neurodegen baseline

The PET scan shown is most consistent with which of the following diseases?

Which of the following medications is an acetylcholinesterase inhibitor that is FDA approved in the U.S. for the treatment of severe Alzheimer’s disease?

A 68-year-old man presents with word-finding difficulties and speech hesitancy. His wife notices that he struggles to name objects and sometimes mispronounces words. On examination, he can name objects but has significant difficulty repeating long sentences. While his spontaneous speech is slow, it is grammatically correct. Which of the following is the most likely diagnosis.

Mutations in the progranulin protein (gene PGRN) can serve as a biomarker for the risk of which of the following diseases?

Which of the following is the mechanism of action for aducanumab?

A 50-year-old man presents with slowly progressive cognitive decline and personality changes. Over the past several months, he has been having trouble remembering the names of relatives/close friends and he has also become more apathetic. He is known to have trisomy 21. His exam is notable for memory deficits, but no rigidity or tremor. What is the name of the region of the brain that synthesizes the neurotransmitter that is decreased in this patient’s disease?

A 57-year-old teacher presents to the clinic with reading difficulties. His first symptoms developed 12 months ago when he had trouble marking students’ homework. He progressively also lost the ability to write and had to retire from his profession. Now he needs help tying his shoes and buttoning his clothes. His visual acuity is 20/20. Funduscopic examination and the rest of the neurological examination are unremarkable. Which of the following is the most likely diagnosis?

A 72-year-old woman is brought to the clinic by her family with concerns about living at home alone. Her daughter states that the patient’s “memory is not what it used to be” and that she will often think that there are little children in her house when no one is there. On exam, you appreciate a bilateral upper extremity resting tremor. A functional PET scan will most likely show which of the following?

What is the pathophysiology that leads to the pathology shown in the image below?

An 83-year-old male with a past medical history of PD is brought to the clinic with his daughter due to worsening behavior at his nursing home. He has become easily agitated with staff and will occasionally yell out at night at “people trying to rob him”. His daughter asks which treatments could be used to help with these symptoms. Which of the following antipsychotic agents would be the best treatment options?

You are caring for a 78-year-old man with slowly progressive memory loss and behavioral change. You are concerned about either frontotemporal dementia (FTD) or Alzheimer’s disease (AD). Which of the following tests would be the most sensitive and specific in differentiating the two?

Patients with trisomy 21 are at an increased risk for which of the following?

Alzheimer’s disease typically has which of the following findings on functional PET imaging?

Which of the following is most often associated with late-onset Alzheimer’s disease?

A patient with Alzheimer’s disease donates their brain for research on neurodegenerative disease. A histopathologic, H&E-stained, sample of his brain tissue is shown below. What is the structure marked by the black arrow?

Patients with Alzheimer’s disease may have hypometabolism in which of the following brain regions?

Which of the following pathologic findings is associated with posterior cortical atrophy?

Identify the darkly stained structures.

Which of the following medications is an NMDA receptor antagonist?

Which of the following is not a recommended screening test for cognitive dysfunction?

A 64-year-old female with no significant past medical history presents to the clinic because of concerns related to her memory. She states that she has difficulty recalling names and recent conversations. She had a planned retirement two years ago and was functioning well at work at that time. She continues to live alone and is independent with her basic and instrumental daily life activities. Depression screening is negative, but she scores a 27/30 on the Montreal Cognitive Assessment (MOCA), with deficits in delayed recall. Her neurological examination is otherwise non-focal. What is the most likely diagnosis?

Identify the pathologic finding marked by the red arrow.

Which of the following is the most common gene associated with early-onset Alzheimer’s disease?

Which of the following is the mechanism of action of memantine?

A 57-year-old male presents to the Emergency Room with a 3-month history of progressive altered mental status. On examination, the patient had ataxia and hyperekplexia. An MRI is performed and shown below. What is the most likely etiology of the patient’s deficits?

A 64-year-old woman is brought to the office by her husband for changes in her speech pattern. While neurocognitive testing was largely unremarkable, her spontaneous speech appears effortful, halting, and laced with grammatical errors. MRI of the brain revealed left posterior frontal and insular atrophy. Which of the following is the most likely diagnosis?

A 72-year-old patient presents to the clinic with an 8-month history of progressive difficulty using his right arm and falls. On exam, there is right arm rigidity, bradykinesia, and intermittent myoclonus. Romberg testing was positive. Extraocular eye movements and memory testing are normal. Which of the following is the most likely diagnosis?

Which of the following is the annual conversion rate of mild cognitive impairment (MCI) to dementia?

Per the American Academy of Neurology guidelines, which of the following diagnostic tests should be included in the routine evaluation of the demented patient?

You are caring for a 50-year-old with amyotrophic lateral sclerosis (ALS). Which of the following disorders is most likely to also develop in patients with ALS?

An 8-year-old girl presents with a progressive neurologic disorder. She initially was noted to have slow learning and clumsiness years ago. Shortly thereafter she developed a seizure disorder of unclear etiology. Now, she has been having progressive visual changes and worsened cognition and memory. To aid in the diagnosis, a muscle biopsy is done and shows autofluorescent curvilinear bodies. Her disorder falls into which category of disease?

A 75-year-old patient is brought by her husband because of word-finding difficulty. On cognitive testing, the most impressive finding was that the patient couldn’t recall many colors of the rainbow. Based on this finding, what kind of memory is most impaired?

Which of the following clinical features is most suggestive of logopenic primary progressive aphasia?

Which of the following medications is approved only for moderate to severe Alzheimer’s Disease?

This gross microscopic image is most consistent with which of the following diseases?

An 82-year-old female presents with her family for a 3-year slow decline in memory. On examination, she has a MOCA of 23/30. She does not feel that she has any issues, but her family feels that she has trouble remembering important dates in her life and will repeat stories to them. Despite this, she has lived alone since her husband died 2 years ago and is able to cook, clean, and bathe on her own. She also balances her own checkbook and does her own grocery shopping. According to the DSM-V, this patient meets the criteria for which disorder?

Patients with Alzheimer’s disease usually have a loss of cholinergic neurons from which of the following regions?

The following gross pathologic coronal section is presented to you for evaluation. The area shown by the black arrow is a sign indicative of which of the following disorders?

A 68-year-old female presents with a one-year history of progressive memory problems and deteriorating mobility. Upon further questioning the patient notes incontinence. A physical exam reveals a wide-based gait with shuffling steps. A brain MRI shows widening of the temporal horns of the lateral ventricles. Which of the following is the best next step in management?

Which of the following is the mechanism of action for amantadine?

A 65-year-old mechanical engineer presents with increasing word-finding difficulties. He is particularly concerned that he is forgetting the names of people, places, and objects. On examination, he has difficulty naming objects when shown (i.e. keys, watch, etc.). Otherwise, his speech is fluent. A SPECT scan shows hypometabolism in the left anterior temporal lobe. Which of the following is the most likely diagnosis?

An 85-year-old man presents back to your clinic for follow-up with his family for a slowly progressive neurologic decline over the past 4 years, namely with memory and cognitive decline. You have already performed a brain MRI and additional testing and suspect that the patient has Alzheimer’s dementia. The family expresses concerns over the patient’s driving. Which of the following, if true, is not a useful tool to identify driving risk according to updated practice guidelines?

A 62-year-old male with a past medical history of hypertension, type 2 diabetes, and dyslipidemia presents with a stepwise progression of worsening forgetfulness over the last three years. Physical examination reveals a score of 20/30 on the Mini-Mental State Examination with deficits in multiple cognitive domains. Hyperreflexia in the left upper and lower extremities is also appreciated. Her axial FLAIR sequence brain MRI scan is shown. Which of the following is the most likely diagnosis?

Which of the following symptoms seen with normal pressure hydrocephalus (NPH) is the most responsive CSF shunt placement?

A 78-year-old woman is brought to the neurology clinic by her husband for memory loss. Her husband states for the past 6 months he has noticed a progressive decline in her memory, particularly with recent events. She has also lost her keys and cell phone on numerous occasions around the house. Her MOCA score in the clinic was 25/30, with points lost for poor delayed recall and attention. Neurodegenerative disease is suspected. Which of the following medications is the most appropriate for this patient to help with her cognition?

Which of the following is an appropriate test to assess for dorsolateral frontal lobe dysfunction?

Gaucher disease is caused by a homozygous mutation in the GBA gene which encodes the enzyme beta-glucocerebrosidase. Which of the following neurologic disorders is also linked to mutations, both homozygous and heterozygous, in the GBA gene?

An 82-year-old woman donated her brain to science for post-mortem analysis (see below). Which of the following symptoms did the patient most likely experience before her passing?

A 50-year-old Caucasian woman with no past medical history is admitted to the psychiatric hospital after trying to jump from a moving bus. She reports the other passengers were laughing at her in their minds, and trying to steal her thoughts. Upon arrival to the psychiatric floor, she is witnessed to have sudden left eye deviation with right arm twitching for a few seconds before turning her head, screaming loudly, and falling to the floor with full-body convulsions, lasting 2 minutes.

You, the attending neurologist, are consulted to help evaluate. On exam she is easily distractible, looking to the corners of the room for security cameras. You contact her husband who states she has never had any problems like this before, but she has gone downhill over the last 3 weeks, forgetting people and places, repeating herself, and acting strangely.

Lab evaluation reveals normal CBC, BMP, TSH, T4, free T4, and T3. The comprehensive drug screen is negative. MRI brain w/ and w/o contrast is normal appearing. Lumbar puncture is with 18 cmH20 opening pressure, 0 WBCs, 1 RBC, 50 protein, and 55 glucose. The autoimmune panel is sent and anti-microsomal antibodies are elevated. What is the most likely diagnosis at this time?

What is the pathophysiology that leads to the pathology shown with a black arrow in the image below?

Which of the following is not a recommended screening test for cognitive dysfunction?

Which of the following CSF biomarker findings would be expected in someone with Alzheimer’s disease when compared to healthy controls?

An 85-year-old man with memory issues donates his brain for research on neurodegenerative disease. A histopathologic, Bodian-stained, sample of his brain tissue is shown below. Based on this image provided, what was the patient’s most likely neurodegenerative diagnosis?

A 50-year-old male is admitted for 3 months of progressive memory loss, visual hallucinations, and myoclonus. Examination revealed myoclonic jerks. Which of the following tests is the most specific for this disease?

A 43-year-old female presents with rapidly declining cognitive function over the past few weeks. She is no longer able to hold a sustained conversation, she is no longer able to drive, and has worsening insomnia. CSF analysis shows increased 14-3-3 protein levels. What is the pathogenesis of this patient’s disease?

A 75-year-old patient is brought in by her husband because of word-finding difficulty.  On cognitive testing, the most impressive finding was that the patient could not recall many colors of the rainbow. Based on this finding, what kind of memory is most impaired?