Sadamoto K, Kinoshita M, Honda M
4th Department of Internal Medicine, Toho University School of Medicine.
Rinsho Shinkeigaku. 1995 Sep;35(9):1034-6.
Among 17 patients with amyloid polyneuropathy type IV in a Japanese family, we found a 72-year-old woman, who showed extraocular symptoms approximately 10 years after the onset of the disease. she developed weakness of the right facial muscles and dysarthria at age 57. She had atrophy and disturbance of movement of the tongue, along with difficulty in swallowing at age 62. At the age of 66, she felt diplopia when she looked toward the left, followed by difficulty of ocular movement. These manifestations progressed and at age 72, she was found to have mild ptopsis, mild to moderate disturbance of almost all extraocular muscles, moderate to severe disturbance of facial, masseter, pharyngeal, tongue and neck muscles. She also had slight weakness and atrophy of the limb and truncal muscles together with slight loss of pain and vibratory sensations in the distal parts of the limbs. FAP IV has sometimes been called cranial amyloidosis, but motor disturbance is limited to the middle and lower cranial nerve territories in the majority of the reported cases, and manifestations of the extraocular muscles are quite rare. According to the present investigation of the world literature, this is the second case of FAP IV with extraocular muscle involvement.
在一个日本家族的17例IV型淀粉样多神经病患者中，我们发现一名72岁女性，在疾病发作约10年后出现了眼外症状。她57岁时出现右侧面部肌肉无力和构音障碍。62岁时出现舌萎缩和运动障碍，同时伴有吞咽困难。66岁时，她向左看时出现复视，随后出现眼球运动困难。这些表现逐渐进展，到72岁时，发现她有轻度上睑下垂，几乎所有眼外肌有轻度至中度障碍，面部、咬肌、咽、舌和颈部肌肉有中度至重度障碍。她还伴有肢体和躯干肌肉轻度无力和萎缩，以及肢体远端轻微的痛觉和振动觉丧失。FAP IV有时被称为颅淀粉样变性，但在大多数报道的病例中，运动障碍仅限于中、下颅神经区域，眼外肌受累的表现非常罕见。根据目前对世界文献的调查，这是第二例伴有眼外肌受累的FAP IV病例。
