Bilateral corneal perforation caused by neurotrophic keratopathy associated with leprosy: a case report.

BACKGROUND: Neurotrophic keratopathy (NK) is a rare degenerative corneal disease caused by damage to the trigeminal nerve. We hereby describe a severe case with bilateral corneal perforation due to leprosy (Hansen's disease)-associated NK. CASE PRESENTATION: An 89-year-old man with a history of leprosy treated 40 years previously in our sanatorium developed bilateral corneal perforation due to NK. He had a history of bilateral persistent epithelial defects and bacterial keratitis. Although epithelialization was obtained with the use of autologous serum eye drops, progressive corneal thinning concomitant with stromalysis led to bilateral perforation. Over one month treatment with topical antibiotics, anti-inflammatory and lubricants resulted in healing of the epithelial defects and corneal perforations. A Cochet-Bonnet esthesiometer demonstrated a total absence of corneal sensation in both eyes. CONCLUSIONS: The present case indicated the irreversible nerve damage due to leprosy that had been cured 23 years ago, which can progress over the years and cause bilateral corneal perforations.

Blink Reflex in Neurotrophic Keratopathy: An Electrophysiological Evaluation.

PURPOSE: Neurotrophic keratitis (NK) is a rare condition which may result in visual loss. This case review investigates if there may be an association between NK and the blink reflex in the absence of facial nerve palsy and lagophthalmos. METHODS: This is a retrospective case review of 5 patients with trigeminal nerve damage referred to the oculoplastic department with suspected anesthetic corneae. Information on etiology, symptoms, duration, associated medical conditions, medications, examination findings including Mackie stage of keratopathy, management of keratopathy, and blink electrophysiology results was obtained. RESULTS: All 5 patients demonstrated absence of corneal sensation. All patients had preserved facial nerve function with no evidence of lagophthalmos. Keratopathy ranged from Mackie stage 0-2. Management ranged from ocular lubricants to Botulinum-toxin-induced ptosis. Blink studies demonstrated reduction in amplitude as well as increased latency in 2 patients, conferring reduced blink strength. Two patients demonstrated an absent blink reflex on the affected side. One patient had blink latency within the normative range; this patient recovered corneal sensation and was discharged. CONCLUSIONS: Our finding of reduced amplitude in blink studies offers both a factor in pathogenesis of NK and a potential therapeutic target. Additionally, blink studies may provide prognostic information for recovery and therefore guide management. We suggest performing blink electrophysiology in patients with trigeminal nerve damage to assess nerve function.

Neurotrophic Keratitis in a Pediatric Patient With Goldenhar Syndrome and Trigeminal Aplasia Successfully Treated by Corneal Neurotization.

Herein, the authors report an unusual case of a 6-year-old boy with right-sided Goldenhar syndrome and trigeminal nerve aplasia who developed neurotrophic keratopathy (NK). Despite the use of therapeutic contact lenses and multiple temporary tarsorrhaphy, NK worsened showing a central corneal scar, neovascularization, and significant stromal thinning, with risk of corneal perforation. Cochet-Bonnet esthesiometry revealed complete corneal anesthesia. To minimize additional corneal complications, the patient underwent indirect corneal neurotization by a sural nerve autograft anastomosed to the contralateral supratrochlear nerve. At 24-month follow up, no epithelial defects, complications, or recurrence were observed. Significant improvements in corneal sensitivity with esthesiometry score of 20 mm and reflex blinking were achieved. This case highlights corneal anesthesia should be suspected among Goldenhar syndrome ophthalmologic abnormalities and monitored before corneal changes become irreversible. Since corneal neurotization can successfully improve corneal sensation, it could be considered as an early therapeutic option to avoid refractory NK.

Trigeminal trophic syndrome: an important simulator of discoid cutaneous lupus erythematosus - a case series.

Trigeminal trophic syndrome occurs secondary to trigeminal nerve injury, leading to anaesthesia and paraesthesia, with consequent vigorous facial skin manipulation and lesion production, simulating other facial diseases such as ulcerative discoid lupus erythematosus, tumours and other artificially produced lesions. Ulceration and destruction of the ala nasi is a typical feature besides scratching end excoriations in the cutaneous segment affected. In this series, we present the features of five patients with trigeminal trophic syndrome, highlighting possible confusion with cutaneous lupus. Differential diagnoses, including discoid lupus erythematosus, are discussed, as well as possible treatment modalities.

Trigeminal-Targeted Treatments in Migraine: Is 60% the Magic Number?

Trigeminal-targeted treatments (TTTs), the most specific and selective therapeutic migraine approach to date, are effective in approximately 60% of patients regardless of treatment type or mechanism, at least if used alone. Sixty percent is also the proportion of migraineurs who develop migraine-like episodes following experimental intravenous administration of trigeminal neuropeptides and roughly 60% is the percentage of patients with a unilateral migraine tracing the area of cutaneous distribution of the trigeminal ophthalmic branch. Hence, mechanisms other than the trigeminovascular activation are probably involved in the 40% of migraineurs who do not respond to TTTs. A closer cooperation between clinical and basic neuroscientists is needed to explore migraine models because only a careful appraisal of migraine endophenotypes may help to unravel their underlying multifaceted pathophysiological machinery.

Chronic orofacial pain.

The issues specific to trigeminal pain include the complexity of the region, the problematic impact on daily function and significant psychological impact (J Dent, 43, 2015, 1203). By nature of the geography of the pain (affecting the face, eyes, scalp, nose, mouth), it may interfere with just about every social function we take for granted and enjoy (J Orofac Pain, 25, 2011, 333). The trigeminal nerve is the largest sensory nerve in the body, protecting the essential organs that underpin our very existence (brain, eyes, nose, mouth). It is no wonder that pain within the trigeminal system in the face is often overwhelming and inescapable for the affected individual.

Thirteen-And-A-Half Syndrome.

We describe a 50-year-old man who developed eight-and-a-half syndrome associated with an ipsilateral trigeminal nerve palsy because of a post-transplant lymphoproliferative disorder. This case widens the spectrum of eight-and-a-half syndrome to include a thirteen-and-a-half syndrome.

Inhibition of G protein-coupled P2Y2 receptor induced analgesia in a rat model of trigeminal neuropathic pain.

BACKGROUNDS: ATP and P2X receptors play important roles in the modulation of trigeminal neuropathic pain, while the role of G protein-coupled P2Y2 receptors and the underlying mechanisms are less clear. The threshold and frequency of action potentials, fast inactivating transient K+ channels (IA) are important regulators of membrane excitability in sensory neurons because of its vital role in the control of the spike onset. In this study, pain behavior tests, QT-RT-PCR, immunohistochemical staining, and patch-clamp recording, were used to investigate the role of P2Y2 receptors in pain behaviour. RESULTS: In control rats: 1) UTP, an agonist of P2Y2/P2Y4 receptors, caused a significant decrease in the mean threshold intensities for evoking action potentials and a striking increase in the mean number of spikes evoked by TG neurons. 2) UTP significantly inhibited IA and the expression of Kv1.4, Kv3.4 and Kv4.2 subunits in TG neurons, which could be reversed by the P2 receptor antagonist suramin and the ERK antagonist U0126. In ION-CCI (chronic constriction injury of infraorbital nerve) rats: 1) mRNA levels of Kv1.4, Kv3.4 and Kv4.2 subunits were significantly decreased, while the protein level of phosphorylated ERK was significantly increased. 2) When blocking P2Y2 receptors by suramin or injection of P2Y2R antisense oligodeoxynucleotides both led to a time- and dose-dependent reverse of allodynia in ION-CCI rats. 3) Injection of P2Y2 receptor antisense oligodeoxynucleotides induced a pronounced decrease in phosphorylated ERK expression and a significant increase in Kv1.4, Kv3.4 and Kv4.2 subunit expression in trigeminal ganglia. CONCLUSIONS: Our data suggest that inhibition of P2Y2 receptors leads to down-regulation of ERK-mediated phosphorylation and increase of the expression of I(A)-related Kv channels in trigeminal ganglion neurons, which might contribute to the clinical treatment of trigeminal neuropathic pain.

Orofacial pain and headache: a review and look at the commonalities.

Headache and facial pain - in particular, temporomandibular disorders (TMDs) - are very prevalent conditions in the general population. TMDs are defined as a collection of symptoms and signs involving masticatory muscles, the temporomandibular joints (TMJs), or both. The pain reported by TMD patients is typically located in the muscles of mastication, in the preauricular area, or in the TMJs. In many cases, headaches and facial pain will occur in the same patient. Much of the research relative to the relationship of these disorders focuses on statistics of association and prevalence data. This review will provide a brief description of the types and classifications of orofacial pains (OFPs), as well as point to relevant research describing the commonalities and potential comorbid nature of these maladies. Finally, several recent papers describing morphologic changes to the brain in headache and TMD individuals will be discussed in an effort to stimulate further research into the potential common pathophysiologic mechanism that may explain the comorbid nature of these disorders.

Neurotrophic keratitis.

Neurotrophic keratitis (NK) is a rare degenerative corneal disease that occurs as a result of partial or total impairment of trigeminal innervations, leading to a reduction (hypoesthesia) in or loss (anaesthesia) of corneal sensitivity. The impairment of sensory innervation causes a reduction in the lacrimation reflex and the vitality, metabolism and mitosis of epithelial cells, with subsequent deficiency in epithelial repair, stromal and intracellular oedema, loss of microvilli, and abnormal development of the basal lamina. Several recent studies have proposed different therapies based on different aetiopathogenetic theories. The aim of the therapy is to treat aetiopathogenesis and, at the same time, promote corneal healing. In this paper, we report the aetiology, diagnosis, management, and medical and surgical treatment of NK, also indicating future treatments based on the most recent studies.

Pain and plasticity: is chronic pain always associated with somatosensory cortex activity and reorganization?

The somatosensory cortex remodels in response to sensory deprivation, with regions deprived of input invaded by neighboring representations. The degree of cortical reorganization is correlated with ongoing pain intensity, which has led to the assumption that chronic pain conditions are invariably associated with somatosensory cortex reorganization. Because the presentation and etiology of chronic pain vary, we determined whether cortical changes in human subjects are similar for differing pain types. Using functional and anatomical magnetic resonance imaging, we found that, while human patients with neuropathic pain displayed cortical reorganization and changes in somatosensory cortex activity, patients with non-neuropathic chronic pain did not. Furthermore, cortical reorganization in neuropathic pain patients was associated with changes in regional anatomy. These data, by showing that pain per se is not associated with cortical plasticity, suggest that treatments aimed at reversing cortical reorganization should only be considered for use in patients with certain types of chronic pain.

PROSE treatment for lagophthalmos and exposure keratopathy.

Prosthetic replacement of the ocular surface ecosystem is a treatment developed by the Boston Foundation for Sight that uses a Food and Drug Administration-approved prosthetic device for the treatment of severe ocular surface disease to improve vision and discomfort in addition to supporting the ocular surface. Facial nerve paralysis has multiple causes including trauma, surgery, tumor, stroke, and congenital lagophthalmos. Subsequent lagophthalmos leading to exposure keratitis has been treated with copious lubrication, tarsorrhapy, eyelid weights, chemodenervation to yield protective ptosis, and palpebral spring insertion. Each of these treatments, however, has limitations and potential complications. The prosthetic replacement of the ocular surface ecosystem device provides a liquid bandage to protect the cornea from eyelid interaction and dessication in addition to improving vision. This report describes 4 patients with exposure keratitis who were successfully treated with prosthetic replacement of the ocular surface ecosystem devices at 2 clinical sites.

Idiopathic trigeminal sensory neuropathy. A case report.

Idiopathic trigeminal sensory neuropathy is a rare clinical condition characterized by sensory disturbances on the face. Its symptoms may be permanent or temporary and a wide variety of diagnostic procedures is usually required to establish the diagnosis. Frequently, it is the first manifestation of a systemic disorder. In the majority of cases causal treatment is not possible, even though patients with trigeminal sensory neuropathy should be carefully monitored by physicians.

Unilateral non-healing ulcers in zosteriform pattern.

Trigeminal trophic syndrome (TTS) is a rare disease that occurs after injury to the trigeminal nerve. Though this condition has been reported in the early 20th century, it is still a rare entity, with only around 200 cases reported so far. It characteristically presents with persistent facial ulceration with loss of sensation and paraesthesia along the distribution of the trigeminal nerve. We here report a case of TTS developing as a complication of herpes zoster, which possibly occurred due to the nerve damage caused by varicella-zoster virus.