Chronic inflammatory
Demyelinating Polyneuropathy
Guillain barre syndrome
Learning objectives
• ETIOLOGY
• STAGES
• PRESENTATION
• MANAGEMENT
• CIDP is a neurological disorder involving progressive weakness and
reduced senses in arms and legs
• Tingling or no feeling in fingers or toes
• Weakness of arms and legs
• Loss of deep tendon reflexes
• Fatigue
• Most common cause of AFP
• Acquired, acute inflammatory (post infective or idiopathic)
polyneuropathy
• Symmetrical flaccid motor weakness, minimal sensory involvement,
autonomic dysfunction
• Rare in infants
• Male to female ratio 1.5 : 1
Pathophysiology
• IMMUNE MEDIATED DISORDER
• DEMYELINATING OR DEGENERATIVE AXONAL INJURY
• MULTIFOCAL AREAS OF INFLAMMATION OR DEMYELINATION WITH
CELLULAR INFILTRATES OF MACROPHAGES & LYMPHOCYTES
• RESULTS IN CONDUCTION BLOCK
• INFLAMMATION INVOLVES PERIPHERAL N ,CRANIAL N, DORSAL ROOT,
GANGLIA, SYMPATHETIC CHAIN
ETIOLOGY
• ANTIGEN ANTIBODY REACTION AGAINST PERIPHERAL NS
• FOLLOWS RESP , GIT INFECTION
• MYCOPLASMA
• VIRUSES :HEP B, CMV, EBV, MUMPS, MEASELS, INFLUNZA
• CAMPYLOBACTER JEJUNI MOST COMMON
STAGES OF GBS
• PROGRESSIVE PHASE : , 6 WKS
• PLATEAU PHASE
• RECOVERY PHASE
• SYMMETRICAL WEAKNESS OF LOWER LIMBS
• ASCENDING SPREAD TO UPPER LIMBS AND CRANIAL NS
• HYPOTONIA, HYPOREFLEXIA LOWER LIMBS
• PLANTERS NORMAL
STAGES OF GBS
• PROGRESSIVE PHASE : , 6 WKS
• PLATEAU PHASE
• RECOVERY PHASE
• SYMMETRICAL WEAKNESS OF LOWER LIMBS
• ASCENDING SPREAD TO UPPER LIMBS AND CRANIAL NS
• HYPOTONIA, HYPOREFLEXIA LOWER LIMBS
• PLANTERS NORMAL
DIAGNOSIS
• CLINICAL FEATURES
• NCS : CONDUCTION BLOCK, DEMYELINATION, DEGENERATION OF
MOTOR OR SENSORY AXONS
• EMG : RED IN RECRUITMENT , ACTIVE DENERVATION
• CSF : ELEVATED PROTEINS , CELL COUNT NORMAL
MANAGEMENT
• Gen Supportive care
• Good medical and nursing care
• ICU admission
• Nutritional support
• Care of bowel and bladder
• Prevention of bedsores and DVT
• Tx of sec bacterial infections
• CIDP- No cure. IVIGs, Multivitamins, Physiotherapy
Management
• IMMUNOGLOBULINS (IVIGs) 400 mg/kg 5 days
• STEROIDS : PREDNISONE 2 mg/kg – 2 wks
• PLASMAPHERESIS
PROGNOSIS
• MOST COMMON CAUSE OF DEATH : AUTONOMIC INSTABILITY
• RESP FAILURE
• RECOVERY : 1-6 m
SUDDEN INFANT DEATH
SYNDROME
• SIDS – sudden death of an infant under 1 year of age
• Also called Crib death
• Usually associated with sleep
• Mostly occurs during first 6 months
• Sudden, unpredictable
• Usually healthy baby before death
• Cannot be explained by review of history, death scene investigation,
autopsy
Risk Factors for SIDS
• Placing the baby on his side or stomach to sleep
• Prematurity
• Low birth weight
• Overheating during sleep
• Sleeping on a soft surface
• Family history of SIDS or FTT
• Smoking during pregnancy
• Mother under 20 yrs of age , received no prenatal care
Triple Risk Model
• Infant has underlying abnormality (brainstem) making him unable to
respond to low O2 or high CO2 blood levels
• Exposure to triggering event e.g sleeping face down on tummy
• Vulnerable stage in infant’s development i.e first 6 months
PREVENTION
• Putting the baby on his back while he sleeps
• Placing baby on tummy while awake
• Sleeping on firm mattress or surface
• Keep babies warm, not too warm
• Baby’s crib in your room for first 6 months
• Avoid bed sharing
• Smoke free environment
• If baby sick, show doctor right away
• Breastfeed the baby
POST POLIO SYNDROME
POST POLIO SYNDROME
• Poliomyelitis acute viral infection
• Asymptomatic in 90-95 % cases
• Mild illness to rapidly progressive illness – encephalitis, paralysis,
death
• Transmission by orofecal route, perinatal
• Paralytic polio –asymmetric paralysis
• Spinal, bulbar, bulbospinal, encephalitic forms
• Diagnosis mainly clinical, CSF , Viral culture of stool and throat swabs
• 30-40% of patients with paralytic polio suffer Post polio syndrome
• 30-40 yrs later
• Muscle pain, exacerbation of existing weakness, development of new
weakness
• Infected with wild type virus
• Risk factors- inc length of time since acute infection, presence of
permanent residual impairment after recovery from acute illness and
female sex.
Management
• Energy Conservation. Resting frequently, assistive devices
• Physical Therapy. Muscle strengthening exercises, swimming, aerobics
• Speech Therapy. Compensate for swallowing difficulties, voice
strengthening exercises
• Medications. Pain relievers
• Stay warm
• Avoid falls
• Healthy lifestyle
Prevention: Polio vaccine OPV and IPV

Chronic inflammatory Demyelinating Polyneuropathy.pptx

  • 1.
  • 2.
    Learning objectives • ETIOLOGY •STAGES • PRESENTATION • MANAGEMENT
  • 3.
    • CIDP isa neurological disorder involving progressive weakness and reduced senses in arms and legs • Tingling or no feeling in fingers or toes • Weakness of arms and legs • Loss of deep tendon reflexes • Fatigue
  • 4.
    • Most commoncause of AFP • Acquired, acute inflammatory (post infective or idiopathic) polyneuropathy • Symmetrical flaccid motor weakness, minimal sensory involvement, autonomic dysfunction • Rare in infants • Male to female ratio 1.5 : 1
  • 5.
    Pathophysiology • IMMUNE MEDIATEDDISORDER • DEMYELINATING OR DEGENERATIVE AXONAL INJURY • MULTIFOCAL AREAS OF INFLAMMATION OR DEMYELINATION WITH CELLULAR INFILTRATES OF MACROPHAGES & LYMPHOCYTES • RESULTS IN CONDUCTION BLOCK • INFLAMMATION INVOLVES PERIPHERAL N ,CRANIAL N, DORSAL ROOT, GANGLIA, SYMPATHETIC CHAIN
  • 6.
    ETIOLOGY • ANTIGEN ANTIBODYREACTION AGAINST PERIPHERAL NS • FOLLOWS RESP , GIT INFECTION • MYCOPLASMA • VIRUSES :HEP B, CMV, EBV, MUMPS, MEASELS, INFLUNZA • CAMPYLOBACTER JEJUNI MOST COMMON
  • 7.
    STAGES OF GBS •PROGRESSIVE PHASE : , 6 WKS • PLATEAU PHASE • RECOVERY PHASE • SYMMETRICAL WEAKNESS OF LOWER LIMBS • ASCENDING SPREAD TO UPPER LIMBS AND CRANIAL NS • HYPOTONIA, HYPOREFLEXIA LOWER LIMBS • PLANTERS NORMAL
  • 8.
    STAGES OF GBS •PROGRESSIVE PHASE : , 6 WKS • PLATEAU PHASE • RECOVERY PHASE • SYMMETRICAL WEAKNESS OF LOWER LIMBS • ASCENDING SPREAD TO UPPER LIMBS AND CRANIAL NS • HYPOTONIA, HYPOREFLEXIA LOWER LIMBS • PLANTERS NORMAL
  • 9.
    DIAGNOSIS • CLINICAL FEATURES •NCS : CONDUCTION BLOCK, DEMYELINATION, DEGENERATION OF MOTOR OR SENSORY AXONS • EMG : RED IN RECRUITMENT , ACTIVE DENERVATION • CSF : ELEVATED PROTEINS , CELL COUNT NORMAL
  • 10.
    MANAGEMENT • Gen Supportivecare • Good medical and nursing care • ICU admission • Nutritional support • Care of bowel and bladder • Prevention of bedsores and DVT • Tx of sec bacterial infections • CIDP- No cure. IVIGs, Multivitamins, Physiotherapy
  • 11.
    Management • IMMUNOGLOBULINS (IVIGs)400 mg/kg 5 days • STEROIDS : PREDNISONE 2 mg/kg – 2 wks • PLASMAPHERESIS PROGNOSIS • MOST COMMON CAUSE OF DEATH : AUTONOMIC INSTABILITY • RESP FAILURE • RECOVERY : 1-6 m
  • 12.
  • 13.
    • SIDS –sudden death of an infant under 1 year of age • Also called Crib death • Usually associated with sleep • Mostly occurs during first 6 months • Sudden, unpredictable • Usually healthy baby before death • Cannot be explained by review of history, death scene investigation, autopsy
  • 14.
    Risk Factors forSIDS • Placing the baby on his side or stomach to sleep • Prematurity • Low birth weight • Overheating during sleep • Sleeping on a soft surface • Family history of SIDS or FTT • Smoking during pregnancy • Mother under 20 yrs of age , received no prenatal care
  • 15.
    Triple Risk Model •Infant has underlying abnormality (brainstem) making him unable to respond to low O2 or high CO2 blood levels • Exposure to triggering event e.g sleeping face down on tummy • Vulnerable stage in infant’s development i.e first 6 months
  • 16.
    PREVENTION • Putting thebaby on his back while he sleeps • Placing baby on tummy while awake • Sleeping on firm mattress or surface • Keep babies warm, not too warm • Baby’s crib in your room for first 6 months • Avoid bed sharing • Smoke free environment • If baby sick, show doctor right away • Breastfeed the baby
  • 17.
  • 18.
    POST POLIO SYNDROME •Poliomyelitis acute viral infection • Asymptomatic in 90-95 % cases • Mild illness to rapidly progressive illness – encephalitis, paralysis, death • Transmission by orofecal route, perinatal • Paralytic polio –asymmetric paralysis • Spinal, bulbar, bulbospinal, encephalitic forms • Diagnosis mainly clinical, CSF , Viral culture of stool and throat swabs
  • 19.
    • 30-40% ofpatients with paralytic polio suffer Post polio syndrome • 30-40 yrs later • Muscle pain, exacerbation of existing weakness, development of new weakness • Infected with wild type virus • Risk factors- inc length of time since acute infection, presence of permanent residual impairment after recovery from acute illness and female sex.
  • 20.
    Management • Energy Conservation.Resting frequently, assistive devices • Physical Therapy. Muscle strengthening exercises, swimming, aerobics • Speech Therapy. Compensate for swallowing difficulties, voice strengthening exercises • Medications. Pain relievers • Stay warm • Avoid falls • Healthy lifestyle Prevention: Polio vaccine OPV and IPV