bluuebunny/crossref_metadata_2025 huggingface dataset:
Immunoreactive myelin basic protein in the cerebrospinal fluid in neurological disorders
Authors: Whitaker John N. , Lisak Robert P. , Bashir Rifaat M. , Fitch Olive H. , Seyer Jerome M. , Krance Robert , Lawrence Jesse A. , Ch'ien Lawrence T. , O'Sullivan Patrick
Published: January 2005

Cerebrospinal fluid from 582 persons was analyzed by a double‐antibody radioimmunoassay for the presence of material cross‐reactive with peptide 43‐88 of human myelin basic protein (BP). In a group of 104 patients with multiple sclerosis (MS), 23 of 33 individuals clinically judged to have had an exacerbation within two weeks prior to the time CSF was obtained had detectable material ranging from 2 to 200 ng/ml. In the remaining 71 MS patients who either were stable or had had an exacerbation more than two weeks before, only 1 patient had a marginally elevated level of immunoreactive material. CSF from 53 persons with cerebrovascular disease was studied, and 13 of 29 with recent infarctions had values of 2 to 540 ng/ml. The degree of elevation in strokes generally paralleled the predicted volume of the lesions, but the amounts detected did not correlate quite so closely temporally with onset as they did with the periods of active disease in MS. Of the remaining 425 patients, 29 had immunoreactive material of 2 to 400 ng/ml in their CSF. Most of these patients with detectable material had acute diseases known to affect the myelin sheath. Eight of 10 persons with acute disseminated encephalomyelitis had no detectable material. The presence in CSF of material cross‐reactive with BP peptide 43‐88 does not have diagnostic specificit for MS but can be used as a means for determining recent myelin injury. The type of BP peptide formed and mechanisms for clearance of BP and BP peptides may be important in determining the biological consequences following release of this potentially immunogenic material from the central nervous system.
Undecussated innervation to the sternocleidomastoid muscle: A reinstatement
Authors: Balagura Saul , Katz Russel G.
Published: January 2005

Clinical evidence is presented to support the contention that the sternocleidomastoid muscle is innervated primarily by undecussated fibers from the ipsilateral hemisphere. Stroke patients often show contraleteral hemiparesis accompanied by weakness in head turning to the side of the paresis.
Rheumatoid disease with encephalopathy
Authors: Kim R. C.
Published: January 2005

A 63‐year‐old woman developed progressive rheumatoid disease complicated by a confusional state as well as persistent cerebrospinal fluid pleocytosis and hypoglycorrhachia. Neuropathological examination revealed extensive rheumatoid lesions in the cranial dura, falx, and choroid plexus. The choroid plexus has not been affected in any of the 16 previously reported cases of pathologically verified rheumatoid disease of the central nervous system. The findings in this patient support the observations of other regarding the role of the choroid plexus in development of CNS manifestations in systemic immune complex disease.
Prolonged relief of tic douloureux from partial root destruction is associated with localized analgesia
Authors: Ecker Arthur
Published: January 2005

Fifty‐eight patients who had been relieved of tic douloureux (TD) for at least 3 years after partial destruction of the trigeminal sensory root by injection of minimal amounts of alcohol were reexamined 31/2 to 21 years after treatment. Of 41 patients still in remission at a median of 10 years after treatment, 32 had analgesia; i.e., sensation of pain from pinprick was absent in all 52 originally neuralgic areas. The other 9 patients in remission had some pain on pinprick in their neuralgic areas. In contrast, of 17 patients with recurrence of TD 31/2 to 11 years (median, 6 years) after treatment, at least 15 had demonstrable pain on pinprick in the neuralgic areas. It is concluded that continuing absence of pain on pinprick in the affected facial mucocutaneous area (or areas) is associated with prolonged relief of tic douloureux.
Isoprinosine treatment in 18 patients with subacute sclerosing panencephalitis: A controlled study
Authors: Haddad Fuad Sami , Risk Winthrop S.
Published: January 2005

Isoprinosine (inosiplex), a proposed antiviral agent, was given to 18 patients with subacute sclerosing panencephalitis (SSPE). No great improvement occurred in any patient soon after treatment, but 2 patients did improve substantially several months after discontinuing Isoprinosine. Spontaneous improvement also occurred in 4 out of 96 controls. Because of the variable natural history of SSPE, differences in clinical improvement between treated patients and controls were not significant. Changes in serial measles antibody titers did not correlate with therapy.
Cerebellar calcification on computerized tomography
Authors: Koller William C. , Klawans Md Harold L.
Published: January 2005

Cerebellar calcification on CT scan was observed in five patients over a two‐year period. It was located bilaterally and symmetrically in the dentate nucleus in all 5 patients and in the cerebellar vermis in one. Calcifications of the basal ganglia and cerebral cortex were associated in two cases each. Skull radiography did not reveal the cerebellar calcifications, and serum calcium levels were normal in all patients. None had symptoms or signs of cerebellar dysfunction, and they had a variety of different clinical diagnoses. Cerebellar calcification may be a form of benign intracerebral calcification.
Disseminated vasculomyelinopathy: An immune complex disease
Authors: Reik Louis
Published: January 2005

The numerous nervous system abnormalities which follow antecedent infections and immunizations appear to share a common pathogenesis involving the immune system. Pathologically, a small vessel vasculopathy involving arterioles and capillaries as well as venules in both gray and white matter is the earliest and most consistent change. Perivascular demyelination appears to develop subsequently. Delayed hypersensitivity to myelin basic protein may not adequately account for these changes.

Humoral immunity may be involved instead. I postulate that antigen‐antibody complexes, formed following the introduction of foreign antigen by infection or inoculation, cause vascular injury with secondary damage to myelin. There is considerable evidence that circulating immune complexes are present in some postinfectious nervous system disorders, as are associated systemic features which suggest immune complex disease. Similar clinical and pathological nervous system changes occur in a variety of disorders in which circulating immune complexes are thought to cause vascular injury.
Brainstem abscess and the syndrome of acute tegmental encephalitis
Authors: Harvey Frederik H. , Carlow Thomas J.
Published: January 2005

A 63‐year‐old man developed the acute onset and rapid progression of signs of lower brainstem dysfunction accompanied by a sterile cerebrospinal fluid containing moderate pleocytosis. Autopsy examination disclosed diffuse acute bacterial inflammation of the brainstem tegmentum. The findings in 55 additional cases of brainstem bacterial infections are reviewed and discussed with respect to differential diagnosis and potential treatment.
PMNs in normal spinal fluid examined by the cytocentrifuge technique
Authors: Simon Roger P. , Koerper Marion A.
Published: January 2005

The presence in unconcentrated CSF specimens of even a single polymorphonuclear (PMN) cel has been regarded as diagnostic of central nervous system (CNS) disease. In 18 of 50 consecutive cytocentrifuged specimens obtained from patients without evidence of CNS disease, however, we found 1 to 6 PMN cells. The observation of occasional PMN cels in concentrated CSF specimens does not appear to be diagnostic of a CNS abnormality.
Impressions of neurology and neurosurgery in the People's Republic of China
Authors: Tower Donald B. , Feindel William
Published: January 2005

During July, 1979, the authors visited the neurological and neurosurgical services of eight major hospitals and associated medical schools in Peking, Tientsin, and Shanghai, and viewed many of their teaching and research facilities. From these visits and our discussions with the staffs of these units, we came away with the impressions reported here.

In the people's Republic of China today there are more than 2,000 neurologists and 1,300 neurosurgeons. At present there are two major clinical centers: the Peking Institute of Neurosurgery, directed by Prof Wang Chung‐Cheng and located in the Xuan Wu Hospital affiliated with the Peking First Medical College; and the Shanghai Institute of Neurology, directed by Prof Chang Yuan‐Cheng and located in the Hua San Hospital affiliated with the Shanghai First Medical College. In every hospital, patient loads are formidable (e.g., several thousand outpatients weekly), but the variety of clinical problems does not differ from those seen elsewhere in the world. The most common problems are head injuries, brain tumors, cerebrovascular disorders, infections, and epilepsy.

Each of the centers is heavily engaged in undergraduate and postgraduate training, and research has been resumed after the major disruptions of the 1966—76 „Cultural Revolution.”︁ Research continues on acupuncture, but present emphasis is directed toward pain mechanisms, notably at the Shanghai Institute of Physiology, directed by Prof Feng De‐Pei. We were most impressed by this, the principal neurophysiology and neuroscience research center in the country.

Clearly, the People's Republic of China has a wealth of clinical material and many competent neurologists and neurosurgeons with stimulating research idesas—characteristics which offer real potentials for collaborative research.
Dyslexia: Regional differences in brain electrical activity by topographic mapping
Authors: Duffy Frank H. , Denckla Martha B. , Bartels Peter H. , Sandini Giulio
Published: January 2005

Electroencephalographic (EEG) and evoked potential data were recorded during behavioral testing from 8 dyslexic and 10 normal boys aged 9 to 11 years. Topographic mapping of their brain electrical activity revealed four discrete regions of difference between the two groups involving both hemispheres, left more than right. Aberrant dyslexic physiology was not restricted to a single locus but was found in much of the cortical region ordinarily involved in reading and speech. Prominent group differences were observed in the bifrontal area in addition to the more expected left temporal and left posterior quadrant regions. Although activation tests produced more prominent group difference, dyslexics differed from normal subjects at rest as well. EEG alpha activity was increased for the dyslexics, suggesting relative cortical inactivity in that group.
Cylindrical spirals in a familial neuromuscular disorder
Authors: Bove Kevin E. , Iannaccone Susan T. , Hilton Pamela K. , Samaha Frederick
Published: January 2005

Cylindrical spirals are unique membranous structures that were detected in skeletal muscle of a mother and one of her two children; all three have percussion myotonia but no evidence of weak or wasted skeletal muscles. Muscle cramps, stiffness, posteffort muscle tightness, myotonic lid lag, and the cylinders appear or progress with age, but the disorder is asymptomatic in the children and only mildly incapacitating in the mother. The cylinders are 8 μ long and 1 μ wide, are composed of spiraling double‐laminate membrane resembling myelin, and are derived from abnormal subsarcolemmal tubulovesicular structures that are interpreted as pathological T‐tubes.
Neuropathology of phenylacetate poisoning in rats: An experimental model of phenylketonuria
Authors: Wen G. Y. , Wisniewski H. M. , Shek J. W. , Loo Y. H. , Fulton T. R.
Published: January 2005

Results of this investigation indicate that the suckling rat treated with phenylacetate should be a useful new model for studying the pathogenesis of phenylketonuria and neuronal development. Both cerebellar and retinal neurons of postnatally treated rats are vulnerable to the adverse effects of phenylacetate. Morphological changes observed in the cerebellum, retina, and optic nerve of treated animals during the fourth to twenty‐first days of life consist of regional reduction in the size of cerebellar vermis lobules IV, V, VIa, and IX, 35 to 40% reduction in thickness of the molecular layer, accumulation of cerebellar external granular cells and retinal neuroblastic cells, fewer parallel fibers in the cerebellar cortex, and fewer myelinated axons in the optic nerve.
Failure to detect abnormal fatty acid profiles in serum lipoproteins in Friedreich's ataxia
Authors: Walker Janet L. , Chamberlain Susan , Robinson Norman
Published: January 2005

A recent report indicated an association between the spinocerebellar degeneration of Friedreich's ataxia and abnormal fatty acid profiles of the cholesterol ester fraction in both total plasma and high‐density lipoprotein. In an effort to substantiate these findings in lipoproteins, we estimated fatty acid profiles of total high‐density lipoprotein and the cholesterol esters of high‐ and low‐density lipoproteins. Those profiles in 10 Friedreich's ataxia patients compared well with previously reported normal values and did not differ significantly from results in 10 age‐matched controls.
Changes in endoneurial fluid pressure, permeability, and peripheral nerve ultrastructure in experimental lead neuropathy
Authors: Myers Robert R. , Powell Henry C. , Shapiro Harvey M. , Costello Michael L. , Lampert Peter W.
Published: January 2005

The dynamics of endoneurial edema were studied by quantifying endoneurial fluid pressure (EFP) during the development of lead neuropathy and correlating these data with changes in blood‐nerve barrier permeability and with morphological alterations in nerves, capillaries, and Schwann cells. EFP measured from the sciatic nerve in control Long‐Evans rats was 2.1 ± 1.0 cm H2O. EFP was significantly elevated 7 weeks after animals were started on a diet containing 6% lead carbonate, and it increased progressively until a plateau in pressure was reached between weeks 9 and 11. Thereafter, EFP gradually returned to normal values. The progressive increase in EFP was highly correlated with the extravasation of osmotically active macromolecules, traced by fluorescein isothiocyanatedextran compounds of graded molecular weight and by horseradish peroxidase (HRP). Electron microscopy revealed extravasation of HRP between endothelial cells, intranuclear inclusions characteristic of lead poisoning in Schwann cell nuclei, demyelination, and remyelination. The observation of intranuclear inclusions consistent with lead deposition in Schwann cells strengthens the hypothesis that extravasated lead in the interstitial fluid causes direct injury to Schwann cells, giving rise to demyelination. Nerve compliance was determined.
Lafora disease: Diagnosis by liver biopsy
Authors: Nishimura R. N. , Ishak K. G. , Reddick R. , Porter R. , James S. , Barranger J. A.
Published: January 2005

We have studied four patients who had a clinical course compatible with Lafora disease. The diagnosis was confirmed in one by the presence of Lafora bodies in central nervous system neurons at autopsy and was supported in another by findings in the cerebral biopsy of a sibling. Our patients had no clinically apparent liver disease, but liver specimens in each instance showed a distinctive histological abnormality, with hepatocytes containing inclusions having a ground‐glass appearance. The liver biopsy findings appear to be relatively specific for this disorder and can easily be differentiated from those in other liver diseases.
Somatosensory evoked potentials following trigeminal nerve stimulation in trigeminal neuralgia
Authors: Stöhr M. , Petruch F. , Scheglmann K.
Published: January 2005

The examination of somatosensory evoked potentials following trigeminal nerve stimulation in 20 subjects with idiopathic trigeminal neuralgia revealed a pathological increase in latency of the first positive component on the involved siEN in 7 (41%) of the 17 patients without anteceENnt surgical treatment. The mean difference in latency between the two siENs was 1.3 msec (2p ≥ 0.005). These results are indicative of microtraumatization of the trigeminal nerve in the posterior fossa by blood vessels, for example, leading to local ENmyelinization. The method seems to be of diagnostic value in selecting patients for nerve ENcompression by posterior fossa operations.
Pattern‐reversal visual evoked potentials in the hereditary ataxias and spinal degenerations
Authors: Bird Thomas D. , Crill Wayne E.
Published: January 2005

Pattern‐reversal visual evoked potentials (PRVEP) were evaluated in 24 patients from 18 separate families with various forms of hereditary ataxia and spinal degeneration. Abnormally delayed latencies were found in 3 of 5 patients with classic Friedreich's ataxia, 1 patient with dominant spastic paraparesis, and 1 patient with recessive dentatorubrospinal degeneration. Fifteen other patients with several different types of dominant and recessive hereditary ataxias had normal PRVEP latencies, including 1 patient with bilateral optic atrophy. Testing of PRVEP will be useful in the clinical delineation of the genetic ataxias and spinal degenerations, and, when interpreted with caution, should be an additional variable evaluated in the differentiation of these disorders from multiple sclerosis.
The role of hydrostatic pressure in ischemic brain edema
Authors: Kogure Kyuya , Busto Raul , Scheinberg Peritz
Published: January 2005

The mechanisms responsible for early prenecrotic ischemic brain edema were investigated in rats by comparing brain metabolism, tissue water (HOH) content, and sodium and potassium ion concentration in brain during ischemia induced by decapitation, by the Pulsinelli‐Brierley technique, and by carotid embolization. Although brain metabolic functions were similarly disturbed in all three groups, an increase in brain HOH occurred only in the embolism model, which allowed collateral perfusion. Early ischemic brain edema is therefore dependent upon (1) impaired energy‐dependent ion pumps and (2) a hydrostatic pressure gradient from patent vascular lumens. Elevated perfusion pressure increases the extent of this early edema. Induced hypertension causes impairment of blood‐brain barrier function, as evidenced by extravasation of Evans blue dye 5 minutes after embolic ischemia, and strikingly increases the extent of macromolecular extravasation 4 hours after ictus. This increased protein leakage is accompanied by elevated HOH content and sodium concentration, as compared to findings in normotensive animals. It is concluded that the use of induced hypertension as a therapeutic modality in patients with acute stroke may be harmful.
Reduced glycine receptor in the spinal cord in amyotrophic lateral sclerosis
Authors: Hayashi Hideaki , Suga Masakazu , Satake Mei , Tsubaki Tadao
Published: January 2005

Transmitter receptor binding was estimated in the spinal cord of 6 subjects with amyotrophic lateral sclerosis (ALS) and 4 control subjects in assays using 3H‐quinuclidinyl benzilate for muscarinic cholinergic receptors, 3H‐strychnine for glycinergic receptors, 3H‐spiroperidol for dopaminergic receptors, 3H‐muscimol for GABAergic receptors, and 3H‐dihydroalprenolol for β‐adrenergic receptors. In ALS, glycinergic receptor binding was greatly reduced in the anterior gray matter. This finding may be attributed to loss of large neurons in the anterior gray matter, known to be characteristic of ALS.
Sleep apnea and hypoventilation syndrome associated with acquired nonprogressive dysautonomia: Clinical and pathological studies in a child
Authors: Frank Yitzchak , Kravath Richard E. , Inoue Kiyoharu , Hirano Asao , Pollak Charles P. , Rosenberg Roger N. , Weitzman Elliot D.
Published: January 2005

A 6‐year‐old girl had subacute onset of hypoventilation and apnea during sleep. Diffuse dysautonomic changes were identified, including dilated, nonreactive pupils, decreased tearing and sweating, and abnormal temperature and cardiovascular control. All‐night polysomnographic studies revealed frequent obstructive and central sleep apnea episodes. Her serum contained cytotoxic antineuroblastoma immunoglobulins. She died two years later during sleep. The general pathological examination revealed a ganglioneuroma originating in the sympathetic ganglia. Abnormalities in the brain were confined to the brainstem and consisted of complete loss of neurons with severe fibrillary gliosis in the region of the Edinger‐Westphal nuclei as well as loss of neurons with gliosis in the locus ceruleus and in the reticular formation bilaterally.
Seizures, hypoxic‐ischemic brain injury, and intraventricular hemorrhage in the newborn
Authors: Hill Alan , Volpe Joseph J.
Published: January 2005

This review deals with neonatal seizures, perinatal hypoxic‐ischemic brain injury, and neonatal intraventricular hemorrhage. Neonatal seizures are the most prominent signals of the largest number of neonatal neurological disorders. The convulsive phenomena may be subtle. The predominant etiological process is hypoxic‐ischemic encephalopathy. Prognosis is related primarily to the neurological disease responsible for the seizures. Treatment may be specific for the underlying disorder (e.g., glucose or calcium) or less specific (i.e., therapy with anticonvulsant drugs). Prompt control of the seizures is important to avoid brain injury secondary to the effects of the seizures on ventilation, perfusion, and brain metabolism.

Hypoxic‐ischemic encephalopathy in the newborn most often is a consequece of intrauterine asphyxia. Diagnosis depends primarily on recognition of the clinical syndrome but also on a variety of neurodiagnostic techniques, including radionuclide and CT brain scans. Prognosis is estimated best by a combination of clinical analysis and specialized neurodiagnostic studies. Management is based principally on vigorous support, particularly of ventilation and perfusion, maintenance of adequate glucose influx, and control of seizures.

Intraventricular hemorrhage is the most common type of neonatal intracranial hemorrhage. The neuropathology is characterized by bleeding from capillaries of the subependymal germinal matrix. Secondary rupture of the ependymal lining then causes intraventricular hemorrhage. Pathogenesis relates to the anatomy of the germinal matrix, the distribution and regulation of cerebral blood flow, and the structure and vulnerability of periventricular capilaries. Precise diagnosis requires a brain imaging procedure; portable, real‐time ultrasound is the preferred approach for critically ill infants. Prognosis relates to the severity of the hemorrhage as well as any preceding hypoxic‐ischemic insults and the subsequent occurrence of hydrocephalus. Choice of therapy for posthemorrhagic ventricular dilation depends upon severity and rapidity of progression and ranges from close observation only to ventriculoperitoneal shunting.
Neclear medicine in clinical neurology: An update
Authors: Oldendorf William H.
Published: January 2005

Isotope scanning using technetium 99m pertechnetate has fallen into disue since the advent of x‐ray computerized tomography. Regional brain blood flow studies have been demonstrated and is of use in localizing such foci. Cisternography as a predictive tool in normal pressure hydrocephalus is fallin into disue. Positron tomographic scanning is a potent research tool that can demonstrate both regional glycolysis and blood flow. Unfortunately, it is extremely expensive and complex to apply in a clinical setting. With support from the National Institutes of Health, seven extramural centers have been funded to develop positron tomographic capabilities, and they will greatly advance our knowledge of stroke pathophysiology, seizure disorders brain tumors and various degenerative diseases. Nuclear magnetic resonance imaging is a potentially valuable tool since it creates tomographic images representing the distribution of brain water. No tissue ionization is produced, and images comparable to second‐generation computerized tomographic scans are already being produced in humans.
Development of a tomographic myelin scan
Authors: Frey K. A. , Wieland D. M. , Brown L. E. , Rogers W. L. , Agranoff B. W.
Published: January 2005

The principle that myelin can be imaged nonivnvasiely using the emission tomographic distribution of a lipophilic radioactive tracer was investigated. Properties of agents suitable for noninvasive myelin scanning are discussed with specific reference to blood‐brain barrier permeability, metabolism, and tracer lipophilicity. The brain distributions of inert tracers are correlated with their partitioning between octanol and saline. A test probe, iodobenzene, was labeled with iodine 125 for preliminary invasive studies in the rabbit. The equilibrium brain distribution, determined either autoradiographically of by regional dissection, corresponded closely to that of myelin. 123I‐labeled iodobenzene, a gamma‐emitting analog, was then administered to a monkey, and tomographic reconstruction revealed a pattern of brain uptake corresponding to white matter.
Extraneural metastases in childhood brain tumors
Authors: Duffner Patricia K. , Cohen Michael E.
Published: January 2005

Althogh extraneural metastases are known to occur with primary tumors of the central system, they are considered a rarity. Large autopsy series have place the frequency at 0.4 to 0.5%. At the roswell Park Memorial Institute, 36 children with brain tumors have been treated with a combination of surgery, radiotherapy, and chemotherapy in the past 12 years. We recently reviewed the autopsies obtained on 15 of these children and found that 4 (26%) had extraneural metastases. An additional child, who was not autopsied, had extraneural extension diagnosed during life.

This inordinately high incidence of metastases outside the nervous system in children with aggressively trated brain tumors raises questions about their cause. The prolonged survival of these patients may have allowed growth of sufficient generations of extracranial metastatic cells for the extraneural disease to become clinically significant. Another possibility is that chemotherapy may alter the patients' immune system and thus promote extracranial tumor growth. As larger numbers of childhood brain tumors are managed with multimodality therapy, it may be possible to evaluate the extent to which tratment allows intracranial neoplasms to spread outside the nervous system.
Endorphins in experimental spinal injury: Therapeutic effect of naloxone
Authors: Faden A. I. , Jacobs T. P. , Mougey E. , Holaday J. W.
Published: January 2005

An experimental model of cat spinal injury was used to investigate the hypotheses that endorphins are involved in the pathophysiology of spinal cord injury and that the opiate antagonist naloxone, by blocking the effects of endorphins, may improve physiological and neurological recovery. Experimental trauma to the cervical spine casued a reduction in blood pressure and spinal cord blood flow (SCBF) associated with increased plasma levels of β‐endorphin–like immunoreactivity; both blood pressure and SCBF were significantly improved by naloxone treatment. Naloxone‐treated animals also showed less prominent spinal cord abnormalities and significantly improved neurological recovery compared with saline controls. Moreover, both the pathological and clinical findings correlated significantly with the blood pressure changes following treatment. The data support a pathophysiological role for endorphins in experimental spinal cord injury and suggest that opiate antagonists might improve the treatment of spinal trauma in humans.
Pharyngoesophageal motor function in patients with myotonic dystrophy
Authors: Swick H. M. , Werlin S. L. , Dodds W. J. , Hogan W. J.
Published: January 2005

Six adults with myotonic dystrophy underwent detailed radiological and manometric studies of pharyngoesophageal function. Four of the patients had no history of swallowing dysfunction, while two had very mild symptoms. All patients showed abnormalities of pharyngoesophageal function. Pharyngeal peristalsis was present but of low amplitude. Resting upper esophageal sphincter pressure was diminished, but the sphincter relaxed normally. Peristalsis in the esophageal body was in variably absent in the striated muscle segment of the esophagus and was either absent or decreased in amplitude in the smooth muscle segment. The lower esophageal sphincter was normal. Myotonia did not occur in the sphincters or the esophageal body. These findings suggest that phyaryngoesophageal dysfunction is common in patients with myotonic dystrophy, even when clinical symptoms are absent. The abnormalities are probably related to muscle weakness rather than to myotonia in the pharyngeal and esophageal musculature.
Effects of dipropylacetate on brain development
Authors: Diaz Jaime , Shields W. Donald
Published: January 2005

Dipropylacetate (DPA; valproci acid) is a new anticonvulsant reported to be effective in many types of seizures, including those that occur early in life. This study describes the effects of chronic administration of DPA upon a developing organism. Four‐day‐old rat pups were injected daily with either 75 mg/kg DPA, 200 mg/kg DPA, or vehicle until day 18. Administration of DPA resulted in decreased body and organ weights, with the greatest reductions in the 200 mg/kg group. To control caloric intake, an additional experiment was conducted. Animals were injected with either vehicle or 200 mg/kg DPA. Each animal was implanted with a chronic intragastric cannula and fed through the cannula. The results of this experiment indicated that body and organ weights were the same for both vehicle and drug groups, except for brain. The animals receiving DPA had significant deficits of 12% in total brain weight and 22% in weight of the cerebellum. The data suggest that chronic administration of DPA early in life may have adverse consequences on brain growth.
Retardation of schwann cell division and axonal regrowth following nerve crush in experimental lead neuropathy
Authors: Ohnishi Akio , Dyck Peter James
Published: January 2005

In experimental lead neuropathy, Schwann cells undergo segmental demyelination and possibly cell death and, concurrently, remyelination and multiplication to create new internodes of myelin and onion bulb formations. In rats fed 4% lead carbonate for three months, the ability of Schwann cells to divide (percentage showing mitotic figures, labeling index, and total number per millimeter of fascicular length of nerve) was studied serially for four weeks. Schwann cell events were also compared in sural nerve distal to crush with and without resection of an intervening length of nerve. In both cases, Schwann cell multiplication as a result of axotomy was retarded in lead‐intoxicated as compared to control animals. On the average, regrown myelinated axons four weeks after and 10 mm below the point of crush in lead‐treated animals were similar in number and smaller in caliber, but they exhibited a normal relationship of myelin thickness to axonal area compared with control animals. These studies provide evidence that Schwann cell division and axonal regrowth after crush are retarded in experimental lead neuropathy.
A unifying hypothesis for the cause of amyotrophic lateral sclerosis, parkinsonism, and alzheimer disease
Authors: Appel Stanley H.
Published: January 2005

The causes of amyotrophic lateral sclerosis, Parkinson disease, and Alzheimer disease are unknown. Furthermore, treatment for two of these conditions is almost totally lacking. The thesis is presented that each of these disorders is due to lack of a disorder‐specific neurotrophic hormone. The hormone would be elaborated or stored in the target of the affected neurons. It would be released by the postsynaptic cell and then exert its effect in a retrograde fashion after being taken up by the presynaptic terminal. In the lower motor neuron syndromes of amyotrophic lateral sclerosis, failure of muscle cells to release the appropriate motor neuron syndromes of amyotrophic lateral sclerosis, failure of muscle cells to release the appropriate motor neurotrophic hormone would result in impaired function of anterior horn cells. In Parkinson disease, the neurotrophic failure would be characterized by inability of striatal cells to provide the required dopamine neurotrophic hormone with resulting impairment of substantia nigra cells. In Alzheimer disease, the abnormalities would lie in failure of the hippocampus and cortical cells to supply the relevant cholinergic neurotrophic hormone with resulting impairment of medial septal and nucleus basalis neurons. Central nervous system tissue culture provides a convenient system in which to assay these neurotrophic hormones and should permit a test of the hypothesis.
Immunological studies in senile dementia of the alzheimer type: Evidence for enhanced suppressor cell activity
Authors: Miller Aaron E. , Neighbour P. Andrew , Katzman Robert , Aronson Miriam , Lipkowitz Rochelle
Published: January 2005

To examine immunological variables in senile dementia of the Alzheimer type (SDAT), we compared SDAT patients with elderly and young controls with regard to concanavalin A (Con A)–induced suppression, lymphocyte proliferation, interferon production, and serum immunoglobulin levels. The results showed exaggerated Con A suppression and reduced lymphocyte proliferation in SDAT patients compared to elderly control subjects. Interferon production after stimulation by Con A and immunoglobulin levels did not differ between SDAT and elderly control subjects. The results suggest imparied immunoregulatory mechanisms in patients with SDAT that may be related to the cause or course of SDAT. Alternatively, these immunological changes may be secondary to pathological changes in the central nervous system.
Evidence for induction of localized amyloid deposits and neuritic plaques by an infectious agent
Authors: Wisniewski Henryk M. , Moretz Roger C. , Lossinsky Albert S.
Published: January 2005

Mice inoculated intracerebrally with mouse brain homogenate infected with a particular strain of scrapie agent developed amyloid and neuritic plaques and amyloid tubercles along the injection track, solid wall‐like infiltrations at the injection site, or both, Our observations implicate an infectious agent in the localized formation of the amyloid and the neuritic plaque—the leading pathological change found in normal elderly people and animals and in patients with senile dementia of the Alzheimer type. Further, these studies indicate that microglia appear to play a central role in the pathogenesis of amyloid formation and could be involved in the processing, production, and assembly of the fibers.
Slowly alternating skew deviation: Description of a pretectal syndrome in three patients
Authors: Corbett James J. , Schatz Norman J. , Shults W. Thomas , Behrens Myles , Berry Richard G.
Published: January 2005

Three patients who had slowly alternating skew deviation are described: each had elements of the Sylvian aqueduct syndrome. This combination of signs supports a pretectal location for lesions associated with alternating skew movements. Postmortem examination of a patient who died of chronic herpes simplex encephalitis showed extensive demyelination and periaqueductal spongiform degeneration; there was preservation of the oculomotor and trochlear nuclei, the medial longitudinal fasciculus, vestibular nuclei, and the interstitial nucleus of Cajal bilaterally. The slowly alternating dysconjugate vertical movements bear a resemblance to both see‐saw nystagmus and the ocular tilt response.
The irrelevance of fusimotor activity to the achilles tendon jerk of relaxed humans
Authors: Burke David , McKeon Brian , Skuse Nevell F.
Published: January 2005

In two normal subjects the sciatic nerve was blocked completely using concentrated lidocaine. The muscle afferent and reflex electromyographic responses to reproducible percussion of the Achilles tendon were recorded while the blocks developed. The intensity of percussion was sufficient to produce an Achilles tendon jerk in one subject when at rest and in the other during reinforcement. The block did not alter the muscle afferent response to tendon percussion in either subject. It is concluded that background fusimotor activity is not a prerequisite for the tendon jerk and that, during complete relaxation, there may be no significant fusimotor drive directed to the triceps surae. The varying ease with which tendon jerks can be elicited in different normal subjects or in different muscles of the same subject appears to be related not to fusimotor activity but to differences in the “central excitability state”.
Cerebellar degeneration in the rat following rapid correction of hyponatremia
Authors: Kleinschmidt‐DeMasters B. K. , Norenberg Michael D.
Published: January 2005

Degenerative lesions in the superior vermis of the cerebellum were produced in 8 of 14 rats that were first made hyponatremic for three days with vasopressin and water and then given hypertonic saline. Within the superior vermis, lesions were predominantly localized to the crests of the folia. These lesions were characterized by demyelination of folial white matter and necrosis of granule cells at the junction with the white matter. In severe degeneration, the entire width of the granule cell layer was involved and Purkinje cell necrosis was found as well. Hyponatremia alone or administration of hypertonic saline to normonatremic rats did not result in lesions. Because of the topographical and histopathological similarity of these lesions to those of alcoholic cerebellar degeneration, our findings raise the possibility of a contribution of electrolyte‐induced injury to the pathogenesis of alcoholic cerebellar degeneration.
Congenital cholinergic nervous system dysfunction in identical twins
Authors: Kien C. L. , Herrmann Jurgen , Hogan Walter
Published: January 2005

We report identical male twins with secretory diarrhea, craniofacial abnormalities often found in G syndrome, hypospadias, and abnormalities of the cholinergic nervous system including achalasia, decreased tearing, corneal hypesthesia, positive Mecholyl test (eye and esophagus), and decreased sweating. The children also lacked fungiform papillae but were clinically distinct from patients with either the Riley‐Day syndrome or acquired dysfunction of the autonomic nervous system. Assessment of autonomic nervous system function should be considered in infants with recurrent diarrhea or swallowing disorders or in children with morphological characteristics suggesting G syndrome.
Binaural voltage summation of brainstem auditory evoked potentials: An adjunct to the diagnostic criteria for multiple sclerosis
Authors: Prasher D. K. , Sainz M. , Gibson W. P. R.
Published: January 2005

Brainstem auditory evoked potential (BAEP) amplitude is modified according to whether or not the stimulus is applied monaurally or binaurally. In normal subjects, wave V amplitude increases by an average of 68.7% upon changing stimulation from monaural to binaural. From earlier studies there is evidence that brainstem potential amplitude is reduced in patients with multiple sclerosis (MS) but none to suggest that binaural stimulation results in increased amplitude. This study evaluated the extent of binaural summation of BAEPs in patients with MS.

In a large majority of patients with MS who have no hearing deficit, BAEPs showed no increase in wave V amplitude on binaural stimulation. This finding is in contrast to the normal group and thus has diagnostic importance. Measurements of binaural summation therefore might usefully be applied to the clinical assessment of disease progression, or lack of it, in individual patients.
Taurine and hyperexcitable human muscle: Effects of taurine on potassium‐induced hyperexcitability of dystrophic myotonic and normal muscles
Authors: Durelli Luca , Mutani Roberto , Fassio Franco , Satta Andrea , Bartoli Ettore
Published: January 2005

Progressively increasing concentrations of potassium chloride in Evans blue saline were administered to patients affected with myotonic dystrophy and to healthy volunteers before and after parenteral treatment with taurine. Excitability changes of thenar eminence muscles were related to the venous potassium and chloride concentrations. The actual electrolyte concentrations were compared to those to be expected if no infused electrolytes had been transported into cells. The expected concentrations were calculated by means of Evans blue dilution. This method permitted quantification of changes of muscle excitability in terms of the potassium chloride concentration capable of disturbing the electrical activity of the studied muscles. The method also provided an indirect evaluation of electrolyte movements across muscle membrane in vivo in humans. Dystrophic myotonic muscles appeared highly sensitive to extracellular potassium and, unlike normal muscles, were unable to accumulate potassium chloride when its external concentration had been raised. Administration of taurine decreased the potassium‐induced muscle hyperexcitability and favored electrolyte accumulation in dystrophic myotonic muscles. The stabilizing effect of taurine is discussed in relation to its ability to increase intracellular potassium concentration, membrane conductance, or both.
Carbamazepine and hematological monitoring
Authors: Hart Robert G. , Easton J. Donald
Published: January 2005

Clinically important hematological toxicity is uncommon with carbamazepine (CBZ). Only 20 patients with CBZ‐associated aplastic anemia have been reported since 1964. Leukopenia and thrombocytopenia occur in about 2% of patients. Based on the available data on hematological complications of CBZ, we question the justification for expensive, excessive laboratory monitoring.
Biochemical findings in a case of parkinsonism secondary to brain tumor
Authors: De Yýbenes J. García , Gervas J. J. , Iglesias J. , Mena M. A. , Del Rio R. Martín. , Somoza E.
Published: January 2005

Neuropathological and biochemical studies were performed in a patient with craniopharyngioma and a long history of parkinsonism. Morphological examination revealed atrophy of the substantia nigra without Lewy bodies. Both the caudate and putamen nuclei showed moderate loss of large cells with glial proliferation. There was a notable decrease in dopamine and its metabolites and a reduction in norepinphrine content in both the caudate and putamen nuclei. Less consistent changes were found in serotonin and amino acid levels. Dopamine receptors, evaluated as 3H‐spiperone binding sites, were unchanged in the putamen but decreased to 30% of control values in the caudate. The findings suggest that parkinsonism was caused by the brain tumor, which damaged both the presynaptic dopaminergic nigrostriatal neurons and the postsynaptic dopamine recptors.
Acute conduction block associated with experimental antiserum‐mediated demyelination of peripheral nerve
Authors: Sumner Austin J. , Saida Kyoko , Saida Takahiko , Silberberg Donald H. , Asbury Arthur K.
Published: January 2005

Intraneural injection of antisera from rabbits with high antigalactocerebroside antibody levels into rat sciatic nerve produced acute nerve conduction block. This was first apparent in some motor axons between 30 and 60 minutes after injection and progressed to completion within 2 to 4 hours. Concurrent morphological evidence of demyelination was present, but structural changes at the time of onset of block were mild and were restricted to the myelin and Schwann cell, particularly at the paranodal areas and Schmidt‐Lanterman clefts. It is suggested that paranodal lesions could account for the observed conduction block.
Macroglobulinemia with peripheral neuropathy simulating motor neuron disease
Authors: Rowland Lewis P. , Defendini Richard , Sherman William , Hirano Asao , Olarte M. R. , Latov Norman , Lovelace Robert E. , Inoue Kiyoharu , Osserman Elliot F.
Published: January 2005

A 48‐year‐old man with an IgM plasma cell dyscrasia died after 14 months of symptoms and signs typical of motor neuron disease, including widespread fasciculation and normal sensation. Two laboratory results were atypical: cerebrospinal fluid protein content of 132 mg/dl and slow motor nerve conduction. At autopsy, no loss or atrophy of anterior horn neurons was found; instead, degeneration of ventral and dorsal roots and retrograde changes of chromatolysis in motor neurons implied peripheral neuropathy. Most reported cases of neuropathy associated with plasma cell dyscrasias have been sensorimotor or purely sensory, but there have been 14 previous cases of motor disorders.
Plasmapheresis and immunosuppressive drug therapy in the Eaton‐Lambert syndrome
Authors: Dau Peter C. , Denys Eric H.
Published: January 2005

Five patients with Eaton‐Lambert syndrome (ELS) without associated malignancy were first treated by plasmapheresis alone, and subsequently by plasmapheresis combined with prednisone and azathioprine therapy. Three of the five were also treated by the immunosuppressive drugs alone. Although all three therapeutic regimens resulted in some clinical and electromyographic (EMG) improvement, the greatest improvement was seen during plasmapheresis combined with prednisone and azathioprine. The initial evoked compound muscle action potential (CMAP) increased during treatment in all patients, while facilitation of the initial CMAP by tetanic stimulation decreased. Overall, the EMG decrement elicited at 2 Hz lessened in four of the five patients. Serious complications probably attributable to guanidine toxicity developed in three patients: two had renal failure and one had gastrointestinal and urinary tract bleeding. Our results suggest that immunosuppressive therapy may have a place in the management of ELS and that circulating factors such as autoantibody may participate in its pathogenesis.
Incomplete manifestations of myotonic dystrophy in a large kinship in labrador
Authors: Pryse‐Phillips William , Johnson Gordon J. , Larsen Bodil
Published: January 2005

In a large kinship affected by myotonic dystrophy (MyD), 133 subjects, including 21 spouses, were examined independently by a neurologist and an ophthalmologist to determine the earliest identifying evidence of the disease. Assessment included tonometry, slit‐lamp examination, facial measurements, and electromyography (EMG). Thirty‐two subjects, all of whom had EMG characteristics of myotonia, were definitely affected. Low intraocular tensions were a more consistent finding than lens opacities; endocrine abnormalities were absent.

Twenty‐seven subjects had one to five features but lacked clinical or electrographic evidence of myotonia; they were designated as having a “partial syndrome.” Signs most commonly found included weakness of the upper face, brachial hyporeflexia, low intraocular tensions, and the presence of sparse colored specks at the posterior poles of the lenses. All but 2 subjects (siblings) with the partial syndrome had a parent with either MyD or the partial syndrome. Linkage studies indicated that 7 subjects should not be carrying the gene, while others could be regarded as having a minor expression of MyD. In most persons with the partial syndrome, we could not predict whether typical MyD would develop later. Transmission of the disease may depend upon more than a single autosomal dominant gene, and nongenetic influences may also be important.