Polyneuropathy Following Nitrous Oxide Abuse / 대한신경근육질환학회지

Nitrous oxide (N2O), an anesthetic gas, has been abused by some people for entertainment purposes. Recently, N2O (under the name of "Happy Balloon") abuse has become problematic among young people in Korea. N2O intoxication can develop a neuropathy, as well as other systemic disorders, either by inactivating vitamin B12 or by a direct neurotoxic mechanism. Here, we report a case of peripheral neuropathy with possible coexisting myelopathy following N2O abuse.

Renal Cholesterol Embolism during Warfarin Treatment

Cholesterol embolization syndrome (CES) usually occurs after endovascular procedures, it may also occurs after using anticoagulants and thrombolytics. We report a case of 66-year-old man with sudden elevation of creatinine after using warfarin due to cortical infarction. Histologic examinations revealed a cholesterol cleft on the arcuate artery. We concluded it as warfarin induced atheroembolic renal disease. Careful observation of kidneys is necessary in the case of renal abnormalities after using anticoagulation, considering the possibility of cholesterol embolism due to anticoagulant therapy.

Diagnostic Usefulness of Neuromuscular Ultrasound in Anatomical Localization of Peripheral Nerve Injury: Detailed Lesion Localization Using Neuromuscular Ultrasound in a Patient with Traumatic Ulnar Nerve Injury at the Hand

In the evaluation of peripheral nerve injury, nerve conduction studies and needle electromyography mainly focus on anatomical localization and functional evaluation of lesions. Whereas neuromuscular ultrasound has an advantage in structural assessment of lesions. In addition, muscle ultrasound can also be used to demonstrate muscle denervation without causing pain. We report a case of traumatic ulnar nerve injury at hand in which muscle ultrasound contributed to precise localization by provided detailed information about the extent of muscle denervation.

Isolated Dorsal Ulnar Cutaneous Nerve Injury Occurring During Activities of Daily Living

The dorsal ulnar cutaneous nerve (DUCN) is the sensory branch of ulnar nerve supplying sensation to the dorsal ulnar aspect of the hand. Isolated DUCN injury is known rare. We report 3 cases of isolated DUCN injury caused by stretch injury and mild blunt injury during activities of daily living. Isolated DUCN injury may more frequently occur than we thought. Nerve conduction study and ultrasonography are useful method to localize the lesion and to evaluate the structural etiology.

Sural Nerve Entrapment by Hypertrophic Peroneal Tubercle of the Calcaneus

No abstract available.

Carpal Tunnel Syndrome Caused by Scar Tissue Invasion of Median Nerve Fascicles

No abstract available.

Fregoli Syndrome Due to Right Hippocampal Vasculitis in Neuro-Behcet's Disease

We report a 40-year-old man with known neuro-Behcet's disease who showed Fregoli syndrome after a seizure attack. A patient with Fregoli syndrome strongly believes that the psychological identity of a familiar person remains unchanged while at the same time there is a marked change in physical identity. The anatomical basis of Fregoli syndrome is still unclear; our patient showed right hippocampal vasculitis in MRI and frontal dysfunction in neuropsychological testing.

Tumor-Associated Lymphocytes Predict Response to Neoadjuvant Chemotherapy in Breast Cancer Patients / 한국유방암학회지

PURPOSE: Tumor-associated lymphocyte numbers in breast cancer have been suggested as a new independent predictor of response to neoadjuvant chemotherapy in breast cancer patients. We therefore evaluated the relationship between pathologic complete response (pCR) and tumor-associated lymphocytes in tumors of such patients. METHODS: Between 2000 and 2009, we retrospectively evaluated 175 patients with primary breast cancer treated with neoadjuvant chemotherapy, followed by definitive surgical resection. Peritumoral lymphocytic infiltration (LI) and CD3+, CD8+, and forkhead box P3 (FOXP3)+ lymphocytes were assessed in pretreatment biopsy specimens. RESULTS: Nineteen (11%) patients achieved pCR. An elevated LI, CD3+, CD8+, or FOXP3+ lymphocytic infiltration; lower clinical T stage; human epidermal growth factor receptor 2 overexpression; and herceptin-based treatment were all significantly associated with pCR. Through a multivariate analysis, LI (odds ratio [OR], 1.26; p=0.024), clinical T stage (OR, 3.06; p=0.041), and the use of a herceptin-based regimen (OR, 4.95; p=0.004) were all significant independent predictors of pCR. Significantly higher numbers of tumor-associated lymphocytes and CD3+, CD8+, and FOXP3+ T-cells were observed in the following: high-grade tumors, tumors of positive nodal status, and tumors negative for hormone receptors. CONCLUSION: Tumor-associated lymphocytes are significantly associated with pCR, suggesting that tumor-associated lymphocytes may be an important pathological factor predicting a response to neoadjuvant chemotherapy in breast cancer patients.

Atypical Cheiro-oral Syndrome Presented With Bilateral Perioral Sensory Symptoms in Unilateral Thalamic Infarction

Cheiro-oral syndrome is a unilateral sensory impairment that is restricted to the homolateral hand and perioral area. An unusual clinical presentation with bilateral perioral involvement has also been described. Most of the responsible lesions involved the paramedian pontine tegmentum; thalamic involvement is extremely rare. We report herein a patient who presented with purely sensory symptoms involving the bilateral perioral regions and the distal thumb and index finger of the left hand following a small infarction in the right lateral thalamus.

Isaacs' Syndrome Associated With Acetylcholine Receptor Antibodies Developed After Removal of Malignant Thymoma

Isaacs' syndrome is a rare and heterogeneous syndrome of continuous muscle fiber activity that originates from peripheral nerves. We report a 56-year-old male patient who showed symptoms of Isaacs' syndrome after the removal of a malignant thymoma. Needle electromyography revealed spontaneously occurring repetitive myokymic discharge in the affected muscles. Acetylcholine receptor (AChR) antibodies were significantly elevated, but clinical and electrophysiologic findings did not indicate the presence of myasthenia gravis. We deduce that in Isaacs' syndrome, raised AChR antibodies may facilitate rather than inhibit cholinergic action.

Significance of Early Perfusion Weighted MRI for the Diagnosis of TIA

We report on three patients with transient focal neurologic deficits that completely resolved. In all cases, initial perfusion-weighted imaging (PWI) performed 1 hour after being free of symptoms showed a defect in the time-to-peak (TTP) map in the area with normal diffusion-weight imaging (DWI) findings. After 24 hours, DWI showed a high signal intensity in exactly the same area as the TTP defect. Therefore, early PWI provides a rapid evaluation of cerebral hemodynamics in transient ischemic attack.

Comparison between Therapeutic Efficacies of Histone Deacetylase Inhibitors and Established Drug Regimens Against Breast Cancer Cells using the Histoculture Drug Response Assay / 한국유방암학회지

PURPOSE: Histone deacetylase inhibitors (HDACIs) induce accumulation of acetylated histones in nucleosomes, which lead to reactivate gene expression and inhibit the growth and survival of tumor cells. This study evaluated the efficacy of HDACIs in breast cancer cells in comparison with other established drug regimens. METHODS: Drug responses of tumor samples from mastectomy specimens of 78 breast cancer patients were evaluated using the histoculture drug response assay (HDRA). Tumor inhibition rates (IRs) of established drug regimens such as doxorubicin, cyclophosphamide, doxorubicin with cyclophosphamide (AC), paclitaxel, docetaxel and doxorubicin with docetaxel (AT), as well as those of three HDACIs (SAHA, PXD101, and a novel compound CG-2) were evaluate. RESULTS: The percentages of chemosensitive tumors (chemoresponsiveness) were 26.9-60.3% with established regimens and 61.5-73.1% with HDACIs when the cutoff value for inhibition rate was set at 30%. Breast cancer cells appeared to be more chemoresponsive to HDACIs than to established drug regimens. Chemoresponsiveness to AT was the highest among the established drug regimens. A combination regimen offered higher activity than did a single drug (doxorubicin vs AT; p<0.001). HER2/Neu-overexpressing breast cancers were chemosensitive to SAHA and AT (p=0.031 and 0.04, respectively). CONCLUSION: Our findings show that breast cancer cells were sensitive to HDACIs, with therapeutic efficacies comparable to those of established drug regimens. Specific biological markers such as HER2/Neu could be assessed for effectiveness as HDACIs chemosensitivity markers in further clinical trials.

Two Cases of Congenital Ocular Motor Apraxia Associated With Cerebellar Vermian Dysplasia

Congenital ocular motor apraxia is a rare syndrome characterized by rotational head thrusts that attempt to compensate for the lack of voluntary eye movements. We describe the clinical, oculographic and magnetic resonance imaging features of two children with congenital ocular motor apraxia.

Two Cases of Methyl Bromide Intoxication Mimicking Bickerstaff's Brainstem Encephalitis

BACKGROUND: Methyl bromide is toxic to the central and peripheral nervous systems. Two patients with occupational exposure to this agent are described. CASE: Two cases of previously healthy young men were involved. They had worked in a fumigating plant spraying fruits using methyl bromide for several months before admission. They showed general weakness, severe ataxia, bilateral dysmetria and bilateral lateral gaze limitation. And gaze-evoked nystagmus was observed in one of them. Brain MRI showed symmetrically increased signal intensity lesions including the brainstem, cerebellum and splenium of corpus callosum. After conservative treatment, they gradually improved over the next few weeks. CONCLUSIONS: Symmetry and topography of our patients??clinical and radiographic abnormalities support the proposal that methyl bromide exposure can produce symptoms similar to Bickerstaff's brainstem encephalitis.

Involvement of Endoplasmic Reticulum Stress Response in the Neuronal Differentiation

Expressions of endoplasmic reticulum stress response (ERSR) genes were examined during the neuronal differentiation of rat fetal cortical precursor cells (rCPC) and rat pheochromocytoma PC12 cells. When rCPC were differentiated into neuronal cells for 7 days, early stem cell marker, nestin, expression was decreased from day 4, and neuronal markers such as neurofilament-L, -M and Tuj1 were increased after day 4. In this condition, expressions of BIP, ATF6, and phosphorylated PERK as well as their down stream signaling molecules such as CHOP, ATF4, XBP1, GADD34, Nrf2 and p58IPK were significantly increased, suggesting the induction of ERSR during neuronal differentiation of rCPC. ERSR was also induced during the differentiation of PC12 cells for 9 days with NGF. Neurofilament-L transcript was time-dependently increased. Both mRNA and protein levels of Tuj1 were increased after the induction, and the significant increase in NeuN was observed at day 9. Similar to the expression patterns of neuronal markers, BIP/GRP78 and CHOP mRNAs were highly increased at day 9, and ATF4 mRNA was also increased from day 7. These results strongly suggest the induction and possible role of ERSR in neuronal differentiation process. Further study to identify targets responsible for neuronal induction will be necessary.

Composite Neuroendocrine Carcinoma with Adenocarcinoma of the Stomach Mimicking Double Primary Cancer

A 67-year-old man presented with a 3-month history for intermittent epigastric pain and postprandial discomfort. Upper gastrointestinal endoscopy and contrast-enhanced axial computed tomographic scan demonstrated two separate tumors of the gastric antrum and the duodenal second portion, suggestive of the preoperative diagnosis of a double primary cancer. Pancreaticoduodenectomy with lymph node dissection was performed, and the microscopic features and immunohistochemical profiles of the resected specimen confirmed the diagnosis of the composite neuroendocrine carcinoma with adenocarcinoma of the stomach (mixed exocrine-endocrine carcinoma).

Prognosis of Bochdalek Hernia in Neonate after Change in Management Principle

There are considerable controversies in the management of congenital diaphragmatic hernia. By 1997, early operation, routine chest tube on the ipsilateral side and maintainingrespiratory alkalosis by hyperventilation were our principles (period I). With a transition period from 1998 to 1999, delayed operation with sufficient resuscitation, without routine chest tube, and permissive hypercapnia were adopted as our practice. High frequency oscillatory ventilation (HFOV) and nitric oxide (NO) were applied, if necessary, since year 2000(period II). Sixty-seven cases of neonatal Bochdalek hernia from 1989 to 2005 were reviewed retrospectively. There were 33 and 34 cases in period I and II, respectively. The neonatal survival rates were 60.6 % and 73.5 %, respectively, but the difference was not significant. In period I, prematurity, low birth weight, prenatal diagnosis, inborn, and associated anomalies were considered as the significant poor prognostic factors, all of which were converted to nonsignificant in period II. In summary, improved survival was not observed in later period. The factors considered to be significant for poor prognosis were converted to be nonsignificant after change of the management principle. Therefore, we recommend delayed operation after sufficient period of stabilization and the avoidance of the routine insertion of chest tube. The validity of NO and HFOV needs further investigation.

Reactivity Patterns of Various Anti-D Reagents in 14 Cases with Partial D / 대한진단검사의학회지

BACKGROUND: A weak D type resulted from a quantitative reduction of the RhD antigen, whereas a partial D type resulted from a qualitatively altered RhD protein. Based on different serological properties from a weak D type, a partial D type was suspected in cases with anti-D in their serum or if nonreactive to some reagents. Most Red Cross Blood Centers pay attention to donors in determining RhD typing with a monoclonal anti-D reagent. This study examined the reactivity patterns of 4 different monoclonal anti-D reagents in RhD typing and a weak D test in 14 cases with partial D. MATERIALS AND METHODS: We collected a total of 201, 847 samples from blood donors and screened out 649 samples as Rh-negative in RhD typing with monoclonal anti-D (Bioscot) and bromelin treatment applied to an automatic analyzer between October 2002 and March 2003. Further, we performed RhD typing and weak D test using the tube method with 4 commercially available monoclonal anti-D reagents. In 14 cases with different reactivity patterns, we performed a confirming test for partial D using a `ID-partial RhD-typing' (Diamed, Switzerland) set consisting of 6 monoclonal antibodies. RESULTS: Partial D(DFR) was observed in 92.9% (13/14) and a partial D(indeterminate) was observed in 7.1% (1/14). The red blood cells from 14 cases with partial D were not agglutinated with 4 various commercially available anti-D reagents. However, in subsequently performed weak-D tests, different reactivity to their anti-D reagents were shown, namely irresponsiveness (Dade Behring, 14/14, 100%), trace-to-1+ responsiveness (Ortho-clinical diagnostics, 13/14, 92.9%), trace-to-3+ responsiveness (Bioscot, 14/14, 100%), and 1+-to-3+ responsiveness (GreenCross, Korea, 14/14, 100%). CONCLUSIONS: Considering that the most partial D discovered in the Southwestern area of Korea was partial D(DFR), it is recommended that RhD typing and/or weak D tests in blood donors should be done using more than two anti-D reagents from different clones.

Use of Human Skin Reconstructs Containing Melanocytes as a Model to Evaluate the Effect of Pigment Modifiers / 대한피부과학회지

BACKGROUND: Recently human skin reconstructs composed of keratinocytes and melanocytes have been developed. OBJECTIVE: We investigated whether human skin reconstructs containing melanocytes could be useful to test the efficacy of drugs that alter pigmentation. METHODS:Human skin reconstructs were made by culturing human keratinocytes and melanocytes on the top of de-epidermized dermis. Reagents that alter melanogenesis such as isobutyl methylxanthine, 1-oleoyl-2-acetyl-sn-glycerol, kojic acid were treated in the culture medium after 7 days of culture at the air-liquid interface. Macroscopic, histological, histochemical and immunohistochemical studies were performed. RESULTS: The effect of pigment modifiers was demonstrated in human skin reconstructs as expected from in vivo data on skin color, numbers of melanocytes and melanin content. CONCLUSION: These results indicate that this model is very useful to assess the efficacy of drugs that alter pigmentation.

Use of Human Skin Reconstructs Containing Melanocytes as a Model to Evaluate the Effect of Pigment Modifiers / 대한피부과학회지

BACKGROUND: Recently human skin reconstructs composed of keratinocytes and melanocytes have been developed. OBJECTIVE: We investigated whether human skin reconstructs containing melanocytes could be useful to test the efficacy of drugs that alter pigmentation. METHODS:Human skin reconstructs were made by culturing human keratinocytes and melanocytes on the top of de-epidermized dermis. Reagents that alter melanogenesis such as isobutyl methylxanthine, 1-oleoyl-2-acetyl-sn-glycerol, kojic acid were treated in the culture medium after 7 days of culture at the air-liquid interface. Macroscopic, histological, histochemical and immunohistochemical studies were performed. RESULTS: The effect of pigment modifiers was demonstrated in human skin reconstructs as expected from in vivo data on skin color, numbers of melanocytes and melanin content. CONCLUSION: These results indicate that this model is very useful to assess the efficacy of drugs that alter pigmentation.