X-Linked Lymphoproliferative Syndrome Presenting as Adult-Onset Multi-Infarct Dementia
نویسندگان
چکیده
منابع مشابه
Ekbom syndrome occurring with multi infarct dementia.
Ekbom Syndrome is characterized by delusion that small living being infests skin. The clinical profile of this disorder has shown it to be associated with organic conditions. Neuroimaging studies implicate putamen as the brain structure involved in the pathophysiology. These are also known as organic delusional disorder and provide an opportunity to study biological causation of delusional diso...
متن کاملX-Linked Lymphoproliferative Disease
2B4 is a surface molecule involved in activation of the natural killer (NK) cell-mediated cytotoxicity. It binds a protein termed Src homology 2 domain-containing protein (SH2D1A) or signaling lymphocyte activation molecule (SLAM)-associated protein (SAP), which in turn has been proposed to function as a regulator of the 2B4-associated signal transduction pathway. In this study, we analyzed pat...
متن کاملX-Linked Lymphoproliferative Disease Presenting as Pancytopenia in a 10-Month-Old Boy
X-linked lymphoproliferative disease, also known as Duncan's syndrome, is a rare genetic disorder that causes exaggerated immune responses to Epstein-Barr virus (EBV) infection and often leads to death. Patient presentation varies but can include signs and symptoms typical of EBV, pancytopenia, and fulminant hepatitis.
متن کاملX-linked lymphoproliferative syndrome. Natural history of the immunodeficiency.
The X-linked lymphoproliferative syndrome is characterized by immunodeficiency to Epstein-Barr virus (EBV) manifested by severe or fatal infectious mononucleosis and acquired immunodeficiency. We studied immune responses in six males of a well-characterized kindred with the X-linked lymphoproliferative syndrome. Two males were studied before and during acute fatal EBV infection. Both individual...
متن کاملAdult-onset MELAS presenting as herpes encephalitis.
OBJECTIVE To report an unusual presentation of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) manifested in late life with a clinical picture of herpes simplex encephalitis. DESIGN Case report. SETTING Clinical neurology department in a tertiary care hospital. CASE DESCRIPTION A 55-year-old woman developed aphasia and delirium during ophthalmic herpes z...
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ژورنال
عنوان ژورنال: Journal of Neuropathology & Experimental Neurology
سال: 2019
ISSN: 0022-3069,1554-6578
DOI: 10.1093/jnen/nlz018