Neuro 5 degenerativeA. Leah Kelly EdD, APRN, BC

Degeneration* Can be in the CNS- as in

MS, Parkinson’s, 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 progression- starts 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 Charcot’s triad

* No coordination, balance issues, vertigo

* Lehrmitte’s 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, can’t 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 Modulators- Interferons* The A, B Cs-- interferons that

are body proteins made in response to foreign stimulus

* Avonex

* Betaseron

* Copaxone

* Rebif

* And now

* Novantrone

* Antegren

Interferon B1b- Betaseron* This is for relapsing

remitting kind

* Given sub Q to reduced new lesions

* Blocks myelin breakdown

* Inject under the skin every other day

Interferon B1a- Avonex* 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

Novantrone- mitoxantrone

* 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 relapsing-remitting MS.

Ampyra- dalfampridine

* 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 MS- decreases 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 hormones- estradiol 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 fatigue-

energizing 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

Parkinson’s Diseases* In 1% of population at age

55

* Then 3% by age 85

* Harry Truman,, Janet Reno,

PDPD

* 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

Parkinson’s 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 striatum- so this is a nigrostriatal disorder- striatum 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

comparisonOn 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

* Can’t initiate movt, increased reaction time, slow to move

* Striated muscles feel wooden- sit still for hrs- can’t turn in bed

* Then get akinesia, dysarthria, drooling (sialorrhea)

* Will freeze in place

* Postural Instability

* Get a festinating gait- slow & shuffling- propulsive and retropulsive

* Head is flexed forward

* Poor balance, fall easily

More PD sx* Excess androgens- greasy,

seborrhea

* Visual disturbances> blepharospasm

* Can’t 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/watch?v=eKhOVaY-YNo&feature=fvwrel

Not to confuse* About 5% of pop have benign

essential tremor- that’s familial

* A symmetrical tremor that is fast and see best with arms outstretched

* Other causes of PD are drugs- antipsychotics, Reglan- = Parkinsonian

* A REST TREMOR disappears with voluntary movement- less severe when relaxed

* No tremor in sleep

* A few with hemiparkinsonism- just 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 won’t 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 L-Dopa 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 patch- Rotigotine* Non ergot dopamine

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

* Start with CoQ too

Sleep in PD* As disease advances- get

more sleep disturbances- and 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 Mirapex-pramipexole

* 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 inhibitors- this 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- Azilect-rasagiline* 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

parkinson’s 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 treat- rotigotine > 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 response-

with 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 movements-

choreiform 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/watch?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 ADLs- dressing, 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 L-Dopa- 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/teaching/tutorials/parkinsons/parkinsons2.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- can’t 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 effects- diarrhea, 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 Gehrig’s 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 cord- degeneration in upper motor neuron leads to spasticity and overactive reflexes

* Other UMN probs are primary lateral sclerosis, pseudobulbar palsy

ALS sxALS Disease of motor neurons to

voluntary muscles from degeneration of anterior horns and corticospinal tracts

* Lower motor neuron begins in cord and synapse with UMN- communication 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 polyneuropathy- where 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 infection- usually symmetrically weak- can’t get up

* DTRs gone, see paresthesias, muscle pain, weakness and neuropathies

GBGB* 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 nerve- presents like MS

* Also extrinsic lesions from compression stretch, dental abscesses

Tic Doloreux

* *Severe pain (similar to an electric shock) in the face, mouth, or teeth of the affected side.

* * Heightened sensitivity around the mouth and nose, which, when touched, may trigger an attack. Other things such as exposure to cold, eating or drinking, particularly cold liquids or food, brushing the hair or teeth, or washing the face may also trigger attacks.

* * Involuntary contortions (also known as tic douloureux) of the face in response to the pain.

* * Bouts of pain for several weeks or months in a row, followed by a spontaneous cessation of symptoms. Periods of remission may last for days or years, but they 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 compression- pulsating 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 nerve- balloon microcompression

* Glycerol injection into nerve to kill

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

Intervents Tic* In Gamma Knife Radiosurgery, a single

highly concentrated dose of ionizing radiation is delivered to a small, precise target at the trigeminal nerve root. Gamma Knife Radiosurgery is non-invasive and avoids many of the risks and complications of open surgery and other treatments. Treatment results in long-term pain relief in nearly 90% of the patients. Some loss of sensation (numbness) occurs in 10% to 15%

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

BellsBells 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