PMID- 30910547 OWN - NLM STAT- MEDLINE DCOM- 20190701 LR - 20190701 IS - 1532-2653 (Electronic) IS - 0967-5868 (Linking) VI - 64 DP - 2019 Jun TI - Biopsy-proven primary angiitis of the central nervous system mimicking leukodystrophy: A case report and review of the literature. PG - 42-44 LID - S0967-5868(19)30070-0 [pii] LID - 10.1016/j.jocn.2019.03.021 [doi] AB - Primary Angiitis of the Central Nervous System (PACNS) is a rare form of idiopathic CNS vasculitis. Neuroimaging is often abnormal and characterized by multifocal brain lesions, but brain biopsy definitely confirms the diagnosis. We report the rare case of a 45-year-old female presenting with symptoms of intracranial hypertension and leukodystrophy-like neuroimaging findings. A comprehensive diagnostic work-up led to the unexpected diagnosis of a definite PACNS which was successfully treated by immunosuppressive treatment. Although rarely, PACNS can present as diffuse leukoencephalopathy on neuroimaging, and mimic even an inherited leukodystrophy. Therefore, in adults with leukodystrophy-like neuroimaging findings, careful examination of clinical and non-clinical features is mandatory to avoid missing the diagnosis of a treatable acquired disease. CI - Copyright (c) 2019 Elsevier Ltd. All rights reserved. FAU - Caputi, Luigi AU - Caputi L AD - Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy. Electronic address: luigi.caputi@istituto-besta.it. FAU - Erbetta, Alessandra AU - Erbetta A AD - Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy. FAU - Marucci, Gianluca AU - Marucci G AD - Department of Neuropathology, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy. FAU - Pareyson, Davide AU - Pareyson D AD - Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy. FAU - Eoli, Marica AU - Eoli M AD - Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy. FAU - Servida, Maura AU - Servida M AD - Department of Neurology, ASST Ovest Milanese - Legnano Hospital, Via Papa Giovanni Paolo II, 20025 Legnano, Italy. FAU - Parati, Eugenio AU - Parati E AD - Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy. FAU - Salsano, Ettore AU - Salsano E AD - Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20190322 PL - Scotland TA - J Clin Neurosci JT - Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia JID - 9433352 RN - 0 (Immunosuppressive Agents) RN - Primary angiitis of the central nervous system SB - IM MH - Biopsy MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Leukoencephalopathies/diagnosis/pathology MH - Middle Aged MH - Vasculitis, Central Nervous System/*diagnosis/drug therapy OTO - NOTNLM OT - Angiitis OT - Biopsy OT - CNS OT - Leukodystrophy OT - Leukoencephalopathy OT - MRI EDAT- 2019/03/27 06:00 MHDA- 2019/07/02 06:00 CRDT- 2019/03/27 06:00 PHST- 2019/01/12 00:00 [received] PHST- 2019/03/11 00:00 [accepted] PHST- 2019/03/27 06:00 [pubmed] PHST- 2019/07/02 06:00 [medline] PHST- 2019/03/27 06:00 [entrez] AID - S0967-5868(19)30070-0 [pii] AID - 10.1016/j.jocn.2019.03.021 [doi] PST - ppublish SO - J Clin Neurosci. 2019 Jun;64:42-44. doi: 10.1016/j.jocn.2019.03.021. Epub 2019 Mar 22.