Neurology
A 37-year-old man is evaluated in the emergency department 12 hours after sudden onset of a global, severe headache with associated neck stiffness. The patient has a 5-year history of migraine treated with sumatriptan. He says that his current headache feels different from previous headaches and did not respond to sumatriptan.
On physical examination, temperature is normal, blood pressure is 148/72 mm Hg, heart rate 86/min and regular, respiration rate is 12/min, and oxygen saturation is 96% with the patient breathing ambient air. The patient has discomfort and appears restless. Funduscopic examination is normal. The left pupil is 2 millimeters larger than the right and poorly reactive to light. Neck stiffness is noted with passive movement.
A CT scan of the head is normal.
Which of the following is the most appropriate next diagnostic test?
A Lumbar puncture
B Magnetic resonance angiography of the neck
C MRI of the brain
D No further testing is necessary
A- Lumbar Puncture
In a patient with a thunderclap headache and normal findings on a CT scan of the head, lumbar puncture should be performed next.
Magnetic resonance angiography is helpful in evaluating a carotid artery dissection, but the patient's examination findings are not consistent with this diagnosis. . This patient has a global headache and an enlarged pupil consistent a compressive process involving the oculomotor nerve (cranial nerve III) and not Horner syndrome.
MRI is neither sensitive nor specific enough to definitively diagnose SAH. Furthermore, MRI is time consuming and not always available.
Given the patient's abnormal neurologic findings, performing no further testing to find the cause of his symptoms is inappropriate.
A 53-year-old man is evaluated for fatigue and arthralgia for the past 2 years. He indicates multiple joints in the hands and lower extremities are involved, but the pain is not exacerbated by joint use and is partially relieved with ibuprofen. He typically consumes two beers per day. His only medication is ibuprofen.
On physical examination, vital signs are normal. The right second and third metacarpophalangeal (MCP) joints are swollen. The examination is otherwise unremarkable.
Hand radiographs show hook-like osteophytes in the MCP joints.
Which of the following is the most appropriate test to perform next?
A- HFE genotyping
B- Liver biopsy
C- Rheumatoid factor and anti–cyclic citrullinated peptide antibodies
D- Transferrin saturation and serum ferritin level
D- Transferrin saturation and serum ferritin level
Atypical presentations of rheumatoid arthritis and osteoarthritis, particularly with hook-like osteophytes of the second and third metacarpophalangeal joints, suggest the possibility of hemochromatosis; evaluating the transferrin saturation and serum ferritin level should be considered.
Patients with hereditary hemochromatosis presented with signs of cirrhosis, diabetes mellitus, and skin pigmentation. Because of earlier recognition, fatigue, arthralgias, and loss of libido are more common presenting symptoms. Patients with hemochromatosis have rheumatic symptoms that typically involve the small joints of the hand, especially the second and third metacarpophalangeal (MCP) joints associated with characteristic radiographic findings, including hook-like osteophytes. Guidelines support initial testing with transferrin saturation to diagnosis iron overload. A transferrin saturation greater than 45% is often chosen as the cutoff to warrant additional testing, although this threshold has lower specificity and positive predictive value compared with higher cutoff values. A normal serum ferritin level and transferrin saturation less than 45% have a negative predictive value of 97% to exclude iron overload.
A 32-year-old woman is evaluated for a 2-year history of dry eyes and dry mouth. She wakes up in the morning with a feeling like something is in her eyes. She cannot eat dry foods without consuming large amounts of water. She has noticed a change in the shape of her face; it has gotten more round. She previously saw an ophthalmologist who found abnormal ocular surface staining consistent with dry eyes. She uses artificial tears as needed.
On physical examination, vital signs are normal. There is no pooled saliva under the tongue. Bilateral parotid and lacrimal enlargement is present. The remainder of the examination is normal.
Antinuclear antibodies, anti-Ro/SSA antibodies, and rheumatoid factor are negative. Screening for HIV and hepatitis B and C is negative. Serum IgG levels are normal.
Chest radiograph is normal.
Which of the following is the most appropriate diagnostic test to perform next?
A CT of the chest
B Lip biopsy
C Parotid biopsy
D Schirmer test
E Sialography
Correct Answer: B
Educational Objective: Diagnose Sjögren syndrome.
Key Point
A lip biopsy should be considered in a patient with sicca and suspected Sjögren syndrome when initial serologic evaluation is uninformative.
A 35-year-old man is evaluated for a 3-year history of epilepsy. Seizures typically occur twice monthly, last 2 minutes, and are characterized by staring, lip smacking, and confusion; approximately once every 6 months, the patient experiences a whole-body convulsion marked by incontinence and prolonged confusion for several hours. Treatment with oxcarbazepine and lamotrigine, although initially reducing seizure frequency, has been largely ineffective. He no longer drives or works because of the seizures. He also has migraines, which are well controlled by sumatriptan.
On physical examination, vital signs are normal. All other physical examination findings, including those from a neurologic examination, are unremarkable.
Results of routine outpatient electroencephalography (EEG) are normal. An MRI of the brain shows right hippocampal atrophy.
Which of the following is the most appropriate next step in management?
A- Levetiracetam
B-Topiramate
C-Vagus nerve stimulation
D-Video EEG monitoring
Video electroencephalography is first step in determining candidacy for epilepsy surgery in patients with medically intractable epilepsy
This patient is having ongoing focal seizures with altered awareness (formerly known as complex partial seizures) because of temporal lobe epilepsy despite taking two antiepileptic drugs and should be referred to an epilepsy center for monitoring by video electroencephalography (EEG). Because his epilepsy is medically intractable, he may be a candidate for epilepsy surgery.
Although levetiracetam and topiramate are reasonable options for treating this seizure type, and topiramate has the added benefit of migraine prophylaxis, the chance of seizure freedom from an additional drug is only approximately 5% to 10%. Additionally, this patient's migraines are already well controlled, so a prophylactic agent is not required.
Use of a vagus nerve stimulator is a palliative measure, is unlikely to result in freedom from seizures, and should be offered only if resection is not an option.
A 45-year-old man is evaluated in the hospital for worsening pain and progressive swelling of the left lower extremity that began abruptly approximately 12 hours ago. Medical history is otherwise noncontributory, and he takes no other medications.
On physical examination, pulse rate is 112/min; other vital signs are normal. He has erythema and marked edema of the entire left lower extremity. The left foot appears cyanotic, with decreased sensation, absent arterial pulses, and delayed capillary refill. The physical examination is otherwise unremarkable.
Doppler ultrasonography of the left lower extremity shows extensive ileofemoral deep venous thrombosis.
Which of the following is the most appropriate management?
A-Alteplase
B-Argatroban
C-Rivaroxaban
D-Unfractionated heparin
E-Unfractionated heparin plus inferior vena cava filter insertion
A- Alteplase
Thrombolysis should be considered as the initial therapy for patients with iliofemoral deep venous thrombosis with acute limb ischemia
A 49-year-old man is evaluated a 3-month history of right knee swelling without significant pain. He does a lot of physical activity as a park ranger in Michigan but does not recall trauma. He likes to walk for exercise and does not have significant pain when he walks. He has only minimal discomfort in the knee and stiffness when he squats. He reports no swelling or pain in other joints and no fever, rash, or other symptoms. He takes no medications.
On physical examination, vital signs are normal. Examination of the right knee shows a large effusion without erythema, but with minimal warmth and tenderness; full range of active motion is noted. The remainder of the musculoskeletal and physical examination is normal.
Knee radiographs confirm a large joint effusion in the right knee; no other abnormalities are seen.
Which of the following tests is most likely to confirm the diagnosis?
A HLA-B27 haplotype testing
B MRI of the right knee
C Serologic testing for Borrelia burgdorferi
D Synovial fluid analysis for crystals
Correct Answer: C
Educational Objective: Diagnose Lyme arthritis.
Key Point
Chronic monoarticular arthritis with a large effusion and stiffness but minimal pain is characteristic of Lyme arthritis; diagnosis is made by serologic testing (enzyme-linked immunosorbent assay followed, if positive, by Western blot).
A 75-year-old man is hospitalized for treatment of extreme agitation and delirium after developing a urinary tract infection (UTI). He has a 1-year history of dementia with Lewy bodies. According to his wife, the patient has nighttime visual hallucinations 4 or 5 times per week but is rarely bothered by them. Home medications are simvastatin and aspirin.
On physical examination, blood pressure is 150/92 mm Hg and pulse rate is 98/min; other vital signs are normal. The patient is agitated and disoriented and appears to be having visual hallucinations. Although his agitation steadily increases, he does not become aggressive. His UTI is being treated appropriately. No other clear sources of the delirium are present.
Environmental interventions are instituted to help abate his symptoms
Which of the following medications is most likely to be effective in treating his acute agitation?
A. Alprazolam
B. Diphenhydramine
C. Donepezil
D. Haloperidol
Correct Answer: C
Educational Objective: Treat a patient who has dementia with Lewy bodies and acute agitation with donepezil.
Haloperidol is absolutely contraindicated in dementia with Lewy bodies; donepezil is a safer alternative and may improve the behavioral and cognitive symptoms associated with dementia.
Benzodiazepines (such as alprazolam) and diphenhydramine have a strong potential for worsening delirium symptoms in an older population and thus should be avoided in this patient.
Haloperidol is absolutely contraindicated in DLB because of the risk of significant worsening of the dementia syndrome. In addition, the patient is not aggressive at this point and is unlikely to harm himself or others, so antipsychotic agents are unnecessary, as are medications with a strong sedating effect (such as diphenhydramine)
A 73-year-old man undergoes follow-up evaluation after autologous hematopoietic stem cell transplantation (HSCT) 3 weeks ago for multiple myeloma. He had induction chemotherapy 1 year ago. He is doing well, without fever, chills, cough, or localizing symptoms of infection. Medications are acyclovir and omeprazole.
On physical examination, vital signs are normal; BMI is 20. He weighs 2.5 kg (6 lb) less than he did 2 months ago before HSCT. Alopecia is noted.
In the next 30 days, this patient is at increased risk for which of the following treatment-related complications?
In the next 30 days, this patient is at increased risk for which of the following treatment-related complications?
A Acute graft-versus-host disease
B Infection
C Lymphoma
D Myelodysplastic syndrome
B- Infection
Identify an increased risk of infection after autologous hematopoietic stem cell transplantation.
Key Point
Immediately following hematopoietic stem cell transplantation, patients are at increased risk of infection because of compromised function of lymphocytes and neutrophils.
A 32-year-old woman is evaluated for a 15-year history of low back pain. The pain is worse with rest, improves with movement, and can awaken her during the night. Family history is notable for three paternal uncles with back problems. She takes naproxen twice daily with some relief.
On physical examination, vital signs are normal. Joint examination does not reveal any warmth, erythema, or swelling. Tenderness over the sacroiliac joints bilaterally and reduction in the range of motion of the lumbar spine are noted.
Laboratory studies are notable for an erythrocyte sedimentation rate of 27 mm/h.
A plain anteroposterior radiograph of the pelvis shows fusion of the sacroiliac joints.
Which of the following is the most appropriate diagnostic test to perform next?
A- ANCA
B- Anti–cyclic citrullinated peptide antibodies
C- Antinuclear antibodies
D-HLA-B27 antigen
E-No additional testing
Correct Answer: E
Educational Objective: Diagnose ankylosing spondylitis.
Key Point
A diagnosis of ankylosing spondylitis can be made in a patient younger than age 45 years with symptoms of inflammatory back pain for 3 months or more and bilateral sacroiliitis on imaging
A 21-year-old man is evaluated in the emergency department for persistent convulsive status epilepticus that began 30 minutes before his arrival. An airway has been secured, and he has received intravenous glucose, thiamine, and two doses of intravenous lorazepam. After receiving the second dose of lorazepam, he continues shaking for another 5 minutes. Medications are levetiracetam and acetaminophen; the patient is allergic to phenytoin.
On physical examination, temperature is normal, blood pressure is 155/89 mm Hg, pulse rate is 108/min, respiration rate is 16/min, and oxygen saturation with the patient breathing 6 L of oxygen via a nasal cannula is 97%. The pupils are reactive but he remains comatose.
Results of a urine drug screen are negative.
Which of the following is the most appropriate next step in management?
A- Brain MRI
B- Electroencephalography
C- Fosphenytoin
D- Lacosamide
E- Valproate
Valproate
Educational Objective: Treat convulsive status epilepticus in a patient allergic to phenytoin with Valproate
Key Point
According to current guidelines, valproic acid is an appropriate second-line therapy for convulsive status epilepticus for patients allergic to phenytoin.
Initial management of CSE requires rapidly assessing airways, breathing, and circulation; checking the blood glucose level; and administering thiamine with glucose, if needed. These steps should be performed simultaneously with initiation of drug treatment
A 68-year-old man is evaluated in the emergency department for fatigue and exertional dyspnea. He has a 5-year history of chronic lymphocytic leukemia, which has not required therapy. He takes no medications.
On physical examination, temperature is 37 °C (98.6 °F), blood pressure is 123/82 mm Hg, pulse rate is 108/min, and respiration rate is 18/min. Oxygen saturation is 95% breathing ambient air. Cervical, axillary, and inguinal lymphadenopathy and splenomegaly are present.
Hemoglobin 5 g/dL (50 g/L)
Leukocyte count 35,000/µL (35 × 109/L) with 85% lymphocytes, 15% neutrophils
Platelet count 180,000/µL (180 × 109/L)
Reticulocyte count 10% of erythrocytes
A direct antiglobulin test result is positive for IgG and C3. The patient is group A-positive and crossmatch incompatible with 5 units of group A-positive blood.
Glucocorticoid therapy is started.
Which of the following is the most appropriate management?
A Avoid transfusion until a compatible unit can be found
B Begin plasma exchange therapy
C Transfuse crossmatch-incompatible blood
D Transfuse O-negative uncrossmatched blood
C- Transfuse crossmatch-incompatible blood
Treat autoimmune hemolytic anemia with transfusion of crossmatch-incompatible blood
Key Point
In patients with severe symptomatic autoimmune hemolytic anemia, the autoantibody typically reacts against all erythrocytes, and a completely crossmatch-compatible unit may be impossible to find; these patients should receive ABO and Rh-matched blood even if it is not crossmatch compatible.
A 76-year-old man is evaluated for fever and a swollen, painful left knee. He was hospitalized 7 days ago for heart failure and appropriately treated. However, he developed fevers up to 38.0 °C (100.4 °F). On examination, lungs were clear to auscultation. The left knee was hot and swollen; 60 mL of turbid fluid was drained from the knee. Gram stain of the synovial fluid was negative; microscopy revealed needle-shaped intracellular crystals. Gout was diagnosed and the knee was drained again, followed by an injection of 80 mg of methylprednisolone, without improvement. Intravenous methylprednisolone, 60 mg/d for 3 days, did not improve the knee or the fevers. History is also significant for hypertension and gout. Other medications are furosemide, lisinopril, metoprolol, subcutaneous heparin, and morphine as needed.
On physical examination today, temperature is 38.0 °C (100.4 °F), blood pressure is 148/92 mm Hg, pulse rate is 116/min, and oxygen saturation is 97% on ambient air. The left knee is warm, swollen, and tender.
Blood, urine, and synovial fluid cultures are negative.
Which of the following is the most appropriate treatment for the knee?
A Anakinra
B Colchicine
C Ibuprofen
D Vancomycin
Correct Answer: A
Educational Objective: Treat refractory acute gout.
Key Point
For patients with severe and refractory gouty attacks or with contraindications to other treatments, off-label use of interleukin-1 inhibitors (anakinra or canakinumab) can be considered.
An 18-year-old woman is evaluated for a 2-year history of repetitive fast movements of the neck that tilt the head to the right side. She reports that these movements are preceded by a feeling of discomfort in the right shoulder and that if she concentrates and taps the right foot immediately after this sensory cue, she usually can avoid the neck movements. She is a senior in high school and says she has not been able to use this method successfully in the classroom, especially during examinations, when she often experiences a cluster of movements that exhaust and distract her. Her mother notes that before onset of the neck symptoms, the patient used to roll her eyes and clear her throat frequently. She has obsessive-compulsive disorder treated with sertraline.
On physical examination, vital signs are normal. During the examination, the patient displays rapid tilting movements of the head followed by rolling of the head and shoulder; these movements repeat several times in a stereotyped manner. The patient can suppress these movements on request.
Which of the following is the most appropriate treatment?
A- Botulinum toxin injection
B- Clonidine administration
C- Cognitive behavioral therapy
D- Haloperidol administration
Clonidine Administration
First-line agents used to treat Tourette syndrome when the associated tics interfere with education, daily function, or work are clonidine, guanfacine, topiramate, and tetrabenazine
Clonidine is a first-line agent used to treat Tourette syndrome. Anti-tic medications should be considered when tics interfere with education, daily function, or work.
Although botulinum toxin can be considered as an off-label option in the treatment of severe refractory cervical tics, this patient has not yet tried medication to control her tics
A 32-year-old woman is hospitalized with progressive exertional dyspnea. She has noted dark urine for the last week and yellowing of her skin for several days. Medical history is unremarkable, and she takes no medications.
On physical examination, temperature is 36.7 °C (98.0 °F), blood pressure is 100/70 mm Hg, pulse rate is 100/min, and respiration rate is 18/min. Icteric sclera and skin are noted. Cardiac examination reveals a grade 2/6 systolic flow murmur. No lymphadenopathy or hepatosplenomegaly is present. The remainder of the examination is noncontributory.
Haptoglobin Undetectable
Hemoglobin 4.8 g/dL (48 g/L)
Leukocyte count 8200/µL (8.2 × 109/L)
Mean corpuscular volume 134 fL
Platelet count 230,000 (230 × 109/L)
Reticulocyte count 12% of erythrocytes
Bilirubin Total 6.7 mg/dL (114.6 µmol/L)
Direct 1.2 mg/dL (20.5 µmol/L)
Lactate dehydrogenase 660 U/L
Urinalysis Dipstick positive for 4+ blood; 0-1 leukocytes/hpf, and 0 erythrocytes/hpf
A peripheral blood smear shows erythrocyte agglutination. A direct antiglobulin (Coombs) test is positive for C3. Diagnostic testing for Mycoplasma and Epstein-Barr virus is negative.
Which of the following is the most appropriate treatment?
A Intravenous immune globulin
B Prednisone
C Rituximab
D Splenectomy
Correct Answer: C
Educational Objective: Treat cold agglutinin disease
Cold avoidance and rituximab are effective treatments for cold agglutinin disease; glucocorticoids, splenectomy, and intravenous immune globulin are not helpful
An 83-year-old woman is evaluated for a 15-year history of gout. Attacks were initially limited to the first metatarsophalangeal joints and occurred once or twice a year. She now has recurrent attacks, occurring every 3 months. Her feet, ankles, knees, wrists, elbows, and hands have been affected. Attacks last for 1 to 2 weeks and have been treated with NSAIDs. History is also significant for recurrent nephrolithiasis, stage 3 chronic kidney disease, and hypertension. Medications are allopurinol, 400 mg/d; colchicine, 0.6 mg/d; and lisinopril. She tolerates her medications well.
On physical examination, vital signs are normal. There are palpable masses at the olecranon processes and white nodules on a few distal and proximal interphalangeal joints of both hands.
Laboratory studies show a serum creatinine level of 1.0 mg/dL (88.4 µmol/L) and a serum urate level of 5.8 mg/dL (0.34 mmol/L).
Which of the following is the most appropriate next step in management?
A Add probenecid
B Discontinue allopurinol; begin pegloticase infusions
C Increase allopurinol
D Increase colchicine
Correct Answer: C
Educational Objective: Treat tophaceous gout with appropriate urate-lowering therapy.
Key Point
For patients with inadequately treated tophaceous gout who tolerate allopurinol, the dose can be titrated to a maximum of 800 mg/d in 100-mg increments to alleviate symptoms.