CASE PRESENTATION

Presenter : Dr monica ram

Moderator : Dr. [Link] sir
Informant : mother, 34yrs
Address : rajanagaram
Education : 10 th class
Reliability : fair.

sasikumari 7year female child born through non consanguinous marriage

of 2 nd birth order
CHIEF COMPLAINT :
• Known case of developmental delay with complaints of
• Fever-3 days
• cough,cold-3 days
• breathlessness-1 days
HISTORY OF PRESENT ILLNESS:
• Fever sudden onset intermittent high grade not associated with
chills and rigor no diurnal variation relieved on taking medica-
tion
• Cold associated with running nose and productive cough
present since 3 days
• Cough productive ,white colour mucoid sputum since 3days as-
sociated with breathlessness since 1 day
• No h/o bleeding gums,melena,rash over the body,hematuria
• no h/o abdominal distension,
• no h/o ear discharge , no h/o recent immunization
• h/o abnormal excessive cry was noted during early infancy.
• h/o stiffening of all lower limbs noted since 7 months of age,
• h/o difficulty in wearing diapers due to stiffening
• h/o wasting /thinning of muscles present
• h/o crossing over of legs present when trying to walk.
• h/o persistent fisting of hand present
• h/o sleep disturbance present
• h/o feeding difficulties present
• No h/o repeated tongue thrusting present.
• h/o constipation present
• No h/o abnormal movements
• No history of any early hand preference
• No h/o drooping of eyelids (follows objects)
• No h/o abnormal deviation of angle of mouth
• no h/o excessive drooling of saliva present.
• no h/o abnormal deviation of eyes
• No h/o of hearing problems(responds when called)
• No h/o nasal regurgitation of feeds
• h/o coughing while feeding
• no h/o difficulty in swallowing/chewing
• Able to perceive hot and cold water during bath.
• Able to perceive touch and pain.
• No h/o nodding movement of head.
• No h/o any bed sores.
PRENATAL HISTORY
•Maternal age at conception -25years
•Father age at conception-40 years
•Conceived spontaneously after 4 year of marietal life.
•pregnancy confirmed by UPT
•1st trimester
•booked case
•H/o periconceptional intake of folic acid.
•No h/o maternal hypothyroidism/ PIH/GDM
•No h/o fever/rash
•No history of radiation/drug intake
•HIV/HBsAg/VDRL - negative.
• 2nd TRIMESTER :
• 2 doses of TT taken one month apart
• Regular IFA supplements taken
• Regular ANC scans done
• QUICKENING movement felt at 5th month.
• 3RD TRIMESTER :
• Antenatal scans – normal
• No h/o fever, rash,uti
• No history of PIH/GDM.
• weight gain during pregnancy -10 kgs.
• NATAL HISTORY
• S/TG/NVD/MCH/3.6kgs
• delayed cry after birth,bag and mask ventilation done
• MSL+
• No H/o cord around neck , birth injuries.
• History of prolonged labour present >24 hrs

• NEONATAL HISTORY
• Baby was breast fed after 5 days of birth
• H/o difficulty in latching present
• h/o Nicu admission for 7 days
Past history
• Mother noticed that child was not able to hold the neck even at till 5
months of age for that the child was taken to hospital but not investig-
ated@ 7 months taken to another hospital MRI done reports unavailable
• @8 th month seizures noted on syp levipill
• Physiotherapy done for few months at 3 years of age later discontinued
Developmental history - GLOBAL DEVELOP-
MENTAL DELAY (DQ -11%)
DOMAIN MILESTONE AGE OF ATTAIN- EXPECTED AGE % DQ
MENT
GROSS MOTOR Neck holding - 3months 0%

FINE MOTOR unidextrous reach 3yrs 6months 17%

SOCIAL MILESTONE Social smile 3 yrs 2months 8.3%

Recognizes mother 3 yrs 3months

LANGUAGE MILE- 18.75%

STONE monosyllables 4year 9 months
Nutritional history
• Exclusively breast fed till 6 months
• Started complimentary feeding from 7th month
• feeding difficulties +
• By 24hr recall method

REQUIRED INTAKE DEFICIT

CALORIES – 1700 kcal/day 1250Kcal/day 450kcal/day
PROTEINS – 30 gm/day 18/day 12gm/day
immunization
AGE VACCINES TO BE GIVEN STATUS
At birth BCG, OPV, Hep B given
6 weeks OPV 1,Penta 1,Rv 1 given
10 weeks Opv 2,Rv 2,penta 2 Given
14 weeks Opv 3,Rv 3,penta 3,Ipv 2 Given
9months ,MR-1,vit A -1 Given
18 months DPT B1,opv B1,MR 2,vit A 2 given
 FAMILY HISTORY
• non consanguineous marriage
• No h/o developmental delay and intellectual disability in the family
• No h/o seizures ,sibling death.
SOCIOECONOMIC HISTORY
• Pukka house : 2 rooms, separate bathrom
• Water supply: corporate water
• Father: 9th standard, Carpenter
• Mother: 10th standard
• Per capita income: 15000
• Modified kuppuswamy scale: 11
• Class: lower middle
SUMMARY
• A 7 years old female child born out of non consanguinous marriage
belonging to class 3 modified kuppuswamy scale presented with gross
developmental delay with stiffening of lower limbs associated with
contractures with feeding difficulties with fever ,cough,cold
• PROVISIONAL DIAGNOSIS
• System involved : CNS
• Etiology : ?Cerebral palsy( static course, motor
problems,developmental delay,seizures)
• Metabolic disorders
GENERAL EXAMINATION
• Alert, eye contact not present
• Posture : supine lying on the bed
• Pallor +, No icterus, cyanosis, clubbing, lymphadenopathy or pedal
edema
VITALS
• PR- 124/min, regular, normal volume, no specific character,all pe-
ripheral pulses felt
• RR- 34/min, thoracic type , spo2 -99% with oxygen
• BP- 90/70 in right arm, supine position
• CRT <3sec
• Temp 99°F
ANTHROPOMETRY
PARAMETER OBSERVED DEVIATION
WEIGHT 17kg <-3SD
Height 110cm -2 to-3 SD
US:LS 1.2:1 -
HEAD CIRCUMFERENCE 44cms <-3SD
HEAD TO TOE EXAMINATION
• Skull- microcephaly, no abnormal shape
• Skin –BCG scar present
• Hair – normal
• Face - no dysmorphism / asymmetry
• Eyes – normal colour, no deviation
• ENT – normal
• Oral cavity – normal
• Tongue –decreased bulk, no fasiculations
• Chest,- normal
• abdomen – normal
• Limbs –upper limbs -normal,lower limbs - contractures present at knee and ankles
• Nails- normal
• Back – no tuft of hair/ sacral dimple
SYSTEMIC EXAMINATION
• HIGHER MENTAL FUNCTIONS
• Conscious
• Speech - monosyllables
• memory -
• orientation to time,place,person -no
CRANIAL NERVE EVALUATION RIGHT SIDE LEFT SIDE
1ST cranial nerve smell Could not be assessed Could not be assessed
2nd nerve Follows objects Yes Yes
Reaches objects Yes Yes
Pupillary reflex B/L PERL B/L PERL

3,4,6th nerve EOM Present Present

Ptosis NIL NIL

Accommodation reflex Could not be assessed Could not be assessed

5th nerve Sensations on face Present Present
Corneal reflex Present Present
Jaw jerk normal normal
7th nerve Facial asymmetry No No
Deviation of angle of no No
mouth(showing teeth)
Drooling of saliva no no
Nasolabial folds Present Present
Corneal reflex Present Present
Conjuctival reflex Present Present
8th nerve Responds to sounds Present Present
9,10th nerve Gag reflex Could not be assessed Could not be assessed
Position of uvula No deviation No deviation
11th nerve scapula deformity No No
Shrugging of shoulders cold not be assessed
12th nerve Tongue bulk , normal, normal
Not able to protrude the
tongue
MOTOR SYSTEM EXAMINATION
• BULK OF MUSCLE
RIGHT LEFT
UPPER LIMB - ARM 11.5cms 11.5 cms
FOREARM 10cms 10 cms
LOWER LIMB – THIGH 22 cms 22cms
- CALF 16 cms 16cms

TONE UPPER LIMBS LOWER LIMBS

INSPECTION NORMAL increased,contractures present at

knee and ankle
PALPATION NORMAL Incrreased

PASSIVE MOVEMENTS Normal decreased

Range of passive movements Normal decreased

POWER
POWER LIMB RIGHT LEFT

NECK UNABLE TO LIFT HEAD

TRUNK UNABLE TO LIFT TRUNK

UPPER LIMB SHOULDER – abduction 4/5 4/5

ELBOW - flexion, exten- 4/5 4/5

sion
WRIST – flexion, exten- 4/5 4/5
sion
POWER LIMB RIGHT LEFT
LOWER LIMB HIP – flexion 3/5 3/5
KNEE – flexion, extension 3/5 3/5
ANKLE – dorsiflexion 3/5 3/5
Plantar flexion 3/5 3/5
REFLEXEX RIGHT LEFT

SUPERFICIAL CONJUCTIVAL Present Present

CORNEAL present present
ABDOMINAL Present Present
PLANTAR Extensor extensor

DEEP BICEPS 1 1
TRICEPS 1 1
SUPINATOR 1 1
ANKLE 3 3
KNEE 3 3
 PRIMITIVE REFLEXES
PRIMITIVE REFLEXES APPEARANCE
MOROS ABSENT
ATNR ABSENT
STNR ABSENT
ROOTING REFLEX ABSENT
SUCKING REFLEX ABSENT
GALANT REFLEX Not done
GLABELLAR TAP NORMAL
PALMAR GRASP ABSENT
PLANTAR GRASP ABSENT
CROSSED EXTENSOR REFLEX Absent
STEPPING REFLEX Not done
PLACING REFLEX Not done
PALMOMENTAL REFLEX ABSENT
• BRAINSTEM REFLEXEX
• ATNR – absent
• STNR – absent
• MIDBRAIN REFLEXEX
• Neck on body reflex(righting reflex) : negative
• POSTURAL REFLEXES
• Parachute reflex : present
• Landau reflex : not done
• CORTICAL REFLEXEX : negative
•SENSORY SYSTEM : normal
•CEREBELLAR SIGNS :
•Gait : couldnotbe assessed
•Dysarthria present
•No nystagmus
•Finger nose test incoordination could not be assessed
•Unable to perform rapid alternate movements
•Unable to walk in a straight line

•No s/o meningeal irritation

•Spine - normal
OTHER SYSTEMS
• CVS – S1S2 present, no murmur
• P/A - soft no HSM, BS+
• RS – B/NVBS+, no added sounds.
Differential diagnosis:
• [Link] palsy- developmental delay,tone
abnormalities,contractures
• [Link] anomalies of brain-
microcephaly,developmental delay
• [Link] disorders-non consanguinous
marriage,developmental delay,
PROVISIONAL DIAGNOSIS
SYMPTOMS SIGNS INFERENCE DIAGNOSIS

Global developmental de- Microcephaly Chronic, non progressive CEREBRAL PALSY

lay significant perinatal spasticity of lower limbs motor abnormality,
event contractures at knee and
ankle

SPASTIC DIPLEGIC TYPE OF CEREBRAL PALSY with GDD with microcephaly with
chronic malnutrition secondary to intrapartum asphyxia ? congenital malformations
of brain?metabolic disorders MAS 2 GMFCS - 4 MACS - 5 CFCS - 4, EADCS - 2
Investigations:
• CT brain - To rule any intracranial calcifications
• MRI brain- To rule out any structural malformation
of brain
• EEG - To know focal or generalised
• thyroid profile
• Serum ammonia,lactate
• ABG
• whole genome sequencing
 Management
Ideally cp management should be done by multidisciplinary team in a single
centre.
It comprises- pediatrician,
Neurologist,
Occupational therapist,
Physiotherapist,
Orthopedician,
Orthotist,
Ophthalmologist,
Audiologist,
Speech &language pathologist
Psychologist,
• Physiotherapy:
• -should be done atleast thrice a day
• - no long term benefit for children with severe disease or bey-
ond 4- 7 yrs of age.
• Occupational therapy:
• -use of techniques & adaptive equipment for activities of daily
living like feeding,dressing.
• -constraint induced movement therapy(CIMT)
• -Hand arm bimanual intensive
• therapy(HABIT):
• Speech therapy:
• -stimulating environment
• -exercises for improving muscles of tongue and throat
• Orthopedic management:
• - aimed to prevent deformities, improve function,relieve pain
• -like passive stretching,splinting,serial casting
-invasive like tendon lengthening,contracture release, stabilization
of joints.
Dietary advice:
-calorie dense,
-balanced semi solid
-small quantities frequently
-restricted cal in non ambulatory to prevent obesity.
Management of spasticity:
-physical therapy: splints,orthotics
-Drugs:
Botulinum toxin A-for localized. spasticity.(<4yrs)
Diazepam,tizanidine- for short term treatment.
Intrathecal baclofen
-surgery:
Selective dorsal rhizotomy
For other neurological issues:
-levodopa,carbamazepine,
trihexyphenidyl for dystonia.
-AED's
-oral glycopyrrolate
Educational placement:
Advocacy and parent support groups:
Management in resource constrained situations:
-management is family
centered,competent,comprehensive,compassionate,continuous,co
mmunity based in india.
Following parental tips should be given:
-proper handling and positioning
-stimulating environment
- promoting speech and communication
-encourage play in different positions
-handle behavioral issues
Outcome
-have higher mortality & morbidity due to health issues.
-poor prognostic factors:
spastic quadriplegia,
Motor delay apparent within 6months
Persistent primitive reflexes by 2yrs,
Inability to sit by 4 yrs
Inability to walk by 7 yrs
Good prognostic factors:
spastic diplegia,
Head balance in prone by 9 months
Sitting by 2 yrs
Crawling by 30 months
Good family support
Access to early intervention
Absence of comorbidities
Prevention
-primary prevention:
Improvement in antenatal health care, during delivery, administra-
tion of antenatal steroids in preterm labour's.
Neonatal period( educating mothers about exclusive breast
feeding,teaching danger signs)
Secondary prevention:
-to perform developmental surveillance in all children with earlier
and more frequent screening in high risk children.
-early detection and timely intervention.
Thank you