Clinical Features of Adie's Tonic Pupil: A Retrospective and Cross-Sectional Study

PURPOSE: To report the clinical features of Adie's tonic pupil. METHODS: The medical records of 22 patients who had been diagnosed with Adie's tonic pupil from February 1998 to February 2009, were retrospectively reviewed. On March 2010, a cross-sectional examination was performed in 16 patients (19 eyes) who underwent a follow-up of more than 1 year. Measurements included pupil size in room light, bright light and in darkness; near point of accommodation; presence of segmental iris palsy; light-near dissociation; denervation supersensitivity; corneal sensitivity; and deep tendon reflex (DTR). RESULTS: Among the patients studied, 16 were women in Adie's tonic pupils. Only 3 of patients had bilateral involvement. The mean age of onset was 38.3 years. The mean size of Adie's tonic pupils was 2.3 mm larger than the fellow eyes. Segmental iris palsy was detected in 93.8% of the patients. Denervation supersensitivity was observed in all patients. Light-near dissociation was present in 88.2% and over 90% of the patients had decreased DTR in the biceps, triceps, knee and ankle jerk. CONCLUSIONS: This cross-sectional study showed Adie's tonic pupil tended to become miotic and recover accommodation power over the years.

Vigabatrin and Visual Field Defects in Pediatric Epilepsy Patients

We studied the prevalence, type and severity of vigabatrin (VGB)-attributed visual field defects (VFDs), and used these data to assess the associated risk factors in pediatric patients. Medical records were retrospectively reviewed for 67 pediatric patients who received VGB alone or in combination with other antiepileptic drugs, and who had undergone visual field examinations using a Humphrey visual field analyzer. Of the 67 patients, 15 had VGB-attributed VFDs: 13 had nasal arcuate type, 1 had nasal and temporal constricted type and 1 had nasal constricted type. In terms of severity, 7 patients had Grade I VGB-attributed VFDs, 5 had Grade II, 2 had Grade III, and 1 had Grade IV. Although there were no significant differences between the VFD and non-VFD groups with regards to all tested parameters, there were no cases of VGB-attributed VFDs in patients with total treatment durations <2 yr and cumulative doses <10 g/kg. In conclusion, the prevalence of VGB-attributed VFDs in VGB-treated pediatric epilepsy patients was 22%. The high frequency of VGB-attributed VFDs indicates that physicians should inform all patients of this risk prior to VGB treatment and perform periodic visual field examinations.

Central Retinal Artery Occlusion and Orbital Abscess following Dental Abscess

PURPOSE: Oral cavity or other head and neck inflammation may invade the orbit because paranasal sinuses are close to the orbital wall. The authors report a case of orbital abscess and central retinal artery occlusion followed by orbital invasion of the abscess after tooth extraction. METHODS: A 61-year old woman complained of persistent dental abscess and sudden visual loss for 2 weeks following tooth extraction. We performed a brain MRI (Magnetic Resonance Imaging) and an orbit CT (Computed Tomography) that revealed orbital cellulitis and orbital abscess. On fundus exam, cherry-red spot and retinal edema were noticed. RESULTS: Although ocular symptoms were improved with intravenous antibiotic therapy and surgical intervention, there was no recovery of visual acuity. CONCLUSIONS: Visual loss can be associated with delayed treatment in the case of orbital abscess. Therefore early diagnosis and proper treatment of orbital invasion following dental abscess is crucial for the recovery of vision.

2 Cases of Traumatic Inferior Oblique Palsy

PURPOSE: Inferior oblique palsy is the least commonly isolated extraocular muscle palsy. We describe the clinical features and managements of 2 cases of traumatic inferior oblique palsy. METHODS: Two adult patients were presented with vertical diplopia and head tilt posture after head trauma. The subjects fulfilled the three-step test criteria, with a hypertropia that worsened on side gaze and head tilt away from the affected eye. They showed free forced duction to elevation in adduction. Both were treated by ipsilateral superior oblique tenotomy and contralateral superior rectus recession with adjustable suture technique. RESULTS: During postoperative 7 month observation, both patients demonstrated orthophoria in primary gaze. Our surgical procedures eliminated the diplopia and abnormal head tilt posture, thereby achieving satisfactory results.

Leber's Hereditary Optic Neuropathy with Dystonia Caused by mtDNA 11778 Mutation

Several mtDNA mutations have been reported in Leber's hereditary optic neuropathy (LHON) associated with dystonia since it was identified as having the 14459 mutation. We report a patient with LHON and dystonia and his family. The patient presented with a slowly progressive bilateral visual loss and generalized dystonia. Brain MRI showed abnormal signal changes in both putamina. The 11778 mutation was confirmed by a Sfa I restriction digestion test. We found, in the literature, only one case of the 11778 mutation associated with dystonia, although it is one of the most common mutations in LHON. Our case suggests that the 11778 mutation should be taken into consideration in the pathogenesis of LHON associated with dystonia.

MR Imaging of the Orbital Apex: Anatomy and Pathology

The apex of the orbit is basically formed by the optic canal, the superior orbital fissure, and their contents. Space-occupying lesions in this area can result in clinical deficits caused by compression of the optic nerve or extraocular muscles. Evenvascular changes in the cavernous sinus can produce a direct mass effect and affect the orbitapex. When pathologic changes in this region is suspected, contrast-enhanced MR imaging with fat saturation is very useful. According to the anatomic regions from which the lesions arise, they can be classified as belonging to one of five groups; lesions of the optic nerve-sheath complex, of the conal and intraconal spaces, of the extraconal space and bony orbit, of the cavernous sinus or diffuse. The characteristic MR findings of various orbital lesions will be described in this paper.

Miller Fisher Syndrome

Miller Fisher syndrome is characterized by acute external ophthalmoplegia, ataxia and areflexia in the abscence of significant motor or sensory deficit in the limbs and usually results in a complete recovery. Most cases have anteceding events like upper respiratory infection or other viral infections. Diagnosis of Miller fisher syndrome can be made with compatible clinical history taking, cardinal symptoms and normal findings of CT or MRI. The prognosis of Miller Fisher syndrome is favorable contrary to other malignant tumors or Guillain-Barre syndrome which sometimes cause a respiratory paralysis. The locations of its anatomic lesion and pathogenesis are nor yet discovered. From 1991 to 1995, we have experienced 11 cases of Miller Fisher syndrome. Thereby we have analyzed anteceding events, cardinal symptoms along with other associated symptoms, electrophysiological studies, cerebrospinal fluid test and its treatment and prognosis. The purpose of this article is that a better understanding of Miller Fisher syndrome can lead us to differenciate with other disorders causing external ophthalmoplegia.

Clinical Profile of the Optic Neuritis in Korea

We performed a retrospective review on 32 patients(37 eyes) with the diagnosis of optic neuritis with follow-up more than 3 months, to determine its clinical characteristics in Korea. They consisted of 18 women and 14 men with a median age of 35.9 years. The initial visual acuity on presentation was variable from 1.2 to NLP, 84% of patients had at least 0.5 or better final visual acuity. Sixty-nine percent of the patients showed swollen disc and the remaining appeared normal in the affected eyes. Visual field examination revealed central or ceco-central scotoma in 17 eyes(48%) and other variable visual field defects. Visual evoked potential was abnormal in 89% of the examined eyes. Magnetic resonance imaging showed optic nerve enhancement in 48% and characteristic findings consistent with multiple sclerosis was seen in 6% of the examined subjects. This result suggests that the optic neuritis in Korea present with more papillitis than retrobulbar optic neuritis and multiple sclerosis is less commonly seen compared with previous reports of Caucasian optic neuritis.

Treatment Effects of MIN GLASSES for Amblyopia and Diplopia

MIN GLASSES is specially manufactured in order to enhance satisfaction of wearer and therefore increase the effectiveness of treatment of an amblyopia and a diplopia. One of the lens is specially grounded and coated to blur vision. Cosmetically, the eye appears to wear regular glasses through the blur lens. We prospectively studied 58 amblyopic children and 10 diplopic adults. Each patient was treated with MIN GLASSES for cosmetic problems. We checked linear visual acuity of pre- and post-treatment. Questionnaires were given to the patients and parents regarding cosmetic satisfaction with treatment. In amblyopic children. 44 patients(75.9%) showed visual acuity improvement of 1.61 +/- 1.50 lines by Han's visual acuity chart. Patient compliance was 87.9%(51 patients) with patient satisfaction based on a questionnaire response. Parents were 87.9%(51 parents) positive response. In diplopic adults, 10 patients(100%) were positive response with satisfaction and demonstrated to be free of symptom of diplopia. MIN GLASSES is effective in high compliance satisfaction of amblyopia and diplopia, and improvement of visual acuity of amblyopia.

A Case of Septo-Optic Dysplasia-Schizencephaly Complex

Septo-optic dysplasia is diagnosed when optic nerve hypoplasia is combined with dysgenesis of the septum pellucidum and nearly two-thirds of them have hypothalamic-pituitary dysfunction. A number of these patients have schizencephaly and usually present seizures. The migrational disorders including schizencephaly, lissencephaly, heterotopia and polymicrogyria are a rare group of congenital malformations of the brain Septo-optic dysplasia-schizencephaly complex is frequently associated with endocrinolo gic, ophthalmologic, and neurologic symptoms and signs. We recently experienced a case of septo-optic dysplasia-schizencephaly, who showed severe visual impairment associated nystagmus and bilateral optic nerve hypoplasia agenesis of septum pellucidum with schizencephaly and hypsarrythmia. Because some forms of migrational disorders and septo-optic dysplasia can be inherited, parental counseling is essential for the accurate diagnosis.

A Case of Palpebral Sarcoidosis Associated with Granulomatous Uveitis

Sarcoidosis is a chronic granulomatous disease of unknown cause characterized by widespread occurence of epithelioic cell. The authors experienced a case of sarcoidosis in 34-year-old female who has lacrimal gland enlargement, severe subcutaneous nodules and bilateral granulomatous uveitis. Histopathologic examination of the subcutaneous nodule of lid revealed sarcoidosis. In the course of corticosteroid therapy, subcutaneous nodules and enlarged lacrimal gland subsided gradually.