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Spasticity Dystonia and Symptom Management in Non-malignant Neurological Disease PowerPoint Presentation

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Description : Check out this medical PowerPoint presentation titled "Spasticity, Dystonia and Symptom Management in Non-malignant Neurological Disease" by Dr Emily HARROP.
This medical PowerPoint presentation talks about Spasticity, Dystonia and Symptom Management in Non-malignant Neurological Disease.
Spasticity and dystonia are two common neurological symptoms that can occur in non-malignant neurological diseases such as multiple sclerosis, cerebral palsy, and Parkinsons disease. These symptoms can greatly affect a persons quality of life and can make it difficult for them to perform daily activities.
Spasticity is a condition where muscles become stiff and difficult to control, causing pain and limiting movement. It is caused by damage to the part of the brain or spinal cord that controls voluntary movement. Treatment options for spasticity include physical therapy, medications such as baclofen or botulinum toxin injections, and surgical procedures such as selective dorsal rhizotomy.
Dystonia is a movement disorder that causes muscles to contract involuntarily, leading to repetitive movements or abnormal postures. It can affect one muscle or group of muscles, or the entire body. Dystonia is often caused by a malfunction in the part of the brain that controls movement. Treatment options for dystonia include medications such as anticholinergics, botulinum toxin injections, and deep brain stimulation.
Symptom management for spasticity and dystonia involves a multidisciplinary approach. This can include a combination of medications, physical therapy, occupational therapy, and assistive devices to improve mobility and reduce pain. It is important for individuals with spasticity or dystonia to work closely with their healthcare team to develop a personalized treatment plan that meets their specific needs.
In addition to traditional treatment options, alternative therapies such as acupuncture, massage, and yoga may also be beneficial for managing spasticity and dystonia symptoms. These therapies can help to promote relaxation and reduce muscle tension, which can improve overall function and reduce pain.
Overall, effective symptom management for spasticity and dystonia requires a comprehensive approach that addresses both the physical and emotional aspects of these conditions. With the right treatment plan, individuals with these symptoms can improve their quality of life and maintain their independence.

Tags : Spasticity, Dystonia, Symptom Management in Non-malignant Neurological Disease, multiple sclerosis, cerebral palsy, Parkinsons disease, Child Neurology PowerPoint presentation, Neurology PowerPoint presentation

Published on : Mar 21, 2023 | Views : 1713 | Downloads : 0

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Slide 1-

Spasticity, Dystonia and Symptom Management in Non-malignant Neurological Disease Dr Emily HARROPConsultant in Pediatric Palliative care
Slide 2-

Introduction Approaching Neurological symptoms from ‘first principles’ Practical aspects of neurological symptom management Specific Symptoms (can be difficult to distinguish) Agitation Seizures Spasticity Dystonia Neuropathic Pain - young / non-verbal patients Case Studies
Slide 3-

Evaluation From First Principles Manifestation Impact Symptom Treatment Pharmacological Non-Pharmacological Quality of life is likely to be the desired outcome measure (including wider family QoL)
Slide 4-

Non Pharmacological Strategies Consider ‘normal’ reasons to be unsettled (hunger, nappy / pad) Address all aspects of symptoms (eg ‘total pain’) Use of multidisciplinary team Physiotherapy, OT, Speech therapy, Play specialist, chronic pain rehabilitation
Slide 5-

Pharmacological Measures Small evidence base and extrapolation from adult studies. Many drugs used are unlicensed in children or “off label” Consider who is administering drug, route and compliance Rational and empirical approach to prescribing
Slide 6-

Prescribing Decisions Matrix Lower impact ‘manifestations’
Slide 7-

Specific Symptoms
Slide 8-

Neurological Symptoms in Palliative care Agitation Seizures in the palliative context Spasticity Dystonia / Muscle spasms Neuropathic Pain & Visceral hyperalgesia
Slide 9-

Agitation
Slide 10-

Agitation Think of treatable causes: Reflux / constipation / gastric erosion Urinary retention Drug causes Muscle spasms / dystonia Hypoxia Raised ICP Fear / anxiety / environmental factors Uncontrolled Pain
Slide 11-

General Measures Reassurance Maintaining a calm environment Physical comfort measures (eg cuddles) Distraction including play therapy Massage Positioning Chronic pain psychology Physiotherapy & OT
Slide 12-

Agitation Buccal midazolam - Half seizure dose Haloperidol - Low dose Levomepromazine - Particularly useful in EoLC
Slide 13-

Seizures
Slide 14-

Seizures Think of possible underlying causes: Intercurrent illness Constipation Hormonal (puberty, periods) Decreased absorption of medication Weight gain Metabolic (low BM, calcium) Disease progression in CNS pathology
Slide 15-

Seizures Maintenance anticonvulsants depend on cause / type – Neurologist driven Clobazam for episodes of poor control Acute seizures, buccal midazolam, can be repeated Paraldehyde PR Sometimes half loads of phenobarbitone third line Then if palliative may aim for sc infusion to allow home treatment: Midazolam or clonazepam and / or phenobarbitone
Slide 16-

Spasticity
Slide 17-

Velocity dependent increase in tonic stretch reflexes causing hypertonia Corticospinal tract should stimulate GABA release at spinal level Damage causes disinhibition of spinal reflex arcs and muscle over activation – ‘positive side’ of upper motor syndrome Treatment is focused on muscle rigidity / hypertonia rather than primarily on the spasticity ‘negative side’ of upper motor syndrome is reduced voluntary motor activity leading to weakness and poor selective motor control
Slide 18-

Spasticity - CP Aims of treatment will be improved function / quality of life Adequate physiotherapy essential Occupational therapy Access to education / peer activities Drugs are only part of the picture…
Slide 19-

Baclofen Benefits Works at post synaptic GABAB receptor in spine to reduce excitation in spinal arc Used as maintenance medication for spasticity Burdens Difficulty crossing BBB Can use IT pump Narrow therapeutic window before problems arise: Truncal hypotonia Sedation Increased seizures
Slide 20-

Tizanidine Benefits Central alpha-2 agonist Better tolerated than baclofen in adults Used as maintenance medication for spasticity Burdens Limited experience in paediatrics Significant side effects: Sedation Hypotension Agitation Depression GI upset Liver toxicity
Slide 21-

Dantrolene Benefits Works directly at the muscle level by inhibiting calcium release from the sarcoplasmic reticulum Third line for background medication Burdens Can cause profound global weakness Catastrophic liver failure in 1% of patients
Slide 22-

Diazepam Benefits Increase pre-synaptic inhibition via GABA at the spinal level Very effective for short term emergency muscle relaxation Useful if sleep is disturbed by muscle tone Burdens Highly sedating Tolerance easily developed Withdrawal after long term use can be particularly difficult in the young
Slide 23-

Dystonia
Slide 24-

Dystonia in Palliative Care Pattern of sustained disturbed muscle contraction causing abnormal postures associated with involuntary movements Related to damage around the area of basal ganglia Mostly secondary dystonia in GMFCS 5 cerebral palsy Also genetic, metabolic, mitochondrial causes Pain Loss of function Sleep disturbance Triggered by intercurrent illness, pain (esp. visceral) Wound up’ by sensory overload (anxiety / noise)
Slide 25-

For example Red – awake and distressed Yellow – awake and not distressed Green - asleep
Slide 26-

Non-pharmacological Management Understand what is ‘driving’ dystonia Pain – particularly visceral pain (gut failure) – venting gastrotomy Intercurrent illness (can this be treated?) Keep a diary of sleep / wake / discomfort Warm water immersion Massage Positive sensory feedback Re-positioning
Slide 27-

Medication in use at referral was studied 29% of children on no medication Median number of meds used was 2 Increased meds with worsening GMFCS 62% experienced significant side effects from medication Commonest drugs used were baclofen, trihexyphenidyl, l-Dopa, diazepam Most side effects from trihexyphenidyl, baclofen, l-Dopa Suggest the need for rationalisation of drug treatment of dystonia
Slide 28-

Gabapentin is used for neuropathic pain, epilepsy and occasionally movement disorder Four year retrospective study looked at gabapentin in severe dystonia in children seen at Evelina (69 children) Significant improvement seen in Sleep quality & amount Mood & agreeableness General muscle tone, involuntary contractions Seating tolerance Gabapentin appears to significantly ameliorate severity and improve activities of daily living and quality of life in dystonia
Slide 29-

Children with dystonia are at increased risk of developing fixed musculoskeletal deformities These cause pain, limit function and interfere with care delivery Retrospective study of 279 children with dystonia 58% has some deformity at referral Hips and spine were the main sites Children with secondary dystonia had a much greater risk of progression to fixed musculoskeletal deformities than those with primary dystonia Children with additional spasticity were at particularly high risk
Slide 30-

Drug Management Gabapentin Sometimes need higher doses than used for neuropathic pain Good first line intervention Diazepam Benzodiazepine with the most potent effect on tone – useful as add on / rescue? Trihexyphenidyl Can be helpful, some issues with side effect profile (tardive dyskinesia) Increase dose very slowly Clonidine Appears to be very helpful Good experience from Evelina Team at high doses (BP tolerates) Transdermal patches available for higher doses Some experience in SC infusions
Slide 31-

Neuropathic Pain
Slide 32-

Neuropathic Pain in childhood Mechanisms operating during development can profoundly modify the consequences of nerve damage (pain circuits under go postnatal maturation) Generally, the later the injury the worse the risk of developing NP (birth injuries to brachial plexus rarely cause it) Phantom limb rare in congenital absence, more common with increased age of surgery (especially with Ca) Fractures to humerus more likely to cause NP >5 years, worse in adolescence
Slide 33-

Neuropathic Pain in Childhood Solid tumours less common in childhood High rate of NP with primary neuronal tumours In brain tumours usually reflects leptomeningeal spread 20-40 adults on chemo get NP – rate unknown in children Vinca alkaloids and platinums are some of the worse Anti-GD2 treatment for NBT includes anti- NP protocol (gabapentin / ketamine) Oncology Non-Onc 6/40 children with Cerebral Palsy developed significant NP after complex orthopaedic spinal surgery Metabolic disease are an area to consider
Slide 34-

Neuropathic pain in the young Different ‘age appropriate’ words used: Weird legs Fuzzy pain Cloud on my shoulder Spade digging my head My head is a dinosaur My back is a tiger on fire I’ve got buzzy bees The pain is ‘poking’ me ‘Hot’ pain
Slide 35-

Neuropathic pain in neurodisability Biological plausibility Metabolic storage disease Mitochondrial disease Changes in behaviour Unexplained screaming Combative behaviour Difficulty with handling / delivering care Unable to enjoy cuddles
Slide 36-

Case Study Poppy
Slide 37-

Case Study One - Poppy Poppy (4) was receiving end of life care as an in-patient, she had atypical leucodystrophy She had very difficult to interpret symptoms, requiring different interventions (including non pharmacological) She had been non verbal for several months, and her ability to communicate was regressing further Manifestation was unexplained screaming
Slide 38-

Poppy Manifestation = screaming Impact – devastating Symptom? Treatment??
Slide 39-

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Slide 40-

Case Study – Ellie
Slide 41-

Novel Subcutaneous Infusion Ellie had a rare mitochondrial disease with Parkinson’s-type features She is was longer absorbing feed, and was felt be approaching EoL Parents preferred care outside of PICU She was dependent on an IV infusion of clonidine and morphine for relief of dystonia / pain How can we achieve this? There is no specific data on clonidine subcutaneous infusion for dystonia in children …. Can it be safely given subcutaneously? Can it be combined with morphine?
Slide 42-

Solving the Mystery Subcutaneous infusions of clonidine are sometimes use in adult chronic pain (as well as intra-thecal) Intra-thecal infusions sometimes contain morphine and clonidine together Contacted adult pain specialist and adult palliative care colleagues for information on their experience of use Conversion at 1:1 but approximately 10% increase needed compared to previous stable dose Successfully given by subcutaneous infusion with good clinical effect and no untoward local reaction EoLC delivered in Helen House
Slide 43-

Case Study - Hadi
Slide 44-

Dystonia & Visceral Pain Hadi is 3 years old and had severe dystonia This is felt likely to be secondary to an undiagnosed mitochondrial disease He struggles to tolerate his feeds and enteral medication Visceral symptoms ‘wind up’ his dystonia – which then worsens his feed tolerance – vicious circle… Baclofen and gabapentin are introduced to manage dystonia Feeds regime is adjusted Medicines remains a problem
Slide 45-

Rationalising enteral tube drugs Hadi has large volumes of medication via his PEG and is often sick / refluxes The Care Team / parents have already tried giving these staggered and also more slowly – to limited avail He now has a PEG-J tube for his feed – how can we rationalise his medication schedule to minimise reflux / vomits? He is on Movicol (Paed), baclofen, ranitidine and diazepam – what would your approach be?
Slide 46-

Solving the Mystery Movicol (Paed) – does not need to be absorbed, works locally in the colon and has a large volume Ranitidine and benzodiazepines have ‘acceptable’ post-pyloric absorption Baclofen is not at all well absorbed beyond the stomach Plan: Spacing out / slowing administration Giving Movicol PEG-J (use sterile water) Trying rantidine or benzodiazepines (one at a time) and monitoring clinical effect Explain to parents that baclofen will need to go via PEG
Slide 47-

‘Breaking down’ a symptom M.I.S.T. Identify the symptom rather than just its manifestation (ie what is the meaning of what we observe?) Asses its impact Is there an underlying cause to consider? Decide whether it needs treatment Evaluate whether there is a simple, non pharmacological solution Decide on the appropriate drug treatment Quality of life is likely to be the appropriate outcome measure...
Slide 48-

Any Questions?