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01-12-2019 | Vasculitis | Letter to the Editor

Hereditary leukodystrophy with axonal spheroids (HDLS) presenting subacutely: a CNS-vasculitis mimic

Authors: Michael Bayat, Nitesh Shekhrajka, Allan Bayat

Published in: Acta Neurologica Belgica | Issue 4/2019

Excerpt

Hereditary leukodystrophy with axonal spheroids (HDLS) or CSF1R-related leukoencephalopathy is an adult-onset leukodystrophy which typically causes progressive cognitive, psychiatric and motor symptoms. The rate of progression differs from patient to patient but typically the disease progresses slowly. We present a case with a subacute presentation which—combined with MRI lesions with diffusion restriction—led to initial diagnostic considerations of acquired autoimmune diseases such as CNS-vasculitis. …

Sundal C, Lash J, Aasly J, Øygarden S, Roeber S, Kretzschman H, Garbern JY, Tselis A, Rademakers R, Dickson DW, Broderick D, Wszolek ZK (2012) Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS): a misdiagnosed disease entity. J Neurol Sci 314(1–2):130–137. https://doi.org/10.1016/j.jns.2011.10.006 CrossRefPubMed

Bender B, Klose U, Lindig T, Biskup S, Nägele T, Schöls L, Karle KN (2014) Imaging features in conventional MRI, spectroscopy and diffusion weighted images of hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS). J Neurol 261(12):2351–2359. https://doi.org/10.1007/s00415-014-7509-2 CrossRefPubMed

Lynch DS, Jaunmuktane Z, Sheerin U, Phadke R, Brandner S, Milonas I, Dean A, Bajaj N, McNicholas N, Costello D, Cronin S, McGuigan C, Rossor M, Fox N, Murphy E, Chataway J, Houlden H (2016) Hereditary leukoencephalopathy with axonal spheroids: a spectrum of phenotypes from CNS vasculitis to parkinsonism in an adult onset leukodystrophy series. J Neurol Neurosurg Psychiatry 87:512–519. https://doi.org/10.1136/jnnp-2015-310788 CrossRefPubMed

Lakshmanan R, Adams ME, Lynch DS, Kinsella JA, Phadke R, Schott JM, Murphy E, Rohrer JD, Chataway J, Houlden H, Fox NC, Davagnanam I (2017) Redefining the phenotype of ALSP and AARS2 mutation-related leukodystrophy. Neurol Genet 3(2):e135. https://doi.org/10.1212/NXG.0000000000000135 CrossRefPubMedPubMedCentral

Title: Hereditary leukodystrophy with axonal spheroids (HDLS) presenting subacutely: a CNS-vasculitis mimic
Authors: Michael Bayat
Nitesh Shekhrajka
Allan Bayat
Publication date: 01-12-2019
Publisher: Springer International Publishing
Keywords: Vasculitis
Vasculitis
Leukodystrophy
Syphilis
Published in: Acta Neurologica Belgica / Issue 4/2019
Print ISSN: 0300-9009
Electronic ISSN: 2240-2993
DOI: https://doi.org/10.1007/s13760-019-01096-5

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Hereditary leukodystrophy with axonal spheroids (HDLS) presenting subacutely: a CNS-vasculitis mimic

Excerpt

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Excerpt

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