Case 18 – A Meaningless World

Abstract

A 54-year-old right-handed highly educated French-speaking father of three was referred for a neurological consultation for “some memory difficulties and behavioral changes” for the past 2 years. His past medical history was unremarkable and no learning disabilities were reported. He denied alcohol and drug abuse and he did not smoke. His father and two of his paternal aunts had been diagnosed with late-onset Alzheimer’s disease. He was concerned by the possibility of being affected by the same neurodegenerative condition.

Case 18 A Meaningless World

Paolo Vitali and Simona Maria Brambati

He reported increasing difficulties in remembering names, such as object and proper names, popular films, or familiar places, for example, the names of the ski trails at the resort where he had worked for a long time as an associate director. He was recently forced to quit his job, one of the reasons being that he seemed unable to understand what his colleagues were saying. Moreover, he forgot the meaning of the items on his shopping list and he needed pictures to pick up the right items at the grocery store. He also reported new difficulties in recognizing people and often questioned whether he had already met the person that was in front of him. He began to forget how to cook and had to be supervised when trying to remember previously known recipes and procedures. He became more confused concerning arithmetical facts and had difficulty understanding and distinguishing numbers (e.g., 200 – 2,000 – 200,000) and with orthography (word spelling). He occasionally had trouble paying attention to his interlocutor because he was focused on trying to remember the appropriate word to say, and the family also noted difficulty with word comprehension as well as with following group conversations. Sometimes he appeared “absent” and indifferent. He needed more encouragement with respect to activities of daily living (cooking, shopping, going out, etc.). However, he was still able to problem solve and fix things, like changing a bicycle wheel, for example. He also continued to be physically active and went to ski regularly.

He tried to cope with his progressive difficulties by keeping a diary in which he noted several specific items belonging to different general categories (e.g., furniture, fruits, vegetables, etc.) as a tool to be referred to in case of a forgotten name. He could remember that the specific item he was looking for was on the list and could find it among others. Nevertheless, he admitted that sometimes he put an item on the list and then forgot what it was for. He also tried to retrieve words by adopting a self-cueing phonological strategy. In fact, sometimes he could remember the first syllable of the word, which was then used as a cue to enhance access to the lexical target. However, he found irregular French words challenging.

On examination, his oral spontaneous expressive language was fluent with a speech rate that was generally high, well-articulated, and grammatically appropriate. However, his speech quality was mildly reduced due to word-finding difficulties (anomia). Although rare, he produced syllable substitutions when generating less frequent words, which resulted in occasional pseudo-words (phonological paraphasia). Oral comprehension was adequate in a familiar environment. Yet, more complex explanations were difficult to follow. The remainder of his neurological examination was normal, including the absence of frontal release signs, parkinsonism, or alien-limb phenomenon.

On further cognitive testing (Table 18.1), he scored 23 out of 30 on the Montreal Cognitive Assessment (MoCA). Executive functions and visuospatial abilities were well preserved. Working memory was only mildly impaired. An apparent dissociation was observed between a preserved visuospatial episodic memory – the delayed recall of the Rey Complex Figure – and an impaired verbal episodic memory. The latter was assessed using a list of 16 words to remember across 3 learning series. Despite a defective performance on free and cued recall and an absence of a learning curve after the 3 repetitions, delayed word recognition was within the normal range with 14 out of 16 correct recognitions and no false intrusions. This finding argues against the hypothesis of an impaired storage mechanism for verbal material in this patient, as usually observed in classic amnestic dementia (e.g., Alzheimer’s disease).

Table 18.1 Neuropsychological and language profiles of svPPA patient

Tests	Scores
Cognitive general functioning
MoCA	23^a
Vocabulary (WAIS)	23^a
Information (WAIS)	8^a
Executive functions
Trail Making Test – A (s)/B (s)	27/76
Working memory
Digit span – forward/backward	4^a/3^a
Visuo-spatial ability
Complex Rey Figure – Copy
Score/Time (s)	34/224
Episodic memory
Complex Rey Figure
Immediate recall: Score/Time (s)	25.5/63
Delayed recall: Score/Time (s)	21.5/55
Gröber and Buschke 16 words
Immediate recall	15
Free recall 1	6^a
Free recall 2	4^a
Free recall 3	4^a
Total recall	15^a
Delayed free recall	3^a
Total delayed recall	3^a
Recognition – true	14
Recognition – false	0
Language
Verbal fluency (90 s)
Phonemic (P)	8^a
Semantic (animals)	6^a
Picture Naming
Boston Naming Test (/60)	5^a
Explicit semantics
Pyramid and Palm Tree Test words (/56)	26^a
pictures (/52)	37^a
Word-to-picture matching: “Repeat and Point test” (/10)	7^a
Mill Hill Synonymous judgment (/34)	11^a
Oral Verbal comprehension
Single word comprehension (Lexis) (/64)	48^a
Sentence comprehension (MT86)	Normal
Written syntactic comprehension
Chapman-Cook Test	2^a
Oral Repetition
ODEDYS Word Repetition Test (/16)	16
ODEDYS Pseudo-Word Repetition Test (/20)	18