Friday, April 24, 2009

Guillain-Barré Overlap syndrome: Signs & Symptoms

Signs & Symptoms:

People with GBS suffer from a wide range of symptoms. Symptoms occur in the context of each patient's unique pattern of focal deficit. Individuals with GBS may develop symptoms rapidly. Symptoms may include varying degrees of weakness and numbness in the legs and arms. Symptoms of GBS can develop over the course of hours or days, or it may take up to three to four weeks. Some patients with GBS overlap syndrome may exhibit symptoms of meningitis or meningoencephalitis. A typical pattern includes an insidious onset of symptoms over a period of hours to several days during the late spring.

The disease is characterized by weakness which affects the lower limbs first, and rapidly progresses in an ascending fashion. Patients generally notice weakness in their legs, manifesting as "rubbery legs" or legs that tend to buckle, with or without dysthesias (numbness or tingling). As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected. Frequently, the lower cranial nerves may be affected, leading to bulbar weakness, (oropharyngeal dysphagia, that is difficulty with swallowing, drooling, and/or maintaining an open airway) and respiratory difficulties. Most patients require hospitalization and about 30% require ventilatory assistance. Facial weakness is also commonly a feature, but eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant.

The constilation of symptoms in GBS are marked general malaise, loss of appetite, nausea, vomiting and stomach pain, accompanied by weakness, ataxia and difficulty ambulating due to unsteadiness, tired feeling, and chills. Fever is low-grade or absent at onset.Presenting symptoms of GBS are acute progressive weakness which may include pain (especially back or joint pain), paresis of the extremeties, cranial nerve compromise, ataxia, and associations of different types of symptoms, or overlaping spectrum of unusual presenting or flu-like symptoms and/or atypical variants of GBS. In certain cases, there may also be evidence of brain involvement indicated by lethargy and migraine headaches.

Neurological examination may reveal "no focal deficits". Patients may present with facial weakness mimicking Bell palsy; together with varying degrees of weakness and/or unsteadiness. Patients often appear to be nauseated and in a state of total exhaustion, marked by a haggard appearance. Neurological examination may be otherwise unremarkable.

Mild cases of Guillain-Barre syndrome may present only with ataxia (difficulty in walking and balancing), whereas severe cases may present with difficulty in respiration due to paralysis of the respiratory muscles and cranial nerves. Involvement of facial, oropharyngeal, and ocular muscles results in facial droop, dysphagia and dysarthria; a mournful or expressionless face and drooping eyelids is caused by facial muscle paralysis; alterations in speech patterns or dysarthria is also attributed to muscle weakness.

Speech may be unintelligible, "slurred" or whispery with GBS, as the various muscles required to form speech are weakened. Vocal cord paralysis is a prominent finding in patients with GBS and there can be a slight to dramatic voice change as the vocal chords become affected. Severe forms of GBS may result in urinary or fecal incontinence. A history of grossly bloody stools may be observed; fecal leukocytes are usually present.

Cues & clues: Change in pitch range of the voice; Speech difficulties, ie. Slurred speech; Facial weakness, dysphasia or dysarthria; early severe ptosis "drooping eyelid," with or without other signs of oculomotor weakness; fatigue, pain and muscular weakness. Fatigue is a major complaint in patients with immune mediated polyneuropathies and fatigue can be one of the most disabling symptoms for GBS patients who often experience excessive fatigue, or complain of being very tired. GBS may herald an onset of sleepiness in a subset of patients, presenting with mild to moderate fatigue, to "sleep drunkenness" in which the patient often appears to be "sedated" due to extreme somnolence.

A recent history of increasing or "diffuse muscle weakness" followed by paralysis suggests a typical presentation of Guillain-Barre syndrome, especially if there was a recent illness, or infection. In about 50 to 60% of cases there is a history of a recent viral or other infection. Symptoms of GBS get worse very quickly. The clinical features of GBS can range from asymptomatic to life threatening. It may only take a few hours to reach the most severe symptoms. The disorder can develop over the course of hours or days, or it may take up to 3 to 4 weeks. The consequences of a missed diagnosis and delayed treatment can be catastrophic.

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