Chronic myoclonia of subcortical origin with antiglutamate receptor antibodies.
We report a 10-year-old girl with chronic nonprogressive continuous myoclonia with mild muscle weakness and dissociated sensory impairment of the ipsilateral side of myoclonic jerks. Irregular myoclonic jerks continuously appeared in the right upper limb. The jerk-locked back averaging of electroencephalographic activity failed to show any activity preceded by the muscle contraction. Magnetic resonance imaging of the brain and cervical spine revealed no abnormal findings. Single photon emission computed tomography showed an increased blood perfusion in the left thalamus. (18)F-deoxyglucose-positron emission tomography (PET) also showed a slight high density in the posterior region of the left thalamus. Antiglutamate receptor epsilon2 and delta2 antibodies were detected in the serum and cerebrospinal fluid. The patient's symptoms have now been stable with clonazepam treatment for 2 years. The left thalamus was suspected to have been the region at least partly responsible for the patient's symptoms.
Matsuo
Muneaki
M
Department of Pediatrics, Faculty of Medicine, Saga University, Saga, Japan. matsuo@cc.saga-u.ac.jp
Takahashi
Yukitoshi
Y
Taniguchi
Kazuto
K
Sasaki
Kazuya
K
Hamasaki
Yuhei
Y
D000918:Antibody Specificity / Q000276:immunology*; D000927:Anticonvulsants / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D002648:Child; D002908:Chronic Disease; D002998:Clonazepam / Q000627:therapeutic use; D004569:Electroencephalography; D004576:Electromyography; D005260:Female; D019788:Fluorodeoxyglucose F18; D005500:Follow-Up Studies; D006801:Humans; D009207:Myoclonus / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D017470:Receptors, Glutamate / Q000097:blood / Q000276:immunology*; D013788:Thalamus / Q000098:blood supply / Q000000981:diagnostic imaging* / Q000503:physiopathology; D015899:Tomography, Emission-Computed, Single-Photon
D000927:Anticonvulsants; D001323:Autoantibodies; D019275:Radiopharmaceuticals; D017470:Receptors, Glutamate; D019788:Fluorodeoxyglucose F18; D002998:Clonazepam