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NEUROPATH 1 > Class 3 & 4 - Neurodegenerative Diseases > Flashcards

Class 3 & 4 - Neurodegenerative Diseases Flashcards

1
Q

In ALS, the most prominent loss of neurons occurs in which tract of the spinal cord?

A

lateral corticospinal tract

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2
Q

What is the prognosis for ALS?

A

incurable; 2-5 years after diagnosis

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3
Q

ALS involves a loss of _____ neurons from the _____ EXCEPT for…

A

motor neurons from the brain stem, except for the oculomotor nerve

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4
Q

10% of cases of ALS are _____

A

familial

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5
Q

What is dysarthria?

A

motor speech disorder from impaired movement of muscles used for speech

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6
Q

What is dysphagia?

A

difficulty swallowing

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7
Q

The progressive spinal muscle atrophy type of ALS sees a loss of the motor neurons in the…

A

anterior gray horn of the spinal cord

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8
Q

What is dysphonia?

A

strained, forced or breathy speech (hoarse voice)

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9
Q

What portion of cases of dystonia are idiopathic (primary)?

A

2/3rds

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10
Q

Which functions will remain normal in someone with ALS?

A 
oculomotor function
possibly intellect (in 50%)
urinary bladder function
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11
Q

What is the most common form of dementia?

A

alzheimer’s

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12
Q

Which regions of the spinal cord has the most prominent loss of neurons in ALS?

A

cervical and lumbar regions of spinal cord + brain stem, especially in the LATERAL CORTICOSPINAL TRACT

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13
Q

What is the most common cause of muscular dystonia?

A

2/3rds of dystonia are idiopathic/primary

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14
Q

What is the most common focal dystonia?

A

cervical dystonia

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15
Q

What are the eventual consequences of cervical dystonia?

A

OA, hypertrophy of SCM

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16
Q

What is the average age of onset for idiopathic dystonia?

A

8

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17
Q

What condition shows up as involuntary, sustained muscle contractions which cause twisting and repetitive motions?

A

dystonia

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18
Q

Secondary dystonia is a result of…

A

damage or scarring in small areas of the brain (from drugs, infections/encephalitis, tumors, demyelination/MS, acute traumatic brain injury, etc)

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19
Q

What is blepharospasm?

A

uncontrolled blinking/closure of the eyes for anywhere from seconds to hours

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20
Q

In adult-onset focal dystonia, when is maximal disability reached?

A

after 5 years (progresses to segmental dystonia)

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21
Q

What happens in the brain with Alzheimer’s disease?

A

amyloid substances (plaques) build up in between nerve cells in the brain, causing disconnection between the neurons –> cells atrophy

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22
Q

What are fasciculations?

A

spontaneous contraction affecting a small number of muscle fibers, often causing a flicker of movement under the skin

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23
Q

Cognitive impairment is noted in what percentage of ALS cases?

A

up to 50%

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24
Q

Indicate whether each disease mainly affects UMN, LMN, or both.

Pseudobulbar Palsy
Progressive Bulbar Palsy
Primary Lateral Sclerosis
Progressive Spinal Atrophy
Amyotrophic Lateral Sclerosis
A

pseudobulbar: UMN

progressive bulbar: UMN + LMN

primary lateral sclerosis: UMN

progressive spinal atrophy: LMN

amyotrophic lateral sclerosis: UMN + LMN

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25
In which ALS-type disease are fasciculations and muscle atrophy NOT seen?
primary lateral sclerosis
26
Progressive spinal muscle atrophy involves a loss of motor neurons in which part of the spinal cord?
anterior gray horn (LMN)
27
Which regions of the spinal cord see the greatest changes in ALS?
cervical + lumbar
28
In pseudobulbar palsy, damage mostly occurs in the _____. In progressive bulbar palsy, mainly the ____ are affected.
corticobulbar tract cranial nerve nuclei (except oculomotor system)
29
What is primary lateral sclerosis? What differentiates it from other types of ALS?
neuronal loss in the cortex, including the corticospinal tract muscle atrophy and fasciculations are NOT seen but there is hyperactivity of tendon reflexes and weakness/spasticity of affected muscles
30
In progressive spinal muscle atrophy, there is progressive loss of motor neurons found where?
in the anterior gray horn
31
What is the most common cause/form of dementia?
Alzheimers
32
5-10% of cases of Alzheimers are...
familial
33
Atrophy in Alzheimer's is mainly seen in...
the frontal and temporal cortices
34
Can Alzheimer's cause death?
Not directly
35
What are the primary risk factors for Alzheimer's?
age, family history, female gender gene mutations hypertension, diabetes, obesity
36
The onset of focal dystonia is usually between...
age 30 and 50
37
In secondary dystonia, what is seen in the brain?
damage or scarring in small areas
38
What is the most common focal dystonia?
cervical dystonia
39
What are 2 common complications of cervical dystonia?
osteoarthritis and SCM hypertrophy
40
What is the prognosis for the following: genetic dystonia beginning in childhood dystonia caused by brain damage close to birth (such as CP) adult-onset focal dystonia
childhood beginning: worsens over the years brain damage near birth: remains static adult-onset focal: progresses to segmental, maximal disability seen after 5 years
41
What are the three characteristics of Huntington's?
abnormal movement abnormal behaviour progressive dementia
42
What occurs in the brain in Huntington's disease?
the ventricles enlarge the caudate nucleus and the putamen atrophy the brain volume decreases up to 20%
43
What is the prognosis for Huntington's?
complete mental incapacitation + death within 20 years of onset
44
What are some observable manifestations of Huntington's disease? (Just name a few)
``` muscle rigidity abnormal eye movement abnormal gait dysarthria dysphagia sleep disorders incontinence (urinary) irritability/depression violence dementia ```
45
__% of restless leg syndrome cases have a familial linkage
60%
46
Restless leg syndrome is associated with a deficit of ___ in the ___
iron, substantia nigra
47
True or false: RLS is associated with a loss of dopaminergic neurons
false, just dysfunction at the junction between pre + post synaptic dopaminergic neurons
48
What is the most common autoimmune CNS disease?
MS
49
What is the leading neurological disease in young adults?
MS
50
What coexisting autoimmune disorders are seen with MS?
hashimoto's psoriasis IBS
51
Explain the pathogenesis of MS
* demyelination causes neuronal apoptosis --> brain atrophy * plaques disseminate in white matter * oligodendrocytes dystrophy * antibodies are present * T cells are sensitized to some component of myelin
52
What are some of the main S&S of MS?
``` blurred vision muscle weakness incoordination numbness impaired cognitive function insomnia neuropathic burning pain ```
53
How is MS diagnosed?
brain lesions evident in MRI's specific antibodies in CSF two separate sets of CNS symptoms occurring in at least 2 episodes
54
What is the prognosis for MS?
incapacitation (physically) in 20-30 years
55
Pathological changes in Parkinson's are mainly seen in...
the extrapyramidal system and the basal nuclei (degeneration of neurons)
56
Describe the pathogenesis of Parkinson's
degeneration of dopaminergic neurons in the substantia nigra
57
What are the 3 main S&S of Parkinson's
tremor (enhanced by stress)/muscle rigidity bradykinesia postural instability
58
The tremor in Parkinson's is usually...
unilateral and less prominent during voluntary movement
59
What is "cogwheel rigidity"?
jerky resistance to passive movement
60
What are 3 treatment options for Parkinson's?
drug therapy deep brain stimulation fetal cell transplantation
61
What are possible causes of Parkinsonism?
iatrogenic after a stroke infectious causes accumulation of manganese

NEUROPATH 1 (11 decks)

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