Cases reported "limbic encephalitis"

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1/46. 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. ( info)

2/46. limbic encephalitis and antibodies to Ma2: a paraneoplastic presentation of breast cancer.

    A patient with atypical medullary breast cancer is described who presented with symptoms of limbic encephalitis. The patient's serum and CSF contained antibodies that reacted with the nervous system and the tumour. These antibodies recognised Ma2, a neuronal protein related to paraneoplastic limbic and brainstem encephalitis in men with testicular tumours. This report highlights the importance of testing for paraneoplastic antineuronal antibodies in cases of unexplained limbic encephalitis and suggests screening for breast cancer in women with antibodies predominantly directed to Ma2. ( info)

3/46. limbic encephalitis not associated with neoplasm as a cause of temporal lobe epilepsy.

    OBJECTIVE: To describe four patients with temporal lobe epilepsy with limbic encephalitis unrelated to neoplasm. methods: The authors performed a retrospective evaluation of patient data obtained during presurgical evaluation, with additional CSF analyses, serum analyses, and histopathologic investigations. RESULTS: The patients shared the following clinical features: onset of the disease in young adulthood with subacute onset or exacerbation of frequent intractable temporal lobe seizures, verbal and visual memory deficits, and affective abnormalities. MRI showed variably extended areas of increased T2 signal in limbic structures and adjacent areas. In the histopathologic investigation, chronic inflammation was observed without evidence of a viral origin. There was no evidence of an underlying malignancy. CONCLUSIONS: Nonparaneoplastic limbic encephalitis should be included in the differential diagnosis of adult patients with temporal lobe epilepsy. ( info)

4/46. 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. ( info)

5/46. memory lost, memory regained: neuropsychological findings and neuroimaging in two cases of paraneoplastic limbic encephalitis with radically different outcomes.

    OBJECTIVE: To report two cases of paraneoplastic limbic encephalitis (PNLE) with similar clinical presentation, but dramatically different outcome and to highlight the role of neuropsychological and radiological evaluation in PNLE. methods: Both patients underwent an extensive battery of neuropsychological tests designed to document general intellectual function, anterograde verbal and visual memory, naming, knowledge and executive ability. In addition, structural (CT and MRI) and functional (HMPAO-SPECT) brain scans were performed. RESULTS: Both patients presented with fairly sudden onset of profound and persistent memory loss in the absence of other neurological symptoms. Their subsequently diagnosed small cell lung cancer was treated with a combination of radiotherapy and chemotherapy, leading to remission of the tumour. The memory of patient 1 recovered fully and he died from an unrelated cause 1 year later; neuropsychological testing showed a severe, but isolated, anterograde amnesia, brain MRI was normal and HMPAO-SPECT showed left medial temporal hypoperfusion. Patient 2 remained densely amnesic despite regression of her lung tumour; neuropsychological testing disclosed both anterograde and extensive retrograde amnesia together with more generalised cognitive deficits including anomia and executive impairments, MRI showed gross atrophy of the hippocampus and amygdala bilaterally, and HMPAO-SPECT showed pronounced frontal and temporal hypoperfusion. CONCLUSION: Complete remission from PNLE may occur and seems to be associated with pure anterograde amnesia without evidence of structural hippocampal damage in MRI. By contrast, cognitive deficits beyond severe anterograde amnesia and evidence of destructive medial temporal lobe pathology on MRI seem to be poor prognostic features. ( info)

6/46. Paraneoplastic limbic encephalitis, a complication of the testicular cancer.

    We report a case of paraneoplastic limbic encephalitis related to a testicular neoplasm. The syndrome is characterized by progressive amnesia and psychiatric disturbances resulting from an inflammatory reaction probably related to an immune response to the neuronal proteins expressed by tumors. It is usually associated with small cell lung cancer. In other cases, testicular cancer occurs more frequently than expected. Cranial magnetic resonance imaging may show increased signal intensity in the limbic cortex. Genitourinary examinations are sometimes inadequate. Testicular ultrasonography is recommended if no tumor is detected on the chest and abdominal computed tomography scan. The outcome of the treatment of the testicular cancer on the paraneoplastic syndrome is variable. ( info)

7/46. Paraneoplastic limbic encephalitis associated with bronchogenic carcinoma: a case report.

    Paraneoplastic limbic encephalitis is a rare clinical entity, associated most often with the oat cell carcinoma of the lung. Clinically, it presents with affective changes in personality, memory loss, confusional state, hallucinations, and seizures; with dementia being the common feature as the disorder progresses. Response to treatment is disappointingly poor. ( info)

8/46. limbic encephalitis associated with recurrent thymoma: a postmortem study.

    The authors report an autopsied patient with limbic encephalitis and recurrent thymoma. The immunohistochemical study showed selective depositions of immunoglobulin g on the neurons in the limbic system and the tumor cells of the recurrent thymoma. The immunoblotting study detected two types of antibodies that react with the human brain, rat brain, and rat thymus. ( info)

9/46. Human herpesvirus 6 limbic encephalitis after stem cell transplantation.

    central nervous system complications are common in stem cell transplant recipients, but selective involvement of the medial temporal area is unusual. The 5 patients reported here presented after stem cell transplantation with increased hippocampal T2 signal on magnetic resonance imaging and increased hippocampal glucose uptake on [F-18]fluorodeoxyglucose-positron emission tomography (FDG-PET) associated with short-term memory loss, insomnia, and temporal lobe electrographic seizure activity. The initial scalp electroencephalograms (EEGs) failed to detect seizure activity in these patients, although the memory dysfunction along with the magnetic resonance imaging and FDG-PET findings suggested subcortical seizure activity. However, extended EEG monitoring revealed repetitive temporal lobe electrographic seizure activity. Follow-up MRIs in 2 patients and postmortem findings on 1 patient suggested that hippocampal sclerosis had developed following the clinical syndrome. cerebrospinal fluid studies revealed the presence of human herpesvirus 6, variant B, dna in all of 3 patients who had lumbar punctures. Immunohistochemical staining for the P41 and P101 human herpesvirus 6 protein antigens showed numerous immunoreactive astrocytes and neurons in the hippocampus of 1 of the patients who died from other causes. Because of its subtle clinical presentation, this syndrome may be underrecognized, but can be diagnosed with appropriate magnetic resonance imaging techniques, EEG monitoring, and cerebrospinal fluid viral studies. ( info)

10/46. Varicella-zoster virus limbic encephalitis in an immunocompromised patient.

    A case of limbic encephalitis in a patient who had undergone prolonged immunosuppressive treatment with i.v. cyclophosphamide and oral prednisolone for a microscopic polyangeitis is reported. A brain MRI scan revealed symmetric mesial temporal lobe lesions. Studies of cerebrospinal fluid (CSF) revealed a positive PCR for varicella-zoster virus (VZV) dna in 2 separate samples. Owing to a delay in diagnosis, intravenous acyclovir was initiated only after 11 d of symptoms. PCR of CSF for VZV dna became negative on day 14 of treatment while brain lesions had resolved on subsequent MRI scans. limbic encephalitis is a novel form of VZV infection. When brain imaging is suggestive of limbic encephalitis in an immunocompromised patient, PCR of CSF for VZV dna should be performed, as early antiviral treatment may improve the outcome. ( info)
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