Sarcoid optic neuropathy.

A 45-year-old woman presented with sudden complete vision loss in her left eye and retroorbital pain worsened by eye movements. A previous milder episode of vision loss had occurred in the same eye 1 year before, with complete recovery after high-dose intravenous methylprednisolone. She had no light perception in the left eye with a swollen optic disc, but with a normal right optic disc. There were no systemic manifestations or infections. MR scan of the brain showed extensive enlargement and enhancement of the left optic nerve and optic chiasm. After excluding infections and autoimmune markers, a left optic nerve biopsy confirmed non-caseating granulomas, leading to a diagnosis of neurosarcoidosis.

Cytokines profile in pure neural leprosy.

Introduction: Pure Neural Leprosy (PNL) is a form of this long time known disease that affects only the peripheral nervous system. Since it is a rare form of the disease, its pathophisiology is still poorly understood. Objective: Describe the cytokines profile in patients with PNL. Methods: 30 Patients diagnosed with PNL in the Souza Araujo Outpatient Clinic and with cytokines evaluated were selected. They were evaluated by neurologists and diagnosed after a nerve biopsy. Serum levels of IL-1 ß, IL-6, IL-10, IL-17, TNF, CCL-2/MCP-1, IFN-ϒ, CXCL-10/IP-10 and TGF-ß were evaluates at the moment of the diagnosis. Results: Neural thickening was a common clinical finding in this groups of patients. Small and medium sensitive fibers signs and symptoms were present in 92% of the patients and motor involvement in 53%. 43% of patients presented neuropathic pain and no one had neuritis TGF-beta, IL-17, CCl-2 and IP-10. CCL-2 levels were associated with demyelinating patters and IP-10 and IL-1o were associated with axonal patterns at NCS. Discussion: PNL patients' cytokine profile appears to be different of other clinical forms of leprosy, with the presence of cytokines described in both tuberculoid and lepromatous leprosy. High levels of CCl-2 may be related to the presence of silent neuritis as well as the presence of IL-10. PNL is unique a form of leprosy, therefore, understanding its immunological profiles essential to better understand the disease itself.

Effectiveness of neurolysis as a treatment for complications of leprosy neuritis: a systematic review.

BACKGROUND: Complications of leprosy neuritis are considered serious and apparent, with the potential to disable and/or limit individuals. These complications affect not only a patient's physical functioning, but also their family and social lives, while directly impacting the ability to work and/or maintain financial independence, subsequently interfering with their overall quality of life. The present review, therefore, aimed to analyze the effectiveness of neurolysis as an alternative treatment for the complications associated with leprosy neuritis. METHODS: The present review was performed based on the Joanna Briggs Institute methodology, in an effort to answer the following research question: what is the effectiveness of neurolysis as a treatment for leprosy neuritis complications? This research question was defined using the patient-intervention-outcome (PIO) framework, where leprosy represents 'P', neurolysis for 'I', and neuropathic pain/motor function/sensorial function/physical disability/quality of life for 'O'. Randomized and non-randomized clinical trials and prospective observational cohort studies were included in the present review, with no time or date restrictions. RESULTS: The present review included 1 randomized clinical trial and 10 prospective studies, published between 1976 and 2020. All of the outcomes showed improvement, with relief from neuropathic pain being the primary finding. CONCLUSIONS: The evidence obtained in the present review suggested that neurolysis is an effective alternative for the treatment of physical disabilities, the recovery of sensory and motor function, the restoration of quality of life, and neuropathic pain relief.

Unexpected Ocular Manifestations Due to Exposure to Highly Concentrated Peracetic Acid: A Case Report.

BACKGROUND Peracetic acid is among the disinfectants that irritate the upper respiratory tract, skin, and conjunctiva. It can cause symptoms of eye irritation, secondary to an inflammatory process that can lead to various manifestations. Irritation happens due to the high reduction potential of the acid, which causes the consequent release of reactive oxygen species. This fact serves to reinforce the importance of personal protective equipment when handling peracetic acid. CASE REPORT During an accident at work, a 21-year-old patient received a strong jet of disinfectant solution directly into both eyes. The composition of the disinfectant solution was 15% peracetic acid, 15-16% hydrogen peroxide, 22-23% acetic acid, and 16-17% horticultural sanitizers. Twenty-four hours after the incident, eye damage (punctate keratitis and low visual acuity) had occurred, and was treated by washing the eye with ice water and frequently applying lubricating eye drops. The next day, the patient returned with an improvement of irritative symptoms, but with a major complaint of low visual acuity in left eye, secondary to optic neuritis, detected by fundoscopy and confirmed by optical coherence tomography. In the following week, fluorescent angiography indicated the persistence of neuritis in the left eye. This was treated with prednisone, 40 mg/day, which brought about gradual improvement. Two months later, the patient returned with test results showing normal magnetic resonance imaging and negative serologies (for syphilis, HIV, and herpes virus), visual acuity 20/20 in both eyes, and normalization of angiography and optical coherence tomography parameters. CONCLUSIONS Until now, there have been no published studies demonstrating neuritis caused by direct contact of peracetic acid into the eyes. Therefore, this is the first report in the world literature of this manifestation of ocular exposure to peracetic acid. This is a chemical formulation that is widely useful and prevents the growth of various pathogens. Further investigation and studies on the subject should be encouraged to improve its management and use.

Evidencing leprosy neuronal inflammation by 18-Fluoro-deoxy-glucose.

BACKGROUND: Leprosy is caused by multiple interactions between Mycobacterium leprae (M. leprae) and the host's peripheral nerve cells. M. leprae primarily invades Schwann cells, causing nerve damage and consequent development of disabilities. Despite its long history, the pathophysiological mechanisms of nerve damage in the lepromatous pole of leprosy remain poorly understood. This study used the findings of 18F-FDG PET/CT on the peripheral nerves of eight lepromatous patients to evaluate the degree of glucose uptake by peripheral nerves and compared them with clinical, electrophysiological, and histopathological evaluations. METHODS: Eight patients with lepromatous leprosy were included in this study. Six patients were evaluated up to three months after leprosy diagnosis using neurological examination, nerve conduction study, 18F-FDG PET/CT, and nerve biopsy. Two others were evaluated during an episode of acute neuritis, with clinical, neurophysiological, and PET-CT examinations to compare the images with the first six. RESULTS: Initially, six patients already had signs of peripheral nerve injury, regardless of symptoms; however, they did not present with signs of neuritis, and there was little or no uptake of 18F-FDG in the clinically and electrophysiologically affected nerves. Two patients with signs of acute neuritis had 18F-FDG uptake in the affected nerves. CONCLUSIONS: 18F-FDG uptake correlates with clinical neuritis in lepromatous leprosy patients but not in silent neuritis patients. 18F-FDG PET-CT could be a useful tool to confirm neuritis, especially in cases that are difficult to diagnose, such as for the differential diagnosis between a new episode of neuritis and chronic neuropathy.

Ultrasonography as a diagnostic tool for Neural Pain in Leprosy.

Leprosy is still a prevalent disease in Brazil, representing 93% of all occurrences in the Americas. Leprosy neuropathy is one of the most worrying manifestations of the disease. Acute neuropathy usually occurs during reaction episodes and is called neuritis. Twenty-two leprosy patients were included in this study. These patients had neural pain associated with ulnar sensory neuropathy, with or without adjunct motor involvement. The neurological picture began within thirty days of the clinical evaluation. The patients underwent a nerve conduction study and the demyelinating findings confirmed the diagnosis of neuritis. Ultrasonographic study (US) of the ulnar nerve was performed in all patients by a radiologist who was blinded to the clinical or neurophysiological results. Morphological characteristics of the ulnar nerve were analyzed, such as echogenicity, fascicular pattern, transverse cross-sectional area (CSA), aspect of the epineurium, as well as their anatomical relationships. The volume of selected muscles referring to the ulnar nerve, as well as their echogenicity, was also examined. Based on this analysis, patients with increased ulnar nerve CSA associated with loss of fascicular pattern, epineurium hyperechogenicity and presence of power Doppler flow were classified as neuritis. Therefore, patients initially classified by the clinical-electrophysiological criteria were reclassified by the imaging criteria pre-established in this study as with and without neuritis. Loss of fascicular pattern and flow detection on power Doppler showed to be significant morphological features in the detection of neuritis. In 38.5% of patients without clinical or neurophysiological findings of neuritis, US identified power Doppler flow and loss of fascicular pattern. The US is a method of high resolution and portability, and its low cost means that it could be used as an auxiliary tool in the diagnosis of neuritis and its treatment, especially in basic health units.

Neurosyphilis and High-Resolution Vessel Wall Imaging: A Powerful Tool to Detect Vasculitis and Neuritis.

Syphilis is a reemergent infection worldwide. There has been a steady increase in incidence across many population groups in the last decades, requiring public health authorities' attention. We report a case of a 67-year-old man with neurosyphilis who presented with abrupt clinical ophthalmic changes and sensorineural hearing loss. The neuroradiological investigation demonstrated cranial nerves and vascular involvement, detected on high-resolution vessel wall imaging in brain magnetic resonance imaging. CSF and blood VDRL test were positive, as well as blood serum fluorescent treponemal antibody absorption test (FTA-ABS) and chemiluminescent magnetic microparticle immunoassay (CMIA). A test for the human immunodeficiency virus was negative. The patient was administered intravenous penicillin G for 21 days and was discharged well, with no neurologic signs.

Leprosy as immune reconstitution inflammatory syndrome in patients living with HIV: Description of French Guiana's cases over 20 years and systematic review of the literature.

BACKGROUND: HIV infection is highly prevalent in French Guiana, a territory where leprosy is also endemic. Since the introduction of Highly Active Antiretroviral Treatment (HAART) in the management of HIV, leprosy has been reported as part of the immune reconstitution inflammatory syndrome (IRIS). METHODOLOGY/PRINCIPAL FINDINGS: We aimed to present a general description of these forms of leprosy as IRIS, highlighting clinical and therapeutic specificities. A retrospective study was conducted in French Guiana, including patients living with HIV (PLHIV) with advanced infection (CD4 < 200/mm3) and developing leprosy or a leprosy reaction within six months of HAART initiation, from 2000 to 2020. Clinical, histological and biological data were collected for all these patients. Six patients were reported in French Guiana. A systematic review of the literature was conducted, and its results were added to an overall analysis. Overall, seventy-three PLHIV were included. They were mainly men (74%), aged 22-54 years (median 36 years), mainly from Brazil (46.5%) and India (32.8%). Most leprosy cases (56.2%) were borderline tuberculoid (BT). Leprosy reactions were frequent (74%), mainly type 1 reaction (T1R) (68.5%), sometimes intense with ulceration of skin lesions (22%). Neuritis was observed in 30.1% of patients. The outcome was always favorable under multidrug therapy (MDT), continuation of HAART and additional corticosteroid therapy in case of neuritis or ulceration. There was no relapse. CONCLUSION: Leprosy as IRIS in PLHIV mainly presents as a BT leprosy in a T1R state, sometimes with ulcerated skin lesions. Response to MDT is usually good. Systemic corticosteroids are necessary and efficient in case of neuritis.

Tibolone restrains neuroinflammation in mouse experimental autoimmune encephalomyelitis.

Multiple sclerosis (MS) is an immune-mediated and degenerating disease in which myelin sheaths are damaged as a result of chronic progressive inflammation of the central nervous system. Tibolone [(7α,17α)-17-hydroxy-7-methyl-19-norpregn-5(10)-en-20-in-3-one], a synthetic estrogenic compound with tissue-specific actions and used for menopausal hormone therapy, shows neuroprotective and antioxidant properties both in vivo and in vitro. In the present study, we analyzed whether tibolone plays a therapeutic role in experimental autoimmune encephalomyelitis (EAE) mice, a commonly used model of MS. Female C57BL/6 mice were induced with the myelin oligodendrocyte glycoprotein MOG35-55 and received s.c. tibolone (0.08 mg kg-1 ) injection every other day from the day of induction until death on the acute phase of the disease. Reactive gliosis, Toll like receptor 4 (TLR4), high mobility group box protein 1 (HMGB1), inflammasome parameters, activated Akt levels and myelin were assessed by a real-time polymerase chain reaction, immunohistochemistry, and western blot analysis. Our findings indicated that, in the EAE spinal cord, tibolone reversed the astrocytic and microglial reaction, and reduced the hyperexpression of TLR4 and HMGB1, as well as NLR family pyrin domain containing 3-caspase 1-interleukin-1ß inflammasome activation. At the same time, tibolone attenuated the Akt/nuclear factor kappa B pathway and limited the white matter demyelination area. Estrogen receptor expression was unaltered with tibolone treatment. Clinically, tibolone improved neurological symptoms without uterine compromise. Overall, our data suggest that tibolone may serve as a promising agent for the attenuation of MS-related inflammation.

Dramatic secukinumab-mediated improvements in refractory leprosy-related neuritis via the modulation of T helper 1 (Th1) and T helper 17 (Th17) immune pathways.

A 39-year-old woman was diagnosed with relapsed multibacillary leprosy and refractory neuritis. Here, we describe an evident loss of therapeutic effectiveness after the third pulse of corticosteroids, which may be attributed to tachyphylaxis and the posterior modulation of interferon- γ (IFN-γ), tumor necrosis factor- α (TNF-α,) interleukin-17A (IL-17A), and IL-12/23p40 after the induction phase of secukinumab. In this case, plasma cytokine analysis showed that secukinumab induced a reduction in IL-17 concomitant with impressive clinical improvements in the patient's neural function. Interestingly, secukinumab induced reductions in cytokines related to Th1 responses and earlier stages of the Th17 response, including IL-23/12.

Clinical prediction rules for the diagnosis of neuritis in leprosy.

BACKGROUND: Diagnosing neuritis in leprosy patients with neuropathic pain or chronic neuropathy remains challenging since no specific laboratory or neurophysiological marker is available. METHODS: In a cross-sectional study developed at a leprosy outpatient clinic in Rio de Janeiro, RJ, Brazil, 54 individuals complaining of neural pain (single or multiple sites) were classified into two groups ("neuropathic pain" or "neuritis") by a neurological specialist in leprosy based on anamnesis together with clinical and electrophysiological examinations. A neurologist, blind to the pain diagnoses, interviewed and examined the participants using a standardized form that included clinical predictors, pain features, and neurological symptoms. The association between the clinical predictors and pain classifications was evaluated via the Pearson Chi-Square or Fisher's exact test (p < 0.05). RESULTS: Six clinical algorithms were generated to evaluate sensitivity and specificity, with 95% confidence intervals, for clinical predictors statistically associated with neuritis. The most conclusive clinical algorithm was: pain onset at any time during the previous 90 days, or in association with the initiation of neurological symptoms during the prior 30-day period, necessarily associated with the worsening of pain upon movement and nerve palpation, with 94% of specificity and 35% of sensitivity. CONCLUSION: This algorithm could help physicians confirm neuritis in leprosy patients with neural pain, particularly in primary health care units with no access to neurologists or electrophysiological tests.

Dramatic secukinumab-mediated improvements in refractory leprosy-related neuritis via the modulation of T helper 1 (Th1) and T helper 17 (Th17) immune pathways

Abstract A 39-year-old woman was diagnosed with relapsed multibacillary leprosy and refractory neuritis. Here, we describe an evident loss of therapeutic effectiveness after the third pulse of corticosteroids, which may be attributed to tachyphylaxis and the posterior modulation of interferon- γ (IFN-γ), tumor necrosis factor- α (TNF-α,) interleukin-17A (IL-17A), and IL-12/23p40 after the induction phase of secukinumab. In this case, plasma cytokine analysis showed that secukinumab induced a reduction in IL-17 concomitant with impressive clinical improvements in the patient's neural function. Interestingly, secukinumab induced reductions in cytokines related to Th1 responses and earlier stages of the Th17 response, including IL-23/12.

Cytokine Levels in Neural Pain in Leprosy.

Pain is a frequent symptom in leprosy patients. It may be predominantly nociceptive, as in neuritis, or neuropathic, due to injury or nerve dysfunction. The differential diagnosis of these two forms of pain is a challenge in clinical practice, especially because it is quite common for a patient to suffer from both types of pain. A better understanding of cytokine profile may serve as a tool in assessing patients and also help to comprehend pathophysiology of leprosy pain. Patients with leprosy and neural pain (n = 22), neuropathic pain (n = 18), neuritis (nociceptive pain) (n = 4), or no pain (n = 17), further to those with diabetic neuropathy and neuropathic pain (n = 17) were recruited at Souza Araujo Out-Patient Unit (Fiocruz, Rio de Janeiro, RJ, Brazil). Serum levels of IL1ß, IL-6, IL-10, IL-17, TNF, CCL-2/MCP-1, IFN-γ, CXCL-10/IP-10, and TGF-ß were evaluated in the different Groups. Impairment in thermal or pain sensitivity was the most frequent clinical finding (95.5%) in leprosy neuropathy patients with and without pain, but less frequent in Diabetic Group (88.2%). Previous reactional episodes have occurred in patients in the leprosy and Pain Group (p = 0.027) more often. Analysis of cytokine levels have demonstrated that the concentrations of IL-1ß, TNF, TGF-ß, and IL-17 in serum samples of patients having leprosy neuropathy in combination with neuropathic or nociceptive pain were higher when compared to the samples of leprosy neuropathy patients without pain. In addition, these cytokine levels were significantly augmented in leprosy patients with neuropathic pain in relation to those with neuropathic pain due to diabetes. IL-1ß levels are an independent variable associated with both types of pain in patients with leprosy neuropathy. IL-6 concentration was increased in both groups with pain. Moreover, CCL-2/MCP-1 and CXCL-10/IP-10 levels were higher in patients with diabetic neuropathy over those with leprosy neuropathy. In brief, IL-1ß is an independent variable related to neuropathic and nociceptive pain in patients with leprosy, and could be an important biomarker for patient follow-up. IL-6 was higher in both groups with pain (leprosy and diabetic patients), and could be a therapeutic target in pain control.

Neurite aguda do nervo trigêmeo em Pastor Alemão / Acute trigeminal nerve neuritis in German Shepherd

Background: Trigeminal nerve is composed by ophthalmic, maxillary and mandibular portion, presenting sensory and motor functions. Its most common conditions include vascular, neoplastic, infectious and inflammatory causes. Neuritis is an inflammation caused by a primary nerve injury that can progress to demyelination and even degeneration of nerve fibers. The present report aims to describe an unusual case of a female dog, German Shepherd breed, with acute manifestation of trigeminal nerve neuritis whose etiology may be associated with erlichiosis, since infection with Ehrlichia spp. through serological test was verified. Case: A 3-year-old female German Shepherd, weighing 26.8 kg and not neutered, was attended at Veterinary Hospital Dr. Halim Atique - UNIRP, São José do Rio Preto, SP, Brazil presenting apathy, sialorrhea and polydipsia for seven days. The tutor reported an episode of foamy and yellowish vomit three days ago and ixodidiosis last week. Physical examination revealed flaccid open-mouthed posture, with mild bilateral masseter and moderate temporal muscle atrophy. Water was offered to the animal and it was observed that it could not properly seize, confirming a false polydipsia. The neurological examination revealed a slight decrease in head sensitivity, difficulty in chewing and seizure of food. It was not observed alterations in the other pairs of cranial nerves or other neurological parameters (postural reactions and spinal reflexes), and the diagnose of bilateral dysfunction of the trigeminal nerve was based on the affected neuroanatomic region. On neurological examination, other lesions of the nervous system were ruled out, suggesting an isolated manifestation of the trigeminal nerve. CBC revealed anemia, intense thrombocytopenia and leukocytosis by neutrophilia. Radiographic examination ruled out the possibility of trauma due to the absence of mandible fracture and also temporomandibular joint alterations. Masseter and temporal muscle biopsy were performed, and myositis…(AU)

Neurite idiopática do nervo trigêmeo em um cão / Idiopathic neuritis of the trigeminal nerve in a dog

Background: Idiopathic trigeminal neuropathy is a peripheral neuropathy whose clinical signs include inability to close themouth. A neurological examination reveals mainly flaccid paralysis of the masticatory muscles, which may be accompaniedby atony/hypotonia of the masseter and temporalis muscles. Altered sensitivity may also be present in very rare cases.This article reports a case of idiopathic trigeminal neuropathy with involvement of the motor and sensory nerve fibers in adog, describing the clinical symptoms, neurological findings, exclusion of other diseases, and evolution of the condition.Case: A 5-year-old bitch showing signs of difficulty in picking up food, swallowing water, and hypersalivation was takento a veterinary clinic. According to her owner, these signs had started spontaneously 2 days earlier. A physical examinationof the animal revealed only a persistently open mouth, although the mouth opening and closing movements could be performed manually by manipulating the jaw. The patient showed swallowing ability, and her tongue movements were intact.The neurological examination revealed that the animal also presented bilateral absence of nasal sensitivity and absenceof palpebral reflex. The animals blood test results showed changes only in the CK and AST levels, which were 1,182.60U/L (reference: 1.5 to 28.4 U/L) and 87.1 IU/L (reference: 6.2 to 13 IU/L), respectively. The animal tested negative fordistemper, leishmaniasis, toxoplasmosis and neosporosis. Abdominal ultrasound and thoracic radiography were performedto investigate neoplasia, as well as radiography of the temporomandibular joints, and none of these imaging tests revealedany alterations. A cerebrospinal fluid (CSF) analysis was also within the normal range of reference parameters. In view ofthe possibility of idiopathic trigeminal neuropathy, prednisolone...(AU)

Neurite idiopática do nervo trigêmeo em um cão / Idiopathic neuritis of the trigeminal nerve in a dog

Background: Idiopathic trigeminal neuropathy is a peripheral neuropathy whose clinical signs include inability to close themouth. A neurological examination reveals mainly flaccid paralysis of the masticatory muscles, which may be accompaniedby atony/hypotonia of the masseter and temporalis muscles. Altered sensitivity may also be present in very rare cases.This article reports a case of idiopathic trigeminal neuropathy with involvement of the motor and sensory nerve fibers in adog, describing the clinical symptoms, neurological findings, exclusion of other diseases, and evolution of the condition.Case: A 5-year-old bitch showing signs of difficulty in picking up food, swallowing water, and hypersalivation was takento a veterinary clinic. According to her owner, these signs had started spontaneously 2 days earlier. A physical examinationof the animal revealed only a persistently open mouth, although the mouth opening and closing movements could be performed manually by manipulating the jaw. The patient showed swallowing ability, and her tongue movements were intact.The neurological examination revealed that the animal also presented bilateral absence of nasal sensitivity and absenceof palpebral reflex. The animal’s blood test results showed changes only in the CK and AST levels, which were 1,182.60U/L (reference: 1.5 to 28.4 U/L) and 87.1 IU/L (reference: 6.2 to 13 IU/L), respectively. The animal tested negative fordistemper, leishmaniasis, toxoplasmosis and neosporosis. Abdominal ultrasound and thoracic radiography were performedto investigate neoplasia, as well as radiography of the temporomandibular joints, and none of these imaging tests revealedany alterations. A cerebrospinal fluid (CSF) analysis was also within the normal range of reference parameters. In view ofthe possibility of idiopathic trigeminal neuropathy, prednisolone...