neuro/musculoskeletal by diana blum rn msn metropolitan community college
TRANSCRIPT
NEURO/M
USCULOSK
ELETA
L
B Y D I A N A B L U M R N M S N
M E T R O P O L I T A N C O M M U N I T Y C O L L E G E
SELECTIVE ANATOMY
12 cranial nerves 31 spinal nerves
Neuron transmits impulses to facilitate movement or sensation
Meninges serve as protection of the brain and spinal cord
Bronca’s area in frontal lobe forms speech
Hypothalamus regulates water, appetite, temp
CSF: surrounds and cushions brain and cord
MENTAL STATUS
Does not decline with age
Caused by drugs or lack of o2 to the brain
As we age LTM is better then STM
FUNCTIONAL ASSESSMENT
Appearance
Speech
Motor function
Family history
Ethnicity
Diet
ADLs
Right handed or left handed◦ Brain injury is more pronounced in dominant hemisphere
PHYSICAL ASSESSMENT
Orientation
LOC
Memory◦ LTM (DOB)◦ STM (mode of transportation to hospital)◦ Immediate memory (repeat 3 words after 5 minutes)
Attention◦ Serial 7 test
Language/copying◦ Follows simple commands
Cognition ◦ Current events
SENSORY ASSESSMENT
Pain and temp◦ Cotton ball vs paper clip◦ Cold vs warm
Touch◦ Pt closes eyes and you touch hand etc and then have them touch
where you touchedABNORMAL FINDINGS
Propioception-position sense below injury Contralateral- loss of sensation in opposite side of body
affected
MOTOR ASSESSMENT
Hand grasps
Foot strength
Arm drift
Coordination
Gait
Balance
Reflexes
ABNORMAL FINDINGS
tremors, weakness, paralysis, jerking muscles
RAPID ASSESSMENT
Glascow coma scale: eye opening, motor response, and verbal response◦ painful stimuli
Supraorbital pressure Sternal rub Mandibular pressure Trapezius squeeze
◦ LOC Decortication-hands/arms turned in Decerebration- hands/ arms turned out
◦ Pupil assess Response to light
The GCS is scored between 3 and 15, 3 being the worst score, and 15 the best.
It is composed of three parts: Best Eye Response, Best Verbal Response, Best Motor Response
When doing a neuro assessment it is important to watch for trends indicating a decreasing LOC.
Keep in mind that when patients have ingested alcohol,
mind altering drugs, have hypoglycemia or shock with a systolic BP <80, the GCS may be invalid.
9 to 12 is a moderate injury
8 or less is a severe brain injury.
7 or less = Coma
A client has a 5 on the Glasgow Coma Scale. When assessing this client, the nurse would expect what level of consciousness?
Sleepy or drowsy
Stuporous
Fully alert and oriented
Comatose
Comatose
A score of 7 or less indicates a comatose client. Above that are varying degrees of consciousness.
DIAGNOSTICS Blood cultures to find
infection Xray for fx, erosion,
etcPET◦ Evaluates drug metabolism◦ Detects alzheimer’s,
epilepsy, etc◦ No caffeine, alcohol, or
tobacco 24 hrs before test◦ NPO 6-12 hours prior◦ No insulin prior◦ Takes 2-3 hrs◦ No special follow-up
Angiography for circulation check◦ NPO for 4-6 hrs prior◦ Preop checklist◦ Remove jewelry and
hairpins◦ Neuro check and vs◦ Empty bladder before◦ After- monitor pulses, cap
refill, color, and vs
MRI Signed consent No food or fluid restrictions Inform of noise and offer ear
plugs Check for hx of pins,
pacemaker, metal objects
Lumbar puncture Needle in subarachnoid
space to obtain CSF for analysis
Signed consent Empty bladder Explain procedure Lie in fetal position for test Bedrest 4-8 hours post Increase fluid for 24 hours
to prevent spinal headache (3000ml)
DIAGNOSTICS CONTINUED
CT◦ No food 4-6 hrs
prior◦ Fluids okay◦ Remove jewelry
and hairpins◦ Monitor for rx to
dye◦ Monitor I/O
EEG◦ Determines brain
activity◦ Determines origin of
seizures◦ Dx of sleep disorders◦ Determines brain death◦ Explain procedure◦ No coffee , tea, or
stimulants◦ May be ordered as
speep deprived◦ Hair should not have
product on it◦ Takes about 1 hour◦ When done remove gel
with acetone
DIAGNOSTICS CONTINUED
EMG◦ Looks at muscle
activity
BRAIN SCAN◦ Locates tumors and
aneurysms◦ Explain the test◦ May need consent◦ 2 hour delay so brain
absorbs isotope◦ Must be still for
duration of test◦ 1-2 hour exam◦ f/u is to increase
fluids to promote elimination of isotope
HEAD INJURY CLASSIFICATION:
Severe Head Injury----GCS score of 8 or less
Moderate Head Injury----GCS score of 9 to 12
Mild Head Injury----GCS score of 13 to 15
(Adapted from: Advanced Trauma Life Support: Course for Physicians, American College of Surgeons, 1993).
SUPERFICIAL INJURIES
Common
Abrasions
“Goose Eggs”
Lacerations Scalp is very vascular
Xray if suspect skull fracture
SKULL FRACTURES
Categorized according to type and severity
Frequently seen in conjunction with brain injuries
Linear skull fractures
Comminuted skull fractures
Basal skull fractures
Possible associated cranial nerve deficits
OPEN SKULL FRACTURESLinear- simple clean break
Depressed - bone pressed in towards tissue
Open -lacerated scalp that creates opening to brain tissue
Comminuted - bone fragments and depresses into brain tissue
Basilar- unique fx at base of skull with CSF leaking though the ear or nose
Racoon eyes/Battles sign
CLOSED SKULL FRACTURES
Closed- blunt trauma Mild concussion-brief LOCDiffuse axonal injury- usually from MVA May go into coma
Contusion-bruising of brainSite of impact (coupe)Opposite side of impact (contrecoupe)
INTRACRANIAL HEMATOMAS
Epidural- bleed b/w skull and dura Laceration of artery or vien
Subdural-bleed below dura and arachoid layers Acute, subacute, chronic
Intracerebral-accumulation of blood in brain tissue Blunt trauma Penetrating wounds Acceleration/deceleration injuries
Increase is caused by an increase in the volume of any of the intracranial components
Drivers of increased ICP Hypoxia – triggers the vasodilatory cascade Ischemia in acute brain injury
INCREASED ICP
Normal ICP 10-15mmHg
Normal increases occur with coughing, sneezing, defecation
Leading cause of death for head trauma
As ICP increases cerebral perfusion decreases causing tissue hypoxia, decrease serum pH, and increase in CO2
ICP CONTINUED
3 types of edema Vasogenic: increase in brain tissue volume Cytotoxic: result of hypoxia Interstitial: occurs with brain swelling
HYDROCEPHALUS
abnormal increase in CSF volume
Causes: impaired reabsorption from subarachnoid hemorrhage or menengitis
BRAIN HERNIATION
Increased ICP will shift and move brain tissue downward
Central HerniationDownward shift to brainstem
S/S Cheyne stokes , pinpoint pupils, hemodynamic instability
The most life threatening is Uncal because it causes pressure on the 3rd cranial nerve
S/S Dilated, nonreactive pupils, ptosis, rapidly decreased
LOC
THE BRAIN
Headaches◦ 3 MAIN types
Migraine-genetic predisposition s/s: sensitive scalp, anorexia, photophobia, N/V Spasming of arteries at the base of the brain causing arterial
constriction, decrease cerebral blood flow, platelets clump, and serotonin released. Other ateries release prostoglandins that cause swelling and inflammation
With aura- sensation that signals onset Most are without aura Atypical- less common Tx: tylenol, migraine medicine, beta blocker, yoga,
meditation, relaxation, etc.
CLUSTER HEADACHE
one sided headache usually felt deep around eye. They come and go
Onset is associated with relaxation, napping or REM sleep
s/s: ipsilateral (one side) tearing of the eye, rhinorrhea(runny nose), ptosis(droopy), eyelid edema, facial sweating, miosis (abn. Constriction of eye). There may be bradycardia, pallor, increased temp.
Tx: same as migraine, wear sunglasses, O2 for 15 minutes, surgery
TENSION HEADACHE
Muscle and shoulder tenderness, base of skull and forehead pain. Similar s/s to migraines
Classic s/s:N/V, photophobia, phonophobia, aggravates with activity
Tx: NSAIDS,muscle relaxers
SEIZURES/EPILEPSY
Seizure: abnormal sudden, excessive, uncontrollable electrical d/c of neurons w/in the brain that may result in altered LOC, motor/sensory ability, and/or behavior.
No known cause but may be from tumors
TYPES OF SEIZURES
Tonic-Clonic: lasts 2-5 minutes Rigidity/stiffening arms/legs and Loss of ConsciousnessTonic: loss of consciousness, muscle contraction and relaxationClonic: rhythmic jerking, may bite tongue, incontinence Post seizure lethargyAbsence: more common in kids, runs in families, blank staring, loss
of consciousness (resembles daydreaming)Myoclonic: brief jerking or stiffening, symmetric or assymetric
movementAtonic (akinetic): sudden loss of muscle tone, lasts for few seconds
confusion after seizure.Partial: begin in one part of cerebral hemisphere, most often in
adults and are less responsive to medical treatmentComplex Partial: blacks out for 1-3 minutes and automatisms
present (lip smacking, picking), amnesia after seizure,temporal lobe most affected
Simple partial: remains conscious, senses unusual sensation, smell, or pain before (déjà vu). Unilateral movement during seizure, and may have tachycardia, flushing, or psychic symptoms
Idopathic: account for ½ of seizures, no known cause
CAUSES
Metabolic disorders
ETOH withdrawl
Electrolyte disturbances
Heart disease
Altered gene function
Defective genes for channels that regulate ions in/out of cell
Myoclonus clients are missing cystain B protein
Etc.
Triggers Physical activity Stress Fatigue Alcohol or caffeine Certain foods
EPILEPSY
Def: chronic disorder characterized by recurrent unprovoked seizure activity.
May be caused from abnormality in electrical neuronal activity, abnormal transmitters, or both.
Approximately 2 million people in the USA with epilepsy
can be defined as abnormal, uncontrolled electrical activity in brain cells.
Nerve cells transmit signals to and from the brain in two ways by
(1) altering the concentrations of salts (sodium, potassium, calcium) within the cell
(2) releasing chemicals called neurotransmitters (gamma aminobutyric acid). The change in salt concentration conducts the impulse from one end of the nerve cell to the other.
TYPES OF EPILEPSY
Primary or idopathicNot associated with identifiable brain lesion
Secondary Most common cause is brain lesion, tumor or trauma
Status epilepticusProlonged seizures that last greater than 5 minutes or repeated seizures over the course of thirty minutes. Causes:
Med withdrawl Infection Acute alcohol withdrawl Head trauma Cerebral edema Metabolic disturbances
CONVULSIVE STATUS EPIEPTICUS IS A NEUROLOGICAL EMERGENCY AND MUST BE TREATED PROMPTLY AND AGGRESSIVELY.
Call 911or staff emergencyGet airway established if needed by RT, Anesthesia O2 as neededEstablish large bore IV accessStart NSGet ABGsTransfer to ICU
EDUCATION OF SEIZURE/EPILEPSY PATIENTTeach importance of taking meds as prescribed
Promote balanced diet, rest, and stress reduction techniques
Instruct pt. to keep a seizure diary to identify causative factors
PHASES OF SEIZURESPreicteral phase: aura present.. The first phase involves
alterations in smell, taste, visual perception, hearing, and emotional state. This is known as an aura, which is actually a small partial seizure that is often followed by a larger event.
Ictus: The seizure.. There are two major types of seizure: partial and generalized. What happens to the person during the seizure depends on where in the brain the disruption of neural activity occurs.
Postictal state: The period in which the brain recovers from the insult it
has experienced. Drowsiness and confusion are commonly experienced during this phase. the period in which the brain recovers from the insult it has experienced
TREATMENT
Nonsurgical Antiepileptic drugs Seizure precautions
During: Protect the client from
injury Do not force anything into
mouth Turn client to side Loosen restrictive clothes Do not restrain
After Take vitals Perform neuro checks Keep on side Allow rest document
Teach family Info about disease Info about medication Support groups available Teach about alcohol
avoidance To investigate state laws
pertaining to driving and working with machinery
Care of seizure client
SURGICAL TREATMENT
Vagal nerve stimulationFor simple or complex partial seizuresStimulating device is surgically placed in the left chest wall with a lead wire on the vagus nerve
Activates with hand held magnet
CorpuscalostomyUsed for tonic-clonic seizuresFor those not candidates for other surgical procedures
Sections of the anterior and 2/3 of the corpus collosum are created to prevent neural discharges
ALZHEIMER’S DISEASE
http://www.youtube.com/watch?v=Z6lA1P2tF0o&feature=related
S/S
Aggressive
Rapid mood swings
Increased confusion at nite (sundowner’s)
Decrease interest in personal appearance
Inappropriate clothing selection
Loss of bowel/bladder
Decreased appetite
DIAGNOSIS
CBC
BMP
Folate level checked
Thyroid and liver function test
Test for syphilis
Drug tox screening (OTC)
Alcohol screening
CT
MRI
PET
EEG
TX
Meds
Prevent overstimulation
Be consistent
Reorient
Promote independence
Bowel/bladder training
Promote facial recognition
Speech therapy
Safety precautions
Minimize agitations
http://www.youtube.com/watch?v=Xv1tMioGgXI
CAUSES OF SCIPrimary Hyperflexion (moved forward excessively) Hyperextension (MVA) Axial loading (blow at top of head causes shattering) Excessive rotation (turning beyond normal range) Penetrating (knife, bullet)Secondary Neurogenic shock Vascular insult Hemorrhage Ischemia Electrolyte imbalance
TYPES
Complete: spinal cord severed and no nerve impulses below level of injury
Cervical/Thoracic
Incomplete: allow some function and movement below level of injury
Includes: Central cord syndrome Anterior cord syndrome Brown-Séquard syndrome
COMPLETE
Tetraplegia (quadriplegia): paralysis from neck down Loss of bowel and bladder control Loss of motor function Loss of reflex activity Loss of sensation Coping issues*Christopher Reeve is example of this injury*
INCOMPLETE
Central Cord Syndrome Hyperextension damage to center of spinal cord Greater loss of function in upper extremities
Anterior Cord Syndrome Cause: Direct injury to anterior spinal cord or disrupted anterior spinal
artery Paralysis, loss of pain and temperature sensation Light touch, vibration, proprioception preserved Prognosis for recovery is variable
INCOMPLETEPosterior cord lesion Damage to posterior white and gray matter Motor function intact, but loss of vibratory sense, crude touch, and
position sensation
Brown Sequard syndrome Result of penetrating injury that causes hemisection of spinal cord. Motor function , proprioception, vibration, and deep touch are lost on
the same side as injury (ipsilateral) On the other side (contralateral) the sensation of pain, temperature
and light touch are affected
ASSESSMENT
1st -respiratory status
2nd - intra-abdominal hemorrhage (hypotension, tachycardia, weak and thready pulse)
3rd assess motor function C4-5 apply downward pressure while the client shrugs C5-6 apply resistance while client pulls up arms C7 apply resistance while pt straightens flexed arms C8 check hand grasp L2-4 apply resistance while the client lifts legs from
bed L5 apply resistance while client dorsiflexes feet S1 apply resistance while client plantar flexes feet
COMPLICATIONS
Cerebral ischemia
DVT/PE
Pneumonia/Atelectasis
Vomiting and Aspiration
GI stress ulcers
Constipation
UTI
Pressure Ulcers
AUTONOMIC DYSREFLEXIA
Severe HTN, bradycardia, sever headache, nasal stuffiness, and flushing
Caused by noxious stimuli like distended bladder or constipation
Immediate interventions Place in sitting position Call doctor Loosen tight clothes Check foley tubing if present Check for impaction Check room temp Monitor BP q10-15 minutes Give nitrates or hydralazine per md order
TREATMENTImmobilize fx- C-collarProper body alignmentTraction is possibleMonitor VS q4 hr and prnNeuro checks q4 hr and prnMonitor for neurogenic shock
(hypotension and bradycardia)Prepare for possible surgeryTeach skin care, ADLs, wound
prevention techniques, bowel and bladder training, medications, and sexuality
NRSG DX FOR SCI
Ineffective tissue perfusion r/t interruption of arterial flow
Ineffective airway clearance r/t SCI
Ineffective breathing pattern r/t SCI
Impaired gas exchange r/t SCI
HUNTINGTON’S DISEASE
Formerly huntington’s chorea
Hereditary
Transmitted as an autosomal dominant trait at time of conception
25000 people in usa have
2 main symptoms are progressive mental status changes and choreiform movements (rapid, jerky) in the limbs trunk and face
No known cause
No known treatment
Only prevention is to not have children
Antipsychotics and monoamine depleting agents used to manage movement
TX: PT, OT, speech therapy, meal planning by dietician, HHC, social work to line up community resources
Osteopenia: low bone mass
Osteoclasic: bone resorption
Decreased bone mineral density
40-45% loss in women throughout lifespan
Trebecular (Spongy bone) is lost first
Then Cortical (compact bone) lost 2nd
Pathophysiology is unknown
CLASSES
Generalized:involves many structures Primary: more common
Post menopausal women Men in 60s-70s
seconday
Regional: limb involved r/t fx, injury, paralysis, joint inflammation Immobilization greater than 8-12 weeks Weightless environment (astronauts)
HEALTH PREVENTION
Teach about exercise
Teach about diet rich in calcium
Teach about bone health
Teach about safety
ASSESSMENT
Risk for falls
Head to toe assessment Inspect and palpate vertebrae
Assess pain
Assess for fallophobia
No definitive lab tests
Bone scan to check density
INTERVENTIONS
Client education is #1
Hormone replacements
Calcium supplements
Multivitamins
Diet
Fall prevention
Exercise
Pain management
Braces
OSTEOMALACIA
Softening of the bone tissue
Inadequate mineralization of osteoid (mature compact and spongy bone)
Vitamin D deficiency is a key player
Similar characteristics with osteoporosis
Rare in USA
Prevent with vitamin D, sun exposure, and diet
s/s: early stages : nonspecific Muscle weakness Bone pain Hypophosphatemia Hypocalcemia Generalized bone tenderness
Metabolic disorder of bone remodeling
Bone deposits that are weak, enlarged, and disorganized
Phases:Active increased osteoclasts cause massive bone destruction Osteoclasts are multinuclear
Mixed Inactive 2nd phase
New bone becomes sclerotic and very hard Osteoclasts return to normal amount
2nd most common bone disease
Most common sites are vertebrae, femur, skull, sternum, and pelvis
Unknown cause
ASSESSMENT 80% asymptomatic
Assess past history of fractures, skin color and temp, gout, hyperparathyroidism, lethargy, hyperuricemia
Pain that is aching, deep, poor description
Pain worsens with weight bearing and pressure
Pain most noticeable at nite or at rest
Arthritis at infected joints
Assess posture, gait, and balance
Assess vision, speech, and swallowing, hydrocephalus,
Neoplasm is the dreaded complication
DIAGNOSTICS
Serum alk phosphate Those treated for paget’s need ALP drawn 3-4 times/year
Urine hydroxyproline Shows bone collagen turnover and degree of severity
Calcium levels are normal or elevated
Increase noted in uric acid May initially be thought to be gout
X-rays, CT, MRI, bone biopsy
TREATMENT
Drugs for pain relief
Drugs to decrease bone resorption
Calcitonin (thyroid hormone)
Mithramycin (antineoplastic)
Biphosphanates
Heat therapy
Gentle massage
Exercise
PT
Diet
Osteotomy or joint replacement
Inflammatory process
Increase in vascularity and edema
Vessel becomes thrombosed once inflamed
Ischemia is next
Then necrosis
Sequestrium forms and retards bone healing
CATEGORIES
Exogenous: infection enters from outside
Endogenous: infection enters from inside
Contiguous: results from skin infection
The most common offending organism is pseudomonas aeruginosa
Staph, salmonella are aslo culprits
TREATMENT
Contact precautions
IV antibx therapy
PICC line
Use sterile techniques
Pain meds
Hyperbaric oxygen therapy
Bone grafts
Muscle flaps
Amputations
CHONDROGENIC
Osteochondroma: most common, benign, tumor…onsets in childhood, grows until skeletal maturity..has a bony stalk like appearance..may become malignant
Chondroma: lesion of mature hyaline cartilage of the hand and feet. Ribs, sternum, spine, and long bones can also be affected…can get at any age or gender
OSTEOGENIC
Osteoid osteoma: pinkish granular appearance..any bone affected..femur and tibia most affected
Osteoblastoma: affects vertebrae and long bones..large in size and lies in spongy bone..reddish granular appearance
Giant cell tumor: origin unknown..aggressive and extensive..affects women 20s-30s
ASSESSMENT/ TX
Assess pain
Palpate involved area
CT scan and MRI done for diagnosis
Interventions Meds and surgery combination Pain meds Meds taken with meals or milk
MALIGNANT BONE TUMORSPrimary: originate in bone / 2nd ary: mets to bone Primary
Osteosarcoma: most common Large lesion, pain and swelling of short duration, warm site, central portion is
sclerotic, usually mets to lung in 2 yrs then death
Ewing’s sarcoma: most malignant Pain and swelling, fever, anemia, leukocytosis, pelvis and lower extremities most
affected, any age..but kids and young adults age 20s more Pelvic yields poor prognosis
Chondrosarcoma: dull pain, swelling for long period.. pelvis and femur fore affected Destroys bone and often calcifies Affect middle age to elders and more in men
Fibrosarcoma: from fibrous tissue; most common in long bones of legs and mets to lungs
Histiocytoma is most malignant type Local tenderness, with or w/o mass palpated
BONE METS
Primary tumors are in prostate, breast, kidney, thyroid, and lung
Fractures are major problem with management Femur and acetabulum
Primarily affects those under 40
ASSESS/ DIAGNOSTICS
Assess pain, swelling, palpate for masses
Monitor vs
Assess ADLs
Assess support structures
Assess coping skills
Check ALP levels for elevation
CT scan
Stage tumor
NURSING DIAGNOSIS/TX
Pain
Anticipatory grieving
Disturbed body image
Fear
Anxiety
Tx Pain management, chemo, radiation, surgery, dressing changes, be
active listener, establish goals, safety precautions, HHC
EDUCATION
Use ergonomic work stations
Teach client to take regular breaks
s/s Parathesia in hands Weak pinch, clumsiness, weakness Hand activity worsens symtoms Swelling may occur
Tx: nsaids, surgery
DUPUYTREN’S CONTRACTURES
Slow progressive contracture
Common problem
Affects 4th or 5th digit of the hand
Trigger finger release surgery performed to fix
DISORDERS OF THE FOOT
Hammertoe: fix with surgery
Tarsal tunnel syndrome: ankle version of carpal tunnel
Plantar fasciitis: inflammation of the plantar fascia located in the arch of the foot
s/s: pain in arch, pain worsens w/ wt bearing Tx: ice, rest, stretches, strapping, nsaids, surgery
Hallux valgus: aka bunion
Most common connective tissue disorders
Most destructive to joints
RA factors looked for in lab
Assess sedrate
Assess immunoglobins
MRIs performed
EMGs are performed to measure function
ASSESSMENT/ S/S CONTINUED
Joint stiffness
Swelling
Pain
Fatigue
Weight loss
Reddened joints
Deformity of joints
Baker’s cysts may occur and cause pain
Dry eyes, dry mouth, dry vagina
Assess ADLs, coping, pain
INTERVENTIONS
Nsaids
Immunosuppressive drug
Rest
Proper positioning
Pain management
Ice
Heart parafin wax
Plasmapheresis
Fish oil tablets
LUPUS
Characteristic sign is butterfly rash
Also alopecia is common
Autoimmune disorder
May have kidney involvement
Women b/w 15-40
African american more than caucasian
GOUT
Type of arthritis
Urate crystals deposit in joints
Primary gout is most common
Inflammation is key sign
2nd ary is when too much uric acid in blood
Can affect kidneys
Meds to treat
Pain management
MARFAN SYNDROME
Inherited, dominant autosomal trait
s/s: excessively tall and lanky with elongated hands and feet, scoliosis, funnel shaped chest, glaucoma
Avg life span is 32 years
Mitral valve prolapse and AAA are common
Tx is palliative and preventitive
FIBROMYALGIA
Chronic pain syndrome
Pain is burning or gnawing
Headache and jaw pain are also common
Chest pain is common
Pain control is the key Muscle relaxers, nsaids, antidepressants
S/S
Open angle: small cresent shaped defect
Angle closure: visual fields quickly decrease, severe pain around eye, headache, n/v, halos, blurred vision
MACULAR DEGENERATION
Central vision declines
Mild blurring or distortion
More rapid to produce in smokers
MUSCULAR DISTROPHIES
9 types
Progression is slow or fast
Most common is severe X linked recessive
Diagnosis is difficult
Comfort is key
Treat symptoms