Symptoma

Primary Progressive Non-Fluent Aphasia

Agramatic Variant of PPA

Parkinsonism was present in 3/10 patients in the AOS/agrammatism group. [neurology.org]

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]

The present study had three aims: (1) to evaluate syntactic production in nfPPA; (2) to document our impression that frank agrammatism may not be universally present in nfPPA patients, even those with preserved motor speech skills, and (3) to address [karger.com]

The resulting Model 1 with three predictors is presented in Table 4. [journals.plos.org]

  • Difficulty Walking

    For example, rigidity and difficulty walking may partially respond to treatments for Parkinson’s disease. Dystonia and myoclonus may respond to muscle relaxants or anti-seizure medications. [rarediseasesnetwork.org]

  • Hypophonia

    In addition, a neurological examination now revealed a hypokinetic-rigid syndrome on the left side of his body, hypomimia with reduced blink frequency, and hypophonia as well as a mild flexed dystonic posture of the left arm. [movementdisorders.org]

  • Cough

    Silences at the start and end of each sample and bursts of intensity due to nonspeech behaviour (e.g. laughing, coughing) were removed prior to measurement. Lexical Stress. The polysyllable word samples were used to examine lexical stress. [journals.plos.org]

  • Muscle Rigidity

    Many patients with the nonfluent variant go on to develop parkinsonian symptoms that overlap with progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), such as an inability to move the eyes side-to-side, muscle rigidity in the arms and [memory.ucsf.edu]

    rigidity in the arms and legs, and weakness in the muscles around the throat. [dementiatoday.com]

    rigidity in the arms and legs, falls, and weakness in the muscles around the throat. [rarediseasesnetwork.org]

  • Slow Speech

    Communication efficacy is enhanced when conversing in a familiar environment at a slow speech rate, supplemented by gestures. [ftd.med.upenn.edu]

    Both pausing and lexical stress changes can indicate difficulty with planning spatial, temporal and amplitude parameters of multi-articulator movements for speech and result in slowed speech rate. [journals.plos.org]

    speech, incomprehension and lack of understanding. [alzheimers.net]

    This leads to slow speech with frequent pauses as people search for the word they want to use. [dementiablog.org]

    The overall result is slow speech and decreased fluency with frequent pauses to search for words. [elsevier.es]

  • Perseveration

    With time, the discourse of individuals with AD becomes impoverished and fragmented, characterized by a lack of coherence, tangentiality, and perseverations. C. [quizlet.com]

    […] empathy; narrowed preoccupations; parsimony Behavioral changes with early preservation of social skills; late behavioral changes similar to behavioral variant frontotemporal dementia Speech and language changes with altered speech output; echolalia†; perseveration [aafp.org]

    There was also a reduction in perseveration (the repetition of a particular response, such as a word, phrase, or gesture, despite the absence or cessation of a stimulus), echolalia (the automatic repetition of vocalizations), and verbal stereotypies [life-enhancement.com]

    Behaviorally, patients often exhibit apathy including decreased motivation and withdrawal, impulsivity and perseveration, an inability to switch tasks or change topic. Depression is also common. [rarediseasesnetwork.org]

  • Dystonia

    These are characterized by involuntary movements such as tremor (a rhythmic involuntary movement of a limb or the head), myoclonus (a sharp, non-rhythmic, jumping movement of a limb or the body), or dystonia (a cramping, involuntary clenching or extending [ftd.med.upenn.edu]

    The main clinical symptoms of CBD are asymmetrical rigidity, apraxia, cortical dysfunction (alien limb phenomenon, cortical sensory loss, myoclonus), and basal nucleus dysfunction (limb dystonia, bradykinesia, tremor). [synapse.koreamed.org]

    Dystonia and myoclonus may respond to muscle relaxants or anti-seizure medications. Memory and behavior problems may or may not respond to treatments, such as donepezil, for Alzheimer's disease. [rarediseasesnetwork.org]

  • Clumsiness

    […] in a single disease spectrum of taupathy. 6 CBD is characterized by the following clinical symptoms: asymmetrical parkinsonism, alien limb, cortical sensory loss, apraxia, and myoclonus. 7, 8 Parkinsonian signs such as asymmetrical rigidity (57%) and clumsiness [synapse.koreamed.org]

    This latter deficit is called apraxia and may be confused for clumsiness or weakness. There may be difficulties in completing specific tasks, such as opening a door or brushing one’s teeth or using tools, like a can opener. [rarediseasesnetwork.org]

  • Bradykinesia

    The main clinical symptoms of CBD are asymmetrical rigidity, apraxia, cortical dysfunction (alien limb phenomenon, cortical sensory loss, myoclonus), and basal nucleus dysfunction (limb dystonia, bradykinesia, tremor). [synapse.koreamed.org]

    […] of inhibition, abnormal motor behaviour, and eating disorders. 38 Extrapyramidal signs in the initial neurological examination are more common in the agrammatic and logopenic variants; they rarely appear in the semantic variant. 31,39 Specifically, bradykinesia [elsevier.es]

[…] 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]

Effects of treatment for sound errors in apraxia of speech and aphasia. Journal of Speech, Language, and Hearing Research, 41, 725–743. Google Scholar Wilson, S. M., Henry, M. L., Besbris, M., Ogar, J. M., Dronkers, N. [jslhr.pubs.asha.org]

Te implementation of the treatment, along with the immediate results post-treatment and after one month of follow-up are described. [redalyc.org]

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]

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]

Neuroimaging (magnetic resonance imaging) usually demonstrates focal atrophy in addition to excluding other etiologies. [aafp.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]

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]

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]

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]

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]