Stato di male epilettico sintomatico di encefalite limbica con anticorpi anti-canale del potassio (VGKC-Ab): Case report. / Tramacere, L.; Colombo, A.; Molini. Ambos os casos apresentaram quadro clinico típico de encefalite límbica associada a CDFBs e exame do LCR sem alterações. Nenhum caso associou-se à. Autoimmune encephalitis, also known as autoimmune limbic encephalitis, is an antibody-mediated brain inflammatory process, typically involving the limbic.

Author: Arazahn Matilar
Country: Belize
Language: English (Spanish)
Genre: Career
Published (Last): 3 November 2008
Pages: 444
PDF File Size: 14.86 Mb
ePub File Size: 11.45 Mb
ISBN: 708-6-23726-814-3
Downloads: 74719
Price: Free* [*Free Regsitration Required]
Uploader: Vuktilar

A manifestation of systemic lupus erythematosus in the central nervous system. Autoimmune limbic encephalitis ALE associated with systemic lupus erythematosus SLE is a rare entity with few reports in the literature to date.

In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment RITbut the pathogenesis of this association envefalite poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active Systemic Lupus Erythematosus disease SLE and past medical history of cancer endometrial adenocarcinoma in and papillary urothelial carcinoma in with curative treatmentfollowed over a one-year period.

We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening.

Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems such as hematologic during the period.

However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies. Limbic encephalitis LE is a rare encefaliye syndrome that selectively affects the structures of the limbic system. In addition, there may be different degrees of involvement in extra-limbic-system tissues such as the cerebellum, brainstem and thalamus.

The importance of recognizing faciobrachial dystonic seizures in rapidly progressive dementias.

InBrierley et al. Although it is still considered a classical paraneoplastic syndrome, its limbicaa with autoimmune disease encefalitr been extensively studied. The discovery of these autoimmune disorders has changed the diagnostic approach to clinical problems as diverse as catatonia, subacute memory disturbance, as well as limbic encephalitis.

For instance, some patients previously thought to have viral encephalitis will be found to have a treatable autoimmune disease. Therefore, there is a lack of understanding on the frequency and power of this association. We report a case study, followed up for a one-year period, of a patient with limbic encephalitis with active Systemic Lupus Erythematosus Disease SLEwho showed a good response to immunosuppressors and whose diagnostic tests excluded underlying active cancer.

These symptoms were followed by anterograde amnesia and temporal disorientation initiated 3 days before the hospitalization. Clinical and neurologic examination was normal except for temporal disorientation, low scores on the Mini-Mental State Examination and episodic memory impairment Table 1. Cognitive performance on baseline and follow-up. The patient reported previous diagnosis of SLE as well as endometrial adenocarcinoma in and papillary urothelial carcinoma inwith a curative treatment in the past, and no complaints related to these diseases.

Moreover, CSF had mild lymphocytic pleocytosis 5 cells Her electroencephalography revealed necefalite TIRDA pattern in the left temporal region with an electrographic seizure in the right temporal region on the same exam Figure 2.


In the follow up, it became normal without epileptic activity not shown. A diagnosis of limbic encephalitis and active SLE was then reached. Additionally, during the hospital stay, a search for tumors was performed. Computed Tomographic imaging of the thorax, abdomen and pelvis, breast and transvaginal ultrasound were normal.

Llmbica of the thorax, abdomen and pelvis was also limbifa and were normal. Video-electroencephalography was performed on the third day of immunosuppressive treatment, while using oxcarbazepine, and showed normal background activity without epileptic discharges.

Her semantic and phonemic fluency were substantially increased. As clinical response was effective, it was decided to maintain the patient under this treatment regimen for at least 6 months to consolidate immunosuppression, with monthly infusions administered in the hospital setting.

A follow-up EEG remained normal, therefore the antiepileptic drug was discontinued. A fully and persistent recovery of her cognitive abilities was observed Table 1 up until her last evaluation. Currently, she has a subjective complaint of slow thinking and normal instrumental activities of daily living.

After concluding 6 months of immunosuppression treatment, she returned to work. The classical manifestation of LE includes episodic memory impairment 9seizures 10confusion 16sleeping problems 18and psychiatric symptoms.

Other authors, Graus and Saiz, revised the criteria, changing some accepted clinical characteristics for diagnosis under this criteria, the patient has to have all four items: All patients with LE should undergo a neuroimaging evaluation of the medial temporal area.

It is crucial to rule out any underlying malignancy as LE is commonly related to neoplasm, as a paraneoplastic manifestation.

Limbic encephalitis – Wikidata

Thus, Fluorodeoxyglucose-PET is useful for detecting many occult malignancies but has limited utility for ovarian teratomas. In this context, SLE for instance can mimic or be associated with limbic encephalitis.

Although autoimmune limbic encephalitis ALE and paraneoplastic limbic encephalitis PLE share an immune-mediated background, they can be separated as distinct medical conditions since they have different pathogenic mechanisms.

Moreover, limbic encephalitis with active SLE, although with a poorly understood pathophysiology, could also share some similarities and differences in this picture. It is believed that a convergence point between the three conditions involves an autoimmunity component shared by all of them. In general, several studies show that five features characterize autoimmune physiopathology. Secondly, the antibodies alter the structure or function of the corresponding neuronal antigen.

Thirdly, the effects of the antibodies limbiica often reversible. Lastly, the clinical picture resembles that of pharmacologic or genetic models in which the antigen is disrupted.

Specifically in the context of ALE and PLE, some researchers propose that a logical way to differentiate these two conditions limbicca to identify whether the target antigen is intracellular, encefalige or on li,bica cell surface and whether the immune response is primarily mediated by cellular or humoral mechanisms.

Moreover, they are paraneoplastic and largely resistant to immunotherapy, even after tumor removal. In contrast, autoimmune encefalote encephalitis is associated with antibodies to synaptic or cell surface antigens.

These are likely to contribute directly to the pathology of the condition. This condition appears to be antibody mediated, and is often highly responsive to treatment. Therefore, with regard to ALE and PLE, there are many types of antibodies against extra or intracellular structures, such as: The most common neuropsychiatric syndromes attributed to SLE are seizure disorders, cerebrovascular disease, necefalite confusional states and neuropathies.

However, general SLE-related disease activity, previous or concurrent neuropsychiatric symptoms, and persistent positivity for antiphospholipid li,bica at moderate-to-high titers have been shown to be the most informa tive indicators of neuropsychiatric events attributed to SLE.


Consequently, irrespective of age, previous medical history and main presentation, ideally all such patients should be tested for these types of antibodies. Unfortunately, these antibodies in our patient could not be tested due to technical laboratory limitations.

In the management of ALE, it is important to highlight the benefits of early treatment General concepts about treatment of classical paraneoplastic CNS syndromes do not apply in these cases.

For example, encefalitte classical paraneoplastic syndromes do not respond to immunotherapy unless the tumor is successfully treated 12encefalihe it then has a limited lkmbica 111621ALE may respond to immunotherapy regardless of tumor removal. In conclusion, the primary objective of this article was to explore the possibility of an association between limbic encephalitis and autoimmune disease, particularly SLE. There is growing interest in the literature to study a possible association between autoimmune diseases, such as SLE, and limbic encephalitis, not thought possible only a few years ago.

Knowledge about limbixa clinical association and the pathophysiological mechanisms involved needs to be furthered. This article intends to contribute by reporting a possible case of LE with active SLE, adding more data to this discussion. The main limitation of this article is the absence of neuronal antibody tests. However, the good evolution of the patient and excellent, rapid response to immunotherapy make it reasonable to assume an underlying autoimmune LE, as this has been discussed previously in the literature.

The presence of other surface membrane antibodies is possible, since there are associations among different types of these as mentioned above.

Orphanet: Encefalite limbica

This case highlighted a need for rapid treatment when an autoimmune cause is suspected. To sum up, further studies are necessary to determine the true association between limbic encephalitis and autoimmune diseases, especially Systemic Lupus Erythematosus.

Efforts should be made to establish whether this association is pathologic. Future studies should explore which antibodies are related to neuropsychiatric lupus and the pathologic mechanism triggered by them.

To this ejcefalite, determining the role of autoantibodies will be essential in order to confirm the true relationship. Serial MRI of limbic encephalitis. Magnetic resonance imaging of limbic encephalitis. Can Assoc Radiol J ; Subacute encephalitis of late adult life mainly affecting the limbic areas. Limbic encephalitis and small cell lung cancer Clinical and imunological features.

Small cell lung cancer presenting with paraneoplastic limbic encephalitis. Asia Pac J Clin Oncol ;7: Rosenfeld Encefapite, Dalmau J. Paraneoplastic limbic encephalitis associated with small-cell lung cancer. White D, Beringer T. Paraneoplastic limbic encephalitis in an elderly patient with small cell lung carcinoma. Ulster Med J ; Immunotherapy-responsive dementias and encephalopathies.

What should you know about limbic encephalitis? Autoantibody testing in encephalopathies.

Rosenfeld Lumbica, Dalmau J. Continuum Minneap Minn ; Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Glutamate receptor antibodies in neurological diseases: J Neural Transm ; Jeltsch-David H, Muller S. Neuropsychiatric systemic lupus erythematosus: Nat Rev Neurol ; Limbic encephalitis associated with systemic lupus erythematosus. Treating lupus with medication.

Limbic encephalitis – a review.