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Source: Chung SJ, et al. Neurology 2000;55:1321-1327.
Previous studies have described the syndrome of spontaneous intracranial hypotension as including: orthostatic headache, low cerebral spinal fluid (CSF) pressure, and diffuse pachymeningeal gadolinium enhancement on MRI without antecedent trauma or lumbar puncture (Fishman RA, et al. Neurology 1993; 43:609-611; Pannullo SC, et al. Neurology 1993;43:919-926). The underlying pathogenic mechanism is thought to be a spontaneous CSF leak from a spinal meningeal diverticulum or a dural tear (Schievik WI, et al. J Neurosurg 1996;84:598-605). Atypical patents, however, have been reported including those without headache or with nonpositional headache; without pachymeningeal enhancement on MRI; and with clinical signs of encephalopathy, cervical myelopathy, or parkinsonism (see article for references). In addition, the identification of patients with typical symptoms and MRI images but without CSF hypotension has caused the term "CSF hypovolemia" to become the preferred name for this syndrome.
Chung and associates retrospectively studied 30 consecutive patients with the syndrome of CSF hypovolemia admitted to the Asian Medical Center in Seoul, Korea, between 1995 and 2000.
Patients were included who met at least two of the following three criteria: orthostatic headache, low CSF pressure, and diffuse pachymeningeal enhancement on cranial MRI. Two patients did not undergo CSF examination and one patient had a normal CSF opening pressure despite the presence of multiple CSF leaks on radioisotope cisternography.
There were 10 men and 20 women aged 25-53 years (mean 37 ± 8.4). No patients gave a history of head or neck trauma. Seven patients had possible causes of CSF leakage including, in one patient each, chiropractic manipulation, playing golf, swimming, and yoga exercise. Two patients each reported prior vigorous physical activity and severe coughing.
All patients had orthostatic headaches that were variously described as tugging in 10, pulsating in four, pressing in three, and splitting in two. The locations of the headaches are shown in Table 1. The location of the symptoms in addition to headache are shown in Table 2. One woman with bilateral subdural hematomas also had disorientation, memory loss, decreased verbal output, and bradykinesia.
|Table 1: Location of Orthostatic Headaches|
Number of patients
|Occipital and bifrontal||3|
|Posterior neck pain||12|
|Table 2: Orthostatic Symptoms in Addition to Headache|
Number of patients
CSF findings were clear in 19, xanthochronic in three, and traumatic in four. Thirteen patients had a CSF pleocytosis, usually up to 50 cells and mostly lymphocytes. Twenty patients had an increased level of CSF protein, the highest being 566 mg/dL. CSF glucose concentration was normal in all patients.
Brain MRI showed diffuse pachymeningeal gadolinium enhancement in 10 of 23 patients. Subdural fluid collections with mass effect were detected in four patients. There was MRI evidence of brain descent in 11 patients. Radioisotope cisternography was abnormal in 21 of 23 patients and showed CSF leaks in 12 (see Table 3).
Location of CSF Leaks Determined by
|Site of leak||Number of patients|
|Cisterna magna (faint)||2|
Overall, headache resolved in all but one patient. Seven patients were treated with bed rest, analgesics, and hydration; 23 with epidural blood patches, and two of them also had surgical drainage of subdural hematomas. In 18 patients headaches resolved completely, and in 11 only partially. The rate of complete resolution of headache (70%) was greater in those who received epidural blood patches as compared with those who received only supportive treatment (29%). In those who received blood patches, complete resolution of headache was achieved less often in the five patients with multiple CSF leaks than in those with a single leak.
This study of a large number of patients with spontaneous CSF hypovolemia syndrome provides useful information, although patients’ symptoms were similar to those in other published series. The results indicate that epidural blood patch is an effective treatment in most patients except those with multiple sites of CSF leakage in whom it was less often successful.
As Neurology Alert went to press, the following relevant tidbit about CSF hypovolemia caught our eyes from Alvarez-Linera and colleagues (Neurology 2000; 55:1895-1897). They briefly report that all 11 female patients with the hypotension syndrome possessed an enlarged pituitary gland (similar findings have been noted by Shimazu and colleagues in a Japanese journal and an abstract by Mokri and Atkinson in Ann Neurol 1999;46:475). Alvarez-Linera et al, however, found that the pituitary swelling disappeared in all of their patients when they recovered from the hypotension syndrome. They attribute the temporary transient anatomic change of the pituitary gland to edema caused by the intracranial venous sinuses engorged by increased venous pressure. To your editor’s knowledge, studies of pituitary function have not yet been undertaken. — John J. Caronna