Neuro 5 degenerative

A. Leah Kelly EdD, APRN, BC

Degeneration
* Can be in the CNS- as in

MS, Parkinsons, Alzheimer's, Huntington's

* Can be in Cord- as in ALS,

MS
* Can be in PNS- as in GB,

Charcot-Marie tooth
* Can be in synapse- as in

MG, radiculopathy
* Can be in cranial nerves-

Bells Palsy

Multiple Sclerosis
* A progressive, chronic, immunogenetic degenerative disease of the myelin sheath * Probably T cell mediated autoimmune * Lots of ongoing research so stay tuned
* Seems genetic susceptibility & VIRUS turn on * Or/& environmental triggers * Immune T cells are activated by myelin antigen and adhere to endothelium thinking the virus is myelin & macrophages eat the myelin

MS Patho contd

* Myelin sensitive lymphocytes get into CNS

* Myelin is destroyed- eaten by macrophages & get plaques * More inflammatory response and mediators released * So get less nerve conduction, lots of white matter lesions

* See patchy destruction thruout CNS

Multiple Sclerosis
* Onset at age 20-40- more in women, cold climates * 20X more common in relatives-

* Exacerbations with stress, injury, illness, pregnancy, sleep problems * So if pt has periodic neuro disability with later recovery need to think MS * 85% are relapsing remitting kind but in 10 yrs half will get secondary progressive

Cost of MS
* Duration is about 30 yrs

and age on onset is 32

* 30% have a severe

disability- 70% are unemployed

* Care costs about $34,000

yr on top of cost of ABC meds

MS DX
* Vague, odd- skilled now with MRI * Look at plaques, evoked potentials and CSF * Plaque- in brain, cord * Evoked potentials- look at vision- oft see as an optic neuritis
* Check out pons and sensory tracts

* CSF with elevated IgG

* Symptoms usually begin in one area but then go everywhere

MS diagnosis
* How it presents depends on location of plaques wh are found on MRI * Usually begin with sx in one area & then they move around * Evoked potentials will measure the travel of electricity in CNS
* Visual evoked potential- in optic nerves, chiasm- see as optic neuritis * Auditory evoked potentials are in pons * Somatosensory are in sensory paths of brain & cord

MS
* No single test to accurately
determine DX

* Now have revised diagnostic criteria * 1. Neuro disturbance like that seen
in MS

* 2. Subjective report or observed * 3. Duration of incident 24+ hrs * 4. 30 days between event 1 & 2

MS sx
* Dx is made by the

symptoms

* How they change over time * Lesions in brain or cord

Types of MS
* Relapsing-remitting RRMS
* This has exacerbations & remission with no progressionstarts as 85%> then 55% * Lasts 24hrs- 2 or more in a month

* Primary Progressive-PPMS
* Continual downhill worsens without rest * Slow stepwise decline over months- 10 %

* Secondary Progressive - SPMS

* Exacerbates & remits with slow progression * 50% of RRMS cross to this after 10 yrs- 30%

* Progressive Relapsing- PRMS

* Progressive with a few relapses 5%

Areas of MS involvement
* Corticospinal issues
* Hits dorsal columns in spinal

tract
* Pt presents with stiffness,

slowing, weakness, fatigue

* Have bowel, bladder

problems, hesitancy, urge

* Paresthesias, abnormal

pain, tic doloreux

* Problems with vibration,

position

Areas of MS involvement
* Cerebellar issues
* Spasticity in limbs with

ataxia
* Dysarthria, intention tremor,

nystagmus
*

This is called Charcots triad

* No coordination, balance

issues, vertigo

* Lehrmittes sign- shock like

sensations along spine with flexion of head, hyperreflexia and a Babinski

Areas of MS involvement
* Cerebral issues
* Mood change- euphoria, depression, lability * Anxiety, depression * Early thinking problems, cant use speech, see well, problems with construction, calculation * So try MMSE * Impaired recall but good recognition

* Intelligence, language & memory intact * This creates employment issues

Areas of MS involvement
* Brainstem issues
* Visual issues- blind spots, scotomas * Optic neuritis, nystagmus * Paralysis of lateral gaze * Blurred vision and impaired color perception

Most common sx
* Fatigue- 88% * Walking issues- 87%

* Evac/void dysf- 66% * Pain- 60% * Visual issues 58% * Cognitive issues- 44% * Tremor 40%

Course of MS
* Will see oft exacerbations &

remissions
* Oft with temp elevations * Also issues with calcium

metabolism
* May get focal nerve attacks
* Shooting tingles, sudden

ataxia

* Feel like are losing mind

MS tx in acute exacerbation
* Sometimes are so sick need to come to hospital- oft before are Dx * Hi dose steroids- Solumedrol to reduce acute inflammation * 6-15 mg/kg/day- go from IV Solumedrol to oral prednisone
* Watch for side effects of mood change, GI bleed, elevated BS, blurred vision, frequent urination * Watch for impaired wound healing & infection

MS treatments

* Working on transplanting

oligodendrocytes that can produce new myelin

* Monoclonal antibodies to <

CD4 cells and suppress immune response

* IV immunoglobins
* Immune proteins to reduce

the inflammation

Immune ModulatorsInterferons
* The A, B Cs-- interferons that are body proteins made in response to foreign stimulus * Avonex

* Betaseron * Copaxone * Rebif * And now

* Novantrone * Antegren

Interferon B1bBetaseron
* This is for relapsing

remitting kind
* Given sub Q to reduced

new lesions
* Blocks myelin breakdown

* Inject under the skin every

other day

Interferon B1aAvonex
* Use daily & stop only for

SE
* Reduces relapses in

relapsing form
* Given once a week * Get generally flu-like side

effects

Interferon B1a- Rebif

* Higher dosing than Avonex

* Automatic injector

Copaxone- Glatimer acetate

* For relapsing forms- a non-

interferon, nonsteroidal
* Stimulates antigen specific

suppressor T cells
* Synthetic myelin basic

protein- reduces the destruction of myelin

* This is a daily injection

Novantronemitoxantrone

* Slows the progression of the neuro disability * Chemotherapy with a max lifetime dose * indicated for reducing neurologic disability and/or the frequency of clinical relapses in patients with secondary progressive, progressive relapsing and worsening relapsingremitting MS.

Ampyradalfampridine

* New med- to help walking

in people with MS that is used in combo with other meds

* Only thing is it can cause

seizures- improves speed

Tysabri
* Natalzumab * For relapsing forms of MSdecreases relapse flare ups * Best in those who cannot tolerate other meds * Not good with weak immune system- lots of allergies

* Weird > incidence of multifocal leukoencepahalopathy PML

Other treatments
* Antegren- etc
* In trials= an adhesion molecule blocker that slows the progression of brain lesions

* Other trials with hormonesestradiol and pregnancy is good for MS * Statins at a higher than cardiac dose * Remyelination -Yale is transplanting Schwann cells in brain or stem cells

IN the pipeline

* cladribine and fingolimod,

>better than existing treatments or placebo, c trials of of 3,800 patients.

* Fingolimod >daily vs

Cladribine is taken in short courses.

* damping the auto-immune

response through chronic inflammation.

* ? risk of side-effects

Other tx
* Symmetrel for fatigueenergizing antiviral

* Provigil for fatigue and wakening * Avitrol for weakness and eyes * Urinary- detrol, ditropan- late botox to muscle * Spasticity- baclofen, zanaflex, dantrium, valium * Tremor- inderal, buspar, mysoline * Pain- neurontin, lyrica * Cognition- aricept, namenda

Acute exacerbation
* Solumedrol- and decadron * Cytoxan

* Interferons * IVIG* plasmapheresis

MS nsg dxs
* Knowledge deficits * Chronic pain * Alt physical mobility, alt

safety
* Depression * The abandonment issues * Final long term care

Other MS interventions

* Now are living a near

normal life-span & many work full time

* Deal with constipation,

activity intolerance
* Alt physical activity

Parkinsons Diseases
* In 1% of population at age

55
* Then 3% by age 85 * Harry Truman,, Janet Reno,

PD PD

* Usually begins at age 55+ * Younger victims have a different form * Disability comes from tremor, rigidity, balance and coordination * Several types
* Postural instability & gait problems * Tremor as main problem * Oft begins asymmetrically- gradual progressive * Oft do not see tremor at first

Different types
* Genetics- a problem of chromosomes 2,4, 6
* May relate to pesticide exposure

* Or post encephalitis- as in awakenings * Some ASHD, toxin, trauma * Toxins are CO poisoning, cyanide, methanol, * Drug induced from depleting stores of dopamine as in some psych drugs
* Also some street drugs destroy substantia nigra paths

* The Parkinsonisms- drug induced, essential tremor, normal pressure hydrocephalus, supranuclear palsy, Shy Drager, Lewy Body

Parkinsons Pathophys

* Normal movmt needs dopamine- a chemical that smooths body movements

* The extrapyramidal system is responsible for movement, posture, balance, walking

* Degeneration of dopamine producing cells in substantia nigra so no transport to striatumso this is a nigrostriatal disorderstriatum is coord center for sev neurochemicals and now have more ACH than dopamine- so problems

PD

* Get symptoms when have 80% cell death * tremor also involves serotonin * 50% have trouble swallowing * Most have a mask like face with no blink or expression * Basal ganglia also lie near the hypothalamus so have problems like excess perspiration, orthostasis, gastric & urinary retention

comparison On PET

* Uptake is decreased in

posterior putamen and see hypermetablic striatum

* Lots of non motor

symptoms
* See spinal cord stuff from

pons
* Medulla- problems wth

autonimic NS
* Issues of smell difference-

may lose smell first as in alzheimers

Classic PD sx >TRAP
* Rest tremor

* Is is asymmetric, regular rhythmic, then get alt flexion & extension * Movement blocks the tremor when thalamus takes control

* Rigidity
* Increased resistance to passive movt from invol contract---resting muscles are contracting- oft cramp * Cogwheel or lead pipe (constant uniform resistance)

* Bradykinesia * Postural Instability & loss or reflexes

Classic PD sx >TRAP

* Rest tremor * Rigidity * Bradykinesia

* Cant initiate movt, increased reaction
time, slow to move

* Striated muscles feel wooden- sit still for

hrs- cant turn in bed

* Then get akinesia, dysarthria, drooling

(sialorrhea)

* Will freeze in place

* Postural Instability
* Get a festinating gait- slow & shufflingpropulsive and retropulsive

* Head is flexed forward * Poor balance, fall easily

More PD sx
* Excess androgens- greasy, seborrhea * Visual disturbances> blepharospasm
* Cant open eyes or keep them open * Little blinking

* Sensory numbness- tingling, akathisia, urinary issues * 20% become demented- most are depressed, apathetic, dependent
* Will have STM deficits, slow thoughts,

PD Depression

* See in 50% of patients

* Oft first are depressed and

then develop the tremor

* R/t lack of catecholamines * May also have an agitated

depression * May also have sleep

disturbances
* Sleep reversal- DFA, EMA

* http://www.youtube.com/wa

tch?v=ylHZWO17W70
* http://www.youtube.com/wa

tch?v=eKhOVaYYNo&feature=fvwrel

Not to confuse
* About 5% of pop have benign essential tremor- thats familial
* A symmetrical tremor that is fast and see best with arms outstretched

* Other causes of PD are drugsantipsychotics, Reglan- = Parkinsonian * A REST TREMOR disappears with voluntary movement- less severe when relaxed
* No tremor in sleep

* A few with hemiparkinsonismjust 1 side

Progression of PD

* Tell via PET scans see poor uptake in Putamen * Unilateral tremor & rigidity * Then bilateral tremor & rigidity * Then unsteady > fall * Then need a cane, walker * Then in wheelchair

Therapy for PD
* Mainstay is medications
* To alleviate signs &

symptoms
* Oft wont treat til sx are real

bad
* Most work on L-Dopa or

block cholinergic fibers

* Try now to give non ergot

dopamine agonists to control symptoms and allow L-Dopa delay

* L-dopa is still the gold

standard

L-Dopa
* A synthesized amino acid that can be ingested and transformed by brain proteins to make dopamine- but little taken in is converted so almost none get this pure coz dopamine cannot cross blood brain barrier * In early PD brain can convert LDopa but not so much later on

* So usually given as Sinemet which is combo of levodopa and carbidopa

Combo
* Levodopa is converted to

dopamine by enzyme DOPA decarboxylase in CNS & PNS to improve PD sx- but activation in periphery cause N&V- so given with carbidopa wh cannot cross blood brain barrie but prevents conversion in periphery so no side effects
* Plus makes more

dopamine avail to CNS

Sinemet
* This is Carbidopa- Levodopa * This is in #- the % of each as 25/100
* Smaller doses more frequently are helpful vs TID- can take up to 8 tabs/day * Carbidopa is left of slash- keeps from wasting the L-Dopa > also prevents N&V * This is more potent in small amts and helps control GI sx- inhibits gig enzymes * CR formulation can be halved, not crushed- lasts longer in blood so steady amt to brain

Sinemet
* Se> constipation, somnolence, vivid dreams, hallucinations as well as orthostasis * Also lots of dyskinesias, dystonias, fatigue, constipation, diarrhea, urine issues, orthostasis * CR formulation costs more * Never stop this abruptly
* Cost is $84/month

anticholinergics
* These work on the acetylcholine to keep balance

* Artane * Cogentin * Kemadrin * Benadryl

* These are for the tremor * Start with OTC benadryl in stress * These are also for antipsychotic tremor * Danger of confusion/hallucin in elders

Symmetrel
* This is amantadine- an antiviral for the flu but helps with movement issues- akinesia, rigidity

* Seems to block reuptake of dopamine and increases release

* Se- nausea, hypotension , hallucinations

* Works on NMDA receptor* Also is antiflu * Oft started as monotherapy- oft stop & start to optimize sx relief * 100-300 mg early in PD

Dopamine Agonists
* These act by stim of dopaminergic receptors so need no conversion- often start with these

* Many neurologists now start with these to hold off on need for Sinemet- these are advertised for restless legs, again ANV, halluc

* Permax * Parlodel * Mirapex * Requip

New patchRotigotine
* Non ergot dopamine

agonist- once a day patch covers 24 hrs- good with those who cannot swallowand nocturnal sx

* Start with CoQ too

Sleep in PD
* As disease advances- get

more sleep disturbancesand most dopaminergic drugs are sedation

* So should not drive * Or some are given provigil

Requip & Mirapex

* Requip-Newer med- dopamine receptor agonist for early tx & as adjunct to L-Dopa- also for restless legs
* 1.25 mg BID, adjust up to 20 mg BID

* Se are dizzy, sleepy, nausea, hallucinate * Cost $135/month

* Also in group is Mirapexpramipexole

* An agonist too- up to 1.5 mg TID, same SE as above- cost $107/month

Parlodel
* Older dopamine agonist-

1.25 mg BID
* Adjust up to 20 mg BID * Gives nausea, headache,

dizzy

* Side effects hard here but

less likely to get dyskinesias, and dystonias * So oft on these dopamine

agonists rather than L-Dopa

APOKYN injection
* (apomorphine

hydrochloride).02 mL .06 mL.02 mL during off periods

* SE Nausea, vomiting, low

blood pressure, sleepiness, dyskinesias, hallucinations, chest pain

* Adjunct levodopa therapy

to treat off periods- new

COMT drugs
* Comtam> these are catecholamine -methyltransferase inhibitorsthis is enzyme all over body & brain
* Help with motor fluctuations

* These drugs are used with Sinemet

* Extend the duration of L-Dopa but use it only with Sinemet to help use a lower dosage * Also use to help with wearing off phenomenon

* Tasmar- can enter brain

* Comtan- does not

Sinemet + entacapone
* Stavelo * NMDA receptor antagonists * This has entacaponein it

improves sx and quality of life, but still get side effects

Seligiline- Eldepryl
* This is a neuroprotector- to

slow progression at 5 AM and 1PM

* Is an MAO with no food

restriction

* Cannot take with Demerol so

d/c before surgery

* Also is a patch marketed as

EMSAM for treatment resistant depression

New Med- Azilectrasagiline

* A new option- MAO-B for

initial tx or for combo therapy along with Sinemet to block breakdown of dopamine- img
* But similar issues are

hallucinations

* Cannot take with SSRIs * Also avoid quinolones,

grapefruit juice

Co enzyme Q
* Use with statins- use with

parkinsons often first

Antipsychotics & new meds

* Antipsychotis Do not cause PD but are oft accused
* They may unmask or cause it to appear earlier

* New emerging agents in patch and other forms to treatrotigotine > new dopamine agonist- in patch- apply anywhere- once daily with contd drug delivery - good in swallow probs, nocturnal issues

Parkinson issues
* Occ have Parkinsonian

crisis
* Dose related motor

fluctuations
* Tremor, decreased dexterity,

vocal softness, loss of sleep refreshment, restlessness

* Oft see delayed gastric

emptying, r/t a protein meal or anticholinergics

* . Get exercise

On-Off Response
* This is a fluctuating responsewith sudden unpredictable shifts between under & over treatment * See abrupt change in muscle tone - from rigidity (off) to normal (on) then back to off again * See this after 5 years of treatment

* On- have L dopa response with good mobility and few symptoms * Off > no response with return of symptoms so are disruptive, depressed & anxious * May need Clozaril

Wearing Off Phenomenon

* Effectiveness of external L dopa regularly and predictably declines * Also called end of dose failure

* Needs to increase dosing intervals, get smaller doses, or give time release formulations * Also add COMT meds * May get sensory issues, psych, motor fluctuations at time of dose failures > paresthesias

Dyskinesias
* Dyskinesic movementschoreiform movements and myoclonus
* Repetitive movements of lower limbs * Oft see at time of peak dosing * Lower the dose or add dopamine agonists like symmetrel

* Dystonia by definition is a painful sustained spasm with posturing when L-dopa is low as in early AM * http://www.youtube.com/watch? v=sf1N0Zf5IqA

Freezing
* Stuck in place * Difficulty starting to walk* So fall * And with it is severe

burning pain, restless legs

* http://www.youtube.com/wa

tch? v=qHTFrUYFkCg&NR=1

Treating the Psych part

* For the depression will need an antidepressant- either stimulating or sedating depending on presentation * Hallucinations > from overdosing= stop the symmetrel, agonist or anticholinergic
* Reduce the dose of L dopa * Add zyprexa, clozaril or seroquel

* For sleep problems > give CR form, desyrel

* Add caffeine in daytime

Surgery in Parkinson's
* Autologous transplant of adrenal medulla * Fetal transplants * Thalamotomy for tremor * Pallidotomy for dyskinesias * Done under microscope with MRI guidance * Usu only do 1 side so fewer complications

Thalamotomy for Tremor

* Same gamma knife irrad

* Then post op create a

lesion
* Irradiate the VML thalamic

nucleus so neurons no longer transmit

Pallidotomy
* For dyskinesias, rigidity * For those with advanced

probs
* Destroy the nucleus * Stereotactic surgery under

MRI guidance
* Usu only one side

Fetal transplant

* Still not doing as well as

hoped

DBS- pacing the brain

* Electrically stim target parts

of brain- so deep brain stimulation to replace Thalamotomy

* To sub thalamic nucleus,

globus pallidus or VMI

Or other modalities
* In DBS wh substitutes for thalamotomy- electrode into thalamus to pace the tremor causing cells

* Also looking to molecular biology for gene therapy

* Idea to package and deliver dopamine cells straight to brain * Collagen into vocal cords to improve voice * Meds via patch and pills

Nursing interventions in PD
* Most will need psych support * Will also need hookup to know what is the active area of research

* Should not go to support group initially- wait til into the problem a while- and exercise strenuously * Ongoing evaluation of ADLsdressing, grooming, walking, writing, driving * Avoid any risk for fall stuff- like scatter rugs, too much furniture, hard faucets * Get a low bed with arm rests, grab bars, OT mod

Nursing interventions in PD
* Exercise for mobility> flexibility

* To prevent contractures, muscle weakness * Teach rocking and rhythmic relaxation so will move self.. * Teach balance and energy conservation * Need breathing and gait training > hamstring stretch * High stepped marching

* Speech therapy for the tongue protrusion, lip smacking, grimacing

* Get to practice swallowing, eating, chewing

Nsg in PD
* Fluid management- need at least 2000 cc

* Helps with constipation- also fiber, exercise * Hi cal, low protein diet coz protein blocks the effects of LDopa- or spread out the protein
* Avoid iron & large meals that also block dopamine also

* Allow time for completion * Take Sinemet 30 minutes before eating * Drink lots of water

PD & alternative therapies

* Massage & acupuncture

are good

* Swimming class * Coenzyme Q as med that

helps ? How

Restless Legs
* A common movement disorder accomp by sleep complaints with irresistible urge to move legs in rest at nite- with unpleasant sensations- worsen at rest- also
* achy,burning, creeping, currents, crawling, restless * Relieved by movt, stretch

* Causation, patho
* Hereditary

* iron deficiency so look at serum ferritin * Also r/t caffeine, nicotine, etoh, antidepressants * also disruption of dopamine ctrs in subcortex * Also hyperexcitable spinal reflexes

Restless legs
* More in women * First fix iron, movement, exercise * Treatment is dopamine agonists- usu not ergot as first line-

* Ropinirole- Requip- 0.25 mg daily FDA * Also Sinemet- esp if intermittent but may not work as well 25/100 * Gabapentin- for less intense or if neuropathy * Darvocet, codeine, or non benzo sleepers

Movt videos
* Webclips * http://medweb.bham.ac.uk/

HTTP/depts/clin_neuro/teac hing/tutorials/parkinsons/par kinsons2.html

Myasthenia Gravis
* Chronic progressive autoimmune disease * Problem at neuromuscular junction- with a problem in Ach receptors
* Loss of acetylcholine in post synaptic neurons related to auto antibodies * See close association with lupus & RA

* Problem of voluntary, striated muscles * Can be precipitated by mycins, Beta Blockers

The problem

MORE MYASTHENIA
* Young women >men after 50 * See fluctuating weakness & fatigue
* Initial presentation is fatigue * Worsens with exercise, improves with rest
* Danger when attacks chest & diaphragm

* Then lose cough, ability to take deep breath

* Also eye symptoms--ptosis, diplopia

* 1st presentation in 40%, total of 85% have eye sx

Myasthenia Sx

* Fatigue * Eye issues > ptosis, double vision * Motor problems> tone, dysphagia, facial droop, swallow problems
* Develop a snarling face- cant smile

* Proximal muscle weak

Patho of MG
* Normal neuromuscular junction
releases acetylcholine from motor nerve terminal in packages- like boats going across the gap * These float across the synapse and bind
to receptors on motor end plate

* Stimulation of motor nerve releases

these packages or boats filled with Ach to float across and depolarize the opp nerve and cause a contraction

* the synaptic membrane on shore is

folded so * are reduced receptors * fewer receptors * Also antibodies on membrane * Also enzyme kills the ships in transport

Dx of MG
* Initial view of symptoms * Then will do Tensilon test

IV or do IM Neostigmine test

* With Tensilon, pt will have

immediate return of strength, This is always done with ptosis

* Also now look at hi serum

levels of AchR antibodies > this shows the immune problem

Dx of MG
* Also will do CT of thymus-

15% have thymus tumors or tissue change but still do not know why

* Disease characterized by

exacerbations & remissions with fluctuating severity

Meds in MG
* Give Anticholinesterase meds wh retard the breakdown of acetylcholine at synapse
* Good for some, not others * Will inhibit the enzyme

* Mestinon and Prostigmin

* Dosage will vary with patient, level of stress, heat * Must give on time every 4-6 hrs to keep level even * So if pt is off floor, send with patient

* Has muscarinic side effectsdiarrhea, cramps * Cannot crush

Meds in MG
* Also decrease the

inflammation with
* Prednisone
* Hi doses til see improvement

then taper off

* What are side effects

* Also give IV

immunoglobins- IVIG
* Immunosuppressants
* Cyclosporine, azathioprine

Nursing Care MG
* Import of cough and

swallow- check and encourage

* Time activity to peak of

meds
* Wear a medi alert bracelet * Do plasmapheresis- to

remove antibodies- protect the access site

* Watch lytes

Thymectomy for MG
* Do a median sternotomy

thymectomy

* Will be best about 3-5 yrs

after surgery

Myasthenic crisis
* Sudden worsening usually precipitated by intercurrent infection
* Also a function of undermedication * So might increase the drug doses * See HTN, respiratory distress, * Pt will have no cough or swallow * Will be incontinent * May need to be intubated

* So Treat the infection, give more meds

Cholinergic Crisis-MG
* Here the patient is overmedicated * May look like myasthenic crisis> but is opposite * Here pt has abdominal cramps, diarrhea, excess pulmonary symptoms * Anxiety, sweating, fasciculations, blurred vision * Respiratory compromise is worse * Pt needs to be intubated and then meds wear off

Sometimes plasmapheresis
* blood is separated into cells

and plasma; plasma is removed and replaced with fresh frozen plasma, and this also removes the antibodies that cause the weakness- 5 exchanges helps for about 2 months

ALS- amyotropic lateral sclerosis

* This is also known as Lou Gehrigs disease * Most common motor neuron disease * A disease of middle aged men* Also in Stephen Hawking * Pt will survive about 2-10 yrs and dies of resp infection * 5000 diagnosed each year but no clue as to whom

ALS
* This is a disease of motor neurons to voluntary muscles from degeneration of anterior horns and corticospinal tracts

* Upper motor neuron begins in brain and goes to corddegeneration in upper motor neuron leads to spasticity and overactive reflexes
* Other UMN probs are primary lateral sclerosis, pseudobulbar palsy

ALS sx ALS
Disease of motor neurons to voluntary muscles from degeneration of anterior horns and corticospinal tracts

* Lower motor neuron begins in cord and synapse with UMNcommunication between two is via glutamate- and then to the muscles * Degeneration here > severe weakness, paralysis * ALS is both UMN and LMN * So an issue of metabolism, transport and glutamate storage

ALS
* 90% have no hereditary pattern * Usually begins with clumsiness,
fatigue, spasticity, hyperreflexia, tripping, muscle twinges

* Then get problems of swallowing,

chewing, speaking, or movement * So have dysarthria, dysphagia, or
drooling

* *

So with upper & lower motor neuron issues get weakness of all voluntary muscles Eye muscles, anus, and bladder are usually spared

So no incontinence and still function sexually

ALS
* Diagnose by clinical presentation and EEGs * Only pain is pain of immobility

* Mental status is fine- but will ultimately dies of respiratory failure * May have exaggerated laugh, cry, yawn
* Oft have labile emotions

* This is relentlessly progressive and will die by pneumonia coz of weak resp muscles

ALS
* No issues of cognition or

intelligence
* So are fully aware of every

problem, loss

* Will use positioning, splints,

breathing exercise
* Anticholinergics and

antihistamines for the secretions

Meds- ALS
* Riluzole-Reluten
* Prolongs survival * Anti glutamate- 50 mg BID will cost $600/month * No cure but slows degeneration and protects what is left

* Neurontin

* Anticonvulsant to reduce levels of glutamate at synapse between UMN and LMN

TX ALS
* Now trialing nerve growth

factors
* Collateral reinervation to

reach widowed muscle fibers and preserve neuron cell bodies

* IGF, GNDF, BDNF * Using brain derived

neurotrophic factors
* Myotrophin slows muscle

degeneration

ALS

* Live in hope and focus on what still can do * Set reasonable, attainable goals * Support the family and patient * Give fluids, position, small frequent high nutrient feeds * Will give muscle relaxers, like valium, Baclofen for the spasticity
* So will do ROM, keep upright, exercise, avoid fatigue

ALS
* Will do incentive spirometry * Give pulmonary care

* Work with speech * Stay on vent to help with

resp paralysis
* 50% will die in 2-5 yrs
* 25% will last 5 yrs * Some will live up to 20 yrs

Guillain Barre Syndrome

* Acute inflammatory demyelinating polyneuropathywhere lymphocytes and macrophages eat the myelin sheath or peripheral or cranial nerves- so have demyelination and axon degeneration

* Progressive polyneuropathy * Seems to be autoimmune of ? Origin

* Degeneration in particular in peripheral nerves * Major cause of generalized paralysis since polio

* Had URI several weeks before

* Seeming like to Epstein Barr, HIV, campylobacter

Guillain Barre
* CSF with increase protein * Nerve conduction velocity slowed- with sensory and motor loss * Presents with paralysis

* Most common type is ascending paralysis but are other variants * Descending will have rapid respiratory involvement

* Worst at 4 weeks p infectionusually symmetrically weakcant get up * DTRs gone, see paresthesias, muscle pain, weakness and neuropathies

GB GB
* Biggest danger is resp muscle paralysis * 25% will have total paralysis

* Remyelinization may take years to be complete * So assessment is

* Weakness, numbness * Pain in 50%- oft severe with movt- shoulder, thighs * Look for the autonomic nervous involvement with cardiac issues, diaphoresis, loss of sphincters, ileus * Can be life threatening

Guillain Barre
* Major issue is dysautonomia
* Arrythmias, malignant hypertension, hypotension and temperature irregularities

* May need respiratory and cardiovascular support * Will mostly recover at 6 mos > 2yrs
* 50% will fully recover * 35% will have residual deficits * 15% will have handicaps

Guillain Barre
* Diagnostic tests to rule out

other disorders
* LP & CSF will show

elevated serum proteins

* EMG with slowed nerve

conduction

GB treatment
* Respiratory support- even ventilator * IV immunoglobins- IVg X 5 days * Stool softeners * Use neurontin, tegretol for pain * plasmapheresis, * Care is supportive* Will need rehab > PT, OT exercise, lovenox * emotional devastation- 85% recover

Nsg DX GB

* Ineffective breathing

pattern r/t neuromusc weakness

* Acute pain * Alt tissue perfusion r/t the

dysautonomia
* Knowledge deficit

Trigeminal Neuralgia
* Also called tic doloreux * Pain along the 5th cranial nerve- the trigeminal that has 3 branches * in any of the 3 branches * 1/25,000 - more often in women

* and usually in middle age

* Intrinsic lesions within nervepresents like MS * Also extrinsic lesions from compression stretch, dental abscesses

Tic Doloreux
* *
*Severe pain similar to an electric shock! in the "ace# mouth# or teeth o" the a""ected side. * Heightened sensitivit$ around the mouth and nose# which# when touched# ma$ trigger an attack. %ther things such as e&posure to cold# eating or drinking# particularl$ cold li'uids or "ood# brushing the hair or teeth# or washing the "ace ma$ also trigger attacks. * (nvoluntar$ contortions also known as tic douloureu&! o" the "ace in response to the pain.

* )outs o" pain "or several weeks or months in a row# "ollowed b$ a spontaneous cessation o" s$mptoms. Periods o" remission ma$ last "or da$s or $ears# but the$ tend to become shorter as the patient ages.

Trigeminal Neuralgia
* Daily or intermittent paroxysms of excruciating pain-usu in women * Learn what causes-> chew, talk etc- & avoid * Oft recurs at intervals- feel fear or return & depression

* Oft from chronic compressionpulsating vessel, wears away nerves protection- or MS * Get MRI to look for cause

Interventions Tic Doloreux

* Want to quiet down the nerves * Tegretol- watch CBC, liver * Dilantin * Lioresal * tricyclics * Surgeries like nerve blocks, rhizotomies

* Nursing interventions are towards

* Pain * Starvation * tears

Tic Doloreux
* Surgery
* Microvascular decompression to keep vessel off of nerve

* Radioablation- probe thru cheek and ablate nerve * Balloon destruction of nerveballoon microcompression * Glycerol injection into nerve to kill

* Stereotactic radiosurgery with gamma knife to ablate- but side effect may be numbness

Intervents Tic
*
(n *amma +ni"e ,adiosurger$# a single highl$ concentrated dose o" ioni-ing radiation is delivered to a small# precise target at the trigeminal nerve root. *amma +ni"e ,adiosurger$ is non.invasive and avoids man$ o" the risks and complications o" open surger$ and other treatments. Treatment results in long.term pain relie" in nearl$ /01 o" the patients. Some loss o" sensation numbness! occurs in 201 to 231

Bells Palsy
* This is at 7th cranial or

facial nerve
* Motor paralysis

* Unilateral presentation * Could be from herpes * This is generally pain free so * think are having a stroke * Tx is analgesia, steroids * Worry about corneal

protection

Bells Bells Palsy

* See presentation

Radiculopathies
* These are nerve problems of the axons that travel up and down the stem to the cord

* Are easily injured- particularly where spinal nerves come off cord * See compression, inflammation & trauma * Radiculopathy > disorder of spinal nerve root * Radiculitis * Trauma can tear, avulse & disrupt * See change in strength, tone and muscle bulk * Pain, paresthesias > also called causalgia

Radiculopathies
* These are nerve problems of the axons that travel up and down the stem to the cord

* This leads to need for pain medicine * Increasing doses of opiates * Addictions * Loss of work and function * May use tens units, neurontin, elavil but pain is oft incapacitating

Spinal surgery again

* Major changes now- minimally invasive techniques- small incisions
* Lumbar microdiscetomy is done outpatient * Metallic instruments to support unstable tissue as bone grows over 2 yrs

* Use different angles and lots of screws, plates, rods * Intraop fluoro helps to pinpoint area * Spinal stenosis now common in elderly> so oft use artificial wedge implants tween spinous processes to expand the canal- avoid decomp, lami

Osteobiology
* Now new products to enhance bony fusion tween vertebral bodies
* Using cadaver bone, demineralized bone matrix and bone marrow aspirates to fill in bony defects and make solid bony fusion

* Scaffold the defect while bone grows over 2 years * Osteoporosis can cause severe back pain and can use cements to help and regain height

Areas of radic pain

* Vary with pressure and

area