Alhashel Jassim Y, Alshubaili Asmahan F, Montasser Ashraf, Anim T
Department of Neurology, Ibn Sina Hospital, Kuwait University, Kuwait.
Med Princ Pract. 2004 Nov-Dec;13(6):369-71. doi: 10.1159/000080476.
To report the unusual occurrence of Guillain-Barre syndrome (GBS) in a case of hereditary spastic paraparesis (HSP) and describe its effect on the preexisting clinical picture as well as the importance of early recognition and treatment.
A 41-year-old man known to have HSP developed an acute and rapid deterioration of muscle power associated with paresthesia in both upper and lower limbs. Clinical examination revealed flaccid quadriparesis with areflexia. Nerve conduction studies were suggestive of demyelinating polyneuropathy and CSF analysis showed increased protein but no cells. The diagnosis of GBS was made in addition to HSP. The patient received intravenous immunoglobulins 20 mg/kg body weight/day for 5 days. He responded very well to the treatment clinically and neurophysiologically and thereafter, he reverted to the preexisting spastic picture of HSP.
The peripheral demyelination with GBS was severe enough to obscure the preexisting clinical picture, but fortunately it responded very well to treatment. The clinical associations with HSP are widely variable, therefore it is very important to recognize acquired treatable causes of weakness in such patients in order to prevent an increase in disability.
报告1例遗传性痉挛性截瘫（HSP）患者出现吉兰 - 巴雷综合征（GBS）这一罕见情况，并描述其对原有临床表现的影响以及早期识别和治疗的重要性。
一名已知患有HSP的41岁男性出现肌肉力量急性快速恶化，并伴有双上肢和双下肢感觉异常。临床检查发现弛缓性四肢瘫且无反射。神经传导研究提示脱髓鞘性多发性神经病，脑脊液分析显示蛋白升高但无细胞。除HSP外，还诊断出GBS。患者接受静脉注射免疫球蛋白，剂量为20mg/kg体重/天，共5天。他在临床和神经生理学方面对治疗反应良好，此后又恢复到HSP原有的痉挛状态。
GBS导致的周围神经脱髓鞘严重到足以掩盖原有的临床表现，但幸运的是它对治疗反应良好。与HSP的临床关联广泛多样，因此识别此类患者中获得性可治疗的肌无力原因对于预防残疾增加非常重要。
