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1/71. 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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2/71. MRI demonstration of intracerebral cryptococcal granuloma.

    We report an intracerebral cryptococcal granuloma in a patient who presented with recent memory disturbance and deteriorating mental status followed by temporary loss of consciousness. To our knowledge, this is the first reported case of an intracerebral cryptococcal granuloma examined by a combination of conventional MRI, fluid-attenuated inversion-recovery and diffusion-weighted imaging and in which the surgical specimen was analysed histochemically.
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3/71. Influenza virus-induced encephalopathy: clinicopathologic study of an autopsied case.

    BACKGROUND: Rapid progressive encephalopathy with a high fever, consciousness loss and recurrent convulsions has been occasionally reported in children during influenza pandemics in japan since 1995. We examined a 2-year old girl with hemorrhagic shock and encephalopathy syndrome associated with acute influenza a virus infection (A/Nagasaki/76/98; H3N2), to answer several questions for which no histologic or virologic data exist. methods: A clinicopathologic study using immunohistochemical staining and viral genome detection by reverse transcriptase polymerase chain reaction (RT-PCR) was performed with this autopsied case. RESULTS: The virus antigen was positive in CD8 T lymphocytes from the lung and spleen. The virus infected a very limited part of the brain, especially Purkinje cells in the cerebellum and many neurons in the pons, without inducing an overt immunologic reaction from the host. The RT-PCR used for detecting the hemagglutinin gene demonstrated positive bands in all frozen tissues and cerebrospinal fluid taken at autopsy and not in samples obtained on admission. CONCLUSIONS: The pathologic change induced by the direct viral invasion cannot be responsible for all of the symptoms, especially for the rapid and severe clinical course of the disease within 24-48 h after the initial respiratory symptoms. Together with the rapid production of several inflammatory cytokines, the breakdown of the blood-brain barrier may induce severe brain edema and can be a major pathologic change for the disease. Any therapeutic strategy to control this multistep progression of the disease could be effective.
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4/71. cyclosporine-induced encephalopathy in a patient with relapsed acute myeloid leukemia treated with unrelated allogeneic bone marrow transplantation.

    cyclosporine (CSP) is the most frequently used immunosuppressive agent for prevention of graft versus host disease (GVHD) in allogeneic bone marrow transplantation (BMT). Some adverse effects such as hepatic and renal toxicity have been frequently encountered, but central nervous system (CNS) toxicity caused by CSP is rare. We report an adult male patient with acute myeloid leukemia who developed CSP-induced encephalopathy under treatment for allogeneic BMT from an unrelated donor. methotrexate and CSP were used for GVHD prophylaxis. Leukocyte and platelet engraftment were successfully achieved on days 21 and 24 after BMT, respectively. Abrupt onset of mental confusion and disorientation occurred on day 25, followed by a generalized tonic clonic seizure and consciousness disturbance. The whole blood CSP level was 160.65 ng/mL. Magnetic resonance (MR) imaging revealed high signal intensities in the bilateral occipital lobes with predominant involvement of the cortical areas. The patient recovered from the CNS toxicity, but with slight memory impairment, 6 days after CSP was discontinued. When patients receiving CSP treatment for allogeneic BMT develop mental confusion, consciousness disturbance, or seizure, CSP-induced CNS toxicity should be taken into consideration.
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5/71. gliosarcoma associated with a huge cyst--case report.

    A 55-year-old female presented with a unique case of gliosarcoma with a huge cystic component manifesting as loss of consciousness, left-sided hemiparesis, and anisocoria. Computed tomography demonstrated a large cyst in the right frontal lobe, and enhancement of the mural nodule after administration of contrast medium. Emergent operation was performed. Xanthochromic fluid was aspirated, and the tumor was resected. The histological diagnosis was gliosarcoma based on the presence of gliomatous and sarcomatous components. She underwent a second operation because of tumor regrowth 3 weeks after the first operation. The postoperative course was satisfactory during radiation therapy with 60 Gy and chemotherapy. The diagnosis of gliosarcoma was difficult to make preoperatively because of the neuroradiological findings similar to low-grade gliomas. gliosarcoma should be included in the differential diagnosis of huge cystic tumors.
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6/71. Reversible delayed leukoencephalopathy following intravenous heroin overdose.

    We present serial neuropsychological, magnetic resonance (MR) imaging and EEG changes in a case of widespread CNS myelinopathy due to intravenous heroin overdose complicated by a period of prolonged unconsciousness. Following recovery from the acute overdose, the subject had the delayed onset of akinetic mutism with urinary incontinence. Sequential formal neuro-psychological assessments over 9 months showed evolution from severe global cerebral dysfunction to moderate disturbance of frontal lobe function. Almost complete resolution of diffuse white matter signal changes, accompanied by the development of a degree of volume loss, was evident on serial MR imaging over the same period, and generalized arrhythmic delta-range slowing on the EEG evolved int o a near normal pattern.
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7/71. Reversible posterior leukoencephalopathy in a patient with minimal-change nephrotic syndrome.

    A 9-year-old boy with nephrotic syndrome was transferred to our hospital because of acute renal failure and disturbance of consciousness after high-dose methylprednisolone therapy. He developed severe headache, visual disturbance, and generalized seizures. Brain computed tomography (CT) scan revealed multiple, bilateral, low-density areas in the parieto-occipital lobes. magnetic resonance imaging (MRI) disclosed a high signal intensity area on T2-weighted images and a low signal intensity area on T1-weighted images in the same lesion. Follow-up brain CT scan and MRI, 2 weeks after the first studies, showed complete resolution of the abnormal lesions, which suggested the diagnosis of reversible posterior leukoencephalopathy syndrome (RPLS). hypertension and high-dose methylprednisolone administration to the patient in the nephrotic state may be causes of this uncommon syndrome in this case. This is the first report of RPLS in nephrotic syndrome with hypertension not associated with cyclosporine administration.
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8/71. marchiafava-bignami disease: two cases with favourable outcome.

    marchiafava-bignami disease (MBD) is a rare disorder of an unknown aetiology but strongly associated with alcoholism. MBD primarily affects the corpus callosum leading to confusion, dysarthria, seizures and frequently to death. Over 250 cases from all races and from almost all nationalities have been reported, most cases being alcoholics. We report two cases with a favourable outcome. magnetic resonance imaging (MRI) demonstrated a typical lesion of the corpus callosum, in both patients. The patients, a 44-year-old male and a 40-year-old female, presented with depressed consciousness and a variety of other symptoms, but finally made a reasonably good recovery leading to home discharge. To the best of our knowledge, only one additional case of MBD from scandinavia has been published. As alcoholism is a major public health problem in scandinavia, we assume that MBD is underdiagnosed and/or under-reported. Non-specific general symptoms and encephalopathy in an alcoholic may harbour undiagnosed MBD. We suggest that the incidence of MBD may be higher and its prognosis may be milder than generally believed.
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9/71. Benign occipital epilepsy mimicking a catastrophic intracranial event.

    OBJECTIVE: To describe the rare, dramatic, presentation of benign occipital epilepsy. methods: We describe three children who presented to the pediatric emergency department from 1992 to 1996 with a clinical picture of catastrophic intracranial event. RESULTS: The main signs and symptoms were loss of consciousness in all patients, apnea in two, hemiclonus in two, general hypertonicity in two, eye deviation in two, fixed dilated pupils in one, and decorticate rigidity in two. All underwent emergency intubation, brain scan, and lumbar puncture, and all were treated with antibiotics, in addition to antiviral drugs in two. Two patients were also treated for suspected increased intracranial pressure. Two patients recovered within a few hours and one within 24 hours of admission without any residual neurologic deficit. Electroencephalograms, done within 48 hours after the event, revealed the classic pattern of occipital epilepsy in two patients and bilateral occipital slow wave in one. A 3- to 5-year clinical and electroencephalographic follow-up supported the diagnosis. CONCLUSION: Benign occipital epilepsy in children can mimic a catastrophic intracranial event. electroencephalography, performed early in the Pediatric intensive care Unit, may avoid or shorten unnecessary and aggressive treatments such as hyperventilation, diuretic agents, and prolonged antiviral therapy.
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10/71. Hyperbaric oxygen for carbon monoxide poisoning-induced delayed neuropsychiatric sequelae.

    The clinical manifestations of delayed neuropsychiatric sequelae after carbon monoxide (CO) intoxication are variable. In addition, there is no specific therapy for these complications. Fortunately, these complications have occurred less frequently in recent years, probably due to the usage of hyperbaric oxygen (HBO) therapy. We report an 8-year-old boy who developed late psychiatric disturbances 2 days after full recovery of consciousness from initial CO intoxication. His neuropsychiatric symptoms included consciousness disturbance, motor dysfunction, chorea, aphasia and agnosias. He received HBO therapy at 2.0 barr for 60 minutes once a day for 7 consecutive days. Three weeks later, he was functioning normally with no neuropsychiatric symptoms. A literature review concluded that HBO may be effective in treating neuropsychiatric sequelae. Moreover, immediate administration of HBO during acute CO intoxication may prevent these complications.
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