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