Systemic mastocytosis is a rare disorder involving the proliferation and accumulation of mast cells in extracutaneous tissues due to a gain of function mutation in the KIT tyrosine kinase gene. The amount of mast cell infiltration, the presence of specific KIT mutations, and serum tryptase levels all aid in the diagnosis of systemic mastocytosis. Neurologic complications, including headaches, back pain, seizures, and changes in consciousness, are rare and have only been described in case reports.

There is only a single case in the literature of seizures in a patient with systemic mastocytosis. The potential development of seizures, and other neurological symptoms, in systemic mastocytosis is theorized to be related to the release of vasoactive substances, including histamine, and from mast cell infiltration within the meninges. Our case highlights the importance of considering seizures in the differential diagnosis of paroxysmal spells in patients with a systemic mastocytosis.