Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 52-year-old woman developed headache with fever followed after several days by a left hemiplegia, paralysis of the right IIIrd, Vth, and VIIth cranial nerves, and a right cerebellar syndrome. The CSF contained 48 white cells/mm3 and 0,80 g/l of proteins. Blood and CSF cultures were negative. In spite of an early massive antibiotic therapy, successive CT scans demonstrated the development of a voluminous rhombencephalic abscess. Clinical improvement occurred only after 1 month of treatment. The diagnosis of listeriosis, suggested clinically, was confirmed by elevated levels of antibodies to listeria Monocytogenes serotype 01 (1/80 to 1/1 280). Signs regressed slowly and hemiplegic sequelae persisted. A review of the literature demonstrated the rare nature of listerian abscesses in the CNS: in 6 of the 9 cases reported the patients were immunodepressed and the abscess was located in the cerebral hemispheres. The elective rhombencephalic lesion of listerian encephalitis may also apply to abscesses, which can develop in previously healthy subjects. The clinical picture is that of a solitary brain stem abscess with a fatal outcome whatever the nature of the germ. Van Gilder, Allen and Lesser (1974) published the first report of a case that recovered after surgical drainage. The present case is the only one of the 6 cases reported in the literature in which a favorable outcome was obtained by antibiotic therapy.
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PMID:[Large listerial abscess of the brain stem. Favorable effect of antibiotic therapy]. 687 77

Six children with acute cerebral insult, ranging in age from 3 days to 8 years, revealed periodic lateralized epileptiform discharges in their electroencephalographic recordings. Their etiologic factors were cerebral infarction, intracranial bleeding, purulent meningitis, acute infantile hemiplegia, and encephalitis. Each patient exhibited a different type of convulsive seizure. Computer tomography or magnetic resonance imaging revealed diffuse lesions covering the cerebral cortex and subcortical white matter in 2 patients, a lesion of the subcortical white matter in 1 patient, a linear lesion in the cortex and along the borderline between the cortex and the subcortical white matter in 1 patient, and localized lesions in the cortex and basal ganglia in 1 patient. There were findings indicating the disconnection of the cerebral cortex with deeper structures in 3 patients. The appearance rate of periodic lateralized epileptiform discharges increased at levels of consciousness from 5 to 7 on a pediatric modification of the Glasgow Coma Scale. At levels of consciousness from 8 to 14 and below 4, the rate was very low.
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PMID:Clinical significance of periodic lateralized epileptiform discharges in children with relation to level of consciousness. 798 89

Rasmussen's encephalitis is a progressive childhood disease of unknown cause characterized by severe epilepsy, hemiplegia, dementia, and inflammation of the brain. During efforts to raise antibodies to recombinant glutamate receptors (GluRs), behaviors typical of seizures and histopathologic features mimicking Rasmussen's encephalitis were found in two rabbits immunized with GluR3 protein. A correlation was found between the presence of Rasmussen's encephalitis and serum antibodies to GluR3 detected by protein immunoblot analysis and by immunoreactivity to transfected cells expressing GluR3. Repeated plasma exchanges in one seriously ill child transiently reduced serum titers of GluR3 antibodies, decreased seizure frequency, and improved neurologic function. Thus, GluR3 is an autoantigen in Rasmussen's encephalitis, and an autoimmune process may underlie this disease.
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PMID:Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis. 803 12

Twelve children with Rasmussen's encephalitis underwent hemispherectomy and have been followed for an average of 9.15 years. Our decision-making process leads to early, rather than late operation. Because hemiplegia is inevitable with or without operation, earlier operation may allow such children to resume a more normal life. We postulate that earlier operation may prevent some of the intellectual decline that accompanies the continued seizures and their treatment. In view of the variable pathologic findings and lack of evidence of a viral etiology, we suggest either use of the term "progressive unilateral encephalopathy of childhood" or "Rasmussen's syndrome" in recognition of its heritage.
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PMID:Progressive unilateral encephalopathy of childhood (Rasmussen's syndrome): a reappraisal. 833 May 74

Functional hemispherectomy, indicated for the control of pharmacologically refractory seizures, has been used at the Montreal Neurological Hospital since 1974. We have used this technique in 18 children suffering from intractable seizures secondary to conditions such as infantile hemiplegia, chronic encephalitis, head trauma, cerebrovascular accident, brain dysplasia and Sturge-Weber angiomatosis. None has developed superficial cerebral hemosiderosis often seen following the classical anatomical hemispherectomy. Eighty-two per cent (82%) of patients have been seizure-free since hospital discharge while another 11.5% have had at least 80% reduction in their seizure frequency. Most patients have shown an improvement in their intellectual capacity and sociability.
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PMID:Functional hemispherectomy in children. 847 13

Mycoplasma pneumoniae infection is no longer a benign condition it was originally thought to be. Many extrapulmonary manifestations affecting major organ systems like the central nervous system, cardiovascular system, haematological system, gastrointestinal system, musculoskeletal system and renal system have been described. Early recognition of these manifestations is often difficult and serological diagnosis may not be helpful. Three patients with large pleural effusions, encephalitis, hemiplegia, hepatitis, autoimmune haemolytic anaemia and renal failure are discussed to highlight the many varied presentations associated with this infection.
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PMID:Unusual manifestations of Mycoplasma pneumoniae infection in children. 855 96

Rasmussen's encephalitis (RE) is a progressive childhood disorder characterized by intractable focal seizures, hemiplegia, dementia, and inflammatory histopathology. The process is typically limited to one cerebral hemisphere. We report four patients with pathologically confirmed RE who were treated with repeated plasmapheresis. Three patients exhibited repeated, dramatic, transient responses to plasmapheresis, manifested by reduced seizure frequency and improved neurologic function. One patient exhibited marginal improvement after treatment with plasmapheresis. These observations indicate that circulating factors, likely autoantibodies, are pathogenic in at least some patients with RE and suggest that RE is an autoimmune disease. Plasmapheresis may be a useful adjunctive therapy in status epilepticus, and can also aid in assessment of residual function in the diseased hemisphere before surgical resection.
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PMID:Plasmapheresis in Rasmussen's encephalitis. 855 27

Subacute focal encephalitis of Rasmussen involves cortical inflammatory infiltration which may on occasion be bilateral. The presence of anti-Glu R3 antibodies in some patients suggests that an autoimmune process may be involved. Although a small number of patients respond transiently to immunoglobulins and the condition of some is partially improved by steroids, only hemispherectomy seems to produce prolonged relief of seizures. Subpial cortical transection may be useful if hemiplegia has not developed.
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PMID:Rasmussen's syndrome. 878 70

Rasmussen's encephalitis (RE) is a progressive, rare childhood disease characterized by severe epilepsy, hemiplegia, dementia, and inflammation of the brain. While one mechanism underlying the pathogenesis of RE has been hypothesized to be mediated by production of excitotoxic GluR3 autoantibodies to the AMPA receptor, other neuropathological etiologies have also been indicated. Whole-cell patch clamp recordings of GABA(A) receptor mediated responses were conducted in neurons acutely isolated from an RE patient, and compared to properties of non-focal human temporal cortical neurons. RE neurons appeared similar anatomically to control cortical neurons. Significant differences in GABAergic responses were evident between RE and control neurons. GABA was significantly more potent in RE than in control cortical neurons (EC50 of 13 microM vs 23 microM, respectively). In addition, the overall efficacy of GABA was significantly decreased in RE neurons, associated with a decrease in postsynaptic GABA current density in RE neurons (5.1 pA/microm2) in comparison to controls (9.2 pA/microm2). Augmentation of GABA responses by the benzodiazepine, clonazepam (CNZ), was significantly reduced in RE in comparison to control neurons (34% vs 99% augmentation at 100 nM). The RE-associated reduced functional efficacy and altered pharmacology of neuronal GABA(A) receptors is consistent with overall disinhibition in RE neurons, and could contribute to the generation of the severe epileptic activity evident in this disorder.
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PMID:Physiological analysis of Rasmussen's encephalitis: patch clamp recordings of altered inhibitory neurotransmitter function in resected frontal cortical tissue. 969 97

The patient, a 51-year-old male with a two year history of AIDS, was admitted to our hospital because of hemiparalysis and vomiting. The MRI study showed multiple lesions with ring-enhancement in the right basal brain area. Empirical therapy for toxoplasma encephalitis was started. After 64 days, the subsequent brain MRI showed deterioration. A 201Tl-SPECT study was performed and the findings were consistent with those of malignant lymphoma (ML). The patient was treated with 40 Gy of whole brain radiation, MRI showed partial response to this therapy, and clinical improvement was achieved. The definitive diagnosis of primary CNS lymphoma can be made only by brain biopsy, and many cases have been diagnosed at autopsy. The clinical and radiological findings of primary CNS lymphoma resemble toxoplasma encephalitis. An empirical therapy for toxoplasma encephalitis is recommended to avoid brain biopsy in these cases. The use of 201Tl-SPECT for the differential diagnosis of these diseases have been reported. Considering the poor prognosis of primary CNS lymphoma in AIDS, the application of 201Tl-SPECT before empirical therapy for toxoplasma must be important for appropriate treatment.
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PMID:[Successful treatment of primary CNS lymphoma diagnosed by 201thallium-single photon emission computed tomography (201Tl-SPECT) with whole-brain radiation therapy in an AIDS patient]. 1042 54


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