Opercular myoclonic-anarthric status (OMASE) secondary to anti-Hu paraneoplastic neurological syndrome

被引：0
|
作者：
Romero, Cesar [1 ]
Quijada, Alonso [1 ]
Abudinen, Gabriel [1 ]
Cespedes, Catherine [1 ]
Aguilera, Ledda [1 ]
机构：
[1] Univ Chile, Hosp Clin, Neurol & Neurosurg Dept, Santiago, Chile
来源：
EPILEPSY & BEHAVIOR REPORTS | 2024年 / 27卷
关键词：
Paraneoplastic neurological syndrome; Autoimmune encephalitis; Status epilepticus; OMASE; Anti-Hu IgG; GLUTAMIC-ACID DECARBOXYLASE; EPILEPSIA PARTIALIS CONTINUA; STATUS EPILEPTICUS; ANTIBODIES;
D O I：
10.1016/j.ebr.2024.100703
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
Focal Opercular Myoclonic - Anarthric Status Epilepticus (OMASE) is a rare form of focal motor status epilepticus caused by several etiologies. It is characterized by fluctuating dysarthria and epileptic myoclonus involving the bilateral glossopharyngeal musculature. We present the case of a 52-year-old woman who experienced gradual and progressive paralysis and myoclonus of facial and bulbar muscles; additional tests revealed the presence of right breast ductal adenocarcinoma and positive serum anti-Hu and anti-GAD65 antibodies. High doses of steroid pulses, anti-seizure therapy, and rituximab partially controlled myoclonus; the tumor resection improved dysphagia and dysarthria.
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