neuromuscular and neurological

multiple sclerosis (MS)

(Myelin Sheath, Multiple Scars, Muscle Spasms)

chronic, progressive degenerative disorder of the CNS

characterized by disseminated demyelination of nerve fibers in the brain and spinal cord

onset at 20-40 years of age, usually women

can be genetic and triggered by a viral infection

marked by chronic inflammation, demyelination, and gliosis

manifestations of multiple sclerosis (MS)

• vision changes: blurred, nystagmus, color distortion, double vision

• extremity muscle weakness

• problems with balance and coordination

• numbness and tingling

• Lhermitte’s sign - shock down the spine when you flex the neck

• pain and spasms in lower back and abdomen

  • “____ hug”

• speech problems

• constipation and spastic bladder = urgency and frequency

• ED and decreased libido

• extreme fatigue

relapsing-remitting vs. progressive-relapsing patterns of multiple sclerosis (MS)

clearly defined attacks of worsening neurologic function (relapses) with partial or complete recovery (remission)

vs.

Progressive disease from onset with clear, acute relapses with or without full recovery. Periods between relapses are characterized by continuing progression

primary-progressive vs. secondary-progressive patterns of multiple sclerosis (MS)

Steadily, worsening neurologic function from the beginning with minor improvements, but no distinct relapses or remissions

vs.

A relapsing-remitting initial course, followed by progression with or without occasional relapses, minor remissions, and plateaus

diagnosis of multiple sclerosis (MS)

• CSF analysis

  • check for increased IgG = inflammation

• MRI with contrast of brain and spinal cord

  • shows plaques, atrophy, tissue destruction

• must have:

  • evidence of 2 or more inflammatory demyelinating lesions in 2 different locations in the CNS

  • 2 or more attacks occurring 1+ month apart

  • all other diagnoses ruled out

what can multiple sclerosis (MS) mimic?

disc problems or stroke

how can a patient manage their multiple sclerosis (MS) symptoms?

• exercise

  • especially water exercises

  • stretching

  • do not overdue it, balance with rest

• drug therapy (corticosteroids)

• PT/OT/speech therapy (helps with swallowing)

• support groups to help cope

• injury prevention

• bladder control (KEGELS)

• avoid triggers (4Ss: sun, stress, sickness, smoking)

(mellow and stay cool)

myasthenia gravis (dryasthenia gravity)

an autoimmune disease of the neuromuscular junction marked by fluctuating weakness of certain skeletal muscles

antibodies attack and destroy the ACh receptors

first sign of myasthenia gravis

ptosis (eye drooping) and double vision

which muscles are affected the most by myasthenia gravis?

muscles engaged in repetitive movements like the eyes, mouth, and respiratory muscles

results in difficulty chewing, swallowing, speaking, seeing, and breathing sometimes

symptoms move with gravity, start at the top and moves down

myasthenic crisis

acute worsening of muscle weakness triggered by respiratory infection, surgery, pregnancy, stress, or certain drugs (4Ss: stress, sun, sickness/sepsis, smoking)

can result in aspiration and respiratory insufficiency → BiPAP or ventilator

electromyography (EMG)

used to diagnose myasthenia gravis

shows decreased response to repeated stimulation of the hand muscles that shows muscle fatigue

edrophonium test

used to diagnose myasthenia gravis

an antiACh agent is given IV → prevents breakdown of ACh → should rapidly improve muscle strength of someone with MG

can diagnosis of myasthenia gravis be done based on H&P?

yes; other diagnostic tests like the EMG and edrophonium test are used for confirmation

“peek” sign

during sustained forced eyelid closure, patient with myasthenia gravis is unable to keeps lids fully closed

manifestation of myasthenia gravis

• muscle weakness later in the day

  • strong muscles in the morning

• decrease in potassium

• weakness that increases with activity

• drooping of the eye

• dysphagia

• dyspnea

• respiratory distress if severe due to weakened diaphragm

drug therapy for myasthenia gravis

• anticholinesterase

  • enhances transmission at neuromuscular junction

• corticosteroids

  • suppresses immune response

• immunosuppressants

what surgeries and therapies can someone with myasthenia gravis get?

• thymectomy

• IV IgG before surgeries or crisis

• Plasmapheresis

  • removes circulating ACh receptor antibodies, filters plasma, and returns it to patient

nursing interventions and education in a myasthenic crisis

• plan activities to be early in the day

• time medication to hit its peak during mealtime

• give semisolid foods NOT thin liquids

• plan activities that match the patient’s interest

• intubation at bedside

Guillian Barre Syndrome (tip: ground up barre)

an autoimmune disorder affecting the PNS; causes injury to the myelin sheath and nerve axons → edema and inflammation → slows down/stops transmission of nerve impulses

occurs a few days or weeks after viral or bacterial infection of GI or respiratory

ex. CMV, Hep. A,B, or E, Zika, Hib, Epstein Barr

can be associated with vaccines

manifestations of Guillian-Barre

• acute, ascending, rapid weakness of the limbs

  • can progress to paralysis

  • bilateral

• paresthesia

• hypotonia (reduced muscle tone)

• orthostatic hypotension

• bradycardia

• bowel and bladder dysfunction

• flushing and diaphoresis

• facial weakness

• decreased/absent DTR

• pain

  • worse at night

  • decreased appetite and interferes with sleep

• severe: respiratory failure - kills them

  • inability to cough or lift head is early sign

how is Guillian-Barre diagnosed?

mainly H&P - has progressive weakness of more than 1 limb and decreased/absent reflexes

can also use EMG, CSF, and nerve conduction studies to rule out other causes

what can be a serious complication of Guillian-Barre and what are our nursing interventions?

respiratory failure

assess respiration rate and depth → if it drops and becomes shallow → ventilation or intubation

care for Guillian-Barre

• ventilatory support in acute phase

• plasmapheresis or IV IgG

  • most effective first 2 weeks of symptom onset

• evaluate sensory and motor function (gag reflex, paralysis, reflexes, cornea)

• monitor ABGs, BP, and cardiac rhythm

  • could have dysrhythmias, orthostatic hypotension, etc.

• note drooling = could have impaired gag reflex

  • may need enteral feeding

• refer to OT/PT/ speech

• may have incontinence

The nurse is teaching a female client with multiple sclerosis. When teaching the client how to reduce fatigue, the nurse should tell the client to:

a. take a hot bath

b. rest in an air-conditioned room

c. increase the dose of muscle relaxants

d. avoid naps during the day

rest in an air-conditioned room

can someone with myasthenia gravis build up tolerance by exercising?

no

causes of dementia

• neurodegenerative disorders ex. Down Syndrome, Parkinsons, ALS

• vascular diseases

• infections ex. AIDS, meningitis

• immunologic ex. lupus, MS

• medications ex. cocaine, heroin

• alcohol use

• hyper and hypothyroidism

• head injury

• brain tumors

some of these may be reversible

dementia

disorder characterized by a decline from a previous level of function in 1 or more cognitive domains:

• complex

• attention

• executive function

• language

• learning and memory

• perceptual motor

• social cognition

NOT a normal part of aging

what is the most common form of dementia?

Alzheimer’s disease

risk factors for Alzheimer’s disease (AD)

• #1 is age (65 years+)

• family history of dementia

• smoking, diabetes, HTN, obesity, high cholesterol increase risk of CVD → less blood supply to brain

• head trauma ex. football players and military

early warning signs of Alzheimer’s disease (AD)

1. memory loss that affects job skills

2. problems with abstract thought (can’t recognize numbers, or do calculations)

3. difficulty doing familiar tasks ex. cook

4. poor or decreased judgement (wear bathrobe to a store)

5. problems with language

6. misplacing things

7. changes in mood

8. changes in personality (easygoing → aggressive)

9. loss of interest

diagnostic tests for Alzheimer’s disease (AD)

• H&P

  • psych eval,

  • Neuropsychologic testing: Mini-Cog (draw a clock or repeat 3 words), Mini-Mental State eval

• CT, MRI, PET of brain

• CBC, LFTs, thyroid function tests

scoring in Mini Cog test

give 1 point to each word recalled after the clock drawing test

if they got 1-2 words after the clock drawing test, use the clock test to determine score (score 2 if drawn correctly, 0 if not

0-2 is positive screen for dementia

3-5 is negative screen for dementia

sundowning and its symptoms

when a patient becomes more confused and agitated in the late afternoon or evening

can be due to a disruption in the circadian rhythm

• agitation

• aggressiveness

• wandering

• resistance to redirection

• increased verbal activity like yelling

can be due to pain, hunger, environment, noise, medications, reduced lighting, or fragmented sleep

how to manage someone who is sundowning?

• create a quiet, calm environment

• maximize exposure to daylight

• limit naps and caffeine

• avoid confrontation

• consult with HCP about drug therapy

education necessary for a caregiver of someone with Alzheimer’s disease (AD)

• establish a routine

• safety: no throw rugs, install handrails, door locks

• label drawers and faucets

• encourage family and visitors

• provide protective wear for incontinence

delirium

state of confusion that develops over days to hours due to drugs, hypoxia, excess dopamine or ACh deficiency

patient has decreased ability to direct, focus, sustain, and shift attention and awareness

can have deficits in memory, orientation, language, and perception

can have emotional problems like fear, euphoria, depression

tends to fluctuate throughout the day

risk factors for delirium

• 65 years + and male

• immobility

• Hx of dementia, depression, cognitive impairment

• dehydration or malnutrition

• alcohol or drug use

• sleep deprivation

• stress

• sepsis

• electrolyte imbalance or CKD

• Hx of stroke

• sensory overload

• ortho or cardiac surgery

mnemonic for causes of delirium

Dementia, dehydration

Electrolyte imbalances, emotional stress

Lung, liver, heart, kidney, brain

Infection, ICU

Rx drug

Injury, immobility

Untreated pain, unfamiliar environment

Metabolic disorders

key difference between delirium and dementia

a patient who has sudden cognitive impairment, disorientation, or clouded sensorium is most likely to have delirium

manifestations of delirium

• decreased ability to direct, focus, sustain, and shift attention and awareness

• hypoactive, lethargic to hyperactivity, agitation, and restlessness

• develops over hours to days

• impaired memory, judgement, and orientation

• rapid speech, rambling, incoherent

• disorganized thinking

• insomnia

• loss of appetite

• hallucinations

can delirium be irreversible

yes but most cases are reversible

diagnostic tests for delirium

• careful medical, psychologic history, and physical assessment

  • OTC and prescriptions

• Confusion Assessment Method (CAM)

• labs: CBC, LFTs, CMP, Cr, BUN, O2 sat

• lumbar puncture if meningitis suspected

• CT or MRI if head injury suspected

Confusion Assessment Method (CAM)

reliable tool for assessing delirium

4 features:

1. evidence of an acute change in mental status from baseline and did behavior fluctuate

2. problems with focusing attention?

   1. squeeze my hand when I say the letter A

3. disorganized thinking? (rambling, irrelevant conversation)

   1. ask simple questions like does a stone float on water

4. LOC (lethargic, vigilant, stupor)

   1. RASS (richmond agitation sedation scale): -4 = deep sedation, 0 = calm, 4 = combative

interventions for delirium

responsible for prevention, early recognition, and treatment

• if caused by drugs → discontinue

• if caused by electrolyte imbalance or nutritional deficiency → correct it

• if caused by infection → antibiotics

• reduce environment stimuli

• have calendars, clocks, and family photos

• remove unnecessary lines

• safe environment

• closer to the nurses station

• address pain and incontinence

considerations when using drug therapy to treat delirium

drug therapy is reserved for patients with severe agitation, especially when it interferes with needed medical treatments since it can put them at risk for falls and injury

drugs to manage agitation should only be used if nonpharmacologic interventions has FAILED because many of the drugs have psychoactive properties

seizure vs epilepsy

a sudden, abnormal, excessive electrical discharge of neurons in the brain → involuntary movements, sensory phenomena, emotional expression, and unusual behaviors

vs.

group of diseases marked by recurring seizures

what are some causes of a seizure?

• for most people it is unknown

• stroke

• brain tumors

• TBI

• neurodevelopmental disorders ex. Autism

• low or high glucose levels

• abnormal electrolytes

• eclampsia

• lack of sleep

• alcohol withdrawal

• heart, lung, liver, and kidney diseases

• lupus and MS (autoimmune disorders)

generalized onset seizure

starts over wide areas of both sides of the brain and are characterized by bilateral, synchronous neural discharges

patient has impaired consciousness for a few seconds to several minutes

subtype include tonic-clonic

tonic-clonic seizure

most common generalized onset motor seizure

lose consciousness and fall to the ground

the body will stiffen for 10-20 seconds and then extremities will jerk

can have cyanosis, excessive salivation, tongue or cheek biting, and incontinence

prodromal phase vs aural phase

sensation or behavior changes that precede a seizure by hours or days

vs.

sensory warning that is similar each time a seizure occurs and is a part of the seizure

ictal phase vs. postictal phase

1st symptoms to the end of the seizure activity

vs.

recovery period after the seizure

focal onset seizures

begin in 1 hemisphere of the brain in a localized region of the cortex

can cause sensory, motor, cognitive, or emotional manifestations

they will not remember what they did during the seizure

can have unexplained feelings of joy, anger, sadness; can walk into traffic or remove clothes

how are seizures diagnosed

most useful tool is an accurate, comprehensive description of seizures and the pt. health history

Electroencephalogram (EEG) can help determine the type of seizure and pinpoint the seizure’s focus; should be done within 24 hours of suspected seizure

get CBC, LFTs, BUN, Cr, urinalysis

priority nursing interventions during a seizure

• ensure patient airway

• protect from injury

  • do not restrain

  • pad side rails

  • remove sharp objects and glasses

• NEVER force anything between teeth

• remove/loosen tight clothing

• establish IV access

• stay with patient until it has passed

• anticipate giving phenytoin or benzos

• suction as needed

after:

• monitor VS, O2 sat, LOC, GCS

• reassure and reorient patient

• maintain NPO status until awake with gag reflex

• give dextrose for hypoglycemia

• PERRLA

if antiepileptic drug levels are below the therapeutic range, what will happen?

continuous seizure activity

when should EMS be called if someone is having a seizure?

if:

• seizure lasts longer than 5 minutes

• person is injured, pregnant, or cannot breathe

• another seizure immediately follows the first

• it is unknown if this was their first seizure

client and caregiver education for seizures

• take medications and teach the complications if you miss a dose

• seizure diary

  • note aura, nature of seizure activity, how long is lasts

• guide them to safety when seizure starts

• wear medical alert bracelet

• eat regular meals and rest

surgical therapy for those with defined site of seizure origin

resection, laser ablation, radiosurgery, or vagal nerve stimulator

Parkinson disease (PD)

chronic, progressive, neurodegenerative disorder characterized by slowness in the initiation and execution of movement (bradykinesia), increased muscle tone (rigidity), tremor at rest, and gait changes (postural instability)

as PD progresses, what other assessment findings may be present

• dysphagia → malnutrition and aspiration

• speech problems

• orthostatic hypotension

• immobility due to weakness

• loss of postural reflexes → falls and injury

• depression and hallucination

• constipation

how is PD diagnosed?

• has 2/4 main manifestations (tremors, rigidity, postural instability, or bradykinesia)

• positive response to antiparkisonian drugs

education for PD

• encourage exercise to avoid contractures, constipation, and muscle atrophy

• physical therapy for personalized exercise program

• OT for dressing and eating

• speech therapy for speaking and swallowing

• remove rugs and unnecessary furniture

• use slip on shoes or velcro and zippers (not buttons)

• use elevated toilet seat to get on and off easier

• well balanced diet

  • limit protein intake

  • 6 small meals a day rather than 3 large meals

  • cut food into bite sized pieces

deep brain stimulation (DBS)

most common surgical treatment for parkinsons

places a small electrode in the thalamus or other part of the brain and connects it to a generator in the upper chest

programmed to deliver a specific current to the targeted brain location to reduce increased neuronal activity and dyskinesia

ablation surgery

involves finding, targeting, and destroying an area of the brain affected by PD

goal is to destroy tissue that produces abnormal chemical or electrical impulses leading to tremors and other symptoms