To present an autopsy-proven case of Leukoencephalopathy with Calcifications and Cysts (LCC) presenting in adulthood with multifocal intracranial hemorrhages. 

First described in 1996 by Lebrune, LCC is a rare disorder with less than 50 cases reported in the literature. Most cases present in children or young adults as progressive neurological decline with dementia, seizures, focal neurological deficits due to cyst formation with edema with multifocal calcifications in the basal ganglia and white matter.  Imaging evidence of microhemorrhages and symptomatic intracerebral hemorrhage has been described but not as the prominent feature of the disorder.  

Case review with clinical, neuroimaging, biopsy and ultimately autopsy findings. 

A 68-year-old man presented acutely with confusion and hemiparesis.  Initial CT and MRI revealed diffuse white matter changes and multifocal intracerebral hemorrhages of varying age, some with peripheral contrast enhancement and peri- hemorrhage edema. The differential diagnosis of tumor, abscess vs amyloid or other vasculitis prompted surgical resection of the largest, symptomatic lesion.  Pathology revealed chronic reactive gliosis and microcalcifications.  Over the ensuing five years, he had recurrent episodes of acute focal worsening due to lobal intracerebral hemorrhage with partial recovery.  He died 7 years after his initial presentation due to infectious complications from being bedbound with spastic quadriplegia, dysphagia and dementia.  

LCC can present with progressive dementia, seizures, and focal neurological deficits and affects children through older adults.  Neuroimaging features precede clinical symptoms and include prominent white matter changes, multiple irregularly shaped parenchymal cysts, and multifocal calcifications.  The unusual neuroimaging features can suggest the diagnosis although the pathological features of brain calcifications, leukodystrophy, and parenchymal cysts are often interpreted as nondiagnostic. LCC should now also be considered as a cause of multifocal recurrent lobar intracerebral hemorrhage.