Neurocognitive And Developmental Disorders

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USMLE Step 2 CK › Neurocognitive And Developmental Disorders

Questions 1 - 10
1

Which of the following is the most appropriate initial pharmacologic treatment?

Methylphenidate

Sertraline

Lorazepam

Risperidone

Explanation

This patient's symptoms are consistent with adult attention-deficit/hyperactivity disorder (ADHD), characterized by a persistent pattern of inattention and/or hyperactivity-impulsivity that interferes with functioning. First-line pharmacologic treatment for ADHD in adults is a stimulant medication, such as methylphenidate or amphetamine. Sertraline is an SSRI for depression/anxiety, lorazepam is a benzodiazepine for anxiety, and risperidone is an antipsychotic.

2

This clinical presentation is most characteristic of which of the following neurodegenerative syndromes?

Behavioral-variant frontotemporal dementia

Corticobasal degeneration

Primary progressive aphasia

Typical Alzheimer disease

Explanation

Primary progressive aphasia (PPA) is a clinical dementia syndrome characterized by the gradual and prominent decline in language abilities, while other cognitive domains like memory and executive function are relatively spared early in the disease course. PPA is considered a form of frontotemporal lobar degeneration. This contrasts with typical Alzheimer disease (early memory loss) and behavioral-variant FTD (early personality and behavioral changes).

3

Which of the following features most specifically suggests a diagnosis of delirium superimposed on a neurocognitive disorder?

Memory impairment

Language difficulties

Impaired executive functioning

Acute onset and fluctuating course

Explanation

While cognitive deficits like memory impairment and executive dysfunction are present in both dementia and delirium, the hallmark features that distinguish delirium are its acute onset (hours to days) and a fluctuating course of symptoms, with variations in attention and arousal level throughout the day. The slow, progressive decline is characteristic of the underlying neurocognitive disorder (dementia), while the recent, rapid change with fluctuations points to delirium.

4

Which of the following is the most appropriate first-line treatment for his ADHD?

Atomoxetine

Amphetamine-dextroamphetamine

Modafinil

Clonidine

Explanation

In patients with ADHD and a co-occurring substance use disorder, particularly with a history of diversion, non-stimulant medications are preferred. Atomoxetine, a norepinephrine reuptake inhibitor, is an effective and FDA-approved non-stimulant treatment for ADHD and lacks abuse potential. Stimulants like amphetamine-dextroamphetamine would be a poor choice due to the high risk of abuse and diversion.

5

What is the most likely diagnosis?

Lewy body dementia

Vascular dementia

Alzheimer disease

Frontotemporal dementia

Explanation

This patient presents with an insidious onset and gradual progression of cognitive decline, primarily affecting recent memory (amnestic presentation) and executive function. This clinical picture in an elderly individual is classic for Alzheimer disease, the most common cause of dementia. The absence of stepwise decline, parkinsonism, hallucinations, or prominent early personality changes makes other dementia subtypes less likely.

6

What is the most likely diagnosis?

Primary CNS lymphoma

HIV-associated neurocognitive disorder

Toxoplasmosis

Progressive multifocal leukoencephalopathy (PML)

Explanation

HIV-associated neurocognitive disorder (HAND) is a common complication in patients with advanced HIV/AIDS (typically CD4 < 200). It characteristically presents as a subcortical dementia with symptoms of apathy, executive dysfunction, and psychomotor slowing. Other opportunistic infections like PML, CNS lymphoma, and toxoplasmosis typically present more acutely with focal neurological deficits and show characteristic focal lesions on MRI.

7

What is the most likely diagnosis?

Delirium

Alzheimer disease with psychosis

Parkinson disease dementia

Dementia with Lewy bodies

Explanation

This patient's presentation includes the core features of dementia with Lewy bodies (LBD): fluctuating cognition, well-formed visual hallucinations, and parkinsonism. The presence of REM sleep behavior disorder (acting out dreams) is also highly suggestive. In LBD, cognitive decline begins before or within one year of the onset of motor symptoms. In Parkinson disease dementia, dementia develops more than one year after the diagnosis of Parkinson disease.

8

This patient's symptoms are most likely caused by degeneration of which of the following brain regions?

Basal ganglia and thalamus

Hippocampus and temporal-parietal cortex

Substantia nigra and brainstem

Frontal and temporal lobes

Explanation

The clinical picture of early and prominent personality change (disinhibition), apathy, and altered food preferences with relatively preserved memory is characteristic of the behavioral variant of frontotemporal dementia (bvFTD). This condition is caused by focal neurodegeneration in the frontal and temporal lobes.

9

Which of the following is the most appropriate next step in management?

Obtain a non-contrast CT scan of the head

Administer haloperidol for agitation

Obtain urinalysis, CBC, and chest x-ray

Increase her dose of donepezil

Explanation

This patient has an acute change in mental status superimposed on her chronic dementia, which is highly suggestive of delirium. The most important initial step is to identify and treat the underlying medical cause. In elderly patients, infections such as urinary tract infections or pneumonia are common precipitants. Therefore, an infection workup including urinalysis, CBC, and chest x-ray is the most appropriate next step.

10

Which of the following is the most likely diagnosis?

Social (pragmatic) communication disorder

Hearing impairment

Autism spectrum disorder

Global developmental delay

Explanation

This child displays the core features of autism spectrum disorder (ASD): 1) persistent deficits in social communication and social interaction (impaired eye contact, lack of response to name, no pretend play), and 2) restricted, repetitive patterns of behavior, interests, or activities (spinning wheels, insistence on sameness). While he has developmental delay, the specific pattern of deficits points to ASD.

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