1. Headache: a clinical tour for residents
Headache a clinical tour for residents part 2:
Secondary headache disorders
Headache: a clinical tour for residents
March 2010
Lucy Vieira MD

2. Headache is the Most Common Symptom that Humans Experience
Headache is the most common symptom that humans experience. There was a major study in the 1950s of >1 million Americans demonstrating that headache was more common than sore throat, stomach ache, back pain, and all other physical symptoms.
>90% of individuals have experienced a headache in their lifetime.
Approximately 5% of the population in North America (and Europe and Asia and South America etc) has chronic daily headache (headache >15 days more month).

3. Classification of Headache
Primary headaches (No underlying cause)
Migraine
Tension-type
TACs
Other
Secondary headaches (Underlying cause)
Medication overuse
Head/neck injury
Space-occupying lesion (i.e. brain tumour)
Vascular cause (i.e. Subarachnoid hemorrhage, intracranial bleed)
Infectious cause (i.e. meningitis or upper respiratory tract infection)
+ many others
Headaches by definition are broken down into 2 main categories: primary headaches and secondary headaches.
Primary headaches by definition have no underlying medical cause.
The vast majority of headaches that primary care physicians encounter are primary headaches.
References:
Headache Classification Committee of the International Headache Society. Cephalalgia 1988; 8 (supp 7):1-96
>65% in patients older than 50
Headache Classification Committee of the International Headache Society,1988

4. Overall Approach to Headache
Think about primary headaches and the way that they present (ICHD) and secondary headaches and the way that they present..then think about secondary headaches presenting as primary headaches ----atypical features or treatment failures…
In migraine diagnosis, secondary headache disorders must first be ruled out, as they are occasionally symptoms of underlying organic disease. When red flags are present , diagnostic tests such as neuroimaging, lumbar puncture, and clinical laboratory studies are necessary for accurate diagnosis.
A detailed patient history is the best tool for diagnosing primary headaches. Physicians should elicit the age when headaches started, the location, frequency, intensity, and duration of pain, any associated symptoms, such as aura or dizziness, precipitating, aggravating, or ameliorating factors, and family history. Medication use, eating habits, and results of any diagnostic tests, such as MRIs or other neuroimaging tests, are helpful to the diagnosis.
During the physical exam, check for focal neurologic signs, and ask the patient about other neurologic symptoms. Take blood pressure and heart rate, perform a neurological examination, examine extracranial structures, sinuses, scalp arteries, temporomandibular joints, and check range of motion and presence of tenderness in the cervical spine and paraspinal/pericranial muscles.
Information gleaned from the history and physical exam can help differentiate primary headaches from secondary headaches, and will help determine the patient’s type of primary headache.
References:
Wolff HG, Silberstein SD, Lipton RB, Dalessio DJ. Wolff's headache and other head pain. Oxford University Press US; 2001, pages  
Any secondary Headache disorder can mimic a primary headache disorder
Wolff HG, et al., 2001

5. Chance of finding a lesion?
1876 consec pts (38yo); 2/3 non acute:1.2%
99% had normal neuro exam
Pituitary adenoma, arachnoid cyct, meningioma, hydrocephalus, chiari I, stroke, cavernoma, glioma..
Half treated surgically
Only one lesion not appreciated on CT
US headache consortium meta-analysis of patient with migraine and normal exam: 0.18% rate of significant pathology therefore imaging not indicated.
Neuroimaging is not needed in the vast majority of patients you see.
List some pathologies not seen on MRI – disections, low csf pressure, some gliomas.
Cephalalgia 2005;25: Neurology 1994;44:

6. IHS: Chronic Primary Headaches
Chronic Migraine
+/- medication overuse
New Daily
Persistent Headache (NDPH)
Chronic Daily Headache (CDH)
Chronic Tension
Type
Headache
Hemicrania Continua
90% of migraineurs have episodic migraine BUT 10% have chronic migraine – a particularly disabling sub-type of migraine. Many MDs do not realized that migraines can be “chronic”.
Advise them that chronic tension-type headache is a diagnosis of exclusion with no unique characteristics. The diagnosis should ONLY be made if there is a CLEAR psychosocial stressor initiating and perpetuating the headache and the patients should all be brought back for follow-up assessment.
References:
Headache Classification Subcommittee of the International Headache Society. Cephalalgia. 2004;24(suppl 1):1-160
Other
Headache Classification Subcommittee of the International Headache Society, 2004

7. What are the Secondary Causes of Chronic Headaches?
Generate interaction with the audience by getting them to generate a list of the secondary causes of chronic headaches.

8. Chronic Daily Headache: Secondary or Sinister Causes
Medication Overuse Headache (MOH)
Cervicogenic headache
Post-traumatic headache
Chronic Daily Headache (CDH)
Sleep apnea
Decreased ICP (spontaneous intracranial hypotension)
Medication Overuse Headache (MOH) is the most common cause of secondary headaches.
Educate them that cervicogenic headache is quite rare (despite what pain clinic physicians diagnose many patients with prior to initiating nerve blocks).
Other
Increased ICP (tumour/mass, pseudotumour cerebri, hydrocephalus)

9. What are the Headache “Red Flags”?

10. Headache Red Flags - “SNOOP”
Systemic symptoms (fever, weight loss)
Secondary risk factors (cancer, HIV/immunocompromised)
Neurologic symptoms or abnormal signs
Onset (i.e. new-onset chronic headache)
Older patient (i.e. new headaches at age >50 yrs)
Previous headache different (i.e. significant change in headache frequency or clinical features)
Positional component (i.e. increases when upright)
Provocative factors (precipitated by coughing, exercise, sex)
Validated pneumonic:
Note nocturnal headache/headache on awakening is a “yellow flag” not red flag as many migraineurs/cluster headache patients wake from sleep with their headaches.
Other “yellow flags” to consider are postpartum headache, headache and escalating medication requirements, headache following dental/ENT procedure, headache in the young obese.
Headache and the red eye, etc.
66% headaches after age 50 are primary headaches. Only 2% migraines start at this age. Only a percent of cluster starts at this age also.

11. Neurologic Examination of Headache Patients
Vitals (particularly BP)
Pupil symmetry, reactivity and fundoscopy
Visual fields
Eye movements
Motor – look for asymmetrical weakness R vs L
Reflexes – look for asymmetry (increased reflexes) R vs L
Sensation – extinction to double simultaneous tactile stimuli
Coordination – finger-nose-finger, gain and tandem gait
Examine/touch the head and neck
References:
Olesen J, Dodick D. The headaches. p 43-53: Lippincott Williams & Wilkins;  
Pryse-Phillips W, Dodick DW, Edmeads JG, et al. Guidelines for the diagnosis and management of migraine in clinical practice. Can Med Assoc J ;156(9):  
Olesen J , et al., 2006
Pryse –Phyllips WEM, et al., 1997

12. Part 2: The secondary headaches

13. Primary or secondary headache?
If pre-existing headache made worse by something else – use judgment: code as both or as the primary headache only
You have to show that the headache disappears when you treat the disorder. Until then, code as “probable”

14. Diagnosis Primary Headache only Primary and Secondary Loose Close
Temporal relation of the other disorder to headache worsening
Loose
Close
Degree of worsening
Slight
Marked
Evidence disorder causes secondary headaches
Weak
Strong
Other disorder eliminated
Headache unchanged
Headache improves
The Headaches, 3rd edition. Lippincott Williams and Wilkins 2006

15. 5. Headache attributed to head and/or neck trauma
Chronic is>3months

16. Post-Traumatic Headache
Secondary headache after injury
Should begin within 7 days of head injury (to meet IHS criteria)
Consider diagnosis: subdural, CSF leak, dissection
Headaches may resemble primary headache disorders (i.e. migraine, tension)
Often assoc with other “post- concussive” symptoms: vertigo, tinnitus, cognitive changes, sleep problems, depression, medication overuse
There is no evidence-based approach and no guidelines
Manage headaches in the usual way that you would treat the phenotypic headache type (i.e. migraine)
Rx with typical non-drug strategies
Rx with typical acute headache meds
Rx with typical preventative headache meds
Rx anxiety, insomnia, depression & post-traumatic stress disorder
Monitor for medication overuse

17. 6. Headache attributed to cranial or cervical vascular disorder
Up to 1/3 pts with stroke
6. Headache attributed to cranial or cervical vascular disorder
With stroke – usually not with lacunar and most common if dissection or basilar territory
Aura vs TIA – sudden vs progressive onset and progressive evolution with positive phenomena
Migrainous stroke – typical aura in pt known for MA lasts>60 min and positive neuroimaging
Unruptured vascular malformation – prevalence of aneurysm may be as high as 5% - long-term, most do not rupture. Risk of rupture below 7mm 0.1%/y

18. -anterior communicating artery (30-35%),
the bifurcation of the internal carotid and posterior communicating artery (30-35%) --the bifurcation of the middle cerebral artery (20%)
bifurcation of the basilar artery, and the remaining posterior circulation arteries (5%)
2-5% of the population
Unruptured aneurysm can cause TCH
MRA detection limit 5mm

19. Utility of CT and LP in SAH
CT: % sensitivity within 24h
80% at 3 days, 50% at 1 week.
MRI FLAIR (3-14 d more sensitive than CT)
LP may be negative less than 2 hours after the bleed;
most sensitive at 12 hours after symptom onset.
Xanthochromia (yellow-to-pink CSF supernatant) usually is seen by 12 hours
At 2 hours need to use spectrophotometry (oxyhemoglobin)
Xanthochromia results from lysis of RBCs with release of oxyhemoglobin, methhemoglobin and bili ( up to 2 weeks)

20. Symptoms Headache 85-95% Neck stiffness 74-84% n/v , photophobia 48%
Mental status 43%
Less common:
Focal deficit, seizures, coma, CN palsy, papilledema, ocular hemorrhage
*sentinel bleed
Excruciating headache may only last 1-2 hours (reason went to the ER). HEADACHE may last 3 to 8 days (var size hemorrhage)
About 50% pts have warning symptoms prior to major SAH – minor leak or expansion – “unusual headache – some TCH

21. First primary sexual or exertional headache
SAH has to be excluded as 1/3 of SAHs occur during activities such as bending, lifting, defecation or sexual intercourse.

22. Case 1 44 year old woman right sided headache and facial pain
Onset to peak pain – 1-2 minutes
1 week ago
Characterized as an intense aching throb “unlike my other headaches”
Associated with intermittent right sided pulsatile tinnitus
Are there any red flags here?
Would you consider investigations here?
Which ones?
CT scan of the brain and LP were normal.
adapted from D. Capobianco June 2006 AHS and Wityk, R. J. JAMA 2001;285:

23. Phx: migraine with/without aura Neurological exam: normal
Why coming now to MD?
“My husband thought I should because of ringing in my ear.”
Phx: migraine with/without aura
Neurological exam: normal

24. Ct scan and LP normal. Sent home after getting a little better with metoclopramide and meperidine IV.
Next day driving car and started to drive on the median and eventually taken to hospital by the police. There she was found to have a subtle right Horner's syndrome, mild slowing of left fine finger movements and left visual extinction.
A diagnostic test was done

25. Imaging of Stroke Wityk, R. J. JAMA 2001;285:2757-2762.
Copyright restrictions may apply.

26. Anatomy of Carotid Artery Dissection
2cm distal to carotid origin
-ends at skull base
Subintimal dissection
-stenosis
Mickey mouse ears: expansion by hyperintense hematoma of the outer lumen of the artery
Other Pt with dissection, Horner's and ipsilateral dysguesia and numbness on the back of the mouth/palate – involvement of the 9th cranial nerve. Dissection around C2
Wityk, R. J. JAMA 2001;285:
Copyright restrictions may apply.

27. Carotid Dissection Fronto-orbital headache before ischemia: 55-100%
Painful Horner’s, Painful tinnitus
Carotid bruit, dysguesia, ipsilateral neck pain, cerebral or retinal ischemia
Triggers: cough, sneeze, trauma
Risks: syphilis, Marfans, Ehlers-Danlos, FMD
Prognosis: good (60% resolve spont.;85% do well)
Headache 1 hour to many days before ischemia; in VBI often comes with other symptoms – wallenburg most common place for ischemia
Referred pain to the face/head unilat and neck originating from the artery – proven from stimulation studies. In VBD – posterior neck and occiput – anterior head regions – innervation by the upper cervical roots and convergence with TNC
If catch patient at the stage of headache alone – strict bedrest and maintain BP and monitor with CTA/MRA or doppler and consider heparin

28. Another case
Pt with dissection, Horner's and ipsilateral dysguesia and numbness on the back of the mouth/palate – involvement of the 9th cranial nerve. Dissection around C2
37 year old after a motorcycle accident Sara Mazzucco, MD; and Nicolo` Rizzuto, MD
Neurology 2006

29. cavernoma
A 45-year-old woman was referred for review of her headache. In 1997 she had a sudden onset of diplopia, which involved the range of movements, and right facial numbness. Both persisted for 3 months. A brain computerized tomography (CT) scan and magnetic resonance imaging (MRI) were performed and a left-sided brainstem vascular malformation was demonstrated, which was considered to have bled
From the time of onset of the diplopia the patient reported persistent right-sided temporal headache, contralateral to the MRI lesion. The persistent pain was punctuated two to three times a week by a severe right-sided throbbing headache with some added features of pressure. The more severe headache was associated with nausea, photophobia and phonophobia, as well as aggravation with movement. There were no cranial autonomic features. During the majority of attacks and lasting for most of the attack, but not preceding it, there was right-sided facial numbness that did not move. At varying points in her clinical course she had used both codeine- and paracetamol (acetaminophen)-containing compounds. These were largely unhelpful for the severe pain and their withdrawal did not alter the headache phenotype.
Her past history was remarkable for a mild head injury without loss of consciousness in She had never been treated with migraine preventatives. She had been otherwise relatively well until initial presentation, with no significant history of headache
On examination her cranial nerves were intact, including normal eye movements, with the exception of a right-sided reduction in facial light touch and pin-prick. Her limb examination, co-ordination and gait were normal.
MRI demonstrating a left-sided cavernoma (a and b) with an associated developmental venous anomaly (c) in the dorsal midbrain region adjacent to the periaqueductal grey matter.
Cephalalgia, Vol. 22, No. 2, (2002)

30. MS
MS patients with a plaque located within the midbrain, in proximity to the PAG, showed a four-fold increase in migraine- like headaches (odds ratio 3.91, 95% confidence interval 2.01 to 7.32; P < ) when compared to MS patients without a plaque in this location
Large series of MS patients. Headache very common but only lesions in this region significantly increased migraine headache.
Headache 2005 Jun;45(6):670-7

31. occipital arteriovenous malformation
34-year-old man started to experience monthly headaches: visual prodrome consisting of scintillating bright lights in the left visual field that slowly expanded over several minutes. Shortly after the visual symptom subsided, right-sided throbbing headaches developed along with nausea and vomiting, which usually lasted 2 to 4 hours. Normal neuro exam.
He was treated by radiosurgery with obliteration of the AVM and resolution of the headaches
34-year-old man started to experience periodic headaches in The attacks usually were heralded by a visual prodrome consisting of scintillating bright lights in the left visual field that slowly expanded over several minutes. Shortly after the visual symptom subsided, right-sided throbbing headaches developed along with nausea and vomiting, which usually lasted 2 to 4 hours. The headaches occurred about monthly. There were no noticeable triggering factors for the episodes. He had no prior episodes or family history of migraine or epileptic seizures. Results of neurological and ophthalmological examinations were unremarkable. Magnetic resonance imaging (MRI) and cerebral angiography disclosed an unruptured right occipital arteriovenous malformation (AVM) with a maximum diameter of 2 cm (Figure 1, A and B) that was treated by radiosurgery in November A single dose of 25 Gy was delivered to the margin of the lesion. The headaches gradually decreased in frequency and disappeared by 18 months after the treatment. Neuroimages performed 2.5 years after the irradiation showed complete obliteration of the AVM (Figure 1, C and D). No complications resulted from the treatment. At the time of this report, he had been free from headache for more than 3 years and remained neurologically intact. No prophylactic medication was used during the treatment course.
COMMENT
Occipital AVMs are well known for their occasional manifestations of visual disturbances and headaches, but it remains controversial whether those symptoms are distinguishable from genuine migraine.1-2 This case illustrates that the symptoms fulfilling the current operational diagnostic criteria for migraine with aura (International Headache Society, 1988)3 can be caused by an AVM in this location. Although the precise mechanism of the migraine in our patient is unknown, the gradual resolution of the symptom parallel to the progressive thrombosis of the AVM indicated that the AVM did cause the symptom. Migraine, as a symptom or a prodrome of organic diseases, is best known in the cases of migrainous strokes.4 Our case indicates that AVM also should be included as a possible lesion causing such "symptomatic migraines" and suggests that patients with late-onset migraine may be better screened by neuroimaging studies to rule out AVMs, which can be cured by surgical or radiosurgical treatment
Kurita, H. et al. Arch Neurol 2000;57:1219

32. A 23 year-old man with known migraine
CASE IN THE ER
A 23 year-old man with known migraine
Cc: generalized tonic-clonic seizure.
He had a second seizure in the emergency room.
He complained of severe dull occipital headaches of recent onset, different from his usual migraines. He denied head injury.
Clinical examination was unremarkable.
CT brain was significant for hemorrhage in the left frontal lobe and MRI shows bi-frontal (parasagital) hemorrhages.
CT and MRI are insensitive. MRV diagnostic

33. CT scan (1A) showing small left frontal hemorrhage (thick white arrow)
CT scan (1A) showing small left frontal hemorrhage (thick white arrow). Fluid attenuation inversion recovery (FLAIR) and diffusion weighted (DWI) sequences (1B and C) showing frontal infarcts (2 in left frontal, 1 right frontal, unfilled white arrows) bilaterally. Magnetic resonance venograms, MRV, showing left lateral sinus (1D, thin white arrow) and superior sagittal sinus (1E, dotted white arrows) filling defects, consistent with cerebral venous thrombosis. Figure 1E is a sagittal view of the venous phase of angiography showing superior sagittal sinus thrombosis (short black arrows).
Treatment: heparin and hydration even is small hemorrhage
He was given phenytoin, anticoagulated with intravenous heparin, and later discharged with oral warfarin for 6 months. He remained well. Procoagulant and autoimmune screens were negative, as was CT scan of the sinuses.
Erle C.H. Lim, Raymond C.S. Seet: Sudden-Onset Headache And Seizures In A Young Man. The Internet Journal of Neurology Volume 4 Number 2

34. 6.6 Cerebral Venous Sinus thrombosis 5 patterns of presentation
Isolated Intracranial hypertension
Progressive onset over days to weeks
Papilledema
CN6 palsy, tinnitus, visual obscurations
Venous infarction with focal signs
Mimics arterial stroke
Chronic and progressive like a tumor or abscess
Subacute encephalopathy
Diffuse HA with decr LOC
Focal signs
Seizures
+/-papilledema
Cavernous sinus thrombosis
Frontal headache
Chemosis, proptosis, opthalmoparesis
Thunderclap headache mimics SAH
80-90% have headache and it is the most common symptom

35. Secondary causes of CVST
Hypercoagable state
Factor V Leiden, Protein C & S,ATIII deficiency, prothrombin gene mutation,etc
Cancer
Sepsis, dehydration, infections
Behcet’s
SLE
Estrogen – pregnancy, puerperium

36. 6.4 Headache attributed to arteritis

37. Arteritis Headache Cerebral vasculitis Systemic vasculitis
Primary angiitis of the CNS
AIDS, zoster
Fungal, viral, parasitic, treponemal meningitis
Drug related - amphetamines, cocaine,
Lymphoma (angioendotheliosis)
Systemic vasculitis
Behcet’s - genital ulcers, arthritis, eye
PAN - fever, arthralgias, myalgias, mononeuropathies
Churg-Strauss - asthma, eosinophilia, neuropathy
Wegener’s – lower/upper resp, ANCA, neuropathy
SLE - fever, rash, arthritis, pleuritis, encephalopathy
Primary CNS vasculitis – headaches with multifocal neurological deficits for at least 6 months. Cerebral angio shows areas of segmental narrowing and biopsy of the leptomeninges or brain shows vascular inflammation. No infection, arteriosclerosis or Neoplasia found. Biopsy or angio abnormal in 75%. LP shows incr protein and cells (up to 400 cells) with normal glucose.
-cause unknown – viral/post viral mycoplasma autoimmune? – small leptomeningeal > parenchymal BV with granulomas sometimes
Headache attributed to secondary CNS angiitis – persistent headache and encephalitic signs: ie: stroke, seizure, chng LOC or cognition. In patient with systemic arteritis.

38. 6.4.1 Headache attributed to giant cell arteritis
ESR – the rate of sedimentation of RBC – the more they clump together, the faster the ESR Westegren >40 (not corrected for anemia); often is 90.
CRP – acute phase plasma protein – more specific for inflammation – not elevated by anemia, macrocytosis or level of globulins (esr is). May be a better way to follow disease.

39. GCA Rare: 15/100000 adults>50 (mean age 70)
Steroid responsive patchy granulomatous vasculitis of BV media with destruction of the internal elastic lamina.
Esp involves: superficial temporal, posterior ciliary, ophthalmic and vertebral arteries (parallels amount of elastic tissue in the media)
Usually not involve intradural BVs.
PMR
Posterior ciliary arteries involvement causes blindness (AION)
PMR is synovial inflammation that accompanies many cases of GCA – stiffness of the shoulders esp the sternoclavicular joint – biopsy shows nonspecific inflammation and not vasculitis. 50% patients with PMR later develop GCA – it is not sure what the management should be. Only about 25% will get serious symptoms. Biopsy may be positive but it is not certain if treatment is necessary if there is no symptoms. Low dose steroids don’t protect from GCA

40. Most common symptom: new headache in older pt.
Local symptoms: headache, visual loss, temporal artery ( beading, irregularities, tenderness, pulselessness), jaw claudication and scalp tenderness.
Systemic symptoms: fever, weight loss, PMR
Schmerling RH JAMA :
Diploplia if it develops is the result of ischemia of the EOMs (muscular branches of the anterior ciliary arteries)
Many cases are chronic and need treatment for years. Second eye involvement usually occurs within a month. Once visual loss occurs, response to steroids is poor – vision doesn’t come back.
Treat with high dose steroids (1 g solumedrol BID) if visual loss then oral steroids and taper over 6-12 months. Keep ESR and CRP low and if they increase, go up on the dose.

41. Table. Clinical Predictors of Temporal Arteritis
Likelihood Ratio
Variable (95% Confidence Interval)
__ Positive Negative Symptoms and Signs
Beaded temporal artery ( ) ( )
Prominent or enlarged temporal artery 4.3 ( ) ( )
Tender temporal artery ( ) ( )
Absent temporal artery pulse ( ) ( )
Any temporal artery finding ( ) ( )
Diploplia ( ) ( )
Jaw claudication ( ) ( )
Laboratory Data
Erythrocyte sedimentation rate
normal ( ) ( )
>50 mm/h ( ) ( )
>100 mm/h ( ) ( )
…None of the negative LRs completely rule out the disease. You have to look at the complete clinical and lab picture.
probability of an individual with the condition having a positive test LR+ = probability of an individual without the condition having a positive test
The probability that a person with GCA doesn’t have this is…
  probability of an individual with the condition having a negative test      LR- = probability of an individual without the condition having a negative test
Schmerling RH JAMA

42. 6.7 Headache attributed to other intracranial vascular disorder

43. *Migraine with aura in 1/3 and may precede MRI changes up to 15 y.
6.7.1 CADASIL
(Chrom. 19)
Ischemic episodes are classic lacunar syndromes and start around age 46.
Cognitive changes in 60% pts – frontal executive function age – slow or stepwise.
Mood disorders in 30%
*Migraine with aura in 1/3 and may precede MRI changes up to 15 y.
*Typical aura except higher freq. long auras and can be hemiplegic or basilar-like.

44. Figure 1a. Transverse FLAIR MR images
Transverse FLAIR MR images. (a) Images obtained in representative patients with biopsy-proved CADASIL (top row) and sSAE (bottom row) display the differentially involved temporopolar and superior frontal WM. Note the marked symmetry of lesions and the extension of lesions into the superficial WM in CADASIL (arrows). (b) Low-signal-intensity appearance of the subcortical nuclei (arrowheads) in a 49-year-old woman with CADASIL (top row) compared with that in a 56-year-old woman with sSAE (bottom row).
Auer, D. P. et al. Radiology 2001;218:
Copyright ©Radiological Society of North America, 2001

45. Figure. Fluid-attenuated inversion recovery MRI scans from a patient with a notch3 mutation shows typical involvement of the anterior temporal poles (arrow in A) and the external capsule (arrow in B), and characteristic images at the level of the lateral ventricles (C)
Markus, H. S. et al. Neurology 2002;59:

46. Figure. (A) FLAIR MR image showing extensive white matter signal hyperintensities in temporopolar regions and cystic changes
Figure. (A) FLAIR MR image showing extensive white matter signal hyperintensities in temporopolar regions and cystic changes. (B) Electron microscopy of skin biopsy shows electron dense granular deposits (arrows) in the basal lamina surrounding vascular smooth muscle cells. (granular osmiophilic material : GOM)
-destroys walls of small and medium sized arteries
TREAT WITH ASA +-DIAMOX?
Smith, B. W. et al. Neurology 2002;59:961

47. 6.7.3 Benign or Reversible Angiopathy (reversible vasoconstriction)
Call fleming syndrome
Diffuse severe HA (can be TCH), string and beads on MRA, CSF normal, 1-2months
**confused with SAH and with vasculitis**

49. Associated conditions
Pregnancy and puerperium
Early puerperium, late pregnancy, eclampsia, preeclampsia, and delayed
postpartum eclampsia
Exposure to drugs and blood products
Phenylpropanolamine, pseudoephedrine, ergotamine tartrate, methergine,
bromocriptine, lisuride, selective serotonin reuptake inhibitors,
sumatriptan, isometheptine, cocaine, ecstasy, amphetamine derivatives,
marijuana, lysergic acid diethylamide, tacrolimus (FK-506),
cyclophosphamide, erythropoetin, intravenous immune globulin, and
red blood cell transfusions
Miscellaneous
Hypercalcemia, porphyria, pheochromocytoma, bronchial carcinoid tumor,
unruptured saccular cerebral aneurysm, head trauma, spinal subdural
hematoma, postcarotid endarterectomy, and neurosurgical procedures
Idiopathic
No identifiable precipitating factor
Associated with headache disorders, such as migraine, primary
thunderclap headache, benign exertional headache, benign sexual
headache, and primary cough headache
Singhal AB, Bernstein RA. Neurocrit Care. 2005;3:91-7.

50. Clinical features
CTA or MRA documenting multifocal segmental cerebral artery vasoconstriction
No evidence for aneurysmal subarachnoid hemorrhage
Normal or near-normal cerebrospinal fluid analysis
Severe, acute headaches, with or without additional neurologic signs or
symptoms
Reversibility of angiographic abnormalities within 12 weeks after onset.
Leonard H. Calabrese, DO; David W. Dodick, MD; Todd J. Schwedt, MD; and Aneesh B. Singhal, MD
Ann Intern Med. 2007;146:34-44.

51. ESR
CSF
GCA N
Primary CNS angiitis
ALWAYS ABNORMAL
Benign CNS
angiopathy
FHM
+/-

52. 7. Headache attributed to non-vascular intracranial disorder

53. 7.1.1 Headache attributed to IIH
TVOs, diploplia

54. 7.1.1 Headache attributed to IIH
A. Diffuse constant headache worsened by coughing or straining
7.1.1 Headache attributed to IIH

55. MRI images

56. Pathophysiology Disorder of homeostasis where there may be:
Increased brain water content
Incr. cerebral blood volume
Incr CSF production
Venous outflow obstruction (increased resistance to CSF outflow)

57. Secondary causes Disruption of outflow Hormonal -hypoparathyroidism
-venous thrombosis, dural fistula
-radical neck dissection
-right heart failure
-COPD
-(obesity)
Hormonal
-hypoparathyroidism
-hyper/hypothyroidism
-Cushing's ?Addison's
-PCO
Toxins/meds
-vitamin A
-Nalidixic acid,tetracycline,nitrofurantoin,indocid,steroids/withdrawal,others
Medical conditions
CRF, SLE, Anemia/polycythemia
Infectious-meningitis,encephalitis,Lyme,HIV
Trauma

58. Tinnitus, and distortion of hearing. Neurologic examination normal.
CASE
40-year-old woman:
posterior neck pain and orthostatic headaches (severe at times, dull or throbbing), worse with cough.
Tinnitus, and distortion of hearing.
Neurologic examination normal.
Head MRI showed….

59. Diffuse pachymeningeal enhancement (T1 with gado)
Predisposing factors: congenital weakness of the dural sac, meningeal diverticula (Marfan’s), connective tissue matrix disorders, disc disease.
Diffuse linear enhancement
Other symptoms: neck or interscapular pain, CDH, afternoon headache, no headache.
nausea, diploplia, dizzy, tinnitus, Hypacusia, photophobia, numbness…coma, parkinsonism, dementia.
Diffuse pachymeningeal enhancement (T1 with gado)
Descent of the brain – tonsils, loss of cisterns, post fossa crowding
Pituitary Enlargement
Flattening of the chiasm
Subdural collections
Engorged venous sinuses and small ventricles.
Classic
Brain MRI
features

60. CSF opening pressure was 3cm H2O
A lumbar epidural blood patch offered no relief.
Water-soluble myelogram/CT-myelogram showed frank extravasation of contrast to the paraspinal soft tissues at the L C7 root sleeve.
Subsequently, the leak was surgically repaired via a left sixth through seventh cervical hemilaminectomy
Myelo – meningeal diverticulum
Blood patch, epidural and rarely intradural infusions, surgical repair
B. Mokri Mayo Clin Proc. 1999;74:

61. Csf buoys 97% of the slightly denser brain.
Most of the blood volume is venous so this is where the compensation comes from

62. 7.2 Headache attributed to low cerebrospinal fluid pressure
(CSF below 5-9 cm H2O)

63. 7.2.1 Post-dural (post-lumbar) puncture headache
Dural rent may be caused by a quincke cut needle – tin-lid – hard to heal
Try to go parallel rather than tranverse to long. Axis of dural cylinder. Use max 20G – use blunt needle
This type of headache occurs within 2 days of LP and gone in 1 week
Usually headache starts/resolves within seconds of change in posture
Bedrest useless – in controlled study

64. Case 47 year old executive, father of 3.
According to wife had a longstanding history of migraine.
Headache continuous for 9 weeks and taking daily analgesics.
Presented to the ER on the weekend as headache no longer responding.
Had been working until the Friday before.

65. In ER, physician described that the patient was behaving in a bizarre fashion, seemed agitated and inconsolable..?drug seeking.
Given Maxeran then Dilaudid then Toradol with no improvement.
The next morning a CT scan of the brain was ordered…

68. Exam
Nice couple completely convinced that this was a terrible migraine that would eventually improve.
Patient unable to lie down. Sitting in the chair grabbing his head and intermittently crying out in pain.
Unable to answer direct questions on orientation and distractible.
Pupils equal and reactive. Left babinski.

69. How would you manage this patient?
Patient was given 10 mg decadron, intubated and sent to the MNI. There he was extubated. Deteriorated a few hours later with transtentorial herniation and was taken to the OR urgently in the night. Patient died. Pathology was of grade 4 GBM

70. What are the early signs of transtentorial herniation?
Decreased LOC
Hypertension/bradycardia?
Ipsilateral parasympathetic dysfunction (dilated pupil)…

71. Section 8 – Headache attributed to a substance or its withdrawal
8.1.1 – NO donor: NTG, nitrates, nitrites
8.1.2 – Phosphodiesterase inhibitor: Viagra, etc – migraineurs most at risk; lower doses better.
8.1.1 – soon get bilateral pressure headache gone in an hour – more in migraineurs. Activation of cGMP. There is also a delayed HA
8.1.2 – more migraine like; esp in migraineurs – increase levels of cGMP – 15% pts hours

72. 10.1 Headache attributed to hypoxia and/or hypercapnia
Sleep apnea headache – bilateral pressing, >15 day per month, resolves in 30 min after awake,

73. 10.3 Headache attributed to arterial hypertension
Paroxysmal abrupt severe elevations in BP

74. 11. Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures

75. Part 3: Cranial neuralgias, central and primary facial pain and other headaches

76. 13. Cranial neuralgias and central causes of facial pain

77. 13. Cranial neuralgias and central causes of facial pain

78. WE don’t need to use this code most of the time!
14.2 Headache unspecified