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.

Long-Term Outcome of Unilateral Acoustic Neuromas With or Without Hearing Loss: Over 10 Years and Beyond After Gamma Knife Radiosurgery.

BACKGROUND: Since the long-term outcomes of 162 patients who underwent gamma knife radiosurgery (GKS) as an initial or adjuvant treatment for acoustic neuromas (ANs) with unilateral hearing loss were first reported in 1998, there has been no report of a comprehensive analysis of what has changed in GKS practice. METHODS: We performed a retrospective study of the long-term outcomes of 106 patients with unilateral sporadic ANs who underwent GKS as an initial treatment. The mean patient age was 50 years, and the mean initial tumor volume was 3.68 cm3 (range, 0.10-23.30 cm3). The median marginal tumor dose was 12.5 Gy (range, 8.0-15.0 Gy) and the median follow-up duration was 153 months (range, 120-216 months). RESULTS: The tumor volume increased in 11 patients (10.4%), remained stationary in 27 (25.5%), and decreased in 68 patients (64.2%). The actuarial 3, 5, 10, and 15-year tumor control rates were 95.3 ± 2.1%, 94.3 ± 2.2%, 87.7 ± 3.2%, and 86.6 ± 3.3%, respectively. The 10-year actuarial tumor control rate was significantly lower in the patients with tumor volumes of ≥ 8 cm3 (P = 0.010). The rate of maintaining the same Gardner-Robertson scale grade was 28.6%, and that of serviceable hearing was 46.4%. The rates of newly developed facial and trigeminal neuropathy were 2.8% and 4.7%, respectively. The patients who received marginal doses of less than 12 Gy revealed higher tumor control failure rates (P = 0.129) and newly occurred facial or trigeminal neuropathy rates (P = 0.040 and 0.313, respectively). CONCLUSION: GKS as an initial treatment for ANs could be helpful in terms of tumor control, the preservation of serviceable hearing, and the prevention of cranial neuropathy. It is recommended to perform GKS as soon as possible not only for tumor control in unilateral ANs with hearing loss but also for hearing preservation in those without hearing loss.

Concomitant Bandage Contact Lens, Oral Nicergoline, and Topical Autologous Serum for Severe Neurotrophic Keratitis.

BACKGROUND: To report the outcomes of using the combination of oral nicergoline, autologous serum, and contact lens to enhance corneal epithelization in neurotrophic keratitis and to discuss the clinical potential of this management. METHODS: This was a prospective consecutive case series study of eight patients treated for neurotrophic keratitis at the "Conde de Valenciana" Institute of Ophthalmology. Oral nicergoline, autologous serum, and bandage contact lens were initiated at the same time, immediately after stage 3 diagnosis keratitis was confirmed clinically, and until corneal epithelialization was achieved or eminent corneal perforation was seen. In patients where diabetes was a cause, glycosylate hemoglobin was measured to asses metabolic control. Corneal esthesiometry and corrected distance visual acuity were assessed before and after treatment. RESULTS: This study included eight eyes of eight patients (5 men [62.5%], average age 57±17.9 years). All patients completed at least 1 month of follow-up after nicergoline and contact lens suspension. Of the eight eyes, no one had positive culture growth and complete epithelial healing was achieved in all cases. Half of patients had diabetes and had a poor metabolic control. Corneal sensitivity improved in all eyes almost 2 centimeters in Cochet-Bonnet esthesiometry ( P= 0.01). In addition, final visual acuity gains were obtained ( P= 0.100). CONCLUSIONS: The combination of oral nicergoline, autologous serum, and bandage contact lens simultaneously could be an alternative in the management of stage 3 neurotrophic keratitis when conventional medical treatment has no improvement of corneal epithelization.

Nonpainful Trigeminal Neuropathy Associated with a Solitary Pontine Lesion: A Case Series.

A solitary pontine lesion (SPL) is a single brainstem lesion on the trigeminal nerve pathway without any other central nervous system lesion. This research aimed to investigate the demographic and clinical features of nonpainful TNO patients with SPL and identify the most frequently affected anatomical areas using lesion mapping techniques. Demographic and clinical features were retrospectively reviewed from the patients' charts. Brain lesions were mapped using MRIcroGL software. The study included 6 patients (three females and three males) with an SPL. The median age of the patients was 57 (range: 46-68) years. Cranial MRI displayed lesions in the dorsolateral pons and the cerebellar peduncle. The lesion mapping revealed that the lesions were on the trigeminal nerve pathway. SPL is an uncommon cause of TNO. Nonpainful SPL patients have demographic, clinical, and radiological features similar to those of painful SPL patients. The lesion mapping showed that the same brainstem areas are affected in painful and nonpainful SPL patients.

Uncommon cause of trigeminal neuritis and central nervous system involvement by herpes labialis: a case report.

BACKGROUND: Trigeminal neuropathy is characterized by numbness in the region innervated by the trigeminal nerves, with or without neuropathic weakness in the muscles of mastication. Trigeminal neuritis is a form of trigeminal neuropathy in which the lesion is caused by an inflammation. Herein, we report a patient with trigeminal neuritis due to central nervous system (CNS) involvement of herpes labialis (HL) infection, which was successfully treated with anti-viral and anti-inflammatory agents. CASE PRESENTATION: A young healthy female presented with numbness in the left hemiface for two weeks. She had a preceding typical HL infection on left facial lip one week before the sensory symptom onset. Brain magnetic resonance imaging revealed high signal intensities and asymmetrical thickening with enhancement along the cisternal segment of the left trigeminal nerve. Additionally, brain MR angiography showed multifocal stenoses in the M1 segment of the middle cerebral artery and the cavernous portion of the internal carotid artery. Cerebrospinal fluid (CSF) examination showed mild pleocytosis with normal protein level, glucose ratio, but CSF polymerase chain reaction assay for specific anti-viral antibodies including herpes simplex virus was negative, and CSF culture also did not identify a specific pathogen. The results of serologic testing including tumor markers and autoimmune markers were all unremarkable. A tentative diagnosis of trigeminal neuritis as a complication of HL involving the CNS was made considering the clinical, neuroradiological, and laboratory findings of the patient. Therefore, the patient was treated with intravenous methylprednisolone and acyclovir for 10 days. After the treatments, her sensory disturbance was markedly improved. Brain MRI at the 3-month follow-up also demonstrated improvement of previously identified high signal intensity lesions and multifocal intracerebral artery stenoses. CONCLUSION: HL is usually a self-limiting, benign disease without complications, but rarely presents as trigeminal neuritis due to CNS involvement. Therefore, meticulous evaluation may be necessary if trigeminal neuritis or CNS involving symptoms occur after HL.

Etiologies and Utility of Diagnostic Tests in Trigeminal Neuropathy.

OBJECTIVE: To evaluate the utility of diagnostic studies in identifying treatable etiologies of trigeminal neuropathy (TNP). PATIENTS AND METHODS: We performed a review of consecutive patients with nontraumatic, noniatrogenic TNP seen at Mayo Clinic between January 1, 2000, and August 31, 2019. Patients were excluded if they had trigeminal neuralgia without neuropathy or if their diagnostic work-up had been completed elsewhere. Data were analyzed to determine which diagnostic studies were most useful in identifying treatable etiologies. RESULTS: In total, 439 patients were included. The mean ± SD age was 56.3±13.6 years and 285 (64.9%) were female. Among the 180 cases in which an etiology was identified (41.0%), neoplasms were causative in 76 (42.2%), while specific connective tissue diseases were implicated in 71 (39.4%). Bilateral TNP (n=83) was associated with the presence of underlying connective tissue disease (P<.01). Identification of etiology was made by magnetic resonance imaging in 88 cases (48.8%), by abnormal connective tissue disease cascades combined with rheumatology consultation in 42 (23.3%), by a previously known connective tissue disorder in 30 (16.7%), and by abnormal connective tissue disease cascades alone in 8 (4.4%). Among the 439 study patients, electromyography was performed in 211 (48.1%) and lumbar puncture in 139 (31.7%), but their diagnostic utility was low. CONCLUSION: Underlying causes of nontraumatic, noniatrogenic TNP can be identified in approximately 40% of cases. Bilateral TNP is strongly associated with underlying connective tissue disease. Careful history taking, dedicated magnetic resonance imaging, and connective tissue panels have the greatest diagnostic utility. Electromyography and cerebrospinal fluid analysis are unlikely to elucidate treatable etiologies of TNP.

[Treatment of neurotrophic keratopathy in combined lesions of the trigeminal and facial nerves]. / Lechenie neirotroficheskoi keratopatii pri sochetannom porazhenii troinichnogo i litsevogo nervov.

Ocular symptomatology in lesions of the facial nerve is associated with disturbed innervation of the circular muscle of the eye that leads to disruption of the protective function of the eyelids and the development of exposure symptoms, and is accompanied by a breach in corneal tear film integrity. The main clinical manifestation of the trigeminal nerve damage is the loss of sensory innervation of the cornea and disruption of the supply of neurotransmitters to its cells, manifesting as corneal hypo- or anesthesia. This triggers a cascade of pathological processes that lead to the development of neurotrophic keratopathy. In combined pathology of the facial and trigeminal nerves, a number of interrelated and mutually aggravating problems arise that require correction of lagophthalmos and functional restoration of the trigeminal nerve, since there is an interaction between the corneal epithelium and trigeminal neurons through trophic neuromodulators, which normally contribute to the proliferation of epithelial cells, their differentiation, migration and adhesion, and are essential for vital functions, metabolism and healing of surface lesions of the eye. Classical methods of treating neurotrophic keratopathy aim to protect the ocular surface, and are palliative or auxiliary, do not provide radical relief of the symptoms of neurotrophic keratopathy. Modern surgical technique of neurotization of the cornea allows restoring the structural growth of the nerve, which provides nerve trophism and corneal sensitivity, and is the only pathogenetically substantiated method of effective treatment of neurotrophic keratopathy. At the same time, direct neurotization has undeniable advantages over methods involving intercalary donor nerves, since neuropeptides from nerve fibers are immediately released into the recipient tissue and start reparative processes. Taking into account the accumulated positive experience of neurotization surgeries, scientific and clinical research should be continued in order to improve the most effective methods of corneal neurotization and promote their wider implementation into clinical practice.

Trigeminal neuropathy as presenting symptom of craniofacial venous metameric syndrome.

AIMS: Trigeminal neuropathy is more likely to suggest neuronal damage and occur due to secondary pathology than trigeminal neuralgia. Evaluation of underlying etiologies are necessary. CASE: A 29-year-old female patient presented with left sided continuous burning pain likened to pins and needles at maxillary distribution for about a year. Her examination was normal except left-sided buccal swelling without any skin or mucosal change. Cranial MRI revealed asymmetrical dilation of left Meckel's cave, bilateral cerebral developmental venous anomaly and left sided slow flow venous malformation from superior temporal fossa to masseter muscle. Cerebral angiography confirmed widespread venous return anomaly in both cerebral hemispheres and slow-flow venous malformation that does not fill in the early arterial phase in the left buccal space and superficial temporal fossa. Cerebrofacial venous metameric syndrome is diagnosed. Percutaneous sclerotherapy with alcohol is planned in three separate sessions, the first of the three planned sessions is performed yet and the patient stated that her neuropathic pain decreased by 40% afterwards. SIGNIFICANCE: Clinical manifestation of the cerebrofacial venous metameric syndrome depends on the localization of the lesions; therefore, venous anomalies in relation with the trigeminal branches can present with painful trigeminal neuropathy.

Clinical Outcomes of Minimally Invasive Corneal Neurotization After Cerebellopontine Angle Neurosurgical Procedures.

PURPOSE: To report 12 patients with neurotrophic keratopathy due to the trigeminal nerve injury after intracranial tumor surgeries underwent minimally invasive corneal neurotization and evaluate the outcomes of corneal reinnervation. METHODS: Twelve patients (12 eyes) with neurotrophic keratopathy caused by the trigeminal nerve injury after intracranial surgeries received minimally invasive corneal neurotization. All the preoperative central corneal sensation was under 5 mm, and minimally invasive corneal neurotization was performed over 6 months after the intracranial surgery. Follow-up was conducted 1 week and 1 month after the surgery and then every 3 months. Twelve healthy age-matched participants were enrolled as controls. The indicators included corneal sensation, best-corrected visual acuity, corneal nerve fiber length and branch density, diameter of nerve trunk, corneal ulcer lesion ratio, and sensation of the contralateral forehead. RESULTS: Mean follow-up was 24.7 ± 7.1 months. Mean central corneal sensation rose from 0.4 ± 1.4 to 31.7 ± 21.8 mm. Corneal nerve fiber length improved from 9.56 ± 5.00 to 14.96 ± 4.65 mm/mm2 and corneal nerve branch density and diameter of nerve trunk both increased (p < .01 and p < .05, respectively). Corneal lesion ratio decreased from 0.17 ± 0.12 to 0.10 ± 0.10 (p < .01). CONCLUSIONS: Minimally invasive corneal neurotization promotes corneal reinnervation for patients with neurotrophic keratopathy induced by the trigeminal nerve injury after intracranial surgeries. The process of corneal reinnervation after minimally invasive corneal neurotization often lasts over 12 months, and it takes about 18 months to return to a higher level. Corneal sensation and corneal nerve fiber length are related to clinical outcomes such as corneal ulcer lesion and best-corrected visual acuity. The effect on the sensation of the contralateral side forehead is temporary, and most patients can restore normal forehead sensation of the contralateral side.

Topical Nerve Growth Factor for the Treatment of Neurotrophic Keratopathy Caused by Wallenberg Syndrome.

PURPOSE: The aim of this study was to report a case of central neurotrophic keratopathy (NK) in Wallenberg syndrome (WS) and its successful management with topical recombinant nerve growth factor (rNGF). METHODS: A 47-year-old man with WS caused by a stroke in the territory of the left vertebrobasilar artery complained of progressive visual loss in his left eye (OS). Examination showed corneal anesthesia associated with a corneal epithelial ulceration consistent with a diagnosis of NK grade 3 of central origin. Topical treatment with rNGF, 1 drop 6 times daily, was started for 8 weeks, and the patient was followed up for 1 year. RESULTS: Topical treatment with rNGF was successful in promoting complete epithelial corneal healing. No recurrence was seen at 1-year follow-up. CONCLUSIONS: Clinicians should be aware that visual loss can also occur from NK of central origin. To the best of our knowledge, this is the first case report of NK caused by WS successfully treated with rNGF reported in the literature.

Pseudomonas aeruginosa-induced nociceptor activation increases susceptibility to infection.

We report a rapid reduction in blink reflexes during in vivo ocular Pseudomonas aeruginosa infection, which is commonly attributed and indicative of functional neuronal damage. Sensory neurons derived in vitro from trigeminal ganglia (TG) were able to directly respond to P. aeruginosa but reacted significantly less to strains of P. aeruginosa that lacked virulence factors such as pili, flagella, or a type III secretion system. These observations led us to explore the impact of neurons on the host's susceptibility to P. aeruginosa keratitis. Mice were treated with Resiniferatoxin (RTX), a potent activator of Transient Receptor Potential Vanilloid 1 (TRPV1) channels, which significantly ablated corneal sensory neurons, exhibited delayed disease progression that was exemplified with decreased bacterial corneal burdens and altered neutrophil trafficking. Sensitization to disease was due to the increased frequencies of CGRP-induced ICAM-1+ neutrophils in the infected corneas and reduced neutrophil bactericidal activities. These data showed that sensory neurons regulate corneal neutrophil responses in a tissue-specific matter affecting disease progression during P. aeruginosa keratitis. Hence, therapeutic modalities that control nociception could beneficially impact anti-infective therapy.

Multicompartmental Epidermoid Cyst Causing Chronic Parotid Gland and Masticator Space Muscle Atrophy.

Epidermoid cysts are rare benign intracranial tumors of congenital origin. They are slow-growing and are seen to insinuate between brain structures. These are commonly located in cerebello-pontine angle and parasellar regions. The symptoms produced are primarily due to mass effect. Hearing loss, facial nerve palsy, and trigeminal neuralgia are reported when cranial nerves are involved; motor palsy of trigeminal nerve is uncommon. Here, we present an interesting case of an extensive multicompartmental epidermoid cyst causing atrophy of trigeminal nerve with radiologic evidence of chronic motor trigeminal nerve palsy characterized by atrophy of masticator space muscles and parotid gland.

Acute Unilateral Masseter Muscle Paralysis Caused by Pontine Infarction.

In the present report, we discussed the case of a 57-year-old man with unilateral masticatory muscle weakness, nystagmus, skew deviation and facial hypesthesia due to pontine tegmental infarction. Trigeminal motor neuropathy attributed to brain infarction is very rare. Brain magnetic resonance imaging revealed a small dot-like infarction lesion in the pontine tegmentum. Masticatory muscle weakness was confirmed by an electrophysiological study performed on the day after admission in which there was an incomplete interference pattern without spontaneous denervation activity, suggesting that the patient's masseter muscle weakness was caused by an infarction of the trigeminal motor nucleus proper or trigeminal motor nerve fascicles rather than Wallerian degeneration of the trigeminal nerve or the progression of masseter muscle degeneration.

Case Report: Ocular Tilt Reaction with Internuclear Ophthalmoplegia and Multiple Cranial Nerve Palsies.

SIGNIFICANCE: Ocular tilt reaction (OTR) is an abnormal eye-head postural reaction that consists of skew deviation, head tilt, and bilateral ocular torsion. Understanding of the pathway of the vestibulo-ocular reflex (VOR) is essential because this will help to localize the pathology. PURPOSE: The aim of this study was to report a case of OTR with contralateral internuclear ophthalmoplegia (INO) and fifth and seventh cranial nerve palsies. CASE REPORT: A 51-year-old gentleman with underlying diabetes mellitus presented with sudden onset of diplopia for 3 days. On examination, his visual acuity was 20/30 bilaterally without a relative afferent pupillary defect. He had a right OTR consisting of a right head tilt, a skew deviation with a left eye hypertropia, and bilateral ocular torsion (right excyclotorsion and left incyclotorsion) with nystagmus. He also had a left adduction deficit and right abduction nystagmus consistent with a left INO. Ocular examination revealed evidence of proliferative diabetic retinopathy bilaterally. Two days after the initial presentation, the patient developed left seventh and fifth cranial nerve palsies. MRI showed left pontine infarction and multiple chronic lacunar infarctions. There was an incidental finding of a vascular loop compression on cisternal portions of the left trigeminal, facial, and vestibulocochlear nerves. Antiplatelet treatment was started on top of a better diabetic control. The diplopia was gradually resolved with improved clinical signs. In this case, the left pontine infarction had likely affected the terminal decussated part of the vestibulocochlear nerve from the right VOR pathway, medial longitudinal fasciculus, and cranial nerve nuclei in the left pons. CONCLUSIONS: The OTR can be ipsilateral to the lesion if the lesion is before the decussation of the VOR pathway in the pons, or it can be contralateral to the lesion if the lesion is after the decussation. In case of an OTR that is associated with contralateral INO and other contralateral cranial nerves palsy, a pathology in the pons that is contralateral to the OTR should be considered. Neuroimaging study can hence be targeted to identify the possible cause.

Optic, trigeminal, and facial neuropathy related to anti-neurofascin 155 antibody.

OBJECTIVE: To characterize the frequency and patterns of optic, trigeminal, and facial nerve involvement by neuroimaging and electrophysiology in IgG4 anti-neurofascin 155 antibody-positive (NF155+ ) chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Thirteen IgG4 NF155+ CIDP patients with mean onset age of 34 years (11 men) were subjected to neurological examination, blink reflex, and visual-evoked potential (VEP) testing, and axial and/or coronal T2-weighted head magnetic resonance imaging (MRI). RESULTS: Among 13 patients, facial sensory impairment, facial weakness, and apparent visual impairment were observed in three (23.1%), two (15.4%), and two (15.4%) patients, respectively. All 12 patients tested had blink reflex abnormalities: absent and/or delayed R1 in 11 (91.7%), and absent and/or delayed R2 in 10 (83.3%). R1 latencies had strong positive correlations with serum anti-NF155 antibody levels (r = 0.9, P ≤ 0.0001 on both sides) and distal and F wave latencies of the median and ulnar nerves. Absent and/or prolonged VEPs were observed in 10/13 (76.9%) patients and 17/26 (65.4%) eyes. On MRI, hypertrophy, and high signal intensity of trigeminal nerves were detected in 9/13 (69.2%) and 10/13 (76.9%) patients, respectively, whereas optic nerves were normal in all patients. The intra-orbital trigeminal nerve width on coronal sections showed a significant positive correlation with disease duration. INTERPRETATION: Subclinical demyelination frequently occurs in the optic, trigeminal, and facial nerves in IgG4 NF155+ CIDP, suggesting that both central and peripheral myelin structures of the cranial nerves are involved in this condition, whereas nerve hypertrophy only develops in myelinated peripheral nerve fibers.