Diffuse CNS vasculopathy with chronic Epstein-Barr virus infection in X-linked lymphoproliferative disease.
We report a patient with X-linked lymphoproliferative disease (XLP) who developed multiple central nervous system (CNS) manifestations of Epstein-Barr virus infection. XLP, or Duncan syndrome, is a rare inherited disorder characterized by the inability to clear Epstein-Barr virus infection. In addition to Epstein-Barr virus encephalitis, CNS lymphoproliferative disease, and lymphoma, this patient also developed MR angiographic evidence of diffuse fusiform aneurysmal dilation of intracranial vessels.
Weeks
J K
JK
Department of Radiological Sciences, St Jude Children's Research Hospital, Memphis, TN 38105-3300, USA.
Helton
K J
KJ
Conley
M E
ME
Onciu
M
M
Khan
R B
RB
D002561:Cerebrovascular Disorders / Q000175:diagnosis / Q000209:etiology*; D002648:Child; D002908:Chronic Disease; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications*; D018810:Magnetic Resonance Angiography; D008297:Male