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Clin Med Fall 2015 > Degenerative Diseases test 3 > Flashcards

Degenerative Diseases test 3 Flashcards

1
Q

Disorders of the CNS

A
  • Cerebrovascular Disease and Stroke
  • Degenerative diseases of CNS
  • infectious disorders (meningitis, encephalitis, abscess)
  • *typically affect adults & results in shorted lifespan
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2
Q

degenerative diseases of CNS

A
  • amyotrophic lateral sclerosis
  • alzheimer’s disease
  • parkingson’s disease
  • fredreich’s ataxia
  • huntington’s disease
  • multiple sclerosis
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3
Q

amyotrophic lateral sclerosis def.

A
  • amyotrophy: peripheral nerve changes resulting in mm fiber atrophy
  • Lateral: refers to the motor neurons in the lateral aspect of the spinal cord, brainstem, and crebral cortex that are involved
  • Sclerosis: degernation and scarring ( LMN, UMN)
  • no cure to stop or change process (mgmt of symptoms)
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4
Q

ALS

A
  • most common form of adult onset progressive motor neuron disorder
  • most physically devastating of the neurogenerative diseases
  • lose ability to eat, communicate and breath
  • no cognitive changes
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5
Q

ALS pathogensis

A
  • Destruction of UMNs of cerebral cortex
    • descend in corticospinal and corticobulbar tracts
    • leads to spasticity
  • can also affect LMNs (alpha motor neurons)
    • leads to denervation and m atrophy
  • does NOT involve cerebellum or frontal cortex (cognition)
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6
Q

ALS- Clinical Manifestations

A
  • Variable depending on whether UMN or LMN are predominately involved
  • early signs:
    • insidious asymmetrical weakness of distal aspect of one limb (hand or foot)
    • cramping w/ volitional mvmt-early morning stiffness
    • m fasculations : spontaneous twitching of mm fibers
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7
Q

ALS clincal manifestations ( mm/reflexes)

A
  • extensor mm weaker than flexors mm (especially in hands)
  • positive Hoffmann’s and Babinski’s signs
  • hyper-reflexive DTRs
  • clonus
  • eye mvmts and sensory, bowel and bladder function and cognition are preserved (may communicate w/ eyes in later stages)
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8
Q

ALS clinical manifestations ( neural)

A
  • progressive bulbar palsy:cranial nuclei involvement
    • weakness in swallowing, chewing, and facial gestures
    • flat affect of face( no control of mm)
    • may be on special diet of thickened food/water -> becomes unsafe-> feeding or GI tube
  • Progressive spinal mm atrophy
    • progressive loss of motor neurons in anterior horns of spinal cord (often first in cervical area) -UE typically more involved
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9
Q

ALS clinical manifestations (other systems

A

Respiratory complications
-shortness of breath, poor cough reflex
-will eventually deteriorate to respiratory failure
(may go on ventilator or get trach)
-increased risk of respiratory infections(cant clear secretions)
Oral Motor complications
-difficulty in food mgmt, chewing, lip closure, and swallowing

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10
Q

ALS prevalence

A
  • ranges from 4-6 individuals per 100,000 have ALS
  • more common in males
  • incidence is increased in pacific islands (Guam-possible environmental factors)
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11
Q

ALS etiology/Risk factors

A
  • 90% ALS cases occur sporadically and have unknown causes
  • Familial ALS (early onset-childhood)
    • autosomal inheritance - chromosome 21
    • autosomal recessive inhertitance -chromosome
  • infection by poliovirus (leads to autoimmune response)
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12
Q

ALS Diagnosis

A
  • clinical presentation (typically 30-40s)
  • EMG
    • fibrillations
    • fasiculations
  • Muscle biopsy: denervation atrophy
  • muscle enzymes:
    • CPK-creatine phosphokinase levels elevated(due to mm breakdown and denervation)
  • normal CSF, no changes on myelogram
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13
Q

ALS Dx

A

-Suspected ALS
-Possible ALS
-Probable ALS
-Definate ALS
( understand related to UMN/LMN symptoms and how many levels/extremities are affected)

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14
Q

ALS medical mgmt

A
  • no known method to stop clincal course of ALS
  • symptomatic therapy
    • anticholinergic drugs( control drooling)
    • Baclofen& Diazepam (spasticity)
    • difficulty in food mgmt; chewing, lip closure, and swallowing-may require modification of consistency and texture of foods and fluids
      - may lead to NG tube feding
    • Ventilator for respiratory failure
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15
Q

ALS Prognosis

A
  • “relentlessly” progressive
  • adult onset type:death usually within 2-5 years
    • usually die from comlications related to respiratory compromise-pneumonia
  • earlier onset likeyl live longer
  • goal is to maintain quality of life for as long as possible
    • respiratory tech
    • referral for assistive tech.
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16
Q

Alzheimer’s Disease (AD)

A

-progressive dementia, characterized by a slow decline in memory, language, visuospatial skills, personality, and cognition

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17
Q

Alzheimer’s Disease Pathphysiology

A
  • cell death and atrophy of cerebral cortex
  • progressive accumulation of insoluble fibrous material (amyloid)
  • senile plaques: amyloid material surrounded by cellular debris (fragmented axons, glial cells)
    • located mostly in cerebral cortex and hippocampus
  • neurofibrillary tangles: bundles of abnormal filaments within neurons accumulated in a tangled mass in cell bodies (limits transmission within CNS)
  • decrease in axonal transport of neurotransmitters
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18
Q

Alzheimer’s Disease incidence and prevalence

A
  • 4 million ppl in US
  • prevalence increases w/ each decade of life
    • 6% over 65
    • 20% over 80
    • 95% over 95
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19
Q

Alzheimer’s Disease risk factors/ etiology

A
  • nun study; looked at writing samples for complexity of cognition
  • individuals w/ higher level of thinking/ complexity and higher cognitive activity were less likely to get AD
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20
Q

Alzheimer’s Disease Clinical Pic of early stages

A
  • subtle personality changes
    • indifference, impulsivity, irritability, egocentricity
  • inability to learn new info
  • inability to handle money, balance a check book
  • diminished decsion making/judgements
  • visuospatial deficits
    • navigating the environment, cooking, manipulating mechanical objects in the home (increase risk of falls)
  • depression
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21
Q

Alzheimer’s Disease Clinical Pic of later stages

A
  • loss of older memories and recall of events
  • language deficits
  • delusion
  • agitation: sundowning ( get more agitated as the day goes on)
  • difficulty sleeping, eating
  • inappropriate sexual behavior
  • pt becomes mute and bedridden
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22
Q

Alzheimer’s Disease Clinical Pic ( motor function issues)

A
  • generalized weakness
  • stereotypical and rigid mvmts
  • postural reflexes diminish (inability to react to perturbation)
  • increased risk of falls (30% of individuals w/AD)
    • decreased perception, postural reflexes, ability to move adequately around objects
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23
Q

Alzheimer’s Stages of Decline

A

I :may have no noticeable symptoms, amyloid plaques form, may occur for many years
II :mild cognitive impairments;forgetful, unable to think of words
III : mild dementia, typical pt of dx, memory loss, changes in personality, diff to communicate - worry that pt will wander away
IV :moderate AD; increased confusion, cant remember who or where they are or why something is happening, increased memory loss, increased assistance need for ADLs, personality changes(agitation)- worry pt may wander away and get lost
V : severe; loss of ability to communicate , not speak, can’t eat become in competent, around the clock care is needed for ADLs

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24
Q

Alzheimer’s - 10 Warning signs

A
  1. recent memory loss which affects job performance
  2. difficulty in performing familiar tasks
  3. problems with language
  4. disorientation
  5. decreased judgment
  6. abstract thinking difficulties
  7. misplacing things
  8. changes in mood or behavior
  9. personality changes
  10. loss of initiative
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25
Q

Alzheimer’s Diagnosis

A
  • Rule out reversible dementia ( or cancer causing metabolic state changes)
    • blood count, chest films, general neuro exam
  • MRI and CT scan can identify brain atrophy
    • atrophy: similar changes with aging
    • neurofibrillary tangles and amyloid plaques not well identifies w/ imaging
    • autopsy to identify plaques and tangles
  • Mini-mental scale
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26
Q

mini mental scale

A
  • assessment of cognitive function ; series of questions testing current knowledge, visuospatial , and ability to follow simple directions
  • standard scores are available(out of 30) based on education received- can track progress over time
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27
Q

Alzheimer’s medical and surgical mangement

A
  • pharmacological mgmt
    • drugs which can lead to decreased amyloid plaques
  • long term care facility tailored to meet the sspecilaized need of individuals with Alzheimer’s
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28
Q

Alzheimer’s Prognosis

A
  • rate of decline is variable
  • 4th leading cause of death in adults
  • on average 7-11 years from dx to death
    • death secondary to dehydration or infection
29
Q

Parkinson’s Disease (Idiopathic Parkisonism)

A
  • chronic progression disease of the motor component of the CNS involving the basal ganglia
  • results in disturbances of tone, abnormal posturing and involuntary mvmts
30
Q

Parkinson’s Disease Pathophysiology

A
  • substantia nigra (basal ganglia) loses its ability to produce dopamine
    • 70-80% lost before see clinical manifestations
  • dopamine is essential for normal function of basal ganglia neurons
  • imbalance between excitatory and inhibitory pathways
31
Q

Anatomy of Basal Ganglia

A
  • loss of dopamine from substantia nigra ciompacta
  • direct pathway:thalamus is more active and increases mvmt
  • indirect pathway: decreased thalamus activity leading to decreased mvmt
  • decreased dopamine can get started and cant stop
32
Q

Parkinson’s Disease Pathophysiology

A
  • Significance of Basal Ganglia;
    • no direct tracts to motor output
    • actively involved in almost all types of mvmt ( postural response, spontaneous mvmts)
  • presentation not a weakness issue but rather a slowing of mvmt and poor coordination
  • why a decrease in dopamine?
    • mitochondria impairment
    • excess free radical formation
    • genetic/familial link
33
Q

Parkinson’s Disease Clinical Picture

A
  • tremor
  • rigidity(lack of transverse plane motion)
  • pill rolling (appear to be)
  • loss of arm swing and pelvic dissociation with gait
  • bradykinesia (slow mvmt)
  • akinesia ( disorder of mvmt initiation)
    • paucity of natural and automatic mvmts
  • diff to initiate mvmt, transition b/w mvmt, stop mvmt ( over time leads to decreases motion bc weakness and decreased ROM)
  • mask-like face/lack of expression (flat affect~rigidty/stiffness affect facial mm -> lack of facial mvmt)
  • garbled speech/drooling
34
Q

Parkinson’s Disease Clinical Picture-Posture

A 
-flexion of neck, trunk, hips,and knees
Gait:
-parkinsonian
-Retropulsion
-Festinating( shuffling)
Increased fall risk
35
Q

Parkinson’s Disease Clinical Picture cont.

A
  • weakness and fatigue once disease becomes generalized
  • generalized pain
  • dementia and intellectual changes in almost 50% of persons with PD
  • depression, apathy, passivity , lack of ambition
36
Q

Parkinson’s Disease Clinical Picture gait

A
  • lack of dissociation of trunk
  • lack of arm swing
  • small steps
  • diff to iniate/change mvmt directions
  • decreased LE ROM ( no hip extension)
  • tremor
  • UE/Trunk flexion
  • shuffling
37
Q

Parkinson’s Disease Incidence

A
  • 60-100 per 100,000 individuals
  • 1% of population older than 55
  • 2.6% population over 85
  • majority of cases begin b/w 50 and 79 y/o
    • 10% initial symptoms before 40 y/o (slower progression)
  • no male to female diff
38
Q

Parkinson’s Disease Diagnosis

A
  • via clinical symptoms
  • diff. dx;
    • depression; expressionless face, reduction on vol. activity
    • Hunington’s disease; rigidity and bradykinesia
39
Q

Parkinson’s Disease Medical and Surgical Mgmt

A
  • Pharmacological Mgmt
    • amantadine (MOA)
    • Anticholinergics (reduce excitatory activity to help restore balance)
    • Dopaminergics-Levadopa (significantly improves bradykinesia)
40
Q

Parkinson’s Disease Medical and Surgical Mgmt cont

A
  • fetal cell transplant
  • sterotactic surgery; thalamus, basal ganglia
  • deep brain stimulation
    • used once exhausted effects of meds to improve symtoms,
    • put in areas that cause inhibition of rigidity and stiffness
    • battery packs deliver cont stimulation to areas to improve function
41
Q

Parkinson’s Disease Prognosis

A
  • generally all clinical manifestations worsen progressively (at diff rates)
  • does not significantly reduce lifespan in those individuals who develop PD in 50 &60s
  • usually die from infection or other conditions associated with debilitation
42
Q

Friedrich’s Ataxia (FA) Spinocerebellar degeneration

A
  • most common form of early onset ataxia
  • characterized by degeneration of ascending and descending fibers of the spinal cord including the spinocerebellar tracts
43
Q

Friedrich’s Ataxia (FA) Spinocerebellar degeneration Onset

A
  • manifests b/w age of 8-15 y/o
  • heredity: 25% of offspring of affected parents develop the disorder
    • autosomal recessive
    • linked to long arm of chromosome 9
  • Incidence:1-2 in 100,000
44
Q

Friedrich’s Ataxia (FA) Spinocerebellar degeneration Pathogenesis

A
  • cell loss in DRG
  • secondary degeneration in posterior columns, corticospinal tracts and dorsal &ventral spinocerbellar tracts
  • corticobulbar tracts and cerebrum are spared
45
Q

Friedrich’s Ataxia (FA) Clinical Picture

A
  • disease usually manifests itself between 8-15 y/o typically before 20-25
  • ataxic gait (most common symptom)
  • staggering/lurching gait, wide BOS
  • clumsiness, tremor in limbs
  • mm tone: normal at rest, flexor spasms common
  • progressive weakness of limbs
  • cardiomyopathy: mm fiber loss and fibrosis (60% of pts)
  • dysarthria, nystagmus (20%)
  • sensory losses:pain, temp, light touch, vibration and position sense
  • MS deformities(2ndary complications): scoliosis, kyphoscoliosis, pes cavus
  • Mentation is usually persevered, can get dementia and decreased intelligence during course of disease
  • diabetes mellitus in 10% with another 20% with impaired glucose tolerance
46
Q

Friedrich’s Ataxia (FA) Diagnosis

A
  • clinical presentation is diagnositic
  • Clinical criteria for dx
    • onset ataxia before 25 y/o
    • progressive course
    • loss of DTRs
  • CT and MRI may be normal or show atrophy in cerebellum or spinal cord
  • diff dx: demyelinating forms of heredity and sensory neuropathies
47
Q

Friedrich’s Ataxia (FA) prognosis

A
  • variable
  • 95% pts are using WC by age 45
  • on average lose ability to walk 15 years after onset of symptoms
  • mean age death mid 30s but survival into 50&60s
48
Q

Multiple Sclerosis (MS)

A
  • def: disease of CNS-white matter

- characterized by inflammation, edema, and demyelination

49
Q

Multiple Sclerosis (MS) pathophysiology

A
  • thought to be virus induced auto-immune disease
  • inflammatory process, body’s own immune system destroys myelin due to presence of Tcells and lymphocytes
  • cell bodies and axons preserved early on
  • if oliogdendroctyes remain present can get some remyelination following demyelination (remitting or relapsing course)
  • when myelin is destroyed -> impaired neural transmission
  • plaques of demyelination and scarring (gliosis) are present {gliosis->sclerotic appearance of tracts}
50
Q

Multiple Sclerosis (MS) pathophysiology location

A
  • occurs primarily in white matter with predilection for lateral and posterior columns especially in cervical region, optic nerve & chaism, and periventricular area
  • tracts in midbrain , pons, and cerebellum also affected
  • as disease progresses gray matter in cerebrum and spinal cord may be affected
51
Q

Multiple Sclerosis (MS) Incidence

A
  • less than 1 per 100,000 in equatorial areas
  • 6 to 14 per 100,000 in southern US
  • 30 to 80 per 100,000 in Canada, northern Europe and Northern US
  • white> black
  • female> male
  • 15% with MS have an affect relative
52
Q

Multiple Sclerosis (MS) Etiology

A

Infections

  • canine, distemper, rabies, measles, herpes simplex
  • may be dormant and then become active after many years
  • Infection->demyelination->inflammatory response->produce antibodies
  • Infection-> autoimmune response against CNS-> demyleinantion
53
Q

Multiple Sclerosis (MS) Diagnosis

A
  • diagnosis of exclusion( rule out other possibilities)
    • clinical judgement based on observation over time
  • early:
  • later:
  • CT, MRI, Myelography, EEG, Blood analysis
  • presence of plaques is not correlaated with disease progression / symptomatology
54
Q

Multiple Sclerosis (MS) Clinical course

A
  • highly variable and unpredictable
  • usually have periods of remission
  • benign (20%)
  • exacerbating/remitting (20-30%)
  • remitting-progressive (10-20%)
55
Q

Multiple Sclerosis (MS) Clinical course

A

-Exacerbations

56
Q

Multiple Sclerosis (MS) Prognosis

A
  • life expectancy is not significantly altered by MS
  • onset:middle age
  • increased frqeuency of episodes or increased progression rate= poorer prognosis
57
Q

Multiple Sclerosis (MS) Clinical Picture (Sensory)

A

-

58
Q

Multiple Sclerosis (MS) Clinical Picture (Motor )

A

-

59
Q

Multiple Sclerosis (MS) Clinical Picture (Tone)

A

-

60
Q

Multiple Sclerosis (MS) Clinical Picture (Charot’s triad)

A

-

61
Q

Multiple Sclerosis (MS) Clinical Picture (Mvmt disorders)

A

-

62
Q

Multiple Sclerosis (MS) Clinical Picture (Fatgiue)

A

-

63
Q

Multiple Sclerosis (MS) Clinical Picture (Visual disturbances )

A

-

64
Q

Multiple Sclerosis (MS) Clinical Picture (bowel/bladder/sexual disturbances)

A

-

65
Q

Multiple Sclerosis (MS) Clinical Picture (cognitive deficits)

A

-

66
Q

Multiple Sclerosis (MS) Clinical Picture (behavioral deficits)

A

-

67
Q

Multiple Sclerosis (MS) Clinical Picture (communication)

A

-

68
Q

Multiple Sclerosis (MS) Clinical Picture

A
  • sensory
  • motor
  • tone
  • Charcot’s Triad
  • Mvmt disorders
  • fatigue
  • visual disturbances
  • bowel/bladder/sexual disturbances
  • cognitive deficits
  • behavioral deficits
  • commuication
69
Q

Multiple Sclerosis (MS) medical mgmt

A
  • directed toward immune system dysfunction
  • megadoses of corticosteriods
  • interferon beta -1b
    • has demonstrated reduction in size and presence of plaques on MRI
  • Treatments of symptoms with drugs
    • spasticity, pain, depression

Clin Med Fall 2015 (10 decks)

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