Neurology pediatric T_T

• patient presented with hx of seizure then reoccurred again currently stable , what is the next step to order?

  EEG
• Rolandic seizure

  Benign epilepsy with centrotemporal Spikes

  • no Neuroglial deficit
  • Occurs at night or at time of waking up
  • resolve spontaneously at age of 13 years old
  • EEG --> Centrotemporal Biphasic Sharp waves
  • Simple partial seizure involving face and oropharynx --> Facial drop , dropping of mouth and drooling of saliva + Aphasia
  • no need for treatment
• Absence seizure :

  start suddenly and end suddenly

  lasts < 30 seconds

  No Post ictal
• Complex Parietal

  Preceded by warning

  post ictal phase

  lasts > 30 seconds

  followed by loss of consciousness
• EEG of absence seizure

  3-4 Hx spikes and slow wave complex
• Febrile seizure :

  6 months - 6 years old

  no underlying CNS lesion

  treatment --> paracetamol
• Basal skill fracture :

  Anterior --> Raccoon's eyes , halo sign , partial hearing loss or smell , restricted eye movement

  Middle --> Carotid artery injury , hearing loss , loss of balance , Battle sign ( bruise behind the ear )

  Posterior --> Spinal cord injury , vertebral artery injury and lower cranial nerve injury
• Risk of future febrile seizures

  strong Fmx

  another seizure within the first 24 hours

  Febrile seizure at low grade fever 38

  Hyponatremia or hypocalcemia at time of presentation
• Simple Vs complex Febrile Seizure

  Simple :

  lasts < 15 min

  generalized

  once per 24 hour

  complex

  > 15 min

  Focal

  More than once within 24 hours
• Night terror :

  agitation and screaming --> Yes

  Sleeping wave --> slow

  Fmx --> yes

  recalling the events --> no

  Incidence --> Common 10%
• Night walking

  agitation and screaming --> no

  Sleeping wave --> slow

  Fmx --> yes

  recalling the events --> no

  Incidence --> Common 5%
• Nightmares :

  agitation and screaming --> Yes

  Sleeping wave --> repetitive

  Fmx --> yes

  recalling the events --> Yes

  Incidence --> Common
• sudden onset of squint , next step is --> Brain CT
• • Kernicterus --> choreoathetosis
  • Neonatal Stroke --> Double hemiplegia
  • Meningomyeloceles --> Paraplegia
  • Preterm Babies --> Diplegia
• Decrease risk of Brain damage and CP in Low apgar score ?

  Hypothermia
• Risk factors for CP:

  Antenatal :

  • Prematurity and low birth weight
  • Intrauterine infection
  • multiple gestation
  • Pregnancy complications

  Perinatal :

  • birth asphyxia

  Post natal :

  • trauma
  • meningitis
• Prevention of CP during pregnancy:

  MG

  antibiotics

  corticosteroids
• Diplegia --> 4 limbs --> lower more than upper

  monoplegia --> one limb

  Quadriplegia --> 4 limbs

  Double hemiplegia --> 4 limbs --> upper more than lower
• Most common cause of CP:

  Hypoxia
• Commonest risk factor of CP:

  Preterm
• Rett's syndrome :

  Abnormal head growth

  weird hand movement --? wringing

  Repetitive language skills

  Lost of interest
• Freidreich Ataxia:

  Ataxia

  foot deformity

  iron accumulation in mitochondria

  Nystagmus
• most common CNS complication after Chicken pox:

  without neck stiffness --> acute cerebral ataxia

  with neck stiffness --> Meningoencephalitis
• Infantile spasm

  Myoclonic sudden jerky movement

  respond to Steroids

  EEG --> hyperthymia

  treatment --> Carbamazepine
• Diagnosis of Neurofibromatosis

  2 out of 7

  • 6 or more cafe latu spots --> 5 mm in young children and 15 mm in older children
  • Axillary freckles more than 2
  • Optic nerve Glioma
  • Iris Hamartomas --> 2 or more
  • positive gene NF1
• Family hx of NF1 + one of the criteria confirms diagnosis
• Cafe latu + Fmx --> reassurance

  Multiple Cafe Latu ---> Genetic testing

  CONIFRMED Positive Fmx and NF1 --> genetic counseling
• Neurofibromatosis type 1 located ?

  Chromosome 17q
• Tuberous sclerosis

  M--> Macules --> cafe latu spots

  R --> Rhabdomyoma

  S --> Shagren batch

  A --> adenoma Sebacium

  F--> fibroma in nail bed

  D--> dominant

  A--> Ash lead Macules

  R--> renal cyst
• Tuberous sclerosis

  2 genes --> chromosome 9 and 16
• Fragile X syndrome

  Large forehead

  long face

  large ears and jaws

  Mental retardation --> most common cause of inhered mental retardation

  Macro orchidism

  same as Marfan syndrome
• Fatigued neonates +not moving eyes from light ?

  Infantile botulism
• Initial test in Duchene muscular dystrophy "

  CK levels
• Dunchene Muscular dystrophy

  • more in males
  • x linked disease --> 50% females will be carriers / 50% males will be affected
  • proximal muscle weakness ( progressive ) + positive Gower sign
  • Cardiopathy
• Myotonic dystrophy

  Autosomal Dominant

  heart block

  Distal muscle weakness
• Anterior horn cells

  sensory changes --> no

  cranial nerves --> affected

  Muscles --> proximal and distal parts equally

  day and night --> no
• repetitive eye movement + otherwise healthy between attacks

  Tics
• Repetitive eye movement +Multiple voice tics

  Tourette syndrome
• when should examine red eye reflex

  at 6 weeks
• 6 weeks old + absent red reflex what is the appropriate management ?

  Examination under anesthesia