Question of the week : neurology
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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.
Question of the week : neurology
- 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