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The vast majority of headaches are of the tension, migraine, and cluster types, which are classified as primary headaches and are discussed in Chapter 18. Unfortunately, many patients develop refractory headaches, which usually consist of one or more primary headache disorders complicated by analgesic medication overuse, poor coping patterns, or failure to identify triggers. In such cases, an interdisciplinary management approach is needed.

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Of particular concern to patients and clinicians are the secondary headaches, also known as organic headaches, accounting for fewer than 10% of all recorded headaches.1 By definition, they are symptomatic of underlying disease, structural pathology, or pain-inducing processes different from those traditionally ascribed to the primary headaches. Organic headaches may be secondary to elevated cerebrospinal fluid (CSF) pressure, known as benign intracranial hypertension or pseudotumor cerebri; to bleeding from congenital aneurysms or arteriovenous malformations (AVMs); to ischemic or hemorrhagic stroke; as well as to pain caused by mass lesions or mass effect, such as tumors, hematomas, AVMs, and trauma, or infectious processes such as meningitis, encephalitis, and cerebral abscesses. The clinical importance of organic headaches—despite their relatively low prevalence compared with primary headaches—illustrates important principles of diagnosis and treatment useful to the clinician prior to obtaining specialist consultation.

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This chapter focuses on the clinical signs, symptoms, and diagnostic workup of selected categories of secondary headaches. Cerebral tumors, stroke, subarachnoid hemorrhage, and vascular anomalies, spinal headache (i.e., spontaneous CSF leaks or those caused by lumbar puncture or epidural misplacement), and infection with the human immunodeficiency virus (HIV) are some of the vehicles used to discuss common clinical scenarios and approaches to decision making. The information that follows is based on both the published literature and our clinical experience as neurologists and headache and pain specialists.

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Special emphasis is placed on the importance of maintaining patient comfort, dignity, and self-esteem. This approach is crucial to the success of therapy, even if success is not always defined as “cure,” and is especially important in cases of terminal disease or those in which the primary diagnosis or headache symptom is disrupting patient and family dynamics.

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Differentiating secondary from primary headaches can be difficult. The quality of pain may be indistinguishable from that of migraine, tension-type, or other primary headaches. In such cases, the International Headache Society (IHS) states that the temporal relationship between the headache and underlying pathology should be the deciding factor.2 Preexisting headaches aggravated by an organic process are still considered primary. If the onset of headache occurs in close proximity to the underlying structural problem, it is considered secondary. Sometimes the question is merely academic or impossible to answer. For example, how does one classify long-standing, stereotypic, but side-locked migraines when a magnetic resonance imaging (MRI) scan of the head, obtained to evaluate the cause of new-onset seizures, reveals the presence of an AVM ipsilateral to the headache, and in a position to cause pain? Are the headaches then primary ...

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