Week 3 Flashcards

(96 cards)

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
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