Degenerative Diseases

Question 1

Degenerative diseases are mostly characterized by what?

Answer 1

A loss of neurons

Question 2

• —Alzheimer Disease
• —Pick Disease/Frontotemporal Dementia
• —Diffuse Lewy Body Disease

are all examples of what type of degenerative disease?

Answer 2

cortical

Question 3

Where does neuronal loss occur for parkinson disease?

Answer 3

substantia nigra and locus ceruleus

Question 4

_____ gait is small, shuffling steps

Answer 4

festinating gait

Question 5

anteropulsion means falling ______

Answer 5

falling forward

Question 6

en block turning means no turning at the ____

Answer 6

torso

Question 7

Lewy bodies – round, eosinophilic inclusion

—Composed of:

►Alpha-_____

►Neuro_____

►Ubiquitin, _____

Answer 7

Lewy bodies – round, eosinophilic inclusion

—Composed of:

►Alpha-synuclein

►Neurofilaments

►Ubiquitin, Parkin

Question 8

What is shown in this image?

Answer 8

Parkinson disease:
Lewy bodies are present in dying neurons mainly in the dopaminergic areas (e.g. substantia nigra). The loss of pigmented neurons leads to pallor of the substantia nigra. Lewy bodies contain α-synuclein

Question 9

What does the following image show?

Answer 9

Huntington disease:
Hydrocephalus ex vacuo can develop due to loss of tissue in the caudate secondary to the neurodegeneration.

Question 10

What is shown in the following image?

Answer 10

Huntington disease:

There is loss of neurons (especially small, spiny neurons). Prominent gliosis is present.

Question 11

Tremor that —occurs at rest (Parkinson)

Answer 11

resting tremor

Question 12

Tremor that occurs when holding body part against gravity (Essential Tremor

Answer 12

postural

Question 13

—Tremor with voluntary movement (Cerebellar)

Answer 13

kinetic/action

Question 14

Movement that is repetitive, brief, rapid, purposeless, stereotyped movements that involve single or multiple muscle groups.

Answer 14

tic

Question 15

Excessive, spontaneous movements that are irregularly timed, nonrepetitive, randomly distributed, and often have a flowing “dancelike” quality that involves multiple body parts

Answer 15

chorea

Question 16

Rapid, shock-like, arrhythmic (usually), and often repetitive involuntary movements.

Answer 16

myclonus

Question 17

unilateral wild flinging movements (subthalamic nucleus lesion

Answer 17

hemiballismus

Question 18

—abnormal, often distorted positions of limbs, trunk, face; slower than athetosis

Answer 18

dystonia

Question 19

Spinocerebellar ataxias

• —Loss of neurons in cerebellum, brainstem, spinal cord; inclusions
• —Autosomal _____; some with repeats

Answer 19

Spinocerebellar ataxias

• —Loss of neurons in cerebellum, brainstem, spinal cord; inclusions
• —Autosomal dominant; some with repeats

Question 20

Ataxia-telangiectasia

• —Loss of neurons in _____ – Purkinje and granule cells
• —Degeneration of dorsal columns, spinocerebellar tracts, anterior motor neurons, and peripheral neuropathy
• —Telangiectasias in CNS, conjunctiva, and skin
• —Clinical – recurrent sinus infections, gait problems, dysarthria, eye movement problems
• —May develop T-cell leukemias or lymphomas, gliomas, and carcinomas
• —Gene product, ATM, is involved in response to breaks in double-stranded DNA

Answer 20

Ataxia-telangiectasia

• —Loss of neurons in cerebellum – Purkinje and granule cells
• —Degeneration of dorsal columns, spinocerebellar tracts, anterior motor neurons, and peripheral neuropathy
• —Telangiectasias in CNS, conjunctiva, and skin
• —Clinical – recurrent sinus infections, gait problems, dysarthria, eye movement problems
• —May develop T-cell leukemias or lymphomas, gliomas, and carcinomas
• —Gene product, ATM, is involved in response to breaks in double-stranded DNA

Question 21

What does this image show?

Answer 21

ALS