Abstract
Anti-DPPX-Encephalitis in A Patient with Cerebral Vasculitis and Connective Tissue Disease
Highlights
In 2013 Boronat et al described an autoimmune encephalitis with gastrointestinal symptoms, cognitive dysfunction and signs of CNS hyperexcitability like myoclonus, hyperekplexia, tremor or epileptic seizures characterized by antibodies against "dipeptidyl peptidase -like protein 6" (DPPX) [1]
According to the etiology CNS vasculitis can be classified as a primary angiitis of the CNS (PACNS) or a secondary form associated with systemic disorders like infections, malignant or connective tissue diseases as well as systemic vasculitides [5]
This patient presented with the triad characteristic for anti-DPPX-encephalitis comprising gastrointestinal symptoms, cognitive dysfunction and signs of CNS hyperexcitability [2]
Summary
In 2013 Boronat et al described an autoimmune encephalitis with gastrointestinal symptoms, cognitive dysfunction and signs of CNS hyperexcitability like myoclonus, hyperekplexia, tremor or epileptic seizures characterized by antibodies against "dipeptidyl peptidase -like protein 6" (DPPX) [1]. DPPX is a regulatory protein associated with the Kv4.2-potassium-channel on the surface of central and peripheral neurons. DPPX-antibodies reversibly decrease DPPX and Kv4.2 protein on hippocampal neurons thereby causing hyperexcitability of enteric and central nervous system neurons [2, 3]. Like anti-DPPX-encephalitis, CNS vasculitis is a rare disease presenting with variable neurological symptoms. Diagnosis of CNS vasculitis is challenging as mimics like reversible cerebral vasoconstriction syndrome (RCVS) have to be considered and as there is no single test permitting definitive diagnosis [6]
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