Cargando…
Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline
OBJECTIVES: To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline. DESIGN: Observational, prospective case series. SETTING: All India Institute of Medical Sciences, New Delhi, In...
Autores principales: | , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Medknow Publications & Media Pvt Ltd
2013
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3841604/ https://www.ncbi.nlm.nih.gov/pubmed/24339583 http://dx.doi.org/10.4103/0972-2327.120476 |
_version_ | 1782292809742548992 |
---|---|
author | Pandit, Awadh Kishor Ihtisham, Kavish Garg, Ajay Gulati, Sheffali Padma, Madakasira Vasantha Tripathi, Manjari |
author_facet | Pandit, Awadh Kishor Ihtisham, Kavish Garg, Ajay Gulati, Sheffali Padma, Madakasira Vasantha Tripathi, Manjari |
author_sort | Pandit, Awadh Kishor |
collection | PubMed |
description | OBJECTIVES: To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline. DESIGN: Observational, prospective case series. SETTING: All India Institute of Medical Sciences, New Delhi, India. MATERIALS AND METHODS: Prospective analysis of 15 patients, who presented with SE, epilepsy, cognitive decline, and other neurological symptoms with positive autoantibodies. Demographic and clinical characteristics were recorded. Brain magnetic resonance imaging (MRI), cerebrospinal-fluid analysis (CSF), and tumor screening were done periodically. Treatment received and responses (categorized as per patients and treating doctor's information) were noted. RESULTS: There were 15 (males = 10) patients of autoimmune encephalitis. The mean age of presentation was 24 years (range: 2-64 years). The most common onset was subacute (64%) and four (29%) patients presented as SE. Predominant clinical presentations were seizures (100%) almost of every semiology. CSF was done in 10 patients; it was normal in 60%. Brain MRI was done in all patients, in six (40%) it was normal, six (40%) showed T2W and FLAIR hyperintensities in bilateral limbic areas. Antibodies found were the N-methyl-D-aspartate receptor antibody in seven (50%), voltage-gated potassium channel antibody in five (36%), two of antiglutamic acid decarboxylase, and one patient with double stranded DNA (dsDNA) antibodies. None showed evidence of malignancy. Patients received immunotherapy, either steroids, intravenous immunoglobulin, or both. Follow-up showed significant improvement in majority of cases, neither further seizures nor relapse in nine (67%) cases. One death occurred, due to delayed presentation. CONCLUSIONS: Uncommon but potentially reversible causes of SE, epilepsy, and cognitive decline may be immune-related and high index of suspicion will prevent missing the diagnosis. |
format | Online Article Text |
id | pubmed-3841604 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-38416042013-12-11 Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline Pandit, Awadh Kishor Ihtisham, Kavish Garg, Ajay Gulati, Sheffali Padma, Madakasira Vasantha Tripathi, Manjari Ann Indian Acad Neurol Original Article OBJECTIVES: To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline. DESIGN: Observational, prospective case series. SETTING: All India Institute of Medical Sciences, New Delhi, India. MATERIALS AND METHODS: Prospective analysis of 15 patients, who presented with SE, epilepsy, cognitive decline, and other neurological symptoms with positive autoantibodies. Demographic and clinical characteristics were recorded. Brain magnetic resonance imaging (MRI), cerebrospinal-fluid analysis (CSF), and tumor screening were done periodically. Treatment received and responses (categorized as per patients and treating doctor's information) were noted. RESULTS: There were 15 (males = 10) patients of autoimmune encephalitis. The mean age of presentation was 24 years (range: 2-64 years). The most common onset was subacute (64%) and four (29%) patients presented as SE. Predominant clinical presentations were seizures (100%) almost of every semiology. CSF was done in 10 patients; it was normal in 60%. Brain MRI was done in all patients, in six (40%) it was normal, six (40%) showed T2W and FLAIR hyperintensities in bilateral limbic areas. Antibodies found were the N-methyl-D-aspartate receptor antibody in seven (50%), voltage-gated potassium channel antibody in five (36%), two of antiglutamic acid decarboxylase, and one patient with double stranded DNA (dsDNA) antibodies. None showed evidence of malignancy. Patients received immunotherapy, either steroids, intravenous immunoglobulin, or both. Follow-up showed significant improvement in majority of cases, neither further seizures nor relapse in nine (67%) cases. One death occurred, due to delayed presentation. CONCLUSIONS: Uncommon but potentially reversible causes of SE, epilepsy, and cognitive decline may be immune-related and high index of suspicion will prevent missing the diagnosis. Medknow Publications & Media Pvt Ltd 2013 /pmc/articles/PMC3841604/ /pubmed/24339583 http://dx.doi.org/10.4103/0972-2327.120476 Text en Copyright: © Annals of Indian Academy of Neurology http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Pandit, Awadh Kishor Ihtisham, Kavish Garg, Ajay Gulati, Sheffali Padma, Madakasira Vasantha Tripathi, Manjari Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline |
title | Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline |
title_full | Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline |
title_fullStr | Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline |
title_full_unstemmed | Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline |
title_short | Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline |
title_sort | autoimmune encephalitis: a potentially reversible cause of status epilepticus, epilepsy, and cognitive decline |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3841604/ https://www.ncbi.nlm.nih.gov/pubmed/24339583 http://dx.doi.org/10.4103/0972-2327.120476 |
work_keys_str_mv | AT panditawadhkishor autoimmuneencephalitisapotentiallyreversiblecauseofstatusepilepticusepilepsyandcognitivedecline AT ihtishamkavish autoimmuneencephalitisapotentiallyreversiblecauseofstatusepilepticusepilepsyandcognitivedecline AT gargajay autoimmuneencephalitisapotentiallyreversiblecauseofstatusepilepticusepilepsyandcognitivedecline AT gulatisheffali autoimmuneencephalitisapotentiallyreversiblecauseofstatusepilepticusepilepsyandcognitivedecline AT padmamadakasiravasantha autoimmuneencephalitisapotentiallyreversiblecauseofstatusepilepticusepilepsyandcognitivedecline AT tripathimanjari autoimmuneencephalitisapotentiallyreversiblecauseofstatusepilepticusepilepsyandcognitivedecline |