bluuebunny/crossref_metadata_2025 huggingface dataset:
Bromocriptine and the Clinical Spectrum of Parkinson's Disease
Authors: Riopelle Richard J.
Published: July 2016

As the direct agonist with the widest clinical use, bromocriptine provides a unique window into the clinical spectrum of Parkinson's disease. The efficacy of bromocriptine for therapy of de novo Parkinson's disease has recently been confirmed using a double-blind design with L-Dopa (Sinemet). Over a period of 5.5 months, bromocriptine was found to be as effective as L-Dopa in reducing the functional and neurological disability of Parkinson's disease. This study complements others and demonstrates a role for bromocriptine as de novo therapy. A longitudinal study comparing bromocriptine with L-Dopa is underway, but previous observations with bromocriptine suggest modest, transient beneficial effects with significantly less fluctuation of disability and less dyskinesia when used alone or in combination with L-Dopa. The transient benefits of bromocriptine on progressive disability suggest that both pre-and post-synaptic defects are eventually involved in Parkinson's disease. While agonists with improved efficacy and minimal side effects are required for symptomatic treatment of Parkinson's disease, strategies to protect pre- and post-synaptic neuron populations against progressive dysfunction must be developed.
D-1 and D-2 Agonists in Parkinson's Disease
Authors: Lieberman Abraham N. , Goldstein Menek , Gopinathan Govindan , Neophytides Andreas
Published: July 2016

We have evaluated 5 DA agonists-bromocriptine, lergotrile, lisuride, pergolide, and mesulergine in studies encompassing 278 patients with advanced PD. In most of our patients the DA agonist was added to levodopa. Most of our patients were no longer satisfactorily responding to levodopa. Previous attempts at managing these patients by changing the dose of levodopa (increasing or decreasing it), the treatment schedule, or the ratio of levodopa to carbidopa or by temporarily discontinuing levodopa [drug holiday] were unsuccessful. The majority of our patients had diurnal fluctuations in performance, either “wearing off” or “on-off” phenomena. The addition of a DA agonist resulted in a decrease in parkinsonian disability in most patients and a decrease in the severity of the diurnal fluctuations in performance. Improvement in many patients was maintained for at least 2 years. Adverse effects included mental changes, dyskinesias, orthostatic hypotension, and nausea. All of the adverse effects were reversible when the agonist was decreased or discontinued. As a group the agonists behaved similarly but individual patients often responded better to one agonist than another. The main role of agonists is in combination with levodopa in the treatment of patients with early PD who have not yet developed dyskinesias or diurnal fluctuations in performance.
Multiple Sclerosis: Assessment of Disease Progression and Effects of Treatment
Authors: Paty D.W.
Published: July 2016

MS could well be a two stage disease. The first stage involves the sequential development of multiple small lesions, mostly inflammatory, that accumulate at a given rate. The second stage could be that of consolidation and confluence of lesions that involves not only demyelination but gliosis. MRI now gives us an opportunity to watch the evolution of these processes and also to monitor treatment effects. It is only after the evolution of this process is understood that we can design rational therapies directed toward the prevention of spasticity in MS.
Chorea
Authors: Klawans Harold L.
Published: July 2016

A variety of neurotransmitters have been implicated in the pathophysiology of chorea as exemplified by Huntington's chorea. These include dopamine, serotonin, acetylcholine, GABA and a variety of neuropeptides including substance P and somatostatin. Despite biochemical data that suggests that alterations in other neurotransmitters may be of greater significance, pharmacologic data still supports a major role of dopamine in the actual clinical manifestation of chorea.
Progressive Supranuclear Palsy
Authors: Jankovic Joseph , Rohaidy Haydee
Published: July 2016

Progressive supranuclear palsy (PSP) was first recognized as a distinct morbid entity by Richardson, Steele and Olszewski a quarter century ago. Subsequent experience has confirmed and extended their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigidity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is an important cause of parkinsonism. Its etiology remains obscure. Familial concentrations have not been observed.

Some cases exhibit no oculomotor dysfunction. Dementia is usually mild. Recent neuropsychological studies have defined features consistent with frontal lobe cortical dysfunction. Seizures and paroxysmal EEG activity may occur.

CT and MRI scans show midbrain atrophy early and later atrophy of the pontine and midbrain tegmentum and the frontal and temporal lobes. PET scans have shown frontal hypometabolism and loss of striatal D-2 dopamine receptors. Postmortem studies have documented involvment of both dopaminergic and cholinergic systems. Treatment remains palliative and unsatisfactory.
Paul Flechsig’s System of Myelogenetic Cortical Localization in the Light of Recent Research in Neuroanatomy and Neurophysiology Part I
Authors: Meyer Alfred
Published: July 2016

In the first part of the paper supplementary information on Flechsig’s personality and his attitude to work is given which had not been sufficiently appreciated in English biographical notices. This is followed by translated excerpts from “Gehirn and Seele”, published in 1896, in which Flechsig introduced his myelogenetic localization of the cerebral cortex.

The second part is mainly a review of recent advances in neuroanatomical and neurophysiological research within those cortical regions in which Flechsig had been interested. A comparison between recent advances and Flechsig’s work demonstrates that Flechsig had definitive ideas on the solution of some of the outstanding problems but had been unable to resolve these with the techniques available at his time.
A Comparison of Visual, Brainstem Auditory, and Somatosensory Evoked Potentials in Multiple Sclerosis
Authors: Purves S.J. , Low M.D. , Galloway J. , Reeves B.
Published: July 2016

Multimodality evoked potentials testing including PVEPs, SEPsand BAEPs was done in 112 patients who were known or suspected to have multiple sclerosis. The incidence of abnormal evoked potential findings in each of these systems was considered in patients in the different diagnostic categories of M.S. Results were also evaluated with respect to the presence of abnormal clinical visual, somatosensory, or brainstem signs. The PVEP was found to be the most frequently abnormal even in patients without clinical involvement in the visual system (45% of patients with definite, probable, or possible M.S.), the SEP was less frequently abnormal in the absence of clinical signs (35% in patients with M.S.), and the BA EP showed the lowest frequency of abnormalities in patients without brainstem signs (14% in patients with M.S.). Combining the three types of evoked potentials significantly increased the percentage of M. S. patients having abnormal findings, compared to any of these tests alone, with 97% of “definite” M.S. patients, 86% of “probable” M.S. patients and 63% of “possible” M. S. patients having at least one of these EP tests abnormal.
Paul Flechsig’s System of Myelogenetic Cortical Localization In the Light of Recent Research in Neuroanatomy and Neurophysiology Part II
Authors: Meyer Alfred
Published: July 2016

In 1896 Flechsig was still under the influence of Hermann Munk (1890) who had assumed that the cortex consisted of sensory centers, each equipped with efferent descending projection systems — olfactory, visual, auditory and — anterior to the last, the somatic sensory sphere (“Körperfühls-phäre”). This sphere also contained the kinaesthetic images of motor action induced by efferent fibers in the subcortical ganglia which, from Burdach (1819-26) until the time of Meynert, had been widely regarded as the highest motor centers. In 1905a, however, Flechsig had shed most of the influence of Munk. By then he had taken cognizance of the research of Grunbaum and Sherrington (1902, 1903) and realized that “in the highest anthropoid all stimulable points of the type of Fritsch and Hitzig are concentrated within one gyrus, namely the precentral gyrus, so that we can call this the motor gyrus … in man, too, the motor centers of the type of Fritsch and Hitzig are restricted to the precentral gyrus and the immediately adjacent part of the first frontal convolution. The central sulcus here too is the posterior boundary of the motor zone … Occasionally a larger or smaller part of the motor centers, especially of the fingers, gets into the postcentral convolution, but, according to my own embryological studies, only exceptionally. As a rule we have to assume that all voluntary motor impulses leave the cortex from the precentrai gyrus …”
Polymyositis Presenting with Distal and Asymmetrical Weakness
Authors: Sundaram Mecheri B. , Ashenhurst Edward M.
Published: July 2016

A 52 year old female with polymyositis presenting with distal and asymmetrical weakness is presented. Similar reported cases are reviewed. These patients respond favorably to steroids. Increased awareness of this unusual distal polymyositis should avoid delay in diagnosis.
Spinal Cord Swelling in Multiple Sclerosis
Authors: Feasby Thomas E. , Paty Donald W. , Ebers George C. , Fox Allan J.
Published: July 2016

Three cases of suhacutely progressive spinal paraparesis had myélographie evidence of spinal cord swelling suggesling the diagnosis of intramedullary tumour. The diagnosis of multiple sclerosis (MS) was made in each case with the aid of CSF protein electrophoresis, evoked responses and CT scan. Because acute MS may produce cord swelling simulating tumor or syrinx, these additional investigations may he necessary to make a correct diagnosis.
Pasteurella Multocida Subdural Empyema: A Case Report
Authors: Khan M.I. , Chan R.
Published: July 2016

The first case of Pasteurella Multocida subdural empyema is reported. This was secondary to hematogenous spread from the pharynx to a chronic subdural hematoma. Positive cultures were obtained from the primary source, the blood and the subdural space. The patient was discharged with no neurological deficit following surgical drainage and parenteral antibiotics. The incidence, predisposing factors, clinical features, bacteriology, diagnostic procedures, treatment and mortality of subdural empyema are briefly discussed.
Abnormal Proximal Axons of Clarke’s Neurons In Sporadic Motor Neuron Disease
Authors: Averback P. , Crocker P.
Published: July 2016

Filamentous axonalmasses, similar to the abnormal enlargements found in the ventral horns, were found in Clarke’s nucleus in six cases of sporadic A LS. Clarke’s nucleus is generally thought to be normal in sporadic A LS, but the present findings, in conjunction with distal spinocerebellar degeneration which is often described, suggest that this system is also pathologically involved.
Viewpoint: What the Marine MolluscAplysiaCan Tell the Neurologist About Behavioral Neurophysiology
Authors: Ruben Peter , Goldberg Jeff , Edstrom Jon , Voshart Karen , Lukowiak Ken
Published: July 2016

Only recently has man begun to regard himself as mundane and not divine. This conceptual liberation has allowed him to ask frank questions concerning the physical and chemical mechanisms which determine or affect his behavior. Unfortunately the answers to these questions have been slow in coming. The reasons for this are two-fold: Basic ethical considerations preclude the experiments necessary to investigate the neural substrates of human behavior in man. Further, man’s behavior and nervous system are both so enormously complex and subtle, it is therefore unlikely that much real fundamental knowledge could be gained from such experiments if performed. It is more expedient to study simple behavior in simpler organisms than man to understand how nervous systems operate in general and, it is hoped, to eventually gain a better understanding of the human in particular. This tactic is known as the “model systems” approach. By discovering the strategies adopted by less complex nervous systems to deal with simple situations one can devise a realistic model of the neural mechanisms that control more complex behavior in more advanced animals.

Many animals have served as valuable sources of model systems. Among them the marine gastropod molluscAplysiahas received considerable attention. In comparison to the human nervous system with approximately 50 billion neurons, theAplysianervous system contains relatively few neurons — about 20,000. Furthermore the study of theAplysianervous system has several other advantageous characteristics. A number of forms of behavioral plasticity that are found in all higher metazoans including man are also found in theAplysia. These simple but non-trivial types of behavioral plasticity include habituation, sensitization and associative learning as well as easily defined qualities of neural function which we choose to call “behavioral states”. In addition the nervous system is composed of neurons which are large and, in many cases, easily identified by anatomical and physiological criteria so that the “same” cell can be studied in more than one animal under more than one set of experimental conditions. The cell bodies of the neurons inAplysia, from which electrical recordings can be fairly easily obtained, are electrically close to their dendrites so that changes in postsynaptic potentials occurring during modifications of behavior can be monitored.
An Electroretinal and Visual Evoked Potential Study in Friedreich’s Ataxia
Authors: Kirkham T. H. , Coupland S. G.
Published: July 2016

We made an electroretinographic (ERG) and visual evoked potential (VEP) study of 12 patients with Friedreich’s ataxia whose diagnosis was established using the Quebec diagnostic criteria. ERGs and VEPs were elicited to the same stimulating conditions. Flash evoked luminance changes and pattern-specific evoked potentials to check and diamond stimuli were used. Statistical analysis of the data was made using independent sample t-tests. Significant VEP delays were present under all test conditions. The presence of significant interocular and interhemispheric delays as well as evidence of abnormal temporal dispersion of the VEP response suggest there to be both diffuse anterior visual system disease and retrochiasmal involvement in Friedreich’s ataxia. The implicit times of the ERG b-waves were statistically within normal limits but the waveforms were of low amplitude and deformed and there were significant interocular implicit time differences. These ERG results suggest there are retinal conduction abnormalities in Friedreich’s ataxia which possibly play a role in the genesis of the abnormal VEPs.
Neonatal Myotubular Myopathy: Neuropathy and Failure of Postnatal Maturation of Fetal Muscle
Authors: Sarnat Harvey B. , Roth Sanford I. , Jimenez Jorge F.
Published: July 2016

The natural course of the pathologic features in striated muscle was studied in a full-term infant with myotubular myopathy. At 5 days of age a muscle biopsy revealed that more than 90 percent of muscle fibers fulfilled histologic, histochemical and electron microscopic criteria of fetal myotubes. The infant died unexpectedly at 9 months of age from spontaneous rupture of a multifocal cavernous hemangioma of the liver. Postmortem examination revealed that progressive maturation of the fetal muscle had not occurred postnatally, and more than 90 percent of myofibers were still apparent myotubes. This maturational arrest was generalized to all striated muscles. The only changes detected since the neonatal period were hypertrophy of the small population of large fibers, but with minor cytoarchitectural alterations, and loss of the incomplete histochemical differentiation with ATPase stains or dedifferentiation not attributed to postmortem diffusion. Involvement of the gubernaculum testis accounted for the undescended testicles. The brain and spinal cord appeared normal. Evidence of degenerating and regenerating axons in the sciatic nerve suggested that the etiology of this maturational arrest of fetal muscle may be neurogenic.
Stroke in Coronary Bypass Surgery
Authors: Martin W.R. Wayne , Hashimoto Stanley A.
Published: July 2016

A retrospective review of 253 patients who underwent coronary bypass surgery was performed. Eight (3.2%) had suffered a stroke either intra-operatively (4) or during the early post-operative period (4). A comparison of pre-operative and intra-operative variables between the stroke group and the stroke-free group showed a significant difference only for the hemoglobin level (p < 0.01). All intraoperative strokes occurred in patients who had undergone femoral cannulation; it is suggested that this may have been a source of embolic material in these patients. An embolic origin for most of the strokes (both intra-operative and post-operative) is postulated on the basis of clinical and pathological findings.
Extramedullary Hematopoiesis Simulating Parasagittal Meningioma
Authors: Kandel R.A. , Pritzker K.P.H. , Gordon A.S. , Bilbao J.M.
Published: July 2016

We report a patient with polycythemia vera and myeloid metaplasia of nine years duration who developed raised intracranial pressure related to a mass obliterating the sagittal fissure. Although clinically and radiologically simulating a meningioma, biopsy revealed extramedullary hematopoiesis involving the falx cerebri. Although uncommon, this case illustrates that myeloproliferative disease can present with symptomatology and radiologic features similar to primary intracranial tumours.
Scoliosis in Friedreich’s Ataxia
Authors: Allard P. , Dansereau J. , Thiry P.S. , Geoffroy G. , Raso J.V. , Duhaime M.
Published: July 2016

The preliminary results based on a three year study on the evolution and management of scoliosis in Friedreich’s ataxia are presented. Thirty-two patients were followed in the Neuromuscular Disease Clinic at Sainte-Justine Hospital where standardized spinal radiographs were taken periodically with the Scoliosis Chariot and the Throne.

Pathomechanics — Between the age groups I (5 – 10 years) and II (10 – 15 years), a substantial increase in the Cobb values occurs. Associated with it, an increase was observed in the thoracic and thoraco-lumbar projected surface area indices. The relative torsion between the thoracic and lumbar segments was associated with a sudden increase in the Cobb measurements.

A computer program which generates a tri-dimensional display of the spine from the coordinates obtained from the standardized radiographs has been adapted to study the importance and influence of the thoracic torsion on the progress of the scoliosis and on the evolution of the total spinal deformity.

Management — Prevention of the progression of established curves was our main objective. Careful examination of the spine, depending on the age of the child, in our preliminary study, stimulated early orthopaedic treatment in any curve of 20° or more. There was always concern for curves of 30° or more. In the growing child, bracing which was recommended is now under a more thorough investigation. The use of spinal pacemakers is being clinically evaluated.

In the older child, the curve was usually stable after sixteen years of age. Surgery was usually attempted in curves over 40° in the growing child. The same curve was usually stable after the growth period.

For the non-ambulatory patients, the present study suggested the prescription of a molded seat with the following characteristics: i) a posterior lumbar support, ii) low thoracic lateral supports and Hi) a slight inclination of the seating system. This was presumed to be beneficial in maintaining stability of the spine.
Cushing’s Disease: 50 Years Later
Authors: Hardy Jules
Published: July 2016

The recent improvements in transsphenoidal microsurgery have once again placed the emphasis on the basophilic adenoma of the pituitary as the etiology of the disease originally described by Harvey Cushing fifty years ago.

In 75 operated cases, tumors were found in 60 cases: 52 were microadenomas, 4 were larger adenomas and 4 were invasive. In 15 cases, no tumor was found at surgery; a selective partial central hypophysectomy was performed in 11 cases and a total hypophysectomy in 4. Among the latter, 3 additional microadenomas were found at pathology. The majority of the microadenomas were located in the center of the gland most often near or within the neural lobe.

In 59 patients with a non invasive tumor, initial cure was obtained in 52 (88%); only 1 of the 4 patients with an invasive tumor was cured.

In the 12 cases without surgical or pathological tumor, partial or total hypophysectomy resulted in 10 cures (83%).
Distribution of Dopamine in 35 Subregions of the Rat Caudate-Putamen: A High Performance Liquid Chromatography with Electrochemical Detection Analysis
Authors: Di Paolo Thérèse , Daigle Michel , Dupont André
Published: July 2016

Topographical variations in the endogenous content of dopamine were studied in the rat caudate-putamen. For this purpose, a high performance liquid chromatography with electrochemical detection procedure for the determination of norepinephrine, epinephrine and dopamine in nervous tissue samples is described. Tissue preparation required only homogenization in acidic solution and centrifugation prior to application to the chromatograph. Detection limits in the low picogram range were found for the catecholamines separated. This assay was used in combination with a micropunch dissection technique. The performance of this analytical technique is illustrated by the detailed mapping of dopamine content in 35 subregions of the rat caudate-putamen. The distribution of dopamine varies along the major axis of the nucleus.
Spino-Cerebello-Cerebral Degeneration with Amyloid Plaques (Gerstmann, Sträussler, Scheinker Syndrome)
Authors: Dolman Clarisse L. , Daly Lyle L.
Published: July 2016

We report the case of a man who died at age 40 after a 9 year illness resembling a hereditary spinocerebellar degeneration, but accompanied also by brain stem signs and dementia. Autopsy disclosed lesions of combined spinocerebellar and ponto-cerebellar degeneration, but also damage to many brain stem nuclei. Prominent amyloid plaques disseminated through the brain indicated a relationship to the Kuru-Creutzfeldt-Jakob group of diseases. Transmission experiments are underway at the National Institutes of Health.
The Neurology of Alice in Wonderland
Authors: Murray T.J.
Published: July 2016

Charles Dodgson, better known as Lewis Carroll, author of the famous Alice stories, developed migraine and associated visual symptoms late in life. There has been considerable speculation that the bizarre phenomena and weird visual imaginery in Alice stories was directly related to the author’s migraine.

This paper reviews several aspects of the character and health of Lewis Carroll including his shy, introspective personality, his stuttering and his attraction to young girls. It is concluded that there is no connection between the visual symptoms of migraine and the phenomena described in the Alice stories which were written over 25 years before the author developed migraine in his mid-fifties.
A Prospective Study of 50 Cases of Familial Parkinson’s Disease
Authors: Roy Madeleine , Boyer Liette , Barbeau André
Published: July 2016

In a recent paper (Barbeau and Pourcher, 1982) we demonstrated that so-called “idiopathic” Parkinson’s disease is not a homogeneous entity, and defined the existence of a sub-group of patients with genetic parkinsonism. To investigate this last possibility, and to uncover possible metabolic clues as to the etiology of such cases, we carried out a prospective study of 50 kindreds with “familial” parkinsonism. Two control groups were similarly studied: 50 kindreds with essential tremor (neurological control group) and 50 kindreds originating from spouses of the previous patients (non neurological control group). We uncovered two main patterns of genetic transmission within the parkinsonian patients: a parkinsonism related to dominant essential tremor (34 kindreds; 10% of all Parkinsonians) and a recessive “akineto-rigid syndrome” (10 kindreds; 3–4% of all Parkinsonians). A further 4 kindreds assumed a pseudo-dominant pattern but were probably recessive. Finally 2 kindreds were obviously other entities presenting as “phenocopies” of Parkinson’s disease. Metabolically, hyperthyroidism appeared to be more frequent in essential tremor and “essential-tremor related parkinsonism” kindreds, while hypothyroidism and possibly hypoparathyroidism (post surgery) seemed more frequent in the recessive akineto-rigid syndrome kindreds.
Lower Motor Neuron Syndrome Following Radiotherapy
Authors: Horowitz Sandra L. , Stewart John D.
Published: July 2016

A 33 year old man developed bilateral leg weakness two years following radiotherapy to the pelvis and lower abdomen for the treatment of a seminoma. Clinical and electrophysiological examinations and nerve and muscle biopsies suggest that this is an example of either post-irradiation myelopathy selectively affecting the anterior horn cells in the lower spinal cord or a purely motor lumbo-sacral radiculopathy.
Tuberous Sclerosis in an Infant of 28 Weeks Gestational Age
Authors: Sharp Daniel , Robertson David M.
Published: July 2016

A case of tuberous sclerosis diagnosed at autopsy in a neonate at 28 weeks gestation is described. This is believed to be the earliest case yet reported. The CNS lesions consist of cortical tuberosities, heterotopic white matter nodules and subependymal nodules. These nodules consist predominantly of giant astrocytes but a small undifferentiated cell component is present as well. Multiple cardiac rhabdomyomas are also present. An explanation for neuronal hypocellularity within the cortical tuberosities is suggested.
Bromocriptine in the Long-Term Management of Advanced Parkinson’s Disease
Authors: David Grimes J. , Hassan Mohamed N.
Published: July 2016

Thirty-seven patients with advanced Parkinson’s disease who initially tolerated, and responded to bromocriptine therapy were followed for 12 to 50 (mean 28) months. Using a method of gradual increase of bromocriptine, with concomitant levodopa reduction, the peak effect of the drug was apparent by three months, at which time the mean daily dose of bromocriptine was 23.9 mg and Sinemet (levodopa + carbidopa) had been reduced by 34 percent.

Eight patients had sustained improvement without further drug changes for an average of 29 (range 14–50) months. After periods of improvement varying between 3 and 30 months, 29 patients had a fall-off from peak effect. Peak effect was regained in 21 of these 29 patients for an average of 16 additional months by initially increasing bromocriptine or Sinemet, or by eventually increasing both drugs. The main adverse effect was a confusional state which necessitated late withdrawal of bromocriptine in four patients. The best results were in younger patients with end-of-dose deterioration and levodopa induced dyskinesias.

With cautious introduction, and intermittent dosage adjustment, bromocriptine can be of long-term benefit to patients with advanced Parkinson’s disease. The majority of patients have a gradual late fall-off in effect which can frequently be reversed with dosage adjustment.
The Murine Ependymoblastoma: Growth Pattern and Survival in C57B1/6J Mice
Authors: Muller Paul J. , Shin Kyu H. , Shin Doo H.
Published: July 2016

The murine ependymoblastoma is a transplantable tumor of cerebral origin. The growth pattern and survival times of the murine ependymoblastoma implanted peripherally and intracranially in non nude C57B1/6J mice have been found to be predictable and consistent when examined by means of Tumor Cell Dose Assessment (end point solution), Tumor Growth and Survival Assessment. The results suggest that a greater tumor cell dose is required to generate peripheral tumor take than brain tumor take. This difference may result from a greater immunologic response to tumor implanted peripherally than into the immunologically privileged brain.
Mitochondrial Myopathy of Cerebro-Hepato-Renal (Zellweger) Syndrome
Authors: Sarnat Harvey B. , Machin Geoffrey , Darwish Husam Z. , Rubin Steven Z.
Published: July 2016

The muscles of four infants with cerebro-hepato-renal (Zellweger) syndrome were studied during life and/or at necropsy. A mitochondrial myopathy was demonstrated, similar to mitochondrial alterations demonstrated in liver and brain in this disease. Muscle fibers with red-staining subsarcolemmal aggregates were identified with Gomori trichome stain in two cases. Subsarcolemmal and intermyofibrillar zones of increased concentrations of NADH-TR, SDH, and cytochrome-c-oxidase activity were demonstrated histochemically in all four cases. Degenerative and cytoarchitectural changes in muscle fibers were not found. Ultrastructural studies showed large aggregates of mitochondria and increased lipid in the subsarcolemmal and intermyofibrillar spaces. Degenerative changes in mitochondria and lipid also were demonstrated, but paracrystalline inclusions were not seen. The distribution of these changes was not uniform between patients or between different muscles in the same patient. The diaphragm was affected more severely than proximal or distal muscles of the extremities. Direct involvement of muscle mitochondria in this disease may interfere with energy metabolism and contribute to the clinical findings of hypotonia, weakness, and respiratory insufficiency. The muscle biopsy with histochemistry and electron microscopy may be used as a diagnostic adjunct in suspected cases, but the variation encountered dictates caution in the interpretation of negative findings.
Infantile Huntington’s Disease
Authors: Haslam R.H.A. , Curry B. , Johns R.
Published: July 2016

A unique case of Huntington’s disease is reported because of the extremely early onset and death, and the atypical mode of presentation including severe behavioural problems and a negative family history. Although rare, Huntington’s disease must be considered along with the established degenerative disorders of white and gray matter peculiar to the pediatric population when one examines an infant or child with progressive motor deterioration, rigidity, mental retardation and behavioural abnormalities. Computed tomography is a reliable and non-invasive method of establishing the diagnosis during life.
Digital Nerve Sensory Potentials in Lesions of Cervical Roots and Brachial Plexus
Authors: Newman Michael , Nelson Norma
Published: July 2016

Normal values for the amplitudes of digital sensory nerve action potentials were obtained from 190 control subjects, using an orthodromic surface-recording technique. Lower limits were calculated, taking into account laterality, age and sex; the ratios of amplitudes between different digits were also estimated. The results in control subjects were compared with those of 20 cases of trauma involving root avulsion or extraspinal injury, 38 patients with intraspinal disease, 6 patients with extraspinal disorder and 9 cases of neuralgic amyotrophy. The variability of the dermatomes is discussed and it is concluded that digital sensory potential amplitudes offer a useful method of determining the site of a nerve lesion, provided certain limitations are recognized.
Anatomy of the Opioid-Systems of the Brain
Authors: Knigge Karl M. , Joseph Shirley A.
Published: July 2016

In 1969, Roger Guillemin and Andrew Schally independently reported the isolation and identification of the first hypothalamic neuropeptide, thyrotropin releasing factor (TRF). Following this landmark event in neuroendocrinology the ensuing years have witnessed a cascade of isolations of new neuropeptides and a virtual revolution in neurobiology. The discipline of neuroendocrinology has been remarkably impacted by the evidence that all of the “hypophysiotrophic” releasing factors presently identified are distributed widely throughout the brain with neurotransmitter or neuromodulator roles quite different from their actions of regulating the secretion of pituitary hormones. The study of these neuropeptide systems in activity of the central nervous system looms as one of the most exciting and significant eras in brain research. Although it is premature to assign specific roles for the presently known neuropeptides in pathogenesis of neurological diseases, our limited current knowledge already points to a numberof syndromes and clinical disorders which may be related to neuropeptide imbalance. Congential insensitivity to pain undoubtedly involves several peptide systems including Substance P, enkephalin, somatostatin and the endorphins. The opiocortins (β-endorphin, ACTH) of the brain as well as those of the pituitary gland are directly involved in the homeostatic mechanisms brought into action by such trauma as brain and spinal cord injury, septic shock and hemorrhage. The role of peptides in regulation of cerebral circulation will likely be identified with the etiology of stroke and the production of painful hemicranial syndromes. Among the most prevelant disorders of the human nervous system are the dementias and psychoses (Alzheimer’s disease, schizophrenia); subtle changes in brain peptide and receptor activity are being considered as responsible contributors to these diseases.
Sensory Nerve Conduction in Chronic Uremic Patients During the First Six Months of Hemodialysis
Authors: D’Amour M.L. , Dufresne L.R. , Morin C. , Slaughter D.
Published: July 2016

In an attempt to find the best electrophysiological indicator of improvement for the neuropathy present in patients with chronic renal failure undergoing hemodialysis, several types of nerve conduction were studied at the beginning of dialysis and six months later. Sural nerve conduction and late response latencies were recorded in addition to conventional motor and sensory nerve conductions. After six months of hemodialysis, sensory nerve conduction velocities in the median, ulnar and sural nerves were improved. These values appear to be the most sensitive indices of the beneficial effect of hemodialysis on the neuropathy.
Clinicopathological Conference Carcinoma of the Pituitary Gland with Metastases to Bone
Authors: Myles S.T. , Johns R.D. , Curry B.
Published: July 2016

The diagnosis of extracranial metastases from a pituitary tumor was confirmed by the use of the immunoperoxidase technique. This is believed to be the first case of carcinoma of the pituitary gland with bony metastases where the diagnosis has been confirmed in that manner.
Intermittent Treatment of Febrile Convulsions with Nitrazepam
Authors: Vanasse Michel , Masson Pierre , Geoffroy Guy , Larbrisseau Albert , David Pierre C.
Published: July 2016

Intermittent oral or rectal administration of diazepam for the prophylactic treatment of febriie convulsions has given results comparable to the continuous use of phenobarbital while limiting side effects and risks of toxicity. Since we believe that nitrazepam is a better anticonvulsant than diazepam, we performed a study to evaluate the effectiveness of this medication in the prophylactic treatment of febrile convulsions. Nitrazepam was given only when the children had fever and almost exclusively in children with a high risk of recurrence (less than 12 months of age at first convulsion; atypical convulsion; one or several previous convulsions). Thirty one children with a high risk of recurrence received nitrazepam. The rate of recurrence in this group was 19.3% after a follow-up of 16 months, compared to 45.8% in 24 children who also had a high risk of recurrence but in whom the parents refused the medication or gave it inadequately (p<0.05). Fifty one children with a low risk of recurrence also were evaluated and followed for at least 12 months (mean 15.4 months). Six were treated with nitrazepam, mostly because of parental anxiety, and none had a recurrence; of the 45 untreated children in this group, 6 (13.6%) had another convulsion. These results show the efficiency of nitrazepam in the prophylactic treatment of febrile convulsions.
A Method for Measurement of Arterial Concentration of Cerebral Blood Flow Tracer for Autoradiographic Experiments
Authors: Menon Devidas , Diksic Mirko , Meyer Ernst , Sako Kazuhiro , Yamamoto Y. Lucas
Published: July 2016

A new method for continuous monitoring of the concentration of 131I-labelled iodoantipy rine during an autoradiographic local cerebral blood flow (LCBF) experiment is described. During infusion of iodoantipy rine, radioactivity in an external femoral arteriovenous loop is monitored by a scintillation detector. Data is collected with a variable time base, and the arterial activity curve displayed visually in its entirety. The main advantage of this method is that there is no blood loss. It will therefore be of particular importance in dual-label autoradiography. This method can also be used for other gamma-emitting blood flow tracers, e.g. 123I-iodoantipyrine.
Oculoskeletal Myopathy with Abnormal Mitochondria
Authors: Bril V. , Rewcastle N. B. , Humphrey J.
Published: July 2016

A clinical, electrophysiological and pathological review of 14 patients having oculoskeletal myopathy with abnormal mitochondria was undertaken. These patients present with ophthalmoplegia, and mild skeletal muscle weakness. The clinical course is slowly progressive. Electromyographic examination shows myopathic changes. Serum enzymes are normal. The diagnosis is confirmed by skeletal muscle biopsy which shows abnormal mitochondria, including crystalloid inclusions on electron microscopy. These patients form a distinct clinical group in which the risk of sudden cardiac death is much less than it is in the Kearns-Sayre syndrome.
Genetic Linkage of the Huntington’s Disease Gene to a DNA Marker
Authors: Gusella James F.
Published: July 2016

Recombinant DNA techniques have provided the means to generate large numbers of new genetic linkage markers. This technology has been used to identify a DNA marker that coinherits with the Huntington’s Disease (HD) gene in family studies. The HD locus has thereby been mapped to human chromosome 4. The discovery of a genetic marker for the inheritance of HD has implications both for patient care and future research. The same approach holds considerable promise for the investigation of other genetic diseases, including Dystonia Musculorum Deformans.
Saccadic Intrusions and Oscillations
Authors: Sharpe James A. , Fletcher William A.
Published: July 2016

There are a number of different types of involuntary saccadic eye movements which differ from normal saccades in that they take the fovea away from its target. This article presents a classification and description of these abnormal eye movements and indicates how they may be used to increase the precision of neurologic diagnosis.
The Dopamine/Neuroleptic Receptor
Authors: Grigoriadis Dimitri , Seeman Philip
Published: July 2016

The neuroleptic/dopamine receptor, with its picomolar affinity for potent neuroleptics, is the functional dopamine receptor of the brain. This receptor has been termed the D2dopamine receptor, and it inhibits or interferes with dopamine-stimulated adenylate cyclase. This D2receptor has two states, each having different affinity for dopamine. The high-affinity state, termed D2high, has a 10 nM affinity for dopamine and is the functional correlate for dopamine autoreceptors and for the dopamine receptor in the pituitary gland. The low-affinity state, termed D2low, has a 2000 nM affinity for dopamine, and may possibly represent the desensitized state of the dopamine receptor or the functional post-synaptic receptor.
Physiology of the Basal Ganglia: An Overview
Authors: Lee Robert G.
Published: July 2016

The major anatomical connections of the basal ganglia are reviewed, emphasizing the inputs to the striatum and efferent projections from the major output nuclei, the internal segment of globus pallidus and the pars reticulata of substantia nigra. The results from lesioning experiments, electrical stimulation, and chronic recording of single neuron activity have provided a wealth of data concerning the physiology of the basal ganglia. Although the deficits resulting from disease of the basal ganglia are well recognized, the specific role which these structures play in the control of normal movements remains speculative.
Familial Subsets in Idiopathic Parkinson’s Disease
Authors: Barbeau André , Roy Madeleine
Published: July 2016

In the present paper we explore in some detail the hypothesis that the presence of familial aggregations in 10–15% of Parkinson’s disease cases is due in great part to the existence of well-defined familial subsets, rather than to chance occurrences. We describe the clinical and genetic characteristics of the two main subsets: “Essential tremor-related Parkinsonism” and the “Familial akineto-rigid Syndrome” previously identified.

The former type of Parkinsonism is associated at random, but with increased frequency, to an autosomal dominant disorder, usually essential tremor but occasionally OPCA. Two possible susceptibility factors were uncovered in this entity: an increased incidence of familial hyperthyroidism (augmentor factor) and a decreased incidence of the generally frequent HLA Haplotypes A1B8or A2B5(Protective factors).

The other presentation, the “familial akineto-rigid syndrome”, appears to be a definite disease entity with an autosomal recessive mode of inheritance (normal parents, increased incidence of identical parkinsonism in sibs, increased consanguinity rate in parents). This newly defined disorder deserves much further genetic and biochemical analysis.
Neurochemical Basis of Dementia in Parkinson’s Disease
Authors: Hornykiewicz Oleh , Kish Stephen J.
Published: July 2016

According to their mental status, patients with Parkinson’s disease can be subdivided into three groups: (1) mentally normal patients; (2) patients with severe cognitive impairment and Alzheimer-type brain pathology (neuritic plaques, neurofibrillary tangles, granulovacuolar changes); and (3) demented patients without any evidence of Alzheimer changes. Neurochemically, irrespective of the presence or absence of Alzheimer-type brain pathology, demented Parkinson patients seem to have the same disturbance of cortical cholinergic neuron function as patients with Alzheimer-type dementia (Alzheimer’s disease), namely, reduced levels of cortical acetylcholine esterase and choline acetyltransferase activity. At present, the question whether the “cortical cholinergic deficiency” is the only (or sufficient) neurochemical basis for the cognitive impairment in Parkinson patients with dementia cannot be answered with certainty; the additional role of other neurotransmitter changes known to occur in the Parkinson brain, especially loss of cortical, hippocampal and subcortical noradrenaline and/or dopamine cannot be ruled out.
Positron Emission Tomography in Movement Disorders
Authors: Martin W. R. Wayne
Published: July 2016

Positron emission tomography provides a method for the quantitation of regional function within the living human brain. Studies of cerebral metabolism and blood flow in patients with Huntington’s disease, Parkinson’s disease and focal dystonia have revealed functional abnormalities within substructures of the basal ganglia. Recent developments permit assessment of both pre-synaptic and post-synaptic function in dopaminergic pathways. These techniques are now being applied to studies of movement disorders in human subjects.
Adrenoleukodystrophy Mimicking Multiple Sclerosis
Authors: Dooley J.M. , Wright B.A.
Published: July 2016

We report a 47 year old woman who was initially diagnosed as having multiple sclerosis. She has subsequently been found to be heterozygous for adrenoleukodystrophy (ALD). The female carrier state for ALD may be associated with intermittent symptoms and it is therefore important to consider ALD in patients diagnosed as multiple sclerosis.
Le Cerveau Influence-t-il le Développement Musculaire du Foetus Humain?
Authors: Sarnat Harvey B.
Published: July 2016

Does the human fetal brain influence muscle development? The importance of suprasegmental cerebral influence on developing human fetal muscle is less well understood than the control of histochemical differentiation of muscle by the motor neuron. Muscle biopsies of 21 hypotonic infants and children with nonprogressive congenital lesions of the cerebellum and/or brainstem were studied by histochemical methods. Two neonates who died with severely dysplastic brains and no descending motor tracts had normal muscle. The others, particularly those with cerebellar hypoplasia, had delayed muscle maturation, selective predominance of type I or II muscle fibres or disproportion in fibre sizes. It is concluded that the motor unit is capable of developing normally without suprasegmental influence, but that an abnormal balance of descending impulses may alter histochemical differentiation of fetal muscle. The small ‘subcorticospinal’ pathways arising in the brainstem probably are more influential than the larger corticospinal tract because of later myelination in the latter. The muscle biopsy thus serves to provide evidence of suprasegmental disease in infantile hypotonia.
Recurrent Chorea Gravidarum in Four Pregnancies
Authors: Ghanem Quais
Published: July 2016

An Arab woman developed recurrent chorea gravidarum in all four pregnancies that went to term, and none in the three pregnancies that ended in spontaneous abortion. She also developed an acute psychosis in one of these four pregnancies. A discussion of similar cases in the literature is undertaken.
Akinetic Mutism and Parkinsonism Associated with Obstructive Hydrocephalus
Authors: Berger L. , Gauthier S. , Leblanc R.
Published: July 2016

We report the case of a patient with idiopathic aqueductal stenosis and hydrocephalus who had several episodes of akinetic mutism, each preceded by shunt malfunction, that resolved with shunt revision. She also developed a parkinson’s syndrome resistant to shunt revision but responsive to antiparkinsonian medications. The Parkinson’s syndrome and the episodes of akinetic mutism may be related to a reduction of dopaminergic input to the striatum and to the cingulate and frontal cortex brought about or worsened by ventricular dilatation.
Pseudoarylsulfatase-A Deficiency in the Neurologically Impaired Patient
Authors: Farrell Kevin , Applegarth D.A. , Toone J.R. , McLeod P.M. , Savage A.V.
Published: July 2016

The demonstration of low arylsulfatase-A (ASA) activity in leucocytes or fibroblasts is used often to establish the diagnosis of metachromatic leucodystrophy (MLD). However, low ASA activity is observed also in pseudo-ASA deficiency which may be as common as MLD. We report two patients with pseudo ASA deficiency who had abnormal neurological findings consistent with atypical MLD. Because the measurement of ASA activity is neither a sensitive nor specific method with which to establish a diagnosis of MLD, this diagnosis should be confirmed by nerve biopsy, measurement of urinary sulfatide or a cerebroside sulfate loading test, using cultured fibroblasts.
Corneal Reflex in Hemisphere Disease II
Authors: Ross R. T. , Johnston J. A.
Published: July 2016

In man, contralateral cerebral decortication or removal of a hemisphere does not permanently abolish the corneal reflex. Destruction of the contralateral thalamus or contralateral mesencephalic trigeminal tract does abolish the corneal reflex.

In the cat however, the corneal reflex remains intact after contralateral decortication, he mi sphere ctomy and total ablation of the thalamus. Some of these procedures diminished the corneal reflex or increased the threshold, but neither of these changes were seen for more than a few days post-operativ ely. In this animal however, the second reflex response, evoked by supra orbital nerve stimulation, is abolished by certain thalamic lesions and by contralateral hemi-transection of the brain stem at various levels.