Autoimmune
Drugs
Diagnosis
Who knew?
100

A 43-year-old woman is seen in a follow-up evaluation of myasthenia gravis diagnosed 2 years ago. Current treatment with daily prednisone and pyridostigmine partially controls her symptoms. She is experiencing weakness with prolonged activity and in the evening. She wishes to minimize long-term need for glucocorticoids and other medications that may suppress her immune system.

On physical examination, vital signs are normal. She has mild bilateral ptosis and impaired left eye adduction and bilateral eye abduction. Speech is nasal. Proximal upper and lower extremity muscle strength and neck flexion are mildly weak.

Acetylcholine receptor–binding antibody is positive.

CT of the chest is normal.

Which of the following is the most appropriate treatment? AKA: What management/treatment is the best way to minimize steroid use?

Thymectomy


Monoclonal Abs

Plasma exchange

Decrease prednisone dose

100

A 72-year-old woman is evaluated in the emergency department for a 1-hour history of left-sided weakness. The patient has hypertension and dyslipidemia. She has had no recent surgery or history of gastrointestinal, genitourinary, or intracranial bleeding. Medications are amlodipine and atorvastatin.

On physical examination, blood pressure is 168/92 mm Hg; the remaining vital signs are normal. Oxygen saturation  is 97% with the patient breathing ambient air. Left facial weakness, dysarthria, decreased sensation to pinprick on the left side of the body, and left arm and leg drift are noted. A bedside swallow examination is positive for dysphagia. Her score on the National Institutes of Health Stroke Scale is 6. Neurologic examination reveals a normal mental status. The remainder of the physical examination is normal.

A CT scan of the head without contrast shows no intracerebral hemorrhages or early hypodensities.

Which of the following is the most appropriate treatment?

IV alteplase


IV LMWH

IV nicardipine

PO ASA

100

A 70-year-old man is evaluated for a 2-day history of agitation. He has not slept and has been rummaging about the house. He has dementia due to Alzheimer disease. His caregiver reports that he has not started any new medications and has not shown any obvious signs of infection or injury. He has not been constipated and has been able to urinate regularly. The patient is unable to give a symptom history. His only medication is donepezil.

On physical examination, vital signs are normal and other findings are unremarkable.

A complete blood count and measurement of serum electrolytes , serum creatinine , and blood urea nitrogen  will be obtained.

Which of the following is the most appropriate additional diagnostic test?

UA


Spinal tap

ABG

CTH

100

A 29-year-old man is hospitalized for suicidal ideation. He was recently diagnosed with bipolar disorder without psychotic features. He has a history of medication-resistant generalized tonic-clonic seizures. He has no other medical problems. His antiepileptic drugs are valproic acid, levetiracetam, and lamotrigine. In addition, he is taking quetiapine. The patient has no other medical problems.

Which of the following drugs should be discontinued in the acute setting for this patient?

Levetiracetam


Lamotrigine

Valproate

Quetiapine

200

A 53-year-old woman was admitted to the hospital for bilateral optic neuritis. She is receiving intravenous methylprednisolone.

On physical examination, vital signs are normal. Visual acuity is 20/200 in both eyes. Reflexes are 3+ in the lower extremities, and plantar response is extensor. The remainder of the examination is normal.

An MRI of the thoracic spine shows an extensive hyperintense lesion in the thoracic cord from T3 to T7 with mild contrast enhancement. An MRI of the orbits shows hyperintensity in the anterior portion of the optic nerve bilaterally with contrast enhancement. Cerebrospinal fluid testing shows leukocytes (180/µL [180 × 106/L) composed mostly of neutrophils.

An MRI of the brain is normal.

Which of the following is the most appropriate management?

Serum aquaporin-4 Ab testing


Interferon beta

Oligoclonal band testing

Serum copper levels

200

A 78-year-old man is evaluated for two generalized tonic-clonic seizures that occurred 2 weeks apart during the past month. Medical history includes hypertension and type 2 diabetes mellitus and a recent diagnosis of mild cognitive impairment. Medications are hydrochlorothiazide and metformin.

On physical examination, vital signs are normal. All other examination findings, including those from a neurologic examination, are unremarkable.

Serum creatinine , electrolytes , and liver chemistry tests are normal.

Findings on brain MRI and electroencephalography are normal.

Which of the following is the most appropriate treatment?

Lamotrigine


Valproic acid

Oxcarbazapine

No meds

200

An 82-year-old woman is evaluated in the emergency department for a 10-week history of a gradual and steady progression of new-onset headaches. She is otherwise well and takes no mediations.

On physical examination, vital signs are normal. Neurologic examination is normal.

A T2-weighted MRI of the brain with contrast shows a well-demarcated, hyperintense lesion arising from lateral ventricles anteriorly and extending bilaterally into the frontal lobes. The lesion shows gadolinium enhancement on T1-weighted imaging.

Vitreous and cerebrospinal fluid sampling are negative for lymphomatous cells.

Which of the following is the most appropriate next step in management?

Brain biopsy


Surgical resection

MTX

Dexamethasone

200

A 58-year-old man is evaluated for disabling rhythmic movements involving his upper extremities. These movements started 8 years ago and now interfere with his job as a chemist. He reports that his handwriting has become illegible. Previous treatment with propranolol, primidone, and clonazepam did not improve these symptoms. He also has a history of kidney stones. Family history includes tremor in his brother and Parkinson disease in his uncle. He takes no medications.

On physical examination, vital signs are normal. The patient's voice quivers during prolonged vowels. Bilateral upper extremities exhibit a high-amplitude rhythmic movement during the finger-to-nose task and with outstretched position. There are no involuntary movements when hands are placed on the chair arms. Rapid alternative movements, tone, and gait are normal.

Which of the following is the most appropriate treatment?

Deep brain stimulation


Botox

Levodopa

Occupational therpay

Topiramate

300

A 32-year-old woman is hospitalized for recent-onset leg weakness. She reports development of pins-and-needles sensation in her upper and lower extremities 10 days ago, followed by low back pain and progressive weakness in her lower extremities. She has otherwise been well and reports no other symptoms. She received an influenza vaccination a month ago. She takes no medications.

On physical examination, temperature is 37.2 °C (98.9 °F), blood pressure is 105/70 mm Hg, pulse rate is 115/min, respiration rate is 18/min. Moderate weakness in hip flexors and mild weakness in foot dorsiflexors and plantar flexors bilaterally are observed. Deep tendon reflexes are absent throughout. Cranial nerves, including extraocular movements, are intact. Plantar responses are flexor. Sensory perception is intact to all modalities.

Cerebrospinal fluid cell count  and protein  and glucose  levels are normal.

Electromyogram shows no neuropathy, radiculopathy, or myopathy.

Cervical, thoracic, and lumbar MRI findings are unremarkable.

Which of the following is the most appropriate treatment?

Plasma exchange


Steroids

Nothing

Doxycycline

300

A 51-year-old woman is evaluated for a 5-year history of increasingly frequent and disabling migraine episodes. Whereas she previously had migraine on the first 2 days of menses, migraine episodes now have increased to 10 days monthly; once monthly, she experiences a scotoma lasting 20 minutes before migraine onset. Oral and subcutaneous sumatriptan have been effective in controlling symptoms, but because of the increased frequency of her headaches she sometimes runs out of medication during the month. Preventive trials of propranolol, amitriptyline, and topiramate over the past 2 years have been unsuccessful.

All physical examination findings, including vital signs, are normal.

Results of laboratory studies are within normal limits.

Which of the following is the most appropriate next step in preventive treatment?

Erenumab


Botox

Sertraline

Verapamil

Ethinyl estradiol

300

A 58-year-old man is evaluated for left lower extremity weakness. Three months ago, severe pain in the left buttock developed. The pain has since diminished, but progressive left-thigh weakness and muscle wasting are now present. He has hypothyroidism, type 2 diabetes, and alcohol use disorder. Current medications are levothyroxine, metformin, and canagliflozin.

On physical examination, vital signs are normal. BMI is 20. There is tenderness to touch over the left buttock and inner thigh. Sensory perception to light touch and pinprick is diminished over the left medial thigh and dorsum of the left foot. Left anterior thigh muscles are weak and wasted. Fasciculation is noted in left quadriceps and lumbar paraspinal muscles. Examination of the toes reveals a flexor plantar response. Patellar and Achilles reflexes are absent on the left side and diminished on the right side. The rest of the neurologic examination is normal.

Laboratory studies show a hemoglobin A1c  level of 6.9% and thyroid-stimulating hormone  level of 5.2 μU/mL (5.2 mU/L).

Electromyogram reveals multifocal sensorimotor axon loss and denervation affecting the proximal more than the distal left lower extremity.

MRI of lumbar spine and CT scan of abdomen and pelvis are unremarkable.

Which of the following is the most likely diagnosis?

Diabetic amyotrophy


Alcoholic polyneuropathy

Hypothyroid plexopathy

Autoimmune ganglionopathy 

300

A 64-year-old woman is evaluated for a 3-month history of daily headaches of sudden, spontaneous onset. Although the patient awakens each morning without any pain, a headache typically develops within 30 minutes after assuming an upright posture; the pain is dull and localized at the top of the head but becomes more intense and global throughout the day. Bedrest will ease her symptoms, but the headaches typically return when assuming the upright position.

Vital signs and physical examination findings are normal.

A contrast-enhanced MRI of the brain reveals 2 mm of cerebellar tonsillar descent and enhanced diffuse smooth dural thickening. MRIs of the cervical, thoracic, and lumbar spines are normal.

Which of the following is the most appropriate treatment of this patient's headaches?

Epidural blood patch


Surgical occipital decompression

Acetazolamide

Methylprednisone

400

A 49-year-old woman is evaluated for a 3-year history of slowly worsening bilateral lower extremity weakness, fatigue, and numbness in the hands. She had a single episode of unilateral optic neuritis 8 years ago that resolved following administration of glucocorticoids. She has no other medical problems and takes no medications.

On physical examination, vital signs are normal. Muscle strength testing shows 4/5 bilateral lower extremity weakness. There is 3+ hyperreflexia throughout in both lower extremities and extensor plantar response.

MRI of the brain shows five periventricular white matter hyperintensities, two with contrast enhancement. A T2-weighted MRI of the cervical and thoracic spine shows multiple hyperintensities without contrast enhancement.

A diagnosis of multiple sclerosis is established.

Which of the following best describes the status of this patient's multiple sclerosis?

Secondary progressive, with progression and activity


Primary progressive, with progression but without activity

Relapsing remitting, with activity

Relapsing remitting, without activity

Secondary progressive, with progression but without activity

400

A 64-year-old man is evaluated for recent emergence of involuntary twisting movements of his neck and extremities. Parkinson disease was diagnosed 8 years ago. Mobility and fatigue improve after each dose of carbidopa-levodopa. A few months ago, he started to feel excessive slowness and anxiety 1 hour before each dose of medication and was treated with an increased dose of his carbidopa-levodopa. Now, he has developed involuntary nonrhythmic movements that peak after each dose. Current medication is carbidopa-levodopa.

On physical examination, vital signs are normal. He is examined half an hour after taking a dose of levodopa. He exhibits cogwheel rigidity, shuffling of gait, and large-amplitude ballistic and flowing movements of the neck and extremities. Other examination findings are unremarkable.

Which of the following is the most appropriate next step in treatment?

Amantadine


Entacapone

Ropinirole

Deep brain stim

Selegiline

400

A 75-year-old man is evaluated for progressive cognitive impairment over the past year that has led to loss of daily function. He loses his train of thought and misplaces items at home. His gait has slowed, and his behavior has changed. He has hypertension, hyperlipidemia, type 2 diabetes mellitus, and coronary artery disease. Current medications are lisinopril, carvedilol, atorvastatin, metformin, canagliflozin, and aspirin.

On physical examination, blood pressure is 153/85 mm Hg and pulse rate is 65/min. Deep tendon reflexes are normal; gait is slowed. No hypomimia, hypophonia, tremor, rigidity, or shuffling gait is observed. He demonstrates slowed processing speed when generating word lists.

MRI of the brain shows diffuse, confluent white matter hyperintensities and no significant hippocampal atrophy.

Which of the following is the most likely diagnosis?

Vascular cognitive dementia


Lewy body

Alzheimer's

Frontotemporal

400

A 20-year-old man is evaluated for a 2-year history of migraine that began when he started college. Migraine episodes occur 6 to 12 days per month, with more frequent episodes associated with increased stress from difficult assignments or final examinations.

The migraine pain is typically bifrontal and steady, becoming severe when he bends forward. When the pain is severe, he also experiences sensitivities to light and noise but has no visual aura, nausea, or vomiting; neck pain and a slight vertiginous sensation may accompany the most intense episodes. Ibuprofen, naproxen, or diclofenac provides only limited pain relief. He has had no other medical problems or symptoms.

All physical examination findings, including vital signs, are unremarkable.

Which of the following is the most appropriate preventive measure?

Venlafaxine


Lamotrigine

Gabapentin

Indomethacin

Citalopram