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Year 3 - Neurology (DP) > Focal Deficits > Flashcards

Focal Deficits Flashcards

1
Q

What is the F:M ratio of MS?

A

3:1

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2
Q

What are the four types of clinical course for MS?

A

Relapsing remitting
Progressive relapsing
Primary progressive
Secondary progressive

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3
Q

What is the pattern of pyramidal dysfunction in MS?

A 
Increased tone
Spasticity
Weakness:
- Extensors of upper limbs
- Flexors of lower limbs
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4
Q

How does optic neuritis present?

A

Painful visual loss over 1-2 weeks

RAPD

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5
Q

How does cerebellar dysfunction present in MS?

A 
Ataxia
Intention tremor
Nystagmus
Past-pointing
Pendular reflexes
Dysdiadokinesis
Dysarthria
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6
Q

Why is there diplopia in MS?

A

CN vi palsy

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7
Q

What causes internuclear ophthalmoplegia in MS?

A

Injury/Dysfunction of medial longitudinal fasciculus

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8
Q

What are the signs/symptoms of MS?

A 
Distortion of binocular vision
Failure of adduction:
- Diplopia
Nystagmus in adducting eye
Lag
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9
Q

How can fatigue in MS be treated?

A

Amantadine
Modafinil (if sleepy)
Hyperbaric oxygen

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10
Q

What causes lower urinary tract dysfunction in MS?

A

Increased tone at bladder neck
Detrusor hyperactivity
Detrusor syphincteric dysenergia

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11
Q

What criteria is used to diagnose MS?

A

McDonald criteria

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12
Q

What CSF finding is highly indicative of MS?

A

Oligoclonal bands (Ig) present in CSF but not serum

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13
Q

How is a mild exacerbation of MS treated?

A

Symptomatic treatment

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14
Q

How is a moderate exacerbation of MS treated?

A

Oral steroids

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15
Q

How is a severe exacerbation of MS treated?

A

Admission

IV steroids

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16
Q

How is pyramidal dysfunction treated conservatively in MS?

A

Physiotherapy

OT

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17
Q

How can spasticity be treated in MS?

A

Oral medication (Baclofen, Tizanidine)
Botox
Intrathecal baclofen/phenol

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18
Q

How can sensory symptoms be treated in MS?

A 
Gabapentin
Amitriptyline
TENS
Acupuncture
Lidocaine infusion
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19
Q

How can lower urinary tract dysfunction be treated in MS?

A

Bladder drills
Anticholinergics (Oxybutynin)
Desmopressin
Catheterisation

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20
Q

What are the first line therapies for MS?

A 
Interferon beta (Avonex, Rebif, Betaseron, Extavia)
Glitiramer Acetate (Copaxone)
Tecfedira
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21
Q

What are the second line therapies for MS?

A

Monoclonal antibodies:
- Tysabri (Natalizumab)
- Lemtrade
Fingolimad

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22
Q

What is the third line therapy for MS?

A

Mitoxantrone

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23
Q

What drug is first line in relapsing remitting MS?

A

Tecfedira

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24
Q

What type of drug is Fingolimad?

A

Sphingosine 1-phosphate modulator

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25
When are Tysabri and Lemtrada useful?
Rapidly evolving severe relapsing remitting MS | Despite use of an Interferon beta
26
How does Tysabri work?
1. Leucocyte migration 2. Leucocyte priming and activation in tissues 3. Modulation of leucocyte apoptosis
27
What is Tysabri associated with? Why?
Progressive Multifocal Leucoencephalopathy: | - Due to JC virus
28
When is Mitoxantrone used?
Relapsing progressive MS
29
How is Mitoxantrone delivered?
12 infusions over 2 years
30
What organ is Mitoxantrone toxic to?
Heart
31
What do the following define: - A brief episode of neuro dysfunction - Due to focal brain/retinal ischaemia - Clinical symptoms lasting <1 hour - Without evidence of acute infarction
TIA
32
What is the Rosier Score?
``` Has there been loss of consciousness/syncope? - Yes = -1 point - No = 0 points Has there been seizure activity? - Yes = -1 point - No = 0 points Is there a new acute onset (1 point for each): - Asymmetrical facial weakness - Asymmetrical arm weakness - Asymmetrical leg weakness - Speech disturbance - Visual field defect ```
33
What does a Rosier score of >0 mean?
Stroke likely
34
How is the size of stroke classified?
Oxford Classification
35
Apart from the left side of the body, what else does the right side of the brain control?
Creativity Music Spatial orientation Artistic awareness
36
Apart from the right side of the body, what else does the left side of the brain control?
Spoken language Reasoning Number skills Written language
37
What do non-dominant hemisphere (usually right sided) cortical events tend to affect?
Spatial awareness resulting in neglect
38
What is Type 1 small vessel disease?
Arteriosclerotic: - Fibrinoid necrosis - Lipohyalinosis - Microatheroma and microaneurysms
39
What is Type 2 small vessel disease?
Sporadic and Hereditary cerebrl amyloid angiopathy
40
What is Type 3 small vessel disease?
Genetic small vessel disease: | - eg. Cerebral Autosomal-Dominant Arteriopathy with Cerebral Infarcts and Leucoencephalopathy (CADASIL)
41
What is Type 4 small vessel disease?
Inflammatory and immunologically related: - EGPA - GPA
42
What is Type 5 small vessel disease?
Venous collagenosis
43
What is Type 6 small vessel disease?
Other: | - eg. Post-radiation angiopathy
44
How much more likely is a patient with AF to have a stroke?
5x
45
What sort of primary intracerebral haemorrhage does hypertension often lead to?
Deep
46
What sort of primary intracerebral haemorrhage does amyloid angiopathy often lead to?
Lobar
47
What typically causes a secondary intracerebral haemorrhage?
AVM Aneurysm Tumour
48
What is the drug of choice in Tayside for thrombolysis in stroke? What does do we give?
Alteplase | 0.9mg/kg IV (max 90mg)
49
When can medical thrombolysis be used in CVA?
When they present within 4.5 hours of symptoms onest AND in who an intracerebral haemorrhage has been excluded
50
What are some indications for imaging immediately at presentation in a stroke?
Indications for thrombolysis/anticoagulation On anticoagulation therapy Known bleeding tendency Reduced consciousness (GCS <13) Unexplained progressive/fluctuating symptoms Papilloedema/Fever/Neck stiffness Severe headache at onset
51
After what time period is a CT not sensitive for blood?
~1 week
52
What is the initial antiplatelet therapy following a stroke? How long does this last?
``` 300mg aspirin (+/- 75mg clopidogrel) 2 weeks ```
53
What anticoagulant therapy is preferred to prevent a stroke?
Rivaroxaban OR Dabigatran
54
What BP do we aim for to prevent strokes?
<140/90
55
How do we manage cholesterol in stroke prevention?
80mg Atorvastatin if rasied OR 20mg if not raised
56
What other condition is important to control to prevent strokes?
Diabetes
57
What lifestyle factor results in a huge contribution to chance of stroke?
Smoking
58
What are the features of a rapid assessment in the TIA clinic?
History Carotid imaging ECG Blood tests
59
If a TIA is diagnosed, what treatment can be commenced?
Aspirin 300mg | Carotid endarterectomy
60
What is the ABCD2 of stroke risk following a TIA?
``` Age >60 years = 1 point Blood pressure >= 140/90 = 1 point Clinical features - Unilateral weakness = 2 points - Speech disturbance ONLY = 1 point - Other = 0 points Duration: - >=1 hours = 2 points - 10-59 minutes = 1 point - <10 minutes = 0 points Diabetes = 1 point ```
61
In terms of the ABCD2 score, what score indicates a high risk for stroke?
>=4
62
What are the general features of Motor Neurone Disease?
Muscle weakness and wasting Upper +/or lower motor signs NO sensory signs Focal onset and continuous spread
63
Where is the most common site of onset of Motor Neurone Disease?
Extremities (Upper > Lower)
64
Are UMN or LMN more commonly affected initially?
LMN
65
Is bulbar onset in Motor Neurone Disease more common in men or women?
Women
66
Primary bulbar onset in Motor Neurone Disease is seen in how many patients?
25%
67
What is the average age of onset of the bulbar variant of Motor Neurone Disease?
60-80 years
68
What are the signs and symptoms of Motor Neurone Disease?
Prominent tongue fasciculations Weak palate Dysarthria Dysphagia
69
The bulbar variant of Motor Neurone Disease involves the LMN degeneration of what cranial nervea?
ix x xii
70
What results in the pseudobulbar variant of Motor Neurone Disease?
Damage to UMN in corticobulbar tract
71
What are the signs and symptoms of pseudobulbar palsy?
Similar to bulbar palsy
72
What causes progressive muscular dystrophy?
LMN degeneration
73
What are the signs and symptoms of progressive muscular dystrophy?
Wasting, weakness and fasciculations: - Usually asymmetric - Starts in a hand/foot and spreads
74
What motor neurones is Primary Lateral Sclerosis confined to?
UMN
75
What are the signs/symptoms of Primary Lateral Sclerosis?
Slowly progressive: - Tetraparesis (usually starts in legs) - Pseudobulbar palsy
76
What are the UMN signs in ALS?
Hyperreflexia and Babinski sign Increased tone Pyramidal weakness Spasticity
77
What are the LMN signs in ALS?
Progressive focal weakness and wasting Cramps Fasciculations
78
What are the criteria of EMG findings that help diagnose motor neurone disease?
El-Escorial criteria
79
What algorithm can aid the use of EMG findings of chronic neurogenic changes in motor neurone disease?
Awaji algorithm
80
What is the most common neuropathogenetic cause of ALS and FTD?
TDP-43 (TARDBP)
81
What are the common misdiagnoses of motor neurone disease?
Carpal tunnel Stroke Neuropathy
82
What are some motor neurone disease mimics that may give false positive results on investigation?
Multifocal Motor Neuropathy Kennedy's Disease Inclusion Body Myopathy Cervical Spondylotic radiculomyelopathy
83
What are the following signs of: - Exaggerated snout reflex - Clonic jaw jerk - Emotional lability - Forced jawing
Bulbar UMN lesion
84
What are some signs/symptoms of cervical and lumbar UMN lesions?
Clonic deep tendon reflexes Preserved reflex in weak/wasted muscles Hoffman reflex Hyperreflexia
85
What reflex is lost in UMN lesions?
Superficial abdominal reflexes
86
What is the commonest intronic hexanucleotide repeat expansion in motor neurone disease?
C9ORF72
87
What is the most common extra-axial brain tumour? Where does it arise from?
Meningioma | Arises from meningeal mesenchymal cells
88
What are some other examples of extra-axial brain tumours?
Pituitary adenomas Craniopharyngiomas Choroid plexus papillomas Acoustic neuromas
89
How common are primary brain tumours in children?
The 2nd most common cancers in kids
90
How do brain tumours tend to present?
Progressive neurological deficit Usually motor weakness Seizures
91
When is a headache worse in the context of a brain tumour?
In the morning (wakes them up) Coughing Leaning forward
92
What can cause the headache in brain tumours?
``` Increased ICP Invasion/Compression of: - Dura - Blood vessels - Periosteum ```
93
What can headaches in brain tumours be secondary to?
Diplopia (CNs iii, iv, vi ; INO) | Difficulty focusing
94
What percentage of neuroepithial tissue tumours are caused by astrocytes? What percentage of these are high grade?
60% | 66.6%
95
What are the WHO Grade i Astrocyric tumours?
Pilocytic Pleomorphic Xanthastrocytoma Subependymal giant cell
96
What is a WHO Grade ii Astrocyric tumour?
Low grade astrocytoma
97
What is a WHO Grade iii Astrocyric tumour?
Anaplastic astrocytoma
98
What is a WHO Grade iv Astrocyric tumour?
Glioblastoma multiforme
99
What is the WHO Astrocytic tumour grading based on?
Pathological findings: - Endothelial proliferation - Cellular pleomorphism - Mitoses - Necrosis (Glioblastoma multiforme)
100
What are the only truly benign astrocytic tumours?
Grade i astrocytomas
101
What populations are Grade i astrocytomas most common in?
Children and young adults
102
Where do pilocytic astrocytomas tend to affect?
Optic nerve Hypothalamic gliomas Cerebellum Brainstem
103
How is a pilocytic astrocytoma treated?
Surgery (curative)
104
What are the three types of Grade ii "low grade" astrocytomas?
Fibrillary Gemistocytic Protoplasmic
105
What histological features do low grade astrocytomas show?
Hypercellularity Pleomorphism Vascular proliferation Necrosis
106
Where are low-grade astrocytomas most common?
Temporal lobe Posterior frontal lobe Anterior parietal lobe
107
How do low-grade astrocytomas tend to present?
Seizures
108
What is the treatment for Grade ii astrocytomas?
Surgery +/- - Serial imaging - Radiation - Chemo
109
How do we decide whether to do adjuvant therapies in Grade ii astrocytomas?
Molecular profile (The following have better prognosis): - IDH-1 - 1p19q
110
What is brachytherapy?
Placement of radioactive isoptopes directly into tissue
111
When is brachytherapy appropriate?
Tumour <4cm Performance status >=70% No subependymal spread
112
What are glioblastomas?
Grade ii astrocytomas
113
Why is surgery carried out on Grade ii astrocytomas?
Seizure control Prevent/Treat herniation Treat CSF obstruction
114
What defines a large Grade ii astrocytoma?
Diameter >6cm | Crossing midline
115
What WHO grades are malignant astrocytomas?
iii and iv
116
What is the median survival for anaplastic astrocytomas (Grade iii)?
2 years
117
What is the most common primary astrocytoma?
Glioblastoma multiforme
118
What is the median survival for Glioblastoma multiformes?
12-14 months
119
How does a Glioblastoma multiforme spread?
White matter tracking | CSF pathways
120
What are some causes of multiple glioblastomas?
NF Tuberous sclerosis Von Hippel-Lindau PML
121
What is the Stupp protocol for treatment of malignant astrocytomas?
Surgery + Radiotherapy + Temozolomide
122
What is the PCV chemotherapy regime?
Procarbazine CCNU Vincristine
123
What does radiotherapy have a clear role in in terms of brain tumours?
Treating malignant tumours post-surgery
124
What are side effects of radiotherapy for brain tumours?
IQ drop by 10 points Skin and hair changes Fatigue
125
Where are oligodendrial tumours most common?
Frontal lobes
126
In what age range are oligodendrial tumours most common?
25-45 years | Smaller peak in kids 6-12 years
127
How do olidodendrial tumours present?
Seizures
128
How is the affinity for the cerebral cortex in oligodendrial tumours displayed?
In the transcortical penetration - Infiltration into: - Subarachnoid space - Leptomeninges
129
How do oligodendrial tumours appear macroscopically?
Solid | Greyish-pink
130
How can oligodendrial tumours be differentiated by astrocytomas?
Calcification (usually peripheral) Cysts Peritumoural haemorrhage
131
What makes up a neoplastic collision-type tumours?
Oligodendrial and astrocytic cells
132
What can neoplastic collision-type tumours arise from?
Common precursor - O2A cells
133
What is the treatment for chemosensitive oligodendrial tumours?
Procarbazine Lomustine Vincristine
134
What effect can radiotherapy have in oligodendrial tumours?
Reduces seizures
135
What is the median survival for low grade oligodendrial tumours?
10yrs
136
What is the M:F ration for meningiomas?
2:3
137
Breast cancer and meningioma?
NF ii = 22q
138
What subgroup of meningioma do the following describe: - Carpet/Sheet-like lesion - Infiltrates dura and sometimes bone
Meningioma en plaque
139
How can we reduce the recurrence of Meningioma en plaque?
Remove involved bone
140
Where are the most common meningioma locations?
Parasagittal Convexity Sphenoid Intraventricular
141
What are the symptoms of a meningioma?
Headaches Skull base -> CN neuropathies Regional anatomical disturbance
142
What percentage of meningiomas are histologically benign?
90%
143
What are the classic types meningiomas?
Meningotheliomatous Fibrous Transitional
144
What are the other three groups of meningiomas?
Angioblastic Atypical Malignant
145
What are the two types of grade ii aggressive meningiomas?
Clear cell | Choroid
146
What are the two types of grade iii aggressive meningiomas?
Rhabdoid | Papillary
147
How do meningiomas appear on CT?
Homogenous and densely enhancing Oedema Hyperostosis/Skull 'blistering'
148
How do meningiomas appear on MRI?
Dural tail | Patency of dural sinuses
149
When is angiography indicated in meningioma?
If embolisation is planned
150
How do meningiomas appear on angiography?
Hypervascular: - Arterial phase - Well into venous phase - Slow washout
151
What vessels are meningiomas commonly supplied by?
Dural arteries
152
What is the five year survival for meningiomas?
90%
153
What condition are vestibular schwannomas associated with?
NF ii
154
What are the symptoms of an acoustic neuroma?
Hearing loss Tinnitus Dysequilibrium
155
What can larger acoustic neuromas affect?
CNs v, vii and viii Brainstem Hydrocephalus
156
What is the medical management of an acoustic neuroma?
Periodic neuro exam Hearing aid Periodic MRI
157
When is gamma knife surgery useful in acoustic neuromas?
If <3cm | If surgery is high risk
158
What negative effects can occur after surgery for an acoustic neuroma?
CN vii palsy Corneal reflex Nystagmus Abnormal eye movements
159
What is grade 1 of facial nerve function?
Normal symmetric function in all areas
160
Incomplete eye closure on maximal effort and spasm suggests what grade of facial nerve function?
Grade iv
161
Incomplete eye closure, slight mouth corner movement and spasms absent suggest what grade of facial nerve function?
Grade v
162
When there is no facial movement and loss of tone, what grade of facial nerve function is that?
Grade vi
163
What is the peak incidence for germ cell tumours?
10-12 years
164
Are germ cell tumours more common in men or women?
Men
165
How do germ cell tumours appear on CT?
Iso- or hyperdense
166
How can germ cell tumours metastasize?
Via CSF
167
What are the most common CNS germ cell tumours?
Germinomas
168
What CNS germ cell tumours are radiosensitive?
Germinomas
169
How are germ cell tumours treated?
Radiotherapy if >3 years | Cis-platin (1st line if <3 years)
170
What can radiotherapy cause if the patient is younger than 3 years?
Excess hypothalamic and cognitive dysfunction
171
What tumours is alpha-fetoprotein present in?
Yolk sac tumours | Teratomas
172
What tumours is hCG present in?
Choriocarinoma | Germinoma
173
What cells produce alpha-fetoprotein?
Yolk sac endoderm | Embryonic intestinal epithelium
174
What cells produce hCG?
Syncytiotrophoblasts
175
What tumours produce placental alkaline phosphatase?
Germinoma (Choriocarcinoma) (Yolk sac tumours)
176
What cells produce placental alkaline phosphatase?
Primordial germ cells | Syncytiotrophoblasts
177
What can a patient be loaded with before surgery for a brain tumours?
Phenytoin OR | Keppra
178
What cover can be provided pre-op for brain tumours?
Dexamethasone with lansoprazole
179
How are post-surgical seizures treated?
Phenytoin OR | Lorazepam
180
Why is diazepam not used to treat post-op seizures?
Stays in fat Leaks out later: - Patient stops breathing
181
When is dexamethasone stopped upon discharge after brain tumour removal?
If benign tumour
182
What is the maintenance dose of dexamethasone upon discharge?
2mg od
183
What are headache red flags?
``` New onset in >55 year olds Known/Previous malignancy Immunosuppressed Early morning headache Exacerbation by valsava (increases ICP) ```
184
What is the one year prevalence of migraine?
10-15%
185
What is the M:F ratio for migraines?
1:2/5
186
What is the usual amount of migraines a sufferer has per month?
1
187
What percentage of migraines are associated with an aura?
20%
188
What is the IHS criteria for diagnosis of migraine without aura?
``` 1. At least 5 attacks lasting 4-72 hours each AND 2. Two of: - Moderate to severe - Unilateral - Throbbing pain - Worst on movement AND 3. One of: - Autonomic features - Photophobia - Phonophobia ```
189
What do stress triggers release in the brain that is linked to migraines?
Serotonin
190
What happens to vasculature in migraines?
Constrict and dilate
191
Why is there pain in migraines?
Chemicals (including substance P) irritate nerves and blood vessels
192
What is the neurophysiology of migraine with aura?
1. Cortical spreading depolarisation/depression 2. Trigeminal vascular system activation resulting in cranial blood vessel dilatation 3. Release of: - Substance P - Neurokinin A - CGRP
193
Where are the migraine centres?
Dorsal raphe nucleus | Locus coeruleus
194
What is the most common manifestation of an aura?
Visual
195
How long do auras tend to last in migraines?
20-60 minutes
196
When does the headache follow the aura?
<1 hour later
197
What are the visual aura patterns in an aura?
Central scotomata Central fortification Hemianopia
198
What can trigger a migraine with aura?
``` Sleep Diet Stress Hormones Physical effort ```
199
What NSAID doses act as an abortive measure for migraines?
Aspirin 900mg Naproxen 250mg Ibuprofen 400mg (All +/- anti-emetic)
200
What percentage of patients who take an NSAID to relieve a migraine have some reduction in pain at 2 hours?
60%
201
What percentage of migraine patients have complete relief from NSAIDs?
25%
202
How do triptans work?
5-HT agonists: - 5-HT1B - 5-HT1-
203
How can triptans be administered?
PO Sublingual S/C
204
When are triptans administered?
At start of headache
205
What triptans are available are what is their relative efficacy?
Rizatriptan = Eletriptan > Sumatriptan
206
What triptans can provide sustained relief?
Frovatriptan
207
When should migraine prophylaxis be considered?
>3 attacks per month OR | Very severe
208
How long must a prophylactic drug for migraine be trialled?
Minimum of 4 months
209
What beta blocker can be used as migraine prophylaxis? What dose is given?
Propanolol | 80-240mg daily
210
When is a beta-blocker contraindicated in migraine prophylaxis?
Asthma Peripheral vascular disease Heart failure
211
What carbonic anhydrase inhibitor (NA/GABA) can be used in migraine prophylaxis? What dose is given?
Topiramate | 25-100mg
212
What are the adverse effects of carbonic anhydrase inhibitors?
Weight loss Paraesthesia Impaired concentration Enzyme inducer
213
What dose of amitriptyline can be used in migraines?
25mg
214
What are the side effects of amitriptyline?
Dry mouth Postural hypotension Sedation
215
What other drugs can be used for migraine prophylaxis?
Gabapentin Pizotifen Sodium valproate
216
When would imaging be considered in migraines?
Late onset >55 years Known malignancy Acephalgic migraine (ie no headache)
217
What are trigeminal autonomic cephalagias characterised by?
Unilateral trigeminal distribution pain
218
What are trigeminal autonomic cephalagias associated with?
Ipsilateral cranial autonomic features: - Ptosis - Miosis - Nasal stuffiness - Nausea and vomiting - Tearing - Eye lid oedema
219
If autonomic features are present in a trigeminal autonomic cephalagia, what investigations are indicated?
MRI brain | MR angiography
220
What are the common populations for cluster headaches?
30-40 year olds | Men (M:F is 5:1)
221
When are cluster headaches common?
``` Striking circadian (around sleep) Seasonal variation ```
222
How long do cluster headaches last?
45-90 mins average | Range of 20 mins to 3 hours
223
How frequent are cluster headaches?
1-8/day
224
How long can bouts of cluster headaches last?
A few weeks to months
225
How is a cluster headache treated?
High flow oxygen for 20 mins S/C sumatriptan 6mg Steroids (reduce course over 2 weeks)
226
What drug can be used for cluster headache prophylaxis?
Verapamil
227
In what populations is paroxysmal hemicrania common in?
50-60 year olds | Female > Male (M:F is 1:2)
228
How long does a paroxysmal hemicrania last?
10-30 minutes | Range of 2 mins to 45 hours
229
How frequent do paroxysmal hemicrania attacks last?
1-40/day
230
How is paroxysmal hemicrania treated?
Indomethicin (absolute response)
231
How can cluster headaches and paroxysmal hemicrania be distinguished?
Cluster headaches last longer and are less frequent
232
What is SUNCT?
Short-lived Unilateral Neuralgiform headache with Conjunctival injection and Tearing
233
How is SUNCT treated?
Lamotrigine | Gabapentin
234
In what populations is trigeminal neuralgia common?
>60 year olds | Women > Men
235
How is trigeminal neuralgia triggered?
Touch
236
What divisions of the trigeminal nerve are most commonly affected in trigeminal neuralgia?
V2 and V3
237
What sort of pain is present in trigeminal neuralgia?
Severe, unilateral, stabbing
238
How long does an attack of trigeminal neuralgia last?
1-90 seconds
239
How frequent are trigeminal neuralgia attacks?
10-100/day
240
How long can bouts of trigeminal neuralgia last before remission?
Few weeks to months
241
What sort of pain is felt in cluster headaches and paryoxysmal hemicrania?
Severe, unilateral boring
242
What sort of pain is felt in SUNCT?
Severe, unilateral, stabbing
243
What is the M:F ratio in SUNCT?
2:1
244
How long do attacks of SUNCT last?
15-120 seconds
245
How frequent are attacks of SUNCT?
1/day - 30/hour
246
What can trigger a cluster headache?
Alcohol
247
What can trigger paroxysmal hemicrania?
Mechanical
248
What can trigger SUNCT?
Cutaneous triggers
249
What is the first line medical treatment for trigeminal neuralgia?
Carbamazepine
250
What other drugs can be used to treat trigeminal neuralgia?
Gabapentin Phenytoin Baclofen
251
What surgical treatments are available for trigeminal neuralgia?
Ablation | Decompression
252
What is the main curare toxin?
D-tubocurarine
253
How do curares cause NMJ dysfunction?
Occupies the same position as Ach on the receptor | Does not open ion channel
254
How can curare toxicity result in morbidity and mortality?
No muscle contraction | No respiration
255
Via what methods of administration is curare toxic?
IM | IV
256
How long does it take for curares to have effect?
1-15 minutes
257
Where are Clostridium botulinum found?
Soil
258
How can IVDUs get botulism?
Black tar heroin
259
How does botulinum toxin affect the NMJ?
1. Cleaves presynaptic proteins involved in vesicle formation 2. Blocks vesicle docking with presynaptic membrane
260
How does botulism present?
Rapid onset weakness (No sensory loss)
261
What is Lambert-Eaton Myaesthenia Syndrome?
Autoantibodies to presynaptic calcium channels (reducing vesicle release)
262
How does Lambert-Eaton Myaesthenia Syndrome present?
Progressive weakness starting in the proximal limbs (legs affected worse than the arms): - Difficulty climbing stairs and standing up from sitting
263
What is Lambert-Eaton Myaesthenia Syndrome associated with?
Small-cell lung cancer (paraneoplastic syndrome)
264
What is Myaesthenia Gravis?
Antibodies against postsynaptic Ach receptors
265
What is the pathophysiology of Myaesthenia Gravis?
Reduced number of Ach receptors and endplate flattening
266
When does Myaesthenia Gravis become symptomatic?
When Ach receptors reduced to 30% of normal
267
In what percentage of Myaesthenia Gravis patients are Ach antibodies present?
80-90%
268
75% of patients with Myaesthenia Gravis also have what?
Hyperplasia of the thymus OR | Thyoma
269
What do the antibodies in Myaesthenia Gravis do?
Block Ach binding | Trigger inflammation damaging folds of postsynaptic membrane
270
What are the two peaks of incidence in Myaesthenia Gravis?
Females in 3rd decade | Males in 6th/7th decades
271
What is the M:F ratio in Myaesthenia Gravis?
2:3
272
What are the clinical features of Myaesthenia Gravis?
Weakness gets worse throughout day Extraocular weakness Facial and bulbar weakness Limb weakness is usually proximal
273
What medical treatment can be used in Myaesthenia Gravis?
Pyridostigmine (Acetylcholinesterase inhibitor)
274
Emergency treatment of Myaesthenia Gravis?
Plasma exchange OR | IV Ig
275
What cause morbidity in Myaesthenia Gravis?
Respiratory failure | Aspiration pneumonia
276
What is each muscle fibre surrounded by?
Endomysium
277
What is each muscle fascicle surrounded by?
Perimysium
278
What are a large number of fascicles surrounded by?
Epimysium
279
What is the actin:myosin ratio in smooth muscles?
10:1
280
What can precipitate fasciculations in a healthy muscle?
Stress Caffeine Fatigue
281
When do pathological fasciculations occur?
Denervated muscle which becomes hyperexcitable
282
What is myotonia and what channel affects it?
Failure of muscle relaxation | Chloride channel
283
What are some signs/symptoms in muscle disease?
Myalgia Muscle weakness Wasting Hyporeflexia
284
What are inflammatory causes of muscle disease?
Dermatomyositis | Polymyositis
285
What are congenital causes of muscle disease?
Congenital myaesthenic syndromes | Congenital myopathies
286
What are inherited causes of muscle disease?
Muscular dystrophies Dystrophinopathies Limb girdle muscular dystrophies Myotonic dystrophy
287
Symmetrical, progressive, proximal weakness over weeks to months Raised CK which responds to steroids
Polymyositis
288
Symmetrical, progressive, proximal weakness over weeks to months Raised CK which responds to steroids 'Heliotrope' rash on face Up to 50% have underlying malignancy
Dermatomyositis
289
Slowly progressive weakness 6th decade Thumb sparing Poor steroid response
Inclusion body myositis
290
What is the mode of inheritance of myotonic dystrophy?
Autosomal dominant
291
What are the features of myotonic dystrophy?
``` Myotonia Weakness (hands/feet/face/neck at first) Cataracts Ptosis Frontal balding Cardiac defects ```
292
What causes myotonic dystrophy?
Trinucleotide repeat disorder with anticipation
293
What causes BMD?
Mutations in dystrophin gene
294
What causes DMD?
Mutations in dystrophin gene
295
What is the mode if inheritance of DMD and BMD?
X-linked recessive
296
What is a viral cause of myopathy?
Coxsackie
297
What cause of infectious myopathy can arise from uncooked pork?
Cistercercosis
298
What other infectious causes are there of myopathy?
Trypanosomiasis | Borrelia
299
What drugs can cause a nectrosing myopathy?
Statins (HMG Co-A reductase inhibitors) Fibrates Nicotinic acid
300
What steroids cause corticosteroid myopathies?
Fluorinated: - Dexamethasone - Triamcinolone
301
What drugs can cause lysosomal storage myopathies?
Hydroxychloroquine | Amiodarone
302
What drugs can cause antimicrotubular myopathies?
Colchicine | Vincristine
303
What drugs can cause hypokalaemic myopathy?
Diuretics | Oral contraceptives
304
What drugs can cause inflammatory myopathies?
D-penicillamine | Interferon-alpha
305
What is rhabdomyolysis?
Damage to skeletal muscle causing leakage of toxic intracellular contents into plasma
306
What can cause rhabdomyolysis?
Crush injuries Toxins Post-convulsions
307
What is the triad of symptoms in rhabdomyolysis?
Myalgia Muscle weakness Myoglobinuria
308
What are complications of rhabdomyolysis?
Acute renal failure | DIC
309
Why is 99.9% of sound reflected?
High impedence of cochlear fluid
310
How many dB of sound is lost from air?
30 dB
311
How does the middle ear overcome sound loss from the air?
Increase the sound pressure (+34dB)
312
What are the three mechanisms for impedence matching?
1. Area ratio of eardrum to stapes is 20:1 (+26dB) 2. Lever action of ossicles is 1.3:1 (+2dB) 3. Buckling of eardrum gives 2x pressure increase (+6dB)
313
What separates the scala vestibuli from the scala media?
Reissner's membrane
314
What separates the scala media from the scala tympani?
Basilar membrane
315
Where do the scala tympani and scala vestibuli connect?
Helicotrema
316
What fluid is in the scala tympani and scala vestibuli?
Perilymph
317
What does the scala tympani meet?
Oval window
318
What does the scala vestibuli meet?
Round window
319
What does pressure at the oval window cause at the round window?
Complimentary motion
320
What is the structure of the basilar membrane?
Thick and taut near base | Thin and floppy near apex
321
Where are hair cells located?
Between: - Basilar membrane - Reticular lamina
322
Where do the tips of hair cells lie?
In the tectorial membrane
323
Where are the cell bodies of hair cells?
In the spiral ganglion
324
What happens when hair cells are forced towards the kinocilium?
TRPA1 channels open Potassium enters Depolarisation
325
What is the function of inner hair cells?
Main source of afferent CN viii signals
326
How many afferent fibres are there per hair cell?
~10
327
How many outer hair cells does one inner hair cell supply with its afferent signals?
Many
328
What is the function of outer hair cells?
Primarily receive efferent inputs Control stiffness Amplify membrane vibration
329
What motor protein is present in the membrane of outer hair cells and what can it change?
Prestin | Changes length of the cell
330
What do outer hair cells respond to?
A receptor potential | A change in length
331
What effect does furosemide have on outer hair cells?
Inactivates the membrane motor
332
What happens when there is a length change of an outer hair cell?
1. Increase movement of basilar membrane 2. Increased bending of inner hair cells 3. Transduction
333
What is the concentration gradient of potassium in the endolymph?
Inwards to the cell (4-5mV EqK)
334
What is the endolymph potential?
80mV
335
What is the Volley principle?
All neurones innervating a single hair cell code frequency together
336
What is the pathway from the cochlear to the auditory cortex?
Cochlea Cochlear nucleus (medulla) Superior olive (pons) Inferior colliculus (caudal midbrain) Medial geniculate body (rostral midbrain) Auditory cortex (sup. temporal gyrus in temporal lobe)
337
What are the three cochlear nuclei?
Dorsal cochlear nucleus Posteroventral cochlear nucleus ANteroventral cochlear nucleus
338
What do the three cochlear nuclei span?
Pontomedullary junction
339
What signal arrives at the left superior olive first?
The signal from the ipsilateral ear
340
What part of the vestibular system is responsible for sensing head angular acceleration?
Semicircular canals
341
When is the sensation of head angular acceleration useful?
During head rotation
342
What parts of the vestibular system are responsible for sensing head linear acceleration?
Saccule | Utricle
343
When is the sensation of head linear acceleration useful?
Translational motion | Gravity (extension head tilt)
344
What are the semicircular canals filled with
Endolymph
345
What part of the semicircular canals contains clusters of hair cells?
Crista
346
What part of the semicircular canals is a bulge containing cristae?
Ampulla
347
Where do the cilia in the semicircular canals project?
Into the gelatinous cupula
348
Where do the hair cells lie in the otolith organs?
Macula
349
What divides the hair cells in the otolith organs into two populations with opposing polarities?
Striola
350
What is the function of the vestibulo-ocular reflex?
Keeps eyes still as the head moves in space
351
What is the function of the vestibulo-colic reflex?
Keeps the head still in space | Keeps head level when walking
352
What is the function of the vestibular-spinal reflex?
Adjusts posture for rapid changes in posture
353
What antibiotics can cause vestibular dysfunction?
Aminoglycoside: - Gentamicin - Streptomicin
354
How does alcohol affect the vestibular system?
``` Enters blood and capula Floats in endolymph Capula bends a little more Hair cells bend (as if rotating) Sensation of spinning ```
355
What is the resting potential of photoreceptors?
-20mV
356
What happens to the membrane potential upon light exposure?
Hyperpolarisation: - PNa is reduced so Na Channels close - PK > PNa - Vm approaches EK so hyperpolarisation
357
What makes up rhodopsin?
Retinal (vit. A) and opsin (GPCR)
358
What happens to rhodopsin when exposed to light?
Changed into: - All-trans-retinal AND - Opsin
359
What enzyme turns all-trans-retinal to 11-cis-retinal?
Isomerase
360
What is the other name for Vitamin A?
All-trans-retinol
361
What does all-trans-retinal activate? What does it do?
Transducin: - Decreases cGMP - Closure of cGMP-gated sodium channels - Hyperpolariation (due to decreased sodium entry)
362
What neurotransmitter is more abundant in the dark?
Glutamate
363
If the space between photoreceptors is decreased, what effect does this have on visual acuity?
Increases acuity
364
What do ON-centre bipolar/ganglion cells detect?
Increases in luminance
365
What do OFF-centre bipolar/ganglion cells detect?
Decreases in luminance
366
What layer do magnocellular LGN neurones project to?
Layer IVc-alpha
367
What layer do parvocellular LGN neurones project to?
Layer IVc-beta
368
What layer do koniocellular LGN neurones project to?
Synapse in layers II and III (bypass IV): | - Blobs visible by cytochrome oxidase staining
369
Where are the most binocular neurones found in striate cortex?
Layer iii

Year 3 - Neurology (DP) (5 decks)

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