Clinical features and genetic analysis of a patient with type 2 neurofibromatosis manifested as oculomotor nerve palsy / 中华医学遗传学杂志

OBJECTIVE@#To report on a rare case of Neurofibromatosis type 2 (NF2) manifesting as oculomotor nerve palsy and explore its genetic basis.@*METHODS@#A patient with NF2 who had presented at Beijing Ditan Hospital Affiliated to Capital Medical University on July 10, 2021 was selected as the study subject. Cranial and spinal cord magnetic resonance imaging (MRI) was carried out on the patient and his parents. Peripheral blood samples were collected and subjected to whole exome sequencing. Candidate variant was verified by Sanger sequencing.@*RESULTS@#MRI revealed bilateral vestibular Schwannomas, bilateral cavernous sinus meningiomas, popliteal neurogenic tumors, and multiple subcutaneous nodules in the patient. DNA sequencing revealed that he has harbored a de novo nonsense variant of the NF2 gene, namely c.757A>T, which has replaced a codon (AAG) encoding lysine (K) at position 253 with a stop codon (TAG). This has resulted in removal of the Merlin protein encoded by the NF2 gene from position 253 onwards. The variant was not found in public databases. Bioinformatic analysis suggested that the corresponding amino acid is highly conserved. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was rated as pathogenic (PVS1+PS2+PM2_Supporting+PP3+PP4).@*CONCLUSION@#The heterozygous nonsense variant c.757A>T (p.K253*) of the NF2 gene probably underlay the disease in this patient with an early onset, atypical but severe phenotype.

Manifestaciones oftalmológicas del dengue, el zika y el chikungunya / Ophthalmic manifestations of dengue, zika and chikungunya

Las infecciones por arbovirus constituyen un reto significativo para los sistemas de salud. Cada vez se incrementa el reconocimiento de complicaciones del sistema nervioso central secundarias a ellas, lo que puede ser un dilema para su diagnóstico y tratamiento. Los arbovirus pueden alterar los mecanismos de inmunidad innatos del ojo al dañar las barreras óculo-hemáticas. En esta revisión nos propusimos caracterizar los principales hallazgos oftalmológicos de las enfermedades transmitidas por mosquito, como el dengue, el zika y el chikungunya, y su posible fisiopatología. Se realizó una búsqueda de la literatura sobre el tema en la base de datos de PubMED. En los pacientes con zika y chikungunya se reconocieron frecuentemente la conjuntivitis no purulenta y la queratitis. En los casos de dengue el edema macular y las hemorragias retinianas maculares fueron frecuentes; causaron disminución de la visión y defectos campimétricos; la vasculitis y coriorretinitis periférica podía ser asintomática si la mácula no estaba comprometida. Estuvieron implicados la trombocitopenia y otros procesos fisiopatológicos. En las enfermedades estudiadas se reportaron casos raros con parálisis de nervios oculomotores o neuritis óptica como respuesta autoinmune tardía. Recientemente se reportó el síndrome de zika congénito que incluyó múltiples anomalías del desarrollo. En los neonatos afectados se describió la atrofia macular, así como la pigmentación macular bilateral, la hipoplasia del nervio óptico, la catarata, entre otros. Existen diversas lesiones oculares secundarias a infecciones por dengue, zika y chikungunya que merecen reconocimiento, pues deterioran la función visual temporal o permanentemente(AU)

Arbovirus infections pose a significant challenge to health systems. Awareness of the secondary central nervous system complications caused by these infections is on the increase, which may be a dilemma for their diagnosis and treatment. Arboviruses may alter the innate immunity mechanisms of the eye by damaging blood-retinal barriers. The objective of this review was to characterize the main ophthalmological findings of mosquito-borne diseases, such as dengue, zika and chikungunya, and their possible physiopathology. A bibliographic search about the topic was conducted in the database PubMed. Non-purulent conjunctivitis and keratitis were frequently found in zika and chikungunya patients. Dengue cases often presented macular edema and macular retinal hemorrhage, which caused vision reduction, as well as campimetric defects. Vasculitis and peripheral chorioretinitis could be asymptomatic if the macula was not involved. Thrombocytopenia and other physiopathological processes were also present. Oculomotor nerve palsy and optic neuritis as a late autoimmune response were rarely reported in the diseases studied. Recent reports refer to congenital zika syndrome, which causes multiple developmental abnormalities. Macular atrophy, bilateral macular pigmentation, optic nerve hypoplasia and cataract, among other disorders, were described in affected neonates. A variety of ocular lesions secondary to dengue, zika and chikungunya infection deserve recognition, for they damage visual function either temporarily or permanently(AU)

Paresia unilateral do terceiro nervo após uso de Metronidazol oral / Unilateral third nerve palsy after use of oral Metronidazole

Resumo Paciente do sexo feminino, 19 anos, com queixa de diplopia, náusea e vômito de início súbito. Ao exame físico, a paciente apresentava rotação da cabeça para a esquerda e limitação da adução do olho direito, sugerindo paresia do músculo reto medial. Ausência de ptose palpebral ou paresia de outra musculatura ocular extrínseca e sem outras alterações na avaliação oftalmológica. Foi relatado pelo paciente o uso de Metronidazol, duas doses de 500 mg, no mesmo dia em que os sintomas começaram. A ressonância magnética do crânio foi solicitada. O resultado mostrou um cisto da glândula pineal, estando os outros aspectos dentro da normalidade. A paresia do músculo reto medial e diplopia persistiram por 14 dias, mesmo após a suspensão do antibiótico, optando, assim, por iniciar a corticoterapia oral, evoluindo com boa resposta clínica, melhora dos sintomas e regressão da paresia muscular.

Abstract Female patient, 19 years old, with a complaint of diplopia, nausea and vomiting of sudden onset. Upon physical examination, the patient presented herself with the head position rotated to the left and limitation of adduction of the right eye, suggesting paresis of the medial rectus muscle. Absence of palpebral ptosis or paresis of other extrinsic musculature of the eye, and without other alterations in the ophthalmological evaluation. It was reported by the patient the use of Metronidazole, two doses of 500 mg, the same day the symptoms started. The magnetic resonance imaging of the skull was requested. The result showed a cyst of the pineal gland, the other aspects being within normality. The paresis of the medial rectus muscle and diplopia persisted for 14 days, even after the antibiotic was discontinued, thus opting to initiate oral corticosteroid therapy, evolving with good clinical response, improvement of symptoms and regression of muscular paresis.

Etiology, localization of the lesion, and prognosis for patients firstly diagnosed in ophthalmology department with oculomotor nerve palsy / 中南大学学报(医学版)

OBJECTIVES@#Oculomotor nerve palsy is a kind of disease with many causes, showing eye movement disorders, abnormal eyelid position, and/or damage of the pupil. The etiology of oculomotor nerve palsy in different departments is different. The study discussed the etiology, localization of the lesion, and prognosis for oculomotor nerve palsy firstly diagnosed in department of ophthalmology.@*METHODS@#Clinical data of 137 hospitalized patients with oculomotor nerve palsy at the Department of Ophthalmology, the First Medical Center of PLA General Hospital from 2009 to 2018 were retrospectively collected. The etiology and its distribution characteristics in different age groups, the location of the lesion, and the prognosis of patients were analyzed.@*RESULTS@#In 137 patients, the top 3 causes for oculomotor nerve palsy were head trauma (38.69%), cavernous sinus lesions (12.40%), and orbital inflammation (9.49%). Other causes included intracranial aneurysm, the intracranial space-occupying lesion, cerebral vessel diseases, infection, orbital tumors, diabetes, the operation of nasal cavity. Traumatic oculomotor nerve palsy was more common in young adults aged 20-49 years and in the patients with cerebral vascular disease in elderly people aged 60-69 years, while diabetic oculomotor nerve palsy is common in middle-aged and elderly people aged 50-69 years. The age distribution of other etiological types was relatively balanced. Seventy-five cases of orbital apex lesions were due to trauma, inflammation, infection, and tumor; 40 cases of cavernous sinus lesions were due to inflammation, tumor, and thrombosis; 6 cases of subarachnoid lesions were due to aneurysms, tumors, and trauma; 5 cases were oculomotor nucleus lesions were due to infarction; 11 cases could not be allocated because of unknown etiology. After treatment, the corrected visual acuity of oculomotor nerve palsy side was not significantly improved. The patients with oculomotor nerve palsy caused by intracranial aneurysm, cerebrovascular disease, and diabetes mellitus had the highest proportion of partial or complete recovery from ptosis and ocular dyskinesia.@*CONCLUSIONS@#Oculomotor nerve palsy is a common cause of ophthalmoplegia and diplopia. Head trauma, cavernous sinus lesions, and orbital inflammation are the most common causes for oculomotor nerve palsy first diagnosed in ophthalmology department. Traumatic oculomotor nerve palsy is common in adolescents. Oculomotor nerve palsy caused by diabetes and cerebrovascular disease are common in the middle-aged and elderly people. Most of the lesions locate in the orbital apex and cavernous sinus. The prognosis of corrected visual acuity is poor. The prognosis of ptosis and ocular dyskinesia caused by intracranial aneurysm, cerebrovascular disease, and diabetes is good. Figuring out the cause timely and accurately is the basis and key to treat oculomotor nerve palsy.

Regeneración aberrante del III nervio craneal por aneurisma cerebral bilateral. Reporte de caso / Aberrant regeneration of the III cranial nerve due to bilateral cerebral aneurism

RESUMEN Los trastornos de la motilidad ocular constituyen motivo de consulta periódica en Oftalmología. La regeneración aberrante, trastorno muy poco reportado, es considerada la sincinesia oculomotora de mayor invalidez y complejidad. Diversas condiciones neuroftalmológicas están implicadas en la etiopatogenia de la enfermedad, la mayoría de las cuales puede ocasionar la muerte. El manejo de los síntomas y signos provocados por paradójicos movimientos oculares conjugados es difícil. Se reportó un caso con remisión tardía a neuroftalmología por diagnóstico inicial y evolución desfavorable. La historia psicofísica arrojó diagnóstico definitivo de regeneración aberrante del III nervio craneal secundario, a aneurisma cerebral de la carótida interna bilateral, agravado por reanastomosis quirúrgica. Una rigurosa, obligatoria e impostergable historia neuroftalmológica, se impone ante toda parálisis del III nervio craneal para brindar un diagnóstico etiológico preciso y de esta forma proteger la vida.

ABSTRACT The disturbances in ocular motility are the cause of periodical consultation in Ophthalmology. The aberrant regeneration, a scarcely reported disturbance, is considered the oculomotor synkinesis of highest disability and complexness. Several neuro-ophthalmologic conditions are implicated in the disease ethiopathogeny, and most of them could lead to death. The management of the symptoms and signs caused by paradoxical conjugated ocular movements is difficult. A case is reported of late remission to Neuro-ophthalmology due to unfavorable diagnosis and evolution. The psycho-physical history led to a definitive diagnosis of aberrant regeneration of the III secondary cranial nerve, to cerebral aneurism of the bilateral internal carotid, worsened by surgical re-anastomosis. In front of any paralysis of the III cranial nerve, it is necessary a rigorous, obligatory and immediate neuro-ophthalmological history to arrive to a precise etiological diagnosis, protecting life in that way.

Pituitary Apoplexy Presenting as Isolated Bilateral Oculomotor Nerve Palsy

PURPOSE: To report a case of pituitary apoplexy presenting as isolated bilateral oculomotor nerve palsy. CASE SUMMARY: A 46-year-old male presented with bilateral ptosis and acute severe headaches for 6 days. He underwent head surgery and bilateral vitrectomy 12 years prior to his visit because of ocular and head trauma. He mentioned that previous visual acuities in both eyes were not good. The initial corrected visual acuity was finger counting in the right eye and 20/500 in the left eye. Ocular motility testing revealed the limitation of adduction, supraduction, and infraduction with complete bilateral ptosis in both eyes, and his left pupil was dilated. He was diagnosed with an isolated bilateral oculomotor nerve palsy. Magnetic resonance imaging indicated pituitary gland hemorrhage with a tumor, which was suspicious of pituitary apoplexy. The patient was treated intravenous with 1.0 g methylprednisolone to prevent the corticotropic deficiency. In addition, he underwent surgical decompression using a navigation-guided transsphenoidal approach and aspiration biopsy. He was confirmed with pituitary adenoma using a pathological examination. The patient's ocular movements began to dramatically improve by the third day postoperatively. At 4 months postoperative follow-up, his ocular movement and double vision were completely recovered. CONCLUSIONS: This was a rare case of pituitary apoplexy with bilateral isolated oculomotor nerve palsy, which was the first report in the Republic of Korea. A full recovery was achieved after early surgical treatment.

A Successful Stent-Assisted Coil Embolization in a Patient with Oculomotor Palsy Due to Partially Thrombosed-Aneurysm in Cavernous Internal Carotid Artery

No abstract available.

Incomplete Oculomotor Nerve Palsy Caused by Internal Carotid Artery Giant Aneurysm with Fibromuscular Dysplasia

No abstract available.

Recurrent Painful Ophthalmoplegic Neuropathy: a Case Report

Upon review, it is noted that recurrent painful ophthalmoplegic neuropathy (RPON) is a rare neurological syndrome characterized by recurrent unilateral headaches and painful ophthalmoplegia of the ipsilateral oculomotor nerve. As seen on brain MRI, thickening and enhancement of the oculomotor cranial nerve can be observed in these cases. We experienced a case of RPON in an adult patient who showed thickening and enhancement of the oculomotor nerve on gadolinium-enhanced 3D-FLAIR image. The authors report a case of RPON with a review of the literature.

Angioleiomyoma in the Orbital Apex: A Case Report

A 56-year woman presented eyeball pain and blurred vision. MRI revealed a small well-delineated solid tumor in the apex of right orbit with optic nerve compression. Intraoperatively, the tumor was found very fibrous, hypervascular and adhesive to surrounding structures. The tumor was completely removed with the combination of endoscopic and microscopic technique. Patient experienced transient oculomotor nerve palsy, which completely recovered 3 months after surgery. Herein we report a rare case of angioleiomyoma in the orbital apex.

Oculomotor nerve palsy associated with internal carotid artery: case reports

Oculomotor nerve palsy limits the specific direction eyeball movement, and represents diplopia, mydriasis, and ptosis. The vascular-associated etiologies of oculomotor nerve palsy are the microvascular ischemia due to hypertension or diabetes, or compression of the nerve by the aneurysm. For the aneurysm, if not treated properly, it may result in mortality or severe neurological impairment. Thorough history taking, physical examinations, and proper imaging modality are needed to make an accurate diagnosis. A 76-year-old female with decreased mentality and anisocoria presented at our emergency department. An 83-year-old female presented with right ptosis and lateral-side deviated of the right eyeball. No definite lesion was noted on the initial non-contrast brain computed tomography (CT) and magnetic resonance imaging diffusion. An aneurysm was detected on CT angiography taken several hours later in the former patient. For the latter patient, a giant aneurysm was detected on magnetic resonance angiography that had been performed at another hospital 4 days earlier. These two patients underwent transfemoral cerebral angiography with coiling. They were discharged with no neurological sequelae.

Arteritis de células gigantes en el sistema vertebrobasilar, isquemias cerebelo-occipitales y parálisis III par: caso clínico / Giant cell arteritis of the vertebrobasilar system, posterior ischemia and palsy of the third nerve: report of one case

Giant cell arteritis is the most common vasculitis in patients aged over 50 years. We report an 89-year-old woman with significant weight loss and persistent frontal-occipital headaches lasting two months. The neurological examination at admission identified a decrease in visual acuity of the left eye, paralysis of the third cranial nerve of the right eye and alterations of body motility without objective signs of damage of the motor or sensitive pathways. Magnetic resonance imaging showed changes of the temporal artery wall and in both vertebral arteries, as well as bilateral cerebellar and occipital ischemic lesions. The Doppler ultrasound of the temporal arteries was compatible with Giant cell arteritis. Treatment with steroids was started. While receiving oral prednisone, the patient suffered new infarcts of the posterior territory, documented with a CAT scan.

Amaurosis and contralateral cranial nerve pairs III and VI paralysis after peribulbar block - Case report / Amaurose e paralisia do III e do VI pares cranianos contralaterais após bloqueio peribulbar - relato de caso

Abstract Background and objectives: Peribulbar anesthesia has emerged as a safer option compared with intraconal retrobulbar block. Still, peribulbar anesthesia may not be considered without risk. Numerous complications have been described when performing this technique. This report aims to describe a rare case of amaurosis and contralateral paralysis while attempting to perform a peribulbar anesthesia. Case report: Male patient, 75-year old, physical status ASA II, undergoing cataract surgery by phacoemulsification with intraocular lens implantation. Sedated with fentanyl and midazolam and subjected to peribulbar anesthesia. There were no complications during surgery. After finishing the procedure, the patient reported lack of vision in the contralateral eye. Akinesia of the muscles innervated by the cranial nerve pairs III and VI, ptosis, and medium-sized pupils unresponsive to light stimulus were observed. Four hours after anesthesia, complete recovery of vision and eyelid and eyeball movements was seen in the non-operated eye. Conclusions: During peribulbar anesthesia, structures located in the intraconal space can be accidentally hit leading to complications such as described in the above report. Following the technical guidelines and using appropriate size needles may reduce the risk of such complication, but not completely.

Resumo Justificativa e objetivos: A anestesia peribulbar surgiu como uma opção mais segura quando comparada com o bloqueio retrobulbar intraconal. Ainda assim, a anestesia peribulbar não pode ser considerada isenta de riscos. Inúmeras complicações foram descritas quando da aplicação dessa técnica. O presente relato tem como objetivo descrever um caso raro caracterizado por amaurose e paralisia contralaterais quando da tentativa de se fazer a anestesia peribulbar. Relato de caso: Paciente masculino, 75 anos, estado físico ASA II, submetido à facectomia por facoemulsificação com implante de lente intraocular. Sedado com fentanil e midazolam e submetido a APB. Não houve intercorrências durante a cirurgia. Após o término do procedimento o paciente relatou ausência de visão no olho contralateral. Foram observadas acinesia da musculatura inervada pelo III e VI pares cranianos, ptose palpebral e pupilas de tamanho médio, não responsivas ao estímulo luminoso. Após quatro horas da anestesia, houve recuperação completa da visão, da movimentação das pálpebras e do globo ocular não operado. Conclusões: Durante a APB, estruturas localizadas no espaço intraconal podem ser atingidas acidentalmente levando a complicações como a descrita no relato acima. O respeito às diretrizes técnicas e o uso de agulhas com o tamanho adequado podem reduzir o risco de tal complicação, mas não de forma completa.

Sutura de tração permanente para tratamento de paralisia congênita de III nervo / Permanent treatment suture for the treatment of congenital nerve palsy of III nerve

Resumo A paralisia do terceiro nervo craniano representa o estrabismo paralítico de tratamento mais complexo e desafiador. Os casos de paralisia completa III par incitam o uso de certas técnicas de cirurgia de estrabismo destinadas a manter o olho voltado para a posição primária do olhar (PPO). Entretanto, as possibilidades terapêuticas são limitadas e complexas e o tratamento cirúrgico tende a hipocorreção e recorrências frequentes a longo prazo.O envolvimento completo e congênito do terceiro nervo craniano requer cirurgias para a exotropia, hipotropia e ptose.Dentre as técnicas cirúrgicas já descritas, optou-se pela realização de uma modificação da técnica cirúrgica de recuo-ressecção, que deu-se em único tempo cirúrgico, sendo suficiente para alcançar o objetivo estético. Este trabalho relata o resultado positivoda manutenção de sutura de tração à carúncula para tratamento cirúrgico de estrabismo paralítico congênito de nervo oculomotor de longa data.

Abstract Paralysis of the third cranial nerve represents the most complex and challenging paralytic squint. The cases of complete III nerve paralysis encourages the use of certain strabismus surgery techniques in order to keep eye in primary position of gaze. However, the therapeutic possibilities are limited and complex and the surgical treatment tends to hypocorrection and frequent recurrences in the long term. Complete and congenital involvement of the third cranial nerve requires surgeries for exotropia, hypotropia and ptosis. Among the surgical techniques already described, we choose a modification of the surgical technique of recession-resection, which occurred in a single surgical time, being suffice to achieve aesthetic objective. This paper reports the positive result of the maintenance of caruncle traction suture as surgical treatment of congenital III nerve paralysis.

Gliomatosis cerebri reporte de un caso y revisión de la literatura / Gliomatosis Cerebri case report and literatura review

La Gliomatosis Cerebri (GC) se define como un compromiso infiltrativo difuso de un tumor glial generalmente astrocítico que compromete al menos 3 lóbulos cerebrales de manera bilateral y usualmente la afección se extiende al tallo cerebral. Su incidencia representa aproximadamente el 1% de la totalidad de los tumores encefálicos y se presenta con mayor frecuencia en adultos jóvenes. El diagnóstico se establece por medio de la clínica, la imagenología y confrimación histológica. Las posibilidades terapéuticas son limitadas y la sobrevida cercana a los 2 años mostrando una alta mortalidad. Reportamos el caso de un paciente masculino de 11 años de edad quien fue atendido en el Hospital Militar Central de Bogotá Colombia donde se realizó el diagnóstico y se instauró el tratamiento

Gliomatosis Cerebri (GC) is defined as a diffuse infiltration of a glial tumor generally astrocytic that involves at least 3 cerebral lobes bilaterally and usually the condition extends to the brainstem. Its incidence represents approximately 1% of all brain tumors and occurs more frequently in young adults. The diagnosis is established for the clinic environment, imaging and histological confirmation. The therapeutic possibilities are limited and the survival close to 2 years showing a high mortality. We report the case of an 11-year-old male patient who was treated at the Central Military Hospital of Bogotá Colombia where the diagnosis was made and treatment was instituted

Periosteal Fixation Applied to Patients with Large-angle Paralytic Strabismus

PURPOSE: To evaluate the effect of periosteal fixation in patients with large-angle paralytic strabismus that was not corrected through conventional strabismus surgery. METHODS: Four eyes of three patients with large-angle paralytic strabismus who underwent periosteal fixation from June 2014 to August 2014 were examined. All patients presented with exotropia > 50 prism diopters (PD). Two of them showed exotropia caused by chronic complete oculomotor nerve palsy; the other two showed exotropia caused by medial rectus muscle injury during endoscopic sinus surgery. RESULTS: The mean preoperative exodeviation using the Krimsky test was 58 ± 29 PD. The postoperative values were 6.5 ± 9.4 PD at 1 week, and 11.25 ± 2.5 PD at 6 months. The mean surgical effect of exodeviation was 43.75 ± 21.36 PD. CONCLUSIONS: Periosteal fixation is an effective surgery for the management of paralytic strabismus that was not corrected through conventional strabismus surgery.

A Case of Partial Oculomotor Nerve Palsy Caused by Vascular Compression in Idiopathic Intracranial Hypertension / 대한평형의학회지

Pupil-involving oculomotor nerve palsy (ONP) is frequently associated with compressive lesion such as intracranial aneurysm originating from the posterior communicating arteries. Vascular variant of posterior intracranial circulation is regarded as an uncommon cause and association between these vascular variants and intracranial hypertension has not been reported. We present an 18-year-old girl with pupil-involving ONP combined with idiopathic intracranial hypertension who revealed compression of oculomotor nerve by a vascular variant of superior cerebellar artery (SCA). This is a rare case of an ONP attributed to compressive effect from an aberrant SCA affected by intracranial hypertension.

Using 17-gauge Spinal Anesthesia Needle as an Insertion Guide for Frontalis Sling Surgery with Silicone Rod

PURPOSE: In this study we investigated the surgical outcome and effectiveness of using a 17-gauge spinal anesthesia needle for guiding insertion of the silicone rod in frontalis sling surgery for patients having severe myogenic or neurogenic ptosis with risk of exposure keratopathy. METHODS: This study included on 8 patients (11 eyes) who previously received frontalis sling surgery with a 17-gauge spinal anesthesia needle because of severe myogenic or neurogenic ptosis with risk of exposure keratopathy. We investigated the technique, surgical outcome, and clinical features following frontalis sling surgery, and evaluated the advantages of using a 17-gauge spinal anesthesia needle. RESULTS: The mean age of patients was 54 years. Third nerve palsy was the most common etiology in severe ptosis with risk of exposure keratopathy (5 of 8, 62.5%). Main advantages of the 17-gauge spinal anesthesia needle are smaller skin incision that allows for minimal damage during tissue passage and easy guide for insertion. Other advantages included sterilization, minimizing infections, sharpness without distortion because it is disposable, and economic value. The mean follow-up period was 21.4 months, and the upper lid margin of all patients was adequately high above the pupil margin. Additionally, no major complications were observed in the patients. CONCLUSIONS: Frontalis sling surgery with a silicone rod is a safe and effective method for patients with severe myogenic or neurogenic ptosis with risk of exposure keratopathy. The 17-gauge spinal anesthesia needle is useful and economical in frontalis sling surgery for guiding insertion of the silicon rod as an alternative option to the Wright needle.

Imaging of Cranial Nerves III, IV, VI in Congenital Cranial Dysinnervation Disorders

Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in aberrant innervation of the ocular and facial musculature. Its diagnosis could be facilitated by the development of high resolution thin-section magnetic resonance imaging. The purpose of this review is to describe the method to visualize cranial nerves III, IV, and VI and to present the imaging findings of congenital cranial dysinnervation disorders including congenital oculomotor nerve palsy, congenital trochlear nerve palsy, Duane retraction syndrome, Möbius syndrome, congenital fibrosis of the extraocular muscles, synergistic divergence, and synergistic convergence.