scholarly journals Gastaut-Geschwind Syndrome, Faciobrachial Dystonic Seizure, and Autoimmune Limbic Encephalitis

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Ori-Michael J. Benhamou ◽  
Mohammad Tavakkoli ◽  
Hande Okan ◽  
Mitchell Nobler
Keyword(s):  
Immune Globulin ◽  
Limbic Encephalitis ◽  
Symptomatic Relief ◽  
Behavioral Changes ◽  
Intravenous Immune Globulin ◽  
Voltage Gated ◽  
Autoimmune Limbic Encephalitis ◽  
Potassium Complex ◽  
Recent Falls

Here we report a case of a 55-year-old male who had presented with recent falls and behavioral changes, including a heightened religious preoccupation, hypergraphia, and paranoid ideations. He was initially treated for psychosis but soon exhibited absence-like seizures, which were consistent with faciobrachial dystonic seizures. Workup for underlying infectious, immunodeficiency, and autoimmune causes revealed antibodies towards the leucine-rich glioma inactivated subunit of the voltage-gated potassium complex. The patient was treated with steroids and intravenous immune globulin with symptomatic relief. In retrospect, the patient met criteria for Gastaut-Geschwind (GG) syndrome, with notable features of hypergraphia and hyperreligiosity. This case illustrates how the GG syndromal pattern contributes to the suspicion of autoimmune limbic encephalitis and may expedite diagnosis and prevent the accumulation of disability.

Autoimmune limbic encephalitis and Morvan’s syndrome

2010 ◽  
pp. 5175-5176
Author(s):  
Camilla Buckle ◽  
Angela Vincent
Keyword(s):  
Cell Surface ◽  
Short Term Memory ◽  
Limbic Encephalitis ◽  
Memory Loss ◽  
Surface Proteins ◽  
Cell Surface Proteins ◽  
Term Memory ◽  
Voltage Gated ◽  
Morvan’S Syndrome ◽  
Autoimmune Limbic Encephalitis

Autoimmune limbic encephalitis—typical presentation is with acute or subacute onset of short term memory loss, seizures and disorientation. MRI characteristically shows striking abnormalities in the hippocampus. Antibodies against cell-surface proteins that are components of voltage-gated potassium channel (VGKC) complexes are found in a high proportion and are probably pathogenic. Aside from supportive care, treatment is with immunosuppression, often comprising corticosteroids with intravenous immunoglobulin and/or plasma exchange....

Limbic encephalitis with voltage gated potassium channel antibodies in a child

2008 ◽  
Vol 39 (01) ◽  
Author(s):  
E Haberlandt ◽  
CG Bien ◽  
A Reiter ◽  
B Simma ◽  
R Crazzolara ◽  
...  
Keyword(s):  
Potassium Channel ◽  
Limbic Encephalitis ◽  
Voltage Gated

Voltage-gated potassium channel limbic encephalitis presenting as functional cognitive impairment

2020 ◽  
Vol 13 (12) ◽  
pp. e233179
Author(s):  
Eric Garrels ◽  
Fawziya Huq ◽  
Gavin McKay
Keyword(s):  
Cognitive Impairment ◽  
Case Report ◽  
Potassium Channel ◽  
Limbic Encephalitis ◽  
Psychiatric Symptoms ◽  
Differential Diagnoses ◽  
Visual Hallucinations ◽  
Voltage Gated ◽  
History Of ◽  
Impaired Cognition

Limbic encephalitis is often reported to present as seizures and impaired cognition with little focus on psychiatric presentations. In this case report, we present a 49-year-old man who initially presented to the Psychiatric Liaison Service with a several month history of confusion with the additional emergence of visual hallucinations and delusions. Due to the inconsistent nature of the symptoms in the context of a major financial stressor, a provisional functional cognitive impairment diagnosis was made. Investigations later revealed a positive titre of voltage-gated potassium channel (VGKC) antibodies, subtype leucine-rich glioma inactivated 1 accounting for his symptoms which dramatically resolved with steroids and immunoglobulins. This case highlighted the need for maintaining broad differential diagnoses in a patient presenting with unusual psychiatric symptoms.

Intravenous Immune Globulin in the Therapy of Myocarditis and Acute Cardiomyopathy

Circulation ◽  
1997 ◽  
Vol 95 (11) ◽  
pp. 2476-2478 ◽  
Author(s):  
Dennis M. McNamara ◽  
Warren D. Rosenblum ◽  
Karen M. Janosko ◽  
Mary K. Trost ◽  
Flordeliza S. Villaneuva ◽  
...  
Keyword(s):  
Immune Globulin ◽  
Intravenous Immune Globulin

scholarly journals Frontotemporal Dementia with Parkinsonism and Epilepsy Associated with VGKC Antibodies: Case Report and Literature Review

2021 ◽  
pp. 205-210
Author(s):  
Matthew Saint ◽  
Vafa Alakbarzade ◽  
Brendan McLean
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Frontotemporal Dementia ◽  
Limbic Encephalitis ◽  
Poor Response ◽  
Double Negative ◽  
Aggressive Treatment ◽  
Voltage Gated ◽  
Negative Results ◽  
Autoimmune Conditions

Antibodies directed against the voltage-gated potassium channel complex (anti-VGKCs) are implicated in several autoimmune conditions including limbic encephalitis and epilepsy. However, emerging evidence suggests that only specific subtypes of anti-VGKCs are pathogenic. We present the case of a 55-year-old man who initially presented with focal unaware seizures and behavioural changes mimicking anti-VGKC-seropositive encephalitis that further progressed to parkinsonism with evidence of frontotemporal dementia and pre-synaptic dopaminergic deficit. Aggressive treatment with immunotherapy was ineffective, and antibody subtyping later revealed the anti-VGKC antibodies to be negative for leucine-rich glioma-associated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) – the two known pathogenic subtypes. The clinical relevance of so-called “double-negative” anti-VGKCs (i.e., those not directed towards LGI1 or CASPR2) has been called into question in recent years, with evidence to suggest they may be clinically insignificant. Our case emphasises the importance of antibody subtyping in cases of anti-VGKC seropositivity; negative results, particularly when combined with a poor response to immunotherapy, should prompt a rapid reconsideration of the working diagnosis.

ASL MRI and 18F-FDG-PET in autoimmune limbic encephalitis: clues from two paradigmatic cases

2021 ◽  
Author(s):  
Alessandro Dinoto ◽  
Marta Cheli ◽  
Miloš Ajčević ◽  
Franca Dore ◽  
Carmelo Crisafulli ◽  
...  
Keyword(s):  
Fdg Pet ◽  
Limbic Encephalitis ◽  
Autoimmune Limbic Encephalitis ◽  
18F Fdg
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