Clin Med: Neuro II Flashcards
Define essential tremor
movement disorder that is characterized by intention tremors
How does an essential tremor progress?
progresses slowly & benign
What are the two types of tremors?
- resting
- action
Define a resting tremor
tremor occurs at rest/with gravity
Define action tremor
voluntary muscle contraction
Define a postural action tremor
holding position against gravity
Define a postural action tremor
muscle contraction against object
Define an intention action tremor
w/ voluntary muscle movement when going to do something
Essential tremor age prevalence
bimodal
- age 20 then 65yos
Essential tremor RFs
FHx
- pattern of autosomal dominant
Essential tremor pathophys
- neurodegeneration involving cerebellum
- abnormal gamma amino-butyric acid (GABA) function resulting in reduced tone, localized in cerebellum & locus coeruleus
Essential Tremor Hx
- starts in hands or forearms, bilateral
- Can be postural (holding arms outstretched) or intention (writing, eating, pouring)
- sometimes present w/ head tremor
- 2/3 or pts–> relief w/n 15mins after drinking alcohol
Ask about family history, medications, use of stimulants
Amphetamine, caffeine, pseudoephedrine, meth, cocaine
Essential Tremor: things to ask about
- FHx
- meds,
- use of stimulants
–> Amphetamine, caffeine, pseudoephedrine, meth, cocaine
Essential Tremor PE
neuro exam- make sure there is no other cause
Essential Tremor Dx
clinically dx
- based on hx & neuro exam
- Tremor Task Force of the International Parkinson & Movement Disorder Society (IPMDS) dx criteria
Describe the criteria of the Tremor Task Force of the International Parkinson & Movement Disorder Society (IPMDS)
- Bilateral upper limb action tremor w/ or w/o tremor in other: locations such as head, voice, or lower limbs
- duration ≥ 3 years
w/o other neuro signs such as dystonia, ataxia, or parkinsonism
Med Tx for Essential Tremor interfering w/ ADLs
- 1st line: Propranolol
- 2nd line: Benzos
- Gabapentin
- Botulinum Toxin (Botox)
Essential Tremor procedures that can be done if meds don’t work
Deep brain stimulation
Define Parkinson’s Dz
a progressive movement disorder characterized by bradykinesia, resting tremor, muscular rigidity & loss of postural reflexes
Parkinson’s Dz gender prevalence
Male > Female
Parkinson’s Dz RFs
- FHx
- environmental factors include
–> consumption of well water
–> agricultural occupations
–> exposure to pesticides
Parkinson’s Dz pathophys
degeneration of dopamine neurons in substantia nigra leads to dopamine depletion in striatum & characteristic motor symptoms
What role does dopamine play in the substania nigra?
Dopamine produced in the substantia nigra communicates w/ the motor cortex to initiate movement
Motor signs & symptoms of Parkinson’s Dz
- resting tremor
- Bradykinesia
- rigidity
- worsened handwriting/dexterity
- postural instability
Nonmotor symptoms of Parkinson’s Dz
- pain
- fatigue
- sleep disturbances
- mood disorders
- cognitive changes
- hallucination/delusion
Parkinson’s Dz PE
- Bradykinesia
- little facial expression
- quiet, stuttering, or monotonous speech
- “pill rolling tremor” resting tremor- improves w/ movement
- Cogwheeling (spastic movement, even w/ passive motion)
- Stooped gait/posture
- Shuffling gait
- DOES NOT PRODUCE WEAKNESS
- Seborrheic Dermatitis
Parkinson’s Dz Dx
- clinical
- rule out other movement disorders
- good response test dose to Levodopa & CAN be used for tx
Parkinson’s Dz Tx: Meds
Amantadine (loses efficacy over time)
Carbidopa/Levodopa
Dopamine agonists
Benztropine- anticholinergic effective for tremors
Parkinson’s Dz Tx: Non-Pharm
Physical/occupational therapy
Tai Chi
Encourage exercise
Counseling
In Parkinson’s Dz, if meds are ineffective, you can do…
deep brain stimulation
Define Huntington Dz
- rare autosomal dominant
- neurodegenerative disorder characterized by progressive motor, cognitive & psychiatric dysfunction
Huntington’s Dz RFs & onset age.
- FHx
- 30-50yo
Huntington’s Dz genetic pattern
autosomal dominant
Huntington’s Dz pathophys
- The gene that codes for glutamine repeats too many times, causing too much production of glutamine
–> Higher # of repeats the more serious & earlier the onset - The glutamine settles in the basal ganglia
Huntington’s Disease Hx
- about 2/3 of pts present w/ initial neuro manifestations such as:
changes in eye movements - decr coordination
- minor involuntary movements
- alt in executive functioning
- depressed or irritable mood
- chorea
Huntington’s Dz HEENT PE
ocular motor apraxia
Huntington’s Dz Neuro PE
- impaired fine motor skills present early in dz
- mild hyperreflexia
- choreatic movements spread to all muscles & can’t be voluntarily suppressed
- inability to maintain steady grip
Huntington’s Dz Psych PE
- depression, apathy, anxiety, aggression, paranoid, hypersexual
- decr conc., memory loss, loss of awareness
Is Huntington’s a clinical Dx?
YES
- based on FHx, progressive motor abnl (chorea), mental changes
How to confirm Huntington’s Dz Dx?
DNA analysis
Why get a MRI w/ Huntington’s Dz?
evaluates level of cerebral/caudate nucleus atrophy
Is there a cure of Huntington’s Dz?
NO
Are their meds that delay the progression of Huntington’s Dz?
NO
Huntington’s Dz Tx
- Haloperidol for behavioral disturbances
- Amantadine
- Genetic counseling- screen all 1st-degree relatives
Most cerebral palsy pts survive ___ years after onset
10-25
Define Cerebral Palsy
non-progressive disorder involving movement & posture due to a perinatal or neonatal
Cerebral palsy RFs
- prenatal exposure to toxins or inflammation
- fetal growth restriction
- birth defects & congenital malformations
- genetics
- premature or postdates delivery
- neonatal complications
- perinatal complications
Cerebral Palsy Hx
- difficulty swallowing (may be first noticeable symptom)
- motor delay (not meeting developmental milestones)
- stiff muscle (spasticity)
- abnl in gait & ambulation
- cognitive or learning impairment
- speech & language problems
- seizure
Most common motor delays in Cerebral Palsy include:
- not sitting by 8mos
- not walking by 18mos
- early hand preference before age 1yr
When should you refer a child for suspected cerebral palsy?
delayed motor milestones or persistent toe walking
What question should be asked to a parent of child with suspected CP, to assess developmental monitoring?
“Do you have any concerns about your child’s development?”
What ages should you do a developmental screening in children with CP?
9, 18, 24-30 months
In children w/ the following early motor features of CP, especially if known RFs for CP are present
- movement abnormalities
- tone abnormalities including hypotonia, spasticity, or dystonia
- abnormal motor development including delayed head control, rolling, and crawling
- feeding difficulties
Cerebral Palsy tx
multiple disciplinary team to tx various symptoms
Cerebral Palsy main goal of tx
Incr level of function & have the most normal life as possible
What meds may a child require
- Baclofen, seizures, GERD/vomiting
Define restless leg syndrome
a neurologic disorder characterized by the irresistible urge to move the legs
- worse at rest, relieved w/ movement & occurring in the evening or at night
Restless Leg Syndrome: gender most & age
- women > men
- all ages but >65yo
Meds that exacerbate restless leg syndrome
- antidepressants
- anticonvulsants
- antihistamines
- beta blockers
- lithium
- serotonin reuptake inhibitors
Restless Legs Syndrome Patho
Binding dysfunction of dopamine to presynaptic & postsynaptic receptors in the basal ganglia
- Dopamine issue w/ the basal ganglia
Restless Legs Syndrome Hx
- overwhelming urge to move legs
- partial or complete relief of urge sensation by movement, for as long as movement continues
- Circadian pattern w/ high freq in evening & at night often interfering w/ sleep
Restless leg syndrome Dx
- Rule out things that can make this worse
- iron studies (related to IDA)
- kidney dz, DM, Vit B12 def, folic acid def, thyroid dysfunction
- “levodopa” test- a single decr symptoms for about 2 hrs
Can Levadopa be used as tx for restless leg syndrome?
NO
1st line tx for restless leg syndrome
Gabapentin/Pregabalin
Restless Leg Syndrome Tx
- Tx of any underlying issues (iron def, thyroid, etc)
- Avoidance of alcohol, caffeine, nicotine
- Sleep hygiene
- Gabapentin/Pregabalin 1st line tx
Define Bell Palsy
inflammation & weakness of the 7th cranial nerve
- facial nerve
Two causes of Bell Palsy
idiopathic or 2nd toherpes simplex 1 infx
The highest incidence of Bell Palsy is b/t what ages?
15 - 45yo
Is testing needed for diagnosis Bell Palsy?
NO
Bell Palsy (suspicion of underlying dz ) Dx Labs
- CBC
- test for syphilis
- HIV test
- fasting glucose
- Lyme titer
- ANA titer (Lupus)
Bell Palsy Tx
- Eye protection & artificial tears (risk of corneal abrasions)
- Steroids (usually prednisone)
- (+/-) antivirals
Define Diabetic Peripheral Neuropathy
Periph neuropathy resulting from damage to the PNS, described as pain &/or impaired sensations in extremities
What % of DM pts will develop diabetic periph neuropathy?
50%
RFs for Diabetic Periph Neuropathy
- Poor glycemic control
- Smoking
- HTN
- Dyslipidemia
Diabetic Peripheral Neuropathy is assoc w/ what other conditions?
diabetic retinopathy & nephropathy
Diabetic Periph Neuropathy patho
Intracellular hyperglycemia leads to:
- Mitochondrial dysfunction
- Incr oxidative stress
- Incr inflammatory injury
Diabetic Periph Neuropathy Tx
- PREVENTION IS KEY (optimize glucose control)
- Meds
- Weight bearing exercise
- May require referral to neurologist or pain management
- Foot inspection at every visit
1st line Med Tx for Diabetic Periph Neuropathy
OTC pain relievers
- doesn’t control pain well
2nd line Med Tx for Diabetic Periph Neuropathy
- Pregabalin (Lyrica)
- Duloxetine (Cymbalta)
- Gabapentin (Neurontin)
Guillain Barre Syndrome aka
Acute idiopathic polyneuropathy
Describe Guillian Barre Syndrome
Demyelinating dz of the PNS
Guillain Barre Syndrome gender prevalence
men>women
When does Guillain Barre Syndrome usually occur?
1-4 after a resp or GIT infx
What infxs are commonly assoc. w/ Guillain Barre Syndrome?
Campylobacter jejuni > CMV, EBV, Influenza A, mycoplasma pneumoniae, hepatitis
Guillain Barre Syndrome pathophys
PNS myelin sheath is attacked by the pts own immune system
- In campylobacter, there is an lipooligosaccharide on the membrane the bacteria that is identical to a ganglioside on the neuron
Guillain Barre Syndrome Hx:S/S
- progressive symmetrical weakness & sensory of distal legs & arms (starts in arms)
- recent infx
What symptoms of autonomic dysfunction are common in Guillain Barre Syndrome?
- Cardiac arrhythmia
- Excessive sweating
- nausea,abdo pain, constipation
- urinary retention
Guillain Barre Syndrome PE
- cardiac arrhythmias or resp distress
- muscle weakness & sensory abnormalities
- reduced/absent tendon reflexes (may be normal in early states)
How is Guillain Barre Syndrome usually diagnosed?
clinical
Guillain Barre Syndrome Dx
- CSF–> elevated PROs + normal WBCs
- nerve conduction studies & EMG
- PFT @ initial exam & intermittently
Guillain Barre Syndrome Tx
- hospital req (2-4 wks) ensure timely transfer to ICU as indicated
- Immunoglobulin OR plasmapheresis STAT
- monitor for life-threatening manifestations: resp failure, arrhythmias, dysphagia, ileus
- Early PT & psychosocial support
When can a pt w/ Guillain Barre Syndrome be discharged?
pt can walk & symptoms have stopped progressing
In Guillain Barre Syndrome what is reported as the cause of death?
resp failure, infx, or autonomic dysfunction w/n those first 2-4 wks
