Spontaneous Spinal Cerebrospinal Fluid Leaks: A Review

, Cedars-Sinai Neurosurgical Institute, Los Angeles, and Department of Neurosurgery, University of California, Irvine, California

Neurosurg Focus. 2000;9(1) 

In This Article

Abstract and Introduction

Abstract

Spontaneous intracranial hypotension has become a well-recognized clinical entity, but it remains an uncommonly, and probably underdiagnosed, cause of headache; its estimated prevalence is only one in 50,000 individuals. The clinical spectrum of spontaneous intracranial hypotension is quite variable and includes headache, neck stiffness, cranial nerve dysfunction, radicular arm pain, and symptoms of diencephalic or hindbrain herniation. Leakage of the spinal cerebrospinal fluid (CSF) is the most common cause of spontaneous intracranial hypotension. A combination of an underlying weakness of the spinal meninges and a more or less trivial traumatic event is often found to cause this event in these patients. Typical magnetic resonance imaging findings include diffuse pachymeningeal enhancement, subdural fluid collections, and downward displacement of the brain, sometimes mimicking a Chiari I malformation. Opening pressure is often, but not always, low, and examination of CSF may reveal pleocytosis, an elevated protein count, and xanthochromia. The use of myelography computerized tomography scanning is the most reliable method for the accurate localization of the CSF leak. Most CSF leaks are found at the cervicothoracic junction or in the thoracic spine. The initial treatment of choice is a lumbar epidural blood patch, regardless of the location of the CSF leak. If the epidural blood patch fails, the blood patch procedure can be repeated at the lumbar level, or a blood patch can be directed at the exact site of the leak. Surgical repair of the CSF leak is safe and generally successful, although a distinct structural cause of the leak often is not found.

Introduction

When a person is in the upright position, intracranial pressure is negative.[9] Intracranial hypotension is defined as an abnormally low intracranial pressure, and it is characterized by headaches that worsen when standing and improve when lying down. The occurrence of such posture-related headaches is well known to all physicians in individuals who have undergone lumbar puncture as well as to neurosurgeons, in particular, who observe these headaches in patients following craniospinal traumatic injury or CSF shunt placement. Although a spontaneous form of intracranial hypotension was described more than 60 years ago,[67] it has only been recently that this condition has become a well-recognized clinical entity. Spontaneous intracranial hypotension is also known as essential aliquorrhea, spontaneous hypoliquorrhea, and Schaltenbrand syndrome. Georg Schaltenbrand 67 was the German physician who first described patients with spontaneously occurring low CSF pressures, but his role in the Third Reich[77] should preclude the eponym from being used.

In this review I discuss recent developments in our understanding of the causes and pathogenesis of spontaneous intracranial hypotension, its clinical spectrum, methods of diagnosis, and approaches to treatment.

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