Showing papers in "JAMA Neurology in 1960"

Handbook of Physiology.

Abstract: This is the first volume of the proposed many-sectioned "Handbook" in which the American Physiological Society intends to present comprehensively the entire field of physiology. The scope and depth of the work may be estimated by realizing that the Section on Neurophysiology alone will comprise three volumes. The present work covers the following principal topics, all but the first of which are divided into several chapters: History of Neurophysiology (M. A. B. Brazier), Neuron Physiology (Introduction, J. C. Eccles), Brain Potentials and Rhythms (Introduction, A. Fessard), Sensory Mechanisms (Introduction, Lord Adrian), and Vision (Introduction, H. K. Hartline). To a large degree, the individual contributors have overcome the difficult task of presenting both the fundamentals and the most recent advances pertaining to their subjects. The historical development of knowledge and hypotheses about most of the particular topics is given detailed consideration, and this aspect alone should help to prevent the material

Speech and Brain-Mechanisms.

Abstract: The story of aphasia is one of the most dramatic and illuminating in the history of neurology. In this book it is well summarized in two scholarly and critical chapters by Roberts (Chaps. IV and VI). As scientific methods improve, the observations of yesterday are not satisfactory today; Broca's pathology did not satisfy Marie; the case records and autopsy findings of Marie, Henschen, Head, and others do not satisfy Penfield and Roberts. Thus, science proceeds by making closer and closer approximations to truth (which may never be attained). The "scientific fact" of today will be an outmoded approximation tomorrow. At the end of Chapter X the authors modestly sum up their important contributions as follows: "In conclusion, the first sure evidence that physicians might hope to distinguish functional units within the brain, appeared about one hundred years ago with the discovery of a speech mechanism within one hemisphere. The purpose

A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study.

Abstract: Introduction Perhaps, with the exception of adrenal insufficiency,1,2postural hypotension is coincident with involvement of the nervous system.3,4Many such cases are secondary to disorders like diabetes, syphilis, and other systemic diseases, which also affect the central or peripheral nervous system.4-12On exclusion of such disorders, however, one is left with an idiopathic orthostatic hypotension, which is almost invariably accompanied by clinical manifestation of nervous system involvement. Such patients may show wide swings in blood pressure, depending on body position, but do not have the pulse changes or symptoms, such as yawning, nausea, or increased sweating, associated with other types of syncope in which the nervous system is intact. The lesions of the nervous system present in a variety of ways, the commonest symptoms being loss of sweating, impotence, and, frequently, loss of sphincter control. Controversy exists as to whether the pathology of the nervous system is

Hippocampal Slow Waves: Distribution and Phase Relationships in the Course of Approach Learning

Abstract: The functions of the temporal lobe, and its preponderant role in the integrative aspects of social behavior, are well known (Bucy and Kluver, 13 1939) and have been the subject of recent reviews (Adey, 2 1959; Baldwin and Bailey, 8 1958). To some extent, physiological dissection of the picture of "psychic blindness" characterizing the complete bilateral temporal lobectomy has been possible with more limited resections, with aspects of hypersexuality, loss of memory, taming, and exaggerated rage responses, each reported as the dominant aspect of a variety of limited resections in man and animals (Adey, 1 1958; Bard and Mountcastle, 10 1948; Green, Clemente, and de Groot, 24 1958; Schreiner and Kling, 45 1953; Scoville and Milner, 46 1957). Historically, the limbic lobe, set as a ring of cortex at the medial margin of the hemisphere, has been described as a somatic integrating center concerned with the integration of somatic influxes

Clinical and electroencephalographic correlations with serum levels of diphenylhydanotin.

Abstract: In a previous study 2 a method was described for determining diphenylhydantoin (5,5-diphenylhydantoin; phenytoin; Dilantin) concentrations in the serum when the drug was given in therapeutic amounts. With this method were determined the rate of rise of diphenylhydantoin in the serum after oral and after intravenous administration, the hourly and daily fluctuations, the ratio of dosage to serum level, and the rate of fall of serum diphenylhydantoin after withdrawal of the drug. In the study presented in this report the correlation is described between the serum level of diphenylhydantoin and the anticonvulsant effect of the drug, the incidence of paroxysmal abnormalities in the electroencephalogram, and the occurrence of toxic side-effects. Patients and Methods Patients. —1. Twelve patients, 19 to 58 years of age—six men and six women, hospitalized in the neuromedical, neurosurgical, or psychiatric department of the University Hospital. They had not taken diphenylhydantoin before admission to the hospital and

Learning and Retention in Monkeys After Amygdala- Hippocampus Resection

Abstract: In 1899 Bechterew3demonstrated a case of bilateral softening of the gyrus uncinatus and cornu ammonis before members of the St. Petersburg Clinic and drew attention to the prominent memory disturbances. Over 50 years later, Glees and Griffith8described an instance of dementia with bilateral destruction of the hippocampus and hippocampal gyrus in which recent memory impairment was an early and prominent symptom. They concluded on the basis of the necropsy material that the integrity of the region of the hippocampus is necessary for the maintenance of recent memory. Since then, a series of clinical studies has provided corroborative evidence and has extended these findings. In 1954, Scoville35reported a grave loss of recent memory in one epileptic and one psychotic patient after bilateral surgical removal of the uncus, hippocampus, and hippocampal gyrus. Formal memory and intelligence testing of eight similar patients again indicated impairment in recent

Relation of peripheral nerve fiber size and sensation in man.

Abstract: Introduction Since the theory of specific nerve energies was proposed by Johannes Muller 1 in 1826, there has been controversy regarding the relationship of the peripheral nerve to the perception of sensation. Conduction of specific modalities of sensation by differentsized fibers has been proposed by Ranson and Billingsley, 2 Adrian et al., 3 and Heinbecker et al. 4 In the laboratory animal, precise selective control and reproducibility of the peripheral afferent input with objective measurement of the central response is possible electrophysiologically. There remains the problem of translating the data obtained from animal experiments in terms of subjective human responses, including both the perception of and the reaction to varying touch, temperature, and painful stimuli. It is with such translation that this study of human responses to electrographically monitored activation of selected portions of the peripheral nerve fiber sizespectrum is concerned. We sought to determine whether a relationship between fiber

Influence of Head Position upon Cerebral Circulation: Studies on Blood Flow in Cadavers

Abstract: Patients with clinical evidence of cerebral vascular insufficiency frequently relate that a particular head position may precipitate their symptoms. Usually, extension of the head, as in looking upward, or lateral rotation, as in looking over the shoulder, is mentioned. In a previous study1we found that many such patients tolerated compression of a carotid artery when their heads were face forward but quickly developed signs and symptoms of insufficiency when carotid compression was repeated after the head had been turned to one side. In the past, those2,3who have recognized this influence which head position can have upon response to carotid compression have usually attributed it to stimulation of a carotid sinus reflex mechanism or to local anatomical factors which permit more effective compression of the sinus when the head is rotated. In this paper we suggest that a more important factor may be change in blood flow

Early descriptions of aphasia.

Abstract: Introduction One of the features of the spirited and prolonged discussion that followed Paul Broca's famous pathological demonstrations before the Academie de Medecine of Paris in 1861 was the "exhumation," to use the caustic term of Desire Bernard, of ancient and long-forgotten descriptions of aphasia. Thus, Jules Falret, 1 in his analysis of the various clinical forms of aphasia, mentioned case reports by Johann Gesner (1770) and Alexander Crichton (1798) as being among the earliest in the field. Trousseau 2 went farther back, citing the anecdotes of the elder Pliny as evidence that aphasia was known as early as the first century A.D., and quoting the comments of Schenck von Grafenberg (1585) to the effect that he had observed patients who were unable to speak because of loss of memory. The discovery of "prehistoric" case reports and references dealing with aphasia continued through the latter decades of the century and

Occlusive vascular disease in the extracranial cerebral circulation.

Abstract: Although there have been many studies of the clinical aspects of occlusive vascular disease in the extracranial carotid and vertebral arteries, studies of the pathology of these arteries have been few and have usually been confined to the carotid system. The true incidence of disease in these arteries has not been established. Reasons for the lack of studies of the arteries supplying blood to the brain are twofold; namely, removal of the carotid and vertebral arteries has been considered technically difficult, and, until recently, both clinicians and pathologists have been unaware of the high incidence of atherosclerotic narrowing in these vessels. Review of the Literature The possible role of cervical carotid-artery disease in the causation of vascular lesions of the brain was suggested by Hunt1in 1914. He examined 20 patients with hemiplegia and found a diminished carotid pulse on the appropriate side in 4. He called attention to

Recurrent Cerebrovascular Episodes

Abstract: In 1951, the advocacy of treatment for recent vascular accidents by vasodilators or by stellate block in some clinics led us to examine closely the mechanism of "vasospasm," at that time commonly held to be the basis of transient hemiplegic episodes The increasing performance of arteriography had by then confirmed the hitherto unsuspected frequency of occlusion of one carotid, 1,2,10 and my co-workers and I, like others, were struck by the common association of such carotid occlusion with a history of recurrent episodes of transient hemiplegia, often over a period of months or years, to which attention was first drawn by Egas Moniz, Lima, and de Lacerda 38 in 1937 In a series of our own cases 5 we cited the common precipitation of a transient attack by a situation that lowered the level of systolic blood pressure or the cardiac output Loss of blood, gastrointestinal bleeding, syncope, and the use

Neurologic Aspects of Waldenström's Macroglobulinemia: Report of a Case

Abstract: Introduction In 1944 Waldenstrom described a clinical syndrome under the title "incipient myelomatosis"1which he subsequently renamed "macroglobulinemia."2S i n c e that time about 200 similar cases were reported principally in the foreign literature. A number of recent reviews3-6have established the following as the outstanding characteristics of the disease. 1. It is a chronic debilitating illness primarily affecting males between the ages of 50 and 80. The usual life expectancy after the first manifestation of symptoms is 2 to 10 years. 2. The onset is insidious, usually characterized by progressive weakness, lassitude, weight loss, and pallor. Edema may be present. 3. Hemorrhagic diathesis is present in at least two-thirds of the cases and is manifested primarily by epistaxis, gingival and retinal hemorrhages, cutaneous p u r p u r a (usually late), and to a lesser extent bleeding from other organs. 4. Painless enlargement

Pain, Itch, and Vibration

Abstract: This paper deals with the activity of a group of cells in the cat's spinal cord and with some sensory phenomena in man. These cells in the cat's cord receive afferents from the skin and respond to all stimuli to which the cat's skin is sensitive. The discovery of a system of cells on which many types of peripheral fibers converge should be interpreted with great caution. The behavior of a cat shows that it is capable of differentiating between various types of skin stimuli, including various degrees of pressure, temperature, and "itch-producing" stimuli. In addition to these differentiated responses, the animal shows reactions such as the flexor reflex and the startle response, in which the modality of the stimulus seems largely irrelevant and only the intensity and rate of increase of the stimulus are important. Therefore, one expects to find convergent central pathways in which cells respond to several

Limbic nuclei of thalamus and connections of limbic cortex.

Abstract: I. Organization of the Projections of the Anterior Group of Nuclei and of the Midline Nuclei of the Thalamus to the Anterior Cingulate Gyrus and Hippocampal Rudiment in the Monkey Introduction The views expressed in the literature on the cortical projection fields of the anterior nuclei, midline nuclei, and lateral dorsal nucleus of the thalamus are vague and often contradictory. LeGros Clark and Boggon5were apparently the first to point out that the anterior thalamic nuclei in the cat project to the whole length of cingulate gyrus. Walker26suggested that the anterior medial nucleus in the monkey projects to the inferior surface of the frontal lobe. Later he was inclined to the view that the anterior group of nuclei projects mainly to the posterior part of the cingulate gyrus and that the anterior part of the gyrus (area 24-LA in terminology used by us) "receives few if any fibers from the thalamus."27Bailey and co-workers,1using the neuronographic method of strychnine-evoked potentials also came to the conclusion that the anterior cingulate gyrus is athalamic. However, Mettler

Electrocortical Activation by Estrogens

Abstract: Introduction The effect of various steroid hormones upon the electrical activity of the mammalian brain has been the subject of many investigations in the past. Cortisone and corticotropin (adrenocorticotrophic hormone) have been reported to exert convulsive effect in animals1and humans,2,3although these steroids have recently been used with some success in the treatment of patients with hypsarhythmia.4,5Desoxycorticosterone has also been found to exert electrical and clinical anticonvulsant effect in humans6and to increase the seizure threshold in animals.1,7Other steroids with anticonvulsant properties in animals are progesterone and certain androgenic hormones.1,7 We have been unable to find previous reports dealing with the direct effect of estrogens on cerebral electrical activity, although several studies indirectly suggest that ovarian hormones augment convulsive discharge in man and animals. For example, ovariectomy has been reported to give relief of seizures in humans8-10and to prevent

Observations on human subjects living in a "slow rotation room" for periods of two days.

Abstract: This report describes a new stressful procedure and the constellation of symptoms manifested by persons subjected to this stress. The procedure consists essentially of carrying out activities in a slowly rotating room. The symptoms most nearly resemble those observed in motion sickness, 4,15,22 but certain similarities are also seen when comparison is made with the symptomatology in vasodepressor states, 5,21 in anxiety and depressive states, in sleep, and, indeed, in various disturbances characterized by psychosomatic symptoms. 20 Inasmuch as the symptoms are the direct or indirect result of stimulation of the semicircular canals, the most precise term covering the general symptomatology is probably "canal sickness." 8 Although the procedure is specifically applicable to studies of the function of the semicircular canals, it has additional usefulness as a means of investigating certain aspects of the brain stem-activating system. 3,6,10,11,14,17 The extraordinary flexibility of the procedure in terms of both the strength

A correlation of autonomic and EEG components in temporal lobe epilepsy.

Abstract: Introduction In recent years it has become increasingly apparent that seizures characterized by curious visceral and psychical states and automatism associated with a predominantly temporal localization of electroencephalographic abnormality form a large proportion of clinical seizure problems. This has been reported to be as high as 75%. 1 As more evidence has accumulated, the problems associated with a sound understanding of its mechanism have vastly expanded. The present study consists in a series of observations of patients subject to attacks of this sort. An attempt has been made in a number of pentylenetetrazol (Metrazol)-induced seizures to record graphically as many clinical, autonomic and electroencephalographic phenomena as possible which are interrelated by the same time base. By this means it was hoped to discover correlates that might be useful in understanding the mechanism of the ictal features of temporal lobe epilepsy. Methods The material selected for the present study consisted of

Metabolic and Electroencephalographic Effects of Hyperventilation: Experimental studies of brain oxygen and carbon dioxide tension, pH, EEG and blood flow during hyperventilation

Abstract: It has been known for over 25 years that in most young and healthy subjects, hyperventilation produces a feeling of lightheadedness, difficulty in concentration, and high voltage slow waves in the electroencephalogram ( EEG ) but until the present time, the pathogenesis of the EEG slowing remains unproven. 3 Lennox, Gibbs, and Gibbs 7 considered that cerebral vasoconstriction occurring during hyperventilation maintained homeostasis of carbon dioxide (CO 2 ) in the brain and that if cerebral PCO 2 levels became reduced, EEG slowing resulted. Davis and Wallace offered an alternative hypothesis that EEG slowing during hyperventilation was due to vasoconstriction with ischemic anoxia. 2 Concurrent recordings of alveolar CO 2 concentration and arterial oxygen saturation in man have shown that hypoxia in the absence of hypocapnia as well as hypocapnia in the absence of systemic hypoxia may produce EEG slowing. 11 On theoretical grounds, hyperventilation might produce EEG slowing either by ischemic hypoxia

Posterior Biparietal Ablations in the Monkey: Changes to Neurological and Behavioral Testing

Abstract: Introduction In an earlier investigation (Ettlinger and Wegener,31958) three monkeys underwent bilateral ablation of the posterior parietal region, and they were subsequently found to show a defect of reaching which might be interpreted as a degree of "disorientation." At the same time their abilities were unimpaired in tests of tactile discrimination of shape and length. Holmes7(1918) observed a disorder of reaching in patients with bilateral parietal lesions caused by gunshot wounds, and he called attention to the fact that Ferrier had described what appeared to be a similar defect following parietal lesions in the monkey. The present work was designed to examine the defect in reaching following bilateral posterior parietal ablations in the monkey in more detail, to investigate further the possible factors appearing to cause "disorientation," and to examine the animals neurologically and also with a more extensive range of quantitative tests. In the previous

An Arteriographic Study of Cerebrovascular Disease in Man: I. Stenosis and Occlusion of the Vertebral-Basilar Arterial System

Abstract: The frequent occurrence of neurological syndromes due to atherosclerotic stenosis and occlusion of the vertebral and basilar arterial system has become recognized only in the past decade, although cases of occlusion of the basilar artery proved at necropsy were first reported by Leyden in 1875 17 and by Marburg in 1911. 20 Most of the early cases were due to syphilitic arteritis. Kubik and Adams 16 and Biemond 2 gave excellent clinical and pathological descriptions of thrombosis due to atherosclerosis of the basilar artery. These authors agreed that the diagnosis could be made during life from the clinical signs but indicated that the condition was rare. During the past five years there have been numerous papers dealing with the symptomatology and medical treatment of insufficiency of the vertebral-basilar arterial system. 6-18,21,22,25-29 Rather than considering atherosclerosis of the basilar artery as rare, we are now of the opinion that the condition

Intracranial bleeding in hemophilia.

Abstract: Three major areas of the nervous system may be involved by bleeding in hemophiliac patients. 1-11 1. Bleeding may compress a peripheral nerve, such as the facial, ulnar, radial, median, sciatic, or perineal, or the cutaneous nerves of the thigh. As a result of the frequency of hemorrhage into the iliopsoas muscles in hemophiliacs, the femoral nerve is most commonly compressed. 2. There may be involvement of the spinal cord, either as hemorrhage within the cord (hematomyelia) or compression from without (spinal sub- or epidural hemorrhage). 3. The third major site is within the cranial cavity, and bleeding here is probably considered the most dreaded complication of the disease, by patient and physician alike. The purposes of this communication are to review the reported occurrences of this last complication of hemophilia, to report 11 new cases, and to discuss the diagnosis and management of intracranial hemorrhage in hemophiliac patients, in

A humoral agent implicated in vascular headache of the migraine type.

Abstract: During the painful phase of attacks of vascular headache of the migraine type there is dilatation of the large and small blood vessels of the head both intra- and extracranially, often more on one side than the other. 1-3 In most attacks, however, the pain stems chiefly from the large subsurface cranial arteries and their branches. Aching pain is experienced when these vessels are distended, pulled upon, or displaced. Yet dilatation of these vessels, for instance that induced by immersion of the body in hot water, is not usually painful and does not induce other focal features of the migraine attack, i.e., edema, tenderness on pressure, and heightened vulnerability of tissue to injury. These observations led to the hypothesis that in addition to vasodilatation, a local sterile inflammation occurs. Also a substance (or substances) accumulates in the walls of the arteries and in the adjacent perivascular, areolar, and supporting tissues

Clinical Aspects of Stereotactic Thalamotomy in the Human: Part I. The Treatment of Chronic Severe Pain

Abstract: Introduction Our group has taken the ventral posterior thalamus as an initial target for stereotactic surgical intervention in the treatment of chronic severe pain. We have done this for several reasons. Clinically, we were often faced with the problem of treating terminal cancer patients with subjective head and neck pain in areas supplied by several cranial and upper cervical nerves. These patients, if not relieved by narcotic medication, often required a section of multiple posterior roots. We felt that any less drastic operative procedure, such as a stereotactic surgical maneuver which could effectively relieve chronic pain, would be a welcome addition to the surgical armamentarium. From a research point of view, the sensory relay nucleus of the thalamus offered us a unique opportunity to check the accuracy of our stereotactic method. We were able to stimulate peripherally and record evoked thalamic potentials; we also stimulated with intrathalamic electrodes in the

Role of encephalitis in pathogenesis of epilepsy.

Abstract: Introduction Seizures are one of the frequent sequelae of healed virus encephalitis. When focal, such seizures have been amenable to surgical therapy, and histological study of the excised areas of epileptogenic brain tissue has ordinarily shown only nonspecific gliosis and atrophy of gray and white matter, with no evidence of continuing inflammatory activity. The unsuspected finding of an apparently active encephalitic process in specimens removed from three patients with no clinical history of encephalitis has raised the question of the possible occurrence of chronic encephalitis which is subclinical except for recurring seizures. 1 Methods In order to assess the possible frequency of such a process as a cause of focal cerebral seizures, it seemed worthwhile to review a sizable segment of the series of pathological specimens removed at the Montreal Neurological Institute in the treatment of focal seizures. The microscopic material from a consecutive series of 512 surgical specimens from

Myoclonus: Clinical Significance and an Approach to Classification

Abstract: Myoclonus, colloquially known as "the jerks," has remained an enigmatic clinical entity for more than 70 years, despite the frequent use of this term by neurologists. During this time, little emphasis has been placed on this symptom with regard to its clinical implications and prognostic signifiicance. It is the purpose of this paper to review the records of patients exhibiting myoclonus, exclusive of patients with the rhythmic type involving the velum palatinum and the pharynx, and to report on follow-up studies on these patients. The pertinent literature will be reviewed, and the clinical and prognostic significance of myoclonus will be elaborated. Definition Myoclonus may be defined as an involuntary, repetitive, desultory, instantaneous, irregular contraction of a group of muscles or, occasionally, a single muscle. Such contractions must be differentiated from the fasciculations seen clinically and in electromyographic studies. It may be difficult to recognize this clinical symptom, and the diagnosis

Functional alteration of deep structures in cats with chronic focal cortical irritative lesions.

Abstract: Introduction The significance of independent paroxysmal abnormalities 2,17,24,45 or multiple spike foci 27,46 in deep cerebral structures of some epileptics has not been clearly understood. The latter finding and relatively low "cure" percentage in his own series of surgical epileptic cases led Meyers to challenge seriously the conceptual basis of current day epilepsy surgery. 28 Although the Montreal school 32 reported a higher percentage of satisfactory results in a larger and carefully selected focal epilepsy population, the importance of further careful analysis of failure cases in surgery for epilepsy can not be overemphasized. Possible reasons for surgical failure may be (1) incomplete excision of the cortical focus or (2) location of the original focus in the depth rather than on the surface. Such possibilities have received substantial support from both clinical and experimental studies. A further possibility is that an independent secondary deep abnormality might be produced by long-standing bombardment

Hemiballism Without Involvement of Luys' Body

Abstract: Introduction The occurrence in adults of abnormal involuntary movements of the limbs on one side has been recorded for a long time. Some of the movements have been like those encountered in the choreas of children and have been called "hemichorea." Others have been ceaseless and violent, have involved the bigger limb joints, and have produced wider movements. These have been called "hemiballism." There has been a tendency to think of the adult hemichoreas and hemiballism as manifestions of the same process, the latter possibly being considered only a severer manifestation of the former. 1 Other workers are chary of such a similarity and prefer to think of hemichorea and hemiballism as separate disorders, with probably different etiologies and dissimilar locales of the causative lesions. 2 Most observers have concurred in the idea expressed by Whittier 3 : "Hemiballism is the apparently inevitable symptom in man of destruction localized in the

Fusimotor nerve function in man. Differential nerve block studies in normal subjects and in spasticity and rigidity.

Abstract: Early clinical and physiological speculations about a special motor system for mammalian muscle tone appeared to be disproved by the correlation of electrical (motor unit) activity with muscle contraction, and conversely, of electrical silence with the resting state.1,2However, evidence accumulated in the last decade indicates that such a system does, indeed, exist, although not in the sense of a mechanism that produces tonic muscular contraction directly. The efferent innervation of muscle spindle tension receptors by a special group of ventral spinal root fibers was first suggested by the finding that stimulation of the small (gamma) ventral root fibers (comprising about 25% of the total in the cat) produced no further muscle shortening beyond that produced by stimulation of the large (alpha) motor fibers.3Leksell4first directly confirmed this function of the gamma efferent (fusimotor) fibers. The sensitivity of muscle spindle stretch receptors is modulated by the

The Proximal Anterior Cerebral Artery: An Anatomic Study

Abstract: Clinically, the majority of congenitally small or occluded anterior cerebral arteries involve the first portion of this vessel from its junction with the internal carotid to the anterior communicating artery. The impetus for this paper was derived from the belief that the symptomatology of anterior cerebral artery occlusion was confined to that area supplied by the first portion of this vessel. 1 The gross anatomy of the first portion of the anterior cerebral artery has been adequately described by many authors, the earliest being Heubner, 2 Beevor, 3 and Ayer. 4 More recently Shellshear, 5 Critchley, 6 Alexander, 7 and Kaplan 8,9 have made contributions to the anatomical studies. In France, Lazorthes, 10 and Foix and Hillemand 11 have also described the general anatomy. The anterior cerebral artery in its first course gives off 8 to 12 small branches from its superior aspect which arise at right angles and perforate,

Neurogenic muscular atrophy simulating muscular dystrophy.

Abstract: Degenerative lower motor neuron disease accompanied by weakness affecting predominantly the proximal muscles of the extremities, a syndrome closely simulating muscular dystrophy, is apparently very rare. Although there have been occasional instances in the neurologic literature where such cases have been mentioned, but without the inclusion of detailed reports, hereditary proximal muscular atrophy was not established as a separate clinical entity until the investigations of Kugelberg and Welander 1 and of Wohlfart, Fex, and Eliasson 2 had been reported. The syndrome described by these authors included (1) onset of muscle weakness in childhood or adolescence; (2) atrophy, hyporeflexia, and weakness predominantly affecting the proximal muscles; (3) very slow progression; (4) hereditary transmission apparently as a non-sex-linked recessive; (5) confirmation of the neurogenic nature of the disorder by muscle biopsy and/or electromyography. Fasciculations were found to be present in many, but not in all, of the reported cases. The patients of