and cerebral electrophysiological activity in induced cerebral ischemia was performed in rats in an effort to obtain helpful clues for the management of occlusive cerebrovascular disease. CBF decreased acutely in both the embolized and nonembolized hemispheres but returned toward normal in 5 minutes. However, total cerebral oxidative metabolism remained depressed throughout the 30‐minute observation period despite improved perfusion. The change in CBF correlated with the development and resolution of tissue acidosis, which was maximal 3 minutes after embolization but became alkaline after 30 minutes, possibly due to accumulation of sodium lactate. Oxidized form of nicotinamide‐adenine dinucleotide and cytochrome a,a3 quickly became reduced in the ischemic core, but a tardy, spontaneous postischemic tissue perfusion resulted in their hyperoxidation. The CBF‐metabolism uncoupling as well as postischemic hyperoxidation of the electron transport system, which is associated with accumulation of pyruvate and lactate, probably resulted from stagnation of electron flow at the entrance to the mitochondrial respiratory processes. Seizures could not account for these results, as paroxysmal changes in the EEG usually appeared only in the nonembolized hemisphere and were not dependent upon lack of energy.

These studies confirm that metabolic failure may persist in ischemic tissue despite adequate reperfusion, which may, in fact, contribute to tissue damage through hyperoxidation.
Cerebral asymmetry and verbal intellectual deficits
Authors: Rosenberger Peter B. , Hier Daniel B.
Published: January 2005

In a group of 53 children and young adults with learning disability, reversed cerebral asymmetry as measured by CT scan correlated highly with the prevalence, and to some extent with the degree, of verbal intellectual deficit as measured by age‐appropriate Wechsler Intelligence Scales. A history of delayed speech also correlated with reversed asymmetry but was not associated with a greater degree of verbal intellectual deficit among the reversedasymmetry cases.
Dysconjugate gaze in hepatic coma
Authors: Caplan Louis R. , Scheiner David
Published: January 2005

During an episode of hepatic encephalopathy, an elderly woman developed ocular divergence at rest and dysconjugate gaze after caloric stimulation. Metabolic encephalopathy may rarely produce oculomotor dysfunction, usually attributed to structural brainstem lesions.
Membrane studies in Huntington's disease: Steady‐state fluorescence studies of intact erythrocytes
Authors: Pettegrew Jay W. , Nichols John S. , Malcolm Stewart R.
Published: January 2005

Recent evidence suggests that a membrane abnormality may be present in Huntington's disease (HD), including peripheral tissues such as erythrocytes. Steady‐state fluorescence spectroscopy was performed on intact erythrocytes from patients with HD and from normal age‐ and sex‐matched controls. Fluorescent probes with known specificity for certain membrane areas were used to survey membrane dynamics; these probes included fluorescamine, which binds to primary amines on the cell surface, and both 12(9)‐anthroyl stearate, 12(9)AS, and 2(9)‐anthroyl stearate, 2(9)AS, which insert at different depths in the hydrocarbon core of membranes.

In the intact HD erythrocyte, fluorescamine revealed decreased polarization, 12(9)AS showed increased polarization, and 2(9)AS showed no difference as compared to normal erythrocytes. These studies suggest that fluorescamine, attached to primary amines of the cell surface in intact HD erythrocytes, has increased rotational mobility whereas the anthroyl moiety of 12(9)AS in the hydrocarbon interior has decreased rotational mobility. These studies thus support the concept of a membrane abnormality in HD.
Occipital horn asymmetry in children
Authors: Strauss Esther , Fitz Charles
Published: January 2005

In 75 young neurological patients ranging in age from 5 months to nearly 18 years, the left occipital horn was longer than the right in 29 instances whereas the right was longer than the left in only 13 cases (p < 0.025). Elongations of the right occipital horn as well as symmetrical horns were associated with cerebral damage incurred prior to the end of the first year of life (p = 0.06).
Sydenham's chorea: A possible presynaptic dopaminergic dysfunction initially
Authors: Naidu Sakkubai , Narasimhachari N.
Published: January 2005

A 10‐year‐old white girl showed chorea with elevated antibody titers indicative of an earlier group A streptococcal infection. Prior to treatment, cerebrospinal fluid homovanillic acid levels were markedly elevated. Haloperidol caused minimal improvement. She was then treated with reserpine alone, which resulted in remarkable improvement within one week. A repeat lumbar puncture one month after reserpine was started showed moderate reduction of homovanillic acid levels. When reserpine was discontinued, symptoms recurred. The data suggest presynaptic dopaminergic overactivity.
Meningeal gliomatosis: A review of 12 cases
Authors: Yung Wai‐Kwan A. , Horten Bruce C. , Shapiro William R.
Published: January 2005

Diffuse or multifocal invasion of the leptomeninges by malignant glioma (meningeal gliomatosis) is believed to be rare. From 1971 through 1977, 11 of 52 patients with intracranial malignant gliomas examined at autopsy were found to have meningeal gliomatosis, and 1 additional patient was diagnosed clinically without autopsy (12 cases total). Eight of the 12 patients were diagnosed antemortem with positive cerebrospinal fluid (CSF) cytology, while the other 4 patients were diagnosed at autopsy only. All 11 autopsied patients had multifocal or diffuse meningeal tumor distant from the primary site; 8 patients had spinal subarachnoid seeding with tumor encroachment of cauda equina and spinal nerve roots, and 9 patients had tumor invasion into the lateral ventricles. Three patients had symptomatic spinal cord compression at the thoracic or lumbar level, and 10 patients had hydrocephalus.

These 12 patients with meningeal gliomatosis were compared with the other 41 autopsied malignant glioma patients without the complication (controls); the patients with meningeal gliomatosis were significantly younger (mean age, 40 versus 57 years; p < 0.005). Patients with meningeal gliomatosis lived somewhat longer (median, 49 weeks) compared to controls (35 weeks), but the difference was not statistically significant. With the advance of chemotherapy, patients with malignant glioma are living longer and the incidence of meningeal gliomatosis may rise. The diagnosis of meningeal gliomatosis can be suspected, especially if hydrocephalus is present, and can often be confirmed by CSF cytology.
Analysis of leukocyte adherence to measles‐infected cells in multiple sclerosis
Authors: Barna B. P. , Goren H. , Jacobs B. , Conomy J. , Deodhar S. D.
Published: January 2005

An assay for leukocyte adherence to measles‐infected cells (MIC) was evaluated in 56 patients with multiple sclerosis (MS), 37 normal subjects, 29 patients with nonneurological diseases, and 57 patients with other neurological diseases. In the assay, rosettes were formed consisting of three or more leukocytes adherent to one MIC. Two aspects of the rosetting phenomenon were analyzed: (1) quantity of rosettes and (2) quantity of leukocytes contained in the rosettes. By both criteria significant differences (p < 0.05) were observed between MS patients and subjects in other groups, but data sets overlapped extensively. Results in MS patients were variable; some MIC lines produced more leukocyte adherence than did others, even though all lines had surface measles antigen present. A low ENgree of leukocyte adherence to uninfected cell lines was also observed frequently in MS. Exposure of MS leukocytes to extracts of MIC or oligodendrolioma tissue prior to the rosette assay significantly (p < 0.05) ENcreased rosette formation. These data suggest that leukocyte adherence to target cells in MS is partially dependendent on target cell expression of ENterminants, which may resemble glial antigens.
Essential tremor treated with propranolol: Lack of correlation between clinical effect and plasma propranolol levels
Authors: Sørensen Per Soelberg , Paulson Olaf B. , Steiness Eva , Jansen Erik C.
Published: January 2005

Five patients with essential tremor were treated with increasing daily doses of propranolol. Tremor intensity was assessed after each propranolol dose had been given daily for a period of at least seven days. The evaluation was made 12 to 15 hours after the last propranolol ingestion. It include a semiquantitative clinical tremor evaluation and quantitative registration with an accelerometer. Electronic integration of the accelerometer curves gave an arbitrary measure of tremor intensity. Plasma propranolol concentration was determined at each tremor measurement.

The reduction in tremor correlated with increasing propranolol doses, whereas plasma propranolol concentrations varied among individuals and could not be correlated with tremor reduction. Most of the patients had considerable diminution in tremor at low propranolol doses, often with an unmeasurable plasma propranolol concentration.

Determination of plasma concentrations has little value in long‐term treatment of essential tremor with propranolol. The dosage can be sufficiently guided by clinical evaluation of effect.
Parkinsonism provoked by alcoholism
Authors: Carlen P. L. , Lee M. A. , Jacob M. , Livshits O.
Published: January 2005

Seven chronic alcoholics, aged 53 to 70, ENmonstrated transient signs of parkinsonism provoked by alcohol withdrawal or chronic severe intoxication. All showed improvement or recovery when they abstained or ENcreased their alcohol intake for several days to weeks. Animal studies have ENmonstrated impaired striatal dopaminergic function during severe ethanol intoxication or withdrawal. Chronic alcoholism apparently can exacerbate or uncover latent central dopaminergic ENficiency.
Spinocerebellar engeneration with parkinsonian features: A clinical and pathological report
Authors: Weir Roger L. , Fan Kuang J.
Published: January 2005

A 20‐year‐old patient had spinocerebellar ENgeneration and parkinsonian tremor that improved with L‐dopa treatment. Postmortem examination revealed posterior column atrophy in the spinal cord and severe ENgeneration in the substantia nigra. Atrophic changes were noted in ENep cerebellar nuclei and superior cerebellar peduncles. This study confirms the relationships between clinical parkinsonism, substantia nigra atrophy, and the therapeutic effect of L‐dopa.
Experimental viral infections of the facial nerve and geniculate ganglion
Authors: Davis Larry E.
Published: January 2005

Although the cause of Bell's palsy is in most cases unknown, a number of viral infections have been associated with the disease. To determine if human viruses could experimentally infect the facial nerve or ganglion, six different viruses were inoculated into newborn hamsters by intracerebral, intralabyrinthine, or intraperitoneal routes. The facial nerve within the temporal bone and the geniculate ganglion were studied by light and immunofluorescent microscopy for the presence of viral infection. Herpes simplex, rubeola, mumps, and reovirus infected the neurons in the geniculate ganglia, while vaccinia virus infected the stroma cells of the facial nerve and ganglia. Influenza virus did not infect either the nerve or ganglia. These studies indicate that several human viruses can experimentally infect the facial nerve and geniculate ganglia, lending support to the hypothesis that Bell's palsy is caused by a virus.
Ultrastructure of tuberous sclerosis: Cortical tuber and subependymal tumor
Authors: Trombley Irene K. , Mirra Suzanne S.
Published: January 2005

Ultrastructural examination of two biopsy cases of tuberous sclerosis, a subependymal tumor and a cortical tuber, revealed giant cells with astrocytic features in both cases. These included multipolar processes containing glial filaments, glycogen, and membrane‐bound dense bodies, as well as formation of hemidesmosomes with pia and vascular basement membranes. Megamitochondria were also seen within glial processes. In addition to the numerous glial‐glial contacts encountered in both cases, rare neuroglial junctions suggesting aberrant synapse formation were observed within the cortical tuber. This observation, in concert with the organization of multiple small neurites along large, aberrant astrocyte processes found within the tuber, mimics features of neuroembryonic development described in lower mammals and nonhuman primates. Such a parallel in ultrastructural morphology supports a primarily dysplastic nature for the lesions in tuberous sclerosis.
Binaural interaction in brainstem potentials of human subjects
Authors: Levine Robert A.
Published: January 2005

Binaural interaction in the short‐latency averaged auditory evoked potentials (AEPs) can be assessed from the binaural difference waveform (BD). The BD is derived by computing the difference between the AEP evoked by simultaneous clicks from both earphones and the sum of two other AEPs: one evoked by clicks from the right earphone alone and the other evoked by clicks from the left earphone alone. Once the contributions of acoustic cross talk and the middle ear reflex are eliminated, the BD can be considered to represent neural binaural interaction. This interaction begins after wave III and has its first major peak during the downslope of wave V. The relative amplitude of this peak to wave V is constant over a wide range of click rates and levels. The BD may have more localizing value than the AEPs alone since the BD probably represents a subpopulation of generators of the AEPs, which show binaural interaction at the single cell level.
Ataxic hemiparesis from a midbrain mass
Authors: Bendheim Paul E. , Berg Bruce O.
Published: January 2005

A 10‐year‐old boy with leukemia developed ipsilateral corticospinal and cerebellar signs: the ataxic hemiparesis syndrome. Computed tomography revealed a mass lesion in the contralateral rostral midbrain. Previous reports of this syndrome placed the lesion in the basis pontis. The anatomical substrate of the ataxic hemiparesis syndrome includes the basis pontis, rostral midbrain, and possibly other areas within the central nervous system.
Methioninemia and myopathy: A new disorder
Authors: Gaull Gerald E. , Bender Adam N. , Vulovic Dimitrije , Tallan Harris H. , Schaffner Fenton
Published: January 2005

A 7½‐year‐old girl with hypermethioninemia, myopathy, and mental deficiency (IQ = 65) is described. The increased methionine was not associated with deficiency of methionine adenosyltransferase, which was normal or increasedin liver, muscle, erythrocytes, and cultured fibroblasts. Methionyl‐tRNA synthetase in fibroblasts was normal. The hypermethioninemia and a concurrently increased blood S‐adenosylmethionine declined on a diet low in methionine. There was a diffuse, symmetrical, moderate proximalmuscle weakness, but muscle atrophy was not discernible, and the deep tendon reflexes were hypoactive but obtainable. Electromygraphic abnormalites were not detected. Electron microscopy of muscle revealed 3 to 6 small myelin figures in the region of the I band in nearly every fiber, with occasional myelin figures at other sites also. These myelin figures were more numerous and smaller than those seen accompanying nonspecific and may reflect a more specific pathological change. Electron microscopy of liver revealed three nonspecific lesions in all hepatocytes: (1) numerous megamitochondria with crystalloid deposit in the matrix; (2) increased numbers of small vesicles of smooth endoplasmic reticulum; and (3) loss of plasma membrane microvilli, with extensive bleb for formation and shedding of eytoplasm into Disse's space.
The ocular motor system
Authors: Spector Robert H. , Troost B. Todd
Published: January 2005

Recent advances in ocular motor neurophysiology are reviewed with emphasis on brainstem control of horizontal saccadic and pursuit eye movements. Certain aspects of the control of vertical gaze and the role of the cerebellum and medial longitudinal fasciculus in normal and pathological states are also discussed. Current nomenclature and concepts are clarified so that they may be applied to the understanding of clinical ocular motor phenomenology.
Saccadic eye movement strategies in patients with homonymous hemianopia
Authors: Meienberg Otmar , Zangemeister Wolfgang H. , Rosenberg Michael , Hoyt William F. , Stark Lawrence
Published: January 2005

Infrared oculographic recordings from three patients with hemianopia due to an occipital lesion showed that these patients employed a consistent set of (presumably unconscious) compensatory strategies to find and fixate objects. For targets in the blind hemifield, patients at first used a staircase strategy consisting of a series of stepwise saccadic search movements. This is safe but slow. When retested later, one patient had adopted a more efficient strategy employing one large saccadc calculated to overshoot the target. Other strategies for finding targets in the blind hemifield were employed in response to specific situations presented by our experiments: a predictive strategy using past experience to anticipate where the target would be found, and special strategies for recovering a lost target and for awaiting the reappearance of the target. To fixate targets in the seeing hemifield, our subjects undershot the target to prevent losing it in the blind hemifield, then held it off‐fovea on the seeing side of the macula.
Characterization of immune complexes in progressive rubella panencephalitis
Authors: Coyle P. K. , Wolinsky Jerry S.
Published: January 2005

In two patients with the slow virus disease progressive rubella panencephalitis, high levels of immune complexes were found in serum and, in one case, spinal fluid. These complexes contained immunoglobulin G. Serum complexes sedimented over a wide density range compared with the more homogeneous spinal fluid complexes. Complexes from both serum and spinal fluid contained antibody directed against rubella virus. Serum complexes were also shown to contain rubella antigen (or antigens) that appeared to differ between the two patients.
Polyneuropathy with monoclonal gammopathy: Studies of 11 patients
Authors: Dalakas Marchinos C. , Engel W. King
Published: January 2005

Among patients with chronic idiopathic nonfamilial polyneuropathy studied 3 to 21 years after onset, we identified 11 cases associated with monoclonal gammopathy (MCG) (5 with IgGK, 4 with IgMK, 2 with IgMλ). The patients, aged 29 to 80 years, presented with sensorimotor polyneuropathy of insidious onset and slow, nonfluctuating progression, delayed motor and sensory nerve conduction, and increased cerebrospinal fluid (CSF) protein. None of the patients in the initial or follow‐up study (mean, 9.2 years) had or developed signs of multiple myeloma, malignant plasma cell dyscrasia, macroglobulinemia syndrome, amyloidosis, neoplasia, or other associated illness.

The CSF revealed abnormalities of protein electrophoresis or immunoelectrophoresis in 9 of the 11 patients. Three of 5 sural nerve biopsies studied by immunofluorescence demonstrated deposition in nerve fibers of the light chain characteristic of the abnormal circulating immunoglobulin. The findings suggest that these patients form a distinct subset of the dysimmune neuropathies. Although the immunoglobulin deposition and abnormal protein patterns in serum and CSF could be secondary to nerve damage, we propose that an immunopathological mechanism underlies the neuropathy. Immunosuppressants had minimal to marked beneficial effect in 4 of 5 patients, indicating that this polyneuropathy is potentially treatable.
Computed tomography in the diagnosis of Canavan's disease
Authors: Rushton Alan R. , Shaywitz Bennett A. , Duncan Charles C. , Geehr Robert B. , Manuelidis Elias E.
Published: January 2005

Computed tomography (CT scan) demonstrated a symmetrical decrease in white matter attenuation of the cerebral hemispheres of two young children with macrocephaly and normal neurological examination. Subsequent developmental delay led to brain biopsy, which documented Canavan's disease (spongy degeneration of the brain, Van Bogaert‐Bertrand type). The CT scans obtained from these patients with proved Canavan's disease appeared to be quite characteristic in differentiating this disorder from Alexander's disease and adrenoleukodystrophy (Schilder's disease). The CT scan may decrease the necessity for diagnostic brain biopsy in these white matter disorders.
Physiological observations in the median nerve during carpal tunnel surgery
Authors: Yates S. K. , Hurst L. N. , Brown W. F.
Published: January 2005

In entrapment of the median nerve in the carpal tunnel, it has been claimed that release of the flexor retinaculum is accompanied by immediate improvement in conduction time across the caarpal tunnel segment. We observed no such immidiate changes in conduction time intraoperatively in eight subjects following retinaculum release or neurolysis. Neurolysis led to conduction block in some median nerves, cating doubt on the value of that procedure.
Myeloma protein synthesis within the CNS by plasma cell tumors
Authors: Siegal Tali , Shorr Joshua , Lubetzki‐Korn Isabelle , Soffer Dov , Naparstek Elizabeth , Tur‐Kaspa Ron , Abramsky Oded
Published: January 2005

Two patients had centrl nervous system involvement from plasma cell tumor, onr with leptomeningeal infiltraion and the other with solitary intracranial plasmacytoma. In both, cerebrospinal fluid (CSF) electrophoresis revealed IgG index consistent with local synthesi of the myeloma protein. the CSF findings were helpful in the diagnosis and evaluation of tumor recurrence.
SSPE, measles virus, and the matrix protein: Report of a case with unusual immunochemical findings
Authors: Mandelbaum David E. , Hall William W. , Paneth Nigel , Wolff Robert R. , DeVivo Darryl C.
Published: January 2005

We report a case of atypical subacute sclerosing panencephalitis (SSPE) in which the diagnosis was confirmed by a new radioimmunoprecipitation method. Antimeasles antibody was absent in the cerebrospinal fluid (CSF) when measured by conventional immunoassay techniques, there was no clinical response to isoprinosine, and the patient showed a selective absence of CSF antibody response to the matrix protein and only a partial serum antibody response to this measles polypeptide. The value of this sensitive immunoprecipitation method in profiling the selective antibody responses to the several measles polypeptides and the advisability of using isoprinosine, an immunopotentiating agent, to treat SSPE are discussed.
Instrumental neutron activation analysis of brain aluminum in Alzheimer disease and aging
Authors: Marchkesbery William R. , Ehmann William D. , Hossain T. I. M. , Alauddin Mohammad , Goodin Daniel T.
Published: January 2005

Instrumental neutron activation analysis procedures were used to determine the aluminum content of various brain regions in histologically verified Alzheimer disease (AD) and in controls. The grand mean aluminum level for 74 AD specimens was 0.372 ± 0.058 μg/gm and for 137 adult controls, 0.467 ± 0.033 μg/gm, both on a wet weight basis. No difference was found at the bulk sample level between AD and adult controls, corrected for age and sex, or when frontal, temporal, and hippocampal specimens were compared. Control specimens (infancy to 85 years) showed an increase in brain aluminum concentration with age. Comparison of freez‐dried to wet weight ratios of AD and controls revealed a small increase in water content in AD brains.
Multiple sclerosis: Heterology among early T cells and TG cells
Authors: Traugott Ute , Scheinberg Labe C. , Raine Cedric S.
Published: January 2005

Circulating early T cells and TG cells were estimated simultaneously in 100 patients with multiple sclerosis and 21 controls. The results were analyzed from individual patients, from patients grouped according to disease activity, and from some patients studied longitudinally. Previously documented fluctuations in these two T cell populations were confirmed. By comparing the fluctuations and by examining the distribution of TG cells in suspensions of isolated early and isolated late T cells, it was found that TG cells are a T cell subpopulation distinct from early T cells.
Brainstem auditory evoked responses and alpha‐patten coma
Authors: Hari Riitta , Sulkava Raimo , Haltia Matti
Published: January 2005

A young comatose man with an alpha‐pattern electroencephalogram is reported. The brainstem auditory evoked responses were severely abnormal, suggesting a pontomesencephalic lesion which was confirmed at autopsy.
D‐lactic acidosis simulating a hypothalamic syndrome after bowel bypass
Authors: Carr Daniel B. , Shih Vivian E. , Richter James M. , Martin Joseph B.
Published: January 2005

A 36‐year‐old man had one year of periodic symptoms suggestive of episodic hypothalamic dysfunction: hypersomnia, thirst, ravenous hunger and gorging behavior, pallor, and irritability. However, neuroendocrine testing proved normal. A mild transient acidosis at the onset of his attacks and a history of bowel bypass five years earlier prompted metabolic screening. Markedly increased urinary D‐lactic and phenolic acids were present, as were intermittent elevations of plasma D‐lactic acid during two symptomatic episodes. Prompt and sustained clinical remission coincided with disappearance of abnormal organic acid excretion during oral antibiotic therapy. D‐Lactic acidosis must be considered in the differential diagnosis of otherwise unexplained neurological syndromes, particularly in patients with altered bowel anatomy.
Multiple sclerosis: Trial of a synthetic polypeptide
Authors: Bornstein M. B. , Miller A. I. , Teitelbaum D. , Arnon R. , Sela M.
Published: January 2005

A synthetic polypeptide, copolymer I (COP I), composed of alanine, glutamic acid, lysine, and tyrosine, has been demonstrated to be nonecephalitogenic and nontoxic in laboratory animals, yet it is capable of suppressing experimental allergic encephalomyelitis. A preliminary open trial examined the ability of COP I to alter the course of disease in 12 patients with chronic progressive and 4 with exacerbating‐remitting multiple sclerosis (MS). After therapy for as long as two years or more, no undersirable side reaction was noted in any patient. Three patients with chronic progressive MS and 2 with exacerbating‐remitting disease are better. These results, which may represent simply a placebo effect or may be a significant response, are now being examined in randomized, placebo‐controlled, double‐blind pilot trials.
Generalized paroxysmal fast activity: Electroencephalographic and clinical features
Authors: Brenner Richard P. , Atkinson Ruth
Published: January 2005

We reviewed electroencephalographic and clinical features of 20 patients with generalized paroxysmal fast activity (GPFA). All had seizure disorders and all but 3 had more than one seizure type. Mental retardation was present in 17 patients. In most cases no cause for the seizures could be found.

GPFA occurred almost exclusively in sleep. Fifteen patients had clinical seizures associated with this activity; in 8 the seizures were tonic. Additional generalized epileptiform abnormalities were present in 18 patients, 10 of whom had slow spike and wave complexes. Only 4 patients had normal background activity.
Dopaminergic deficiency causes delayed visual evoked potentials in rats
Authors: Onofrj Marco , Bodis‐Wollner Ivan
Published: January 2005

Flash and pattern visual evoked potentials (VEPs) to two temporal frequencies of stimulation were studied in nineteen rats. The effect of a tyrosine hydroxylase inhibitor and of a dopamine receptor blocker on the VEP was explored in ten animals. Significant latency change occurred following injection of either drug, while only the hydroxylase inhibitor reduced the VEP amplitude. These changes were not caused by the anesthesia used in these experiments, although the same anesthetics in higher doses did depress VEP amplitudes. When dopamine blockade was followed by administration of apomorphine, a dopamine agonist, VEP delays could be partially reversed. Besides conduction defects of myelinated axons, synaptic malfunction may also cause delays in sensory evoked potentials.
Kuru with incubation periods exceeding two decades
Authors: Prusiner Stanley B. , Gajdusek D. Carleton , Alpers Michael P.
Published: January 2005

The clinical characteristics of kuru in 15 patients are described. All the patients had a history of joint pains preceding difficulty walking. The severity of the neurological dysfunction varied from mild truncal and limb ataxia necessitating the use of a stick for walking to terminal illness accompanied by marked ataxia, rigidity, spasticity, and dementia. All the patients with kuru in this study had an apprehensive, frightened facial expression. Most of the patients examined showed diminished or absent optokinetic nystagmus bilaterally. Apprehensive facies and diminished optokinetic nystagmus have not previously been described in kuru. The other clinical features of the current patients with kuru are similar to those recorded twenty years ago. Epidemiological surveillance, anamnesis, and missionary reports strongly suggest that all the patients described in this study were exposed to the kuru agent more than two decades ago through ritualistic cannibalism. Thus the incubation period or time interval from exposure to the onset of clinical illness exceeds two decades, while the duration of illness is two years or less. The cause of these extraordinarily long incubation periods is unknown but may result in part from exposure to small doses of the kuru agent through an inefficient oral route.
Misonidazole neuropathy: A clinical, electrophysiological and histological study
Authors: Melgaard B. , Hansen H. Sand , Kamieniecka Z. , Paulson O. B. , Pedersen A. Gersel , Tang Xiaofu , Trojaborg W.
Published: January 2005

We studied eight patients with carcinoma of the pharynx and larynx (five cases) or lungs (three cases) who, during treatment with the radiosensitizing drug misonidazole, developed peripheral neuropathy dominated by severe sensory symptoms and signs mainly localized to the lower extremities. The symptoms partially subsided within months after cessation of therapy. Electrophysiological and histological findings indicated an axonal neuropathy with loss of large fibers and secondary demyelination. The neurotoxic property of misonidazole limits its therapeutic use.
The laboratory diagnosis of mild myasthenia gravis
Authors: Kelly John J. , Daube Jasper R. , Lennon Vanda A. , Howard Frank M. , Younge Brian R.
Published: January 2005

We report our experience with single‐fiber electromyogrphy (SF‐EMG), acetylcholine receptor antibody assays, and Lancaster Red‐Green tests of ocular motility with edrophonium chloride in the diagnosis of myasthenia gravis (MG). Over a period of almost 3 years, 43 patients in whom repetitive‐stimulation nerve conduction tests were nondiagnostic had SF‐EMG studies; 34 (79%) were positive. Examination of more proximal muscles yielded a higher likelihood of positive results. Anti‐acetylcholine receptor antibodies were detected in 25 (71%) of 35 patients whose sera were assayed, and Lancaster Red‐Green tests of ocular motility were positive in 13 (81%) of 16 patients studied. No single test was superior in diagnosing MG, but the three were complementary. If patients with mild MG are tested by all three methods, at least 95% should have laboratory confirmation of MG.
Sleep‐induced nonobstructive hypoventilation associated with diaphragmatic paralysis
Authors: Thorpy Michael J. , Schmidt‐Nowara Wolfgang W. , Pollak Charles P. , Weitzman Elliot D.
Published: January 2005

A patient with adult‐onset spinal muscular atrophy causing mild proximal limb muscle weakness had sleep‐induced nonobstructive hypoventilation due to diaphragmatic paralysis. Nocturnal dyspnea and daytime somnolence were associated with frequent arousals from sleep due to arterial oxygen desaturation. The nocturnal use of a cuirass respirator improved the oxygen saturation, resulting in less disrupted sleep, relief of the somnolence, and improved daytime ventilation.
Involuntary movements in chorea‐acanthocytosis: A comparison with Huntington's chorea
Authors: Shibasaki Hiroshi , Sakai Tetsuo , Nishimura Hiroyuki , Sato Yuji , Goto Ikuo , Kuroiwa Yoshigoro
Published: January 2005

The involuntary movements in three patients with chorea‐acanthocytosis (CA) and three with Huntington's chorea (HC) were studied by polygraphic recordings and jerk‐locked averaging technique. Choreic movements in patients with CA were suppressed by mental calculation, voluntary muscle contraction, and instruction to stop them, whereas movements in HC were generally enhanced by these tasks. Backward averaging of the EEG time‐locked to the choreic movement of an extremity or the trunk demonstrated a cortical slow negativity before the movement, similar to the Bereitschaftspotential (“readiness potential”), in cases of CA but not those of HC. Involuntary movements in CA appear to differ from those in HC and might be generated at least in part by a mechanism similar to that of voluntary movement.
Childhood brain tumors presenting as chronic uncontrolled focal seizure disorders
Authors: Blume Warren T. , Girivin John P. , Kaufmann John C. E.
Published: January 2005

Sixteen of 35 patients (46%) 21 years old or less who underwent surgical resection of a longstanding epileptic focus harbored tumors. The median duration of the seizure disorder prior to operation was 6.0 years for patients with and 7.6 years for those without tumor. Among the 35 patients, tumor was more common when intelligence and results of neurological examination were each normal, a plausible cause for uncontrolled seizures was lacking, and persistent focal delta activity occurred in a majority of electroencephalograms (EEGs). The type of seizures and the distribution of EEG spikes failed to distinguish patients with tumor from those without. Multifocal EEG spikes appeared in a majority of each group.
Toxic effect of phenytoin on developing cortical neurons in culture
Authors: Swaiman Kenneth F. , Neale Elaine A. , Schrier Bruce K. , Nelson Phillip G.
Published: January 2005

Studies were undertaken to determine the effect of chronic phenytoin exposure on developing neurons. Cerebral cortex from 16‐day fetal mice was utilized to prepare primary dissociated cell cultures. Phenytoin weas added to the cultures 10 days after plating and the cultures were harvested on day 17. Cortical cultures were assayed for neuronal cell number by phase microscopy and for high‐affinity uptake of 3H‐labeled γ‐aminobutyric acid (GABA) by both radioautography and scintillation spectrometry.

Neuronal cell counts demonstrated a highly significant decrement in the number of neurons in cultures exposed to phenytoin at 15, 25, and 50 μg/ml. 3H‐GABA‐labeled neurons constituted 13% of the neurons present in both control and phenytoin‐exposed cultures.

These data indicate that phenytoin is toxic to cortical neurons in culture and that GABAergic neurons are affected to the same extent as the total neuronal population.
Serum mitochondrial aspartate aminotransferase in patients with polymyositis
Authors: Ogasahara Saburo , Takahashi Mitsuo , Kang Jin , Yorifuji Shiro , Wada Keiji , Hazama Takanori , Takeuchi Hiroaki , Tarui Seiichiro
Published: January 2005

The clinical significance of serum aspartate aminotransferase (GOT) isozymes was studied in 18 patients with polymyositis. Abnormally high levels of mitochondrial GOT (mGOT) (6.2 ± 1.2 IU/L, mean ± SEM; normal, <2.0 IU/L) and cytosol GOT (sGOT) (95 ± 21.6 IU/L; normal, <25 IU/L) were observed in sera. In polymyositic muscles, the sGOT level was significantly decreased but mGOT was not. The levels of serum sGOT and mGOT and the ratio of mGOT/tGOT before corticosteroid therapy correlated well with the severity of muscle weakness. Serial determination of CPK, sGOT, and mGOT during corticosteroid therapy revealed that mGOT most rapidly returned to normal. Exercise did not increase serum mGOT in polymyositis.
A dominantly inherited syndrome with continuous motor neuron discharges
Authors: Ashizawa Tetsuo , Butler Ian J. , Harati Yadollah , Roongta Suresh M.
Published: January 2005

A distinct syndrome with continuous motor neuron discharges apparently developed in seven members of a single family, involving both sexes and spanning three generations. Persistent vermiform twitching and episodic stiffness predominantly in lower extremity muscles occurred in early childhood and tended to be less severe in adulthood. In 2 patients the clinical manifestations improved with oral phenytoin and carbamazepine but not with parenteral diazepam. Insertional activity was normal, and continuous, rhythmical, normal‐appearing muscle discharges were observed on electromyography. The cerebrospinal fluid levels of homovanillic acid and 5‐hydroxyindoleacetic acid were increased in the proband. The disappearance of continuous muscle discharges during spinal anesthesia and the lack of response to diazepam indicated generation of the discharges from the proximal portion of the motor unit.
Neuromuscular dysfunction and ultrastructural pathology in children with chronic cholestasis and vitamin E deficiency
Authors: Werlin Steven L. , Harb Joseph M. , Swick Herbert , Blank Ellen
Published: January 2005

The ultrastructural pathology of nerve and muscle and the neurological dysfunction in children with cholestatic liver disease and vitamin E deficiency have not been previously correlated. We studied two children with this syndrome. One child, 11 years of age, had severe hyporeflexia and decreased vibratory sense. Nerve conduction was delayed. The second child, 2 years of age, was neurologically normal.

Both children showed ultrastructural evidence of damage to the sural nerve and accumulation of electron‐dense deposits in the muscle fibers. Abnormalities of the nerves included disruption of the myelin sheath and separation and degeneration of the inner and outer components of the Schmidt‐Lanterman incisure.
Removal of sedative‐hypnotic antiepileptic drugs from the regimens of patients with intractable epilepsy
Authors: Theodore William H. , Porter Roger J.
Published: January 2005

Sedative‐hypnotic antiepileptic drugs have potentially toxic effects, but their removal is often thought to be difficult and dangerous. We completely withdrew all barbiturates and benzodiazepines from 78 patients with intractable epilepsy (48 inpatients and 30 outpatients). Initially, 19 patients had plasma levels of sedative drugs above the therapeutic range; 28 were taking more than one of these drugs. Dosages of nonsedative antiepileptic drugs were adjusted to provide optimal seizure control.

After 6 months of outpatient follow‐up, 69 patients remained on a nonsedative regimen: 35 (51%) showed improvement in both drug toxicity and seizure control, 13 (19%) in toxicity alone, 8 (12%) in seizure control alone; 12 (16%) were unchanged, and 1 was worse. Of 9 patients restarted on sedative antiepileptic drugs by their private physicians, 4 had more toxic symptoms than at discharge, 1 had more frequent seizures, 3 were unchanged, and 1, who had had a temporal lobectomy after drug withdrawal, had less frequent seizures.

Sedative drugs are not necessary for optimal seizure control, even in intractable epilepsy, and they may be safely withdrawn.
Disease activity and emotional state in multiple sclerosis
Authors: Dalos Nancy P. , Rabins Peter V. , Brooks Benjamin R. , O'Donnell Pat
Published: January 2005

The prevalence and nature of emotional disturbance in patients with multiple sclerosis (MS) was studied prospectively in 64 MS patients and 23 spinal cord–injured (SCI) control patients by administration of the 28‐item subscale General Health Questionnaire (GHQ). MS patients in remission had a mean GHQ score of 4.4, and patients with acute exacerbation or progressive nonremitting MS had a mean score of 15.7, a significant difference (p < 0.001). The prevalence of emotional disturbance was 90% in exacerbating or progressing patients, 39% in stable patients, and 12% in SCI control patients. The presence of emotional disturbance was not related to age, sex, or other demographic variables, to duration or severity of disease, or to the degree of disability. In the group of MS patients in remission, somatic complaints, anxiety, and social dysfunction were more prevalent than symptoms of depression.
Long‐term prognosis in two forms of childhood epilepsy: Typical absence seizures and epilepsy with rolandic (centrotemporal) EEG foci
Authors: Loiseau P. , Pestre M. , Datigues J. F. , Commenges D. , Barberger‐Gateau C. , Cohadon S.
Published: January 2005

Findings in children seen between 1955 and 1965 during the year of onset of typical absence seizures (90 patients) or rolandic epilepsy (79 patients) were analyzed by actuarial methods. One hundred and eighteen patients were followed for more than 15 years. Rolandic epilepsy is a true benign epilepsy ending with puberty. Although school and family problems are common during the acute stage of the disease, the social adaptability of such patients is excellent. We considered only typical absences occurring as a first epileptic sign in normal children. Myoclonic or atonic absences have a poor prognosis. Many patients with simple and automatic absences experience remission 15 years after withdrawal of medication. The overall cessation rate in those experiencing absences was only 57.5%, however, and 36% of patients developed tonic‐clonic seizures. Social adaptability was often inadequate. Simple and automatic absences (constituting a homogeneous group) are not truly a benign form of epilepsy, even though prognosis for those afflicated is better than that for those with other forms of primary generalized epilepsy.
Evidence that lack of deoxyribonucleic acid repair causes death of neurons in xeroderma pigmentosum
Authors: Robbins Jay H. , Polinsky Ronald J. , Moshell Alan N.
Published: January 2005

Xeroderma pigmentosum (XP) is an autosomal recessive disorder with hypersensitivity to the lethal effects of ultraviolet radiation caused by inherited defects in deoxyribonucleic acid (DNA) repair processes. Some patients with XP develop a primary neuronal degeneration which has been thought to result from unrepaired damage in neuronal DNA. Five years ago we reported that cultured skin fibroblasts from a 12‐yearold girl with XP, who then had only one major neurological abnormality of the disease, had a sensitivity to ultraviolet radiation intermediate between that of XP patients with numerous neurological abnormalities and those with none. Recent neurological studies reveal that she has a slowly but progressively developing sensorineural deafness as well as cerebellar and motor dysfunction typical of XP. The results support the postulate that defective DNA repair is associated with premature neuron death.
Amyotrophic lateral sclerosis: Alterations in neurotransmitter receptors
Authors: Whitehouse Peter J. , Wamsley James K. , Zarbin Marco A. , Price Donald L. , Tourtellotte Wallace W. , Kuhar Michael J.
Published: January 2005

Loss of motor neurons is the primary pathological hallmark of amyotrophic lateral sclerosis. Drug and neurotransmitter receptors are neuronal markers and can be indicators of neuronal connectivity. Knowledge of alterations in receptors in amyotrophic lateral sclerosis should contribute to our understanding of normal spinal cord neurotransmitter systems as well as of the pathophysiology of amyotrophic lateral sclerosis. We therefore used a sensitive, light microscopic in vitro labeling receptor autoradiographic technique to map and quantitate muscarinic cholinergic, glycinergic, and benzodiazepine receptors in three levels of spinal cord from six patients with amyotrophic lateral sclerosis and six age and sex‐matched control patients. In control tissues, the receptor distributions were similar in the three levels of spinal cord and also similar to those found in previous studies with animals. In amyotrophic lateral sclerosis, major reductions in receptor densities were noted in Rexed layer IX, the region containing motor neurons. Reductions were noted in other laminae as well, particularly for muscarinic receptors. The changes in muscarinic receptors were caused solely by changes in high‐affinity agonist sites. Reductions in glycine and muscarinic receptors were highly correlated with the degree of motor neuron loss found in the amyotrophic lateral sclerosis patients. The findings in this study point out the usefulness of this receptor mapping technique in understanding the changes in neuronal populations that occur in the degenerative neurological diseases.
Limitations of ultrasound in detecting cerebral ischemic lesions in the neonate
Authors: Davis Suzanne L. Wilson , Lo Warren , Filly Roy A.
Published: January 2005

Ultrasound scans of three newborn infants with cerebral ischemic lesions demonstrated on computed tomographic scan showed unexpected changes in echogenicity of brain parenchyma in addition to lateralized mass effect. Areas of increased echogenicity were present adjacent to regions of infarction identified on computed tomographic scan. The findings suggest that caution should be exercised in the interpretation of echo‐dense areas in unusual locations for hemorrhage in infants at risk for cerebral ischemic lesions.