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Hydrocephalus: a rare complication of primary central nervous system vasculitis
  1. Joana Lopes1,
  2. Hugo Doria2,
  3. Ricardo Taipa3 and
  4. Raquel Fonseca Samões1
  1. 1Neurology, Centro Hospitalar Universitário do Porto EPE, Porto, Portugal
  2. 2Neuroradiology, Centro Hospitalar Universitário do Porto EPE, Porto, Portugal
  3. 3Neuropathology Unit, Centro Hospitalar Universitário do Porto EPE, Porto, Portugal
  1. Correspondence to Dr Joana Lopes; joanalopes-jl{at}


Hydrocephalus is a known complication of central nervous system (CNS) vasculitis secondary to infectious diseases. We present an unusual case of primary CNS vasculitis (PCNSV) complicated by communicating hydrocephalus. A patient in their 50s with a few months’ history of headache, psychomotor slowing and frequent falls presented with an acute left temporo-parieto-occipital infarction. Angiography revealed multiple arterial irregularities in the anterior circulation bilaterally, CSF was inflammatory and the remaining study was negative, fulfilling criteria for possible PCNSV. One month after successful treatment with corticosteroid, there was worsening of gait, urinary incontinence and neuropsychiatric symptoms. The investigation was remarkable only for active hydrocephalus. An external ventricular shunt was placed, followed by a ventriculoperitoneal shunt, and cyclophosphamide was started with subsequent recovery. Our discussion is that communicating hydrocephalus in PCNSV, due to impaired CSF flow, should be considered on subacute/chronic worsening of patients with PCNSV.

  • Hydrocephalus
  • Neuroimaging
  • Stroke
  • Immunology

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  • Contributors JL was responsible for planning, conducting and reporting of the work described in the article. RFS identified and managed the patient and was responsible for the reporting of the case. HD and RT managed the case and contributed to the reporting article.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests Yes.

  • Provenance and peer review Not commissioned; externally peer reviewed.