Case 14 – Speechless at First Sight

Abstract

A 66-year-old right-handed man presented to the memory clinic with a complaint of decreased concentration.

Case 14 Speechless at First Sight

Marie-Pierre Thibodeau and Fadi Massoud

14.1 Case History

A 66-year-old right-handed man presented to the memory clinic with a complaint of decreased concentration.

He was of Portuguese descent, highly educated and was retired from employment in airport security. He spoke three languages fluently. He lived with his second wife and was active and independent in his activities of daily living. His wife had always been responsible for house chores, but the patient managed his finances and drove without major difficulties. He had however been released from his job for unclear reasons 5 years earlier.

Past medical history only included benign prostate hypertrophy and recent surgery for inguinal hernia. He had no personal or family history of neurological or mental conditions. There was no history of alcohol or drug abuse.

The patient’s spontaneous complaint was of decreased concentration while reading for the past 3 years. Upon further questioning it was found that he had difficulty interpreting what was on the paper; he needed to turn his head in a specific position to read and got easily tired. His attention and memory were preserved when he managed to read adequately. He also drove more slowly for fear of not reacting fast enough to upcoming visual obstacles. He was reported driving at 30 km/h on small roads because of this fear of hitting a child or an animal. His wife described symptoms of simultagnosia, the patient having difficulties finding objects in cluttered spaces such as the refrigerator or cupboard. He also reported problems putting a key in a keyhole or pouring wine in a glass. He had no disorientation in space or time, and his memory was globally preserved. At the time of the first evaluation the patient reported also some very mild word finding difficulty that gradually increased on follow-up. He was also described as being more anxious without any other behavioral change. He described his vision as being normal. He had no hallucinations or delusions, and no other neurological symptoms were reported.

14.2 Physical Examination

Throughout history and physical examination the patient was eager to perform, anxious and overly appreciative of the care he was given. On physical examination significant difficulties copying finger movements on visual cues, but not on verbal cues were noted. He easily pretended to comb his hair or brush his teeth, but was unable to imitate the examiner taping his fingers or making hand signs. He had agraphesthesia without astereognosia bilaterally. Cranial nerves, strength, reflexes, and cerebellar function were normal. He had no parkinsonism or gait problems.

14.3 Cognitive Examination

On cognitive examination, visuoperceptual difficulties were mainly noted. He was unable to copy the pentagons on the Mini-Mental State Examination (MMSE) and his clock drawing was inadequate. While describing the cookie jar figure he was unable to see the global picture or to identify specific objects because of visual interference. The Navon letter test was abnormal. He had right-left agnosia, dyslexia, and dysgraphia. At first, mild anomia was noted and seemed mainly related to his visuoperceptual problems. He had no speech apraxia, dysarthria, agrammatism, or semantic loss. Memory was spared.Neuropsychological examination revealed simultagnosia, left hemi-inattention, left-right distinction vulnerability, construction and ideomotor apraxia, alexia, agraphia, lexical access problems, and decreased executive functions with preserved verbal memory and behavior.

The patient was sent for an ophthalmological examination that revealed right homonymous hemianopsia.

A brain scan was performed and showed mild atrophy and leurcoaraiosis without any mass. A PET scan was also performed and revealed severe parietal hypoperfusion bilaterally more on the left than the right and mild temporal hypo metabolism bilaterally L>R (Figure 14.1).

Figure 14.1 [¹⁸F]FDG PET study. Transaxial (a), coronal (b), and sagittal slices (radiological orientation, right side of subject on the left side of the figure) showing severely diminished metabolism in parietal and occipital regions (left worse than right), with mild extension into the posterior region of the left temporal lobe. Prefrontal associative cortices remain normal. The posterior portions of the cingulate gyri also show normal distribution (c). 3D-SSP representation confirms that pattern (d).

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