Trigeminal trophic syndrome and neurotrophic keratopathy following stroke

Ipek Gungor-Dogan; Sezgi Sarıkaya-Solak

doi:10.4103/NSN.NSN_96_20

CASE REPORT

Year : 2020 | Volume : 37 | Issue : 4 | Page : 231-233

Trigeminal trophic syndrome and neurotrophic keratopathy following stroke

Ipek Gungor-Dogan¹, Sezgi Sarıkaya-Solak²
¹ Department of Neurology, Trakya University Medical Faculty, Edirne, Turkey
² Department of Dermatology, Trakya University Medical Faculty, Edirne, Turkey

Date of Submission	25-Jun-2020
Date of Decision	14-Aug-2020
Date of Acceptance	04-Oct-2020
Date of Web Publication	29-Dec-2020

Correspondence Address:
Ipek Gungor-Dogan
Department of Neurology, Trakya University, Balkan Campus, Edirne (22000)
Turkey

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/NSN.NSN_96_20

Abstract

Neurotrophic ulceration of the face and cornea are rare complications of trigeminal nerve damage. Their rarity makes them potentially underreported and misdiagnosed conditions. The neurogenic basis and self-manipulating nature of facial ulceration also require a rapid diagnosis to provide a rare syndrome-specific approach. Pain management for paresthesias and educating patients about their self-manipulating behaviors are key management strategies for successful treatment and improved outcomes under the multidisciplinary approach.

Keywords: Carbamazepine, neurotrophic keratopathy, trigeminal nerve, trigeminal trophic syndrome, Wallenberg syndrome

How to cite this article:
Gungor-Dogan I, Sarıkaya-Solak S. Trigeminal trophic syndrome and neurotrophic keratopathy following stroke. Neurol Sci Neurophysiol 2020;37:231-3

How to cite this URL:
Gungor-Dogan I, Sarıkaya-Solak S. Trigeminal trophic syndrome and neurotrophic keratopathy following stroke. Neurol Sci Neurophysiol [serial online] 2020 [cited 2023 May 29];37:231-3. Available from: http://www.nsnjournal.org/text.asp?2020/37/4/231/305394

Introduction

Trigeminal trophic syndrome (TTS) is a rare condition characterized by self-manipulation of the facial skin that leads to ulceration which occurs after an injury to the trigeminal nerve or its central sensory connection.^[1] In 1901, Wallenberg first described TTS as cutaneous ulceration in the trigeminal dermatome.^[2] Since then, the syndrome has appeared in the literature with different names originating from different etiologies such as surgical interventions aiming to treat trigeminal neuralgia, or cerebrovascular disease (especially Wallenberg syndrome; WS), or less commonly trauma, infections, and neoplasms.^[1],[3],[4]

Neurotrophic keratitis (NK) is a degenerative corneal disease characterized by corneal thinning, ulceration, and perforation resulting from ocular, central, and systemic diseases that impair trigeminal nerve innervation at any level.^[5],[6] NK secondary to WS is not frequently reported.

Here, we report the case of a 48-year-old man who presented with both cutaneous manifestations of TTS and NK secondary to WS and we aim to shed light on the complex nature of trigeminal system disorders.

Case Report

A 48-year-old man presented with a 3-month history of painless progressive ulceration on the right side of his face accompanied by vision loss in the right eye. His medical history was remarkable for posterior circulation stroke 3 months ago. The patient stated that the facial ulceration and vision loss developed approximately 1 month after the stroke. His chief complaints were itching, tickling, and crawling sensations on the right side of his face. The patient and his family reported repeated scratching and rubbing of the affected area. He also suffered from ongoing gait imbalance and numbness in the left upper and lower limbs following stroke. As he was a heavy smoker and drinker for years, he also had newly diagnosed hypertension.

Physical examination revealed three nonhealing ulcerations with hemorrhagic crusts and irregular borders located on the right eyelid, inferomedial aspect of the right eye, and right ala nasi with sparing of the nasal tip [Figure 1]. The ophthalmologist diagnosed neurotrophic keratopathy as the cause of corneal opacification and vision loss in the right eye. There was right-sided ptosis on neurological examination. There was only light perception in the right eye, and the corneal reflex was also absent. There was a loss of pain and temperature sensation on the right side of his face and contralateral body. Right-sided limb ataxia and a mild degree of truncal ataxia were also noted.

Figure 1: Picture of the non.healing ulcerations located on the right eyelid, inferomedial aspect of the right eye, and right ala Nasi with sparing of the nasal tip

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The results of laboratory tests, including a complete blood count and biochemistry, were all normal. In the earlier diffusion-weighted magnetic resonance imaging (MRI) of the patient, there were acute ischemic regions on the right side of the medulla and cerebellum [Figure 2]a. Later MRI revealed an increased T2-weighted signal on the right side of the medulla corresponding to the anatomic location of the spinal trigeminal nucleus and tract and also right-sided atrophy of the medulla secondary to infarction [Figure 2]b. The diagnosis of occlusion of the right vertebral artery was made with computed tomography angiography [Figure 2]c. Skin biopsy from one of the ulcerations was performed to rule out other causes of skin ulcerations including cutaneous malignancies, vasculitis, and infections.

Figure 2: Diffusion-weighted magnetic resonance imaging of the patient is shows acute ischemic regions on the right side of the medulla and cerebellum (a). The later magnetic resonance imaging is revealing an increased T2-weighted signal on the right side of the medulla corresponding to the anatomic location of the spinal trigeminal nucleus and tract and also right-sided atrophy of the medulla secondary to infarction (b). Computed tomography angiography of the patient is showing the occlusion of the right vertebral artery (c)

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Based on all findings which were temporally correlated with the patient's stroke history, we diagnosed him with TTS and NK secondary to WS. The patient was treated with carbamazepine (400 mg daily), which helped him to reduce self-manipulating behaviors. Furthermore, he was prescribed topical treatment with occlusive dressings. After 1 month, the facial ulcerations had reduced in size with these medications.

Discussion

TTS is a rare but important cause of facial ulceration, mostly secondary to surgical interventions aiming to treat trigeminal neuralgia or associated with cerebrovascular disorders. The syndrome is related to sensory abnormalities such as anesthesia and paresthesia that provoke self-manipulation of facial skin.^[1] The typical picture of facial trauma is unilateral crescentic ulceration of the ala nasi in which the tip of the nose is mostly spared. In severe cases, the ulceration can spread onto the cheek and upper lip or other locations such as the forehead, scalp, preauricular skin, palate, tongue, and eyelid.^[1],[2],[4] As seen in our patient, most of the patients with TTS complain about paresthesia, and most of them and their family members report self-manipulation of the affected areas.^[2],[4] It is believed that paresthesia provokes these behaviors. However, the big question requiring an answer is why only some of the patients with the same risk factors experience TTS and the others do not. According to Weintraub et al ., some people are more prone to self-manipulating behaviors.^[4]

There is no specific diagnostic algorithm for TTS, but it can be highly recognizable in a patient with a history of trigeminal nerve damage at any level. As the neurotrophic ulceration can occur weeks or years after nerve injury, detailed medical history needs to be obtained by the clinician. Furthermore, the triad of trigeminal anesthesia, paresthesia, and ulceration of the ala nasi is almost pathognomonic.^[3],[4] Treatment of TTS is also challenging. Minimizing the self-induced trauma is the key management strategy, in addition to topical skin treatments. This requires medical treatment for paresthesia provoking self-manipulating behaviors and educating patients about these behaviors. Carbamazepine was shown to be effective in treating paresthesia.^[7]

Neurotrophic ulceration of the cornea secondary to WS is also a very rare cause of corneal injury. There are only a few reports in the ophthalmic literature describing NK related to WS.^[8],[9] However, the rarity of corneal manifestations of WS may be related to insufficient ocular investigation of TTS cases which mostly feature in dermatology journals. Sawada et al . drew attention to this point in their review by reporting 18% corneal involvement among sixty patients with a diagnosis of TTS and highlighted the necessity for ophthalmologic consultation in susceptible individuals.^[10] Reduced or absent corneal innervation could be insidious and progressive in the affected eye and may result in corneal perforation with permanent scarring and vision loss, as occurred in our patient.^[5]

In conclusion, in a patient with a history of trigeminal nerve damage, clinicians should be aware of TTS and NK to avoid permanent facial scarring and vision loss. Pain management for paresthesia and educating patients about their self-manipulating behaviors are key management strategies for facial ulceration, in addition to topical skin treatments, but ophthalmologic consultation should also be considered in susceptible individuals to assess corneal involvement.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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