Ann Short Rep | Volume 2, Issue 2 | Case Report | Open Access
Beatriz Echeveste1*, Miguel Idoate2, Eric McDade3 and Mario Riverol1
1Department of Neurology, Clinica Universidad de Navarra, Spain
2Department of Anatomic Pathology, Clinica Universidad de Navarra, Spain
3Department of Neurology, Washington University School of Medicine, USA
*Correspondance to: Beatriz Echeveste
Fulltext PDFWe describe a patient with a 2 month course of dementia, Parkinsonism and myoclonus highly suggestive of a prion disease. We review the differential diagnosis and subsequent comprehensive assessment, including brain biopsy, to identify treatable causes in the setting of possible prion-related encephalopathy. Brain MRI showed a nodular parenchymal and linear leptomeningeal enhancement but no vessel abnormalities and cerebrospinal fluid sample had 324 leukocytes/μl (98% lymphocytes) and 150.35 mg/dl proteins leading to final differential diagnosis of CNS lymphoma, infection, chronic granulomatous disease or a primary central nervous system vasculitis. Brain biopsy confirmed a primary angiitis of the central nervous system. The combination of rapidly progressive dementia with Parkinsonism and myoclonus is highly suggestive of a prion encephalopathy. However, determining potentially treatable causes is imperative, particularly atypical presentations of uncommon disorders such as primary angiitis of the central nervous system. Common presentations of primary angiitis of the central nervous system are headache or seizures, with a rapidly progressive dementia being less frequent. This case illustrates the need for a comprehensive assessment of rapidly progressive dementia, especially when a prion disorder appears to be the cause.
Prion disease; Encephalopathy; Vasculitis
Echeveste B, Idoate M, McDade E, Riverol M. Primary Angiitis of the Central Nervous System Mimicking a Prion-Like Disease. Ann Short Reports. 2019;2:1036.