Neuro/Degenerative Disorders

Neurologic Dysfunction Disorders

• Altered Level of Consciousness

• Delirium

• Dementia

• Seizures

• Headaches

Altered Level of Consciousness: Etiology

• Result of multiple pathophysiologic phenomena

  • Neurologic (head injury, stroke)

  • Toxicologic (drug overdose, alcohol intoxication)

  • Metabolic (hepatic or kidney injury, DKA)

• Disruption of cells, neurotransmitters, or brain anatomy → dysfunction in how the CNS communicates & coordinates function

Altered Level of Consciousness: Clinical Manifestations

• Changes in alertness & consciousness → alterations in pupillary response, eye opening, verbal response & motor response

• Behavioral changes: restlessness, anxiety

• Comatose:

  • Pupils responsive to light? → Possible toxic or metabolic etiology

Altered Level of Consciousness: Range of Categories (image + info)

Coma = state of unarousable unresponsiveness to internal and external stimuli, but can be responsive painful stimuli & brain stem reflexes may be present ---- presentation varies

• Akinetic mutism: not responsive to environment through voluntary movement

• Vegetative state: unresponsive but resumes sleep-wake cycles after coma; cognitive or affective mental function absent

• Minimally conscious state: similar to vegetative but they have some reproducible signs of awareness

• Locked-in syndrome: paralysis & inability to speak but are aware of environment; vertical eye movements & lid elevation intact

Altered Level of Consciousness: Assessment + Diagnostics

Assessment:

• Altered LOC can affect other body systems

• Evaluation of:

  • Mental status

  • Cranial nerve function

  • Cerebellar function (balance & coordination)

  • Reflexes

  • Motor & sensory function

• Glasgow Coma Scale (GCS):

  • Score of 3 = severe impairment of neuro function

  • Score of 15 = fully responsive

Diagnostics:

• Imaging:

  • CT, PCT

  • MRI/MRS

    • MRI = better at showing tumor size and blood vessel location

    • MRS = compares the chemical composition of the normal brain tissue w/ the abnormal

  • EEG

  • PET/SPECT

    • 3D images of the head that help w/ assessing tumors and how responsive they are to treatment

• Laboratory Tests:

  • Blood glucose

  • Comprehensive Metabolic Panel (CMP) - electrolytes, serum ammonia, liver function tests

  • Calcium level

  • Blood urea nitrogen (BUN)

  • Serum osmolality

  • Partial thromboplastin (PT) & prothrombin times (PTT)

  • Serum ketones

  • Alcohol & drug concentrations

  • Arterial blood gases

Altered Level of Consciousness: Management

• Obtain & maintain a patent airway:

  • Oral or nasal intubation

  • Tracheostomy

  • Mechanical ventilation may be required until patient’s ability to breathe on their own is clear

• Monitor circulatory status (blood pressure & heart rate)

• Obtain intravenous (IV) catheter for fluids & meds

• Nutritional support—via feeding tube or gastrostomy tube (G-tube)

• Determine and treat underlying cause of altered LOC

• Pharmacological management (depending on cause)

• Prevent further complications

Delirium

Acute confusional state that starts with disorientation that can progress to changes in level of consciousness, irreversible brain damage, and sometimes death.

Delirium: Background

• Can be considered a medical emergency d/t alteration in LOC

• Also known as acute confusional state

• Often mistaken for dementia

• Acute & unexpected onset

• Up to 80% of ICU patients affected

• Risk factors:

  • Use of benzodiazepines

  • Administration of blood transfusions

  • Age

  • Presence of dementia

  • Prior coma

  • Recent emergency surgery or trauma

Delirium: Clinical Manifestations

• Disorientation, confusion

• Agitation, restlessness, aggressive behavior

• Lethargy, withdrawn behavior

Delirium: Assessment + Diagnostics

• Ongoing mental status assessments

• Routine screening for all critically ill patients (for better recognition):

  • The Confusion Assessment Model (CAM) & Delirium Index (DI)

    • CAM = questions about acute onset, inattention, disorganized thinking, altered LOC, disorientation, memory impairment, perceptual disturbances, psychomotor agitation, psychomotor slowing, altered sleep-wake cycle

• Patient’s baseline mental status - onset of change

• Lab tests & imaging to rule out other causes—NO definitive lab tests or diagnostic procedure to diagnose

Delirium: Management

• Prevention is most effective

• Treat the underlying cause

• Minimize use of psychoactive drugs

Delirium: Nursing Interventions

• Assess for changes to cognition, memory, judgment and personality

• Providing therapeutic activities for cognitive impairment:

  • Safe, quiet, & calm environment

  • Provide familiar environmental cues to reorient

  • Alternative communication methods

• Maintain oxygen levels and fluid & electrolyte balance – Monitor I&Os carefully

• Include the family in care, if appropriate

• Ensure early, safe mobilization

• Pain control

• Notify provider about any nonessential meds that can be discontinued

• Promote proper sleep hygiene

• Encourage use of eyeglasses & hearing aids

• Understand the patient’s usual mental status

• Provide toileting schedule

• Provide finger foods

• Patient & family support:

  • Can be very disturbing for families

Dementia

Multiple cognitive, functional, and behavioral changes that destroy a person’s ability to function over time.

Dementia: Background

• Onset: subtle, but progresses slowly over time

• Usually cause from neurodegeneration

• Most common: Alzheimer’s disease (AD)

• After diagnosis, average survival is ~10 years

• Risk factors:

  • Advanced age, chemical imbalances, family history, previous head injury, assigned female at birth, ethnicity/race

  • AA & Hispanic people at increased risk

Dementia: Clinical Manifestations

• Memory problems

• Personality changes

• Loss of awareness, wandering behavior

• Disrupted sleep/wake cycle

• Decline in cognition

• Ataxia

• Progression different for each person

Dementia: Assessment + Diagnostics

• Health history (family as historian typically)

• Lab testing to rule out other causes

• MRI, CT/CAT, PET, EEG

• LP/CSF evaluation

Dementia: Nuring Interventions + Management

• Assess for changes in cognition, memory, judgment and personality

• Establish schedule for bowel & bladder program

• Family education about home safety measures:

  • Rugs, door locks & alarms, place mattress on the floor, shower chairs, medical ID bracelets

• Sundowning: phenomenon among patients with dementia experience increased agitation, confusion in late afternoon & early evening; more wandering, restlessness, etc.

  • Unknown cause – possible fatigue, circadian rhythm disturbances

  • Keep a consistent routine

  • Calm environment

  • Address triggers: hunger, pain, etc.

• Promote safe, quiet & calm environment

• Frequent walks

• Weekly skin checks

• Encourage cognitive stimulation & memory training

• Medications to target behavioral & emotional issues

• AD meds: donepezil, memantine, cholinesterase, pimavanserin

Seizures

Paroxysmal transient disturbance of brain, leading to discharge of abnormal electrical activity

Seizures: Pathophysiology

• Electrical disturbance (arrhythmia) in nerve cells in one part of the brain

• Episodes of abnormal motor, sensory, autonomic or psychic activity (or a combo of these)

• Abnormal, recurring uncontrolled electrical discharge

Seizures: Background

• Causes:

  • Allergies, brain tumor, cerebrovascular disease, CNS infections, drug & alcohol withdrawal, childhood fever, head injury, hypertension, hypoxemia of any cause

  • Metabolic & toxic condition: kidney injury, hyponatremia, hypocalcemia, hypoglycemia, pesticide exposure

  • Genetic etiology: structural & metabolic abnormalities

  • Epilepsy: more than one unprovoked seizure

Seizures: Clinical Manifestations

Varies depending on the location of discharging neurons:

• Epileptic cry—contraction of diaphragm & chest muscles

• Limb shaking

• Mouth jerking

• Dizziness

• Speak unintelligibly

• Unusual sensations

• Postictal state—may be confused, lethargic, agitated

Seizures: Assessment + Diagnostics

Assessment:

• During:

  • What happened before - consider type of stimuli, disturbances, sleep, hyperventilation

  • Presence of an aura

  • Characteristics of the episode - what does the episode look like?

  • Size & reactivity of pupils

  • Incontinence of urine or stool

  • Time of onset & duration of each phase

• After:

  • Any obvious paralysis or weakness of arms or legs after seizure?

  • Movements after? Is the patient sleepy? Able to talk?

  • Cognitive status - confused?

Diagnostics:

• Aim = to determine type of seizure, frequency & severity and factors that trigger them

  • Physical & neurologic exams

  • MRI

  • EEG (sometimes with video)

  • Telemetry & pulse oximetry

  • SPECT

Seizures: Medical Management

• Pharmacologic Therapy:

  • Control > cure

  • Anticonvulsants

  • Monitor for drug toxicity

• Surgical Management:

  • EEGs with depth electrodes

  • Hemostasis

  • Reduction surgery

  • Vagus nerve stimulator

Seizures: Nursing Interventions

• Protect the patient’s airway:

  • Risk for hypoxia, vomiting, & aspiration

• Perform suctioning as needed

• Ongoing assessments of respiratory & cardiac function:

  • Monitor responsiveness

• Provide privacy & maintain safety

• Ease to floor & protect head

• Place in side-lying position

• Loosen constrictive clothing, remove glasses

• Avoid restraining or prying jaw open

• Maintain seizure precautions:

  • Place bed in lowest position

  • Raise 2-3 side rails & displace pillows

  • Suction, O2, padded side rails

• Note the time of onset, duration, characteristics

• Reorient the patient to the environment:

  • Guide to bed or chair if wandering

  • Keep distance but able to visualize if agitated

Seizures: Forms of Presentation

• Tonic-clonic: full body stiffening & jerking w/ intermittent relaxing; loss of consciousness

  • Tonic: loss of consciousness, hypertonia

  • Clonic: rhythmic jerking & relaxing; last minutes

• Absence: blinking, loss of consciousness; last seconds

• Myoclonic: brief jerking & stiffening; last seconds

• Atonic/akinetic: loss of muscle tone + confusion; few seconds

• Complex partial: unconsciousness behaviors (lip smacking)

• Simple partial: conscious; unusual sensations

• Unknown: idiopathic; doesn’t fit into other categories

Status Epilepticus

• Medical Emergency!

• Lasts ≥ 5 minutes without full recovery

• Stop seizures ASAP to ensure cerebral oxygenation

• Vigorous muscular contractions → heavy metabolic demand → irregular respirations

Seizures: Special Considerations

Women:

• ↑ frequency during menses d/t ↑ sex hormones → affecting excitability of neurons in cerebral cortex

• Family planning

• Pregnancy:

  • ↑ risk of congenital fetal anomaly 2-3x higher

  • Maternal seizures, anticonvulsants, genetic predisposition - possible malformations

  • Anticonvulsants - need careful monitoring

  • High risk pregnancies - higher risk of epilepsy

    • Damage to fetus during pregnancy or delivery

• Bone loss r/t long-term use of anticonvulsant meds:

  • Assess for low bone mass & osteoporosis

Older Adults:

• Altered pharmacokinetics

• New onset epilepsy

  • Leading cause: CV disease

• Treatment depends on cause

• Polypharmacy & interactions

• Monitor closely for adverse reactions & toxicity

• At risk for osteoporosis

Headaches: Cranial Arteritis—Background + Clinical Manifestations

Background:

• Common in elderly (older adults)

• Inflammation of cranial arteries

• Localized around temporal arteries

Clinical Manifestations:

• Fatigue, malaise

• Weight loss

• Fever

• Heat, redness, swelling, tenderness or pain over involved artery

• Vision issues

Headaches: Tension—Background + Clinical Manifestations

Background:

• Chronic, less severe

• Very common

• Stress → contraction of neck & scalp muscles

• Patho unclear

• Possibly dilation of orbital & adjacent extracranial arteries

• Biopsy of artery to confirm

Clinical Manifestations:

• Constant, band-like pressure on forehead, temple, and/or back of neck

• “a weight on top of my head”

Headaches: Cluster—Background + Clinical Manifestations

Background:

• Pretty uncommon

• More frequent in men than woman

• Triggered by cough, exertion, and sexual activity

• Type of trigeminal autonomic cephalalgias  

Clinical Manifestations:

• Unilateral, migrating pain around eye and orbit that comes in episodes

  • Trigeminal autonomic cephalalgias—noted by pain on one side of the head with autonomic symptoms

    • Tearing, redness, nasal congestion on same side

• Rhinorrhea, watery eyes

• Pain described as penetrating, varying intensity

Headaches: Migraine—Background

• Marked by periodic, recurrent attacks of severe headaches lasting from hours to days

  • Cause still unclear…

• With or without aura (warning sensation)

What are some migraine headache triggers?

• Menses

• Bright lights

• Stress

• Depression

• Sleep deprivation

• Fatigue

• Odors

• Cheese, red wine, beer, chocolate (tyramine)

• Monosodium glutamate (MSG)

• Oral contraceptives for some

Headaches: Migraine—Pathophysiology (theory)

Stimulation → vascular changes, inflammation and continued pain signal stimulation

• Hyper-excitable brain that’s more vulnerable to a wave of depolarization over the cerebral cortex, cerebellum, & hippocampus →

• Inflammatory neuropeptides & other neurotransmitters activating →

• Stimulation of meningeal nociceptors

Headaches: Migraine—Clinical Manifestations

1. Premonitory:

   • Depression, irritability

   • Feeling cold, light & sound sensitivity

   • Change in activity level

   • Food cravings, anorexia

2. Aura:

   • Vision disturbances

   • Slight extremity weakness

   • Mild confusion; drowsiness, dizziness

   • Numbness and tinging of lips, face or hands

3. Headache:

   • Pain, light & sound sensitivity

   • Nausea/vomiting

   • Cognitive difficulties & mood changes

4. Postdrome:

   • Fatigue, weakness

Headaches: Assessment + Diagnostics

Assessment: OLDCARTS

• Detailed history:

  **Purpose of hx - assess headache while considering possible factors that were precipitating or provoking the episode (different person to person!)

  **Have the patient describe headache in their own words

  • Medications

    • Prescribed, OTC; complete hx

      • Anti-HTN, diuretics, anti-inflammatory agents, MAO inhibitors - known to provoke headaches

  • Family history

  • Triggers: stress, poor sleep habits, food

  • Occupational history

    • Toxins?

  • Medical & surgical history of all body systems

• Physical assessment with focus on head & neck:

  • Frequency, location, quality, duration

    • Persistent headaches warrant investigation because it can be serious (brain tumors, subarachnoid hemorrhages, strokes, severe hypertension, meningitis, head injuries)

    • Neck stiffness— meningitis? spinal injury?

• Thorough neurologic assessment

Diagnostics:

• Usually not helpful due to lack of objective findings

• CT

• Cerebral angiography

• MRI—to detect possible causes like tumors or aneurysm

• Electromyography (EMG)—sustained contraction of neck, scalp, or facial muscles

• CBC

• Erythrocyte sedimentation rate (ESR)

• Electrolytes

• Glucose

• Creatinine

• Thyroid hormones

Headaches: Interventions

Goal: to relieve pain & provide comfort

• Cranial arteritis:

  • Corticosteroids—prevent loss of vision d/t vascular occlusion or rupture --- DO NOT d/c

• Migraine: abortive & preventative approaches

  **Abortive = relieving or limit a headache at onset or while it's in progress

  **Preventive = used for pts with more frequent episodes at regular or predictable intervals & might have medical condition that makes abortive therapies ineffective

  **Migraine cocktail

  • Non-invasive neuromodulation devices

  • Triptans (serotonin receptor agonists)

  • NSAIDs, antispasmodic agents, neuroleptics

  • Anti-emetics

  • Prophylaxis:

    • Beta-blockers, antiepileptics, antidepressants, ACE inhibitors, ARBs

• Cluster:

  • 100% O2 by facemask for 15 minutes

  • Sumatriptan (subcutaneous) & Zolmitriptan (nasal)

    • Vasoconstriction, anti-inflammatory, reduce pain

• Tension:

  • Local heat or massage

  • Analgesics, antidepressants, muscle relaxants

Headaches: Prevention

• Avoid specific triggers:

  • Alcohol—vasodilation of blood vessels

  • Nitrites, vasodilators, histamines

  • Foods with tyramine - chocolate, cheese, coffee, dairy products

  • Long periods between meals

• Adhering to medication regimen

• Regular sleep

• Stress management—meditation, exercise, biofeedback

• Environment:

  • Dark, quiet room

  • Elevating HOB to 30°

• Cold compress

• Headache diary

• Hormonal fluctuations from menstrual cycle pattern can also affect migraines

• Symptom management

Infections of the Nervous System

• Meningitis

• Herpes Simplex Encephalitis

• Creutzfeldt-Jakob disease (CJD) & Variant CJD

Meningitis

Inflammation of the meninges, which cover and protect the brain & spinal cord

Meningitis: Background

• Main types: bacterial (septic) & viral (aseptic)

  • Streptococcus pneumoniae and Neisseria meningitidis

  • Aseptic - secondary to cancer or r/t immunosuppression

    • Commonly due to enteroviruses

• Immunization:

  • Haemophilus influenzae type B (Hib) vaccine

  • Pneumococcal polysaccharide vaccine (PPSV)

  • Meningococcal vaccine (MCV4): 1st year college students & members of the military

  • No vaccine for viral meningitis

Meningitis: Risk Factors

• Bacterial: bacterial-based infections (otitis media, pneumonia, sinusitis) caused by N. meningitidis, S. pneumoniae, or H. influenzae

• Viral: mumps, measles, herpes, arboviruses

• Immunosuppression

• Direct contamination of spinal fluid

• Invasive procedures, skull fractures, or penetrating wounds

• Environment—living in close quarters

Pathogen can travel through nose & throat, crossing blood-brain-barrier → bloodstream → CSF:

• Head trauma, sinusitis → abscess, or organism just enters the bloodstream due to another infection

• Can pass through the endothelial membrane to the subarachnoid space

• Consequence of infection can be devastating → vascular necrosis and endothelial damage → neurological deficits, organ failure, seizures, etc.

Meningitis: Cause

Pathogen travels into bloodstream

↓

Crosses blood brain barrier

↓

Multiplies in CSF

↓

Host immune response

↓

Inflammation of subarachnoid space & pia mater

↓

↑ intracranial pressure

↓

Inflammatory materials circulates via CSF

↓

Endothelial damage & vascular necrosis

Meningitis: Clinical Manifestations

• Fever & chills

• Steady, throbbing headache

• Nuchal rigidity:

  • Neck immobility—an early sign!

  • Trying to flex the neck causes spasms…

• Positive Kernig sign:

  • Patient lying down with thigh flexed on abdomen, leg cannot be extended

    • If bilateral: meningeal irritation

• Positive Brudzinski sign:

  • Patient's neck flexed → knees & hips flexed too

    • Lower extremity of one side is passively flexed, the same thing happens on the opposite extremity

    • Brudzinski > Kernig when indicating meningitis

• Photophobia

• Rash:

  • Common feature with meningococcal meningitidis (half of patients present with rash)

  • Skin lesions that have develop into varying degrees of petechiae, lesions and bruising

• Disorientation & memory impairment

• Seizures

• Mental status changes

• Irritability

• Hyperactive DTRs

**Older adults - have more behavior changes & focal neurologic deficits

Meningitis: Assessment + Diagnostics

• CT

• MRI

• Lumbar puncture & CSF analysis:

  • Glucose, ↑WBCs, ↑ protein, pressure

• CBC

• Urine, throat, nose, & blood culture & sensitivity

---

CT/MRI before LP

• Rule out other conditions, especially those that may cause herniation from LP (altered LOC, papilledema, hx of CNS disease, being immunocompromised)

• Specimen cultured and gram stained + blood culture

• Gram staining → quick ID of causative bacteria à quickly starts on correct ABX therapy

CSF - assessment starts at appearance

• Measure pressure with manometer (looks like a giant traditional glass thermometer)

• Cloudy (bacterial) & clear (viral)

• ↓ glucose (bacterial)

• Collected before ABX!!!

Symptom driven: throat & nose cultures less common (not definitive for meningitis)

Meningitis: Treatment + Complications

Treatment:

• Early administration of ABX

  • Penicillin G + cephalosporin IV

  • ABX → cross BBB into subarachnoid space → halt bacteria multiplication

• Dexamethasone

  • Acute bacterial meningitis & pneumococcal if given with ABX & every 6 hrs for next 4 days

• Fluid volume expanders

  • Shock & dehydration

  • Shown to improve outcomes in adults, no increased risk of GI bleed

• Anticonvulsants

• Treatment for ICP, if indicated

• Also consider vaccine + ABX prophylaxis for anyone living with meningococcal infection

• 2 vaccines = recommended for children and adults who are at-risk

• Exposed individuals:

  • Those exposed – 3 ABX

    • Rifampin, ciprofloxacin, or ceftriaxone

  • Started within 24 hours post exposure

  • Vaccine + ABX chemoprophylaxis

    • H. influenzae & S. pneumoniae vaccines

Complications:

• Increased ICP:

  • Inflammation possibly to point of herniation

    • Monitor for s/sx

• SIADH:

  • d/t abnormal simulation of area near hypothalamus → excess secretion of ADH

    • Concentrated urine (high osmolality) with lots of sodium

    • Low SERUM sodium

    • Daily weights

• Septic emboli:

  • Forms during infection & moves to other parts of body → causing gangrene → DIC or stroke

    • Skin assessment + neurovascular assessments

    • Fever management

Meningitis: Interventions

• Prevention!

  • Meningococcal conjugated vaccine

• Isolate the patient immediately!

  • Droplet until 24 hrs post ABX

• Report meningococcal infections to public health department

• Supportive care

• Manage fever

• Neuro assessments & vital signs continually

• Hydration

• Monitor for early signs of shock

• Seizure precautions

• Prevent secondary complications

• Family support

• Rehabilitation

• Health promotion

***Tell patient to try to avoid coughing and sneezing – could increase ICP

Herpes Simplex Encephalitis

Acute inflammatory process of brain tissue

Herpes Simplex Encephalitis: Background

Caused by herpes simplex virus (HSV) —most common (acute) cause of encephalitis

• Marked by hemorrhagic tissue death → edema

• Progressive deterioration of nerve cell bodies

Herpes Simplex Encephalitis: Clinical Manifestations

• Initial symptoms of HSV: Fever, headache, confusion & hallucinations

• Behavioral changes

• Focal seizures

• Dysphasia

• Hemiparesis

• Altered LOC

Herpes Simplex Encephalitis: Assessment + Diagnostics

• EEG: diffuse slowing or focal changes in temporal lobe for most

• CSF analysis (LP): high opening pressure, ↑ protein

• MRI: detect inflammation = hypertense (bright) area

• Polymerase chain reaction (PCR)*: early diagnostic test for HSV; very sensitive to genetic material of HSV

Herpes Simplex Encephalitis: Interventions

• Antiviral agent: IV acyclovir for up to 3 weeks

  • Slow administration over 1 hour*—Admin slow d/t crystallization of med in urine

  • Early administration → improves prognosis

  • Inhibits viral DNA replication

  • ↓ dose with pt hx of renal insufficiency

• Ongoing neuro assessment

• Supportive care—dim lights, limit noise, cluster care

• Opioids ⚠—mask neuro symptoms!

• Injury prevention & safety—monitor for sx & changes in LOC

• Family support—help ease anxiety

• Monitor blood chemistries & UOP—watching for renal complications r/t antiviral therapy

Creutzfeldt-Jakob disease (CJD) & Variant CJD

Rare, degenerative and fatal disorder caused by abnormal proteins (prions) resulting in rapid cognitive & neurological deterioration

Creutzfeldt-Jakob disease (CJD) & Variant CJD: Background

• Transmissible spongiform encephalopathies (TSE): group of degenerative, infectious neurologic disorders

  • TSE = broader term for CJD, vCJD and other prion diseases

• Rare with unknown cause

• CJD: mostly sporadic; can be caused by mutation, hereditary, or iatrogenic

• vCJD = human variant of bovine spongiform encephalopathy (BSE) (mad cow disease)

  • Result of ingesting prion-infested meat

  • Prions → TSEs

• Shared characteristic of CJD & vCJD = lack of CNS inflammation

• No definitive treatment

• Fatal outcomes within 1 year of symptom onset

• Mostly in UK; risk low in US:

  • Risk low in US d/t cattle fed mainly with soy-derived feed vs. animal-containing feed

Creutzfeldt-Jakob disease (CJD) & Variant CJD: Pathophysiology

• Prions crosses BBB → deposits in & breaks down brain tissue → cell death → spongiform changes proliferation

• Prions—in lymphoid tissue & blood:

  • American Red Cross & UK donations

Creutzfeldt-Jakob disease (CJD) & Variant CJD: Clinical Manifestations

CJD:

• Mental deterioration, ataxia, visual disturbances

• Memory loss, involuntary movement, paralysis, mutism

• Late: psychiatric symptoms

• Mean age of onset: 65 years

• Survival after presentation: < 1 year

vCJD:

• Early: Psychiatric symptoms

• Behavior changes, sensory disturbances, limb pain

• Muscle spasms & rigidity, dysarthria (difficulty speaking), incoordination, cognitive impairment & sleep disturbances

• Mean age of onset: 27 years

• Survival after presentation: ~14 months

Creutzfeldt-Jakob disease (CJD) & Variant CJD: Assessment + Diagnostics

• Immunologic assessment + EEG + MRI

  • Confirm by brain biopsy - NOT recommended

  • Post-mortem is preferred

  • Detection of protein kinase inhibitor (14-3-3)

  • EEG: starts with periodic activity; then periodic spikes alternating with slow periods

  • MRI: symmetric or unilateral hyperintense signals from basal ganglia

Creutzfeldt-Jakob disease (CJD) & Variant CJD: Management

• No effective treatment for CJD & vCJD

• Supportive & palliative:

  • Prevention r/t immobility & dementia, promotion of patient comfort, provision of support & education for family

• Patient & family support:

  • Grief & loss

  • Hospice services – at home or inpatient

• Transmission prevention:

  • Standard isolation precautions—no specific patient isolation

  • ⚠  Handling of brain, spinal cord, pituitary gland and eye tissue

  • Careful handling of specimens

  • Disposable surgical instruments:

    • Sterilizations doesn’t kill prions

    • Bleach & extended sterilization time if not disposable

Autoimmune Nervous Disorders

• Multiple Sclerosis

• Myasthenia Gravis

• Gullian-Barré Syndrome

Multiple Sclerosis

Immune-mediated, progressive demyelinating disease of the CNS

Multiple Sclerosis: Background

• Demyelination = destruction of the fatty & protein material that encapsulates certain nerve fibers in brain and spinal cord ⟶ impaired transmission of nerve impulses

• Affects 400,000 people in US

• Can occur at any age:

  • Most common between age 20-50

• More common in women > men

• Cause unknown; ongoing research:

  • Autoimmune activity—demyelination but not clear about specific sensitized antigen

  • Environmental factors—obesity, ↓ Vit D exposure, high salt diet in teens

  • Geographical relationship:

    • More frequent in northern colder latitudes

    • ↑ prevalence in Europe, New Zealand, southern Australia, northern US and southern Canada ---- less common in Asian populations

  • Genetic variations r/t MS—not necessarily hereditary – genetic variations

  • Infectious trigger

  • Other factors—physical injury, emotional stress, pregnancy, fatigue, overexertion, temperature extremes, hot shower/bath

• 4 main clinical forms:

  • Remitting-relapsing (RRMS):

    • Most common; patient reports development of new findings and function loss (relapse) --- symptoms mild to moderate & resolve within a few weeks to months (remission)

  • Secondary progressive (SPMS):

    • Starts as RRMS → continuously progressive

  • Primary progressive (PPMS):

    • MS with gradual & continuous reduction of CNS deterioration with remission

  • Progressive-relapsing (PRMS):

    • MS with frequent relapses with partial recovery w/o return to baseline

Multiple Sclerosis: Pathophysiology

Sensitized T & B lymphocytes cross BBB

↓

Sensitized T lymphocytes stay & promote infiltration of other agents

↓

Damage to immune system

↓

Inflammation

↓

Destruction of mostly white matter of CNS myelin & oligodendroglial cells; plaque formation

↓

Interrupted flow of nerve impulses

↓

Degeneration of axons

↓

Permanent & irreversible damage

Multiple Sclerosis: Clinical Manifestations

• Dependent on area of CNS affected by demyelination

• Fatigue—influenced by heat, depression, anemia, meds, etc

• Weakness, numbness

• Dysphagia

• Difficulty in coordination, loss of balance

• Spasticity

• Pain: (lesions on sensory pathways)

  • Perimenopausal women: pain r/t osteoporosis—loss of estrogen, immobility and corticosteroids → osteoporosis

    • Bone mineral density testing recommended!

  • ALSO Pins & needles, abnormal sense of touch, proprioception loss

• Visual disturbances

• Cognitive & psychosocial difficulties:

  • Depression

  • Memory loss, emotional lability, ↓ concentration—Dementia in severe, rare cases

• Ataxia, tremors

• Bowel, bladder & sexual dysfunction

• Exacerbations & remissions

• Less severe forms:

  • RIS: no symptoms

  • CIS: uniliteral optic neuritis, focal symptoms or partial myelopathy

  Main forms:

  • RRMS: complete recovery with each relapse but may occur over time → decline in function

    • Most RRMS cases → SPMS (can occur with or without relapses)

  • PPMS: disabling symptoms gradually increase + rare plateaus & temporary minor improvement

    • Quadriparesis, cognitive dysfunction, visual loss, brainstem syndromes

  • PRMS: relapses with continuous disabling progression b/t exacerbations

Multiple Sclerosis: Gerontologic Considerations

• Life expectancy: 7-14 years shorter than average

• Specific physical & psychosocial needs

• Altered pharmakinetics due to age:

  • Monitor for adverse effects & toxicity

  • Might have chronic health problems + other medications that they’re taking → interactions

    • Osteoporosis ←→ corticosteroids

• Cost of medications = chance for poor adherence, esp. for those on fixed income

• Growing concerns:

  • Progressive disability

  • Family burden, marital concerns

  • Possibility of home nursing care

  • Immobility, physical challenges → loneliness & depression

Multiple Sclerosis: Assessment + Diagnostics

• Based on clinical findings, imaging & lab findings

• Neuropsychological testing—test for cognitive impairment

• MRI: plaques in CNS distributed in different areas

• LP/CSF analysis: presence of oligoclonal binding

• Evoked potential studies—measure electrical activity in parts of CNS in response to stimuli

• Urodynamics—– to diagnose underlying bladder dysfunction

Multiple Sclerosis: Management

• Incurable

• Goal: to delay progression of disease, manage chronic symptoms, treat acute exacerbations

• Immunomodulators

• Interferon beta-1a & interferon beta-1b

• Glatiramer acetate

• Teriflunomide, fingolimod, dimethyl furmarate

• IV methylprednisolone

• IV Mitoxantrone

• Symptom management:

  • Treat spasticity: antrolene, baclofen, diazapam

  • Paresthesia: carbamazepine

  • Treat constipation: docusate sodium

  • Manage bladder dysfunction: anticholinergics

  • Ataxia: propranolol, clonazepam

  • Treat fatigue: amantadine, pemoline, dalfampridine, baclofen, tizanidine

Multiple Sclerosis: Nursing Interventions

• Assess for changes in visual acuity, activity tolerance level, skin integrity, speech & swallowing abilities

• Look out for cognitive changes

• Monitor I&Os & encourage fluid intake

• Promote physical mobility & independence when possible

• Exercise, stretching, ROM exercises

• Energy conservation—avoid overexertion

• Reduce risk for injury

• Coping strategies—stress management

• Alternative therapies: yoga, meditation, aromatherapy, acupuncture, massage

• Monitoring & managing complications

• UTIs, constipation, pneumonia

• Medication education

• Outpatient management:

  • Interprofessional team: neurology, ophthalmology, physical therapy, OT, mental health provider, case management, social work

Myasthenia Gravis

Autoimmune disorder that affects myoneural junction

Myasthenia Gravis: Background

• Characterized by varying degrees of weakness in voluntary muscles

• Relatively uncommon but affects 9-30 in 1 million in US

• More prevalent in women in 20-30s

• But more common in men after age 50

Myasthenia Gravis: Pathophysiology

Antibodies targeting acetylcholine receptors

↓

Impaired impulse transmission

↓

Less receptors available for stimulation

↓

Voluntary muscle weakness that gets worse with ongoing activity

_____

**Normal: chemical impulse → release of acetylcholine from vesicles on nerve terminal myoneural junction → ACh attaches to receptor site on the motor endplate → muscle contraction

**Continuous binding of ACh receptor site REQUIRED for muscle contraction to be sustained

Myasthenia Gravis: Clinical Manifestations

• Highly variable

• Types: Ocular & Clinical

• Diplopia

• Ptosis—drooping of eyelids

• Muscle weakness:

  • Face & throat (bulbar symptoms)

  • Respiratory

• Dysphonia (voice impairment) & Dysphagia

• Respiratory failure = myasthenic crisis

Myasthenia Gravis: Assessment + Diagnostics

• Acetylcholinesterase inhibitor test:

  • Administer edrophonium chloride IV & give 30 sec → facial muscle weakness & ptosis resolved for 5 min.

  • Atropine for possible side effects—to control bradycardia, asystole, bronchoconstriction, sweating, cramping

• Ice test:

  • Ice pack over eyes x 1 minute to temporarily resolve ptosis

• Antibody testing—Multiple blood tests

• Repetitive nerve stimulation (RNS)—↓ successive action potentials

• Single-fiber electromyography (EMG)—picks up on delay or failure of neuromuscular transmission; has 99% sensitivity

• Enlarged thymus gland - can be identified with MRI:

  • thymus = site of acetylcholine receptor antibody production

Myasthenia Gravis: Management

• Goal: To improve function & to reduce and remove circulating antibodies

  • Pyridostigmine bromide - 1st line of therapy**

    • PB = anticholinesterase —inhibits breakdown of neuromuscular junction → muscle strength & control fatigue

      • Dose gradually increase to a daily max, then given in divided doses (~4 x day)

      • Side effects: diarrhea, abdominal cramps, and/or excessive saliva

      • PB has fewer side effects than other anticholesterase meds

  • Immunosuppressive therapy:

    • Corticosteroids (Prednisone)

    • Cytotoxic meds (azathioprine)

    • Adverse Effects: leukopenia & hepatotoxicity → monthly liver enzymes & WBC checks

• IVIG—for exacerbation but sometimes long-term basis (monthly infusions)

  • Pooled human gamma-globulin – lasts for ~4 weeks

  • Complications: headache, flu-like symptoms, aseptic meningitis

• Plasmapheresis—(therapeutic plasma exchange)—helps treat exacerbation

  • Plasma & plasma contents removed through CVL, large-bore double lumen

  • Blood cells & antibody-containing plasma separated à cells and plasma substitute reinfused

  • Temporary reduction in antibodies in blood

  • Daily or alternating days

• Thymectomy—removal of thymus gland (produces antigen-specific immunosuppression)

  • Only treatment for complete remission (only 35%)

  • Best for those under age 60 with a diagnosis of MG for 3 years

  • Plasmapheresis or pre-op IVIG → less time on the vent

  • ~3 years post op to see benefit (circulating T cells have long life)

Myasthenia Gravis: Nursing Interventions

• Education on outpatient self-care:

  • Energy conservation

  • Strategies for life with visual impairment

  • Prevention & management of complications:

    • Triggers

• Medication management—lots of meds might be contraindicated for MG & could exacerbate symptoms

  • Procaine—should be avoided, notify dentist of diagnosis

• Risk of choking & aspiration

• Psychosocial support

Myasthenic Crisis

• Exacerbation of disease characterized by severe generalized muscle weakness, respiratory and bulbar weakness → respiratory failure

  • Inadequate cough, impaired gag reflex → ineffective airway clearance

• Most common trigger: respiratory infection

• Worsening pulmonary function test—– 1^st clinical sign of declining resp function

  • Negative inspiratory force & vital capacity

• Respiratory support:

  • Intubation & mechanical ventilation

  • Noninvasive positive pressure ventilation (BiPAP, CPAP)

  • Chest physiotherapy

  • ABGs, serum electrolytes, I&Os, daily weights

• Enteral feeds—if risk for aspiration

• Temporarily stop cholinesterase inhibitors:

  • Cholinergic crisis = due to overmedication with cholinesterase inhibitors (rare)

    • Reason for temporarily stopping during myasthenic crisis – it could mimic or worsen the symptoms

    • Also results in resp. failure due to weakness of respiratory muscle & bulbar weakness

Gullian-Barré Syndrome

Autoimmune attack on the peripheral nerve myelin

Gullian-Barré Syndrome: Background

• Acute idiopathic neuritis

• Leads to acute, rapid sections of demyelinated peripheral nerves & some cranial nerves → ascending weakness with dyskinesia (inability to execute voluntary movements), hyporeflexia & paresthesias (sensation of numbness & tingling; “pins and needles”)

• Usually preceded by a viral infection:

  • *Campylobacter jejuni

  • Cytomegalovirus

  • Epstein-Barr virus

  • Mycoplasma pneumoniae

  • H. influenzae

  • Zika virus

• Several subtypes

• 1-2 per 100,000 people affected annually;

  • 5-10% result in death from resp. failure, autonomic dysfunction, sepsis or pulmonary embolism

  • 70% fully recover, remainder left with varied disability

Gullian-Barré Syndrome: Pathophysiology

Infectious organism has an amino acid that imitates that of peripheral nerve myelin protein

↓

Immune system can’t distinguish between the two; attacks & destroys peripheral myelin at GM1b

↓

↑ in macrophages & other immune-mediated agents to attack myelin

↓

Inflammation & destruction, interrupted nerve conduction & axonal loss

Gullian-Barré Syndrome: Clinical Manifestations

Symptoms typically show up 1-3 after preceding event

• Typically beginning with leg weakness → progressing upward to neuromuscular resp. failure & bulbar weakness

• Reaches peak around 2 weeks but doesn’t last longer 4 weeks

• > 4 weeks = chronic inflammatory demyelinating polyneuropathy

  __________________

• Bilateral muscle weakness, hyporeflexia in lower extremities:

  • Could become tetraplegia

• Bulbar weakness

• Paresthesias of hands & feet

• Pain

• Blindness

• Difficulty swallowing or clearing secretions

• Autonomic dysfunction—tachy, brady, hypertension, hypotension --- resolve quickly!

• Neuromuscular respiratory failure

***Does NOT affect cognition or level of consciousness

Gullian-Barré Syndrome: Assessment + Diagnostics

• Health history, physical exam (if any recent viral illness)

• Negative inspiratory force, vital capacity

• Electrophysiology studies—measure nerve conduction velocity which can tell how much the disease has affected nerve conduction

Gullian-Barré Syndrome: Management

Medical Emergency!

• Plasmapheresis (therapeutic plasma exchange)

• IVIG

• May require mechanical ventilation

• IV fluids

• Alpha-adrenergic blocking agents

Gullian-Barré Syndrome: Nursing Interventions

• Monitor need for intubation - signs of respiratory failure

• Suctioning as needed

• Monitoring for autonomic dysfunction - ECG monitoring

• Prevent complications from immobility:

  • Position changes, anticoagulants, SCDs, range-of-motion exercises

• Administer IV fluids or parenteral nutrition

• Reduce fatigue & maintaining independence

• Rehabilitation

• Psychological support to patient & family:

  • Identify support needs in preparation for discharge

Degenerative Disorders

• Muscular Dystrophies

• Huntington’s Disease

• Amyotrophic Lateral Sclerosis

• Degenerative Disc Disease

Muscular Dystrophies

Group of incurable muscle disorders marked by progressive weakening & wasting of skeletal or voluntary muscles

Muscular Dystrophies: Background

• 30 different types, mostly inherited:

  • Differentiated based on genetic pattern of inheritance, muscles involved, age at onset, rate of disease progression

• Most common: Duchenne muscular dystrophy

• Pathogenic features:

  • Degeneration & loss of muscle fibers

  • Variation in muscle fiber size

  • Phagocytosis and regeneration

  • Replacement of muscle wasting by connective tissue

• Prognosis depends on type of dystrophy:

  • Unique needs for newly aging population

Muscular Dystrophies: Clinical Manifestations

• Varying degrees of muscle weakness & wasting

• Abnormal elevation of serum levels of muscle enzymes

• Decreased respiratory reserve

• Cardiomyopathy

Muscular Dystrophies: Management

• Supportive care—to promote activity and optimal normal function & keeping functional deterioration at a minimum

• Prevention of complications

• Therapeutic exercise programs—–prevents muscle tightness, contractures, disuse atrophy

• Night splints, stretching exercises—delays development of contractures of joints (ankles, knees & hips)

• Braces—support body to compensate for weakness of muscles

• Spinal deformity: muscle weakness & spinal collapse

  • Orthotic vest (to prevent)—improves stability when sitting and supports CV status

  • Spinal fusion, eventually

• Pulmonary function—d/t disease progression or deformity of thorax secondary to severe scoliosis

  • URIs & fractures from falls – addressed promptly to avoid immobilization since contractures are a lot worse with inactivity

• Other difficulties:

  • Dental hygiene

  • Speech & swallowing problems

  • GI issues—gastric dilation, rectal prolapse, fecal impaction

  • Cardiomyopathy

  • Genetic counseling—encouraged for parents & siblings of patients d/t strong genetic component

Muscular Dystrophies: Nursing Interventions

• Goal: to maintain function at optimal levels & improve quality of life

• Maintain home care routine when able

  • Consider your patient’s insight and knowledge when making decisions – they, along with family, know what strategies have worked for them to function at home

    • Try to promote home care routine during hospitalization as much as possible

    • Important component of caring for patient with chronic issues – helps them maintain some sense of normalcy

• Transition from pediatric care to adult care (newly aging population – helping with transition into adulthood)

• Promote independence as much as possible safely

• Disease progression education

• Rehabilitation programs

• Range-of-motion exercises

• Patient & family support:

  • End-of-life decisions

  • Mental health

Huntington’s Disease

Chronic, progressive herditary disease of nervous system that causes progressive involuntary choreiform movement & dementia

Huntington’s Disease: Etiology + Pathophysiology

• Premature death of cells in:

  • Striatum of basal ganglia (involved in movement control) + Cortex (involved with thinking, memory, perception, judgment & behavior) + Cerebellum (voluntary muscle activity coordination)

• Cause not known but possibly due to glutamine collecting in cell nucleus abnormally → cell death

• Hereditary; passed on by autosomal dominant gene:

  • Each child of parent with disease have 50% chance of inheriting

  • Genetic testing:

    • Identify gene presence but not timing of onset

• Everyone has the gene for Huntington Disease BUT the people with the expansion of the gene can develop the disease and pass it on

  • Affects 1 in 10,000 men and women at midlife—rare

  • Choreiform movements even when asleep

Huntington’s Disease: Clinical Manifestations

Characteristic triad:

• *Motor dysfunction: (chorea - rapid, jerky, involuntary, purposeless movements); facial tics & grimaces

• *Cognitive impairment: difficulties with attention & emotion recognition

• *Behavioral features: apathy, blunted affect

Constant writing, twisting, uncontrollable movements of whole body (choreiform movements)

• Disorganized gait

Other clinical manifestations: difficulties with chewing & swallowing, bladder & bowel incontinence

Huntington’s Disease: Assessment + Diagnostics

• Clinical presentation of characteristic symptoms

• Positive family history

• Known presence of genetic marker cytosine-adenine-guanine (CAG) repeating on Huntington gene (HTT)

• CT/MRI: symmetrical striatal atrophy prior to motor symptoms developing

Huntington’s Disease: Management

• No curative or reversing treatment

• Multidisciplinary: social work, occupational therapy, speech, physical therapy, palliative care

• Goal: To optimize quality of life with supportive treatment

• Ongoing assessment of motor signs to evaluate drug efficacy:

  • Akathisia (motor restlessness) with overmedication = DANGEROUS

    • Can be seen as restless fidgeting of illness & could be overlooked

    • Hypokinetic motor impairment -- resembles Parkinson's

  • Misleading symptoms

  • Tetrabenazine—manage chorea

  • Benzodiazepines & neuroleptic drugs

  • Antiparkinson (Levodopa)—manage rigidity sometimes

• SSRIs, tricyclic antidepressants—psychiatric symptoms

Huntington’s Disease: Transitional Care

• Coping with progression

• Supportive care

• Follow-up visits

• Home care, day care centers

• Respite care, skilled long-term care

• End-of-life care planning

Amyotrophic Lateral Sclerosis

Loss of motor neurons in anterior horns of spinal cord & motor nuclei of lower brainstem

Amyotrophic Lateral Sclerosis: Etiology + Pathophysiology

Also known as Lou Gehrig disease

• Cellular death of motor neurons → progressive weakness & atrophy of muscle fibers in extremities

• Cause unknown:

  • Thought to be d/t overexcitation of nerve cells by glutamate → cell injury & neuronal degeneration

• Risk factors: age, autoimmune diseases, environmental exposure to toxins, family hx, smoking, viral infections

  • Onset age: 40-60 years old

  • Affects men a little more often than women

  • 10% of cases are familial; onset occurs 10 years earlier than average

• Clinical presentation & prognosis dependent on area of CNS involved & speed of disease progression

Amyotrophic Lateral Sclerosis: Clinical Manifestations

• Fatigue

• Progressive muscle weakness, cramps, twitching

• Lack of coordination

• Spasticity

• Overactive deep tendon reflexes

• Emotional lability, cognitive impairment

• Soft palate & upper esophagus weakness → Difficulty with liquids

  • Posterior tongue & palate weakness → inability to laugh, cough, or blow nose

  • Bladder & anal sphincter intact d/t spinal nerves not affecting rectum & bladder

  • Death—usually from infection, respiratory insufficiency, or aspiration

• Difficulty speaking, swallowing & eventually breathing

Amyotrophic Lateral Sclerosis: Assessment + Diagnostics

• No ALS specific testing

• Electromyography (EMG)

• Muscle biopsy studies of affected muscles

• MRI

• Neuropsychological testing

**EMG & muscle biopsy studies of affected muscles - reduction in number of functioning motor units

**MRI - high signal intensity of corticospinal tracts

Amyotrophic Lateral Sclerosis: Management

• No cure

• Maintain or improve function, well-being, & quality of life

• Riluzole & edaravone = ALS treatments but unsure how it works

• Symptom management:

  • Baclofen, dantrolene sodium, diazepam (all—painful spasticity)

  • Modafinil—fatigue

  • Medications for pain, drooling, constipation

    • Depression & functional impairment

• Rehabilitative measures

• Clinical trials:

  • ALS registry

• Life at home:

  • Hospitalizations

  • End-of-life issues

• Mechanical ventilation:

  • Non-invasive positive pressure ventilation (NPPV)

  • Tracheotomy

• PEG tube

Degenerative Disc Disease

Disorder that results from the breakdown of intervertebral discs in spine due to aging or injury, causing pain, stiffening or decreased mobility 

Degenerative Disc Disease: Background

• Low back pain—2nd most common neurological disorder:

  • Migraines = most common

• Most common reason for missed work & ↓ productivity

• Commonly associated with depression, anxiety, smoking, alcohol abuse, obesity, stress

• Most recover within 4-6 weeks

  • Acute pain < 3 months; chronic pain is > 3 months