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Dr. Tareq Esteak
Resident, Neurology
National Institute of Neurosciences and Hospital
 A 50-year-old male presented with weakness of all four limbs
 Name : Masud Rana
 Age : 50 years
 Marital status : married
 Occupation : retired bank accountant
 Address : Sadar, Noakhali
 Weakness of all four limbs – 2 years
Insidious onset
Gradually progressive
Involving both proximal and distal group of muscles
Initially the weakness was mild
For last 2 month he cant walk without walking aid
Associated with tingling and numbness of hand and feet
No history of trauma to the head or neck
No history of bowel-bladder abnormality
No nasal regurgitation, swallowing difficulty, change in voice,
muscle twitching
No history of joint pain, rash, high colored urine, oral ulcer or
photosensitivity
No relevant drug history
2008
Rt sided painful dimness of
vison
left sided dimness of vision
within a week
Treated with IV
methylprednisolone
Full recovery of vision
After 2 month again similar
attack ; treated same way
This time partial recovery
2013
Left sided weakness
Acute onset;
Rapidly progressive
Admitted & Treated
conservatively
Full recovery within 7-8
months
2018
Loss of balance
Tendency to fall towards right
more than the left
acute onset,
gradually progressive
Tremor on hand movement
(rt>lt)
Treated with IV
methylprednisolone
Without any significant
improvement
2019
Weakness,Tingling,
numbness of hands and feet
Insiduous onset,
Slowly progessive
Treated with tricyclic
antidepressant ,pregabalin
Without any significant
improvement
Still progressing
 Hypertensive for 7 years
 On amlodipine OD, since then
 Compliant with medication and advice
 Blood pressure well controlled
 No significant past history
 None of his family member suffering from similar illness.
 Parents died due to age related illness, his other family members are in good
health
 Retired bank accountant
 Had to left the job due to disease progression
 Middle class
 Normal dietary habits
 Smoker-10 pack year( 10 sticks/day for last 20 years)
 Tobacco chewing
 H/o unprotected extramarital sexual exposure
40 years old gentleman
Weakness of all four limbs for last couple of years.
 Insidious onset and gradually progressive, involving both proximal
and distal group of muscles. Associated with tingling and numbness
of hand and feet
Since last couple of months he cant walk without walking aid.
Had a bilateral, painful dimness of vision twice, in 2008.
Had a left sided weakness of body in 2013 and has been suffering
from a tendency to fall toward right since 2018 .
 Multiple sclerosis with peripheral neuropathy
 Vasculitis
 Neurosyphilis
 Vit B12 deficiency
 Neurosarcoidosis
 Conscious, alert
 Anxious looking
 Intravenous cannula in situ
 Pulse:78/min, regular
 BP:130/90mmHg,no postural drop
 RR:18/minute
 Temp:99 F
 Lymph Node: Not palpable
 Higher Psychic Function : Intact
 Speech: scanning of speech
 Cranial nerves including Fundoscopy :
o Visual acuity : Bilaterally reduced finger counting
o Visual field : intact
o Color vision : Intact
o Fundoscopy : Bilateral primary optic atrophy
Upper limb Lower limb
Bulk normal normal normal normal
Tone reduced reduced reduced reduced
Power 4/5 4/5 3/5 3/5
Reflexes absent absent absent absent
Hoffman negative negative - -
Plantar - - Extensor Extensor
 All modalities of sensory functions were intact.
 Romberg’s sign : couldn’t be evaluated due to postural instability
 Cerebellar signs were present bilaterally(rt>lt)
 Finger nose test : positive
 Dysdiadokokinesia : Positive
 Hill-shin test : Positive
 Stance : wide based
 Gate : Ataxic, can’t perform the tandem gate
 No sign of meningism
 No nerve thickening
 No abnormality found
40 years old male
Weakness of all four limbs for last couple of years.
 Insidious onset and gradually progressive, involving both
proximal and distal group of muscles. Associated with tingling
and numbness of hand and feet
Since last two month he cant walk without walking aid.
Had a bilateral, painful dimness of vision twice, in 2008.
A hemiparesis in 2013 and has been suffering from a tendency to
fall toward right since 2018 .
Scanning of speech , reduced visual acuity in both eyes and
bilateral primary optic atrophy. His power was 4/5 in the upper
limb and 3/5 in lower limb. All of his deep tendon reflexes are
with an intact sensory function but plantar were bilaterally
extensor.
 Multiple sclerosis with peripheral neuropathy
 Vasculitis
 Neurosyphilis
 CBC:
o Hb :12.1 g/dl
o ESR :15 in 1st hour
o TC :14× 103
/Cu.mm
o DC : neutrophil:75%; Lymohocyte:23%
o PCV : 38%
o Platelet : 290 × 103
/Cu.mm
►HbA1c : 6.4%
►Electrolytes : Na:133 ; K:4.1 ;Cl:99
►S. Creatinine : 0.76 mg/dl
►CRP : 5.68 (reference value: <5 mg/l)
►ANA : 0.47 (reference value: <1 )
►cANCA : 1.06 (reference value: <5 U/ml)
►pANCA : 0.33 (reference value: <5 U/ml)
►S. Calcium : 2.20 mmol/L
►S. ACE : 9.0 IU/L (12-68 IU)
►S.VDRL : non-reactive
►S.TPHA : non-reactive
►S. Vitamin B12 : 1012 pg/ml (reference value: 187-883 pg/ml)
►Anti-HIV antibody : negative
►TSH : 4.44 u IU/mL
►FT4 : 1.06 ng/dl
Routine
 Glucose : 3.7 mmol/L
 Protein : 60 mg/dl
 TC : 10/cumm
 DC : 100% lymphocyte
Special test
 Oligoclonal band : Absent
 IgG index : 0.72 (<0.70)
 CSF VDRL : Reactive
 CSF TPHA : Non-reactive
 T1 & T1C+
 T1 & T1C+
 T2WI
MRI 2021 MRI 2019
MRI 2021 MRI 2019
Impression :Demyelinating Motor
Polyradiculoneuropathy
 Can we explain the both CNS and PNS feature with an unifying diagnosis?
 How do we explain the isolated VRDL positivity in CSF?
 What should be the management approach in this patient?
Peeling, R., Mabey, D., Kamb, M. et al. Syphilis. Nat Rev Dis Primers 3, 17073 (2017).
MULTIPLE SCLEROSIS IN CONJUNCTION
WITH PERIPHERAL NEUROPATHY
 Rarely MS have a coexisting polyneuropathy or mononeuropathy multiplex,
 Several autopsy cases have shown demyelinating lesions in both the central white matter and
scattered in peripheral nerves.
 The infrequency of the combination, however, suggests a purely coincidental occurrence,
perhaps with another underlying disease as an explanation (e.g., Lyme disease, AIDS).
 Another view, expressed by Thomas and colleagues and by Mendell et al, is that an
autoimmune demyelination has been incited in both spinal cord and peripheral nerve, the latter
taking the form of a chronic inflammatory polyradiculoneuropathy.
 Of course, myelination is provided by oligodendrocytes in the CNS and schwan cells in the
peripheral nervous system, implying that there are differences in antigens that typically separate
MS and CIDP.
 More commonly in MS, radicular and neuropathic symptoms are the result of involvement of
myelinated fibers in the root entry zone of the cord or fibers exiting from the ventral white
matter.
Primary progressive multiple sclerosis can be diagnosed in patients
with:
1 year of disability progression and 2 of the following:
>1 T2-hyperintense lesions* characteristic of multiple sclerosis
in one or
more of the following brain regions
• Two or more T2-hyperintense lesions* in the spinal cord
• Presence of CSF-specific oligoclonal bands

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Clinical presentation on multiple sclerosis

  • 1. Dr. Tareq Esteak Resident, Neurology National Institute of Neurosciences and Hospital
  • 2.  A 50-year-old male presented with weakness of all four limbs
  • 3.  Name : Masud Rana  Age : 50 years  Marital status : married  Occupation : retired bank accountant  Address : Sadar, Noakhali
  • 4.  Weakness of all four limbs – 2 years
  • 5.
  • 6. Insidious onset Gradually progressive Involving both proximal and distal group of muscles Initially the weakness was mild For last 2 month he cant walk without walking aid Associated with tingling and numbness of hand and feet
  • 7. No history of trauma to the head or neck No history of bowel-bladder abnormality No nasal regurgitation, swallowing difficulty, change in voice, muscle twitching No history of joint pain, rash, high colored urine, oral ulcer or photosensitivity No relevant drug history
  • 8.
  • 9. 2008 Rt sided painful dimness of vison left sided dimness of vision within a week Treated with IV methylprednisolone Full recovery of vision After 2 month again similar attack ; treated same way This time partial recovery 2013 Left sided weakness Acute onset; Rapidly progressive Admitted & Treated conservatively Full recovery within 7-8 months 2018 Loss of balance Tendency to fall towards right more than the left acute onset, gradually progressive Tremor on hand movement (rt>lt) Treated with IV methylprednisolone Without any significant improvement 2019 Weakness,Tingling, numbness of hands and feet Insiduous onset, Slowly progessive Treated with tricyclic antidepressant ,pregabalin Without any significant improvement Still progressing
  • 10.  Hypertensive for 7 years  On amlodipine OD, since then  Compliant with medication and advice  Blood pressure well controlled
  • 11.  No significant past history
  • 12.  None of his family member suffering from similar illness.  Parents died due to age related illness, his other family members are in good health
  • 13.  Retired bank accountant  Had to left the job due to disease progression  Middle class  Normal dietary habits  Smoker-10 pack year( 10 sticks/day for last 20 years)  Tobacco chewing  H/o unprotected extramarital sexual exposure
  • 14. 40 years old gentleman Weakness of all four limbs for last couple of years.  Insidious onset and gradually progressive, involving both proximal and distal group of muscles. Associated with tingling and numbness of hand and feet Since last couple of months he cant walk without walking aid. Had a bilateral, painful dimness of vision twice, in 2008. Had a left sided weakness of body in 2013 and has been suffering from a tendency to fall toward right since 2018 .
  • 15.
  • 16.  Multiple sclerosis with peripheral neuropathy  Vasculitis  Neurosyphilis  Vit B12 deficiency  Neurosarcoidosis
  • 17.
  • 18.  Conscious, alert  Anxious looking  Intravenous cannula in situ  Pulse:78/min, regular  BP:130/90mmHg,no postural drop  RR:18/minute  Temp:99 F  Lymph Node: Not palpable
  • 19.  Higher Psychic Function : Intact  Speech: scanning of speech  Cranial nerves including Fundoscopy : o Visual acuity : Bilaterally reduced finger counting o Visual field : intact o Color vision : Intact o Fundoscopy : Bilateral primary optic atrophy
  • 20. Upper limb Lower limb Bulk normal normal normal normal Tone reduced reduced reduced reduced Power 4/5 4/5 3/5 3/5 Reflexes absent absent absent absent Hoffman negative negative - - Plantar - - Extensor Extensor
  • 21.  All modalities of sensory functions were intact.  Romberg’s sign : couldn’t be evaluated due to postural instability
  • 22.  Cerebellar signs were present bilaterally(rt>lt)  Finger nose test : positive  Dysdiadokokinesia : Positive  Hill-shin test : Positive  Stance : wide based  Gate : Ataxic, can’t perform the tandem gate
  • 23.  No sign of meningism  No nerve thickening
  • 25. 40 years old male Weakness of all four limbs for last couple of years.  Insidious onset and gradually progressive, involving both proximal and distal group of muscles. Associated with tingling and numbness of hand and feet Since last two month he cant walk without walking aid. Had a bilateral, painful dimness of vision twice, in 2008. A hemiparesis in 2013 and has been suffering from a tendency to fall toward right since 2018 . Scanning of speech , reduced visual acuity in both eyes and bilateral primary optic atrophy. His power was 4/5 in the upper limb and 3/5 in lower limb. All of his deep tendon reflexes are with an intact sensory function but plantar were bilaterally extensor.
  • 26.  Multiple sclerosis with peripheral neuropathy  Vasculitis  Neurosyphilis
  • 27.  CBC: o Hb :12.1 g/dl o ESR :15 in 1st hour o TC :14× 103 /Cu.mm o DC : neutrophil:75%; Lymohocyte:23% o PCV : 38% o Platelet : 290 × 103 /Cu.mm ►HbA1c : 6.4% ►Electrolytes : Na:133 ; K:4.1 ;Cl:99 ►S. Creatinine : 0.76 mg/dl
  • 28. ►CRP : 5.68 (reference value: <5 mg/l) ►ANA : 0.47 (reference value: <1 ) ►cANCA : 1.06 (reference value: <5 U/ml) ►pANCA : 0.33 (reference value: <5 U/ml) ►S. Calcium : 2.20 mmol/L ►S. ACE : 9.0 IU/L (12-68 IU) ►S.VDRL : non-reactive ►S.TPHA : non-reactive ►S. Vitamin B12 : 1012 pg/ml (reference value: 187-883 pg/ml) ►Anti-HIV antibody : negative ►TSH : 4.44 u IU/mL ►FT4 : 1.06 ng/dl
  • 29. Routine  Glucose : 3.7 mmol/L  Protein : 60 mg/dl  TC : 10/cumm  DC : 100% lymphocyte Special test  Oligoclonal band : Absent  IgG index : 0.72 (<0.70)  CSF VDRL : Reactive  CSF TPHA : Non-reactive
  • 30.
  • 31.  T1 & T1C+
  • 32.
  • 33.
  • 34.  T1 & T1C+
  • 36.
  • 37. MRI 2021 MRI 2019
  • 38. MRI 2021 MRI 2019
  • 39.
  • 40.
  • 42.  Can we explain the both CNS and PNS feature with an unifying diagnosis?  How do we explain the isolated VRDL positivity in CSF?  What should be the management approach in this patient?
  • 43.
  • 44.
  • 45.
  • 46. Peeling, R., Mabey, D., Kamb, M. et al. Syphilis. Nat Rev Dis Primers 3, 17073 (2017).
  • 47.
  • 48. MULTIPLE SCLEROSIS IN CONJUNCTION WITH PERIPHERAL NEUROPATHY  Rarely MS have a coexisting polyneuropathy or mononeuropathy multiplex,  Several autopsy cases have shown demyelinating lesions in both the central white matter and scattered in peripheral nerves.  The infrequency of the combination, however, suggests a purely coincidental occurrence, perhaps with another underlying disease as an explanation (e.g., Lyme disease, AIDS).  Another view, expressed by Thomas and colleagues and by Mendell et al, is that an autoimmune demyelination has been incited in both spinal cord and peripheral nerve, the latter taking the form of a chronic inflammatory polyradiculoneuropathy.  Of course, myelination is provided by oligodendrocytes in the CNS and schwan cells in the peripheral nervous system, implying that there are differences in antigens that typically separate MS and CIDP.  More commonly in MS, radicular and neuropathic symptoms are the result of involvement of myelinated fibers in the root entry zone of the cord or fibers exiting from the ventral white matter.
  • 49.
  • 50. Primary progressive multiple sclerosis can be diagnosed in patients with: 1 year of disability progression and 2 of the following: >1 T2-hyperintense lesions* characteristic of multiple sclerosis in one or more of the following brain regions • Two or more T2-hyperintense lesions* in the spinal cord • Presence of CSF-specific oligoclonal bands