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Wasted tongue in neuromyelitis optica spectrum disorders due to hypoglossal nerve involvement Shanthi Viswanathan Mult Scler published online 4 June 2014 DOI: 10.1177/1352458514537698 The online version of this article can be found at: http://msj.sagepub.com/content/early/2014/06/02/1352458514537698

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case-report2014

MSJ0010.1177/1352458514537698Multiple Sclerosis JournalS Viswanathan

MULTIPLE SCLEROSIS MSJ JOURNAL

Case Report

Wasted tongue in neuromyelitis optica spectrum disorders due to hypoglossal nerve involvement

Multiple Sclerosis Journal 1­–4 DOI: 10.1177/ 1352458514537698 © The Author(s), 2014. Reprints and permissions: http://www.sagepub.co.uk/ journalsPermissions.nav

Shanthi Viswanathan

Abstract:  We report two cases of neuromyelitis optica spectrum disorder with hypoglossal nerve involvement resulting in a wasted tongue associated with other brainstem symptoms of hypogeusia, hypersalivation, hiccough, increased sweating, hyperemesis and myelitis (in the second patient). This occurred due to involvement of the hypoglossal, tractus solitarius and dorsal vagal nuclei. Though the myelitis and other brainstem signs recovered the hypoglossal nerve involvement resulting in a unilateral wasted tongue did not. It is important to consider neuromyelitis optica and its spectrum disorders in the differential diagnosis of a wasted tongue though its occurrence is rare. Keywords:  Neuromyelitis optica spectrum disorder, hypoglossal nerve, wasted tongue Date received: 20 January 2014; revised: 18 April 2014; accepted: 7 May 2014 Introduction The occurrence of a wasted tongue following a demyelinating attack involving a medullary lesion in neuromyelitis optica spectrum disorders (NMOSDs) secondary to hypoglossal nerve involvement is unusual. Hypoglossal nerve involvement in the medulla as a co-occurrence with other cranial nerve palsies and pyramidal signs has been reported due to multiple sclerosis and acute demyelinating encephalomyelitis.1,2 Here we report two cases with hypoglossal nerve involvement in association with hiccough, vomiting, hypogeusia, hypersalivation and myelitis as part of NMOSD followed by wasting of the tongue, which to the best of our knowledge has not been reported prior to this. Case 1 A 17 year old girl presented with hiccough, intractable vomiting, hypogeusia and increased sweating three months prior to being referred to our center. A year before, she had had an episode of left eye blurring of vision with poor recovery. Two years prior to the current episode, she had experienced symptoms of hiccough, vomiting and left sided body weakness recovering within a month. Neurological examination revealed reduced left eye visual acuity (20/30), relative afferent pupillary defect and optic atrophy.

Examination of the other cranial nerves was unremarkable except for deviation of the tongue to the left with weak movements, wasting and fasciculations over the left side. Motor system examination revealed pyramidal signs over the lower limbs. Cerebellar and sensory systems were intact. At the time of the attack, her Expanded Disability Status Scale (EDSS) was 4.0, which improved subsequently to 3.0.

Correspondence to: S Viswanathan Kuala Lumpur Hospital, Jalan Pahang, Kuala Lumpur, 50586, Malaysia. [email protected] Shanthi Viswanathan Kuala Lumpur Hospital, Malaysia

Case 2 A 51 year old female presented to a private hospital with intractable hiccough, vomiting, increased salivation and bulbar symptoms of inability to swallow, speak or move the tongue. This was followed by weakness over the lower limbs, power of 0/5 bilaterally and a sensory level in the thoracic region at T6. Tongue examination revealed a weak tongue, deviated more to the right side, reduced gag and a normal jaw jerk. She required intensive care monitoring and tube feeding. She was referred to our center three weeks later. At this point, the patient was noted to have a wasted fasciculating tongue, worse over the right side, with recovery of her speech and swallowing. However, she needed support with ambulation for short distances, that is, EDSS of 7.0 with persistent fecal and urinary incontinence. Visual acuity and examination of other cranial nerves were within

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Multiple Sclerosis Journal 

Figure 1.  Case 1. Left unilateral atrophy and deviation of the tongue three weeks post onset of disease (left). MRI brain axial flair showing a hyperintensity within the dorsomedial medulla and ponto-medullary midline region (center and right). Please go to: http://msj.sagepub.com/ for colour plates.

normal limits. Lower limb examination revealed weakness with a power of 4/5, hyper-reflexia, increased tone bilaterally, a sensory level to pin prick at T6, impaired vibration and joint position sense. Blood investigations for both patients including full blood count, basic metabolic panel, serum B12 levels, anti-nuclear antibody, extractable nuclear antigen, serum angiotensin converting enzyme, paraneoplastic screen, Anti Hu, Ro, Ri, chest X-ray and erythrocyte sedimentation rate were within normal limits. Cerebrospinal fluid tested for oligoclonal bands was negative in the first case but faintly positive in the second case. Anti-aquaporin 4 antibody (Anti-AQP4 ab) tested by indirect immunofluorescence (EUROIMMUNE IIFT) method was positive in the first patient but negative in the second patient. Axial magnetic resonance imaging (MRI) T2WI of the brainstem in the first patient revealed a lesion over the posterior part of the medulla near the floor of the fourth ventricle and the ponto-medullary junction in the midline. Axial MRI T2WI and flair of the brainstem in the second patient revealed medullary hyperintensities seen posteriorly within the floor of the fourth ventricle, involving the mammillary bodies, hypothalamus (which was asymptomatic), right temporal lobe and the thoracic cord from C6-T2, T10T12 and conus medullaris. Patient 1 was diagnosed as seropositive NMOSD and patient 2 was diagnosed as seronegative possible NMOSD (Figures 1 and 2). Both patients received intravenous methylprednisolone 1g o.d. for five days. The first patient recovered well from her vomiting, brainstem and autonomic symptoms except for persistence of the wasted fasciculating tongue, which has not recovered even after a year. The second patient recovered slowly

and needed plasmapheresis after two weeks. Eventually her EDSS of 7.0 improved to 4.0 and she is ambulating independently. Though her bulbar and sphincter functions improved gradually over three months, her tongue wasting did not. Both patients are currently on oral prednisolone 10 mg o.d. and azathioprine 150 mg and 100 mg respectively, relapse free. Discussion These two cases highlight the occurrence of hypoglossal nerve involvement in isolation or with other cranial nerves in patients with anti-AQP4 positive and negative forme fruste of NMOSD accompanied by brainstem and cord (second patient) symptoms. Though other brainstem and cord symptoms recovered the persistent wasted fasciculating tongue did not due to lower motor neurone involvement of the hypoglossal nerve within the posterior region of the medulla. We plan to repeat the Anti-AQP4 ab in the seronegative case in the future using cell based assays. Though hypoglossal nerve palsy in neurological conditions is not uncommon its occurrence in demyelinating diseases is not well reported.1–3 Hypoglossal nerve involvement at the nuclear or fascicular level can lead to ipsilateral and bilateral wasting. In unilateral disease deviation of the tongue towards the side of the lesion with wasting of intrinsic and extrinsic muscles and fasciculations occurs.1–3 The hypoglossal nucleus in the medulla can be affected by a variety of pathologies such as stroke, hemorrhage, tumor, botulism, amyotrophic lateral sclerosis, infection, syringobulbia, Guillain–Barré syndrome, aneurysms of vertebral arteries, internal carotid artery dissection and trauma.1–3

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S Viswanathan

Figure 2.  Case 2. Note the wasted tongue present more over the right side with deviation to the right (extreme left). MRI brain axial and sagittal flair (middle two scans) showing a lesion within the dorsal medulla and hypothalamus. MRI Flair cervico-thoracic spine sagittal view showing a longitudinally extensive cord lesion (extreme right) and axial MRI spine T2W1 showing a holocord appearance (bottom left). Please go to: http://msj.sagepub.com/ for colour plates.

Hypoglossal nerve involvement due to multiple sclerosis has been rarely reported.1–3 Isolated cranial nerve palsies when it occurs in multiple sclerosis are usually limited to the optic nerve, third, fourth, sixth, seventh and eighth nerves.2 In Keane’s original report looking at hypoglossal nerve palsies, only six patients had multiple sclerosis.3 Other demyelinating diseases such as acute demyelinating encephalomyelitis, especially in children, have been reported with hypoglossal nerve involvement as a post-infectious sequelae with Epstein–Barr virus.1,4 Recently, Okada et al. reported an unusual case of NMOSD with hiccoughs, myelitis and unilateral hypoglossal nerve involvement with cardiorespiratory arrest.5 The hypoglossal nerve is a pure motor nerve and it innervates the intrinsic and extrinsic tongue muscles. It has five segments and in demyelinating diseases it is the medullary (nuclear) part that is involved.6 The nucleus lies in the dorsomedial part of the medulla with the dorsal motor nucleus of the vagus nerve (a secretomotor parasympathetic nucleus), superior and inferior salivatory nuclei and the solitarius nuclei lying laterally.6–8 The involvement of these nuclei would have accounted for the autonomic symptoms seen in both patients, such as the hypersalivation and reduced taste (hypogeusia).9 MRI of the brain also showed involvement of the area post-rema, which is located at the infero-posterior limit of the fourth ventricle and connects to the nucleus of the solitary tract and dorsal motor nucleus. Involvement of this area and the nucleus of the solitary tract is responsible for the hiccoughs and intractable vomiting which were seen in both our patients as reported by other authors.10

In conclusion, it is important to consider neuromyelitis optica and its spectrum disorders in the differential diagnosis of unilateral or bilateral hypoglossal nerve involvement. Acknowledgement We would like to thank the Director General of Health of Malaysia for permission to publish this article and the Head of Department of Neurology, Dato Dr Mohd Hanip Rafia, for his support in the development of this study. Conflict of interest None declared. Funding This research received no specific grant from any funding agency in the public, commercial or not-for profit sectors. Permissions All patients who contributed to this study gave consent in writing for publication of their photographs.

References 1. Boban M, Brinar VV, Habek M, et al. Isolated hypoglossal nerve palsy: A diagnostic challenge. Eur Neurol 2007; 58: 177–181. 2. Thomke F, Lensch E, Ringel K, et al. Isolated cranial nerve palsies in multiple sclerosis. J Neurol Neurosurg Psychiatry 1997; 63: 682–685. 3. Keane J. Twelfth-nerve palsy: Analysis of 100 cases. Arch Neurol 1996; 53: 561–566.

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Multiple Sclerosis Journal  4. DeSimone P and Snyder D. Hypoglossal nerve palsy in infectious mononucleosis. Neurology 1978; 28: 844–847. 5. Okada S, Takarabe S, Nogawa S, et al. Persistent hiccups followed by cardiorespiratory arrest. Lancet 2012; 380: 1444.

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6. Combarros O, Alvarez de Arcaya A and Berciano J. Isolated unilateral hypoglossal nerve palsy: Nine cases. J Neurol 1998; 245: 98–100. 7. Marwah N, Agnihotri A and Goel M. Idiopathic unilateral isolated hypoglossal nerve palsy: A

case report. J Oral Health Comm Dent 2008; 2(3): 62–64. 8. Leonard CH and Gray H. The concise Gray’s anatomy. Cosimo, Inc., 2005. 9. Hashimoto T, Doden T, Ono Y, et al. Bilateral hypogeusia caused by a small lesion in the lower midbrain tegmentum. BMJ Case Rep 2012. DOI: 10.1136/bcr-2012–006837. 10. Misu T, Fujihara K, Nakashima I, et al. Intractable hiccough and nausea with periaqueductal lesions in neuromyelitis optica. Neurology 2005; 65: 1479–1482.

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Wasted tongue in neuromyelitis optica spectrum disorders due to hypoglossal nerve involvement.

We report two cases of neuromyelitis optica spectrum disorder with hypoglossal nerve involvement resulting in a wasted tongue associated with other br...
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