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NEURO > PASSMED > Flashcards

PASSMED Flashcards

1
Q

What is tuberous sclerosis

A

genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neurocutaneous.

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2
Q

cutaneous features of tuberous sclerosis

A

depigmented ‘ash-leaf’ spots which fluoresce under UV light

roughened patches of skin over lumbar spine (Shagreen patches)

adenoma sebaceum (angiofibromas): butterfly distribution over nose

fibromata beneath nails (subungual fibromata)

café-au-lait spots* may be seen

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3
Q

neurological features of tuberous sclerosis

A

developmental delay

epilepsy (infantile spasms or partial)

intellectual impairment

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4
Q

other features of tuberous sclerosis

A

retinal hamartomas: dense white areas on retina (phakomata)

rhabdomyomas of the heart

gliomatous changes can occur in the brain lesions

polycystic kidneys, renal angiomyolipomata

lymphangioleiomyomatosis: multiple lung cysts

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5
Q

features of 3rd nerve palsy

A

eye is deviated ‘down and out’
ptosis
pupil may be dilated (sometimes called a ‘surgical’ third nerve palsy)

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6
Q

causes of third nerve palsy

A

diabetes mellitus
vasculitis e.g. temporal arteritis, SLE
false localizing sign* due to uncal herniation through tentorium if raised ICP
posterior communicating artery aneurysm
pupil dilated
often associated pain
cavernous sinus thrombosis
Weber’s syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes
other possible causes: amyloid, multiple sclerosis

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7
Q

what is charcot marie tooth disease

A

most common hereditary peripheral neuropathy. It results in a predominantly motor loss. There is no cure, and management is focused on physical and occupational therapy.

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8
Q

features of charcto marie tooth disease

A 
There may be a history of frequently sprained ankles
Foot drop
High-arched feet (pes cavus)
Hammer toes
Distal muscle weakness
Distal muscle atrophy
Hyporeflexia
Stork leg deformity
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9
Q

what is syringomyelia

A

a collection of cerebrospinal fluid within the spinal cord.

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10
Q

what is syringobulgia

A

a fluid-filled cavity within the medulla of the brainstem.

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11
Q

causes of syringomyelia

A

a Chiari malformation: strong association
trauma
tumours
idiopathic

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12
Q

features of syrignomyelia

A

‘cape-like’ (neck and arms) loss of sensation to temperature and pain BOTH SIDES

this is because the spinothalamic passes the ventral white commisure which is compressed here

LMN signs - compression of ventral horn

spastic weakness (predominantly of the upper limbs)
paraesthesia
neuropathic pain
upgoing plantars
bowel
bladder dysfunction
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13
Q

Ix for syringomyelia

A

full spine MRI with contrast to exclude a tumour or tethered cord. A brain MRI is also needed to exclude a Chiari malformation.

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14
Q

features of cauda equidina

A

lower back pain
urinary incontinence/retention
reduced sensation in the perianal area
decreased anal tone

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15
Q

Ix for cauda equina

A

urgent MRI

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16
Q

causes of speech and language distrubance

A 
stroke
broca
wernicke
MS
cerbellar
bulbar palsy
CJD
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17
Q

symptoms of CJD

A
  • loss of intellect and memory
  • changes in personality
  • loss of balance and co-ordination
  • slurred speech
  • vision problems
    these are early signs of CJD
  • abnormal jerking movements
  • progressive loss of brain function and mobility
  • blindness
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18
Q

what is CJD

A

degenerative

abnormal infectious protein called a prion.

These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells

aren’t destroyed by the extremes of heat and radiation used to kill bacteria and viruses, and antibiotics or antiviral medicines have no effect on them.

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19
Q

CJD types

A

sporadic - most commone type
45-75

variant CJD - mad 🐄 disease

familial

iatrogenic

20
Q

L5 nerve lesion features

A

weakened dorsiflexion, inversion and eversion of the ankle with normal plantarflexion.

reduced sensation in the first web-space between her big toe and second toe.

21
Q

what is footdrop

A

weakness of the foot dorsiflexors.

22
Q

causes of foot drop

A

common peroneal nerve lesion - the most common cause
L5 radiculopathy
sciatic nerve lesion
superficial or deep peroneal nerve lesion
other possible includes central nerve lesions (e.g. stroke) but other features are usually present

23
Q

causes of syringomyelia

A

hydrocephalus

arnold chiar formation 2 causing dilation of central canal

24
Q

treatment for syringomyeloa

A

surgery

25
Q

prognosis of CJD

A

will die within a year of the symptoms starting

26
Q

what is sporadic CJD

A

most common type

45-75 symptoms airse in 60-65

27
Q

what is variant CJD

A

consuming meat from a cow that have had mad cow disease

28
Q

characteristic appearance of CJD

A

spongiform - holes in barain

29
Q

how is CJD diagnosed

A

EEG - specific pattern

CSF tests - second generation Real Time-Quaking-Induced Conversion (RT-QuIC). - detects pathogenic prion protein in the CSF

MRI - 90% accurate

30
Q

how is CJD confirmed diagnosis

A

brain biopsy or autopsy

31
Q

treatment for CJD

A

no cure

relieving symptoms
- opiate drugs to relieve pain
- clonazepam and sodium valporate - relieve myoclonu
later stages - IV fluids and artificial feeding

32
Q

define autonomic dysreflexia

A

The combination of severe hypertension, flushing and sweating without a congruent response in heart rate in the context of spinal cord injury

33
Q

in who does autonomic dysreflexia occur in

A

patients who have had a spinal cord injury at, or above T6 spinal level

34
Q

what medication is given to treat cerebral/vasogenic oedema

A

dexamethasone

35
Q

what is vasogenic oedema

A

break in the blood brain barrier

36
Q

Mx of neuropathic pain

A

first-line treatment*: amitriptyline, duloxetine, gabapentin or pregabalin
if the first-line drug treatment does not work try one of the other 3 drugs
in contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added
tramadol may be used as ‘rescue therapy’ for exacerbations of neuropathic pain

Other options

Capsaicin cream (chilli pepper cream) for localised areas of pain
Physiotherapy to maintain strength
Psychological input to help with understanding and coping
37
Q

causes of neuropathic pain

A

Postherpetic neuralgia from shingles is in the distribution of a dermatome and usually on the trunk
Nerve damage from surgery
Multiple sclerosis
Diabetic neuralgia typically affects the feet
Trigeminal neuralgia
Complex Regional Pain Syndrome (CRPS)

38
Q

features of neuropathic pain

Ix

A 
Burning
Tingling
Pins and needles
Electric shocks
Loss of sensation to touch of the affected area

DN4 Questionnaire

39
Q

Subacute combined degeneration of the spinal cord (vitamin B12 & E deficiency)

A
  1. Lateral corticospinal tracts
  2. Dorsal columns
  3. Spinocerebellar tracts
  4. Bilateral spastic paresis
  5. Bilateral loss of proprioception and vibration sensation
  6. Bilateral limb ataxia
40
Q

typical features of a post LP headache

A
  • usually develops within 24-48 hours following LP but may occur up to one week later
  • may last several days
  • worsens with upright position
  • improves with recumbent position
41
Q

Mx of post LP headache

A

supportive - analgesia rest
blood patch, epidural saline, IV caffeine
>72 hours

42
Q

what is duchenne muscular atrophy

A

x-linked recessive - absence of dystrophin which connects cytoskeleton of muscle cells

CFs
no abnormality at birth
- apparent by the fourth year
- wheelchair bound by 10
- death by 20 from resp failure or cardiac complications
- proximal muscle weakness
- gower’s sign -> has to climb his legs with his hands
- waddling, difficulty rising from sitting, crouching or lying

Dx
- clinically 
CK grossly elevated
EMG - myopathic
DNA

Mx
- steroids short term improvement

43
Q

what is beckers muscular dystrophy

A

x-linked recessive
dystrophin is altered

begin int first decade
continue to walk into early adult life
cramps ass w exercise
cardiomyopathy worse -> arrhythmias

44
Q

what is myotonic dystrophy

A

autosomal dominant female transmission is the worst

CFs
- progressive proximal and distal muscle weakness in the upper limbs 
- myotonia worse in the cold
- cataracts
- ptosis
dysarthria
- DM
45
Q

what is autonomic dysreflexia

A

spinal cord injury at, or above T6 spinal level

riggered by faecal impaction or urinary retention

extreme hypertension, flushing and sweating above the level of the cord lesion, agitation

NEURO (30 decks)

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