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Resumen El espasmo hemifacial consiste en un infrecuente trastorno del movimiento que afecta a la musculatura inervada por el nervio facial, en especial la de la hemicara superior. Existen dos formas clínicas, una primaria en la que la causa subyacente está representada por un conflicto neurovascular, y una secundaria, en la que la alteración del nervio facial es producida por algún tipo de lesión ocupante de espacio. Resulta de especial interés para el otorrinolaringólogo conocer esta entidad y ser capaz de diferenciar ambas formas clínicas dado el diferente enfoque terapéutico que pueden requerir. Aportamos una visión general de esta patología repasando su epidemiología y fisiopatología, además, de nuestra experiencia en forma de una serie de cuatro casos que ilustran las variadas formas de presentación de esta entidad, así como los signos y síntomas de alarma que pueden ayudar a realizar un correcto diagnóstico y manejo.
Abstract Hemifacial spasm consists of an unfrecuent movement disorder involving facial muscles, especially those from the upper half of the face. Two different clinical presentations are described. Primary hemifacial spasm is defined by a neurovascular conflict, and secondary occurs when facial nerve is damaged by a space occupying lesion. It is of special interest for the otorhinolaryngologist to get to know about this condition and to be able to acknowledge its clinical presentations due to the different therapeutical approach that may be needed for each of them. We contribute with a general vision of this entity reviewing its epidemiology and patophysiology. Furthermore, we show our experience by sharing a four-case series which we believe to illustrate the different ways of presentation as well as the alarm signs and symptoms that may be helpful in order to accomplish an accurate diagnose and treatment.
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Introducción: La Academia Americana de Neurología propone que, para que un paciente sea candidato a descompresiva neurovascular trigeminal (DNV), se debe demostrar la presencia de contacto neurovascular a través de la resonancia magnética (RM). Sin embargo, recientes estudios han demostrado que la sensibilidad de la RM para diagnosticar un conflicto neurovascular (CNV) es muy variable. Estos conceptos antagónicos ubican al neurocirujano ante un verdadero dilema a la hora de tomar decisiones. El objetivo del presente estudio es evaluar la utilidad de la clínica y la RM como factor predictor de un verdadero CNV. Materiales y Métodos: Estudio analítico retrospectivo que incluye a 81 pacientes a los que se les realizó una DNV por neuralgia trigeminal (NT), desde enero de 2013 hasta abril de 2019, en la provincia de Tucumán. Resultados: Al considerar la serie completa: A) Un total de 65 pacientes cursaron con NT Típicas; de estos, 64 (98,5%) presentaron CNV durante la cirugía y B) De las 16 Atípicas, ninguna presentó CNV en el intraoperatorio. Al considerar los pacientes con NT primaria: A) un 98% de los pacientes con clínica de NT típica presentaran CNV en el intraoperatorio y B) hubo un grupo de pacientes donde se evidencio un CNV en el intraoperatorio pero en su RM preoperatoria no se objetivo el mismo (n=15), el valor predictivo negativo de la RM fue sólo un 6%. Conclusión: La clínica del paciente es más efectiva que la RM para decidir si realizar o no una cirugía de DNV.
Introduction: The American Academy of Neurology proposes that, for a patient to be a candidate for trigeminal neurovascular decompression (NVD), the presence of neurovascular contact must be demonstrated through magnetic resonance imaging (MRI). However, recent studies have shown that the sensitivity of MRI to diagnose a neurovascular conflict (NVC) is highly variable. These antagonistic concepts put, the neurosurgeon, in a real dilemma when making decisions about this entity. The aim of this study is to evaluate the usefulness of clinical and MRI as a predictor of a real NVC. Methods: This is a retrospective analytical study including 81 patients undergoing NVD to treat trigeminal neuralgia (TN), from January 2013 to April 2019, in Tucumán. Results: When considering the complete series: A) Of the total of 65 patients who had typical TN, 64 (98.5%) presented NVC during surgery and B) Of the 16 atypical TN, none of then presented NVC during the surgery. When considering patients with primary TN: A) 98% of the patients with typical NT presented a NVC during surgery and B) there was a group of patients where NVC was evidenced during surgery but it wasn`t noticed during the preoperative MRI (n=15), the negative predictive value of the MRI was only 6%. Conclusion: The patient's clinic is more effective than the MRI deciging whether or not to perform NVD surgery.
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Humans , Trigeminal Neuralgia , Therapeutics , NeuralgiaABSTRACT
Objective To investigate the value of MRI in analyzing relationship between compression site of the vascular compression and the facial pain area in patients with primary trigeminal neuralgia (PTN).Methods MRI data of 123 patients with unilateral PTN were retrospectively analyzed,including imaging of three-dimensional-time of flight MRA (3D TOF MRA) sequence,three-dimensional-fast imaging employing steady state acquisition sequence and contrast-enhanced 3D TOF MRA sequence.Neurovascular conflict (NVC) sites were divided into 8 sites according to the sectional anatomy of trigeminal nerve,and the relations between topography of pain and site of NVC were analyzed in 60 PTN patients with a single offending vessel and a single site of NVC.Distance (d) from the root entry point to the site of NVC,the length (L) of cisternal segment of the trigeminal nerve were both measured,and d/L was calculated in 123 patients with 206 NVC points.Results Of 60 patients with single offending vessel and single site of NVC,95.00％ (57/60) sites of NVC matched to topography of pain,5.00％ (3/60) did not,whereas NVC points with d/L≤1/4 accounted for 58.33％ (35/ 60),d/L≤1/2 accounted for 85.00％ (51/60).Among 206 NVC points,the meand was (2.50±1.35)mm,and NVC points with d/L≤1/4 accounted for 44.66％ (92/206),d/L≤1/2 accounted for 74.27％ (153/206).Conclusion MRI is helpful to detecting the offending vessels in PTN patients through judging relationship between the topography of pain and the site of NVC,as well as measuring the distance from the root entry point to the site of NVC.
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OBJECTIVE: The purpose of this study was to evaluate the characteristics and surgical outcomes of familial hemifacial spasm (HFS) and to discuss the role of genetic susceptibility. METHODS: Between 2001 and 2011, 20 familial HFS patients with ten different pedigrees visited our hospital. The data from comprehensive evaluation of these patients, including clinical, radiological and electrophysiological data and surgical outcomes were reviewed to characterize familial HFS and to compare the characteristics between familial HFS and sporadic HFS. RESULTS: According to the family tree, the inheritance pattern was difficult to define clearly using these data. Radiologic findings suggested that the vertebral artery (VA) was a more frequent offender in familial HFS than in sporadic cases (35.0% vs. 10.0%, p<0.001). Chi-square test showed that there were no correlation between VA tortuosity and underlying morbidity such as diabetes or hypertension (p=0.391). Eighteen out of 19 patients who underwent microvascular decompression showed no residual spasm. Other features of familial HFS overlap with sporadic cases. These findings suggest that certain genetic susceptibilities rather than hypertension or diabetes may influence vascular tortuosity and HFS development. CONCLUSION: In this study, familial HFS seems not so different from sporadic cases. Authors thought familial HFS could have heterogeneous etiology. Further study of familial HFS including clinical, anatomic, genetic, and molecular information may help identify a gene or trait that can provide insight into the mechanisms of sporadic and familial HFS.