Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.
Clin EEG Neurosci. 2014 Apr;45(2):126-8. doi: 10.1177/1550059413490930. Epub 2013 Sep 4.
Diagnosis of aphasic status epilepticus is sometimes not easy because of its rarity and electroclinical dissociation. Although most cases are associated with organic brain lesions, nonketotic hyperglycemia (NKH)-related aphasic status epilepticus is rare, especially if it is isolated (without other clinical seizure activity). On the other hand, unlike other metabolic disorders, or hypoglycemia-related generalized seizures, focal motor seizure and epilepsia partialis continua can occur in 25% of NKH, with seizures being the initial manifestation in up to 50% of patients. However, the presentation of epileptic aphasia is rare in NKH patients. We report a rare case of NKH presenting initially as persistent and isolated aphasic status epilepticus. Brain magnetic resonance imaging did not reveal any focal lesion, but ictal electroencephalography (EEG) disclosed left frontotemporal continuous theta to delta waves, intermingled with epileptiform discharges. Correcting the hyperglycemia failed to improve the language disorder, and the seizure was controlled only by the addition of carbamazepine. Patients with NKH may initially present with isolated aphasic status epilepticus. Unlike stroke-related aphasia, accurate diagnosis is difficult if based solely on neurologic examination and brain neuroimaging. Use of EEG and blood sugar determination should be helpful in this special condition.
癫痫性失语状态的诊断有时并不容易，因为其罕见且存在电临床分离。虽然大多数病例与器质性脑损伤有关，但非酮症高血糖（NKH）相关的癫痫性失语状态较为罕见，尤其是孤立（无其他临床癫痫发作活动）的情况。另一方面，与其他代谢紊乱或低血糖相关的全身性发作不同，25%的 NKH 中可能出现局灶性运动性发作和部分性持续癫痫，高达 50%的患者以癫痫发作为初始表现。然而，在 NKH 患者中，癫痫性失语的表现较为罕见。我们报告了一例罕见的 NKH 病例，最初表现为持续且孤立的癫痫性失语状态。脑磁共振成像未显示任何局灶性病变，但发作期脑电图（EEG）显示左额颞部连续的θ至δ波，伴有癫痫样放电。纠正高血糖未能改善语言障碍，仅通过添加卡马西平才能控制癫痫发作。NKH 患者最初可能表现为孤立性癫痫性失语状态。与中风相关的失语症不同，如果仅基于神经检查和脑部神经影像学检查，准确诊断较为困难。在这种特殊情况下，脑电图和血糖测定的使用应该有所帮助。
