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Alteration in mobility R/T imbalance and poor coordination
 
The loss of postural and righting reflexes is a reflection of the cerebellar abnormalities. They occur earlier and progress much faster in MSA than PD.2 Daily exercises will maximize and maintain the patient’s flexibility, strength, balance and coordination skills. PT plays a major role in assisting patients to attain this goal which fosters independence and a sense of well being. Patients, especially if they live alone, should have an emergency response alarm in case of falls or injury. Assistive devices e.g. hand rail, tub bar, shower chair, allow a patient to retain independence in ADL’s. A walker definitely enhances safety when a person has bradykinesia and difficulty with balance and coordination. Simple cues e.g. take small steps in a circle formation rather than pivoting or when rising from a chair, go to the edge of the chair, lean forward, and use the arms of the chair to push upward. The patient should never grasp the walker and pull himself upright. These interventions relieve some of the family’s stress and worry concerning the patient’s safety.

Alteration in elimination R/T neurogenic dysfunction

ANS deterioration affects both the genitourinary system and bowel elimination. Urological symptoms in MSA usually precede neurological by years.2 In the early stage of MSA urologic symptoms are related to detrussor muscle hyperreflexia, but later hyporeflexia predominates.2 Initial clinical manifestations are urgency and frequency. Urodynamic studies will indicate the degree of muscle tone and patient’s ability to respond to a full bladder. This allows realistic goals to be determined. Electromyography (EMG) shows that many patients with MSA have abnormal external urethral sphincter tone related to nerve degeneration.8

Fluids should be limited in the evening to decrease nocturia, which increases risk for falls. Anti-spasmodic, anti-cholinergic medications e.g. oxybutrin (Ditropan). solifenacin (Vesicare), tolterodine (Detrol LA) reduce urgency, frequency and incontinence. Urinary retention is effectively managed by catheterization, using clean technique. Catheterizing just before sleep and first thing in the morning is effective in managing severe nocturia.
 
Male impotence is an early sign of MSA and may be managed with medications e.g. Sildenafil (Viagra), tadafil (Cialis), (both PDE5 inhibitor) or Yohimbine, which is an alphas-2 adrenergic blocker. Other approaches e.g. intracavernosal papaverine injections or penile implant are seldom selected by patients.

Constipation results from impaired autonomic function, minimal physical activity, decreased fluids and dietary factors.. Patients can lessen constipation by increasing fluids (e.g. warm prune juice, coffee), fiber and exercise, particularly walking after meals and abdominal muscle strengthening exercises. Bulk-laxatives, pericolace, lactulose, or more potent laxatives may be appropriate. Suppositories and/or enemas allow bowel regulation. It is important to avoid straining because the valsalva maneuver effects cardiac function, causing bradycardia and increasing orthostatic hypotension.

Alteration in sleep R/T obstructive sleep apnea

Patients with advancing MSA manifest respiratory stridor, inspiratory gasping, and dysrhythmic breathing and have periods of sleep apnea. Sleep studies are used to confirm the diagnosis of sleep behavior disorder (RBD) which is seen as increased electromyocgraphic (EMG) activity during REM sleep and active, sometimes combative movements that are associated with disturbing, unpleasant dreams. Upsetting dreams are effectively treated with clonazepam.9

Obstructive sleep apnea (OSA) becomes more severe in the latter stages of MSA. Loss of brainstem ventilator drive centers is thought to cause OSA, which is further enhanced by laryngeal dystonia. Patients with MSA show minimal response to hypoxia, particularly when sleeping, which may contribute to sudden death. One study showed that one-third of patients with MSA died suddenly.7 The use of C-PAP is very beneficial in treating apnea, increasing oxygen levels and allowing patients to have a restful, healthy sleep. This decreases fatigue, allows greater socialization and participation in activities.

Alt in self-esteem R/T dysphasia and isolation

With MSA hypophonia (low volume), monotonous speech and dysartria (poor articulation) are common speech abnormalities due to poor neuro-muscular function. In one study 100% of the patients with MSA-C had ataxic speech, which is described as monopitch, with irregular articulatory breakdown and bursts of loudness.10 Voice may be hoarse, if vocal cords remain in open position. A speech therapist’s evaluation will suggest appropriate exercises and devices that facilitate communication. Some patients find speech exercises very tiring and frustrating and prefer an alternative method of communicating e.g. pictures, communication board and/or lap top.
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