Cases reported "Brain Diseases"

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1/144. Encephalopathy associated with haemophagocytic lymphohistiocytosis following rotavirus infection.

    A 2-year-old Japanese boy with a haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy which developed after rotavirus infection is described. The neurological symptoms consisted of coma, seizures and spastic quadriplegia. On therapy with steroids, etoposide and cyclosporin A, the patient recovered without any neurological deficits. The interferon-gamma levels in serum and CSF were elevated at onset of the disease but had returned to normal at the time of clinical remission. Brain MRI revealed diffuse white matter abnormalities and parenchymal volume loss. Proton magnetic resonance spectroscopy revealed elevated lactate in the abnormal lesions observed on MRI, indicating that macrophages not exhibiting aerobic metabolism had infiltrated the CNS. At the time of clinical remission, the white matter abnormalities and brain lactate had disappeared. These findings suggested that the neurological symptoms resulted from the overproduction of cytokines by activated T-cells and macrophages. The pathophysiology of a HLH associated encephalopathy was considered to be a local immune response within the CNS, because interferon-gamma can induce the expression of major histocompatibility complex class I and II antigens on glial cells in the CNS. CONCLUSION: Haemophagocytic lymphohistiocytosis associated encephalopathy should be considered early in the differential diagnosis of cases with acute onset neuropathy.
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2/144. cyclosporine-induced white and grey matter central nervous system lesions in a pediatric renal transplant patient.

    Major neurologic complications secondary to cyclosporine are well documented and are known to include confusion, cortical blindness, seizure, spasticity, paresis, ataxia and coma. Most previous reports attribute these to white matter central nervous system (CNS) lesions or white/grey matter border lesions. Many predisposing factors have been identified, including: elevated levels of cyclosporine, hypomagnesemia, hypocholesterolemia, aluminium toxicity, high dose steroids, hypertension and infection. However CNS events attributed to cyclosporine have been reported without any of these risk factors. We report a case of a child developing multiple white and grey matter thalamic and cortical lesions along with acute neurologic deterioration, and then review cyclosporine mediated CNS injury, including the roles of p-glycoprotein and cyclophilin.
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3/144. Successful use of alternate waste nitrogen agents and hemodialysis in a patient with hyperammonemic coma after heart-lung transplantation.

    BACKGROUND: Lethal hyperammonemic coma has been reported in 2 adults after lung transplantation. It was associated with a massive elevation of brain glutamine levels, while plasma glutamine levels were normal or only slightly elevated. In liver tissue, glutamine synthetase activity was markedly reduced, and the histologic findings resembled those of reye syndrome. The adequacy of therapy commonly used for inherited disorders of the urea cycle has not been adequately evaluated in patients with this form of secondary hyperammonemia. OBJECTIVE: To determine whether hemodialysis, in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy, would be efficacious in a patient with hyperammonemic coma after solid-organ transplantation. DESIGN: Case report. SETTING: A children's hospital. PATIENT: A 41-year-old woman with congenital heart disease developed a hyperammonemic coma with brain edema 19 days after undergoing a combined heart and lung transplantation. methods: Ammonium was measured in plasma. amino acids were quantitated in plasma and cerebrospinal fluid by column chromatography. The effectiveness of therapy was assessed by measuring plasma ammonium levels and intracranial pressure and performing sequential neurological examinations. RESULTS: The patient had the anomalous combination of increased cerebrospinal fluid and decreased plasma glutamine levels. To our knowledge, she is the first patient with this complication after solid-organ transplantation to survive after combined therapy with sodium phenylacetate, sodium benzoate, arginine hydrochloride, and hemodialysis. Complications of the acute coma included focal motor seizures, which were controlled with carbamazepine, and difficulty with short-term memory. CONCLUSIONS: The aggressive use of hemodialysis in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy may allow survival in patients after solid-organ transplantation. An acute acquired derangement in extra-central nervous system glutamine metabolism may play a role in the production of hyperammonemia in this illness that resembles reye syndrome, and, as in other hyperammonemic disorders, the duration and degree of elevation of brain glutamine levels may be the important determining factors in responsiveness to therapy.
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keywords = coma
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4/144. Pontine cryptococcoma in a nonimmunocompromised individual: MRI characteristics.

    The case of a pontine cryptococcoma in a nonimmunocompromised, previously healthy 16-year-old boy is presented. The patient had slowly progressive brainstem signs with right cranial nerves V, VII, and VIII palsies, and contralateral corticospinal and spinothalamic deficits. Magnetic resonance images (MRI) revealed, within the right pons, a 1-cm diameter round mass lesion, hypointense on T1-weighted images, hyperintense on T2-weighted images, and with rim enhancement after infusion of gadopentetate dimeglumine. This is the only report of the MRI findings in an isolated pontine cryptococcoma in an immunocompetent patient. Early recognition of this specific MRI pattern is essential, because complete recovery can be achieved with prompt antifungal treatment.
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keywords = coma
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5/144. Fatal chaetomium cerebritis in a bone marrow transplant patient.

    The number of opportunistic infections in the central nervous system (CNS) has been steadily increasing because of a rising number of immunocompromised patients. A rare form of CNS infection can be caused by chaetomium species, one of the largest genera of saprophytic ascomycetes. The CNS lesions in the present case were caused by chaetomium atrobrunneum. The main characteristic of almost all chaetomium species is presence of hairs or setae covering the ascomata. Microbiological studies are the only definitive way to correctly identify this fungal organism. The rapid evolvement of the cerebral infection suggests that the brain tissue provides a favorable environment for growth and proliferation of these fungi. This is the second documented case of a fatal brain abscess caused by chaetomium atrobrunneum, and the first case report in a bone marrow transplant patient.
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keywords = coma
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6/144. Cerebral herniation after lumbar puncture in sarcoid meningitis.

    A patient with chronic meningitis due to neurosarcoidosis became comatose within minutes of a lumbar puncture and died 24 h later. The diagnosis of neurosarcoidosis was made post mortem. Development of cerebral herniation may have been exacerbated by lumbar puncture. It was proposed that arachnoid villi dysfunction may have contributed to very high intracranial pressures in this patient, since post mortem examination revealed communication between the ventricles and outlet foramina of the fourth ventricle, and that herniation was in part due to an acute pressure differential caused by lumbar puncture.
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keywords = coma
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7/144. Bilateral optic neuropathy associated with diffuse cerebral angiomatosis in sturge-weber syndrome.

    Visual loss in patients with encephalotrigeminal angiomatosis or sturge-weber syndrome usually results from secondary glaucoma or from damage to the retrogeniculate pathways by the angiomatous lesions. Optic neuropathy has not been reported as a component of this syndrome. This report describes a patient who developed bilateral optic neuropathy with progressive visual loss associated with diffuse cerebral angiomatosis in the setting of sturge-weber syndrome.
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ranking = 1
keywords = coma
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8/144. influenza a virus encephalopathy with symmetrical thalamic lesions.

    During an epidemic of influenza A infection in japan, a 7-year-old boy was admitted to our hospital because of high fever, convulsions, coma, and liver dysfunction on the 2nd day of a cold-like illness. His serum CPK was markedly elevated, but there was no hyperammonaemia or hypoglycaemia. His CSF showed an increased protein level, but the cell count and glucose level were normal. CT and MRI of the brain showed symmetrical thalamic lesions, and he was diagnosed with acute necrotizing encephalopathy in childhood. He had a significant increased in antibodies to influenza A H1N1 in serum and CSF, but the CSF was negative for influenza virus using virus isolation and a polymerase chain reaction assay. CONCLUSION: Antibody production without detectable levels of influenza virus in cerebrospinal fluid suggests that virus infection occurred, but the virus did not replicate in sufficient numbers in his central nervous system. The thalamic lesion, the hallmark of acute necrotizing encephalopathy in childhood, may be initiated by a local virus infection and develop with subsequent local changes such as breakdown of the blood-brain barrier and the extravasation of blood.
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ranking = 1
keywords = coma
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9/144. Migration of silicone oil into the brain: a complication of intraocular silicone oil for retinal tamponade.

    PURPOSE: To report a case in which intravitreal silicone oil migrated along the intracranial portion of the optic nerve and into the lateral ventricles of the brain after the repair of a retinal detachment secondary to cytomegalovirus retinitis. methods: A 42-year-old man with acquired immunodeficiency syndrome (AIDS) developed a rhegmatogenous retinal detachment in his left eye secondary to a cytomegalovirus infection of the retina. The detachment was repaired using 5000 cs intraocular silicone oil for a long-term tamponade. Subsequently, the affected eye developed glaucoma, which was poorly controlled. Fifteen months after the retinal surgery, he developed a peripheral neuropathy that was thought to be AIDS related. Computed tomography and magnetic resonance imaging of the head were performed to investigate the neuropathy. RESULTS: The patient was found to have a foreign substance within his lateral ventricles that shifted with position and was identical with respect to its imaging properties to the remaining intraocular silicone oil. Additional material was found along the intracranial portion of his optic nerve. CONCLUSION: Under certain circumstances, intraocular silicone oil may migrate out of the eye, along the intracranial portion of the optic nerve, and into the lateral ventricles of the brain.
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ranking = 1
keywords = coma
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10/144. neurocysticercosis in pregnancy: a case initially diagnosed as eclampsia.

    BACKGROUND: neurocysticercosis is an infection of the central nervous system with the pork tapeworm's cysticercus. CASE: A 21-year-old Hispanic primigravida presented at 33 weeks' gestation with acute onset of mental status changes preceded by headaches and emesis. She was transferred comatose to our institution with a diagnosis of postictal state secondary to eclampsia. Upon arrival, the patient developed anisocoria, papilledema, posturing, and hypertension. neuroimaging showed an intraventricular cyst. The patient was treated with ventriculostomy, induction of labor, postdelivery shunting, albendazole, and prednisone. CONCLUSION: neurocysticercosis should be considered in the differential diagnosis of pregnant patients with coma and/or seizures, especially if the patient has emigrated from or traveled to an endemic area. albendazole, with shunt procedure, is the treatment of choice for intraventricular neurocysticercosis.
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ranking = 2
keywords = coma
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