Color Atlas of Neurology (Thieme 2004)

Central Nervous System

Headache

Tension Headache

Tension headache often involves painful cervical muscle spasm, may change with the weather, and is frequently ascribed by patients and others to cervical spinal degenerative disease, visual disturbance, or life stress. It consists of a bilateral, prominent nuchal pressure sensation that progresses over the course of the day. Patients may report feeling as if their head were being squeezed in a vise or by a band being drawn ever more tightly around it, or as if their head were about to explode, though the pain is rarely so severe as to impede performance of the usual daily tasks, e. g., at work. It may be accompanied by malaise, anorexia, lack of concentration, emotional lability, chest pain, and mild hypersensitivity to light and noise. Unlike migraine, it is not aggravated by exertion (e. g., climbing stairs), nor does it produce vomiting or focal neurological deficits. It can be episodic (!15 days/month, pain lasting from 30 minutes to 1 week) or chronic ("15 days/month for at least 6 months). Some patients suffer from pericranial tenderness (posterior cervical, masticatory, and cranial muscles). Isolated attacks of sudden, stabbing pain (ice-pick headache) may occur on one side of the head or neck. Tension headache rarely wakes the patient from sleep. Its cause usually cannot be determined, though it may be due to disorders of the temporomandibular joint or psychosomatic troubles such as stress, depression, anxiety, inadequate sleep, or substance abuse. Tension headache combined with migraine is termed combination headache. Pathogenesis. It is theorized that diminished activity of certain neurotransmitters (e. g., endogenous opioids, serotonin) may lead to abnormal nociceptive processing (p. 108) and thus produce a pathological pain state.

Headache Due to Vascular Processes (Other than Migraine)

the actual vascular event (arterial dissection, arteriovenous malformation, vasculitis), may occur simultaneously with the event (subarachnoid hemorrhage, intracerebral hemorrhage, epidural hematoma, cerebral venous thrombosis, giant cell arteritis, carotidynia, venous outflow obstruction in goiter or mediastinal processes, pheochromocytoma, preeclampsia, malignant hypertension), or may follow the event (subdural hematoma, intracerebral hemorrhage, endarterectomy).

Chronic Daily Headache

Rational treatment is based on the classification of primary daily headache by clinical characteristics (see Table 23, p. 373), and of secondary (symptomatic) headache by etiology.

The nociceptive innervation of the extracranial and intracranial vessels is of such a nature that pain arising from them is often projected to a site in the head that is some distance away from

182the responsible lesion. Thus, specific diagnostic studies are usually needed to pinpoint the location of the disturbance. The pain may precede

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Headache

			Persistent, variably severe headache
Depression			Transient
			stabbing pain
Anxiety
Stress
			Episodic
Noise
Alcohol
Medications			Chronic
	Tension headache

Carotid artery (common, external, internal)		Internal carotid a., cavernous sinus

Vertebral, basilar, posterior cerebral arteries;	Superior sagittal sinus
transverse/sigmoid sinus
Referred pain due to cerebrovascular lesions

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183

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Central Nervous System

Headache

Migraine

Migraine is a periodic headache often accompanied by nausea and sensitivity to light and noise (photophobia and phonophobia). A typical attack consists of a prodromal phase of warning (premonitory) symptoms, followed by an aura, the actual headache phase, and a resolution phase. Attack characteristics often change over time. Attacks often tend to occur in the morning or evening but may occur at any time. They typically last 4–72 hours.

! Symptoms and Signs

Prodromal phase. The migraine attack may be preceded by a period of variable prodromal phenomena lasting a few hours to two days. Most patients complain of sensitivity to smells and noise, irritability, restlessness, drowsiness, fatigue, lack of concentration, depression, and polyuria. In children, the chief complaints are abdominal pain and dizziness.

Aura. This is the period preceding the focal cerebral symptoms of the actual migraine headache. Some patients experience attacks without an aura (common migraine), while others have attacks with an aura (classic migraine) that develops over 5–20 minutes and usually lasts less than one hour, but may persist as long as one week (prolonged aura). In some cases, the aura is not followed by a headache (“migraine equivalent”). Auras typically involve visual disturbances, which can range from undulating lines (resembling hot air rising), lightning flashes, circles, sparks or flashing lights (photopsia), or zig-zag lines (fortification figures, teichopsia, scintillating scotoma). The visual images, which may be white or colored, cause gaps in the visual field and usually have scintillating margins. Unilateral paresthesiae (tingling or cold sensations) may occur. Emotional changes (anxiety, restlessness, panic, euphoria, grief, aversion) of variable intensity are relatively common.

often accompanied by anorexia, malaise, nausea, and vomiting.

Resolution phase. This phase is characterized by listlessness, lack of concentration, and increased pain sensitivity in the head.

! Pathogenesis

During the interval between attacks, various disturbances (genetically determined) may be observed, e. g., cerebral hypomagnesemia, elevated concentration of excitatory amino acids (glutamate, aspartate), and increased reactivity of cranial blood vessels. The cumulative effect of these disturbances is a heightened sensitivity to nociceptive stimuli (migraine pain threshold). Impulses from the cortex, thalamus, and hypothalamus activate the so-called migraine center responsible for the generation of migraine attacks, putatively located in the brain stem (serotonergic raphe nuclei, locus ceruleus). The migraine center triggers cortical spreading depression (suppression of brain activity across the cortex) accompanied by oligemia, resulting in an aura. Trigeminovascular input from meningeal vessels is relayed to the brain stem, via projecting fibers to the thalamus and then, by the parasympathetic efferent pathway, back to the meningeal vessels (trigeminal autonomic reflex circuit). Perivascular trigeminal C-fiber endings (trigeminovascular system) are stimulated to release vasoactive neuropeptides such as substrate P, neurokinin A, and calcitonin gene-regulated polypeptide (CGRP), causing a (sterile) neurogenic inflammatory response. Vasoconstriction and vascular hyperesthesia with subsequent vasodilatation spread via trigeminal axon reflexes. The perception of pain is mediated by the pathway from the trigeminal nerve to the nucleus caudalis, thalamus (p. 94) and cortex. Trigeminal impulses also reach autonomic centers.

Headache phase. Most patients (ca. 60%) complain of pulsating, throbbing, or continuous pain on one side of the head (hemicrania). Others have pain in the entire head, particularly behind

184the eyes (“as if the eye were being pushed out”), in the nuchal region, or in the temples. Migraine headache worsens on physical exertion and is

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			Headache
		Interval between attacks
	Migraine attacks
		Headache phase
	Prodromal phase	Aura	Resolution phase
				System
				Nervous
			Thalamocortical	Central
	Triggers		projections
	Trigeminal lemniscus		Thalamus
	Cerebral cortex
	Hypothalamus
	Locus ceruleus, raphe nuclei
	Trigeminal nerve
	Spinal tract of trigeminal nerve
		Fortification
		spectra
		Dura mater	Aura
		Perivascular
			(spreading cortical
		trigeminal axons
			depression)
		Luminal narrowing
			Nucleus caudalis
			Nausea, vomiting,
			autonomic disturbances
			Platelets (serotonin
			release)
	Axon reflexes,	P	Vasodilatation,
			extravasation of plasma
	neuropeptide release	ain		185
			(via NO, neuropeptides)
	Trigeminovascular system

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Headache

Trigeminal Neuralgia

		Trigeminal neuralgia (tic douloureux) is charac-
		terized by the sudden onset of excruciating, in-
		tense stabbing pain (during waking hours).
		Several brief attacks (!2 minutes each) gener-
		ally occur in succession. The pain is almost al-
		ways precipitated by a trigger stimulus or activ-
		ity (e. g., chewing, speaking, swallowing, touch-
		ing the face, cold air, tooth brushing, shaving)
		and is located in the distribution of one or two
	System	branches of the trigeminal nerve, usually V/2
		and/or V/3. Involvement of	V/1, all three
		branches, or both sides of the face is uncommon.
		The attacks may persist for weeks to months or
	Nervous	may spontaneously remit for weeks, or even
		taken for odontogenic pain, sometimes result-
		years, before another attack occurs. Trigeminal
		neuralgia in the V/3 distribution is often mis-
	Central	ing in unnecessary tooth extraction. Typical (id-
		iopathic) trigeminal neuralgia must be distin-
		guished from secondary forms of the syndrome
		(see below).
		Pathogenesis. Idiopathic trigeminal neuralgia
		much evidence points to microvascular com-
		pression of the trigeminal nerve root (usually by
		a branch of the superior cerebellar artery)
		where it enters the brain stem, leading to the
		development of ephapses or suppression of cen-
		tral inhibitory mechanisms.	Symptomatic

trigeminal neuralgia cerebellopontine angle tumors, multiple sclerosis, vascular malformations.

Cluster Headache (CH)

Episodic cluster headache. Attacks of very severe burning, searing, stabbing, burning, needlelike, or throbbing pain develop over a few minutes on one side of the head, behind or around the eye, and may extend to the forehead, temple, ear, mouth, jaw, throat, or nuchal region. If untreated, attacks last ca. 15 minutes to 3 hours. They are predominantly nocturnal, waking the patient from sleep, but can also occur during the day. Attacks come in episodes (clusters) consisting of 1–3 daily bouts of pain for up to 8 weeks. A seasonal pattern of occur-

186rence may be observed. During a cluster, the pain can be triggered by alcoholic drinks,

histamines, or nitrates. Temporal pressure or the

application of heat to the eye may alleviate the pain. Unlike migraine patients, who seek peace and quiet, these patients characteristically pace restlessly, and may even strike their aching head with a fist. The headache may be accompanied by ipsilateral ocular (watery eyes, conjunctival injection, incomplete Horner syndrome, photophobia), nasal (nasal congestion, rhinorrhea), and autonomic manifestations (facial flushing, tenderness of temporal artery, nausea, diarrhea, polyuria, fluctuating blood pressure, cardiac arrhythmia). Cluster headache is more common in men.

Chronic cluster headache. Attacks do not occur in clusters, but rather persist for more than one year at a time, punctuated by remissions lasting no longer than two weeks. Chronic cluster headache may arise primarily, or else as a confluence of clusters in what began as episodic cluster headache.

Pathogenesis. One hypothesis attributes CH to dilatation of the carotid artery within the carotid canal, causing compression of the periarterial sympathetic plexus. There is also evidence suggesting a role for inflammatory dilatation of the intracavernous venous plexus. The result is abnormal function of the sympathetic and parasympathetic fibers in the region of the cavernous sinus ( autonomic dysfunction, activation of trigeminovascular system).

Chronic Paroxysmal Hemicrania (CPH)

CPH is a very rare condition characterized by the daily onset of pain similar to that of cluster headache. The daily attacks of CPH are much more frequent (10–20 times/day) and shorter (5–30 minutes) than those of cluster headache. The pain of CPH typically responds to indomethacin.

Sinus Headache

The pain of frontal, sphenoid, or ethmoid nasal sinusitis is usually felt in the middle of the forehead and above the eyes. That of maxillary sinusitis radiates to the upper jaw and zygomatic region and worsens when the patient bends forward.

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Headache

Brief paroxysms of pain

Precipitating factors (triggers)

Trigeminal neuralgia

Cluster
			Prominent temporal artery
May be precipitated		Ptosis, miosis, reddening of eyes
by triggers					Lacrimation
					Rhinorrhea
		Cluster headache

Increasing pain intensity

Frontal sinus

Maxillary sinus

Central Nervous System

187

Sinus headache

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Central Nervous System

Headache

Nociceptive Transmission

The brain tissue itself is insensitive to pain. The major cranial and proximal intracranial vessels and dura mater of the supratentorial compartment derive nociceptive innervation from the ophthalmic nerve (V/1, p. 94), while those of the posterior fossa are innervated by C2 branches. Because nociceptive impulses from the anterior and middle fossae, the venous sinuses, the falx cerebri, and the upper surface of the tentorium travel through V/1, the pain that is experienced is referred to the ocular and frontoparietal regions; similarly, pain arising from the lower surface of the tentorium, the posterior cranial fossa, and the upper 2–3 cervical vertebrae (mediated by C2) is referred to the occipital and nuchal region. Small regions of the dura mater are innervated by CN IX and X; pain arising here is, accordingly, referred to the throat or ear. These neuroanatomical connections also explain the referral of pain from the upper cervical region to the eye (shared trigeminal innervation), and why tension and migraine headache can cause pain in the neck.

Cervical Syndrome (Upper Cervical

Syndrome)

Cervical syndrome typically causes pain in the frontal, ocular, and nuchal regions. The pain is usually continuous, without any circadian pattern, but may be more severe during the day or night. It may be worsened by active or passive movement of the head. It is usually due to a lesion affecting the C2 root and is characterized by muscle spasm, tenderness, and restricted neck movement. The diagnosis is based on the typical clinical findings, and cannot be based solely on radiographic evidence of degenerative disease of the cervical spine. For other causes of neck pain, see Table 23 (p. 373). For cervical distortion (whiplash), see p. 272. For Posttraumatic headache, see p. 270.

cocaine, marijuana, nitrates, and dihydropyridines (calcium antagonists). The headache is usually a pressing, piercing, or pulsating pain, and is typically bifrontal or frontotemporal. It may be accompanied by nausea, chest tightness, dizziness, abdominal complaints, lack of concentration, or impairment of consciousness.

Rebound headache. Persons suffering from recurrent or chronic headache are at risk for the excessive or uncontrolled use of medications, singly or in combination (analgesics, benzodiazepines, ergot alkaloids, combined preparations). This may result in daily rebound headache, persisting from morning to night and characterized by pressurelike or pulsating, unilateral or bilateral pain, accompanied by malaise, nausea, vomiting, phonophobia, and photophobia. Patients may also complain of lack of concentration, disturbed sleep, blurred or flickering vision, a feeling of cold, and mood swings. These patients change medications frequently and tend to take medication even at the first sign of mild pain, because they fear a recurrence of severe pain. Eventually, drug tolerance develops, resulting in persistent headache. The original migraine or tension headache may be largely masked by the rebound headache. Other drug side effects may include ergotism, gastritis, gastrointestinal ulcers, renal failure, physical dependence, and epileptic seizures (withdrawal seizures).

Substance-Induced Headache

Acute headache can be induced by a number of

188vasoactive substances. Triggers include alcohol consumption or withdrawal (“hangover”), caffeine or nicotine withdrawal, sodium glutamate,

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Headache

Ophthalmic nerve

Vagus nerve Lesser occipital n.

Greater occipital n.

Glossopharyngeal n.

Referred pain

Pain latency

Cervical syndrome

Medications

Episodic

Alcohol

Chronic

Illegal drugs

Substance-induced headache

Central Nervous System

189

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Central Nervous System

190

Headache

Treatment

The proper treatment of headache depends on its cause. Episodic or chronic tension headache and migraine are by far the most common types of headache. Structural lesions are a rare cause of headache (!5% of all headaches); such headaches typically start suddenly, worsen quickly, and represent a new type of pain that the patient has never had before. If a structural lesion is suspected, neuroimaging studies should be performed.

! General Treatment Measures

Good patient–physician communication is essential for the diagnosis and treatment of head-

ache. The most important clues to differential diagnosis are derived from the case history. The medication history must be obtained, and psychological factors must also be considered; headache is often associated with anxiety, e. g., fear of a brain tumor, of a mental illness, or of not having one’s complaints taken seriously. Patients must be instructed how they themselves can improve their symptoms (behavioral modification) through lifestyle changes (e. g., avoidance of alcohol, dietary changes, physical exercise, adequate sleep) and nonpharmacological measures (relaxation training, biofeedback, stress management, keeping a pain diary).

! Acute Treatment

Type of Headache	General Measures	Pharmacotherapy
Episodic tension headache	Behavioral therapy, ice packs	Peppermint oil to forehead and temples;
		aspirin, acetaminophen, ibuprofen, or
		naproxen
Migraine	Rest, ice packs	Antiemetic (metoclopramide or domperi-
		done) + aspirin or acetaminophen; if ineffec-
		tive, triptans1
Cluster headache	Hot compresses	Oxygen inhalation; if ineffective, triptan s.c.
		or ergotamine
Chronic paroxysmal		Indomethacin
hemicrania
Trigeminal neuralgia	Avoidance of triggers	Carbamazepine, gabapentin, phenytoin, ba-
		clofen, or pimozide2

1 This group includes sumatriptan, almotriptan, eletriptan, frovatriptan, naratriptan, rizatriptan, and zolmitriptan. 2 Patients with primary or secondary resistance to medical treatment should be treated neurosurgically (percutaneous thermocoagulation or retroganglionic glycerol instillation; microvascular decompression).

! Prophylaxis

Type of Headache	General Measures	Pharmacotherapy
Episodic or chronic tension	Behavioral therapy	Tricyclic antidepressants (e. g., amitryptiline,
headache		doxepin, amitryptiline oxide)
Migraine	Behavioral therapy	1st line: Beta-blockers (e. g., metoprolol, pro-
		pranolol); 2nd line: flunarizine or valproate;
		3rd line: methysergide or pizotifen
Episodic cluster headache	Avoidance of alcohol (during	Prednisone, ergotamine, verapamil, methy-
	cluster), nitrates, histamines,	sergide, or lithium
	and nicotine
Chronic cluster headache		Lithium, verapamil, or pizotifen

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Headache

YES

Neck stiffness

Normal

NO

Migraine

Tension headache Substance-induced headache (nitrates, glutamate, analgesics) Sinusitis

Cervical syndrome Temporal arteritis After lumbar puncture Systemic lupus erythematosus

Diagnostic classification

(acute or subacute headache)

Neurological examination is normal

Neurologic

deficits

Diagnostic classification

(facial pain)

1Computed tomography

2Subarachnoid hemorrhage (SAH), intracerebral/intraventricular hemorrhage

			Hemorrhage2
			Abscess
CT1			Hydrocephalus
			Neoplasia
			Pseudotumor
			cerebri

Lumbar puncture meningoencephalitis, spinal hemorrhage, leptomeningeal metastases; pseudotumor cerebri

Doppler, MRI; MR angiotomography, angiography arterial dissection, venous sinus thrombosis, cerebral infarction

Sinusitis

Trigeminal neuralgia Cluster headache Atypical facial

pain

Herpes zoster Chronic paroxysmal hemicrania

Oromandibular dysfunction, odontogenic Acute glaucoma

Optic neuritis Temporal arteritis Thalamic pain

Cluster headache Diabetic neuropathy Lesion in cavernous sinus

Supraor infratentorial mass

Lesion of brain stem or trigeminal nerve Herpes zoster Tolosa-Hunt syndrome

Central Nervous System

191

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