The glossopharyngeal nerve (IX cranial nerve pair): anatomical considerations and neuralgia: literature review / O nervo glossofaringeo (IX par de nervo craniano): considerações anatômicas e neuralgia: revisão da literatura

The glossopharyngeal nerve (IX cranial nerve) is a mixed nerve, with both motor and sensory function. This relates to the tongue and pharynx. Glossopharyngeal neuralgia is a rare nervous neuropathy, with poristic, lancinating and paritary crises, usually unilateral. The aim of the study was to review the literature on glossopharyngeal neuralgia of the nerve (IX cranial nerve), highlighting the anatomical aspects of this nerve and the possible causes and complications of neuralgia as well as forms of treatment. A literature review was carried out in the international Pubmed database. The literature review included 72 articles from 2015 to 2021. The keywords used were: "anatomy of glossopharyngeal neuralgia". Of the 72 articles, 7 were used for this literature review. Uncommon as nervous/glossophingeal etiologies and pathologies are neurological abnormalities/neurovarises and pathologies are neurovascular/neurovariseal lesions. Pharmacological treatment approaches mentioned in the literature were therapy with antiepileptics and antidepressants such as carbamazepine and gabapentin; a microvascular decompression; and gamma knife radiosurgery(AU)

O nervo glossofaríngeo (IX par de nervo craniano) é um nervo misto, contendo função tanto motora como sensitiva. Este nervo relaciona-se com a língua e com a faringe. A neuralgia do nervo glossofaríngeo é uma neurapatia rara, sendo caracterizada por crises dolorosas, lancinantes e paroxísticas, geralmente unilaterais. O objetivo do estudo foi realizar uma revisão de literatura sobre a neuralgia do nervo glossofaríngeo (IX par de nervo craniano), destacando os aspectos anatômicos deste nervo e as possíveis causas e complicações da neuralgia bem como formas de tratamento. Foi realizada uma revisão da literatura na base de dados internacional Pubmed. A revisão da literatura incluiu 72 artigos no período de 2015 a 2021. As palavras-chave utilizadas foram: "anatomia da neuralgia do glossofaríngeo". Dos 72 artigos, 7 foram utilizados para esta revisão de literatura. Verificouse que a neuralgia do nervo glossofaríngeo é incomum e as etiologias mais encontradas foram compressão neurovascular/variações vasculares, patologias e traumas. As abordagens dos tratamentos mencionadas na literatura foram a terapia farmacológica da área com antiepilépticos e antidepressivos, como carbamazepina e gabapentina; a descompressão microvascular; e radiocirurgia com faca gama(AU)

Comparative anatomical studies on the cranial nerves of the fully formed embryos of the Nile tilapia Oreochromis niloticus (Ostiechthyes-Cichlidae). I. Nervus glossopharyngeus / Estudos anatômicos comparativos dos nervos cranianos de embriões totalmente formados da tilápia do Nilo Oreochromis niloticus (Ostiechthyes-Cichlidae). I. Nervus glossopharyngeus

The organization of the roots, ganglia and the peripheral distribution of the cranial nerves of the fully formed embryos of Oreochromis niloticus are examined in the transverse serial sections. These nerves carry fibers, which were also analyzed. The results of this study demonstrated that the glossopharyngeal nerve originates by means of only one root, which leaves the cranium through the glossopharyngeal foramen. This nerve gives fibers (visceromotor) to the first internal and external levator arcus branchialis muscles. There is a single epibranchial (petrosal) ganglion located extracranially. Nervus glossopharyngeus has three rami; pharyngeus, pretramticus and posttrematicus. The ramus pharyngeus carries only viscerosensory fibers; general for the pharyngeal epithelium and special ones for the pseudobranch. General viscerosensory fibers are also carried by rami pretrematicus and posttrematicus for the pharyngeal epithelial lining. The special sensory fibers are carried by the ramus pretrematicus for the taste buds and by ramus posttrematicus for the gill filaments. The ramus pretrematicus also carries visceromotor fibers for the first adductor arcus branchialis and to the first obliquus ventralis muscles.

A organização das raízes, gânglios e a distribuição periférica dos nervos cranianos dos embriões totalmente formados de Oreochromis niloticus são examinados nas seções transversais seriais. Esses nervos carregam fibras, que também foram analisadas. Os resultados deste estudo demonstraram que o nervo glossofaríngeo se origina por meio de apenas uma raiz, que sai do crânio pelo forame glossofaríngeo. Este nervo fornece fibras (visceromotoras) para os primeiros músculos levantadores do arco branquial interno e externo. Existe um único gânglio epibranquial (petroso) localizado extracranialmente. Nervus glossopharyngeus tem três ramos; faríngeo, pretramticus e póstrematicus. O ramo faríngeo contém apenas fibras viscerossensoriais ­ gerais para o epitélio faríngeo e especiais para o pseudobrânquio. Fibras viscerossensoriais gerais também são transportadas por ramos pretrematicus e posttrematicus para o revestimento epitelial da faringe. As fibras sensoriais especiais são transportadas pelo ramus pretrematicus para as papilas gustativas e pelo ramus posttrematicus para os filamentos branquiais. O ramo pretrematicus também carrega fibras visceromotoras para o primeiro adutor arcus branchialis e para o primeiro músculo oblíquo ventral.

Comparative anatomical studies on the cranial nerves of the fully formed embryos of the Nile tilapia Oreochromis niloticus (Ostiechthyes-Cichlidae). I. Nervus glossopharyngeus / Estudos anatômicos comparativos dos nervos cranianos de embriões totalmente formados da tilápia do Nilo Oreochromis niloticus (Ostiechthyes Cichlidae). I. Nervus glossopharyngeus

The organization of the roots, ganglia and the peripheral distribution of the cranial nerves of the fully formed embryos of Oreochromis niloticus are examined in the transverse serial sections. These nerves carry fibers, which were also analyzed. The results of this study demonstrated that the glossopharyngeal nerve originates by means of only one root, which leaves the cranium through the glossopharyngeal foramen. This nerve gives fibers (visceromotor) to the first internal and external levator arcus branchialis muscles. There is a single epibranchial (petrosal) ganglion located extracranially. Nervus glossopharyngeus has three rami; pharyngeus, pretramticus and posttrematicus. The ramus pharyngeus carries only viscerosensory fibers; general for the pharyngeal epithelium and special ones for the pseudobranch. General viscerosensory fibers are also carried by rami pretrematicus and posttrematicus for the pharyngeal epithelial lining. The special sensory fibers are carried by the ramus pretrematicus for the taste buds and by ramus posttrematicus for the gill filaments. The ramus pretrematicus also carries visceromotor fibers for the first adductor arcus branchialis and to the first obliquus ventralis muscles.

A organização das raízes, gânglios e a distribuição periférica dos nervos cranianos dos embriões totalmente formados de Oreochromis niloticus são examinados nas seções transversais seriais. Esses nervos carregam fibras, que também foram analisadas. Os resultados deste estudo demonstraram que o nervo glossofaríngeo se origina por meio de apenas uma raiz, que sai do crânio pelo forame glossofaríngeo. Este nervo fornece fibras (visceromotoras) para os primeiros músculos levantadores do arco branquial interno e externo. Existe um único gânglio epibranquial (petroso) localizado extracranialmente. Nervus glossopharyngeus tem três ramos; faríngeo, pretramticus e póstrematicus. O ramo faríngeo contém apenas fibras viscerossensoriais — gerais para o epitélio faríngeo e especiais para o pseudobrânquio. Fibras viscerossensoriais gerais também são transportadas por ramos pretrematicus e posttrematicus para o revestimento epitelial da faringe. As fibras sensoriais especiais são transportadas pelo ramus pretrematicus para as papilas gustativas e pelo ramus posttrematicus para os filamentos branquiais. O ramo pretrematicus também carrega fibras visceromotoras para o primeiro adutor arcus branchialis e para o primeiro músculo oblíquo ventral.

Craniofacial and neurological manifestations in patients with Guillain-Barré syndrome / Manifestaciones neurológicas craneofaciales en pacientes con síndrome de Guillain-Barré

Introducción: Among the broad range of symptoms of the Guillain-Barré Syndrome (GBS), patients can present craniofacial manifestations. Consequently, the participation of the dental surgeon in the evaluation of the stomatognathic system using a multidisciplinary approach model is very important. Objective: To identify the craniofacial and neurological manifestations of the Guillain-Barré Syndrome in patients from three hospitals in the district of Lambayeque, Peru, in 2019. Material and Methods: A descriptive, prospective, and cross-sectional study was carried out in 59 patients diagnosed with GBS by means of a clinical evaluation carried out by previously calibrated neurologists (k=0.911). Additionally, an oral evaluation of the oral hygiene index, the periodontal status, and the oral pH was carried out. Data were processed using frequency distribution tables. Results: 4 4.07% of the patients presented at least one functional alteration, such as unilateral limitation for facial expressions (25.42%), dysphagia (18.64%), alteration in phonation (11.86%), and loss of the sense of taste (6.78%), showing involvement of the corresponding cranial nerves. In addition, unfavorable conditions of oral hygiene and acid pH were observed in some cases, which proved adverse for the periodontal structures of the patients. Conclusion: The study found craniofacial manifestations of GBS, such as cases of unilateral facial palsy, dysphagia, difficulty in phonation, and loss of the sense of taste, due to the alteration of the cranial nerves that command these functions. In addition, poor oral hygiene, and the presence of acidic pH in some patients created an adverse environment for the integrity of the periodontal structures

Introducción: El Síndrome de Guillain-Barré (SGB) dentro de su amplia sintomatología puede presentar manifestaciones craneofaciales, por lo que resulta importante la participación del cirujano dentista en la evaluación del sistema estomatognático desde un modelo de abordaje multidisciplinario. Objetivo: Identificar las manifestaciones neurológicas craneofaciales del Síndrome de Guillain-Barré en pacientes de tres establecimientos de salud de Lambayeque, Perú en el año 2019. Materiales y Metodos: Se realizó un estudio descriptivo, prospectivo y transversal, con 59 pacientes diagnosticados con SGB, mediante una evaluación clínica realizada por médicos neurólogos previamente calibrados (k=0,911). Adicionalmente se realizó una evaluación bucodental del índice de higiene oral, del estado periodontal y del pH bucal, siendo procesados los datos mediante tablas de distribución de frecuencias. Resultados: El 44,07% de pacientes presentaron por lo menos una alteración funcional, identificando limitación unilateral para expresiones faciales (25.42%), disfagia (18.64%), alteración en la fonación (11.86%) y pérdida del sentido del gusto (6.78%), evidenciando compromiso de los pares craneales correspondientes. Además se observaron condiciones desfavorables de higiene oral y de pH ácido en algunos casos, que resultaron adversas para las estructuras periodontales de los pacientes. Conclusión: Fueron encontradas manifestaciones craneofaciales del SGB, identificando casos con parálisis facial unilateral, disfagia, dificultad para la fonación y pérdida del sentido del gusto, debido a la alteración de los pares craneales que comandan esas funciones. Además las condiciones desfavorables de higiene oral y la presencia de pH ácido en algunos casos, propiciaron un ambiente adverso para la integridad de las estructuras periodontales.

Complex strabismus: a case report of hypoplasia of the third cranial nerve with an unusual clinical presentation / Estrabismo complexo: um caso relacionado à hipoplasia do terceiro nervo craniano com apresentação clínica incomum

ABSTRACT Congenital cranial dysinnervation disorders are a group of complex strabismus syndromes that present as congenital and non-progressive ophthalmoplegia. The genetic defects are associated with aberrant axonal targeting onto the motoneurons, development of motoneurons, and axonal targeting onto the extraocular muscles. We describe here the surgical management of a 16-year-old boy who presented with complex strabismus secondary to hypoplasia of the third cranial nerve and aberrant innervation of the upper ipsilateral eyelid.

RESUMO Os distúrbios de inervação craniana congênita en­globam um grupo de síndromes associadas a estrabismos complexos, que se apresentam como oftalmoplegia congênita e não progressiva e são frequentemente herdadas. Os defeitos dos genes estão associados a erros no desenvolvimento ou direcionamento axonal dos motoneurônios, e erros no direcionamento axonal para os músculos extraoculares. Este caso descreve o caso de um menino que apresenta estrabismo complexo secundário à hipoplasia do terceiro nervo craniano e inervação aberrante da pálpebra superior ipsilateral, bem como o resultado após a correção cirúrgica.

Anatomía radiológica de la base de cráneo y los nervios craneales parte 2: Nervios craneales / Skull base and cranial nerves radiologic anatomy part 2: Cranial nerves

Resumen: La anatomía de la base del cráneo es compleja. Numerosas estructuras neurovasculares vitales pasan a través de múltiples canales y agujeros ubicados en la base del cráneo. Con el avance de la tomografía computarizada (TC) y la resonancia magnética (RM), es posible la localización cada vez más precisa de lesiones y la evaluación de su relación con las estructuras neurovasculares adyacentes. El trayecto de los nervios craneales sigue un recorrido conocido y se transmiten a la cara y cuello por los forámenes de base de cráneo. La tomografía computada y la resonancia magnética son complementarias entre sí y, a menudo, se usan juntas para demostrar la extensión total de la enfermedad. La segunda parte de esta revisión se centra en el estudio radiológico de los nervios craneales.

Abstract: The skull base anatomy is complex. Many vital neurovascular structures course through the skull base canals and foramina. With the advancement of CT and MRI, the localization of lesions has become more precise as their relationship with adjacent neurovascular structures. There is a known course of the cranial nerves as well as their skull base exiting foramina to the head and neck. CT and MRI are complimentary modalities and are often used together to map the full extent of disease. The second article in this review focus on the radiologic study of the cranial nerves.

Abordaje retrosigmoideo para clipaje de aneurisma distal de la AICA / Retrosigmoid approach for clipping a distal aneurysm of the AICA

Introducción: Los aneurismas cerebrales de la circulación posterior, representan solo del 8 al 10 % del total de los aneurismas cerebrales, y de estos los de la arteria cerebelosa anteroinferior (AICA) son muy poco frecuentes, por lo que no hay algoritmo de manejo para estas lesiones, se requieren conocimientos anatómicos de vascular y de base de cráneo para el tratamiento de estos, hablando del abordaje quirúrgico, podemos mencionar, el extremo lateral, retrosigmoideo, orbitozigomatico y presigmoideo, que depende del tamaño y localización del aneurisma, ya sea distal o proximal. Presentamos este caso, que tratamos mediante un abordaje retrosigmoideo convencional, el cual nos ofreció un corredor quirúrgico suficiente, sin necesidad de un abordaje mas amplio, en este caso fue un aneurisma distal de la AICA, localizado en el segmento meatal, que se encontró en el complejo neurovascular medio del ángulo pontocerebeloso, muy próximo a los nervios craneales V, VII y VIII. Objetivos: Demostrar que los aneurismas distales de la AICA se pueden manejar mediante un abordaje retrosigmodeo convencional con éxito. Materiales y métodos: El paciente se colocó en posición park bench, bajo fijación esquelética de 3 puntos, con el asterion como punto mas elevado. Se realizo una incisión en forma de C en la mastoides y se realizo un abordaje dirigido hacia el ángulo pontocerebeloso. Resultados: Se logró realizar el clipaje del aneurisma de forma satisfactoria, con un buen resultado clínico del paciente, quien fue egresada a los 4 días sin déficit neurológico. Conclusión: El abordaje retrosigmoideo es una opción terapéutica para el manejo de los aneurismas ubicados en el ángulo pontocerebeloso, específicamente del AICA distal y también se puede usar incluso para los aneurismas distales del PICA.

Background: Cerebral aneurysms of the posterior circulation, representing only 8 to 10% of the total cerebral aneurysms, but the aneurysms of the anteroinferior cerebellar artery (AICA) are very rare, so there is no management algorithm for these lesions, anatomical knowledge of vascular and skull base are known for the treatment of these lesions, in relation of the surgical approach, we can identify, the far lateral, retrosigmoid, orbitozigmatic or presigmoid approaches, which depends on the size and location of aneurysm, either distal or proximal. We present this case, which we treated through a conventional retrosigmoid approach, which offers us a sufficient surgical corridor, without the need for a broader approach, in this case it was a distal AICA aneurysm, located in the meatal segment, which was found in the middle neurovascular complex of the pontocerebellar angle, very close to the cranial nerves V, VII and VIII. Objective: Demonstrate that distal aneurysms of AICA can be managed using a successful conventional retrosigmoid approach. Results: The clipping of the aneurysm was achieved satisfactorily, with a good clinical result of the patient, who was discharged at 4 days without neurological deficit. Conclusion: The retrosigmoid approach is a therapeutic option for the management of aneurysms located in the pontocerebellar angle, specifically of the distal AICA and can also be used even for distal aneurysms of the PICA.

Nervios olfatorio, trigémino, facial, glosofaríngeo y vago: vínculos con sars-cov-2 y prospectivas neurosemiológicas / Olfactory, trigeminal, facial, glossopharyngeal and vagus nerves: links with sars-cov-2 and neurosemiological prospects

Se presenta una breve revisión los nervios olfatorio, trigémino, facial, glosofaríngeo y vago, el primero funcionalmente relacionado con la inervación quimiosensitiva olfativa en la mucosa nasal, los siguientes para el registro de dolor endocraneal y para la mucosa orofaríngea, a efectos de transducción sensitiva gustativa. Estos nervios se vinculan con los síntomas en pacientes positivos para Covid-19, que manifiestan como dolor de cabeza, disosmia, anosmia, disgeusia, ageusia entre otras características neurosemiológicas. Se concluye que estas características semiológicas se puedan deber a mecanismos neurotrópicos y transinápticos por lo que se debe realizar un examen neurológico más riguroso sobre síntomas y signos de pacientes con Covid-19.

A brief review of olfactory, facial, glossopharyngeal and vagus nerves is presented, the first one functionally related to odoriferous chemosensory innervation in the nasal mucosa, the following four cranial nerves to endocranial headache and oropharyngeal mucosa for purposes of gustatory sensory transduction. These nerves are associated with symptoms in Covid-19 positive patients, which dysosmia, anosmia, dysgeusia, ageusia manifestation, among other neurosemiological characteristics. It is concluded that these semiological characteristics may be due to neurotropic and transynaptic mechanisms, therefore a more rigorous neurological examination should be performed on symptoms and signs of Covid-19 patients.

Miastenia gravis en la infancia: una serie de tres casos / Myasthenia gravis in infancy: a series of three cases

La miastenia gravis es una enfermedad neuromuscular crónica debida a deficiencia de transmisión nerviosa en la unión neuromuscular, de origen generalmente autoinmune en el adulto, que se caracteriza por grados variables de debilidad de los músculos esqueléticos del cuerpo, que aumenta durante los períodos de actividad y disminuye después de períodos de descanso. Sin embargo en la infancia cobran especial relevancia los síndromes miasténicos congénitos, que encuentran su origen en mutaciones de genes que codifican proteínas que juegan papeles clave en el mantenimiento de la transmisión neuromuscular, teniendo edad de inicio, distribución de debilidad y respuesta a tratamiento variables. Se presentan tres casos con el objetivo de describir el comportamiento clínico de la enfermedad y la utilidad de estudios complementarios ya que es de suma importancia su precoz identificación y tratamiento. Palabras claves: Miastenia gravis, test de estimulación repetitiva, ptosis palpebral, unión neuromuscular, pares craneanos

Myasthenia gravis is a chronic neuromuscular disease due to deficiency of nerve transmission in the neuromuscular junction, usually of an autoimmune origin in the adult, which is characterized by varying degrees of weakness of the skeletal muscles of the body, which increases during periods of activity and decreases after periods of rest. In childhood, however, congenital myasthenic syndromes, which find their origin in mutations of genes that encode proteins that play key roles in maintaining neuromuscular transmission, which may have a varying age of onset, distribution of weakness and response to treatment, are particularly relevant. Three cases are presented with the aim of describing the clinical presentation and course of the disease and the usefulness of complementary studies, since its early diagnosis and treatment is of paramount importance.Keywords: Myasthenia gravis, repetitive stimulation test, palpebral ptosis, neuromuscular junction, cranial pairs.

Síndrome de Tolosa-Hunt, uma oftalmoplegia dolorosa / Tolosa Hunt Syndrome, a painful ophthalmoplegia

Resumo A síndrome de Tolosa-Hunt (STH) é uma doença rara caracterizada por oftalmoplegia dolorosa unilateral de início súbito causada por uma inflamação granulomatosa inespecífica no seio cavernoso ou fissura orbital superior (ou ambos). A oftalmoparesia ocorre quando os nervos cranianos III, IV e VI são acometidos pela inflamação. Disfunções pupilares podem estar presentes e está relacionado com acometimento das fibras simpáticas que passam pelo seio cavernoso na porção da artéria carótida interna ou fibras parassimpáticas ao redor do nervo oculomotor. O acometimento do primeiro ramo do trigêmeo pode provocar parestesia território correspondente à distribuição desde ramo (testa). Raramente, pode haver extensão da inflamação para além do seio cavernoso ou fissura orbital superior podendo acometer também o nervo óptico. Há uma boa resposta com o uso de corticoides e pode haver remissões espontâneas. Recidivas ocorrem em 40% dos casos. A doença é mais comum após a segunda década de vida. Afeta ambos os gêneros de forma igualitária. O presente estudo trata-se de um relato de caso de um paciente que se apresentou com oftalmoplegia dolorosa de início súbito à direita com 4 dias de evolução seguido de amaurose ipslateral após um dia do início da dor.

Abstract Tolosa-Hunt syndrome (STH) is a rare disease characterized by sudden onset unilateral painful ophthalmoplegia caused by non-specific granulomatous inflammation in the cavernous sinus or superior orbital fissure (or both). Ophthalmoparesis occurs when the cranial nerves III, IV and VI are affected by inflammation. Pupillary dysfunctions may be present and is related to involvement of the sympathetic fibers that pass through the cavernous sinus in the portion of the internal carotid artery or parasympathetic fibers around the oculomotor nerve. The involvement of the first branch of the trigeminal can cause paresthesia corresponding to the distribution from the first branch (forehead). Rarely, there may be extension of inflammation beyond the cavernous sinus or superior orbital fissure and may also affect the optic nerve. There is a good response with the use of corticosteroids and there may be spontaneous remissions. Relapses occur in 40% of cases. The disease is most common after the second decade of life. It affects both genders equally. The present study is a case report of a patient who presented with painful ophthalmoplegia of sudden onset on the right with 4 days of evolution followed by ipsilateral amaurosis after one day of onset of pain.

Síndrome da fissura orbital superior associada a traumatismos craniomaxilofaciais / Superior orbital fissure syndrome associated with craniomaxillofacial traumatism

Introdução: A Síndrome da Fissura Orbital Superior (SFOS) é uma condição de ocorrência rara, inicialmente descrita por Hirchfield em 1858. Caracterizada pela presença de oftalmoplegia, ptose da pálpebra superior e midríase, podendo ocorrer parestesia da pálpebra superior e da região frontal, associada à lesão dos pares de nervos cranianos: oculomotor, troclear, abducente (III, IV e VI) e, por vezes, o nervo trigêmeo (V). A identificação da SFOS é importante, visto que sua incidência é rara no trauma, e sua identificação pode ajudar a direcionar o tratamento de forma mais adequada. Relato de caso: O presente trabalho descreve dois casos da SFOS associados a traumas craniofaciais, cita as possíveis etiologias relacionadas a essa síndrome e descreve a situação de trauma agudo. Considerações Finais: A avaliação das condições sistêmicas do paciente e de exames complementares, como tomografias computadorizadas, auxilia o diagnóstico diferencial entre patologias que acometem a região orbital e a base de crânio, fraturas e traumas craniofaciais. A identificação da SFOS no trauma agudo orienta a abordagem imediata ou precoce quando indicada, como nos casos de hematomas retrobulbares ou em grandes deslocamentos ósseos maxilofaciais com necessidade de redução cirúrgica. Nos casos com indicação de abordagem tardia, as fraturas craniofaciais são tratadas de forma conservadora, e o paciente é encaminhado para atendimento especializado... (AU)

Introduction: The superior orbital fissure syndrome (SOFS) is a rare condition and initially described by Hirchfield in 1858. Characterized by the presence of ophthalmoplegia, upper eyelid ptosis and mydriasis, and there may be paresthesia of the upper eyelid and forehead associated by the injury of the cranial nerves like: oculomotor, trochlear, abducens (III, IV and VI) and sometimes the trigeminal nerve (V). The identification of SOFS is important, since its incidence is rare in trauma, and its identification may help to target the treatment more adequately. Case report: The present study describes two cases of SFOS associated with craniofacial trauma, cites the possible etiologies related to this syndrome and describes the situation of acute trauma. Final considerations: The evaluation of the patient's systemic conditions and complementary exams, such as computed tomography, help the differential diagnosis between pathologies that affect the orbital region and the skull base, and fractures and traumatic head injuries. The identification of SFOS in acute trauma guides the immediate or early approach when indicated, as in cases of retrobulbar hematomas or large maxillofacial bone dislocations requiring surgical reduction. In cases with indication for late approach, craniofacial fractures are treated conservatively and the patient is referred for specialized care... (AU)

Trochlear-oculomotor synkinesis: another congenital cranial dysinnervation disorder? / Sincinesia troclear-oculomotora: outro distúrbio congênito de denervação craniana?

ABSTRACT This report documents an unusual phenomenon. A 6-year-old girl with trochlear-oculomotor synkinesis presented with superior oblique and palpebral levator co-contraction. The literature was reviewed and the possibility of classifying this entity as a congenital cranial dysinnervation disorder was speculated.

RESUMO Este relato descreve um fenômeno incomum. Uma menina de 6 anos com sincinesia troclear-oculomotora apresentou co-contração do oblíquo superior e do levantador da pálpebra. A literatura foi revisada e especulou-se a possibilidade de classificar essa desordem como um distúrbio da congenital cranial dysinnervation disorder.

Paquimeningitis hipertrófica relacionada con IgC4: comunicación de otros 11 casos y revisión de la literatura / IgG4-related hypertrophic pachymeningitis: communication of eleven other cases and literature review

La paquimeningitis es un raro desorden caracterizado por engrosamiento focal o difuso de la duramadre, siendo una potencial manifestación de la enfermedad relacionada con IgG4. Presentamos 11 pacientes, seis hombres y cinco mujeres, entre 39-73 años, que consultaron por cefalea, alteraciones visuales, acúfenos, hipoacusia, pérdida de peso, agrandamiento de glándulas salivales, dorsalgia, cuadriplejía y compromiso de nervios craneales. Algunos de ellos presentaron elevación de la proteína C reactiva o del valor de sedimentación globular, mientras que la mayoría presentó niveles séricos normales de IgG4. Todos los pacientes mostraron engrosamiento de la duramadre en la resonancia magnética. La biopsia de duramadre, de vesícula biliar, hipófisis o glándula lagrimal, mostró un infiltrado linfoplasmocitario con o sin fibrosis estoriforme, con más de 10 plasmocitos IgG4 (+) y un rango IgG4: IgG que osciló del 20 al 60 por ciento. Los pacientes recibieron prednisona sola o con rituximab, metotrexate, ciclofosfamida o azatioprina, con respuesta favorable(AU)

Pachymeningitis is a rare disorder characterized by focal or diffuse thickening of the dura mater, being a potential manifestation of the disease related to IgG4. We present 11 patients, six men and five women, aged 39-73 years, who consulted for headache, visual disturbances, tinnitus, hearing loss, weight loss, salivary gland enlargement, dorsalgia, quadriplegia and cranial nerve involvement. Some of them presented elevation of the C-reactive protein or the erythrocyte sedimentation value, while the majority had normal serum levels of IgG4. All patients showed thickening of the dura in magnetic resonance imaging. The biopsy of dura mater, gallbladder, pituitary gland or lacrimal gland showed a lymphoplasmacytic infiltrate with or without storiform fibrosis, with more than 10 IgG4 (+) plasmocytes and an IgG4: IgG range that ranged from 20 to 60 percent. Patients received prednisone alone or with rituximab, methotrexate, cyclophosphamide or azathioprine, with favorable response(AU)

Lower Cranial Nerve Palsy Due to Vertebrobasilar Dolichoectasia

No abstract available.

Pattern of Extraocular Muscle Involvements in Miller Fisher Syndrome

BACKGROUND AND PURPOSE: The most-common initial manifestation of Miller Fisher syndrome (MFS) is diplopia due to acute ophthalmoplegia. However, few studies have focused on ocular motility findings in MFS. This study aimed to determine the pattern of extraocular muscle (EOM) paresis in MFS patients. METHODS: We consecutively recruited MFS patients who presented with ophthalmoplegia between 2010 and 2015. The involved EOMs and the strabismus pattern in the primary position were analyzed. Antecedent infections, other involved cranial nerves, and laboratory findings were also reviewed. We compared the characteristics of the patients according to the severity of ophthalmoplegia between complete ophthalmoplegia (CO) and incomplete ophthalmoplegia (IO). RESULTS: Twenty-five patients (15 males and 10 females) with bilateral ophthalmoplegia were included in the study. The most-involved and last-to-recover EOM was the lateral rectus muscle. CO and IO were observed in 11 and 14 patients, respectively. The patients were aged 59.0±18.4 years (mean±SD) in the CO group and 24.9±7.4 years in the IO group (p<0.01), and comprised 63.6% and 21.4% females, respectively (p=0.049). Elevated cerebrospinal fluid protein was identified in 60.0% of patients with CO and 7.7% of patients with IO (p=0.019) for a mean follow-up time from the initial symptom onset of 3.7 days. CONCLUSIONS: The lateral rectus muscle is the most-involved and last-to-recover EOM in ophthalmoplegia. The CO patients were much older and were more likely to be female and have an elevation of cerebrospinal fluid protein than the IO patients.

Möbius Syndrome Demonstrated by the High-Resolution MR Imaging: a Case Report and Review of Literature

Möbius syndrome is a rare congenital condition, characterized by abducens and facial nerve palsy, resulting in limitation of lateral gaze movement and facial diplegia. However, to our knowledge, there have been few studies on evaluation of cranial nerves, on MR imaging in Möbius syndrome. Herein, we describe a rare case of Möbius syndrome representing limitation of lateral gaze, and weakness of facial expression, since the neonatal period. In this case, high-resolution MR imaging played a key role in diagnosing Möbius syndrome, by direct visualization of corresponding cranial nerves abnormalities.

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.

Ophthalmologic Clinical Features of Facial Nerve Palsy Patients

PURPOSE: To understand the ophthalmic clinical features and outcomes of facial nerve palsy patients who were referred to an ophthalmic clinic for various conditions like Bell's palsy, trauma, and brain tumor. METHODS: A retrospective study was conducted of 34 eyes from 31 facial nerve palsy patients who visited a clinic between August 2007 and July 2017. The clinical signs, management, and prognosis were analyzed. RESULTS: The average disease period was 51.1 ± 20.6 months, and the average follow-up duration was 24.0 ± 37.5 months. The causes of facial palsy were as follows: Bell's palsy, 13 cases; trauma, six cases; brain tumor, five cases; and cerebrovascular disease, four cases. The clinical signs were as follows: lagophthalmos, 24 eyes; corneal epithelial defect, 20 eyes; conjunctival injection, 19 eyes; ptosis, 15 eyes; and tearing, 12 eyes. Paralytic strabismus was found in seven eyes of patients with another cranial nerve palsy (including the third, fifth, or sixth cranial nerve). Conservative treatments (like ophthalmic ointment or eyelid taping) were conducted along with invasive procedures (like levator resection, tarsorrhaphy, or botulinum neurotoxin type A injection) in 17 eyes (50.0%). Over 60% of the patients with symptomatic improvement were treated using invasive treatment. At the time of last following, signs had improved in 70.8% of patients with lagophthalmos, 90% with corneal epithelium defect, 58.3% with tearing, and 72.7% with ptosis. The rate of improvement for all signs was high in patients suffering from facial nerve palsy without combined cranial nerve palsy. CONCLUSIONS: The ophthalmic clinical features of facial nerve palsy were mainly corneal lesion and eyelid malposition, and their clinical course improved after invasive procedures. When palsy of the third, fifth, or sixty cranial nerve was involved, the prognosis and ophthalmic signs were worse than in cases of simple facial palsy. Understanding these differences will help the ophthalmologist take care of patients with facial nerve palsy.

Apparent origin of glossopharyngeal, vagus and accessory nerves: an aspect to consider in human neuroanatomy teaching / El origen aparente de los nervios glosofaríngeo, vago y accesorio: un aspecto para considerar en la enseñanza de neuroanatomía humana

In various neuroanatomy texts and articles related to this area of knowledge, there is a conceptual vacuum associated with the precise sites where the roots of the cranial nerves emerge. The objective of the study was to establish the exact location of the apparent origin of the glossopharyngeal, vagus and accessory cranial nerves in the medulla oblongata of the human being 120 human brainstems, previously fixed in formalin solution at 10 % were assessed, the location where such nerve roots emerge was identified by direct examination and once the piamater was removed at both right and left sides as it has been stated in the literature. It was found that in 100 % of the studied brainstems their nerve roots emerge on average at about 2.63 mm behind the retro-olivary groove, different to what has been stated in the literature. Glossopharyngeal, vagus and accessory human nerves do not emerge directly from the retroolivary groove, as commonly reported; instead, they emerge behind the said groove, specifically in the retro-olivary groove area, where they form a continuous line of nerve roots.

En diversos textos de neuroanatomía y artículos relacionados con esta área del conocimiento, se evidencia un vacío conceptual asociado con los sitios precisos por donde emergen los pares craneales. El objetivo de este estudio fue stablecer la ubicación exacta del origen aparente de los nervios craneales glosofaríngeo, vago y accesorio en el bulbo raquídeo de 120 tallos cerebrales humanos, previamente fijados en solución de formalina al 10 %. Fueron evaluados, el lugar donde surgen tales raíces nerviosas se identificó mediante examen directo y una vez que se retiró la piamadre tanto en el lado derecho como en el izquierdo como se ha dicho en la literatura. Se encontró que en el 100 % de los troncos cerebrales estudiados, sus raíces nerviosas emergen en promedio a unos 2,63 mm detrás del surco retroolivar, diferente a lo que se ha dicho en la literatura. Los nervios humanos glosofaríngeos, vago y accesorio no emergen directamente de la ranura retroolivar, como se informa comúnmente, sino que emergen detrás de dicha ranura, específicamente en el área de surco retroolivar, donde forman una línea continua de raíces nerviosas.

Controle neural da deglutição / Neural control of swallowing

ABSTRACT BACKGROUND: Swallowing is a motor process with several discordances and a very difficult neurophysiological study. Maybe that is the reason for the scarcity of papers about it. OBJECTIVE: It is to describe the chewing neural control and oral bolus qualification. A review the cranial nerves involved with swallowing and their relationship with the brainstem, cerebellum, base nuclei and cortex was made. METHODS: From the reviewed literature including personal researches and new observations, a consistent and necessary revision of concepts was made, not rarely conflicting. RESULTS AND CONCLUSION: Five different possibilities of the swallowing oral phase are described: nutritional voluntary, primary cortical, semiautomatic, subsequent gulps, and spontaneous. In relation to the neural control of the swallowing pharyngeal phase, the stimulus that triggers the pharyngeal phase is not the pharyngeal contact produced by the bolus passage, but the pharyngeal pressure distension, with or without contents. In nutritional swallowing, food and pressure are transferred, but in the primary cortical oral phase, only pressure is transferred, and the pharyngeal response is similar. The pharyngeal phase incorporates, as its functional part, the oral phase dynamics already in course. The pharyngeal phase starts by action of the pharyngeal plexus, composed of the glossopharyngeal (IX), vagus (X) and accessory (XI) nerves, with involvement of the trigeminal (V), facial (VII), glossopharyngeal (IX) and the hypoglossal (XII) nerves. The cervical plexus (C1, C2) and the hypoglossal nerve on each side form the ansa cervicalis, from where a pathway of cervical origin goes to the geniohyoid muscle, which acts in the elevation of the hyoid-laryngeal complex. We also appraise the neural control of the swallowing esophageal phase. Besides other hypotheses, we consider that it is possible that the longitudinal and circular muscular layers of the esophagus display, respectively, long-pitch and short-pitch spiral fibers. This morphology, associated with the concept of energy preservation, allows us to admit that the contraction of the longitudinal layer, by having a long-pitch spiral arrangement, would be able to widen the esophagus, diminishing the resistance to the flow, probably also by opening of the gastroesophageal transition. In this way, the circular layer, with its short-pitch spiral fibers, would propel the food downwards by sequential contraction.

RESUMO CONTEXTO: A deglutição é um processo motor com muitas discordâncias e de difícil estudo quanto a sua neurofisiologia. Talvez por essa razão sejam tão raros os artigos sobre esse tema. OBJETIVO - Descrever o controle neural da mastigação e a qualificação do bolo que se obtém durante a fase oral. Revisar os nervos cranianos envolvidos com a deglutição e suas relações com o tronco cerebral, cerebelo, núcleos de base e córtex. MÉTODOS: Revisão da literatura com inclusão de trabalhos pessoais e novas observações buscando dar consistência a necessária revisão dos conceitos, muitas vezes conflitantes. RESULTADOS E CONCLUSÃO: Em relação a fase oral da deglutição consideramos o controle neural em cinco distintas possibilidades. Fase oral nutricional voluntária, fase oral cortical voluntária primaria, fase oral semiautomática, fase oral em goles subsequentes e fase oral espontânea. Em relação ao controle neural da fase faríngea da deglutição, pode-se observar que o estímulo que dispara a fase faríngea não é o toque produzido pela passagem do bolo, mas sim a distensão pressórica, tenha ou não conteúdo em passagem. Na deglutição nutricional, alimento e pressão são transferidos, mas na fase oral da deglutição primária cortical somente pressão é transferida e temos resposta faríngea similar a nutricional. A fase faríngea incorpora como parte de sua dinâmica as atividades orais já em curso. A fase faríngea se inicia por ação do plexo faríngeo composto pelos nervos glossofaríngeo (IX), vago (X), e acessório (XI), com envolvimento do trigêmeo (V), do facial (VII), glossofaríngeo (IX) e hipoglosso (XII). O plexo cervical (C1, C2), e o nervo hipoglosso, a cada lado, formam a alça cervical de onde, com origem cervical, um ramo segue para o músculo gênio-hioide, um músculo que atua na dinâmica de elevação do complexo hiolaríngeo. Foi também considerado o controle neural da fase esofágica da deglutição. Além de outras hipóteses foi considerado que é possível que a camadas musculares consideradas como longitudinal e circular para o esôfago sejam a longitudinal composta por fibras espirais de passo longo e a circular por fibras espirais de passo curto. Essa morfologia associada ao conceito de preservação de energia, nos permite admitir que a contração da camada longitudinal por seu arranjo espiral seja capaz de alargar o esôfago diminuindo sua resistência ao fluxo e provavelmente e também abrindo a transição esofagogástrica. Desse modo a camada circular, espiral de passo curto, pode propelir o bolo por constrição sequencial de cranial para caudal.