M. Marcel Maya

Diagnostic Criteria for Spontaneous Spinal CSF Leaks and Intracranial Hypotension

BACKGROUND AND PURPOSE:Comprehensive diagnostic criteria encompassing the varied clinical and radiographic manifestations of spontaneous intracranial hypotension are not available. Therefore, we propose a new set of diagnostic criteria. MATERIALS AND METHODS: The diagnostic criteria are based on results of brain and spine imaging, clinical manifestations, results of lumbar puncture, and response to epidural blood patching. The diagnostic criteria include criterion A, the demonstration of extrathecal CSF on spinal imaging. If criterion A is not met, criterion B, which is cranial MR imaging findings of spontaneous intracranial hypotension, follows, with at least one of the following: 1) low opening pressure, 2) spinal meningeal diverticulum, or 3) improvement of symptoms after epidural blood patch. If criteria A and B are not met, there is criterion C, the presence of all of the following or at least 2 of the following if typical orthostatic headaches are present: 1) low opening pressure, 2) spinal meningeal diverticulum, and 3) improvement of symptoms after epidural blood patch. These criteria were applied to a group of 107 consecutive patients evaluated for spontaneous spinal CSF leaks and intracranial hypotension. RESULTS: The diagnosis was confirmed in 94 patients, with use of criterion A in 78 patients, criterion B in 11 patients, and criterion C in 5 patients. CONCLUSIONS:A new diagnostic scheme is presented reflecting the wide spectrum of clinical and radiographic manifestations of spontaneous spinal CSF leaks and intracranial hypotension.

Cranial MRI predicts outcome of spontaneous intracranial hypotension

The outcome of spontaneous intracranial hypotension has been unpredictable. The results of initial MRI were correlated to outcome of treatment in 33 patients with spontaneous intracranial hypotension. A good outcome was obtained in 25 (97%) of 26 patients with an abnormal MRI vs only 1 (14%) of 7 patients with a normal MRI (p = 0.00004). These findings show that normal initial MRI is predictive of poor outcome in spontaneous intracranial hypotension.

Frequency of spontaneous intracranial hypotension in the emergency department

Spontaneous intracranial hypotension is considered a rare disorder. We conducted a study on the frequency of spontaneous intracranial hypotension in the emergency department (ED). We identified patients with spontaneous intracranial hypotension evaluated in the ED of a large urban hospital between 1 January 2003 and 31 December 2006. For comparison, we also identified all patients with spontaneous subarachnoid haemorrhage (SAH). Eleven patients with previously undiagnosed spontaneous intracranial hypotension were evaluated in the ED during the four-year time period. All patients presented with positional headaches and the duration of symptoms varied from one day to three months. None of the patients were correctly diagnosed with spontaneous intracranial hypotension in the ED. During the same time period, 23 patients with aneurysmal SAH were evaluated. Spontaneous intracranial hypotension is more common than previously appreciated and the diagnosis in the ED remains problematic.

Pseudo-subarachnoid hemorrhage: a CT-finding in spontaneous intracranial hypotension.

Increased attenuation in the basilar cisterns or along the tentorium cerebelli resembling subarachnoid hemorrhage (SAH) may be found on CT in the absence of blood (pseudo-SAH). The authors found pseudo-SAH on CT in four of 40 patients with spontaneous intracranial hypotension. All four patients had brain sagging with obliteration of the cisterns and pachymeningeal enhancement along the tentorium cerebelli. Spontaneous intracranial hypotension should be included in the differential diagnosis of pseudo-SAH.

Screening for intracranial aneurysms in patients with bicuspid aortic valve

Objective: Bicuspid aortic valve (BAV) is a common congenital heart defect affecting half to 2% of the population. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in this patient population. We therefore screened a group of patients with BAV for the presence of intracranial aneurysms. Methods: Magnetic resonance angiography or CT angiography of the brain was used in 61 patients with BAV (age, 29–70 years [mean 48 years]) and in 291 controls (28–78 years [mean 56 years]). Results: Intracranial aneurysms were detected in 6 of 61 patients with BAV (9.8%; 95% confidence interval [CI] 2.4%–17.3%). This was significantly higher than in the control population (3/291 [1.1%; 95% CI 0%–2.2%]) (p = 0.0012). Female sex (p = 0.02) and advanced age (p = 0.003), risk factors for intracranial aneurysm development, were more common in the control population than among the patients with a BAV. No significant differences were detected in age, sex, smoking, arterial hypertension, alcohol use, aortic diameter, or frequency of aortic coarctation between BAV patients with and without intracranial aneurysms. Conclusion: In this case-control study, the frequency of intracranial aneurysms among our bicuspid aortic valve patient population was significantly higher than in the control population.

Frequency of incidental intracranial aneurysms in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is often mentioned among the heritable connective tissue disorders associated with intracranial aneurysms, but the association has not been firmly established. We therefore reviewed a contemporary series of hospitalized patients with NF1, many of whom underwent brain magnetic resonance imaging (MRI). We identified patients with NF1 who were hospitalized at Cedars‐Sinai Medical Center, Los Angeles, California, between January 1, 1997 and December 31, 2001 through the hospitals centralized medical records system using DRG codes. The mean age of the 39 patients was 30.4 years, and 22 patients had undergone MRI of the brain for the evaluation of symptoms due to the presence of central or peripheral nervous system tumors. Incidental intracranial aneurysms were detected in 2 (5%) of the 39 patients. Limiting the patient population to the 22 patients who had undergone MRI examination, the detection rate was 9%. This was significantly (P < 0.005) higher than the aneurysm detection rate in a control population (0/526 [0%]) of patients hospitalized for primary or secondary brain tumors, all of whom had undergone MRI examination. Our study suggests that patients with NF1 are at an increased risk of developing intracranial aneurysms.

ANAPHYLACTIC REACTIONS TO FIBRIN SEALANT INJECTION FOR SPONTANEOUS SPINAL CSF LEAKS

Spontaneous intracranial hypotension due to a spontaneous spinal CSF leak is an important cause of new-onset headaches, particularly in young and middle-aged adults.1 Treatment has traditionally involved epidural blood patching or surgical CSF leak repair if conservative treatment, e.g., bed rest, fails.1 Recently, percutaneous placement of fibrin sealant has emerged as an alternate therapy.1-3 The fibrin sealant is injected through a large-bore needle into the epidural space at the site of the CSF leak. Allergic reactions following topical placement of fibrin sealants are caused by the presence of aprotinin, a proteinase inhibitor derived from bovine lung, but they are extremely rare (0.005% of applications).4-6 Fibrin sealants contain a small amount of aprotinin to prevent lysis of the clot. Allergic reactions following intravascular administration of aprotinin during cardiac surgery are more common, occurring in less than 0.1% of initial applications but in 3% upon re-exposure.6 In approving aprotinin, the Food and Drug Administration recommended against its …

Reversible Cerebral Vasoconstriction in Spontaneous Intracranial Hypotension

Myelography showed an opening pressure of 0 cm H2O and multiple thoracic meningeal diverticula in a 52‐year‐old woman suffering from orthostatic headaches of instantaneous onset. MR‐angiography showed severe segmental arterial stenosis of the anterior and posterior circulation, which resolved over a 4‐day period following an epidural blood patch. Spontaneous intracranial hypotension should be considered in the differential diagnosis of reversible cerebral vasoconstriction.

Quadriplegia and cerebellar hemorrhage in spontaneous intracranial hypotension

Spontaneous intracranial hypotension due to a spinal CSF leak can cause new headaches in young and middle-aged adults.1 Typically, patients present with orthostatic headaches, but other symptoms have been reported, including neck pain, diplopia, hearing abnormalities, parkinsonism, dementia, and coma.1 We report a patient with spontaneous intracranial hypotension who presented with acute quadriplegia. A 54-year-old woman reached backward while sitting in a parked car and suddenly experienced a sensation of “pins and needles” in her head and noticed that she was unable to move her arms and legs. After approximately 30 to 40 seconds, she rapidly regained full strength in her extremities. In the emergency department, a head CT was interpreted as normal, a diagnosis of stroke was made, and aspirin was administered. Approximately 2 hours after the ictus, she noticed pain in the posterior neck and head, which was relieved by the upright position and worsened within minutes of lying down. Two weeks later, MRIs were obtained, and these were interpreted as showing a Chiari malformation and “posttraumatic” changes in …

Spontaneous spinal cerebrospinal fluid leaks as the cause of subdural hematomas in elderly patients on anticoagulation

Subdural hematoma is a relatively common complication of long-term anticoagulation, particularly in the elderly. The combination of anticoagulation and cerebral cortical atrophy is believed to be sufficient to explain the subdural bleeding. The authors report a series of elderly patients who were on a regimen of anticoagulation and developed chronic subdural hematomas (SDHs) due to a spontaneous spinal CSF leak. They reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension who were evaluated at Cedars-Sinai Medical Center. Among 141 patients with spontaneous spinal CSF leaks and spontaneous intracranial hypotension, 3 (2%) were taking anticoagulants at the time of onset of symptoms. The mean age of the 3 patients (1 woman and 2 men) was 74 years (range 68-86 years). All 3 patients had chronic SDHs measuring between 12 and 23 mm in maximal diameter. The SDHs resolved after treatment of the underlying spontaneous spinal CSF leak, and there was no need for hematoma evacuation. Epidural blood patches were used in 2 patients, and percutaneous placement of a fibrin sealant was used in 1 patient. The presence of an underlying spontaneous spinal CSF leak should be considered in patients with chronic SDHs, even among the elderly taking anticoagulants.