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1/45. 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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ranking = 1
keywords = brain injury, traumatic brain injury, traumatic brain, brain, injury, trauma
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2/45. 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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ranking = 0.025191975881997
keywords = brain
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3/45. 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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ranking = 0.014269120450774
keywords = brain, injury
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4/45. Subacute encephalopathy in a 5-year-old boy.

    A 5-year-old boy presented with an acute ataxia and altered mental status. Although he initially recovered from these symptoms, he presented a second time with myoclonus and seizures and rapidly became vegetative. cerebrospinal fluid studies, magnetic resonance imaging, and brain biopsy all confirmed the presence of subacute sclerosing panencephalitis. Despite courses of therapy with cimetidine, amantadine, ribavirin, and inosine, no clinical improvement has been seen. Clinicians need to be alert to the possibility of subacute sclerosing panencephalitis even in the vaccinated child in the appropriate clinical setting.
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ranking = 0.012595987940998
keywords = brain
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5/45. 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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ranking = 0.57796598314106
keywords = brain injury, traumatic brain injury, traumatic brain, brain, injury, trauma
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6/45. Words without mind.

    A woman (LR), unconscious for 20 years, spontaneously produces infrequent, isolated words unrelated to any environmental context. Fluorodeoxy-glucose-positron emission tomography (FDG-PET) imaging coregistered with magnetic resonance imaging (MRI) revealed a mean brain metabolism equivalent to deep anesthesia. Nevertheless, PET imaging demonstrated islands of modestly higher metabolism that included Broca's and Wernicke's areas. Functional brain imaging with magnetoencephalographic (MEG) imaging, a technique providing a temporal resolution of better than 1 msec, identified preserved dynamic patterns of spontaneous and evoked brain activity in response to sensory stimulation. Specifically, we examined spontaneous gamma-band activity (near 40 Hz) and its reset or modification during early auditory processing, a measure that correlated with human perception of sensory stimuli (Joliot, Ribary, & Llinas, 1994). Evidence of abnormal and incomplete gamma-band responses appeared in the left hemisphere only in response to auditory or somatosensory stimulation. MEG single-dipole reconstructions localized to the auditory cortex in the left hemisphere and overlapped with metabolically active regions identified by FDG-PET. The observation demonstrates that isolated neuronal groups may express well-defined fragments of activity in a severely damaged, unconscious brain. The motor fixed-action pattern character of her expressed words supports the notion of brain modularity in word generation.
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ranking = 0.062979939704991
keywords = brain
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7/45. 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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ranking = 0.014269120450774
keywords = brain, injury
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8/45. 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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ranking = 0.57964372470525
keywords = brain injury, traumatic brain injury, traumatic brain, brain, injury, trauma
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9/45. 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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ranking = 0.0016777415641908
keywords = injury, trauma
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10/45. 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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ranking = 0.012595987940998
keywords = brain
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