Honey Christopher R, Morrison Murray D, Heran Manraj K S, Dhaliwal Baljinder S
Divisions of1Neurosurgery and.
2Otolaryngology, Department of Surgery; and.
J Neurosurg. 2018 Jul 20;130(6):1865-1869. doi: 10.3171/2018.2.JNS172952. Print 2019 Jun 1.
Inducible laryngeal obstruction has been described under at least 40 different monikers, including vocal cord dysfunction, paroxysmal vocal fold motion, and irritable larynx. The etiology of this condition is believed to be laryngeal hyperactivity in response to psychological issues or acid reflux. Most patients are treated with some combination of proton pump inhibitors, speech therapy, and psychotherapy. However, a small cohort of patients remains refractory to all medical interventions. The authors describe a novel condition, hemi-laryngopharyngeal spasm (HELPS), which can cause severe episodic stridor leading to unconsciousness in association with cough. The first recognized and surgically cured patient with HELPS was reported in an earlier issue of this journal. Three additional patients have been followed up for at least a year postoperatively, and their cases are reported here.Each patient presented with a similar pattern of episodic coughing and choking that increased in frequency, severity, and duration over years. The episodes eventually occurred while sleeping and could cause severe stridor with loss of consciousness. All three patients were initially misdiagnosed with a psychiatric illness and subjected to multiple intubations and one tracheostomy. Unilateral botulinum toxin injections in the vocal fold eased the severity of the throat contractions but not the cough. Magnetic resonance imaging showed a looping posterior inferior cerebellar artery juxtaposed to a vagus nerve in each case. Microvascular decompression (MVD) of that vessel relieved all symptoms.The introduction of this new medical condition may help a small cohort of patients with inducible laryngeal obstructions that have not responded to the current standard treatments. Patients are asymptomatic between episodes of progressively severe coughing and choking with stridor that may lead to intubation. Severe anxiety about the unpredictable symptoms is expected and may contribute to a psychiatric misdiagnosis. Microvascular decompression for HELPS is more difficult than that for trigeminal neuralgia because the involved nerve is more susceptible to manipulation. Ultimately, the final proof that HELPS is a real and distinct syndrome will require its recognition and successful treatment by colleagues around the world.
诱发性喉梗阻至少有40种不同的名称，包括声带功能障碍、阵发性声带运动障碍和易激惹喉。这种疾病的病因被认为是喉部对心理问题或胃酸反流的过度反应。大多数患者采用质子泵抑制剂、言语治疗和心理治疗的某种组合进行治疗。然而，一小部分患者对所有医疗干预均无反应。作者描述了一种新的疾病，半喉咽痉挛（HELPS），它可导致严重的发作性喘鸣，并伴有咳嗽导致意识丧失。该杂志早期曾报道过首例被识别并通过手术治愈的HELPS患者。另外3例患者术后至少随访了1年，现将他们的病例报告于此。每位患者都表现出类似的发作性咳嗽和呛咳模式，多年来发作频率、严重程度和持续时间不断增加。这些发作最终在睡眠时发生，并可导致严重喘鸣和意识丧失。所有3例患者最初均被误诊为精神疾病，并接受了多次插管和1次气管切开术。声带单侧注射肉毒杆菌毒素减轻了喉部收缩的严重程度，但咳嗽症状未缓解。磁共振成像显示，每例患者的小脑后下动脉呈袢状并与迷走神经毗邻。对该血管进行微血管减压术可缓解所有症状。这种新疾病的发现可能有助于一小部分对当前标准治疗无反应的诱发性喉梗阻患者。患者在逐渐加重的咳嗽和伴有喘鸣的呛咳发作期间无症状，而喘鸣可能导致插管。对不可预测症状的严重焦虑是预期的，这可能导致精神疾病误诊。HELPS的微血管减压术比三叉神经痛的微血管减压术更困难，因为受累神经更容易受到操作影响。最终，要证明HELPS是一种真实且独特的综合征，需要全球各地的同行对其进行识别并成功治疗。
