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Review Question - QID 214378

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QID 214378 (Type "214378" in App Search)
A 75-year-old man is referred to a neurologist for "abnormal behavior." He is accompanied by his daughter who said that the patient had a fall approximately 1 week ago but did not injure his head and that his movements began to appear "slow" about 2 months ago. He has garbled speech and episodes of confusion where he does not know where he is. However, after these episodes he does return to his mental baseline - he knows where he is, his speech is intelligible, and he recognizes his family. The daughter endorses that the first episode of confusion happened about 1 year ago. At times, he appears agitated at things that the daughter does not see, and once asked her why there was a dog in the house, even though no one owns a dog. His mother had Alzheimer disease, and his father died due to myocardial infarction. Physical examination is notable for upper extremity limb rigidity and short-stepped and bradykinetic gait. Which of the following is most likely the diagnosis?

Alzheimer dementia

0%

0/5

Dementia with Lewy bodies

40%

2/5

Frontotemporal dementia

40%

2/5

Parkinson disease

20%

1/5

Vascular dementia

0%

0/5

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This patient is presenting with visual hallucinations (e.g., seeing a dog and being agitated by things no one else sees) and features of parkinsonism (e.g., limb rigidity and bradykinesia) which are highly suggestive of dementia with Lewy bodies.

Dementia with Lewy bodies (DLB) is neurodegenerative dementia believed to be due to alpha-synuclein deposition within neurons. Patients with DLB present with fluctuating cognition (i.e., level of alertness and attention), visual hallucinations (can be formed images, seeing humans and animals or simple shape and colors), parkinsonism (bradykinesia or akinesia, rigidity, postural instability leading to falls, and resting tremor), and rapid eye movement (REM) sleep disorder. The diagnosis is clinically made when at least 2 of these findings are met. Magnetic resonance imaging will typically demonstrate generalized atrophy of the brain. The mean age of presentation is around 75 years of age and is more common in men. DLB is considered the second most common cause of dementia with Alzheimer being the first.

Incorrect Answers:
Answer 1: Alzheimer dementia is a neurodegenerative dementia. Alzheimer dementia does not present with visual hallucinations and parkinsonism. Typically, patients with Alzheimer presents with short-term memory loss, spatial disorientation, and language deficits.

Answer 3: Frontotemporal dementia is a neurodegenerative dementia that classically presents with disinhibition (e.g., making inappropriate remarks and urinating in public), compulsive behavior, apathy, and atrophy of the frontotemporal region of the brain.

Answer 4: Parkinson disease is a neurodegenerative disorder that leads to decreased dopaminergic neurons, especially in the basal ganglia. Patients present with resting tremor, rigidity, bradykinesia or akinesia, and postural instability. Visual hallucinations and fluctuating cognition are not typically seen in Parkinson disease compared to dementia with Lewy bodies.

Answer 5: Vascular dementia is a form of dementia that is caused by cerebrovascular disease. Patients classically present with a stepwise cognitive decline such that patients may initially have a cognitive decline that is temporally related to cerebrovascular events. Impaired executive function is classically associated with this dementia. MRI will demonstrate cerebrovascular disease.

Bullet Summary:
Dementia with Lewy bodies presents with at least 2 of the following: fluctuating cognition, visual hallucinations, parkinsonism, and rapid eye movement (REM) sleep disorder.

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