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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
Q

In which ALS-type disease are fasciculations and muscle atrophy NOT seen?

A

primary lateral sclerosis

26
Q

Progressive spinal muscle atrophy involves a loss of motor neurons in which part of the spinal cord?

A

anterior gray horn (LMN)

27
Q

Which regions of the spinal cord see the greatest changes in ALS?

A

cervical + lumbar

28
Q

In pseudobulbar palsy, damage mostly occurs in the _____. In progressive bulbar palsy, mainly the ____ are affected.

A

corticobulbar tract

cranial nerve nuclei (except oculomotor system)

29
Q

What is primary lateral sclerosis? What differentiates it from other types of ALS?

A

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
Q

In progressive spinal muscle atrophy, there is progressive loss of motor neurons found where?

A

in the anterior gray horn

31
Q

What is the most common cause/form of dementia?

A

Alzheimers

32
Q

5-10% of cases of Alzheimers are…

A

familial

33
Q

Atrophy in Alzheimer’s is mainly seen in…

A

the frontal and temporal cortices

34
Q

Can Alzheimer’s cause death?

A

Not directly

35
Q

What are the primary risk factors for Alzheimer’s?

A

age, family history, female gender
gene mutations
hypertension, diabetes, obesity

36
Q

The onset of focal dystonia is usually between…

A

age 30 and 50

37
Q

In secondary dystonia, what is seen in the brain?

A

damage or scarring in small areas

38
Q

What is the most common focal dystonia?

A

cervical dystonia

39
Q

What are 2 common complications of cervical dystonia?

A

osteoarthritis and SCM hypertrophy

40
Q

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

A

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
Q

What are the three characteristics of Huntington’s?

A

abnormal movement
abnormal behaviour
progressive dementia

42
Q

What occurs in the brain in Huntington’s disease?

A

the ventricles enlarge
the caudate nucleus and the putamen atrophy
the brain volume decreases up to 20%

43
Q

What is the prognosis for Huntington’s?

A

complete mental incapacitation + death within 20 years of onset

44
Q

What are some observable manifestations of Huntington’s disease? (Just name a few)

A 
muscle rigidity
abnormal eye movement
abnormal gait
dysarthria
dysphagia
sleep disorders
incontinence (urinary)
irritability/depression
violence
dementia
45
Q

__% of restless leg syndrome cases have a familial linkage

A

60%

46
Q

Restless leg syndrome is associated with a deficit of ___ in the ___

A

iron, substantia nigra

47
Q

True or false: RLS is associated with a loss of dopaminergic neurons

A

false, just dysfunction at the junction between pre + post synaptic dopaminergic neurons

48
Q

What is the most common autoimmune CNS disease?

A

MS

49
Q

What is the leading neurological disease in young adults?

A

MS

50
Q

What coexisting autoimmune disorders are seen with MS?

A

hashimoto’s
psoriasis
IBS

51
Q

Explain the pathogenesis of MS

A
  • 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
Q

What are some of the main S&S of MS?

A 
blurred vision
muscle weakness
incoordination
numbness
impaired cognitive function
insomnia
neuropathic burning pain
53
Q

How is MS diagnosed?

A

brain lesions evident in MRI’s
specific antibodies in CSF
two separate sets of CNS symptoms occurring in at least 2 episodes

54
Q

What is the prognosis for MS?

A

incapacitation (physically) in 20-30 years

55
Q

Pathological changes in Parkinson’s are mainly seen in…

A

the extrapyramidal system and the basal nuclei (degeneration of neurons)

56
Q

Describe the pathogenesis of Parkinson’s

A

degeneration of dopaminergic neurons in the substantia nigra

57
Q

What are the 3 main S&S of Parkinson’s

A

tremor (enhanced by stress)/muscle rigidity
bradykinesia
postural instability

58
Q

The tremor in Parkinson’s is usually…

A

unilateral and less prominent during voluntary movement

59
Q

What is “cogwheel rigidity”?

A

jerky resistance to passive movement

60
Q

What are 3 treatment options for Parkinson’s?

A

drug therapy
deep brain stimulation
fetal cell transplantation

61
Q

What are possible causes of Parkinsonism?

A

iatrogenic
after a stroke
infectious causes
accumulation of manganese

NEUROPATH 1 (11 decks)

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