A 46-year-old woman is seen in the emergency department for severe headache accompanied by nausea since she awoke this morning. She was diagnosed with hypertension 7 years ago and took medications for 2 years. The patient stopped the medications and did not follow up with doctors as she felt fine. She has a history of remote but not active intravenous drug use. She is a current smoker with a 25-pack-year history.
Her temperature is 37.8° C (100.1° F), blood pressure is 176/105 mm Hg, and pulse is 100/min. She appears lethargic with mild neck stiffness. Cardiac auscultation shows an apical 2/6 holosystolic murmur. Lungs are clear to auscultation bilaterally. Deep abdominal palpation shows bilateral firm flank masses. There is no peripheral edema or skin rash. There is no focal muscle weakness or cranial nerve deficit.
ECG shows sinus tachycardia with left ventricular hypertrophy and secondary ST-segment and T-wave changes.
Laboratory results are as follows:

Which of the following is the most likely cause of this patient's headache?

Question

A 46-year-old woman is seen in the emergency department for severe headache accompanied by nausea since she awoke this morning.  She was diagnosed with hypertension 7 years ago and took medications for 2 years.  The patient stopped the medications and did not follow up with doctors as she felt fine.  She has a history of remote but not active intravenous drug use.  She is a current smoker with a 25-pack-year history.
Her temperature is 37.8° C (100.1° F), blood pressure is 176/105 mm Hg, and pulse is 100/min.  She appears lethargic with mild neck stiffness.  Cardiac auscultation shows an apical 2/6 holosystolic murmur.  Lungs are clear to auscultation bilaterally.  Deep abdominal palpation shows bilateral firm flank masses.  There is no peripheral edema or skin rash.  There is no focal muscle weakness or cranial nerve deficit.
ECG shows sinus tachycardia with left ventricular hypertrophy and secondary ST-segment and T-wave changes.
Laboratory results are as follows:

Which of the following is the most likely cause of this patient's headache?

Quizplus · Accepted Answer

This patient's presentation of hypertension, hematuria, proteinuria, renal insufficiency, and bilateral flank masses suggests likely autosomal dominant polycystic kidney disease (ADPKD). Major extrarenal complications of ADPKD are: Intracranial aneurysms Hepatic and pancreatic cysts Cardiac valve disease and aortic root dilatation Diverticulosis Intracranial aneurysms Hepatic and pancreatic cysts Cardiac valve disease and aortic root dilatation Diverticulosis Nearly 5% of young adults and 20% of patients > age 60 with ADPKD develop intracranial aneurysms that can rupture causing intracerebral or subarachnoid hemorrhage. This risk is higher in patients with family history of ruptured aneurysms. Patients typically present with "thunderclap" headache, nausea, and vomiting. Computed tomography (CT) scan usually diagnoses subarachnoid hemorrhage but can sometimes miss small bleeds. Lumbar puncture is indicated in patients with negative CT scan and usually shows xanthochromia. Angiography is recommended for patients with positive CT scan or lumbar puncture. Screening for intracranial aneurysms in ADPKD is controversial. This is because routine screening can identify small aneurysms (< 7 mm) that are at low risk for rupture, but the surgical intervention of these aneurysms can result in severe neurologic complications. Guidelines recommend screening patients with previous aneurysm rupture, family history of intracerebral bleed or aneurysm, and high-risk occupations (eg, pilots). (Choice A) Uncontrolled hypertension, sudden-onset headache, and neck stiffness suggest possible subarachnoid hemorrhage. Bilateral flank masses in this situation indicate underlying ADPKD rather than renal cell carcinoma with metastasis. (Choice B) Although this patient may be at risk for hypertensive encephalopathy, her low-grade fever and neck stiffness indicate subarachnoid hemorrhage. Low-grade fever in general is common with intracranial hemorrhage. Her blood pressure is also not severe enough to cause hypertensive encephalopathy (>180/120 mm Hg). (Choice C) Obstructive or non-communicating hydrocephalus is usually caused by space-occupying lesions. (Choice E) Although this patient's fever and neck stiffness can be due to viral meningitis/encephalitis, the bilateral flank masses, hematuria, and renal insufficiency make ADPKD more likely. Educational objective: Intracranial aneurysms occur in up to 20% of individuals with autosomal dominant polycystic kidney disease. However, routine screening for aneurysms is controversial. If chosen, screening is best performed with time-of-flight magnetic resonance angiography, which does not require gadolinium contrast and can be done at any glomerular filtration rate level. Computed tomography angiography is an alternate screening method. __________ References: Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease. (http://www.ncbi.nlm.nih.gov/pubmed/21551026) Follow-up of intracranial aneurysms in autosomal-dominant polycystic kidney disease. (http://www.ncbi.nlm.nih.gov/pubmed/15086900)

A 46-Year-Old Woman Is Seen in the Emergency Department for Severe