Guillain-Barre SyndromeDMW Dharmakeerthi (MD)Senior Registrar in ClinicalNeurophysiology
• 1859, Landry published a report on 10patients with an ascending paralysis.• 1916, 3 French physicians (Guillain, Barré, andStrohl) described 2 French soldiers with-motor weakness-areflexia-albuminocytological dissociation
Clinical features• Weakness usually starts in the legs• In about 10 percent it begins in the arms or facial muscles• Need for ventilatory support develops in 10 to 30 percent• Facial weakness occurs in more than 50 percent• Oropharyngeal weakness eventually occurs in 50 percent• Oculomotor weakness occurs in about 15 percent• Paresthesias in the hands and feet accompany theweakness in more than 80 percent of patients, but sensoryabnormalities on examination are frequently mild.• Pain, typically located in the back and extremities, can be apresenting feature in 66 % of patients with all forms of GBS
• AIDP and the Miller Fisher -focal inflammatoryresponse develops against Schwann cells orperipheral myelin.• Infiltration of the epineural and endoneuralsmall vessels (mostly veins) by lymphocytesand monocytes causes segmental myelindegeneration throughout the nerve.• The inflammation is more intense at thejunction of the dorsal and ventral roots.
PRINCIPAL ANTI-GLYCOLIPID ANTIBODIES IMPLICATED INIMMUNE NEUROPATHIES
Electrophysiological tests• The most specific and sensitive tests for diagnosis ofthe disease Partial motor conduction block Slowed nerve conduction velocities Abnormal temporal dispersion Prolonged distal latencies
H-reflex• H-reflex was the single most sensitive test for early GBS,being absent in 97 percent tested within seven days of theonset of neurologic symptoms.• Monosynaptic reflex response - similar in pathway to thetendon jerk.• The electrical stimulus activates the Ia afferents and actionpotentials travel orthodromically to the spinal cord.• Ia afferents make excitatory monosynaptic connections tothe alpha motor neurons and a volley of action potentials isset up in the motor nerve that runs orthodromically theentire length of the nerve from the cell bodies to themuscle.
F-response• Recurrent firing of an anterior horn cell after ithas been invaded by an antidromic actionpotential.• To produce an F-response, an action potentialmust travel twice through the proximalsegment of the motor nerve.
The prognosis and main prognostic indicators of Guillain-Barrésyndrome A multicentre prospective study of 297 patients(Italy)• The chance of recovery was significantlyaffected by- age- antecedent gastroenteritis- disability- electrophysiology of axonopathy- latency to nadir- duration of active disease.
prognosis• Muscle Nerve. 2006 Jun;33(6):766-70Nagasawa K, Kuwabara S,Misawa S, Fujii K, Tanabe Y, Yuki N, Hattori T, Kohno Y.• 31 Japanese GBS -AIDP (35%)- AMAN (48%)-unclassified (16%).• By 6 months after onset, all the AIDP and 80% of the AMANchildren had regained the ability to walk.• By 2 years, all but one of the AMAN children could walk.• In Japanese childhood GBS, the proportion of AIDP and AMANappears to be similar.• Recovery is generally favorable in both subtypes, but some of theAMAN children experienced delayed recovery.
• Pediatr Neurol. 2003 Apr;28(4):295-9.(Turkey)• Retrospective study of 23- AIDP- 44%-AMAN- 35%- AMSAN- 21%• In the acute phase AMAN >AIDP (measured by GBSscores)• No significant difference at 6 months in GBS scoresbetween AIDP and axonal forms of GBS.• After IV IG therapy, children with axonal forms recovermore slowly than those AIDP, but outcome at 12months appears to be equally favorable in two groups.
McKhann GM, Griffin JW, Cornblath DR, et al(1988)• 245 patients• In the multivariate analysis, four factors correlate withpoorer outcomes:• Mean amplitude of compound muscle action potentialon stimulating distally of 20% of normal or less.• Older age.• Time from onset of disease of 7 days or less• Need for ventilatory support.• The most powerful predictor of outcome was theabnormal mean amplitude of compound muscle actionpotential on stimulating distally.
Rees JH, Soudain SE, Gregson NA, Hughes RA. Campylobacter jejuniinfection and Guillain-Barré syndrome. N Engl J Med 1995; 333:1374• 96 patients with GBS and 7 patients with MFS• Infection with C. jejuni often precedes theGuillain-Barré syndrome and is associatedwith axonal degeneration, slow recovery, andsevere residual disability.
Practice points• Diagnostic criteria for the diagnosis of GBS include progressive paralysis ofmore than one limb , areflexia and disease progression over less than fourweeks.• Patients should be monitored for deteriorating bulbar function, vitalcapacity, and for cardiac arrhythmia.• A raised cerebrospinal fluid white cell count should alert the clinician toalternative diagnoses.• Neurophysiology is helpful to subclassify disease and predict the outcome.• Trials of treatment show plasma exchange and IVIg to be equally effectivewhen given to non-ambulant patients in the first two weeks of disease.• There is no evidence supporting the use of corticosteroids in thetreatment of GBS.• The Miller Fisher variant of GBS is strongly associated with the presence ofanti-GQ1b antibodies, which are likely to be pathogenic.• About 25% of patients with the commonest type of GBS in the UK–AIDP–possess antiganglioside antibodies.