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11/84. 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. ( info)

12/84. 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. ( info)

13/84. 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. ( info)

14/84. 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. ( info)

15/84. Late occurrence of diffuse cerebral swelling after intracerebral hemorrhage in a patient with the hellp syndrome--Case report.

    hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome can occur at any time in the course of pregnancy and is associated with many complications including fatal stroke. A 37-year-old female presented with hellp syndrome causing an intracerebral hematoma, which was treated by evacuation and mild hypothermia. Unexpected diffuse cerebral swelling occurred on the 15th day of the initially favorable postoperative course. Considerable impairment of consciousness persisted despite conservative therapy. Serial computed tomographic findings indicated delayed cerebral vasospasm as the cause of the swelling. Particularly careful management is required even beyond the first 2 weeks for patients with stroke as a complication of hellp syndrome. ( info)

16/84. Severe traumatic brain injury from unmotorized scooter.

    The popularity and use of unmotorized, foot-propelled scooters has risen dramatically in the last few years. Increasing reports of traumatic injuries from these scooters have paralleled their commercial sales and success in this country and worldwide. We report a case of a previously healthy 48-year-old woman who suffered a severe traumatic brain injury while riding one of these scooters, resulting in a devastating neurologic outcome and permanent vegetative state. This case emphasizes the importance of public awareness regarding the potential dangers and the need for appropriate precautions and protective gear when riding these recreational devices. ( info)

17/84. What shall we do with Norman? An experiment in communal discernment.

    We were a group of Christian friends searching for affirmations that lay at the heart of our faith and reached to the limits of our existence and moral authority. As we have reflected on our role in deciding whether and to what extent we could assist in allowing our terminally ill friend, seventy-nine-year-old, Norman to die, we were deeply troubled by the moral ambiguity of our involvement. Through a careful process of authority through communal discernment, our responsibility for Norman became clear: we were to assist him in living the life he embraced in baptism -- a life which included a destiny that was conformed to the crucified and risen one. That was not the destiny we chose for Norman; it was the destiny he owned. We recognized with Norman that our lives are not our own to be guided by autonomy and liberty, but rather to be lived for the glory of Jesus the Christ. ( info)

18/84. Cognitive recovery from "persistent vegetative state": psychological and personal perspectives.

    This study reports on the case of a young woman who, at the age of 26, developed a severe encephalomyelopathy and was in a vegetative state or minimally conscious state for 6 months. She showed a sleep-wake cycle, but no evidence of cognitive functioning. Six months after her illness, she began to respond to her environment and eventually returned home to the care of her parents, with regular periods of respite care in a home for people with severe physical disabilities. She remains in a wheelchair with a severe dysarthria and communicates via a letter board. Two years after her illness, staff at the home requested an assessment of her cognitive functioning. On the WAIS-R verbal scale and the Raven's Progressive Matrices, the woman's scores were in the normal range. So too were her recognition of real versus nonsense words and her memory functioning (apart from a visual recognition memory test which was in the impaired range). Although she enjoyed the tests, she became distressed when asked about her illness and previous hospitalization. She was reassessed 1 year later, when there were few significant changes in her test scores but she could talk about her illness and hospitalization without becoming distressed. She was angry, however, about her experiences in the first hospital. Further tests suggested good executive functioning. In short, this woman's cognitive functioning is in the normal range for most tasks assessed, despite a severe physical disability and dysarthria, and despite the fact that she was vegetative for 6 months. Although some recovery following 6 months of being vegetative/minimally conscious is not unknown, it is rare, particularly for those with non-traumatic injuries, and the majority of people similarly affected remain with significant cognitive deficits. This client has, by and large, made an almost complete cognitive recovery. She feels positive about her life now and says the formal assessment showed people she was not stupid and this made her happy. The paper concludes with the young woman's own comments and views about what happened to her and her present feelings. ( info)

19/84. Prognostic value of evoked potentials and sleep recordings in the prolonged comatose state of children. Preliminary data.

    OBJECTIVES: sleep recordings and evoked potentials (EPs) were used in five comatose children to evaluate their predictive value for outcome following a severe comatose state. methods AND SUBJECTS: The protocol included EEG, Brainstem Evoked Responses (BERs), Somatosensory evoked potentials (SEPs) and polysomnography. From 10 to 15 days post-coma (D10 to D15), EEG and clinical examinations were carried out every second day, then one day in four from 15 to 30 days post-coma (D15 to D30), and one day in seven from D30 to six months (M6). evoked potentials and polysomnography were recorded on D10-D15 or D30 in the second month (M2) and in M6. Of the five children, three were in anoxic coma and two in traumatic coma. All had extensive lesions and a glasgow coma scale (GCS) score of less than five. The results of the EEG, polysomnographic and EP recordings were compared to the clinical outcome. RESULTS AND CONCLUSION: In the three anoxic comas we observed BER abnormalities and the absence of SEP N20 associated with wide cortical lesions with brainstem extension. sleep recordings showed major alterations of the wake-sleep cycle without any improvement in M6. Abnormalities included loss of the normal REM-sleep pattern associated with alteration of NREM sleep and periods of increase in motor activity without EEG arousal. This sleep pattern appeared to be associated with involvement of the brainstem. In the two traumatic comas, alterations of the early cortical SEP responses were less severe and the BERs were normal. Some sleep spindles were observed as well as the persistence of sleep cycles in the first weeks post-coma. The combined use of EEG, EPs and polysomnography improved the outcome prediction in comparison with the use of just one modality. EPs and sleep recordings were far superior to clinical evaluation and to GCS in the appreciation of the functional status of comatose children. The reappearance of sleep patterns is considered to be of favorable prognosis for outcome of the coma state, as is the presence of sleep spindles in post-trauma coma. This study showed that EPs and sleep recordings help to further distinguish between patients with good or bad outcomes. ( info)

20/84. Agitation assessment in severe traumatic brain injury: methodological and clinical issues.

    The aim of this single case study was to evaluate the applicability of a graphic and statistical time-series analyses in the observation of an agitation disturbance in a 16-year-old patient who had sustained a severe traumatic brain injury. The agitation was measured using the Agitated Behaviour Scale. The experimental model was of the A-B type: phase A corresponded to the period of vegetative state, and phase B to the period following the reawakening from coma. The data were submitted to visual and statistical analysis by the split-middle trend line method, function of autocorrelation, and C statistic. The results show the different nature and frequency of the agitated behaviour during the vegetative state and after reawakening from coma. The application of a statistical analysis to establish whether the behavioural disturbance is random or a response to the environment allows the adoption of specific and potentially more efficacious treatments. ( info)
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