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1/12. association of herpes simplex virus encephalitis and paraneoplastic encephalitis - a clinico-pathological study.

    A 57 year-old woman developed acute limbic encephalitis and brainstem dysfunction. Anti-HU antibodies were repeatedly detected in serum and CSF. Postmortem examination showed necrotic and hemorrhagic lesions in the temporal lobes characteristic of herpes simplex virus encephalitis, which was confirmed by immunocytochemistry, and Purkinje cell loss with proliferation of Bergman glia and myelin loss in the external aspect of the dentate nuclei characteristic of paraneoplastic encephalitis. PCR-assay performed on temporal tissue extracts was positive for HSV-1. There was no identifiable neoplasm. This unusual association raises the possibility of a link between the two diseases.
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keywords = encephalitis
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2/12. Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody.

    A woman developed brain stem encephalopathy in association with serum anti-Ma2 antibodies and left upper lobe lung mass. T2 weighted MRI of the brain showed abnormalities involving the pons, left middle and superior cerebellar peduncles, and bilateral basal ganglia. Immunohistochemical analysis for serum antineuronal antibodies was confounded by the presence of a non-neuronal specific antinuclear antibody. Immunoblot studies showed the presence of anti-Ma2 antibodies. A premortem tissue diagnosis of the lung mass could not be established despite two CT guided needle biopsies, and the patient died as a result of rapid neurological deterioration. The necropsy showed that the lung lesion was an adenocarcinoma which expressed Ma2 immunoreactive protein. Neuropathological findings included prominent perivascular inflammatory infiltrates, glial nodules, and neuronophagia involving the brain stem, basal ganglia, hippocampus and the dentate nucleus of the cerebellum. Ma2 is an autoantigen previously identified in patients with germ cell tumours of the testis and paraneoplastic brain stem and limbic encephalitis. Our patient's clinical and immunopathological findings indicate that this disorder can affect women with lung adenocarcinoma, and that the encephalitic changes predominate in those regions of the brain known to express high concentrations of Ma proteins.
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ranking = 0.45454545454545
keywords = encephalitis
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3/12. Paraneoplastic encephalitis associated with cystic teratoma is detected by fluorodeoxyglucose positron emission tomography with negative magnetic resonance image findings.

    The role of fluorodeoxyglucose (FDG) positron emission tomography (PET) as a functional imaging technique used in the evaluation of a variety of malignancies has been well known. Paraneoplastic encephalitis is a rare central nervous complication, which has been reported in some tumors. Traditionally, magnetic resonance imaging of the brain is performed to aid in its diagnosis. The authors report a case of paraneoplastic encephalitis, associated with cystic teratoma, which had positive FDG-PET findings but appeared normal on magnetic resonance imaging.
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ranking = 0.54545454545455
keywords = encephalitis
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4/12. Complicated paraneoplastic neurological syndromes: a report of two patients with small cell or non-small cell lung cancer.

    Paraneoplastic neurological syndromes are frequently associated in patients with small cell lung cancer (SCLC) and antineuronal antibodies are involved in the autoimmune mechanism. Multiple syndromes are sometimes complicated in a single patient with SCLC. However, little is known about non-SCLC-associated neurological manifestations. We report two patients with complicated paraneoplastic neurological syndromes. Patient 1 showed paraneoplastic limbic encephalitis (PLE), paraneoplastic sensory neuropathy (PSN) and lambert-eaton myasthenic syndrome (LEMS) associated with SCLC. Patient 2 developed opsoclonus-ataxia and probable PLE associated with non-SCLC. Analysis of various antineuronal antibodies revealed that anti-Hu and P/Q-type voltage-gated calcium channel (VGCC) antibodies were positive in Patient 1 but any antibodies were not in Patient 2. Brain MRI demonstrated high intensity signals in temporal lobes particularly on fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted images. These findings suggest that complicated paraneoplastic neurological syndromes occur in non-SCLC as well as SCLC and that unidentified antineuronal autoantibodies may underlie the pathophysiology.
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ranking = 0.090909090909091
keywords = encephalitis
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5/12. Calgary experience with west nile virus neurological syndrome during the late summer of 2003.

    BACKGROUND: Between August 25 and September 25, 2003 seven patients with west nile virus neurological manifestations were identified through the hospital neurology consultation services in Calgary, alberta, canada. Three of the seven patients were treated with interferon alpha-2b (IFN alpha-2b). In this report we document the clinical characteristics of these seven cases. methods: Clinical and laboratory information was obtained from a retrospective review of patient hospital and clinic charts. patients were included if they had serological evidence of west nile virus infection and had clinical evidence of aseptic meningitis, encephalomyelitis, cerebellar syndrome or motor neuronopathy. Three patients received a treatment course of three million units IFN alpha-2b, administered by subcutaneous injection once per day for 14 days. RESULTS: Four patients had cerebellar signs without change in consciousness, two had both encephalitis and neuromuscular weakness, and one patient had focal lower motor neuron arm weakness. The mean age was 52 (range 24 - 73). All patients had flu-like illness and fever as presenting symptoms and six had severe headaches. Two patients were immunocompromised prior to infection. Two patients with cerebellar signs (one with opsoclonus-myoclonus) improved spontaneously and exhibited only mild residual deficits on discharge. The other two patients with cerebellar findings developed brainstem involvement, one coinciding with and one subsequent to the cerebellar symptoms. Within one week of treatment with IFN alpha-2b these latter two patients showed marked improvement. One patient with encephalitis and neuromuscular weakness, was treated with IFN alpha-2b and subsequently recovered. INTERPRETATION: In this case review of seven patients, multiple neurological symptoms occurred in each patient and the neurological presentation was varied. Four patients had predominant cerebellar findings and one patient had opsoclonus-myoclonus, not previously reported. The marked improvement in three patients who received IFN alpha-2b raises preliminary optimism towards this potential treatment.
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ranking = 0.18181818181818
keywords = encephalitis
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6/12. Paraneoplastic neurologic syndrome and autoimmune addison disease in a patient with thymoma.

    A 48-year-old man with autoimmune addison disease developed the following paraneoplastic neurologic syndromes (PNNS): limbic encephalitis, opsoclonus/myoclonus, and sensorimotor and autonomic neuropathies. An anterior mediastinal mass detected on a chest computed tomographic scan was found on resection to be a noninvasive lymphocytic thymoma. The PNNS went into remission 1 year after the thymectomy. This is the first case of thymoma associated with autoimmune addison disease and PNNS to be described in the literature.
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ranking = 0.090909090909091
keywords = encephalitis
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7/12. Paraneoplastic recurrent multifocal encephalitis presenting with epilepsia partialis continua.

    We report a 46-year-old female patient in whom epilepsia partialis continua was the initial presentation of small cell lung cancer. magnetic resonance imaging revealed multiple, bilateral cortical lesions, which were originally misinterpreted and treated as brain metastases. Intracranial lesions and neurological symptoms remitted after corticosteroids, chemotherapy, and radiotherapy. After an asymptomatic interval of 18 months, neurological symptoms recurred with more extensive involvement of completely different parts of the central nervous system (limbic, brainstem and cerebellar structures) without any evidence of tumor recurrence. Both episodes showed a distinctive response to immunosuppressive therapy. The diagnostic challenges of the highly variable clinical presentations and therapeutic approaches to paraneoplastic multifocal encephalitis are discussed with relevant literature review.
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ranking = 0.45454545454545
keywords = encephalitis
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8/12. Multiple neurological syndromes during Hodgkin lymphoma remission.

    We report a young patient who developed a stiff man syndrome (SMS) long after remission of Hodgkin lymphoma. This patient is remarkable because he has had several other potentially autoimmune or paraneoplastic neurological syndromes including limbic encephalitis and demyelinating polyneuropathy which also occurred years after remission from hodgkin disease.
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ranking = 0.090909090909091
keywords = encephalitis
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9/12. Focal paraneoplastic limbic encephalitis presenting as orgasmic epilepsy.

    PURPOSE: To report orgasmic epilepsy as a manifestation of paraneoplastic limbic encephalitis in a patient with small cell lung cancer. CASE REPORT: A 57 years-old woman presented with 2 month history of daily spells that consisted of a sudden pleasure provoking feeling described 'like an orgasm' lasting for 30 s to 1 min. She was a heavy smoker and had noted recent weight loss. Bronchial biopsy, following the finding of a right lung mass, confirmed the diagnosis of small cell lung cancer (SCLC). Spells subsided after starting carbamazepine. The lung cancer was treated with chemotherapy and chest radiation therapy resulting in a complete radiologic response. RESULTS: Brain magnetic resonance imaging (MRI) revealed left temporal lobe area of increased signal on T2 and FLAIR sequence. T1-weighted images after contrast administration demonstrated a circumscribed area of enhancement in the left anterior medial temporal lobe. Electroencephalogram (EEG) showed focal left mid-temporal sharp waves and intermittent slowing. Anti-Hu antibodies were detected in her serum supporting a diagnosis of paraneoplastic limbic encephalitis as the cause of her orgasmic epilepsy. The patient has been followed for 2 years after treatment without tumor recurrence or neurological deterioration. CONCLUSION: Orgasmic epilepsy is another mode of presentation of paraneoplastic limbic encephalitis leading to the diagnosis of an occult SCLC. EEG and MRI findings suggest that in this case the seizures originated from the left hemisphere. It is possible that early recognition and treatment of the SCLC will improve the prognosis of this neurologic entity.
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ranking = 0.63636363636364
keywords = encephalitis
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10/12. BR serine/threonine kinase 2: a new autoantigen in paraneoplastic limbic encephalitis.

    We describe a new antigen, BR serine/threonine kinase 2 (BRSK2), identified by an antibody present in the serum of a patient with limbic encephalitis and small-cell lung cancer (SCLC). Patient's serum immunolabeled the neuronal cytoplasm and, less intense, the neuropil of rat brain but did not immunoreact with other rat tissues with the exception of testis. Immunoblots of rat brain homogenate identified several immunoreactive bands in the range of 88-82 kDa and a weaker broad band of 47-43 kDa. Probing a rat hippocampus expression library with the patient's serum resulted in the isolation of BR serine/threonine kinase 2 (BRSK2), a protein (also know as SAD1B kinase) preferentially expressed in the brain and testis and implicated in neuronal polarization as well as synaptic development. Eluted IgG from the BRSK2 clone gave a similar immunolabeling than the patient's serum by immunohistochemistry and immunoblot of rat brain and testis. BRSK2 antibodies reacted with two SCLC from patients without paraneoplastic neurological syndromes. No anti-BRSK2 antibodies were found in the serum of 50 patients with SCLC without PNS, 19 with limbic encephalitis without onconeural antibodies, 50 with anti-Hu antibodies and several paraneoplastic neurological syndromes, including 14 with limbic encephalitis, and 160 with a variety of non-paraneoplastic neurological syndromes. Our study suggests BRSK2 may be an autoantigen involved in the pathogenesis of SCLC-associated limbic encephalitis.
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ranking = 0.72727272727273
keywords = encephalitis
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