Hydrocephalus Flashcards

1
Q

monro kellie hypothesis

A
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2
Q

signs of hydrocephalus from birth

A
  • large head, thin and shiny scalp withb easily visible veins
  • bulging fontanelle
  • sunsetting eyes
  • poor feeding, irritability, muscle stiffness, vomiting, sleepiness etc
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3
Q

prostitute sign

A

eyes accomodate but dont react to light

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4
Q

chiari malformation I

A

displacement of the cerebellar tonsils downthrough the foramen magnum

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5
Q

what can intrauterine hydrocephlaus do to the cerebellar tonsils

A

cause them to herniate down through FM –> Chiari malformation I

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6
Q

which type of chiari is more common and less severe

A

I

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7
Q

CF of chiari I

A

headache (coughing, suboccipital pain, can be brought on by neck extension), downbeat nystagmus, central cord symptoms, ataxic gait

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8
Q

chiari malformation II

A

caudal displacement of the cerebellum and medulla below foramen magnum with herniation of 4th ventricle

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9
Q

how is chiari associated with spina bifida

A

spina bifida creates an opening in the spinal cord, CSF is lost, and the brain is pulled down into teh spinal cord (Chiari)

leads to obstructive hydrocephalus

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10
Q

where is a shunt placed

A

one end into lateral ventricles, drains into abdominal cavity

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11
Q

symptoms of shunt over drainage

A
  • slit like ventricles
  • subdural haematoma
  • orthostatic headache (relieved when lying down - CSF flows to brain??)
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12
Q

how does shunt over drainage cause subdural haematoma

A

ventricles collapse, tear blood vessels, haemorrhage, haematoma

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13
Q

what organisms are usually the cause of shunt infection

A

skin commensals (staph epidermidis most common), or 2y to abdominal infection

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14
Q

how can shunt infection be reduced

A

impregnate tube with silver or ABx

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15
Q

what symptoms does a blocked shunt cause

A

hydrocephalus

  • headache and vomiting
  • sunsetting in kids, lack of upgaze in adults
  • stomach pain (drains into abdomen)
  • beware of blurred vision! papilloedema precedes blindness but can be rapid
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16
Q

what investigations can be done for shunt malfunction

A
  • tap in extreme cases - send CSF to microbiology
  • CT scan to demonstrate hydrocephalus
  • surgery to replace shunt
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17
Q

why is there a big risk associated with tapping shunt valve

A

contamination with skin flora

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18
Q

normal pressure hydrocephalus

A

accumulation of CSF causes ventricles to become enlarged, with little/no increase in ICP of CSF, despite this there is the potential to respond to CSF diversion

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19
Q

name 2 risk factors for NPH

A

old age and vascular disease (decreased arterial compliance is thought to be an aspect of the underlying pathology)

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20
Q

classic traid of NPH presentation

A

ataxia, memory decline and incontinence

also CF of hydrocephalus

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21
Q

what is DD often misdiagnosed as

A

Parkinsons or Alzheiemrs

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22
Q

which symptom precedes all others in NPH

A

ataxia - essential to diagnosis

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23
Q

describe the ataxia with NPH

A
  • Inability to walk in standing position, able to make walking movements while lying
  • Broad based shuffling gait
  • Difficulty rising from chair
  • Tendency to fall backwards
  • Difficulties initiating gait, slow cautious gait
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24
Q

what drug is the ataxia in NPH unresponsive to

A

levodopa

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25
Q

presentation of dementia in NPH

A

Mental slowing (e.g. increased response latency, attention difficulties, impairment of abstract thinking and insight) and memory impairment (e.g. impaired recall)

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26
Q

what is the onset of urinary incontinence like in NPH

A

generally insidious

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27
Q

imaging of NPH

A

CT or MRI

May be normal, show evidence of ventricular enlargement or damage to white matter around cerebral ventricles

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28
Q

levodopa challenge in NPH

A
  • Levodopa challenge in all patients with suspected Parkinson’s disease – diagnosis ruled out if ataxia symptoms show no response
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29
Q

further investigations of NPH

A
  • LP to measure ICP - normal/slightly raised
  • LP tap test - to determine if there wil be a benefit in shunting and to support diagnosis
  • lumbar drainage studies
  • infusion studies
30
Q

outline a LP tap test procedure

A
  • remove 30ml CSF through LP
  • assess gait symptoms etc
  • if there is an improvement - indication for shunting
31
Q

what are the expected outcomes after shunting NPH?

A

tends to have good results for gait problems, poor results incases of pronounced dementia

32
Q

how long do the benefits of shunting tend to last in NPH

A

around a year

33
Q

is the patient with IIH alert or confused?

A

alert and orientated

34
Q

clinical features of IIH

A

increased ICP: headache, papilloedema, constricted visual fields

35
Q

what is the headache like in IIH

A

pulsatile, morning, associated nausea

36
Q

what happens to your visiion in IIH

A

constricted visual fields (usualy mild at first and not noticed by patients, inferonasal portions of visual field affected first)

enlarged blind spot

precedes loss of visual acuity

37
Q

what structural damage can IIH go on to cause, and the clinical sign

A

CNVI palsy - horizontal diplopia

38
Q
A
39
Q

what is the classical patient associated with IIH

A

female of child bearing age - almost always associated with increased weight

40
Q

what condition is IIH strongly associated with

A

sleep apnoea

41
Q

what causes the onset of IIH

A

can just happen, often occurs in the setting of weight gain

42
Q

investigations of IIH

A

MRI, visual field, optic photography, LP

43
Q

what is seen on MRI of IIH

A

negative intracranial pathology, empty sella

44
Q

what is seen on visual field testing and fundoscopy of IIH

A
  • Visual field testing: enlargement of blind spot and loss of inferonasal portions of visual field
  • Fundoscopy: bilateral papilloedema
45
Q

what is the course of IIH like

A

can be self limiting or life long chronic

46
Q

main treatment for IIH

A

weight loss, low soidum weight reduction programme is recommended

47
Q

pharmacological management of IIH

A
  • acetazolamide
  • loop diuretics
  • prednisolone
48
Q

how does acetazolamide work for IIH

A

dec the production of CSF by inhibiting CA - causes a reduction in the transport of Na ions across the choroid plexus

49
Q

surgical management of IIH

A

LP/VP shunt

50
Q

what surgical option is available to help with visual problems

A

optic nerve sheath fenestration - incision in meninges to relieve pressure

subtemporal compression - remove some bone to relieve pressure

51
Q

what causes spontaenous intracranial hypotension (SIH)

A

CSF leak

  • post surgical, post LP
  • idiopathic
  • collagen and connective tissue disorders
  • trauma
52
Q

how do collagen and connective tissue disorders predispose one to SIH

A

predispose pt to dural diverticuli, can rupture

eg marfans, PKD, NF

53
Q

presentatio of SIH

A

orthostatic headache

  • worse with upright posture, eases off when lying down
  • onset is usually gradual
  • generalised, throbbing headache

also, neck/interscapular pain, visual field defects, dizziness, muffled hearing etc

54
Q

investigation of SIH

A

MRI of head

LP - low CSF pressure

55
Q

what is a syrinx

A

fluid filled cavity within the spinal cord

56
Q

what does syringomyelia mean

A

cavity within the brainstem

57
Q

which congenital malformation is syringomyelia v commonly associated with

A

arnold chiari

58
Q

describe the disease course/symptom onset of syringomyelia

A

is a very slow but progressive disease, cavity will expand and lengthen over time

symptoms will likely be long standing and progressive, symptoms can be static for years and suddenly worsen

59
Q

will syringomyelia go away by itself

A

no, sponatenous resolution is rare - surgery required

60
Q

pathology of syringomyelia

A

The expanding cavity gradually destroys spinothalamic neurons, anterior horn cells and lateral corticospinal tracts. In the medulla (syringobulbia), lower central nerve nuclei are affected.

61
Q

is syringomyelia more common in male or female

A

neither

62
Q

mean age of presentation of syringomyelia

A

30s

63
Q

acquired causes of syringomyelia

A

POST-TRAUMATIC

  • Common in traumatic paraplegia victims
  • Can occur after spinal trauma
  • Intrinsic cord tumours
64
Q

what is a holocord syrinx

A

involves the entire spinal cord

65
Q

key points in history of syringomyelia

A

recent head or neck trauma

recent meningitis

ARNOLD CHIARI

66
Q

sensory symptoms of syringomyelia

A
  • damage to ALS tract in the cervical and upper thoracic regions causes loss of pain and temperature sensation in a cape like distribution
  • fine touch, vibration and proprioception are preserved (DCML)
67
Q

what clinical signs reflect the damage to ALS tract

A

cuts and burns on hands

68
Q

motor symptoms in syringomyelia

A

upper limb motor impairement and loss of upper limb reflexes

leads to small muscle wasting in the hands - clawed hands and Charcot’s arthropathy

this can progress to involve arms, shoulders and respiratory muscles

69
Q

what features indicate syringomyelia has damaged the autonomic nervous system

A

hyperhidrosis, Horner’s, bladder and bowel dysfunction

70
Q

what disease are CHiari malformations and syringomyelia associated with

A

sleep apnoea