Reversible Gait Disturbance Due to Vitamin B12 Deficiency

Vitamin B12 deficiency causes a variety of symptoms in the hematologic, gastrointestinal tract, and neurologic deficit. We report a rare case of progressive gait disturbance, bradykinesia and dysarthria which was that vitamin B12 deficiency was diagnosed as the cause, and symptoms improved after intramuscular cobalamin treatment. Vitamin B12, homocysteine, and methylmalonic acid tests are recommended for patients with progressive gait disturbance, even in the absence of obvious anemia symptoms.

Reversible Cerebral Vasoconstriction Syndrome Misdiagnosed as Moyamoya Disease with Transient Ischemic Attack as Initial Manifestation

Reversible cerebral vasoconstriction syndrome (RCVS) is a disease characterized by reversible and multiple stenoses of cerebral blood vessels that improve within 3 months, accompanied by thunderclap headache. Here, we report an interesting case of RCVS initially misdiagnosed as Moyamoya disease with transient ischemic attack. A 45-year-old woman visited the Neurology Department of Eulji University Hospital. The patient was initially diagnosed with Moyamoya disease with transient ischemic attack. However, follow-up magnetic resonance angiography performed 12 months after the patient was appropriately diagnosed as having RCVS.

Low-dose steroid-induced tumor lysis syndrome in a hepatocellular carcinoma patient

Tumor lysis syndrome is rare in hepatocellular carcinoma (HCC), but it has been reported more frequently recently in response to treatments such as transcatheter arterial chemoembolization (TACE), radiofrequency thermal ablation (RFTA), and sorafenib. Tumor lysis syndrome induced by low-dose steroid appears to be very unusual in HCC. We report a patient with hepatitis-C-related liver cirrhosis and HCC in whom tumor lysis syndrome occurred due to low-dose steroid (10 mg of prednisolone). The patient was a 90-year-old male who presented at the emergency room of our hospital with general weakness and poor oral intake. He had started to take prednisolone to treat adrenal insufficiency 2 days previously. Laboratory results revealed hyperuricemia, hyperphosphatemia, and increased creatinine. These abnormalities fulfilled the criteria in the Cairo-Bishop definition of tumor lysis syndrome. Although the patient received adequate hydration, severe metabolic acidosis and acute kidney injury progressed unabated. He finally developed multiple organ failure, and died 3 days after admission. This was a case of tumor lysis syndrome caused by administration of low-dose steroid in a patient with HCC.

Visceral Fat as a Useful Parameter in the Differential Diagnosis of Crohn's Disease and Intestinal Tuberculosis

BACKGROUND/AIMS: Because of the similarities in the clinical presentations of Crohn's disease (CD) and intestinal tuberculosis (ITB), differential diagnosis is critical. Mesenteric adipose tissue hypertrophy and creeping fat are characteristic features of CD. The purpose of this study was to assess the usefulness of visceral fat for the differential diagnosis of CD and ITB. METHODS: We conducted a retrospective review of 50 patients with findings of CD or ITB between January 2005 and July 2008. Abdominal computed tomography (CT) was performed on all subjects during their first evaluation. The abdominal fat area was assessed using quantitative abdominal CT. RESULTS: The ratio of visceral fat to total fat (VF/TF) was significantly higher in male CD patients than in male ITB patients. The ratio of visceral fat to subcutaneous fat (VF/SF) was also higher in CD patients than in patients with ITB. For a VF/TF cut-off value of 0.46, the sensitivity and specificity for the diagnosis of CD were 42.1% and 93.3% respectively, with positive and negative predictive values of 88.9% and 56.0%, respectively. CONCLUSION: Measurement of the abdominal fat area using CT can be clinically useful for the differential diagnosis of CD and ITB.

A Case of Enteritis Cystica Profunda Accompanied by a Lipoma in the Duodenal Bulb / 대한내과학회지

Enteritis cystica profunda (ECP) is a very rare hyperplastic benign lesion of the small intestine. Histologically, it is characterized by mucin-filled cystic spaces frequently lined by benign epithelium within the submucosa and muscularis propria. ECP occurs much less frequently than gastritis cystica profunda (GCP) or colitis cystica profunda (CCP). It is most often seen in adults with Crohn's disease, Peutz-Jeghers syndrome, hamartomatous polyp, or congenital anomalies. We encountered a case of ECP (90 x 15 mm) accompanied by an elongated lipoma in the duodenal bulb, in the absence of any other disease. An endoscopic polypectomy proved sufficient for diagnosing and treating the ECP. To our knowledge, this is the first reported case of ECP accompanied by a lipoma in the duodenal bulb in the absence of any other intestinal disease.

Synchronous Double Primary Hepatic Cancer: Hepatocellular Carcinoma and Intrahepatic Cholangiocarcinoma / 대한소화기학회지

No abstract available.

Cervical Myelopathy Resulting From Non-Rheumatoid Atlantoaxial Subluxation

Degenerative pannus arising from the atlanto-axial articulation is a rare entity, which is associated with chronic atlanto-axial instability in degenerative arthropathies. Due to cervical instability and the mass effect on the spinal cord, it can produce severe neck pain and compressive myelopathy. We report a case of cervical myelopathy resulting from non-rheumatoid atlanto-axial subluxation. The posterior stabilization provided excellent neurological improvement and pannus regression. Early recognition of this condition is important as the clinical condition will deteriorate without surgical interventions.

A Case of Intestinal Behcet's Disease Complicated Enterocutanous Fistula with a Good Response to Adalimumab

Behcet's disease (BD) is a multisystemic disorder, which is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesion. Although intestinal involvement is an uncommon manifestation of the BD, it leads to a poor prognosis, as a result of a high complication, such as intestinal perforation, fistula formation, and massive hemorrhage. Intestinal BD has the tendency for the resistance to conventional medical treatment, and it often requires a surgical intervention because of severe complication. Although the causes of intestinal BD are unknown, some studies show that tumor necrosis factor Alpha (TNF-alpha) plays a key role in the pathogenesis of BD. Therefore, anti-TNF-alpha monoclonal antibody, such as adalimumab, is one of the useful treatment for refractory and relapsed intestinal BD. We describe a patient who had intestinal BD complicated enterocutanous fistula with a good response to adalimumab.

A Case of Klinefelter's Syndrome Accompanying with Polymyositis

Klinefelter's syndrome which is characterized by hypogonadism with karyotype abnormality (47 XXY or 46 XY/47 XXY) in males has been reported to be associated with autoimmune diseases including rheumatoid arthritis and systemic lupus erythematosus. However, Klinefelter's syndrome accompanying with polymyositis has rarely been reported. We report a case of KFS with polymyosits in a 38-year old man for the first time in Korea.

A Case of Pneumocystis Jirovecii Pneumonia in a Patient with Rheumatoid Arthritis

We report a 67-year-old woman who developed Pneumocystis jirovecii pneumonia (PJP) as a complication of treatment with immunosuppressant, such as methotrexate and leflunomide, for rheumatoid arthritis. She had mild fever and non-productive cough, but there was neither symptoms of dyspnea nor typical X-ray findings at initial presentation. However, rapid progression on a chest X-ray, respiratory symptoms and findings of high-resolution chest computed tomography in a few days suggested a possibility of PJP infection. A polymerase chain reaction-based detection of Pneumocystis jirovecii in induced sputum allowed us an early diagnosis of PJP. Through a preemptive treatment with Trimethoprim-sulfamethoxazole, she fully recovered from PJP.

Autoimmune Pancreatitis Accompanied by Tubulointerstitial Nephritis / 대한내과학회지

Autoimmune pancreatitis (AIP) is type of chronic pancreatitis characterized by an autoimmune inflammatory process. IgG4-positive plasma cells can be found in both the pancreas and at extrapancreatic sites of involvement. A 60-year-old man presented with abdominal discomfort. Computed tomography revealed enlargement of the pancreas and kidneys as well as low-attenuated lesions in both kidneys. MRCP showed narrowing of the distal common bile duct and the proximal pancreatic duct. The serum IgG level was elevated, but IgG4 was normal. Histologic examination revealed lymphoplasma infiltration in the renal tubulointerstitium associated with fibrosis along with many IgG4-positive plasma cells, thereby confirming the diagnosis of tubulointerstitial nephritis-associated AIP. The abnormalities in the clinical, laboratory, and radiological findings improved after oral steroid treatment. This is a case of autoimmune pancreatitis accompanied by tubulointerstitial nephritis diagnosed by renal biopsy. Clinicians should suspect the possibility of extrapancreatic organ involvement in autoimmune pancreatitis, especially when abnormalities in other organs are suggested. Proper examinations must be performed.

Platelet Function Assay for Clopidogrel and Ticlopidine in Patients With Ischemic Stroke

BACKGROUND: The rapid platelet function assay (RPFA) has recently been developed and used to monitor the antiplatelet effects on the P2Y12 ADP receptor. We describe the platelet response to clopidogrel and ticlopidine using the RPFA and identify the clinical factor related to laboratory resistance in patients with ischemic stroke. METHODS: Of the 172 outpatients with ischemic stroke or transient ischemic attack (TIA) enrolled in this study, 86 were taking clopidogrel (75 mg/day) and 86 were taking ticlopidine (500 mg/day). Demographic data, vascular risk factors, stroke subtypes, and the results of blood tests were recorded. Inhibition is described as the percentage change from baseline aggregation, and is calculated from the P2Y12 reaction unit (PRU) and the base PRU on the RPFA. Those patients who displayed ineffective aggregation-inhibition (inhibition <20%) on the RPFA were defined as nonresponders. RESULTS: The response of platelet aggregation-inhibition to clopidogrel and ticlopidine exhibited a variable distribution (PRU; coefficient of variability, 0.477). Ineffective platelet inhibition was detected in 25.6% of the clopidogrel group and 3.5% of the ticlopidine group (p<0.001). In addition to clopidogrel, TIA and diabetes exhibited significantly higher ineffective platelet inhibition in a univariate analysis. In the multivariate analysis, clopidogrel and TIA remained significant, and diabetes fell to borderline significance (p=0.061). CONCLUSIONS: The response to clopidogrel and ticlopidine can vary between patients. Platelet inhibition is lower for clopidogrel than for ticlopidine on the platelet function test in patients with ischemic stroke. The clinical impact of these results remains uncertain; further investigations are needed.

Correlation between Complicated Diverticulitis and Visceral Fat

The aim of this study was to examine the relationship of complications related to diverticulitis and visceral obesity. The study was based on a retrospective case note review conducted at the Hanyang University Hospital. Patients were diagnosed with diverticulitis based on clinical symptoms and abdominal computed tomography (CT) findings and divided into two groups: those admitted with complicated diverticulitis and those with a simple diverticulitis episode. We compared the body mass index (BMI) and degree of visceral obesity, measured by abdominal CT. The study included 140 patients, 87 (62.1%) were simple diverticulitis and 53 (37.9%) were complicated diverticulitis. In the complicated diverticulitis group, 9 (6.4%) cases were recurrent, 29 (20.7%) were perforation or abscess patients, and 28 (20%) were patients with systemic inflammatory response syndrome (SIRS). Of the SIRS patients, 13 were involved in other complication groups. When comparing in the two groups, the complicated diverticulitis group had a significantly higher visceral fat area (128.57 cm2 vs 102.80 cm2, P = 0.032) and a higher ratio of visceral fat area/subcutaneous fat area (0.997 vs 0.799, P = 0.014). Visceral obesity is significantly associated with complications of diverticulitis.

Novel Swine-Origin Influenza A (H1N1) Viral Encephalitis

The World Health Organization declared that a new strain of novel swine-origin influenza A (H1N1) virus was responsible for the pandemic infection in June 2009. We report a case of encephalitis diagnosed as the H1N1 virus infection. We describe a 17-year-old patient who had a seizure attack, diagnosed with a H1N1 virus infection via real time reverse-transcriptase polymerase chain reaction (RT-PCR). The H1N1 virus infection can be causative of the encephalitis, as with other influenza virus infections. Careful monitoring is essential for reducing complications.

Intracranial Lesions in a Patient With Carney Complex

Carney complex (CNC) is an autosomal dominant disorder that involves the heart, multiple endocrine systems, testis, skin, breast, and bone. We report a case of a 38-year-old woman, diagnosed with CNC, with a mass involving the petrous bone, a pituitary adenoma, a cavernous angioma, multiple cerebral aneurysms, and venous malformations. CNC should be included in the differential diagnoses of patients who have an atrial myxoma, brain tumor, or intracranial vascular malformations.

Electrical stimulation can facilitate vestibular compensation following unilateral labyrinthectomy in rats

To investigate the effects of electrical stimulation on vestibular compensation, which is the recovery of vestibular symptoms following unilateral labyrinthectomy (UL), intermittent electrical stimulation was applied to the injured vestibular portion in Sprague-Dawley rats. Vestibuloocular and vestibulospinal reflexes, electrical activity and expression of c-Fos protein in medial vestibular nuclei (MVN) were measured with time following UL. Spontaneous nystagmus occurred with frequency of 2.9+/-0.2 beats/sec at 2 hours after UL and disappeared after 72 hours. Electrical stimulation decreased the frequency of nystagmus significantly till 24 hours after UL. Roll head deviation was 107+/-9.7degree at 2 hours after UL and the deviation was maintained till 72 hours, but electrical stimulation decreased the deviation significantly 6 hours after UL. Resting activity of type I neurons in ipsilateral MVN to the injured vestibular side decreased significantly compared with control at 6 and 24 hours after UL, but the activity of type I neurons was recovered to control level by electrical stimulation at 24 hours after UL. Gain of type I neurons induced by sinusoidal rotation of 0.1 Hz decreased significantly till 24 hours after UL, but electrical stimulation restored the activity at 24 hours. The gain of type II neurons decreased significantly at 6 hours after UL, but electrical stimulation restored the activity. Expression of c-Fos protein was asymmetric between bilateral MVN till 24 hours after UL, but the asymmetry disappeared by electrical stimulation 6 hours after UL. These results suggest that electrical stimulation to the injured vestibular portion facilitates vestibular compensation following UL by restoration of symmetry of neuronal activity between bilateral vestibular nuclei resulting from increased activity in ipsilateral vestibular nuclei to the injured side.

A Case of Intradermal Nevus of the External Auditory Canal / 대한이비인후과학회지

While intradermal nevi are common benign pigmented skin tumors, their occurrence within the external auditory canal(EAC) is uncommon. We experienced a case of huge intradermal nevus which almost completely obstructed EAC without accompanying conductive hearing loss. It originated in the anterior wall of cartilaginous portion of EAC. It was treated by en bloc excision with split skin graft. The clinical and pathologic features of the intradermal nevus arising within the external auditory canal are presented, and the literatures were reviewed.

Clinical Study of Surgical Treatment for Frontal Sinus Disease: Osteoplastic Frontal Sinus Surgery versus Endoscopic Sinus Surgery / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Depending on the pathologic process the treatment of frontal sinus disease has consisted of obliteration of the sinus, or restoration of drainage into the nose. Endoscopic sinus surgery (ESS) gives advantages for dramatically reducing operative morbidity of surgery for frontal sinus disease by offering a minimally invasive alternative to previous osteoplastic or other radical frontal sinus surgery. We present our experience with 18 frontal sinus diseases in which osteoplastic approach or endoscopic sinus were attempted. MATERIALS AND METHODS: Retrospective review of 18 cases were done. Ten patients were treated by ESS, and 8 cases by osteoplastic frontal sinus surgery (OFSS) with obliteration. RESULTS: During the follow-up period, 10 patients treated by ESS had complete resolution of all symptoms but 3 cases treated by OFSS had at least one episode of headache. No recurrence of ESS patients has been noted to date according to the endoscopic follow-up of up to 16 months, and gradual absorbtion of obliterated fat without recurrence was observed. But there was no absorption of obliterated hydroxyapatite granule after long-term period. The frontal sinus can be visualized with follow-up endoscopy, and the difficult evaluation of the obliterated cavity can be avoided. CONCLUSION: ESS is much better than OFSS, but osteoma, fracture and some limited situations are bound to be treated by OFSS. In case of OFSS, hydroxyapatite obliteration shows long-term stability than fat.

Effect of Antioxidants on the Toxicity of Oxygen Radicals to the Cultured Mouse Schwann Cells in vitro / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Oxygen radical scavengers and inhibitors are known to have protective functions (or roles) against hypoxia and noise exposure in the cochlea and brain. The purpose of this study was to examine the toxic effect of oxygen radicals (xanthine oxidase and hypoxanthine) on cultured mouse facial nerve Schwann cells, and determine if antioxidants (TPEN and DFO) might ptotect Schwann cells from oxidant-induced neurotoxicity. MATERIALS AND METHODS: Dissociated cell cultures were prepared from the facial nerve of a mouse. After dissociation of Schwann cells, isolated cells were washed, resuspended in feeding medium, and plated onto poly-L-lysine-coated Aclar plastic cover slips (12 mm diameter) in petri dishes or in 96 well multichambers at cell density of 2X105 ceIls/coverslip or lX10(5) ceIls/we11. The feeding medium consisted of Eagle's minimum essential medium (MEM) containing 5% horse serum, 5 mg/ml D-glucose, and 25 ng/ml gentamicin. Cultures were grown in 5% CO2/95% atmosphere at 37degreesC, and the medium was renewed twice a week. Cultures grown for 4-5 days were utilized for experiments. Oxygen radical exposure was done using XO and HX, and antioxidant pretreatment was carried out using tetrakis (2-pyridylmethyl) ethylenediamine (TPEN) and desferrioxamine (DFO). Cytotoxicity assay was performed by MTT [3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide cytotoxic assay and inverted microscopy. RESULTS: Cell viability of cultured mouse Schwann cells treated with markedly decreased in a dose-dependent manner. Cultured mouse Schwann cells exposed to XO/HX for 4 hours showed degenerative changes such as the decrease of cell number and process. Pretreatment of 80 uM TPEN for 2 hours increased remarkably the cell viability of cultured Schwann cells exposed to 20 mU/ml XO/0.1 mM HX, while DFO did not show any protective effects on oxidant-induced neurotoxicity in these cultures. CONCLUSION: It is suggested that oxygen radicals induce neurotoxic effect on cultured mouse Schwann cells, and that selective antioxidants such as TPEN is very effective in blocking oxidant-induced neurotoxicity.

Salivary and Blood Flow Responses to Different Frequency Stimulation of the Cervical Sympathetic Nerve of the Submandibular Gland in the Cat / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: It has been assumed that salivary glands receive secretory fibers both from parasympathetic and sympathetic nerves. In fact, however, the existence of sympathetic secretory fibers in the cervical sympathetic nerve has not been established yet, because the salivary response to the cervical sympathetic stimulation is variable and short-lasting, and it tends to cease in spite of continued stimulation. This study investigated whether or not the cervical sympathetic nerve contains specific secretory fibers. MATERIALS AND METHODS: Salivary and blood flow responses to different frequency stimulation of the cervical sympathetic nerve, and often some autonomic drugs administration were observed from the submandibular gland in chloralose-anesthetized cats. RESULTS: 1) Low frequency stimulation (1-2 Hz) of the sympathetic nerve did not evoke salivary outflow and any change of blood flow, whereas high frequency stimulation of the nerve evoked salivary outflow and decrease of blood flow, in which salivary response tended to cease in spite of continued stimulation. 2) The salivary and blood flow responses to high frequency stimulation (20 Hz) of the nerve were not affected by the intravenous administration of propranolol, but were abolished by regitine. 3) Noradrenalin evoked salivary outflow and decreased blood flow which were not affected by the administration of propranolol but were abolished by regitine. 4) Isoproterenol increased blood flow but did not evoke salivary outflow, and the blood flow response was abolished by propranolol. CONCLUSION: These results suggest that the cervical sympathetic nerve does not contain specific secretory fibers and salivary outflow response to high frequency stimulation of the nerve may be due to either excitation of motor fibers innervating contractile elements of the excretory duct or chemical transmitters released from the vasomotor fibers.