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
Q

Management of febrile seizure

A

Reassurance and education

Buccal midazolam If Hx of prolonged seizures

26
Q

Define seizure

A

Paroxysmal abnormality in motor/sensory/autonomic/cognitive function due to transient brain dysfunction

27
Q

What is epileptic seizure

A

Seizure due to excessive and hypersynchronous electrical activity

28
Q

What is epilepsy

A

Brain disorder that predisposes patient to have unprovoked epileptic seizures

29
Q

What is convulsion

A

Seizure with motor components - tonic, clonic, myoclonic, spams, hypermotor

30
Q

Causes of seizures

A

Epilepsy
Acute symptomatic seizures
Febrile seizures

Non-epileptic seizures:
Syncope
Sudden rise in ICP
Sleep disorders
Functional/behavioural
31
Q

What is acute symptomatic epileptic seizures

A

Provoked epileptic seizure due to brain injury

E.g. stroke, traumatic brain injury, intracranial infection, hypoglycaemia, electrolyte disturbance, toxins

32
Q

What are paroxysmal disorders

A

Funny turns

Recurrent movement disorders with acute onset and ending

33
Q

Causes of paroxysmal disorders

A
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
Q

Clinical features of non-epileptic attack disorder

A
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
Q

Clinical features of breath holding spells

A

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
Q

Clinical features of reflex asystolic seizure

A

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
Q

Clinical features of syncope

A

Happens in standing position
Accompanied by pallor and seating
Quick recovery and no post-ictal phase

38
Q

Clinical features of parasomnia

A

Occur in first few hours of sleep
Not repetitive in same night
Normal EEG during event

39
Q

Clinical features of GORD

A

Infant gasping and becoming apnoeic and stiff
Change in skin colour
Sandifer Syndrome - spastic torticollis, dystonic movements
Occurs within 1 hour of feed

40
Q

Aetiological Classification of epilepsy with generalised seizures

A
Genetic
Structural
Metabolic
Infectious
Immune 
Unknown
41
Q

Pathophysiology of epilepsy

A

Increased neuronal excitability and synchronicity

Abnormal neuronal activity Originate at single point within brain and rapidly engage bilaterally distributed networks

42
Q

Classification of epilepsy type

A

Focal:
Originate in networks limited to one hemisphere

Generalised:
Originate in bilaterally distributed networks
Loss of consciousness >3 seconds

43
Q

Differences bw clonic and Myoclonic movements

A

Tonic: rhythmic muscular jerking movements
Myoclonic: arrhythmic muscular jerking movements

44
Q

Classification by seizure type of focal epilepsy

A
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
Q

Classification of generalised epilepsy by seizure type

A
Non-motor/absence: 
Typical 
Atypical
Myoclonic
Eyelid myoclonia
Motor:
Tonic clonic
Tonic
Clonic
Myoclonic
Myoclonic tonic clonic
Myoclonic atonic
Atonic
Epileptic spasms
46
Q

What causes genetic generalised epilepsy, what syndromes does it cover

A

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
Q

Clinical features of infantile spasms

A

<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
Q

Clinical features of Lennox-Gastaut syndrome

A

Early childhood: 1-6
Multiple seizure types: Atonic, atypical absence, tonic clonic, myoclonic
Associated with: Neurodevelopmental arrest, regression, behaviour disorder

Poor prognosis

49
Q

Clinical features of panayiotopoulos syndrome

A

Early childhood: 1-6
Autonomic features, vomiting
Unresponsive staring in sleep
Head and eye deviation

EEG: occipital discharges when eyes shut

50
Q

Clinical features of childhood absence epilepsy

A
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
Q

Clinical features of benign rolandic epilepsy

A

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
Q

Clinical features of juvenile absence epilepsy

A

Adolescence: 10-14
Absences and generalised tonic clonic seizures
Often photosensitivity

53
Q

Clinical features of Juvenile myoclonic epilepsy

A

Adolescent: 10-14
Myoclonic seizures, Generalised tonic clonic seizures, Absences
Often after waking - throwing cereal in morning

54
Q

Risk factors for epilepsy

A
Genetic: family history,  Fragile X syndrome, Angelman syndrome 
Perinatal asphyxia 
Metabolic disorders
Neurodegenerative disorders
Head trauma
Structural abnormalities of CNS
55
Q

Clinical features of epileptic seizures

A

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
Q

Clinical features of absence seizure

A

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
Q

Clinical features of tonic clonic seizures

A

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
Q

Investigations for epilepsy

A

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
Q

Management of epileptic spasms

A

Vigabatrin + ACTH/corticosteroid

60
Q

Management of Lennox gastaut Syndrome

A

Sodium valproate / clonazepam

61
Q

Treatment for other generalised epilepsy syndromes

A

Sodium valproate / Lamotrigine / Levetiracetam

62
Q

Treatment for focal epilepsy

A

Carbamazepine