Professor Neonatology - Neocon2019NORMAL GM (DEFINITION) •1st used by Dr Prechtl in 1990 –A...

PHOTO

Professor (Neonatology)

PGIMER, Chandigarh

MD,DM

RESEARCH AND AREA OF

INTEREST

Neurodevelopment

RIGHT TOOLS FOR EARLY DIAGNOSIS OF CEREBRAL PALSY FOR AT RISK NEWBORN

DIAGNOSIS OF CEREBRAL PALSY

Clinical HISTORY + Neurological

Supported by neuroimaging

Most common physical disability in childhood

Physical disability worse in LMIC due to sepsis

PREVALENCE OF CEREBRAL PALSY

• 14.6% in extreme preterm (22-27 weeks )

• 6.2% in very preterm (28-31 weeks )

• 0.7% in late preterm ( 32-36 weeks)

• 0.11% in term

(Oskoui M et al. A systematic review and meta-analysis, DMCN 2013)

OUR CP RATES IN VLBW AND ELBW COHORTS

• Neurodevelopmental and Behavioral Outcome of

Very Low Birth Weight Babies at Corrected Age of

2 Years ( Mukhopadhyay et al, IJP,2010) - 3% had CP

• Neurodevelopmental Outcome of Extremely Low

Birth Weight Children at Corrected Age of Two

Years (Mukhopadhyay et al, IP, 2016) – 10% had CP

IN EARLY LATE 80’S AND EARLY 1990’S…….

Diagnosis was based on

• RISK FACTORS

• CLINICAL and NEURO EXAMINATION

• ULTRASOUND/CT SCAN

WAIT TILL 1 YEAR BEFORE FINAL LABELING OF CP IS DONE !!!

It could be transient

Preterm often have transient dystonia

which may improve

TYPICAL RISK FACTORS

• h/o SB, abortion,low SES

• ART, abn genetics, PEAntenatal

• Hypoxia, seizures, hypoglycemia

• IUGR, infections,NNJ, prematurity

• IVH,PVL, STEROIDSPerinatal

• trauma

• infections

Post-neonatal

SUMMARY OF RISK FACTORS FROM SYSTEMATIC REVIEWS

Risk fators Risk

Lower GA (<37 WKS) 8.3% in <28 wks

4.3% in 28-31 wks

0.7% in 32-36 wks

Low birth weight (<2500 g) 5.6% in <1000g

5.9% in 1000-1499 g

0.1% in 1500-2499 g

SGA OR 2.34 , 95% CI 1.4-3.8

Chorioamnionitis (<37 wks) RR 1.9 , 95%CI 1.4-2.5

Postnatal steroids <32 wks) ,

EARLY USE (<7 days)

RR 1.45 , 95% CI 1.06 – 1.98

TYPICAL NEURO EXAMINATION

Warning signs

• Lack of alertness

• Decreased spontactivity

• Abnormal movements

• Fisting after 2-3 mo

Abnormal signs

• Low HC

• Delayed social smile

• Persistent NNR/ATNR

• EXCESS EXTENSOR TONE

• ASYMMETRY

Associated signs

• Ocular –roving eye movements

• Squint

• Poor fixation and following

• Auditory –poor response

NEUROIMAGING

• USG

• CT scan ( not preferred any more, helpful to see

calcification in IU infections)

• MRI

CRANIAL ULTRASOUND

• Excellent bedside tool

• Being used since 70’s

• Can detect major brain injuries

- IVH, PVH (Periventricular haemorrhagic infarcts)

- CEREBELAR HAEMORRHAGE (58% children with

CP have cerebellar injury after IVH)

- PVL - cystic

Grade IV IVH, risk of CP 47%Cystic PVL, PPV 77%

CAN WE DIAGNOSE CP EARLY ?

WHY EARLY DIAGNOSIS IMPORTANT

• Delayed intervention if diagnosis is delayed

• Plasticity of brain period is missed

• To maximize functional outcomes

• To reduce parents anxiety

• TO AVAIL SOCIAL BENEFITS

EARLY DIAGNOSIS < 5 MONTHS

• History of high risk factors

+

• Standardized motor assessment (if available)

+

• Standardized neurological examination

+

• Neuroimaging (MRI)

EARLY DIAGNOSIS-BEST PREDICTIVE VALIDITY

• Prechtl general movement assessment (GMA)

(98% sensitivity)

+

• Hammersmith Infant Neuro Exam ( 90% sensitivity)

Best combination of sensitivity and specificity

(Novak 2017,Bosanquet 2013,Spittle AJ 2008)

• MRI (86-89% sensitivity)

STANDARIZED NEUROLOGICAL EXAMINATIONS

• HAMMERSMITH NEONATAL/INFANT

NEUROLOGICAL EXAMINATION ( HNNE/HINE)

( Dubowitz et al, 1998)

• Amiel-Tison scale for neurological

examination – no scoring

HNNE ( RICCI D ET AL, 2008)

HNNE

• Tone and posture – UL, LL, Neck ,trunk

• Reflexes

• Movements

• Abnormal signs

• Behavioral signs

• Vision

• Hearing

HINE

• Standarized 26 item neuro exam for 2-24 months

• Part I (SCORED) – 5 components

• Part II – motor milestones

• Part III - behavior

Dubowitz L et al, J Pediatrics1998;133:406-416

Haataja L et al, J Peds 1999;135: 153-61

PART –I. HINE (SCORING )

1.Cranial nerve function - Max 15

2.Posture - max 18

3.Movements – max 6

4.Tone – max 24

5.Reflexes and reactions –max 15

INTERPRETATION OF HINE SCORES

• Global score 0 - 78

• Score of < 40 always predicted CP

• Score of > 73 never associated with CP

• At 3 months , score of < 56 was highly predictive of

CP at 2 years ( Romeo DM, 2008 , Eur J Ped Neuro)

PREDICTIVE VALIDITY OF CP BY GENERAL MOVEMENTS ASSESSMENT

• SENSITIVITY - 98% ( 95% CI 73-100%)

• SPECIFICITY – 91% ( 95% CI 83-95%)

(Bosanquet M, DMCN 2013)

GENERAL MOVEMENTS

• Definition

• Typical normal pattern

• What is abnormal GM

• Role in predicting Cerebral Palsy

CAN’T SHOW VIDEO DUE TO COPYRIGHT AND CONSENTISSUES

NORMAL GM (DEFINITION)

• 1st used by Dr Prechtl in 1990 – A variety of spontaneous motor

movements starting in fetal life

- Variable sequence of arm, leg, neck and trunk

- Variable amplitude and speed and variable intensity

- Gradual beginning and end

- Fluent and elegant rotations

• These are generated by central pattern generators (CPG) located

in brain stem and modulated by supra spinal pathways

( Prechtl 1990 , Einspieler 2005 )

6-9 wks post term - writhing3-5 months post term - fidgety

NORMAL GM - DEFINITION

Preterm

Large amplitude

Faster spped

Sometimes jerky

Term

Moderate amp

Writhing

Mod speed

Fidgety

• Small amplitude

• All directions

• Variable acceleration

• Moderate speed

• Throughout body

Writhing

WHAT IS ABNORMAL GM?

• During writhing period (upto 6 weeks) post-term

Poor repertoire (PR)

Cramped synchronized (CS)- simultaneous stiff limb and

trunk movements

Chaotic

• During Fidgety period ( 6-20 weeks post-term)

absent

Abnormal/exaggerated

INTERPRETATION

Absent fidgety movements at 12-16 weeks

• Sensitivity of 95-100%

• Specificity of 85% for prediction of CP in high risk population

(Spittle AJ 2009, Noble Y 2012)

Cramped –synchronized (CS) GMs – good

predictor of CP ( Prechtl1997,Einspieler 2012b)

GENERAL MOVEMENT ASSESSMENT +

HINE

BEST COMBINATION OF SENSITIVITY

and SPECIFICITY to predict CP

GM TO PREDICT COGNITION- SYSTEMATIC REVIEW

( EINSPIELER ,2016) • Preterm with consistently abnormal GMs upto 8 weeks post term

had lower IQ at school as compared to those in whom GM

normalized

• From 3-5 months, various postural patterns predicted intelligence

at 7-10 years

• Association weak if CP excluded

• Normal GMs and normal repertoire are markers of

normal cognitive development at least till 10 years of age

CAUTION FOR GM ASSESSMENTS

• Needs expertise – basic and advanced course

• Assessment time is important (writhing to fidgety )

• Low specificity ( high false positive), may cause more anxiety in parents

• False negatives ( milder cases, assessor bias)

• Topography and type of CP not well predicted as of now

• May be use “GM optimality score” alongwith (Einspieler- 2016)

MRI – STRUCTURAL AND FUNCTIONAL

• Sensitivity - 86-89%

• Abnormal signal in white matter

• Ventriculomegaly

• Small brain size

• Presence of cysts

• Decreased myelination

• Corpus callosum thickening

• Mod to severe white matter injury – odds for CP

9.55 ( 3.2-28.3)

CLASSICAL AGE OLD SIGNS

• History – poor sleep pattern, excessive irritability, lack of interest

in surroundings, poor interaction, choking during feeding, excessive

drooling

• Tends to stand up while pull to sit

• Examination – poor head growth/small head, excess head lag,

tight fisting after 3 months, crossed legs, back arching, persistent

MORO/ATNR after 3-4 months, brisk DTR

KEEP IN MIND DIFFERENTIALS

• Global developmental delay

• Neuroregression – slowly progressive

• Spinal/Muscular/neuromuscular disorders

• Metabolic

• Transient dystonia

TAKE HOME MESSAGES

Regular follow up of high risk

neonates

Early diagnosis based on risk factors,

examination and neuroimaging

Early referral and intervention