Steroid Treatment of Primary Progressive Aphasia

Decker, David A.; Heilman, Kenneth M.

doi:10.1001/archneur.65.11.1533

Cited by 23 publications

(12 citation statements)

References 13 publications

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“…Despite these limitations, the present findings build on previous work39 40 and warrant careful review for a history of autoimmunity in all patients with neurodegenerative disease with particular emphasis on FTLD pathologies. These findings may open up a suite of new diagnostic tools and therapeutic approaches to FTLD-TDP.…”

Section: Discussionsupporting

confidence: 53%

TDP-43 frontotemporal lobar degeneration and autoimmune disease

Miller

Rankin

Graff‐Radford

et al. 2013

Journal of Neurology, Neurosurgery & Psychiatry

144

132

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Background The aetiology and pathogenesis of non-genetic forms of frontotemporal dementia (FTD) is unknown and even with the genetic forms of FTD, pathogenesis remains elusive. Given the association between systemic inflammation and other neurodegenerative processes, links between autoimmunity and FTD need to be explored. Objective To describe the prevalence of systemic autoimmune disease in semantic variant primary progressive aphasia (svPPA), a clinical cohort, and in progranulin (PGRN) mutation carriers compared to neurologically healthy normal controls (NC) and Alzheimer’s disease (AD) as dementia controls. Design Case control. Setting Academic medical centres. Participants 129 svPPA, 39 PGRN, 186 NC, and 158 AD patients underwent chart review for autoimmune conditions. A large subset of svPPA, PGRN, and NC cohorts underwent serum analysis for tumor necrosis factor α (TNF-α) levels. Outcome Measures Chi-square comparison of autoimmune prevalence and follow up logistic regression. Results There was a significantly increased risk of autoimmune disorders clustered around inflammatory arthritides, cutaneous disorders, and gastrointestinal conditions in the svPPA and PGRN cohorts. Elevated TNF-α levels were observed in svPPA and PGRN compared to NC. Conclusions svPPA and PGRN are associated with increased prevalence of specific and related autoimmune diseases compared to NC and AD. These findings suggest a unique pattern of systemic inflammation in svPPA and PGRN and open new research avenues for understanding and treating disorders associated with underlying transactive response DNA-binding protein 43 (TDP-43) aggregation.

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Section: Discussionsupporting

confidence: 53%

TDP-43 frontotemporal lobar degeneration and autoimmune disease

Miller

Rankin

Graff‐Radford

et al. 2013

Journal of Neurology, Neurosurgery & Psychiatry

144

132

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“…However, a large randomized clinical trial specifically in lvPPA has not been completed. Case studies have reported improvement in language with steroid treatment 110 or Omentum Transposition Therapy 111 , but these effects have not been replicated. Theoretically, medications that enhance neuroplasticity, such as selective serotonin reuptake inhibitors, might augment the effects of tDCS, but the combination of interventions has not been studied in PPA.…”

Section: Introductionmentioning

confidence: 99%

Treatment of Primary Progressive Aphasia

Tippett

Hillis

Tsapkini

2015

Curr Treat Options Neurol

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Primary progressive aphasia (PPA) is a neurodegenerative disease that primarily affects language functions and often begins in the fifth or sixth decade of life. The devastating effects on work and home life call for the investigation of treatment alternatives. In this paper, we present a review of the literature on treatment approaches for this neurodegenerative disease. We also present new data from two intervention studies we have conducted, a behavioral one and a neuromodulatory one using transcranial direct current stimulation (tDCS) combined with written production intervention. We show that speech-language intervention improves language outcomes in individuals with PPA; and especially in the short term, tDCS augments generalization and maintenance of positive language outcomes. We also outline current issues and challenges in intervention approaches in PPA.

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“…[14][15][16]18,19,38,39 As for efficacy in cognition, two studies showed no change, 14,37 whereas four others reported a clinical improvement, also not statistically significant. 13,17,38,40 Overall, we found that 1/3 of the studies reported significant improvement in behavior symptoms with SSRI, 1/3 showed clinical but not statistically significant changes and 1/6 showed no differences from baseline evaluation. Also, 1/6 observed clinical improvement in anxiety, self-harm, retardation, agitation and delusions, but also observed worsening in apathy and functioning although this was not statistically significant.…”

Section: Resultsmentioning

confidence: 72%

“…The largest sample comprised 49 patients 12 and seven studies were single-case reports. [13][14][15][16][17][18][19] Nine studies did not define the subtype of FTLD analyzed (n = 124). Among the studies that defined the FTLD subtypes, the behavioral variant was the most common (n = 202), followed by progressive non-fluent aphasia (n = 38) and semantic dementia (n = 25).…”

Section: Resultsmentioning

confidence: 99%

Pharmacological treatment of frontotemporal lobar degeneration: systematic review

Portugal

Marinho

Laks

2011

Rev. Bras. Psiquiatr.

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Objective: To identify the therapeutic options available for treatment of cognitive and behavioral symptoms in frontotemporal lobar degeneration. Method: Systematic review using the descriptors "frontotemporal lobar degeneration" OR "frontotemporal dementia" OR "fronto-temporal dementia" OR "fronto-temporal degeneration" OR "Pick's disease" OR "Pick's atrophy" OR "semantic dementia" OR "progressive aphasia" AND "pharmacotherapy" OR "treatment" OR "efficacy" OR "effects" OR "management" was performed in the Medline and Lilacs databases. Selection criteria: Quality A -randomized clinical trials. Quality Bopen studies or reports of six or more cases. Quality C -reports of five or fewer cases. Two reviewers independently assessed the clinical studies. Information collected included diagnostic criteria used, sample size, duration, efficacy and tolerability measures used and results obtained. Results: From the 532 studies found, 29 complied with the inclusion criteria. All studies worked with a small sample, had short duration of treatment and used non-uniform measures in evaluating efficacy and tolerability. Studies showed disparate results with respect to behavior and cognition. Conclusion: There is still little, and poor, evidence available for treatment of frontotemporal lobar degeneration and studies with better methodological background are needed. Descriptors IntroductionThe clinical syndromes related to frontotemporal lobar degeneration (FTLD) are the second most common cause of pre-senile primary dementia. 1 A recent Brazilian epidemiological study found a dementia prevalence of 7.1% in individuals over

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Steroid Treatment of Primary Progressive Aphasia

Cited by 23 publications

References 13 publications

TDP-43 frontotemporal lobar degeneration and autoimmune disease

TDP-43 frontotemporal lobar degeneration and autoimmune disease

Treatment of Primary Progressive Aphasia

Pharmacological treatment of frontotemporal lobar degeneration: systematic review

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