Neurodevelopment Flashcards
Autism red flag signs
12 months
- no babbling
- no pointing or waving
- no single words
- no spontaneous 2 word phrases
- any loss of any language or social skills
Side effects of Ritalin
headache and stomachache
suppression of appetite
can cause insomnia
hearing defects need to be referred first
Epilepsy
enduring predisposition to generate seizure with neurological cognitive psychological and social consequences
- at least 2 unprovoked / reflex seizures > 24 hours apart
- one unprovoked/ reflex seizure + a probability >/= 60% of further seizures over the next 10 year
- diagnosis of an epilepsy syndrome
recurrent unprovoked seizures
Treatment of epilepsy
First line
absence - sodium valproate
focal and generalised TC - carbamazepine
Side effects of drugs
Phenobarbitone - increases hyperactivity
Valproate - liver toxicity
Carbamazepine - exacerbates absence and myoclonic seizures
Phenytoin - worsen MG, headache, N and V
Lamotrogine - N/V
Benzodiazepines - induce T/C seizures in LGS
Status epilepticus
seizures for 30 minutes or remains unconscious between seizures
DONT wait 30 min before treating
start on flow chart if fitting for 10 min
- Maintain vital function - ABC
- Stop convulsions - drugs
- IV line
- draw blood
- give bolus glucose
- see the two tables for the drugs to be given - Determine the cause
- Prevent more convulsions
Step 1 = Lorazepam (Ativan) or Diazepam (Valium)
Step 2 = Phenytoin (Epanutin) or Sodium Valproate (Epilim) - 20mg/kg
Step 3 = Thiopentone infusion or Midazolam or Propofol
Neonate treatment
Step 1 = phenobarbitone or lorazepam
Step 2 = phenytoin - NO VALPROATE
Step 3 = thiopentone or midazolam infusion
Febrile seizures
6m-3-4(5) years
fever without evidence of intracranial infection
average age onset 18 to 22 months
boys more girls
1/3 have at least one recurrence
2% risk developing epilepsy
fever can come after seizure
simple - GTC, < 15min, no recurrence in 24hr
complex - focal, > 15min, cluster 2 or more within 24hr
Management febrile seizures
identify underlying disease - LP CT/ MRI - NOT simple routine EEG seldom necessary LT use AED not indicated unless complex - phenobarbitone or sodium valproate rectal diazepam antipyretics?
Contraindication to LP
decreased level of consciousness Glascow < 13
focal deficit - unequal pupils
too sick - haemodynamically unstable or respiratory compromise
septicaemia with petechiae or purpura
low platelets - bleeding disorder
local infection
relative CI - increased ICP
absolutely CI if following is seen on CT - midline shift, loss of cisterns, mass in post fossa
BUT
do blood cultures and start treatment
Clinical signs of raised ICP
Papilloedema
decreased LOC
pushing reflex
CSF findings
bacterial - predominantly neutrophils
increased protein decreased glucose
viral - predominantly lymphocytes
mildly increased protein WNL glucose
TBM - predominantly lymphocytes -
severely increased protein
decreased glucose
Causes of aseptic meningitis
Partially treated meningitis TBM viral meningitis leukaemia uncommon infections - syphillis - mycoplasma - toxoplasmosis
Coma
Unarousable for at least 1 hour total unawareness with closed eyes lack of wakefulness lack of movement noxious stimuli - inappropriate responses
Persistent vegetative state
wakeful unconsciousness diagnosed 1 month after onset of coma
or a period of at least 3 months is required in a baby younger than 6 months
sleep and wake cycles are present
brainstem function and spinal reflexes are present
no cortical fx
autonomic fx is preserved
may shed tears
Complications of TBM
Communicating hydrocephalus Brain abscess Brain oedema SIADH Convulsions Subdural effusions Deafness and blindness Learning problems
Tics
most common movement disorder in children
sudden twitches, movements or sounds
motor, vocal or sensory
involuntary but CAN be suppressed
aggravated by stress/anxiety/fatigue
provisional tic disorder - motor or vocal tics less 12 months in a row
persistent or chronic tic disorder - motor or vocal tics for more than a year
Tourette syndrome - at least 2 motor and at least one vocal tic for at least a year
treatment rarely needed - can give haloperidol or risperidone
PANDAS
Paediatric Autoimmune Neuropsychiatric Disorder Associated with Strep infection tics and dystonia post GABHS infections auto-antibody at basal ganglia - prepubertal - tics or OCD - sudden onset/fluctuating course - Associated with GABHS infection - neurological abn
Developmental regression
SSPE AIDS Anti-epileptic drugs Corticosteroids SLE Sturge Weber TBM Sickle cell Huntington Spinocerebellar ataxia HAS CHILD LOST ANY SKILL THAT HE HAD PREVIOUSLY ACQUIRED? IT IS NEVER OK TO LOSE ANY PREVIOUS MILESTONES RECOGNISE AND REFER
How does the neural system develop?
Neurulation Prosencephalization Neuronal proliferation Migration Formation of gyri Organization Myelination
Name preconceptual risk factors to neurodevelopment
Poor maternal health
Epilepsy
Genetic predisposition
Name antenatal risk factors to neurodevelopment
Poor maternal health
Toxins
Infections
Name postnatal risk factors to neurodevelopment
Environmental factors
Infections
Trauma
Discuss complications of an issue in each neurodevelopmental stage
Neuralation - NTD
Prosencephalization - holoprosencephaly spectrum (Dandy walker, hydrocephaly)
Hindbrain - cerebellar + rhombencephalic abnormalities
Neuronal proliferation - microcephaly, macrocephaly
Migration - gyral formation (schizencephaly, lisencephaly, pachygyria) , heterotopia
Organization (autism, schizophrenia, depression)
Myelination (dysmyelination, demyelination)
What is the spectrum of holoprosencephaly?
Alobar
Semilobar
Lobar
Middle interhemispheric variant (MIHV)
Name causes of microcephaly
AR (microcephaly vera) AD X-linked recessive Teratogenic Syndromic Perinatal hypoxia Intrauterine infections Chromosomal abnormalities Familial Severe metabolic disorders
Name causes of macrocephaly
Isolated macrocephaly Assoc growth disturbance Neurocutaneous syndrome Chromosomal abnormalities Hemimegalencephaly Hydrocephalus Storage disorder SOL Familial Sotos syndrome
Give examples of neurocutaneous syndromes
Neurofibromatosis
Sturge Weber
What is Dandy Walker syndrome?
A brain malformation that occurs during embryonic development of the cerebellum and 4th ventricle
How are neurons organized?
Differentiation Alignment Orientation Layering Synaptogenesis Cell death Glial proliferation
What is a common disorder of myelination?
Periventricular leukomalacia in spastic diplegia
What is your approach to a neurodevelopmental examination?
History General impressions Higher functions Head and face Cranial nerves Neck and back Signs of ICP Motor system Sensory system Basal ganglia Cerebellar function Autonomic system Markers Developmental assessment
What are neurodevelopmental warning signs?
- Inconsolable crying
- Bruxism
- Hand admiration after 20w
- Everything to mouth after 9m
- Purposeless throwing of toys after 9m
- Self-stimulating behaviour
- Self-injurious behaviour
What should you look at for higher functions?
Sensorium Behaviour Mood Thought content Communication and speech Intellectual ability
Name head shapes and their causes
Plagiocephaly
Scaphocephaly
Brachycephaly
What kinds of fontanels can you have?
Late closure
Bulging
Tense
Sunken
What causes a tense fontanel?
Raised ICP
Name causes of late anterior fontanel closure
Hydrocephalus T21 Hypothyroidism Rickets Skeletal dysplasia
What causes overriding sutures?
Craniosynostosis
Name myopathic facial features
Restricted eye movements
Long thin face
Drooping mouth
Lack of expression
What are you looking for on the back in a neuro examination?
Spinal dysraphism
Scoliosis
Kyphosis
Lordosis
Name causes of meningeal irritation
Meningitis
SAH
Apical pneumonia
Pyelonephritis
What are the signs of raised ICP?
Headache Vomiting Diplopia Bulging fontanel Papilloedema McEwan cracked pot Cushing response Sun setting sign
What are you looking for concerning gait in a neuro examination?
Broad vs narrow based Toe/flat foot walking Foot eversion Limping Hemiplegia (circumduction) Ataxic Waddling Slapping
Explain a diplegic gait and who presents with one?
Adduction of the legs
Flexion of the wrists
CP patients
Explain a waddling gait and who presents with one?
Issue with pelvic stabilising muscles
Myopathic gait
What is a neuropathic gait also known as?
Equine gait
Name signs of proximal upper limb weakness
Shoulder falls through
Cannot wheelbarrow walk
Cannot press against wall
Name signs of distal upper limb weakness
Cannot pull self up when held by hands
Name signs of proximal lower limb weakness
Gower’s
Cannot climb stairs
Name signs of distal lower limb weakness
Cannot stand on toes
What is the sensory innervation of the lateral surface of the arm?
C5
What is the sensory innervation of the tip of the thumb?
C6
What is the sensory innervation of the web of the index finger?
C7
What is the sensory innervation of the tip of the little finger?
C8
What is the sensory innervation of medial surface of the lower arm?
T1
What is the sensory innervation of the medial surface of the upper arm?
T2
What is the sensory innervation of the inguinal ligament?
L1
What is the sensory innervation of the middle of the anterior thigh?
L2
What is the sensory innervation of the medial aspect of the knee?
L3
What is the sensory innervation of the medial aspect of the calf?
L4
What is the sensory innervation of the lateral aspect of the calf?
L5
What is the sensory innervation of the sole of the foot?
S1
What is the sensory innervation of the axilla?
T3
What is the sensory innervation of the lower edge of ribs and xiphisternum?
T8
What is the sensory innervation of the skin of the umbilicus?
T10
What is the sensory innervation of the skin of the pubis?
T12
What do you look for in the legs in cerebellar exam?
Dysmetria
Heel on shin
Pendullar reflexes
What do you look for in the trunk in cerebellar exam?
Ataxia
What do you look for in the eyes in cerebellar exam?
Nystagmus
Opsoclonus
What do you look for in the arms in cerebellar exam?
Dysmetria
Intention tremor
Finger-nose test
Dysdiadochokinesia
What do you look for in the speech in the cerebellar exam?
Slow
Slurred
How do you assess autonomic dysfunction in neuro exam?
Sphincter control
BP
Sweating
Horner’s
What disease markers should you look for?
Neurofibromatosis
Sturge Weber
Tuberous sclerosis
Hypomelanosis
How can you assess development?
Gross motor = GMFCS Fine motor = MACS Hearing and language = CFCS Cognition = DQ and IQ Psychosocial/emotional = behaviour
Name signs of visual impairment
Nystagmus Strabismus Clumsiness Does not reach out Does not fixate at 4mo Photophobia Rubbing of eyes Regression in school work
Give an approach to abnormal movements
Slow
- dystonia
- athetosis
Fast 1. Stereotyped Rhythmic - tremor - stereotypies Non-rhythmic - tics 2. Non stereotyped - chorea - myoclonus
Where is a tic most common?
Face (central musculature)
How do you diagnose Tourette syndrome?
At least 2 motor and 1 vocal tic >1 year
How do you describe a tremor?
Rapid
Rhythmical
Frequency and amplitude
What is important about stereotypies?
Can be distracted
How do you describe chorea?
Arrhythmical, asymmetrical
Sudden, random movements
What are 2 signs of chorea?
Milkmaid grip
Jack in the box tongue
What must you do for a patient with Sydenham’s chorea?
Cardiac imaging
Penicillin prophylaxis
How would you describe dystonia?
Involuntary sustained/intermittent muscle contractions using twisting or reptitive movements
Can be painful
Name causes of dystonia
Primary
- genetic (primary torsion dystonia)
Secondary
- metabolic
- drug induced
- brain injury
How can you identify that a dystonia is primary?
Imaging is normal
What are the main features of cerebellar pathology?
Ataxic gait Abnormal head posture Ocular motor dysfunction Hypotonia Dysarthria Intention tremor Dysmetria
Name causes of ataxia
Acute
- cerebellitis
- intoxication
- tumour
Chronic non-progressive
- ataxic CP
- congenital malformation
- prematurity
Chronic progressive
- spinocerebellar ataxia (SCA)
- ataxia telangiectasia
- Friedrich’s ataxia
What is the most common cause of acute ataxia seen in children?
Post varicella cerebellitis
What is the most common cause of chronic progressive ataxia seen in children?
Ataxia telangiectasia
Discuss approach to LMN examination
Gait Pull up Shoulder girdle Tone Reflexes - remember ankle and compare sides Power - eliminate gravity if needed - assess feet in 4 directions Motor Tongue
Discuss the neurological examination of the newborn
Response to light Response to sound Move head to assess eye CN Rooting Arm traction Arm recoil Scarf sign Flex hips Leg traction Leg recoil Popliteal angle Heel to ear Neck tone Head lag Head control Prone Ventral suspension Vertical suspension DTR Moro reflex Plantar reflex Galant reflex Stepping Fontanelles Sutures
What must you remember to check in the 6month old?
Assym neck tone
Landau reflex
Lateral prop
Synostosis of which suture causes scaphocephaly?
Sagittal
Synostosis of which suture causes brachycephaly?
Coronal
When is clonus in a 3mo normal?
Few beats = normal
Sustained = abnormal
When does rooting reflex integrate?
4mo
When does Moro reflex integrate? What does it indicate if it has not integrated by this time?
5mo
UMN lesion
When does galant reflex integrate?
4mo
When does plantar grasp reflex integrate?
6-12mo
When does palmar grasp reflex integrate?
4-5mo
When does ATN reflex integrate? What does it indicate if it has not integrated by this time?
6mo
UMN lesion
What does asymmetry with arm recoil indicate?
Erb’s palsy
What does consistent fisting >3mo indicate?
UMN lesion
Why is Babinski normal in children <1y?
Incomplete myelination
What is creeping?
Crawling but with trunk off the ground