GUILLAIN BARRE SYNDROME (GBS) / Polyradiculoneuritis

-Symmetrical, bilateral, peripheral

polyneuritis characterized by
ascending paralysis

-Can occur at any age

-Affects women and men equally

-Cause unknown; but it sometimes

is triggered by a respiratory
infection or the stomach flu
=antecedent viral infection

-Inflammation usually affects the

myelin sheath=demyelination
Assessment findings

-Mild sensory changes; in some clients severe misinterpretation of

sensory stimuli resulting in extreme discomfort
-Clumsiness: usually first symptom
-Progressive motor weakness in more than one limb
(classically is ascending and symmetrical)
-Cranial nerve involvement (dysphagia)
-Ventilatory insufficiency if paralysis ascends to respiratory muscles
-Absence of deep tendon reflexes

Diagnostic tests

CSF studies: increased protein

EMG: slowed nerve conduction

Medical Management

Considered as medical emergency

and patient is managed in the ICU

Mechanical ventilation if respiratory

problems present

Plasmapheresis to reduce
circulating antibodies

Continuous ECG monitoring to

detect alteration in heart rate and
rhythm

Propranolol to prevent tachycardia

Atropine may be given to prevent

episodes of bradycardia during
endotracheal suctioning and
physical therapy.
Nursing interventions

-Maintain adequate ventilation.

-Check individual muscle groups every 2 hours in acute phase to check for
progression of muscle weakness.
-Assess cranial nerve function: check gag reflex and swallowing ability;
ability to handle secretions; voice.
-Monitor vital signs and observe for signs of autonomic dysfunction such
as
acute periods of hypertension fluctuating with hypotension, tachycardia,
arrhythmias.
-Administer corticosteroids to suppress immune reaction as ordered.
-Administer antiarrhythmic agents as ordered.
-Prevent complications of immobility.
-Promote comfort (especially in clients with sensory changes)
-Promote optimum nutrition.
Check gag reflex before feeding.
Start with pureed foods.
Assess need for nasogastric tube feedings if unable to swallow.
 -A disease of
UNKNOWN cause in
which there is loss of
motor neurons in the
anterior horns of
the spinal cord & the
motor nuclei of the
lower brain stem
-Affects more men than women
-Onset occurring
usually in the 5th or 6th
decade of life
 Pathophysiology
 
Motor neurons in the anterior horns
of the spinal cord and motor nuclei of
lower brainstem dies.

Muscle fibers that they supply
undergo atrophic changes.

Neuronal degeneration may occur in
both upper and lower neuron system

Leading theory: overexcitation of nerve

cells by neurotransmitter glutamate
leads to cell injury and neuronal
degeneration.
Dxtic tests:
diagnosed on the basis of the signs & sxs
no clinical or lab test are specific for this disease
EMG- may indicate reduction in the # of functioning motor units
MRI -may show high signal intensity in the corticospinal tracts
-differentiates it from a multifocal motor neuropahy
 
Symptoms:
Progressive weakness and atrophy of the muscles of the arms, trunk, or legs
Dysarthria, dysphagia
Spasticity
Fasciculations("muscle twitch")
-a small, local, involuntary muscle contraction visible under the skin
DTRs becomes brisk and overactive
Respiratory insufficiency
 
25% of patients: weakness starts in the muscles supplied b the cranial nerves
= difficulty talking, swallowing and ultimately breathing
 
Death usually occurs as a result of infection, respiratory failure, or aspiration and generally
occurs about 3 years after the onset of the disease. Few patients survive for longer periods.
 
 
 
Medical Management
-Drugs
> Riluzole (Rilutek)-glutamate antagonist; slows deterioration of motor neurons
> Baclofen (Lioresal)/ Diazepam (Valium) -used to control spasticity that interferes with ADL
> Quinine -relieve muscle cramps
-NGT feeding
-Cervical esophagostomy or gastrostomy
to prevent aspiration & for long-term nutritional support
-Mechanical ventilation if hypoventilation develops
 

Nursing interventions
Provide nursing measures for muscle weakness and dysphagia.
Promote adequate ventilatory function.
Prevent complications of immobility.
Encourage diversional activities; spend time with the client.
Provide compassion and intensive support to client/significant
others.
Provide or refer for physical therapy as indicated.
Promote independence for as long as possible.
===also called LOU GEHRIG’S DISEASE after the famous baseball player who suffered from it
HUNTINGTON’S DISEASE (HD) ; HUNTINGTON’S CHOREA (dance)
-A chronic, progressive, hereditary disease of the nervous system that results in
slow
progressive involuntary choreiform movement and dementia
-Onset occurs between the ages of 35 & 45 years, though 10% of patients are
children
-Affects men and women of all races
-Transmitted as an autosomal dominant genetic disorder: each child of a parent
w/ HD has a
50% risk of inheriting the illness
Pathophysiology

Premature death of cells in the striatum, (caudate &

putamen) of the basal ganglia (for control of movts)

+ loss of cells in the cortex (for thinking, memory,
perception & judgment)

+ loss of cells in the cerebellum (for coordination)

Cells’ destruction results in lack of GABA & AcH

A  in GABA & Ach (both excitatory
neurotransmitters leads to brisk, jerky, purposeless
movements, particularly the hands, face tongue and
legs which the
client is unable to stop
 
 
Accdg to researches, GLUTAMINE –
building block for protein abnormally
collects in the cell nucleus, causing cell
death
 
Reason that the protein destroys only
certain brain cells is UNKNOWN
Dxtic tests:
clinical presentation of characteristic sxs
(+) family hx
CT & MRI - may show atrophy of the caudate nuclei once the dse is well established
 
2 Main Symptoms:
 progressive mental status changes leading to dementia
=significant loss of intellectual abilities such as memory capacity, severe enough to
interfere
with social or occupational functioning
 choreiform movements (rapid, jerky movements) in the limbs, trunk & facial muscles
Other sxs:
-emotional disturbance: fits of anger, suicidal depression, impaired judgment & memory,
hallucinations, delusions & paranoid thinking
3 stages:
• Onset of neurologic or psychological sxs
• ng dependence on others for care
• Loss of independent functions
 
Death follows from complications such as choking,
fall, infection, pneumonia or heart failure and
generally occurs 10-20 years after onset of the
disease.
 
Medications:
1. Phenothiazine –blocks dopamine receptors
2. Reserpine –depletes presynaptic dopamine
3. Tetrabenezine –reduces dopaminergic transmission
Nursing interventions:
 Frequent assessment/ evaluation of patient’s motor signs
 Interact with the patient in a creative manner
 Learn how this particular patient expresses need and want

FI NAL STAG E S
Deborah Martin is in the final stages of Huntington's Disease.
She is in a wheelchair and rarely leaves the nursing home.
She can't walk, talk or feed herself.
 
A feeding tube has been keeping her alive for the last three+
years.
 
Shana Martin with her Mom .
She chatted, laughed and held her mother's hand.
 
"She doesn't make eye contact," Shana Martin said.
"She doesn't talk. She does seem more alert when we're
around but the final stages of Huntington's. . . .you are very
rigid.
You're just kind of curled up in a ball basically."
END