Original article Primary angiitis of the central nervous system: a study of histopathological patterns and review of the literature

Abstract

A b s t r a c t

Intracranial Atherosclerosis Versus Primary Angiitis of the Central Nervous System: a Case Report

“…As per Hajj-Ali et al, the most common imitator is a group of disorders known as the reversible cerebral vasoconstriction syndromes (RCVS) which includes: migrainous vasospasm, Call-Fleming syndrome, thunderclap headache associated with vasospasm, drug-induced cerebral arteritis, postpartum cerebral angiopathy, benign angiopathy of the CNS, and CNS pseudovasculitis [ 2 ]. Other conditions that should be considered in the differential, as Noufal and Schmidley and Suri et al describe, include but are not limited to: atrial myxoma with embolization to the brain, secondary vasculitides, collagen vascular disorders, viral or bacterial infections, neoplasms, hypercoagulable states, and substance abuse [ 4 - 5 ].…”

Mass‐forming primary angiitis of central nervous system with Rosai‐Dorfmann disease‐like massive histiocytosis with emperipolesis

“…Primary angiitis of the central nervous system is defined as a rare idiopathic vasculitis confined to the CNS without being a systemic disease . The exact incidence and prevalence of PACNS is unknown, since they are extremely rare.…”

“…Hence, surgery yielded an optimal clinical outcome for our patient. However, it is important to consider that a combination of steroid and pulsed cyclophosphamide is recommended for primary treatment . Most of the previously reported mass‐forming PACNS cases had been improved clinically and/or on image follow‐up with surgical resection only or with adjuvant steroid or cyclophosphamide therapies after biopsy (Table ).…”

“…Primary angiitis of the central nervous system (PACNS) is a rare non‐infectious, CNS‐restricted vasculitis. Histopathologically, it can include various types of vasculitis, such as granulomatous, lymphocytic, or necrotizing (neutrophilic) vasculitis with hypoxic/ischemic changes in the affected region . The precise diagnosis of PACNS is very challenging due to multifarious clinical manifestations, non‐specific MRI findings, unknown etiology, as well as difficulty in getting tissue‐biopsy.…”

“…Therefore, diagnosis of PACNS can be made by excluding other possible diagnoses. However, in the case of PACNS, an early diagnosis and prompt treatment are essential to avoid incorrect treatment and permanent brain damage …”