Persistent Postural Perceptual Dizziness (PPPD)

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Disease Entity

Persistent postural perceptual dizziness (PPPD) is a disorder caused by mismatch between visual and vestibular input and processing mechanisms. The symptoms of PPPD include dizziness and postural instability exacerbated by movement, geometric patterns, or lighting in the environment. PPPD is associated with a non-environmental vertigo and perceived unsteadiness. Although PPPD is often preceded by peripheral vestibular disorders (e.g., benign paroxysmal positional vertigo (BPPV), Meniere’s disease, or vestibular neuritis), the disorder has additional features which suggest central visual to vestibular processing mismatch[1].

History

German neurologists described syndromes involving dizziness provoked by moving visual stimuli in the 19th century. One such condition was called Platzschwindel meaning “vertigo in a plaza or square.” Patients experienced anxiety in situations which provoked their dizziness and therefore developed avoidant behaviors[1].

In 1986, Brandt and Dieterich described phobic postural vertigo (PPV) which included visual stimulus-provoked vertigo, phobic symptoms, and obsessive-compulsive personality traits[2]. In 1995, Bronstein described visual variant vertigo (VVV) in patients with a history of central or peripheral vestibular disease who developed vertigo associated with visual stimuli even after recovery of vestibular function [3]. Joseph Staab described chronic subjective dizziness (CSD) in 2007 as dizziness associated with moving visual stimuli in patients without a history of vestibular disease or psychiatric illness[4]. The diagnostic criteria for PPPD were published in 2014 and were first included in the International Classification of Disease (ICD) in 2017[5].

Epidemiology

As PPPD was only recently included in the ICD, its true incidence is difficult to determine. However, prospective studies which followed patients with vestibular disorders (BPPV, vestibular neuritis, Meniere’s disease) for 3-12 months found that approximately 25% went on to develop PPPD or VVV after resolution of their vestibular dysfunction[6][7][8]. PPPD effects women 4 times more often than men[9].

Risk Factors

PPPD occurs in patients with preexisting vestibular disorders (BPPV, vestibular neuritis, vestibular migraine, etc.). Anxiety, depression, and migraine are conditions frequently associated with PPPD[5].

Diagnosis

There are five clinical criteria which must all be met for a diagnosis of PPPD as described in the International Classification of Vestibular Disorders[10]:

1.     Dizziness for more than half of the days in a 90-day period of varying severity and lasting for hours

2.     Vestibular symptoms worsened by standing, motion, and visualization of complex patterns or moving stimuli

3.     History of another cause of vestibular dysfunction such as BPPV, brain injury, or Meniere’s disease which precipitated PPPD

4.     Symptoms result in distress or impairment in daily activities

5.     Symptoms cannot be better explained by another diagnosis such as orthostatic hypotension or other vestibular disorders

The severity of symptoms and functional impairment can be assessed using the Niigata PPPD questionnaire[11]. In addition to the patient’s history, there are several questionnaire instruments that may be useful diagnostic tools for PPPD. There is no biomarker or neuro-imaging finding in PPPD.

Differential Diagnosis

Phobic postural vertigo[2]

PPV is characterized by subjective non-spinning dizziness and instability that is provoked by visual stimuli and is associated with phobic symptoms and obsessive-compulsive personality traits. Vertigo symptoms of PPV last for seconds at a time rather than for hours as in PPPD. Additionally, PPV may develop following a vestibular disorder or a period of emotional or physical stress. The association with obsessive-compulsive personality traits, the short duration of vertigo symptoms, and the development of PPV following times of stress differentiates the condition from PPPD.

Visual variant vertigo[3]

VVV is dizziness that is provoked by moving or complex visual stimuli in patients with a history of central or peripheral vestibular disease. It differs from PPPD in that patients with VVV do not experience postural instability and unsteadiness. Acetazolamide has been found to provide relief of vertigo symptoms in some patients with visual variant vertigo and further investigation into its potential use as a treatment for PPPD is warranted[12].

Chronic subjective dizziness[4]

CSD is persistent dizziness and oversensitivity to movement of oneself or the environment in patients without an active vestibular disorder. The association between CSD and anxiety disorders is well-described. Balance is usually preserved in CSD whereas PPPV is often associated with unsteadiness.

There is considerable overlap however in these various syndromes.

Management

General treatment

Patients should be reassured that PPPD is relatively common and potentially treatable. Management is multifactorial and involves both vestibular rehabilitation and treatment of underlying psychiatric co-morbidities as it is a functional disorder. In practice, patients with the condition are prescribed a selective serotonin re-uptake inhibitor or serotonin norepinephrine re-uptake inhibitor for one year duration. The effectiveness of these antidepressant medications in treating PPPD is not limited to patients with co-morbid depression and anxiety as patients without psychiatric illness have benefitted from their use [13].

Vestibular rehabilitation is recommended to decrease the vestibular response to moving and complex visual stimuli. Patients can be provided with information regarding exercises to practice on their own, but referral to a vestibular therapist is recommended. These exercises should gradually be incorporated as tolerated. Cognitive behavioral therapy has shown promise as an adjuvant treatment to vestibular rehabilitation[13][14]. It is especially useful for management of the fear of falling and subsequent avoidance behaviors experienced by many patients with PPPD. A study published in 2021 found that patients experienced significant reduction of PPPD symptoms when using virtual reality therapy in addition to vestibular rehabilitation[15].

References

Popkirov, S., Staab, J. P., & Stone, J. (2018). Persistent postural-perceptual dizziness (PPPD): a common, characteristic and treatable cause of chronic dizziness. Practical Neurology, 18(1), 5–13. https://doi.org/10.1136/practneurol-2017-001809.

Brandt, T. H., & Dieterich, M. (1986). Phobischer attacken-schwankschwindel, ein neues syndrom. Münch Med Wochenschr, 128, 247-250.

Bronstein, A. M. (1995). Visual vertigo syndrome: clinical and posturography findings. Journal of Neurology, Neurosurgery & Psychiatry, 59(5), 472-476.

Staab, J. P., & Ruckenstein, M. J. (2007). Expanding the differential diagnosis of chronic dizziness. Archives of Otolaryngology–Head & Neck Surgery, 133(2), 170. https://doi.org/10.1001/archotol.133.2.170

Knight, B., Bermudez, F., Shermetaro, C. (2022). Persistent Postural-Perceptual Dizziness. In StatPearls. StatPearls Publishing.

Best, C., Tschan, R., Eckhardt-Henn, A., & Dieterich, M. (2009). Who is at risk for ongoing dizziness and psychological strain after a vestibular disorder?. Neuroscience, 164(4), 1579–1587. https://doi.org/10.1016/j.neuroscience.2009.09.034

Godemann, F., Siefert, K., Hantschke-Brüggemann, M., Neu, P., Seidl, R., & Ströhle, A. (2005). What accounts for vertigo one year after neuritis vestibularis - anxiety or a dysfunctional vestibular organ?. Journal of Psychiatric Research, 39(5), 529–534. https://doi.org/10.1016/j.jpsychires.2004.12.006

Heinrichs, N., Edler, C., Eskens, S., Mielczarek, M. M., & Moschner, C. (2007). Predicting continued dizziness after an acute peripheral vestibular disorder. Psychosomatic Medicine, 69(7), 700–707. https://doi.org/10.1097/PSY.0b013e318151a4dd

Staab J. P. (2020). Persistent Postural-Perceptual Dizziness. Seminars in Neurology, 40(1), 130–137. https://doi.org/10.1055/s-0039-3402736

Staab, J. P., Eckhardt-Henn, A., Horii, A., Jacob, R., Strupp, M., Brandt, T., & Bronstein, A. (2017). Diagnostic criteria for persistent postural-perceptual dizziness (PPPD): Consensus document of the committee for the Classification of Vestibular Disorders of the Bárány Society. Journal of Vestibular Research: Equilibrium & Orientation, 27(4), 191–208. https://doi.org/10.3233/VES-170622

Yagi, C., Morita, Y., Kitazawa, M., Nonomura, Y., Yamagishi, T., Ohshima, S., Izumi, S., Takahashi, K., & Horii, A. (2019). A Validated Questionnaire to Assess the Severity of Persistent Postural-Perceptual Dizziness (PPPD): The Niigata PPPD Questionnaire (NPQ). Otology & Neurotology: Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 40(7), 747–752. https://doi.org/10.1097/MAO.0000000000002325

Yu, Y. C., Xue, H., Zhang, Y. X., & Zhou, J. (2018). Cognitive Behavior Therapy as Augmentation for Sertraline in Treating Patients with Persistent Postural-Perceptual Dizziness. BioMed research international, 2018, 8518631. https://doi.org/10.1155/2018/8518631

Sluch, I. M., Elliott, M. S., Dvorak, J., Ding, K., & Farris, B. K. (2017). Acetazolamide: A New Treatment for Visual Vertigo. Neuro-Ophthalmology (Aeolus Press), 41(6), 315–320. https://doi.org/10.1080/01658107.2017.1326944

Waterston, J., Chen, L., Mahony, K., Gencarelli, J., & Stuart, G. (2021). Persistent Postural-Perceptual Dizziness: Precipitating Conditions, Co-morbidities and Treatment with Cognitive Behavioral Therapy. Frontiers in Neurology. https://doi.org/10.3389/fneur.2021.795516

Mempouo, E., Lau, K., Green, F., Bowes, C., & Ray, J. (2021). Customized vestibular rehabilitation with the addition of virtual reality based therapy in the management of persistent postural-perceptual dizziness. The Journal of Laryngology and Otology, 135(10), 887–891. https://doi.org/10.1017/S0022215121002127

  1. 1.0 1.1 Popkirov, S., Staab, J. P., & Stone, J. (2018). Persistent postural-perceptual dizziness (PPPD): a common, characteristic and treatable cause of chronic dizziness. Practical Neurology, 18(1), 5–13. https://doi.org/10.1136/practneurol-2017-001809.
  2. 2.0 2.1 Brandt, T. H., & Dieterich, M. (1986). Phobischer attacken-schwankschwindel, ein neues syndrom. Münch Med Wochenschr, 128, 247-250.
  3. 3.0 3.1 Bronstein, A. M. (1995). Visual vertigo syndrome: clinical and posturography findings. Journal of Neurology, Neurosurgery & Psychiatry, 59(5), 472-476.
  4. 4.0 4.1 Staab, J. P., & Ruckenstein, M. J. (2007). Expanding the differential diagnosis of chronic dizziness. Archives of Otolaryngology–Head & Neck Surgery, 133(2), 170. https://doi.org/10.1001/archotol.133.2.170
  5. 5.0 5.1 Knight, B., Bermudez, F., Shermetaro, C. (2022). Persistent Postural-Perceptual Dizziness. In StatPearls. StatPearls Publishing.
  6. Heinrichs, N., Edler, C., Eskens, S., Mielczarek, M. M., & Moschner, C. (2007). Predicting continued dizziness after an acute peripheral vestibular disorder. Psychosomatic Medicine, 69(7), 700–707. https://doi.org/10.1097/PSY.0b013e318151a4dd
  7. Godemann, F., Siefert, K., Hantschke-Brüggemann, M., Neu, P., Seidl, R., & Ströhle, A. (2005). What accounts for vertigo one year after neuritis vestibularis - anxiety or a dysfunctional vestibular organ?. Journal of Psychiatric Research, 39(5), 529–534. https://doi.org/10.1016/j.jpsychires.2004.12.006
  8. Best, C., Tschan, R., Eckhardt-Henn, A., & Dieterich, M. (2009). Who is at risk for ongoing dizziness and psychological strain after a vestibular disorder?. Neuroscience, 164(4), 1579–1587. https://doi.org/10.1016/j.neuroscience.2009.09.034
  9. Staab J. P. (2020). Persistent Postural-Perceptual Dizziness. Seminars in neurology, 40(1), 130–137. https://doi.org/10.1055/s-0039-3402736
  10. Staab, J. P., Eckhardt-Henn, A., Horii, A., Jacob, R., Strupp, M., Brandt, T., & Bronstein, A. (2017). Diagnostic criteria for persistent postural-perceptual dizziness (PPPD): Consensus document of the committee for the Classification of Vestibular Disorders of the Bárány Society. Journal of Vestibular Research: Equilibrium & Orientation, 27(4), 191–208. https://doi.org/10.3233/VES-170622
  11. Yagi, C., Morita, Y., Kitazawa, M., Nonomura, Y., Yamagishi, T., Ohshima, S., Izumi, S., Takahashi, K., & Horii, A. (2019). A Validated Questionnaire to Assess the Severity of Persistent Postural-Perceptual Dizziness (PPPD): The Niigata PPPD Questionnaire (NPQ). Otology & Neurotology: Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 40(7), 747–752. https://doi.org/10.1097/MAO.0000000000002325
  12. Sluch, I. M., Elliott, M. S., Dvorak, J., Ding, K., & Farris, B. K. (2017). Acetazolamide: A New Treatment for Visual Vertigo. Neuro-Ophthalmology (Aeolus Press), 41(6), 315–320. https://doi.org/10.1080/01658107.2017.1326944
  13. 13.0 13.1 Yu, Y. C., Xue, H., Zhang, Y. X., & Zhou, J. (2018). Cognitive Behavior Therapy as Augmentation for Sertraline in Treating Patients with Persistent Postural-Perceptual Dizziness. BioMed research international, 2018, 8518631. https://doi.org/10.1155/2018/8518631
  14. Waterston, J., Chen, L., Mahony, K., Gencarelli, J., & Stuart, G. (2021). Persistent Postural-Perceptual Dizziness: Precipitating Conditions, Co-morbidities and Treatment with Cognitive Behavioral Therapy. Frontiers in Neurology. https://doi.org/10.3389/fneur.2021.795516
  15. Mempouo, E., Lau, K., Green, F., Bowes, C., & Ray, J. (2021). Customized vestibular rehabilitation with the addition of virtual reality based therapy in the management of persistent postural-perceptual dizziness. The Journal of Laryngology and Otology, 135(10), 887–891. https://doi.org/10.1017/S0022215121002127
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