BACKGROUND AND PURPOSE: Recently published, evidence-based guidelines should alter the management of amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). However, the newest recommendations for ALS/MND therapy are not reflected in actual clinical practice. We sought to evaluate the current status of the diagnosis and management of ALS in Korea. METHODS: The Korean ALS/MND research group was organized in 2010, involving more than 50 neurologists from neuromuscular centers in Korea. Participating centers collected data from April to September 2010 on the diagnosis and management of patients with ALS. Data forms from the ALS patient care database, which is a component of the ALS clinical assessment, research, and education program (http://www.outcomes-umassmed.org/ALS/), were modified and used for data collection. RESULTS: In total, 373 sporadic ALS cases from 35 centers were enrolled. The demographic features and clinical findings were similar to those in previous reports from other countries. The mean age at onset was 50-60 years, and a slight male predominance was observed. The enrolled patients predominantly showed focal onset of cervical or lumbosacral symptoms. Only about one-half of the indicated patients (31.4%) received a physician's recommendation for a parenteral gastrostomy, and 18.1% underwent the procedure. Noninvasive ventilation was recommended in 23% of patients, but applied in only 9.5% of them. Tracheostomy was performed in 12.7% of patients. CONCLUSIONS: The demographic and clinical features of the diagnosis and management of ALS in Korea are similar to those reported in other countries; however, supportive management, as recommended in evidence-based guidelines, are not yet widely recommended or performed for patients with ALS in Korea.
Amyotrophic Lateral Sclerosis ; Cross-Sectional Studies ; Gastrostomy ; Humans ; Korea ; Male ; Neurons ; Noninvasive Ventilation ; Palliative Care ; Patient Care ; Tracheostomy

Objective: : Given the high risk of rebleeding and recurrence of blood blister-like aneurysms (BBAs), we treated ruptured BBAs of the internal carotid artery (ICA) with stent-assisted coil embolization (SAC). This study aimed to evaluate the efficacy and safety of SACs. Methods: : We retrospectively reviewed clinical and radiological data from eight patients with ruptured BBAs of the supraclinoid ICA. The modified Rankin Scale (mRS) was used to assess clinical outcomes, while radiological outcomes were evaluated on angiographs. For a pooled analysis, data from literature reporting the outcomes of ruptured BBAs treated with SAC were collected and analyzed in conjunction with our data. Results: : In our cohort, the mean Raymond classification score was 1.57±0.53 immediately after initial endovascular treatment. There were no perioperative complications or rebleeding events during the follow-up period. The mean mRS score at patient discharge was 1.00±0.81 and improved to 0.28±0.48 by the last follow-up day. The recurrence rate was 25% with an asymptomatic presentation and successful treatment with multiple stent insertion. Pooled analysis of 76 cases of SAC revealed a complete occlusion rate immediately after treatment of 54.8%, rebleeding rate 7.94%, and recurrence rate 24.2%. Good clinical outcomes with mRS score 0–2 were observed in 89.9% by the last clinical follow-up. Total mortality rate was 7.7%. Conclusion : This treatment appears to not only minimize the hemodynamic burden on the fragile dome specific to this type of aneurysm, but also provides an opportunity for safe and effective treatment in recurrent cases.

Objective: : The etiology of angiographically occult spontaneous subarachnoid hemorrhage (AOsSAH) is unclear. Threedimensional (3D) high-resolution vessel wall magnetic resonance imaging (HVM) might be useful in detecting the hidden arterial wall angiopathy in patients with AOsSAH. We aimed to demonstrate the feasibility of HVM for detecting the arterial cause of AOsSAH. Methods: : Patients, who were diagnosed with AOsSAH in the first evaluations and underwent HVM, were enrolled. Their clinical and radiologic data were retrospectively reviewed. Especially, focal enhancement of arterial wall on HVM and repetitive catheterized angiograms were precisely compared. Results: : Among 251 patients with spontaneous SAH, 22 patients were diagnosed with AOsSAH in the first evaluations (8.76%). After excluding three patients who did not undergo 3D-HVM, 19 patients were enrolled and classified as convexal (n=2) or perimesencephalic (n=4), and diffuse (n=13) groups. In convexal and perimesencephalic groups, no focal enhancement on HVM and no positive findings on repetitive angiography were noted. In diffuse group, 10 patients showed focal enhancement of arterial wall on HVM (10/13, 76.9%). Repeated angiography with 3D reconstruction revealed four patients of angiographically positive causative arteriopathy and possible lesion in one case in the concordant location of intramural enhancement on 3D-HVM (5/10, 50%). Three of them were treated with endovascular stent insertion. All patients, except one, recovered with good clinical outcome (3-month modified Rankin score, 0 and 1). Conclusion : 3D-HVM was useful in detecting hidden true arteriopathy in AOsSAH. It may provide new insights into the etiologic investigation of AOsSAH by proving information about the arterial wall status.

OBJECTIVE: The feasibility and usefulness of transradial catheterization for coronary and neuro-intervention are well known. However, the anatomical change in the catheterized radial artery (RA) is not well understood. Herein, we present the results of ultrasonographic observation of the RA after routine transradial cerebral angiography (TRCA).METHODS: Patients who underwent routine TRCA with pre- and post-procedure Doppler ultrasonography (DUS) of the catheterized RA were enrolled. We then recorded and retrospectively reviewed the diameter and any complicated features of the RA observed on DUS, and the factors associated with the diameter and complications were analyzed.RESULTS: A total of 223 TRCAs across 181 patients were enrolled in the current study. The mean RA diameter was 2.48 mm and was positively correlated with male gender (p < 0.001) and hypertension (p < 0.002). The median change in diameter after TRCA was less than 0.1 mm (range, -1.3 to 1.2 mm) and 90% of changes were between -0.8 and +0.7 mm. Across 228 procedures, there were 12 cases (5.3%) of intimal hyperplasia and 22 cases (9.6%) of asymptomatic local vascular complications found on DUS. Patients with abnormal findings on the first procedure had a smaller pre-procedural RA diameter than that of patients without findings (2.26 vs. 2.53 mm, p=0.0028). There was no significant difference in the incidence of abnormal findings for the first versus subsequent procedures (p=0.68).CONCLUSION: DUS identified the pre- and post-procedural diameter and local complications of RA. Routine TRCA seems to be acceptable with regard to identifying local complications and changes in RA diameter.
Catheterization ; Catheters ; Cerebral Angiography ; Follow-Up Studies ; Humans ; Hyperplasia ; Hypertension ; Incidence ; Male ; Radial Artery ; Retrospective Studies ; Ultrasonography ; Ultrasonography, Doppler