• Annals of Clinical Neurophysiology
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• v.13 no.1
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• pp.44-47
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• 2011

Background: Miller-Fisher syndrome (MFS) is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered a variant form of Guillain-Barre syndrome. Although some cases of delayed-onset facial palsy in MFS have been reported, the characteristics of this facial palsy are poorly described in the literature. Methods: Between 2007 and 2010, six patients with MFS were seen at our hospital. Delayed facial palsy, defined as a facial palsy that developed while the other symptoms of MFS began to improve following intravenous immunoglobulin treatment, was confirmed in four patients. The clinical and electrophysiological characteristics of delayed facial palsy in MFS, as observed in these patients, are described here. Results: Four patients with delayed-onset facial palsy were included. Delayed facial palsy developed 8-16 days after initial symptom onset (5-9 days after treatment). Unilateral facial palsy occurred in three patients and asymmetric facial diplegia in one patient. The House-Brackmann score of facial palsy was grade III in one patient, IV in two patients, and V in one patient. None of the patients complained of posterior auricular pain. Facial nerve conduction studies revealed normal amplitude in all four patients. The blink reflex showed abnormal prolongation in two patients and the absence of action potential formation in two patients. Facial palsy resolved completely in all four patients within 3 months. Conclusions: Delayed facial palsy is a frequent symptom in MFS and resolves completely without additional treatment. Thus, standard treatment and patient reassurance are sufficient in most cases.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.33 no.5
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• pp.449-453
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• 2011

Orbital infection is typically caused by spread of inflammation from the paranasal sinuses. Less common causes can be skin infections, trauma, and hematogenous spread from other infections located elsewhere in the body. Odontogenic orbital infections account for 2~5% of all orbital infections, and occur as a result of periodontitis, odontogenic abscess from caries, tooth extraction, and oral surgery. Orbital infections can be divided into preseptal infection, orbital subperiosteal abscess, orbital abscess, and postorbital abscess. Symptoms which can be observed are swelling of the eyelids and erythema, orbital edema, displacement of the eyeball, exophthalmos, ophthalmoplegia, and even impairment of the optic nerve. Here we present the case of a patient who had an orbital abscess secondary to an abscess of the right maxillary third molar. Rapid recovery occurred following surgical treatment and antibiotic therapy. In addition a brief review of the literature is included.

• Radiation Oncology Journal
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• v.17 no.4
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• pp.293-298
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• 1999

Intracranial involvement by adenoid cystic carcinoma (ACC) is very rare and there is no report of intracranial extension from the palate ACC in Korea. Intracranial involvement can occur in one of three ways: direct extension, perineural spread, and hematogenous spread. A case report of a 35-year-old woman with intracranial ACC is presented. Initially she had ACC of the right palate and was treated by surgery and postoperative radiation therapy. Three years and 10 months later, the paresthesia in the distribution of ophthalmic and maxillary branch of right trigeminal none developed without evidence of recurrence in CT scan. Ptosis and total ophthalmoplegia developed sequentially and the second operation was peformed. It was suggested that the tumor was spread perineurally along the trigeminal news into the Gasserian ganglion and then cavernous sinus and orbit. Seven years and 6 months after the first operation, direct intracranial extension into the right temporal lobe developed via sphenoid bone, sphenoid sinus and temporal bone and the third operation was done. And then lung metastasis was diagnosed. She is alive for 9 years 5 months after first operation.

• Journal of Acupuncture Research
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• v.29 no.3
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• pp.139-148
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• 2012

Objectives : The purpose of this case is to report the improvement of two patients diagnosed with eight and a half syndrome after combination therapy of oriental and western medicine. Methods : We treated the patients with combination therapy of oriental and western medicine such as acupuncture, moxibustion, herbal medication, antithrombotic agents and steroid therapy. Changes of peripheral facial paralysis were evaluated using House-Brackmann facial grading system and the degree of dizziness, tenderness and pain of upper abdomen were assessed using numerous rating scale. Changes in motor grade of upper and lower extremities were evaluated using medical research council scale. Results : We have recently experienced two cases of eight and a half syndrome - a syndrome characterized by the coexistence of one and a half syndrome, a rare ophthalmoparetic syndrome characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other, and cranial nerve VII palsy. The one and a half syndrome was caused by acute cerebral infarction. Two patients in this report were improved through combination therapy of oriental and western medicine. Conclusions : We report the clinical course and treatment methods of eight and a half syndrome.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.95-98
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• 2018

A striking feature of mitochondrial disorders is the vast heterogeneity in their clinical symptoms that ranges from a single organ to severe multisystem involvement. Though a variety of ocular symptoms such as ptosis, pigmentary retinal degeneration, external ophthalmoplegia, and optic nerve atrophy can occur in association with mitochondrial cytopathies, progressive bilateral cataracts are rare among their ocular findings. A 5-year-old girl with no previous medical history came to our hospital presenting symptoms of seizure. She started showing progressive developmental regression, increased seizure frequency, hypotonia, general weakness, dysphagia and decreased vision. Lactic acidosis was noted in metabolic screening test and we confirmed mitochondrial respiratory chain complex I defect in spectrophotometric enzyme assay using the muscle tissue. Progressive bilateral cataracts then developed and were fully evident at the age of 7. She underwent cataract extraction with posterior chamber lens implantation. We are reporting a case of mitochondrial respiratory chain defect with multiorgan involvements including bilateral progressive cataract, an uncommon ocular manifestation. Ophthalmologic evaluation is highly recommended not to overlook the possible ocular manifestations in mitochondrial disorders.

• The Journal of the Society of Stroke on Korean Medicine
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• v.16 no.1
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• pp.81-88
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• 2015

■ Objectives The purpose of this clinical study is to evaluate the effect of Traditional Korean Medicine(KM) on a patient with Tolosa-Hunt syndrome. ■ Methods We treated a 58 years old male patien t with Tolosa-Hunt syndrome who had several clinical symptoms such as ocular pain, limitaion of ocular movement and unilateral ptosis. We used herbal medication, acupuncture, electro-acupuncture(EA), cupping, moxibustion, bee-venom injection. Then we evaluated the improvement by Numerical Rating scale(NRS), Distance of eye opening and ratio of pupil movement. ■ Results We demonstrated the decrease of pain score and improvement of equal ocular movement(EOM) and ptosis after the KM treatment. ■ Conclusion This study proved the effect of KM treatment on Tolosa-Hunt syndrome. It could cooperative treatment would be more effective than mono treatment of western medicine.

• Archives of Craniofacial Surgery
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• v.25 no.1
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• pp.27-30
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• 2024

Fungal sinusitis is relatively rare, but it has become more common in recent years. When fungal sinusitis invades the orbit, it can cause proptosis, chemosis, ophthalmoplegia, retroorbital pain, and vision impairment. We present a case of an extensive orbital floor defect due to invasive fungal sinusitis. A 62-year-old man with hypertension and a history of lung adenocarcinoma, presented with right-side facial pain and swelling. On admission, the serum glucose level was 347 mg/dL, and hemoglobin A1c was 11.4%. A computed tomography scan and a Waters' view X-ray showed right maxillary sinusitis with an orbital floor defect. On hospital day 3, functional endoscopic sinus surgery was performed by the otorhinolaryngology team, and an aspergilloma in necrotic inflammatory exudate obtained during exploration. On hospital day 7, orbital floor reconstruction with a Medpor Titan surgical implant was done. In principle, the management of invasive sino-orbital fungal infection often begins with surgical debridement and local irrigation with an antifungal agent. Exceptionally, in this case, debridement and immediate orbital floor reconstruction were performed to prevent enophthalmos caused by the extensive orbital floor defect. The patient underwent orbital floor reconstruction and received intravenous and oral voriconazole. Despite orbital invasion, there were no ophthalmic symptoms or sequelae.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.17 no.4
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• pp.447-455
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• 1995

Cavernous sinus thrombosis is one of the major complications of abscesses of the maxillofacial region. The initial symptoms of CST are usually pain in the eye and tenderness to pressure. this is associated with high fluctuating fever, chills, rapid pulse, and sweating. Venous obstruction subsequently causes edema of the eyelids, lacrimation, proptosis, chemosis and retinal hemorrhages. Blindness is sometimes an accompaniment of cavernous sinus thrombosis when the infection also involves the orbit. There is also cranial nerve involvement (oculomotor, troclear, abducence) and ophthalmoplegia, diminished or absent corneal reflex, ptosis, and dilation of the pupil occur. The terminal stages bring signs of advanced toxemia and meningitis. Infections of the face can cause a septic thrombosis of the cavernous sinus. Furunculosis and infected hair follicles in the nose are frequent causes. Extractions of maxillary anterior teeth in the presence of acute infection and especially curettage of the sockets under such circumstances can cause this condition. The infection is usually staphylococcal. The inflection may spread directly through the pterygoid plexus of veins and the pterygomaxillary space and then ascend into the sinus or it may spread directly from the pterygopalatine space to the orbit. This is possible because of the absence of valves in the angular, facial, and ophthalmic veins. The treatment is empirical antibiotic therapy followed by specific anbibiotic therapy based on blood or pus culture. The inflection usually involves one side, however, it may easily spread to the opposite side through the circulus sinus. Unless it is treated early, the prognosis is poor even in this doses. Occasionally the antibiotics will not adequately resolve the septic thrombus, and death ensues. the use of anticoagulants to prevent venous thrombosis has been recommended, but the efficacy of such therapy has not been substantiated. Surgical access through eye enucleation has been suggested. We report a case which demonstrates cavernous sinus thrombosis by the infection after the functional neck dissection and the intraoral reconstruction with auriculomastoid fascio-cutaneous island flap.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.30-34
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• 2018

Niemann Pick type C disease (NPC) is an inherited progressive neurodegenerative disorder, due to defects of intracellular lipid trafficking and storage. Hepatosplenomegaly may prevail, while progressive neurodegenerative symptoms such as cerebellar involvement, dystonia, vertical supranuclear ophthalmoplegia, cataplexy, and eventually seizures starting at juvenile or late infantile period may accompany after normal early development. Here we describe a 3-year-old Korean boy with NPC who presented with splenomegaly at age 3. Liver biopsy showed characteristic foamy cell stained by periodic acid-schiff, and molecular analysis for NPC1 identified the compound heterozygous mutations, novel mutation of c.1631G>A (p.Trp544Ter) and c.2662C>T (p. Pro888Ser) as a known mutation. Filipin was strongly stained with unesterified cellular cholesterol in the patient's skin fibroblasts. The patient has received migulstat since age 3 years and his long-term outcome is needed to be observed.

• The Journal of Korean Medicine
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• v.17 no.1 s.31
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• pp.190-211
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• 1996

Generally the Myasthenia Gravis is classified into two of hereditary factor and acquement. Aquired Myasthenia Gravis was Quite well known to be caused by the autoimmune mechanism. Not in accurateness, on the hereditary, acetylcholine receptor antibody was to be analyzed very high in their parents and brothers. Also Myasthenia Gravis is a chronic disease characterized by voluntary muscle weakness and fatigue. above all, ocular Myasthenia Gravis is characterized clinically by blepharoptosis and external ophthalmoplegia and to be showed abut 90% cases and so oriental medicine can not but deal with myasthenia gravis at blepharoptosis. Accordinglv 20 out patients with Myasthenia Gravis were clinical study and observation as to the sex, age, progress state of MG, blood type, history, main symptom, liking for warm and cool food and tepidity, state of pulse, treatment of acupuncture and administration of oriental medicine etc. The results were as follows. 1. There was investigaed on the frequency of attack for sex, age, oculus dexter, oculus sinister, oculus uterque. Among the 20 patients, the number of female were 60% with 12 cases and male were 40% with 8 cases, therefore it was the rate of 6 : 4. the patients under 10 ages and 40 ages were 20% with 4 cases, 10 ages and 50 ages were 15% with 3 cases, 20, 30, 60 ages were 10% with 2 cases. And then oculus uterque was 90% with 18 cases, oculus sinister was 10% with 2 cases and oculus dexter were none of them. 2. Stage I were 50% with 10 patients, stage $II_A$ were 30% with 6 patients and stage $II_B$ were 20% with 4 patients, on the clinical stage and too class I were 20% with 4, class II were 45% with 9, class III were 35% with 7, in the functional activity the patients with chest heavy were 15% with 3 and hyperthyroidism were 10% with 2. 3. Hospital which patients had used to before came to this hospital were 10 university hospital and 6 local clinic. 4. The duration of disease was from 3 months to 30 years, the patients suffering between 3 months and within 1 year were 25% with 5 cases, 1-2 years were 30% with 6 cases, therefore within 2 years were 55%. 4-5 years were 15%, over 7 years were appeared less than 10%. 5. In the main symptom, all of patients were appeared to be heavy in opening their eyes the patients with blephroptosis were 70% with 14 cases on the oculus uterque, oculus sinisterf and oculus dexter, there were 20% with 4 eases each other in the oculogyation incomplete. visual failing, ophthalmoxerosis, strabismus etc and indigetion, frequency of urine(feel hurt), mild stools(or diarrea), oversensitiveness etc. but in addition, all of the other were 10%. 6. In the distribution of blood type, 0 types were 45% with 9 cases, A types were 25% with 5 cases, B and AB types were 15% each other. 7. For the rates of patients of liking for warm and cool food or tepidity, patients of liking for warm food possess 45% with 9 cases, and cool food possess 35% with 7 and tepidity possess 20% with 4, and then most of patients liking for warm food were females and cool food were much more males than females. 8. Hyunsae(弦細) were 40% with 8 cases, Buhurl(浮滑) were 20% with 4 cases, Hyunsak(弦數) were 15% with 3 cases, and in addition, the others were 10%, among 7 types of pulses. 9. The patients with less than 1 week were 40% with 8 cases, and there were female most of them and over 4 weeks were 20% and 1-2weeks were 15%, in the duration acupuncture treatment. 10. 15 kinds of prescriptions were administrated with oriental medicine from 1 week to 20weeks 1_2 weeks were 25.71% with 9 cases, 3 weeks were 17.14% with 6 cases and 6 weeks were 11.42% with 4 cases and also Gamibaetaugunbitang(加味培土健脾湯) were 28.57% with 10 cases, Gamijeounyongtang(加味正容湯) were 14.28% with 5 cases, Gamibojoongyigitung (加味補中益氣湯), Gamiyinsamyangyoungtung (加味人蔘養榮湯) were 8.57% with 3 cases each other and also Gamisamgitung (加味蔘?湯), Gamisamuloajatung(加味四物五子湯) Gamigoudungum (加味鉤藤飮), etc were applied.