1. cognitive deficit most typical of idiopathic normal pressure hydrocephalus
    slow processing
  2. behavioral variant of frontotemporal dementia What is the most likely pathologic finding?
    • tau inclusions
    • either tau or TDP-43
    • FUS accumulations are less common
  3. microglial proliferation
    a nonspecific finding in various central nervous system infectious and inflammatory conditions
  4. abnormal titer of N-methyl-d-aspartate receptor (NMDA) antibody
    • high-dose IV corticosteroid treatment
    • usually the first step in acute therapy
    • Azathioprine, mycophenolate mofetil, and rituximab may eventually be used in the chronic therapy regimen
  5. distinguishing between dementia due to mitochondrial disease and Jakob-Creutzfeldt disease
    • cardiomyopathy
    •    present in mitochondrial disease
    •    absent in J-C disease
    • both have:
    •    acute dementia
    •    visual symptoms
    •    extrapyramidal symptoms
    •    positive family history
    • MRI may not be useful
  6. dementia with Lewy bodies
    • key features:
    •    fluctuations of cognition
    •    visual hallucinations
    •    longstanding sleep behavior suggestive of premorbid rapid eye movement (REM) sleep behavior disorder
    • occipital hypometabolism in FDG-PET
    • early in the course of dementia with Lewy bodies, problems with attention, executive function, and visuospatial skills usually dominate
  7. Mini-Mental State Examination (MMSE) is most likely to be abnormal early in the disease course of Alzheimer disease
    • delayed recall
    • in early Alzheimer disease, episodic memory is the predominant impaired domain, which would most commonly be tested with delayed recall of three words after a brief time delay
  8. MRI sequences would be most useful when looking for evidence of prior hemorrhage
    • susceptibility-weighted imaging (SWI)
    • single lobar, cortical, or cortico-subcortical hemorrhage and focal or disseminated superficial siderosis satisfies the modified Boston criteria for probable cerebral amyloid angiopathy
  9. MCI
    • feature that distinguishes MCI from dementia is that the patient’s daily function is impaired in dementia but spared in MCI
    • regularly forgets the names of casual acquaintances can still function in daily life, whereas people who frequently get lost when driving to work, have difficulty dressing, or are unable to manage personal finances or operate home appliances have functional deficits that are too great to be consistent with MCI
  10. diagnostic test abnormality more common in dementia with Lewy bodies than it is in Alzheimer disease
    • reduced dopamine transporter levels on single-photon emission computed tomography (SPECT) scan
    • typical of both dementia with Lewy bodies and Parkinson disease
    • 90-100% specificity for differentiating these conditions from Alzheimer disease
  11. Alzheimer disease
    • hippocampal atrophy on MRI scan
    • increased spinal fluid levels of tau protein
    • temporoparietal hypometabolism on FDG-PET scan
  12. N-methyl-D-aspartate (NMDA) receptor antibody
    • a stereotyped disorder that often evolves in stages
    • starting with headache, fever, or nausea
    • then prominent psychiatric symptoms and behavioral changes including social withdrawal and stereotyped behavior
    • eventually reduced responsiveness
  13. metachromatic leukodystrophy
    • autosomal recessive
    • arylsulfatase A deficiency
    • adult-onset cases present with:
    •    slow-onset psychiatric, behavioral, and cognitive changes
    •    spasticity or peripheral neuropathy
    • cholecystitis is an important non-neurologic manifestation
    • MRI findings are typical of most leukodystrophies with bilateral symmetrical T2 changes in the white matter, usually more frontally located
  14. adrenoleukodystrophy
    • X-linked
    • a mutation in the ABCD1 gene
    • rapidly progressive white matter syndrome manifesting with parieto-occipital involvement on imaging
    • cognitive impairment with visual processing disturbances as well as seizures and spastic paraparesis
    • not expected to cause high degree of impairment in females
    • cannot be transmitted to a male by his father
    • 90% have associated adrenal insufficiency
  15. Krabbe disease
    • autosomal recessive
    • rarely presents in adulthood
    • when it does, cognitive and psychiatric symptoms appear later
  16. Alexander disease
    • autosomal dominant
    • presents with bulbar dysfunction in adults
    • ataxia, spasticity, ocular motility disturbances, palatal myoclonus, and sleep disturbance
    • toxic gain of function mutation in the GFAP gene
    • MRI features are different than other leukodystrophies, with adult forms demonstrating medullary atrophy and changes in the dentate nuclei and middle cerebellar peduncles
  17. cognitive domain most likely to be impaired in early Alzheimer disease
    • episodic memory
    • specifically for recent events
    • lose objects and repeat questions in a conversation
    • procedural memory for task performance becomes impaired later
    • working memory and semantic memory are often preserved until late in the disease course
  18. cerebrotendinous xanthomatosis
    • autosomal recessive
    • mutations in the gene for mitochondrial sterol 27-hydroxylase
    • tendon xanthomas (especially in the Achilles tendon)
    • patients present with early psychiatric manifestations and later develop neurologic manifestations such as ataxia and upper motor neuron signs
    • palatal myoclonus
    • dentate nucleus changes
    • white matter changes
    • psychiatric and cognitive manifestations
    • juvenile cataracts
    • treatment with chenodeoxycholic acid may prevent neurologic deterioration
  19. normal pressure hydrocephalus
    • a gait disorder that can include a variety of parkinsonian features, such as difficulty initiating gait, shuffling, tripping, falling, festination, retropulsion, anteropulsion, or difficulty with turns
    • gait deficits are nearly universal among patients with idiopathic normal pressure hydrocephalus
    • cognitive deficits and problems with sphincter control are less consistently present
  20. behavioral variant of frontotemporal dementia
    progressive behavioral syndrome manifested by disinhibition
  21. semantic variant primary progressive aphasia
    • neurodegenerative pattern of progressive language impairment
    • losing the meaning of speech
    • difficulty assigning meanings to words, specifically demonstrating impaired single-word comprehension
    • dominant temporal lobe atrophy is also supportive
  22. nonfluent agrammatic variant primary progressive aphasia
    • the problem is more in expressive speech than understanding the content of speech
    • She called a hammock ‘‘ know...between’’
  23. nonstroke vascular cognitive impairment
    multiple vascular risk factors, relatively greater impairment in executive function and processing speed than in episodic memory, reflex asymmetry, and neuroimaging findings suggestive of multiple small infarcts
  24. biomarkers associated with an increased likelihood of developing Alzheimer disease dementia
    • medial temporal lobe atrophy on MRI
    • temporoparietal hypometabolism on FDG-PET
    • diffuse uptake on amyloid positron emission tomography (PET)
    • low levels of amyloid-β42 (Aβ42) in CSF (amyloid is deposited in brain tissue)
    • high levels of tau protein (both t-tau and p-tau) in CSF (degeneration of tau-containing neurons)
    • presence of the (APOE)ε4 haplotype
  25. dural arteriovenous fistula (DAVF)
    • rapidly progressive dementia
    • confusion and memory loss as well as seizures, gait instability, headaches, or focal neurologic deficits
    • MRI demonstrates white matter hyperintensities and enlarged blood vessels over the surface of the cortex
    • diagnosis can be confirmed with cerebral angiography
  26. antineuronal nuclear antibody-1 (ANNA-1 or anti-Hu)
    • small cell lung carcinoma
    • 90% of patients with limbic encephalitis and ANNA-1 (anti-Hu)
  27. dementia with Lewy bodies is differentiated from Parkinson disease dementia on what basis
    • timing of dementia onset relative to the onset of parkinsonism
    • in patients who have dementia with Lewy bodies, dementia usually begins before the onset of parkinsonism, and at the latest it begins 1 year after the onset of parkinsonism
  28. Immunotherapy is most likely to be effective in patients who have encephalopathy associated with
    • voltage-gated potassium channel (VGKC) complex antibody
    • prognosis is generally better for patients who have encephalopathy associated with antibodies targeting neural surface antigens than for patients who have encephalopathy associated with antibodies targeting intracellular antigens
    • these target intracellular antigens:
    •    antineuronal nuclear antibody-1 (ANNA-1, anti-Hu)
    •    antineuronal nuclear antibody-2 (ANNA-2, anti-Ri)
    •    glutamic acid decarboxylase 65 (GAD65) antibody
    •    Purkinje cell cytoplasmic antibody type 2 (PCA-2)
  29. Stroop test
    • a test of executive function designed to assess inhibitory control
    • read names of colors (eg, green, blue, red) that are printed in a different color than denoted by the word (eg, the word “green” printed in blue ink)
    • they may impulsively say the color of the font when asked to read the word, or vice versa
  30. test parietal visuospatial function
    construction of intersecting pentagons
  31. reading comprehension
    tests language
  32. short-term recall
    tests memory
  33. serial 7’s
    test attention and calculation ability
  34. Susac syndrome
    • a triad of rapidly progressive encephalopathy, branch retinal artery occlusions, and hearing loss
    • headaches possible
  35. psychiatric condition associated with an increased risk of conversion from mild cognitive impairment to dementia
  36. which mental status test provides the strongest evidence of impaired executive function
    • performance on alternating pattern tasks
    • require the subject to shift attention from one motor program to another in a purposeful way corresponding to specified goals
  37. Whipple disease (due to infection with Tropheryma whippelii)
    • a treatable cause of rapidly progressive dementia
    • patient manifests with gastrointestinal symptoms (especially chronic diarrhea), systemic symptoms (fever, arthralgia) and neurologic involvement, which may include cognitive impairment as well as hemiparesis, seizures, or ataxia
    • diagnosis can be made with CSF or blood polymerase chain reaction (PCR), or with intestinal biopsy, immunohistochemistry and PCR
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