Primary Progressive Aphasia (Progressive Nonfluent Aphasia)

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35 relevant articles (2 outcomes, 2 trials/studies) found for this Disease

Description: A progressive form of dementia characterized by the global loss of language abilities and initial preservation of other cognitive functions. Fluent and nonfluent subtypes have been described. Eventually a pattern of global cognitive dysfunction, similar to ALZHEIMER DISEASE, emerges. Pathologically, there are no Alzheimer or PICK DISEASE like changes, however, spongiform changes of cortical layers II and III are present in the TEMPORAL LOBE and FRONTAL LOBE. (From Brain 1998 Jan;121(Pt 1):115-26)

Also Known As:

Progressive Nonfluent Aphasia; Aphasia, Primary Progressive; Mesulam's Syndrome; Aphasia, Progressive Nonfluent; Aphasias, Primary Progressive; Aphasias, Progressive Nonfluent; Nonfluent Aphasia, Progressive; Nonfluent Aphasias, Progressive; Primary Progressive Aphasias; Progressive Aphasia, Primary; Progressive Aphasias, Primary; Progressive Nonfluent Aphasias; Syndrome, Mesulam; Syndrome, Mesulam's; Mesulam Syndrome

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Alzheimer Disease (Alzheimer's Disease)
2.	Dementia (Dementias)
3.	Syndrome
4.	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
5.	Pick Disease of the Brain (Pick's Disease)

Experts

1.	Weintraub, Sandra: 3 articles (12/2006 - 11/2004)
2.	Johnson, Nancy: 2 articles (12/2006 - 12/2005)
3.	Mesulam, M-Marsel: 2 articles (12/2005 - 11/2004)
4.	Piccirilli, M: 1 article (01/2008)
5.	Provinciali, L: 1 article (01/2008)
6.	Blair, M: 1 article (01/2008)
7.	Viticchi, G: 1 article (01/2008)
8.	Light, M: 1 article (01/2008)
9.	Davidson, W: 1 article (01/2008)
10.	Snowden, J S: 1 article (01/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Primary Progressive Aphasia:

1.	TNFR-Fc fusion protein (etanercept)FDA Link 01/01/2008 - "Perispinal etanercept produces rapid improvement in primary progressive aphasia: identification of a novel, rapidly reversible TNF-mediated pathophysiologic mechanism." 09/01/2009 - "Subsequent case studies have documented rapid clinical improvement following perispinal etanercept in both AD and primary progressive aphasia, providing evidence of rapidly reversible, TNF-dependent, pathophysiological mechanisms in AD and related disorders. " 01/01/2008 - "CONCLUSION: In combination with the previously reported results of perispinal etanercept in Alzheimer's disease and primary progressive aphasia, these results further argue that larger scale studies of this therapeutic intervention, including Phase 3 trials, are warranted in dementias. " 01/01/2008 - "BACKGROUND: Recent clinical studies point to rapid and sustained clinical, cognitive, and behavioral improvement in both Alzheimer's disease and primary progressive aphasia following weekly perispinal administration of etanercept, a TNF-alpha inhibitor that acts by blocking the binding of this cytokine to its receptors. " Order ALL the reference details at left...
2.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 01/01/2008 - "BACKGROUND: Recent clinical studies point to rapid and sustained clinical, cognitive, and behavioral improvement in both Alzheimer's disease and primary progressive aphasia following weekly perispinal administration of etanercept, a TNF-alpha inhibitor that acts by blocking the binding of this cytokine to its receptors. " Order ALL the reference details at left...
3.	tau Proteins (tau Protein)IBA 08/01/2007 - "Reported here is a new missense mutation V363I in exon 12 of the microtubule-associated protein tau (MAPT) gene associated with progressive nonfluent aphasia, with onset at the age of 69 years in a woman. " 11/15/2002 - "Abeta peptide 1-42, Tau protein and S-100B protein level in cerebrospinal fluid of three patients with primary progressive aphasia." 10/01/2001 - "We report five patients with clinical and radiological features of degenerative dementia with predominantly frontal involvement and one patient with primary progressive aphasia Diagnostic work-up included computed tomography (CT), magnetic resonance imaging (MRI), PET and tau-protein and beta-amyloid level determination in CSF. " Order ALL the reference details at left...
4.	UbiquitinIBA 11/01/2005 - "However, for FTD with motor neurone disease (FTD+MND), semantic dementia or primary progressive aphasia (PA), the histological profile was either ubiquitin type or DLDH type; Pick-type histology was seen in only 1 case of PA. " 10/01/2005 - "There is a corresponding clinical dichotomy combining the behavioral variety of FTD presentation with semantic dementia and usually ubiquitin positive tau negative pathology on one hand and the association of primary progressive aphasia and cortical basal degeneration/PSP syndrome with tau positive pathology on the other. " Order ALL the reference details at left...
5.	Biological Markers (Surrogate Marker)IBA 10/01/2008 - "Primary progressive aphasia and the growing role of biomarkers in neurological diagnosis." 01/01/2008 - "Subtype analysis revealed that the most AD-like profile of biomarkers were observed in FTLD with motor neuron signs, whereas the most non-AD profile were observed in patients with primary progressive aphasia. " Order ALL the reference details at left...
6.	Galantamine (Galanthamine)IBA 01/01/2008 - "METHOD: Thirty-six behavioral variety FTD and primary progressive aphasia (PPA) patients were treated in an open-label period of 18 weeks and a randomized, placebo-controlled phase for 8 weeks with galantamine. " 01/01/2008 - "Galantamine in frontotemporal dementia and primary progressive aphasia." 03/01/2007 - "Improvement has been reported in FTD for all drugs except piracetam, guanfacine and galantamine, although there was improvement on galantamine in primary progressive aphasia. " Order ALL the reference details at left...
7.	Apolipoproteins E (ApoE)IBA 02/01/2006 - "Apoe epsilon2-epsilon4 genotype is a possible risk factor for primary progressive aphasia." 05/04/2001 - "The role of the Apolipoprotein E (APOE) alleles in syndromes associated with focal cerebral atrophy (fronto-temporal dementia, primary progressive aphasia, corticobasal degeneration) is still controversial. " Order ALL the reference details at left...
8.	ApolipoproteinsIBA 07/01/1997 - "We obtained apolipoprotein E genotyping in a population of 12 consecutive patients who fulfilled rigorous criteria for the clinical diagnosis of primary progressive aphasia (PPA). " 07/01/1997 - "Apolipoprotein E genotypes in primary progressive aphasia." 05/04/2001 - "The role of the Apolipoprotein E (APOE) alleles in syndromes associated with focal cerebral atrophy (fronto-temporal dementia, primary progressive aphasia, corticobasal degeneration) is still controversial. " Order ALL the reference details at left...
9.	Amyloid (Amyloid Fibrils)IBA 10/01/2008 - "Abeta amyloid and glucose metabolism in three variants of primary progressive aphasia." 10/01/2001 - "We report five patients with clinical and radiological features of degenerative dementia with predominantly frontal involvement and one patient with primary progressive aphasia Diagnostic work-up included computed tomography (CT), magnetic resonance imaging (MRI), PET and tau-protein and beta-amyloid level determination in CSF. " Order ALL the reference details at left...
10.	Apolipoprotein E2IBA 07/01/2009 - "APOE epsilon 2/epsilon 4 genotype a risk factor for primary progressive aphasia in women." Order ALL the reference details at left...

Therapies and Procedures

1.	Vasectomy (Vas Occlusion) 12/01/2006 - "OBJECTIVE: To study the frequency of vasectomy in men with primary progressive aphasia (PPA). " 12/01/2006 - "Vasectomy in men with primary progressive aphasia." 08/01/2009 - "Most recently, a manuscript from the neurology literature has described an association between vasectomy and primary progressive aphasia, a rare variety of frontotemporal dementia. " Order ALL the reference details at left...
2.	Activities of Daily Living (ADL) 09/27/2005 - "CONCLUSIONS: Although activities of daily living are well maintained during the first years of the disease, patients with primary progressive aphasia (PPA) may lose autonomy 6 to 7 years after onset. " 04/01/1996 - "Primary progressive aphasia (PPA) is defined as progressive decline in language for 2 or more years with preservation of activities of daily living and general cognitive functions. " 01/01/2007 - "Functional ability was assessed in individuals in the early stages of AD (N=100), the behavioral variant of frontotemporal dementia (FTD) (N=57), and primary progressive aphasia (PPA) (N=61), using the activities of daily living questionnaire (Johnson et al, 2004). " 07/01/2001 - "Primary progressive aphasia (PPA) is an uncommon neurodegenerative syndrome characterized by a relatively isolated dissolution of language function at the beginning, followed by deterioration of general cognitive function and of activities of daily living after 2 or more years. " 06/01/1992 - "The clinical syndrome of primary progressive aphasia corresponds to the gradual onset and worsening of language dysfunction in patients who otherwise remained independent for many years in daily living activities, insight, judgement and behaviour. " Order ALL the reference details at left...

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