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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 23  |  Issue : 1  |  Page : 59-62

A rare case of bilateral trigeminal palsy presenting as a complication of Sjogren syndrome


1 A&E, University Hospital of South Manchester, Manchester, UK
2 Department of Neurology, Shifa International Hospital, Islamabad, Pakistan
3 Department of Pulmonology, University Hospital of South Manchester, Manchester, UK

Date of Submission17-Nov-2016
Date of Acceptance21-Dec-2016
Date of Web Publication29-May-2017

Correspondence Address:
Faleha Zafar
Department of Neurology, Shifa International Hospital, H-8/4, Islamabad, 44000
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kamj.kamj_57_16

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  Abstract 

Sjogren syndrome is a chronic disorder affecting the exocrine glands of the body, mostly lacrimal and salivary glands, resulting in sicca symptoms. Diagnosis is aided by the presence of anti-Ro and anti-La antibodies and gland biopsy showing lymphocytic infiltration. Bilateral loss of facial sensation in Sjogren syndrome (SS) has not been reported before, although unilateral trigeminal palsy as a presentation of SS has been reported in literature. A 25-year-old lady presented in the neurology clinic with complete facial numbness and vision loss for 1 month. The vision worsened, leading to complete blindness. She had dry mouth and dry eyes for the past 3 months. She was diagnosed with SS using American–European consensus Sjogren criteria. Electrophysiological blink reflex testing confirmed complete trigeminal nerve palsy. SS was treated with prednisolone, 1 mg/kg/day, with a poor response. Symptomatic treatment was given for sicca symptoms, which improved significantly. Corneal transplantation was done for visual loss, but limited improvement was seen.

Keywords: blink reflex, complete blindness, corneal transplant, Sjogren syndrome, trigeminal nerve palsy


How to cite this article:
Ilyas A, Malik WT, Abrar A, Zafar F. A rare case of bilateral trigeminal palsy presenting as a complication of Sjogren syndrome. Kasr Al Ainy Med J 2017;23:59-62

How to cite this URL:
Ilyas A, Malik WT, Abrar A, Zafar F. A rare case of bilateral trigeminal palsy presenting as a complication of Sjogren syndrome. Kasr Al Ainy Med J [serial online] 2017 [cited 2017 Aug 21];23:59-62. Available from: http://www.kamj.eg.net/text.asp?2017/23/1/59/207193


  Introduction Top


Sjogren syndrome (SS), first described by M.D. Henry Sjogren, is a rare endocrine disorder characterized by chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function [1],[2],[3]. SS can be classified as primary disease or secondary to other connective tissue disorders. The disease mostly affects middle-aged individuals and women (female : male ratio is 9 : 1) [4].

At least one-third of patients with SS present with systemic manifestations including neurological, renal, pulmonary, articular, and gastrointestinal [5]. Neuro-Sjogren is a rare disease that manifests with myelitis, hypophysitis, devics disease, autonomic, and peripheral neuropathy. We are presenting a case of complete facial sensory loss, which is secondary to bilateral trigeminal neuropathy that was later on diagnosed as having SS. This patient was having complete blindness due to neurotrophic corneal ulceration.

This case is rare; to the best of our knowledge, there has been no case of bilateral trigeminal palsy presenting as a complication of SS reported so far in PubMed. However, unilateral trigeminal palsy as a presentation of SS has been reported in literature. Hull et al. [1] in their article ‘Sjögren’s syndrome presenting as a severe sensory neuropathy including involvement of the trigeminal nerve’ described the involvement of trigeminal nerve in SS; ‘Sjogren’s syndrome-associated neuropathy’ was published by Koike et al.[2], and there are a number of articles in literature regarding trigeminal neuropathy and SS association, although, to best of our knowledge, in all these articles involvement of trigeminal nerve was unilateral.


  Case history Top


A 25-year-old woman, with no known comorbidities, presented to neurology OPD with complete loss of vision for the past 2–3 days. She was in usual state of health 1 month back when she developed sudden, painless, bilateral loss of facial sensations and blurring of vision. The vision had been progressively worsening since, and for the past 2–3 days she could not see anything at all.

On further questioning, she said that for the past 3 months she had noticed gritty sensation and excessive watering of both eyes along with dryness of mouth. These symptoms had been worsening progressively since then. However, she did not seek any medical advice for these symptoms, nor used any medications, as it was not interfering with her daily life activity.

She denied having any similar symptoms before. She had no associated complaints of diplopia, eye pain, and redness of eye, dysphagia, hearing difficulty, headache, fever, rash, or focal weakness. There was no history of trauma before these symptoms. Her past medical and surgical history was insignificant; there was no previous hospitalization or recent travel history. She is unmarried, sexually inactive, and has no drug addictions. She has no known drug allergies and is not on any long-term medications. Her family history was insignificant for any long-term illness.

On physical examination, she appeared to be a young lady, alert, anxious, oriented in time, place and person, not in acute distress. She was vitally stable. Her neurological examination revealed bilateral loss of sensation on ophthalmic, maxillary, and mandibular division of trigeminal nerve. There was no facial asymmetry noticed; all other cranial nerve examinations were normal. She had normal tone, power, and bulk in all four limbs. Superficial and deep reflexes were intact and cerebellar signs were normal. Plantars were bilaterally down-going and gait was normal.

On eye examination, there was no conjunctival redness, and pupils were bilaterally round, regular, and reactive to light. However, corneal opacities were seen in both eyes. Corneal reflex was absent. On fundoscopy, red reflex was absent and hence fundus could not be visualized.

The following investigations were ordered. Complete blood picture, electrolytes, renal function tests, liver function tests, and thyroid profile were all within normal limit. Erythrocyte sedimentation rate was slightly elevated (39 mm/h). A complete autoimmune profile, as shown in [Table 1] below, was advised; it showed raised anti-SSA (Ro) antibodies.
Table 1 The results of the autoimmune screening

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MRI brain with contrast was normal. Electrophysiological blink reflex testing was done, which showed complete trigeminal loss on both sides of the face; refer to [Table 2] below. Facial nerve was normal.
Table 2 Direct facial nerve stimulation and blink reflex study

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Ophthalmology consultation was sought. They diagnosed her with neurotrophic corneal ulceration secondary to trigeminal nerve palsy. The patient was counselled regarding corneal transplantation. After consent, corneal transplantation was done, following which only mild improvement in her vision was seen.

She was also referred to a rheumatologist for opinion. She was diagnosed as having SS and started on symptomatic treatment. She was prescribed tear drops to help her with gritty eye sensation and for dryness of mouth she was started on oral secretagogue, both of which she found very helpful. She was also prescribed steroids (prednisolone×1 mg/kg/day×3 months), but as no improvement was seen it was tapered off.


  Discussion Top


SS is a rare chronic autoimmune disorder characterized by diffuse lymphocytic infiltration of exocrine glands. Autoimmune exocrinopathy of salivary glands leads to dryness of mouth and that of lacrimal gland leads to dryness of eyes, both of which are chief symptoms of the disease [3].

American–European consensus Sjogren’s criteria is the most widely used criteria for diagnosis of SS [6]. [Table 3] below shows the diagnostic criteria.
Table 3 The American–European consensus criteria for the diagnosis of Sjogren syndrome

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Our patient was diagnosed with Sjogren disease according to the criteria mentioned in [Table 3]. She had ocular and oral symptoms for a few months, Schirmer’s test was positive, and both anti-SSA and anti-SSA antibodies were positive.

SS can be classified as primary disease or secondary to other connective tissue disorders. The disease mostly affects middle-aged individuals and women (female : male ratio is 9 : 1) [4].

At least one-third of the patients with SS present with systemic manifestations including neurological, renal, pulmonary, articular, and gastrointestinal [5]. Neurological manifestations can be categorized into two: central nervous system (CNS) and peripheral nervous system. CNS involvement varies from headache to aseptic meningitis. Peripheral nervous system manifestations include sensory polyneuropathy, polyradiculopathies, autonomic neuropathies, and cranial nerve palsy. The mechanism behind CNS involvement in the disease remains unknown [6]. To the best of our knowledge, there has been no case of bilateral trigeminal palsy presenting as a complication of SS reported so far.

SS is associated with severe functional impairment and negatively affects the quality of life. Treatment comprises two parts: symptomatic relief and improvement in quality of life. Symptomatic relief includes use of lubricant eye drops and gustatory stimulating agents for dryness of eye and mouth, respectively. [7]. Studies have shown that rituximab is effective in improving systemic manifestations and quality of life in patients with primary SS [8]. High dose of steroids along with immunosuppressant is recommended in patients with CNS involvement. Managing patients with SS requires a multidisciplinary approach involving many specialities depending on systemic complications [9].

Acknowledgements

Dr. Farrukh Mateen was kind enough to get the consent from the patient, as the patient returned to her native country Afghanistan. Dr. Saadia Farooq performed the corneal transplant of the patient and helped us throughout the process with her expertise. Dr. Tahir Hashmi was looking after the rheumatological aspects, and he was quite helpful. The authors thank them all.

Dr. Ali Ilyas: substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; drafting the article and revising it critically for important intellectual content; and final approval of the version to be published. Dr. Wasim Tariq Malik: concept, drafting the article, and revising it critically for important intellectual content, and final approval of the version to be published. Dr. Anam Abrar: acquisition of data, or analysis and interpretation of data drafting the article. Dr. Faleha Zafar: corresponding author, drafting the article, and revising it critically for important intellectual content, and final approval of the version to be published.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hull RG, Morgan SH, Harding AE, Hughes GR. Sjogren’s syndrome presenting as a severe sensory neuropathy including involvement of the trigeminal nerve. Br J Rheumatol 1984; 23:301–303.  Back to cited text no. 1
[PUBMED]    
2.
Koike H, Sobue G. Sjogren’s syndrome-associated neuropathy [Article in Japanese]. Brain Nerve. 2013; 65:1333–1342.  Back to cited text no. 2
    
3.
Ngo DY, Thomson WM, Nolan A, Ferguson S. The lived experience of Sjogren’s syndrome. BMC Oral Health 2016; 16:7.  Back to cited text no. 3
    
4.
Ma D, Lu H, Qu Y, Wang S, Ying Y, Xiao W. Primary Sjogren’s syndrome accompanied by pleural effusion: a case report and literature review. Int J Clin Exp Pathol 2015; 8:15322–15327.  Back to cited text no. 4
    
5.
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE et al. Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002; 61:554–558.  Back to cited text no. 5
    
6.
Horai Y, Nishino A, Nakashima Y, Suzuki T, Okada A, Kawashiri SY et al. Case of Sjogren’s syndrome presenting as trigeminal nerve palsy. Jpn J Clin Immunol 2012; 35:199–202.  Back to cited text no. 6
    
7.
Valim V, Trevisani VF, Pasoto SG, Serrano EV, Ribeiro SL, Fidelix TS et al. Recommendations for the treatment of Sjogren’s syndrome. Rev Bras Reumatol 2015; 55:446–457.  Back to cited text no. 7
    
8.
Meijer JM, Meiners PM, Vissink A, Spijkervet FK, Abdulahad W, Kamminga N et al. Effectiveness of rituximab treatment in primary Sjogren’s syndrome: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum 2010; 62:960–968.  Back to cited text no. 8
    
9.
Danieli MG, Pettinari L, Morariu R, Monteforte F, Logullo F. Intravenous immunoglobulin and mycophenolate mofetil for long-standing sensory neuronopathy in Sjogren’s syndrome. Case Rep Immunol 2012; 2012:186320.  Back to cited text no. 9
    



 
 
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  [Table 1], [Table 2], [Table 3]



 

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