Introduction
First step: distinguishing primary from secondary CDH
Red flag | Consider | Possible investigation(s) |
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Sudden-onset headache | Subarachnoid haemorrhage, bleed into a mass or AVM, mass lesion (especially posterior fossa) | Neuroimaging, lumbar puncture (after neuroimaging evaluation) |
Worsening-pattern headache | Mass lesion, subdural haematoma, medication overuse | Neuroimaging |
Headache with cancer, HIV or other systemic illness (fever, neck stiffness, cutaneous rash) | Meningitis, encephalitis, Lyme disease, systemic infection, collagen vascular disease, arteritis | Neuroimaging, lumbar puncture, biopsy, blood tests |
Focal neurologic signs, or symptoms other than typical visual or sensory aura | Mass lesion, AVM, collagen vascular disease | Neuroimaging, collagen vascular evaluation |
Papilloedema | Mass lesion, pseudotumour, encephalitis, meningitis | Neuroimaging, lumbar puncture (after neuroimaging evaluation) |
Triggered by cough, exertion or Valsalva | Subarachnoid haemorrhage, mass lesion | Neuroimaging, consider lumbar puncture |
Headache during pregnancy or post-partum | Cortical vein/cranial sinus thrombosis, carotid dissection, pituitary apoplexy | Neuroimaging |
Headache associated with vascular disorders |
Cerebrovascular disease including carotid artery dissection and arteriovenous malformation |
Arteritis including giant cell arteritis |
Headache associated with non-vascular intracranial disorders |
Low CSF pressure syndrome (spontaneous or post-traumatic CSF “leak”) |
High CSF pressure without papilloedema |
Intracranial: Lyme disease, human immunodeficiency virus, encephalitis, fungal meningitis, etc. |
Headache associated with substances or their withdrawal |
Overuse of acute headache medications (rebound or toxic drug overuse syndromes) |
Headache associated with cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures |
Otolaryngologic disease, including chronic sphenoid sinusitis (or other sinus disease) |
Nasopharyngeal disorders, including carcinoma |
Disorders of the trigeminal nerve, including dental and oral disease, jaw pathology |
Subacute angle closure glaucoma, optic neuritis and other ocular disorders |
Occipitocervical disease, including Arnold-Chiari Malformation Type I; upper cervical joint, root or nerve (neuralgic) syndromes |
Headache associated with non-cephalic infection, metabolic or systemic disturbances |
Hepatitis, renal disease, B12 deficiency, anaemia, exposure to carbon monoxide and other toxins |
Hormonal disturbances/endocrinologic disease (oestrogen, thyroid disease, hyperprolactinaemia, etc.) |
Vasculitis/rheumatic/connective tissue disorders |
Miscellanea |
Mediastinal and thoracic processes including angina, mass lesions, superior vena cava syndrome |
Red flags triggered by the temporal profile of headache
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Sphenoid sinusitis may cause NDPH and may be missed radiologically unless appropriate studies are performed [10].
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Headaches that start after age 55 suggest an organic disorder for CDH, such as a mass lesion or giant cell arteritis [11]. Giant cell arteritis is often under-diagnosed, and is an important cause of preventable blindness in the elderly.
Red flags triggered by concurrent events or provoking activity
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Chronic headaches (NDPH) that occur in the peripartum period may be due to dural sinus thrombosis [12].
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Orthostatic CDH suggests a low CSF pressure headache (from a spontaneous CSF leak, a previous lumbar puncture, or an epidural block) [13].
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CDH exacerbated by straining, coughing or sneezing suggests a hindbrain malformation, occipitocervical junction disorder or increased intracranial pressure [14].
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CDH that is worse in the morning in the absence of medication overuse, that may suggest raised intracranial pressure. CDH with medication overuse is often worse in the morning.
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In contrast, subjects with CDH that is better in the morning and worse at night that may suggest low CSF volume headache [13].
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Similarly, CDHs that are worse on awakening sometimes occur with obstructive sleep apnoea [13].
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Search for opportunistic infections, including toxoplasmosis and cryptococcal meningitis, in patients who may have HIV infection or HIV risk factors [18].
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A previous mild to moderate head or neck injury is frequently overlooked in the standard history, despite the fact that it can render an individual refractory to standard headache treatments [19]. A diagnosis of post-traumatic headache may lead to additional treatment including trigger point injections and facet joint and cervical nerve blocks.
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Consider ocular disturbances, such as subacute angle closure glaucoma, or infection, if the CDH is worse in the periorbital region [22].
Second step: classifying primary CDH based on duration and frequency (Fig. 2)
The chronic daily headaches of shorter duration (Fig. 3)
Chronic daily headaches of long duration (Fig. 4)
Chronic ronic or transformed migraine
Silberstein-Lipton From 1996 | ICHD-2R | |||
---|---|---|---|---|
Transformed migraine | Chronic migraine | |||
A. | Daily or almost daily (>15 days a month) head pain for >1 month |
Diagnostic criteria:
| ||
B. | Average headache duration of >4 h per day (if untreated) | Headache on ≥15 days/month for >3 months. | ||
C. | At least one of the following: | Occurring in a patient with at least 5 prior migraine attacks. | ||
1. | History of episodic migraine meeting any IHS criteria 1.1–1.6 | On ≥8 days per month, for at least three months, headache fills criteria C1 and C2 | ||
2. | History of increasing headache frequency with decreasing severity of migrainous features over at least 3 months | |||
3. | Headache at some time meets IHS criteria for migraine 1.1–1.6 other than duration |
1.
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Unilateral
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Throbbinge
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Moderate or severe
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D. | Does not meet criteria for new daily persistent headache (4.7) or hemicrania continua (4.8) |
Aggravated by physical activity
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Nausea and/or vomiting
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Photophobia and phonophobia
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2. | Treated or relieved with triptans or ergotamine compounds. | |||
D. | No medication overuse and not attributable to another causative disorder. |
ICHD-2 code | Diagnosis | Amount of medication |
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8.1 | Ergotamine overuse headache | Ergotamine intake on ≥10 days per month on a regular basis for >3 months |
8.2 | Triptan overuse headache | Triptan intake (any formulation) on ≥10 days per month on a regular basis for >3 months |
8.3 | Analgesic overuse headache | Intake of simple analgesics on ≥15 days per month on a regular basis for >3 months |
8.4 | Opioid overuse headache | Opioid intake on ≥10 days per month on a regular basis for >3 months |
8.5 | Combination analgesic overuse headache | Intake of combination analgesic medications on ≥10 days per month on a regular basis for >3 months |
8.6 | MOH attributed to combination of acute medications | Intake of any combination of ergotamine, triptans, analgesics, and/or opioids on ≥10 days per month on a regular basis for >3 months without overuse of any single class |
8.7 | Headache attributed to other medication overuse | Regular overuse for >3 months of a medication other than those described above |
8.8 | Probable MOH | Overused medication has not yet been withdrawn or medication overuse has ceased within the last 2 months but headache has not so far resolved or has reverted to its previous pattern |