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Sarah Paeds > Neuro > Flashcards

Neuro Flashcards

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1
Q

West syndrome

A 
Infantile spasms 
Intellectual disability (deteriorating social interaction)
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2
Q

5 types generalised epilepsy

A

Absence
Atonic - loss of muscle tone and sudden fall to floor
Tonic - increased tone all over
Myotonic - repetitive jerky movements of limbs, head, trunk
GTC - tonic, followed by rhythmical contraction of muscle groups

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3
Q

Ix for hydrocephalus

A

Measuring HC
CrUSS
CT/MRI

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4
Q

Features of a migraine with aura

A

Lasts a few hours
Children go to lie down in a dark room and sleep

Genetic predisposition

Triggers - reduced stress, certain foods

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5
Q

NF1 features

A 
Cafe au lait spots (6+)
2+ neurofibroma 
Axillary freckles 
Optic glioma 
Lisch nodule (harmatoma of iris)
Bony lesions 
Fhx (first degree)
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6
Q

Origin of neurocutaneous syndromes

A

Nervous system and skin have common ectodermal origin. Embryological disruption = problems in both systems

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7
Q

Non-AED treatment for epilepsy

A

Surgery - hemispherotomy
Ketogenic diets
Vagal nerve stimulation

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8
Q

Muscular dystrophies

A

Inherited disorders with muscular degeneration. Often progressive

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9
Q

Features of a generalised tonic clonic seizure

A

Loss of tone followed by rhythmical contraction of muscle groups.

Lasts seconds to minutes

Can become cyanotic (hold breath)

Drooling saliva,
Bitten tongue
Incontinence

Post ictal deep sleep

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10
Q

Medical mx epilepsy

A

AED - monotherapy starting at the lowest effective dose

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11
Q

Side effects carbamazepine

A

Rash, neutropenia, hyponatraemia, interference with other medication

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12
Q

Mx of breath holding attacks

A

Distraction and behavioural techniques

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13
Q

Ddx seizures

A

Epilepsy

Non-epileptic:

  • Febrile convulsion
  • Breath holding attack
  • Reflex anoxic
  • West Syndrome
  • Hypoglycaemia
  • Hypocalcaemia
  • Meningitis
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14
Q

Features of tuberous sclerosis

A

Ash leaf patches - depigmented macules
Shargreen patches - rough areas over lumbar spine
Adenoma sebaceum - angiofibromata in butterfly distribution

Infantile spasms, developmental delay often with autistic features, focal epilepsy, intellectual impairment.

Subungual fibromata
Rhabdomyomara
Polycytic kidneys

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15
Q

Diagnosis myasthaenia gravis

A

Anti-AChR Abs

Anti-MuSK

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16
Q

NF2 features

A

Bilateral acoustic neuroma

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17
Q

Is cerebral palsy progressive?

A

No (although things appear to change as child grows)

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18
Q

Mx CP saliva

A

SLT
Anticholinergics - hyoscine hydrobromide
Botox into salivary glands

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19
Q

Difference between Duchenne’s and Becker’s

A

Some functional dystrophin is produced in Becker’s, unlike DMD.
Later onset and slower progression of disease in Becker’s

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20
Q

Ix West syndrome

A

EEG - hypsarrythmia

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21
Q

Commonest type focal seizure

A

Temporal

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22
Q

Two examples inflammatory myopathies

A

Dermatomyositis

Myotonic dystrophy

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23
Q

Two broad categories of epilepsy

A

Generalised

Focal

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24
Q

Commonest cause of motor impairment in children

A

CP

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25
Which type of CP does PVL typically predipose to?
Spastic diplegia (legs>arms)
26
Ix in child with seizures and developmental delay
Metabolic screen
27
Life expectancy Becker's MD
Late 40s
28
Red flags for other neuro conditions (not CP)
o Absence of risk factors o Family history of progressive neurological disorder oLoss of already attained cognitive or developmental abilities o Development of unexpected focal neurological signs o MRI findings suggestive of progressive neurological disorder o MRI findings not in keeping with CP
29
Age presentation febrile convulsions
6mos-3yrs
30
Side effects gabapentin
Insomnia
31
Genetic abnormality in DMD
Deletion of Xp21 = mutation in dystrophin gene
32
Mx CP sleep
Sleep hygiene, | Melatonin
33
Mx CP eating and drinking
SLT assessment Thickened fluids Postural management Optimise nutrition Video fluoroscopy if necessary
34
Two broad categories of motor disorders
Central | Peripheral
35
Average age of diagnosis DMD
5.5 yrs
36
3 types of CP
Spastic Dyskinetic Ataxic
37
Features of dermatomyositis
Fever, misery Symmetrical, proximal muscle weakness Helioptrope rash Gottren's nodules
38
Mx headaches
Assess the impact on daily life EXCLUDE RED FLAGS Rescue treatment - analgesia - anti-emetics - triptans (nasal) Prophylactic treatment - topiramate (Na channel blocker) - beta blockers - tricyclics - acupuncture Psychosocial suport - identifying stressors - relaxation techniques
39
Rescue therapy epilepsy
Buccal midazolam for seizures lasting more than 5 minutes
40
Advice for parents for febrile convulsion
``` Do not restrain child Protect from injury Do not put anything in their mouth Check airway Recovery position once stops Medical advice if >5 mins ```
41
Tuberous sclerosis management
Laser therapy for ash leaf patches and shargreen patches AEDs for epilepsy Anti-arrhythmics for cardiac involvement anti-HTNs for PCKD
42
Features of spasms in West syndrome
Violent flexor spasms of head, trunk, limbs followed by extensor spasms of the arms
43
Features reflex anoxic episode
Trigger eg. fear, cold, head trauma Child goes pale and falls to floor Hypoxia may induce a GTC Fast recovery
44
Commonest muscular dystrophy
DMD
45
Side effects lamotrigine
Rash
46
Features of motor cortex disorder
Loss of fine finger movements Brisk hyperreflexia Extended plantars
47
Ix epilepsy
History (?triggers) EEG - asymmetry, hyperexcitation MRI (if focal neuro signs) - structural and functional Metabolic screen if developmental delay
48
Status epilepticus
Seizure lasting more than 5 minutes
49
Function of dystrophin (absent/mutated in MD)
Connects cytoskeleton to ECM
50
West syndrome prognosis
Poor - loss of skills, learning disabilities and continuing epilepsy
51
Prevalence of epilepsy in children
0.5%
52
Ix dermatomyositis
Bloods: Raised ESR, CRP | Muscle biopsy: inflammatory infiltrations/atrophy
53
Tuberous sclerosis inheritance patterm
AD
54
Which type of migraine has a genetic predisposition?
With aura
55
What % of children suffer from febrile convulsions?
3%
56
Cr USS finding predisposes to CP
Periventricular leukomalacia (HIE)
57
Delayed motor milestones in CP
Not sitting unsupported by 8 mos Not walking before 18 mos Hand preference before 1 yr
58
Mx status epilepticus (5 steps)
1) ABCDE. Secure airway, check hypoglycaemia, O2. 2) IV lorazepam. 3) If no response after 10 mins, given second dose IV lorazepam 4) Phenytoin infusion 20mg/kg for 20 mins (phenobarbitone if already on phenytoin) 5) RSI
59
SOL presentation
``` Morning/wake up headaches Change in mood, personality, school performance, Visual field changes Cranial nerve abnormalities Abnormal gait Growth failure ```
60
Which surgeries may be appropriate in muscular dystrophy
Tendoachilles lengthening and scoliosis surgery
61
AED for focal seizures
Carbamazepine | Lamotrigine
62
Inheritance pattern neurofibromatosis 1
AD - highly penetrant
63
When can children come off AED
2 years seizure free
64
Features of temporal focal seizure
Warning/aura, lip-smacking/plucking/automations. Impaired consciousness
65
Mx if febrile convulsion lasts longer than 5 min
Call ambulance Measure blood glucose Bucal midazolam/ PR diazepam
66
Age presentation West Sydrome
4-6 months
67
What is neurofibromatosis associated with?
MEN, phaeo, renal artery stenosis, HTN, gliomatous change
68
Initial mx epilepsy
Refer all patients to neurologist (first fit clinic) Specialist epilepsy nurse Record future seizures Start a diary INFORM SCHOOL
69
Pathophysiology of muscular dystrophy
Reduced dystrophin Reduced intracellular firing = reduced calmodulin = increased free radicals = necrosis = muscle destruction and release of CK
70
Causes of CP
80% antenatal 10% HIE 10% postnatal
71
Migraine ddx
Abdominal migraine Cyclical vomiting BPPV
72
What % of childhood migraines are with aura?
10%
73
Example NMJ dysfx disorder
Myasthenia gravis
74
Features of CP
Abnormal limb/trunk posture Delayed motor milestones Slowed head growth Feeding difficulties due to locomotor coordination Abnormal gait (scissoring, tip toes) Asymmetric hand function
75
Example central motor disorder
Cerebral palsy
76
Epilepsy
Recurrent, unprovoked seizures - transient signs and symptoms of abnormal/asynchrous/excessive firing of neurons
77
4 types focal seizure
Frontal - motor cortex with clonic/atonic features Temporal - warning/aura, lip-smacking/plucking/automations. Impaired consciousness Parietal - impaired sensation Occipital - visual distortion
78
Two examples peripheral neuropathies
GBS | Facial nerve palsies e.g. Bell's
79
4 types neurocutaneous syndromes
NF1 NF2 Tuberous sclerosis Sturge-Weber
80
Cerebral palsy
Abnormality of movement and posture disturbance
81
Life expectancy of DMD
Late 20s | Eventual resp failure
82
Presenting features DMD
Waddling gait Gower sign Language delay Pseudohypertrophy of calves (muscle replaced with fat/fibrous tissue)
83
Mx CP speech, language, comms
SLT Posture control, breath control, Alternative communication - comms boards, pictures, etc.
84
Age presentation dermatomyositis
5-10 years
85
Mx CP pain
Reducig regimen of paracetamol
86
Pathophysiology reflex anoxic episode
Cardiac asystole from vagal inhibition - trigger
87
Features of hydrocephalus
Increases HC centiles Bulging fontanelle Sun-setting gaze (fixed-downward) Distended scalp veins
88
4 types of peripheral neuro disorders and their features
Anterior horn cell - weakness, hyporeflexia, fasciculations Neuropathy - weakness, reduced sensation NMJ dysfunction - progressive weakness Myopathy - proximal weakness, wasting, abnormal gait,
89
Causes of postnatal CP
Kernicterus, meningitis/encephalitis, hypoglycaemia
90
Advice for parents epilepsy
Parents/carers - how to recognise a seizure Record future seizures Start a diary Avoid dangerous activities (e.g. swimming until seizures under control)
91
Average age wheelchair dependence DMD
10-14 yrs
92
Ix for DMD
CK Muscle biopsy DNA USS/MRI
93
Mx hydrocephalus
Ventriculo-peritoneal shunt | Ventriculostomy
94
What % of childhood migraines are without aura?
90%
95
Causes of secondary epilepsy
Infection, HIE, tumour, trauma, | Neurodegenerative disorder
96
Average age onset Becker's MD
11 years
97
Mx myelomeningocele
Surgical repair | Physiotherapy
98
Mx neurofibromatosis
Medical - regular r/v ophtalmology, BP, skeletal complications) Surgery - laser removal of nodules
99
Inheritance pattern DMD
XR
100
Features of myelomeningocoele
Meninges protrude with cord. Paralysis of legs, sensory loss, bladder/bowel dennervation, hydrocephalus
101
Causes of hydrocephalus
Increased CSF production CSF flow obstruction Inhibited CSF reabsorption = ventricular dilatation
102
Mx CP bones
Ca, vit D, phosphate | Non-weight bearing
103
Example of anterior horn cell disease
Spinal muscular atrophy
104
Side effects sodium valproate
Weight gain, hair loss, liver failure
105
Features of nocturnal hypoxia
Daytime headache Irritability Reduced feed
106
Features myotonic dystrophy
Hypotonia | Feeding/resp difficulty
107
Features of Sturge-Weber syndrome
Port-wine stain in trigeminal region (V2) Similar intracranial lesion Haemangiomatous
108
Examples peripheral motor disorder
``` Neuromuscular Anterior horn cell Neuropathy NMJ Myopathy ```
109
Mx dermatomyositis
Physiotherapy to reduce risk of contractures Steroids Methotrexate
110
Features of GBS
2-3 weeks after GI infection (e.g. campylobacter) Ascending, symmetrical muscle weakness Increased protein in CSF
111
Three types of primary headache
Tension Migraine Cluster
112
Members of MDT CP
``` Paediatrician Nurse Physio OT SLT Dietetics Psych ```
113
Mx West syndrome
Prednisolone | Vigabatrin
114
Limitation of using EEG as ix for epilepsy
Need to catch the seizure
115
AED for generalised seizures
Sodium valproate Keppra
116
Two categories of muscule disorders
Muscular dystrophies | Inflammatory myopathies
117
When does the neural plate fuse?
Within 28 days of conception
118
Features of basal ganglia disorder
Dystonia Dyskinesia Difficulty initiating movements Chorea
119
Mx of DMD
Physiotherapy to help prevent contractures Exercise Psych support CPAP (weakened ICM lead to nocturnal hypoxia) Surgery - tendoachilles lengthening and scoliosis surgery Prednisolone (delay wheelchair dependence) Cardioprotective drugs if LVEF drops
120
Mx myotonic dystrophy
``` Physiotherapy OT (utensils) Ortho (foot drop) SLT - E,D, speech Pain - gabapentin/NSAIDs Mixeletine - for myotonia ```
121
Inheritance pattern myotonic dystrophy
AD - nucleotide triplet expansion, with anticipation thru generations
122
Three categories of headache
Primary (tension, migraine, cluster, other) Secondary (Raised ICP, trauma, infection) Neuralgias
123
Two types of migraine
w/ aura | w/o aura
124
When do febrile convulsions result in an increased risk of later epilepsy
If they are prolonged and/or recurrent
125
Mx absence seiures
- aimed at maximsing education potential and supporting social development.
126
Features of a migraine without aura
Lasts 1-72 hours Pulsatile over the temporal/parietal region Unpleasant GI disturbance - Nausea, vomiting, pain Photophobia Worsened by physical activity
127
What is the decision to treat epilepsy based on?
Risk of recurrence Impact on life How dangerous/impairing seizures are
128
What are common triggers for a migraine with aura?
Reduced stress | Certain foods
129
What % of epilepsy is idiopathic?
70-80%
130
What proportion of West syndrome have underlying neuro cause?
2/3
131
When appropriate to r/f febrile convulsion to paediatrician
``` If <18 mos Diagnostic uncertainty over cause Seizure lasted >15 mins Focal features during seizure Incomplete recovery after 1 hour ```
132
Mx CP muscle stiffness
Baclofen/diazepam
133
Mx myasthaenia gravis
Neostygmine Prednisolone Azathioprine

Sarah Paeds (20 decks)

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