SLT and Neurodegenerative Conditions Past Paper PDF December 2022

Neurodegenerative Conditions 1: Introduction and Parkinson’s Disease Sophie MacKenzie 1 By the end of this series of mini lectures, we will:  Be able to list a number of neurodegenerative disorders  Identify a number of common communication and swallowing...

Neurodegenerative Conditions 1: Introduction and Parkinson’s Disease Sophie MacKenzie 1 By the end of this series of mini lectures, we will:  Be able to list a number of neurodegenerative disorders  Identify a number of common communication and swallowing difficulties related to progressive neurological conditions  Discuss implications for SLT management 2 What do we mean by neurodegenerative?  Disease of neurological origin  Affecting CNS, PNS or both  Progressive in nature 3 Neurodegenerative diseases may affect:  Speech (dysarthria, AOS)  Language (aphasia)  Other cognitive abilities  Swallowing  Behaviour  Physical abilities  Mental health  All 4 dimensions of WHO ICF  Families and carers 4  Parkinson’s Disease  Idiopathic  First described by James Parkinson (1817) ‘An essay on the shaking palsy’  Secondary (e.g. multiple trauma in boxing)  Parkinson Plus syndromes (multiple system atrophy)  Begins in mid-to-later life  1 in 500 people affected (prevalence)  Symptom onset > death is about 9 years. 5  PD: what is it?  Progressive disease of central nervous system, specifically basal ganglia 6 What causes PD?  Associated with depletion of dopamine > loss of dopamine-secreting neurons in the midbrain area known as the substantia nigra (part of basal ganglia)  No conclusive cause for the death of these neurons has been found.  Recent evidence suggests that harmful ‘clumps’ of protein in the neurons of the substantia nigra may be the source of cell death. Basal Ganglia recap  Basal ganglia is a set of subcortical nuclei: Corpus striatum (caudate and putamen) Globus pallidus Subthalamic nucleus Substantia nigra  They work together to regulate motor function  By selectively activating and supressing learned motor programs they facilitate initiation and termination of movement and suppress extraneous movement PD: what are the salient characteristics?  Tremor – resting, “pill-rolling”  Bradykinesia (hypokinesia) – slowness of movement, inability to initiate movement, leading to:  Rigidity – increased tone, so-called “cogwheel rigidity”  For SLTs, hypokinetic dysarthria, dysphagia, associated language/ cognitive issues (dementia) 9 Non-motor symptoms  Understanding of Parkinsons is changing and we now recognise there are numerous non- motor symptoms that can present associated with PD (Park and Stacey, 2009) PD – Neuropsychiatric Symptoms  Memory and cognitive changes.  Dementia.  Depression.  Anxiety.  Hallucinations.  Hypokinetic dysarthria  Imprecise consonants  Monopitch  Monoloudness  Reduced loudness  Short rushes of speech may see accelerated AMRs, e.g. /pəpəpə/  Repeated phonemes (neurogenic dysfluency?)  Palilalia - repeated utterances (words, phrases)  ↓ vital capacity 12  PD: Cognitive/ language issues  Distractibility  ↓organisation/ planning skills (executive skills)  Difficulties with shared attention  Memory difficulties  ↓ ability to learn new information  Word retrieval difficulties  Dementia (approx 30%)  See also Lewy Body Dementia/ Dementia with Lewy Bodies (DLB) 13  PD: typical swallowing difficulties Oral-prep/ Oral stages: - ↑ mastication time - Limited movement of mandible - Repetitive tongue pumping - ↓ ability to form cohesive bolus - Slow oral transit 14 PD: typical swallowing difficulties Pharyngeal stage: - Delay in swallow trigger - ↓hyolaryngeal excursion > reduced airway protection - Piecemeal deglutition - ↓ pharyngeal constriction - Residue in valleculae and pyriform sinuses - Silent aspiration - Anterior escape of saliva - ↓ spontaneous swallows 15 PD: typical swallowing difficulties Oesophageal stage: - Feelings of oesophageal obstruction - Reflux - Chest pain - General gastrointestinal dysfunction 16  PD medication  Levodopa (L-dopa)  Levodopa/ carbidopa combo e.g. Sinemet  On-off effects – impacts on assessment/ intervention  Peak-dose dyskinesia: overcorrection of dopamine 17  PD: Aims of SLT management  Maximising residual skills (e.g. LSVT, breath support)  Maintaining functional communication  AAC e.g. amplifiers  Ensuring safety of oral intake  Reviewing for change and altering management accordingly  Support and advice – holistic 18 Lee Silverman Voice Treatment®  http://www.lsvtglobal.com/  http://www.lsvtglobal.com/news/video  http://www.youtube.com/watch?v=UJb54lrJ0nA  http://www.youtube.com/watch?v=ja4Gpwy1VF4  Large and growing evidence base  Other variants, such as voice modulation therapy 19 Progressive Supranuclear Palsy PSP  Now seen as discrete from, but may be confused with, PD  Unknown cause  Rare (5 new cases per 100 000 people over 50 per year)  Onset > death approx 10 years  Physical issues: balance, general mobility, eye mobility and focussing problems  Behaviour issues: irritability, apathy  Cognitive problems: memory, reduced concentration  Speech (typically spastic dysarthria) and swallowing (often more severe than in PD) http://link.springer.com/article/10.1007%2FPL0000952 8# 20 Poem Reflecting on Antony Gormley’s ‘Another Place’ I stand alone in another place. I am a man cast of iron. I watch the Irish Sea move further away, almost out of sight. The sea is kind at this distance. I take my secret sad breaths, quietly, more easily now. I am testimony to solitude amongst a community of others the same. We stand together, alone, watching, sensing, feeling, crying to be heard. We stand, as iron spirits cast amongst mortals. They will never know what we see, what we sense, how we feel. How can they ever know us? The sea spirits sing of creeping Manx fogs hiding Vannin’s mystic hoards. We lean forward to greet them. Surely they come to free us from our cast iron trap, to end our tidal punishment. I cannot warn the lazy English of the Buggane and its ghostly howling ranks. Why should I anyway? These mortals stare and touch with hearts colder than mine, giggling their unwillingness to know art’s fine intent. Within these iron human forms the artist cruelly expresses the futility of life and living. The mortals mock, but they fear how we represent the futility of their own lives, the artist’s life too. Clouds move quickly on, rain is in the air. Easterly winds chill my iron core, the weakling mortals are gone now. Gusts play across the beach and over the sea, and the mists disappear failing to rescue me yet again. As the sea returns to overwhelm me, and as I hold my breath once more, I ask. When will our saline torture end, will this be my last time in ‘another place’? I hope so. Ian Procter – November 2015 21  Another Place Antony Gormley 22 HD: what is it?  Inherited autosomal dominant degenerative neuropsychiatric disease  Symptoms typically begin to develop in 4th decade  Death occurs 15-20 years after onset  Affects 4-7 individuals per 100 000  Equal male: female ratio 23 HD: symptoms  Chorea  Hypertonia > rigidity  Dystonia  Incoordination  Eye movement abnormalities  Weight loss  Dysarthria (hyperkinetic)  Dementia  Dysphagia  Personality changes  Attention deficits  Dementia  Depression  Specific language deficits 24 HD: specific language deficits (from Yorkston, Miller and Strand 1995) Language task Findings Evidence Connected speech Reduced number of words Illes (1989) Decreased syntactic Gordon and Illes (1989) complexity Speedie et al (1990) Decreased melody line Decreased phrase length Decreased articulatory agility Increased paraphasic errors Word-finding diffs Naming More visually-based errors Lawrence et al (2000) than normals Decreased confrontation Wallesch and Fehrenbach naming (1988) Auditory Decreased understanding of Speedie et al (1990) subtle prosodic aspects comprehension Reduced Token Test scores Wallesch and Fehrenbach Discourse comprehension (1988) Murray and Stout (1999) 25 HD: Hyperkinetic dysarthria  Choreic movements can affect respiration, phonation, articulation: - respiration: intensity may be affected, sudden forced expiration/ inspiration - phonation: pitch may be raised, harsh vocal quality - articulation: phonemes may be prolonged, distorted 26 HD: speech Managing dysarthria in HD: Mild (e.g. lgl relaxation techniques) Moderate (e.g. environmental changes, supported conversation) Severe (AAC options but NB physical, cognitive, behavioural issues) 27 HD: swallowing  Physical,positioning difficulties  Whole body chorea  Choreic movements of lips/ jaw/ tongue impact on oral stage  Behavioural issues may affect swallow safety, e.g. eating inappropriate consistencies  Need for additional calories 28  HD: specific management issues  Issues related to inherited nature of disease: - seeing an older family member - children - genetic testing - who else is/ might be affected? 29 Huntington’s Disease “Here’s biological determinism in its purest form…Your future is fixed and easily foretold…Between ten and twenty years to complete the course, from the first small alterations of character, tremors in the hands and face, emotional disturbance, including – and most notably – sudden uncontrollable alterations of mood, to the helpless jerky dance-like movements, intellectual dilapidation, memory failure, agnosia, apraxia, dementia, total loss of muscular control, rigidity sometimes, nightmarish hallucinations and a meaningless end. This is how the brilliant machinery of being is undone by the tiniest of faulty cogs, the insidious whisper of ruin…” Saturday by Ian McEwan 30 Motor Neurone Disease  MND is characterised by loss of motor neurons in:  motor cortex (UMN)  brainstem (UMN and LMN)  cranial nerves (LMN)  spinal nerves (LMN).  Affects corticonuclear (corticobulbar) and corticospinal tracts. Motor Neuron(e) Disease Stats from MNDA:  Most people diagnosed with the disease are over the age of 40  Highest incidence occurring between the ages of 50 and 70  Men are affected approximately twice as often as women  Incidence: about two people in every 100,000 will develop MND each year  Prevalence: number of people living with MND at any one time is approximately seven in every 100,000 32 MND: types  Amylotrophic Lateral Sclerosis (ALS)  Progressive Bulbar Palsy (PBP)  Primary Lateral Sclerosis (PLS)  Progressive muscular atrophy (PMA) 33 Types of MND Amyotrophic Progressive Primary Progressive Lateral Bulbar Palsy Lateral Muscular Sclerosis Sclerosis Atrophy Damage to Damage to CN Damage to Damage to corticospinal nuclei corticospinal anterior horn tract and tract cells anterior horn cells UMN and LMN UMN and LMN UMN Spinal nerves (LMN) 2-5 years 6 months-3 May not always 5-10 years post- years affect life diagnosis expectancy. Symptoms of MND Site of Neural Involvement Upper Motor Neuron Lower motor Neuron Weakness, slow Weakness, muscle atrophy, movement Increased Fasciculations of lips, tone of lip, tongue, soft tongue and soft palate; palate and jaw; Voice may be breathy Voice may be strained/strangled Weakness, slow Weakness, muscle atrophy movement, Increased Fasciculations in arms and tone of arms and legs legs from Yorkson et al. 2013, page 3 SLT diagnoses in MND  Dysarthria  Anarthria  AOS  Cognitive issues  Dysphagia 36 We no longer classify MND as just a motor disorder. For example, there is often prefrontal, frontal and temporal lobe involvement which can be associated with behavioural disturbance, dysexecutive syndrome and in around 15% of cases, frontotemporal dementia (Baumer, Talbot and Turner, 2014) MND: SLT role in communication  Early intervention: addressing difficulties before they arise  Begin therapeutic relationship as early as possible  Early intervention allows client and family time to “come to terms with” changes  Maintenance of functional communication  Exercises to strengthen muscles/ increase ROM are contraindicated (fatigue)  Clients may benefit from articulation tips (e.g. over- articulation)  Compensatory techniques, strategies, changes to environment, advice to listeners)  Light tech AAC  High tech AAC 38  Swallowing problems in MND  Speech and swallow problems OFTEN co-occur  Consistent pattern of decline but can be variability in rate of decline – be responsive!  Fatigue (reduced breath support exacerbated by swallow apnoea)  Reduced breath support → reduced airway protection  Reduced respiratory support may affect possibility of surgical intervention as disease progresses  Weakness → reduced mastication, fatigue, reduced oral manipulation, reduced lipseal, pgl weakness  Coughing on thin liquids often initial sign of dysphagia (NB dehydration)  Saliva changes: texture/ xerostomia/ saliva escape 39 MND: Early stage dysphagia intervention  Chin tuck  Swallow manoeuvres (e.g super supraglottic swallow)  Texture modification: (e.g. avoid foods requiring a lot of chewing, crumbly foods, thickened fluids)  Advice re: distractions, what to do in the event of choking)  Enteral feeding discussions 40 MND: later dysphagia management  More texture modification (soft, puree)  Thickening of fluids  Increase sensory awareness (eg temperature, taste) 41  MND: Late stage dysphagia management  Enteral feeding (PEG)  May be indicated: multiple choking/ aspiration episodes, fear/ aversion, pt not meeting nutrition/ hydration needs orally  Oral + PEG, NBM 42  MND: dysphagia management  Regular review of swallowing  Early discussion of eating/ drinking options  Close liaison with other members of MDT  Advice  Support: client and family  Support and supervision for you 43 Multiple Sclerosis  Progressive  May also be remitting-relapsing  Demyelination of CNS  Myelin stripped away by macrophages, exposing axon  Scar tissue develops - sclerosis refers to the scars that form in the white matter of the CNS as a result of demyelination.  Nerve conduction blocked  Remitting-relapsing type begins between 18-40 years  Progressive type begins later  ?viral  ?genetic predisposition  http://www.youtube.com/watch?v=GG0PZhjfWq4 44  MS: symptoms  Depends on where lesions are in CNS CNS site Impairment Cerebrum Cognitive, affective, behavioural changes Spinal cord Weakness, spasticity, numbness, bowel and sexual dysfunction Brain stem Vertigo, nystagmus, dysarthria dysphagia Cerebellum Ataxia, tremor, dysarthria Optic nerve Optic neuritis (loss of vision, pain) (adapted from Yorkston, Miller and Strand 1995) 45  MS: Communication  Speech: AOS rare, dysarthria (spastic-ataxic, mixed) occurs in approx 50%  Language: aphasia rare, higher level language deficits possible (WFDs, executive skills)  Cognitive: memory, learning, problem-solving  Affect: depression  Fatigue 46  MS: management of communication issues  Dysarthria: - Breath control exercises for phonation and volume Beware fatigue - Working on appropriate rate (pacing) for equal-and-excess-stress patterns Beware fatigue - Speech augmentation, e.g. use of alphabet board - AAC options but NB tremor, visual issues, spasticity, ataxia 47 MS: EDS  Associated with lesions to brainstem  Delayed swallow reflex  ↓ pharyngeal peristalsis  Treat according to presenting difficulty: texture modification, positioning, reduction of distractions 48 Neurodegenerative disorders  PD, HD, MND, MS  Friedreich’s Ataxia  Progressive supranuclear palsy  Dementia  Support and supervision 49