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1/8. 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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2/8. 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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3/8. 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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4/8. Acquired heterotopic ossification in the settings of cerebral anoxia and alternative therapy: two cases.

    Acquired Heterotopic Ossification (HO) has been well described in the literature as a recognized complication following spinal cord injury, traumatic brain injury and joint arthroplasty. Commonly, large proximal limb joints are affected. The underlying mechanisms for ectopic bone formation remain poorly elucidated. Post-stroke hemiplegia as a cause of neurogenic HO is rare, and no published reports of HO occurring after anoxic brain injury in adults have been documented. This study reports two unusual cases of acquired HO: (1) Polyarticular HO involving the ankle joint in a 24-year-old Chinese female who suffered severe anoxic encephalopathy following near drowning which resulted in persistent vegetative state; and (2) elbow HO in chronic post-stroke hemiplegia occurring as a complication of alternative therapy following repeated forceful manipulation by a traditional practitioner in a 46 year-old male.
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5/8. pregnancy in a persistent vegetative state: case report, comparison to brain death, and review of the literature.

    Severe maternal neurologic injury during pregnancy has the potential for fetal demise without advanced critical care support to the mother. brain death is the unequivocal and irreversible loss of total brain function, whereas patients in a vegetative state, by contrast, have preserved brain stem function but lack cerebral function. They can appear to be awake, have sleep-wake cycles, be capable of swallowing, and have normal respiratory control, but there are no purposeful interactions. These conditions have different maternal prognoses, but both have resulted in near-normal neonatal outcomes with long latencies from maternal injury to delivery in previously published cases. This article compares and contrasts the 11 cases of brain death with 15 cases of persistent vegetative state in pregnancy. We found that the mean latency between maternal brain injury and delivery was significantly shorter in the brain-dead patients as compared with those in a vegetative state (46 days vs. 124 days, P persistent vegetative state in pregnancy at our institution with both maternal and neonatal death in the context of previously published literature with a focus on obstetric and ethical management. We hope this information will help elucidate the issues for providers confronted with these unique and challenging cases. TARGET AUDIENCE: Obstetricians & Gynecologists, family physicians. learning OBJECTIVES: After completion of this article, the reader should be able to state the difference between coma, persistent vegetative state and brain death, to describe the neurologic aspects of a patient in a persistent vegetative state, and to list the fetal effects of maternal brain injury.
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6/8. Paediatric near-drowning: mortality and outcome in a temperate climate.

    The decision whether to continue to resuscitate the paediatric victim of near-drowning is influenced by potential poor neurological outcome. A low core body temperature at presentation is frequently cited as a reason to continue resuscitation. We report the case of an 11 month old infant admitted to the intensive care unit following near-drowning and a prolonged resuscitation. The infant's core body temperature was 29 degrees C. cardiac output was restored, but the child remains in a persistent vegetative state. We present the results of a ten year review of near-drowning in a tertiary referral institution, to evaluate the mortality and outcome in a temperate climate. Thirteen patients were identified in the review. The mortality was 23%. The incidence of a persistent vegetative state was 15%. Asystole, immersion time greater than 15 minutes, resuscitation time longer than 30 minutes, the administration of epinephrine, and a low core body temperature were associated with a poor outcome.
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7/8. Drug induced arousal from the permanent vegetative state.

    BACKGROUND: Zolpidem is an omega 1 specific indirect GABA agonist that is used for insomnia, but may have efficacy in brain damage. The long term efficacy of zolpidem in the permanent vegetative state is described in three patients. METHOD: Two motor vehicle accident patients and one near drowning patient, all of them in the permanent vegetative state for at least three years, were rated according to the Glasgow Coma and Rancho Los Amigos scale before and after zolpidem application. Long term response to daily application of this drug was monitored for 3-6 years. RESULTS: All patients were aroused transiently every morning after zolpidem. glasgow coma scale scores ranged from 6-9/15 before to 10-15/15 after zolpidem. Rancho Los Amigos Cognitive scores ranged from I-II before to V-VII afterward. Drug efficacy did not decrease and there were no long term side effects after 3-6 years daily use. CONCLUSION: Zolpidem appears an effective drug to restore brain function to some patients in the permanent vegetative state.
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8/8. Immunoablation does not delay the neurologic progression of X-linked adrenoleukodystrophy.

    We report the results of a near total myeloablation in preparation for bone marrow transplantation in a boy with minimal symptoms of X-linked adrenoleukodystrophy. Severe cerebral X-linked adrenoleukodystrophy developed in the patient after failure of bone marrow transplantation. This experience suggests that immunotherapy alone is not responsible for the improvement observed in some patients with X-ALD after BMT.
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