scholarly journals TMEM106B, an unexpected point of contact between FTD, ageing and a hypomyelination disorder

Brain ◽  
2020 ◽  
Vol 143 (6) ◽  
pp. 1628-1631
Author(s):  
James J Doyle ◽  
J Alex Parker ◽  
Andrew Bateman
Keyword(s):  
Frontotemporal Dementia ◽  
Treatment Strategies ◽  
Dementia Treatment

This scientific commentary refers to ‘Loss of TMEM106B leads to myelination deficits: implications for frontotemporal dementia treatment strategies’, by Zhou et al. (doi:10.1093/brain/awaa141).

The effect of a lollipop on vocally disruptive behavior in a patient with frontotemporal dementia: a case-study

2014 ◽  
Vol 26 (12) ◽  
pp. 2023-2026 ◽  
Author(s):  
W. F. Fick ◽  
J. P. van der Borgh ◽  
S. Jansen ◽  
R. T. C. M. Koopmans
Keyword(s):  
Beneficial Effect ◽  
Knowledge Base ◽  
Frontotemporal Dementia ◽  
Disruptive Behavior ◽  
Treatment Strategies ◽  
Limited Knowledge ◽  
People With Dementia

ABSTRACTA problematic and disturbing behavior which can develop in people with dementia, is vocally disruptive behavior (VDB). To date, the study of VDB is underdeveloped and with only a limited knowledge base. Medications commonly used in VDB have limited benefits and specific risks in patients with dementia. This report details the case of a patient with frontotemporal dementia with VDB, which responded very well by providing a lollipop. Subsequently, we pose theory-based hypotheses in order to try to explain the beneficial effect of this intervention. This may contribute to a better understanding of VDB and possible treatment strategies.

P2-435: The chessboard square of frontotemporal dementia treatment: Results from a prospective multicenter study

2008 ◽  
Vol 4 ◽  
pp. T501-T501
Author(s):  
◽  
Bernd Ibach ◽  
Hans Gutzmann ◽  
Stefan Poljanski ◽  
Winfried Barta ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Multicenter Study ◽  
Treatment Results ◽  
Prospective Multicenter Study ◽  
Dementia Treatment

scholarly journals Loss of TMEM106B leads to myelination deficits: implications for frontotemporal dementia treatment strategies

Brain ◽  
2020 ◽  
Vol 143 (6) ◽  
pp. 1905-1919 ◽  
Author(s):  
Xiaolai Zhou ◽  
Alexandra M Nicholson ◽  
Yingxue Ren ◽  
Mieu Brooks ◽  
Peizhou Jiang ◽  
...  
Keyword(s):  
Mouse Model ◽  
Frontotemporal Dementia ◽  
Quantitative Polymerase Chain Reaction ◽  
Treatment Strategies ◽  
Significant Loss ◽  
Risk Haplotype ◽  
Wild Type ◽  
Knock Out ◽  
Brain Ageing ◽  
Cellular Markers

Abstract Genetic variants that define two distinct haplotypes at the TMEM106B locus have been implicated in multiple neurodegenerative diseases and in healthy brain ageing. In frontotemporal dementia (FTD), the high expressing TMEM106B risk haplotype was shown to increase susceptibility for FTD with TDP-43 inclusions (FTD-TDP) and to modify disease penetrance in progranulin mutation carriers (FTD-GRN). To elucidate the biological function of TMEM106B and determine whether lowering TMEM106B may be a viable therapeutic strategy, we performed brain transcriptomic analyses in 8-month-old animals from our recently developed Tmem106b−/− mouse model. We included 10 Tmem106b+/+ (wild-type), 10 Tmem106b+/− and 10 Tmem106−/− mice. The most differentially expressed genes (153 downregulated and 60 upregulated) were identified between Tmem106b−/− and wild-type animals, with an enrichment for genes implicated in myelination-related cellular processes including axon ensheathment and oligodendrocyte differentiation. Co-expression analysis also revealed that the most downregulated group of correlated genes was enriched for myelination-related processes. We further detected a significant loss of OLIG2-positive cells in the corpus callosum of Tmem106b−/− mice, which was present already in young animals (21 days) and persisted until old age (23 months), without worsening. Quantitative polymerase chain reaction revealed a reduction of differentiated but not undifferentiated oligodendrocytes cellular markers. While no obvious changes in myelin were observed at the ultrastructure levels in unchallenged animals, treatment with cuprizone revealed that Tmem106b−/− mice are more susceptible to cuprizone-induced demyelination and have a reduced capacity to remyelinate, a finding which we were able to replicate in a newly generated Tmem106b CRISPR/cas9 knock-out mouse model. Finally, using a TMEM106B HeLa knock-out cell line and primary cultured oligodendrocytes, we determined that loss of TMEM106B leads to abnormalities in the distribution of lysosomes and PLP1. Together these findings reveal an important function for TMEM106B in myelination with possible consequences for therapeutic strategies aimed at lowering TMEM106B levels.

Neurotransmitter Dysfunction and Neurotransmitter Replacement Therapy as Part of Frontotemporal Dementia Treatment

2005 ◽  
Vol 1 (3) ◽  
pp. 345-351 ◽  
Author(s):  
Martine Vercelletto ◽  
Michel Bourin ◽  
Lucette Lacomblez ◽  
Patrice Verpillat ◽  
Pascal Derkinderen
Keyword(s):  
Replacement Therapy ◽  
Frontotemporal Dementia ◽  
Dementia Treatment

A Language Approach to Treatment of Phonological Process Problems

1983 ◽  
Vol 14 (1) ◽  
pp. 47-53 ◽  
Author(s):  
Edna Carter Young
Keyword(s):  
Treatment Strategies ◽  
Phonological Process ◽  
Conceptual Skills

Treatment strategies and therapy materials for remediation of phonological process problems are described. This approach uses the child's language and conceptual skills to facilitate the use of the sound contrasts necessary to convey meaning to the listener.

Multidisciplinary Evidence to Treat Bilingual Individuals with Aphasia

2008 ◽  
Vol 15 (3) ◽  
pp. 66-72 ◽  
Author(s):  
José G. Centeno
Keyword(s):  
Stroke Rehabilitation ◽  
Culturally Diverse ◽  
Treatment Strategies ◽  
The United States ◽  
Intervention Strategies ◽  
Steady Increase ◽  
Clinical Procedures ◽  
Bilingual Speakers ◽  
Aphasic Clients

Abstract The steady increase in linguistic and cultural diversity in the country, including the number of bilingual speakers, has been predicted to continue. Minorities are expected to be the majority by 2042. Strokes, the third leading cause of death and the leading cause of long-term disability in the U.S., are quite prevalent in racial and ethnic minorities, so population estimates underscore the imperative need to develop valid clinical procedures to serve the predicted increase in linguistically and culturally diverse bilingual adults with aphasia in post-stroke rehabilitation. Bilingualism is a complex phenomenon that interconnects culture, cognition, and language; thus, as aphasia is a social phenomenon, treatment of bilingual aphasic persons would benefit from conceptual frameworks that exploit the culture-cognition-language interaction in ways that maximize both linguistic and communicative improvement leading to social re-adaptation. This paper discusses a multidisciplinary evidence-based approach to develop ecologically-valid treatment strategies for bilingual aphasic individuals. Content aims to spark practitioners' interest to explore conceptually broad intervention strategies beyond strictly linguistic domains that would facilitate linguistic gains, communicative interactions, and social functioning. This paper largely emphasizes Spanish-English individuals in the United States. Practitioners, however, are advised to adapt the proposed principles to the unique backgrounds of other bilingual aphasic clients.

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