APPROACH TO HEADACHE AND CRANIO FASCIAL PAIN.pptx

headache and craniofacial pain , differentials and approach

1. HEADACHE AND CRANIO FASCIAL PAIN Dr Ashish sharma SR NEUROLOGY

2. APPROACH TO HEADACHE Primary headache disorders are the most frequent types encountered in OPD. Primary headache disorders Diagnosis is primarily on the history basis ---- Number of lifetime episodes needed to diagnose the headache disorder Minimum required characteristics of the headache Minimum requirements for associated symptoms ( eg , photophobia, nausea, trigeminal autonomic features) Not attributable to another ICHD-3 disorder

3. Secondary headaches have a common diagnostic structure as well: A secondary cause was diagnosed (ICP disorder, SAH, tumor, infection, inflammatory condition) There is a temporal relationship between the onset of the headache and the secondary diagnosis. The headache improves or resolves when the underlying cause is treated

4. WHAT TO ASK? What is the primary concern that brings you here? Tell me about your headaches ? Do you get any warning that you are getting a headache before the pain starts? Let’s talk about how your head pain starts. where is it located at first? Does it spread or travel? How would you describe the pain? what does it feel like?

5. How severe is the pain when it starts and how severe does it get? How long does it take for the pain to reach peak intensity? Do your headaches occur at any particular time of the day or night? Do they awaken you from sleep? are they present upon awakening? Are there other symptoms of a primary headache disorder?

6. what do you prefer to do (physically) during an attack? Is your skin sensitive to touch during the headache? How long does the severe pain last? How many days in one month are you completely headache free and “clear headed”? Do any of your family members have headaches (or the patient’s specific type)?

7. RED FLAGS OF HEADACHE

8. PHYSICAL EXAMINATION Vital signs (blood pressure, temperature, height, weight, heart rate) General neurologic examination, a fundoscopic examination Palpation of cranial peripheral sensory nerves for tenderness or pain reproduction Nuchal range of motion and palpation for tenderness. Neck circumference and Mallampatti grade

9. Assessment of jaw opening distance, crepitus , tenderness. Palpation of the temporal arteries. Otoscopic examination. Trendelenburg test (This is useful for evaluating patients with a suspected spinal CSF leak. Place the patient in 5 to 10 degrees of Trendelenburg for 5 to 10 minutes.) Improvement of headache or other symptoms in the Trendelenburg position is highly suggestive of this diagnosis

10. CLASSIFICATION OF HEADACHE Part One: The Primary Headaches 1. Migraine 2. Tension-type headache 3. Trigeminal autonomic cephalalgias 4. Other primary headache disorders Part Two: The Secondary Headaches 5. Headache attributed to trauma or injury to the head and/or neck 6. Headache attributed to cranial and/or cervical vascular disorder 7. Headache attributed to non-vascular intracranial disorder 8. Headache attributed to a substance or its withdrawal 9. Headache attributed to infection 10. Headache attributed to disorder of homoeostasis 11. Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure 12. Headache attributed to psychiatric disorder Part Three: Painful Cranial Neuropathies, Other Facial Pain and Other Headaches

11. MIGRAINE migraine term derives -- ancient Greek word hemikranios means half head. Four phases: the premonitory , aura, headache and postdrome . Migraine 1-year prevalence of 12% including 18% of women and 6% of men. Attacks 1–4 times per Month are considered to have “episodic migraine

12. 2% of population has “chronic migraine,” have headaches on at least 15 days per month, including at least 8 days on symptoms of full-blown migraine attacks. Mc migraine attack triggers – emotional stress, fluctuating female hormones, missed meals, weather factors, sleep deprivation, odors, certain visual stimuli, alcohol, muscle tension, physical exercise, overheated

13. MIGRAINE WITHOUT AURA Diagnostic criteria for Migraine without aura : A . At least five attacks fulfilling criteria B-D B . Headache attacks lasting 4-72 hours C . Headache has at least two of the following four characteristics: 1. unilateral location 2. pulsating quality 3. moderate or severe pain intensity 4. aggravation by or causing avoidance of routine physical activity D . During headache at least one of the following: 1. nausea and/or vomiting 2. photophobia and phonophobia E. Not better accounted for by another ICHD-3 diagnosis .

14. Migraine with aura Diagnostic criteria: A . At least two attacks fulfilling criteria B and C B . One or more of the following fully reversible aura symptoms: visual(mc 90%) , sensory , speech and/or language , motor brainstem, retinal C . At least three of the following six characteristics: 1. at least one aura symptom spreads gradually over 5 mins 2. two or more aura symptoms occur in succession 3. each individual aura symptom lasts 5-60 minutes 4. at least one aura symptom is unilateral 5. at least one aura symptom is positive 6. the aura is accompanied, or followed within 60 minutes, by headache D . Not better accounted for by another ICHD-3 diagnosis .

15. Migraine with brainstem aura –Criteria of Migraine with aura + Aura with both of the following: 1.At least two of the following fully reversible brainstem symptoms: A.Dysarthria,b.vertigo,c.tinnitus,d.hyperacusis E. Diplopia f.ataxia g. GCS<13 2. no motor or retinal symptoms. Hemiplegic migraine :Criteria of migraine with aura + Aura consisting of both of the following: 1. fully reversible motor weakness 2. fully reversible visual, sensory ,language symptoms.

16. PATHOPHYSIOLOGY Vascular versus neuronal : primarily due to brain dysfunction, perhaps with secondary vascular effect. Historically, the migraine aura due to cerebral vasoconstriction and headache was thought due to cerebral vasodilation . Evidence building against the role of the intracranial vasculature in generating the migraine attack.

17. Atypical brain function in areas -- responsible for processing pain, visual and auditory stimuli, olfactory stimuli, regulating sleep wakefulness, and awareness. A migraine generator. Brain region responsible for triggering migraine. FMRI:functional coupling of the hypothalamus(premonitory symptoms) with spinal trigeminal nuclei and with the dorsal rostral pons or individually act as trigger.

18. Cortical spreading depression: CSD is considered the electrophysiological substrate of the migraine aura. CSD tends to start in the occipital lobe and spreads forward over the cerebral hemisphere at a rate of about 2–4 mm/min. Some data suggest that CSD evokes a series of cortical, meningeal , and brainstem events consistent with the development of headache.

19. The trigeminocervical system and migraine headache Basic anatomical system that underlies the migraine attack. consists of the trigeminal and cervical sensory afferents from the pain-sensitive intracranial and extracranial structures . projections to the trigeminal ganglion and trigeminal nucleus caudalis . Ascending fibers from the trigeminal nucleus caudalis to multiple brainstem . Activation of the trigeminocervical system leads to release of vasoactive neuropeptides from sensory afferents that innervate the major intracranial arteries, a process termed “ neurogenic inflammation

20. The hyperexcitable migraine brain F-MRI studies show brain to have greater stimulus-inducted activations of regions that facilitate the perception of stimuli. Brainstem regions that are primarily pain inhibiting have been demonstrated to hypoactivate in response to pain. Recurrent activation of the TVS can lead to peripheral and central sensitization. Sensitized neurons have lower thresholds for activation, increased spontaneous activity, and receptive field expansion.

21. Treatment and Management ● Complete pain relief at 2 hours after treatment should be the goal in acute migraine therapy Patients should keep acute migraine medications with them and treat at the first sign of an attack. ● Treatment should be matched to the severity of the headache.

22. ● Aspirin, ibuprofen, naproxen, diclofenac , and celecoxib -- Level A evidence . ● All seven triptans are more effective than placebo for acute migraine T/T .(50% of the time. S/C sumatriptan , rizatriptan and zolmitriptan orally disintegrating tablets and eletriptan tablets were the most effective formulations. Long half-lives of naratriptan and frovatriptan may reduce recurrence.

23. ● Acute migraine treatments in pregnancy are Metoclopramide , phenhydramine (adjunct therapy), triptans , and ondan . Subcutaneous sumatriptan can be used as a rescue 2 hours after shorter-acting triptans and 4 hours after longer-acting triptans . The total triptan exposure should be limited to 2 doses per 24-hour period. DHE nasal spray can be used 24 hours after a triptan dose. Indomethacin can be used if above are contraindicated .

24. PROPHYLAXIS OF MIGRAINE appropriate when attacks occur weekly or several times a month, ( disabiling or prolonged) This target dosage is maintained for at least 3 months. if there is a beneficial response, Continue till clinical stabilization for at least 6-12 months. β-Adrenergic blockers: doses of 80-240 mg/day, if tolerated, should be given a trial of 2-3 months.

25. CCB: prevent vasoconstriction prevention of platelet aggregation and alterations in release and reuptake of serotonin. Verapamil in doses of 80-160 mg three times a day reduces the incidence of migraine with aura Antidepressants :least 3 months after reaching a therapeutic dose. The optimal dose usually 50-150 mg for amitriptyline and nortriptyline ). Anticonvulsants: divalproex sodium (500-1750/day divided dose and topiramate gradually escalate(15-25mg/wk)to 75-200 mg/day

26. Other prophylactic cyproheptadine , methylsergide , magnesium, omnabotulinum A for chronic migraine not episodic. For patients 4-7 monthly headache days and at least moderate disability despite at least two 6-week trials of AAN level A or B preventive therapies, CGRP-based immunotherapy would be indicated. For patients reporting 8-14 monthly headache days, a CGRP-based immunotherapy is indicated at any disability level.. Finally, CGRP-based immunotherapies are indicated for patients with chronic migraine if they have not responded to either two 6-week oral preventive trials or two quarterly injection cycles of onabotulinumtoxinA .

27. MENSTRUAL MIGRAINE The attacks may occur exclusively during menstruation not on anytime during the cycle. k/a pure menstrual migraine (PMM). Menstrual related migraine : occurs whole cycle more during mestruation . occurs in up to 60% of female migraineurs . Menstrual migraines have more severe, disabling, and treatment-refractory. Estrogen withdrawal before menstruation is a trigger for migraine in some women.

28. Perimenstrual prophylaxis commences a few days before the period is expected and continues until the end of menstruation. In women whose cycles are difficult to predict,

29. CHRONIC DAILY HEADACHE “chronic” in CDH refers >15 days in tension type and migraine and >1yr with remission <3 month in cluster headache. Headache>4 hours include chronic migraine, chronic tension-type headache, hemicrania continua, and new daily persistent headache. less than 4 hours, include cluster headache, paroxysmal hemicrania , hypnic headache,SUNA primary exertional and cough headache.

30. TRIGEMINAL AUTONOMIC CEPHALGIA

31. Cluster Headache 90% have episodic and 10% have the chronic form. male predominance of 2:1 to 4:1, Peak age of onset in the twenties . first-degree relatives may have 18-times higher risk second-degree have a 1- to 3-times higher risk . autosomal dominant with variable penetrance;with autosomal recessive also found

32. During a cluster, typically 1 to 3 or more attacks in 24 hours. occur at similar times of day for several weeks to months. Onset during the night, or 1–2 hours after falling asleep is common. pain is strictly unilateral and almost always remains on the same side. the retro-orbital and temporal regions (upper syndrome) but may be maximal in the cheek or jaw (lower syndrome). Described as steady or boring and of terrible intensity (so-called suicide headache). 55 to 60% rate in the korean and american population.

33. Pain intensifies very rapidly, peaking in 5–10 minutes and persists for 45 minutes to 2 hours. Avoid the recumbent position as it increases pain intensity . More than 70% of patients have all five features of conjunctival injection, lacrimation , nasal congestion, rhinorrhea , and ptosis . superficial temporal artery may be visibly distended.

34. Differential diagnosis : Hypnic headaches have a circadian pattern, ( 15 to 240 minutes) without autonomic features. Migraine is differentiated on basis of long duration of headache. Trigeminal neuralgia, SUNCTand SUNA have cutaneous triggers. Tolosa hunt-3 rd CN workup includes vascular imaging of the head and neck [MRA] of the head and carotid ultrasound), pituitary function testing, polysomnography .(if no relief after t/t)

35. TREATMENT ACUTE TREATMENTS— SC sumatriptan intramuscular DHEand High-flow oxygen are first-line acute treatments for cluster headache. Non rebreather mask is highly preferred @15/min. BRIDGE TREATMENTS. Corticosteroids are the first-line bridge treatment ,occipital block, ergotamine tartarate . The first-line preventive medication is verapamil (80 mg 3 times a day) increases from 40 to 80 mg every 7-14 day. The newest available preventive medication is galcanezumab . Galcanezumab is the only CGRP blocker or CGRP-receptor blocker currently approved for cluster headache .

36. Lithium carbonate, 300 mg three times a day, can be given initially and the dose adjusted at 2 weeks to obtain a serum lithium level of about 1 Eq /L. The best position for O2 inhalation is sitting on the edge of a chair or bed and leaning forward with arms on knees PREGNANCY AND LACTATION. O2 and intranasal lidocaine are typically considered safe. Intranasal ketamine effective as an acute treatment .

37. Psycolibin -- The treatment protocol was 0.143 mg/kg given 3 times, with each dose separated by 3 to 7 days. Surgery: last resort radiofrequency thermocoagulation of the gasserian ganglion, trigeminal sensory rhizotomy , microvascular decompression of the trigeminal nerve, and sphenopalatine ganglion radiofrequency ablation . Neurostimulation procedures involving central or peripheral nervous system targets have been employed to treat refractory chronic cluster headache .

38. SUNA/SUNCT Prevalence 6.6 per 100,000 individuals. slight male predominance (1.5:1) peak onset between ages 35 and 65 years. The average attack duration is 61 sec and the frequency is 28 per day, with high variability. Sharp, stabbing, electric, or shooting. Cutaneous triggers with a refractory period which is not in trigeminal neuralgia. IV lidocaine (1 mg/kg/hr to 3.5 mg/kg/hr).single most effective treatment.

39. OTHER PRIMARY HEADACHE Primary Cough Headache: sudden onset, precipitated by a brief, nonsustained Valsalva maneuver such as coughing, laughing,sneezing , The mean age at onset is around 60 years. Chiari type 1 malformation mc abnormality, Others are middle cranial or posterior fossa tumors, brain mets, pituitary tumors, posterior fossa arachnoid cysts. spontaneous spinal CSF leaks secondary to CSF-venous fistulas headache occurred in isolation to Valsalva maneuvers in 12% of patients; The treatment of choice is indomethacin ,

40. Primary Exercise Headache B/L throbbing headache that is precipitated by prolonged physical exercise. onset for primary exertional headache was 40 years. The headache is not explosive in onset but rather builds in intensity and lasts between 5 minutes and 48 hours. beta-blocker or indomethacin on a daily basis is effective in some primary exertional headache patients

41. Primary Headache Associated With Sexual Activity dull bilateral ache increases with sexual excitement. “ preorgasmic headache” abrupt explosive headache at orgasm “orgasmic headache ” Mean duration 30 min . male predominance. Indomethacin (25–50 mg) given 30–60 minutes prior to sexual activity .

42. Nummular Headache coin-shaped headache meningiomas , arachnoid cysts, and fibrous dysplasia of the skull mimics similar type. gabapentin (300–1800 mg daily), tricyclic antidepressants, and onabotulinumtoxinA . pressure or stabbing is frequently accompanied by hypesthesia , dysesthesia , paresthesia , allodynia .

43. New Daily Persistent Headache daily headache that is unremitting from onset or very soon after onset. Infection, flu-like illness, surgery, and stressful life events may precede NDPH. either migraine or tension-type headache Rule out secoundry cause and then on type of headache give prophylaxis.

44. TRIGEMINAL NEURALGIA Trigeminal Neuralgia:”tic douloureux .” Mc cranial neuralgia Brief, lancinating , shock like neuropathic pain in a localizable distribution. Lifetime prevalance of 0.16% To 0.3% . Slightly more common in women Average age of onset is in the sixth decade. classical trigeminal neuralgia refers evidence of vascular compression of the trigeminal nerve (by MRI or surgery), with associated nerve root atrophy or displacement.

45. Secondary trigeminal neuralgia is due to an underlying disease such as multiple sclerosis or ICSOL. In classical type, no sensory impairment, and the motor division of the nerve is intact. presence of sensory loss or masticatory muscle weakness suggests a secondary cause . Imaging with MRI to look for structural lesions such as a pontine lacunar infarct, demyelinating plaque, meningioma or schwannoma of the posterior fossa . High-resolution MRI and MRA to identify vascular compression in select cases

46. Vascular compression increases with age lead to focal demyelination of primary trigeminal afferents (i.e., nerve root entry zone) focal demyelination -- focal hyperexcitability , leading to repetitive neuronal discharges. TREATMENT: surgical exploration and decompression of the nerve. carbamazepine or oxcarbazepine –DOC Second-line – gabapentin , pregabalin , phenytoin , baclofen , lamotrigine , valproate , Topiramate .

47. MC performed surgical procedures include on the trigeminal nerve or gasserian ganglion ( rhizotomy ), Gamma Knife radiosurgery , and microvascular decompression. An alcohol block of the peripheral branch of the division of the trigeminal nerve 0.5–0.75 mL of absolute alcohol. percutaneous radiofrequency Thermocoagulation of sensory root as it leaves the gasserian ganglion is the procedure of choice.(93% success).

48. GLOSSOPHARYNGEAL OR OCCIPPITAL NEURALGIA felt in distribution of IX nerve and the sensory distribution of the upper fibers of the vagus nerve. Throat pain, the tonsillar region, the posterior third of the tongue, the larynx, the nasopharynx , and deep in the ear. Triggered by swallowing, speaking, laughing, or coughing and is unilateral in most patients. Secondary glossopharyngeal neuralgia may also be due to oropharyngeal malignancies, peritonsillar infections .

49. a paroxysmal jabbing pain in the greater (C2), lesser (C2–C3), and or third (C3) occipital nerve distribution. Severe stabbing, electric shock-like, or sharp shooting pain that starts at the nuchal region spreads toward the vertex. Paroxysms can start spontaneously. Cervical range of motion may be restricted, and local posterior neck muscle spasms may be found.

50. Nervus Intermedius Neuralgia brief paroxysms of pain felt deeply in the auditory canal. The intermediate nerve of Wrisberg (the nervus intermedius ), a small sensory branch of the CN VII, and the geniculate ganglion are believed to be the affected structures. Middle-aged women frequently affected. Nervus intermedius neuralgia can develop during an episode of Ramsay-Hunt syndrome

51. Neck-Tongue Syndrome paroxysmal sharp pain in the neck, occiput , associated with ipsilateral sensory changes in tongue. Early childhood to adulthood. subluxation of the atlantoaxial joint, may compromise the second cervical dorsal root during sudden neck rotation. Benign condition

52. REFERENCES CONTINNUM HEADACHE-2024 ICHD 3 CLASSIFICATION BRADLEY AND DAROFF”S NEUROLOGY IN CLINICAL PRACTISE – EIGHT EDITION HARRISON”S INTERNAL MEDICINE TEXTBOOK 21 ST EDITION