AN INTERESTING CASE OF DIPLOPIA PROF. P. VIJAYARAGHAVAN’S UNIT (M4 UNIT) DR. STALIN
<ul><li>Malliga, a 48 year old female presented with  </li></ul><ul><li>c/o </li></ul><ul><li>Inability to close the Left ...
H/O Present illness <ul><li>Patient  was apparently normal 3 days ago, on waking up in the morning she found her  left eye...
<ul><li>Two days later she was unable to stand from squatting position without support. However gripping of slippers were ...
<ul><li>No symptoms ascribable to higher mental  function abnormality. </li></ul><ul><li>No h/o dizziness on standing. </l...
PAST HISTORY; <ul><li>Known T2DM for past 3 years on OHA and recently started on insulin.  </li></ul><ul><li>She was admit...
PERSONAL HISTORY <ul><li>Mixed diet </li></ul><ul><li>No addictions  </li></ul>
FAMILY HISTORY <ul><li>No family history of neurological complaints. </li></ul><ul><li>No h/o contact with a known tubercu...
GENERAL EXAMINATION <ul><li>Pt conscious , </li></ul><ul><li>oriented to time, place, person </li></ul><ul><li>Afebrile </...
<ul><li>Red eye (left exposure keratitis)  + </li></ul><ul><li>Pupil bilateral 3mm reacting to light  </li></ul><ul><li>Th...
Systemic examination <ul><li>CVS : </li></ul><ul><li>S1,S2 heard  </li></ul><ul><li>no murmurs </li></ul><ul><li>RS  : </l...
CNS EXAMINATION <ul><li>Higher mental function; </li></ul><ul><li>Conscious, </li></ul><ul><li>Oriented to time place pers...
MOTOR SYSTEM RIGHT LEFT UPPER LIMB LOWER LIMB UPPER LIMB LOWER LIMB BULK NORMAL NORMAL NORMAL NORMAL TONE NORMAL NORMAL NO...
CRANIAL NERVES; <ul><li>1 - Normal </li></ul><ul><li>2 - COLOUR, FIELD OF VISION - NORMAL    PUPIL 3mm REACTING  TO LIGHT ...
Right abducent palsy Lool left Look straight  Look right
Bells phenomenon
Sensory system CORTICAL SENSATION - NORMAL ROMBERG'S – UNSTEADINESS BOTH EYES OPEN & CLOSED RIGHT LEFT UL LL UL LL PAIN NO...
<ul><li>CEREBELLAR SIGNS </li></ul><ul><ul><ul><ul><li>FINGER NOSE TEST – NORMAL  </li></ul></ul></ul></ul><ul><ul><ul><ul...
<ul><li>PROBLEMS: </li></ul><ul><ul><li>T2 Diabetes mellitus </li></ul></ul><ul><ul><li>Multi nodular goitre </li></ul></u...
Possible structures involved <ul><li>Lower cranial nerves – LMN type (nuclear/infranuclear) (bilateral and asymmetric) </l...
Diagnosis with differentials: <ul><li>Bilateral, symmetrical proximal > distal, flail weakness of lower limbs with intact ...
Normal  symptom Mitochondrial myopathy MS / ADEM Botulism  Viral encephalitis Tick paralysis diphtheria Weakness  Hemipare...
INVESTIGATIONS <ul><li>CBC: </li></ul><ul><li>Hb -12.2gm </li></ul><ul><li>TC - 6200 </li></ul><ul><li>DC – P-50% L-48% E-...
<ul><li>RFT: </li></ul><ul><li>Blood  Sugar  - 169 mg </li></ul><ul><li>Blood  Urea  -  15mgs </li></ul><ul><li>Serum Crea...
USG NECK & THYRAID; <ul><li>Isthmus 3cm </li></ul><ul><li>RT lobe 3.2*1.3cm </li></ul><ul><li>2 nodes are normal 1*0.3cm <...
<ul><li>Thyroid function test – normal  </li></ul><ul><li>CSF ANALYSIS </li></ul><ul><ul><li>Protein – 48mg/dl </li></ul><...
<ul><li>Sr. calcium – 9.0 mg/dl </li></ul><ul><li>Sr. phosphate – 3.6mg/dl </li></ul><ul><li>Sr. magnesium – 1.8 mg/dl </l...
 
 
 
 
MRI report <ul><li>Right optic nerve meningeoma </li></ul><ul><li>Spine screening - normal </li></ul>
NEUROLOGY OPINION : <ul><li>Clinically,  </li></ul><ul><li>RT lateral rectus palsy </li></ul><ul><li>Left adductor nystagm...
ENT OPINION: <ul><li>B/L tympanic membrane intact </li></ul><ul><li>Nose mid line </li></ul><ul><li>Throat normal </li></u...
OPHTHALMOLOGIST OPINION <ul><li>O/E alternating convergent squint +, Hirschberg test  15* </li></ul>RIGHT LEFT EYE LIDS NO...
<ul><li>FUNDUS: B/L MEDIA CLEAR </li></ul><ul><ul><li>DISK & VESSELS NORMAL </li></ul></ul><ul><ul><li>RT EYE – MACULA </l...
NCS RIGHT PERONEAL NERVE RIGHT MEDIAN NERVE Conduction velocity 34.08m/s >51 Prox CMAP Amplitude 1.0mV N>4.4 Conduction ve...
NCS report  <ul><li>UL- median and  ulnar CMAP latency amplitude  and velocity F wave latency  within limits  </li></ul><u...
Course in the hospital  <ul><li>The reflexes diminished and became absent on the 4 rd  day of admission prompting the diag...
Facial weakness improves
Patient now able to look right
NEUROLOGIST REVIEW <ul><li>CLINICALLY,  </li></ul><ul><li>CONCIOUS ,ORIENTED, </li></ul><ul><li>PRESENTING WITH DIPLOPIA <...
 
FINAL DIAGNOSIS <ul><li>GBS – LOWER CRANIAL NERVE VARIANT </li></ul><ul><ul><li>   BICKERSTAFF   BRAINSTEM ENCEPHALITIS <...
Questions  <ul><li>Can unilateral abducent palsy be a feature of MFS / BBE? </li></ul><ul><li>Why was the patient diagnose...
CRITERIA FOR DIAGNOSING BBE <ul><li>Progressive external ophthalmoplegia  and  ataxia  of <4 weeks duration. </li></ul><ul...
Miller Fisher Syndrome <ul><li>Epidemiology: </li></ul><ul><ul><li>Onset: Mean 40 years; Range 13 to 78 years </li></ul></...
<ul><li>Clinical </li></ul><ul><ul><li>Onset </li></ul></ul><ul><ul><ul><li>Diplopia (Asymmetric) (80%) </li></ul></ul></u...
<ul><ul><li>Ataxia (100%): Dysmetria; Gait ataxia; Arms & Legs </li></ul></ul><ul><ul><li>Areflexia (100%): By 1 week of d...
GBS – The cranial nerve variants <ul><li>MFS-Cranial nerve variants: Often associated with IgG vs GQ1b or GT1b ganglioside...
<ul><li>Laboratory </li></ul><ul><ul><li>CSF </li></ul></ul><ul><ul><ul><li>Protein: 20 to 60 mg/dl </li></ul></ul></ul><u...
<ul><ul><li>Serum antibodies </li></ul></ul><ul><ul><ul><li>IgG vs GQ1b  (80%) </li></ul></ul></ul><ul><ul><ul><li>IgG sta...
Bickerstaff brainstem encephalitis <ul><li>Epidemiology: Most reports from Japan </li></ul><ul><li>Antecedent illness (92%...
<ul><ul><li>Weakness: Flaccid tetraparesis (60%); Respiratory failure </li></ul></ul><ul><ul><li>Pyramidal signs </li></ul...
Clinical profile of patients with BBE
Antibody profile in BBE
Diagnostic Criteria for Guillain-Barrý Syndrome (Ashbury et al) <ul><li>  Features required for diagnosis </li></ul><ul><u...
REFRENCES <ul><li>Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guill...
<ul><li>A case of Guillain-Barré syndrome with bulbar palsy showing the elevations of the anti-GD1a and GT1b antibodies. <...
<ul><li>Unilateral Abducens Nerve Palsy as an Early Feature of Multiple Mononeuropathy Associated with Anti-GQ1b Antibody ...
Follow up <ul><li>At 45 days the patient came for follow up </li></ul><ul><ul><li>There was no demonstrable weakness </li>...
Picture of the patient Ophthalmoparesis    complete/partial Symmetrical proximal weakness Facial weakness Ataxia
 
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A Case of GBS - Lower Cranial Nerve Variant

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A Case of GBS - Lower Cranial Nerve Variant

  1. 1. AN INTERESTING CASE OF DIPLOPIA PROF. P. VIJAYARAGHAVAN’S UNIT (M4 UNIT) DR. STALIN
  2. 2. <ul><li>Malliga, a 48 year old female presented with </li></ul><ul><li>c/o </li></ul><ul><li>Inability to close the Left eye – 3 days </li></ul><ul><li>Redness of Left eye - 3 days </li></ul><ul><li> Double vision - 3 days </li></ul><ul><li>Difficulty in swallowing - 3 days </li></ul>
  3. 3. H/O Present illness <ul><li>Patient was apparently normal 3 days ago, on waking up in the morning she found her left eye was red on looking into the mirror. While attempting to close she was unable to close the Left eye . </li></ul><ul><li>She had double vision , which was maximum on looking to the right. On closing one eye double vision disappeared . Relatives noted inward ( nasal) deviation of right eye ball. </li></ul><ul><li>She was also having difficulty in swallowing both for solid and liquids , however the symptoms were mild and despite it she was able to eat normally at the time of admission. She had mild slurring of speech </li></ul>
  4. 4. <ul><li>Two days later she was unable to stand from squatting position without support. However gripping of slippers were normal and there was no stiffness of limbs. </li></ul><ul><li>She also has un-steadiness while walking. </li></ul><ul><li>She was able to feel the warmth of tap water and able to feel her clothes. </li></ul><ul><li>No difficulty in using her upper extremities. </li></ul><ul><li>No h/o bladder and bowel disturbances. </li></ul><ul><li>No h/o breathing difficulty. </li></ul>
  5. 5. <ul><li>No symptoms ascribable to higher mental function abnormality. </li></ul><ul><li>No h/o dizziness on standing. </li></ul><ul><li>h/o cough with expectoration. </li></ul><ul><li>No h/o fever prior to the episode. </li></ul><ul><li>No h/o diarrhea. </li></ul><ul><li>No h/o drugs, injections. </li></ul>
  6. 6. PAST HISTORY; <ul><li>Known T2DM for past 3 years on OHA and recently started on insulin. </li></ul><ul><li>She was admitted for increased blood sugar two weeks ago and was started on insulin at a private hospital. </li></ul><ul><li>Not a known SHT/ COPD/PT </li></ul><ul><li>No h/o similar complaints in the past </li></ul>
  7. 7. PERSONAL HISTORY <ul><li>Mixed diet </li></ul><ul><li>No addictions </li></ul>
  8. 8. FAMILY HISTORY <ul><li>No family history of neurological complaints. </li></ul><ul><li>No h/o contact with a known tuberculosis patient. </li></ul>
  9. 9. GENERAL EXAMINATION <ul><li>Pt conscious , </li></ul><ul><li>oriented to time, place, person </li></ul><ul><li>Afebrile </li></ul><ul><li>No pallor </li></ul><ul><li>Not icteric </li></ul><ul><li>No cyanosis </li></ul><ul><li>No clubbing </li></ul><ul><li>No pedal edema </li></ul><ul><li>No lymphadenopathy </li></ul>
  10. 10. <ul><li>Red eye (left exposure keratitis) + </li></ul><ul><li>Pupil bilateral 3mm reacting to light </li></ul><ul><li>Thyroid swelling + </li></ul><ul><li>No neuro-cutaneous markers </li></ul><ul><li>VITALS </li></ul><ul><li>PR-84/min, Regular rhythm </li></ul><ul><li>BP 120/90 mm Hg </li></ul><ul><li>RR -16/min </li></ul><ul><li>JVP- normal </li></ul>
  11. 11. Systemic examination <ul><li>CVS : </li></ul><ul><li>S1,S2 heard </li></ul><ul><li>no murmurs </li></ul><ul><li>RS : </li></ul><ul><li>NVBS </li></ul><ul><li>no added sounds </li></ul><ul><li>P/A : Soft </li></ul><ul><li>no organomegaly </li></ul><ul><li>No free fluid </li></ul>
  12. 12. CNS EXAMINATION <ul><li>Higher mental function; </li></ul><ul><li>Conscious, </li></ul><ul><li>Oriented to time place person </li></ul><ul><li>Memory recent and remote- normal </li></ul><ul><li>Speech- mild dysarthria </li></ul>
  13. 13. MOTOR SYSTEM RIGHT LEFT UPPER LIMB LOWER LIMB UPPER LIMB LOWER LIMB BULK NORMAL NORMAL NORMAL NORMAL TONE NORMAL NORMAL NORMAL NORMAL POWER 5/5 4/5 (PROX>DIST) 5/5 4/5 (PROX>DIST) DEEP TENDON REFLEXES + ++ + ++ PLANTAR FLEXOR FLEXOR
  14. 14. CRANIAL NERVES; <ul><li>1 - Normal </li></ul><ul><li>2 - COLOUR, FIELD OF VISION - NORMAL PUPIL 3mm REACTING TO LIGHT </li></ul><ul><li>3,4,6- RT Abduction impaired  </li></ul><ul><li>RT lateral rectus palsy, </li></ul><ul><li> Dolls eye reflex  Rt Abduction impaired </li></ul><ul><li>5- Normal </li></ul><ul><li>7- Left LMN type facial muscle weakness </li></ul><ul><li>8- Normal </li></ul><ul><li>9,10 - Gag reflex – B/L absent </li></ul><ul><li>11- B/L shoulder shrugging, head turning minimally weak </li></ul><ul><li>12- Normal </li></ul>
  15. 15. Right abducent palsy Lool left Look straight Look right
  16. 16. Bells phenomenon
  17. 17. Sensory system CORTICAL SENSATION - NORMAL ROMBERG'S – UNSTEADINESS BOTH EYES OPEN & CLOSED RIGHT LEFT UL LL UL LL PAIN NORMAL NORMAL TEMPERATURE NORMAL NORMAL TOUCH NORMAL NORMAL VIBRATION NORMAL NORMAL POSITION NORMAL NORMAL
  18. 18. <ul><li>CEREBELLAR SIGNS </li></ul><ul><ul><ul><ul><li>FINGER NOSE TEST – NORMAL </li></ul></ul></ul></ul><ul><ul><ul><ul><li>HEEL SHIN KNEE TEST – NORMAL </li></ul></ul></ul></ul><ul><ul><ul><ul><li>NO DYSDIADOCHOKINESIA, </li></ul></ul></ul></ul><ul><ul><ul><ul><li>GAIT – ATAXIA+ (TANDEM WALKING - IMPAIRED) </li></ul></ul></ul></ul><ul><li>SPINE AND CRANIUM - NORMAL </li></ul><ul><li>MENINGIAL SIGNS - NIL </li></ul><ul><li>B/L CAROTID ARTERY - NORMAL </li></ul>
  19. 19. <ul><li>PROBLEMS: </li></ul><ul><ul><li>T2 Diabetes mellitus </li></ul></ul><ul><ul><li>Multi nodular goitre </li></ul></ul><ul><ul><li>LMN type lower cranial nerve involvement (right 6 th , left 7 th bilateral 9,10,11) </li></ul></ul><ul><ul><li>Gait ataxia </li></ul></ul><ul><ul><li>Proximal weakness of lower limbs </li></ul></ul>
  20. 20. Possible structures involved <ul><li>Lower cranial nerves – LMN type (nuclear/infranuclear) (bilateral and asymmetric) </li></ul><ul><li>Cerebellum & its connections (mainly midline) </li></ul><ul><li>Muscle problem or a mixed UMN, LMN lesion. </li></ul>
  21. 21. Diagnosis with differentials: <ul><li>Bilateral, symmetrical proximal > distal, flail weakness of lower limbs with intact reflexes, with gait ataxia, LMN type lower cranial nerve involvement without bladder or autonomic involvement of acute onset (over days). </li></ul><ul><li>Mitochondrial myopathies </li></ul><ul><li>CNS demyelination – ADEM/multiple sclerosis </li></ul><ul><li>Basal meningitis </li></ul><ul><li>Botulism </li></ul><ul><li>Tick paralysis </li></ul><ul><li>Diphtheria </li></ul><ul><li>Atypical viral encephalitis </li></ul>
  22. 22. Normal symptom Mitochondrial myopathy MS / ADEM Botulism Viral encephalitis Tick paralysis diphtheria Weakness Hemiparesis/ recurrent prox flaccid quadriparesis Pyramidal hemi/quadriparesis Flaccid quadriparesis, descending Focal, pyramidal Flaccid quadriparesis, ascending Flacid quadriparesis, descending Cranial nerves Only ocular Any cranial nerve Pupils, any cranial nerve Any cranial nerve Ocular, bulbar Any cranial nerve Ataxia +/- +/- - -/+ - - NCS Mild +/- + Characteristic finding not present in our case <ul><li>Life span↓ </li></ul><ul><li>Recurrence </li></ul><ul><li>Seizure </li></ul><ul><li>Psychosis </li></ul><ul><li>Cognitive dysfunction </li></ul><ul><li>Recurrence </li></ul><ul><li>MRI lesions </li></ul><ul><li>Pupillary paralysis </li></ul><ul><li>Respiratory paralysis </li></ul><ul><li>Prodrome </li></ul><ul><li>ARAS, Respiratory center likely to be affected </li></ul><ul><li>Presence of tick </li></ul><ul><li>Neurological manifestation in proportion to pharyngeal </li></ul>
  23. 23. INVESTIGATIONS <ul><li>CBC: </li></ul><ul><li>Hb -12.2gm </li></ul><ul><li>TC - 6200 </li></ul><ul><li>DC – P-50% L-48% E-2% </li></ul><ul><li>ESR – 5/12mm </li></ul><ul><li>PCV – 34 </li></ul><ul><li>MCV- 86 </li></ul><ul><li>PLATELETS – 1.2 Lakhs </li></ul>
  24. 24. <ul><li>RFT: </li></ul><ul><li>Blood Sugar - 169 mg </li></ul><ul><li>Blood Urea - 15mgs </li></ul><ul><li>Serum Creatinine - 0.7mgs </li></ul><ul><li>ELECTROLYTES: </li></ul><ul><li>Sodium- 134 </li></ul><ul><li>Potassium – 4.2 </li></ul><ul><li>Chloride – 97 </li></ul><ul><li>Bicarbonate - 21 </li></ul><ul><li>ECG : </li></ul><ul><li>SR/NORTH WEST AXIS/S1S2S3 SYNDROME </li></ul><ul><li>X-ray chest P/A view – normal </li></ul><ul><li>7-6-11 FBS – 114mg </li></ul><ul><li>8-6-11 FBS – 129mg </li></ul>
  25. 25. USG NECK & THYRAID; <ul><li>Isthmus 3cm </li></ul><ul><li>RT lobe 3.2*1.3cm </li></ul><ul><li>2 nodes are normal 1*0.3cm </li></ul><ul><li>Another node 1.8*2 cm in RT Lobe </li></ul><ul><li>Nodules are heteroehoic </li></ul><ul><li>Left lobe 4.1*2.1cm </li></ul><ul><li>3.3*1.8 cm heteroehoic lesion with multiple lesion seen in left lobe </li></ul><ul><li>Great vessels normal </li></ul><ul><li>b/l submandibular gland & parotid gland normal </li></ul><ul><li>No significant lymphadenopathy </li></ul><ul><li>IMPRESSION; </li></ul><ul><li>MULTINODULAR GOITER </li></ul><ul><li>HPE TO R/O malignancy </li></ul><ul><li>FNAC THYROID; </li></ul><ul><li>NODULAR COLLAID GOITER WITH CYSTIC DEGENARATION, NO E/O MALIGNANCY </li></ul>
  26. 26. <ul><li>Thyroid function test – normal </li></ul><ul><li>CSF ANALYSIS </li></ul><ul><ul><li>Protein – 48mg/dl </li></ul></ul><ul><ul><li>Sugar – 112mg/dl </li></ul></ul><ul><ul><li>Acellular </li></ul></ul><ul><li>Urine myoglobin – negative </li></ul><ul><li>Urine Bence Jones protein - negative </li></ul><ul><li>Serum CPK – 126 IU, MB fragment -12 IU </li></ul>
  27. 27. <ul><li>Sr. calcium – 9.0 mg/dl </li></ul><ul><li>Sr. phosphate – 3.6mg/dl </li></ul><ul><li>Sr. magnesium – 1.8 mg/dl </li></ul><ul><li>ANA – negative </li></ul><ul><li>HIV – negative </li></ul><ul><li>Mx – negative </li></ul><ul><li>Sputum c/s – no growth </li></ul><ul><li>Blood c/s – no growth </li></ul>
  28. 32. MRI report <ul><li>Right optic nerve meningeoma </li></ul><ul><li>Spine screening - normal </li></ul>
  29. 33. NEUROLOGY OPINION : <ul><li>Clinically, </li></ul><ul><li>RT lateral rectus palsy </li></ul><ul><li>Left adductor nystagmus </li></ul><ul><li>Left facial weakness </li></ul><ul><li>Gag reflex diminished bilaterally </li></ul><ul><li>Proximal > distal weakness </li></ul><ul><li>DTR +++ </li></ul><ul><li>Plantar – flexor </li></ul><ul><li>MRI – normal study </li></ul><ul><li>IMPREESSION: </li></ul><ul><li>? Demyelinating illness </li></ul><ul><li>?GBS variant </li></ul><ul><li>?miller fisher syndrome </li></ul>
  30. 34. ENT OPINION: <ul><li>B/L tympanic membrane intact </li></ul><ul><li>Nose mid line </li></ul><ul><li>Throat normal </li></ul><ul><li>B/L mild conductive hearing loss </li></ul>
  31. 35. OPHTHALMOLOGIST OPINION <ul><li>O/E alternating convergent squint +, Hirschberg test 15* </li></ul>RIGHT LEFT EYE LIDS NORMAL NORMAL EYE LASHES NORMAL NORMAL CONJUCTIVA NORMAL NORMAL CORNEA CLEAR , SENSATION INTACT CLEAR, SENSATION INTACT IRIS COLOUR, PATTERN -NORMAL COLOUR, PATTERN -NORMAL PUPIL PUPIL 3mm, REACTING TO LIGHT PUPIL 3mm, REACTING TO LIGHT ANT CHAMBER NORMAL DEPTH NORMAL DEPTH LENS MINIMAL CHANGES MINIMAL CHANGES RETINOSCOPY +1.5/+1 +.5/+.5 EOM ABDUCTION  RESTRICTED DEXTRO ELEVATION, DEXTRO DEPRESSION  RESTRICTED LEVO ELEVATION, DEPRESSION  NORMAL EOM – FULL
  32. 36. <ul><li>FUNDUS: B/L MEDIA CLEAR </li></ul><ul><ul><li>DISK & VESSELS NORMAL </li></ul></ul><ul><ul><li>RT EYE – MACULA </li></ul></ul><ul><ul><li>LT EYE – MACULA  PIGMENT EPITHELIAL DETACHMENT+ </li></ul></ul><ul><li>COLOUR VISION BE – NORMAL </li></ul><ul><li>FIELD OF VISION BE – NORMAL </li></ul><ul><li>FORCED DUCTEL TEST RE  NEGATIVE </li></ul><ul><li>IMPRESSION: </li></ul><ul><li>MULTIPLE CRANIAL NERVE PALSY WITH LEFT EYE NYSTAGMUS </li></ul><ul><li>LEFT EYE MACULO DEGENERATIVE DISEASE </li></ul>
  33. 37. NCS RIGHT PERONEAL NERVE RIGHT MEDIAN NERVE Conduction velocity 34.08m/s >51 Prox CMAP Amplitude 1.0mV N>4.4 Conduction velocity 54.92m/s >48 Prox CMAP Amplitude 8.7mV N>4.4
  34. 38. NCS report <ul><li>UL- median and ulnar CMAP latency amplitude and velocity F wave latency within limits </li></ul><ul><li>On proximal stimulation Segmental conduction block seen in Left median Nerve. </li></ul><ul><li>Median and ulnar SNAP’s within normal limits </li></ul><ul><li>  </li></ul><ul><li>LL- both peroneal and left tibial velocity reduced, </li></ul><ul><li>Right peroneal amplitude reduced </li></ul><ul><li>On proximal stimulation Segmental conduction blocks seen in both tibial Nerves. </li></ul><ul><li>Both peroneal and tibial F waves absent. </li></ul><ul><li>Right sural SNAP latency prolonged, Left sural SNAP absent </li></ul><ul><li>Imp: suggestive of demyelinative radiculo neuropathy ( LL more affected than UL) </li></ul>
  35. 39. Course in the hospital <ul><li>The reflexes diminished and became absent on the 4 rd day of admission prompting the diagnosis of MFS following which plasma exchange was started </li></ul><ul><li>The facial weakness completely subsided in 1 week. </li></ul><ul><li>At the end of one week patient had only mild symmetric proximal weakness with ataxia, areflexia and right abducent palsy. </li></ul><ul><li>However the patient started developing paresthesias in both lower limbs in the 2 nd week. But there was no objective sensory loss. </li></ul>
  36. 40. Facial weakness improves
  37. 41. Patient now able to look right
  38. 42. NEUROLOGIST REVIEW <ul><li>CLINICALLY, </li></ul><ul><li>CONCIOUS ,ORIENTED, </li></ul><ul><li>PRESENTING WITH DIPLOPIA </li></ul><ul><li>MRI BRAIN - NO VENTRICULO MEGALY, </li></ul><ul><li> NO SOL </li></ul><ul><li> RT OPTIC MENINGIOMA </li></ul><ul><li>IMPRESSION : </li></ul><ul><li>PT IMPROVING </li></ul><ul><li>ATXIA , RT LR PALSY </li></ul><ul><li> CONTINUE PLASMA EXCHANGE </li></ul>
  39. 44. FINAL DIAGNOSIS <ul><li>GBS – LOWER CRANIAL NERVE VARIANT </li></ul><ul><ul><li> BICKERSTAFF BRAINSTEM ENCEPHALITIS </li></ul></ul><ul><ul><li> MILLER FISHER/BICKERSTAFF OVERLAP </li></ul></ul>
  40. 45. Questions <ul><li>Can unilateral abducent palsy be a feature of MFS / BBE? </li></ul><ul><li>Why was the patient diagnosed as BBE instead of MFS? </li></ul><ul><li>How do you explain the paresthesia? </li></ul><ul><li>Why was nerve biopsy/ EMG not done? </li></ul>
  41. 46. CRITERIA FOR DIAGNOSING BBE <ul><li>Progressive external ophthalmoplegia and ataxia of <4 weeks duration. </li></ul><ul><li>Disturbance of consciousness OR hyperreflexia </li></ul><ul><li>Exclude other condition affecting the brainstem and produce similar findings eg </li></ul><ul><ul><li>Brainstem stroke, tumor, lymphoma, ADEM, Multiple sclerosis, botulism, pituitary apoplexy, neuro behcets, vasculitis. </li></ul></ul>
  42. 47. Miller Fisher Syndrome <ul><li>Epidemiology: </li></ul><ul><ul><li>Onset: Mean 40 years; Range 13 to 78 years </li></ul></ul><ul><ul><li>Seasonal: Higher frequency in Spring (March to May) </li></ul></ul><ul><ul><li>Clinical prodrome: Respiratory most common </li></ul></ul><ul><ul><li>Frequency: 25% of GBS in Japan; 1% of GBS in US </li></ul></ul><ul><ul><li>Associated infections </li></ul></ul><ul><ul><ul><li>Campylobacter jejuni: Often serotype O-2 or O-10 </li></ul></ul></ul><ul><ul><ul><li>Hemophilus influenzae: 7% of MFS patients with positive serology </li></ul></ul></ul>
  43. 48. <ul><li>Clinical </li></ul><ul><ul><li>Onset </li></ul></ul><ul><ul><ul><li>Diplopia (Asymmetric) (80%) </li></ul></ul></ul><ul><ul><ul><li>Myalgia & Paresthesias </li></ul></ul></ul><ul><ul><ul><li>Vertigo & Ataxia </li></ul></ul></ul><ul><ul><li>Eye </li></ul></ul><ul><ul><ul><li>External ophthalmoplegia (100%): Symmetric or Asymmetric </li></ul></ul></ul><ul><ul><ul><li>Pupillary dysfunction (42%): Mydriasis </li></ul></ul></ul><ul><ul><ul><li>Ptosis (58%) </li></ul></ul></ul>
  44. 49. <ul><ul><li>Ataxia (100%): Dysmetria; Gait ataxia; Arms & Legs </li></ul></ul><ul><ul><li>Areflexia (100%): By 1 week of disease </li></ul></ul><ul><ul><li>Sensory </li></ul></ul><ul><ul><ul><li>Distal & Facial paresthesias & dysesthesias (24%) </li></ul></ul></ul><ul><ul><ul><li>Sensory loss: Minimal; Definite in 20% </li></ul></ul></ul><ul><ul><li>Weakness: 20% </li></ul></ul><ul><ul><li>Autonomic: Bladder disorders 16% </li></ul></ul><ul><ul><li>Other Cranial nerve disorders </li></ul></ul><ul><ul><ul><li>Oropharyngeal weakness (26%) </li></ul></ul></ul><ul><ul><ul><li>Facial weakness (32%) </li></ul></ul></ul>
  45. 50. GBS – The cranial nerve variants <ul><li>MFS-Cranial nerve variants: Often associated with IgG vs GQ1b or GT1b gangliosides </li></ul><ul><ul><li>GBS overlap: Ophthalmoplegia; Weakness; ± Ataxia </li></ul></ul><ul><ul><li>Internal ophthalmoplegia: Dilated pupils; Light-near dissociation </li></ul></ul><ul><ul><li>Acute external ophthalmoplegia: Complete or partial </li></ul></ul><ul><ul><li>Acute ataxia: May progress to Weakness & GBS </li></ul></ul><ul><ul><li>Visual impairment </li></ul></ul><ul><ul><li>Acute neuropathies with bulbar dysfunction: Pharyngo-cervical-brachial variants </li></ul></ul><ul><ul><li>Bickerstaff brainstem encephalitis: Brainstem signs </li></ul></ul>
  46. 51. <ul><li>Laboratory </li></ul><ul><ul><li>CSF </li></ul></ul><ul><ul><ul><li>Protein: 20 to 60 mg/dl </li></ul></ul></ul><ul><ul><ul><li>Cells: Few or None; 0 to 5/mm 3 </li></ul></ul></ul><ul><ul><li>Nerve conduction studies </li></ul></ul><ul><ul><ul><li>Sensory </li></ul></ul></ul><ul><ul><ul><ul><li>Axonal loss </li></ul></ul></ul></ul><ul><ul><ul><ul><li>SNAPs: Reduced amplitude </li></ul></ul></ul></ul><ul><ul><ul><li>Motor </li></ul></ul></ul><ul><ul><ul><ul><li>Peripheral nerve: Normal CMAPs </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Facial: Reduced CMAP amplitude </li></ul></ul></ul></ul><ul><ul><ul><li>F-waves : Prolonged; Dispersed; Absent </li></ul></ul></ul><ul><ul><ul><li>H reflexes: Absent from soleus </li></ul></ul></ul>
  47. 52. <ul><ul><li>Serum antibodies </li></ul></ul><ul><ul><ul><li>IgG vs GQ1b  (80%) </li></ul></ul></ul><ul><ul><ul><li>IgG staining of cerebellar molecular layer </li></ul></ul></ul><ul><ul><li>MRI </li></ul></ul><ul><ul><ul><li>Cranial nerve enhancement (gadolinium) may occur </li></ul></ul></ul><ul><ul><ul><li>Brainstem or Cerebellar lesions: Some patients </li></ul></ul></ul><ul><li>Treatment: </li></ul><ul><ul><li>IV IG or plasmapheresis </li></ul></ul>
  48. 53. Bickerstaff brainstem encephalitis <ul><li>Epidemiology: Most reports from Japan </li></ul><ul><li>Antecedent illness (92%): Most commonly upper respiratory infection </li></ul><ul><li>Age: 3 to 91 years </li></ul><ul><li>Onset: Diplopia or gait disorder most common </li></ul><ul><li>Clinical: Brainstem signs </li></ul><ul><ul><li>Reduced consciousness (74%): Drowsy, stupor or coma </li></ul></ul><ul><ul><li>Ataxia: Often trunk & limb </li></ul></ul><ul><ul><li>Eyes </li></ul></ul><ul><ul><ul><li>Ophthalmoplegia, external (100%): Relatively symmetric </li></ul></ul></ul><ul><ul><ul><li>Pupil disorders (34%) </li></ul></ul></ul><ul><ul><ul><li>Ptosis (29%) </li></ul></ul></ul><ul><ul><ul><li>Nystagmus (27%) </li></ul></ul></ul><ul><ul><li>Other cranial nerves </li></ul></ul><ul><ul><ul><li>Facial diplegia (45%) </li></ul></ul></ul><ul><ul><ul><li>Bulbar weakness (34%) </li></ul></ul></ul>
  49. 54. <ul><ul><li>Weakness: Flaccid tetraparesis (60%); Respiratory failure </li></ul></ul><ul><ul><li>Pyramidal signs </li></ul></ul><ul><ul><ul><li>Tendon reflexes: Variable; Hyperreflexia to Absent </li></ul></ul></ul><ul><ul><ul><li>Plantar responses (40%): Extensor </li></ul></ul></ul><ul><ul><li>Sensory loss </li></ul></ul><ul><ul><ul><li>Small fiber (31%) </li></ul></ul></ul><ul><ul><ul><li>Large fiber (16%) </li></ul></ul></ul><ul><ul><ul><li>Hemisensory loss </li></ul></ul></ul><ul><ul><li>Course </li></ul></ul><ul><ul><ul><li>Often good prognosis: Complete remission in 51%; Death 4% </li></ul></ul></ul><ul><li>Laboratory </li></ul><ul><ul><li>Serum IgG binding to GQ1b ganglioside (66%) </li></ul></ul><ul><li>Electrophysiology </li></ul><ul><ul><li>Motor axon degeneration </li></ul></ul>
  50. 55. Clinical profile of patients with BBE
  51. 56. Antibody profile in BBE
  52. 57. Diagnostic Criteria for Guillain-Barrý Syndrome (Ashbury et al) <ul><li>  Features required for diagnosis </li></ul><ul><ul><li>Progressive weakness of both legs and arms </li></ul></ul><ul><ul><li>Areflexia </li></ul></ul><ul><li>Clinical features supportive of diagnosis </li></ul><ul><ul><li>Progression over days to 4 wk </li></ul></ul><ul><ul><li>Relative symmetry or signs </li></ul></ul><ul><ul><li>Mild sensory symptoms or signs </li></ul></ul><ul><ul><li>Cranial nerve involvement (bifacial palsies) </li></ul></ul><ul><ul><li>Recovery beginning 2-4 wk after progression ceases </li></ul></ul><ul><ul><li>Autonomic dysfunction </li></ul></ul><ul><ul><li>Absence of fever at onset </li></ul></ul><ul><li>Laboratory features supportive of diagnosis </li></ul><ul><ul><li>Elevated cerebrospinal fluid protein with < 10 cells/ μ L </li></ul></ul><ul><ul><li>Electrodiagnostic features of nerve conduction slowing or block </li></ul></ul>
  53. 58. REFRENCES <ul><li>Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barre syndrome. (encephalitis) </li></ul><ul><ul><li>Brain. 2003 Oct;126(Pt 10):2279-90. Epub 2003 Jul 07 </li></ul></ul><ul><ul><li>Odaka M, Yuki N, Yamada M, Koga M, Takemi T, Hirata K, Kuwabara S. </li></ul></ul><ul><ul><li>Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi  880, Mibu, Shimotsuga, Tochigi 321-0293, Japan. </li></ul></ul>
  54. 59. <ul><li>A case of Guillain-Barré syndrome with bulbar palsy showing the elevations of the anti-GD1a and GT1b antibodies. </li></ul><ul><ul><li>Rinsho Shinkeigaku  2001 Apr-May;41(4-5):202-5 </li></ul></ul><ul><ul><li>[Article in Japanese] </li></ul></ul><ul><ul><li>Ito S ,  Hirose Y ,  Mokuno K ,  Kusunoki S . </li></ul></ul><ul><ul><li>Source </li></ul></ul><ul><ul><li>Department of Neurology, Toyohashi Municipal Hospital. </li></ul></ul>
  55. 60. <ul><li>Unilateral Abducens Nerve Palsy as an Early Feature of Multiple Mononeuropathy Associated with Anti-GQ1b Antibody </li></ul><ul><ul><li>Ryuta Kinno, *  Hiroo Ichikawa, Hiroto Tanigawa, Kazuhiro Itaya, and Mitsuru Kawamura </li></ul></ul><ul><ul><li>Department of Neurology, Showa University School of Medicine, Tokyo, Japan </li></ul></ul>
  56. 61. Follow up <ul><li>At 45 days the patient came for follow up </li></ul><ul><ul><li>There was no demonstrable weakness </li></ul></ul><ul><ul><li>Cranial nerves were normal </li></ul></ul><ul><ul><li>Reflex were just elicitable </li></ul></ul><ul><ul><li>Paresthesia however persisted </li></ul></ul><ul><ul><li>Repeat nerve conduction planned 2 wks later </li></ul></ul>
  57. 62. Picture of the patient Ophthalmoparesis  complete/partial Symmetrical proximal weakness Facial weakness Ataxia
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