MYASTHENIA GRAVIS

MYASTHENIA GRAVIS
By Terra Cunningham

DEFINITION
* “A disorder of neuromuscular function thought to be due to the presence of antibodies to acetylcholine receptors at the neuromuscular junction”3

DISCOVERED IN
* First described in 1672 by Thomas Willis and later described in 1890 by German physicians, Samuel Goldflam, Wilhelm Erb, and Friedrich Jolly.

SYMPTOMS
* Drooping of the eyelids
* Double vision
* Difficulty smiling, speaking, swallowing
* Difficulty raising the arms
* Difficulty walking
* Difficulty breathing if chest muscle are affected

CAUSE
* The cause is unknown
* “Antibodies act against the acetylcholine receptor making a nerve come in contact with the muscle. The nerve cell conveys its message to tell the muscle to contract. The antibodies interfere with the message and the muscle contracts less efficiently. Resulting in the weakness of the arms or legs or of the muscles of the head.”4

PREVALENCE
* Today there are an estimated 50,000 cases in the United States
* Myasthenia Gravis can be found in anyone, but it is “most common in females around the third decade of life”1

TREATMENT
* Symptomatic treatment: medications that enhance the function of the acetylcholine system at the neuromuscular junction.
* Medications include – Prostigmin, Mestinon, Mytelase, Tensilon
* Long range treatment is thymectomy.
* Thymectomy is the surgical removal of the thymus gland that lies behind the breastbone and overlies the heart.

Lifestyles
* A person with Myasthenia Gravis can lead a normal life if the medications are taken in the correct dosages and at the right time of the day.

References
* Collier’s Encyclopedia with Bibliography and Index, 1988, volume 17.,Macmillan
* Dictionary of Medical Syndromes, 3rd edition, Magalini
* Dorland's Illustrated Medical Dictionary, 26th edition, 1985, Saunders
* Encyclopedia Americana, 1993, volume 19., Grolier
* The Medical and Health Encyclopedia, volume 1., Southwestern Company

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A Potpourri of Neurological Disorders

A Potpourri of Neurological Disorders
By:Stephanie Rodriguez MS RN

Chronic Neurological Problems
Headaches
* Classified based on characteristics of the headache
* Functional vs. Organic type
* May have more than one type of headache
* History & neurologic exam diagnostic keys

Not always chronic…be careful
Pattern
Tension
Migraine
Cluster
Site
Bilateral, basilar, band-like
Unilateral, anterior
Unilateral, occular
Quality
Squeezing, constant
Throbbing
Severe
Pattern
Cycles, years
Periodic, years
Remitting, relapsing
Duration
Days, weeks, months
Hours, days
30-90 min
Onset
Anytime
Prodrome, starts in AM
Nocturnal
Assoc. Sx
Stiff neck
N&V, photo/phono-phobia
Horner syndrome
ONSET: Not reliable or diagnostic

Horner’s Syndrome
HA: Essential History
* Onset this particular headache
* Character of pain, severity and duration
* Associated symptoms
* Prior history, pattern
* Original onset: prior testing, treatment
* Exertional aspects
* Other therapeutic regimens

Physical Exam
* Neurologic examination
* Inspect for local infections, nuchal rigidity
* Palpation for tenderness, bony swellings
* Auscultation for bruits over major arteries

Organic vs. Traumatic vs. Functional: Diagnostics
* CBC: underlying illness, anemia
* Chem panel: if associated vomiting, dehydrated
* U/A
* CT scan: for focal neurological signs, sinuses
* SED rate: if recent onset, > 50 yrs (temporal arteritis)
* No LP for suspected ICP; ↑ association with brain herniation (question from last time: ARTICLE

Don’t Miss It
* Caused by subarachnoid hemorrhage from an aneurysm or head injury
* “Worse headache of my life”
* Changes in LOC, focal neurological signs
* Highly correlated with CVA
* Untreated, 50 % mortality

Headache Teaching Guide
* Keep a calendar/diary
* Avoid triggers
* Medications (purpose, side effects)
o Imitrex: take med at 1st sign of HA, ↓blood flow to brain
* Stress reduction
o Dark quiet room, exercise, relaxation
* Regular exercise
* When to contact PCP

Multiple Sclerosis
Definition
MS is a chronic, progressive, degenerative disorder of the CNS characterized by disseminated demyelination of the nerve fibers of the brain and spinal cord.

Multiple Sclerosis
Etiology
* Cause is unknown.
o Possibly related to viral, immunologic, and genetic factors.
* Susceptibility appears to be inherited with 1st, 2nd, and 3rd degree relatives at slight increased risk.

Pathophysiology
* MS is characterized by chronic inflammation, demyelination, and gliosis in the CNS.
* Autoimmune process is orchestrated by autoreactive T cells (lymphocytes)
* Activated T cells migrate to CNS causing blood-brain barrier disruption.
* Subsequent antigen-antibody reaction within the CNS activates the inflammatory response leading to demyelination of axons, with plaque formation.

A: Normal Nerve Cell
B: Normal Axon
C: Myelin Breakdown
D:Myelin completely disrupted

U-tube: animation
* http://www.youtube.com/watch?v=eE_Y3KMYC0g

Disease Process: Early
* Myelin sheaths of neurons in the brain and spinal cord are initially attacked.
* Myelin sheath is damaged but nerve fiber still intact.
* Nerve impulses are still transmitted but patient may complain of weakness.
* Remission occurs if inflammatory process stops and myelin regenerates.

Disease Process: Late
* If remission does not occur, myelin disruption continues and axon is affected.
* Myelin is replaced by glial scar tissue which forms plaques in the CNS.
* Lacking myelin, nerve impulses slow, axons are destroyed, resulting in permanent loss of function.`

Symptoms
* Initially insidious with symptoms evolving over months to years.
* Systems affected depend on distribution of lesions.
* Disease process may be chronic and progressive or have periods of remission and exacerbation.
* Even in relapsing disease, overall trend is progressive deterioration of neurologic function.

Symptoms: Motor
* Weakness
* Paralysis of limbs, trunk or head.
* Diplopia
* Scanning speech
* Muscle spasticity

Symptoms: Sensory
* Numbness, tingling, parathesia.
* Scotomas (patchy blindness)
* Blurred vision
* Vertigo
* Tinnitus, decreased hearing
* Neuropathic pain
* Radicular pain

Symptoms: Cerebellar
* Nystagmus
* Ataxia
* Dysarthia
* Dysphagia

Symptoms: Emotional
* Emotional stability may be affected
* Depression common co morbidity
* Range of emotions include anger to euphoria.
* Cognitive function usually not affected.
* Emotional lability may negatively affect progress of disease.

Symptoms: Bowel & Bladder
* Symptoms depend on where sclerotic plaque is located.
* Spastic bladder indicates lesion above S2 resulting in small capacity. This yields frequency, urgency, dribbling or incontinence.
* Flaccid bladder results form disruption of reflex arc resulting in loss of sensation or desire to void.
o Crede Method
* Bowel symptoms are usually constipation.

Multiple Sclerosis Drug Therapy
Agent
Corticosteroid (ACTH, Prednisone)
Immunomodulators (B-interferon,Betaseron, Copaxone)
Immunosuppresants (Novantrone)
Cholinergics (Urecholine, Prostigmin)
Anticholinergics (Pro-bathine, Ditropan)
Muscle Relaxants (Valium, baclofen, Zanaflex)

Target Symptom
Exacerbations
Urinary retention; flaccid bladder
Urinary freq. and urgency; spastic bladder
Spasticity

Nursing Care Planning
* Impaired physical mobility
* Self-care deficit
* Impaired skin integrity; risk/actual
* Impaired elimination; urinary, bowel
* Sexual dysfunction
* Interrupted family process

Myasthenia Gravis
Definition & Pathophysiology
* Autoimmune disease of the neuromuscular junction in which antibodies attack acetylcholine (ACh) receptors.
* ACh is prevented from attaching and stimulating muscle contraction
* 90% of patients have ocular manifestations (ocular myasthenia)
* Generalized myasthenia affecting trunk and limbs less common.
* 15% of patients have abnormal thymus glands.
* No other neural disorders accompany MG.
o no sensory loss, reflexes are normal, atrophy is rare.

Myasthenic Crisis
An acute exacerbation of muscle weakness
o triggered by infection, surgery, stress, overdose or or inadequate management of medications
o If muscle weakness affects swallowing and breathing: aspiration, respiratory insufficiency, and infection may result.

Myasthenia Gravis: Symptoms
* Weakness of skeletal muscle
o Muscles are strongest in morning
o Evening: muscle weakness is prominent
* Impaired facial mobility and expression
* Prolonged speech resulting in fading voice
* Possible difficulty in chewing, swallowing
* Proximal muscles of neck, shoulder and hip may be affected
* Course of the disease is variable It most commonly affects young adult women (under 40) and older men (over 60), but it can occur at any age.
* CRISIS: myasthenic crisis is weakness affecting the muscles that control breathing, creating a medical emergency and requiring a respirator for assisted ventilation
Diagnosis
* Diagnosis may be made by H&P.
* Blood tests:
o antibodies to ACh in 90%
* EMG:
o show decreasing response to repeated stimulation of hand muscles
* Tensilon test: shows improved contractility with IV injection of medication.
* NEURO ASSESSMENT: MG expected findings: impairment of eye movements or muscle weakness without any changes in the individual's ability to feel things

Treatment
* Drug therapy – anticholinesterase agents, corticosteroids, immunosuppressants
* Surgical therapy - Thymectomy
* Plasmapheresis
* Intravenous IgG
* Rest!

Huntington’s Disease
* Autosomal dominant genetic disorder
* Deficiency in the acetylcholine and GABA results in excess dopamine.
* Symptoms:
o Clinically opposite of Parkinson’s
o Chorea, intellectual decline, emotional lability
http://www.youtube.com/watch?v=UKbC1jRjs3M
http://www.youtube.com/watch?v=gBtji6Ibbso

Huntington’s Disease: Symptoms
* Abnormal & excessive involuntary movements (chorea)
* Writhing and twisting movements of face, limbs, body.
* Facial involvement affects speech, chewing, swallowing causing aspiration, malnutrition.
* Gait deteriorates with loss of mobility.
* Mental function declines including intellect, emotional lability and psychotic behavior.

Huntington’s Disease Diagnosis
* By symptoms
* By genetic testing as an adult: IMPLICATIONS??
* By prenatal screening

Treatment
* Palliation, symptomatic
* Neuroleptics
* Antidepressants
* Chorea antagonists (Klonopin)
* Fetal tissue transplant (experimental)

Parkinson’s Disease
Definition & Pathophysiology
* Disease of the basal ganglia
* Substantia Nigra:
o Degeneration of dopamine producing neurons
o Disrupts normal balance between dopamine and acetylcholine in the basal ganglia
* Dopamine is essential for normal function of extrapyramidal motor system which controls
o posture
o support
o voluntary motion

Dopaminergic Synaptic Activity
Good to know: These neurotransmitters and functions highly related to “reward” and to psych drug actions
Symptoms
* Onset:
o gradual, insidious, and prolonged
* Classic triad:
o tremor, rigidity and bradykinesia
* Initially:
o may involve one side of the body with mild tremor, slight limp and decreased arm swing
* With progression:
o classic shuffling and propulsive gait with flexed arms and loss of postural reflexes develops
o Slowed reflexes include decrease blinking, drooling, and masked facies

Parkinson’s Presentation

Parkinson’s Disease

A firm diagnosis of Parkinson’s is made only if at least two of the classic triad symptoms are present.

Diagnosis Confirmed: improvement of symptoms once anti-parkinsonian drugs initiated

Parkinson’s Disease: Treatment

Drug
* Dopaminergic (levodopa, sinemet, parlodel, etc.)
* Anticholinergic (artane, cogentin, symmetrel, etc.)
* Antihistamine * MAO inhibitor
* COMT inhibitor’s (comtan, tasmar)

Symptom relieved
* Bradykinesia, tremor, rigidity
* Tremor
* Tremor, rigidity
* Bradykinesia, tremor, rigidity
* Slows breakdown of levodopa

Parkinson’s Disease: Treatment Surgical
* Ablation
* Deep brain stimulation
* Fetal tissue transplant

Nutritional
* Hi fiber
* Mechanical soft
* Frequent small meals
* Lo-protein in HS

Nursing Care Planning
* Impaired physical mobility
o Teaching necessary
* Dressing/grooming self-care deficit
* Impaired skin integrity-risk/actual
* Impaired urinary elimination
* Sexual dysfunction
* Interrupted family process

Seizure Disorders & Epilepsy
Definitions
Seizure:
o paroxysmal, uncontrolled electrical discharge of neurons in the brain that interrupts normal function
Epilepsy:
o spontaneously recurring seizures caused by a chronic underlying condition

Question:
* The population with the highest prevalence of new-onset epilepsy is:

A: over the age of 60
B: children under 5
C: adolescents between 12-18 yrs
D: middle-aged men

Seizure Classification
http://www.youtube.com/watch?v=CDccChHrgRA

Seizure Disorders & Epilepsy: Classifying Seizures
Two major classes:
* Generalized
* Partial

Depending on type, phases may include:
* Prodromal phase- signs & activity preceeding seizure
* Aural phase- sensory warning
* Ictal phase- full seizure
* Postictal phase- recovery

Seizure Disorders & Epilepsy: Generalized
* Absence
* Myoclonic
* Clonic
* Tonic
* Tonic-Clonic
* Atonic

Seizure Disorders & Epilepsy: Partial Seizures
* Simple: no impairment of consciousness
o Symptoms: motor, somatosensory, autonomic, psychic
* Complex: impairment of consciousness
o Simple with progression to LOC/impairment
+ Symptoms: no other features, simple partial seizure features, automatisms
o Impairment of consciousness at onset
+ Symptoms: no other features, simple partial seizure features, automatisms

Seizure Disorders & Epilepsy: Status Epilepticus
* Medical emergency
* Seizure repeated continuously
o Tonic clonic: hypoxia could develop if muscle contraction is lengthened. Also: hypoglycemia, acidosis, hypothermia, brain damage, death
+ IV administration of antiepileptics
+ Maintain airway patency

Seizure Disorders & Epilepsy: Diagnostic Studies
* Most useful tool:
o a reliable and accurate description of the event, and the patient’s health history
* PE
* Electroencephalography (EEG):
o only useful when it shows abnormalities
* Labs:
o CBC, U/A, BMP
* PET scan, CT, MRI, MRA, MRS

Drug Therapy
* Can be helpful in preventing ongoing seizures following a head injury and should be first priority to decrease likelihood of second seizure

Seizure Disorders & Epilepsy Drug Therapy for Tonic-Clonic and Partial Seizures
* Carbamezepine/ Tegretol
* Divalproex/ Depakote
* Gabapentin/ Neurontin
* Lamotrigine/ Lamictal
* Levetiracetam/ Keppra
* Phenytoin/ Dilantin
* Tiagabine/ Gabitril
* Topiramate/ Topamax
* Valproic Acid/ Depakene
* Felbamate/ Felbatol *
* Phenobarbitol**

*Felbatol has been associated with aplastic anemia
**Phenobarbitol is a barbituate
Seizure Disorders & Epilepsy
Drug Therapy for:
Absence, Akinetic, & Myoclonic Seizure
* Clonazepam/ Klonopin
* Divalproex/ Depakote
* Valproic Acid/ Depakene
* Ethosuximide/ Zarontin
* Phenobarbitol

Seizure Disorders & Epilepsy Toxic Side Effects
* Diplopia
* Drowsiness
* Ataxia
* Mental slowing
assess for dose related toxicity including nystagmus, hand and gait coordination, cognitive function, general alertness
Seizure Disorders & Epilepsy
Idiopathic Side Effects
* Skin rash
* Gingival hyperplasia (dilantin)
* Bone marrow & blood dyscrasia
* Abnormal liver function
* Abnormal kidney function

Seizure Disorders & Epilepsy: Nursing Care

* Assure oxygen and suction equipment at bedside
o http://www.youtube.com/watch?v=H2vH1igOoh0
* Safety precautions in active stage
o Support/ protect head
o Turn to side
o Lossen constricted clothing
o Ease to floor
* Time seizure, record details of seizure and post-ictal phase
* Patient teaching:
o importance of good seizure control using medication as ordered
o Medical alert bracelet
o Avoid decreased sleep, increased EtOH, fatigue
o Regular meals/ snacks

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Myasthenia Gravis

Myasthenia Gravis

Chronic autoimmune disease of the neuromotor junction presents as muscle weakness and fatigue

Clinical manifestations
* Weakness
* Fatigue
* Ptosis
* Diplopia
* Facial muscles weakness
* Dysphagia
* Nasal quality to speech
* Respiratory distress
* Muscles involved – eyes, eyelids, chewing, swallowing
* Speech affected
* Muscles of the trunk and limbs less affected
* Proximal muscles of the neck, shoulder and hips are affected
* No sensory loss
* Reflexes normal
* Muscle atrophy rare
* Pt may have exacerbation and remission

Exacerbation of MG
* Emotional stress
* Pregnancy
* Menses
* Secondary illness
* Trauma
* Temperature extremes
* Hypokalemia
* Drugs – aminoglycosides antibiotics, beta blockers, procainamide, quinidine, phentoin, and some psychotropic drugs

Diagnostic
* History and physical
* Antibodies to ACH receptors
* Upward gaze
* EMG
* Tensilon test

Management
* Anticholinesterase drugs – mestinon, prostigmin
* Corticosteriods
* Immunosuppressant drugs – imuran, cytoxan
* Must check for other drug interactions antibiotic, antiarrhythmics, diuretics etc.
* Surgery – removal of thymus gland
* plasmapheresis

Nursing care
* Admin. anticholinesterase drugs
* Respiratory assessment – suction
* Elevate HOB when eating
* Check swallow reflex – oral motor strength
* Plan activities – muscles strongest in morning
* Assess muscle strength before and after activity

Myasthenic crisis
* Due to exacerbation of myasthenia or failure to take drug
* S/S – improved strength with anticholinesterase drugs, inc. weakness of skeletal muscles, ptosis, difficulty on swallowing, articulating words, dyspnea

Cholinergic crisis
* Due to overdose
* S/S – weakness within 1 hour of taking anticholinesterase drug, ptosis, dyspnea, blurred vision, salivations, diarrhea, N/V, abd. cramps, inc. bronchial secretions, sweating, lacrimation
* Due to overdose
* S/S – weakness within 1 hour of taking anticholinesterase drug, ptosis, dyspnea, blurred vision, salivations, diarrhea, N/V, abd. cramps, inc. bronchial secretions, sweating, lacrimation, difficulty swallowing, dyspnea

Nursing DX
Discharge teaching
* Instruct on disease process
* Importance of drug regime – sch drugs at peak action at mealtime, other drug interactions
* Suction equipment at home
* S/S of underdose and overdose of meds
* Instruct on precipitating factors
* Diet – semisolid food
* Rest, Plan activities
* Use of adaptive devices – OT, home care
* MG support group, Community resources

Amyotrophic Lateral Sclerosis
* Known as Lou Gehrig’s disease
* Cause unknown
* Motor neurons in the brainstem and spinal cord gradually degenerate
* Electrical and chemical messages originating in the brain do not reach the muscles to activate them
* Death within 2-6 years after diagnosis

ALS
* S/S – weakness of upper extremities
* Dysarthria
* Dysphagia
* Weakness may begin in legs
* Muscle wasting, fasciculations
* Sensory intact
* Death usually results from respiratory infection

Dx
* Difficult to dx- rule out other diseases
* EMG
* MRI
ALS
* Treatment – Riluzole to slow progression
* No cure
* Cognition is intact

Management
* Supportive therapy – OT, PT, RT
* Assess client’s ability to do ADL
* Conserve energy
* Encourage small freq meals
* Suction equipment
* Soft collar to stabilize head
* Adaptive equipment
* Allow time to complete activities
* Avoid exposure to anyone with respiratory infection
* Good posture and swallowing techniques
* Diaphragmatic breathing
* Follow up pulmonary tests
* Home care
* ALS support group

Multiple Sclerosis
* A chronic progressive degenerative disease that affects the myelin sheath of neurons in the CNS
* Cause unknown – genetic, virus, autoimmune response, inherited, antigen-antibody reaction

Clinical Course
* Relapsing – remitting: relapses with full recovery and residual deficit with recovery
* Primary – progressive: dx progression from onset with occ plateaus and temp. minor improvements
* Secondary – progressive: relapsing-remitting course followed by progression with or without relapses, minor remission and plateaus
* Progressive – relapsing: Progressive dx, with acute relapses with or without full recovery, periods between relapses cont. progression

Dx
* History and Physical
* CSF analysis
* CT scan
* MRI

Clinical manifestations
* S/S may vary
* Motor- weakness or paralysis,of the limbs, trunk, or head, speech problems, spasticity of the muscles
* Sensory – numbness, tingling, paresthesia, visual changes, vertigo, tinnitus, decrease hearing, chronic neuropathic pain, radicular pain (pain in thoracic area and abdominal region), lhermitte’s phenomenon – electric shock radiating down the spine, into the limbs with the flexion of the neck

Clinical manifestations
* Cerebellar signs – nystagmus, ataxia, dysarthria, dysphagia
* B/B function can be affected
* Constipation a problem
* Spastic bladder – incont.
* Flaccid bladder – no sensation of voiding
* Mood swings, Intellect intact
* S/S may be triggered by physical, emotional trauma, fatigue and infection

Medical Management
* Corticosteriods
* Immunomodulators – B-interferon
* Immunosuppressants
* Cholinergics - flaccid bladder
* Anticholinergic – spastic bladder
* Muscle relaxants
* Surgery – control tremors

Nursing Dx
Guillain Barre
* An acute form of polyneuritis
* Etiology unknown
* A cell mediated immunologic reaction directed at the peripheral nerves
* Involves degeneration of the myelin sheath of the peripheral nerves
* In half of cases, an upper respiratory or GI infection precedes the onset of the syndrome by 1-4 weeks
* Antecedent illness-cytomegalovirus, Epstein Barr virus, mycoplasma pneumonia, salmonella typhosa, campylobacter jejuni, HIV
* A chronic form of GB paralysis evolves more slowly with no involvement of respiratory of cranial nerves
* With support, pt will recover

DX
* History and physical exam
* Electrophysiological studies
* Cerebrospinal fluid with elevated protein levels
* EMG

Characteristics of GB
* Ascending weakness usually beginning in the lower extremities and spreading to trunk, upper extremities and face
* Improvement and recovery occur with remyelination; if nerve axons are damaged
* Some residual deficit may remain
* Recovery is usually 6 months with 85%-90% of clients recovering completely
* 10% have recurrence and 20% have long term disabilities/emotional trauma

Guillian Barre
* Complication- is respiratory failure
* Impt to monitor respiratory rate, depth, vital capacity
* Client may be intubated with mechanical ventilation
* Complications can occur due to immobility

Clinical Manifestations
* Flaccid quadraplegia
* Facial weakness, dysphagia, diplopia, hypotonia
* Autonomic dysfunction found in severe muscle involvement and respiratory muscle paralysis – orthostatic hypotension, hypertension, pupillary disturbances, sweating dysfunction, bradycardia, paralytic ileus, urinary retention
* Weakness
* Paresthesia of the limbs
* Loss of deep tendon reflexes
* Deep, aching muscle pain in shoulder and thighs
* Respiratory compromise or failure-dyspnea, dec. breath sounds, dec. tidal volume (air in & out)

Medical/Nursing Management
* Supportive care
* Immunoglobulin therapy
* Pain control worse at night due to paresthesia, muscle aches and cramps
* Problems - airway, aspiration, communication problems, orthostatic hypotension, nutritional intake
* Plasmaphoresis
* ABGs
* Assist ability to perform self care
* Set communication system
* Work closely with PT, OT
* Monitor for complications of immobility
* Safety measures provided

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