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RT Journal Article
SR Electronic
T1 Widening the Neuroimaging Features of Adenosine Deaminase 2 Deficiency
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 975
OP 979
DO 10.3174/ajnr.A7019
VO 42
IS 5
A1 Geraldo, A.F.
A1 Caorsi, R.
A1 Tortora, D.
A1 Gandolfo, C.
A1 Ammendola, R.
A1 Alessio, M.
A1 Conti, G.
A1 Insalaco, A.
A1 Pastore, S.
A1 Martino, S.
A1 Ceccherini, I.
A1 Signa, S.
A1 Gattorno, M.
A1 Rossi, A.
A1 Severino, M.
YR 2021
UL http://www.ajnr.org/content/42/5/975.abstract
AB SUMMARY: Adenosine deaminase 2 deficiency (OMIM #615688) is an autosomal recessive disorder characterized by a wide clinical spectrum, including small- and medium-sized vessel vasculopathies, but data focusing on the associated neuroimaging features are still scarce in the literature. Here, we describe the clinical neuroimaging features of 12 patients with genetically proven adenosine deaminase 2 deficiency (6 males; median age at disease onset, 1.3 years; median age at genetic diagnosis, 15.5 years). Our findings expand the neuroimaging phenotype of this condition demonstrating, in addition to multiple, recurrent brain lacunar ischemic and/or hemorrhagic strokes, spinal infarcts, and intracranial aneurysms, also cerebral microbleeds and a peculiar, likely inflammatory, perivascular tissue in the basal and peripontine cisterns. Together with early clinical onset, positive family history, inflammatory flares and systemic abnormalities, these findings should raise the suspicion of adenosine deaminase 2 deficiency, thus prompting genetic evaluation and institution of tumor necrosis factor inhibitors, with a potential great impact on neurologic outcome.DADA2adenosine deaminase 2 deficiencyTNFtumor necrosis factorPANpolyarteritis nodosaPRESposterior reversible encephalopathy syndrome