Child Neuropsychiatry: Cerebral Palsy and Brain Injuries

Motor System Organization

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS CHILD NEUROPSYCHIATRY Prof. M. Sorrentino Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoMOTOR SYSTEM

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Functional organization of Motor System

• Ideation and planning of movement
• Coordination of movement
• Execution of movement

Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoMOTOR SYSTEM

Ideation and Planning of Movement

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Functional organization of Motor System

• Ideation and planning of movement
  • Cortical association areas
    • Prefrontal cortex, premotor area, supplementary motor area, posterior parietal lobe, mirror neurons

Example of impairment: Apraxia in Alzheimer's Disease Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoMOTOR SYSTEM

Coordination of Movement

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Functional organization of Motor System

• Coordination of movement
  • Basal Ganglia
  • Cerebellum (cerebellar pathways cross the midline twice, so a cerebellar injury produces an ipsilateral deficit)

Examples of Impairments

• Basal Ganglia syndromes = e.g. Parkinson's Disease (resting tremor, hypertonia > rigidity, bradikynesia, impaired gait, posture and balance), Huntington's Disease (chorea, rigidity or dystonia, impaired gait, posture and balance)

Cerebellar or Ataxic syndromes = action tremor, increased deep tendon reflexes, dysmetria (finger-to-nose test), clumsiness, gait ataxia (wide based gait, back, forth and sideways oscillations). Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoMOTOR SYSTEM

Execution of Movement

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Functional organization of Motor System

• Execution of movement
  • Cortico-spinal or pyramidal pathways It is composed by:
    • First or upper motor neuron in the primary motor cortex
    • Second or lower motor neuron in the brainstem (for the cranial nervese) or in the anterior horn of spinal cord (for the spinal nerves)

Pyramidal pathways cross the anatomical midline at the junction between the brainstem and spinal cord, forming the pyramidal decussation (so a brain injury in the upper motor neuron of corticalspinal traits produces a controlateral deficit). Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoMOTOR SYSTEM

Motor Cortex and Spinal Cord Pathways

TY OF HEA EALTH SCIENCES INT In Lumine Tuo Videbimus Lumen SAINT CAMILLUS

Motor cortex Internal capsule 13 Toes Brow Eyelid Nares Lips Tongue Larynx Lateral aspect of cerebral cortex to show topographic projection of motor centers on precentral gyrus Midbrain Basis pedunculi Pons Basis pontis Medulla Pyramids Medulla Decussation of pyramids Above mid- thoracic level Motor endplate Spinal cord Below mid- thoracic level Anterior corticospinal tract Lateral corticospinal tract € Motor endplate JOHN A.CRAIG_AD CIEN Jones HR, Burns TM, Aminoff, MJ Scott, Pomeroy SL. The Netter Collection of Medical Illustrations: Nervous System - Part I. 2nd Edition. Philadephia: Elsevier Saunders; 2013. Motor system Fibers originate in motor cortex and descend via posterior limb of internal capsule to basis pedunculi of midbrain Longitudinal bundles branch upon entering basis pontis and rejoin to enter pyramids of medulla At lower medulla, bulk of fibers cross median plane to form lateral corticospinal tract; some fibers continue downward in ipsilateral lateral corticospinal tract; others descending ipsilateral anterior corticospinal tract Synapse occurs at spinal level: Lateral corticospinal fibers synapse on ipsilateral anterior horn cells; anterior corticospinal fibers synapse on contralateral anterior horn cells Hip Trunk Knee Ankle Shoulder Elbow Wrist Fingers Thumb NeckMOTOR SYSTEM

Cortico-Spinal Syndromes

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Functional organization of Motor System

• Execution of movement
  • Cortico-spinal or pyramidal pathways We distinguish two different cortico-spinal syndromes:
    • First or Upper Motor Neuron syndrome Increased tone (hypertonia > spasticity; flexor hypertonia in upper limb; extensor hypertonia in lower limbs), weakness, very little muscle wasting, brisk deep tendon reflexes (hyper-reflexia), Babinski sign. Examples: Spastic Cerebral Palsy, Post-stroke Palsy.
    • Second or Lower Motor Neuron syndrome Reduced tone (hypotonia > flaccidity), significant weakness, muscle wasting (atrophy), reduced deep tendon reflexes (hyporeflexia). Examples: Spinal Muscular Atrophy (SMA), Amyotrophic Lateral Sclerosis (ALS).

Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoMOTOR SYSTEM

Clinical Spectrum of Motor Neuron Involvement

CLINICAL SPECTRUM OF UPPER AND LOWER MOTOR NEURON INVOLVEMENT PLS HSP Upper motor neuron signs Increased tone (spasticity) Motor cortex Weakness Internal capsule Brainstem Sporadic Hereditary Upper Motor Neuron -Spinal cord Brisk reflexes (hyper- reflexia) Very little muscle wasting Primitive reflexes (Babinski) ALS FALS Interneuron Lower motor neuron signs Muscle wasting (atrophy) Anterior horn cell Weakness Peripheral nerve- Sporadic Hereditary Lower Motor Neuron Motor end plate Hyporeflexia Fasciculations (low threshold for irritation of the motor neuron) PMA SMA $ Davanzo Jones HR, Burns TM, Aminoff, MJ Scott, Pomeroy SL. The Netter Collection of Medical Illustrations: Nervous System - Part I. 2nd Edition. Philadephia: Elsevier Saunders; 2013. TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Reduced or normal tone (flaccidity)CEREBRAL PALSY

Cerebral Palsy Definition

TY OF HEA EALTH SCIENCES INT In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Definition

• "Cerebral palsy (CP) describes a group of disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behaviour, and/or by a seizure disorder." (Bax, Goldstein, Rosenbaum et al. 2005)

Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoCEREBRAL PALSY

Critical Aspects of Cerebral Palsy

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Definition: : Cerebral palsy Critical aspects:

• The lesion does not only and always affect the cerebrum, but it can also affect and impair other structures (cerebellum, brainstem, etc.) > Child Encephalic Kinesiophaty
• Every injury in the motor system during dvelopmental period is an injury of the whole system, involving all the levels of motor organization (ideation and planning, coordination, execution) even if it occurs only in a limited part of it.
• The notion of alteration in development is essential to the CP concept. It distinguishes CP from phenotypically similar disorders in children or adults due to late-acquired lesions. The "developmental" aspect of CP is also important with regard to management strategies and developmental trajectories.

Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoCEREBRAL PALSY

Developmental Deadlines in Cerebral Palsy

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Definition: : Cerebral palsy Critical aspects:

• During the construction of functions, precise development deadlines or windows of opportunity can be recognized. Within these deadlines the child has to become aware of his needs and of the rules related to the mechanisms and processes needed to comply with them. "Functional deadlines are dates within which different developmental competences, that are individual, neuromotor, cognitive, emotional, environmental, technical, family-related and social, must come together to develop those functions which are critical for the development, for example walking. The lack of even one of this requisites at its appointment deadline can be sufficient to block a motor competence that otherwise would be potentially ready" (Papini and Allori, 1999).

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Environmental Influence on CNS Development

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Definition: : Cerebral palsy Critical aspects:

• The influence of the environment on the CNS during extrauterine life has been well documented for a long time. Nervous system functions, especially adaptive ones, although produced by genetically programmed structures, need contact with the environment in order to develop and stabilize (epigenesis according to Changeaux, 1983). In the case of the CNS lesion, to accomplish potentially "undamaged" functions and to "recover" the affected ones (plasticity) this epigenetic process becomes even more relevant.

Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoCEREBRAL PALSY

Genetic Characteristics and Function Activation

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Definition: : Cerebral palsy Critical aspects:

• The genetic characteristics of the structure will not be available forever to meet those of the environment in order to fix a certain function; but, as shown by Cowan (1973), CNS development is also made up of processes for the removal and the re-attribution to different goals of what was potentially available but had not been used. Quite simply, the function allows the CNS to save the structure only if it is activated within a determined period of time (date or critical period, meant as time of structure "fertility").

Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzatoCEREBRAL PALSY

Rehabilitation and Chronological Acquisitions

TY OF HEA EALTH SCIENCES INI In Lumine Tuo Videbimus Lumen SAINT CAMILLUS Definition: : Cerebral palsy Critical aspects:

• In rehabilitation, it must be clearly understood that some functions can be proposed to the child only within specific periods of time (in time) and that development is not just a sequence of chronological acquisitions, regardless of why (needs and desires), when (importance of the experience), and how (influence of models and environment).

Prof. Michele Sorrentino Tutti i diritti sono riservati - È vietata la riproduzione e/o l'utilizzo non autorizzato