Cases reported "Limbic Encephalitis"

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1/7. limbic encephalitis and hyperactive foci on PET scan.

    Two cases of patients with paraneoplastic limbic encephalitis, difficult to control seizures, and unilateral hippocampal hypermetabolism on positron emission tomography (PET) are described. Two women aged 33 and 61 presented with uncontrolled complex partial seizures, profound memory loss and cognitive decline. One was later diagnosed with breast cancer and the other with lung cancer. Video-EEG on the first patient recorded multifocal sharp waves and bilateral independent seizure onsets. The second patient had no epileptiform discharges and bitemporal ictal onset, even though the clinical seizures suggested a right temporal onset. magnetic resonance imaging (MRI) was normal in both patients. PET scans obtained in the interictal state showed right hippocampal hypermetabolism in both patients. In the second patient, the lung cancer was irradiated with resolution of seizures and improvement of memory function. A PET scan six months later was normal. Subsequent seizure recurrence and worsening of memory led to the discovery of widespread metastases. limbic encephalitis should be considered in the differential diagnosis of intractable partial epilepsy, particularly if accompanied by severe memory loss and cognitive decline. Treatment of the underlying cancer may be lead to improved seizure control. Hippocampal hypermetabolism may be a common feature on PET, and may indicate subclinical seizure activity.
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2/7. limbic encephalitis investigated by 18FDG-PET and 3D MRI.

    Two patients with clinically probable or possible limbic encephalitis (LE) are reported, both cases with typical findings in clinical symptoms (severe neuropsychological deficits and complex partial seizures) and in routine magnetic resonance imaging (MRI) (hyperintense mesiotemporal lesions). Underlying malignancy was identified (rectal carcinoma) in one case but could not be detected in the other patient. The 2 patients were investigated by cerebral 18F-fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET) and 3-dimensional (3D) MRI, and abnormalities in metabolic activity were mapped using coregistration of spatially normalized PET and MRI. Highly significant focal hypermetabolism in bilateral hippocampal areas was found in both cases. The authors' findings support FDG-PET coregistered to 3D MRI as a potentially valuable additional tool in the imaging diagnostics of LE. Results are discussed with respect to the clinical symptoms and previously reported imaging findings in the disease.
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3/7. Paraneoplastic limbic encephalitis: case report.

    Paraneoplastic limbic encephalitis, which is also known as the remote neurological effect of cancer, is a rare neurological paraneoplastic disorder that usually presents with changes in mental status and behavioral abnormalities. Paraneoplastic limbic encephalitis had been diagnosed by clinical, laboratory and pathological studies in the pre-MRI era, but the diagnosis became much easier with MRI. We present MRI pictures of a case of paraneoplastic limbic encephalitis associated with small cell carcinoma of the lung.
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4/7. Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody.

    OBJECTIVES: To describe the clinical features and coexisting serum autoantibodies in seven patients with encephalitis associated with autoantibodies to alpha-dendrotoxin-sensitive voltage-gated potassium channels (VGKCs), and to compare this disorder with other autoimmune encephalopathies. methods: Clinical information was obtained from a retrospective review of medical records and telephone interviews. All autoantibody testing was performed in a single laboratory. RESULTS: The seven patients were examined for subacute cognitive and behavioral changes. seizures, usually temporal-onset complex partial type, were documented in six patients, and all seven patients had EEG abnormalities. None had symptoms or signs of neuromuscular hyperexcitability. One described hypersalivation. Four patients had additional autoantibody markers of neurologic autoimmunity (muscle acetylcholine receptor, striational, P/Q-type calcium channel, or GAD65), and two had thyroperoxidase antibodies. Two patients had a history of cancer: one had active prostate adenocarcinoma, and the second had a remote history of tongue carcinoma. Cranial MRI demonstrated mesial temporal lobe abnormalities in all patients. One patient improved spontaneously, and six were treated with IV methylprednisolone. Three improved remarkably with treatment. At follow-up evaluation, one had no cognitive deficits, four had mild persistent short-term memory dysfunction, and two had persistent disabling behavioral deficits. CONCLUSIONS: Voltage-gated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. The neurologic manifestations of this disorder are indistinguishable from paraneoplastic limbic encephalitis but are distinct from Morvan syndrome and Hashimoto encephalopathy.
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5/7. Pilomotor seizures and status in non-paraneoplastic limbic encephalitis.

    BACKGROUND AND AIMS: To describe an unusual clinical presentation of a patient with voltage-gated potassium channel Ab- positive, non-paraneoplastic limbic encephalitis. methods: We performed video-EEG monitoring, structural MRI, (18)F-FDG-PET, (1)H-MRS, neuropsychological testing and antibody serology. RESULTS: A 42-year-old male patient presented in an acute phase of non-paraneoplastic limbic encephalitis confirmed by MRI, with antibodies to voltage-gated potassium channels. His pilomotor status was pharmacoresistant to antiepileptic drugs, but responded to corticosteroid and azathioprine treatment. The MRI findings improved. The pilomotor seizures recurred when the immunosuppressive therapy was discontinued after 18 months. MRI at that time was consistent with hippocampal sclerosis. Complete seizure control was achieved after reintroduction of steroids. CONCLUSION: Pilomotor seizures were the predominant seizure type in this case of non-paraneoplastic limbic encephalitis. Immunosuppressive therapy may provide recovery including seizure control. However, long-term immunosuppression may be necessary to prevent relapse. Hippocampal sclerosis and chronic epilepsy might evolve as sequelae of limbic encephalitis.
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ranking = 2060.2423734448
keywords = status
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6/7. Anti-Hu-associated paraneoplastic limbic encephalitis presenting as rapidly progressive non-convulsive status epilepticus.

    We report a 68-year-old woman who developed refractory non-convulsive generalized status epilepticus secondary to anti-Hu antibodies, detected by immunofluorescence and confirmed by Western immunoblotting. The patient presented with rapidly evolving impairment in consciousness and electroencephalographic evidence of lateralized pseudoperiodic sharp-wave discharges. ataxia and sensory neuropathy developed within the first two weeks. To our knowledge, this is the first description of a very rapidly progressive non-convulsive status epilepticus of paraneoplastic origin. serum anti-Hu antibodies deserve to be considered among the investigations required in the evaluation of rapidly progressive epileptic syndromes even when little or no imaging abnormalities are found.
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keywords = status epilepticus, epilepticus, status
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7/7. Navigation around london by a taxi driver with bilateral hippocampal lesions.

    The time-scale of hippocampal involvement in supporting episodic memory remains a keenly debated topic, with disagreement over whether its role is temporary or permanent. Recently, there has been interest in how navigation by hippocampally-compromised patients in environments learned long ago speaks to this issue. However, identifying patients with damage that is primarily hippocampal, control subjects matched for navigation experience, and testing their in situ navigation, present substantial problems. We met these challenges by using a highly accurate and interactive virtual reality simulation of central london (UK) to assess the navigation ability of a licensed london taxi driver who had sustained bilateral hippocampal damage. In this test, patient TT and matched control taxi drivers drove a virtual london taxi along the streets they had first learned 40 years before. We found that the hippocampus is not required for general orientation in the city either in first person or survey perspectives, detailed topographical knowledge of landmarks and their spatial relationships, or even for active navigation along some routes. However, in his navigation TT was very reliant on main artery or 'A' roads, and became lost when navigation depended instead on non-A roads. We conclude that the hippocampus in humans is necessary for facilitating navigation in places learned long ago, particularly where complex large-scale spaces are concerned, and successful navigation requires access to detailed spatial representations.
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