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NEURO-MUSCULAR
DYSFUNCTION IN CHILDREN
PRESENTEDTO SIR EHTISHAM
INTRODUCTION
• (THETERM SPINA BIFIDA MEANS “SPLIT SPINE.”) SPINA BIFIDA IS USUALLY APPARENT AT
BIRTH.
• SPINA BIFIDA IS CAUSED BYTHE INCOMPLETE DEVELOPMENT OFTHE FETUS’ SPINE DURING
SPINE DURINGTHE FIRST MONTH OF PREGNANCY. THE CONDITIONVARIES IN DEGREE, FROM
DEGREE, FROM MILDWITH NO SYMPTOMSTO SEVERE WITH NERVE DAMAGE.
• THE CONDITION IS ATYPE OF NEURALTUBE DEFECT (NTD).
• THIS SOUNDS SCARYAND SOMETYPESOF SPINA BIFIDAAREVERY SERIOUS. BUT IT’S
IMPORTANTTO KNOWTHATTHE CONDITIONVARIESWIDELY IN DEGREE. MOST CASES ARE
SO MILDTHEY HAVE NO SYMPTOMSAND DON’T EVEN NEEDTREATMENT (THIS OCCURS
WITH SPINA BIFIDA OCCULTA, OR “HIDDEN SPINA BIFIDA”).
• HOWEVER, INFANTS BORNWITHA MORE SERIOUSTYPE OFTHIS DISORDER HAVE OPEN
LESIONSONTHEIR SPINEWHERETHERE’S SIGNIFICANT DAMAGETO NERVESANDTHE
SPINALCORD.THE OPENINGCAN BE REPAIREDTHROUGH SURGERY, BUTTHE NERVE
DAMAGE ISN’T RESOLVEDANDTHAT CAUSES PERMANENT DISABILITY. SPINA BIFIDA CAN
OCCURANYWHERE ALONGTHE BACKBONE, BUT IS MOST OFTEN FOUND INTHE SMALL OF
THE BACK OR FURTHER DOWN.
TYPES OF SPINA BIFIDA
THERE ARE THREE MAIN TYPES OF SPINA BIFIDA:
• SPINA BIFIDA OCCULTA.
• MENINGOCELE.
• MYELOMENINGOCELE.
SPINA BIFIDA OCCULTA
• SPINA BIFIDA OCCULTA ISTHE MILDEST AND MOST COMMON FORM OFTHIS DISORDER. IT
USUALLY ONLY INVOLVES A MINIMAL PORTION OFTHE SPINE; IT USUALLY SHOWS NO
SYMPTOMS, AND IT DOES NOT REQUIRETREATMENT.WHEN AN INFANT IS BORNWITH SPINA
BIFIDA OCCULTA,THE SKIN COVERSTHE DEFORMITY OFTHE SPINAL BONE.
• SPINA BIFIDA OCCULTA LITERALLY MEANS "A HIDDEN SPOT ONTHE SPINE,"
• RARELY, SPINA BIFIDA OCCULTA WILL CAUSE PROBLEMS WHEN A CHILD GROWSTO
ADOLESCENCE.
MENINGOCELE
• INTHIS LEAST COMMONTYPE OF SPINA BIFIDA,THE MENINGES (MEMBRANE
SURROUNDINGTHE SPINALCORD) PROTRUDETHROUGHTHE OPENING CAUSINGA LUMP
OR SAC ONTHE BACK.
• MORE SEVERETHAN SPINA BIFIDA OCCULTA
• MENINGOCELECAN BE REPAIREDTHROUGH SURGERYWITH LITTLE OR NO NERVE DAMAGE
RESULTING.
• THE SURGERY IS PERFORMEDAT ANYTIME DURING INFANCY.WITH MENINGOCELES,THE
SPINALCORD HAS DEVELOPED NORMALLYAND IS UNDAMAGED.THECHILD HAS NO
NEUROLOGICAL PROBLEMS.
MYELOMENINGOCELE
• MYELOMENINGOCELE ISTHE MOST SEVERE FORM OF SPINA BIFIDA, OCCURRING NEARLY
ONCE FOR EVERY 1,000 LIVE BIRTHS.
• FOR INFANTS BORNWITH A MYELOMENINGOCELE, THE SPINAL CORD DOES NOT FORM
PROPERLY AND A PORTION OFTHE UNDEVELOPED CORD PROTRUDESTHROUGHTHE BACK. A
SAC CONTAINING CEREBROSPINAL FLUID AND BLOODVESSELS SURROUNDSTHE
PROTRUDING CORD, WHICH IS USUALLY NOT COVERED BY SKIN SOTHATTHE NERVES AND
TISSUES ARE EXPOSED.
CONT.….
• INFANTS BORNWITH MYELOMENINGOCELEOFTEN HAVE PARALYSIS ORWEAKNESS
BELOWTHE LEVEL OFTHE SPINAL LESION.
• THISAFFECTSTHE LOWER LIMBSALONGWITH PROBLEMSWITH BLADDERAND BOWEL
FUNCTION. IN EXTREMECASES,THETRUNK AND UPPER EXTREMITIESARE INVOLVED.
CAUSES
•THE EXACT CAUSE OFTHE OCCURRENCE OF SPINA BIFIDA ISN’T CLEAR. A
COMBINATION OF GENETICS AND ENVIRONMENTAL FACTORS AND FAMILY
HISTORY OR NUTRITIONAL SUCH AS A FAMILY HISTORY OF NEURAL
TUBE DEFECTS AND FOLATE (VITAMIN B-9) DEFICIENCY ISTHOUGHT
TO BETHE MAIN CAUSE
DIAGNOSTIC TEST
SPINA BIFIDA CAN USUALLY BE DETECTED INTHE FETUS, BUT NOT ALWAYS.THESETESTS INCLUDE:
• A BLOODTEST:TAKEN DURINGTHE 16THTO 18TH WEEKOF PREGNANCY,THIS SCREENINGTESTS
SCREENINGTESTSTHE AMOUNT OF AFP (ALPHA-FETOPROTEIN) INTHE BLOOD.THE AMOUNT IS
HIGHER IN ABOUT 75%TO 80% INWOMENWHO CARRY A FETUSWITH SPINA BIFIDA.
• AN ULTRASOUND (SONOGRAM): PROBLEMSWITHTHE FETUS’S SPINE MAY BE SPOTTEDTHROUGH
THROUGH IMAGING.
• AMNIOCENTESIS: FLUID FROMTHEWOMB IS REMOVEDTHROUGH ATUBETOTEST FOR PROTEIN
PROTEIN LEVELS.
TREATMENT
THE TWO MAIN SPINA BIFIDA TREATMENT OPTIONS ARE
FETAL SURGERY DURING PREGNANCY
SURGERY ON THE BABY RIGHT AFTER BIRTH.
NURSING CARE
NURSING CARE PLANNING GOALS FOR CLIENTS WITH SPINA BIFIDA INCLUDE
• PREVENT INFECTION,
• MAINTAIN SKIN INTEGRITY,
• PREVENT TRAUMA RELATED TO DISUSE,
• INCREASE FAMILY COPING SKILLS,
• EDUCATION ABOUT THE CONDITION, AND SUPPORT.
REHABILITATIVE CARE
• REHABILITATIVE TREATMENTS FOR CHILDREN WITH SPINA BIFIDA.
• PHYSICAL, OCCUPATIONAL, AND SPEECH THERAPY ARE OFTEN AN INTEGRAL
PART OF CARE.
• THEY CAN HELP CHILDREN IMPROVE MUSCLE COORDINATION AND BALANCE,
BUILD STRENGTH, AND OVERCOME LANGUAGE DELAYS. OUR
NEUROMUSCULAR EXPERTS ALSO HELP CHILDREN LEARN TO FUNCTION
INDEPENDENTLY, BUILD SOCIAL SKILLS, AND GAIN CONFIDENCE.
MEDICAL AND SURGICAL MANAGEMENT
• INITIAL SURGERY TO REPAIR THE SPINE
• TREATING HYDROCEPHALUS
• PHYSIOTHERAPY
• OCCUPATIONAL THERAPY
• MOBILITY AIDS
• TREATING BONE AND JOINT PROBLEMS
• TREATING BLADDER PROBLEMS
• TREATING BOWEL PROBLEMS
INSTITUTES HELPING CHILD WITH SPINA BIFIDA
•SPINA BIFIDA FOUNDATION PAKISTAN
•SPARC(SOCIETY FOR THE PROTECTION OF THE RIGHTS OF THE
CHILD) ADVOCATES FOR PROTECTION OF CHILD RIGHTS BY
PROVIDING TRAININGS TO CHILD RIGHTS ACTIVISTS
CEREBRAL PALSY
•NEUROLOGICAL DISORDER WHICH MAY OCCUR BEFORE BIRTH,
DURING BIRTH OR AFTER BIRTH.
•ACCORDING TO CDC OUT OF EVERY 1000 CHILDREN CEREBRAL
PALSY AFFECTS 1.5 TO 4 CHILDREN WORLD WIDE.
CEREBRAL PALSY
• CEREBRAL……HAVING TO DO WITH BRAIN
• PALSY……. WEAKNESS OR PROBLEMS WITH BODY MOVEMET.
MEDICAL CONDITION USUALLY CAUSED BY ABNORMAL BRAIN DEVELOPMENT
BEFORE OR AT BIRTH THAT CAUSES THE LOSS OF CONTROL OF THE ARMS AND
LEGS.
CONGENITAL DISORDER OF MOVEMENT, MUSCLE TONE OR POSTURE.
SIGNS AND SYMPTOMS:
•THE BRAIN CAUSING CP DOES NOT CHANGE WITH TIME SO THE
SYMPTOMS USUALLY DON’T WORSEN WITH AGE.
VARIATION IN MUSCLES TONE.
DELAYS IN REACHING MOTOR SKILLS MILESTONES.
ATAXIA
SPASTICITY
DIFFICULTY IN WALKING, WRITING, SPEAKING AND LEARNING.
EXCESSIVE DROOLING AND PROBLEM WITH SWALLOWING.
NEUROLOGICALPROBLEMSASSOCIATEDWITHCP:
DIFFICULTY IN SEEING AND HEARING.
INTELLECTUAL DISABILITIES.
SEIZURES.
MENTAL HEALTH CONDITION.
ORAL DISEASES.
URINARY INCONTINENCE.
CAUSES:
GENE MUTATION
MATERNAL INFECTION.
FETAL STROKE.
INTRACRANIAL HEMORRHAGE.
INFANT INFECTION.
TRAUMATIC HEAD INJURY.
LACK OF O2 (ASPHYXIA).
RISK FACTORS:
1.MATERNAL HEALTH.
2.INFANT HEALTH.
1.MATERNAL HEALTH:
 CYTOMEGALOVIRUS
 GERMAN MEASLES (RUBELA)
 SYPHILLIS.
 TAXOPLASMOSIS.
1.INFANT ILLNESS:
 BACTERIAL MENINGITIS.
 VIRAL ENCEPHALITIS.
 SEVERE OR UNTREATED JAUNDICE;
 BLEEDING INTO THE BRAIN.
TYPES OF CP:
a.SPASTIC CP:
b.DYSKINETIC CP.
c.HYPOTONIC CP.
d.ATAXIC CP.
SPASTIC CP:
AFFECTING APPROXIMATELY 80% OF PEOPLE WITH CP.
MUSCLES WEAKNESS AND PARALYSIS MAY BE PRESENT.
THE SYMPTOMS CAN AFFECT THE ENTIRE BODY OR JUST ONE SIDE OF BODY.
SYMPTOMS:
INVOLUNTARY LIMB MOVEMENT.
CONTINUOUS MUSCLES SPASMS.
ABNORMAL WALKING .
FLEXION AT THE ELBOW, WRISTS,FIGERA ETC.
a.DYSKINETIC CP:
•THIS CAUSE INVOLUNTARY, ABNORMAL MOVEMENT IN THE ARMS
LEGS AND HANDS.
a.HYPOTONIC CP:
•CAUSES DEMINISHED MUSCLES TONE AND OVERLY RELAXED
MUSCLES.
•THE ARMS AND LEGS MOVE VERY EASILYAND APPEAR FLOPPY.
ATAXIC CP:
• CHARACTERIZED BY VOLUNTARY MUSCLES MOVEMENT THAT OFTEN APPEAR
DISORGANIZED,CLUMPSY AND JERKY.
• PEOPLE WITH THIS FORM OF CP USUALLY HAVE PROBLEMS WITH BALANCE AND
CORDINATION.
SYMPTOMS:
• UNSTEADY MOVEMENT DUE TO DIFFICULTY IN BALANCE
• TREMORS
• SLOW EYE MOVEMENT
• DIFFICULTY IN FINGERS MOVEMENT
MIXED CEREBRAL PALSY:
•SOME PEOPLE HAVE A COMBINATION OF SYMPTOMS FROM THE
DIFFERENT TYPES OF CP,THIS IS CALLED MIXED CP.
•IN MOST CASES OF MIXED CP, PEOPLE EXPERIENCE A MIX OF SPASTIC
AND DYSKINETIC CP.
•PREVENTION:
•MAKE SURE YOU ARE VACCINATED (RUBELLA)
•TAKE CARE OF OF YOUR SELF BY TAKING HEALTHY DIET AND
REGULAR MEDICINES.
•REGULAR VISIT TO THE DR DURING PREGNANCY;
•PREVENT HEAD INJURIES.
•AVOID ALCOHOL ,TOBACCO AND ILLEGAL DRUGS DURING
PREGNANCY.
MEDICAL MANAGEMENT:
•MEDICATIONS AND ORTHOPEDIC SURGERIES.
•ASSISTIVE AIDS:
•EYE GLASSES.
•HEARING AIDS.
•WALKING AIDS.
•WHEELCHAIRS ETC.
OTHER TREATMENT:
• SPEECH THERAPY
• PHYSICAL THERAPY
• OCCUPATIONAL THERAPY.
• RECREATIONAL THERAPY
• PSYCHOTHERAPY
• SOCIAL SERVICES CONSULTATION.
NURSING MANAGEMENT:
•PROVIDE ADEQUATE NUTRITION.
•MAINTAIN SKIN INTEGRITY.
•PROMOTE SAFETY.
•PROMOTE GROWTH AND DEVELOPMENT.
•TEACH PARENT SHOW TO CARE FOR CHILD.
•PROVIDE EMOTIONAL SUPPORT..
MUSCULAR DYSTROPHY
INTRODUCTION:
 MUSCULAR DYSTROPHIES (MDS) CONSTITUTE THE LARGEST AND
MOST IMPORTANT SINGLE GROUP OF MUSCLE
DISEASES OF CHILDHOOD.
 THE MDS HAVE A GENETIC ORIGIN IN WHICH THERE IS GRADUAL
DEGENERATION OF
MUSCLE FIBERS, AND THEY ARE CHARACTERIZED BY
PROGRESSIVE WEAKNESS AND WASTING OF SYMMETRIC
GROUPS OF SKELETAL MUSCLES, WITH INCREASING DISABILITY
AND DEFORMITY.
THE MOST COMMON FORM,
I. DUCHENNE MUSCULAR DYSTROPHY (DMD).
II.FACIOSCAPULOHUMERAL (LANDOUZY-DEJERINE) MUSCULAR DYSTROPHY
• LIMB-GIRDLE MUSCULAR DYSTROPHY (LGMD
CLINICAL MANIFESTATION:
• ALTHOUGH GIRLS CAN BE CARRIERS AND MILDLY AFFECTED, IT'S MUCH MORE
COMMON IN BOYS.
• SIGNS AND SYMPTOMS, WHICH TYPICALLY APPEAR IN EARLY CHILDHOOD, MIGHT
INCLUDE:
 FREQUENT FALLS
 DIFFICULTY RISING FROM A LYING OR SITTING POSITION
 TROUBLE RUNNING AND JUMPING
 WADDLING GAIT
 WALKING ON THE TOES
 LARGE CALF MUSCLES
 MUSCLE PAIN AND STIFFNESS
 LEARNING DISABILITIES
 DELAYED GROWTH
CAUSES
 CERTAIN GENES ARE INVOLVED IN MAKING PROTEINS THAT PROTECT MUSCLE
FIBERS. MUSCULAR DYSTROPHY OCCURS WHEN ONE OF THESE GENES IS
DEFECTIVE.
 EACH FORM OF MUSCULAR DYSTROPHY IS CAUSED BY A GENETIC MUTATION
PARTICULAR TO THAT TYPE OF THE DISEASE. MOST OF THESE MUTATIONS ARE
INHERITED.
COMPLICATION
•THE COMPLICATIONS OF PROGRESSIVE MUSCLE WEAKNESS INCLUDE:
 TROUBLE WALKING. SOME PEOPLE WITH MUSCULAR DYSTROPHY
EVENTUALLY NEED TO USE A WHEELCHAIR.
 TROUBLE USING ARMS. DAILYACTIVITIES CAN BECOME MORE
DIFFICULT IF THE MUSCLES OF THE ARMS AND SHOULDERS ARE
AFFECTED.
 SHORTENING OF MUSCLES OR TENDONS AROUND JOINTS
(CONTRACTURES). CONTRACTURES CAN FURTHER LIMIT MOBILITY.
 BREATHING PROBLEMS. PROGRESSIVE WEAKNESS CAN AFFECT THE
MUSCLES ASSOCIATED WITH BREATHING. PEOPLE WITH MUSCULAR
DYSTROPHY MIGHT EVENTUALLY NEED TO USE A BREATHING
ASSISTANCE DEVICE (VENTILATOR), INITIALLYAT NIGHT BUT POSSIBLY
ALSO DURING THE DAY.
CURVED SPINE (SCOLIOSIS). WEAKENED MUSCLES MIGHT
BE UNABLE TO HOLD THE SPINE STRAIGHT.
 HEART PROBLEMS. MUSCULAR DYSTROPHY CAN
REDUCE THE EFFICIENCY OF THE HEART MUSCLE.
 SWALLOWING PROBLEMS. IF THE MUSCLES INVOLVED
WITH SWALLOWING ARE AFFECTED, NUTRITIONAL
PROBLEMS AND ASPIRATION PNEUMONIA CAN DEVELOP.
FEEDING TUBES MIGHT BE AN OPTION.
CHARACTERISTICS OF MUSCULAR DYSTROPHY:
•EARLY ONSET, USUALLY BETWEEN 3 AND 7 YEARS OLD
• PROGRESSIVE MUSCULAR WEAKNESS, WASTING, AND
CONTRACTURES
• CALF MUSCLE PSEUDOHYPERTROPHY IN MOST
PATIENTS
• LOSS OF INDEPENDENT AMBULATION BY 9 TO 12
YEARS OLD
• SLOWLY PROGRESSIVE, GENERALIZED WEAKNESS
DURING TEENAGE YEARS
NURSING CARE
 THE MAJOR EMPHASIS OF NURSING CARE IS TO HELP THE CHILD
AND FAMILY COPE WITH A CHRONIC,
PROGRESSIVE, INCAPACITATING DISEASE;
 THE GOALS OF CARE SHOULD ALSO INVOLVE DECISIONS
REGARDING QUALITY OF LIFE,
ACHIEVEMENT OF INDEPENDENCE, AND TRANSITION TO
ADULTHOOD.
 NURSES CAN ASSIST WITH DECISION MAKING BY EXPLORING ALL
AVAILABLE OPTIONS AND RESOURCES
AND SUPPORT THE CHILD AND FAMILY IN THE DECISION.
 OLDER BOYS WITH MD MAY ALSO NEED PSYCHIATRIC OR
PSYCHOLOGICAL COUNSELING TO DEAL WITH ISSUES SUCH AS
DEPRESSION, ANGER, AND QUALITY OF LIFE.
 NURSES CAN BE ALERT TO THE PROBLEMS AND NEEDS AND MAKE
NECESSARY REFERRALS
WHEN SUPPLEMENTARY SERVICES ARE INDICATED.
REHABILITATION CARE
 MINIMIZING CONTRACTURES BY INTRODUCING REGULAR
STRETCHING INTO YOUR DAILY ROUTINE
 MAINTAINING FUNCTION AND ADAPTING TO ANY LOSS OF FUNCTION
 MONITORING FUNCTION OVER TIME THROUGH STANDARD TESTS AND
MEASURES
 ASSESSING FOR AND MANAGING COMPROMISED SKIN INTEGRITY
 PREVENTING AND MANAGING PAIN
 PRESCRIBING EXERCISE AND SUPERVISING SAFE PHYSICALACTIVITY
(I.E. LOW LOAD, LOW RESISTANCE EXERCISE)
 RECOMMENDING MOBILITY DEVICES, ADAPTIVE SEATING, AND OTHER
EQUIPMENT
 REHABILITATION AFTER INJURY OR FRACTURE
MEDICAL MANAGEMENT
 CORTICOSTEROIDS, SUCH AS PREDNISONE AND DEFLAZACORT (EMFLAZA), WHICH CAN HELP
MUSCLE STRENGTH AND DELAY THE PROGRESSION OF CERTAIN TYPES OF MUSCULAR
DYSTROPHY. BUT PROLONGED USE OF THESE TYPES OF DRUGS CAN CAUSE WEIGHT GAIN AND
WEAKENED BONES, INCREASING FRACTURE RISK.
 HEART MEDICATIONS, SUCH AS ANGIOTENSIN-CONVERTING ENZYME (ACE) INHIBITORS OR BETA
BLOCKERS, IF MUSCULAR DYSTROPHY DAMAGES THE HEART.
 GOLODIRSEN (VYONDYS 53) FOR TREATMENT OF SOME PEOPLE WITH DUCHENNE DYSTROPHY
WHO HAVE A CERTAIN GENETIC MUTATION.
SURGICAL MANAGEMENT
 SURGERY MIGHT BE NEEDED TO CORRECT CONTRACTURES OR A SPINAL
CURVATURE THAT COULD EVENTUALLY MAKE BREATHING MORE DIFFICULT.
HEART FUNCTION MAY BE IMPROVED WITH A PACEMAKER OR OTHER CARDIAC
DEVICE.
OVERVIEWOF INSTITUTE WORKING IN PAKISTAN
I. MUSCULAR DYSTROPHY REGISTRY OF PAKISTAN:
• THE MUSCULAR DYSTROPHY REGISTRY OF PAKISTAN HAS BEEN SETUP WITH A GOAL TO
IMPROVE OUTCOMES AND QUALITY OF LIFE IN PATIENTS WITH MUSCULAR DYSTROPHIES AND
RELATED NEUROMUSCULAR DISORDERS. THE REGISTRY IS CURRENTLY RECRUITING PATIENTS
WITH DUCHENNE/BECKER MUSCULAR DYSTROPHY AND SPINAL MUSCULAR ATROPHY.
I. BAQAI INSTITUTE OF PHYSICAL THERAPY REHABILITATION MEDICINE
•THE DEPARTMENT OF PHYSIOTHERAPY WAS ESTABLISHED IN 2004. THE
DEPARTMENT AND ITS MANAGEMENT ARE ALWAYS COMMITTED TO SERVE
THE RURAL POPULATION SPECIALLY.
PHYSIOTHERAPIST IN THE DEPARTMENT WORK WITH THE
MULTIDISCIPLINARY TEAM OF HEALTH CARE PROVIDERS TO PROVIDE
HOLISTIC CARE THAT IS ALIGNED WITH PATIENT’S TREATMENT GOALS.
THE DEPARTMENT STRIVES TO PROVIDE INPATIENT AND OUTPATIENTS
SERVICES THAT IS BASED ON THE HIGH STANDARDS OF CARE AND QUALITY.
THE DEPARTMENT OFFERS ITS SERVICES TO THE HOSPITAL’S ICUS, HDUS,
AND MULTIDISCIPLINARY WARDS.
THE INPATIENT AND OUTPATIENT PHYSIOTHERAPY SERVICES OFFER
ASSESSMENT FUNCTION, MOBILIZATION AND ABILITY TO FUNCTION
INDEPENDENTLY AND TO RESTORE QUALITY OF LIFE.
GUILLAIN BARRE SYNDROME
Rehabilitation care
Approximately 40% of patients who are
hospitalized with GBS will require admission to
inpatient rehabilitation. For GBS persons
necessitating admission to inpatient rehabilitation,
the requirement of prior ventilator support most
strongly predicts an extended length of stay on
inpatient rehabilitation.
Overview in Pakistan
The Aga Khan University Hospital, Karachi, Pakistan. )
Thirty-four cases of GBS were admitted to the hospital during the study
period, with an age range of 3 to 70 years. The mean age for disease
onset was 35.2 years for female patients, compared to 30 years for
males; the male/female ratio was 1.6:1.Gastrointestinal infections (12/22,
54.6%) were the most common antecedent event, followed by upper
respiratory tract infections (9/22, 40.9%) and skin lesions (1/22, 4.5%).
Most patients developed GBS within one month of the preceding
infection. Cranial nerve abnormalities (30/34, 88.2%), autonomic
dysfunction (21/34, 61.8%) and respiratory failure requiring intubation
(19/34, 55.9%) were also common
Overview in Pakistan
We found that GBS occurred at all ages and was slightly
more common in males. Majority of patients had an
antecedent history of infection and had severe disease on
presentation. The patients were treated with either
plasmapheresis or intravenous immunoglobulins and there
was no significant difference in outcome in the two groups.
Despite severe persistent disability, in-hospital mortality
was low

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Peads presentation.pptx

  • 2.
  • 3.
  • 4. INTRODUCTION • (THETERM SPINA BIFIDA MEANS “SPLIT SPINE.”) SPINA BIFIDA IS USUALLY APPARENT AT BIRTH. • SPINA BIFIDA IS CAUSED BYTHE INCOMPLETE DEVELOPMENT OFTHE FETUS’ SPINE DURING SPINE DURINGTHE FIRST MONTH OF PREGNANCY. THE CONDITIONVARIES IN DEGREE, FROM DEGREE, FROM MILDWITH NO SYMPTOMSTO SEVERE WITH NERVE DAMAGE. • THE CONDITION IS ATYPE OF NEURALTUBE DEFECT (NTD).
  • 5. • THIS SOUNDS SCARYAND SOMETYPESOF SPINA BIFIDAAREVERY SERIOUS. BUT IT’S IMPORTANTTO KNOWTHATTHE CONDITIONVARIESWIDELY IN DEGREE. MOST CASES ARE SO MILDTHEY HAVE NO SYMPTOMSAND DON’T EVEN NEEDTREATMENT (THIS OCCURS WITH SPINA BIFIDA OCCULTA, OR “HIDDEN SPINA BIFIDA”). • HOWEVER, INFANTS BORNWITHA MORE SERIOUSTYPE OFTHIS DISORDER HAVE OPEN LESIONSONTHEIR SPINEWHERETHERE’S SIGNIFICANT DAMAGETO NERVESANDTHE SPINALCORD.THE OPENINGCAN BE REPAIREDTHROUGH SURGERY, BUTTHE NERVE DAMAGE ISN’T RESOLVEDANDTHAT CAUSES PERMANENT DISABILITY. SPINA BIFIDA CAN OCCURANYWHERE ALONGTHE BACKBONE, BUT IS MOST OFTEN FOUND INTHE SMALL OF THE BACK OR FURTHER DOWN.
  • 6. TYPES OF SPINA BIFIDA THERE ARE THREE MAIN TYPES OF SPINA BIFIDA: • SPINA BIFIDA OCCULTA. • MENINGOCELE. • MYELOMENINGOCELE.
  • 7. SPINA BIFIDA OCCULTA • SPINA BIFIDA OCCULTA ISTHE MILDEST AND MOST COMMON FORM OFTHIS DISORDER. IT USUALLY ONLY INVOLVES A MINIMAL PORTION OFTHE SPINE; IT USUALLY SHOWS NO SYMPTOMS, AND IT DOES NOT REQUIRETREATMENT.WHEN AN INFANT IS BORNWITH SPINA BIFIDA OCCULTA,THE SKIN COVERSTHE DEFORMITY OFTHE SPINAL BONE. • SPINA BIFIDA OCCULTA LITERALLY MEANS "A HIDDEN SPOT ONTHE SPINE," • RARELY, SPINA BIFIDA OCCULTA WILL CAUSE PROBLEMS WHEN A CHILD GROWSTO ADOLESCENCE.
  • 8.
  • 9. MENINGOCELE • INTHIS LEAST COMMONTYPE OF SPINA BIFIDA,THE MENINGES (MEMBRANE SURROUNDINGTHE SPINALCORD) PROTRUDETHROUGHTHE OPENING CAUSINGA LUMP OR SAC ONTHE BACK. • MORE SEVERETHAN SPINA BIFIDA OCCULTA • MENINGOCELECAN BE REPAIREDTHROUGH SURGERYWITH LITTLE OR NO NERVE DAMAGE RESULTING. • THE SURGERY IS PERFORMEDAT ANYTIME DURING INFANCY.WITH MENINGOCELES,THE SPINALCORD HAS DEVELOPED NORMALLYAND IS UNDAMAGED.THECHILD HAS NO NEUROLOGICAL PROBLEMS.
  • 10.
  • 11. MYELOMENINGOCELE • MYELOMENINGOCELE ISTHE MOST SEVERE FORM OF SPINA BIFIDA, OCCURRING NEARLY ONCE FOR EVERY 1,000 LIVE BIRTHS. • FOR INFANTS BORNWITH A MYELOMENINGOCELE, THE SPINAL CORD DOES NOT FORM PROPERLY AND A PORTION OFTHE UNDEVELOPED CORD PROTRUDESTHROUGHTHE BACK. A SAC CONTAINING CEREBROSPINAL FLUID AND BLOODVESSELS SURROUNDSTHE PROTRUDING CORD, WHICH IS USUALLY NOT COVERED BY SKIN SOTHATTHE NERVES AND TISSUES ARE EXPOSED.
  • 12.
  • 13. CONT.…. • INFANTS BORNWITH MYELOMENINGOCELEOFTEN HAVE PARALYSIS ORWEAKNESS BELOWTHE LEVEL OFTHE SPINAL LESION. • THISAFFECTSTHE LOWER LIMBSALONGWITH PROBLEMSWITH BLADDERAND BOWEL FUNCTION. IN EXTREMECASES,THETRUNK AND UPPER EXTREMITIESARE INVOLVED.
  • 14. CAUSES •THE EXACT CAUSE OFTHE OCCURRENCE OF SPINA BIFIDA ISN’T CLEAR. A COMBINATION OF GENETICS AND ENVIRONMENTAL FACTORS AND FAMILY HISTORY OR NUTRITIONAL SUCH AS A FAMILY HISTORY OF NEURAL TUBE DEFECTS AND FOLATE (VITAMIN B-9) DEFICIENCY ISTHOUGHT TO BETHE MAIN CAUSE
  • 15. DIAGNOSTIC TEST SPINA BIFIDA CAN USUALLY BE DETECTED INTHE FETUS, BUT NOT ALWAYS.THESETESTS INCLUDE: • A BLOODTEST:TAKEN DURINGTHE 16THTO 18TH WEEKOF PREGNANCY,THIS SCREENINGTESTS SCREENINGTESTSTHE AMOUNT OF AFP (ALPHA-FETOPROTEIN) INTHE BLOOD.THE AMOUNT IS HIGHER IN ABOUT 75%TO 80% INWOMENWHO CARRY A FETUSWITH SPINA BIFIDA. • AN ULTRASOUND (SONOGRAM): PROBLEMSWITHTHE FETUS’S SPINE MAY BE SPOTTEDTHROUGH THROUGH IMAGING. • AMNIOCENTESIS: FLUID FROMTHEWOMB IS REMOVEDTHROUGH ATUBETOTEST FOR PROTEIN PROTEIN LEVELS.
  • 16. TREATMENT THE TWO MAIN SPINA BIFIDA TREATMENT OPTIONS ARE FETAL SURGERY DURING PREGNANCY SURGERY ON THE BABY RIGHT AFTER BIRTH.
  • 17. NURSING CARE NURSING CARE PLANNING GOALS FOR CLIENTS WITH SPINA BIFIDA INCLUDE • PREVENT INFECTION, • MAINTAIN SKIN INTEGRITY, • PREVENT TRAUMA RELATED TO DISUSE, • INCREASE FAMILY COPING SKILLS, • EDUCATION ABOUT THE CONDITION, AND SUPPORT.
  • 18. REHABILITATIVE CARE • REHABILITATIVE TREATMENTS FOR CHILDREN WITH SPINA BIFIDA. • PHYSICAL, OCCUPATIONAL, AND SPEECH THERAPY ARE OFTEN AN INTEGRAL PART OF CARE. • THEY CAN HELP CHILDREN IMPROVE MUSCLE COORDINATION AND BALANCE, BUILD STRENGTH, AND OVERCOME LANGUAGE DELAYS. OUR NEUROMUSCULAR EXPERTS ALSO HELP CHILDREN LEARN TO FUNCTION INDEPENDENTLY, BUILD SOCIAL SKILLS, AND GAIN CONFIDENCE.
  • 19. MEDICAL AND SURGICAL MANAGEMENT • INITIAL SURGERY TO REPAIR THE SPINE • TREATING HYDROCEPHALUS • PHYSIOTHERAPY • OCCUPATIONAL THERAPY • MOBILITY AIDS • TREATING BONE AND JOINT PROBLEMS • TREATING BLADDER PROBLEMS • TREATING BOWEL PROBLEMS
  • 20. INSTITUTES HELPING CHILD WITH SPINA BIFIDA •SPINA BIFIDA FOUNDATION PAKISTAN •SPARC(SOCIETY FOR THE PROTECTION OF THE RIGHTS OF THE CHILD) ADVOCATES FOR PROTECTION OF CHILD RIGHTS BY PROVIDING TRAININGS TO CHILD RIGHTS ACTIVISTS
  • 21.
  • 22. CEREBRAL PALSY •NEUROLOGICAL DISORDER WHICH MAY OCCUR BEFORE BIRTH, DURING BIRTH OR AFTER BIRTH. •ACCORDING TO CDC OUT OF EVERY 1000 CHILDREN CEREBRAL PALSY AFFECTS 1.5 TO 4 CHILDREN WORLD WIDE.
  • 23. CEREBRAL PALSY • CEREBRAL……HAVING TO DO WITH BRAIN • PALSY……. WEAKNESS OR PROBLEMS WITH BODY MOVEMET. MEDICAL CONDITION USUALLY CAUSED BY ABNORMAL BRAIN DEVELOPMENT BEFORE OR AT BIRTH THAT CAUSES THE LOSS OF CONTROL OF THE ARMS AND LEGS. CONGENITAL DISORDER OF MOVEMENT, MUSCLE TONE OR POSTURE.
  • 24. SIGNS AND SYMPTOMS: •THE BRAIN CAUSING CP DOES NOT CHANGE WITH TIME SO THE SYMPTOMS USUALLY DON’T WORSEN WITH AGE. VARIATION IN MUSCLES TONE. DELAYS IN REACHING MOTOR SKILLS MILESTONES. ATAXIA SPASTICITY DIFFICULTY IN WALKING, WRITING, SPEAKING AND LEARNING. EXCESSIVE DROOLING AND PROBLEM WITH SWALLOWING.
  • 25. NEUROLOGICALPROBLEMSASSOCIATEDWITHCP: DIFFICULTY IN SEEING AND HEARING. INTELLECTUAL DISABILITIES. SEIZURES. MENTAL HEALTH CONDITION. ORAL DISEASES. URINARY INCONTINENCE.
  • 26. CAUSES: GENE MUTATION MATERNAL INFECTION. FETAL STROKE. INTRACRANIAL HEMORRHAGE. INFANT INFECTION. TRAUMATIC HEAD INJURY. LACK OF O2 (ASPHYXIA).
  • 28. 1.MATERNAL HEALTH:  CYTOMEGALOVIRUS  GERMAN MEASLES (RUBELA)  SYPHILLIS.  TAXOPLASMOSIS. 1.INFANT ILLNESS:  BACTERIAL MENINGITIS.  VIRAL ENCEPHALITIS.  SEVERE OR UNTREATED JAUNDICE;  BLEEDING INTO THE BRAIN.
  • 29. TYPES OF CP: a.SPASTIC CP: b.DYSKINETIC CP. c.HYPOTONIC CP. d.ATAXIC CP.
  • 30. SPASTIC CP: AFFECTING APPROXIMATELY 80% OF PEOPLE WITH CP. MUSCLES WEAKNESS AND PARALYSIS MAY BE PRESENT. THE SYMPTOMS CAN AFFECT THE ENTIRE BODY OR JUST ONE SIDE OF BODY. SYMPTOMS: INVOLUNTARY LIMB MOVEMENT. CONTINUOUS MUSCLES SPASMS. ABNORMAL WALKING . FLEXION AT THE ELBOW, WRISTS,FIGERA ETC.
  • 31. a.DYSKINETIC CP: •THIS CAUSE INVOLUNTARY, ABNORMAL MOVEMENT IN THE ARMS LEGS AND HANDS. a.HYPOTONIC CP: •CAUSES DEMINISHED MUSCLES TONE AND OVERLY RELAXED MUSCLES. •THE ARMS AND LEGS MOVE VERY EASILYAND APPEAR FLOPPY.
  • 32. ATAXIC CP: • CHARACTERIZED BY VOLUNTARY MUSCLES MOVEMENT THAT OFTEN APPEAR DISORGANIZED,CLUMPSY AND JERKY. • PEOPLE WITH THIS FORM OF CP USUALLY HAVE PROBLEMS WITH BALANCE AND CORDINATION. SYMPTOMS: • UNSTEADY MOVEMENT DUE TO DIFFICULTY IN BALANCE • TREMORS • SLOW EYE MOVEMENT • DIFFICULTY IN FINGERS MOVEMENT
  • 33. MIXED CEREBRAL PALSY: •SOME PEOPLE HAVE A COMBINATION OF SYMPTOMS FROM THE DIFFERENT TYPES OF CP,THIS IS CALLED MIXED CP. •IN MOST CASES OF MIXED CP, PEOPLE EXPERIENCE A MIX OF SPASTIC AND DYSKINETIC CP. •PREVENTION: •MAKE SURE YOU ARE VACCINATED (RUBELLA) •TAKE CARE OF OF YOUR SELF BY TAKING HEALTHY DIET AND REGULAR MEDICINES. •REGULAR VISIT TO THE DR DURING PREGNANCY; •PREVENT HEAD INJURIES. •AVOID ALCOHOL ,TOBACCO AND ILLEGAL DRUGS DURING PREGNANCY.
  • 34. MEDICAL MANAGEMENT: •MEDICATIONS AND ORTHOPEDIC SURGERIES. •ASSISTIVE AIDS: •EYE GLASSES. •HEARING AIDS. •WALKING AIDS. •WHEELCHAIRS ETC.
  • 35. OTHER TREATMENT: • SPEECH THERAPY • PHYSICAL THERAPY • OCCUPATIONAL THERAPY. • RECREATIONAL THERAPY • PSYCHOTHERAPY • SOCIAL SERVICES CONSULTATION.
  • 36. NURSING MANAGEMENT: •PROVIDE ADEQUATE NUTRITION. •MAINTAIN SKIN INTEGRITY. •PROMOTE SAFETY. •PROMOTE GROWTH AND DEVELOPMENT. •TEACH PARENT SHOW TO CARE FOR CHILD. •PROVIDE EMOTIONAL SUPPORT..
  • 37.
  • 38. MUSCULAR DYSTROPHY INTRODUCTION:  MUSCULAR DYSTROPHIES (MDS) CONSTITUTE THE LARGEST AND MOST IMPORTANT SINGLE GROUP OF MUSCLE DISEASES OF CHILDHOOD.  THE MDS HAVE A GENETIC ORIGIN IN WHICH THERE IS GRADUAL DEGENERATION OF MUSCLE FIBERS, AND THEY ARE CHARACTERIZED BY PROGRESSIVE WEAKNESS AND WASTING OF SYMMETRIC GROUPS OF SKELETAL MUSCLES, WITH INCREASING DISABILITY AND DEFORMITY.
  • 39. THE MOST COMMON FORM, I. DUCHENNE MUSCULAR DYSTROPHY (DMD). II.FACIOSCAPULOHUMERAL (LANDOUZY-DEJERINE) MUSCULAR DYSTROPHY • LIMB-GIRDLE MUSCULAR DYSTROPHY (LGMD
  • 40. CLINICAL MANIFESTATION: • ALTHOUGH GIRLS CAN BE CARRIERS AND MILDLY AFFECTED, IT'S MUCH MORE COMMON IN BOYS. • SIGNS AND SYMPTOMS, WHICH TYPICALLY APPEAR IN EARLY CHILDHOOD, MIGHT INCLUDE:  FREQUENT FALLS  DIFFICULTY RISING FROM A LYING OR SITTING POSITION  TROUBLE RUNNING AND JUMPING  WADDLING GAIT  WALKING ON THE TOES  LARGE CALF MUSCLES  MUSCLE PAIN AND STIFFNESS  LEARNING DISABILITIES  DELAYED GROWTH
  • 41. CAUSES  CERTAIN GENES ARE INVOLVED IN MAKING PROTEINS THAT PROTECT MUSCLE FIBERS. MUSCULAR DYSTROPHY OCCURS WHEN ONE OF THESE GENES IS DEFECTIVE.  EACH FORM OF MUSCULAR DYSTROPHY IS CAUSED BY A GENETIC MUTATION PARTICULAR TO THAT TYPE OF THE DISEASE. MOST OF THESE MUTATIONS ARE INHERITED.
  • 42. COMPLICATION •THE COMPLICATIONS OF PROGRESSIVE MUSCLE WEAKNESS INCLUDE:  TROUBLE WALKING. SOME PEOPLE WITH MUSCULAR DYSTROPHY EVENTUALLY NEED TO USE A WHEELCHAIR.  TROUBLE USING ARMS. DAILYACTIVITIES CAN BECOME MORE DIFFICULT IF THE MUSCLES OF THE ARMS AND SHOULDERS ARE AFFECTED.  SHORTENING OF MUSCLES OR TENDONS AROUND JOINTS (CONTRACTURES). CONTRACTURES CAN FURTHER LIMIT MOBILITY.  BREATHING PROBLEMS. PROGRESSIVE WEAKNESS CAN AFFECT THE MUSCLES ASSOCIATED WITH BREATHING. PEOPLE WITH MUSCULAR DYSTROPHY MIGHT EVENTUALLY NEED TO USE A BREATHING ASSISTANCE DEVICE (VENTILATOR), INITIALLYAT NIGHT BUT POSSIBLY ALSO DURING THE DAY.
  • 43. CURVED SPINE (SCOLIOSIS). WEAKENED MUSCLES MIGHT BE UNABLE TO HOLD THE SPINE STRAIGHT.  HEART PROBLEMS. MUSCULAR DYSTROPHY CAN REDUCE THE EFFICIENCY OF THE HEART MUSCLE.  SWALLOWING PROBLEMS. IF THE MUSCLES INVOLVED WITH SWALLOWING ARE AFFECTED, NUTRITIONAL PROBLEMS AND ASPIRATION PNEUMONIA CAN DEVELOP. FEEDING TUBES MIGHT BE AN OPTION.
  • 44. CHARACTERISTICS OF MUSCULAR DYSTROPHY: •EARLY ONSET, USUALLY BETWEEN 3 AND 7 YEARS OLD • PROGRESSIVE MUSCULAR WEAKNESS, WASTING, AND CONTRACTURES • CALF MUSCLE PSEUDOHYPERTROPHY IN MOST PATIENTS • LOSS OF INDEPENDENT AMBULATION BY 9 TO 12 YEARS OLD • SLOWLY PROGRESSIVE, GENERALIZED WEAKNESS DURING TEENAGE YEARS
  • 45. NURSING CARE  THE MAJOR EMPHASIS OF NURSING CARE IS TO HELP THE CHILD AND FAMILY COPE WITH A CHRONIC, PROGRESSIVE, INCAPACITATING DISEASE;  THE GOALS OF CARE SHOULD ALSO INVOLVE DECISIONS REGARDING QUALITY OF LIFE, ACHIEVEMENT OF INDEPENDENCE, AND TRANSITION TO ADULTHOOD.  NURSES CAN ASSIST WITH DECISION MAKING BY EXPLORING ALL AVAILABLE OPTIONS AND RESOURCES AND SUPPORT THE CHILD AND FAMILY IN THE DECISION.  OLDER BOYS WITH MD MAY ALSO NEED PSYCHIATRIC OR PSYCHOLOGICAL COUNSELING TO DEAL WITH ISSUES SUCH AS DEPRESSION, ANGER, AND QUALITY OF LIFE.  NURSES CAN BE ALERT TO THE PROBLEMS AND NEEDS AND MAKE NECESSARY REFERRALS WHEN SUPPLEMENTARY SERVICES ARE INDICATED.
  • 46. REHABILITATION CARE  MINIMIZING CONTRACTURES BY INTRODUCING REGULAR STRETCHING INTO YOUR DAILY ROUTINE  MAINTAINING FUNCTION AND ADAPTING TO ANY LOSS OF FUNCTION  MONITORING FUNCTION OVER TIME THROUGH STANDARD TESTS AND MEASURES  ASSESSING FOR AND MANAGING COMPROMISED SKIN INTEGRITY  PREVENTING AND MANAGING PAIN  PRESCRIBING EXERCISE AND SUPERVISING SAFE PHYSICALACTIVITY (I.E. LOW LOAD, LOW RESISTANCE EXERCISE)  RECOMMENDING MOBILITY DEVICES, ADAPTIVE SEATING, AND OTHER EQUIPMENT  REHABILITATION AFTER INJURY OR FRACTURE
  • 47. MEDICAL MANAGEMENT  CORTICOSTEROIDS, SUCH AS PREDNISONE AND DEFLAZACORT (EMFLAZA), WHICH CAN HELP MUSCLE STRENGTH AND DELAY THE PROGRESSION OF CERTAIN TYPES OF MUSCULAR DYSTROPHY. BUT PROLONGED USE OF THESE TYPES OF DRUGS CAN CAUSE WEIGHT GAIN AND WEAKENED BONES, INCREASING FRACTURE RISK.  HEART MEDICATIONS, SUCH AS ANGIOTENSIN-CONVERTING ENZYME (ACE) INHIBITORS OR BETA BLOCKERS, IF MUSCULAR DYSTROPHY DAMAGES THE HEART.  GOLODIRSEN (VYONDYS 53) FOR TREATMENT OF SOME PEOPLE WITH DUCHENNE DYSTROPHY WHO HAVE A CERTAIN GENETIC MUTATION.
  • 48. SURGICAL MANAGEMENT  SURGERY MIGHT BE NEEDED TO CORRECT CONTRACTURES OR A SPINAL CURVATURE THAT COULD EVENTUALLY MAKE BREATHING MORE DIFFICULT. HEART FUNCTION MAY BE IMPROVED WITH A PACEMAKER OR OTHER CARDIAC DEVICE.
  • 49. OVERVIEWOF INSTITUTE WORKING IN PAKISTAN I. MUSCULAR DYSTROPHY REGISTRY OF PAKISTAN: • THE MUSCULAR DYSTROPHY REGISTRY OF PAKISTAN HAS BEEN SETUP WITH A GOAL TO IMPROVE OUTCOMES AND QUALITY OF LIFE IN PATIENTS WITH MUSCULAR DYSTROPHIES AND RELATED NEUROMUSCULAR DISORDERS. THE REGISTRY IS CURRENTLY RECRUITING PATIENTS WITH DUCHENNE/BECKER MUSCULAR DYSTROPHY AND SPINAL MUSCULAR ATROPHY.
  • 50. I. BAQAI INSTITUTE OF PHYSICAL THERAPY REHABILITATION MEDICINE •THE DEPARTMENT OF PHYSIOTHERAPY WAS ESTABLISHED IN 2004. THE DEPARTMENT AND ITS MANAGEMENT ARE ALWAYS COMMITTED TO SERVE THE RURAL POPULATION SPECIALLY. PHYSIOTHERAPIST IN THE DEPARTMENT WORK WITH THE MULTIDISCIPLINARY TEAM OF HEALTH CARE PROVIDERS TO PROVIDE HOLISTIC CARE THAT IS ALIGNED WITH PATIENT’S TREATMENT GOALS. THE DEPARTMENT STRIVES TO PROVIDE INPATIENT AND OUTPATIENTS SERVICES THAT IS BASED ON THE HIGH STANDARDS OF CARE AND QUALITY. THE DEPARTMENT OFFERS ITS SERVICES TO THE HOSPITAL’S ICUS, HDUS, AND MULTIDISCIPLINARY WARDS. THE INPATIENT AND OUTPATIENT PHYSIOTHERAPY SERVICES OFFER ASSESSMENT FUNCTION, MOBILIZATION AND ABILITY TO FUNCTION INDEPENDENTLY AND TO RESTORE QUALITY OF LIFE.
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  • 72. Rehabilitation care Approximately 40% of patients who are hospitalized with GBS will require admission to inpatient rehabilitation. For GBS persons necessitating admission to inpatient rehabilitation, the requirement of prior ventilator support most strongly predicts an extended length of stay on inpatient rehabilitation.
  • 73.
  • 74. Overview in Pakistan The Aga Khan University Hospital, Karachi, Pakistan. ) Thirty-four cases of GBS were admitted to the hospital during the study period, with an age range of 3 to 70 years. The mean age for disease onset was 35.2 years for female patients, compared to 30 years for males; the male/female ratio was 1.6:1.Gastrointestinal infections (12/22, 54.6%) were the most common antecedent event, followed by upper respiratory tract infections (9/22, 40.9%) and skin lesions (1/22, 4.5%). Most patients developed GBS within one month of the preceding infection. Cranial nerve abnormalities (30/34, 88.2%), autonomic dysfunction (21/34, 61.8%) and respiratory failure requiring intubation (19/34, 55.9%) were also common
  • 75. Overview in Pakistan We found that GBS occurred at all ages and was slightly more common in males. Majority of patients had an antecedent history of infection and had severe disease on presentation. The patients were treated with either plasmapheresis or intravenous immunoglobulins and there was no significant difference in outcome in the two groups. Despite severe persistent disability, in-hospital mortality was low