Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy.

BACKGROUND: Cerebral amyloid angiopathy (CAA) typically presents with lobar intracerebral macrohemorrhages (ICH) or microbleeds (MBs). Several case reports also found superficial siderosis (SS) in patients with CAA. We aimed to assess the value of SS for the in vivo diagnosis of CAA, and tested whether the inclusion of SS as a criterion alters the sensitivity and specificity of the Boston criteria for CAA-related hemorrhage. METHODS: We retrospectively analyzed the T2*-weighted MRIs of 38 patients with histopathologically proven CAA and of 22 control patients with histopathologically proven non-CAA ICHs regarding the presence of ICHs, MBs, and SS. We compared the sensitivity and specificity of the classic Boston criteria to that of modified criteria, which included SS as a criterion. RESULTS: ICHs were present in 71% of the patients with CAA, and in all control patients. MBs were found in 47.4% of patients with CAA and in 22.7% of controls. SS was detected in 60.5% of patients with CAA, but in none of the controls. The classic criteria had a sensitivity of 89.5% for CAA-related hemorrhage, while inclusion of SS increased their sensitivity to 94.7% (not significant). On the contrary, the specificity of the Boston criteria was 81.2% both for the classic and for the modified criteria. CONCLUSIONS: Superficial siderosis (SS) occurs with high prevalence in cerebral amyloid angiopathy (CAA) and is rare in non-CAA forms of intracerebral hemorrhages. Thus, we propose that inclusion of SS in the Boston criteria might enhance their sensitivity for CAA-related hemorrhage without loss of specificity.

Analysis of health-related quality of life and muscle impairment in individuals with amyotrophic lateral sclerosis using the medical outcome survey and the Tufts Quantitative Neuromuscular Exam.

OBJECTIVE: The Tufts Quantitative Neuromuscular Exam (TQNE) is commonly used to assess the rate of disease progression in individuals with amyotrophic lateral sclerosis (ALS). The Medical Outcome Study Short Form (SF-36) is a general method to assess health-related quality of life (HRQL). This study examined the relationship between the TQNE and SF-36, established the reliability and responsiveness of each, and contrasted the HRQL between individuals with ALS and the general population. DESIGN: Subjects (31) completed the SF-36 and TQNE within 1 week to determine reliability. Subjects (17) also completed both the TQNE and SF-36 each month for 1 year after diagnosis of ALS to establish the relationship between the two assessment tools. SETTING: A primary care university teaching hospital. PATIENTS: Thirty-one subjects with an age range of 27 to 76 years (mean 59.1, SD 10.32), recently diagnosed with ALS. RESULTS: Each test was highly reliable and responsive. The intraclass correlations (2, 1) were consistently higher for the TQNE (.93 to .98) than for the SF-36 (.57 to .90). Changes in physical function were correlated to changes in lower extremity force megascores (.48). CONCLUSION: Both the TQNE and the SF-36 are reliable and responsive and appear important in characterization of patient status after ALS is diagnosed.

The effects of a home exercise program on impairment and health-related quality of life in persons with chronic peripheral neuropathies.

BACKGROUND AND PURPOSE: The effects of a home exercise program for persons with chronic peripheral neuropathies (CPN) have not been documented. We compared changes in impairment and health-related quality of life (HRQL) between exercise and control groups, investigated the relationship between HRQL and measures of impairment, and contrasted the HRQL of individuals with CPN to that previously described for the general population. SUBJECTS: Twenty-eight subjects with CPN, aged 23 to 84 years (mean = 56.2, SD = 14.9), completed the study. METHODS: Impairment measures included average muscle score (AMS), handgrip force, walking time, and forced vital capacity. The HRQL instrument measured the eight scales of the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and the component scales. The exercise group (n = 14) completed a 6-week home exercise program. The control group (n = 14) did not participate in a home exercise program. RESULTS: There was an increase in the AMS in the exercise group compared with the control group. No other between-group differences were found. The exercise group improved in scores on the role limitation scales of the SF-36. The AMS and walking time were moderately correlated with the physical function scale of the SF-36 (r = .55 and -.62, respectively). The SF-36 scores of individuals with CPN were lower than scores previously described for the general population. CONCLUSION AND DISCUSSION: The home exercise program appears to be an important component of the treatment of persons with CPN. Compared with the general population, patients with CPN appear to have a lower HRQL, but some areas appear to improve following a home exercise program.