Alice in wonderland syndrome “through the looking-glass” in a rare presentation of non-convulsive status epilepticus in cerebral venous sinus thrombosis and COVID-19

Cortex. 2023 Jul 24;167:218-222. doi: 10.1016/j.cortex.2023.06.020. Online ahead of print.

ABSTRACT

Alice in Wonderland Syndrome (AIWS) is a rare perceptual disorder, rarely associated with epileptic etiology. We report the case of a 23-year-old man with subacute onset of right peri-orbital headache and visual misperceptions consistent with AIWS Type B, who underwent laboratory tests, brain CT with venography, ophthalmic examination, and neurological assessment that turned out to be normal except for visuospatial difficulties and constructional apraxia. A nasopharyngeal SARS-CoV2 swab taken as screening protocol was positive. The EEG performed because of the persistence of AIWS showed a focal right temporo-occipital non-convulsive status epilepticus; a slow resolution of clinical and EEG alterations was achieved with anti-seizure medications. Brain MRI showed right cortical temporo-occipital signal abnormalities consistent with peri-ictal changes and post-contrast T1 revealed a superior sagittal sinus thrombosis, thus anticoagulant therapy was initiated. AIWS is associated with temporo-parieto-occipital carrefour abnormalities, where visual and somatosensory inputs are integrated to generate the representation of body schema. In this patient, AIWS is caused by temporo-occipital status epilepticus without anatomical and electroencephalographic involvement of the parietal region, consistent with the absence of somatosensory symptoms of the syndrome. Status epilepticus can be the presenting symptom of cerebral venous sinus thrombosis (CVST) which, in this case, is possibly due to the hypercoagulable state associated with COVID-19.

PMID:37572532 | DOI:10.1016/j.cortex.2023.06.020