Migraine with brainstem aura

Unusual and rare subtype, formerly known as basilar-type migraine

Introduction

Migraine with brainstem aura is a rare subtype of migraine with aura and studies investigating it are limited.  The symptoms will include two or more of the following:

  • visual disturbances in both eyes
  • speaking difficulties
  • hearing problems
  • tingling in the hands and feet
  • dizziness
  • vertigo
  • ringing in the ears.

Former names/previously used terms for migraine with brainstem aura

  • basilar artery migraine
  • basilar  migraine
  • basilar-type migraine

More information

Below you will find the personal experience of one sufferer of migraine with brainstem aura. This is then followed by an overview from our trustee Dr Brendan Davies. Please note that this text refers to migraine with brainstem aura as basilar-type migraine (its previous name).

Suzanne shares her experience

I woke up on the 18th May 2006 and had no feeling in my right side – from my face down to my right leg; I could hardly stand up and my right arm felt like it was hanging off.  I realised that I’d lost muscle control in my mouth and could not talk or swallow.  I got to Casualty and I could instantly tell that the Doctor thought I’d had a stroke.  When they asked me “Do you usually talk like this?” or “Do you usually hold your head like that?” I was unable to reply other than in unintelligible mumbles.  One month earlier I had been in a minor car crash with major consequences, in that I hadn’t known I’d had concussion, and completed a full day’s work on the day of the crash.

I had a CAT scan and lay on a trolley for four hours thinking the worst.  Finally the Doctor came and as he was talking I realised that my speech had started to return, as had some of the feeling in my right side.  He said I hadn’t had a stroke, nor was I in immediate danger of having a stroke, and I needed to see a neurologist.  I went home, emotional, confused and staggering around like I was drunk.  Although my speech had returned to some extent, I was unable to articulate very well, I was slurring my words and my brain did not appear to want to communicate with my mouth – my mind would say one thing and my mouth would speak differently.

Five years on, I now know that was a basilar migraine attack.  In 2011 I had over 100 basilar attacks although none as severe as that first one.  My attacks now are likely to consist of losing the feeling in my right side followed by the mix of numbness, pins and needles and pain, throbbing pains in my head, slurring of speech, the appearance of being drunk, a decreased level of consciousness, nausea and dizziness.  The main part of the attack lasts 4-6 hours but the dizziness can last for eight hours and the numbness may linger for days.  After every attack I go into the ‘hangover’ or ‘jet lag’ stage, where I’m unable to function as I would wish.  In addition to the basilar attacks I live with a permanent low level migraine and have pressure pains in my head.  My concentration and short-term memory are poor.

I have balancing problems.  Every basilar attack affects my balance and I’ll usually stumble or fall.  Working on your balance is a nightmare – the exercises you have to do basically disturb your balance and make you feel sick, with the hope that you are retraining your brain to recalibrate properly.  I’ve a long list of balancing exercises, but as they make me far worse (the most awful vertigo) I find myself having to make a clear plan to do the exercises rather than just when I feel like it.  Every day I stay in bed my balance decreases by 10% – so a prolonged migraine attack can affect me in more ways than one.

My treatment is a Greater Occipital Nerve (GON) block into the area where I must have hit my head in the car crash five years ago, I still have a huge bump on the back of my head.  At a recent consultation (with Dr Anish Bahra at the National Hospital for Neurology and Neurosurgery in London) bi-lateral blocks were recommended to see if this helps with the migraine aura and then to try flunarizine.  I have never got on well with any of the recommended medicines, (propranolol, amitriptyline, nortriptyline, topiramate).  The injections have made a huge difference to the pain levels which have reduced from 6-8 almost every day, to a pain level of 1-3, but nothing has helped with the aura.  I use Maxalt (rizatriptan) for the most severe attacks, maybe once a month.

Everything seems to be a trigger: caffeine, alcohol, processed foods, tiredness, lack of sleep, loud music, flashing lights, the cinema, too much TV, too much fun.  My diary is planned to manage my migraine, I rarely do anything on two consecutive days and if I go somewhere I am usually the first to leave.  I avoid my triggers as much as possible.

My life is almost unrecognisable from how it was before – I used to have a busy job as a sales manager, juggling lots of projects, going out all day and meeting clients.  I had my own small business too.  Now I work part-time from home running my business, planning my working days around my attacks.  I used to be so reliable but now my friends and family – all of whom are positively brilliant – know that I may cancel things at the last minute.  I miss about 70% of things that I wish I could attend.  On the MIDAS headache scale I score 76 out of 78 points.

I always believe I will get better and I will win this.  I may modify things in my life but I refuse to give up. I’m trying to cycle again as part of the balancing exercises.  My dream is to be well enough to cycle the C2C–140 miles from Whitehaven to Tynemouth.  When I do this you’ll be the first to know.

Dr Brendan Davies comments on basilar-type migraine

The scenario described in the aforementioned description highlights the anxieties and uncertainties that an individual migraine sufferer often experiences during their first severe migraine with aura attack.

Basilar-type migraine is an unusual and relatively rare subtype of migraine with aura.  It is characterised by neurological symptoms that typically include at least two of the following symptoms:

  • Slurring of speech (dysarthria)
  • Vertigo (ie. an illusion of movement)
  • Ringing in the ears (tinnitus)
  • Double vision (diplopia)
  • Unsteadiness of gait as if drunk (ataxia)
  • Transient impairment of consciousness (syncope)
  • Simultaneous bilateral sensory symptoms eg. pins and needles and /or numbness affecting both arms and/or legs
  • Simultaneous bilateral visual aura.

Basilar aura symptoms often develop gradually and typically precede or accompany typical migraine headache in those who experience it.

A recent study from Denmark identified that basilar migraine occurs in about 10% of individuals who suffer migraine with typical visual aura.  Vertigo, slurred speech, tinnitus and diplopia were the commonest reported symptoms.  Some individuals experience disorientation or confusion in addition to transient loss of consciousness termed syncope.

In general most individuals with basilartype migraine first experience symptoms associated with their migraine headache in early adult life, ie. often between late adolescence into their 20s.

The Danish study suggested that basilar-type migraine starting from the first time over 50 years of age was unusual.  However the occurrence of basilar-type migraine attacks can potentially occur at any age.  Individuals experiencing their first attack especially if it occurs later in life, eg. after age 50 years often need investigation such as brain imaging, eg. brain MRI to ensure that other neurological conditions that might cause similar symptoms are not responsible.  The Danish study also identified that sufferers of basilar-type migraine often had other migraine attacks only associated with typical visual aura.

Basilar-type migraine can sometimes be confused with another rare migraine aura variant termed hemiplegic migraine.  Individuals who have hemiplegic migraine experience weakness typically of the arm and face as part of their migraine aura in contrast to more common migraine aura.  It is not uncommon for sufferers of hemiplegic migraine to have associated basilar migraine like symptomatology during their hemiplegic migraine attack in addition to weakness.  The duration of hemiplegic migraine and basilar-type migraine aura symptoms is typically longer than the visual aura experienced by most.

When individuals first present with headache accompanied by the aforementioned neurological symptoms to doctors not aware of basilar-type migraine other conditions are sometimes considered and/or misdiagnosed.  Ménière’s disease, ischaemic stroke, epilepsy, vestibular disorders affecting the ear causing vertigo are the common alternative considerations.

Cortical spreading depression (CSD) is believed to be the neuronal mechanism that generates basilar aura similar to typical visual aura in migraine.  However the current hypothesis is that cortical spreading depression occurs either in the brainstem (the base of the brain) or simultaneously and bilaterally in the cerebral cortex, ie. the surface of the brain.

Genetic studies have now identified at least three genes that cause the familial form of hemiplegic migraine.  These genes all cause dysfunction of ion channels on brain nerve cells and lead to an increase in the levels of an excitatory brain neurotransmitter, ie. glutamate and make the sufferers brain more susceptible to CSD.  Whilst three genes have so far been identified as being responsible for familial hemiplegic migraine by contrast genetic studies looking at individuals who experience attacks of pure basilar migraine without weakness (hemiparesis) have not shown that the same genes are commonly implicated. Thus routine genetic testing is not indicated.

Regrettably there are no good randomised trials that guide how best to treat basilar-type migraine partly due to its infrequent occurrence.  Consensus suggests the preventative therapy with drugs that are considered effective in helping suppress cortical spreading depression in the brain may be the best first line treatments for the treatment of frequent basilar or migraine aura.  Examples of some drugs commonly used include topiramate, sodium valproate and flunarizine, but this is not an all-inclusive list.  Often individuals are also treated with other conventional migraine preventative therapies dependent on the prevalent symptom of head pain, nausea, etc.

Similarly there are no good trials looking at the treatment of acute migraine headache in the context of a basilar-type migraine attack.  In fact such individuals were often excluded from the trials of abortive agents such as triptans.  However several subsequent reports in the medical headache literature and anecdotal clinical experience combined with our theoretical understanding that cortical spreading depression producing aura is likely no different in basilar migraine compared with migraine with typical visual aura leads me to conclude that triptans are not absolutely contraindicated for the treatment of headache once basilar aura has resolved.  There is however no evidence to support their usage for the treatment of basilar aura, ie. the neurological symptoms.

Basilar-type migraine like other forms of migraine with aura can be triggered by all the usually recognised migraine triggers.  If basilar-type migraine attacks occur in the context of hemiplegic migraine then head trauma however minor is also a well-recognised triggering factor.

Finally, as basilar-type migraine is simply a more unusual variant of migraine with aura it is considered to have the same associations as typical migraine with aura.  It is therefore not thought to have an excess ischaemic stroke risk over and above what is considered for migraine with typical visual aura.