Vogt-Koyanagi-Harada Disease Presented with Bilateral Optic Disc Edema

Vogt-Koyanagi-Harada (VKH) disease is a multisystem inflammatory disease that occurs when body tissues including melanocytes are damaged by autoimmunity. The typical symptoms of VKH are bilateral multifocal uveitis and serous retinal detachment. Our patient was misdiagnosed as idiopathic intracranial hypertension because there were no other characteristic neurological symptoms other than bilateral optic disc edema. However, intracranial pressure was normal and pleocytosis was found, so VKH disease could be diagnosed. Bilateral optic disc edema may be a initial manifestation of VKH disease.

Sodium Tetradecyl Sulphate Sclerotherapy for Lateral Malleolar Bursitis of the Ankle

Background@#The aim of this study was to evaluate clinical outcomes of sodium tetradecyl sulphate (STS) sclerotherapy for conservative treatment of lateral malleolar bursitis of the ankle. @*Methods@#We reviewed data from 20 consecutive patients (20 ankles) who underwent STS sclerotherapy between August 2018 and June 2019. After aspiration of fluid from the lateral malleolar bursal sac, 2 mL (20 mg) STS was injected into the sac. Clinical outcomes and side effects and complications were evaluated at 2 weeks, 3 months, 1 year, and 2 years after sclerotherapy. Responses to treatment were assessed according to degree of fluctuation, shrinkage of the bursal sac, and soft-tissue swelling. The 36-item short form survey (SF-36) was completed for each patient before and after therapy. @*Results@#Complete response was observed in 17 patients (85%), and partial response was observed in 3 patients (15%) after STS sclerotherapy. SF-36 physical component scores improved from 62.2 (interquartile range, 5.2) before therapy to 70.0 (interquartile range, 7.9) at last follow-up (p < 0.05). One patient (5%) experienced transient hyperpigmentation at the injection site. No major complications occurred. @*Conclusions@#STS sclerotherapy was an effective and safe treatment for patients with lateral malleolar bursitis of the ankle.

Diesel Exhaust Particles Impair Therapeutic Effect of Human Wharton’s Jelly-Derived Mesenchymal Stem Cells against Experimental Colitis through ROS/ERK/cFos Signaling Pathway

Background and Objectives@#Epidemiological investigations have shown positive correlations between increased diesel exhaust particles (DEP) in ambient air and adverse health outcomes. DEP are the major constituent of particulate atmospheric pollution and have been shown to induce proinflammatory responses both in the lung and systemically. Here, we report the effects of DEP exposure on the properties of human Wharton’s jelly-derived mesenchymal stem cells (WJ-MSCs), including stemness, regeneration, and immunomodulation. @*Methods@#and Results: Non-apoptotic concentrations of DEP (10 μg/ml) inhibited the migration and osteogenic differentiation capacity of WJ-MSCs. Gene expression profiling showed that DEP increased intracellular reactive oxygen species (ROS) and expression of pro-inflammatory and metabolic-process-related genes including cFos. Furthermore, WJ-MSCs cultured with DEP showed impaired suppression of T cell proliferation that was reversed by inhibition of ROS or knockdown of cFos. ERK inhibition assay revealed that DEP-induced ROS regulated cFos through activation of ERK but not NF-κB signaling. Overall, low concentrations of DEP (10 μg/ml) significantly suppressed the stemness and immunomodulatory properties of WJ-MSCs through ROS/ERK/cFos signaling pathways. Furthermore, WJ-MSCs cultured with DEP impaired the therapeutic effect of WJ-MSCs in experimental colitis mice, but was partly reversed by inhibition of ROS. @*Conclusions@#Taken together, these results indicate that exposure to DEP enhances the expression of pro-inflammatory cytokines and immune responses through a mechanism involving the ROS/ERK/cFos pathway in WJ-MSCs, and that DEP-induced ROS damage impairs the therapeutic effect of WJ-MSCs in colitis. Our results suggest that modulation of ROS/ERK/cFos signaling pathways in WJ-MSCs might be a novel therapeutic strategy for DEP-induced diseases.

The Efficacy of Three-Dimensional Sweeping Mode Extracorporeal Shockwave Treatment for Plantar Fasciitis / 대한족부족관절학회지

Purpose@#This was a pilot study to examine the clinical usefulness of the newly developed three-dimensional sweep mode extracorporeal shockwave treatment (ESWT) in patients with plantar fasciitis. @*Materials and Methods@#Three-dimensional sweep mode ESWT was performed once a week for 5 weeks in patients with plantar fasciitis who showed no improvement with the conventional conservative treatment. A 100-mm visual analogue scale (VAS) reading for pain from walking and at rest after walking were collected before the treatment and 8 and 16 weeks after the initial treatment. In addition, the Foot and Ankle Outcome Score (FAOS) and EuroQol-5-dimension (EQ-5D) scores before and 16 weeks after the treatment were evaluated. @*Results@#VAS for pain for walking improved from 50.60±8.38 to 19.80±15.61 at 8 weeks after the initial treatment (p=0.008) and 9.80±9.62 at 16 weeks after the treatment (p<0.001). VAS for pain at rest after walking improved from 36.60±19.55 to 11.80±12.95 at 8 weeks after the initial treatment (p=0.052) and 8.80±8.87 at 16 weeks after the treatment (p=0.024). Preoperative FAOS increased from an average of 74.80±9.73 before the treatment to an average of 81.00±8.86 at week 16 after the procedure (p=0.49) and compared to pre-treatment levels, there was a decrease of one level in the anxiety/depression domain of the EQ-5D, post-treatment. @*Conclusion@#The results of this preliminary study confirmed that the newly developed EWST with the smart forging sweep mode was effective in improving pain and function in plantar fasciitis.

Serious Neurological Disorders That Mimic Bell’s Palsy: A 10-Year Experience / 대한신경근육질환학회지

Background@#Unilateral peripheral facial nerve palsy may have a detectable cause (secondary facial nerve palsy) or may be idiopathic (Bell’s palsy). Facial palsy is attributable to various causes ranging from mild infection to severe neurological disorders. We investigated the prevalence and types of serious neurological disorders in patients with unilateral facial palsy. @*Methods@#We reviewed the medical records of patients with unilateral facial nerve palsy and identified patients diagnosed with facial palsy secondary to serious or life-threatening causes. We investigated the clinical characteristics, as well as electrodiagnostic and imaging findings in these patients. @*Results@#Of 924 patients with facial palsy, 11 patients (1.2%) were diagnosed with the following serious neurological disorders: acoustic schwannoma in two patients, facial nerve schwannoma, glossopharyngeal schwannoma, meningioma, epidermoid cyst, parotid gland tumor, pontine infarct, skull base osteomyelitis, brain metastasis, and pachymeningitis. @*Conclusions@#Although unilateral facial palsy is rarely associated with serious neurological disorders, early detection of the etiopathogenetic contributors is important for prompt initiation of optimal management. Therefore, clinicians should be mindful of disorders that can mimic Bell’s palsy.

Clinical approach to patients with dizziness

Although dizziness is common, it is one of the most challenging symptoms in clinical medicine. Defining the type of dizziness has been considered the first step when approaching patients with dizziness. This approach was based on the belief that each type of dizziness reflects a specific underlying mechanism. A more recent approach involves grouping patients into 4 major categories: 1) acute prolonged spontaneous dizziness, 2) recurrent spontaneous dizziness, 3) recurrent positional dizziness, and 4) chronic persistent dizziness and imbalance. Vestibular neuritis and strokes are the most common causes of acute prolonged spontaneous dizziness, and neuro-otologic examination findings play a key role in the differential diagnosis. Careful history-taking is extremely important in diagnosing the disorders that cause recurrent spontaneous dizziness since the findings of the clinical examination and laboratory evaluations are often negative without a confirmatory diagnostic tool. Benign paroxysmal positional vertigo is a predominant cause of recurrent positional vertigo and can easily be treated with canalith-repositioning maneuvers. Chronic persistent dizziness and imbalance occur in various degenerative or psychiatric disorders that frequently require a referral to a specialist. This new approach may be more practical for managing patients with dizziness.

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 approach to patients with dizziness

Although dizziness is common, it is one of the most challenging symptoms in clinical medicine. Defining the type of dizziness has been considered the first step when approaching patients with dizziness. This approach was based on the belief that each type of dizziness reflects a specific underlying mechanism. A more recent approach involves grouping patients into 4 major categories: 1) acute prolonged spontaneous dizziness, 2) recurrent spontaneous dizziness, 3) recurrent positional dizziness, and 4) chronic persistent dizziness and imbalance. Vestibular neuritis and strokes are the most common causes of acute prolonged spontaneous dizziness, and neuro-otologic examination findings play a key role in the differential diagnosis. Careful history-taking is extremely important in diagnosing the disorders that cause recurrent spontaneous dizziness since the findings of the clinical examination and laboratory evaluations are often negative without a confirmatory diagnostic tool. Benign paroxysmal positional vertigo is a predominant cause of recurrent positional vertigo and can easily be treated with canalith-repositioning maneuvers. Chronic persistent dizziness and imbalance occur in various degenerative or psychiatric disorders that frequently require a referral to a specialist. This new approach may be more practical for managing patients with dizziness.

Orbital Myositis of the Superior Rectus Muscle Presenting as Vertical Diplopia

No abstract available.

Post Infectious Cerebellar Ataxia with Abnormal Brain Magnetic Resonance Imaging and Single Photon Emission Computed Tomography Findings / 대한평형의학회지

A 84-year-old woman presented with a two weeks history of dizziness, slurred speech and ataxia. The neurological exam showed spontaneous left beating nystagmus, horizontal gaze evoked nystagmus and limb ataxia. A few weeks earlier, she had an upper airway infection. Brain MRI revealed diffuse leptomeningeal enhancement in the both cerebellar hemisphere and brain single photon emission computed tomography (SPECT) showed hyperperfusion in both cerebellar hemisphere. Extensive laboratory studies with cerebrospinal fluid analysis did not reveal any etiologic factors. She was started on methylprednisolone (1 g/day for 7 days), gradually improved over the weeks. Post infectious cerebellar ataxia is a neurologic complication that occasionally follows systemic viral and bacterial infections. This case demonstrates that cerebellar abnormalities can be detected by brain magnetic resonance imaging and SPECT.

Comparison of Four Methods calculating Ankle Brachial Indexes for Coexistent Intracranial and Extracranial Artery Stenoses in the Patients with Ischemic Stroke

BACKGROUND: The ankle brachial index (ABI) is a useful screening tool for peripheral arterial disease. It has been documented that the cerebral atherosclerosis is more frequent in stroke patients with abnormal ABI than in those with normal ABI. The present study compared the different calculation methods of ABI for coexistent intracranial and extracranial arterial stenosis (IECAS) in ischemic stroke. METHODS: We reviewed the data of patients admitted for acute ischemic stroke or transient ischemic attack. The following four distinct ABIs were calculated: the ratio of the higher (ABI-H), lower (ABI-L), or either of the systolic blood pressures (SBPs) of the posterior tibial (ABI-PT) and dorsalis pedis (ABI-DP) arteries relative to the higher of the brachial SBP. We compared the values of these four ABI measures relative to the presence of any IECAS using receiver operating characteristic (ROC) curve analysis. RESULTS: ABI-H, ABI-L, ABI-PT, and ABI-DP were abnormal (≤ 0.9) in 19 (13.1%), 36 (24.8%), 29 (20%), and 30 (20.7%) of 145 patients, respectively. The area under the ROC curve was significantly larger for ABI-H, ABI-L, and ABI-DP than for ABI-PT for any type of IECAS. The extracranial stenosis was more frequent when any of the four ABIs was abnormal, while intracranial stenosis was not correlated with the four ABIs. The IECAS was more frequent for abnormal ABI-H and ABI-DP than for normal ABIs. CONCLUSIONS: Our studies suggested that ABIs based on a higher SBP of the PT or DP (i.e., ABI-H) are more strongly associated with the presence of IECAS than are those using the SBP of the PT (ABI-PT). Extracranial artery stenosis seems to be more strongly associated with ABI than is intracranial stenosis.

Positional Dizziness and Vertigo without Nystagmus and Orthostatic Hypotension / 대한평형의학회지

According to the Barany Society classification of vestibular symptoms, positional dizziness or vertigo is defined as dizziness or vertigo triggered by and occurring after a change of head position in space relative to gravity. Thus, positional dizziness or vertigo should be differentiated from orthostatic dizziness or vertigo, which is triggered by and occurs upon rising. Benign paroxysmal positional vertigo (BPPV) is the most common positional vertigo and accompanied by a characteristic paroxysmal positional nystagmus. But a problem occasionally encountered in clinical practice is the presence of a positive history of BPPV with a negative diagnostic maneuver for positional nystagmus. Orthostatic hypotension may be dependent upon various neurogenic and non-neurogenic disorders and conditions. Combination of non-pharmacological and pharmacological treatment improve orthostatic tolerance.

Extension Type Locked Metacarpophalangeal Joint of the Little Finger due to Hyperextension Injury / 대한정형외과학회잡지

The extension type locked metacarpophalangeal joint of the little finger is an extremely rare condition characterized by loss of flexion with little or no loss of extension. The most common cause for locking is entrapment of a strap of the ruptured palmar plate. We treated a locked metacarpophalangeal joint of the little finger due to a hyperextension injury. The mechanism of locking was a torn part of the palmar ligament that forcefully rides across the prominence of the radial condyle of the metacarpal bone together with the accessory collateral ligament. These dislocated parts of the ligament formed a constricting bundle that prevented closed reduction. Locking was released successfully by an open reduction using the dorsal approach of the metacarpophalangeal joint without complication.

Passive Skeletal Muscle Excursion after Tendon Rupture Correlates with Increased Collagen Content in Muscle

PURPOSE: This study was designed to measure time-dependent changes in muscle excursion and collagen content after tenotomy, and to analyze the correlation between muscle excursion and collagen content in a rabbit model. MATERIALS AND METHODS: Twenty-four rabbits underwent tenotomy of the second extensor digitorum longus (EDL) muscles on the right legs and were randomly assigned to three groups based on the period of time after tenotomy (2, 4, and 6 weeks). The second EDL muscles on left legs were used as controls. At each time after tenotomy, passive muscle excursion and collagen content, determined by hydroxyproline content, were measured bilaterally, and the ratio of each value to the normal one was used. RESULTS: The mean ratio of muscle excursion after tenotomy to the value of the control decreased in a time-dependent fashion: 92.5% at 2 weeks, 78.6% at 4 weeks, and 55.1% at 6 weeks. The mean ratio of hydroxyproline content in muscle to the value of the control increased in a time-dependent fashion: 119.5% at 2 weeks, 157.3% at 4 weeks, and 166.6% at 6 weeks. There was a significant negative correlation between the ratio of hydroxyproline content in muscle after tenotomy to the control values and the ratio of muscle excursion after tenotomy to the control values (r=-0.602, p=0.002). CONCLUSION: The decrease in muscle excursion seems to correlate with the increase in collagen content in the muscle in a time-dependent fashion following tenotomy.

Cerebral Arteriovenous Malformation Associated with Moyamoya Disease

The coexistence of moyamoya disease (MMD) with an arteriovenous malformation (AVM) is exceedingly rare. We report two cases of AVM associated with MMD. The first case was an incidental AVM diagnosed simultaneously with MMD. This AVM was managed expectantly after encephalo-duro-arterio-synangiosis (EDAS) as the main feeders stemmed from the internal carotid artery, which we believed would be obliterated with the progression of MMD. However, the AVM persisted with replacement of the internal carotid artery feeders by new external carotid artery feeders from the EDAS site. The AVM was eventually treated with gamma knife radiosurgery considering an increasing steal effect. The second case was a de novo AVM case. The patient was initially diagnosed with MMD, and acquired an AVM eight years later that was slowly fed by the reconstituted anterior cerebral artery. Because the patient remained asymptomatic, the AVM is currently being closely followed for more than 2 years without further surgical intervention. Possible differences in the pathogenesis and the radiologic presentation of these AVMs are discussed with a literature review. No solid consensus exists on the optimal treatment of MMD-associated AVMs. Gamma knife radiosurgery appears to be an effective treatment option for an incidental AVM. However, a de novo AVM may be managed expectantly considering the possible risks of damaging established collaterals, low flow characteristics, and probably low risks of rupture.

Microvascular Decompression for Familial Hemifacial Spasm : Single Institute Experience

OBJECTIVE: The purpose of this study was to evaluate the characteristics and surgical outcomes of familial hemifacial spasm (HFS) and to discuss the role of genetic susceptibility. METHODS: Between 2001 and 2011, 20 familial HFS patients with ten different pedigrees visited our hospital. The data from comprehensive evaluation of these patients, including clinical, radiological and electrophysiological data and surgical outcomes were reviewed to characterize familial HFS and to compare the characteristics between familial HFS and sporadic HFS. RESULTS: According to the family tree, the inheritance pattern was difficult to define clearly using these data. Radiologic findings suggested that the vertebral artery (VA) was a more frequent offender in familial HFS than in sporadic cases (35.0% vs. 10.0%, p<0.001). Chi-square test showed that there were no correlation between VA tortuosity and underlying morbidity such as diabetes or hypertension (p=0.391). Eighteen out of 19 patients who underwent microvascular decompression showed no residual spasm. Other features of familial HFS overlap with sporadic cases. These findings suggest that certain genetic susceptibilities rather than hypertension or diabetes may influence vascular tortuosity and HFS development. CONCLUSION: In this study, familial HFS seems not so different from sporadic cases. Authors thought familial HFS could have heterogeneous etiology. Further study of familial HFS including clinical, anatomic, genetic, and molecular information may help identify a gene or trait that can provide insight into the mechanisms of sporadic and familial HFS.

A Case of Neonatal Isoimmune Hemolytic Disease due to Anti-Mi(a) Antibody with Massive Fetomaternal Hemorrhage / 대한주산의학회잡지

Authors experienced a newborn treated with severe anemia transferred to our hospital due to pulselessness and apnea shortly after birth. Laboratory analysis of the blood on admission revealed hemoglobin 3.1 g/dL, reticulocyte 11.0%. Kleihauer-Betke test for fetal hemoglobin from maternal blood was seen Hgb F 7%, then we suggested almost 180 ml fetomaternal hemorrhage. But, anemia was not improved despite repeated packed RBC transfusion. So, we evaluated the other cause of intractable anemia. The results were as follows; the Coombs' test was positive. The antibody identification test using mother's serum revealed anti-Mia antibody. The patient improved with supportive treatment, but got hypoxic brain injury due to massive fetomaternal hemorrhage. At day 29, the infant was doing well and was discharged. We report a case of neonatal isoimmune hemolytic disease due to anti-Mia with massive fetomaternal hemorrhage with a brief review of the related literatures.

Delayed Unilateral Soft Palate Palsy without Vocal Cord Involvement after Microvascular Decompression for Hemifacial Spasm

Microvascular decompression is a very effective and relatively safe surgical modality in the treatment of hemifacial spasm. But rare debilitating complications have been reported such as cranial nerve dysfunctions. We have experienced a very rare case of unilateral soft palate palsy without the involvement of vocal cord following microvascular decompression. A 33-year-old female presented to our out-patient clinic with a history of left hemifacial spasm for 5 years. On postoperative 5th day, patient started to exhibit hoarsness with swallowing difficulty. Symptoms persisted despite rehabilitation. Various laboratory work up with magnetic resonance image showed no abnormal lesions. Two years after surgery patient showed complete recovery of unitaleral soft palate palsy. Various etiologies of unilateral soft palate palsy are reviewed as the treatment and prognosis differs greatly on the cause. Although rare, it is important to keep in mind that such complication could occur after microvascular decompression.

WEBINO (Walleyed Bilateral Internuclear Ophthalmoplegia) Syndrome in Progressive Supranuclear Palsy

WEBINO (walleyed bilateral internuclear ophthalmoplegia) syndrome is a rare disorder characterized by bilateral exotropia on primary gaze, bilateral internuclear ophthalmoplegia, and impaired convergence. It is mainly reported in patients with cerebrovascular diseases and multiple sclerosis, but rare cases have also been described in patients with neurodegenerative diseases such as progressive supranuclear palsy (PSP). We report WEBINO syndrome in a PSP patient.

Association of Apolipoprotein E Gene Polymorphism With Cognitive Function of the Elderly Residents in a Rural Community

BACKGROUND: It is not clear whether polymorphism of the apolipoprotein E (ApoE) gene influences the cognition of community residents. The aim of this study was to establish the association between ApoE gene polymorphism and cognitive function in an elderly rural community in Korea. METHODS: A total of 388 subjects aged 65 and over were recruited. Demographic characteristics, past history of illness, and scores on the Korean version of the Mini Mental State Examination (K-MMSE), the Geriatric Depression Scale . Short Form (GDS-S), and the Korean version of Instrumental Activities of Daily Living (K-IADL) were evaluated. The lipid profile and ApoE genotype were sampled from 377 of the participants. RESULTS: Of the entire cohort, 75% had less than 6 years of education, and 30% were illiterate. The frequencies of the ApoE epsilon2, ApoE epsilon3, and ApoE epsilon4 alleles were 48 (6.6%), 372 (86.9%), and 49 (6.5%), respectively. The K-MMSE score was much lower in those with two ApoE epsilon3 alleles than in those with only one ( p=0.046). However, the numbers of ApoE epsilon2 alleles (p=0.976) and ApoE epsilon4 alleles (p=0.934) carried by the individual were not associated with K-MMSE score. Both K-IADL (p<0.001) and GDS-S (p<0.001) scores were significantly correlated with K-MMSE score. Grouping of the participants into three groups according to K-MMSE score (i.e., 0-17 , 18-24, and 25-30) also revealed that this score was correlated with K-IADL score (p<0001), GDS-S score (p<0.001), and the ApoE epsilon3 allele (p=0.035). CONCLUSIONS: These results suggest that the ApoE epsilon3 allele has a negative influence on cognitive function (K-MMSE) in this rural community. Surprisingly, we were unable to detect any relationship between the ApoE epsilon4 allele and cognitive function. There was a positive correlation between K-MMSE, K-IADL, and GDS-S scores.