Showing papers in "Neurology in 1966"

Acetylcholine released from cerebral cortex in relation to state of activation

Abstract: THERE IS a growing amount of experimental evidence to support the view that certain forms of activation of the cerebral cortex may be mediated by acetylcholine ( ACh) . It has long been known that desynchronization of the electrical activity of the cortex comparable to that seen in the transition from the state of sleep to arousal or alertness can be simulated by ACh or by agents which antagonize its hydrolysis such as neostigmine bromide (Prostigmin@) . This forni of activation or “arousal” is blocked by atropine.1-5 Activation to epileptiform discharge by ACh has also been demonstrated by numerous investigators.6-9 More direct evidence has been obtained by measurements of the “bound or “stored” concentration of ACh in cerebral tissue during different states of activation, as in the studies of Richter and Crosslandlo who found an increase in the tissue ACh, that is, the “bound form, during anesthesia as compared to the state of wakefulness and a marked decrease during convulsive seizures. These results were confirmed by Pepeu and Mantegazzinill in experiments involving a hemisection of the midbrain. These authors found that the hemisphere showing electrical activity characteristic of sleep contained more bound ACh than the opposite hemisphere which showed an arousal pattern in the electroencephalogram (EEG), thereby concluding that more ACh was being released in the “waking” half of the brain. Direct measurements of the rate of release of ACh from the surface of the cerebral cortex have also been shown to vary with its degree of activation by the use of a profusion chamber placed in the skull over the cortex in which could be placed an “eserinized” physiological salt solution.12-14 These studies have shown

A quantitative study of Meissner's corpuscles in man

Abstract: THE MEISSNER corpuscle is an oval, encapsulated nerve ending, approximately 30 by 80 p, that is located almost exclusively in the dermal papillae of nonhairy skin (Fig. 1). The structure and location of this nerve ending strongly suggest that it is a receptor of mechanical stimuli, much in the manner of hair follicle endings in hairy skin;I but, at present, knowledge is lacking concerning the threshold of sensitivity of an isolated Meissner corpuscle to various sensory stimuli. Thus, the specificity of this cutaneous receptor has yet to be elucidated.2 In the few previous attempts in which silver stains were used in quantitating the normal density (number per unit) area of Meissner corpuscles, results were not in good this may have been due partly to the small number of subjects studied. Staining by cholinesterase methods outlines Meissner’s corpuscles in a discrete fashion which allows them to be easily identified and counted (Fig. 2). Recently, Dickens and coworkers5 studied, in 41 patients with sensory loss and in 37 control subjects, the density of Meissner’s corpuscles as demonstrated by this technique. They found that patients with lesions of the central nervous system had a density of Meissner’s corpuscles similar to that of the control group, whereas those with peripheral neuritis appeared to have a decreased density. Therefore, this technique might be useful as another method of investigating and classifying the peripheral neuropathies but before doing this, it was decided to document more thoroughly the normal quantitative variation of Meissner’s corpuscles. This study is concerned with the normal density, distribution, size, and shape of Meissner’s corpuscles and with the variation in these characteristics with age, sex, and occupation of the subject and with the size of the digit.

Myasthenia gravis: Evaluation of treatment in 1,355 patients

Abstract: The Myasthenia Gravis Clinics of Massachusetts General Hospital and The Mount Sinai Hospital were established in 1935 and 1951, respectively. Patients have been seen regularly at each clinic to the present time, and standardized methods of diagnosis, treatment, and follow-up have been carried out over the years. Details concerning diagnosis and treatment are available in previous publications from each clinic.l.2 Thymectomy, in selected cases, has been employed at Massachusetts General Hospital since 1941 and at The Mount Sinai Hospital since 1951. Eight cases were lost to follow-up in Boston before 1942, and 21 cases were lost to follow-up in New York before 1952. Therefore, surgical therapy was available to all but 29 of the total number of patients. Patients in each clinic were surveyed to determine eligibility for inclusion in the study. Adequate information concerning the initial diagnostic evaluation and at least one followup visit were the criteria for inclusion. Pertinent data concerning each patient from the onset of disease to the most recent evaluation or death were processed separately by each clinic, and the information was coded in a uniform manner on IBM cards. The information included sex, race, age at onset, type and

Facial recognition in brain-damaged patients. An experimental approach.

Abstract: THE FIELD OF AGNOSIA recently has been broadened by a new form which, in the opinion of many authors, possesses an individual character and a certain significance for localization: agnosia for faces or prosopagnosia. Patients with this disorder complain that they are no longer able to recognize even very familiar faces, such as those of dose relatives, without recourse to noting significant features of the face, such as a birthmark, mustache, or scar; visual details not connected with the face, such as clothing; or nonvisual characteristics, such as tone of voice. Bodamerl was the first to distinguish between this form of agnosia and object agnosia, to which it previously had been ascribed. The cases which have been described since then, as well as the interpretation given to them, recently have been summarized by Gentili and associates2 and H6caen and Angelergues.3 The explanations of the nature of this symptom may be outlined as follows: 11 Prosopagnosia is a distinctive type of agnosia caused by the impairment of a specialized perceptual ability (the recognition of human faces) which starts developing separately during the first months after birth.4 21 Prosopagnosia is not an autonomous condition, just as the other specialized forms of agnosia are not autonomous conditions. All these errors in visual recognition may be related to impairment of visual perception, which is altered quantitatively as well as qualitatively in terms of either an abnormal “Funktionswandel”5,6 or a deficit of morphosynthesis.2 A majority of the authors who have examined prosopagnostic patients, however, are skeptical that a postulated defect in visual analyzers can suffice to explain the symptom. Furthermore, HCcaen and Angelergues3 note that, in contrast to object agnosia, prosopagnosia tends to be more frequent in patients with brain damage in the right hemisphere than in those suffering from left-hemisphere damage. This suggests the existence of specialized centers for this type of recognition and makes it difficult to ascribe the disorder to a simple alteration of the sensory function. On the other hand, the prominence of prosopagnosia in patients with right-hemisphere brain damage, although evident in the cases reported by Hhcaen and Angelergue~,~ cannot be accepted without reservation. Clinical observation of the symptom might be masked by concomitant aphasia in patients suffering from left-hemisphere damage. As one studies a number of the cases described in the literature, it becomes clear that many times prosopagnosia is discovered accidentally by the physician in the course of his interview with the patient without the latter mentioning it at all. Thus, the deficit could well be overlooked in the case of aphasic patients. 31 The basic defect in prosopagnosia is the incapacity to detect the individual character of objects or stimulus patterns within a single homogenous category from the standpoint of overall f ~ r m . ~ , ~ ~ It must be noted that these patients recognize a face to be a face and fre-

Surface‐negative, slow‐potential shift associated with conditioning in man

Abstract: IN THE STUDY of the cerebral electrical correlates of behavior, recent work has focused upon two electrographic phenomena: [l] changes in evoked potentials and [Z] the appearance of slowly changing or direct current (D.C.) potentials. Galambos and associates1 found during simple operant conditioning procedures in cats that auditory-evoked responses were larger and appeared more frequently and with wider distribution than at other times. It is significant to the subject of this paper that the greatest change in the evoked potential under these conditions was an increase in the slow, surfacenegative component. Gerken and Neff? have also described such a change during conditioning in animals. They suggest that learning per se is not the only cause of the changes in evoked responses seen under these conditions. Attention, changes in alertness, and emotional state are mentioned as perhaps adding to the specific learning effects. Other investigators\"-\" have demonstrated increases in the size of primary-evoked potentials during conditioning in animals, generally attributing the change to an increase in excitability level of the cortical or subcortical areas involved. Similar studies have been done in man. Peimeri and Katss conditioned subjects to make a specific motor response to a signaI. Evoked responses to the conditioned stimulus (CS) underwent complex sequences of change, but the major findings were an increase in size and a wider area of spread of these potentials. Peimer points out the particular increase in the negative phase of the response. Haider and co-worker9 showed that reduced attentiveness in human subjects is paralleled by corresponding reductions in amplitude of visual, cortical-evoked responses. The related question is dealt with by DavislO who has shown that the averaged, slow responses evoked by auditory stimuli and recorded from the vertex of the scalp can usually be enhanced if the listener is required to make a difficult auditory discrimination, thereby presumably increasing his attentiveness. In the records shown, the increase is in the slow, positive component of the evoked response. The eiTects of applied or externally induced steady potentials have been studied. Polarization of the cortex by direct current can result in changes in cortical excitability11.1~ and has an effect upon the performance of conditioned avoidance responses.lS Spreading cortical depression, which is accompanied by a D.C. change in the cortex, has been shown by Bures and BuresovQl.' to impair the establishment of conditioned learning. In a unique approach to the study of conditioning in man, W. Grey Walter and his associates1\"-\" combined D.C. recording and evoked potential techniques. The Bristol group was studying the interaction of evoked responses to paired stimuli as recorded from the frontal areas of the brain. They compared records from scalp electrodes with those from muItipIe implanted electrodes and found, as

Neuroepithelial (colloid) cysts of the nervous system: Further observations on pathogenesis, location, incidence, and histochemistry

Abstract: Tm. FIRST NEUHOEPITHELIAL (colloid) cyst of the third ventricle was reported by Wallmann’ in 1858. Sjovalls in 1910 suggested the paraphysis as the site of origin, a widely accepted concept resulting in the popular term “paraphyseal cyst.” The paraphysis lies at the rostra1 end of the diencephalic roof; hence, the belief arose that neuroepithelial cysts occurred only in the anterior part of the third ventricle. The cyst was regarded as rare.:’ A few author#-R including ourselvesQ.1” have presented evidence from developmental and comparative anatomy that the paraphysiq is an extraventricular choroid plexus. Both choroid plexus and ependyma are derived from a common neuroepithelium. We therefore proposed that neuroepithelial cysts occur anywhere along the neuroepithelial-lined course of the central nervous system from either choroid plexus or ependyma.9 Most cysts described a s “colloid” have an inner neuroepithelial lining. The more frequent cysts in the stroma of the choroid plexus are lined by connective tissue and have been thought to be the result of cystic degeneration associated with increased age. We will present evidence that neuroepithelial cysts are common, that they are encountered throughout the nervous system and at any age, and that the mechanism of formation is similar, whether the epithelium lies inside or outside the cyst. To extend observations made previously on mucicannine-reactive material in developing hiiman choroid plexuses.10 tests for this substance were also performed in mature choroid plexuses of man.

The nature of the narcoleptic sleep attack.

Abstract: THE SYNDROME of narcolepsy has been defined by 4 elements: [l] narcolepsy proper, overwhelming sleepiness; [ 21 cataplexy, episodes of muscular weakness usually induced by laughter or anger; [ 3 ] sleep paralysis, dttacks of inability to move developing in the transition between arousal and sleep; and [4] hypnagogic haliucinations, vivid visual and auditory sensations occurring at the onset of sleep. Only the presence of narcolepsy proper has been required to permit the diagnosis. In most cases, 1 or more of the other members of the tetrad are also present. The narcoleptic sleep attack frequently occurs after meals or during monotonous activity; hence, it is often said that the essential pathology of the illness is merely an exaggeration of normal tendencies toward somnolence. However, cataplexy and sleep paralysis as well as hypnagogic hallucinations are less readily explained on this basis. Even so, narcolepsy has been regarded as a sleep abnormality and it is assumed that knowledge pertaining to normal sleep mechanisms will aid in understanding the pathophysiology of the illness. Perhaps the most important recent addition to our knowledge of normal sleep mechanisms is the realization that sleep, long conceived of as an essentially unitary state, actually consists of 2 states which differ dramatically in their properties and have almost nothing in common except recumbency and outward quiescence. One of these states is the recently elucidated paradoxical phase of sleep, otherwise known as activated sleep or rapid eye movement (REM) sleep. The other, which occupies the greatest part of behavioral sleep, is the well-known electroencephalographically defined state characterized by the presence of slow waves and spindles known as nonrapid eye movement (NREM) sleep.

Restless legs syndrome in uremic neuropathy

Abstract: IN 1944, Ekboml published a monograph on the “restless legs syndrome.” Thomas Willis2 was the first to describe this in 1685, and since then descriptions have been given by Wittmaack,3 Beard,4 Oppenheimer,s Mussio Fournier,c Jolivet,? Ask-Upmark,s Nordlander,g and Gorman.10 The salient symptoms of the syndrome are peculiar creeping, crawling, prickling, and itchy sensations in the lower limbs. These are most frequently localized between the knee and ankle but also occur in the thighs and feet and less commonly in the arms and hands. The creeping sensations are not felt in the skin but deep in the muscles. The sensations appear to be worse when the limbs are at rest and are almost invariably worse in the evening and at night. They are relieved by movement. Poorly defined feelings of weakness and pain also have been associated with this syndrome. Though no specific etiology was established, Ekbom felt it probably was vascular in origin. Our observations of this syndrome in 4 uremic patients with slow nerve conduction and in 1 patient with peripheral neuropathy suggest that in some patients it may be an early sign of peripheral nerve damage.

Ultrastructure of atrophic muscle in amyotrophic lateral sclerosis.

Abstract: THE HISTOPATHOLOGY of neurogenic atrophy in skeletal muscle has been described in extenso under experimental and clinical situations.'.* The cytopathology of this process, however, is less well known. At the time of this writing, only five studies dealing with the ultrastructure of muscle in neurogenic muscular atrophy have been reported. Pellegrino,3 Wechsler,4.5 and Lee6 described the electron microscopic findings of denervation atrophy in rats. Wechsler and Hager7 published their observations on a biopsy from a patient bearing the diagnosis of Werdnig-Hoffmann disease and Roth and associates8 reported findings on biopsies from patients with a Kugelberg-Welander type of atrophy. The present report is concerned with the ultrastructure of muscular atrophy in amyotrophic lateral sclerosis. The patients included in this study were 4 unrelated adults: 3 males and 1 female. Their ages ranged from 53 to 69 years, and the duration of their illness varied from six months to two years. In every case, the clinical picture was characterized by the presence of progressive weakness, atrophy, and hyperreflexia without demonstrable sensory deficits. Electromyography in all four patients showed ecidence of denervation in the tested muscles and normal conduction velocities of peripheral nerves. The electromyographic studies preceded the biopsy and were performed in areas

Ctenoids in healthy youths. Controlled study of 14- and 6-per-second positive spiking.

Abstract: SINCE 1951, when Gibbs and Gibbsl first published their description of the electroencephalographic (EEG) phenomenon which they designated as 14and 6-per-second positive spiking, there has been growing interest in this finding. It appears to be most prevalent in the 5to 15-year-age group and seldom appears outside of the stages of drowsiness and light sleep. Typical examples are shown in Figures 1, 2, 3, and 4. It is possible that much of the interest in these bursts may derive from their striking appearance and rather complex morphology, for which we have proposed the name “ctenoids,” from the Greek KTEN6S = comb, as a snbstitute for the more unwieldy and only illusorily precise phrase “14 & 6/second positive spikes.” Equally remarkable are the uniformity of their distribution and ease of identification. According to Gibbs and Gibbs? a rather broad spectrum of clinical manifestations was associated with the occurrence of ctenoids. Almost as puzzling as the congeries of clinically unrelated symptoms has been the diversity of presumed etiologies. Subsequent workers, while in earlier years denying either the reality or the significance of these complexes, gradually began to take them more and more seriously, variously stressing their association with complaints of vegetative dysfunction or of behavioral disturbances, both notorious sonrces of perplexing diagnostic problems. Perhaps the most complete paper dealing with the association between ctenoids and the autonomic nervous system is that of Kellaway and associates,3 who formulated a specific electroclinical syndrome in 459 children with primary complaints of headache or abdominal pain associated with other auto-

Depression of parahydroxylation of diphenylhydantoin by antituberculosis chemotherapy

Abstract: IT HAS BEEN observed that some patients who receive diphenylhydantoin in the usual dosage of 300 mg. or 4 to ij mg. per kilogram of body weight simultaneously with antituberculosis chemotherapy develop nystagmus, ataxia, and drowsiness, the common signs of diphenylhydantoin intoxication. This apparent “hypersensitivity” or low tolerance to diphenylhydantoin has been observed in patients who had tolerated their anticonvulsant medications well for years but began to show evidence of diphenylhydantoin intoxication shortly after they had contracted tuberculosis and were given antituberculosis chemotherapy. It has also been observed in patients who have been receiving antituberculosis chemotherapv and then given diphenylhydantoin. In order to explain this phenomenon of decreased tolerance, diphenylhydantoin metabolism was studied in patients receiving diphenylhydantoin and antituberculosis chemotherapy. Evidence of lowered tolerance was found in 16 patients over a four-year period from an active tuberculosiq service.

Spongiform encephalopathy with chronic progressive external ophthalmoplegia. Central ophthalmoplegia mimicking ocular myopathy.

Abstract: CHRONIC PROGRESSIVE external ophthalmoplegia is a rare condition which usually begins with bilateral ptosis and progresses to involve all the extraocular musculature in a symmetrical fashion. The end result is complete immobility of the eyes with preservation of the pupillary reactions. The onset is often in childhood but may be at any age. In the nineteenth century literature, reviewed bv Wilson1 and Kiloh and Nevin,' the syndrome usually was attributed to brainstem nuclear atrophy. This concept prevailed despite the lack of good anatomical verification. The first case with well-described brainstem lesions was reported by Langdon and Cadwalader\"4 in 1928. They studied an 84-year-old woman who had chronic progressive external ophthalmoplegia for forty years. There were changes in the third, fourth, and sixth cranial nerve nuclei which the authors felt were responsible for the ophthalmoplegia. The only other anatomical support for a nuclear etiology was by Jedlowski5 in 1943. He examined the brain of a 29-year-old woman with the disorder who died of pneumonia. There was considerable degeneration of the pertinent cranial nerve nuclei. The extraocular muscles were said to reveal neurogenic atrophy. However, the fascicles and nerves were normal. SandiferG in 1946 had biopsied a medial rectus muscle in a patient with chronic progressive external ophthalmoplegia and stated that the, changes were indicative of myopathy. In 1950, Scharf,' without anatomical support, argued that the clinical findings were most compatible with supranuclear lesions. One year later, Kiloh and Nevi9 reported 3 patients with this syndrome, 2 of whom had extraocular muscle biopsies which were said to be diagnostic of a myopathy. All 3 had muscle weakness elsewhere in the body. No examinations of the brainstems or nerves were included. These authors contended that their cases proved that chronic progressive external ophthalmoplegia was an ocular myopathy and further indicated that there was no significant evidence that nuclear degeneration ever produced this syndrome. Langdon and Cadwalader's reports334 were discounted because of the advanced age of the patient and the mild degree of nuclear changes. They felt that Jedlowski's case5 was unconvincing because of the normal fascicles and nerves. Also, Kiloh and Nevin2 stated that the nuclear degeneration may have been secondary to primary muscle disease. Since Kiloh and Nevin's paper there has been almost universal agreement in subsequent reports, based primarily on extraocular muscle biopsies, that chronic progressive external ophthalmoplegia is an ocular myopathy. Others have concurred that the concept of primary nuclear degeneration be abandoned.8-10 Only occasional mention of supranuclear factors are found, but without verifying data from autopsy analysis.11 The following case report is of a girl with chronic progressive external ophthalmoplegia in whose brain a widespread status spongiosus

Cerebrospinal fluid perfusion for central nervous system neoplasms

Abstract: CENTHAL NERVOUS system neoplasms have long defied effective treatment.1-4 The functional anatomy has limited the surgical approach.2,4,5 The unique physiology and microscopic anatomy implied in the concept of a “blood-brain barrier,” limits the entrance of many chemotherapeutic agents from blood to brain+-s and, by so doing, makes systemic chemotherapy inadequate.9 Local arterial perfusion, which is promising in some anatomical sites, does not circumvent the “blood-brain barrier” and is in addition fraught with prohibitive toxicity.10-12 The unique anatomy, physiology, and pharniacology of the central nervous system can, however, be beneficially utilized and their previously frustrating limitations advantageously applied. It has been shown recently that lipid insoluble and highly ionized organic moleculeslx can pass, by diffusion, from cerebrospinal fluid to brain,GJ4Jb while the “bloodbrain barrier” limits movement of such substances in the opposite direction, that is, from brain to blood. Accordingly, a system was devised utilizing cerebrospinal fluid pathways for the continuous and restricted delivery of high concentrations of chemotherapeutic agents from ventricular and spinal fluid to brain.l(;J7 The great disparity in deoxyribonucleic acid (DNA) metabolism between normal brain and malignant tissue should make these tumors an ideal target for chemotherapeutic agents.lx-’O

Treatment of status epilepticus with diazepam.

Abstract: STATUS EPILEPTICUS constitutes a medical emergency, as well as a difficult problem of therapy. This statement has been made authoritatively and sufficiently frequent so that to belabor the point further is unnecessary (Gowers,l Clark,2 Turner,3 Wilson,4 and the more recent review of the problem by Hunter”. Two features of the problem seem worthy of stress here, as they do not seem to have received much attention in the literature. The first concerns the possible iatrogenic effects attributable to excessive treatment. In the emergency presented by status epilepticus, the tendency is often to overdose. A patient in status is likely to have dissipated much of his vital reserves and may more easily become a victim of the depressant effects characteristic for most, if not all, antiepileptic agents. The cardiac and respiratory centers and functions, already overburdened, are particularly vulnerable. In the course of an analysis concerned in establishing the immediate cause of death in patients that succumbed in status epilepticus at the Children’s Hospital Medical Center over a ten-year period, just short of a quarter of the total number was found to have had cardiac or respiratory arrest, or both, closely following the intravenous administration of a hypnotic agent.6 Further, there are some disquieting reports dealing with central nervous system lesions attributable to other nonbarbituric drugs, when administered parentally and in the large doses often used in status.?-9 It would seem, therefore, that a need still exists for drugs that would interrupt the vicious state of “continuous epilepsy” but would not be the source of equally dangerous iatrogenic effects. Such need has been obviously felt by many, as shown by the proposal to use intravenous urea, as advocated by Carter,lo or the intracarotid injections of amylobarbital, as advocated by Bladin.ll The other point needing clarification in a

Measurement of the pain threshold determined by electrical stimulation and its clinical application.i. method and factors possibly influencing the pain threshold

Abstract: INVESTIGATIONS of the sensory threshold to stimulation of the skin have become increasingly intensive during the last sixty years.lg2 Electrical stimuli were selected in this investigation to determine the pain threshold because of the following advantages of this method: 11 It is a simple way in which an exactly measurable and, therefore, reproducible stimulus can be applied over various regions of the body and can easily be expressed in physical terms (milliamperes). 21 It has the least chance of damaging the tissues. 31 It produces an easily recognizable and easily definable “pricking pain” sensation. The clinician needs to measure the pain sense quantitatively instead of relying on his subjective impression obtained with the help of his so-called “neurologic pin.” In this study, we define pain threshold in the same way as Bonica,3 that is, as the lowest electrical current-expressed in milliampereswhich at a fixed frequency and impulse duration first evokes a sensation of (pricking) pain. The purpose of this study was to develop a practical and reliable method for clinically determining pain thresholds of the skin, particularly in patients with disturbances of pain sensation. Specifically, this study attempted to establish those characteristics of a reproducible electrical stimulus necessary to measure and standardize the pain threshold of the skin. The following problems also were investigated: [l] the relationship and characteristics of an electrical stimulus required for its being exactly reproducible as pain threshold stimulus, [2] factors which might influence the pain threshold, [3] possible variations of the pain threshold in the course of time (in the same individual), [4] the pain threshold of different parts of the body surface, [5] possible variation of the pain threshold in different individuals, and [6] the influence of diseases of the nervous system on the pain threshold. The electrical method with square wave impulses has many advocates as noted by BjOrt~,~ Sige1,S Chemnitius and associates,6-H and Blake and associate^.^ Harris and Blockuslo described this method as being perhaps one of the most suitable for future use because it is very simply applied and constant reproducibility of the stimulus is possible. A survey of the literature regarding electrical stimulation of the skin indicates that control of voltage alone is quite unsatisfactory. In this study, it is stressed that in electrical skin stimulation, the applied voltage does not

Transient ischemic strokes: A report of a study of anticoagulant therapy

Abstract: THE TERM “transient ischemic stroke” or “transient cerebral ischemic attack is used to designate neurological disturbances of brief duration due to focal cerebral ischemia caused by, or related to, occlusive vascular disease. The brief duration or transiency is a key issue. In this report, we include only patients whose symptoms cleared completely within a few minutes or a few hours and who were neurologically normal within twenty-four hours. Many conditions besides transient disturbances of cerebral circulation may produce similar symptomatology. These must be carefully excluded. Concepts of pathogenesis and pathophysiology are frequently implied or assumed in discussions. They are important but are difficult to elucidate and interpret. For this reason, the term transient cerebral ischemic attack is used in this report as a descriptive diagnostic grouping based on clinical findings.

The bobble‐head doll syndrome: Report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children

Abstract: RECENTLY WE HAVE observed two children with to-and-fro bobbing or nodding of the head and trunk. The movement is reminiscent of that seen in dolls with weighted heads resting on a coiled spring, and thus it is named “bobble-head doll syndrome.” Each patient had a large cyst in the region of the third ventricle and dilated lateral ventricles. The disorder was unique in our experience, and no reference to a similar syndrome could be found in the literature.

The response of human cerebral edema to glucosteroid administration. An electron microscopic study.

Abstract: THE HIGH MORTALITY associated with brain swelling led very early to attempts to discover methods for its relief. Lack of basic understanding of the process has hampered development of effective therapies. For many years, the only reasonable treatment available was a variety of decompressive operative procedures.1 The first rational medical therapy resulted from Weed’s descriptions of the effects of hypertonic solutions upon brain bulk.”3 These substances have proved effective for short-term use. The appearance of both normal and edematous brain following infusion of these hypertonic solutions has been studied with both the light microscope and the electron microscope. The findings, in general, support the hypothesis4-6 that their effectiveness relates to their osmotic properties. More recently, adrenal cortical steroids have been found to be effective in the long-term control of symptoms due to cerebral However, few studies comparable to the above have been carried out to show the effectiveness of the corticoids or to help elucidate the mechanism of action. I t is the purpose of this report to present electron microscopic studies designed to show the response of cerebral edema to adrenal glucocorticoid therapy in human beings.

The sympathetic chain in patients with diabetic and alcoholic polyneuropathy.

Abstract: AUTONOMIC FUNCTION may be defective in patients with diabetic and alcoholic neuropathy. Thus, reflex circulatory adjustments which occur after sudden changes in posture and depend on sympathetically mediated vasoconstriction are impaired in some patients with these disorders.1p2 Thus far, however, no systematic investigation has been undertaken in an attempt to correlate these functional deficits with the morphological appearance of the autonomic ganglia or nerves. The present study is based upon an examination of sympathetic chains obtained at autopsy in patients dying at the Massachusetts General Hospital. They all had a well-documented history of diabetes mellitus or chronic alcoholism, and some of them had a complicating polyneuropathy.

Granulomatous giant-celled angiitis of the central nervous system.

Abstract: ARTERIAL DISEASE of the central nervous system may be secondary to disease processes such as syphilis, tuberculosis, and pyogenic leptomeningitis or it may be a primary condition. With the exception of arteriosclerosis, the primary disease processes affecting the cerebral arteries are all rare. Of those that have been described, the most important are periarteritis nodosa.1 temporal arteritis,2 and thrombotic microangiopathy.3 Thromboangiitis obliterans4 involving the nervous system has been described, b u t recently the separate identity of this disease process has been questioned. Pulseless disease5 also affects the central nervous system, but the pathology of this obscure condition is centered in the aorta and other major arterial trunks. Distinct from all the diseases mentioned is a rare arteritis of the small cerebra1 arteries which are affected by a granulomatous giantcelled inflammatory reaction. Eight cases of this nature have been previously reported.6-11 The same type of disease process affected the cerebral arteries of all eight patients. The following case report of a patient seen at the Radcliffe Infirmary, Oxford, was similar to the eight reports previously published.

A histochemical study of developing human skeletal muscle

Abstract: THE APPLICATION of histochemical techniques to human skeletal muscle has consistently revealed the existence of two muscle fiber populations. Type I fibers are identified by their intense activity with many of the mitochondria1 oxidative enzyme reactions, while the type I1 react strongly with myosin adenosine triphosphatase ( ATPase) and phosphorylase.1,' In other vertebrates, a third intermediary fiber population is present?-5 with eight subclassifications of fiber type identified in the albino rat.fi.7 In prior studies, the general principle has held that those fibers which are rich in most oxidative enzyme activities are poor in most myofibrillary and sarcoplasmic enzyme reactions and vice versa. The present paper summarizes the sequence of events in the development of the two fiber types of human skeletal muscle. Similar studies have been carried out in mouses and chick enilxyosg which have indicated species' differences. In the muscles of infants with spinal muscular atrophy, type I fibers have been shown to undergo atrophy and hypertrophy earlier than type I1 fibers.l\" This observation has not been extended to anterior horn cell disease of later life and may suggest embryologic differences between the two fiber types.

Deep cerebral venous system in man. A microangiographic study on its areas of drainage and its anastomoses with the superficial cerebral veins.

Abstract: IhiPROVEhlENTS in radiological technique have resulted in the main trunks of the deep cerebral veins being visualized during cerebral angiography.1 In order to evaluate variations in contrast filling, localization, and caliber of the deep cerebral veins during such investigations, more detailed knowledge of the normal cerebral venous system anatomy is desirable than found in classical anatomic works.2 During recent years, the study of the cerebral circulation in man has attracted much interest.3-5 Increased knowledge about the anatomy of the deep cerebral venous system and its anastomoses may lead to a better understanding of regional cerebral circulation under normal and pathologic conditions. The cerebral veins differ from other veins by having no valves. They may therefore be filled with a contrast agent by retrograde injection. This circumstance has been exploited to stud\\: the anastomoses between deep and superficial cerebral veins and to map out areas of brain drained by the deep cerebral veins.

Syndrome of akinetic mutism associated with obstructive hydrocephalus

Abstract: AKIhETIC MUTISM associated with an epidermoid cyst of the third ventricle has been discussed as a case report by Cairns and associates.’ Aspirations of the cyst produced transient improvement in the akinetic state, and surgery with resection of the cyst produced a satisfactory clinical recovery. From this case, the authors concluded that the state of akinetic mutism was related to pressure transmitted to the diencephalon by the dilated walls of the third ventricle. The increased intracranial pressure was not considered to be a factor, and although obstructive hydrocephalus was present and mentioned, it was not thought to be contributing to the symptomatology. Following this early report, numerous cases of akinetic mutism have been described in the literature and found to be related to a variety of different etiologies. Klee? found cases of akinetic mutism related to tumors of the third ventricle, hemangioma of the mesencephalon, basilar artery thrombosis, Wernicke’s encephalopathy, encephalitis, bullet wounds of the frontal lobes, and also a number of cases of unverified etiology. Sours,a in a similar review of the literature, found Cairns’ syndrome in cases of malacia of the pons; lesions in the peiiaqueductal gray, diffuse posttraumatic states; hemorrhages in the thalamus; malacies in the corpus callosum; infiltrating tumors in the frontal lobes; and bilateral subdural hematomas. In these reports, no specific mention is made of the possible influence of increased intracranial pressure or obytructive hydrocephalus or both. We are presenting 2. cases where we feel that both obstructive hydrocephalus and variations in the gradients of intracranial pressure seem to be the major underlying factors in the production of akinetic mutism and that akinetic mutism represents a global apraxia. We also propose that obstructive hydrocephalus may be a possible common denominator, tying together a number of unrelated etiologies producing similar states of akinetic mutism. We will present 2 additional cases with incomplete manifestations of akinetic mutism. Cairns and associates1 gave such a clear description of akinetic mutism that we feel it worthwhile to repeat it here: “The patient sleeps more than normally, but he is easily roused. In the fully developed state he makes noisound and lies inert, except that his eyes, regard the observer steadily, or follow the movement of objects, and they may be diverted by sound. Despite his steady gaze, which seems to give promise of speech, the patient is quite mute, or he answers only in whispered monosyllables. Oft-repeated commands may be.carried out in a feeble, slow, and incomplete manner, but usually there are no movements of a voluntary character; no restless movements, struggling, or evidence of negativism. Emotional movement also is almost in abeyance. A painful stimulus produces reflex

The trigeminal artery and associated aneurysms.

Abstract: THE ANGIOGRAPHIC demonstration of the most common type of carotid-basilar anastomosisthe trigeminal arteiy-is no longer a rarity. According to Wollschlaeger and Wollschlaeger,' who reviewed the literature and added 2 cases of their own, 134 cases had been described by early 1963. Over the last three years, 3 trigeminal arteries were angiographically demonstrated at the Addington and King Edward VlII hospitals in Durban. I n these cases, aneurysms arising from the circle of Willis and from the trigeminal artery itself were noted. The association is the subject of this paper.

Neurological syndromes occurring in patients receiving synthetic steroids (oral contraceptives).

Abstract: Case studies of 34 women with neurological syndromes associated with oral contraceptive use are discussed with a view to identifying contraindications of progestational steroid use. The patients were classified by occlusive cerebral arterial disease (5 patients) cortical vein thrombosis (1 patient) and vascular headaches (28 patients). Studies are cited reporting the relationship of oral contraceptives to arterial thrombosis coronary artery thrombosis cerebralvascular disorders and intravascular thrombosis. The possible relation of vascular disorders to thrombosis is mentioned. It is concluded that there is not enough evidence to demonstrate a specific correlation between neurological disorders and progestational steroids. It is recommended that oral contraceptives be administered with caution under these conditions: 1) hypertension 2) history of recurrent vascular headaches 3) history of Raynauds or other vasospastic phenomena 4) history of occlusive arterial disease and 5) epilepsy.

Syndrome of progressive spastic ataxia and apraxia associated with occult hydrocephalus.

Abstract: MANY PATIENTS, especially elderly persons, are referred to the neurologist with unassuming complaints such a s “progressive difficulty in gait” or “frequent falls.” This gait difficulty is often thought to be a manifestation of such conditions as cerebral arteriosclerosis with chronic brain syndrome, parkinsonism, senile dementia, or a variety of other neurologic disorders. Other diagnostic impressions are scmetimes considered in younger patients. They include degenerative disease, demyelinating disease, or, if alcohol appears to have been a factor, alcoholic degeneration. From our observations of a number of such cases, it is suggested that many of them may represent a fairly discrete neurologic entity which is not well known but which may have considerable diagnostic significance. The main feature common to these cases has often been a spasticity and gait disturbance-either an ataxia or apraxia of gait. The degree of pyramidal tract involvement may vary from mild hyperreflexia to overt spasticity, but weakness is frequently insignificant or absent. Early in the course, the findings may be confined to the gait, with little or no apparent involvement of the upper extremities, cranial nerves, or even mental status. From our observations, it appears that this syndrome may reflect a significant-to-marked internal hydrocephalus, either so-called “idiopathic” or “nonobstructive” atrophic white matter degeneration with ventricular dilatation, or it could be obstructive hydrocephalus due to a variety of obstructing lesions. The similarity in histories and findings between these 2 categories seems to warrant emphasis on features which have not been

Aphasia due to a right hemisphere tumor in a right-handed man.

Abstract: IN COMMENTING on the role of the “minor hemisphere” of the brain in speech, MacDonald Critchleyl notes the “rare appearance of aphasia after lesions of the right hemisphere in subjects who have shown no personal nor familial evidence of sinistrality whatsoever.” We have recently cared for such a patient during the last stages of a fatal illness. Consideration of his case, and a review of the literature on this problem, has led us to some unconventional speculations which we hope may be provocative.

Myelopathy due to cervical spondylosis treated by collar immobilization

Abstract: THE CLINICAL FEATURES and diagnosis of myelopathy associated with degenerative cervical spondylosis have been fully described by Brain and associates' and Clarke and Robinson.2 Factors involved in the pathology of the cord lesion-compression by osteophytic bars3 and ligamenta flava,4 trauma due to neck movements,5*6 and ischemia7~s-are recurrently the subject of review, but the contribution of each of these and other factors in the production of the lesion is still debatable and treatment therefore remains empirica1.Q Evaluation of the results of treatment has been complicated by limited knowledge of the course of the untreated condition and by the fact that in all previous reports there has been selection of patients for the various forms of treat rnent available. Lees and Turnerlo recommend a more conservative approach to the management of what they conclude is a relatively benign condition on the basis of their long-term study of its natural history in fortyfour treated and untreated patients. Reports of the results of surgical treatment are numerous but there i s surprisingly little information on the results of treatment by collar immobilization. Such reports as there are iefer to series of patients from among whom there has been selection for surgical treatment on the grounds of the severity or other characteristics of the myelopathy or its failure to respond to conservative management. These were not considered grounds for surgical intervention in the series reported here. In this respect, therefore, there has been no selection for treatment by collar immobilization. at the Regional Neurological Unit, Brook Hospital, London. The period reviewed was August 1958 to March 1965 because it was his practice at that time to admit for myelography all patients in whom this diagnosis was considered and where it was confirmed to recommend immobilization of the neck in a plastic or metal frame collar preceded by two to three weeks' bed rest in hospital. Surgical treatment consisted in this period of decompression laminectomy above and below the level of the main lesions with division of the denticulate ligaments. This procedure was considered of dubious and unpredictable benefit and was not recommended except where complete myelographic block indicated marked cord compression. The diagnosis of myelopathy due to cervical spondylosis was found to have been confirmed in thirty-eight patients in the period under review. Three of these had had exploratory laminectomy because a neoplastic lesion could not confidently be excluded, another because the myelographic appearances were those of an intramedullary neoplasm, and another for a supposed extramedullary tumor found at operation to be an arachnoid cyst associated with spondylosis. Two patients had laminectomies for large spondylotic bars producing complete myelographic block. Three patients remained untreated because of age and general infirmity associated with a long and nonprogressive history and another because she discharged herself from hospital. One had died of a probable cardiac infarct, another had developed evidence of disseminated sclerosis, and another developed a pseudobulbar palsy.