Sudden Onset Chronic Daily Headache

ISSN 0017-8748 Headache doi: 10.1111/head.12724 VC 2015 American Headache Society Published by Wiley Periodicals, Inc. Residents and Fellows Headache Rounds: Sudden Onset Chronic Daily Headache Stephanie Wrobel Goldberg, MD; William Young, MD, FAHS, FAAN Key words: headache, spontaneous intracranial hypotension, low cerebrospinal fluid pressure, pseudo-Chiari, Trendelenburg position (Headache 2016;56:379-388) The following article is a Thomas Jefferson several emergency department visits, a head com- Headache Center headache rounds presentation. A puted tomography was performed revealing chronic 37-year-old woman, who reports a history of sinus disease that led her to sinus surgery with no chronic sinusitis, developed sudden onset headache improvement of symptoms. For several months, she 1 1/2 years prior to her initial presentation at the was unsuccessfully treated with preventive therapy Jefferson Headache Center. At that time she noted including topiramate, duloxetine, gabapentin, ona- acute severe pressure-like pain bilaterally in her botulinumtoxin A, and abortive therapy including neck, which radiated to her forehead above her triptans, nonsteroidal anti-inflammatory drugs, bar- eyebrows. She denied fever, rashes, or nasal dis- biturates, and muscle relaxants. Acupuncture and charge at the time. The pain was much more occipital nerve blocks provided limited relief. The intense than her usual “sinus headaches” and asso- patient denied a prior medical and family history of ciated with a positional component, occurring only migraines. Since the onset of symptoms, the patient upon standing, reaching a peak intensity of 10/10 continued to have intermittent explosive frontal that would only last seconds and remaining as a headaches that would be triggered by standing and dull milder continuous frontal pain for up to 12 improved upon lying down. As time elapsed, she hours. She had nausea and vomiting but no photo- also noticed suboccipital pain and neck discomfort phobia, phonophobia, or osmophobia. There was no rhinorrhea, conjuctival injection, or eye tearing. Section Editors: Morris Levin, MD (Department of Neurol- The pain was alleviated upon lying down. After ogy, University of California-San Francisco, San Francisco, CA, USA), and Matthew Robbins, MD (Department of Neurology, Montefiore Headache Center, Albert Einstein From the Department of Neurology, Thomas Jefferson College of Medicine, Bronx, NY, USA). Headache Center, 900 Walnut Street, Unit 200, Philadelphia, Conflict of Interest: None. PA, 19107, USA. William B. Young has the following disclosures: Address all correspondence to S. Wrobel Goldberg, Depart- ment of Neurology, Washington University School of Medi- Allergan: Consultant. cine, 517 S Euclid, Box 8111, St. Louis, MO 63110, USA. Research Support: Alder, Allergan, Autonomic Technology, Colucid, Dr. Reddy Laboratories, Electro Core, Eli Lilly, Accepted for publication August 18, 2015. Eneura Inc, Merck, St. Jude Medical. 379 380 February 2016 worsened by Valsalva maneuvers such as straining diagnosis of migraine headache was considered. As and coughing. She also reported bilateral upper her headaches progressed, this diagnosis became extremity paresthesias along with subjective even less likely especially with the significant pos- weakness. tural component, which is not typical for persons with migraine. Patients with migraine may report Query by Discussant William B. Young, MD, improvement of their headache upon lying down but FAHS, FAAN, Professor of Neurology, this should be further clarified as being related to Thomas Jefferson University, Philadelphia, PA rest and control of exacerbating factors, not neces- How would you initially approach this case? sarily to the supine position. In our case, failure of What would be the possible differential diagnosis? common abortive and preventive therapy, the abrupt Response by Stephanie W. Goldberg, Headache onset of pain without antecedent escalation of head- Fellow, Thomas Jefferson University, Philadelphia, ache frequency, and absent personal or family his- PA.—Several differential diagnoses should be con- tory of migraines also spoke against this diagnosis. sidered for a headache that is daily from onset Low pressure headache without apparent provo- including primary and secondary headaches depend- cation is termed spontaneous intracranial hypotension ing if an underlying disease process is present. (SIH) and usually presents as a postural headache – By definition, primary headache (such as migraine eased by lying down and worsened in the upright posi- and tension-type headache) is a category of disorders tion. SIH may present as a daily headache from onset unto itself, not a result of another underlying disease or not responsive to traditional treatment similar to process. Secondary headache, conversely, is a manifes- NDPH.2 When a prior procedure like a dural punc- tation of a separate underlying condition and, therefore, ture, trauma, or neurosurgical intervention is believed when suspected, requires more extensive investigation. to be the cause of the leak, the terminology changes When managing patients with headaches, the clinician to either postdural puncture headache or cerebrospi- should be attentive to signs and symptoms suggestive of nal fluid (CSF) fistula headache. secondary headaches also referred to as “red flags” A variety of conditions such as postural orthostatic especially as some of these conditions can be life threat- tachycardia syndrome (POTS), the syndrome of the ening. In our patient, the red flags included the abrupt trephined and colloid cyst of the third ventricle may onset of headaches that can be a clinical presentation of present with a positional headache that is not necessar- ruptured aneurysm and subarachnoid hemorrhage, ily caused by a CSF leak.3-5 Autonomic studies showing change in baseline headaches (sinus headaches), pres- evidence of orthostatic intolerance with an increase in ence of focal neurological complaints (upper extremity heart rate of 30 beats per minute helps establishing weakness and paresthesias), and headache triggered by the diagnosis of POTS. Syndrome of the trephined or Valsalva maneuver. Finally, an associated postural com- “sinking skin flap syndrome” is a rare complication fol- ponent warranted investigation of abnormal intracranial lowing a large craniectomy, with a sunken skin above pressure. the bone defect associated with many neurological New daily persistent headache (NDPH) is a pri- symptoms besides severe headache such as mental sta- mary headache syndrome marked by a unique tem- tus changes, focal deficits, and seizures.6 poral profile that is daily from onset. That being said, Further Comments on Differential Diagnosis as many primary and secondary headaches manifest as Time Elapsed and New Symptoms Developed – Dr. daily from onset, making NDPH a diagnosis of Young.—As time elapsed, patient developed Valsalva exclusion.1 induced headaches. Many headaches can be triggered Among the chronic primary headache disorders, or aggravated by this maneuver (such as coughing, migraine and tension-type headache rarely present sneezing, straining, heavy lifting, bending, pushing). as daily from onset and rather evolve from their epi- In congenital Chiari malformation type I, as the cere- sodic forms.2 Given our patient’s age, sex, and asso- bellar tonsils descend, by definition >5mm,thesubar- ciated symptoms of nausea and vomiting, an initial achnoid space at the craniocervical junction can be Headache 381 Table 1.—Differential Diagnosis of Sudden Onset Chronic Response by Dr. Goldberg.—First described by Daily Headache Schaltenbrand in 1938, SIH is thought to result from loss of cerebral spinal fluid through an occult breach Primary Headaches Secondary Headaches in the dura mater and, consequently, low CSF pressure.13 Most leaks occur typically at a spinal level and 14 New daily persistent Spontaneous intracranial only rarely from the skull base. It was only later, headache hypotension after 1950s, that new imaging technology such as radi- Provoked intracerebral hypotension oisotope cisternography and CT myelography sup- 15 Chronic migraine Colloid cyst of the third ported these findings. With the advance of magnetic ventricle resonance imaging (MRI), a broader clinical and radi- Chronic tension-type Postural orthostatic tachycardia 8 headache syndrome ological spectrum of the disorder was recognized Primary cough headache Chiari I or pseudo-Chiari although, unfortunately, the syndrome remains com- and other Valsalva (when associated 16 induced headaches with cerebrospinal fluid monly misdiagnosed. CSF hypovolemia is believed hypovolemia) to be the main pathogenetic factor of this disorder Primary thunderclap Secondary thunderclap while CSF opening pressure, MRI findings, and clini- headache headache: subarachnoid hemorrhage, cerebral venous cal features are highly variable and not necessarily sinus thrombosis, carotid abnormal.17 Some authors even question the termi- artery dissection, and pituitary apoplexy nology SIH and prefer alternative terms such as CSF volume depletion, CSF hypovolemia, or spontaneous CSF leak.18,19 The estimated prevalence of this condition is 1 in 50,000 adults,20 although the true incidence compressed leading to obstruction of CSF outflow. is unknown. Headache is usually occipital or suboccipital, of short Several authors have suggested that an underly- duration (less than 5 minutes), and can be provoked ing connective tissue disorder may lead to dural 7 by cough or other Valsalva-like

Load more