PROGNOSIS OF MOTOR NEURON DISEASE IN NIGERIAN AFRICANS - PROSPECTIVE STUDY OF 92 PATIENTS
PROGNOSIS OF MOTOR NEURON DISEASE IN NIGERIAN AFRICANS - PROSPECTIVE STUDY OF 92 PATIENTS
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DOI：
10.1093/brain/97.1.385
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发表时间：
1974-01-01
期刊：
影响因子：
14.5
通讯作者：
BADEMOSI, O
中科院分区：
文献类型：
--
作者：
OSUNTOKUN, BO;ADEUJA, AOG;BADEMOSI, O
方法根据临床和实验室检查结果诊断MND。每位患者的诊断标准包括：（a）神经肌肉疾病（下运动神经元、上运动神经元或两者的组合）的潜伏性发作和缓慢演变;（B）局限于运动系统的疾病，而无周围神经病变的临床证据;（c）存在去神经支配的临床证据，包括四肢和舌头肌肉的肌束震颤;（d）以自发性纤维颤动、肌束震颤、尖齿电位的形式证明去神经支配的肌电图证据，运动单位或亚单位的偶发性假强直放电，运动单位电位的同步，最大意志收缩的干扰模式减少，多相运动单位过多，其振幅大于2000微伏且持续时间长（15毫秒或以上）;（e）运动神经传导速度正常的证明。此外，在某些患者中，标准还包括：（/）肌肉活检组织学检查发现神经性或神经源性萎缩（35例）当（ae）似乎没有绝对令人信服;（g）脑脊液检查结果正常（50例患者）和脊髓造影（25例）当判断有必要排除其他可能的病理时，特别是有创伤括约肌障碍病史的患者，当X线平片显示脊柱改变时，同时对所有患者进行胸部X线平片和常规血液学检查，甲状腺功能检查，甲状腺功能测定，甲状腺激素水平测定等测定了30例ALS患者、8例PMA患者和5例CSMA患者的血浆结合碘、I125 T3树脂或红细胞摄取量;对54例患者进行空腹血糖浓度测定，对25例患者进行口服葡萄糖耐量试验（50 g）;对2例持续空腹血糖低的患者进行静脉甲苯磺丁脲试验（低于45毫克/100毫升静脉血）;测定钙、磷酸盐、碱性磷酸酶、胆固醇、转氨酶的血清浓度;标准肝功能检查和约75%患者的红细胞沉降率。本文对9例CSMA患者的血清醛缩酶（Sibley和Lehninger，1949）和肌酸磷酸激酶（Creatineprophokinase，Tanzer和Gilvarg，1959）进行了测定。在50名患者（45名患有ALS，5名患有PMA）中，按照Osuntokun和Aladetoyinbo（1970）的描述测定血浆硫氰酸盐和氰化物浓度，以评估慢性氰化物中毒。在3例临床证据怀疑有腹腔内恶性肿瘤的患者中，检查包括造影剂放射学检查（钡餐和灌肠，静脉肾盂造影）和剖腹手术。伊巴丹大学洛克菲勒病毒研究实验室对13例ALS患者的血液和CSF样本以及9例ALS患者的经皮活检（患者自愿知情同意）获得的肝脏样本进行了筛选，以寻找既往病毒感染的证据。
METHODS The diagnosis of MND was made on clinical grounds and on the results of laboratory investigations. The criteria for diagnosis in each patient include:(a) neuromuscular disease (lower motor neuron, upper motor neuron, or combination of both) of insidious onset and slow evolution;(b) disease confined to the motor system without clinical evidence of peripheral neuropathy;(c) presence of clinical evidence of denervation, including fasciculations in muscles of the limbs and tongue;(d) demonstration of electromyographic evidence of denervation in forms of spontaneous fibrillations, fasciculations, sharp-toothed potentials, occasional pseudomyotonic discharges of motor units or subunits, synchronization of motor unit potentials, reduced interference pattern on maximal volitional contraction, excess of polyphasic motor units, the amplitude of which were greater than 2000 microvolts and of long duration (15 milliseconds or more);(e) demonstration of normal motor nerve conduction velocity. In addition, in some patients the criteria also included:(/) the finding of neuropathic or neurogenic atrophy on histological examination of muscle biopsy (in 35 patients) when (ae) did not appear absolutely convincing;(g) normal results of examination of CSF (in 50 patients) and myelography (in 25 patients) when it was judged necessary to exclude other possible pathology, especially in patients who gave history of trauma sphincteric disturbance, when plain radiogiaphs showed spondylotic changes, and those in whom symptoms and signs were asymmetrical and confined mainly to the upper limbs.The following investigations were also carried out: plain radiographs of the chest and routine screening for hsematological disorders in all patients; thyroid function tests (plasma bound iodine, I125T3 resin or red blood cell uptake) in 30 patients with ALS, 8 with PMA and 5 with CSMA; determination of fasting blood sugar concentrations in 54 patients and oral glucose tolerance tests (50 g) in 25 patients; intravenous tolbutamide test in 2 patients who had persistently low fasting blood sugar (of below 45 mg/100 ml of venous blood); determination of serum concentrations of calcium, phosphate, alkaline phosphatase, cholesterol, transaminases; standard liver function tests, and erythrocyte sedimentation rate in about 75 per cent of the patients. The concentration of serum aldolase (by the method of Sibley and Lehninger, 1949) and serum creatine phosphokinase (by the method of Tanzer and Gilvarg, 1959) were determined in 9 patients with CSMA. In 50 patients (45 suffered from ALS and 5 suffered from PMA), concentration of plasma thiocyanate and cyanide were determined as described by Osuntokun and Aladetoyinbo (1970) to assess chronic cyanide intoxication. In 3 patients suspected on clinical evidence to have intra-abdominal malignancy, investigations included contrast radiological investigations (barium meal and enema, intravenous pyelography), and laparotomy. In 13 patients with ALS, samples of blood and CSF, and in 9 patients with ALS, samples of liver obtained by percutaneous biopsy (to which the patients gave their free and informed consent) were screened for evidence of previous viral infections by the Rockefeller Virus Research Laboratory, University of Ibadan.