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.