Hemiplegic Migraine

Medical Student Clinical Pearl – January 2020

Alyssa BeLong, B.Sc.(Hon)

Dalhousie Medicine New Brunswick

M.D. Candidate, Class of 2021

Reviewed and Edited by Dr. David Lewis


Case Presentation

A 45-year-old female presented with sudden-onset left-sided vision loss, right arm paralysis and auditory changes 24 hours ago. She subsequently developed a throbbing pain (6/10) behind her left eye which radiated over her scalp, with a sensation of water dripping down the back of her neck. Her symptoms resolved within 30 minutes except for ongoing headache and photophobia.


Differential Diagnosis

A variety of conditions may present with transient unilateral weakness or hemiplegia: (4)

  • Hemiplegic Migraine
  • Transient Ischemic Attack (TIA): Typically present with sudden onset of all symptoms rather than progression from one to another. A TIA is also less likely to present with headache, nausea, photophobia, phonophobia.
  • Brain Tumor: Typically present as progressive rather than transient neurologic symptoms.
  • Epilepsy with Post-Ictal Paralysis: Would expect paroxysmal symptoms at time of onset or change in level of consciousness as well as post-ictal confusion. Duration of symptoms also makes this unlikely.
  • Stroke-like Migraine Attacks After Radiation Therapy (SMART)
  • Other possible but rare/unlikely diagnoses include headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL), CNS infection, Sturge-Weber syndrome as well as certain inherited disorders and metabolic disturbances.

Case Continued – History and Physical Exam

Clarification of visual field disturbance revealed a left homonymous hemianopia rather than loss of vision in the left eye. There was no change in speech or facial droop. There were no precipitating events and there were no alleviating or aggravating factors. The patient noted herself to be particularly stressed lately. She was otherwise healthy with a past medical history of migraines without aura many years prior. Family history was negative for thromboembolic events, she was not taking any medications and had no history of smoking or substance use.

On physical exam, the patient appeared well with all vital signs within normal limits. Cranial nerve exam was unremarkable apart from ongoing photophobia in her left eye. There was normal motor, strength, sensation, tone and reflexes bilaterally. There was no evidence of gait disturbance or dysdiadochokinesia.


Migraine Overview

Migraines typically present as severe episodic headaches often accompanied by photophobia, phonophobia and/or nausea, however presence of an aura can yield a variety of presentations. Migraines are currently thought to be neurologic in origin, although the exact pathophysiology remains unknown (2). Migraines were previously thought to be due to vascular changes, with vasodilation causing headache and vasoconstriction causing aura, however this theory is no longer viable (2).

Migraines affect 17% of women and 6% of men, with an overall prevalence of 12% (2). Migraines typically flow through four phases (2):

  1. Prodrome: Change in affect or vegetative symptoms 24-48hrs prior to onset of headache.
  2. Aura: Focal neurologic symptoms, including visual, sensory, language or motor disturbance.
  3. Headache: Often unilateral but can be bilateral, typically throbbing or pulsatile in quality, frequently accompanied by photophobia, phonophobia, nausea or vomiting.
  4. Postdrome: Sudden movement may trigger transient pain in location of the resolved headache.

While many types of migraines exist, 75% of migraines do not have an aura (2). Some patients also experience aura without headache. Factors thought to be involved in precipitation of migraine include stress, menstruation, fasting, weather, nitrates, wine and visual triggers (2, 3).  

Hemiplegic Migraine

  1. At least two attacks fulfilling criteria B and C
  2. Aura consisting of both of the following:
    1. Fully reversible motor weakness
    2. Fully reversible visual, sensory and/or speech/language symptoms
  3. At least two of the following four characteristics:
    1. At least one aura symptom spreads gradually over ≥5 minutes, and/or two or more symptoms occur in succession
    2. Each individual non-motor aura symptom lasts 5 to 60 minutes, and motor symptoms last <72 hours
    3. At least one aura symptom is unilateral
    4. The aura is accompanied, or followed within 60 minutes, by headache
  4. Not better accounted for by another ICHD-3 diagnosis, and transient ischemic attack and stroke have been excluded

Familial hemiplegic migraine requires one first or second degree relative to meet the above criteria for hemiplegic migraine. Sporadic hemiplegic migraine encompasses those who do not meet familial criteria. (4, 5).

  1.  

Treatment

Treatment of acute migraine in the emergency department follows similar principles to abortive management in an outpatient setting (6):

Abortive Agents

  • Triptans
    • Sumatriptan 6mg SC
  • Antiemetics / Dopamine Receptor Blockers
    • Metoclopramide 10mg IV, Prochlorperazine 10mg IV or Chlorpromazine 0.1mg/kg IV up to 25mg IV)
    • Diphenhydramine: given with parenteral antiemetics to prevent akathisia or dystonia. 12.5-25mg IV (q1h up to two doses)
  • Dihydroergotamine 1mg IV + Metoclopramide 10mg IV can be given if Metoclopramide monotherapy is ineffective.
  • Dexamethasone 10-25mg IV (or IM): Recommended in conjunction with the above treatments to lower risk of early headache recurrence.

In general, hemiplegic migraines can be treated the same as typical migraine with aura (4). Triptans and ergotamine are currently contraindicated due to their effect on vasoconstriction and theoretical risk of ischemic events, although this recommendation may change with evolving theory of migraine pathophysiology (4, 7).

Opioids are not recommended as first-line therapy and should not be routinely used in the acute management of migraine (6, 8).  


Case Continued – Treatment

The following medications were given in the emergency department:

  1. 10mg Metoclopramide IV
  2. 1mg Benztropine IV (for prevention of dystonia)
  3. 10mg Dexamethasone IV

Case Conclusion

The patient’s headache resolved with IV medications. She was advised to take it easy and consider scaling back on her shifts at work – a significant source of her stress. The patient was very pleased with her treatment and was discharged home.


Sources

  1. Donnelly K (2011). Homonymous Hemianopsia. In: Kreutzer J.S., DeLuca J., Caplan B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, New York, NY. DOI: https://doi.org/10.1007/978-0-387-79948-3_739
  2. Cutrer F. Pathophysiology, clinical manifestations, and diagnosis of migraine in adults. In: UpToDate, Eichler A (Ed), UpToDate, Waltham, MA. (Accessed on December 23rd, 2019.)
  3. Martin VT, Behbehani MM (2001). Toward a rational understanding of migraine trigger factors. Medical Clinics of North America 85(4):911.
  4. Robertson C. Hemiplegic Migraine. In: UpToDate, Eichler A (Ed), UpToDate, Waltham, MA. (Accessed on December 23rd, 2019.)
  5. Headache Classification Committee of the International Headache Society (IHS) (2013). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 33(9):629-808. DOI: 10.1177/0333102413485658
  6. Smith J. Acute Treatment of Migraine in Adults. In: UpToDate, Eichler A (Ed), UpToDate, Waltham, MA. (Accessed on December 23rd, 2019.)
  7. Russell MB, Ducros A (2011). Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurology 10(5):457-70. DOI: 10.1016/S1474-4422(11)70048-5
  8. Friedman BW, West J, Vinson DR, Minen MT, Restivo A, Gallagher EJ (2015). Current management of migraine in US emergency departments: an analysis of the National Hospital Ambulatory Medical Care Survey. Cephalalgia 35(4):301.
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EM Reflections – June 2019 – Part 2

Thanks to Dr. Joanna Middleton for leading the discussions this month

Edited by Dr David Lewis 


Discussion Topics

  1. When is a pregnancy not a pregnancy? (see part 1)
  2. Caustic Ingestions (see part 1)
  3. Transient Ischemic Attack – ED Questions

 

Transient Ischemic Attack – ED Questions

 

Transient Ischemic Attack (TIA): A brief episode of neurological dysfunction caused by focal brain, spinal cord or retinal ischemia, with clinical symptoms and without imaging evidence of acute infarction. Transient ischemic attack and minor stroke are the mildest form of acute ischemic stroke in a continuum that cannot be differentiated by symptom duration alone, but the former typically resolves within one hour.

https://www.strokebestpractices.ca/

 

Dual Anti-Platelet Therapy (DAPT)?

Patients who present within 48 hours of a suspected transient ischemic attack are at the highest risk for recurrent stroke

Uptodate – DAPT for high-risk TIA, defined as an ABCD2 score of ≥4

For CVA – ASA only unless already on ASA, then DAPT.  For minor CVA/TIA – DAPT


Hold Birth Control?


 

Admission?

Of all ischemic strokes during the 30 days after a first TIA, 42 percent occurred within the first 24 hours.

 


Stroke Assessment Pocket Cards

Saskatchewan TIA Referral Pathway

Saskatchewan TIA Patient Information Leaflet

 

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It’s all in your head, literally! – Seizures versus Psychogenic Non-epileptic Seizures

It’s all in your head, literally! – Seizures versus Psychogenic Non-epileptic Seizures

Resident Clinical Pearl (RCP) May 2019

Allyson Cornelis – PGY2 FMEM Dalhousie University, Saint John NB

Copyedited by Renee Amiro

Reviewed by Dr. David Lewis

 


 


 

Background

When patients present with seizure like activity it can be difficult to distinguish true seizure/epilepsy from psychogenic non- epileptic seizures (PNES; also known as pseudoseizures). This task is made more difficult by the fact that 10-30% of patients with PNES can have true epilepsy as well4. The risks associated with diagnosing a psychogenic non-epileptic seizure as true seizure are mainly associated with administration of anti-epileptic drugs during both acute episodes and chronically, with the potential for associated side effects3-4,6. The most severe of these include sedation and even intubation if large enough doses are administered during an acute seizure episode. Additionally, there is added cost to both the patient and the healthcare system for continued use of medications and hospital admissions/investigations.

The underlying mechanism for PNES is believed to be psychiatric in origin, often attributed to conversion disorders, and patients are often not aware of their seizure like behaviours.


 

Risk factors for PNES include:

  1. childhood trauma
  2. PTSD
  3. depression
  4. anxiety
  5. personality disorders
  6. female gender

The challenge remains distinguishing between true seizures and PNES. There are various historical features and seizure characteristics that can assist in differentiating the two, though no one feature is confirmatory for seizure.


 

Distinguishing between PNES and true seizure3-8

Sign/symptom Seizure PNES
Eyes *open Closed, resist forced opening by examiner

 

*Fluttering

Seizure onset *abrupt Gradual
Awareness during seizure Not aware * awareness during episode
Influence of the presence of others Does not change seizure *May intensify or alleviate

 

activity may only occur/be triggered by the presence of others

Seizure activity Generalized tonic clonic

 

Synchronous

 

Stereotyped (first stiff and in extension, then develops synchronous clonic activity)

May be asynchronous, asymmetrical, waxing and waning

Thrashing/violent

Pelvic thrusting

Post ictal *Confusion May recall events during their apparent unresponsive event
head One sided Side to side head turning during event
**incontinence common occasional
***Tongue biting Common, may be severe, usually on SIDE of tongue Occasional, rare to be severe, may be on tip of tongue or the lip
Post ictal corneal reflex impaired normal
Post ictal babinksi upgoing downgoing
Hand drop test negative Positive (patient moves hand away from face)
Response to sternal rub/nail bed pressure Usually nonresponsive May stop seizing, withdraw from stimuli
****Vital signs Desaturation more likely

Ictal apnea

Ictal bradycardia

 

 

 

*represents elements found to be most useful in distinguishing PNES and ES8

** incontinence has little utility in distinguishing between PNES and true seizure5

*** lateral tongue biting was 100% specific for true seizure vs 38% sensitivity and 75% specificity for any type of tongue bite5

****prospective trial7


 

Lab Values

No lab value has proven consistently useful for confirming seizure versus PNES.

A note on Prolactin:

The American Academy of Neurology released guidelines in 2005 recommending the use of prolactin following a seizure event2.

  1. Best when drawn 10-20 minutes after the event and can be used to differentiate between PNES and true seizure
  2. If >6 hours later prolactin should be at baseline levels
  3. Cannot be used to differentiate seizure from syncope
  4. Not applicable in status epilepticus or repetitive seizures

 

Bottom Line: 

  1. Challenging to differentiate between PES and true seizure and some patients can have both!
  2. No definitive distinguishing measure but eye opening, abrupt seizure onset, and confused post-ictal state can help point toward true seizure.
  3. A normal prolactin is more helpful in ruling out seizure while an elevation is non-specific and cannot be used to confirm seizure.

 

References

  1. Abubakr A, Wambacq I. Diagnostic value of serum prolactin levels in PNES in the epilepsy monitoring unit. Neurol Clin Pract. 2016 Apr; 6(2): 116–119.
  2. Graham L. AAN releases guidelines for the use of serum prolactin assays in diagnosing epileptic seizures. Am Fam Physician. 2006. Apr; 73(7): 1284.
  3. Huff JS, Murr N. Seizure, Pseudoseizures. [Updated 2018 Oct 27]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2018 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441871/
  4. Mellers JDC. The approach to patients with “non-epileptic seizures.” Postgrad Med J. 2005 Aug;81(958):498-504.
  5. Nowacki T, Jirsch JD. Evaluation of the first seizure patient: Key points in the history and physical examination. 2017 Jul;49:54-63. doi: 10.1016/j.seizure.2016.12.002. Epub 2016 Dec 8.
  6. Panayiotopoulos CP. The Epilepsies: Seizures, Syndromes and Management. Oxfordshire (UK): Bladon Medical Publishing; 2005. Chapter 1, Clinical Aspects of the Diagnosis of Epileptic Seizures and Epileptic Syndromes. Available from: https://www.ncbi.nlm.nih.gov/books/NBK2609/
  7. Pavlova M, Abdennadher M, Singh K, Katz E, Llewellyn N, Zarowsly M, et al. Advantages of respiratory monitoring during video- EEG evaluation to differentiate epileptic seizures from other events. Epilepsy Behav. 2014 Mar; 32: 142–144.
  8. Syed Tu, LaFrance WC Jr, Kahriman ES, Hasan SN, Rajasekaran V, Gulati D, et al. Can semiology predict psychogenic nonepileptic seizures? A prospective Ann Neurol.2011 Jun;69(6):997-1004
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EM Reflections – October 2017

Thanks to Dr Joanna Middleton for leading the discussion this month

Edited by Dr David Lewis

Top tips from this month’s rounds:

  1. Imaging reports can underestimate the clinical impact of an incidental finding

  2. Neuro ICU in the Emergency Department?

 


Imaging reports can underestimate the clinical impact of an incidental finding

Not all benign conditions have a benign outcome. A CT report will occasionally underestimate the clinical impact of an incidental finding. Its always worth reviewing the images yourself.

For example – a report might read – “No acute bleed or infarct, incidental finding of frontal bone fibrous dysplasia” –  may sound innocuous and unrelated to the patient’s headache, until you review the scans yourself:

 

Fibrous dysplasia is a benign condition which can present with new craniofacial asymmetry. Whilst the condition itself may be benign, the location and speed of growth can result in symptoms, especially headache and even cranial nerve compression.

Clinical Guidelines for managing craniofacial fibrous dysplasia

 


Neuro ICU in the Emergency Department?

 

Management of Intracranial Hemorrhage in the Emergency Department can be complex. The diagnosis is usually straightforward with CT (providing it has been considered as a possibility – subarachnoid hemorrhage can present with syncope alone) and the broad category of bleed determined by the history, patient age, CT appearance, etc.

ED Management will depend on the category of bleed (Primary ICH, Subdural, Epidural, Traumatic SAH, Spontaneous SAH).

From ALIEM.com, click here for the full article

 

Initial management of intracranial hemorrhage can be simplified / summarized as follows:

Airway – ET Intubation if GCS < 9

Breathing – Ventilate if GCS < 9 (SaO2 >94%, ETCO2 35-45 mmHg)

Circulation

  1. Stop the bleeding
    1. Neurosurgery (see here for indications)
    2. Reverse anticoagulation
    3. ?Tranexamic acid
  2. Maintain an adequate cerebral perfusion pressure (CPP) to ensure adequate tissue oxygenation
    1. CPP = Mean Arterial Pressure (MAP) – Intracranial Pressure (ICP)
      1. Seems simple enough? – ensure the patient’s blood pressure is high enough to overcome the ICP
    2. However, the optimal CPP following acute brain injury is not known (general consensus suggest 50-70 mmHg)
      1. In the normal brain CPP is maintained by autoregualtion
      2. Autoregulation is less effective after brain injury
      3. If the CPP is too low brain hypoxia occurs
      4. If the CPP is too high there may be a risk of hematoma expansion
    3. However, it’s not easy to measure the ICP
      1. Methods of non-invasive ICP estimation:
        1. Level of consciousness
        2. Papilledema
        3. CT appearances
        4. Transcranial doppler
        5. Sonographic Optic Nerve Sheath Diameter
        6. Lots of others
        7. None of these are perfect
      2. Invasive ICP measurement
        1. External Ventricular Drain – Neurosurgical procedure
        2. Setting up the EVD and measuring ICP requires experienced nursing staff (see below)
    4. Even measuring the MAP is not without its own problems in the ED
      1. MAP = (Systolic BP + 2(Diastolic BP))/3
      2. However non invasive measurement of MAP (based on SBP and DBP peripheral sphygmomanometry) is not accurate.
      3. An accurate measurement of MAP requires invasive monitoring via an arterial line.
    5. Assuming that we are able to accurately measure ICP and MAP, there is then the question of how to adjust these values reliably via therapeutic interventions.
      1. ICP Management (Normal = 0-15, Goal < 20)
        1. Patient position, head up
        2. Sedation and paralysis, if patient aggitated
        3. Mannitol – potential risk of acute kidney failure in prolonged use
        4. Hyperventilation – will also reduce cerebral blood flow – so PaCO2 no lower than 35 mmHg
        5. CSF Drainage : 
        6. Hypothermia
      2. MAP Management
        1. IV Fluid (crystalloid vs colloid?)
        2. Diuretics / Antihypertensives vs Inotropes
        3. A very detailed guide to blood pressure management in stroke can be viewed here: BP-Stroke


I suspect that most emergency physicians/nurses are wondering whether this level of care falls within their remit. In most hospitals the answer will be NO, these cases are stabilised and managed in an Intensive Care Unit. However, there are occasions when this level of care is required prior to transfer to another unit/hospital, in which case it is likely that the care will be directed by the local neurosurgeon / neurointensivist and the receiving specialists.


EVD Drainage System and ICP Monitoring

 

Suggest ICP Protocol from Vancouver General ICU

Download (PDF, 110KB)

 


 

CME QUIZ

 

ED Reflections - CME Quiz - Oct 2017

ED Reflections – CME Quiz – Oct 2017

 


 


Click Print, PDF or Email to save a record of this CME

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ED Rounds – May 2016

Imaging Decisions in Vascular Disease

Presented by Dr. Dylan Blacquiere (Neurologist)

 


 

Download (PDF, 11.39MB)

 


New Imaging Recommendations. Dr Jake Swan (Radiologist)

After meeting with Dr. Blacquiere and the ER department regarding stroke management and SAH management, I’m recommending the following based on new literature and evolving management in “high risk” patients.

1) High risk TIA patients, such as those who had a profound motor / speech deficit that is resolving should have a CTA carotid / COW as well as their standard CT head.

2) SAH patients should have CT done prior to LP due to false positive LP rates.  If there is any question about vascular malformation / aneurysm, follow with a CTA. The CTA isn’t necessary for every headache patient, etc, just those with a positive bleed on the unenhanced CT.


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HINTS exam in Acute Vestibular Syndrome

The eyes are the window to the brain: HINTS exam in acute vestibular syndrome

Resident Clinical Pearl – January  2016

Jacqueline MacKay, PGY2 iFMEM, Dalhousie University, Saint John, New Brunswick

Reviewed by: Dr Joanna Middleton and Dr David Lewis

 

Acute vestibular syndrome (AVS) is the rapid onset of vertigo, nausea/vomiting, and gait unsteadiness combined with head-motion intolerance and nystagmus that lasts days-weeks. Often these dizzy patients have a benign, self-limiting cause for their symptoms, however it is estimated that up to 25% of AVS presentations to emergency departments are due to posterior circulation infarcts.

 

CT scan has low sensitivity for identifying acute infarct, especially in the posterior fossa. MRI is not always available, and will often have false-negative results in acute posterior circulation strokes. Are bedside predictors able to identify central causes of acute vestibular syndrome?

 

The HINTS exam is a bedside test that carefully assesses eye movements. HINTS stands for Head Impulse-Nystagmus-Test of Skew.


Untitled

Head Impulse: test of vestibulo-ocular reflex function. A normal Head Impulse test (HIT) strongly indicates a central localization for the AVS. An abnormal HIT usually indicates a peripheral lesion.


Untitled1

Nystagmus: bilateral nystagmus which changes direction on eccentric gaze or primarily vertical nystagmus is predictive of central pathology.


Untitled2

Skew Deviation: a vertical ocular misalignment that is assessed by alternate cover testing


Watch the video! A short and excellent description of the exam with good examples of normal and abnormal:

https://vimeo.com/133033089 (Courtesy of EMCrit)


 

Interpretation

A benign HINTS exam is defined as abnormal HIT + direction-fixed horizontal nystagmus + absent skew.

A dangerous HINTS exam is defined as any one of:

  • Normal/untestable HIT
  • or direction-changing horizontal nystagmus present/untestable
  • or skew deviation present/untestable

(Untestable refers to those patients with obvious oculomotor pathology or lethargy in whom the tests were unable to be completed).

 

The acronym INFARCT can be used to remember what constitutes a dangerous HINTS exam:

Impulse Normal

Fast-phase Alternating

Refixation on Cover Test.

 

A dangerous HINTS result was found to be 100% sensitive and 96% specific for the presence of a central lesion when applied to patients with acute vestibular syndrome (continuous vertigo and nystagmus) with at least one stroke risk factor. In fact, the HINTS exam is more accurate than MRI to diagnose stroke in patients with AVS in the first 48 hours!


 

Bottom Line:

In the acutely dizzy patient with at least one stroke risk factor, remember the HINTS to an INFARCT

 


References:

  1. Kattah, J. C., Talkad, A. V., Wang, D. Z., Hsieh, Y. H., & Newman-Toker, D. E. (2009). HINTS to diagnose stroke in the acute vestibular syndrome three-step bedside oculomotor examination more sensitive than early MRI diffusion-weighted imaging. Stroke, 40(11), 3504-3510. DOI: 10.1161/STROKEAHA.109.551234
  2. EMCrit http://emcrit.org/misc/posterior-stroke-video/ – original source of the videos is http://novel.utah.edu/Newman-Toker/collection.php

 

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