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Objective To investigate the clinical manifestations of nodo-paranodopathy(NPP)with anti-neurofascin 186(NF186)antibody positive.Methods The clinical data of a NPP patient with cranial nerve damage caused by anti-NF186 antibody positive was retrospectively analyzed.Results The patient was a 70-year-old male with sudden speech disorder and dysphagia one month ago.Glucocorticoid therapy was discontinued after improvement.The patient's speech,swallowing,chewing,bristling,turning and head-up movements were laborious and progressively aggravated 5 days ago.The EMG examination of the limbs was normal,and the serum and CSF anti-NF186 antibody were positive.The curative effect of glucocorticoid treatment was not obvious,and the symptoms were significantly improved after plasma exchange treatment.Conclusions Anti-NF186 antibody-positive NPP has late onset age,severe illness and accompanied with cranial nerves damage.Conventional hormone therapy is not effective,but plasma exchange therapy is effective.
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Objective To investigate the clinical features of rhino-orbital-cerebral mucormycosis(ROCM)with bilateral cranial nerve palsies as the clinical manifestation.Methods The related clinical data about ROCM with bilateral cranial nerve palsy as a clinical manifestation was collected,analyzed as well as discussed in the manuscript.And the relevant literatures were reviewed.Results This patient was a healthy young man with new-onset diabetes and diabetic ketoacidosis.The patient developed rapidly with acute onset,bilateral blindness,blepharoptosis and extraocular muscle paralysis.The presence of mucormycosis was confirmed by CSF second-generation sequencing,fungal fluorescent staining and culture.Although effective antifungal therapy was performed early,but the patient quickly presented with cerebral hemorrhage and herniation.Eventually,the patient died after discharge.Conclusions ROCM is a rare and high-mortality infectious disease.This case indicated that the clinicians should consider the presence of ROCM in patients with diabetes/diabetic ketoacidosis when the bilateral cranial nerves paralysis are the clinical symptom,whicn can not be explained by other lesions.CSF next-generation sequencing is helpful for rapid diagnosis.
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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.
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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.
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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.
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HumainsRésumé
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.
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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.
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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.
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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.
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Humains , Mâle , Femelle , Enfant , Adolescent , Adulte , Adulte d'âge moyen , Sujet âgé , Sujet âgé de 80 ans ou plus , Jeune adulte , Syndrome de Guillain-Barré/complications , Maladies du système nerveux/étiologie , Pérou/épidémiologie , Phonation , Acides , Santé buccodentaire , Épidémiologie Descriptive , Études transversales , Nerfs crâniens , Expression faciale , Paralysie facialeRésumé
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.
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Humains , Base du crâne/innervation , Base du crâne/imagerie diagnostique , Nerfs crâniens/imagerie diagnostique , Imagerie par résonance magnétique , Tomodensitométrie , Nerfs crâniens/anatomie et histologieRésumé
Introduction: Cranial nerves, leptomeninges and cavernoussinus are often involved in lymphomas. Isolated oculomotornerve palsy as the first manifestation of a lymphoma is rare,particularly when none of its other manifestations are initiallyidentified.Case report: A 31 year old man with no known co-morbidities,came with complaints of acute onset of blurring of visionand drooping of the right eyelid. Neurological examinationrevealed isolated right medial rectus palsy with dilated pupiland ptosis of right eyelid, suggesting complete oculomotornerve palsy. CT Brain, CT Angiogram and CSF study werenormal.Chest X Ray showed mediastinal widening and CECTThorax showed anterior mediastinal mass. CT guided biopsysuggested lymphoproliferative disorder. IHC was diagnosticfor Thymoma. The mass was resected. Ten days later, thepatient returned, with worsening of his symptoms. Neurologicexamination showed third, fourth and sixth cranial nervepalsy. IHC of the resected specimen revealed high gradeB cell Lymphoblastic Lymphoma. Repeat CSF analysisshowed leptomeningeal involvement. Patient was started onchemotherapy for high grade lymphoma and his extra ocularmovements improved. One week later, he developed bilateralfacial palsy and Left CN IX, X Palsy. Chemotherapy wascontinued until he was discharged at request. Patient was lostto follow up.Conclusion: This atypical presentation of NHL can bringabout a delay in the diagnosis due to the variability of itspresenting symptoms and wide differential diagnosis. Thusearly diagnosis and aggressive management is essential.
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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.
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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.
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Humains , Mâle , Adulte d'âge moyen , Douleur , Ophtalmoplégie/diagnostic , Syndrome de Tolosa-Hunt/diagnostic , Syndrome de Tolosa-Hunt/thérapie , Prednisone/usage thérapeutique , Imagerie par résonance magnétique , Sinus caverneux/anatomopathologie , Sinus caverneux/imagerie diagnostique , Classification internationale des maladies , Nerfs crâniens/imagerie diagnostique , Syndrome de Tolosa-Hunt/classification , Diagnostic différentiel , CéphaléeRésumé
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.
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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.
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Humains , Femelle , Enfant , Troubles de la motilité oculaire/congénital , Nerfs crâniens/malformations , Atteintes du nerf trochléaire/congénital , Syncinésie/congénital , Muscles oculomoteurs/innervation , Troubles de la motilité oculaire/classification , Troubles de la motilité oculaire/anatomopathologie , Atteintes du nerf trochléaire/classification , Atteintes du nerf trochléaire/anatomopathologie , Maladies rares , Syncinésie/classification , Syncinésie/anatomopathologie , Paupières/malformationsRésumé
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.
Résumé
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.
Sujets)
Humains , Adulte , Nerf vague/anatomie et histologie , Tronc cérébral/anatomie et histologie , Nerf glossopharyngien/anatomie et histologie , Nerf accessoire/anatomie et histologie , Nerfs crâniens/anatomie et histologieRésumé
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).
Sujets)
Humains , Mâle , Enfant , Ophtalmologie , Astigmatisme/diagnostic , Malformations , Syndrome de Moebius/diagnostic , Paralysie faciale/diagnostic , Hypermétropie/diagnostic , Astigmatisme/génétique , Techniques de physiothérapie , Syndrome de Moebius/complications , Syndrome de Moebius/étiologie , Syndrome de Moebius/génétique , Syndrome de Moebius/épidémiologieRésumé
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.
Sujets)
Tronc cérébral/physiologie , Cortex cérébral/physiologie , Nerfs crâniens/physiologie , Déglutition/physiologieRésumé
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.