Showing papers in "Journal of Neurology, Neurosurgery, and Psychiatry in 1963"

A follow-up study of surgery in temporal lobe epilepsy

Abstract: Since the pioneering work of Wilder Penfield (Penfield and Flanigin, 1950), of Morris (1950), and of Bailey and Gibbs (1951), the surgical possibilities of relieving intractable epilepsy originating in the temporal lobe have interested several neurosurgical centres. In 1955 our own group (Falconer, Hill, Meyer, Mitchell, and Pond) surveyed the literature and presented the results of operation in 30 consecutive patients who had been followed up for periods of from one to four years. A further report on 50 consecutive patients was made in 1958 (Falconer, Hill, Meyer, and Wilson, 1958). In the earlier paper 12 of the 30 patients were reported as apparently free of seizures and 14 others greatly benefited. All but one of these patients had also exhibited a psychiatric disorder pre-operatively, and 20 of these were improved, 17 markedly so. The published results of surgery from other centres at that time were also mentioned. However, it was stated that 'in pioneering any new form of therapy its sponsors must realize that several years must elapse before its merits can be accurately assessed'. We therefore now propose to give such an accounting, and to set out the results of surgery in 100 consecutive patients operated on and followed up for periods ranging from two to 10 years.1

Trans-synaptic retrograde degeneration in the visual system of primates.

Abstract: Although the first observations of trans-synaptic degeneration in the nervous system were made in the nineteenth century, the phenomenon has been something of an infrequent curiosity. From an experimental point of view it is not a particularly attractive field for study due to the time required for the full effects to become evident and may entail many months or even years of waiting. Consequently the majority of the observations have been made in man. Naturally occurring human lesions, even old cerebral infarctions, which are perhaps the most useful for anatomical purposes, practically never approach the discreteness of an experimental ablation since cerebral vascular disease is seldom a localized process. Although this material is of great value, usually the neuropathologist cannot undertake a comprehensive study of the entire brain in serial section so that one is left with the somewhat uncomfortable thought that possibly significant lesions had been missed in the neuropathological sampling. In the course of another study on the topographical anatomy of the ganglion cell layer of the retina in primates (Van Buren, 1963), long-term chronic lesions were placed in the visual system. This study presents three cases of retrograde trans-synaptic degeneration. In two cases cavitary degeneration appeared in the inner nuclear layer of the retina after section of the axons of the ganglion cells at the optic chiasm. In the last case degeneration of the ganglion cell layer of the retina followed an occipital ablation.

Treatment of hydrocephalus: an historical and critical review of methods and results.

Abstract: Hydrocephalus has been recognized as a clinical and pathological entity since the days of Hippocrates; but it is fair to say that as late as the beginning of the present century the pathology of this condition was obscure, no rational methods of therapy had been developed, and, so far as I can determine, no successful surgical treatment of hydrocephalus had ever been achieved. Then between 1913 and 1929, Walter Dandy, almost single-handed, established the true pathology of hydrocephalus and developed sound physiological and surgical principles for its treatment. Dandy and Blackfan (1913, 1914; Dandy, 1919, 1929) first proved that the cerebrospinal fluid is formed within the ventricles, principally if not entirely by the choroid plexuses; that approximately 800 to 1,000 ml. of cerebrospinal fluid is formed each 24 hours within the ventricles; that the only escape of the cerebrospinal fluid from the lateral and third ventricles is by way of the aqueduct of Sylvius, the fourth ventricle, into the cisterna magna, and thence into the other subarachnoid spaces; that the circulation of cerebrospinal fluid from the lateral ventricles to the subarachnoid spaces normally requires only two to three minutes; that the absorption of the cerebrospinal fluid back into the blood stream is from the subarachnoid spaces directly into the rich capillary beds within the subarachnoid spaces. Dandy and Blackfan (1913, 1914) then proved the existence of two distinct types of hydrocephalus: 1, the obstructive (or non-communicating) type, and 2, the non-obstructive (or communicating) type. They established that the cause of non-communicating (obstructive) hydrocephalus is the inability of the cerebrospinal fluid to escape from the obstructed ventricles to the subarachnoid system where it can be absorbed by natural processes; and that the cause of communicating (non-obstructive) hydrocephalus is impaired absorption of the cerebrospinal fluid after it has reached the subarachnoid system because of congenital mal-development of the subarachnoid spaces or their obliteration by post-inflammatory adhesions. To distinguish, clinically, between the communicating and the non-communicating types of hydrocephalus, Dandy and Blackfan (1913, 1914; Dandy, 1919) devised an extremely simple (dye) test which successfully serves this purpose.

The refractory and supernormal periods of the human median nerve

Abstract: While the excitability changes which follow a propagated nerve impulse have been the subject of numerous studies in animals, there have been few references to this subject in man. An attempt to study the refractory period of the human ulnar nerve was made by Wagman and Flick (1951), but as recordings were made from the hypothenar muscles rather than from the nerve itself, the results are difficult to interpret. Krnjevic, Kilpatrick, and Aungle (1955) applied pairs of shocks to the ulnar nerve at the wrist, and investigated the least interval at which a response to the second shock could be recorded from the nerve at the elbow; only two subjects were examined and no estimate of the relative refractory period was made. Brown (1960) also used stimulation of the ulnar nerve at the wrist with recording at the elbow in four subjects, and noted that the recovery of normal responsiveness was followed by transient supernormality. In the present work we have studied the refractory and supernormal periods of the human median nerve with stimulation at the wrist, action potentials being recorded from the nerve above the elbow by a technique similar to that originally described by Dawson and Scott (1949). A briefpreliminaryaccount of this work has been published elsewhere (Gilliatt and Willison, 1962).

Nerve fibre size in the carpal tunnel syndrome

Abstract: The syndrome due to compression of the median nerve in the carpal tunnel was first defined by Ramsey Hunt (1911, 1914) and Marie and Foix (1913), although its relationship to the acroparaesthesia syndrome was not appreciated until recently (see Kremer, Gilliatt, Golding, and Wilson, 1953). Marie and Foix (1913) examined the pathological changes in a single case, but no further studies on the histological alterations in this condition have appeared since. Recently, the opportunity presented itself for such an examination in a patient who died from an unrelated disorder. Particular attention has been given to alterations in nerve fibre size, which are of interest in relation to the changes that occur in nerve conduction (Simpson, 1956; Carpendale, 1956; Thomas, 1960).

Defect of memorizing of hippocampal-mammillary origin: a review

Abstract: Thehistory of'amnesie dememoration' hasevolved through three stages. Thefirst, aclinical one,started in1887withKorsakoffs description ofamorbid association characterized by twobasiccriteria. These were, first, apsychological disorder associated withdegenerative polyneuritis, andsecondly a mental syndrome whichincluded aspecial formof amnesia. Thissyndrome captured theattention of psychiatrists likeSeglas (1895), Regis(1923), and Dupre(1903) wholooked onthememorydisorder asamental aberration andstressed theinterest of thepsycho-polyneuritis syndrome, theclinical actuality ofwhichhadbeenfully accepted. However,thesemeiotic significance oftheamnesic syndrome described byKorsakoff wasnotoverlooked byauthors like Bonhoeffer (1904) inGermany andChaslin (1895; 1912) inFrance. As pointed outbyAngelergues (1958), Chaslin mustbepraised foremphasizing theimportance ofKorsakoff's mentalsyndrome whichhewastodefine asa symptomatic triad ofamnesia, fabrication, and

The effect of metabolic acidosis and alkalosis on the blood flow through the cerebral cortex

Abstract: There is conflicting evidence on the effect of 'acids' and 'alkalis' on the cerebral blood vessels. Although Schieve and Wilson (1953) and Lambertsen, Semple, Smyth, and Gelfand (1961) have clearly dissociated the cerebral vasodilatation which occurs on raising the arterial PCO2 from the concomitant fall in blood pH, some workers have claimed that acids dilate and alkalis constrict the cerebral blood vessels (Wolff and Lennox, 1930; Geiger and Magnes, 1947; Kety, Polis, Nadler, and Schmidt, 1948), while opposite effects have been reported by Schieve and Wilson (1953) and by Bronk and Gesell (1927). The recent development by Lassen and Ingvar (1961) of a rapid and easily repeatable method of estimating the blood flow through the brain cortex has enabled us to follow changes in blood flow through a wide range ofpH values in the anaesthetized dog, while maintaining a steady arterial carbon dioxide tension by the use of controlled passive ventilation.

Extradural cysts of the spinal canal

Abstract: Extradural cysts are rare, space-occupying lesions of the spinal canal. In 1955 Wise and Foster summarized 33 cases from the literature and added one of their own. Nugent, Odom, and Woodhall reviewed the same cases in 1959, adding several more from their own experience and from the literature. The literature of these lesions is still limited. Fifty-six cases were found described, including those previously reviewed. There are only two cases of cervical cyst among them. The purpose of this paper is to add five cases to the literature, two of which are cervical, and to contribute to the discussion on the clinical aspects, aetiology, treatment, and prognosis of these lesions.

The effect of ischaemia on nerve conduction in the carpal tunnel syndrome

Abstract: It is now generally accepted that the carpal tunnel syndrome is caused by a lesion of the median nerve at the wrist under the flexor retinaculum. The site of the lesion has been established by clinical observations; for example, a local abnormality of the nerve just above or under the retinaculum is seen at operation in many patients, and, even in those in whom the nerve appears normal, attacks of pain and paraesthesiae are permanently relieved by division of the retinaculum (Kremer, Gilliatt, Golding, and Wilson, 1953). Furthermore, electrophysiological studies have demonstrated abnormalities of' motor and sensory nerve conduction below the wrist in these patients (Simpson, 1956; Gilliatt and Sears, 1958). The mechanism by which the symptoms are produced, however, remains uncertain. Thus, there is still considerable doubt as to the relative importance of direct pressure on the nerve and of ischaemia due to interference with its blood supply. The fluctuating nature of the symptoms in many patients suggests that vascular factors may be important and this possibility has been discussed by various authors (e.g., Brain, Wright, and Wilkinson, 1947; Kremer et al., 1953; Kendall, 1960). Gilliatt and Wilson (1953, 1954) have demonstrated that the median nerve of many patients with the carpal tunnel syndrome is abnormally susceptible to ischaemia. They studied sensory changes in the hand after a pneumatic cuff had been inflated round the arm to above arterial pressure. In some patients intense paraesthesiae in the fingers supplied by the median nerve developed within three minutes of the onset of ischaemia, accompanied by pain in the hand which often spread up the arm. Mild paraesthesiae occurred in normal subjects, but in the carpal tunnel patients they were more intense and predominantly median in distribution. Patients described the symptoms occurring during ischaemia as being similar to those experienced in their spontaneous attacks; this supports the suggestion that spontaneous attacks are caused by ischaemia. Gilliatt and Wilson (1954) also found that during ischaemia loss of sensation in the fingers supplied by the median nerve occurred earlier in patients with the carpal tunnel syndrome than in control subjects. These observations, however, depended on subjective changes reported by the patients. Nerve conduction may be studied objectively by electrophysiological techniques and the present work is based on the study of conduction in motor fibres of the median nerve during ischaemia. By correlating this with clinical and other electrophysiological abnormalities in patients with the carpal tunnel syndrome further information about the mechanism of production of symptoms and abnormal conduction in the damaged nerve fibres has been obtained.

The descending respiratory pathway in man.

Abstract: The descending respiratory pathway may be considered to be the most important tract of the spinal cord; yet its position in man is unknown. It is important to work out the location of these descending fibres not only to complete anatomical knowledge but also to provide those practising surgery on the cervical cord with essential information. The absence of this knowledge contributed to the death of some of our patients after cordotomy and this has also been a cause of death in patients from other series of cordotomies. Following Stookey's (1943) report of his experience of high cervical cordotomies, Penfield (1943) said: 'I have had a patient in whom I sectioned the pain tract at the second cervical segment under local anaesthesia. Suddenly, as the wound was being sutured, while he was talking and in the midst of a sentence, he died . . . Autopsy showed no explanation of it.' Stookey replied that he had never had such an experience following unilateral cordotomy but he had with the bilateral operation. He added: 'In bilateral cordotomy, I think that sectioning of the anterior lateral columns probably interferes with the higher respiratory pathway coming down from the medulla to the diaphragm ... The difficulties I have had have been with bilateral cordotomies and I felt they were respiratory deaths, due to interruption of an upper respiratory pathway.' Undoubtedly many neurosurgeons have had the same experience: patients having had unilateral and more commonly bilateral cordotomies in the cervical region may die suddenly, often in their sleep, and at necropsy no explanation has been forthcoming. It will be shown in this paper that an adequate high cervical cordotomy will damage most of the descending fibres controlling respiratory movements. In 1830, Sir Charles Bell made the suggestion that the tract for controlling respiratory movements lies in the lateral part of the cord, somewhere between the anterior and posterior roots. Although since then there has been work on animals that confirms Sir Charles Bell's conjecture, there has been, as far as I can ascertain, no evidence produced for man. In 1895, Porter looked for the location of the pathway running 'from the bulb to the phrenic

Normal plantar response: integration of flexor and extensor reflex components

Abstract: The reflexes elicited by painful stimulation of the plantar surface of the foot have been studied extensively for a long time and the relation between the reflexes obtained in normal and in pathological cases has been the subject of considerable debate. An excellent survey of previous investigations is to be found in the review by Walshe (1956). As in most studies of human reflexes, the technique commonly used has, however, not permitted an exact determination of the latency values of the reflexes, and it has thus not been possible to judge with certainty to what extent the movements studied have been purely spinal and to what extent of cerebral origin. By means of brief electric stimuli and an electromyographic recording technique these latency values can, however, be exactly determined, and in this way a clear distinction can be made between purely spinal reflexes and movements of a more uncertain origin. Using this technique, the spinal skin reflexes of the trunk (Kugelberg and Hagbarth, 1958; Hagbarth and Kugelberg, 1958), the leg (Hagbarth, 1960; Kugelberg, Eklund, and Grimby, 1960), and the foot (Eklund, Grimby, and Kugelberg, 1959; Kugelberg et al., 1960) have previously been investigated, and these studies have established the existence of an extensive system of spinal skin reflexes representing a highly purposeful defence mechanism for appropriate withdrawal reactions. In investigations of the spinal pain reflexes normally elicited from the plantar surface of the foot Kugelberg et al. (1960) found that stimulation of the ball and hollow of the foot evokes plantar flexion of the toe, whereas, conversely, stimulation of the ball of the great toe produces dorsiflexion of the toe. It was further established that in extreme pathological cases (with pronounced Babinski signs) painful stimulation of the plantar surface of the foot elicits a stereotyped flexor reflex with dorsiflexion of the great toe, independently of the stimulus site, and that the main difference between this pathological reflex and that normally evoked by hallux stimulation lies in the extent of the receptive field. The conclusion was drawn that the two reflexes -are essentially identical but that in normal cases, due to the suprasegmental control of the reflex centres, the receptive field of the reflex is limited to the skin area where it is adequate for protective purposes, viz., the ball of the great toe. Previous investigations (Eklund et al., 1959; Kugelberg et al., 1960) have shown that the main difference between the electromyographic pattern of a flexor plantar response and that of an extensor plantar response is that the reflex plantar flexion of the great toe is associated with activity in the short hallux flexor and reciprocal inhibition of the voluntary activity in the short hallux extensor, whereas, conversely, reflex dorsiflexion of the great toe is accompanied by activity in the short hallux extensor and reciprocal inhibition of the voluntary activity in the short hallux flexor. Landau and Clare (1959) have, however, put forward the view that the flexor plantar response differs from the extensor plantar response in that the long hallux extensor is engaged in thlatter but not in the former reflex type. The controversial results thus obtained in the two investigations will be discussed below. On the basis of the results reached by Kugelberg et al. (1960), on painful stimulation of the plantar surface of the foot, investigations were started on pathological cases showing few pronounced or uncertain Babinski signs. In the course of the experiments it was, however, soon apparent that before being able to judge of the results obtained in abnormal cases a closer study had to be undertaken of the receptive fields and the variability normally existing; the present work gives an account of the results obtained in this investigation of normal cases. In the present study, the latency values of the recorded bursts of activity have been determined (sect. 1). Only reflexes of shorter latencies than 200 msec. have been included. The interest has been focused on, and all conclusions drawn from, responses recordable within 100 msec. The receptive fields of the various foot and toe reflexes which could be elicited by stimulation of the plantar surface of the foot have been examined in detail under varying experimental conditions (sect. 2). Special attention has been given to the hallux movements evoked, as 39 P rocted by coright.

A study of visual perseveration

Abstract: The manifestations of disordered visual perception may, as established by Hughlings Jackson for neurological deficits in general, be divided into the'negative' and the 'positive'. The negative commonly present as defective areas of visual field. The positive, also not uncommon, consist in visual impressions which are either endogenous or represent an exaggerated or distorted perception of the outside world. An unusual variant of the latter is the increased apparent duration of visual stimuli known as visual perseveration (Robinson and Watt, 1947). This consists in the continuation or repetition of a visual sensation after the stimulus object which gave rise to it has been removed from sight (Critchley, 1950). Holmes (1931) mentioned a patient who, having seen someone walk past her bed, had the false impression that this had happened again immediately afterwards. Adler's (1944) patient reported the superimposition of successive visual impressions during recovery from carbon monoxide poisoning, and Robinson and Watt (1947) recorded transitory episodes of visual perseveration in association with post-traumatic epilepsy. The largest series of such cases was that of Critchley (1951), and further instances have been reported by Le Beau, Wolinetz, and Rosier (1952), Castaigne and Graveleau (1953), Potzl (1954), and Bekeny and Peter (1961). In two of his cases (1 and 4) Critchley (1951) was able to elicit the phenomenon on clinical examination. He nevertheless remarked: 'We are dealing with a phenomenon-within the province of a subjective experience. The examiner is largely at the mercy of the patient's own description of events, and it is not easy to check the accuracy of his statements and to measure and record the extent of the defect' (Critchley, 1953). For a study of visual perseveration in terms of disordered visual physiology, subjects with a not too evanescent, variable, or intermittent disorder are required. Also needed is a yardstick against which to measure the responses and criteria against which to assess their consistency. In the present study two suitable subjects were examined by methods which have in the past been used to establish the properties of 'after-sensations' as they occur in normal visual perception. Their responses were assessed against the yardstick of present knowledge of these properties, in order to determine whether 'visual perseveration' can be regarded not as created de novo by the pathological process, but as representing a quantitative alteration of normally occurring visual perceptual processes.

Aberrant nerve fibres within the spinal cord

Abstract: Raymond (1893), in a necropsy account of a case of syringomyelia, described in the wall of the syrinx small bundles of fine nerve fibres ensheathed with Schwann cells. He traced the origin of these fibres from the posterior root entry zone and decided that they arose from axons of the cell bodies in the posterior root ganglia. Similar findings in syringomyelia were subsequently recorded by Schlesinger (1902), Bischofswerder (1901), Jonesco-Sisesti (1929), and Druckman and Mair (1953). Similar bundles of nerve fibres within and around the spinal cord have been described in other disease processes: in tabes dorsalis by Nageotte (1899), in Pott's paraplegia by Fickler (1900), in malignant cord compression by Bielschowsky (1901) and by Druckman and Mair (1953), in traumatic cord lesions by Henneberg (1907), by Roussy and Lhermitte (1918), and by Klaue (1949), in multiple sclerosis by Berchenko (1935), and in cervical spondylosis by Mair and Druckman (1953). These references do not exhaust the world literature, and further instances are given in a review of the subject undertaken by Druckman (1955) for a symposium on regeneration in the central nervous system, held at Bethesda, Maryland, in 1954. Most of these quoted references refer to single instances of this phenomenon which appears to be rare although its exact incidence cannot be stated. In the present paper we record our own observations on the occurrence of these abnormal nerve fibres, illustrated by nine personally studied examples. The principal clinical features and neuropathological findings are given in the Table. In the account that follows we have confined our observations to the detailed microscopical studies of the aberrant nerve fibres.

Reflex vasomotor abnormalities in the hands of migrainous subjects

Abstract: Many symptoms and signs of migraine can be explained by changes in the calibre of cerebral vessels (Wolff, 1948). Abnormalities in the size and a reduction in the amplitude of pulsation of extracranial vessels during intervals free of headache have been described (Wennerholm, 1961; Tunis and Wolff, 1953). Anatomical variations from the normal adult pattern of the capillaries of the finger nail folds in migrainous subjects have also been found (Hauptmann, 1946). Because of these findings in extracranial vessels it was of interest to study vasomotor reflexes in the hand vessels of such patients during intervals free of headache. Kerslake and Cooper (1950) described vasodilatation in the hands of normal subjects in response to heating of the skin elsewhere. They showed that this increase in hand blood flow is dependent on neural afferents from the heated area. This is a reflex response different from the vasodilatation obtained with prolonged heating of the body, and depends on warm blood causing release of vasomotor tone (Pickering and Hess, 1933). This paper reports reflex responses observed in the blood flow in the hand of migrainous subjects.

Diagnosis of Krabbe's infantile leucodystrophy.

Abstract: Increasing interest isnowbeingpaidtoacorrect diagnosis intheearly stages ofthedifferent types of degenerative diseases ofthecentral nervous system ofinfancy andchildhood. Amongthediseases which affect thewhite matter ofthenervous system asubgroupisrecognized astheheredo-degenerative type ortheleucodystrophies. In1956Poserandvan Bogaert (1956) stated thatitwasalmost imposslble tomakeaclinical diagnosis oftheleucodystrophies unless there wasawell-documented family history. However, bythedemonstration ofacharacteristic sulphatide pattern ofurinary sediment lipids by paperchromatography themetachromatic typeof leucodystrophy (sulphatidosis) cannowbediagnosed duringlife(Hagberg and Svennerholm, 1960; Hagberg, Sourander, andSvennerholm 1961). It canalso bediagnosed through thedemonstration of brownmetachromatic granular deposits inperipheral nervebiopsy specimens (Thieffry andLyon,1959; Hagberg, Sourander, andThoren, 1962). Inthe other important typeofleucodystrophy, thegloboid cell typeofKrabbe's disease, nocharacteristic clinical laboratory tests havehitherto beendescribed. However, diagnostic help canbeobtained fromanalysis oftheproteins ofthecerebrospinal fluid. Thiswill beshowninsixwell-documented cases ofKrabbe's disease.

Sporadic and familial varieties of tonic seizures

Abstract: The term 'tonic seizure' implies the involuntary assumption of an abnormal posture for a limited time without associated clonic movements. One form of tonic seizure is familiar to the clinician and its mechanism is fairly well understood, the 'tonic cerebellar fit'. The attitude of the body during such a fit is one of extension of the neck, arms, and legs, with pronation of the forearm, flexion of wrists and fingers, and plantar flexion and inversion of the feet. This resembles the decerebrate posture of the experimental animal, and is considered to be caused similarly by any lesion which periodically produces a functional transection of the midbrain (Kinnier Wilson, 1920). However, there is another type of tonic cerebellar fit which was described by Hughlings Jackson (1906) in a patient with a tumour of the cerebellum, in which the posture differed from that of decerebrate rigidity by the upper limbs being in flexion, a feature which led Jackson to call the fits 'tetanus-like'. Consciousness may be retained in such attacks. In 1924 Sterling reported a series of patients who suffered from transient painful spasms of the face, neck, and limbs in the course of encephalitis lethargica, using the adjective 'tetanoid' to describe the postures of hands and feet. The term 'extrapyramidal epilepsy' applied to these attacks by Sterling is not really appropriate, since some of the spasms lasted as long as six hours, and their mechanism was, and still is, unknown. Wimmer (1925) recorded the case of a boy who developed attacks of torticollis with unilateral tonic spasm of the limbs in extension. Five years after the start of the attacks disorder of movement became generalized and continuous with the appearance of athetosis and torsion spasm. Wimmer regarded the initial tonic spasms as being an epilepsy originating in the corpus striatum. The histories of two patients with brief tonic spasms were reported by Spiller (1927) under the title of 'subcortical epilepsy'. One of these patients, whose spasms involved a lower limb, had clinical evidence of multiple lesions in the optic nerves and spinal cord. The other suffered from unilateral carpopedal

Epidemiology of multiple sclerosis in the counties of Northumberland and Durham

Abstract: Considerable variations in the scope and the methods of ascertainment employed in different epidemiological studies, to say nothing of divergent diagnostic criteria, have seriously jeopardized the value ofmany such observations in estimating the true prevalence of multiple sclerosis in various parts of the world. Acheson has pointed out that studies based on mortality statistics (Limburg, 1950; Acheson, 1961) are subject to misreporting and under-reporting which reduce their accuracy and comparability. Studies of hospital records (Chiavacci, Hoff, and Polvan, 1950), augmented on occasion by interviews of practising physicians and examination of death certificates (Ipsen, 1950), represent further attempts to obtain more accurate data. Investigations of special population groups such as U.S. veterans (Acheson and Bachrach, 1960; Acheson, Bachrach, and Wright, 1960), for which comprehensive medical records are available, have afforded opportunities for more circumscribed studies. However, the personally conducted population survey, drawing on every available source to compile a comprehensive register of patients, has been recognized as a superior method. Essential to the survey is the establishment of firm diagnostic criteria, which not only lend reliability to the investigation but also permit the subsequent employment of data in authentic comparative studies. One such survey in the field of multiple sclerosis was published more than 30 years ago (Allison, 1931) and important contributions on similar lines have been made by MacLean, Berkson, Woltman, and Schionneman (1950), Allison and Millar (1954), Hyllested (1956), Sutherland (1956), and Alter, Allison, Talbert, and Kurland (1960). These observations furnish a valuable frame of reference for studies now under way in several other parts of the world.

Skeletal muscle glycogenosis: an investigation of two dissimilar cases

Abstract: Glycogen is found in almost every tissue in the body, but is especially abundant in the liver where the content is very variable, and in skeletal muscle where the content is relatively constant except during prolonged exertion. The regulation of glycogen metabolism in these tissues is a matter of delicate equilibrium between a number of enzyme systems, defects in which may cause profound alterations in the amount and structure of the glycogen. The clinical results of such alterations have been known for many years as diseases of glycogen storage, or better, as the glycogenoses. The first cases, affecting liver and kidneys, were recognized by von Gierke in 1929. Since then other types of glycogenosis have been described and elucidated, including some which involve skeletal muscle. This study deals with the clinical, histological, and biochemical identification of two dissimilar cases of skeletal muscle glycogenosis. One, in a 48-year-old man, is the fourth of its type to be formally elucidated; the other, in a 4-year-old boy, is the second case reported and the first to be fully described.

The relationship between enzyme activity and neuroglia in plaques of multiple sclerosis

Abstract: Since Dawson's (1916) pathological studies on multiple sclerosis little further information has been obtained about the nature of the neuroglial reaction in the plaques of this disease and it is not known whether this response is primary or a secondary event in the process of demyelination. It has been suggested by Lumsden (Lumsden, 1951; McAlpine, Compston, and Lumsden, 1955) that the oligodendrocyte is selectively damaged in multiple sclerosis. 'A complete loss of this cell' was observed 'from the plaques of demyelination both in the acute and chronic lesions of multiple sclerosis. The oligodendrocytes disappear as abruptly as the myelin sheath.' Friede (1961a), reporting on the enzyme histochemistry of multiple sclerosis, records increased activity of both oxidative enzymes (DPNHtetrazolium reductase and succinic dehydrogenase) and hydrolytic enzymes (acid and alkaline phosphatases), as well as increased neuroglial and mitochondrial populations at the edge of active plaques, but he observed no such increase at the edge of inactive plaques. The increase in neuroglial population at the edge of the active plaque was partly due to oligodendrocytes. Friede agreed with Lumsden that there were no oligodendrocytes in the centre of the plaque. To increase our scanty knowledge of the pathogenesis of multiple sclerosis it is clearly important to know whether the oligodendroglia are primarily or secondarily affected in the disease and whether or not these cells do in fact disappear from the plaque. For this reason we have analysed the neuroglial population and examined certain histochemical enzyme systems in 33 plaques from 10 cases of multiple sclerosis.

Results of antero-lateral cordotomy for pain in cancer

Abstract: In this paper the effects and the practical results of antero-lateral cordotomy are reported. These results provide a guide to prognosis for those recommending the operation and material for comparison with other methods of treating intractable pain, although no comparison is made here between this and other methods. The anatomical aspects of our findings are not given in detail here. The series of patients presented in this paper has been collected over the past 15 years. In all the patients cancer was the cause of the intractable pain. Neurosurgery was resorted to only when it was clear that in reasonable doses analgesic drugs would not control the pain. All patients had previously had as much radiotherapy for the cancer as was considered advisable. The majority of the patients were observed daily and they were investigated in considerable detail before and after the cordotomies; the main purpose of the investigation was to study various aspects of the anatomy and physiology of the spinal cord in man. Sixty-eight of the 114 operations were performed by Mr. Wylie McKissock; the other operations were performed by 15 other neurosurgeons in this country. All the patients whose cases are reported are now dead. The period of survival after the cordotomy was six months or more in 18 patients; in three patients it was less than a week; in all others the duration of survival falls between a week and six months. The number of patients and operations are shown in Table I. One patient had two bilateral cordotomies and one patient had a unilateral cordotomy followed by a bilateral cordotomy. Seven patients had two unilateral cordotomies, first on one side and then on the other; the interval between the two operations was two months or less. One patient had two unilateral cordotomies, both on the same side. The numbers of patients and the kinds of neoplasm are listed in Table II.

The relationship between generalized paroxysmal E.E.G. discharges and various test situations in two epileptic patients

Abstract: Thisinvestigation wascarried outontwopatients suffering fromso-called primary subcortical epilepsy. Serial studies weremadebothofvariations inthe amountofparoxysmal electrical activity recorded fromthebrain during theperformance ofpsychological tests andofchanges intestperformance in thepresence ofparoxysmal discharges. Thereisno doubtthatchanges inthealpha rhythm canbeshowntoaccompany alterations in behaviour inallsubjects. Theparoxysmal E.E.G. discharges recorded fromepileptics haverarely been studied fromthispoint ofview. Itis,however, widely heldthatpetit malattacks donotoccur randomly withrespect tomental state. Lennoxand Lennox(1960), whotended toequate theelectroencephalographic dysrhythmia withtheclinical ictus, considered thatpetit malattacks occurless often whenthepatient iskeptoccupied. According toPond(1961), petit malattacks maybeprecipitated byemotional disturbance. Inthepresent investigationa series ofE.E.G.examinations andof psychological tests wascarried outparipassuin order todetermine whether there weresystematic differences intheamountofparoxysmal activity recorded indifferent test situations. Suchdifferences aswerefoundwerethenfurther explored inan attempt todiscover thefactors responsible forthem. Autonomic function seemed tobeonevariable of possible significance, andrespiration andpulse rate weretherefore recorded throughout. Theinvestigation wasalsoplanned topermit the studyofchanges intestperformance during the occurrence ofgeneralized paroxysmal discharges. Patients wereselected whoseE.E.G.records contained brief generalized wave-spike discharges, without clinical accompaniments. A distinction is oftendrawn(Kiloh andOsselton, 1961)between such'inter-seizure' discharges andtheelectrical disturbance associated withpetit mal attacks. However, brief discharges donotdiffer qualitatively intheir E.E.G. parameters frombursts oflonger duration andmight beexpected tohavebehavioural correlates whichcouldbemeasured bya more refined methodthanclinical observation. A number ofstudies havebeenmadeofresponses topsychological tests during brief discharges butthefindings areconflicting. Schwab(1941) andCornil, Gastaut, andCorriol (1951) stated thatreaction times are delayed, butPrechtl, Boeke, andSchut (1961) found noimpairment intherateofperformance onaserial reaction test, andMilstein andStevens (1961) foundno impairment inauditory learning and avoidance conditioning during brief generalized discharges. KooiandHovey(1957) recorded spontaneous paroxysmal discharges inepileptics during intelligence testing. Theystated that although thequestions wereusually heardan adequate solution wasnevergiven during bursts ofbilateral synchronous activity. However, theydonotstate theduration andnumberofthedischarges, andit isnotclear whether thereported association is significant. Although these findings areconflicting, there are

Cerebral pathology in subarachnoid haemorrhage.

Abstract: Subarachnoid haemorrhage due to ruptured intracranial aneurysm is very common but there have been few reports of the pathology in brains of fatal cases. The work which has been done has largely been concerned with the distribution of clots and macroscopic infarcts (Robertson, 1949; Wilson, Riggs, and Rupp, 1954; Crompton, 1962). Tomlinson (1959) comments on ischaemic lesions observed macroscopically and histologically but only in the territory of the aneurysm-bearing vessel. Robertson (1949) and Birse and Tom (1960) mention cases in which ischaemic changes were found outside that territory. The present study is confined to the brains of patients dying from the consequence of subarachnoid haemorrhage without significant cerebral disruption or intracerebral clot. Of 30 brains examined macroscopically, only eight fulfilled these criteria, three from patients who were not operated on and five from patients who had undergone various surgical procedures. The brains were investigated by embedding large blocks in low-viscosity nitrocellulose, nine blocks being taken from each brain.

Trigeminal root and ganglion injections using phenol in glycerine for the relief of trigeminal neuralgia.

Abstract: By no means all those responsible for the treatment of severe trigeminal neuralgia view trigeminal root or ganglion injections with favour. Their objections are based on the difficulty of precisely placing the needle point (with consequent uncertainty of a successful result) and on real or imagined dangers. The results of trigeminal root and ganglion injections described here have been obtained using phenol dissolved in glycerine. This agent was introduced by Maher (1955) for injection in the spinal canal, and its use relieved pain with only partial sensory loss. To control trigeminal neuralgia without producing total numbness of the affected areas of the face would be of undoubted advantage to the patient. Those who have used the Hartel approach (Hartel, 1912) for intracranial trigeminal injections know that the needle may enter Meckel's cave without impaling the third division of the trigeminal nerve, or its ganglion, or its posterior root. Under these circumstances small quantities of alcohol, e.g., < 0-2 ml., may prove ineffective. By using Maher's agent with the patient in the upright position it was envisaged that the heavy solution of phenol in glycerine could be made to flow over the ganglion and to bathe the posterior root within Meckel's cave. In this way it was hoped to create a satisfactory neurological lesion without having to place the needle point as precisely as when alcohol is the medium to be injected. The extent to which reality has accorded with these hopes is set out below.

Seizures induced by movement: report of a case relieved by operation

Abstract: Under the title 'seizures induced by movement' Lishman, Symonds, Whitty, and Willison (1962) have recently reported seven cases of a type of epilepsy which they consider rare. These seizures present a fairly uniform pattern, and are characterized by a sudden tonic spasm which may involve the whole body, but more often is localized to one limb or one side of the body. There are no clonic movements or loss of consciousness. Sometimes the movements take an athetoid form. Such seizures usually begin in childhood, last for a few seconds to a minute, but may recur frequently, up to several times daily. They are generally precipitated by sudden movement, and are more likely to occur after a period of rest. Movements of the legs initiate them more often than do movements of the arms, and startle sometimes seems important. Often a state of emotional tension or anxiety precedes them, while conversely mental relaxation or the slow initiation of movement tend to reduce them. Often also a sensory aura briefly precedes the spasm. Sometimes attacks can be voluntarily induced, provided the provoking movement is abrupt and forceful. All the authors' reported cases were males. Lishman et al. (1962) could find only two other cases reported in the literature in sufficient detail to be included in their group (Pitha, 1938; Michaux and Granier, 1945), although Gowers (1901) had earlier discussed three cases in which 'attacks can be brought on by voluntary motion'. They therefore included these two cases in their analysis, and argued that the attacks were epileptic. In addition to the clinical evidence, they pointed out that there are often E.E.G. abnormalities either in the resting record or on overbreathing, when the abnormalities take the form of a build-up of paroxysmal slow activity and/or the emergence of spikes. In six of their seven personal cases anticonvulsant drugs reduced the frequency and severity of attacks and in one abolished them. Lishman et al. could not decide where these attacks arose and speculated whether they could result from some lesion or dysfunction in the basal ganglia. None of their cases was submitted to surgical exploration. We therefore wish to report a case which on clinical and E.E.G. grounds conformed to the seizure pattern described above; the patient appears to have been completely relieved by removal of a cicatrix situated on the superior border of one hemisphere immediately in front of the primary motor cortex.

A perceptual maze test sensitive to brain damage.

Abstract: Following a report (Elithorn, 1955) that performance on a perceptual maze was sensitive to changes due to age and to small focal cerebral lesions, one of us (A.L.B.) decided to include this test in an extensive battery of psychological tests which were given to a group of brain-damaged patients and a comparable group of control subjects. The present paper reports the findings with this test and records briefly the relationships of these findings to the results obtained with the other tests. Some of the latter results have been reported in previous papers (e.g., Bechtoldt, Benton, and Fogel, 1962; Benton, 1962; Benton and Fogel, 1962).

The radiological appearances of spinal extradural arachnoid cysts.

Abstract: Of the earlier clinical descriptions of this condition, probably the most often quoted is that by Elsberg, Dyke, and Brewer (1934): 'The individual is an adolescent with the history of symptoms of a progressive spastic paraplegia. Pain is absent or not a prominent symptom. The objective disturbances of sensibility are slight and their upper level is in the mid-thoracic dermatome. The manometric tests demonstrate a subarachnoid block with the characteristic spinal fluid changes of cord compression. Measurements on A-P x-ray films show that the interpedicular spaces of three or more vertebrae are enlarged. The pedicles of the affected vertebrae, especially those of the sixth, seventh, and eighth, are narrowed and atrophic'. The authors go on to say that these cysts are due to congenital diverticula of the dura or to a herniation of the arachnoid through a congenital defect in the dura. Cloward and Bucy (1937) added another component to this syndrome by drawing attention to the frequent association of kyphosis dorsalis juvenilis (Scheurmann's disease) with spinal extradural cysts. According to them the changes seen in the vertebral bodies were due to venous stasis and congestion, produced by the cyst occluding the large posterior central draining vein. Tumbull (1939) and others have remarked upon the favourable effects of immobility, either in bed or in a plaster jacket, and the later recurrence of symptoms with activity. Turner (1947) reported striking improvement after removal of the spinal fluid in the power of the lower extremities in an 11-year-old boy, and suggested that this could be taken as a possible diagnostic aid, for, in all other forms of spinal compression one would instead expect deterioration or no change after such a measure. Wise and Foster (1955) discussed in detail all these features and reviewed 34 cases that they were able to gather from recorded material, adding one of their own. In the present paper three cases are described and one observation common to all three offered as another diagnostic aid, namely, the presence in antero-posterior radiographs of rounded paravertebral shadows caused by the lateral extensions of these cysts, a feature which to my knowledge has so far not even been mentioned.

The morphology of Marinesco bodies (paranucleolar corpuscles) in the melanin-pigmented nuclei of the brain-stem

Abstract: In 1902 Marinesco described the presence of spherical acidophilic bodies in the nucleoplasm of the cells of the human nucleus substantia nigra and the nucleus locus caeruleus. He wrote: 'The number of these corpuscles vary from one to six, and generally there are one, two or often three. When they are numerous we find them heaped in groups in the nuclear sap, and the position they occupy with regard to the nucleolus is equally variable. Sometimes they are situated in the neighbourhood of the latter, sometimes they are set aside and can even reside at the two poles of the nucleus. As to their relative sizes, they are seldom larger than the nucleolus, sometimes they can be nearly equal, but generally they are smaller than the latter, especially when they are numerous. The diameter of these paranucleolar corpuscles can reach 7,u'. 'The size of these corpuscles does not appear to be related with age, for the biggest diameter which we found was in a 30-year-old man. Generally speaking, they are more numerous in adults and old people than young persons. In the latter, in fact, they are rarely found and are absent in children.' Marinesco elaborated further on the staining properties of these paranucleolar corpuscles in 1909. Remarkably little attention has been paid to them, at least in the British literature, since that time. Greenfield (1958) and Lipkin (1959) refer briefly to them, and Wolf and Orton (1932) have described similar intranuclear inclusions in unpigmented cells of the central nervous system in diverse disease states. This paucity of interest is all the more surprising in view of the widespread interest in cytoplasmic inclusions in the cells of the substantia nigra and locus caeruleus in paralysis agitans and other extrapyramidal syndromes. The aim of the present study was to determine the frequency with which Marinesco bodies could be found and whether their presence could be correlated with specific or general types of disease or with the age of the patient. We also sought to determine further the histochemical properties of the inclusions and any relationship which the inclusions might have to melanin pigmentation.