Daily Headache that Won’t Go Away

A 14-year-old girl presents in October for an initial evaluation in the Multidisciplinary Headache Clinic at CHOP for headaches that began in August of this year. She describes that she was previously healthy and after a week of summer camp in Massachusetts, had a fever with cough that persisted for more than a month. Her fever seemed to respond to a course of antibiotics, but the cough was incessant and nagging for a total of 6 weeks. She noted that toward the end of the illness she had a headache that seemed to get increasingly worse as her cold symptoms improved.

She describes throbbing pain in the front and sides (bilateral frontotemporal area), sometimes spreading to the back of the head (occiput). She has already missed 15 days of school because she is unable to make it through the whole day. The headache is made worse by loud noises and bright lights. Her pain is minimal upon awakening and seems to get worse by the end of the day. The pain is made worse by exercise, and she has noticed that on the day she has gym glass she almost always has to call her parent to take her home about an hour later.

She can recall the exact date that the headache started. Initially she and her parents thought it was a “normal headache” so they did not seek care. She has recognized that as long as she rests and avoids exercise this headache is manageable. It is worsened by an upright position and improved by laying down. She has had a daily headache since August.

The patient and her parents deny any significant stressors and report that her mood has been positive despite the persistent pain. There is no known history of trauma, though she played competitive hockey last winter. She endorses dizziness, problems concentrating, visual blurring with the headache, and chronic nausea. She is not taking any medications regularly. Her general medical and neurologic exams are otherwise completely normal except for findings of hyper-extensibility and flexibility.

Discussion

This patient has new daily persistent headache (NDPH), a descriptive diagnosis for a headache that is constant from the start and lasts for at least 3 months. NDPH is a subtype of chronic daily headache, which occurs in at least 1.5% of children. NDPH usually has some features of tension-type headache with bilateral, nonpulsatile pain, and superimposed migraine with photophobia, phonophobia, nausea, and worsening with activity. The important historical feature in NDPH is the abrupt onset of pain. This may be associated with a viral infection, extracranial surgery, or minor head trauma. Many patients report comorbid sleep disturbance, mood problems, and dizziness.

Given its abrupt onset, NDPH is a diagnosis of exclusion, and it is important to exclude sinusitis, meningitis, tumor, idiopathic intracranial hypertension, cerebral sinus venous thrombosis, carotid dissection, and spontaneous intracranial hypotension, especially if the patient presents within 3 months of headache onset.

Figure 2: MRI of the brain demonstrate extensive and diffuse thickening of the meninges which have high T2 signal and extensive enhancement. The venous structures appear engorged and the brain is sagging downwards. We did some imaging and were able to confirm a diagnosis of spontaneous intracranial hypotension, with imaging showing abnormal enhancement and downward placement of brain structures (See Figure 2). She was successfully treated with an epidural blood patch and has been headache free since then.

This was a fortunate case because the cause of NDPH was found. In most cases, the actual cause is not identified. In those cases, it can self-resolve in a few months or it can be refractory to treatment for years. Because we cannot predict when the headache will improve, we recommend multimodal treatment for all patients.

Initial management should include reassurance, encouragement to resume normal activities (which will help to control symptoms long term), guidance on sleep hygiene, hydration, and diet. Frequent use of analgesic medications can exacerbate the pain over time, so these should be limited to 3 days per week or less. Medications may help to reduce the intensity of the constant headache and decrease the frequency of severe headaches, but often these take several weeks to work. Sometimes procedures such as infusions, anesthetic nerve blocks, and botulinum toxin injections may be used for symptom relief. Exciting new therapies have been approved in the last year which target calcitonin gene-related peptide (CGRP); these are the first ever migraine-specific therapies approved for this indication.