Abstract
EEP is a Brazilian, 68-year-old, right-handed man, with 15 years of schooling (graduated in business school). He is married and has a daughter and a son.
15.1 Clinical History – Main Complaint
EEP is a Brazilian, 68-year-old, right-handed man, with 15 years of schooling (graduated in business school). He is married and has a daughter and a son.
Now retired, he had worked for many years in his own real estate business. His main work-related activities involved sales and purchase of land, which were accomplished with the help of an office assistant.
At the age of 63 his wife noticed that at times when trying to express himself, EEP presented with difficulties characterized by pauses and stuttering episodes. Although this fact had been pointed by her to him, initially EEP did not care enough for these symptoms and did not find it necessary to seek medical advice. Over the following 3 years symptoms have gradually become worse and in some occasions his speech was blocked and word-finding difficulties also emerged. At that time, he decided to seek medical advice. His main complaint was about speech production, characterized by fluency problems (pauses and prosodic changes, including hesitations and stuttering). His wife also had noticed word-finding difficulties, including reading and writing activities.
Despite those speech and language problems EEP continued working (with the help of his assistant), driving a vehicle properly, and did not present with memory or any other cognitive complaints. Functional performance in activities of daily living was preserved except for those involving language. Social activities had been gradually reduced as expression problems became worse.
15.2 General History
The patient was healthy, with no reported comorbidities. He had no history of developmental learning or language problems, previous psychiatric or neurological problems. In his free time, he had a busy social agenda, engaging in several meetings with friends and also in sport activities. He had been a nonprofessional athlete for over 30 years, did not smoke, and had healthy diet habits. His mood was good at that first evaluation, and no behavioral changes could be emphasized. Depressive symptoms emerged just when the patient become aware of his difficulties, in a moderate stage of impairment.
15.4 Examination
15.4.1 Neurological Exam
His neurological exam was normal, without motor or sensory deficit or equilibrium impairment. Only cognitive impairment was detected.
15.4.2 Neuropsychological Evaluation
EEP scored 27/30 in the Mini-Mental State Examination (MMSE), 12/18 in the Frontal Assessment Battery, 11/30 in forward and backward digit span (WAIS III battery) and 49″ and 144″ in the Trails test parts A and B, respectively.
Combined, those neuropsychological tests pointed to a mild impairment of executive functions, including phonological short-term memory and working memory difficulties. Other cognitive functions were preserved. He was oriented in time and space (MMSE), nonverbal episodic memory tests were within the normal range and autobiographical memory also seemed preserved. According to his wife, he was independent and functional for basic and instrumental activities of daily living, except for more complex administrative and financial tasks, in which he showed a progressively increased need for assistance with more marked difficulties appearing in the previous year (2 years after the onset of speech symptoms).
15.4.3 Speech and Language Assessment
Speech and language was assessed in a functional communicative evaluation (semi-structured interview) and also with formal tests. In the functional situation, auditory comprehension was quite well preserved as well as pragmatic resources (suitable communicative exchange shifts, maintenance of eye contact and nonverbal communication). On the other hand, oral production was marked by anomia, word-finding difficulties, and large quantity of pauses between words and sentences. Moreover, there was important output reduction, characterized by short and simplified sentences with omission of verbs and function words (i.e., prepositions, conjunctions, etc.), as well as substitutions of verb inflections. Speech was effortful, slow, and hesitant. Occasional phonemic paraphasias (omission and substitutions of phonemes) were also observed. Mild prosodic changes (intonation, stress, and rhythm) were also present. The results of the main language tests employed in the formal speech and language assessment are as follows (Table 15.1):
– Oral and Buccofacial Praxis: The patient had no difficulty with nonverbal agility, however he manifested mild apraxia of speech in tests of verbal agility and diadochokinesia test.
– Oral Naming: EEP produced spontaneously 35/60 correct responses in the Boston Naming test. His responses suggested problems in lexical access with preservation of semantics. He was able to provide definitions of items he was unable to name and in most of them, when given phonemic cues (i.e., the first phoneme or syllable) he was able to produce the correct name.
– Verbal fluency was markedly impaired both for semantic category (animals) and for letters (FAS), although letter fluency impairment was more severe (only two items in 3 min).
– Repetition: The ability for short and frequent sentences was preserved. Word repetition was preserved except for longer multi-syllabic words. Phonological errors as well as word omission or semantic substitutions were observed in the repetition of low frequency and long sentences.
– Oral picture description: Speech and language production difficulties were evident in the oral discourse production task of Boston aphasia assessment (cookie theft picture). In this task, EEP presented telegraphic speech, that is, he was unable to produce long and complete sentences and expressed mainly through content words. Verb production was markedly reduced (only three verbs) and errors in verb tenses (past participle used instead of gerund) were also observed.
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