Guillian Barre Syndrome (GBS) is also called as ‘acute inflammatory demyelinating poly – radiculo – neuropathy’. GBS is defined as an acute, predominantly motor neuropathy, which progresses to its peak in less than 4 weeks.
Causes of Guillian Barre Syndrome
- Usually affects 20 – 50 years of age.
- Usually male predominate.
- In about 70% of patients, GBS precedes an upper respiratory illness or gastrointestinal illness. Infectious agents commonly identified as a precipitation feature are cytomegalovirus and campylobacter jejuni.
- Probably a cell mediated immune response to preceding infection is the pathogenesis of GBS.
Symptoms of Guillian Barre Syndrome
- Prodromal stage: It presents with headache, vomiting, slight fever, pain in the back and limbs.
- Latent period is few days to several days.
- Stage of paralysis: Some patients may directly present with this stage, initial symptoms being absent. Alarming clinical picture with motor weakness progressing to paralysis and various sensory disturbances is characteristic.
- Motor symptoms: Onset of paralysis is sudden or may be gradual. All four limbs may be paralysed simultaneously or first lower, then upper proximal and distal segment muscles are equally affected. Involvement of muscles of neck and trunk is seen.Loss of superficial and deep reflexes is seen.
- Sensory symptoms: Patients complain of radicular pain most commonly in the proximal portion of limbs with numbness and tingling. All forms of sensations may be impaired at periphery and may be associated with muscle tenderness.
- Cranial nerve paralysis: Facial and bulbar paralysis is common.
- Reflexes are usually depressed or absent.
- Symptoms of toxemia include fever, rash, albuminuria and leucocytosis.
Diagnosis of Guillian Barre Syndrome (GBS)
Cerebrospinal fluid (CSF) shows typical elevation of protein with normal cell count. Nerve conduction studies typical early findings are prolonged distal motor latencies in either upper or lower limbs.
Asbury’s Diagnostic Criteria: Features required for diagnosis are progressive motor weakness of more than one limb.
Areflexia: Features strongly supporting diagnosis are
- Clinical features
- Progression of symptoms over days up to 4 weeks.
- Relative symmetry of symptom.
- Mild sensory symptom.
- Cranial nerve involvement.
- Recovery beginning 2 – 4 weeks after progression stops.
- Autonomic dysfunction.
CSF picture:
- Elevated CSF protein after 1 week of symptoms.
- Cell count of CSF increased.
- Electro-diagnostic studies.
Development of Guillian Barre Syndrome
- Within a period of 3 weeks GBS progresses to maximum disability, often with complete quadriparesis (paralysis of four limbs).
- Recovery without significant disability occurs in about 80% of patients.
Management and treatment options for Guillian Barre Syndrome
- No specific therapy is known.
- Analgesics and hot packs are used as general measures.
- Corticosteroids for patients with bulbar symptoms or generalized weakness.
- Supportive therapy-intravenous (IV) or intra-gastric feeding may be necessary.
- Physiotherapy: Early active and passive movements. Splints to prevent foot and wrist drop.
- Plasma exchange.
- IV immunoglobulin as an alternative to plasma exchange.
- Liquorpheresis (CSF filtration) done in few cases. It is a technique to purify CSF from pathological factors (probably responsible to GBS).