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

Abstract

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.

Late-onset leukoencephalopathy with cerebral calcifications and cysts: case report and review of the literature

“…Extensive Microcalcifications + concentric fine calcification around blood vessel walls. Headache for 4 months, progressive unsteadiness of gait for 1 month, recurrent vomiting for 1 week Exam: papilledema, finger-nose and heel-knee dyscoordination, dysdiadochokinesia; spastic-atactic gait Dur-ing child-hood as-soci-ated with fever Posterior fossa- crani-ectomy with resection of a cyst Ummer et al 2010 [ 22 ] 55 F Normal Abnormalities Widespread leukoencephalopathy more prominent anteriorly with multifocal cysts up to 1 cm in diameter. Numerous punctate-enhancing lesions distributed throughout the cerebrum.…”

Leukoencephalopathy with calcifications and cysts

“…[ 8 ] Preoperative diagnosis of LCC based only on clinical and radiological profiles is quite challenging, and definitive diagnosis depends on pathological biopsy. [ 6 , 9 ]…”

“…About 22% of reported cases showed thickening and degeneration of vessel walls and lymphocyte aggregation, further supporting the pathological change of small vessels. [ 4 , 5 , 9 , 15 , 17 , 18 ] We speculated that the main cause of tissue degeneration, calcification, and necrosis in LCC might be tissue ischemia and hypoxia as a result of repeated vasculopathy.…”

Hallazgos por neuroimagen de la microangiopatía cerebral retiniana con calcificaciones y quistes

“…En la mayoría de los casos los síntomas aparecen en la infancia temprana o durante la adolescencia y progresan rápidamente, sobre todo a partir de una hemorragia cerebral, y debido al efecto de masa producido por las lesiones quísticas (4). La presentación clínica de esta entidad es insidiosa y variable e incluye disminución en el rendimiento cognitivo, demencia, trastorno convulsivo, y una combinación de signos extrapiramidales, cerebelosos y piramidales (1,4). Inicialmente los síntomas se producen por aumento de la presión intracraneal y, en forma tardía, por déficits neurológicos focales (6).…”