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Immunopathogenesis and Treatment of Guillain-Barre Syndrome and Chronic Inflammatory Demyelinating Polyneuropathy

Elena Grebenciucova and Kourosh Rezania

Guillain-Barre Syndrome (GBS)

GBS represents a spectrum of polyneuropathies, which arise from immune-mediated attack on different myelin or axonal antigens of peripheral and/or cranial nerves. GBS is the most common cause of flaccid paralysis worldwide after the elimination of poliomyelitis [1]. GBS encompasses a spectrum of diseases (i.e., subtypes) with varied clinical manifestations, reflective of the target antigen of autoimmune attack (myelin vs. axon) as well as the location of immunopathology within the peripheral nervous system (nerve roots, plexi, distal nerves, cranial nerves). Besides the autoimmune etiology, the GBS subtypes share the acute to subacute onset and albu- minocytological dissociation in the CSF.

Subtypes of GBS have been defined based on the clinical manifestations, neurophysiological features, and presence of different antibodies to neural glycolipid components. Acute inflammatory demyelinating polyneuropathy (AIDP) constitutes the typical primarily demyelinating form of the disease. AIDP is the most common subtype of GBS in Europe and North America and is typically characterized by acute onset of flaccid, hypo-, or areflexic paralysis [1, 2]. The clinical course consists of progressive weakness within hours to days and maximum weakness and disability within 4 weeks. Muscle weakness (including proximal limb and respiratory) usually dominates the clinical presentation. However, sensory symptoms, usually a distal paresthesia, very often allow distinguishing AIDP from some of its mimics such as myasthenia gravis and botulism. Dysautonomia is prevalent in AIDP and is one of its life-threatening manifestations. A less common, atypical presentation, which is encountered in 8% of the patients, is paraparesis without arm weakness. [3] Patients with paraparetic GBS, however, usually have sensory symptoms and areflexia, as well as abnormal conduction studies in the upper

E. Grebenciucova • K. Rezania, MD (*)

University of Chicago Medical Center, Department of Neurology, Chicago, IL, USA e-mail: This email address is being protected from spam bots, you need Javascript enabled to view it

© Springer International Publishing AG 2017

A. Minagar, J.S. Alexander (eds.), Inflammatory Disorders of the Nervous System, Current Clinical Neurology, DOI 10.1007/978-3-319-51220-4_10

extremities [3]. Acute motor axonal neuropathy (AMAN) is the second most common form of GBS in North America and Europe, accounting for 6-78% of the cases, and the most common in China and Bangladesh [4]. AMAN patients have a purely motor picture (positive sensory symptoms in only 10% of patients). In contrast to AIDP, dysautonomia and cranial nerve involvement are rare, and deep tendon reflexes are often normal to brisk in AMAN [4]. AMAN is also associated with a more rapid progression early in the course, with earlier peak than AIDP (11.5 vs. 18 days) [5]. Acute motor and sensory axonal neuropathy is the third GBS subtype which has sensory involvement (in contrast to AMAN) and is characterized by less favorable recovery because of axonal degeneration. Miller Fisher syndrome (MFS), the fourth major subtype of GBS, accounts for 5-12% of the GBS cases [6]. MFS typically presents with a triad of ophthalmoparesis, ataxia, and areflexia, and the patients generally do not develop significant weakness or respiratory impairment and have a good prognosis. MFS by itself has different clinical subtypes: acute ataxic neuropathy (without ophthalmoplegia), acute ophthalmoparesis (without ataxia), and a variant with CNS symptoms such as hypersomnolence (Bickerstaff’s encephalitis) [1]. Yet another less common, local subtypes of MFS include pharyngeal-cervical-brachial variant, which is characterized by rapidly progressive weakness of oropharyngeal, cervical, and upper extremity muscles accompanied by areflexia of the upper extremities [7].

Examination of cerebrospinal fluid (CSF) demonstrates albuminocytological dissociation in all the variants of GBS. Another useful diagnostic test is nerve conduction study and abnormal nerve conduction study, which demonstrates segmental demyelination in AIDP and axonal neuropathy in AMAN, AMSAN, and MFS and its variants [8]. It should be noted that conduction block, which is characteristic for AMAN, is secondary to functional blockage of axonal salutatory conduction and not secondary to segmental demyelination, leading to the recommendation that at least two sets of nerve conduction studies over time to differentiate AIDP from AMAN [9].

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