Neurogenic Dysphagia Pt 2

Degenerative CNS

diagnosed at some point, and you expect it to decline, most cases develop over time

Parkinson’s disease

• Affects basal ganglia

• 2nd most common movement disorder

• Over 1 million people in US currently live with PD

• progressive motor system dysfunction resulting from reduced dopamine production (50%)

• Cardinal motor symptoms:

  • tremor

  • falls

  • bradykinesia (slowed movements; for swallowing- slowed tongue movements etc)

  • Rigidity: chest wall rigidity; not as much air in lungs before speaking

• Up to 100% will develop dysphagia

  • develops from basal ganglia dysfunction

  • basal ganglia runs on dopamine; dopamine is a neurotransmitter and over time the brain develops less of it

  • reduction of dopamine gives motor deficits (tremor)

  • slow, rigid, feel frozen; taking medication makes writhing motion

PD: timing of evaluation

• most patients are followed by multi-disciplinary clinics

• Early SLP assessment is helpful to establish baseline

  • follow-up in 6-12 month increments as indicated

  • compensations

  • rehabilitation: patients respond well to rehab

• In-patient admission often dysregulates PD meds and increases dysphagia risks

  • nurses get busy etc. patients may miss scheduling of pill

  • worse swallow function at bed side

PD: Patient Report

• most common complaints include:

  • coughing/choking during meals

    • greater difficulty with liquids

  • globus sensation/reflux; may often have co-occurring esophageal dysfunction

  • unintentional weight loss; hyperbolic state; reduced appetite; fatiguing quickly

  • Pnemonia- always watch pnemonia patients more carefully

  • monitor diet modification

  • Sialorrhea- excessive drooling

Brady kinesia

Hypokinesia: small, impoverished movement (shuffling gate)

Akinesia: without movement “freezing”

Rigidity

• cogwheel or lead-pipe tone

• decreased arm swing

• hypomimia (masked face)

Resting Tremor

• stops with intentional movement

• 75% of PD patients are temulous

• Postural instability

  • late manifestation in idiopathic PD

  • early onset implies atypical PD

• Additional motor symptoms

  • hypokinetic dysarthria

  • dysphagia

  • dystonia: prolonged muscle contraction

  • micrographia (handwriting issues)

  • unilateral onset

Parkinson’s Disease: Neuropathophysiology & Signs and Symptoms

• dysregulation of basal ganglia causes tremor, freezing, rigidity, and bradykinesia of oropharyngeal structures; you can see dysfunction across all motor aspects of a swallow

• basal ganglia regulates all movement

• this is a basal ganglia disorder

  • it is going to impact all motor movement

  • it is degenerative: you expect it to get worse over time

Oral Phase of PD

• anterior labial leakage

• delayed initiation of swallowing

• tongue pumping

• impaired bolus formation

• lingual tremor; tongue

• fast oral transit times

• pre-swallow spill

Pharyngeal phase of PD

• lack of epiglottic inversion

• penetration/aspiration

• marked valleculae and pyriform sinus residue

• slow and fast pharyngeal transit time

• reduced extent/duration of CP opening

• inadequate hyolaryngeal excursion

Impact of PD treatments on swallowing

• dopaminergic medication

  • timing of oral intake can impact extent of dysphagic symptoms (they habituate to meds over time, they need more and more mg over time and more frequently)

  • diet impacts medication absorption

  • overtime induces dyskinesias

  • inhaled dopamine newly available improving delivery and inconsistencies with pills

• Deep brain stimulation (DBS)

  • not everyone is a candidate; other disorders can benefit as well

  • surgery to fix dyskinesia; fix side effect from the oral medication

  • can cause risk to intelligibility and swallowing

  • for medication-refractory PD

  • unilateral or bilateral to STN, GPi, or VIM

  • markedly improves motor function and quality of life

  • generally agree that implantation does not improve axial symptoms such as swallowing

PD: airway protection

• research has shown that reflex and voluntary cough function is predictive of swallowing function in PD

  • weak cough can likely mean swallowing dysfunction

• Aspiration pneumonia is a leading cause of death in PD

  • results from a combination of dysphagia and dystussia (impaired cough reflux)

  • lack insight into deficits

What about PD+

• demonstrate similar symptomotology of PD dysphagia; however, at a much faster progression

• not responsive to dopaminergic medication

• PEG tube placement more common

• we see them more often; more proactive for treatment

• increased meal times, more difficulty w/ liquids first

• Progressive supranuclear palsy (PSP)

  • eye signs— can’t look up/down; eyes very wide

  • spasticity— trouble with movement

• Corticobasal Syndrome/degeneration (CBD)

  • apraxia (not in parkinson’s so may be PD+) — difficulty planning + carrying out movement

  • alien limb- limbs do things without conscious awareness; i.e. arm lifts up

  • asymetrical

• Multiple System Atrophy

  • MSA- Parkinsonism: falls, soft speech

  • MSA- Autonomic: excessive sweating, hypotension

  • MSA- Cerebellar: slurred speech

Huntington’s disease

• also basal ganglia disorder, but presents differently

• Huntington’s chorea is an autosomal dominant genetic movement disorder that induces dance-like/jerky involuntary movements

• progressive motor system dysfunction caused by neural brain cell death in basal ganglia

  • chorea — dance-like, wheel chair bound usually

  • rigidity

  • dementia

• presents between 30-50 years; prognosis - 17 years

• no cure

• dysarthria occurs in 90% of patients

• dysphagia and aspiration in 100% of patients

  • weight loss is first sign

• hyperkinetic = too much movement

Huntington’s disease: Signs and Symptoms, Oral phase

oral phase:

• abnormal rate of food consumption

• inadequate mastication

• lingual chorea; all over the place, hard to coordinate

• poor bolus formation and control

• respiratory stridor and dyscoordination

• delayed swallowing initiation

• repetitive swallows

• striater = writhing in tongue

Huntington’s disease: signs and symptoms pharyngeal phase:

• prolonged laryngeal elevation

• inability to stop respiration

• penetration/aspiration

• residue

Huntington’s Disease: Evaluation and Treatment

• VFS over FEES because they are moving a lot; tube down nose may cause them discomfort

• repeat every 12 months or as needed

• dysphagia therapy and compensatory techniques have been shown to be beneficial

• oral hygeine important

• PEG tube often recommended: progression is usually faster

  • consider pleasure feeds (for quality of life)

• can have DBS (deep brain stimulation) in Huntington’s too

ALS: bulbar

• dysphagia and dysarthria present first

• approximately 25% of all cases present with bulbar-onset form with dysarthria and dysphagia as the initial problem

• tongue is typically first bulbar muscle to show signs of involvement (particularly LMN— atrophies)

• faster progression than spinal onset ALS

ALS: Spinal

• spinal onset symptoms begin in limbs and trunk

• at first, speech and swallowing are preserved, but body movements decline first

ALS: impact of airway protective deficits

• aspiration pneumonia is a leading cause of death in ALS

  • swallowing and coughing dysfunction

• prognosis is better when patients are followed by multidisciplinary team

• VFS evaluation at baseline and then every 3-6 months as indicated; their progress is SO FAST

Management:

• SLPs have used compensatory or energy saving techniques- consider EMST results now (build strength in muscles)

• PEG tube, chin tuck, dietary modifications, small bites/sips, palatal lifts

• oral hygiene education/training- prevent infection

ALS dysphagia management

• suction toothbrush

• PEG tube (can still eat orally as long as they want to)

Considerations for a PEG tube

• consider overall nutrition/hydration of patient

• FVS (forced vital capacity)

• What is the current diet?

• How long does it take to eat?; if it takes too long, consider PEG tube

• Coughing/choking? how often? what consistencies?

• Fatigue: meals >30 mins?

• oral hygiene

• can the patient manage their own secretions?

• anxiety

• patient autonomy (are they existing or living on PEG tube)

• vital capacity: how much air do you have access to?

PEG education and counseling

• provide unbiased education about PEG early

• address misconceptions

• address what it is like to live with a PEG

• how intrusive is the PEG?

• are PO pleasure feeds possible?

• emphasize patient autonomy

• Care of PEG

• Cost of PEG

• QOL: studies suggest PEG tube does increase quality of life

LMN dysfunction ALS: Swallowing Signs and Symptoms- Oral Transit deficits

• solid food dysphagia may be 1st manifestation

• poor pressure generation

• poor lingual force and coordination

• nasal regurgitation

• lingual atrophy/fasciculations

LMN dysfunction ALS: Swallowing Signs and Symptoms- Pharyngeal deficits

• delayed pharyngeal transit

• reduced pharyngeal peristalsis

• penetration/aspiration

• marked residue throughout mechanism

• globus sensations

• esophageal transit deficits

• UES high pressure zone unable to function appropriately

Multiple Sclerosis (MS)

myelin: fatty tissue on axons that speed up impulses transmitted from neuron to neuron

• progressive demyelinating disease that involves central and peripheral nerves

  • conduction delays

  • eventual loss of function

• Dysphagia occurs in 24-43% of patients with mS but is more common as disability increases (80%)

• Lesions in the anterior insula or operculum (S1 and M1) are often involved in manifestation of dysphagia

• Dysfunction across oropharyngeal structures and UES

• one study found 40% of MS patients were silent aspirators

MS: Swallowing Impairments

• DYMUS: questionnaire for assessment of dysphagia

  • 10 items

• Commonality of dysphagia makes monitoring of these patients using VFS and FEES critical

  • generally every 6-12 months as indicated

• monitoring patients long-term

Dementia/Alzheimer’s Disease

• overall decline in intellectual function, memory, and motor skills involving sequenced activity

• AD results from deposition of protein near frontal cortex and motor circuits that impairs neural function

• Dementia can result from HD, Pick’s disease, LBD

• Delayed neuromuscular responses, xerostomia, decreased alertness

Swallowing Impairments in Dementia

• 1/3 of patients with dementia/AD aspirate (on VFS)

• aspiration pneumonia is a leading cause of death

Oropharyngeal dysfunction

• impairments to feeding

• food pocketing

• vocal fold immobility

• reduced cough effectiveness

• prolonged swallowing gestures

• poor oral hygiene

• malnutrition (don’t care as much, not paying attention)

Dystonia

• movement disorder characterized by sustained or intermittent contractions causing abnormal, often repetitive movements, postures, or both (think like hand cramp after writing)

• worsened by voluntary action

• Focal

  • oromandibular dystonia (affects mandible, hard to masticate, can feel stuck)

  • cervical dystonia (spasmodic toricollis)

  • Spasmodic dysphonia- ab and ADductor

• Segmental

• Generalized

• Dysphagia symptoms vary

Dystonia: SD- Spasmodic Dysphonia (to larynx)

• primarily affecting anxious individuals, possibly brought on by pressures and stress in the middle stage of life

• 3 types

  • adductor - most common

    • strained, spastic, tremulous vocal quality caused by hypercontraction of the TA

  • Abductor

    • weak, breathy vocal quality

  • Mixed - most rare

    • perceived as vocal stoppages

  • Preferred treatment = botox- injected into vocal folds

    • beware unskilled botox injection — temporary PEG tube placement

Peripheral Myopathy Etiologies of dysphagia

• cranial nerves, spinal nerves

• muscular junction level