Background Anti-γ-aminobutyric acid A receptor (GABAA receptor) encephalitis is a rare but severe subtype of autoimmune encephalitis (AE), typically characterized by refractory seizures and rapidly progressive neurological dysfunction. Although structural abnormalities on magnetic resonance imaging (MRI) are frequently observed, the temporal relationships among clinical manifestations, electrophysiological changes, immune activity, and neuroimaging findings remain insufficiently characterized. Case presentation We report a 61-year-old man with anti-GABAA receptor encephalitis who underwent longitudinal multimodal evaluation. In the early stage, the patient presented with nonspecific vestibular symptoms accompanied by subtle cortical abnormalities on MRI. The disease rapidly progressed to status epilepticus and cognitive impairment. Electroencephalography (EEG) revealed widespread epileptiform discharges, while cerebrospinal fluid (CSF) analysis demonstrated inflammatory changes, and both serum and CSF were positive for anti-GABAA receptor antibodies. Following timely immunotherapy, the patient exhibited rapid clinical improvement, marked attenuation of epileptiform activity, and subsequent antibody negativization. Notably, structural MRI abnormalities persisted and resolved more slowly than clinical and electrophysiological recovery. Translocator protein positron emission tomography (TSPO-PET) using the 18F-DPA714 demonstrated increased tracer uptake in the bilateral temporo-parieto-occipital cortices, indicating persistent neuroinflammation despite clinical improvement. Conclusions This case provides longitudinal multimodal observations supporting the concept that anti-GABAA receptor encephalitis may represent a dynamic and potentially reversible disorder of network hyperexcitability rather than a purely structural inflammatory injury. The temporal dissociation between functional recovery and delayed structural resolution highlights the critical value of multimodal monitoring, particularly EEG and immunological biomarkers, in assessing disease activity and guiding immunotherapy.
During disease progression, innovative sequential therapy with efgartigimod, an FcRn antagonist, was followed by the anti-CD20 monoclonal antibody rituximab (RTX) to reduce the risk of profound B-cell depletion, thereby demonstrating synergistic efficacy.
Jun-Tong Liu, Wen-Ping Zhu, Lan Gao et al.· Frontiers in Immunology· 1 citation
Findings support the possibility that anti-mGluR1 encephalitis may, in rare instances, present with predominant brainstem involvement and testing for anti-mGluR1 antibodies may be considered in patients with otherwise unexplained subacute inflammatory brainstem syndromes.
Although the unilateral conventional imaging findings did not meet the bilateral mesial temporal criterion required for definite autoimmune limbic encephalitis, MR relaxometry revealed bilateral temporomesial abnormalities, this case highlights a rare presentation of antibody-atypical limbic encephalitis in the setting...
Florian Awin Stolz, Andrei Manzhurtsev, M. Bönstrup et al.· Frontiers in Radiology· 0 citations
Objective: Among autoimmune limbic encephalitis syndromes, glutamic acid decarboxylase 65 (GAD65) antibody-associated syndromes represent a relatively rare and clinically heterogeneous subtype characterized by prominent temporal lobe involvement and diverse neurological manifestations. GAD65 is an intracellular antigen...
Matthew S. Phillips, Malorie E. Watson, Brian Ramanauskas et al.· Clinical Neuropsychologist· 0 citations
Re demonstrated heterogeneous electroclinical and neuroradiological evolution across pediatric and adult patients, and the coexistence of an older age profile and a predominance of left hemisphere involvement may suggest age-related phenotypic variability.
Ülkühan Öztoprak, C. Günbey, Rahşan Göçmen et al.· Brain & development (Tokyo....· 0 citations
Anti-N-methyl-D-aspartate receptor encephalitis frequently presents with isolated psychiatric symptoms indistinguishable from primary psychosis, delaying diagnosis. The authors have described a previously healthy 35-year-old woman admitted with abrupt first-episode psychosis, who (within 48 hours and without seizures,...
M. Basta, C. Bikis, A. Miliaras et al.· The Permanente Journal· 0 citations
We use cookies to run the site and, with your consent, for analytics and to show ads.
See our Cookie Policy.