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Miller Fisher Syndrome
ELECTRODIAGNOSIS
Dr Mohamed ibrahim khalil
physical medicine Rheumatology
Alexandria University, EG...
History
A 24 year-old female patient , married housewife
from el Behira presented with:
• Headache
• Gait instability
• Ri...
The condition started 2 months ago with
subacute onset of :
• Continuous headache: occipital , bursting ,
awaken the patie...
A week later , the patient experienced :
• Generalized body numbness
• Difficulty in handling objects by both hands
• Gait...
** She sought medical advice , CT Brain was done
which revealed normal findings , and Fundus
examination showed mild papil...
After 10 days , she developed
• Weakness of Right facial muscles:
1- Inability to close Right eye.
2- Accumulation of food...
•
•
•
•
•
•
•
•
•

No history of similar attacks
No history of head trauma
No history of fever, common cold or diarrhea
No...
• Past Medical History : NO DM - NO HTN
• Family History : Irrelevant
• Vital signs :
Pulse 95 b/min
Respiration 16 /min
B...
Physical examination
1- Mental or intellectual function:
• The Patient is fully conscious, alert ,well
oriented to time , ...
3- Cranial nerves :
Right facial nerve LMNL
Left Abducent Palsy other cranial nerves are clinically free
4- Motor examination :
• Muscle state : Normal
• Muscle Tone :HYPOTONIA in both upper
and lower Limbs
• Muscle Power :Grad...
5- Reflexes
A) Superficial reflexes :
Abdominal : absent
Planter : FLEXOR
B)Deep Reflexes :
AREFLEXIA In upper and Lower l...
6- Coordination:
Mildly affected finger to nose test and heel
knee test more on the Left side
Dysdiadikokinesia more on th...
8- Sensory examination :
a) Superficial : Normal in UL and LL
b) Deep:
*Sense of movements : Normal
*Sense of Position : ?...
9- Gait :
Gait instability with difficult walking along a
straight line
10- Romberg sign : negative
Investigations
• CT Brain  Free
• MRI Brain  Free
• Cervical MRI  Free
• MRI and MRV using open Magnet  unremarkable

...
Current treatment
• Gabapentin 300 mg / 8h
• Methyl Prednisolone 60 mg daily for 2 w
then tapering
• Vitamin B 6,10,12
• P...
SUM UP
•
•
•
•
•
•
•
•

PERIPHERAL
Gait instability
Numbness of both UL
and LL
Hypotonia
Areflexia
Flexor Planter
No Sphin...
What is the Role of electrophysiological study ?
1. Assess LMNL ( NCS , F wave , H reflex and EMG ) , to
Diagnose Guillian...
Differential diagnosis
1. Miller Fisher syndrome associated with Benign
intracranial hypertension
2. Bickerstaff brainstem...
(5) (6)
(7)

(6) (5)
(7)
•
•
•
•

NB :
1- Negative rombergism
2- Increased Intracranial Pressure
3- Numbness of both UL and LL
Sensory 1a afferent
new technique for
electrodiagnosis
1.
2.
3.
4.

Submaximal
stimulation
Low Gain
Voluntary movement
Ave...
• Follow up assessment after 4 days
What causes Lost H reflex
Bilateral with normal Peripheral
nerve conduction ?
Electrodiagnosis Results
1. Normal sensory and motor conduction and F
waves against the diagnosis of GBS or CIDP
2. Abnorm...
Diagnosis

• The Multiple cranial neuropathy , ophthalmoplegia (with
Free MRI study of the brain ) associated with abnorma...
Explanation
• Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and ...
• Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and Limb incoord...
• Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and Limb incoord...
• Sensory Ia afferent affection explains the
Hypotonia , Areflexia , lost H
Reflex , the
Gait
instability and Limb incoord...
• ?? Effect of steroid therapy on the Results
of electrodiagnosis
Pathophysioly of MFS

• Hypothesis of molecular mimicry
•

Infection by C. jejuni if also
implicated in the development of
MFS by the same mechanism of
molecular mimicry. MFS pat...
GQ1b Antibody
Bickerstaff brain stem
encephalitis ( BBSE)
•
•
•
•
•
•
•
•
•

Pathophysiology :
BBSE is caused by Autoimune Response agai...
Electrodiagnosis
• Lost H Reflex
• Decrease in the Somatosensory evoked
Potentials Amplitude
• Blink Reflex showing Poor r...
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
Miller fisher syndrome Electrodiagnosis
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Miller fisher syndrome Electrodiagnosis

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Transcript of "Miller fisher syndrome Electrodiagnosis"

  1. 1. Miller Fisher Syndrome ELECTRODIAGNOSIS Dr Mohamed ibrahim khalil physical medicine Rheumatology Alexandria University, EGYPT To contact Dr M. Ibrahim Reverie-forever@hotmail.com
  2. 2. 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
  3. 3. 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.
  4. 4. A week later , the patient experienced : • Generalized body numbness • Difficulty in handling objects by both hands • Gait instability and difficulty in getting upstairs
  5. 5. ** 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 .
  6. 6. 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. • She was admitted to El-Hadara University Hospital – Neurology Department
  7. 7. • • • • • • • • • 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
  8. 8. • 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
  9. 9. 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
  10. 10. 3- Cranial nerves : Right facial nerve LMNL Left Abducent Palsy other cranial nerves are clinically free
  11. 11. 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
  12. 12. 5- Reflexes A) Superficial reflexes : Abdominal : absent Planter : FLEXOR B)Deep Reflexes : AREFLEXIA In upper and Lower limbs
  13. 13. 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:
  14. 14. 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
  15. 15. 9- Gait : Gait instability with difficult walking along a straight line 10- Romberg sign : negative
  16. 16. 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
  17. 17. 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
  18. 18. SUM UP • • • • • • • • PERIPHERAL Gait instability Numbness of both UL and LL Hypotonia Areflexia Flexor Planter No Sphincteric Right facial LMNL Left Abducent • • • • • • 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 ?
  19. 19. 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 )
  20. 20. 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
  21. 21. (5) (6) (7) (6) (5) (7)
  22. 22. • • • • NB : 1- Negative rombergism 2- Increased Intracranial Pressure 3- Numbness of both UL and LL
  23. 23. Sensory 1a afferent new technique for electrodiagnosis 1. 2. 3. 4. Submaximal stimulation Low Gain Voluntary movement Average analysis
  24. 24. • Follow up assessment after 4 days
  25. 25. What causes Lost H reflex Bilateral with normal Peripheral nerve conduction ?
  26. 26. 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)
  27. 27. 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
  28. 28. 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
  29. 29. • Sensory Ia afferent affection explains the Hypotonia , Areflexia , lost H Reflex , the Gait instability and Limb incoordination Lost H Reflex in Miller fisher Syndrome
  30. 30. • Sensory Ia afferent affection explains the Hypotonia , Areflexia , lost H Reflex , the Gait instability and Limb incoordination • A Reflexia in Miller fisher syndrome
  31. 31. • Sensory Ia afferent affection explains the Hypotonia , Areflexia , lost H Reflex , the Gait instability and Limb incoordination • Ataxia in Miller fisher syndrome
  32. 32. • ?? Effect of steroid therapy on the Results of electrodiagnosis
  33. 33. Pathophysioly of MFS • Hypothesis of molecular mimicry
  34. 34. • 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).
  35. 35. GQ1b Antibody
  36. 36. Bickerstaff brain stem encephalitis ( BBSE) • • • • • • • • • 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
  37. 37. 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
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