A 56-year-old male with history of polycystic kidney disease presented to the emergency room because of sudden onset severe headache. He has never had this type of headache in the past. Examination shows some nuchal rigidity. Neurologic examination is within normal limits. Which of the following is the most likely diagnosis?

Question

A 56-year-old male with history of polycystic kidney disease presented to the emergency room because of sudden onset severe headache.  He has never had this type of headache in the past.  Examination shows some nuchal rigidity.  Neurologic examination is within normal limits.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

Although the major pathologic process in Autosomal Dominant Polycystic Kidney Disease (ADPKD) is in the kidneys, it is a systemic disorder. Cysts and other anomalies are noted in different organs. Intracranial berry aneurysms arise in the circle of Willis, and when these aneurysms rupture they cause subarachnoid hemorrhage (SAH). The clinical presentation described in this question is suggestive of SAH. Patients complain of a severe headache ("the worst headache of their life"); neck stiffness is usually present or develops in the next 24 hours as blood in the subarachnoid space acts as a meningeal irritant. Papilledema and pupillary dilation may be noted, but there are no focal neurologic findings. CT scan of the brain without contrast is the most commonly used initial imaging study for diagnosing SAH and should be done during the first 24 hours from the onset of symptoms. This is positive in up to 90% of patients. If the CT scan does not show SAH, but the clinical suspicion remains high, a lumbar puncture needs to be performed to evaluate the cerebrospinal fluid for the presence of xanthochromia (blood in the CSF). Thus, presence of the xanthochromia in a clinical scenario suggestive of SAH is the most sensitive test for diagnosing SAH. (Choice A) The classic presentation of meningitis includes fever, headache, neck stiffness, photophobia, nausea, vomiting, and signs of cerebral dysfunction (eg, lethargy, confusion, and coma). Additionally, remember to look for clues leading you to the correct answer. For example, the question writer here mentioned that this patient has ADPKD for a reason, to lead you to SAH. (Choice B) Migraine headache is throbbing or pulsatile. It is initially unilateral and localized in the frontotemporal and ocular area, then progresses posteriorly and becomes diffuse. It typically lasts from several hours to an entire day. (Choice C) Intracerebral hemorrhage manifests with headache, vomiting and sudden onset of focal neurological deficits (hemiplegia, hemianesthesia, cranial nerve deficits, aphasia etc). Focal neurologic manifestations are unusual with SAH. (Choice E) An elderly male with scalp tenderness over the temporal area (or headache), blurred vision and intermittent claudication of jaw is a typical presentation of temporal arteritis. This condition is also accompanied by a very high ESR (erythrocyte sedimentation rate) and many times occurs in the setting of polymyalgia rheumatica. Rapid treatment with corticosteroids is necessary to prevent potential blindness. (Choice F) Cluster headache consists of recurrent brief attacks of sudden, severe, unilateral periorbital pain associated with ipsilateral rhinorrhea, and watering of the eyes. Educational Objective: Intracranial berry aneurysms of the circle of Willis are often seen in patients with ADPKD; when ruptured, they cause subarachnoid hemorrhage that presents with sudden onset of "thunderclap headache".

A 56-Year-Old Male with History of Polycystic Kidney Disease Presented