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Symptomatology The typical case of GBS is readily identi ed Paresthesias and slight numbness in the toes and ngers are the earliest symptoms; only infrequently are they absent throughout the illness The major clinical manifestation is weakness that evolves more or less symmetrically over a period of several days to a week or two, or somewhat longer The proximal as well as distal muscles of the limbs are involved, usually the lower extremities before the upper (thus the older term Landry s ascending paralysis); the trunk, intercostal, neck, and cranial muscles may be affected later The weakness progresses in about 5 percent of patients to total motor paralysis with respiratory failure within a few days In severe cases the ocular motor nerves are paralyzed and even the pupils may be unreactive More than half of the patients complain of pain and an aching discomfort in the muscles, mainly those of the hips, thighs, and back; these symptoms are frequently mistaken for lumbar disc disease, back strain, and various orthopedic diseases A few describe burning in the ngers and toes and if this appears as an early symptom, it may become a persistent management problem Sensory loss occurs to a variable degree during the rst days and may be barely detectable By the end of a week, vibration and joint position sense in the toes and ngers are usually reduced; when such loss is present, deep sensibility (touch-pressure-vibration) tends to be more affected than super cial (pain-temperature) Reduced and then absent tendon re exes are consistent ndings Rarely, only the ankle re exes are lost during the rst week of illness At an early stage, the arm muscles may be less weak than the leg muscles, and in a few cases, they are spared almost entirely Facial diplegia occurs in more than half of cases, sometimes bilaterally at the same time, or sequentially over days Other cranial nerve palsies, if they occur, usually come later, after the arms and face are affected; infrequently they are the initial signs in a variant pattern of disease as described below Disturbances of autonomic function (sinus tachycardia and less often bradycardia, facial ushing, uctuating hypertension and hypotension, loss of sweating, or episodic profuse diaphoresis) are common in minor form, and only infrequently do these abnormalities become pronounced or persist for more than a week Urinary retention occurs in about 15 percent of patients soon after the onset of weakness, but catheterization is seldom required for more than a few days There are in addition numerous medical complications secondary to immobilization and respiratory failure, as discussed further on, under Treatment At the onset of illness, the temperature is normal, and lymphadenopathy and splenomegaly, if they occur, are related to a preceding viral infection Variants of the Guillain-Barre Syndrome (See Table 46-3) Por tions of the clinical picture frequently appear in isolated or abortive form and cause diagnostic confusion Whereas in most patients the paralysis ascends from legs to trunk, arms, and cranial muscles and reaches a peak of severity within 10 to 14 days, occasionally the pharyngeal-cervical-brachial muscles are affected rst or constitute the entire illness, creating dif culty in swallowing as well as neck and proximal arm weakness (Ropper, 1986) Ptosis, sometimes with ophthalmoplegia, may be added This pattern may persist without the development of limb weakness The differential diagnosis then includes myasthenia gravis, diphtheria, and botulism and a lesion affecting the central portion of the cervical spinal cord and lower brainstem A syndrome comprising virtual or complete ophthalmoplegia with ataxia and are exia that probably represents a variant of GBS was described by Fisher (and is called Fisher syndrome) A purely.

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Table 46-3 Variants of Guillain-Barre syndrome Regional Fisher syndrome of ophthalmoplegia, ataxia, and are exia Cervico-brachial-pharyngeal, often with ptosis Oculopharyngeal weakness Predominant paraparesis Bilateral facial or abducens weakness with distal paresthesias Ophthalmoplegia with GQ1b autoantibodies Functional Generalized ataxia without dysarthria or nystagmus Pure sensory Pure motor Pandysautonomia Axonal

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ophthalmoplegic form also exists; it may be coupled with the pharyngeal-cervical-brachial pattern mentioned earlier The ophthalmoplegia, whether occurring alone or with weakness or ataxia of other parts, is almost uniformly associated with a speci c antineural antibody, anti-GQ1b The ophthalmoplegic pattern raises the possibility of myasthenia gravis, botulism, diphtheria, tick paralysis, and basilar artery occlusion Bilateral but asymmetrical facial and abducens weakness, coupled with distal paresthesias or with proximal leg weakness, are other fairly common variants in our experience (Ropper, 1994) The tendon re exes may be absent initially only at the ankles or at the knees Lyme disease and sarcoidosis are then considerations in diagnosis Paraparetic, ataxic, and purely motor or purely sensory forms of the illness have also been observed Less dif culty attends these diagnoses if paresthesias in the acral extremities, progressive reduction or loss of re exes, and relative symmetry of weakness appear after several days of signs The laboratory tests that af rm the diagnosis of typical GBS give similar but generally milder abnormalities if they are carefully sought in all these variant forms There has been a recent tendency to separate a group of cases with presumed diffuse axonal damage on the basis of an abrupt and explosive onset, severe paralysis, minor sensory features, and the electrophysiologic nding of inexcitability of nerves This axonal type of GBS represents 5 percent (or fewer) of cases but its identi cation has some value in that a poor response to treatment and very protracted recovery are to be expected in most cases This variant is described later In a few patients the weakness continues to evolve for 3 to 4 weeks or even longer From this group a chronic form of demyelinative neuropathy (chronic in ammatory demyelinating polyneuropathy, or CIDP) often emerges and an intermediate group that progress for 4 to 8 weeks and then improve can be identi ed (see further on) Acute Axonal Form of Guillain-Barre Syndrome This subject has been alluded to earlier Feasby and colleagues drew attention to an acute are exic polyneuropathy clinically similar to GBS but characterized pathologically by widespread and severe axonal degeneration In their initial report they described ve patients, all with a rapid evolution of polyneuropathy and very slow and poor recovery Unlike the common form of GBS, muscle atrophy in these patients became apparent relatively early in the disease (within weeks) The de ning EMG features were the presence of.

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