Persistent Postural-Perceptual Dizziness (PPPD) and Other Chronic Vestibular and Balance Problems

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1 Minnesota Physical Therapy Association Annual Conference Persistent Postural-Perceptual Dizziness (PPPD) and Other Chronic Vestibular and Balance Problems Jeffrey P. Staab, MD, MS Professor of Psychiatry Consultant, Departments of Psychiatry and Psychology and Otorhinolaryngology Head and Neck Surgery 21 April 2018 permission. 1

2 Disclosure Commercial support None Grant support National Institutes of Health Mayo Clinic Off-label medication use Serotonergic antidepressants for persistent postural-perceptual dizziness (PPPD) permission. 2

3 Overview 1. Historical background 2. Classification of Vestibular Disorders 3. PPPD -- symptoms, psychology, physiology, brain function 4. Other chronic vestibular and balance problems 5. Treatment strategies Patients want an explanation for their suffering. They do not want to be laughed at, or worse, considered to be insane. - Carl Westphal, 1871 permission. 3

4 Die Agoraphobie (fear of marketplace) Patients find it impossible to cross open squares and walk along certain streets. Fear restricts their mobility, [but] they insist that they are not aware of any reasons for their anxiety. It seems to arise as an alien force as soon as a square is crossed or approached. With the anxiety, as part of one process, occurs the thought of not being able to cross and a perception of an enormous expanse of space. Carl Westphal, 1871 Kuch and Swinson, Can J Psychiatry, 1992 permission. 4

5 From the 19 th to the 20 th century Platzschwindel (vertigo in plaza) 1870 Die Agoraphobia (fear of marketplace) 1871 Platzangst (fear in plaza) th century 20 th century Habituation by systematic exposure 1873 Chronic vestibulopathy Otologic triggers Anxious predisposition 1898 Agoraphobia Psychogenic dizziness Balaban and Jacob, J Anxiety Disorders, 2001 permission. 5

6 From Platzschwindel to PPPD 147 years 1870 Platzschwindel (Benedikt) 1871 Die Agoraphobie (Westphal) 1872 Platzangst (Cordes) Psychogenic dizziness, Chronic vestibulopathy Supermarket syndrome, space phobia, etc Phobic postural vertigo (Brandt & Dieterich) 1993 Space-motion discomfort (Jacob, et al.) 1995 Visual vertigo (Bronstein) 2004 Chronic subjective dizziness (Staab & Ruckenstein) 2017 Persistent postural-perceptual dizziness (Bárány Society) permission. 6

7 The dichotomous hierarchy of the 20 th century Faulty logic of Medically Unexplained When does B = Not A? A A B B U physiologic vs. aphysiologic real vs. not real organic vs. functional It is impossible to define anything solely by what it is not! permission. 7

cellular) Metabolic Functional Dieterich, Staab, Brandt, Functional (psychogenic)

8 20 th Century B = Not A Explained 21 st Century A, B, C (and U) may exist in any combination and develop in any order Unexplained rule out Structural (including cellular) Metabolic Functional Dieterich, Staab, Brandt, Functional (psychogenic) dizziness in Hallett et al., eds., Functional Neurologic Disorders, 2017 rule in permission. 8

9 What is a functional disorder? [A] functional disorder [is] so called because the symptoms seem arise from a change in the mode of action of the organ, unconnected with any perceptible alteration of structure. Observations on Mental Derangement: Being an Application of the Principles of Phrenology to the Elucidation of the Causes, Symptoms, Nature, and Treatment of Insanity. Andrew Combe, MD, 1831 Scottish physician and President, Edinburgh Phrenological Society In the International Classification of Vestibular Disorders (ICVD), PPPD is a chronic functional vestibular syndrome. permission. 9

10 Symptoms PPV SMD VV CSD Dizziness when upright Unsteadiness fluctuating constant Motion of self Illusory Increased awareness when exposed constant Increased sensitivity Motion in environment Visual complexity Precision visual tasks Vestibular deficits trigger / possible Anxiety comorbid Depression comorbid Obsessive personality risk factor permission. 10

11 ICVD definition of PPPD A. Dizziness, unsteadiness, or non-spinning vertigo present on most days for 3 months or more. Symptoms must be present for prolonged (hours-long) periods, but need not be continuous throughout the entire day. B. Persistent symptoms are present without specific provocation, but are exacerbated by three factors: Upright posture, active or passive motion, and exposure to moving visual stimuli or complex visual patterns. C. The disorder is triggered by events that cause vertigo, unsteadiness, dizziness, or problems with balance: Acute, episodic, or chronic vestibular syndromes, other neurologic or medical illnesses, and psychological distress. D. Symptoms cause significant distress or functional impairment. E. Symptoms are not better attributed to another disease or disorder. Staab, et al., J Vestib Res, 2017; WHO ICD-11, apps.who.int/classifications/icd11/ permission. 11

Illness Profiles: Vertigo, Unsteadiness, Dizziness 100 PPPD 100 BPPV 80 80 60 60 40 40 20 20 0 secs mins hrs days wks cont 0 secs mins hrs

12 Illness Profiles: Vertigo, Unsteadiness, Dizziness 100 PPPD 100 BPPV secs mins hrs days wks cont 0 secs mins hrs days wks cont 100 Menière s disease 100 Migraine secs mins hrs days wks cont 0 secs mins hrs days wks cont permission. 12

13 Sensitivity CSD with/without BPPV, VM, Menière s (N=410) Sensitivity: 85.9% Specificity: 91.4% Persistent dizziness Persistent unsteadiness No active vertigo Motion sensitivity Visual complexity Precision tasks o Anxiety o Depression Specificity permission. 13

14 Pathophysiologic mechanisms of PPPD Predisposing factors Precipitating factors Promoting factors Transition from acute to chronic dizziness part of one process Perpetuating factors Postural change Visual dependence Reduced cortical integration of space-motion processes Supported by research data permission. 14

Psychology #1: Predisposing factor (CSD & PPPD) NEO-PI neurotic, introverted temperament CSD Comparison Group (Neurotologic + anxiety disorder) Absent 33% Present 67% Absent

15 Psychology #1: Predisposing factor (CSD & PPPD) NEO-PI neurotic, introverted temperament CSD Comparison Group (Neurotologic + anxiety disorder) Absent 33% Present 67% Absent 75% Present 25% OR = 6.0, p<0.01 Staab et al., J Psychosom Res, 2013; Tschan, et al., J Neurol, 2011; Yan, et al., Int J Audiol, 2016; Chiarella, et al., JVR, 2016 permission. 15

16 Responses to sound-evoked vestibular stimulation Brain activity correlated with neuroticism: A. Cerebellum B. Pons C. Visual cortex (V2) D. Vestibular cortex (Supramarginal gyrus) Indovina, et al., J Psychosom Res, 2014 permission. 16

17 Riccelli, et al., Hum Brain Map, 2017 Calm Persons Nervous Persons Calm Persons Nervous Persons permission. 17

18 Precipitants (from studies of CSD) Anxiety - Panic attacks 15% - Generalized anxiety 15% Vestibular syndromes - with/without secondary anxiety 25% Neurologic Illnesses - migraine 20% - traumatic brain injury 15% - autonomic dysregulation 7% Other Medical Conditions - dysrhythmias, adverse drug reactions 3% N=345 Staab & Ruckenstein, Arch Oto-HNS, 2007 permission. 18

19 Psychology #2: promoting effect of anxiety Acute vestibular event + acute anxiety chronic dizziness Acute vestibular neuritis (N=75) Recovered (N=53) Acute anxiety (85%) Chronic dizziness (N=22) Vestibular tests Compensated (N=20) Non-compensated (N=2) 1 year Godemann, et al., J Psychiatric Res, 2005; Heinrichs, et al., Psychol Med, 2007; Best, et al., Neuroscience, 2009; Mahoney, et al., Am J Otolaryngol, 2013; Cousins, et al., Ann Clin Trans Neuro, 2017 permission. 19

20 Physiology functional shift #1: high-risk postural control Stiffer postural control due to co-contraction of ankle musculature Floor level 3.2m platform Detected in: Normal individuals (at height) Patients with: Fear of heights Fear of falling CSD and PPV Normal CSD CSD Davis JR, et al., J Neurophysiol, 2011; Ödman & Maire, Acta Oto-Laryngol, 2008 permission. 20

21 Mean SOT score Posturography in PPPD a,b a,b a,c a,c a,c a,c C1 C2 C3 C4 C5 C6 CS PPPD Recovered Control Sohsten EM, et al., J Vest Res, 2015 permission. 21

22 Physiology functional shift #2: Visual dependence Prospective follow-up: Acute vestibular neuritis 10 weeks and 6 months Primary predictors of chronic dizziness: Visual dependence Autonomic arousal Not structural variables (caloric deficit) Cousins, et al., PLoS 2014; Cousins, et al., Ann Clin Trans Neuro, 2017 permission. 22

occipital cortex Anterior insula/inferior

Hippocampus functional connectivity in PPPD

23 Brain Function #2: fmri sound-evoked vestibular stimulation in patients with PPPD vs. healthy controls Posterior insula/superior temporal gyrus (PIVC) Middle occipital cortex Anterior insula/inferior frontal gyrus Anterior cingulate cortex Hippocampus functional connectivity in PPPD regional activity in PPPD Indovina, et al., Front Behav Neuro, 2015 permission. 23

24 Pathophysiologic Processes in the Development of PPPD Precipitants 1. Vestibular crisis 2. Medical event Predisposing Factors 1. Neurotic 3. Acute anxiety temperament Failure of Readaptation 2. Pre-existing anxiety Behavioral Comorbidity 1. Anxiety disorders 2. Depression High-risk postural control strategies; Visual dependence Acute Adaptation 1. Visual-somatosensory dependence 2. High-risk postural control strategies 3. Environmental vigilance Recovery 1. Neurotologic 2. Medical 3. Behavioral Staab, Behavioural Neuro-otology in Bronstein (ed), Oxford Textbook of Vertigo, 2013 Provoking Factors 1. Upright posture 2. Motion of self Active Passive 3. Visual stimuli Complex Moving permission. 24

Neurologic Other medical Acute Adaptation

Stiffer gait and stance Visual dependence

introvert) Pre-existing anxiety 3 sessions of

Medical Psychological 20 15 10 5 0 10 0-100

Depression T 20 High-risk 0 Home VBRT postural

(pathological) Failure to Readapt 0 2 4 8 12

25 Pathophysiologic Processes of PPPD Precipitants Vestibular Psychological Neurologic Other medical Acute Adaptation High-risk postural control (physiological) Stiffer gait and stance Visual dependence Predisposing Factors Temperament (neurotic introvert) Pre-existing anxiety 3 sessions of CBT may abort illness Recovery Neuro-otologic Medical Psychological Psychiatric Comorbidity Anxiety Phobia Depression T 20 High-risk 0 Home VBRT postural reduces control dizziness (pathological) Failure to Readapt LOCF Provoking Factors Upright posture Motion Visual stimuli Staab JP, Continuum, 2012 permission. 25

26 Treatment Education & Physical Therapy Education about the disorder Normal and high-risk postural control strategies Failure to readapt (persistence of shift in functioning) Vestibular habituation therapy Habituation exercises (3-6 months for PPPD) Head/body movement and visual stimuli Performed twice daily at home Additional interventions for comorbidity Gait reshaping for functional gait disorders Balance confidence/ falls prevention for fear of falling Compensation exercises for uncompensated vestibular deficits Thompson, et al., J Vest Res, 2015 permission. 26

27 Locomotion Which person is more likely to have: Slower gait? Shorter strides? Longer response time on the phone? Every step you take, every move you make, I ll be watching you. -- Every Breath You Take The Police, 1983 Carpenter, et al., Neurosci Lett, 2004; Ohno, et al., Neurosci Lett, 2004 permission. 27

28 Dizziness Handicap Inventory scores Sertraline treatment of CSD (66-84% response rate) 20 *p<0.05 **p< open trials Physical All 6 SSRIs fluoxetine, sertraline, ** 5 Functional paroxetine, (es)citalopram, fluvoxamine ** ** 2 of 6 SNRIs Emotional ovenlafaxine, 0 milnacipran LOCF PPPD-type dizziness Not mediated by anxiety, depression Weeks of Treatment * * * ** ** ** Staab et al., Laryngoscope, 2004 permission. 28

29 Treatment Psychotherapy For PPPD (from studies of CSD and PPV) Randomized controlled trials of cognitive behavior therapy (CBT) For early illness 3 sessions of CBT large and sustained benefits Uncertain for chronic illness 12 sessions of CBT initial benefit lost at follow-up For co-existing anxiety and depressive disorders Clinical experience Reductions in psychiatric symptoms Better adherence to rehabilitation Holmberg, et al., J Neurol, 2007; Mahoney, et al., Am J Otolaryn, 2013 permission. 29

30 Red Flags that it s Not PPPD Progressive symptoms slowly worsening over years Neurodegenerative disorder Peripheral neuropathy, progressive vestibular loss Cerebellar degeneration, Parkinson s disease Indistinct onset possible, but not common Early in the course of progressive neurotologic disease Generalized anxiety disorder, dysautonomias may present this way Falls gait disturbance is not part of PPPD Peripheral or central neurotologic disorder Cardiovascular or autonomic disorder Functional gait disorder Constant symptoms regardless of provocative factors Often with other physical complaints somatic symptom disorder Staab et al., J Vest Res, 2017 permission. 30

31 Conclusion PPPD Formal diagnostic criteria Barany Society ICVD (2017) Contained in WHO ICD-11 (2018) Putative pathophysiologic mechanisms (change in mode of action) Structural + psychological functional Promoted initially by anxiety-related processes Sustained used of high-risk postural control strategies Persistently high visual dependence Likely associated with reduced cortical integration Treatments Vestibular habituation SSRIs/SNRIs CBT done early Combination treatment permission. 31

32 Psychiatric vestibular disorders Five psychiatric syndromes Episodic anxiety Chronic anxiety Fear of falling Vestibular illness anxiety Depression May be the cause or consequence of vestibular symptoms May co-exist with other disorders that cause vestibular symptoms Prevalence Primary diagnosis 8-10% of tertiary neurotology patients Co-existing problem 30-50% within 5 years of 1 st symptoms permission. 32

33 Vestibular symptoms and episodic anxiety (panic attacks) A. Recurrent episodes of dizziness, unsteadiness, or vertigo that occur suddenly and spontaneously. B. Vestibular symptoms are accompanied by intense fear or apprehension and three or more of the following: Palpitations or increased heart rate Sweating Trembling or shaking Shortness of breath Chest pain or discomfort Chills or hot flushes Numbness or tingling Fear of dying C.Symptoms reach a peak within a few minutes and then resolve, but residual symptoms may linger for hours. permission. 33

34 Vestibular symptoms and chronic anxiety (generalized anxiety) A. Chronic dizziness, unsteadiness or non-spinning vertigo that may wax and wane in severity. B. Vestibular symptoms are accompanied by general apprehension or worry and three or more of the following: Muscle tension or motor restlessness Feeling of nervousness Symptoms of autonomic over-activity Difficulty concentrating Irritability Insomnia C. Symptoms occur on more days than not. May have a gradual or stuttering onset. permission. 34

35 Fear of falling (FoF) A. Marked fear or anxiety about falling or situations where falling could occur that is out of proportion to the actual risk of falling. B. Exposure to situations where falling could occur almost always provokes immediate fear or anxiety. C. Situations where falling could occur are actively avoided or endured with intense fear or anxiety. Paradoxically increases risk of actual falls or near falls. permission. 35

36 Vestibular illness anxiety 1. Conviction that a serious illness is responsible for vestibular symptoms 2. Excessive vigilance about vestibular symptoms 3. Catastrophic interpretations of vestibular symptoms 4. Excessive seeking of reassurance about vestibular symptoms repeated requests for medical tests exhaustive reviews of medical information OR Avoidance of medical evaluations of vestibular symptoms Present in 12-15% of patients regardless of vestibular diagnosis. permission. 36

37 Depression complicating a vestibular syndrome 1. Presence of an acute, episodic or chronic vestibular syndrome 2. Dysphoria or anhedonia lasting for at least two weeks in conjunction with the vestibular syndrome 3. Four or more of the following symptoms are present at the same time : Appetite change Poor concentration Sleep change Hopelessness Poor energy or fatigue Feelings of worthlessness or excessive guilt Restlessness or sluggishness Recurrent thoughts of death or suicide Non-specific dizziness may be a harbinger of depression. permission. 37

38 Detection of psychiatric morbidity Listen for clues in the history. Ask three questions: 1. Does the patient have an active neurotologic condition? 2. Does the neurotologic condition explain all of the patient s symptoms? 3. Does the patient have behavioral symptoms indicative of psychiatric morbidity? Use a validated screening tool. Dizziness Handicap Inventory (total score >25) Patient Health Questionnaire (PHQ-9) depression Generalized Anxiety Disorder Scale (GAD-7) anxiety PHQ-4 (first 2 questions of PHQ-9 & GAD-7) permission. 38

integrated balance function (e.g., posturography) Greater effect Often misinterpreted Cevette, et al.

39 Effects of psychiatric morbidity on vestibular tests Tests of basic vestibular reflexes (e.g., caloric, VEMP) Tendency for small, clinically insignificant increase in response Possibility of an isolated abnormality Tests of integrated balance function (e.g., posturography) Greater effect Often misinterpreted Cevette, et al., Otolaryn Head Neck Surg, 1995; Furman, et al., J Vest Res, 2007; Jacob, et al., JNNP, Normal Vestibular Psychiatric Conditions of Sensory Organization Test permission. 39

40 Psychiatric vestibular disorders -- Treatment Patient education Starts during the evaluation process ( Every step you take ) Psychiatric and psychological care Standard interventions for the psychiatric condition Medications SSRIs/SNRIs as first line (not for FoF) Psychotherapy Cognitive behavior therapy and others Physical therapy Restore balance confidence Counteract avoidance General conditioning permission. 40

41 Conclusion Psychiatric vestibular disorders Five syndromes Episodic anxiety Chronic anxiety Fear of falling Vestibular illness anxiety Depression May be a cause or consequence of vestibular symptoms May co-exist with other causes of vestibular symptoms Detection Awareness, self-reports, proper interpretation of tests Treatment Patient education Standard psychiatric and psychological therapies Physical therapy permission. 41

42 International Classification of Vestibular Disorders Project of the Barany Society (international neuro-otologic society) International Classification of Vestibular Disorders (ICVD)] Definitions published by the Journal of Vestibular Research 1. Vestibular symptoms 2. Vestibular migraine 3. Menière's disease 4. Benign paroxysmal positional vertigo 5. Vestibular paroxysmia 6. Bilateral vestibular loss 7. Persistent postural-perceptual dizziness 8. Others in progress Home Issues Featured Articles ICVD Bárány Society Meetings For Authors Editorial Board Subscriptions Links International Classification of VestibularDisorders (ICVD) ICVD A series of consensus documents is currently under development by the Committee for the Classification of Vestibular Disorders of the Bárány Society. Its goal is to define key vestibular symptoms as a basis for a subsequent classification of specific vestibular disorders and then to build consensus around these formalized definitions. Members of the Bárány Society are invited to contribute to the consensus process. Bárány Society Members Please make sure your address is registered with the Society The Bárány Society President invites new members to join the society. Find out more Published in the Journal of Vestibular Research in Partnership with the Bárány Society ICVD consensus process Bárány Society initiative for the establishment of the International Classification of Vestibular Disorders (ICVD) Full Text Already published Classification of vestibular symptoms: Towards an international classification of vestibular disorders Alexandre Bisdorff, Michael Von Brevern, Thomas Lempert, David E. Newman-Toker Journal of Vestibular Research, Volume 19 (2009), Numbers 1-2, pp Full Text Vestibular migraine: Diagnostic criteria Consensus document of the Bárány Society and the International Headache Society Thomas Lempert, Jes Olesen, Joseph Furman, John Waterston, Barry Seemungal, John Carey, Alexander Bisdorff, Maurizio Versino, Stefan Eversi and David Newman-Toker Journal of Vestibular Research, Volume 22 (2012), Number 4, pp Full Text Diagnostic criteria for Meniére s Disease Consensus document of the Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society Jose A. Lopez-Escamez, John Carey, Won-Ho Chung, Joel A. Goebel, Måns Magnusson, Marco Mandala, David E. Newman-Toker, Michael Strupp, Mamoru Suzuki, Franco Trabalzini and Alexandre Bisdorff Journal of Vestibular Research, Volume 25 (2015), Number 1, pp. 1-7 Full Text jvr-web.org/icvd.html Benign paroxysmal positional vertigo: Diagnostic criteria Consensus document of the Committee for the Classification of Vestibular Disorders of the Bárány Society Michael von Brevern, Pierre Bertholon, Thomas Brandt, Terry Fife, Takao Imai, Daniele Nuti and David Newman-Toker Journal of Vestibular Research, Volume 25 (2015), Numbers 3-4, pp Full Text Vestibular paroxysmia: Diagnostic criteria Consensus document of the Committee for the Classification of Vestibular Disorders of the Bárány Society Michael Strupp, Jose A. Lopez-Escamez, Ji-Soo Kim, Dominik Straumann, Joanna Jen, John Carey, Alexandre Bisdorff and Thomas Brandt Journal of Vestibular Research, Volume 26 (2016), Numbers 5-6, pp of 2 7/4/17, 10:50 AM permission. 42

ASHA Essentials of Chronic Subjective Dizziness, Fear of Falling, and Other Behavioral Complications of Vestibular Disorders

ASHA Essentials of Chronic Subjective Dizziness, Fear of Falling, and Other Behavioral Complications of Vestibular Disorders ASHA 2014 Essentials of Chronic Subjective Dizziness, Fear of Falling, and Other Behavioral Complications of Vestibular Disorders Jeffrey P. Staab, MD, MS November, 22, 2014 2013 MFMER slide-1 Disclosure