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Neurology Flashcards

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1
Q

How does a medulloblastoma present?

A

Arise in midline of posterior fossa and may spread through CNS via CSF –> spinal metastases
–> truncal ataxia, coordination difficulties, abnormal eye movements and morning vomiting

Younger child
50% 5 year survival

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2
Q

How does a brainstem glioma present?

A

Commoner in young children
Cranial nerve defects, pyramidal tract signs, ataxia and normal ICP (refusing to walk, unable to climb stairs)

Very poor prognosis

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3
Q

How does a craniopharyngioma present?

A

Developmental tumour arising from remnants of Rathke’s pouch
–> bitemporal hemianopia, pituitary failure (growth failure, weight gain and diabetes insipidus)

Good survival

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4
Q

What are clinical features of brain tumours?

A 
Headache - worse in morning
Vomiting - worse in morning
Behaviour/personality changes
Visual disturbances
Papilloedema
Separating of fontanelles
Increased head circumference
Head tilt
Developmental delay

Spinal tumours –> back pain, peripheral weakness of arms/legs or bladder/bowel dysfunction

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5
Q

How does an astrocytoma present?

A

Vary from benign to highly malignant
Occur in cerebral hemispheres, thalamus an hypothalamus
–> seizures, headaches, hemiplegia, focal neurological signs

Teenager
Poor prognosis

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6
Q

What causes a motor deficit?

A

Central motor deficit - cerebral palsy
Congenital myopathy
Spinal cord lesions - spina bifida
Global developmental delay

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7
Q

How do gross motor skills develop?

A 
Newborn - flexed limbs, head lag pulling up
8 weeks - raises head to 45 degrees
6 months - sits unsupported
9 months - crawling
10 months - cruising
12 months - walks unsteadily
15 months - walks steadily
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8
Q

How do fine motor skills and vision develop?

A 
6 weeks - follows moving objects
4 months - reaches for toys
6 months - grasps toys
7 months - passes hand to hand
10 months - pincer grips
18 months - makes marks with pen
5 years - draws triangle
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9
Q

What is cerebral palsy?

A

An abnormality of movement and posture
May be associated with disturbances of cognition, communication, seizure disorder
Lesion is non-progressive but manifests over time
Must occur before 2 years

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10
Q

What causes cerebral palsy?

A

Antenatal (80%) - vascular occlusion, cortical migration disorders, structural maldevelopment of brain

Perinatal (10%) - hypoxia-ischaemic injury, peri-ventricular leucomalacia

Postnatal (10%) - meningitis/encephalitis, encephalopathy, head trauma, hypoglycaemia, hyperbilirubinaemia

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11
Q

How does cerebral palsy present?

A
  • Abnormal limb/trunk posture or tone with delayed motor milestones
  • Feeding difficulties, slow feeding, gagging, vomiting
  • Abnormal gait
  • Asymmetric hand function before 12 months
  • Persistence of primitive reflexes
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12
Q

What is spastic cerebral palsy?

A

Damage to upper motor neurone pathway (pyramidal or corticospinal tract)
Increased limb tone (spasticity) and reflexes
Presents early

Hemiplegia - unilateral involvement of arm and leg

  • present at 4-12 months
  • fisting of hand, flexed arm
  • tiptoe (toe-heel) walk

Quadriplegia - all four limbs affected (severe)

  • involvement of trunk, poor head control
  • associated with seizures, microcephaly and intellectual impairment

Diplegia - all limbs, but legs worse than arms
- associated with pre-term birth due to peri-ventricular brain damage

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13
Q

What is dyskinesic cerebral palsy?

A

Involuntary movements more evident with active movement and stress
Muscle tone is variable and primitive reflexes continue
- chorea - irregular, sudden and brief non-repetitive movements
- athetosis - slow, writhing movements occurring distally
- dystonia - simultaneous contraction of agonist and antagonist muscles of the trunk and proximal muscles –> twisting appearance

Intellect may be unimpaired
Commonly caused by HIE –> damage to basal ganglia

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14
Q

What is ataxic (hypotonic) cerebral palsy?

A

Mostly genetic
Early trunk and limb hypotonia, poor balance
Intention tremor
Ataxic gait

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15
Q

How is cerebral palsy treated?

A

Physical therapy
Devices and equipment - crutches, casts (strengthen muscles in another part of the body), wheelchairs
Antispasmodics - baclofen, botox, diazepam
Antiepileptics - lamotrigine
Anticholinergics (help with uncontrollable movements and drooling)
Surgery - increase range of movement, prevent hip dislocation, scoliosis, selective dorsal rhizotomy (cut nerves to tight muscles in legs)

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16
Q

What are generalised seizures and which types are there?

A 
Discharges arise from both hemispheres
Includes:
- absence
- myoclonic
- tonic
- tonic-clonic
- atonic
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17
Q

Describe an absence seizure.

A

Transient loss of consciousness with abrupt onset and termination
Can be precipitated by hyperventilation

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18
Q

Describe a myoclonic seizure.

A

Brief but repetitive jerking movements of limbs, neck and trunk

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19
Q

Describe a tonic seizure.

A

Increase in tone therefore very stiff

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20
Q

Describe a tonic-clonic seizure.

A

Rhythmic contraction of muscle groups following tonic phase
Fall to ground and become cyanosed
Tongue biting and incontinence
Followed by unconsciousness or deep sleep

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21
Q

Describe an atonic seizure.

A

Often combined with myoclonic jerk followed by loss of muscle tone causing drop to floor

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22
Q

What is a focal seizure?

A

Discharges arise from one hemisphere

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23
Q

How does a frontal seizure present?

A

Motor/premotor cortex
Clonic movement
Or assymmetrical tonic seizure

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24
Q

How does a temporal seizure present?

A

Most common type of all
Strange warning feelings with smell and taste anomalies
Lip-smacking
Deja-vu

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25
How does an occipital seizure present?
Visual distortion
26
How does a parietal seizure present?
Altered sensation or distorted body image
27
What is West syndrome?
``` 4-6 months EEG shows hypsarrhythmia Flexor spasms of head, trunk and limbs followed by extension of arms Spasms occur for 1-2 seconds Repear 20-30 times ```
28
What is Lennox-Gastaut syndrome?
1-3 years | Drop attacks, tonic seizures and atypical absences
29
What is childhood absence epilepsy?
4-12 years | EEG shows 3 sec spike and wave discharge
30
What is benign epilepsy?
Tonic-clonic seizures in sleep
31
What is early onset benign childhood occipital epilepsy?
Periods of unresponsiveness in young children | Hallucinations in older children
32
What is juvenile myoclonic epilepsy?
Myoclonic seizures/generalised tonic-clonic or absences after waking
33
What are the side effects of valproate?
Weight gain Hair loss Liver failure
34
What are the side effects of carbamazepine?
Rash Neutropenia Hyponatraemia Ataxia
35
What are the side effects of lamotrigine?
Rash
36
What are the side effects of benzos?
Sedation | Tolerance
37
What are causes of funny turns?
``` Breath holding attacks Reflex anoxic seizures Syncope Migraine BPV Cardiac ```
38
What are common causes of ataxia?
Acute - medications, drugs, alcohol Post viral - varicella Posterior fossa lesions Genetic and degenerative disorders - ataxic cerebral palsy, Friedreich's ataxia, ataxia telangiectasia
39
What is Friedreich's ataxia?
Worsening ataxia Distal wasting in legs Absent lower limb reflexes except Babinski sign Death at 40 due to cardiorespiratory compromise
40
What is ataxia telangiectasia?
Disorder of DNA repair Mild delay in motor development in infancy Subsequent deterioration requiring wheelchair for mobility
41
What is the typical history of breath holding?
Cyanotic - occurs in response to anger or frustration, skin turns blue-purple Pallid - occurs in response to fear, pain or injury esp after head injury
42
What are common causes of developmental regression?
Battens disease - 4-10 years, onset of visual problems and seizures Retts syndrome Leukodystrophies - dysfunction of white matter Wilson's disease - reduced synthesis of copper binding protein therefore accumulation in liver, brain, kidney and cornea SSPE - chronic, progressive encephalitis caused by persistant infection of immune resistant measles
43
What are the types of hydrocephalus?
Obstructive - within ventricular system or aqueduct Communicating - obstruction at arachnoid villi, site of absorption for CSF External - immaturity of villi not absorbing fast enough
44
What are the common causes of macrocephaly?
Head circumference >98th centile ``` Tall stature Familial Raised ICP Hydrocephalus Chronic subdural haematoma Cerebral tumour ```
45
What are the common causes of microcephaly?
Head circumference
46
What is a tension headache?
Symmetrical headache with gradual onset | Usually no other symptoms but could have abdo pain
47
What is a migraine?
Without aura (90%), last 1-72hrs Bilateral, pulsatile over temporal or frontal areas GI disturbances - N+V, abdo pain, photo/phonophobia Physical activity aggravates ``` With aura (10%) - visual disturbances Last a few hours and need to sleep in dark room ```
48
How do reflex anoxic seizures present?
In infants or toddlers Triggers - pain, cold food, fright or fever Become very pale and fall to floor Due to cardiac asystole from vagal inhibition
49
What causes subdural haematoma?
Tearing of veins across subdural space Characteristic in NAI Retinal haemorrhage usually present Can occur falling from height
50
How does craniosynostosis present?
Sutures of skull bone start to fuse during infancy Premature fusion causes distortion of head shape, usually localised May be part of Crouzon syndrome
51
How does myotonic dystrophy present?
Delayed relaxation after sustained muscle contraction can be seen on electromyography Onset between 20-50 years Poor feeding, failure to meet milestones and hypotonia Progressive distal muscle weakness, ptosis, carp mouth, cataracts, frontal balding, cardiomyopathy
52
What is Guillain-Barre?
2-3 weeks post URTI, may take up to 4 years?!? Ascending symmetrical weakness with loss of reflexes and autonomic involvement Involvement of bulbar muscles leads to difficulty chewing and swallowing (may require ventilation) Recovery make take up to 2 years CILP is chronic version
53
What is Bells palsy?
Isolated lower motor neurone paresis of 7th cranial nerve --> facial weakness ?HSV Corticosteroids may help in first week
54
What is Charcot Marie Tooth?
Distal muscle wasting and sensor loss with progression Onset by 10 years: Spinal deformities No cure but most have normal life expectancy
55
What is spinal muscular atrophy?
Autosomal recessive degeneration of anterior horn cells --> progressive weakness and wasting of skeletal muscle Type 1: very severe presenting in early infancy and die within 12 months Type 2: can sit but not walk Type 3: do walk and present later
56
What indications and contraindications for LP?
Indications - suspected meningitis or encephalitis - suspected subarachnoid if CT is normal - septic screen in neonates Contraindications - cardiorespiratory instability - focal neurological signs - raised ICP - coagulopathy - local infection at site
57
What are the values of normal CSF?
Clear fluid Few white cells 0.15-0.4g/l protein Glucose >50% of blood
58
What are LP values in bacterial meningitis?
Turbid (cloudy) fluid Very increased neutrophils Increased protein Decreased glucose
59
What are LP values in viral meningitis?
Clear fluid Increased lymphocytes Normal or increased protein Normal or decreased glucose
60
What are LP values in TB meningitis?
Turbid/clear/viscous fluid Increased lymphocytes Very high protein Very low glucose
61
What are the diagnostic criteria for paediatric migraine?
5 attacks lasting 1-72 hours Unilateral, pulsing, severe pain, aggravated by physical exercise With N+V or photo/phonophobia Made better by sleep
62
How does tension headache present?
Symmetrical headache of gradual onset like a band | Onset in evening
63
What features suggest a space occupying lesion?
``` Headache worse lying down Morning vomiting Night time waking Change in mood/personality Visual field defect Cranial nerve abnormalities ```
64
How are headaches managed?
Rescue treatments - NSAIDs - anti-emetics (prochlorperazine, metochlopramide) - serotonin agonists (sumatriptan) Prophylactic - pizotifen - propanolol - sodium channel blockers (valproate)

Paediatrics (14 decks)

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