Leukoencephalopathy, cerebral calcifications, and cysts: A clinical case involving a long-term follow-up and literature review

J Neurol Sci. 2017 Feb 15:373:60-65. doi: 10.1016/j.jns.2016.12.014. Epub 2016 Dec 11.

Abstract

Background: Leukoencephalopathy, cerebral calcifications, and cysts (LCC) is a rare disease that was first reported by Labrune in 1996. A case of adult-onset LCC was successfully followed up for a long period.

Case presentation: A 30-year-old female presented with visual field disturbance and seizure on several occasions. Radiographic images revealed multiple supratentorial cysts and calcifications in the bilateral nucleus basalis and cerebella. Aspiration, Ommaya reservoir placement, and nodule removal were performed for the responsible cysts, and the patient had a good postoperative course.

Discussion: A tiny, strongly enhanced nodule was identified before cyst formation on her radiographic images. Thus, cyst growth may be related to nodule microbleeding. According to our review, if the responsible cyst is located on the noneloquent area, surgical removal of the cyst should be considered. However, if the responsible cyst is located on the eloquent area, the nodule should be first removed because nodules can bleed and enlarge cysts.

Conclusion: Careful follow-up is needed, especially for cysts with a strongly enhanced nodule.

Keywords: Calcification; Cyst; Leukoencephalopathy; Nodule.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Brain / diagnostic imaging
  • Brain / pathology
  • Brain / surgery
  • Calcinosis / diagnosis*
  • Calcinosis / diagnostic imaging
  • Calcinosis / pathology
  • Calcinosis / therapy*
  • Central Nervous System Cysts / diagnosis*
  • Central Nervous System Cysts / diagnostic imaging
  • Central Nervous System Cysts / pathology
  • Central Nervous System Cysts / therapy*
  • Diagnosis, Differential
  • Female
  • Follow-Up Studies
  • Humans
  • Leukoencephalopathies / diagnosis*
  • Leukoencephalopathies / diagnostic imaging
  • Leukoencephalopathies / pathology
  • Leukoencephalopathies / therapy*

Substances

  • Leukoencephalopathy Brain Calcifications and Cysts