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Year : 2020  |  Volume : 13  |  Issue : 6  |  Page : 685-687  

Unilateral hypoglossal nerve palsy with malignancy of unknown origin

Department of Palliative Care and Psychooncology, Tata Medical Center, Kolkata, West Bengal, India

Date of Submission09-Sep-2019
Date of Decision22-Oct-2019
Date of Acceptance31-Dec-2019
Date of Web Publication6-Nov-2020

Correspondence Address:
Shrikant Atreya
Department of Palliative Care and Psychooncology, Tata Medical Center, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_252_19

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Paralysis of hypoglossal nerve is rare and often underreported. The paralysis leads to the deviation of tongue, dysarthria, dysphagia, and airway dysfunction, affecting the quality of life. We report a case of a middle-aged woman with malignancy of unknown origin with bone and brain metastasis who presented with unilateral deviation of the tongue. Detailed workup inferred with metastasis to the clivus bone which is related to the hypoglossal canal at the base of the skull and the primary origin could not be established. Intravenous steroids, radiotherapy, and systemic chemotherapy were helpful in improvement of the nerve palsy in our patient. Hypoglossal nerve palsy is often an underrated and underreported lesion; however, it can be a starting point for a major underlying problem.

Keywords: Hypoglossal nerve palsy; malignancy of unknown origin; palliative care

How to cite this article:
Patil CR, Atreya S. Unilateral hypoglossal nerve palsy with malignancy of unknown origin. Med J DY Patil Vidyapeeth 2020;13:685-7

How to cite this URL:
Patil CR, Atreya S. Unilateral hypoglossal nerve palsy with malignancy of unknown origin. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2021 Sep 20];13:685-7. Available from: https://www.mjdrdypv.org/text.asp?2020/13/6/685/300135

  Introduction Top

Hypoglossal nerve is the 12th cranial nerve and primarily motor in its physiological course. Paralysis of this nerve is associated with atrophy and weakness of extrinsic and intrinsic muscles of the tongue often associated with dysarthria, dysphagia, and airway dysfunction, significantly affecting the quality of life.[1] Unilateral involvement of this nerve is rare and often underreported.[2] A case series by Keane reported that the most common etiology of hypoglossal nerve palsy was malignancy,[3] most often originating from breast,[4],[5] lung,[6] and lymphoma[7] or a direct invasion of head-and-neck tumor.[8] The present case talks about a middle-aged woman with malignancy of unknown origin with bone and brain metastasis who presented with unilateral deviation of the tongue.

  Case Report Top

A 41-year-old female patient diagnosed as malignancy of unknown origin with metastasis to multiple axial and appendicular skeleton, soft-tissue lesion in the left breast, and abdominal lymphadenopathy, presented to the emergency department with 7-day history of swelling in both the lower limbs and severe pain (Edmonton Symptom Assessment Score [ESAS] of 7/10 describes the pain) in both of her lower limbs, which was gnawing in nature with no signs of radiculopathy. On physical examination, the patient had calf tenderness (Homan's sign was positive) and significant edema of both the lower limbs. She also had a 4-month history of moderate pain (ESAS – 6/10) in her lower back, dull-aching type of pain, and was localized. A Doppler ultrasonography of both the lower limbs and the pelvis was done that revealed venous thrombosis of the right femoral and saphenous veins, left common femoral vein, left superficial femoral vein, great saphenous vein, left popliteal vein, and left posterior tibial vein. Basic investigations such as renal function test, liver function test, and complete blood count were unremarkable. She was admitted under palliative care team for pain management and simultaneously evaluated by the medical oncology team for her primary site of malignancy. She was started on low-molecular-weight heparin (LMWH) for her venous thrombosis, continuous intravenous infusion of fentanyl (for dose titration), and nonsteroidal anti-inflammatory drugs (NSAIDs) for her pain.

On day 2 of admission, she developed a deviation of the tongue to the left side [Figure 1]. She had no dysarthria or dysphagia. On further clinical examination except for the tongue deviation to the left side, there was no other cranial nerve deficit. In view of a diagnosis of unilateral hypoglossal nerve palsy we suspected with a differential diagnosis of brain metastasis, infection, and vascular malformations. So, magnetic resonance imaging (MRI) of the brain was advised. The MRI of the brain revealed heterogeneous bony lesions on the left side of the clivus with marrow edema and lesions on the left greater wing of sphenoid, lateral wall of orbit, frontal process of left orbital bone, and left occipital condyle [Figure 2]. She was started on intravenous dexamethasone 8 mg three times a day and referred to a radiation oncologist for opinion on whole-brain radiotherapy. She received radiotherapy (20 Gray in five fractions) to the base of the skull. She was further investigated using immunohistochemistry markers to confirm the diagnosis, but they failed to ascertain the origin of the malignancy. The medical oncology team started her on palliative chemotherapy in the background of malignancy of unknown origin. At discharge, she was administered fentanyl transdermal patch of 50 μg/h (after dose titration with fentanyl infusion), NSAIDs, and tapering course of steroids. The swelling in her lower limbs improved with LMWH. On repeat follow-up in the outpatient department, her lower limb edema had reduced, pain was controlled, and the deviation of the tongue also had improved.
Figure 1: Photograph showing deviation of the tongue toward left side

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Figure 2: Magnetic resonance imaging showing the metastasis to the base of the skull

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  Discussion Top

Hypoglossal nerve is the most caudal and final nerve from the brain. Physiologically, it is responsible for the functions of mastication, speech, airway protection, and swallowing.[1] Hypoglossal nuclei are paired and have bilateral innervations that originate anterior to the medulla in the preolivary sulcus which separates the olive and the pyramid.[9] Nerve fibers from these nuclei have a course superior to the vertebral artery and through the hypoglossal canal (clivus bone forms the superior part of the hypoglossal canal on both the sides), they leave the skull base. Internal carotid artery and jugular bulb lie lateral to the hypoglossal nerve at this level. On its further course, the nerve transits through the nasopharyngeal carotid space closely associated with glossopharyngeal and spinal accessory nerves. At the level of mastoid tip, the nerve approaches the mylohyoid and hyoglossus muscles in the sublingual region. The hypoglossal nerve communicates with various other nerves such as sympathetic trunk, pharyngeal nerves, and vagus nerve in this course.[10]

Hypoglossal nerve palsy provides motor supply to the tongue, wherein a lesion of the nerve can lead to tongue deviation and atrophy of the tongue muscles; however, it will be imperative as a clinician to know the level of the nerve lesion. The deviation of the tongue toward the same side of the lesion often indicates an infranuclear lesion as the contralateral genioglossus is acting opposed. On the contrary, in supranuclear lesions, the deviation will be on the opposite side.[11] As this nerve is related to glossopharyngeal nerve near the carotid space, there can be presentations with associated dysphagia in patients.[11]

Of importance to note here is the two anatomical landmarks in the course of hypoglossal nerve which act as crucial factors in causing the nerve palsy. The first landmark is the skull base where fractures to the base of the skull, skull base tumors such as glomus jugulare or metastasis,[4],[5],[6],[7] and nasopharyngeal neoplasms[8] lead to hypoglossal nerve palsy. Identifying the primary site for cases with metastasis is important in planning the specific treatment of the patient. A locally advanced breast cancer patient presented with isolated history of atrophy and deviation of the tongue. On further evaluation, she was found to have metastasis to the clivus bone affecting the hypoglossal canal.[4] Another published case report by Jamison[5] described a case of hypoglossal nerve palsy which was referred to his department, quoting it to be suspected stroke. Upon evaluation, the patient was found to have metastatic deposits to the skull base and the primary was found to be breast. Bryer and Henry[7] reported a rare case of peripheral T cell lymphoma with unilateral hypoglossal nerve palsy. The evaluation revealed meningeal thickening at the hypoglossal canal as the source of palsy in this patient. Radiation to the base of the skull, systemic steroids, and systemic chemotherapy are the mainstay treatment for reversing the hypoglossal nerve palsy in the above-described cases.

The second landmark is carotid and sublingual spaces where infection[8],[12],[13],[14] and malignancy are common. Very rarely, carotid artery dissection also leads to hypoglossal nerve palsies.[15] A clinical note published by Afifi et al.[14] describes a case of infectious mononucleosis to be the etiological factor for isolated, reversible hypoglossal nerve paralysis. Despite the best investigation, the underlying etiological factors are difficult to diagnose in rare cases.[12],[13],[16],[17]

Our patient had isolated infranuclear hypoglossal nerve lesion due to metastasis to the clivus bone. Timely investigation followed by steroid and radiotherapy helped reverse the palsy.

  Conclusion Top

Hypoglossal nerve palsy is often an underrated and underreported lesion; however, it can be a starting point for a major underlying problem. As palliative care physicians, it is essential for us to identify the underlying cause and direct treatment toward symptoms and the underlying cause. As in our case though the patient presented to us as a metastatic cancer of unknown origin, we had the opportunity to improve her general health condition and transfer her to the oncology department for further disease-directed treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Rea P. Hypoglossal Nerve. In: Rea P, editor. Clinical Anatomy of the Cranial Nerves. San Diego: Academic Press; 2014. p. 127-35.  Back to cited text no. 1
Dubal PM, Svider PF, Gupta A, Eloy JA, Liu JK. Injuries of the cranial nerves. In: Tubbs RS, editor. Nerve and Nerve Injuries. San Diego: Academic Press; 2015. p. 451-68.  Back to cited text no. 2
Keane JR. Twelfth-nerve palsy. Analysis of 100 cases. Arch Neurol 1996;53:561-6.  Back to cited text no. 3
Chen YG, Dai MS, Ho CL, Huang TC. Isolated hypoglossal nerve paralysis. Am J Med 2014;127:926-7.  Back to cited text no. 4
Jamison A. Isolated hypoglossal nerve palsy as a result of metastatic breast cancer: A case report. Oral Surg 2019;12:71-4.  Back to cited text no. 5
Juneja H, Dabla S, Yadav M, Pahuja I, Kumar S, Singh P. Isolated hypoglossal nerve palsy in a patient with non- small cell lung carcinoma: A rare paraneoplastic neurological syndrome. Annu Clin Case Reports 2017;2:1-3.  Back to cited text no. 6
Bryer E, Henry D. Isolated hypoglossal nerve palsy as a presenting symptom of metastatic peripheral T-cell lymphoma – Not otherwise specified (PTCL-NOS): A unique case & a review of the literature. Int J Hematol Oncol 2018;7:IJH03.  Back to cited text no. 7
Sharma B, Dubey P, Kumar S. Isolated unilateral hypoglossal nerve palsy?: A study of 12 cases. J Neurology Neurosci 2010;2:4.  Back to cited text no. 8
Loh C, Maya MM, Go JL. Cranial nerve XII: The hypoglossal nerve. Semin Ultrasound CT MR 2002;23:256-65.  Back to cited text no. 9
Shoja MM, Oyesiku NM, Shokouhi G, Griessenauer CJ, Chern JJ, Rizk EB, et al. A comprehensive review with potential significance during skull base and neck operations, Part II: Glossopharyngeal, vagus, accessory, and hypoglossal nerves and cervical spinal nerves 1-4. Clin Anat 2014;27:131-44.  Back to cited text no. 10
hompson E, Smoker R. Hypoglossal Nerve: A segmental approach. Radiographics 1994;14:939-54.  Back to cited text no. 11
Yoon JH, Cho KL, Lee HJ, Choi SH, Lee KY, Kim SK, et al. A case of idiopathic isolated hypoglossal nerve palsy in a Korean child. Korean J Pediatr 2011;54:515-7.  Back to cited text no. 12
Ho MW, Fardy MJ, Crean SJ. Persistent idiopathic unilateral isolated hypoglossal nerve palsy: A case report. Br Dent J 2004;196:205-7.  Back to cited text no. 13
Afifi AK, Rifai ZH, Faris KB. Isolated, reversible, hypoglossal nerve palsy. Arch Neurol 1984;41:1218.  Back to cited text no. 14
Riancho J, Infante J, Mateo JI, Berciano J, Agea L. Unilateral isolated hypoglossal nerve palsy associated with internal carotid artery dissection. J Neurol Neurosurg Psychiatry 2013;84:706.  Back to cited text no. 15
Shibata A, Kimura M, Ishibashi K, Umemura M. Idiopathic isolated unilateral hypoglossal nerve palsy: A report of 2 cases and review of the literature. J Oral Maxillofac Surg 2018;76:1454-9.  Back to cited text no. 16
Freedman M, Jayasundara H, Stassen LF. Idiopathic isolated unilateral hypoglossal nerve palsy: A diagnosis of exclusion. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:e22-6.  Back to cited text no. 17


  [Figure 1], [Figure 2]


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