Task force of the Brazilian Society of Otology — evaluation and management of peripheral facial palsy

Abstract Objective To review key evidence-based recommendations for the diagnosis and treatment of peripheral facial palsy in children and adults. Methods Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on peripheral facial palsy were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results The topics were divided into 2 main parts: (1) Evaluation and diagnosis of facial palsy: electrophysiologic tests, idiopathic facial palsy, Ramsay Hunt syndrome, traumatic peripheral facial palsy, recurrent peripheral facial palsy, facial nerve tumors, and peripheral facial palsy in children; and (2) Rehabilitation procedures: surgical decompression of the facial nerve, facial nerve grafting, surgical treatment of long-term peripheral facial palsy, and non-surgical rehabilitation of the facial nerve. Conclusions Peripheral facial palsy is a condition of diverse etiology. Treatment should be individualized according to the cause of facial nerve dysfunction, but the literature presents better evidence-based recommendations for systemic corticosteroid therapy.

Clinical analysis of chronic natural killer cell lymphoproliferative disease associated peripheral neuropathy / 中华神经科杂志

Objective:To investigate the clinical characteristics, diagnosis and treatment of chronic natural killer cell lymphoproliferative disease (CLPD-NK) associated peripheral neuropathy.Methods:The clinical data of 1 case of CLPD-NK associated peripheral neuropathy who was hospitalized in Beijing Chaoyang Hospital on March 17, 2022 were reported. The clinical characteristics, laboratory examinations, nerve biopsy, treatment and prognosis of CLPD-NK associated peripheral neuropathy were summarized in combination with domestic and foreign literature reports.Results:This patient was a 62-year-old woman with progressive weakness and numbness of limbs. Nerve conduction test revealed peripheral nerves injures of limbs. No abnormality was found in a routine biochemistry test of cerebrospinal fluid. Lymphocyte infiltration was found in the nerve bundle of sural nerve with CD56(+), TIA-1(+). After prednisone combined with cyclophosphamide treatment, her weakness and numbness of limbs were relieved, and the white blood cells count decreased. A total of 10 cases of CLPD-NK associated peripheral neuropathy were summarized, including 5 males and 5 females. The median onset age was 64 years. The peripheral blood white cells count was 5.50×10 9/L-24.66×10 9/L, the lymphocytes count was 1.54×10 9/L-32.00×10 9/L, and cerebrospinal fluid protein increased in 3 cases. Biopsy of sural nerve showed NK cell infiltration in all patients. Immunosuppression was the main treatment, and most patients had a good prognosis. Conclusions:CLPD-NK associated peripheral neuropathy is rare. Peripheral nerve biopsy is the important diagnostic method. Corticosteroids and immunosuppressants are effective for this disease.

Anatomy and evaluation of the optic nerve head / Anatomia e avaliação da cabeça do nervo óptico

ABSTRACT Evaluation of the optic disc is important for the correct diagnosis and follow-up of optic neuropathies, especially glaucoma. The characteristics of the optic disc depend on various factors, including demographic and population aspects, and analysis of these characteristics may vary according to the methods used. The size and format of the neural rim along with the nerve fiber layer are important to the clinician's judgment regarding the susceptibility of the subject to develop glaucoma. In this study, we reviewed the literature to summarize the main methods and its characteristics in the evaluation of the optic nerve head.

RESUMO A avaliação do disco óptico é de suma importância para o diagnostico correto e acompanhamento de neuropatias ópticas, especialmente o glaucoma. Características do disco óptico dependem de uma grande variedade de fatores, incluindo aspectos demográficos e populacionais, e também podem variar de acordo com os métodos usados. Tamanho e formato da rima neurorretiniana junto com a camada de fibras nervosas são importantes ao julgamento clinico a respeito da susceptibilidade do paciente desenvolver glaucoma. Nesse estudo, nós revisamos a literatura para resumir os principais métodos e suas características para a avaliação da cabeça do nervo óptico.

Iridochorioretinal coloboma associated with buried optic nerve drusen: a case report / Coloboma irido-coriorretiniano associado a drusas enterradas do nervo óptico: Relato de caso

ABSTRACT Improper closure of the embryonic fissure results in ocular coloboma. Optic nerve head drusen are hyaline deposits located anterior to the lamina cribosa that grow and calcify over time. It is rarely associated with ocular coloboma, with only two cases reported. We present a patient with an irido-chorioretinal coloboma, poorly defined optic nerve limits in the right eye, and increased papillary vascular ramification and peripapillary atrophy in the left eye, without any visible drusen. Fundus autofluorescence, high-resolution optical coherence tomography, and B-scan ultrasonography confirmed the diagnosis of bilateral buried optic nerve head drusen. The association between irido-chorioretinal colobomas and optic nerve drusen in the absence of a systemic disease is exceptional. Our case demonstrates that multimodal imaging is important to correctly diagnose buried optic nerve head drusen.

RESUMO O coloboma ocular é o resultado de um fechamento impróprio da fissura embrionária. As drusas da cabeça do nervo óptico são depósitos hialinos localizados anteriormente à lâmina cribosa que crescem e se calcificam com o tempo. A associação de ambos é rara, com apenas dois casos descritos na literatura. Apresentamos um paciente com coloboma irido-coriorretiniano e limites do nervo óptico mal definidos em seu olho direito, e aumento da ramificação vascular papilar e atrofia peripapilar em seu olho esquerdo, sem drusas visíveis. Autofluorescência de fundo, tomografia de coerência óptica de alta resolução e ultrassonografia B-scan foram realizadas confirmando o diagnóstico de drusas enterradas bilaterais da cabeça do nervo óptico. A associação entre colobomas irido-coriorretinianos com drusas do nervo óptico na ausência de doença sistêmica é excepcional. Nosso caso demonstra a importância da imagem multimodal para o diagnóstico correto de drusas enterradas de cabeça do nervo óptico.

Parálisis del nervio motor ocular externo luego de anestesia neuroaxial combinada epidural-espinal / Paralysis of the external oculomotor nerve after combined epidural-spinal neuroaxial anesthesia

Introducción: El abordaje del espacio subaracnoideo fue descrito por Quincke en el 1891. En la actualidad es práctica común para la realización de la anestesia neuroaxial subaracnoidea en las pacientes obstétricas. Las complicaciones descritas, asociadas a esto, son varias. Dentro de estas, la parálisis del nervio abducens o VI par no es frecuente y en ocasiones, no está relacionada a la punción ya que se produce días después del evento. Objetivo: Revisar la información relacionada con la complicación de parálisis del VI par. Presentación del caso: Paciente de 33 años de edad, femenina, de profesión médico, con antecedentes personales de migraña, historia de anestesia neuroaxial epidural sin complicaciones, que para la realización de una cesárea de segmento arciforme y salpinguectomia parcial bilateral, recibió una anestesia combinada peridural-espinal. El transoperatorio transcurre con estabilidad hemodinámica, hizo cefalea al tercer día del posoperatorio, que la atribuyó al antecedente de migraña y fue tratada sin evaluación por anestesiología con dipirona. A los 10 días de operada hace desviación de la mirada y diplopia, se diagnostica parálisis del VI par. Fue tratada por Neurología y se plantean varios diagnósticos diferenciales. Los estudios imagenológicos resultan negativos, se trató con vitaminas y se produjo remisión a las 6 semanas. Conclusiones: El diagnóstico de esta complicación es necesario ya que puede pasar inadvertida la relación con la anestesia y, por tanto, ser mal conducido su tratamiento(AU)

Introduction: The approach to the subarachnoid space was described by Quincke in 1891. It is now a common practice to perform subarachnoid neuroaxial anesthesia in obstetric patients. The complications described, associated with this, are several. Within these, the paralysis of the abducens nerve or sixth pair is not frequent and sometimes is not related to the puncture, since it occurs days after the event. Objective: To review the information related to the complication of paralysis of the sixth pair. Case presentation: A 33-year-old female patient, a physician, with a personal history of migraine, a history of epidural neuroaxial anesthesia without complications, who underwent combined epidural-spinal anesthesia for performing a cranial segment cesarean section and bilateral partial salpingectomy. The transoperative period runs with hemodynamic stability. There was headache three days after surgery, which was attributed to the migraine history and the patient was treated, without evaluation by anesthesiology, with dipyrone. At 10 days after surgery, the eyes are diverted and diplopia is manifested, paralysis of the sixth pair is diagnosed. She was treated by neurology and several differential diagnoses were proposed. Imaging studies are negative. She was treated with vitamins and remission occurred at six weeks. Conclusions: The diagnosis of this complication is necessary, since the relationship with anesthesia may go unnoticed and, therefore, its treatment may be poorly conducted(AU)

Safety and efficacy of microvascular decompression in elderly patients with cranial neuropathy / 中华老年医学杂志

Objective To investigate the safety and clinical efficacies of microvascular decompression(MVD) on cranial neuropathy such as trigeminal neuralgia (TN),hemifacial spasm (HFS)and glossopharyngeal neuralgia(GN)in elderly patients.Methods Clinical data of 72 patients aged 70 years and over diagnosed as cranial neuropathy and undergoing microvascular decompression in our department from January 2015 to August 2017 were retrospectively analyzed.The curative effect and complications were analyzed after treatment.Results All patients successfully underwent MVD under general anesthesia.The facial pain completely disappeared or was significantly improved in 85.7 ％ (30/35)of TN patients one year after operation.The intermittent tonic contractions of the facial muscles were completely disappeared in 94.3％ (33/35)of HFS patients.Clinical symptoms were completely disappeared in 2 patients with GN,4 patients with TN and HFS and 1 patients with TN and GN.Conclusions The surgery goes well and the patients have good postoperative recovery,with no severe complications such as intracranial hematoma,infarction,cerebrospinal fluid leakage,acute hydrocephalus and death.After an adequate preoperative evaluation,MVD is safe and effective with a few complications.It can significantly improve the quality of life in elderly patients with trigeminal neuralgia,hemifacial spasm,and glossopharyngeal neuralgia.

Recurrent Painful Ophthalmoplegic Neuropathy: a Case Report

Upon review, it is noted that recurrent painful ophthalmoplegic neuropathy (RPON) is a rare neurological syndrome characterized by recurrent unilateral headaches and painful ophthalmoplegia of the ipsilateral oculomotor nerve. As seen on brain MRI, thickening and enhancement of the oculomotor cranial nerve can be observed in these cases. We experienced a case of RPON in an adult patient who showed thickening and enhancement of the oculomotor nerve on gadolinium-enhanced 3D-FLAIR image. The authors report a case of RPON with a review of the literature.

Pituitary Apoplexy Presenting as Isolated Bilateral Oculomotor Nerve Palsy

PURPOSE: To report a case of pituitary apoplexy presenting as isolated bilateral oculomotor nerve palsy. CASE SUMMARY: A 46-year-old male presented with bilateral ptosis and acute severe headaches for 6 days. He underwent head surgery and bilateral vitrectomy 12 years prior to his visit because of ocular and head trauma. He mentioned that previous visual acuities in both eyes were not good. The initial corrected visual acuity was finger counting in the right eye and 20/500 in the left eye. Ocular motility testing revealed the limitation of adduction, supraduction, and infraduction with complete bilateral ptosis in both eyes, and his left pupil was dilated. He was diagnosed with an isolated bilateral oculomotor nerve palsy. Magnetic resonance imaging indicated pituitary gland hemorrhage with a tumor, which was suspicious of pituitary apoplexy. The patient was treated intravenous with 1.0 g methylprednisolone to prevent the corticotropic deficiency. In addition, he underwent surgical decompression using a navigation-guided transsphenoidal approach and aspiration biopsy. He was confirmed with pituitary adenoma using a pathological examination. The patient's ocular movements began to dramatically improve by the third day postoperatively. At 4 months postoperative follow-up, his ocular movement and double vision were completely recovered. CONCLUSIONS: This was a rare case of pituitary apoplexy with bilateral isolated oculomotor nerve palsy, which was the first report in the Republic of Korea. A full recovery was achieved after early surgical treatment.

Clinical application of post mastoid transverse incision in keyhole microvascular decompression / 中华神经医学杂志

Objective To explore the clinical value of post mastoid transverse incision in keyhole microvascular decompression (MVD) for cranial neuropathy.Methods Fifty-eight patients with trigeminal neuralgia,hemifacial spasm or glossopharyngeal neuralgia,admitted to and accepted postmastoid transverse incision keyhole surgery in our hospital from October 2015 to October 2017,were chosen.Their clinical data and efficacy were retrospectively analyzed.Results Lesions of cranial nerves in all 58 patients were exposed satisfactorily (trigeminal nerve,facial nerve,or glossopharyngeal nerve).Postoperative complications included severe facial numbness in one patient,scalp hydrops in one patient,ear discomfort in two patients,and hearing loss in two patients.No cerebrospinal fluid leakage or intracranial infection,no facial paralysis or ear deafness,no hoarseness or drinking cough,and no intracranial hemorrhage or death were observed.All patients were followed up for 3-24 months,enjoying total effective rate of 98.3％ (57/58);and no recurrence or aggravation was noted.Conclusion MVD of post mastoid transverse incision in keyhole is a safe and effective surgical method for treatment of cranial nerve disorders;the steps of craniotomy and craniotomy in this method are simple,easy accessed,and fully neurologically exposed,having high surgical safety and good postoperative cosmetic results,which is worth of promoting application.

Anconeus Epitrochlearis Muscle as a Cause of Ulnar Nerve Compression at the Elbow

Ulnar nerve entrapment is the second most common compressive neuropathy in the upper limb, after carpal tunnel syndrome (Dellon, 1986). One of the causes that must be considered is the accessory anconeus epitrochlearis muscle, which is present in 4% to 34% of the general population (Husarik et al, 2010; Vanderpool et al, 1968; Nellans et al, 2014). We describe a patient with symptoms of compression of the left ulnar nerve at the elbow and the result of the surgical treatment. The patient presented with hypoesthesia in the fourth and fifth fingers of the left hand, and reduction of strength in the fifth finger abduction. No alterations were found in the thumb adduction. Initially, the treatment was conservative (splint, physiotherapy, analgesics); surgical treatment was indicated due to the continuity of the symptoms. The ulnar nerve was surgically released and transposed, with complete recovery after 6 months of follow-up. Ulnar nerve entrapment at the elbow by the anconeus epitrochlearis muscle is not common, but it must not be ignored (Chalmers, 1978). Ultrasonography (Jung et al, 2013; Bargalló et al, 2010), elbow magnetic resonance imaging (MRI) (Jeon, 2005), and electromyography (Byun, 2011) can help establish the proper diagnosis.

A compressão do nervo ulnar é a segunda causa mais frequente de neuropatia compressiva no membro superior, após a síndrome do túnel do carpo (Dellon, 1986). Uma das causas que dever ser considerada é a presença do músculo anconeu epitroclear, que está presente em cerca de 4% a 34% da população (Husarik et al, 2010; Vanderpool et al, 1968; Nellans et al, 2014). Descrevemos uma paciente com sintomas de compressão do nervo ulnar esquerdo no cotovelo, e o resultado do tratamento cirúrgico. A paciente apresentava hipoestesia no IV e V dedos da mão esquerda, e diminuição de força na abdução do V dedo; não foram encontradas alterações na adução do polegar. Inicialmente, o tratamento foi conservador (uso de splint, fisioterapia e analgésicos); a cirurgia foi indicada pela persistência dos sintomas. O nervo ulnar foi cirurgicamente liberado e transposto, com melhora total dos sintomas após 6 meses de acompanhamento. A compressão do nervo ulnar no cotovelo não é comum, mas não deve ser ignorada (Chalmers, 1978). Ultrassonografia (Jung et al, 2013; Bargalló et al, 2010), ressonância magnética do cotovelo (Jeon, 2005) e eletromiografia (Byun, 2011) auxiliam no diagnóstico.

The status and progress of morning glory syndrome / 中华眼底病杂志

Morning glory syndrome (MGS) is a congenital optic disc anomaly. The characteristic ophthalmoscopic findings consist of a generally enlarged, funnel-shaped and excavated optic disc, surrounded by an elevated annulus of chorioretinal pigment disturbance, with a central glial tuft, multiple narrow branches of retina vessels radiating from the disc. There are peripheral non-perfusion retinal areas in most cases. The pathogenesis of MGS remains unclear. MGS might be associated with many ocular and systemic abnormalities, involving facial, central nervous, cerebrovascular and endocrine systems. Persistent hyperplastic primary vitreous and retinal detachments (RD) are the most common ocular complications of MGS. The mechanism RD in MGS is unclear. Vitrectomy with long-acting gas or silicone tamponade and photocoagulation around the breaks or the enlarged disc might be efficient for rhegmatogenous RD of MGS. Early diagnosis is crucial for recognition and treatment of the ocular and systemic complications, and maintenance of the visual function.

The status and progress of morning glory syndrome / 中华眼底病杂志

Morning glory syndrome (MGS) is a congenital optic disc anomaly. The characteristic ophthalmoscopic findings consist of a generally enlarged, funnel-shaped and excavated optic disc, surrounded by an elevated annulus of chorioretinal pigment disturbance, with a central glial tuft, multiple narrow branches of retina vessels radiating from the disc. There are peripheral non-perfusion retinal areas in most cases. The pathogenesis of MGS remains unclear. MGS might be associated with many ocular and systemic abnormalities, involving facial, central nervous, cerebrovascular and endocrine systems. Persistent hyperplastic primary vitreous and retinal detachments (RD) are the most common ocular complications of MGS. The mechanism RD in MGS is unclear. Vitrectomy with long-acting gas or silicone tamponade and photocoagulation around the breaks or the enlarged disc might be efficient for rhegmatogenous RD of MGS. Early diagnosis is crucial for recognition and treatment of the ocular and systemic complications, and maintenance of the visual function.

Mechanism of CCL2/MCP-1 in its relevant diseases / 中国药理学通报

Chemokine is a small protein which plays an impor-tant role in men's physiological function.It has chemotactic ac-tivity and is often secreted by immune cells and glial cells like microglia or astrocytes.Through the effect of chemokine recep-tors on target cells,various immune cells can achieve directional migration and play an important role in the diseases related with immunity and inflammation.CCL2,also known as monocyte chemotactic protein-1 (MCP-1 ),is one member of chemokine CC subfamily (βsubfamily).It can chemokine monocytes, macrophages and T lymphocytes to affect their phagocytosis func-tion and produce antibodies to combat invading microorganisms. In recent years,it has been found that CCL2 plays a key role in the occurrence and development of the problems concerning cen-tral nervous system and immune system as well as cancer, AIDS,leukemia,diabetes and other diseases.This thesis is to give an elaboration on the latest research on CCL2 and the rele-vant diseases.

Sixth Cranial Nerve Palsy Caused by Gastric Adenocarcinoma Metastasis to the Clivus

Tumors of the clivus and metastases to the clivus are very rare. Metastasis involving the clivus has previously been described in only two case reports. In skull metastasis, the breast and prostate are the most common primary foci, while metastasis from gastric carcinoma is extremely rare. A review of the English literature revealed only one published case of clivus metastases from gastric adenocarcinoma. There is no literature thoroughly explaining the differential diagnosis between chordoma and metastasis. Here we report a rare case of metastasis to the clivus from a gastric adenocarcinoma in a 42-year-old female patient with sudden blurry vision, presenting as bilateral cranial nerve VI palsy.

Beyond ten cycles of cabazitaxel for castrate-resistant prostate cancer.

Background: There are limited data regarding cabazitaxel use beyond 10 cycles. Patients and Methods: Retrospective analysis of prospectively collected data of patients with metastatic castrate-resistant prostate cancer who received over 10 cycles of cabazitaxel after docetaxel failure. Results: Four patients received between 14 and 27 cycles. Reasons for stopping cabazitaxel were toxicity (2), progression (1) and logistics (1). Two of the three patients with measurable disease attained a partial remission (PR). Three patients continued to have a PSA response after 10 cycles; PSA nadir occurred between 17 and 23 cycles. Other than peripheral neuropathy (PN), all the cabazitaxel-related toxicities occurred after the initial cycles and did not increase cumulatively. Clinically significant neuropathy occurred after 15-17 cycles. The cabazitaxel-induced PN was partially reversible, with improvement from grade 3 to grade 2 after a 3-5-month long drug holiday. Conclusion: Cautiously continuing cabazitaxel until progression or intolerable toxicity may maximize efficacy.

Immunization and glaucoma / 中华实验眼科杂志

Glaucoma,the major cause of global irreversible blindness,is a chronic neurodegenerative disease of the optic nerve.Apoptosis of retinal ganglion cells (RGCs) and progressive loss of optic nerve axons results in structural and functional deficits in glaucoma patients.Growing evidence obtained from clinical and experimental studies over the last decade strongly suggests the involvement of the immune system in the neurodegenerative process of glaucoma.This review aims to provide a perspective on the complex interplay of cellular events during glaucomatous neurodegeneration that involves aberrances or dysfunctions of immune system,such as ocular immune privilege,glial activation response,T cell-mediated immune responses,autoantibody-mediated immune responses,complement fixation reaction and aging,oxidative stress.The complex interplay of cellular events amplify the primary injury process and contribute to disease progression by oxidative stress and immune response,ultimately lead to cell death with loss of RGCs.

Glufosinate Herbicide Intoxication Causing Unconsciousness, Convulsion, and 6th Cranial Nerve Palsy

Although glufosinate ammonium herbicides are considered safe when used properly, ingestion of the undiluted form can cause grave outcomes. Recently, we treated a 34-yr-old man who ingested glufosinate ammonium herbicide. In the course of treatment, the patient developed apnea, mental deterioration, and sixth cranial nerve palsy; he has since been discharged with full recovery after intensive care. This case report describes the clinical features of glufosinate intoxication with a focus on sixth cranial nerve palsy. Our observation suggests that neurologic manifestations after ingestion of a "low-grade toxicity herbicide" are variable and more complex than that was previously considered.

A Patient Presented With Unilateral Abducens Nerve Palsy: A Variant Form of Guillain-Barre Syndrome With Anti-GT1a Antibody

No abstract available.

Ramsay-Hunt Syndrome Without Vesicle Presenting With Ulilateral Abducens and Vestibular Paralysis Followed by V, VII, IX, and X Nerve Palsies

No abstract available.

Ramsay-Hunt Syndrome Without Vesicle Presenting With Ulilateral Abducens and Vestibular Paralysis Followed by V, VII, IX, and X Nerve Palsies

No abstract available.