1/49. 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 = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
2/49. Progressive multifocal leukoencephalopathy in a child with hyperimmunoglobulin E recurrent infection syndrome and review of the literature.Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease due to infection with polyomavirus JC (JCV). PML occurs almost exclusively in immunocompromised patients, and although it has increased markedly in relation to AIDS, remains exceptional in children. We present the case of an immunocompromised child with hyperimmunoglobulin E recurrent infection syndrome (HIES) and pathologically-proven PML. HIES is a rare congenital immunodeficiency that to our knowledge has never before been reported in association with neurological complications. Following a recurrence of bronchopneumonia, the child's motor and cognitive functions deteriorated progressively in parallel with alterations on cerebral MRI. The neurological onset coincided with lymphocyte subset changes. PCR for JCV DNA did not detect the virus in CSF, and brain biopsy was required to secure the diagnosis. Antiviral treatment with cidofovir produced no benefit. autopsy revealed the typical neuropathological findings of PML which were associated with inflammatory eosinophilic infiltrate (a marker of HIES). In accordance with the few pediatric PML cases reported and here reviewed, the child died five months after neurological onset.- - - - - - - - - - ranking = 0.041037982549006keywords = immunodeficiency (Clic here for more details about this article) |
3/49. Surgical treatment of nocardial brain abscesses.OBJECTIVE: Nocardial brain abscesses are associated with significant morbidity and mortality rates. The optimal management remains unclear. We reviewed the surgical outcomes of patients treated with a relatively uniform policy at a single institution. methods: Eleven patients were treated at the Royal Adelaide Hospital between 1970 and 2001. Their clinical presentations, surgical treatment, and outcomes were reviewed. RESULTS: Clinical presentations most frequently involved focal neurological deficits (91%). Predisposing factors were identified for 63% of the patients. Nine patients were treated only with aspiration and long-term chemotherapy. Two patients underwent craniotomy and lesion excision. The majority of patients required either one or two procedures. There were no deaths in this series. Management complications were observed for three patients. Abscess aspiration was complicated by parenchymal hemorrhage and ventriculitis for one patient and temporary worsening of hemiparesis for two patients. CONCLUSION: Our results suggest that aspiration alone (repeated as clinically indicated) is a safe, efficacious treatment for the majority of patients with nocardial brain abscesses.- - - - - - - - - - ranking = 0.01249089563885keywords = aid (Clic here for more details about this article) |
4/49. Fatal subacute cytomegalovirus encephalitis associated with hypogammaglobulinemia and thymoma.Parathymic syndromes are systemic disorders that occur in association with thymoma. One such parathymic syndrome, hypogammaglobulinemia, was initially identified by Good in 1954 and has been referred to as Good syndrome. patients with this syndrome develop a variety of recurrent infections due to the associated immunodeficiency. We describe a patient with cytomegalovirus encephalitis associated with Good syndrome and discuss the pathologic findings present on autopsy. The possibility of a cytomegalovirus infection should be considered early in the evaluation of patients with Good syndrome if appropriate clinical symptoms are present.- - - - - - - - - - ranking = 0.041037982549006keywords = immunodeficiency (Clic here for more details about this article) |
5/49. Varicella-zoster virus encephalitis in acquired immunodeficiency syndrome: report of four cases.Four patients with acquired immunodeficiency syndrome, a 27-year-old female intravenous drug abuser and three males (two drug addicts aged 27 and 33 years and a 40-year-old homosexual) presented with a rapidly progressive encephalopathy. Two had generalized varicella-zoster virus skin infection, one had had a regressive thoracic zoster rash 7 months previously and one had no history of cutaneous eruption. Neuropathological examination revealed, in each case, multifocal necrotic changes with numerous, intranuclear Cowdry type A inclusion bodies in glial cells, endothelial cells, macrophages and neurons, within and around the lesions. These inclusion bodies were stained positively for varicella-zoster virus by immunocytochemistry and contained herpes virus nucleocapsids by electron microscopy. molecular biology using the polymerase-chain-reaction method demonstrated viral genome. In one case, zoster-induced non-inflammatory vasculopathy involved medium sized leptomeningeal vessels and was associated with circumscribed areas of cortico-subcortical infarction. In another case, varicella-zoster virus encephalitis was associated with human immunodeficiency virus encephalitis and a secondary cerebral lymphoma. Multinucleated giant cells expressing human immunodeficiency virus proteins in their cytoplasm, were found in the lymphomatous deposits and in the varicella-zoster virus necrotic lesions. In these latter lesions, Cowdry type A inclusion bodies could be seen in the nuclei of some multinucleated giant cells confirming previous observations of MGCs co-infected by HIV and CMV, and supporting the hypothesis that dna viruses interact with HIV, thus increasing its effect.- - - - - - - - - - ranking = 1.2807784869118keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
6/49. A common variable immunodeficient patient who developed acute disseminated encephalomyelitis followed by the Lennox-Gastaut syndrome.common variable immunodeficiency (CVID) is a primary disorder characterized by impaired antibody production. CVID patients may develop recurrent infections, autoimmune disorders, and malignant lymphomas, but to our knowledge, there is no report on CVID patients who develop acute disseminated encephalomyelitis (ADEM) or the Lennox-Gastaut syndrome. We describe a 1-yr-old female CVID patient with ADEM who evolutionally manifested the Lennox-Gastaut syndrome. She was admitted with convulsions and T2-weighted magnetic resonance imaging (MRI) revealed high-intensity areas in the right temporal lobe and the left fronto-parietal region but she became conscious soon. Her serum findings showed severe hypogammaglobulinemia and a follow up MRI revealed that these areas had diminished. Consequently, she was diagnosed as having CVID with ADEM. After 5 months, she fell to having tonic and absence seizures and we diagnosed her as having the Lennox-Gastaut syndrome from electroencephalograms (EEG) and the seizure pattern. She is now 7 yr old and her tonic seizures are controlled with valproic acid, clobazam, and immunoglobulin replacement therapy which is administrated every 2 wk. It is well known that the immune and neurologic systems have a close relationship. We suspect that a genetic defect in the immune system of our patient might also be associated with the neurologic disorders of ADEM and the Lennox-Gastaut syndrome.- - - - - - - - - - ranking = 0.041037982549006keywords = immunodeficiency (Clic here for more details about this article) |
7/49. An autopsy case of necrotizing ventriculo-encephalitis caused by cytomegalovirus in Hodgkin's disease.A 59-year-old Japanese woman with Hodgkin's disease developed progressive dementia and died of pneumonia. The autopsy revealed necrotizing ventriculo-encephalitis caused by cytomegalovirus (CMV) infection, which was confirmed by immunohistochemical and electron microscopic examinations. It is suggested that CMV ventriculo-encephalitis could occur not only in patients with acquired immunodeficiency syndrome, but also in other immunocompromised hosts.- - - - - - - - - - ranking = 0.23974050436275keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
8/49. diagnosis of first case of Balamuthia amoebic encephalitis in portugal by immunofluorescence and PCR.We report here the first Portuguese case of acute fatal granulomatous encephalitis attributed to balamuthia mandrillaris, initially thought to be a brain tumor, which had a progressive and fatal outcome. balamuthia mandrillaris is a free-living amoeba recognized as an uncommon agent of granulomatous encephalitis. Infections have been identified in immunocompromised hosts and in immunocompetent pediatric patients. Balamuthia infections are very rare, with only two reported cases in europe. The case presented here occurred in a previously healthy boy who died 5 weeks after the onset of the symptoms. No evidence of immunological deficiency was noted, and testing for human immunodeficiency virus antibodies was negative. The symptoms were initially thought to be the result of a tumor, but histopathologic examination showed evidence of amoebic infection. Immunofluorescence staining of brain tissue identified B. mandrillaris as the infectious agent. The diagnosis was confirmed with PCR by detecting Balamuthia DNA in formalin-fixed brain tissue sections. Despite initiation of empirical antimicrobial therapy for balamuthiasis, the patient died 3 weeks after being admitted to the hospital. No source of infection was readily apparent.- - - - - - - - - - ranking = 0.041037982549006keywords = immunodeficiency (Clic here for more details about this article) |
9/49. Fatal disseminated hemorrhagic toxoplasmic encephalitis as the initial manifestation of AIDS.A 28-year-old woman presented with progressive coma after being asymptomatic for 1 year after the demonstration of seropositivity for human immunodeficiency virus and high serum immunoglobulin g toxoplasma titers. Computed tomographic scanning showed multiple rounded cerebral hemorrhages and massive cerebral edema. Postmortem examination disclosed toxoplasma gondii cysts scattered throughout the brain. An overwhelming fatal toxoplasmosis associated with multiple cerebral hemorrhages may be the first presentation of the acquired immunodeficiency syndrome. We raise the possibility that frequent neuroradiological monitoring may be indicated in asymptomatic human immunodeficiency virus-infected patients with high immunoglobulin g toxoplasma titers.- - - - - - - - - - ranking = 0.32181646946076keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
10/49. cytomegalovirus (CMV) encephalomyeloradiculitis and human immunodeficiency virus (HIV) encephalitis: presence of HIV and CMV co-infected multinucleated giant cells.A 25-year-old homosexual male with AIDS presented with a cauda equina syndrome clinically suggestive of cytomegalovirus (CMV) myeloradiculitis. He was treated with ganciclovir with transient improvement of neurological signs and died 4 months after onset of neurological signs. Neuropathological examination revealed human immunodeficiency virus (HIV) encephalitis, CMV subependymal encephalitis and CMV myeloradiculitis. The latter was characterised by myelin loss, Schwann cell proliferation and presence of CMV early antigens in the nuclei of S-100 protein-positive cells in the spinal roots. In the subependymal regions, morphologically characteristic multinucleated giant cells, positive for CD68, contained early CMV antigens (E13) in their nuclei and hiv antigens (gp41 and p24) in their cytoplasm. The observation that HIV and CMV can co-infect the same cell in vivo raises the possibility of a direct synergistic interaction of both viruses at cell level. This suggests that CMV may play a role as a co-factor in the pathogenesis of HIV encephalopathy.- - - - - - - - - - ranking = 0.20518991274503keywords = immunodeficiency (Clic here for more details about this article) |
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