Abstract
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by a mutation in the Bruton agammaglobulinemia tyrosine kinase gene that results in severe B-cell deficiency. So far, neurological complications of XLA have been primarily related to acute and/or chronic central nervous system enteroviral infections. In the last few years a progressive neurodegenerative syndrome of unknown etiology has been described in XLA patients. We describe and present a video of an XLA patient who developed a fatal dementing, dystonia-Parkinsonism syndrome 14 years into his immune disorder. Physician awareness of this rare syndrome may lead to its better characterization and management.
Original language | English (US) |
---|---|
Pages (from-to) | 1664-1666 |
Number of pages | 3 |
Journal | Movement Disorders |
Volume | 22 |
Issue number | 11 |
DOIs | |
State | Published - Aug 15 2007 |
Keywords
- Dystonia
- Extrapyramidal syndrome
- IVIG
- X-linked agammaglobulinemia
ASJC Scopus subject areas
- Clinical Neurology
- Neuroscience(all)