Cases reported "Guillain-Barre Syndrome"

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1/8. Persistent paraneoplastic neurologic syndrome after successful therapy of Hodgkin's disease.

    Paraneoplastic neurologic syndromes may develop in Hodgkin's disease (HD). We describe three young female patients with neurological disorders, not explained by other causes, preceding diagnosis or relapse of HD. The lack of response of the paraneoplastic syndrome to successful treatment of HD among our three patients emphasizes the poor prognosis of longstanding paraneoplastic neurologic symptoms in HD.
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2/8. High-dose chemotherapy and APSCT as a potential cure for relapsing hemolysing AILD.

    Angioimmunoblastic lymphadenopathy with dysproteinemia (or dysgammaglobulinemia) (AILD) is a lymphoproliferative disorder with cytogenetic and molecular abnormalities characteristic of malignant T-cell lymphoma (angioimmunoblastic T-cell lymphoma -- AITL). We report the clinical course of a 58-year-old male patient with unusually aggressive AILD, including severe hemolysis and guillain-barre syndrome, who entered complete remission after CHOP therapy, but had a full relapse after 2 months. At relapse, treatment with high-dose chemotherapy followed by autologous peripheral stem cell transplantation (APSCT) with CD34 selected cells was shown to be successful. The patient is alive and disease-free 3 years after diagnosis and 32 months after APSCT. Considering the poor prognosis of the majority of patients with AILD, intensive treatment followed by APSCT, may be a subject for further studies.
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3/8. Overlapping guillain-barre syndrome and Bickerstaff's brainstem encephalitis associated with anti-GQ1b IgG antibody after herpes simplex virus infection.

    herpes simplex virus (HSV) is a rare, antecedent infectious agent in guillain-barre syndrome (GBS). We report a patient with overlapping GBS and Bickerstaff's brainstem encephalitis (BBE). The patient had a vesicular lesion on her nose. Antecedent HSV type 1 (HSV-1) infection was confirmed by isolation of the virus and detection of the presence of serum anti-HSV-1 IgM antibody during the acute phase. Her serum IgG had high anti-GQ1b antibody titer. External ophthalmoplegia has been noted in 2 of 4 reported cases of HSV-associated GBS. Herpetic brainstem encephalitis cases of poor prognosis are known, but only 2 cases of benign brainstem encephalitis secondary to HSV infection, in which there was acute ophthalmoplegia and clinical features consistent with those of BBE have been reported.
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4/8. Primary pleural effusion posttransplant lymphoproliferative disorder: Distinction from secondary involvement and effusion lymphoma.

    pleural effusion presentation of posttransplant lymphoproliferative disorder (PTLD) is relatively uncommon. Most examples of effusion-based PTLD have been secondary to widespread solid organ involvement, and are associated with an aggressive clinical course. We report on a case of primary effusion PTLD in a 70-yr-old male liver transplant recipient with a history of hepatitis b infection. Cytomorphologically, the pleural fluid specimen showed a monomorphous population of intermediate to large-sized transformed lymphoid cells, with irregular multilobated nuclear contours and readily identifiable mitotic figures. Flow cytometric immunophenotypic studies revealed a CD5-negative, CD10-negative, lambda immunoglobulin light chain-positive, monoclonal B-lymphocyte (CD19-positive/CD20-positive) population. The immunocytochemical stain for CD30 antigen was negative. in situ hybridization study for Epstein-Barr virus (EBV) early rna (EBER) and Southern blot analysis for EBV terminal repeat sequences were both positive. Southern blot analysis for human herpes virus-8 (HHV-8) was negative. No solid-organ PTLD was identified, and the cytologic results supported the diagnosis of primary effusion PTLD. immunosuppression was decreased, and 8 mo following the diagnosis of pleural fluid PTLD, the patient was stable and his pleural effusion had markedly diminished. Recognition of primary effusion PTLD and its distinction from PTLD secondarily involving the body fluids and from other lymphomas is important, since the behavior and prognosis appear different.
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5/8. GB syndrome with herpes simplex infection.

    G.B. Syndrome is an acute flaccid lower motor neuron (LMN) paralysis. The diagnosis is based on clinical presentation, course of illness with supportive investigations. This article reports an uncommon case of GB Syndrome caused by HSV infection in a 2 yr-9 mth-old child and have analysed the natural history, etiopathology, treatment and prognosis of the disease.
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6/8. Polyradiculoneuritis with myelitis: a rare differential diagnosis of guillain-barre syndrome.

    AIM: To describe the symptoms, signs, findings and prognosis in children suffering simultaneously from polyradiculoneuritis and myelitis. methods: Retrospective review of eight patients aged 2 to 13 years out of 210 patients with polyradiculoneuritis reported from 70 hospitals. Diagnostic criteria for polyradiculoneuritis were: flaccid paresis with loss of tendon reflexes, increased CSF protein and slowing of nerve conduction velocity. Criteria for myelitis were: severe and persistent bladder dysfunction, a sharply defined sensory level and/or evolving spastic paresis, with or without myelitic changes in spinal MRI. RESULTS: In the disease's earliest stage it was difficult to differentiate polyradiculoneuritis with myelitis from classical GBS. However, onset was often unusually rapid compared to GBS. Five patients developed a sensory level and seven suffered from severe bladder dysfunction. Four of the six children studied showed focal myelitic changes in MRI. All seven children with sufficient follow-up remained with residual paresis and significant long-term motor deficits. CONCLUSION: Due to its severe long-term prognosis, polyradiculoneuritis with myelitis must be differentiated from classical GBS. In the disease's early stage, the detection of a sensory level, severe bladder dysfunction and an unusually rapid onset can be helpful. The effect of high-dose corticosteroids is not yet clear. After the acute phase, most children require extended rehabilitation.
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7/8. Major surgery with guillain-barre syndrome: a case report.

    guillain-barre syndrome (GBS) is an acute demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia. The pathogenesis of GBS is unknown, but it is generally believed to result from aberrant humoral and cellular immune responses against components of the peripheral nervous system. The overall prognosis of GBS is quite good with approximately 85% of survivors making a good functional recovery. When a diagnosis of GBS has been made, appropriate treatment should be started as early as possible. This may include supportive care in intensive care units, ventilatory assistance, monitoring of blood pressure, fluid status, cardiac rhythm, nutritional supports and medical therapy. Our patient reached maximum deficiency 3 weeks after the onset of GBS. Full recovery took 8 months. The occurrence of GBS after major surgery is rare. We believe that major surgical stress may be the potential triggering factor for the occurrence of GBS in this case report.
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8/8. Adverse effect of polyvalent immunoglobulin in the treatment of guillain-barre syndrome.

    BACKGROUND: Acute polyradiculoneuropathy or guillain-barre syndrome is a neurological disease which may present with severe forms which have a poor prognosis. The patient's management requires multidisciplinary specialised care. morbidity has been reported to be significantly improved with initial therapy using high-dose intravenous immunoglobulin (IVIG). However, this therapy represent an immunological risk which has remained overlooked by clinicians in the majority of cases and is not clearly stated by the pharmaceutical companies. Therefore, the use of IVIG in the intensive care unit can cause some problems. CASE REPORT: A 32-year-old woman presented with clinical signs of Guillain-Barre syndrome. The patient received high-dose intravenous immunoglobulin (TEGELINE). Nine days after beginning therapy, she presented with severe immunological hemolytic anaemia; the IVIG was suspected as the cause. The blood cell count returned to normal approximately two months after the onset of the hemolytic syndrome. CONCLUSION: Despite the effectiveness of IVIG therapy in the management of various diseases, intensive care clinicians should be aware of possible major adverse effects which make a careful assessment of the patient necessary before treatment. It may also be important to consider the patient's ABO blood group before initiating IVIG treatment, particularly in patients bearing A and/or B blood group antigens.
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