Abstract
ObjectiveTo evaluate the diagnostic accuracy and safety of extended stereotactic brain biopsy (ESBB) in a single center cohort with suspected primary angiitis of the central nervous system (PACNS).MethodsA standardized stereotactic biopsy targeting MRI-positive lesions and collecting samples from the meninges and the cortex as well as from the white matter was performed in 23 patients with clinically suspected PACNS between 2010 and 2017. The relationship between biopsy yield and clinical characteristics, cerebrospinal fluid parameters, MR-imaging, time point of biopsy and exact localization of biopsy as well as number of tissue samples were examined.ResultsPACNS was confirmed in 7 of 23 patients (30.4%). Alternative diagnoses were identified in 7 patients (30%). A shorter time period between the onset or worsening of symptoms (p = 0.018) and ESBB significantly increased the diagnostic yield. We observed only minor and transient postoperative complications in 3 patients (13.0%). ESBB led to a direct change of the therapeutic regime in 13 of 23 patients (56.5%). Careful neuropathological analysis furthermore revealed that cortical samples were crucial in obtaining a diagnosis.ConclusionESBB is a safe approach with good feasibility, even in critically ill patients, and high diagnostic accuracy in patients with suspected PACNS changing future therapies in 13 of 23 patients (56.5%). Early biopsy after symptom onset/worsening is crucial and (sub)acute MRI-lesions should be targeted with a particular need for biopsy samples from the cortical layer.
Highlights
Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disorder with an estimated annual incidence rate of 2–3 per one million individuals [1, 2]
Between 2010 and 2017, 23 patients (11 females, 48%) received extended stereotactic brain biopsy (ESBB) for clinically suspected or at least possible PACNS according to current guidelines and after thorough clinical work-up without a definitive diagnosis
Stereotactic brain biopsy of an ischemic or contrast enhancing lesion was conducted in each patient (n = 23; 100%), an additional cortical surface and meningeal biopsy in 19 patients (83%)
Summary
Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disorder with an estimated annual incidence rate of 2–3 per one million individuals [1, 2]. Tissue sampling from the meninges, the cortical surface and the deep white matter is considered the most accurate way to achieve a definite diagnosis [3, 4, 7] Criticism of this approach stems from alleged high false negative rates (I) invasiveness of the procedure, (II) the notion that biopsies cannot be repeated multiple times in one individual, (III) and lack of standardized protocols (IV) [2]. Frame-less craniotomy techniques and stereotactic, frame-based approaches have been proposed It remains unclear, whether biopsy of the cortical surface or of deep-seated white matter lesions is more likely to yield a positive histological result for PACNS or differential diagnoses
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