ILA- neuro Flashcards

(94 cards)

1
Q

What are the four fields of development?

A

Gross motor
Fine motor and vision
Hearing, speech and language
social

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2
Q

List the developmental milestones that you expect a child to have achieved by six months?

A

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

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3
Q

List the developmental milestones that you expect a child to have achieved by twelve months?

A

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

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4
Q

What is the Moro reflex?

A

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)

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5
Q

What are the other primitive reflexes?

A

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

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6
Q

What is the significance of the abnormal persistence of primitive reflexes?

A

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

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7
Q

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.

A

cerebral palsy

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8
Q

What type of cerebral palsy is this boy most likely to have?

A

Quadriplegic - a type of spastic CP

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9
Q

what are the Other types of cP?

A

dyskinetic/ athetoid (extrapyramidal- think like PD), ataxic (cerebellar)

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10
Q

What is cerebral palsy?

A

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.

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11
Q

What causes cerebral palsy?

A

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

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12
Q

Are there any investigations that will confirm your diagnosis of CP?

A

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

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13
Q

What professionals are involved in the care of children with cerebral palsy?

A

PT, OT, SALT, nurse, dr, child development, dietician, opthamology, ENT, audiology, orthopaedics, education

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14
Q

When would botox be prescribed in children with cerebral palsy?

A

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.

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15
Q

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?

A

Absence seizure
Adhd
Hearing problem
daydreaming

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16
Q

How could you investigate her symptoms further?

A

EEG – may be normal, may see spike then bilateral wave

video seizure

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17
Q

How is absence seizure usually diagnosed?

A

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 .

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18
Q

Could a delay in treatment of absence seizure be harmful?

A

Potentially – dangers of absence seizure eg in road

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19
Q

What is the first-line treatment of absence seizure?

A

Ethosuximide
Lamotrigine
Sodium valproate

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20
Q

Give two possible side effects of treatment for absence seizure.

A
Nausea and vomiting 
Headache 
Tired 
Diarrhoea 
Sodium valporate teratogenic and weight gain
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21
Q

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?

A

Early morning sudden myoclonic jerks, especially of the arms and shoulders.

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22
Q

What is the diagnosis - TC seizure and clumsiness in morning?

A

Juvenile myoclonic epilepsy

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23
Q

How would you treat juvenile myoclonic epilpesy?

A

Sodium valporate - first line

Also lamotragine and levetiracetam

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24
Q

What is the prognosis of juvenile myoclonic epilpesy?

A

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.

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25
Do these two separate presentations of seizures have linkage?
juvenile myoclonic epilpesy often later develop generalised tonic-clonic (GTC) seizures.
26
What is the names of a squint where the eyes turned inwards vs outwards
Exotropia – eyes turn outwards | endotripia - inwards
27
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
28
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.
29
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)
30
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
31
What could happen if you didn’t treat a strabismus?
Possible ambylopia / diplopia
32
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.
33
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
34
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
35
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
36
What is the most common type of CP?
spastic
37
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
38
What causes the spasticity in CP?
Spastic CP: damage to UMN (pyramidal/ corticospinal tract) causing spasticity (constantly increased tone – velocity dependent – increased the faster is pulled), increased reflexes, tremor and weakness
39
What is hemiplegic CP?
Unilateral/ hemiplegia: Initially may be floppy before affected sign becomes spastic. arm>leg, fisting of one hand, flexed and pronated arm, tiptoe walk on affected side – looks like a stroke, cerebral artery affected
40
What is quadriplegic CP?
Bilateral/ quadriplegia: all four limbs, trunk extensor posturing, poor head control
41
What is diplegic CP?
Bilateral/ diplegia: legs affected much more than arms so arms appear normal. get scissoring or the legs from excessive adduction of the hips, crouch gait when older
42
What is dyskinesia?
chorea (irregular and non-repetitive movements), athetosis (slow writhing distal movements eg fan fingers), dystonia (agonist and antagonist muscles contract simultaneously and appears twisty)
43
mx of CP?
Mx: PT, OT, botox, baclofen, diazepam, deep brain stimulation, selective dorsal rhizotomy
44
neuro differentials for TLOC in child
syncope – eyes rolled back, jerking, stiffening NEA- Arms pat, head shakes, hip thrust Benign neonate sleep myoclonia – jerking in babies sleep Cataplexy Febrile seizure – fever with no intracranial problem,Fhx, brief TC, 6 months to 6 yrs,
45
list the generalised vs focal seizures?
Generalised: absence, myoclonic, tonic, atonic, tonic clonic Local: frontal/ occipital/ parietal/ temporal
46
difference between simple and complex seizures?
Simple (retains conscious) vs complex (not conscious)
47
describe childhood absence seizure?
lasts few seconds, brief staring, can fiddle, multiple times a day
48
list other eg of childhood epilepsies?
infantile spasms; Lennox-gastaut ; Benign rolandic; Juvenile myoclonic epilepsy:
49
Describe infantile spasms?
This is also known as West syndrome. starting in infancy at around 6 months of age. It is characterised by clusters of full myoclonuc body spasms that occur upon waking up. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free. It can be difficult to treat but first line treatments are: Prednisolone Vigabatrin
50
Describe Lennox-Gastaut syndrome?
1-3 yrs, multiple seizures, usually have learning disabilities, may have drop attack (atonic) seizure
51
What is Benign Rolandic epilepsy?
``` aka benign focal epilepsy. 4-10 yrs occurs in sleep/ near sleep facial twitching and aphasia TC ```
52
Describe Juvenile myoclonic epilepsy.
effects teens Early morning sudden myoclonic jerks, especially of the arms and shoulders. Often later develop generalised tonic-clonic (GTC) seizures. May be inherited as autosomal dominant.
53
how does a headache caused by SOL present?
SOL- raised ICP, worse lie down and morning, vision defects, CNS defects- abducens nerve is usually affected first causing a squint and loss of abduction
54
How do migraines present in children? what is the mx?
most common headache in children Migraine – usually without aura. Bilateral, pulsatile, N+V, photo and phonophobia, worse with activity. Aura can include hemianopia, scotoma, spectra (zigzags), Possible Mx: NSAIDs, antiemtics (cyclizine), triptans. Prophylaxis (beta blockers, sodium channel blockers eg valproate, tricyclics
55
What is the childhood version of MND?
Spinal muscular atrophy | it is a type of anterior horn disease
56
What is the other cause of anterior horn disease?
polio
57
how does anterior horn cell disease present?
progessive weakness, loss reflexes, fasiculation, wasting due to denervation, hypotonia, areflexia, milestone delay, abnormal gait
58
what are the signs of myopathies?
proximal muscle weakness, gower’s sign, pes cavus, gait distrubed, wasting
59
What may cause a myopathy?
inherited eg duchennes, beckers | acquired eg polymyositis, dermatomyositis, endocrine and rheumatological causes
60
What ix are done for myopathies?
``` myopathy: plasma creatine kinase for duchennes and becker dystrophy and inflammaotry myopathies. Muscle biopsy DNA test – genes US muscle for progress ```
61
describe duchennes dystrophy
Dystrophies – Duchenne’s - X linked recessive – motor and speech delay, do stairs one by one, gower’s sign (use hands to climb up body when getting up). Manage with PT, if get nocturnal hypoxia need CPAP, give corticosteroids to prevent scoliosis
62
What is an example of neuromuscular junction disorders and the key sx?
myasthenia gravis Neuromuscular junction – fatigueability as run out of Ach stores Usually present ptosis first, have difficulty chewing. Check for thyoma.
63
ix for neuropathy?
Nerve conduction studies DNA test Nerve biopsy EMG
64
How do neuropathies present and what are examples of these?
weakness, pain/ temperature, loss reflexes | eg GBS, bells palsy, charcot marie tooth
65
What is more minor duchennes or beckers dystrophy?
beckers
66
How does dermatomyositis present?
fever, muscle weakness (as with all myopathies), rash on eyelids, periorbital oedema, calcinosis. Treat with corticosteroids
67
Describe Charcot marie tooth disease
– symmetrical slow progressive wasting, inherited, present tripping and foot drop,, motor and sensory neuropathy
68
describe GBS and its mx
- ascending and progressive symmetrical weakness, loss reflexes, give IV immunoglobulins if severe
69
describe Bells palsy and its mx
– paresis of CN 7 post viral – give acyclovir, steroids in first week can reduce
70
mx of Myasthenia gravis .
Give anticholinesterase drugs eg Pyridostigmine. Identify acetylcholine receptor antibodies. Plasma exchange if in crisis.
71
What is the most common form of inherited ataxia?
Friederichs - autosomal recessive – ataxia, wasting, absent reflexes, loss of proprioception and vibration sense.
72
WHat is the most common type of brain haemorrhage in children and how does it present?
Subdural haematoma | Caused by tearing of the bridging veins as they cross the subdural space – associated with trauma or shaking in children
73
What are the three types of spinal birth defect? summarise them?
Spina bifida occulta – failure of fusion of vertebral arch – may have birthmark/ patch hair in lumbar region Meningocele –sac outside but not cord or nerve- fixed with surgery Myelomeningocele – worst, nerves and spinal cord visible- associated with paresis and sensory loss, bowel and bladder issues, scoliosis
74
How are neural tube defects decreased?
Decreased with screening and folic acid
75
What is hydrocephalus? What causes it?
Accumulation of CSF in brain Causes: obstruction of ventricles –non- communicating problem eg congenital, haemorrhage, neoplasm. Or failure to reabsorb CSF at arachnoid vili – communicating problem eg meningitis, SAH
76
What are the sx of Hydrocephalus
Sx: HC disproportionately large, separation of skull sutures, bulging anterior fontanelle, distended scalp veins, when older get sx of raised ICP
77
mx of Hydrocephalus
Treat with a ventriculoperitoneal shunt
78
What is NF1?
a neurocutaneous disorder AD condition 2 or 3 of the following criteria: Present: 6 or more café au lait spots reater than 5mm before puberty or greater than 15mm after puberty; more than one neurofibroma (nodular growth on nerve), axillary freckling, optic glioma, lisch nodule, bony lesions from sphenoid dysplasia, Fhx first degree relative Associated with visual problems, endocrine problems and tumours
79
What is the difference between a paralytic and non-paralytic strabismus?
Non paralytic/ concomitant squint = usually due to refractive error which is corrected via glasses – normally convergent Paralytic = rare, motor nerves paraysed, be suspicious of sinister cause --> diplopia is maximal when attempting to look in the direction requiring the action of the weak muscle the image from the paralysed eye is always peripheral to the image from the normal eye the angle between the longitudinal axies of the eyes varies during testing of eye movements
80
What is a strabismus?
misalignment visual axis
81
What are the different types of strabismus?
Esophoria - latent convergent - inwards Exophoria - latent divergent -outwards Hyperphoria-latent up Hypophoria - latent down
82
What is the most common type of strabismus?
esophoria (moves inwards on testing - unlike esotropia which is visible w/o tests)
83
What is latent deviation?
Eyes look straight ahead normally but when cover one eye deviates
84
How may latent deviation present?
headaches and intermittent Diplopoda
85
What are the primary causes of strabismus?
``` transient misalignment (resolves itself) Fhx Refractive error – commonly uncorrected hypermetropia (long sighted – fixed with convex lens) and myopia (short sightedness) ```
86
What are secondary causes of strabismus?
``` Secondary to loss of vision Neuro cause - need to rule out eg CP, acute onset esotropia , SOL Febrile illness Craniofacial synostosis Cataracts retinoblastoma ```
87
What should be looked for in a hx and exam of strabismus?
HISTORY: is it constant? What age? Forcep delivery? Fhx of cataracts? Detection of a squint may be made by the corneal light reflection test - holding a light source 30cm from the child's face to see if the light reflects symmetrically on the pupils Cover test-identifies nature ask the child to focus on an object -> cover one eye -> observe movement of uncovered eye -> cover other eye and repeat test, do far and near as some squints appear at different points Visual acuity
88
What is intermittent and pseudostrabismus?
Intermittent - lose control and have strabismus at certain distances Pseudostrabismus - looks malaligned but is just epicanthal folds or may be deep set or prominent eye, asymmetrical faces or unilateral ptosis etc as tests are normal
89
What are the management options for a strabismus?
Conservative – glasses, prisms, orthoptic exercises to improve control over eye muscles Surgery: Strengthening procedure – resection Weakening procedure – recession of the muscle on the side the eye goes towards Esotropia (eye pointing inwards) – strengthen lateral rectus by resection, recession of the medial rectus Exotropia – opposite Botox injections – paralyse the muscle that is pulling the eye in a certain direction, last 4-6 months
90
What are the different types of ambylopia?
Strabismus (especially constant unilateral) Anisometropic Ametropic Meridianal Stimulus deprivation
91
Why does strabismus sometimes cause ambylopia?
This is because different images from each eye results in one eye being switched off to avoid double vision
92
What are the Ix for ambylopia?
refraction (glasses test), fundus and media check Ocular movements to check muscle strength (exclude paralytic strabismus eg nerve palsy 7 or 4 – moebius syndrome)
93
What is the mx for ambylopia?
treat early remove underlying cause if poss eg cataracts glasses (refractive adaptation for refractive errors), atropine drops to dilate the pupil and paralyse accommodation (makes dominant eye blurry- alt to eye patch) occlude better seeing eye which forces lazy eye to work and develop better vision (if ocular dominance)
94
What are the complications of CP?
``` Pain S+L epilepsy vision bladder sleep hearing DDH ```