Neo, Ray Jen;
Mehta, Arpan R;
Weston, Mikail;
Magrinelli, Francesca;
Quattrone, Andrea;
Gandhi, Sonia;
Joyce, Eileen M;
(2024)
Neuropsychiatric Presentation of Anti-DPPX Progressive Encephalomyelitis with Rigidity and Myoclonus.
Movement Disorders Clinical Practice
, 11
(1)
pp. 97-100.
10.1002/mdc3.13928.
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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.
| Type: | Article |
|---|---|
| Title: | Neuropsychiatric Presentation of Anti-DPPX Progressive Encephalomyelitis with Rigidity and Myoclonus |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1002/mdc3.13928 |
| Publisher version: | https://doi.org/10.1002/mdc3.13928 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions. |
| Keywords: | ataxia, autoimmunity, dipeptidyl-peptidase-like protein-6, hyperekplexia, stiff person spectrum disorder |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10183394 |
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