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Results for “Limbic Encephalitis”

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At least 19 recordsLinked to original sources
PubMed1999

Small cell lung carcinoma associated with paraneoplastic limbic encephalitis.

Limbic encephalitis is an unusual presentation of paraneoplastic syndrome. We report a case of small cell lung carcinoma associated with limbic encephalitis. A 67-year-old man presented with convulsions, memory impairment, and neuropsychiatric disturbances as initial symptoms. Subsequently, small cell lung carcinoma was diagnosed by bronchoscopic biopsy. The cerebrospinal fluid studies, electroencephalography, and magnetic resonance imaging findings had distinctive features compatible with paraneoplastic limbic encephalitis. The neuropsychiatric symptoms improved significantly after six cycles of systemic chemotherapy and adjuvant radiotherapy, resulting in partial remission of the tumor. A follow-up computed tomography scan of the head showed no evidence of intracranial metastasis 7 months after the diagnosis of cancer. Limbic encephalitis may be an initial manifestation of lung cancer. Greater awareness for diagnosis and early treatment of the primary tumor offers the best chance for improvement in patients with lung cancer presenting with limbic encephalitis.

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PubMed2001

Potassium channel antibodies in two patients with reversible limbic encephalitis.

Limbic encephalitis (LE) is often associated with lung, thymic, or testicular tumours and antibodies to Hu, CV2, or Ma2 (Ta) antigens. In these cases, it generally has a poor prognosis. Here we describe two patients with symptoms of LE, negative for typical paraneoplastic antibodies, in whom antibodies to voltage-gated potassium channels (VGKC) were detected retrospectively in serial serum samples. Patient 1 had a thymoma recurrence, but in patient 2 no tumour has been detected in the years following presentation. Plasma exchange was effective in reducing VGKC antibody levels, with substantial improvement in mental symptoms in patient 1. In patient 2, the VGKC antibodies fell spontaneously over two years, with almost complete recovery of mental function. Although neither patient had obvious neuromyotonia at presentation, both showed excessive secretions. We suggest that patients with limbic symptoms and excessive secretions should be tested for VGKC antibodies, and, if they are present, prompt and effective immunosuppressive treatment should be considered.

Aged↗
PubMedDate not supplied

[Partial non convulsive epileptic status as initial presentation of limbic encephalitis].

INTRODUCTION: Limbic encephalitis is an unusual presentation of paraneoplastic syndrome, which includes among its symptoms seizures. CLINICAL CASE: We report a case with a rare presentation of limbic encephalitis as initial symptom of small cell lung carcinoma. A 69 year-old woman presented with partial non convulsive status epilepticus and neuropsychiatric disturbances. Chest radiography and computed tomography showed mediastinal lymphadenopathy and lung nodules. Subsequently, small cell lung carcinoma was diagnosed by lymph node biopsy. The cerebrospinal fluid study was normal. The electroencephalography and magnetic resonance imaging (MRI) findings had distinctive features compatible with temporo-limbic dysfunction. The anti-Hu antibodies were negative. The neuropsychiatric symptoms improved significantly after systemic chemotherapy and adjuvant radiotherapy. A serial follow-up MRI of the head showed no evidence of intracranial metastasis three months after the diagnosis of cancer. Limbic encephalitis may be an initial manifestation of lung cancer. Paraneoplastic limbic encephalitis is considered a remote effect of cancer commonly associated with anti-neuronal antibodies (anti-Hu) and small cell lung carcinoma. CONCLUSIONS: Status epilepticus could be an early sign of limbic encephalitis. The absence of anti-Hu antibodies does not rule out the presence of an underlying small cell lung carcinoma in patients with a clinical diagnosis of limbic encephalitis. Greater awareness for diagnosis and early treatment of the primary tumor offers the best chance for improvement in patients with lung cancer presenting with limbic encephalitis.

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PubMed2005

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.

Fatal Outcome↗
PubMed1993

[Schizophreniform psychosis in paraneoplastic limbic encephalitis].

Paraneoplastic limbic encephalitis is seldom mentioned in the psychiatric literature and premortem diagnosis is rare. Affective symptoms, agitation, and memory impairment are the core features, which can predate the diagnosis of carcinoma. We report the case of a patient with bronchial carcinoma, whose clinical picture could hardly be distinguished from that of endogenous schizophrenia.

Adult↗
PubMedDate not supplied

MR imaging of paraneoplastic limbic encephalitis.

Paraneoplastic limbic encephalitis is a rare disorder that has been previously diagnosed on clinical and pathologic grounds without good radiologic correlation. We present the case of a 42-year-old woman who developed gradually progressive limbic dysfunction 4 years after undergoing mastectomy for breast cancer. Although CT scans were normal, magnetic resonance (MR) imaging showed signal abnormalities in the medial portions of both temporal lobes, the amygdaloid nuclei, and the hypothalamus. An MR-guided temporal lobe biopsy confirmed the presence of encephalitis.

Adult↗
PubMed1990

Paraneoplastic limbic encephalitis: neuropsychiatric presentation.

Limbic encephalitis as a distinct clinicopathological entity is becoming increasingly familiar to neurologists. However, despite its classical clinical presentation of mental status changes and behavioral abnormalities, the disorder is not well known in the psychiatric literature and premortem diagnosis is rare. We recently participated in the care of a patient who spent two months on a psychiatric service and in whom a medical disorder was consistently suspected but not confirmed until autopsy revealed paraneoplastic limbic encephalitis and two primary systemic malignancies. A detailed neuropsychiatric description of this clinical entity is provided from presentation to autopsy with review of the literature.

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PubMedDate not supplied

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.

Carcinoma, Small Cell↗
PubMed2004

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.

Aged↗
PubMedDate not supplied

[Limbic encephalitis: a probably under-recognized syndrome].

Limbic encephalitis was identified as a clinicopathological entity in 1968. Up to a few years ago, 200 cases were described, most associated with lung cancer and more infrequently with other tumors. The recent identification of patients with this syndrome, idiopathic limbic encephalitis, who never develop cancer and have high titers of antibodies to voltage-gated potassium channels (VGKC) and an excellent response to immunosuppressive therapy, has extended the etiological spectrum and suggests that the syndrome may be under-recognized. The disorder, which develops in a few days or weeks, is characterized by the development of short-term memory loss, seizures, confusion and psychiatric features. The presence of symptoms beyond the limbic system is highly suggestive of a paraneoplastic origin. When limbic encephalitis is suspected, the following tests should be performed in order to demonstrate: a) involvement of the temporal lobes (EEG and brain MRI); b) presence of inflammatory abnormalities in the CSF, and c) the presence of onconeural antibodies or anti-VGKC. Once the diagnosis is confirmed by the clinical picture and MRI findings, treatment must be initiated without waiting for the antibody results because its negativity does not exclude the diagnosis. Detection of an onconeural antibody will confirm that the limbic syndrome is paraneoplastic and will help us to search for an underlying tumor and to predict possible response to the treatment. The recommended treatment is cycles of methylprednisolone (1 g/day for 3 to 5 days). Therapeutic response in the idiopathic limbic encephalitis is excellent and may be good in limbic encephalitis with anti-Ma2 or without onconeural antibodies. On the contrary, immunosuppressant treatment is not usually effective in limbic encephalitis associated to anti-Hu antibodies.

Humans↗
PubMed2004

[Paraneoplastic limbic encephalitis and lung cancer].

Paraneoplastic limbic encephalitis (PLE) is a disorder characterized by severe cognitive dysfunction and seizures. It is usually associated with small cell lung carcinoma. Diagnosis is difficult because clinical markers are often lacking, and symptoms usually precede the diagnosis of cancer. Serological detection of antineuronal antibodies can be useful. We describe a patient with symptoms of limbic encephalitis, negative for paraneoplastic antibodies, in whom lung cancer was detected.

Autoantibodies↗
PubMed1994

[Limbic encephalitis].

A case of limbic encephalitis diagnosed in a 56-year-old man with small-cell lung cancer is reported. Death occurred after 6 1/2 months due to cardiorespiratory insufficiency. Psychopathology, course and diagnostic findings of the syndrome are described, and pathogenetic hypotheses are discussed.

Brain↗
PubMed2001

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.

Acyclovir↗
PubMed2004

[Limbic encephalitis: etiology, pathogenesis, diagnosis and therapy].

Limbic encephalitis affects the mesial temporal lobes and is characterized by subacute onset of memory impairment, personal change, temporal seizures and autonomic nervous disorders. It can occur as viral infections, especially caused by Herpes simplex, paraneoplastic syndrome as a remote effect of cancer, CNS complication of well defined autoimmune diseases. Recently acute reversible limbic encephalitis which probably autoimmune mediated disorders has been reported. Anti-immunotherapy including steroids, plasma exchange and intravenous immunoglobulin often improves this condition and anti-voltage gated potassium channel (VGKC) antibody or anti-glutamate receptor epsilon2 antibody has been detected in some patients. Establishing the means of early detection of these antibodies as well as other characteristic paraneoplastic antibodies should now be the aim. Detection of neurotropic viral genomes in CSF by PCR is also important for differential diagnosis. As complete recovery of higher cerebral function is generally difficult, immunotherapy and anti-convulsants in addition to vitamin B, and acyclovir should be considered in an early stage of disease.

Humans↗
PubMed2004

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.

Amnesia↗
PubMed1993

Limbic encephalitis occurring in association with Alzheimer's disease.

Paraneoplastic limbic encephalitis is a rare cause of subacute dementia. A patient with limbic encephalitis and small-cell lung carcinoma is reported in whom the onset of subacute cognitive impairment was obscured by concurrent Alzheimer's disease. MRI revealed increased T2 signal in medial temporal lobes which corresponded to inflammatory pathology demonstrated at necropsy. High titres of antineuronal antibody (type II, anti-Hu) were present at death. Direct immunofluorescent staining of necropsy tissue revealed IgG bound to most remaining neurons in the temporal lobe. Antineuronal antibody screening and MRI are important in detecting limbic encephalitis.

Aged↗
PubMed1994

[A case of paraneoplastic limbic encephalitis in autoptic occult primary malignancy].

We report a case of limbic encephalitis in a 71-year-old male who presented with amnesia, confusion and disorientation. The patient's history of long standing alcoholism suggested the diagnosis of Korsakow syndrome. Autopsy, however, revealed a regional lymph-node metastasis of a small-cell bronchial carcinoma. No primary malignoma could be detected. These findings indicate that in cases of limbic encephalitis without detectable primary malignoma a paraneoplastic syndrome cannot be excluded. This has to be taken into consideration when discussing the etiology of limbic encephalitis (paraneoplastic versus virus-induced).

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