ARTICLE IN PRESS
YBJOM-4241; No. of Pages 3
Available online at www.sciencedirect.com
British Journal of Oral and Maxillofacial Surgery xxx (2014) xxx–xxx
Short communication
Persistent idiopathic unilateral hypoglassal nerve palsy: a case report Anna Sayan a,∗ , A.H.M.K. Abeysinghe b , Peter A. Brennan c , Velupillai Ilankovan a a b c
Poole Hospital NHS Foundation Trust, United Kingdom University of Peradeniya, Sri Lanka Portsmouth Hospital NHS Foundation Trust, United Kingdom
Accepted 7 April 2014
Abstract Paralysis of the hypoglossal nerve, the twelfth (XII) cranial nerve, leads to atrophy, deviation of the tongue to the affected side, and varying degrees of fasciculation. The injury to the nerve can occur from its origin to the tongue itself. The causes of hypoglossal nerve palsy are well documented, but idiopathic, isolated nerve palsy that does not recover, is rare. To our knowledge we describe the first case that failed to resolve, and discuss the importance of meticulous investigation to make a diagnosis and rule out sinister disease. Crown Copyright © 2014 Published by Elsevier Ltd. on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved.
Keywords: Hypoglossal Nerve; Palsy; Idiopathic
Introduction Hypoglossal nerve palsy is a rare neurological abnormality that causes characteristic clinical manifestations including unilateral atrophy of the musculature of the tongue. It is most commonly caused by tumours (49%), trauma (12%), strokes (6%), and infection (5%).1 Only 3% of cases are diagnosed as idiopathic. We report an adult patient with persistent, idiopathic, unilateral hypoglossal nerve palsy which failed to resolve, and describe the investigations used in diagnosis. Case report A 76-year-old white woman was referred with a 3-week history of dysphagia and speech impairment. Her medical ∗
Corresponding author. Tel.: +44 07814753716; fax: +44 01202 448410. E-mail addresses: [email protected], [email protected], [email protected] (A. Sayan).
history was unremarkable. There was no history of trauma, operation, infection, or cerebrovascular accident, and to our knowledge she did not take any drugs regularly other than botulinum toxin to rejuvenate the muscles of the forehead. On examination she had no facial asymmetry, but had mild dysarthria and found it difficult to articulate certain words. Intraorally there was wasting of the right side of the tongue with fasciculation, and on protrusion it deviated to the affected side (Fig. 1). The oral cavity was otherwise normal and neurological examination showed no abnormality. She had a range of haematological and biochemical investigations which were all within normal ranges, including her clotting profile. Immunological studies (rheumatoid factor, complement, antinuclear and anti-DNA antibodies) were also normal. She had no antibodies for Epstein–Barr virus, cytomegalovirus, and herpes simplex virus, and cerebrospinal fluid showed no abnormality. The normal chest radiograph excluded sarcoidosis and tuberculosis. Computed tomography and magnetic resonance imaging showed no disease along the intracranial or
http://dx.doi.org/10.1016/j.bjoms.2014.04.005 0266-4356/Crown Copyright © 2014 Published by Elsevier Ltd. on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved.
Please cite this article in press as: Sayan A, et al. Persistent idiopathic unilateral hypoglassal nerve palsy: a case report. Br J Oral Maxillofac Surg (2014), http://dx.doi.org/10.1016/j.bjoms.2014.04.005
YBJOM-4241; No. of Pages 3
2
ARTICLE IN PRESS A. Sayan et al. / British Journal of Oral and Maxillofacial Surgery xxx (2014) xxx–xxx Table 2 Special investigations for hypoglossal nerve palsy.
Fig. 1. Deviation of the tongue to the affected side on protrusion.
extracranial course of the nerve, and there was no evidence of intracranial aneurysm or arteriovenous malformation. Electromyography of the tongue showed 99% evidence of denervation of the right hypoglossal nerve. In the absence of any evidence of infection, traumatic or structural injury, or extracranial or intracranial lesions, she was diagnosed with localised vascular assault to the hypoglossal nerve. She was followed up every 3 months for the next 3 years, but it did not resolve. Discussion The hypoglossal nerve is divided into 5 anatomical sections: the medullary, cisternal, skull base, carotid space, and sublingual segments,2 each of which is usually affected by different disorders (Table 1).2,3 The medullary segment could be affected by medullary infarction, haemorrhage, multiple sclerosis, and other Table 1 Disorders that can affect segments of the hypoglossal nerve. Medullary segment Glioma Demyelination Infarction Haemorrhage Cisternal segment Aneurysm Basilar ectasia Meningioma Rheumatoid arthritis Skull base segment Metastasis Nasopharyngeal carcinoma Glomus tumours Nerve sheath tumour Carotid space segment Nodal/extranodal carcinoma Metastasis Dissection Sublingual segment Carcinoma Infection
Full blood count Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP) Coagulation (PT, INR, APTT) Haematinic status (serum folate, red cell folate, serum B12) Biochemistry (random fasting glucose, renal function test, liver function test) General immunology (IgG, IgA, IgM, C3, C4 and serum electrophoresis) Serology (herpes simplex virus, CMV, EBV) Autoimmune antibodies (antineutrophil cytoplasmic antibodies, liver kidney microsomal antibodies, smooth muscle antibodies, and parietal cell antibodies) Analysis and culture of cerebrospinal fluid Radiology Chest radiograph Computed tomogram of head and neck Magnetic resonance imaging of head and neck Neurological studies
neoplasms. Hypoglossal nerve palsy can be caused by syringobulbia, poliomyelitis, and botulism,4 and radiotherapy to the head and neck is known to cause unilateral palsy.5,6 Most idiopathic injuries to the nerve are seen in younger patients and they recover spontaneously.7–9 Some authors think that it is a clinical entity similar to that of Bell’s palsy. Unfortunately, in our patient all investigations were normal. Table 2 lists the investigations done. In a review of 26 years’ experience of hypoglossal nerve palsy, Keane reported 100 patients who ranged in age from 8 to 73 years.1 Three of them were idiopathic cases, but no details were given about age or spontaneous recovery. Combarros et al. reported 9 patients with isolated unilateral hypoglossal nerve palsy, and no cause was found in 4.10 Again, the ages were not reported, but all patients recovered. To our knowledge this is the first reported case of solitary idiopathic hypoglossal nerve palsy that did not resolve. The most likely explanation was a possible vascular injury that solely affected the XII cranial nerve. If the patient had been seen within a few days of injury, and if all investigations had shown no abnormality, anticoagulation treatment could have potentially prevented permanent damage. Ethical approval Not required. Conflict of interest None.
References 1. Keane JR. Twelfth-nerve palsy. Analysis of 100 cases. Arch Neurol 1996;53:561–6.
Please cite this article in press as: Sayan A, et al. Persistent idiopathic unilateral hypoglassal nerve palsy: a case report. Br J Oral Maxillofac Surg (2014), http://dx.doi.org/10.1016/j.bjoms.2014.04.005
YBJOM-4241; No. of Pages 3
ARTICLE IN PRESS A. Sayan et al. / British Journal of Oral and Maxillofacial Surgery xxx (2014) xxx–xxx
2. Thompson EO, Smoker WR. Hypoglossal nerve palsy: a segmental approach. Radiographics 1994;14:939–58. 3. Sharp CM, Borg HK, Kishore A, MacKenzie K. Hypoglossal nerve paralysis following tonsillectomy. J Laryngol Otol 2002;116:389–91. 4. Khoo SG, Ullah I, Wallis F, Fenton JE. Isolated hypoglossal nerve palsy: a harbinger of malignancy. J Laryngol Otol 2007;121:803–5. 5. Lin YS, Jen YM, Lin JC. Radiation-related cranial nerve palsy in patients with nasopharyngeal carcinoma. Cancer 2002;95:404–9. 6. Berger PS, Bataini JP. Radiation-induced cranial nerve palsy. Cancer 1977;40:152–5.
3
7. Bagán-Sebastián JV, Milián-Masanet MA, Pe˜narrocha-Diago M, Lloria de Miguel E. Persistent idiopathic unilateral hypoglossal nerve palsy. J Oral Maxillofac Surg 1998;56:507–10. 8. Yoon JH, Cho KL, Lee HJ, et al. A case of idiopathic isolated hypoglossal nerve palsy in a Korean child. Korean J Pediatr 2011;54:515–7. 9. Ho MW, Fardy MJ, Crean SJ. Persistent idiopathic unilateral isolated hypoglossal nerve palsy: a case report. Br Dent J 2004;196: 205–7. 10. Combarros O, Alvarez de Arcaya A, Berciano J. Isolated unilateral hypoglossal nerve palsy: nine cases. J Neurol 1998;245:98–100.
Please cite this article in press as: Sayan A, et al. Persistent idiopathic unilateral hypoglassal nerve palsy: a case report. Br J Oral Maxillofac Surg (2014), http://dx.doi.org/10.1016/j.bjoms.2014.04.005
YBJOM-4241; No. of Pages 3
Available online at www.sciencedirect.com
British Journal of Oral and Maxillofacial Surgery xxx (2014) xxx–xxx
Short communication
Persistent idiopathic unilateral hypoglassal nerve palsy: a case report Anna Sayan a,∗ , A.H.M.K. Abeysinghe b , Peter A. Brennan c , Velupillai Ilankovan a a b c
Poole Hospital NHS Foundation Trust, United Kingdom University of Peradeniya, Sri Lanka Portsmouth Hospital NHS Foundation Trust, United Kingdom
Accepted 7 April 2014
Abstract Paralysis of the hypoglossal nerve, the twelfth (XII) cranial nerve, leads to atrophy, deviation of the tongue to the affected side, and varying degrees of fasciculation. The injury to the nerve can occur from its origin to the tongue itself. The causes of hypoglossal nerve palsy are well documented, but idiopathic, isolated nerve palsy that does not recover, is rare. To our knowledge we describe the first case that failed to resolve, and discuss the importance of meticulous investigation to make a diagnosis and rule out sinister disease. Crown Copyright © 2014 Published by Elsevier Ltd. on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved.
Keywords: Hypoglossal Nerve; Palsy; Idiopathic
Introduction Hypoglossal nerve palsy is a rare neurological abnormality that causes characteristic clinical manifestations including unilateral atrophy of the musculature of the tongue. It is most commonly caused by tumours (49%), trauma (12%), strokes (6%), and infection (5%).1 Only 3% of cases are diagnosed as idiopathic. We report an adult patient with persistent, idiopathic, unilateral hypoglossal nerve palsy which failed to resolve, and describe the investigations used in diagnosis. Case report A 76-year-old white woman was referred with a 3-week history of dysphagia and speech impairment. Her medical ∗
Corresponding author. Tel.: +44 07814753716; fax: +44 01202 448410. E-mail addresses: [email protected], [email protected], [email protected] (A. Sayan).
history was unremarkable. There was no history of trauma, operation, infection, or cerebrovascular accident, and to our knowledge she did not take any drugs regularly other than botulinum toxin to rejuvenate the muscles of the forehead. On examination she had no facial asymmetry, but had mild dysarthria and found it difficult to articulate certain words. Intraorally there was wasting of the right side of the tongue with fasciculation, and on protrusion it deviated to the affected side (Fig. 1). The oral cavity was otherwise normal and neurological examination showed no abnormality. She had a range of haematological and biochemical investigations which were all within normal ranges, including her clotting profile. Immunological studies (rheumatoid factor, complement, antinuclear and anti-DNA antibodies) were also normal. She had no antibodies for Epstein–Barr virus, cytomegalovirus, and herpes simplex virus, and cerebrospinal fluid showed no abnormality. The normal chest radiograph excluded sarcoidosis and tuberculosis. Computed tomography and magnetic resonance imaging showed no disease along the intracranial or
http://dx.doi.org/10.1016/j.bjoms.2014.04.005 0266-4356/Crown Copyright © 2014 Published by Elsevier Ltd. on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved.
Please cite this article in press as: Sayan A, et al. Persistent idiopathic unilateral hypoglassal nerve palsy: a case report. Br J Oral Maxillofac Surg (2014), http://dx.doi.org/10.1016/j.bjoms.2014.04.005
YBJOM-4241; No. of Pages 3
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ARTICLE IN PRESS A. Sayan et al. / British Journal of Oral and Maxillofacial Surgery xxx (2014) xxx–xxx Table 2 Special investigations for hypoglossal nerve palsy.
Fig. 1. Deviation of the tongue to the affected side on protrusion.
extracranial course of the nerve, and there was no evidence of intracranial aneurysm or arteriovenous malformation. Electromyography of the tongue showed 99% evidence of denervation of the right hypoglossal nerve. In the absence of any evidence of infection, traumatic or structural injury, or extracranial or intracranial lesions, she was diagnosed with localised vascular assault to the hypoglossal nerve. She was followed up every 3 months for the next 3 years, but it did not resolve. Discussion The hypoglossal nerve is divided into 5 anatomical sections: the medullary, cisternal, skull base, carotid space, and sublingual segments,2 each of which is usually affected by different disorders (Table 1).2,3 The medullary segment could be affected by medullary infarction, haemorrhage, multiple sclerosis, and other Table 1 Disorders that can affect segments of the hypoglossal nerve. Medullary segment Glioma Demyelination Infarction Haemorrhage Cisternal segment Aneurysm Basilar ectasia Meningioma Rheumatoid arthritis Skull base segment Metastasis Nasopharyngeal carcinoma Glomus tumours Nerve sheath tumour Carotid space segment Nodal/extranodal carcinoma Metastasis Dissection Sublingual segment Carcinoma Infection
Full blood count Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP) Coagulation (PT, INR, APTT) Haematinic status (serum folate, red cell folate, serum B12) Biochemistry (random fasting glucose, renal function test, liver function test) General immunology (IgG, IgA, IgM, C3, C4 and serum electrophoresis) Serology (herpes simplex virus, CMV, EBV) Autoimmune antibodies (antineutrophil cytoplasmic antibodies, liver kidney microsomal antibodies, smooth muscle antibodies, and parietal cell antibodies) Analysis and culture of cerebrospinal fluid Radiology Chest radiograph Computed tomogram of head and neck Magnetic resonance imaging of head and neck Neurological studies
neoplasms. Hypoglossal nerve palsy can be caused by syringobulbia, poliomyelitis, and botulism,4 and radiotherapy to the head and neck is known to cause unilateral palsy.5,6 Most idiopathic injuries to the nerve are seen in younger patients and they recover spontaneously.7–9 Some authors think that it is a clinical entity similar to that of Bell’s palsy. Unfortunately, in our patient all investigations were normal. Table 2 lists the investigations done. In a review of 26 years’ experience of hypoglossal nerve palsy, Keane reported 100 patients who ranged in age from 8 to 73 years.1 Three of them were idiopathic cases, but no details were given about age or spontaneous recovery. Combarros et al. reported 9 patients with isolated unilateral hypoglossal nerve palsy, and no cause was found in 4.10 Again, the ages were not reported, but all patients recovered. To our knowledge this is the first reported case of solitary idiopathic hypoglossal nerve palsy that did not resolve. The most likely explanation was a possible vascular injury that solely affected the XII cranial nerve. If the patient had been seen within a few days of injury, and if all investigations had shown no abnormality, anticoagulation treatment could have potentially prevented permanent damage. Ethical approval Not required. Conflict of interest None.
References 1. Keane JR. Twelfth-nerve palsy. Analysis of 100 cases. Arch Neurol 1996;53:561–6.
Please cite this article in press as: Sayan A, et al. Persistent idiopathic unilateral hypoglassal nerve palsy: a case report. Br J Oral Maxillofac Surg (2014), http://dx.doi.org/10.1016/j.bjoms.2014.04.005
YBJOM-4241; No. of Pages 3
ARTICLE IN PRESS A. Sayan et al. / British Journal of Oral and Maxillofacial Surgery xxx (2014) xxx–xxx
2. Thompson EO, Smoker WR. Hypoglossal nerve palsy: a segmental approach. Radiographics 1994;14:939–58. 3. Sharp CM, Borg HK, Kishore A, MacKenzie K. Hypoglossal nerve paralysis following tonsillectomy. J Laryngol Otol 2002;116:389–91. 4. Khoo SG, Ullah I, Wallis F, Fenton JE. Isolated hypoglossal nerve palsy: a harbinger of malignancy. J Laryngol Otol 2007;121:803–5. 5. Lin YS, Jen YM, Lin JC. Radiation-related cranial nerve palsy in patients with nasopharyngeal carcinoma. Cancer 2002;95:404–9. 6. Berger PS, Bataini JP. Radiation-induced cranial nerve palsy. Cancer 1977;40:152–5.
3
7. Bagán-Sebastián JV, Milián-Masanet MA, Pe˜narrocha-Diago M, Lloria de Miguel E. Persistent idiopathic unilateral hypoglossal nerve palsy. J Oral Maxillofac Surg 1998;56:507–10. 8. Yoon JH, Cho KL, Lee HJ, et al. A case of idiopathic isolated hypoglossal nerve palsy in a Korean child. Korean J Pediatr 2011;54:515–7. 9. Ho MW, Fardy MJ, Crean SJ. Persistent idiopathic unilateral isolated hypoglossal nerve palsy: a case report. Br Dent J 2004;196: 205–7. 10. Combarros O, Alvarez de Arcaya A, Berciano J. Isolated unilateral hypoglossal nerve palsy: nine cases. J Neurol 1998;245:98–100.
Please cite this article in press as: Sayan A, et al. Persistent idiopathic unilateral hypoglassal nerve palsy: a case report. Br J Oral Maxillofac Surg (2014), http://dx.doi.org/10.1016/j.bjoms.2014.04.005
