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SATURDAY FORUM SATURDAY FORUM 66thth AUGUEST 2005 AUGUEST 2005
A .S HENNAYAKEA .S HENNAYAKE
PC PC Facial Numbness 1 dayFacial Numbness 1 dayNumbness of both UL & LL Numbness of both UL & LL HPCHPCHe He waswas Well 10 days back and Developed fever Well 10 days back and Developed fever
which was associated with chills and subsided 5 which was associated with chills and subsided 5 days back .He was free of fever for Next 5 days days back .He was free of fever for Next 5 days except for a Non productive coughexcept for a Non productive cough
Then he Developed numbness of both LL & UL . Then he Developed numbness of both LL & UL . And developed weakness of both LL & ascended And developed weakness of both LL & ascended both UL , Meanwhile he noticed BL facial both UL , Meanwhile he noticed BL facial numbness & weakness.numbness & weakness.
He had Immunization for poliomyelitis ,No Hx of He had Immunization for poliomyelitis ,No Hx of food poisoning . Bowel habits & Urinary habits food poisoning . Bowel habits & Urinary habits normal normal
PMH-No Hx of similar illness , DM or PMH-No Hx of similar illness , DM or Connective tissue diseaseConnective tissue disease
FH-No Hx of similar illness , autoimmune FH-No Hx of similar illness , autoimmune disease , , connective tissue disease,DM disease , , connective tissue disease,DM -Father-Father
DH - NADDH - NAD
SH – School boy, living with Mother & Father SH – School boy, living with Mother & Father .no social problems..no social problems.
SummerySummery
14 year old school boy presented with 14 year old school boy presented with Numbness & Weakness of Both UL & LL Numbness & Weakness of Both UL & LL & Facial Weakness following a febrile & Facial Weakness following a febrile Illness. Bowel habits & Urinary habits Illness. Bowel habits & Urinary habits Normal .Father is having DM.Normal .Father is having DM.
FH of DM ,Immunization for poliomyelitis FH of DM ,Immunization for poliomyelitis donedone
OE – ObeseOE – Obese
AfebrileAfebrile
CVSCVS
PR - 80 minPR - 80 min
BP – 130 /70 mmhgBP – 130 /70 mmhg
Ht S 1 ,S2 NormalHt S 1 ,S2 Normal
RSRS
RR -35 min RR -35 min
Chest movements symmetricalChest movements symmetrical
Breath holding counting 30 Breath holding counting 30
VB presentVB present
MotorMotor
Power BL UL Grade 4Power BL UL Grade 4
BL LL Grade 2BL LL Grade 2
Tone BL UL reducedTone BL UL reduced
BL LL reducedBL LL reduced
Reflexes BL UL ReducedReflexes BL UL Reduced
BL LL absentBL LL absent
Plantar BL down Plantar BL down
Joint position sense absentJoint position sense absent
Gait- difficult to check Gait- difficult to check
ABD- Soft ABD- Soft Liver ,spleen not palpableLiver ,spleen not palpable Kidneys not BalatableKidneys not Balatable NO FF ,BS+NO FF ,BS+CNS-Conscious ,RationalCNS-Conscious ,Rational BL facial weakness +BL facial weakness + No Neck stiffnessNo Neck stiffness Fundi –NormalFundi –Normal No cerebellar signsNo cerebellar signs No Sensory Loss.No Sensory Loss.
IxIx
Hb 10 . 3 gdlHb 10 . 3 gdl Pcv 31 %Pcv 31 % WBC 11 x10 9WBC 11 x10 9
N 75 %N 75 %
L 21 %L 21 %
E 2%E 2%
M 2%SSSM 2%SSS
SummerySummery14 year old school boy presented with 14 year old school boy presented with
Numbness & Weakness of Both UL & LL Numbness & Weakness of Both UL & LL & Facial Weakness following a febrile & Facial Weakness following a febrile Illness. Bowel habits & Urinary habits Illness. Bowel habits & Urinary habits Normal .Father is having DM.Normal .Father is having DM.
FH of DM ,Immunization for poliomyelitis FH of DM ,Immunization for poliomyelitis donedone
OE –Obese, BL facial weakness and flaccid OE –Obese, BL facial weakness and flaccid paralysis of both Upper & Lower limbs paralysis of both Upper & Lower limbs with absent reflexes and joint position with absent reflexes and joint position sensessenses
ESR 10 mmhESR 10 mmh
UFR- NADUFR- NAD
BU- 4.2 mmolBU- 4.2 mmol
SE –Na 144meqlSE –Na 144meql
K 4.5 meqlK 4.5 meql
S. Protein -6 gdlS. Protein -6 gdl
A -3.9gdlA -3.9gdl
G -2.1gdlG -2.1gdl
CXR - NADCXR - NAD
ECG -NAD ECG -NAD
RBS-100mgdlRBS-100mgdl
CSF CSF
Sugar 77.7gdlSugar 77.7gdl
Protein 160 mg dlProtein 160 mg dl
Cells L 5Cells L 5
RBC 3 RBC 3
Nerve conduction -Nerve conduction -
GUILLAIN –BARRE SYNDROMEGUILLAIN –BARRE SYNDROME
Incidence 8.5 cases per millionIncidence 8.5 cases per million 3/10000 per y3/10000 per y Monophasic illness Monophasic illness Demyelinating neuropathy ,Rarely axonal Demyelinating neuropathy ,Rarely axonal Does not recurDoes not recur Probably has auto allergic process to myelin Probably has auto allergic process to myelin
sheathssheaths
Usually follows Upper respiratory tract Usually follows Upper respiratory tract infections ,Entero virus ,EB virus infections ,Entero virus ,EB virus Camphylobactor jejuni ,Mycoplasma ,CMV , Camphylobactor jejuni ,Mycoplasma ,CMV , HIV HIV
Clinical featuresClinical features Ascending paralysis lasting for few days to Ascending paralysis lasting for few days to
4 weeks ( subacute 8 weeks)4 weeks ( subacute 8 weeks)Can present with lumbar or spinal painCan present with lumbar or spinal painMotor paralysis distal , proximal or Motor paralysis distal , proximal or
Respiratory musclesRespiratory musclesSome sensory ,tactile , vibratory sensation Some sensory ,tactile , vibratory sensation
and postural sensibility and postural sensibility
Cranial nerves can affect ( facial)Cranial nerves can affect ( facial)
Bulbor involvement can occur Bulbor involvement can occur
Can present as complete Locked in stateCan present as complete Locked in state
Autonomic symptoms like atonic Bladder Autonomic symptoms like atonic Bladder ,illeus Hypertension , orthostatic ,illeus Hypertension , orthostatic HypotensionHypotension
Papilloedema ( CMV)Papilloedema ( CMV)
Combination of external opthalmoplagia Combination of external opthalmoplagia ,ataxia & tendon areflexia ( Miller Fisher ,ataxia & tendon areflexia ( Miller Fisher syndrome )syndrome )
IXIX
Histology –Demeyelination maximal spinal Histology –Demeyelination maximal spinal roots , peripheral nerves roots , peripheral nerves
CSF-Protein elevated , no pleocytosisCSF-Protein elevated , no pleocytosis
Electrophysiological changes ( marked slowing Electrophysiological changes ( marked slowing of conduction velocities ,delayed later of conduction velocities ,delayed later responses ,prolong distal latencies ,dispersion responses ,prolong distal latencies ,dispersion of evoked responses and evidence of of evoked responses and evidence of conduction block)conduction block)
Diagnosis Diagnosis
Clinical groundsClinical grounds
Confirmed by nerve conduction studies and CSFConfirmed by nerve conduction studies and CSF
Course and managementCourse and management
Paralysis progress rapidlyParalysis progress rapidly
May require ventilatory supportMay require ventilatory support
Subcutaneous Heparin ( venous thrombosis)Subcutaneous Heparin ( venous thrombosis)
High dose gamma globulin (IGA deficiency should High dose gamma globulin (IGA deficiency should be checked )be checked )
Plasmapheresis Plasmapheresis
Corticosteroids no valueCorticosteroids no value
Spontaneous recovery begins in few days Spontaneous recovery begins in few days to 3 weeks from the onset with without to 3 weeks from the onset with without treatmenttreatment
Prolong ventilation may be necessaryProlong ventilation may be necessary
Improve over few months Improve over few months
Improvement may be complete or Improvement may be complete or incompleteincomplete
DDDD Poliomyelitis Poliomyelitis BotulinismBotulinism Cord compressionCord compression Primary muscle diseasePrimary muscle disease Transverse MayelitisTransverse Mayelitis