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Sudden Onset of Vestibular Migraine Complicated with BPPV and Mal de Debarquement Syndrome -
a Diagnostic Dilemma
Perkovie R.1, Maslovara S.2, 3, Kovaeevie Stranski K.4, Buljan K.1, Tomie S.1, Jurie S.1
'Department of Neurology, J.J. Strossmayer University, Osijek University Hospital Centre, Osijek, Croatia; 2Department
of Otorhinolaryngology, Vukovar County General Hospital, Vukovar, Croatia; 3Department of Otorhinolaryngology and Maxillofacial Surgery, Medical Faculty, J.J. Strossmayer University, Osijek, Croatia; 4Department of Rheumatology, Imunology and Allergology, J.J. Strossmayer University, Osijek University Hospital Centre, Osijek, Croatia ' 3 4Trg Svetog Trojstva 3, 31000, Osijek, Croatia; 2Zupanijska 35, 32000, Vukovar, Croatia
Vestibular migraine is combination of migraine and vestibular symptoms. In clinical examination it can be replaced with benign paroxysmal positional vertigo (BPPV) cupulolithiasis, but also BPPV is common comorbidity in migraine patients. There is also high association between vestibular migraine and Mal de Debarquement syndrome. Patient came to hospital with vertigo that was diagnosed as left PC-BPPVcanalolithi-asis. After first Epley's maneuver symptoms didn't resolved. Week after, at second Epley's maneuver performed patient developed left PC-BPPV cupulolithiasis. Month after, at third Epley's maneuver BPPV resolved but patient developed Mal de Debarquement syndrome. Laboratory testing showed hyperhomocisteinemia and homozygous MTHFR C677T and PAI, with low vitamin D. After reviewing the vestibular symptoms in the first attack which was misdiagnosed as BPPV canalolithiasis, and history of migraine, patient was diagnosed with vestibular migraine. Patient well responded to migraine diet and supplementation with B complex. Vestibular disorders are similar to each other and they can overlap. More attention in taking detailed medical history should be given to patients with vertigo or dizziness.
Keywords: vestibular migraine; BPPV; Mal de Debarquement syndrome. Contact: Romana Perkovie:; romana.per@gmail.com
For reference: Perkovie: R, Maslovara S, Kovaeevie: Stranski K, et al. Sudden Onset of Vestibular Migraine Complicated with BPPV and Mal de Debarquement Syndrome — a Diagnostic Dilemma. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 202';'3(6):'39-'4'. DOI: '0.'44'2/2074-27''-202'-6-'39-'4'
Vestibular migraine (VM) is combination of migraine and vestibular symptoms such as dizziness or vertigo. Migrainous headaches begin earlier than vestibular attacks in most vestibular migraine patients [1]. VM can occur with or without an actual headache. Patients can also have an attenuated headache when they start to have vertigo, as compared with their migraine headache before [2]. The etiology is still unknown. Both cortical spreading depression and trigemino-vascular theory were accepted as they can explain part of symptoms.
Vestibular symptoms commonly seen in VM are spontaneous vertigo, positional vertigo, vertigo on exposure to complex visual stimulations, head motion induced vertigo, ataxia, dizziness, lightheadedness and extreme sensitivity to motion of variable duration from seconds to days. Accompanying symptoms are nausea, phonophobia, photophobia, osmophobia, neck stiffness, scalp allodynia, tinnitus, visual aura, nystagmus and headache. There is no specific diagnostic toll for VM. During attacks, patients with VM can have peripheral or central features of nystagmus, or a mixture of both [3]. Highly suggestive of VM is low-velocity sustained nystagmus during positional testing on a symptomatic day [4]. Neurological and otological findings between attacks are usually normal. There is no right cure for VM, the treatment options include migraine prophylaxis and lifestyle changes.
Woman, 30 years old, complained that she felt short violent rotational vertigo with nausea after waking up and twice again in the
afternoon. Next day when she laid on her left side she felt violent rotational vertigo which subsided when she seat upright, but vertigo started every time she closed her eyes. The patient was referred to an emergency clinic where Dix-Hallpike was performed with positioning nystagmus (PN) when turning left and right followed by subjectively fast intensity of rotational vertigo, more when lying on her left side. After two Epley's maneuvers were done with 5 minutes intervals, there was no satisfactory recovery and the patient was told to come to the emergency clinic in a week for another Epley's.
During the second Dix-Hallpike maneuver, one week after, PN was also seen when lying on the left and right side with subjectively twice as fast rotatory vertigo when lying on the left side than during the first examination. Upon arriving home, the patient noticed that she had a short rotatorional vertigo during each bending of the head with worsening of symptoms with each attempt to lie on her back, especially when turning her head to the left. Because of short latency and extended duration of the attack suspicion had been raised that cupulolithiasis of the posterior canal (PC-BPPV) was caused by the last Epley's maneuver. Between the short episodes of daily rotational vertigo, the patient had gait instability, nausea, pallor, and occasional sweating. One month after the onset of symptoms, the patient was examined by an ENT specialist who also underwent Dix-Hallpike. There was apogeotropical vertical-rotatory PN nystagmus on the left side after a latency of about a few seconds, lasting up to 30 seconds, and also apogeotropic PN on the right, after a latency of'5seconds, for more than 30seconds and less than 60sec-onds. Upon repositioning, the patient developed ataxia and nausea.
Medical history raised the suspicion of vestibular migraine after the patient said she had occasional photophobia and a visual aura for the past month. Migraine headaches subsided when vestibular symptoms started.
Two days after the last Epley's maneuver the nausea and ataxia were reduced, rotatory vertigo had no longer occurred when the head was bent down, but photophobia, phonophobia and visual aura worsened. Disequilibrium and oscillopsia started. Oscillopsia (moving objects from one side to another) lasted for 3 months without a break. Disequilibrium was episodic, only at the peak of vestibular migraine attack. There had also been continuous swaying or rocking in sitting or standing position. Symptoms have been accompanied by anxiety and avoidance of leaving the house. The patient noticed that her symptoms only subside if she ride a bike, walk or ride in a car for a short time. When exiting the vehicle or stopping on the traffic lights swaying and rocking started again. She was diagnosed with persistent Mal de Debarquement syndrome because of continuously presented rocking and swaying, more than one month, which temporarily reduced with exposure to passive motion.
In medical history she has had motion sickness in childhood, migraine headaches up to four times a month last 10 years and BPPVattack seven years earlier which completly recovered after one Epley's maneuver. She also had a history of frequent head and neck trauma: couple of car accidents, 15 years of martial arts practice and one time strike by lightning while laying down — lightning hit by her left ear leaving her with transient pain on left side of face and transient slight hearing loss.
In laboratory were found homozygous mutation of the flavoen-zyme 5,10-methylenetetrahydrofolate reductase (MTHFR) and plasminogen activator inhibitor (PAI), which resulted in hyperhomo-cysteinemia (18 ^mol/L). A low amount of vitamin D3 of 22 nmol/L (reference interval 20—100 nmol/L) was also found. Complete blood count, biochemical, immunological and hormonal processing was normal. Head CT, brainstem audio-evoked potentials and audiolog-ical testing were normal.
Patient took the analgesics but because of persistent everyday symptoms, she had started preventive treatment with erenumab 70 mg s.c. She was hypersensitive to the drugs and erenumab had the fewest recorded side effects. First two weeks she felt better with less symptoms, then her migraine transformed into hot flushes and high intenisty neck spasms accompanying with depressive thoughts. After four weeks of erenumab, symptoms were subjectively 30% less but we discontinued the medication because of depression side effect. After stopping erenumab, depression subsided but all vestibular and migraine symptoms returned with the same intensity. She received antiemetic drugs on three occasions due to nausea, after each intake she developed mild extrapyramidal side effects including hand tremor and jaw spasms which subsided immediately after the drug was excreted.
Due to drug hypersensitivity, the patient began a migraine diet and supplementation with magnesium 400 mg, B complex and vitamin D 400 i.u. The visual aura disappeared three weeks after starting the migraine diet. After four months of diet, the vestibular symptoms slowly decreased in our patient. After six months of waking up every couple of hours with spinning sensation in the sleep she had again good sleep quality.
Discussion
Vestibular migraine is frequently unrecognized but it is the most common cause of central, spontaneously occuring, episodic vertigo. VM is diagnosed by rulling out all other conditions
and then by following diagnostic criteria published by International Barany Society and the International Headache Society. Diagnostic criteria includes at least 5 episodes of vestibular symptoms of at least moderate severity lasting between 5 minutes to 72 hours, a current or previous history of migraine, and migrainous symptoms involved in at least two vertiginous attacks [2]. In VM patients motion sickness history and anxiety are common.
Our patient also developed symptoms and met criteria for BPPV and Mal de Debarquement syndrome probably caused by positional testing. Migraine has been found to be three times more common in patients with idiopathic BPPV than in patients with BPPV secondary to trauma or surgical procedures [5]. In BPPV, attacks of rotational vertigo are recurrent, short and provoked by head position changes.
There is diagnostic dilemma because there are close similarities between BPPV cupulolithiasis and VM. They both can be presented by positional nystagmus in the Dix-Hallpike maneuver, with short latency and prolonged duration with sense of vertigo in the provoking position. Our patient stopped having rec-curent rotational vertigo when bending down after Epley's maneuver, so we lean to the side that she developed cupulolithi-asis more than this was just vestibular migraine symptom. Pathophysiological link between migraine and BPPV could be repetitive vasospasms or disturbance of vestibule-cochlear microvasculature might play a role in inner ear insult, resulting in damages of the epithelium in the vestibule, causing dislodge of the otoconia from utricular macula into semicircular canal, and thus give rise to BPPV [6].
Mal de Debarquement syndrome is also frequently unrecognized. On average, patients must undergo 19 visits to a various doctoral offices before receiving a diagnosis [7]. MDDS is subjective perception of self-motion following exposure to passive motion (motion triggered MDDS), or can occur spontaneously (spontaneous-onset MDDS). Most common trigger for MDDS are boat trips or a cruise, air travel and land travel. Other triggers are stress, positional changes, head movements and hormonal changes [8]. Most common symptoms of MDDS are rocking, bobbing and swaying that is often accompanied by unsteadiness and disequilibrium. There are no findings on diagnostic tests that are pathognomonic of MDDS. There is high association between MDDS and migraine, but also MDSS and motion sickness, from which our patient had suffered before [9].
Diagnostic criteria for MDDS includes non-spinning vertigo characterized by an oscillatory perception («rocking», «bobbing», or «swaying») presented continuously or for most of the day, onset which occurs within 48 hours after the end of exposure to passive motion, symptoms temporarily reduced with exposure to passive motion, symptoms continued for >48 hours and symptoms not better accounted for by another disease or disorder [10].
Symptoms during vestibular migraine attacks for patients that have MDDS and VM (MDDS-VM) are intracephalic spinning, lightheadedness, floating, ground-shifting, unsteadiness, oscillopsia, visually induced vertigo, spinning, retro-orbital haedache, occipital headache, fullness/pressure in ears, muffled hearing, blurry vision, neck ache, cognitive slowing (brain fog), fatigue, nausea, photophobia, phonophobia, osmophobia, visual aura and orthostatic hypotension. Self-reported triggers for MDDS-VM ictal episodes are stress,
menses, sleep deprivation, missing meals, prolonged computer use, following prolonged drives, weather changes, heat, seasonal allergies, bright lights, strong odors, noisy environments, neck pain and dietary triggers [11]. Relieveing factors are exposure to passive motion, lying down, rocking chair use, moving around and excercise. MDDS-VM patients appear to be more disabled than patients with only MDDS, in terms of severity of dizziness, job impact, and number of symptoms, but have good potential for improvement with migraine prophylactic treatment [12].
In laboratory search for potential cause of vestibular and migraine disorders we found that our patient has hyperhomocis-teinemia and is homozygous for MTHFR C677T and PAI. The MTHFR has a main role in the pathogenesis of migraine and hyperhomocysteinemia, he regulates the flow of folate (vitamin B9). Cause of hyperhomocysteinemia is abnormal methionine biosynthesis which occurs because of deficiencies vitamin B9, vitamin B12, and vitamin Bg [12]. Studies [13, 14] reported that supplementing with B6, B9, B12 vitamins significantly reduce homocysteine levels and also reduce the severity of migraine headache and disability among migraineurs when compared to placebo. Carriers of the C allele of the MTHFR C677T variant, compared with those with TT genotype, show better results in reductions of homocysteine levels and in reduction of migraine symptoms.
We had great success introducing B complex vitamins which would speak in favor of hyperhomocysteinemia as one of the causes of the symptoms. B complex reduced frequency and intensity of attacks which is based on previous studies in patients with MTHFR C677T variant such as our patient.
Also the symptoms were reduced with the help of a migraine diet. Migraine diet is temporary restrictive diet with low tyramine, low histamine, without food additives especially monosodium glutamate (MSG) and without nuts, chocolate, alcohol, aged cheese and caffeine, also there are some restrictions in certain fruits and vegetables. Main goal of the migraine diet is identifying triggers in food, so you can eliminate that food and increase your threshold which will reduce the severity, frequency and intensity of attacks. Our patients also went gluten free and diary free. After four months of diet, specific food can be slowly introduced back and see if it is still trigger for migraine.
Conclusion
Vestibular disorders are similar to each other, they can overlap and patients can have normal neurological and otologi-cal findings. Path to diagnosis is long and demanding and requires a multidisciplinary approach. Due to the severe disability that goes with vestibular disorders, more attention in taking detailed medical history should be given to patients with vertigo or dizziness.
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Received/Reviewed/Accepted 19.07.2021/02.09.2021/05.09.2021
Conflict of Interest Statement
The investigation has not been sponsored. There are no conflicts of interest. The authors are solely responsible for submitting the final version of the manuscript for publication. All the authors have participated in developing the concept of the article and in writing the manuscript. The final version of the manuscript has been approved by all the authors.
