Non-motor symptoms in patients with hereditary spastic paraplegia caused by SPG4 mutations.

BACKGROUND AND PURPOSE: Non-motor manifestations are frequently overlooked in degenerative disorders and little is known about their frequency and clinical relevance in SPG4 hereditary spastic paraplegia (SPG4-HSP). METHODS: Thirty patients with SPG4-HSP and 30 healthy controls answered the Modified Fatigue Impact Scale, Epworth Sleepiness Scale, Brief Pain Inventory and Beck Depression Inventory. Student's t test was used to compare groups and linear regression was used to assess correlations. RESULTS: Patients had higher fatigue scores than controls (31.0 ± 16.5 vs. 14.5 ± 16.0, P = 0.002) as well as pain (3.4 ± 2.7 vs. 1.0 ± 1.6, P = 0.001) and depression (12.7 ± 8.9 vs. 4.4 ± 3.8, P < 0.001, respectively). Fatigue was associated with depression and possibly with disease severity (P = 0.008 and 0.07, respectively). CONCLUSIONS: Fatigue, pain and depression are frequent and often severe manifestations in patients with SPG4-HSP.

Clinical evaluation of eye movements in spinocerebellar ataxias: a prospective multicenter study.

BACKGROUND: Ocular motor abnormalities reflect the varied neuropathology of spinocerebellar ataxias (SCAs) and may serve to clinically distinguish the different SCAs. We analyzed the various eye movement abnormalities detected prospectively at the baseline visit during a large multicenter natural history study of SCAs 1, 2, 3, and 6. METHODS: The data were prospectively collected from 12 centers in the United States in patients with SCAs 1, 2, 3, and 6, as part of the Clinical Research Consortium for Spinocerebellar Ataxias (NIH-CRC-SCA). Patient characteristics, ataxia rating scales, the Unified Huntington Disease Rating Scale functional examination, and clinical staging were used. Eye movement abnormalities including nystagmus, disorders of saccades and pursuit, and ophthalmoparesis were recorded, and factors influencing their occurrence were examined. RESULTS: A total of 301 patients participated in this study, including 52 patients with SCA 1, 64 with SCA 2, 117 with SCA 3, and 68 with SCA 6. Although no specific ocular motor abnormality was pathognomonic to any SCA, significant differences were noted in their occurrence among different disorders. SCA 6 was characterized by frequent occurrence of nystagmus and abnormal pursuit and rarity of slow saccades and ophthalmoparesis and SCA 2 by the frequent occurrence of slow saccades and infrequent nystagmus and dysmetric saccades. SCA 1 and SCA 3 subjects had a more even distribution of eye movement abnormalities. CONCLUSIONS: Prospective data from a large cohort of patients with SCAs 1, 2, 3, and 6 provide statistical validation that the SCAs exhibit distinct eye movement abnormalities that are useful in identifying the genotypes. Many of the abnormalities correlate with greater disease severity measures.

Early and intermediate term clinical outcome after multiple coronary stenting.

OBJECTIVE: To examine the immediate and intermediate term clinical outcome of multiple coronary stenting. DESIGN: Consecutive patients were prospectively entered on a dedicated database. Follow up information was obtained from outpatient and telephone interviews with patients and family physicians. SETTING: A tertiary referral centre. PATIENTS: 140 consecutive patients underwent multiple coronary stenting between April 1994 and November 1996. Most patients had unstable coronary syndromes. MAIN OUTCOME MEASURES: Death, cerebrovascular accidents, myocardial infarction (MI), coronary artery bypass surgery (CABG), and repeat angioplasty (PTCA). RESULTS: The angiographic success rate was 100% and the clinical procedural success rate 93%. The mean (SD) follow up was 11.9 (7.2) months (range 2-32). The mean (SD) number of stents per patient was 2.4 (0.7). The mean (SD) number of lesions treated per patient was 1.4 (0.6). There were four in-hospital deaths (2.9%) and five patients (3.6%) had an MI before hospital discharge. All in-hospital deaths occurred in patients presenting with an acute MI and cardiogenic shock. Three patients (2.2%) had a late MI. One patient with stent thrombosis underwent emergency CABG. Three patients (2.2%) underwent late CABG. Eight patients (5.7%) had a repeat PTCA. Eighty three patients (61.5%) were asymptomatic at follow up and 121 (86.4%) were free from major clinical events. CONCLUSION: In an era of increased operator experience, high pressure stent deployment, and reduced anticoagulation with antiplatelet treatment alone, multiple coronary stenting may be performed with a high procedural success rate and good intermediate term outcome.

Multiple coronary stenting in unstable angina: early and late clinical outcomes.

We examined clinical outcomes in 110 consecutive patients with unstable angina who underwent multiple coronary stenting over a 32-mo period. The main outcome measures were death, stroke, myocardial infarction, bypass surgery, and repeat angioplasty. The angiographic success rate was 100%, and the procedural success rate was 96%. There were no in-hospital deaths and five (4.5%) patients had a myocardial infarction prior to discharge. There were four (3.6%) stent thromboses with one (0.9%) patient requiring urgent bypass surgery and two (1.8%) requiring repeat angioplasty. At late follow-up (11.9+/-7.1 mo), there was (0.9%) death and three patients (2.8%) suffered myocardial infarction. Three (2.8%) patients underwent late bypass surgery and five (4.6%) had a repeat angioplasty. At follow-up, 86% of patients were event free. We conclude that multiple coronary stenting in unstable angina may be performed with a high procedural success rate and good long-term outcome.

Left main intervention revisited: early and late outcome of PTCA and stenting.

We reviewed our experience with 28 unselected, consecutive patients undergoing left main coronary artery (LMCA) angioplasty who had been considered unsuitable for coronary artery bypass graft surgery (CABG). Fourteen patients (50%) had a protected LMCA circulation. Balloon angioplasty was performed in 11 patients (39.3%), and stents were implanted in 17 patients (60.7%). The procedure was elective in 22 patients (78.6%) and acute in the setting of myocardial infarction/cardiogenic shock in 6 (21.4%). The mean follow-up duration was 15.9 +/- 12 months. There were 5 early (before hospital discharge) and 4 late deaths (total 32.1%), 1 myocardial infarction (3.6%), 6 repeat angioplasties (21.4%), and 3 subsequent CABG (10.7%). All 5 early deaths occurred in patients with cardiogenic shock and unprotected circulation. The results of our study suggest that when patients have prohibitive surgical risks, elective LMCA angioplasty and/or stenting may be undertaken with a high procedural success rate. However, our data do not support intervention in the presence of acute myocardial infarction/cardiogenic shock.

Comparison of bailout versus elective stenting: time to reassess our benchmarks of outcome.

The aim of this study was to compare the clinical outcome of elective and bailout stenting in an era of improved stent deployment techniques and antithrombotic therapy. We retrospectively analysed 136 consecutive patients undergoing stent implantation over a 15 mo period. There was no significant difference in the follow-up duration, length of hospital stay, or the total incidence of major complications between the two groups. Bailout stenting, as compared to elective stenting, was not associated with an increased incidence of in-hospital (no death vs. 1.4%, 4.6% non-Q myocardial infarction vs. 0%, 7.7% vascular complications vs. 7.0%) or late complications (no death vs. 1.4%, no non-Q myocardial infarction vs. 2.8%, 3.1% repeat angioplasty vs. 5.6%, 1.5% coronary artery bypass surgery vs. 1.4%). Stents can be implanted in cases of failed angioplasty with a success rate similar to that of elective stent implantation with a low incidence of complications and equivalent clinical outcome.

Antiplatelet therapy alone is safe and effective after coronary stenting: observations of a transition in practice.

OBJECTIVE: To evaluate the safety and efficacy of transition in practice after coronary stenting to antiplatelet therapy alone compared with anticoagulation with warfarin. DESIGN: Retrospective analysis of coronary stent management in a tertiary Canadian centre. PATIENTS: A total of 136 consecutive patients (146 lesions) were analyzed who underwent Palmaz-Schatz coronary stenting over a 15-month period and were treated with anticoagulation with warfarin (56 patients) or antiplatelet therapy alone with ticlopidine and acetylsalicylic acid (80 patients) during the transition in poststenting therapy in the authors' practice. Treatment was continued for 30 days in both groups. High pressure stent deployment was used in the majority of cases (greater than 90%), and use of intravascular ultrasound was infrequent (less than 12%). MAIN RESULTS: At 30 days, there were no clinical manifestations of stent thrombosis, coronary artery bypass surgery or repeat angioplasty in either group. One death occurred in the antiplatelet group. Periprocedural non-Q wave myocardial infarction occurred in two patients in the antiplatelet group and in one patient in the warfarin group. There was a significantly higher incidence of vascular complications in the warfarin group than in the antiplatelet group (14.3% versus 2.5%, respectively, P = 0.04). The length of hospital stay was significantly shorter in the antiplatelet group than in the warfarin group (3.0 +/- 1.8 versus 6.7 +/- 2.6 days, respectively, P < 0.001). CONCLUSIONS: Reduced anticoagulation with antiplatelet therapy alone after coronary stenting, despite infrequent use of intravascular ultrasound, is an effective and safe strategy with a low rate of vascular complications, a relatively short hospital stay and a low incidence of clinical manifestations of stent thrombosis.

Guidelines for the performance of outpatient catheterization and angiographic procedures.

Cardiac catheterization and angiography is a safe procedure performed on patients with suspected or potentially serious cardiovascular disorders. Complications of the procedure are related both to heart disease itself and to elements of the procedure. For a variety of social and financial reasons, the performance of cardiac catheterization and coronary angiography on an outpatient basis has increased recently. The purpose of this paper is to state the guidelines of The Society for Cardiac Angiography and Interventions regarding the performance of outpatient catheterization in a hospital setting.

Preliminary experience at a Canadian centre with directional coronary atherectomy for complex lesions.

OBJECTIVE: To evaluate preliminary experience of directional coronary atherectomy for complex coronary artery lesions. DESIGN: Nonrandomized, sequential patients with coronary arterial lesions that were ostial, eccentric, bulky, recurrent or membranous. SETTING: Cardiac catheterization laboratory of a tertiary referral general hospital. PATIENTS: Twenty-three patients with angina pectoris refractory to medical therapy who were suitable candidates for coronary bypass surgery. INTERVENTIONS: Directional coronary atherectomy with associated balloon angioplasty, if required, to reduce lesion stenosis to less than 25%. MAIN RESULTS: Primary success was achieved in 29 of 33 lesions (88%) by atherectomy alone and in 31 of 33 lesions (94%) by additional use of balloon angioplasty. Atherectomy retrieved tissue in 30 of 33 attempts (91%). One patient suffered Q wave myocardial infarction; one had acute occlusion after atherectomy requiring emergency balloon angioplasty; and one required repair of a false aneurysm of the femoral artery. CONCLUSIONS: Directional coronary atherectomy is safe and efficacious for ostial, bulky and eccentric lesions not optimally suited to balloon angioplasty. Lesions which have tortuous segments immediately beyond, restricting movement of the stiff nose-cone, and which are membranous or bandlike, may not be indicated for directional coronary atherectomy.

T cell activation: independent induction of killing activity and interleukin 2 secretion in cytolytic hybridomas.

Memory-like cytotoxic T lymphocytes (CTL) hybridomas exhibiting inducible killing activity and IL2 production were used to analyze the anamnestic response of CTL. Four activating agents were examined; anti-Thy-1 monoclonal antibody G7, staphylococcal enterotoxin B, interferon (IFN)-alpha/beta and IFN-gamma. These agents seemed to affect CTL activities in three distinct ways. Anti-Thy-1 monoclonal antibody, like specific antigen, was found to be a potent inducer of specific killing and IL2 production, whereas staphylococcal enterotoxin B induced IL2 production, but not cytolytic activity. On the other hand, IFN-alpha/beta and IFN-gamma effectively stimulated cytotoxicity without inducing IL2 production. The independent triggering of specific killing and IL2 secretion in the monoclonal cytolytic hybridomas suggests that in CTL distinct signals stimulate killing activity and IL2 production. The results also suggest that IFN-alpha/beta and IFN-gamma trigger the cytolytic program through an alternative activation pathway which does not involve the T cell receptor.

Ethchlorvynol (Placidyl) intoxication and its treatment by hemodialysis.

Twenty hours after ingesting 15-25 g. of ethchlorvynol, a 37-year-old woman was admitted comatose and in shock. The blood level of ethchlorvynol was 21.6 mg. % (method of Wallace). Supportive measures were instituted and hemodialysis, carried out for 10 hours, removed 5.49 g. of the drug. The post-dialysis blood level was 9.05 mg. % and the rate of dialysance was 50.5 ml./min. Only 0.6 g. of the substance was recovered from the urine over the same period.Although dialysis removed significant amounts of the drug and sustained life, the patient remained comatose for five days before withdrawal symptoms and seizures developed.