Adam Zeman

The syndrome of transient epileptic amnesia

Transient amnesia can be the principal manifestation of epilepsy. This diagnosis, however, is seldom suspected by clinicians and remains controversial. The amnestic attacks are often associated with persistent memory complaints. This study was designed to provide the first description of transient epileptic amnesia in a substantial series of patients.

Persistent vegetative state

SIR—Adam Zeman (Sept 13, p 795) is to be congratulated on his excellent discussion of the concept of persistent vegetative state (PVS). As he points out, this concept is based on a particular model of consciousness and wakefulness that has certain shortcomings. The shortcomings are well illustrated by two consecutive sentences in his conclusion: “Consciousness [or awareness], in all its aspects, is a matter of degree”, and “The vegetative state [is] a condition of wakefulness without awareness”. The first correctly identifies awareness as a continuum rather than a quality that is either present or absent. The second should therefore define PVS as a state of wakefulness without a set amount of awareness. This definition makes the concept less superficially attractive (and begs the question how awareness is measured and how much is allowed in 5-HT2A receptor gene polymorphisms, anorexia nervosa, and obesity

fMRI in patients with motor conversion symptoms and controls with simulated weakness

Background: Conversion disorder (motor type) describes weakness that is not due to recognized disease or conscious simulation but instead is thought to be a “psychogenic” phenomenon. It is a common clinical problem in neurology but its neural correlates remain poorly understood. Objective: To compare the neural correlates of unilateral functional weakness in conversion disorder with those in healthy controls asked to simulate unilateral weakness. Methods: Functional magnetic resonance imaging (fMRI) was used to examine whole brain activations during ankle plantarflexion in four patients with unilateral ankle weakness due to conversion disorder and four healthy controls simulating unilateral weakness. Group data were analyzed separately for patients and controls. Results: Both patients and controls activated the motor cortex (paracentral lobule) contralateral to the “weak” limb less strongly and more diffusely than the motor cortex contralateral to the normally moving leg. Patients with conversion disorder activated a network of areas including the putamen and lingual gyri bilaterally, left inferior frontal gyrus, left insula, and deactivated right middle frontal and orbitofrontal cortices. Controls simulating weakness, but not cases, activated the contralateral supplementary motor area. Conclusions: Unilateral weakness in established conversion disorder is associated with a distinctive pattern of activation, which overlaps with but is different from the activation pattern associated with simulated weakness. The overall pattern suggests more complex mental activity in patients with conversion disorder than in controls. BA = Brodmann area; DLPFC = dorsolateral prefrontal cortex; DSM-IV = Diagnostic and Statistical Manual of Diseases, fourth revision; EPI = echo planar imaging; fMRI = functional magnetic resonance imaging; ICD-10 = International Classification of Diseases, version 10; MNI = Montreal Neurological Institute; MRI = magnetic resonance imaging; NCS = nerve conduction studies; PET = positron emission tomography; SMA = supplementary motor area; SPECT = single photon emission computed tomography; SPM99 = statistical parametric mapping software.

Transient epileptic amnesia: regional brain atrophy and its relationship to memory deficits

Transient epileptic amnesia (TEA) is a recently recognised form of epilepsy of which the principle manifestation is recurrent, transient episodes of isolated memory loss. In addition to the amnesic episodes, many patients describe significant interictal memory difficulties. Performance on standard neuropsychological tests is often normal. However, two unusual forms of memory deficit have recently been demonstrated in TEA: (i) accelerated long-term forgetting (ALF): the excessively rapid loss of newly acquired memories over a period of days or weeks and (ii) remote autobiographical memory loss: a loss of memories for salient, personally experienced events of the past few decades. The neuroanatomical bases of TEA and its associated memory deficits are unknown. In this study, we first assessed the relationship between subjective and objective memory performance in 41 patients with TEA. We then analysed MRI data from these patients and 20 matched healthy controls, using manual volumetry and voxel-based morphometry (VBM) to correlate regional brain volumes with clinical and neuropsychological data. Subjective memory estimates were unrelated to performance on standard neuropsychological tests but were partially predicted by mood, ALF and remote autobiographical memory. Manual volumetry identified subtle hippocampal volume loss in the patient group. Both manual volumetry and VBM revealed correlations between medial temporal lobe atrophy and standard anterograde memory scores, but no relation between atrophy and ALF or remote autobiographical memory. These results add weight to the hypothesis that TEA is a syndrome of mesial temporal lobe epilepsy. Furthermore, they suggest that although standard anterograde memory test performance is related to the degree of mesial temporal lobe damage, this is not true for ALF and autobiographical amnesia. It is possible that these unusual memory deficits have a more diffuse physiological basis rather than being a consequence of discrete structural damage.

Accelerated forgetting of real-life events in Transient Epileptic Amnesia.

Transient Epileptic Amnesia (TEA) is a form of temporal lobe epilepsy associated with ictal and interictal memory disturbance. Some patients with TEA exhibit Accelerated Long-term Forgetting (ALF), in which memory for verbal and non-verbal material is retained normally over short delays but fades at an unusually rapid rate over days to weeks. This study addresses three questions about ALF in TEA: (i) whether real-life events undergo ALF in a similar fashion to laboratory-based stimuli; (ii) whether ALF can be detected within 24h; (iii) whether procedural memories are susceptible to ALF. Eleven patients with TEA and eleven matched healthy controls wore a novel, automatic camera, SenseCam, while visiting a local attraction. Memory for images of events was assessed on the same day and after delays of one day, one week, and three weeks. Forgetting of real-life events was compared with forgetting of a word list and with performance on a procedural memory task. On the day of their excursion, patients and controls recalled similar numbers of primary events, associated secondary details (contiguous events, thoughts and sensory information) and items from the word list. In contrast, patients showed ALF for primary events over three weeks, with ALF for contiguous events, thoughts and words over the first day. Retention on the procedural memory task was normal over three weeks. The results indicate that accelerated forgetting in TEA: (i) affects memory for real-life events as well as laboratory stimuli; (ii) is maximal over the first day; and (iii) is specific to declarative memories.

Autobiographical amnesia and accelerated forgetting in transient epileptic amnesia

Background: Recurrent brief isolated episodes of amnesia associated with epileptiform discharges on EEG recordings have been interpreted as a distinct entity termed transient epileptic amnesia (TEA). Patients with TEA often complain of autobiographical amnesia for recent and remote events, but show normal anterograde memory. Objective: To investigate (a) accelerated long term forgetting and (b) autobiographical memory in a group of patients with TEA. Methods: Seven patients with TEA and seven age matched controls were evaluated on a range of anterograde memory tasks in two sessions separated by 6 weeks and by the Galton-Crovitz test of cued autobiographical memory. Results: Patients with TEA showed abnormal long term forgetting of verbal material, with virtually no recall after 6 weeks. In addition, there was impaired recall of autobiographical memories from the time periods 1985–89 and 1990–94 but not from 1995–1999. Conclusions: TEA is associated with accelerated loss of new information and impaired remote autobiographical memory. There are a number of possible explanations including ongoing subclinical ictal activity, medial temporal lobe damage as a result of seizure, or subtle ischaemic pathology. Future analyses should seek to clarify the relationship between aetiology, seizure frequency, and degree of memory impairment.

Functional weakness and sensory disturbance

In the diagnosis of functional weakness and sensory disturbance, positive physical signs are as important as absence of signs of disease. Motor signs, particularly Hoovers sign, are more reliable than sensory signs, but none should be used in isolation and must be interpreted in the overall context of the presentation. It should be borne in mind that a patient may have both a functional and an organic disorder.

Dualism Persists in the Science of Mind

The relationship between mind and brain has philosophical, scientific, and practical implications. Two separate but related surveys from the University of Edinburgh (University students, n= 250) and the University of Liège (health‐care workers, lay public, n= 1858) were performed to probe attitudes toward the mind–brain relationship and the variables that account for differences in views. Four statements were included, each relating to an aspect of the mind–brain relationship. The Edinburgh survey revealed a predominance of dualistic attitudes emphasizing the separateness of mind and brain. In the Liège survey, younger participants, women, and those with religious beliefs were more likely to agree that the mind and brain are separate, that some spiritual part of us survives death, that each of us has a soul that is separate from the body, and to deny the physicality of mind. Religious belief was found to be the best predictor for dualistic attitudes. Although the majority of health‐care workers denied the distinction between consciousness and the soma, more than one‐third of medical and paramedical professionals regarded mind and brain as separate entities. The findings of the study are in line with previous studies in developmental psychology and with surveys of scientists’ attitudes toward the relationship between mind and brain. We suggest that the results are relevant to clinical practice, to the formulation of scientific questions about the nature of consciousness, and to the reception of scientific theories of consciousness by the general public.

Dyspraxia in a patient with corticobasal degeneration: the role of visual and tactile inputs to action

OBJECTIVES To investigate the roles of visual and tactile information in a dyspraxic patient with corticobasal degeneration (CBD) who showed dramatic facilitation in miming the use of a tool or object when he was given a tool to manipulate; and to study the nature of the praxic and neuropsychological deficits in CBD. METHODS The subject had clinically diagnosed CBD, and exhibited alien limb behaviour and striking ideomotor dyspraxia. General neuropsychological evaluation focused on constructional and visuospatial abilities, calculation, verbal fluency, episodic and semantic memory, plus spelling and writing because impairments in this domain were presenting complaints. Four experiments assessed the roles of visual and tactile information in the facilitation of motor performance by tools. Experiment 1 evaluated the patient’s performance of six limb transitive actions under six conditions: (1) after he described the relevant tool from memory, (2) after he was shown a line drawing of the tool, (3) after he was shown a real exemplar of the tool, (4) after he watched the experimenter perform the action, (5) while he was holding the tool, and (6) immediately after he had performed the action with the tool but with the tool removed from his grasp. Experiment 2 evaluated the use of the same six tools when the patient had tactile but no visual information (while he was blindfolded). Experiments 3 and 4 assessed performance of actions appropriate to the same six tools when the patient had either neutral or inappropriate tactile feedback—that is, while he was holding a non-tool object or a different tool. RESULTS Miming of tool use was not facilitated by visual input; moreover, lack of visual information in the blindfolded condition did not reduce performance. The principal positive finding was a dramatic facilitation of the patient’s ability to demonstrate object use when he was holding either the appropriate tool or a neutral object. Tools inappropriate to the requested action produced involuntary performance of the stimulus relevant action. CONCLUSIONS Tactile stimulation was paramount in the facilitation of motor performance in tool use by this patient with CBD. This outcome suggests that tactile information should be included in models which hypothesise modality specific inputs to the action production system. Significant impairments in spelling and letter production that have not previously been reported in CBD have also been documented.

Time to end the distinction between mental and neurological illnesses.

Mental and neurological conditions are classified in different chapters of diagnostic manuals. P D White, H Rickards, and A Z J Zeman argue that this distinction is inconsistent with current scientific understanding and that the conditions should be grouped together as disorders of the nervous system