bluuebunny/crossref_metadata_2025_split huggingface dataset:
Muscarinic Binding and Choline Acetyltransferase in Postmortem Brains of Demented Patients
Authors: Waller Steven B. , Ball Melvyn J. , Reynolds Mark A. , London Edythe D. 
Published: July 2016

Abstract:Postmortem human brain samples were taken from non-neurological controls as well as demented subjects who died with Alzheimer's disease (AD), multi-infarct dementia (MID), or a combination of AD and MID dementia (MIXED). Choline acetyltransferase (Ch AT) activity was measured radiometrically using [ 1 -l4C]acetyl-coenzyme A as the substrate, muscarinic binding was assayed with [3H]quinuclidinyl benzilate, and the proportion of binding associated with high affinity agonist sites was measured by carbamylcholine displacement of the radioligand.Relative to control, ChAT activity was significantly reduced (p ≤0.01) in samples taken from the temporal, frontal, and hippocampal areas of demented patients. A small elevation in Bmax was noted in the hippocampal endplate (p ≤0.01) (AD vs. control) and the H1-subiculum region (p ≤0.05) (AD vs. all other groups). In addition, the percentage of binding associated with high affinity agonist sites was greater in the frontal cortex of AD and MID samples (p≤0.05), compared with MIXED and control, and in the temporal cortex of the AD group compared with all other groups (p≤0.05). The results suggest a regionally specific upregulation of cerebral muscarinic receptors in dementia, especially in AD.
Cerebral Glucose and Dopa Metabolism in Movement Disorders
Authors: Martin W.R. Wayne , Hayden Michael R. 
Published: July 2016

ABSTRACT:The development of positron emission tomography (PET) has enabled us to perform in vivo measurements of certain aspects of regional cerebral function. Regional cerebral glucose metabolism may be readily quantified with [18F] fluoro-2-deoxyglucose (FDG) and presynaptic dopaminergic function may be studied with the labelled dopa analog 6-[18F] fluoro-L-dopa. We have applied a model to the analysis of 6-FD/PET data with which in vivo age-related changes in dopaminergic function may be demonstrated in normal subjects. With this technique, we have studied a series of asymptomatic MPTP-exposed subjects and have shown evidence of subclinical nigrostriatal pathway damage. Studies of regional cerebral glucose metabolism with FDG in early Huntington's disease have shown a characteristic impairment in caudate function which precedes the development of caudate atrophy. In addition, some asymptomatic individuals who are at risk for HD have caudate hypometabolism. We feel that, at the present time, PET provides information which is complementary to the clinical examination in establishing a diagnosis of HD. In the future these studies may also help in the investigation of at risk individuals
Vestibular Control of Muscular Tone and Posture
Authors: Markham Charles H. 
Published: July 2016

ABSTRACT:The vestibulospinal system helps to maintain upright posture and head stability. The semicircular canals and their short latency connections to the neck motoneurons, largely via the medial vestibulospinal tract, respond to angular accelerations so as to stabilize the head in space. The paired otolith organs, the utricles placed approximately horizontally, and the saccules vertically, respond to linear acceleration including gravity. Their influence leads, via the lateral vestibulospinal tract, to excitation of ipsilateral extensor motoneurons of the limbs and trunk, and to inhibition of reciprocal flexor motoneurons. Linear displacement of the otoliths leads to bracing of the limbs and body so as to maintain upright posture, and to extend the limbs so as to help in landing after sudden falls.
Treatment of Spasticity with Tizanidine in Multiple Sclerosis
Authors: Lapierre Y. , Bouchard S. , Tansey C. , Gendron D. , Barkas W.J. , Francis G.S. 
Published: July 2016

ABSTRACT:Spasticity is a frequent and often disabling symptom in MS patients. Current drugs used as antispastic agents include Dantrolene Sodium, Baclofen and Diazepam. Tizanidine (5-chloro-4-(2imidazolin-2 yl amino) -2, 1, 3-benzothialdiazole) is a new antispasticity agent that has purported central action. A double blind placebo controlled trial was performed to study the efficacy of this drug in MS patients. Sixty-six patients entered an eight week therapeutic trial and fifty-nine completed the trial. Patients were assessed at 0, 2, 3 and 8 weeks of therapy for clinical effects. Electrophysiologic tests were performed at 0 and 8 weeks.A statistically significant benefit was noted in spastic muscle groups in the legs with concomitant significant reduction in hyperactive stretch reflexes and ankle clonus. Side effects most frequently cited included dry mouth and drowsiness. Two patients developed elevated liver function test that decreased with cessation of therapy. Other clinical details, side effects and electrophysiologic data will be presented.Tizanidine appears to reduce clinical spasticity and hyperreflexia in MS patients although no change in functional status was detected. Tizanidine may well serve as an alternate antispastic agent, alone or in combination with other agents.
Local Treatment of Spasmodic Torticollis with Botulinum Toxin
Authors: Tsui Joseph K.C. , Fross Robin D. , Calne Susan , Calne Donald B. 
Published: July 2016

ABSTRACT:Fifty-six patients with spasmodic torticollis were treated with local injections of botulinum toxin. The drop out rate was 21%. The remaining 44 patients were followed for a period of 3 to 21 months. Thirty-two patients (76%) had pain relief out of 42 presenting with pain; 37 (66%) improved in the amount of sustained movements of torticollis. The efficacy was reproducible after repeated injections.
Method of Addition of Bromocriptine to the Drug Regime of Patients with Advanced Parkinson’s Disease
Authors: Grimes J. David , Hassan Mohamed N. 
Published: July 2016

SUMMARYUsing a method of a gradual increase of bromocriptine with a concomitant reduction of Sinemet® (levodopa 250 mg + carhidopa 25 mg), 19 patients with advanced Parkinson’s disease have been treated for periods of up to 22 months and 16 of them have shown improvements of varying degrees. Eighteen patients were able to tolerate bromocriptine addition, with early transient adverse effects occurring in seven cases. In contrast to several previously reported studies, it was found necessary to withdraw bromocriptine in only one case.With the drugs currently available, bromocriptine has a role in the management of patients with advanced Parkinson’s disease. The method described here may allow a greater number of patients to be given a trial with this drug.
Multiple Sclerosis and Associated Diseases: A Relationship to Diabetes Mellitus
Authors: Warren S. , Warren K.G. 
Published: July 2016

SUMMARYOne hundred multiple sclerosis (MS) patients and hospital controls were compared for coexisting illnesses, and other illnesses among their family members. The patients and controls were compared for allergy, migraine, and cancer among themselves and for a family history of these illnesses. The findings were negative. However a relationship was found between multiple sclerosis and diabetes mellitus. More MS patients than controls were either diabetic or reported a blood relative with diabetes. MS patients who reported another blood relative with MS were also more likely to report a family history of diabetes mellitus than MS patients who had no other relative with multiple sclerosis.
Immunofluorescent Staining of Rat Brain Glial Cells With Multiple Sclerosis Serum
Authors: Singh Vuendra K. , Mashal Robert D. 
Published: July 2016

SUMMARY:Antibodies directed against glial cells may be involved in autoimmunity in multiple sclerosis (MS). Using a tissue culture system, the presence of glial cell antibodies in MS-patient serum was detected through immunofluorescent technique. Thirty one of 73 MS-sera were strongly positive for anti-glial cells, 13 were equivocal and 29 were negative. The antibody staining was either cytoplasmic or associated with cell surface membrane, and involved IgG type of antibody.
Porphyric Neuropathy: An Ultrastructural and Quantitative Case Study
Authors: Thorner P.S. , Bilbao J.M. , Sima A.A.F. , Briggs S. 
Published: July 2016

SUMMARY:We report a case of acute neuropathy in a 46 year old female with porphyria variegata. Histologic, electron microscopic, and quantitative examinations of peripheral nerves were performed at onset of the neuropathy and at autopsy. The results revealed severe qualitative and quantitative changes in myelinated and unmyelinated fibers showing features indicative of an axonopathy with a distribution in keeping with a dying-back phenomenon.
Meningitis and Brain Abscess Due to Clostridium Perfringens And Clostridium Paraputrificum Following Orbital Trauma
Authors: Girouard Yves , Delage Gilles , Mathieu Jean-Pierre , Larbrisseau Albert 
Published: July 2016

SUMMARY:An 8 year old boy developed cerebral abscess and purulent meningitis due to Clostridium perfringens and Clostridium paraputrificum after trauma to the left orbit. The patient made a satisfactory recovery with surgical treatment and antibiotic therapy. A review of the literature revealed that meningitis due to Clostridia is rare and usually related to penetrating trauma, and that penetrating trauma to the orbit is associated with significant central nervous system morbidity.
Familial Benign Intracranial Hypertension and Depression
Authors: Coffey C. Edward , Ross Donald R. , Massey E. Wayne , Olanow C. Warren 
Published: July 2016

SUMMARY:Two sisters developed benign intracranial hypertension (BIH) two weeks following the resolution of a major depressive episode. The association of BIH and a major affective disorder in genetically related individuals has not been previously reported to our knowledge. Both conditions are associated with disturbances in the hypothalamic-pituitary-adrenal axis. Falling corticosteroid levels in a resolving depression may result in impaired cerebrospinal fluid absorption and subsequent BIH.
Neonatal Myasthenia Gravis in the Infant of an Asymptomatic Thymectomized Mother
Authors: Olanow C. Warren , Lane Russell J.M. , Hull Keith L. , Roses Allen D. 
Published: July 2016

SUMMARY:A case of neonatal myasthenia gravis is reported in the infant of an asymptomatic thymectomized mother with comparably elevated acetylcholine receptor (AChR) antibody titers. The mother remained asymptomatic despite elevated antibody titers while the infant became asymptomatic in association with the disappearance of the AChR antibody. It is suggested that the AChR antibody plays an essential role in the development of neonatal myasthenia gravis. It is also suggested that a thymic factor is necessary for the development of clinical symptomatology accounting for the lack of correlation between the clinical state of the mother and infant.
Urodynamic Evaluation of Patients with Hereditary Ataxias
Authors: Vezina J.G. , Bouchard J.P. , Bouchard R. 
Published: July 2016

ABSTRACT:Seventeen patients with Friedreich’s ataxia or spastic ataxia were subjected to an urodynamic evaluation. Fifty-three per cent (53%) of the patients presented with urinary symptoms consisting of urgent micturition and urgency incontinence. Cystometric evaluation showed a lack of inhibition of the detrusor in 7 patients (41%). Abnormal electric hyperactivity of the external sphincter was documented in 6 cases (37.5%) by electromyography. Some hypotheses are presented to explain the etiology of these abnormal findings.
Influence of Nicotinamide on Neurobehavioral Effects of 3-Acetylpyridine
Authors: Jolicoeur F.B. , Barbeau C.M. , Michele G. De , Barbeau A. 
Published: July 2016

ABSTRACT:The purpose of the present study was to examine the ability of nicotinamide to prevent the appearance of neurobehavioral symptoms induced by 3-acetyl pyridine (3-AP) in rats. Nicotinamide in doses of 5,50 and 500 mg/kg was injected immediately after administration of 65 mg/kg 3-AP, and neurobehavioral measurements were made at 6, 12, 24, 48 and 72 hours after injections. The effects of 500 mg/kg nicotinamide injected at 3 and 6 hours after 3-AP treatment were also investigated. The results indicate that, starting at 50 mg/kg, nicotinamide can protect animals against most of the neurobehavioral effects of 3-AP. However, the muscular rigidity induced by 3-AP can only be reversed by 500 mg/kg nicotinamide, and the depressing influence of 3-AP on locomotor activity is not blocked by any of the doses of nicotinamide tested. In terms of time course, the protective action of 500 mg/kg is seen when injected 3, but not 6 hours after 3-AP.
Glutamate and Aspartate Do Not Modify the Ataxic Gait of Acrylamide Treated Animals
Authors: Michele G. De , Jolicoeur F.B. , Barbeau A. 
Published: July 2016

SUMMARY:The purpose of this experiment was to examine the effects of intraventricular injections of glutamate and aspartate on the gait of animals rendered ataxic by the administration of acrylamide. Contrary to their previously reported corrective Influence on ataxia induced by 3-acetyl pyridine, these amino acids did not modify the ataxic gait of acrylamide treated animals. This suggests that glutamate and aspartate can act in cerebellar but not in peripheral types of ataxia in animals.
Pituitary Responses to a Neuroactive Tripeptide (TRH) in Friedreich’s Ataxia Families
Authors: Tolis G. , Mehta A. , Andermann E. , Harvey C. , Barbeau A. 
Published: July 2016

ABSTRACT:Oral glucose tolerance, thyroid function tests, as well as thyrotropin, prolactin and growth hormone release after administration of thyrotropin releasing hormone, were evaluated in patients with Friedreich’s ataxia and unaffected family members. Impaired glucose tolerance was found in the majority of family members, affected or not. Thyroid hormone levels and PRL and TSH responses to TRH, were similar in all and normal. However, GH responses to TRH were abnormal in half of the patients, but in none of the unaffected family members. Paradoxical responses to neuropeptides may characterize some Friedreich’s ataxia patients, and may predict the possibility of therapeutic maneuvers with such peptides in these patients.
Plasma Lipoprotein Lipase and Hepatic Lipase Activities in Friedreich’s Ataxia
Authors: Blache D. , Bouthillier D. , Barbeau A. , Davignon J. 
Published: July 2016

SUMMARY:Plasma triglycerides although within the normal range have been shown to be higher in Friedreich’s ataxia than in control subjects. To determine whether this difference could be ascribed to a reduced catabolism of triglyceride-rich lipoproteins, the activities of lipoprotein lipase (LPL) and hepatic triglyceride lipase (HL), released into plasma after an heparin injection, were measured in 13 cases of Friedreich’s ataxia and 14 control subjects of comparable signs. LPL was found to be significantly lower in the ataxic patients. Moreover about half of the cases clustered below the normal range for both lipase activities. This subgroup of Friedreich’s patients had significantly higher plasma triglycerides than those with normal lipase activities. Further studies are needed to relate these findings to other characteristics of the disease.
Reduced Formation of Hippuric Acid After Oral Benzoic Acid in Friedreich’s Ataxia
Authors: Barbeau A. , Bouchard R. , Cloutier T. , Bouchard J.P. 
Published: July 2016

SUMMARY:We have observed a markedly decreased formation of hippuric acid after benzoic acid load in patients with typical Friedreich’s Ataxia compared to normal control subjects. Since there is evidence for normal or even enhanced tauro-conjugation in the bile of patients with this disease, with a decreased G/T ratio, it is unlikely that co-factor or enzyme concentrations are the cause of this defect. We postulate decreased availability of the enzyme for glycine conjugation either to bile acids in the usual situation or to benzoic acid in the artefactual test condition. This could be due to the enzyme’s preference for an increased amount of taurine substrate in the liver. The relationship of this observation to the other biochemical changes observed in Friedreich’s Ataxia must still be established.
Epidural Hematoma: Report of Seven Cases with Delayed Evolution of Symptoms
Authors: Benoit B.G. , Russell N.A. , Richard M.T. , Hugenholtz H. , Ventureyra E.C.G. , Choo S.H. 
Published: July 2016

SUMMARY:Epidural hematomas occasionally have a prolonged clinical course with gradual evolution of the neurologic symptoms. Seven such cases are reviewed in this report. Although the clinical course is insidious, there are certain features which should signal the presence of a slowly expanding hematoma. After an apparently minor head injury, the patient who is usually in the younger age group, develops headache. This persists and is accompanied by other non-specific neurologic symptoms which may lead to a mistaken diagnosis of “post-concussion syndrome”. Papilledema and focal neurologic deficits eventually appear. Definitive diagnosis is made by the CT scan, although contrast enhancement may be necessary to confirm the nature of the lesion. The treatment is craniotomy and evacuation of the hematoma before serious neurologic deterioration occurs.
Pathobiology of Neurosarcoidosis and Clinicopathologic Correlation
Authors: Manz Herbert J. 
Published: July 2016

SUMMARY:In a retrospective study of 4,294 consecutive postmortem cases, there were 15 with histologically proved sarcoidosis, including 4 with CNS involvement. Whereas in previously reported autopsy series the prevalence of neurosarcoidosis was 15%, it is 27% in this review. Five cases with incidental, clinically inapparent sarcoidosis died at a mean age of 54.6 years; those six with systemic sarcoidosis, not involving the CNS, died at a mean age of 47.0 years; while those four with a variety of neurologic manifestations in addition to systemic sarcoidosis died at a mean age of 38.2 years. Thus, it appears that at one end of the spectrum of morbidity, sarcoidosis may cause no or only trivial symptoms and permit prolonged survival. At the other extreme, sarcoidosis may affect a younger population more severely. CNS involvement occurs relatively early in the course and is rather rapidly progressive, accounting for the poor prognosis, despite appropriate medical and neurosurgical management. In its turn, neurosarcoidosis may present with a variety of signs and symptoms, depending on the site of involvement along the craniospinal axis. This feature is illustrated by the manifestations of compression myelopathy, hydrocephalus with dementia, hydrocephalus with seizures and ataxia, and anosmia, blindness, seizures, and diabetes insipidus. In addition, one patient developed a Nocardia brain abscess as a complication of the altered immune system in sarcoidosis.
Brain Swelling and Ventricle Size
Authors: Ross R.T. 
Published: July 2016

SUMMARY:Cerebral ventricle size was measured in one hundred normal people, age 10 to 50 years. The measurements were obtained from computed tomographic (CT) scan examinations, using the Huckman (1975) method. Normal ventricles were significantly bigger at age 45 than at age 15. These normals were compared to groups of patients with pseudotumor cerebri, water intoxication and cerebral edema, and cerebral hypoxia and edema due to cardio-respiratory arrest.The relative ventricular size was the same in the normal group and all three disease groups. Measurement of ventricle size from CT scan images is not even a crude index of cerebral edema in these three diseases.
Sensorineural Deafness in Early Acquired Syphilis
Authors: Saltiel Philippe , Melmed Calvin A. , Portnoy David 
Published: July 2016

SUMMARY:A 36 year old male developed bilateral sensorineural deafness as the chief manifestation of secondary syphilis. Cerebrospinal fluid showed pleocytosis. Treatment with penicillin and prednisone resulted in good recovery of hearing. Initial recovery seemed dependent on corticosteroids. Deafness can complicate acquired syphilis in both early and late stages of the disease and may be its sole manifestation. Early acquired syphilitic deafness is usually the result of a meningitis affecting the eighth nerve and responds well to treatment. These features are contrasted with those of late acquired syphilitic deafness.
Delayed Auditory Tone Perception in Multiple Sclerosis
Authors: Quine Douglas B. , Regan David , Murray Thomas J. 
Published: July 2016

SUMMARY:Delays of auditory perception at three frequencies were measured in 30 multiple sclerosis patients using a pscyhophysical technique. Nineteen patients had abnormal delays at one or more tone frequencies, though 15 had normal audiograms at those frequencies. In addition, auditory acuity for left-right asynchrony was abnormally poor in 13 patients, 9 of whom had normal audiograms. Such delays of auditory perception within a restricted frequency band may provide a partial explanation for degraded speech comprehension in some multiple sclerosis patients.
Kinetics of CSF Phenytoin in Children
Authors: Koren G. , Barzilay Z. , Schachar E. , Brand N. , Danee S. , Halkin H. , MacLeod S.M. 
Published: July 2016

SUMMARY:The efficacy of intravenous phenytoin for the treatment of status epilepticus is related to the rapid entry of phenytoin into brain parenchyma. There is no information concerning the correlation between phenytoin serum and CSF concentrations in children, and the application of CSF data to clinical use. We report 7 children (2–11 yrs) who were treated or exposed to phenytoin in doses between 10.5–230 mg/kg. Lumbar puncture was performed 9 times in 6 of the patients. In one patient, an intraventricular catheter permitted successive assessment of CSF phenytoin concentrations. The ratio of CSF/serum phenytoin concentrations was 0.16 ± 0.08, with gradual increase over the first 8 hours as the serum phenytoin concentration decreased. There was good correlation between therapeutic outcome and CSF phenytoin levels higher than 2 mcg/ml. In one patient the coma state secondary to phenytoin intoxication was associated with high CSF concentration (6 mcg/ml).
Cauda Equina Paraganglioma
Authors: Tsao Ming-Sound , Bilbao Juan , Richardson Peter , Finlayson Morrison 
Published: July 2016

SUMMARY:Two paragangliomas resected from the cauda equina had similar morphology, both containing cells having dense core secretory granules with no associated sustentacular or supporting cells. The cells were arranged in characteristic lobules that were surrounded by basement membrane and separated by a fibrovascular stroma. This tumor, although rarely found elsewhere in the central nervous system, does occur in the region of the filum terminale, and causes a clinical syndrome typical of tumors in that area.
An Atypical Case of Progressive Supranuclear Palsy
Authors: Gomori Andrew J. , Sima Anders A.F. 
Published: July 2016

SUMMARYWe report a 59 year old woman who presented with double vision, nuchal pain and mild dementia. On neurological examination she demonstrated third, sixth and seventh nerve palsies and ataxia. Following intravenous ACTH and oral prednisone therapy she showed a remarkable recovery which left her with only a left facial weakness. She remained well for two years. She then developed bulbar palsy and profound dementia. Pathological examination revealed progressive supranuclear palsy (PSP). This patient demonstrated a greater variability in the course of PSP than has previously been recognized.