Headache

Major causes of HA
Things you don't want to miss
Reversible Cerebral Vasoconstriction Syndrome
Headache History
HA (Migraine specific) history - Neurology Clinics
2SNOOP5Y
Headache syndromes
Migraine HA:
The pathophysiology of migraine is complex.
International Headache Society Criteria for Migraine Headaches
Migraine with aura
Criteria for Migraine with Brainstem Aura: ICHD (beta version of IIIrd edition)
Familial hemiplegic migraine:
The Basics of Sporadic and Familial Hemiplegic Migraine
Symptoms of Hemiplegic Migraine
Treatment of hemiplegic migraine
Chronic migraine
Status migrainosus:
Ophthalmoplegic migraine:
Retinal Migraine.
Vestibular migraine:
Trauma triggered migraine headaches
Selected medication use for acute therapy of migraine:
ER treatment of migraine
Neuromodulation.
International Headache Society Criteria for Tension-Type Headaches
Trigeminal Autonomic Cephalgias:
Symptoms common to all TAC:
Cluster Headache: Diagnostic criteria
Hemicrania Continua: (ICHD-II) criteria:
Paroxysmal Hemicrania: (ICHD-II) criteria:
Chronic paroxysmal hemicrania:
SUNCT/SUNA
Clinical Vignette - Cluster HA
Trigeminal neuralgia:
International Headache Society Criteria for Idiopathic Trigeminal Neuralgia
DDx of trigeminal neuralgia:
Chronic Daily Headache:
ICHD-II criteria for NDPH
Medication Overuse Headache (rebound headache)
Postconcussion headache:
Idiopathic intracranial hypertension (pseudotumor cerebri).
Temporal arteritis:
Occipital Neuralgia/cervicogenic headache
International Headache Society Criteria for Headache Attributed to Spontaneous Low CSF Pressure
Coital HA
Idiopathic Intracranial HTN (pseudotumor cerebri):
Brain freeze or cold stimulus headache
Cough headache,
Headache and pregnancy
Nervus intermedius neuralgia
Intravenous dihydroergotamine protocol
Headache Questionnaire

...Like your head isn't pounding by now...

COVID-19

Major causes of HA

Cause of HA and facial pain based on nature of onset.

Things you don't want to miss

Reversible Cerebral Vasoconstriction Syndrome

Headache History

Headache symptoms that suggest a serious underlying d/o:

CT or MRI is often needed to evaluate recent onset HA

Physcial Exam:

 Laboratory Data

Management

HA (Migraine specific) history - Neurology Clinics

Demographic.

Headache:

Triggers:

What medications were tried in the past

What is the current pain regimen

Investigations:

Assessment:

Headache syndromes

Primary HA are those in which HA and its associated features are the disorder in itself without an exogenous cause.  The key structures involved in primary HA are:

Secondary HA are those caused by exogenous d/o.

Relatively few cranial structures are pain-producing:

There are no pain receptors in the ventricular ependyma, choroid plexus, pial veins, and much of the brain parencyhma by itself.  Therefore, headache is caused by mechanical traction inflammation, or irritation of other structures in the head that are innervated, including the blood vessels, meninges, scalp, and skull.  The supratentorial dura (most of the intracranial cavity) is innervated by the trigeminal nerve (CN V), while the dua of the posterior fossa is innervated mainly by CN X, but also by CN IX and the first three cervical nerves.

Migraine HA: 

Clinical vignette.  A 23 year-old woman presents with 5 episodes of headache during the past 2 months.  Each episode begin with yawning, sensitivity to light, and a depressed mood that followed by the gradual onset of neck pain that spread to the occipital region and eventually to the retro-orbital region on the right side. The pain became incapacitating over a period of 1 to 2 hours and was associated with nausea and sensitivity to light and sound.  With two of the episodes, she had jagged lines in her vision for 15 minutes as the neck pain was beginning; with all episodes, she had severe fatigue and difficulty concentrating and finding words.  The headache lasted approximately 24 hours, and, after resolution, she had several hours of residual neck soreness, fatigue, and depressed mood.  

Migraine headache is a chronic and recurrent neurological disorder characterized by attacks of moderate to severe headaches associated with sensitivity to light, nausea, or a reduced ability to function.

Prevalence: 7 leading cause of time spent disabled worldwide. 2nd most common cause of HA. 

Lifetime prevalence of 25% for women and 6% for men in the United States.

1 in 25 women may have chronic migraine with headache on more than 15 days per month. 

It may begin early in childhood, but its prevalence rises steeply at 10 to 14 years of age and continues to increase until 35 to 39 years, after which it decreases gradually, particularly among women after menopause.  

It is also associated with increased risks of several other disorders, including asthma, stroke, anxiety and depression, and other pain disorders. However, it is exceedingly rare for cerebral ischemia or infarction to occur during a migraine attack. 

Pathogenesis: Dysfunction in monoaminergic sensory control systems in brain stem and thalamus.

The pathophysiology of migraine is complex. 

Cortical spreading depression is a phenomenon which is invoked in migraine with auras and the march of epileptic discharges.  Gilal calcium waves mediated through gap junctions appear, and initiate a circumferentially expanding negative potential that propagates at a characteristic speed of 20 m/s. It is associated with intracellular potassium.

Activation of hypersensitive “central generator” (it is debated whether the initiating trigger for migraine occurs in the cortex or in the brain stem) → Disruption of ion homeostasis, release of neurochemicals, and transient dysfunction of neuronal function → Meningeal blood vessel dilation and activation of trigeminovascular system → Release of vasoactive neuropeptides (calcitonin gene-related peptide (CGRP), neurokinins, prostaglandins, substance P, etc.) from activated trigeminal sensory nerves leading to a milleu of sterile neurogenic inflammation → Worsening vasodilation, increasing firing of trigeminal afferents causing pain intensification → Trigeminal nociceptive afferents carry pain signals to trigeminal nucleus caudalis (TNC) for processing and ascent through thalamus to cortex →  Continuous ascending pain signals activate more neurons leading to associated symptoms such as photo/phonophobia, nausea, and vomiting → Continuous TNC firing, leads to central sensitization if activated pathways are not stopped.

When a migraine attack progresses, cutaneous allodynia reflects central sensitization of second-order (trigeminothalamic) and third-order (thalamocortical) neurons. This is the result of a ‘‘wind-up’’ phenomenon involving increased glutamatergic and nitric oxide transmission.

Recent clinical trials support the efficacy of new therapies targeting calcitonin-gene-related-peptide (CGRP) for the treatment of acute migraine and for migraine prevention.

Brain regions implicated though functional neuroimaging:

Migraineur brains are sensitive to environmental and sensory stimuli; do not get easily acclimated.

International Headache Society Criteria for Migraine Headaches

Migraine without aura:  Recurrent headache disorder 

Migraine with aura

Diagnostic Criteria:

Subforms of aura:  The IHS criteria recognizes six subforms of aura with migraine headache:

Criteria for Migraine with Brainstem Aura: ICHD (beta version of IIIrd edition)

Basilar migraine

Familial hemiplegic migraine:

The Basics of Sporadic and Familial Hemiplegic Migraine

Migraine can present in a variety of ways. Hemiplegic migraine is a rare form of migraine where people experience weakness on one side of their body (hemiplegia) in addition to the migraine headache attack. The weakness is a form of migraine aura and occurs with other forms of typical migraine aura like changes in vision, speech or sensation. Hemiplegic migraine is divided into Familial hemiplegic migraine (runs in the family) or Sporadic hemiplegic migraine (happens only in one individual). This is a very rare migraine type so if you ever experience new or never-evaluated weakness with your headache, you should seek immediate medical evaluation and not assume you have hemiplegic migraine.

Both familial and sporadic hemiplegic migraines often begin in childhood. Diagnosing hemiplegic migraine can be difficult, as the symptoms can mimic stroke, seizures or other conditions. A full neurological work up, including obtaining imaging of the brain and vessels in the head, and careful review of medical history and symptoms are necessary to rule out other causes and confirm a diagnosis of hemiplegic migraine. Family medical history is especially helpful in diagnosing familial hemiplegic migraine.

Currently there are four genes related to familial hemiplegic migraine: CACNA1A, ATP1A2, SCN1A, and possibly PRRT2. These genes are related to channels on nerve membranes that control the movement of substances like sodium, calcium and potassium across the nerve. Mutations of these genes result in over excitability of nerves. Genetic testing is available but not necessary for all people. Genetic testing may be of highest yield in people with early onset hemiplegic migraine associated with eye movement abnormalities (nystagmus), seizures or other persistent neurologic symptoms (ataxia).

Symptoms of Hemiplegic Migraine

The symptoms can last for hours to days, or rarely weeks, but most resolve completely.

Please refer to the International Classification of Headache Disorders 3rd edition (beta version) website for more information on the criteria used to diagnosis hemiplegic migraine: https://www.ichd-3.org/1-migraine/1-2-migraine-with-aura/1-2-3-hemiplegic-migraine/

Treatment of hemiplegic migraine

Treatment of hemiplegic migraine can be challenging. The care of a headache specialist is often required, as many other doctors may never have treated a case of hemiplegic migraine.

Acute treatment: Triptans and ergotamines are currently contraindicated in the treatment of hemiplegic migraine because of their vasoconstrictive properties (risk of vessel spasm) and concerns about stroke. One small study was conducted, safely using triptans with patients with hemiplegic migraine, but more trials are needed before they’re considered a safe option. Other treatments such as NSAIDs, antiemetics, and sometimes narcotic analgesics are used for symptomatic relief of hemiplegic migraine. Intranasal ketamine has been shown to shorten the duration of aura symptoms in patients with hemiplegic migraine.

Preventive: Given the severity of the symptoms and the contraindication of certain acute medications (triptans and ergotamines), preventive regimens (medications taken daily to prevent the attacks whether you have a headache or not) are considered especially important in the treatment of hemiplegic migraine. There are small studies reporting use of verapamil, acetazolamide, flunarizine, ketamine, lamotrigine and naloxone for treatment of hemiplegic migraine. Since hemiplegic migraine is a subset of migraine with aura, certain preventive medications commonly used to treat typical migraine with aura, including amitriptyline, topiramate, and valproic acid may be beneficial. Beta-blockers are generally avoided for people with hemiplegic migraine out of theoretical concern that it may affect the ability of vessels to dilate.

Chronic migraine

Status migrainosus: 

Ophthalmoplegic migraine: 

Retinal Migraine.  

Retinal migraine refers to repeated attacks of reversible monocular visual disturbance including scintillations or blindness that appears gradually over 5 minutes and lasts 5 to 60 minutes. Reversible vasospasm has infrequently been documented when patients are examined acutely during an episode. The contribution of retinal spreading depression remains controversial; while it has been documented in chickens, it has not been documented in mammals.  Prophylactic treatment with calcium channel blockers can be helpful.

Vestibular migraine:  

Selected medication use for acute therapy of migraine:

Neuropeptides belonging to the family of calcitonin family: Calcitonin, amylin, adrenomedullin, intermedin.  In humans two forms are present: alpha-CRGP which is a 37-amino acid peptide and beta-CRGP which is the main isoform of enteric nervous system; differs in 3 amino acids. 

When to Use Migraine Prevention

Medications for prevention.

For migraine prevention, individual medications have been put into treatment groups based on their established clinical efficacy, significant adverse events, safety profile, and clinical experience of the US Headache Consortium participants: 

Exercise and migraine: Moderate physical activity, approximately 30 to 40 minutes of aerobic activity per day at least 3 times per week, has been shown to decrease migraine frequency.

Diet and migraine: Eating smaller more frequent meals and eating meals rich in complex carbohydrates can be associated with a lower risk of migraine attacks. Meals with complex carbohydrates may prevent some of the reactive hypoglycemia seen with intake of simple carbohydrates, which are thought to potentially trigger migraines.

Protocol for emergency room treatment of status migrainosus

ER treatment of migraine

ED regimen (highly effective): 1 L normal saline as well as 50 mg of IV Benadryl, 10 mg of IV Compazine, and 30 mg of IV Toradol (check pregnancy if in 3rd trimester, cannot give; check Sr. Cr for renal insufficiency).

One measure of success is the frequency with which the patient returns to a headache-free, fully functional state within 2 hours of treatment, with no recurrence within 24 hours

Neuromodulation.

Transcranial magnetic stimulation for acute and preventive treatment of migraine with and without aura (sTMS Savi dual-eneura).

Transcutaneous supraorbital neurostimulation (tSNS) for prevention and acute care of migraine (Cefaly).

Non-invasive vagal nerve stimulator (VNS, gammaCore) for acute treatment of Cluster Headaches and Migraine + Prevention

Remote Neuromodulation - (REN, Nerivio).

Occipital and supraorbital  neuromodulation (Relivion).

DFN-02 Sumatriptan NS with Permeation Enhancer (Tosymra)

DHE NS with POD device (Trudhesa).

PACAP38

PACAP27


International Headache Society Criteria for Tension-Type Headaches

Infrequent Episodic Tension-Type Headache

Frequent Episodic Tension-Type Headache

The same as infrequent episodic tension-type headache except for:

Chronic Tension-Type Headache

The same as infrequent episodic tension-type headache except for:

Tx: Amitriptyline has been the most successful medication for prophylaxis for patients with chronic TTH. Venlafaxine 150 mg po daily can also be used.

In a study of adult patients, 44% of patients with chronic tension-type headache reported significant improvement or complete resolution on 10-year follow-up.

Trigeminal Autonomic Cephalgias:

Trigeminal autonomic cephalalgias are generated through the trigeminal activation reflex that involves connections between the trigeminovascular system, cranial autonomic system, and hypothalamus.   All three systems are linked together through the trigeminal autonomic reflex, hypothalamic-trigeminal nucleus connections, and hypothalamic autnomic connections.  

The trigeminal nucleus caudalis and the superior salivary nucleus play a central role.  The superior salivary nucleus provides preganglionic parasympathetic fibers that are carried via the facial nerve, pass through the geniculate ganglion, and synapse in the pterygopalatine (sphenopalatine) ganglion. These parasympathetic neurons then project via the superior petrosal nerve to the effectors in the eye and nasal cavity, where they elicit vasodilation and increase secretion.

The trigeminovascular system is the pain component of TACs and starts with the ophthalmic or V1 branch of the trigeminal nerve, which receives inputs from the forehead, eye, dura, and large cranial vessels.  The ophthalmic branch projects to several nociceptive nuclei in the brainstem and upper cervical cord (together these nuclei are known as the trigeminocervical complex, which includes the occipital nerve), then to the thalamus, and finally to the pain neuromatrix (a collection of brain areas that modulate many types of pain).  Functional MRI (fMRI) and anatomic MRI studies have shown changes in the pain neuromatrix in patients with cluster headache.  Interestingly, rare cases of secondary cluster headache have been reported from meningiomas, carotid dissections, and venous sinus thromboses, which are all inputs to the trigeminovascular system.  The trigeminovascular system has several signaling molecules including calcitonin gene-related peptide (CGRP), which is elevated during a cluster attack.

The autonomic system is responsible for lacrimation, conjunctival injection, and other cranial autonomic features. Most of these features involve either parasympathetic overactivation or sympathetic inactivation.  The autonomic areas in TACs include a pathway from the superior salivatory nucleus to the sphenopalatine ganglion.  By placing an electrode over the sphenopalatine ganglion in patients with cluster headaches, a cluster attack can be triggered or aborted by changing the stimulation parameters.  The autonomic system has several signaling molecules, including vasoactive intestinal peptide, which is elevated during a cluster attack.

The hypothalamus may explain many of the other clinical features of the TACs, and research suggests a large role for the hypothalamus in all the TACs.  The hypothalamus includes the circadian system and aggression areas, which may explain the clocklike regularity of cluster headaches and the restlessness seen in patients with TACs.  Positron emission tomography (PET) has shown activation of the posterior hypothalamus at the beginning of a cluster attack that was triggered by nitroglycerin.  Activation of the hypothalamus has also been seen in functional imaging of paroxysmal hemicrania, hemicrania continua, and SUNCT/SUNA.  Molecules modulated by the hypothalamus, such as melatonin, are altered in patients with cluster headaches.  Ultimately, the hypothalamus appears to be the first area activated during a cluster attack, followed by trigeminovascular and autonomic activation.  

A possible genetic basis also exists for these disorders; familial cases are rare but have been reported for all the TACs.  A genome-wide screen of 259 patients with cluster headaches and 267 controls failed to find any individual genes, and the conclusion was that the genetics of cluster headache is complex.  Similar to migraine, multiple susceptibility genes likely exist for the TACs.

The TACs are composed of five diseases: cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicrania continua.

Symptoms common to all TAC: 

Cluster headache is a strictly unilateral headache with a rapid onset over minutes. 

Cluster Headache: Diagnostic criteria

Episodic Cluster Headache:  Occurs in periods lasting 7 days to 1 year separated by pain-free periods lasting 1 month or more.

Diagnostic Criteria:

Chronic Cluster Headache:  Attacks occur for more than 1 year without remission or with remissions lasting less than 3 months.

Diagnostic Criteria:

Acute Treatment:

Transitional medications: The mainstays of short-term prophylaxis are greater occipital nerve blocks (with local anesthetic plus steroids) or a course of oral steroids.  The most effective formulations are unknown; multiple types and doses of steroids have been used for greater occipital nerve blocks and for oral steroids.  For oral steroids, a taper over 3 weeks is generally recommended because of the risk of osteonecrosis of the hip, especially with prolonged steroid use. The use of steroids should also be limited to 2 to 3 courses per year.  Steroids are ideal for patients with brief headache cycles or when up-titrating medications such as verapamil.

Prophylaxis:  The drug of choice for cluster headache prevention is verapamil.  A typical total daily maintenance dose is generally 480 mg to 720 mg divided into 3 doses.  Although data are limited, the immediate-release formulation is generally preferred.  Cardiac conduction abnormalities are a feared consequence of high doses of verapamil, usually caused by lengthening the PR interval.  A pretreatment ECG and a consideration of ECGs after dose increases is recommended in a survey of cardiologists.  A proposed schedule is ECG monitoring before initiation, 10 to 14 days after each dose change, and every 6 months thereafter  hile on the medication.  

Second-line medications for cluster headaches include topiramate and lithium.  Melatonin is helpful and is often used as an adjunct preventive.  Other medications with data supporting use as a second or third-line treatment of cluster headaches include baclofen and valproic acid.   For episodic cluster headaches, preventives should be up-titrated early in the headache cycle to an effective dose, using transitional medications if needed.  When the patient is headache-free for about 2 weeks and is presumably out of their headache cycle, the preventive medication can be downtitrated and discontinued.

Corticosteroids with preventive agent: A typical regimen might be prednisone 60 mg daily for 5-7 days, followed by a tapering dose of 10 mg every 3 days while simultaneously initiating a preventive agent on day 1.

Verapamil, 80 mg tid, 120-480 mg daily it 3-4 divided doses. - Verapamil plus (all other meds added to Verapamil).

Lithium 300 mg 3-4 times a day, (Li conc <1.2 mEq/L)

Valproate 250 bid to -1500 mg daily in divided doses

Topiramate 75-200 mg daily.

Melatonin 15 mg po qhs

Nitroglycerin and histamine are known to trigger attacks in patients who have cluster headaches, for which reason nitroglycerin should be avoided in treating elevated blood pressure in patients with an acute cluster headache, and patients with cluster headaches should avoid histamine-rich foods such as red wine. Morphine would not be useful in the treatment of acute cluster headaches since it releases histamine.

Refractory patients:  When extensive medication trials are unsuccessful, a sleep study and pituitary laboratory studies should be considered,  For patients who are refractory to treatments, more invasive procedures can be considered.  Neuromodulation is generally the preferred technique as it is minimally destructive. Sphenopalatine ganglion stimulation, occipital nerve stimulation, and deep brain stimulation of the hypothalamus have all been proposed as invasive neuromodulation treatments for cluster headaches.  Patient selection for these procedures is key, and considerations for their use include 2 years of daily or almost daily attacks, extensive medication trials, management by a single provider over at least 1 year, and a psychological evaluation.  Current American Headache Society guidelines support the use of sphenopalatine ganglion stimulation (level B evidence: probably effective) but are unfavorable toward deep brain stimulation (level B evidence: probably ineffective).  The guidelines also note that not enough studies of occipital nerve stimulation have been performed, but the existing data suggest a benefit.  Sphenopalatine ganglion stimulation is available in Europe but not in the United States, although trials have been performed and are awaiting FDA review.  Some of the neuromodulation devices mentioned are MRI-compatible while others are not.  If MRIs are anticipated in the future, this point should be discussed with the implanting surgeon.

Hemicrania Continua: (ICHD-II) criteria:

It must have all of the following criteria: 

Paroxysmal Hemicrania: (ICHD-II) criteria:

Paroxysmal hemicrania is an indomethacin-responsive headache. Usually, complete resolution is seen within 24 to 48 hours of starting indomethacin. There is a variable range of doses (maintenance doses may vary between 12.5 mg/d and 300 mg/d, and sometimes dosing is tailored to attack severity). If indomethacin is discontinued, the pain recurs within 1 day to a couple of weeks 

Chronic paroxysmal hemicrania: 

1) Unilateral headache of moderate intensity, with episodes lasting under 45 minutes on average, occurring multiple times throughout the day, and at a 3:1 ratio female: male

2) Absolute response to indomethacin. 

Patients may also complain of a continuous low-grade headache in between the more severe attacks (hemicrania continua).


SUNCT/SUNA 

Clinical Vignette - Cluster HA

A 33-year-old woman with a history of infrequent tension-type headaches presented for evaluation of a new type of headache that had been occurring for 3 years.  She reported extreme pain in the right eye, which lasted approximately 30 minutes and occurred 1 to 5 times per day.  The pain had never occurred on the left side.  During a headache, her right eye became watery, bloodshot, and sensitive to light.  The skin around the right eye felt swollen and hot, and she sat alone and rocked back and forth.  One of the headaches invariably occurred at 2:00 AM and woke her up from sleep.  These headaches occurred every day for 6 weeks in September and October, then she had only the occasional tension headache until the following September, when the right-sided headaches resumed at 2:00 AM.  She had tried naproxen, ibuprofen, and acetaminophen without relief, and hydrocodone “took the edge off a little.”

Comment:  This is a presentation of an as-yet undifferentiated trigeminal autonomic cephalalgia.  This patient meets most criteria for both cluster headache and paroxysmal hemicrania, as these two conditions overlap in the duration and frequency of the headache attacks.  Statistically, the female sex makes her more likely to have paroxysmal hemicrania, while the circadian pattern and restlessness are more likely to be cluster headaches.  An indomethacin trial is warranted in this patient.  If ineffective, the patient should be treated for cluster headaches.  Patients with trigeminal autonomic cephalalgias can have migrainous features such as photophobia, but they tend to be only ipsilateral to the pain.  The restlessness in this patient may not be immediately obvious; while most patients with restlessness will pace or move about the room, some patients will give a history of staying in one place but continuously rocking or moving.

Trigeminal neuralgia: 

International Headache Society Criteria for Idiopathic Trigeminal Neuralgia

Patients tend to be asymptomatic between paroxysms, though a subset of patients may develop prolonged continuous background pain, with fluctuations in intensity and periods of remission and recurrence that parallel the paroxysmal pain.  Most patients present after age 40, women are more affected than men. 

Triggers:  gentle touching of the face (83%), talking (59%), chewing (41%) and tooth brushing (36%).  Some patients notice a transient refractory period following a paroxysmal pain, where additional pain cannot be triggered.  

If trigeminal neuralgia is associated with trigeminal sensory loss, other cranial nerve deficits, or onset is before age 40; MRI should be performed to rule out neoplasm or multiple sclerosis.

History in evaluation patient with lower facial pain:

DDx of trigeminal neuralgia:

Treatment: Carbamazepine, 200 to 600 mg p.o. t.i.d. or oxcarbazepine, 300-900 mg p.o. b.i.d.

Phenytoin, 300-400 mg p.o. daily is an alternative to carbamazepine but is less effective. Baclofen, 10-20 mg 4 times a day, gabapentin, 100-800 mg 3 times a day, valproate 250-500 mg t.i.d., or topiramate 50-300 mg p.o. b.i.d. are reported be effective in some patients.

In refractory cases, microvascular decompression of the trigeminal nerve, radiofrequency and balloon compression rhizotomy, glycerol rhizotomy, or gamma knife of the trigeminal nerve may be required

Chronic Daily Headache:

ICHD-II criteria for NDPH 

Includes a headache that, within 3 days of onset, is present daily, and is unremitting, for more than 3 months. 

It must have at least two of the four following criteria: 

Medication Overuse Headache (rebound headache)

Postconcussion headache: 

Treatment: NSAIDs, reassurance. In protracted cases, a tricyclic antidepressant or centrally acting muscle relaxant such as baclofen 10 mg t.i.d. or tizanidine 4 mg t.i.d. can be used. Headaches following head injury can be multi-factorial, and evaluation of the cervical spine, brain to rule out SDH or hydrocephalus may be needed.

Idiopathic intracranial hypertension (pseudotumor cerebri). 

Characterized by a prior of headache, papilledema, and increased intracranial pressure in the absence or intracranial mass lesion or hydrocephalus. Occurs most commonly in young obese woman. Diagnoses of exclusion after ruling out other causes of increased intracranial pressure, including a mass lesion, dural sinus thrombosis, chronic meningitis, hypervitaminosis A, and tetracycline oral corticosteroid exposure.

Depressed level of consciousness and FND do not occur, except cranial nerve 6  palsy which is non-specific sign of increased intracranial pressure. The main problem in idiopathic intracranial hypertension as a visual  loss results from optic nerve damage and can be permanent. MRI of the brain and MRV should be performed to rule out a mass lesion, hydrocephalus, or dual sinus thrombosis. If the MRI is normal, he'll be establishes the diagnosis was by the finding of normal CSF under increased pressure greater than 20 cm of water.

Treatment: One 30s and have spontaneous remission of headache after the first LP. Removal of CSF performed every few days to every few weeks. Weight reduction is recommended. Acetazolamide, 250-500 mg p.o. t.i.d. 4 furosemide 40-80 mg p.o. daily and reduce CSF production and intracranial pressure. Topiramate, 50-300 mg p.o. b.i.d.

Check baseline visual field and visual equity testing and serial visual field testing to valid the blind spots. Fundus photography. The visual loss progresses despite medical therapy, a lumboperitoneal shunt or optic nerve sheath fenestration may be necessary.

Temporal arteritis: 

This is an illness of elderly patients, characterized by inflammatory infiltrates of lymphocytes and giant cells and extradural  and cranial arteries dated patient usually are about 50 years of age.

Symptoms: Low-grade fever, diffuse myalgias, weight loss, weakness, and malaise (PMR). 

Labs: Pts. > 50: ESR is elevated in almost all patients usually up to level  60-120 mm/h. Jaw claudication or tongue claudication is uncommon but useful feature for diagnosis.

CRP. 

pts age 40 or less, do hypercoagulable w/up: protein C, S, factor V leiden, ATIII, PT, PTT, LA, anticardiolipin abs (IgG, IgM, IgA), B2-glycoprotein, fibrinogen.

TEE, doppler u/s carotids.

Treatment: Ischemic optic neuropathy occurs in 10-30% of untreated patients because of involvement of the ophthalmic artery.

 Prednisone 100 mg p.o. daily and schedule the patient for temporal artery biopsy. Maintenance steroids 10-20 mg per day can usually be discontinued within 6 months to 2 years.

Occipital Neuralgia/cervicogenic headache

International Headache Society Criteria for Headache Attributed to Spontaneous Low CSF Pressure

A. Diffuse and/or dull headache that worsens within 15 minutes after sitting or standing, with at least 1 of the following and fulfilling criterion D:

B. At least 1 of the following:

C. No history of dural puncture or other cause of CSF fistula

D. Headache resolves within 72 hours after epidural blood patching

According to the Headache Classification Committee of the International Headache Society, headache after lumbar puncture is defined as “bilateral headaches that develop within 7 days after an lumbar puncture and disappears within 14 days. The headache worsens within 15 min of resuming the upright position, disappears or improves within 30 min of resuming the recumbent position”. This definition helps to avoid confusion with migraine or simple headache after lumbar puncture.

International Headache Society Criteria for Hypnic Headache ("Alarm clock headache") REM headache.

This older patient with headaches that occur exclusively during sleep at the same time every night and last more than 15 minutes after awakening meets the criteria for the primary headache disorder hypnic headache.  Caffeine is first-line abortive therapy for this headache type. 

Coital HA

Idiopathic Intracranial HTN (pseudotumor cerebri): 

HA, papilledema, pulsatile tinnitus, visual loss, and diplopia (as a result of CN6 palsy). Women > men. Most are obese. Peak incidence 30s. 

HA is diffuse.

CT and MRI. MRI shows slit-like ventricles and empty sella. Dilation of the optic nerve sheath and flattening of the back of the globe are characteristic. LP with high OP. Removal of 20-40 mL CSF improves conditions.

Tx: Acetazolamide, 1-2 g/day or, furosemide, 40-60 mg PO bid. In refractory cases, optic nerve sheath fenestration, or LP or VP shunting.

Brain freeze or cold stimulus headache 

It is an acute, short-lasting, frontal non-pulsatile pain that happens in susceptible people upon the passage of a cold stimulus in any form on the palate or the pharynx. Usually, it resolves up to 5 minutes after removing the stimulus. The pathophysiology is not well understood and is thought to be a trigeminal or glossopharyngeal mediated pain. Some studies raised the possibility of a vascular phenomenon that could explain why it is more common in patients with migraine

Cough headache, 

It is a transient, severe head pain upon coughing, sneezing, weight-lifting, bending, or stooping, is part of a group of headaches called benign exertional headaches (diagnosed after serious secondary causes are ruled out) and may be self-limiting. However, at least 75% of patients with cough headache respond to indomethacin.

The new International Headache Society Classification, second edition, criteria for menstrual migraine define pure menstrual migraine without aura as attacks in a menstruating woman that fulfill criteria for migraine without aura and occur exclusively on days –2 to +3 of menstruation in at least two out of three menstrual cycles and at no other times of the cycle. 

In contrast, menstrually related migraine without aura is defined as attacks in a menstruating woman that fulfill criteria for migraine without aura and occur on days –2 to +3 of menstruation in at least two out of three menstrual cycles and additionally at other times of the cycle

Lower vitamin D levels linked to increased frequency of headache in men.

Headache and pregnancy

Most common headaches seen in pregnancy are Tension-type headache and migraine, while cluster headache is much less common.

Often there is a history of these primary headaches prior to pregnancy. 

When headaches occur for the first time during pregnancy or postpartum, a diagnostic evaluation is needed.  Check for possibility that it is related to a complication of pregnancy, like severe preeclampsia/eclampsia.  This always needs to be excluded in women over 20 weeks of gestation. 

Migraine headache typically is less frequent in pregnancy. Women with menstrual migraine, migraine with aura has less migraine during pregnancy and have recurrence in the 1st week to 1st month post-partum.  Women who breast fed has less migraine attacks, post-partum.  Tension type headache is not affected by pregnancy.

Other causes:  MOH, Carotid or vertebral artery dissection, Intracranial hemorrhage (eg, arteriovenous malformation, aneurysm, trauma), cerebral venous sinus thrombosis, benign intracranial hypertension, post dural puncture headache, mass lesion/tumor, primary thunderclap headache, RSCVS (caused by pseudephedrine, ergots, cocaine), pituitary apoplexy, meningioma, PRES, primary angiitis of the central nervous system, infection (eg, sinusitis, meningitis, encephalitis), and stroke. 

No need to do a complicated and duplicate workup if the cause of her primary headache is known and the nature and characteristic of headache has not changed from her usual type of headaches. 

Neuroimaging:  Focal neurological deficits prompt neuroimaging workup ASAP.

Other investigations: 

Treatment strategy for migraine headache in pregnancy:

HaNDL.  The International Classification of Headache Disorders diagnostic criteria for HaNDL are: 

(A) episodes of moderate or severe headache lasting hours; 

(B) CSF pleocytosis with lymphocytic predominance and normal neuroimaging, CSF culture and other tests for aetiology; 

(C) episodes of headache are accompanied by transient neurological deficits; and 

(D) episodes of headache and neurological deficits recur over <3 months. 

Thereafter, HaNDL is an exclusion diagnosis that mainly depends upon the experience and awareness of the physician. Moreover, given that it is an acute or subacute disease which presents with focal neurological deficits, it may mimic stroke.

Neck-tongue syndrome is a rare headache disorder characterized by sudden paroxysmal neck and occipital pain lasting approximately 1 minute, followed immediately by transient ipsilateral numbness of the tongue lasting seconds to up to 5 minutes. Associated symptoms can include dysphagia, dysarthria, lingual pseudoathetosis, and tongue paralysis. Symptoms of neck-tongue syndrome are almost always triggered by sudden rotation of the neck, almost always to the same side as the pain and numbness. Frequent participation in a number of sporting activities has been implicated in some cases of neck-tongue syndrome, including swimming, softball pitching, bowling, and gymnastics. Other possible mechanisms include trauma and poor posture, but the most common mechanism remains idiopathic at this time.

A population-based CAMERA  (Cerebral Abnormalities in Migraine, an Epidemiologic Risk Analysis) study demonstrated an elevated prevalence of syncope and orthostatic intolerance in migraineurs without clear interictal signs of autonomic nervous system dysfunction.  NEUROLOGY 2006;66:1034–1037

http://ihs-classification.org/en/

https://americanmigrainefoundation.org/

https://consultqd.clevelandclinic.org/cgrp-inhibitors-for-migraine-prevention-what-prescribers-need-to-know/

Nervus intermedius neuralgia 

The intermediate nerve of Wrisberg (the nervus intermedius) is a small sensory branch of the facial nerve (cranial nerve VII) carrying general visceral efferent, special visceral afferent (taste), and general somatic afferent fibers.  The cell bodies of the sensory afferents dwell in the geniculate ganglion, and their peripheral axons innervate the inner ear, the middle ear, the mastoid cells, the eustachian tube, and part of the pinna of the ear.  Nervus intermedius neuralgia is an extremely uncommon pain syndrome in which the pain is very similar to that of trigeminal neuralgia but in the distribution of n. intermedius.  Generally it is presumed that its etiology is the cross compression of the nerve at its central-peripheral myelin junction.  Few cases have been reported since the original report by Clark and Taylor in 1909.

Paroxysmal, brief (seconds to a few minutes), shock-like or lightning-like pain that follows a peripheral or cranial nerve distribution and can spread to adjacent areas in the course of the attack.  By definition, no objective neurologic deficits are found in the distribution of the affected nerve.  Attacks can be provoked by nonpainful stimulation (allodynia) of trigger points or zones. A refractory period follows attacks; the duration of the refractory period shortens as the disease progresses.  Nervus intermedius neuralgia is a rare disorder characterized by brief paroxysms of pain felt deeply in the auditory canal. Other terms previously used for this condition used are geniculate neuralgia and Hunt neuralgia

Pathophysiology:  There is no proven pathologic substrate for nervus intermedius neuralgia.  In addition, there is no indisputable evidence that the neuralgic pain originates in the geniculate ganglion or the nervus intermedius.  Some investigators believe that compression of the nerve by a blood vessel is an important mechanism, and relief of pain by vascular decompression of the intermediate nerve provides some support for this position.  However, there are multiple cranial nerve afferents in the ear, and otalgia in these cases could have resulted from a compromise to the nervus intermedius and/or other cranial nerves.  In different surgical reports, section or decompression of a combination of cranial nerves V, VII, VIII, IX, X, and/or XI has been necessary to obtain relief.  Thus, the role of vascular compression in the pathogenesis of geniculate and other neuralgias is disputed

Clinical manifestations:  The syndrome consists of brief (usually lasting seconds, rarely minutes), severe paroxysmal pain, felt within the depths of the ear, and sometimes is associated with a trigger-zone in the posterior wall of the ear canal.  The pain can be sharp or burning, may involve the auricle, and sometimes radiates from the auditory canal to the parieto-occipital region or to trigeminal sensory zones.  Altered taste perception, such as a sense of bitter taste, can occur in some individuals, as can disorders of lacrimation or salivation.  The presence of an attack trigger (ie, auditory canal stimulation) was once considered a characteristic feature of nervus intermedius neuralgia.  However, a systematic review found that a trigger was described in only 3 of 10 patients with available data.  

Diagnosis:  The diagnosis of nervus intermedius neuralgia is made on clinical grounds and is based upon pain description. The diagnosis requires ruling out other neurologic and otolaryngologic causes of ear pain.  

Diagnostic criteria: The diagnosis of classic nervus intermedius neuralgia, according to the International Classification of Headache Disorders 3rd edition (ICHD-3), requires fulfilling all of the following criteria :

The condition is rare if one carefully adheres to these criteria.  Although the ICHD-3 diagnostic criteria mandate the presence of a trigger area in the auditory canal, this is not always present .

Differential diagnosis:  The differential diagnosis for otalgia is broadly divided into primary (pathology within the ear such as infection, inflammation, trauma, or neoplasm) versus secondary (referred) causes.  Nerves referring pain to the ear include branches of cranial nerves V, VII, IX, and X, and upper cervical nerve roots (C2-C3) via the great auricular and lesser occipital nerves.  Pathology affecting any of these neural structures can potentially refer pain to the ear.

Some patients fitting ICHD-3 diagnostic criteria for nervus intermedius neuralgia may have a variant of glossopharyngeal neuralgia with pain paroxysms restricted to the ear.  This is the main disorder in the differential diagnosis.  In such cases, the typical pharyngeal pain of glossopharyngeal neuralgia may not occur until years later. 

In a few case reports, vascular compression of cranial nerve VII, the intermediate nerve, and cranial nerve VIII by a loop of the anterior inferior cerebellar artery was identified as the cause of intractable paroxysmal ear pain.  One patient also had ipsilateral sensorineural hearing loss, tinnitus, and positional vertigo; another had hemifacial spasm .

Secondary nervus intermedius neuropathy is most commonly caused by reactivation of latent varicella-zoster virus (ie, herpes zoster) within the geniculate ganglion, with subsequent spread of inflammation to nearby ganglia and cranial nerves, leading to ear pain, ipsilateral facial paresis, and vesicular eruption in the auditory canal and auricle (ie, the Ramsay Hunt syndrome). 

Evaluation:  As noted above, it is necessary to rule out other neurologic, otolaryngologic, and infectious causes of ear pain in cases of suspected nervus intermedius neuralgia.  We recommend a brain MRI (contrast-enhanced) and MRA for patients with suspected classic nervus intermedius neuralgia to rule out a structural lesion or vascular compression.  The workup requires a contrast-enhanced magnetic resonance imaging (MRI) of the brain, focusing on the internal auditory meatus.  Brain MRI is useful for identifying structural brain lesions (eg, demyelinating lesions or a mass lesion in the cerebellopontine angle).  Magnetic resonance angiography (MRA) is useful for demonstrating an ectatic blood vessel compressing the nerve.  However, the sensitivity and specificity of these imaging studies for identifying a secondary cause of ear pain in patients with suspected nervus intermedius neuralgia and a normal neurologic examination is unknown.  Brain MRI, however, does not image all areas that can refer pain to the ear. Therefore, MRI of the face and soft tissues of the neck may be needed in some cases.  Urgent brain imaging is warranted if there are neurologic deficits.

Treatment:  The rarity of nervus intermedius neuralgia has precluded accumulation of high-quality data to guide therapy.  Pharmacotherapy is first-line treatment, based on efficacy for other cranial neuralgias.  Surgical approaches are reserved for patients with refractory symptoms. 

Pharmacologic treatment is based largely upon extrapolation from treatments that are effective for other cranial neuralgias, mainly trigeminal neuralgia. 

Carbamazepine for most patients with nervus intermedius neuralgia.  The usual starting dose of carbamazepine is 100 to 200 mg twice daily.  The dose can be increased slowly over several weeks as needed for pain relief, with a typical maintenance dose of 600 to 1200 mg daily in divided doses.

Adverse effects of carbamazepine include drowsiness, dizziness, nausea, and vomiting; slow titration may minimize these effects.  Carbamazepine-induced leukopenia is a potential adverse effect, but it is usually benign.  Aplastic anemia is a rare side effect.  The Stevens-Johnson syndrome and toxic epidermal necrolysis are additional rare complications, particularly during the first eight weeks of therapy.  They are significantly more common (estimated incidence of 5 percent) among patients with the HLA-B*1502 allele.  This allele occurs almost exclusively in patients of Asian ancestry, including South Asian Indians.  Screening for this allele is recommended in patients with Asian ancestry prior to starting carbamazepine. 

For patients who do not tolerate or respond to carbamazepine, likely the best next alternative drugs are oxcarbazepine or baclofen.  Other reasonable options are lamotrigine. gabapentin, pregabalin, and phenytoin.  Tricyclic antidepressants amitriptyline are only occasionally effective.

Neurosurgery is considered as a last resort when pharmacotherapy fails or when adverse effects of medications significantly reduce quality of life.  However, there are limited data to guide operative techniques.  The opinion of an experienced neurosurgeon, familiar with the detailed anatomy of the nervus intermedius and its variants, and with intraoperative anatomic findings, should be obtained to choose the most appropriate intervention.  Alone, sectioning of the nervus intermedius is often ineffective .  Most often, the procedure involves excision of the nervus intermedius and geniculate ganglion, with or without exploration and/or section of cranial nerves V, IX, and X.  The surgeon may elect to perform vascular decompression if a potential offending vessel is seen during exploration.  One series of 64 patients who had excision of the nervus intermedius and geniculate ganglion reported "excellent" results in 63 patients, but outcomes were not objectively defined in this report.  Complications included a temporary partial facial paralysis in 11 patients (17 percent).  In addition, permanent ipsilateral xerophthalmia is an expected outcome, due to sectioning of the greater petrosal nerve as part of the surgical approach to the geniculate ganglion.  Microvascular decompression of cranial nerves V, IX, and X, with or without section of the nervus intermedius, is another option.  In a series of 10 patients with nervus intermedius neuralgia treated with microvascular decompression who had long-term (>12 months) follow-up, an "excellent" outcome was reported for three patients, and partial relief for six 

Clinical vignette:  A 72-year-old man presented with a 4-month history of left ear pain associated with dysesthesia and allodynia on the left half of his mandibular region.  His major complaint was a continuous pulsating pain and, most of all, an intermittent electric shock-like pain deep in his left ear.  He had had upper airway infection symptoms four months previous.  During this period, first he had a vesicular rash in his mouth, especially around the peritonsillar region, and then a left facial paralysis had developed.  After high-dose steroid therapy, these symptoms had begun to improve. Just after this, a pain occurred at the left part of his face.  The onset of pain had been on the whole left face, including the temporal and parietal area behind the ear.  It was a continuous burning and sharp pain.  That initial pain had declined spontaneously in a few weeks and was replaced by dysesthesia and allodynia on the left mandibular region.  During this interval, a sharp, intermittent electric shock-like pain had appeared deep in his ear.  Before he came to our pain clinic, he had used many kinds of anti-inflammatory drugs and some weak opioids.  But he had no relief.  He had also used B1-B6 vitamin complex. After evaluation, we concluded that a n. intermedius neuralgia had developed after a herpes zoster infection of the facial nerve.

At the time he had come to our pain clinic, he had no rash and no sequelae of paralysis.  We started amitryptiline 10 mg and carbamazepine 200 mg orally.  One week later, we saw that the frequency of electric shock-like pain had diminished, dysesthesia and allodynia on his left mandible region had disappeared, and the continuous pain in his ear was reduced.  The residual pain was more prominent at night.  At this dose, he felt drowsiness as a side effect of the carbamazepine and we eventually stopped it.  We changed the antiepileptic medication to lamotrgine 100 mg daily.  In a week, we increased the dose to 200 mg per day, with no side effects.  One month later, he had no pain or side effects for more than twenty days.  After 6 months, he had no pain with 200 mg lamotrigine.

Neuralgia can affect any cranial nerve that has somatic afferents, such as n. intermedius.  The patient complains of intermittent stabbing pain, like electric shock, deep in the ear.  The syndrome is always unilateral and generally seen in the elderly.  Herpes zoster can also lead to both acute and chronic pain syndromes.  The patient with zoster infection of geniculate ganglion usually has vesicular eruption in the distribution of nerve.  The onset of pain is followed within a few days by the appearance of grouped vesicles and spontaneously disappears as the disease resolves.  When an ipsilateral facial palsy accompanies the painful eruption, this disorder is known as the Ramsey–Hunt syndrome.  In these cases, high dose of 100 mg prednisolone daily for one week may be used for the treatment of facial palsy.  The main differential diagnosis criteria of this syndrome is the pain, which is constant and burning and can be readily discriminated from the intermittent stabbing pain of n. intermedius neuralgia.  The initial feature of our case fit the Ramsey–Hunt syndrome.  In a few days after the eruption and the facial palsy recovered, n. intermedius neuralgia symptoms were apparent.

Similar to trigeminal neuralgia, the genesis of n. intermedius neuralgia is a mystery.  It is presumed that the etiology of n. intermedius neuralgia is analogous to that of trigeminal neuralgia.  Calvin and colleagues concluded that both peripheral and central mechanisms are required for the production of trigeminal neuralgia.  Fromm and associates proposed that a peripheral nerve lesion (in the trigeminal root or distal) is the first event in a process that leads to central synaptic changes.  The response of the central synapses to altered peripheral events leads to the development of the trigeminal neuralgia.

We suggest that edema of the facial in the bony channel, which could occur with zoster, might trigger this peripheral mechanism.  The peripheral sensory distribution of the n. intermedius lies in areas also supplied by sensory fibers of cranial nerves V and X.  This may be the reason why the patient was feeling dysesthesia and allodynia on the left mandibular region.

The management of n. intermedius neuralgia is similar to trigeminal neuralgia.  In a medical approach, the main drug is carbamazepine.  Many patients tolerate this drug poorly, predominantly because of side effects related to the central nervous system.  For patients who cannot tolerate carbamazepine, lamotrigine is a new option.

In conclusion, n. intermedius neuralgia, which is a very uncommon type of neuralgia, may follow zoster infection of geniculate ganglion. Because this syndrome is very similar to trigeminal neuralgia, the therapeutic approaches are the same.

Intravenous dihydroergotamine protocol

Generic name: Dihydroergotamine (DHE)

Available dosage form: 1 mg/1 mL

Indication/procedure: DHE is used in the treatment of medically refractory migraine and cluster headache

Admission Check any complicating medicines, such as triptans, 5-HT1B/1D receptor agonists, have been discontinued Vital sign recording: heart rate, blood pressure, respiratory rate, temperature, oxygen saturation, upon admission and then prior to each DHE dose

Baseline EKG Weight Laboratory tests: complete blood count with differential, sodium, potassium, chloride, blood urea nitrogen, creatinine, glucose, calcium, magnesium, phosphate, prothrombin time, partial thromboplastin time, international normalized ratio Urine: for pregnancy (if female) and toxicology screen

Potential side effects/adverse events Nausea and vomiting, leg cramps, limb pain, chest discomfort, abdominal cramps, diarrhea, parasthesias Cardiovascular effects: vasospasms, tachycardia, bradycardia, hypertension Coldness of the skin and/or numbness and tingling of the extremities may indicate ergotism, which can include gangrene

Contraindications/warnings Peripheral vascular disease, coronary heart disease, history of cerebrovascular event, severe or poorly controlled hypertension Impaired liver or renal function Pregnancy

Adult dosing : intermittent IV infusion of DHE for patients older than 16 years or weighing more than 50 kg (it is essential to control nausea during the use of dihydroergotamine; dose and rate of infusion may need to be adjusted as described below) The patient should be pretreated with ondansetron (ondansetron may be substituted for granisetron or other appropriate antiemetic drugs based on local clinical practice or particular clinical settings; the key practice point is to strive to minimize nausea) 4 mg IV every 8 hours, 30 minutes before each DHE infusion. If the patient has baseline nausea, consider using 8 mg ondansetron as premedication. When available, domperidone 10–20 mg orally or by suppository may be used.

Day 1: First dose: 0.5 mg in 100 mL of normal saline over 1 hour

If well tolerated, escalate dosing as follows:

Second dose, 8 hours later: 0.75 mg in 250 mL of normal saline over 1 hour

Day 2–5: Third and subsequent doses: 1 mg in 250 mL of normal saline over 1 hour every 8 hours for 10 doses with the goal of a cumulative total dosage of 11.25 mg (± 1 mg)

Pediatric dosing: weight-based dosing recommendations

Dosing should be adjusted and may require some individualization:

Dose (mg) = (adult dose in mg) × (patient weight in kg) × (0.014) mg

Side effect management If the patient has moderate or severe nausea, even with the routine premedication with ondansetron, consider:

1. Increasing the ondansetron dose, either by increasing the standing order to 8 mg every 8 hours or by adding 4 mg as an every 8 hour PRN dose to the 4 mg every 8 hours routine, standing order.

2. Add in another antiemetic such as promethazine 12.5–25 mg IV every 12 hours as needed.

3. Slowing the rate of infusion to over 2 or 3 hours.

4. Not escalating the dose or if already at 1 mg, consider reducing the dose to the highest that the patient can tolerate. For muscle cramping or joint pain, consider naproxen 500 mg every 12 hours as needed

Headache Questionnaire 

How many headache days in last 90 days? 

Average duration of headaches? 

Average severity of headaches? 

How many headache days were you disabled by 50% or more? 

 Headache onset age: 

Frequency of headaches: days/month

Frequency of moderate-severe headache or migraine:  days/month ( days/week on average)

Duration: - hours

Quality: pounding, squeezing, shooting

Severity: /10

Location: cervico-occipital, bitemporal, retrobulbar/periorbital

Palliative factors: rest, sleep, quiet/dark room, ice pack over eyes, Tylenol

Triggers: physical activity, bright lights, flickering lights, certain head/neck movements (neck extension), weather changes, noises, exercise, reading, hormonal changes, strong odors, menstrual cycle, missing a meal

Aura: none

Associated features/symptoms: photophobia, phonophobia, nausea, blurred vision, diplopia, flashing lights/floaters, vertigo, feeling heartbeat in head, numbness/tingling, difficulty speaking, difficulty concentrating, trouble sleeping, eye watering, generalized weakness

- denies vision loss/ocular pain, denies pulsatile tinnitus

Headaches triggered by any of the following?

            - coughing/sneezing: no

            - bending forward: no

            - physical activity: no

            - sexual activity: no

            - Valsalva maneuver: no

Positional component to headache? no

Family history of headaches/migraine? no

Family history of intracerebral hemorrhage or cerebral aneurysm? No

- maternal grandmother had Alzheimer's dementia (lived to be 93)

Sleep: 4-6 hours/night, not rested, snores

Caffeine intake: none

Mood: good

Previous CT/MRI brain? Yes - MRI brain (9/2020)

Previous Lumbar puncture? No

Previous sleep study? Yes - admits to hx of OSA, intolerant to CPAP/BiPAP; trying to find dentist that can fit her for OMAD

Prior abortive therapies tried: acetaminophen 1500 mg (taking 2x/week), sumatriptan PO and intranasal (caused vomiting), zolmitriptan (caused vomiting), diclofenac, ketorolac, meloxicam, muscle relaxant, metoclopramide, diphenhydramine, tramadol, narcotics.

 Prior preventative therapies tried: topiramate 50 mg BID (on currently), nortriptyline 25 mg (on currently - started on 5/22), pregabalin (75 mg in AM/150 mg in PM), verapamil (tried for 7 weeks, caused side effects - worsened HA), valproic acid (caused significant weight gain), losartan (allergic), Escitalopram (allergic), gabapentin (did not work)

 Additional therapies/supplements tried: Currently on FOD-MAP, diabetic, and IC diet; acupuncture, chiropractic therapy, sleep modification


Medications Used

Prior prophylactic medications: 

Antihypertensives:

(A) Propranolol 120 mg/d - improvement in palpitations

(A) Metoprolol - no

(C) Candesartan - no

(C) Lisinopril - no

(U) Verapamil - no

(A) Timolol - no

(B) Nadolol - no

(B) Atenolol - no

 

Antidepressants:

(B) Amitriptyline 10 mg/d - ineffective

(B) Venlafaxine - no

Duloxetine 60 mg/d - ineffective

Nortriptyline - no

 

Antiepileptics:

(A) Divalproex - no

(A) topiramate - suboptimal, 100 mg/d - ???

(U) Gabapentin - no

zonisamide - suboptimal

 

Other:

(A) Botox - no

(C) Cyproheptadine - no

(U) Erenumab (Aimovig) - no

(U) Eptinezumab (Vyepti) - no

(U) Galcanezumab (Emgality) 120 mg/m - ineffective

(U) Fremanezumab (Ajovy) - no

(U) Rimegepant (Nurtec) - no

(U) Atogepant (Qulipta) - no

Tizanidine 16 mg/d - ineffective

 

Prior abortive medications: 

Common:

(A) Tylenol - ineffective

(A) Aspirin - ineffective

(A) Ketorolac - no

(A) Ibuprofen - ineffective

(A) Naproxen - ineffective

(A) Excedrin - ineffective

 

Triptans:

(A) Sumatriptan - no

(A) Rizatriptan - no

(A) Eletriptan - no

(A) Amerge 2.5 mg/d - effective for frontal headaches, ineffective for occipital headaches

(A) Frovatriptan - no

(A) Zolmitriptan - no

(A) Almotriptan - no

 

Other:

(A) DHE Nasal Spray - no

(B) DHE IV/IM/SubQ - no

(U) Nurtec - no

(U) Ubrelvy - no

(U) Reyvow - no

(U) Compazine 5 mg - effective

(U) Reglan - no

(U) Droperidol - no

(U) Reyvow - no

(C) Fioricet - no

 

Prior procedures:

Nerve blocks - ineffective