Department of Biomedical, Metabolic and Neural Science, University of Modena and Reggio Emilia, Modena, Italy.
Neuroscience Head Neck Department, Azienda Ospedaliero-Universitaria Di Modena, Modena, Italy.
Neurol Sci. 2024 Jul;45(7):3509-3511. doi: 10.1007/s10072-024-07465-0. Epub 2024 Mar 28.
Hemiballism (HB) and hemichorea (HC) are the most frequent secondary movement disorders, usually caused by cerebrovascular diseases. In only a minority of cases, these involuntary movements are not self-limited, and they may severely compromise patients' quality of life, so that symptomatic treatments are required. Typical and atypical neuroleptics as well as tetrabenazine are considered therapies of choice. However, anecdotal reports of antiseizures medications and botulinum neurotoxin injection effectiveness have been described.
We described a case of severely disabling acute-onset lesional HB/HC, where high dosage of first- and second-line therapies was contraindicated due to patient's comorbidities.
After botulin neurotoxin (BoNT) injections in his left upper limb muscles (biceps brachii, triceps brachii, teres major, and deltoid), the patient experienced gradual reduction of hyperkinetic movements. The gradual discontinuation of topiramate (TPM) did not worsen the clinical picture.
The reduction of hyperkinetic movements led to rhabdomyolysis resolution as well as cutaneous injuries healing with renal function improvement, so that the patient was able to be eligible for rehabilitation, which was prevented by HB/HC itself. The clinical improvement was consistent with BoNT pharmacokinetic. The administration of BoNT early after the onset of lesional HB/HC remarkably modified the clinical management and drove toward comorbidities resolution and rehabilitation.
The present case highlights the effectiveness of unconventional therapeutic options in disabling acute onset lesional HB/HC when first-line therapies are contraindicated. Particularly, this report may encourage BoNT application in the early stage of movement disorder emergencies.
Hemiballism(HB)和 hemichorea(HC)是最常见的继发性运动障碍,通常由脑血管疾病引起。在极少数情况下,这些不自主运动不会自行限制,并且可能严重影响患者的生活质量,因此需要进行对症治疗。典型和非典型的抗精神病药以及四苯嗪被认为是首选治疗方法。然而,已经描述了抗癫痫药物和肉毒杆菌神经毒素注射有效性的偶发报道。
我们描述了一例严重致残的急性起病病灶性 HB/HC 病例,由于患者的合并症,一线和二线治疗的高剂量治疗是禁忌的。
在其左上肢体肌肉(肱二头肌、肱三头肌、大圆肌和三角肌)中进行肉毒神经毒素(BoNT)注射后,患者的多动运动逐渐减少。逐渐停用托吡酯(TPM)并没有使临床症状恶化。
多动运动的减少导致横纹肌溶解症的缓解以及肾功能改善后皮肤损伤的愈合,从而使患者有资格接受康复治疗,而 HB/HC 本身则阻止了这一治疗。临床改善与 BoNT 药代动力学一致。在病灶性 HB/HC 发病后早期给予 BoNT 治疗,显著改变了临床管理,并促使解决合并症和康复。
当一线治疗禁忌时,本病例强调了非常规治疗选择在治疗致残性急性起病病灶性 HB/HC 中的有效性。特别是,本报告可能鼓励在运动障碍急症的早期应用 BoNT。