Conversion disorder (‘hysteria’) was largely considered to be a neurological problem in the 19th century, but without a neuropathological explanation it was commonly assimilated with malingering. The theories of Janet and Freud transformed hysteria into a psychiatric condition, but as such models decline in popularity and a neurobiology of conversion has yet to be found, today's neurologists once again face a disorder without an accepted model. This article explores how today's neurologists understand conversion through in-depth interviews with 22 neurology consultants. The neurologists endorsed psychological models but did not understand their patients in such terms. Rather, they distinguished conversion from other unexplained conditions clinically by its severity and inconsistency. While many did not see this as clearly distinct from feigning, they did not feel that this was their problem to resolve. They saw themselves as ‘agnostic’ regarding non-neuropathological explanations. However, since neurologists are in some ways more expert in conversion than psychiatrists, their continuing support for the deception model is important, and begs an explanation. One reason for the model's persistence may be that it is employed as a diagnostic device, used to differentiate between those unexplained symptoms that could, in principle, have a medical explanation and those that could not.
conversion disorder; hysteria; malingering; deception; factitious disorder
Neurologists face a dilemma when communicating with their conversion disorder patients – whether to be frank, and risk losing the patient's trust, or to disclose less, in the hope of building a therapeutic relationship. This study reports how neurologists in the UK described dealing with this dilemma in their practice.
Practicing consultant neurologists from an NHS region were recruited by snowball sampling. Twenty-two of 35 consultants in the region were interviewed in depth, and the interviews qualitatively analysed.
The neurologists were reluctant to disclose conversion disorder as a differential diagnosis until they were certain. They were guided by the receptivity of their patients as to how psychological to make their eventual explanations, but they did not discuss their suspicions about feigning. They described their communications as much easier now than they had seen in training.
Neurologists adapt their disclosure to their patients, which facilitates communication, but imposes some limits on truth-telling. In particular, it may sometimes result in a changed diagnosis.
An optimum strategy for communicating diagnoses will need to balance ethical considerations with demonstrated therapeutic benefit.
Conversion disorder; Factitious disorder; Malingering; Hysteria; Truth-telling; Deception; Neurology
Monetary incentives are an effective way of increasing response rates to surveys, though they are generally less effective in physicians, and are more effective when the incentive is paid up-front rather than when made conditional on completion.
In this study we examine the effectiveness of pre- and post-completion incentives on the response rates of all the neurologists in the UK to a survey about conversion disorder, using a cluster randomised controlled design. A postal survey was sent to all practicing consultant neurologists, in two rounds, including either a book token, the promise of a book token, or nothing at all.
Three hundred and fifty-one of 591 eligible neurologists completed the survey, for a response rate of 59%. While the post-completion incentive exerted no discernible influence on response rates, a pre-completion incentive did, with an odds-ratio of 2.1 (95% confidence interval 1.5 - 3.0).
We conclude that neurologists, in the UK at least, may be influenced to respond to a postal survey by a pre-payment incentive but are unaffected by a promised reward.
The National Institute for Clinical Excellence in the UK has issued guidelines stating all individuals with epilepsy be given information about sudden unexpected death in epilepsy (SUDEP).
We conducted a survey of current practice among UK neurologists, using a questionnaire sent to all practising neurologists in the UK listed on the Association of British Neurologists database, asking under what circumstances they told patients about SUDEP.
Of the validated respondents, 5% discussed SUDEP with all patients, 26% with a majority, 61% with a few, and 7.5% with none. The commonest reasons for SUDEP to be discussed were the patient asking about it and the neurologist counselling people with known risk factors for SUDEP.
The variation we found, although not necessarily in tune with the guidelines, reflects the variation in patients' need for knowledge about their condition.
Understanding the roles and responsibilities of physicians who manage mutual patients is important for assuring good patient care. Among physicians expressing a preference to involve a neurologist in the care of a patient, we evaluated agreement between neurologists and primary care physicians for the extent of specialty involvement in the evaluation and management of the patient, and the factors influencing those preferences.
DESIGN AND SETTING
A self-administered survey containing 3 clinical scenarios was developed with the assistance of a multispecialty advisory board and mailed to a stratified probability sample of physicians.
Six hundred and eight family physicians, 624 general internists, and 492 neurologists in 9 U.S. states.
For each scenario, those respondents who preferred involvement of a specialist were asked about the preferred extent of that involvement: one-time consultation with and without test/medication ordering, consultation and limited follow-up, or taking over ongoing care of the specialty problem as long as it persists.
Survey response rate was 60%. For all 3 scenarios, neurologists preferred a greater extent of specialty involvement compared to primary care physicians (all P <.05). Other physician and practice characteristic factors, including financial incentives, had lesser or no influence on the extent of specialty involvement preferred.
The disagreement between primary care physicians and specialists regarding the preferred extent of specialist involvement in the care of patients with neurological conditions should raise serious concerns among health care providers, policy makers, and educators about whether mutual patient care is coordinated and appropriate.
referral and consultation; physician's practice patterns
The prevalence of psychiatric morbidity in inpatients with neurological disorders and the extent to which it is detected by neurologists were measured by using a two stage model of psychiatric assessment and from information recorded in the patients' medical notes. The prevalence of psychiatric morbidity was estimated as 39%, of which 72% was unrecognised by the neurologists. Only a minority of patients with an uncertain physical diagnosis had a psychiatric illness, showing the error in assuming that a patient's physical symptoms arise from a psychological disturbance if an organic aetiology cannot be determined. When the patients were interviewed on their discharge from hospital they were divided on whether they had wished to discuss their mood with neurologists while they were in hospital. The reasons that they gave suggested that interactions between patients and doctors and the lack of ward facilities for private consultations with doctors are important determinants of hidden psychiatric morbidity in medical inpatients.
The term ‘functional’ has a distinguished history, embodying a number of physiological concepts, but has increasingly come to mean ‘hysterical’. The DSM-V working group proposes to use ‘functional’ as the official diagnostic term for medically unexplained neurological symptoms (currently known as ‘conversion disorder’). This study aimed to explore the current neurological meanings of the term and to understand its resilience.
Mixed methods were used, first interviewing the neurologists in a large UK region and then surveying all neurologists in the UK on their use of the term.
The interviews revealed four dominant uses—‘not organic’, a physical disability, a brain disorder and a psychiatric problem—as well as considerable ambiguity. Although there was much dissatisfaction with the term, the ambiguity was also seen as useful when engaging with patients. The survey confirmed these findings, with a majority adhering to a strict interpretation of ‘functional’ to mean only ‘not organic’, but a minority employing it to mean different things in different contexts - and endorsing the view that ‘functional’ would one day be a neurological construct again.
‘Functional’ embodies real divisions in neurologists' conceptualisation of unexplained symptoms and, perhaps, between those of patients and neurologists: its diversity of meanings allows it to be a common term while meaning different things to different people, or at different times, and thus conceal some of the conflict in a particularly contentious area. This flexibility may help explain the term's longevity.
the use of corticosteroids in multiple sclerosis as recommended by
United Kingdom consultant neurologists.
questionnaire covering the use of corticosteroids for acute multiple
sclerosis relapse and chronic progressive multiple sclerosis with
regard to frequency of use, type of corticosteroid, and dosage regime
was sent to all members of the Association of British Neurologists with
a United Kingdom address.
and twelve United Kingdom consultant neurologists replied to the survey
(74% response rate). Eighty six per cent indicated that they
prescribed corticosteroids in more than one quarter of acute multiple
sclerosis relapses seen. Intravenous methylprednisolone was recommended
at some time by 99% of consultant neurologists, the most popular
regime being 1g daily for 3 days (74%; 154/208). Over one half (53%;
109/206) never recommended a subsequent tapering course of oral
corticosteroids; of those that did, 25% (24/96) recommended a tapering
course lasting more than 1 month. Eighty eight per cent (181/206) of
prescribers of intravenous methylprednisolone were able to offer the
course as a day case on the ward; 7% (15/206) at an outpatient clinic;
and 5% (11/206) at home. Almost three quarters of neurologists
recommended oral corticosteroids for some acute relapses, although the
most popular response was for occasional use only (48%; 101/212).
Forty five per cent (96/211) at least occasionally recommended steroids for patients with chronic multiple sclerosis not experiencing an acute relapse.
the vast majority of consultant neurologists would prescribe
intravenous methylprednisolone for acute multiple sclerosis relapse at
some time, the use of corticosteroids for multiple sclerosis was
otherwise variable. There seemed to be little consensus about the use
of oral steroids in acute relapse, the prescribing of a tapering course
of oral steroids after intravenous methylprednisolone, or the utility
of steroids in chronic multiple sclerosis. Variability of prescribing
recommendations probably reflects a lack of clear evidence in the face
of a wide range of clinical situations, variable access, and timing of
access to neurologists in the acute phase of relapse and pressure on
neurologists to treat in an otherwise "hopeless" situation. Large
multicentred trials are needed to consider these issues.
Clinical neurologists in the health care system of the future should have a multifaceted role. Advances in the basic understanding of the nervous system and therapeutics of neurologic disease have created, for the first time in human history, an ethical imperative to correctly diagnose neurologic disease. In many situations, the neurologists may function as a consultant and principal physician for patients with primary nervous system disorders including Parkinson's disease, multiple sclerosis, Alzheimer's disease, epilepsy, migraine, cerebrovascular disease, movement disorders, and neuromuscular disease. Other important roles for neurologists include the training of future physicians, both neurologists and primary care physicians, the application of cost-effective approaches to care, and the support of health care delivery research and academic programs that link basic research efforts to the development of new therapy. To be successful, future residency training programs should include joint certification opportunities in both neurology and general medicine, and training programs for clinical investigators should be expanded. Despite its threats to specialists, managed care should also provide opportunities for new alliances among neurologists, other specialists, and primary care physicians that will both improve patient care and increase efficiency and cost-effectiveness.
Fibromyalgia (FM) is a condition characterized by widespread pain and is estimated to affect 0.5-5% of the general population. Historically, it has been classified as a rheumatologic disorder, but patients consult physicians from a variety of specialties in seeking diagnosis and ultimately treatment. Patients report considerable delay in receiving a diagnosis after initial presentation, suggesting diagnosis and management of FM might be a challenge to physicians.
A questionnaire survey of 1622 physicians in six European countries, Mexico and South Korea was conducted. Specialties surveyed included primary care physicians (PCPs; n=809) and equal numbers of rheumatologists, neurologists, psychiatrists and pain specialists.
The sample included experienced doctors, with an expected clinical caseload for their specialty. Most (>80%) had seen a patient with FM in the last 2 years. Overall, 53% of physicians reported difficulty with diagnosing FM, 54% reported their training in FM was inadequate, and 32% considered themselves not knowledgeable about FM. Awareness of American College of Rheumatology classification criteria ranged from 32% for psychiatrists to 83% for rheumatologists. Sixty-four percent agreed patients found it difficult to communicate FM symptoms, and 79% said they needed to spend more time to identify FM. Thirty-eight percent were not confident in recognizing the symptoms of FM, and 48% were not confident in differentiating FM from conditions with similar symptoms. Thirty-seven percent were not confident developing an FM treatment plan, and 37% were not confident managing FM patients long-term. In general, rheumatologists reported least difficulties/greatest confidence, and PCPs and psychiatrists reported greatest difficulties/least confidence.
Diagnosis and managing FM is challenging for physicians, especially PCPs and psychiatrists, but other specialties, including rheumatologists, also express difficulties. Improved training in FM and initiatives to improve patient-doctor communication are needed and may help the management of this condition.
Although small treatment trials have examined the efficacy of front-line medications in tremor reduction in essential tremor (ET), an overall survey of pharmacological management has not been undertaken in a large sample of ET cases.
To conduct a survey of medication usage among several hundred ET patients.
A computerized database at the Neurological Institute of New York was used to identify 223 ET patients cared for by movement disorder neurologists and 37 cared for by general neurologists. Each had been seen one or more times within past five years. Lifetime treatment data were abstracted from clinical records.
One-hundred-sixty-three (73.1%) of 223 patients cared for by movement disorder neurologists had taken medication for ET during their lifetime; 53/163 (32.5%) had taken ≥4 medications; 31/163 (19.0%) had taken ≥5 medications; and 3 (1.8%), ≥10 medications. Nearly three-quarters (158/223 [70.9%]) had taken primidone or propranolol, yet 89/158 (56.3%) had discontinued both. Among 11 patients who had undergone ET surgery, the mean number of ET medications tried was 6.0±2.8 (range = 3–12). Medication usage by general neurologists was less than by movement disorder specialists (p<0.001). One-third of general neurologists’ patients (14/37 [37.8%]) had taken primidone or propranolol at some point and 6/14 (42.9%) had discontinued both.
In this large survey, a substantial proportion of ET patients failed treatment with both front-line medications. Among patients seeing movement disorder specialists, the number of medications they had had to try was sizable. These data demonstrate the limitations of current pharmacological options for ET.
Essential tremor; clinical; epidemiology; treatment; medication
Although Parkinson's disease is primarily a neurologic disorder, primary care physicians should be knowledgeable about the disease and its treatment because most patients will see their primary care physician first for their symptoms. Furthermore, in today's setting of managed care, primary care physicians will likely be called on even more to assume primary responsibility for the treatment of patients afflicted with Parkinson's disease; neurologists will likely play the role of consultants who see a patient only periodically and offer recommendations and advice for the primary care physicians to implement.
Between 10 and 30% of patients seen by neurologists have symptoms for which there is no current pathophysiological explanation. The objective of this review is to answer questions many neurologists have about disorders characterised by unexplained symptoms (functional disorders) by conducting a multidisciplinary review based on published reports and clinical experience. Current concepts explain functional symptoms as resulting from auto-suggestion, innate coping styles, disorders of volition or attention. Predisposing, precipitating, and perpetuating aetiological factors can be identified and contribute to a therapeutic formulation. The sympathetic communication of the diagnosis by the neurologist is important and all patients should be screened for psychiatric or psychological symptoms because up to two thirds have symptomatic psychiatric comorbidity. Treatment programmes are likely to be most successful if there is close collaboration between neurologists, (liaison) psychiatrists, psychologists, and general practitioners. Long term, symptoms persist in over 50% of patients and many patients remain dependent on financial help from the government. Neurologists can acquire the skills needed to engage patients in psychological treatment but would benefit from closer working relationships with liaison psychiatry or psychology.
A case analysis has been performed on 4000 successive outpatient referrals to one consultant neurologist, representing 72% of all referrals to Charing Cross Hospital and 82% to Hillingdon Hospital. A specific diagnosis was not possible in 1013 patients (25.3%). Amongst the remainder certain diagnoses were predictably common: for example, migraine (241), tension headaches (296) and epilepsy (470). Based on data obtained for the incidence of various neurological disorders in the community, an attempt has been made to assess what proportion of patients with certain diagnoses are likely to be seen by a neurologist. The rarity with which certain classical conditions, for example syringomyelia, is encountered is stressed and the implications for teaching discussed.
Diagnosis and decisions on life-sustaining treatment (LST) in disorders of consciousness, such as the vegetative state (VS) and the minimally conscious state (MCS), are challenging for neurologists. The locked-in syndrome (LiS) is sometimes confounded with these disorders by less experienced physicians. We aimed to investigate (1) the application of diagnostic knowledge, (2) attitudes concerning limitations of LST, and (3) further challenging aspects in the care of patients. A vignette-based online survey with a randomized presentation of a VS, MCS, or LiS case scenario was conducted among members of the German Society for Neurology. A sample of 503 neurologists participated (response rate 16.4%). An accurate diagnosis was given by 86% of the participants. The LiS case was diagnosed more accurately (94%) than the VS case (79%) and the MCS case (87%, p < 0.001). Limiting LST for the patient was considered by 92, 91, and 84% of the participants who accurately diagnosed the VS, LiS, and MCS case (p = 0.09). Overall, most participants agreed with limiting cardiopulmonary resuscitation; a minority considered limiting artificial nutrition and hydration. Neurologists regarded the estimation of the prognosis and determination of the patients’ wishes as most challenging. The majority of German neurologists accurately applied the diagnostic categories VS, MCS, and LiS to case vignettes. Their attitudes were mostly in favor of limiting life-sustaining treatment and slightly differed for MCS as compared to VS and LiS. Attitudes toward LST strongly differed according to circumstances (e.g., patient’s will opposed treatment) and treatment measures.
End-of-life decisions; Limitation of life-sustaining treatment; Diagnosis; Vegetative state; Minimally conscious state
New more powerful therapies for the treatment of multiple sclerosis may also confer a potential for unprecedented life-endangering side effects. How does a physician respond to a patient’s request for a treatment the benefit of which cannot be clearly established as worth its risk? The current challenge with prescription of natalizumab (Tysabri®, Biogen Idec) is used to illustrate how this conflict creates an opportunity to re-examine our goals as physicians and the nature of the physician–patient relationship. Understanding the physician’s role in that partnership, and the ethical and psychological issues impacting on how reasonable risk is determined, can improve the neurologist’s capacity to explicate such quandaries. Redefining what is required to mediate disagreement between doctors and patients about reasonable risk is at the heart of why many of us became physicians. However, such nuanced interpersonal dynamics of patient care can be neglected due to the time and resource pressures of our practices. These demands have increased the seductiveness of the efficiencies promoted by the trend toward the pseudo-objectification of evidence-based care, which has arguably monopolized the healing conversation often to the detriment of the shared narrative. We examine and attempt to reframe the fiduciary and biopsychosocial contretemps of the doctor and patient disagreeing on risk, emphasizing its humanistic, relational dimensions.
multiple sclerosis; natalizumab; medical ethics; medical decision-making; patient-physician relationship
Visual complaints without a physical basis are not uncommon presentations to the general physician, the neurologist, or the ophthalmologist. These alleged visual disturbances may be psychogenic or feigned. The diagnosis is made when all possible contributory pathology of the visual system is excluded, and reassurance remains the cornerstone of management.
Keywords: blindness; electroretinography; evoked potentials; psychogenic illness
Rapidly progressive dementia (RPD) is a unique set of disorders resulting in cognitive, behavioral, and motor decline within 2 years. A variety of etiologies may contribute to RPD including neurodegenerative, inflammatory, infectious, and toxic-metabolic conditions. Jakob-Creutzfeldt disease (CJD) is frequently the most concerning diagnosis on the differential. The challenge for the neurologist is distinguishing prion disease from reversible processes that result in dementia.
This review discusses the clinical aspects and the diagnostic work-up of RPD. Particular focus is given to both CJD and the potentially treatable inflammatory conditions that may cause a similar presentation. Furthermore, a standardized step-wise approach is outlined for patients presenting with RPD.
Neurologists should adopt a standardized approach to the rapidly presenting disease processes that may mimic CJD in their clinical and radiological features.
Rapidly progressive dementia; Jakob-Creutzfeldt disease; Immune-mediated dementia; Paraneoplastic syndrome; Hashimoto’s Encephalopathy
The purpose of this study was to identify differences in perceptions of care regarding postherpetic neuralgia, including communication patterns between patients and physicians and levels of satisfaction with therapies and care.
A survey was developed for physicians (neurologists, internists, and family physicians) and patients with postherpetic neuralgia in order to determine their perspectives on its management.
A total of 142 eligible patient respondents were included in the study, and responses were compared with those of 150 primary care physicians and 76 neurologist respondents. Few patients and physicians indicated satisfaction with the currently available treatments for postherpetic neuralgia. While nearly all physicians responded that they discuss the cause of postherpetic neuralgia with a patient, one in four patients indicated that their physician did not discuss the cause. Similarly, one in four patients were not aware of the duration of postherpetic neuralgia, the treatment side effects, or what to expect from treatment. Patients may be less likely to discuss treatment side effects and quality of life issues than physicians perceive.
Physicians and patients have similar perceptions regarding treatment options for postherpetic neuralgia; however, certain gaps in communication were evident, which may be attributable to physician knowledge and communication skills with patients. Strategies to improve issues of expected outcomes and side effects of treatment may be useful to physicians.
postherpetic neuralgia; pain; patient communication; case vignette
Background and Purpose: Intra-arterial reperfusion therapies are expanding frontiers in acute ischemic stroke (AIS) management but there is considerable variability in clinical practice. The use of general anesthesia (GA) is one example. We aimed to better understand sedation practices in AIS. Methods: An online survey was distributed to the 68 active members of the Society of Vascular and Interventional Neurology (SVIN). Survey development was based on discussions at the SVIN Endovascular Stroke Round Table Meeting (Chicago, IL, 2008). The final survey contained 12 questions. Questions were developed as single and multiple-item responses; with an option for a free-text response. Results: There was a 72% survey response rate (N = 49/68). Respondents were interventional neurologists in practice 1–5 years (71.4%, N = 35). The mean (±SD) AIS interventions performed per year at the respondents’ institutions was 42.5 ± 25, median 35.0 (IQR 20, 60). The most frequent anesthesia type used was GA (anesthesia team), then conscious sedation (nurse administered), monitored anesthesia care (anesthesia team), and finally local analgesia alone. There was a preference for GA because of eliminating movement (65.3% of respondents; N = 32/49), perceived procedural safety (59.2%, N = 29/49), and improved procedural efficacy (42.9%, N = 21/49). However, cited limitations to GA included risk of time delay (69.4%, N = 34), of propagating cerebral ischemia due to hypoperfusion or other complications (28.6%, N = 14), and lack of adequate anesthesia workforce (20.4%, N = 7). Conclusions: The most frequent type of anesthesia used by Neurointerventionalists for AIS interventions is GA. Prior to making GA standard of care during AIS intervention, more data are needed about effects on clinical outcomes.
anesthesia; acute ischemic stroke; endovascular; intra-arterial; neurointerventional
Conversion disorder has a history that may reach back into antiquity, and it continues to present a clinical challenge to both psychiatrists and neurologists. This article reviews the current state of knowledge surrounding the prevalence, etiology, and neurobiology of conversion disorder. There have been improvements in the accuracy of diagnosis that are possibly related to improved technologies such as neuroimaging. Once the diagnosis is made, it is important to develop a therapeutic alliance between the patient and the medical team, and where comorbid psychiatric diagnoses have been made, these need to be adequately treated. While there have been no formal trials of medication or psychoanalytic treatments in this disorder, case reports suggest that a combination of antidepressants, psychotherapy, and a multidisciplinary approach to rehabilitation may be beneficial.
conversion disorder; hysteria; diagnosis
Attempts to determine the ideal number of consultant neurologists that will be required in the United Kingdom in the future are hampered by a lack of information on a variety of topics, one of which concerns the workload of the average neurologist at the present time. This paper attempts to correct this deficiency by examining the clinical workload of a single handed neurologist practising in the south west of England. Diagnostic information is given on the 3020 new patients seen during 1984-1986 and is compared with similar data on 836 new patients seen in 1975. The pattern of diagnoses on these patients varies little from year to year, indicating a constancy of referral habit of those who seek neurological advice. However, the referral rates for different conditions do not correspond with what would be expected from epidemiological data, for when the incidence of particular conditions in the neurology clinic is compared with the calculated incidence in the community, very wide variations are noted. The implications of these data are discussed and it is suggested that further studies should be performed before detailed predictions are made on how many neurologists will be needed in this country in the future.
Headache is the neurological symptom most frequently presented to GPs and referred to neurologists, but little is known about how referred patients differ from patients managed by GPs.
To describe and compare headache patients managed in primary care with those referred to neurologists.
Design of study
Eighteen general practices in south-east England.
This study examined 488 eligible patients consulting GPs with primary headache over 7 weeks and 81 patients referred to neurologists over 1 year. Headache disability was measured by the Migraine Disability Assessment Score, headache impact by the Headache Impact Test, emotional distress by the Hospital Anxiety and Depression Scale and illness perception was assessed using the Illness Perception Questionnaire.
Participants were 303 patients who agreed to participate. Both groups reported severe disability and very severe impact on functioning. Referred patients consulted more frequently than those not referred in the 3 months before referral (P = 0.003). There was no significant difference between GP-managed and referred groups in mean headache disability, impact, anxiety, depression, or satisfaction with care. The referred group were more likely to link an increased number of symptoms to their headaches (P = 0.01), to have stronger emotional representations of their headaches (P = 0.006), to worry more (P = 0.001), and were made anxious by their headache symptoms (P = 0.044).
Patients who consult for headache experience severe disability and impact, and up to a third report anxiety and/or depression. Referral is not related to clinical severity of headaches, but is associated with higher consultation frequency and patients' anxiety and concern about their headache symptoms.
headache; migraine disorders; neurology; primary health care; referral and consultation
Since the description of athetosis in 1871 by American neurologist William Alexander Hammond (1828–1900) the disorder has been a source of controversy, as were many aspects of Hammond’s career.
Primary sources have been used to review controversies in the 50-year period since the initial description of athetosis, in particular those concerning clinical features, differentiation from other movement disorders, associated conditions, and pathology. Controversies concerning treatment will be addressed in a subsequent article.
Hammond struggled to establish athetosis as a distinct clinical–pathological entity, and had successfully predicted the striatal pathology in his initial case (albeit somewhat serendipitously). Athetosis was, nevertheless, considered by many neurologists to be a form of post-hemiplegic chorea or part of a continuum between chorea and dystonia. European neurologists, and particularly the French, initially ignored or discounted the concept. Additional controversies arose over whether the movements persisted during sleep, whether athetosis was, or could be, associated with imbecility or insanity, and how it should be treated.
Some controversies concerning athetosis served to identify areas where knowledge was insufficient to make accurate statements, despite prior self-assured or even dogmatic statements to the contrary. Other controversies illustrated established prejudices, even if these biases were often only apparent with the greater detachment of hindsight.
History of neurology; nineteenth century; history of neurology; twentieth century; athetosis; dystonia; chorea; diagnosis; pathology; sleep-related movement disorders; mental retardation; dementia
Neurologists often diagnose brain death (BD) and explain BD to families in the intensive care unit. This study was designed to determine whether neurologists agree with the standard concept of death (irreversible loss of integrative unity of the organism) and understand the state of the brain when BD is diagnosed.
A previously validated survey was mailed to a random sample of 500 board-certified neurologists in the United States. Main outcomes were: responses indicating the concept of death that BD fulfills and the empirical state of the brain that would rule out BD.
After the second mailing, 218 (44%) surveys were returned. Few (n = 52, 27%; 95% confidence interval (CI), 21%, 34%) responded that BD is death because the organism has lost integrative unity. The most common justification was a higher brain concept (n = 93, 48%; 95% CI, 41%, 55%), suggesting that irreversible loss of consciousness is death. Contrary to the recent President's Council on Bioethics, few (n = 22, 12%; 95% CI, 8%, 17%) responded that the irreversible lack of vital work of an organism is a concept of death that the BD criterion may satisfy. Many responded that certain brain functions remaining are not compatible with a diagnosis of BD, including EEG activity, evoked potential activity, and hypothalamic neuroendocrine function. Many also responded that brain blood flow and lack of brainstem destruction are not compatible with a diagnosis of BD.
American neurologists do not have a consistent rationale for accepting BD as death, nor a clear understanding of diagnostic tests for BD.