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Neuro Flashcards

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1
Q

What are periodic syndromes?

A

Syndromes which affect children and are often precursors of migraine
Child is well between episodes

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2
Q

What is cyclical vomiting?

A

A periodic syndrome.
Recurrent stereotyped episodes of vomiting and intense nausea associated with pallor and lethargy
Child is well in between episodes

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3
Q

What is abdominal migraine?

A

Idiopathic recurrent disorder
Episodic midline abdominal pain in bouts lasting 1-72 hr
Pain moderate to severe in intensity
Associated with vasomotor symptoms, nausea and vomiting

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4
Q

What is benign paroxysmal vertigo of childhood?

A

Heterogenous disorder
Characterised by brief episodes of vertigo which occur without warning and resolve spontaneously
Children otherwise healthy with normal neuro, auditory and vestibular exam

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5
Q

When can sumatriptan be given in children with headaches? Route

A

Nasal preparation

Licensed for use in children over 12 years of age

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6
Q

What are febrile seizures?

A

Seizure accompanied by a fever in absence of intracranial infection due to meningitis or encephalitis

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7
Q

Incidence and age prevalence of febrile seizures?

A

3% of children between 6 months and 6 years

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8
Q

Inheritance of febrile seizures

A

Genetic predisposition with 10% chance if first degree relative had them

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9
Q

When during illness does seizure usually occur

A

Early on in viral infection when temperature is rising rapidly

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10
Q

What sort of seizures are febrile seizures?

A

Usually tonic-clonic seizures

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11
Q

What % of 1st febrile seizure will have further ones

A

30-40%

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12
Q

What increases likelihood of having more febrile seizures? x4

A

Earlier on in illness
Younger age of child
Lower temperature at time of seizure
Positive family history

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13
Q

When is there an increased risk of developing epilepsy with febrile seizures? x3

A

If complex febrile seizures

Aka, prolonged, repeated in same illness or focal seizures

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14
Q

If febrile seizure goes on longer than 5 mins

A

Buccal midazolam or rectal diazepam

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15
Q

What happens in breath-holding attacks?

A

Happens in toddlers when they are upset

Child cries, holds breath, goes blue. Will sometimes briefly lose consciousness but rapidly recover

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16
Q

What is reflex anoxic seizure

A

Occurs in infants or toddlers when scared or shocked (head trauma, cold food, fright or fever)
Becomes pale and falls to the floor
Hypoxia may induce tonic-clonic seizure
Due to cardiac asystole from vagal inhibition
Child recovers quickly after brief seizure

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17
Q

Pseudoseizures vs fabricated seizures

A

Pseudoseizures - child feigns seizure

Fabricated - by parents

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18
Q

When do you do metabolic investigations with seizures?

A

When there is developmental regression or when seizures are related to feeds or fasting

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19
Q

What is West syndrome?

A

Violent flexor spasms of head, trunk and limbs followed by extension of arms
Flexor spasms last 1-2s and usually 20-30 spasms
Often occur on waking
EEG signs
2/3 have underlying neuro cause

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20
Q

What age is West syndrome?

A

4-6 months

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21
Q

How do you treat West Syndrome? Response rate and future development

A

Vigabatrin or corticosteroids
Good response in 30-40%
Most lose skills and develop learning disability or epilepsy

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22
Q

What is Lennox-Gastaut syndrome?

A

Multiple seizure types - mostly drop attacks, tonic seizures and atypical absences
Also have neurodevelopmental arrest/regression and behavioural disorder

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23
Q

Age and prognosis of Lennox-Gastaut

A

1-3 years
Often other complex neurological problems and history of infantile spasms
Prognosis poor

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24
Q

First line for tonic-clonic seizures x2

A

Valproate and carbamazepine

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25
First line for absence seizure x2
Valproate and ethosuximide
26
First line for myoclonic seizure x1
Valproate
27
First line for focal seizures x2
Carbamazepine and valproate
28
Signs of myopathy in children?
Waddling gait or positive Gowers sign (need to turn prone to rise to standing, use arms to stand up)
29
When is positive Gowers sign normal?
Before the age of 3 years
30
Investigative signs of myopathy
Serum creatine phosphokinase markedly elevated in muscular dystrophy and inflammatory myopathies
31
Presentation of anterior horn cell disorders x4
Weakness, wasting, absent reflexes and fasciculation
32
What is spinal muscular atrophy?
Autosomal recessive degeneration of the anterior horn cells | Progressive weakness and wasting of skeletal muscles
33
What gene is mutated in spinal muscular atrophy?
Survival motor neurone (SMN) gene
34
What is Werdnig-Hoffman disease
Spinal muscular atrophy type 1 | Very severe and presents early at birth/in infancy
35
What is noticed in pregnancy with Werdnig-Hoffman disease
Diminished fetal movements
36
What is noticed at birth with Werdnig-Hoffman disease
There may be arthrogryposis (positional deformities of the limbs with contractures of at least two joints)
37
Other signs of Werdnig-Hoffman disease x5
``` Lack of antigravity power in hip flexors Absent deep tendon reflexes Intercostal recession Fasciculation of the tongue Never sit unaided ```
38
Prognosis of Werdnig-Hoffman disease
Death from respiratory failure at 12 months
39
Features of type 2 spinal muscular atrophy
Can sit but never walk independantly
40
Features of type 3 spinal muscular atrophy (Kugelberg-Welander)
Do walk and can present later in life
41
What are the hereditary motor sensory neuropathies
A peripheral neuropathy with slowly progressive muscular wasting (distal) Type 1 = Charcot-Marie-Tooth disease Nerves can be hypertrophic due to demyelination followed by attempted remyelination
42
What limb is more affected in hereditary motor sensory neuropathies. Age of onset
Legs more than arms | Usually onset in first decade
43
What is GBS? how does it arise?
Acute post-infectious polyneuropathy | 2-3 weeks after URTI or campylobacter gastroenteritis
44
Features of GBS x7
Ascending symmetrical weakness Loss of reflexes Autonomic involvement Sensory symptoms may also be present Bulbar muscle involvement = difficulty with chewing and swallowing - risk aspiration Respiratory depression may need ventilation
45
Recovery from GBS
95% fully recover | May take up to 2 years
46
Main complication of Bells Palsy
Conjunctival infection due to incomplete eye closure on blinking
47
Age of presentation of juvenile myasthenia gravis
Onset usually after 10 years | Presents with ophthalmoplegia and ptosis, loss of facial expression and difficulty chewing
48
Treatment of MG
Anti-muscarinics (eg. atropine to reduce the muscarinic side effects of acetylcholinesterase inhibitors) Neostigmine or pyridostigmine - prevent breakdown of acetylcholine Longer term immunosuppression with prednisolone or azathioprine can help
49
Presentation of Duchenne Muscular Dystrophy and gene
``` Wadding gait and/or language delay Xp21 site (codes for dystrophin) ```
50
Average age of diagnosis of Duchenne
5.5 years but symptoms will be present earlier | No longer ambulant by age 10-14
51
What is used in Duchenne to preserve mobility and prevent scoliosis x4
Exercise, stretching to avoid contractures, brace for scoliosis Prednisolone 10days per month Mechanism of it helping is not known
52
What is Becker muscular dystrophy
Milder form of Duchenne | Average onset 11 years and can walk into 20s (only 11-14 in Duchenne)
53
Presentation of metabolic myopathies
Floppy infant or muscle weakness and cramps on exercise in older infants
54
What is benign acute myositis?
Assumed to be post-viral Follows UTRI and runs self-limiting course Pain and weakness in affected muscles CPK raised
55
What is dermatomyositis?
``` Systemic illness, symmetrical proximal muscle weakness Characteristic violaceous (heliotrope) rash to eyelids and periorbital oedema ```
56
Where can rash extend to in dermatomyositis?
Extensor surfaces of joints and can get subcutaneous calcification
57
How and when does dermatomyositis present?
Presents usually between 5-10 years with fever, misery and eventually muscle weakness
58
Treatment of dermatomyositis x3
Physiotherapy to prevent contractures Corticosteroids are mainstay Other immunosuppressants eg. methotrexate, ciclosporin, may be needed
59
What do adults develop with dystrophia myotonica?
Cataracts (myotonia, delayed relaxation of muscles after contraction) and muscular dystrophy
60
What do males develop with dystrophia myotonica
Baldness and testicular atrophy
61
What is Friedreich ataxia?
Ataxia due to progressive damage of the nervous system - poor coordination and impairment of joint position and vibration sense - autosomal recessive Eventually wheelchair is required for mobility
62
How does Friedreich ataxia present x4
With worsening ataxia Distal wasting in legs Absent lower limb reflexes Absent extensor plantar responses
63
What happens in Freidreich ataxia due to pyramidal involvement? x2
Pes cavus and dysarthria
64
Life expectancy with Freidreich ataxia
Death at 40-50 years due to developing kyphoscoliosis and cardiomyopathy - cardiorespiratory compromise
65
Type of haemorhage in non-accidental injury or direct trauma in children
Subdural haematoma
66
Most common causes of stroke in children x3
Cardiac or sickle cell disease | Varicella infection
67
Mothers with neural tube defect child - chance of having another child with NTD
10x increased risk of second affected child
68
What has reduced risk of NTD
Folic acid
69
What is anencephaly?
Failure of development of most of the cranium and brain Usually stillborn or die shortly after birth Can detect antenatally and terminate pregnancy
70
What is encephalocele?
Extrusion of brain and meninges through midline skull defect Can be corrected surgically Often underlying associated cerebral malformations
71
What is spina bifida occulta?
Failure of fusion of vertebral arch Can be overlying skin lesion Often detected incidentally on x-ray
72
What is diastematomyelia
Underlying tethering of cord to skin overlying spina bifida | As grows can cause neurological deficits of lower limbs and bladder
73
What is meningocele?
Protrusion of dura and CSF through vertebral arch
74
Prognosis of meningocele
Good prognosis following surgical repair
75
What is myelomeningocele
Dura and spinal cord through unfused vertebral arch
76
What can myelomeningocele be associated with x6
``` Variable paralysis of legs Muscle imbalance with dislocations Sensory loss Bladder and bowel denervations (neuropathic bladder/bowel) Scoliosis Hydrocephalus ```
77
Management of myelomeningocele
Back lesion usually closed soon after birth - manage complications/resulting symptoms
78
What is hydrocephalus?
Obstruction to flow of CSF therefore dilatation of ventricular system proximal to obstruction
79
What is non-communicating/obstructive hydrocephalus
Obstruction within ventricular system or aqueduct
80
What is communicating hydrocephalus
Obstruction at arachnoid villi (site of absorption)
81
Features of hydrocephalus x4
Skull sutures have not fused therefore head circumference disproportionately large or excessive growth rate Anterior fontanelle bulges Sutures separate Scalp veins distended
82
Advanced sign of hydrocephalus
Fixed downward gaze or sun setting of the eyes
83
Treatment of hydrocephalus
Insertion of ventriculoperitoneal shunt is mainstay | Endoscopic treatment to create ventriculostomy can now be performed
84
How do neurocutaneous syndromes arise?
Nervous system and skin have common ectodermal origin | Therefore embryological disruption causes syndromes involving abnormalities of both systems
85
Prevalence of neurofibromatosis 1
1 in 3000 live births | 1/3 have new mutations
86
What features are present in NF1 x7 (need 2 or more for diagnosis)
6 or more cafe au lait spots >5mm before puberty or >15mm afterwards More than one neurofibroma Axillary freckles Optic glioma Lisch nodule Bony lesions from sphenoid dysplasia (can cause eye protrusion) 1st degree relative with NF1
87
Where can neurofibromata appear in NF1
Anywhere on peripheral nerve - including visual/auditory nerves Look unslightly and can cause neurological signs if where nerve passes through bone
88
Features of NF2 x4
Presents in adolescence Less common Bilateral acoustic neuromata Sometimes cerebellopontine angle syndrome (VII CN paresis and cerebellar ataxia) (Also get cataracts and peripheral neuropathy and less defined skin features - possible schwannomas)
89
What is tuberous sclerosis
Benign tumours grow on brain, other vital organs and skin
90
Cutaneous features of tuberous sclerosis x4
Depigmented 'ash leaf' shaped patches - fluoresce under UV light Roughened patches of skin - usually over lumbar spine = Shagreen patches Adenoma sebaceum - butterfly distribution over nose and cheeks Periungual fibroma
91
Neurological features of tuberous sclerosis x3
Infantile spasms and developmental delay Epilepsy (often focal) Intellectual impairment
92
Nail features of tuberous sclerosis
Fibromata beneath the nails - subungual fibromata
93
What is Sturge-Weber syndrome?
Port-wine stains on one side of the face with tumours and CNS neurological defects
94
Where is facial lesion in Sturge-Weber syndrome?
In distribution of trigeminal nerve - opthalmic division is always involved
95
Neurological signs of Sturge-Weber syndrome x3
Epilepsy, Learning disability and hemiplegia - in most severe form
96
What are neurodegenerative disorders
Deterioration in motor and intellectual function with abnormal neurological features, abnormal head circumference, visual/hearing loss and behavioural changes
97
Causes of neurodegenerative disorders x5
Lysosomal storage diseases Peroxisomal enzyme defects (involved in long chain fatty acid oxidation therefore LCFA accumulation) Heredodegenerative disorders eg. HTT disease Wilson disease Subacute scelrosing panencephalitis (SSPE)
98
Prophylactic agents for headache x3
Pizotifen (5-ht antagonist) - can cause weight gain and sleepiness B-blockers - propanaolol - CI in asthma Sodium channel blockers (valproate or topiramate)
99
Management if epilepsy is suspected x4
EEG is always indicated (to add supportive evidence for diagnosis not to diagnose) Can also do sleep or sleep-deprived EEG or 24 ambulatory EEG MRI or CT is neurological signs are present between seizures or if they are focal PET or SPECT (functional imaging to identify epileptogenic lesions) Metabolic (see other flashcard)
100
Typical age for childhood absence epilepsy
4-12 years
101
What sort of diet might help epileptic children?
Ketogenic (fat-based) diet
102
Valproate side effects x3
Weight gain, hair loss and rare idiosyncratic liver failure
103
Carbamazepine/oxcarbezepine side effects x5
Rash, neutropenia, hyponatraemia, ataxia | Liver enzyme induction
104
Vigabatrin side effects x2
Sedation | Restriction of visual fields
105
Lamotrigine side effect
Rash
106
Probable cause of Bells Palsy
Post-infectious | Association with herpes simplex virus in adults
107
Management of Bells Palsy
Corticosteroids - reduce oedema in facial canal
108
Important DDX of Bells Palsy
Cerebellopontine angle tumour
109
Bilateral Bells Palsy DDX?
Sarcoidosis | Lyme disease
110
DX of MG x3
Administration of IV edrophonium (anticholinesterase) leads to improvement Ach receptor antibodies (present in 60-80% of cases) More rarely - anti-muscle specific kinase (anti-MuSK) antibodies
111
What are myotonic disorders?
Delayed relaxation after sustained muscle contraction - clinically and on EMG eg. slow release of handshake
112
Presentation of dystrophia myotonica in newborns
Hypotonia, feeding and respiratory difficulties due to muscle weakness
113
What is ataxia telangiectasia?
Disorder of DNA repair - mild delay in motor development in infancy and oculomotor problems with incoordination
114
What becomes evident at school age in ataxia telangiectasia x3
Telangiectasia in conjunctiva, neck and shoulders from about 4 years of age Difficulty with balance and coordination Wheelchair needed in early adoloscence
115
Complications in ataxia telangiectasia x2
Increased susceptibility to infection | Develop malignant disorders, esp, acute lymphoblastic leukaemia
116
What is raised in serum in ataxia telangiectasia
Alpha fetoprotein
117
Complications of neurofibromatosis 1
60% develop learning difficulties 50% have ADHD High blood pressure due to increased renin release from kidneys Physical development abnormalities eg. large head, scoliosis, smaller weight and height than normal Migraines, epilepsy and brain tumours

Paediatrics (16 decks)

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