JNS.jpgThe JULY issue of the Journal of the Neurological Sciences Vol 438 is now available online.


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Issue highlights

Full length article

Influence of inpatient rehabilitation after crisis in patients with myasthenia gravis: A retrospective cohort study using a nationwide administrative database in Japan

Otaka et al.

Published online: May 13, 2022

Growing evidence indicates that carefully programmed rehabilitation can improve symptoms in patients with myasthenia gravis (MG). However, evidence on rehabilitation to aid in recovery from a myasthenic crisis is lacking. This study aimed to examine the association between inpatient rehabilitation and recovery in the activities of daily living (ADL) after a myasthenic crisis.

High-intensity rehabilitation performed in current inpatient settings may not contribute to recovery in the ADL after a myasthenic crisis in patients with MG. Despite this study's large sample size, further research is necessary to reach firm conclusions.

Full length article

Evolution of teriflunomide use in multiple sclerosis: A real-world experience

Lorefice et al.

Published online: May 17, 2022

The present study aims to describe the evolution of teriflunomide use for multiple sclerosis (MS) in the clinical setting, in particular for naïve patients and young women. Predictors of treatment response were also investigated.

The study confirms a major evolution in teriflunomide use in clinical settings, particularly for naïve patients and young women.

Full length article

Criteria-unfulfilled multiple system atrophy at an initial stage exhibits laterality of middle cerebellar peduncles

Furuta et al.

Published online: May 13, 2022

To elucidate clinically useful imaging characteristics of multiple system atrophy with predominant cerebellar ataxia (MSA-C) at the initial stage showing pure cerebellar ataxia but unfilling consensus criteria (MSA-pc), clinical and neuroradiological analyses on cerebral MRI and single-photon emission computed tomography (SPECT) for measuring regional cerebral blood flow (rCBF) were performed.

Seven MSA-pc patients meeting the above condition at an initial evaluation were identified, and all the MSA-pc patients later developed autonomic dysfunction and finally fulfilled the criteria for probable or possible category of MSA-C. For comparison, two patients with spinocerebellar ataxia type 6 and three patients with idiopathic cerebellar ataxia who did not exhibit autonomic dysfunction for more than three years were enrolled in this study (non-MSA-pc).

As non-ataxic controls without cerebellar involvement, seven patients with Parkinson's disease were also enrolled. As a result, MRI analysis clarified a smaller pontine area and significant laterality of middle cerebellar peduncle (MCP) width in MSA-pc in comparison to non-MSA-pc and controls. SPECT analysis revealed that pontine rCBF was reduced even at the initial stage of MSA-pc. Moreover, the laterality of cerebellar rCBF values and the laterality of MCP width in MSA-pc patients exhibited a significant positive correlation, indicating anatomical and functional laterality of afferent projections to cerebellum is a characteristic finding for MSA-pc.

These neuroimaging characteristics could be clinically useful to consider the possibility of the criteria-unfulfilled MSA and promote an earlier intervention after obtaining a diagnosis of probable MSA-C.

Full length article

Intermediate alleles of HTT: A new pathway in longevity

Ingannato et al.

Published online: May 4, 2022

Centenarians are the best example of successful aging, reaching extreme longevity escaping age-related diseases. Genome sequencing studies provided evidence for genetic factors linked to heathy long life, including genes related to age-dependent diseases. HTT (Huntingtin) gene is linked to Huntington's Disease, but also associated to longevity in capuchins and mice. HTT Intermediate alleles (IAs) are defined as CAG repeat expansion between 27 and 35.

According to recent data IAs might increase Alzheimer's Disease risk, but also might have a neuroprotective effect and can confer an advantage in brain development. Here, we investigated, for the first time, the possible implication of HTT IAs in extreme longevity and their possible association in cognitive decline.

We analysed the distribution of IAs in Italian Centenarians (n = 143) and compared with pathological controls with cognitive decline (n = 232, including 80 Alzheimer's Disease, 78 Frontotemporal Dementia and 74 Subjective Cognitive Decline patients) and healthy controls (n = 104).

Our data show a statistically significant higher frequency of IAs in Centenarians with respect to pathological controls with cognitive decline (p = .031; OR = 2.3097 95% CI 1.0591 to 5.0371), with a percentage of 11.2 respect to 5.4 respectively. The highest presence of IAs in Centenarians confirms and extends in humans a possible implication of HTT gene in exceptional lifespan and in brain development with a neuroprotective effect.