Síndrome de Moebius y ortotropía en posición primaria: ¿es infrecuente esta asociación? (Colombia) / Moebius syndrome and orthotropia in primary position: is this association uncommon? (Colombia) / Síndrome de Moebius e ortotropia em posição primária: é uma associação incomum? (Colômbia)

El síndrome de Moebius es una enfermedad congénita poco común que se caracteriza por el compromiso unilateral o bilateral del VI y VII par craneal, lo que compromete los músculos que controlan la oculomotricidad, produciendo una parálisis en la abducción del globo ocular y los músculos involucrados en la expresión facial. Su presentación clínica y grados de severidad son variables, puede presentar compromiso simétrico o asimétrico. Adicionalmente, gran parte de los casos se relacionan con trastornos del lenguaje, anomalías musculoesqueléticas y orofaciales. En el presente artículo se presenta el caso de una paciente femenina de 3 años producto de un embarazo trigemelar con diagnóstico clínico de síndrome de Moebius al nacer, confirmado por neuroimagen en la que se evidencia la ausencia bilateral del nervio facial en ángulos pontocerebelosos, adicionalmente con un defecto completo en los movimientos oculares de abducción y aducción lo que impide el estrabismo convergente común en estos pacientes.

Moebius syndrome is a rare congenital disease characterized by unilateral or bilateral involvement of the VI and VII cranial nerves, which compromises the muscles that control ocular motricity with paralysis in the abduction of the eyeball and the muscles involved in the facial expression. Its clinical presentation and degrees of severity are variable, and it can be symmetric or asymmetric. Additionally, most of the cases are related to language disorders, musculoskeletal and orofacial anomalies. This paper presents the case of a 3-year-old female patient, product of a trigemellar pregnancy with a clinical diagnosis of Moebius syndrome at birth, confirmed by neuroimaging, which shows the bilateral absence of the facial nerve in point-lateral angles. Additionally she has a complete defect in abduction and adduction eye movements, which prevents the common convergent strabismus in these patients.

A síndrome de Moebius é uma doença congênita rara caracterizada pelo envolvimento unilateral ou bilateral dos nervos cranianos VI e VII, que compromete os músculos que controlam a oculomotricidade com paralisia na abdução do globo ocular e dos músculos envolvidos na expressão facial. Sua apresentação clínica e graus de gravidade são variáveis, podendo ser um comprometimento simétrico ou assimétrico. Além disso, a maioria dos casos está relacionada a distúrbios de linguagem, anomalias musculoesqueléticas e orofaciais. Este paper apresenta o caso de uma paciente de 3 anos de idade, fruto de uma gravidez trigêmea com diagnóstico clínico de Síndrome de Moebius ao nascimento, confirmado por neuroimagem em que é evidente a ausência bilateral do nervo facial nos ângulos ponto-cerebelares. Além disso, ela tem um defeito completo nos movimentos oculares de abdução e adução, o que impede o estrabismo convergente comum nesses pacientes.

Complicaciones de la otitis media con parálisis del sexto par craneal contralateral en pediatría / Cranial complications of otitis media with paralysis of the contralateral sixth cranial pair in pediatrics

Resumen. La otitis media es una infección frecuente en la infancia, la cual puede producir complicaciones, incluidas las neurológicas graves, en cuatro de cada 100 niños en países en desarrollo. Se presenta el caso de una niña de nueve años sin antecedentes de enfermedad que consultó por otitis media derecha, otorrea, síndrome de hipertensión intracraneal y parálisis del VI nervio craneal contralateral a la lesión. La tomografía computarizada de cráneo y la resonancia magnética cerebral revelaron otomastoiditis crónica, apicitis petrosa, y trombosis de los senos transverso y sigmoide, el bulbo yugular y la vena yugular interna derecha. Recibió tratamiento antibiótico y quirúrgico. Este caso refleja el espectro de complicaciones intracraneales y extracraneales asociadas con la otitis media aguda en la era antibiótica. El examen físico permite la detección precoz de la hipertensión intracraneal, con signos como el papiledema y la parálisis del VI par contralateral como hallazgo inusual.

Abstract. Otitis media is a frequent infection during childhood. Complications may be present in up to 4 of 100 children including serious neurological complications, particularly in developing countries. We report the case of a 9-year-old girl with no disease history who presented with otitis media, otorrhea, intracranial hypertension syndrome, and paralysis of the VI cranial nerve contralateral to the lesion. A computed tomography scan of the skull and a brain magnetic resonance imaging revealed chronic otomastoiditis, petrous apicitis, and thrombosis of the transverse and sigmoid sinus, the jugular bulb, and the right internal jugular vein. She received antibiotics and surgical treatment. This case shows the spectrum of intra and extracranial complications associated with acute otitis media in the antibiotic era. The physical examination allows early identification of intracranial hypertension with signs such as papilledema and sixth contralateral nerve palsy as an unusual finding.

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

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

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

Abducens Nerve Palsy as Initial Manifestation of Chronic Subdural Hematoma: Case Report

Chronic subdural hematoma (CSDH) is a form of progressive intracranial hemorrhage, typically associated with cases of trauma. The manifestation of this comorbidity with abducens palsy is a rare finding. The present work aims to describe the case of an adult patient with abducens nerve palsy as a manifestation of CSDH. Chronic subdural hematoma is most commonly found in elderly patients, with systemic hypertension as amanifestation. The relation with the sixth cranial nerve is unusual and draws attention to the case reported. In addition, the prognosis is positive, since trepanation and drainage surgery was performed, as it is recommended in the literature.

Treatment of lateral rectus paralysis by transposition of superior rectus to lateral rectus / Tratamento de paralisia de reto lateral pela transposição do reto superior para o reto lateral

ABSTRACT Objective: To evaluate the results of the surgical technique described by Crouch in the correction of lateral rectal paralysis (LR) esotropia. Methods: A study conducted of six patients with VI cranial nerve palsy, with more than three months, and associated contracture of the medial rectus muscle. The causes of paralysis varied in traumatic, congenital and neuropathic. The patients underwent surgical correction, performed with a technique consisting of the instillation of the superior rectus muscle (SR) and its suture above the insertion of the LR muscle, completed with Foster´s suture (suture joining, 8mm of the muscle insertion, the body of the SR and LR). The patients were followed for six months. Results: Five patients presented preoperative deviation between 30 and 50 prismatic diopter (PD), and one patient presented a deviation greater than 100 PD, and therefore, the patient required surgical reintervention due to residual deviation. The other five patients presented postoperative orthoppy, with better visual acuity and no vertical deviations. Conclusion: The surgery proposed by Crouch has been shown to be a good alternative to conventional techniques, such as Carlson-Jampolsky surgery, being a technically simpler procedure with good results.

RESUMO Objetivo: Avaliar resultados da técnica cirúrgica descrita por Crouch na correção de esotropia por paralisia do reto lateral (RL). Métodos: Estudo realizado com seis pacientes diagnosticados com paralisia de IV par craniano, com mais de três meses, e com contratura associada do musculo reto medial. As causas da paralisia variaram em traumática, congênita e neuropática. Os pacientes foram submetidos à correção cirúrgica, realizada com técnica que consiste na desinserção do músculo reto superior (RS) e sua sutura acima da inserção do músculo RL, completada com ponto de Foster (sutura unindo, a 8mm da inserção muscular, o corpo do RS e RL). Os pacientes foram seguidos por seis meses. Resultados: Cinco pacientes apresentaram, no pré-operatório, desvio entre 30 e 50 dioptrias prismáticas (DP), e um paciente apresentava desvio maior que 100DP, sendo, portanto o paciente que necessitou de reintervenção cirúrgica, por desvio residual. Os outros cinco pacientes apresentaram ortotropia no pós-operatório, com melhor da acuidade visual e sem desvios verticais. Conclusão: A cirurgia proposta por Crouch demostrou-se uma boa alternativa às técnicas convencionais, como cirurgia de Carlson-Jampolsky, sendo um procedimento tecnicamente mais simples e com bons resultados.

Delayed Onset Abducens Nerve Palsy and Horner Syndrome after Treatment of a Traumatic Carotid-cavernous Fistula

PURPOSE: We report a patient with delayed-onset abducens nerve palsy and Horner syndrome after endovascular treatment of traumatic carotid-cavernous fistula (CCF). CASE SUMMARY: A 68-year-female visited our ophthalmic department complaining of gradual-onset ptosis of the left eye and horizontal diplopia. She had undergone endovascular treatment to treat left-sided traumatic CCF after a car accident 10 years before; she had been told at that time that the treatment outcome was favorable. The left-sided ptosis gradually developed 6 years after the procedure, accompanied by diplopia. The left eye exhibited miosis and the extent of anisocoria increased in dim light. An extraocular examination revealed 30 prism diopters of left esotropia in the primary gaze and a −4 abduction limitation of the left eye. CCF recurrence was suspected; however, magnetic resonance imaging with magnetic resonance angiography of brain did not support this. The esotropia did not improve during the 6-month follow-up and strabismus surgery was performed. CONCLUSIONS: Delayed-onset abducens nerve palsy and Horner syndrome can develop even after successful endovascular treatment of CCF. Strabismus surgery should be considered in patients whose diplopia does not spontaneously improve.

Isolated Abducens Nerve Palsy due to a Dural Arteriovenous Fistula with Drainage into the Inferior Petrosal Sinus

No abstract available.

Posterior Inferior Cerebellar Artery Infarction Originating at C1-2 after C1-2 Fusion / 대한신경손상학회지

Vertebral artery injuries associated with C1 lateral mass screw insertion rarely occur during C1-2 fusion. The posterior inferior cerebellar artery (PICA) is uncommonly located at the C1 lateral mass insertion position. A 71-year-old woman with atlanto-axial subluxation and cord compression underwent C1-2 fusion. Sixth nerve palsy and diplopia were detected postoperatively, and decreased consciousness occurred on postoperative day 4. Brain magnetic resonance image (MRI) and computed tomography (CT) revealed PICA infarction. In the preoperative CT angiography, the PICA originated between the C1 and C2 level. In the postoperative CT scan, the PICA was not visible. The patient was treated conservatively for two weeks and recovered. PICA originating between the C1 and C2 level comprises 1.1–1.3% of cases. Therefore, vertebral artery anomalies should be evaluated prior to C1-2 fusion to prevent vessel injuries.

Palsy of the Contralateral Abducens Nerve after Aneurysm Surgery and Temporal Meningioma in the Presence of Petroclival Meningioma: A Case Report

The sixth abducens nerve is subject to injury after rare complications of intracranial hypotension caused by procedures such as dural punctures and spinal surgeries. The purpose of this case report is to discuss the mechanism of nerve palsy in these situations. Therefore, we describe a case of onset of contralateral sixth cranial nerve palsy after intracranial aneurysm and temporal meningioma surgery. Moreover, in this case there is a singularity due to the presence of the petroclival meningioma that amplified the unfolding of the lesion.

O sexto nervo abducente está sujeito a lesões após raros eventos de hipotensão intracraniana gerada por procedimentos como punções de dura-máter e cirurgias de coluna. O propósito deste relato de caso é discutir o mecanismo da paralisia deste nervo nestas situações. Para isso, descrevemos um caso de aparecimento de paresia do sexto nervo craniano contralateral após cirurgia de aneurisma intracraniano e de meningioma temporal. Além do mais, neste caso há uma singularidade em razão da presença do meningioma petroclival contralateral, que amplificou o desdobramento da lesão.

Epidural blood patch treatment of diplopia that developed after headache resolution in a patient with spontaneous intracranial hypotension

Sudden headache onset may rarely be caused by spontaneous intracranial hypotension (SIH). Other associated symptoms in patients with SIH are nausea, vomiting, vertigo, hearing alteration, and visual disturbance. This case report describes a 43-year-old female diagnosed with SIH who developed diplopia after resolution of an abrupt-onset headache, which was managed with conservative treatments, including bed rest and hydration. She was also diagnosed with secondary right sixth cranial nerve palsy. Although conservative management relieved her headache, the diplopia was not fully relieved. Application of an autologous epidural blood patch successfully relieved her diplopia, even after 14 days from the onset of visual impairment.

Abducens Nerve Palsy and Optic Perineuritis Caused by Fungal Sphenoidal Sinusitis

PURPOSE: To report a case of abducens nerve palsy and optic perineuritis caused by fungal sphenoidal sinusitis. CASE SUMMARY: A 48-year-old male visited emergency department for retrobulbar pain, decreased vision, and horizontal diplopia for 3 days. He reported that previous medical history was non-specific, however, blood glucose level was 328 mg/dL (70–110). He had experienced severe headache for 7 days. The best corrected visual acuity was 20/20 at right eye and 20/25 at left eye. The pupil of left eye did not have relative afferent pupillary defect. Left mild proptosis was noted. The extraocular examination showed 30 prism diopters left esotropia at primary gaze and −4 abduction limitation of left eye. The left eye showed mild optic disc swelling and inferior field defect by field test. Brain magnetic resonance imaging showed enhancement of sphenoidal sinus, ethmoidal sinus, and around optic nerve at left eye. Three days after antibiotics treatment, the vision of left eye deteriorated to 20/40 and periorbital pain developed. The drainage and biopsy of sphenoidal sinus were performed. The histopathologic examination showed hyphae consistent with aspergillosis. The ocular symptoms were improved with anti-fungal treatment. Follow-up magnetic resonance imaging performed 1 month after treatment showed improvement of lesion at left orbit. Five months after surgery, the visual acuity of left eye was improved to 20/25. The patient showed orthotropia at primary gaze without limitation. CONCLUSIONS: The abducens nerve palsy and optic perineuritis can be caused by fungal sphenoidal sinusitis. The early diagnosis and appropriate treatment can lead to favorable outcome.

Abducens Nerve Palsy Caused by the Ophthalmic Segment of an Internal Carotid Artery Aneurysm

PURPOSE: To report a case of isolated abducens nerve palsy caused by the ophthalmic segment of an internal carotid artery (ICA) aneurysm which improved after endovascular coil trapping. CASE SUMMARY: A 59-year-old female visited the ophthalmology department for a sudden onset of horizontal diplopia for 10 days. The best corrected visual acuity was 20/20 in both eyes. The pupils showed normal response to light and near stimulation in both eyes. The extraocular examination showed 35 prism diopters left esotropia at primary gaze and −4 abduction limitation of the left eye. The patient suffered intermittent headaches in the left temporal area and left retrobulbar pain for 1 month. Magnetic resonance imaging with magnetic resonance angiography of the brain was performed. A focal protruding lesion of the left ICA suggested an aneurysm. The patient consulted with the neurosurgery department. The left ophthalmic segment of the ICA aneurysm was confirmed by transfemoral cerebral angiography and treated with coil placement and the patient showed gradual improvement after the procedure. Three months after the procedure there was no diplopia. The patient showed orthotropia at primary gaze without abduction limitation. CONCLUSIONS: Isolated abducens nerve palsy can be caused by the ophthalmic segment of an ICA aneurysm, which should be considered in the differential diagnosis of ocular motility disorders. The disorder improved with coil replacement treatment. Differential diagnosis as a cause of abducens nerve palsy is important for prompt and appropriate treatment. Neuroimaging should be considered in patients with isolated abducens nerve palsy with a non-ischemic origin.

A Case of Orbital Apex Syndrome with Central Retinal Artery and Vein Occlusion Following Trauma

PURPOSE: To report a case of orbital apex syndrome (OAS) combined with central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) following blunt trauma. CASE SUMMARY: A 4-year-old female visited the hospital following a traffic accident. She was admitted because of multiple fractures of the skull and pneumocephalus. On day 5, she was referred to us with decreased visual acuity in her right eye. Her initial visual acuity was hand motions in the right eye and 0.8 in the left eye. The right eye showed a dilated pupil, ptosis, and total ophthalmoplegia, and the left eye showed limited abduction. A fundus examination revealed multiple retinal hemorrhages, tortuous veins, and an edematous white retina with a cherry-red spot in the right eye. Brain magnetic resonance imaging revealed an entrapped right optic nerve because of bony fragments in the orbital apex. The patient was diagnosed with OAS accompanied by CRAO and CRVO in the right eye, and with traumatic abducens nerve palsy in the left eye. After 6 months, the visual acuity was hand motions, and the fundus examination showed absorbed retinal hemorrhages, pale discs, and general retinal thinning of the right eye. Ptosis of the right eye and extraocular muscle movement of both eyes were improved. CONCLUSIONS: Combined CRAO and CRVO following trauma is very rare and is even more rarely associated with OAS. It is important for clinicians to be aware of the potential for central retinal vessel occlusions and OAS in cases of blunt ocular trauma.

Cerebral Venous Thrombosis Presenting in Pregnancy with Thrombocytosis and Janus Kinase 2 Valine-to-Phenylalanine Mutation

A 30-year-old woman in her first pregnancy was admitted with headache and horizontal diplopia indicating left abducens nerve palsy. Brain magnetic resonance imaging revealed a cerebral venous thrombosis. She had thrombocytosis in the peripheral blood, and a genetic test for thrombocytosis revealed the presence of the valine-to-phenylalanine (V617F) mutation of the Janus kinase 2 (JAK2) gene. Treatment with low-molecular-weight heparin resolved her symptoms of headache and diplopia. The presence of genetic disorders such as the JAK2-V617F gene mutation should be assessed in patients with cerebral venous thrombosis and coexisting thrombocytosis.

Abducens Nerve Palsy Associated with Ramsay-Hunt Syndrome / 대한평형의학회지

Ramsay-Hunt syndrome is an infectious disease caused by the varicella zoster virus. It is usually associated with facial and vestibulocochlear nerve palsy, but other cranial nerve dysfunction can be accompanied. We present a 68-year-old woman with abducens nerve palsy associated with Ramsay-Hunt syndrome. She showed abduction limitation of left eye with peripheral facial palsy and vestibulopathy of the left side. Varicella zoster virus polymerase chain reaction of cerebrospinal fluid was positive and internal auditory canal magnetic resonance imaging was revealed enhancement of labyrinthine segment of left facial nerve. Although abducens nerve palsy is uncommon feature of Ramsay-Hunt syndrome, but it can be developed by several different mechanisms.

Clinical Course and Prognostic Factors of Acquired Third, Fourth, and Sixth Cranial Nerve Palsy in Korean Patients

PURPOSE: This study aimed to evaluate the clinical course and prognostic factors of acquired third, fourth, and sixth cranial nerve (CN) palsy grouped according to etiology. METHODS: This study involved a retrospective review of the medical records of 153 patients who were diagnosed with acquired paralytic strabismus from January 2004 to July 2015. Outcomes, recovery rates, and time to recovery were investigated according to the affected CN: CN3, CN4, and CN6 palsies. The patients were classified into four groups based on etiology: idiopathic, traumatic, neoplastic, and vascular. RESULTS: The mean age of the patients was 59.8 ± 14.5 years and the mean follow-up period was 10.8 months. Out of the 153 patients, 63 (41.2%) had CN3 palsy, 35 (22.9%) had CN4 palsy, and 55 (35.9%) had CN6 palsy. The most common causes were vascular related (54.9%), followed by idiopathic (28.1%), trauma (8.5%), and neoplasm (5.88%). About 50% of the patients recovered within six months. Among the four etiologic groups, the idiopathic group showed the best prognosis because about 50% of the patients in this group recovered within three months. This was followed by the vascular, traumatic, and neoplastic groups. Cox proportional hazard analysis revealed a significant association between the baseline prism diopter and recovery rate. CONCLUSIONS: The prognosis and natural history of paralytic strabismus vary depending on its cause. The vascular group had the best recovery rate and shortest recovery time, whereas the neoplastic group required the longest time to recover.

Presentación del síndrome de Wernicke-Korsakoff secundario a encefalitis por citomegalovirus, a propósito de un caso / Wernicke-Korsakoff syndrome secondary to cytomegalovirus encephalitis: A case report

Resumen El citomegalovirus (CMV) es uno de los microorganismos oportunistas con mayor prevalencia en pacientes inmunocomprometidos, aunque su reactivación ha descendido después de la introducción de la terapia antirretroviral altamente activa (Highly Active Antiretroviral Therapy, HAART). En las coinfecciones, la encefalitis se ha reportado como una de las condiciones más frecuentes. Se presenta el caso de un paciente adulto joven con infección por virus de la inmunodeficiencia humana (HIV) que tuvo un rápido deterioro neurológico evidenciado en síntomas y signos clínicos clásicos del síndrome de Wernicke-Korsakoff y que no presentaba factores de riesgo para deficiencia de tiamina. En las imágenes de la resonancia magnética cerebral, se detectaron hallazgos típicos del síndrome, y se identificó citomegalovirus (CMV) en el líquido cefalorraquídeo. Con el tratamiento específico para el CMV, se logró el control de los síntomas, aunque hubo secuelas neurológicas que mejoraron. Este es uno de los pocos casos reportados a nivel mundial de síndrome de Wernicke secundario a encefalitis por citomegalovirus.

Abstract Cytomegalovirus (CMV) is one of the opportunistic microorganisms with the highest prevalence in immunocompromised patients. Reactivation has decreased after the introduction of highly active antiretroviral therapy (HAART). Encephalitis has been reported in the coinfection as one of the most frequent presentations. We present the case of a young adult patient with HIV infection and rapid neurological deterioration due to classic clinical symptoms and signs of the Wernicke-Korsakoff syndrome, with no risk factors for thiamine deficiency, with images by nuclear magnetic resonance typical of the syndrome, and identification of cytomegalovirus in cerebrospinal fluid. The specific treatment for CMV managed to control the symptoms with neurological sequelae in progression towards improvement. This is one of the few cases reported in the literature of Wernicke syndrome secondary to cytomegalovirus encephalitis.

Síndrome de Moebius: manifestaciones neurológicas, musculoesqueléticas y del lenguaje / Moebius syndrome: Neurological, musculoskeletal and speech anomalies

El síndrome de Moebius es una enfermedad congénita caracterizada por parálisis facial no progresiva y alteración de la abducción ocular de uno o ambos ojos por compromiso del VI par, pudiendo extenderse a otros pares oculomotores, así como al IX, X y XII. Se ha asociado con manifestaciones musculoesqueléticas y epilepsia. Presentamos el caso de un preescolar con antecedente de intento fallido de la madre de interrupción voluntaria del embarazo en el primer trimestre. Al nacimiento se evidencia diplejía facial, parálisis bilateral del VI par y pie equino varo. A los 17 días de vida presenta crisis tónico-clónicas y se diagnostica síndrome de Moebius y epilepsia de difícil control. Durante el seguimiento presenta retardo global del desarrollo, con predominio en el área del lenguaje y alteración de la deglución...(AU)

Moebius syndrome consists of congenital non-progressive facial nerve palsy and unilateral or bilateral paralysis of the sixth cranial nerve with inability to abduct the eye. The oculomotor cranial nerves, as well as, cranial nerves IX, X and XII, may also be affected. This syndrome has also been associated with anomalies of the musculoskeletal system and epilepsy. A case is presented in a preschool boy whose mother tried to voluntarily interrupt pregnancy in the first trimester but failed to abort. Facial diplegia, bilateral sixth cranial nerve palsy and bilateral equinovarus deformity of the feet were evidenced at birth. At 17 days of life he presented a tonic-clonic seizure and was diagnosed with Moebius syndrome and difficult to treat epilepsy. Delayed overall development, predominantly swallowing and speech difficulties, were evidenced upon follow up...(AU)

A Case of Coincidental Intrasellar Chordoma and Pituitary Adenoma

Although chordomas are midline tumors, primarily intrasellar chordomas are extremely rare. In this report, the authors describe the case of a 68-year-old female with partial abducens nerve palsy in the right eye due to the intrasellar cystic tumor. After endonasal trans-sphenoidal surgery, intraoperative and histopathological findings confirmed the co-occurrence of an entirely intrasellar chordoma and pituitary adenoma. To our knowledge, the present case is the third reported case of an intrasellar chordoma with a pituitary adenoma.