More on Spontaneous Intracranial Hypotension
Abstract & Commentary
Source: Schievink WI, et al. Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage. Neurosurgery. 2001;48:513-517.
The clinical features, pathogenesis, and treatment of spontaneous intracranial hypotension (SIH) were recently reviewed in Neurology Alert (Caronna JJ. Neurology Alert 2001;19:43-44.) Nevertheless, the present study is notable because it describes an unexpected mode of presentation of that condition. Among 28 patients with a documented cerebral spinal fluid (CSF) leak, 4 presented with an excruciating headache of instantaneous onset ("thunderclap headache") as the initial manifestation of SIH. The mean age of the 4 patients (2 women and 2 men) was 35 years (range, 24-45). A stiff neck was present in 3 patients who were diagnosed initially as having a subarachnoid hemorrhage (SAH) and, therefore, they underwent emergency brain CT scanning, lumbar puncture, and cerebral angiography, all of which were normal. All 4 patients had meningeal enhancement and brain sagging on MRI scanning. Treatment consisted of ligation of 2 meningeal diverticula in 1 patient and repair of a dural tear caused by an osteophyte in another. Two patients who did not have a structural lesion identified on CT myelography underwent successful epidural blood patching.
Commentary
Schievink and colleagues found that thunderclap headache was a relatively common (4/28 patients) presentation of SIH, an observation that has not been reported previously (Day JW, Raskin NH. Lancet. 1986;2:1247-1248). Therefore, they have added another diagnosis to the differential diagnosis of thunderclap headache that already includes SAH, cervical artery dissection, cerebral vein thrombosis, pituitary apoplexy, and migraine.
The diagnosis of acute severe headache involves the exclusion of serious intracranial disease, therefore, the initial evaluation of thunderclap headache must include a CT scan and lumbar puncture to rule out bleeding and increased intracranial pressure. If the findings on these tests are normal, then Schievink et al suggest the best subsequent investigation is a contrast-enhanced brain MRI rather than cerebral angiography. —John J. Caronna
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