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CP3 Medicine > NEURO - MND/Dementia/Neuropathy/Myopathy > Flashcards

NEURO - MND/Dementia/Neuropathy/Myopathy Flashcards

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1
Q

What is motor neurone disease?

A

Degenerative disease of the UMN/LMN of SC/CN + cortex

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2
Q

Which aspect is NOT involved in motor neurone disease?

A

Sensory

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3
Q

What are the 4 types of MND

A

ALS - Amlyotrophic lateral sclerosis
Progressive muscular atrophy
Primary lateral sclerosis
Bulbar

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4
Q

Which is the most common type of MND?

A

ALS

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5
Q

What parts of the nervous system are affected in ALS

A

Loss of Spinal and brainstem LMN

Loss of cortical UMN

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6
Q

Sx ALS (3)

A

LMN weakness - starting in hands + progressing to arms/legs
UMN spastic weakness - starts in legs –> arms
+/- Bulbar/pseudobulbar palsy

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7
Q

O/E ALS (4)

A

Mm wasting
Fasciculations
Brisk reflexes
Increased plantars

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8
Q

Features progressive muscular atrophy MND (3)

A

Loss = RESTRICTED TO SPINAL LMN
Hence
LMN signs only
wasting + fasciculations

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9
Q

Features - primary lateral sclerosis MND (2)

A

Loss RESTRICTED TO CORTICAL UMN

Hence only UMN signs

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10
Q

Features - bulbar MND

A

Bulbar Sx w/ PRESERVATION LIMB FCT

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11
Q

Why is the prognosis for bulbar MND so poor?

A

Early respiratory involvement

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12
Q

Causes of death MND (2)

A

Pneumonia

Ventilatory failure

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13
Q

Dementia - criteria for diagnosis

A

Impairment memory in 1+ of:
Language impairment
Apraxia (motor)
Agnosis (can’t interpret senses)

+
Impairment of fct
Present for at least 6 months
No other medical/physical causes

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14
Q

Modifiable RF dementia (6)

A 
Smoking 
Atherosclerosis 
Alcohol
Cholesterol 
Obesity
Low education
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15
Q

Non-modibiable RF dementia (3)

A

Genetics - APP/PSEN 1/2
AGE
MCI

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16
Q

macroscopic changes - Alzheimers (2)

A

Atrophy of the hippocampus

Enlarged ventricles

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17
Q

Microscopic changes ALzheimers (2)

A

Decr NT function

Abnormal protein depositon –> tangles + plaques

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18
Q

Which 3 genes have been associated with early onset alzheimers

A

APP
PSEN-1
PSEN-2

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19
Q

Which gene has been associated with late onset Alzheimers

A

APOE

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20
Q

Subtypes of vascular dementia (3)

A

Multi-infarct
Subcortical vascular dementia
Post stroke

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21
Q

What is multi-infarct vascular dementia

A

Cortical vascular dementia from small strokes in the cortex

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22
Q

What is subcortical vascular dementia

A

Small vessel deep damage

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23
Q

RF vascular dementia (4)

A

Smoking
High fat diet
Diabetes
HTN

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24
Q

LB Dementia pathology

A

Lewy bodies build up in the cortex and basal ganglia -> disruption NT function

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25
Sx LB dementia (3)
Fluctuations in cognition Visual hallucinations Parkinsonism
26
Difference between Parkinsons dementia and LB dementia
LBD - cognitive dysfunction within 2 years of motor Sx | PDD - presents later
27
What % of frontotemporal dementia is though to be inherited
50%
28
Frontotemporal dementia Sx
Changes in behaviour emotion and language
29
MCI
Impairment of memory more than that of expected age but not enough to affect function
30
Does having MCI put you at greater risk of developing dementia?
Yes
31
What is dysphasia
Cant find words
32
What is agnosia
Difficulty recognising faces
33
Early Dementia Sx (3)
Memory loss Slow to grasp new ideas Errors of judgement
34
Mild-Mod dementia Sx (3)
Difficulty w/ daily tasks Decline in language Behaviour change
35
End stage dementia Sx (4)
Increased need of care COmmunication impaired Incontinence Swallowing difficulty
36
Ix dementia
``` FBC B12/folate ESR LFT U+E TFT Glucose Lipids ECG CT = 1st line 2nd line = MRI or HMPaO-SPECT ```
37
Drugs for Tx alzheimers dementia
ACEase inhibitors - rivastigmine | NDMA antagoists - memantine
38
Can you Tx vascular dementia w/ alzheimers meds
No
39
Use of antipsychotics in dementia
FOr behaviour and psychological Sx
40
Radiculopathy
Process affecting nn roots
41
What is a neuropathy
Peripheral nn affected
42
What is mononeuritis multiplex
Many single nn affected
43
4 most common causes of neuropathies (4)
DM Carcinomatos neuropathy Vit B defic Drugs
44
Other caused neuropathies
``` Folate/thiamine deficiency Uraemia in CKD Alcohol/Pb RA/CT or myxoedema G-B, CIPD Syphillis/ HIV (infective) Vascular Neoplasm ```
45
Drugs causing neuropathies (3)
Amiodarone Statins Hydralazine
46
Inherited neuropathies (2)
Charcot Marie Tooth | Freidrich's ataxia
47
Charcot Marie tooth
Before 20 W/ progresive neuropathy Typically affecting hands + feet 1st
48
Features of Freidrich's ataxia
Cerebellar ataxia in the 1st decade UMN limb signs Peripehral neuropathy
49
How are demyelinating vs axonal neuropathies differentiated?
Nn conduction studies
50
Demyelinating neuropathie
Damages spare axons Affects swann cells --> decreased conduction velocity
51
E.g. demyelinating neuropathy
GBS | Common in immune-mediatedidsease
52
Prognosis demyelinating neuropathy
Schwann cells can regrow hence improve w/ Tx
53
Axonal neuropathy
.Nn cell bodsies untable to maintain long axonal processes Degenerating starts in the periphery ==> neuronal cells Leading to decrease amplitude of impulses
54
Prognosis of axonal neuropathy
Axons cant regrow | Hence poor outcome
55
Wallerian degeneration
Aftect nerve section or microinfarction
56
Compression neuropathy
Leads to focal demyelination
57
Nn infiltration
Malignant or granulomatous infilatration
58
Ix neuropathies (7_
``` FBC U+E LFT Glucose B12/folate Autoantibodies Nn covnduction studies LP - incr GBS/CIDP Peripheral nn biopsy ```
59
What is the most common acute polyneuropathy
GBS
60
Is GBS demyelinating or axonal
Demyelinating
61
How long after 1' infection does GBS occur
3 ish weeks
62
PS GBS (4)
Ascending paralysis Loss of reflexes Affecting lower limbs Progresses over several days to weeks
63
Complications of GBS (3)
VTE BP irregularities Arrhythmias
64
Mx GBS (5)
``` Admit to ITU/HDU Monitor FVC reg Ventilatory support SC heparin + TEDS High dose IVIG within 2w ```
65
What % GBS make a complete recovery over months
90%
66
Mortality rate in acute phase GBS
10%
67
What is shingles
Reactivation of VXV within dosral root ganglion
68
Which dermatomes are most commonly affected by shingles
Low thoracic
69
What is Ramsay Hunt syndrome
infection of geniculate ganglion --? facial palsy, facial/ear pain + vesicules in ear canal/pinna + soft palate
70
Mx Shingles
Aciclovir 5-7days | Paracetamol/amitriptylline - pain
71
What is post-herpetic neuralgia
Pain in a previous shingles zone
72
Mx post-herpetic neuralgia
Amitriptylline
73
What is myasthenia gravis
Disorder of neuromuscular transmission resulting from autoantibodies to components of the NM junction
74
Which receptor is most commonly affected in myasthenia gravis?
Acetylcholine receptor
75
PS myasthenia gravis (6)
Mm weakness + FATIGABILITY - gets worse throughout day Fluctuating proximal weakness (> upper limb) Symmetrical diplopia + ptosis Dysphagia + speech difficulties Wasting + resp difficulties after many years
76
What is preserved in myasthenia gravis
Tendon reflexes | Heart also not affected
77
What are the 3 types of muscle disease
Muscular dystrophies Myopathies Neurogenic mm diseases
78
DMD genetics
X linked recessive | mutation in dystriphin gene - mm breaks down
79
Features DMD (3)
Global weakness Calf pseudohypertrophy Gowers sign
80
Ix DMD
CK increased
81
What is Becker Muscular dystrophy
Milder + better prog version of DMD
82
Gene issue Becker Muscular dystrophy
Partially functioning dystrophin
83
Prognosis Becker Muscular dystrophy
40s
84
Myotonic dystrophy genetics
AD
85
What is the issue with in Myotonic dystrophy
Cl channelopathy
86
PS Myotonic dystrophy
Mm weakness | Myotonia - can't relax
87
Ix mm disease
CK Electromyography Mm biopsy
88
Spinothalamic tract - test (2)
Pain w/ neurotip | Temp - cool and warm object
89
Dorsal column - test (3)
Vibration - tuning fork 128hz Joint proprioception - joint movement Light touch - cotton wool
90
Pyramidal pathway - anatomy
Pathways from cerebral cortex to mm
91
Physiological movement - pyramidal vs extrapyramidal
``` pyramidal = voluntary extrapyramidal = involuntary ```
92
Pathological movements in pyramidal pathology (4)
Paralysis Hyperreflexia Spasticity Paresis
93
Pathological movements in extrapyramidal pathology (5)
``` Chorea Dystonia Myoclonus Tremor Akathisia ```

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