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
diet as epilepsy treatment
- 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
brain surgery as epilepsy treatment
- 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
VNS as epilepsy treatment
- 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
multiple sclerosis (MS)
- age 30s
- women 2 or 3:1 ratio
- uneven geographic distribution suggests environmental factor
- further from equator, MS more likely
29
what happens in CNS with MS?
- 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
risk of MS increased dramatically after infection with ___, leading to conclusion that infection with this virus causes MS
epstein barr virus (EBV)
31
symptoms of MS
- 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
4 types of MS
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
progressive-relapsing MS
- 5%
- steady decline since onset with superimposed attacks
- graph is steadily worse with some bad peaks in between
34
secondary-progressive MS
- 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
primary-progressive MS
- 10%
- steady increase in disability without attacks
- graph gets steadily worse (linear graph)
36
relapsing-remitting MS
- 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
treatment for MS
- no cure
- disease modifying medications that slow progression and alter immune response
- first major treatment is beta-interferon
- 12 medications approved
38
MS and pregnancy
- 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
alzheimer's disease causes
- 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
APOE e2
- least common
- reduce risk of Alzheimers
41
APOE e4
- more common than e2
- increase risk of Alzheimers
- not a determinant but increases risk
42
APOE e3
- most common
- doesn't seem to affect risk of Alzheimers in either direction
43
apolipoprotein E combines with __ to form lipoproteins and carries __ in the bloodstream
fats, fats
44
alzheimers symptoms
- 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
dementia
progressive decline in memory, mental function, acquired intellectual skills
46
other causes of dementia besides AD
- 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
CNS changes in AD
- 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
plaques in AD
contain amyloid protein and degenerated cells (precursor for this protein coded by gene on chromosome 21)
49
tangles in AD
in pyramidal cells (intracellular)
50
in AD, __ __ degenerates
nucleus basalis
51
treatments for AD
- 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
3 common issues in neurological disorders that relate to dental care
- mobility impairments
- fine motor control
- money
