Neurology 1 Flashcards
1
Q
Pathophysiological background of MS?
A
Autoimmune condition causing episodes of inflammation, demyelination and subsequent sclerosis along CNS nerves (impairing motor and or sensory function), disseminated over space and time
2
Q
What is the most common type of MS?
A
Relapsing-remitting
3
Q
What form of MS eventually follows on from relapsing-remitting?
A
Secondary progressive
4
Q
4 classifications of MS?
A
Relapsing/remitting
Secondary progressive
Primary progressive
Fulminant
5
Q
Typical patient with MS?
A
Caucasian woman in her early adulthood
6
Q
What effect does pregnancy have on MS?
A
Less likely to remit during pregnancy
More likely to remit just postpartum
7
Q
Common visual presentation of MS?
A
Optic neuritis - often unilateral painful movement of the eye, diplopia, decline in central vision
8
Q
What is Uhthoff’s phenomenon?
A
MS - vision is worse in hot temps or after exercise
9
Q
What is the most common eye sign of MS?
A
Symmetrical horizontal jerking nystagmus (due to lateral rectus weakness)
10
Q
What is Lhermitte’s sign?
A
MS - neck flexion in a patient with cervical spinal cord lesions causes ‘electric shocks’ in trunk and limbs
11
Q
What type of paraesthesia is classical of MS?
A
Perineal/genitalia
12
Q
What type of motor deficits can occur in MS?
A
UMNL - spastic weakness
Transverse myelitis - loss of everything below level of lesion
13
Q
What is Devic’s syndrome?
A
Special MS - Neuromyelitis optica + Devic’s syndrome
14
Q
Common sexual/GU Sx of MS?
A
Perineal/genital paraesthesia
ED
Continence issues or retention
15
Q
What is the diagnostic criteria of ‘attacks’ in MS?
A
Attacks last > 1hr, >30d between attacks
16
Q
Is a bilateral optic neuritis suggestive of MS?
A
No not really
17
Q
What criteria is used in clinical diagnosis of MS?
A
McDonalds criteria
18
Q
3 investigations that can be used to support a diagnosis of MS?
A
MRI
CSF analysis
Evoked potentials - visual, auditory, somatosensory
19
Q
What might be visible on CSF analysis in MS?
A
Oligoclonal IgG bands, high Ig index
20
Q
What CSF finding is highly specific for Devic’s syndrome?
A
NMO-IgG
21
Q
What does NMO-IgG in CSF point towards?
A
Devic’s syndrome
22
Q
What is given in acute relapse management of MS?
A
Methylprednisolone
23
Q
What is the role of interferon use in R/R MS?
A
Reducing relapses
24
Q
2 alternatives to interferon to reduce relapses in R/R MS?
A
Glatiramer acetate
Dimethyl fumerate
25
Most commonly used Mab in MS?
Natalizumab
26
What type of motor neurone lesion is MS? (UMNL vs LMNL)
UMNL
27
What is the pathophysiology behind PD?
Loss of dopamine at the ventral tier substantia nigra pars compacta leading to degeneration of the nigrostriatal pathway
Lewy body deposition
28
RFs for PD?
Elderly male
Pesticide exposure
Head trauma
29
PART of PD symptoms?
Postural instability
Akinesia/bradykinesia, hypokinesia
Rigidity - lead pipe
Tremor at rest
30
How does bradykinesia in PD manifest clinically?
Slow gait, reduced arm swing
Reduced amplitude of repeated action -> micrographia
Reduced eye blinking, mask like facial expression
31
How does rigidity manifest in PD?
Difficulty initiating movement
| Resistance to passive movement - lead pipe, cogwheeling
32
What is characteristic of the tremor in PD?
Starts unilateral, often becomes bilateral and generalised
Slow (4-6Hz) tremor at rest, may be obliterated with concentration
Worse in stress and often absent at night
33
Describe the gait in PD?
Festinant, shuffling - difficulty initiating movement, difficulty in tight spaces
Unsteady with reduced arm swing
34
3 symptoms of PD that may precede diagnosis by years?
Anosmia
Constipation
Sleep disturbance
35
Non-motor features of PD?
```
Voice - quiet, slurred, monotone
Micrographia
Incontinence
Greasy skin
Drooling and neuromuscular dysphagia
Sleep disturbance
Pain
Cognitive deficit (inability to multitask) and dementia
```
36
What happens to the skin in PD?
Becomes greasy -> seborrhoeic dermatitis
37
What are the 3 types of PD? (In terms of dominance)
Tremor dominant
Akinesia dominant
Mixed
38
Exclusion criteria for PD?
Background causes e.g. Stroke, head injury, neuroleptics
SN gaze palsy
Cerebellar signs, Babinski
39
When is PD dementia more likely than Alzheimer's dementia in a known PD patient?
If it occurs after 1-2 years of diagnosis
40
Although PD is a clinical diagnosis, what imaging scans may help to support a diagnosis?
CT/MRI
| DAT/SPECT scans
41
What must be given alongside LevoDopa for PD and why?
A dopamine decarboxylase inhibitor DDCI
| Because dopamine can't cross the BBB
42
2 levodopa + DDCI combos?
Sinemet - Cocareldopa - Ldopa + carbidopa
| Madopar - Cobeneldopa - Ldopa + benserazide
43
Short term SEs of L Dopa treatment?
Nausea and vomiting, dizziness
Confusion
Hypotension
44
Long term SEs of L Dopa treatment?
'Wearing off'
End of dose/peak dose dyskinesia
On and off fluctuation
DA dysregulation syndrome
45
What are non-ergot DA agonists? Who are they used for?
Rapinorole
Cabergoline
For younger patients
46
What is apomorphine and what is it used for?
Powerful DA agonist
47
What are 2 COMT inhibitors?
Entacapone, tolcapone
48
MAOI inhibitor used in PD?
Rasagiline
49
What does benign essential tremor traditionally affect?
Hands and voice
50
Cerebellar vs PD tremor?
Cerebellar is intention tremor causing past pointing
| PD is low frequency resting tremor
51
What limbs does vascular PD typically affect? Why is it interesting in terms of RFs vs normal PD?
Affects legs
| Normal PD rare in smokers
52
What is Sydenham's chorea?
Post strep pyogenes (GAS) rheumatic fever infection
| Most common cause of acute chorea +/- psych Sx
53
What are the 2 Parkinson's plus syndromes?
Progressive supranuclear palsy PSNP
| Multi-system atrophy
54
What facial signs are suggestive of PSNP?
Mega brow lines due to frontalis hyperactivity, as patient can't voluntarily look up or down so the muscle overcompensated
55
What neck sign is suggestive of multi-system atrophy?
Can't look down voluntarily -> cord involvement, incontinence, autonomic failure
56
Symptoms of brown sequard/cord hemisection?
Contralateral pain and temp loss below lesion
Ipsilateral touch vibration proprioception loss below lesion
Ipsilateral spastic paralysis below lesion
57
Symptoms of LMNL? What side are they?
```
Flaccid paraplegia/paralysis
Fasciculations
Muscle wastage
Hypotonia
Hyporeflexia
On ipsilateral side of lesion
```
58
UMNL symptoms? What side are they?
```
Pyramidal (spastic) weakness/paralysis
Hyperreflexia
Hypertonia
Positive Babinski
Loss of abdo reflexes
Clasp knife responses, Hoffmans
Contralateral signs
```
59
What side of the body are unilateral cerebellar lesions?
Ipsilateral
60
DANISH of cerebellar signs?
```
Dysdiadochokinesia and dysmetria
Ataxic gait
Nystagmus
Intention tremor
Speech - dysarthria, scanning
Hypotonia, hyposensitivity
```
61
What does Rombergs sign indicate? Is it a cerebellar sign?
Sensory ataxia
| Not a sign of cerebellar dysfunction
62
What is Kernig's sign?
Flex one leg at hip and knee
| Then straighten leg -> resisted by spasm in hamstrings, other limb may flex
63
Why is Kernig's sign so useful in detecting meningeal irritation?
Because it's not positive in localised neck stiffness
64
Brudzinski neck sign?
Flexion of neck leads to flexion of hips, indicating meningeal irritation
65
3 classical signs of meningism?
Headache
Neck stiffness
Photophobia
66
What is subacute combined degeneration of the spinal cord?
B12 deficiency - both UMNL cortiospinal/post column and peripheral nerve degeneration leading to peripheral vibration, proprioception and possibly pain and temp (glove and stocking) loss as well as +ve Babinski and spasticity
67
What is Bells Palsy?
A unilateral LMNL of CN7 causing upper and lower facial muscle weakness on the affected side, often preceded by pain
68
What is the characteristic look of a unilateral UMNL of the facial nerve?
There is upper facial muscle sparing because there is bilateral cortical representation of these nerves
Lower facial muscles are weak
69
What is Ramsey Hunt syndrome?
CN7 invasion by HZV, can affect CN8
70
What side does the uvula deviate to in a CN10 LMNL?
Away from the side of the lesion
71
What does a recurrent laryngeal nerve palsy cause?
Dysphonia and a bovine cough
72
What does dysphonia and a bovine cough suggest?
Recurrent laryngeal nerve damage
73
How do you assess for dysarthria and dysphonia?
Listen to speech
Repeat yellow lorry (lingual)
Say baby hippopotamus (labial)
Tongue twister (she sells sea shells...)
74
What is the test for CN9 sensation that isn't routinely performed in CN exam?
Gag reflex
75
What is bilateral weakness and wastage of the sternocleidomastoids characteristic of?
Myotonic dystrophy
76
What is myotonia?
Slow relaxation of muscles after contraction
77
5 things characteristic of myotonic dystrophy?
```
Myotonia
Bilateral sternocleidomastoid weakness and wastage
Cataracts
Heart defects
Endocrine abnormalities
```
78
What does a unilateral LMNL of CN12 do?
Makes the tongue lick the wound - weakness on side of and deviation towards the side of the lesion
79
What causes a bulbar palsy?
LMNL to CNs 9, 10, 11, 12
| Often caused by MND (progressive bulbar palsy)
80
What are the characteristic signs of bulbar palsy?
Dysarthria NO dysphonia
Dysphagia
Tongue weakness, wastage, fanciful action
No jaw jerk or emotional lability
81
What causes a pseudobulbar palsy?
Bilateral UMNL to 9, 10, 11, 12 and often 5 and 7
| Often in context of MND, CVA or occasionally MS
82
Symptoms of a pseudobulbar palsy?
```
Dysarthria and dysphonia
Dysphagia
Spastic and conical tongue
+ve jaw jerk, emotional lability
And CN7 signs of UMNL
```
83
What is the most common type of MND?
ALS Amyotrophic Lateral Sclerosis
84
What is progressive bulbar palsy?
A type of MND which affects CNs first
85
What is progressive muscular atrophy?
A type of MND that is pure LMNL, affecting the small muscles in hands and feet first
86
What is primary lateral sclerosis?
A type of MND causing a pure UMNL of leg muscles
87
MND type affecting CNs first?
Progressive bulbar palsy
88
MND type providing pure LMNL of small hand/feet muscles?
Progressive muscular atrophy
89
Type of MND affecting leg muscles in UMNL fashion?
Primary lateral sclerosis
90
What is the type of motor neurone lesion in MND?
Mixed picture of UMNL and LMNL
| However LMNL often predominates (think progressive bulbar palsy)
91
What is the most common presentation of ALS?
Focal onset affecting one muscle group in upper limb (unless primary lateral sclerosis)
92
What is often the first signs of progressive bulbar palsy?
Dysarthria and tongue signs
Dysphagia is late
Emotional lability may be there
93
What sign (of LMNL) is often prominent in MND?
Fasciculations
94
Is there any pain or sensory disturbance in MND? What does this differentiate from?
Nope, unlike in radiculopathies
95
2 diagnostic pointers of MND?
Asymmetrical distal weakness
| Brisk reflexes in a wasted limb
96
What are the Charcot Marie Tooth diseases?
A group of inherited peripheral neuropathies (LMNLs) that typically cause distal limb muscle wasting and sensory disturbance, progressing proximally over time
97
What are 'inverted champagne bottle' legs typical of?
CMT disease
98
What is the pathogenesis behind myasthenia gravis?
Autoimmune disease causing destruction of the post-synaptic Ach receptors at the nmj
99
When does MG normally present in each sex?
Younger women - 20s
| Older men - 50s/60s
100
Tumour linked with MG?
Thymoma
101
What neurological disorder is associated with thymoma?
MG
102
Common presentation of MG?
Ocular muscle weakness (class I MG)
103
Worst presentation of MG?
Severe weakness in multiple groups (class V/generalised MG)
104
What is the characteristic feature of MG?
Easy muscle fatiguability - worsened by exercise e.g. Progressive weakness whilst counting up to 30, ocular muscle weakness and ptosis
105
Are there any abnormal neurological findings on exam in MG?
No - just fatiguability
106
Over what sort of time period have most MG patients progressed from grade I -> V?
Over a year
107
What is an acute MG attack called and what is the major danger?
Myaesthenic crisis, major danger is respiratory inadequacy
108
What simple test is highly sensitive and specific for MG?
Ice cube test - putting ice on eyes improved ptosis
109
Two blood tests that are highly specific for MG?
AchR Abs
| MnSK Abs
110
What medication can be given to transiently improve Sx in MG and may be used in investigation?
Achesterase inhibitors
111
Medical management of MG?
Immunosuppressants
112
What is Eaton-Lambert myaesthenic syndrome?
A paraneoplastic syndrome associated with SCLC, causing proximal muscle fatiguability eased by exercise
113
3 types of neurogenic syncope?
Vasovagal
Situational
Carotid sinus hypersensitivity
114
3 major causes of cardiac syncope?
LVOT obstruction e.g. Aortic stenosis
Arrhythmias - 3rd degree heart block, VT
Cardiomyopathy (e.g. Hypertrophic obstructive)
115
What type of tongue biting specifically is more indicative of seizure?
Lateral tongue biting
116
3 major signs of seizure as opposed to syncope?
Not regaining full consciousness/awareness until being in ambulances etc.
Lateral tongue biting
Lip cyanosis (respiratory apnoea)
117
What is a simple partial seizure?
Seizure affecting one cerebral hemisphere, retaining full awareness
118
What is a complex partial seizure?
Loss of awareness but still only affecting one cerebral hemisphere
119
What is a secondary generalised seizure?
A generalised seizure that has began in one cerebral hemisphere before spreading across the corpus callosum
120
What are automatisms?
Coordinated, involuntary simple movements associated with seizure activity
121
What features are highly specific of a medial temporal lobe complex partial seizure?
Epigastric rising feeling
Odd smells, emotions, déjà vu, automatisms
Loss of awareness
122
What process which may be related to childhood febrile seizures may predispose to medial temporal lobe epilepsy?
Hippocampal sclerosis
123
3 major types of idiopathic generalised epilepsy?
Tonic-clinic
Absence
Myoclonic
124
In what age groups do absence seizures typically present?
Childhood
125
2 visual features highly suggestive of MS eye disease?
Blurring of vision
| Desaturation
126
What sign on fundoscopy suggests optic neuritis?
Swollen discs
127
What is the relevance of oligoclonal bands in MS diagnosis?
Unmatched oligoclonal bands in CSF vs. serum is suggestive
128
What cells are responsible for myelin production in the CNS?
Oligodendrocytes
129
Why does spasticity happen in UMNLs?
Loss of inhibitory fibre activity from above
130
What is autonomic dysreflexia?
Spinal cord injury T6 or above eventually causing pale blotchy cold below lesion and hypertension, headache, bradycardia, flushing above
131
Common causes of autonomic dysreflexia?
Noxious stimulus - full bladder or bowel
