A 38-year-old woman presented with a week’s history of binocular horizontal double vision and acute vertigo with gaze-induced nystagmus. We considered a diagnosis of one of the six syndromes of the sixth cranial nerve and evaluated several causes. She had history of severe anemia, vitamin B12 deficiency, and hypertension. Magnetic resonance imaging with angiography showed stenosis of the right vertebral artery and hyperintensity on both basal ganglia. As we describe here, we should consider vertebrobasilar insufficiency as a cause for sixth cranial nerve palsy if a patient has high risk for microvascular ischemia, even in the absence of acute brain hemorrhage or infarction.

Axial mesodermal dysplasia complex (AMDC) arises in variable combinations of craniocaudal anomalies such as musculoskeletal deformities, neuroschisis, or rhombencephalic developmental disorders. To the best of our knowledge, the co-existence of AMDC with associated musculoskeletal anomalies, medullary neuroschisis with mirror movements, and cranial nerve anomalies has not yet been reported. Here, we report the case of a 4-year-old boy whose clinical features were suggestive of Goldenhar syndrome and Poland syndrome with Sprengel deformity. Moreover, he showed mirror movements in his hands suspected of rhombencephalic malformation, and infranuclear-type facial nerve palsy of the left side of his face, the opposite side to the facial anomalies of Goldenhar syndrome. After conducting radiological studies, he was diagnosed with medullary neuroschisis without pontine malformations and Klippel-Feil syndrome with rib anomalies. Based on these findings, we propose that clinical AMDC can be accompanied by a wide variety of musculoskeletal defects and variable degrees of central nervous system malformations. Therefore, in addition to detailed physical and neurological examinations, imaging studies should be considered in AMDC.
Central Nervous System ; Child, Preschool ; Congenital Abnormalities ; Cranial Nerves ; Facial Nerve ; Goldenhar Syndrome ; Hand ; Humans ; Klippel-Feil Syndrome ; Male ; Medulla Oblongata ; Mesoderm* ; Neurologic Examination ; Paralysis ; Poland Syndrome ; Rhombencephalon ; Ribs

OBJECTIVE: To investigate whether motor evoked potential (MEP) amplitude ratio measurements are sufficiently objective to assess functional activities of the extremities. We also delineated the distribution between the presence or absence of MEPs and the Medical Research Council (MRC) scale for muscle strength of the extremities. METHODS: We enrolled 183 patients with first-ever unilateral hemiplegia after stroke. The MEP parameters were amplitude ratio (amplitude of affected side/amplitude of unaffected side) recorded at the first dorsal interosseous (FDI) and tibialis anterior (TA) muscles. We performed frequency analyses using the MRC scale for muscle strength and the presence or absence of evoked MEPs. Change on the MRC scale, hand function tests (HFTs), and the Modified Barthel Index (MBI) subscore were compared between the evoked MEP and absent MEP groups using the independent t-test. Receiver operating characteristic curves were used to determine the optimal cutoff scores for the MEP amplitude ratio using the HFT results and MBI subscores. Correlations between the MEP amplitude ratio and the MRC scale, HFTs, and MBI subscore were analyzed. RESULTS: About 10% of patients with MRC scale grades 0-2 showed evoked MEPs at the FDI muscle, and 4% of patients with MRC scale grades 3-5 did not show MEPs. About 18% of patients with MRC scale grades 0-2 showed evoked MEPs at the TA muscle, and 4% of patients with MRC scale grades 3-5 did not show MEPs. MEP amplitude increased with increasing MRC scale grade. The evoked MEP group had more significant changes on the MRC scale, HFT, and the climbing stair score on the MBI than those in the group without MEPs. Larger MEP amplitude ratios were observed in patients who had more difficulty with the HFTs and ambulation. The MEP amplitude ratio was significantly correlated with the MRC scale, HFT, and MBI subscore. CONCLUSION: We conclude that the MEP amplitude ratio may be useful to predict functional status of the extremities in patients who suffered stroke.
Activities of Daily Living ; Evoked Potentials, Motor* ; Extremities* ; Hand ; Hemiplegia ; Humans ; Motor Skills ; Muscle Strength ; Muscles ; ROC Curve ; Stroke* ; Walking

BACKGROUND AND PURPOSE: The occurrence of PWD in neurodegenerative disease is very rare, and this is the first report of it being related to early-onset AD. We describe a patient with early-onset Alzheimer's disease (AD) who presented with pure word deafness (PWD). CASE REPORT: The patient had experienced PWD for 2 years, followed by other cognitive deficits suggestive of parietotemporal dysfunction. Brain imaging including 18FDG-PET and [11C] PIB-PET supported the diagnosis of AD. CONCLUSIONS: Our case highlights the clinical variability that characterizes early-onset AD.
Alzheimer Disease ; Aphasia, Primary Progressive ; Deafness ; Humans ; Neurodegenerative Diseases ; Neuroimaging

Patients with severe neurological deficit, such as hypoxic ischemic injury, cerebral infarction, and traumatic brain injury, often show comatose mental status and require maintenance of long-term tracheostomy for pulmonary toileting. However, several complications, which are mostly related to the cannula, invariably occur. Permanent tracheostoma is a short, skin-lined, noncollapsing, self-sustaining opening by suturing the denuded skin lining to the margin of the tracheal stoma. This tube-free method is a useful alternative to make long-term airway without tube-related complications in chronic diseases, such as obstructive sleep apnea, and laryngeal cancer, however, it has not yet been reported in chronic brain injured patients. This case report illustrates 3 cases of vegetative patients in our rehabilitation clinic who underwent successful procedure of permanent tracheostoma. Permanent tracheostoma has some benefits associated with the free of tube-related complications, and can be considered as a useful alternative way for chronic brain injured patients with long-term tracheostomy.
Brain Injuries/complications/*rehabilitation ; Humans ; Male ; Middle Aged ; Trachea/*surgery ; Tracheostomy/*methods ; Treatment Outcome

No abstract available.
Lipomatosis* ; Neoplasm Metastasis* ; Small Cell Lung Carcinoma*

PURPOSE: This study aimed to test whether rotavirus-associated necrotizing enterocolitis (RV+NEC) produced diffe rent clinical findings or outcomes from those of non-rotavirus necrotizing enterocolitis (RV-NEC). METHODS: Eight patients from the RV+NEC group and 22 patients from the RV-NEC group diagnosed with modified Bell stage II or higher NEC were selected for this study. Fecal specimens from all infants were tested for rotavirus infection using a monoclonal antibody-based enzyme immunoassay (EIA). Clinical, radiographic, and clinical outcome data were analyzed retrospectively. RESULTS: RV+NEC infants had a significantly higher birth weight and were born at a significantly higher gestational age (33.5+/-3.3 weeks vs. 29.3+/-4.4 weeks; P=0.01). There were no differe nces in the occurrence of thrombocytopenia, mural gas, and pneumoperitoneum between the 2 groups. However, portal vein gas was more common in the RV+NEC group (88% vs. 9%; P<0.01). Neither the incidence of Bell stage III (or higher) NEC nor surgical inte rvention differed between the two groups. The number of complications and mortality rates were also similar. CONCLUSION: Rotavirus-associated NEC occurs in infants with a higher birth weight and those born at a greater gestational age. However, the severity of the condition and the resulting outcomes did not differ from those for infants affected by non-rotavirus NEC.
Birth Weight ; Enterocolitis ; Enterocolitis, Necrotizing ; Gestational Age ; Humans ; Immunoenzyme Techniques ; Incidence ; Infant ; Infant, Newborn ; Pneumoperitoneum ; Portal Vein ; Retrospective Studies ; Rotavirus Infections ; Thrombocytopenia

OBJECTIVE: To assess the altered pattern of intracortical excitability of the affected and unaffected hemispheres in stroke patients using paired-pulse transcranial magnetic stimulation (TMS). METHODS: We evaluated intracortical inhibition (ICI) and intracortical facilitation (ICF) in both hemispheres at acute and subacute stages of 103 stroke patients using paired-pulse TMS. The patients were divided into two groups: mild-to-moderate patients whose motor evoked potential (MEP) was recorded in the affected hemisphere; and severe patients whose MEP was not recorded in the affected hemisphere. RESULTS: In mild-to-moderate patients, the value of ICI in the affected hemisphere was increased from 70.3% to 77.9% and the value of ICI in the unaffected hemisphere was decreased from 74.8% to 70.3% with eventual progression in acute to subacute stages of stroke. In severe patients, the value of ICI in the unaffected hemisphere was increased from 65.4% to 75.6%. The changes in ICF were not significantly different in this study. CONCLUSION: We conclude that the unaffected hemisphere was more disinhibited than the affected hemisphere in acute phase of mild-to-moderate stroke, and the affected hemisphere was more disinhibited in the subacute stage. The unaffected hemisphere was inhibited in severe cases in acute-to-subacute phases of stroke. This finding facilitates appropriate neuromodulation of acute-to-subacute phases in mild-to-severe stroke patients.
Evoked Potentials, Motor ; Humans ; Stroke* ; Transcranial Magnetic Stimulation*

Inflammatory pseudotumor (IPT) is a relatively rare and benign disease characterized by chronic inflammatory cell infiltration with fibrosis. It is difficult to diagnose IPT because of the absence of specific symptoms or unique radiological findings. IPT can be mistaken for a malignant tumor, such as cholangiocarcinoma or hepatocellular carcinoma, due to their similar clinical and radiological findings. Unfortunately, hepatic resection is often performed due to misdiagnosis. Presented here is a case of a woman aged over 60 years who presented with general weakness lasting more than one month and unexplained body weight loss.
Body Weight ; Carcinoma, Hepatocellular ; Cholangiocarcinoma ; Diagnostic Errors ; Female ; Fibrosis ; Granuloma, Plasma Cell* ; Humans ; Liver*

Purpose: The aim of this study was to develop an evidence-based guideline for stoma management providing institutional policy, assessment, complications and follow-up care. Methods: The guideline adaptation manual consisting of 23 steps developed by the National Evidence-Based Healthcare Collaborating Agency was used for this study. It presents an overview of the process used to develop the guideline and lists specific recommendations from the guideline. Results: It provides 55 recommendations that include the following 8 topics: 1) Organization and policy recommendations, 2) Preoperative nursing; Ostomy education, stoma site marking, 3) Ostomy formation, 4) Postpoperative nursing; education, assessment, high output stoma management, 5) Selection of ostomy products, 6) Colostomy irrigation, 7) Stomal and peristomal complications, 8) Follow-up care after discharge. Conclusion The guideline can be used to address stoma management in hospital settings. The intent of the guideline is to provide information that will assist healthcare providers to manage adult patients with ostomies, prevent or decrease complications, and improve patients’ outcomes.