Disclosures RECOGNITION AND MANAGEMENT Research : NIH, Great Lakes - PowerPoint PPT Presentation

2/15/2019 FUNCTIONAL MOVEMENT DISORDERS: Disclosures RECOGNITION AND MANAGEMENT • Research : NIH, Great Lakes Neurotechnologies, and the Michael J Fox Foundation • Consultant/scientific advisory board : Abbvie, Neuroderm, Impax, Acadia, Acorda, Sunovion, Lundbeck, Osmotica Pharmaceutical, and USWorldMeds 52 nd Annual Recent Advances • Honoraria : USWorldMeds, Lundbeck, Acadia, Sunovion, in Neurology February 4-16, 2019 the American Academy of Neurology, and the Movement IUCSF, San Francisco Disorders Society • Royalties : Lippincott Williams & Wilkins, Cambridge University Press, and Springer JM Charcot, 1887: “Une leçon clinique à la Salpêtrière“ (André Brouilet ,1887) Alberto J. Espay, MD, MSc Professor of Neurology Director and Endowed Chair James J. and Joan A. Gardner Family Center for Parkinson’s Disease and Movement Disorders University of Cincinnati Academic Health Center Functional disorders: general features Outline  Movements are inconsistent and • Phenotype and representative cases incongruent with organic disorders • Primacy of examination features  A psychological causation is rarely overt • De-emphasis on historical and psychiatric  1.5% to 26% of all patients admitted to features a neurological service • Bizarre presentations: not enough for  Age of onset = 27-50 years (confirmed diagnosis cases in 80s) • Diagnosis: Importance of examination  Women are most often involved (3-4:1) features • Treatment Morgante, Edwards and Espay. Continuum (Minneap Minn). 2013 Oct;19(5 Movement Disorders):1383-96.

2/15/2019 Historical clues suggesting What’s the relative frequency of FMD? functional movement disorders N= 1245 FMD patients (8 specialized centers) Supportive but neither sufficient nor necessary Abrupt onset Tremor 37.5%* Dystonia 29.3 Maximum deficits at onset (not always) Myoclonus 11.7 Spontaneous remissions Gait Disorders 5.7%** Spread to multiple non-anatomically adjacent sites Parkinsonism 4.8% Paroxysmal symptoms (generally nonkinesigenic) Tic 2.3% Multiple somatizations/undiagnosed conditions Others 5.1% Previous history of minor injury * Upper (84%) > lower limbs (24%) > generalized (20%) ** If associated to other PMD = 42.3% Lang, 2006 Modified from Gupta & Lang, Curr Op Neur, 2009 Signs supporting functional Required for clinically definite PMD movement disorders INCONSISTENCY Hoover sign Hip extension normal with contralateral hip • Variability of phenomenology and/or severity over time flexion against (frequency, amplitude, direction/distribution of movement) resistance • Disproportionate disability to the extent of objective motor deficits Hip extension • Suppression or clear change in phenomenology with complex tasks weak Tubular vision defect • Magnification of the disability when attention is focused on the affected body part INCONGRUENCE Drift without pronation of the weak arm Movement is incongruous with the presentation, progression, and disability associated with known organic movement disorders SUGGESTIBILITY Other signs: False (give-away) weakness Amelioration or worsening with application of non physiologic interventions Non-anatomical sensory loss Organic drift Functional drift (somatic trigger points, tuning fork, electrotherapy) or placebo. with pronation without pronation Espay et al, JAMA Neurol 2018

2/15/2019 Required for clinically definite psychogenic Optional for clinically definite psychogenic movement disorders movement disorders (if present: diagnostic) Demands extensive experience on Demands adequate rapport with the organic disorders* patient and non-judgmental sharing of the outcome of suggestibility* * A rule that applies to the approach to all psychogenic INCONGRUENCE movement disorders –and would ensure initial steps in treatment Movement is incongruous with the presentation, progression, and disability associated with known organic movement disorders SUGGESTIBILITY Amelioration or worsening with application of non physiologic interventions * Implying, among other things, that a psychiatrist cannot be relied (somatic trigger points, tuning fork, electrotherapy) or placebo. upon to make or confirm the diagnosis The diagnosis is ”clinically Fahn-Williams Criteria for PMD definite” if these neurological (Adv Neur, 1988) features are all present* DOCUMENTED Remittance with suggestion, physiotherapy, psychotherapy, placebo, ‘while unobserved’. CLINICALLY ESTABLISHED Inconsistent over time/incongruent with clinical conditions. other manifestations: other ‘false’ signs, multiple somatizations, obvious psychiatric disturbance PROBABLE “Bizarre” alone does not suffice Inconsistent/incongruent - no other features POSSIBLE “Stressors” alone do not suffice obvious emotional disturbance Pitfalls for probable/possible categories of certainty o “Possible” or “probable” PMD criteria overlap with many organic movement disorders with emotional disturbances

2/15/2019 Functional tremor Functional tremor: variability, suppressibility, and entrainability Clinically definite if all Clinically definite if all present present 1. Entrainment or full 1. Entrainment or full suppressibility suppressibility 2. Distractibility 2. Distractibility 3. Tonic coactivation 3. Tonic coactivation at tremor onset at tremor onset 4. Pause of tremor 4. Pause of tremor during contralateral during contralateral ballistic movements ballistic movements 5. Variability in 5. Variability in frequency, axis, and/or frequency, axis, and/or distribution distribution SUPPRESSIBILITY and/or ENTRAINMENT Key features shown Change of the original tremor frequency to match the frequency of a Variability of the tremor frequency both at the native rate and after task repetitive task performed in another limb (e.g. tapping) or side-to-side performance, which matches the frequency of a repetitive task performed in tongue movements. another limb (entrainability) Functional dystonia (Fixed dystonia) Functional facial dystonia Clinical definite if all present 1. Rapid onset* 2. Fixed dystonia at rest 3. Variable resistance to manipulation and/or distractibility (or absence when unobserved) The most common pattern: tonic, sustained, lateral, and/or downward * Only phenotype for which protrusion of one side of the lower a historical feature must be present lip with ipsilateral jaw deviation (84.3%). Ipsi- or contralateral blepharospasm Typical foot pattern and excessive platysma contraction occurred in isolation or combined with Most common pattern of functional foot dystonia: fixed foot inversion and the fixed jaw dystonia (60.7%). plantar flexion Fasano et al, MDJ 2012

2/15/2019 Functional dystonia Propriospinal myoclonus Tonic contraction of the fixed posturing of hand mouth with jaw deviation fixed posturing of foot Clinical definite if all present 1. Variability in duration and/or distribution of jerks or their latency 2. Entrainment or full suppressibility 3. Distractibility 1. Rapid onset Clinical definite if all 2. Fixed dystonia at present Courtesy: Dr. Francesca Morgante rest • Axial jerks are worse when supine Recommended : Surface EMG to 3. Variable resistance assess variability and EMG-EEG to manipulation and/or • Usually trunk flexion, some with averaging for Bereitschaftspotential flexion of hips and knees distractibility (or absence when unobserved) Espay et al, JAMA Neurol 2018 Functional Parkinsonism Functional parkinsonism with dystonia: the common signs  Parkinsonian features are inconsistent and incongruent with organic forms of parkinsonism Clinically definite if all Signs present  A psychological causation is rarely Deliberate overt 1. Marked slowness on slowness examined manual  Excessive, deliberate slowness Variable tasks discordant with discordant with casual manual tasks resistance to casual manual tasks passive Pseudo-rigidity: resistance varies  (e.g., buttoning, tying manipulation with varying force of passive shoe laces) Preservation of manipulation 2. Variable resistance the pincer against passive  Associated fixed dystonia common function movements without “Huffing and  Women are relatively less affected (2:1 cogwheel rigidity puffing” with ratio) compared with other functional tasks and upon disorders (3-4:1) standing Jankovic J. J Neurol Neurosurg Psychiatry. 2011 Dec;82(12):1300-3. Hallett M. J Neurol Sci. 2011 Nov 15;310(1-2):163-5

2/15/2019 Caveat – Bizarre gait does not equal Most common functional gait: knee-buckling pattern functional gait Chorea acanthocytosis (Rodrigues et al, Mov Disord. 2008 Oct 30;23(14):2090-3) Electrophysiologic demonstration of tremor entrainment Bizarre gait ≠ functional gait in a patient with psychogenic tremor Status cataplecticus (“limp man syndrome”) Morgante, Edwards, Espay, Continuum Neur 2013 (Simon et al, Mov Disord. 2004 Jul;19(7):838-40)