Síndrome de pseudo-Duane secundario a Schwannoma de nervio abducens: Reporte de caso y revisión / Pseudo-Duane syndrome secondary to abducens nerve schwannoma: Case report and review

Reporte de caso de síndrome de pseudo-Duane asociado a schwannoma de nervio abducens y neurofibromatosis tipo 2 (NF2). Este síndrome es raro, caracterizado por limitación en abducción, retracción ocular, disminución de hendidura palpebral en abducción y restricción del recto medial ipsilateral en test de ducción forzada. La NF2 es también una enfermedad infrecuente caracterizada por tumores de sistema nervioso central y periférico. Los schwannomas de nervio abducens son también poco frecuentes, y se presentan comúnmente con diplopia. Se revisan brevemente estas enfermedades. Es el primer caso reportado de pseudo-Duane secundario a schwannoma de nervio abducens y NF2 según nuestro conocimiento.

A pseudo-Duane syndrome case associated with abducens nerve schwannoma and neurofibromatosis type 2 (NF2) is presented. This syndrome is a rare disease characterised by abduction limitation, ocular retraction, narrowing of the palpebral fissure in abduction, and ipsilateral medial rectus restriction on forced duction test. NF2 is also an uncommon disease which is characterised by peripheral and central nervous system tumours. Abducens nerve schwannomas are also uncommon and presents usually as diplopia. A short review of these diseases is given. This is the first case of pseudo-Duane secondary to abducens nerve schwannoma, to our knowledge.

Síndrome de Pierre Robin associada à Síndrome de Duane familiar do tipo III / Pierre Robin Syndrome associated with type III familial Duane Retraction Syndrome

Resumo A síndrome de Pierre Robin (PRS) consiste em uma tríade de anomalias caracterizada por micrognatia, glossoptose e fissura de palato, comumente associada com outras síndromes e ocasionalmente com alterações oculares. Na Síndrome de Duane (DRS), há uma falha na inervação do reto lateral pelo VI nervo, com inervação anômala do reto lateral por fibras do III nervo. Ainda que a PRS já tenha sido associada com mais de 50 outras síndromes, não existe na literatura relato de casos de associação com a DRS familiar. Dessa forma, esse trabalho tem por objetivo relatar um caso dessa associação em um paciente de 29 anos com recorrência das síndromes na família.

Abstract The Pierre Robin Syndrome (PRS) consists of a triad of anomalies characterized by micrognathia, glossoptosis and fissure of the palate, usually associated with other syndromes e occasionally associated with ocular variations. In Duane Retraction Syndrome (DRS), there is a failure in the lateral rectus innervation by the VI cranial nerve, with anomalous innervation of the lateral rectus by fibers of the III nerve. Even though PRS has already been associated with more than 50 other syndromes, there is not any report in literature of association with familial DRS. Thus, this work aims to report a case of this association in a 29 years old patient with recurrence of the syndromes in the family.

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.

Ossification of the roof of the porus trigeminus with duplicated abducens nerve / 대한해부학회지

Ossification of parts of the intracranial dura mater is common and is generally accepted as an age-related finding. Additionally, duplication of the abducens nerve along its course to the lateral rectus muscle is a known, although uncommon anatomical variant. During routine cadaveric dissection, an ossified portion of dura mater traveling over the trigeminal nerve's entrance (porus trigeminus) into the middle cranial fossa was observed unilaterally. Ipsilaterally, a duplicated abducens nerve was also observed, with a unique foramen superolateral to the entrance of Dorello's canal. To our knowledge, there has been no existing report of a simultaneous ossified roof of the porus trigeminus with an ipsilateral duplicated abducens nerve. Herein, we discuss this case and the potential clinical and surgical applications. We believe this case report will be informative for the skull base surgeon in the diagnosis of neuralgic pain in the frontomaxillary, andibular, orbital, and external and middle ear regions.

Endoscopic-Assisted Infralabyrinthine Approach to Petrous Apex Cholesterol Granuloma / 대한이비인후과학회지

A cholesterol granuloma is the most common primary lesion of the petrous apex, and accounts for 40% of the pathologies that arise in this region. The primary treatment for symptomatic lesions is by surgery to decompress and drain or to completely remove the lesion. Here we describe the use of infralabyrinthine approach to access a lesion through the temporal bone and completely remove it with the assistance of a 0-degree endoscope. A 43-year-old man visited our clinic for diplopia. Magnetic resonance imaging revealed a 2.3-cm cholesterol granuloma located in the left petrous apex that caused deviation of the left abducens nerve. The tumor was completely removed using the endoscopic-assisted infralabyrinthine approach; the patient is currently being followed up, and there is no evidence of disease recurrence. This case report describes the successful completion of a petrous apex cholesterol granuloma that preserved the cochlear and vestibular systems.

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.

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.

Dorello´s canal or abducens nerve canal: constancy or inconstancy? / El canal de Dorello o canal para el nervio abducente: ¿constancia o inconstancia?

Dorello´s canal is an eponym term referring to a small fibro osseous landmark, by way of arc, located at the apex of the petrous temporal region and for which the nerve abducens passes before reaching the cavernous sinus. This landmark is not referenced in Terminologia Anatomica, even as a variable structure. We selected 74 adult dry skulls and six heads fixed in formalin 10 % without classifying them by ethnicity or gender. As inclusion criteria, we selected those where the middle cranial fossa and the apex of the petrous temporal region were in good condition. Fifty seven (57) skulls (83, 82 %) showed the presence of canal bilaterally; 4 (5.89 %) showed it unilaterally to the right; 4 (5,89 %) were unilaterally at the left and 3 (4,41 %) had no canal. The results of this study indicate that this bone landmark is present in most of the skulls, even bilaterally. Since it is not referenced in the Terminologia Anatomica, we propose the term canal abducens nerve to avoid using the eponymous term Dorello´s canal.

El "canal de Dorello" es un epónimo que hace referencia a un pequeño relieve osteofibroso, a manera de arco, ubicado a nivel del ápex de la región petrosa del temporal y por el cual pasa recostado el nervio abducente antes de llegar al seno cavernoso. Dicho relieve no se encuentra referenciado en Terminologia Anatomica, ni siquiera como estructura inconstante. Se seleccionaron 74 cráneos secos y completos de adultos y seis cabezas fijadas con formalina al 10 %, sin clasificarlos por grupos étnicos ni por sexo. Como criterio de inclusión, se escogieron aquellos que tuvieran en buen estado la fosa craneal media y el ápex de la región petrosa del temporal. 57 cráneos (83,82 %) mostraron presencia del canal en forma bilateral; 4 (5,89 %) lo mostraron unilateralmente a la derecha; 4 (5,89 %) lo tenían unilateralmente a la izquierda y 3 (4,41 %) no presentaban el canal. Los resultados de este estudio señalan que este relieve óseo se presenta en la mayoría de los cráneos, incluso en forma bilateral. Puesto que no aparece referenciado en Terminologia Anatomica, proponemos el término "canal para el nervio abducente", con el fin de evitar el uso del término epónimo "canal de Dorello".

Paresia recurrente de sexto nervio craneano asociado a hipoplasia de sexto nervio / Recurrent paresis of sixth cranial nerve associated with sixth nerve hypoplasia

La paresia de sexto nervio craneano recurrente es una entidad ampliamente conocida en la población pediátrica que ha sido asociado a múltiples etiologías, siendo las más frecuentes las infecciones virales o postinmunizaciones. A continuación, exponemos el caso de una niña de 5 años que presenta 2 episodios de déficit de abducción del ojo izquierdo debido a paresia de sexto nervio craneano, que se resolvieron espontáneamente. El estudio con resonancia magnética reveló hipoplasia del sexto nervio craneano izquierdo.(AU)

Recurrent sixth nerve palsy is a well-known entity in pediatric population associated to multiple causes, most frequently viral infection or immunization. We report a case of a 5 years old girl who presents two episodes of left abduction deficit due to sixth nerve palsy that partially resolved spontaneously. A magnetic resonance imaging study revealed a hypoplastic left sixth nerve.(AU)

Clinical Features of Acquired Paralytic Strabismus

PURPOSE: The purpose of our study was to evaluate the cause of acquired third, fourth, and sixth nerve palsy while also establishing recovery rates and important factors for recovery. METHODS: A retrospective chart review was performed for 92 patients who visited the ophthalmologic department of Konyang University Hospital with acquired third, fourth, and sixth nerve palsy from March 2015 to February 2016. Recovery rates and factors for recovery were evaluated in only 66 patients who received first ocular exam within 2 weeks of onset and who were followed up for at least 6 months. Complete recovery was defined as both complete recovery of the angle of deviation and the restoration of eye movement in all directions. For the degree of ocular motor restriction, −4 was defined as not crossing the midline and −2 was defined as 50% eye movement. The degree of ocular motor restriction was analyzed from −1/2 to 4. RESULTS: The fourth nerve was affected most frequently (n = 37, 40.2%), followed by the sixth cranial nerve (n = 33, 35.9%), the third cranial nerve (n = 18, 19.6%), and a combination of 2 or more cranial nerves (n = 4, 4.3%). Vasculopathy (n = 44, 47.8%) was the most common etiology, followed by trauma (n = 14, 15.2%), idiopathic (n = 13, 14.1%), inflammation(n = 10, 10.9%), neoplasm (n = 9, 9.8%), and aneurysm (n = 2, 2.2%). Complete recovery rate occurred for 66.7% (n = 44) of patients, and the overall recovery rate (i.e., at least partial recovery) was 86.3% (n = 57). Significant factors for complete recovery were the initial deviation angle and the limitation of extraocular movement (p < 0.001, p = 0.005, respectively, according to univariate analysis). CONCLUSIONS: In this study, paralytic strabismus due to vasculopathy was the most common etiology, and a lower degree of initial deviation resulted in an improved complete recovery rate. In addition, a high overall recovery rate was possible through quick diagnosis and early treatment of cranial nerve palsy.

A Case of Idiopathic Unilateral Abducens Nerve Palsy in an Adolescent Patient

PURPOSE: To report a case of idiopathic unilateral abducens nerve palsy in an adolescent patient. CASE SUMMARY: A healthy 14-year-old boy presented with binocular horizontal diplopia that started that same day. He did not have any history of trauma, vaccination or infectious disease and showed no other neurological signs. The best corrected visual acuity of both eyes was 20/20. Slit lamp and fundus examinations revealed no other abnormalities. On the alternative prism cover test, he had 6 prism diopter (PD) esotropia with −1 degree abduction limitation in the left eye. After 1 week, the abduction limitation in the left eye was progressed to −3.5 degrees, so we performed brain imaging. The brain magnetic resonance imaging (MRI) and systemic evaluation (serologic test, cerebrospinal fluid examination) were normal therefore, we suspected idiopathic unilateral abducens nerve palsy. Thus, intravenous steroid injection was started, and the patient was partially recovered after 5 days of treatment. At 5 weeks after presentation, diplopia and abduction limitation in the left eye were completely resolved, and recurrence was not found during the 1 year of follow-up. CONCLUSIONS: We report a case of progressive idiopathic unilateral abducens nerve palsy in an adolescent which has resolved completely after intravenous steroid treatment in a short period.

The Characteristics and Prognosis of Miller Fisher Syndrome

PURPOSE: In the present study, the clinical characteristics and prognosis of patients clinically diagnosed with classic Miller Fisher syndrome were evaluated. METHODS: We retrospectively investigated the clinical and laboratory findings as well as treatment outcomes using the medical records of patients diagnosed with Miller Fisher syndrome. Symptom triad including acute ophthalmoplegia, ataxia, and areflexia were evaluated. RESULTS: This study included 10 patients. Nine patients had antecedent infectious illness which took an average of 11 ± 9.7 days for onset of diplopia from antecedent infectious systemic illness. Seven patients showed bilateral paralytic strabismus. Specifically, 5 patients showed the involvement of vertical and horizontal extraocular muscles. Pupil impairment and blepharoptosis were observed in 4 patients, limb weakness in 3 patients, dysarthria in 3 patients and facial palsy in 1 patient. Two patients showed contrast enhancement of the abducens nerve on brain magnetic resonance imaging (MRI) and 2 patients showed albumin-cell dissociation on cerebrospinal fluid (CSF) analysis. Eight patients had anti-GQ1b antibodies in their blood serum analysis. Six patients were treated with intravenous immunoglobulins and the other patients were observed with regular follow-ups. The duration of diplopia was 2.9 ± 1.2 months in the treatment group and 3.1 ± 1.7 months in the control group (p > 0.05). The duration of ataxia was 1 ± 0.4 months in the treatment group and 1 ± 0.9 months in the control group (p > 0.05). CONCLUSIONS: Miller Fisher syndrome should be considered in patients with antecedent infection; acute ophthalmoplegia, ataxia and areflexia as well as anti-GQ1b antibody can be helpful for diagnosis. Final outcomes in the treated group were not significantly different from the control group and all patients showed good final outcomes.

A Case of Tolosa-Hunt Syndrome Presented with Headache, Ptosis and Vision Loss

Cavernous sinus syndrome (CSS) is defined as the involvement of two or more of the third, fourth, fifth (V1, V2) or sixth cranial nerves or involvement of only one of them in combination with a neuroimaging-confirmed lesion in the cavernous sinus. Some cases of CSS are attributed to Tolosa-Hunt syndrome (THS), an idiopathic inflammatory disease of the cavernous sinus. THS is characterized by painful ophthalmoplegia due to granulomatous inflammation in the cavernous sinus. THS is a diagnosis of exclusion that requires a vigorous series of differential diagnoses, and corticosteroid therapy is known to dramatically resolve clinical findings of THS. We report a case of a patient with painful ophthalmoplegia associated with vision loss, which was suspected to be THS. This patient followed a relatively typical clinical course of THS on steroid pulse therapy. We emphasize the differential diagnosis of THS, its presentation, and treatment.

Risk Factors and Prognosis of Isolated Ischemic 3rd, 4th, 6th Cranial Nerve Palsy

PURPOSE: To investigate the clinical features and risk factors of ischemic third, fourth, sixth cranial nerve palsy. METHODS: Retrospectively, we reviewed the medical records of 46 eyes of 46 patients who were diagnosed with ischemic third, fourth, sixth nerve palsy alone such as age of onset, risk factors, recovery rate and recovery time. RESULTS: The mean age of onset was 64.9 years. Of the 46 patients, 15 patients (32.6%) in third cranial nerve palsy group, 15 patients (32.6%) in fourth cranial nerve palsy group, 16 patients (34.8%) in sixth cranial nerve palsy group. The risk factor of hypertension in 30 patients (65.2%) was the most common than other risk factors such as diabetes, hyperlipidemia, elevated blood hematocrit, ischemic heart disease, left ventricular hypertrophy, smoking. The mean number of risk factors was 2.3 +/- 0.5 in third cranial nerve palsy group, 1.6 +/- 1.1 in sixth cranial nerve palsy group, 1.4 +/- 1.1 in fourth cranial nerve palsy group. Of the 46 patients, 42 patients (91.3%) were recovered. There was no significant difference in recovery rate by cranial nerve palsy. Recovery time of intracranial abnormalities group (10.5 +/- 2.9 weeks) in brain imaging study was late as compared with that of no intracranial abnormalities group (7.5 +/- 5.1 weeks). CONCLUSIONS: The overall recovery rate of isolated ischemic third, fourth, sixth cranial nerve was high. But if there are intracranial abnormalities in imaging study, it took a long time to recover. Also ischemic third cranial nerve palsy had multiple risk factors characteristically.

Risk Factors and Prognosis of Isolated Ischemic 3rd, 4th, 6th Cranial Nerve Palsy

PURPOSE: To investigate the clinical features and risk factors of ischemic third, fourth, sixth cranial nerve palsy. METHODS: Retrospectively, we reviewed the medical records of 46 eyes of 46 patients who were diagnosed with ischemic third, fourth, sixth nerve palsy alone such as age of onset, risk factors, recovery rate and recovery time. RESULTS: The mean age of onset was 64.9 years. Of the 46 patients, 15 patients (32.6%) in third cranial nerve palsy group, 15 patients (32.6%) in fourth cranial nerve palsy group, 16 patients (34.8%) in sixth cranial nerve palsy group. The risk factor of hypertension in 30 patients (65.2%) was the most common than other risk factors such as diabetes, hyperlipidemia, elevated blood hematocrit, ischemic heart disease, left ventricular hypertrophy, smoking. The mean number of risk factors was 2.3 +/- 0.5 in third cranial nerve palsy group, 1.6 +/- 1.1 in sixth cranial nerve palsy group, 1.4 +/- 1.1 in fourth cranial nerve palsy group. Of the 46 patients, 42 patients (91.3%) were recovered. There was no significant difference in recovery rate by cranial nerve palsy. Recovery time of intracranial abnormalities group (10.5 +/- 2.9 weeks) in brain imaging study was late as compared with that of no intracranial abnormalities group (7.5 +/- 5.1 weeks). CONCLUSIONS: The overall recovery rate of isolated ischemic third, fourth, sixth cranial nerve was high. But if there are intracranial abnormalities in imaging study, it took a long time to recover. Also ischemic third cranial nerve palsy had multiple risk factors characteristically.

Acute Bilateral Mydriasis and Abducens Nerve Palsy with anti-GQ1b Antibody

Ophthalmoplegia without ataxia has various etiologies. An atypical Miller Fisher syndrome implies an ophthalmoplegia without ataxia, areflexia or both. The presence of anti-GQ1b antibody supports the diagnosis of an atypical Miller Fisher syndrome. A 4-year-old Russian girl visited our hospital because of acute bilateral abducens nerve palsy and mydriasis. Although the muscle power of extremities was normal and she didn't show an ataxia, the deep tendon reflex of both knees and ankles was absent. The results of nerve conduction study and cerebrospinal fluid analysis were normal. Magnetic resonance imaging (MRI) showed an enhancement of the bilateral abducens nerve. The anti-Gq1b antibody titer was elevated. The diagnosis of atypical Miller Fisher syndrome was made and a therapy with intravenous immunoglobulins led to the clinical recovery. We report a girl with atypical Miller Fisher syndrome with acute bilateral abducens nerve palsy and mydriasis, diagnosed by of anti-GQ1b antibody positivity.