11.1 Case History

A 69-year old, left-handed man presented at initial consultation with a history of difficulty with short-term recall for 18 months. There were neither obvious behavioral changes nor changes in long-term memory. He also denied any difficulty with lexical retrieval. There was no difficulty in comprehension and no topographic disorientation. He would occasionally feel down, but it seemed to be appropriate to the situation with no sustained depression. On collateral history from his wife, she noted that the cognitive symptoms began about 3 years prior. This was described as gradually progressive memory loss initially having difficulty in recalling recent events, and then subsequently needing written cues or reminders for appointments. An example of this was he could not recall who they had dinner with from several nights prior. Another would be he would tend to forget that they had just eaten recently and could not recall what they ate.

His wife also noted some lexical retrieval problems with him and he was less sure of directions when driving. There was no note of any personality or behavioral changes and he still had a good sense of humor. No hallucinations or language problems were noted. He had nocturia and had difficulty in going back to sleep afterward, but there were no nightmares or excessive movements during sleep. Functionally, he was independent on activities of daily living and able to do household chores such as cleaning the windows. He can care for himself and is aware of the financial situation. He still was able to go shopping and prepare his meal, though at times somewhat forgetful.

From his neurologic review, his gait was noted to be slightly slower, but his balance remained good and still managed to climb a ladder without any concern. There were no involuntary movements noted, nor any visual changes or diplopia. There was no difficulty with chewing, swallowing, or speech, and no problems with fine motor control. He would only occasionally have mild headaches occurring once a week and was mostly stress related.

11.2 Past Medical History

He was diagnosed with rheumatic fever at age 54 but no other details were available. There were no reports of chest pain or palpitations. He was prone to indigestion but otherwise no gastrointestinal symptoms. He was recently diagnosed to have prostate cancer with an elevated PSA level requiring further management. He had no regular medication and had no known allergies.

On review of his risk factors for dementia, it was noted that he had chronic use of alcohol daily (2–3 oz) but no history of smoking. There have not been any sustained symptoms of depression, though it was noted that his wife felt there is some dysphoria at times. There was no history of head injury.

11.3 Family History

There was a strong history of dementia over at least three generations on his maternal side. Most prominent was that of his brother, who was evaluated at age 57 and was diagnosed with Alzheimer’s dementia. He died at age 62 with an autopsy reporting changes consistent with dementia of the Alzheimer-type (reported in 1990s), although his history also mentioned significant executive dysfunction and personality change. His mother died at age 76 with dementia; however no autopsy was done. His maternal grandmother had late onset dementia with symptom onset at 88. She was provisionally diagnosed with “chronic brain syndrome due to senile brain disease,” with psychotic reaction and was 96 years old at the time of death.

He was the eldest of three siblings. His younger brother died at age 62 (as mentioned above) and one younger sister who was healthy with no memory or cognitive problem. His father died at age 94 after a fall with no reported cases of dementia from his paternal family.

11.4 Social History

The patient was born in Vancouver, BC, and completed high school. He was of English ancestry both from his paternal and maternal side. He worked as a pharmaceutical salesman for 13 years and retired at the age of 61 with no problems with work at that time. He was married for 43 years and had two daughters and two grandchildren.

11.5 Initial Clinical Examination

The patient was fully cooperative during the examination, blood pressure was 150/70 mmHg and pulse rate of 72, with occasional ectopy. His general examination was notable for the absence of cranial or carotid bruits. He had a grade II/VI systolic murmur at the apex, radiating to the base. Abdominal examination was unremarkable. Skin and joints were also normal.

On cognitive assessment, he had a score of 21/30 on Mini-Mental State Examination (MMSE) and 67/100 on 3MS. On more detailed testing, he was noted to be significantly temporally disoriented while spatially oriented. He encoded test objects readily. At 5 min, he recalled 1/5 objects spontaneously, 2/5 with categorical cueing, and 3/5 with forced choices. There were some syntax errors noted when reading out a paragraph aloud. There was also a significant loss of concept in detail on this memory task.

On test of language, he was fluent in his ability to generate a word list to semantic category (11 four-legged animals in 30 s). Body part naming was normal. He named 8/10 items visually presented, improving with minimal cueing to nine. He had some restriction in his ability to abstract similarities. He did have difficulty with hand length on the clock drawing, suggesting some degree of visuospatial deficits.

He did perform the Luria hand sequences normally. There were some difficulties on tests of mental control, particularly in serial 7s (2/5) and other mental calculations. There were no frontal release reflexes.

Cranial nerves: Visual fields were full to confrontation. Discs were visualized and were normal. Extraocular movements were normal. No facial weakness with palatal function and speech normal.

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  Motor: There were no abnormalities in his upper and lower extremities.

  
  
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  Reflexes: DTRs were 2+ and symmetric bilaterally and both plantar responses were flexor.

  
  
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  Sensory: There were no abnormalities to the primary modalities tested.

  
  
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  Coordination: Finger-to-nose and heel-to-shin responses were accurate. His gait appeared to be slightly slow, but steady.

11.6 Additional Investigations

Blood works such as CBC, B12, folate, TSH, RPR, and FTA-ABS were all within normal range.

A computed tomography (CT) scan of the head with contrast showed mild degree of diffuse cortical tissue loss, no focal lesion seen.

Magnetic resonance imaging (MRI) of the brain showed bilateral temporal lobe atrophy with marked prominence of both Sylvian fissures. There was less marked atrophy with the frontal and parietal lobes. Several nonspecific high-signal foci within the left corona radiata and right centrum semiovale were also noted as well as a left temporal arachnoid cyst.