seizures for 30 minutes or remains unconscious between seizures DONT wait 30 min before treating start on flow chart if fitting for 10 min 1. Maintain vital function - ABC 2. Stop convulsions - drugs - IV line - draw blood - give bolus glucose - see the two tables for the drugs to be given 3. Determine the cause 4. 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

Neurodevelopment Flashcards by Rachel Glass

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

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Side effects of Ritalin

headache and stomachache
suppression of appetite
can cause insomnia
hearing defects need to be referred first

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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

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Treatment of epilepsy

First line
absence - sodium valproate
focal and generalised TC - carbamazepine

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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

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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

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Neonate treatment

Step 1 = phenobarbitone or lorazepam
Step 2 = phenytoin - NO VALPROATE
Step 3 = thiopentone or midazolam infusion

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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

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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?

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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

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Clinical signs of raised ICP

Papilloedema
decreased LOC
pushing reflex

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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

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Causes of aseptic meningitis

Partially treated meningitis 
TBM 
viral meningitis 
leukaemia 
uncommon infections 
- syphillis 
- mycoplasma 
- toxoplasmosis

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Coma

Unarousable for at least 1 hour 
total unawareness with closed eyes 
lack of wakefulness 
lack of movement 
noxious stimuli - inappropriate responses

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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

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Complications of TBM

Communicating hydrocephalus 
Brain abscess 
Brain oedema 
SIADH 
Convulsions 
Subdural effusions 
Deafness and blindness 
Learning problems

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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

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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

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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

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How does the neural system develop?

Neurulation
Prosencephalization
Neuronal proliferation
Migration
Formation of gyri 
Organization
Myelination

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Name preconceptual risk factors to neurodevelopment

Poor maternal health
Epilepsy
Genetic predisposition

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Name antenatal risk factors to neurodevelopment

Poor maternal health
Toxins
Infections

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Name postnatal risk factors to neurodevelopment

Environmental factors
Infections
Trauma

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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)

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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?

1. Inconsolable crying 2. Bruxism 3. Hand admiration after 20w 4. Everything to mouth after 9m 5. Purposeless throwing of toys after 9m 6. Self-stimulating behaviour 7. 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?

What is the sensory innervation of the tip of the thumb?

What is the sensory innervation of the web of the index finger?

What is the sensory innervation of the tip of the little finger?

What is the sensory innervation of medial surface of the lower arm?

What is the sensory innervation of the medial surface of the upper arm?

What is the sensory innervation of the inguinal ligament?

What is the sensory innervation of the middle of the anterior thigh?

What is the sensory innervation of the medial aspect of the knee?

What is the sensory innervation of the medial aspect of the calf?

What is the sensory innervation of the lateral aspect of the calf?

What is the sensory innervation of the sole of the foot?

What is the sensory innervation of the axilla?

What is the sensory innervation of the lower edge of ribs and xiphisternum?

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