2.6 . Degenerative Nervous System Disorders


Multiple Sclerosis (MS) is a chronic, progressive disease of the central nervous system characterized by the destruction of myelin. Myelin is the fatty and protein material that covers certain nerve fibers in the brain and spinal cord.

The cause of MS is unknown. Research is investigating the possibilities of infection by slow virus, alteration in the immune system, and genetic factors. MS primarily affects adults between 20 and 40 years of age.

Signs and symptoms include:


  • Weakness.
  • Visual disturbances (nystagmus, blurred vision, blindness).
  • Slurred, hesitating speech.
  • Intention tremor.
  • Abnormal reflexes (absent or hyperactive).
  • Ataxia.
  • Paraplegia.
  • Urinary and bowel incontinence/retention.
  • Emotional lability; (depressed, euphoric)

The objectives of medical and nursing management are to keep the patient as active and functional as possible in order to lead a purposeful life, and relieve the patient's symptoms and provide him/her with continued support.

It is equally important to instruct patient to perform muscle stretching exercises to minimize join contractures, with particular emphasis on hamstrings, gastrocnemius, hip adductors, biceps, wrist and finger flexors. Instruct the family about passive range of motion exercises for patients with severe spasticity, and advise patient to prevent muscle fatigue with frequent rest periods. Instruct patient to participate in walking exercises to improve gait affected by loss of position sense in legs.

You will administer muscle relaxants as ordered, and utilize braces, canes, crutches, walkers when necessary to keep patient ambulatory.

Advise the patient to avoid skin pressure and immobility. Pressure sores, for example, will accompany severe spasticity in an immobile patient due to sensory loss. So change patient's position every 2 hours even if the patient is in a wheelchair.

Give careful attention to sacral and perineal hygiene.

Meticulous skin care is required for the incontinent patient. Support the patient with bladder disturbances, observing patient closely for retention and catheterize, as ordered. Support the patient with bladder incontinence by initiating a bladder training program. In some instances you may need to instruct the patient for self-catheterization. Of course you should administer urinary antiseptics, as ordered.

Assist the patient to establish a routine of regular bowel evacuation. Administer corticosteroids, as ordered during periods of exacerbation. This may reduce severity of exacerbation by reducing edema and inflammation. Encourage bedrest during the acute stage as activity seems to worsen attack; and keep in mind that the residual effects of the disease may increase with each exacerbation.

Support the patient with optic and speech defects. Use an eye patch to block vision impulses for patient with diplopia, and obtain services of speech therapist.

Discharge planning considerations are to instruct patient and family in activities of daily living using assistive and self-help aids, and assist the patient and family to cope with the stress of multiple sclerosis.

The patient with MS will experience behavioral changes such as euphoria, depression, denial, and forgetfulness. So avoid physical and emotional stress as they may worsen symptoms, and assist the patient to accept his new identity as a handicapped person.


Amyotrophic lateral sclerosis (ALS) is a progressive, incapacitating, and fatal disease of unknown cause. It is characterized by loss of motor neurons in the anterior horns of the spinal cord and lower brain stem. Amyotrophic lateral sclerosis is commonly known as Lou Gehrig's Disease.

Signs and symptoms include:


  • Symptoms vary, depending upon the location of affected motor neurons.
  • Progressive weakness and atrophy of muscles of arms, trunk, or legs.
  • Progressive difficulty in speaking and swallowing, speech may be nasal and unintelligible.
  • Excessive drooling.
  • Muscle twitching.
  • Mental facilities are not affected.
  • Death usually occurs 3-5 years after onset.

The primary objective of medical and nursing management is to support the patient and improve quality of life.

It is important for the patient to perform active exercises and range of motion exercises to strengthen uninvolved muscles and prevent disuse atrophy. Instruct the patient on the use of braces, splints, canes, etc., to keep him mobile as long as possible.

Assist the patient to prevent complications that may result from symptoms:

Keep suction apparatus at bedside, as aspiration is a constant danger.

Instruct patient to drink and eat in an upright position with the neck flexed.

Use soft cervical collar if patient has difficulty holding head up.

Give semi-soft foods. Avoid easily-aspirated pureed foods and mucous producing foods (milk).

Keep in mind that patient may have frequent outbursts of laughing and crying.

Develop communication system when speech is lost.

Give patient and family compassionate and caring support. For instance, allow expressions of feelings and frustrations about losses and eventual outcome. And always remember (and instruct family members and other visistors) that the patient is alert and retains vision, ocular movement, intelligence, and consciousness even though he/she is paralyzed.

Advise patient's family of helping services of ALS Society of America.


Parkinson's disease is a progressive neurological disorder affecting the brain centers that are responsible for control of movement. Primary degenerative changes of the basal ganglia and their connections prevent motor transmission of automatic movements (blinking, facial expressions, muscle tone). The exact cause of Parkinson's is unknown. Suspected causes include genetic factors, viruses, chemical toxicity, encephalitis, and cerebrovascular disease.

Signs and symptoms include:


  • Bradykinesia, which usually becomes the most disabling symptom
  • Tremor which tends to decrease or disappear on purposeful movements
  • Rigidity, particularly of large joints
  • Classic shuffling gait
  • Muscle weakness which affects eating, chewing, swallowing, speaking, writing
  • Mask-like facial expression with unblinking eyes
  • Depression
  • Dementia

The objectives of medical and nursing management are built around drug therapy, physical therapy, rehabilitation techniques, and patient and family education..

You should encourage patient to participate in physical therapy and an exercise program to improve coordination and dexterity. Emphasize importance of a daily exercise program, and instruct patient in postural exercises and walking techniques to offset shuffling gait and tendency to lean forward. Encourage warm baths and showers to help relax muscles and relieve spasms.

Instruct patient to establish a regular bowel routine with a high fiber diet and plenty of fluids. Constipation is a problem due to muscle weakness, lack of exercise, and drug effects. Point out the importance of eating a well-balanced diet. Nutritional problems develop from difficulty chewing and swallowing and dry mouth from medications.

Encourage patient to be an active participant in his/her therapy and in social and recreational events, as Parkinsonism tends to lead to withdrawal and depression, and remind him about the American Parkinson's Disease Foundation for patient education and group support.


Myasthenia gravis is an autoimmune disorder affecting the neuromuscular transmission of impulses in the voluntary muscles of the body. In normal individuals, transmission of impulses from the nerve to the motor end plate of the muscle is accomplished by the transmitter substance acetylcholine.

Acetylcholine is released at the nerve ending and moves to the muscle end plate, causing muscle contraction. It is then broken down into acetate and choline by the substance cholinesterase. In myasthenia gravis, one of three physiological abnormalities may exist: (a) There may be too much cholinesterase present, and acetylcholine is destroyed too quickly, (b) There may be too little acetylcholine released from the nerve fiber, resulting in inadequate depolarization of the motor end plate, or (c) The motor end plate is not sensitive to the action of acetylcholine.

Signs and symptoms include:


  • Diplopia (double vision).
  • Ptosis (dropping of one or both eyelids).
  • Abnormal muscle weakness; characteristically worse after effort and improved by rest.
  • Sleepy, mask-like facial expression with difficulty smiling.
  • Speech weakness (high-pitched nasal voice).
  • Difficulty swallowing.
  • Choking, aspiration of food.

Medical and nursing management includes primary drug therapy (anticholinesterase drugs to enhance the action of acetylcholine at the myoneural junction). This drug must be given exactly on time to control symptoms. After initial medication adjustments are made, patient learns to take his medication according to his/her needs. Of course the patient needs explicit instructions regarding medications: actions, reasons for timing, dosage adjustment, and symptoms of overdosage and actions to take should such a crisis occur.

Further responsibilities for the caregiver:

Have mealtimes coincide with peak effect of anticholinergics, when ability to swallow is best.

Obtain medic alert bracelet signifying that patient has myasthenia gravis.

Wear an eyepatch over one eye (alternating from side to side) if diplopia occurs.

Control factors which lead to fatigue.

Emphasize importance of avoiding contact with individuals with colds or respiratory infections, since these conditions could be devastating to the myasthenic patient. Instruct patient to inform dentist of myasthenia condition since Novocaine is usually poorly tolerated.. And inform the patient to rest at frequent intervals and avoid fatigue.

Your need to manage myasthenic crisis may result from natural deterioration of the disease, emotional upset, upper respiratory infection, surgery, or steroid therapy. For example, the patient may be temporarily resistant to anticholinesterase drugs or need increased dosage; but on the other hadn, cholinergic crisis may result from overmedication with anticholinergic drugs.

With the onset of a myasthenic crisis, the patient must be placed in an intensive care unit for continuous monitoring of the patient's respiratory status. You shold be prepared to provide ventilatory assistance, endotracheal intubation, mechanical ventilation, if required. Also administer appropriate medications, as determined by patient's status and cause of the crisis, support patient's fluid and nutritional needs, as ordered and indicated by patient's condition, and give continued psychological support during crisis period, as patient is still alert.