When To Refer….. JAN LEWIS BRANDES, M. D. ASSISTANT CLINICAL PROFESSOR, VANDERBILT UNIVERSITY NEUROSCIENCE/CARDIOLOGY UPDATE ST DOMINIC’S NEW ORLEANS, LOUISIANA FEB 1 2014 When to refer….  REFER - when diagnosis is uncertain  when appropriate diagnostic studies don’t help  when pattern does not meet diagnostic criteria  when patient is not improving  when co-morbidities interfere  when reasonable medication trials are ineffective The Tasks of Differential Diagnosis in Headache What’s wrong? Is it serious? Is it treatable? User-Friendly IHS Classification  Two Major Categories  Primary headaches (benign headache disorders)   Migraine (with or without aura)  Tension type headache (episodic or chronic)  Cluster headache  Other benign headache  Post traumatic headache  Drug rebound headache Secondary headache (headaches that are symptoms of organic disease) Diagnostic Algorithm for Headache The Headache History      Age, circumstance, suddenness of onset Intensity/character of pain Duration/frequency Location(s) and radiation of pain Preceding and associated symptoms         Course Provoking/aggravating factors Hormonal influences Ameliorating factors History (medical and medication) Family history Social/emotional Impairment/impact Physical, Laboratory, and Other Diagnostic Evaluations     Physical exam Lab testing* Neurodiagnostic tests*  Key Elements  General and neurologic exam  Sed rate, TSH, hematocrit, glucose  CT, MRI/MRA, EEG, LP, arteriogram  Dental, ENT, allergy evaluation Other* *Appropriate for variant or atypical forms Diagnostic Alarms in Evaluation of Headache  Headache begins after age 50 Temporal arteritis, mass lesion  Sudden-onset headache SAH, pituitary apoplexy, bleed into mass or AVM, mass lesion (post. fossa)  Accelerating or changing pattern of headache Mass lesion, subdural hematoma, medication overuse Diagnostic Alarms in Evaluation of Headache  New-onset headache in patient with cancer or HIV Meningitis (chronic or carcinomatous), brain abscess, metastasis  Headache with systemic illness (fever, stiff neck, rash) Meningitis, encephalitis, Lyme, systemic infection, collagen vascular disease  Focal neuro symptoms or signs of disease (other than aura) Mass lesion, AVM, stroke, collagen vascular (inc. antiphospholipid Ab)  Papilledema Mass lesion, pseudotumor, meningitis Sixty-seven Year Old Man with Cough Headache Benign or Symptomatic? Ten week history of headache HA occurs within seconds to minutes of coughing Bilateral dull pain, progresses to sharp within minutes Normal exam, recent bronchitis Rx with Abx, now clear CXR Twenty-seven Year Old Man with Cough Headache Benign or Symptomatic? Ten week history of headache HA occurs within seconds to minutes of coughing or after weight lifting or running Bilateral dull pain, progresses to sharp within minutes Normal exam, recent bronchitis Rx with Abx, now clear CXR Cough Headache: Benign or Symptomatic ?  Benign  Mean onset age 55  4x more common in men  Immediate onset within seconds of coughing, or ^’ed pressure  Lasts seconds to 30 minutes  Neuro exam - normal  Symptomatic  Mean onset age is lower, about 39  Multiple precipitants, besides coughing  Lasts seconds to days  Posterior fossa abnormalities often causal  Neuro exam – posterior fossa signs Sixty-seven Year Old Man with Cough Headache WHEN to Refer ? First, treated with indomethacin  No significant improvement = Rx indomethacin 50mg TID  Reduced the pain severity, but otherwise no change in attacks. Next?  Prophylaxis with nortriptyline  Differential Diagnosis for Benign Cough Headache           Arnold Chiari Malformation Basilar Impression from Paget’s Disease Posterior fossa meningioma Midbrain cyst Acoustic neuroma Neoplasm - malignant Subdural hematoma Cerebral aneurysm, carotid stenosis, VB disease Other – Sarcoid Exertional HA, effort migraine, coital headache Sixty-seven Year Old Man with Cough Headache - (cont)  MR imaging of brain with and without contrast was normal  Nortriptyline was added to Rx when chest CT read as normal  Decision to proceed with bronchoscopy. Sixty-seven Year Old Man with Cough Headache  Bronchoscopy revealed non-small cell lung carcinoma  Chemotherapy initiated, and cough eventually suppressed with resolution of headache  Survived ten months after differential diagnosis of cough headache pursued Twenty seven year old man with cough/exertional headache  Increased hydration before exercise  Indomethacin 50mg thirty minutes before exercise  MRI of brain was normal  Indomethacin withdrawn after 6 months with no return of symptoms “Real” Reasons for Testing To aid in diagnosis (rule out comorbid disease) To establish diagnosis To eliminate fear Often Cited Reasons for Testing Clinical shortcut Patient expectations Financial incentives Medicolegal issues Methods of Investigation  Electroencephalography (EEG)  Computerized tomography (CT)  Magnetic resonance imaging (MRI)  Magnetic resonance angiography (MRA)  Cerebral angiography  Lumbar puncture  Thermography  Electromyography (EMG) Indications for Neuroimaging  The first or worst headache of the patient’s life  A change in frequency, severity or clinical features  An abnormal neurologic examination  A progressive or new daily persistent headache  An atypical headache that does not meet the criteria for an established primary headache  Head pain which is always on the same side  Headache unresponsive to routine therapy Imaging in Headache In adult patients with recurrent headache, defined as migraine, including aura, no recent change in pattern no history of seizures no focal neuro signs/symp. routine imaging studies are not warranted. American Academy of Neurology guidelines, 1996 Indications for Electroencephalography  Alteration or loss of consciousness  Suspected encephalopathy  Persistent residual neurologic deficits  Baseline prior to institution of medicines or procedures which could induce seizure  Transient neurologic symptoms without ensuing headache Indications for Lumbar Puncture  The first or worst headache of the patient’s life  A severe, rapid onset, recurrent headache  A progressive headache  An atypical chronic intractable headache  Headache with alteration in mental status, fever, meningeal signs Investigation of Specific Headache Types  Migrainous infarction  Migraine with prolonged or atypical aura  Basilar type migraine  Migraine aura without headache WHEN to refer: Repeated attacks  62 year old woman  No hx of migraine – childhood history of severe motion sickness  Feb 2013 : sudden onset of right arm heaviness, slowed speech and arm and face numbness and tingling – SBP 190  CT/MRI negative  Repeat episodes Late Feb 2013, August 2013, December 2013  All with repeat CT/repeat MRIs/Cerebral arteriogram  Terrified ---- CLUE ---- sequence ---- timing of episodes, all lasting 30 minutes with right arm spread and resolution, +/- headache  Migrainous infarction with recurrent attacks  Tx stroke and migraine prevention Migrainous Infarction vs. “Complicated migraine Timing is Everything….. “Clues” - Focal weakness, numbness, visual or speech difficulty.  Magnetic resonance angiography or arteriography  Cardiac echocardiography (esp. TEE)  Carotid duplex scanning, transcranial doppler  EKG or Holter monitoring  Anti-phospholipid Ab, protein S, protein C, other hypercoagulable labs, factor V/Leiden  Interpret in setting of stroke risk factors Migraine Aura Without Headache - Migraine equivalent - Late life migraine accompaniment - “Mimics” cerebral thrombosis, embolism, dissection, epilepsy, coagulopathies, blood dyscrasias - “Clues” - Gradual appearance of symptoms - over minutes not seconds - Positive visual symptoms - Serial progression - Duration of 15-25 minutes Headache of Sudden Onset Primary headache disorders  Crash migraine  Cluster  Benign exertional headache  Benign orgasmic cephalgia Headache of Sudden Onset Secondary headache disorders  Associated with vascular disorders - Unruptured saccular aneurysm - Subarachnoid hemorrhage - Internal carotid artery dissection - Cerebral venous thrombosis - Acute hypertension Headache of Sudden Onset Secondary headache disorders  Intermittent hydrocephalus  Benign intracranial hypertension  Pituitary apoplexy  Cephalic infection  Acute mountain sickness  Ophthalmic - optic neuritis, glaucoma In Headache in Clinical Practice, 2009 Epidural Hematoma Epidural Hematoma Diagnostic Problem Solving  Where is the historical clue?  Where are the exam clues?  What study/studies are critical to management? Key Clinical Features of Migraine: Prodrome and Aura  Prodrome  Premonitory phenomena  Occurs hours to days before headache onset  Affects 60% of migraineurs  Symptoms vary widely, but may be consistent per individual  Nonfocal, constitutional symptoms Key Clinical Features of Migraine: Prodrome and Aura  Aura  Most often visual(scintillations, scotomas)  Often has hemianoptic distribution  Usually develop and fades within 30 minutes  May reflect wide range of neural deficits  Affects 20% of migraineurs Key Clinical Features of Migraine: Headache and Postdrome  Headache  Postdrome/Postict us Usually unilateral with throbbing pain Aggravated by physical activity, bright light, loud sounds Onset is gradual, peaks, and then subsides over a course of 4 to 72 hours Nausea occurs in up to 90%, vomiting in ~33% Pain wanes, leaving tiredness, irritability, listlessness 48 year woman  8-12 days of headache per month  Sleep  Using > 18 triptans per month  Increase water  Trials of botulinum toxin, topiramate, nortriptyine, propranolol, amitriptyline, verapamil, magnesium, gabapentin, fluoxetine, paroxetine, lamotragine, metoprolol, valproate, among others  Decrease caffeine  Increase exercise  Longer, higher  Bridging drug for moderate headache  No treatment for mild headache  Injection for severe without narcotics  Longer, higher prevention dosing  Behavioral factors GENERAL PRINCIPLES OF PREVENTIVE TREATMENT Start low and increase dose slowly  Use long-acting formulation if compliance an issue Adequate trial (2 to 3 months) at an appropriate dosage Avoid interfering, overused, and contraindicated medications Evaluate therapy Use headache calendar (diary)  Attempt to taper and discontinue treatment when headaches well controlled  When to refer …..when not enough time or patient needs more support than you have to give  Set expectations  Keep a DIARY !!  At least 2-3 months, of adequate dose  Encourage behavioral treatments  To reduce, perhaps not completely stop attacks  Avoid medication overuse  Address hormonal issues  Help with comorbid diagnoses  Support  To make acute medications more effective  To increase function  Keep out of ER, urgent care IHS Criteria for Tension Type Headache  Headache lasting from 30 min - 7 days  Headache pain accompanied by two of the following symptoms:    Pressing/tightening (nonpulsating) quality  Bilateral location  Not aggravated by routine physical activity  Mild or moderate intensity (may inhibit, but not prohibit activities) Headache pain accompanied by both of the following symptoms:  No nausea or vomiting  Photophobia and phonophobia absent or only one present Fewer than 15 day per month with headache Medication Overuse/rebound Headache        Diffuse, bilateral headache every day or nearly every day Aggravated by mild physical or mental exertion Waking with early morning headache Restlessness, nausea, forgetfulness, asthenia, depression Medication withdrawal symptoms when ergotamine, a barbiturate, or codeine is involved Tolerance to acute/abortive migraine medication No response to preventive migraine medication IHS Criteria for Migraine with Aura At least 2 attacks, fulfilling… At least 3 of the following 4 characteristics:  At least one fully reversible aura symptom indicating focal cerebral cortical and/or brainstem dysfunction  At least one aura symptom developing gradually over 4 minutes, or at least two symptoms occurring in succession  No aura symptom lasting more than 60 minutes; if more than one aura symptom is present, expected duration is proportionally increased  Migraine headache follows aura within 60 minutes; it may also begin before or simultaneously with aura Challenges Associated with Migraine Diagnosis  Clinical diagnosis based on symptoms and “story” as reported by the patient  No blood test or X-ray can confirm  Symptoms may vary from one attack to another  Migraine may be comorbid with other disorders  Incomplete/lack of information on family history  Patients with migraine may also have tension-type or druginduced headache  Patient evaluation can be time-consuming and laborintensive Successful Diagnostic Aids  Unstructured account  Onset  Location/duration  Frequency, timing  Quality/severity  Associated features  Precipitating/aggravating/ameliorating factors  Social/Family history  Impact of headache Barriers to Receiving Effective Treatment 39 46 54 59 Summary  REFER - when diagnosis is uncertain  when appropriate diagnostic studies don’t help  when patterns does not meet diagnostic criteria  when patient is not improving  when co-morbidities interfere  when reasonable medication trials are ineffective