Quantitative Evaluation of Post-stroke Spasticity Using Neurophysiological and Radiological Tools: A Pilot Study

OBJECTIVE: To determine the possibility of a new measurement tool using electromyography and ultrasonography for quantitative spasticity assessment in post-stroke patients. METHODS: Eight hemiplegic stroke patients with ankle plantarflexor spasticity confirmed by a Modified Ashworth Scale (MAS) were enrolled. Spasticity was evaluated using the MAS and Modified Tardieu Scale (MTS). Each subject underwent surface electromyography (sEMG) using the Brain Motor Control Assessment (BMCA) protocol and was compared with a healthy control group. Using ultrasonography, muscle architecture and elasticity index were measured from the medial gastrocnemius muscle (GCM) on the affected and unaffected sides. RESULTS: MAS and MTS revealed significant correlation with sEMG activity. The fascicle length and pennation angle were significantly decreased in the medial GCM on the hemiplegic side compared with the unaffected side. The elasticity index of the spastic medial GCM was significantly increased compared with the unaffected side. The MTS X and R2–R1 values were significantly correlated with the elasticity index in the hemiplegic GCM. The relationship between clinical evaluation tools and both BMCA and sonoelastography was linear, but not statistically significant in the multiple regression analysis. CONCLUSION: The BMCA protocol and ultrasonographic evaluation provide objective assessment of post-stroke spasticity. Further studies are necessary to conduct accurate assessment and treatment of spasticity.

Identifying the KAT6B Mutation via Diagnostic Exome Sequencing to Diagnose Say-Barber-Biesecker-Young-Simpson Syndrome in Three Generations of a Family

Diagnostic exome sequencing (DES) is a powerful tool to analyze the pathogenic variants leading to development delay (DD) and intellectual disability (ID). Recently, heterozygous de novo mutation of the histone acetyltransferase encoding gene KAT6B has been recognized as causing a syndrome with congenital anomalies and intellectual disability, namely Say-Barber-Biesecker-Young-Simpson (SBBYS) syndrome. Here we report a case of SBBYS syndrome in a third generation Korean family affected with a missense mutation in KAT6B, c.2292C>T p.(His767Tyr) identified by DES. This is the first confirmed familial inherited mutation of the KAT6B reported worldwide. Our case emphasizes again the importance of basic physical examination and taking a family history. Furthermore, advances in genetic diagnostic tools are becoming key to identifying the etiology of DD and ID. This allows a physiatrist to predict the disease's clinical evolution with relative certainty, and offer an appropriate rehabilitation plan for patients.

Analysis of Prognostic Factors for Return of Spontaneous Circulation and Survival in Elderly Patients with Cardiac Arrest

BACKGROUND: Recently the incidence of cardiac arrest in the elderly has been on the rise due to aging and the rapid increase in cardiovascular disease. Nevertheless, there has been only a few studies done regarding the factors affecting return of spontaneous circulation (ROSC) and survival in this population within Korea. We investigated the prognostic factors for ROSC and survival in cardiac arrest patients over 65 years visiting a single local emergency center. METHODS: We conducted a single center retrospective observational study, and 87 elderly patients with cardiac arrest were enrolled. They visited the emergency medical care center via ambulance from November 2013 to October 2014. Primary outcomes were ROSC and survival and the secondary outcome was 100 days cumulative survival rate. RESULTS: The level of potassium was the only significant factor for ROSC. The increase in potassium level reduced the rate of ROSC (odds ratio, 0.64; 95% confidence interval [CI], 0.44-0.92; p=0.01). However, higher potassium level was not related to survival (p=1.00). For the 100 days cumulative survival rate, neither hypokalemia (hazard ratio [HR], 1.1; 95% CI, 0.57-2.38; p=0.66) nor hyperkalemia (HR, 1.5; 95% CI, 0.89-2.59; p=0.11) was related to survival compared to normokalemia. CONCLUSION: The potassium level may be considered a valid prognostic factor for ROSC in elderly patients with cardiac arrest. However, it was unrelated to survival.

CD25 Monoclonal Antibody (Basiliximab) Used in High Risk Kidney Recipient (Single Center Experience) / 대한이식학회지

PURPOSE: CD25 monoclonal antibody (basiliximab, BA) has been reported an excellent effect on induction immunosuppression than ALG, ATG, OKT3 in renal transplantation. Since we can use BA only in the group of high risk kidney recipient, we want to evaluate the effect of BA treated group by comparing with conventional 3 drugs combination group (calcineurin inhibitor/cyclosporine or tacrolimus, mycophenolate mofetil and prednisolon). METHODS: Total 103 recipients were treated by BA and conventional 3 drugs from March 2000 through February 2006, and these cases were compared with 122 recipients without BA. Government guidelines for using BA were in cadaveric transplantation, more than 4 HLA mismatching, zero DR antigen matching, retransplantation and more than 80% positive PRA. The episode of acute rejection (AR), steriod resistant acute rejection, change of serum creatinine, maintaining dosage of calcineurin inhibitor (CNI) and other side effects were compared between groups. RESULTS: The rate of AR within 12 months after transplantation was 11.7% in BA group while 9.0% in control group (P=0.451). The steroid resistant acute rejection was 16.6% in BA group and 27.3% in control group (P=0.119). Rejection free graft survival adjusted various clinical risk factors by Cox-regression test were no significance between groups. Serum creatinine level at one year after transplantation was 1.61+/-.1 and 1.46+/-.7 mg/dL in BA and control group, and recipients number whose SCr over 1.5 mg/dL were 39.0% and 32.7% (P=0.326). Cyclosporin dosage at one year in BA and control group were 4.21 and 3.62 mg/kg (P=0.202) and 0.11 and 0.17 mg/kg in tacrolimus group (P=0.291). Incidence of PTDM and viral infection were all no difference statistically between groups. CONCLUSION: In conclusion, BA induction immunosuppression with CNI, mycophenolate mofetil and prednisolon used in high risk kidney recipient effectively control the acute rejection and steroid resistant acute rejection for one year at least the same as control group. But since there was no more beneficial effect in BA added to Tac group than conventional Tac based immunosuppression, this 4 drug combination is better avoided if possible.

A Case of Combined Hepatocellular-Cholangiocarcinoma with Sarcomatous Transformation and Second Primary Colon Cancer / 대한간학회지

Combined hepatocellular-cholangiocarcinoma is a rare form of primary liver cancer, featuring both hepatocellular and biliary epithelial differentiations. An intrahepatic tumor may be considered as a metastatic lesion. It has been suggested in the literature that the likelihood of metastasis in the cirrhotic liver is lower than that in the non-cirrhotic liver. A rare case of combined hepatocellular-cholangiocarcinoma and second primary colon adenocarcinoma in a 67-year-old male patient with liver cirrhosis is presented. Histologically, the intrahepatic mass was composed of a spindle cell sarcomatous component; a hepatocellular carcinoma component; and a cholangiocarcinoma component. There were focal transitional regions among the different components. Immunohistochemically, the cholangiocarcinoma component of the intrahepatic mass showed positive reactions for CK-7 but negative reactions for CK-20. The adenocarcinoma of the colon showed positive reactions for CK-20 but negative reactions for CK-7.

Risk Factors for Chronic Rejection in Renal Allograft Recipients / 대한이식학회지

Chronic rejection is a major barrier to long-term renal allograft survival. Cyclosporine, though effective at reducing the graft loss due to acute rejection, has had little impact on the incidence of chronic rejection. Between December 1984 and April 1995, 221 patients received a primary living donor kidney transplantation. In our study, 154 patients(70%) never had an episode of acute rejection, 22% had only one, and 8% had more the one. The incidence of chronic rejection was 12% in those who had no acute rejection, 39% in those with one or more rejection episode. The 5-year graft survival was 28% for recipients with biopsy-proven chronic rejection versus 83% for without(Por=4 mg/kg) were significant risk factors for chronic rejection. In a logistic regression of multivariate analysis, we found that risk factors for chronic rejection were acute rejection episode(P<0.0001) and donor age(P<0.01).

A Case of Oguchi's Disease

Oguchi's disease is an unusual form of congenital stationary night blindness that is characterized by a peculiar grey-white discoloration of the retina that gives a metallic sheen. After prolonged dark adaptation, the unusual fundus discoloration disappeared and the retina slowly reverted to its original metallic color after exposure to the light. A 20-year-old man was referred for study because of a complaint of night blindness since childhood. As results of diagnostic work up, especially based on typical ocular fundus finding, dark adaptometry and electrophysiologic studies of the retina the authors have diagnosed as a Oguchi's disease. We report this case with the review of the literature.

Changes in ERG b-wave and Oscillatory Potential in Relation to the Dark Adaptation and Light Adaptation Time

Authors studied characteristics of ERG b-wave and oscillatory potential, which can be applied to ERG protocol for clinical purposes. Following conclusions were obtained from the ERG test performed on 20 normal men(40 eyes) during dark adaptation and light adaptation time, under the conditions where other factors were equal. 1. In the scotopic ERG test, 88%(364/414uv) of maximum b-wave amplitude in 30 minutes were obtained in first 1 minute of dark adaptation. By using this shortening scotopic protocol for the acquired retinal disease, sufficient result can be expected without the over 20 minutes dark adaptation. 2. In the photopic ERG test, if over 30 minutes dark adaptation time were given, it would take at least 15 minutes to reach the regular initial photopic value. Therefore, it is recommended that photopic ERG test be done before the scotopic ERG test. 3. In the oscillatory potential test, light adaptation time has little effect on summed photopic oscillatory potential amplitudes and implicit time. Summed scoto pic oscillatory potential amplitude reaches the highest 1 minute after the dark adaptation. Therefore, it is recommended that to obtain higher amplitude and more sensitive test result, oscillatory potential test be done 1 minute after the dark adaptation.

VEP Change in Refractive Errors

In present, clinically, the VEP has special value in the areas of refraction, infant acuity, diseases of the optic nerve, color blindness, amblyopia and field defects. VEP reflects the the activity of the visual system from the level of the photoreceptors to the occipital cortex, and it is more directly related to vision than retinoscopy. Authors studied the YEP change in refractive errors. We used Horizon computer with UTAS-E and the check sizes used were 16 X 16(50 min.), 32 X 32(25 min.), 64 X 64(12.5 min). One eye was occluded and then lenses of different power were successively placed before the other eye, and a seperate VEP was recorded for each lens power. The result were as follows: 1. Amplitude change according to change of check size in different trial lens powers. 1) +3, +4, +5D (64 X 64): statistically significant decrease. 2) -D(except -6D, 32 X 32): statistically not significant. 2. Latency change according to change of check size in different trial lens powers. 1) +D(64 X 64 and +4, +5D(32 X 32): statistically significant increase. 2) -D(64 X 64) and -10D(32 X 32): statistically significant increase. 3. Amplitude change according to change of trial lens power in different check sizes. 1) All check size(+4, +5D) and 64 X 64 size(+3D): statistically significant decrease. 2) All check size(-D): statistically significant decrease. 4. Latency change according to change of trial lens power in different check sizes. 1) 32 X 32 size(+4, +5D) and 64 X 64size(+3, +4, +5D): statistically significant increase. 2) 16 X 16 size(-6, -8, -10D) 32X32 size(-4, -6. -8, -10D) and 64X64 size (all D): statistically significant increase. 5. Change of amplitude and latency between each trial lens power in different check sizes. 1) +D(64 X 64), amplitude and Latency: statistically significant. 2) -D amplitude-all check size: statistically significant, latoncy-32 X 32 size: statistically significant.