DPPX (dipeptidyl-peptidase-like protein 6) is a type of autoimmune encephalitis.

Target: subunit of the Kv4.2 potassium channel (affects somatodendritic signal integration and attenuation of back-propagation of action potentials)


Clinical presentation:

(1) diarrhea

(2) other gastrointestinal symptoms

(3) unexplained weight loss


Neurologic features:

(1) cognitive and mental deficits

(2) CNS excitability (tremor, myoclonus, seizures, hyperekplexia)

(3) occasionally PERM (muscle rigidity, brainstem symptoms, myoclonus)


Laboratory findings:

(1) presence of IgG1 and IgG4 antibodies to DPPX


The syndrome is usually responsive to immunotherapy.

To read more or access our algorithms and calculators, please log in or register.