With the above pharmacological treatment caveats in mind, an interesting approach could be the implementation of non-pharmacological interventions. In spite of lacking definite positive results, at least, in our understanding, they constitute beneficial procedures to deal with the behavior changes encountered in patients, to support their engagement in activities and to ensure an optimal quality of life. Such approaches must include caregiver and family education. In fact, neuropsychiatric changes are known to be more stressful to caregivers than cognitive or functional decline [74, 75]. Recent evidence from our group has shown that the way caregivers cope with these behavior changes influences caregivers’ burden and depression . As such, personality, as an individual resource of the caregiver, must be taken into account in the planning of intervention programs. Also environmental, behavioral, and physical interventions designed to minimize the occurrence and consequences of undesired behaviors may be introduced. Additional helpful cognitive-based interventions in PPA also include speech and language therapy (SLT). We addressed this issue in a study aimed to assess the effect of SLT in the mitigation of language deficits in PPA. The results suggested that the implementation of a language-based intervention in this syndrome might attenuate the progression of naming deficits . An appropriate treatment plan, encompassing this type of intervention and psychosocial education and support can be outlined for both patients and their caregivers with better knowledge of the behavior changes associated with PPA. The emergence of behavior changes, particularly throughout the disease course, can have a significant impact in the optimization of the communication and may interfere with the patient’s ability to comply with the intervention procedures and the established goals. As such, an effective management of the behavior changes, either through behavioral-targeted interventions or a pharmacological approach to (at least) some of these symptoms, may maximize the potential benefit of an SLT program.
Finally, the implementation of trials of putative disease-modifying therapies may also benefit from measuring neuropsychiatric symptoms, in addition to language, as an indicator that an intervention can slow the progression or ameliorate the symptoms of patients with PPA.
Case 1: An Example of a Semantic Variant PPA
A 62-year-old woman, without relevant personal or family history, was referred to our clinic with a one-year history of loss of vocabulary and inability to name common objects. According to her family, she used frequently generic terms to refer to common entities (e.g., animal for dog, bird, etc.; document for bill or letter). Despite these language problems, she showed no signs of decline in other areas of cognition and had complete autonomy for instrumental activities of daily living: she used to drive every day to pick up her grandchildren at school; in the previous year, she had successfully sold a property. The neurological examination was unremarkable. MRI showed a severe atrophy of both temporal lobes, more marked on the left and with enlargement of the ipsilateral Sylvian fissure. The neuropsychological assessment revealed core language deficits, with the patient showing a fluent, well-articulated, slightly empty speech with occasional semantic paraphasias and severe object- and picture-naming deficits, but preserved word, pseudo-word, and sentence repetition. Auditory comprehension for simple and complex commands was within normal limits, but she showed a marked impairment on semantic processing (low scores on vocabulary and associative matching). She presented regularization errors on the spelling of irregular words (surface dysgraphia). The remaining cognitive functions were within normal limits albeit a slight decrease in planning and problem-solving abilities. A diagnosis of semantic variant of primary progressive aphasia was established. In the clinical interview and throughout the assessment, the patient evidenced a reduced self-awareness of her deficits and some obsessive behaviors such as clock watching and handling constantly her glasses. According to the information obtained with the caregiver, the patient showed reduced mental flexibility, inattention, diminished emotional control (increased irritability), poor judgment, inconvenience in social situations, and mild hyperactivity. A year after the first assessment, her naming and semantic processing deteriorated, and behavior changes previously reported increased in frequency and severity, particularly obsessive-compulsive behaviors. These behavior changes have been affecting her treatment compliance: she now refuses to take the prescribed medication and cognitive stimulation sessions (that she had initiated the previous year) and does not accept the help of relatives, arguing that she’s perfectly able to take care of herself and her life. She also continues to drive, despite obvious deterioration of her driving skills, noticed by some family members.
This case illustrates how early subtle behavior changes may anticipate future problems and the need for early management by the clinician and the patient’s caregivers.