Overview
Definition:
New daily persistent headache (NDPH) is a primary headache disorder characterized by the abrupt onset of a daily headache that has persisted for more than three months, with a distinct change from prior headache patterns.
Epidemiology:
NDPH is relatively uncommon but represents a significant proportion of chronic daily headaches in pediatric populations
Its incidence is not precisely known, but it affects children and adolescents, with no clear gender predilection in this age group, although adult studies show a female predominance.
Clinical Significance:
NDPH can be disabling for affected children, leading to significant school absenteeism, reduced academic performance, and impaired quality of life
Prompt and accurate diagnosis is crucial to rule out secondary causes and initiate appropriate management to alleviate suffering and prevent chronicity.
Clinical Presentation
Symptoms:
Abrupt onset of a new daily headache within 24 hours
Headache occurs every day, or almost every day
Headache persists for more than three months
Typical headache characteristics include being bilateral, pressing or tightening in quality, mild to moderate in intensity, and not aggravated by routine physical activity
Some patients may experience associated symptoms like photophobia or phonophobia.
Signs:
Physical examination, including a thorough neurological assessment, is often normal in children with NDPH
Absence of focal neurological deficits, papilledema, or signs of meningeal irritation is reassuring and points towards a primary headache disorder
Fundoscopy to rule out papilledema is essential.
Diagnostic Criteria:
According to the International Classification of Headache Disorders, 3rd Edition (ICHD-3), NDPH requires: 1
A new daily headache present for >3 months
2
The headache occurs daily and persistently
3
At least one of the following: a) The headache has not previously occurred
b) The headache has characteristics (e.g., location, quality, intensity, duration) that are different from any previous headaches
4
The headache is not better accounted for by another ICHD-3 diagnosis.
Diagnostic Approach
History Taking:
Detailed history is paramount
Ascertain the exact onset date and nature of the headache change
Inquire about triggers, exacerbating and relieving factors, associated symptoms (nausea, vomiting, photophobia, phonophobia, visual disturbances), impact on daily activities (school, sleep, social life), and prior headache history
Crucially, identify red flags: sudden severe onset ("thunderclap"), fever, neurological deficits, papilledema, systemic illness, new onset in a very young child (<5 years), or change in headache pattern suggestive of serious underlying pathology.
Physical Examination:
A complete physical examination, including vital signs, anthropometric measurements, and detailed neurological assessment, is mandatory
This includes assessment of cranial nerves, motor and sensory function, reflexes, coordination, and gait
Fundoscopic examination is critical to exclude papilledema
Examination of the neck for stiffness and palpation of the head and neck for tenderness are also important.
Investigations:
Neuroimaging, typically MRI brain with contrast, is often indicated in the initial evaluation of children with new daily persistent headache to rule out structural lesions, inflammation, or other secondary causes
EEG may be considered if there are seizure-like symptoms
Lumbar puncture may be performed if meningitis or encephalitis is suspected, or if neuroimaging is equivocal
Blood tests (CBC, ESR, CRP) may be useful to investigate for inflammatory or infectious causes.
Differential Diagnosis:
Conditions to consider include: Migraine (especially chronic migraine), tension-type headache (chronic), medication overuse headache, secondary headaches due to intracranial pathology (tumor, arteriovenous malformation, hydrocephalus, meningitis, encephalitis), post-traumatic headache, and headaches associated with systemic illness or infections
Differentiating NDPH from chronic migraine can be challenging, but NDPH is typically not throbbing and not aggravated by routine physical activity.
Management
Initial Management:
Establish a diagnosis and reassure the patient and family if a benign cause is identified
Address any immediate concerns or significant functional impairment
Educate the patient and family about the chronic nature of the headache and the management plan.
Medical Management:
Treatment is often individualized and may involve a combination of acute and prophylactic strategies
Acute treatment can include NSAIDs (e.g., ibuprofen, naproxen) or triptans if migraine features are present
Prophylactic medications are frequently necessary and include topiramate (initial dose 0.5-1 mg/kg/day, titrated up to 1-2 mg/kg/day bid, max 200 mg/day), amitriptyline (initial dose 0.25-0.5 mg/kg/day, titrated up to 1-2 mg/kg/day, max 50 mg/day), or propranolol (0.5-1 mg/kg/day divided tid, max 40 mg/day)
Fluoxetine or venlafaxine may also be considered
Treatment trials should be for at least 6-8 weeks.
Surgical Management:
Surgical interventions are generally not indicated for NDPH itself
They are reserved for cases where a specific underlying secondary cause is identified and amenable to surgical correction, such as removal of a brain tumor or treatment of an arteriovenous malformation
Operative decompression for specific conditions like Chiari malformation might be considered if indicated.
Supportive Care:
Behavioral therapies are an integral part of management
Cognitive Behavioral Therapy (CBT), biofeedback, and relaxation techniques can help children cope with chronic pain and reduce disability
Lifestyle modifications, including regular sleep hygiene, a balanced diet, and adequate hydration, are important
Encouraging regular physical activity, within limits of headache exacerbation, is also beneficial
Multidisciplinary pain clinics can offer comprehensive support.
Complications
Early Complications:
Significant functional impairment, leading to missed school days, social withdrawal, and emotional distress (anxiety, depression).
Late Complications:
Development of medication overuse headache if acute treatments are used too frequently
Chronic pain syndrome, leading to long-term disability and reduced quality of life
Impact on academic and personal development.
Prevention Strategies:
Early diagnosis and initiation of appropriate prophylactic medical and behavioral management
Avoiding frequent use of acute headache medications
Establishing healthy lifestyle habits
Regular follow-up to monitor treatment efficacy and adherence.
Prognosis
Factors Affecting Prognosis:
The prognosis for NDPH is variable
Some children experience resolution within a few years, while others have persistent headaches
Factors that may influence prognosis include the presence of migraine features, severity of pain, impact on daily life, and response to treatment
Early diagnosis and intervention are generally associated with better outcomes.
Outcomes:
Complete remission is possible in a significant proportion of children, although it can take months to years
Many children achieve substantial improvement in headache frequency and intensity with appropriate management, allowing for a return to normal daily functioning
Some individuals may experience a chronic or relapsing course.
Follow Up:
Regular follow-up appointments with a pediatric neurologist or headache specialist are crucial, typically every 3-6 months, to assess treatment response, monitor for side effects, adjust medications, and address any new concerns
Long-term monitoring is necessary as the course of NDPH can evolve.
Key Points
Exam Focus:
Differentiate NDPH from chronic migraine
Recognize red flags requiring urgent neuroimaging
Understand the ICHD-3 diagnostic criteria
Know common prophylactic agents (topiramate, amitriptyline) and their typical pediatric dosing ranges
Emphasize the importance of a thorough history and normal neurological exam.
Clinical Pearls:
The key to diagnosing NDPH is the *abrupt onset* and *persistence* of a *new* daily headache pattern
Always consider secondary causes even if the presentation is typical for primary headache
Behavioral therapies are as important as pharmacotherapy
Collaborate with schools to manage absences and academic support.
Common Mistakes:
Failing to inquire about the exact onset and nature of headache change
Overlooking red flags suggesting secondary headaches
Inadequate trial duration for prophylactic medications
Relying solely on pharmacological management without incorporating behavioral strategies
Dismissing the impact of chronic headache on a child's life.