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2020-10-01 ERN-RND Webinar 01.10.20 The Natural History of Hereditary Spastic Paraplegias Rebecca Schüle Center for Neurology and Hertie Institute for Clinical Brain Research, Tübingen 1 Webinar outline • Phenotypic spectrum of HSPs: core symptoms and additional features • Typical gait patterns in HSP • Age of onset • Disease progression 2 1 01.10.20 Learning objectives • recognize typical signs and symptoms of HSP • recognize typical gait patterns in HSP • distinguish HSP from other motoneuron diseases and movement disorders • monitor disease progression in clinical practice 3 What is your professional background? a) Adult neurologist b) Pediatric neurologist c) Resident (Neurology) d) Resident (Pediatrics) e) Geneticist f) Researcher g) Rehabilitation specialist h) Nurse i) Patient / family member / patient advocate single choice 4 2 01.10.20 How much experience do you have with HSP? a) I consider myself a specialist for HSPs b) I routinely see HSP patients as part of my clinical practice c) I have seen some HSP patients and would like to learn more d) I do not have any practical experience with HSP (have never (knowingly) seen a patient) e) I am a researcher working on HSP and am interested in learning about the clinical aspects of HSP f) I or a friend/family member is affected by HSP and I would like to learn more multiple choice 5 Core symptoms of HSP 6 3 01.10.20 Heterogeneity > 150 known HSP genes phenotype core features SPG 1 – 82 maintained by omim (www.omim.org) Challenges: facultative complicating many HSP genes without SPG number or signs and symptoms part of different classification (e.g. ARCA, NBIA, CMT) 7 Selective vulnerability of UMNs > 150 known HSP genes selective vulnerability Selective vulnerability upper motoneurons 8 4 01.10.20 Axonopathy of upper motoneurons length-dependent axonopathy of upper motoneurons soma synapse axon 9 Clinical core features length-dependent axonopathy of upper motoneurons unilateral spasticity: Wernicke-Mann pattern • slowly progressive lower limb spasticity and weakness • brisk reflexes spastic paraplegia: • clonus • hip adductor • spastic Jerks • knee extensor • contractures • plantar flexor • extensor plantar response (positive Babinski sign) Kurzlehrbuch Neurologie, Thieme 10 5 01.10.20 Typical gait pattern in HSP (1) • narrow base • Pronounced balancing movements of the trunk are used to elevate the contralateral hip • Knees fixed in slightly bent position • Forefoot weight baring; natural heel-to-ball movement of the foot eliminated; heel does not touch the ground (à central dropped foot) 11 Typical gait pattern in HSP (2) • balancing movements of the trunk can be less pronounced • supination of the foot is common • can be combined with inversion of the foot 12 6 01.10.20 Typical gait pattern in HSP (3) • narrow base and scissor gait • Trendelenburg sign • severe dropped foot; feet are pulled forward over the top of the feet 13 Classification and differential diagnosis 14 7 01.10.20 • spastic paraparesis pure HSP • impairment of vibration sense • neurogenic bladder disturbance complicated HSP variable: cognitive impairment, optic atrophy, cerebellar ataxia, peripheral neuropathy, ... Classification of the hereditary ataxias and paraplegias. Lancet, 1983. 1(8334): p. 1151-5. 15 extrapyramidal sensory system system extra- cortical neurological systems UMN LMN PLS ALS SMA cerebellum HSP CMT Axonopathies 16 8 01.10.20 Which of the following diseases are typical differential diagnoses to consider when evaluating an HSP patient? a) Primary lateral sclerosis (PLS) b) Primary progressive multiple sclerosis (PPMS) c) Cerebellar ataxia with pyramidal affection d) Cervical myelopathy e) all of the above single choice 17 Diagnostic criteria of HSP Neurodevelopmental or slowly progressive neurodegenerative condition with • spastic paraplegia or • spastic tetraplegia with early and/or severe affection of the lower limbs or • spastic paraplegia as an early and/or prominent symptom of a multisystemic disease GeNeMove network 18 9 01.10.20 Age of onset 19 Age of onset distribution A. 80 13% 12% 60 11% • biomodal distribution: early childhood 10% 8% and ~40y Number 7% 7% 40 7% 7% 5% 5% • age of onset can vary even within 4% 20 3% families by several decades 1% 1% • no reliable prediction of age of onset 0 10 20 30 40 50 60 70 Age of Onset n = 608 B. 1,00 • limited correlation of age of onset with simplex 0,75 the underlying genotype 0,50 recessive Proportion 0,25 dominant 10 30 50 70 10 30 50 70 Age of Onset Age of Onset C. Schüle et al. Ann Neurol 2016 SPG3 10 (44) (n=7) 20 SPG4 35 (21) (n=142) SPG5 18 (16) (n=9) SPG7 (n=25) 36 (16) 10 SPG11 17 (19) (n=11) 020401030 50 60 70 Age of Onset 01.10.20 Additional Signs & Symptoms 21 Frequent additional signs & symptoms Bladder problems Sensory deficits 40% 30% • Detrusor hyperactivity • Frequent: vibration & 20% • Detrusor sphincter no, joint position yes, dyssynergy 47% • Surface sensation frequency 10% 53% 0% none mild severe moderate Pain Ataxia 60% 50% • Spastic? yes, 40% • Joints? 29% 30% • Neuropathic? 20% 16% 14% 16% • Spastic ataxia frequency • SPG7, POLR3A, ARSACS 10% 0% no, 71% non e mild severe moderate 22 11 01.10.20 Disease progression 23 How many years after disease onset do persons affected by HSP use a walking aid on a regular basis? a) ~ 1 year after onset b) ~ 5 years after onset c) ~ 10 years after onset d) ~20 years after onset e) Persons affected by HSP do not typically use walking aids single choice 24 12 01.10.20 Disease progression: walking aid dependency A. B. walking aid dependency wheelchair dependency • 22 years after disease onset 50% of persons 1,0 1,0affected by HSP use a walking aid on a regular basis. 0,8 0,8 0,6 0,6 0,4 0,4 0,2 0,2 median: 22 n = 608 Q1: 37 n = 608 A. B. walking01020304050607080 aid dependency wheelchair dependency01020304050607080 1,0 disease duration1,0 disease duration Schüle et al. Ann Neurol 2016 C.0,8 0,8 D. 25 walking aid dependency walking aid dependency age at onset age at onset 0,6 late early0,6 late early 1,0 Q4 Q3 Q2 Q1 1,0 Q4 Q3 Q2 Q1 0,4 0,4 total cohort total cohort 0,20,8 0,2 0,8 genetically confirmed genetically confirmed median: 22 simplex unsolved Q1: 37 simplex unsolved Disease0,6 progression: walking aid dependency0,6 01020304050607080 01020304050607080 disease duration disease duration 0,4 0,4 C. D. walking aid dependency walking aid dependency 0,2 age at onset 0,2 age at onset late early late early total cohort • Early age of onset is associated withgenetically walking confirmed 1,0 Q4 Q3 Q2 Q1 1,0 Q4 Q3 Q2 Q1 aid dependency later in the disease course! 01020304050607080 01020304050607080 0,8 0,8 disease duration disease duration 0,6 0,6 0,4 0,4 0,2 0,2 total cohort genetically confirmed 01020304050607080 01020304050607080 disease duration disease duration Schüle et al. Ann Neurol 2016 26 13 01.10.20 Disease progression: wheelchair dependency A. B. walking aid dependency wheelchair dependency 1,0 1,0 • 37 years after disease onset, 25% of persons affected by HSP use a 0,8 0,8 wheelchair on a regular basis • Earlier age of onset is associated 0,6 0,6 with later wheelchair dependency (data not shown). 0,4 0,4 0,2 0,2 median: 22 n = 608 Q1: 37 n = 608 01020304050607080 01020304050607080 disease duration disease duration C. D. Schüle et al. Ann Neurol 2016 walking aid dependency walking aid dependency age at onset 27 age at onset late early late early 1,0 Q4 Q3 Q2 Q1 1,0 Q4 Q3 Q2 Q1 0,8 0,8 0,6 0,6Spastic Paraplegia Rating Scale (SPRS) 0,4 0,4 6 • 13 item scale that measures disease severity in HSP Walking distance without pause 0,2 • Measurement of functional impairment, not Gait quality 0,2 Maximum gait speed neurological signs without functional relevance. total cohort genetically confirmed Climbing stairs • Only symptoms of pure spastic paraplegia are part of Speed of stair climbing Arising from chair 01020304050607080 01020304050607080the core scale. Complicating symptoms are recorded in an inventory. 4 disease duration disease duration Spasticity - hip adductor muscles • Easy to use under outpatient conditions Spasticity - knee flexion • For each item 0-4 points are awarded. The total score Weakness - hip abduction is the sum of the individual items. Weakness - foot dorsiflexion 3 Contractures of lower limbs Pain due to HSP related symptoms Bladder and bowel function 13 items, maximum score: 52 points Schüle et al. Neurol 2006 28 14 01.10.20 Spastic Paraplegia Rating Scale (SPRS) • Progression rate in SPG4 (most common subtype of HSP) is ~1 point per year (scale ranges from 0 – 52) 29 Spastic Paraplegia Rating Scale (SPRS) • SPG4 patients lose the ability to walk independently at an SPRS of ~19 points 30 15 01.10.20 Spastic Paraplegia Rating Scale (SPRS) 38 • individual progression rate: 1.4 points per year 22 500m 500m 40m 10-20m 10m 55y 67y 31 Key points • HSPs are axonopathies of upper motorneurons. • Progression is slow over decades. • Early disease onset is associated with slower progression rates (cave: rapid progression rates in some genetically defined HSPs) • Walking aid dependence, wheelchair dependence are patient-centered indicators of disease progression. • SPRS and gait distance can be used in clinical practice (and research) to monitor disease progression. 32 16 01.10.20 In a potential future webinar on HSP, I would like to learn more about… a) genetics of HSP and genetic testing strategy b) genotype phenotype correlations and individual subtypes of HSP c) therapeutic management of HSP d) physical therapy in HSP e) other topics (--> use chat function) multiple choice 33 This webinar has been supported by ERN- RND , which is partly co-funded by the European Union within the framework of the Third Health Programme “ERN-2016 - Framework Partnership Agreement 2017- 2021." Next Webinar: ‚Treatment of spasticity in HSP and leukodystrophies‘ by Annemieke Buizer 6.
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