Arnold-Chiari Malformation Type III With Meningoencephalocele: A Case Report

Arnold-Chiari malformation type III (CM III) is an extremely rare anomaly with poor prognosis. An encephalocele with brain anomalies as seen in CM II, and herniation of posterior fossa contents like the cerebellum are found in CM III. The female infant was a twin, born at 33 weeks, weighing 1.7 kg with a huge hydrocele on the craniocervical junction. After operations were performed, she was referred to the department of rehabilitation medicine for poor motor development, swallowing dysfunction, and poor eye fixation at 22 months. The child was managed with neurodevelopmental treatment, oromotor facilitation, and light perception training. After 14 months, improvement of gross motor function was observed, including more stable head control, rolling, and improvement of visual perception. CM III has been known as a condition with poor prognosis. However, with the improvement in operative techniques and intensive rehabilitations, the prognosis is more promising than ever before. Therefore, more attention must be paid to the rehabilitation issues concerning patients with CM III.

The Quantitative Relationship Between Physical Examinations and the Nerve Conduction of the Carpal Tunnel Syndrome in Patients With and Without a Diabetic Polyneuropathy

OBJECTIVE: To understand the quantitative correlation between the clinical severity and physical examinations along with the electrodiagnostic findings by subjects with carpal tunnel syndrome (CTS) and also the influence of diabetic polyneuropathy (DPN) on physical examinations by subjects with CTS. METHODS: Among 200 patients suffering from hand tingling sensations, 68 patients were diagnosed with CTS on at least one hand by nerve conduction tests. Therefore, the Phalen test (PT), hand elevation test (HET), Tinel sign (TS) results were recorded on both hands. The physical examination grades were compared with the electrophysiological CTS grades in 126 hands of 68 patients. Also the comorbidity effect of DPN to CTS was evaluated. For the evaluation of the severity correlations between CTS, PT, HET, and TS, the Spearman analysis was used. An attempt was started to create a formula which could depict the electrophysiological severity of CTS. RESULTS: Out of the 68 tested subjects, 31 were diagnosed with both DPN and CTS, and 37 with CTS only. Both PT and HET correlated well with the severity of CTS where the correlation of PT was higher than that of HET. The formula were the motor distal latency (MDL)=(72.4-PT)/5.3 and MDL=(76-HET)/7.2. Both PT and HET showed in the presence of DPN a relatively higher relation with CTS without significance. CONCLUSION: PT and HET would be useful screening tools for the diagnosis and treatment of CTS as the grade of PT and HET present the severity of CTS well. During this study, a formula was created expecting the severity of nerve conduction study with PT and HET through the time domain value of physical examinations.

Effects of Night Sleep on Motor Learning Using Transcranial Magnetic Stimulation

OBJECTIVE: To investigate the effects of night sleep on motor cortical excitability with TMS (transcranial magnetic stimulation) and finger tapping performance. METHOD: Eight volunteers were enrolled to investigate the effects of day wake or night sleep on motor learning and finger performance. Each subject underwent a finger tapping task over a 12 hour period, which was employed to evaluate the motor cortical excitability affected by motor learning. Starting at 9:00 am for the day wake cycle and restarting at 9:00 pm for the night sleep cycle. The finger tapping task was the index finger of the non-dominant hand with the Hangul word personal computer (PC) training program. The data was assessed by comparing the changes observed with the cortical excitability and finger tapping performance tests between the day wake and night sleep after equivalent amounts of training. RESULTS: The results showed that in paired-pulse techniques, there was a significant decrease of intracortical inhibition (ICI) in the morning following the night sleep cycle (p<0.05), but no significant change was seen in the ICI in the evening for the day wake cycle. In addition a significant decrease of the ICI was observed in comparison to the morning following the night sleep cycle and the evening following the day wake cycle (p<0.05). The 140% recruitment curve (RC) and accuracy of the finger tapping performance demonstrated a significant improvement for both cycles (p<0.05). CONCLUSION: Through this study, we observed that the Hangul typing practice requires both explicit and implicit skill learning. And also the off-line learning during a night of sleep may be affected by an inhibitory neurotransmitter related synaptic plasticity and by the time dependent learning with recruitments of remote or less excitable motor neurons in the primary motor cortex.

Clinical Characteristics and Prognostic Factors of Patients with Severe Pulmonary Hypertension

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate the clinical characteristics and predictors of the prognosis for patients with severe pulmonary hypertension (PHT). SUBJECTS AND METHODS: A total of 169 patients with severe PHT (systolic pulmonary arterial pressure > or =70 mmHg by Doppler echocardiogram) were divided into two groups according to the mortality during 2.8+/-2.2 year clinical follow-up period: group I (moribund group, 34 patients, 60.0+/-15.7 years, 18 males) and group II (survived group, 135 patients, 57.2+/-16.9 years, 65 males). The clinical, electrocardiographic and echocardiographic variables were analyzed. RESULTS: The three most common etiologies were valvular heart disease (VHD)(n=55: 32.9%), pulmonary thromboembolism (n=29: 17.2%) and ischemic cardiomyopathy (n=17: 10.1%). The common presenting clinical symptoms were dyspnea on exertion, leg edema, cough and chest discomfort. Among the variables, the WHO functional classification at presentation [95% confidence interval (CI) 2.065-221.168, p=0.010], atrial fibrillation (AF)(95% CI 1.014-10.161, p=0.041) and the left ventricular ejection fraction (LVEF)(95% CI 1.011-4.545, p=0.047) were significantly associated with the prognosis. CONSLUSION: VHD was the most common cause of severe PHT. The WHO functional classification at presentation, the presence of AF and low LVEF at presentation were the significant predictors of mortality for patients with severe PHT.

Effects of Oxygen Free Radical on Action Potential in Mouse Atrial Myocardium

BACKGROUND AND OBJECTIVES: Reactive oxygen species are known to be produced when atrial fibrillation develops. This study was performed to investigate the effects of hydrogen peroxide (H2O2) on the action potential parameters of the mouse atrium. SUBJECTS AND METHODS: Mouse (ICR) atrial fibers were excised and immersed in cold bicarbonate-containing Tyrode's solution. The preparations were then perfused with oxygenated (95% O2, 5% CO2) Tyrode's solution and driven by an electrical stimuli 1 ms in duration at a frequency of 1 Hz. The transmembrane potentials were recorded at 0, 2.5, 5, 10, 20 and 30 minute, and compared between groups I (control), II (H2O2 0.1 mM), III (H2O2 0.5 mM) and IV (H2O2 1 mM). RESULTS: In group I, the maximal diastolic potential (MDP), action potential amplitude (APA), maximal slope at phase 0 depolarization (Vmax), action potential duration until 50% and 90% of repolarization (APD50, APD90) were unchanged with increasing time. In group II, the MDP and APA were unchanged, but the Vmax was decreased, and the APD50 and APD90 prolonged. In group III, the MDP was increased and the Vmax decreased; the APD50 and APD90 were prolonged, but the APA unchanged. In group IV, the MDP was increased, the Vmax and APA decreased And the APD50 and APD90 prolonged. After-depolarization was observed in 40% (8/20) and 54.5% (12/22) of groups III and IV, respectively, and asystole occurred in 18.2% (4/22) of group IV. CONCLUSION: Hydrogen peroxide changed the action potential parameters in both time and dose dependent manner, and also elicited after-depolarization at higher concentrations. These results suggest reactive oxygen species are involved in the electrical remodeling and arrhythmogenesis in atrial myocardium.

Influence of Vasoactive Substances on the Regulation of Cardiac ATP-Sensitive Potassium Channel Activity

BACKGROUND AND OBJECTIVES: It has been known that various vasoactive agents are involved in the regulation of cardiac function through the modification of the K+ channel activities, including the ATP-sensitive K+ channel (KATP). We examined the effects of several vasoactive agents on the cardiac KATP currents in isolated cardiac myocytes. MATERIALS AND METHODS: Ventricular myocytes were isolated from the hearts of ICR mice by enzymatic digestion. The channel currents were recorded by the excised inside-out and cell-attached patch clamp configurations. RESULTS: In the excised inside-out patches, bradykinin (BRK; 1-10 micrometer) and prostaglandin I2 (PGI; 10-50 micrometer) did not affect the channel activities, whereas the vasodilators increased the attenuated channel activities in the presence of 100 micrometer ATP. BRK and PGI in parallel shifted the dose-response curves of ATP (1-1,000 micrometer), and this inhibited the KATP currents to the right. Endothelin (ET-1; 0.1-1 nM) and leukotriene D4 (LTD; 3-10 micrometer) decreased the channel activities immediately after making the inside-out patches. However, the vasoconstrictors did not affect the attenuated channel activities by ATP. In the cell-attached patches, both BRK and PGI increased the channel activities and these effects were markedly attenuated by glibenclamide (50 micrometer). ET-1 and LTD did not affect the baseline channel activities in the cell-attached patches, but they markedly attenuated the dinitrophenol-induced activities. CONCLUSION: It was inferred that certain vasoactive substances are involved in the regulation of cardiac KATP channel activities, and that bradykinin and PGI2 enhance the channel activities, and ET-1 and LTD4 inhibit the channel activities.