Taguchi Soutarou, Niwa Jun-ichi, Ibi Tohru, Doyu Manabu
Department of Neurology, Aichi Medical University.
Rinsho Shinkeigaku. 2015;55(3):182-4. doi: 10.5692/clinicalneurol.55.182.
Non-physiological, excessive dopaminergic stimulation can cause dyskinesia-hyperpyrexia syndrome (DHS), which was initially reported by Gil-Navarro and Grandas in 2010. A 70-years-old woman with a 13-years history of Parkinson's disease (PD) was hospitalized due to difficulty walking, despite being treated with levodopa/carbidopa (600 mg/day), immediate-release pramipexole (3 mg/day), and selegiline (5 mg/day). Immediate-release pramipexole was changed to extended-release pramipexole without changing the dose or levodopa equivalent dose (LED). The patient's adherence to drugs was good. The parkinsonism gradually improved and the patient was discharged. One month later, the patient developed severe generalized athetotic dyskinesia with visual hallucinations and hyperpyrexia that lasted for a week, and she was readmitted to hospital. On admission, the patient was conscious but slightly disoriented. Body temperature was 40.3°C with hyperhidrosis. Leukocyte count in the peripheral blood was 1.78×10(4)/ml and serum creatine kinase was >3×10(4) U/l. Chest survey, whole-body computed tomography, and cranial magnetic resonance imaging showed no abnormalities. The patient was diagnosed with DHS and treated by tapering the oral administration of dopaminergic drugs, including extended-release pramipexole. Her clinical condition recovered without dyskinesia, and serum creatine kinase level swiftly normalized. DHS and resemblant conditions are reported to occur in long-term PD patients with motor complications. In advanced stage PD, loss of dopaminergic neurons impairs the dopamine holding capacity of the striatum and exogenous dopaminergic drugs can result in uncontrollable and excessive fluctuations in dopamine concentration. Our case recommends caution when switching to long-acting dopaminergic drugs, even if the dose is unchanged, could lead to excessive dopaminergic stimulation. This case highlights the importance of considering both the LED and the duration of action of dopaminergic drugs when adjusting medication.
非生理性、过度的多巴胺能刺激可导致运动障碍 - 高热综合征（DHS），该综合征最初由吉尔 - 纳瓦罗和格兰达斯于2010年报道。一名患有13年帕金森病（PD）病史的70岁女性因行走困难入院，尽管她接受了左旋多巴/卡比多巴（600毫克/天）、速释普拉克索（3毫克/天）和司来吉兰（5毫克/天）治疗。速释普拉克索改为缓释普拉克索，剂量或左旋多巴等效剂量（LED）不变。患者对药物的依从性良好。帕金森症状逐渐改善，患者出院。一个月后，患者出现严重的全身性手足徐动症、幻视和高热，持续了一周，随后再次入院。入院时，患者意识清醒但略有定向障碍。体温为40.3°C，伴有多汗。外周血白细胞计数为1.78×10⁴/ml，血清肌酸激酶>3×10⁴U/l。胸部检查、全身计算机断层扫描和头颅磁共振成像均未显示异常。患者被诊断为DHS，并通过逐渐减少包括缓释普拉克索在内的口服多巴胺能药物进行治疗。她的临床状况恢复，无运动障碍，血清肌酸激酶水平迅速恢复正常。据报道，DHS及类似情况发生在患有运动并发症的长期PD患者中。在晚期PD中，多巴胺能神经元的丧失损害了纹状体的多巴胺容纳能力，外源性多巴胺能药物可导致多巴胺浓度不可控和过度波动。我们的病例提示，即使剂量不变，改用长效多巴胺能药物时也应谨慎，因为这可能导致过度的多巴胺能刺激。该病例强调了在调整药物时考虑多巴胺能药物的LED和作用持续时间的重要性。
