Cases reported "Brain Damage, Chronic"

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1/32. Folinic acid-responsive neonatal seizures.

    We report three cases of folinic acid-responsive intractable neonatal seizures. All patients were born at term following normal gestation and delivery. In the first infant, seizures began on the 5th day of life and were unresponsive to phenobarbital, pyridoxine, and valproate, but stopped within 24 hours of initiation of folinic acid treatment at the age of 6 months. Her sibling had died at age 6 months with intractable seizures. In the second infant, seizures began in the 2nd hour of life. These were initially controlled with phenobarbital; however, at 3 months of age she developed status epilepticus refractory to anticonvulsants, steroids, and pyridoxine and she required repeated induction of pentobarbital coma. seizures stopped within 24 hours of starting folinic acid. seizures and encephalopathy were noted in the third infant on the 2nd day of life. These were controlled with phenobarbital, but at 8 weeks of age seizures recurred and were difficult to control despite the addition of phenytoin. Immediately after folinic acid was initiated the seizures stopped. Breakthrough seizures in all patients have responded to increases in folinic acid; two of the three remain on standard anticonvulsants. All patients have global developmental delay. Cranial magnetic resonance imaging in the second patient shows diffuse atrophy, and in the third patient shows increased signal on T2 images in the white matter of the frontal and parietal lobes. Analysis of cerebrospinal fluid from these patients using high-performance liquid chromatography with electrochemical detection has consistently revealed an as-yet unidentified compound, which can be used as a marker for this condition. We suggest that cerebrospinal fluid be analyzed for the presence of this compound and a trial of folinic acid be considered in neonates with unexplained early onset intractable seizures.
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2/32. 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.
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3/32. rehabilitation outcome in a patient awakened from prolonged coma.

    BACKGROUND: This article describes the rehabilitation of a patient recovering from a prolonged coma (defined as lasting longer than 4 weeks). The case is noteworthy because it exemplifies the possibilities and difficulties entailed in treating these patients, who are often regarded as too severely impaired to justify intensive rehabilitation efforts. CASE REPORT: The patient is a 28-year old Polish male, unmarried, who suffered serious closed head injuries in an automobile accident in April of 1999. He was in a comatose state for more than two months, with a GCS score of 5. When admitted for rehabilitation he was bedridden, with global aphasia, agraphia, limb apraxia, and executive dysfunction. The rehabilitation program developed for him is described in detail. RESULTS: Over the course of rehabilitation, which began in December 1999 and continues to this writing, the patient has regained locomotion capabilities (though with impairments), and his speech has improved considerably. The apraxia has largely resolved, and he is able to write his name and copy words. He is now capable of performing many activities of daily living. CONCLUSIONS: A comprehensive program of rehabilitation characterized by a strategic, heuristic approach is capable of achieving a good outcome even in very difficult cases, such as prolonged coma.
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4/32. Autosomal dominant acute necrotizing encephalopathy.

    OBJECTIVE: To define the clinical and biochemical abnormalities of an autosomal dominant form of acute encephalopathy. methods: The clinical details of 11 affected family members in comparison with 63 unaffected relatives were analyzed. RESULTS: Affected children become comatose after onset of a febrile illness. Outcomes include full recovery, permanent neurologic impairment, and death. Recurrences produce more severe impairments. Lesions of necrotizing encephalopathy of the thalamus and brainstem are present on autopsy and MRI. oxidative phosphorylation of intact mitochondria from a muscle biopsy shows loose coupling. Unaffected family members, including obligate carriers, share no clinical characteristics, demonstrating incomplete penetrance. CONCLUSIONS: Characteristic pathology and MRI findings define this disorder of autosomal dominant acute encephalopathy. Leigh syndrome and sporadic acute necrotizing encephalopathy share similarities but are distinct.
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5/32. Bilateral periodic lateralized epileptiform discharges in Mycoplasma encephalitis.

    status epilepticus and prolonged coma developed in two patients with respiratory tract infections caused by mycoplasma pneumoniae. Serial electroencephalography initially revealed bilateral, independent, periodic, lateralized epileptiform discharges. This pattern was replaced several days later by other electroencephalographic abnormalities.
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6/32. Delusional reduplication following closed-head injury.

    Somatic delusions following brain injury are not uncommon, and have been well documented in the literature. This study documents a case of somatic delusion which was seen in a patient following a head injury secondary to a motorcycle accident. Although perhaps not typical it serves to illustrate an interesting example of a somatic delusion following head trauma. On recovery from coma this patient reported the existence of a 'third arm' adjacent to the limb that had received the greatest impact in the accident. The patient was unreceptive to any counter-persuasions and in fact remained largely unconcerned about this addition to his anatomy. A thorough neuropsychological evaluation was carried out in an attempt to seek an explanation for this phenomenon. The results suggest that the phenomenon has at least a partly psychiatric aetiology rather than a purely neurological foundation.
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7/32. Acute necrotizing encephalopathy of childhood associated with influenza type B virus infection in a 3-year-old girl.

    Acute necrotizing encephalopathy of childhood represents a novel entity of acute encephalophathy, predominantly affecting infants and young children living in taiwan and japan. It manifests with symptoms of coma, convulsions, and hyperpyrexia after 2 to 4 days of respiratory tract infections in previously healthy children. The hallmark of acute necrotizing encephalopathy of childhood consists of multifocal and symmetric brain lesions affecting the bilateral thalami, brainstem tegmentum, cerebral periventricular white matter, or cerebellar medulla. The etiology and pathogenesis of this kind of acute encephalopathy remain unknown, and there is no specific therapy or prevention. The prognosis is usually poor, and less than 10% of patients recover completely. We report a 3-year-old previously healthy girl presenting with acute necrotizing encephalopathy of childhood associated with influenza type B virus infection, which resulted in severe neurologic sequelae. We also review the current knowledge of the clinical, neuroimaging, and pathologic aspects of acute necrotizing encephalopathy of childhood.
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8/32. Nonketotic hyperglycemic coma in toddlers after unintentional methadone ingestion.

    methadone overdoses are increasing in parallel with the increased frequency of opiate substitution therapy in adults. Although unintentional methadone intoxication in children is rare, it is becoming more frequently recognized. We report 3 cases of unintentional methadone overdose in toddlers who initially displayed central nervous system depression associated with severe nonketotic hyperglycemia and discuss the possible pathophysiologic mechanisms of an underrecognized symptom of opiate intoxication in young children.
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keywords = coma
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9/32. Cognitive rehabilitation: a neuropsychological speciality comes of age.

    In its first decade cognitive rehabilitation has emerged from infancy with the energy and much of the turmoil of youth. It has become an established speciality in brain-injury rehabilitation and has inspired many neuropsychologists to broaden their expertise beyond diagnosis and address their efforts to intervention. Restoration, although controversial, is an important and valid goal at the outset of cognitive rehabilitation, whereas the substitution of other means of goal achievement and environmental redesign are significant components of a comprehensive neuro-rehabilitative treatment programme. Efficacy is being demonstrated in many ways, ranging from increased patterns of use to controlled empirical studies. Few continue to raise the naive issue of whether there is any scientific basis for this neuropsychological speciality; instead, questions have become more focused on understanding the process of change to best serve human needs. In the next decade the refinement of current approaches can be anticipated, as well as the breaking of new ground. Neuropsychologists specializing in cognitive rehabilitation are likely to be turning their attention to underserved populations, such as those emerging from protracted coma, individuals suffering from 'progressive' neurological conditions, and persons whose brains were injured early in development. Scientific (theoretical, methodological and empirical) developments in cognitive psychology will help to delve more precisely into the underlying basic processes, including the sensory substrate. We will be challenged to address specific applications, such as advising people with known or suspected cognitive deficits who wish to drive.
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10/32. 'The ten pound note test': suggestions for eliciting improved responses in the severely brain-injured patient.

    The severely brain-injured patient may demonstrate a limited repertoire of responses. Anecdotal accounts suggest that stimuli of personal relevance, or of an unusual or innovatory nature, may sometimes elicit more meaningful responses. Two cases of severe acquired brain injury are described, in whom overall levels of responsiveness had been very low since coma ended. The use of stimuli with personal relevance provoked strong responses and presaged further behavioural change. The implications are discussed.
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