Leukoencephalopathy with intracranial calcifications and cysts in an adult: Case report and review of literature

• Main Authors: Ummer K, Salam K, Noone Mohan, Pradeep Kumar V, Mampilly Neena, Sivakumar S
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2010-01-01
• Series: Annals of Indian Academy of Neurology
• Subjects: Cysts; intracranial calcifications; leukoencephalopathy
• Online Access: http://www.annalsofian.org/article.asp?issn=0972-2327;year=2010;volume=13;issue=4;spage=299;epage=301;aulast=Ummer

Leukoencephalopathy, intracranial calcifications, and cysts (LCC) is a very rare cerebral disorder, first described in 3 children in 1996. It has subsequently been reported in adults and children from Europe and America, but has not so far been reported from Asia. We report an adult patient with pathologically proven LCC from a tertiary care hospital in South India. He presented with features of ataxia and raised intracranial pressure. Magnetic resonance imaging of the brain showed multiple bilateral cerebral cystic lesions along with diffuse white matter lesions in the cerebral and cerebellar white matter, and computed tomography of brain showed multiple calcifications in the white matter and basal ganglia. A large right cerebellar cyst causing mass effect was surgically excised. Histopathologic features were consistent with earlier reports of LCC and showed Rosenthal fibers, angiomatous changes, and calcifications. Our report suggests that although it is rare, LCC has a global distribution.