Wernicke encephalopathy is usually accompanied with peripheral neuropathy, known as dry beriberi. In contrast, wet beriberi presenting as cardiovascular symptoms rarely occurs. The acute type of wet beriberi can be fatal, if untreated quickly. It is reported that the cerebellar vermis has a role of the coordination and control of cardiovascular and autonomic reflex activities. We report a 58-year-old man showing acute wet beriberi in Wernicke encephalopathy with cerebellar vermis lesion.
Beriberi* ; Cerebellar Vermis ; Humans ; Middle Aged ; Peripheral Nervous System Diseases ; Reflex ; Wernicke Encephalopathy*

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (¹⁸F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in ¹⁸F-FDG PET.
Atrophy ; Brain ; Cerebellar Ataxia ; Cerebellum ; Diagnosis ; Gyrus Cinguli ; Humans ; Neuroimaging ; Positron-Emission Tomography ; Primary Dysautonomias ; Thalamus ; Vestibular Neuronitis

Pneumoperitoneum, Pneumomediastinum, subcutaneous emphysema and a pneumothorax are some of the mechanical complications of bronchial asthma. The incidence of pneumoperitoneum during an attack of acute asthma is rare. The pathogenesis is free gas track from the overdistended alveoli, through the bronchovascular sheaths to the mediastinum. If the high pressure is maintained, air can escape retroperitoneally into the abdomen and burst into the peritoneal cavity. A 43-year-old woman was admitted due to a severe asthma attack. She was required endotracheal intubation and AMBU(air mask bag unit) ventilation. Immediately after these procedures, pneumoperiotneum, pneumomediastinum, and subcutaneous emphysema daveloped. She was treated with mechanical ventilation and medical therapy. The pneumoperitoneum was resolved after 27 days. Here, we report this case with the review of the relevant literature.
Abdomen ; Adult ; Asthma* ; Female ; Humans ; Incidence ; Intubation, Intratracheal ; Masks ; Mediastinal Emphysema* ; Mediastinum ; Peritoneal Cavity ; Pneumoperitoneum* ; Pneumothorax ; Respiration, Artificial ; Subcutaneous Emphysema ; United Nations ; Ventilation*

Focal subarachnoid hemorrhage occasionally presents as transient focal neurologic episodes mimicking transient ischemic attack (TIA). Unless properly diagnosed, it may aggravate cerebral hemorrhage by administering antithrombotic agents. Therefore, clinicians need to be aware that such focal subarachnoid hemorrhage sometimes cannot be detected on noncontrast computed tomography and blood-sensitive magnetic resonance imaging can detect even a small amount of hemorrhage. We describe an 85-year-old woman with focal subarachnoid hemorrhage and possible cerebral amyloid angiopathy who presented transient left arm weakness recurrently, which mimicked TIA.
Aged, 80 and over ; Arm ; Cerebral Amyloid Angiopathy ; Cerebral Hemorrhage ; Female ; Fibrinolytic Agents ; Hemorrhage ; Humans ; Ischemic Attack, Transient ; Magnetic Resonance Imaging ; Subarachnoid Hemorrhage

Secondary intracranial hypertension (IH) is a syndrome with various etiologies, including a brain tumor, head trauma, hypoparathyroidism, hydrocephalus, meningitis, drug use, or venous obstruction. It is reasonably straightforward to diagnose secondary IH induced by these diseases. However, diagnosing secondary IH induced by unpredictable extracranial lesion is difficult, and is based largely on subtle neurological symptoms or signs and usually can be achieved by an extracranial evaluation. Here we describe four cases of secondary IH with unusual causes.
Brain Neoplasms ; Craniocerebral Trauma ; Headache ; Hydrocephalus ; Hypoparathyroidism ; Intracranial Hypertension ; Meningitis ; Spinal Cord Neoplasms

BACKGROUND AND OBJECTIVES: Congenital nystagmus (CN) is an ocular oscillation that usually manifests during early infancy. To report a novel mutation in FERM domain containing 7 (FRMD7) gene in a Korean family with CN. MATERIALS AND METHODS:Genomic DNA was prepared from peripheral blood leukocytes and direct sequencing of the entire coding and adjacent intronic regions was performed to detect sequence variation of FRMD7 gene, where mutations were found recently in patients with familial CN. The family showed an X-linked pattern of inheritance without father-to-son transmission. RESULTS: Three family members with CN exhibited two sequence variations which were a novel mutation (c. 875T>C; Leu292Pro) and a polymorphism (c. 1403G>A; Arg468His, dbSNP rs#6637934). The proband was hemizygous for both variations and his mother and maternal grandmother were heterozygous carriers. CONCLUSION: This study provides an additional evidence for mutations in FRMD7 as a common cause of X-linked CN and expands its mutation spectrum.
Clinical Coding ; DNA ; Humans ; Introns ; Leukocytes ; Mothers ; Nystagmus, Congenital* ; Wills

Percutaneous balloon valvuloplasty (PBV) was done in 7 adults of pulmonary valvular stenosis, which was diagnosed with catheterization and cine-angiography of rigtht heart, in Seoul National University Hospital from Jan. 1986 to Dec. 1986. The results were as follows : 1) Among the seven cases six were male and the range of age was from 19 to 40 years. Atrial septal defects associated in 2 cases were not significant clinically. 2) The peak systolic gradient from right ventricle to pulmonary artery before the procedure was below 50 mmhg in 1 cases, 50 to 100 mmHg in 1 case, and above 100 mmHg in 5 cases. 3) Before PBV the mean systolic pressure of right ventricle was 121+/-42 mmHg and the peak systolic gradient was 106+/-41 mmHg. After PBV those were 58+/-30 mmHg and 40+/-31 mmHg, revealing significant improvement. 4) Balloon catheter (Medi-tech, 9F) was used in all cases. single balloon of 20 mm outer diameter was applied in 2 cases and double balloons of various sizes were applied in 5 cases in which single balloon showed inadequate dilatation. It is suggested that percutaneous balloon valvuloplasty is safe and effective alternative to surgical valvotomy of pulmonary valvular stenosis in adults.
Adult* ; Balloon Valvuloplasty* ; Blood Pressure ; Catheterization ; Catheters ; Constriction, Pathologic* ; Dilatation ; Heart ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Male ; Pulmonary Artery ; Seoul

BACKGROUND: Paroxysmal atrial fibrillation (PAF) causes not only severe symptoms and hemodynamic changes, but may progress to chronic atrial fibrillation. Autonomic nervous system or atrial premature beat (APB) has been suggested to contribute to the spontaneous initiation of PAF, but the exact mechanism has been largely unknown. METHODS: One hundred and twenty nine episodes of PAF lasting longer than 5 sec were analyzed in 18 patients (M:F=11:?). Two minutes of normal sinus rhythm before the onset of PAF, and the initial one minute of PAF were printed and analyzed. RESULTS: Most of PAFs were initiated by APBs (38%) or rapid atrial tachycardias (AT, 59%). The frequency of APBs tended to increase immediately before PAF onset (p=0.08). The coupling intervals and coupling indices were not significantly different between PAF-producing APBs and benign APBs. More than half of PAF episodes were initiated by rapid ATs (rate, 357+/-50 bpm). After the onset, they accelerated over several seconds and then degenerated into AF. In some cases, transition from AF to atrial flutter and vice versa were observed. Heart rate, measured at 60-second intervals during 2 minutes before PAF onset, did not change significantly (p=0.44). CONCLUSION: Most of PAFs were initiated by APBs or rapid ATs. Heart rate did not change significantly but the frequency of APBs tended to increase immediately before PAF onset. Rapid ATs frequently accelerated and degenerated into AF. In this regard, Holter monitoring could be useful in identifying patients with PAF triggered by rapid ATs.
Atrial Fibrillation* ; Atrial Flutter ; Autonomic Nervous System ; Cardiac Complexes, Premature ; Electrocardiography, Ambulatory* ; Heart Rate ; Hemodynamics ; Humans ; Tachycardia

This study was conducted to evaluate the influence of the C-factor on the bond strength of a 6th generation self-etching system by measuring the microtensile bond strength of four types of restorations classified by different C-factors with an identical depth of dentin. Eighty human molars were divided into four experimental groups, each of which had a C-factor of 0.25, 2, 3 or 4. Each group was then further divided into four subgroups based on the adhesive and composite resin used. The adhesives used for this study were AQ Bond Plus (Sun Medical, Japan) and Xeno III (DENTSPLY, Germany). And composite resins used were Fantasista (Sun Medical, Japan) and Ceram-X mono (DENTSPLY, Germany). The results were then analyzed using one-way ANOVA, a Tukey's test, and a Pearson's correlation test and were as follows. 1. There was no significant difference among C-factor groups with the exception of groups of Xeno III and Ceram-X mono (p < 0.05). 2. There was no significant difference between any of the adhesives and composite resins in groups with C-factor 0.25, 2 and 4. 3. There was no correlation between the change in C-factor and microtensile bond strength in the Fantasista groups. It was concluded that the C-factor of cavities does not have a significant effect on the microtensile bond strength of the restorations when cavities of the same depth of dentin are restored using composite resin in conjunction with the 6th generation self-etching system.
Adhesives ; Composite Resins ; Dentin ; Dentin-Bonding Agents ; Humans ; Molar

The balloon dilatation or Savary-Gilliard dilatation was performed in 59 patients with benign esophageal stricture in Chungnam National University Hospital from September 1990 to August 1995. We reviewed the effect and the safty of each therapeutic method and the results were as foillows: 1) The cause of stricture were corrosive stricture(28 cases, 49,4%), anastomotic stenosis after gastroesophageal surgery(26 cases, 44.1%), stricture after endoscopic variceal sclerotherapy(3 cases, 5.1%), esophageal web(1 case, 1.7%) or stricture complicated by reflux esophagitis(l case). 2) The overall cure rate of balloon dilatation was 50%(l2/24 cases) and that of Savary-Gilliard dilatation was 77.1%(27/35 cases). The Savary-Gilliard dilatation group had a better result than the balloon group. 3) The perforation after Savary-Gilliard dilatation occurred in 4 cases~(6.7%). One case was treated surgically and three cases wiere treated medically. But there was no fatal complication. 4) The overall cure rate of anastomotic stenosis was 84.6%(32/38 cases) and that of corrosive stricture was 46.4%(13/28 cases). 5) The cure rate of Savary-Gilliard dilatation in corrosive esophageal stricture was 64.7%(ll/17) and that of balloon dilatation was 18.1%(2/11). 6) According to site of stricture, the cure rate of dilatation was 84.2% (32/38) in thoracic esophagus, 42.8%(3/7) in cervical esophagus and 28,5%(4/14) in multiple or long segmented stricture.(continue...)
Chungcheongnam-do ; Constriction, Pathologic ; Dilatation* ; Esophageal Stenosis* ; Esophagus ; Humans