Guillain-Barré syndrome classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, MBBS [2]
Overview
Guillain-Barré syndrome (GBS) is an acute, autoimmune, polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by an acute infectious process. It is included in the wider group of peripheral neuropathies. There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, acute inflammatory demyelinating polyneuropathy (AIDP). It is frequently severe and usually exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes.
Classification
Six different subtypes of Guillain–Barré syndrome exist:
- Acute inflammatory demyelinating polyneuropathy
- Commonest form of GBS, and the term is often used synonymously with GBS.
- Caused by an auto-immune response directed against Schwann cell membranes.
- Commonly preceded by a bacterial or viral infection.
- Campylobacter jejuni is the commonest causative agent (positive in approximately 2 out of 5 patients).
- Peripheral nerve demyelination is present. Symptoms generally resolve with remyelination.
- Miller Fisher syndrome
- Accounts for approximately 5% of GBS cases
- Unlike Acute inflammatory demyelinating polyneuropathyit manifests as a descending paralysis
- It usually affects the eye muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia.
- The ataxia predominantly affects the gait and trunk, with the limbs relatively spared.
- Anti-GQ1b antibodies are present in 90% of cases.
- Acute motor axonal neuropathy (AMAN),[1] also known as Chinese paralytic syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. It is probably due to an auto-immune response directed against the axoplasm of peripheral nerves. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies[2] are present. Anti-GD3 antibodies are found more frequently in AMAN.
- Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but also affects sensory nerves with severe axonal damage. Like AMAN, it is probably due to an auto-immune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete.[3]
- Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathy. It is associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias. Frequently occurring symptoms include impaired sweating, lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, and constipation unrelieved by laxatives or alternating with diarrhea. Initial nonspecific symptoms of lethargy, fatigue, headache, and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of eyes, and disturbed micturition. The most common symptoms at onset are related to orthostatic intolerance, as well as gastrointestinal and sudomotor dysfunction (Suarez et al. 1994). Parasympathetic impairment (abdominal pain, vomiting, constipation, ileus, urinary retention, dilated unreactive pupils, loss of accommodation) may also be observed.
- Bickerstaff's brainstem encephalitis (BBE) is a further variant of Guillain–Barré syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski's sign. The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla are described in the literature. BBE despite severe initial presentation usually has a good prognosis. Magnetic resonance imaging (MRI) plays a critical role in the diagnosis of BBE. A considerable number of BBE patients have associated axonal Guillain–Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.
References
- ↑ McKhann GM, Cornblath DR, Ho T; et al. (1991). "Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China". Lancet. 338 (8767): 593–7. doi:10.1016/0140-6736(91)90606-P. PMID 1679153.
- ↑ Ho TW, Mishu B, Li CY; et al. (1995). "Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies". Brain. 118 ( Pt 3) (3): 597–605. doi:10.1093/brain/118.3.597. PMID 7600081.
- ↑ Griffin JW, Li CY, Ho TW; et al. (1995). "Guillain–Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases". Brain. 118 ( Pt 3) (3): 577–95. doi:10.1093/brain/118.3.577. PMID 7600080.