1/17. 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.- - - - - - - - - - ranking = 1keywords = seizure, epilepsy (Clic here for more details about this article) |
2/17. 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.- - - - - - - - - - ranking = 0.56394073356254keywords = seizure, epilepsy (Clic here for more details about this article) |
3/17. 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.- - - - - - - - - - ranking = 0.11566083545612keywords = seizure (Clic here for more details about this article) |
4/17. 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.- - - - - - - - - - ranking = 0.11566083545612keywords = seizure (Clic here for more details about this article) |
5/17. 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.- - - - - - - - - - ranking = 0.46264334182446keywords = seizure (Clic here for more details about this article) |
6/17. potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis.patients presenting with subacute amnesia are frequently seen in acute neurological practice. Amongst the differential diagnoses, herpes simplex encephalitis, Korsakoff's syndrome and limbic encephalitis should be considered. limbic encephalitis is typically a paraneoplastic syndrome with a poor prognosis; thus, identifying those patients with potentially reversible symptoms is important. Voltage-gated potassium channel antibodies (VGKC-Ab) have recently been reported in three cases of reversible limbic encephalitis. Here we review the clinical, immunological and neuropsychological features of 10 patients (nine male, one female; age range 44-79 years), eight of whom were identified in two centres over a period of 15 months. The patients presented with 1-52 week histories of memory loss, confusion and seizures. Low plasma sodium concentrations, initially resistant to treatment, were present in eight out of 10. brain MRI at onset showed signal change in the medial temporal lobes in eight out of 10 cases. Paraneoplastic antibodies were negative, but VGKC-Ab ranged from 450 to 5128 pM (neurological and healthy controls <100 pM). CSF oligoclonal bands were found in only one, but bands matched with those in the serum were found in six other patients. VGKC-Abs in the CSF, tested in five individuals, varied between <1 and 10% of serum values. Only one patient had neuromyotonia, which was excluded by electromyography in seven of the others. Formal neuropsychology testing showed severe and global impairment of memory, with sparing of general intellect in all but two patients, and of nominal functions in all but one. Variable regimes of steroids, plasma exchange and intravenous immunoglobulin were associated with variable falls in serum VGKC-Abs, to values between 2 and 88% of the initial values, together with marked improvement of neuropsychological functioning in six patients, slight improvement in three and none in one. The improvement in neuropsychological functioning in seven patients correlated broadly with the fall in antibodies. However, varying degrees of cerebral atrophy and residual cognitive impairment were common. Over the same period, only one paraneoplastic case of limbic encephalitis was identified between the two main centres. Thus, VGKC-Ab-associated encephalopathy is a relatively common form of autoimmune, non-paraneoplastic, potentially treatable encephalitis that can be diagnosed by a serological test. Establishing the frequency of this new syndrome, the full range of clinical presentations and means of early recognition, and optimal immunotherapy, should now be the aim.- - - - - - - - - - ranking = 0.11566083545612keywords = seizure (Clic here for more details about this article) |
7/17. Paraneoplastic limbic encephalitis: diagnostic relevance of CSF analysis and total body PET scanning.We report two cases of paraneoplastic limbic encephalitis (PLE) that differed in their clinical patterns, the underlying tumours, and the associated paraneoplastic antibodies. The first patient was a young adult male, with anti-MA-2 antibodies and testicular tumour. The clinical picture was restricted to limbic involvement. The second patient was a 56-year old, female heavy smoker; with seizures and depression, but also vertigo and diplopia. A low level of serum anti-Hu antibodies led to the detection of a small cell lung carcinoma by total body PET-scanning. In both cases, intrathecal synthesis of CSF oligoclonal IgG bands and of the corresponding paraneoplastic antibodies was demonstrated.- - - - - - - - - - ranking = 0.11566083545612keywords = seizure (Clic here for more details about this article) |
8/17. Paraneoplastic limbic encephalitis: ovarian cancer presenting as an amnesic syndrome.BACKGROUND: Paraneoplastic limbic encephalitis (PLE) is a rare neurologic manifestation of malignancy. Paraneoplastic limbic encephalitis typically presents with short-term memory loss, seizures, or other limbic system abnormalities. The majority of PLE cases are associated with lung and testicular cancer. CASE: We present the first case of PLE attributable to an epithelial ovarian cancer. The 58-year-old woman presented with rapidly progressing short-term memory loss and amnesia. The diagnosis of ovarian cancer was suspected on the basis of computed tomography scan findings. The patient showed dramatic clinical improvement within 2 weeks of debulking surgery and after the initiation of plasmapheresis and chemotherapy. CONCLUSION: Paraneoplastic limbic encephalitis is a rare complication of ovarian tumors that is potentially reversible with prompt surgical management of the primary tumor, plasmapheresis, and chemotherapy.- - - - - - - - - - ranking = 0.11566083545612keywords = seizure (Clic here for more details about this article) |
9/17. A pre-senile case of limbic encephalitis and cerebellar degeneration, with subacute onset of progressive dementia.In case a pre-senile patient presented subacutely progressive dementia, secondary dementia, such as paraneoplastic neurological syndrome (PNS), hypothyroidism, confusion, early phase of primary degenerative dementia and prion diseases are to be considered. It is a case of pathologically confirmed, and clinico-pathologically assessed limbic encephalitis with cerebellar degeneration. The patient was a 63-year old male, with a well followed up medical history of gastric cancer 8 years earlier. Four weeks after he presented himself at our hospital his memory and disorientation progressively declined. A neurological examination revealed gaze nystagmus, with potential secondary dementia. However, no abnormal findings were detected from systemic radiological examination, or from chemical analyses. Two months later, after the onset of the disease, he presented additional symptoms, including seizure, gait disturbance, and insomnia. On admission, neurological examinations revealed gaze nystagmus and progression of dementia; however, his thought process was relatively preserved. No paroxysmal synchronized discharge was seen on electroencephalogram. Chest x-rays showed an inflammatory infiltration. In spite of anti-biotic medication, he died due to respiratory failure. The autopsy was limited to the brain. Histologically, limited lymphocytic infiltration into the hippocampus through the entorhinal cortex, with marked neuronal loss and gliosis was observed. Neuronophagia, microglial nodules, and perivascular lymphocytic infiltration were also seen. Additionally, most of the purkinje cells in the cerebellum were lost, with Bergmann's gliosis and sparse lymphocytic infiltration. No tumor was observed in the brain. Pathological findings of the brain were compatible with paraneoplastic limbic encephalitis and cerebellar degeneration, though no neoplasm, clinically or pathologically, was detected in this patient. Consequently, it is suggested that when a senile patient presents sub-acute onset of progressive dementia, with a variety of neurological symptoms, paraneoplastic syndrome is to be taken into consideration, even if a tumor or an auto-antibody is not detected since the resection of the tumor is still the best therapeutic means. Otherwise immuno-suppressive and steroid therapies should be used.- - - - - - - - - - ranking = 0.11566083545612keywords = seizure (Clic here for more details about this article) |
10/17. Paraneoplastic limbic encephalitis.Paraneoplastic limbic encephalitis (PLE) is a rare neurological consequence of a variety of cancers, most commonly originating from lung, breast and testis. The aetiology is believed to be immune-mediated, caused by tumour-induced autoimmunity launching an attack against one's own central nervous system. The patient may present with amnesia, depression, anxiety, seizures and/or personality changes. The onset of these symptoms may precede the diagnosis of malignancy by a period of up to 2 years. The malignancy may be occult and unless the syndrome is recognised, it may fail to be detected. The diagnosis of PLE is suggested by the clinical picture, MRI evidence of mesial temporal lobe abnormality and CSF abnormalities such as the presence of oligoclonal bands. It may be further supported by the presence of paraneoplastic antibodies in the serum. immunosuppression has been tried in some cases but memory impairment is often irreversible. There are several case reports in the literature of paraneoplastic limbic encephalitis but few emphasise the resulting impact that this may have on the patient's quality of life and their carers. The accompanying amnesia is often far more distressing to the carers, who are aware of the limitations of treatment of the underlying malignancy. hospices offer the appropriate palliative environment for such patients as well as physical and psychological respite to the carers.- - - - - - - - - - ranking = 0.11566083545612keywords = seizure (Clic here for more details about this article) |
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