Acute Leukemia Relapse Presenting as Recurrent Involvement of the Optic Nerve

PURPOSE: To report two cases with recurrent involvement of the optic nerve as the initial sign of acute leukemic relapse.CASE SUMMARY: An 8-year-old male with acute lymphoblastic leukemia on the maintenance chemotherapy was referred for a decrease in visual acuity in the right eye. The visual acuity and optic disc swelling were completely resolved with high-dose steroid therapy. Two months after the initial presentation, the symptoms recurred and brain/orbit magnetic resonance imaging (MRI) showed high intensity along the right optic nerve from the retrobulbar area to the optic chiasm. The visual acuity was restored after high-dose steroid therapy. One month after the second attack, the symptoms recurred and the cerebrospinal fluid cytology was positive for lymphoblasts. Three weeks after the intrathecal chemotherapy, the visual acuity improved fully, but optic disc atrophy developed. A 45-year-old male, who received allogenic peripheral blood stem cell transplantation for acute myeloid leukemia, presented with a decrease in visual acuity in the left eye. The left optic disc swelling improved with high-dose steroid therapy, but the medication was restarted due to the recurrence of symptoms 3 weeks later. Brain MRI showed a mass lesion compressing the left optic nerve, presumed to be a myeloid sarcoma. One month after local irradiation, the visual acuity was no light perception in the left eye.CONCLUSIONS: In patients with a prior history of acute leukemia, the recurrent involvement of the optic nerve should be considered as a central nerve system relapse, regardless of improvement with steroid treatment.

Clinical Features of Children with +4.00 Diopters or More Hyperopia Weaning with Age

PURPOSE: To evaluate the clinical features of children with high hyperopia weaning with age. METHODS: The medical records of 203 children wearing spectacles due to hyperopia of +4.00 diopters (D) or greater in at least one eye based on the cycloplegic refraction and with follow-up for 3 years or more were reviewed. The patients were divided into those who showed a decrease in the spherical equivalent (SE) of 1.50 D or greater and those who maintained. The age of wearing spectacles, the magnitude of hyperopia, the angle of deviation, the ratio of accommodative-convergence to accommodation (AC/A), and the frequency of amblyopia and anisometropia were compared. RESULTS: Forty seven patients with decreased hyperopia and 156 patients with sustained hyperopia were included. The decreased-group started to wear spectacles later than the sustained-group (5.0 ± 2.3 years vs. 4.1 ± 2.4 years). The mean SE of the hyperopic eye in the decreased-group was significantly greater at the initial visit than in the sustained-group (6.29 ± 2.18 D vs. 5.47 ± 1.38 D); was identical at the 1 year follow-up (4.83 ± 1.72 D vs. 4.89 ± 1.55 D); and significantly lower at the last follow-up (3.15 ± 1.72 D vs. 4.65 ± 1.56 D). In the decreased-group, the mean hyperopia of 3.14 ± 2.02 D decreased during a mean period of 3.9 years, especially during the first year after spectacle correction. At baseline, the frequency and angle of esotropia at both distant and near with/without hyperopic correction was significantly larger in the sustained-group. The frequency of amblyopia and anisometropia and the AC/A were identical between the two groups, while the frequency of amblyopia at the last follow-up was significantly lower in the decreased-group. CONCLUSIONS: Some patients with hyperopia of +4.00 D or greater who had none or a small angle of esotropia and improved amblyopia showed a decrease in hyperopia with age.

Spontaneous Repetitive Constriction and Dilatation of a Unilateral Pupil in a Brain Death Patient

In brain death state, bilateral pupil light reflexes are disappeared, and pupils are fixed with dilated. However, spontaneous movements such as ocular microtremor or bilateral cyclical constriction-dilatation of pupils have been rarely reported in brain death patients. We present a brain death patient whose right pupil displayed spontaneously repetitive constriction and dilatation regardless of external stimuli such as light and pain. Early recognition of this phenomenon may prohibit the delay in the diagnosis of brain death and organ transplantation.

Choroidal Thickness Indicates Subclinical Ocular and Systemic Inflammation in Eyes with Behçet Disease without Active Inflammation

PURPOSE: To investigate whether subfoveal choroidal thickness, measured using enhanced depth imaging optical coherence tomography (EDI-OCT), is an indicator of subclinical ocular or systemic inflammation in eyes with Behçet disease (BD) without active ocular inflammation. METHODS: A retrospective analysis was used to examine clinical features of non-uveitic patients with BD (NUBD group), patients with a previous history of Behçet uveitis in an inactive state (IUBD group), and healthy controls were evaluated from October 2014 to September 2015. Subfoveal choroidal thickness was measured using EDI-OCT. RESULTS: The NUBD group included 46 eyes in 24 patients; the IUBD group included 16 eyes in 11 patients; and the control group included 35 eyes in 23 individuals. The mean subfoveal choroidal thicknesses differed significantly among these groups. Choroidal thickness was significantly greater in the NUBD (310.5 ± 81.0 µm) than in the IUBD (263.1 ± 56.6 µm, p = 0.013) and control (256.9 ± 67.9 µm, p = 0.002) groups. The disease activity score was significantly higher in the NUBD than in the IUBD group (p < 0.001), while the use of cyclosporine was significantly associated with choroidal thickness in eyes with NUBD (p = 0.039). CONCLUSIONS: Subfoveal choroidal thickness, as measured by EDI-OCT, may be a clinical indicator of subclinical ocular inflammation and systemic inflammation in BD patients without active ocular inflammation.

Lumbar Stenosis: A Recent Update by Review of Literature

Degeneration of the intervertebral disc results in initial relative instability, hypermobility, and hypertrophy of the facet joints, particularly at the superior articular process. This finally leads to a reduction of the spinal canal dimensions and compression of the neural elements, which can result in neurogenic intermittent claudication caused by venous congestion and arterial hypertension around nerve roots. Most patients with symptomatic lumbar stenosis had neurogenic intermittent claudication with the risk of a fall. However, although the physical findings and clinical symptoms in lumbar stenosis are not acute, the radiographic findings are comparatively severe. Magnetic resonance imaging is a noninvasive and good method for evaluation of lumbar stenosis. Though there are very few studies pertaining to the natural progression of lumbar spinal stenosis, symptoms of spinal stenosis usually respond favorably to non-operative management. In patients who fail to respond to non-operative management, surgical treatments such as decompression or decompression with spinal fusion are required. Restoration of a normal pelvic tilt after lumbar fusion correlates to a good clinical outcome.

A Case of Inflammatory Fibroid Polyp in the Duodenum / 대한소화기내시경학회지

Inflammatory fibroid polyp is a relatively uncommon, localized benign lesion of the gastrointestinal wall. It is seen mostly as a single entity and the majority of inflammatory fibroid polyps have been reported with pathologic examination in the stomach but rarely in small intestines. A 70-year-old woman was presented with intermittent epigatric discomfort and gastroduodenoscopy showed a polypoid lesion with oval shaped, central depression, and normal mucosal covering. Endoscopic ultrasonography showed 8x6 mm sized hypoechoic tumor which was located mainly in the third layer of the duodenal wall with indistinct margin. The tumor was successfully removed by endoscopic resection after the injection of hypertonic saline solution with epinephrine and banding. The tumor consisted of loose fibrous stroma, small blood vessels and inflammatory cells. We present a case of inflammatory fibroid polyp of the duodenum with review of the literatures.

The Comparison of Sensitivities of Electrophysiological Parameters in Carpal Tunnel Syndrome

BACKGROUND: Carpal tunnel syndrome (CTS) is the most common mononeuropathy encountered in clinical practice. When performing routine diagnostic nerve conduction studies (NCS) in patients with CTS, we sometimes happen to be confronted with patients who have no definite electrophysiological evidence of CTS. We therefore evaluated the diag-nostic sensitivities of several parameters in NCS for CTS patients. METHODS: We analyzed 187 patients (262 hands) who were diagnosed with CTS clinically and electrophysiologically for the past 4 years in this hospital. RESULTS: Significant abnormal findings of nerve conduction velocity (NCV) were noted in the palm to wrist (P-W) seg-ment( 98.5%), thumb-wrist (I-W) segment (97.3%), distoproximal ratio in the third finger (95.0%), and the terminal latency (71.4%) of the median nerve. There were no statistical differences in the abnormal NCVs between the male and female groups. The sensitivity of each compartment parameters were obtained in the female groups and statistical dif-ferences were found in the sensory NCV of the P-W segment and compound nerve action potential (CNAP) amplitude of the I-W segments in patients between the ages of 40 and 60. The sensory NCV of the P-W segment was especially more significant in this age group. There were statistical differences in the amplitude of the index finger to wrist (F-W) and P-W segments between the denervated electromyography group and normal group (p < 0.05). CONCLUSIONS: In clini-cally suspected CTS, the most sensitive tests were the sensory NCV of the P-W segment, I-W segment, and distoproxi-mal ratio in the third finger. Therefore, the sensory NCV of the P-W segment should be performed first. If the results of this test are unremarkable, then, the next tests should include the sensory NCV of the I-W segment and the distoproxi-mal ratio should be performed before ruling out the diagnosis of CTS.

Diagnostic Sensitivity of Several Muscles in Repetitive Nerve Stimulation Test for Myasthenia Gravis / 영남의대학술지

BACKGROUND: This study was undertaken to evaluate the diagnostic sensitivity of several muscles in repetitive nerve stimulation test (RNST) for myasthenia gravis (MG) patients. MATERIALS AND METHODS: The study population consisted of 39 MG patients classified by modified Ossermann's classification. Using Stalberg's method, RNST was systematically performed in facial (orbicularis oculi and nasalis) and upper extremity (flexor carpi ulnaris, abductor digiti quinti and anconeus) muscles. RESULTS: The significant electrodecremental response of RNST were noted in orbicularis oculi (58.9%), nasalis (51.3%), flexor carpi ulnaris (42%), anconeus (41%) and abductor digiti quinti muscles (27%). Among the 3 muscles of upper extremity (abductor digiti quinti, flexor carpi ulnaris and anconeus), the positive electrodecremental response of anconeus muscles was significantly higher than other two muscles (p<0.05) in type IIa, IIb and there were no statistical differences of the positive electrodecremental response between orbicularis oculi and nasalis muscles. The facial muscles showed more prominent decremental responses than upper extremity muscles in type I MG(p<0.05). In type IIa MG patients, there were no significant statistical differences between facial and upper extremity muscles but significant statistical differences among upper extremity muscles. In type IIb MG patients, there were no significant statistical differences in all tested muscles in spite of the increased positive electrodecremental response of RNST. CONCLUSION: On the basis of this study, RNST would be initially performed for the orbicularis or nasalis in type I MG and for the anconeus in type IIa or IIb MG.

A CLINICAL STUDY ON THE MIDFACE FRACTURE

With the increase of industrialization, leisures, sports activities and traffic, the risk of trauma has increased markedly. Midfacial bones are relatively common sites of trauma, and are difficult sites of correct reduction and reconstruction, because midface is composed of mostly thin cortical bone surrounding cavities filled with either air or fat with discrete areas of bony condensation. A clinical study on 208 patients with midfacial bone fracture who visited Sanggye Paik Hospital during 5 years(1990~1995) was done by analyzing sex, age, cause, fracture site, treatment method, complication and involvement of other body part, etc. The results obtained were as follows : 1. The occurrence was more frequent in male than in female with ratio of 2.7 : 1 and most frequently in twenties. 2. Traffic accident was the most common cause of midface fractures. 3. Zygoma was the most frequently occurred site(50%) of midface fractures. 4. Simple fracture(75%) was more frequently occurred than compound fracture(25%). 5. As treatment method, open reduction(76.4%) was used more frequently than closed reduction(23.6%) 6. Generally, operations are done in 5 days after admission(59.1%), and intermaxillary fixation was done in 78.4% of cases. 7. Teeth and alveolar bone damages were occurred in 32.2% of cases. 8. Other injuries that were related to midface fracture occurred in 63.3% of cases. 9. Post-operative complications occurred in 9% of cases.

A Case of Leber's Hereditary Optic Nouropathy Showing 11778 Point Mutation of Mitochondrial DNA / 영남의대학술지

Leber's hereditary optic neuropathy(LHON) is an optic nerve disease that causes blindness and is associated with maternally inherited mitochondrial DNA(mt DNA) mutations. The most common mitochondrial DNA mutation among LHON patients is a point mutation at the nucleotide 11778 in the subunit 4 of complex I. In one 45-year old male LHON patient with bilateral optic neuropathy, we investigated the presence of a point mutation of mitochondrial DNA and identified a single guanine to adenine transition mutation in the mitochondrial DNA at nucleotide point 11778.

A case of primary antiphospholipid antibody syndrome showing vegetation on the mitral valve through echocardiography / 영남의대학술지

Antiphospholipid antibody syndrome(APS) is a well-known clinical syndrome characterized by recurrent arterial or venous thromboses, recurrent fetal loss, thrombocytopenia, together with high titers of sustained anticardiolipin antibody(aCL) or lupus anticoagulant(LA). Although systemic lupus erythematosus(SLE) and APS may coexist, a high proportion of patients manifesting the APS do not suffer from classical lupus or other connective tissue disease. The patient has been defined as having a primary antiphospholipid antibody syndrome. We experienced one case of primary APS with recurrent fetal loss, recurrent cerebral infarctions, positive anticardiolipin antibody IgG and fluttering vegetation on the mitral valve, without other connective tissue diseases including SLE. Forty-three old female had 2 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia and positive antinuclear antibody, but did not meet whole criteria. The patient was treated with ticlopidine, and anticoagulant therapy was recommended.

Comparison of Usefulness of Laboratory Tests in Diagnosis of Myasthenia Gravis / 영남의대학술지

PURPOSE: This study was undertaken to evaluate the clinical usefulness of Tensilon test, repetitive nerve stimulation test(RNST), single fiber EMG(SFEMG) test and acetylcholine receptor antibody(AchR Ab) assay for making diagnosis of myasthenia gravis(MG). METHOD: These tests were performed in 21 MG patients which were classified into 11 ocular, 5 mild generalized, 4 moderate generalized, and 1 chronic severe MG. RESULT: The overall positivity of Tensilon test, SFEMG and AchR Ab was 95%, 87%, and 76% respectively. The overall positivity of RNST was 67%; 38% on flexor carpi ulnaris, 43% on adductor digiti quinti and 62% on orbicularis oculi muscles. The positivity of each test was higher in generalized MG group than in ocular MG group. But we could observe the statistically significant difference only in the RNST(p<0.05). CONCLUSION: Tensilon test showed the highest positivity in all MG groups. So we would like to recommend the Tensilon test for the diagnosis of MG at first, followed by RNST and AchR Ab assay, and SFEMG would be indicated to MG group which showed relatively low postivity in other tests.

Mucoepidermoid carcinoma of the palate