bluuebunny/crossref_metadata_2025 huggingface dataset:
Hydrocephalus caused by increased intracranial venous pressure: A clinicopathological study
Authors: Rosman N. Paul , Shands Kathryn N.
Published: January 2005

A child developed progressive communicating nonobstructive hydrocephalus as a result of increased intracranial venous pressure. The child had been treated for congenital heart disease at age 3 weeks by surgical creation of an anastomosis between his superior vena cava and right pulmonary artery. Although his cardiac symptoms were alleviated, intracranial venous hypertension resulted, giving rise to progressive head enlargement and other signs of hydrocephalus. Postmortem examination at age 3 years disclosed no other lesions that could have caused the hydrocephalus. Increased intracranial venous pressure can lead to either pseudotumor cerebri or hydrocephalus, the former in children 3 years or older, the latter in infants 18 months or less. Possible mechanisms accounting for these differences are discussed.
Nonsurgical cure of brain abscess: Early diagnosis and follow‐up with computerized tomography
Authors: Berg Bruce , Franklin Gary , Cuneo Richard , Boldrey Edwin , Strimling Bruce
Published: January 2005

Four patients with clinical findings and computerized tomographic brain scan evidence of brain suppurative disease were treated and cured with high‐dose intravenous antibiotics without neurosurgical intervention. More data are required before any statement can be made regarding the long‐term efficacy of nonsurgical versus surgical management of brain abscess.
Acetylcholine release in diaphragm of rats with chronic experimental autoimmune myasthenia gravis
Authors: Kelly John J. , Lambert Edward H. , Lennon Vanda A.
Published: January 2005

Recent evidence indicates that in chronic experimental autoimmune myasthenia gravis (EAMG) and in human myasthenia gravis, the defect of neuromuscular transmission results from immune‐mediated destruction of postsynaptic membrane at the neuromuscular junction, with a reduction in the density of acetylcholine (ACh) receptors and decreased sensitivity to ACh released by nerve impulses. In the present study, the amount of ACh released by nerve impulse in rats with chronic EAMG and control rats of the same age, weight, and sex was compared. Phrenic nerve–hemidiaphragm preparations were stimulated in vitro, and the amount of ACh released was meansured by bioassay. Despite a marked reduction in the amplitude of miniature end‐plate potentials in chronic EAMG, ACh output at rest and during stimulation was not different from that of control rats. These data support the concept that the defect of neuromuscular transmission is due to a reduction of postsynaptic sensitivity to ACh.
Tics and tourette's: A continuum of symptoms?
Authors: Golden Gerald S.
Published: January 2005

Analysis of the families of 39 unselected children with Tourette syndrome revealed other members with tic disorders in twenty kindreds. In eight families there were 13 individuals with chronic multiple tics, usually motor, not vocal. Twelve different families contained 18 subjects with Tourette syndrome other than the index patient. In three of these families there were 6 additional individuals with chronic motor tics, forming a bridge to the first group. An autosomal dominant mode of inheritance was suggested in all cases. Tourette syndrome and chronic motor tics appear to represent conditions along a continuum and have, in many instances, a hereditary basis.
Mixed intracranial sarcomas: Rare forms and a new association with previous radiation therapy
Authors: Averback P.
Published: January 2005

The first two instances of mixed sarcoma‐glioblastoma with a history of therapeutic irradiation to the head are reported, both occurring within one year of radiation therapy (for pituitary adenoma and residual meningioma). Two novel variants of mixed sarcomas of brain with extreme tumor metaplasia (fibromyxoosteochondrosarcoma and fibrochondroosteosarcoma‐glioblastoma multiforme) are documented, and some of the problems concerning the origin of brain tumors with mixed population are discussed.
Cranial computed tomography in the diagnosis of systemic lupus erythematosus
Authors: Gonzalez‐Scarano F. , Lisak Robert P. , Bilaniuk Larissa T. , Zimmerman Robert A. , Atkins Paul C. , Zweiman Burton
Published: January 2005

The cranial computed tomograms of 29 patients with systemic lupus erythematosus (SLE) were reviewed. Twenty‐two patients had a clinical course consistent with central nervous system involvement. Of these, 20 had abnormal CT studies during the course of their CNS symptoms. The most common finding was sulcal enlargement, either with or without ventricular enlargement, and it was prominent in patients with either psychosis or dementia. Infarcts and intracranial hemorrhages were seen as well. Seven CT studies were obtained in SLE patients without a clear diagnosis of CNS involvement. Only one of these was abnormal.
Voluntary motor commands in human ballistic movements
Authors: Desmedt John E. , Godaux E.
Published: January 2005

The integrated electromyogram of the first dorsal interosseous muscle in normal humans presents a brief early burst in voluntary ballistic abduction of the index finger. Unexpected decrease in torque load does not change the burst. By contrast, in fast ramp abduction with a time‐to‐peak of 0.5 second, the electromyographic activity is fairly continuous up to the peak displacement, and it is silenced with a delay of 50 msec by unexpected unloading. Thus fast ramp, but not ballistic, voluntary contractions are under current long loop control from proprioceptive input. In a second set of experiments, the analysis of single motor unit potentials with highly selective electrodes in interosseous muscle indicated that ballistic thresholds are smaller than ramp thresholds under either isotonic or isometric conditions. However, the recruitment rank of the motor units was identical in finger abductions carried out in either ramp or ballistic modes.
Precise characterization and quantification of infantile spasms
Authors: Kellaway Peter , Hrachovy Richard A. , Frost James D. , Zion Thomas
Published: January 2005

With the use of a time‐synchronized video and polygraphic recording system, 5,042 infantile spasms were monitored and analyzed in 24 infants aged 1 to 43 months. Of these, 33.9% were flexor, 22.5% extensor, and 42.0% mixed flexor‐extensor. Sometimes the spasms were followed by a period of akinesia and diminished responsiveness lasting up to 90 seconds, and rarely (1.0%) this “arrest” effect constituted the entire seizure. More than one type of seizure occurred in 21 of the 24 infants. In the same number, 78.3% of the seizures occurred in clusters, and the intensity and frequency of the spasms in each cluster often increased to a peak, then progressively decreased until they stopped. Predominantly, the clusters occurred soon after arousal from sleep. The number of seizures occurring at night (55.2%) was similar to the diurnal number (44.8%). The electroencephalographic seizure pattern was variable, but a marked generalized attenuation of electrical activity was a feature of 71.7% of the attacks. Attenuation episodes of similar degree and duration occurred with no evidence of a seizure.
Mechanisms of acute carotid stroke
Authors: Pessin Michael S. , Hinton Richard C. , Davis Kenneth R. , Duncan Gary W. , Roberson Glenn H. , Ackerman Robert H. , Mohr J. P.
Published: January 2005

In a three‐year study, the clinical course and results of intracranial angiography were compared in patients having an acute stroke in the carotid artery territory combined with angiographic abnormalities indicating severe were delineated. In one group, the angiographic intracranial abnormalities strongly suggested the presence of embolism in the cerebral vessels supplied were delineated. In one group, the angiographic intracranial abnormalities by the stenotic or occluded carotid artery; many of these patients had no obvious transient ischemic attacks prior to their stroke and experienced a moderate to severe clinical deficit. In the other group, evidence of embolism was absent; many showed a widespread delay in cerebral arterial perfusion, experienced a greater frequency of transient ischemic attacks before their stroke, and had a milder stroke than did those with embolism.
Primary aberrant third nerve regeneration
Authors: Boghen Dan , Chartrand Jean‐Pierre , Laflamme Pierre , Kirkham Trevor , Hardy Jules , Aube Marc
Published: January 2005

The clinical and neuroradiological features in 4 patients with the syndrome of aberrant regeneration of the third nerve are presented. In contrast to what is commonly seen, the syndrome was not preceded by third nerve palsy.

A petrous apex‐cavernous sinus meningioma was the underlying lesion in all 4 patients. Surgical and pathological confirmation of the diagnosis was obtained in 3. Regardless of whether the other ocular motor nerves are spared (as in our patients) or involved (as in other cases reported in the literature), primary aberrant regeneration of the oculomotor nerve appears to be characteristic of a cavernous sinus meningioma.
Multiple sclerosis: Circulating antigen‐reactive lymphocytes
Authors: Traugott Ute , Scheinberg Labe C. , Raine Cedric S.
Published: January 2005

Circulating lymphocyte populations were examined in 85 patients with multiple sclerosis (MS), 26 of whom showed exacerbations; 48 patients with other neurological diseases (OND); 14 patients suffering from psychiatric disorders; and 2 normal subjects. The study involved the assay of early (active, high‐affinity rosetting) T‐cells, myelin basic protein (MBP)‐reactive early T‐cells, late (total, 24‐hour rosetting) T‐cells, and B‐cells. Early T‐cells levels were significantly lower in MS (p < 0.01) than in OND subjects. Exacerbations in MS were usually accompanied by further decreases in early T‐cells. The lower levels of early T‐cells in MS and their fluctuations are believed to reflect disease activity. MBP‐reactive early T‐cells were more frequently increased in MS (75% of cases) than OND (50%), and while this might be indicative of increased sensitization against myelin antigens, it was found not to be an MS‐specific phenomenon.
Myelopathy in mucopolysaccharidosis type II (Hunter syndrome)
Authors: Ballenger Clarence E. , Swift Thomas R. , Leshner Robert T. , El Gammal Taher A. , McDonald Thomas F.
Published: January 2005

A 24‐Year‐old man with Hunter syndrome had spastic quadriparesis due to impingement of thickened meninges upon the cervical spinal cord. Tracheal narrowing due to submucosal deposits (presumably mucopolysaccharide) produced serious ventilatory complications during induction of anesthesia and necessitated tracheostomy before surgical decompression of the spinal cord could be attempted. Recognition of compressive myelopathy and tracheal compromise as late complications of Hunter syndrome may promote early therapy and prevent respiratory catastrophe.
Neurofibrillary tangles, granulovacuolar degeneration, and neuron loss in down syndrome: Quantitative comparison with alzheimer dementia
Authors: Ball M. J. , Nuttall K.
Published: January 2005

The degree of neurofibrillary tangle formation, granulovacuolar degeneration, and nerve cell loss was quantified in serial sections of the hippocampal formation from the brains of 5 adults dying with Down syndrom (mongolism). These morphometric results were compared with values obtained from a series of mentally normal elderly patients reported previously.

Granulovacuolar change appeared to be related as much to patient age as to the existence of mongolism. By contrast, the number of Alzheimer neurofibrillary tangles and loss of pyramidal neurons from the hippocampus exceeded levels found in considerably older subjects and fell into the same range of severity as was noted in 8 patients with Alzheimer dementia. The neuron population in the hippocampus of Down syndrome patients may be only half the expected normal number for their decade.

These quantitative similarities stimulate the search for a common pathogenetic mechanism underlying cerebral changes in mongolism and senile dementia of the Alzheimer type.
Cerebral herniation in bacterial meningitis in childhood
Authors: Horwitz Samuel J. , Boxerbaum Bernard , O'Bell John
Published: January 2005

Among 302 infants and children with acute bacterial meningitis, the syndrome of cerebral herniation occurred in 3 of 10 fatal cases and in 15 patients who survived. The interval from time of admission to herniation was 8 hours or less, and no predictive factors could be determined. Seizures occurring immediately prior to cerebral herniation made diagnosis more difficult. Early recognition of cerebral herniation and prompt treatment with osmotic diuretics can reduce the mortality of bacterial meningitis.
Cryoglobulinemic neuropathy: A pathological study
Authors: Vallat J. M. , Desproges‐Gotteron R. , Leboutet M. J. , Loubet A. , Gualde N. , Treves R.
Published: January 2005

A 53‐year‐old woman developed symmetrical polyneuropathy of the lower limbs a few months after she was found to have myeloma with cryoglobulinemia. In musculocutaneous nerve biopsy material, electron microscopy showed both axonal degeneration and demyelination. The most striking finding was the presence, in the endoneurial space, of numerous masses of closely packed tubular structures. These masses also were found in the walls of all the vasa nervorum and within the lumen of some vessels. The morphological features and dimensions of the deposits within nerve were identical to those of cryoprecipitates extracted from serum and examined with the electron microscope. An example of myeloma neuropathy with cryoglobulin deposits within the endoneurial space has not been reported previously.
Subsequent neurological events in patients with atrial myxoma
Authors: Sandok Burton A. , von Estorff Irene , Giuliani Emilio R.
Published: January 2005

Review of 35 patients from the Mayo Clinic with histologically proved atrial myxomas (7 with prior neurological events) who were followed for 12 to 235 months revealed that none had subsequent delayed neurological events attributable to their atrial myxomas.
Mucopolysaccharidoses: Ultrastructure of leukocyte inclusions
Authors: Markesbery William R. , Robinson Richard O. , Falace Peggy V. , Frye Michael D.
Published: January 2005

Ultrastructural study of leukocytes from 7 patients with mucopolysaccharidosis (MPS) I, II, or III demonstrated a variety of membrane‐bound inclusions in 34 to 68% of lymphocytes and larger mononuclear cells. The most common inclusions were membrane‐bound vacuoles containing faintly osmiophilic material, but a variety of clear vacuoles, dense granular inclusions, and myelin figures were also seen. Fingerprint profiles were present in small percentages in MPS II and III, indicating that they are not as specific as previously considered. Granulocytes, lymphocytes, and larger mononuclear cells from a patient with MPS VII showed many small and larger membrane‐bound vacuolated inclusions, some of which contained large dense granules. The presence of alterations in a readily accessible tissue such as blood indicates that ultrastructural study of leukocytes may be a useful adjunct in guiding further biochemical studies in MPS, es pecially in early or obscure cases.
Acute autonomic and sensory neuropathy
Authors: Colan Richard V. , Carter Snead O. , Oh Shin J. , Bashir Kashlan M.
Published: January 2005

A previously well 9‐year‐old boy developed acute autonomic and sensory neuropathy. Recovery was incomplete over a thirteen‐month period. Spinal fluid protein was increased during the acute phase. Sensory nerve potentials were absent in the presence of normal motor and mixed nerve conduction velocities. Sural nerve biopsy revealed marked axonal degeneration of myelinated as well as unmyelinated fibers. This case represents a widespread acute ganglionopathy of unknown cause.
Intermittent ataxia and immunodeficiency with multiple carboxylase deficiencies: A biotin‐responsive disorder
Authors: Sander James E. , Malamud Nathan , Cowan Morton J. , Packman Seymour , Amman Arthur J. , Wara Diane W.
Published: January 2005

A small group of inborn errors of metabolism are manifested by intermittent cerebellar ataxia. We have previously reported a family with an inherited metabolic defect resulting in multiple carboxylase deficiencies which were responsive to pharmacological doses of blotin. Affected children presented with a skin rash, infections, acute intermittent ataxia, and lactic acidosis. Two affected siblings died prior to diagnosis and therapy, and a detailed postmortem examination was performed on one of them. The brain was characterized by atrophy restricted to the superior vermis of the cerebellum, a finding strikingly similar to that found in chronic alcoholism. Intermittent ataxia would suggest a potentially treatable metabolic disease, and clinical evaluation should include studies of intermediary metabolism and immune function.
Brachial plexus involvement in familial pressure‐sensitive neuropathy: Electrophysiological and morphological findings
Authors: Peter Bosch E. , Chui Helena C. , Martin Michelle A. , Cancilla Pasquale A.
Published: January 2005

Two family members with hereditary pressure‐sensitive neuropathy are reported. One patient presented atypically with acute brachial plexus neuropathy following transaxillary removal of the first rib. Electrophysiological studies showed slowing of motor nerve conduction in clinically affected and unaffected nerves. In vitro recording of the compound action potential of the subclinically involved sural nerve showed pronounced slowing in conduction of large and small myelinated fiber groups. These alterations correlated with morphological studies of the sural nerve that showed tomacula with acute and healed segmental demyelination. An inherited, generalized neuropathy manifested by a morphological abnormality of myelination may render peripheral nerves unduly susceptible to mechanical trauma, including positional pressure or traction effects during general anesthesia.
No measles virus genetic information detected in multiple sclerosis‐ derived brains
Authors: Stevens J. G. , Bastone V. B. , Ellison G. W. , Myers L. W.
Published: January 2005

Measles virus nucleotide sequences were searched for in diseased and normal‐appearing white matter from brains of four multiple sclerosis victims. At the level of sensitivity established, 2.5 to 7.8 measles virus genome‐equivalents of RNA, or 4.9 genome‐equivalents of putative DNA per diploid cell quantity of DNA, could have been detected. At these levels, no measles virus genetic information was found in any of the four brains.
Brain tissue from persons dying of creutzfeldt‐jakob disease causes scrapie‐like encephalopathy in goats
Authors: Hadlow William J. , Prusiner Stanley B. , Kennedy Richard C. , Race Richard E.
Published: January 2005

Two goats became affected with an encephalopathy indistinguishable from scrapie 43 months after they were inoculated intracerebrally with 10% suspensions of brain from two persons dying of Creutzfeldt‐Jakob disease. Although this observation does not establish the common identity of Creutzfeldt‐Jakob disease virus and scrapie virus, it is thought to provide strong evidence of a close etiological relationship between the two diseases.
“Wrong‐way eyes” in supratentorial hemorrhage
Authors: Pessin Michael S. , Adelman Lester S. , Prager Robert J. , Lathi Ellen S. , Lange Dale J.
Published: January 2005

A patient ENveloped conjugate horizontal eye ENviation to the siEN opposite a large frontal‐perisylvian hemorrhage from a ruptured aneurysm. Autopsy examination confirmed the hemispheric locus of the hemorrhage and failed to disclose any lesion of the brainstem or thalamus to explain the “wrong‐way” eye ENviation.
Treatment of bacterial infections of the central nervous system
Authors: Bell William E.
Published: January 2005

Antimicrobial agents for the treatment of bacterial meningitis and other pyogenic intracranial infections have now been in use for approximately 45 years. The last decade, however, has been a period of tremendous advances in knowledge of the pharmacokinetics and other characteristics of the available drugs. The identification of factors that affect their in vivo metabolism and excretion, the importance of drug interactions, and the development of methods for estimation of serum and cerebrospinal fluid levels have added both complexity and sophistication to the clinical utilization of the antimicrobial agents. The number of available antimicrobials has expanded greatly during recent years, although the penicillins, chloramphenicol, and the aminoglycosides remain the mainstay of antibiotic treatment in the majority of cases of bacterial infection of the central nervous system.
Catecholamine‐associated refractory hypertension following acute intracranial hemorrhage: Control with propranolol
Authors: Feibel John H. , Baldwin Claire A. , Joynt Robert J.
Published: January 2005

Seven patients (4 with subarachnoid hemorrhage, 2 with intracerebral hemorrhage, and 1 with massive cerebral infarction) had acute arterial hypertension refractory to control by several antihypertensive drugs (hydralazine, sodium nitroprusside, α‐methyldopa, and trimethaphan camsylate) used singly or in combination. In each case, catecholamine excretion–measured by urinary norepinephrine plus epinephrine–was markedly elevated, averaging 218 m̈g/day. Patients without the acute refractory hypertension had normal or only slightly elevated urinary catecholamine levels (mean, 72 m̈g/day). The β‐adrenergic blocking agent propranolol, in doses between 20 and 40 mg every 6 hours, successfully controlled blood pressure, while other agents failed. The intense sympathetic nervous system discharge resulting in acute refractory hypertension may be due to injury to the diencephalon or brainstem (or both) or to diffuse brain dysfunction from increased intracranial pressure or intracranial blood.
Prognostic indicators in hemiparetic cerebral palsy
Authors: Cohen Michael E. , Duffner Patricia K.
Published: January 2005

In an attempt to define prognostic indicators of intelligence and seizures in the hemiparetic cerebral palsy population, birth histories, electroencephalograms, and computerized tomographic (CT) scans were reviewed in 52 children with hemiparetic cerebral palsy. Cases were excluded when the hemiparesis might have been related to events beyond the neonatal period, such as meningitis, trauma, focal seizures, tumor, or vascular insults. Detailed neurological histories and examinations were done in all patients. Intellectual evaluations, electroencephalograms, and CT scans were obtained. There was no significant relationship of birth history to subsequent seizure development or mental retardation. In contrast, findings in electroencephalograms and CT scans correlated well with the development of seizures and abnormal intelligence. Children who shared anatomical abnormalities of commissural pathways, association pathways, or cerebral cortex were found to have a much higher incidence of seizures and abnormal intelligence.
Ectopic impulse generation in demyelinated axons: Effects of PaCO2, pH, and disodium edetate
Authors: Burchiel Kim J.
Published: January 2005

Demyelinating lesions in the trigeminal root were produced by chronic implantation of chromic suture in 12 cats. Two types of abnormal repetitive action potential generation were recorded from demyelinated fibers: reflected spikes and afterdischarge. Both types of abnormal impulse generation were increased by hypocalcemia induced by disodium edetate infusion, hypocapnia produced by hyperventilation, and, to a lesser extent, alkalosis secondary to sodium bicarbonate administration. This hyperexcitability of fibers could be inhibited by hypoventilation with elevation of Pa or by calcium chloride administration. These results may help explain the pathophysiological basis of paroxysmal symptoms in multiple sclerosis and other disorders in which demyelination is a prominent feature.
Treatment of fungal infections of the central nervous system
Authors: Bell William E.
Published: January 2005

The recent development of new antifungal antimicrobials that can be administered in combination with amphotericin B or as alternatives to it has expanded the dimensions of treatment for fungal infections of the central nervous system. These disorders have acquired increasing importance as patients with malignant and other illnesses associated with immunosuppression survive longer and as renal transplantation is more widely applied. Amphotericin B has remained the most effective therapeutic preparation for most types of neurological fungal disease, although important roles for 5‐fluorocytosine, miconazole, and, more recently, ketoconazole are being recognized. Bell WE: Treatment of fungal infections of the central nervous system.
Mood change following left hemispheric brain injury
Authors: Robinson Robert G. , Szetela Brian
Published: January 2005

Eighteen patients with left hemispheric strokes were compared to 11 patients with traumatic brain injury for frequency and severity of depression, using several mood scales. More than 60% of the stroke patients had clinically significant depressions as compared with about 20% of the trauma patients, even though the two groups had comprable impairments in their activities of daily living and global cognitive functions. Analyses of brain CT scans revealed that the two groups had similar‐sized lesions, but the areas of ischemic injury were more anterior than the traumatic lesion. When the results were controlled for lesion location, there were no significant differences in mood between the two groups. The severity of depression was directly correlated with the closeness of the lesion to the frontal pole. These results suggest that depression following left hemispheric brain injury may not be a nonspecific neurological or psychological response, but rather may be a symptom of injury to specific pathways, such as the catecholamine‐containing ones, as they pass through the frontal cortex.
20,25‐diazacholesterol myotonia: An electrophysiological study
Authors: Furman R. E. , Barchi R. L.
Published: January 2005

The electrophysiological characteristics of the myotonic syndrome produced syndrome produced in mammalian skeletal muscle by administration of 20,25‐diazacholesterol (20,25‐D) were studied in detail. In vivo electromyographic recordings confirmed widespread repetitive electrical activity, but delayed relaxation was evanescent and required isotonic rather than issometric conditions, with long recovery periods between stimuli, for clear demonstration. Subsequent administration of a potent inhibitorof membrane chloride conductance (Gci) induced profound delays in relaxation different from that after chornic 20,25‐D alone. Intracellular passive cable analysis revealed only a small decrease in membrane Gci and none in potassium conductance. Potassium current‐voltage relationships did not differ in control and treated animals. Intracellular microelectrode recordings consistently showed multiple driven action potentials during long depolarizations but no spontaneous myotonic discharges after cessation of the stimulus. Variations in temperature, buffer, and external ionic concentrations also failed to produce spontaneous activity. Anode break excitation under mild depolarizing conditions, however, did elicit repetitive membrane electrical activity.

The myotonia induced by 20,25‐D is not due to low membrane Gci. The relationship between delayed mechanical relaxation and membrane repetitive electrical activity remains to be clearly established in this myotonic syndrome.
Ulnar F‐wave conduction velocity in epidural metastatic root lesions
Authors: De Visser Bram W. Ongerboer , Van Der Sande Jaap J. , Kemp Bob
Published: January 2005

In 40 controls and 19 patients with neoplastic lesions of the eighth cervical and first thoracic nerve roots confirmed by myelography, ulnar F‐wave conduction velocity (FWCV) was estimated using the shortest F‐wave latency (FWL). F‐waves were recorded from needle electrodes inserted in the abductor digiti minimi manus muscle after stimulation of the ulnar nerve at the wrist. Motor nerve conduction velocities of distal ulnar segments were normal. It was found that the ulnar FWCV decreases significantly with increasing age. This is not true for FWL. During the initial examinations ulnar FWCV was delayed in 18 patients. At follow‐up, ulnar signs correlated well with changes in FWCV. Low F‐wave frequency and wide FWL differences are also important features in ulnar root disease.
Hysterical seizures: Suggestion as a provocative EEG test
Authors: Cohen Robert J. , Suter Cary
Published: January 2005

We studied 57 patients with poorly controlled or atypical seizures to identify hysterical attacks with a provocative test consisting of initiation and termination of an attack with suggestion and saline injection during electroencephalographic (EEG) monitoring. Using this method, we diagnosed 48 patients as having hysterical attacks. Three additional patients had spontaneous hysterical attacks during routine recordings. Of the 51 patients with hysterical attacks, 78% were female. Two‐thirds of the patients were between the ages of 20 and 35 years. Thirty‐two patients had taken anticonvulsants before the study. Each patient's hysterical attack, aura, and postictum was stereotyped, and the 6 patients subject to both hysterical and organic seizures described the two types of attacks as different stereotyped behaviors. Tongue laceration, urinary incontinence, and postictal confusion were common. Twelve patients had evidence of past or present neurological disease. Abnormal EEGs were recorded in 37% but only 12% had spike or spike and wave discharges. The provocative EEG test employing saline and suggestion is a useful tool in the diagnosis of hysterical “seizures”.
Distribution of papovavirus, myelin‐associated glycoprotein, and myelin basic protein in progressive multifocal leukoencephalopathy lesions
Authors: Itoyama Yasuto , Webster Henry deF. , Sternberger Nancy H. , Richardson Edward P. , Walker Duard L. , Quarles Richard H. , Padgett Billie L.
Published: January 2005

To study how viruses interact with oligodendroglia and produce demyelination, we immunostained paraffin and epon sections of lesions from patients with progressive multifocal leukoencephalopathy (PML) with antisera to papovaviruses, oligodendroglial myelin‐associated glycoprotein (MAG), and myelin basic protein (MBP) according to the peroxidase‐antiperoxidase method. In paraffin sections from a rapidly progressive case of PML, hyperimmune JC virus antiserum stained single oligodendroglia which were located in white matter that appeared normal histologically and stained normally with MAG and MBP antisera. In zones surrounding areas of demyelination, virus‐containing oligodendroglia were most numerous and MAG staining of periaxonal regions was decreased, but there was little change in MBP staining. In demyelinated regions, both MAG and MBP staining were severely altered; also there was much less JC virus staining. In tissue from three other chronic cases, viral antiserum stained fewer oligodendrocytes and the differences in MAG and MBP staining were much less striking. In epon sections from two biopsies of central nervous system tissue, we studied the electron microscopic appearance of oligodendroglia that also had been stained by JC virus antiserum. Virions were present in all nuclei and in some cytoplasmic regions. The results suggest that changes in MAG distribution are useful indicators of early oligodendroglial abnormalities which can cause myelin breakdown.
A newly recognized congenital myasthenic syndrome attributed to a prolonged open time of the acetylcholine‐induced ion channel
Authors: Engel Andrew G. , Lambert Edward H. , Mulder Donald M. , Torres Carlos F. , Sahashi Ko , Bertorini Tulio E. , Whitaker John N.
Published: January 2005

Five familial cases (in two families) and one sporadic case of a new congenital myasthenic syndrome were investigated. Symptoms arise in infancy or later life. Typically, one finds selective involvement of cervical, scapular, and finger extensor muscles, ophthalmoparesis, and variable involvement of other muscles. There is a repetitive muscle action potential to single nerve stimulus in all muscles and a decremental response at 2 to 3 Hz stimulation in clinically affected muscles. Microelectrode studies reveal markedly prolonged end‐plate potential (epp), miniature end‐plate potential (mepp), and miniature end‐plate current; normal quantum content of the epp; and a smaller than normal or low‐normal mepp amplitude. Light microscopy demonstrates predominance of type I fibers, small groups of atrophic fibers, tubular aggregates and vacuoles near end‐plates, abnormal end‐plate configuration, and nonspecific myopathic changes. Abundant acetylcholinesterase activity is present at all end‐plates, and the activity and kinetic properties of this enzyme in muscle are normal. Calcium accumulated at the end‐plate in one patient. Quantitative electron microscopy shows decrease in the size of nerve terminals, increase in the density of synaptic vesicles, and reduction in the length of postsynaptic membranes. There is focal degeneration of junctional folds with corresponding loss of acetylcholine receptor, most marked in cases with the lowest mepp amplitude. There are no immune complexes at the end‐plate. Fiber regions near end‐plates display dilation, proliferation, and degeneration of the sarcoplasmic reticulum; nuclear, mitochondrial, and myofibrillar degeneration; and vacuoles resembling those found in periodic paralysis. A prolonged open time of the acetylcholine‐induced ion channel is considered to be the basic abnormality and may account for the physiological, morphological, and clinical alterations.
Inclusion‐body myositis: Clinicopathological studies and isolation of an adenovirus type 2 from muscle biopsy specimen
Authors: Mikol Jacqueline , Felten‐Papaiconomou Annie , Ferchal Françoise , Perol Yvonne , Gautier Bernard , Haguenau Michel , Pepin Bernard
Published: January 2005

A case of inclusion‐body myositis appearing clinically as an atypical distal atrophy lasting for more than 14 years was diagnosed by visualization of characteristic filamentous inclusions in subsarcolemmal nuclei and in the sarcoplasm near vacuoles. A strain of adenovirus type 2 was isolated from two successive muscle biopsy specimens, and the serum neutralizing antibody titer to this strain was 1:64. The data provide objective support for the assumption that inclusion‐body myositis is caused by a virus infection of muscle, although the filamentous inclusions differ from all known viral structures.
Neurotransmitter chemistry in feline GM1 gangliosidosis: A model for human ganglioside storage disease
Authors: Singer Harvey S. , Coyle Joseph T. , Weaver Debbie L. , Kawamura Nariko , Baker Henry J.
Published: January 2005

Assays for synaptosomal high‐affinity uptake activity (glutamate, γ‐aminobutyric acid, norepinephrine), neurotransmitter synthesizing enzymes (choline acetyltransferase, glutamate decarboxylase, tyrosine hydroxylase), and endogenous neurotransmitters were performed in cats with advanced inherited GM1 gangliosidosis. A significant reduction in uptake activity, ranging from 24 to 77% of control, was demonstrated in motor, occipital, and cerebellar brain regions. This reduction was unassociated with comparable alterations in neurotransmitter levels or synthesizing enzyme activity. We hypothesize that the defect of neurotransmitter inactivation is part of an overall abnormality of synaptic membrane function that could contribute to the neurological symptoms seen in the hereditary gangliosidoses.
Pathological features of persistent infantile sleep apnea with reference to the pathology of sudden infant death syndrome
Authors: Armstrong Dawna , Sachis Paul , Bryan Charles , Becker Larry
Published: January 2005

A child with alveolar hypoventilation secondary to abnormal control of respiration survived for two years with phrenic pacemakers. Autopsy revealed brainstem abnormalities and other tissue changes that also have been reported in sudden infant death syndrome. Quantitative changes in the distribution of myelinated fibers of the vagus nerve also were similar to those described in sudden infant death syndrome.
3‐O‐ methyldopa blocks dopa metabolism in rat corpus striatum
Authors: Reches Avinoam , Fahn Stanley
Published: January 2005

3‐O‐Methyldopa (OMD) given to rats inhibits striatal uptake and utilization of L‐dopa. Thus, the accumulation of L‐dopa, dopamine, 3,4‐dihydroxyphenylacetic acid, and homovanillic acid in OMD‐pretreated rats after L‐dopa injection is significantly lower compared with control rats. This effect of OMD is dose dependent. OMD inhibits L‐dopa accuumlation in the striatum after inhibition of aromatic amino acid decarboxylase activity with 3‐hydroxybenzylhydrazine‐HCL. This effect is not mediated through inhibition of firing in dopaminergic neurons, since the accumulation of dopamine in the striatum after γ‐butyrolactone injection was also significantly reduced by OMD. It is suggested that OMD competes with L‐dopa and tyrosine uptake into the brain. These findings are in line with clinical observations which indicate that high plasma levels of OMD in parkinsonian patients are associated with poor response to L‐dopa. The data presented here indicate that use of catechol‐O‐methyltransferase inhibitors with L‐dopa may be of value in the treatment of parkinsonian patients.
Characterization of 5‐hydroxytryptamine receptors in human temporal arteries: Comparison between migraine sufferers and nonsufferers
Authors: Skärby Tor , Tfelt‐Hansen Peer , Gjerris Flemming , Edvinsson Lars , Olesen Jes
Published: January 2005

An increase in the sensitivity of temporal arteries to 5‐hydroxytryptamine (5‐HT) has repeatedly been suggested on indirect evidence as an essential component of a migraine attack. We examined the 5‐HT reactivity of isolated human temporal arteries removed from migraine sufferers and from patients operated on because of cerebral disorders. 5‐HT constricted these vessels in a concentration‐dependent manner, the response being attenuated by 10−8 to 10−6M methysergide. There was no difference in reactivity to 5‐HT or methysergide between vessels from the two patient groups. The pA2 value calculated for methysergide in controls was 7.8 ± 0.1 and in migraine patients, 7.6 ± 0.2, which closely agreed with data obtained graphically using Schild plots: 7.8 and 7.7, respectively. No difference existed between the patient groups due to age or sex. Thus, clear evidence has been obtained demonstrating the presence of 5‐HT receptors in human temporal arteries, although we could observe no difference in the character of the 5‐HT receptros between migraine patients and controls.
Cataplexy in variant forms of Niemann‐Pick disease
Authors: Kandt Raymond S. , Emerson Ronald G. , Singer Harvey S. , Valle David L. , Moser Hugo W.
Published: January 2005

A combination of cataplexy and a variant form of Niemann‐Pick disease has occurred in five patients described in the literature as well as in two of our patients. In our Patient 1, cataplexy was differentiated from epilepsy by electroencephalographic telemetry and narcolepsy was confirmed by polysomnography. Cataplexy was abolished in both of our patients by treatment with protriptyline. Four of the seven patients had impairment of vertical eye movements. All seven patients had a disorder best classified as type C or D Niemann‐Pick disease or the Neville, Wenger, or Wiedemann variant. This appears to be the first identified relationship between cataplexy and a specific disease.
The pathophysiology of brain ischemia
Authors: Raichle Marcus E.
Published: January 2005

Brain ischemia due to a critical reduction in cerebral blood flow is a well recognized and common cause of irreversible brain damage. The observation that brain cells are more resistant to ischemia than was previously assumed on the basis of clinical experience has stimulated considerable investigative work designed to determine those factors responsible for irreversible ischemic cell damage. At this time, data from these investigations indicate that cellular acidosis and biochemical disturbances initiated by abnormal intracellular ion homeostasis may be especially important in determining the ultimate survival of nerve cells. This review examines the biochemical events initiated by ischemia and their potential role in determining the ultimate survival of brain cells.
Syndrome of opsoclonus‐myoclons caused by coxsackie B3 infection
Authors: Kuban Karl C. , Ephros Moshe A. , Freeman Roy L. , Laffell Lori Burtz , Bresnan Michael J.
Published: January 2005

Coxsackie B3 was cultured from 2 children who presented within two weeks of each other from the Cape Cod, Massachusetts, area showing opsoclonus and myoclonus. The organism was recovered from the cerebrospinal fluid of 1 patient and from the stools of both. Both children had cerebrospinal fluid pleocytosis and gradual, spontaneous resolution of their symptoms.
Observations on the efficacy of propranolol for the prophylaxis of migraine
Authors: Rosen James A.
Published: January 2005

Among 1,036 patients treated for migraine headache between 1973 and 1980, 865 were given a daily maximum dose of 320 mg of propranolol, while 171 served as differently treated controls. At least one year of treatment was prescribed, at the end of which time 84% of the propranolol group reported sustained improvement compared with similar improvement in only 32% of controls (p < 0.01). The success rate progressively increased during the entire year.
Edentulous orodyskinesia
Authors: Koller William C.
Published: January 2005

Twelve of 75 (16%) edentulous patients (mean age, 62 years) were found to have orofacial dyskinesias. Tooth extraction averaged 12 years before the onset of movements. No dentures were present in 50% of dyskinetic patients and in 9.5% of edentulous patients without dyskinesia. Movements of the lips, tongue, and jaw were marked in 3 patients and mild in 9. Functional disability was minimal. None of 75 elderly patients (mean age, 68 years) without tooth extractions had oral dyskinesias.
Neurological and neuroradiological examination of chronic cannabis smokers
Authors: Hannerz Jan , Hindmarsh Thomas
Published: January 2005

Twelve subjects, eight male and four female, who had smoked on average more than 1 gm of cannabis daily for 10 years, were investigated with a clinical neurological examination and computed tomography (CT) of the brain. No subject had a history of major head trauma or cerebral infection, and just one abused alcohol. Only the subject with a history of alcoholism showed any abnormal cerebral function on clinical tests or any abnormality in the CT scan, compared with normal controls.
Neurotransmitter alterations in a model of perinatal hypoxic‐ischemic brain injury
Authors: Johnston Michael V.
Published: January 2005

Vulnerability of neurotransmitter‐specific neurons to hypoxia‐ischemia was examined in the immature rat corpus striatum. Carotid artery ligation plus 2 hours of 8% oxygen atmosphere at 1 week of age produced ipsilateral striatal injury and reduced hemisphere mass 2 and 6 weeks later. Striatal injury was always more severe than damage to overlying cortex. Over half the animals had status marmoratus, a neuropathological change seen in the basal ganglia and thalamus after hypoxic‐ischemic injury in full‐term human infants. Two weeks after the insult, markers for choinergic, dopaminergic, and γ‐aminobutyric acid–containing neurons were all reduced, but the reduction in cholinergic markers was greater than that for the other two transmitters. Muscarinic cholinergic receptors were relatively preserved, but their distribution was disrupted. In adult animals specific activity of cholinergic neuronal markers was normal, suggesting that the balance of neurotransmitters was restored after the early insult.
Freeze‐fracture analysis of plasma membrane cholesterol in duchenne muscle
Authors: Fischbeck Kenneth H. , Bonilla Eduardo , Schotland Donald L.
Published: January 2005

We estimated cholesterol in Duchenne muscle plasma membrane using the sterol‐specific ligand digitoing. In measuring the extent of digitonin‐cholesterol complex formation with freeze‐fracture electron microscopy, we found a significantly greater proportion of surface area taken up by complexes in Duchenne muscle (60% ± 9%) than in controls (36% ± 4%). This finding suggests that a population of Duchenne muscle fibers has a higher concentration of cholesterol in the plasma membrane. Membrane cholesterol is known to affect membrane deformability and the activity of membrane‐bound enzymes and transport proteins; thus a difference in membrane cholesterol might account for structural and functional abnormalities in Duchenne plasma membrane.
Maturational changes in postictal refractoriness and seizure susceptibility in developing rats
Authors: Moshé Solomon L. , Albala Bruce J.
Published: January 2005

The ability of the central nervous system to suppress recurrent seizures as a function of age was determined in rats. Eight electrical stimulations were delivered to the amygdala at 2‐minute intervals in adult and suckling rats that were previously kindled from the left amygdala. During this 16‐minute period, prolonged and severe convulsions were repeatedly elicited in the 16‐day‐old rat pups, whereas convulsions were not always elicited in adul rats. The results suggest that immature rats are more prone to develop status epilepticus than adult rats. Subsequently, the rat pus and implanted but not stimulated littermates were allowed to grow. At maturity, all the surviving animals were kindled from the contralateral (right) amygdala. The previously kindled rats developed seizures significantly faster than did their littermates. However, their respective abilities to suppress recurrent seizures did not differ at 150 and 210 days of age, being similar to the seizure suppression abilities of naive controls and significantly greater than those evidenced in infancy. We conclude that the propensity to develop single seizures in adulthood is directly related to infant seizure history, whereas the ability to suppress multiple seizures is a dynamic process that is modified by age, being minimal early in life and enhanced with maturation independently of history of infantile convulsions.
Nuclear magnetic resonance imaging in multiple sclerosis
Authors: Lukes Steven A. , Crooks Lawrence E. , Aminoff Michael J. , Kaufman Leon , Panitch Hillel S. , Mills Catherine , Norman David
Published: January 2005

Ten patients with definite multiple sclerosis underwent hydrogen nuclear magnetic resonance imaging with a 3.5 kilogauss superconducting magnet, using the inversion recovery and spin‐echo techniques of signal acquisition. Results were compared with high‐resolution x‐ray computed tomography. Spin‐echo images demonstrated abnormal regions as areas of variably increased signal intensity. The contrast between abnormal and normal white matter improved as the intervals between sequential radiofrequency pulses and between pulse administration and signal sampling were increased. Inversion recovery images demonstrated abnormal areas as regions of decreased signal intensity but did not visualize lesions as well as spin‐echo imaging. Spin‐echo and inversion recovery imaging each demonstrated more extensive abnormalities than did computed tomography.