Chapter 28: Neurodegenerative Disorders

Question 1

Disease characterized by slow-onset memory loss, impaired cognition, language difficulty, dementia, neuritic plaques, and neurofibrillary tangles

Answer 1

Alzheimer’s disease

Question 2

In Alzheimer’s disease, Neuritic plaques are compromised of 1___ which is derived from beta-cleavage of 2 ___ and neurofibrillary tangles are comprised of aggregates of 3____

Answer 2

1. A-Beta amyloid
2. amyloid precursor protein (APP)
3. hyperphosphorylated tau protein

Question 3

Disease characterized by chorea, dystonia, lateral ventricular enlargement, and caudate nuclei atrophy

Answer 3

Huntington’s disease

Question 4

<p>
In Huntington&#39;s disease, there is a trinucleotide repeat of 1\_\_\_ within the huntingtin gene and atrophy of the caudate occurs due to degeneration of 2\_\_\_\_\_ neurons.</p>

<p>
Bonus: What enzyme will also be deficient here?</p>

Answer 4

<p>
1. CAG 2. GABAergic</p>

<p>
Bonus: glutamic acid decarboxylase</p>

Question 5

With regard to Alzheimer’s disease, 1___ allele of APOE is associated with increased risk while 2____ allele is associated with decreased risk

Answer 5

1. e4

2. e2

Question 6

Trinucleotide repeat syndrome characterized by lack of coordination of the upper and lower limbs and often presenting with lower-limb areflexia, sensory loss, and extensor plantar reflexes

Answer 6

Friedrich ataxia

Question 7

Systemic abnormailities associated with Friedrich ataxia include

Answer 7

Scoliosis, diabetes mellitus, and hypertrophic cardiomyopathy

Question 8

Disease characterized by resting tremors, muscular, cogwheel rigidity, bradykinesia, and shuffling gait

Answer 8

Parkinson’s disease

Question 9

In Parkinson’s disease there is degenerative loss of 1____ neurons in the 2_____ of the basal ganglia. Within affected neurons are round eosinophilic inclusions of 3______ which are also known as 4____ bodies

Answer 9

1. Dopaminergic
2. substantia nigra pars compacta
3. alpha-synuclein
4. Lewy

Question 10

Histopathological studies of a patient presenting with muscular weakness, fasciculations, and wasting who later dies, reveals loss of neurons and mild gliosis in the anterior/ventral horn of the spinal cord. What disease did this patient have?

Answer 10

Amyotrophic lateral sclerosis

Question 11

Since the gene for amyloid precursor protein (APP) is found on chromosome 21, in a patient with Down syndrome and progressive loss of memory and cognition, neuropathologic studies of the cortex would reveal extracellular 1____ and intracellular, flame-shaped 2____

Answer 11

1. neuritic plaques

2. neurofibrillary tangles