PURPOSE: In line with the various functions of the insula and its widespread network of connections, insular epilepsy may feature a variety of early ictal manifestations from somatosensory, visceral, olfactory, gustatory, or vestibular manifestations. In this study, we report a new, not described yet clinical autonomic feature related to non-dominant anterior insular lobe seizures with ipsilateral cyanotic distal coloration with “glove and sock” representation.

METHODS: A 17 years old girl has been diagnosed with epilepsy related to lesion in right insula. She had pharmacoresistant seizures with vomiting, laryngeal constriction. She has been evaluated with interictal and interictal long term video-EEG monitoring which showed epileptiform discharges in right TP area, results concordant with the lesion described by imagistic evaluations (cerebral MRI – hyperintense T2 and Flair lesion in right insula and FDG-PET – hypometabolism same area).

She has been operated and the right insula was partial removed, with a small remnant of anterior superior short gyri. The anatomo-pathological exam demonstrated focal cortical dysplasia type IIa.

After the surgical intervention, she started to have again her typical seizures and also other episodic events, only with distal modifications of the skin color as cyanosis with representation in ‘glove and sock’. The episodes have been video-EEG monitored and have been demonstrated as being seizures.

RESULTS: She has been re-operated with complete removal of the remnant insula with good outcome, without any other seizures.

CONCLUSIONS: Unilateral objective skin coloration with representation in ‘glove and sock’ can be seizures related to focal cortical anterior type IIa anterior-superior insula.