Neurology Flashcards
What is CP?
A group of disorders caused by a non progressive disturbance of the developing brain affecting movement and posture as well as activity limitations and associated disturbances.
What is the pathophysiology of organophosphate poisoning?
Acetylcholinesterase inhibitor - acetylcholine not broken down = increased parasympathetic sx = hypersalivation, bronchospasm, lacrimation, muscle weakness, headache, slurred speech, ataxia, confusion
How do you treat organophosphate poisoning?
Atropine to reverse cholinergic signs
Pralidoxime (cholinesterase reactivator)
What is the definition of microcephaly?
Less than two standard deviations below the mean for race, age and sex
What is the definition of microcephaly?
More than 2 standard deviations for race, age and sex.
How does cerebral palsy present?
> UMN signs = increased tone (spastic/rigid), increased reflexes, pathological reflexes
Developmental delay (motor/global)
+/- microcephaly
abnormal movements (dystonia)
What is the dilemma of imaging in CP patients?
The imaging can identify the point in development when the insult occurred, but it cannot indicate the CAUSE
What neurogenetic disorders mimic cerebral palsy?
Dyskinetic disorders
>mitochondrial disorders
>neurotransmitters
>glucose transport defects
>brain iron accumulation
>organic acid disorders
Spastic disorders
>hereditary spastic paraplegias
>lysosomal storage disorders
>disorders of forebrain cleavage
>migrational disorders
What are the general principles of the management of CP patients?
> Individualise
If a child sits independently before age 2, he will walk
Activity based rehab interventions induce neuroplasticity
Very early intervention, close to the time of incident, optimises neuroplasticity
90% of movement potential is achieved by 5years of age or less if severe
there is no single treatment that will allow a patient to jump from one level up to another
What are complications of cerebral palsy?
Pain
Constipation
Behaviour
Reflux
Epilepsy
Spasticity
What are the risk factors for developing CP?
Prenatal
>poor maternal health
>epilepsy
>genetic predisposition
>hydrocephalus
>ANC bleeding
Perinatal
>poor maternal health
>small for GA
>Premature
>HIE
>infections
Postnatal
>infections
>trauma
>environmental factors
>vascular (AVMs, strokes, thrombosis)
What are the signs of raised intracranial pressure?
> vomiting
headache
Diplopia
bulging fontanelle
papilloedema
diastasis of sutures
worse on lying down
worsened by coughing/sneezing
wakes child from sleep
What are the associated problems with CP?
Intellectual impairment
Learning disabilities
Epilepsy
Language disorders
Vision and hearing abnormalities
Behavioural problems
Malnutrition
What are some possible clinical features of cerebral palsy?
> hypertonic = fisting, leg scissoring, equines deformity
truncated hypotonia = asked head lag, opisthotonus due to excessive truncated and nuchal tone
brisk deep tendon reflexes = positive babinski reflex,
What is the differential diagnosis of a floppy infant?
> sick child
-cardiac
- malnutrition
-any acute illness
syndromic
neurological
-UMN - cerebral/cerebellar,
-LMN - muscular dystrophy, spinal muscular atrophy, NMJ
metabolic/hyperlaxity
What are the signs of UMN lesions?
Hyperreflexia
NOT weak
Evidence of CNS involvement (delayed milestones)
What are signs of LMN lesions?
Absent/decreased reflexes
Weak/decreased power
What is epilepsy?
> /= 2 unprovoked/reflex seizures >24hours apart
OR
One unprovoked seizure and a probability of >60% of further seizures over the next 10 years
OR
Diagnosis of an epilepsy syndrome
What is an epilepsy syndrome?
Seizure types + specific EEG patterns + brain imaging + genetic profile = cluster of features with recognisable pattern = determines further investigations + specific treatment
What is a febrile seizure?
Seizures in children between 6months and 5 years in association with fever but without evidence of intracranial infection0
How are febrile seizures classified?
Simple febrile seizures
Vs
Complex febrile seizures
What’s the difference between simple febrile seizures and complex febrile seizures?
Simple
>generalised tonic cloning
>lasts <15mins
>doesn’t recur within 24hrs
Complex
>focal
> usually >15mins
>cluster of 2+ in 24hrs
How do you manage febrile seizures?
Identify cause = exclude meningitis with LP
No CT/MRI/EEG warranted
Tx:
>antiepileptic drugs long term is NOT indicated unless complex = phenobarb
>rectal diazepam
>antipyretics
What factors does the drug of choice for epileptic seizures depend on?
> age
seizure type/syndrome
dosage according to weight
least side effects
dosage schedule
family and patient
availability
cost
What drug is first line tx for absent seizures?
Sodium valproate
What is the first line drug tx for generalised and focal seizures?
Carbamazepine
What are the contraindications for a LP?
> low platelets/coagulopathy (haematoma risk)
local infection
previous surgery in that area
raised ICP
haemodynamically unstable/resp compromise
decreased LOC <13
focal deficit eg unequal pupils
What positioning is needed for an LP?
Lateral recumbent position (spine perpendicular to bed)
OR
Fetal position (spine parallel to bed)
What layers do you go through when going an LP?
1)skin and subcut tissue
2)supraspinous ligament
3)interspinous ligament
4)ligamentum flavour
5)epidural space
6)dura
7)subarachnoid space
What tests would you do on the CSF?
1)measure opening pressure
2)dipstix
3)cell count
4)gram stain
5)capsular antigen
6)culture
7)biochemistry
8)other CSF specimen for virology
What are NORMAL CSF findings?
No neutrophils
0-6 lymphocytes
Protein 0,15-0,45
Glucose 3,6-5,6
What are the CSF findings in bacterial infections?
Predominantly neutrophils
Increased protein
Decreased glucose
Polymorphs
What are the CSF findings in viral infection?
Predominantly lymphocytes
Increased protein (less than bacterial)
Glucose within normal limits
What are the CSF findings of TB meningitis?
Predominantly lymphocytes
Grossly increased protein
Grossly decreased glucose (NB check CSF/serum glucose ratio)
What are complications of lumbar puncture?
Haematoma
Herniation
Pain
Post puncture headache
CSF leakage
Subarachnoid epidermal cysts
Cardioresp arrest
Infection
What are the steps to follow when called to casualty for a seizing child?
1) stabilise/resus (ABC)
2) quick history
3) examination
4) diagnostic tests
5) treatment
What is NB to remember to check when resusing a seizing child?
Check glucose!!
What do you ask on your quick history when you have a child seizing in casualty?
Acute onset?
>infection
>bleed
>stroke
>toxins
>seizures
>metabolic derangement
Subacute onset?
>brain tumor
>hydrocephalus
>inborn errors of metabolism
What do you look for in your examination of a child seizing in casualty?
Vitals
Intracranial pressure
Meninges irritation
Skull = signs of trauma/non-accidental injury
Seizure activity = tongue laceration/incontinence
Neuro exam = GCS, breathing pattern,eyes, posture, focal signs (tone, reflexes, brain stem reflexes, CN)
Rest of body clues
What breathing pattern indicates midbrain lesion?
Central neurogenic hyperventilation
What pattern of breathing indicates medullary lesion?
Ataxic breathing
What pupil reaction indicates lesion in midbrain?
Midposition, fixed
What pupil reaction indicates lesion in pons?
Pinpoint
How do you treat raised intracranial pressure?
Head upright
Restrict fluids
Normocarbia
Mannitol
Specific cause
>meningitis = antibiotics/antivirals
>SOL = neurosurgery
>ingestion = remove toxin
>status = see protocol
How do you treat a child <1/neonate who has status epilepticus?
1) phenobarbital 20mg/kg
2)phenytoin 20mg/kg if previous step failed (NOT VALPROATE)
3) thiopentone OR midazolam infusion
How do you treat a child more than 1y old with status epilepticus?
1) lorazepam 0,1mg/kg IVI OR diazepam 0,3-0,5 mg/kg IV or PR
Repeat x1
2) phenytoin 20mg/kg in SALINE 20-30min infusion to avoid cardiac arrest OR valproate 20mg/kg over 5mins (NOT IF CHILD YOUNGER 2y)
3) ICU, EEG, INTUBATE. thiopentone infusion (keep on infusion until 48hrs seizure free) OR midazolam 0,3mg/kg loading dose then 0,1-0,4mg/kg/hr
What are red flag signs for autism?
12 months = no babbling, no pointing/waving
16 months = no single words
24 months = no spontaneous 2 word phrases
Any stage = loss of language or social skills
What developmental milestones should be met at 3 months?
GM: lifts head in prone
FM: Hands open, follow 180 degrees
Coms: coo
What developmental milestones should be met at 6 months?
GM: Roll over both ways, sit with support, braces shoulders when pulls to sit
FM: transfer hand to hand, palmar grasp
Comms: babbles
Social: express emotion, takes everything to mouth
What developmental milestones should be met at 12 months?
GM: bear walks, stands independently
FM: release objects on request, throws, places cubes in container
Comms: nouns “where’s mommy”, knows name, knows one word phrases
Social: finger eats
What developmental milestones should be met at 2 years?
GM: jumps, kicks ball, walk upstairs both feet on step, runs
FM: 6 cube tower, circle shapes, hand preference, match puzzle shapes, turn multiple pages
Comms: names one body part, points at 7body parts, knows pronouns, verbs “what do you eat with”
Social: can dress, possessive
What developmental milestones should be met at 3 years?
GM: walks up stairs individually
FM: 9/bridge, copies circle, draw straight line, can use scissors, turn individual pages
Comms: adjective negative “which one is red” “which one is NOT the animal”, knows name and gender, can count to 10
Social: uses fork, empathy, bowel trained, interactive and cooperative play
What developmental milestones should be met at 4 years?
GM: jumps on one foot, hopscotch, walk down stairs individually
FM: 12blocks/step, copy a cross, draw person with head, arms and legs
Comms: complex instruction plan, knows age, address, points at colours, knows 4 numbers
Social: handles buttons, bladder trained, washes hands, make believe play
What development milestones do you expect in a 5 year old?
GM: walk in straight line
FM: draw square, draw triangle, cut along lines
Comms: knows 10 numbers, recites alphabet
Social: uses knife, chooses friends, does laces
What are red flag signs in a child with a headache?
> signs of raised ICP
signs of meninges irritation
neurological deficits/seizures
described as “worst headache ever” -subarachnoid haemorrhage
What are the clinical signs of cerebellar dysfunction?
> ataxia
nystagmus
intention tremor
dysarthria
dysdiadocokinesis
past pointing
What is friedrichs ataxia?
A progressive neurodegenerative disorder (AR) causing progressive damage to your nervous system and movement problems
What is ataxic telangiectasia?
It is a complex multi system disorder characterised by progressive neurological impairment, cerebellar ataxia, immunodeficiency, impaired organ maturation, ocular and skin telangiectasia and predisposition to malignancy.
