1/138. adult-onset MELAS presenting as herpes encephalitis.OBJECTIVE: To report an unusual presentation of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) manifested in late life with a clinical picture of herpes simplex encephalitis. DESIGN: Case report. SETTING: Clinical neurology department in a tertiary care hospital. CASE DESCRIPTION: A 55-year-old woman developed aphasia and delirium during ophthalmic herpes zoster infection treated with oral prednisone and ophthalmic steroids, which was followed by progressive cognitive decline without acute neurologic events for 5 years. At age 60, the patient presented with new onset of seizures, hemiparesis, and hemianopsia. Subsequently she developed cortical blindness, multiple traumatic soft tissue injuries from falls, acute psychosis, and severe dementia with periods of agitation. She died in a nursing home in March 1997, 6 years after initial presentation. RESULTS: magnetic resonance imaging scan of the brain showed hyperintensity on T2-weighted images involving temporal, parietal, and occipital lobes bilaterally as well as mild atrophy of brainstem and cerebellum. Single photon emission computed tomographic imaging showed hypoperfusion of temporal, parietal, and occipital lobes. Results of video electroencephalographic monitoring showed periodic lateralizing epileptiform discharges in temporal and occipital areas. The serum lactate level was normal in May 1996 and elevated in October 1996. The creatine kinase level was elevated with a 100% MM fraction in August 1991 and normal in March 1996. Results of repeated cerebrospinal fluid analyses indicated elevated protein levels. Analysis of dna was diagnostic of MELAS by mitochondrial dna point mutation at position 3243. The results of autopsy showed moderate cerebral, cerebellar, and brainstem atrophy with signs of infarction in temporal and parietal lobes bilaterally. CONCLUSIONS: The clinical presentation as well as age at onset of MELAS are highly variable. Onset of mitochondrial disorders can be provoked by febrile illness when there is mismatch between energy requirements and availability. In the differential diagnosis of herpes encephalitides, melas syndrome should be considered.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/138. 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 = 3keywords = brain (Clic here for more details about this article) |
3/138. Organ recovery from a donor with presumed viral encephalitis: a case report and review.This article reviews the pathophysiology of viral encephalitis, which is specifically infectious to transplant recipients, and discusses the potential infectivity of donors who had this virus. In addition, the case report demonstrates one center's experience in placing organs from a donor who was presumed--but not confirmed--to have viral encephalitis. When a patient with viral encephalitis is considered for organ donation, it is recommended that a brain biopsy be obtained prior to organ placement to identify the suspected virus or confirm the absence of any viral entity.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
4/138. Diaschisis in chronic viral encephalitis with Koshevnikov syndrome.The authors report a 61-year-old man with chronic viral encephalitis and Koshevnikov syndrome occurring 42 months after initial symptom of right hemiparesis. Serial computed tomography of the brain showed changes in the attenuation of the left temporal lobe lesion over time. Magnetic resonance images of the brain showed enlargement of left temporoparietooccipital lobes with cortical gyral enhancement on T1-weighted images following intravenous administration of gadolinium-DTPA. 99mTc-HMPAO single-photon emission computerized tomography showed increased radioactivity and hyperperfusion in the left temporoparietal region with paradoxically decreased local tissue perfusion at the contralateral right hemisphere. Follow-up magnetic resonance images of the brain 4 years later showed atrophy of bilateral cerebral hemispheres. We postulate that a "transcallosal diaschisis" with subsequent degeneration is a possible mechanism. A brain biopsy from the left temporal lobe lesion showed pictures compatible with viral encephalitis probably herpes simplex encephalitis.- - - - - - - - - - ranking = 1.3333333333333keywords = brain (Clic here for more details about this article) |
5/138. Operative treatment of tentorial herniation in herpes encephalitis.herpes simplex virus is the most common cause of acute viral encephalitis in children. Due to the variety of possible clinical manifestations the diagnosis is often overlooked in the early stages of the disease. Anti-viral therapy with acyclovir should be started whenever HSE is suspected. When there is further deterioration under virostatic therapy, a brain biopsy should be performed to verify the diagnosis. But even when the adequate medical therapy is established, massive brain edema and brain shift resulting in tentorial herniation can develop. Up to now the reported mortality of these patients is still around 30%. Here we report on a child with severe necrotizing herpes simplex encephalitis who developed severe tentorial herniation due to a right-sided mass lesion. The patient's status markedly improved after decompressive anterior temporal lobe resection. To our knowledge a similar case has not yet been reported in the literature. We suggest that anterior temporal lobe resection and decompressive craniotomy is of benefit in selected cases with tentorial herniation because both decompression and reduction of infectious material can be achieved.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
6/138. bk virus as the cause of meningoencephalitis, retinitis and nephritis in a patient with AIDS.BACKGROUND: The two widely spread human polyomaviruses, bk virus (BKV) and jc virus (JCV) establish latency in the urinary tract, and can be reactivated in AIDS. JCV might cause progressive multifocal leucoencephalopathy, but although up to 60% of AIDS patients excrete BKV in the urine there have been few reports of BKV-related renal and/or neurological disease in AIDS. OBJECTIVE: To report on an AIDS patient with progressive renal and neurological symptoms involving the retina. DESIGN: Case report. SETTING: Venhalsan, Soder Hospital, Stockholm, sweden. methods: The brain, eye tissue, cerebrospinal fluid, urine and peripheral blood mononuclear cells were analysed by nested PCR for polyoma-virus dna. Macroscopical and microscopical examination were performed of the kidney and brain post mortem. Immunohistochemical stainings for the two BKV proteins, the VP1 and the agnoprotein, were performed on autopsy material and virus infected tissue culture cells. RESULTS: BKV could be demonstrated in the brain, cerebrospinal fluid, eye tissues, kidneys and peripheral blood mononuclear cells. CONCLUSION: During 6 years, approximately 400 cerebrospinal fluid samples from immunosuppressed individuals with neurological symptoms have been investigated by PCR for the presence of polyomaviruses. BKV dna has, so far, only been found in the case reported here. Although reports of BKV infections in the nervous system are rare, there is now evidence for its occurrence in immunocompromised patients and the diagnosis should be considered in such patients with neurological symptoms and signs of renal disease. The diagnosis is simple to verify and is important to establish.- - - - - - - - - - ranking = 1.2361301051713keywords = brain, nervous system (Clic here for more details about this article) |
7/138. Interferon gamma (IFN-gamma) deficiency in generalized Epstein-Barr virus infection with interstitial lymphoid and granulomatous pneumonia, focal cerebral lesions, and genital ulcers: remission following IFN-gamma substitution therapy.A 26-year-old previously healthy woman developed granulomatous pneumonitis, encephalitis, and genital ulceration during primary Epstein-Barr virus (EBV) infection. EBV dna was demonstrated by polymerase chain reaction analysis of serum, lung tissue, and genital ulcer specimens. serology verified primary EBV infection. The patient lacked lymphocytes cytotoxic to autologous EBV-transformed B lymphocytes. No spontaneous or in vitro EBV-induced interferon gamma (IFN-gamma) production was evident in peripheral blood. The cells had normal IFN-gamma production when stimulated with staphylococcus aureus exotoxin A. In the bone marrow and peripheral blood, the number of large granular CD56 lymphocytes (natural killer cells) increased 39%-55%, but no CD4 or CD8 cell lymphocytosis was initially found. A partial clinical response was achieved with treatment with acyclovir, corticosteroids, and intravenous gamma-globulin. Because of persistent granulomatous central nervous system and lung involvement, subcutaneous IFN-gamma therapy was started but was discontinued after 3 months because of development of fever, pancytopenia, and hepatitis. This therapy initiated a complete clinical recovery, which occurred parallel to development of EBV-specific cytotoxic CD8 T lymphocytes and normalization of natural killer cell lymphocytosis. These findings provide evidence for an EBV-induced lymphoproliferative disorder due to a T lymphocyte dysfunction associated with a selective lack of IFN-gamma synthesis.- - - - - - - - - - ranking = 0.9365109955474keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/138. Fatal encephalitis due to nipah virus among pig-farmers in malaysia.BACKGROUND: Between February and April, 1999, an outbreak of viral encephalitis occurred among pig-farmers in malaysia. We report findings for the first three patients who died. methods: Samples of tissue were taken at necropsy. Blood and cerebrospinal-fluid (CSF) samples taken before death were cultured for viruses, and tested for antibodies to viruses. FINDINGS: The three pig-farmers presented with fever, headache, and altered level of consciousness. myoclonus was present in two patients. There were signs of brainstem dysfunction with hypertension and tachycardia. Rapid deterioration led to irreversible hypotension and death. A virus causing syncytial formation of vero cells was cultured from the CSF of two patients after 5 days; the virus stained positively with antibodies against hendra virus by indirect immunofluorescence. IgM capture ELISA showed that all three patients had IgM antibodies in CSF against Hendra viral antigens. Necropsy showed widespread microinfarction in the central nervous system and other organs resulting from vasculitis-induced thrombosis. There was no clinical evidence of pulmonary involvement. inclusion bodies likely to be of viral origin were noted in neurons near vasculitic blood vessels. INTERPRETATION: The causative agent was a previously undescribed paramyxovirus related to the hendra virus. Close contact with infected pigs may be the source of the viral transmission. Clinically and epidemiologically the infection is distinct from infection by the hendra virus. We propose that this Hendra-like virus was the cause of the outbreak of encephalitis in malaysia.- - - - - - - - - - ranking = 1.2698443288807keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
9/138. Acute encephalopathy and intractable seizures in a 10-year-old boy.We report a 10-year-old child with Robinow's syndrome who had a 2-week history of headaches and dizziness. On the day of admission, he developed a focal onset seizure with rapid secondary generalization. The seizures were intractable despite adequate doses of benzodiazepine, phenytoin, and phenobarbital, requiring a pentobarbital drip. Continuous electroencephalogram (EEG) monitoring showed persistence of the epileptiform discharges for 13 days. cerebrospinal fluid and brain biopsy studies were unrevealing. Mycoplasma pneumonia titers showed elevation of both immunoglobulins G and M that doubled during the tenth hospital day. High-dose methylprednisolone was begun, and within 12 hours of initiation the patient sat up and began to follow commands appropriately. The overall EEG background markedly improved. central nervous system mycoplasma pneumoniae infection should be suspected in patients with an encephalopathy of unclear etiology.- - - - - - - - - - ranking = 0.56946343850459keywords = brain, nervous system (Clic here for more details about this article) |
10/138. High-grade glioma mimicking acute viral encephalitis--three case reports.The clinical features of viral encephalitis consist of headache, fever, seizures and encephalopathy. We report three patients with high-grade gliomas presenting with encephalitic illnesses. The diagnosis of brain tumour should always be borne in mind if definite evidence for a viral infection is not obtained.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
| | Next -> |