Neurocognitive And Developmental Disorders
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USMLE Step 2 CK › Neurocognitive And Developmental Disorders
Which of the following is the most appropriate initial pharmacologic treatment?
Lorazepam
Risperidone
Sertraline
Methylphenidate
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.
This clinical presentation is most characteristic of which of the following neurodegenerative syndromes?
Corticobasal degeneration
Behavioral-variant frontotemporal dementia
Typical Alzheimer disease
Primary progressive aphasia
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).
Which of the following features most specifically suggests a diagnosis of delirium superimposed on a neurocognitive disorder?
Acute onset and fluctuating course
Language difficulties
Memory impairment
Impaired executive functioning
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.
Which of the following is the most appropriate first-line treatment for his ADHD?
Atomoxetine
Clonidine
Amphetamine-dextroamphetamine
Modafinil
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.
What is the most likely diagnosis?
Alzheimer disease
Vascular dementia
Lewy body dementia
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.
What is the most likely diagnosis?
HIV-associated neurocognitive disorder
Primary CNS lymphoma
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.
What is the most likely diagnosis?
Delirium
Parkinson disease dementia
Alzheimer disease with psychosis
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.
This patient's symptoms are most likely caused by degeneration of which of the following brain regions?
Substantia nigra and brainstem
Hippocampus and temporal-parietal cortex
Basal ganglia and thalamus
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.
Which of the following is the most appropriate next step in management?
Obtain urinalysis, CBC, and chest x-ray
Obtain a non-contrast CT scan of the head
Administer haloperidol for agitation
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.
Which of the following is the most likely diagnosis?
Social (pragmatic) communication disorder
Hearing impairment
Global developmental delay
Autism spectrum disorder
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.