Guillain-Barré Syndrome (GBS) is a rare, serious and potentially fatal auto-immune disorder which affects between one and two in every 100,000 people per year of all races, genders and age groups. There is no cure and the exact cause or trigger of the syndrome is unknown, although it is linked to various acute respiratory and gastrointestinal viral and bacterial infections, vaccinations, child-birth and post-surgery. There exist four types of the GBS, acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor-sensory axonal neuropathy (AMSAN) and miller fisher syndrome (MFS).
With GBS, an individual’s immune system malfunctions and inappropriately attacks components of their peripheral nervous system (PNS). The peripheral nervous systems compromises of all the nerves in the body outside of the central nervous system (CNS – the spinal cord and the brain). Axons (bundles of nerve fibres that transmit nerve signals) and the myelin sheath (the insulating cover that surrounds the axons and increases the speed at which a nerve signal travels) transmit nerve signals between the CNS and the PNS which facilitates the function, complex movement and behaviours of the body’s limbs and skin. When the immune system malfunctions, the axons and the myelin sheath are damaged or degenerates resulting in delays and/or disruption of the transmission of nerve signals. As a result, the brain is unable to communicate with the body’s limbs and skin thereby affecting muscle movement and sensory signals such as temperature, touch and pain; the resulting symptoms include blurred vision and dizziness, numbness or tingling in the fingers and toes, severe lower back pain, uncoordinated movements, areflexia (loss of reflexes), sensory loss, progressive muscle weakness and/or paralysis in the legs, arms, torso and face and the in-ability to feel sensations. In some cases of GBS, the autonomic nervous system (ANS – the part of the nervous system that regulates the internal organs and controls breathing, the heartbeat, blood pressures and the digestive processes) is also compromised resulting in difficulty swallowing and breathing, abnormal heart-rates, changes in kidney and liver function and fluctuations in blood pressure and body temperature.
GBS is diagnosed subsequent a physical examination and on consideration of varying signs and symptoms which follow a specific pattern. The diagnosis can be confirmed by a lumbar puncture (spinal tap) – (with GBS the cerebrospinal fluid that surrounds the spinal cord and brain will contain higher than normal levels of protein), an electromyography (EMG – a nerve function test that analyses electrical activity from the muscles) or a peripheral nerve conduction study (PNCS – a test that analyses the responsiveness of nerves and muscles to electrical pulses).
The condition is primarily treated by Plasmapheresis (whole blood is removed from the body, processed to separate the red and white blood cells from the plasma thereby purifying it of harmful antibodies and returning only the blood cells to the body) or Intravenous Immunoglobulin (administering high doses of protein which reduces the severity of the attack to the nervous system). Critically important to any treatment protocol for GBS is maintaining an individual’s body functions with the help of life support systems (mechanical ventilator and heart monitor) while preventing secondary complications like pneumonia, bed sores or venous sludge (the build-up of red blood cells in veins, reduced blood flow and deep vein thrombosis in the limbs).
The severity of the disorder and its duration varies significantly in each case. The disorder is characterized by phases. In the first phase, there is rapid deterioration in the signs and symptoms of the disorder until they peak (reach the most severe point). In the second phase, called the plateau, signs and symptoms stabilize and do not change until the final or recovery phase where symptoms improve. Estimates vary but the recovery time from the initial onset of the symptoms is between 6 weeks to 3 years with approximately 80% of people recovering completely. The remaining 20% include people who remain severely disabled to those with only minor residual effects such as fatigue, muscle weakness and pain. Only 30% of people afflicted with GBS will need ventilatory support. The mortality rate is between 3% and 7% and only 5% of all persons affected by GBS will experience a complete relapse.
GUILLAIN-BARRÉ SYNDROME (GBS) – MY DIAGNOSIS
My diagnosis was a severe case of the GBS variant acute motor axonal neuropathy (AMAN), subsequent to either Denger Fever, the Chikungunya or Zika Virus (virus transmitted by mosquitoes which typically causes mild to severe infections in humans). The illness presented with acute, ascending and flaccid paralysis of the legs, arms, torso, respiratory and facial muscles, motor axonal degeneration (axon death and subsequent breakdown, with secondary breakdown of the myelin sheath) and areflexia (loss of reflexes). Prolonged use of life support systems was required. The treatment protocol was Intravenous Immunoglobulin. Nine weeks elapsed between the initial onset of symptoms and the recovery phase. My recovery is on-going.