Progressive encephalomyelitis with rigidity: A Taiwanese case and review of literature.

Clinical Neurology and Neurosurgery
Anna ChangWei-Hung Chen


Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disorder. However, the outcome is still variable with different serological and tumor associations, and the elements to good response with less relapse is yet to be elucidated. We present a case and obtain a literature review of patients with PERM and make comparisons based on different serological groups. We also analyze patients with idiopathic PERM that had detailed medical records. 81 patients were collected and analyzed. The largest group were glycine receptor-antibody (GlyR-Ab)-positive (70%), and the seropositive-GlyR-Ab-negative group had better response to immunotherapy. Malignancy can occur up to 2 years from the presentation of PERM. Among the 18 cases with detailed records, the patients who had good outcome initiate immunotherapy within 2 months from presentation. 9 of the 12 patients who experienced no relapse had non-steroid immunotherapy. The maximal interval time of relapse was 24 months. We recommend tumor surveillance up to 2 years in patients with PERM and early administration of immunotherapies and maintain with non-steroid immunotherapy with or without oral corticosteroid for a minimum of 2 years to reduce the risk of relapse in GlyR...Continue Reading


Sep 1, 1979·Journal of Neurology, Neurosurgery, and Psychiatry·D A HowellP J Toghill
Mar 1, 1976·Brain : a Journal of Neurology·A M WhiteleyH Urich
Dec 1, 1989·Journal of Neurology, Neurosurgery, and Psychiatry·P A McCombeM P Pender
Dec 7, 2007·Clinical Neurology and Neurosurgery·Markus Kraemer, Peter Berlit
Apr 9, 2008·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·S SaidhaM J Hennessy
Jan 30, 2010·Journal of the Neurological Sciences·Caroline SchmidtT Pfefferkorn
Dec 15, 2010·Journal of Neurology, Neurosurgery, and Psychiatry·Natalia MasFrancesc Graus
Nov 7, 2012·Movement Disorders : Official Journal of the Movement Disorder Society·Elke PeetersWim Vandenberghe
Jan 15, 2014·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·David BourkeNeil Anderson
Jun 2, 2014·Journal of the Neurological Sciences·Eva WuerfelKnut Brockmann
Jun 22, 2014·Brain : a Journal of Neurology·Alexander Carvajal-GonzálezAngela Vincent
Apr 12, 2016·JAMA Neurology·Eugenia Martinez-HernandezJosep Dalmau
Jun 13, 2017·Journal of Immunotherapy·Dmitry Tchapyjnikov, Alexandra J Borst
Feb 22, 2018·Neurology. Neuroimmunology and Neuroinflammation·Shannon R HinsonAndrew McKeon
Jun 16, 2018·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·A SwayneS Blum
Sep 16, 2018·Movement Disorders : Official Journal of the Movement Disorder Society·Valentina DamatoSarosh R Irani
Oct 27, 2018·Movement Disorders Clinical Practice·Bettina Balint, Hans-Michael Meinck
Feb 23, 2020·Journal of Neuroimmunology·Francisco BernardoMariana Santos
Jul 1, 2020·Journal of the Neurological Sciences·Lauren GluckPanos Stathopoulos

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