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When a patient presents to the office with a chief complaint of headache, the main concern is to differentiate between primary and secondary pathology because the evaluation and treatment pathway are affected by that initial determination.

During the visit, red flags can easily be identified by the following: a detailed history of the headache, including any previous headache, localization, acute changes, age of onset, severity, duration, triggers, and associated symptoms; family history; medications; medical history; and a detailed physical and neurologic examination. When appropriately done, the gathered information helps the provider to not only classify the presenting headache but also guide the clinical decision making.1

The red flags2 or concerning symptoms in children and adolescents are summarized as follows:

  1. New-onset headache (<6 months)

  2. Worst headache ever

  3. Persistent occipital headache

  4. Headache when waking the child from sleep

  5. Intractable vomiting

  6. Abnormal vital signs and/or physical examination (eg, fever, papilledema, abnormal neurologic examination, including neck stiffness)

  7. Sudden change in headache pattern

  8. No family history of headache

  9. A child younger than 6 years old with a history that does not fit all the International Classification of Headache Disorders, Third Edition (ICHD-3) criteria of headache

  10. Changes in mental status

  11. Prolonged aura

This chapter will summarize secondary headaches that can present to a pediatric neurology office, including headaches due to a central nervous system (CNS) vascular abnormality such as hemorrhages (eg, intracranial, subarachnoid, subdural), headaches associated with genetic disorders in childhood and adolescence (eg, mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes [MELAS], cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), headache occurring with intracranial pressure changes (idiopathic increase of cerebrospinal fluid [CSF] pressure and low-pressure headaches), headache secondary to inflammatory processes (infectious or noninfectious), and headache due to space-occupying lesions or congenital anomalies such as neoplasms and Chiari I malformation.3,4 Headache related to trauma and concussion will be reviewed in Chapter 16 in detail.3,4


Intracerebral, subarachnoid, and acute subdural hemorrhages and cervical arterial dissection are causes of secondary headache. Headache in any CNS hemorrhage is acute, very intense, and associated with other neurologic symptoms, including nuchal rigidity, mental status changes, motor or sensory deficits, and more specific neurologic abnormalities related to the localization of the hemorrhage. The headache usually occurs in close temporal relation to other intracranial hemorrhage symptoms and improves with the stabilization of the hemorrhage. It is usually described as a thunderclap headache that is worst on the day of onset and is localized to the site of the hemorrhage. The headache can be an early feature of hemorrhage, especially in cerebellar hemorrhage. As soon as an intracranial hemorrhage is suspected, imaging and neurosurgical evaluation are needed urgently because delay in the diagnosis could be clinically catastrophic. A noncontrast computed ...

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