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Neurology Flashcards

1
Q

Define cerebral palsy

A

Permanent disorder of movement and/or posture and motor function due to non-progressive abnormality of developing brain

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2
Q

Causes of cerebral palsy

A

80% antenatal - prematurity, genetic, infection
10% intrapartum - hypoxic-ischaemic encephalopathy
10% postnatal - infection, trauma, hyperbilirubinaemia

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3
Q

What are the early clinical features of CP

A

Abnormal posture and tone, delayed motor milestones
Feeding difficulties
Abnormal fair
Asymmetric hand function before 12 months

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4
Q

What are the types of CP

A

Spastic: unilateral, bilateral Diplegia, bilateral quadriplegia
Dyskinetic
Ataxic

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5
Q

Cause of spastic CP

A

UMN lesions
Unilateral - perinatal MCA infarct
Bilateral diplegic - damage to periventricular areas
Bilateral quadriplegic - extensive damage to periventricular areas

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6
Q

What are clinical features of spastic CP

A

Unilateral
spastic or dystonic tone,
arm > leg,
walks w or wo aid (GSM 1-2)

Biplegic
legs > arms
young child - walk on toes + scissoring,
older - crouch gait

Quadriplegic 
Learning difficulty 
Feeding difficulty 
Problems with speech, vision, hearing 
Seizures 
Requires powered mobility 
Dependent for ADLs
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7
Q

Clinical features of dyskinetic CP

A 
Floppiness
Delayed motor development 
Poor trunk control 
Abnormal movements - dystonia, chorea, athetosis 
Feeding difficulty 
Dependent on ADLs
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8
Q

What causes dyskinetic CP

A

Hypoxic-ischaemic encephalopathy at term

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9
Q

Define chorea, athetosis, dystonia

A

Chorea: irregular, sudden, non-repetitive movements
Athetosis: slow writhing movements occurring more dismally
Dystonia: twisting movements due to simultaneous contraction of agonist and antagonist muscles

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10
Q

What causes Ataxic CP

A

Genetic

Acquired - cerebellar injury

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11
Q

Clinical features of ataxic CP

A 
Hypotonia 
Poor balance
Delayed motor development 
Incoordination
Intention tremor
Ataxic gait
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12
Q

CP investigations

A

History - for static neurological insult

MRI brain - periventricular leukomalacia in prematurity

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13
Q

CP management

A 
Complex multidisciplinary input 
Stretching exercises 
Orthoses, wheelchair, sleeping and standing systems 
Botulinum toxin injection 
Speech therapy 
Learning support
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14
Q

Floppy infant - causes?

A 
Floppy strong (CNS) 
Prematurity 
Hypoxic-ischaemic encephalopathy 
Trisomy 21 / chromosomal 
In-born errors of metabolism 
Hypoglycaemia
Hypothyroidism 
Sepsis 
Floppy weak (PNS, NMJ, muscle disease) 
Spinal muscular atrophy
Myasthenia gravis
Congenital myotonic dystrophy 
Congenital muscular dystrophy 
Metabolic myopathies 
Congenital myopathies 
Peripheral neuropathies
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15
Q

Floppy infant - clinical features

A 
All:
Generalised hypotonia
Frog-leg posture
Respiratory failure 
Hx obstetric complications - polyhydramnios, breech

Central:
Encephalopathy
Reasonable muscle strength
Increased tendon reflexes

Peripheral: 
Muscle - weakness, myotonia, fasciculations, fatiguing 
Reduced tendon reflexes 
Little facial expression 
Ptosis
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16
Q

Floppy infant - investigations

A 
Exclude severe systemic illness
O2 + ventilatory support (for Resp failure) 
Family, antenatal Hx
Exam (to distinguish cause) 
Central: 
glucose, U+E, Ca, Mg, septic screen,  CRP,  TFT, Karyotype, IEM screen 
cranial uss, MRI, EEG 
Peripheral: 
Muscle USS 
EMG, nerve conduction studies
Muscle or Surat nerve biopsy
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17
Q

Classification of headaches

A

Primary:
Tension
Migraine
Cluster

Secondary: 
Medication overuse
Medication withdrawal 
Intracranial haemorrhage, Vascular malformation 
Head/neck trauma
Meningitis/encephalitis 
Acute sinusitis, acute glaucoma 
HTN, hypercapnia

Cranial neuralgias, central facial pain:
Trigeminal neuralgia

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18
Q

Uncommon forms of migraine

A

Familial hemiplegic migraine
Sporadic hemiplegic migraine
Basilar-type migraine: vomiting, nystagmus, cerebellar signs
Periodic syndromes:
Cyclical vomiting - recurrent episodes of N+V
Abdominal migraine - recurrent episodes of midline abdo pain
Benign paroxysmal vertigo of childhood - recurrent episodes of vertigo

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19
Q

Management of headaches

A

Rescue:
Analgesics - NSAIDs, paracetamol
Antiemetic
Triptan (serotonin agonist)

Preventative: 
Topiramate (Na channel blockers) 
Propranolol 
Tricyclists (pizotifen, amitriptyline) 
Acupuncture 
Supportive:
Lifestyle changes 
cold compress, warm pads 
psychological support 
relaxation techniques
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20
Q

Define febrile seizure

A

Epileptic seizure accompanied by pyrexia

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21
Q

Epidemiology of febrile seizure

A

5%

6 months - 6yrs

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22
Q

Risk factors for febrile seizures

A

Family Hx

Viral illness - rapid rise in temperature

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23
Q

Clinical features of febrile seizure

A

Generalised tonic clonic seizure

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24
Q

Prognosis of febrile seizure

A

Self resolving, within 5 minutes
Not associated with brain damage
Not associated with increased risk of epilepsy
Increased risk of epilepsy if complicated - prolonged, focal, recurrent

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25
Management of febrile seizure
Reassurance and education | Buccal midazolam If Hx of prolonged seizures
26
Define seizure
Paroxysmal abnormality in motor/sensory/autonomic/cognitive function due to transient brain dysfunction
27
What is epileptic seizure
Seizure due to excessive and hypersynchronous electrical activity
28
What is epilepsy
Brain disorder that predisposes patient to have unprovoked epileptic seizures
29
What is convulsion
Seizure with motor components - tonic, clonic, myoclonic, spams, hypermotor
30
Causes of seizures
Epilepsy Acute symptomatic seizures Febrile seizures ``` Non-epileptic seizures: Syncope Sudden rise in ICP Sleep disorders Functional/behavioural ```
31
What is acute symptomatic epileptic seizures
Provoked epileptic seizure due to brain injury | E.g. stroke, traumatic brain injury, intracranial infection, hypoglycaemia, electrolyte disturbance, toxins
32
What are paroxysmal disorders
Funny turns | Recurrent movement disorders with acute onset and ending
33
Causes of paroxysmal disorders
``` Epilepsy Blue breath holding spells Reflex asystolic syncope Syncope Migraine Long QT Syndrome Self gratification Non-epileptic attack disorder Induced illness GORD Paroxysmal movement disorders - tics ```
34
Clinical features of non-epileptic attack disorder
``` Provoked by emotional precipitants Do not occur during sleep Intractable despite medicine Onset gradual Injury during fall fare No post-ictal phase or amnesia Hx of abuse ```
35
Clinical features of breath holding spells
Precipitated by anger Vigorous cry followed by period of blocked respiration Resulting in cyanosis, loss of consciousness Sometimes opisthotonos posturing - muscle spasm leading to pronounced hyper-extension with head and lower limbs bend backwards and trunk arched forward)
36
Clinical features of reflex asystolic seizure
Precipitated by mild injury - cold food, head trauma, fever Becomes pale and loss of consciousness Cerebral hypoxia may lead to opisthotonic posturing and brief clonic movements
37
Clinical features of syncope
Happens in standing position Accompanied by pallor and seating Quick recovery and no post-ictal phase
38
Clinical features of parasomnia
Occur in first few hours of sleep Not repetitive in same night Normal EEG during event
39
Clinical features of GORD
Infant gasping and becoming apnoeic and stiff Change in skin colour Sandifer Syndrome - spastic torticollis, dystonic movements Occurs within 1 hour of feed
40
Aetiological Classification of epilepsy with generalised seizures
``` Genetic Structural Metabolic Infectious Immune Unknown ```
41
Pathophysiology of epilepsy
Increased neuronal excitability and synchronicity | Abnormal neuronal activity Originate at single point within brain and rapidly engage bilaterally distributed networks
42
Classification of epilepsy type
Focal: Originate in networks limited to one hemisphere Generalised: Originate in bilaterally distributed networks Loss of consciousness >3 seconds
43
Differences bw clonic and Myoclonic movements
Tonic: rhythmic muscular jerking movements Myoclonic: arrhythmic muscular jerking movements
44
Classification by seizure type of focal epilepsy
``` Focal Aware / Focal Impaired awareness Motor onset: Automatism Atonic Clonic Epileptic spasms Hyperkinetic Myoclonic Tonic ``` ``` Non-motor onset: Autonomic Behaviour arrest Cognitive Emotional Sensory ```
45
Classification of generalised epilepsy by seizure type
``` Non-motor/absence: Typical Atypical Myoclonic Eyelid myoclonia ``` ``` Motor: Tonic clonic Tonic Clonic Myoclonic Myoclonic tonic clonic Myoclonic atonic Atonic Epileptic spasms ```
46
What causes genetic generalised epilepsy, what syndromes does it cover
Caused by genetic mutations at several loci, thus complex inheritance and de novo mutations Childhood absence epilepsy, Juvenile absence epilepsy, juvenile myoclonic epilepsy, generalised tonic clonic seizures
47
Clinical features of infantile spasms
<1 years (excluding neonates) Violent flexor spasms of head, trunk, limbs Lasts 1-2 seconds, in multiple bursts of 20-30 seconds Child distressed Social interaction deteriorates EEG: hysparrhythmia - chaotic background with slow-wave activity
48
Clinical features of Lennox-Gastaut syndrome
Early childhood: 1-6 Multiple seizure types: Atonic, atypical absence, tonic clonic, myoclonic Associated with: Neurodevelopmental arrest, regression, behaviour disorder Poor prognosis
49
Clinical features of panayiotopoulos syndrome
Early childhood: 1-6 Autonomic features, vomiting Unresponsive staring in sleep Head and eye deviation EEG: occipital discharges when eyes shut
50
Clinical features of childhood absence epilepsy
``` Middle childhood: 6-10 Momentary unresponsive stare with motor arrest May twitch eyelids, hands, mouth Sudden onset, lasts <30 seconds Child has no recall Developmentally normal ``` Induced by hyperventilation Good prognosis - 80% remission
51
Clinical features of benign rolandic epilepsy
Middle childhood: 6-10 Tonic clonic seizures in sleep or Focal aware seizures with abnormal sensation in tongue and distortion of face (rolandic area of brain) EEG: Focal sharp waves in rolandic area Remits in adolescence Does not require AEDs
52
Clinical features of juvenile absence epilepsy
Adolescence: 10-14 Absences and generalised tonic clonic seizures Often photosensitivity
53
Clinical features of Juvenile myoclonic epilepsy
Adolescent: 10-14 Myoclonic seizures, Generalised tonic clonic seizures, Absences Often after waking - throwing cereal in morning
54
Risk factors for epilepsy
``` Genetic: family history, Fragile X syndrome, Angelman syndrome Perinatal asphyxia Metabolic disorders Neurodegenerative disorders Head trauma Structural abnormalities of CNS ```
55
Clinical features of epileptic seizures
Incontinence Tongue biting Post-ictal phenomena: sleepiness, headaches, amnesia, confusion Precipitated by fatigue of lack of sleep Precipitated by light or noise Developmental delay - Lennox gastaut Syndrome, myoclonic absence Intercurrent illness
56
Clinical features of absence seizure
Staring spells or inattention +/- Motor arrest or stereotyped movements Typical: behavioural arrest or staring, lasting 5-10 seconds, interrupting normal activity, can be hyperventilation induced Atypical: less distinct beginning and end, not precipitated by hyperventilation
57
Clinical features of tonic clonic seizures
Tonic phase - fall unconscious and extension/flexion of extremities Clonic phase - violent muscle contractions Associated with: eyes roll back in head, tongue biting, incontinence May be preceded by aura Followed by post ictal phenomena
58
Investigations for epilepsy
EEG: inter-ictal recording shows abnormal rhythms specific to epilepsy syndrome, can be provoked by sleep deprivation, hyperventilation, photic stimulation ECG: exclude cardiac cause FBC Blood glucose Metabolic panel: exclude metabolic and electrolyte abnormalities Genetic tests: if developmental delay MRI brain: if structural cause suspected
59
Management of epileptic spasms
Vigabatrin + ACTH/corticosteroid
60
Management of Lennox gastaut Syndrome
Sodium valproate / clonazepam
61
Treatment for other generalised epilepsy syndromes
Sodium valproate / Lamotrigine / Levetiracetam
62
Treatment for focal epilepsy
Carbamazepine

Paediatrics (15 decks)

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