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

Can Ebru Bekircan-Kurt; Çağrı Temuçin; Şule Apraş Bilgen; Sevim Erdem-Ozdamar

doi:10.4103/NSN.NSN_25_20

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

Can Ebru Bekircan-Kurt¹, Çağrı Temuçin¹, Şule Apraş Bilgen², Sevim Erdem-Ozdamar¹
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 Submission	11-Sep-2019
Date of Decision	08-Jan-2020
Date of Acceptance	30-Mar-2020
Date of Web Publication	16-Sep-2020

Correspondence Address:
Can Ebru Bekircan-Kurt
Department of Neurology, Hacettepe University School of Medicine, Sıhhiye, Ankara
Turkey

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/NSN.NSN_25_20

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 2023 Jun 9];37:148-51. Available from: http://www.nsnjournal.org/text.asp?2020/37/3/148/295177

Introduction

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

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

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.

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