1/66. 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 = hemiparesis (Clic here for more details about this article) |
2/66. 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 = hemiparesis (Clic here for more details about this article) |
3/66. rhabdomyolysis and acute renal failure associated with influenza virus type A.Two patients with rhabdomyolysis-induced acute renal failure due to influenza a virus infection are presented. Both had influenza symptoms, with high fever and severe muscular pain leading to walking problems. In addition, they were dehydrated due to vomiting and diarrhoea. Both had evidence of an ongoing influenza infection according to serological tests. Muscle injury due to the viral infection gave rise to rhabdomyolysis with efflux of myoglobin from the muscles, causing renal failure. In conclusion, influenza a virus infection can cause rhabdomyolysis accompanied by reversible acute renal failure.- - - - - - - - - - ranking = 0.0031883884103464keywords = muscle (Clic here for more details about this article) |
4/66. Unusual presentation and MRI findings in Rasmussen's syndrome.Rasmussen's syndrome is a chronic disorder characterized by uncontrollable focal seizures and eventually epilepsia partialis continua, ipsilateral hemiparesis, developmental arrest, and cerebral inflammation. Viral and autoimmune etiologies have been postulated. A patient is presented who illustrates the wide variability of clinical and radiographic presentations in this disorder. The patient is an 8-year-old female who developed intermittent facial twitching at 2 years of age that eventually progressed to epilepsia partialis continua. electroencephalography demonstrated clinical seizures that emanated from the right parasagittal area. Cranial magnetic resonance imaging revealed pronounced atrophy of the right caudate nucleus, globus pallidus, and putamen, with mild increased T2-weighted signal in the right striatum, without accompanying cortical atrophy. Ictal single-photon emission computed tomography revealed markedly reduced uptake in the right hemisphere that was maximum in the right basal ganglia. cerebrospinal fluid, blood, and urine collected for metabolic and immunologic screening and dna testing for a wide variety of disorders were all unremarkable. Neuropsychologic testing demonstrated difficulties in memory, attention, and calculation. Brain biopsy revealed mild microglial activation, rare glial nodules, and collections of lymphocytes and histiocytes, consistent with the clinical diagnosis of Rasmussen's syndrome. After a modified hemispherectomy, she demonstrated marked clinical improvement.- - - - - - - - - - ranking = 1keywords = hemiparesis (Clic here for more details about this article) |
5/66. Unusual encephalitic illnesses in a child with acute leukaemia in remission: possible role of measles virus and toxoplasma gondii.We report a girl who developed an encephalitic illness with visual loss after two years treatment for acute lymphoblastic leukaemia. The visual loss was found to be due to bilateral macular degeneration. She later developed radiological evidence of intracranial calcification and temporal lobe epilepsy. A second episode of encephalitis occurred when she had been off all antileukaemic treatment for three years and this left her with a right hemiparesis. Investigation suggested involvement with both measles virus and toxoplasma gondii as a cause for these illnesses.- - - - - - - - - - ranking = 1keywords = hemiparesis (Clic here for more details about this article) |
6/66. Toxoplasmic encephalitis in patients with acquired immunodeficiency syndrome--four case reports.Four patients, all males aged 40-64 years, presented with toxoplasmic encephalitis associated with human immunodeficiency virus (hiv) infection manifesting as nonspecific neurological deficits such as epilepsy or hemiparesis. magnetic resonance imaging showed single or multiple lesions with ring enhancement, mimicking metastatic brain tumor or brain abscess. Marked eosinophilia was noted in three patients. Two patients who received anti-toxoplasma chemotherapy in the early stage had a good outcome. However, the other two patients suffered rapid neurological deterioration and needed decompressive surgery, resulting in a poor outcome. toxoplasma diffusely infects the whole central nervous system from the early stage. The outcome for patients who needed emergency surgery was poor. Therefore, this rare but increasingly common infectious disease must be considered in the differential diagnosis of a patient with neuroimaging findings similar to those of metastatic tumor or brain abscess. Appropriate chemotherapy should be started immediately after hiv-positive reaction is identified in patients with single or multiple mass lesions with ring enhancement.- - - - - - - - - - ranking = 1keywords = hemiparesis (Clic here for more details about this article) |
7/66. Functional hemispherectomy: radical treatment for Rasmussen's encephalitis.Rasmussen's encephalitis is a progressive, debilitating disease that usually appears in the pediatric population but also may affect adults. Uncontrolled seizures, cognitive decline, and a progressive hemiparesis accompany this disorder. Treatment options are limited and consist of medical management, which is somewhat experimental and ineffective; the use of either intravenous antiviral agents or steroids; or surgical resection of the affected hemisphere. patients undergoing hemispherectomy for Rasmussen's encephalitis are complex. nurses caring for these patients require a high degree of knowledge and specialized skills. Patient outcome is highly affected by the knowledge and skill of the entire team of healthcare professionals needed to manage these patients.- - - - - - - - - - ranking = 1keywords = hemiparesis (Clic here for more details about this article) |
8/66. The pathology of human west nile virus infection.west nile virus (WNV) was identified by immunohistochemistry (IHC) and polymerase chain reaction (PCR) as the etiologic agent in 4 encephalitis fatalities in new york city in the late summer of 1999. The fatalities occurred in persons with a mean age of 81.5 years, each of whom had underlying medical problems. Cardinal clinical manifestations included fever and profound muscle weakness. autopsy disclosed encephalitis in 2 instances and meningoencephalitis in the remaining 2. The inflammation was mostly mononuclear and formed microglial nodules and perivascular clusters in the white and gray matter. The brainstem, particularly the medulla, was involved most extensively. In 2 brains, cranial nerve roots had endoneural mononuclear inflammation. In addition, 1 person had acute pancreatitis. Based on our experience, we offer recommendations for the autopsy evaluation of suspected WNV fatalities.- - - - - - - - - - ranking = 0.0031883884103464keywords = muscle (Clic here for more details about this article) |
9/66. Positive response to immunomodulatory therapy in an adult patient with Rasmussen's encephalitis.Rasmussen's encephalitis (RE) is a rare and progressive neurologic condition of uncertain etiology that typically has a childhood onset. The authors describe a 45-year-old woman with adult-onset progressive aphasia, right hemiparesis, severe drug refractory epilepsy, and left cerebral hemisphere atrophy. High-dose corticosteroids and plasmapheresis were not effective. She improved with high-dose therapy with human IV immunoglobulin.- - - - - - - - - - ranking = 1keywords = hemiparesis (Clic here for more details about this article) |
10/66. Paradoxical lateralization of parasagittal spikes revealed by back averaging of EEG and MEG in a case with epilepsia partialis continua.Our aim was to localize the generator site of parasagittal epileptiform discharges in a patient with epilepsia partialis continua (EPC) in the right leg. We examined a 32-year-old woman with EPC whose conventional EEG did not show any epileptic discharge. We performed the jerk-locked back averaging (JLA) of EEG and magnetoencephalography (MEG) to localize the dipole source of sharp transients. The myoclonic discharges in the right soleus muscle were used as a trigger pulse. JLA revealed consistent EEG and MEG sharp transients that coincided consistently and constantly preceded the myoclonic jerks. JLA of EEG demonstrated sharp waves paradoxically distributed over the vertex and right hemisphere. However, the estimated dipoles of MEG were localized in a restricted area in the primary leg motor area in the left hemisphere, which was closely located in the abnormal lesion on the brain MRI. JLA of MEG is considered to be a useful non-invasive method for localizing the epileptogenic area in EPC even when paradoxical lateralization of electroencephalographic discharges was noted.- - - - - - - - - - ranking = 0.0031883884103464keywords = muscle (Clic here for more details about this article) |
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