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

  • Michael BayatEmail author
  • Nitesh Shekhrajka
  • Allan Bayat
Letter to the Editor


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.


A previously healthy 44-year-old woman presented to our department due to cognitive symptoms evolving in the course of 6 months. There was no family history of neurological disease. The symptoms progressed rather quickly and at the time of admission she was no longer able to manage her job as a cleaning lady, to drive or to manage household chores such as cooking and cleaning. She also suffered from emotional lability. The neurological examination was normal....


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical standards

The case report been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

The patient has consented to the submission of this case report to the journal.


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Copyright information

© Belgian Neurological Society 2019

Authors and Affiliations

  1. 1.Aalborg UniversitetshospitalAalborgDenmark
  2. 2.Department of PediatricsUniversity Hospital of CopenhagenCopenhagenDenmark

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