Adults Cognitive Impairments

Primary progressive aphasia (PPA) is a type of cognitive impairment characterized by the progressive degradation of speech and language abilities. It manifests as a result of nerve cell death in the brain region that controls speech and language. However, such nerve cell loss is not triggered by a stroke, head trauma, infection, or malignancy. PPA causes language impairment for two years before other cognitive skills deteriorate. The most noticeable clinical sign is difficulties or loss of verbal function (aphasia) (Gorno-Tempini et al., 2011). Language difficulties lead to a reduction in the patient’s level of activity. Diagnosis of PPA is unique because its symptoms cannot be accounted by others as degenerative nervous or medical disorder. Besides, it cognitive disturbances cannot be considered by a psychiatric diagnosis. Also, there is no frank initial episodic memory, visual memory, and visuoperceptual impairments. It also does not have any apparent initial behavioral disturbances. This paper aims to cover the impact of PPA on cognitive and language function, the assessment and management of PPA, and the consequences of the syndrome on patients if not managed.

PPA Variants

The pattern of language impairment and patterns of atrophy on imaging indicate that the PPA syndrome has a prevalence of 2-15/100,000 individuals. Besides, the victims of the disorder can survive up to 7 years. However, that depends on the time when the diagnosis is done and the type variant (Sapolsky et al., 2011). The syndrome begins mostly at the late 50s (Grossman, 2014). It estimated that only 75-80% of individuals diagnosed of PPA receive a reliable diagnosis (Nickels & Croot, 2014). PPA disorder is closely associated with Alzheimer’s disease. About with 30%-40% of individuals diagnosed with PPA are usually having Alzheimer’s disease (Bettcher & Sturm, 2014). Besides, PPA is caused by Frontotemporal Lobar Degeneration (FTLD). It is estimated that around 60%-70% of individuals with the disorder are having PPA. PPA disorder could occur due to many factors. One such driver is genetics through mutations. Secondly, the syndrome can occur as a result of focal structural lesions. It can appear due to the late manifestation of development or genetic weakness of language (Rogalski, Weintraub, & Mesulam, 2013). Thirdly, PPA can happen due to learning disability caused by developmental dyslexia. PPA is divided into three variants, namely, agrammatic/nonfluent (nfv PPA), Semantic variant (svPPA) and Logopenic variant PPA (lvPPA) (Gorno-Tempini et al., 2011).

Agrammatic/Nonfluent (nfv PPA)

Nfv PPA patients use short and simple phrases and tend to omit function words. They also tend to have effortful and halted speech (Gorno-Tempini et al., 2011). Such speech tends to slow labored, distorted, disrupted and may have some part deleted or substituted. The secondary features of this variant of PPA include impaired comprehension of complex sentences, spared single word comprehension, spared object knowledge. It is mainly caused by the degeneration in the front lobar temporal (FLTD). Nfv PPA cognitive factors include mild to moderate difficulty in executive function tasks, difficulty with tests of set-shifting and visuospatial processing and verbal episodic memory (Bettcher & Sturm, 2014). Clinical observation of Nfv PPA requires intact social skills, aware of performance on tasks and speech production Mild disinhibition and apathy as the disease progresses.

Logopenic (lv-PPA)

Lv PPA patient normally demonstrates impaired word retrieval characterized by phonological paraphasias, slow rate, and frequent pauses. They also tend to have a high repetition of sentences and phrases (Gorno-Tempini et al., 2011). Some secondary features of this syndrome include Speech errors in spontaneous and naming, spared single word comprehension and object knowledge, spared motor speech, and absence of frank agrammatism characterized by halting but due to word finding difficulties. It is caused mainly by Alzheimer’s disease and in some cases FLTD. Besides, lv-PPA cognitive symptoms include mild visuospatial deficits like localizing and constructing, difficulties in phonological working memory and impairments in numbers and complex calculations (Bettcher & Sturm, 2014). Lv PPA Patients may display apathy, anxiety, and mild irritability and show awareness of difficulties during clinical observation.

Semantic (svPPA)

Semantic patients tend to have impaired naming of objects due to circumlocution, simplification or semantic paraphasia (Mesulam et al., 2014). They also demonstrate impaired single word comprehension characterized by reduced frequency items and problems in object and person recognition. Some secondary impact of the syndrome to the patient includes impaired knowledge of object especially for those objects that are less familiar, surface dyslexia or dysgraphia, spared repetition and spared speech production. It attacks the anterior temporal lobe of the brain. Like the other variant of PPA, Semantic is caused by FLTD. Patients with the disorder experience difficulties in the verbal area in episodic memory (Bettcher & Sturm, 2014). Once it attacks a patient, there are changes in social and emotions.

Assessment

Mrs. LP experiences slowed or halted speech, unusual word order, hesitation, mild impairments in auditory and reading comprehension speech and language, word comprehension, repetition, writing and reading skills and cognitive functions. Mrs. LP condition is genetically inherited and has worsened to appoint that it frustrate her. The process of assessment will cover the history of Mrs. LP, neurological examination, neuropsychiatric and Neuropsychological evaluation and rain imaging (Nickels, Taylor & Croot, 2011). The primary aim of evaluation is to determine whether Mrs. LP is suffering from PPA. It will also intend to determine whether there is reduced speech and utterance than under normal condition. Besides, the assessment will cover cases of articulatory planning impairment leading to dysfluency and errors in speech production (Nickels, Taylor & Croot, 2011). These main areas of assessment are discussed below.

Speech and Language Evaluation

The first assessment on Mrs. LP would be speech and language. Assessment of speech and language involves syntax and grammar and the motor aspects of speech. Assessing Mrs. LP grammar and syntax requires the use of picture descriptions, story retells, analysis of language sample, and constrained syntax production activities (Gorno-Tempini et al., 2011). The grammar and syntax assessment aims to determine the speech rate, fluency, grammatical errors, hesitations, recalls among other variables in Mrs. LP (Harciarek et al., 2014). The process employs seven-point rating scale to measure various aspects of speech characteristics. It also uses Northwestern Anagram Test to measure sentence production. Besides, Grammatical Competence and Paraphasias of the patient is assessed. In this case, the ability of Mrs. LP to converse in grammatically correct sentences and paraphrasing is examined (Sapolsky et al., 2011). Determining the motor aspect of speech involves analysis of Mrs. LP fluency and language functions (Thompson & Mack, 2014). The evaluation process employs such tools as seven-point rating scale, Boston Diagnostic Aphasia Examination (BDAE) for testing various perceptual modalities, length scale, and Western Aphasia Battery (WAB), picture and speech description. Through, the assessment, the presence PPA in Mrs. LP can be ascertained. In the process of evaluation, the variant of PPA is also determined.

Word Comprehension

Assessment of the Mrs. LP word comprehension involves looking into the single word and sentence comprehension and the semantics. Under the single word comprehension, the aim is to determine the ability of Mrs. LP to produce a complete word. Failure of word comprehension could indicate that PPA is likely to be present. In this evaluation, various tools are employed. These tools include BDAE Word Comprehension, Peabody Picture Vocabulary Test, Spoken Word-Picture Matching and WAB Auditory Word Recognition. The objective of assessing sentences is to determine grammar in the patient (Mrs. LP). The presence of too many errors, for example, failure to follow subject- verb agreement or omit the needed determiners may indicate a like hood of PPA (Harciarek et al., 2014). The organization of thoughts in a sentence involving proper use of object names and features is crucial in this evaluation stage. Like in the word assessment, BDAE and WAB tools are employed. Other tools used in evaluating the client sentences are CYCLE, Sentence Picture Matching, and PAL Sentence Comprehension.

Furthermore, assessing the patient’s semantics aims to determine the logical arrangement or ideas or thoughts in sentences during conversing. PPA patients always depict weak semantic characterized by disjointed sentences that do not make sense. The tools used in this level include PALPA spoken-word picture Matching, Peabody Picture Vocabulary Test, PALPA synonym judgments and Pyramids and Palm Tree test (Henry, Mooney, & Morhardt, 2015; Nickels, Taylor, & Croot, 2011). Another area that would need to be assessed includes repetition of sentences, phrases and words using the BDAE and WAB tools. Besides, the ability of Mrs. LP to name object correctly would needed be examined (Sapolsky et al., 2011). Finally, the ability of Mrs. LP to read and write would be assessed using similar tools like those in repetition but involving written assignments.

Cognitive Evaluation

The cognitive assessment includes the perceptual motor function, language, learning and memory, complex attention, social cognition and executive function. In the perceptual motor function, a therapist will examine Mrs. LP visual perception, coordination, and visuo-constructional reasoning. Any defect in these areas may indicate symptoms of PPA (Rogalski, Weintraub, Mesulam, 2013). The patient ability to name objects, fluency, grammar and syntax, word finding and use of receptive language is also considered under this evaluation (Thompson & Mack, 2014). Just like in the perceptual motor function, deficiency in language can be a symptom of the PPA syndrome. Under the learning and memory, the therapist would aim to determine how Mrs. LP has a free recall, cued recall, recognition memory, implicit learning and long-term memory.

However, under executive function, the therapist will attempt to examine Mrs. LP decision making, planning, response to feedback and flexibility. Deficiency in these areas is likely to be a symptom of PPA. Other aspect mentioned that are vital include the examination of the patient to recognize her emotion, the level of attention and procession speed of the patient. In the cognitive assessment, the therapist would employ such tools as Cognitive Linguistic Quick Test, Digit Span Backward, Trail Making Test and Mini-Mental State Exam Digit Span Forward. Therapist frequently longitudinal monitoring to examine PPA and this involve the use of Progressive Aphasia Severity Scale. The scale allows the therapist to carry out language assessment and interview the care partner and patients.

Speech and Language Treatment

Promising treatment effect, especially for participants with mild PPA may not be generalized but rather maintain language abilities (Rising, 2014). Treatment of Mrs. LP condition aims to help Mrs. LP improve her capacity to communicate, restore linguistic and cognitive abilities as much as possible and to coach family and care staffs on how to handle the patient (Henry et al., 2013). The short term objective of the intervention is to help Mrs. LP hear using assistive technologies. It will also help Mrs. LP to find words. In the long term, the treatment and management initiative will aim at helping Mrs. LP hear, organize words correctly and to be able to communicate with others and especially the grandchildren and husband fluently. The treatment will include language Retraining, compensatory strategies, partner training and combined approaches (Harciarek et al., 2014). Learning training will include self-cueing approaches, script training and motor sequence strategies aimed at improving Mrs. LP speech and pronunciation of syllables. The compensatory plan would require the use of other devices such as iPad and communication partners supports. Partner training approach would aim at teaching the effective way of communication to Mrs. LP. It will also improve her quality of life by enhancing her participation in activities and offer her education on the disorder.

There are many specific interventions that a therapist can apply on Mrs. LP to improve their communication. Since she has word finding difficulties, the therapist can combine pictures with gestures to enhance her sentence production (Schneider et al., 2009). The picture would also assist the patient to communicate since she has auditory issues. Besides, the therapist can produce personalized recordings. These would help her recalling scenes and events and in word organization. Another strategy is the use of recall strategy. These approaches include mnemonic, cueing, chunking, loci and space retrieval. The approach entails linking of stories, poems, numbers, and photographs to what the patient should remember. In this case, Mrs. LP would be assisted to easily find words and memorize important items (Rising, 2014). An example of recall approaches includes face-name, number and story recall.

The therapist can also improve the Mrs. LP fluency through training. This involves the use of picture which has texts. Once these images have been shown to them, they are allowed to name or state what is written on them the strategy help to improve Mrs. LP fluency. Mrs. LP seems to have semantic impairment due to her inability to talk and to organize the word (Khayum, et al., 2012. The therapist could carry out repetitive rehearsals involving naming objects, words or concepts using their pictures. Since Mrs. LP has auditory issues, it would be essential for the therapist to use assistive communication technologies such as communication aid to help her hear. Finally, the therapist could coach the husband on ways to communicate with her or to improve her communication as discussed (Rising, 2014).

The PPA disorder would affect the ability of Mrs. LP to contact her grandchildren, friends and worse with her partner. Lack of effective communication can result in isolation, loneliness, anxiety and even depression. Thus, the disorder is likely to generate other illness such as depression and high blood pressure (Tsapkini et al., 2014). Besides, her abnormal word order in speech may make her lose her job because it may be difficult to communicate with her employer effectively. Failure to control the syndrome may completely affect her emotions. She may feel frustrated, valueless and unworthy compared to other people. This can further affect her interaction with others.

However, through collaboration between SLT and family or multidisciplinary team, Mrs. LP can be assisted to avoid these consequences. The multidisciplinary team consisting of a range of specialists such as a psychiatrist, pediatric, and clinical personnel could ensure that Mrs. LP receives all-round treatment, necessary for the condition. These different experts would ensure that she is well taken care of according to her needs. Besides, the collaboration between STL and the family members, such as the grandchildren, would ensure that Mrs. LP receives the best attendance available. She can continue to receive her medication through such collaboration. The SLT can help her continue to improve her speech and grammar despite the effect of the PPA syndrome.

References

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