Guillain-Barre Syndrome — DVA claim example

Diagnostic Assessment — Guillain-Barre Syndrome

Example 1 of 1 · fictitious patient (Veteran AK)

Diagnostic Assessment

Guillain-Barre Syndrome

No Statement of Principles applies to this condition (non-SOP claim).

ADF History

Veteran AK, date of birth 27 January 1990, Australian Army, Driver (Royal Australian Corps of Transport), enlisted 14 January 2009, medically discharged 03 August 2019. Operational service: Afghanistan, 18 February 2013 to 30 October 2013 (warlike). Other overseas service: humanitarian assistance and disaster relief in Fiji after a tropical cyclone, 26 February 2016 to 08 April 2016.

Occupational History

As a transport driver the veteran drove heavy trucks and recovery vehicles on long convoys, loaded and unloaded stores, and worked in field conditions for weeks at a time. On humanitarian and disaster relief deployments drivers moved water, food and building materials into damaged communities, cleared debris and roofing iron, and lived in field camps with improvised water supplies, field kitchens and shared ablutions.

Disaster zones in the tropical Pacific carry a high risk of gastrointestinal infection from contaminated water and food, mosquito-borne viral infections such as dengue and Zika, respiratory infections spread in crowded camps, and lacerations from debris. Deployed members receive vaccinations before and during deployment and are treated in field and ship-borne medical facilities, with limited access to specialist neurological care.

The veteran's earlier warlike service in Afghanistan in 2013 involved long-distance convoy driving on roads threatened by improvised explosive devices. Although the triggering exposures for this condition occurred on the 2016 relief deployment, the field hygiene conditions, shared accommodation and vaccination programs of deployed service were common to both.

History

Veteran AK, a transport driver in the Australian Army, developed Guillain-Barre syndrome of the acute inflammatory demyelinating type on 18 April 2016, ten days after returning from disaster relief service in Fiji, where in the preceding weeks he had Campylobacter jejuni gastroenteritis, Zika and dengue virus infections, influenza and Mycoplasma pneumoniae infection, an influenza vaccination and surgery for a laceration, with serological evidence of recent cytomegalovirus, Epstein-Barr virus and hepatitis E infection. The condition worsened to respiratory failure on 16 May 2016 with an intercurrent pneumonia.

Timeline

• 21 Mar 2016 — While deployed on disaster relief in Fiji the veteran received the 2016 seasonal influenza vaccine from the deployed health team as part of the force health protection program. No immediate reaction was recorded. He was working 12-hour days moving water and building materials and clearing cyclone debris. "influenza vaccine given, no reaction"
• 25 Mar 2016 — The veteran sustained a deep laceration of the left forearm from a sheet of roofing iron while clearing debris from a damaged school. It was debrided and repaired under general anaesthetic in the surgical facility of an amphibious ship supporting the operation, and he returned to light duties ashore two days later. "forearm laceration, debrided under GA"
• 28 Mar 2016 — The veteran developed fever, abdominal cramps and bloody diarrhoea, with eight to ten motions a day, three days after a meal from a local market in a community whose water supply had been contaminated by the cyclone. Stool culture grew Campylobacter jejuni. He was treated with azithromycin and oral rehydration and recovered over five days. "Campylobacter jejuni on stool culture"
• 02 Apr 2016 — Two days after the onset of fever and cough, for which a nasopharyngeal swab on 30 March 2016 was positive for influenza B and Mycoplasma pneumoniae, the veteran developed a maculopapular rash, conjunctivitis, arthralgia and high fever. Urine PCR was positive for Zika virus and serum dengue NS1 antigen was positive. He was managed in the field and returned to Australia with his unit on 08 April 2016. "Zika PCR and dengue NS1 positive"
• 19 Apr 2016 — The veteran presented to the garrison health centre with one day of tingling in both feet and hands, low back pain and difficulty climbing into his truck cab. The medical officer recorded reduced but present ankle reflexes, diagnosed post-viral fatigue and lumbar strain, prescribed analgesia and returned him to duty with review in a week. "tingling feet, back pain, duty"
• 25 Apr 2016 — The veteran was admitted to hospital unable to walk unaided, with symmetrical weakness of all four limbs, Medical Research Council sum score 38 of 60, absent reflexes and mild facial weakness. Nerve conduction studies showed prolonged distal latencies, conduction block and absent F-waves, cerebrospinal fluid protein was 1.4 g/L with 2 cells, and anti-GM1 antibodies were positive. Cytomegalovirus, Epstein-Barr virus and hepatitis E IgM were positive, with an alanine aminotransferase of 312 U/L. A neurologist diagnosed Guillain-Barre syndrome and commenced intravenous immunoglobulin on 26 April 2016. "AIDP, CSF protein 1.4 g/L"
• 16 May 2016 — After initial stabilisation, the veteran developed aspiration pneumonia and a catheter-associated urinary tract infection, and his weakness deteriorated over three days, with the Medical Research Council sum score falling from 44 to 22 and forced vital capacity from 3.1 to 1.1 litres. He was intubated and ventilated in intensive care for 19 days and given a second course of intravenous immunoglobulin for a treatment-related fluctuation. "ventilated, MRC sum score 22"
• 20 Feb 2017 — After four months of inpatient rehabilitation the veteran walked independently with ankle-foot orthoses. The neurologist recorded residual distal weakness, Medical Research Council sum score 52, a Guillain-Barre syndrome disability score of 2, persisting neuropathic pain and severe fatigue. He was medically discharged on 03 August 2019. "residual weakness, GBS disability 2"

Symptoms

At onset the veteran had tingling and numbness that began in the feet and hands and spread upwards over several days, deep aching low back and thigh pain, and rapidly progressive weakness of the legs and then the arms, so that within a week he could not climb stairs, rise from a chair or walk unaided. He also had facial weakness and difficulty swallowing.

In the week after onset the weakness ascended from the ankles to the hips and then to the shoulders and hands. He had difficulty gripping the steering wheel, fell twice on stairs, and developed double vision, slurred speech and drooping of both sides of the face, with episodes of a racing heart and fluctuating blood pressure from autonomic involvement.

At the worsening he became breathless and unable to clear secretions, requiring ventilation, with near-complete paralysis. He now reports persistent weakness of the ankles and hands, foot drop requiring orthoses, burning pain in the feet, poor balance, severe fatigue that limits him to a few hours of activity a day, and difficulty with fine hand tasks.

Imaging

• 25 Apr 2016 — MRI lumbar spine with contrast: "Smooth enhancement of the cauda equina nerve roots. No compressive lesion."
• 16 May 2016 — Chest X-ray: "Right lower lobe consolidation consistent with aspiration pneumonia. Reduced lung volumes."

Onset Date

The most likely date of onset of this condition is 18 Apr 2016.

At that date the veteran was serving full time with a transport regiment in Australia, ten days after returning from disaster relief service in Fiji. He woke with tingling in both feet and hands and low back pain, and over the next day had difficulty climbing into his truck cab.

This date is appropriate because his post-deployment health screen on 11 April 2016 recorded full recovery from his infections in Fiji, normal power and normal reflexes, and he first presented with neurological symptoms on 19 April 2016, giving a history of one day of tingling. The diagnosis was confirmed on 25 April 2016. It falls within continuous full-time service.

For the infection, vaccination and surgery causes, the 6 weeks before onset run from 07 Mar 2016 to 18 Apr 2016, all of it during or immediately after his disaster relief deployment. The influenza vaccination on 21 Mar 2016, the surgery on 25 Mar 2016, the Campylobacter gastroenteritis on 28 Mar 2016, the influenza and Mycoplasma infection from 30 Mar 2016, and the Zika and dengue infections from 02 Apr 2016 all fall within that window. The positive cytomegalovirus, Epstein-Barr virus and hepatitis E IgM antibodies on 25 Apr 2016, with seroconversion against negative pre-deployment screening and a positive hepatitis E PCR, place those infections in the same weeks.

For the worsening factors, the documented worsening is 16 May 2016, when the Medical Research Council sum score fell from 44 to 22 and he required ventilation. The 2 weeks before the worsening run from 02 May 2016 to 16 May 2016, during which the aspiration pneumonia began on 13 May 2016 and the urinary tract infection on 12 May 2016.

1. What is the formal diagnosis of the condition claimed above?

The formal diagnosis is Guillain-Barre syndrome, acute inflammatory demyelinating polyradiculoneuropathy type (ICD-10 G61.0). No Statement of Principles has been determined for this condition.

Guillain-Barre syndrome is an acute immune-mediated disorder of the peripheral nerves and nerve roots. In most cases it follows an infection, which provokes antibodies and immune cells that cross-react with components of the nerve myelin or axon (molecular mimicry). It causes rapidly progressive, symmetrical weakness with loss of reflexes, often with sensory symptoms, pain and cranial nerve involvement, reaching its worst within four weeks. Up to a quarter of patients need mechanical ventilation, and autonomic instability can be life-threatening. Treatment is intravenous immunoglobulin or plasma exchange. Many patients are left with weakness, pain and fatigue.

In this veteran the condition began on 18 April 2016 after multiple infections, vaccination and surgery during disaster relief service in Fiji. It was confirmed on 25 April 2016 and worsened to respiratory failure on 16 May 2016.

2. For each diagnosis identified, please also provide the following dates:

When did the veteran first experience symptoms attributable to this condition?

18 April 2016.

When did the veteran first present to a health / medical provider for this condition?

19 April 2016.

When was the condition confirmed / formally diagnosed?

25 April 2016.

When did the veteran first present to you (or your practice) for this condition?

10 September 2025.

3. How was this diagnosis confirmed? i.e. what were the key symptoms and signs, investigation results, specialist opinions?

The diagnosis was made by a neurologist on 25 April 2016. The veteran had progressive bilateral weakness of the arms and legs with areflexia, progressing over seven days, with facial weakness and distal sensory symptoms. Cerebrospinal fluid showed albuminocytological dissociation, with a protein of 1.4 g/L and 2 white cells, and nerve conduction studies showed prolonged distal latencies, conduction block and absent F-waves consistent with acute demyelinating polyradiculoneuropathy. MRI showed enhancement of the cauda equina nerve roots and anti-GM1 antibodies were positive. These findings meet Brighton Collaboration level 1 diagnostic certainty and the National Institute of Neurological Disorders and Stroke criteria for Guillain-Barre syndrome.

The worsening was confirmed on 16 May 2016 by a fall in the Medical Research Council sum score from 44 to 22 and in forced vital capacity from 3.1 to 1.1 litres, requiring intubation, ventilation and a second course of immunoglobulin.

4. What do you consider to be the cause(s) of the condition in this veteran? Give consideration to constitutional and common risk factors, pre-existing injuries or diseases, and occupational or lifestyle risks, as well as direct causative mechanisms.

No Statement of Principles has been determined by the Repatriation Medical Authority for Guillain-Barre syndrome. The claim is therefore decided on the sound medical-scientific evidence of what causes and worsens the condition, without the limits a Statement of Principles would impose. The recognised causes below are drawn from the neurological and epidemiological literature, and each is addressed against the veteran's service and clinical history.

Legislation: the clinical onset of this condition was on 18 April 2016, after 1 July 2004, during continuous full-time service. This is a Military Rehabilitation and Compensation Act 2004 (MRCA) claim. Where a Statement of Principles exists, the Reasonable Hypothesis instrument applies to operational (warlike and non-warlike) service and the Balance of Probabilities instrument to peacetime service; the disaster relief deployment to Fiji in which the triggering exposures occurred was peacetime service. Each cause must be related to the person's service. Under the beneficial approach required by the legislation, the absence of documentation does not mean that an event or exposure did not occur: lack of evidence is not evidence of lack.

Recognised causes on the medical-scientific evidence (no Statement of Principles)

Causative factors

Cause 1: having an infection with Campylobacter jejuni within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- On 28 March 2016, during disaster relief service in Fiji, the veteran developed fever, cramps and bloody diarrhoea after eating food from a market in a community whose water supply had been contaminated by the cyclone, and stool culture grew Campylobacter jejuni. This was 21 days before onset on 18 April 2016. Campylobacter jejuni is the most frequently identified trigger of Guillain-Barre syndrome, and the positive anti-GM1 antibodies found on 25 April 2016 are the antibody pattern typical of post-Campylobacter disease.

Cause 2: having an infection with Zika virus within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- On 02 April 2016 the veteran developed rash, conjunctivitis, arthralgia and fever, and urine PCR was positive for Zika virus, which was circulating in Fiji at the time. He was exposed to mosquitoes while working long days outdoors in flooded, cyclone-damaged communities. The infection was 16 days before onset. Zika virus outbreaks in the Pacific and the Americas were followed by marked increases in Guillain-Barre syndrome.

Cause 3: having an infection with dengue virus within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- Serum dengue NS1 antigen was positive on 02 April 2016, with dengue IgG seroconversion on 25 April 2016, during the dengue outbreak that followed the cyclone in Fiji. The infection was acquired from mosquito bites during his relief duties, 16 days before onset. Dengue virus infection is a recognised antecedent of Guillain-Barre syndrome.

Cause 4: having an infection with cytomegalovirus within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- Cytomegalovirus IgM was positive on 25 April 2016 with low-avidity IgG, and IgG had been negative on his pre-deployment screening in January 2016, confirming a primary infection in the weeks before onset, while he lived in crowded field accommodation in Fiji. Cytomegalovirus is the second most common infectious trigger of Guillain-Barre syndrome.

Cause 5: having an infection with Epstein-Barr virus within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- Epstein-Barr virus viral capsid antigen IgM was positive on 25 April 2016, with nuclear antigen antibodies negative, indicating a recent primary infection, acquired in shared field accommodation and ablutions during the deployment in March and April 2016, within the 6 weeks before onset. Epstein-Barr virus is a recognised antecedent infection.

Cause 6: having an infection with Mycoplasma pneumoniae within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- On 30 March 2016 the veteran developed fever and cough during a respiratory outbreak in the field camp, and a nasopharyngeal PCR swab was positive for Mycoplasma pneumoniae, 19 days before onset. Mycoplasma pneumoniae infection, which provokes anti-galactocerebroside antibodies, is a recognised trigger of Guillain-Barre syndrome.

Cause 7: having an infection with influenza virus within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- The same nasopharyngeal swab on 30 March 2016 was positive for influenza B. The infection was acquired during the respiratory outbreak in the crowded relief camp, 19 days before onset. Influenza-like illness carries a several-fold increase in the risk of Guillain-Barre syndrome in the following weeks.

Cause 8: receiving an influenza vaccination within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- The veteran received the 2016 seasonal influenza vaccine from the deployed health team in Fiji on 21 March 2016 under the Defence force health protection program, 28 days before onset on 18 April 2016. Influenza vaccination is associated with a small increase in the risk of Guillain-Barre syndrome, about one additional case per million doses, concentrated in the 6 weeks after vaccination.

Cause 9: undergoing surgery under general anaesthesia within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- On 25 March 2016 the veteran's forearm laceration, sustained clearing roofing iron on relief duties, was debrided and repaired under general anaesthetic in the surgical facility of an amphibious ship, 24 days before onset. Surgery is associated with an increased risk of Guillain-Barre syndrome in the following weeks, thought to result from immune activation by tissue injury.

Cause 10: having an infection with hepatitis E virus within the 6 weeks before the clinical onset of Guillain-Barre syndrome; — MET

- Liver function tests on 25 April 2016 showed an alanine aminotransferase of 312 U/L, and hepatitis E virus IgM and serum PCR were positive, confirming acute hepatitis E infection acquired from contaminated water or undercooked pork during the relief deployment in Fiji in late March or early April 2016, within the 6 weeks before onset. He had not been jaundiced. Hepatitis E is found in up to one in twenty cases of Guillain-Barre syndrome in endemic settings and is a recognised antecedent infection.

Worsening factors

Worsening 1: having an intercurrent infection within the 2 weeks before the clinical worsening of Guillain-Barre syndrome; — MET

- A catheter-associated urinary tract infection with Escherichia coli was confirmed on 12 May 2016, and aspiration pneumonia, with right lower lobe consolidation on chest X-ray, on 13 May 2016, while he was in hospital for Guillain-Barre syndrome developed in service. Within three days his Medical Research Council sum score fell from 44 to 22 and he required ventilation on 16 May 2016. Intercurrent infection is a recognised cause of deterioration and treatment-related fluctuation.

Worsening 2: inability to obtain appropriate clinical management for Guillain-Barre syndrome. — MET

- When the veteran first presented to the garrison health centre on 19 April 2016 with ascending tingling, back pain and early weakness after multiple infections on deployment, the condition was misdiagnosed as post-viral fatigue and lumbar strain and he was returned to duty. He was not admitted until 25 April 2016, by which time he could not walk, and immunoglobulin was not started until 26 April 2016. Under Brew v Repatriation Commission (1999) this is an inability to obtain appropriate clinical management.

The % contribution of the causes is 100% and significant.

Sequelae

Met. The Guillain-Barre syndrome has left the veteran with residual distal motor and sensory polyneuropathy, bilateral foot drop requiring orthoses, chronic neuropathic pain and severe fatigue. The ventilation in intensive care was followed by critical illness weakness and an adjustment disorder. Each of these should be considered for separate claims as sequelae of the accepted condition.

Unintended Consequence

Met. The influenza vaccination given by the deployed health team on 21 March 2016 and the surgery under general anaesthesia on 25 March 2016 were medical treatment provided by the Commonwealth while the veteran was serving, and both preceded onset within the recognised risk window. The misdiagnosis on 19 April 2016 delayed treatment. These are unintended consequences of treatment provided by the Commonwealth (section 6A of the DRCA and the equivalent provisions of the MRCA).

Inability to Attain Appropriate Medical Management

MET. The veteran presented on 19 April 2016 with the classic early features of Guillain-Barre syndrome, ascending distal paraesthesia, back pain and early weakness, two weeks after multiple documented infections on deployment. He was diagnosed with post-viral fatigue and lumbar strain and returned to driving duties, and was not admitted until he could no longer walk on 25 April 2016. Immunoglobulin was started eight days after onset. Applying the objective and subjective tests in Brew v Repatriation Commission (Full Federal Court, 10 September 1999, Merkel J), the failure of the garrison health system to recognise and refer an evolving neurological emergency amounts to an inability to obtain appropriate clinical management, and the progression to severe weakness, and later respiratory failure, followed.

5. Please provide a Health Summary and a medication / prescribing history.

See attached report.

References

1. 21 Mar 2016, Deployed immunisation record, Nursing Officer, disaster relief task group
2. 25 Mar 2016, Operation report, Surgeon, ship-borne surgical facility
3. 28 Mar 2016, Deployed health record and stool culture, Medical Officer
4. 30 Mar 2016, Respiratory PCR report, Pathology
5. 02 Apr 2016, Zika PCR and dengue NS1 reports, Pathology
6. 11 Apr 2016, Post-deployment health screen, Medical Officer, Garrison health centre
7. 19 Apr 2016, Outpatient health record, Medical Officer, Garrison health centre
8. 25 Apr 2016, Neurology admission, nerve conduction study, CSF and MRI reports, Neurologist
9. 25 Apr 2016, Viral serology report, Pathology
10. 16 May 2016, Intensive care admission record and chest X-ray, Intensive care physician
11. 20 Feb 2017, Rehabilitation discharge summary, Rehabilitation physician
12. 20 Feb 2017, Neurology review, Neurologist