Neurology Flashcards
1
Q
Define Ataxia
A
Abnormality in gait that is wide based/staggering/unsteady
2
Q
Give three causes of Ataxia in children
A
Posterior fossa tumours
Brainstem Encephalitis
Friedreich’s Ataxia
3
Q
Give four other clinical features that might be associated with a presentation of Ataxia
A
Increased separation of speech syllables
Abnormal Proprioception
Positive Romberg
Nystagmus
4
Q
How could you investigate Ataxia in a child?
A
Cerebral Imaging
Plasma and CSF samples (particularly for Varicella, Strep, and Inborn Errors of Metabolism)
5
Q
What is Chorea? Give four causes
A
Jerk like movements that may involve face/arms/legs
Drugs (anticonvulsants), SLE, Sydenhams Chorea, Benign Familial
6
Q
What is Sydenham’s Chorea?
A
Chorea often associated with streptococcal infection, occurring in older children
Child is normally well
20% Rheumatic Fever
7
Q
How is Syndehams Chorea managed?
A
High dose Pen V then daily prophylaxis
If IEM is excluded, then start Sodium Valproate
Can give Benzodiazepines/Haloperidol as symptom management
8
Q
What is PANDAS?
A
Paediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus
9
Q
How does PANDAS present?
A
OCD
Tics
Irritability
Anxiety
10
Q
Define Athetosis
A
Sinuous, slow, involuntary writhing movements affecting fingers/hands/toes/feet
11
Q
Name three causes of Athetosis
A
Asphyxia
Neonatal Jaundice
Trauma
12
Q
How is Athetosis managed?
A
Diazepam/Haloperidol
Surgery and restraining technique
13
Q
What is Primary Pure Dystonia?
A
Autosomal Dominant dystonic spasms of legs on walking
Progresses to whole body within ten years
14
Q
What are Tics?
A
Repetitive stereotyped movements that can be initiated voluntarily and also suppressed voluntarily
Can be simple (twitch in same site, occurring in 25%) or complex (multiple tics, associated with tourettes)
15
Q
What could the term ‘floppy’ mean?
A
Decrease in muscle tone
Decrease in muscle power
Ligamentous laxity/increased ROM
16
Q
Define Hypotonia
A
Low resistance to passive stretch around the joint
17
Q
What is Phasic Tone?
A
Response of muscles to rapid stretch
18
Q
What is Postural Tone?
A
Response to sustained low intensity stretch
19
Q
What in their antenatal history would you want to know about a ‘Floppy Infant’?
A
Reduced foetal movements
Polyhydramnios
Breech
20
Q
What in their family history would you want to know about a ‘Floppy Infant’?
A
Muscle Disease
Stillbirth
Consanguinity
21
Q
What in their birth history would you want to know about a ‘Floppy Infant’?
A
Duration of labour Method of delivery Rescucitation Apgar Score Cord Gases
22
Q
What associated features might you see in a ‘Floppy Infant’?
A
Poor swallowing
Weak Cry
Paradoxical Breathing (intercostal muscles paralysed but intact diaphragm)
23
Q
If the cause of the ‘Floppy Infant’ was central/UMN, how would it present?
A
Poor truncal tone
Normal reflexes
Loud cry
24
Q
If the cause of the ‘Floppy Infant’ was Peripheral/LMN, how would it present?
A
Frog like posture
Reduced/Absent reflexes
Weak Cry
25
Describe three central causes of the ‘Floppy Infant’
Dysgenesis - Downs Syndrome, Prader Willi
Degeneration - Metabolic Disorders, Enzyme Disorders (eg Tay Sachs)
Encephalopathy (HIE, ICH, Congenital or Acquired infections)
26
Give 5 classes of non central causes of the ‘Floppy Infant’
Spinal Cord (Syringomyelia, Birth Trauma)
Anterior Horn (SMA, Poliomyelitis)
Peripheral Nerve (Guillaine Barre, Hereditary - Charcot Marie Tooth)
Neuromuscular Junction (Neonatal Myasthenia Gravis, Infantile Botulism)
Muscle (Muscular Dystrophy, Metabolic Myopathies, Congenital Myopathies)
27
How can you investigate central causes of the ‘Floppy Infant’?
```
Range of bloods
Sepsis screen
Plasma Amino Acids
Cranial Ultrasound and MRI
Microarray
LP
```
28
How can you investigate peripheral causes of the ‘Floppy Infant’?
CK
Muscle Biopsy
Microarray
Conduction studies
29
Name 7 causes of headaches in children
```
Tension
Migraines
ENT Infection
Vision Problems
Raised ICP
Meningitis
Brain Tumours
```
30
How do young children with tension headaches present?
Non Specific
Quiet
Stop Playing
Turn Pale
31
How do older children with tension headaches present?
Mild ache across forehead in band like pattern
Gradual onset and resolution with no pulsing/visual changes
32
Migraines are a complex condition that causes headache attacks and other symptoms. Name five different types.
```
Migraine without aura
Migraine with aura
Silent migraine
Hemiplegic
Abdominal
```
33
How do Migraines present?
Unilateral, throbbing headache
Associated - visual aura, photophobia, nausea and vomiting
34
If Migraines are impacting on QoL, what drugs can you prevent them with?
Propanolol (avoided in Asthma)
Pizotifen (Drowsiness)
Topirimate (Teratogenic - COCP required)
35
Abdominal Migraines may be the start of traditional migraines developing when they’re older. How do they present?
Episodes of central abdominal pain lasting for more than one hour, with associated Nausea and Vomiting/Anorexia/Headache/Pallor
36
How are Abdominal Migraines managed?
Pizotifen, Sumatriptans
Avoid Caffiene/Cheese/Marmite
37
Give three causes of Focal Seizures
Cortical Dysplasia
Trauma
Tumour
38
Temporal lobe is the most common type of focal epilepsy. How can it present?
Odd Smell/Sensation
Deja Vu
Repetitive Automatisms
39
How do Frontal Lobe Seizures present?
- Often night waking
- Thrashing and Bicycle Movements
- Jacksonian March (movements travelling proximally)
- Tonic arms raised in air
40
How do Occipital Lobe Seizures present?
Often mistaken for migraines
Flickering light, Visual Hallucinations
41
How do Parietal Lobe tumours present?
Tingling in hands/feet
Body image distortion
42
Generalised seizures occur in both hemispheres. How does an Absence Seizure present?
Generally starts between age of 4-7 and resolves in adolescence
<10seconds unresponsive
Occasional Automatisms
Triggered by hyperventilation
43
Generalised seizures occur in both hemispheres. How does an Myoclonic Seizures present?
Jerking Movements with retained conscious
Child may require helmet
44
What is Progressive Myoclonic Epilepsy?
Seizures + Unsteadiness+ Rigidity + Mental Deterioration
45
Ohtahara Syndrome is a cause of seizures in the first 28d of life. How does it present?
Primary tonic seizures occuring in the first two weeks
Caused by metabolic disorders or brain damage
Often die within weeks/months
46
Benign Familial Seizures is a cause of seizures in the first 28d of life. How does it present?
Recurrent seizures in newborns lasting one to two minutes
| Usually stops at four months old
47
Give two causes of INFANTILE seizures
West Syndrome (Jerking followed by stiffening, often due to Hypoxic birth injury)
Dravet Syndrome (Sodium Channel Mutation)
48
Give four Neurological causes of Seizures
Rett Syndrome
Angelman Syndrome
Tuberous Sclerosis
Sturge Weber (Port Wine Stain)
49
Define Epilepsy
Umbrella term for condition where there is a tendency to have seizures (transient episodes of abnormal electrical activity)
50
How does a Generalised Tonic Clonic Seizure present?
Loss of consciousness then tonic clonic movements
Associated tongue biting, incontinence, irregular breathing
Post Ictal - Prolonged drowsiness, confusion, irritability
51
How are Generalised Tonic Clonic seizures managed?
1st line - Sodium Valproate
| 2nd line - Lamotrigine or Carbemazepine
52
How are Focal Seizures managed?
1st line - Lamotrigine/Carbemazepine
2nd line - Sodium Valproate
IE opposite to generalised tonic Clonic
53
How are Absence Seizures managed?
Typically grow out of them
1st line - Sodium Valproate or Ethosuxamide
54
What are Atonic Seziures?
AKA Drop Attacks
Brief lapses in muscle tone, normally not lasting longer than 3 minutes
No post ictal drowsiness
55
Describe the typical EEG of childhood absence seizures
3Hz spike and wave
56
How are Myoclonic Seizures managed?
First line - Sodium Valproate
| Second line - Lamotrigine/Topirimate
57
How is Infantile Spasms/West Syndrome managed?
Prednisolone and Vigabatrin
58
How is Epilepsy investigated?
EEG (after SECOND tonic Clonic seizure)
Bloods/LP/Cultures
ECG
59
When would you do an MRI brain in Epilepsy?
If the first seizure is when they’re <2
Focal Seizures
No response to antiepileptic medication
60
What general advice should be given to epileptic children? Give four points
Take showers rather than baths
Be cautious with swimming
Be cautious with heights and traffic
Older teenagers may need to avoid driving
61
Describe Sodium Valproate’s MOA
Increases GABA Activity
62
Name three side effects of Sodium Valproate
Teratogenic
Hepatitis
Tremor
63
Name two side effects of Carbemazepine
Agranulocytosis
| CYP inducer
64
Describe Carbemazepine’s MOA
Sodium Channel Blocker
65
State three side effects of Phenytoin
Folate deficiency
Vit D Deficiency
Osteomalacia
66
State two side effects of Ethosuxamide
Night terrors
| Rashes
67
State two side effects of Lamotrigine
Steven Johnson Syndrome
| Leucopenia
68
How should you manage an acute seizure in the community?
Recovery position with soft item under head
Note start and end of seizure
Call an ambulance if first episode or lasts more than five minutes
69
Define Status Epilepticus
Seizures lasting >5 minutes, or >3 in an hour
70
How is Status Epilepticus managed in hospital?
A to E approach
IV Lorazepam
Secure airway
If persists - IV Phenytoin
71
How should Status Epilepticus be managed in the community?
A to E approach
Buccal Midazolam or Rectal Diazepam
72
What is an Extradural Haemorrhage?
Collection of blood in potential space between dura and skull
Normally a venous bleed in children
Typically caused by trauma to temple
73
How does an Extradural Haemorrhage present in a child?
Headache
Nausea and Vomiting
Lucid Interval until consciousness deteriorates
May experience seizures
74
How should Extradural haemorrhages be investigated?
Head CT
75
How should an Extradural Haemorrhage be managed?
A to E
Small bleed - conservative
Raised ICP - Mannitol, Burr Holes
>30cm2 bleed - Surgical Evacuation
76
The prognosis of Extradural haemorrhage in children is excellent. Name two complications.
Neurological deficits
| Post traumatic seizures
77
Define Migraine
Recurrent headache occurring with or without aura, lasting from 30 minutes to 48 hours
78
A Basilar Migraine is a type of migraine. What is it associated with?
Dizziness
| Syncope
79
Childhood Periodic Syndromes are commonly migraine precursors. Name three.
```
Cyclical Vomiting (intense vomiting at night)
Abdominal Migraine
BPPV
```
80
Describe the aura of a migraine
Can be visual, sensory, cognitive or motor
Aura may be more distressing than the headache itself
81
When does a Migraine require further investigation?
Neurological abnormalities
Seizure history
Head trauma
82
What drugs can be used in an Acute Migraine
Simple Analgesia
Sumatriptans (only if over 6y and simple is ineffective)
Domperidone/Prochlorperazine
83
Define Muscular Dystrophy
Umbrella term for genetic conditions causing gradual wasting and weakness of muscles
84
What is Gower’s Sign?
Children with proximal muscle weaknessGet on their hands and knees, push hips back and up, then walk hands up legs in order to stand
Due to weakness of pelvic muscles
85
The most well known Muscular Dystrophy is Duchennes. Describe the pathophysiology
X Linked defective gene for Dystrophin, which normally connects muscular cytoskeleton to ECM
Results in myofibre necrosis
86
Name four presenting features of Duchennes MD
Waddling Gait
Language Delay
Gowers Sign
Pseudo Hypertrophy of Calves (muscle replaces with fat and subcut tissue)
87
What is the prognosis of Muscular Dystrophy?
Usually wheelchair bound by teenage years
Life expectancy 25-35
88
How is Duchennes MD managed?
Oral steroids to slow progression
Creatine supplements
Physiotherapy
May require overnight CPAP
Ataluren
89
How does Ataluren work for MD?
Skips non sense mutations allowing production of small amounts of dystrophin
90
What is Becker’s Muscular Dystrophy?
Similar mutation to Duchennes, but some dystrophin is produced
91
How does Becker’s MD vary from Duchennes?
Clinical course is less predictable
Symptoms start at 8-12y
Patients may require wheelchair in late 20s to 30s
92
What is Myotonic Dystrophy?
Genetic disorder that normally presents in adulthood
Progressive muscular weakness and prolonged muscle contractions (unable to let go after shaking hand)
Associated cataracts and cardiac arrhythmias
93
What is Fascioscapulohumeral MD?
Presents in childhood with weakness around the face progressing to shoulders and arms
Initial signs - sleeping with eyes open, weakness in pursing lips, unable to blow out cheeks
94
What is Oculopharyngeal Muscular Dystrophy?
Usually presents into late adulthood
Weakness of ocular muscles and pharynx
Bilateral ptosis, restricted eye movements, swallowing problems
95
What is Limb Girdle Muscular Dystrophy?
Presents in teenage years with progressive weakness around limb girdle
96
What is Emery Dreifuss MD?
Contracture occurring at elbows and ankles
97
A raised ICP is a pressure above 20mmHg. Describe the normal ranges in infants, young children and older children respectively
Infants - 1.5 to 6
Young - 3 to 7
Older - 10 to 15
98
What is the Monroe Doctrine?
An increase in any constituents of the brain will cause a compensatory decrease in others in order to maintain ICP
Constituents being blood, CSF and brain tissue
99
Give five causes of raised ICP?
```
Masses
Haemorrhage
Impaired CSF flow
Meningitis
Reyes Syndrome
```
100
In raised ICP, herniation can occur. State the three different types.
Subfalcine (cingulate gyrus displaced under falx, may compress ACA)
Tentorial (medial temporal lobe under tentorium cerebelli)
Tonsillar (cerebellar tonsils through foramen magnum, can compress respiratory centres)
101
Describe five classical features of raised ICP
```
Headache worse on waking
Vomiting
Visual Disturbance
Change in mood
Abnormal Pupils
```
Note: May be subtle if mild
102
How does Severely Raised ICP present?
Papilloedema
Sun setting Eyes
Cushing’s Response (bradycardia and hypertension)
103
Name three red flags with raised ICP
GCS<8
Abnormal respiratory pattern
Abnormal Posture (decorticate, decerebrate)
104
How is raised ICP managed?
A to E approach and high flow O2
Tilt 20-30 degrees up
Consider Mannitol, Antibacterials, Antivirals
Urgent CT when stable
105
Define Spinal Muscular Atrophy
Rare autosomal recessive condition that causes progressive loss of motor neurones, leading to muscular weakness
106
As Spinal Muscular Atrophy affects lower motor neurones, how does it present?
```
Fasciculations
Reduced Muscle Bulk
Reduced Tone
Reduced Power
Reduced reflexes
```
107
What are the five types of Spinal Muscular Atrophy?
Type 0 - Prenatal onset, death within 6 months
Type 1 - onset in first few months, progressing to death within 2 years
Type 2 - onset in first 18 months, most never walk but survive into adulthood
Type 3 - onset after first year, walk without support then lose that ability
Type 4 - onset in 20s, able to walk short distances but easily fatigued
Note: in Type 3 and 4 life expectancy is not affected
108
How is SMA managed?
Supportive only
Physiotherapy
PEG?
May have scoliosis and respiratory failure that needs managing
109
Give four causes of Spinal Cord Injury in children
Road Traffic Accidents
Breech Delivery
Shoulder Dystocia
Horse Riding
110
Why are children at risk of Spinal Cord Injury?
Vertebral column is relatively elastic and dura doesn’t provide a lot of mechanical protection
Head is relatively large compared to weak neck
111
How could Spinal Cord Injury present?
Cervical - Tetraplegia with Sensory Loss
Thoracic - Weak Intercostals, breathing assistance with ventilation required
Lumber and Sacral - Paraplegia and loss of control of bladder/bowel/sexual organs
112
How should a suspected SCI be investigated
XRay and CT first, if high suspicion then MRI
Beware of SCIWORA due to flexibility of paediatric spine
113
Describe the Frankel Grading of Spinal Cord Function
```
A - Complete Paralysis
B - Sensory function below injury
C - Incomplete motor function below injury
D - Good Motor function
E - Normal Function
```
114
How are Spinal Cord Injuries in children managed?
A to E
Rehab
Corticosteroids to reduce swelling
115
Name four causes of Subdural Haemorrhage in children
Trauma (inc Birth and NAI)
Cerebral Infections
Coagulation disorders
Hypernatraemia
116
How does a Subdural Haemorrhage present?
```
Encephalopathy
Vomiting
Pallor
Tense Fontanelle
Seizures
```
117
If considering NAI in a Subdural Haemorrhage presentation, what associated features should you look out for?
Retinal Haemorrhages
| Skull Fractures that are depressed/branching
118
How should a Subdural Haemorrhage be investigated?
CT and MRI
119
Name five potential causes of SAH in children
```
AV Malformation
Cerebral Aneurysm
Neoplasms
Leukaemia
Haemophilia
```
120
How does SAH present in <6 month old?
Seizures in 65%
| Apnoea, Irritability, Bulging Fontanelle
121
How does SAH present in >6 month old?
Severe headaches
Seizures
Focal neurology
122
How should suspected SAH be investigated?
USS in neonates
CT
Clotting
LP - Xanthechromia
123
How is SAH managed?
Intubation and ventilation as required
Analgesia
Nifedipine (prevents Vasospasm)
Clipping/coiling where appropriate
124
What is required to diagnose Tension Headaches?
Atleast 10 headaches for 30 minutes of 7 days with atleast two of:
Bilateral Pain
Tightening quality
Not worsened by physical activity
125
What is a Squint/Strabismus?
Misalignment of the eyes
| Images on retina will not match and patient will experience double vision
126
Describe the physiology of a squint in childhood
Brain copes by reducing signal from less dominant eye, creating dominant eye and lazy eye
If lazy eye is not treated it becomes worse (Amblyopia)
127
What are concomitant squints?
Differences in control of Extra- Ocular muscles
128
State the different types of eye movement in Strabismus
Esotropia - Inward moving
Exotropia - Outward moving
Hypertropia - Upward moving
Hypotropia - Downward moving
129
The cause of Strabismus is normally idiopathic. Describe two tests that can be done OE
Hirschbergs - shine pen torch, reflection should be equal (if not then its a squint)
Cover Test - cover one eye, ask patient to focus on an object then switch cover. If eye moves back in then it was an Exotropia etc
130
Management of Strabismus needs to start before 8 years while visual field still develops. How is this done?
Occlusive patch over good eye
| Atropine drops over good eye
131
What is a Scotoma?
Defect surrounded by a normal visual field
Can be relative (ie high luminance objects seen) or absolute
132
What are the five different types of Hemianopia?
```
Bitemporal
Homonymous
Altitudinal
Quadrantopia
Sectoral
```
133
What is Glaucoma?
Loss of Optic Nerve axons associated with raises Intraocular Pressure, manifesting in optic disc cupping and visual loss
In children less than 3y the sclera is stretchy so raised IOP causes globular enlargement and corneal blurring
134
Give three causes of Glaucoma in Children
Congenital
Implicated in Sturge Weber (Port Wine Stain)
Steroid Therapy
135
How can Glaucoma be managed medically?
Topical Beta Blockers
Topical Prostaglandin Analogues
Topical Miotics
Acetazolamide
136
Give 5 causes of Sensorineural Hearing Loss in Children
Lesion at Cochlea/Auditory nerve
Genetic
Antenatal (infection, HIE)
Postnatal (Meningitis,Encephalitis, Aminoglycosides)
137
How is Sensorineural Hearing Loss managed in Children?
Amplification or Cochlear Implant
138
Give three causes of Conductive Hearing Loss
Glue Ear
Wax
Eustacian Tube Dysfunction (Downs, Cleft Palate)
139
How is Conductive Hearing Loss managed?
Conservative
Amplification
Grommets
140
What is Tay Sach’s Disease?
Accumulation of lipids in brain tissue
Associated with: Muscle weakness, Myoclonic jerks, exaggerated startled response, gradual visual deterioration, loss of previously learned skills
141
Name 5 childhood paroxysmal events that could be mistaken for seizures
```
Breath Holding Attacks
Reflex Asystolic Syncope
Migraine
BPPV
Pseudo seizures
```
142
What are Reflex Asystolic Syncope episodes?
Vagally induced cardiac asystole, secondary to cold food/fright/head bump
Child stops breathing, goes limp and blue
Hypoxia may induce Tonic Clonic
143
How might BPPV present in toddlers?
Pale and frightened
May vomit
Exclude temporal lobe epilepsy
144
How might Pseudoseizures present?
Typically adolescent girls
Dramatic
Rolling around
Flinging limbs
Gradual onset
145
West Syndrome is an example of a childhood Seizure Syndrome. How does it present?
Typically onset between 3 and 12 months
Multiple busts of flexor spasms causing distress
Salaam attacks (rapid flexion extension)
Gradually lose skills and develop learning disability
146
How do Infantile Spasms/West Syndrome present on EEG?
Hypsarrhythmia
Burst supression
147
Lennox Gastaut Syndrome is an example of a childhood Seizure Syndrome. How does it present?
Can be : Atonic, Absence (with blinking or head nodding) or Tonic (stiffening of arms and legs at night)
Poor neurodevelopment progression
148
How does Lennox Gastaut Syndrome present on EEG?
Slow generalised spike and wave discharge (1-3 Hz)
149
Benign Rolandic Epilepsy is an example of a childhood Seizure Syndrome. How does it present?
Starts during sleep or when about to wake
Pins and needles on one side of mouth
Can get Associated twitching or can involve throat (affecting speech)
Occasionally the whole body is affected
Most grow out at Puberty
150
How does Benign Rolandic Epilepsy present on EEG
Posterior sharp waves
| Occipital discharges
151
Juvenile Myoclonic Epilepsy Syndrome is an example of a childhood Seizure Syndrome. How does it present?
Normally onset between 10-20y
Can be Myoclonic or tonic Clonic
Unlikely to grow out of but control is good
152
How does Juvenile Myoclonic Epilepsy present on EEG?
3-6 Hz Polyspike and Wave discharge
153
Describe some hearing test milestones for children
Newborn - Otoacoustic Emission (looking for a soft echo meaning normal tympanic membrane)
If Otoacoustic Emission abnormal - Auditory Brainstem
Distraction test at 6-9 months
Pure Tone Audiometry upon school entry
