好色先生

好色先生

Explore the latest content from across our publications
Join The AAN

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

A Case of Progressive Multifocal Leucoencephalopathy Associated with Thymoma
Infections/AIDS/Prion Disease
P03 - (-)
256
BACKGROUND: The association of thymoma and immunosuppression is known as Good's syndrome. Two cases of Good's syndrome associated with PML have been reported in the literature. Four other reports have associated thymoma and PML without making the connection to Good's syndrome. We wish to share our experience and bring forward this rare condition to the attention of clinical neurologists.
DESIGN/METHODS: We report a case of PML associated with an AB type thymoma. Good's syndrome was formally identified with immunological testing.
RESULTS: A 51-years-old male was admitted to our neurology unit to investigate a rapidly progressive cognitive impairment. Six months prior to admission, the patient underwent the resection of a fortuitously detected type AB thymoma. Since the last four months, his wife noticed progressive behavioral changes. He developed apathy and memory problems. Magnetic resonance imaging (MRI) of the brain showed FLAIR and T2-weighted hyperintensities in the subcortical white matter of both frontal lobes and left parietal area, involving U-fibers. Gadolinium enhancement was not detected. Cerebrospinal fluid analysis confirmed the presence of JC virus, thus corroborating the diagnosis of PML. Investigation for a source of immunodeficiency yielded a negative HIV test, normal liver and kidney function and a normal PET scan. The patient didn't take any immunosuppressant drug. Immunological testing demonstrated an hypogammaglobulinemia with low IgG and IgM levels along with a dramatic decline of B cells. A diagnosis of Good's syndrome, a rare combination of B and T cell immunodeficiency in patients with thymoma, was made.
CONCLUSIONS: Treatment with mirtazapine, mefloquine and replacement immunoglobulins was started. This patient was eventually discharged home in a stable but unimproved neurological condition.
Authors/Disclosures
Alexandre Hamelin, MD (CHUS FLEURIMONT)
PRESENTER 		No disclosure on file
Eric Frenette, MD (CHUS-Service de neurologie) No disclosure on file
Frederik Barkhof, MD, PhD (Image Analysis Center) Frederik Barkhof has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Frederik Barkhof has received personal compensation in the range of $0-$499 for serving as a Consultant for Biogen. Frederik Barkhof has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Combinostics. Frederik Barkhof has received personal compensation in the range of $500-$4,999 for serving as a Consultant for IXICO. Frederik Barkhof has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Frederik Barkhof has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EISAI.