1. Miller Fisher Syndrome
ELECTRODIAGNOSIS
Dr Mohamed ibrahim khalil
physical medicine Rheumatology
Alexandria University, EGYPT
To contact Dr M. Ibrahim
Reverie-forever@hotmail.com

3. History
A 24 year-old female patient , married housewife
from el Behira presented with:
• Headache
• Gait instability
• Right Facial weakness
• Squint of Left eye with diplopia
• Numbness of both upper and lower limbs
• Dysphagia
Of 2 Months Duration

4. The condition started 2 months ago with
subacute onset of :
• Continuous headache: occipital , bursting ,
awaken the patient from sleep and not
responding to analgesic .
• Nausea and vomiting , not related to meal
and usually at the peak of headache .
• Blurring of vision
• Numbness at the back of the neck and upper
limbs.

5. A week later , the patient experienced :
• Generalized body numbness
• Difficulty in handling objects by both hands
• Gait instability and difficulty in getting
upstairs

6. ** She sought medical advice , CT Brain was done
which revealed normal findings , and Fundus
examination showed mild papilloedema .
** She received analgesics vitamins and
Performed Hagama with No improvement .

7. After 10 days , she developed
• Weakness of Right facial muscles:
1- Inability to close Right eye.
2- Accumulation of food on right side.
3- Drippling of saliva from right side.
4- Deviation of mouth to the left side .
• Mild dysphagia for solid food.
• Left eye squint with diplopia.
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She was admitted to El-Hadara University Hospital – Neurology
Department

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No history of similar attacks
No history of head trauma
No history of fever, common cold or diarrhea
No history of deafness , tinnitus or vertigo
No sphincteric disturbance
No history of change of consciousness
No history of convulsions
NO history of involuntary movements
No history of dysarthria or dysphasia

9. • Past Medical History : NO DM - NO HTN
• Family History : Irrelevant
• Vital signs :
Pulse 95 b/min
Respiration 16 /min
Blood Pressure 125/90
Temperature 37 C

10. Physical examination
1- Mental or intellectual function:
• The Patient is fully conscious, alert ,well
oriented to time , person and place and
with intact memory and average
intelligence .
2- Speech and articulation : Normal

11. 3- Cranial nerves :
Right facial nerve LMNL
Left Abducent Palsy other cranial nerves are clinically free

12. 4- Motor examination :
• Muscle state : Normal
• Muscle Tone :HYPOTONIA in both upper
and lower Limbs
• Muscle Power :Grade 5 all over .
• No fasiculations
• No involuntary movements

13. 5- Reflexes
A) Superficial reflexes :
Abdominal : absent
Planter : FLEXOR
B)Deep Reflexes :
AREFLEXIA In upper and Lower limbs

14. 6- Coordination:
Mildly affected finger to nose test and heel
knee test more on the Left side
Dysdiadikokinesia more on the left side
7- No Involuntary movement:

15. 8- Sensory examination :
a) Superficial : Normal in UL and LL
b) Deep:
*Sense of movements : Normal
*Sense of Position : ? Affected at
left side
*Vibration sense : intact
c) cortical sensation : normal

16. 9- Gait :
Gait instability with difficult walking along a
straight line
10- Romberg sign : negative

18. Investigations
• CT Brain  Free
• MRI Brain  Free
• Cervical MRI  Free
• MRI and MRV using open Magnet  unremarkable
• Fundus examination showed Mild
Papilledoema
• CSF  Free
• CSF Pressure Increased

22. Current treatment
• Gabapentin 300 mg / 8h
• Methyl Prednisolone 60 mg daily for 2 w
then tapering
• Vitamin B 6,10,12
• Paracetamol 500 mg if nessecary
• Local treatment for the right eye

24. SUM UP
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PERIPHERAL
Gait instability
Numbness of both UL
and LL
Hypotonia
Areflexia
Flexor Planter
No Sphincteric
Right facial LMNL
Left Abducent
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CENTRAL
↑ ICP , Headache ,
vomiting, Papilledema
Right facial LMNL
Left Abducent
Dysphagia
Incordination
Gait instability
• MRI and CSF FREE
Is there any sure sign of Upper motor neuron Lesion ?
Could hypotonia and areflexia be Present early in UMNL ?
The Sure LMNL is facial and abducent but can be present in
brain stem lesion
Could combination of LMNL + UMNL be Present ? DD
How can electrodiagnosis help in solving the Puzzle ?

26. What is the Role of electrophysiological study ?
1. Assess LMNL ( NCS , F wave , H reflex and EMG ) , to
Diagnose Guillian barre syndrome , CIDP and Miller
Fisher syndrome
** Assess Ia afferent fibers conduction
1. Exclusion of UMNL ( H/M < 50 % )
2. Assess central and Posterior column Lesion ( SEP )
3. Examine the functional integrity Brain stem
Reflex)
( Blink
4. Assess the nature of facial neuropathy and possibility of
trigeminal neuropathy ( Blink Reflex and facial NCS /
EMG )

27. Differential diagnosis
1. Miller Fisher syndrome associated with Benign
intracranial hypertension
2. Bickerstaff brainstem encephalopathy
3. Gillian barre syndrome associated with cranial
neuropathy
4. CIDP
5. Central Lesion ( brain stem – cerebellar )
6.
? Imflamatory , ? Demylinating
7. Idiopathic intracranial hypertension

28. (5) (6)
(7)
(6) (5)
(7)

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NB :
1- Negative rombergism
2- Increased Intracranial Pressure
3- Numbness of both UL and LL

43. Sensory 1a afferent
new technique for
electrodiagnosis
1.
2.
3.
4.
Submaximal
stimulation
Low Gain
Voluntary movement
Average analysis

44. • Follow up assessment after 4 days

47. What causes Lost H reflex
Bilateral with normal Peripheral
nerve conduction ?

48. Electrodiagnosis Results
1. Normal sensory and motor conduction and F
waves against the diagnosis of GBS or CIDP
2. Abnormal conduction of the Ia afferent fibers
3. Right Facial and Trigeminal axonal neuropathy
4. Lost H reflex on left side , Decreased amplitude
on right side .
5. The intact Left R1 ( with clinical evidence of left
abducens neuropathy ) is suggestive of intact
brain stem
6. Decreased SEP Amplitude (? ICP , ? Central
Lesion)

49. Diagnosis
• The Multiple cranial neuropathy , ophthalmoplegia (with
Free MRI study of the brain ) associated with abnormal
Ia afferent conduction is highly suggestive of :
Miller Fisher Syndrome
Associated with Benign intracranial hypertension

50. Explanation
• Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and Limb incoordination
• Abnormal SEP is attributed mostly to ↑
ICP rather than central Lesion
• Blink Reflex Pattern of right Trigeminal facial
neuropathy rather than Pontine Lesion
• H reflex changes attributed to abnormal Ia
afferent conduction , no evidence of UMNL

51. • Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and Limb incoordination
Lost H Reflex in Miller fisher
Syndrome

52. • Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and Limb incoordination
• A Reflexia in Miller fisher syndrome

53. • Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and Limb incoordination
• Ataxia in Miller fisher syndrome

54. • ?? Effect of steroid therapy on the Results
of electrodiagnosis

57. Pathophysioly of MFS
• Hypothesis of molecular mimicry

58. •
Infection by C. jejuni if also
implicated in the development of
MFS by the same mechanism of
molecular mimicry. MFS patients
present with symptoms similar to
other forms of GBS along with
problems controling eye
movements, or ophthalmoplegia.
Autoantibodies have been
isolated from these patients that
bind to human ganglioside GQ1b
as well as the GQ1b epitope
present within the LOS of C.
jejuni isolated from MFS
patients. Also, the dominant C.
jejuni serotypes associated with
MFS were HS:2 and HS:4. The
gene polymorphism associated
with the development of antiGD1b autoantibodies was found
to be cstII(Asn51) (Koga et al.,
2006). This provides a clear link
to the clincal presentation of
MFS since the GQ1b ganglioside
is found predominantly in human
oculomotor nerves (Ang et al.,
2004; Kuwabara, 2004).

59. GQ1b Antibody

60. Bickerstaff brain stem
encephalitis ( BBSE)
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Pathophysiology :
BBSE is caused by Autoimune Response against CNS
Clinical Picture :
Acute oncet of ophthalmopligia , Ataxia , Areflexia usually
associated with bilateral facial Palsy
Drowziness or Lost consiousness with affection of the Glasco coma
scale
In the Shock stage : Hypotonia , Areflexia and Lost H Reflex –( DD .
Miller Fisher syndrome )
After few days UMNL signs appear : Hyperreflexia , extensor Planter
Response
Free MRI Brain , CSF may show Increased Albumen : Low cell
Ratio
Lab : GQ1b antibody in serum and CSF
Good Prognosis and Recovery with Plasmaphareses and IVIG

61. Electrodiagnosis
• Lost H Reflex
• Decrease in the Somatosensory evoked
Potentials Amplitude
• Blink Reflex showing Poor response
• Peripheral sensory and motor conduction is
usually normal or very mild affection in the form
of decreased amplitude unrelated to SCV
• Affection of the sensory Ia afferent
• NB : Recent studies showed affection of the
cerebellum