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A case of vagus related neurogenic cough due to unilateral vascular encroachment of its root A case of vagus related neurogenic cough due to unilateral vascular encroachment of its root
A case of vagus related neurogenic cough due to unilateral vascular encroachment of its root A case of vagus related neurogenic cough due to unilateral vascular encroachment of its root

A 60-year older man was presented to the hospital with a complaint of medically refractory, non-productive cough from past 15 years. Any viral rash, trauma, respiratory tract infection or exposure to harsh fumes did not precede the cough. The patient had no history of allergy, asthma or acid reflux. Initially, the cough was mild, but with time it became more frequent, severe and occurred for a longer duration. Sometimes, the cough was severe enough to faint him and wake him up from sleep. The cough used to happen spontaneously, but many activities like exercise, singing, laughing or exercise triggered the cough. The patient reported his voice to be normal in between the episodes of coughing. The patient did not have throat pain; choking or spasm of vocal cords. He reported that coughing had significantly affected his work and he was unable to work in 2016. In 2018, an improvement in symptoms of cough occurred for a few months, and it relapsed again. The patient had a history of angina. Three cardiac stents with clopidogrel were inserted. In 1962 and 1965, the patient has a tonsillectomy and myringotomy. In 1977, he quit smoking. His reports of all allergy testing and methacholine challenge had been negative. He was not able to tolerate pH monitor due to a strong gag reflex but was not even benefitted with pantoprazole 40 mg and chronic antacids. After being coughing for more than 10 years, treatment with ACE inhibitor, ramipril for hypertension was initiated. After 3 months, it was discontinued as it provided no benefit to the patient. His chest CT was found to be normal. Investigations performed after multiple consultations with the physicians (allergist, cardiologist, pulmonologists, otolaryngologist) were also negative.

What could be the most likely diagnosis of this case presentation?

  • Pulmonary tuberculosis
  • Upper airway cough syndrome
  • Vagus related neurogenic cough
  • Chronic hypersensitivity pneumonitis


Neurogenic cough, as defined by Altman et al. in his latest review, is a chronic cough for more than eight weeks without external stimuli to the cough mechanism. This could be defined as an excluded diagnosis which is made after complete investigation to reduce the chances of other causes. As per the American College of Chest Physicians (ACCP), the possible reasons to be included are asthma, nonasthmatic eosinophilic bronchitis, gastroesophageal reflux disease and sinonasal disease.

The latest theories on the etilogy of a neurogenic cough have shown some similarities with trigeminal neuralgia, due to which vascular compression of a vagus nerve rootlet could be the possible cause of neurogenic cough. Trigeminal neuralgia is most commonly caused by the compression of the trigeminal vessel. Anti-neuralgic medicines pharmacologically manage it while microvascular decompression is the possible neurosurgical care. The patients who are not able to take medication due to poor tolerance or side effects are advised for surgical care. Neurogenic cough can be managed with medications like amitryptiline, tramadol, gabapentin, tramadol and non-conventional approaches like speech therapy. A group of patients with neurogenic cough may develop VANCOUVER syndrome (Vagus Associated Neurogenic Cough due to Unilateral Vascular Encroachment of its Root). If the patient is not responding to the pharmacological treatment, microvascular decompression could be tried for subsequent benefit.

A rare case of unilateral vascular compression of a vagus nerve rootlet at the brainstem resulting in the neurogenic cough has been presented. No previous research has reported this etiology of the neurogenic cough. The case study described the features of the patient's cough and his response to numerous treatments. Imaging and surgical outcomes have also been presented. The treatment model for the rare disease condition has been introduced.

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Poster abstract

A 60 year old man diagnosed with unilateral vascular compression of a vagus nerve rootlet at the brainstem resulting in the neurogenic cough was recovered after neurosurgical microvascular decompression of that nerve. 

Medical history

The patient had a history of angina for which three cardiac stents with clopidogrel were inserted.

Examination & lab investigations

On physical examination, no abnormalities in cranial nerves and lymphadenopathy were observed. On nasal examination, no signs of sinonasal disease were present. Laryngoscopy was conducted and revealed normal findings, i.e. normal mucosal wave, no masses, normal vocal cord closure with a small gap posteriorly, normal mobility bilaterally, no post-cricoid or arytenoid edema or erythema and no supraglottic hyperfunction. The score of cough severity index (CSI) 1 was 20 out of 40, and the score of the Leicester quality of life Cough Questionnaire (LCQ) 2 was 93 out of 133. The reflux symptom index and voice handicap index were found to be normal at 11/45 and 0/40.4, respectively.


A trial with carbamazepine 200 mg once a day was stated and post one week, the dose was increased to 200 mg twice a day. The frequency and intensity of the cough was greatly reduced (80%) in the second week. But, due to the significant side-effects of carbamazepine (emotional lability, suicidal ideation, etc.), it has to be stopped, and relapse of the cough occurred. Psychiatric consultation showed no signs of depression post-termination of carbamazepine. Also, no signs of past psychopathy, malingering or conversion disorder were observed. Based on the outcomes of the first trial, gabapentin and amitryptiline trial was rejected. The latest experience of researchers related to cough linked with Hemi-laryngopharyngeal spasm due to vascular compression of the vagus nerve rootlets provoked them to perform brain MRI in the patient. A unilateral vascular encroachment of a vagus nerve rootlet at the left brainstem was revealed as per CISS sequence. The cough halted for almost 8 hours via breathing humidified air with 5cc of nebulized 1% lidocaine. However, there was no benefit with first left and post-1 week right unilateral superior laryngeal nerve block. A severe attack of a rootlet of the left vagus nerve by a loop of the posterior inferior cerebellar artery (PICA) was confirmed by the neurological examination of the left lower brainstem.

The procedure involving microvascular decompression was carried out. PICA was moved away from the vagus and was anteromedially secured with Teflon and Tiseel. In the postoperative recovery room, the patient had coughing, but his cough was completely gone after a few hours. He was able to project his voice and even sign without coughing. For the past one year, the patient remained free from his previous pathological cough. His quality of life (depicted by higher LCQ scores) and severity of cough (described by lower CSI scores) were significantly improved. He did not experience ant postoperative dysphonia, limb dysmetria, dysphonia or dysarthria. Normal vocal cord function was observed in the laryngoscopy, conducted after six weeks of operation.


The case study presented was a rare disease condition in which the vascular compression of a vagus nerve rootlet triggered a chronic, non-productive neurogenic cough. The cough responds well to antineuralgia treatment while it is unmanageable with anti-reflux medicines, speech therapy, antibiotics and psychotherapy. The cough progresses continually but with some relief and lapses like trigeminal neuralgia. MRI showing a vascular compression of the vagus and unilateral benefit from a vagus nerve block will confirm the diagnosis of this rare condition. The disease condition can be cured, but neurosurgery near the brainstem is related to significant risks.

The present case may provide preliminary guidance on the symptoms and pathophysiology of this rare disease condition. However, the further case series will confirm the findings. The disease being unmanageable with the numerous medical therapies and the absence of any psychiatric history decreases the chances of observed outcomes due to the placebo effect. A specific test for detecting patients with chronic cough from etiology other than the incidental contact of a vessel against vagus nerve will be needed. The sequential unilateral proximal vagus nerve block could be the valid diagnostic test. The suspension of cough post unilateral vagus nerve block ipsilateral but not contralateral to an MRI affirmed vascular compression to be the diagnostic test of choice for VANCOUVER syndrome. In the present case, unilateral bronchial ventilation with lidocaine and unilateral superior laryngeal nerve block were tried for symptom elimination. The diagnosis could have been confirmed by the unilateral positive response, but the negative one failed to rule it out.

The patient before the surgery had a clearing understanding about the fact that the decompression of the vessel impinging his left vagus nerve may not have ceased his coughing. Post-vascular compression of the trigeminal nerve, focal demyelination at the compression site may occur supporting ephaptic transmission from nerve fibers conveying touch (Aβ) to those fibers carrying sharp or burning pain. The tracheobronchial sensory and laryngeal afferents via vagus nerve move to the brainstem. Similarly, vagus compression could provide ephaptic transmission from fibers carrying information from non-cough receptors to those from cough receptors. The vagus nerve also carries motor fibres apart from 80% sensory fibres. The patient would come up with coughing and Hemi-laryngopharyngeal spasm if ephaptic transmission of electrical signals moving in the vagus nerve crossed over to those motor fibers.


The findings of this case suggest the need to evaluate patients presented with medically refractory, no productive cough, for unilateral vascular compression of a vagus nerve. It also provides preliminary guidance on the symptoms, pathophysiology and treatment of the rare disease condition.


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The Annals of otology, rhinology, and laryngology


Vagus Associated Neurogenic Cough Occurring Due to Unilateral Vascular Encroachment of Its Root: A Case Report and Proof of Concept of VANCOUVER Syndrome.


Honey CR et al.

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