Presentation
Parkinsonism was present in 3/10 patients in the AOS/agrammatism group. [neurology.org]
We present a review of the literature on Primary Progressive Aphasia (PPA) together with the analysis of neuropschychological and neuroradiologic profiles of 42 PPA patients. [ucdavis.pure.elsevier.com]
In the present case, language impairment was the first symptom, and fluency was the most prominant symptom. [synapse.koreamed.org]
Here, we describe the initial presentation of naPPA in a patient with right hemisphere atrophy, but left hemisphere language dominance, who developed a clinical phenotype of CBD. [movementdisorders.org]
Sixteen months after presentation, she is said to have had seizures. Any cortical form of dementia can cause seizures. She died 28 months after presentation. Summary of presentation The crucial features are these. She was only 49. [jnnp.bmj.com]
Treatment
[…] and at post-treatment. [clinicaltrials.gov]
TREATMENT OF NONFLUENT/AGRAMMATIC PRIMARY PROGRESSIVE APHASIA It is important to achieve an accurate diagnosis because treatment trials are beginning to emerge that can help block the accumulation of misfolded tau in the brain. [ftd.med.upenn.edu]
Treatment of Primary Progressive Aphasia. Current Treatment Options Neurology ; August 2015. [theaftd.org]
Te implementation of the treatment, along with the immediate results post-treatment and after one month of follow-up are described. [redalyc.org]
Several medications have been investigated to measure efficacy of treatment in FTD, often with mixed results. [life-enhancement.com]
Prognosis
In these patients, prognosis is obviously poorer and management more complicated. Patients who do not develop these additional symptoms are able to preserve their independence and active lifestyle for a longer period of time. [theaftd.org]
What is the prognosis for people with PPA? This is a difficult question to answer as there has been very little research into it and it can be extremely variable from person to person. [raredementiasupport.org]
Prognosis There is limited research on the prognosis of FTD. [aafp.org]
Treatment and Prognosis At this time, there is no specific treatment for CBD. Instead individual symptoms are targeted with specific medications. [rarediseasesnetwork.org]
Etiology
Neuroimaging (magnetic resonance imaging) usually demonstrates focal atrophy in addition to excluding other etiologies. [aafp.org]
The diagnosis and understanding of apraxia of speech: Why including neurodegenerative etiologies may be important. Journal of Speech, Language, and Hearing Research, 55 (Suppl.), S1518–S1522. Google Scholar Folstein, M. F., Folstein, S. [jslhr.pubs.asha.org]
Etiologies of Aphasia include(s) which of the following: A. Primary progressive aphasia & Vascular B. Infectous (bacterial & viral) C. Metabolic D. Neoplastic Traumatic E. [quizlet.com]
A target for etiologically-driven therapy in these patients thus has been identified. A manuscript under review characterizes the clinical and pathological characteristics of these patients (Grossman et al 2007b). [dana.org]
In an era of etiology-specific treatments for neurodegenerative conditions, determining the histopathological basis of PPA is crucial. [nature.com]
Epidemiology
Epidemiology and genetics of the frontotemporal dementia/Pick’s disease. Annals of Neurology, 54 (5), 29-31. Diehl-Schmid, J., Schmidt, E.M., Nunnemann, S., Riedl, L., Kurz, A., Förstl, H., Wagenpfeil, S., & Cramer, B. (2013). [revista.infad.eu]
Journal of Clinical Epidemiology, 63 (5), 535 – 542. Hsieh, S., Irish, M., Daveson, N., Hodges, J.R., & Piguet, O. ( 2013 a). When one loses empathy: Its effect on carers of patients with dementia. [cambridge.org]
Development We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. [elsevier.es]
Although epidemiologic studies are limited, approximately 20 to 50 percent of persons younger than 65 years with dementia have FTD, a prevalence similar to that of Alzheimer disease in persons 45 to 64 years of age (15 per 100,000). 1 – 3 FTD is underdiagnosed [aafp.org]
Pathophysiology
Conclusion: PPA with AOS is aligned with the syndrome previously designated progressive nonfluent aphasia; agrammatism may emerge as the syndrome evolves, or alternatively, the pure AOS group may be pathophysiologically distinct. [neurology.org]
It may be that limb apraxia is an early sign of the development of a parkinsonian syndrome, consistent with previous suggestions of a close pathophysiological relation between these deficits [ 5 ]; longitudinal studies of PNFA cohorts will be required [link.springer.com]
B 30 Pathophysiology Common pathologic findings in FTD are atrophy and neuronal loss affecting the frontal and temporal lobes of the brain. 10 There is histologic heterogeneity in patients with FTD, but about 55 percent of patients have FTD with ubiquitin-positive [aafp.org]
Tobinick Perispinal etanercept produces rapid improvement in primary progressive aphasia: identification of a novel, rapidly reversible TNF-mediated pathophysiologic mechanism Medscape J Med, 10 (2008), pp. 135 [94] S.J. Banks, S. [elsevier.es]
Prevention
These include use of a walking aid with a heavy front to prevent falling backwards, eating more solid foods and less thin liquids, and physical therapy or exercise programs to improve mobility. [rarediseasesnetwork.org]
A Caregiver’s Guide to Dementia explores the use of activities and other techniques to prevent, reduce and manage the behavioral symptoms of dementia. [dementiaresources.org.au]
This report illustrates that systematic testing of language and semantic memory and a high index of suspicion can prevent misdiagnosis and aid sub typing of PPA. » References 1. Hodges JR. [neurologyindia.com]
Significance : This study may lead to the identification of the chromosome(s) responsible for a genetically determined language disorder, providing a target for interventions to prevent this disorder. [dana.org]
However, the findings reported here suggest that specific apraxic speech features emerge or reach a critical threshold when cortical regions for speech motor control are affected bilaterally, preventing or reversing any cross-hemispheric compensatory [journals.plos.org]