• Title/Summary/Keyword: ophthalmoplegia

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Adult-onset Leigh syndrome due to m.9176T>C mutation with cortical involvement

  • Lee, Hyuk-je;Lee, Myungah;Lee, Jung Hwan
    • Annals of Clinical Neurophysiology
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    • v.24 no.2
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    • pp.107-110
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    • 2022
  • We describe the case of a 22-year-old female complaining of ophthalmoplegia, which deteriorated with seizure. Leigh syndrome (LS) was diagnosed by identifying the m.9176T>C mutation. She improved with vitamin cocktail therapy plus intravenous methylprednisolone, and had an excellent prognosis. This was the first case of an adult patient with LS presenting with the m.9176T>C mutation and reporting cortical symptoms, which in this case comprised cortical vision loss, and cortical, basal ganglia, and brainstem signal changes on magnetic resonance imaging.

Treatment of Eyelid Ptosis due to Kearns-Sayre Syndrome Using Frontalis Suspension

  • Weitgasser, Laurenz;Wechselberger, Gottfried;Ensat, Florian;Kaplan, Rene;Hladik, Michaela
    • Archives of Plastic Surgery
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    • v.42 no.2
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    • pp.214-217
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    • 2015
  • Blepharoptosis is a common indication for surgery in plastic surgery units, yet its possible underlying pathology frequently remains unidentified. A 52-year-old man with a 20-year history of progressive bilateral ptosis (right>left) presented with recurrent ptosis of both eyes; he had undergone an operation on the levator aponeurosis 12 years prior. Due to the suspicion of an underlying disease, he was evaluated further. Chronic progressive external ophthalmoplegia in transition to the more severe syndromic variant Kearns-Sayre syndrome, a mitochondrial disorder causing myopathy, was diagnosed. The patient was treated with coenzyme Q10, and he underwent ptosis surgery on both eyes. This case illustrates a potentially multi-systemic disease that was diagnosed by a further evaluation of a common symptom, in this case worsening blepharoptosis. Awareness of myopathic symptoms is necessary to prevent overlooking serious yet improvable conditions.

Cavernous Hemangioma in the Middle Cranial Fossa & Cavernous Sinus

  • Park, Chang-Kyu;Lee, Mou-Seop;Kim, Young-Gyu;Kim, Dong-Ho
    • Journal of Korean Neurosurgical Society
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    • v.40 no.4
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    • pp.277-280
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    • 2006
  • Extracerebral cavernous hemangiomas are rare vascular tumors that are very difficult to remove because of severe intraoperative bleeding. We report a case of 57-year-old male with extracerebral cavernous hemangioma with review of 126 cases in the literature. Patient presented with blurred vision, diplopia, numbness on the left side of his face. Magnetic resonance imaging revealed a well defined mass of $3{\times}4{\times}3cm$ size with heterogenous iso-or hypointensity on T1-weighted image showing strong homogenous contrast enhancement and marked hyperintensity on T2-weighted image. Digital subtraction angiography[DSA] revealed a faint tumor blush by feeders from the left internal carotid artery[ICA] and left external carotid artery[ECA] in the delayed phase. Even with profuse intratumoral bleeding, near total removal was achieved. In addition to preoperative neurologic deficits such as ophthalmoplegia, facial numbness in the V1-2 dermatomes, ptosis appeared postoperatively.

Aneurysmal Bone Cyst of the Orbit : A Case Report with Literature Review

  • Yu, Jae-Won;Kim, Ki-Uk;Kim, Su-Jin;Choi, Sun-Seob
    • Journal of Korean Neurosurgical Society
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    • v.51 no.2
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    • pp.113-116
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    • 2012
  • Aneurysmal bone cyst (ABC) is benign vascular lesion destructing the cortical bone by the expansion of the vascular channel in the diploic space that usually involve long bone and spine. Orbital ABC is rare and the clinical symptoms deteriorate rapidly after initial slow-progression period for a few months. A 12-year-old female patient visited ophthalmologist due to proptosis and upward gaze limitation of the right eye, and orbital mass was noted in the upper part of right eye on orbital MRI. Five months later, exophthalmos was worsened rapidly with other features of ophthalmoplegia. Orbital mass was enlarged on MRI with intracranial extension. Surgery was done through frontal craniotomy and intracranial portion of the tumor was removed. Destructed orbital roof and mass in the orbit was also removed, and surrounding bone which was suspected to have lesion was resected as much as possible. Histopathological diagnosis was aneurysmal bone cyst. Postoperative course was satisfactory and the patient's eye symptoms improved. Authors report a rare case of orbital ABC with review of the literature. Exact diagnosis by imaging studies is important and it is recommended to perform surgical resection before rapid-progressing period and to resect the mass completely to prevent recurrence.

Carotid Cavernous Sinus Fistula with Abducens Nerve Palsy after Le Fort I Osteotomy : A Case Report (상악골절단술 후 외전신경마비를 동반한 경동맥 해면정맥동루)

  • Lee, Won-Hak;Kim, Dong-Ryul;Hong, Kwang-Jin;Lee, Jeong-Gu
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.22 no.2
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    • pp.243-248
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    • 2000
  • Carotid cavernous sinus fistula(CCSF) is an abnormal communication at the base of the skull between the internal carotid artery and the cavernous sinus. Fistula is almost associated with extensive facial trauma as a result of direct or indirect forces. Most fistulas of traumatic origin develop as a result of fractures through the base of the skull, which cause the laceration of the internal carotid artery near the cavernous sinus. The signs and symptoms of CCSF are pulsating exophthalmosis, orbital headache, pain, orbital or frontal bruit, loss of visual acuity, diplopia and ophthalmoplegia. Angiography reveals a definite CCSF and a detachable balloon embolization is known to be the treatment of choice. Even though carotid cavernous sinus fistula is an uncommon complication after orthognathic surgery, several cases of CCSF due to congenital anomalies, pre-existing aneurysms and abnormally thickened maxillary posterior wall have been reported in the literature. We have experienced a case of CCSF after Le Fort I osteotomy for maxillary advancement in skeletal class III patient and the cause, pathogenesis, diagnosis and treatment of this case.

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A Case of Unusual Tolosa-Hunt Syndrome (비전형 Tolosa-Hunt Syndrome 1예)

  • Lee, Sun-Sook;Kim, Kyung-Sook;Han, Young-Jin;Choe, Huhn
    • The Korean Journal of Pain
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    • v.2 no.2
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    • pp.198-202
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    • 1989
  • The Tolosa-Hunt syndrome is one of the rare disease with facial, especially peri-orbital pain, and ophthalomoplegia associated mostly with granulomatous lesions in cavernous sinus or superior orbital fissure. In addition to ophthalmoplegia by multiple cranial nerve involvement, the sympathetic nervous system may also be involved leading to Horner's syndrome. A typical Tolosa-Hunt syndrome has a neuro-radiologic finding of an increased density in the involved region, and a laboratory finding of an elevated ESR, as well as a dramatic response to systemic corticosteroid therapy. An unusual case of the Tolosa-Hunt syndrome with normal radiologic and laboratory findings, unresponsive to systemic corticosteroid, and some response of pain relief to a stellate ganglion block, is presented.

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A CASE REPORT OF RHINOCEREBRAL MUCORMYCOSIS ASSOCIATED WITH UNCONTROLLED DIABETES MELLITUS (당뇨병과 연관된 비대뇌형 모균증의 증례)

  • Choi, Sung-Weon;Yun, Dong-Hee;Kim, Jung-Su;Kang, Bo-Won;Kim, Sung-Moon;Lim, Jae-Suk;Kwon, Jong-Jin
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.16 no.2
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    • pp.152-156
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    • 1994
  • Rhinocerebral mucormycosis is an acute fulminant opportunistic fungal infection in debilitated patients with underlying pathologic conditions, which are diabetic ketoacidosis, immunosuppressed disease. We experienced a case of rhinocerebral mucormycosis. The patient was 41 year-old female, who had diabetes for five years and suffered from left facial swelling and pain, left ophthalmoplegia and facial skin necrosis. Total maxillectomy was done combined with systemic Amphotericin B therapy, Biopsy proved mucormycosis but she expired.

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Serial Electrophysiological Studies in Miller Fisher Syndrome (Miller Fisher 증후군1예에서 일련의 신경생리학적 소견)

  • Jun, Dong Chul;Park, Chun-Kang;Lee, Kyu-Yong;Lee, Young Joo;Kim, Juhan
    • Annals of Clinical Neurophysiology
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    • v.3 no.2
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    • pp.156-159
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    • 2001
  • Miller Fisher syndrome(MFS) has been the focus of conflicting opinions regarding the peripheral versus the central nature of the site of major neural injury. We present our electrophysiological findings in one case of MFS to help clarify the pattern of peripheral nerve injury in this syndrome. A 45-year-old man visited our hospital due to sudden diplopia. Initial examination revealed internuclear opthalmoplegia. The next day, his symptoms rapidly aggravated to complete external ophthalmoplegia, ataxia, and areflexia with hand and foot numbness. Serial electrophysiological studies were performed. The results of brainstem evoked potential(BAEP) and blink reflex were normal in the serial studies. Motor and sensory nerve conduction study(NCS) were normal findings in second hospital day, but ulnar sensory nerve shows no sensory nerve action potential(SNAP) and sural sensory conduction velocity was delayed in 7th hospital day. Our patient's clinical presentation began to improve on 15th hospital day, and his electrophysiologic study showed improvement on 29th hospital day. We believe that all the manifestations of MFS can be explained by the involvement of peripheral nerves without brainstem or cerebellar lesion with the serial electrophysiological studies.

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Primary orbital tuberculosis on the lower eyelid with cold abscess

  • Yoon, Hyun Sik;Na, Young Cheon;Lee, Hye Mi
    • Archives of Craniofacial Surgery
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    • v.20 no.4
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    • pp.274-278
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    • 2019
  • Orbital tuberculosis is a rare form of extrapulmonary tuberculosis, even in endemic areas. It may involve the soft tissue, lacrimal gland, periosteum, or bones of the orbital wall. We present a case of orbital tuberculosis on the lower eyelid. An 18-year-old woman with no underlying disease visited our clinic for evaluation of an oval nodule ($1.5{\times}1.2cm$) on the right lower eyelid. Incision and drainage without biopsy was performed 2 months ago in ophthalmology department, but the periorbital mass had deteriorated, as the patient had erythematous swelling, tenderness, and cervical lymphadenopathy. Visual acuity was normal; there were no signs of proptosis, diplopia, or ophthalmoplegia. Computed tomography revealed a small abscess cavity without bony involvement. We performed an excision and biopsy through a percutaneous incision under local anesthesia. Histological examination revealed a granuloma and was diagnosed as orbital tuberculosis. The patient was additionally treated with anti-tuberculosis therapy for 6 months and recovered without complication or recurrence by 7 months. Orbital tuberculosis occurs in patients with or without associated pulmonary tuberculosis, and should be considered as a differential diagnosis in patients with inflammatory orbital disease and an orbital mass. If recurrence occurs despite adequate initial treatment, we recommend an additional examination and excisional biopsy.

A Case Report of Oculomotor Nerve Palsy Treated with Acupuncture and Moxibustion (침구 치료로 호전된 동안신경마비 치험 1례)

  • Kim, Seo-Hee;Lee, Ju-Hyun;Kyung, Da-Hyun;Hong, Seok-Hoon
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.35 no.2
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    • pp.61-71
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    • 2022
  • Objectives : The aim of this study is to report the effect of acupuncture and moxibustion treatment for patient with oculomotor nerve palsy. Methods : We treated a patient who had suffered from right. ptosis, diplopia, ophthalmoplegia and diagnosed with oculomotor nerve palsy with acupuncture(including pharmacopuncture, electroacupuncture), moxibustion, cupping therapy. Photographs of eye movement were used to evaluate the changes in limitation of eye movement. We also evaluated symptoms such as diploma, ptosis. Results : After treatment, patient's symptoms such as limitation of eye movement, diplopia, ptosis were improved. There are no adverse effects and no relapse after treatments. Conclusions : This case report suggests that acupuncture and moxibustion treatment can be effective for patient with limitation of eye movement, diplopia, ptosis in oculomotor nerve palsy.