Trigeminal sensory neuropathy associated with systemic sclerosis: report of three Brazilian cases

Abstract

Systemic sclerosis (SSc) is a connective tissue disease manifest by fibrotic tissue changes, microvascular disease, and autoimmune abnormalities. The prevalence of different neurological manifestations in SSc has ranged from 0.8% 1 to 18.5% 2 according to the adopted criteria. Trigeminal sensory neuropathy (TSN) causes numbness in the mandibular or maxillary divisions of the nerve in about 2/3 of the cases, and in the distribution of all divisions in the remaining cases. The sensory abnormalities evolve slowly and usually spread contralaterally in an asymmetric pattern. The numbness may be accompanied by burning dysesthesia that is distinct from trigeminal neuralgia 3 . TSN is an infrequent complication of SSc. Although epidemiological studies are scarce, the prevalence of TSN associated with SSc in the largest series available was 4% 4 . A fairly diligent review of the literature revealed no previous report of TSN as a complication of SSc in Brazil. We present three cases of such association. CASES Case 1A 42-year-old male had presented progressive facial hypoesthesia and dysesthesia for the last three years and was diagnosed with the diffuse form of SSc two years ago. At his first neurological evaluation, he had sclerodermic fascies, sclerodactyly, Raynaud's phenomenon, bilateral facial and lingual hypoesthesia, left hemifacial dysesthesia, and an absent left corneal reflex. Nailfold capillaroscopy was performed and showed devascularization and ectasias. ANA was positive (1/640) and anti-RNP was also positive. Nerve conduction studies (NCS) revealed sensory myelinic polyneuropathy in all limbs and face. Blink reflex studies (BRS) demonstrated absent responses on both sides to left supraorbital nerve stimulation. Electromyography (EMG) was normal. He was on methotrexate and prednisone without improvement of the TSN. Gabapentin 800 mg daily and nortryptilin 100 mg daily were introduced with only a partial response at their maximum tolerable doses. Case 2A 47-year-old female had presented Raynaud' s phenomenon and arthritis in her hands for the last three years and was feeling progressive numbness in the inferior half of the face for the last two years, without any previous diagnosis. At her first neurological evaluation, she had sclerodermic fascies, sclerodactyly, hypoesthesia in the maxillary and mandibular divisions territory bilaterally. ANA was positive (1/80), an esophagogram showed an increased esophageal caliber and a thoracic tomography confirmed this finding and revealed signs of pulmonary fibrosis. All electrophysiological studies performed (NCS, BRS, EMG) were normal. She had never been on medication for TSN or SSc yet. Case 3A 50-year-old female had presented hypoesthesia only in the maxillary division of the right trigeminal nerve about three years before her first neurological evaluation. Six months later, she started presenting hypoesthesia in the mandibullary division, effort dyspnea, Raynaud' s phenomenon and myalgia. She was diagnosed with SSc in overlap with myositis 10 months before our firs...

Evaluation of dry eye signs and symptoms in patients with systemic sclerosis

“…Future studies should be carried out to evaluate corneal sensation in SSc. Given the fact that SSc is associated with peripheral neuropathy [36,37], decreased corneal sensation may be a manifestation of the systemic disease itself, regardless of the severity of dry eye.…”

The spectrum of orofacial manifestations in systemic sclerosis: a challenging management

“…However, evidence of primary nervous system involvement in SSc is currently increasing (Amaral et al, 2013). Moreover, neurologic manifestations may be underdiagnosed as they can be easily confused with symptoms related to skin hardening (Ribeiro et al, 2009).…”

“…Indeed, the 73 TN associated with SSc that have been documented in the literature account for more than 15% of all reported peripheral nervous system events (Amaral et al, 2013). However, the literature addressing TN during SSc principally consists of case series and case reports (Teasdall et al, 1980;Jimenez-Moreno et al, 1998;Fischoff and Sirois, 2000;Mohyuddin et al, 2009;Ribeiro et al, 2009;Vincent et al, 2009), and the link between the two conditions has only been investigated in a small number of studies (Farrell and Medsger, 1982;Hagen et al, 1990;Amaral et al, 2013). In their retrospective analysis on 442 SSc patients, Farrel and Medsger described an association with TN in 4% of cases (16 patients) (Farrell and Medsger, 1982).…”

“…Moreover, autoimmune response against basement membrane antigens could account for vascular injury, leading to peripheral nerves dysfunction (Jimenez-Moreno et al, 1998;Denton and Ong, 2004). Jimenez-Moreno et al propose that TN could be regarded as an entrapment neuropathy due to the inflammatory response with edema (Jimenez-Moreno et al, 1998;Ribeiro et al, 2009). Local compressive nerve damage should also be considered in the pathogenesis of some TN cases as suggested by Fischoff et al who described a case of severe mandibular resorption resulting in a painful neuropathy due to the compression of the inferior alveolar nerve (Fischoff and Sirois, 2000).…”

Maladies systémiques et vascularites

“…Une hypothyroïdie, une névralgie du trijumeau ou un syndrome sec ont également rarement été rapportés [15,204,221,273,335,336] . Il s'agit essentiellement d'un reflux gastro-oesophagien, d'une dysphagie ou de divers symptômes secondaires à l'atonie intestinale .…”