Paeds Neuro Flashcards

1
Q

Chiari Malformation

  1. Pathology
  2. S&S
  3. Mx
A

Chiari malformation

  1. Low-lying cerebellar tonsils
    - CSF flow blockage
  2. Sx
    - Headache
    - Loss of balance on jumping
    - Chiari crisis (death due to acute episode)
  3. Mx
    - MRI spine (syrinx)
    - Surgical intervention
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2
Q

Dandy Walker Malformation

  1. Pathology
  2. Mx
A

Dandy-Walker malformation

  1. Cerebellar vermis malformation
    - Poor CSF Drainage
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3
Q

Aicardi syndrome

  1. Prevalence
  2. Pathology
A

Aicardi syndrome

  1. 1/100,000
    - Mostly girls survive
  2. Autoimmune
    - Corpus callosum malformation
    - Seizures
    - Skull and Eye abnormalities
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4
Q

Neurocutaneous diseases
- Three main disease

A

Neurocutaneous disease

  1. Neurofibromatosis (NF)
    - NF1, NF2
    - Schwannomatosis
  2. Tuberous sclerosis
  3. Sturge-Weber Disease
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5
Q

Neurofibromatosis (NF)

  1. Types
  2. Presentation
  3. Mx
A

Neurofibromatosis

  • Types
    1. NF1, NF2
    2. Schwannomatosis
  • Presentation
    1. Cafe-au-lait spots, Lisch nodules (on iris)
    2. Neurofibromas
    3. Pain
    4. Neurological deficits
  • Mx
    1. Close monitoring of all growths
    2. Removal of malignant or troubling tumours
    3. Counselling and therapy
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6
Q

Tuberous Sclerosis (TS)

  1. S&S
  2. Mx
A

Tuberous sclerosis

  • S&S
    1. Shagreen Patches
    2. Hyperpigmentation
    3. Seizure
    4. Cardiac rhabdomyoma
  • Mx
    1. Symptom relief and complication minimisation
    2. Epilepsy management
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7
Q

Sturge-Weber Disease

  1. S&S
  2. Mx
A

Sturge-Weber Disease
(1/50,000)

  • S&S
  1. Angiomas
    - Port-wine stain
    - Epilepsy
  2. Headaches
  3. Visual disturbances
  4. Developmental delay
  • Mx
    1. Aspirin
    2. Carbamazepine
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8
Q

Epilepsy
- Diagnosis

A

Epilepsy Diagnosis

  1. Seizure video
    - ?Conscious state
    - ?Bodily movements
  2. Diary
    - Event record
  3. Description
    - Collateral history
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9
Q

Absence seizure
- Features

A

Absence seizure

  1. Automatism of mouth/finger
  2. Facial paralysis
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10
Q

Tic Disorder

  1. Types
  2. Pathophysiology
A

Tic Disorders

  • Types
    1. Motor
  • Simple/Complex
    2. Vocal
  • Simple/Complex
  • Pathophysiology
    1. 80% premonitory sensation
    2. Primary
  • Transient <1yr
  • Persistent >1yr
  • Tourettes (Idiopathic/inherited)
  • Unknown origin
    3. Secondary
  • Infection/trauma
  • Drug/metabolic
  • Stroke/neurodegenerative
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11
Q

Tic Disorder
- Mx

A

Tic Disorder Mx

  1. Psychoeducation
  2. CBIT
    - Cognitive behavioural intervention for tic
  3. Alpha 2 agonists
  4. Botox
  5. Aripiprazole/risperidone
  6. Topiramate
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12
Q

SUDEP

  1. Risk
A

SUDEP

  • Risk
    1. 7-12% lifetime risk
    2. Increased with severe epilepsy
    3. Non-adherence
    4. Living alone
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13
Q

EEG
- Wave patterns

A

EEG

Alpha - Awake (8-12 Hz)
Beta - Benzos (13-30Hz)
Theta - Drowsy (4-8Hz)
Delta - Asleep (1-4Hz)

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14
Q

SMA

  1. Features
  2. Mx
A

Spinal muscular atrophy

  • S&S
    1. Floppy/weak arms/legs
    2. Twitchy
  • Mx
    1. Feeding
  • Diet
  • NG
    2. Breathing
  • Exercises
  • Suction
  • Supplementation
    3. Mobility
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15
Q

Congenital hydrocephalus
- Causes
- Mx

A

Congenital hydrocephalus

  • Causes
    1. CSF obstruction
  • Anatomical
  • Lesions
  • Infections
    2. Intrauterine infection
  • Rubella/syphilis
  • Mx
    1. Shunt
  • VP
  • VA (atria)
  • LP (lumbar peritoneal)
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16
Q

Cerebral Palsy
- Definition

A

Cerebral Palsy Definition

  • Non progressive
  • Relative to birth
    1. Before
    2. During
    3. After
  • Change to development
    1. Movement
    2. Posture
  • CP does NOT cause Cognitive impairment
  • though they two conditions can co-exist
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17
Q

Cerebral Palsy
- 1-3 Trim causes

A

Cerebral Palsy Causes

1st Trimester
i) Chicken P/Rubella/CMV/Toxoplasmosis
ii) Chromosomal

2nd Trimester
i) Infections
ii) NMDs
- Neural migration disorders
iii) REDF/AREDV
- Reversal of umbilical artery end-diastolic flow
- Reverse doppler
iv) IUGR

3rd trimester
i) Nutrition
ii) Nuchal cord
iii) Fetal ischaemia

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18
Q

Cerebral palsy
- Peri-natal and post-natal causes

A

Cerebral palsy causes

  1. Perinatal
    i) PRoM
    ii) Maternal fever

iii) Neonatal sepsis
- Strep B (BenPen and Gent)
- Meningitis (Double dose and length)
- E Coli meningitis (3 weeks ABx)

  1. Post-natal
    i) HIE (birth asphyxia)
    - APGAR
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19
Q

APGAR Score
- Components

A

APGAR

A ppearance
- Pale all over/blue
- Peripheral cyanosis
- Pink

P ulse
- 0, <100, 100-140

G rimace
- Unresponsive
- Grimace or weak cry
- Cry when stimulated

A ctivity
- Floppy
- Some flexion
- Well flexed and resists extension

R espiration
- Apnoea
- Slow, irregular
- Strong cry

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20
Q

Cerebral Palsy
- Epidemiology
- Types

A

CP

  • Epidemiology
    1. Most common childhood disability
    2. 2/1000
    3. 70x higher in Very low BW
    4. Multiple births at risk
  • 12.6 twins
  • 44.8 triplets
    5. Resource poor countries
  • Types
    1. Spastic (80%)
  • Motor cortex
  1. Dyskinetic (6%)
    - Basal ganglia
  2. Ataxic (6%)
    - Cerebellar
  3. Mixed
    - Combination damage
21
Q

Cerebral Palsy
- Features of each type

A

CP Features

  • Spasticity (80%)
    1 Monoplegia
    2 Hemiplegia
    3 Diplegia
    4 Quadriplegia
  • Dyskinesis
    1. Dystonia
    2. Athetosis
    3. Chorea
  • Ataxia
    1. Abnormal force
    2. Abnormal rhythm
    3. Impaired accuracy
22
Q

Cerebral palsy Mx

Spasticity

A

CP Mx

  • Spasticity
    1. OT
  • Bimanual upper-extremity activities
  • Symmetry of gait and posture
  1. PT
    - CIMT (constraint-induced movement therapy)
    - Mobility/treadmill
  2. SALT
    - Educate and assist family
    - Developmental milestones

+ Orthoses
- Casting and bracing

+ Adaptive equipment

+ Injectable pharmacotherapy
- Botulinum toxin type A (/phenol)
- Delayed onset 1-2 days
- Day 10 effect
- 3-4 months effect

+ Ortho surgery
- Resistant contractures/bony deformity
- Tendon transfers
- First web-space release
- Wrist fusion

23
Q

Cerebral Palsy Mx
- Dyskinesia

A

CP Dyskinesia Mx

  1. OT/PT
  2. SALT
  3. Orthoses
  4. Adaptive equipment
  5. Dystonia mx
    - Carbidopa/Levodopa
    - Diazepam/trihexyphenidyl
    - Botulinum toxin type A
    - Intrathecal baclofen pump (GABA)
    - Deep brain stimulation (DBS)
  6. Athetosis (slow writhing movements)
    - Tetrabenazine
    - Carbidopa/levodopa
    - Diazepam
    - Dantrolene
24
Q

CP Management
- Ataxia

A

CP Ataxia Mx

  1. OT/PT
  2. SALT
  3. Orthoses
  4. Adaptive equipment
  5. Pharmacotherapy
    - Tremor
    - Depression
25
Q

Intellectual disability

  • Classification
A

Intellectual disability

  1. Generalised
    - Cognitive impairment
  2. Specific to one area
    - Learning difficulty
26
Q

Cognitive impairment
1. Definition
2. Gradation

A

Cognitive impairment

  • Definition
    1. Intellectual disability
    2. Generalised
  • Gradation
    70 > IQ
  • Cognitive impairment
    50 < IQ < 70
  • Mild cognitive impairment
27
Q

Learning difficulty
- Three common examples

A

Learning difficulties

  1. Specific language impairment
  2. Dyslexia
  3. Dyscalculia
28
Q

Cognitive impairment vs dyslexia

1.Prevalence

  1. Associations
A

Cognitive impairment

  1. Prevalence
    - 2-3%
  2. Associations
    - T21
    - FAS

Learning disability

  1. Prevalence
    - 10%
    - More common in male sex
  • Dyslexia (5-12% of children)
29
Q

Fragile X syndrome

  1. Epidemiology
  2. Pathology
A

Fragile X

  • Epidemiology
    1. 1:6000 men
    2. 1:5000 women
    3. 30% of males have AD
    4. Diagnosis by age 3
  • Pathology
    1. Repeated CGG on X chromosome
    2.FMRP overproduction
  • Fragile X mental retardation protein
    3. IQ < 70
    4. Delayed milestones
30
Q

Fragile X syndrome

  1. S&S
  2. Mx
A

Fragile X syndrome

  • S&S
  1. IQ < 70
  2. Delayed milestones
    - Clumsiness
    - Echolalia
    - Perseveration
  3. High forehead, facial asymmetry
  4. Macrognathia, long ears
  5. Hyperextensible fingers/flat feet
  6. Soft skin
  7. Large testicles
  8. Mitral valve prolapse
  9. Anxiety/OCD
    - Repetitive actions
    - Avoidance of gaze
  10. Emotional lability/aggression
31
Q

Self-harm

  1. Epidemiology
    11 - 16
    17 - 19
    Method
  2. Relation to suicide
A

Self-harm

  • Epidemiology
    1. 11-16
  • 7.3% Girls
  • 3.6% Boys
    2. 17-19
  • 21.5% Girls
  • 9.7% Boys
31
Q

Self-harm

  1. Epidemiology
    11 - 16
    17 - 19
    Method
    Repetition
  2. Relation to suicide
A

Self-harm

  • Epidemiology
    1. 11-16
  • 7.3% Girls
  • 3.6% Boys
    2. 17-19
  • 21.5% Girls
  • 9.7% Boys
  1. Majority self-poisoning
  2. 20% repeat within a year
  • Suicide
    1. 30-50 fold increase in risk
  • After hospital presentation
32
Q

Self-harm Mx

  1. Info
  2. Confidentiality
  3. Safeguarding
  4. Carers
A

Self Harm Mx

  • Info
    1. Support and treatments
    2. Injuries and scars
    3. Care plans and Safety Plans
  • Plan of healthcare/treatment provided
  • Avoiding crisis/coping/escalating
    4. Emergencies
  • Confidentiality
    1. Capacity/MHA
    2. Involved decision making
  • Safeguarding
    1. 2014 Care act
    2. Ask about concerns eg. abuse/violence
    3. Document
    4. Multi-agency approach
  • Carers
    1. Consent young person
    2. Safeguarding concerns
    3. Empower young person
    4.
33
Q

Self-harm Mx
- Three assessments

A

Self-harm assessments

  1. Assessment by health and social
    - Establish and remove harm
    - Consent and capacity
    - Liaise with carers
    - Consider referral to MH/social
    - Regular review
    - Medicines review
    - Signposting
  2. Psychosocial
    - MH professional
    - Care plan
    - Values/wishes
    - MH/support/family
    - Past, present, future, protective factors
    - Social/home/school
    - Social media
    - Safeguarding
  3. Risk Assessment
    - Not predictive
    - Not prescriptive
    - Focus on needs
    - Immediate and LT safety
34
Q

Self-harm Mx

  1. Hospital
    2 Aftercare
A

Self-harm hospital mx

  • Hospital
    1. Admit if concerns of safety
    2. Urgent psychosocial assessment
    3. Discharge only when
  • Psychosocial assessment
  • MDT Plan
  • MDT discharge meeting
  • Aftercare including primary care
  • After care
    1. Discuss and agree
  • Purpose
  • Format
  • Frequency
    2. 48 hours follow-up if concerns
  • GP
  • Community MH
35
Q

Self-harm Mx

  1. Interventions
  2. Support
  3. Prescribing
A

Self-harm mx

  • Interventions
    1. Co-morbidities consideration
  • Addiction
  • ASD/ADHD/Intellectual disability
  • MH
  1. CBT
  2. DBT-A
    - Dialectical BT for adolescents
  3. Safety plan
  4. Harm minimisation
  5. Therapeutic risk-taking
  • Support
  1. Continuity of care
  2. Communication of care plans
  3. Least restrictive balance
  • Prescribing
    1. Toxicity
    2. Recreational considerations
    3. Shared decision making
    4. Review
36
Q

Cerebral Palsy Mimics
- Prominent spasticity x3
- Prominent dyskinesia x3
- Prominent ataxia x3

A

CP Mimics
- Prominent spasticity

  1. HSP - Hereditary spastic paraplegias
  2. Arginase deficiency
  3. Leukodystrophies
37
Q

Cerebral Palsy Mimics
- Prominent dyskinesia

A

Cerebral Palsy Mimics
- Prominent dyskinesia

  1. DRD - Dopa-responsive dystonia
  2. GA1 - Glutaric aciduria Type I
  3. Cerebral folate deficiency
38
Q

Cerebral palsy mimics
- Prominent ataxia

A

Cerebral palsy mimics
- Prominent ataxia

  1. PLP-1 dysmyelination (proteolipid protein 1)
    - PMD - Pelizaeus-Merzbacher disease
    - SPG2 - Spastic paraplegia type II
  2. Angelman syndrome
    - Chromosome 15
  3. Maple syrup urine disease
    - Leucine, isoleucine, valine
39
Q

Metabolic myopathies
- 3 Examples

A

Metabolic myopathies

  1. McArdle Disease
    - Myophosphorylase deficiency
  2. Pompe’s disease/Acid Maltase disease/Glycogen storage disease II
    - Acid alpha-glucosidase deficiency
  3. Mitochondrial metabolic myopathies
40
Q

Intracranial cysts

  1. Epidemiology
  2. Pathophysiology
A

Intracranial cysts

  • Epidemiology
    1. Pre-natal US
  • Pathophysiology
    1. Fluid-filled collection
  • Extra-axial/intraparenchymal/intraventricular
    2. Exerts a mass effect
    3. Possible CPVL cystic peri-ventricular leukomalacia
    4. Choroid plexus cysts
  • Benign
  • Mx
    1. Most are benign
41
Q

Corpus callosum agenesis

  • Epidemiology
  • Sequelae
  • Mx
A

Corpus callosum agenesis

  • Epidemiology
    1. Isolated
    2. Associated
  • Ventriculomegaly
    3.
  • Sequelae
    1. Mild LD
    2. CP
    3. ASD
    4. Severe intellectual disability
  • Mx
    1. Sx management
42
Q

Febrile Seizure

  1. Epidemiology
  2. Mx
    - Simple/complex
    - Status
A

Febrile seizure

  • Epidemiology
    1. 6mo - 5yo
    2. <3yo first presentation
  • Mx
    1. Simple/complex
  • Anti-pyretic
    +Anti-convulsant
  1. Status
    - Neurology/ITU management
43
Q

Febrile convulsion
- Simple vs Complex

A

Febrile convulsion

  • Complex seizure
    1. Focal
    2. Prolonged
  • > 15 minutes
    3. Multiple seizures
  • within 24 hours
44
Q

Neonatal hypothermia

  • S&S
A

Neonatal hypothermia
- S&S

  1. Respiratory distress
    - BAT thermogenesis
  2. Metabolic acidosis
  3. Hypoxia
  4. PPHN
    — Persistent pulmonary HTN of newborn
45
Q

Paeds Nerve Palsies
- Peripheral trauma

A

Paeds peripheral palsies

  1. Radial
    - Supracondylar humeral fractures
    - Humeral shaft fracture
  2. Median
    - Supracondylar fracture
    - Elbow dislocation
  3. Sciatic
    - Accidental crush/laceration
    - Buttock injection
    - Breech delivery stretch
  4. Common peroneal
    - Fibular head
    - Knee manipulations
  5. Lumbar plexus
    - Blunt abdo trauma
    - Birth injury
46
Q

Radial nerve injury
- Axilla vs Groove

A

Radial nerve injury

  • Axilla
    1. Shoulder dislocation/ Humeral fracture
    2. Motor
  • Posterior compartment (unopposed flexion)
  • Wrist drop
    3. Sensory
  • Arm (lateral/posterior)
  • Forearm (posterior)
  • Hand (dorsal 3.5)
  • Radial groove
    1. Humeral shaft
    2. Motor
  • Weakened triceps
  • Posterior forearm
    3. Sensory
  • Dorsal hand only
47
Q

Erb’s Palsy

  1. Branches
  2. Motor
  3. Sensory
A

Erb’s Palsy

  • Branches
    1. C5/C6
    2. Upper plexus
  • Motor
    1. Shoulder abduction + flexion
    2. Arm lateral rotation
    3. Forearm supination
  • Sensory
    1. Lateral upper limb
  • Dermatomal
48
Q

Klumpke’s Palsy

  1. Roots
  2. Motor
  3. Sensory
A

Klumpke’s Palsy

  • Roots
    1. C8-T1
    2 Ulnar and median peripherals
  • Motor
    1. Intrinsic hand
  • Clawed hand
  • Sensation
    1. Medial upper limb
  • Dermatomal