Cases reported "Agammaglobulinemia"

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1/36. Polio vaccine virus-associated meningoencephalitis in an infant with transient hypogammaglobulinemia.

    The case of an infant with transient hypogammaglobulinemia who developed meningoencephalitis, retinitis and sensorineural hearing loss is presented. The neurovirulent variant of the Sabin type 2 oral poliovirus vaccine was detected in cerebrospinal fluid and stool.
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ranking = 1
keywords = encephalitis, meningoencephalitis
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2/36. herpes zoster infection and Ogilvie's syndrome in non-Hodgkin's lymphoma with hypogammaglobulinemia.

    The case of a 43-year-old male with non-Hodgkin's lymphoma (stage IV B), and hypo-IgG and IgM, who developed acute colonic pseudo-obstruction or Ogilvie's syndrome during chemotherapy, is presented. The simultaneous occurrence of a unilateral segmental vesicular rash indicative of herpes zoster infection suggests an etiopathogenetic relationship between the colonic pseudo-obstruction and herpetic involvement of the motor celiac sympathetic ganglia. The rapid resolution of the abdominal dilation and the functional recovery from the colonic pseudo-obstruction after anti-viral therapy is also consistent with the diagnostic hypothesis.
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ranking = 0.00046000137101577
keywords = herpes
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3/36. Non-progressive viral myelitis in X-linked agammaglobulinemia.

    We report a 14-year-old boy with X-linked agammaglobulinemia (XLA) complicated by isolated non-progressive myelitis caused by Coxsackie virus B1. Despite the absence of immunoglobulin supplement and persistence of the virus for the initial 2 years, motor impairment did not show any progression for 3 years. This report shows that the prognosis of central nervous system infection in XLA is not determined by immunoglobulin levels alone, and that it is not always progressive or fatal. The balance between host immunity and the virulence of the causative virus may be involved in the prognosis of meningoencephalitis in XLA.
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ranking = 0.2
keywords = encephalitis, meningoencephalitis
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4/36. 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.
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ranking = 0.69436486832535
keywords = encephalitis
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5/36. carbamazepine-induced hypersensitivity syndrome associated with transient hypogammaglobulinaemia and reactivation of human herpesvirus 6 infection demonstrated by real-time quantitative polymerase chain reaction.

    Drug-induced hypersensitivity syndrome (HS) is a rare but severe disease with multiorgan failure. Many different precipitating factors have been reported, but the pathophysiology of HS remains unknown. However, the association of the human herpesvirus (HHV) family, particularly of HHV-6, has recently been reported in patients with HS. We report a 14-year-old boy who was diagnosed as having carbamazepine-induced HS based on the clinical course, laboratory data and results of drug-induced lymphocyte stimulation tests. In addition, the reactivation of HHV-6 was demonstrated by real-time quantitative polymerase chain reaction and by significantly increased levels of the specific antibody in his paired sera. Furthermore, transient hypogammaglobulinaemia was detected in the early stage of the disease. In addition, serum levels of interferon-gamma, interleukin (IL)-6, IL-5 and eosinophil cationic protein, which were increased on admission, decreased dramatically after steroid therapy. This is the first report of carbamazepine-induced HS associated with reactivation of HHV-6, transient hypogammaglobulinaemia, increased serum levels of inflammatory cytokines and activated eosinophils. This case might contribute to the understanding of the pathophysiology of HS.
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ranking = 0.0023000068550789
keywords = herpes
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6/36. magnetic resonance imaging in a patient with X-linked agammaglobulinemia and chronic meningoencephalitis.

    The case of a boy with congenital agammaglobulinemia is reported. In spite of regular immunoglobulin replacement therapy (fresh plasma transfusion from family donors--20 ml/kg/month), he developed chronic meningoencephalitis (ME). Besides characteristic clinical signs of ME, he also presented at cerebrospinal fluid analysis pleocytosis with lymphocyte predominance and class II cytomorphology, and delta and theta waves in the EEG. Computerized tomography showed dilatation of the ventricles and marked cortical fissures (sulci). magnetic resonance imaging showed a disease affecting white and gray matter. After diagnosis of ME, replacement therapy with Sandoglobulin (700 mg/kg every 2 weeks) was started. His condition gradually worsened, and coma and death occurred after a follow-up of 18 months. The etiological agent could not be identified.
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ranking = 1
keywords = encephalitis, meningoencephalitis
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7/36. Chronic enteroviral meningoencephalitis in agammaglobulinemia: case report and literature review.

    Chronic enteroviral meningoencephalitis is a well-recognized complication in patients with X-linked agammaglobulinemia (XLA). The majority of published cases refers to its occurrence in patients on no replacement therapy or on only intramuscular immunoglobulin. The advent of intravenous immunoglobulin (IVIg) in the early 1980s and its widespread use in XLA was thought to have virtually eradicated enteroviral meningoencephalitis in these patients. We describe the development of echovirus meningoencephalitis in an 11-year-old boy on regular IVIg replacement whose serum IgG levels were maintained at between 6 and 8 g/L (NR 6-13 g/L). Treatment with daily high-dose IVIg was commenced, with significant clinical improvement being noted within a few weeks in association with a reduction in blood-brain barrier permeability. The persistence of live virus, however, necessitated the use of intraventricular immunoglobulin. The virus proved resistant to two courses of specific intraventricular immunoglobulin and a 6-week course of oral ribavirin and eventually proved fatal 5 months after presentation. In view of the therapeutic uncertainties we have reviewed the use of immunoglobulin in the treatment of enteroviral meningoencephalitis over the past 6 years.
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ranking = 1.6
keywords = encephalitis, meningoencephalitis
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8/36. A case of X-linked agammaglobulinemia with progressive encephalitis.

    This report describes a case of agammaglobulinemia with progressive encephalitis. The patient was a 6-year-old male who was diagnosed as having Bruton-type agammaglobulinemia at age 6 months. After the diagnosis was made, he received monthly intravenous immunoglobulin replacement with a residual immunoglobulin g level of more than 400 mg/dL. At 5 years of age, he presented with symptoms of mental deterioration and gait disturbance. He had no history of infection of the central nervous system. Brain biopsy revealed CD8-positive T-cell infiltration with cortical damage, but no infectious agents were observed by either immunohistochemistry or virus isolation. Treatment with subcutaneous interferon-alpha and high-dose intravenous immunoglobulin was begun, and clinical symptoms improved within a month. Hence, patients with agammaglobulinemia should be carefully monitored for complications of the central nervous system even if there is no history of infection.
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ranking = 0.69436486832535
keywords = encephalitis
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9/36. Interstitial pneumonitis and coinfection of human herpesvirus 6 and Pneumocystis carinii in a patient with hypogammaglobulinemia.

    Human herpesvirus 6 (HHV-6) has occasionally been associated with cases of interstitial pneumonitis, mainly in individuals with impaired cellular immunity. Here we report for the first time severe interstitial pneumonitis with simultaneous HHV-6 and pneumocystis carinii infections in the lung tissue of a young patient with hypogammaglobulinemia.
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ranking = 0.0023000068550789
keywords = herpes
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10/36. Drug-induced hypersensitivity syndrome due to cyanamide associated with multiple reactivation of human herpesviruses.

    Drug-induced hypersensitivity syndrome (DIHS), characterized by serious adverse systemic reactions in addition to skin rash, has unknown pathogenesis. Its association with human herpesvirus (HHV), mainly HHV-6, has been reported recently. A 46-year-old Japanese man is described in whom a generalized eruption developed about 1 month after taking cyanamide, a drug for alcoholism. This was associated with the following manifestations: high fever, lymphadenopathy, facial edema, marked leukocytosis with eosinophilia and atypical lymphocytes, lymphocytopenia, liver and renal dysfunction, and low IgG level. He was treated with 8 mg betamethasone daily and his condition improved, but he needed low-dose corticosteroid for almost 1 year because of several episodes of recurrence. HHV-6, HHV-7, herpes simplex virus (HSV), and cytomegalovirus (CMV) specific IgG titers showed more than a four-fold rise sequentially. Significant numbers of copies of HHV-6 and HHV-7 dna were detected in the peripheral white blood cells by real-time polymerase chain reaction (PCR). HHV-6 and CMV dna were detected in the serum by nested PCR. A patch test for cyanamide was positive. The diagnosis of DIHS due to cyanamide, which has never been reported as a causal drug of DIHS, accompanied by reactivation of not only HHV-6, but also HHV-7, CMV, and HSV, was made. Disturbance of the immune system was suggested by the persistent low level of IgG, and consecutive viral reactivation may have participated in the prolonged course in this case.
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ranking = 0.0027600082260946
keywords = herpes
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