Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

NEURODEVELOPMENTAL DISABILITIES AMONG CHILDREN IN INDIA: AN INCLEN STUDY

INCLEN Module on NEUROMOTOR IMPAIRMENTS

THE INCLEN TRUST INTERNATIONAL

Neuromotor Impairment

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

NEUROMOTOR IMPAIRMENTS

1. LEARNING OBJECTIVES

OBJECTIVES

After completing this module, the participants should be able to:



Define cerebral palsy, neuromuscular disorders, and other neuromotor impairments



Recognize the different clinical presentations of neuromotor impairments



Differentiate cerebral palsy from neuromuscular disorders and other neuromotor impairments



Evaluate a child with suspected neuromotor impairment

2. INTRODUCTION 2.1 What are Neuromotor Impairments? These are conditions associated with static (i.e. difficulty remains same or improves with time) or progressive difficulty in performing daily activities and/or coordinated movements due

to

neurological

causes.

Neuromotor

Critical concept

impairments can be broadly divided into three types: 

Cerebral palsy (CP)



Neuromuscular disorders (NMD)



Other neuromotor impairments (Other

Motor problems due to neurological or neuromuscular cause are called neuromotor impairments

NMI) The term “static” is used to refer to the brain insult/injury which is a one-time insult and does not progress. Examples include perinatal asphyxia, and neonatal jaundice. As the child grows older, the brain matures, and therefore the child improves in motor function with time

Neuromotor Impairment

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

What is NOT Neuromotor Impairment? Non neurological causes can also lead to motor problems. These are not included under the term “neuromotor impairments”. These conditions include: 

Developmental dysplasia of the hip



Club foot (Congenital talipes equinovarus)



Bone and joint disease: Trauma, infections of the joints (pyogenic or tubercular arthritis), rickets and rheumatoid arthritis



Skin and soft tissue conditions: Painful lesions like in-growing toe nail, corn in the feet, ecthyma gangrenosum etc



Contractures following burns or trauma

What is Cerebral Palsy (CP)? 

It is a disorder of tone, movement and posture.



It occurs due to static or non-progressive disturbances that have occurred in the developing brain ( e.g. birth asphyxia, kernicterus)



The motor problems are often accompanied by disturbances of sensation, cognition and communication; and seizures, feeding problems and sleep disturbances may also be present.

What is NOT Cerebral Palsy? 

Disorders of tone, posture and movement due to a progressive neurological disorder



Loss of previously attained milestones (Regression)



Delayed cognitive and language milestones, if not associated with motor delay (dysarthria from orofacial involvement in cerebral palsy may be associated with neuromotor, and speech and language delay)



Children with developmental delay in all domains (i.e. global developmental delay), but without any tone or movement abnormalities



Children with transient tone and postural abnormalities which resolve by 1 years of age should also be excluded from the diagnosis of CP. This is characteristically seen in some preterm children.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

2.2 What are Neuromuscular Disorders (NMD)? 

Disorders resulting from pathology of the anterior horn cell, nerve roots, peripheral nerve, neuromuscular junction and/or the muscle



These present with lower motor neuron (LMN) type of weakness



Their clinical course may be static or progressive



They manifest at different ages with a broad spectrum of symptoms, signs and have a variable severity

2.3 What are “Other Neuromotor Impairments” (Other NMI)? 

All neurological conditions other than cerebral palsy and neuromuscular disorders, which

cause

motor

weakness

and/or

Critical concepts

problems in activities of daily living (ADL) 

This group includes motor problems due to sequelae of central nervous system (CNS) infections,

neurodegenerative

disorders,

sequelae of neural tube defects, spinal cord injury and so on 

This group also includes consequences of one time brain insults in older children (eg. Hemiparesis due to stroke or head trauma or meningitis in child aged 8 years)



Cerebral palsy: Motor problems due to one-time insult to the developing brain i.e. before 2 years of age Neuromuscular disorders: LMN type of weakness due to disease of anterior horn cell, nerve, neuromuscular junction or the muscle Other neuromotor impairments: NMI not satisfying the definition of either CP or NMD

Their clinical course may be static or impairing.

2.4 When to Suspect Cerebral Palsy? 

There is a delay in attainment of motor milestones or, the motor milestones may be much more delayed compared to other domains



There are abnormalities of muscle tone or posture (e.g. scissoring, toe walking, hypertonia)



The clinical course is either static or improving



In acquired cases, there may be normal attainment of initial milestones till a particular age when the child suffers a CNS insult

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

2.5 Early Pointers Early identification of CP is often possible with a high risk approach especially when a baby has difficulties at birth such as asphyxia, intracranial hemorrhage or problems in postnatal life like seizures, serious infections (septicemia, pneumonia, meningitis etc.), severe jaundice requiring phototherapy or exchange transfusion, etc. Some children may show an initial delay in motor milestones, but they cannot be labelled to have CP unless they show signs of abnormal muscle tone and abnormal postural patterns associated with movement difficulties i.e. motor abnormalities are an essential feature of any child with CP. Early identification is essential to institute early intervention programs which utilize neuronal plasticity and improve neurocognitive outcome in these children.

Features in early infancy are: 

Delayed attainment of motor developmental milestones



Excessive stiffness of limbs and/or trunk (spasticity / rigidity)



Abnormal twisting postures of the limbs and /or trunk (dystonia)



Persistence or a symmetry of Moro‟s and the Asymmetric Tonic Neck Reflex (primitive reflexes) (When the infant‟s head is turned suddenly to one side, extension of arm and leg occur on face side and flexion of arm and leg occur on the other side) beyond 6 months of age



Early hand preference



Presence of cortical thumb

Features in later infancy and childhood: 

Persistence of asymmetry in posture, movement and reflexes



Excessive stiffness or abnormal twisting postures of limbs and/or trunk



Persistent Asymmetric Tonic Neck Response (ATNR)



Delay in achieving milestones like sitting without support beyond 12 months and walking without support beyond 20 months

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

3. CLINICAL SPECTRUM 3.1 Classification of CP There are various dimensions of classification including type of motor abnormality, degree of associated impairment and disability and anatomic-clinicopathologic correlates. (i) Based on type of motor abnormality 

Spastic



Dyskinetic (choreoathetoid and dystonic sub types)



Ataxic



Hypotonic



Mixed

(ii) On the basis of topography/anatomic distribution of motor weakness  Quadriplegia: All four limbs are severely affected  Diplegia: lower limb involvement is more pronounced than that of the upper limbs. Significant truncal involvement is a part of the clinical picture  Hemiplegia: Refers to the involvement of one side of the body as observed by paucity of movements and marked tone abnormalities of the affected side  Monoplegia: involvement of one limb  Triplegia (involvement of 3 limbs) is rare but can occur with variable degrees of truncal involvement

Figure 1: Child with Quadriplegia

Neuromotor Impairment

Figure 2: Child with right sided hemiplegia

Figure 3: Child with diplegia

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

3.2 Co morbidities One or more of the following co morbidities are commonly present in a child with CP 

Seizures



Intellectual disability



Feeding Difficulties



Speech and Language Disorders



Hearing Impairment



Vision Impairment



Inattention and/or Behavioural Problems



Sleep Disturbances

3.3 Evolution of CP As the child with CP grows, the delay in motor milestones become more prominent, tone abnormalities can become more marked and functional limitation can increase in the absence of active intervention. The evolution of the motor problems depends on the type of CP, severity of the problems and co morbid conditions and most importantly on interventions provided. Contractures and bone or joint deformities are commonly seen in children who are not closely monitored and managed. Consensus Clinical Criteria for diagnosis of Cerebral palsy*

Cerebral palsy is characterized by ALL of the following: 1.

Predominant motor impairment with abnormalities of (one or more of the following) a. Tone b. Movement c. Posture

*

2.

Non-progressive in nature

3.

Onset before two years of age

4.

Cerebral in origin

Developed by consensus among experts (Technical Advisory Group)

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

4. NEUROMUSCULAR DISORDERS Most children with an acute or chronic flaccid weakness have NMD. NMDs can be broadly divided into myopathies, in which the pathology is confined to the muscle; and the neuropathies, in which the weakness is secondary to an abnormality of the peripheral nerve from the anterior horn cell. A third category includes the neuromuscular junction disorders such as myasthenia gravis. Further categorization can be made into hereditary and acquired or acute and chronic disorders. A wide variety of disorders are included under the term “Neuromuscular disorders” as given below: 

Congenital myopathies



Muscular dystrophies



Spinal muscular atrophy (SMA)



Hereditary and acquired neuropathies



Myasthenia gravis



Acute flaccid paralysis (AFP) o

Paralytic poliomyelitis

o

Guillain-Barre syndrome (GBS)

o

Transverse myelitis

o

Traumatic neuritis

4.1 Other Neuromotor Impairments This includes all neurological disorders associated with motor problems but not satisfying the definition of either CP or NMD. The etiological diagnosis is predominantly based on history and the impairment is confirmed on clinical examination. Common conditions included in this category are: 

Sequelae of infections of CNS (e.g., meningitis, encephalitis etc)†



Sequelae of neural tube defects (NTD)



Sequelae of head trauma†



Stroke and its sequelae†



Other rare conditions e.g. neurodegenerative disorders, demyelinating disorders like acute disseminated encephalomyelitis (ADEM), etc.

†

Only if occurring in children more than two years of age. In children less than two years, it is classified as cerebral palsy (Reference: Bax M, Goldstein M, Rosenbaum P, Leviton A, Paneth N, Dan B, Jacobsson B, Damiano D; Executive Committee for the Definition of Cerebral Palsy. Proposed definition and classification of cerebral palsy, April 2005.Dev Med Child Neurol. 2005 Aug;47(8):571-6.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

4.2 Early Pointers to NMD 

Decreased fetal movements during antenatal period



Floppy baby/infant



Delay in attaining motor milestones



Abnormal mode of walking (high stepping gait, toe walking or waddling gait)



Difficulty in running, climbing stairs, getting up from the floor and frequent falls



Enlargement/wasting of muscles



Overt muscle weakness of recent origin

Chronic progressive weakness may be classified clinically into distal and proximal weakness. Proximal weakness 

In general, myopathies follow a proximal distribution of weakness with muscle wasting (except myotonic muscular dystrophy which presents with distal muscle weakness)



Juvenile SMA is the only childhood denervating disorder in which weakness is more proximal than distal

Distal weakness 

Weakness associated with neuropathies is generally distal in distribution (except juvenile SMA and Guillain-Barre syndrome)



These are usually hereditary. Common acquired neuropathy is Guillain Barre Syndrome (GBS), which usually has an acute onset.



Commonest presenting feature of neuropathy is progressive symmetrical distal weakness affecting the legs and then arms, with the progression of weakness and sensory loss in a distal to proximal direction (glove-andstocking distribution). Tendon reflexes are lost early in the course of illness.



Further differentiation into a disorder of cell body i.e. anterior horn cell, axon or myelin sheath is accomplished by electromyography (EMG), nerve conduction studies, muscle biopsy and nerve biopsy.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Other features 

Deep tendon reflexes are generally lost in neuropathies and are preserved proportionate to the muscle strength in myopathies



Fasciculations of muscle, often best seen in the resting tongue, are a sign of denervation and is seen in anterior horn cell disease of spinal muscular atrophy



Sensory loss is commonly seen in neuropathy



Fatigability, fluctuating weakness and improvement with rest are characteristic of neuromuscular junction disorders such as myasthenia gravis

4.3 Co-morbidities of NMD and other NMI 

Involvement of cardiac and respiratory muscles may lead to respiratory insufficiency and congestive cardiac failure



Decubitus ulcers are frequent in children who are bed-ridden, without good nursing care



Lack of proper physiotherapy frequently leads to joint contractures



Spinal deformities like scoliosis and kyphoscoliosis are also common

4.4 Natural History of NMD and other NMI 

Some of these disorders are progressive in nature (e.g. neurodegenerative diseases and muscular dystrophies)



Conditions like stroke and sequelae of CNS infections cause one-time insult and are associated with static weakness or improvement with intervention



Age at onset and rate of progression varies with individual type of disorder. Muscular dystrophies like Duchene muscular dystrophy are gradually progressive leading to loss of ambulation before 13 years of age. Polio is a one time insult with static asymmetric LMN weakness. Stroke may lead to static hemiparesis. Spinal trauma may lead to paraparesis and bladder problems.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Consensus Clinical Criteria for diagnosis of NMD and Other NMI‡

Probable diagnosis of NMD / Other NMI is made only if both A and B are fulfilled Static or progressive difficulty in performing activities of daily living or A coordinated movements. ANY of the following is present-

B

i.

Findings suggestive of static or progressive LMN dysfunction (NMD)

ii.

Findings suggestive of progressive UMN dysfunction

iii.

Static UMN dysfunction with onset after age of two years§ (Other NMI)

(Other NMI)

*LMN: Lower motor neuron; UMN: Upper motor neuron

4.5 Evaluation Procedure 

Assessment of children with suspected NMI is begun by asking history of any static or progressive difficulty in performing daily activities or coordinated movements (Section-1)



If answer to ANY of the triage questions is YES, you should proceed to detailed neurological assessment



If there is no suggestive history i.e. answer to ALL the triage questions is NO, you should proceed to observations for abnormality in walking, hands and coordination (Section-2)



If there is abnormal observation, then detailed neurological assessment should be done to confirm the presence of NMI



If history (Section-1) and the observations (Section-2) are normal, then the child does not have NMI

‡

Developed by consensus among experts (Technical Advisory Group) Bax M, Goldstein M, Rosenbaum P, Leviton A, Paneth N, Dan B, Jacobsson B, Damiano D; Executive Committee for the Definition of Cerebral Palsy. Proposed definition and classification of cerebral palsy, April 2005.Dev Med Child Neurol. 2005 Aug;47(8):571-6. §

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Section I: Triage questions for Neuromotor Impairments 0: No

1: Yes

8: Not applicable

1. Does your child have difficulty in ANY of the following? A. Sitting B. Getting up from floor C. Standing D. Walking E. Running

2. Did your child start performing the following activities later than children of his/her age? A. Started sitting without support beyond his/her first birthday B. Started walking without support beyond his/her second birthday

3. Does your child have ANY of the following? A. Excessive tightness/ limpness of the body B. Toe-walking C. Abnormal posture of any limb D. Decreased/ unequal use of any limb E. Frequent falls

4. Does your child have difficulty in performing ANY of the following activities? (For children above 4 years age only) A. Bathing/cleaning himself/herself B. Toileting C. Dressing D. Feeding self

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Section II: Observations: Write responses code in the box. 0: No

1: Yes

5. Observe for the following when the child is walking A. Limping B. Unsteadiness of gait (Ataxia) C. Toe walking D. Waddling gait E. Scissoring gait F. High stepping gait G. Unable to walk H. Any other gait abnormality (please specify) _______________________

6. Observe for the following when the child is standing up from floor A. Requires assistance for standing up from floor / unable to stand B. Gowers‟ sign positive C. Any other abnormality 7. Observe hands and look for the following A. Tremors B. Unequal power of hand grip C. Fisting of one or both hands Please specify reason for Non-applicability of any item

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Evaluation Algorithm for Diagnosis of Neuromotor Impairments All children Ask 4 triage questions (Section- 1)

Answer to ANY ONE of the triage questions is YES

Answer to ALL triage questions is NO

Detailed Neurological Assessment (Section-3)

Normal Neuromotor Examination

Abnormal Neuromotor Examination

Findings suggestive of lower motor neuron dysfunction

Observe for walking, hand function & coordination (Section-2)

Abnormal

Findings suggestive of upper motor neuron dysfunction

No Neuromotor Impairment Findings are abnormal but not fitting in any of the defined category

Progressive

Static

Indeterminate Timing of CNS Insult

Neuromusculardisorder

Other neuromotor impairments

 2 yrs OR could not be determined

< 2 yrs of age

Cerebral Palsy

NOTE: All 3 sections in questionnaire to be done irrespective of flow chart (this will help in reclassifying indeterminate cases by TAG)

Source: Developed and validated by INCLEN TAG members

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Section I: Triage questions for Neuromotor Impairments Instructions for completing section I 

This section is aimed at identifying even the slightest motor problem that may be present in a child



When there is a doubtful motor problem in any of the triage questions, consider that as abnormal, and you should perform detailed neurological assessment



This section should be completed by taking relevant history from parents



Read out the questions slowly, legibly and loudly so that parents can understand. Questions can be repeated if parents did not understand.



Parents‟ responses should be entered in the form of codes, as follows: 0: No

1: Yes

8: Not applicable

Question 1: Does your child have difficulty in ANY of the following: Sitting/ getting up from sitting position/ Standing / Walking/ Running Description: By this question you are trying to identify motor problems of lower limbs. By the age of two years, normal children should be doing these tasks without any difficulty. Any difficulty in performing these activities should be considered as abnormal in a child older than two years age. How to ask: Ask each component of the question separately and wait for parent‟s response. After getting clear response, then move on to next component. Mark YES: If the child has difficulty in ANY of the above listed activities or If the child has NOT ATTAINED that milestone Mark NO: If the child has no difficulty in the above activities

Question 2: Did your child start performing the following activities later than children of his/her age (Sitting without support beyond his/her first birthday/ Walking without support beyond 2nd birthday) Description: This question is to identify delay in development of common motor milestones which is an early feature of Neuromotor impairments beginning in infancy. How to ask: Ask each component of the question separately and wait for parent‟s response. After getting clear response, then move on to next component.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Mark YES: If the child had DELAY in attainment of these motor milestones If the child had NOT ATTAINED these milestones yet Mark NO: If the child had NO DELAY in attainment of these motor milestones

Question 3: Does your child have ANY of the following? Excessive tightness or limpness of the body/ Toe-walking/ abnormal posture of any limb/ decreased or unequal use of any limb/ Frequent falls Description: By this question, you are trying to identify tone abnormalities and their manifestations which might be noticed by the parents. How to ask: Ask each component of the question separately and wait for parent‟s response. After getting clear response, then move on to next component. Mark YES: If the child has abnormality mentioned Mark NO: If the child has no abnormality as listed in the question Mark Not Applicable: for components “frequent falls” and toe walking” if the child is unable to walk or not yet attained walking

Question 4: Does your child have difficulty in performing ANY of the following activities? (For children above 4 years age only) Bathing or cleaning self/ Toileting/ Dressing/ Feeding self Description: This question explores the motor problems in another set of day-to-day activities of a child. Note that, this question is for children aged 4 years or more only. Children above 4 years should be able to perform these daily tasks on their own. How to ask: Ask each component of the question separately and wait for parent‟s response. After getting clear response, then move on to next component. Mark YES: If there is difficulty in any of above listed activities NO: If the child has NO difficulty in any of the above activities Mark Not Applicable: For children between 2-4 years of age If parents say that “we have never allowed the child to do these tasks; we do this ourselves”

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

5. Observe for the following when the child is walking Limping/ Unsteadiness of gait (Ataxia)/ Toe walking/ Waddling gait/ Scissoring gait/ High stepping gait/unable to walk Description: Normal mature gait is a narrow-based heel-toe strike with associated reciprocal swings of arms. Following are the various types abnormal gait that may be seen in children with Neuromotor impairments Waddling gait (duck like gait): It may be seen in Duchenne muscular dystrophy (DMD), congenital dislocation of hip (bilateral), and polymyositis Foot drop gait (high stepping gait) seen in neuropathies Toe walking: Spastic diplegia (because of tightness in tendoachilles) and muscular dystrophies (because of weakness of ankle dorsiflexors) Ataxic gait: Patient walks like a drunken man with unsteady swaying, broad-based gait with tendency to fall and cannot walk in straight line Hemiplegic gait (circumduction gait): Patient circumducts a leg dragging the toe, placing the ball down without a heel strike, with the ipsilateral arm held in plantar flexion. Scissoring gait: It is due to excess adductor tone at hip joint, associated with spasticity

Figure 4A: Toe walking

Figure 4B: Left Hemiplegic gait

Figure 4C: Scissoring gait

How to test: Ask the child to walk across the room (at least 10 meters) comfortably and observe the gait. If you are attentive, you can also observe the gait when the child enters examination room.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Mark as YES: If the child has any of the abnormal gait described above If the child is unable to walk Mark as NO: If the child‟s gait is normal Mark as Not Applicable: If the child has not attained walking yet

6. Observe for the following when the child is standing up from floor Requires assistance for standing up from floor or unable to stand/ Gowers’ sign Description: When asked to stand from sitting position, the child with proximal muscle weakness has difficulty in doing so. It consists of first flexing the trunk at the hips, then placing the hands on the knees, and then extending the trunk (Butt Up approach) by using the hands to walk up the legs (see the diagram below). See if child “climbs on self‟, i.e. Gower‟s sign. Gowers’ sign:

Figure 5: Gowers’ sign in a child with Duchenne muscular dystrophy

How to test: Make the child sit down on the floor completely and then ask him/her to get up to standing position without holding the furniture. Mark as YES: If the child has difficulty in getting up from sitting on floor If the child requires assistance to stand from sitting position If the child has not learned standing yet If Gower‟s sign is positive Mark as NO: If the child has NO difficulty in getting up from sitting on floor If the child DOES NOT require assistance to stand from sitting

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

7. Observe hands and look for the following Tremors/ Unequal power of hand grip/ Fisting of one or both hands Description: In some children who apparently look normal, there may be subtle weakness in the hands indicating Neuromotor impairment. This can be identified by presence of tremors (rhythmic, regular oscillations of a body part), or unequal power of hand grip. Sometimes, the child may keep one or both of his/her hands fisted (closed fists) which indicates upper motor neuron type of weakness. How to testTremors

Ask the child to stretch his/her arms and spread out the fingers

Unequal power of hand grip

Ask the child to grasp your two fingers as forcefully as he/she can, while you try to separate your fingers

Fisting of one or both hands

Observe during the course of examination

Mark as YES: If the child has any of the above listed abnormalities in hands Mark as NO: If the child has none of the abnormalities in hands

8. Muscle Power 0: Normal

1: Decreased

Description: Muscle power is the strength of the muscle. This is graded as follows: 5-Normal (movement against gravity and full resistance) 4-movement against gravity and some resistance 3-Movement against gravity but not against additional resistance 2-Movement with gravity eliminated 1-Visible or palpable flicker of contraction but no visible movement of the part/joint 0-No movement at all Any grade from 0-4 is recorded as decreased muscle power How to test: Test at least two muscles in each limb and note down the power whether normal or decreased. In upper limbs, shoulder abductors and wrist extensors are to be tested; and in lower limbs hip extensors and ankle dorsiflexors are to be tested.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Muscle group

Testing position

How to test

Shoulder abductors

Sitting

Ask the child to elevate arms above the level of head; try to counter this by applying some pressure at elbow

Wrist extensors

Hip abductors

Sitting

with

hand Ask the child to lift the wrist up

kept flat on table top

forcefully against your hand

Prone position

Ask the child to lift knee from couch and hold it up. Place your hand on popliteal fossa and try to press it down with slight resistance

Ankle dorsiflexors

Sitting

with

legs Ask the child to dorsiflex ankle

hanging from couch

against resistance by your hand placed over dorsum of foot

9. Muscle Tone 0: Normal

1: Hypotonia

2: Hypertonia

Description: Tone is the resistance offered by muscle to passive movement of a joint. Normal tone is said to be present when a slight resistance is felt through the whole range of movement. Hypotonia is loss of/decreased resistance. It is noted in all lower motor neuron disorders, shock period of upper motor neuron lesion (few minutes to weeks), and some systemic disorders (protein energy malnutrition, Rickets etc). Marked loss of tone is called flaccidity. Hypertonia is increased tone, which is of two main types: spasticity and rigidity. Spasticity is an initial resistance/catch followed by yielding when a patient‟s resting extremity is manipulated briskly. This catch-and-yield sequence is also referred to as clasp knife spasticity. This is a sign of upper motor neuron dysfunction. Rigidity is increased resistance felt throughout the range of movement when a patient‟s resting extremity is manipulated. This steady resistance feels like bending a lead pipe (lead pipe rigidity) and when coupled with tremor it feels like ratchet like catches during spasticity movement (cog wheel rigidity).

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Figure 6: A: Spasticity causing scissoring of legs, C-Hypotonic child with frog-leg posture

How to test:

See the posture of limbs: the hypotonic limbs assume posture of limpness and lie externally rotated. If both lower limbs are hypotonic they will be in “frog-like” position. Hypertonic muscles stand out prominently and depending on the muscles involved, limb will have different postures, e.g. scissoring, Feel the muscle: hypotonic muscle is soft and flabby and hypertonic muscle is rigid or spastic Do: Passive movements of the joints and range of motion at the joints. Feel for the resistance to passive movements; whether increased or reduced. Shake the limb and look for easy flappability which indicates hypotonia. In the upper limbs, gently flex and extend the elbows and wrist joints and then rotate the arm at elbow (pronation and supination). The earliest sign of spasticity in upper limbs is often detected during pronation and supination (pronator catch). In the lower limbs, spasticity is best observed in hip extensors, knee extensors and plantae flexon of foot. 10. Deep tendon reflexes (biceps, triceps, knee and ankle jerks) 0: Normal

1: Diminished or absent

2: Exaggerated

Description: You should elicit deep tendon reflexes at biceps and triceps in upper limbs, and knee and ankle in lower limbs. DTRs are grades as follows 0: absent, 1+: Sluggish or reduced, 2+: normal, 3+: Exaggerated, 4+: Brisk with presence of clonus

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

How to test: If any reflex is not obtainable directly, then ask the patient to do some reinforcement like linking hands across the chest and pull them apart as you try for reflex. Biceps Jerk: Place patient‟s hand on his abdomen. Place your index finger on tendon. Swing hammer on to it. Watch contraction of biceps muscle and elbow flexion Triceps Jerk: Draw the arms across the chest, holding the wrist and elbow at 900. Strike the triceps tendon directly with hammer. Watch the contraction of muscle and extension of elbow Knee Jerk: Place the arm below the knee so that the knee is at 300. Strike below the patella; watch the quadriceps contraction and extension of knee Ankle Reflex: Hold the foot at 900 with medial malleolus facing the ceiling. The knee should be flexed and lying to the side, strike Achilles tendon and watch for plantar flexion of ankle

11. Plantar Response Description: It is a superficial reflex and helps to differentiate UMN from LMN dysfunction. Normal plantar response is flexor response. Extensor plantar response indicates UMN lesion. It may be normally seen till the age of 2 years. Flexor plantar response in the presence of hypotonia and decreased muscle power indicates LMN dysfunction.

How to test: Gently draw a blunt stimulus along the lateral border of the sole. Watch the big toe and remainder of the toes. Possible responses are as follows:

Response

Interpretation

All toes flex

Flexor response

Extension of great toe and fanning of other toes

Extensor plantar response

No movement

Not elicitable

Big toe extends, other toes extend, ankle dorsiflexion

Withdrawal response

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

12. Balance and Coordination 0: Normal

1: Poor balance of trunk or limbs (Ataxia)

Description: Poor balance of limbs result in unsteadiness while walking, called ataxic gait. Poor balance of trunk is called truncal ataxia. How to test: Truncal ataxia is seen as swaying/unsteadiness while sitting Ataxic gait can be identified by asking the child to walk

13. Abnormal Movements 0: No abnormal movements 1: Abnormal movements Description: Abnormal movements like choreoathetoid movements, dystonia, and ballismus may be seen in children with cerebral palsy (dyskinetic type of CP). Dystonia: It is disorder of movement characterized by sustained co-contraction of agonist and antagonist muscles resulting in repetitive twisting movements and abnormal postures of trunk, neck, face, arms or legs. The stiffness associate with dystonia is usually increased in waking state and during movement of the body part. It is reduced during sleep and at rest.

Figure 7: A-Dystonia of hand

Tremor: Rhythmic, regular oscillations of a body part. Types: resting tremor, Parkinsonism

tremor, intentional tremor, action tremor, positional or postural tremor Chorea: Sudden, jerky, nonrepetitive, quasi purposive movements. It is most prominent in the extremities but can be seen in tongue, face, exaggerated by emotion, attention and action, and disappears during sleep Athetosis: Slow writhing twisting movements of the periphery, mostly fingers and wrists Hemiballismus: Violent flinging movements of one-half of the body

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

How to identify: Look for these abnormal movements during the course of examination. For tremors, ask the child to stretch out his hands and observe for tremors. In younger children, tremors can be observed when the child reaches for objects. The twisting movements and abnormal postures of dystonia are best observed in an awake child when the child is attempting movements of a body part. 14. Interpretation A. Neuromotor examination* 0: No neuromotor dysfunction (Responses to ALL of 8-13 is “0”) 1: UMN dysfunction (At least TWO out of 9-11 is “2”) 2: LMN dysfunction (Response to 8 is “1”, AND response to 9 or 10 is 1 AND 11 is not 2 3: Incoordination/ Abnormal movements (Response to 12 OR 13 is “1”) 9: Indeterminate (If the findings are abnormal but not fitting in any of the above) B. Onset of symptoms (Only for UMN dysfunction) 0: At or before 2 years of age 1: After 2 years of age or cannot be ascertained 8: Not applicable C. Course of the child’s illness** 0: Static or improving 1: Progressive 8: Not applicable D. Is there a clear Spinal cord pathology resulting in impairment Description: This question helps you to identify whether the child‟s motor problem is progressive i.e. gradually increasing or static/improving. Static/improving motor problems due to UMN dysfunction are a feature of cerebral palsy. Progressive course indicates NMD or Other NMI. One important thing you should remember is that in children with cerebral palsy, there may be apparent worsening of function due to increasing deformities. This is commonly seen when the child is not receiving any form of rehabilitation/ physiotherapy. You should NOT interpret this as progressive.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

How to ask: Ask the parents whether the child‟s motor problems are improving over a period of time or worsening or have remained static. If response to 14 A is „0‟ i.e. no neuromotor dysfunction, then responses to 14 B & C should be written as “8” i.e. Not applicable

Examine the spine for presence of any stigmata of neural tube defect e.g., tuft of hair, pits, or lump in the lumbosacral region

15. Diagnosis 0: No Neuromotor impairment (Neuro motor examination not indicated i.e. in Section 1 all questions 1 to 4 have all reponses 0 and Section 2 questions 5 to 7 have all responses is 0 OR When neuromotor examination indicated : Responses to ALL of 8-13 are “0”) 1: Cerebral palsy (Response to 14 A is 1 and/or 3 AND B, C, D is “0”) 2: Neuromuscular disorder (Response to 14A is “2”) 3: Other Neuromotor impairment (Response to 14A is NOT “0”, but not fulfilling criteria for CP or NMD) 9: Indeterminate (Response to 14 A is 9) Findings

Interpretation

No Neuromotor dysfunction UMN dysfunction Probable time of CNS insult is ≤ 2 years Static/improving course

No Neuromotor impairment Cerebral palsy

Mark as 0 1

LMN dysfunction Neuromuscular disorder UMN dysfunction Other Neuromotor impairment Progressive UMN dysfunction Other Neuromotor impairment Probable time of CNS insult > 2 years, Spinal UMN dysfunction

2 3

If it is difficult to comment whether the Indeterminate motor problems are of neurological origin or skeletal and soft tissue origin

9

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3

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

5.

CASE VIGNETTES

Case Vignette 1: Understanding the definition of ‘Cerebral Palsy’ Simran, a 3 year old girl is brought to the OPD with the complaint of delay in attaining developmental milestones. She attained neck holding at the age of 10 months, sitting without support at 18 months and walking unassisted at 2 ½ years of age. She can not feed by herself properly and spills the food. She has age appropriate social-adaptive development and speaks 2 word sentences like “give water” etc. Simran was born to a primigravida mother at 33 weeks gestation and developed severe jaundice on day 4 of life, requiring phototherapy for about 72 hours. She never had seizures. There are no similar problems in the family members. Q. What were the “early pointers” to the possibility of cerebral palsy in Simran? A. Early pointers to CP in Simran were: History of risk factor in the neonatal period (severe jaundice), delayed attainment of gross motor milestones, and feeding problems.

Q. What conclusions can be drawn from history regarding onset of illness and subsequent course? A. Onset of illness was probably in neonatal period (neonatal hyperbilirubinemia). The child is gradually attaining milestones, although with delay. With a history of delay in motor milestones and a suggestive clinical course in a child less than 2 years of age, the likelihood of the child having CP is high. On examination, Simran has spasticity and hyperreflexia involving all 4 limbs, with extensor plantar response. A diagnosis of „cerebral palsy‟ was made and she was referred to the multidisciplinary team for further management. Case Vignette 2: Diagnosis of Cerebral Palsy using CCC (Clinical Consensus Criteria) Pawan, a 4 ½ year old boy, is brought with complaints of not being able to walk without support. He can walk few steps with support. He always keeps his right fist closed. His mother also says that, his right arm and right leg feel stiffer than left. Pawan was born to 2nd gravida mother, with uneventful perinatal period. His speech and language milestones were appropriate to his age. According to Pawan‟s mother, he is late in attaining motor milestones, but has not lost any milestones. He also had seizures, which are now controlled with oral Phenytoin. There is no similar illness in any family member. Q. What is the diagnosis? Justify your answer? A. Pawan probably has „cerebral palsy‟. The reasons are: he has postural abnormalities, which is non-progressive and onset is before 2 years of age. Q. What further assessment would you like to do? A. I would like to examine Pawan to look for posture, tone, deep tendon reflexes and plantar response.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Case vignette 2 continued… On examination: Pawan had right hemiparetic gait, spasticity in right upper & lower limbs with brisk reflexes and extensor plantar response on right side. Non-contrast CT scan of head revealed cerebral atrophy on the left side. Q. Is CT scan necessary for making diagnosis of CP? Give reasons. A. Pawan has abnormalities of posture & tone, which is non-progressive and cerebral in origin with onset before 2 years of age. Hence, the diagnosis of CP can be made using CCC. CT scan of head is not required for making diagnosis of CP. However, CT scan may help in further management once diagnosis is made, and perhaps clinico-pathologic correlation. Case Vignette 3: Identifying common Neuromuscular Disorders Salman, an 8 year old boy, is brought by his mother to the children‟s OPD with the complaints of difficulty in walking. He was apparently normal till 6 years of age and started going to school also. His difficulty in walking is gradually worsening and at present, he is unable to walk without assistance. Other than this, he has no other problems. He is average in studies. On further probing, Salman‟s mother recollects that, one of her four brothers (Salman‟s maternal uncle) had similar problem at the age of 7 years and he died at 14 years of age. Q. What are the diagnostic possibilities? A. Since Salman has illness which is progressive and has positive family history, neuromuscular disorders must be considered in the differential diagnosis. These may include muscular dystrophies, neurodegenerative disorders, hereditary neuropathies and myopathies. On examination, Salman has enlarged calf and shoulder muscles. Tone is normal, ankle jerks are normally elicitable, but knee jerk and other deep tendon reflexes are absent. Q. What is the diagnosis? A. Neuromuscular disorder, probably Duchenne Muscular Dystrophy. Case Vignette 4: Understanding Other Neuromotor Impairments Pooja, a 5 year old girl, is brought to the hospital with complaint of difficulty in walking, which was noticed 18 months back and gradually increasing since then. It is associated with toe-walking. There is no difficulty in hand movements or hand function. Her perinatal history, development and family history are unremarkable. There is no history of trauma or fever. Case vignette 4 continued… Q. What are the diagnostic possibilities? A. Neuromuscular disorders (muscular dystrophy, hereditary motor sensory neuropathy), neural tube defects, stroke with sequelae

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Examination reveals spasticity, brisk deep tendon reflexes in lower limbs and extensor plantar response. Tone and reflexes in the upper limbs are normal. Examination of the back revealed a tuft of hair at lower back in midline. MRI of the spine demonstrated the presence of tethering of the spinal cord at that level. Q. What is the final diagnosis? Give the reasons. A. Neural tube defect with sequelae in the form of spastic paraparesis. Spasticity, exaggerated deep tendon reflexes and extensor plantar response are suggestive of upper motor neuron type of weakness. Signs of UMN dysfunction in the presence of a midline defect in the back suggest a diagnosis of neural tube defect.

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Annexure – I INCLEN Diagnostic Tool for Neuro-motor Impairment (INDT-NMI) for Primary Care Physician: Development and Validation

Section I: Triage questions for Neuromotor Impairments 0: No

1: Yes

8: Not applicable

1. Does your child have difficulty in ANY of the following? A. Sitting B. Getting up from floor C. Standing D. Walking E. Running 2. Did your child start performing the following activities later than children of his/her age? A. Started sitting without support beyond his/her first birthday B. Started walking without support beyond his/her second birthday

3. Does your child have ANY of the following? A. Excessive tightness/ limpness of the body B. Toe-walking C. Abnormal posture of any limb D. Decreased/ unequal use of any limb E. Frequent falls

4. Does your child have difficulty in performing ANY of the following activities? (For children above 4 years age only) A. Bathing/cleaning himself/herself B. Toileting C. Dressing D. Feeding self

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Section II: Observations: Write responses code in the box. 0: No

1: Yes

5. Observe for the following when the child is walking A. Limping B. Unsteadiness of gait (Ataxia) C. Toe walking D. Waddling gait E. Scissoring gait F. High stepping gait G. Unable to walk H. Any other gait abnormality (please specify) _______________________

6. Observe for the following when the child is standing up from floor A. Requires assistance for standing up from floor / unable to stand B. Gowers‟ sign positive C. Any other abnormality........

7. Observe hands and look for the following A. Tremors B. Unequal power of hand grip C. Fisting of one or both hands Please specify Reason for Non-applicability of any item:

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Section III: Record findings of detailed neurological assessment as codes in the respective boxes 8. Muscle power 0: Normal

1: Decreased

A. Right upper limb (Shoulder abductors & wrist extensors) B. Right lower limb (Hip abductors & ankle dorsiflexors) C. Left upper limb (Shoulder abductors & wrist extensors) D. Left lower limb (Hip abductors & ankle dorsiflexors) Overall impression: If abnormal, write the power of the abnormal side 0: Normal muscle power

1: Decreased muscle power

9. Muscle tone 0: Normal

1: Hypotonia

2: Hypertonia

A. Tone in right upper limb (Elbow & Wrist) B. Tone in right lower limb (Hip adductors, knee & ankle) C. Tone in left upper limb (Elbow & Wrist) D. Tone in left lower limb (Hip adductors, knee & ankle) Overall impression: If abnormal, write the tone of the abnormal side 0: Normal muscle tone

1: Hypotonia

2: Hypertonia

10. Deep tendon reflexes (biceps, triceps, knee and ankle jerks) 0: Normal

1: Diminished or absent

2: Exaggerated

A. Right biceps jerk B. Left biceps jerk C. Right triceps jerk D. Left triceps jerk E. Right knee jerk F. Left knee jerk G. Right ankle jerk H. Left ankle jerk

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

Overall impression: If abnormal, write the DTRs of the abnormal side 0: Normal DTRs

1: Diminished or absent DTRs

2: Exaggerated DTRs

11. Plantar response 0: Flexor response 2: Extensor response 3: Withdrawal / Not elicitable A. Right side B. Left side Impression: Write the plantar response of the abnormal side 12. Balance and coordination 0: Normal

1: Poor balance of trunk or limbs (Ataxia)

13. Abnormal movements 0: No abnormal movements 1: Abnormal movements 14. Interpretation A. Neuromotor examination* 0: No neuromotor dysfunction (Responses to ALL of 8-13 is “0”) 1: UMN dysfunction (At least TWO out of 9-11 is “2”) 2: LMN dysfunction (Response to 8 is “1”, AND 9 or 10 is “1” AND 11 is not “2”) 3: In coordination/ Abnormal movements (Response to 12 OR 13 is “1”) 9: Indeterminate (If the findings are abnormal but not fitting in any of the above) B. Onset of symptoms (Not for LMN dysfunction) 0: At or before 2 years of age 1: After 2 years of age or cannot be ascertained 8: Not applicable C. Course of the child’s illness** 0: Static or improving 1: Progressive 8: Not applicable D. Is there a clear spinal cord pathology resulting in impairment? 0: No

Neuromotor Impairment

1: Yes

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Neurodevelopmental Disabilities Among Children in India: An INCLEN Study

15. Diagnosis 0: No Neuromotor Impairment (Neuro motor examination not indicated i.e. in Section 1 all questions 1 to 4 have all reponses 0 and Section 2 questions 5 to 7 have all responses is 0 OR When neuromotor examination indicated : Responses to ALL of 813 are “0”) 1: Cerebral Palsy (Response to 14 A is 1 and/or 3 AND B, C, D is “0”) 2: Neuromuscular Disorder (Response to 14A is “2”) 3: Other Neuromotor Impairment (Response to 14A is NOT “0”, but not fulfilling criteria for CP or NMD) 9: Indeterminate (Response to 14 A is 9) * The motor problem should not be solely explained by skeletal/soft tissue abnormality (traumatic deformity, club foot, post burn contractures) or painful skeletal or soft tissue condition.

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