Neuro Flashcards
(412 cards)
1
Q
What is Charcot-Marie-Tooth disease?
A
an autosomal dominant inherited disease that affects the peripheral motor + sensory nerves
There are various types with different genetic mutations + different pathophysiology
they cause dysfunction in the myelin or the axons
2
Q
Charcot-Marie-Tooth disease
when do symptoms start to appear
A
before 10 years but can be delayed until 40 or later
3
Q
Charcot-Marie-Tooth disease
Signs (7)
A
- Pes cavus (high foot arches)
- distal muscle wasting causing ‘inverted champagne bottle legs’
- weakness in the lower legs, esp loss of ankle dorsiflexion
- weakness in the hands
- reduced tendon reflexes
- reduced muscle tone
- peripheral sensory loss
4
Q
What are the causes of peripheral neuropathy?
A
ABCDE Alcohol B12 deficiency Cancer + CKD Diabetes + Drugs (isoniazid, amiodarone, cisplatin Every vasculitis
5
Q
Charcot-Marie-Tooth disease
Mnx
A
Purely supportive:
- neurologists + geneticists
- physios
- occupational therapists
- podiatrists
- orthopaedic surgeons to correct disabling joint deformities
6
Q
Tuberous Sclerosis
Cause
A
mutations in either
- TSC1 gene on Ch9 which codes for hamartin
- TSC2 gene on Ch16 which codes for tuberin
Hamartin + tuberin interact with each other to control the size + growth of cells.
Abnormalities in one of these proteins leads to abnormal cell size + growth
7
Q
Tuberous Sclerosis
characteristic feature
A
hamartomas: benign neoplastic growths of the tissue that they origin from
8
Q
Tuberous Sclerosis
where do hamartomas commonly affect?
A
- skin
- brain
- lungs
- heart
- kidneys
- eyes
9
Q
Tuberous Sclerosis
skin signs (6)
A
- ash leaf spots
- Shagreen patches
- Angiofibromas
- Subungual fibromata
- Cafe-au-lait spots
- Poliosis
10
Q
Tuberous Sclerosis
ash leaf spots?
A
depigmented areas of skin shaped like an ash leaf
11
Q
Tuberous Sclerosis
Shagreen patches
A
thickened, dimpled, pigmented patches of skin
12
Q
Tuberous Sclerosis
Angiofibromas
A
small skin coloured or pigmented papules that occur over the nose + cheeks
13
Q
Tuberous Sclerosis
Subungual fibromata
A
fibromas growing from the nail bed
usually circular painless lumps that grow slowly + displace the nail
14
Q
Tuberous Sclerosis
Poliosis
A
isolated patch of white hair on the head, eyebrows, eyelashes or beard
15
Q
Tuberous Sclerosis
neuro features (2)
A
- epilepsy
- learning disability + development delay
16
Q
Tuberous Sclerosis
other features (5)
A
- rhabdomyomas in the heart
- gliomas (tumours of the brain + spinal cord)
- polycystic kidneys
- lymphangioleimyomatosis: abnormal growth in smooth muscle cells, often affecting the lungs)
- retinal hamartomas
17
Q
Tuberous Sclerosis
typical presentation
A
a child presenting with epilepsy found to have skin features of tuberous sclerosis
18
Q
Tuberous Sclerosis
mnx
A
supportive
monitor + treat complications such as epilepsy
no trx for the underlying gene defect
19
Q
Facial Nerve Palsy
what is it
A
isolated dysfunction of the facial nerve
typically presents with a unilateral facial weakness
20
Q
Facial Nerve Palsy
where does the facial nerve exit the brainstem
A
at the cerebellopontine angle
21
Q
Facial Nerve Palsy
where does the facial nerve pass through to get to the face
A
temporal bone and parotid gland
22
Q
Facial Nerve Palsy
what are the 5 branches of the facial nerve
A
To Zanzibar By Motor Car
Temporal Zygomatic Buccal Marginal mandibular Cervical
23
Q
Facial Nerve Palsy
name 3 functions of the facial nerve
A
- motor
- sensory
- parasympathetic
24
Q
Facial Nerve Palsy
motor function of the facial nerve
A
supplies:
- the muscles of facial expression
- stapedius in the inner ear
- the posterior digastric, stylohyoid + platysma muscles in the neck
25
Facial Nerve Palsy
sensory function of the facial nerve
carries taste from the anterior 2/3 of the tongue
26
Facial Nerve Palsy
parasympathetic function of the facial nerve
provides the parasympathetic supply to the submandibular + sublingual glands and the lacrimal gland (stimulating tear production)
27
Facial Nerve Palsy
patient w/ a new onset upper motor neurone facial nerve palsy mnx
refer urgently w/ suspected stroke
28
Facial Nerve Palsy
patient w/ a new onset lower motor neurone facial nerve palsy immediate mnx
reassured and managed in the community
29
Facial Nerve Palsy
each side of the forehead has UMN innervation by ____ of the brain
both sides
30
Facial Nerve Palsy
each side of the forehead has LMN innervations from ___ of the brain
one side
31
Facial Nerve Palsy
UMN lesion forehead
forehead will be spared
pt can move their fod on the affected side
32
Facial Nerve Palsy
LMN lesion forehead
fod will not be spared
pt cannot move their forehead on the affected side
33
Facial Nerve Palsy
unilateral UMN lesion causes (2)
- strokes
| - tumours
34
Facial Nerve Palsy
bilateral UMN lesion causes (2)
rare:
- Pseudobulbar palsies
- MND
35
Facial Nerve Palsy
Bell's palsy cause
idiopathic
unilateral LMN facial nerve palsy
36
Facial Nerve Palsy
Bell's palsy mnx presenting within 72 hours of developing sx
Prednisolone either:
- 50mg for 10d
- 60mg for 5d followed by a 5d reducing regime of 10mg/day
lubricating eye drops and tape
37
Facial Nerve Palsy
why give lubricating eye drops and tape in Bell's palsy
to prevent the eye drying out + being damaged
38
Facial Nerve Palsy
Bell's palsy pt develops pain in the eye
need an ophthalmology review for exposure keratopathy
39
Facial Nerve Palsy
what is Ramsay-Hunt Syndrome caused by
varicella zoster virus
40
Facial Nerve Palsy
Ramsay-Hunt Syndrome presentation
unilateral LMN facial nerve palsy
painful + tender vesicular rash in the ear canal, pinna + around the ear on the affected side. Can extend to the anterior 2/3 of the tongue + hard palate
41
Facial Nerve Palsy
trx of Ramsay-Hunt Syndrome
initiate within 72hrs:
- Prednisolone
- Aciclovir
lubricating eye drops
42
Facial Nerve Palsy
infection causes of LMN facial nerve palsy (4)
- otitis media
- malignant otitis externa
- HIV
- Lyme's disease
43
Facial Nerve Palsy
Systemic disease causes of LMN facial nerve palsy (5)
- diabetes
- sarcoidosis
- leukaemia
- multiple sclerosis
- guillain-barré syndrome
44
Facial Nerve Palsy
tumour causes of LMN facial nerve palsy
- acoustic neuroma
- parotid tumours
- cholesteatomas
45
Facial Nerve Palsy
trauma causes of LMN facial nerve palsy (3)
- direct nerve trauma
- damage during surgery
- base of skull fractures
46
Huntington's Chorea
inheritance pattern
autosomal dominant
causes a progressive deterioration in the nervous system
47
Huntington's Chorea
when do sx usually begin
30-50yrs
48
Huntington's Chorea
where is the genetic mutation
in the HTT gene on Ch4
a 'trinucleotide repeat disorder'
49
Huntington's Chorea
what is genetic 'anticipation'
a features of trinucleotide repeat disorders
where successive generation have more repeats in the gene resulting in:
- earlier age of onset
- increased severity of disease
50
Huntington's Chorea
presentation
insidious, progressive worsening of symptoms
beings with: cognitive, psychiatric + mood problems
then movement disorders
51
Huntington's Chorea
what are the movement disorder sx
- chorea
- eye movement disorders
- dysarthria: speech difficulties
- dysphagia (swallowing difficulties)
52
Huntington's Chorea
what is chorea
involuntary, abnormal movements
53
Huntington's Chorea
dx
made in a specialist genetic centre using a genetic test for the faulty gene
involves pre-test and post-test counselling
54
Huntington's Chorea
mnx
supporting the person + family:
- MDT
- effectively breaking bad news
- speech + language therapy
- genetic counselling
- advanced directives
- end of life care planning
discontinue unnecessary meds to minimise adverse effects
55
Huntington's Chorea
what medications can suppress the disordered movement
- antipsychotics (olanzapine)
- benzos (diazepam)
- dopamine-depleting agents (tetrabenazine)
56
Huntington's Chorea
prognosis
life expectancy is around 15-20 years after the onset of symptoms
57
Huntington's Chorea
what is death due to
respiratory disease eg. pneumonia as patient becomes more susceptible and less able to fight off illnesses
suicide
58
Neurofibromatosis
what is it
a genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system
benign but causes neuro and structural problems
59
Neurofibromatosis
what are the types of Neurofibromatosis and which is more common
Neurofibromatosis type 1 is more common than Neurofibromatosis type 2
60
Neurofibromatosis
where is the NF1 Gene found
on Ch17
61
Neurofibromatosis
what does the NF1 gene code for
a protein called neurofibromin
which is a tumour suppressor protein
62
Neurofibromatosis
NF1 gene inheritance pattern
autosomal dominant
63
Neurofibromatosis
NF1 criteria
CRABBING
at least 2/7 features indicate dx:
Café-au-lait spots: ≥6 measuring ≥5mm in children or ≥15mm in adults
Relative with NF1
Axillary or inguinal freckles
BB - Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia
Iris hamartomas (Lisch nodules): ≥2 yellow brown spots on iris
Neurofibromas: ≥ or 1 plexiform neurofibroma
Glioma of the optic nerve
64
Neurofibromatosis
inx
none required to make a definitive dx. Based on clinical criteria
genetic testing if in doubt
X-rays to investigate bone pain and lesions
CT + MRI: lesions in brain, spinal cord etc
65
Neurofibromatosis
mnx
control symptoms, monitor disease and treat complications
66
Neurofibromatosis
3 main complications
1. renal artery stenosis causing HTN
2. malignant peripheral nerve sheath tumours
3. GI stromal tumour
67
Neurofibromatosis
other complications
- migraines
- epilepsy
- ADHD
- scoliosis of the spine
- vision loss secondary to optic nerve gliomas
- brain tumours
- spinal cord tumours w/ assc neurology
- increased risk of cancer
- leukaemia
68
Neurofibromatosis
where is the NF2 gene found
Ch22
69
Neurofibromatosis
what does the NF2 gene code for
a protein called merlin, a tumour suppressor protein important in Schwann cells
70
Neurofibromatosis
what can mutations in the NF2 gene lead to the development of?
Schwannomas (benign nerve sheath tumours of the Schwann cells)
71
Neurofibromatosis
NF2 inheritance pattern
autosomal dominant
72
Neurofibromatosis
what are NF2 most associated with?
acoustic neuromas: tumours of the auditory nerve innervating the inner ear
73
Neurofibromatosis
NF2: sx of an acoustic neuroma
- hearing loss
- tinnitus
- balance problems
74
Neurofibromatosis
what may bilateral acoustic neuromas present as?
NF2
75
Neurofibromatosis
mnx of NF2
surgery to resect tumours although risk of permanent nerve damage
76
Stroke
new definition of TIA
transient neurological dysfunction secondary to ischaemia without infarction
old definition: symptoms of a stroke that resolve within 24hrs
77
Stroke
What is a crescendo TIA
2 or more TIAs within a week
this carries a high risk of developing in to stroke
78
Stroke
what clinical scoring tool is used
ROSIER: recognition of stroke in emergency room
stroke is likely if the pt scores anything above 0
79
Stroke
mnx
- admit to specialist stroke centre
- exclude hypoglycaemia
- immediate CT to exclude haemorrhage
- aspirin 300mg and continue for 2w
- thrombolysis with alteplase given within 4.5hrs
- if not, thrombectomy within 24hrs
80
Stroke
what is alteplase
a tissue plasminogen activator that rapidly breaks down clots and can reverse the effects of stroke
81
Stroke
why should BP not be lowered during a stroke
this risks reducing the perfusion to the brain
82
mnx of TIA
start 300mg aspirin daily
secondary prevention measures for CVD
referred and seen within 24hrs by a stroke specialist
83
Stroke
gold standard imaging technique
diffusion-weighted MRI
CT is an alternative
84
Stroke
which imaging would you use to assess for carotid stenosis
carotid US
85
Stroke
what should be considered if there is carotid stenosis
endarectomy (remove plaques)
or carotid stenting (widen lumen)
86
secondary prevention of stroke
- clopidogrel 75mg OD
- artovastatin 80mg (not started immediately)
- carotid endarterecomy or stenting in patients with carotid artery disease
- treat modifiable RFs
87
Parkinson's Disease
what is it
a condition where there is a progressive reduction of dopamine in the basal ganglia leading to disorders of movement
sx are characteristically asymmetrical
88
Parkinson's Disease
what is the classic triad
1. Resting tremor
2. Rigidity
3. Bradykinesia
89
Parkinson's Disease
what is the basal ganglia responsible for
- coordinating habitual movements:
walking
looking around
- controlling voluntary movements
- learning specific movement patterns
90
Parkinson's Disease
which part of the basal ganglia produces dopamine
substantia nigra
91
Parkinson's Disease
what is dopamine essential for
the correct functioning of the basal ganglia
92
Parkinson's Disease
describe the typical pt
older man aged around 70
- stooped posture
- facial masking
- forward tilt
- reduced arm swing
- shuffling gait
93
Parkinson's Disease
describe the tremor
- frequency of 4-6Hz (occurs 4-6 times/s)
- pill rolling tremor
- more pronounced at rest
- improves on voluntary movement
- worsens if pt is distracted
94
Parkinson's Disease
why is it called cogwheel rigidity
tension in their arm that gives way to movement in small increments (like little jerks)
95
Parkinson's Disease
what does bradykinesia describe
their movement gets slower and smaller
96
Parkinson's Disease
how can bradykinesia be presented
- handwriting decreases in size
- shuffling gait
- difficulty initiating movement
- difficulty turning round when standing, takes lots of little steps
- hypomimia (reduced facial movements + facial expressions)
97
Parkinson's Disease
other features that may affect pts
- depression
- insomnia
- anosmia (loss of smell)
- postural instability
- cognitive impairment + memory problems
98
describe a benign essential tremor
- symmetrical
- 5-8Hz
- improves at rest
- worse with intentional movement
- no other Parkinson features
- improves with alcohol
99
Parkinson's Disease
name some Parkinson's-plus syndromes
- Multiple System Atrophy
- Dementia with lewy bodies
- Progressive Supranuclear Palsy
- Corticobasal Degeneration
100
Parkinson's Disease
what is Multiple System Atrophy
rare condition where the neurones of multiple systems in the brain degenerate
The degeneration of the basal ganglia lead to a Parkinson’s presentation.
101
Parkinson's Disease
what features other than parkinson presentation does Multiple System Atrophy have
autonomic dysfunction:
- postural hypotension
- constipation
- abnormal sweating
- sexual dysfunction
cerebellar dysfunction:
- ataxia
102
Parkinson's Disease
how is a dx made
clinically by a specialist
NICE recommend using the UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria.
103
Parkinson's Disease
what is Levodopa
synthetic dopamine
104
Parkinson's Disease
why is levodopa often combined with another drug
the other drug stops levodopa being broken down in the body before it gets the chance to enter the brain
105
Parkinson's Disease
what drug is levodopa often combined with
peripheral decarboxylase inhibitors e.g. carbidopa and benserazide
106
Parkinson's Disease
what is in co-benyldopa
levodopa and benserazide
107
Parkinson's Disease
what is in co-careldopa
levodopa and carbidopa
108
Parkinson's Disease
when is levodopa used
often reserved for when other treatments are not managing to control symptoms.
109
Parkinson's Disease
what do patients develop when levodopa dose is too high
patients develop dyskinesia:
| abnormal movements associated with excessive motor activity.
110
Parkinson's Disease
levodopa SE's
dyskinesias:
- dystonia
- chorea
- athetosis
111
Parkinson's Disease
levodopa SE's: what is dystonia
excessive muscle contraction leads to abnormal postures or exaggerated movements
112
Parkinson's Disease
levodopa SE's: what is chorea
abnormal involuntary movements that can be jerking and random.
113
Parkinson's Disease
levodopa SE's: what is athetosis
involuntary twisting or writhing movements usually in the fingers, hands or feet
114
Parkinson's Disease
what are COMT inhibitors
inhibitors of catechol-o-methyltransferase
e.g. entacapone
115
Parkinson's Disease
how do COMT inhibitors work
COMT enzyme metabolises levodopa in both the body and brain.
Entacapone is taken with levodopa (and a decarboxylase inhibitor) to slow breakdown of the levodopa in the brain.
It extends the effective duration of the levodopa.
116
Parkinson's Disease
what are dopamine agonists
they mimic dopamine in the basal ganglia and stimulate the dopamine receptors
117
Parkinson's Disease
what are dopamine agonists used for
to delay the use of levodopa and are then used in combination with levodopa to reduce the dose of levodopa that is required to control symptoms
118
Parkinson's Disease
SE of dopamine agonists
pulmonary fibrosis
119
Parkinson's Disease
examples of dopamine agonists
- Bromocryptine
- Pergolide
- Cabergoline
120
Parkinson's Disease
what are Monoamine Oxidase-B inhibitors
Monoamine oxidase enzymes break down neurotransmitters: dopamine, serotonin and adrenaline
monoamine oxidase-B enzyme is more specific to dopamine
block this enzyme and therefore help increase the circulating dopamine.
121
Parkinson's Disease
when are monoamine oxidase-B inhibitors used
to delay the use of levodopa and then in combination with levodopa to reduce the required dose.
122
Parkinson's Disease
examples of monoamine oxidase-B inhibitors
Selegiline
| Rasagiline
123
Epilepsy
inx and why
EEG: can show typical patterns in different forms of epilepsy and support dx
MRI brain: diagnoses structural problems and other pathology e.g. tumour
ECG: exclude heart problems
124
Epilepsy
presentation of tonic-clonic seizures
- loss of consciousness
- tonic (muscle tensing)
- clonic (muscle jerking) episodes
- tongue biting
- incontinence
- groaning
- irregular breathing
- post-ictal period
125
Epilepsy
what is the post-ictal period
where the person is confused, drowsy and feels irritable or depressed
126
Epilepsy
mnx of tonic-clonic seizures
1st line: sodium valproate
2nd line: lamotrigine or carbamazepine
127
Epilepsy
| where do focal seizures start
in temporal lobes
128
Epilepsy
presentation of focal seizures
- hallucinations
- memory flashbacks
- deja vu
- doing strange things on autopilot
129
Epilepsy
trx for focal seizures
1st line: carbamazepine or lamotrigine
2nd line: sodium valproate or levetiracetam
130
Epilepsy
| mnx of absence seizures
1st line: sodium valproate or ethosuximide
131
Epilepsy
what are atonic seizures
aka drop attacks
brief lapses in muscle tone lasting <3 min
typically begin in childhood
132
Epilepsy
what may atonic seizures be indicative of
Lennox-Gastaut syndrome
133
Epilepsy
mnx for atonic seizures
1st line: sodium valproate
2nd line: lamotrigine
134
Epilepsy
presentation of myoclonic seizures
- sudden brief muscle contractions like a sudden 'jump'
- remains awake
- typically happen in children in juvenile myoclonic epilepsy
135
Epilepsy
mnx of myoclonic seizures
1st line: sodium valproate
other options: lamotrigine, levetiracetam or topiramate
136
Epilepsy
what are infantile spasms
aka West syndrome
clusters of full body spasms
137
Epilepsy
when do infantile spasms begin
in infancy at around 6m
138
Epilepsy
1st line treatments for infantile spasms
prednisolone
vigabatrin
139
Epilepsy
how does sodium valproate work
by increasing the activity of GABA
which has a relaxing effect on the brain
140
Epilepsy
SEs of sodium valproate
- Teratogenic
- liver damage + hepatitis
- hair loss
- tremor
141
Epilepsy
SEs of carbamazepine
- agranulocytosis
- aplastic anaemia
- induces the P450 system so there are many drug interactions
142
Epilepsy
SEs of phenytoin
- folate and vit D deficiency
- megaloblastic anaemia (folate deficiency)
- osteomalacia (vit D deficiency)
143
Epilepsy
SEs of Ethosuximide
- night tremors
| - rashes
144
Epilepsy
Lamotrigine SEs
- Stevens-Johnson sydrome or DRESS syndrome
| - Leukopenia
145
Status Epilepticus
definition
seizures lasting >5m
or >3 seizures in 1 hr
146
Status Epilepticus
mnx in the hospital
ABCDE
- secure airway
- high conc o2
- assess cardiac + resp function
- check blood glucose levels
- gain IV access
IV lorazepam 4mg, repeated after 10min if the seizure continues
if persists: IV phenobarbital or phenytoin
147
Status Epilepticus
medical options in the community
buccal midazolam
rectal diazepam
148
Brain Tumours
presentation
- focal neurological sx
| - raised ICP
149
Brain Tumours
pt has had an unusual change in personality and behaviour; Where is the tumour
in the frontal lobe
150
Brain Tumours
what is a key finding on fundoscopy which indicates raised ICP
papilloedema
151
Brain Tumours
causes of raised ICP
- brain tumours
- intracranial haemorrhage
- idiopathic intracranial hypertension
- abscesses or infection
152
Brain Tumours
what are the concerning features of a headache that should prompt further examination and inx (5)
- constant
- nocturnal
- worse on waking
- worse on coughing, straining or bending forward
- vomiting
153
Brain Tumours
presenting features of raised ICP
- altered mental state
- visual field defects
- seizures (particularly focal)
- unilateral ptosis
- 3rd and 6th nerve palsies
- papilloedema
154
Brain Tumours
what is papilloedema
swelling of the optic disc secondary to raised ICP
155
Brain Tumours
what will fundoscopy show in papilloedema
- blurring of the optic disc margin
- elevated optic disc (vessels curve over a raised disc)
- loss of venous pulsation
- engorged retinal veins
- haemorrhages around optic disc
- Paton's lines (creases in the retina around the optic disc
156
Brain Tumours
types
- secondary metastases
- gliomas
- meningiomas
- pituitary tumours
- acoustic neuroma aka vestibular schwannoma
157
Brain Tumours
what are the common cancers that metastasise to the brain
- lung
- breast
- renal cell carcinoma
- melanoma
158
Brain Tumours
what are gliomas
tumours of the glial cells in the brain or spinal cord
159
Brain Tumours
what are the 3 types of gliomas (from most to least malignant)
- Astrocytoma (glioblastoma multiforme in the most common)
- Oligodendroglioma
- Ependymoma
160
Brain Tumours
how are gliomas graded
1-4
1: most benign
4: most malignant
161
Brain Tumours
what are meningiomas
tumour growing from the cells of the meninges in the brain and spinal cord
162
Brain Tumours
are meningiomas benign
mostly, however takes up space --> raised ICP --> neuro sx
163
Brain Tumours
are pituitary tumour benign
tend to be
164
Brain Tumours
what visual defect can occur with pituitary tumours and why
bitemporal hemianopia
large ones can press on the optic chiasm
165
Brain Tumours
describe the visual change in bitemporal hemianopia
loss of the outer half of the visual fields in both eyes
166
Brain Tumours
what can pituitary tumours cause
hypopituitarism or release excess hormones leading to:
- acromegaly
- hyperprolactinaemia
- cushing's disease
- thyrotoxicosis
167
Brain Tumours
what is an acoustic neuroma
tumours of the Schwann cells surrounding the auditory nerve that innervates the inner ear
168
Brain Tumours
where do acoustic neuromas occur around
the cerebellopontine angle
169
Brain Tumours
classic sx of acoustic neuroma (3)
- tinnitus
- hearing loss
- balance problems
can also be associated with facial nerve palsy
170
Brain Tumours
what may a bilateral acoustic neuroma suggest
neurofibromatosis type 2
171
Brain Tumours
mnx options
- palliative care
- chemo
- radiotherapy
- surgery
172
Brain Tumours
trx of pituitary tumours
- trans-sphenoidal surgery
- radiotherapy
- bromocriptine
- somatostatin analogues
173
Brain Tumours
how does bromocriptine help in the trx of pituitary tumours
it blocks the prolactin-secreting tumour
174
Brain Tumours
how does somastostatin analogues (e.g. ocreotide) help in the trx of pituitary tumours
they block growth hormone-secreting tumours
175
Parkinson's
impulse control disorders are more common in which medication
dopamine agonists
176
Intracranial Bleeds
what are the layers of the brain from top to bottom
```
skull
dura mater
arachnoid mater
subarachnoid space
pia mater
brain
```
177
Intracranial Bleeds
RFs (6)
- head injury
- HTN
- aneurysms
- ischaemic stroke can progress to haemorrhage
- brain tumours
- anticoagulants such as warfarin
178
Intracranial Bleeds
key feature
sudden onset headache
179
Intracranial Bleeds
presentation
- sudden onset headache
- seizures
- weakness
- vomiting
- reduced consciousness
- other sudden onset neuro sx
180
Intracranial Bleeds
what does the Glasgo Coma Scale assess
level of consciousness
181
Intracranial Bleeds
GCS: eyes
```
4 = spontaneous
3 = speech
2 = pain
1 = none
```
182
Intracranial Bleeds
GCS: verbal
```
5 = orientated
4 = confused
3 = inappropriate words
2 = sounds
1 = nothing
```
183
Intracranial Bleeds
GCS: motor
```
6 = obeys commands
5 = localises pain
4 = normal flexion
3 = abnormal flexion
2 = extends
1 = none
```
184
Intracranial Bleeds
what is a subdural haemorrhage caused by
rupture of the bridging veins in the outermost meningeal layer
185
Intracranial Bleeds
where does a subdural haemorrhage occur
between the dura mater and arachnoid mater
186
Intracranial Bleeds
CT scan findings in a subdural haemorrhage
- crescent shaped
| - they can cross over the cranial sutures
187
Intracranial Bleeds
whom do subdural haemorrhages occur most frequently in
the elderly and alcoholics
188
Intracranial Bleeds
why do the elderly and alcoholics more frequently get subdural haemorrhages
they have more atrophy in their brains making vessels more likely to rupture
189
Intracranial Bleeds
what is the cause of extradural haemorrhages
rupture of the middle meningeal artery
190
Intracranial Bleeds
where do extradural haemorrahges occur
in the temporo-parietal region
between the skull and dura mater
191
Intracranial Bleeds
what fracture are extradural haemorrhages associated with
fracture of the temporal bone
192
Intracranial Bleeds
CT scan findings in extradural haemorrhages
- bi concave shape
| - limited by the cranial sutures
193
young pt with traumatic head injury that has ongoing headache. Periods of improved neuro sx and consciousness followed by rapid decline over hours. What is it
extradural haemorrhage as the haematoma gets large enough to compress the intracranial contents
194
Intracranial Bleeds
what is an intracerebral haemorrhage
bleeding into the brain tissue. presents similarly to an ischaemic stroke
195
Intracranial Bleeds
| where can intracerebal hameorrhages be
- Lobar intracerebral haemorrhage
- Deep intracerebral haemorrhage
- Intraventricular haemorrhage
- Basal ganglia haemorrhage
- Cerebellar haemorrhage
196
Intracranial Bleeds
how can intracerebral haemorrhages occur
- spontaneously
- result of bleeding into an ischaemic infarct
- tumour
- rupture of an aneurysm
197
Intracranial Bleeds
what is a subarachnoid haemorrhage
bleeding in to the subarachnoic space
198
Intracranial Bleeds
where do subarachnoids occur
where the CSF is located, between the pia mater and the arachnoid membrane
199
Intracranial Bleeds
what is the usual cause of a subarachnoid haemorrhage
rupured cerebral aneurysm
200
sudden onset occipital headache. Occurs during weight lifting or sex. Thunderclap headache. What is it
subarachnoid haemorrhage
201
Intracranial Bleeds
what is a subarachnoid associated with
cocaine and sickle cell anaemia
202
Intracranial Bleeds
principles of mnx
- Immediate CT head to establish the diagnosis
- Check FBC and clotting
- Admit to a specialist stroke unit
- Discuss with a specialist neurosurgical centre to consider surgical treatment
- Consider intubation, ventilation and ICU care if they have reduced consciousness
- Correct any clotting abnormality
- Correct severe hypertension but avoid hypotension
203
MND
what is it
an umbrella term for when motor neurones stop functioning
NO SENSORY SX
204
MND
what is the most common MND (Stephen Hawking)
Amyotrophic lateral sclerosis (ALS)
205
MND
what is the 2nd most common
Progressive bulbar palsy
206
MND
what muscles are primarily affected in progressive bulbar palsy
talking and swallowing muscles
207
MND
name 4 MNDs
- progressive muscular atrophy
- primary lateral sclerosis
- amyotrophic lateral sclerosis
- progressive bulbar palsy
208
MND
degeneration in?
- progressive degeneration of BOTH UMN + LMN
| - sensory neurones are spared
209
MND
RFs
- genetic component
- smoking
- exposure to heavy metals
- certain pesticides
210
60yr old man w/ affected relative.
insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech
what is it
MND
211
MND
where is the weakness first noticed
in the upper limbs
212
MND
may there be increased fatigue
yes when exercising
213
MND
what may pts complain of
clumsiness, dropping things or tripping over
dysarthria (slurred speech)
214
MND
signs of LMN disease
- muscle wasting
- reduced tone
- fasciculations
- reduced reflexes
215
MND
signs of UMN disease
- increased tone or spasticity
- brisk reflexes
- upgoing plantar responses
216
MND
dx
clinical presentation and excluding other conditions that can cause motor neurone sx
217
MND
is there effective trx for halting or reversing the progression of the disease
no
218
MND
medical mnx
- Riluzole
- Edaravone
- NIV
219
MND
what can Riluzole do
slow the progression of the disease and extend survival by a few months in ALS
220
MND
where is Edaravone used
in the US, not the UK
221
MND
how does NIV help
used at home to support breathing at night
improves survival and QoL
222
MND
how to support the person + family
- effectively breaking bad news
- MDT
- advanced directives: document pt's wishes
- end of life care planning
223
MND
what do pts usually die from
resp failure or pneumonia
224
what is myasthenic crisis characterised by
acute respiratory failure characterised by:
- FVC<1L
- negative inspiratory force (NIF) of 20 cm H2O or less
- need for ventilatory support
225
Myasthenia Gravis
what is it
an autoimmune condition that causes muscle weakness that get progressively worse with activity and improves with rest
226
Myasthenia Gravis
at what age are pts usually affected by it
woman <40
man >60
227
Myasthenia Gravis
what is it strongly linked with
thymomas (tumours of the thymus gland)
228
Myasthenia Gravis
pathophysiology
ACh receptor antibodies bind to post synaptic NMJ receptors
this prevents ACh from being able to stimulate the receptor and trigger muscle contraction
229
Myasthenia Gravis
why is there more muscle weakness the more muscles are used
As the receptors are used more during muscle activity, more of them become blocked up.
This leads to less effective stimulation of the muscle with increased activity.
230
Myasthenia Gravis
pathophysiology: what further worsens sx
ACh receptor antibodies also activate the complement system within the NMJ, leading to damage to cells at the postsynaptic membrane
231
Myasthenia Gravis
dx
test directly for antibodies:
- ACh receptor antibodies (85%)
- Muscle-specific kinase (MuSK) antibodies (10%)
- low-density lipoprotein receptor-related protein 4 (LRP4) antibodies (<5%)
232
Myasthenia Gravis
what do the proteins MuSK and LRP4 do
creation and organisation of the ACh receptor
233
Myasthenia Gravis
characteristic feature
weakness that gets worse with muscle use and improves with rest
sxs minimal in the morning and worst at the end of the day
234
Myasthenia Gravis
which muscles are most affected
proximal muscles and small muscles of the head and neck
235
Myasthenia Gravis
why is there diplopia
extraocular muscle weakness
236
Myasthenia Gravis
why is there ptosis
Eyelid weakness causing drooping of the eyelids
237
Myasthenia Gravis
signs and sx
- diplopia
- ptosis
- Weakness in facial movements
- Difficulty with swallowing
- Fatigue in the jaw when chewing
- Slurred speech
- Progressive weakness with repetitive movements
238
Myasthenia Gravis
examination to elicit fatiguability in the muscles
- Repeated blinking will exacerbate ptosis
- Prolonged upward gazing will exacerbate diplopia on further eye movement testing
- Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides
239
Myasthenia Gravis
what to look for and test in examination
check for a thymectomy scar
test for FVC
240
Myasthenia Gravis
what test can be helpful where there is doubt about dx
Edrophonium Test
241
Myasthenia Gravis
what is the Edrophonium Test
- IV edrophonium chloride (or neostigmine)
- edrophonium blocks cholinesterase enzymes in the NMJ
- ACh levels increase
- briefly relieves weakness. This establishes dx
242
Myasthenia Gravis
trx
- Reversible acetylcholinesterase inhibitors (usually pyridostigmine or neostigmine)
- Immunosuppression (e.g. prednisolone or azathioprine)
- Thymectomy
243
Myasthenia Gravis
trx if standard trx not effective
Rituximab
| targets B cells and reduces the production of antibodies
244
Myasthenic Crisis
what is it
often triggered by another illness such as a respiratory tract infection
life-threatening complication of myasthenia gravis
245
Myasthenic Crisis
why may pts require BiPAP (non-invasive ventilation) or full intubation and ventilation
weakness in the muscle of respiration can result in resp failure
246
Myasthenic Crisis
medical trx
immunomodulatory therapies such as IV immunoglobulins and plasma exchange
247
what is Bulbar palsy
a 'lower motor neurone' lesion affecting cranial nerves 9, 10 and 12. This causes impairments in speech and swallowing.
248
Bulbar palsy
clinical features
- absent or normal jaw jerk reflex
- absent gag reflex
- flaccid fasciculating tongue
- nasal quiet speech
- signs suggestive of the cause e.g. limb fasciculations of MND
249
Bulbar palsy
causes
- MND (progressive bulbar palsy variant)
- myasthenia gravis
- Guillain-Barré syndrome
- brainstem stroke (the lateral medullary syndrome)
- syringobulbia
250
Hallucinations (auditory/gustatory/olfactory), Epigastric rising/Emotional, Automatisms (lip smacking/grabbing/plucking), Deja vu/Dysphasia post-ictal)
Where is the likely site of the seizure onset
Temporal lobe (HEAD)
251
Head/leg movements, posturing, post-ictal weakness, Jacksonian march
Where is the likely site of the seizure onset
Frontal lobe (motor)
252
Paraesthesia
Where is the likely site of the seizure onset
Parietal lobe (sensory)
253
Floaters/flashes
Where is the likely site of the seizure onset
Occipital lobe (visual)
254
CT: Hyper-attenuation within the superior sagittal sinus
what is it
Intracranial Venous Thrombosis in the superior sagittal sinus
255
a lesion in which lobe would cause contralateral homonymous superior (upper) quadrantanopia
temporal lobe
| PITS
256
a lesion in which lobe would cause contralateral homonymous inferior (lower) quadrantanopia
parietal lobe
| PITS
257
a lesion in which lobe would cause contralateral homonymous hemianopia
occipital lobe
258
what medication is useful for managing tremor in drug-induced parkinsonism?
anti-muscarinics: procyclidine, benzotropine, trihexyphenidyl (benzhexol)
259
what is the difference between foot drop and L5 radiculopathy
L5 radiculopathy presents with foot drop and an inability to both invert and evert the foot.
in foot drop you can invert the foot
260
Guillain-Barré Syndrome
pathophysiology
molecular mimicry
B cells create antibodies against the antigens on the pathogen that causes the infection
the antibodies also match the proteins on the nerve cells
may target proteins on the myelin sheath of the motor nerve cell or the nerve axon.
261
Guillain-Barré Syndrome
what usually triggers it
infection from campylobacter jejuni, cytomegalovirus and Epstein-Barr virus.
262
Guillain-Barré Syndrome
presentation
- Symmetrical ascending weakness
- Reduced reflexes
- may be peripheral loss of sensation or neuropathic pain
- may progress to the cranial nerves and cause facial nerve weakness
263
Guillain-Barré Syndrome
when do sx start from the preceding infection
4w
264
Guillain-Barré Syndrome
what criteria can be used for dx
The Brighton criteria
265
Guillain-Barré Syndrome
what inx can support the dx
- nerve conduction studies
| - LP
266
Guillain-Barré Syndrome
what will nerve conduction studies show
reduced signal through the nerves
267
Guillain-Barré Syndrome
what will CSF show in the LP
raised protein with a normal cell count and glucose
268
Guillain-Barré Syndrome
mnx
- IV immunoglobulins
2nd line: plasma exchange
- supportive care
- VTE prophylaxis
269
Guillain-Barré Syndrome
what is the leading cause of death
PE
270
Guillain-Barré Syndrome
when may pts need intubation, ventilation and admission to ICU
severe cases with resp failiure
271
Headaches
red flags
- Fever, photophobia or neck stiffness (meningitis or encephalitis)
- New neuro sx (haemorrhage, malignancy, stroke)
- Dizziness (stroke)
- Visual disturbance (temporal arteritis, glaucoma)
- Sudden onset occipital headache (subarachnoid haemorrhage)
- Worse on coughing, straining (raised ICP)
- Postural, worse on standing, lying or bending over (raised ICP)
- Severe enough to wake the pt from sleep
- Vomiting (raised ICP or CO poisoning)
- History of trauma (intracranial haemorrhage)
- Pregnancy (pre-eclampsia)
272
Headaches
what may papilloedema indicate on fundoscopy
raised ICP due to brain tumour, intracranial HTN or an intracranial bleed
273
Headaches
mnx for sinusitis
- usually resolves within 2-3 w
- nasal irrigation with saline can be helpful
- steroid nasal spray of prolonged sx
274
Headaches
presentation of cervical spondylosis
- neck pain worse with movement
| - headache
275
Headaches
what are the 3 branches of the trigeminal nerve
- Ophthalmic (V1)
- Maxillary (V2)
- Mandibular (V3)
276
Headaches
presentation of trigeminal neuralgia
- intense facial pain
- few sec - hours
- electricity-like shooting pain
277
Headaches
triggers for trigeminal neuralgia
- cold weather
- spicy food
- caffeine
- citrus fruits
278
Headaches
1st line mnx for trigeminal neuralgia
carbamazepine
other option: Surgery to decompress or intentionally damage the trigeminal nerve
279
Headaches
what condition is trigeminal neuralgia associated with
multiple sclerosis
280
Headaches
cause of trigeminal neuralgia
thought to be caused by compression of the nerve
281
Headaches
presentation of cluster headaches
- severe, unbearable unilateral headaches, usually around the eye. red swollen, watering
- pupil constriction
- eyelid drooping
- nasal discharge
- facial sweating
282
Headaches
how long would a cluster headache occur
15 min- 3 hours
cluster attacks: e.g. 3-4 attacks a day for weeks or months followed by a pain-free period lasting 1-2 years
283
Headaches
what can cluster headaches be triggered by
alcohol, strong smells and exercise
284
Headaches
acute mnx for cluster headache
- SC 6mg sumatriptan
| - High flow 100% oxygen for 15-20 min
285
Headaches
prophylaxis mnx for cluster headache
- Verapamil
- Lithium
- Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)
286
Migraines
what are the 4 types
- Migraine without aura
- Migraine with aura
- Silent migraine (migraine with aura but without a headache)
- Hemiplegic migraine
287
Migraines
how long do they last
4-72h
288
Migraines
sx
- mod-severe intensity
- pounding/throbbing
- unilateral (can be bi)
- photophobia
- phonophobia
- with or without aura
- nausea and vomiting
289
Migraines
what is aura
the visual changes associated with migraines:
- sparks
- blurring
- lines across
- loss of different visual fields
290
Migraines
what can a hemiplegic migraine mimic
stroke
291
Migraines
sx of a hemiplegic migraine
- typical migraine sx
- sudden or gradual onset
- hemiplegia (unilateral weakness of limbs)
- ataxia
- changes in consciousness
292
Migraines
triggers
- stress
- blind lights
- strong smells
- food (choc, cheese, caffeine)
- dehydration
- menstruation
- abnormal sleep patterns
- trauma
293
Migraines
what are the 5 stages
- premonitory/ prodromal
- aura
- headache
- resolution
- postdromal
294
Migraines
medical mnx
- Paracetamol
- Triptans (e.g. sumatriptan 50mg as the migraine starts)
- NSAIDs (e.g ibuprofen or naproxen)
- Antiemetics if vomiting occurs (e.g. metoclopramide)
295
Migraines
how do triptans work
5HT (serotonin) receptors agonists:
- Smooth muscle in arteries to cause vasoconstriction
- Peripheral pain receptors to inhibit activation of pain receptors
- Reduce neuronal activity in the central nervous system
296
Migraines
conservative mnx
avoid triggers
acupuncture?
297
Migraines
medical prophylactic mnx
1st line: propranolol or amitriptyline
- topiramate
298
what is pseudobulbar palsy
a bilateral lesion affecting the corticobulbar tracts (running from the motor cortex to the motor nuclei of cranial nerves 9, 10, and 12 in the medulla)
299
2 week history of dysphagia, difficulty speaking, and emotional lability. On physical examination there is slow “hot-potato” speech and a brisk jaw jerk reflex. what is it
pseudobulbar palsy
300
Lambert-Eaton Myasthenic Syndrome
which pts tend to get this
patients with small-cell lung cancer.
301
Lambert-Eaton Myasthenic Syndrome
pathophysiology
antibodies are produced against voltage-gated calcium channels in small cell lung cancer (SCLC) cells.
they also target and damage voltage-gated calcium channels in the presynaptic terminals of the NMJ
302
Lambert-Eaton Myasthenic Syndrome
presentation
- proximal leg muscle weakness
- diplopia
- ptosis
- slurred speech and dysphagia
- autonomic dysfunction
- reduced reflexes
303
Lambert-Eaton Myasthenic Syndrome
what happens to tendon reflexes
reduced reflexes but
they become temporarily normal for a short period following a period of strong muscle contraction
e.g. maximally contract the quads for a period, then have their reflexes tested immediately afterwards, and display an improvement in the response
this is POST-TETANIC POTENTIATION
304
Lambert-Eaton Myasthenic Syndrome
medical mnx
Amifampridine (blocks voltage-gated K channels in the presynaptic cells which prolongs the depolarisation of the cell membrane and assists Ca channels in carrying out their action)
other:
- prednisolone, azathioprine
- IV immunoglobulins
- plasmapheresis
305
parkinson sx + arm flailing around. Dx?
Cortico-basal degeneration (CBD)
306
Parkinsonism and vertical gaze palsy. Dx?
Progressive supranuclear palsy
307
Parkinsonism and early autonomic clinical features such as: postural hypotension, incontinence, and impotence. Dx?
Multiple system atrophy
308
what causes Brown-Sequard syndrome
lateral hemisection of the spinal cord:
- Cord trauma (penetrating injuries being the most common)
- Neoplasms
- Disk herniation
- Demyelination
- Infective/ inflammatory lesions
- Epidural hematomas
309
features of Brown-Sequard syndrome
- ipsilateral weakness below lesion
- ipsilateral loss of proprioception and vibration sensation
- contralateral loss of pain and temperature sensation
310
what are the typical findings on MRI in Huntington's disease
atrophy of the caudate nucleus and putamen
311
what are the typical findings on MRI in Alzeheimer's
cerebral atrophy with enlarged ventricles and sulcal widening
312
arm was forcefully abducted --> small hand muscle paralysis, dermatomal sensory disturbance and ptosis (possible Horner's syndrome)
dx?
Klumpke's palsy: damage to lower brachial plexus C8-T1
313
cerebellar signs, tremor and rhythm abnormalities in a young patient
dx?
Wilson's disease
314
presentation of unilateral X and XI palsies:
- soft palate and uvula deviation
- weakness of head twisting and shoulder shrugging
Jugular foramen syndrome
315
Subarachnoid Haemorrhage
where is the bleed
in to the subarachnoid space
where the CSF is located
between the pia mater and arachnoid membrane
316
Subarachnoid Haemorrhage
what is it due to
a ruptured cerebral aneurysm
317
Subarachnoid Haemorrhage
presentation
- thunderclap headache
- sudden onset, occpital
- neck stiffness
- photophobia
- vision changes
- neuro sx: speech, weakness, seizure, loss of conscioussness
318
Subarachnoid Haemorrhage
trigger
strenuous activity such as weight lifting or sex
319
Subarachnoid Haemorrhage
who is it more common in
Black patients
Female patients
Age 45-70
320
Subarachnoid Haemorrhage
particularly associated with?
- Cocaine use
- Sickle cell anaemia
- Connective tissue disorders (such as Marfan syndrome or Ehlers-Danlos)
- Neurofibromatosis
- Autosomal dominant polycystic kidney disease
321
Subarachnoid Haemorrhage
1st line inx
immediate CT head
322
Subarachnoid Haemorrhage
CT result
hyperattenuation in the subarachnoid space
323
Subarachnoid Haemorrhage
when is lumbar puncture used
to collect a sample of the CSF if the CT head is negative.
324
Subarachnoid Haemorrhage
CSF signs
- raised red cell count
| - xanthochromia (the yellow colour of CSF caused by bilirubin)
325
Subarachnoid Haemorrhage
inx once a subarachnoid haemorrhage is confirmed to locate the source of the bleeding.
Angiography (CT or MRI)
326
Subarachnoid Haemorrhage
mnx
- supportive
- coiling: placing platinum coils into the aneurysm and sealing it off from the artery
- or clipping
327
Subarachnoid Haemorrhage
common complication that can result in brain ischaemia following a subarachnoid haemorrhage
Vasospasm
328
Subarachnoid Haemorrhage
mnx to prevent vasospasm
Nimodipine
329
Subarachnoid Haemorrhage
what is required to treat hydrocephalus
Lumbar puncture or insertion of a shunt
330
Subarachnoid Haemorrhage
what can be used to treat seizures
Antiepileptic medications
331
S1 dermatome
back of the thigh and some of the back of the calf
332
S1 myotome
ankle plantarflexion.
333
L2 dermatome
lateral upper thigh extending towards the groin
334
L2 myotome
hip flexion
335
which nerve roots does the femoral nerve come off
L2-L4
336
L3 dermatome
medial side of the thigh
337
L3 myotome
knee extension
338
L4 dermatome
lateral aspect of the upper shin + dorsal medial foot
339
L4 myotome
ankle dorsiflexion
340
Multiple Sclerosis
what is it
chronic and progressive condition that involves demyelination of the myelinated neurones in the CNS
341
Multiple Sclerosis
what covers the axons of neurones in the CNS
myelin
helps the electrical impulse move faster along the axon
342
Multiple Sclerosis
what cells provide the myelin in the peripheral NS
Schwann cells
343
Multiple Sclerosis
what cells provide the myelin in the CNS
oligodendrocytes
344
Multiple Sclerosis
does MS affect the CNS or peripheral
typically the CNS (oligodendrocytes)
345
Multiple Sclerosis
what does it mean when lesions are 'disseminated in time and space'
lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time.
346
Multiple Sclerosis
cause
unclear but combination of:
- Multiple genes
- Epstein–Barr virus (EBV)
- Low vitamin D
- Smoking
- Obesity
347
Multiple Sclerosis
what is the most common presentation of MS
optic neuritis
348
Multiple Sclerosis
signs and sx
- optic neuritis
- eye movement abnormalities
- focal weakness
- focal sensory sx
- ataxia
349
Multiple Sclerosis
why may pts present with double vision
lesion with the 6th CN (abducens nerve)
350
Multiple Sclerosis
6th cranial nerve palsy presentation
- internuclear ophthalmoplegia
| - conjugate lateral gaze disorder
351
Multiple Sclerosis
what is internuclear ophthalmoplegia
internuclear: CN nuclei that control eye movement (3,4,6) to ensure eyes move together
opthalmoplegia: Problem with muscle around eye
352
Multiple Sclerosis
what is conjugate lateral gaze disorder
conjugate: connected
lateral gaze: where both eyes move together to look laterally to the left or right.
When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct.
353
Multiple Sclerosis
what focal weakness may be present
- bell's palsy
- horner's syndrome
- limb paralysis
- incontinence
354
Multiple Sclerosis
what are the focal sensory sx
- Trigeminal neuralgia
- Numbness
- Paraesthesia (pins and needles)
- Lhermitte’s sign
355
Multiple Sclerosis
what is Lhermitte's sign
an electric shock sensation that travels down the spine and into the limbs when flexing the neck.
It indicates disease in the cervical spinal cord in the dorsal column.
It is caused by stretching the demyelinated dorsal column.
356
Multiple Sclerosis
what is sensory ataxia
- loss of proprioception
357
Multiple Sclerosis
what can a positive Romberg's test indicate
sensory ataxia
358
Multiple Sclerosis
what is cerebellar ataxia
problems with the cerebellum coordinating movement.
This suggestions cerebellar lesions.
359
Multiple Sclerosis
what is a clinically isolated syndrome
the first episode of demyelination and neurological signs and symptoms
cannot be diagnosed as MS as not been disseminated in time and space
360
Multiple Sclerosis
what is the most common pattern
relapsing-remitting
361
Multiple Sclerosis
relapsing-remitting: active
new symptoms are developing or new lesions are appearing on MRI
362
Multiple Sclerosis
relapsing-remitting: not active
no new symptoms or MRI lesions are developing
363
Multiple Sclerosis
relapsing-remitting: worsening
there is an overall worsening of disability over time
364
Multiple Sclerosis
relapsing-remitting: not worsening
there is no worsening of disability over time
365
Multiple Sclerosis
what is the secondary progressive pattern
there was relapsing-remitting disease at first, but now there is a progressive worsening of symptoms with incomplete remissions
366
Multiple Sclerosis
what are the types of secondary progressive pattern
- active
- not active
- progressing
- not progressing
367
Multiple Sclerosis
what is primary progressive pattern
worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions
sx have to be progressive >1y
368
Multiple Sclerosis
what are the types of primary progressive pattern
- active
- not active
- progressing
- not progressing
369
Multiple Sclerosis
what inx can support dx
- MRI: lesions
| - lumbar puncture
370
Multiple Sclerosis
what would lumbar puncture show
oligoclonal bands in the CSF
371
Multiple Sclerosis
presentation of optic neuritis
- unilateral reduced vision developing over hrs - days
- Central scotoma. This is an enlarged blind spot.
- pain on eye movement
- impaired colour vision
- Relative afferent pupillary defect
372
Multiple Sclerosis
causes of optic neuritis
- MS
- Sarcoidosis
- SLE
- Diabetes
- Syphilis
- Measles
- Mumps
- Lyme disease
373
Multiple Sclerosis
mnx of optic neuritis
steroids and recovery takes 2-6 weeks.
374
Multiple Sclerosis
mnx
- MDT approach
| - disease modifying drugs + biologic therapy
375
Multiple Sclerosis
how to treat relapses
methylprednisolone
500mg PO OD 5d
or 1g IV OD 3-5d 2nd line
376
Multiple Sclerosis
what can spasticity be managed with
baclofen, gabapentin and physiotherapy
377
presentation of Internuclear ophthalmoplegia
- impaired adduction of the eye on the same side as the lesion
- horizontal nystagmus of the abducting eye on the contralateral side
378
pathophysiology of Internuclear ophthalmoplegia
lesion in the medial longitudinal fasciculus (MLF)
379
causes of Internuclear ophthalmoplegia
- MS
| - vascular disease
380
features of cerebellar dysfunction
DANISH
Dysdiadochokinesia
Ataxia
Nystagmus
Intention tremor
Slurred speech
Hypotonia
381
Neuropathic Pain
what is it caused by
abnormal functioning of the sensory nerves delivering abnormal and painful signals to the brain.
382
Neuropathic Pain
what is used to assess the characteristics of the pain and examination of the affected area
DN4 Questionnaire
scored out of 10 fpr their pain
383
Neuropathic Pain
what score indicates neuropathic pain on the DN4 questionnaire
4 or more
384
Neuropathic Pain
what are the four 1st line trx
- Amitriptyline (TCA)
- Duloxetine (SNRI antidepressant)
- Gabapentin (anticonvulsant)
- Pregabalin (anticonvulsant)
NICE: if 1 doesn't work, stop and start an alternative until all 4 have been tried
385
Neuropathic Pain
when to use tramadol
ONLY as a rescue for short term control of flares
386
Neuropathic Pain
when to use Capsaicin cream (chilli pepper cream)
for localised areas of pain
387
Neuropathic Pain
what is Complex Regional Pain Syndrome
areas are affected by abnormal nerve functioning causing neuropathic pain and abnormal sensations. It is usually isolated to one limb.
388
Neuropathic Pain
what is Complex Regional Pain Syndrome often triggered by
an injury to the area.
389
Neuropathic Pain
presentation of complex regional pain syndrome
- area very painful and hypersensitive e.g. wearing clothes
- intermittently swell, change colour, temp, flush with blood
- abnormal sweating
- abnormal hair growth
390
Neuropathic Pain
trx of complex regional pain syndrome
guided by a pain specialist and is similar to other neuropathic pain.
391
Which sign on MRI would support the diagnosis of encephalitis?
Bilateral medial temporal lobe involvement
392
74 year old T1DM
L common peroneal nerve palsy leading to motor and sensory loss. recovered spontaneously after 3w
3m later: transient left-sided facial weakness - recovered spontaneously after 2w
O/E peripheral sensory loss affecting both feet
what is it
Mononeuritis multiplex
393
what is the difference between type 1 and type 2 charcot-marie tooth disease
type 1: demyelinating condition which is more common (and includes the PMP22 subtype)
type 2: axonal
394
what does the anterior spinal artery supply
the anterior 2/3 of the spinal cord (not the dorsal column)
395
what is the dorsal column responsible for
vibration, two-point discrimination, and proprioception
396
39 year old man
now + then jerks his head violently to one side
what kind of seizure could this be
myoclonic
397
Creutzfeldt-Jakob disease
what is it
a group of neuro-degenerative diseases caused by prions (mis-shaped proteins).
398
Creutzfeldt-Jakob disease
presentation
- myoclonus
- rapidly progressive dementia
- psych
399
Creutzfeldt-Jakob disease
what will CSF show
normal or abnormal proteins e.g. 14-3-3 protein
400
Creutzfeldt-Jakob disease
what will EEG show
- biphasic, high amplitude sharp waves (only in sporadic CJD)
401
Creutzfeldt-Jakob disease
what will MRI show
hyperintense signals in the basal ganglia and thalamus
402
Creutzfeldt-Jakob disease
dx
tonsil/olfactory mucosal biopsy
403
Creutzfeldt-Jakob disease
mean age of onset between sporadic CJD and new variant CJD
sporadic CJD: 65y
new onset variant CJD: 25y
404
what is cushings triad and what does it indicate
raised ICP
1. bradycardia
2. hypertension
3. irregular/abnormal breathing.
405
what is the most common cause of surgical 3rd nerve palsy
posterior communicating artery aneurysm, located in the circle of Willis.
406
why is there hyperacusis in Bell's Palsy
Paralysis of the stapedius muscle prevents its function in dampening the oscillations of the ossicles, causing sound to be abnormally loud on the affected side
407
why look in the ear in Bell's Palsy
Check for ramsey hunt vesicles
408
what is the most important side effect to look out for for pts on Ropinirole (a dopamine agonist)
impulsivity
409
30 year old woman has severe headache 24 hours after a spinal anaesthetic. What is the most likely diagnosis
low pressure headache
410
73yo: 3m of increasing weakness of R hand w/ reduced sensation of forearm. Wasting of all intrinsic muscles of R hand. Weakness of finger abduction + adduction, + thumb adduction. Finger flexion is normal. Mild altered light touch sensation along ulnar aspect of forearm. Biceps, supinator + triceps reflexes are normal. The lower limbs and L arm are normal.
Where is the most likely site of the lesion causing his symptoms?
- intrinsic hand muscle wasting suggests T1
- normal reflexes and normal other arm are against a cord lesion.
- The sensory loss on the forearm excludes median and ulnar nerve lesions.
- T1 dermatome is often thought to be higher in the arm medially
411
immediate mnx of malignant spinal cord compression
dexamethasone
412
A 17 year old boy has repeated episodes characterised by a funny 'racing' sensation in his abdomen, followed by loss of awareness. His girlfriend describes that he has a vacant stare and waves his left arm around in a writhing manner during these attacks
Which is the most likely site of origin of these episodes
right temporal lobe
the aura implicates one of the temporal lobes
