Resistance Training Effective for Pompe Disease, PPMS Similar to SPMS, Factors Associated With Modified Atkins Diet


Neurology News Network for the week ending March 4, 2022. [WATCH TIME: 4 minutes]

WATCH TIME: 4 minutes

Welcome to this special edition of Neurology News Network. I’m Marco Meglio.

Results from a pilot study showed that a combination approach of resistance training, respiratory training, and enzyme replacement therapy (ERT) is safe for patients with late-onset Pompe disease (LOPD) and may provide positive benefits on muscular strength, functional capacity, and respiratory function. The study included 8 male and 2 female adults with confirmed diagnosis of LOPD who underwent respiratory muscle training 6 days per week combined with 24-week supervised resistance training program that had trainings 3 times per week. At baseline, all subjects had overall similar proximal muscle weakness. Following the 24-week program, there were significant improvements observed in muscle strength in multiple areas, including elbow flexors and elbow extensor. Additionally, the leg extensor strength improved by 10.5 Nm and leg flexors improved by 12.1 Nm. The approach was proven to be safe as well, with no changes in creatinine kinase and lactic dehydrogenase levels throughout the study.

Analyses of pooled placebo arm data from clinical trials in multiple sclerosis (MS) suggest that the percentage of patients with disability progression and improvement within the first year were similar between those with primary progressive multiple sclerosis (PPMS) and secondary progressive multiple sclerosis (SPMS). These findings were also observed after stratifying for baseline disability severity.Findings were presented at the Americas Committee for Treatment and Research in Multiple Sclerosis (ACTRIMS) Forum 2022, February 24-26, in West Palm Beach, Florida, and further support similarities between the 2 progressive MS patient populations.Investigators posited the need for more transformational treatments to improve disability, due to observed high disability progression, between 9.2% and 11.1%, and limited disability improvement, between 5.6% and 6.5% at 6 months, confirmed at 12 months in patients with PPMS and SPMS receiving placebo. For those with PPMS, 46.2% had a baseline Expanded Disability Status Score (EDSS) of 5.5 or greater, compared with those with SPMS, where 62.1% had a baseline EDSS score of 5.5 or greater.

Factors such as the number of antiseizure medications, a lower age of epilepsy onset, and a history of status epilepticus were found to be associated with modified Atkins diet (MAD) initiation in adults with drug-resistant epilepsy (DRE), according to the findings of a recent study. Additionally, investigators concluded that chronic cases of DRE may be motivation for MAD initiation. Investigators retrospectively collected data on 136 patients with DRE at least 16 years of age that failed at least 2 antiseizure medications. The investigators identified 5 factors that were associated with MAD initiation. First, patients who tried the MAD had an average of 1 more ASM trial in life and an additional current ASM. Furthermore, these patients had lower epilepsy onset age than those who did not initiate MAD. There was a negative association between the presence of temporal-onset epilepsy and MAD trial, while a history of status epilepticus increased the odds of a MAD trial by almost 4 times. The 2 groups remained similar on all other demographic or clinical factors.

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