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Showing papers in "JAMA Neurology in 1970"


Journal ArticleDOI
TL;DR: The purpose of the present communication is to point out some of the problems involved in the classification of fibers and to add new information of value in the analysis of human biopsy material.
Abstract: STUDIES on normal and pathological striated muscle are increasingly clouded by inconsistencies in the definition of fiber types and lack of correlation between different systems of nomenclature. The purpose of the present communication is to point out some of the problems involved in the classification of fibers and to add new information of value in the analysis of human biopsy material. The histochemical reaction for myosin adenosine triphosphatase (ATPase) and the pH lability of this reaction is used to characterize the various types of fibers. Material and Methods Muscle was obtained by biopsy in man, rat, and rabbit. Gastrocnemius and soleus were investigated in the animals. The human biopsies were taken from the biceps. The methods used for histochemical analysis have been given elsewhere. 1 In summary, unfixed frozen material was sectioned at 10μ thickness in the cryostat and the following histochemical reactions were carried out: (1) reduced diphosphopyridine

2,132 citations


Journal ArticleDOI
TL;DR: This is the first atlas of the ultrastructure of the mature nervous system and it is a relatively complete collection of elegant micrographs with an accompanying text that explores the role that thin astrocytic processes may play in isolating receptive surfaces, thereby preventing axon terminals from influencing.
Abstract: This is the first atlas of the ultrastructure of the mature nervous system and it is a relatively complete collection of elegant micrographs with an accompanying text. The differing quantity and distribution of perikaryal organelles are compared in pyramidal, Purkinje, and granule cells, neurons from the dorsal root ganglion, and in anterior horn cells. Criteria are listed for the identification of dendrites and axons, as well as for the special characteristics of the axon hillock and node of Ranvier. Considerable space has been given to the illustration of the different types of synapses, especially to their presynaptic and postsynaptic organelles and synaptolemmal membranes. Evidence relating synaptic vesicles to transmitter substances and their possible function is discussed. Protoplasmic and fibrous astrocytes are demonstrated and their possible functions pointed out. Of particular interest is the role that thin astrocytic processes may play in isolating receptive surfaces, thereby preventing axon terminals from influencing

2,034 citations


Journal ArticleDOI
TL;DR: This report summarizes the current analytical techniques and conceptualizations, illustrated by examples from the present and previous investigations, and investigates whether there is selective or nonselective involvement of muscle fiber types in each human and animal condition studied.
Abstract: DURING the past few years, our studies of the histochemical changes of skeletal muscle in human neuromuscular diseases and experimental animal models have gradually led to the development of an analytical approach which places major emphasis upon determining whether there is selective or nonselective involvement of muscle fiber types in each human and animal condition studied. Such determinations provide a new approach to the pathogenesis and etiology of the neuromuscular disorders. This report summarizes our current analytical techniques and conceptualizations, illustrated by examples from our present and previous investigations. Background Data and Hypotheses.— Histochemistry of fresh frozen muscle biopsy specimens from humans and animals demonstrates different degrees of enzyme activity in different fibers within the same muscle. The distinct histochemical characteristics of a given muscle fiber (which is a single, long, multinucleated cell) are maintained throughout its length, making it possible to obtain histochemical profiles of the individual

259 citations


Journal ArticleDOI
TL;DR: The pH hypothesis is physiologically attractive, but evidence against it comes from a series of experiments in several laboratories in which prolonged passive hyperventilation of animals and man producing sustained, constant arterial hypocapnia is produced.
Abstract: IT IS WELL established that the arterial carbon dioxide tension (Pco2) is an important factor controlling cerebral vascular resistance (CVR) and cerebral blood flow (CBF) in healthy animals and man. An acute rise in Pco2causes a decrease in CVR which increases the CBF, and a fall in Pco2has the opposite effect. However, during sustained alteration of Pco2, the CBF and absolute carbon dioxide tension often fail to correlate closely, and much recent evidence summarized by Lassen1suggests that alterations of the pH of the brain's extracellular space mediate the cerebral vascular response to carbon dioxide and that brain interstitial fluid pH is the major regulator of CBF. The pH hypothesis is physiologically attractive, but evidence against it comes from a series of experiments in several laboratories in which prolonged passive hyperventilation of animals2-5and man6producing sustained, constant arterial hypocapnia

249 citations


Journal ArticleDOI
TL;DR: The postcentral hand area is identified by electrical stimulation, excised a small amount of tissue there, and later observed a mild disturbance of light touch, pressure, two-point discrimination, and position sense in the postcentral gyrus.
Abstract: ELECTRICAL stimulation of the cerebral cortex in conscious patients has shown the major cortical representation of somatic sensation to be in the postcentral gyrus, with a minor representation in the precentral gyrus. 1-4 On both sides of the rolandic fissure, the entire body form is represented in sequence, throat lying just above the fissure of Sylvius and toes at the midline. There is some debate, however, concerning the role of this central region in somatosensory discrimination, and the extent to which such discriminatory functions are focally represented in man's cortex. Early clinical reports described patients with small unilateral cortical excisions who showed partial interference with sensory function in the corresponding body parts. Thus, van Valkenburg 5 identified the postcentral hand area by electrical stimulation, excised a small amount of tissue there, and later observed a mild disturbance of light touch, pressure, two-point discrimination, and position sense in the

216 citations


Journal ArticleDOI
TL;DR: There are two different conditions to which the term akinetic mutism or vigilant coma are properly applied, in which the patient lies in bed, immobile yet seemingly alert, ready to be aroused, and to follow with his gaze whatever events happen in the visual fields.
Abstract: THERE are two different conditions to which the term akinetic mutism or vigilant coma are properly applied. In one form of akinetic mutism the patient lies in bed, immobile yet seemingly alert, ready to be aroused, and to follow with his gaze whatever events happen in the visual fields; there is no evidence of oculomotor paralysis. This state of inertia can be interrupted in such patients by brief bouts of excitement, restlessness, and motor agitation. If stimulated enough, they may react with a word or two. This is a state best described as "hyperpathic" akinetic mutism or, if the term "coma" is to be used, this is a state of "vigilant" coma. In the other form, the patient is apathetic and somnolent most of the time. When he opens his eyes, he may keep them open if stimulation is sustained by calling the patient by name, pinching him, etc,

197 citations


Journal ArticleDOI
TL;DR: F fluorouracil, like other agents which block DNA synthesis, is toxic chiefly to rapidly dividing normal cells, ie, epithelial cells of the alimentary tract and hematopoietic elements of bone marrow and lymphoid tissues.
Abstract: THE PYRIMIDINE analog fluorouracil has been used extensively in the chemotherapy of malignant neoplasms since its introduction by Heidelberger and associates in 1957. 1 The carcinostatic property of fluorouracil seems to be due to anabolic reactions. Although fluorouracil is readily converted to acid-soluble fluorouracil nucleotides, undergoes incorporation into RNA, and interferes with RNA synthesis in mammalian tissues and tumor cells, 2 its major chemotherapeutic and toxic effects are attributable to an interference with DNA synthesis and cell division. The latter effect is due to inhibition of thymidylate synthetase, the enzyme which catalyzes the conversion of deoxyuridylate of thymidylate, by the fluorouracil derivative fluorodeoxyuridylate (FUDR). 2 Thus fluorouracil, like other agents which block DNA synthesis, is toxic chiefly to rapidly dividing normal cells, ie, epithelial cells of the alimentary tract and hematopoietic elements of bone marrow and lymphoid tissues. 3-5 Recently, Riehl and Brown 6 described an acute neurological disorder

171 citations


Journal ArticleDOI
TL;DR: 16 cases of psychiatric disturbances which occurred among 45 patients treated with levodopa are reported, which are reported to be the most effective agent presently available for the treatment of parkinsonism.
Abstract: IN RECENT years levodopa has proved to be the most effective agent presently available for the treatment of parkinsonism. 1-6 Among the side effects of levodopa therapy, psychiatric disturbances have been noted. 3-6 Levodopa is not only the precursor of dopamine hydrochloride but also of norepinephrine. 7 Although the action of these two catecholamines upon the central nervous system is incompletely understood, evidence has been accumulated in the last two decades upon their action in behavior. 8,9 In this context we would like to report 16 cases of psychiatric disturbances which occurred among 45 patients treated with levodopa. Method Levodopa was administered to 45 patients (31 men and 14 women) with Parkinson's syndrome. Forty-one patients had primary parkinsonism or Parkinson's disease, three had postencephalitic parkinsonism, and one had arteriosclerotic parkinsonism. Of the patients willing to enter the program, only those with decompensated cardiovascular disease, renal disease, psychosis

170 citations


Journal ArticleDOI
TL;DR: It was noted that within hours of complete ventricular obstruction, a number of changes, developing in response to acute ventricular enlargement, are seen in the ependyma and surrounding brain that are severe and probably accompanied by a diffuse alteration in the permeability of the ventricular surface.
Abstract: IT HAS recently been shown1that following effective obstruction of the fourth ventricle and caudal aqueduct, hydrocephalus develops as an acute, rapidly progressive process. The initial rate of progression is precipitous, and within a few hours the hydrocephalic changes are advanced. Thereafter, the rate of ventricular enlargement slows abruptly and continues to decrease until a moderate, stable, and chronically progressive rate is established. Within hours of complete ventricular obstruction, a number of changes, developing in response to acute ventricular enlargement, are seen in the ependyma and surrounding brain.2The changes are severe and are probably accompanied by a diffuse alteration in the permeability of the ventricular surface.3It was noted that once these changes are well established, the rate of ventricular enlargement slows perceptibly. The current paper reports some further observations on the structural and permeability changes of the ventricular surface in acute obstructive hydrocephalus. The

169 citations


Journal ArticleDOI
TL;DR: The aim of this communication is to give an account of the clinical and histopathologic characteristics of the disease, to discuss it in relation to allied conditions, and to entertain a possible pathogenetic mechanism.
Abstract: ACONGENITAL neurocutaneous disorder with the distinguishing histopathologic features of dysgenesis and neoplasia of the adipose tissue is presented in a 51/2-year-old epileptic, mentally retarded boy. To our knowledge, no such form of neurocutaneous lipomatosis has been reported. Thus the aim of this communication is threefold: (1) to give an account of the clinical and histopathologic characteristics of the disease; (2) to discuss it in relation to allied conditions; and (3) to entertain a possible pathogenetic mechanism. Report of a Case History.— A white boy, second in order of birth, was born on Aug 11, 1963. Hereditary and family history revealed no abnormalities. The mother's health during pregnancy was good, and the labor was uneventful. The birth weight was 2,779 gm (6 lb 2 ounces). He cried spontaneously. Abnormality of the right side of head, face, and right eye was noted at birth. Roentgenogram of the skull showed a slight

165 citations


Journal ArticleDOI
TL;DR: HEDT-RASMUSSEN and Skinhoj 1,2 were the first to report bilateral reduction of hemispheric blood flow (HBF) in patients with unilateral cerebral infarction.
Abstract: HOEDT-RASMUSSEN and Skinhoj 1,2 were the first to report bilateral reduction of hemispheric blood flow (HBF) in patients with unilateral cerebral infarction. They measured HBF by means of intracarotid injection of a bolus of radioactive inert gas and an uncollimated detector placed over each hemisphere and found the HBF to be reduced on the "healthy" side as well as in the diseased hemisphere. They did not measure cerebral metabolism but hypothesized that unilateral infarction reduced metabolism, and consequently, resulted in reduced blood flow in the contralateral hemisphere due to transneural depression. There were only six patients with unilateral cerebral infarction in their series, three of whom had thrombosis or embolic occlusion of the internal carotid artery. The smallness of their series was complicated further by the fact that one of the requirements of their method is that the bolus be injected into the internal carotid artery to avoid extracranial

Journal ArticleDOI
TL;DR: The neurologic problems occurring in systemic scleroderma as determined from the charts of patients with PSS at the Mount Sinai Hospital, New York are reviewed and discussed.
Abstract: PROGRESSIVE systemic sclerosis (PSS) or systemic scleroderma is a disease characterized pathologically by alterations in collagen tissue and clinically by diffuse induration of the skin. In the majority of cases there is evidence of dysfunction of visceral structures, particularly the gastrointestinal tract, kidneys, heart, and lungs. Vasomotor and endocrine disturbances may also be present. Unlike other collagen diseases, such as systemic lupus erythematosus,1thrombotic thrombocytopenic purpura,2and periarteritis nodosa,3the nervous system is rarely involved in PSS. Little has been written in the large literature on PSS of the neurologic manifestations which do occur in this disease. The purpose of this communication is to review and discuss the neurologic problems occurring in systemic scleroderma as determined from the charts of patients with PSS at the Mount Sinai Hospital, New York. Materials and Methods Between 1935 and 1966, 155 patients with the diagnosis of scleroderma were admitted

Journal ArticleDOI
TL;DR: The great majority of chronic convulsions may be arranged in two classes, which are usually called epileptic, and sometimes cases of "genuine" or "idiopathic" epilepsy.
Abstract: A convulsion is but a symptom, and implies only that there is an occasional, an excessive, and a disorderly discharge of nerve tissue on muscles. This discharge occurs in all degrees; it occurs with all sorts of conditions of ill health, at all ages, and under innumerable circumstances. But in this article I shall narrow my task to the description of one class of chronic convulsive seizures. The great majority of chronic convulsions may be arranged in two classes. 1. Those in which the spasm affects both sides of the body almost contemporaneously. In these cases there is either no warning, or a very general one, such as a sensation at or about the epigastrium, or an indescribable feeling in the head. These cases are usually called epileptic, and sometimes cases of "genuine" or "idiopathic" epilepsy. 2. Those in which the fit begins by deliberate spasm on one side of the body

Journal ArticleDOI
TL;DR: The effect of undernutrition on the development of cerebral cortex is better studied in suckling rats during the first three postnatal weeks when growth of cortex and cytoplasmic differentiation of neurons is maximal.
Abstract: STUDIES of malnutrition indicate the importance of the "sparing effect": of all organs of the body, the brain is least affected by starvation during growth.1-5This widely accepted but partially correct conclusion is based on observations of rats weaned at 21 postnatal days and then undernourished. In 1917 and 1918, Sugita6,7emphasized that the normal growth of the cerebral cortex of the rat is precocious in relation to the body; the cortex reaches adult width and develops six layers during the first three weeks of life, but the maximal increment of growth of the body occurs between three and seven weeks. Sugita therefore suggested that the effect of undernutrition on the development of cerebral cortex is better studied in suckling rats during the first three postnatal weeks when growth of cortex and cytoplasmic differentiation of neurons is maximal.8 Recent investigations9-11on growth of whole

Journal ArticleDOI
TL;DR: It subsequently became apparent that this side of the inferior cerebellar peduncle has been associated with palatal myoclonus or hypertrophic olivary degeneration, and a triangular relationship among the red nucleus and inferior olive on one side and the contralateral dentate nucleus was proposed.
Abstract: THE SIGNIFICANCE of a pathway directly linking the dentate nucleus of the cerebellum and the contralateral inferior olive has been revealed by clinicoanatomical study of the phenomenon of palatal myoclonus. 1-8 The lesion which has been most constantly demonstrated in cases of palatal myoclonus (as in rhythmic skeletal myoclonus) is a specific hypertrophic (presumably transynaptic) degeneration of the inferior olive. This hypertrophic degeneration, with very few exceptions, 8,9 is associated with a primary lesion in the ipsilateral central tegmental tract 1,10-12 or the contralateral dentate nucleus. 3,13-14 In the first attempt to explain these associations, Guillain and Mollaret 4 proposed a triangular relationship among the red nucleus and inferior olive on one side and the contralateral dentate nucleus. However, since no lesion of the olivodentate fibers within the inferior cerebellar peduncle has been associated with palatal myoclonus or hypertrophic olivary degeneration, it subsequently became apparent that this side of

Journal ArticleDOI
TL;DR: The present paper reviews 35 cases of rhinorrhea treated at the Columbia-Presbyterian Medical Center during the past 36 years, and the importance of spontaneous leakage through the middle fossa and sphenoid sinus is discussed.
Abstract: CEREBROSPINAL fluid (CSF) rhinorrhea is sometimes difficult to diagnose and treat. Trauma is the most common cause, and nontraumatic cases ("spontaneous") are considered rare. Intracranial tumors have been regarded 1 as a common cause for nontraumatic rhinorrhea and the cribriform plate area in the anterior fossa as the most likely site of CSF leakage. The present paper reviews 35 cases of rhinorrhea treated at the Columbia-Presbyterian Medical Center during the past 36 years. This series is unique because of the large number of cases of rhinorrhea unassociated with trauma or tumor (11). The importance of spontaneous leakage through the middle fossa and sphenoid sinus is discussed. Method The records of the Columbia-Presbyterian Medical Center were used to locate all patients with CSF rhinorrhea between 1932 and 1968. Thirty-five records were obtained. Results Trauma.— Nine cases were associated with a definite history of trauma (Table 1). In four the rhinorrhea

Journal ArticleDOI
TL;DR: The changes in sleep pattern when normal young adults were subjected to an acute inversion of sleep-waking cycles in man are described.
Abstract: PAST studies of the sleep-waking circadian cycle in man emphasized the temporal relationship to other rhythmic physiological and chemical functions. 1 It was implicitly assumed in these studies that sleep is a unitary process. Recent evidence, however, clearly indicates that sleep is composed of recurring short-term physiologic events. 2,3 In man, during each daily sleep period, a consistent sequence of sleep patterns occurs, characterized by four or five recurrent 90-minute cycles. Future studies of circadian phase relationships between sleep and physiologic variables should take into account the qualitative and quantitative differences between sleep stage patterns. We have begun to study the time relation of sleep stages and neuroendocrine processes. 4 The method of cycle phase shift of 180° (sleep-waking cycle inversion) has been used in man. 5-8 This report describes the changes in sleep pattern when normal young adults were subjected to an acute inversion of sleep-waking cycles in

Journal ArticleDOI
TL;DR: The muscular stiffness and continual EMG activity were abolished by curare but persisted during spinal anesthesia and after peripheral nerve blocks, suggesting that the syndrome was due to isolated, spontaneous, peripheral nerve hyperactivity.
Abstract: THE PERIPHERAL nerve disorders, unlike central nervous system and muscle diseases, have not been widely recognized as causes of generalized muscular stiffness. Isaacs,1,2in 1961 and 1967, reported three patients with an entity of generalized muscular stiffness, fasciculations, continual electromyographic (EMG) activity at rest and depressed deep tendon reflexes. The muscular stiffness and continual EMG activity were abolished by curare but persisted during spinal anesthesia and after peripheral nerve blocks, suggesting that the syndrome was due to isolated, spontaneous, peripheral nerve hyperactivity. Isaacs also discovered that diphenylhydantoin (DPH) induced a substantial and sustained decrease in the muscular stiffness. Similar cases were reported by Mertens and Zschocke3and by Levy et al,4both groups confirming the effects of spinal anesthesia and curare. Mertens and Zschocke3also found carbamazepine as effective as DPH in treatment. Sig wald et al,5Gardner-Medwin and Walton,6and Hughes and

Journal ArticleDOI
TL;DR: The vasomotor phenomenon described by Cushing in 1902 has become known as the Cushing "reflex" and the reflex nature of the response has never been clearly established, however, in this paper it will be referred to as theCushing response.
Abstract: THAT an acute elevation of intracranial pressure results in a rise of the systemic blood pressure and a fall of heart rate has been known for many years 1-3 In 1902, Cushing 4,5 first demonstrated the quantitative nature of this response, showing that it was graded and occurred when the pressure within the head exceeded that of the systolic blood pressure Furthermore, the response he observed was reversibly abolished by instilling cocaine into the cisterna magna Cushing speculated that ischemia of the brain stem was the stimulus for the response and that it was in some manner mediated by the caudal brain stem Since his report, the vasomotor phenomenon he described has become known as the Cushing "reflex" Because the reflex nature of the response has never been clearly established, however, in this paper it will be referred to as the Cushing response It was never proved by Cushing, nor

Journal ArticleDOI
TL;DR: The purpose of this report is to present the clinical, morphological, and biochemical studies of the two patients in whom unique defects were found, in the hope that these studies will contribute to the further understanding of the pathogenesis of similar neurological disorders.
Abstract: INVESTIGATION of an unusual hereditary disease of the nervous system and muscle revealed a marked reduction in the demonstrable cytochrome b content of the muscle mitochondria and other abnormalities of the cytochrome system. The purpose of this report is to present the clinical, morphological, and biochemical studies of the two patients in whom these unique defects were found, in the hope that these studies will contribute to the further understanding of the pathogenesis of similar neurological disorders. Report of Cases CASE 1.— This 46-year-old man was in excellent health until age 33, when his gait became unsteady and his speech became slurred. Shortly thereafter, movements of his hands became clumsy and his handwriting deteriorated, and, within two years, he experienced weakness of the muscles of his legs and shoulders. All of these symptoms were insidiously progressive, but cramplike muscle pains, which persisted for approximately six years after the onset of

Journal ArticleDOI
TL;DR: The author's experience in treating cases of multiple sclerosis with a low fat diet for more than 20 years is assessed, both in treatment of the data and in conclusions.
Abstract: THIS paper assesses the author's experience in treating cases of multiple sclerosis with a low fat diet for more than 20 years. Previous reports of these studies 1-5 were preliminary in intent. The present paper is intended to be final, both in treatment of the data and in conclusions. Materials and Methods Patient Material.— Evaluation and discussion of the materials and methods were presented in detail in a previous paper. 4 The more pertinent points, however, will be included here. From December 1948 to April 1954, 264 patients with multiple sclerosis were examined at the Montreal Neurological Institute; 108 were seen only a few times. The remaining 156 patients maintained contact to and beyond April 1954, and no patients were added after this date. Two were rejected because of uncertain diagnosis, and eight were lost during the ensuing years. The remaining 146 patients are included in this report of

Journal ArticleDOI
TL;DR: The use of electrical stimulation of the supraorbital nerve evokes two separate contractile responses in the orbicularis oculi muscle which are almost identical to the response to glabellar tap.
Abstract: THE RESPONSE of the orbicularis oculi muscle to a tap over the brow has two separate components: an early ipsilateral reflex and a late bilateral reflex. 1 Electrical stimulation of the supraorbital nerve also evokes two separate contractile responses in the orbicularis oculi muscle (Fig 1) which are almost identical to the response to glabellar tap. 2,3 The use of electrical stimulation has made it possible to apply this method in the ordinary electromyographic laboratory, and normal values and variations of this reflex have been well established. 4,5 In recent years, alteration of the reflex in various diseases of the nervous system has been recognized. 2-6 The reflex arc may be affected by lesions of the trigeminal nerve (afferent path), the facial nerve (efferent path), or of the pons (central connections). Practical application of this technique to the study of peripheral facial nerve paralysis 5 and of trigeminal nerve

Journal ArticleDOI
TL;DR: Observations are made on the tolerance of the cerebral circulation to a reducion in the forward blood flow through the extracranial vertebral arteries bilaterally in cases studied arteriographically during life.
Abstract: IN A clinicopathologic study of 178 cases of atherosclerosis of the vertebral and carotid arteries, 1 infarction in the vertebral-basilar territory was related to occlusive disease situated intracranially . This was in contrast to the findings in infarction in the territory of the internal carotid artery in which the occlusions lay predominantly extracranially . The number of cases of infarction was small, and generalizations were unwarranted, but we were prompted to make further observations on the tolerance of the cerebral circulation to a reducion in the forward blood flow through the extracranial vertebral arteries bilaterally . Since postmortem examination fails to provide information about hemodynamics, attention has been focused on cases studied arteriographically during life, with particular regard to the pathway by which the intracranial vertebral-basilar system is supplied. Bilateral extracranial vertebral compromise results from occlusion or stenosis of both vertebral arteries, or of one vertebral artery when the other is vestigial

Journal ArticleDOI
TL;DR: The clinical manifestations of the disease are thought to be due either to direct involvement of various tissues by lipid deposits or to arise secondarily to vascular disease from involvement of small arteries and arterioles.
Abstract: ANGIOKERATOMA corporis diffusum or Fabry's disease is a genetically determined disorder of lipid metabolism which displays a sex-linked recessive transmission. In hemizygous male subjects, it produces the characteristic skin lesions, dilated conjunctival blood vessels, and corneal opacities, together with renal failure, and cerebral and cardiac complications. In the heterozygous female subjects, abnormalities are usually limited to the eyes, although the abnormal lipid deposits have been dected in other organs. The abnormality responsible for the disorder has been shown by Brady et al 1 to be a deficiency of ceramide trihexosidase, which results in the accumulation of ceramide trihexoside in various tissues. 2,3 The clinical manifestations of the disease 4,5 are thought to be due either to direct involvement of various tissues by lipid deposits or to arise secondarily to vascular disease from involvement of small arteries and arterioles. Frequently, an organ such as the heart, kidney, or brain may be

Journal ArticleDOI
TL;DR: The earliest histological changes seen in minimally weak, or even clinically normal, muscles of patients with Duchenne muscular dystrophy are small foci of grouped muscle fibers undergoing necrosis or regeneration, all fibers of the group being in about the middle.
Abstract: THE ETIOLOGY and pathogenesis of childhood x-linked pseudohypertrophic muscular dystrophy ("Duchenne dystrophy") remains obscure, even though the histopathology and clinical picture were described by Duchenne 1 in 1868, and Erb 2 formalized the concept of a primary dystrophic disease of muscle in 1891. Meryon 3 in 1864 suggested that it may be "... an idiopathic disease of the muscle, dependent perhaps on defective nutrition." Recent emphasis has centered on multiple possibilities, including a metabolic defect intrinsic to the muscle fiber or muscle-fiber membrane, 4-7 a functional vascular abnormality, 8-11 an autoimmune process, 12 an abnormal circulating serum factor, 13 an endocrine abnormality, 10,14,15 and a primary defect involving connectivetissue proliferation. 16 The earliest histological changes seen in minimally weak, or even clinically normal, muscles of patients with Duchenne muscular dystrophy are small foci of grouped muscle fibers undergoing necrosis or regeneration, all fibers of the group being in about the

Journal ArticleDOI
TL;DR: In this article, histochemical studies of affected muscle in two patients with Cushing's syndrome are described and indicate that the grossly visible wasting can be accounted for by a diminution in diameter of practically all muscle fibers, with atrophy being more prominent in the type II (high in myofibrillar ATPase, high in most mitochondrial oxidative enzymes) fibers.
Abstract: CUSHING'S syndrome or therapy with any of the glucocorticoids may cause proximal muscular weakness.1,2Muscle wasting is usually apparent clinically. Occasional fibers undergoing degeneration and scattered atrophic fibers have been described in muscle biopsy specimens.1,3,4In this paper, histochemical studies of affected muscle in two patients with Cushing's syndrome are described and indicate that the grossly visible wasting can be accounted for by a diminution in diameter of practically all muscle fibers, with atrophy being more prominent in the type II (high in myofibrillar adenosine triphosphatase [ATPase] and phosphorylase) than in the type I (low in myofibrillar ATPase, high in most mitochondrial oxidative enzymes) fibers.5There was no evidence of a myopathic process in the usual sense of necrosis, phagocytosis, cellular infiltrates, or endomysial connective tissue proliferation. The potassium content of muscle was diminished. Report of Cases CASE1.—This 67-year-old woman was confined to bed during

Journal ArticleDOI
TL;DR: That destruction of Broca's area causes aphasia appears to be established beyond doubt through such cases as the striking one of Simon, which actually resembled an experiment, but other conscientious and experienced observers are also correct in insisting that Broca’s area is not the only speech center, and that circumscribed lesions in the region of the Sylvian fissure can produce aphasIA.
Abstract: .... That destruction of Broca's area causes aphasia appears to be established beyond doubt through such cases as the striking one of Simon, which actually resembled an experiment. However, other conscientious and experienced observers are also correct in insisting that Broca's area is not the only speech center, and that circumscribed lesions in the region of the Sylvian fissure can produce aphasia. We may now ask what lies near the Sylvian fissure, and we note a gyrus on the convex surface of the cerebrum, running in a curve directed posteriorly and superiorly, almost enclosing the Sylvian fissure. From the central sulcus it runs anteriorly in a distinct longitudinal tract, the ... first frontal gyrus. Its posterior peduncle is in the first temporal gyrus, just clearly discernible as the longitudinal tract. That the whole is to be considered as one gyrus is clear from comparison with the brains of animals, such as dogs.

Journal ArticleDOI
TL;DR: Anticoagulant therapy is not advisable for patients with cerebral venous thrombosis after neurologic signs have developed for fear of evoking cerebral hemmorhage.
Abstract: 2 cases are presented of dural sinus and cerebral venous thrombosis in obese but healthy women who were using oral contraceptives. The first was 35-year-old with 5 children and a history of eclampsia and hypertension. She had used Enovid (norethynodrel and mestranol) intermittently for 2 years. She first noted right sided headache then gradually nausea vomiting diarrhea seizures weakness urinary incontinence. On hospitalization 5 days later an echoencephalogram showed shift of midline structures to the left a brain scan showed increased uptake on the right and an angiogram suggested an avascular mass. Craniotomy revealed an intracerbral hematoma and cortical vein thrombosis. She died 1 week later. The autopsy demonstrated thrombi in the superior sagittal sinus and a right frontal hematoma suggesting that the cortical vein thrombi propagated back leading to stasis infarction and hemorrhage. The second case was a 27-year-old mother of 3 who had been taking Ortho-Novum (norethindrone and mestranol) for 12 days. She had 2 days of severe headache then loss of dexterity and visual acuity. No venous pulsation could be seen in the optic fundi. Angiogram showed lack of filling of cortical veins. Spinal fluid had an opening pressure of 400 mm 320 red cells /ml and 80 mg protein/ml. On the fifth day she had Jacksonina seizures and on the eleventh day maximal shift of midline structures 8 cm to the left was measured. Except for thrombophlebitis of right leg and pulmonary embolism she gradually improved. Anticoagulant therapy is not advisable for patients with cerebral venous thrombosis after neurologic signs have developed for fear of evoking cerebral hemmorhage.

Journal ArticleDOI
TL;DR: The present report describes the case of a young woman in whom a left atrial myxoma with disseminated emboli gave rise to pathologic changes in cerebral vessels unlike any that the authors had previously seen or that had been described by others.
Abstract: THERE are now over 350 reported cases of cardiac myxoma, a slowly-growing intracavitary tumor, most frequently occurring in the left atrium. 1,2 Cardiac myxomas manifest themselves clinically by obstruction of cardiac flow, by constitutional effects, and by embolization. Systemic emboli have been reported in up to 45% of patients with left atrial myxoma. 3-5 In cases where inconspicuous cardiac symptoms and signs are combined with multiple cerebral emboli, a difficult diagnostic problem confronts the clinician. Maroon and Campbell 6 have pointed out the importance of atrial myxoma as one of the potentially treatable causes of cerebrovascular occlusion. The present report describes the case of a young woman in whom a left atrial myxoma with disseminated emboli gave rise to pathologic changes in cerebral vessels unlike any that we had previously seen or that had been described by others. Because of the puzzling clinical course, the striking abnormalities visualized by

Journal ArticleDOI
TL;DR: A study was undertaken with the hope of clarifying the relationship between hydantoin therapy and malignant lymphoma.
Abstract: LYMPHADENOPATHY has been recognized as one of the less common side effects of the hydantoin drugs 1-3 for more than a quarter of a century. These drugs have been available for the treatment of epilepsy since 1938. Lymphadenopathy in these patients had been considered a hypersensitivity reaction because of the frequent occurrence of eosinophilia, and benign since it disappeared with discontinuation of the hydantoin. There is a dearth of knowledge about the mechanism of hydantoin lymphadenopathy. The benignancy of this lymphadenopathy has been questioned. 4-6 Great difficulty has been encountered in the differentiation between "benign" and malignant lymphoid reactions in general. 4 Hyman and Sommers 7 reported six cases of lymphoma in association with hydantoin therapy. A study was undertaken with the hope of clarifying the relationship between hydantoin therapy and malignant lymphoma. A review of the literature was processed, and then a retrospective study of necropsies undertaken to