Neuromuscular weakness or paralysis in children 2021

Neuromuscular weakness or paralysis in children

1. Neuromuscular
Weakness or Paralysis
in Children
Types, Clinical Presentation, Diagnosis,
Assessment, Management
Prof. Imran Iqbal
Fellowship in Pediatric Neurology (Australia)
Prof of Paediatrics(2003-2018)
Prof of Pediatrics Emeritus, CHICH
Prof of Pediatrics, CIMS
Multan, Pakistan

2. (God Almighty speaking to Prophet Muhammad (PBUH)
The Rahman (The All-Merciful Allah) has taught the Qur‘an.
He has created man. He has taught him (how) to express himself.
The Holy Quran; surah Al-Rahman 55:1-4
In the name of Our Creator Allah, the most Gracious, the most Merciful

3. CNS

4. Brain

5. Our perceptions and Movements

6. Evaluation
of the Child with
Neurological Symptoms

7. CNS –Abnormal Symptoms
•Convulsions
•Altered Consciousness
•Delayed development
•Intellectual handicap
•Speech problem
•Motor weakness / Walking problem
•Sensory changes
•Headache
•Unable to see / listen

8. History –Ask about Symptoms
•Neurological Symptoms –
•Onset
•Frequency
•Severity
•Progression
•Birth history
•Development history
•Family history

9. CNS –Abnormal Signs
•Developmental milestones (in relation to age)
•Impaired Conscious level (GCS)
•Convulsions
•Intellectual impairment (retardation / handicap)
•Motor weakness / Paralysis / Paresis
•Abnormal gait
•Abnormal movements
•Visual impairment
•Auditory impairment

10. CNS Examination
in Children

11. Examination of CNS in Children
•OBSERVE –observe the child
•General Physical Examination
•Developmental Examination
•NEUROLOGICAL EXAMINATION –
•Higher Mental Functions
•Cranial Nerves
•Motor System
•Sensory system
•Skull and Spine
•SOMI

12. Neurological Examination

13. NEUROLOGICAL EXAMINATION
•Higher Mental Functions
•Cranial Nerves
•Motor System
•Sensory system
•Skull and Spine
•SOMI

14. Higher Mental Functions
•Conscious Level (Glasgow Coma Scale)
•Recognition and Response
•Behavior and activity
•Speech
•Memory

15. Cranial Nerves
•Visual focusing and following
•Light reflex
•Facial Movements
•Response to sound
•Sucking and Swallowing
•Palate movement and Gag reflex

16. Motor System
•Presence of spontaneous voluntary movements in
infant
•Size and Nutrition of muscles
•Tone
•Power
•Deep Tendon Reflexes
•Planter Reflex
•Gait
•Co-ordination
•Involuntary Movements

17. Sensory System
•Touch
•Pain
•Temperature
•Position
•Vibration

18. Skull & Spine
•Shape of skull
•Anterior fontanel
•Occipito-frontal Circumference (OFC)
•Spine –Curvature, local swelling

19. SOMI –Signs of Meningeal Irritation
•Bulging Anterior fontanel
•Neck rigidity
•Kernig'ssign
•Brudzinski'ssign

20. Clinical Presentation
of
CNS diseases

21. CNS disease –Clinical Presentation
•Seizures / Convulsions / Fits
•Impaired Conscious level
•Developmental delay
•Intellectual handicap
•Neurological Paralysis / Paresis
•Neuromuscular motor weakness
•Movement disorders
•Headache
•Regression of acquired milestones
•Behaviour problems

22. Neuromuscular Weakness or Paralysis
Types, Clinical Presentation, Diagnosis,
Assessment, Management

23. Neuromuscular Weakness or Paralysis
Clinical Evaluation
•What is the Pathophysiology ?
•Upper motor neuron disease ?
•Lower motor neuron disease ?
•Where is the lesion ?
•Brain ?
•Spinal Cord ?
•Peripheral nerves ?
•Muscles ?
•What is the disease ?

24. Motor System Weakness or Paralysis
UMN and LMN lesions –Physiology
Upper Motor Neuron
•Motor cortex to Anterior
Horn Cell
•Neurons in Corticospinal
tracts
•Dysfunction causes Spastic
paresis / paralysis
•Muscle tone and DTR
increased
•Planter response is
extensor (up-going)
Lower Motor Neuron
•Anterior Horn Cell to
Neuro-muscular Junction
•Neurons in Peripheral
nerves
•Dysfunction causes Flaccid
paresis / paralysis
•Muscle tone and DTR
decreased
•Planter response is flexor
(down-going)

25. Motor System Weakness or Paralysis
UMN and LMN lesions –Site and Etiology
Upper Motor Neuron
Brain and Spinal Cord
Cortico–Spinal disorders
Lesions in:
•Cerebral Cortex
•Internal capsule
•Brain stem
•Spinal Cord
Lower Motor Neuron
Peripheral Nerve and Muscle
Neuro –Muscular disorders
•Diseases of:
•Anterior Horn Cell
•Peripheral nerves
•Neuromuscular Junction
•Muscle fibers

26. Neuromuscular Weakness or Paralysis
Etiology and Clinical Presentation
Etiology
•Cortico–spinal diseases
•Neuro –muscular diseases
Clinical Presentation
•Hemiparesis / Hemiplegia
•Para-paresis / Paraplegia
•Quadri-paresis / Quadriplegia

27. Neuromuscular Weakness or Paralysis
Differential Diagnosis
Not a Neurological Disease ?
•Pseudo -paralysis –(due to pain)
•Etiology -Arthritis, Osteomyelitis, Unrecognized trauma
•Examination -Antalgic gait, CNS Examination normal,
•Diagnosis -Complete Physical Examination of limbs and
joints
•Conversion Reaction / Malingering (Psychological)
•Etiology -Psychological motives
•Examination -Child unwilling to walk unsupported and falls
in different positions
•Diagnosis –Motor system examination is normal

28. Cortico–Spinal disorders
Upper Motor Neuron diseases

29. Neuromuscular Weakness or Paralysis
Hemiplegia / Paraplegia / Quadriplegia
Cortico-spinal diseases –Etiology
•Acute brain damage (meningitis, encephalitis, CVA)
•Cerebral Palsy
•Space occupying lesions (SOL)
•Degenerative Brain disease (grey matter, white matter)
•Spinal cord lesions (trauma, transverse myelitis, TB spine)

30. Cranio–Spinal disorders
Upper Motor Neuron diseases
Clinical Presentations
Acute onset of Muscular Weakness / Paralysis
•Acute Hemiplegia (Brain)
•Acute Paraplegia (Spinal cord)
•Acute Quadriplegia (Brain / Spinal cord)
•Gradual onset of Muscular Weakness / Paralysis
•Hemipresis(Brain)
•Parapresis(Spinal cord)
•Quadriparesis(Brain/Spinal cord)

31. Hemiplegia in Children
Upper Motor Neuron Lesions

32. Hemiplegia in Children –UMN
Etiology
Acute onset
•Cerebral Thrombosis
•Cerebral Embolism
•Intracranial hemorrhage
•Meningitis / Encephalitis
•Epilepsy
Gradual onset
•Cerebral abscess
•SOL (Space Occupying Lesions) Brain

33. Hemiplegia in Children –UMN
Etiology
Acute onset
•Cerebral Thrombosis (Carotid arteritis, dehydration, SCA)
•Cerebral Embolism (Infective endocarditis)
•Intracranial hemorrhage (Hemorrhagic disorders, trauma)
•Meningitis / Encephalitis (Pyogenic, Tuberculous, Viral)
•Post-epileptic Paralysis (after seizures)
Gradual onset
•Cerebral abscess (Cyanotic Congenital Heart Disease)
•SOL (Space Occupying Lesions) –brain tumors

34. Hemiplegia in Children –UMN
Clinical Presentation
•Cranial shock –Initial hypotonia of paralyzed muscles in
acute onset hemiplegia
•Clinical picture –Spasticity, brisk DTR, Babinski’s sign
•Arm is more affected than the leg
•Homonymous hemianopia (visual field defect may be
present)
•Dysphasia in lesions of the dominant hemisphere

35. Hemiplegia in Children –UMN
Investigations
•CBC, CRP, Coagulation profile, Hemoglobin electrophoresis
•CT / MRI Brain
•Echocardiography
•CT angiography / MR angiography Brain
•CSF Examination

36. Hemiplegia in Children –UMN
Management
•Blood component therapy –Hemorrhage
•Anti-platelet drugs –Thrombosis, Infarction
•Antibiotics –Meningitis
•Neuro-surgery –Hemorrhage, SOL, Brain Abscess
•Anti-epileptic Drugs –Seizures
•Physiotherapy –Passive and Active movements
•Skeletal Muscle Relaxants –Baclofen

37. Paraplegia / Quadriplegia
in Children
Upper Motor Neuron Lesions

38. Paraplegia / Quadriplegia –UMN
Etiology
Acute onsetParaplegia / Quadriplegia (Spinal cord lesions)
•Acute Myelitis / Transverse Myelitis
•Trauma
Gradual onset Paraplegia / Quadriplegia (Brain / Spinal cord lesions)
•Cerebral palsy
•Brain White matter degenerations / demyelinating disorders are
rare causes (associated with cognitive changes and seizures)
•Meningo-myelocele
•Tuberculosis of spine
•Spinal tumors

39. Spinal Paraplegia / Quadriplegia –UMN
Spinal Cord –Site of Lesion
•Acute Paraplegia / Quadriplegia is almost always caused by
lesions in the spinal cord (not in the Brain)
•Quadriparesis/ Quadriplegia is produced by Spinal Lesions
above the cervical region in spinal cord
•Paraparesis/ Paraplegia is produced by Spinal Lesions below
the cervical regionin spinal cord

40. Spinal Paraplegia / Quadriplegia –UMN
Clinical Presentation
•Spinal shock –Initial hypotonia of paralyzed muscles in
acute onset Paraplegia / Quadriplegia (for days to weeks)
•Clinical picture –Spasticity, brisk DTR, Babinski’s sign
•Loss of sensations with an upper spinal level corresponding
to motor deficitin spinal cord lesions
•Visceral presentation (retention of urine and stools) in spinal
cord lesions

41. Paraplegia / Quadriplegia –UMN
Investigations
•CBC, CRP, Coagulation profile,
•MRI Spine (cervical, dorsal, lumber, sacral)
•CT / MRI Brain
•Other investigations as needed

42. Paraplegia / Quadriplegia –UMN
Management
•Corticosteroids –Acute Myelitis / Transverse Myelitis
•Antibiotics –epidural abscess, vertebral osteomyelitis
•Anti-tuberculous drugs –Spinal tuberculosis
•Neuro-surgery –SOL, Abscess
•Physiotherapy –Passive and Active movements
•Skeletal Muscle Relaxants –Baclofen

43. Neuromuscular disorders
LowerMotor Neuron Diseases

44. Neuro –Muscular disorders
Lower Motor Neuron / Muscle diseases
Etiology
Anterior Horn Cells
•Infantile spinal muscular atrophy
(Werdnig-Hoffman disease)
•Poliomyelitis
Peripheral Nerves
•Acute Demyelinating Polyneuropathy (Guillain–Barre syndrome)
•Peripheral Neuropathy
•Traumatic neuritis
Neuro-muscular Junction
•Myasthenia Gravis
Skeletal Muscles
•Myopathy
•Muscular dystrophy

45. Neuro –Muscular disorders
Lower Motor Neuron / Muscle diseases
Clinical Presentations
Acute onset of Muscular Weakness / Paralysis
•Acute Monoplegia (Poliomyelitis, Plexopathy(Erb’spalsy),
Traumatic neuritis)
•Acute Paraplegia (GBS, Acute myositis)
•Acute Quadriplegia (GBS)
Slow onset of Muscular Weakness / Paralysis
•Quadriparesis-Infantile spinal muscular atrophy, Congenital
myopathies, Muscular dystrophy, Dermatomyositis
•Paraparesis-HMSN, Duchene muscular dystrophy

46. Neuromuscular Weakness or Paralysis
Neuromuscular disorders
Presentation and Etiology
Neuromuscular Weakness in Infancy / Childhood
Presentation –Acute Flaccid Paralysis
•GBS (acute demyelinating polyneuropathy)
(Quadriparesis)
•Poliomyelitis (Monoparesis)
•Traumatic neuritis (Monoparesis)
•Transverse myelitis (spinal cord lesion)

47. Neuromuscular Weakness or Paralysis
Neuromuscular disorders
Presentation and Etiology
Neuromuscular Quadriparesisin Infancy
Presentation –Hypotonia (Floppy infant)
•Infantile spinal muscular atrophy
(Werdnig-Hoffman disease)
•Myopathy
•Congenital Muscular dystrophy

48. Poliomyelitis
Anterior Horn Cell Disease
Viral Infection
•Etiology –Polio virus infection
of the anterior horn cells
•Pathogenesis –Paralysis of muscle fibers supplied by the
affected neurons
•Clinical Features –
Fever,
Monoplegia or Paraplegia,
Hypotonia,
Deep Tendon reflexes absent
•Diagnosis –Polio virus culture (stool specimen)

49. Poliomyelitis
Anterior Horn Cell Disease
Viral Infection
•Management –
Rest,
Positioning of limbs,
Analgesics,
•Rehabilitation of Residual Weakness –
Physiotherapy (active and passive exercizes),
Splints (prevent deformity and contractures),
Braces (walking support),
Surgery (tendon lengthening and transplant)
•Prevention –Vaccination

50. Guillain–Barre Syndrome
Acute Demyelinating Polyneuropathy
Post –infection auto-immune inflammation
•Etiology –Auto-immune inflammation of peripheral nerves
•Pathogenesis –Demyelination of motor nerve fibers
•Clinical Features –
Para-paresis, Quadri-paresis,
Ascending paralysis of lower to upper limbs
Muscle tone and Power decreased, DTR absent,
•Complications –Respiratory muscle paralysis, Respiratory
failure, Residual weakness
•Diagnosis –NCS (Nerve Conduction Studies),

51. Guillain–Barre Syndrome
Acute Demyelinating Polyneuropathy
Post –infection auto-immune inflammation
•Diagnosis –NCS (Nerve Conduction Studies),
•Management –
IVIG,
Plasmapheresis,
Artificial Ventilation
•Rehabilitation of Residual Weakness –
Physiotherapy (active and passive exercises),
Splints (prevent deformity and contractures),
Braces (walking support),
•Prognosis –Gradual recovery

52. Plexopathy
Brachial Plexus Damage
Erb’sPalsy
•Etiology –Brachial Plexus damage during birth
•Pathogenesis –damage to C5 and C6 nerve roots
•Clinical Features –Erb’spalsy,
Arm in pronated position
Failure to abduct the arm
Fingers in flexed position
•Diagnosis –
•Clinical (specific position of affected arm),
•NCS (Nerve Conduction Studies)

53. Plexopathy
Brachial Plexus Damage
Erb’sPalsy
•Management –Positioning the arm on the chest
•Rehabilitation of Residual Weakness –
Physiotherapy (active and passive exercises)
Splints (prevent deformity and contractures)
Surgery (nerve grafts, tendon transfers)
•Prevention –Training of Health Care Providers, Care during
birth

54. Traumatic Neuritis
Sciatic Nerve Damage
Post-Injection
•Etiology –a gluteal area injectiondamaging the sciatic
nerve
•Pathogenesis –damage to the sciatic nerve results in
paralysis of calf muscles (foot dorsiflexors)
•Clinical Features –Foot drop,absent Ankle Jerk
•Diagnosis –NCS (Nerve Conduction Studies)

55. Traumatic Neuritis
Sciatic Nerve Damage
Post-Injection
•Management –Positioning,
•Rehabilitation of Residual Weakness –
Physiotherapy (active and passive exercises),
Splints (prevent deformity and contractures),
Braces (walking support),
Surgery (tendon lengthening and transplant)
•Prevention –Training of Health Care Providers

56. Spinal Muscular Atrophy
Werdnig -Hoffman Disease
Autosomal Recessive familial disease
•Etiology –mutation in SMN gene on Ch. 5
•Pathogenesis –ProgressiveDegeneration of Anterior Horn
Cells
•Clinical Features –
increasing skeletal muscular weakness in infancy,
quadri-paresis,internal rotation of arms,
hypotonia, DTR absent,
fasciculation visible on tongue,
intercostal recessions, respiratory failure,
cognitive functions intact

57. Spinal Muscular Atrophy
Werdnig -Hoffman Disease
Autosomal Recessive familial disease
•Diagnosis –
NCS (Nerve Conduction Studies),
PCR for genetic diagnosis
•Management –
Physiotherapy,
Chest physiotherapy
•Prevention –
Genetic counselling,
Prenatal Diagnosis

58. Myopathies
Autosomal Recessive familial diseases
Congenital muscular weakness
•Etiology –Genetic abnormalities (single gene)
•Pathogenesis –Muscle fiber abnormalities
•Types –
•central core, nemaline, mitochondrial myopathy
•Clinical Features –
oquadri-paresis,
osevere skeletal muscular weakness in infancy,
ohypotonia DTR absent,
odelayed motor development

59. Myopathies
Autosomal Recessive familial diseases
Congenital muscular weakness
•Diagnosis –
oCPK normal,
oMuscle biopsy,
oGenetic diagnosis
•Management –
oPhysiotherapy,
oChest physiotherapy
•Prevention –
oGenetic counselling,
oPrenatal Diagnosis

60. Duchene Muscular Dystrophy
Abnormal dystrophin protein
Progressive muscle weakness
•Etiology –Genetic (X –linked recessive), abnormal
Dystrophin gene
•Pathogenesis –muscle degeneration due to abnormal
dystrophin muscle protein,
fat deposition results in pseudo-hypertrophy
•Clinical Features –onset by 3 to 8 years
o--delayed motor development
o--skeletal muscle weakness, hypotonia
o--pseudo –hypertrophy of calf muscles
o--Toe –walking, Waddling gait
o--Gower sign positive

61. Duchene Muscular Dystrophy
Abnormal dystrophin protein
Progressive muscle weakness
•Complications –
oinability to walk,
ocardiomyopathy,
orespiratory failure
•Diagnosis –
oCPK very high,
oGenetic diagnosis
•Management –Physiotherapy,Rehabilitation, Wheel chairs
•Prognosis –loss of ambulation by 10 years of age
•Prevention –Genetic counselling, Prenatal Diagnosis

62. Prof Imran Iqbal
Fellowship Pediatric Neurology-1991
Royal Children Hospital, Melbourne, Australia