Neurology Flashcards

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
Q

How does an occipital seizure present?

A

Visual distortion

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26
Q

How does a parietal seizure present?

A

Altered sensation or distorted body image

27
Q

What is West syndrome?

A
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
Q

What is Lennox-Gastaut syndrome?

A

1-3 years

Drop attacks, tonic seizures and atypical absences

29
Q

What is childhood absence epilepsy?

A

4-12 years

EEG shows 3 sec spike and wave discharge

30
Q

What is benign epilepsy?

A

Tonic-clonic seizures in sleep

31
Q

What is early onset benign childhood occipital epilepsy?

A

Periods of unresponsiveness in young children

Hallucinations in older children

32
Q

What is juvenile myoclonic epilepsy?

A

Myoclonic seizures/generalised tonic-clonic or absences after waking

33
Q

What are the side effects of valproate?

A

Weight gain
Hair loss
Liver failure

34
Q

What are the side effects of carbamazepine?

A

Rash
Neutropenia
Hyponatraemia
Ataxia

35
Q

What are the side effects of lamotrigine?

A

Rash

36
Q

What are the side effects of benzos?

A

Sedation

Tolerance

37
Q

What are causes of funny turns?

A
Breath holding attacks
Reflex anoxic seizures
Syncope
Migraine
BPV
Cardiac
38
Q

What are common causes of ataxia?

A

Acute - medications, drugs, alcohol
Post viral - varicella
Posterior fossa lesions
Genetic and degenerative disorders - ataxic cerebral palsy, Friedreich’s ataxia, ataxia telangiectasia

39
Q

What is Friedreich’s ataxia?

A

Worsening ataxia
Distal wasting in legs
Absent lower limb reflexes except Babinski sign
Death at 40 due to cardiorespiratory compromise

40
Q

What is ataxia telangiectasia?

A

Disorder of DNA repair
Mild delay in motor development in infancy
Subsequent deterioration requiring wheelchair for mobility

41
Q

What is the typical history of breath holding?

A

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
Q

What are common causes of developmental regression?

A

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
Q

What are the types of hydrocephalus?

A

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
Q

What are the common causes of macrocephaly?

A

Head circumference >98th centile

Tall stature
Familial
Raised ICP
Hydrocephalus
Chronic subdural haematoma
Cerebral tumour
45
Q

What are the common causes of microcephaly?

A

Head circumference

46
Q

What is a tension headache?

A

Symmetrical headache with gradual onset

Usually no other symptoms but could have abdo pain

47
Q

What is a migraine?

A

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
Q

How do reflex anoxic seizures present?

A

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
Q

What causes subdural haematoma?

A

Tearing of veins across subdural space
Characteristic in NAI
Retinal haemorrhage usually present
Can occur falling from height

50
Q

How does craniosynostosis present?

A

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
Q

How does myotonic dystrophy present?

A

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
Q

What is Guillain-Barre?

A

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
Q

What is Bells palsy?

A

Isolated lower motor neurone paresis of 7th cranial nerve –> facial weakness
?HSV
Corticosteroids may help in first week

54
Q

What is Charcot Marie Tooth?

A

Distal muscle wasting and sensor loss with progression
Onset by 10 years:
Spinal deformities

No cure but most have normal life expectancy

55
Q

What is spinal muscular atrophy?

A

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
Q

What indications and contraindications for LP?

A

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
Q

What are the values of normal CSF?

A

Clear fluid
Few white cells
0.15-0.4g/l protein
Glucose >50% of blood

58
Q

What are LP values in bacterial meningitis?

A

Turbid (cloudy) fluid
Very increased neutrophils
Increased protein
Decreased glucose

59
Q

What are LP values in viral meningitis?

A

Clear fluid
Increased lymphocytes
Normal or increased protein
Normal or decreased glucose

60
Q

What are LP values in TB meningitis?

A

Turbid/clear/viscous fluid
Increased lymphocytes
Very high protein
Very low glucose

61
Q

What are the diagnostic criteria for paediatric migraine?

A

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
Q

How does tension headache present?

A

Symmetrical headache of gradual onset like a band

Onset in evening

63
Q

What features suggest a space occupying lesion?

A
Headache worse lying down
Morning vomiting
Night time waking
Change in mood/personality
Visual field defect
Cranial nerve abnormalities
64
Q

How are headaches managed?

A

Rescue treatments

  • NSAIDs
  • anti-emetics (prochlorperazine, metochlopramide)
  • serotonin agonists (sumatriptan)

Prophylactic

  • pizotifen
  • propanolol
  • sodium channel blockers (valproate)