61 - Neurodegenerative Diseases II

Question 1

Cerebral Amyloid Angiopathy

Answer 1

Deposits of β-amyloid
In small cortical and leptomeningeal arterioles and capillaries
Sporadic
Rarely familiar
Co-morbid with AD
Oft asymptomatic
Can cause traumatic hemorrhage or (alternately) ischemia through obliteration of vessel lumen
Decently common non-traumatic hemorrhages
Cortical “Lobar” hemorrhages
Oft occipital

Question 2

Cerebral Amyloid Angiopathy - Histo

Answer 2

Double Barrel Lumen

Question 3

Vascular Dementia

Answer 3

Diverse Etiologies
5% - 15% of dementia cases
Volume of infarcted tissue is important
LOCATION is more important
High comorbidity with Alzheimer's (cerebrovascular lesions are found in 30 - 60%)

Question 4

Frontemporal Dementia

Answer 4

FTLD with ubiquitin-positive inclusions
Pick disease
Dementia lacking distinctive histology
FTLD linked to Chromosome 17 (Tau)

Question 5

Clinical Signs for FTLD

Answer 5

Frontal signs:

Personality change
Inappropriate behavior
Impaired language

Question 6

Pick Disease

Answer 6

Knife Edge Atrophy

Pick bodies

Question 7

Pick bodies

Answer 7

Cytoplasmic
Round
Agyrophilic
Tau positive
Ubiquitin positive
10 - 15 μm across

Question 8

FTLD with ubiquitin-positive inclusions

Answer 8

Ubiquitin-positive inclusions

Question 9

ALS

Answer 9

Motor neuron disease
After age 40
Progressive weakness
muscle wasting
fasciculations
asymmetrical at first (lower motor neuron)
Upper motor neuron signs later
Loss of motor neurons in the ventral horn
Histo - Spheroids

Distal - proximal dying back axonopathy
CD68 stain striking
Large numbers of microglia

Question 10

TDP43

Answer 10

Accumulated in ALS & FTLD with ubiquitin-positive inclusions
Involved in RNA splicing and binds to DNA