CHICAGO—“Spontaneous cerebrospinal fluid (CSF) leaks are increasingly recognized, and a broader clinical and imaging spectrum of the disorder is emerging,” said Bahram Mokri, MD. “Headache, typically orthostatic, is the most common clinical presentation,” he added, “but recent evidence suggests that spontaneous cerebrospinal fluid leaks may sometimes present as exertional headaches.” Dr. Mokri is a Professor of Neurology at Mayo Medical School, Rochester, Minnesota.

CSF LEAKS—PRESENTATION AND TREATMENT PROTOCOLS

“Rarely are there any dangers associated with spontaneous cerebrospinal fluid leaks,” Dr. Mokri said. In addition to the headache, other manifestations that may be present include pain or stiffness in the neck, nausea or emesis, diplopia due to unilateral or bilateral sixth nerve palsy, dizziness, changes in hearing, visual blurring, photophobia, intrascapular pain, facial numbness or weakness, galactorrhea, and radicular upper limb symptoms. Only rarely have patients exhibited stupor or encephalopathic manifestations, he added.

The headache derived from cerebrospinal fluid leaks may be characterized by cervical and/or intrascapular pain preceding the headaches for days or weeks; by lingering, non-orthostatic headaches preceding orthostatic headaches by days, weeks, or months; or by the acute, thunderclap-like onset of orthostatic headaches themselves. At times the orthostatic features are blurred into lingering chronic daily headaches, Dr. Mokri said. “If the patient’s headaches have evolved into a mild lingering headache with which he or she can live and get by with [intermittent] use of simple analgesics, so much the better,” he added. “But at the other extreme, there are patients who are virtually totally disabled by severe orthostatic headaches and have to remain bedridden almost all of the time or much of the time. In between, you have all kind of variations.”

Many treatments are employed for cerebrospinal fluid leak headaches, Dr. Mokri noted. The use of caffeine “has been somewhat effective in some of the patients, while analgesics and nonsteroidals are used by others. Corticosteroids have also been used, but the claims of effectiveness have been mostly anecdotal. Furthermore, some of the patients make spontaneous recovery after variable periods of time.”

If pain and symptoms persist without suitable relief from pharmacologic therapies, there are also invasive options that may be considered, Dr. Mokri observed. “Invasive therapy refers to an epidural blood patch or surgery,” he elaborated. “Fortunately, surgery only infrequently becomes necessary. The epidural blood patch is the most commonly used invasive therapeutic measure. Epidural infusions of fluid or fibrin glue have been utilized, but much less frequently and typically in cases where epidural blood patches have failed. In recommending and designing the treatment, one should consider the level of the patient’s discomfort, the nature of the headache, and its intensity. These factors should guide the aggressiveness of the therapeutic efforts,” he said.

THE VALSALVA DISGUISE

“The first patient was a previously healthy, right-handed man with a distant history of nasal polypectomate and partial turbinate removal. In 1997, at age 42, he began to note occipital headaches provoked by Valsalva-type maneuvers. These headaches were somewhat throbbing, each lasting 20 to 40 minutes,” Dr. Mokri reported. Magnetic resonance imaging (MRI) in March 1998 showed diffuse pachymeningeal gadolinium enhancement and descent of the cerebral pontis below the foramen magnum, as well as crowding of the posterior fossa and obliteration of the perichiasmatic cistern. A left frontal meningeal biopsy showed only normal dura with reactive meningeal cells. Microbiology studies were negative, and antibiotic treatments brought no relief. In late October 1998, ACT-myelogram showed a 5-mm meningeal direticulum at T12 and an 8-mm direticulum at T8, both on the left. An indium-111 radioisotope cisternography showed only slight asymmetry of uptake over the cerebral convexities.

“In January 1999, the patient was seen at Mayo Clinic Rochester. By this time the headaches had nearly resolved. The neurologic examination was normal. A repeat head MRI showed resolution of meningeal enhancement. The recommendation was to further observe rather than proceed with invasive therapeutic procedures,” Dr. Mokri said. During the next several weeks, the headaches completely resolved. However, several months later there was a recurrence of headaches and a reappearance of meningeal enhancement. “These headaches were less severe and were shorter in duration (five to 10 minutes). The patient had learned how to avoid provoking factors and how to cope with the headaches and decided not to subject himself to invasive procedures,” he added.

In July 1986, the patient was seen by a neurologist, and his neurologic examination was normal, Dr. Mokri continued. The headaches were diagnosed as benign exertional headaches. An unenhanced head MRI was normal. The patient was treated with indomethacin without benefit. A subsequent trial of long-acting propranolol (80 mg/d and later 120 mg/d) also offered no relief. Indomethacin was tried again with a dose of 25 mg tid and again there was no benefit. Thereafter, “the patient tried to avoid provoking factors as much as possible and lived with intermittent headaches when they occurred.”

In the late 1980s and early 1990s, “he began to make the observation that sometimes, with Valsalva-type maneuvers, a small amount of clear fluid leaked from the nose. Indium-111 radioisotope cisternography demonstrated abnormal tracer distribution with very little activity over the cerebral convexities even at 24 hours. Furthermore, radioactivity counts of the nasal pledgets showed increased activity in the right nasal pledgets, suggesting a right-sided cerebrospinal fluid leak,” Dr. Mokri observed.

As of December 2000, “the patient’s intermittent headaches had persisted, although he had learned how to avoid provoking factors as much as possible. His neurologic examination was normal and another gadolinium-enhanced MRI revealed no abnormalities. The patient did not wish to pursue more invasive studies as he thought he had learned to live with the problem.”

FINAL EVALUATION

With refinements and increased availability of MRI and with the greater awareness of physicians, an increasing number of patients with cerebrospinal fluid leak are diagnosed. “It is likely that this trend will continue for some time and that we will learn more about the spectrum of this entity as well as about cerebrospinal fluid dynamics and its implications,” Dr. Mokri added.

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