Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 2415  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,511 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this Article
   References
   Article Figures

 Article Access Statistics
    Viewed2381    
    Printed23    
    Emailed0    
    PDF Downloaded42    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents    
LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 6  |  Page : 1313-1316

Recurrent isolated abducens nerve palsy – unusual manifestation of a known syndrome: Report of two cases


Department of Neurology, MS Ramaiah Medical College and Hospital, Bengaluru, Karnataka, India

Date of Web Publication11-Nov-2016

Correspondence Address:
Rohan Mahale
Department of Neurology, MS Ramaiah Medical College and Hospital, Bengaluru, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.193807

Rights and Permissions



How to cite this article:
Mahale R, Mehta A, Buddaraju K, Shankar AK, Javali M, Acharya P, Srinivasa R. Recurrent isolated abducens nerve palsy – unusual manifestation of a known syndrome: Report of two cases. Neurol India 2016;64:1313-6

How to cite this URL:
Mahale R, Mehta A, Buddaraju K, Shankar AK, Javali M, Acharya P, Srinivasa R. Recurrent isolated abducens nerve palsy – unusual manifestation of a known syndrome: Report of two cases. Neurol India [serial online] 2016 [cited 2019 May 24];64:1313-6. Available from: http://www.neurologyindia.com/text.asp?2016/64/6/1313/193807


Sir,

Abducens nerve has the longest intracranial course after emerging at the pontomedullary sulcus in the caudal pons. It is vulnerable to compression owing to its points of fixation along its course, its tortuosity, and its attachment to the skull base. Following are the etiologies of abducens nerve palsy: Idiopathic (8–30%), ischemic (0–36%), traumatic (3–30%), aneurysmal compression (0–6%), and miscellaneous (10–30%).[1] Microvascular cause is the most common attributable cause for an acute isolated ocular motor mononeuropathy. Non-microvascular causes such as neoplasms and inflammation should be considered in cases of isolated relapsing-remitting abducens nerve palsies.[2] Here, we report two patients who presented with isolated relapsing-remitting abducens nerve palsies. The first patient had primary Sjőgren's syndrome (SS) and the second patient had idiopathic intracranial hypertension (IIH). Primary SS and IIH presenting as isolated relapsing-remitting abducens nerve palsy have not been reported.

Patient 1

A 44-year-old lady presented with a history of throbbing pain in the right periorbital region radiating to the right frontotemporal region of 1-week duration. This was followed by double vision lasting for two days mainly in the primary position of the eye and also towards the right lateral gaze that disappeared on closing one eye. There was no redness of eyes, proptosis, fever, or vomiting. She did not have any other comorbidities. Two months prior to the present symptomatology, she had similar symptoms of pain in the right periorbital region radiating to the right frontotemporal region along with double vision. She was diagnosed to be having a right lateral rectus weakness due to right abducens nerve palsy and was treated with oral prednisolone for 2 weeks. She had symptomatic improvement following which the steroids were tapered and stopped. Neurologically, she was conscious and oriented. There was esotropia of the right eye with impaired abduction suggestive of right lateral rectus palsy. Other cranial nerves were normal. There were no other abnormalities on neurological examination. A clinical diagnosis of recurrent right abducens nerve palsy was made. Magnetic resonance images (MRI) of the brain with gadolinium enhancement did not reveal abducens nerve or meningeal enhancement. Cerebrospinal fluid (CSF) analysis showed a normal opening pressure, protein 72 mg/dL, cell count 3 cells/mm 3 (lymphocytes), and no malignant cells. Complete hemogram showed an elevated erythrocyte sedimentation rate (ESR; 35 mm/h). The renal, hepatic, and thyroid function tests were normal. Plasma glycated haemoglobin was normal. Serum antinuclear, anti-SS-A, and SS-B antibodies were positive. Serological testing for human immunodeficiency virus (HIV) and hepatitis B and C were negative. Schirmer's test to assess the adequacy of lacrimal secretions was positive (5 mm of tear flow over 5 min). On further questioning, the patient did not report any history of dry eyes or mouth. Lip biopsy showed aggregated foci of lymphocytes in the minor salivary glands [Figure 1], [Figure 2], [Figure 3]. She was started on oral steroids (1 mg/kg/day) with azathioprine (2 mg/kg/day). At follow-up after 8 weeks, there was significant improvement in her symptomatology with amelioration of the lateral rectus palsy. She remains asymptomatic at subsequent follow-up conducted every 3 months for 2 years.
Figure 1: Hematoxylin and eosin preparation of lip biopsy specimen showing moderately intense patchy interstitial lymphocytic infiltration of minor salivary glands lobules (black arrow)

Click here to view
Figure 2: Hematoxylin and eosin preparation of lip biopsy specimen showing periductal chronic inflammation (black arrow)

Click here to view
Figure 3: Hematoxylin and eosin preparation of lip biopsy specimen magnified view showing periductal chronic inflammation (black arrow)

Click here to view


Patient 2

A 36-year-old male patient presented with a history of double vision of 2-day duration mainly on primary position and looking towards the left lateral gaze. The diplopia disappeared on closing one eye. There was no redness of eyes, proptosis, blurring of vision, fever, or headache. He did not have any other comorbidity. Previously, he had 2 similar episodes; one 4 years ago and another 2 years ago. On both the occasions, he had painless binocular double vision in the left lateral gaze. Brain MRI with gadolinium enhancement was normal. He was treated as a case having a left abducens nerve palsy in another hospital with oral steroids and improved completely. Neurologically, he was conscious and oriented. Fundus examination showed blurring of the nasal disc margin of both the eyes. Pupils were equal and reactive. There was esotropia of the left eye with impaired abduction suggestive of left lateral rectus palsy. Other cranial nerves were normal. A clinical diagnosis of recurrent left abducens nerve palsy was made. Complete hemogram and renal, hepatic, and thyroid function tests were normal. Plasma glycated hemoglobin was normal. Serum antinuclear antibodies were negative. Serological testing for HIV, hepatitis B surface antigen, and Venereal Disease Research Laboratory test were negative. Brain MRI with gadolinium enhancement did not reveal abducens nerve or meningeal enhancement. MR venography was normal. CSF pressure was high (390 mm of CSF) with normal protein, glucose, and cell count. A diagnosis of isolated relapsing-remitting sixth nerve palsy without headache due to IIH was made. He was treated with acetazolamide and oral glycerol. Visual field charting showed concentric constriction of the visual fields in both the eyes. He was referred to an ophthalmologist for optic nerve fenestration. There was improvement in lateral rectus function at a 3-month follow-up.

Isolated abducens nerve palsy in young individuals should be evaluated with neuroimaging to rule out meningeal infiltrative and metastatic processes.[1] Isolated abducens nerve palsy in adults more than 50 years of age are usually ischemic if there are associated vascular risk factors such as diabetes mellitus, hypertension, or dyslipidemia. Usually, isolated ischemic abducens nerve palsy returns to normality within 3 months. Persistent abnormality beyond 3 months require investigations to determine the presence of other etiologies, such as infective and inflammatory causes, namely, syphilis, collagen vascular disease, and sarcoidosis.[1],[3] Following are the causes for recurrent isolated ischemic abducens nerve palsy: Aneurysm,[4] vasculopathy,[5] sphenoiditis,[6] metastases and tumors like a parasellar/petrous apex meningioma.[3]

The neurological involvement in primary SS ranges from peripheral nerve (10–20%) to cranial nerve and central nervous system involvement (1.5 to 20%).[7] The first patient was diagnosed to be having a primary SS, which caused recurrent cranial mononeuropathy but sicca symptoms or systemic manifestations were absent. Patient had positive anti-SS-A and SS-B antibodies, diagnostic histopathological features in salivary gland biopsy, and a positive Schirmer's test. Three out of 6 criteria set by European Community Study (ECS) group for the diagnosis of primary SS were, therefore, fulfilled.[8]

The absence of xerostomia or xerophthalmia does not exclude the diagnosis of SS. Delalande et al., reported that 38% patients had sicca symptoms before, 15% patients had these symtoms at the same time, whereas 47% patients had these symptoms after the onset of neurologic features.[9] The exact pathomechanisms underlying the cranial neuropathy in SS are largely unknown. Vasculitis in small arteries and arterioles with subsequent development of an axonopathy is the postulated mechanism for multiple cranial neuropathies, except for trigeminal neuropathy, which is due to ganglionopathy/ganglionitis.

There are only a few reports on the isolated cranial mononeuropathy caused by SS. Galbussera et al., reported a case of isolated oculomotor nerve palsy related to SS.[7] Lui et al., reported a patient with isolated oculomotor nerve palsy due to SS showing rapid and almost complete recovery after 2 months of corticosteroid and azathioprine treatment.[10] Chu et al., reported a patient with isolated trochlear nerve palsy due to SS.[11] Isolated abducens nerve palsy due to SS has not been reported.

IIH presenting as isolated relapsing-remitting sixth nerve palsy without headache has not been reported. IIH is a syndrome in which there is increased intracranial pressure (ICP) of an unknown cause. Revised modified Dandy criteria is used to define the syndrome: (1) Symptoms and signs of raised ICP (headache, nausea, vomiting, pulsatile tinnitus, transient visual obscurations, and papilledema); (2) no localizing signs, except for abducens nerve palsy; (3) no cause for raised ICP identified on neuroimaging (brain MRI); (4) CSF opening pressure of greater than 25 cmH2O, with normal CSF composition; and (5) no alternate explanation for the raised ICP.[12] Headache is the most common symptom at presentation and is typically holocranial, worse after waking up. Papilledema is the most common sign at presentation. Untreated papilledema results in progressive irreversible visual loss and secondary optic atrophy. The patients may have binocular horizontal diplopia due to unilateral or bilateral abducens nerve palsy. Vertical diplopia, due to third nerve palsy, fourth nerve palsy, or skew deviation, has been rarely reported in patients with a high CSF opening pressure.

The present case series depicts two different etiologies for isolated relapsing-remitting abducens nerve palsy in middle aged patients. Both etiologies are known syndromes but the presenting manifestations in these patients were unusual. Primary SS should be considered in patients with recurrent cranial mononeuropathy even in the absence of sicca symptoms. Measurement of CSF opening pressure should be done in patients with recurrent isolated abducens nerve palsy to rule out IIH.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Richards BW, Jones FR Jr, Younge BR. Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol 1992;113:489-96.  Back to cited text no. 1
    
2.
Tamhankar MA, Biousse V, Ying GS, Prasad S, Subramanian PS, Lee MS, et al. Isolated third, fourth, and sixth cranial nerve palsies from presumed microvascular versus other causes: A prospective study. Ophthalmology 2013;120:2264-9.  Back to cited text no. 2
    
3.
Chan JW, Albretson J. Causes of isolated recurrent ipsilateral sixth nerve palsies in older adults: A case series and review of the literature. Clin Ophthalmol 2015;9:373-7.  Back to cited text no. 3
    
4.
Nguyen DQ, Perera L, Kyle G. Recurrent isolated sixth nerve palsy secondary to an intracavernous carotid artery aneurysm. Eye 2006;20:1416-7.  Back to cited text no. 4
    
5.
Sanders SK, Kawasaki A, Purvin VA. Long-term prognosis in patients with vasculopathic sixth nerve palsy. Am J Ophthalmol 2002;134:81-4.  Back to cited text no. 5
    
6.
Gupta N, Michel MA, Poetker DM. Recurrent contralateral abducens nerve palsy in acute unilateral sphenoiditis. Am J Otolaryngol 2010;31:372-5.  Back to cited text no. 6
    
7.
Galbussera A, Tremolizzo L, Tagliabue E, Ceresa C, Cilia R, Ruffmann C, et al. Third cranial nerve palsy? Look for a sicca syndrome. J Neurol Sci 2007;253:88-9.  Back to cited text no. 7
    
8.
Vitali C, Moutsopoulos HM, Bombardieri S. The European Community Study Group on diagnostic criteria for Sjögren's syndrome. Sensitivity and specificity of tests for ocular and oral involvement in Sjögren's syndrome. Ann Rheum Dis 1994;53:637-47.  Back to cited text no. 8
    
9.
Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: A study of 82 patients. Medicine 2004;83:280-91.  Back to cited text no. 9
    
10.
Lui NL, See SJ, Thumboo J. A rare cause of reversible unilateral third nerve palsy. J Neurol Sci 2008;275:188-90.  Back to cited text no. 10
    
11.
Chu K, Kang DW, Song YW, Yoon BW. Trochlear nerve palsy in Sjögren's syndrome. J Neurol Sci 2000;177:157-9.  Back to cited text no. 11
    
12.
Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492-95.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
Print this article  Email this article
   
Online since 20th March '04
Published by Wolters Kluwer - Medknow