Chronic Neurologic Disorders

multiple sclerosis (MS)

• progressive demyleniation of neurons in the brain, spinal cord, and cranial nerves

• different types of MS

  • variation in effects, severity, and progression

• loss of myelin interferes with conduction of impulses in affected fibers

  • may affect motor, sensory, and autonomic fibers

  • occurs in diffuse pathches in the nervous system

• onset usually between ages 20 -40

• cause unknown

  • may be an autoimmune disease

  • may be nutritional deficit

  • may be change in blood flow to neurons

• may have genetic, immunologic, and enviromental compononents

• increased risk for close relatives of affected individuals

MS - earliest lesions

• inflammatory response

• loss of myelin in white matter of brain or spinal cord

MS - plaques

• larger areas of inflammation and demyleniation

• develop later, become visible

  • frequetntly beside the lateral ventricles, brainstem, and optic nerve

• plaques vary in size

  • several may coalesce into a single patch

Ms - recurrence

• initial inflammation may subside

• neural func may return to normal for short period of time

• in time, neural degeneration becomes irreversible

  • func is permanently lost

  • each recurrence causes additional areas of CNS to become affectsMS varies in severity

MS - signs and symptoms

• early

  • manifestations determined by areas of demyelination

  • blurred vision; weakness in legs

  • diplopia (double vision); scotoma (spot in visual field)

  • dysarthria

  • paresthesia, numbness, burning, tingling

• late

  • progressive weakness and paralysis extending to the upper limbs

  • loss of coordination; bladder, bowel and sexual dysfunc; chronic fatigue

MS - distribution of lesions

MS - diagnostic tests and treatment

• diagnostic tests

  • no definite test

  • MRI for diagnosis and monitoring

• treatment

  • no definitive treatment approved at this time

  • several research trials in progress

  • therapy includes physical therapy, occupational therapy

  • manifestations require individual attention

parkinsons disease

• progressive degenerative disorder

• dysfunc of the extrapyramidal motor system

  • progressive degeneration in bassal nuclei

  • imbalance between excitation and inhibition in basic nuclei

  • excess stimulation affects movement and posture

    • resting tremors

    • muscular rigidity

    • difficulty initiating movement

    • postural instability

Parkinsons disease - primary/secondary

• primary or idiopathic Parkinsons disease

  • usually develops after age 60

• secondary parkinsonism due to

  • encephalitis

  • trauma - ex: sports injuries

  • vascular disease

  • drug induced (phenothiazine tranquilizers

Parkinsons disease - early signs and symptoms

• fatigue

• muscle weakness, muscle aching

• decreased flexibility

• less spontaneous changes in facial expression

• tremors in the hands at rerst, repetitive “pill rolling” motions of hands

Parkinsons disease - later signs and symptoms

• tremors affect hands and feet, face, tongue, lips

• increased muscle rigidity

• difficulty initiating movements

• slow movements

• lack of associated involuntary movements

• characteristic standing posture is stooped leaning forward

• propulsive gait

• complex activities become slow and difficult

Parkinsons disease - what they look like

Parkinsons disease - other affected funcs

• low voice, devoid of inflection

• dysarthria

• chewing and swallowing become difficult

  • prolonged eating time

  • reccurent drooling

• face might resemble a mask

• blinking of eyelids reduced

• blank staring face

• impairs communication

• autonomic dysfunc

  • urinary retention

  • constipation

• orthostatic hypotension

  • threat of falls increases

• urinary tract and respiratory tract infections are common comps

• dementia develops late in course of disease

Parkinsons disease - treatment

• removal of cause if known

• dopamine replacement therapy

  • levodopa - dopamine precursor

• anticholinergic drugs

• speech and language pathologist

• PT

• OT

  • improve balance, coordination

• monitoring and treatment of respiratory and UTIs

amyotrophic lateral sclerosis (ALS)

• also referred to as Lou gehrig disease

• no identified cause

  • genes on various chromosomes have been linked to the disease

• progressive degerative disease affecting upper motor neurons in the cerebral cortex and lower motor neurons in brain stem and spinal cord

• no idication of inflammation around the nerves

• cognition unimpaired

• loss of upper motor neurons in cerebral cortex

  • spastic paralysis and hyperreflexia

• damage to lower motor neurons

  • flaccid paralysis

  • decreased muscle tone and reflexes

• progressive muscle weakness and loss of fine motor coordination

  • stumbling and falls are common

• death occurs due to respiratory failure

ALS - treatment

• no specific treatment to slow degeneration

• stem cell therapy under investigation

• riluted (drug) to slow further damage to neurons

• moderate exercise and rest

• RT, appropriate nutrition, speech pathology, OT, PT, psychological treatment

Myasthenia gravis

• autoimmune disorder

• auto antibodies to ACh receptors form

  • destruction of receptor site

    • prevention of further muscle stimulation

  • skeletal muscle weakness

  • facial and occular muscles usually affected first

  • not dysphagia and aspiration are significant problems

Myasthenia gravis - diagnostic tests and s/sx

• diagnostic tests

  • electromyography

  • serum antibody test

• s/sx

  • muscle weakness in face and eyes

  • impaired vision; monotone speech

  • difficulty chewing and swallowing

  • head droops; arms become weaker

  • upper respiratory infections common

Myasthenia gravis - treatment

• anticholinesterase agents

  • temp improvement of neuromuscular transmission

• glucocorticoids

  • suppression of immune system

• plasmaphoresis

  • removal of antibodies from the blood

• thymectomy

huntington disease

• inherited disease

  • autosomal dominnat gene

    • carried on chrom 4

• does not usually manifest until 40+ years

• progressive atrophy of brain

  • particularly in basal ganglia (nuclei) and frontal cortex

• depletion of GABA in the basal nuclei

• levels of ACh in brain appears to be reduced

huntington disease - s/sx, diagnostic tests, treatment

• s/sx

  • mood swings, personality changes

  • restlessness, choreiform movements in arms and face

• diagnostic tests: DNA analysis

• treatment

  • currently no therapy to slow progress of disease

  • symptomatic therapy only

dementia

• progressive chronic disease

  • cortical func decreased

    • impaired cognitive skills

    • impaired thinking, judgment and learning

    • memory loss

    • confusion

    • behavioral and personality changes

• many causes of dementia

  • vascular disease

  • infections

  • genetic disorders

alzheimer disease (AD)

• progressive cortical atrophy

  • neurofibrillary tangles and plaques

  • ACh deficit due to loss of neurons

• no definite diagnostic tests available

  • exclusion of other disorders

  • careful medical and psychological history

• specific cause unknown

  • repetitive DNA sequences on different chromosomes have been associated with AD

AD - S/sx

• signs and symptoms extend over 10-20 yrs

• early

  • behavioral changes

    • irritability, hostility, mood swings

  • gradual loss of memory and lack of concentration

  • impaired learning, poor judgment

  • decline of cognitive func, memory, and language

• late

  • change in food intake

  • inability to recognize family, lack of enviromental awareness, incontinence, inability to func

AD - treatment

• no specific treatment

• anticholinesterase drugs

  • some temp improvements

• OT, psychologists, speech therapy

• team approach needed to support client and caregivers

vascular dementia

• Caused by cerebrovascular disease

• Often a result of multiple small brain infarctions

• Common in persons older than 70

  • Especially in those with hypertension

• Onset insidious

  • Memory loss, apathy, inability to manage daily routines

• Progression may be in stages.

• Other neurologic impairment is common.

Aids dementia

• Common in later stages of AIDS

• Virus invades brain tissue.

• May be exacerbated by other infections and tumors

• Gradual loss of memory and cognitive ability

• Impaired motor function

• Children with congenital HIV infection

  • Brain frequently affected

  • Severe developmental delay