Approach to evaluation of a child with upper motor neuron disorder

EVALUATION OF A CHILD WITH
UPPER MOTOR NEURON DISORDER
BY DR ALEYA REMTULLAH
APPROACH
• Signs of UMND from LMND
• UMND- in brain or spinal cord
• In brain- parts of brain and
lesions/pathologies
• Spinal cord and its lesions and pathologies
TABLE OF CONTENTS
1. CORTICAL LESIONS (grey and white matter)
• Frontal
• Parietal
• Temporal
• Occipital
2. BASAL GANGLIA- movement disorders
• Hyperkinetic
• Hypokinetic
TABLE OF CONTENTS
3. BRAINSTEM LESIONS
• Demyelinating
• Neoplastic
4. SPINAL CORD LESIONS
5. CEREBELLAR DISORDERS- ataxia
UMND Vs LMND
CEREBRAL CORTEX
ANATOMY
• Cerebral cortex consists of;
Gyri
Sulci
Lobes
• Frontal lobe
Primary motor and pre motor cortex
Broca’s area
Frontal
Pre frontal
ANATOMY
• Parietal lobe;
Primary somatosensory
Wernicke’s area
• Temporal lobe
Primary auditory cortex
• Occipital lobe
Visual and visual association cortex
PATHOLOGIES
1. WHITE MATTER
2. GREY MATTER
• DEVELOPMENTAL DELAY
• DEVELOPMENTAL REGRESSION
• GLOBAL DELAY
WHITE MATTER PATHOLOGIES
• Rare group of progressive, metabolic, genetic
diseases that affect brain, spinal cord and
peripheral nerves.
• Genetic mutations which lead to destruction
of myelin sheaths in white matter of brain
• Each type of LD affects a different part of the
myelin sheath- range of neurological problems
WHITE MATTER PATHOLOGIES
• SIGNS AND SYMPTOMS
• Some begin shortly after birth and others develop later on in life
• Abnormal muscle movement
• Behavioural problems
• Decreased mental function
• Decreased muscle tone
• Difficulty in walking
• Difficulty in eating or feeding
• Frequent falls
• Spasticity
• Peripheral numbness
• Seizures
• Developmental delay
GREY MATTER
• Paroxysmal events
a) Epileptic
b) Non-epileptic
EPILEPTIC TYPE
• SEIZURE- a transient occurrence of signs/
symptoms due to abnormal excessive or
simultaneous neuronal activity within the
brain
• EPILEPSY- is a disease characterized an
enduring predisposition to generate epileptic
seizures and by the neurobiological, cognitive,
psychological and social consequences of this
condition
TYPES OF EPILEPSY
a) Focal epilepsy
 Simple partial
 Complex partial
a) Generalized epilepsy
Tonic clonic- above 1 year of age
Absence seizures
Chilhood absence epilepsy
Juvenile myoclonic epilepsy
Myoclonic seizures
Drop attacks
TYPES OF EPILEPSY
C) Epilepsy syndromes
Infantile spasms
Severe myoclonic syndrome
Lennox-Gastaut syndrome
Landau-Kleffner syndrome
NEPEs
• Events that mimic an epileptic seizure but are not
caused by abnormal electrical activity of the brain
• Types:
Reflex (pallid) anoxic seizures
Vasovagal episodes
Breath holding spells
Sleep related phenomena
Behavioural events
Psychiatric events- tics, anxiety/panic attacks
DEVELOPMENTAL DELAY
• APPROACH
History
Physical exam
Developmental assessment
Assessment for behavioural syndromes
Ddx with secondary disabilities
Approach to evaluation of a child with upper motor neuron disorder
DEVELOPMENTAL ASSESSMENT
HEARING IMPAIRMENT
• Is the most common cause of speech delay
• May occur with global developmental retardation/ retarded
children who fail to imitate sounds
• May be due to; rubella embryopathy, cytomegalic inclusion
disease, neonatal meningitis, kernicterus, and several
genetic disorders
• Requires audiometric testing
• Crude testing is inadequate
• Other clues- excessive gesturing and staring at the lips of
people who are talking
• Brain auditory evoked potentials offer good screening for
neonatal hearing deficits
INFANTILE AUTISM
• Is a syndrome which is seen in infants.
• They fail to meet few or any developmental
milestones
• Cause unknown but hypothesized as genetic with
neuropathology
• Diagnostic criteria (DSM 5)
Deficits in social communication and social
interaction
Restrictive repetitive behaviours, interests and
activities
INFANTILE AUTISM
• CAUSES:
Genetics- mostly in monozygotic twins
Environmental- prenatal, natal and post natal
PRESENTATION
INFANTILE AUTISM
• Diagnosis is clinically!!
• Screening checklists
M-CHAT
INDT-ASD
• Diagnostic tools
Autism diagnostic interview- revised
Childhood autism rating scale
APPROACH TO MX
DDX
COMPLICATIONS
• Cognition- lower IQ
• Sleep- difficulty in initiating sleep, frequent
awakening, insomnia, early morning
awakening
• Feeding- selective eaters
• Epilepsy in 8-46%
• Other psychiatric conditions
BASAL GANGLIA
• Group of subcortical nuclei in base of
forebrain
• Associated with functions like;
Control of voluntary movements, postural
control, control of muscle tone
Procedural learning
Routine behaviours e.g. eye movements, teeth
grinding, cognition and emotion
ANATOMY
HYPERKINETIC MOVEMENT
DISORDERS
• Pathophysiology
• Most movement disorders are due to defects
in functional connectivity rather than a single
lesion
• Direct and indirect pathways
POST STROKE MVT DISORDERS
TYPES OF MVT DISORDERS
1. CHOREA/BALLISM
• Due to STN inactivation and reduction in
basal ganglia output
• share common pathophysiology
• a/w BG infarction
• Presents with irregular, abrupt, rapid, and
transient movements that can affect the
entire body, and typically manifests in the
distal region of the body
TYPES OF MVT DISORDERS
• Ballism- severe form on chorea, presents with
proximal limb involvement
• May occur together post stroke
• May also present as hemiballism/ hemichorea
• Both develop immediately after a stroke
• In total, 54% of cases of chorea associated
with infarctions in the basal ganglia circuit
completely resolve; however, chorea
associated with STN lesions do not resolve.
TYPES OF MVT DISORDERS
2. DYSTONIA
• Lesions in the posterior putamen and Gpe
(lentiform nucleus)
• Sustained muscle contractions or abnormal
posture
• commonly presents in combination with
hemidystonia on the contralateral side
• May resolve completely/partially
TYPES OF MVT DISORDERS
• Onset is delayed- approx 9 months later
• If post stroke- may develop dystonia once hemiparesis
resolves
3. TICS
• Sudden, repetitive, nonrhythmic motor movement or
vocalization
• Due to infarctions of caudate and lentiform nucleus
• Presents in combination with dystonia
• Due to disruption in cortico-striato-thalamo-cortical
circuit
TYPES OF MVT DISORDERS
4. MYOCLONUS
5. ASTERIXIS
6. TREMOR
7. COMPLEX
8. RESTLESS LEG SYNDROME
9. AKATHISIA
10. ISTEREOTYPIC
11. BLEPHAROSPASM
HYPOKINETIC MVT DISORDERS
• Pathophysiology
1. PARKINSONISM
• Group of neurological disorders that cause
movement problems such as tremors, stiffness
and slow movement
• Aka atypical parkinson’s disease
• Progresses more rapidly cf parkinsons disease
• Additional sx- early falling,
dementia/hallucinations
• Do not respond to L-dopa
PARKINSONISM VS PARKINSON’S
DISEASE
METABOLIC CAUSES OF
PARKINSONISM
1) Thyroid disease
2) Renal failure
3) Calcium disorders
4) Iron metabolism
5) Liver failure
6) Electrolyte disturbance
7) Mitochondrial disease
8) Inborn errors of metabolism
HYPOKINETIC MVT DISORDERS
PARKINSONISM (vascular)
• Aka artherosclerotic parkinsonism
• Unilateral/bilateral
• lesions to the lentiform nucleus and striatum, as
well as pontine lesions and extensive subcortical
white matter lesions
• 2 types
a) After BG infarction
b) Chronic diffuse white matter degeneration
VASCULAR PARKINSONISM
• Presents symmetrically
• No tremors
• Lower body involvement
• Poor response to L-dopa
BRAIN STEM
BRAINSTEM LESIONS
1. DEMYELINATING LESIONS
• Diagnosis is clinically /histopath post mortem
• Common types- MS, ADEM, PML and
extrapontine myelinolysis
• Classification based on pathogenesis;
a) Inflammatory process
b) Acquired metabolic derangements
c) Hypoxic ischemic insults
d) Focal compression
ADEM
• Autoimmune inflammatory demyelinating
disease
• Affects white matter and spinal cord
• Commonly occurs within 3 weeks of infection,
vaccination or due to certain drugs
• Self limiting
• Monophasic disease (if more than one
relapse, consider MS)
ADEM
• PRESENTATION
Ataxia
Headache
Weakness
Slurred or impaired speech
CN palsies
Agitation, seizures
Delirium, stupor, AMS
Constitutional symptoms
ADEM
• INVESTIGATIONS
Platelets and ESR raised
CSF for WBCs, RBCs, biochemistry, protein is
elevated
CT scan brain- multiple, bilateral and
asymmetrical lesions with CNS
Margins of lesions are smudged
MRI brain- to r/o ddx
EEG- change in sleep patterns
ADEM
ADEM
• TREATMENT:
High dose i.v. corticosteroids- 20-30mg/kg/d
methylprednisolone for 3-5days
Then oral prednisolone at 2mg/kg. then taper
over 4-6 weeks
With or without; IVIG 2g/kg i.v as single dose
or over 3-5 days
MULTIPLE SCLEROSIS
• Accounts for 10% of all cases in children
• Neurologic symptoms disseminated in time and
space
• Immune mediated disease with recurrent
episodes of CNS demyelination
• Commonly in children under 18 years
• Difficult to diagnose especially in pre pubertal
children due to atypical presentation
• Cause- genetic or environmental
• Types- 4
MULTIPLE SCLEROSIS
• Clinical presentation
 Features of encephalopathy
 Optic neuritis
 Ataxia
 Fatigue
 Constipation
 Weakness
 Paraesthesia
 Cognitive impairment
 Early onset with slow progression
MULTIPLE SCLEROSIS
• Diagnosis is made clinically!!
• MRI- multiple or single T2 hyperintense lesion
and differentiate from ADEM
• McDonald criteria 2010
MULTIPLE SCLEROSIS
MS TREATMENT
• High dose corticosteroids (acute
exacerbations)
• Plasmapheresis – for those with severe
exacerbations refractory to high dose CS
• IVIGg- 400mg/kg/day for 5 days- who cant
tolerate CS
• Early initiation of DMD
MS COMPLICATIONS
• Pain
• Depression
• Bladder and bowel incontinence
• Difficulties in speech and swallowing later
• Language is intact but over time they develop
expressive language deterioration
NEUROMYELITIS OPTICA
• Autoimmune disorder where auto antibodies
acttack optic nerve and SC
• Thus present with optic neuritis and acute
transverse myelitis
• Visual loss and subsequently develop
paraplegia and sensory loss, but the order
may be reversed
• Demyelination is antibody dependent and
complement mediated
NEUROMYELITIS OPTICA
• Causes pain in the eye, visual loss
• Damage to SC causes weakenss/paralysis,
paraesthesia, incontinence
• Is a relapsing-remitting disease
• Cause is due to attack of autoantibodies to
aquaporin-4 water channel located in optic
nerve and SC
NEUROMYELITIS OPTICA
• Diagnosis
• Via modified clinical criteria by international
panel
• Absolute criteria and supportive criteria
• 2 absolute and 2 of 3 supportive criteria is
diagnostic
NEUROMYELITIS OPTICA
ABSOLUTE CRITERIA SUPPORTIVE CRITERIA
OPTIC NEURITIS MRI BRAIN NOT MEETING MS
ACUTE MYELITIS MRI SC T2 LESIONS OVER >3 VERTEBRAE
NMO-Igg POSITIVE
NEUROMYELITIS OPTICA
• Investigations-
• Autoantibodies against AQP4
• Anti AQP IgG
• MRI Brain
• MRI SC
• Mx-no cure but supportive mx
• High dose CS/ plasmapheresis
• Secondary prevention is immunosuppressive
drugs to reduce frequency and severity of
relapses
ACUTE TRANSVERSE MYELITIS
• Is inflammation of white and grey matter in one or
more adjacent SC segments
• Affects the spinal cord in its entire width (transversely)
• Usually thoracic region
• Blocks transmission of nerve impulses
• Trigger- unknown
• Maybe due to autoimmune reaction
• Risk factors- MS, neuromyelitis optica, bacterial
infections (lyme disease, syphilis or TB), SLE, viral
meningoencephalitis, drugs, vaccination,
ACUTE TRANSVERSE MYELITIS
• SYMPTOMS
Sudden pain in the back
Band like tightness around affected area
(chest/abdomen)
Tingling, numbness, muscle weakness within days
(ascending)
Urine incontinence/ urgency
Paralysis, loss of sensation
Urine retention
ACUTE TRANSVERSE MYELITIS
• INVESTIGATIONS
MRI spine- variable enlargement of SC
LP- elevated WBCs and protein
MRI brain
Others- HIV, serology for lymes disease,
copper and zinc levels, ANA, VDRL, vit B12
levels
ACUTE TRANSVERSE MYELITIS
• TREATMENT
Underlying cause (infectious, nutritional def)
If no cause identified; high dose
coritcosteroids followed by plasma exchange
(efficacy of such tx is uncertain)
NEOPLASTIC BRAIN STEM LESIONS
• Types;
a) Infratentorial lesions- contains cerebellum
b) Supratentorial lesions- contains cerebrum
INFRATENTORIAL LESIONS & DDX
SUPRATENTORIAL LESIONS & DDX
SPINAL CORD LESIONS
• Lesions affecting the spinal cord
Transverse myelitis
Infectious-
spinal abscess
Herpes myelitis
Tb spine- pott’s disease
SPINAL CORD LESIONS
• Spinal abscess can occur anywhere along spinal
axis
• Most commonly affects posterior thoracic spinal
cord
• Presentation depends on level of spinal cord
involvement and duration of infection
• Common causative org- staph and strep spp
• Sx- weakness, paraesthesia, dysesthesia, bladder
and bowel incontinence and acute paraplegia
• Chronic cases- gradual progression
SPINAL ABSCESS
• INVESTIGATIONS
LP- CSF exam
Abscess C/S
CSF microscopy to look for parasites
Gadolinium enhanced MRI (ring enhancement)
Mx- surgical drainage of abscess, c/s, appropriate
abx post op and steroids to reduce inflammation
HERPES MYELITIS
• A form of acute ascending necrotizing myelitis
• Caused by HSV-2
• Thoracic and lumbosacral are most common sites
• Rare, mostly reported in patients with Acquired
IDS and malignancy
• Ascending myelopathy
• Due to primary infection or reactivation
• Transmitted- in utero, sexually
HERPES MYELITIS
• SX- Pain, limb numbness, paraesthesia,
weakness
• A/w herpetic skin lesions
• MRI spine- enlargement of lower cord or
conus medullaris
• Contrast enhancement of adjacent nerve
roots
IMAGE
TB SPINE
• Aka pott’s disease, tuberculous spondylitis
• Caused by Mycobacterium TB
• Manifests as a combination of osteomyelitis and
arthritis
• Involves more than one vertebrae (usually
anterior aspect)
• Progressive bone destruction leads to vertebral
collapse and kyphosis
• Spinal canal gets narrowed by abscess/tissue-
spinal cord compression and neuro sx
TB SPINE
• SX- constitutional sx (wgt loss and fever)
• Duration is 4 months (average)
• Chronic back pain
• SC compression- paraplegia, paresis, impaired
sensation, nerve root pain and cauda equina
syndrome
• Cervical vertebrae- pain and stiffness
• Lower cervical- hoarseness, neuro deficits and
torticollis
TB SPINE
• Commonly affects thoracic spine
• Local pain
• Muscle rigidity and spasm
• Skin manifestations
• Cold abscess in psoas muscle
TB SPINE
• Tuberculin skin test
• ESR
• Microbio studies- bone tissue/ abscess sample for
gene expert, culture and sensitivity, microscopy
• CT guided needle biopsy of lesions
• Xray spine
• CT scan
• MRI- for disc space infection and osteomyelitis,
soft tissue involvement
Approach to evaluation of a child with upper motor neuron disorder
TB SPINE
• THANK YOU FOR LISTENING!
1 sur 79

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Approach to evaluation of a child with upper motor neuron disorder

  • 1. EVALUATION OF A CHILD WITH UPPER MOTOR NEURON DISORDER BY DR ALEYA REMTULLAH
  • 2. APPROACH • Signs of UMND from LMND • UMND- in brain or spinal cord • In brain- parts of brain and lesions/pathologies • Spinal cord and its lesions and pathologies
  • 3. TABLE OF CONTENTS 1. CORTICAL LESIONS (grey and white matter) • Frontal • Parietal • Temporal • Occipital 2. BASAL GANGLIA- movement disorders • Hyperkinetic • Hypokinetic
  • 4. TABLE OF CONTENTS 3. BRAINSTEM LESIONS • Demyelinating • Neoplastic 4. SPINAL CORD LESIONS 5. CEREBELLAR DISORDERS- ataxia
  • 7. ANATOMY • Cerebral cortex consists of; Gyri Sulci Lobes • Frontal lobe Primary motor and pre motor cortex Broca’s area Frontal Pre frontal
  • 8. ANATOMY • Parietal lobe; Primary somatosensory Wernicke’s area • Temporal lobe Primary auditory cortex • Occipital lobe Visual and visual association cortex
  • 9. PATHOLOGIES 1. WHITE MATTER 2. GREY MATTER • DEVELOPMENTAL DELAY • DEVELOPMENTAL REGRESSION • GLOBAL DELAY
  • 10. WHITE MATTER PATHOLOGIES • Rare group of progressive, metabolic, genetic diseases that affect brain, spinal cord and peripheral nerves. • Genetic mutations which lead to destruction of myelin sheaths in white matter of brain • Each type of LD affects a different part of the myelin sheath- range of neurological problems
  • 11. WHITE MATTER PATHOLOGIES • SIGNS AND SYMPTOMS • Some begin shortly after birth and others develop later on in life • Abnormal muscle movement • Behavioural problems • Decreased mental function • Decreased muscle tone • Difficulty in walking • Difficulty in eating or feeding • Frequent falls • Spasticity • Peripheral numbness • Seizures • Developmental delay
  • 12. GREY MATTER • Paroxysmal events a) Epileptic b) Non-epileptic
  • 13. EPILEPTIC TYPE • SEIZURE- a transient occurrence of signs/ symptoms due to abnormal excessive or simultaneous neuronal activity within the brain • EPILEPSY- is a disease characterized an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological and social consequences of this condition
  • 14. TYPES OF EPILEPSY a) Focal epilepsy  Simple partial  Complex partial a) Generalized epilepsy Tonic clonic- above 1 year of age Absence seizures Chilhood absence epilepsy Juvenile myoclonic epilepsy Myoclonic seizures Drop attacks
  • 15. TYPES OF EPILEPSY C) Epilepsy syndromes Infantile spasms Severe myoclonic syndrome Lennox-Gastaut syndrome Landau-Kleffner syndrome
  • 16. NEPEs • Events that mimic an epileptic seizure but are not caused by abnormal electrical activity of the brain • Types: Reflex (pallid) anoxic seizures Vasovagal episodes Breath holding spells Sleep related phenomena Behavioural events Psychiatric events- tics, anxiety/panic attacks
  • 17. DEVELOPMENTAL DELAY • APPROACH History Physical exam Developmental assessment Assessment for behavioural syndromes Ddx with secondary disabilities
  • 20. HEARING IMPAIRMENT • Is the most common cause of speech delay • May occur with global developmental retardation/ retarded children who fail to imitate sounds • May be due to; rubella embryopathy, cytomegalic inclusion disease, neonatal meningitis, kernicterus, and several genetic disorders • Requires audiometric testing • Crude testing is inadequate • Other clues- excessive gesturing and staring at the lips of people who are talking • Brain auditory evoked potentials offer good screening for neonatal hearing deficits
  • 21. INFANTILE AUTISM • Is a syndrome which is seen in infants. • They fail to meet few or any developmental milestones • Cause unknown but hypothesized as genetic with neuropathology • Diagnostic criteria (DSM 5) Deficits in social communication and social interaction Restrictive repetitive behaviours, interests and activities
  • 22. INFANTILE AUTISM • CAUSES: Genetics- mostly in monozygotic twins Environmental- prenatal, natal and post natal
  • 24. INFANTILE AUTISM • Diagnosis is clinically!! • Screening checklists M-CHAT INDT-ASD • Diagnostic tools Autism diagnostic interview- revised Childhood autism rating scale
  • 26. DDX
  • 27. COMPLICATIONS • Cognition- lower IQ • Sleep- difficulty in initiating sleep, frequent awakening, insomnia, early morning awakening • Feeding- selective eaters • Epilepsy in 8-46% • Other psychiatric conditions
  • 28. BASAL GANGLIA • Group of subcortical nuclei in base of forebrain • Associated with functions like; Control of voluntary movements, postural control, control of muscle tone Procedural learning Routine behaviours e.g. eye movements, teeth grinding, cognition and emotion
  • 30. HYPERKINETIC MOVEMENT DISORDERS • Pathophysiology • Most movement disorders are due to defects in functional connectivity rather than a single lesion • Direct and indirect pathways
  • 31. POST STROKE MVT DISORDERS
  • 32. TYPES OF MVT DISORDERS 1. CHOREA/BALLISM • Due to STN inactivation and reduction in basal ganglia output • share common pathophysiology • a/w BG infarction • Presents with irregular, abrupt, rapid, and transient movements that can affect the entire body, and typically manifests in the distal region of the body
  • 33. TYPES OF MVT DISORDERS • Ballism- severe form on chorea, presents with proximal limb involvement • May occur together post stroke • May also present as hemiballism/ hemichorea • Both develop immediately after a stroke • In total, 54% of cases of chorea associated with infarctions in the basal ganglia circuit completely resolve; however, chorea associated with STN lesions do not resolve.
  • 34. TYPES OF MVT DISORDERS 2. DYSTONIA • Lesions in the posterior putamen and Gpe (lentiform nucleus) • Sustained muscle contractions or abnormal posture • commonly presents in combination with hemidystonia on the contralateral side • May resolve completely/partially
  • 35. TYPES OF MVT DISORDERS • Onset is delayed- approx 9 months later • If post stroke- may develop dystonia once hemiparesis resolves 3. TICS • Sudden, repetitive, nonrhythmic motor movement or vocalization • Due to infarctions of caudate and lentiform nucleus • Presents in combination with dystonia • Due to disruption in cortico-striato-thalamo-cortical circuit
  • 36. TYPES OF MVT DISORDERS 4. MYOCLONUS 5. ASTERIXIS 6. TREMOR 7. COMPLEX 8. RESTLESS LEG SYNDROME 9. AKATHISIA 10. ISTEREOTYPIC 11. BLEPHAROSPASM
  • 37. HYPOKINETIC MVT DISORDERS • Pathophysiology 1. PARKINSONISM • Group of neurological disorders that cause movement problems such as tremors, stiffness and slow movement • Aka atypical parkinson’s disease • Progresses more rapidly cf parkinsons disease • Additional sx- early falling, dementia/hallucinations • Do not respond to L-dopa
  • 39. METABOLIC CAUSES OF PARKINSONISM 1) Thyroid disease 2) Renal failure 3) Calcium disorders 4) Iron metabolism 5) Liver failure 6) Electrolyte disturbance 7) Mitochondrial disease 8) Inborn errors of metabolism
  • 40. HYPOKINETIC MVT DISORDERS PARKINSONISM (vascular) • Aka artherosclerotic parkinsonism • Unilateral/bilateral • lesions to the lentiform nucleus and striatum, as well as pontine lesions and extensive subcortical white matter lesions • 2 types a) After BG infarction b) Chronic diffuse white matter degeneration
  • 41. VASCULAR PARKINSONISM • Presents symmetrically • No tremors • Lower body involvement • Poor response to L-dopa
  • 43. BRAINSTEM LESIONS 1. DEMYELINATING LESIONS • Diagnosis is clinically /histopath post mortem • Common types- MS, ADEM, PML and extrapontine myelinolysis • Classification based on pathogenesis; a) Inflammatory process b) Acquired metabolic derangements c) Hypoxic ischemic insults d) Focal compression
  • 44. ADEM • Autoimmune inflammatory demyelinating disease • Affects white matter and spinal cord • Commonly occurs within 3 weeks of infection, vaccination or due to certain drugs • Self limiting • Monophasic disease (if more than one relapse, consider MS)
  • 45. ADEM • PRESENTATION Ataxia Headache Weakness Slurred or impaired speech CN palsies Agitation, seizures Delirium, stupor, AMS Constitutional symptoms
  • 46. ADEM • INVESTIGATIONS Platelets and ESR raised CSF for WBCs, RBCs, biochemistry, protein is elevated CT scan brain- multiple, bilateral and asymmetrical lesions with CNS Margins of lesions are smudged MRI brain- to r/o ddx EEG- change in sleep patterns
  • 47. ADEM
  • 48. ADEM • TREATMENT: High dose i.v. corticosteroids- 20-30mg/kg/d methylprednisolone for 3-5days Then oral prednisolone at 2mg/kg. then taper over 4-6 weeks With or without; IVIG 2g/kg i.v as single dose or over 3-5 days
  • 49. MULTIPLE SCLEROSIS • Accounts for 10% of all cases in children • Neurologic symptoms disseminated in time and space • Immune mediated disease with recurrent episodes of CNS demyelination • Commonly in children under 18 years • Difficult to diagnose especially in pre pubertal children due to atypical presentation • Cause- genetic or environmental • Types- 4
  • 50. MULTIPLE SCLEROSIS • Clinical presentation  Features of encephalopathy  Optic neuritis  Ataxia  Fatigue  Constipation  Weakness  Paraesthesia  Cognitive impairment  Early onset with slow progression
  • 51. MULTIPLE SCLEROSIS • Diagnosis is made clinically!! • MRI- multiple or single T2 hyperintense lesion and differentiate from ADEM • McDonald criteria 2010
  • 53. MS TREATMENT • High dose corticosteroids (acute exacerbations) • Plasmapheresis – for those with severe exacerbations refractory to high dose CS • IVIGg- 400mg/kg/day for 5 days- who cant tolerate CS • Early initiation of DMD
  • 54. MS COMPLICATIONS • Pain • Depression • Bladder and bowel incontinence • Difficulties in speech and swallowing later • Language is intact but over time they develop expressive language deterioration
  • 55. NEUROMYELITIS OPTICA • Autoimmune disorder where auto antibodies acttack optic nerve and SC • Thus present with optic neuritis and acute transverse myelitis • Visual loss and subsequently develop paraplegia and sensory loss, but the order may be reversed • Demyelination is antibody dependent and complement mediated
  • 56. NEUROMYELITIS OPTICA • Causes pain in the eye, visual loss • Damage to SC causes weakenss/paralysis, paraesthesia, incontinence • Is a relapsing-remitting disease • Cause is due to attack of autoantibodies to aquaporin-4 water channel located in optic nerve and SC
  • 57. NEUROMYELITIS OPTICA • Diagnosis • Via modified clinical criteria by international panel • Absolute criteria and supportive criteria • 2 absolute and 2 of 3 supportive criteria is diagnostic
  • 58. NEUROMYELITIS OPTICA ABSOLUTE CRITERIA SUPPORTIVE CRITERIA OPTIC NEURITIS MRI BRAIN NOT MEETING MS ACUTE MYELITIS MRI SC T2 LESIONS OVER >3 VERTEBRAE NMO-Igg POSITIVE
  • 59. NEUROMYELITIS OPTICA • Investigations- • Autoantibodies against AQP4 • Anti AQP IgG • MRI Brain • MRI SC • Mx-no cure but supportive mx • High dose CS/ plasmapheresis • Secondary prevention is immunosuppressive drugs to reduce frequency and severity of relapses
  • 60. ACUTE TRANSVERSE MYELITIS • Is inflammation of white and grey matter in one or more adjacent SC segments • Affects the spinal cord in its entire width (transversely) • Usually thoracic region • Blocks transmission of nerve impulses • Trigger- unknown • Maybe due to autoimmune reaction • Risk factors- MS, neuromyelitis optica, bacterial infections (lyme disease, syphilis or TB), SLE, viral meningoencephalitis, drugs, vaccination,
  • 61. ACUTE TRANSVERSE MYELITIS • SYMPTOMS Sudden pain in the back Band like tightness around affected area (chest/abdomen) Tingling, numbness, muscle weakness within days (ascending) Urine incontinence/ urgency Paralysis, loss of sensation Urine retention
  • 62. ACUTE TRANSVERSE MYELITIS • INVESTIGATIONS MRI spine- variable enlargement of SC LP- elevated WBCs and protein MRI brain Others- HIV, serology for lymes disease, copper and zinc levels, ANA, VDRL, vit B12 levels
  • 63. ACUTE TRANSVERSE MYELITIS • TREATMENT Underlying cause (infectious, nutritional def) If no cause identified; high dose coritcosteroids followed by plasma exchange (efficacy of such tx is uncertain)
  • 64. NEOPLASTIC BRAIN STEM LESIONS • Types; a) Infratentorial lesions- contains cerebellum b) Supratentorial lesions- contains cerebrum
  • 67. SPINAL CORD LESIONS • Lesions affecting the spinal cord Transverse myelitis Infectious- spinal abscess Herpes myelitis Tb spine- pott’s disease
  • 68. SPINAL CORD LESIONS • Spinal abscess can occur anywhere along spinal axis • Most commonly affects posterior thoracic spinal cord • Presentation depends on level of spinal cord involvement and duration of infection • Common causative org- staph and strep spp • Sx- weakness, paraesthesia, dysesthesia, bladder and bowel incontinence and acute paraplegia • Chronic cases- gradual progression
  • 69. SPINAL ABSCESS • INVESTIGATIONS LP- CSF exam Abscess C/S CSF microscopy to look for parasites Gadolinium enhanced MRI (ring enhancement) Mx- surgical drainage of abscess, c/s, appropriate abx post op and steroids to reduce inflammation
  • 70. HERPES MYELITIS • A form of acute ascending necrotizing myelitis • Caused by HSV-2 • Thoracic and lumbosacral are most common sites • Rare, mostly reported in patients with Acquired IDS and malignancy • Ascending myelopathy • Due to primary infection or reactivation • Transmitted- in utero, sexually
  • 71. HERPES MYELITIS • SX- Pain, limb numbness, paraesthesia, weakness • A/w herpetic skin lesions • MRI spine- enlargement of lower cord or conus medullaris • Contrast enhancement of adjacent nerve roots
  • 72. IMAGE
  • 73. TB SPINE • Aka pott’s disease, tuberculous spondylitis • Caused by Mycobacterium TB • Manifests as a combination of osteomyelitis and arthritis • Involves more than one vertebrae (usually anterior aspect) • Progressive bone destruction leads to vertebral collapse and kyphosis • Spinal canal gets narrowed by abscess/tissue- spinal cord compression and neuro sx
  • 74. TB SPINE • SX- constitutional sx (wgt loss and fever) • Duration is 4 months (average) • Chronic back pain • SC compression- paraplegia, paresis, impaired sensation, nerve root pain and cauda equina syndrome • Cervical vertebrae- pain and stiffness • Lower cervical- hoarseness, neuro deficits and torticollis
  • 75. TB SPINE • Commonly affects thoracic spine • Local pain • Muscle rigidity and spasm • Skin manifestations • Cold abscess in psoas muscle
  • 76. TB SPINE • Tuberculin skin test • ESR • Microbio studies- bone tissue/ abscess sample for gene expert, culture and sensitivity, microscopy • CT guided needle biopsy of lesions • Xray spine • CT scan • MRI- for disc space infection and osteomyelitis, soft tissue involvement
  • 79. • THANK YOU FOR LISTENING!

Notes de l'éditeur

  1. frontal lobe  -primary motor cortex and premotor cortex are involved in contralateral movement -Broca's area is involved in producing speech -frontal eye fields are involved in eye movement -prefrontal cortex is involved (this is a simplification) in; restraint, initiative, order
  2. Parietal lobe; -primary somatosensory cortex is involved in receiving contralateral sensory information -Wernicke's area is involved in language comprehension Temporal lobe- processing sound Occipital lobe- recognizing visual stimuli
  3. Paroxysmal events- are sudden fits or outbursts OR sudden onset of neurological dysfunction- episodic phenomenon May be epileptic- present with seizures which appear due to bursts in abnormal brain waves NEPEs- mimic epileptic seizures, but not caused by abnormal electrical activity of brain
  4. Seizure is an event Epilepsy is the disease involving recurrent unprovoked seizures
  5. Focal epilepsy- 1. Simple partial- consciousness is preserved, motor automatisms 2. Complex partial- LOC Investigations- sleep EEG Generalized epilepsy- Tonic- extension and stiffening of the body, usually associated with apnea and upward deviation of the eyes clonic- Repeated, irregular slow clonic movements (1 to 3 jerks/second) affecting one limb or both limbs on one side Absence seizures- involves staring spells, brief (<15secs) Chilhood absence epilepsy- Juvenile myoclonic epilepsy Myoclonic seizures- Brief, nonrhythmic extension and flexion movements of the arms, the legs, or all limb Drop attacks
  6. 1. Pallid anoxic seizures usually occur in child under 18 months of age Provoked by pain stimulus with minimal distrss which leads to LOC, stiffening, down beating of eyelids and incontinence 2. Vasovagal episodes- preceeded by postural, emotional or situational factors which lead to lightheadedness, weakness, pallor, sweating and visual distortion. May also have progressive loss of tone, bradycardia, LOC, incontinence, jerking. Followed by rapid mental recovery 3. breath holding- provoked by anger, frustration, pain or fright. Preceded by vigorous crying 4. sleep related phenomena- sleep related- hypnic jerks, sleep walking (somnambulism) 5. behavoiral events- includes day dreaming, self gratification, posturing, vocalization, aggressive outbursts- “zone out”
  7. Hx- keep broad and open mind. Don’t rush through Pre natal , natal or postnatal hx to r/o causes Family hx- anyone who went to special school/ similar delay/ with learning disabilities Open qns about how the child plays with their toys/ range of motion/ restrictions/ interests Other assesments for dev of child includes- movements and posture, hand function and personal care, vision, hearing, communication, feeding, social communication and relationships, behaviour, cognition and sleep End with open ended questions- ‘is there anything else you think might be important, that I have not asked you about’? To get any oter missing details Phy exam- Anthopometric measurements Head to toe exam for any outstanding features- dysmorphic CNS exam Do gowers maneuvre Fundoscopic exam Audiometry Developmental assesment
  8. Autism- is a spectrum disorder as it may range from mild to severe form Defined as a set of behaviours that are developmentally delayed, including socialization, communication and repetitive /aggressive behaviours Slow to acquire skills Lower IQ lack of social skills, interest to play with other children, excessive aggression, temper tantrums Self stimulating repititive bahaviours Flapping or wringing of hands Types- infantile and regressive autism regressive autism refers to when children develop normally till 18 months of age then begin to regress. Global incidence- 1:68 children CDC (2012) and is incresing
  9. Prenatal- congenital rubella syndrome, exposure to teratogens eg thalidomide and valproic acid Natal- LBW, Premature and BA Post natal- autoimmune, vit D def, mercury toxicity
  10. Modified checklist for autism in toddlers For children btn 16-30months Aap recommends at 18 and 24 months together developmental assessment at 9,18 and 24 months
  11. PATHO- lesions of the STN decrease the excitation of the basal ganglia output, and consequently reduce thalamic inhibition. The striatum is commonly involved in hyperkinetic movement disorders. According to the box theory, lesions in the striatum interrupt the transport of GABA to the GPe and, as a result, inhibit the STN. This change can lead to the loss of control of the GPi and consequently relieve the inhibition of thalamic output. In turn, the decrease in striatal inhibition of neurons in the globus pallidus enhances STN inhibition, thereby relieving the inhibition of basal ganglia output to the thalamus. The GABAergic and dopaminergic system might also contribute to post-stroke movement disorders.
  12. Post stroke patho- Animal studies demonstrated that ischemic insult alters dopaminergic receptors and that this change leads to the degeneration of the GABAergic system in the SNr [12,13]. Notably, GABA plays a major role in the modulation of inhibitory synaptic transmission in the brain. Risk factors and variables for post stroke mvt disorders -location, the severity of the injury, individual susceptibility, and age of occurrence, may play a role in determining movement disorder after basal ganglia infarction.
  13. Chorea and ballism- share common patho
  14. PATHO A reduction in dopamine levels stimulates the indirect pathway and results in a strong inhibition of neurons in the GPe. These events lead to the tonic inhibition of the STN, and exert a strong excitatory effect on basal ganglia output neurons. Thus, hypokinetic movements in Parkinson’s disease are attributed to the strong inhibition of basal ganglia output
  15. Thyroid disease- hypothyroidism- shares features with parkinsonism- rigidity, fatigue, constipation, masked facial expression, depression and slow mvts Renal failure- parkinsonism seen in pts with uremia due to ESRD esp with DM Calcium disorders- hypoparathyroidism seen in parkinsonism in presence/absence of BG calcifications Iron metabolism- hereditary hemochromatosis- abnormal iron accumulation in organs- causes mvt disorders such as dystonia seen in parkinsonism Liver failure- non-wilsonian liver disease found to be a/w parkinsonism Electrolyte disturbances- rapid correction of na levels, extrapontine and central pontine myelinolysis syndromes Inborn errors of metabolism- gauchers disease- neuro signs PKU
  16. Functions- midbrain- vision, hearing, eye and body mvt Pons- motor control and sensory analysis Medulla- maintaining vital body functions- breathing and heartrate
  17. Common types- multiple sclerosis, acute‐disseminated encephalomyelitis, progressive multifocal leucoencephalopathy and extrapontine myelinolysis Inflammatory- ADEM, MS and acute hemorrhagic leucoencephalitis
  18. Vaccine- rabies vaccine- Semple form (NAMED AFTER DAVID SEMPLE) and measles vaccine 1:800 Infections- URTI viral- MMR, mono, varicella Bacteria- GAS, campylobacter jejuni, mycoplasma pneumoniae
  19. Study by Callen et al., features to distinguish first MS attack from ADEM in a child.. Criteria; any 2 will distinguish MS from ADEM Absence of a diffuse bilateral lesion pattern Presence of black holes Presence of 2 or more periventricular lesions
  20. i.V CS and IVIG in severe cases
  21. Genetic- monozygotic twins and major histocompatibility complex on chromosome 6p21 Environ- from prevalence and migration studies Autoimmune conditions Viral infections- EBV, CMV , HSV 1 and HHV 6 (direct injury to CNS and myelin sheath or molecular mimicry) Query abt hep B vaccine and MS- no correlation Types- Relapsing-remitting MS- common Secondary progressive MS Primary progressive MS Progressive relapsing MS Others- Classical MS Acute MS Neuromyelitis optica Concentric MS
  22. Features of encephalopathy may present with multifocal neuro deficits (pre puberty) Or monofocal deficits (adolescents) Sx are mostly due to relapse. Clinical presentation in primary disease is rarely diagnosed (5%)
  23. Mc donalds criteria- MRI evidence on basis of one relapse/ attack which should last for more than 24 hours with MS neuro deficits And 30 days btn attacks so as to count them as 2 separate attacks
  24. i.v. methly prednisolone 20-30mg/kg for 3-5 days DMD- reduces relapses and reduces inflammation DMD- interferon beta and glatiramer- first line DMDs
  25. Can be polyphasic- multiple relapses and remissions Or monophasic- can be either fulminant and fatal or a/w degrees of recovery
  26. AQ- aqua protein
  27. Drugs- anti parasitic/ antifungal/ i.v heroine/amphetamine use
  28. Degree of disability depends on location and severity of inflammation
  29. Ddx- GBS/ cord compression/ SOL/ spinal cord tumors
  30. Infra- below tentorium cerebelli Supra- above
  31. Acute sx- fever, chills, back pain, malaise
  32. Csf findings- elevated protein and leucocytes
  33. Presentation depends on- stage of disease, affected site, presence of complications e.g. neuro deficits, abscesses or sinus tracts Cauda equina syndrome- is a pattern if neuromuscular and urogenital sx due to compression of multiple lumbosacral nerve roots below level of conus medullaris Torticollis-to and fro mvts of head due to dystonia- literally means twisted neck
  34. Findings on xray- lytic destruction Incereased ant wedging Collapsed vertebral body Management- 2 months RIPE, 10 months IR Steroids- to prevent complications hydrocephalus