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1/62. Impaired effective cortical connectivity in vegetative state: preliminary investigation using PET.

    Vegetative state (VS) is a condition of abolished awareness with persistence of arousal. awareness is part of consciousness, which itself is thought to represent an emergent property of cerebral neural networks. Our hypothesis was that part of the neural correlate underlying VS is an altered connectivity, especially between the associative cortices. We assessed regional cerebral glucose metabolism (rCMRGlu) and effective cortical connectivity in four patients in VS by means of statistical parametric mapping and [18F]fluorodeoxyglucose-positron emission tomography. Our data showed a common pattern of impaired rCMRGlu in the prefrontal, premotor, and parietotemporal association areas and posterior cingulate cortex/precuneus in VS. In a next step, we demonstrated that in VS patients various prefrontal and premotor areas have in common that they are less tightly connected with the posterior cingulate cortex than in normal controls. These results provide a strong argument for an alteration of cortical connectivity in VS patients.
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2/62. Assessment of command-following in minimally conscious brain injured patients.

    OBJECTIVE: To develop a method for establishing the presence of command-following in individuals with traumatic brain injury, based on the principles of single-subject experimental design. DESIGN: A series of single-subject experiments, individualized to the particular command-following question about a particular patient. SETTING: An inpatient rehabilitation hospital with a specialized program for vegetative and minimally conscious brain injured patients. patients: Eight individuals with serious brain injury of traumatic or nontraumatic origin, presenting in vegetative or minimally conscious states. INTERVENTIONS: The frequency of performance of the behavior in question was assessed in response to commands and in relation to appropriate control conditions. Data were analyzed with chi2 or Fisher's exact test, as well as measures derived from signal detection theory. MAIN OUTCOME MEASURES: The frequency of performance of a specific behavior in the presence of a command and in relevant contrasting conditions. RESULTS: This method identified whether a specific behavior was being performed in response to command and whether the reliability of this behavior was changing over time either spontaneously or in response to treatment. CONCLUSIONS: Quantitative assessment of command-following based on principles of single-subject experimental design can determine whether patients are capable of following commands and whether this ability changes over time or in response to treatment.
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3/62. consciousness in congenitally decorticate children: developmental vegetative state as self-fulfilling prophecy.

    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 cerebral cortex but who, nevertheless, possessed discriminative awareness: for example, distinguishing familiar from unfamiliar people and environments, social interaction, functional vision, orienting, musical preferences, appropriate affective responses, and associative learning. These abilities may reflect 'vertical' plasticity of brainstem and diencephalic structures. The relative rarity of manifest consciousness in congenitally decorticate children could be due largely to an inherent tendency of the label 'developmental vegetative state' to become a self-fulfilling prophecy.
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4/62. Cerebral arteriovenous malformation in pregnancy: presentation and neurologic, obstetric, and ethical significance.

    Cerebral arteriovenous malformations infrequently complicate pregnancy. We sought to determine the neurologic, obstetric, and ethical significance of such malformations. We present the clinical course of 2 pregnant women with arteriovenous malformations who experienced cerebral hemorrhage and a loss of capacity for decision making. We also review the neurologic and obstetric significance of arteriovenous malformations in pregnancy. Various treatment options with concern for pregnancy and the prognosis for arteriovenous malformations are outlined. The ethical issues involved for pregnant patients whose decisional capacity is compromised as a result of cerebral injury are explored. A review of persistent vegetative state and brain death (death by neurologic criteria) occurring in pregnancy allows us to explore many issues that are applicable to decisionally incapacitated but physiologically functioning pregnant women. We outline a document, the purpose of which is to obtain advance directives from pregnant women regarding end-of-life decisions and to appoint a surrogate decision maker. We believe that evaluation and treatment of the arteriovenous malformation may be undertaken without regard for the pregnancy and that the pregnancy should progress without concern for the arteriovenous malformation.
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5/62. Assessment of minimally responsive patients: clinical difficulties of single-case design.

    Improved management of very severely central nervous system (CNS) injured individuals has given rise to an increasing number of patients in a minimally responsive state. There is a growing literature stressing the importance of accurately determining these patients' level of cognitive functioning and its role in appropriate rehabilitation and long term management. The single case design model appears to be the intervention of choice, with its great flexibility and tailored approach to each individual case. The recent literature has focused on the technical aspects of the assessment, offering clear procedural guidelines. Unfortunately, there is a dearth of information about clinical factors such as clinical setting and family involvement, which may interfere with or prevent a planned intervention. The case of MT is presented, who was the subject of a single case intervention 9 months following an extremely severe traumatic brain injury. The planned intervention was to examine the effects of a psychostimulant on MT's level of arousal, in order to improve his participation in the rehabilitation programme. Beyond the results (which were equivocal), the clinical difficulties in conducting single case study designs in rehabilitation are discussed. Ways to minimize these difficulties are proposed.
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6/62. Neuropsychological assessment of a potential "euthanasia" case: a 5 year follow up.

    McMillan reported a neuropsychological assessment procedure which was used to determine whether or not there was evidence for sentience in a young woman who had been rendered tetraplegic and anarthric as a result of a road traffic accident. An application to court had been made to withdraw feeding and this was supported by medical evidence which gave the view that the individual was functioning little beyond vegetative state, had a poor quality of life and had little prospect of further recovery. Evidence for an ability to communicate reliably was found including for a wish to continue living, and as a consequence the application to court was withdrawn. This paper describes further recovery 2-4 years after the original assessment (i.e. 4-6 years post-injury). At follow-up, she remained dependent for all care, but was now feeding orally and was talking. She could learn new information, some of which she retained for at least 12 months and had greater insight into her condition. She now reported low mood and some pain. As before, she consistently reported a wish to live. The implications of the follow-up are discussed in the context of assumptions made about quality of life and decision making about euthanasia in brain injured people who are severely disabled, but are not in a vegetative state.
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7/62. Heterotopic ossification in childhood and adolescence.

    Heterotopic ossification, or myositis ossificans, denotes true bone in an abnormal place. The pathogenic mechanism is still unclear. A total of 643 patients (mean age, 9.1 years) admitted for neuropediatric rehabilitation were analyzed retrospectively with respect to the existence of neurogenic heterotopic ossification. The purpose of this study was to obtain information about incidence, etiology, clinical aspect, and consequences for diagnosis and therapy of this condition in childhood and adolescence. Heterotopic ossification was diagnosed in 32 patients (mean age, 14.8 years) with average time of onset of 4 months after traumatic brain injury, near drowning, strangulation, cerebral hemorrhage, hydrocephalus, or spinal cord injury. The sex ratio was not significant. In contrast to what has been found in adult studies, serum alkaline phosphatase was not elevated during heterotopic ossification formation. A persistent vegetative state for longer than 30 days proved to be a significant risk factor for heterotopic ossification. The incidence of neurogenic heterotopic ossification in children seems to be lower than in adults. A genetic predisposition to heterotopic ossification is suspected but not proven. As a prophylactic regimen against heterotopic ossification we use salicylates for those patients in a coma or persistent vegetative state with warm and painful swelling of a joint and consider continuous intrathecal baclofen infusion and botulinum toxin injection for those patients with severe spasticity. We prefer to wait at least 1 year after trauma before excision of heterotopic ossification.
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8/62. Fatal fat embolism syndrome: a case report.

    Fat embolism syndrome is a dire complication of long bone trauma. It is usually associated with neurological, hematological and respiratory involvement, the latter being the major cause of death. We present a case of severe fat embolism syndrome occurring 3 hours after a long bone injury, leading to permanent vegetative state and death without any respiratory signs. The diagnosis was confirmed by cytology of the bronchoalveolar lavage fluid. Clinical presentation of the puzzling fat embolism syndrome and diagnostic tests in suspected fat embolism syndrome are reviewed.
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9/62. Subacute central nervous system degeneration in a child: an unusual manifestation of ifosfamide intoxication.

    A 5-year-old child with desmoplastic small round-cell tumor was treated with a protocol of very-high-dose, short-term chemotherapy, containing HD-CAV (cyclophosphamide, doxorubicin, vincristine, and mesna), ifosfamide, and etoposide. Two days after the initiation of ifosfamide, he exhibited new-onset lethal encephalopathy manifested by subacutely progressive cerebellar and then temporal and frontocortical degeneration leading to a vegetative state and eventually to death. A full work-up, including brain biopsy, was negative, excluding infections and metabolic or vascular causes. ifosfamide is known to be capable of causing acute encephalopathy that can be severe but is generally reversible. This child showed a very atypical progressive, lethal course of ifosfamide toxicity. The possibility of this complication should be considered when high-dose ifosfamide treatment is planned for children.
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10/62. Anaesthetic management of a pregnant patient in a persistent vegetative state.

    pregnancy in a patient in a persistent vegetative state presents challenging therapeutic questions about the level of supportive management required, the assessment of fetal well-being, the timing and mode of delivery and the anaesthetic management of labour and delivery. We report the case of a 29-yr-old woman who had a favourable fetal outcome despite suffering hypoxic brain damage after a suicide attempt by a drug overdose. She was managed until the onset of labour on an intensive care unit and had a spontaneous vaginal delivery assisted by epidural anaesthesia.
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