Paeds Neuro Flashcards
Chiari Malformation
- Pathology
- S&S
- Mx
Chiari malformation
- Low-lying cerebellar tonsils
- CSF flow blockage - Sx
- Headache
- Loss of balance on jumping
- Chiari crisis (death due to acute episode) - Mx
- MRI spine (syrinx)
- Surgical intervention
Dandy Walker Malformation
- Pathology
- Mx
Dandy-Walker malformation
- Cerebellar vermis malformation
- Poor CSF Drainage
Aicardi syndrome
- Prevalence
- Pathology
Aicardi syndrome
- 1/100,000
- Mostly girls survive - Autoimmune
- Corpus callosum malformation
- Seizures
- Skull and Eye abnormalities
Neurocutaneous diseases
- Three main disease
Neurocutaneous disease
- Neurofibromatosis (NF)
- NF1, NF2
- Schwannomatosis - Tuberous sclerosis
- Sturge-Weber Disease
Neurofibromatosis (NF)
- Types
- Presentation
- Mx
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
Tuberous Sclerosis (TS)
- S&S
- Mx
Tuberous sclerosis
- S&S
1. Shagreen Patches
2. Hyperpigmentation
3. Seizure
4. Cardiac rhabdomyoma - Mx
1. Symptom relief and complication minimisation
2. Epilepsy management
Sturge-Weber Disease
- S&S
- Mx
Sturge-Weber Disease
(1/50,000)
- S&S
- Angiomas
- Port-wine stain
- Epilepsy - Headaches
- Visual disturbances
- Developmental delay
- Mx
1. Aspirin
2. Carbamazepine
Epilepsy
- Diagnosis
Epilepsy Diagnosis
- Seizure video
- ?Conscious state
- ?Bodily movements - Diary
- Event record - Description
- Collateral history
Absence seizure
- Features
Absence seizure
- Automatism of mouth/finger
- Facial paralysis
Tic Disorder
- Types
- Pathophysiology
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
Tic Disorder
- Mx
Tic Disorder Mx
- Psychoeducation
- CBIT
- Cognitive behavioural intervention for tic - Alpha 2 agonists
- Botox
- Aripiprazole/risperidone
- Topiramate
SUDEP
- Risk
SUDEP
- Risk
1. 7-12% lifetime risk
2. Increased with severe epilepsy
3. Non-adherence
4. Living alone
EEG
- Wave patterns
EEG
Alpha - Awake (8-12 Hz)
Beta - Benzos (13-30Hz)
Theta - Drowsy (4-8Hz)
Delta - Asleep (1-4Hz)
SMA
- Features
- Mx
Spinal muscular atrophy
- S&S
1. Floppy/weak arms/legs
2. Twitchy - Mx
1. Feeding - Diet
- NG
2. Breathing - Exercises
- Suction
- Supplementation
3. Mobility
Congenital hydrocephalus
- Causes
- Mx
Congenital hydrocephalus
- Causes
1. CSF obstruction - Anatomical
- Lesions
- Infections
2. Intrauterine infection - Rubella/syphilis
- Mx
1. Shunt - VP
- VA (atria)
- LP (lumbar peritoneal)
Cerebral Palsy
- Definition
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
Cerebral Palsy
- 1-3 Trim causes
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
Cerebral palsy
- Peri-natal and post-natal causes
Cerebral palsy causes
- 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)
- Post-natal
i) HIE (birth asphyxia)
- APGAR
APGAR Score
- Components
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
Cerebral Palsy
- Epidemiology
- Types
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
- Dyskinetic (6%)
- Basal ganglia - Ataxic (6%)
- Cerebellar - Mixed
- Combination damage
Cerebral Palsy
- Features of each type
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
Cerebral palsy Mx
Spasticity
CP Mx
- Spasticity
1. OT - Bimanual upper-extremity activities
- Symmetry of gait and posture
- PT
- CIMT (constraint-induced movement therapy)
- Mobility/treadmill - 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
Cerebral Palsy Mx
- Dyskinesia
CP Dyskinesia Mx
- OT/PT
- SALT
- Orthoses
- Adaptive equipment
- Dystonia mx
- Carbidopa/Levodopa
- Diazepam/trihexyphenidyl
- Botulinum toxin type A
- Intrathecal baclofen pump (GABA)
- Deep brain stimulation (DBS) - Athetosis (slow writhing movements)
- Tetrabenazine
- Carbidopa/levodopa
- Diazepam
- Dantrolene
CP Management
- Ataxia
CP Ataxia Mx
- OT/PT
- SALT
- Orthoses
- Adaptive equipment
- Pharmacotherapy
- Tremor
- Depression
Intellectual disability
- Classification
Intellectual disability
- Generalised
- Cognitive impairment - Specific to one area
- Learning difficulty
Cognitive impairment
1. Definition
2. Gradation
Cognitive impairment
- Definition
1. Intellectual disability
2. Generalised - Gradation
70 > IQ - Cognitive impairment
50 < IQ < 70 - Mild cognitive impairment
Learning difficulty
- Three common examples
Learning difficulties
- Specific language impairment
- Dyslexia
- Dyscalculia
Cognitive impairment vs dyslexia
1.Prevalence
- Associations
Cognitive impairment
- Prevalence
- 2-3% - Associations
- T21
- FAS
Learning disability
- Prevalence
- 10%
- More common in male sex
- Dyslexia (5-12% of children)
Fragile X syndrome
- Epidemiology
- Pathology
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
Fragile X syndrome
- S&S
- Mx
Fragile X syndrome
- S&S
- IQ < 70
- Delayed milestones
- Clumsiness
- Echolalia
- Perseveration - High forehead, facial asymmetry
- Macrognathia, long ears
- Hyperextensible fingers/flat feet
- Soft skin
- Large testicles
- Mitral valve prolapse
- Anxiety/OCD
- Repetitive actions
- Avoidance of gaze - Emotional lability/aggression
Self-harm
- Epidemiology
11 - 16
17 - 19
Method - Relation to suicide
Self-harm
- Epidemiology
1. 11-16 - 7.3% Girls
- 3.6% Boys
2. 17-19 - 21.5% Girls
- 9.7% Boys
Self-harm
- Epidemiology
11 - 16
17 - 19
Method
Repetition - Relation to suicide
Self-harm
- Epidemiology
1. 11-16 - 7.3% Girls
- 3.6% Boys
2. 17-19 - 21.5% Girls
- 9.7% Boys
- Majority self-poisoning
- 20% repeat within a year
- Suicide
1. 30-50 fold increase in risk - After hospital presentation
Self-harm Mx
- Info
- Confidentiality
- Safeguarding
- Carers
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.
Self-harm Mx
- Three assessments
Self-harm assessments
- 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 - Psychosocial
- MH professional
- Care plan
- Values/wishes
- MH/support/family
- Past, present, future, protective factors
- Social/home/school
- Social media
- Safeguarding - Risk Assessment
- Not predictive
- Not prescriptive
- Focus on needs
- Immediate and LT safety
Self-harm Mx
- Hospital
2 Aftercare
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
Self-harm Mx
- Interventions
- Support
- Prescribing
Self-harm mx
- Interventions
1. Co-morbidities consideration - Addiction
- ASD/ADHD/Intellectual disability
- MH
- CBT
- DBT-A
- Dialectical BT for adolescents - Safety plan
- Harm minimisation
- Therapeutic risk-taking
- Support
- Continuity of care
- Communication of care plans
- Least restrictive balance
- Prescribing
1. Toxicity
2. Recreational considerations
3. Shared decision making
4. Review
Cerebral Palsy Mimics
- Prominent spasticity x3
- Prominent dyskinesia x3
- Prominent ataxia x3
CP Mimics
- Prominent spasticity
- HSP - Hereditary spastic paraplegias
- Arginase deficiency
- Leukodystrophies
Cerebral Palsy Mimics
- Prominent dyskinesia
Cerebral Palsy Mimics
- Prominent dyskinesia
- DRD - Dopa-responsive dystonia
- GA1 - Glutaric aciduria Type I
- Cerebral folate deficiency
Cerebral palsy mimics
- Prominent ataxia
Cerebral palsy mimics
- Prominent ataxia
- PLP-1 dysmyelination (proteolipid protein 1)
- PMD - Pelizaeus-Merzbacher disease
- SPG2 - Spastic paraplegia type II - Angelman syndrome
- Chromosome 15 - Maple syrup urine disease
- Leucine, isoleucine, valine
Metabolic myopathies
- 3 Examples
Metabolic myopathies
- McArdle Disease
- Myophosphorylase deficiency - Pompe’s disease/Acid Maltase disease/Glycogen storage disease II
- Acid alpha-glucosidase deficiency - Mitochondrial metabolic myopathies
Intracranial cysts
- Epidemiology
- Pathophysiology
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
Corpus callosum agenesis
- Epidemiology
- Sequelae
- Mx
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
Febrile Seizure
- Epidemiology
- Mx
- Simple/complex
- Status
Febrile seizure
- Epidemiology
1. 6mo - 5yo
2. <3yo first presentation - Mx
1. Simple/complex - Anti-pyretic
+Anti-convulsant
- Status
- Neurology/ITU management
Febrile convulsion
- Simple vs Complex
Febrile convulsion
- Complex seizure
1. Focal
2. Prolonged - > 15 minutes
3. Multiple seizures - within 24 hours
Neonatal hypothermia
- S&S
Neonatal hypothermia
- S&S
- Respiratory distress
- BAT thermogenesis - Metabolic acidosis
- Hypoxia
- PPHN
— Persistent pulmonary HTN of newborn
Paeds Nerve Palsies
- Peripheral trauma
Paeds peripheral palsies
- Radial
- Supracondylar humeral fractures
- Humeral shaft fracture - Median
- Supracondylar fracture
- Elbow dislocation - Sciatic
- Accidental crush/laceration
- Buttock injection
- Breech delivery stretch - Common peroneal
- Fibular head
- Knee manipulations - Lumbar plexus
- Blunt abdo trauma
- Birth injury
Radial nerve injury
- Axilla vs Groove
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
Erb’s Palsy
- Branches
- Motor
- Sensory
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
Klumpke’s Palsy
- Roots
- Motor
- Sensory
Klumpke’s Palsy
- Roots
1. C8-T1
2 Ulnar and median peripherals - Motor
1. Intrinsic hand - Clawed hand
- Sensation
1. Medial upper limb - Dermatomal
