Guillain-Barre Syndrome

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Limb wasting in Guillain-Barre syndrome - A rapidly progressive, acute inflammatory demyelinating polyneuropathy characterized by muscle weakness and paralysis of the extremities and possible respiratory paralysis with abnormal sensation and loss of reflexes.

Causes and Incidence The cause is unknown, but Guillain-Barre syndrome (GBS) is hypothesized to be an autoimmune disorder involving sensitization of peripheral nerve myelin. It is thought to be connected to a previous nonspecific infection and has been associated with inoculation for the swine flu. The incidence of GBS in the United States is 1.7 per 100,000 individuals, and the disorder occurs across age and gender lines.

Disease Process Mononuclear cells infiltrate the peripheral nervous system and set up an inflammatory response in the blood vessels of the cranial and spinal nerves. Demyelination of the peripheral nerves results, causing muscle weakness that begins in the lower extremities and ascends through the body in a symmetric fashion. Respiratory paralysis and facial weakness occur in 30% to 40% of cases. In some cases axonal destruction can cause atrophy in distal muscles and permanent neurologic impairment.

Symptoms The first sign is symmetric muscle weakness in the distal extremities accompanied by paresthesia. This weakness spreads upward to the arms and trunk and then to the face. This ascension usually peaks about 2 weeks after onset. Deep tendon reflexes are absent. Difficulty chewing, swallowing, and speaking may occur, and respiratory paralysis may develop. Bladder atony, postural hypotension, tachycardia, and heart block may be seen. Deep, aching muscle pain is also common.

Potential Complications About 5% of affected individuals die of respiratory failure. Another 10% have permanent residual neurologic deficits. About 90% of survivors make a full recovery, but the recovery time may be as long as 3 years.

Diagnostic Tests The diagnosis is based on the clinical presentation and cerebrospinal fluid samples, which show an increase in protein without an increase in lymphocyte count. Electromyography produces abnormal nerve conductionresults.

Treatments

Surgery
Tracheostomy to provide ventilation in the event of respiratory failure.

Drugs
Immunoglobulin given IV to counteract neurologic defect; narcotic analgesics for pain; prophylactic antiinfectives

Corticosteroids are contraindicated because they worsen the ultimate outcome.

General
Plasma exchange to speed recovery of neurologic deficit; respiratory monitoring and mechanical ventilation for respiratory paralysis; cardiac monitoring for sinus tachycardia, bradyarrhythmia; communication systems if ventilator is used or with facial paralysis; passive range-of-motion exercises; turning to prevent contracture and skin breakdown; rehabilitation to aid neurologic recovery; counseling and support of individual and family for long-term adaptation.

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Irritable Bowel Syndrome

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- A noninflammatory motility disorder of the large bowel that alters bowel habits and causes abdominal pain and distention.

Causes and Incidence The cause of irritable bowel syndrome (IBS) is unknown, but it is associated with diet, drugs, toxins, gastrointestinal (GI) hormones, prostaglan-dins, and emotional factors. IBS is a common GI disorder that accounts for about half of all presenting GI complaints in the United States. Women are affected more often than men, and whites and Jews more often than other ethnic groups. All age groups are affected, although the disease is predominant in those under 35 years of age.

Disease Process The pathophysiology of IBS is still unclear. However, two patterns can be identified: one with painful constipation and diarrhea and the other with painless diarrhea. Hypermotility with high-amplitude pressure waves is present in painful IBS, and hypomotility in painless IBS. Myoelectric activity is increased in both patterns, as is contractile activity after meals.

Symptoms The primary symptoms are either painless, urgent diarrhea that occurs after meals, or alternating diarrhea and constipation accompanied by abdominal pain, bloating, flatulence, headache, and fatigue.

Potential Complications IBS is associated with an increased risk of diverticulitis and colon cancer.

Diagnostic Tests A careful history of bowel habits and emotional stimuli, along with a rectal examination that elicits pain in a tender rectum, is important. Manometric studies are done to evaluate electrical response, as are tests to rule out other bowel diseases.

Treatments

Surgery
None.

Drugs
Anticholinergics to reduce pain; bulk-forming agents, antidiarrheal drugs to regulate stool.

General
Highfiber, lowlactose, caffeine-free, low-fat diet; counseling for emotional effects.

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Organic Mental Syndromes (Organic Brain Syndrome)

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- A constellation of behavioral signs and symptoms associated with transient or permanent dysfunction of the brain and characterized by impaired intellectual functioning, confusion, and agitation. The most common categories and the ones discussed here are delirium and dementia. Delirium is a reversible, self-limiting condition characterized by a reduced ability to maintain attention to or appropriately shift attention among external stimuli. Dementia is a structurally caused, permanent decline in memory, abstract thinking, and judgment.

Causes and Incidence Delirium most often occurs as a result of withdrawal from intoxication in chronic alcohol and barbiturate abusers and in acute inflammatory disorders such as meningitis and encephalitis. The most common cause of dementia is Alzheimer’s disease; other causes include vascular disease, HIV infection, central nervous system infection, severe head injury, toxic metabolic disturbances, normal pressure hydrocephalus, underlying neurologic disease (parkinsonism, Huntington’s chorea, multiple sclerosis, Pick’s disease), and drug, alcohol, or nutritional abuse. More than 1 million individuals in the United States have dementia, and the elderly are at greatest risk. As the incidence of AIDS increases, the incidence of dementia is expected to increase (an estimated 50% of individuals with end-stage AIDS develop dementia).

Disease Process The pathophysiology of organic mental syndromes is not yet understood. Pathologic changes vary by causation, and in Alzheimer’s-related dementia include atrophy of brain tissue with wide sulci and dilated ventricles, senile plaque formation, and neurofibrillary tangles. Vascular disease–induced dementia is characterized by multiple cerebral infarcts. In AIDS-related dementia, the neurons are infected with HIV, and in hydrocephalus, cerebrospinal fluid circulation and absorption are impeded.

Symptoms

Delirium
Rapid onset; disorientation, including loss of self-recognition in some instances; impaired memory; inability to maintain or shift attention; irritability, agitation, restlessness, hyperactivity; perceptual disturbance, hallucinations, delusions; rambling, fragmented speech; impaired

sleep-wake cycle; lucid intervals, symptoms worse at night; duration about 1 week on average

Dementia
Symptoms vary widely, but the overall picture is a slow, insidious disintegration of personality and intellect with impaired insight and judgment and loss of affect; memory impairment is often the most prominent initial symptom, and others include increasing rigidity of thought; restricted interests; easy distractibility; lack of initiation; speech disturbances; loss of impulse control; change of former traits or exaggeration of those traits (e.g., a neat person becomes slovenly or becomes obsessively preoccupied with orderliness); depression

Potential Complications Delirium may lead to dementia. Dementia (except that caused by trauma) is progressive; the individual eventually becomes totally oblivious to his or her surroundings and ultimately dies. Individuals with dementia are more susceptible to accidents and infection.

Diagnostic Tests The diagnosis of delirium is based on the clinical presentation, particularly the fluctuation of symptoms with periods of lucidity, and a history of one or more etiologic agents. Electroencephalography shows a generalized slowing of background activity. A diagnosis of dementia is warranted with demonstrable impairment of long- and short-term memory and demonstrable disturbances in abstract thinking, judgment, personality, or other higher cortical functions that interfere with social activities and relationships. Attention and arousal tend to be normal in dementia, and manifestations are relatively stable, worsening over time. A definitive diagnosis is available only on autopsy.

Treatments

Surgery
None.

Drugs
Delirium: withdrawal of toxic agents (alcohol, barbiturates) and IV sedation with antianxiety agents for agitation, seizure activity, and tremors Dementia: treatment of underlying disorders; antianxiety agents as disease progresses to relieve anxiety and frustration.

General
Delirium: adequate fluid and electrolytes; seizure precautions; safety precautions (e.g., to prevent wandering, climbing over bedrails); long-term treatment for substance abuse when it is the etiologic agent Dementia: kept in familiar surroundings with minimal environmental changes; use of frequent orientation devices (clocks, calendars, schedules, memory books, name tags); encouragement to do familiar, repetitive routines; safety precautions to prevent wandering; use of adult day care, respite care, or home care to relieve caregiver; family support groups and counseling; prevention of disuse syndrome in end-stage disease.

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