TY - JOUR
T1 - Teaching NeuroImage
T2 - Calcifying Pseudoneoplasm of the Neuraxis in the Setting of Hereditary Hemorrhagic Telangiectasia and Seizures
AU - Debs, Luca H.
AU - Helton, Austin
AU - Belakhlef, Sami
AU - Sharma, Suash
AU - Rahimi, Scott Y.
N1 - Publisher Copyright:
© American Academy of Neurology.
PY - 2023/8/29
Y1 - 2023/8/29
N2 - We report a 50-year-old man presenting with grand-mal seizures. Workup included MRI of the brain demonstrating a heterogeneously enhancing focus, likely hemorrhagic, in the left occipital region (Figure 1). A vascular etiology was presumed due to the patient's history of Hereditary Hemorrhagic Telangiectasia. Furthermore, digital subtraction angiography revealed an irregular blush with the absence of early venous drainage to suggest an arteriovenous malformation (AVM). On surgical resection, the specimen showed substantial calcifications (Figure 2). Final pathology diagnosed a calcifying pseudoneoplasm of the neuraxis (CAPNON), presumably arising from an AVM remnant.1 They are rare, slow-growing lesions believed to form secondary to tissue insult. The benefits of this finding over an AVM are 2-fold; cerebral autoregulation is maintained, and future surveillance angiograms are avoidable. CAPNONs have been observed after trauma, infections, neoplasms, and inflammation.2 Complete resection was confirmed by intraoperative angiogram and postoperative MRI. The patient had no complications and returned to his seizure-free neurologic baseline.
AB - We report a 50-year-old man presenting with grand-mal seizures. Workup included MRI of the brain demonstrating a heterogeneously enhancing focus, likely hemorrhagic, in the left occipital region (Figure 1). A vascular etiology was presumed due to the patient's history of Hereditary Hemorrhagic Telangiectasia. Furthermore, digital subtraction angiography revealed an irregular blush with the absence of early venous drainage to suggest an arteriovenous malformation (AVM). On surgical resection, the specimen showed substantial calcifications (Figure 2). Final pathology diagnosed a calcifying pseudoneoplasm of the neuraxis (CAPNON), presumably arising from an AVM remnant.1 They are rare, slow-growing lesions believed to form secondary to tissue insult. The benefits of this finding over an AVM are 2-fold; cerebral autoregulation is maintained, and future surveillance angiograms are avoidable. CAPNONs have been observed after trauma, infections, neoplasms, and inflammation.2 Complete resection was confirmed by intraoperative angiogram and postoperative MRI. The patient had no complications and returned to his seizure-free neurologic baseline.
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U2 - 10.1212/WNL.0000000000207385
DO - 10.1212/WNL.0000000000207385
M3 - Article
C2 - 37130804
AN - SCOPUS:85169185885
SN - 0028-3878
VL - 101
SP - E982-E983
JO - Neurology
JF - Neurology
IS - 9
ER -