Leven B
Fortschr Neurol Psychiatr Grenzgeb. 1976 Nov;44(11):661-70.
43 patients and 27 relatives suffering from a hypertrophic type of peroneal muscular atrophy have been examined. Following the classification of peroneal muscular atrophy by Dyck and Lambert early age of onset and considerable reduction of nerve conduction velocity served as criteria for allotting them to the hypertrophic type. In all patients a typical deformity of the foot developed before the age of 10. Among the relatives there were some without this deformity and with only slight clinical signs. Assessing consistency and thickness of peripheral nerves by palpation proved difficult and unreliable. These findings also varied considerably between members of one family. The motor nerve conduction velocity was less than one half of the normal on average. Even those patients with only minor clinical signs had a nerve conduction velocity reduced by one fifth. In about 60% of the patients there was dominant inheritance, in 3 it was recessive and in about one third nothing was known. 10 of the patients without dominant inheritance had rather severe neurologic disturbance and a kyphoscoliosis. Whether this is hypertrophic neuritis of Dejerine und Sottas is discussed. Data form the literature differ. Another common type of peroneal muscular atrophy is the neuronal type. In contrast to the hypertrophic type it generally begins in adults and nerve conduction velocity is hardly affected.
对43例肥厚型腓骨肌萎缩症患者及27名亲属进行了检查。按照戴克（Dyck）和兰伯特（Lambert）对腓骨肌萎缩症的分类，发病年龄早和神经传导速度显著降低作为将他们归入肥厚型的标准。所有患者在10岁前均出现典型的足部畸形。在亲属中，有一些人没有这种畸形，只有轻微的临床体征。通过触诊评估周围神经的一致性和粗细被证明是困难且不可靠的。这些结果在一个家族的成员之间也有很大差异。运动神经传导速度平均不到正常速度的一半。即使是那些只有轻微临床体征的患者，其神经传导速度也降低了五分之一。约60%的患者为显性遗传，3例为隐性遗传，约三分之一情况不明。10例无显性遗传的患者有相当严重的神经功能障碍和脊柱侧凸。文中讨论了这是否为德热里纳（Dejerine）和索塔斯（Sottas）型肥厚性神经炎。文献数据存在差异。另一种常见的腓骨肌萎缩症类型是神经元型。与肥厚型不同，它通常在成年人中发病，神经传导速度几乎不受影响。
