Andy Griffith contracts Guillain-Barre syndrome after getting the flu following his wife Cindi's tonsillectomy. He experiences intense pain and paralysis. Doctors are unable to diagnose him for several days until a spinal tap reveals Guillain-Barre. There is no cure, so he must wait for it to run its course while managing pain. Guillain-Barre is an autoimmune disorder where the immune system attacks the peripheral nervous system, causing inflammation of nerves and paralysis. Proper management focuses on supportive care, respiratory support, pain management, and potentially plasma exchange or IVIG treatment.

2. Journey To Health
by Andy Griffith
Manteo, North Carolina
“Cindi had had frequent and major attacks of sore throat all of her life. So,
shortly after our marriage we returned to Los Angeles and went to see a throat
specialist, Dr. Robert Feder. He determined her sore throats were coming from
her tonsils, and scheduled an operation. Early the next morning we drove over
to Cedars-Sinai Medical Center and Dr. Feder took out her tonsils. While she
was recuperating, I got a bad case of the flu. Not exactly a honeymoon of the
rich and famous.
My illness was strange. As I got better, the symptoms of influenza were
replaced by pain--terrible, searing pain that ricocheted through my entire body.
Cindi and I joked about our invalid status and settled in that Saturday to watch
the Kentucky Derby on television.
But after the race, when I stood up and took a few steps, I pitched headlong
into a nightmare. I was overcome by pain so encompassing that I couldn't feel
my feet. I had no control over them, and fell to the floor in agony.

3. We couldn't reach any of our doctors that weekend. Yet I was so
desperate for relief from the pain that I took some of the codeine
prescribed for Cindi for her throat. It barely made a difference.
On Monday my doctor met us at a local hospital. There a roomful of
doctors attempted to find out what was wrong. For four days they
hadn't a clue. Finally they did a spinal tap. When the results came in,
one mystery was solved. I had Guillain-Barre syndrome, a rare form of
nerve inflammation. It's thought to be caused by an allergic reaction to
a viral infection, such as the flu. The nerves become inflamed and
begin to send erroneous and scrambled messages to the brain. In some
people it causes little pain but extensive paralysis; in cases such as
mine, it causes little paralysis but intense pain.
There are no drugs or surgery to treat Guillain-Barre--so the doctor sent
me home. "There is nothing we can do," he said. "You've got to ride it
out. I'll prescribe some pain medication, but use as little as possible.
Come back in a week."

5. Introduction
Guillain-Barré syndrome (GBS) is an acute,
frequently severe, and fulminant
polyradiculoneuropathy that is autoimmune in nature.
collection of clinical syndromes that manifests as an
acute inflammatory polyradiculoneuropathy with
resultant weakness and diminished reflexes.

6. 1 to 2 per 100,000 population.
Males are at 1.5-fold higher risk for GBS than females,
in western countries adults are more frequently
affected than children.
With poliomyelitis under control in developed
countries, GBS is now the most important cause of
acute flaccid paralysis

7. History
The French physician Jean Landry first described
the disorder in 1859.
In 1916, Georges Guillain, Jean Alexandre Barré,
and André Strohl diagnosed two soldiers with the
illness and described the key diagnostic abnormality
of increased spinal fluid protein production, but
normal cell count.

8. Epidemiology
Worldwide, the annual incidence is about 0.6–4
occurrences per 100,000 people.
Men are one and a half times more likely to be
affected than women.
The incidence increases with age; there are
approximately 1 cases per 100,000 people aged below
30 years and about 4 cases per 100,000 in those older
than 75 years.
The incidence of GBS during pregnancy is 1.7 cases
per 100,000 of the population.
Congenital and neonatal Guillain–Barré syndrome
have also been reported

9. GBS is also known as
acute idiopathic polyradiculoneuritis,
acute idiopathic polyneuritis,
French polio,
Landry's ascending paralysis and
Landry Guillain Barré syndrome.

10. Related anatomy and physiology

14. Pathophysiology
Guillain-Barré is the result of a cell-mediated immune
attack on peripheral nerve myelin proteins.
The best-accepted theory is that an infectious organism
contains an amino acid that mimics the peripheral nerve
myelin protein.
The immune system cannot distinguish between the two
proteins and attacks and destroys peripheral nerve myelin.
the ganglioside GM1b, is the most likely target of the
immune attack.
With the autoimmune attack there is an influx of
macrophages and other immune-mediated agents that
attack myelin, cause inflammation and destruction, and
leave the axon unable to support nerve conduction.

16. Research Input
Campylobacter jejuni infection in Guillain-Barré syndrome:
A prospective case control study in a tertiary care hospital
A Sharma1, V Lal1, M Modi1, C Vaishnavi2, S Prabhakar1
Background: This study was carried out to determine the
relationship between C. jejuni infection and GBS in an Indian
setting.
Materials and Methods: This prospective study was carried out on
a cohort of 50 patients with GBS who were treated in a tertiary
care hospital in India. Based on electrophysiological findings the
patients were divided into various subtypes. Serology for C.
jejuni (Immunoglogulin G, IgG and Immunoglogulin, IgM) using
ELISA was done both in patients and 40 age, sex and
geographically matched controls.

17. Conclusions
Preceding C. jejuni infection is common among GBS
patients and is often associated with the axonal variety
of GBS. Axonal variety of GBS generally presents in a
younger age group as compared to AIDP.

18. SIGNS & SYMPTOMS
Areflexic motor paralysis with or without sensory
disturbance.
Bulbar weakness
Pain
Deep tendon reflexes attenuate or disappear
Cutaneous sensory deficits
Bladder dysfunction

19. Subtypes
• 1.Acute inflammatory demyelinating
polyradiculoneuropathyAIDP
• 2.Acute motor axonal neuropathyAMAN
• 3.Acute motor and sensory axonal
neuropathyAMSAN
MFS 4.Miller fisher syndrome

20. 1.Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP)
is the most common form and accounts for around 85–
90% of cases.
The clinical features are of symmetrical ascending motor
weakness with hypo- or areflexia.
The underlying pathological process involves
inflammation and destruction of the myelin sheaths
surrounding peripheral nerve axons by activated
macrophages.
This leads to slowing and blockage of conduction within
peripheral nerves causing muscle weakness.
Severe cases may develop secondary axonal damage

21. Acute motor axonal neuropathy (AMAN)
is more common in Japan and China, amongst young
people and in the summer months.
It has an association with precedent infection with
Campylobacter jejuni.
Clinical features are similar to AIDP but tendon reflexes
may be preserved.
Electrophysiological testing may distinguish from other
variants as selective motor nerve and axonal involvement
is demonstrated.
In AMAN the pathological process involves binding of
antibodies to ganglioside antigens on the axon cell
membrane, macrophage invasion, inflammation and
axonal damage.

22. Acute motor and sensory axonal
neuropathy (AMSAN)
is a variant of GBS in which both motor and sensory
fibres are involved and which can be demonstrated on
electrophysiological studies.
It is more severe and associated with prolonged or
even partial recovery.
Clinical features are similar to AMAN but also
involve sensory symptoms.
The underlying pathological process is similar to that
for AMAN (i.e. antibody mediated axonal damage).

23. Miller Fisher syndrome (MFS)
presents with ataxia, areflexia and ophthalmoplegia.
25% of patients may develop limb weakness.
Electrophysiological studies show primarily sensory
conduction failure.
Antiganglioside antibodies to GQ1b are found in
90% of patients and are associated with
ophthalmoplegia .
There have been limited pathological studies in MFS
but demyelination of nerve roots has been
demonstrated.

24. INVESTIGATIONS
Serum biochemistry:evidence of SIADH or renal
dysfunction
Inflammatory markers :ESR is usually raised and
CRP is sometimes

25. INVESTIGATIONS
Antiganglioside antibodies :
Anti-GM1
• It is
positive
in 25% of
pts and is
a w worse
outcome
Anti-GD1a
• AMAN
subtype
of GBS
Anti-GQ1b
• Miller-
Fisher
syndrome

26. INVESTIGATIONS
Infection screen :
Campylobacter jejuni,
Cytomegalovirus,
Epstein-Barr virus,
Herpes simplex virus,
Mycoplasma pneumoniae.
HIV antibodies

27. INVESTIGATIONS
Radiological: A CT brain is indicated to exclude
other causes of symptoms and evidence of raised
intracranial pressure prior to performing a lumbar
puncture. An MRI of the spine may show selective
anterior spinal nerve root enhancement with
gadolinium and will exclude cervical nerve
impingement

28. INVESTIGATIONS

29. INVESTIGATIONS
Lumbar puncture: Cell count and glucose are
usually normal with a raised protein, although the
latter may also be normal in first two weeks.

30. INVESTIGATIONS
Nerve conduction studies:
– Findings depend on subtype of GBS.
– The majority show demyelinating pattern while
– some patients may show evidence of axonal loss
with little or no demyelination.

31. INVESTIGATIONS
Respiratory function tests:
– These may show reduced vital capacity, maximal
inspiratory and expiratory pressures.
– Arterial blood gases may indicate progressive
respiratory failure.

32. DIFFERENTIAL DIAGNOSIS
Neurological
– Myasthenia gravis
– Eaton-Lambert (myasthenic) syndrome
– Multiple sclerosis
– Transverse myelitis

33. DIFFERENTIAL DIAGNOSIS
Metabolic
– Hypokalaemic periodic paralysis
– Hypermagnesaemia
– Hypophosphataemia
– Acute intermittent porphyria

34. DIFFERENTIAL DIAGNOSIS
Infective
– Post diphtheria neuropathy
– Polio
– Botulism
– Tick paralysis

35. DIFFERENTIAL DIAGNOSIS
Drugs / toxins
– Heavy metal poisoning (e.g. lead)
– Biological toxins (including snake and scorpion
toxins)
– Drugs (including stavudine, nitrofurantoin and
aminoglycosides)

36. DIFFERENTIAL DIAGNOSIS
Other
– Acute polymyositis
– Critical illness myopathy

37. MANAGEMENT
Supportive Immunomodulatory

38. Supportive
Airway and respiratory
Cardiovascular
Gastrointestinal
Neurological
Psychological
Rehabilitation

39. Immunomodulatory
Intravenous immunoglobulin
– most effective if administered within two weeks of
the onset of symptoms
– Indications for IVIg include
• muscle weakness and
• respiratory depression

40. IVIG
IVIg contains pooled donor IgG antibodies and may
reduce the severity of autoimmune inflammation in
GBS by blocking Fc receptors.
This prevents the Fc portion of antibodies binding
and thus interrupts antibody mediated cell
destruction.
Complement activation is also altered.

41. Plasma exchange
The aim of plasma exchange is to remove antibodies
associated with the underlying autoimmune response.
passage of blood through an extracorporeal cell
separator.
The plasma fraction of the blood is removed then and
replaced with FFP or human albumin solution.
Anticoagulants are administered during the
procedure.

42. Recommendations for the treatment of
GBS:
1)plasma exchange (PE) is recommended for
nonambulant adult patients with GBS who seek treatment
within 4 weeks of the onset of neuropathic symptoms.PE
should also be considered for ambulant patients examined
within 2 weeks of the onset of neuropathic symptoms.
2) IV IG is recommended for nonambulant adult patients
with GBS within 2 or possibly 4 weeks of the onset of
neuropathic symptoms;
3) Corticosteroids are not recommended for the
management of GBS.
4) sequential treatment with PE followed by IV IG or
immunoabsorption followed IV IG is not recommended
for patients with GBS ;and
5) PE and IV IG are treatment options for children with
severe GBS

43. Recommendations For Rx
1)plasma exchange (PE) is recommended for
nonambulant adult patients with GBS who seek treatment
within 4 weeks of the onset of neuropathic symptoms.PE
should also be considered for ambulant patients
examined within 2 weeks of the onset of neuropathic
symptoms.
2) IV IG is recommended for nonambulant adult patients
with GBS within 2 or possibly 4 weeks of the onset of
neuropathic symptoms;
3) Corticosteroids are not recommended for the
management of GBS.
4) sequential treatment with PE followed by IV
IG or
immunoabsorption followed IV IG is not
recommended for patients with GBS ;and
5) PE and IV IG are treatment options for children
with severe GBS

44. PROGNOSIS
Most patients with GBS recover fully but this may
take many months of intensive therapy.
15% of patients suffer persistent disability.
10% are unable to walk unaided at one year.
There may be a recurrence in 2–5% of cases.
The mortality from GBS ranges from 2–12%.

45. Prognosis
Common causes of death include venous
thromboembolism, pneumonias, arrhythmias and
complications related to dysautonomia.
Markers of poor prognosis include
– age > 40 years,
– rapid onset of symptoms,
– severe weakness(especially if mechanically ventilation
is required or there is marked upper limb weakness),
– association with precedent diarrhoeal illness or
campylobacter infection,
– evidence of axonal damage on electrophysiological
studies and
– lack of treatment with either plasma exchange or
IVIg.

46. Nursing Management
Assessment:
Acute onset (hours to weeks) of progressive, usually
ascending muscle weakness and fasciculation,
possibly leading to paralysis (maximal weakness is
reached within 2 weeks).
Paresthesia and painful sensations.
Possible hypoventilation due to chest muscle
weakness.
Difficulty with swallowing, chewing, speech, and
gag, indicating fifth (trigeminal) and ninth
(glossopharyngeal) cranial nerve movement.
Reduce or absent deep tendon reflexes, position and
vibratory perception.

47. Assessment:
Autonomic dysfunction with orthostatic hypotension
and tachycardia.
Check for previous history of viral illness or surgical
procedure
Urinary incontinence
Check for facial nerve paralysis. Inspect the patient’s
face at rest and during conversation.Assess for any
problems during talking,swallowing,and chewing.
Assess for any change in the vital signs

48. Maintaining respiratory function
incentive spirometry and chest physiotherapy
Mechanical ventilation
Close monitoring
Suctioning

49. Enhancing physical mobility
paralyzed extremities are supported in functional
positions, and
passive range-of-motion exercises are performed at least
twice daily
Range-of-motion exercises,
altering positioning,
anticoagulation,
thigh-high elastic compression stockings or
sequential compression boots, and
adequate hydration

50. Providing adequate nutrition
IV fluids and parenteral nutrition
monitors for the return of bowel sounds
gastrostomy tube
assesses the return of the gag reflex and bowel sounds
before resuming oral nutrition.

51. Improving communication
picture cards or an eye blink system
Collaboration with the speech therapist

52. Decreasing fear and anxiety
participate in physical care
providing information about the condition,
emphasizing a positive appraisal of coping resources,
teaching relaxation exercises and
distraction techniques
Encouraging visitors,
engaging visitors or volunteers to read to the patient,
listening to music or books on tape, and
watching television

53. Monitoring and managing potential
complications
Thorough assessment of respiratory function at
regular intervals
cardiac dysrhythmias, which necessitate ECG
monitoring, transient hypertension, orthostatic
hypotension, DVT, pulmonary embolism, urinary
retention, and other threats to any immobilized and
paralyzed patient.
These require monitoring and attention to prevent
them and prompt treatment if indicated.