{Reference Type}: Case Reports
{Title}: Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM)-like Symptoms Associated with Anti-ganglionic Acetylcholine Receptor Antibodies.
{Author}: Kitazaki Y;Ikawa M;Kishitani T;Kamisawa T;Nakane S;Nakamoto Y;Hamano T;
{Journal}: Intern Med
{Volume}: 60
{Issue}: 14
{Year}: Jul 2021 15
{Factor}: 1.282
{DOI}: 10.2169/internalmedicine.6419-20
{Abstract}: This report describes a 59-year-old woman who presented with progressive encephalomyelitis with rigidity and myoclonus (PERM)-like symptoms and severe dysautonomia, including orthostatic hypotension, sinus bradycardia, dysuria, and prolonged constipation. Her neurological symptoms improved after immunotherapy, but the dysautonomia persisted. Anti-ganglionic acetylcholine receptor (gAChR) α3 subunit antibodies, which are frequently identified in patients with autoimmune autonomic ganglionopathy, were detected in the pre-treatment serum. The central distribution of the nicotinic acetylcholine receptors, a target of anti-gAChR antibodies, and immunotherapeutic efficacy observed in this case indicate that anti-gAChR α3 subunit antibodies are associated with the PERM-like features accompanied by autonomic manifestations.