Dysphagia Affecting Quality of Life in Cerebellar Ataxia—a Large Survey

Rönnefarth, Maria; Hanisch, N.; Brandt, Alexander U.; Mähler, Anja; Endres, Matthias; Paul, Friedemann; Doss, Sarah

doi:10.1007/s12311-020-01122-w

Cited by 26 publications

(16 citation statements)

References 36 publications

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“…As weight loss is a significant indicator of the progression of NPC1 disease [ 34 , 49 ], the diminished weight loss can be regarded as a therapeutic effect in NPC1 −/− mice when compared to the respective None- or Sham-treated groups [ 4 , 16 , 34 , 71 , 79 , 80 , 81 , 82 , 83 , 84 , 85 ].…”

Section: Discussionmentioning

confidence: 99%

Gender-Specific Effects of Two Treatment Strategies in a Mouse Model of Niemann-Pick Disease Type C1

Holzmann

Witt

Rolfs

et al. 2021

IJMS

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In a mouse model of Niemann-Pick disease type C1 (NPC1), a combination therapy (COMBI) of miglustat (MIGLU), the neurosteroid allopregnanolone (ALLO) and the cyclic oligosaccharide 2-hydroxypropyl-β-cyclodextrin (HPßCD) has previously resulted in, among other things, significantly improved motor function. The present study was designed to compare the therapeutic effects of the COMBI therapy with that of MIGLU or HPßCD alone on body and brain weight and the behavior of NPC1−/− mice in a larger cohort, with special reference to gender differences. A total of 117 NPC1−/− and 123 NPC1+/+ mice underwent either COMBI, MIGLU only, HPßCD only, or vehicle treatment (Sham), or received no treatment at all (None). In male and female NPC1−/− mice, all treatments led to decreased loss of body weight and, partly, brain weight. Concerning motor coordination, as revealed by the accelerod test, male NPC1−/− mice benefited from COMBI treatment, whereas female mice benefited from COMBI, MIGLU, and HPßCD treatment. As seen in the open field test, the reduced locomotor activity of male and female NPC1−/− mice was not significantly ameliorated in either treatment group. Our results suggest that in NPC1−/− mice, each drug treatment scheme had a beneficial effect on at least some of the parameters evaluated compared with Sham-treated mice. Only in COMBI-treated male and female NPC+/+ mice were drug effects seen in reduced body and brain weights. Upon COMBI treatment, the increased dosage of drugs necessary for anesthesia in Sham-treated male and female NPC1−/− mice was almost completely reduced only in the female groups.

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Section: Discussionmentioning

confidence: 99%

Gender-Specific Effects of Two Treatment Strategies in a Mouse Model of Niemann-Pick Disease Type C1

Holzmann

Witt

Rolfs

et al. 2021

IJMS

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“…In humans, Oya et al (2014) 65 reports a high proportion of cerebellar tumour detection with associated AN. Similarly, cerebellar degeneration associated with ataxia correlates with increased likelihood of being underweight or experiencing abnormal appetite patterns [66][67][68][69] . Due to appetite disturbances emerging post cerebellar structural abnormalities, these studies indicate that cerebellar deficits could play a causative role in the loss of appetite.…”

Section: -The Cerebellum: Volumetric Studies Of Over-and Undereatingmentioning

confidence: 99%

The Cerebellum Plays More Than One Role in the Dysregulation of Appetite: Review of Structural Evidence from Typical and Eating Disorder Populations

Sader

Waiter

Williams

2022

Preprint

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BackgroundDysregulated appetite control is characteristic of anorexia nervosa (AN) and obesity (OB). Studies using a broad range of methods suggest the cerebellum plays an important role in appetite control, and it is implicated in both AN and OB with reports of aberrant grey matter volume (GMV) compared to non-clinical populations. As functions of the cerebellum are anatomically segregated, specific localization of aberrant anatomy may indicate how it affects appetite control in different states.ObjectiveTo determine if there were consistencies in regions of cerebellar GMV changes affected in AN and OB, and across normative variation.Methodsystematic review and meta-analysis using Ginger ALE.ResultsTwenty publications were identified as either case-control studies (with total n=619) or regressed weight from normative data against brain volume (with total n=3,518). AN and OB analyses both showed consistently decreased GMV within the left cerebellum, but volume reduction was anterior for AN and posterior for OB, with minimal overlap. Analysis of the normative dataset identified a cluster in right posterior lobe.DiscussionThese findings suggest that more than one area of the cerebellum is involved in control of eating behaviour and is differentially affected in normal variation and pathological conditions. Specifically, we hypothesise an association with sensorimotor and emotional learning in AN, but with executive function in OB.

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“…A study in Taiwan found that dysphagia is a critical factor in aspiration pneumonia and malnutrition (14). ; dehydration; and malnutrition, which in turn can lead to prolonged hospital stay (5); psychological disorders such as stress, anxiety, and depression; decreased quality of life (15); increased health costs; increased mortality and decreased patient outcomes after stroke (16). Based on a study by Takeda et al, aspiration was observed in 16 of 172 patient when swallowing 3 ml of water during Video Fluoroscopy was conducted (17).…”

Section: Dysphagiamentioning

confidence: 99%

Dysphagia Screening Protocol for Acute Stroke Patient: A Literature Review

Mulyatsih¹,

Pandin²

2021

Preprint

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Background: Nearly two-thirds of acute stroke patients have dysphagia. Dysphagia is difficulty to swallow food or liquids. Early detection of dysphagia is crucial in stroke patients as a result of increased morbidity and mortality due to malnutrition and respiratory tract infections. Aim: Our purpose was to conduct a literature review of dysphagia screening for stroke patient. Methods: We used the bolean operator to search articles of “or” and “and” with the key words were "Dysphagia" or “Screening”, AND "Stroke" or Acute Stroke” AND "Nursing". Data based used were Scopus, Proquest and Science Direct with inclusion criteria using full text in English which published from 2019 to 2021. We obtained 240 articles and then we screened by reading the main focus of articles with paying attention to the topics and the suitability of article content.Result: Twenty five publications relating to dysphagia screening met the inclusion criteria. There are five methods of dysphagia screening performed by nurses or other health workers: 1) a simple Questionnaire Test (4QT) method; 2) Water Swallow Test (WST) method; 3) Bed Side Screening Tool for Dysphagia (BSTD) method; 4) Volume Viscosity Swallow Test (V-VST) method; 5) EAT-10 method.Conclusion: screening is the first step in the identification of swallowing impairment or dysphagia of stroke patient. Dysphagia is an independent predictor of poor patient outcome and prolonged recovery time. Nurse has an important role to conduct a screening and must ensure that the selected tools has high reliability and concurrent validity. Key Words: Dysphagia, Nursing, Screening, Stroke

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Dysphagia Affecting Quality of Life in Cerebellar Ataxia—a Large Survey

Cited by 26 publications

References 36 publications

Gender-Specific Effects of Two Treatment Strategies in a Mouse Model of Niemann-Pick Disease Type C1

Gender-Specific Effects of Two Treatment Strategies in a Mouse Model of Niemann-Pick Disease Type C1

The Cerebellum Plays More Than One Role in the Dysregulation of Appetite: Review of Structural Evidence from Typical and Eating Disorder Populations

Dysphagia Screening Protocol for Acute Stroke Patient: A Literature Review

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