UCNS HA Board Prep - ICHD-3 Summary PDF

ICHD-3 Summary 1. Migraine Migraine without aura >5 HA x4-72 hours (2-72 hours in children) 2 of: Unilateral, pulsating, mod/sev, worse w/ activity 1 of: Nausea or vomiting, photophobia and phonophobia Prodrome Up to 2 days before migraine...

ICHD-3 Summary 1. Migraine Migraine without aura >5 HA x4-72 hours (2-72 hours in children) 2 of: Unilateral, pulsating, mod/sev, worse w/ activity 1 of: Nausea or vomiting, photophobia and phonophobia Prodrome Up to 2 days before migraine Fatigue, difficulty concentrating, neck stiffness, sensitivity to light and/or sound, nausea, blurred vision, yawning, pallor Pathophys: May have DA mechanism; DA blockers (domperidone/Motilium, metoclopramide/Reglan); long-acting triptans (naratriptan/frovatriptan) used for HA abortion during prodrome Postdrome Up to 48 hours after migraine Tired/weary, difficulty concentrating, neck stiffness Migraine with aura >2 attacks of aura: visual, sensory, speech/language, motor, brainstem, retinal >3 of 6: Spreads over >5 mins, 2+ aura symptoms, each symptom 5-60 mins*, unilateral, positive (scintillations, pins/needles), w/ HA or HA within 60 mins Typically visual → sensory → aphasia * Aura can be >60 mins but will usually have at least 2 other characteristics Visual aura Most common, up to 90% MA Fortification spectrum: Zigzag near fixation → spreads to be laterally convex with angulated scintillating edge → absolute/relative scotoma Scotoma alone can occur, but will enlarge gradually Children/adolescents: Can be bilateral Sensory aura Paresthesias moving slowly from origin along one side of body, face, or tongue. May or may not be followed by numbness, or can be numbness alone Aphasia Always unilateral; dysarthria may or may not be Typical aura Binocular visual, sensory, and/or speech/language AND no motor, brainstem, retinal symptoms Brainstem aura 2 of: Dysarthria (not aphasia), vertigo, tinnitus, hypacusis, diplopia, ataxia (not a/w “Basilar-type” sensory deficit), GCS 5 mins, symptoms lasting 5-60 mins, w/ HA or HA within 60 mins Chronic migraine >15 HA days, at least 8+ migraine days, 3+ months MOH common contributor: ~50% revert to episodic after withdrawal Status migrainosus >72 hours, debilitating pain or associated symptoms May remit up to 12 hours d/t medication or sleep Persistent aura w/o Aura symptoms >1 week, may last months or years infarction Can be bilateral Migrainous infarction Mostly younger women, in posterior circulation MA triggered seizure Seizure during or within 1 hour of MA; no evidence for a/w MO Episodic syndromes Recurrent GI >5 attacks abdominal pain/discomfort, and/or nausea and/or vomiting disturbance May be infrequent, periodic, chronic Cyclic vomiting >5 attacks, stereotypical/predictable periodicity, may be a/w pallor/lethargy N/V >4x/hr, each attack >1 hr-10 days, attacks >1 week apart Abdominal migraine >5 attacks, 2-72 hours 2 of: Midline, periumbilical or poorly localized; dull or “sore”; mod/sev 2 of: anorexia, nausea, vomiting, pallor Benign paroxysmal >5 attacks of sudden onset vertigo (episodic unsteadiness noted by parent), max at vertigo onset → spontaneous resolution in minutes to hours, w/o LOC 1 of: nystagmus, ataxia, vomiting, pallor, fearfulness Benign paroxysmal Recurrent attacks of head tilt to either side +/- slight rotation → spontaneous torticollis resolution in hours or days. Head tilt can be overcome to the neutral position. 1 of: pallor, irritability, malaise, vomiting, ataxia Recurrence tends to be monthly, ataxia most likely in older children D/Dx: GERD, idiopathic torsional dystonia, complex partial sz; evaluate posterior fossa/craniocervical junction 2 2. Tension-type headache Prevalence 30-78% Pathophysiology Unknown; suspected peripheral pain mechanisms for infrequent/frequent episodic TTH, ­role central pain mechanisms in chronic TTH Pericranial tenderness All TTH subdivided into +/- pericranial tenderness ­pericranial tenderness likely related to pathophys, typically present interictally and worse during HA Score 0-3 each: frontal, temporal, masseter, pterygoid, SCM, splenius, trapezius Episodic TTH >10 HA x30 mins-7 days Infrequent: 15 days/month), hours-days or unremitting 2 of: bilateral, pressing/tightening, mild-mod, not worse with activity May have mild nausea (no vomiting), photophobia, or phonophobia If specific day remembered and unremitted from 2 attacks; Remission: >3 months Chronic TAC Attacks: >1 year without remission, or remission 24 hours HC, unremitting Continuous >1 year, no remission periods of >24 hours Cranial autonomic At least 1 of: symptoms Conjunctival injection/lacrimation Nasal congestion/rhinorrhea Eyelid edema Forehead and facial sweating Miosis/ptosis Pathophysiology Nociceptive trigeminal activation ® cranial parasympathetic activation Evaluation ­prevalence of TACs in pituitary tumors TAC Attacks Duration Freq* Location Intensity CAS RL/agi IM Eval resp for HC -- >3 mo -- -- +exacerbations 1/5 Yes OR Yes (F>M) of mod/sev ­mvmt CH 5 15-180m 1 QOD Orbital/SO Sev-very sev 1/5 Yes No MS (M>F) Avg 60m to 8/day Temporal (VPM) PH 20 2-30m >5/day Orbital/SO Sev 1/5 Yes Yes (F>M) Avg 15m Avg 10 Temporal SUNA 20 1-600s >1/day Orbital/SO Mod-sev NA: 1/5 No No Post SUNCT Avg 1m Avg >30 Temporal Single stabs, CT: Conj (LMT) fossa (M>F) Trigeminal group of stabs, inj + lac lesion saw-tooth *Must have required frequency >50% of time during attack period Indomethacin- HC > PH (responds within days), primary cough, stabbing, sex headaches responsive headaches Indomethacin trial: >150mg/day, increased to up to 225mg/day for at least a week (generally 75mg TID) for confirmation – afterwards can use smaller doses. IV dose 100-200mg. SUNA/SUNCT vs. TN Location Duration Frequency Refractory CAS Gender SUNA/ V1 > V2/V3 1-600s >1/day (may No Yes M>F SUNCT be hundreds) TN V2/V3 > V1 M can ­ over hundreds time 4 4. Other primary headaches Duration Symptomatic Cough 1 sec-2 hrs, peaks quickly 40%; many ACM I. SIH, SDH, MCF/posterior fossa tumors, vascular (carotid/vertebrobasilar disease, RCVS). Subtentorial tumors >50% space-occupying lesions in kids. Exercise Onset during/after, 3 mos Acute traumatic injury, ­ICP, SIH Primary cough 2 HA x1s-2h, sudden onset (peaks quickly), only in a/w coughing, straining, headache Valsalva (brief efforts vs. exercise HA) Incidence: Rare; 50% space-occupying lesions Features: Bilateral, posterior; >40y Tx: Indomethacin 50-200mg/day Primary exercise 2 HAs 5 min, severe, abrupt onset (max intensity in 3 months, no cranial autonomic symptoms Symptomatic: Sleep apnea (but does not exclude diagnosis), nocturnal HTN, hypoglycemia, MOH, intracranial disorders Features: Usually >50y, mild-moderate pain, 2/3 bilateral; most persistent daily/near-daily but can be episodic (3 months Moderate/severe TBI 1 of: LOC >30 mins, GCS 24 hrs, ALOC >24 hrs, imaging evidence (skull fracture, ICH, brain contusion) Mild TBI None of mod/severe TBI features, and 1 of: transient confusion/disorientation/impaired consciousness, or Loss of memory for events immediately before, or >2 of: N, V, visual disturbances, dizziness/vertigo, gait/postural imbalance, impaired memory/concentration Whiplash Sudden/inadequately restrained acceleration/deceleration w/ flexion/extension of neck, either high or low impact Onset within 7 days of whiplash (most commonly MCA) Craniotomy Onset: Within 7 days of craniotomy, regaining of consciousness, or regaining the ability to sense/report pain (discontinuing meds) Must exclude secondary causes (cervicogenic d/t positioning, HA from CSF leak, infections, hydrocephalus, ICH). If crani d/t head injury, code as mod/severe TBI Typically skull base surgery, max at site (but can be diffuse or look like TTH/migraine), begins in first few days after crani, resolves during acute postop 25% develop persistent Post-concussion Dizziness, fatigue, ¯concentration, psychomotor slowing, mild memory problems, syndrome personality changes, irritability, insomnia, anxiety 7 6. Vascular intracranial/cervical disorders Acute Resolved within 3 months, or not yet resolved but 3 months has not yet passed 3 months counted from stabilization, not onset of disorder Persistent >3 months Ischemic ICHD-3 features Etiology/Differential Presentation Acute ischemic None Exclude RCVS, arterial HA: Mod, ipsi or bilateral; basilar > stroke dissection Carotid >> Lacunar Embolic: isolated/ thunderclap HA Cerebellar: Dizziness/vertigo most common; N/V >50%, >1/3 HA; ataxia, dysarthia, nystagmus, hemiparesis TIA positive phenomena onset focal deficit dissection Basilar > Carotid but HA rarely a MA: Progressive deficit, prominent sx positive > negative Intracranial hemorrhage Location Typical etiology Lobar Cerebral amyloid angiopathy (beta-amyloid deposition ® ­risk hemorrhages) Basal ganglia (putaminal) HTN Cerebellar HTN Brainstem Cavernous malformation, AVMs, HTN IVH Ruptured AVM, AVF; IV tumors, IV aneurysms in lenticulostriate/choroidal arteries (Moya-Moya); occasional saccular (Acomm, PICA, basilar tip) SAH Ruptured saccular aneurysm Convexal SAH (cSAH) CAA: Older + sensorimotor dysfunction, aura-like spells, no significant HA RCVS: Younger, recurrent thunderclap HAs SDH Spontaneous cortical artery rupture, aneurysm rupture, AVM, DAVF, tumors/mets, coagulopathies, Moyamoya, CVST, intracranial hypotension ICHD-3 features Presentation Etiology/Differential ICH, non- 1 of: Sudden Focal deficits usually more Cerebellar ICH: HA can be traumatic thunderclap, max on notable than HA, but pain prominent early (a/w ­early day of onset, more severe vs. AIS mortality in ICH but not in AIS) localized to ICH area HA d/t subarachnoid blood + mass effect, less likely d/t ­ICP SAH, non- Sudden thunderclap ~50% report sudden, severe HA Ruptured saccular aneurysm traumatic onset within 4 weeks prior Perimesencehpalic/pretruncal: Venous origin, cisterns around CT: M 85% anterior: MCA ICHD-3 1 of: Sudden bifurcation, Most common thunderclap, painful Acomm/ACA, cause of SAH CN3 palsy Pcomm/ICA) Posterior: top of Risk factors basilar, basilar/SCA, Classic: CN3 palsy, Genetic: PCKD, EDS, basilar/AICA, retroorbital pain, dilated AVM, Moya-Moya vert/PICA pupil (PComm or carotid Acquired: HTN, terminus aneurysm) smoking, cocaine, Exclude ICH, RCVS meth Dural AVF ICHD-3 1 of: 50s-60s Typically acquired HA can have features of ophthalmoplegia; intracranial hypotension pulsatile tinnitus (can Exclude ICH, CVST (¯venous outflow, sinus be painful), thrombosis) progressive, worse in AM, coughing, or Carotidocavernous fistula: bending over Painful ophthalmoplegia Cavernous Usually unrelated 30-50% in Sporadic; Seizures, ICH, focal deficits malformation May have features of 20s-40s congenital or Isolated HA rare, but (angioma) CH, SUNCT, or (some teens) acquired common w/ sz/ICH (code to migraine Familial: AD, CCM1- those) 3 (KRIT1=CCM1) CA/KRIT1: Only 4% w/ HA Usually multiple as presenting symptom progressive CA Exclude ICH 9 Vasculopathies, part I ICHD-3 features Presentation Etiology/Differential Intracranial 2 of: temporal onset, Asians: Intracranial arterial > arterial resolves in 1 mo, cervical artery dissection dissection unilateral/ipsilateral, characteristic HA HA features: 1 of: Sudden, thunderclap or severe Cervical Unilateral and ipsilateral Generally sudden, Isolated or warning symptom carotid or to the affected artery thunderclap onset prior to ischemic stroke vertebral artery disorder Cervical artery 1 of: severe, continuous HA +/- neck pain; often 1st Can be a/w intracranial artery dissection for days or longer; sx (avg 4 days duration) dissection ® SAH precedes signs of retinal *Signs of CA dissection: Dx: Cervical MRI + fat supp, or cerebral ischemia* Painful Horner's, painful duplex scan, MRI/CT angio, DSA tinnitus, painful CN12 palsy Tx: Hep to Warfarin x3-6 mo Cerebral None HA most frequent symptom, Strongly consider in setting of venous often 1st symptom recent persisting headache, thrombosis No specific features but underlying prothrombotic (CVT/CVST) often severe, diffuse, >90% focal deficits/sz, ­ICP condition (Factor V Leiden (aP-C progressive; can also be signs, subacute resistance), protein C/S def, ATIII unilateral (mimics encephalopathy, or def, hyperhomocysteinemia) primary) cavernous sinus syndrome Risk factors: ¯intravasc volume (dehydration, malnutrition, sepsis), OCPs/HRT, pregnancy RCVS 1 of: HA w/wo focal Most frequent cause of 50% secondary, post-partum or deficits/sz ® angio w/ recurrent thunderclap HAs drugs (illicit drugs, a- "string of beads" or over days to weeks; sympathomimetics – clonidine, characteristic HA typically HA only but can be phenylephrine, midodrine, a/w seizures, fluctuating methyldopa, serotoninergic) HA Features: 1 of: focal deficits Exclude aneurysmal SAH thunderclap, triggers, MRI abnormal 30-80%: present or recurrent cSAH, ICH, SDH, stroke, Triggers: Sex, exertion, Valsalva, during 1 mo MR/CT angio & DSA can be Consider PACNS if after onset normal in 1st week –give encephalopathic M w/ "probably RCVS" dx progressive HA Probably RCVS All: >2 HAs in 1 mo May need to repeat angio 2-3 (thunderclap, severe, weeks after HA onset OR DSA >5min), >1 HA triggered (incl bending), no new HA >1 mo after onset 10 Vasculopathies, part II ICHD-3 features Presentation Etiology/Differential Giant cell HA w/ scalp tenderness HA + amaurosis fugax (esp. Most closely a/w HA of all arteritis and/or jaw claudication repeated attacks); risk of arteritides, collagen disorders blindness d/t AION ­risk ischemic events, dementia PACNS None HA dominant symptom (50- Less likely w/o HA ­CSF nuc 80%) but lacks specific Exclude RCVS (esp. w/ recurrent features; HA d/t stroke, thunderclaps), CNS infection, inflammation, ­ICP, SAH CNS neoplasia. SACNS difficult to ID: inflammatory, infectious, malignant, toxic CADASIL 1 of: MA earliest, or 1/3 have typical MA but AD/NOTCH3, some sporadic MA improves/stops auras unusually prolonged Dx: Skin biopsy NOTCH3 ab when other sx appear & Recurrent small deep EM for extracellular granular worsen infarcts, mood disturbances, osmiophilic material in arteries subcortical dementia MA onset ~30y (15y before MRI c/w cog dysfunction stroke, 20-30y before death) MELAS 1 of: recurrent MO/MA, HAs (migraine-like or 80% m.3243A>G in MT-TL1 acute HA a/w focal stroke); CNS involvement (sz, deficits or sz PN, episodic vomiting, encephalopathy, deafness, dementia, hemiparesis, hemianopia, cortical blind) Sturge-Weber Migraine-like, bilateral/ 90% seizures, ~50% post-sz Sporadic, somatic mosaic CNAQ syndrome localized, contralateral HA (code to sz), glaucoma Encephalotrigeminal or aura (motor, prolonged) leptomeningeal angiomatosis Moya-Moya Causation via both: Early childhood/adolescence SCA, DS, radiation therapy angiopathy temporal onset, or Ischemic/hemorrhagic HA worsens w/ process stroke ® acute HA; typically or HA improves after similar to MO/MA (some revascularization hemiplegic), TTH; rarely CH Cerebral New attacks of Late onset MA-like aura Small-vessel progressive amyloid amyloid migraine-like aura Sporadic >> familial deposition in leptomeningeal angiopathy w/wo mild HA Major cause of lobar ICH, and cortical vessels (CAA) Causation via 1 of: cSAH, cognitive impairment, temporal onset, aura transient focal episodes in Pathophys of focal episodes: worsens w/ process, or elderly (positive MA-like, superficial cortical siderosis, onset >50y negative TIA-like) cSAH? RVCLSM Recurrent migraine-like 50% migraine-like attacks; TREX1, C-terminal frame-shift Retinal attacks w/wo aura focal deficits, sz, cognitive mutations ® early death from vasculopathy impairment, psychiatric D/O, progressive cerebral WM lesions w/ cerebral vascular retinopathy, Younger patients may have leukoenceph- systemic (impaired function normal MRI brain and p/w mild alopathy and liver/kidney, anemia, GI Raynaud's, MO>MA, psych systemic bleeding, HTN) disturbances Other chronic Recurrent migraine-like HIHRATL (AD, COL4A1): infantile hemiparesis, retinal arterial vasculopathy attacks w/wo aura tortuosity, leukoencephalopathy 11 Post-procedural ICHD-3 criteria Presentation Etiology/Differential Post-CEA 2 of: onset within 1 week, 3 subforms: Isolated or warning resolves within 1 mo, Mild diffuse pain: first few symptom prior to typically characteristic HA features days after CEA (benign, hemorrhagic stroke HA Features: unilateral HA, most frequent) Hyperperfusion syndrome: ipsilateral to CEA and 1 of: Unilateral cluster-like: ~2 HA on 3rd day precedes mild diffuse pain, severe weeks ­BP, onset of sz/focal pulsating pain, cluster-like Unilateral severe deficits on 7th day) – can 1-2/d x2-3h pulsating: 3 days after herald hemorrhage CEA (hyperperfusion syndrome) Exclude arterial dissection Post endarterial 3 of: onset within 1 week, 1st subform usually after Exclude arterial rupture procedure resolves within 1 month, balloon inflation or and dissection ipsilateral or bilateral, embolization of AVM or characteristic features aneurysm Features: 1 of: 3rd subform: Migraine Severe, abruptly within triggered by procedure seconds, lasting 24 hrs Pt with MO/MA + features of typical HA Angiography 2 of: Onset during angio or 1st/2nd subforms more Contrast angio within 24 hrs, resolves common in those with contraindicated in within 72 hrs, characteristic primary HA but are different hemiplegic migraine (may features from usual HA trigger life-threatening attack w/ prolonged Features: 1 of: 3rd subform: Migraine hemiplegia/coma) During contrast, 24 hrs both) Pt with MO/MA + features of typical HA Post-carotid or All: Onset within 1 week, ~1/3 have HA, usually within Exclude arterial dissection vertebral resolves within 1 mo, and 10 min of procedure and Can be part of angioplasty or ipsilateral resolving in 10 min; mild, hyperperfusion syndrome stenting ipsi, frontotemporal, pressure-like Cranial venous All: Onset within 1 week, Exclude within-stent stenting resolves within 3 mos, and venous thrombosis ipsilateral 12 7. Non-vascular intracranial disorders Increased ICP Intracranial HTN is a CSF >250mm CSF (280mm CSF in obese children), normal CSF composition temporary dx Causation by 2 of: temporal, relieved by ¯ICP, papilledema IIH 1 of: pulsatile tinnitus, papilledema; relief after CSF removal NOT diagnostic HA often migrainous or TTH-like but no specific features Imaging features: Empty sella turcica, optic nerve protrusion, distension of the optic nerve sheath, optic nerve tortuosity, posterior globe flattening, and transverse sinus stenosis 60% pulsatile tinnitus, unilateral or bilateral; most likely in obese F of childbearing age Metabolic, toxic, Potential causes: acute hepatic failure, renal failure, hypercarbia, acute HTN hormonal causes crisis, Reye's syndrome, CVST, right heart failure Hormonal substances: Thyroid hormone replacement (in children), all-trans retinoic acid/retinoids, tetracyclines, chlordecone, vitamin A toxicity, steroid withdrawal Other medications coded separately Chromosomal causes Turner syndrome, Down syndrome Hydrocephalus NPH: HA rare, occasionally mild/dull Decreased ICP Intracranial CSF 2 attacks ophthalmoplegic neuropathy Burning mouth Burning pain in superficial oral mucosa, >2 hrs/day >3mos syndrome Typically bilateral, most commonly at tip of tongue Persistent idiopathic facial pain Central neuropathic pain Central post-stroke pain 24