Glycine receptor antibodies are detected in progressive encephalomyelitis with rigidity and myoclonus (PERM) but not in saccadic oscillations.
Glycine receptor (GlyR) antibodies were recently identified in a few patients with progressive encephalomyelitis with rigidity and myoclonus (PERM); none of these patients had antibodies against glutamic acid decarboxylase (GAD). An inhibitory glycinergic transmission defect has also been implicated in the mechanism underlying saccadic oscillations, including ocular flutter or opsoclonus; GlyR antibodies have not been reported in these patients. The purpose was to determine whether GlyR antibodies are found in patients with PERM, ocular flutter syndrome (OFS), and opsoclonus-myoclonus syndrome (OMS). GlyR antibodies were first measured in archived sera and CSF from five patients, including one patient with GAD antibody-positive PERM, two patients with OFS, and two patients with OMS. GlyR antibodies were also measured in archived sera from nine other adult patients with OMS. GlyR antibodies and GAD antibodies were both found at high titers in the serum and CSF of the patient with PERM, and their levels paralleled disease activity over time. GlyR antibodies were not found at significant levels in 13 patients with saccadic oscillations. GlyR and GAD antibodies can co-exist in PERM and follow the clinical course. Although saccadic oscillations are a feature of this condition, GlyR antibodies are not commonly found in patients with isolated saccadic oscillations.
Department of Neurology, Kitasato University, School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374, Japan. takahiro@med.kitasato-u.ac.jp
Takahiro
T
Iizuka
Maria I
MI
Leite
Bethan
B
Lang
Patrick
P
Waters
Yoshiaki
Y
Urano
Saori
S
Miyakawa
Junichi
J
Hamada
Fumihiko
F
Sakai
Hideki
H
Mochizuki
Angela
A
Vincent
D000328:Adult; D001323:Autoantibodies / Q000096:biosynthesis*; D018450:Disease Progression; D004679:Encephalomyelitis / Q000175:diagnosis / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009127:Muscle Rigidity / Q000175:diagnosis / Q000276:immunology*; D009207:Myoclonus / Q000175:diagnosis / Q000276:immunology*; D015835:Ocular Motility Disorders / Q000175:diagnosis / Q000276:immunology*; D018009:Receptors, Glycine / Q000276:immunology*; D012438:Saccades / Q000276:immunology*
D001323:Autoantibodies; D018009:Receptors, Glycine