Assistant Professor Rush University Chicago, Illinois, United States
Disclosure(s):
MAITRI JETLY, n/a: No financial or non-financial relationships to disclose
Lisa A. LaGorio, PhD, MPH, CCC-SLP: Rush University: Financial - Salary (Ongoing). No non-financial relationships to disclose
Abstract: Patient A was suspected to have Jugular Foramen Syndrome (JFS), based on initial neurological evaluation, imaging studies and symptom presentation. However, he did not clearly fit the criteria for JFS which led to further probing into the etiology eventually diagnosed with base of tongue squamous cell carcinoma (T4N2cM0). This case highlights the role of SLP in differential diagnosis of complex dysphagia cases.
Summary of Presentation : Introduction: Jugular Foramen Syndrome (JFS) is the paralysis of the Glossopharyngeal Nerve (CN IX), Vagus Nerve (CN X) and Spinal Accessory Nerves (CN XI) traversing through the jugular foramen (Das & Khalili, 2019; Gutierrez et al., 2020.). The clinical manifestations of JFS have typically been secondary to metastatic diseases, vascular lesions, traumatic brain injuries or viral infections (Das & Khalili, 2019; Gutierrez et al., 2020; Hayward et al., 2012). The syndrome consists of hoarseness, dysphagia, and unilateral dull and aching pain in the involved occipital and pharyngeal regions ‘‘behind the ear’’ (Laigle-Donadey et al., 2005).
Methods: Multidisciplinary approach was employed to arrive at the diagnosis. The diagnostic team included the Primary Care Physician (PCP), Neurologist, Otolaryngologist and SLP. Oral Mechanism Evaluation (OME) and Video Fluoroscopic Swallow Study (VFSS) were conducted by the Speech Language Pathology based on referral from the Neurologist.
CASE STUDY SUMMARY: Patient A is a 52-year-old male, with a complex medical history of Obstructive Sleep Apnea (OSA), Type II Diabetes Mellitus (DM-2), Chronic Kidney Disease (CKD) and Hypertension (HTN), who complained of progressively worsening swallowing difficulties and odynophagia accompanied with right-sided otalgia, hoarseness with intermittent dysphonia, and right-sided neck pain. He first presented to his PCP who ordered a battery of imaging exams; all were negative. Approximately 6 months later as symptoms worsened, patient saw a neurologist. Neurology evaluation revealed intact CN XI, proprioceptive skills, and motor coordination, narrowing the possible etiology to JFS or a mass lesion or vascular compression; but, spinal tap results were inconclusive. Because of the underlying swallowing symptoms, neurologist recommended VFSS by SLP. SLP completed OME revealed diminished sensation on the right-side lower jaw, poor palatal elevation on the right, right tongue hemi-atrophy, reduced gag reflex and trismus (Jaw opening: < 45mm). Of note, this facial asymmetry emerged post the prior neurological evaluation. The results of the VFSS evaluation revealed mild to moderate oropharyngeal dysphagia. Oral phase characterized by impaired bolus formation, impaired mastication, impaired anterior-posterior transit, and piecemeal deglutition for all liquids and solids. Pharyngeal phase is characterized by generalized bilateral weakness; impaired/reduced hyolaryngeal excursion with limited elevation and no anterior movement; impaired tongue base retraction with absent epiglottis retroflexion; reduced pharyngeal constriction overall, but, worse for the inferior constrictor in the hypopharynx; decreased airway protection with decreased laryngeal vestibule closure during the swallow; and decreased duration of UES relaxation. Together, these resulted in small amount of penetration of thin liquids with penetration-aspiration score = 5. Overall, oral phase deficits were consistent with R-sided CN XII impairment, while pharyngeal phase deficits were consistent with L-sided CN X deficit. This asymmetry was an unexpected finding. Additionally, laryngeal movement was symmetric during phonation, and L-side appeared smaller than R side, another unexpected finding. ENT consult was recommended. ENT evaluation with laryngoscopy revealed an irregularity at the base of the tongue and vallecula, Repeat imaging revealed denervation injury of right hemi-tongue, absent abnormalities in the right jugular foramen and ill-defined heterogenous mass in the floor of the mouth extending into the pre-epiglottic space. Biopsy findings confirmed moderately differentiated squamous cell carcinoma lesion in the right base of the tongue (R SCC-BOT)
RESULTS AND Discussion: Initial neurologist evaluation suggested right-sided JFS, but, results of further testing by SLP a few weeks later indicated additional damage to L-sided CN X, as well as impaired mandibular branch of trigeminal nerve (CN V3) and facial nerve (CN VII), and intact CN XI, suggesting that clinical presentation did not distinctly fit the criteria for JFS. Additionally, during the VFSS, the patient adequately cleared penetrated liquids and oropharyngeal residue by initiating cough reflex and multiple swallows respectively, indicating intact sensory pathways, also not consistent with JFS. This sensory finding aided in confirming the neuromotor nature of the symptoms. Furthermore, the presence of laryngeal movement during phonation was inconsistent with unilateral clinical presentation of signs and symptoms evidenced from the OME. Finally, the absence symptoms usually seen in JFS including lingual fasciculations, decreased salivation, unilateral vocal cord paralysis and loss of sensory functioning, stressed the need for further probing into the etiology. Ultimately, results from various disciplines indicated the need for substantial evidence. The MRI scans confirmed the etiology of R SCC BOT, simultaneously ruling out the possibility of JFS.
Conclusion: Dysphagia is the most frequent presenting symptom in individuals with JFS. Some of the clinical manifestations of JFS overlap with oropharyngeal carcinomas thus emphasizing the need for accurate differential diagnosis. The case study highlights the vital role of Speech Language Pathologists (SLP) in providing valuable supplemental information to assist in the differential diagnosis process.
References: Gutierrez, S., Warner, T., McCormack, E., Werner, C., Mathkour, M., Iwanaga, J., Uz, A., Dumont, A. S., & Tubbs, R. S. (2020). Lower cranial nerve syndromes. Neurosurgical Review, 44(3), 1345–1355. Hayward, D. M., Morgan, C., Biller, J., Emami, B., & Prabhu, V. C. (2012). Jugular foramen syndrome as initial presentation of metastatic lung cancer. Journal of Neurological Surgery Part B: Skull Base, 73(S 01). Johnson, D.E., Burtness, B., & Leemans, C.R. (2020). Head and neck squamous cell carcinoma. Nat Rev Dis Primers, 6, 92. Laigle-Donadey, F., Taillibert, S., Martin-Duverneuil, N., Hildebrand, J., & Delattre, J.-Y. (2005). Skull-base metastases. Journal of Neuro-Oncology, 75(1), 63–69. Silva, P.F., Junior, J.R., & Campos, M.F. (2021). Jugular foramen meningioma manifesting as Jackson syndrome. Journal of Neurology and Neuroscience, 12(404), 1–3. Tamrazian, E., & Mehta, B. (2020). Dysphagia and tongue deviation: A rare case of collett-sicard syndrome after blunt head trauma. Neurology International, 12(3), 136–139.
Learning Objectives:
At the completion of this activity, participants should be able to:
Upon completion participant will be able to understand the completion of Jugular Foramen Syndrome.
Upon completion the participant will be able to apply the knowledge of symptomatology of Jugular Foramen Syndrome during dysphagia evaluation.
Upon completion, participant wil be able to understand the role of Speech Language Pathologist (SLP) as a crucial collaborator of the multidisciplinary diagnostic team in complex dysphagia cases.