Frontotemporal dementia

Question 1

3 types

Answer 1

• frontal lobe degeneration type
• Pick’s type
• Motor Neurone Disease (MND) type

Question 2

Frontal lobe degeneration type

Answer 2

• most common type
• spongiform degeneration or microvacuolation of the superficial neuropil is seen chiefly in layers 3 and 5 of the cortex

Question 3

Pick’s type

Answer 3

• Pick’s disease is characterised by atrophy in the frontotemporal regions
• these regions also have a loss of large cortical nerve cells, abundant gliosis and neuronal PIck’s bodies, which are masses of cytoskeletal elements
• Hirano bodies are also seen but in less frequncy than in Alzheimers

Question 4

Pick cells

Answer 4

-abnormal swollen oval-shaped neuronal cells with loss of Nissl’s substance and peripherally displaced nucleus

Question 5

Pick’s bodies

Answer 5

• not necessary for a diagnosis

- argentiophilic, tau and ubiquitin reactive filamentous inclusions

Question 6

Motor neurone disease (MND) type

Answer 6

• cerebral atrophy is less marked; limbic areas are largely preserved
• loss of large cortical nerve cells, microvacuolation and mild gliosis
• ubiquinated but not tau-immunoreactive inclusions are present within the frontal cortex and hippocampus
• MND pathology is seen in the anterior horn cells

Question 7

Huntington’s dementia

Answer 7

• severe loss of small neurons in the caudate and putamen with subsequent astrocytosis
• characteristic protein deposits form nuclear inlusions in neurons of HD patients
• with loss of cells, the head of the caudate becomes shrunken and there is ‘ex-vacuo’ dilation of the anterior horns of the lateral ventricles