Neuro Flashcards
(121 cards)
1
Q
What is hydrocephalus
A
Enlargement of the cerebral ventricles due to excessive accumulation of CSF
2
Q
What are the 2 types of hydrocephalus and where are the blockages
A
Non communicating - obstruction in ventricles / aqueduct
Communicating - arachnoid villi (where CSF absorbed)
3
Q
Causes of non - communicating hydrocephalus
A
Brain tumour, inter ventricular haemorrhage, congenital malformation, dandy walker syndrome, Arnold - chiari malformation
4
Q
Causes of communicating hydrocephalus
A
sub arachnoid haemorrhage, tuberculous meningitis, duct stenosis
5
Q
Features of hydrocephalus
A
Head circumference - disproportionally large & rapid growing
Increased pressure on ant fontanelle - sutures separate, scalp veins prominent
6
Q
What can increased pressure in hydrocephalus lead to if untreated ? What’s this sign called
A
Eyes deviate downward - setting sun sign
7
Q
Features of hydrocephalus in older children
A
features of raised ICP
Headache, vomiting, respiratory abnormalities, pupil size / reaction, papilloedema, decerebrate (extended) / decorticate (flexed arms)
8
Q
Diagnosis of hydrocephalus
A
Uss - if sutures still open can assess level of ventricular dilation
CT/MRI - establish diagnosis and cause
9
Q
Treatment of hydrocephalus
A
Ventriculoperitoneal (VP) shunt
10
Q
Complications of vp shunts
A
Obstruction, infection
11
Q
What is craniosynostosis? Cause?
A
Premature fusion of skull sutures.
Part of syndrome in 30% - rest idiopathic
12
Q
Features of craniosynostosis
A
Abnormal head shape - fused sutures cause over expansion of other sutures to compensate
13
Q
Most common craniosynostosis and shape?
A
Sagittal -> long thin head
14
Q
Raised ICP in craniosynostosis treated with?
A
Cranioectomy
15
Q
Egs. Of neural tube defects
A
Spina bifida Meningocele Myelomeningocele Encephalocele Anencephaly
16
Q
What causes spina bifida occulta ? Sign?
A
Vertebral arch fails to fuse
Hairy patch on skin over birth
17
Q
What happens in meningocele
A
Meninges herniate through a vertebral defect -> outpouch
18
Q
Difference between myelomeningocele and menigocele
A
Myelo - meninges & spinal cord herniate
Most severe form of spinal dysplasia
19
Q
What is encephalocele
A
Extrusion of brain and meninges though a mid line skull defect
20
Q
What is anencephaly
A
Brain and cranium fail to develop (detected an antenatal USS & TOP usually performed )
21
Q
What is cerebral palsy
A
Disorder of motor function (movement +/- posture) due to a non-progressive static lesion of the developing brain
22
Q
Why does cerebral palsy evolve with time even though the lesson is static
A
Manifestations evolve as CNS develops - due to CNS plasticity in young children
23
Q
Does cerebral palsy just affect movement
A
No, often have other problems reflecting more widespread Brain damage
24
Q
When is most cerebral palsy caused? Egs?
A
Antenatal 80% - cerebral dysgeneis, congenital infections (rubella, cmv, toxoplasmosis)
25
Intrapartum cause of cerebral palsy
Birth asphyxia / hypoxia -> ischemic brain injury
26
Post natal causes of cerebral palsy
```
Preterm birth - interventricular haemorrhage, hypoxic-ischemic encephalopathy, periventricular leukomalacia
Hyper bilirubinaemia
Hypoglycaemia
Intracranial infections
Head trauma
```
27
Features of CP
Delayed motor milestones
Abnormal tone and posture
Asymmetric hand function before 1 year old
Feeding difficulties (decreased oromoter coordination)
Speech and language delay
28
Diagnosis of cp
Examination - tone 👆/👇, power (eg. Hemiparesis), reflexes (brisk -> UMN)(*prolonged primitive reflexes*) abnormal movements / posture / gait
29
When should primitive reflexes disappear
4-6/12
30
Classes of cp
Spastic -70%
Ataxic - 10%
Dyskinetic 10%
Mixed 10%
31
Where is damage for spastic cp? Associated with?
UMN (pyramidal / corticospinal)
| Brisk deep tendon reflexes & extensor plantar response
32
Why might hypotonia still be spastic cp
Turns spastic with age
33
What type of spastic cp is associated with birth asphyxia
Quadriplegic
| Other types are hemi/para
34
What does spasticity of bulbar muscles cause
Dysphasia and dribbling
35
What is the clasp knife response
Increased limb tone suddenly gives way under increasing pressure (UMN)
36
What causes ataxic cp
Damage to cerebellumans pathways
37
Early / late signs of ataxic cp
Early - *uncoordinated movements*, trunk and limb hypotonia, *delayed motor development*, poor balance
Late - intention tremor and ataxic gait
38
What causes dyskinetic cp? Other name?
Damage to basal ganglia / Extrapyramidal pathways (eg by kernicterus)
Choreoathetoid
39
What is causes kernicterus
Bilirubin
40
Presentation of dyskinetic cp
Hypotonia & delayed motor development
| Abnormal movements often don't appear until 12 months
41
Abnormal movements in dyskinetic cp
Chorea - abrupt, jerky
Athetosis - slow, writhing, continuous
Dystonia - sustained abnormal posture
42
Intellect impairment in dyskinetic cp
Completely / relatively unimpaired
43
Problems associated with cp
*mental retardation, opthalmic & auditory abnormalities, seizure, GOR, recurrent LRTI/pneumonia*
Feeding problems / failure to thrive
44
Members of MDT for cp
Paediatrician, carers / parents, gp, health visitor, SALT, physio, OT
45
Management of cp
Motor function - physio, muscle relaxants (hypertonia), Botox injections to specific muscles
Treat associated features eg. Epilepsy, constipation, malnutrition, behavioural
46
Eg of muscle relaxants for cp
Diazepam, baclofen
47
Definition of epilepsy
A chronic disorder of the brain characterised by recurrent, unprovoked seizures
48
Egs of epilepsy mimics (funny turns)
Breath holding attacks
Reflex anoxic seizures
Syncope
49
What is a breath holding attack? Demographic?
Provoked by temper / frustration in 6/12 - 6years
| Child holds breath, goes blue, then limb, then makes rapid recovery
50
What are triggers for reflex anoxic seizures?
Physiology?
What does it look like ? Possible ->?
Prognosis ?
Pain (usually mild head injury), fear, cold food.
Increased Vagal tone -> cardiac asystole.
Child becomes pale & falls to floor - hypoxia may cause GTC seizure.
Brief and child rapidly recovers
51
Usual triggers for syncope?
Prodromal Sx?
What happens?
Recovery?
Emotion / hot environment.
Nausea & dizziness
Loss of consciousness & posture
Rapid recovery
52
Egs of generalised seizures
Absence, myoclonic, clonic, tonic clonic, atonic
53
Difference between simple and complex partial seizures
Consciousness is retained in simple
54
Lobe in motor seizure
Frontal
55
Somatosensory / special sensory Sx seizure lobe?
Temporal (smells/ taste), parietal (sensations)
56
Usual lobe for complex partial seizure
Temporal
57
Usual cause of epilepsy
Idiopathic 75%
58
Egs of secondary causes of epilepsy
Cortical dysgenesis / malformations / tumours, neurocutaneous syndromes, downs, fragile X, inborn errors of metabolism (eg PKU), cerebral damage
59
Eg of neurocutaneous syndromes
Neurofibromatosis, tuberous sclerosis
60
Eg of causes od cerebral damage
Non accidental injury, birth asphyxia, hypoxia-ischemia following meningitis / encephalitis
61
Egs of generalised epilepsy syndromes
Infantile spasms (west syndrome), Lennox-gastaut syndrome, childhood absence epilepsy, juvenile myoclonic epilepsy
62
When are infantile spasms (west syndrome) most common? What happens in the seizures ?
```
4-6/12
Myoclonic seizures (salaam attacks) - violent flexion of head, trunk and limbs followed by extension of arms
```
63
What are infantile spasms often misdiagnosed as ?
Colic
64
Seen on EEG for infantile spasms ?
*hypsarrhythmia* - chaotic, large-amplitude slow waves with spikes & sharp waves
65
Usual cause of infantile spasms
Secondary - *tuberous sclerosis / hypoxic-ischemic encephalopathy*
66
Treatment for infantile spasms
ACTH
| Vigabatrin (TS)
67
Important side effect of vigabatrin
Permanent visual field defects
68
Usual age for Lennox-gastraut syndrome?
Usual seizure and frequency ?
Other key feature?
2-6 years.
Daily seizures - nocturnal tonic most common (90%), myoclonic drop attacks next.
Slowed / arrested psycho-motor development
69
Usual age for childhood absence epilepsy?
What are the seizures like?
How common are the seizures ?
4-12 years (peak at 6-7).
Transient 5-15s, - unaware blank spells without loss of body tone.
Can be up to several hundred / day
70
How can absence seizures be induced ? What is this useful for? What's seen ?
Hyperventilation for EEG.
| *Generalised bilaterally synchronous 3Hz (3/sec) spike and wave *
71
Juvenile myoclonic epilepsy presentation
After a GTC seizure, often with Hx of *clumsiness in morning * ("cornflake / toothbrush epilepsy")
72
What causes clumsiness in morning in juvenile myoclonic epilepsy
Brief myoclonic seizures usually after waking up
73
What can often progress into juvenile myoclonic epilepsy
Childhood absence epilepsy
74
Prognosis of Juvenile myoclonic epilepsy
Persist into adulthood - need to advise girls re pregnancy and medication
75
Non pharmacological Management of epilepsy
Supervision during baths , don't lock doors, wear cycle helmets, avoid climbing rocks / trees, supervision when swimming
76
Pharmacological management of epilepsy ? Generalised ? Partial?
Aim for monotherapy.
G- sodium valproate
P- carbamazepine
77
What should be used for girls of child bearing age with generalised epilepsy
Lamotrigine
78
What is a febrile seizure
Seizure associated with fever in a child (6/12 - 6 years) in the absence of intracranial infection or an identifiable neurological disorder
79
Features of febrile seizure
*Rapid increase in temp* -> brief (1-2 minute) *GTC* seizure
80
What is the usual cause of fever -> febrile seizures ? What is another cause and needs to be ruled out? How?
Viral infection
Bacterial meningitis
LP
81
When are febrile seizures more worrying
In the presence of developmental delay
82
Prognosis of febrile seizures
30% will have a recurrence - more likely if first seizure before 18/12
83
Management of febrile seizures
Septic screen - identify source
| Keep patient cool - antipyretics (paracetamol / ibuprofen), tepid sponging
84
What to be done in prolonged febrile seizure (>10mins)
Terminate using rectal diazepam
85
Which is more common , NF type 1 or 2
```
Type 1 (1/4000)
2 - 1/40000
```
86
Genetics of type 1
Autosomal dominant - 50% have no family Hx
87
Features of NF1 ? When seen?
*>6 cafe-au-lait spots* >5mm (before puberty
88
What characterises NF2 ? What does this lead to?
Bilateral acoustic neuroma -> deafness & sometimes cerebellopontine angle syndrome (CN VII palsy & Cerebellar ataxia)
89
Mutations for NF type 1 / 2
1- chromosome 17
| 2- chromosome 22
90
Genetics of tuberous sclerosis
Autosomal dominant - 75% new mutations
| 1/7000 births
91
Cutaneous features of TS
De-pigmented "ash leaf" shaped patches which *fluoresce under woods light*
Roughened patches of skin (*shagreen patches*) usually *over lumbar spine*
Facial *angiofibromas in butterfly distribution *
92
Neurological features of TS
West syndrome & developmental delay
Focal epilepsy
Intellectual impairment
93
What else is affected in TS
Multi system - heart, lungs, kidneys also
94
What type of syndrome is sturge - Weber syndrome
Neurocutaneous (like TS / NF)
95
Characteristic of sturge Weber syndrome
Unilateral facial naevus (*port wine stain*) in the distribution of the *opthalmic division of CN V*
Angiomas involving the leptomeningeal vessles in the brain (->seizures)
Haemangiomas in the spinal cord
96
Where are there abnormal blood vessles in sturge Weber syndrome and what do they cause?
Surface of the brain -> seizures, hemiplegia & learning difficulties.
Ocular involvement -> glaucoma
97
What's seen in CT head in sturge Weber syndrome
Calcification of gyri in characteristic *rail road track* pattern
98
Egs of muscular dystrophies
Duchenne, Becker, myotonic
99
Genetics of duchenne? How many new mutations? Gene codes for what?
X linked recessive (1/4000), 1/3 new mutations.
| Gene encodes *dystrophin* - sarcolemmal membrane protein
100
When does duchenne present? Do they ever walk ?
Sx at 2-4 years
| Independent walking tends to be delayed and these children never run
101
Features of duchenne
*pseudohypertrophy of calf muscles & proximal muscle weakness*
*positive Gowlers sign*
Scoliosis
Dilated cardiomyopathy
Mild learning difficulties
102
What is Gowlers sign
3-5 years - hands used to push on legs to achieve upright posture (weakness of lower back and pelvic griddle muscles)
103
*Diagnosis of duchennes *
Serum creatinine kinase (10-20x increased)
Muscle biopsy and EMG
DNA analysis to identify dystrophin gene mutations (+ve in 65%)
104
Management of duchennes ?
Supportive
Walking prolonged using orthoses
Scoliosis helped by truncal brace
Genetic counciling for female carriers
105
Prognosis of duchennes
Cardiomyopathy worsens with age -> death
106
What is Becker muscular dystrophy ? How does it compare to duchenne
Milder than duchenne but caused by a mutation in same gene.
| Onset is later (second decade) with prolonged survival
107
Genetics of myotonic dystrophy ? What does it affect?
Acquired autosomal dominant .
| Muscle, endocrine, cardiac function, CNS & immunity
108
Features of myotonic dystrophy
Hypotonia
Progressive muscle wasting
Typical facial features
Myotonia
109
Typical facial features in myotonic dystrophy
Inverted *v shaped upper lip*, thin cheeks, *high arched palate*
110
What is myotonia ? When is it seen?
Seen beyond 5 years
| Slow relaxation of muscle after contraction
111
How can you test myotonia
Ask to make tight fists then quickly open hands
112
Diagnosis of myotonic dystrophy
DNA -> CTG repeat
| Serum Creatine kinase usually normal (unlike duchennes)
113
Treatment of myotonic dystrophy
Supportive
114
What is spinal muscular atrophy ? Genetics?
Autosomal recessive.
| Degeneration of anterior horn cells -> progressive weakness & wasting of skeletal muscles
115
What's seen in spinal muscular atrophy
Wasting & weakness
Hypotonia and weak tendon reflexes
Fasiculations in tongue, deltoids / biceps -> characteristic & sign of denervation
116
What is ataxia ? Cause?
Incoordination of movement, speech and posture.
| Cerebellar / posterior sensory pathway problems
117
Egs of cause of Cerebellar ataxia
Acute - drugs / medications (alcohol and solvent abuse)
Post viral - usually after varicella infection
Posterior fossa lesions - medulloblastoma
Genetic / degenerative disorders - ataxic CP, freidrich ataxia, ataxia- telangiectasia
118
Cause of freidrichs ataxia ? How does it present ? Sensory impairment ?
Autosomal resseive mutation in frataxin gene .
*worsening ataxia, distal wasting in legs, absent lower limb reflexes* pes cavus & dysarthria.
Impairment of proprioception and vibrosence
119
What is ataxia-telangiectasia ? How does it present ?
Autosomal recessive disorder of DNA repair.
| Ataxia with developmental delay. Telangiectasia develop in the conjunctiva, neck and shoulders from 4 years.
120
What must be ? In acute strabismus
Raised ICP
121
What does no red light reflex indicate ? What needs to happen ?
Cataracts / retinoblastoma . Repair to avoid ambylopia in affected eye
