Nonparaneoplastic Anti-GAD Limbic Encephalitis: Seizure Outcome and Long-term Neuropsychological Follow-up After Immunotherapy.

Antibodies against glutamate decarboxylase (GAD-Abs), especially GAD65 antibodies, are associated with limbic encephalitis (LE) manifested by temporal lobe epilepsy and neuropsychological deficits. We present the case of a 42-year-old Greek woman with nonparaneoplastic anti-GAD LE, discussing the therapeutic management and highlighting the role of neuropsychological assessment. The patient underwent functional and structural brain studies and was investigated longitudinally over a 6-year period with a battery of neuropsychological tests that were designed to document her intellectual function and verbal and visual memory. The patient suffered from refractory temporal-impaired awareness seizures and memory impairment that was mediated by autoimmune nonparaneoplastic LE and comorbid autoimmune disorders (ie, Hashimoto thyroiditis and vitiligo). Neuroimaging studies demonstrated hyperintensities in the medial temporal lobes bilaterally on T2WI MRI sequences. Serial EEGs showed bitemporal intermittent delta activity as well as epileptiform discharges. Tumor blood markers and onconeural antibodies were negative. Immunological screening revealed extremely high GAD-Abs titers in both serum and CSF, as well as the presence of CSF oligoclonal bands. Neuropsychological testing revealed anterograde amnesia with relative preservation of more remote, premorbid memories. The patient underwent first-line immunotherapy followed by immunosuppressive maintenance treatment that led to a reduction of seizures, EEG improvement, and a significant decline in GAD-Abs titers. Neuropsychological evaluations at 5 months, 1 year, and 6 years posttreatment demonstrated improvement, particularly in recent memory and everyday functionality. In this case of anti-GAD LE, the long-term seizure reduction and the improvement of neuropsychological deficits were most likely related to the immunotherapy.

An atypical meningioma demystified and advanced magnetic resonance imaging techniques.

A 40-year-old male presented with visuospatial processing disturbances. Family history was free. Conventional and advanced magnetic resonance imaging (MRI) studies were performed. On T2 and fluid attenuation inversion recovery images, an increased signal intensity extra-axial lesion was demonstrated. Post-contrast scans depicted homogeneous intense contrast medium enhancement. T2* star sequence was negative for hemorrhagic or calcification foci. Diffusion-weighted imaging findings were indicative of malignant behavior and magnetic resonance venography confirmed superior sagittal sinus infiltration. Increased cerebral blood volume values were observed and peri-lesional oedema on perfusion-weighted imaging was also demonstrated. The signal intensity-time curve depicted the characteristic meningioma pattern. Spectroscopy showed increased choline and alanine levels, but decreased N-acetyl-aspartate levels. Conventional MRI is adequate for typical types of meningiomas. However, the more atypical ones, in which even the histopathologic specimen may demonstrate characteristics of typical meningioma, could be easier diagnosed with advanced MRI techniques.