Progressive Neurodegenerative Disease

What are the progressive neurologic disorders

Multiple Sclerosis, Guillain Barre Syndrome, Amyotrophic Lateral Sclerosis, and Parkinson’s disease

Upper Motor Neuron Dysfunction

control resting muscle tone

initiates voluntary movement

Upper Motor Neuron Dysfunction may result in

spasticity/weakness

brisk tendon reflexes (muscles react faster/stronger than normal)

extensor plantar response (abnormal neurologic reflex)

Lower Motor Neuron Dysfunction

can’t communicate with the muscles

muscle paralysis or paresis (including diaphragm)

Lower Motor Neuron Dysfunction may result in

areflexia (lack of reflexes)

flaccidity and atrophy

fasciculations (spontaneous muscle twitches)

Bulbar Dysfunction

PD and ALS

lower cranial nerves in brain stem

Bulbar Dysfunction may result in

dysarthria (slurred speech because of muscle weakness in the face)

dysphagia (difficulty swallowing)

facial/tongue weakness and poor secretion management

Multiple Sclerosis

chronic auto-immune disease of the central nervous system

inflammation (scarring) in areas of the CNS white matter; plaques

results in demyelination

What is the cause (etiology) of Multiple Sclerosis

unknown exact cause

immune mediated response (body’s immune system is attacking its own tissues)

genetic link trending

not one specific factor but possibly multi-factorical

What are the sensory symptoms of Multiple Sclerosis

vision changes, sensory deficits, weakness

diplopia, paresthesia, blurred vision, impaired balance

neuropathic pain, temperature sensitivity, and vestibular dysfunction

What are the motor/cognitive symptoms of Multiple Sclerosis

fatigue and muscle weakness

executive functioning, processing speed, memory, and attention

Multiple Sclerosis: Neurogenic bladder

incontinence, frequency, urgency

urinary tract infections and catheterization

Multiple Sclerosis: Neurogenic bowel

incontinence, constipation, and dietary management

How do you diagnose Multiple Sclerosis

symptom identification

neurologic exam

MRI to detect lesions (sclerotic areas in brain and spinal cord)

EMG to measure conduction along sensory pathways

What are the 4 main types of Multiple Sclerosis

benign, relapse remitting (non-progressive), relapse remitting (progressive), and primary progressive

Multiple Sclerosis: Benign

experiences 1 or 2 episodes of neurological deficits with no residual impairments (they resolve)

Multiple Sclerosis: Relapse Remitting (Non-progressive)

returns to the previous level of function after each exacerbation with no residual deficits

Multiple Sclerosis: Relapse Remitting (Progressive)

some residual impairment with each remission

unpredictable with varied patterns of exacerbation and remission

Multiple Sclerosis: Primary progressive

gradual decline and no distinct remission

Disease management for Multiple Sclerosis

disease-modifying drugs to reduce relapses

second-line medications to promote antibodies

Symptom management for Multiple Sclerosis

occupational therapy (energy conservation, activity modification, family/caregiver education)

medications to manage spasticity

antidepressants, pain medications

lifestyle changes and low impact exercise

Guillain Barre Syndrome

autoimmune condition where the immune system attacks peripheral nerves

causes demyelination

results in motor and sensory deficits

most people fully recover

What are the symptoms of Guillain Barre Syndrome

ascending weakness (start in legs and works its way up)

paresthesia and dysphagia

difficulty speaking and breathing

loss of reflexes (very extreme cases)

neuropathic pain (heightened sensitivity and aching, cramping, electrical pain)

What is the disease progression of Guillain Barre Syndrome

initial acute phase (hospitalization and acute symptoms)

plateau (no worsening of deficits and no evident recovery)

recovery (function restored)

What is the etiology of Guillain Barre Syndrome

exact cause is unknown

most cases follow a viral or bacterial infection

vaccines, surgery

What is the incidence of Guillain Barre Syndrome

increases with age

men > 50

Medical management of Guillain Barre Syndrome

immunotherapy

Intravenous immunoglobulin infusion (burst of antibodies)

plasma exchange

Rehabilitation for Guillain Barre Syndrome

strengthening and maintaining ROM (intentional manner to not over fatigue)

energy conservation (limit fatigue/lessen restrictive activity)

adaptive equipment/DME

family/caregiver education

Amyotrophic Lateral Sclerosis

fatal, progressive, degenerative motor neuron disease

Amyotrophic Lateral Sclerosis: Scarring locations

UMNs in the corticopspinal pathways

LMNs in the brainstem

Anterior horn cells in the spinal cord

Amyotrophic Lateral Sclerosis: Neurodegeneration of the brain

corpus callosum

frontotemporal lobes

extramotor cerebral regions

What is the etiology of Amyotrophic Lateral Sclerosis

sporadic (no genetic link and most common)

exposure to environmental toxins in conjunction with genetic risk factors

familial (genetically determined; only 10% of cases)

no known cause

What is the prevalence of Amyotrophic Lateral Sclerosis

age 55-75

males > females

How to diagnose Amyotrophic Lateral Sclerosis

differential diagnoses (ruling out other things)

MS, Neuropathy, Lyme Disease, and Spinal Cord injury/tumor

electromyogram

MRI or spinal tap to rule out other conditions

What are some clinically definite features of Amyotrophic Lateral Sclerosis

signs of UMN and LMH involvement in 3 regions

muscle twitching, fasciculations, atrophy, hyperreflexia, spasticity

What are the symptoms of Amyotrophic Lateral Sclerosis

always progressive

hand weakness, asymmetrical foot drop, progressive loss of muscle

spasticity, difficulty speaking, and dysphagia

progressive atrophy, temperature regulation, and cognitive impairment

What is Amyotrophic Lateral Sclerosis’ disease course?

within 2 years motor and communication deficits are typically present

fast and progressive

Later Stages of Amyotrophic Lateral Sclerosis

respiratory failure (mechanical ventilation/most common cause of death)

severe muscle paralysis

loss of speech and secretions

inability to eat or drink

Medical management of Amyotrophic Lateral Sclerosis

no treatment exists to adequately slow or stop disease

riluzole (can help stay in mild stage for longer)

mechanical ventilation for respiratory failure

feeding tube for nutrition management

Symptom management for Amyotrophic Lateral Sclerosis

aspiration risk reduction

pressure ulcer prevention

pain management and contracture prevention

Rehabilitation for Amyotrophic Lateral Sclerosis

energy conversation and activity pacing

ADL compensatory strategies

functional mobility (power wheelchair training)

family/caregiver support and education

community resources

Parkinson’s Disease

progressive movement disorder

weakened and damaged cells in the substantia nigra

impairs dopamine production

lewy bodies in damaged brain cells

neuron degenderation progresses beyond the substantia nigra and brain stem

What is the etiology and prevalance of Parkinson’s Disease

average onset at 60 years old

no cause identified (genetic link but its not the only determinant)

progression of symptoms is variable and non-linear

Parkinson’s Disease: Preclinical phase

degeneration of dopamine production, no symptoms

Parkinson’s Disease: Prodromal phase

minimal symptoms, symptoms not clear enough for a diagnosis

Parkinson’s Disease: Clinical phase

recognizable symptoms

What are the motor symptoms of Parkinson’s Disease

resting tremor, muscle rigidity, bradykinesia, and shuffling gait

What are some non-motor symptoms of Parkinson’s Disease

cognitive impairment, visual perception deficits, sleep distribution

hallucinations, monotone speech, flat affect, and emotional disorders

Stage 1 of Parkinson’s Disease

mild symptoms

minimal impact on ADLs and Unilateral tremor

Stage 2 of Parkinson’s Disease

bilateral tremor and rigidity

postural and mobility deficits

ADLs progressively more difficult

Stage 3 of Parkinson’s Disease

mid-stage

balance impairment (falls)

mild to moderate disability

Stage 4 of Parkinson’s Disease

severe disability

assistance with ADLs

use of assisted device for mobility

Stage 5 of Parkinson’s Disease

stiffness significantly limits mobility

assistance for all ADLs

Medical management for Parkinson’s Disease

no medications to prevent

dopamine replacement drugs (increase the uptake of dopamine in the brain)

medications to slow disease progression

effectiveness can decrease over time

Surgical management for Parkinson’s Disease

deep brain stimulation (electrical impulses to the brain)

high intensity focused ultra-sound (uses sound waves from MRI)

thalamotomy, pallidotomy (surgical excision of part GP or thalamus)

Rehabilitation for Parkinson’s Disease

neuromuscular interventions

ADL and IADL engagement

coordination interventions and fall prevention

family/caregiver education