Late-onset leukoencephalopathy with cerebral calcifications and cysts: case report and review of the literature. Issue 1 (December 2016)

• Record Type: Journal Article
• Authors: Stephani, C.; Pfeifenbring, S.; Mohr, A.; Stadelmann, C.
• Is Part Of: BMC neurology. Volume 16:Issue 1(2016)
• Page Start: 1
• Page End: 12
• Publication Date: 2016-12
• Subjects: Leukoencephalopathy -- Calcifications -- Cysts -- Glucocorticoids; Neurology -- Periodicals; 616.8005
• DOI: 10.1186/s12883-016-0543-1
• Languages: English
• ISSNs: 1471-2377

Abstract

Abstract Background Leukoencephalopathy with calcifications and cysts (LCC or Labrune disease) is a relatively recently defined and exceptionally rare disease in which parenchymal cysts and calcifications within a widespread leukoencephalopathy can cause a broad spectrum of neurological symptoms. The cause of the disease is unknown. Manifestation is usually in childhood or adolescence, while onset in adulthood has been described in 19 cases. Case presentation Here we report a case of an adult-onset LCC of a Caucasian woman who became symptomatic at age 70 as confirmed by typical neuroimaging and neuropathological findings. After resection of left mesioparietal space-occupying cystic brain tissue the patient has so far remained clinically stable during one year of follow-up with a continuous treatment with glucocorticosteroids. Conclusion To our knowledge this report of a patient who became symptomatic at age 70 represents the oldest age-at-onset case of LCC described so far.