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Guillain-Barre Syndrome. William Woodfin MD. K.F. 40 y.o. r/h woman. 3/17 Nausea, diarrhea & severe myalgias Son dxed c rotavirus 1 wk. Previously 4/21 “Creepy-crawlies” legs>arms 4/25 Weakness legs progressing 4/26 Handwriting looks like “hen scratch”. K.F. 40 y.o. woman.

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guillain barre syndrome

Guillain-Barre Syndrome

William Woodfin MD

k f 40 y o r h woman
K.F. 40 y.o. r/h woman

3/17 Nausea, diarrhea & severe myalgias

Son dxed c rotavirus 1 wk. Previously

4/21 “Creepy-crawlies” legs>arms

4/25 Weakness legs progressing

4/26 Handwriting looks like “hen scratch”

k f 40 y o woman
K.F. 40 y.o. woman

4/28 Admitted to outside hospital.

L.P. wnl

EMG positive waves in some leg

muscles

NCVs absent H-reflexes

F responses & motor latencies wnl

k f 40 y o woman4
K.F. 40 y.o. woman

4/29 Transferred to PHD

Hx.: diabetic x 10 yrs.

hypothyroid- treatedx yrs.

no sphincter disrubance

aching pain low back & buttocks

mild postural light headedness

no SOB or palpatations

slide5
Exam

BP 150/90 P 80 Wt. 250 lbs.

Mild weakness neck flexors

4/5 biceps, grip & interossei- symmetric

2/5 iliopsoas & quadriceps

3/5 hamstrings & adductors

4/5 abductors

4/5 ankles & toes- extensors & flexors

slide6
Exam

Sensory- intact

DTRs- biceps, BR, knees are trace c

reinforcement. Triceps & ankles

unobtainable

Plantars- flexor

F to N- intact

Gait- not testable

slide7
Lab

H/H 10.3/33.5 c microcytic indices

A1c Hgb 10.1

TSH 0.97

LDL 182

Serum immunofixation- wnl. No IgA def.

FVCs- consistently 4+ liters

slide8
MRI

LS spine s & c contrast- no nerve root enhancement

course in hospital
Course in hospital

Treated c IVIG 0.4 gms/kgm daily x 5

Strength fluctuated only mildly

Blood sugars ok in AM, high in afternoons

Repeated NCVs show mild dispersion of F waves

Transferred back to referring hospital 5/6

telephone fu
Telephone FU

Ambulating fairly well c walker. Strength

clearly improving.

Still bothered by “creepy-crawlies”

what is the gbs
What is the GBS?
  • Due to the breadth of clinical presentation it is of limited help to try to define rigid diagnostic criteria.
  • Thomas Munsat 1965: “…The GBS is easy to diagnose but difficult to define
the typical illness evolves over weeks usually following an infectious disease and involves
1. Paresthesiaes usually hearld the disease

2. Fairly symmetric weakness in the legs, later the arms and, often, respiratory and facial muscles

3. Dimunition and loss of the DTRs

4. Albuminocytologic dissociation

5. Recovery over weeks to months

The typical illness evolves over weeks usually following an infectious disease and involves:
history
Waldrop 1834

Olliver 1837

Landry 1859

Graves 1884

Ross & Bury 1893

Brussel’s Conf. 1937

Haymaker & Kernohan 1949

Waksman & Adams 1955

Miller Fisher 1956

Asbury, Aranson & Adams 1969

History

Guillain, Barre & Strohl

1916-1920

note sur la paralysie ascendante aigue 1859
Note sur la paralysie ascendante aigue 1859
  • March 16- a febrile illness
  • May 11- mild sensory symptoms in the fingers and toes
  • June 13- knees buckle
  • June 16- unable to walk
  • Subsequent respiratory failure and death.
  • Autopsy unrevealing. Peripheral nerves probably not examined
late 19 th century
Late 19th century
  • Westphal 1876- “Landry’s Ascending Paralysis”
  • Graves 1884- localized neurologic disease to the peripheral nerves, “the nervous cords”
  • Ross & Bury 1893- 90 cases. A disease of the peripheral nerves
  • Numerous reports emphasizing various aspects of the disease with most authors crediting Landry
guillain barre strohl 1916 revue neurologique
Guillain, Barre & Strohl 1916Revue Neurologique
  • Two soldiers in Amiens developing paralysis and loss of DTRs.
  • A new diagnostic feature: albuminocytologic dissociation in the CSF
  • No mention of Landry
foundations
Foundations
  • Quincke- CSF observations 25 years earlier
  • Siccard & Foix- “albuminocytologic dissociation” in Pott’s disease

Late 19th century: examination of the reflexes had become a part of the neurologic exam with appreciated as a sign of neuropathy based on observations in tabes dorsalis areflexia

haymaker kernohan 1949
Haymaker & Kernohan 1949
  • Landmark in pathological description c 50 fatal cases & detailed review of clinical findings
  • Emphasized prominent damage to proximal nerves often at junction of ventral & dorsal roots. Little study of more distal nerves
  • Unified findings of Landry & Guillain, Barre & Strohl
waksman adams 1955
Waksman & Adams 1955
  • Experimental Allergic Neuritis
  • First animal model of a noninfectious inflammatory neuritis
  • Rabbit nerve and Freund’s adjuvant injected intradermally
  • Target of activated T cells uncertain
asbury aranson adams 1969
Asbury, Aranson & Adams 1969
  • 19 pts. All with well developed mononuclear infiltrates in spinal roots and nerves within days of clinical onset
  • Pathological hallmark: perivascular mononuclear inflammatory infiltrates to adjacent to the areas of demyelination
overview of adaptive immunity
Overview of Adaptive Immunity
  • Lymphocytes: “command & control,” identify antigen components, respond specifically, mobilize other elements and direct the attack c memory for each antigenic assault
  • Antibodies: specialized immunoglobulin molecules directly neutralize and remove antigen
t lymphocytes
T lymphocytes
  • CD8- recognize epitopes paired c MHC-I
  • CD4- activate and control the immune response
  • Scavenger cells break down antigen into small peptide fragments (T cell epitopes), MHC-II epitope complexes are expressed on the surface & the scavenger become an APC which docks on a CD4 c a compatible TCR. CD4 proliferates releasing cytokines.
antibodies
Antibodies
  • Cytokines activate other lymphocytes including B cells that differentiate into plasma cells and serve as immunoglobulin factories.
  • Abs are Ig molecules that recognize, bind, neutralize and opsonize Ag for phagocytosis. They activate complement(membrane attack complex) & induce target cells to activate the inflammatory response
self tolerance
Self-tolerance
  • The process of self recognition
  • T & B cells learn self tolerance during maturation
  • Autoimmunity occurs when the mechanisms of self protection are defective
mechanisms of autoimmunity
Mechanisms of Autoimmunity
  • Molecular mimicry- microbe cell surface Ag resembles self protein. Damage results from “friendly fire” The inciting Ag is usually unidentified & may not exist as a single stimulus.
  • Excessive cytokine release due to profound immune stimulus may awaken self tolerant T cells or may cause expression of MHC complexes.
  • Self Ags bound to drugs may lose tolerated status
antecedent events infectious
Antecedent Events: Infectious
  • Viral: Influenza, Coxsackie, EBV, Herpes,

HIV, Hepatitis, CMV, WNV

Bacterial: Campylobacter jejuni,

Mycoplasma, E. coli

Parasitic: Malaria, Toxoplasmosis

antecedent events systemic disease
Antecedent Events: Systemic disease
  • Hodgkins
  • CLL
  • Hyperthyroidism
  • Sarcoidosis
  • Collagen Vascular d.
  • Renal d.
other antecedent events
Other antecedent events
  • Surgery
  • Immunization
  • Pregnancy
  • Envenomization
  • Bone marrow transplantation
  • Drug ingestion
features of aidp
Features of AIDP
  • 2/3s have identifiable preceding event
  • 50% begin with paresthesias followed by weakness in legs; 10% begin with arm weakness; rarely begins in face
  • Ophthalmoplegia: partial 15%, total 5%
  • Autonomic dysfunction in 65%, arrhythmias, hypotension,urinary retention in 10-15%, pupillary inequality
slide34
AIDP
  • Progresses for days to 4 weeks
  • 15% with severe disability
  • Mortality 3-5%
  • CSF: protein may be normal early, elevated in 90% by clinical nadir, cells< 10 in 95%, >50 suggests HIV
  • EDX: prolonged F & distal motor latencies, conduction block 30-40% in routine studies
slide35
AIDP
  • Pathology: immune attack directed at schwann cell plasmalemma esp. at nerve roots with IgG & complement deposits preceding demyelination
slide37
CIDP
  • Evolves over months
  • Fluctuates
  • Respiratory failure, dysautonomia, facial weakness, ophthalmoplegia- all are rare
  • CSF protein often highly elevated
  • Marked slowing of motor nerve conduction
  • Steroid responsive
features of amsan
Features of AMSAN
  • Commonly preceded by diarrhea esp. c. jejuni
  • Abrupt onset of weakness c rapid progression to quadriplegia & respiratory insufficiency
  • Other features as c AIDP
  • Longer recovery, more residual & mortality 10-15%
amsan
AMSAN
  • CSF as in AIDP
  • EDX: no response in some motor nerves, decreased amplitude of the CMAPs, fibrillations on needle study, absent SNAPs
  • Immune attack directed at axon plasmalemma at nodes of Ranvier. Wallerian degeneration
features of aman
Features of AMAN
  • Often preceded by diarrhea affecting younger population in China. Sporadic in USA
  • Prognosis similair to AIDP
  • Mortality <5%
  • EDX: reduced CMAPs c normal F & distal motor latencies and sensory studies. Fibrillations in 2-3 weeks
slide41
AMAN
  • Pathology: again axonal plasmalemma at nodes of Ranvier sometimes limited to physiologic dysfunction c nodal lengthening. May go on to extension through axonal basal lamina. Most axons recover s Wallerian degeneration
miller fisher syndrome
Miller Fisher Syndrome
  • Ophthalmoplegia, Ataxia, Areflexia
  • May be heterogonous: 1. Related to other patterns of GBS

2. Related to brainstem encephalitis, Bickerstaff

1952

3. CNS demyelination in association with GBS

miller fisher syndrome44
Miller Fisher Syndrome
  • 95% have serum IgG Ab to ganglioside GQ1b
  • Studies show preferential location of anti-GQ1b to cerebellar molecular layer & Cranial Nerves 3,4 & 6
  • May act at N-M junction depleting acetylcholine from nerve terminals
acute panautonomic neuropathy
Acute Panautonomic Neuropathy
  • Manifests over 1-2 weeks but may be of subacute onset
  • Frequent preceding infection
  • DTRs lost in 1/3, distal sensory changes 1/4
  • Albuminocytologic dissociation
  • EDX: NCVs usually normal
  • Recovery is gradual and incomplete
differential diagnosis
Differential Diagnosis
  • Consider the possibility of an upper motor neuron lesion
  • Other considerations are rare. Diphtheritic neuritis & poliomyelitis belong more to the history section of this presentation. A new possibility is West Nile Virus.
differential
Differential
  • N-M: MG, LES, Antibiotics
  • Toxic: Cigutera (ciguatoxin), Pufferfish (tetrodotoxin), Shellfish (saxitioxin), Botulism, Tick paralysis (Lone Star tick, Gulf Coast tick), Glue sniffing, Buckthorn
  • Mononeuritis multiplex assoc. c Wegner’s. PAN, SLE, RA, Sjogren’s, Cryoglobulinemia etc.
differential48
Differential
  • Metabolic: Periodic paralyses, Hypokalemia, Hypermagnesemia, Hypophoshatemia c parenteral hyperailimentation, Thyrotoxicosis, ICU myoneuropathy (CIP)
  • Heavy metal: Lead, Arsenic, Thallium, Barium c hypokalemia
differential miller fisher syn
Differential: Miller Fisher Syn.
  • Multiple sclerosis
  • Encephalitis
  • Posterior circulation ischemia or infarct
  • Other: Botulism, MG, Tick
treatment
Treatment

Respiratory failure

Autonomic dysfunction

DVT & PE

Pain

Positioning & Skin care

Physical therapy

Nutrition

respiratory failure
Respiratory Failure
  • Oropharyngeal weakness in ~25% with impaired swallowing of secretions & aspiration
  • Mechanical respiratory failure- mainly due to diaphragmatic weakness (Phrenic nerves.) Inspiratory c MIF (Max. Inspir. Force) a good supplement measure to FVC
respiratory failure52
Respiratory Failure
  • ~33% require intubation
  • Avg. time to intubation is 1 week & these pts. have substantially longer recovery time
  • Need is unlikely if patient does well for 2 wks. post onset of paresthesiaes
  • Guidelines: FVC <15 mL/kgm

MIF < 25 cm water

psychological
Psychological
  • Fear
  • Helplessness
  • Communication
  • Pain
  • Sleep deprivation & hallucinosis
  • Depression
  • Visits from other GBS patients
personal experience
Personal Experience
  • Bowes, Denise; The doctor as patient: an encounter with Guillain-Barre syndrome. Can Med Assoc J 131:1343-1348
corticosteroids
Corticosteroids
  • Lancet 1993 242 pts.

IV Methylprednisilone 500 mgm/day x 5.

Ineffective

May cause relapse

plasma exchange
Plasma Exchange
  • Removal of the blood’s liquid soluble components including complement, immunoglobulin, immune complexes, cytokines and interleukins
  • A typical session removes about 60% of the body mass of plasma proteins which is replaced c saline, albumin & FFP
  • Done qod for 3-5 sessions
plasma exchange57
Plasma Exchange
  • Various studies since 1985
  • Time on ventilator reduced by ½
  • Full strength regained at 1 year: Exchange 71%, Untreated 52%
  • Limitations: Limited availability

Avoid with autonomic instability

intravenous immune globulin
Intravenous Immune Globulin
  • Originally used for immune insufficiency
  • Use as an immunosuppresant “seems to defy reason”
  • 1981 Rx for ITP
  • 5,000-10,000 donors/batch. Diversity of Abs from large donor pool maximizes effect
slide59
IVIG
  • Mechanism of action- unknown

? Antiidiotypic antibody action

? Inhibition of cytokines

? “Sponging” of complement

? Binding to Fc receptors so macrophages

can’t bind

slide60
IVIG
  • Dosage: 0.4 gms/kgm/day x 5 c each dose given over 3-4 hours preceded by IV diphenhydramine &/or po ibuprofen
  • Caution c renal insufficiency or IgA deficiency
  • 38 Center trial in 1997
  • Equal to plasma exchange
j h c 48 yo welder
J.H.C. 48 yo welder
  • June ’02 H.A. followed in 2 wks by Lt. Facial weakness
  • June ’03 Rhinorrhea & cough
  • August 6 Pain lt. Hip spreading over a few days to back 7 legs
  • August 15 Legs buckle c lt.facial weakness 1 wk later. LP c protein of 70. NCVs c prolonged F waves
slide62
1 Week post discharge, elevated titers to West Nile Virus
  • Follow up at 1 month- continued improvement