scholarly journals Clinical Heterogeneity in Acute Symptomatic Seizures due to Autoimmune Encephalitis Related to GAD65 Antibodies

2021 ◽  
pp. 1-6
Author(s):  
Xuemei Fan ◽  
Xinxin Li ◽  
Shuai Wang ◽  
Yafei Zhu ◽  
Songyan Liu ◽  
...  
Keyword(s):  
Clinical Features ◽  
Brain Mri ◽  
Autoimmune Encephalitis ◽  
Mri Findings ◽  
Ocular Movement ◽  
Symptomatic Seizures ◽  
Gad 65 ◽  
Gad 65 Antibodies ◽  
Acute Symptomatic Seizures ◽  
Gad65 Antibodies

<b><i>Introduction:</i></b> This study aimed to explore the diversity and clinical features of acute symptomatic seizures due to autoimmune encephalitis related to anti-glutamate decarboxylase (GAD) 65 antibodies. <b><i>Methods:</i></b> Clinical data of a series of 6 patients positive for anti-GAD65 antibodies were retrospectively analyzed. <b><i>Results:</i></b> Five of the patients were male and 1 was a female, with a median age of 44.1 years (range 18–70 years). Seizure forms were varied in 6 patients when they were admitted to the hospital: 3 cases of seizures only and 3 accompanied by other symptoms, such as mental disorder, cognitive impairment, cerebellar ataxia, and ocular movement disorder. Three patients (50%) had coexisting systemic autoimmune diseases, including diabetes mellitus, vitiligo, and hyperthyroidism. Five patients (83%) had abnormal brain MRI findings. They were all treated by immunotherapy, 5 of 6 patients improved significantly but relapsed after withdrawing methylprednisolone, and 1 patient got deteriorated. None of them were diagnosed with tumors. <b><i>Conclusions:</i></b> Clinical features of acute symptomatic seizures related to GAD65 antibodies are diverse, and early and continuous immunotherapy is necessary for patients.

Rare presentation of anti-GAD-65 antibody-positive autoimmune encephalitis and simultaneous onset of type 1 diabetes mellitus in a paediatric patient

2021 ◽  
Vol 14 (3) ◽  
pp. e237913
Author(s):  
Kristina Kern ◽  
Brooke A Shuster
Keyword(s):  
Diabetes Mellitus ◽  
Type 1 Diabetes ◽  
Type 1 Diabetes Mellitus ◽  
Brain Mri ◽  
Autoimmune Encephalitis ◽  
Acid Decarboxylase ◽  
Rare Presentation ◽  
Gad 65 ◽  
Gad 65 Antibodies

A 16-year-old female patient presented with subacute onset of headaches, changes in acute mental status, expressive aphasia and auditory hallucinations. New oedema and enhancement of the temporal lobe were seen on brain MRI, with correlating subclinical seizures seen on electroencephalogram. Simultaneously, our patient was diagnosed with new-onset type 1 diabetes mellitus, with positive anti-glutamic acid decarboxylase (anti-GAD-65) antibodies in the serum. Cerebrospinal fluid studies remained negative, including anti-GAD-65 antibodies. Clinical remission was achieved with corticosteroids and intravenous immunoglobulins.

Large hemispheric lesions in autoimmune encephalitis associated with anti-GAD 65 antibodies

2020 ◽  
Vol 44 ◽  
pp. 102331
Author(s):  
Bahareh Sianati ◽  
Sandeep S. Rana ◽  
Murat Sari ◽  
Jody Leonardo ◽  
Thomas F. Scott
Keyword(s):  
Autoimmune Encephalitis ◽  
Gad 65 ◽  
Gad 65 Antibodies

scholarly journals Different MRI patterns in MS worsening after stopping fingolimod

2019 ◽  
Vol 6 (4) ◽  
pp. e566 ◽  
Author(s):  
Caterina Lapucci ◽  
Damiano Baroncini ◽  
Maria Cellerino ◽  
Giacomo Boffa ◽  
Ilaria Callegari ◽  
...  
Keyword(s):  
Clinical Features ◽  
Brain Mri ◽  
Activity Level ◽  
Lesion Volume ◽  
Mri Findings ◽  
Volume Increase ◽  
Mri Features ◽  
Disability Status ◽  
The Mean

ObjectiveTo analyze MRI images in patients with MS who experienced worsening of neurologic status (WNS) after stopping fingolimod (FTY).MethodsIn this retrospective study, demographic, clinical, and radiologic data of patients with MS who experienced WNS after stopping FTY were retrospectively collected. We introduced the “δExpanded Disability Status Scale (EDSS)-ratio” to identify patients who, after FTY withdrawal, showed an inflammatory flare-up exceeding the highest lifetime disease activity level. Patients with δEDSS-ratio > 1 were enrolled in the study.ResultsEight patients were identified. The mean (SD) age of the 8 (7 female) patients was 35.3 (4.9) years. The mean FTY treatment duration was 3.1 (0.8) years. The mean FTY discontinuation–WNS interval was 4 (0.9) months. The 4 patients with δEDSS-ratio ≥ 2 developed severe monophasic WNS (EDSS score above 8.5), characterized by clinical features and MRI findings not typical of MS, which we classified as “tumefactive demyelination pattern” (TDL) and “Punctuated pattern” (PL). Conversely, patients whose δEDSS-ratio was between 1 and 2 had clinical features and brain MRI compatible with a more typical, even if aggressive, MS relapse. In patients with TDL and PL, the flare-up of inflammatory activity led to severe tissue damage resulting in T2 but also T1 lesion volume increase at 6-month follow-up.ConclusionsPeculiar MRI features (TDL and PL), different from a typical MS flare-up, might occur in some patients who experienced WNS after stopping FTY. Further studies, also involving immunologic biomarkers, are necessary to investigate TDL or PL pathophysiology.

scholarly journals P.050 Autoimmune encephalitis associated with GAD65 antibodies: brief review of the relevant literature

2016 ◽  
Vol 43 (S2) ◽  
pp. S33-S33
Author(s):  
M Gagnon ◽  
M Savard
Keyword(s):  
Memory Impairment ◽  
Brain Mri ◽  
Relevant Literature ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Systemic Autoimmune Disease ◽  
Acid Decarboxylase ◽  
Distinctive Pattern ◽  
Temporal Lobes ◽  
Gad65 Antibodies

Background: Recently, many cases of autoimmune encephalitis with positive GAD65 (Glutamic acid decarboxylase) antibodies have been described in the literature. However, it remains an understudied topic. Methods: We conducted a search on reported cases of anti-GAD65 encephalitis. Specific variables were identified as general characteristics, clinical manifestations, MRI and EEG findings, concomitant systemic autoimmune disorders and cancer, and outcome and autoantibodies findings. Results: We identified a total of 58 cases, from one to 70 years old. It most frequently presented with seizures (97%) and memory impairment (59%). It commonly occurred in association with systemic autoimmune disease, particularly diabetes (28%). Brain MRI was usually abnormal (78%); involvement of temporal lobes was more frequent than multifocal abnormalities (59% vs 16%). GAD65 antibodies were reported positive in CSF and/or serum (31% in serum only). Other antibodies such as GABABR, GABAAR and VGKC were concurrently reported positive in some cases (19%). However, we found that the vast majority of cases were not tested for all those cell-surface antibodies. Overall, no distinctive pattern of clinical and paraclinical findings was found. Persistent impairments were common. Optimal treatment remained undefined. Conclusions: Prospective studies recruiting patients with autoimmune encephalitis are needed to better elucidate the contributions of GAD65 autoantibodies, and to evaluate treatment and outcomes in this population.

scholarly journals Clinical Features and Brain MRI Findings in Korean Patients with AGel Amyloidosis

2021 ◽  
Vol 62 (5) ◽  
pp. 431
Author(s):  
E-Nae Cheong ◽  
Wooyul Paik ◽  
Young-Chul Choi ◽  
Young-Min Lim ◽  
Hyunjin Kim ◽  
...  
Keyword(s):  
Clinical Features ◽  
Brain Mri ◽  
Mri Findings ◽  
Korean Patients

scholarly journals Cryptogenic NORSE

2017 ◽  
Vol 4 (6) ◽  
pp. e396 ◽  
Author(s):  
Takahiro Iizuka ◽  
Naomi Kanazawa ◽  
Juntaro Kaneko ◽  
Naomi Tominaga ◽  
Yutaka Nonoda ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Clinical Features ◽  
Brain Mri ◽  
Ventilatory Support ◽  
Autoimmune Encephalitis ◽  
Refractory Status Epilepticus ◽  
Oligoclonal Bands ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Nmda Receptor Encephalitis

Objective:To report the distinctive clinical features of cryptogenic new-onset refractory status epilepticus (C-NORSE) and the C-NORSE score based on initial clinical assessments.Methods:A retrospective study was conducted for 136 patients with clinically suspected autoimmune encephalitis who underwent testing for autoantibodies to neuronal surface antigens between January 1, 2007, and August 31, 2016. Eleven patients with C-NORSE were identified. Their clinical features were compared with those of 32 patients with anti-NMDA receptor encephalitis (NMDARE).Results:The clinical outcome of 11 patients (median age, 27 years; 7 [64%] women) with C-NORSE was evaluated after a median follow-up of 11 months (range, 6–111 months). Status epilepticus was frequently preceded by fever (10/11 [91%]). Brain MRIs showed symmetric T2/fluid-attenuated inversion recovery hyperintensities (8/11 [73%]) and brain atrophy (9/11 [82%]). Only 2 of the 10 treated patients responded to the first-line immunotherapy, and 4 of the 5 patients treated with IV cyclophosphamide responded to the therapy. The long-term outcome was poor in 8 patients (73%). Compared with 32 patients with NMDARE (median age, 27 years; 24 [75%] women), those with C-NORSE had more frequent prodromal fever, status epilepticus, ventilatory support, and symmetric brain MRI abnormalities, had less frequent involuntary movements, absent psychobehavioral symptoms, CSF oligoclonal bands, or tumor association, and had a worse outcome. The C-NORSE score was higher in patients with C-NORSE than those with NMDARE.Conclusions:Patients with C-NORSE have a spectrum of clinical-immunological features different from those with NMDARE. The C-NORSE score may be useful for discrimination between them. Some patients could respond to immunotherapy.

scholarly journals Clinical features and surgical treatment of epilepsy after viral encephalitis

2019 ◽  
Vol 5 (1) ◽  
pp. 41-50 ◽  
Author(s):  
Yiou Liu ◽  
Wenjing Zhou
Keyword(s):  
Surgical Treatment ◽  
Viral Infection ◽  
Clinical Features ◽  
Viral Encephalitis ◽  
Brain Parenchyma ◽  
Neurological Sequelae ◽  
Symptomatic Seizures ◽  
The Central Nervous System ◽  
Underlying Mechanisms ◽  
Acute Symptomatic Seizures

Encephalitis is an acute inflammatory process of the brain parenchyma, which is often caused by viral infection. It is an vital cause of acute symptomatic seizures and subsequent epilepsy. The incidence of unprovoked and recurrent seizures after previous infections of the central nervous system is high and accounts for 1%~5% of the cases of epilepsy. Viral encephalitis (VE) is directly caused by viral infection. The occurrence of seizures after VE is associated with poor prognosis. In survivors of VE, among other neurological sequelae, the risk of developing epilepsy is increased 10-fold. The risk of severe neurological sequelae after VE is particularly high in very young children. Studies on seizure occurrence, possible underlying mechanisms, clinical characteristics, and clinical treatment (especially surgical treatment) of VE have yielded only limited detailed data. We reviewed the most recent literature on the clinical features and surgical treatment of post-VE epilepsy.

scholarly journals Acute symptomatic seizures secondary to autoimmune encephalitis and autoimmune‐associated epilepsy: Conceptual definitions

Epilepsia ◽  
2020 ◽  
Vol 61 (7) ◽  
pp. 1341-1351 ◽  
Author(s):  
Claude Steriade ◽  
Jeffrey Britton ◽  
Russell C. Dale ◽  
Avi Gadoth ◽  
Sarosh R. Irani ◽  
...  
Keyword(s):  
Autoimmune Encephalitis ◽  
Symptomatic Seizures ◽  
Acute Symptomatic Seizures

Headache among CADASIL patients with R544C mutation: Prevalence, characteristics, and associations

Cephalalgia ◽  
2013 ◽  
Vol 34 (1) ◽  
pp. 22-28 ◽  
Author(s):  
Jay Chol Choi ◽  
Sook-Keun Song ◽  
Jung Seok Lee ◽  
Sa-Yoon Kang ◽  
Ji-Hoon Kang
Keyword(s):  
Clinical Features ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Cross Sectional Study ◽  
Tension Type Headache ◽  
Mri Findings ◽  
Cross Sectional ◽  
Asian Patients ◽  
Type Headache

Background and aim There are variations of migraine prevalence in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) among different regions of the world. Previous studies on Asian CADASIL patients have not provided detailed descriptions of the characteristics of their headaches. The aims of this study were to determine prevalence and characteristics of headaches and to investigate associations between headache and other clinical symptoms or brain magnetic resonance imaging (MRI) findings among homogenous group of patients having the same R544C mutation. Methods In this cross-sectional study, we enrolled 53 CADASIL patients with the R544C mutation between May 2010 and April 2011. We obtained the history of headache using a structured questionnaire and detailed interview. Other clinical features and brain MRI findings were also assessed for potential associations. Results Overall headache prevalence was 45.3% (24 patients). Among them, 21 patients (87.5%) were classified as having tension-type headache, followed by migraine (two patients) and unclassifiable headaches (one patient). Except for alcohol consumption, no significant associations were observed between the headaches and other clinical features or brain MRI findings. Conclusion Migraine was found in only 3.8% of CADASIL patients with the R544C mutation. Such a low prevalence of migraine may hinder clinical detection of CADASIL among ethnically Asian patients.

scholarly journals P.009 Improving Detection of Creutzfeldt-Jakob Disease Mimics in Clinical Practice

2021 ◽  
Vol 48 (s3) ◽  
pp. S22-S22
Author(s):  
ES Lazar ◽  
AL Porter ◽  
CC Prusinski ◽  
S Dunham ◽  
A Lopez-Chiriboga ◽  
...  
Keyword(s):  
Clinical Features ◽  
Autoimmune Encephalitis ◽  
Motor Dysfunction ◽  
Mri Findings ◽  
Progressive Dementia ◽  
Clinical Criteria ◽  
Presenting Symptoms ◽  
Jakob Disease ◽  
Study Sites ◽  
Motor Abnormalities

Background: Assays capable of detecting prions in CSF (e.g., RT-QuIC) have greatly improved the antemortem diagnosis of Creutzfeldt-Jakob disease (CJD) yet take time to conduct and are not widely accessible. There is a need to identify clinical features and common tests that identify mimics at presentation. Methods: Mimics were identified within longitudinal studies of rapidly progressive dementia at study sites. Mimics met clinical criteria for probable CJD but did not have CJD. Clinical features were compared between mimics and patients with CJD assessed at Mayo Clinic Enterprise (n=79) and Washington University in St. Louis (n=10; Jan-2014 to Oct-2020). Results: Mimics (10/155; 6.5%) were diagnosed with autoimmune encephalitis (n=7), neurosarcoidosis, frontotemporal lobar degeneration with motor neuron disease, and unknown dementia. Age-at-symptom onset, gender, presenting symptoms, and EEG and MRI findings were similar between mimics and CJD patients. Focal motor abnormalities (49/93, 10/10), elevations in CSF leukocytosis (4/92, 5/10) and protein (39/92, 9/10) were more common in mimics (p<0.01). Neural-specific autoantibodies associated with autoimmune encephalitis were detected within the serum (4/9) and CSF (5/10) of mimics, but not CJD cases. Conclusions: Autoimmune encephalitis, neurosarcoidosis and neurodegenerative diseases may mimic CJD at presentation and should be considered in patients with early motor dysfunction and abnormal CSF studies.

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