Cerebellar Ataxia in Multiple Sclerosis
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Prof Jon Marsden, School of Health Professions, University of Plymouth
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Cerebellar Ataxia in Multiple Sclerosis
- 1. Cerebellar Ataxia in Multiple Sclerosis Prof Jon Marsden School of Health Professions University of Plymouth
- 2. Cerebellar Ataxia In MS Anatomy and Physiology Signs and Symptoms Potential mechanisms of Recovery
- 3. Dysarthria Co-morbidity Sensory Dysfunction Vestibular Dysfunction Abilities Participation Sequelae Immobility 2o Pain Deconditioning & Fatigue Respiratory Distress Social Isolation Cerebellar Ataxia Dysmetria Dyssynergia Tremor Impaired balance & gait
- 4. Cerebellar Ataxia in MS: Clinical • In brainstem/cerebellar signs in clinically isolated syndromes(CIS) predicts long term disability Sastre- Garriga et al 2010 • Cerebellar Relapse at disease onset associated with increased risk of cerebellar involvement during subsequent relapses • 10% relapses are cerebellar • Cerebellar relapses associated with poor relapse recovery and earlier onset of progressive disease onset Wilikins 2017 • Motor Cerebellar Signs are the main clinical symptom in 11-33% of pw MS Wenshenker et al 1996 • Poor Prognostic indicator for Rehabilitation Langdon and Thompson,1999
- 5. Superior Cerebellar Peduncle Middle Cerebellar Peduncle Inferior Cerebellar Peduncle Pons Olive Cross Section of the Cerebellum Cerebellar Cortex Pons 4th Ventricle Dentate Interpositus Fastigial (Snell, 1992 Clinical Neuroanatomy for medical students)
- 6. Cerebellar Ataxia in MS: Pathology • In clinically definite MS • Gray and White matter pathology in cerebellum seen • Demyelination affects 14-39% of cerebellum on average • Axonal damage and neuronal loss seen with cerebellar cortical lesions – can occur independent of focal lesions Parmar et al 2018 • Cerebellar peduncles are common lesional sites • In Animal models (EAE) models Purkinje cell loss and altered ion channels and firing patterns seen. • Demyelination and volume loss predicts deficits in motor skills and cognitive dysfunction Parmar et al 2018 Demyelination in MS PL staining Wilkins Front Neurology 2017 Article 312
- 8. Gross Anatomy Cerebrocerebellum Spinocerebellum Vermis and Flocculonodular lobe
- 9. Signs and Symptoms of Cerebellar Ataxia
- 10. Signs and Symptoms • Oculomotor abnormalities • Dysarthria • Deficits of Limb Movements • Abnormalities of gait and posture • Cognitive and affective Signs
- 12. Occulomotor Abnormalities Saccades Stabilization of visual image on the retina Test Ocular Fixation in different Positions Saccades Pursuit OKN VOR VOR suppression Anatomy
- 13. Oculomotor Abnormalities Flocculus •Gaze Evoked Nystagmus •Jerky (saccadic) smooth pursuit •Poor suppression of the Vestibulo-ocular reflex
- 14. Oculomotor Abnormalities Gaze Evoked Nystagmus Nystagmus-associated gray matter degeneration in medial cerebellar, posterior medullar, central pontine, and superior collicular regions. Bede et al 2018 Front in Neurology v9 article 691
- 15. Oculomotor Abnormalities Dorsal Vermis and Fastigial Nucleus Saccadic hypo-or hyper-metria
- 16. Oculomotor Abnormalities Nodulus Periodic Alternating Nystagmus Increases vestibular time constant In MS see with demyelination of central vestibular connections at the Cerebellar peduncles
- 17. Oculomotor Retraining in people with MS and Cerebellar Signs OPTIMEYES Oculomotor Re-training in Progressive MS with Cerebellar Signs Intervention N=15 Vs n=13 Control Commercially Available Apps Vision Tap Focus BuilderClinical Measures Lab based measures of Motor Control Box and Blocks Functional Reach SARA 9 Hole Peg Test Eye-Hand Tracking Postural SwaySmooth Pursuit & Saccades • Differences seen between Healthy Controls and pwMS Significant changes in eye-hand tracking No change in clinical outcome Subjective reports of improvements With balance in complex environments
- 18. Dysarthria
- 19. Dysarthria Test Sustained Vowel phonation “ah” “ee” Pa/ta/ka Read test / repeat sentence Characteristics Scanned speech 71% Slowness 62% Slurring 48% Not Understand syllables/words 29% Add pauses 24% Loss of intonation 24% Voice tremor 10% Hestitations Accentuate some syllables Omit appropriate pauses Add inappropriate pauses Left Superior Paravermal Anatomy
- 20. Dysarthria Rusz et al 2018 Multiple Scleosis and Related disorders 62-69 Rusz et al 2019 Brain and Language 58-64 56% pw MS have dysarthria Pyramidal (spastic) Ataxic Mixed Differences between Ataxic-pyramidal and pyramidal presentation • Oral diadochokinesis • Excessive loudness variation • Slow speech Reduced maximum speed during Oral diadochokinesis test associated with cerebellar volume loss
- 21. Deficits in Limb Movements
- 22. Deficits of Limb Movements Dysmetria Tremor Hypotonia Children>adults Acute>Chronic Decomposition of Movements Dysdiadochokinesis
- 23. Deficits of Limb Movements Tests Upper Limb •Finger to nose •Finger to finger •Alternate Pronation-supination •Stewart-Holmes Manoeuvre •Writing •Drawing Lower Limb •Heel-shin test •Sustained leg raise Bain JNNP (2002) 72 i3-i9
- 24. Dysmetria Cool Control Position Velocity Acceleration Biceps Triceps 0 700 ms Rapid Elbow Flexion Motor Cortex Cerebellum Spinal Cord Agonist Antagonist Villis and Hore , 1980. J.Neurophysiol.
- 25. Tremor Position Velocity Biceps Triceps Spring Inertia Tremor driven by stretch reflex loops (Flament et al, 1984. Exp. Neurol). (Adapted from. Liu et al, 1997. Movement Disorders) Tremor enhanced by visual feedback Both on Target off Cursor off Controls MS
- 26. Tremor Tremor Rest Action Postural Kinetic Intention An involuntary oscillation of a body part MS :Tremor seen in 58 % - action tremor only affecting Arms >>legs ~ head ~trunk Alusi et al Brain 2001 Bain JNNP (2002) 72 i3-i9
- 27. Deficits in Gait and Posture
- 28. Abnormalities of Gait and Posture • Stance eyes open / closed • Single leg / tandem • Mini- Best Test • Berg Balance Scale • Timed Up and Go • ICARS • SARA Winser et al APMR 2017 270-276 Tests Sway Reactive Balance Anticipatory Postural Adjustments
- 29. What is wrong with Cerebellar Gait? Postural Control and Gait Lateral Excursion During Weight Shifting Error of foot Placement onto Visual Target Morton & Bastain, 2003 J Neurophysiol
- 30. Cerebellar Postural Control and MS • Diffusion tensor imaging: • Superior cerebellar peduncle integrity with kinetic deficits and poor postural sway with eyes open • Inferior cerebellar peduncle integrity associated with worse reactive balance and poor postural sway with eyes open and closed • Fallers have smaller gray and white matter cerebellar volume compared to non- fallers Gera et al 2019 Kalron et al 2018
- 31. Cognitive and Affective Signs
- 32. Cognitive and Affective Signs (Schmahann and sherman, 1998. Brain) Bilateral Posterior Inferior Cerebellar Artery Infarct • Disturbances of executive function • Visuospatial disorganisation and impaired visual memory • Personality change • Linguistic difficulties (Schmahmann and Sherman 1997) Post recovery 3 months later Post-infectious Cerebellitis Normal Rey-Osterrieth figure
- 33. •More pronounced after large bilateral + acute disorders •Especially with lesions of the posterior lobe •The vermis consistently involved with pronounced affective presentations Cognitive and Affective Signs Post recovery 3 months later Post-infectious Cerebellitis Normal Rey-Osterrieth figure
- 34. Cognitive and Affective Signs in MS Cerebellar Lesion in MS worse on: Information processing speed tests Verbal fluency Weier et al 2014 ; Cerasa et al 2013 SDMT α cerebellar intracortical lesion load α cerebellar volume Damasceno et al 2014 Weier et al 2014 Middle cerebellar Peduncle lesion can result in cognitive impairment Tobyne et al 2017
- 35. Potential Mechanisms of Recovery
- 36. N=42 Degenerative Diseases RCT Therapy 4 weeks (6hrs / week) Strength exercises Range of Motion Static and Dynamic Balance Walking outside / Stair Climbing Improvements Walking Speed Reduction in falls Maintained at 12 weeks Miyai et al 2012 Neurorehab and Neural Repair 26(5) 515-522 Intensive Co-ordination Training Cerebellar (n=10) or Afferent pathway degeneration (n=6) 4 weeks 3hrs / week Improvements in Walking And Co-ordination Ilg et al 2009 Neurology 73 1823-1830 Trials in Degenerative Ataxias
- 37. Mechanisms of Recovery I Substitution: Within the Cerebellum Contralateral Cerebellum (Amrani et al 1996)
- 38. Changes in Cerebellar Peduncle Integrity with training in MS 12 weeks of video-based balance training associated with increased integrity in the superior cerebellar peduncles Prosperini et al 2014
- 39. Mechanisms of Recovery II Substitution: Other Sensorimotor Areas Somatosensory Cortex (Keller et al, 1990; Mackel, 1987) Deep Cb Nuclei Lesion Recovery over 30-40 days Lesion Sensory Cx Symptoms re-appear Synapses Sensory Cx to Motor Cx Pyramidal Cells
- 40. Improvements in Hand Function with Motor Cortex Stimulation Rapid TMS Motor Cortex Stimulation (5HZ Stimulation 10 stimuli x 18) 100% Resting motor Threshold 8 pw MS + cerebellar Signs Percentage improvement in 9HPT Immediately after 10 min later 20 min later Koch et al, 2008 MS 14 995-998
- 41. Summary • Motor and Cognitive signs can be seen • Poor Prognostic Indicator for Recovery • Cerebellar Signs and symptoms vary with lesion location • Effects of training oculomotor control in pwMS and cerebellar dysfunction unclear • RCTs in pure cerebellar degenerations highlight that functional improvements can occur with training • Potentially several mechanisms of Recovery