1/764. Improvement in adult-onset Rasmussen's encephalitis with long-term immunomodulatory therapy.OBJECTIVE: To study the immediate and chronic effects of high-dose, long-term human i.v. immunoglobulin (h i.v.Ig) therapy in two patients with advanced adult-onset Rasmussen's encephalitis (RE). BACKGROUND: Despite advances in our understanding of the autoimmune pathogenesis of RE, medical options for chronic treatment are limited. methods: In an open-label treatment trial, treatment started with monthly cycles of high-dose h i.v.Ig (0.4 g/kg/d for 5 days) followed by maintenance therapy (0.4 g/kg 1 day each month) after the patients' conditions began to improve. Outcome measures included clinical, psychological, functional, and laboratory assessments before and at relevant intervals throughout 1 year of treatment. RESULTS: In both patients, unrelenting pretreatment deterioration halted, and after this they displayed striking improvements in seizure control, hemiparesis, and cognition that produced useful recovery of function. Improvements were delayed until after 2 to 4 monthly cycles of high-dose h i.v.Ig and continued when patients switched to maintenance treatment. Their recoveries were accompanied by increased cerebral perfusion on interictal SPECT and suppression of inflammatory markers in CSF. CONCLUSIONS: h i.v.Ig can be a useful, possibly disease-modifying, long-term therapy for adult-onset RE that should be considered before radical surgery is performed. Because improvements can be delayed, we propose guidelines for intensive and prolonged trials of immunomodulatory therapy in adults with this syndrome.- - - - - - - - - - ranking = 1keywords = encephalitis (Clic here for more details about this article) |
2/764. A case of the subacute brainstem encephalitis.A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case.- - - - - - - - - - ranking = 1keywords = encephalitis (Clic here for more details about this article) |
3/764. Acute hemorrhagic leukoencephalitis in patients with acute myeloid leukemia in hematologic complete remission.The authors describe the cases of three patients affected by acute myeloid leukemia, in complete remission, who rapidly developed neurologic symptoms leading to death. Neither clinical characteristics, nor radiological or microbiological procedures, allowed an etiological diagnosis of the neurologic syndrome. Post-mortem examination of the brain showed both macroscopic and microscopic findings compatible with acute hemorrhagic leukoencephalitis. The difficulty in distinguishing this entity from other CNS disease-related complications (e.g. leukemia infiltration, drug toxicity, hemorrhages) should not lead to an underestimation of the true incidence of this complication. We believe that with more attention to the possibility of this complication there would probably be both a greater possibility of collecting clinical informations about the real impact of this dramatic disease and a stronger hope of finding the right treatment for it.- - - - - - - - - - ranking = 1keywords = encephalitis (Clic here for more details about this article) |
4/764. Acute measles encephalitis of the delayed type.An acute measles encephalitis with epilepsia partialis continua occurred three months after measles in a previously healthy child with no detectable immunological defect. Levels of measles antibodies in serum and cerebrospinal fluid were high, and tubular inclusions of the type seen in subacute sclerosing panencephalitis were found in nerve cell nuclei. A communicating high-pressure hydrocephalus developed in the later stage of the disease. This case demonstrates that measles encephalitis of the delayed type should systematically be considered in children with acute encephalitis.- - - - - - - - - - ranking = 1.6keywords = encephalitis (Clic here for more details about this article) |
5/764. plasma exchange in Rasmussen's encephalitis.The authors observed a 4-year-old girl who has Rasmussen's encephalitis. She started with frequent localized and generalized seizures. Standard antiepileptic treatment was almost ineffective. The frequency of the generalized seizures decreased, but the myoclonic jerks of the left part of the body persisted. An EEG showed partial status epilepticus. The results of the CT scan were normal. antibodies to viruses were absent from the blood and cerebrospinal fluid. An MR scan showed a T2-weighted hypersignal zone in the right frontal region. Intravenous bolus injections of corticosteroids and drips of immunoglobulins were inefficient, and we started plasma exchanges which have continued for 9 months. The clinical state stabilized, and the images on the MR scan improved, but the results of the EEG did not improve. The authors discuss the effect of the plasma exchange, the use of which is questionable in this disease.- - - - - - - - - - ranking = 1keywords = encephalitis (Clic here for more details about this article) |
6/764. Successful plasmapheresis in the not-so-benign Bickerstaff's brain stem encephalitis associated with anti-GQ1b antibody.A patient with Bickerstaff's brain stem encephalitis (BBE) associated with anti-GQ1b antibody developed coma, severe weakness, and respiratory distress. The patient required ventilatory support. After having failed to improve on steroids, she was treated with plasmapheresis. She improved concomitantly with the plasmapheresis treatment and made a complete recovery. BBE associated with anti-GQ1b antibody is generally considered to be benign, and specific treatments have not been established. The results with this patient suggest that the condition is not always benign, and plasmapheresis may be beneficial in this disorder.- - - - - - - - - - ranking = 1keywords = encephalitis (Clic here for more details about this article) |
7/764. herpes simplex encephalitis after brain surgery: case report and review of the literature.Intracranial infection after neurosurgical intervention most often is caused by bacteria. A rare case of fatal herpes simplex encephalitis after removal of a meningioma is described and similar cases reported in the literature are reviewed. Recent diagnostic tools, including detection of herpes viral dna sequences by polymerase chain reaction, complement clinical suspicion and facilitate mandatory early diagnosis, because herpes encephalitis, without rapid initiation of treatment, may lead to severe disability or death.- - - - - - - - - - ranking = 1.2keywords = encephalitis (Clic here for more details about this article) |
8/764. Imaging and laboratory investigation in herpes simplex encephalitis.A 14 day old baby presented with signs of an acute encephalitis. Clinically, herpes simplex encephalitis (HSE) was suspected. Early MRI and EEG were normal and there was rapid clinical improvement. A negative polymerase chain reaction (PCR) result on the initial CSF sample seemed to make HSE most unlikely. This diagnosis was subsequently proved after demonstration of specific antibody production using immunoelectrophoresis of the CSF. The child had extensive damage to brain tissue. The need for sequential analysis of CSF in making or refuting this diagnosis is illustrated.- - - - - - - - - - ranking = 1.2keywords = encephalitis (Clic here for more details about this article) |
9/764. small cell lung carcinoma associated with paraneoplastic limbic encephalitis.limbic encephalitis is an unusual presentation of paraneoplastic syndrome. We report a case of small cell lung carcinoma associated with limbic encephalitis. A 67-year-old man presented with convulsions, memory impairment, and neuropsychiatric disturbances as initial symptoms. Subsequently, small cell lung carcinoma was diagnosed by bronchoscopic biopsy. The cerebrospinal fluid studies, electroencephalography, and magnetic resonance imaging findings had distinctive features compatible with paraneoplastic limbic encephalitis. The neuropsychiatric symptoms improved significantly after six cycles of systemic chemotherapy and adjuvant radiotherapy, resulting in partial remission of the tumor. A follow-up computed tomography scan of the head showed no evidence of intracranial metastasis 7 months after the diagnosis of cancer. limbic encephalitis may be an initial manifestation of lung cancer. Greater awareness for diagnosis and early treatment of the primary tumor offers the best chance for improvement in patients with lung cancer presenting with limbic encephalitis.- - - - - - - - - - ranking = 1.8keywords = encephalitis (Clic here for more details about this article) |
10/764. Demyelinative brainstem encephalitis responsive to intravenous immunoglobulin therapy.We describe 2 patients with acute demyelinating brainstem encephalitis who were treated with intravenous immunoglobulin (IVIG). Both patients improved rapidly, concomitant with the course of therapy. To the best of our knowledge, these are the first reported cases of brainstem demyelinating lesions in children treated with IVIG. Our experience suggests that IVIG should be considered as first-line treatment for similar cases.- - - - - - - - - - ranking = 1keywords = encephalitis (Clic here for more details about this article) |
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