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)

Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disorder caused by persistent measles infection. Here, we report two neurologically handicapped cases presenting with atypical features of SSPE. Patient 1 who had mild mental retardation manifested acute encephalopathy with partial seizures and hemiplegia, mimicking encephalitis. He showed a fulminant course without myoclonia or a periodic electroencephalogram complex. Although SSPE is usually associated with an increased diffusion pattern, diffusion-weighted imaging of our patient showed decreased diffusion in the right hippocampus. Patient 2 with infantile hemiparesis presented with secondary generalized seizures, followed by asymettrical myoclonias involving the side contralateral to the hemiparesis. A periodic electroencephalogram complex was absent on the previously damaged brain regions. Our findings show that preexisting neurological disorders may modify the clinical or electrophysiological findings of SSPE, leading to atypical presentations. SSPE should be considered in the differential diagnosis of acute encephalopathy with lateralizing signs or unidentified seizures. Decreased diffusion resolution in diffusion-weighted-imaging may correlate with rapid clinical progression in SSPE.
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PMID:Atypical presentations of subacute sclerosing panencephalitis in two neurologically handicapped cases. 2013 78

We report the longitudinal history of a 48-year-old, right-handed woman with Rasmussen's encephalitis (RE) who presented with seizures and cerebral atrophy confined to the left hemisphere, as well as with bilateral uveitis, during her 21-year disease course. Neurological symptoms included recurrent partial seizures with secondary generalized convulsions, reduced visual acuity of the left eye with optic atrophy, right hemianopsia, right hemiplegia and aphasia. MRI T2-weighted images revealed progressive atrophy and high signal intensity lesions localized in the left cerebral hemisphere. An interictal electroencephalogram showed slowing of background activities to 4-7c/s and epileptiform discharges in the left hemisphere. Anti-glutamate receptor (GluR) epsilon2 IgG and IgM antibodies were detected in her serum. Our diagnosis was RE. Intravenous administration of high-dose methylpredonisolone immediately ameliorated her condition. Use of interferon beta-1b, as well as immunosuppressants, appeared to reduce seizure frequency, prevented exacerbation of her other central nervous system symptoms and slowed development of brain hemiatrophy. Her case is notable because it was complicated with bilateral uveitis and managed favorably by immunotherapy.
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PMID:Twenty-one-year course of adult-onset Rasmussen's encephalitis and bilateral uveitis: case report. 2044 55

Acute encephalopathy in childhood is frequently associated with common infections, especially in East Asia. Various types have been identified although many cases remain unclassified. Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease presenting impairment of cortisol biosynthesis. We report three CAH children with acute infection-related encephalopathy. They exhibited disturbed consciousness or seizures, which did not improve after glucocorticoid administration, accompanied by clinical and laboratory findings of adrenal insufficiency. Brain MRI disclosed various patterns of white matter lesions, suggesting different types of acute encephalopathy such as clinically mild encephalitis/encephalopathy with a reversible splenial lesion or hemiconvulsion-hemiplegia syndrome. Acute encephalopathy should be considered and brain MRI immediately performed when impairment of consciousness does not improve after intravenous glucocorticoid administration in CAH patients. Further research is required to elucidate the epidemiology and pathogenic mechanisms of acute encephalopathy in CAH.
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PMID:Clinical and MRI characteristics of acute encephalopathy in congenital adrenal hyperplasia. 2149 29

We investigated seizure, intelligence quotient (IQ), and neurological outcomes including the process of motor function recovery after functional right hemispherectomy in 3 children with Rasmussen's encephalitis (RE). Before the procedure, they were unable to walk, nor sit without support due to progressive worsening of left hemiplegia and relentless epilepsia partialis continua (EPC) of the left extremities, which were refractory to antiepileptic drug and immunological treatment. After functional right hemispherectomy, EPC completely disappeared, although complete left hemiplegia was sustained. However, they recovered up to being able to walk independently with assistance devices, and to have an ordinary life with family support within 1.5 to 5 months through rehabilitation. At the same time, the interictal EEG improved on the unaffected side of hemisphere, exhibiting a posterior alpha rhythm. Their IQ also improved, and they were able to attend school. Early functional hemispherectomy should be considered before patients with RE are left in a serious condition due to progressive worsening of hemiplegia and seizures refractory to the available treatment.
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PMID:[Three children with Rasmussen encephalitis showing marked improvement in daily life activity following the functional hemispherectomy]. 2194 44

The role of immunity and inflammation in epilepsy have long been suggested by the anticonvulsant activity of steroids in some infancy and childhood epilepsies. The role of fever and infection in exacerbating seizures due to possible proinflammatory molecules, the increased frequency of seizures in systemic autoimmune diseases like systemic lupus erythematous, and, recently, the detection of autoantibodies in some unexplained epilepsies reinforced the causal place of immunity and inflammation in epilepsies with unknown etiology. In this article, we summarize epilepsies where clinical and biologic data strongly support the pathogenic role of autoantibodies (e.g., limbic encephalitides, N-methyl-d-aspartate [NMDA] encephalitis) and epilepsies where immune-mediated inflammation occurs, but the full pathogenic cascade is either not clear (e.g., Rasmussen's encephalitis) or only strongly hypothesized (idiopathic hemiconvulsion-hemiplegia syndrome [IHHS] and fever-induced refractory epilepsy in school-aged children [FIRES]). We emphasize the electroclinical features that would help to diagnose these conditions, allowing early immunomodulating therapy. Finally, we raise some questions that remain unclear regarding diagnosis, mechanisms, and future therapies.
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PMID:Autoimmune and inflammatory epilepsies. 2294 22

Dengue is the most important mosquito-borne viral disease in the world and is caused by four distinct viruses (type 1 to 4) that are closely related antigenically. Infection by dengue virus may be asymptomatic or may lead to undifferentiated fever, dengue fever or dengue haemorrhagic fever. Recent observations indicate that the clinical profile of dengue is changing and the neurological complications are being reported more frequently. The neurological features includeheadache, seizures, neck stiffness, depressed sensorium, behavioural disorders, delirium, paralysis and cranial nerve palsies. Such neurological symptoms in dengue fever wereattributed to cerebral oedema, haemorrhage, haemoconcentration due to increasing vascular permeability, coagulopathy and release of toxic substances. Cerebral oedema, encephalitis-like changes (oedema and scattered focal lesions), intracranial haemorrhages as well as selective involvement of bilateral hippocampus in dengue infection have been reported previously on selective neuro-imaging but thalamic involvement is rare. We here report a case of a typical presentation of encephalopathy with left sided complete hemiplegia due to thalamic involvement in dengue infection.
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PMID:Dengue fever with unusual thalamic involvement. 2302 32

Rubella is a mild viral illness in children. Rubella encephalitis is an extremely uncommon complication of rubella affecting unvaccinated children, aged between 5 and 14 years. From May to June 2011, we observed 9 cases of rubella encephalitis diagnosed during an epidemic of rubella. All were previously healthy (8 boys and 1 girl). None of them had received rubella vaccine. The mean age was 11.6 years. The onset of neurological symptoms occurred within 1-5 days after the typical rush and was associated with seizures and altered consciousness in all cases. The presence of serum immunoglobulin M antibody against rubella virus was demonstrated in all patients. EEGs showed slow wave activity in all patients and brain MRI was normal in the 9 cases. Full recovery was obtained in all patients. However, 4 of them required intensive care unit referral. Acute encephalitis is an extremely rare complication of rubella. The main neurological findings are headache, ataxia, and hemiplegia. Epileptic seizure and altered consciousness are rarely observed. Rubella encephalitis is generally self-limiting with about 80% recovery rate with no sequelae. However, severe courses have been reported. These cases illustrated the potential severity of rubella and they should be prevented by encouraging widespread early childhood vaccination. In Tunisia, rubella encephalitis has been reported once previously and vaccination against rubella virus has only recently been included in the national vaccination program, prescribed only for adolescent females. Following this rubella epidemic, vaccination strategies in Tunisia have been revised.
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PMID:[Epidemic of rubella encephalitis]. 2383 99

Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection-related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear. Treatment is disappointing, particularly in FIRES, except for a response to ketogenic diet (KD) in half of patients. In IHHS, children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. Patients with FIRES develop refractory epilepsy with severe cognitive deficit affecting the temporal and frontal lobe functions. The role of inflammation is hypothesized with a vicious circle involving inflammation and seizure activity facilitated by brain maturation putting them under the concept of "acute encephalopathy with inflammation-mediated status epilepticus."
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PMID:FIRES and IHHE: Delineation of the syndromes. 2400 Oct 74

Low incidence of human immunodeficiency virus (HIV) infections and acquired immunodeficiency syndrome (AIDS) has been detected in Croatia so far. Toxoplasmic encephalitis (TE) is the most common opportunistic cerebral infection in AIDS patients and is highly responsive to antiparasitic chemotherapy, if treated at an early stage. We present the case of the brain biopsy confirmed as TE on a 36-year-old female patient who at admission presented with unconsciousness and a right hemiplegia. A MSCT was performed and two hypodense lesions were diagnosed. The patient's family initially denied the presence or history of any medical problem or infection. An MRI showed multiple ring-enhanced mass lesions. An infectologist required a brain biopsy to exclude cerebral lymphoma and multiple metastases. Pathohistological analysis suggested TE. Meanwhile, patient's blood samples were found to be HIV positive. The patient was transferred to University Hospital for Infectious Diseases in Zagreb, where she died 2 days following admission. The patient's family terminally confessed that the patient had been HIV positive for 10 years and had refused any treatment. Family's denial of infection as well as 'hiding information' concerning patient's health from physicians involved in her treatment caused a delay in proper on-time patient treatment. We would like to emphasize that TE must be considered as a differential diagnosis in patients presenting with multiple cerebral lesions, including patients without acknowledged past history of HIV infection. A stigma towards HIV infection and ignorance of the disease still exist and therefore hinders proper treatment.
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PMID:Difficulties with diagnosis and consequential poor outcome due to stigma of acquired immunodeficiency syndrome - a case report. 2409 Oct 82

A 28 years old female presented with headache, fever, altered sensorium and right side weakness for one week. She was febrile and drowsy with right sided hemiplegia and papilledema. Tuberculous or bacterial meningitis, tuberculoma and abscess were at the top of the diagnosis list followed by Herpes simplex meningo-encephalitis (HSE). MRI showed abnormal signal intensity of left temporal lobe without significant post-contrast enhancement and midline shift. CSF examination was normal, gram stain and Ziehl-Neelsen stain showed no micro-organism, or acid fast bacilli. CSF for MTB PCR was negative. PCR DNA for Herpes simplex 1 on CSF was detected. Acyclovir was started and the patient was discharged after full recovery. A high index of suspicion is required for HSE diagnosis in Pakistan where other infections predominantly affect the brain and HSE may be overlooked as a potential diagnosis.
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PMID:Herpes simplex encephalitis presenting with normal CSF analysis. 2416 94


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