Category Archives: Atypical BPPV

Positional DBN central or peripheral

Woman 53yr with symptoms which resembles BPPV history, but not very convincing. Her vertigo started in the morning while she was still in the bed with feeling of sinking. All the time she is dizzy and she can’t move the head neither eyes because it worsens her dizziness; slight instability. She vomited for two times. The same symptoms she had two months ago after yoga exercises and three days after, canalith repositioning maneuver completely resolved her symptoms.

No spont Ny. VHIT is good. But Dix-Hallpike shows downbeating nystagmus which is accompanied with slight vertigo (slightly stronger at the right side).

OK. Let’s check for the central vestibular function.
VNG shows good saccades but smooth pursuit is rather saccadic at both directions more pronounced to the right. But the patient is very drowsy because of Chloropyramine (antihystamine).
I’ve performed Epley (for the right) and demi-Semone.

On the next day she is still dizzy and Dix-Hallpike still shows downbeat nystagmus. Also there’s slight myosis at the right eye.
VNG shows better tracking gains but gain to the right is significantly lower!
Deep-head hanging maneuver and Epley didn’t resolved the downbeat nystagmus (slightly less intense).

Few days later, she is without vertigo and also without any positional nystagmus.

MRI shows arachnoid cyst in the right CPA.
CPA arachnoid cyst

Would you attribute her positional downbeat nystagmus:
– to BPPV (anterior or apogeotropic posterior) or
– to arachnoid cyst in contact with 8th cranial nerve and right cerebellar lobe?

Do you send every positional down-beat nystagmus to MRI?
What’s the significance of asymmetric smooth pursuit gain?
According to Timothy C. Hain asymmetric smooth pursuit gain is because of:
– Acute parietal lobe disorder
– Acute frontal lobe disorder
– Superimposed nystagmus
– Lesion of pontine nuclei

Atypical BPPV – unidirectional Ny of horizontal canal BPPV

I would like to present you a patient with a classical clinical picture of BPPV. Positioning tests show horizontal nystagmus to the left.
Canalolithiasis and cupulolithiasis of the horizontal canal is not so rare. Canalolithiasis, which is a more common form of lateral BPPV presents with geotropic nystagmus, while cupulolithiasis with ageotropic nystagmus. But in our patient, we see strong ageotropic nystagmus on the right side, on the back also to the left and on the left side we see weak and short geotropic nystagmus. In literature, this rare BPPV type is called Direction-fixed paroxysmal nystagmus lateral canal benign paroxysmal positioning vertigo.

Vannucchi 2011. describes a patient with ageotropic form of lateral canal BPPV, in whom during the treatment, nystagmus changed to geotropic at healthy side, but continuing with treatment in becomes completely geotropic form which is easier to treat.

Califano 2013. describes five patients with an unusual form of lateral canal BPPV. Within these patients, the ageotropic nystagmus was on the affected side, while on the healthy side it was geotropic. The authors have named this rare form of BPPV – Direction-fixed paroxysmal nystagmus lateral canal benign paroxysmal positioning vertigo.

Both works offer the following explanation: it’s canalolithiasis of the horizontal canal, where the deposit of otoconia simultaneously in ampular and the distal part of the horizontal semicircular canal, and in the supine position with the affected side ampullofugal flow endolymph consequence of gravity to deposit otoconia in ampullar part of the semicircular canals, while the smaller otoconia deposit in the distal part is not moving; and when turning on the healthy side ampullofugal flow of endolympha is explained by the action of gravity on the distal, non-ampular deposit otoconia. Switching of one sided nystagmus to a both sided geotropic happens by otoconias from ampullar part of the canal cross to the last non-ampular part of the canal.

In the above-described patient with direction fixed lateral canal BPPV, I did a BBQ maneuver to the right with shaking and Ny transferred to a geotropic at both sides, actualy it was strong geotropic on the right side, and very weak, barely noticeable geotropic on the left side. After that, Gufoni maneuver toward a healthy side was done three times. Over the next two days, the patient had less severe dizziness when turning in bed, and then is completely without any dizziness for the next five days, but than after one bending over she felt very strong vertigo and came to me. This time she had geotropic nystagmus on the right side and a barely noticeable geotropic on the left. Positioning the patient from sitting to lying produced Ny to the right. Gufoni to the left was performed three times. Thereafter, the patient is completely without any vertigo and dizziness.

Downbeat Ny at posterior canal BPPV

Here we present a patient with a classic clinical picture of BPPV. She experienced the same type of positional vertigo a year ago, which spontaneously passed after a couple of weeks.

Dix-Hallpike test to the left: a strong sense of vertigo, and after a long latency, a barely noticeable Ny which suits the left posterior canal (cw). I immediately perform Epley maneuver for the left ear, but in the second position, appears strong Ny, characteristic for canalolithiasis of the right posterior. Maneuver completed and control Dix-Hallpike test was completely bilateraly negative.

The next day the patient returns with worsening of symptoms and even stronger positional vertigo. DH to the right causes a very intense downbeat Ny, while on the left it has also a torsional component in ccw direction. Everything suggests canalolithiasis of the right anterior canal (Ny down and with torsional component to the rightside). However, it sounds unlikely for someone who had a canalolithiasis of the left posterior a day before to return the next day with canalolithiasis counterpart on the opposite side. So since we know that the canalolithiasis of the left posterior is in question, we ask ourselves how is it possible to manifest with downebeathing nystagmus?! However, I am not the first one to encounter this phenomenon:

Posterior Semicircular Canal Benign Paroxysmal Positional Vertigo Presenting with Torsional Downbeating Nystagmus: An Apogeotropic Variant

Anterior canal BPPV and apogeotropic posterior canal BPPV: two rare forms of vertical canalolithiasis

The authors of these two works explain this phenomenon by stating that the otoliths got stuck in the ampular arm of the canal.

I treated this patient with an Epley maneuver for the left ear, and repeated it several times. The patient was much better, but strange feeling in the head didn’t resolved; somthing like “dizziness”, but without any positional vertigo. The patient performed exercises by Brandt-Daroff every day and she complained on discomfort when repeating the exercise on the right side. After two weeks, the patient comes in with a worsening of symptoms; a strong vertigo while going to bed the nightbefore, but sincethan constant dizziness disapeared. Now the DH test revealed characteristic nystagmus for the posterior left semicircular canal. Only one Epley maneuver was enough to completely resolve symptoms of vertigo and dizziness and since than she is cimpletely fine.

After this, how do we know whether the patient has the canalolithiasis of the anterior canal, or an apogeotropic form of the canalolithiasis of the posterior canal? In the aforementioned papers one difference was suggested: canalolithiasis of the anterior canal is downbeat component of the nystagmus is dominant where torsional component is absent or weak, since at the ageotropic form of posterior canal BPPV the torsional component of the nystagmus is more pronounced. In our case this remark wasn’t obvious. Anyhow, it’s important when you see positional downbeat nystagmus to distinguish benign lesion such BPPV from much more serious central lesion, since we know that such lesions could present with positional downbeating nystagmus!
After discard the possibility of central vestibular lesion, you can switch to repositioning maneuvers. I recommend to try with “deep head hanging” repositioning maneuver suggested for both anterior canals; if not successfull than proceed with Epley. The aim is to reposition dislodged otoconia back to utriculus and to resolve patient from vertigo.

Positional downbeating Ny in patient with instability as dominant symptom

Three weeks ago 75year old woman wakes up with slight instability and nausea. Hour later when she tried to lay down something strongly pushes her to bad, strong vertigo with nausea starts. The vertigo didn’t last long (very restrictive movements) but she had very pronounced instability and strong nausea for the whole day. Since that day she is very unstable but walks by herself. She mentioned that she had also one episode of worsening of her instability when she tried to get up.
This patient has artificial valve and takes anticoagulant therapy; she has hypertension, hyperlipidemia and diabetes and tremor of arms.

Neurootological exam: smooth pursuit, saccadic movements, OKN functions ok. Cerebellar exam ok (tremor of hands)
Bitherlmal caloric test: 17% paresis of left side

VHIT: VOR gain is bilaterally reduced 0.74 with predominantly covert saccades
reduction in VOR gain can be explained by her age

Dix-Hallpike: very strong downbeating nystagmus, stronger on the right side !

Deep head hanging maneuver was performed twice with no improvement at all.
Epley maneuver for the right ear was performed, and after each maneuver, Ny was lessen. Finaly after third Epley Ny disappeared; control DH test at both side was negative. Patient felt better but left my office still unstable.
From day after she has no vestibular problems, almost everything returned to normal. She walks much stable but not as before (she’s afraid).
Brain MRI shows two lacunar ischemic lesions subcorticaly.