bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hereditary Motor Sensory Neuropathy Type I Presenting as Scapuloperoneal Atrophy (Davidenkow Syndrome) Electrophysiological and Pathological Studies
Authors: Ronen Gabriel M. , Lowry Noel , Wedge John H. , Sarnat Harvey B. , Hill Alan 
Published: July 2016

ABSTRACT:A 14 year old boy with scapuloperoneal muscular atrophy, pes cavus, areflexia and distal sensory loss (Davidenkow syndrome) is described. Nerve conduction velocities were diminished. Sural nerve biopsy demonstrated a reduction in the number of myelinated fibers and early “onion-bulb” formation. These observations support the hypothesis that the scapuloperoneal amyotrophy associated with distal sensory loss may represent a variant of type I hereditary motor sensory neuropathy.
Multiple Sclerosis Presenting as Isolated Oculomotor Nerve Palsy
Authors: Uitti Ryan J. , Rajput A.H. 
Published: July 2016

ABSTRACT:A 23-year old woman came to the emergency room with an isolated oculomotor nerve palsy (including pupillary dilatation) of rapid onset. Investigations and history revealed no cause. The subsequent course of events indicated a diagnosis of multiple sclerosis. While the third nerve has been shown to be involved during the course of multiple sclerosis, this is the first report of a case presenting as an isolated oculomotor nerve paralysis.
Bilateral Facial Paralysis Following Ethylene Glycol Ingestion
Authors: Mallya K.B. , Mendis T. , Guberman A. 
Published: July 2016

ABSTRACT:Bilateral facial weakness, diminished hearing and dysphagia developed in a patient with a large overdose of ethylene glycol. Our case and two others previously reported draw attention to the fact that cranial nerve dysfunction, especially bilateral facial palsy, may occur in addition to encephalopathy, coma and renal failure with ethylene glycol ingestion.
Hemifacial Spasm in Childhood
Authors: Ronen Gabriel M. , Donat Jeffery R. , Hill Alan 
Published: July 2016

ABSTRACT:A nine-year-old girl presented with right hemifacial spasm (HFS) of 27 months duration. Stimulation of the right supraorbital nerve produced synkinesis of the ipsilateral facial muscles. Latency and amplitude of the blink reflex were equal bilaterally. Other investigations including computerized tomography (CT) were negative. Treatment with carbamazepine was not helpful. This is the third reported case of HFS in childhood.
X-Linked Hydrocephalus
Authors: Kuzniecky Ruben I. , Watters Gordon V. , Watters Lise , Meagher-Villemure Kathleen 
Published: July 2016

ABSTRACT:Two French-Canadian families with seven cases of hydrocephalus in two generations are presented. The pattern of inheritance is consistent with an X-linked recessive transmission. The clinical and pathologic characteristics of this entity are reviewed. The anomaly of adducted thumbs was present in one patient and its cause is considered. The hypothesis of primary hydrocephalus and secondary compression of the aqueduct as the mechanism for aqueductal stenosis is discussed.
Animal Models of Alzheimer's Disease: Behavior, Pharmacology, Transplants
Authors: Haroutunian V. , Kanof P.D. , Tsuboyama G.K. , Campbell G.A. , Davis K.L. 
Published: July 2016

Abstract:Physostigmine, oxotremorine, RS-86, and a combination of piracetam and lecithin, have all been studied in patients with Alzheimer's disease. Intravenous physostigmine produced a significant improvement in patients' ability to recognize words and particularly to distinguish words they had never seen before from words previously presented. A subgroup of Alzheimer's patients had a clinically meaningful improvement to treatment with oral physostigmine, with the degree of improvement correlating with the ability of oral physostigmine to increase the nocturnal secretion of Cortisol. No statistically significant differences of piracetam or piracetam and lecithin, compared to placebo were noted, however, the ratio of red cell to plasma choline might be associated with treatment responsivity. The potential therapeutic efficacy of oxotremorine proved all but impossible to assess due to concomitant adverse effects, particularly dysphoria. Results with another cholinergic agonist, RS-86, will be reported. This drug appeared to be better tolerated than oxotremorine.Animals with a kianic acid induced cortical depletion of choline acetyltransferase were found to have a significant impairment in retention of a passive avoidance task, an abnormality that was readily reversible by physostigmine, oxotremorine and 4-amino-pyridine. Cysteamine, a depletor of somatostatin, also produced a comparable deficit.
The Dissolution of Language in Alzheimer's Disease
Authors: Kertesz Andrew , Appell Julian , Fisman Michael 
Published: July 2016

Abstract:Language impairment in Alzheimer's disease has become an important clinical issue. It has been recognized for some time that the disease may begin with aphasia and even before frank aphasia develops, some of the earliest changes in a large number of individuals consist of impairment of word fluency and semantic access manifesting itself in word finding difficulty. The second major issue concerning language in Alzheimer's disease is that the cases which have early severe language impairment may represent a more progressive familial variety of disease as it has been suggested in the literature. This is still subject to controversy. Finally, it will be documented that the later stage of the disease shows language invariably impaired and goes through stages of dissolution that resemble anomic, transcortical sensory, Wernicke's and global aphasias. Accurate assessment of language may turn out to be one of the most reliable predictors of the stages of Alzheimer's disease and provides important insights into the cerebral organization of language, semantic access, relationship of semantic and episodic memory and the pathophysiology of the disease.
Aluminum Neurotoxicity — Potential Role in the Pathogenesis of Neurofibrillary Tangle Formation
Authors: Perl Daniel P. , Pendlebury William W. 
Published: July 2016

Abstract:Alzheimer's disease is a progressive neurodegenerative disease characterized neuropathologically by the development of large numbers of neurofibrillary tangles in certain neuronal populations of affected brains. This paper presents a review of the available evidence which suggests that aluminum is associated with Alzheimer's disease and specifically with the development of the neurofibrillary tangle. Aluminum salts innoculated into experimental animals produce neurofilamentous lesions which are similar, though not identical, to the neurofibrillary tangle of man. Although a few reports have suggested evidence of increased amounts of aluminum in the brains of Alzheimer's disease victims, such bulk analysis studies have been difficult to replicate. Using scanning electron microscopy with x-ray spectrometry, we have identified accumulations of aluminum in neurofibrillary tangle-bearing neurons of Alzheimer's disease. Similar accumulations have been identified in the neurofibrillary tangle-bearing neurons found in the brains of indigenous natives of Guam who suffer from parkinsonism with dementia and amyotrophic lateral sclerosis. This ongoing research still cannot ascribe a causal role of aluminum in the pathogenesis of neurofibrillary tangle formation; however, it does suggest that environmental factors may play an important part in the formation of this abnormality.
Cortical Metabolism, Acetylcholinesterase Staining and Pathological Changes in Alzheimers Disease
Authors: McGeer E.G. , McGeer P.L. , Kamo H. , Tago H. , Harrop R. 
Published: July 2016

Abstract:The local cerebral metabolic rate for glucose (LCMRgl) was determined by positron emission tomography (PET) using the 18F-fluorodeoxyglucose method in a series of Alzheimer patients and normal controls. The LCMRgl declined in the cerebral cortex with age, but the decrement was significantly greater in the clinically diagnosed Alzheimer's cases. Comparison of PET and psychological data indicated that, as the disease progressed clinically, the reduction in cortical LCMRgl and the number of cortical regions involved also increased. Variable regions of cortex were involved in the early stages but the temporal, parietal and frontal regions were most typically affected. One case coming to autopsy showed that the severity of the LCMRgl decline paralleled loss of neurons in the cortex and their replacement with astroglia.A case of Pick's disease coming to autopsy had shown a different and highly characteristic pattern of cortical metabolic defect. In this case also a poor metabolic rate was associated with extensive gliosis.Acetylcholinesterase (AChE) staining of the cerebral cortex in elderly normals and Alzheimer's disease cases with a new, highly sensitive method showed that in Alzheimer's disease there was an extensive loss of AChE-positive fibers with senile plaques frequently incorporating AChE-positive fiber debris. AChE staining of the substantia innominata area, where the cells giving rise to these neocortical fibers are presumably located, also showed evidence of degenerating cells and fibers.
Dementia in Movement Disorders
Authors: Teräväinen H. , Hietanen M. , Stoessl J. , Calne D.B. 
Published: July 2016

Abstract:Of all the movement disorders, Huntington's disease has been most consistently associated with dementia, while it is only over the last decade that intellectual and cognitive decline have been recognized as common features of Parkinson's disease. It is now known that the pathology in these two conditions reflects differential involvement of the striatum. The Huntington lesion is primarily in the caudate, while the Parkinson lesion preferentially affects the putamen. Both conditions have more diffuse pathology, and dementia may also occur in a wide range of other extrapyramidal diseases, such as progressive supranuclear palsy, the parkinsonism-dementia complex of Guam, and certain spinocerebellar degenerations. Clinicopathological correlations will be reviewed in these disorders of primarily subcortical pathology, and comparisons will be made with Alzheimer's disease, a disorder of predominantly cortical pathology.
Environmental Risk Factors for Parkinson's Disease: The Epidemiologic Evidence
Authors: Schoenberg Bruce S. 
Published: July 2016

ABSTRACT:Descriptive data from several studies suggest variations in the frequency of Parkinson's disease in different population groups. Door-to-door surveys were carried out among a biracial U.S. population (blacks and whites) and in communities in Nigeria and the People's Republic of China. The U.S. investigation revealed no substantial differences in the age-adjusted prevalence ratios by race. However, blacks in Nigeria have a much lower prevalence ratio than blacks in the U. S., suggesting an environmental etiologic factor. Prevalence ratios derived from China are also lower than the U.S. figures. Studies of temporal trends in the incidence rates in one U.S. population (Rochester, Minnesota) show virtually no change over 35 years, indicating that the primary cause(s) of Parkinson's disease must have been present in this nonindustrialized community for many years. Analytic studies generally reveal an inverse association between Parkinson's disease and cigarette smoking, although epidemiologic evidence does not support a direct protective effect of smoking. Preliminary investigations suggest an increased risk associated with the rural environment and the consumption of well water. Further studies are required to discover as yet unknown environmental factors that heighten the risk of Parkinson's disease.
Geography, Drinking Water Chemistry, Pesticides and Herbicides and the Etiology of Parkinson's Disease
Authors: Rajput A.H. , Uitti Ryan J. , Stern W. , Laverty W. , O'Donnell K. , O'Donnell D. , Yuen W.K. , Dua A. 
Published: July 2016

ABSTRACT:In 1984 we made the first observation of a correlation between early age exposure to rural environment (and drinking well water) and development of idiopathic Parkinson's disease (IPD).13 These findings were subsequently confirmed elsewhere (Barbeau, 1985 ;25 Tanner, 198526). Analysis of all early age onset IPD (EPD) cases born and raised in Saskatchewan revealed that 20 of 22 had exclusively rural exposure during the first 15 years of life. This distribution was significantly different from the general population (p = 0.0141). Further study of the EPD group included sampling and metal analysis of childhood sources of drinking water in 18 cases and 36 age and sex-matched controls. Water collected from the two groups was analyzed for 23 metals (including 7 elements implicated in the etiology of IPD). There was no difference in the metal composition of the water between the two groups.17 Finally, a review of herbicide and pesticide use in Saskatchewan agriculture was undertaken to determine if there was an increased incidence of EPD following utilization of any particular chemical. No increase was found in the incidence of EPD with the introduction of any pesticide or herbicide, including Paraquat, for agricultural use. We conclude that there is a strong correlation between early age rural environmental exposure and development of IPD. We believe well water is a likely vehicle for the causal agent, but neither water metal concentration nor any of the herbicides and pesticides used in Saskatchewan agriculture are related to the cause.
Update on Dopamine Agonists in Parkinson's Disease: “Beyond Bromocriptine”
Authors: Lang Anthony E. 
Published: July 2016

ABSTRACT:Since the initiation of bromocriptine therapy for Parkinson's disease several newer dopamine agonists have been developed. Pergolide has reached the stage of Phase 3 clinical trials and will probably be available for general use sometime in the foreseeable future. Lisuride shows most promise in its parenteral form for infusion therapy of patients with severe fluctuations. Mesulergine, another ergot-derivative and ciladopa, a new non-ergot agonist, have been withdrawn from further clinical use due to tumorogenesis in rats. It is questionable how applicable these findings are to the use of the drugs in elderly humans with parkinsonism. Recently a small number of drugs have been found to have postsynaptic dopamine agonist properties only in the setting of denervated supersensitive dopamine receptors. These agents may be particularly effective in the early treatment of patients with Parkinson's disease. This paper will review a number of the dopamine agonists which have been developed since the introduction of bromocriptine therapy. Several of these have shown beneficial effects in early clinical trials while others show promise in preclinical studies of animal models of parkinsonism.
Action of 5-Hydroxytryptamine, Substance P, Thyrotropin-Releasing Hormone and Clonidine on Motoneurone Excitability
Authors: Bédard Paul J. , Tremblay L.E. , Barbeau H. , Filion M. , Maheux R. , Richards C.L. , DiPaolo T. 
Published: July 2016

ABSTRACT:We have investigated the influence on the excitability of lumbar motoneurons of 5-hydroxytryptamine (5-HT), substance P and thyrotropin releasing hormone (TRH), three substances which coexist in the same bulbospinal descending pathway and end in large part around motoneurons. We have also studied the effect of clonidine, an alpha 2 noradrenergic agonist. This was done in spinalized rats (T5) treated three weeks before with 5-7-dihydroxytryptamine. Under those circumstances 5-HTP (LP.), 5-HT (intrathecally) TRH (LP. or I.T.) and substance P (I.T.) all elicited a strong excitation of motoneurons as measured by integrated EMG of the hindlimb muscles. Substance P reduced by almost half the subsequent response to 5-HTP, 1 hour and 24 hours later. TRH given acutely did not modify the response to 5-HTP but given chronically for twenty one days by means of Alzet minipump, markedly increased the response to 5-HTP. Clonidine by itself decreased the excitability of motoneurons and antagonized the excitatory effect of 5-HTP and TRH. In a pilot trial, cyproheptadine, a 5-HT antagonist was shown to decrease the manifestations of spasticity in patients with a partial spinal lesion. Clonidine also appears to be of potential use in the treatment of spasticity.
Valproic Acid and Plasma Levels of Primidone and Derived Phenobarbital
Authors: Bruni J 
Published: July 2016

SUMMARYFive patients were studied to determine whether kinetic interaction occurs between valproic acid and primidone. During concurrent administration of primidone and valproic acid no significant interaction was observed. Primidone and derived phenobarbital levels did not change significantly. No adjustment of primidone dose was required when valproic acid was used as adjunctive therapy.
Experimental Porphyric Neuropathy: A Preliminary Report
Authors: Sima Anders A.F. , Kennedy James C. , Blakeslee Dennis , Robertson David M. 
Published: July 2016

SUMMARY:An experimental model for the study of porphyric neuropathy is presented. Injection of either tetraphenylporphinesulfonate (TPPS), hematoporphyrin derivative (HpD), or delta-aminolevulinic acid (ALA) into mice resulted in markedly decreased motor nerve conduction velocity (MNCV). The MNCV returned to normal within one week following the injection of large doses of A LA, and within three weeks following the injection of close to lethal doses of HpD. hut there was no recovery of nerve function within 60 days following injection of substantially smaller doses of TPPS. Ultrastructural examination of motor nerves at various times following TPPS injection revealed the gradual development of structural abnormalities. Ultrastructional examination of the same nerves after a single dose of either A LA or Hp D failed to demonstrate any abnormalities.The present observations call for precaution as to the use of TPPS as photosensitizer in human cancer treatment.
Chronic Levodopa and Renal Function
Authors: Hoehn Margaret M. 
Published: July 2016

SUMMARY:Renal function studies were performed in seventeen patients, under metabolic ward conditions, before the initiation of therapy with levodopa. These studies were repeated during the first two to three weeks of treatment and, again, after one to two years of chronic therapy. There were no significant differences between the results of pre- and post-therapy studies, except that the blood urea nitrogen was slightly, but significantly, elevated in the nine patients who had been on the drug for one to two years. During the early weeks of treatment, there was an insignificant trend towards hypotension and increased excretion of sodium. This did not persist in those patients followed for one to two years after the initiation of treatment. Glomerular filtration rate, as measured by an endogenous method, was unchanged by chronic therapy with levodopa. These results are in contrast to the acutely increased glomerular filtration rate, as measured by an exogenous method, and the increased sodium excretion following a single dose of levodopa or dopamine.
A Case of Huntington’s Chorea With Unilateral Ectopic Gray Matter
Authors: Nauta Haring J.W. , Platts M.E. 
Published: July 2016

SUMMARY:The authors report a single case of Huntington’s chorea associated with a unilateral focus of ectopic gray matter. The patient’s symptoms began at age 45 and included typical involuntary jerking movements of all extremities and face. Mental deterioration may have proceeded the choreiform movements. The family history was positive for Huntington’s chorea. Pneumoencephalogram showed atrophy of the caudate nuclei bilaterally early in the disease. The patient improved transiently with haloperidol therapy.The major pathologic features included mild generalized cerebral atrophy with marked atrophy of the caudate nuclei and putamen. Within the white matter of the left frontal lobe, there were irregular nodules of ectopic gray matter with an overall diameter of 2 cm.The rarity of either unilateral ectopia or Huntington’s chorea alone, makes it impossible to judge if the two lesions might be linked by a common pathologic mechanism. The significance such a linkage might hold is discussed in light of several currently postulate pathologic mechanisms.
Intraoperative Regional Cerebral Blood Flow During Carotid Endarterectomy
Authors: Rowed David W. , Vilaghy Miklos I. 
Published: July 2016

SUMMARY:Regional cerebral blood flow (rCBF) during internal carotid artery (ICA) occlusion for endarterectomy can be measured without inconvenience using the probe holder illustrated.When mean ipsilateral hemispheric CBF exceeds 20 ml/100 gm/min, an intraluminal bypass is not necessary (63% of patients), except in patients with extensive cerebrovascular disease in whom rCBF should also exceed 20 ml/100 gm/min in all areas. ICA “stump” pressure is falsely high in about 20% of patients, and is therefore not a dependable criterion for selecting patients who need shunting.While intraoperative shunting is capable of restoring pre-occlusion CBF levels, it does not eliminate the risk of intraoperative ischemic neurological deficit of probable embolic origin.
Effects of Ethyl Ester Derivatives of Valproic Acid Metabolites on Pentylenetetrazol Seizures in Mice
Authors: Bruni J. , Hammond E.J. , Wilder B.J. 
Published: July 2016

SUMMARY:The anticonvulsant activity of the ethyl esters of the major valproic acid metabolites was assessed against minimal pentylenetetrazol seizures in adult male ICR mice. The ethyl ester 3-hydroxy-propylpentanoic acid was found to possess significant anticonvulsant activity.
Plasma Exchange of Malignant Multiple Sclerosis
Authors: Warren K.G. , Gordon P.A. , McPherson T.A. 
Published: July 2016

SUMMARY:A 17-year old female with “malignant” multiple sclerosis died 45 weeks after the onset of the disease. Postmortem examination confirmed the clinical diagnosis of multiple sclerosis. After 30 weeks of rapid clinical progression, the patient was treated with two courses of plasma exchange therapy without immunosuppression. This form of therapy caused a drop in cerebrospinal fluid myelin basic protein levels in one instance, but not in another. Controlled clinical trials are required to determine whether plasma exchange therapy is of value in the management of patients with multiple sclerosis, and the monitoring of cerebrospinal fluid myelin basic protein, if elevated, may be one useful marker to follow as part of these studies.
Cerebrospinal Fluid and Blood Thiamine Concentrations in Phenytoin-Treated Epileptics
Authors: Botez M.I. , Joyal Claude , Maag Urs , Bachevalier Jocelyne 
Published: July 2016

SUMMARY:Thiamine and folate levels in blood and cerebrospinal fluid (CSF) were determined by microbiological assays in 23 control subjects and 11 phenytoin-treated epileptics. There was no significant difference between the two groups for serum and CSF folate levels. There was, however, a statistically significant difference between the groups for both whole blood thiamine and CSF thiamine levels. Epileptic patients being treated with phenytoin had lower values than control subjects (p < 0.001)
La Méningite de Mollaret
Authors: Saint-Martin M. , Duplantis F. , Laverdière M. , Lachapelle J. , Rousseau S. , Roy L.E. , Boileau J. 
Published: July 2016

ABSTRACT:Clinical and biological aspects of a case of Mollaret meningitis are reported. Some of the typical characteristics observed in the CSF of this rare entity will be discussed.
Electrophysiological Investigation of the Auditory System in Friedreich’s Ataxia
Authors: Taylor M.J. , McMenamin J.B. , Andermann E. , Watters G.V. 
Published: July 2016

ABSTRACT:Auditory brainstem responses (ABRs) and cortical auditory evoked responses (AERs) were studied in a series of 16 Friedreich’s ataxia patients who varied in age, degree of clinical involvement and duration of the disorder. The ABRs were markedly abnormal in all but the youngest patient, and the abnormalities reflected the severity and duration of the disease. The latencies of the AERs were significantly longer in the Friedreich’s ataxia patients compared to normal controls, suggesting cortical as well as peripheral involvement of the auditory system. These data are discussed in terms of the neuropathology of the disorder and the similarities with the other sensory systems in Friedreich’s ataxia patients.