scholarly journals Autoimmune Encephalitis Resembling Dementia Syndromes

2021 ◽  
Vol 8 (5) ◽  
pp. e1039
Author(s):  
Anna E.M. Bastiaansen ◽  
Robin W. van Steenhoven ◽  
Marienke A.A.M. de Bruijn ◽  
Yvette S. Crijnen ◽  
Agnes van Sonderen ◽  
...  
Keyword(s):  
Brain Mri ◽  
Autoimmune Encephalitis ◽  
Disease Course ◽  
Gamma Aminobutyric Acid ◽  
Red Flags ◽  
Dementia Syndrome ◽  
Neurodegenerative Dementia ◽  
Observational Cohort ◽  
Csf Pleocytosis ◽  
Inflammatory Changes

ObjectiveAs autoimmune encephalitis (AIE) can resemble neurodegenerative dementia syndromes, and patients do not always present as encephalitis, this study evaluates how frequently AIE mimics dementia and provides red flags for AIE in middle-aged and older patients.MethodsIn this nationwide observational cohort study, patients with anti–leucine-rich glioma-inactivated 1 (LGI1), anti–NMDA receptor (NMDAR), anti–gamma-aminobutyric acid B receptor (GABABR), or anti–contactin-associated protein-like 2 (CASPR2) encephalitis were included. They had to meet 3 additional criteria: age ≥45 years, fulfillment of dementia criteria, and no prominent seizures early in the disease course (≤4 weeks).ResultsTwo-hundred ninety patients had AIE, of whom 175 were 45 years or older. Sixty-seven patients (38%) fulfilled criteria for dementia without prominent seizures early in the disease course. Of them, 42 had anti-LGI1 (48%), 13 anti-NMDAR (52%), 8 anti-GABABR (22%), and 4 anti-CASPR2 (15%) encephalitis. Rapidly progressive cognitive deterioration was seen in 48 patients (76%), whereas a neurodegenerative dementia syndrome was suspected in half (n = 33). In 17 patients (27%; 16/17 anti-LGI1), subtle seizures had been overlooked. Sixteen patients (25%) had neither inflammatory changes on brain MRI nor CSF pleocytosis. At least 1 CSF biomarker, often requested when dementia was suspected, was abnormal in 27 of 44 tested patients (61%), whereas 8 had positive 14-3-3 results (19%). Most patients (84%) improved after immunotherapy.ConclusionsRed flags for AIE in patients with suspected dementia are: (1) rapidly progressive cognitive decline, (2) subtle seizures, and (3) abnormalities in ancillary testing atypical for neurodegeneration. Physicians should be aware that inflammatory changes are not always present in AIE, and that biomarkers often requested when dementia was suspected (including 14-3-3) can show abnormal results. Diagnosis is essential as most patients profit from immunotherapy.

scholarly journals Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABR encephalitis

Neurology ◽  
2019 ◽  
Vol 92 (19) ◽  
pp. e2185-e2196 ◽  
Author(s):  
Marienke A.A.M. de Bruijn ◽  
Agnes van Sonderen ◽  
Marleen H. van Coevorden-Hameete ◽  
Anna E.M. Bastiaansen ◽  
Marco W.J. Schreurs ◽  
...  
Keyword(s):  
Side Effects ◽  
Medical Information ◽  
Autoimmune Encephalitis ◽  
Epileptic Seizures ◽  
Aminobutyric Acid ◽  
Seizure Freedom ◽  
Disease Course ◽  
Gamma Aminobutyric Acid ◽  
Psychotic Behavior ◽  
New Onset

ObjectiveThis nationwide cohort study evaluates seizure responses to immunotherapy and antiepileptic drugs (AEDs) in patients with anti-leucine-rich glioma-inactivated 1 (LGI1), anti-NMDA receptor (NMDAR), and anti-gamma-aminobutyric-acid B receptor (GABABR) encephalitis.MethodsAnti-LGI1, anti-NMDAR, and anti-GABABR encephalitis patients with new-onset seizures were included. Medical information about disease course, AEDs and immunotherapies used, effects, and side effects were collected. Outcome measures were (1) seizure freedom while using AEDs or immunotherapy, (2) days to seizure freedom from start of AEDs or immunotherapy, and (3) side effects.ResultsOf 153 patients with autoimmune encephalitis (AIE) (53 LGI1, 75 NMDAR, 25 GABABR), 72% (n = 110) had epileptic seizures, and 89% reached seizure freedom. At least 53% achieved seizure freedom shortly after immunotherapy, and 14% achieved seizure freedom while using only AEDs (p < 0.0001). This effect was similar in all types (p = 0.0001; p = 0.0005; p = 0.013, respectively). Median time to seizure freedom from AEDs start was 59 days (interquartile range [IQR] 27–160), and 28 days from start of immunotherapy (IQR 9–71, p < 0.0001). Side effects were psychotic behavior and suicidal thoughts by the use of levetiracetam, and rash by the use of carbamazepine. Carbamazepine was more effective than levetiracetam in reducing seizures in anti-LGI1 encephalitis (p = 0.031). Only 1 patient, of 86 surviving patients, developed epilepsy after resolved encephalitis.ConclusionEpilepsy after resolved encephalitis was rare in our cohort of patients with AIE treated with immunotherapy. In addition, seizure freedom is achieved faster and more frequently after immunotherapy. Therefore, AEDs should be considered as add-on treatment, and similar to treatment of other encephalitis symptoms, immunotherapy is crucial.

Compulsive Behaviors as Presenting Symptoms of Frontotemporal Dementia

1997 ◽  
Vol 10 (4) ◽  
pp. 154-157 ◽  
Author(s):  
Mario F. Mendez ◽  
Kent M. Perryman ◽  
Bruce L. Miller ◽  
J. Randolph Swartz ◽  
Jeffrey L. Cummings
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Single Photon ◽  
Photon Emission ◽  
Presenting Symptoms ◽  
Younger Age ◽  
Dementia Syndrome ◽  
Compulsive Behaviors ◽  
Neurodegenerative Dementia

Frontotemporal dementia (FTD) is a common neurodegenerative dementia syndrome. Compulsive behaviors frequently occur in FTD and may be presenting symptoms of this disorder. This study evaluated compulsive behaviors as presenting symptoms in 29 patients with FTD compared to 48 patients with Alzheimer's disease (AD) enrolled in the UCLA Alzheimer's Disease Center. The FTD patients met the Lund and Manchester criteria for FTD and had predominant frontal hypoperfusion on single-photon emission computer tomography neuroimaging. The AD patients met National Institute of Neurological and Communicative Disorders-Alzheimer's Disease and Related Disorders criteria for clinically probable AD. Compulsive behaviors occurred in 11 FTD patients (38%) versus 5 AD patients (10%) (χ2 = 6.73, P < .01). This difference persisted after controlling for the younger age of the FTD group. There was a range of compulsive behaviors, with the most frequent being repetitive checking activities. Compulsive behaviors are common presenting symptoms among FTD patients and may result from an inability to inhibit urges to perform compulsive movements from damage to frontal-striatal circuits.

scholarly journals Does CSF pleocytosis have a predictive value for disease course in MS?

2019 ◽  
Vol 6 (5) ◽  
pp. e584 ◽  
Author(s):  
Itay Lotan ◽  
Felix Benninger ◽  
Rom Mendel ◽  
Mark A. Hellmann ◽  
Israel Steiner
Keyword(s):  
Clinical Course ◽  
Medical Center ◽  
Oligoclonal Bands ◽  
Disease Course ◽  
Disability Status ◽  
Relapsing Remitting ◽  
Csf Pleocytosis ◽  
Csf Oligoclonal Bands ◽  
Rabin Medical

ObjectiveMS is a demyelinating CNS disorder with a spectrum of clinical patterns regarding course and prognosis. Although several prognostic factors are considered in the initial evaluation of patients, biological markers defining the disease course and guiding treatments are currently lacking. It is unknown whether patients with CSF pleocytosis differ in regard to symptoms, disease course, and prognosis from those without. The aim of this study was to evaluate whether CSF pleocytosis during the initial presentation has an impact on the clinical course and progression of MS.MethodsWe retrospectively evaluated patients attending the MS Clinic at Rabin Medical Center between January 1999 and January 2016 who underwent lumbar puncture (LP) at disease presentation, considering CSF cell count, clinical diagnosis (clinically isolated syndrome [CIS] and relapsing-remitting MS [RRMS]), annualized relapse rate (ARR), paraclinical findings (imaging, CSF oligoclonal bands, and evoked potentials), and disease progression, expressed by the Expanded Disability Status Scale (EDSS).ResultsOne hundred fourteen patients (72 females) underwent LP at disease presentation (RRMS: n = 100, CIS: n = 14). Age at diagnosis was 32.4 ± 12.2 years, and the follow-up time was 9.4 ± 3.8 years. Forty-six patients showed a pleocytic CSF (≥5 cells per μL). Compared with patients with <4 cells per μL, patients with pleocytosis had a higher ARR (0.60 ± 0.09 vs 0.48 ± 0.04; p = 0.0267) and a steeper increase (slope) in the EDSS score throughout the follow-up period (correlation coefficient: r2 = 0.04; p = 0.0251).ConclusionsCSF pleocytosis may be considered a biological unfavorable predictive factor regarding disease course and progression in MS.

scholarly journals Seizure-related 6 homolog like 2 autoimmunity

2020 ◽  
Vol 8 (1) ◽  
pp. e916
Author(s):  
Jon Landa ◽  
Mar Guasp ◽  
Mar Petit-Pedrol ◽  
Eugenia Martínez-Hernández ◽  
Jesús Planagumà ◽  
...  
Keyword(s):  
Hippocampal Neurons ◽  
Brain Mri ◽  
Index Patient ◽  
Clinical Syndrome ◽  
Extrapyramidal Symptoms ◽  
Hek293 Cells ◽  
Neuronal Cultures ◽  
Neurologic Disorders ◽  
A Cell ◽  
Csf Pleocytosis

ObjectiveTo describe the clinical syndrome of 4 new patients with seizure-related 6 homolog like 2 antibodies (SEZ6L2-abs), study the antibody characteristics, and evaluate their effects on neuronal cultures.MethodsSEZ6L2-abs were initially identified in serum and CSF of a patient with cerebellar ataxia by immunohistochemistry on rat brain sections and immunoprecipitation from rat cerebellar neurons. We used a cell-based assay (CBA) of HEK293 cells transfected with SEZ6L2 to test the serum of 95 patients with unclassified neuropil antibodies, 331 with different neurologic disorders, and 10 healthy subjects. Additional studies included characterization of immunoglobulin G (IgG) subclasses and the effects of SEZ6L2-abs on cultures of rat hippocampal neurons.ResultsIn addition to the index patient, SEZ6L2-abs were identified by CBA in 3/95 patients with unclassified neuropil antibodies but in none of the 341 controls. The median age of the 4 patients was 62 years (range: 54–69 years), and 2 were female. Patients presented with subacute gait ataxia, dysarthria, and mild extrapyramidal symptoms. Initial brain MRI was normal, and CSF pleocytosis was found in only 1 patient. None improved with immunotherapy. SEZ6L2-abs recognized conformational epitopes. IgG4 SEZ6L2-abs were found in all 4 patients, and it was the predominant subclass in 2. SEZ6L2-abs did not alter the number of total or synaptic SEZ6L2 or the AMPA glutamate receptor 1 (GluA1) clusters on the surface of hippocampal neurons.ConclusionsSEZ6L2-abs associate with a subacute cerebellar syndrome with frequent extrapyramidal symptoms. The potential pathogenic effect of the antibodies is not mediated by internalization of the antigen.

scholarly journals The diffuse involvement of anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yun Jiang ◽  
Jianpeng Ma ◽  
Tao Gong ◽  
Hongjun Hao ◽  
Haibo Chen
Keyword(s):  
Basal Ganglia ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Mass Effects ◽  
Glucose Levels ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

Relationship between brain MRI lesion load and short-term disease evolution in non-disabling MS: a large-scale, multicentre study

2010 ◽  
Vol 17 (3) ◽  
pp. 319-326 ◽  
Author(s):  
Marco Rovaris ◽  
Maria A Rocca ◽  
Frederik Barkhof ◽  
Massimiliano Calabrese ◽  
Nicola De Stefano ◽  
...  
Keyword(s):  
Clinical Data ◽  
Disease Duration ◽  
Brain Mri ◽  
Large Population ◽  
Lesion Volume ◽  
Disease Course ◽  
Short Term ◽  
Disease Evolution ◽  
Edss Score

Background and Objectives: We evaluated clinical and conventional MRI features of a large population of patients with non-disabling MS to identify potential markers of a benign disease course. Methods: In seven MAGNIMS centres we retrospectively identified 182 patients with benign (B) MS (EDSS score ≤3.0, disease duration ≥15 years) and 187 patients with non-disabling relapsing–remitting MS (NDRRMS) (Expanded Disability Status Scale score ≤3.0, disease duration between 5 and 14 years), in whom clinical data were collected within two weeks from a brain T2-weighted scan. Brain T2 lesion volume (LV) was measured in all patients. In 146 BMS and 146 NDRRMS patients, clinical data were also available after a median follow up of 29 months (range: 7–104 months). Results: Mean LV was higher in BMS than in NDRRMS patients ( p < 0.001), but the mean ratio between LV and disease duration was higher in NDRRMS than in BMS patients (1.1 vs. 0.6 ml/year, p < 0.001). In BMS patients, brain LV was correlated with EDSS score increase at follow up ( r = 0.18, p = 0.03). Conclusions: An overall low rate of brain LV increase during a long-lasting disease course might be a feature of BMS. In BMS patients, a high brain LV might be associated with worsening of locomotor disability at short-term follow up.

scholarly journals The diffuse involvement of Anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

2019 ◽  
Author(s):  
Yun Jiang ◽  
Jianpeng Ma ◽  
Tao Gong ◽  
Hongjun Hao ◽  
Haibo Chen
Keyword(s):  
Basal Ganglia ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Mass Effects ◽  
Glucose Levels ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. The anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special anti-NMDAR encephalitis patient who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, and the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions The anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. The recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

scholarly journals Autoantibody-associated psychiatric syndromes: a systematic literature review resulting in 145 cases

2020 ◽  
pp. 1-12 ◽  
Author(s):  
Dominique Endres ◽  
Viktoria Maier ◽  
Frank Leypoldt ◽  
Klaus-Peter Wandinger ◽  
Belinda Lennox ◽  
...  
Keyword(s):  
Literature Review ◽  
Cell Surface ◽  
Systematic Literature Review ◽  
Case Reports ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Immunomodulatory Drugs ◽  
Cell Surface Antigens ◽  
Medline Search ◽  
Neurodegenerative Dementia

Abstract Background Autoimmune encephalitis (AE) is an important consideration during the diagnostic work-up of secondary mental disorders. Indeed, isolated psychiatric syndromes have been described in case reports of patients with underlying AE. Therefore, the authors performed a systematic literature review of published cases with AE that have predominant psychiatric/neurocognitive manifestations. The aim of this paper is to present the clinical characteristics of these patients. Methods The authors conducted a systematic Medline search via Ovid, looking for case reports/series of AEs with antineuronal autoantibodies (Abs) against cell surface/intracellular antigens combined with predominant psychiatric/neurocognitive syndromes. The same was done for patients with Hashimoto encephalopathy/SREAT. Only patients with signs of immunological brain involvement or tumors in their diagnostic investigations or improvement under immunomodulatory drugs were included. Results We identified 145 patients with AE mimicking predominant psychiatric/neurocognitive syndromes. Of these cases, 64% were female, and the mean age among all patients was 43.9 (±22.1) years. Most of the patients had Abs against neuronal cell surface antigens (55%), most frequently against the NMDA-receptor (N = 46). Amnestic/dementia-like (39%) and schizophreniform (34%) syndromes were the most frequently reported. Cerebrospinal fluid changes were found in 78%, electroencephalography abnormalities in 61%, and magnetic resonance imaging pathologies in 51% of the patients. Immunomodulatory treatment was performed in 87% of the cases, and 94% of the patients responded to treatment. Conclusions Our findings indicate that AEs can mimic predominant psychiatric and neurocognitive disorders, such as schizophreniform psychoses or neurodegenerative dementia, and that affected patients can be treated successfully with immunomodulatory drugs.

scholarly journals Antibody-associated CNS syndromes without signs of inflammation in the elderly

Neurology ◽  
2017 ◽  
Vol 89 (14) ◽  
pp. 1471-1475 ◽  
Author(s):  
Domingo Escudero ◽  
Mar Guasp ◽  
Helena Ariño ◽  
Carles Gaig ◽  
Eugenia Martínez-Hernández ◽  
...  
Keyword(s):  
Neurodegenerative Disorder ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Surface Antigens ◽  
Clinical Analysis ◽  
Correct Identification ◽  
Cognitive Deterioration ◽  
Antibody Testing

Objective:To report the CNS syndromes of patients ≥60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes.Methods:This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ≥60 years, no inflammatory abnormalities in brain MRI, and no CSF pleocytosis. Antibodies were determined with reported techniques.Results:Among 155 patients ≥60 years of age with neurologic syndromes related to antibodies against neuronal surface antigens, 35 (22.6%) fulfilled the indicated criteria. The median age of these 35 patients was 68 years (range 60–88 years). Clinical manifestations included faciobrachial dystonic seizures (FBDS) in 11 of 35 (31.4%) patients, all with LGI1 antibodies; a combination of gait instability, brainstem dysfunction, and sleep disorder associated with IgLON5 antibodies in 10 (28.6%); acute confusion, memory loss, and behavioral changes suggesting autoimmune encephalitis (AE) in 9 (25.7%; 2 patients with AMPAR, 2 with NMDAR, 2 with GABAbR, 2 with LGI1, and 1 with CASPR2 antibodies); and rapidly progressive cognitive deterioration in 5 (14.3%; 3 patients with IgLON5 antibodies, 1 with chorea; 1 with DPPX antibody–associated cerebellar ataxia and arm rigidity; and 1 with CASPR2 antibodies).Conclusions:In patients ≥60 years of age, the correct identification of characteristic CNS syndromes (FBDS, anti-IgLON5 syndrome, AE) should prompt antibody testing even without evidence of inflammation in MRI and CSF studies. Up to 15% of the patients developed rapidly progressive cognitive deterioration, which further complicated the differential diagnosis with a neurodegenerative disorder.

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