A case of post-leptospirosis autoimmune epilepsy presenting with sleep-related hypermotor seizures

2017 ◽  
Vol 19 (4) ◽  
pp. 456-460 ◽  
Author(s):  
Prashant Makhija ◽  
Siby Gopinath ◽  
Sudheeran Kannoth ◽  
Kurupath Radhakrishnan
Keyword(s):  
Autoimmune Epilepsy ◽  
Hypermotor Seizures

THE INSULA: ISLAND OF REIL

2015 ◽  
Vol 86 (11) ◽  
pp. e4.155-e4
Author(s):  
Ray Wynford-Thomas ◽  
Rob Powell
Keyword(s):  
Speech Processing ◽  
Brain Function ◽  
Insular Cortex ◽  
Normal Brain ◽  
Temporal Epilepsy ◽  
Normal Brain Function ◽  
Temporal Structures ◽  
Hypermotor Seizures ◽  
Posterior Insula ◽  
As Effects

Just as ‘no man is an island’, despite its misleading name, the insula is not an island. Sitting deeply within the cerebrum, the insular cortex and its connections play an important role in both normal brain function and seizure generation. Stimulating specific areas of the insula can produce somatosensory, viscerosensory, somatomotor and visceroautonomic symptoms, as well as effects on speech processing and pain. Insular onset seizures are rare, but may mimic both temporal and extra-temporal epilepsy and if not recognised, may lead to failure of epilepsy surgery. We therefore highlight the semiology of insular epilepsy by discussing three cases with different auras. Insular onset seizures can broadly be divided into three main types both anatomically and according to seizure semiology:1. Seizures originating in the antero-inferior insula present with laryngeal constriction, along with visceral and gustatory auras (similar to those originating in medial temporal structures).2. Antero-superior onset seizures can have a silent onset, but tend to propagate rapidly to motor areas causing focal motor or hypermotor seizures.3. Seizures originating in the posterior insula present with contralateral sensory symptoms.

Autoimmune Epilepsy

2008 ◽  
pp. 441-444
Author(s):  
Sarosh R. Irani ◽  
Bethan Lang
Keyword(s):  
Autoimmune Epilepsy

scholarly journals Update on Intravenous Immunoglobulin in Neurology: Modulating Neuro-autoimmunity, Evolving Factors on Efficacy and Dosing and Challenges on Stopping Chronic IVIg Therapy

2021 ◽  
Author(s):  
Marinos C. Dalakas
Keyword(s):  
Intravenous Immunoglobulin ◽  
Autoimmune Encephalitis ◽  
Ivig Therapy ◽  
Antigenic Peptides ◽  
Factors Affecting ◽  
Autoimmune Epilepsy ◽  
Healthy Donors ◽  
Chronic Therapy ◽  
Individualized Dosing ◽  
Genes Encoding

AbstractIn the last 25 years, intravenous immunoglobulin (IVIg) has had a major impact in the successful treatment of previously untreatable or poorly controlled autoimmune neurological disorders. Derived from thousands of healthy donors, IVIg contains IgG1 isotypes of idiotypic antibodies that have the potential to bind pathogenic autoantibodies or cross-react with various antigenic peptides, including proteins conserved among the “common cold”-pre-pandemic coronaviruses; as a result, after IVIg infusions, some of the patients’ sera may transiently become positive for various neuronal antibodies, even for anti-SARS-CoV-2, necessitating caution in separating antibodies derived from the infused IVIg or acquired humoral immunity. IVIg exerts multiple effects on the immunoregulatory network by variably affecting autoantibodies, complement activation, FcRn saturation, FcγRIIb receptors, cytokines, and inflammatory mediators. Based on randomized controlled trials, IVIg is approved for the treatment of GBS, CIDP, MMN and dermatomyositis; has been effective in, myasthenia gravis exacerbations, and stiff-person syndrome; and exhibits convincing efficacy in autoimmune epilepsy, neuromyelitis, and autoimmune encephalitis. Recent evidence suggests that polymorphisms in the genes encoding FcRn and FcγRIIB may influence the catabolism of infused IgG or its anti-inflammatory effects, impacting on individualized dosing or efficacy. For chronic maintenance therapy, IVIg and subcutaneous IgG are effective in controlled studies only in CIDP and MMN preventing relapses and axonal loss up to 48 weeks; in practice, however, IVIg is continuously used for years in all the aforementioned neurological conditions, like is a “forever necessary therapy” for maintaining stability, generating challenges on when and how to stop it. Because about 35-40% of patients on chronic therapy do not exhibit objective neurological signs of worsening after stopping IVIg but express subjective symptoms of fatigue, pains, spasms, or a feeling of generalized weakness, a conditioning effect combined with fear that discontinuing chronic therapy may destabilize a multi-year stability status is likely. The dilemmas of continuing chronic therapy, the importance of adjusting dosing and scheduling or periodically stopping IVIg to objectively assess necessity, and concerns in accurately interpreting IVIg-dependency are discussed. Finally, the merit of subcutaneous IgG, the ineffectiveness of IVIg in IgG4-neurological autoimmunities, and genetic factors affecting IVIg dosing and efficacy are addressed.

Autoimmune epilepsy: the search for a definition

2014 ◽  
Vol 57 (5) ◽  
pp. 402-403 ◽  
Author(s):  
Sukhvir Wright ◽  
Ming Lim
Keyword(s):  
Autoimmune Epilepsy

scholarly journals Autoimmune epilepsy: Findings on MRI and FDG-PET

2018 ◽  
pp. 20170869 ◽  
Author(s):  
Julie Guerin ◽  
Robert E. Watson ◽  
Carrie M. Carr ◽  
Greta B. Liebo ◽  
Amy L. Kotsenas
Keyword(s):  
Fdg Pet ◽  
Autoimmune Epilepsy

Autoimmune Epilepsy: The Evolving Science of Neural Autoimmunity and Its Impact on Epilepsy Management

2018 ◽  
Vol 38 (03) ◽  
pp. 290-302 ◽  
Author(s):  
Orna O'Toole ◽  
Amy Quek
Keyword(s):  
Patient Outcomes ◽  
Treatment Options ◽  
Immunosuppressive Treatment ◽  
Substantial Reduction ◽  
Risk Scores ◽  
Seizure Freedom ◽  
Clinical Approach ◽  
Treatment Trial ◽  
Autoimmune Epilepsy ◽  
Immune Mediated

AbstractAutoimmune epilepsy is increasingly recognized as a distinct clinical entity, driven in large part by the recent discovery of neural autoantibodies in patients with isolated or predominant epilepsy presentations. Detection of neural autoantibodies in high-risk epilepsy patients supports an immune-mediated cause of seizures and, if applicable, directs the search for an underlying cancer when the paraneoplastic association of the associated antibody is compelling. Early diagnosis of autoimmune epilepsy is crucial, as prompt initiation of immunosuppressive treatment increases the likelihood of achieving either seizure freedom or a substantial reduction in seizure frequency. A practical clinical approach that incorporates risk scores to guide patient selection on the basis of clinical features, neural autoantibodies, and a treatment trial of immunotherapy is suggested. Elucidating an immunological basis of epilepsy provides neurologists with wider treatment options (incorporating immune-suppressive treatment), in addition to standard antiepileptic drugs, which often improves patient outcomes.

scholarly journals Autoimmune Epilepsy: New Development and Future Directions

Seizures ◽  
2018 ◽  
Author(s):  
Sandra Orozco-Suarez ◽  
Angélica Vega-Garcia ◽  
Iris Feria-Romero ◽  
Lourdes Arriaga-Pizano ◽  
Emmanuel Rodriguez-Chavez ◽  
...  
Keyword(s):  
Future Directions ◽  
Autoimmune Epilepsy ◽  
New Development

Application of the APE2-CHN and RITE2-CHN scores for autoimmune epilepsy in Chinese patients: a retrospective study

2020 ◽  
Author(s):  
Wei-ping Liu ◽  
Mian Wang ◽  
Chen Zhang ◽  
Charlie Weige Zhao ◽  
Bo Xiao ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Sensitivity And Specificity ◽  
Chinese Patients ◽  
Unknown Etiology ◽  
Thyroid Peroxidase ◽  
Seizure Outcome ◽  
Receptor Antibody ◽  
Autoimmune Epilepsy ◽  
Patients With Epilepsy ◽  
New Onset

Abstract Background : Autoimmune epilepsy is recognized as a distinct entity of epilepsy with underestimated incidence. Our previous study reported that prompt diagnosis and early-initiated immunotherapy led to better outcome. We proposed to assess the feasibility and reasonability of the Antibody Prevalence in Chinese Patients with Epilepsy and Encephalopathy (APE 2 -CHN) and Response to Immunotherapy in Chinese Patients with Epilepsy and Encephalopathy (RITE 2 -CHN) scores in predicting Chinese patients with autoimmune epilepsy. Methods : We conducted a retrospective study of consecutive patients from Xiangya Hospital, Central South University (01/01/2017-02/28/2019) whose serum and/or cerebrospinal fluid (CSF) samples were examined for autoimmune encephalitis antibodies. Of these, patients with new-onset epilepsy or established epilepsy of unknown etiology were selected in our study. An APE 2 -CHN score was assigned to each patient and a RITE 2 -CHN score was calculated for each patient who received immunotherapy. Results : 191 patients meeting the diagnostic criteria for epilepsy were enrolled in our study. 36 were subsequently identified with specific etiologies. The rest of the 155 patients had an unknown etiology. Central nervous system-specific antibodies were detected in 76 (49.0%) of them, after excluding solely thyroid peroxidase antibody or glutamic acid decarboxylase antibody. N-methyl-D-aspartate receptor antibody (48.7%, 37/76) was the most common antibody specificity, followed by γ-aminobutyric acid type B receptor antibody (14.5%, 11/76). Certain clinical features such as new-onset epilepsy, autonomic dysfunction, viral prodrome, facio-brachial dystonic seizures/oral dyskinesia, inflammatory CSF profile, and mesial temporal magnetic resonance imaging abnormalities correlated with positive antibody results. Sensitivity and specificity of an APE 2 -CHN score ≥ 5 to predict the presence of specific neural auto-antibodies in our study were 85.5% and 58.9%, respectively. In the subset of patients who received immunotherapy (n = 112), sensitivity and specificity of a RITE 2 -CHN score ≥ 8 to predict favorable seizure outcome were 98.6% and 63.2% respectively. Conclusion : The APE 2 -CHN and RITE 2 -CHN scores were preferable tools in predicting positive serologic findings and prognosis of autoimmune epilepsy in Chinese patients with epilepsy.

Goose Bumps and Memory Loss

2021 ◽  
pp. 96-97
Author(s):  
Jeffrey W. Britton ◽  
Bhavya Narapureddy ◽  
Divyanshu Dubey
Keyword(s):  
Mycophenolate Mofetil ◽  
Cognitive Dysfunction ◽  
Immunoglobulin G ◽  
Oral Prednisone ◽  
Memory Loss ◽  
Oligoclonal Bands ◽  
Fluid Analysis ◽  
Autoimmune Epilepsy ◽  
Fluid Attenuated Inversion Recovery ◽  
Interictal Discharges

A 46-year-old man had an episode of loss of awareness while driving home. He was found in a cul de sac by his neighbor and was acting confused. He was brought to the emergency department; while there, he started having recurrent episodes of goose bumps (goose flesh) involving half of his body associated with a “wavelike” sensation that would typically begin in the lower extremities and spread upward. He also had some speech difficulty. Physical and neurologic examinations were unremarkable, except for mild cognitive deficits. Video electroencephalographic monitoring showed frequent independent left and right temporal ictal and interictal discharges. Magnetic resonance imaging of the brain showed fluid-attenuated inversion recovery hyperintensity in the bilateral hippocampi. Cerebrospinal fluid analysis showed a mildly increased total protein concentration but no supernumerary oligoclonal bands and normal nucleated cell count and immunoglobulin G index. Serum autoimmune epilepsy evaluation was remarkable for leucine-rich, glioma-inactivated protein 1-immunoglobulin G seropositivity. The patient was diagnosed with leucine-rich, glioma-inactivated protein 1- immunoglobulin G antibody–associated autoimmune seizures presenting with pilomotor seizures. Before autoimmune work-up, the patient had been treated with a gradual escalation of antiseizure medications. The seizures continued. He was subsequently started on intravenous methylprednisolone. He was seizure free and was transitioned to an oral prednisone taper. He was subsequently started on a 6-week regimen of intravenous immunoglobulin. He was also started on mycophenolate mofetil. On follow-up clinic visits, the patient had no recurrence of seizures and disclosed no cognitive dysfunction except for mild inattention. Three years after the patient’s initial episode, the antiseizure medications and mycophenolate mofetil were gradually tapered, without recurrence. Leucine-rich, glioma-inactivated protein 1-immunoglobulin G–associated autoimmunity is typically seen among older patients (>50 years), more commonly men. Symptoms commonly include seizures and cognitive dysfunction, presenting as memory loss and disorientation.

Autoimmune Epilepsy

2021 ◽  
pp. 189-206
Author(s):  
Khalil Husari ◽  
Divyanshu Dubey
Keyword(s):  
Autoimmune Epilepsy
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