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Neo, Ray Jen;
Mehta, Arpan R;
Weston, Mikail;
Magrinelli, Francesca;
Quattrone, Andrea;
Gandhi, Sonia;
Joyce, Eileen M;
(2023)
Neuropsychiatric Presentation of Anti-DPPX Progressive Encephalomyelitis with Rigidity and Myoclonus.
Movement Disorders Clinical Practice
10.1002/mdc3.13928.
(In press).
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Abstract
Progressive encephalomyelitis with rigidity and myoclonus (PERM) falls at the severe end of stiff-person-syndrome spectrum disorders, being characterized by brainstem and autonomic involvement in addition to axial and limb rigidity, and central nervous system (CNS) hyperexcitability.1 Autoantibodies against glycine receptor (GlyR) are associated with 70% of PERM cases.2 Other PERM-associated autoantibodies encompass antibodies against glutamic acid decarboxylase (GAD), dipeptidyl peptidase-like-protein-6 (DPPX), and amphiphysin.3 Here, we report an atypical case of anti-DPPX-associated PERM with prominent neuropsychiatric prodrome.
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