Craven Claudia L, Mullins Sophie, Pradini-Santos Laura, Watkins Laurence D
Neurosurgery, National Hospital for Neurology and Neurosurgery, London, GBR.
Cureus. 2023 Jul 24;15(7):e42372. doi: 10.7759/cureus.42372. eCollection 2023 Jul.
Treatment for a large symptomatic syrinx associated with a Chiari 1 is predominately surgical, via a foramen magnum decompression (FMD), with the aim to normalise cerebrospinal fluid (CSF) movement. Whilst theories of underlying hyperdynamic states in Chiari 1 and Syringomyelia exist, to date there is no effective medical treatment to reverse Syringomyelia. A 17-year-old female was referred with a seven-month history of gradually progressive impaired temperature sensation in her left upper limb. She had also been concomitantly diagnosed with thyrotoxicosis. Magnetic resonance imaging (MRI) confirmed a Chiari 1 with a large syrinx. The patient preferred to avoid surgery in the first instance. She underwent treatment for her thyrotoxicosis. The eight-month, 20- and 36-month follow-up MRI scans demonstrated a gradual resolution of the Chiari 1 malformation and the syrinx. Whilst there have been reports of Chiari 1 malformation association with hyperthyroidism, this is the first report describing syrinx resolution following treatment of thyrotoxicosis. Hyperdynamic circulation can result in syrinx formation through various mechanisms. We hypothesise that the treatment of thyrotoxicosis resulted in normalisation of CSF pulse amplitude and subsequent syrinx resolution. Hyperthyroidism evaluation may be explored in studies of CM1 and Syrinx or other CSF disorders.
与Chiari 1畸形相关的大型有症状脊髓空洞症的治疗主要是手术治疗,即通过枕骨大孔减压术(FMD),目的是使脑脊液(CSF)流动正常化。虽然存在关于Chiari 1畸形和脊髓空洞症潜在高动力状态的理论,但迄今为止,尚无有效的药物治疗方法来逆转脊髓空洞症。一名17岁女性因左上肢温度感觉逐渐进行性受损7个月前来就诊。她还同时被诊断为甲状腺毒症。磁共振成像(MRI)证实为Chiari 1畸形合并大型脊髓空洞症。患者最初倾向于避免手术。她接受了甲状腺毒症的治疗。8个月、20个月和36个月的随访MRI扫描显示Chiari 1畸形和脊髓空洞症逐渐消退。虽然有关于Chiari 1畸形与甲状腺功能亢进症相关的报道,但这是第一份描述甲状腺毒症治疗后脊髓空洞症消退的报告。高动力循环可通过多种机制导致脊髓空洞症形成。我们推测,甲状腺毒症的治疗使脑脊液脉冲幅度正常化,随后脊髓空洞症消退。在Chiari 1畸形合并脊髓空洞症或其他脑脊液疾病的研究中,可能需要探索甲状腺功能亢进症的评估。