Lower Four Cranial Nerves in the Management of Glomus Jugulare: Anatomical Study

Abstract Introduction The surgical management that achieves minimal morbidity and mortality for patients with glomus and non-glomus tumors involving the jugular foramen (JF) region requires a comprehensive understanding of the complex anatomy, anatomic variability, and pathological anatomy of this region. Objective The aim of this study is to propose a rational guideline to expose and preserve the lower cranial nerves (CNs) in the lateral approach of the JF. Methods The technique utilized is the gross and microdissection of 4 fixed cadaveric heads to revise the JF's surgical anatomy and high part of the carotid sheath compared with surgical cases to understand and preserve the integrity of lower CNs. The method involves radical mastoidectomy, microdissection of the JF, facial nerve, and high neck just below the carotid canal and the JF. The CNs IX, X, XI, and XII are microscopically dissected and kept in sight up to the JF. Results This study realized well the surgical and applied anatomy of the lower CNs with relation to the facial nerve and JF. Conclusions The JF anatomy is complicated, and the key to safely operate on it and preserving the lower CNs is to find the posterior belly of the digastric muscle, to skeletonize the facial nerve, to remove the mastoid tip preserving the stylomastoid foramen, to skeletonize the sigmoid sinus and posterior fossa dura not only anterior but also posteroinferior to reach and drill the jugular tubercle.

Orbital Apex Syndrome – A Case Report

Orbital apex syndrome is characterized by vision loss and ophthalmoplegia due to the involvement of the orbital apex. The signs and symptoms vary depending upon the involvement of the structures within the orbital apex, the superior orbital fissure or the cavernous sinus. Clinical evaluation is the key to the diagnosis which is aided by neuro-imaging modalities including brain and orbital Magnetic Resonance Imaging and Computed Tomography scans. In rare instances, a biopsy may be needed to aid in diagnosis. Treatment depends on what the nature of the lesion.

Oftalmoplejía infecciosa en un paciente con virus de la varicela-zóster / Infectious ophthalmoplegia in patients with varicella-zoster virus

Introducción: Una de las complicaciones de la reactivación del virus de la varicela-zóster es el compromiso de los nervios craneales; sin embargo, es inusual que se presente como una oftalmoplejía completa. Objetivo: Describir el caso de un adulto inmunocompetente que desarrolló una oftalmoplejía infecciosa por reactivación del virus de la varicela-zóster. Caso clínico: El paciente presentó alteración completa de la motilidad de los músculos extraoculares del ojo izquierdo con compromiso del reflejo pupilar, disminución en la agudeza visual y neuralgia trigeminal concomitante; no tuvo signos o síntomas sugestivos de encefalitis o meningitis. Días antes de la oftalmoplejía aparecieron vesículas en la región frontal y periorbitaria izquierdas. Mediante el estudio del líquido cefalorraquídeo (LCR) con panel para meningitis/encefalitis FilmArray® se documentó positividad solo para el virus de la varicela-zóster. El paciente fue tratado con aciclovir, esteroides y neuromoduladores, con lo cual obtuvo mejoría parcial de sus síntomas a las dos semanas. La discusión se realizó a partir de los pocos reportes de casos encontrados en diferentes bases de datos. Conclusiones: Este caso amplía el entendimiento clínico y terapéutico de una manifestación inusual de esta enfermedad frecuente, que combina un compromiso patológico de varios nervios craneales por la reactivación del virus de la varicela-zóster.

Introduction: Cranial nerve involvement is one of the complications of varicella-zoster virus reactivation; however, presenting complete ophthalmoplegia is unusual. Objective: To describe the case of an immunocompetent adult who developed an infectious ophthalmoplegia due to varicella-zoster virus reactivation. Clinical case: The patient presented complete alteration of the extraocular muscle motility of the left eye with pupillary reflex compromise, decrease in visual acuity and concomitant trigeminal neuralgia. The patient did not present signs or symptoms suggestive of encephalitis or meningitis. Days before the ophthalmoplegia, vesicles appeared in the left frontal and periorbital regions. Cerebrospinal fluid (CSF) examination with FilmArray® meningitis/encephalitis panel documented positivity for varicella-zoster virus only. The patient was treated with acyclovir, steroids and neuromodulators, resulting in partial improvement of his symptoms after two weeks. The discussion was based on the few case reports found in different databases. Conclusions: This case broadens the clinical and therapeutic understanding of an unusual manifestation of this common disease, which combines pathologic involvement of several cranial nerves due to varicella-zoster virus reactivation.

Magnetic resonance imaging of the cranial nerves in infectious, neoplastic, and demyelinating diseases, as well as other inflammatory diseases: a pictorial essay / Ressonância magnética dos nervos cranianos em doenças infecciosas, neoplásicas, desmielinizantes e outras doenças inflamatórias: ensaio iconografico

Abstract The cranial nerves, which represent extensions of the functional structures of the brain, traverse the head and neck. They are connected to various cranial structures and are associated with several diseases. An in-depth understanding of their complex anatomy and normal imaging appearance allows the examiner to identify and characterize abnormalities with greater precision. One important tool for evaluating the cranial nerves is contrast-enhanced magnetic resonance imaging, especially that employing three-dimensional steady-state free precession sequences, which provide high soft-tissue and spatial resolution, despite the slen-derness of the nerves. In most cases of cranial nerve abnormalities, the imaging findings are nonspecific. Therefore, to narrow the differential diagnosis, it is necessary to take a full patient history, perform a focused physical examination, and order laboratory tests. In this pictorial essay, we review, illustrate, and discuss, from a pathophysiological perspective, infectious, neoplastic, and demyelinating disorders, as well as other inflammatory disorders, affecting the cranial nerves, the aim being to provide a practical, tangible reference for radiologists to use in daily practice.

RESUMO Os nervos cranianos, que representam extensões das estruturas funcionais do cérebro, atravessam a cabeça e o pescoço. Eles estão conectados a várias estruturas cranianas e estão associados a várias doenças. Uma compreensão profunda de sua complexa anatomia e aparência normal por imagem permite ao examinador identificar e caracterizar as anormalidades com maior precisão. Uma ferramenta importante para avaliar os nervos cranianos é a ressonância magnética com contraste, especialmente as sequências tridimensionais steady-state free precession, que proporcionam alta resolução espacial e de partes moles, apesar da fina espessura dos nervos. Na maioria dos casos, os achados radiológicos não são específicos. Para estreitar o diagnóstico diferencial é necessário fazer uma anamnese completa do paciente, realizar um exame físico dirigido e solicitar testes laboratoriais. Neste ensaio iconográfico revisamos, ilustramos e discutimos, sob uma perspectiva fisiopatológica, os distúrbios infecciosos, neoplásicos, inflamatórios e desmielinizantes, visando a ser uma referência prática e tangível para a prática diária dos radiologistas.

Varied presentations of congenital ocular synkinesis: do they all fit congenital cranial dysinnervation disorder spectrum? / Apresentações variadas de sincinesias oculares congênitas: todas elas se enquadram ao espectro das desordens congênitas da desnervação craniana?

ABSTRACT Purpose: Synkinesis results from nerve miswirings and causes aberrant movements of the affected muscles. We present a series of cases of rare congenital ocular synkinesis involving the extraocular muscles and the levator palpebrae superioris and speculate the possibility of classifying these entities in the spectrum of congenital cranial dysinnervation disorder. Methods: Records of patients with the diagnosis of congenital ocular synkinesis were analyzed retrospectively. We analyzed the sex, laterality, and complete features of the ocular motility of each patient. Results: Nine patients with congenital ocular synkinesis were included. A slight predominance of women was noted. In terms of laterality, four patients had only the right eye involved, four had only the left eye, and one had both eyes involved. Notably, 55.5% were orthotropic in the primary position. The third, fourth, and sixth cranial nerves were involved in the miswiring in 100%, 44.4%, and 11.1% of the cases, respectively. Conclusions: Congenital synkinesis might present in a very eclectic and uncommon fashion. The aberrant innervation in these cases classifies them into the group of congenital cranial dysinnervation disorders.

RESUMO Objetivo: Sincinesias são resultado de inervações anômalas e ocasionam movimentos aberrantes dos músculos envolvidos. Apresentamos uma série com casos raros de sincinesias oculares congênitas dos músculos extraoculares e do levantador da pálpebra superior e especulamos a possibilidade de classificá-las dentro do espectro das desordens congênitas da desnervação cranianana. Métodos: Prontuários de pacientes com diagnóstico de sincinesia ocular congênita foram estudados retrospectivamente. Analisamos sexo, lateralidade e as características completas do exame de motilidade de cada paciente. Resultados: Nove pacientes com sincinesias oculares congênitas foram incluídos. Houve discreta predominância no sexo feminino. Em termos de lateralidade, o olho direito foi o único envolvido em 4 casos, o olho esquerdo também em 4 casos e 1 caso apresentou acometimento bilateral. 55,5% dos pacientes eram ortotrópicos na posição primária. Os III, VI e IV nervos participaram da sincinesia em 100%, 44,4% e 11,1% dos casos, respectivamente. Conclusões: Sincinesias oculares congênitas podem se apresentar de modo bastante eclético e incomum. A inervação aberrante presente em cada um desses casos os coloca na lista de candidatos a integrar o grupo das desordens congênitas da desenervação craniana.

Magnetic resonance imaging of the cranial nerves in congenital, traumatic, and vascular diseases: a pictorial essay / Ressonância magnética dos nervos cranianos em doenças congênitas, traumáticas e vasculares: ensaio iconográfico

Abstract The cranial nerves, which represent extensions of the functional structures of the brain, traverse the head and neck. They are connected to various cranial structures and are associated with several diseases. An in-depth understanding of their complex anatomy and normal imaging appearance allows the examiner to identify and characterize abnormalities with greater precision. One important tool for evaluating the cranial nerves is contrast-enhanced magnetic resonance imaging, especially three-dimensional steady-state free precession sequences, which provide high soft-tissue and spatial resolution, despite the slenderness of the nerves. In most cases, imaging findings are nonspecific. Therefore, to narrow the differential diagnosis, it is necessary to take a full patient anamnesis, perform a focused physical examination and order laboratory tests. In this pictorial essay we review, illustrate and discuss, from a pathophysiological perspective, congenital, traumatic, and vascular diseases of the cranial nerves.

Resumo Os nervos cranianos representam extensões das estruturas funcionais do cérebro que cruzam a cabeça e o pescoço. Eles estão ligados a diferentes estruturas cranianas e se relacionam com diversas doenças. Uma compreensão profunda de sua complexa anatomia e aparência normal de imagem permite ao examinador identificar e caracterizar com mais precisão as anormalidades. A ressonância magnética com contraste é uma importante ferramenta para avaliar essas estruturas, especialmente as sequências tridimensionais de precessão livre de estado estacionário, que proporcionam alta resolução espacial e de partes moles, apesar da fina espessura dos nervos cranianos. Na maioria dos casos, os achados de imagem são inespecíficos, e uma anamnese completa, exame físico dirigido e testes laboratoriais são necessários para estreitar o diagnóstico diferencial. Este ensaio iconográfico ilustra e discute doenças congênitas, traumáticas e vasculares dos nervos cranianos, mostrando imagens em uma abordagem fisiopatológica.

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.

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.

Use of Magnetic Resonance Neurography for Evaluating the Distribution and Patterns of Chronic Inflammatory Demyelinating Polyneuropathy

OBJECTIVE: To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency.MATERIALS AND METHODS: Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2- weighted turbo spin-echo sequences were performed for neurography of the brachial and lumbosacral (LS) plexus and cauda equina, respectively. Clinical data and scores of the inflammatory Rasch-built overall disability scale (I-RODS) in CIDP were obtained.RESULTS: The bilateral extracranial vagus (n = 11), trigeminal (n = 12), and intercostal nerves (n = 10) were hypertrophic. Plexus hypertrophies were observed in the brachial plexus of 19 patients (61.3%) and in the LS plexus of 25 patients (80.6%). Patterns of hypertrophy included uniform hypertrophy (17 [54.8%] brachial plexuses and 21 [67.7%] LS plexuses), and multifocal fusiform hypertrophy (2 [6.5%] brachial plexuses and 4 [12.9%] LS plexuses) was present. Enlarged and/or contrast-enhanced cauda equina was found in 3 (9.7%) and 13 (41.9%) patients, respectively. Diameters of the brachial and LS nerve roots were significantly larger in CIDP than in controls (p < 0.001). The largest AUC was obtained for the L5 nerve. There were no significant differences in the course duration, I-RODS score, or diameter between patients with and without hypertrophy.CONCLUSION: MRN is useful for the assessment of distribution and characteristics of the peripheral nerves in CIDP. Compared to other regions, LS plexus neurography is more sensitive for CIDP.

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.

El verdadero origen aparente de los nervios glosofaríngeo, vago y accesorio / The true apparent origin of glossopharyngeal, vagus and accessory nerves

Resumen Introducción. Existe un vacío conceptual asociado con los sitios precisos por donde emergen las raíces de los nervios glosofaríngeo, vago y accesorio, un conocimiento que es de suma importancia para los neurocirujanos. Objetivo. Determinar el sitio preciso por donde las raíces de los nervios glosofaríngeo, vago y accesorio emergen como origen aparente en la médula oblongada. Materiales y métodos. Se valoraron 67 troncos encefálicos humanos que con anterioridad habían sido fijados en solución de formalina al 10%. Mediante inspección directa, luego de retirar las meninges, se examinó y registró el sitio preciso por donde emergen las raíces de tales nervios y se comparó con lo registrado en la literatura. Resultados. En el 100% de los troncos encefálicos estudiados se encontró que las raíces nerviosas emergen entre 2mm a 3mm por detrás del surco retro-olivar, distinto a lo reportado en la literatura consultada. Conclusión. Hay disparidad de criterios en cuanto al origen aparente de los nervios glosofaríngeo, vago y accesorio, lo que amerita un estudio más amplio que permita llegar a un consenso generalizado sobre el sitio preciso por donde las raíces de tales nervios hacen su aparición.

Abstract Introduction: There is a conceptual gap associated with the sites where the roots of the glossopharyngeal, vagus and accessory nerves emerge, which is information of utmost importance for neurosurgeons. Objective: To determine the exact site where the roots of the glossopharyngeal, vagus and accessory nerves emerge, considering the medulla oblongata as their apparent place of origin. Materials and methods: 67 human brainstems that had previously been fixed in 10% formalin solution were examined. After removing the meninges, the precise site where the roots of these nerves emerged was analyzed by direct inspection and the results recorded and compared to the literature. Results: In 100% of the brainstems studied, it was found that the nerve roots emerge between 2mm and 3mm behind the retro-olivary groove, which differs from the reports of the consulted literature. Conclusion: There are different opinions about the apparent origin of the glossopharyngeal, vagus and accessory nerves. This requires a deeper study that allows reaching a general consensus on the precise place where the roots of these nerves originate.

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.

Effect analysis of microvascular decompression for patients with trigeminal neuralgia caused by vertebrobasilar dolichectasia / 中国脑血管病杂志

Objective To investigate the safety and efficacy of microvascular decompression for patients with trigeminal neuralgia caused by vertebrobasilar dolichectasia ( VBD). Methods From April 2013 to August 2016, the clinical data of 13 consecutive patients with trigeminal neuralgia caused by VBD and treated with microvascular decompression at the Department of Neurosurgery,the 174th Hospital of PLA (n = 2) and the Department of Neurosurgery, Xiamen Humanity Hospital (n = 11) were enrolled retrospectively. Among them,4 were females and 9 were males,aged from 37 to 72 (mean 59 ± 10) years; the duration of the disease was 3-12 months,with a median of 36.0 (9.5,54.0) months; 1 patient was complicated with cerebral infarction before operation, 1 had transient ischemic attack;9 patients were complicated with hypertension,2 had diabetes, and 1 had coronary heart disease; 10 were treated with carbamazepine before operation, and 2 were treated with radiofrequency before operation; pain was mainly distributed in the second and third branches of trigeminal nerve. Preoperative and postoperative scores were assessed using the Baro Nervous Institute ( BNI) pain grading; preoperative head MRI was performed (including three-dimensional time of flight sequence) in order to confirm the diagnosis. Clinical follow-up was performed by outpatient and telephone after operation. Results (1) Preoperative BNI pain grading in 13 patients; grade IV in 4 cases and grade V in 9 cases. Both MRI and intraoperative findings were consistent with VBD characteristics,and the trigeminal nerve was significantly displaced by pressure. The distribution of guilty vessels was 6 cases in basilar artery,3 in basilar artery + superior cerebellar artery,and 4 in vertebral artery. Twelve patients were treated with microvascular decompression alone,and 1 was treated with microvascular decompression + selective partial posterior rhizotomy (SPPR). (2) All 13 patients completed clinical follow-up. The follow-up period ranged from 24 to 64 months, mean 48 ± 13 months. All 13 patients were effective immediately after operation (BNI grade I ) ,including ipsilateral hearing loss in 1 case, recurrence 1 year after operation in 1 case ( BNI grade HI) ,and recurrence in 1 case 3 years after operation (BNI grade M). Eleven patients were effective during the foUowed-up (BNI grade I ). Conclusions Microvascular decompression has a short-term curative effect for trigeminal neuralgia caused by VBD. Its safety is better,but because of the small sample size and VBD is a chronic progressive disease,its long-term efficacy needs further observation.

Síndrome de Moebius incompleto. Presentación de un caso clínico / Incomplete Moebius syndrome. Clinical case presentation

RESUMEN El síndrome de Moebius es un trastorno polimalformativo no progresivo que se caracteriza por parálisis facial congénita. Se define como una "parálisis congénita de los núcleos de los pares craneales VI y VII, cuyo espectro clínico es variable y se asocia a múltiples malformaciones óseas y musculares. Es poco frecuente y de etiología vascular, genética o multifactorial. El trabajo, basándose en los fundamentos teóricos más actualizados, pretendió describir las manifestaciones clínicas del síndrome de Moebius y su posible etiología, a propósito de un caso. Se trató de un paciente de 11 años de edad, que al nacimiento presentó asimetría facial, desviación de la comisura labial hacia la izquierda, boca semiabierta, lagrimeo constante y pabellón auricular derecho malformado. Por ser una entidad clínica poco conocida, se expuso el presente caso, portador de un síndrome de Moebius incompleto de causa vascular y multifactorial (AU).

ABSTRACT Moebius syndrome is a non-progressive poli-formative disorder characterized by facial congenital paralysis. It is defined as a congenital paralysis of the VI and VII cranial nerves nuclei, the clinical spectrum of which is variable and associated to several bone and muscular malformations. It is few frequent and has vascular, genetic or multifactorial etiology. This work, based on more updated theoretical fundaments, pretended to describe the clinical manifestations of the Moebius syndrome and its possible etiology on the purpose of a case. It is the case of a patient, aged 11 years, who presented facial asymmetry, lips commissure deviation to the left, semi-opened mouth, constant lagrimeo and deformed right auricular pavilion (pabellon auricular). Because it is a little known clinical entity, this case of a patient having an incomplete Moebius syndrome of vascular and multifactorial cause was presented (AU).

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.

Acometimento de nervos cranianos na granulomatose com poliangeíte (GPA) C-ANCA negativo / Cranial nerve impairment in granulomatosis with polyangeitis (GPA) C-ANCA negative

RESUMO O presente relato tem o objetivo de mostrar um caso incomum de Granulomatose com Poliangeíte (GPA), que previamente era denominada Granulomatose de Wegener. Trata-se de é uma doença multissistêmica, caracterizada por inflamação granulomatosa necrotizante e vasculite que envolve principalmente o trato respiratório superior e inferior, embora não raramente, exista comprometimento neurológico.

ABSTRACT This report aims to show an unusual case of granulomatosis with polyangeitis (GPA), previously known as Wegener's granulomatosis. It is a multisystemic disease characterized by necrotizing granulomatous inflammation and vasculitis involving mainly the upper and lower respiratory tract, although not infrequently, there is neurological impairment.

Anatomía radiológica de la base de cráneo y los nervios craneales parte 1: generalidades y base de cráneo / Skull base and cranial nerves radiologic anatomy part 1: basics and skull base

La anatomía de la base del cráneo es compleja. Numerosas estructuras neurovasculares vitales pasan a través de múltiples canales y formámenes de 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 a través de los forámenes de base de cráneo. La TC y la RM son complementarias entre sí y, a menudo, se usan en conjunto para demostrar la extensión completa de la enfermedad. La primera parte de esta revisión se centra en generalidades del estudio radiológico y anatomía de base de cráneo.

The skull base anatomy is complex. Many vital neurovascular structures course through the skull base canals and foramina. With the routine use of CT and MRI, the localization of lesions has become more precise as well as their relationship with adjacent neurovascular structures. There is a known anatomical course of the cranial nerves and their skull base s they course through the foramina towards the head and neck. CT and MRI are complimentary modalities and are often used together to map the full extent of disease. The first part of this review article series focus on the radiologic approach to disease and the skull base anatomy.

A study of ocular movements and pupillary changes in acute stroke patients

Background: Cerebrovascular disease or stroke rank first in frequency and importance, among all the neurological diseases of adult life. It is the third most common cause of death in the world. Different neuroanatomical pathways are involved in the control of pupil, the integrity and the functionality of these neurological pathways can be often be ascertained through the analysis and interpretation of pupillary behavior. This makes the pupil size and the pupillary light reflex an important factor to be considered in many clinical conditions. Aim and objective: To study the ocular movements and pupils in acute stroke patients with its clinical correlation and imaging. Materials and methods: The study sample included was 50 patients with acute stroke confirmed by CT/MRI findings of both sexes and who belonged to the age group of 20 to 80 years from RMMCH. A detailed clinical history was taken for these patients who were included in this study. All these patients were examined thoroughly with particular importance to ocular movements and pupils. Results: Of the 50 patients, 16 patients were having an altered level of consciousness(32%), 30 patients were having speech disturbances(72%), 18 patients were having gaze paresis(36%), all the patients who were included in the study were having facial palsy (100%), Of the 18 patients with abnormal pupillary findings, there were 9 patients (18%) with dilated pupils which were not reacting to light during the first 12 hours, i.e. from the time of admission, 9 patients (18%) with round, constricted pupils, sluggishly reacting to light and 32 patients (64%) did not show any changes in the pupils. Of the 20 patients (40%) with abnormal ocular movements, the commonly observed ocular changes in the study sample were 3rd nerve palsy, 6th nerve palsy, multiple cranial nerve palsies, conjugate eye deviation to the side of the lesion, nystagmus on left lateral gaze, horizontal nystagmus on lateral gaze to the side of lesion. K. Babu Raj, G. Arul Venkadesh. A study of ocular movements and pupillary changes in acute stroke patients. IAIM, 2018; 5(8): 9-16. Page 10 Conclusion: The Anterior circulation stroke (78%) was more common than the Posterior circulation stroke (22%) in our study group. Although the abnormal pupillary changes and ocular movements were very common in posterior cerebral artery territory infarcts, the incidence of abnormal pupillary changes and ocular movements was more common in posterior cerebral artery territory (50%) and also in middle cerebral artery territory (45%) in our study group.

Anatomía comparada del neurocráneo y el encéfalo de la raya mariposa Gymnura micrura (Batoidea: Gymnuridae) / Comparative anatomy of the neurocranium and encephalon of the butterfly ray, Gymnura micrura (Batoidea: Gymnuridae)

Mediante la comparación del neurocráneo de la "raya mariposa" Gymnura micrura con especies representantes de cinco familias de Batoidea, se reconoce que corresponde al patrón anatómico del orden Myliobatiformes, por la ausencia del rostro, la presencia de una sola fontanela y el mayor desarrollo de los procesos postorbitarios. La especie en estudio difiere de los Urotrygonidae y Dasyatidae por tener los procesos postorbitarios subdivididos. La organización del encéfalo de G. micrura corresponde al patrón anatómico de los Batoidea, sin embargo, a diferencia de los Rhinobatidae, Urotrygonidae y Dasyatidae, el cerebelo es simétrico; en contraste con los Myliobatidae los hemisferios cerebrales son reducidos; y la principal diferencia con relación a los Narcinidae es la ausencia de los lóbulos eléctricos.

By comparing the neurocranium of the "butterfly ray" Gymnura micrura with representative species of five families of Batoidea, it is recognized that it corresponds to the anatomical pattern of the order Myliobatiformes, because of the absence of the rostrum, the presence of a single fontanelle and the development of the postorbital processes. The species under study differs from the Urotrygonidae and Dasyatidae by having subdivided postorbital processes. The organization of the encephalon of G. micrura corresponds to the anatomical pattern of the Batoidea. However, unlike the Rhinobatidae, Urotrygonidae, and Dasyatidae, the cerebellum is symmetrical; in contrast with the Myliobatidae, the cerebral hemispheres are reduced; and, the main difference in relation to the Narcinidae, is the absence of electrical lobes.