Daniel Shewmon

• UCLA

Discrepancies between the Uniform Determination of Death Act (UDDA) and the adult and pediatric diagnostic guidelines for brain death (BD) (the “Guidelines”) have motivated proposals to revise the UDDA. A revision proposed by Lewis, Bonnie and Pope (the RUDDA), has received particular attention, the three novelties of which would be: (1) to specify...

From the start, I followed the case of Jahi McMath with great interest. In December 2013, she clearly fulfilled the diagnostic criteria for brain death. As a neurologist with a special interest in chronic brain death, I was not surprised that, after she was flown to New Jersey, where she became statutorily resurrected and was treated as a comatose...

At its inception, “brain death” was proposed not as a coherent concept but as a useful one. The 1968 Ad Hoc Committee of the Harvard Medical School to Examine the Definition of Brain Death gave no reason that “irreversible coma” should be death itself, but simply asserted that the time had come for it to be declared so. Subsequent writings by chair...

This article clarifies some issues raised by Dr. Ariane Lewis in her recent “Current Opinion/Arguments” article on the case of Jahi McMath. Review of case materials. Jahi’s case most likely represents an instance of global ischemic penumbra (GIP) mimicking brain death (BD), with intracranial blood flow too low to support neuronal function or to be...

A 2-year-old boy with severe head trauma was diagnosed brain dead according to the 2011 Pediatric Guidelines. Computed tomographic (CT) scan showed massive cerebral edema with herniation. Intracranial pressures were extremely high, with cerebral perfusion pressures around 0 for several hours. An apnea test was initially contraindicated; later, one...

In debates about criteria for human death, several camps have emerged, the main two focusing on either loss of the "organism as a whole" (the mainstream view) or loss of consciousness or "personhood." Controversies also rage over the proper definition of "irreversible" in criteria for death. The situation is reminiscent of the proverbial blind men...

A 17-year-old male presented with sudden onset of persistent focal neurological symptoms. Magnetic resonance imaging (MRI) demonstrated a nonenhancing white matter lesion that appeared hyperintense on fluid-attenuated inversion recovery sequence and diffusion-weighted imaging, while hypointense on apparent diffusion coefficient mapping correspondin...

Why is a patient with a destroyed brain considered dead rather than moribund and irreversibly comatose? The world has been grappling with this question for the past four decades with little success. The recently released white paper of the President's Council on Bioethics is in many respects a refreshing, thoughtful, and comprehensive reexamination...

Alan Rubenstein highlights the crux of the debate over "brain death" or "total brain failure." It boils down to whether the original term "coma dépassé" represented an insight or an exaggeration; also to whether the "uniqueness" of total brain failure is anything more than that of any extreme of a spectrum. Total brain failure's supposed uniqueness...

American society traditionally has assumed a univocal notion of "death," largely because we have only one word for it and, until recently, have not needed a more nuanced notion. The reality of death-processes does not preclude the reality of death events. Linguistically, "death" can be understood only as an event; there are other words for the proc...

Neurophysiological research on the vegetative state (VS) presupposes a clearly defined patient population. VS has been variously defined in three domains: anatomy, behavior, and consciousness. Research on each conceptual type of "VS" is reviewed. Certain key elements of official VS dogma are cast into doubt or flatly contradicted by recent noninvas...

In any discussion pertaining to this world, human beings are bound to the medium of language. We are often unaware of the degree to which language, as the physical shape of all thought, exerts its influence on the distinctions we make and consequently on the formation of our notions and ideas. The way we think shapes the way we speak, but also conv...

Although some decades have passed, there are still worldwide controversies about a concept of human death on neurological grounds. There are also disagreements on the diagnostic criteria for brain death, whether clinical alone or clinical plus ancillary tests. Moreover, some scholars who were strong defenders of a brain-based standard of death are...

For over three decades now, it has been widely accepted that the death of one particular organ — the brain — constitutes human death. Much less a matter for agreement, however, has been the reason for that equivalence. The various proposed rationales can be subdivided into three main categories, corresponding to three fundamentally different concep...

A prerequisite for any scientific discussion or study is a clear definition of the matter under consideration. With few topics is this both more pertinent and less heeded than with persistent vegetative state (PVS). The more one studies the literature on PVS, the less clear an entity it becomes. Multiple quasi-official definitions have been propose...

Objective: To establish consensus recommendations among health care specialties for defining and establishing diagnostic criteria for the minimally conscious state (MCS). Background: There is a subgroup of patients with severe alteration in consciousness who do not meet diagnostic criteria for coma or the vegetative state (VS). These patients demon...

Prediction of outcome from coma is a frequent and important task of neurologists. It is difficult enough in adult patients and even more difficult in children. Part I of this review considers some of the methodological problems and caveats besetting clinical research in this field: the very definition of coma, definition of the study population and...

Prediction of outcome from coma is a frequent and important task of neurologists. It is difficult enough in adult patients and even more difficult in children. Part I of this review considers some of the methodological problems and caveats besetting clinical research in this field: the very definition of coma, definition of the study population and...

Young children with refractory symptomatic epilepsy are at risk for developing neurologic and cognitive disabilities. Stopping the seizures may prevent these disabilities, but it is unclear whether resective surgery is associated with adequate long-term seizure control. This study determined pre- and postsurgery seizure frequency and antiepileptic...

The most appropriate time to consider cortical resection to treat medically intractable infantile spasms has not been clearly defined. The risks that need to be reconciled to make this decision are: What is the risk of loss of developmental potential if surgery is delayed too long versus what is the risk of unnecessary surgery if it is done too soo...

According to traditional neurophysiological theory, consciousness requires neocortical functioning, and children born without cerebral hemispheres necessarily remain indefinitely in a developmental vegetative state. Four children between 5 and 17 years old are reported with congenital brain malformations involving total or near-total absence of cer...

The somatic pathophysiology of high spinal cord injury (SCI) not only is of interest in itself but also sheds light on one of the several rationales proposed for equating 'brain death' (BD) with death, namely that the brain confers integrative unity upon the body, which would otherwise constitute a mere conglomeration of cells and tissues. Insofar...

Increased risk of death has been reported in patients with intractable epilepsy (IE) taking nitrazepam (NZP). Between January 1983 and March 1994, 302 patients with IE were entered into a NZP compassionate-plea protocol. NZP was discontinued if there was < 50% seizure reduction or significant side effects. In some patients with > 50% reduction, it...

One rationale for equating "brain death" (BD) with death is that it reduces the body to a mere collection of organs, as evidenced by purported imminence of asystole despite maximal therapy. To test this hypothesis, cases of prolonged survival were collected and examined for factors influencing survival capacity. Formal diagnosis of BD with survival...

The author challenges brain-based diagnoses of death by re-examining the concept of death, its definition, the anatomical criterion, and the clinical signs or tests. Dr. Shewmon challenges the fundamental assumptions underlying brain death: (1) that the brain is the body's "critical system"; and (2) that the body even has a localized "critical syst...

Two-year postsurgical developmental outcomes were assessed in 24 children with infantile spasms who underwent resective surgery. The mean age of onset of infantile spasms was 12.0 weeks and the mean age at surgery was 20.8 months. Developmental outcomes were assessed using the Vineland Adaptive Behavior Scales (VABS). There was a significant increa...

Pyridoxine dependency often presents with intractable seizures in neonates and infants.We describe an infant with pyridoxine-dependent seizures and report the first PET and evoked potential results and EEG and MRI findings. All studies show either diffuse structural or functional abnormality. Our data suggest this coenzyme deficiency causes a gener...

Fifty-eight children who underwent anatomical, functional, or modified anatomical hemispherectomy for intractable seizures from 1986 to 1995 were evaluated for seizure control, motor function, and complications. Age at surgery ranged from 0.3 to 17.3 years (median 2.8 years). Twenty-seven anatomical, 27 functional, and 4 modified anatomical hemisph...

Infantile spasms most commonly show symmetric behavioral and electroencephalogram (EEG) manifestations. Asymmetric and asynchronous behavioral spasms occur occasionally, but their relationship to ictal EEG and to other localizing studies has not received much attention. We reviewed 75 consecutive video-EEG recordings, done at UCLA from 1982 to 1992...

To determine whether microscopic cortical lamination defects in patients with infantile spasms, not initially identifiable on MR, may be inferred from evolving changes in the adjacent white matter. Three infants between 3 and 6 months of age presented with infantile spasms. Based on negative metabolic assessment and normal MR findings, they were cl...

To determine seizure propagation patterns, we analyzed ictal positron emission tomography (PET) studies of regional cerebral glucose utilization in 18 children (11 male and 7 female aged 2 weeks to 16 years) with epilepsy (excluding infantile spasms IS). Three major metabolic patterns were determined based on degree and type of subcortical involvem...

Twenty-three infants and children underwent cortical resection (n = 15) or hemispherectomy (n = 8) for intractable infantile spasms. Infantile spasms were present at the time of surgery in 17 of the 23 patients; in six, spasms had evolved to other seizure types during surgical evaluation. Children with a remote history of infantile spasms were excl...

Decreased glucose utilization in the epileptogenic zone is typically observed interictally on positron emission tomography (PET), whereas ictal PET studies reveal complex patterns of increased and decreased metabolism. PET findings of 7 children, ages 2 months to 16 years, are described and demonstrate small focal regions of hypermetabolism in the...

Increasing concern about children in a persistent vegetative state (PVS) prompted a survey of members of the Child Neurology Society regarding aspects of the diagnosis and management of this disorder. Major findings of those responding to this survey (26% response rate) were as follows: (1) 93% believed that a diagnosis of PVS can be made in childr...

Refractory status epilepticus (RSE) is defined as status epilepticus that continues despite aggressive treatment. A 9.8-year-old boy with a past history of daily left focal motor seizures was transferred to University of California at Los Angeles (UCLA) Hospital in pentobarbital coma after 4 days in RSE. The RSE was treated with very high doses of...

Infantile spasms are generalized seizures specific to early infancy, and are believed to result from complex cortical-subcortical interactions during a critical period of development. We used positron emission tomography (PET) to determine local cerebral metabolic rates for glucose (1CMRG1c) in 44 infants with spasms, in an attempt to define the ne...

A 10-year-old girl with intractable complex partial seizures developed aphasia, coprolalia, and repetitive motor behaviors involving touching, sexual touching, and aggressive acts. Her symptoms subsided following surgical resection of a left anterior temporal lobe ganglioglioma and control of seizures. Possible neurobehavioral implications of the r...

In our series of 33 children who underwent temporal and extended temporal lobe resections because of seizures, the average age at surgery was 7 years, 11 months. Sixteen cases (48%) were diagnosed as having tumors: low-grade astrocytoma (6), hamartoma (5), and ganglioglioma/neuroma (5). Other pathologic diagnoses included one or more cytoarchitectu...

Although infantile spasms were initially described in 1841, remarkably little progress has been made in understanding the pathophysiology of this "peculiar form of infantile convulsions." Consequently, our ability to treat infantile spasms is limited. Infantile spasms are classified as a "generalized" seizure disorder in the international classific...

The relationship between focal disturbances of glucose utilization demonstrated by positron emission tomography (PET) and electrophysiologic abnormalities defined by intraoperative electrocorticography (ECoG) was studied in eight children (aged 13 months to 12 years) who underwent cortical resection because of intractable seizures. None of the chil...

Despite the clinical and basic scientific importance of neonatal seizures, a standard operational definition of them is lacking. Virtually all quantitative studies of phenomenology, prevalence, treatment efficacy, and prognosis have been undermined by inadequate or inconsistent criteria for the object of investigation. This is not so much a fault o...

Positron emission tomography (PET) of local cerebral glucose metabolism in 13 children with infantile spasms of undetermined cause (cryptogenic spasms) revealed unilateral hypometabolism involving the parieto-occipito-temporal region in 5 female infants. Cranial computed tomography showed normal findings in all infants. Magnetic resonance imaging (...

As desirable as it might be to predict early in the course of coma whether a patient will do well or poorly, all studies of coma prognosis are plagued by inherent methodologic problems that tend to diminish the utility of the derived criteria: especially the tendency of poor prognoses to be self-fulfilling, the rapid drop-off in patient population...

We have previously reported that focal occipital interictal epileptiform discharges (spikes) cause transiently prolonged reaction time (RT) and increased nonperception of stimuli, especially in the visual field contralateral to the spike. One subject with very frequent spikes was capable of carrying out a visual recognition task along with the RT t...

In Reply.— The President's Commission1 stated: "Obviously, slippery slope arguments must be very carefully employed lest they serve merely as an unthinking defense of the status quo.... Nevertheless, the Commission has found that [where] human life is at issue, valid concerns warrant being especially cautious before adopting any policy that weaken...

To the Editor. —Grigg and colleagues1 in a past issue of the Archives have documented that electroencephalographic (EEG) activity, sometimes bearing a striking resemblance to physiologic sleep, can persist in the presence of extensive brain-stem infarcts. This is hardly surprising nor is it new information. The only really new contribution of the...

The recent abandonment of the only active US protocol for harvesting organs from anencephalic "donors" indicates both the practical and the ethical problems inherent in such an effort. Various data suggest that surprisingly few such organs would actually end up benefiting other children. Attempts to revise either the Uniform Anatomical Gift Act or...

The recent abandonment of the only active US protocol for harvesting organs from anencephalic "donors" indicates both the practical and the ethical problems inherent in such an effort. Various data suggest that surprisingly few such organs would actually end up benefiting other children. Attempts to revise either the Uniform Anatomical Gift Act or...

Electroencephalography (EEG) was performed on 16 neonates between 38-45 weeks conceptional age with hypoplastic left heart syndrome (HLHS). The findings were compared blindly with EEGs recorded from 16 infants with other congenital heart defects and 15 infants referred for EEGs with noncardiac diagnoses or problems. EEGs from HLHS infants demonstra...

Un groupe de travail s'est attache a analyser les criteres permettant de determiner la mort cerebrale chez les enfants; les publications de ce groupe sont commentees, avec deux questions importantes: quel degre de certitude est-il necessaire pour declarer le deces, quel est le risque d'un diagnostic «faux positif». Le probleme du don eventuel d'org...

Several elements of anencephaly are discussed: problems of diagnosis, its declining prevalence and the effect of prenatal screening and subsequent abortion, the extent of neurological functioning and consciousness of anencephalic infants, their life span and cause of death, and the utility of organs for transplantation. The author concludes that pr...

We have performed positron emission tomography (PET) with 2-deoxy-2[18F]fluoro-D-glucose (FDG) in eight infants and children (aged 18 days to 5 years) with medically refractory epilepsy of neonatal onset. It was hypothesized that in at least some of these infants a surgical approach (focal resection, cerebral hemispherectomy) might be of benefit in...

The effect of focal interictal spikes on visual perception and reaction time (RT) was studied in 3 subjects, by means of a computerized system of spike detection, presentation of visual stimuli, and timing of a button press. A total of 8646 such trials were analyzed, comparing spike-locked and control performances within the same subject. For stimu...

By means of a computerized system of spike detection, presentation of visual stimuli, and timing of a button press, we showed previously that single posterior interictal spikes resulted in transient prolongation of reaction time and increased non-response rate in 3 subjects. By varying the response hand and the visual field of stimulus, conditions...

By means of a computerized system of spike detection, presentation of visual stimuli, and registration of reaction times (RTs), we have shown previously that focal posterior interictal spikes cause transiently prolonged RT and increased nonperception and misperception of stimuli, especially contralateral to the spike. We report here the temporal pr...

A great need persists for diagnostic criteria for both brain death in young children and irreversible loss of consciousness at all ages. This article examines the inferences derived from a hypothetical confirmatory study in which all of the N patients who fulfilled the criterion did in fact experience brain death (irreversibility). A Bayesian metho...

Fifty-two patients were enrolled in a four-week randomized multicenter study comparing nitrazepam and corticotropin in the treatment of infantile spasms. The drugs' efficacy was evaluated in 48 patients, all less than 2 years of age. Both treatments resulted in a statistically significant reduction in spasm frequency from that at baseline, but the...

Clinical and electrographic seizures were recorded in an infant with atelencephaly. Because the infant had no cerebral hemispheres, the ictal discharges were presumed to arise from the disorganized diencephalic derivatives that occupied the entire supratentorial space. The case provided strong support for the concept that, unlike epileptic seizures...

Atelencephaly is a rare and lethal form of microcephaly that is important to distinguish from other severe dysmorphogenetic or destrcutive cerebral anomalies because of varying genetic implications. It consists of an absence of cerebral hemispheres and ventricular system, with preserved cranial bones, scalp, and brainstem. Two previous reports, fou...

A method is described for off-line reformatting of recorded EEG data to any desired montage, gains, filter settings and paper speed. Thirty-two channels, including every scalp electrode, are recorded in a common referential montage and multiplexed onto videocassettes . Playback onto an EEG machine faithfully reproduces the original signals, so that...

The majority of infants with congenital rubella shed rubella virus during the neonatal period,1,2 but over the first year of life this shedding steadily decreases so that by 1 year of age only 2 to 20% of infants are still rubella virus-positive.1"3 With the exception of three children noted by Michaels4 and an adult studied by Menser et al.,5 vira...

One to 2 weeks after severe brain ischemia, four infants developed an unusual vascular lesion with a characteristic appearance on computed tomography that has not been reported previously. Restricted areas (most frequently the basal ganglia and thalamus) displayed increased attenuation, which enhanced further upon infusion of contrast medium. Autop...

In a 23-year-old man, radiation necrosis developed in the left hemisphere after radiation of a right-sided intracranial tumor. Since normal tissue had to be resected around the tumor, the contralateral temporal lobe received the highest cumulative radiation dose for brain tissue. This report underscores the need for careful reconstruction of the ra...