Parkland Morning Report: Hemiplegic Migraine

Today, Dr. Hunter Stone presented a case of atypical migraine presenting as left sided hemiparaesthesias, left-sided weakness and blurred vision of the left eye.

Case:

A 40-year old Hispanic woman with a history of poorly controlled hypertension (typical blood pressures in 170-210s/100-110s) with medication non-adherence who presented with left face, tongue, arm, and leg numbness, blurred vision of the left eye, and distal left sided weakness of the left hand and foot of approximately 8 hours duration associated with hypertension with systolic blood pressure in the 200s. Speech and gait were normal. There was an absence of headache, nausea, fevers, post-ictal confusion, tremor, rash, and weight loss or gain. Of note, this is her first recurrence of a similar constellation of symptoms that spontaneously resolved one month prior; she did not seek medical attention at that time. Family history was negative for relatives with neurologic diseases or headaches. Social history did not demonstrate tobacco, alcohol, or elicit substance abuse. MRI of the head was unremarkable. MRA of the head and neck was notable for for focal severe stenosis of the right posterior cerebral artery. Transthoracic echocardiogram with bubble study did not identify any interatrial shunt. Her basic metabolic panel and her complete blood count was unremarkable.

Throughout her 2-day hospital course, her blood pressure was controlled with a combination of triple oral therapy and a brief course of aggressive treatment with a nicardipine infusion once stroke was ruled out. However, her symptoms waxed and waned independent of her blood pressure. In the absence of strokes or focal enhancing lesions on MRI, consideration was given to atypical migraine. She was treated with abortive triptan that resulted in complete resolution of symptoms.

Discussion:

The above patient presented with transient and recurrent episodes of left-sided hemiparaesthesias and weakness associated with blurred vision of the left eye and severe hypertension. Initial concerns were for demyelinating disease, stroke (either hypertensive or embolic in phenomenon), vasculitis (infectious or otherwise), and posterior reversible leukoencephalopathy syndrome. However, imaging was not consistent with a cerebrovascular phenomenon, inflammatory process, demyelinating disease, embolic source, or any other white matter phenomenon. In the absence of the above processes and resolution with abortive triptan therapy, recurrent and transient nature, the diagnosis of hemiplegic migraine was highly suspected.

Migraine headaches are broadly classified into two major subtypes: migraine with aura and migraine without aura.

The International Classification of Headache Disorders (3rd edition) criteria for migraine without aura are as follows:

A) At least five attacks fulfilling criteria B through D

B) Headache attacks lasting 4 to 72 hours (untreated or unsuccessfully treated)

C) Headache has at least two of the following 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, vomiting, or both
  • Photophobia and phonophobia

E) Not better accounted for by another ICHD-3 diagnosis

The International Classification of Headache Disorders (3rd edition) criteria for migraine with aura are as follows:

A) At least two attacks fulfilling criterion B and C

B) One or more of the following fully reversible aura symptoms:

  • Visual
  • Sensory
  • Speech and/or language
  • Motor
  • Brainstem
  • Retinal

C) At least two of the following four characteristics:

  • At least one aura symptom spreads gradually over ≥5 minutes, and/or two or more symptoms occur in succession
  • Each individual aura symptom lasts 5 to 60 minutes
  • At least one aura symptom is unilateral
  • The aura is accompanied, or followed within 60 minutes, by headache

D) Not better accounted for by another ICHD-3 diagnosis, and transient ischemic attack has been excluded

Among migraines with aura, there is a typical presentation (migraine with typical aura) as well as various subtypes.

Migraines with typical aura are defined by the following criteria:

A) At least two attacks fulfilling criteria B through D

B) Aura consisting of visual, sensory and/or speech/language symptoms, each fully reversible, but no motor, brainstem, or retinal symptoms

C) At least two of the following four characteristics:

  • At least one aura symptom spreads gradually over ≥5 minutes, and/or two or more symptoms occur in succession
  • Each individual aura symptom lasts 5 to 60 minutes
  • At least one aura symptom is unilateral
  • The aura is accompanied, or followed within 60 minutes, by headache

D) Not better accounted for by another ICHD-3 diagnosis, and transient ischemic attack has been excluded

Migraine subtypes include: hemiplegic migraine, migraine with brainstem aura, retinal migraine, vestibular migraine, catamenial migraine, and chronic migraine. Descriptions for the first two are noted below:

Hemiplegic migraine, which the above patient was ultimately diagnosed with is a rare disorder that has both familial types as well as sporadic. It is characterized by motor weakness as the aura but may also include headache, visual scotomata, visual disturbances, paraesthesias, numbness, fever, and lethargy.

Diagnostic criteria are as follows:

A) At least two attacks fulfilling criteria B and C

B) Aura consisting of both of the following:

  • Fully reversible motor weakness
  • Fully reversible visual, sensory and/or speech/language symptoms

C) At least two of the following four characteristics:

  • At least one aura symptom spreads gradually over ≥5 minutes, and/or two or more symptoms occur in succession
  • Each individual non-motor aura symptom lasts 5 to 60 minutes, and motor symptoms last <72 hours
  • At least one aura symptom is unilateral
  • The aura is accompanied, or followed within 60 minutes, by headache

D) Not better accounted for by another ICHD-3 diagnosis, and transient ischemic attack and stroke have been excluded

Migraine with brainstem aura (also known as basilar migraine) should be considered with migraines with clinical features of a brainstem pathology without motor weakness. If there is motor weakness, hemiplegic migraine is the likely diagnosis.

The diagnostic criteria for migraine with brainstem aura are:

A) At least two attacks fulfilling criteria B through D

B) Aura consisting of visual, sensory and/or speech/language symptoms, each fully reversible, but no motor or retinal symptoms

C) At least two of the following brainstem symptoms:

  • Dysarthria
  • Vertigo
  • Tinnitus
  • Hypacusis
  • Diplopia
  • Ataxia
  • Decreased level of consciousness

D) At least two of the following four characteristics:

  • At least one aura symptom spreads gradually over ≥5 minutes, and/or two or more symptoms occur in succession
  • Each individual aura symptom lasts 5 to 60 minutes
  • At least one aura symptom is unilateral
  • The aura is accompanied, or followed within 60 minutes, by headache

E) Not better accounted for by another ICHD-3 diagnosis, and transient ischemic attack has been excluded