Publications by authors named "Z Uzelac"
General anesthesia (GA) earlier than recommended (as first- or second-line treatment) was recently described to improve status epilepticus (SE) outcome. We aimed to assess the impact of early GA on outcome in matched groups. Data from a multicenter, prospective cohort of 1179 SE episodes in 1049 adults were retrospectively analyzed.
View Article and Find Full Text PDFBackground: This prospective study investigates the pattern of pareses in 5q-associated spinal muscular atrophy (SMA) to identify disease-specific characteristics and potential differences from amyotrophic lateral sclerosis (ALS) and spinobulbar muscular atrophy (SBMA). Detailed knowledge about pareses patterns in SMA facilitates differential diagnosis and supports therapeutic monitoring.
Methods: Between January 2021, and June 2021, 66 SMA patients (59.
Background/objective: Insufficiency of respiratory muscles is the most important reason for mortality in the natural history of SMA. Thus, improvement or stabilization of respiratory function by disease-modifying therapies (DMT) is a very important issue.
Methods: We examined respiratory function using forced vital capacity (FVC) in 42 adult SMA patients (2 SMA type 1, 15 SMA type 2, 24 SMA type 3, 1 SMA type 4, median age 37 years, range 17-61 years) treated with nusinersen for a median of 22.
Article Synopsis
- - In April 2023, tofersen, an antisense oligonucleotide, was FDA-approved for treating ALS after it significantly reduced levels of neurofilament light chain (NfL), a marker of neurodegeneration.
- - A follow-up study involving 24 ALS patients in Germany showed that while ALS functional scores declined, there was a notable reduction in both serum NfL and phosphorylated neurofilament heavy chain (pNfH) levels in the cerebrospinal fluid (CSF).
- - The therapy was considered safe, with no ongoing symptoms, despite some patients showing signs of immune response in the CSF, pointing to potential autoimmune reactions.
Article Synopsis
- The study examined the long-term efficacy and safety of nusinersen in adults with 5q-associated spinal muscular atrophy (SMA) over a period of 38 months, utilizing a large cohort from Germany, Switzerland, and Austria.
- Overall, significant improvements were noted in various motor performance measures (HFMSE, RULM, and 6MWT) at multiple time points compared to baseline, indicating ongoing benefits from the treatment.
- No new safety concerns were found, reinforcing the idea that nusinersen remains a viable therapy for adults with SMA over extended periods.