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JL Neurology > Week 3 > Flashcards

Week 3 Flashcards

1
Q

What inheritance is DMD?

A

X-linked recessive

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2
Q

What inheritance is Huntingtons disease?

A

Autosomal dominant

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3
Q

What age of onset does huntingtons disease usually have?

A

30 - 50 years

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4
Q

Name two clinical features of huntington disease?

A

Involuntary movements

Dementia

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5
Q

What are these early clinical signs of - clumsiness, agitation, irratability, apathy, anxiety, disinhibition, delusions/hallucinations, abnormal eye movements and depression?

A

Huntington disease

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6
Q

What gene is mutated in HD?

A

CAG

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7
Q

Name three features of the pathology of alzheimer disease?

A 
Loss of cortical neurones
Neurofibrillary tangles (intracellular)
Senile plaques (extracellular)
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8
Q

What is the term for extracellular protein deposits containing amyloid beta protein?

A

Senile plaques

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9
Q

What type of meningitis shows a thick layer of suppurative exudate covering the leptomeninges over the surface of teh brain?

A

Pyogenic meningitis (exudate in basal and convexity surface, neutrophils in subarachnoid space)

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10
Q

What viruses tend to cause viral meningitis?

A

Enteroviruses e.g. ECHO

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11
Q

How do you diagnose viral meningitis?

A

Viral stool culture, throat swab and CSF PCR.

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12
Q

How do you treat herpes simplex viral encephalitis?

A

Aciclovir IV high doses

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13
Q

What is the meningism triad?

A

nuchal rigidity (neck stiffness), photophobia (intolerance of bright light) and headache.

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14
Q

In neonates - what are the three common causes of bacterial meningitis?

A
  1. Listeria
  2. Group B streptococci
  3. E.coli
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15
Q

In children - what is the commonest cause of bacterial meningitis?

A

H. influenza

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16
Q

21 onwards - what is the c ommonest cause of bacterial meningiti?s

A

Pneumococcal

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17
Q

In the elderly what are the two common causes of bacterial meningitis?

A

Pneumococcal and listeria

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18
Q

Name a risk factor for getting pneumococcal bacterial meningitis?

A

Fracture of the cribiform plate

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19
Q

Name a risk factor for getting staphylococcus, gram negative rods, bacterial meningitis?

A

Neurosurgery/head trauma

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20
Q

Name a risk factor for getting listeria bacterial meningitis?

A

Decreased CMI

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21
Q

What type of H.influenzae is the most common cause of meningitis in children under 4?

A

Type B

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22
Q

What are the antibiotics of choice for listeria?

A

IV ampicillin/amoxicillin

ceftriaxone no value as intrinsically resistant

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23
Q

How do you treat cryptococcal meningitis?

A

IV amphotericin B/Flucytosine

Fluconazole

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24
Q

Fever, stiff neck and alteration in consciousness?

A

Bacterial meningitis

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25
Q

When should you do a lumbar puncture for bacterial meningitis?

A

Only if feasible, treat with antibiotics first

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26
Q

What is the Glu (CSF/SERUM) for CSF predictive of bacterial meningitis?

A

less than 0.23

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27
Q

Aseptic meningitis describes a spinal fluid formula that typically has what?

A
  1. Low number of WBC
  2. Minimally elevated protein
  3. Normal glucose
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28
Q

In relation to acute adult bacterial meningitis - what should be done on all patients with papilloedema or focal neurological signs?

A

CT scan

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29
Q

Should they undergo CT prior to lumbar puncture for meningitis in - immunocompromised state?

A

Yes

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30
Q

Should they undergo CT prior to lumbar puncture for meningitis in - patients with mass lesion or stroke?

A

Yes

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31
Q

Should they undergo CT prior to lumbar puncture for meningitis in - new onset seizures and papilledema?

A

Yes

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32
Q

What is the empiric antibiotic therapy for bacterial meningitis?

A
  1. IV ceftriaxone
  2. Add IV ampicillin/amoxicillin if listeria
  3. If allergy - chloramphenicol IV with vancomycin IV
  4. If allergy - cotrimoxazole
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33
Q

In relation to bacterial meningitis - what do you do with steroids?

A

Give to all patients suspected of it before or with first dose of antibiotics

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34
Q

What has prominent dopaminergic neuron loss in the substantia nigra pars compacta with alpha-synuclein-containing Lewy bodyies and Lewy neurites?

A

Parkinsons

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35
Q

Rigidity, akinesia/bradykinesia and resting tremor?

A

Parkinsonian syndrome

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36
Q

`Dystonia?

A

Prolonged muscle spasms and abnormal postures

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37
Q

Fragments of movements flow irregularly from one body segment to another causing a dance-like appearance.

A

Chorea

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38
Q

What are these non-motor features of - olfactory dysfunction, cognitive impairment, sleep disorders, pain and fatigue?

A

Parkinsosn

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39
Q

How do you diagnose rapid eye movement sleep disorder?

A

Overnight polysomnography

40
Q

How do you treat rapid eye movement sleep behaviour?

A

Clonazepam

Melatonin at bedtime

41
Q

Where is Lewy body pathology found other than brain?

A

Spinal cord and peripheral nervous system

42
Q

What are misfolded alpha-synuclein which is insoluble and aggreagated, forms intracellular inclusions?

A

Lewy bodies

43
Q

What are a neurohistological hallmark of PD?

A

Lewy bodies

44
Q

What is teh greatest PD risk factor?

A

Age

45
Q

What is teh dominant PD gene?

A

LRRK2

46
Q

What is the recessive PD gene?

A

parkin

47
Q

What is teh greatest genetic risk factor fort PD?

A

Mutations in GBA

48
Q

Name a symptomatic treating drug for PD?

A
  1. Levodopa - dopamine agonist
49
Q

What can be effective for tremor in PD?

A

Anticholinergic agent - trihexyphenidyl or clozapine

50
Q

Naem three side effects of dopamine agonist levodopa?

A

Nausea, daytime somnolence and oedema

51
Q

What drug causes gamblong, hypersexuality, binge eating and compulsive spending?

A

Dopamine agonist

52
Q

How is psychosis in PD managed?

A

Clozapine

53
Q

What is late stage demeintia in PD trated with?

A

Rivastigime

54
Q

What type of tremor is pill rolling?

A

Resting

55
Q

Where is cog wheel rigidity normally felt?

A

Wrist

56
Q

What type of parkinsons affects predominantly lower limbs, rest tremor uncomon, brain lesions might be present, poor levodopa response?

A

Vascular parkinsonism

57
Q

What type of parkinsonism tends to be symmetrical, often coarse postural tremor?

A

Drug induced

58
Q

Name a common cause of degenerative parkinonism?

A

Multi system atrophy

59
Q

Dysautonomia, cerebelalr features, parkinsonism?

A

Multi system atrophy

60
Q

What sign is seen on MRI in multi system atrophy?

A

Hot cross bun sign

61
Q

What is symmetric akinetic rigid syndrome with predominantly axial involvement?

A

Progressive supranuclear palsty

62
Q

What type of injury causes relase of excitatory amino acids, binding to receptors, relwease of intracellular calcium, activation of phospholipases, cell swelling and apoptosis?

A

Primary head injury

63
Q

What does MAP - ICP =

A

CPP (cerebral perfusion pressure)

64
Q

Raccoon or panda eyes?

A

Anterior cranial fossa skill base fracture

65
Q

Battle sign over mastoid area?

A

Middle cranial fossa skull base fracture

66
Q

What three categories make up GCS?

A

Eye opening
Verbal response
Best motor response

67
Q

4 eye opening stages in GCS?

A
  1. Spontaneosuly
  2. To command
  3. To pain
  4. None
68
Q

5 verbal response stages in GCS?

A
  1. Orientated
  2. Confused
  3. Inappropriate words
  4. Incomprehensible sounds
  5. None
69
Q

6 best motor response stages in GCS?

A
  1. Obeys command
  2. Localises pain
  3. Flexes to pain
  4. Abnormal flexion
  5. Extension
  6. None
70
Q

Define coma?

A

Do not open eys
Do not obey commands
Do not speak
GCS less than 8

71
Q

When should you do CT scan in head trauma?

A

Any patient with skull fracture
Not orientated GCS less than 15
Focal neurological signs
Taking anti-coagulants (warfarin)

72
Q

During intensive care management of head injury - what is done to reduce cerebral metabolic rate, reduce cerebral blood flow and reduce ICP?

A

Sedation

73
Q

During intensive care management of head injury - what is done to maintain adequate oxygenation and maintain normocapnia?

A

Ventilation

74
Q

During intensive care management of head injury - what is done to manipulate to maintain CPP of more than 60?

A

Blood pressure

75
Q

During intensive care management of head injury - what is done to maintain normoglycaemia?

A

Glucose

76
Q

During intensive care management of head injury - what is done to maintain euthermia?

A

Temperature

77
Q

Give three late effects of head injury?

A

Epilepsy
CSF leak
Cognitive problems

78
Q

Rhythmic sinusoidal oscillation of body part?

A

Tremor

79
Q

Involuntary sterotyped movements or vocalixations?

A

Tics

80
Q

Brief irregular purposeless movements which fit and flow from one body part to another ?

A

Chorea

81
Q

Brief electric shock like jerks?

A

Myoclonus

82
Q

Abnormal posture of the affected body part?

A

Dystonia

83
Q

Give two causes of a kinetic tremor?

A
  1. Cerebellar disease

2. Wilsons disease

84
Q

What investigation might you do for tremor in a young patient?

A

Thyroid function test and copper + coeruloplasmin

85
Q

Give two first line treatment options for dystonic tremor?

A

Propanolol

Primidone

86
Q

What three main physiological abnormalities have been found in patients with dystonia?

A
  1. Loss in reciprocal inhibition
  2. Alterations in brain plasticity
  3. Alterations in sensory function
87
Q

What condition starts before age of 28, usually childhood, starts in limb, usually legs and often family history present?

A

DYT1 - torsion dystonia

88
Q

What surgery is now procedure of choice in dystonia?

A

Deep brain stimulation - but after botox trial

89
Q

Name an inherited cause of chorea?

A

Huntingtons disease

90
Q

Name an autoimmune cause of chorea?

A

SLE

91
Q

Autosomal dominant inherited neurodegenerative disorder characterized by progressive behavioural disturbance, dementia, and movement disorder, usually chorea.

A

hUNTINGTONS DISEASE

92
Q

What is age of onset of tourette syndrome?

A

Less than 18

93
Q

What is symptomatic treatment for tourette syndrome?

A

Clonidine and tetrabenazine

94
Q

What conditions has typical precipitatns of the myoclonic jerks and seizures being alcohol and sleep deprivation?

A

Juvenile Myoclonus Epilespy

95
Q

What does EEG show for Juvenile Myoclonus Epilepsy?

A

3-5 Hz polyspike and wave pattern

96
Q

What are sodium valproate and levetiracetam effective in treating?

A

Juvenile Myoclonus Epilepsy

JL Neurology (12 decks)

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