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Physiology > 2. Neurological Disorders > Flashcards

2. Neurological Disorders Flashcards

1
Q

childhood onset =

A

developmental disability (DD)
ex: intellectual disability, autism, cerebral palsy, epilepsy, neurological impairment, traumatic brain injury

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2
Q

down syndrome

A
  • cause: trisomy 21, extra copy of chromosome 21
  • also instances of trisomy mosaic
  • trisomy can result in spontaneous miscarriage
  • can be detected from prenatal testing
  • higher risk in older parents but most are born to younger women
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3
Q

down syndrome characteristics

A
  • affects development of CNS and structure of face and mouth
  • narrow, higher arched palate, underdeveloped midface
  • large range of IQ
  • motor function: hypotonia, delayed motor milestones
  • brains are smaller and cortex is affected more than subcortical structures
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4
Q

associated medical problems with down syndrome

A
  • surgery for heart may be needed
  • vision: strabismus (eyes don’t work together), myopia (nearsighted), spontaneous nystagmus (abnormal eye movements)
  • short stature
  • obesity
  • shorter life expectancy (~60)
  • tongue may protrude (tongue thrust), hard to understand their speech
  • increased risk of seizure disorders
  • high risk of early onset alzheimer’s due to gene for key protein (beta amyloid precursor protein APP) on chromosome 21
  • cervical spine instability - “atlantoaxial subluxation”: too much movement at joint between skull and cord, danger of spinal cord injury in positioning
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5
Q

down syndrome treatments

A
  • OT, PT, speech therapies
  • special education and social services
  • support groups
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6
Q

down syndrome and dental care

A
  • increased frequency of gum disease is major problem (immune system dysfunction)
  • abnormal teeth development (shape, numbers, baby teeth retention, late eruption, different eruption sequence)
  • mismatch tongue and mouth size
  • behavior management issues
  • seizures
  • cervical spine instability
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7
Q

epilepsy

A
  • seizure disorder
  • recurrent, unprovoked seizures
  • spontaneous, recurrent, simultaneous, massive discharges of neurons (all generate AP at same time)
  • seizure focus: region of brain where abnormal activity begins
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8
Q

causes of epilepsy

A
  • brain structural differences (developmental disabilities, CP, DS, others)
  • genetic mutations in genes affecting receptors and/or transmitters in the brain
  • open or closed head injury (scar tissue can become a seizure focus)
  • autoimmune epilepsy: Abs to key brain proteins
  • paraneoplastic syndrome, epilepsy secondary to cancer/tumor, class of autoimmune epilepsy
  • brain tumors (first symptom is seizure, mechanical injury)
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9
Q

types of seizures

A
  • over 40 types, can vary
  • can last from a few seconds to a few minutes (<5)
  • old terms: grand mal = tonic clonic
  • petit mal = absence (childhood)
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10
Q

seizure classification scheme

A
  • focal onset
  • generalized onset
  • unknown onset
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11
Q

2 types of focal seizures

A
  • focal aware
  • focal impaired
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12
Q

focal seizures

A
  • abnormal electrical activity is restricted
  • symptoms are limited and reflect the function of the part of the brain affected
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13
Q

focal aware

A
  • movement or sensory experience with no loss of consciousness or postural control
  • if restricted to motor cortex, movements start in one part of body and move across the body
  • progression reflects map of the body in motor cortex (Jacksonian march of epilepsy)
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14
Q

focal impaired

A
  • temporal lobe origin, complicated movement like lipsmacking
  • patients are not fully conscious and might not remember seizure
  • may be preceded by “aura” or may have no warning at all and occur unpredictably
  • can begin as focal and spread/generalize
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15
Q

2 types of generalized seizures

A
  • motor (grand mal, tonic clonic)
  • nonmotor (petit mal, absence)
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16
Q

generalized motor seizure

A
  • immediate loss of consciousness and falling
  • tonic phase: increased muscle tone, body rigid all over, followed by clonic phase
  • clonic phase: back and forth movement of all body parts (alternating contraction of agonists and antagonists)
  • often loss of bladder control
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17
Q

seizure triggers

A
  • environmental trigger
  • ex: flashing lights or loud sounds
  • only happens in some people
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18
Q

seizure first aid and precautions

A
  • generalized motor are not dangerous
  • falling, drowning, car accidents will cause injury
  • exception: status epilepticus (prolonged, >5mins, or repeated seizures with no recovery in between; medical emergency, treated by IV drugs and life support)
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19
Q

seizure durations and postictal period

A
  • duration varies, few seconds to few mins (longer than 5 mins is med emergency)
  • recovery time also varies (called post-ictal period)
  • recovery can be few minutes of confusion to several hours of deep sleep
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20
Q

generalized nonmotor seizure

A
  • in children, frequently outgrown
  • look like staring spells
  • abnormal activity over brain but no loss of postural tone (no falling)
  • may seem like child is daydreaming or not paying attention, might not be recognized as seizure
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21
Q

treatment of seizures

A
  • goal: control/prevent seizures
  • risk of death in epilepsy: SUDEP, sudden unexpected death in epilepsy (1:1000 epilepsy patients, 1:150 uncontrolled patients)
  • medications
  • modified diets
  • brain surgery
  • vagal nerve stimulator, VNS
22
Q

electroencephalogram (EEG)

A
  • brain wave recording
  • non invasive
  • electrodes are placed on scalp to record activity of cells
  • find “seizure focus” if there is one and determine type of seizure
  • usually takes under an hour
  • often no seizure observed, just abnormal activity
  • sometimes prolonged recording over days = long term monitoring
  • in-patient procedure
23
Q

anti-epileptic drugs (AEDs)

A
  • some seizures can be controlled/prevented by medication
  • 25 AEDs, also used for psychiatric conditions, bipolar, mood stabilizer, pain, migraines, RLS
  • varies on how many drugs to take and whether it works
  • “breakthrough seizures” even if pt is compliant with meds
24
Q

AED side effects

A
  • sedation
  • weight gain/loss
  • dilantin can cause gingival overgrowth, needs surgery
  • AEDs can interact with other drugs and with each other
25
Q

diet as epilepsy treatment

A
  • tightly controlled diet, mainly fat
  • changes metabolism
  • brain uses ketone bodies for energy
  • decreases or eliminates seizures in some pt
  • variations: ketogenic, modified Atkins, low glycemic index
26
Q

brain surgery as epilepsy treatment

A
  • remove seizure focus: invasive EEG with electrodes on dura can determine exact location and extent of seizure focus for surgery
  • cut corpus callosum (axons interconnecting the two hemispheres): prevent abnormal activity spreading from one hemisphere to the other
  • hemispherectomy: entire hemisphere removed in kids with severe epilepsy
27
Q

VNS as epilepsy treatment

A
  • vagal nerve stimulator
  • implanted pacemaker-like device that delivers electrical stimulation to the brain via vagus n.
  • can be activated externally by a magnet
28
Q

multiple sclerosis (MS)

A
  • age 30s
  • women 2 or 3:1 ratio
  • uneven geographic distribution suggests environmental factor
  • further from equator, MS more likely
29
Q

what happens in CNS with MS?

A
  • autoimmune process causes inflammation and destruction of myelin
  • loss of myelin of axons at multiple sites in CNS
  • replaced with scar tissue –> sclerosis
  • areas of inflammation/demyelination are called plaques (white spots on MRI)
30
Q

risk of MS increased dramatically after infection with ___, leading to conclusion that infection with this virus causes MS

A

epstein barr virus (EBV)

31
Q

symptoms of MS

A
  • sensory, motor, cognitive, affective (all depends on CNS site)
  • conduction of nerve impulses are impaired
  • increased frequency of seizures
  • worsening of symptoms with higher environmental temperatures, conduction of nerve impulses slows down or fails
  • progressive disease
32
Q

4 types of MS

A

several subtypes of MS, vary in speed of progression (varies in amount of myelin being lost over time)
1. progressive-relapsing (5%)
2. secondary-progressive (after 10 years)
3. primary-progressive (10%)
4. relapsing-remitting (85%; can transition to secondary-progressive)

33
Q

progressive-relapsing MS

A
  • 5%
  • steady decline since onset with superimposed attacks
  • graph is steadily worse with some bad peaks in between
34
Q

secondary-progressive MS

A
  • initial relapsing-remitting MS that suddenly begins to have decline without periods of remission
  • graph gets steadily worse with a few bad peaks and flat lines (no change)
35
Q

primary-progressive MS

A
  • 10%
  • steady increase in disability without attacks
  • graph gets steadily worse (linear graph)
36
Q

relapsing-remitting MS

A
  • 85%
  • unpredictable attacks which may or may not leave permanent deficits followed by periods of remission
  • graph gets worse with peak, remission, peak, remission, and so on
37
Q

treatment for MS

A
  • no cure
  • disease modifying medications that slow progression and alter immune response
  • first major treatment is beta-interferon
  • 12 medications approved
38
Q

MS and pregnancy

A
  • MS occurs in women of childbearing age
  • pregnancy reduces number of MS exacerbations with increase after pregnancy (increase in naturally-occurring immunosuppressants)
  • none of the meds can be used while pregnant or breast feeding
  • may be hard to physically care for children if motor/cognitive function impaired
39
Q

alzheimer’s disease causes

A
  • cause unknown
  • early onset before 65
  • may be genetic; mutations in APP, PSEN1, PSEN2 genes
  • genetic contribution: apolipoprotein E (APOE) 3 forms (APOE e2, e3, e4)
40
Q

APOE e2

A
  • least common
  • reduce risk of Alzheimers
41
Q

APOE e4

A
  • more common than e2
  • increase risk of Alzheimers
  • not a determinant but increases risk
42
Q

APOE e3

A
  • most common
  • doesn’t seem to affect risk of Alzheimers in either direction
43
Q

apolipoprotein E combines with __ to form lipoproteins and carries __ in the bloodstream

A

fats, fats

44
Q

alzheimers symptoms

A
  • dementia
  • loss of cognitive function and memory
  • progressive neurological disease that affects more and more of the brain
  • motor functions remain much longer but eventual loss of voluntary movement, bladder, bowel control
  • death from medical causes (infections, pneumonia, etc.)
45
Q

dementia

A

progressive decline in memory, mental function, acquired intellectual skills

46
Q

other causes of dementia besides AD

A
  • vascular dementia/cognitive impairment: comes from impaired blood supply (stroke or smaller bleeds or blockages)
  • dementia with Lewy bodies: abnormal accumulations of protein, alpha-synuclein
  • mixed dementia: combo of vascular dementia, AD, and dementia with Lewy bodies
47
Q

CNS changes in AD

A
  • pathological, microscopic changes in brain
  • cerebral cortex and subcortical structures of the limbic system, structures mediating memory (hippocampus) and affect (limbic system) are affected earliest
  • AD starts in one specific structure of brain and then spreads
  • microscopic changes in cortex; neurons die eventually
48
Q

plaques in AD

A

contain amyloid protein and degenerated cells (precursor for this protein coded by gene on chromosome 21)

49
Q

tangles in AD

A

in pyramidal cells (intracellular)

50
Q

in AD, __ __ degenerates

A

nucleus basalis

51
Q

treatments for AD

A
  • no cures
  • change levels of transmitters (keep ACh levels high by blocking the enzyme that degrades it - “aricept”)
  • drug to work on a different transmitter system, glutamate (“namenda” - blocks one class of glutamate receptors); too much glutamatergic activity may cause cell damage
  • drugs to block cellular changes and antibodies against the abnormal proteins
52
Q

3 common issues in neurological disorders that relate to dental care

A
  • mobility impairments
  • fine motor control
  • money

Physiology (14 decks)

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