Endocrine disorder

1. Headache syndromes
Dr Okanga

2. Introduction
• Approximately one-half of the adult population worldwide is affected by a
headache disorder.
• The International Headache Society classification and diagnostic criteria can help
clinicians differentiate primary headaches (e.g., tension, migraine, cluster) from
secondary headaches (e.g., those caused by infection or vascular disease).
• A thorough history and physical examination, and an understanding of the typical
features of primary headaches, can reduce the need for neuroimaging, lumbar
puncture, or other studies
• The most common types of headaches are tension-type headaches, migraines,
and cluster headaches, which affect approximately 40, 10, and 1 percent of the
adult population, respectively.
• Most headache diagnoses are based entirely on the patient history. Only rarely
does physical examination provide clues to the diagnosis

3. Epidemiological factors
• Patient age, gender, and medical history may suggest specific
headache etiologies in some instances.
1. Patients older than 55 are at risk for temporal arteritis.
2. Young women are the population that is most likely to have
migraines.
3. Middle-aged men are susceptible to cluster headaches.
4. Obese young and middle-aged women are the patients most likely
to have pseudotumor cerebri.
5. Puerperal women and those with hypercoagulable states are at
greatest risk for cerebral venous sinus thrombosis.

4. Location of headache
• Most headaches are frontal or holocranial - location may not be
particularly helpful in establishing headache etiology.
• Occipital or nuchal headaches are most commonly tension headaches
or occipital neuralgia.
• Common causes of unilateral retro-orbital headaches are migraine
and cluster headaches.

5. Character – most helpful factor
• Dullness and squeezing - tension headaches, brain tumors, temporal
arteritis, and pseudotumor cerebri.
• Throbbing and pulsation - migraines and other vascular headaches.
• Sharp, stabbing pain is – cluster headaches and indomethacin-
responsive headaches.

6. Rapidity of onset
• Most headaches develop over minutes to hours,
• Sudden onset, severe headaches - require immediate attention, as
they often represent life-threatening neurological emergencies such
as subarachnoid hemorrhage, carotid artery dissection, and pituitary
apoplexy.

7. Duration
• Cluster headaches and indomethacin-responsive headaches are very
brief, usually lasting from just a few seconds to several minutes at a
time.
• Most headaches can last for half an hour at a time or for days on end.

8. Diurnal variation
• Most headaches, including tension headaches and migraines, develop
in the late morning or early afternoon.
• Cluster headaches and headaches caused by increased intracranial
pressure, which are usually worse at night and may awaken a patient
from sleep.

9. Associated symptoms - Aura
• is a neurological symptom that begins several minutes prior to
migraine onset and most commonly takes the form of visual
hallucinations of flashing lights, lightning strikes, starburst patterns, or
distortions in size such as micropsia and macropsia.
• Auras may have sensorimotor manifestations including tingling,
weakness , and numbness .
• Aura symptoms characteristically spread over 15–20 minutes, which
distinguishes them from stroke (sudden-onset symptoms) and seizure
(symptoms that develop over seconds).

11. Other neurological symptoms
• Sudden unilateral or sequential visual loss - suggests temporal
arteritis.
• Seizures or other rapidly developing, fixed neurological signs point to
serious pathologies including intracranial hemorrhage, mass lesions,
venous sinus thrombosis, encephalitis, or hypertensive
encephalopathy

12. Systemic symptoms
• Nausea, vomiting, photophobia, and phonophobia frequently
accompany migraine.
• Conjunctival injection, lacrimation, and rhinorrhea are features of
both cluster and indomethacin-responsive headaches
• Visual loss, scalp tenderness, jaw claudication (pain with chewing),
low-grade fever, and proximal muscle tenderness - systemic
symptoms of temporal arteritis

13. Exacerbating factors
• High stress levels worsen almost all headaches.
• Actions that strain the neck including excessive head turning, staring
at a computer screen for prolonged periods, and even sleeping in the
wrong position tend to precipitate tension headaches.
• Not eating, poor sleep, excessive caffeine or caffeine withdrawal, the
menstrual period, chocolate, cheese, and red wine all exacerbate
migraine headaches.
• Lying flat and sleeping worsen headaches due to increased
intracranial pressure, while standing precipitates or worsens low-
pressure headaches secondary to spontaneous intracranial
hypotension or lumbar puncture.

14. Alleviating factors
• Rest in a quiet, dark room improves migraines.
• Loosening a tight necktie, massage, or applying heat - helps tension
headaches.
• Lying down improves low-pressure headaches, while standing up
improves headaches due to increased intracranial pressure.

15. Frequency
• cluster headache – multiple episodes of headache occur within a span
of a few weeks or months,
• Patients with frequent migraine attacks may benefit from prophylactic
medication, while those with infrequent attacks need symptomatic
treatment only.

16. Disability
• Headache that frequently interferes with daily commitments warrants
treatment.

20. Sudden-onset dangerous
headache syndromes
SAH, CAROTID DISSECTION, PITUITARY APOPLEXY

21. Subarachnoid hemorrhage
• SAH due to aneurysmal rupture.
• S/S - Headache is usually severe and builds to a climax in just a few
seconds and is sufficiently jarring to stop patients in their tracks.
• Vomiting and stiff neck, seizure and loss of consciousness occur at
onset.
• Some patients may have a sentinel headache due to leakage of a
small amount of blood from the responsible aneurysm and preceding
the SAH by up to 2 weeks.
• Incidence of sentinel headache – up to 40%

22. SAH investigations
• Noncontrast head CT, the first diagnostic test.
• Likelihood of finding subarachnoid blood after SAH ↓ with time
1. 95% between 0 and 1 days after rupture
2. 90% between 1 and 2 days after rupture
3. 85% at 5 days after rupture
4. 50% at 1 week after rupture
5. 30% at 2 weeks after rupture
6. almost zero at any time after 3 weeks

23. SAH - Tx
• If SAH suspected and CT is negative - lumbar puncture to look for
subarachnoid blood (Xanthochromic CSF, Bilirubin levels in the CSF)
• Once SAH confirmed – Conventional angiography (CTA and MRA may
miss aneurysms < 5mm)
• Treatment – Coiling preferred, if no expertise then clipping.

24. Unruptured aneurysm
• Are almost always asymptomatic and are noted when neuroimaging is
performed to evaluate headache, memory loss, or another vague
neurological complaint.
• Risk for aneurysmal rupture must be weighed against the risk for
surgical or endovascular interventions.
• International Study of Unruptured Intracranial Aneurysms ISUIA DATA
– 1 year risk for death or poor neurological outcome is approximately
13% for open surgical clipping and 9% for endovascular coiling.
• Poor out come risks - older age, larger aneurysm size, and posterior
circulation location

25. 5-year risk for aneurysmal rupture from the International Study of Unruptured
Intracranial Aneurysms (ISUIA)

26. SAH …
• The patients with the most obvious benefit from intervention are
younger patients with aneurysms between 7 and 24 mm in diameter.
• Patients with aneurysms smaller than 7 mm in diameter or
asymptomatic intracavernous aneurysms should not undergo
intervention

27. Carotid artery dissection
• May produce a sudden-onset, severe headache.
• Precipitants - Trauma, especially that caused by vigorous exercise, yoga, or
chiropractic manipulation,
• Headache – typically unilateral, throbbing, and retrobulbar, (maybe
misdiagnosed as migraine)
• Diagnosis - CTA or MRA of the neck.
• Complication – embolic stroke; thrombus at the dissection site is fertile
ground for small clots, which are thrown distally into the anterior
circulation.
• Antiplatelet therapy or anticoagulation required to prevent distal
embolization (risk of stroke with either therapy is 2%)

28. Pituitary apoplexy
• Rapidly growing pituitary adenoma - outstrips its vascular supply →
infarction of the pituitary gland.
• S/S – Sudden onset headache, bitemporal hemianopsia (compression
of the optic chiasm) and ophthalmoplegia (involvement of the CN III
in the adjacent cavernous sinus).
• Biggest risk – Hypotension (from the acute loss of ACTH).
• Treatment – dexamethasone (4 mg IV) to prevent adrenal
insuffciency, and intravenous fluid boluses to maintain adequate BP.
• Definitive management- endocrinologist (hormonal replacement)
and neurosurgeon ( removal of tumor)

29. Subacute and chronic dangerous
headache syndromes
Temporal arteritis, CVST, meningitis, SOL, pseudotumor cerebri, Hypertensive
encephalopathy.

30. Temporal arteritis (Giant cell arteritis)
• is a vasculitis of medium- and large-sized arteries characteristically
involving one or more branches of the carotid artery, particularly the
temporal artery.
• Age group - >55yrs
• Headache in GCA/TA has no specific features.
• Clues - monocular visual loss, jaw claudication, scalp tenderness, and fever.
• Polymyalgia rheumatica , characterized by pain in the shoulders and hips,
frequently accompanies temporal arteritis.
• Diagnosis - ↑ESR and CRP, Temporal artery biopsy confirms diagnosis.
• Treatment – Steroids for > 1yr.

31. Cerebral venous sinus thrombosis
• S/S - headache, seizures, encephalopathy, and venous strokes.
• Takes several weeks to develop, earliest stages - nonspecific
headache that resembles migraine or tension headache
• Population at risk - Women in the puerperium, oral contraceptives
use, Sepsis, malignancy, dehydration and hypercoagulable states
• Neurological examination may be normal, may show an
encephalopathy, or may show focal signs that reflect a venous stroke.
• Contralateral leg weakness due to superior sagittal sinus thrombosis
is particularly suggestive of stroke due to CVST.

32. CVST investigations and treatment
• Neuroimaging - positive delta sign on contrast-enhanced CT scan,
which indicates collateral channels surrounding a torcular thrombus.
• A CT scan may also show hemorrhagic infarction or cerebral edema.
• In milder cases, the diagnosis is made only with the aid of magnetic
resonance venography (MRV).
• Complications – seizure, ↑ ICP, stroke.
• Treatment – Heparin and warfarin for 3 – 6 months (INR 2-3)

33. Meningitis
• characterized by fever and sometimes by headache and stiff neck.
• Headache may be the most prominent or a solitary symptom in
immunocompromised patients

34. Headache secondary to mass lesions
• Classically worse while recumbent, may awaken the patient in the
middle of the night, and may be associated with focal neurological
signs or seizures.
• Classical presentation – rare, most patients have headaches that
resemble tension headaches.

35. Pseudotumor cerebri (idiopathic intracranial
hypertension )
• Etiology – uncertain.
• S/S – headache (non specific resembling tension headache) and visual
loss (blurriness or transient orthostatic visual loss progressing to
blindness)
• Pop at risk - young, obese women (hence diagnosis often missed in
other groups).
• Precipitants – obesity, excess vitamin A, lithium, tetracyclines, and
both steroid administration and withdrawal.

36. Pseudotumor cerebri diagnostic tests
1. CT scan or MRI of the brain to exclude the possibility of true tumor
or another cause of ↑ ICP.
2. LP - opening pressure of at least 20 cm H₂O.
3. Dilated funduscopic examination to look for papilledema.
4. Formal perimetry to determine the exact extent of visual field loss.

37. Treatment of pseudotumor cerebri
• Discontinue any potentially responsible medications.
• Weight loss
• acetazolamide , administered at doses ranging from 250 mg bid to
1000 mg bid.
• serial lumbar punctures (impractical for doctor and patient alike)
• If serial lumbar punctures provide consistent relief,
→ventriculoperitoneal shunting as a more permanent method to
remove CSF.
• Patients with rapidly progressive visual loss may need optic nerve
sheath defenestration to prevent complete blindness.

38. Hypertensive encephalopathy
• Occurs in patients with either very high blood pressure or rapid
increases in blood pressure.
• S/S – Headache, seizures, visual loss, confusion, any focal neurological
finding.
• Frequently accompanied by other problems related to malignant
hypertension including angina, pulmonary edema, and renal failure.
• Treatment - lower the blood pressure to prevent irreversible
neurological and systemic damage.

39. “Benign” headaches
Migraine,

40. Migraine
• Typical migraine - characterized by a throbbing, unilateral headache
associated with nausea, vomiting, photophobia, and phonophobia.
• May be preceded by a variety of aura symptoms - visual loss, scintillating
scotoma, tingling in the extremities, and even weakness resembling stroke.
• M:F =1:3 Age of 1st attack – teens – early 20s, rare for the disorder to first
appear after age 50.
• Diagnosis – made on clinical grounds, with neuroimaging studies being
used to exclude other more serious conditions.
• Treatment – Lifestyle adjustments, abortive medication and prophylaxis.

41. Migraine without Aura ICHD -3 Criteria
A. At least five attacks fulfilling criteria B-D
B. Headache attacks lasting 4-72 hr (untreated or unsuccessfully treated)2;3
C. Headache has at least two of the following four characteristics:
• unilateral location
• pulsating quality
• moderate or severe pain intensity
• aggravation by or causing avoidance of routine physical activity (eg, walking or
climbing stairs)
D. During headache at least one of the following:
• nausea and/or vomiting
• photophobia and phonophobia
E. Not better accounted for by another ICHD-3 diagnosis.

42. Other migraine variants
• Migraine with aura - aura is accompanied or followed within 60
minutes by headache with or without migraine characteristics.
• Typical aura without headache - Migraine with typical aura in which
aura is neither accompanied nor followed by headache of any sort.
• Hemiplegic migraine - Migraine with aura including motor weakness.
• Basillar migraine ( Migraine with brain stem aura)

43. Migraine with brainstem aura
• Attacks fulfilling criteria for Migraine with aura and criterion below. Aura with
both of the following:
1. at least two of the following fully reversible brainstem symptoms:
2. a) dysarthria
3. b) vertigo
4. c) tinnitus
5. d) hypacusis
6. e) diplopia
7. f) ataxia not attributable to sensory deficit
8. g) decreased level of consciousness (GCS ≤13)
9. no motoror retinal symptoms.

44. • Migraine headache in children and adolescents (aged under 18 years)
is more often bilateral than is the case in adults; unilateral pain
usually emerges in late adolescence or early adult life. Migraine
headache is usually frontotemporal
• Very frequent migraine attacks are now distinguished as Chronic
migraine. When there is associated medication overuse, both
diagnoses, Chronic migraine and Medication-overuse headache,
should be applied.

46. Migraine pathogenesis
• Various theories have been proposed – vascular, neurovascular,
cortical spreading depression etc.
• Currently the underlying process is believed to be sensory sensitivity
due to dysfunction of monoaminergic sensory control systems located
in the brainstem and hypothalamus.

47. Lifestyle modifications
• Ask patient about migraine precipitants such as sleep deprivation,
stress, not eating, menstruation, and foods such as chocolate, cheese,
caffeine, and red wine.
• Headache diary – Helpful to keep track of these exposures and the
consequent migraine frequency.
• Lifestyle modifications may reduce headache frequency considerably
or even cure the headaches in a very small minority.

48. Abortive medication
• 1st line - acetaminophen, ibuprofen, or naproxen,
• 2°line - triptan (serotonin 1B/1D agonist); effective in aborting migraines if
given early enough in the course of an attack: ideally, a patient should take
the triptan within 30 minutes of developing a headache, but they may still
benefit up to 3 hours after headache onset.
• side effects of triptans - chest pain, flushing, nausea, and grogginess.
• Triptans should be avoided in patients with cardiovascular or
cerebrovascular disease and in those who are taking monoamine oxidase
inhibitors
• 3 or more attacks per month or attacks that interfere with the patient’s
ability to work or to attend school – Propylaxis.

54. Tension headache.
• bifrontal, holocranial, nuchal, or occipital squeezing or tightness.
Severe tension headaches may be accompanied by nausea and
vomiting, symptoms that are more typical of migraines
• Precipitants - neck strain, sitting still for a prolonged time, and
sleeping in an awkward position.
• Treatment – Mild analgesics (acetaminophen or ibuprofen),
• Severe attacks not responding to NSAIDS - Heat application and
stretching exercises may help. Muscle relaxants such as diazepam (2–
5 mg tid), metaxalone (400–800 mg bid), baclofen (10–40 mg bid), or
cyclobenzaprine (5–10 mg tid) are also often helpful.

55. Tension headache diagnostic criteria
• Lasting from 30 minutes to 7 days
• At least two of the following four characteristics:
• bilateral location
• pressing or tightening (non-pulsating) quality
• mild or moderate intensity
• not aggravated by routine physical activity such as walking or climbing stairs
• Both of the following:
• no nausea or vomiting
• no more than one of photophobia or phonophobia
• Not better accounted for by another ICHD-3 diagnosis

56. Tension headache variants
• Infrequent episodic tension-type headache - At least 10 episodes of
headache occurring on <1 day/month on average (<12 days/year) and
fulfilling criteria for tension headache. May or may not be
accompanied by pericranial tenderness.
• Frequent episodic tension-type headache - At least 10 episodes of
headache occurring on 1-14 days/month on average for >3 months
(≥12 and <180 days/year) and fulfilling criteria ± Pericranial
tenderness.
• Chronic tension-type headache - Headache occurring on ≥15
days/month on average for >3 months (≥180 days/year), fulfilling
criteria ± pericranial tenderness.

57. Cluster headaches
• Daily bouts of one to more attacks of relatively short-duration unilateral
pain for 8–10 weeks a year;
• Attacks last seconds to minutes.
• Pain is retroorbital, excruciating, nonfluctuating, and explosive in quality
• At least one of the daily attacks of pain recurs at about the same hour each
day for the duration of a cluster bout.
• Ipsilateral Horner’s syndrome is a frequent finding.
• Patients with cluster headache tend to move about during attacks, pacing,
rocking, or rubbing their head for relief; some may even become aggressive
during attacks.
• M;F 3:1 50% of the attack occur at night waking the patient up.

58. Cluster headache diagnostic criteria
A. At least five attacks fulfilling criteria B-D
B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180
minutes (when untreated)1
C. Either or both of the following:
1. at least one of the following symptoms or signs, ipsilateral to the headache:
• conjunctival injection and/or lacrimation
• nasal congestion and/or rhinorrhoea
• eyelid oedema
• forehead and facial sweating
• miosis and/or ptosis
2. a sense of restlessness or agitation
C. Occurring with a frequency between one every other day and 8 per day
D. Not better accounted for by another ICHD-3 diagnosis

59. • Episodic cluster headache - attacks occurring in periods lasting from 7
days to one year, separated by pain-free periods lasting at least 3
months.
• Chronic cluster headache - attacks occurring for one year or longer
without remission, or with remission periods lasting less than 3
months

60. Cluster headache treatment
• Acute treatment - inhaled 100% oxygen, triptans or intranasal
lidocaine (1 ml 4% solution).
• Prophylaxis - verapamil (120–240 mg qd) or lithium (300 mg bid,
titrated to 600 mg bid with a goal plasma level between 0.6 and 1.2
mmol/l).

61. Chronic paroxysmal hemicrania
• Characterized by brief, episodic unilateral headaches, which are
accompanied by conjunctival injection, tearing, and rhinorrhea.
• Most frequent in young women, sometimes confused with migraine
or cluster headaches.
• It is distinguished from other forms of headache by its exquisite
sensitivity to indomethacin at doses of 25–100 mg bid–tid.

62. Visual strain headaches
• Following excessive reading, TV watching, or computer work, patients
note an aching or burning pain behind the eyes accompanied by
ocular fatigue and sometimes by conjunctival injection.
• The pain often radiates into the forehead or temples.
• Correction of refractive errors often helps to reduce the frequency
and severity of these headaches.

63. Medication-related headache
• Headache is listed as a side effect in the product inserts of almost
every medication.
• Common offenders include beta-blockers, cyclosporine, dipyridamole,
isotretinoin, and vasodilators such as nitroglycerin.
• It is often difficult to distinguish between headaches caused by
medication and those that are caused by a primary headache
disorder.
• A brief trial of withdrawing the presumed precipitant may be
warranted.

64. Postlumbar puncture headaches
• Occurs in 20–30% of patients following lumbar puncture,
• Caused by persistent leakage of CSF through the puncture site.
• Headache develops between 1 - 2 days after the LP is performed, Fronto-
occipital bilat, appear within seconds of assuming an upright position,
relieved by lying flat.
• Treatment - patient to lie flat for 24 hours and drink caffeinated,
carbonated beverages. If symptoms do not resolve, try the combination of
caffeine/butalbital/acetaminophen for no more than 72 hours.
• If conservative mx fails - epidural blood patch to promote sealing of dural
tear.

65. Spontaneous intracranial hypotension
• caused, by a traumatic dural tear with resulting spinal fluid leakage.
• Holocranial or occipital headache develops suddenly and occurs when
the patient is upright, and improves or resolves completely when they
lie flat. The patient may report vertigo and diplopia, and some
patients develop sixth nerve palsies.
• Imaging – sagging of the cerebellar tonsils and diffuse
pachymeningeal enhancement
• Treatment - aggressive hydration, caffeine, and epidural blood
patches, surgical closure if no improvement.

66. Headache in pregnancy
• Uncommon but dangerous causes of headache that may develop during
pregnancy include cerebral venous sinus thrombosis, eclampsia, and
pituitary apoplexy.
• Any woman without a prior history of headaches should undergo MRI of
the brain, especially if the headaches are associated with neurological
findings.
• Most common headache types in pregnancy - migraines (tends to improve
in frequency and severity during pregnancy) and tension headache
(worsens during pregnancy).
• Treatment – Avoid medicines if possible, paracetamol, ice packs, codeine or
short courses of NSAIDS (Avoid in late trimester 3),
• Consider propranolol or nortryptiline for women who require migraine
prophylaxis

67. Status migrainosus
• Migraine that lasts continuously or >72 hours.
• Precipitants - noncompliance with prophylactic medications, stress, poor
sleep, and head trauma.
1. Place the patient in a dark, quiet room and obtain intravenous access to
administer medications and replace fluids.
2. DVT prophylaxis
3. Antiemetic agents – Promethazine, Prochlorperazine Ondansetron
4. Acute pain – Ketorolac iv, Narcotics
5. Dihydroergotamine (1 mg IV), vasoconstrictor and serotonin 1B/1D
agonist may help abort migraine symptoms, even after 72 hours of
symptoms
6. Sleep aid – Diazepam iv

68. Assignment
Chronic daily headache
Trigeminal neuralgia
Glossopharyngeal neuralgia
Sinus headache