ILA- neuro Flashcards
What are the four fields of development?
Gross motor
Fine motor and vision
Hearing, speech and language
social
List the developmental milestones that you expect a child to have achieved by six months?
Sitting, rolls, rocks and can hold head up, sits tripod, postural reflex
Palmar grasp, pass from hand to hand
Babbling, turns to voice
Stranger anxiety, memory lasts 24 hrs, social smile
Puts things to mouth
List the developmental milestones that you expect a child to have achieved by twelve months?
Walking few unsteady steps with wide based gait/ cruising
Pincer grip, feeds with fingers, throws object
Knows 1 word other than mama or dada, understands no, understands simple command with action
Understands cause and effect and trail and error, peek a boo, waves bye and hello, imitates sounds, points at wanted item, use beaker to drink
What is the Moro reflex?
The Moro reflex is an infantile reflex that develops between 25–30 weeks of gestation and disappears between 3–6 months of age. It is a response to a sudden loss of support and involves three distinct components:
spreading out the arms (abduction)
pulling the arms in (adduction)
crying (usually)
What are the other primitive reflexes?
palmar 28 weeks to 2-3 months
Rooting 32 weeks to 1 month
Tonic neck reflex 35 weeks-7 months
Parachute reflex-7-8 mths - persists throughout life
What is the significance of the abnormal persistence of primitive reflexes?
Primitive reflexes are mediated by extrapyramidal functions, many of which are already present at birth. They are lost as the pyramidal tracts gain functionality with progressive myelination. They may reappear in adults or children with loss of function of the pyramidal system due to a variety of reasons
Sign of atypical neurology eg CP
Diagnosis: A six month old boy is referred because of concerns about his development. He smiled by six weeks of age. He now responds to noises and voices and is able to fix and follow. However, he still has poor control of his head and trunk and cannot sit even with support. He is able to grasp objects placed in his hand but doesn’t actively reach for toys or pass them from hand to hand. he has been difficult to feed and does have a tendency to cough and splutter. On examination he looks alert, and does appear to interact. However he has no head control with reduced tone centrally. His reflexes are slightly brisk. You are able to elicit a Moro reflex. his gross motor skills fail to progress and the peripheral tone in all four of his limbs increases. His upper limbs become more flexed, and his lower limbs extended. He is found to have a squint, and has been having episodes of abnormal movements thought to be seizures.
cerebral palsy
What type of cerebral palsy is this boy most likely to have?
Quadriplegic - a type of spastic CP
what are the Other types of cP?
dyskinetic/ athetoid (extrapyramidal- think like PD), ataxic (cerebellar)
What is cerebral palsy?
Non progressive brain lesion that affects tone and movement
Although the disorder causing cerebral palsy is not progressive, the symptoms and restriction of activities are often progressive and become worse with time. However, the symptoms of cerebral palsy range from mild to severe.
What causes cerebral palsy?
Damage to immature brain eg:
Congenital: premature, neonate encephalopathy most commonly due to hypoxic-ischaemic brain injury!!
sepsis
strctural maldevelopment
Most common cause used to be kernicterus due to hyperbilirubinaemia in rhesus disease
Are there any investigations that will confirm your diagnosis of CP?
clinical exam diagnoses but exclude differentials with:
Gene, metabolic, infection, imaging, neurophyxiology, histopathology, hearing and vision test
TFT, chromosome analysis, pyruvate and lactate (mitochondrial)
Organic and amino acid levels to exclude inborn errors of metabolism presenting with neurological symptoms.
Cerebrospinal fluid - protein, lactate and pyruvate levels may be helpful in determining whether there has been any asphyxia in the neonatal period.
CT/MRI to look at cause
EEG shows potentially hypoxic damage
What professionals are involved in the care of children with cerebral palsy?
PT, OT, SALT, nurse, dr, child development, dietician, opthamology, ENT, audiology, orthopaedics, education
When would botox be prescribed in children with cerebral palsy?
Botulinum toxin type A can be considered if focal spasticity is impeding fine motor function, compromising care and hygiene, causing pain, disturbing sleep, impeding tolerance of other treatments (such as orthoses) or causing cosmetic concerns to the child or young person.
Botulinum toxin type A treatment can also be considered if rapid-onset spasticity is causing postural or functional difficulties, or if focal dystonia is causing serious problems, such as postural or functional difficulties or pain.
A 6 year old girl is referred to the outpatient clinic because she is having repeated episodes of daydreaming at school. These have been occurring over at least the last year. There are no concerns about her development, although the school have noticed that her work has deteriorated since these episodes started. Examination is unremarkable; in particular neurological examination is normal. What differentials are there for her symptoms?
Absence seizure
Adhd
Hearing problem
daydreaming
How could you investigate her symptoms further?
EEG – may be normal, may see spike then bilateral wave
video seizure
How is absence seizure usually diagnosed?
The diagnosis is usually suggested by the history.
+ can trigger an absence seizure in over 9 in every 10 children with typical CAE. eg blow on windmill .
Could a delay in treatment of absence seizure be harmful?
Potentially – dangers of absence seizure eg in road
What is the first-line treatment of absence seizure?
Ethosuximide
Lamotrigine
Sodium valproate
Give two possible side effects of treatment for absence seizure.
Nausea and vomiting Headache Tired Diarrhoea Sodium valporate teratogenic and weight gain
Absence seizures stop. However she is referred again at twelve years of age following an uncomplicated generalized tonic-clonic seizure. As before, her behaviour and school work remain good. However, the parents do mention that she is quite clumsy in the morning. Is the clumsiness relevant? If so, then how?
Early morning sudden myoclonic jerks, especially of the arms and shoulders.
What is the diagnosis - TC seizure and clumsiness in morning?
Juvenile myoclonic epilepsy
How would you treat juvenile myoclonic epilpesy?
Sodium valporate - first line
Also lamotragine and levetiracetam
What is the prognosis of juvenile myoclonic epilpesy?
Spontaneous remission before puberty occurs in fewer than 20% of children with juvenile myoclonic epilepsy. Also drug withdrawal is often unsuccessful even after 2 or more years of seizure-freedom, with at least 70% of patients relapsing. Often lifelong treatment will be required to ensure seizure-freedom.
Do these two separate presentations of seizures have linkage?
juvenile myoclonic epilpesy often later develop generalised tonic-clonic (GTC) seizures.
What is the names of a squint where the eyes turned inwards vs outwards
Exotropia – eyes turn outwards
endotripia - inwards
causes of a strabismus ?
Convergence insufficiency is a common form of exotropia (treat with vision therapy)
cerebral palsy, Down’s syndrome (and other genetic disorders), hydrocephalus and space-occupying lesions are more likely to develop strabismus
Fhx, refractive error eg. far and long sighted and astigmatism, cataract, retinoblastoma, premature, neuro, mechanical
astigmatism – where the front of the eye is unevenly curved, causing blurred vision
What is convergence insufficiency?
an inability of the eyes to work together when used for near viewing, such as reading. Instead of the eyes focusing together on the near object, one deviates outward.
How would you treat convergence insufficiency ?
Eye exercises
Any concurrent ambylopia needs treatment with eye patching and drops
Correct refractive errors – glasses
if this fails then surgery - A combination of muscle recession (it is moved backwards on the globe and so its action is weakened) and antagonistic muscle resection (a segment of muscle is removed, so strengthening its action)
What is ambylopia?
Ambylopia = Partial or complete loss of visual acuity, aka lazy eye, usually in one eye, commonly due to refractive error, squint, cataract or ptosis
What could happen if you didn’t treat a strabismus?
Possible ambylopia / diplopia
What type of squint may require imaging of the head and orbits?
DO A SCAN IF a paralytic squint – look for nerve palsy or muscle/structure problem
CT scan of the head and orbit to assess for fractures or eye muscle changes due to thyroid disease
MRI scans of the head and orbits to determine whether the eye misalignment is caused by nerve or muscle problems or by inflammation.
What is the difference between primitive and postural reflexes?
Primitive reflexes (such as the Moro, grasp, and Galant) are normally present in the term infant and diminish over the next 4 to 6 months of life
Postural reflexes (such as the positive support reflex, Landau, lateral propping and parachute) emerge at 3 to 8 months of age.
Persistent primitive reflexes and lack of postural reflex development indicates UMN abnormality
Important features of a childs neuro hx and exam?
development
head circumfurence
Do somatic growth and skin search (look for café au lait spots or ash leaf lesions)
Dysmorphic features – look at face particularly midface
Fundoscopy
How may CP present?
Growth retardation may occur due to undernourishment or poor self-feeding skills
Poor nutrition means poor growth with reduced muscle strength, poor circulation and increased infection likelihood
Sx: abnormal posture of limbs or trunk, delayed motor milestones, feeding difficulties eg gagging and vomiting, poor gait, asymmetric hand function, primitive reflexes persist
What is the most common type of CP?
spastic
What are the different types of spastic CP?
Monoplegia: one limb affected
Hemiplegia: one side of the body affected
Diplegia: four limbs are affects, but mostly the legs
Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments