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Flashcards in Week 3 Deck (96):
1

What inheritance is DMD?

X-linked recessive

2

What inheritance is Huntingtons disease?

Autosomal dominant

3

What age of onset does huntingtons disease usually have?

30 - 50 years

4

Name two clinical features of huntington disease?

Involuntary movements
Dementia

5

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

Huntington disease

6

What gene is mutated in HD?

CAG

7

Name three features of the pathology of alzheimer disease?

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

8

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

Senile plaques

9

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

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

10

What viruses tend to cause viral meningitis?

Enteroviruses e.g. ECHO

11

How do you diagnose viral meningitis?

Viral stool culture, throat swab and CSF PCR.

12

How do you treat herpes simplex viral encephalitis?

Aciclovir IV high doses

13

What is the meningism triad?

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

14

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

1. Listeria
2. Group B streptococci
3. E.coli

15

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

H. influenza

16

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

Pneumococcal

17

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

Pneumococcal and listeria

18

Name a risk factor for getting pneumococcal bacterial meningitis?

Fracture of the cribiform plate

19

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

Neurosurgery/head trauma

20

Name a risk factor for getting listeria bacterial meningitis?

Decreased CMI

21

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

Type B

22

What are the antibiotics of choice for listeria?

IV ampicillin/amoxicillin
(ceftriaxone no value as intrinsically resistant)

23

How do you treat cryptococcal meningitis?

IV amphotericin B/Flucytosine
Fluconazole

24

Fever, stiff neck and alteration in consciousness?

Bacterial meningitis

25

When should you do a lumbar puncture for bacterial meningitis?

Only if feasible, treat with antibiotics first

26

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

less than 0.23

27

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

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

28

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

CT scan

29

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

Yes

30

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

Yes

31

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

Yes

32

What is the empiric antibiotic therapy for bacterial meningitis?

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

33

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

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

34

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

Parkinsons

35

Rigidity, akinesia/bradykinesia and resting tremor?

Parkinsonian syndrome

36

`Dystonia?

Prolonged muscle spasms and abnormal postures

37

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

Chorea

38

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

Parkinsosn

39

How do you diagnose rapid eye movement sleep disorder?

Overnight polysomnography

40

How do you treat rapid eye movement sleep behaviour?

Clonazepam
Melatonin at bedtime

41

Where is Lewy body pathology found other than brain?

Spinal cord and peripheral nervous system

42

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

Lewy bodies

43

What are a neurohistological hallmark of PD?

Lewy bodies

44

What is teh greatest PD risk factor?

Age

45

What is teh dominant PD gene?

LRRK2

46

What is the recessive PD gene?

parkin

47

What is teh greatest genetic risk factor fort PD?

Mutations in GBA

48

Name a symptomatic treating drug for PD?

1. Levodopa - dopamine agonist

49

What can be effective for tremor in PD?

Anticholinergic agent - trihexyphenidyl or clozapine

50

Naem three side effects of dopamine agonist levodopa?

Nausea, daytime somnolence and oedema

51

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

Dopamine agonist

52

How is psychosis in PD managed?

Clozapine

53

What is late stage demeintia in PD trated with?

Rivastigime

54

What type of tremor is pill rolling?

Resting

55

Where is cog wheel rigidity normally felt?

Wrist

56

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

Vascular parkinsonism

57

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

Drug induced

58

Name a common cause of degenerative parkinonism?

Multi system atrophy

59

Dysautonomia, cerebelalr features, parkinsonism?

Multi system atrophy

60

What sign is seen on MRI in multi system atrophy?

Hot cross bun sign

61

What is symmetric akinetic rigid syndrome with predominantly axial involvement?

Progressive supranuclear palsty

62

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

Primary head injury

63

What does MAP - ICP =

CPP (cerebral perfusion pressure)

64

Raccoon or panda eyes?

Anterior cranial fossa skill base fracture

65

Battle sign over mastoid area?

Middle cranial fossa skull base fracture

66

What three categories make up GCS?

Eye opening
Verbal response
Best motor response

67

4 eye opening stages in GCS?

1. Spontaneosuly
2. To command
3. To pain
4. None

68

5 verbal response stages in GCS?

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

69

6 best motor response stages in GCS?

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

70

Define coma?

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

71

When should you do CT scan in head trauma?

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

72

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

Sedation

73

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

Ventilation

74

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

Blood pressure

75

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

Glucose

76

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

Temperature

77

Give three late effects of head injury?

Epilepsy
CSF leak
Cognitive problems

78

Rhythmic sinusoidal oscillation of body part?

Tremor

79

Involuntary sterotyped movements or vocalixations?

Tics

80

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

Chorea

81

Brief electric shock like jerks?

Myoclonus

82

Abnormal posture of the affected body part?

Dystonia

83

Give two causes of a kinetic tremor?

1. Cerebellar disease
2. Wilsons disease

84

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

Thyroid function test and copper + coeruloplasmin

85

Give two first line treatment options for dystonic tremor?

Propanolol
Primidone

86

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

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

87

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

DYT1 - torsion dystonia

88

What surgery is now procedure of choice in dystonia?

Deep brain stimulation - but after botox trial

89

Name an inherited cause of chorea?

Huntingtons disease

90

Name an autoimmune cause of chorea?

SLE

91

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

hUNTINGTONS DISEASE

92

What is age of onset of tourette syndrome?

Less than 18

93

What is symptomatic treatment for tourette syndrome?

Clonidine and tetrabenazine

94

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

Juvenile Myoclonus Epilespy

95

What does EEG show for Juvenile Myoclonus Epilepsy?

3-5 Hz polyspike and wave pattern

96

What are sodium valproate and levetiracetam effective in treating?

Juvenile Myoclonus Epilepsy