A 78-year-old woman presented with mild cognitive impairment based on her Mini-Mental Status Examination score. Her MRI showed extensive white matter hyperintensities without ventriculomegaly. She had a history of hypertension but normal lab results. The most likely diagnosis is vascular cognitive impairment given her history of hypertension and MRI findings of diffuse white matter lesions, which have been associated with vascular causes of dementia.

1. Question of the week
Ahmed Mohamed Badheeb
Prof Of Oncology & Internal Medicine

2. • A 78-year-old woman with a history of
hypertension and hypercholesterolemia is
brought to the clinic by her daughter, who
reports that during the past 12 months her
mother has been slow to accomplish things
around the house, lacks initiative, and walks
more slowly than usual.

3. • The patient denies feeling depressed and
does not report any recent illnesses. Her
blood pressure has been under good control,
and she has adhered to her medications

4. Mini–Mental Status Examination
• She scores 24 out of 30 on the Folstein Mini–
Mental Status Examination, indicating mild
cognitive impairment.
• She lost points for calculation and orientation
but had intact registration, immediate
memory, delayed recall, and visuospatial
construction.

7. The neurologic examination
• The rest of her neurologic examination is
normal, except for a reduced gait speed.

8. Laboratory evaluation
• normal vitamin B12 and thyroid-stimulating
hormone levels and a negative rapid plasma
reagin test.

9. MRI of the brain
• extensive periventricular and subcortical
white-matter hyperintensities without any
ventriculomegaly.

10. Which one of the following diagnoses is most
likely in this patient?
A. Vascular cognitive impairment
B. Alzheimer disease
C. Dementia with Lewy bodies
D. Normal-pressure hydrocephalus
E. Creutzfeldt–Jakob disease

11. The correct answer : A
A. Vascular cognitive impairment
B. Alzheimer disease
C. Dementia with Lewy bodies
D. Normal-pressure hydrocephalus
E. Creutzfeldt–Jakob disease

12. Key Learning Point
• The most likely diagnosis in a patient with a
history of hypertension who demonstrates a
gradual loss of cognitive function and whose
MRI is found to have diffuse periventricular
white-matter hyperintensities is vascular
cognitive impairment.

13. Vascular cognitive impairment
• cognitive impairment
• either a history of clinical stroke
• or
• imaging that reveals features of cerebrovascular
disease, such as diffuse periventricular white-
matter intensities.
• Periventricular white-matter hyperintensities
have been most closely associated with vascular
causes of dementia and predict an increased risk
for stroke, cerebrovascular events, dementia, and
death.

14. Binswanger's disease, also known as subcortical
leukoencephalopathy, is a form of small vessel
vascular dementia caused by damage to the white
brain matter. White matter atrophy can be caused
by many circumstances including chronic
hypertension as well as old age.

15. • more memory impairment as the presenting
feature, whereas patients with vascular dementia usually
have greater impairment with executive functioning.
Alzheimer disease

17. Normal-pressure hydrocephalus
• usually have ventriculomegaly out of
proportion to cortical atrophy on MRI.

18. Creutzfeldt–Jakob disease
The human form of mad cow disease ..
• typically causes rapidly progressive dementia with global cognitive
impairment that may be accompanied by gait abnormalities and
stimulus-induced myoclonus.
Cortical and basal ganglia abnormalities on diffusion-weighted MRI sequences.

19. Creutzfeldt–Jakob disease
The human form of mad cow disease ..
• Definite:
• Diagnosed by standard neuropathological techniques; and/or immunocytochemically;
and/or Western blot confirmed protease-resistant PrP; and /or presence of scrapie-
associated fibrils.
• Probable:
• Rapidly progressive dementia; and at least two out of the
following four clinical features:
• Myoclonus
• Visual or cerebellar signs
• Pyramidal/extrapyramidal signs
• Akinetic mutism
• AND a positive result on at least one of the following laboratory tests:
• a typical EEG (periodic sharp wave complexes) during an illness of any duration; and/or
• a positive 14-3-3 cerebrospinal fluid (CSF) assay in patients with a disease duration of less than 2 years
• Magnetic resonance imaging (MRI) high signal abnormalities in caudate nucleus and/or putamen on
diffusion-weighted imaging (DWI) or fluid attenuated inversion recovery (FLAIR)
• AND without routine investigations indicating an alternative diagnosis.

20. Dementia with Lewy bodies
• Fluctuations in cognition
• Visual hallucinations
• Signs of parkinsonism.

21. Reference
• NEJM Knowledge+ Question of the Week for
December 6, 2016