{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:55:29Z","timestamp":1790729729232,"version":"4.1.0"},"reference-count":33,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"13","license":[{"start":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T00:00:00Z","timestamp":1790553600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurosurg Case Lessons"],"published-print":{"date-parts":[[2026,9,28]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Labrune syndrome, also known as leukoencephalopathy with cerebral calcifications and cysts (LCC), is a rare autosomal recessive cerebral microangiopathy. Because of its rarity and microangiopathic pathology of recurrent cysts, no curative treatment is available, which makes long-term management challenging for clinicians.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>\n                      A 34-year-old woman born to consanguineous parents presented in 2022 with chronic headache and blurred vision. Imaging revealed diffuse leukoencephalopathy, intracranial calcifications, and multiple parenchymal cysts, with a dominant left hemispheric lesion causing mass effect. She underwent left-sided cyst decompression with good symptomatic relief, followed-up by imaging surveillance. Three years later, she developed recurrent headache and worsening vision. Imaging demonstrated enlargement of a right-sided cyst with significant midline shift. Right-sided decompression surgery again improved her symptoms. Histopathological specimens from both surgeries showed no neoplasia; additionally, the second specimen demonstrated gliosis and hyalinized microvasculature. Subsequent genetic testing identified a novel homozygous\n                      <jats:italic>SNORD118<\/jats:italic>\n                      variant, supporting the LCC diagnosis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>\n                      This case highlights that LCC diagnosis is supported by radiological findings, pathological features, and\n                      <jats:italic>SNORD118<\/jats:italic>\n                      genetic findings. Additionally, long-term follow-up is essential due to metachronous enlargement of cysts, and repeat decompressions could provide symptomatic relief.\n                    <\/jats:p>\n                    <jats:p>\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/thejns.org\/doi\/10.3171\/CASE26650\">https:\/\/thejns.org\/doi\/10.3171\/CASE26650<\/jats:ext-link>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case26650","type":"journal-article","created":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T00:04:06Z","timestamp":1790121846000},"source":"Crossref","is-referenced-by-count":0,"title":["The first reported case of Labrune syndrome in Vietnam with 4-year longitudinal follow-up: illustrative case"],"prefix":"10.3171","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-0686-6476","authenticated-orcid":true,"given":"Duy","family":"Pham","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, University of Medicine and Pharmacy Hospital\u2013Linh Dam, Hoang Liet, Hanoi"},{"name":"Faculty of Medicine, University of Medicine and Pharmacy, Vietnam National University, Cau Giay, Hanoi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hung Manh","family":"Ngo","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Medicine and Pharmacy Hospital\u2013Linh Dam, Hoang Liet, Hanoi"},{"name":"Faculty of Medicine, University of Medicine and Pharmacy, Vietnam National University, Cau Giay, Hanoi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quang Dung","family":"Doan","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery 2, Viet Duc University Hospital, Hoan Kiem, Hanoi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Duc Thuong","family":"Ho","sequence":"additional","affiliation":[{"name":"Department of Anatomic Pathology, Cytological Pathology and Forensic Medicine, Viet Duc University Hospital, Hoan Kiem, Hanoi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-8802-749X","authenticated-orcid":true,"given":"Son Tung","family":"Tran","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Medicine and Pharmacy Hospital\u2013Linh Dam, Hoang Liet, Hanoi"},{"name":"Faculty of Medicine, University of Medicine and Pharmacy, Vietnam National University, Cau Giay, Hanoi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-6733-1832","authenticated-orcid":true,"given":"Mai Phuong","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Medical Biology and Genetics, Hanoi Medical University, Kim Lien, Hanoi, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-9393-1996","authenticated-orcid":true,"given":"Trong Hiep","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Medicine and Pharmacy Hospital\u2013Linh Dam, Hoang Liet, Hanoi"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"10","key":"bib1","series-title":"Nat Genet","first-page":"1185","article-title":"Mutations in SNORD118 cause the cerebral microangiopathy leukoencephalopathy with calcifications and cysts","volume":"48","author":"Jenkinson","year":"2016","unstructured":"Jenkinson EMRodero MPKasher PRMutations in SNORD118 cause the cerebral microangiopathy leukoencephalopathy with calcifications and cysts. 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