Primary Progressive Aphasia.docx

SHORT PAPER 2

















Primary Progressive Aphasia
Darryl Lynn Jones, MAEd., M.S.
Southern New Hampshire University
September 6, 2015













Primary progressive aphasia (PPA) encompasses degeneration of cerebral functioning. Multiple aphasic disorders, such as transcortical sensory aphasia caused by isolated damage to the posterior language area, contribute to its progression (Carlson, 2013; Harciarek & Kertesz, 2011; Hsu, Chen & Chiu, 2004). For example, "People can repeat what other people say to them…" (Carlson, 2013, p. 341). "However they cannot comprehend the meaning of what they hear and repeat, nor can they produce meaningful speech of their own" (Carlson, 2013, p. 341). It originates from cerebral trauma and can be isolated to more than one segment of the brain. Although the definition of aphasia excludes escalation of brain damage due to genetics or non-trauma related wounds; research is finally moving toward encapsulation of the term and its multitude of characteristics (Harciarek & Kertesz, 2011; Hsu, Chen & Chiu, 2004). Damaged cerebral regions, traumatic causes, potential impairments, observed deficits, and interventions for geriatric as well as juvenile patients comprise this exploration.
Neuroanatomy
Harciarek and Kertesz (2011) describe neuroanatomical associations with atrophy of brain sections with the onset of aphasia. It had not been connected to deficits without direct brain injury until recently and had been a neglected subject until the early 21st century. Researchers cast aside apathy and delved deeper into the phenomena as technology and possible remedies became real possibilities. Magnetic resonance imaging (MRI) is a breakthrough approach for localization of veiled brain injuries and cognitive deficits (Bigler, 1996; Braga, Souza, Najjar & Dellatolas, 2007).
Several areas of the brain, such as the frontotemporal, left posterior fronto-insular region including the "…inferior frontal gyrus (Broca area), insula, premotor and supplementary motor areas"; have been observed as localized segments of injury and degeneration specific to multiple aphasic types (Harciarek & Kertesz, 2011). Primary progressive aphasia and its derivations continue to be challenged about whether or not manifestation occurs due to a motor or non-motor impairment (Harciarek & Kertesz, 2011, p. 274).
Expected Deficits
Harciarek and Kertesz, (2011) expect, "…reading difficulties, although these problems are typically mild and phonemic in nature (Patterson et al. 2006; Rohrer et al. 2010a)", and visual issues. "By comparison, writing is often markedly impaired, with a vast number of grammatical errors (Graham et al. 2004)" (Harciarek & Kertesz, 2011). Suffice it to say, primary progressive aphasia develops from more than one injured cerebral area and promotes synaptic impedance toward synaptic pruning with permanent loss of cognitive functioning (Harciarek & Kertesz, 2011; Hsu, Chen & Chiu, 2004). The ability to speak, read, write, and associate stimuli suggests degeneration upon the presence of symptoms. For example, ocular dysmetria, sticky fixation, simultagnosia, amnesia, ideomotor apraxia, visual-constructional problems, and deficient verbal fluency comprise a short list of deficits across age groups (Harciarek & Kertesz, 2011). The onset of amnesia also suggests attention deficits relative to awareness of the ailment's presence by the afflicted person and related to soci-emotional and comprehension impairments (Harciarek & Kertesz, 2011). In other words, the patient continues to believe cognitive abilities are not impaired; thus proceeds with daily activities despite marked display of reduced capacities.
Children with PPA display attention deficit and hyperactivity disorder. The inability to retain data, incoherent expression of frustration, and inadequate visual capacity exacerbates the need to focus on the easiest soci-academic tasks; while appearing to be lackadaisical or aloof to activities outside of one's immediacy. Furthermore (Harciarek & Kertesz, 2011, p. 274), "…extrapyramidal features of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP),… (Kertesz et al. 2000, 2005, 2007; Rohrer et al. 2010b)", further earmark its degenerative impact. "In some cases…, progressive limb apraxia can be a prominent feature (Fukui et al. 1996; Rohrer et al. 2010c),…" (Harciarek & Kertesz, 2011).
Interventions for Children with the Ailment
Despite the horrific cognitive impact of PPA, children with it must be micro-supervised and provided highly interactive curriculums. Computer-based learning and "flipping" the classroom can have positive effects. Physical activity must be limited to groups of children with similar challenges; while recreational locations need high-end security with multiple caretakers to ensure reduced probability of additional injury.
Moreover, early stages of child development may display listlessness, fussiness, and lack of responsiveness to stimuli. As the child grows and cerebral degeneration persists, visual impairment and retarded language capacity will emerge. Once the child reaches elementary and middle school, there will be social issues and soci-emotional deficits which will contribute to anxiety, alienation, depression, and aggression toward objects; including proximal property, animals, and eventually oneself and others. If the child is not directed toward medical and soci-psychological therapy, then degeneration will continue at its current rate. Eventual vegetative consequences, pursuant to the combination of limb apraxia, language development deficits, and visual-constructional issues will root (Bigler, 1996); Braga, Souza, Najjar & Dellatolas, 2007). In other words, image recognition, control of eye and limb movements, memory impairment, and other motor limitations characterize sufferers (Bigler, 1996; Braga, Souza, Najjar & Dellatolas, 2007; Carlson, 2013; Harciarek & Kertesz, 2011; Hsu, Chen & Chiu, 2004). Although corticobasal degeneration (CBD), is most common among people over 40 years old, and progressive supranuclear palsy (PSP), better known as Parkinson's Disease, is more common among people over 60 years old, the chance of a child's brain injury causing similar symptoms, escalating to PPA, does exist (Bigler, 1996; Braga, Souza, Najjar & Dellatolas, 2007; Harciarek & Kertesz, 2011; Hsu, Chen & Chiu, 2004). It would eventually incapacitate the child to the point of being required to have a full-time attendant and a wheelchair around the clock (Bigler, 1996; Braga, Souza, Najjar & Dellatolas, 2007).
.
















References
Bigler, E. (1996). Brain imaging and behavioral outcome in traumatic brain injury. Journal of Learning Disabilities. 29(5). 515-530. Retrieved from ldx.sagepub.com
Braga, L., Souza, L., Najjar, Y. & Dellatolas, G. (2007). Magnetic Resonance Imaging (MRI) findings and neuropsychological sequelae in children after severe traumatic brain injury: The role of cerebellar lesion. Journal of Child Neurology. 22(9). 1084-1089. DOI: 10.1177/0883073807306246
Carlson, N. (2013). Foundations of behavioral science. 9th edition. Boston, MA: Pearson Education, Inc. Retrieved from http://view.ebookplus.pearsoncmg.com/ebook/launcheText.do?values=bookID::13672::pl
Harciarek, M. & Kertesz, A. (2011). Primary progressive aphasias and their contribution
to the contemporary knowledge about the brain-language relationship. Neuropsychology Review. 21. 271–287. DOI 10.1007/s11065-011-9175-9
Hsu, J., Chen, W. & Chiu, H. (2004). Cortical sensory loss in a patient with posterior cortical atrophy: A case report. Neurocase. 10(1). 48-51. Retrieved from http://pn8vx3lh2h.search.serialssolutions.com/?ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-
Southern New Hampshire University. (2015). Module eight: Language and communication. Retrieved from www.mysnhu.edu
University of British Columbia. (2014). Anatomy of the brain. Retrieved from http://www.neuroanatomy.ca/stroke_model/mca_info.html



Running head: SHORT PAPER 1