Download Case Studies Three and Four NUR 7202

Running head: CASE STUDIES THREE AND FOUR NUR 7202
Case Studies Three and Four NUR 7202
Ashley Peczkowski
Wright State University
NUR 7202
1
CASE STUDIES THREE AND FOUR NUR 7202
2
Case Study Three
1.
Which of the following processes can produce postoperative hypotension?
A. Hypovolemia secondary to blood or fluid loss
B. Sepsis
C. Adrenal insufficiency
D. Perioperative myocardial infarction
E. All of the above
Postoperative hypotension can be caused by many complications such as hypovolemia
secondary to blood or fluid loss, sepsis, adrenal insufficiency, and perioperative myocardial
infarction. Fluid or blood loss is a common cause of hypotension in abdominal surgeries. There
is much controversy over how much fluid should be given during abdominal surgery because of
the delicate fluid balance necessary to maintain tissue perfusion without developing edema. Fluid
loss during abdominal surgery is related to blood loss, gastric secretion loss, and insensible fluid
loss. Insensible fluid loss is not measureable however it can be estimated based on surgery. Each
of these parameters should be closely monitored and replaced as accurately as possible.
Normovolemia with aggressive fluid replacement has been shown to reduce postoperative
mortality, promote faster recovery, increase tissue oxygenation, and increase microcirculatory
perfusion. However, this balance is often difficult to achieve and therefore the patient will
frequently experience hypotension or fluid overload. Hypotension secondary to fluid loss without
adequate fluid resuscitation results in hypovolemia, hypoperfusion, and organ dysfunction; while
fluid overload leads to tissue edema, impaired oxygenation, and predisposition to anastomotic
breakdown. Replacement of loss fluid is further complicated by extravascular fluid shifts that
develop from increased endothelial permeability secondary to release of cytokines from
CASE STUDIES THREE AND FOUR NUR 7202
3
mechanical stress, endotoxin exposure, ischemia-reperfusion injury, and inflammation brought
on by surgical trauma. To ensure adequate fluid resuscitation, central venous oxygen saturation
and goal directed fluid therapy should be monitored. The central venous oxygenation saturation
is an early indicator of hypoxia because it monitors the delivery consumption relationship. Low
venous oxygenation saturation levels with adequate respiratory ventilation indicate a
hypovolemic state. In the goal directed fluid therapy an esophageal Doppler probe is used to
monitor blood measurements in the descending aorta. Through this a continuous measurement of
the aorta’s blood velocity and diameter is obtained and fluid adjustments are made based on
these readings (Futier et al., 2010).
Infection and abscess formation after abdominal surgery is not an uncommon
complication due to the bacteria naturally present in the bowel. Occasionally these infections can
lead to sepsis; a condition known to cause hypovolemia. Sepsis leads to immunosuppression,
lymphocyte apoptosis, and increased vascular permeability which in turn lead to multisystem
organ failure. Before the development of multisystem organ failure, sepsis results in
subcutaneous and body-cavity edema from increased vascular permeability which creates
decreased oxygenation and microvascular collapse. Increased permeability is the result of gaps
that develop in the endothelium from displacement of binding proteins. Bacteria presence in the
blood activates the inflammatory process and the release of vascular endothelial growth factors.
These growth factors cause VE-cadherin displacement to the interior of the endothelium cell.
VE-cadherin is a protein complex responsible for tightly binding endothelial cells together to
prevent gaps. When the growth factors displace the VE-cadherin then gaps, large enough for
leukocytes to enter are created and intravascular fluid develop. The increased permeability leads
to fluid shifts related to the intravascular fluid moving to extravascular space, increasing the
CASE STUDIES THREE AND FOUR NUR 7202
4
parenchymal and interstitial fluid. This excess fluid results in excessive pressure on the
microvascular system and incites collapse. Decreased intravascular fluid and vascular collapse
are the mechanisms behind the hypotension seen in sepsis (Lee & Slutsky, 2010).
Adrenal insufficiency should be considered in patients with postoperative hypotension
despite adequate fluid resuscitation. The hypothalamic-pituitary-adrenal (HPA) axis controls the
body’s response to stress caused by events, such as surgery, by increasing the body’s blood
pressure to maintain hemodynamics. This process is impaired in adrenal insufficiency, resulting
in uncontrolled hypotension. The main cause is decreased mineralocorticoids and can be
improved by administration of mineralocorticoids. This deficiency results in hypotension by
reducing vascular response to angiotensin II and norepinephrine and inhibiting production of
renin substrate, while increasing prostacyclin production. These are all common features in
shock hence why adrenal insufficiency crisis often presents itself as hypovolemic shock (Hohl &
Schwartz, 2010; Nieman, 2012).
Finally perioperative myocardial infarction (MI) is a complication that can result in
postoperative hypotension. Many patients experience perioperative MI and often have delayed
diagnosis related to surgery effects. Sometimes the only presentation of a perioperative MI is
hypotension, tachycardia, and congestive heart failure. Because of the muscle skeletal injury the
creatinine kinase-MB isoenzyme is already elevated and not a reliable test for cardiac
musculoskeletal injury. Continuous heart monitoring will commonly only show a slight STsegment depression that is either prolonged, transient, or develops postoperatively. While STsegment elevation can occur, this occurs in less than two percent. ST-segment depressions are
more difficult to detect and are often not diagnosed until 24-48 hours after injury. Historically
troponin levels have been diagnostic for MI injury; however, in postoperative high risk cardiac
CASE STUDIES THREE AND FOUR NUR 7202
5
patients it is common to see lower, but statistically significant, increases in troponin levels
without evidence of cardiac ischemia. Close monitoring of all parameters are necessary to
quickly diagnosis a perioperative MI. Tachycardia is a common finding postoperatively because
of the increased oxygen demand placed on the body from surgery. This further increases the
myocardial oxygen demand leading to cardiac ischemia and MI. Further insult to the delivery of
oxygen to the heart can be caused by hypovolemia/ hypoxia from blood loss, fluid loss, and
hypercarbia. Ischemia to the myocardial cells results in cellular dysfunction, death, and
apoptosis. This causes ventricular dyssynchrony and reduced cardiac output causing further
hypotension (Landesberg, Beattie, Mosseri, Jaffe, & Alpert, 2009).
2.
Which of the following is the most appropriate method to diagnose BMAH?
A. Cortrosyn stimulation test
B. CT scan of adrenal glands
C. CT scan of adrenal glands and Cortrosyn stimulation test
D. Random plasma cortisol level
The most appropriate method to diagnosis bilateral massive adrenal hemorrhage (BMAH)
is a cortrosyn stimulation test and a computed tomography (CT) scan of the adrenals. There are
several tests used to assess the HPA axis depending on the patient (ambulatory, hospitalized, or
critically ill). The best test for a critically ill patient is the cortrosyn stimulation test because it
does not rely on normal blood levels of blood sugar or albumin. The cortrosyn test is performed
by injecting either 1µg (low dose) or 250µg (high dose) of cosyntropin into the blood stream.
Cortisol levels are measured by blood collection at 30 minutes and then again at 60 minutes. The
level of cortisol measured indicates the amount of HPA axis stimulation response (Yong,
Coulthard, & Wrzosek, 2012). The low dose cortrosyn test is usually reserved for non-critically
CASE STUDIES THREE AND FOUR NUR 7202
ill patients due to the fact that in critically ill patients, low dose cortisol variation was shown to
increase the mortality rate and length of vasopressor therapy. The high cortrosyn test has a
sensitivity of 0.68 and a specificity of 0.65 with a baseline cortisol level less than ten µg/dL
having a high specificity of critical illness-related corticosteroid insufficiency. Low cortisol
levels less than 10µg/dL are indicative of adrenal insufficiency; whereas higher levels greater
than 34-44µg/dL indicate normal adrenal response. After the 250µg/dL cortrosyn injection, the
30 minute cortisol level should be increased by 7mcg/dL and increased by 11mcg/dL after 60
minutes, indicating a normal response. Less than this indicates adrenal insufficiency and further
testing is needed to determine primary or secondary adrenal insufficiency. High dose cortrosyn
reduced mortality by 70% compared to low dose (100% mortality) leading practitioners to
assume mortality in the ICU was related to this adrenal dysfunction. This is where steroid use in
septic shock trials originated. Based on this information, patients with less than 9µg/dL were
placed on hydrocortisone and fludrocortisone for seven days. These patients compared to the
placebo group showed a 53% decreased mortality rate compared to the 63% decrease in the
placebo group (hazard ratio, 0.67; 95% CI, 0.47-0.95; p=0.02) and were shown to have reduced
vasopressor withdrawal time. This test however also proves that a patient may have an
inadequate response when stimuli such as shock and hypoglycemia produce lower levels of
cortisol resulting in a test with higher specificity and a lower sensitivity (Moraes, Czepielewski,
Friedman, & Lucas de Borba, 2011).
Once the diagnosis of primary adrenal insufficiency has been established then a
computed tomography (CT) scanning of the adrenal glands can be performed to help with the
differential diagnosis. Enlarged or calcified adrenal glands may indicate infection, hemorrhage,
or metastatic cause (Ioachimescu & Hamrahian, 2010). Bilateral massive adrenal hemorrhage is
6
CASE STUDIES THREE AND FOUR NUR 7202
7
uncommon but carries a high mortality rate. Adrenal hemorrhage has characteristic findings on
CT of round or oval mass, periadrenal stranding, retroperitioneal hemorrhage, and crural
thickening. Attenuation will be high in acute bleeds and decreases over time with resolution at
one year. Chronic hematomas are called adrenal pseudocysts and have hypodense centers with or
without calcifications (Simon & Palese, 2009).
A random plasma cortisol level is a useful test only in critically ill patient with an
albumin level greater than 2.5g/dL. Cortisol is mostly bound to cortisol-binding globulin and
albumin so only a small amount of free cortisol is active. During critically ill times a random
cortisol level less than 15µg/dL is indicative of adrenal insufficiency. If the patient has an
albumin level less than 2.5g/dL then the patient will have subnormal serum total cortisol
concentrations. There are currently no studies with normal free cortisol ranges under different
levels of stress (Ioachimescu & Hamrahian, 2010).
3.
Which of the following can occur in patients with primary adrenal insufficiency?
A. Electrolyte abnormalities
B. Hypotension
C. Mental status changes
D. Abdominal pain
E. All of the above
Common findings in adrenal insufficiency include electrolyte abnormalities, hypotension,
mental status changes, abdominal pain, and many other findings. Signs and symptoms associated
with adrenal insufficiency are related to the glucocorticoid and mineralocorticoid deficiency and
can develop slowly over time or acutely in times of stress. Adrenal insufficiency affects almost
every aspect of the body, presenting with a wide variety of symptoms depending on the patient.
CASE STUDIES THREE AND FOUR NUR 7202
8
Musculoskeletal manifestations include muscle weakness, fatigue, and joint or muscle pain;
gastrointestinal symptoms include weight loss, anorexia, nausea, vomiting, abdominal pain,
constipation or diarrhea; integumentary symptoms are vitiligo, hyperpigmentation or
hypopigmentation, bluish/ black oral mucosa, and decreased body hair; cardiovascular symptoms
include orthostatic hypotension, dehydration, anemia, and arrhythmias; neurological
manifestation are headache, lethargy, depression, confusion, mood swings, and tremors; finally
other symptoms include decreased sweat tolerance, fever, and salt craving (Crawford & Harris,
2011). Many symptoms resonate from the inability of the body to control electrolytes.
Normocytic anemia is common because cortisol is needed to mature blood progenitor cells. This
also affects the body’s response to immune and inflammatory changes because lymphocytosis
and eosinophilia are usually present. Hypoglycemia is common because decreased
glucocorticoid secretion inhibits the body’s ability to regulate blood glucose levels and decreased
cortisol release depletes glycogen stores. Other electrolytes are altered by reduced
mineralocorticoid production. Mineralocorticoid deficiency reduces aldosterone secretion which
affects the renin-angiotensin-aldosterone system which in turn causes increased sodium and
water excretion causing hyponatremia and dehydration. Severe dehydration can lead to vascular
collapse and kidney damage creating increased serum creatinine levels. Potassium retention
related to kidney damage is also common which can lead to hyperkalemia, arrhythmias,
hypercalcemia, and reabsorption of hydrogen ions causing acidosis and decreased urea nitrogen
excretion. Finally adrenal androgen deficiency causes low serum levels of
dehydroepiandrosterone (DHEA) sulfate in patients less than 40 year of age and can cause loss
of body hair, dry skin, and loss of libido. Life threatening abnormal laboratory finding can
CASE STUDIES THREE AND FOUR NUR 7202
9
develop quickly in times of high stress of the body such as during surgery, trauma, or infection
when higher levels of cortisol and aldosterone are needed (Arlt, 2009).
Orthostatic hypotension or atrial hypotension is often present and exacerbated by acute
stress. Due to the mineralocorticoid deficiency, sodium and therefore water is excreted
abnormally without the correction response from the renin-angiotensin aldosterone system. This
leads to dehydration and hypotension. In acute crisis, this dehydration leads to shock requiring a
central line and a large volume of crystalloid fluids. Vague to severe abdominal pain is also
report in adrenal insufficiency. The exact cause of abdominal pain is multifaceted or without
cause. Dehydration and hypotension leads to decreased vascular supply to the gastrointestinal
system which can lead to necrosis and pain. Also seen in adrenal insufficiency is reduced gastric
secretion and reduce absorption of nutritional elements (Arlt, 2009; Crawford & Harris, 2011).
Lastly mental status changes such as confusion, depression, and coma can be seen in primary
adrenal insufficiency. This is caused mainly by the acidosis from increase absorption of
hydrogen ions in the renal tubules. Acidosis reduced the amount of calcium that is bound to
albumin which causes elevated serum calcium levels. Calcium is an essential electrolyte for
neurological function and neuromuscular function. Hypercalcemia alters the neuro-electrical
transmission, preventing important neurological transmission from transmitting. This is the
primary cause of the altered mental status (Skugor & Milas, 2010).
4.
Which of the following is not a risk factor for developing BMAH?
A. Postoperative state
B. Coagulopathy
C. Thromboembolic disease
D. Diabetes
CASE STUDIES THREE AND FOUR NUR 7202
10
E. Sepsis
Diabetes is the only answer that is not a risk factor for development of bilateral massive
adrenal hemorrhage (BMAH). Adrenal hemorrhage is a complication that can occur after acute
stress, neonatal stress, blunt trauma, sepsis, underlying tumor, coagulopathic state, or idiopathic
disease. The number one cause (80%) of all BMAH is blunt force trauma and account for 0.8%
to two percent of all traumas (Zhu, Van der Schaaf, Van der Valk, Bartenlink, & Nix, 2010).
Historically BMAH has been diagnosed only post mortem and had a high mortality rate due to
poor treatment. This missed diagnosis has become increasing discovered with the development
and improvement of imaging. The exact pathology of BMAH is not well understood and is
thought to be multifactorial. Trauma being the most common cause is related to disruption of the
vascular system from a blunt trauma. This can also include extracorporeal shock-wave lithotripsy
and electroconvulsive therapy. Spontaneous BMAH can occur from a variety of conditions and
predisposing factors such as adrenal neoplasia and sepsis. Sepsis causing BMAH is called
Waterhouse-Frederichson’s syndrome and is commonly associated with meningococcal
septicaemia. Other conditions and risk factors include: myoleiosarcoma, phaochromocytoma,
antiphospholopid syndrome, heparin induced thrombocytopenia, anticoagulation or non-steroidal
anti-inflammatory use, thrombocytosis, thromocytopaenia, adrenal aneurysms, steroid use,
known adrenal insufficiency, ACTH administration, pancreatitis, stress, post-surgery, burns,
pregnancy, pre-eclampsia, hypertension, and hypovolemia. Possible pathophysiology
explanation for spontaneous BMAH is that, the adrenals are very sensitive to changes in vascular
supply and are unable to tolerate vascular strain. Increased strain plus the addition of endothelial
damage from risk factors causes increased pressure resulting in vascular breakage and therefore
hemorrhage (Bharucha, Broderick, Easom, Roberts, & Moore, 2012).
CASE STUDIES THREE AND FOUR NUR 7202
11
A postoperative state is a risk factor because patients postoperatively experience the use
of anticoagulation, had intraoperative procedures performed, experience hypovolemia, may have
pre-existing adrenal insufficiency, or they may be taking or placed on chronic steroid use after.
Coagulopathy caused by the use of anticoagulation therapy, such as warfarin, or physiological
conditions such as thrombocytopenia, predisposes the adrenals to massive bleeding under any
minor increase in strain to the adrenals. Thromboembolism reduces to outflow of blood from the
adrenals while creating increased backflow pressure leading to rupture and hemorrhage. This
vascular supply sensitivity is due to the physiology of the adrenals which have three arteries
feeding the adrenals but only one vein to drain into. Finally sepsis can lead to BMAH by
multiple causes. The high level of stress on the body causing continuous release of ACTH has
been shown to lead to BMAH; although the exact cause is unknown. Sepsis also causes
coagulopathies, inflammatory state, and hypovolemia, all of which are known risk factors
(Bharucha, Broderick, Easom, Roberts, & Moore, 2012).
5.
Which of the following statements regarding the long-term management of patients
with BMAH is correct?
A. Glucocorticoid therapy is needed only during acute illnesses
B. Patients should be discharged on maintenance doses of glucocorticoids
and mineralocorticoids
C. Patients do not need mineralocorticoid therapy
D. Adrenal function is likely to recover over 4 to 6 months with no further need
for glucocorticoids
CASE STUDIES THREE AND FOUR NUR 7202
12
Patients with BMAH need to be discharged home on maintenance doses of oral
glucocorticoids and mineralocorticoids. Based on the cause of the hemorrhage, most patients do
not need exploratory surgery and can be managed non-operatively, unless in the presence of
ongoing bleeding. Typically adrenal hematomas decrease in size over time with resolution or
calcification at one year. Atrophy of the adrenals is commonly seen after hematoma resolution
indicating little to no function remaining. Non-operative therapy includes supportive care,
assessment of adrenal function, and long term oral steroid replacement. Most patients will need
to be on oral therapies for life; however, some studies have shown that some long term follow up
patients developed some recovered adrenal function (Zhu et al., 2010). Glucocorticoid therapy is
administered immediately after diagnosis with 15-25mg of hydrocortisone orally. The body
naturally produces about five to ten mg/m2 of cortisol daily, so to meet this amount 15-25mg of
oral hydrocortisone is needed. Hydrocortisone has a variable peak concentration and has shown
to produce a supraphysiological range followed by a rapid decline five to seven hours after
ingestion. Because of this it is recommended to administer hydrocortisone one of two ways:
15mg orally in the morning followed by five mg six hours later or ten mg orally in the morning
followed by five mg four hours and then again at eight hours. Neither the two dose regimen or
three dose regimen has shown to be more effective than the other, so patient preference is
encouraged. This drug is a CYP3A4 hepatic inducer so co-administered drugs need to be
thoroughly evaluated to prevent under or over treatment. Initial levels of T4 and TSH are
encouraged before staring glucocorticoid therapy because hyperthyroidism causes increased
hydrocortisone metabolism whereas hypothyroidism can precipitate an adrenal crisis. The last
dose of hydrocortisone should not be taken in the late evening as this has been shown to cause
sleep disturbances and fatigue. This effect is caused by the normal circadian rhythm of cortisol
CASE STUDIES THREE AND FOUR NUR 7202
13
production in the body where levels rise between two and four am, peak one hour after waking,
and drop to low levels in the evening. Other glucocorticoid therapy options include cortisone
acetate, prednisolone, and dexamethasone. Cortisone acetate has a more variable
pharmacokinetics with 25mg of cortisone acetate being equivalent to 15mg of hydrocortisone.
Prednisolone and dexamethasone are long acting glucocorticoid steroids and are used as a last
resort. Side effects of these long acting glucocorticoid steroids are higher cortisol levels at night
causing sleep disturbances and fatigue, decreased insulin sensitivity, and osteoporosis.
Prednisolone is indicated for insulin dependent diabetes to prevent rapid changes in glucose
control that can be seen in short acting hydrocortisone. Glucocorticoid level adjustment is based
solely on clinical judgment based on signs of over replacement (weight gain, central obesity,
stretch marks, osteoporosis, impaired glucose tolerance, and hypertension) and under
replacement (weight loss, fatigue, nausea, myalgia, and lack of energy) (Arlt, 2009). According
to the Ohio Board of Nursing (OBN) nurse practitioners may prescribe glucocorticoids (Ohio
Board of Nursing, 2013).
Next the patient should be started on mineralocorticoid therapy. Fludrocortisone is the
drug of choice for mineralocorticoid therapy because fludrocortisone binds to the
mineralocorticoid receptor and produces exclusive mineralocorticoid action. A staring dose of
100µg of oral fludrocortisone daily in the morning is recommended with the dosing range
varying from 50-250µg every 24 hours based on parameters. Replacement therapy and dose
range is guided by blood pressure, edema, serum sodium and potassium levels, and renin levels.
Signs of over replacement include hypertension, edema, and altered renin activity. Signs of under
replacement mainly include postural hypotension. Fludrocortisone dose many need to be
increased in patient with high salt diet or live in a warmer, humid climate whereas, pregnant
CASE STUDIES THREE AND FOUR NUR 7202
individuals many need lower doses in the third trimester (Arlt, 2009). According to the OBN a
nurse practitioner may only prescribe mineralocorticoids if it has been physician initiated or
consulted (Ohio Board of Nursing, 2013).
14
CASE STUDIES THREE AND FOUR NUR 7202
15
Case Study Four
A 43 year old male presents to the emergency room with complaints of weakness,
tingling, and numbness in his arms and legs. The patient states he started developing muscle
weakness and numbness about 5 hours ago in his feet that has gradually gotten worse and
progressively moved up his legs and arms. He states he first noticed it about five hours ago when
he tripped while walking up his stairs at home. His assessment reveals 3/5 strength in his
bilateral lower extremities, 4/5 strength in his bilateral upper extremities, quadriparesis that is
more distal than proximal, and hyporeflexia. He was recently seen by his primary care physician
two weeks ago for a viral illness, skin rash, and arthralgias but is has otherwise been healthy. He
does not smoke, drink, or take illegal drugs.
A lumbar puncture is performed in the emergency department that showed
abluminocytologic dissociation, white blood count of 35/µl, and increased protein level. The
patient was admitted to the critical care unit for observation. A nerve conduction study was
performed that indicated Guillain-Barre syndrome and treatment therapy was started.
1. What is the differential diagnosis for this patient? Place in order of most likely to
least likely.
2. Create a table of CFS findings in Guillain-Barre syndrome compared to normal
findings.
3. What is the most likely cause of the patients Guillain-Barre?
4. What is the most appropriate treatment?
CASE STUDIES THREE AND FOUR NUR 7202
1.
16
What is the differential diagnosis for this patient?
Some of the differential diagnosis for this patient in order of most likely to least likely is:
Guillain-Barre syndrome (GBS), hypokalemia, myasthenia gravis (MG), and acute myopathy.
There are a wide variety of differential diagnosis available for GBS including infection related
(lyme disease, diphtheria), inflammatory related (neurosarcoid), paraneoplastic, malignant,
vasculitic, metabolic (beri-beri), and of course autoimmune/ post-infectious such as GBS;
however, the previously mentioned are the most common and will be discussed. Guillain-Barre
syndrome is the most likely cause of this patient’s neuropathy because of the CSF findings and
ascending nature of the weakness. The GBS was discovered in 1916 and is to date the most
common cause of acute paralysis. About 20% of all patients with GBS remain disabled while the
mortality rate remains up to five percent despite treatment. The ration of men to women is about
1.78 with a 95% confidence interval (1.36 to 2.33) (Yuki & Hartung, 2012). The exact cause of
GBS is unknown but over 60% of patients who develop GBS have recently had an upper
respiratory infection or gastrointestinal infection. Other risk factors are recent immunization
(more commonly the influenza vaccination), recent surgery, pregnancy, or trauma. GuillainBarre syndrome occur in about one to two per 100,000 a year and is characterized during the
early stages by vague symptoms such as weakness, neck pain, back pain, or paresthesia. Atypical
symptoms are also seen in these patients such as unusual disturbances of weakness in the upper
extremities or respiratory muscles only, making diagnosis difficulty. The typical presentation of
a patient with GBS presents with symmetrical quadriplegia, possible respiratory, facial, or bulbar
muscle weakness, pyramidal weakness distribution, hyporeflexia followed by areflexia,
progression of weakness in two or more limbs from normal to nadir in less than four weeks, and
pain. A variant of GBS called Miller-Fisher syndrome (MFS) also includes ataxia,
CASE STUDIES THREE AND FOUR NUR 7202
17
opthalmoplegia, and areflexia but can also include widespread cranial nerve involvement
(Pritchard, 2010). There are several different sub-types of GBS with acute inflammatory
demyelinating polyradiculoneuropathy (AIDP) being the most common. Other sub-types include
acute motor axonal neuropathy (AMAN) which affects motor fibers only and acute motor and
sensory axonal neuropathy (AMSAN) which affects motor and sensory fibers. In North America
about 95% of all GBS are AIDP sub-type with the remaining five percent the AMAN and
AMSAN sub-types. Worldwide AMAN and AMSAN account for 30-47% of all cases. While
any infection can cause GBS Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, and
Mycoplasma are associated with a higher incident of GBS development within six weeks of
infection. Campylobacter jejuni is typically the bacterial causative agent when clusters of GBS
are seen post outbreak of bacterial enteritis (Hughes & Cornblath, 2005)
In AIDP GBS the pathology of the disease involves multifocal mononuclear cell
infiltration in the peripheral nervous system. This inflammation in the peripheral system is what
defines the classical symptoms seen in GBS. This is caused by CD4 T-cell de-regulation in
which the DC4 T-cells mediate a response against one of the myelin proteins: P2, P0, or PMP22
(Pritchard, 2010). The CD4 T-cell crosses the blood-nerve barrier and cross react with antigens
in the endoneurium, releasing cytokines which attract the macrophages. Macrophages, targeted
by antigens, enter intact myelin sheaths and denude the axons. These antigens are located on the
Schwann cells or myelin sheath causing entering macrophages to attack the Schwann cell
basement membrane through matrix metalloproteinases, toxic nitric oxide radicals, and other
mediators. The other possible cause is that the antigen binds to the Schwann cell and fixation of
the complement causing damage to the cell and vesicular dissolution of the myelin prior to
CASE STUDIES THREE AND FOUR NUR 7202
18
macrophage attacks. This cause is evident in the early course of the disease (Hughes &
Cornblath, 2005).
In AMAN sub-type the pathology of the disease is different. Macrophages attack the
nodes of Ranvier by targeting Fc-receptor-mediated binding against ganglioside antigens. T-cells
and IL-2 receptor concentrations are increased in the acute phase with oligoclonal expansion of
Vβ and Vδ gene usage caused by T-cell dis-regulation. The exact antigenic target of the disregulated T-cell is unknown but contraindicating reports highlight P0 and PMP22. Simultaneous
loss of regulatory mechanisms seen in patient with immunosuppression and is associated with
cytomegalovirus infections. The macrophages move in between the axon and the Schwann cell
axolemma which leaves the myelin sheath intact. In more severe cases the axons in the ventral
root are damaged which in turn causes the degeneration of the whole axon. More commonly the
damage blocks conduction without severing the axon giving to the classic presentation of
AMAN in which patients becomes nadir quicker but also recovers faster than in AIDP. The last
sub-type AMSAN is similar to AMAN except for in AMSAN the dorsal and ventral root is
affected (Hughes & Cornblath, 2005; Yuki & Hartung, 2012).
Hypokalemia is the most commonly missed differential diagnosis for GBS (Hughes &
Cornblath, 2005). Hypokalemia periodic paralysis (HypoPP) presents as recurrent generalized
flaccid weakness brought on by carbohydrate-induce hypokalemia or cold exposure. Progressive
weakness seen in HypoPP is caused by structural myopathy with vacuoles and T-tubular
aggregates. Hypokalemia induces weakness by depolarizing myofiber membranes through
extracellular potassium concentration reduction which diminishes currents with hyperpolarizing
effects. This hyperpolarization outward, decreases with depolarization that progresses until other
outward potassium currents stabilize the membrane at a less negative value. Stabilization at a
CASE STUDIES THREE AND FOUR NUR 7202
19
less negative value causes bimodal distribution of membrane potential leading to weakness.
HypoPP is caused by mutations in two voltage-gated cation channels in the skeletal muscle.
Mutation-induced leak of cations through aberrant pores that remain open during the resting
potential lead to permanent inward sodium current in which the Na/K-ATPase pump cannot
compensate. Mycoplasmic sodium levels exceeding normal values are myotoxic leading to
edema and vacuoles or T-tubular aggregates; again leading to muscle weakness. Hypokalemia is
differentiated from GBS by the generalized allover progressive weakness seen as compared to
GBS where weakness presents starting in two limbs and is ascending in nature.
Myasthenia gravis (MG) is the next differential diagnosis to consider in this patient.
Myasthenia gravis is an autoimmune disorder whose origin of formation is unknown. In many
patients with MG the thymus gland is found to be hyperplasia or neoplasia indicating the residual
thymus tissue many be implicated in the development of anti-skeletal muscle acetylcholine
receptors (AChR). Acquired MG presents as fatigable weakness involving specific muscle
groups that fluctuates from day to day or hour to hour. Weakness progresses with activity and
gets better with rest. The most common presentation is ocular weakness, which presents as ptosis
or diplopia. Other characteristics include dysarthria, dysphagia, dyspnea, facial weakness, or
limb fatigue or axial weakness. The incidence of MG has increased to 20 per 100,000 and affect
women three times more than men during adulthood less 40 years of age; equal prevalence
during puberty and after 40 years of age; and higher in men after 50 years of age. Neuromuscular
transmission starts with the nerve action potential entering the nerve terminal. This initiates the
release of acetylcholine causing calcium to enter the depolarized nerve terminal through voltagegated Ca2+ channels to exocytosis the vesicles around the acetylcholine. The acetylcholine then
diffuses across the synaptic cleft and binds with the AChRs in the postsynaptic membrane which
CASE STUDIES THREE AND FOUR NUR 7202
20
depolarizes the endplate potential and is terminated by acetylcholinesterase. In MG the AChRs
lose their function, lowering the endplate potential amplitude below the threshold for muscle
fiber action potential causing neuromuscular transmission failure. This AChR loss of function is
caused by antibodies (IgG1 or IgG3) binding to extracellular domain of the AChR molecule
resulting in the destruction of the muscle endplate. Acquired MG is differentiated from GBS by
the selective muscle group weakness that varies by day or hour, improves with rest, and worsens
with activity; whereas, GBS affects bilateral extremities in an ascending manner, progressively
worsens, and does not improve with rest (Meriggioli & Sanders, 2009).
Acute myopathy is more commonly seen in intensive care patients (ICU) but can be
present in non ICU patients. Acute myopathy is characterized by profound muscle weakness or
paralysis that follows an inflammatory/immune event such as mechanical ventilation, multiple
organ failure, or sepsis. Respiratory and limb fatigue are both common in myopathy and can take
weeks to months and even years to regain. Patients present with a wide variety of symptoms
related to muscle weakness including: acute respiratory failure with normal chest x-ray,
tachypneic, dyspnea, diaphoresis, tachycardia, inadequate cough, hypoxia, staccato speech, use
of accessory muscles, and symmetrical limb weakness without facial weakness. Although acute
myopathy is similar to GBS, it is less likely to be the diagnosis in this patient because he has not
suffered an ICU admission, was not mechanically intubated, or has not suffered a severe
systemic infection. Acute myopathy is difficult to differentia from GBS based on clinical
symptoms because of the similarities. Sensory nerve conduction studies and needle
electromyography in the upper and lower limbs is the best test to differentiate acute myopathy
from GBS (Latronico & Rasulo, 2010).
CASE STUDIES THREE AND FOUR NUR 7202
2.
21
Create a table of CFS findings in Guillain-Barre syndrome compared to normal
findings.
Table 1
Cerebral Spinal Fluid (CFS) Findings
CSF findings: Normal and in GBS
Normal
GBS
Pressure
70-180mm H2O
70-180mm H2O
Appearance
Clear, Colorless
Clear to Cloudy
Total Protein
20-45mg/dl
Greater than 45mg/dl
Glucose
50-80mg/dl or greater than 2/3
50-80mg/dl or greater than 2/3
of blood sugar levels
of blood sugar levels
0-5/µl
Normal to 10/µl but not
Lymphocytes
greater than 50/µl
Hemoglobin
None
none
Adapted from: (1) Griggs, R., Jozefowicz, R., & Aminoff, M. (2007). Approach to the patient
with neurologic disease. In L. Goldman & D. Ausiello (Eds.), Cecil Medicine (23rd ed.). (chpt
418). Philadelphia Pa: Sanders Elsevier. (2) Moses, S. (2013). Cerebrospinal fluid examination.
Family Practice Notebook: Neurology Book. Retrieved from:
http://www.fpnotebook.com/neuro/lab/CrbrspnlFldExmntn.htm. (3) Pritchard, J. (2010).
Guillain-Barre syndrome. Clinical Medicine, 10(4). 399-401. Retrieved from:
http://dx.doi.org/10.7861/clinmedicine.10-4-399.
CASE STUDIES THREE AND FOUR NUR 7202
3.
22
What is the most likely cause of the patients Guillain-Barre?
The most likely cause of this patient GBS is a post viral autoimmune reaction. This
patient was recently seen by his primary care physician for an upper respiratory infection, skin
rash, and arthralgia. The exact cause is unknown and the timing of occurrence of GBS post
infection varies but peaks between two to four weeks and should not exceed eight weeks.
Evidence shows that molecular mimicry might be responsible for the body’s immune response
against the axonal sheath. Many viral and bacterial bodies are composed of lipooliosaccharide’s
which closely resemble gangliosides which are ceramide attached to one or more sugars and
contain sialic acid linked to an oligosaccharide core. Gangliosides are important components in
the peripheral nerve make up. There are four gangliosides identified in the peripheral nerve:
GM1, GD1a, GT1a, and GQ1b. Studies show that some bacterial components obtained in
patients with GBS has GM1 or GD1a like lipooligosaccaride and those with Miller-Fisher
syndrome had GQ1b like lipooligosaccharides (Hughes & Cornblath, 2005). This mimicry is
what’s thought to induce the autoimmune reaction to the body previously discussed. Antibodies
to GM1 and GD1a are common in AMAN and AMSAN but not in AIDP. The difference in
AMAN preferential motor-axon injury is explained from the fact that motor and sensory nerves
have GM1 and GD1a complexes in the same quantities but there expression in different types of
tissues varies. Patients with Miller-Fisher syndrome have IgG antibodies to GQ1b and cross react
with GT1a. Those patients with pharyngeal, cervical, or respiratory muscle weakness are more
likely to have IgG antibodies to GT1a and cross react with GQ1b but less likely to have GD1a
antibodies. As the CD4 T-cells interact with the infectious membrane, it identifies the GM1,
GD1a, or GQ1b like lipooligosaccaride and produces antibodies. After the antibodies are
released macrophages are activated to kill the infectious agent. Some times after the infectious
CASE STUDIES THREE AND FOUR NUR 7202
23
agent has been fully removed dis-regulated DC4 T-cells identify the GM1, GD1a, or Gq1b
gangliosides as the previously recognized lipooligosaccaride and start reproducing antibodies.
This attracts macrophages to the Schwann and axonal sheath, where toxic components are
released as previously discussed (Hughes & Cornblath, 2005; Pritchard, 2010; Yuki & Hartung,
2012).
4.
What is the most appropriate treatment?
Treatment for GBS first starts with admission to the hospital for careful observation of
declining respiratory function and medical complications. Of the patients admitted with GBs
about five percent of them will die from complications such as sepsis, pulmonary embolism, and
unexplained cardiac arrest, which are thought to be due to dysautonomia. Admission to a critical
care unit with cardiac and pulse ox monitoring is preferred because even patients without
respiratory distress may need mechanical ventilation if they have one major or two minor
criteria. Major criteria include hypercarbia, hypoxemia, and vital capacity less than 15ml per
kilogram of ideal body weight. Minor criteria include weak cough, dysphagia, or atelectasis.
Cardiac monitoring is an important aspect of GBS treatment because GBS patients have a 20%
chance of developing autonomic dysfunction such as an arrhythmia, extreme hypertension,
hypotension, and severe bradycardia requiring pacemaker placement. Some patients with GBS
are non-ambulatory and require DVT prophylaxis such as compression stocking and heparin to
prevent pulmonary embolism (Yuki & Hartung, 2012). An acute care nurse practitioner can
prescribe heparin for DVT prophylaxis according the OBN (Ohio Board of Nursing, 2013).
Other concerns include urinary retention, constipation, and pain management therapy. Pain
management in patient with GBS is most effective with opioids during the acute phase in
combination with either gabapentin or carbamazepine (Hughes & Cornblath, 2005). Medications
CASE STUDIES THREE AND FOUR NUR 7202
24
for pain management (ultram, hydrocodone-acetaminophen, oxycodone-acetaminophen,
morphine, hydromorphone, and fentanyl are commonly prescribed), neuropathic pain
medications, laxatives, and flowmax for urinary retention are all medications a nurse practitioner
may prescribe according to the OBN (Ohio Board of Nursing, 2013).
The first treatment found to improve GBS and decrease recovery time was plasma
exchange. The most effective time to start treatment is in the first two weeks after onset and
appeared to be more effective in patients who were unable to walk. Plasma exchange works by
removing all antibodies in the blood not specific to GBS antibodies. Plasma exchange takes,
depending on the patients’ severity, two to five exchanges over two weeks (Pritchard, 2010).
Since the development of immune globulins, immune globulins have now become the treatment
of choice in non-severe cases. Immune globulins have the same initiation criteria with treatment
being started within the first two weeks of onset being the most effective. The same principle is
applied to both plasma exchange and immune globulins. In plasma exchange antibodies are
removed before extensive damage has developed and in immune globulin antibodies are
neutralized and autoantibody-mediated complement activation is inhibited preventing further
nerve damage and promoting faster improvement. Immune globulins are more convenient and
more accessible than plasma exchange. Immune globulins are given as two grams per kilogram
of body weight over five days. Not every patient responds the same and some patients have been
shown to develop a smaller rise in IgG, leading to a poorer prognosis. These patients have been
shown to benefit from a second dose of immune globulins (Hughes & Cornblath, 2005; Yuki &
Hartung, 2012). According to the OBN acute care nurse practitioners can prescribe immune
globulins when it has been physician initiated or physician consulted (Ohio Board of Nursing,
2013). Lastly corticosteroids have not been shown to significantly improve GBS process or
CASE STUDIES THREE AND FOUR NUR 7202
25
symptoms and in some studies have been shown to generate less improvement (95% confidence
interval, 0.16 more to 0.88 less improvement). Based on this Cochrane review of studies it is not
recommended to treat patients with GBS with corticosteroids (Hughes, Swan, & Van Doorn,
2010).
CASE STUDIES THREE AND FOUR NUR 7202
26
References
Arlt, W. (2009). The approach to the adult with newly diagnosed adrenal insufficiency. Journal
of Clinical Endocrinology Metabolism, 94(4), 1059-1067.
http://dx.doi.org/10.1210/jc.2009-0032
Bharucha, T., Broderick, C., Easom, N., Roberts, C., & Moore, D. (2012). Bilateral adrenal
hemorrhage presenting as epigastric and back pain. Journal of the Royal Society of
Medicine, 3(3), 15. http://dx.doi.org/10.1258/shorts.2011.011107
Crawford, A., & Harris, H. (2011). Adrenal cortex disorders: Hormones out of kilter.
Nursing2011 Critical Care, 7(4), 20-35.
http://dx.doi.org/10.1097/01.NURSE.0000419427.99685.16
Futier, E., Constantin, J., Petit, A., Chanques, G., Kwiatkowski, F., Flamein, R., ... Bazin, J.
(2010). Conservative vs restrictive individualized goal-directed fluid replacement
strategy in major abdominal surgery. JAMA Surgery, 145(12), 1193-1200. Retrieved from
http://archsurg.jamanetwork.com
Griggs, R., Jozefowicz, R., & Aminoff, M. (2007). Approach to the patient with neurologic
disease. In L. Goldman, & D. Ausiello (Eds.), Cecil Medicine (23rd ed.). Philadelphia,
Pa: Saunders Elsevier
Hohl, B., & Schwartz, S. (2010). How to manage perioperative endocrine insufficiency. The
Clinics of Anesthesiology, 28, 139-155. http://dx.doi.org/10.1016/j.anclin.2010.01.003
Hughes, R., & Cornblath, D. (2005). Guillain-Barre syndrome. The Lancet, 366, 1653-1666.
Retrieved from http://jvsmedicscorner.com/Medicine_files/Guillain%20Barre.pdf
CASE STUDIES THREE AND FOUR NUR 7202
27
Hughes, R., Swan, A., & Van Doorn, P. (2010). Corticosteroids for Guillain-Barre syndrome
(Review). Cochrane Database of Systematic Reviews, 1-42.
http://dx.doi.org/10.1002/14651858.CD001446.pub3
Ioachimescu, A., & Hamrahian, A. (2010). Endocrinology: Diseases of the adrenal gland.
Disease Management Project, (2nd ed.). Retrieved from
http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/
Landesberg, G., Beattie, S., Mosseri, M., Jaffe, A., & Alpert, J. (2009). Perioperative myocardial
infarction. Journal of the American Heart Association, 119, 2936-2944.
doi:10.1161/CIRCULATIONAHA.108.828228
Latronico, N., & Rasulo, F. (2010). Presentation and management of ICU myopathy and
neuropathy. Current Opinion in Critical Care, 16, 123-127.
http://dx.doi.org/10.1097/MCC.0b013e328336a229
Lee, W., & Slutsky, A. (2010). Sepsis and endothelial permeability. The New England Journal of
Medicine, 363(7), 689-691. Retrieved from
http://warrenleelab.com/uploads/nejmcibr1007320.pdf
Meriggioli, M., & Sanders, D. (2009). Autoimmune myasthenia gravis: Emerging clinical and
biological heterogeneity. Lancet Neurology, 8(5), 475-490.
http://dx.doi.org/10.1016/S1474-4422(09)70063-8
Moraes, R., Czepielewski, M., Friedman, G., & Lucas de Borba, E. (2011). Diagnosis of adrenal
failure in critically ill patients. Arq Bras Endocrinol Metab, 55(5), 295-302.
http://dx.doi.org/10.1590/S0004-27302011000500001
Moses, S. (2013). Cerebral spinal fluid examination. In Family Practice Notebook: Neurology
Book. Retrieved from http://www.fpnotebook.com/neuro/lab/CrbrspnlFldExmntn.htm
CASE STUDIES THREE AND FOUR NUR 7202
28
Nieman, L. (2012). Clinical manifestations of adrenal insufficiency in adults. Up to Date.
Retrieved from http://www.uptodate.com/contents/clinical-manifestations-of-adrenalinsufficiency-inadults?detectedLanguage=en&source=search_result&search=adrenal+insufficiency&sele
ctedTitle=1%7E150&provider=noProvider
Ohio Board of Nursing (2013). The formulary developed by the committee on prescriptive
governance. Ohio Board of Nursing, 1-36. Retrieved from www.nursing.ohio.gov
Pritchard, J. (2010). Guillain-Barre syndrome. Clinical Medicine, 10(4), 399-401.
http://dx.doi.org/10.7861/clinmedicine.10-4-399
Simon, D., & Palese, M. (2009). Clinical update on the management of adrenal hemorrhage.
Current Urology Reports, 10(1), 78-83. http://dx.doi.org/10.1007/s11934-009-0014-y
Skugor, M., & Milas, M. (2010). Endocrinology: Hypercalcemia. Disease Management Project
(2nd ed.). Retrieved from
http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/endocrinology/h
ypercalcemia/
Yong, S., Coulthard, P., & Wrzosek, A. (2012). Supplemental perioperative steroids for surgical
patients with adrenal insufficiency. Cochrane Database of Systemic Reviews 2012, 1-26.
http://dx.doi.org/10.1002/14651858.CD005367.pub3
Yuki, N., & Hartung, H. (2012). Guillain-Barre syndrome. The New England Journal of
Medicine, 366(24), 2294-2304. http://dx.doi.org/10.1056/NEJMra1114525
Zhu, X., Van der Schaaf, I., Van der Valk, J., Bartenlink, A., & Nix, M. (2010). Acute adrenal
insufficiency due to bilateral adrenal hemorrhage. Belgian Journal of Radiology, 93, 1920. Retrieved from http://www.rbrs.org/dbfiles/journalarticle_0830.pdf