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 Table of Contents  
CASE REPORT AND LITERATURE REVIEW
Year : 2020  |  Volume : 37  |  Issue : 3  |  Page : 148-151

Trigeminal sensory-motor neuropathy in a patient with mixed connective tissue disease and review of the literature


1 Department of Neurology, Hacettepe University School of Medicine, Sıhhiye, Ankara, Turkey
2 Department of Rheumatology, Hacettepe University School of Medicine, Sıhhiye, Ankara, Turkey

Date of Submission11-Sep-2019
Date of Decision08-Jan-2020
Date of Acceptance30-Mar-2020
Date of Web Publication16-Sep-2020

Correspondence Address:
Can Ebru Bekircan-Kurt
Department of Neurology, Hacettepe University School of Medicine, Sıhhiye, Ankara
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/NSN.NSN_25_20

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  Abstract 


Trigeminal neuropathy is an infrequent condition, usually limited to the sensorial component of the nerve, and it is one of the most common neurologic manifestations of mixed connective tissue disease (MCTD). However, to the best of our knowledge, no cases have been reported with MCTD presenting as trigeminal sensory and motor neuropathy. Herein, we report a 28-year-old female with unilateral trigeminal sensory–motor neuropathy who presented with numbness over the distribution of the right trigeminal region and right masseter atrophy. We also reviewed the literature for trigeminal neuropathy associated with MCTD.

Keywords: Blink reflex, mixed connective tissue disease, trigeminal sensory–motor neuropathy


How to cite this article:
Bekircan-Kurt CE, Temuçin &, Bilgen &A, Erdem-Ozdamar S. Trigeminal sensory-motor neuropathy in a patient with mixed connective tissue disease and review of the literature. Neurol Sci Neurophysiol 2020;37:148-51

How to cite this URL:
Bekircan-Kurt CE, Temuçin &, Bilgen &A, Erdem-Ozdamar S. Trigeminal sensory-motor neuropathy in a patient with mixed connective tissue disease and review of the literature. Neurol Sci Neurophysiol [serial online] 2020 [cited 2020 Sep 28];37:148-51. Available from: http://www.nsnjournal.org/text.asp?2020/37/3/148/295177




  Introduction Top


Trigeminal neuropathy is an infrequent condition, usually limited to the sensorial component of the nerve, manifesting as numbness in the region innervated by the affected branch of the nerve.[1] It is usually caused by trauma, tumor infiltration, and collagen diseases.

Sharp et al. described mixed connective tissue disease (MCTD) in 1972, as a mixture of systemic lupus erythematosus (SLE), progressive systemic sclerosis (SSc), and polymyositis with high titers of anti-U1RNP.[2] Later, rheumatoid arthritis (RA) was added to the definition of MCTD.[3],[4] There has been some controversy as to whether MCTD is truly a distinct disease because it shares some symptoms with SLE, RA, SSc, PM, and RA. However, the presence of a high titer of U1RNP antibodies (antibodies to ribonuclease -sensitive extractable nuclear antigen [ENA]) and the association with HLA-DR2 and HLA-DR4 suggest that MCTD is a distinct entity.[4]

“Pure” trigeminal sensory neuropathy is one of the most common neurologic manifestations of MCTD.[5],[6] However, to the best of our knowledge, there are no case reports regarding MCTD presenting as trigeminal sensory and motor neuropathy. Herein, we report a patient with unilateral trigeminal sensory–motor neuropathy diagnosed who was diagnosed as having MCTD. In addition, we reviewed the literature of trigeminal neuropathy associated with MCTD.


  Case Report Top


A 28-year-old female referred to our Neurology Department because of numbness in the nose, mouth, tongue, and chin on the right side for 5 months. In addition, she reported paroxysmal tingling and pins and needles sensations in the same region. Her past medical history was significant for iron-deficiency anemia and Raynaud's phenomenon. Detailed history and physical examination were significant for paleness of fingers on exposure to cold ambient environments and swollen digits for 1 year. A neurologic examination revealed hypoesthesia in the cutaneous territory corresponding to the maxillary and mandibular branches of the right trigeminal nerve and absence of the right corneal reflex. In addition, atrophy of the right masseter muscle was observed.

After supraorbital nerve stimulation, right and left orbicularis oculi muscle responses were recorded simultaneously. With right supraorbital nerve stimulation, latency of ipsilateral R1 and R2 and contralateral R2 responses was prolonged, and their amplitudes were low compared with the left supraorbital nerve stimulation [Figure 1] and [Figure 2]. The blink reflex study located the lesion to the right trigeminal nerve. Fluorescent antinuclear antibody (ANA) was positive with a titer of 1/1000 speckled pattern. Besides high level of rheumatoid factor (RF, 1170 IU/mL, normal range is 0-20 IU/mL) and positive anti-RNP with a high titer of extractable nuclear antigen (ENA) were depicted during the laboratory workup. The other autoantibody testing was negative such as anti-dsDNA, anti-Scl-70, anti-centromere, anti-Jo-1, and anti-Ro/La. Gadolinium -enhanced cranial magnetic resonance imaging (MRI) was normal, with special attention given to the trigeminal nerve and brain stem with thin sections.
Figure 1: The initial blink reflex study showed prolonged latency of ipsilateral R1 and R2 and contralateral R2 responses after right stimulation

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Figure 2: The initial blink reflex study showed prolonged latency of ipsilateral R1 and R2 and contralateral R2 responses after left stimulation

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The patient was referred to the rheumatology department, assuming that she might have connective tissue disease. Capillaroscopy showed a hemorrhagic region with giant capillaries. Esophagography depicted a minimal reduction in esophageal motility in the lying position. Echocardiography depicted minimal mitral and tricuspid insufficiency.

Based on Raynaud's phenomenon, swollen fingers, esophageal dysmotility, and positive serology, she was diagnosed with MCTD according to the Kasukawa classification criteria.[7] Hydroxychloroquine sulfate 400 mg/day, acetylsalicylic acid 100 mg/day, nifedipine 30 mg/day, 10 mg/day prednisolone, and lansoprazole were initiated. During her follow-up, the prednisolone dose was tapered down to 4 mg every other day. Her positive sensory symptoms disappeared after 1 year, however, sensory loss persisted. Abnormal prolongation of latencies and drop of amplitudes at right stimulation that was observed at initial blink reflex study disappeared [Figure 3] and [Figure 4].
Figure 3: The blink reflex study after treatment was normal (right stimulation)

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Figure 4: The blink reflex study after treatment was normal (left stimulation)

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Clinical and research consequences

The trigeminal nerve is responsible for the sensation of the face and innervation of the masticatory muscles. The trigeminal neuropathy refers to the dysfunction of the sensorial component of the nerve characterized by skin and mucosal numbness in the region of the affected branches. Malignancy, trauma, connective tissue diseases, and infections may cause the trigeminal neuropathy, which presents with loss of sensation in the region of the nerve. In these patients, trigeminal motor functions and corneal reflex are generally preserved.[1] Among the connective tissue diseases, MCTD may first manifest acute, sensory-type, and anatomically variable motor-sparing trigeminal neuropathy.[7] Shortly after the description of MCTD by Sharp et al., several patients with MCTD who developed trigeminal neuropathy were published [Table 1].[4],[8],[9],[10],[11],[12] The patients were generally women, and all reported isolated sensorial symptoms and signs, usually impaired cornea reflex, with or without trigeminal neuralgia. Moreover, in a review of 81 patients with trigeminal sensory neuropathy, 21 patients (26%) were diagnosed as having MCTD.[11] Although trigeminal nerve involvement can be as frequent as 26% in MCTD, the pathogenesis of trigeminal neuropathy in MCTD has not been clarified, and there are no postmortem studies. The formation of immune complexes in the nerve or in the sensory root is believed to cause the neuropathy.[1] Previous publications reported that sensorial symptoms did not benefit from steroid, intravenous immunoglobulin, or immunosuppressive treatments such as azathioprine and hydroxychloroquine.[5],[10],[11],[14],[15],[16]
Table 1: Trigeminal neuropathy associated with mixed connective tissue disease in the English literature

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  Conclusion Top


In the present report, trigeminal neuropathy manifested with both motor and sensorial signs and symptoms. Although the majority of previous reports showed trigeminal deficits of only the sensory branches, the masseter atrophy and the blink reflex of our patient proved a lesion of motor and sensory branches of the trigeminal nerve. Cranial MRI revealed no pathology of the brainstem. As suggested previously, the patient was investigated for underlying systemic etiologies and ANA, ENA-RNP, and RF positivity was demonstrated, and she was diagnosed as having MCTD. Although corticosteroid treatment is considered to be ineffective for the sensory symptoms in the majority of the patients with MCTD in the literature, our patient benefited from the corticosteroid treatment.[1],[4],[8],[9],[10],[13],[14] Trigeminal neuropathy may be the first presentation of MCTD; therefore, it is important to exclude connective tissue diseases as a cause of trigeminal neuropathy because this may have therapeutic implications.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Peñarrocha M, Cervelló MA, Martí E, Bagán JV. Trigeminal neuropathy. Oral Dis 2007;13:141-50.  Back to cited text no. 1
    
2.
Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR. Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med 1972;52:148-59.  Back to cited text no. 2
    
3.
Gendi NS, Welsh KI, Van Venrooij WJ, Vancheeswaran R, Gilroy J, Black CM. HLA type as a predictor of mixed connective tissue disease differentiation. Ten-year clinical and immunogenetic followup of 46 patients. Arthritis Rheum 1995;38:259-66.  Back to cited text no. 3
    
4.
Cappelli S, Randone SB, Martinovic D, Tamas MM, Pasalic K, Allanore Y, et al. “To be or not to be,” ten years after: Evidence for mixed connective tissue disease as a distinct entity. Semin Arthritis Rheumatism 2012;41:589-98.  Back to cited text no. 4
    
5.
Bennett RM, Bong DM, Spargo BH. Neuropsychiatric problems in mixed connective tissue disease. Am J Med 1978;65:955-62.  Back to cited text no. 5
    
6.
Ashworth B, Tait GB. Trigeminal neuropathy in connective tissue disease. Neurology 1971;21:609-14.  Back to cited text no. 6
    
7.
Kasukawa R. Mixed connective tissue disease. Intern Med 1999;38:386-93.  Back to cited text no. 7
    
8.
Dent TH, Johnson VW. Aspects of mixed connective tissue disease: A review. J R Soc Med 1992;85:744-6.  Back to cited text no. 8
    
9.
Edmondstone WM, Shepherd TH, Price DK, Gooddy WW. Mixed connective tissue disease presenting as trigeminal neuropathy. Postgrad Med J 1982;58:237-8.  Back to cited text no. 9
    
10.
Searles RP, Mladinich EK, Messner RP. Isolated trigeminal sensory neuropathy: Early manifestation of mixed connective tissue disease. Neurology 1978;28:1286-9.  Back to cited text no. 10
    
11.
Vincent FM, Van Houzen RN. Trigeminal sensory neuropathy and bilateral carpal tunnel syndrome: The initial manifestation of mixed connective tissue disease. J Neurol Neurosurg Psychiatry 1980;43:458-60.  Back to cited text no. 11
    
12.
Hagen NA, Stevens JC, Michet CJ Jr. Trigeminal sensory neuropathy associated with connective tissue diseases. Neurology 1990;40:891-6.  Back to cited text no. 12
    
13.
Lecky BR, Hughes RA, Murray NM. Trigeminal sensory neuropathy. A study of 22 cases. Brain 1987;110 (Pt 6):1463-85.  Back to cited text no. 13
    
14.
Hojaili B, Barland P. Trigeminal neuralgia as the first manifestation of mixed connective tissue disorder. J Clin Rheumatol 2006;12:145-7.  Back to cited text no. 14
    
15.
Hamoir B, Giroux M, Outteryck O, Launay D, Vermersch P. Mixed connective tissue disease presenting as trigeminal neuropathy. Acta Neurol Belg 2014;114:245-6.  Back to cited text no. 15
    
16.
Danve A, Zabad R, Erickson A. Intravenous immunoglobulin for mixed connective tissue disease presenting with bilateral trigeminal neuropathy. Am J Ther 2018;25:e383-5.  Back to cited text no. 16
    


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