Cephalagia aka Chronic Headache..(part 1)



Headache aka Cephalagia is a painful and/or non-painful discomfort affecting the head and /or the surrounding structures.

Every day, thousands of patients visit doctors complaining of headache.
These consultations are rewarding as the chief skill is in INTERPRETING the history—not in taking it, so much as in allowing it to unfold. Let patients tell you about all the headache’s associations, or even who their headache is.

Tension headache is the most common cause of headache, for which stress relief may have more to offer than a neurologist, but some headaches are disabling and treatable (migraine, cluster headache), while others are sinister (space-occupying lesions, meningitis, subarachnoid haemorrhage).

👉 40% of persons have severe headache annually.
👉Lifetime prevalence : 91-96%
👉Peak incidence :2nd – 4th decades
👉Females > Males

Osuntokun BO et al. Acta Neuro Scand. 1987

👉Distention, traction or dilatation of intra/extracranial arteries.

👉Traction or displacement of large veins or there dural envelope .

👉Compression, traction or inflammation of cranial and spinal nerves .

👉Spasm, inflammation and trauma to cranial and cervical muscles or bone .

👉 Meningeal irritation and raised ICP

👉Referred pain from shared nerve supply

👉 Cell membrane abnormalities, ionic imbalances

👉 Plasma protein extravasation..sterile meningeal inflammation..trigeminovascular system, low Mg in hemiplegic migraine. BOLD Vasodilatation ffd by vasoconstriction. Laeo’s spreading depression.

👉Perturbation of intracerebral serotonergic projections .

👉 Release of neurotransmitters: Serotonin Histamine Substance P Prostaglandins , CGRP Endorphins VIP, Neurokinin A, Neuropeptide Y


There’s a very broad Classification of headaches by the Headache Classification Committee of the International Headache Society (IHS).

Primary headaches are those that occur for no obvious reasons and are not the result of any underlying disease or
Secondary headaches are the result of serious underlying conditions like brain tumor, inflammatory brain conditions etc

Pls note that primary headaches are far more common than secondary headaches.


👉 Tension headache
👉Vascular headache
👉 Pressure headache (raised ICP)
👉Referred headache
👉 Headache due to Inflammation

Also, Headaches could be
👉 Acute single episode
👉 Recurrent acute attacks of headaches
👉 Headaches of subacute onset
👉 Chronic headaches

Let’s look at some of them now.


👉 With meningism: If the headache is acute, severe, felt over most of the head and accompanied by meningeal irritation (neck stiffness) you must exclude:

*meningitis : fever, photophobia, stiff neck, purpuric rash, coma.

*encephalitis: fever, odd behaviour, fits, or consciousness↓.

*subarachnoid haemorrhage: sudden-onset, ‘worst ever’ headache, often occipital, stiff neck, focal signs, consciousness↓.

Admit immediately for urgent CT head. If CT –ve, do LP to look for signs of infection or blood products in the CSF.

👉 Head injury:Headache is common at the site of trauma but may be more generalized. It lasts ~2wks; often resistant to analgesia. Do CT head to exclude subdural or extradural haemorrhage if drowsiness ± lucid interval, or focal signs.

👉 Venous sinus thrombosis: Subacute or sudden-onset headache, papilloedema.

👉 Sinusitis causes dull, constant, aching pain over the frontal or maxillary sinus, with tender overlying skin ± postnasal drip. Pain is worse on bending over. Ethmoid or sphenoid sinus pain is felt deep in the midline at the root of the nose. Common with coryza. The pain lasts ~1–2 weeks. CT can confirm diagnosis but is rarely needed.

👉 Tropical illness:eg malaria: travel history, flu-like illness; typhus .

👉 Acute glaucoma:Typically elderly, long-sighted people. Constant, aching pain develops rapidly around one eye, radiating to the forehead.Symptoms: Markedly reduced vision, visual haloes, nausea/vomiting.Signs: Red, congested eye ; cloudy cornea; dilated, non-responsive pupil, may be oval; ↓acuity. Attacks may be precipitated by dilating eye-drops, emotional upset or sitting in the dark, eg the cinema. Treatment :Seek expert help at once. If delay in treatment of >1h is likely, start acetazolamide 500mg IV over several minutes.


1. Migraine

2. Cluster headache

3. Trigeminal neuralgia

4. Recurrent (Mollaret’s) meningitis: Suspect if fever/meningism with every headache. Send CSF for herpes simplex PCR. Is there access to the subarachnoid space via a skull fracture, or a recurring cause of aseptic meningitis (SLE, Behçet’s, sarcoid)?

CLUSTER HEADACHE (AKA migrainous neuralgia)

👉 Cluster headache may be the most disabling of the primary headache disorder.

👉 One theory (among many) is that this is caused by superficial temporal artery smooth muscle hyperreactivity to 5HT.
There are related hypothalamic grey matter abnormalities too.
An autosomal dominant gene also has a role.

👉 ♂:♀ ≥ 5: 1; onset at any age; commoner in smokers.

👉 Symptoms
Rapid onset severe pain around ONE EYE which may become watery and bloodshot with lid swelling, lacrimation, facial flushing, rhinorrhoea, miosis ± ptosis (20% of attacks; permanent in 5%).
Pain is strictly UNILATERAL and almost always affects the same side.

👉 It lasts 15–160min, occurs once or twice a day, and is often nocturnal.

👉 Clusters last 4–12wks and are followed by pain-free periods of months or even 1–2yrs before the next cluster.

👉 Sometimes it is chronic rather than episodic.

👉 Management
Acute attack:
100% O2 for ~15min via non-rebreathable mask (CI in COPD); 18 sumatriptan SC 6mg at the attack’s onset.

1st line are verapamil or topiramate. Also lithium, steroids, methysergide (highly effective but SE of retroperitoneal fibrosis ì take a 1 month ‘drug holiday’ every 6 months).


👉 Symptoms:
Paroxysms of intense, stabbing pain, lasting seconds, in the trigeminal nerve distribution. It is unilateral, typically affecting mandibular or maxillary divisions.
👉The face screws up with pain (hence TIC DOLOUREUX ).

Washing affected area, shaving, eating, talking, dental prostheses.

👉Typical patient:
♂ >50yrs old; in Asians ♀:♂≈2:1.

👉 Secondary causes:
compression of the trigeminal root by anomalous or aneurysmal intracranial vessels or a tumour, chronic meningeal inflammation, MS, zoster, skull base malformation (eg Chiari). MRI is necessary to exclude secondary causes (~14% of cases).

👉 ℞
Carbamazepine (start at 100mg/12h PO; max 400mg/6h; lamotrigine; phenytoin 200–400mg/24h PO; or gabapentin (see section).

👉 If drugs fail, surgery may be necessary. This may be directed at the peripheral nerve, the trigeminal ganglion or the nerve root.

👉Microvascular decompression:
Anomalous vessels are separated from the trigeminal root. Stereotactic gamma knife surgery can work, but length of pain relief and the time to treatment response are limiting factors.


👉 Symptoms
Classically: • Visual or other aura (see below) lasting 15–30min followed within 1h by unilateral, throbbing headache.
Or: • Isolated aura with no headache;
• Episodic severe headaches without aura, often premenstrual, usually unilateral, with nausea, vomiting ± photophobia/phonophobia (‘common migraine’).
There may be allodynia—all stimuli produce pain: “I can’t brush my hair, wear earrings or glasses, or shave, it’s so painful”.

👉 Signs:

👉 Associations:
Obesity (weight loss may ↓ excess oestrogen/estradiol production in adipose tissue—but benefit is unproven); patent foramen ovale (some say catheter closure may help).

👉 Tests:
None if typical history.

👉 Prodrome:
Yawning, food cravings, or changes in sleep, appetite or mood may precede the headache by hours or days.

👉 Aura:
Precedes the headache by minutes and may persist during it. • Visual: chaotic cascading, distorting, ‘melting’ and jumbling of lines, dots, or zigzags, scotomata or hemianopia; • Somatosensory: paraesthesiae spreading from fingers to face;
• Motor: dysarthria and ataxia (basilar migraine), ophthalmoplegia, or hemiparesis;
• Speech: (8% of auras) dysphasia or paraphasia, eg phoneme substitution.

Diagnostic criteria if no aura
≥5 headaches lasting 4–72h with either nausea/ vomiting or photo/phonophobia, and ≥2 of: • Unilateral; • Pulsating; • Interferes with normal life; • Worsened by routine activity, eg climbing stairs.

CHOCOLATE or: Cheese, oral contraceptives, caffeine (or its withdrawal), alcohol, anxiety, travel, or exercise. In ~50%, no trigger is found, and in only a few does avoiding triggers prevent all attacks.

Cluster or tension headache, cervical spondylosis, ↑BP, intracranial pathology, sinusitis/otitis media, caries. TIAs may mimic migraine aura. Migraine is rarely a sign of other pathology: don’t look too hard for antiphospholipid syndrome, arteriovenous malformations, or microemboli (but in some they may be important).


NSAIDs (eg ketoprofen 100mg, dispersible aspirin 900mg/6h) are preferred as there is less chance of developing medication misuse headache , and they have similar efficacy to oral 5HT agonists (triptans and ergot alkaloids).

Triptans are generally better tolerated than ergots: in one QALY-based study, rizatriptan was better/cheaper than sumatriptan , which was better/cheaper than Cafergot ® .

👉 Triptans are C/I  if IHD, coronary spasm, uncontrolled ↑BP, recent lithium, SSRIs, or ergot use.
Rare SE: arrhythmias or angina ± MI, even if no pre-existing risk.Ergotamine 1mg PO as headache starts, repeated at ½h, up to 3mg in a day, and 6mg in a week; or better, as a Cafergot ® suppository (2mg ergotamine + 100mg caffeine up to 2 in 24h; then ≥4 days without).
Emphasize dangers of ergotamine (gangrene, vascular damage).
CI: peripheral vascular disease, IHD; pregnancy; breast-feeding; hemiplegic migraine; Raynaud’s; liver or renal impairment; BP↑.


👉 Giant cell arteritis: Exclude in all >50yrs old with a headache that has lasted a few weeks.

Tender, thickened, pulseless temporal arteries; jaw claudication; ESR >40mm/h.

  • 👉 Prompt diagnosis and steroids avoid blindness.


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