2. Neurological Disorders Flashcards
1
Q
childhood onset =
A
developmental disability (DD)
ex: intellectual disability, autism, cerebral palsy, epilepsy, neurological impairment, traumatic brain injury
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
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
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
5
Q
down syndrome treatments
A
- OT, PT, speech therapies
- special education and social services
- support groups
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
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
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)
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)
10
Q
seizure classification scheme
A
- focal onset
- generalized onset
- unknown onset
11
Q
2 types of focal seizures
A
- focal aware
- focal impaired
12
Q
focal seizures
A
- abnormal electrical activity is restricted
- symptoms are limited and reflect the function of the part of the brain affected
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)
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
15
Q
2 types of generalized seizures
A
- motor (grand mal, tonic clonic)
- nonmotor (petit mal, absence)
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
17
Q
seizure triggers
A
- environmental trigger
- ex: flashing lights or loud sounds
- only happens in some people
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)
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
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
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
