10.1 Case History

A 62-year-old right-handed lady presented at initial consultation with an 18 months history of slowly progressive short-term memory problems and personality change. She denied having any problems herself, suggesting some lack of insight. However, the collateral history obtained from her daughter made it clear that she had problems with progressive worsening of her short-term memory in the past 18 months, severe enough to be of concern in the last year. She reported that she had a poor episodic memory for conversations and recent events. As an example, her daughter gave examples that the patient was frequently unable to remember previous conversations. Also, she mentioned that she was prone to forget the discussed topic in the middle of conversations.

She did not have difficulties remembering proper nouns or recognizing people. Her semantic memory and long-term memory seemed to be well preserved, and her recall of past events was accurate. Her daughter mentioned that her organizational abilities declined compared to her previous skills, for example, she was having problems keeping track of her credit card and finances. Regarding her language, her daughter noted occasional naming problems. Her language output was normal, as well as her grammar. No paraphasias were noted. Her speech was also normal. Her daughter described her visuospatial skills and topographic orientation as being good.

From the psychobehavioral standpoint, her daughter reported that her personality had changed. She used to be a short-tempered and anxious person, but she became calmer and much more easy going. On the other hand, she also showed some increased impulsive behavior, such as shouting at cashiers when she had problems paying for items when shopping. Also, she was spending in excess and giving away her money indiscriminately; as an example, once she bought seven leather jackets in a day, and she gave money to a family member with addiction problems. No other behavioral issues were noted, and her mood was generally described as upbeat and stable. There were no hallucinations. She had no obsessive behaviors. Her sleeping pattern was fine, with no evidence of REM sleep behavior disorder or any other parasomnia, but she has a long history of benzodiazepine use. Her appetite was normal with no change in her eating preferences. There were no motor issues, no tremor, no falls.

Functionally, she was independent with her basic activities of daily living. However, she had impairment in some of her instrumental activities. As an example, she was having problems with her finances.

10.2 Past Medical History

Her past medical history was notable for diabetes mellitus type 2 and hypothyroidism for over 5 years. She was on metformin 500 mg two times a day, pravastatin 20 mg/day, spironolactone 50 mg/day, levothyroxine 0.075 mg/day, and oxazepam 15–30 mg every night at bedtime for sleep, for a long time, due to insomnia starting in her 40s.

On review of her risk factors for dementia, it was noted that she had a 50 pack-year history of smoking. Additionally, she was a heavy drinker in her 30s, when she also had problems with depression that required hospitalization and antidepressant treatments. At the time of the initial consultation, she was still drinking, but only a glass of wine occasionally. She had no significant head injuries. There were no stroke-like episodes or other neurological symptoms.

10.3 Family History

Her mother died at 61 from heart attack and had no cognitive problems at the time. Her father also died at 84 from heart problems and was mentally alert. There is no information on the extended family history.

The patient was the 12th born of 15 children. Two died in childbirth. One sister died at 32 from brain aneurysm. From the remaining siblings, two brothers and one sister developed early-onset dementia in their late 50s, passing away in their early 60s. No autopsies were performed. Medical records were only available on one family member, and that was compatible with probable Alzheimer’s dementia.

10.4 Social History

The patient was born in the province of Manitoba with French ancestry on the maternal side and Polish ancestry on the father’s side. She had a grade six education and worked as a clerk and waitress in the past. She was married for the third time and her husband was suffering from several chronic medical problems that were limiting his mobility and independence. She had a daughter and a son.

10.5 Clinical Examination

Her general physical examination was normal. Her blood pressure was 120/70, with a regular pulse of 76. She had no cranial or carotid bruits. Breath sounds were clear and heart sounds were normal. Abdomen was soft, lax with no organomegaly. Her peripheral pulses were intact. She had no lower limb edema.

She was alert and mostly cooperative during examination and not under stress, but occasionally irritable. She was well dressed and in good hygiene. She did not exhibit any abnormal behavior, stereotypic or utilization behavior. Her speech was normal.

On cognitive assessment, she scored 24/30 on MMSE and 77/100 on 3MS. She lost points on mental reversal, and temporal and spatial orientation from the MMSE, but was able to remember three words. On the 3MS she lost further points on naming four-legged animals, describing similarities. She also made mistakes scoring 2/3 on the three-stage command.

On more detailed cognitive assessment, her spontaneous language and verbal output was normal with no lexical retrieval difficulties, paraphasias, or dysarthria. Her phonemic fluency was impaired, naming only four words beginning with letter S in 1 min. Her visual confrontation naming was also impaired, managing to name three out of six objects with a slight improvement after lexical cues. Her semantic knowledge about those objects was preserved.

In her logical memory testing, there was evidence of short-term memory impairment noted on her paragraph-free recall of specific details, but the concept was retained.

Her executive functioning also demonstrated impairment on testing, with problems on abstractions and describing similarities. She completed the three-step Luria Sequence easily.

Her visuospatial functioning was abnormal. While her free clock drawing was intact, she made a number of errors copying a 3D cube. On a task of visuospatial problem solving, she scored 5/6 on square fitting.

Cranial nerves: Pupils were equal and reactive. Saccades and smooth pursuit were normal. She had no facial weakness or asymmetry. Palatal function, gag, and tongue movements were normal.

Motor: There was no drift on her outstretched upper limbs. Tone, power and bulk were normal. No other motor abnormalities were noted. Deep tendon reflexes were 2 in the upper and lower extremities. Plantar responses were both flexor. There was no parkinsonism and no abnormal movements at her initial assessment.

Sensory: The primary modalities of sensation were normal in the upper and lower extremities.

Coordination: Finger-to-nose and heel-to-shin responses were accurate. Tandem balance and her speed of movements were normal.

10.6 Additional Investigations

Blood work including blood count, liver function, electrolytes, renal function, Ca, thyroid function, folate, and B12 were in the normal range.

CT head scan showed no evidence of any structural lesions. However, it did show early atrophy, more prominent in the frontotemporal sulci compared to the posterior parietal region.

MRI showed no evidence of any structural lesions. However, it showed generalized atrophy with increased cerebral sulci, greater than expected for her age. The atrophy was more pronounced in the temporal lobes, while hippocampus appeared normal.

10.7 Neuroimaging

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