Intern Report 6.2

Case Presentation by Dr. Mike Antoniolli

Chief Complaint: “I can’t walk” 

History of Present Illness

This is a 60-year-old male with PMH of Multiple Myeloma on chemotherapy and HTN who presents to the ED with a 3-day history of bilateral lower extremity weakness.  He reports waking up 3 days ago with difficulty walking.  He states that his legs kept “giving out”, requiring him to use a cane to ambulate.  He says that he went to an outside hospital 3 days ago and underwent spine radiographs for severe back pain and was subsequently discharged. He has a history of chronic low back pain, but says that the pain is much worse the past 3 days.  The pain is midline and is exacerbated by movement.  He denies any loss of bowel or bladder continence, but notes urinary urgency and frequency.  He also describes a shooting sensation that travelled up and down his back when he was moved abruptly by EMS.  He denies headache, fever, nausea, vomiting, or altered mentation.

Review of Systems

Constitutional:  No fever, No chills.

Eye:  No recent visual problem, No blurring, No double vision.

Ear/Nose/Mouth/Throat:  No decreased hearing, No nasal congestion, No sore throat.

Respiratory:  No shortness of breath, No cough.

Cardiovascular:  No chest pain, No palpitations.

Gastrointestinal:  No nausea, No vomiting, No diarrhea, No abdominal pain.

Hematology/Lymphatics:  No bruising or bleeding tendencies

Musculoskeletal: Low back pain

Integumentary:  No rash, No abrasions.

Neurologic: +LE Weakness. No abnormal balance, No confusion, No numbness, No tingling.

 

PMH:  Multiple myeloma (diagnosed 2 years ago) s/p chemotherapy and radiation- still on chemo, HTN (patient believes this resolved after weight loss, but he is on multiple medications and is unsure if any of these are for hypertension).

Meds:  Multiple medications, patient is unaware of names

Allergies: No known allergies

PSH: Denies

SH: No tobacco, occasional alcohol, and occasional marijuana

FH: HTN, DM, CAD

Physical Examination

Vitals:  35.0, 150/59 (89), 66, 18, 99% RA

General:  Alert and oriented, no acute distress

Eye:  Pupils are equal, round and reactive to light, Extraocular movements are intact.

HENT:  Atraumatic/Normocephalic, Oral mucosa is moist.

Neck:  Supple, Non-tender, No jugular venous distention, No lymphadenopathy.

Respiratory:  Lungs are clear to auscultation, Respirations are non-labored, Breath sounds are equal.

Cardiovascular:  Normal rate, Regular rhythm, No murmur, 2+ radial/DP/PT bilaterally

Gastrointestinal:  Soft, Non-tender, Non-distended, Normal bowel sounds.  No organomegaly or pulsatile mass.  Decreased anal sphincter tone.

Musculoskeletal:  Patient is tender to palpation along the lumbar spinous processes, not extending laterally.

Integumentary:  Warm, Dry.  No rashes, ecchymosis, or petechiae

Neurologic:  Alert and Oriented to person, place, and time. Patient is unable to raise legs off of bed or keep legs elevated after being passively raised.  Hip flexion strength is 1/5 bilaterally, Knee flexion is 3/5, Plantarflexion/dorsiflexion of the foot is 4/5 bilaterally.  Strength in the upper extremities is 5/5 throughout.  Sensation to light touch is intact throughout.  Reflexes are diminished throughout.  Cranial nerves intact, no focal deficits.

Cognition and Speech:  Speech is clear and coherent.

Review / Management

Laboratory Results:

BNP:

137/3.5| 99/32| 31/2.2<143

Ca2+: 15.7

 CBC:

6.3> 11.8/33.8<185

Imaging Studies:

CT Head: Calvarial masses consistent with multiple myeloma

CT Lumbar Spine:  Innumerable lytic lesions throughout the lumbar vertebra

Questions:

1)    Which of the following must be considered first as a cause of this patient’s weakness?

a) Neoplastic epidural spinal cord compression (cauda equina)

b) Severe hypercalcemia

c)     Amyotrophic lateral sclerosis

d)    Sensorimotor peripheral neuropathy

e)     Neuromuscular junction disease

f)     Inflammatory myopathy

2)     What is the initial treatment for patients with symptomatic hypercalcemia?

a)     Thiazide Diuretics

b)    Calcium channel blockers

c)     IV hydration with normal saline

d)    Vitamin D

e)     Magnesium

3)     What else should be administered to this patient to correct his calcium?

a)     Pamidronate and calcitonin

b)    Vasopressin and Vitamin D

c)     Sodium bicarbonate and Dextrose

d)    Hydroclorthiazide, after restoration of intravascular volume

e)     Magnesium and KCl

 

Answers 

1)    Which of the following must be considered first as a cause of this patient’s weakness?

a.     Neoplastic epidural spinal cord compression (cauda equina)

-Although hypercalcemia can cause hyporeflexia and muscle weakness, spinal cord compression is a neurosurgical emergency and requires immediate attention.  Also, hypercalcemia typically causes diffuse muscle weakness, and so would not explain the patient’s localized symptoms.

2)    What is the initial treatment for patients with symptomatic hypercalcemia?

c.     IV hydration with normal saline

– Treatment for hypercalcemia should be initiated in any symptomatic patient, or if Ca2+ is >14 milligrams/dL. Treatment consists of volume repletion with normal saline (as these patients are volume depleted secondary to impaired renal tubular reabsorption of water), decreasing Ca2+ mobilization from bone, and correction of the underlying disorder.

It’s important to note that both a bisphosphonate and calcitonin (question 4) are part of the initial treatment as well.  Also, diuretics are still commonly employed in the treatment of hypercalcemia; however, there is no direct evidence to support the common practice of using loop diuretics to enhance Ca2+ elimination. In fact, because hypercalcemia itself results in hypokalemia and hypomagnesemia from renal wasting, diuretics (which can worsen volume depletion, hypokalemia, and hypomagnesemia) are no longer recommended for malignancy-related hypercalcemia.  If loop diuretics are to be used, it is important that the patient is adequately hydrated first.  Thiazide diuretics must be avoided at all times, as these will result in increased serum calcium.

3)    What else should be administered to this patient to correct his calcium?

a.     Pamidronate and calcitonin

See question 3 explanation

Bonus Question:

What imaging study should be obtained as soon as possible?

MRI entire spinal cord

See discussion for explanation

 

Follow up:

Neurosurgery was consulted for possible acute spinal cord compression. Initially, an MRI of the lumbosacral spine was obtained that revealed diffuse lesions consistent with multiple myeloma throughout the lumbosacral vertebrae; however, it did not reveal any compressive or infiltrative lesions.  At that time, neurosurgery believed that hypercalcemia might have been the cause of the patient’s weakness. Thus, he was admitted to the MICU for correction of his hypercalcemia and frequent neuro checks.  Because he continued to have localized weakness, an MRI of his cervical and thoracic spine was obtained, which revealed extensive posterior extraosseous epidural myeloma deposits compressing the cord from C6-C7 through T11-T12.  After discussion with radiation oncology and hematology, neurosurgery decided not to operate, and the patient was started on localized radiotherapy with some improvement in his symptoms after the first treatment.

Discussion:

This patient initially presented to the ED with acute onset bilateral lower extremity weakness, low back pain, and urinary retention. On examination he was found to have lower extremity proximal muscle weakness, hyporeflexia, and loss of anal sphincter tone.  This is highly concerning for acute spinal cord compression (SCC), which is a potentially devastating condition that requires immediate consideration.  His history of multiple myeloma makes a compressive or infiltrative process even more likely.

Given this patient’s preponderance of lower extremity symptoms, his pretest probability was highest for conditions involving his lumbar spine, such as cauda equina syndrome from metastatic disease or pathologic fracture. An MRI of the lumbosacral spine was obtained initially out of concern for cauda equina syndrome. However, when investigating the possibility of epidural compression secondary to a neoplasm, an MRI of the entire spine is warranted, for at least three reasons:

1) Physical examination may falsely localize the spinal lesions1

2) Nearly 20% of patients with tumor spread to the spine will have disease at multiple levels2

3) The presence of additional tumors may alter patient management1

This patient’s physical exam findings and Cervical/Thoracic MRI results support this assertion, and given the grave consequences of delaying treatment for SCC, it is clear that this imaging should have been obtained initially.

To avoid unnecessary scans, it is helpful to consider epidural compression syndrome as a collective term that includes SCC, cauda equina syndrome, and conus medullaris syndrome.  These three syndromes have similar presentations and require similar initial evaluation and management.

Detecting patients with early signs and symptoms of compression can be difficult, as the initial differential diagnosis for conditions that cause weakness, sensory changes, or autonomic dysfunction is broad.  The history usually includes back pain, saddle anesthesia, bowel or bladder incontinence with or without urinary retention, and multiple level radiculopathies often involving both legs.1,2  Diagnosis can sometimes be hindered by atypical presentations, with equivocal neurologic compromise and only mild to moderate pain.  Important red flags include history of malignancy and rapid progression of neurologic symptoms, especially bilateral symptoms.

Findings on physical examination will vary depending on the level and degree of compression, as well as the region of the spinal cord/cauda equina that is compressed. Lower extremity weakness/stiffness, gait difficulty, parasthesias or sensory deficits, and +SLR test are common exam findings in epidural compression.  Depending on the level of compression, reflexes may be exaggerated or diminished (would expect diminished reflexes in cauda equina syndrome because the nerve roots are being compressed; however overlap with the conus may occur resulting in mixed findings).  The same is true for more proximal compressions of the spinal cord with nerve root involvement. Saddle anesthesia is the most common sensory deficit with a sensitivity of 75%, and 60-80% of cases will have decreased anal sphincter tone on rectal examination.  Urinary retention with or without overflow incontinence is the most consistent finding, and is highly sensitive (90%) and specific (95%) for cauda equina syndrome.1,2

There are many possible causes of epidural compression including, but not limited to, malignancy, epidural abscess, disk herniation, and spinal canal hemorrhage (transverse myelitis and cord infarction are intrinsic cord lesions that can mimic epidural compression).  Of particular interest to this case is epidural compression secondary to a neoplasm, which constitutes an oncologic emergency requiring urgent treatment to prevent deterioration and preserve function.  When suspected, the initial management involves administration of dexamethasone 10 mg IV, before any confirmatory testing, to potentially minimize edema, progression of compression, and thus resultant neurologic damage.  As mentioned above, MRI of the entire spine should be obtained.  Often times, faster imaging modalities are utilized first. However, some authorities recommend preceding directly to MRI in patients with known neoplasm.2  These patients will often require surgical intervention, radiation therapy, and high dose steroids.  Oncology, neurosurgery, and interventional radiology are all appropriate consultations for this patient.1,2

Restoration of neurologic function in epidural compression from a neoplasm is dependent on symptoms at presentations.  Of patients who are paraplegic at the time of treatment, only 5% regain the ability to walk.  Patients who are not paraplegic and are able to walk with assistance have a 50% chance of ambulating again.  Of those who are able to walk without assistance, 60% remain ambulatory.  Of patients who require catheterization for urinary retention at the time of treatment, 82% require a catheter after treatment. This is particularly important given the large number of patients who present with urinary retention.1,2

 

References

1.  Tintinalli JE, Stapczynski JS. Tintinalli’s emergency medicine : a comprehensive study guide. New York: McGraw-Hill; 2011.

2. Marx JA, Hockberger RS, Walls RM, Adams J. Rosen’s emergency medicine : concepts and clinical practice. St. Louis: Mosby; 2002.

Intern Report 6.1

Case Presentation by Dr. Eric Malone

 

HISTORY OF PRESENT ILLNESS:

This previously healthy 41-year-old patient comes to the emergency department complaining primarily of the acute onset of a severe headache 48 hours prior to presentation.  He states that he was sitting at home when the headache began, and denies any associated physical exertion.  He describes the headache as a severe, throbbing pressure unilateral, left-sided, temporal, and without radiation.  He has an associated feeling of dizziness, but no loss of vision, and no weakness or parasthesias in his extremities. He has had headaches in the past, but states that this headache is different in quality and is more severe.  Associated with the headache are 3 episodes of nonbilious, nonbloody vomiting over the past 48 hours. He has had persistent associated nausea. He felt well enough to try to go to the casino earlier the day of presentation, but his headache returned, and became more severe, so he went home.

No fever, URI, CP, SOB or other symptoms.

He is accompanied by family members, who state that he is usually not one to complain about pain. They are concerned because he does not seem to be acting like himself. They describe him as sleepy, and seeming “out of it.”

Review of Systems: Negative except as per HPI

Past Medical History, Surgical History, Medications: None

Allergies: None

Social history: Occasional social alcohol use, occasional marijuana use. Denies tobacco and intravenous drug use.

Family History: Notable for a grandmother who passed away from an aneurysm.

Physical Examination:

Vital Signs: Blood pressure was 158/98, pulse was 66, respirations were 18, temperature was 36.0 by mouth and oxygen saturations were 100% on room air

General: Awake, alert, lying in bed in moderate distress.  The patient keeps his eyes closed and is complaining of pain. He is drowsy but easily arousable. He responds to questions, but slowly.. Well-developed, well-nourished.  Appears stated age.

HEENT: Normocephalic, atraumatic.  Pupils equal round and reactive to light, photophobia, but no papilledema.  Extraocular movements are intact. Neck is supple, without lymphadenopathy. Mucous membranes are moist. Posterior oropharynx is nonerythematous. No meningismus.

Cardiovascular: Heart is regular rate and rhythm.  There are no murmurs, rubs, or gallops.  Distal pulses are palpable in all 4 extremities.  There is no evidence of peripheral edema.

Respiratory: Respirations are nonlabored, CTAB

Gastrointestinal: Abdomen is soft, nontender, and nondistended.

Musculoskeletal: The patient has full range of motion in all four extremities. There is no swelling.

Skin: No rash. Skin is clean, dry, and intact.

Neurologic: Alert, and oriented x3.  Pupils are equal round and reactive to light.  Extraocular eye movements are intact.  Sensation is intact in the face.  Jaw clench strength is intact.  Smile is symmetric.  The palate and uvula elevate symmetrically.  Neck rotation and shoulder shrug strength is intact.  The tongue protrudes in midline.  Sensation is intact to light touch in all 4 extremities.  Strength is 5/5 in all 4 extremities.

IV access was established and the patient was provided 4mg morphine IV, 10mg metoclopramide, and a 1L 0.9% NaCl bolus.

APTT, PT, INR within normal limits

CT head without contrast was performed and was negative for any acute intracranial process.

Following an explanation of the risks and benefits of the procedure,lumbar puncture was performed. CSF was obtained on the first attempt without difficulty. The CSF returned – (See picture below). Glucose and Protein were within normal limits. Cell count performed on Tube #2 demonstrated a RBC count of 6020. RBC count in Tube #4 was 3440.

Questions:

1) Does the above picture CSF represent a traumatic spinal tap or significant finding?

a) Traumatic Tap

b) Significant CSF finding

c) Normal Tap

d) This is urine and not CSF

2) What percentage of patients presenting with Subarachnoid hemorrhage demonstrate no evidence of blood on initial head CT?

a) 10%

b) 20%

c) 50%

d) Percent will vary depending on length of time from onset of bleeding

3) Which of the following is the most sensitive for the presence of SAH on CSF analysis?

a) Xanthochromia

b) Visual inspection of CSF

c) A persistent RBC count from Tube #1 to Tube #4.

d) Opening pressure

4) How long must RBCs remain in the CSF before Xanthochromia can be detected?

a)  2 hours

b) 6 hours

c) 12 hours

d) 24 hours

 

Case Discussion & Answers

1) b

2) d

3) a

4) c

Given the history of severe headache, and the provided lumbar puncture results, this patient went on to have CT-angiography performed. This demonstrated a 6mm basilar artery tip aneurysm. The patient then underwent angiography which demonstrated a small, wide based, basilar artery aneurysm as well as a small posterior communicating artery aneurysm that was embolized uneventfully.

The patient was observed in the NICU for 24 hours after embolization and was then observed on the general neurology floor for a further 24 hours before being discharged home on 81mg aspirin and 5mg amlodipine.

Discusssion:

This patient originally presented with the acute onset of severe headache that was different in quality than previous headaches. On initial examination, he was drowsy and photophobic, but demonstrated no neurologic deficits. His family history was remarkable for a family member that had passed away from an aneurysmal bleed. Given the character of the headache, duration of symptoms, and the patient’s seemingly depressed mental status, the initial concern was for subarachnoid hemorrhage. Subarachnoid hemorrhage is a rare, life threatening cause of headache in the emergency department. Most (~75%) arise secondary to aneurysm rupture. The incidence of SAH in patients presenting to the ED has been reported as 1-4%.  Risk factors associated with development of SAH include hypertension, smoking, excessive alcohol consumption, and the use of sympathomimetic drugs. Incidence increases with age.

The classic clinical presentation of a new onset SAH would be the sudden onset of a severe headache that reaches maximal intensity within minutes—the so-called “thunderclap” headache. There is additional association with exertional activities and those which require a Valsalva maneuver. Of the patients who present to the ED with a thunderclap headache, between 11 and 25% will have SAH. Any headache that is different in quality from the patient’s usual headache should raise concern for SAH on the differential. All patients with SAH do not present with headache: alternate initial presentations would include altered mental status, photophobia, or persistent nausea/vomiting

It is well established that a patient presenting with severe, acute onset headache should undergo evaluation with Head CT. Head CT is highly sensitive for SAH within hours after presentation. If performed within the first 6 hours of symptom onset, CT is 100% sensitive for SAH. CSF is continually circulated with resulting breakdown and dilution of blood, so this sensitivity decreases to 98% at 12 hours from onset and further decreases to 93% at 24 hours. Thus, any patient presenting after 6 hours from the onset of headache will need lumbar puncture performed to rule out the presence of blood in the CSF. CT is preferred to MRI given the increased sensitivity of CT to detect acute blood.

There has been some suggestion that in a select group of neurologically intact patients with normal vital signs and no evidence of elevated intracranial pressure that an LP first strategy may consume fewer resources and, given clinicians tendency to eschew LP when initial Head CT returns normal, result in fewer missed diagnoses. However, no clinical trials have yet assessed this method.

Xanthochromia

There remains question as to the best method of distinguishing a traumatic lumbar puncture from CSF containing blood from a SAH. Analysis for xanthochromia—the yellowish discoloration of CSF that occurs with increasing catabolic products of hemoglobin—is one such method. By far the most common method of assessing for xanthochromia involves the comparison of a centrifuged tube of CSF against a similar volume of water, both held against a white background. Approximately 99% if US hospital laboratories use this method. The more accurate method involves the spectrophotometric measurement of centrifuged CSF. Particularly when spectrophotometric analysis is to be performed, the tubes of CSF should be taken immediately to the lab, and kept in darkness so as to not accelerate the process of bilirubin degradation.

A second method of distinguishing traumatic lumbar puncture from true SAH involves the comparison of RBC counts across tubes of CSF. In a traumatic tap, the RBC count will decrease across tubes and the RBC in the last tube will approach zero. However, it is essential to note that a simple decrease in RBC count does not imply traumatic tap, nor does the use of an arbitrary cutoff of 25% decreased from Tube #1 to Tube #4.

Initial ED treatment and management involves resuscitation and stabilization. Patients with a significantly depressed level of consciousness or those at risk for respiratory center compromise should be intubated and sedated. It is essential that blood pressure be carefully monitored with most suggesting a target systolic blood pressure of 160 mm Hg, maintained with a titratable IV infusion if necessary. Nimodipine is typically initiated soon after the diagnosis of SAH to decrease the risk of vasospam. Pain should be aggressively treated with opioids as needed for persistent headache.

Early consultation with a Neurosurgeon or Neuro-internventionalist is crucial as a means to ultimately treat a bleeding aneurysm. Treatments of choice would involve clipping or coil embolization. Final prognosis depends primarily on initial presentation. The Hunt and Hess scale provides a method for grading patients based on initial clinical picture. Those patients initially presenting with low Hunt and Hess scores (I and II) tend to do well. Those with high grades (IV and V) have a poor prognosis.

Clinical Pearl:

Any patient presenting after 6 hours from the onset of headache will need lumbar puncture performed to rule out the presence of blood in the CSF. CT is preferred to MRI given the increased sensitivity of CT to detect acute blood.

References:

  1. Rosen’s Emergency Medicine, seventh edition, 2010, pages  1360-1361, Marx
  2. Tintinalli’s Emergency Medicine, 7th Edition, 2011, pages  1118-1120, Tintinalli
  3. Aneurysmal Subarachnoid Hemorrhage: An Update for Emergency Physicians. Journal of Emergency Medicine. Journal of Emergency Medicine. Vol 34, 2008. Edlow et al.

Rosh Review A1.4

A 22-year-old man presents to the ED with clonus of his neck to the right.  Which of the following drugs is he most likely to be taking?

A. Benztropine

B. Cocaine

C. Haloperidol

D. Ziprasidone

This man is having a dystonic reaction, which is a common side effect of haloperidol and other typical antipsychotic drugs.  Haloperidol is a high potency antipsychotic that blocks dopamine-2 receptors at the basal ganglia which can lead to acute dystonia shortly after drug initiation (50% occur within 48 hours, 90% within 5 days) and sustained movement disorders (parkinsonism and tardive dyskinesia) with prolonged use. Symptoms of acute dystonia include intermittent, involuntary and uncoordinated hyperkinetic movements most often affecting the tongue, face, neck, trunk or extremities.  Treatment is with IM or IV benztropine or diphenhydramine and recovery is rapid after medication administration.

Benztropine (A) has both anticholinergic and antihistamine activity and is commonly used for the treatment of movement disorders. Although cocaine (B) does not typically cause dystonia, it can increase the risk for dystonic reactions and is associated with choreoathetoid movements that are referred to as crack-dancing.  Ziprasidone (D) is an atypical antipsychotic drug that is much less likely to cause dystonia and other extrapyramidal symptoms.

Rosh Review Q1.4

A 22-year-old man presents to the ED with clonus of his neck to the right.  Which of the following drugs is he most likely to be taking?

A. Benztropine

B. Cocaine

C. Haloperidol

D. Ziprasidone

Answer will post on Thursday morning

Rosh Review A1.3

A 32-year-old woman who is at 20 weeks gestational age presents to the ED after a seizure. Her vital signs are BP 115/70, HR 105, RR 16, T 98.7°F, and pulse oximetry 98% on room air. On exam, you note some confusion, but otherwise there are no focal deficits. Lab results reveal a hemoglobin of 7 g/dL and platelets of 12,000/microliter. A peripheral blood smear reveals schistocytes. Which of the following is the most appropriate treatment for her condition?

A. Delivery of fetus

B. Magnesium sulfate

C. Plasmapharesis

D. Platelet transfusion

The patient has thrombotic thrombocytopenic purpura (TTP). The classic pentad of TTP includes CNS abnormalities, renal pathologyfevermicroangiopathic hemolytic anemia, and thrombocytopenia. However, diagnostic criteria have recently been simplified to include all adults with microangiopathic or microvascular hemolytic anemia and thrombocytopenia with no other explanation for these findings. TTP shares many clinical and laboratory features as HELLP syndrome. HELLP syndrome is less common before 24 weeks gestation and the derangements in hemoglobin and platelet levels are more severe in TTP. The treatment mainstay of treatment for TTP is plasmapheresis (plasma exchange), which can achieve remission of disease in 80% of patients. If plasmapheresis cannot be immediately performed, fresh frozen plasma (FFP) should be administered, until pheresis can be performed.

The patient’s fetus is nonviable at 20 weeks gestation (A) and cannot be delivered. If the fetus is viable, delivery is an option. However, the patient is at high risk for bleeding complications and medical therapy and plasmapheresis is the first line of treatment. Magnesium sulfate (B) is used in the treatment of eclampsia. Although this patient had a seizure, her blood pressure is normal and her lab results are more consistent with TTP than eclampsia. While platelet levels are low in TTP,platelet transfusion (D) is reserved only for patients with life-threatening bleeding. Any administration of platelets will result in destruction from platelet aggregation in the microvascular circulation.

Questions are taken from RoshReview.com