Hatakeyama K, Aihara M, Shimizu A, Uchida N, Inukai T
Department of Pediatrics, Yamanashi Medical College.
No To Hattatsu. 1991 Jan;23(1):76-80.
Higher cerebral dysfunctions such as aphasia, apraxia and agnosia have seldom been reported in multiple sclerosis (MS). 12 year-old right-handed boy felt unsteadiness of the body and headache for several days. Two months later, he had the same episode and complained of visual disturbance, and weakness and sensory disturbance on the face and the extremities. Additionally, he showed amnestic aphasia, acalculia, ideomotor apraxia, finger agnosia and right-left disorientation. Cerebrospinal fluid examinations revealed increases IgG, myelin basic protein and neuron specific enolase (11%, 25 ng/ml and 28.8 ng/ml, respectively). X-ray CT scan and MRI-CT examinations revealed sclerotic lesions on the left parietal white matter and the right mid-brain. The diagnosis was made as MS. He was treated with m-PSL (methyl-prednisolone) pulse therapy for three weeks and consecutively treated with PSL for four weeks. He recovered gradually, but visual disturbance and facial palsy remained. After seven months MRI-CT showed a high signal intensity on the left parietal white matter in spite of the disappearance of the lesion on X-ray CT scan. We suggest that these higher cerebral dysfunctions may result from the lesion of the left parietal white matter which produces a disconnection between each cortical area.
诸如失语症、失用症和失认症等高级脑功能障碍在多发性硬化症（MS）中鲜有报道。一名12岁的右利手男孩数天来感到身体不稳和头痛。两个月后，他出现了同样的症状，并伴有视觉障碍、面部及四肢无力和感觉障碍。此外，他还表现出遗忘性失语、失算症、观念运动性失用症、手指失认症和左右定向障碍。脑脊液检查显示免疫球蛋白G、髓鞘碱性蛋白和神经元特异性烯醇化酶升高（分别为11%、25 ng/ml和28.8 ng/ml）。X线CT扫描和磁共振成像CT检查显示左侧顶叶白质和右侧中脑有硬化性病变。诊断为MS。他接受了甲基强的松龙脉冲治疗三周，随后连续接受强的松治疗四周。他逐渐康复，但仍有视觉障碍和面瘫。七个月后，尽管X线CT扫描显示病变消失，但磁共振成像CT显示左侧顶叶白质有高信号强度。我们认为这些高级脑功能障碍可能是由左侧顶叶白质病变导致各皮质区域之间联系中断所致。
