Abstract
Mr. C, a 75-year-old man, noticed difficulty remembering names and adding numbers after wakening while on vacation. Although these problems were quite subtle, his wife was puzzled because he seemed to have no difficulty the day before. One week later, he noted that “something was wrong” but could not describe the changes in detail. He felt that his balance was “not right” and experienced difficulty keeping track of his golf scores. Over the next few months, he developed word-finding problems and had difficulty expressing himself. He was very forgetful, had trouble problem solving, was distractible, and was unable to do simple calculations. Whereas he had previously been very reserved, he started talking with others in a more open way than he would normally have done. He continued to complain of balance problems and started to notice changes in his handwriting.
30.1 Clinical History
Mr. C, a 75-year-old man, noticed difficulty remembering names and adding numbers after wakening while on vacation. Although these problems were quite subtle, his wife was puzzled because he seemed to have no difficulty the day before. One week later, he noted that “something was wrong” but could not describe the changes in detail. He felt that his balance was “not right” and experienced difficulty keeping track of his golf scores. Over the next few months, he developed word-finding problems and had difficulty expressing himself. He was very forgetful, had trouble problem solving, was distractible, and was unable to do simple calculations. Whereas he had previously been very reserved, he started talking with others in a more open way than he would normally have done. He continued to complain of balance problems and started to notice changes in his handwriting.
Mr. C was seen by his primary care physician who referred him to a geriatric psychiatrist. He was then referred to a neurologist. Six months after the onset of symptoms, he was seen in our Memory Disorders Clinic. By this time, he had significant difficulty communicating and was unable to take care of his finances. However, he continued to remain independent in basic activities of daily living. His wife experienced challenges in coping with the unexplained changes happening to her husband.
30.2 General History
Mr. C was born in Canada and had 16 years of education. He was married with children. He retired from his position as a high-level executive of a major corporation 10 years prior to presentation. He had a history of migraine-type headaches, hypertension, bladder cancer, skin cancer, and bilateral Dupuytren contractures. He also had a renal mass that was suspicious for a renal cell carcinoma. There was no family history of a neurodegenerative disorder.
30.3 Examination
Neurological examination performed 6 months after onset of symptoms was remarkable for bilateral grasp reflexes. He had agraphesthesia in both hands. There was mildly reduced arm swing on the right. Plantar responses were equivocal bilaterally. He displayed a full range of affect. He had significant word-finding difficulties and difficulty with simple mathematical calculations. Score on the Montreal Cognitive Assessment (MoCA) was 17/30.
30.4 Special Studies
Neuropsychology assessment was completed 3 months after onset of symptoms. This showed impairment in the following domains: auditory and visual working memory; mental tracking and processing speed; arithmetic ability (thought to be related mainly to working memory); and clock reading. Semantic fluency was more impaired than phonemic fluency. He had difficulty with word and memory retrieval.
Speech-language pathology assessment was completed 8 months after onset of symptoms. He was impaired in word finding, naming, reading, and writing with patterns of surface dyslexia and dysgraphia. Semantic knowledge was intact. There was no profile specific to any variant of primary progressive aphasia. Motor speech was intact, i.e. there was no evidence of dysarthria or apraxia of speech.
Single-photon emission computed tomography (SPECT) scan of the brain at 6 months after onset of symptoms showed mildly reduced perfusion in the high left parietal lobe and inferior right temporal lobe. Although magnetic resonance imaging (MRI) at 4 months was normal, repeat MRI at 8 months showed bilateral symmetrical cortical diffusion restriction and T2-fluid-attenuated inversion recovery (FLAIR) signal abnormality in the posterior aspect of both cerebral hemispheres involving the occipital and posterior temporoparietal lobes, as well as the frontal lobes. There was no involvement of the basal ganglia (Figure 30.1). These findings were highly suspicious for Creutzfeldt-Jakob disease (CJD).
Figure 30.1(a–c) Bilateral symmetrical cortical diffusion restriction seen on DWI. This is most pronounced in the posterior region of both hemispheres but also involves the frontal lobes. There is no involvement of the basal ganglia.
Related posts:
Case 3 – A Young Man with Memory and Walking Difficulties
Case 9 – A 59-Year-Old Man with Weakness and Personality Changes
Case 16 – Middle-Aged Man Looking for Words
Case 21 – Who Are These People in My Living Room?
Case 28 – Young Woman Feeling Sick and Confused
Case 29 – A Man with Urinary Incontinence and Trouble Walking
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
