GUILLAIN BARRE SYSYTEM (GBS)
GUILLAIN BARRE SYNDROME (GBS)
• Worldwide, no sex, age predilection
• Preceded by GI or respiratory infection 1 to 3 weeks in 60% of
cases, post vaccinal
• Campylobacter jejuni- most frequent identifiable
• 1.7 cases per 100,000 per year
• Autoimmune
• Anti-GQ1b (ophthalmoplegia)
• Anti-GM1
 A disease wherein the main problem is demyelination of spinal
nerve root or peripheral nerve
 This is a disease that is not so common
 Not an infectious illness
 This is a post infectious demyelinating type of illness
 If your patient has fever at the time neurologic deficit manifest
itself, then most likely you are wrong in considering GBS
 It can be a emergence of a vaccination
 But the experts are saying that fear of GBS as complication of any
vaccination is not enough to discouraged the use of immunization
 Most frequently, identifiable etiologic agent or most identifiable
bacteria that is culture in patient with GBS is CAMPYLOBACTER
JEJUNI (most documented)
 However, theoretically all of these microorganisms can cause GBS
(CMV, EBV, HIV, MYCOPLASMA, LYMPHOMA)
 Antibodies that will be secreted by the person
 Antibodies against gangliosides
 So the gangliosides is the component, major component of the
myelin sheath
 2 types of antibodies
o Anti-GQ1b (ophthalmoplegia)
o Anti-GM1
 In Miller Fisher form of GBS, this is the antibody that is most
frequently found in pts. With GBS
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NEUROLOGY
Clinical features
o Cardiovascular autonomic instability
o Dysautonomia
o Respiratory depression
o Immobility
o Facial diplegia
 The unique – facial diplegia – patient with bilateral weakness of the
face and the weakness involving the upper and lower facial muscles
 Bell’s palsy – wherein the upper and lower facial muscles are weak
but this time it is bilateral
 It may start unilaterally on one side of the face
 Pt. who initially diagnosed with Bell’s palsy always give a warning
that is now the right side of the face that is paralyzed
 In the subsequent days, there must not be any involvement of the
opposite side because if there is, you ask the pt. to come back
because Bell’s palsy is the wrong diagnosis
 It means it started with unilateral facial weakness, therefore there
is also a weakness on the opposite side
 In the Philippines, until proven otherwise, GBS is a consideration
when you have facial diplegia
 What are the other manifestations? Demyelination of peripheral
nerve weakness – a prominent manifestation
 Sometimes, C4 nerve roots are affected such that you can have
weakness on the diaphragm, so this is one reason pt. needs to be
confine
 Weakness of the muscle necessitating the ventilator support in pt.
with GBS
 Cardiovascular autonomic instability – increased BP, decreased BP,
bradycardia, tachycardia; needs monitoring, needs to be confine
 Other forms of dysautonomia: orthostatic hypotension, altered
sweat pattern
 GBS means spinal nerve root or peripheral nerve root involvement
 Sensory nerve or spinal nerve will have 3 functions: sensory, motor,
autonomic
 In here, you have 2- motor and autonomic manifestations, so what
is missing? The sensory
 So in pts with GBS, they may not have sensory complaints, because
you have pure motor forms of GBS
 But in some pts, may have sensory complaints in form of
dysesthesia
 DYSESTHESIA – tingling sensation or pins and needles sensation in
the digits
 A definite level of sensory loss, “mula po dito sa leeg ko pababa
walang pakiramdam – NOT A GBS!
 Because the sensory level is not that definite in a pt. with GBS
 In contrast to pt with TRANSVERSE MYELITIS, the problem is in the
spinal cord so meron ng autonomic dysfunction in the form of
bladder and bowel and definite level of sensory loss which are all
absent in pt. with GBS
 Autonomic manifestation do not come in form of bladder and bowel
 Alteration in heart rate, alteration in sweat pattern, alteration in
blood pressure but not constipation, not urinary retention
Probable cellular events in GBS
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 GUILLAIN BARRE SYSYTEM (GBS)
A - Attachment of lymphocyte to endoneurial vessel
With transformation
B – Demyelination with axonal sparing
C – Lymphocyte and PMN; Axonal death
D – Intense root or nerve lesion dissolution
 Infiltrations of axon by lymphocytes and eventually inflammatory
process will now cause demyelination
 There’s now dissolution of myelin in the portion of axon
 In most instances, the axon is spared, only the myelin is stored
 Main pathology of GBS is demyelination – segmental
demyelination
 In some pts., there are too much inflammatory infiltrate that causes
destruction of the axon
 Destruction of the axon comes much later than demyelination
 Axonal degeneration as consequences of demyelination
Required:
 Progressive weakness of 2 or more limbs due to neuropathy
- Weakness due to neuropathy – symmetrical, distal
- Usually 4 – most cases
- MOST IMPORTANT CRITERIA
- Hyporeflexia – areflexia
- Hx: progression of weakness in less than a month
 GBS is also known as ACUTE INFLAMMATORY DEMYELINATING
POLYNEUROPATHY (AIDP)
 Why less than 4 weeks?
 Opposite to that, we have CIDP (CHRONIC INFLAMMATORY
DEMYELINATING NEUROPATHY)
 Weakness is progressive for more than 4 weeks
Supportive:
 Mild sensory involvement – no definite level of sensory loss
 Kasi kung meron definite level of sensory loss, extreme na yun!
 Post infectious event, there must be an absence of fever
 If ever there is a fever, make sure you have known focus
Hindi lang motor weakness ang meron, meron pala UTI because the
pt. is more than 1 week bedbound, may have pneumonia as
complication
 Pt. should be sick of something else to explain the fever
 In GBS, the pt should be afebrile
 You can still do your lumbar puncture when you considering GBS
 Lumbar Puncture is acellular – no elevation of WBC
Pleuocytosis is not present
 The normal WBC count of CSF – less than 5 at the most to less than
10
 Increase in protein level – not due to increase in WBC but due to
increase in immunoglobulin lead to increase in IgG level
 Nerve Stimulation test – slow conduction velocity
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NEUROLOGY
Latency of nerve conduction should increased
e.g. give stimulation now at 3.11, kalian dapat magreresponse?
 Demyelination – later ang pagresponse ng nerve
 Latency – before the nerve fires will be much later as compared to
person with normal nerve latency
 Recovery usually begins in 2-4 weeks following the plateau phase
 Nagwoworsen siya, progressive weakness, after that no additional
symptom anymore
 There will be improvement in 2-4 weeks after non progression
 Unti – unti lalala tapos mararating yung pinakagrabe wala ng
maadadagdag – PLATEAU PHASE
 2 weeks from plateau phase expect improvement
 Aside from cranial nerve VII – facial nerve – involved in 70% of the
time, other cranial nerves that can be involved are XII, X, III, IV, VI
and XI
 Several types of GBS
 Classic GBS – Acute inflammatory demyelinating
polyneuropathy
Pathology: demyelinating
 AMAN – Acute Motor Axonal Neuropathy
 AMSAN – Acute Motor Sensory Axonal Neuropathy
 How will you know clinically?
 You will know it through electrophysiologic studies
o Very hard clinically to distinguish the 3 syndromes
o Theoretically, sensory and motor deficit siya; puro motor
siya
 Miller Fisher Syndrome – variant of GBS
 Very uncommon
 Ophthalmoplegia – hallmark
- Extraocular muscles are paralyzed
- Hindi gumagalaw ang mata ng pt.
 Peripheral nerves caters to proprioceptive cells
 There is demyelination
 Ataxia – sensory
 Expect the patient will POSITIVE Rombergs
 As a variant of GBS, dapat andun yung second
component of the criteria – areflexia
 Antibody that is elevated – anti GQ1B antibodies
Treatment:
IV Ig 0.4 mg/kg/day for 5 days (2 GM/KG)
Plasma exchange within first 2 weeks of onset of symptoms 200 to 250 ml/kg
of plasma per exchange 4-6 exchanges on alternate days
 Mas mabigat ang pasyente, mas mapapamahal siya
 IV Ig – very expensive; available in the Philippines
 Alternative treatment: Plasmapheresis / plasma exchange
 Sulit yung gastos if you catch the pt. within 1 week of the onset of
the symptoms
 Better prognosis
 More than 1 week, parang sugal. Gagastos pero not guarantee pt.
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 GUILLAIN BARRE SYSYTEM (GBS) NEUROLOGY
will improve - Chewing may become difficult
 Best given during 1st week if you resolve (7-14th day) - Severe jaw weakness may cause jaw to hang open
 Beyond 2 weeks, wag na ibigay, walang silbi - Swallowing may be difficult and aspiration may occur with fluids,
 The expense for plasma exchange and IV Ig are almost the same coughing and choking while drinking
 Advantage of IV Ig – needs a single instrument – Intravenous line;
convenient Neck flexors
 Plasmapheresis – needs a machine; tertiary hospitals - Neck flexors affected more than extensors

 Course of GBS: SELF LIMITED  Other manifestations:

o Easily recover if the pt. is on respirator plus - Nasality of voice “nangongongo”
administration of IV Ig - Episodes of nasal regurgitation
o Worth ang gastos kasi you lessen the complication and - Muscles innervated by the cranial nerve
increases the chance of improvement when you give IV - Facial muscles – muscles most affected by myasthenia
Ig, if the pt. can afford the IV Ig - Chewing may become difficult
o Ikakamatay ba niya kung walang IV Ig? Puwede – kung - Dysphagia
hindi mo sinupport yung respiration, kailangan naka- - Episodes of coughing and choking while drinking
ventilator
o If there no diaphragmatic weakness, only skeletal  REMEMBER: In neuro, it is harder to swallow liquid, it is much
muscle weakness, puwede na hindi mag IV Ig kung easier to swallow solids
talagang hindi na kaya as long as the pt. understand the - Mas nasasamid, bakit?
consequences - Kasi yung liquid pag nandyan na sa bibig, hindi maidirect
to the left / right, sumabog yung tubig sa bibig mo
MYASTHENIA GRAVIS - For example: STROKE
If the right side is weak, you can always add aid (?) on
Epidemiology difficulty on swallowing, if you instruct the relatives to
place the food in the mouth opposite the weak side, pag
- F-M (6:4) nag chew, ang gagalaw nasa strong side, paglunok niya,
maitutulak ng dila yung food niya sa left which is the
- Mean age of onset (M-42, F-28)
strong side
- Incidence peaks M: 6-7TH decade; F – 3rd decade - Water paano itutulak on one side? Naka scatter sa
tongue
Risk factors: Age >40, thymoma
 Trademark / hallmark of myasthenia
 Neuromuscular junction weakness - Diurnal variation
 Lower motor neuron type of weakness - Within the day, there is variability in the type of
 Age of onset in female – reproductive age group – more prone to weakness
have myasthenia - The dictum is the muscle becomes weak when it is used,
 Male much older muscles becomes strong when the muscle is at rested
 Presence of thymoma is believe to make pt. prone to have - Unang kausap mo yung pasyente, malakas yung boses
myasthenia niya, klaro, 30 minutes history taking napapansin mo
 In the absence of thymoma, still recommending that some pts. Is nagiging nasal na siya tapos humihina na yung boses
subjective to thymectomy niya
 Pag may tumor, automatic you will do the thymectomy - If you do ROS, kalian po kayo nasasamid? Pagsimula pa
 There are some pts who will benefit from tymectomy in the absence lang ng pagkain o yung malapit na kayo matapos
of thymoma kumain?
 What’s in the thymus gland? Myasthenia is considered as an - Initially becomes weak?
autoimmune disease - The muscle with normal activation of acetylcholine when
 Antibodies screened (?) by the thymus gland against acetylcholine it starts to contract for the first time and over time as
receptors you repeatedly used the muscle it is static, so there is no
 Acetylcholine will not be able to bind with the acetylcholine replenishment of acetylcholine, so lalo siya manghihina
receptor, then your pt. will become weak
 Pathophysiology
Clinical Presentation  Thymus gland producing T cell antibodies
 Your T cell produces acetylcholine receptor antibodies
 Facial muscle weakness is almost always present which reduces normal neurotransmission
 Ptosis and bilateral facial muscle weakness  How does this happen?
 Sclera below limbus may be exposed due to weak lower lids  Alteration of the function of the receptor by blocking
acetylcholine binding and acetylcholine receptor
 The hallmark is the facial muscle weakness that is almost always activation
present  Promote endocytosis of acetylcholine resulting to
 Most affected part of the face is the eye degradation of acetylcholine receptor
 Ptosis – unilateral or bilateral but in most cases, bilateral  And the effect when you have these antibodies it
 Sclera below limbus may be exposed due to weak lower lids – destroys post synaptic surface leading to a decrease
parang nagkaroon ng ectropion (can also be manifestation of lower number in acetylcholine receptor
lid weakness  To make it simple, acetylcholine receptor are not
 What is affected? Levator palpebrae, extraocular muscle that functioning well
becomes weak  So even if you have acetylcholine in ytour system, it will
 Pupils are always normal not exert its biologic effect because the attachment site
which is the receptor is dysfunctional so eventually with
Bulbar muscle weakness repetitive use of the muscle, you have muscle fatigue
- Palatal muscles
- Nasal voice, nasal regurgitation

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 GUILLAIN BARRE SYSYTEM (GBS) NEUROLOGY
Ocular movement

Frequency: 50-75% of initial presentation

Ocular myasthenia gravis - 90% of cases

Ptosis – unilateral / bilateral

- worsens with prolonged upgaze

Diplopia – simulate any cranial nerve paresis

- supranuclear

 So these are the clinical manifestations

 Diplopia and ptosis – clinical manifestations that can
create in your clinic
 Yung maneuver sa EOM ulit ulitin mo yan and eventually
yung pt mo will have diplopia
 Patingin mo lang siya sa kisame “tingin lang po kayo
diyan sa ksiame steady lang po huwag ibababa yung
mata
 Later on makikita mo there will be ptosis
 Pumasok siya sa clinic mo n walang diplopia, walang
 How do you diagnose myasthenia?
ptosis
 If the patient for example has hyperthyroidism or
 Resting of the muscle will improve the muscle strength
hypothyroidism or a throat infection; these symptoms
 Pikit po kayo, wait for 5 minutes, pagdilat niya ulit, wala may exacerbate the symptoms of myasthenia gravis
na yung ptosis, wala na yung diplopia
 Pag nagisip kang myasthenia, irule out mo hypothyroid
 This is a reproducible type of weakness ba o hyperthyroid
 Ang problema na lang ay kung related to the voice,  Second, under any other autoimmune illnesses present in
pwede mo siya pabasahin ng diyaryo, matagal na ano the patient because the patient can also have this
yun disease concurrent with myasthenia
 Any infection will now exacerbate myasthenia symptoms
 Kapag may sipon o ubo sa family mo, lumayo na sila
sayo at ikaw na may myasthenia, mag mask ka na
 So reverse isolation

Modified Osserman’s Classification

Class 1 Patients with ocular involvement alone

Class 2 Mild weakness, not incapacitating without oropharyngeal or
respiratory muscle involvement
Class 3 Moderate weakness, not incapacitating including oropharyngeal
and respiratory muscle weakness
Class 4 Incapacitating weakness of any muscle system incl. oropharyngeal&
respiratory muscle weakness
Class 5 Life-threatening respiratory insuffiency requiringventilatory
assistance
 Normal postsynaptic area, you have the normal appearance of
acetylcholine receptors  This is how you classify myasthenia gravis
 But in myasthenia, decrease number in acetylcholine receptors and  The higher the number, the more severe the myasthenia
you may now have widened synaptic spaces, ito narrowed lang siya  So these are pts with ocular involvement alone – CLASS 1
diba? (normal)  In CLASS 2, mild weakness without oropharyngeal or respiratory
 So there’s really a problem in the acetylcholine receptor
muscle involvement. So kumbaga meron siyang skeletal muscle
problem
 Feeling kop o mas mahina ako sa hapon kaysa sa umaga
 But when you check the brainstem and brachial muscles
are normal
 In CLASS 3, mas mahina na siya but this time there is also weakness
in oropharyngeal and respiratory muscle
 CLASS 4 – bakit severe weakness? So parang ang differentiating line
mo is there an involvement of muscles of mastication, muscles of
swallowing and respiratory for you to decide that the pt. has also
has already a more severe type/ severe class of myasthenia

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 GUILLAIN BARRE SYSYTEM (GBS) NEUROLOGY
 Repetitive Nerve stimulation also known as Jolly Testing
 A greater than 15% in the decrement
 When you give titanic stimulation to your pt. and there
will be new muscle taht is contracting and there will be
an amplitude
 For example, ang amplitude ng muscle contraction – 10
mm, you can stimulate the muscle further so papagurin
mo yung muscle diba?
 Overtime, you expect there should be a lower amplitude
of muscle contraction
 As you repeatedly stimulate the muscle, and the
decrement that it should be more than 15%
 So kunwari 10 millimeter yung initial amplitude,
overtime nagging 5 mm na lang siya, e di positive yun
 POSITIVE JOLLY TESTING / REPETITIVE NERVE
STIMULATION

 Single fiber electromyography

 One way of diagnosing myasthenia
 Much better than repetitive nerve

DIAGNOSIS OF MYASTHENIA
Lab Testing:
Anti AChR immuno assay - 85% generalized MG
Repetitive Nerve Stimulation
Simple fiber electromyography
Edrophonium Chloride
Ocular / cranial MG: exclude intracranial lesions by CT/MRI
 So how do you diagnose?
 Aside from history, evidence like fluctuating weakness –
yun yung criteria sa history
 Fluctuating weakness – how does it fluctuate? Simple,
relieved by rest, aggravated by exercise
 You can examine for anti acetylcholine receptor
antibody so this is 85% positive in pts. With myasthenia
 However if the myasthenia symptoms is only ocular, it is
50% positive only.
 If the symptom is ocular, it is definitive diagnosis if
positive
 Negative result does not exclude myasthenia gravis
 In some pts with generalized myasthenia who have
antiacetylcholine receptor antibody negative, you can
request for an anti MuSK antibodies (muscle skeletal
antibody)
 Meron dito na acetylcholine receptor antibody assay
JOLLY TESTING
HYPOTHENAR MUSCLES
DECREMENT
INCREMENT
 Tensilon Test
o where you inject Edrophonium chloride to your patient
o halimbawa, pinagod pagod ko di ba? Tinakpan, nag
ptosis siya, so anong puwede kong gawin?
o Puwede ako maginject ng Edrophonium chloride
o So instead of waiting for 5 minutes na ipaparest ko
o Pag binigay yung Edrophonium Cl within 1 second yung
ptosisniya nagdisappear
o So now a POSITIVE TENSILON TEST
o Yiung muscle na nagging weak, nagimprove when you
give Edrophonium HCl , so this is an acetylcholine
agonist
 Sabi natin, meron tayong thymoma, usually in pts diagnosed with
myasthenia gravis, we oftenly request for CT SCAN of the chest kasi
titingnan mo yung state ng thymus gland
 Ito kasi this is specific already for the diagnosis of myasthenia
 There are other myasthenic syndromes, so pag napunta ka ng
Africa / Bukidnon, pwede ka makagat ng ahas
 Do you remember that snake venom have the same effect as
myasthenia because snake venom and myasthenia are post
synaptic acetylcholine receptor defect
 Yung presynaptic, uso pa ba to? Botulinum toxicity
 E dib a tayo pag obvious naman pag deformed ang lata,
dina nga natin binibili diba? Lalo na kung lumolobo na
yung lata
 Remember, Clostridium is a gas forming microorganism,
so it will now cause expansion of the can which is the
food that has been expired usually
Lambert Eaton Myasthenic Syndrome (LEMS)
 Seen this in pts with bronchogenic Ca
 Manifestation is neuromuscular junction dysthesia but the problem
is presynaptic
 The result of Jolly testing is incremental because the problem in
neurons is the release of calcium
 Habang ginagamit mo yung muscle sa legs, lumalakas yung muscle
which is in contrast to myasthenia habang ginagamit mo yung
muscle nagweweaken yung muscle
 An incremental response is POSITIVE FOR LEMS
 A decremental response of more than 15% is POSITIVE FOR
MYASTHENIA
As a general physician, ito yung gusto ko matandaan mo kasi baka ikaw pa
makapatay ng pasyenteng may myasthenia
Ano ang tatandaan mo bilang doctor?

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 GUILLAIN BARRE SYSYTEM (GBS)
 Very common: Sino ba doctor hindi nagpprescribe ng
antibiotics?
 Antibiotics na BAWAL
 Aminoglycoside – MYCIN!
Streptomycin – Pulmo
Tobramycin - Optha
 Quinolones – Ciprofloxacin ; favourite
Levofloxacin – Pneumonia
Ofloxacin – UTI
 Macrolides – pts who are allergic to penicillin
We give Erythromycin
Azithromycin – Community acquired pneumonia
 I always tell my patient whenever you consult another doctor, isa
agad sa sasabihin mo “Ako po si Juan Dela Cruz, may myasthenia
po akjo para magulat agad yung doctor, making agad siya sa iyo,
kasi it is very uncommon illness, kailangan aware yung doctor na
may kakaiba kang sakit
 Non depolarizing muscle relaxants for surgery
 Usually hindi naman natin ginagamit ito –
anaesthesiologist
 Forget it kung di naman anaesthesiologist
 Beta Blocking agents
 Even in psychiatry gumagamit ako ng propranolol as
anti anxiety drug
 Do not forget that ity is a drug that exacerbate
myasthenia
 Cardiologists uses procainamide
 Surgeons uses xylocaine – a local anesthetic pero good news in
large amount lang siya magkakaroon ng effect
 Paano po ako magpapaganda e may myasthenia ako?
 Botox injection that you need to remove wrinkles pwede
pa din naman kahit may myasthenia kasi ang effect ng
botox toxin e very local siya
 Pag ngumiti ka may wrinkle ka ditto (eyes) gusto mo
ipatanggal kahit may myasthenia ka, pwede pa naman,
kasi yung ayaw natin yung muscles of respiration and
mastication
 Clhloroquine – Malaria; Infectious disease specialist
 Magnesium – OB – Eclampsia
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 Magnesium sulfate – it can exacerbate symptoms of
myasthenia
 Monitor for reflexes ; If it is down then you are reaching
toxicity level
 Penicillamine – anti rheumatic arthritis not very used
frequently
 Edrophonium (Tensilon Test) – although it is a good drug, not only
exclusive to myasthenia
 It can improve also the strength of muscle with
Amyotrophic lateral sclerosis, polio and some peripheral
neuropathies
 Effect is not that instantaneous
 Kumbaga kung myasthenia ka halos instantaneous
talaga parang kumurap ka lang gumaling na yung
weakness ng patient
Treatment:
 AChE inhibitors
 Immunomodulating therapies
 Plasmapheresis
 Thymectomy – if thymoma is present
AChE Inhibitor
Pyridostigmine bromide (Mestinon)
 Starts working in 30-60 minutes and last 3-6 hours
 Individualize dose
 Adult dose: 60-960 mg/d PO; 2 mg IV/IM every 2-3 hours
 Caution: cholinergic crisis
Immunomodulating therapy – Prednisone
 May problema yung acetylcholine receptors, so you want to prolong
the biologic effect of acetylcholine
 So you now give esterase inhibitors / ACE Inhibitor, pyridostigmine
 Immunomodulating therapy – give your steroids – Prednisone
 You can have your plasmapheresis from time to time especially if
you have myasthenia crisis, yung sobrang hina ng pasyente mo,
gusto mo matanggal yung mga antibodies diba?
 Thymectomy can be an option if there is thymoma
 Pyridostigmine – available in the Philippines
 Starts working in 30 miutes to 1 hour
 Effect last for 3-6 hours
 So kung nagbigay ka niyan, tatanong mo kung maagap
ba kayo sa susunod na dose which is presumably 6
hours
 Usually kasi malayo ang interval , mag 6 hours ka
muna ngayon
 When the pt comes back for the follow up bot ba yung
gamut mo? Malakas po ba siya? Hanngang 6 hours
kung hindi, e di you lessen or shorten the interval kung
dati every 6 gawin mong every 4 hours
 Drug is given per orem, as maintenance basis, no
parenteral preparation
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 Neostigmine – available in parenteral preparation
 So in our oatient, oral medication – Pyridostigmine,
brand name worldwide (Mestinon)
 Prednisone – you want to suppress the immune system so there will
be less antibody formation
Complications:
 Respiratory failure
 Dysphagia
 Complications secondary to drug treatment – long term steroid use
 Osteoporosis, cataracts, hyperglycemia, hypertension
 Gastritis, peptic ulcer disease
 Pneumocystis carinii
 Complications will be base on how the weak the patient is, in terms
of muscle weakness
 Respiratory failure – diaphragm is so weak
 Dysphagia – weakness of muscle of mastication, muscle of
swallowing
 But the most complication that will see is the long term use of
steroids, kasi these are the pts you maintain for life in steroids
 It is also give your immunosuppressant – Methotrexate
 But usually when we discuss methotrexate to pts, mas gusto nila
yung steroids kasi it is cheaper than methotrexate
 Look at the side effects: gastritis, peptic ulcer disease, osteoporosis,
cataract
 It induces the blood level of your pt., retain more sodium, so you are
inclined to have hypertension
 Make sure patients take calcium supplements
Consensus Statement PNA Benefit from Thymectomy Level 2B
– GENERALIZED MYASTHENIA GRAVIS
– AGE BET. PUBERTY AND 60 YEARS
– FEMALE GENDER
– DURATION OF DISEASE WITHIN 2 YEARS FROM ONSET
– POSITIVE ANTI-ChR Ab
 The Philippine Neurologic Association has this consensus with
regards to thymectomy
 If the ages between puberty and 6o years old, relatively the patient
is female
 Onset of myasthenia quietly within 2 years
 Positive acetylcholine receptor antibody
 Has generalized myasthenia gravis
 Pag ocular lang yung myasthenia you are not going to undergo
thymectomy
 CLASS 2-4, pwede magthymectomy
 As long as you fulfil all the other criteria
MYOPATHY
Myopathy vs Dystrophy
 Hypothyroidism – weakness, cramps, pain and stiffness,
muscle hypertrophy
 Slow contraction and relaxation (25%)
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NEUROLOGY
 Hyperthyroidism – proximal weakness with atrophy
 Adrenal disorder – glucocorticoid excess causes
myopathy associated with muscle wasting
 Hyperaldosteronism (Conn’s) – potassium depletion
 “Channelopathies” – problem in sodium, potassium and
calcium
 Dystrophy – inherited muscle disease
 In adult neuro, what we will have are myopathies
 Pediatrician / pedia neuro, they will have dystrophic
cases
 May history taking ka, you have a child patient who
have problems related to ambulation, siya lang ba yung
meron hganyuan sa pamilya? Yung kapatid ba niya may
ganun?ang diagnosis mo ay DYSTROPHY
 Myopathy – acquired muscle disease
 You have now endocrine disease that can present as
myopathic symptoms
 You have hypothyroidism, hyperthyroidism,
hyperparathyroidism and hypopoarathyroidism
 Pag may problem ka sa thyroid, may problem ka sa
parathyroid , rather it is up or down, you can have
myopathic symptoms
 In adrenal disorders, if you have chronic glucocorticoid
intake, it says that chronic intake of steroids can weaken
your muscle called STEROID MYOPATHY
 Kaya minsan in a patient with myasthenia sometimes
yan yung difficulty dalawa yung nagweweaken – disease
itself – yung impaired acetylcholine receptor and the
consequence of chronic steroid intake that you can cause
steroid myopathy
 In pts with hyperaldosteronism, you can produce
hypokalemia so meron muscle weakness
 Channelopathies - problem in sodium, calcium and
potassium channels
 Very popular: HYPOKALEMIC PERIODOC PARALYSIS
(HPP)
 Pero in HPP, you have a problem in calcium / sodium
channel
 Ang problem in sa sodium o potassium for HPP, normal
ang potassium channel ng may hypokalemia just so
happen when they measure the electrolytes of these
patients the level of potassium are low
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 GUILLAIN BARRE SYSYTEM (GBS) NEUROLOGY
 But they are not able to explain how low the potassium
brings about muscle weakness, there is no physiologic
explanation

 All of your channelopathies are inherited by autosomal

dominant
 Kahit na common clinically ang HPP, hindi puwede
sabihin lahat may myopathy
 Ano ba ibig sabihin ng myopathy? Proximal muscle
weakness
 Yung braso mahina pero it is wrong na nakakita lka ng
pt. na may proximal muscle weakness ay bigyan mo ng
potassium kasi hypokalemia yan, MORTAL SIN naman
yun
 Hintayin mo muna yung potassium level kasi meron
opposite nun, HYPERKALEMIA PERIODOC PARALYSIS
and the presentation is also myopathy
 Clinically parehas sila nagiging weak so pag nag bunung
braso weak yung proximal whether it is hypokalemia o
hyperkalemia in terms of same level of potassium
 Baka makapatay ka ng pasyente kung automatic ka
magbibigay ng potassium

 Ano ba yung myotonic? Inability of the muscle to relax

 PERCUSSIION MYOTONIA – get the reflex hammer,
percuss the muscle , once you percuss you will see the
muscle rippling
 Sige po bukas sara, yung myotonia gusto na
ng pasyente ibukas yung kamay niya pero
ayaw bumukas
 Ganundin yung paramyotonic congenita
 “Mama, para! Para!”Humawak ka sa estribo,
ayaw bumitaw ng kamay mo ayaw magrelax ng
muscle mo at the time you want your muscle to
relax
 Lahat sila episodic weakness
 In a patient with hypokalemia, what are the aggravating
factors?
 There is the type of weakness precipitated by
high carbphydrate load
 Monday – nagccramps, masakit yung paa at
legs
 Tuesday – hindi na siya makatayo, go back what
happen nung weekend
 Saturday – basketball, feel na feel! idol si
Pingris!
 Ano nangyari sa kanya? Ang init. Nakaubos ng
1L ng coke at 1 loaf ng pan de Americano, isang
mangkok na pansit – HIGH CARBOHYDRATE LOAD
 Sunday-Monday – brassy (?) sensation, feeling
niya tagal na hindi nagbabasketball, natural lang to\
 Tuesday – hindi na makatayo, so hypokalemia
yun, OK?
 Ito yung effect ng potassium loading
 If it is hyperkalemia – increased myotonia then
eventually weaken
 Fibric Acid derivative – finofibrate
 HMG CoA reductase inhibitor – STATIN
 Niacin (nicotinic acid)
 Lahat yan pwede mag aggravate ng myopathic
weakness
 Kung dati wala, ngayon nanghihina, tanungin
mo, may gamut ka ba iniinom pang pababa ng
cholesterol?
 Are you taking any form of steroids?
 With drugs of abuse can lead to muscle breakdown,
rhabdomyolysis, myoglobinuria
 Para na siya naghazing diba?
 It should be chronic use
TO SUMMARIZE
 Peripheral nervous system – can affect the periphreral nerve,
neuromuscular junction and the muscle
 Anterior horn cell – Polio, amyotrophic lateral sclerosis
 Weakness:
Peripheral nerve – distal, usually symmetrical
Muscle – usually proximal
Neuromuscular – proximal; cranial nerve innervated muscles, so
bulbar
 Sensory deficit – peripheral nerve lang, all the rest motor in function
 Autonomic – peripheral nerve, parang endocrine na to ha;
 Peripheral nerve – para na rin siyang sakit ng spinal nerve
 From your spinal nerve comes your peripheral nerve so autonomic
reflexes yun
 Autonomic signs : decreased sweating, decreased tear, altered
temperature
 Reflexes of peripheral nerve – hyporeflexia
 Peripheral nerve disease – exemplified by GBS

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 GUILLAIN BARRE SYSYTEM (GBS) NEUROLOGY
 Muscle disease – HPP
 Reflexes are usually down in lower motor neuron disease
 The weaker the muscle, that will be the grade of the reflex
 Kung malakas pa yung muscle, kunwari yung NMT mo is 4/5 lang of
the proximal, ito 5/5, so iniisip mo pwedeng normal pa yung reflex,
relatively malakas pa yung muscle
 Pag nagging 0/5, as the grade of NMT lowers, that was the time
there was a diminution in your reflexes

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Et factum estutamicistranscribit 2014 -2015

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durumsimul in unum! medicine vade MARIE