Cerebral vasculitis and multi-focal neurological deficits due to allopurinol-induced hypersensitivity syndrome

Abstract

Drug-induced hypersensitivity syndrome (DIHS) is a multi-system syndrome resulting from an idiosyncratic reaction to medication. While it commonly results in multi-organ involvement, particularly the liver, there are few reports of DIHS causing cerebral vasculitis and neurological deficits. We report the case of a 63-year old woman with DIHS secondary to allopurinol leading to multiple neurological deficits with magnetic resonance imaging findings consistent with a cerebral vasculitis.

CT findings in a patient with bilateral metachronous carotidynia

“…It is unclear whether allopurinol administered to our patient prior to the first episode of carotidynia had a role in the development of the initial bout of symptoms. However, two cases in the literature describe a systemic hypersensitivity response to allopurinol resulting in cerebral vasculitis [17,18].…”

DRESS syndrome: cerebral vasculitic-like presentation

“…A fourth case was reported by Lim [11]. A 63-year-old woman with DRESS syndrome associated to allopurinol.…”

“…The management is the immediate cessation of the offending drug. High-dose systemic steroids, typically tapered slowly over 4-12 months, are the mainstay of treatment of the more severe cases [11]. While Tohyama [16] and others have demonstrated an association with HHV-6 reactivation at initial presentation and relapse, treatment with systemic corticosteroids alone is adequate in most cases.…”

Successful treatment of DRESS syndrome with plasmapheresis during the course of sero-negative autoimmune encephalitis: a case report

“…There are also reports on DRESS syndrome triggering cerebral vasculitis [23,24]. In a recent review by Y. Kano et al, it is suggested that regulatory T cells (Tregs) loss of function may be responsible for the association.…”

“…In a recent review by Y. Kano et al, it is suggested that regulatory T cells (Tregs) loss of function may be responsible for the association. Systemic corticosteroids administration may prevent the gradual loss of Treg function by repairing Treg activity [23]. Similarly, with plasmapheresis, the balance between Treg and other immunoactive cells is restored perhaps averting the pathology of the disease.…”