A 29-year-old man is brought to the emergency department in a comatose
state a few hours after complaining of sudden onset of excruciating
headache. Neurologic examination reveals dilated pupils poorly
responsive to light. A CT scan of the head without contrast demonstrates
hyperdensity within the suprasellar cistern, while MRI is unremarkable.
Lumbar puncture shows hemorrhagic cerebrospinal fluid. Which of the
following is the most likely diagnosis?
a)Amyloid angiopathy-related hemorrhage
b)Cavernous sinus thrombosis
c)Hemorrhagic infarction
d)Pituitary apoplexy
e)Ruptured berry aneurysm
Explanation:
The correct answer is
E.
Headache of sudden onset ("thunderclap" headache), rapid
deterioration of mental status and blood in the CSF are virtually
diagnostic of ruptured berry aneurysms. Note the characteristic
hyperdensity on CT of the suprasellar cistern, indicating blood in the
subarachnoid space. Rupture of a berry aneurysm is the most common cause
of subarachnoid bleeding. Berry aneurysms develop as a result of
congenital weakness at branching points of the arteries in the circle of
Willis. These outpouchings tend to expand progressively, but in most
cases they remain asymptomatic. Hypertension facilitates development and
rupture of berry aneurysm. One third of patients recover, one third
die, and one third develop re-bleeding. Rapid onset of coma is an
ominous sign.
Amyloid angiopathy-related hemorrhage (choice A) would manifest as a
cortical-based hematoma in a lobar distribution. It is due to
accumulation of Aβ amyloid in blood vessel walls.
Cavernous sinus thrombosis (choice B) is a rare complication of
conditions leading to coagulation abnormalities, such as sepsis,
antiphospholipid antibody syndrome, and leukemias. It leads to
hemorrhagic infarction of large areas of hemispheric gray and white
matter.
Hemorrhagic infarction (choice C) usually develops as a result of
embolic occlusion of an intraparenchymal artery. It gives rise to a
hyperdense wedge-shaped area in a cortical field corresponding to a
specific vascular territory.
Pituitary apoplexy (choice D) refers to hemorrhage in the pituitary
gland. It may occur in the setting of a large pituitary adenoma or in
pregnancy. It manifests with rapid onset of panhypopituitarism.
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