Neurology Flashcards
1
Q
Why can the parasympathetic fibres of the oculomotor nerve become paralysed without the motor fibres?
A
They lie on the outside of the nerve.
2
Q
Why are 3rd cranial nerve palsies common?
A
The nerve lies over the apex of the petrous part of the temporal bone.
3
Q
What are the signs of a 3rd parasympathetic nerve palsy?
A
Fixed dilated pupil.
4
Q
What separates the posterior cranial fossa (cerebellum) from the cerebrum?
A
Tentorium
5
Q
What is the consequence of the foramen of Magendie or the foramina of Lushka getting blocked?
A
Obstructive hydrocephalus
6
Q
Give two signs of cerebellar syndrome.
A
- Ataxia
- Nystagmus
7
Q
If the cerebellum is injured, is the deficit ipsilateral or contralateral?
A
Ipsilateral
8
Q
What is the brainstem bound by superiorly and inferiorly?
A
SUPERIORLY
- thalamus and internal capsule
INFERIORLY
- spinal cord
9
Q
How is the cerebellum ‘connected’ to the rest of the brain?
A
Via peduncles connecting to the brainstem.
10
Q
Which dorsal column carries sensation from the lower limbs?
A
Fasiculus gracilis (medial)
11
Q
Which dorsal column carries sensation from the upper limbs?
A
Fasiculus cuneatus (lateral)
12
Q
What sensations does the DCML pathway carry?
A
- Proprioception
- Fine touch
- Vibration
13
Q
Where does the DCML decussate?
A
Medulla
14
Q
What sensations does the spinothalamic tract carry?
A
ANTERIOR
- Crude touch
LATERAL
- Pain
- Temperature
15
Q
Where do the spinothalamic tracts decussate?
A
2-3 levels above entry to the spinal cord
16
Q
Where does the corticospinal tract decussate?
A
ANTERIOR
- Just before they exit the spinal cord
LATERAL
- Pyramids of medulla
17
Q
What does the anterior corticospinal tract innervate?
A
Proximal muscles (trunk)
18
Q
What does the lateral corticospinal tract innervate?
A
Muscles of the limbs
19
Q
What does the corticobulbar tract innervate?
A
Head and neck muscles
20
Q
Give five functions of the reticular activating system.
A
- Alertness
- Sleep/wake
- REM/nonREM sleep
- Respiratory centre
- Cardiovascular drive
21
Q
Give two brain structures associated with the reticular activating system.
A
- Peri-aqueductal grey
- Floor of fourth ventricle
22
Q
Give seven criteria for brainstem death.
A
- Absent pupillary reflexes
- Absent corneal reflex
- Absent caloric vestibular reflex
- Absent cough reflex
- Absent gag reflex
- Absent respirations
- No response to pain
23
Q
Describe Aa nerve fibres (size, myelination, what information do they carry?).
A
- Large
- Myelinated
- Proprioception
24
Q
Describe Ab nerve fibres (size, myelination, what information do they carry?).
A
- Large
- Myelinated
- Light touch, pressure, vibration
25
Describe Adelta nerve fibres (size, myelination, what information do they carry?).
- Small
- Myelinated
- Cold and pain
26
Describe C nerve fibres (size, myelination, what information do they carry?).
- Small
- Unmyelinated
- Warmth and pain
27
Give six mechanisms of nerve damage.
- Demyelination
- Axonal damage
- Wallerian degeneration
- Compression
- Infarction
- Infiltration
28
What would a nerve conduction study show in demyelinated nerves?
Slower conduction velocities
29
What would a nerve conduction study show in axonal damage?
Reduced amplitudes of potentials
30
Is demyelination or axonal damage more common?
Axonal damage
31
What causes wallerian nerve degeneration?
Section of a nerve
32
What does nerve compression result in?
Focal demyelination
33
Describe what causes infarction of a nerve and give two conditions in which it occurs.
Infarction of vasa nervosum in vasculitis and diabetes.
34
Give seven general causes of axonal peripheral neuropathies.
- Systemic diseases
- Inflammatory
- Infectious
- Ischaemic
- Metabolic
- Hereditary
- Toxins
35
Give 11 systemic diseases that can cause axonal damage.
- Diabetes mellitus
- B12 deficiency
- Coeliac disease
- Chronic kidney disease
- Excessive alcohol
- Hypothyroidism
- Amyloidosis
- Connective tissue disease
- Paraproteinaemia
- Paraneoplastic syndrome
- Critical illness polyneuropathy
36
Give three infections that may cause axonal peripheral neuropathies.
- Hepatitis
- HIV
- Lyme
37
Give a condition which makes mononeuropathies more common.
Diabetes
38
Give three mononeuropathies, and the nerves which are involved.
- Carpel tunnel syndrome (median nerve)
- Ulnar neuropathy (ulnar nerve)
- Peroneal neuropathy (peroneal nerve)
39
Which cranial nerves most common are affected in mononeuropathies?
III and VII
40
What is the most common cause of a mononeuropathy?
Focal demyelination
41
What do the sensory symptoms of a peripheral neuropathy depend on?
Which fibres (Aa, Ab, Adelta, C) are lost
42
What is ataxia?
Poor balance
43
What two methods can cause ataxia?
- Sensory (loss of Aa proprioception)
| - Cerebellar
44
How can you tell the difference between sensory and cerebellar ataxia?
When sensory, ataxia gets worse with the eyes closed or in the dark.
45
Give five motor symptoms of peripheral neuropathy.
- Muscle cramps
- Weakness
- Fasciculations
- Atrophy
- Absent reflexes
46
What is a radiculopathy?
A neuropathy affecting the nerve roots.
47
Give three patterns of peripheral neuropathies.
- Symmetrical sensorimotor
- Asymmetrical sensory
- Asymmetrical sensorimotor
48
Which is the most common pattern of peripheral neuropathies?
Symmetrical sensorimotor
49
Which nerve fibres are affected first in symmetrical sensorimotor neuropathies?
Long fibres
50
Describe the presentation of symmetrical sensorimotor neuropathies.
Initially starts with sensory features affecting fingers and toes, and eventually motor symptoms develop.
51
What is another name for asymmetrical sensory neuropathies?
Sensory ganglionopathy
52
Describe where the symptoms are felt in asymmetrical sensory neuropathies.
Patchy distribution
53
What nerve structure is affected in asymmetrical sensory neuropathies?
Dorsal root ganglia
54
Give three conditions that are associated with asymmetrical sensory neuropathies.
- Paraneoplastic syndromes
- Sjogren’s
- Coeliac disease
55
Give another name for asymmetrical sensorimotor neuropathy.
Mononeuritis multiplex
56
Give four conditions that are associated with asymmetrical sensory motor neuropathies.
- Vasculitis
- Diabetes
- Sarcoidosis
- Amyloidosis
57
What three aspects of the clinical examination are important in peripheral neuropathies?
- Reduced or absent tendon reflexes
- Sensory deficit
- Weakness/muscle atrophy
58
What special investigation can be used to assess peripheral neuropathies?
Neurophysiological examination (nerve conduction studies)
59
Give three features of chronic idiopathic axonal polyneuropathy.
- Develops over at least 6 months
- No aetiology can be identified
- Axons are affected (most commonly in proportion to length)
60
Give two examples of immune-mediated chronic demyelinating peripheral neuropathies.
- Chronic inflammatory demyelinating polyneuropathy (CIDP)
| - Multifocal motor neuropathy (MMN)
61
Give two examples of genetic chronic demyelinating peripheral neuropathies.
- Charcot Marie Tooth Disease
| - Hereditary sensory and autonomic neuropathies
62
What is Charcot Marie Tooth disease also known as?
Peroneal muscular atrophy
63
Name an acute polyneuropathy.
Gullian Barre syndrome
64
What mechanism usually causes Gullian Barre syndrome?
Usually demyelinating, but occasionally can be axonal.
65
What causes Gullian Barre syndrome?
Immune mediated, usually after GI infection (eg. Campylobacter)
66
Describe the clinical presentation of Gullian Barre syndrome.
Rapid ascending paralysis and sensory deficits, usually occurring over a few days.
67
What is the treatment for Gullian Barre Syndrome?
IMMEDIATE IV Ig or plasma exchange.
68
Give three approaches to the treatment of peripheral neuropathies.
- Treat pain
- Treat cramps
- Improve balance
69
What can be used to treat cramps in peripheral neuropathies?
Quinine
70
What is the treatment for chronic inflammatory demyelinating polyneuropathy?
IV Ig/plasma exchange in flare ups, + long term steroids.
71
What is meningitis?
Inflammation of the meninges
72
Give three non-infective causes of meningitis.
- Paraneoplastic
- Drug side effects
- Autoimmune (vasculitis, SLE)
73
Give three causative organisms of meningitis in neonates.
- Listeria
- Group B Strep
- E.coli
74
Give three causes of meningitis in children.
- Neisseria meningitidis
- Strep.pneumoniae
- Hib
75
Give two causes of meningitis in adults.
- Neisseria meningitidis
| - Strep.pneumoniae
76
Give three causes of meningitis in the elderly.
- Neisseria meningitidis
- Strep.pneumoniae
- Listeria
77
What is the condition called if Neisseria meningitidis is found in the blood culture?
Meningococcal septicaemia
78
Give three viruses that can cause acute meningitis.
- Enterovirus
- Herpes simplex virus
- Varicella zoster virus
79
Give three organisms that can cause chronic meningitis.
- Mycobacterium tuberculosis
- Syphilis
- Cryptococcus
80
Give four ways that infection can spread to the meninges.
- Extracranial infection (nasal, otitis media, sinusitis)
- Neurosurgical complications
- Trauma
- Bloodstream
81
Describe the pathophysiology of bacterial meningitis.
- Bacteria enter CSF and replicate
- Blood vessels dilate and allow WBC to enter CSF
- This causes meningeal inflammation +/- brain swelling
82
What are the symptoms of meningitis?
- Fever
- Headache
- Neck stiffness (meningism)
- Photophobia
- Leg pains
- Cold hands and feet
- May develop neurological signs
83
What is the management of meningitis if the patient presents to primary care?
- IM benzylpenicillin
| - Send to hospital
84
Describe the immediate management of suspected meningitis in hospital.
- Assess GCS
- Blood cultures
- Broad spectrum antibiotics
- Steroids (IV dexamethosone)
85
Why are steroids given in suspected meningitis?
They reduce brain swelling and neurological sequelae
86
What empirical antibiotics should be given in suspected meningitis?
Ceftriaxone/Cefotaxime
87
Describe the reasoning behind the choice of empirical antibiotics in meningitis.
Cephalosporins are bactericidal and can cross BBB.
88
What empirical antibiotics should be given in meningitis if the patient has a penicillin allergy?
Chloramphenicol
89
What empirical antibiotics should be added (and why) for immunocompromised patients with suspected meningitis?
Amoxicillin for Listeria
90
What empirical antibiotics (and why) should be added in suspected meningitis in patients with a recent history of travel?
Vancomycin for penicillin resistant Strep pneumoniae
91
What investigation should be carried out in suspected meningitis, and what further investigations should be carried out on the sample obtained?
Lumbar puncture
- Microscopy
- Gram stain
- Culture
- Protein
- Glucose
- Viral PCR
92
Give three contraindications to lumbar puncture in suspected meningitis.
- Abnormal clotting
- Petichial rash
- Raised intracranial pressure
93
Give three unique clinical features of TB meningitis.
- Weight loss
- Night sweats
- Insidious onset
94
What would you find on performing a lumbar puncture in cryptococcal meningitis?
High opening pressure
95
Describe the differences between the clinical features of bacterial and viral meningitis.
BACTERIAL may appear septic with focal neurology and rash.
| VIRAL is less severe.
96
Give a group of patients more likely to get viral meningitis.
Small children
97
Describe the appearance of the CSF in bacterial meningitis.
Cloudy
98
Describe the appearance of the CSF in viral meningitis.
Clear
99
Describe the appearance of the CSF in TB meningitis.
Fibrin web
100
Describe the appearance of the CSF in cryptococcal meningitis.
Fibrin web
101
What cells would be seen on CSF microscopy in bacterial meningitis?
Neutrophils
102
What cells would be seen on CSF microscopy in viral meningitis?
Lymphocytes
103
What cells would be seen on CSF microscopy in TB meningitis?
Lymphocytes
104
What cells would be seen on CSF microscopy in cryptococcal meningitis?
Lymphocytes
105
What stain would be used to see cryptococcus on CSF microscopy?
India Ink
106
Describe the CSF protein levels in meningitis.
high
107
Which type of meningitis will show a normal (instead of low) CSF glucose?
Viral
108
Give six differential diagnoses of meningitis.
- Subarachnoid haemorrhage
- Migraine
- Flu/other viral illness
- Sinusitis
- Brain abscess
- Malaria
109
What is the mortality rate in treated meningitis?
5%
110
What percentage of patients with treated meningitis will have permanent effects?
20%
111
Give five complications of bacterial meningitis.
- Skin scars
- Amputation
- Hearing loss
- Seizures
- Brain damage
112
Who should be called in cases of meningitis?
Public health
113
How are close contacts of patients with Neisseria meningitidis treated?
Antibiotic prophylaxis of ciprofloxacin or rifampicin
114
What is encephalitis?
Inflammation of the brain
115
What is the most common cause of encephalitis?
Herpes simplex
116
How does Herpes simplex cause encephalitis?
Reactivation in the trigeminal ganglion
117
Give nine causes of encephalitis.
- Herpes simplex
- Varicella zoster
- Measles
- Mumps
- Rubella
- EBV
- HIV
- CMV
- Coxsackie
118
Is encephalitis usually bacterial or viral?
Viral
119
Give four causes of encephalitis that are associated with travel.
- Japanese encephalitis virus
- Tick-borne encephalitis
- Rabies
- West Nile
120
Give two non-infective causes of encephalitis.
- Autoimmune
| - Paraneoplastic
121
Describe the clinical presentation of encephalitis.
- Preceding flu-like illness hours to days before
- Altered GCS (confusion, drowsiness, coma)
- Fever
- Seizures
- Memory loss
- May have meningism
122
What investigations should be carried out in encephalitis?
- MRI head +/- EEG
- Lumbar puncture
- HIV test
123
What is the treatment for encephalitis?
- Mostly supportive (neurorehab)
| - Aciclovir if HSV or VZV
124
What is tetanus and what organism is it caused by?
Clinical syndrome caused by Clostridium tetani
125
How is tetanus contracted by humans?
Through skin
126
Where is Clostridium tetani found?
Globally in soil
127
What is the incubation period for Clostridium tetani?
8 days
128
What are the two toxins produced by Clostridium tetani, and what are their effects?
- Tetanolysin (tissue destruction)
| - Tetanospasmin (clinical tetanus)
129
Describe the pathophysiology of tetanus.
- Tetanospasmin toxin travels retrogradely along axons
- Interferes with neurotransmitter release to increase neurone firing
- Unopposed muscle contraction and spasm
130
Give two presentations of generalised tetanus.
- Risus sardonicus (facial muscles)
| - Opisthotonos (whole body)
131
What is it called if tetanus only affects the muscles around the injury?
Localised tetanus
132
Describe the management of tetanus.
- Vaccinate if patient has risk injury
- Supportive (muscle relaxants, paracetamol, cooling)
- Immunoglobulin to mop up toxin
- Metronidazole for residual bacteria
133
Is rabies a bacterial or viral infection?
Viral
134
Where is rabies found?
Saliva of animals (dogs, cats, foxes, bats)
135
How do humans catch rabies?
Inoculation through the skin (eg. Lick, bite, splash)
136
Where is there high risk of Rabies?
Parts of Africa and South East Asia
137
What is the incubation period for rabies?
Can be 2 weeks to years, depending on size and site of inoculation (virus has to get to CNS)
138
How does rabies affect nerves?
Travels retrogradely along them
139
Describe the presentation of rabies.
- Paraesthesia around bite
| - Furious or paralytic presentation once it reaches CNS
140
Describe the furious reaction to rabies.
- Hydrophobic
| - Aerophobic
141
What is the management for rabies?
- Sedatives (fatal once symptomatic)
| - Pre and post exposure prophylaxis (vaccination and immunoglobulin)
142
Is non-missile or penetrating head trauma more common?
Non-missile
143
Give four types of diffuse head trauma.
- Diffuse axonal injury
- Diffuse vascular injury
- Hypoxia-ischaemia
- Swelling
144
Give two focal head traumas affecting the scalp.
- Contusions
| - Lacerations
145
Give a focal head trauma involving the skull.
Fracture
146
Give two focal head traumas involving the meninges.
- Infection
| - Haemorrhage
147
Give four focal head traumas involving the brain.
- Contusions
- Lacerations
- Haemorrhage
- Infection
148
Define contusion.
A region of injured tissue/skin in which capillaries have been ruptured (a bruise).
149
Define laceration.
A deep cut or tear in skin or flesh.
150
Give three possible consequences of skull fracture.
- Haematoma
- Infection
- Aerocele
151
Describe the type of skull fracture associated with pointed objects.
Localised fractures that are open or depressed.
152
Describe the skull fractures that can be caused by flat surfaces.
Linear fractures that can extend to skull base.
153
What can be used to work out the order that skull fractures were sustained?
New fracture lines don’t cross pre-existing fracture lines.
154
Which artery is often torn to form an extradural haematoma?
Middle meningeal artery
155
What other head injury is extradural haematoma usually associated with?
Skull fracture
156
Describe the rate of onset of extradural haematoma.
Occurs slowly (hours)
157
Describe the clinical presentation of an extradural haematoma.
Patient usually has a lucid interval before deterioration.
158
Give three consequences of an extradural haematoma.
- Brain displacement
- Raised intracranial pressure
- Herniation
159
What vessels tear to cause a subdural haematoma?
Bridging veins which cross the subdural space.
160
Describe what causes acute and chronic subdural haematoma.
- SLOW onset in elderly
| - QUICK onset in trauma
161
What type of head trauma commonly causes subdural haematoma?
Acceleration/deceleration
162
Describe the appearance of subdural haematoma.
Usually surrounded by a membrane of granulation tissue.
163
Describe the severity of trauma required to cause a subdural haematoma in the elderly.
Can be caused by mild trauma
164
Give four causes of traumatic subarachnoid haematoma.
- Contusions/lacerations
- Base of skull fracture
- Vertebral artery rupture/dissection
- Intraventricular haemorrhage
165
What causes deep and superficial cerebral and cerebellar haemorrhage?
SUPERFICIAL - Severe contusion
| DEEP - acceleration and deceleration (related to diffuse axonal injury)
166
What type of head injury commonly causes infection?
Skull fracture
167
What is a ‘coup’ contusion?
Contusion at the site of impact
168
What is a ‘contrecoup’ injury?
Injury away from the site of impact.
169
Where do most contrecoup injuries occur and why?
Base of the brain due to a more bumpy skull.
170
Describe how brain contusions change over time.
- Haemorrhagic at first
- Then become brown/orange and soft
- Then indented or cavitated after months/years
171
When does laceration occur in relation to contusion?
Laceration occurs when contusion is severe enough to tear Pia Mater.
172
Give two diffuse brain lesions which take time to develop.
- Swelling
| - Hypoxia-ischaemia
173
Give two diffuse brain lesions which occur immediately on trauma.
- Diffuse axonal injury
| - Diffuse vascular injury
174
What is diffuse axonal injury?
Syndrome of widespread axonal damage. Can be caused by a variety of processes.
175
What head trauma usually causes diffuse traumatic axonal injury?
Acceleration and deceleration.
176
What does traumatic acceleration and deceleration of the head cause?
Tearing of long structures (blood vessels and axons)
177
Describe the prognosis of mild diffuse traumatic axonal injury.
Recovery of consciousness +/- long term deficit
178
Describe the prognosis of severe diffuse traumatic axonal injury.
Unconscious from impact and remain unconscious or severe disability.
179
Describe the histology of diffuse axonal injury.
Build up of transported materials due to tear in axon and degeneration of involved fibre tracts.
180
In what percentage of head injury patients does brain swelling occur?
75%
181
Give two consequences of brain swelling.
- Raised intracranial pressure
| - Hypoxic-ischaemic change
182
Give three mechanisms of brain swelling.
- Congestive brain damage
- Vasogenic oedema
- Cytotoxic oedema
183
Describe congestive brain damage.
Vasodilation and increased cerebral blood volume.
184
Describe how vasogenic oedema causes brain swelling.
Extravasation from damaged blood vessels.
185
Describe how cytotoxic oedema causes brain swelling.
Increased water content of neurones and glia.
186
What is the prognosis for diffuse vascular injury in the brain.
Usually results in near immediate death.
187
Describe the appearance of diffuse vascular injury of the brain.
Multiple petechial haemorrhages throughout the brain.
188
Give two causes of brain herniation.
- Bleeding
| - Brain swelling
189
What is seen on radiology in brain herniation?
Midline shift
190
Give three types of brain herniation.
- Subfalcine herniation of cingulate gyrus
- Transtentorial herniation of medial temporal lobe
- Transforaminal herniation of cerebellar tonsil
191
Give three risk factors for hypoxia-ischaemia of the brain.
- Clinically evident hypoxia
- Hypotension (<80mmHg for >15mins)
- Raised intracranial pressure
192
Give two vulnerable regions where brain hypoxia-ischaemia may occur.
- Susceptible neurones
| - Border zones between major cerebral territories
193
How long after head injury does chronic traumatic encephalopathy occur?
Years
194
Describe the type of head injury that usually causes chronic traumatic encephalopathy.
Repetitive mild traumatic brain injury
195
Describe the initial presentation of chronic traumatic encephalopathy.
- Irritability
- Impulsivity
- Aggression
- Depression
- Memory loss
196
Describe the later presentation of chronic traumatic encephalopathy.
- Dementia
- Gait problems
- Speech problems
- Parkinsonism
- Some have motor neurone disease like symptoms
197
Give four parts of the brain that are atrophied in chronic traumatic encephalopathy.
- Neocortex
- Hippocampus
- Diencephalon
- Mamillary bodies
198
Describe the ventricles and septum pellucidum in chronic traumatic encephalopathy.
- Enlarged ventricles
| - Fenestrated septum pellucidum
199
Describe protein/plaque build up in chronic traumatic encephalopathy.
- Tau-positive neurofibrillary and astrocytic tangles
| - TDP-43 pathology
200
Give three regions of the brain where tau-positive neurofibrillary tangles accumulate in chronic traumatic encephalopathy.
- Frontal/temporal cortex
- Limbic regions
- Depths of sulci and limbic regions
201
What is dementia?
A syndrome encompassing progressive deficits in several cognitive domains.
202
What is the most common first presentation of dementia?
Memory loss over months/years
203
Give three symptoms that may develop in later stage dementia.
- Agitation
- Aggression
- Apathy
204
What is the most common cause of dementia?
Alzheimer’s dementia
205
What are the second and third most common causes of dementia?
```
2nd = Vascular
3rd = Lewy bodies
```
206
Briefly describe the pathology of vascular dementia.
Cumulative effects of many small strokes.
207
Give a characteristic history in vascular dementia.
Sudden onset and stepwise deterioration.
208
What is fronto-temporal (Pick’s) dementia?
Frontal and temporal atrophy without Alzheimers.
209
Give twelve less common causes of dementia.
- Hypothyroidism
- Low B12/folate
- Thiamine deficiency (alcohol)
- Syphilis
- Tumours
- Subdural haematoma
- Parkinson’s
- CNS cysticercosis
- HIV
- Hydrocephalus
- Whipple’s disease
- Pellagra
210
Give four features of vascular/mixed dementia.
- Attention difficulties
- Motor/cognitive slowing
- Executive problems
- Visuo-spatial difficulties
211
Briefly describe the pathology in Dementia with Lewy bodies.
Intracytoplasmic Lewy bodies in the neurones.
212
Give five features of Dementia with Lewy bodies.
- Fluctuating cognition
- Pronounced disturbances in attention and concentration
- Working memory and early visuospacial deficits
- Visual hallucinations
- Spontaneous Parkinsonism
213
What is Alzheimer’s disease?
A neurodegenerative disease characterised clinically by dementia and histopathologically by neuronal loss in the cerebral cortex in association with numerous amyloid plaques and neurofibrillary tangles.
214
What is a major component of neurofibrillary tangles?
Tau
215
What is the most common variant of Alzheimer’s dementia?
Amnesic
216
Describe the pattern of degeneration in the amnesic variant of Alzheimer’s dementia.
Early degeneration in medial temporal lobe, then spreads to temporal neocortex, frontal and parietal association regions.
217
Give five things that the temporal lobe is responsible for.
- Hearing
- Language comprehension
- Semantic knowledge
- Memory
- Emotional/affective behaviour
218
Give three of the first features in amnesic Alzheimer’s dementia.
- Selective amnesia
- Semantic impairment
- Language impairment
219
What is the second thing to be affected in amnesic Alzheimer’s dementia?
Attention (divided, selective, and attention switching)
220
What deficits appear in late stage amnesic Alzheimer’s dementia?
- Visuospacial
- Sustained attention
- Executive functioning
- Global deficits
221
Give three areas of the brain which may be affected in visual Alzheimer’s dementia.
- Posterior occipitoparietal
- Occipitotemporal
- Primary visual cortex
222
Give five deficits experienced in visual Alzheimer’s dementia.
- Visual deficits
- Dyspraxia
- Dysgraphia
- Simultanagnosia
- Eventuall global deficits
223
What area of the brain is affected in linguistic Alzheimer’s dementia?
Lateral temporal regions.
224
What is the deficit experienced in linguistic Alzheimer’s dementia?
Progressive aphasic syndrome.
225
Give four psychiatric changes which may occur later on in Alzheimer’s dementia.
- Subtle behaviour changes
- Apathy/disengagement
- Psychotic symptoms (delusions/hallucinations)
- Agitation/anxity
226
Give five behaviour changes which may occur in Alzheimer’s dementia.
- Inattentiveness
- Mild cognitive dulling
- Social withdrawal
- Emotional withdrawal
- Agitation
227
Give three criteria in the history/examination of the patient for Alzheimer’s dementia.
- Presence of early and significant memory impairment
- Gradual and progressive change in memory function over more than 6 months
- Objective evidence of amnesic syndrome of hippocampal type (based on performance of episodic memory test)
228
Give three pieces of in vivo evidence for Alzheimer’s dementia.
- Decreased b-amyloid and increased T-tau/P-tau in CSF
- Increased tracer retention on amyloid PET
- AD autosomal dominant mutation present
229
Give three genes which are associated with Alzheimer’s dementia.
- PSEN1
- PSEN2
- APP
230
How is Down’s Syndrome associated with Alzheimer’s dementia?
The APP gene which produces amyloid precursor protein is on chromosome 21 so people with Down’s syndrome will have more b-amyloid.
231
Give seven clinical criteria which can exclude Alzheimer’s dementia.
- Sudden onset
- Early occurrence of gait disturbances/seizures
- Major and prevalent behaviour changes
- Focal neurological features
- Early extrapyramidal signs
- Early hallucinations
- Cognitive fluctuations
232
Give three specialised diagnostic tests for Alzheimer’s dementia.
- Structural MRI
- Amyloid imaging (PET)
- Functional MRI
233
Give five ‘criteria’ for diagnosing amnesic prodrome (preclinical Alzheimer’s dementia).
- Poor performance on episodic memory tests
- General cognitive function preserved
- Activities of daily living intact
- No evidence of dementia
- Score >24/30 on MMSE
234
Give nine lifestyle factors that may help to prevent Alzheimer’s dementia.
- Smoking cessation
- Healthy BMI
- Healthy diet
- Physical activity
- Low alcohol
- Socially active
- Cognitively active
- Control vascular risk factors
- Treat mood/anxiety
235
Give two general categories of medication that can be used in Alzheimer’s dementia.
- Acetlycholineesterase inhibitors
| - Anti-glutamate
236
Give three examples of acetylcholineesterase inhibitors used in Alzheimer’s dementia.
- Donepezil
- Rivastigmine
- Galantamine
237
Give an anti-glutamate medication that can be used in Alzheimer’s dementia.
Memantine
238
Give three differential diagnoses of Alzheimer’s dementia.
- Vascular/mixed dementia
- Dementia with Lewy bodies
- Depressive pseudo-dementia
239
Compare the onset of dementia and depression.
DEMENTIA
- Vague, insidious onset
DEPRESSION
- Onset and decline often rapid (may follow trigger)
240
Compare the patient’s experience of symptoms in dementia and depression.
DEMENTIA
- Unaware or attempt to hide problems
DEPRESSION
- Patient complains of memory loss
241
Compare the patient’s mood in dementia and depression.
DEMENTIA
- Mood may be labile
DEPRESSION
- Patient distressed/unhappy
242
Describe the cognitive performance in dementia and depression.
DEMENTIA
- Cognitive performance consistent
- Attempts all questions
DEPRESSION
- Variability in cognitive performance
- ‘Don’t know’ answers
243
What is multiple sclerosis?
A relapsing and remitting demyelinating disease of the CNS in which episodes of neurological disturbance affect different parts of the CNS at different times.
244
At what age does MS usually begin?
20-40yrs
245
Describe and explain the geographical distribution of MS.
Much less common near equator, perhaps due to more vitamin D.
246
Is MS more common in white or black people?
White
247
Is MS more common in males or females?
Females
248
When might the potential for developing MS be established?
In early life (eg. A childhood virus)
249
Give nine conditions which may affect a person’s susceptibility for developing MS.
- Race
- Latitude
- Age
- Diet
- Sanitation
- Socioeconomic status
- Multiple gene loci
- Climate
- Mutations
250
Describe the pathophysiology of MS.
- Genetic susceptibility + environmental trigger activate autoreactive T lymphocytes
- T cells activate B cells to produce autoreactive antibodies
- Activated macrophages, T cells, antibodies, and complement cause inflammatory attack with demyelination
251
Describe Uhthoff’s Phenomenon.
Heat (eg. Hot shower, exercise) stresses the nerves and causes temporary decline in MS symptoms
252
Compare the presence of demyelination breakdown products in active and inactive MS.
ACTIVE
- Breakdown products present
INACTIVE
- Breakdown products absent
253
Compare the oligodendrocyte loss in active and inactive MS.
ACTIVE
- Variable oligodendrocyte loss
INACTIVE
- Variable oligodendrocyte loss
254
Compare the cell content of plaques in active and inactive MS.
ACTIVE
- Hypercellular plaque edge due to infiltration of tissue with inflammatory cells
INACTIVE
- Hypocellular plaque
255
Compare the inflammatory infiltrate in active and inactive MS.
ACTIVE
- Perivenous inflammatory infiltrate (mainly macrophages and T lymphocytes)
INACTIVE
- Variable inflammatory infiltrate
256
Compare blood-brain barrier disruption in active and inactive MS.
ACTIVE
- Extensive BBB disruption
INACTIVE
- Moderate to minor BBB disruption
257
Compare the gliosis of plaques in active and inactive MS.
ACTIVE
- Older plaques may have central gliosis
INACTIVE
- Plaques gliosed
258
Are most presentations of MS monosymptomatic or polysymptomatic?
Monosymptomatic
259
Describe the types of symptoms experienced in MS when the cerebral hemispheres are affected.
Large variety of symptoms, but also may be silent lesions.
260
Describe the types of symptoms experienced in MS when the spinal cord is affected.
- Weakness
- Paraplegia
- Spasticity
- Tingling
- Numbness
- Lhermitte’s sign
- Bladder and sexual dysfunction
261
What is Lhermitte’s sign?
Feeling of ‘electric shock’ down spine upon bending head forward.
262
Describe the types of symptoms experienced in MS when the optic nerve is affected.
- Impaired vision
| - Eye pain
263
Describe the types of symptoms experienced in MS when the medulla and pons are affected.
- Dysarthria
- Double vision
- Vertigo
- Nystagmus
264
Describe the types of symptoms experienced in MS when the cerebellar white matter is affected.
- Dysarthria
- Nystagmus
- Intention tremor
- Ataxia
265
What are the typical signs/symptoms of MS?
- Optic neuritis
- Other cranial nerve symptoms
- Spasticity
- Weakness
- Tremor
- Ataxia
- Sensory signs and symptoms (paraesthesiae, numbness)
- Lhermitte’s sign
- Nystagmus
- Double vision
- Vertigo
- Bladder/sexual dysfunction
- Cognitive problems and depression
- Fatigue
266
Give five symptoms that are not typical of MS.
- Aphasia
- Hemianopia
- Extrapyramidal movement disturbance
- Severe muscle wasting
- Muscle fasciculation
267
Describe relapsing/remitting MS.
Clearly defined disease relapses with full recovery or with sequelae and residual deficit upon recovery.
Periods between disease relapses characterised by a lack of disease progression.
268
Describe primary progressive MS.
Disease progression from the onset with occasional plateaus and temporary minor improvements allowed.
269
Describe secondary progressive MS.
Initial relapsing/remitting disease course followed by progression with or without occasional relapses, minor remissions, and plateaus.
270
Describe progressive/relapsing MS.
Progressive disease from onset, with some clear relapses, with or without full recovery, with periods between relapses characterised by continuing progression.
271
Give two essential diagnostic criteria of MS.
- Two or more CNS lesions disseminated in time or space
| - Exclusion of conditions giving a similar clinical picture
272
Give three tests that may aid in the diagnosis of MS.
- Evoked potentials
- MRI
- CSF electrophoresis
273
How might evoked potentials help to diagnose MS.
Speed of conduction may be slower.
274
What might the MRI scan show in MS?
Inflammation
275
What might be seen on CSF electrophoresis which suggests MS?
Thicker IgG band than on plasma electrophoresis, showing inflammation is contained to the CNS.
276
Give eight factors suggesting that suspected MS may not actually be MS.
- No objective neurological deficits
- No objective evidence for dissemination of lesions in time or space
- Strongly positive family history
- Progressive disease from outset in young patients
- No eye involvement
- Localised disease
- No CSF abnormalities
- Pain as the predominant symptom
277
Give seven differential diagnoses of MS.
- Autoimmune (SLE, Sjogrens)
- Infectious diseases (Lyme, syphilis, AIDS)
- Adrenomyeloceuropathy
- Mitochondrial encephalopathy
- Arnold-Chiari malformation
- Olivopontocerebellar atrophy
- Cardiac embolic event
278
What treatment can be used to shorten relapses in MS?
Methyprednisolone
279
How can interferons (betaferon) help in MS?
It decreases relapses and lesion accumulation on MRI.
280
Give three possible side effects of betaferon.
- Flu symptoms
- Depression
- Abortion
281
Describe two monoclonal antibodies that can be used in MS.
- Alemtuzumab against T lymphocytes
| - Natalizumab against receptors allowing immune cells across BBB
282
Give an immunosuppressive treatment used in MS.
Azathioprine
283
How can spasticity be treated in MS?
- Baclofen
- Diazepam
- Dantrolene
- Tizanidine
- Peripheral nerve blocks (botox)
- Surgery
284
How can tremor be treated in MS?
- Beta blockers
- Barbiturates
- Gabapentin
- Orthotic devices (wristbands with weights)
- Surgery
285
How can sexual dysfunction be treated in MS?
- Counselling
- Intracorporeal injection
- Penile prosthesis
- Lubricating gels
286
What is an epileptic seizure?
Paroxysmal event in which changes of behaviour, sensation, or cognitive processes are caused by excessive, hypersynchronous neuronal discharges in the brain.
287
What is syncope?
Paroxysmal event in which changes in behaviour, sensation, and cognitive processes are caused by an insufficient blood or oxygen supply to the brain. This can be related to the heart or low blood pressure.
288
What is a nonepileptic seizure?
Paroxysmal event in which changes in behaviour, sensation, and cognitive function caused by mental processes are associated with psychosocial distress.
289
Compare the durations of epileptic seizures, syncope, and non-epileptic seizures.
EPILEPTIC
30-120secs
SYNCOPE
5-30secs
NON-EPILEPTIC
1-20mins
290
Describe the ictal symptoms in an epileptic seizure.
Positive - feeling something that isn’t there
291
Describe the ictal symptoms in non-epileptic seizures.
- Ictal crying and speaking
- Eyes closed
- Dramatic motor phenomena or prolonged atonia
292
Describe the recovery in epileptic seizures, syncope, and non-epileptic seizures.
EPILEPTIC
- (Normally negative) postictal symptoms
SYNCOPE
- Recovery within 30 secs
NON-EPILEPTIC
- Surprisingly rapid or slow postictal recovery
293
Describe how one person’s seizures differ in epilepsy.
Seizures are normally always the same for that one individual.
294
Describe the movements experienced in syncope.
Can involve jerking, but duration much shorter than tonic-clonic seizures.
295
Which out of epileptic seizures, syncope, and non-epileptic seizures can occur from sleep?
Epileptic seizures
296
Which of epileptic seizures, syncope, and non-epileptic seizures are situational?
Syncope
| Non-epileptic seizure
297
Instead of sleep, where does syncope typically occur from?
Sitting or standing
298
Give six features of a seizure suggestive of epilepsy.
- Tongue biting
- Head turning
- Muscle pain
- Loss of consciousness >5mins
- Cyanosis
- Postictal confusion
299
Give four features of loss of consciousness/seizure suggestive of syncope.
- Prolonged upright position
- Sweating prior to loss of consciousness
- Nausea
- Presyncopal symptoms
300
Give some features of seizures suggestive of a non-epileptic seizure.
```
- Pelvic thrusting-
No ictal injury
- No seizures from sleep
- No incontinence
- No tongue biting
- Pre-ictal anxiety
- Unusually rapid or slow recovery
- Long duration
- Eyes closed
- Mouth closed during tonic-clonic movements
- No cyanosis
- Ictal crying/weeping
- Vocalisation during tonic-clonic phase
- Change in amplitude but no frequency of motor activity
```
301
What may cause focal epilepsy?
Localised brain abnormality (eg. Scarring from encephalitis or prolonged febrile seizure)
302
At what age does focal epilepsy typically start?
May start at any age
303
Give three types of seizures which occur in focal epilepsy.
- Simple partial seizure
- Complex partial seizure
- Secondary generalised seizure
304
Describe simple partial seizures.
Partial seizures without impairment of consciousness.
305
Describe complex partial seizures.
Partial seizures with impairment of consciousness.
306
Describe secondary generalised seizures.
Starts focally but spreads widely.
307
Describe the brain abnormality usually associated with generalised epilepsy.
No abnormality
308
At what age does generalised epilepsy usually present?
Childhood (<30yrs)
| *Can grow out of it
309
Give four types of generalised seizure.
- Absence seizure
- Myoclonic seizure
- Primary generalised tonic-clonic seizure
- Atonic (akinetic) seizure
310
Give two examples of epilepsy which feature absence seizures.
- Childhood absence epilepsy
| - Juvenile absence epilepsy
311
Describe absence seizures.
Stops taking mid-sentence (<10secs) then resumes.
312
Give an example of epilepsy which features myoclonic seizures.
Juvenile myoclonic epilepsy
313
Describe myoclonic seizures.
Sudden jerking of a limb/face/trunk.
314
Describe a tonic-clonic seizure.
- Tonic phase (limbs stiffen) followed by clonic phase (limbs jerk)
- Loss of consciousness
- Post-ictal confusion and drowsiness
315
Describe atonic seizures.
- Sudden loss of muscle tone causing a fall
| - No loss of consciousness
316
What proportion of people with epilepsy have normal brain scans?
7/10
317
When can epilepsy be diagnosed after only one seizure?
If a scan/EEG is suggestive of a high risk of other seizures.
318
Give 11 differential diagnoses of epilepsy.
- Postural syncope
- Dystonia
- TIA
- Cardiogenic syncope
- Migraine
- Hypoglycaemia
- Parasomnia
- Benign paroxysmal positional vertigo
- Cataplexy
- Hyperventilation
- Nonepileptic seizure
319
Give three potential treatments for focal epilepsy.
- Carbamazepine
- Lamotrigine
- Surgery
320
Give two possible treatments for generalised epilepsy.
- Valporate
| - Lamotrigine
321
How many different drugs are usually used to treat epilepsy?
Monotherapy is usually used but combination therapy can be tried.
322
What percentage of patients become seizure-free with anti-epileptic drugs?
70%
323
Give seven different mechanisms how anti-epileptic drugs can act.
- Block voltage gated Na channels
- Increase K channels
- Blocks Ca channels
- Blocks neurotransmitter release
- Targets GABA receptor
- Inhibits GABA degradation
- Inhibits GABA transport out of synapse
324
What are the common side effects of anti-epileptic drugs (valproate)?
VALPROATE
- Appetite increase/weight gain
- Liver failure
- Pancreatitis
- Reversible hair loss (grows back curly
- Oedema
- Ataxia
- Teratogenicity, tremor, thrombocytopenia
- Encephalopathy
325
Give four potential treatments for refractory epilepsy.
- Resective surgery
- Hemispherectomy
- Tractotomy
- Electrostimulation (vagus nerve stimulation)
326
Give some non-epileptic causes of seizures.
- Trauma
- Stroke
- Haemorrhage
- Raised intracranial pressure
- Alcohol/benzodiazepine withdrawal
- Hypoxia
- Hyper/hyponatraemia
- Hypocalcaemia
- Hyper/hypoglycaemia
- Uraemia
- Liver disease
- Infection
- High temperature
- Drugs
327
What is Todd’s palsy?
Transient paralysis after a frontal lobe seizure.
328
What are automatisms?
Complex motor phenomena with impaired awareness and no recollection.
Occur in temporal lobe seizures.
329
What is the embryological derivation of somatic motor and sensory nerves?
Somites
330
What is the embryological derivation of branchial motor nerves?
Pharyngeal arches
331
What spinal levels supply the autonomic nerve supply to the heart?
T1-T4
332
How many cervical spinal nerves are there?
8
333
Give two consequences of a trapped nerve.
- Numbness
| - Pain
334
At which vertebral level does the spinal cord end?
L1
335
How is the needle for an epidural different from a lumbar puncture needle?
Epidural needle is blunt so that it doesn’t penetrate the dura.
336
Which arteries branch to give the vertebral arteries?
Right and left subclavian arteries
337
Give three structures that the vertebral arteries give branches to as they ascend.
- Neck muscles
- Spinal meninges
- Spinal cord
338
Describe the course of the vertebral arteries.
- Enter foramina transversarium at C6
| - Ascend and enter foramen magnum anterolateral to medulla
339
Give three branches of the vertebral arteries before they join together.
- Anterior spinal artery
- Small medullary perforators
- Posterior inferior cerebellar artery
340
What artery is formed from the two vertebral arteries?
Basilar artery
341
Give four branches of the basilar artery.
- Perforating branches to brainstem
- Posterior cerebral artery
- Superior cerebellar artery
- Anterior inferior cerebellar artery
342
Describe the course of the posterior cerebral artery.
- Partially encircles midbrain
| - Supplies cortical territories
343
Give four structures that the posterior cerebral artery supplies as it encirles the midbrain.
- Thalamus
- Geniculate bodies
- Cerebral peduncles
- Tectum
344
What cortical territories does the posterior cerebral artery supply?
- Inferior temporal lobe
- Posterior 1/3 of interhemispheric surface
- Visual cortex and occipital lobe
345
At what vertebral level do the common carotid arteries bifurcate?
C3-C4
346
Name the four divisions of the internal carotid artery.
- Cervical
- Petrous
- Cavernous
- Supraclinoid
347
Describe the path of the cervical internal carotid arteries.
Ascend anterior and medial to internal jugular vein.
348
Describe the path of the petrous internal carotid arteries.
Penetrates temporal bone and runs horizontally (anteromedially) in carotid canal.
349
Describe the path of the cavernous internal carotid arteries.
Enter cavernous sinus, then pierces dura at the level of the anterior clinoid process.
350
Give three structures that are supplied by small branches of the cavernous internal carotid artery.
- Dura
- CN III-VI
- Posterior pituitary
351
Give three small branches of the supraclinoid internal carotid artery.
- Ophthalmic artery
- Superior hypophyseal artery
- Anterior choroidal artery
352
Give four structures supplied by the superior hypophyseal artery.
- Pituitary gland
- Stalk
- Hypothalamus
- Optic chiasm
353
Give five structures supplied by the anterior choroidal artery.
- Choroid plexus
- Optic tract
- Cerebral peduncle
- Internal capsule
- Medial temporal lobe
354
Give the three big branches of the internal carotid artery.
- Anterior cerebral artery
- Middle cerebral artery
- Posterior communicating artery
355
Describe the course of A1 of the anterior cerebral artery.
Runs medially to connect with the contralateral ACA via anterior communicating artery.
356
Describe the course of A2 of the anterior cerebral artery.
Runs in interhemispheric fissure to genu of corpus callosum and 2 cortical branches.
357
What does A3 of the anterior cerebral artery consist of?
Cortical branches
358
Describe the course of M1 of the middle cerebral artery.
Runs laterally to limen insulae.
359
What branches does M1 of the middle cerebral artery give off?
Lateral lenticulostriate arteries.
360
Give three structures supplied by the lateral lenticulostriate arteries.
- Lentiform nucleus
- Caudate nucleus
- Internal capsule
361
Describe the path of M2 of the middle cerebral artery.
Runs in insular cistern
362
Describe the path of M3 of the middle cerebral artery.
Emerges onto brain surface
363
What does M4 of the middle cerebral artery consist of?
Vessels on the brain surface.
364
What is the normal role of Tau in neurones?
It forms part of the cytoskeleton.
365
Describe how beta amyloid plaques are formed in Alzheimer’s disease.
Amyloid precursor protein is usually broken down by alpha and gamma secretase and waste products are soluble.
If alpha secretase is replaced by beta secretase the breakdown product is no longer soluble.
366
How are Lewy Bodies formed in Lewy Body dementia?
Misfolding of alpha synuclein protein.
367
Describe the compensatory response for a small increase in brain volume.
- Reduction in CSF volume
| - Little/no increase in pressure
368
Describe the brain’s response to a large increase in volume.
- Pressure increases exponentially
| - Ventricle outflow may become obstructed, leading to even bigger increase in pressure
369
Describe a herniation (duret) haemorrhage.
- Pontine arteries torn when the brain is pushed
- Haemorrhages seen in medulla/pons
- Usually fatal
370
Give three consequences of tonsillar herniation.
- Ataxia
- VI nerve palsy
- Upgoing plantar responses
- Compression of cardiorespiratory centre
371
Give four consequences of lateral tentorial (uncal) herniation.
- Oculomotor/parasympathetic nerve palsies
- Posterior cerebral artery infarction (homonymous hemianopia)
- Haemorrhage into midbrain or cardiorespiratory centre
- Motor weakness due to pressure on cerebral peduncles
372
What may be seen in a subfalcine (cingulate) hernia?
Ischaemic stroke of anterior cerebral artery.
373
Give three anatomical effects of mass lesions of the brain.
- Local deformity and shift of structures
- Decreased volume of CSF
- Pressure gradients lead to internal herniation
374
Give six signs of raised intracranial pressure.
- Decreased conciousness
- Focal neurological signs
- Papilloedema
- Increased blood pressure*
- Irregular breathing*
- Bradycardia*
* Cushing’s triad
375
How is raised intracranial pressure diagnosed?
- CT/MRI for mass lesions
| - As a last resort can measure opening pressure on lumbar puncture (However this is usually contraindicated)
376
What should be normal intracranial pressure be?
15mmHg
377
Give five possible treatments for raised intracranial pressure.
- Treat underlying cause
- External ventricular drain
- Decompressive craniotomy
- Osmotic therapy (mannitol)
- If intubated, hyperventilate to decrease CO2 (this causes cerebral vasoconstriction)
378
What does the external carotid artery supply?
- Skull
- Meninges
- Everything outside skull
379
What causes lateral medullary syndrome?
Embolus in the anterior inferior cerebellar artery or posterior inferior cerebellar artery.
380
Give four structures that become ischaemic in lateral medullary syndrome.
- CN VII
- CN VIII
- CN IX
- Middle cerebellar peduncle
381
Why are the lenticulostriate arteries prone to rupture?
They have a thin adventitia.
382
Does a haemorrhagic stroke usually kill grey or white matter, and what is the prognosis for recovery?
Usually kills white matter so there can be substantial recovery.
383
Does an embolic stroke usually kill white or grey matter and what is the prognosis for recovery?
Usually kills grey matter (cell bodies).
| No recovery.
384
Where are the meningeal vessels found?
Extradural space
385
In which layer of the meninges is the Circle of Willis found?
Subarachnoid space
386
Which blood vessels are found deep to the pia mater?
There are no vessels deep to the pia.
387
Which sensations pass through the thalamus?
All of them apart from olfaction.
388
What usually causes an extradural haemorrhage?
Skull fracture
389
Describe the pathology of an extradural haemorrhage.
Tearing of the middle meningeal artery causes blood to accumulate between bone and dura.
390
Describe the presentation of an extradural haemorrhage.
- Lucid interval (may be few hours to days) before deteriorating consciousness
- Headache
- Vomiting
- Confusion
- Seizures
- May be hemiparesis, brisk reflexes, upgoing plantar
391
Describe the appearance of an extradural haemorrhage on imaging.
Rounded convex shape
392
What is the management and prognosis for an extradural haemorrhage?
Good prognosis if transferred to neurology early.
393
Give four groups of people in who subdural haemorrhage is more common.
- Alcoholics
- Dementia
- Elderly
- Children
394
Describe the pathology of a subdural haemorrhage.
- Bleeding from bridging veins (low pressure, soon stops)
- Days/weeks later haematoma starts to autolyse
- Massive increase in oncotic and osmotic pressure sucks water into haematoma
- Gradual rise in ICP over many weeks
395
Describe the symptoms of a subdural haemorrhage.
- Fluctuating level of consciousness
- Insidious physical or intellectual slowing
- Sleepiness
- Headache
- Personality change
- Unsteadiness
396
Give three signs of a subdural haemorrhage.
- Raised intracranial pressure
- Seizures
- Focal neurological symptoms (often appear late)
397
Give two signs on imaging of a subdural haemorrhage.
- Midline shift
| - Crescent shape over one hemisphere
398
Give three management points for a subdural haemorrhage.
- Irrigation/evacuation
- Craniotomy
- Address causes of haemorrhage
399
What is the usual cause of a subarachnoid haemorrhage?
Berry aneurysms
400
Describe the pathology of a subarachnoid haemorrhage.
Rupture of arteries forming the circle of Willis
401
Describe the symptoms of a subarachnoid haemorrhage.
- Sudden (usually occipital) ‘thunderclap’ headache
- Photophobia
- Vomiting
- Collapse
- Seizures
- Coma
* May have experienced sentinel headache earlier due to small leak from offending vessel
402
Give four signs of a subarachnoid haemorrhage.
- Neck stiffness
- Kernig’s sign
- Retinal/subhyaloid/vitreous bleeds (Terson’s syndrome)
- Focal neurology
403
How is a subarachnoid haemorrhage diagnosed?
- CT detects >90% in first 48hrs
| - If CT-ve and no contraindications, do lumbar puncture
404
Describe the CSF in subarachnoid haemorrhage.
Bloody early on but then turns yellow due to breakdown products.
405
Describe the midline shift seen on imaging in subarachnoid haemorrhage.
No midline shift because bleeding spreads round both sides of the brain at the same time.
406
Describe the management of a subarachnoid haemorrhage.
- Refer to neurosurgery
- Observation
- Maintain cerebral perfusion (hydrate)
- Nimodipine (Ca channel blocker to reduce vasospasm)
- Endovascular coiling
407
What is the prognosis in subarachnoid haemorrhage?
- 1/3 die instantly from tonsillar herniation
- 1/3 become unconscious with high risk of mortality or permanent neurological deficit
- 1/3 have good outcome (if no rebleeding)
408
Give seven disorders of motor neurones.
- Motor neurone disease
- Hereditary spastic paraplegia
- Spinal muscular atrophy
- Kennedy’s disease
- Post-polio syndrome
- Motor neuropathies
- Hyperactivity disorders of motor neurones (eg. Stiff person syndrome)
409
Define paresis.
Impaired ability to move a body part at will (weakness).
410
Define paralysis.
Ability to move a body part in response to will is completely lost.
411
Define ataxia.
Willed movements are clumsy, ill-directioned, or uncontrolled (incoordination).
412
Define ‘involuntary movements’.
Spontaneous movement of a body part, independently of will.
413
Define apraxia.
Disorder of consciously organised patterns of movement or impaired ability to recall acquired motor skills.
Can’t put a series of movements together.
414
What does a motor unit consist of?
Lower motor neurone + the supplied muscle fibres.
415
Which motor neurones innervate muscle spindles?
Y motor neurones
416
What are four neural inputs to the final common motor pathway?
- Reflex arc
- Corticospinal/pyramidal pathway
- Extrapyramidal/basal ganglia system
- Cerebellum
417
Give the six ‘stations’ in the brain that the corticospinal tracts pass through.
- Motor cortex
- Corona radiata
- Internal capsule
- Cerebral peduncle in midbrain
- Pyramidal bundles in pons
- Pyramid in medulla
418
Give the clinical features of an upper motor neurone lesion.
- Increased muscle tone (spasticity)
- Brisk tendon reflexes
- Extensor plantar responses
- Upper limb extensors weaker than flexors
- Lower limb flexors weaker than extensors
- Fine skillful movements most severely impaired
- Emotional lability may be present
- Pyramidal drift
419
What is pyramidal drift?
When a patient with an UMN lesion puts arms out (supinated), one arm (contralateral) will pronate and drop.
420
Describe two features of an UMN lesion which suggests it may arise from the cortex.
- Contralateral weakness
| - Dispersion over wide area
421
Describe how an internal capsule lesion is likely to present.
Complete contralateral hemiparesis.
422
Give three features suggestive of a brainstem Motor neurone lesion.
- Often bilateral weakness (involvement of both corticospinal tracts)
- Involvement of cranial nerve nuclei
- Bulbar involvement often present
423
Which side would the weakness be on in a spinal cord motor neurone lesion?
Often bilateral lesions
424
What clinical feature suggests a cervical spinal cord motor neurone lesion?
Tetraparesis
425
What clinical feature suggests a spinal cord motor neurone lesion below the cervical region?
Paraparesis
426
Give five causes (And examples) of upper motor neurone lesions.
- Vascular (stroke)
- Inflammatory (MS)
- Compression/degeneration (tumour)
- Infiltration of corticospinal tract (tumour)
- Neurodegenerative disease of UMN +/- LMN (MND)
427
Give three tests (and what they look for) that can be used to assess UMN lesions.
- Neuroimaging (MRI)
- Blood tests (metabolic disease)
- CSF (oligoclonal bands)
428
Give the features of a lower motor neurone disorder.
- Normal/reduced muscle tone (flaccid)
- Muscle wasting
- Fasciculation
- Reflexes depressed/absent
429
Give six examples of diseases which can cause a lower motor neurone lesion in the Brainstem/spinal cord.
- Motor neurone disease
- Spinal muscular atrophy
- Poliomyelitis
- Syringomyelia/syringobulbia
- Spinal cord/brainstem compression
- Vascular disease
430
Give four causes of LMN lesions in the spinal roots.
- Prolapsed intervertebral disk
- Cervical/lumbar spondylosis
- Tumours
- Malignant infiltration
431
Give two causes of peripheral nerve LMN lesions.
- Axonal degeneration
| - Demyelinating
432
Give three examples of LMN diseases affecting the neuromuscular junction.
- Myaesthenia gravis
- Lambert Eaton myasthenic syndrome
- Congenital diseases
433
Give two features Of the pattern of disease that can help distinguish between a LMN and a muscle disease.
- Nerve affects distal muscles first and reflexes are lost
| - Muscle affects proximal limb and trunk muscles, and reflexes are preserved until late
434
Give four investigations (and what they’re looking for) to help assess LMN disorders.
- Neurophysiology nerve conduction studies/EMG
- Neuroimaging (MRI)
- Blood tests (muscle enzymes, autoantibodies)
- Lumbar puncture
435
What percentage of malignant childhood tumours do brain tumours account for?
20%
436
Where are the majority of adult brain tumours found?
Supratentorial
437
Where are the majority of childhood brain tumours found?
Posterior fossa
438
Give two groups of people who are more likely to get CNS lymphoma.
- Elderly
| - Immunosuppressed
439
Give a group of patients in who germ cell tumours are more common.
Children
440
What percentage of brain tumours are malignant?
55%
441
Where are brain tumours likely to spread?
They NEVER spread outside the CNS
442
Give three potential risk factors for developing a brain tumour.
- Ionising radiation (especially in childhood)
- Family history (sometimes)
- Immunosuppression (CNS lymphoma)
443
Give six groups of brain tumours according to the WHO classification.
- Neuroepithelial tumours (gliomas)
- Cranial and spinal nerve tumours
- Meningeal tumours
- Lymphomas
- Germ cell tumours
- Metastatic tumours
444
Which cells form gliomas?
Neurones or glial cells
445
Which is the largest group of brain tumours?
Neuroepithalial tumours (gliomas)
446
Give seven types of gliomas.
| Which is the most common?
- Astrocytic (most common)
- Oligodendroglial
- Ependymal
- Neuronal and neuro-glial
- Pineal
- Embryonal
- Choroid plexus
447
Give four general clinical manifestations of a brain tumour.
- Loss of function (focal neurological symptoms)
- Non-focal neurological symptoms
- Seizures
- Headache
448
What is the ‘most predictive’ symptom of a brain tumour?
Seizures
449
Give four examples of focal neurological symptoms that may occur with a brain tumour.
- Weakness
- Sensory loss
- Visual/speech disturbance
- Ataxia
450
Describe the rate of onset of focal neurological symptoms associated with a brain tumour.
Usually come on over a few weeks or days
451
Give three examples of non-focal neurological symptoms that can occur in a brain tumour.
- Personality/behaviour change
- Memory disturbance
- Confusion
452
Why do people with brain tumours get headaches?
From raised intracranial pressure
453
Describe the features of the headache associated with raised intracranial pressures/brain tumours.
- Woken by headache
- Worse in morning and when lying down
- Associated with nausea and vomiting
- Exacerbated by coughing/sneezing
- Associated with drowsiness
454
Give two signs of a brain tumour.
- Papilloedema
| - Focal neurological deficit
455
Give four focal neurological deficit signs that can occur in association with brain tumours.
- Hemiparesis
- Hemisensory loss
- Visual field defect
- Dysphasia
456
Give four red flags for brain tumours.
- Headache (+ features of raised ICP/focal neurology)
- New onset focal seizure
- Rapidly progressing focal neurology
- Past history of other cancer
457
How do low grade brain tumours typically present?
Seizures, but can be incidental finding.
458
How does the brain adapt in low grade brain tumours?
Because the tumour is slow growing the brain can move out of the way so important structures aren’t affected.
459
How do high grade brain tumours typically present?
- Rapidly progressive neurological deficit
| - Symptoms of raised ICP
460
What investigations should be carried out to diagnose a brain tumour?
- Contrast CT/MRI (MRI is better)
| - Brain biopsy
461
How will the brain scan appear in a high grade brain tumour?
- Lots of oedema
| - Lights up with contrast dye
462
What is the five year survival in brain tumours?
19%
463
Describe the treatment for high grade gliomas.
- Steroids reduce oedema
- Surgery (resection, relief of raised ICP)
- Radiotherapy (mainstay)
- Chemotherapy
464
Give two chemotherapy options for brain cancer.
- Temozolamide
| - PCV
465
Describe the treatment options for low grade gliomas.
- Surgery (resection)
| - Radiotherapy + chemotherapy
466
How does radiotherapy affect the prognosis of low grade gliomas?
Delays disease progression but doesn’t improve survival.
467
What age do astrocytic tumours usually occur in?
Adults
468
Name a WHO grade I astrocytic tumour.
Pilocytic astrocytoma
469
Name a WHO grade II astrocytic tumour.
Diffuse astrocytoma
470
Name a WHO grade III astrocytic tumour.
Anaplastic astrocytoma
471
Name a WHO grade IV astrocytic tumour.
Glioblastoma
472
Who is more likely to get a primary (de novo) glioblastoma.
Older people
473
Describe a secondary glioblastoma.
Progresses from a lower grade astrocytoma.
474
Describe the behaviour of diffuse astrocytomas.
- Infiltrate diffusely
| - Undergo progressive anaplasia (progress from low to high grade)
475
Describe the isocitrate dehydrogenase status in grade II/III and glioblastoma de novo astrocytomas.
- Grade II/III = isocitrate dehydrogenase 1 mutation
| - Glioblastoma de novo = wild type IDH1
476
Describe the prognosis for a pilocytic astrocytoma.
Good
477
Describe the histological criteria for a diffuse astrocytoma.
Nuclear atypia
478
Describe the prognosis for a diffuse astrocytoma.
>5yrs
479
Describe the histological criteria for an anaplastic astrocytoma.
- Nuclear atypia
| - Mitoses
480
Describe the prognosis for an anaplastic astrocytoma.
2-5yrs
481
Describe the histological criteria for a glioblastoma.
- Nuclear atypia
- Mitoses
- Necrosis + vascular proliferation
482
Describe the prognosis for a glioblastoma.
<1yr
483
What is MGMT?
An enzyme which repairs DNA damage.
484
Describe how the MGMT methylation status in astrocytomas affect the prognosis and treatment.
If MGMT is methylated there is a good response to chemotherapy.
485
Who do pilocytic astrocytomas usually occur in?
Children
486
Will pilocytic astrocytomas usually progress to glioblastomas?
No - they are their own entity
487
Where do pilocytic astrocytomas usually occur?
Posterior fossa
488
Describe the appearance of a pilocytic astrocytoma.
Often cystic
489
What are rosenthal fibres, and what do they indicate when seen in a brain tumour?
Protein aggregated often seen in grade I lesions, therefore indicating a good prognosis.
490
At what age are oligodendrogliomas most common?
4th/5th decade
491
Describe the common clinical presentation of oligodendrogliomas.
May have seizures
492
What WHO grade are oligodendrogliomas?
WHO grade II
493
Describe the appearance of the anaplastic variant of oligodendrogliomas.
They have mitotic figures and microvascular proliferation.
494
What feature on a scan can help to diagnose oligodendrogliomas?
Calcification
495
Give two molecular criteria for an oligodendroglioma to be diagnosed.
- Mutation in isocitrate dehydrogenase 1
| - 1p19q co-deletion
496
At what age do medulloblastomas occur?
Childhood
497
Describe the appearance of a medulloblastoma.
- Primitive ‘small blue cell’ tumour
- Densely cellular
- Embryonal tumour
498
Where do medulloblastomas occur?
Cerebellum
499
Describe the prognosis/treatment for medulloblastomas.
- Highly malignant but may respond to excision/radiotherapy/chemotherapy
- Potentially curable
500
What is the issue with treating medulloblastomas with chemo/radiotherapy?
The brain is still developing.
501
What is the risk of the medulloblastoma if the tumour is desmoplastic (connective tissue is present)?
Lower risk
502
What is the risk of a medulloblastoma if the tumour is large cell anaplastic?
Higher risk
503
Give two mutations that give a better prognosis in medulloblastomas.
- WNT
| - SHH
504
How is a WNT mutation identified in a medulloblastoma?
Presence of nuclear b-catenin protein
505
Describe the activity of meningioma.
Dural based and push into brain.
506
What grade are most meningiomas?
```
Grade I
(There are more aggressive variants (Grade II/III))
```
507
Describe the appearance of brain metastases on a scan.
- Tend to be rounded
| - Likely to be multiple tumours, but solitary tumours can also be mets
508
Give five cancers that are likely to spread to the brain.
- Lung
- Breast
- Melanoma
- GI tract
- Kidney
509
Name three ‘movement disorders’.
- Parkinson’s disease
- Essential tremor
- Dystonia
510
Give two movement disorders that are due to ‘hardware problems’ (AKA cell death) in the basal ganglia.
- Parkinson’s disease
| - Huntington’s disease
511
Give two diseases that are due to ‘software problems’ (AKA no cell death) in the basal ganglia.
- Dystonia
| - Tourette
512
What percentage of people over 65 years will have Parkinson’s disease?
3%
513
What causes Parkinson’s disease?
Genetic and environmental factors.
514
Describe the pathology of Parkinson’s disease.
- Loss of dopaminergic neurones in the substantia nigra
| - Presence of intracytoplasmic Lewy bodies in substantia nigra
515
What methods cause cell loss in Parkinson’s disease?
- Mitochondrial dysfunction
| - Oxidative stress
516
Give the three cardinal features of Parkinson’s disease/Parkinsonism.
- Bradykinesia/Akinesia
- Resting tremor
- Rigidity
517
Describe the symptoms/signs of Parkinson’s disease.
- Shuffling
- Stooped gait
- Drag (usually one) leg while walking
- Problems with intricate movements
- Writing smaller
- Resting tremor (may be unilateral)
- Stiffness (increased tone)
- Pain (not usually a major presenting feature)
- Problems with turning in bed
- Decreasing amplitude/accuracy of repetitive movements (much better at beginning, gradual worsening)
518
Describe the symmetry/asymmetry of symptoms in Parkinson’s disease.
Symptoms always start (or are much worse) on one side.
519
Give four features that are more suggestive of pressure hydrocephalus and should not be present in Parkinson’s disease (at least at the start).
- Incontinence
- Dementia
- Symmetry
- Early falls
520
Describe how a diagnosis of Parkinson’s disease is made.
- Usually clinical
| - Can do DaTSCAN
521
What would a DaTSCAN show in Parkinson’s disease?
Reduced dopamine supply to the striatum.
522
What is the aim of treatment in Parkinson’s disease?
Compensate for loss of dopamine (treat symptoms)
523
Give two lifestyle treatments that may help Parkinson’s disease.
- Maintain healthy weight
| - Exercise as much as possible
524
Give four groups of medications that can be used to treat Parkinson’s disease.
- L-dopa
- Dopamine agonists
- Catachol-O-Methyl-Transferase (COMT) inhibitors
- Monoamine oxidase (MAO) inhibitors
525
Which is the most powerful drug class used in Parkinson’s disease?
L-dopa
526
What is the first line drug treatment for Parkinson’s disease in younger patients?
Dopamine agonists
527
Give three possible side effects of dopamine agonists.
- Tiredness
- Gambling
- Hypersexuality
528
Give seven examples of dopamine agonists.
- Ropinirole
- Pramipexole
- Rotigotine
- Bromocriptine
- Pergolide
- Cabergoline
- Amantadine
529
Give two examples of COMT inhibitors used in Parkinson’s disease.
- Entacapone
| - Tolcapone
530
Which class of Parkinson’s drugs has the best neuroprotective effect?
MOA inhibitors
531
What is the downside of using MAO inhibitors to treat Parkinson’s disease?
They aren’t that powerful
532
Give two examples of MAO inhibitors used to treat Parkinson’s disease.
- Rasagiline
| - Selegiline
533
Why aren’t anticholinergics used to treat Parkinson’s disease?
They have side effects
534
Give three side effects of anti-cholinergic drugs.
- Cognition
- Confusion
- Systemic
535
When should treatment for Parkinson’s disease be started?
Doesn’t have to be straight away - discuss with patient.
536
Give four drug complications of long-lasting Parkinson’s disease.
- Wearing off (drug doesn’t work as long)
- On-dyskinesias (when drugs work)
- Off-dyskinesias (when drugs don’t work)
- Freezing (unpredictable loss of mobility)
537
Give five complications that may develop in Parkinson’s disease
- Depression
- Psychotic problems (phobias, anxiety disorder, hallucinations)
- Dementia
- Autonomic problems (constipation, increased urinary frequency)
- Anosmia
538
Describe the prognosis in Parkinson’s disease.
Disease is slowly progressive
539
Describe the clinical features of essential tremor.
- Postural and intention tremor
- Gradual worsening
- No increased tone or problems with fine finger movements
540
Give four potential treatments for essential tremor.
- Beta blocker
- Primidone
- Gabapentin
- Clonazepam
541
What is generalised dystonia?
Syndrome of sustained muscle contraction, frequently causing twisting and repetitive movements as well as abnormal posture.
542
Describe the typical history/onset of generalised dystonia.
- Often positive family history
| - Typical onset in childhood
543
What is motor neurone disease?
A group of neurodegenerative diseases characterised by the selective loss of motor neurones in the motor cortex, cranial nerve nuclei, and anterior horn cells.
544
Are upper or lower motor neurones affected in motor neurone disease?
Both
545
Is motor neurone disease more common in men or women?
Slightly more common in men
546
At what age do most cases of motor neurone disease present?
50-70yrs
547
What are most cases of motor neurone disease caused by?
Idiopathic
548
Give three genes that may be implicated in some cases of motor neurone disease.
- SOD1
- TDP-43
- FUS
549
Give a macroscopic change that may occur in motor neurone disease.
Atrophic anterior roots of spinal cord.
550
What microscopic change may be seen in motor neurone disease?
Selective loss of motor neurones in the motor cortex and anterior horns of the spinal cord.
551
Give the four clinical patterns of motor neurone disease.
- Amyotrophic lateral sclerosis
- Progressive bulbar palsy
- Progressive muscular atrophy
- Primary lateral sclerosis
552
Describe the pathology of amyotrophic lateral sclerosis.
Loss of motor neurones in the motor cortex and anterior horn of spinal cord.
553
Describe the pattern of signs/symptoms in ALS.
UMN and LMN signs
554
Describe split hand sign (and when it may occur).
Thumb side of hand seems cast adrift owing to excessive wasting around it (much less hypothenar wasting).
Seen in ALS.
555
What is the most common clinical pattern of MND?
ALS
556
Describe the pathology of progressive bulbar palsy.
MND only affecting cranial nerves IX-XII
557
Describe the clinical presentation of progressive bulbar palsy.
LMN signs in tongue and muscles of talking/swallowing.
558
Describe the pathology in corticobulbar palsy.
UMN lesion of muscles of swallowing and talking due to bilateral lesions above mid pons.
559
Is bulbar or corticobulbar palsy more common?
Corticobulbar palsy
560
Give four signs/symptoms of corticobulbar palsy.
- Slow tongue movements
- Slow deliberate speech
- Increased jaw jerk
- Increased pharyngeal/palatal reflexes
561
Describe the pathology in progressive muscular atrophy.
MND with lesions in the anterior horn cells only.
562
Describe the UMN signs in progressive muscular atrophy.
Absent
563
Which muscle groups are affected first in progressive muscular atrophy.
Distal muscle groups
564
Compare the prognosis for progressive muscular atrophy with ALS.
Better prognosis for progressive muscular atrophy.
565
Describe the pathology in primary lateral sclerosis.
Loss of Betz cells in the motor cortex.
566
Describe the pattern of signs/symptoms in primary lateral sclerosis.
Mainly UMN signs
567
Give three signs of primary lateral sclerosis.
- Marked spastic leg weakness
- Pseudobulbar palsy
- No cognitive decline
568
Describe the signs of MND in the limb muscles.
- Weakness
- Wasting
- Fasciculation
- Spasticity
569
Describe the symmetry/asymmetry of signs in MND.
Asymmetric
570
Give five signs of MND affecting the face.
Difficulty:
- Swallowing
- Chewing
- Speaking
- Coughing
- Breathing
571
Do cognitive changes occur in MND?
Potentially
572
Give a feature that can distinguish MND from MS/polyneuropathies.
No sensory loss/sphincter disturbance.
573
Give a feature that can distinguish MND from Myaesthenia gravis.
MND never affects eye movements.
574
What test is carried out to diagnose MND?
There is no diagnostic test
575
Why can a brain/cord MRI be helpful when diagnosing MND?
To exclude structural causes
576
Why can a lumbar puncture be useful when diagnosing MND?
To exclude inflammatory causes
577
Why can neurophysiology tests be useful when diagnosing MND?
They can detect subclinical denervation.
578
What is the diagnostic criteria for definite MND?
Lower + upper motor neurone signs in 3 regions
579
What is the diagnostic criteria for probably MND?
Lower + upper motor neurone signs in 2 regions
580
What is the diagnostic criteria for probable (+ lab support) MND?
Lower and upper motor neurone signs in 1 region with EMG showing acute denervation in 2 or more limbs
OR
Upper motor neurone signs in one or more regions with EMG showing acute denervation in 2 or more limbs
581
What is the diagnostic criteria for possible MND?
Lower + upper motor neurone signs in 1 region.
582
What is the diagnostic criteria for suspected MND?
Upper or lower motor neurone signs only - in 1 or more regions.
583
Give an antiglutaminergic drug that can help to treat MND.
Riluzole
584
How can antiglutaminergic drugs help MND?
Prolong life by about 3 months
585
Give 6 side effects of anti-glutaminergic drugs.
- Raised LFTs
- Vomiting
- Fast pulse
- Somnolence
- Headache
- Vertigo
586
Give two possible treatments for drooling in MND.
- Propantheline
| - Amitiptyline
587
Give three possible treatments for dysphagia in MND.
- Blend food
- NG tube
- Percutaneous catheter gastrostomy
588
Give a treatment for joint pains/distress in MND.
Analgesia
589
Give a treatment for respiratory failure in MND.
Non-invasive ventilation
590
How long do people with MND usually survive?
2-4yrs
591
What is the most common cause of death in MND?
Aspiration pneumonia
592
What is Huntington’s disease?
An inherited neurodegenerative disorder caused by a mutation of the HTT gene.
593
At what age does Huntington’s disease present?
Most present between 35-45yrs but can occur at any age.
594
Is Huntington’s disease more common in men or women?
Equal
595
Describe the inheritance pattern in Huntington’s disease.
Autosomal dominant
596
Describe the genetic element of Huntington’s disease.
- HTT contains a sequence of CAG trinucleotide repeats
- Usually <36 repeats
- Mutant HTT has >36 repeats
597
Describe how Huntington’s disease changes with increasing number of trinucleotide repeats.
- Fuller penetrance
| - Younger age of onset
598
Describe how the number of trinucleotide repeats changes between generations in Huntington’s disease.
Tends to get larger (anticipation)
599
What protein does HTT code for?
Huntingtin
600
In which cells is Huntingtin expressed?
All cells, but more in brain and testis.
601
How does mutated Huntingtin cause Huntington’s disease?
Mutated Huntingtin thought to be cytotoxic to certain cell types, most notably neurones in the caudate nucleus and putamen.
602
Give the three cardinal features of Huntington’s disease.
- Chorea
- Dementia
- Psychiatric problems
603
Describe the presentation (and how this changes over time) of Huntington’s disease.
- Irritability
- Depression
- Incoordination
- Chorea
- Over time, there is motor, neuropsychiatric, and cognitive decline
- Will eventually develop dementia
604
What is chorea?
Uncontrolled, random jerky movements
605
Give three psychiatric problems experienced in Huntington’s disease.
- Personality change
- Depression
- Psychosis
606
Give four signs of Huntington’s disease.
- Abnormal eye movements (problems initiating saccades, broken pursuit)
- Chorea
- Ataxia (problems with heel to toe walking)
- Often have a touch of Parkinsonism
607
Give two macroscopic features found in Huntington’s disease.
- Atrophy of caudate nucleus and putamen
| - Cortical atrophy may be present
608
Give two microscopic features that may appear in Huntington’s disease.
- Marked neuronal loss from caudate nucleus
| - Surviving neurones contain abundant amounts of Huntingtin protein
609
How is chorea treated in Huntington’s disease?
Neuroleptic (sulpiride)
610
How is depression treated in Huntington’s disease?
Selective serotonin reuptake inhibitor (seroxate)
611
How is psychosis treated in Huntington’s disease?
Neuroleptic (haloperidol)
612
How is aggression treated in Huntington’s disease?
Risperidone
613
What is the average survival time from the onset of symptoms in Huntington’s disease?
20yrs
614
Give two common causes of death in Huntington’s disease.
- Pneumonia
| - Cardiac failure
615
Why does cardiac failure occur in Huntington’s disease?
Abnormal Huntingtin expressed in cardiac muscle
616
What are neuroleptic drugs?
Antipsychotics which depress nerve functioning.
617
Give seven causes of spinal cord compression.
| Which is the most common?
- Secondary malignancy in spine (most common)
- Infection (epidural abscess)
- Cervical disk prolapse
- Haematoma (Warfarin)
- Intrinsic cord tumour
- Atlanto-axial subluxation
- Myeloma
618
Describe the symptoms of spinal cord compression.
- Weak legs
- Spinal or root pain
- Arm weakness
- Bladder and anal sphincter involvement
619
Compare the arm and leg weakness in spinal cord compression.
Arm weakness is usually less severe than leg weakness
620
What does arm weakness indicate in spinal cord compression?
Cervical lesion
621
At what stage of spinal cord compression is there bladder and anal sphincter involvement?
Late
622
How does bladder and anal sphincter involvement manifest in spinal cord compression?
- Hesitancy
- Frequency
- Painless retention
623
Describe the leg weakness in spinal cord compression.
Spastic and hyperreflexic
624
Describe the clinical examination findings above the level of the lesion in spinal cord compression.
Normal
625
Describe the clinical examination findings of spinal cord compression at the level of the lesion.
Lower motor neurone signs
626
Describe the clinical examination findings of spinal cord compression below the level of the lesion.
Upper motor neurone signs
627
What is the definitive imaging modality to evaluate spinal cord compression?
MRI
628
How is the nature of a mass causing a spinal cord compression investigated?
Biopsy or surgical exploration
629
Give eight blood investigations that should be carried out in spinal cord compression.
- FBC
- ESR
- B12
- Syphilis
- U and E
- LFT
- PSA
- Serum electrophoresis
630
Give another image/scan that must be carried out in spinal cord compression and explain why.
CXR to look for lung malignancy/TB
631
What treatment should be carried out for cord compression caused by a malignancy, while considering more specific chemo/radiotherapy?
Dexamethosone
632
Give a method of relieving spinal cord compression.
Decompressive laminectomy
633
How should epidural abscesses be treated in spinal cord compression?
Surgical decompression and antibiotics
634
Give eight differential diagnoses of spinal cord compression.
- Transverse myelitis
- MS
- Carcinomatous meningitis
- Cord vasculitis
- Spinal artery thrombosis
- Trauma
- Dissecting aneurysm
- Guillain-Barre
635
Give five causes of unilateral foot drop.
- Diabetes mellitus
- Common peroneal nerve palsy
- Stroke
- Prolapsed disk
- MS
636
Give three causes of weak legs with no sensory loss.
- Motor neurone disease
- Polio
- Parasagittal meningioma
637
Give ten causes of chronic spastic paraparesis.
- MS
- Cord tumour
- Cord metastases
- Motor neurone disease
- Syringomyelia
- Subacute combined degeneration of the spinal cord
- Hereditary spastic paraparesis
- Taboparesis
- Histiocytosis X
- Parasites (Schistosomiasis)
638
Give two causes of chronic flaccid paraparesis.
- Peripheral neuropathy
| - Myopathy
639
Give three causes of absent knee jerks and extensor plantars.
- Combined cervical and lumbar disk disease
- Conus medullaris lesions
- MAST (MND, ataxia, Subacute combined degeneration, Taboparesis)
640
Describe the leg weakness in cauda equina and conus medullaris lesions.
Flaccid and areflexic
641
Give the signs/symptoms of a conus medullaris lesion.
- Mixed UMN/LMN leg weakness
- Early urinary retention and constipation
- Back pain
- Sacral sensory disturbance
- Erectile dysfunction
642
Describe the signs/symptoms of cauda equina lesions.
- Back pain
- Radicular pain down legs
- Asymmetrical, atrophic, areflexic paralysis of the legs
- Sensory loss in a root distribution
- Decreased sphincter tone
643
Give four things to address when taking care of a paralysed patient.
- Turn to avoid pressure sores
- Anticoagulant, compression stockings, movement to avoid DVT
- Bladder care (eg. Catheter)
- Bowel evacuation may be needed
644
What is myasthenia gravis?
An organ-specific autoimmune disease caused by the production of autoantibodies directed against the nicotinic acetylcholine receptor (nAChR) at the neuromuscular junction.
645
At what age is myasthenia gravis more common in women?
<50yrs
646
At what age is myasthenia gravis more common in males?
>50yrs
647
What percentage of patients with myasthenia gravis have a thymus abnormality?
75%
648
What percentage of patients with myasthenia gravis have a thymoma?
5-10%
649
Describe the pathology of myasthenia gravis.
Autoantibodies binding to the nAChR limit depolarisation at the motor end plate and therefore impair muscle contraction.
650
What are the symptoms of myasthenia gravis?
Increasing muscular fatigue
651
What muscles are affected in myasthenia gravis (in order of appearance of symptoms)?
- Extraocular
- Bulbar
- Face
- Neck
- Limb girdle
- Trunk
652
What are the signs of myasthenia gravis?
- Ptosis
- Diplopia (complex ophthalmoplegia)
- Myasthenic snarl on smiling
- Dysarthria
- Head drop
- Limb fatigability
- Peek sign
- Voice fading
- Normal tendon reflexes
- Ice pack test (ptosis improves after application of ice pack)
- Cogan’s lid twitch
653
Give thirteen conditions/things that can worsen weakness in myasthenia gravis.
- Pregnancy
- Hypokalaemia
- Infection
- Over-treatment
- Change of climate
- Emotion
- Exercise
- Gentamicin
- Opiates
- Tetracycline
- Quinine
- Procainamide
- Beta blockers
654
Give three investigations that should be carried out in myasthenia gravis.
- Antibodies
- Neurophysiology
- Imaging
655
If the antibody test for AChR antibody is negative in myasthenia gravis, what other antibody should be looked for?
Anti-MuSK
656
What does MuSK stand for?
Muscle specific tyrosine kinase
657
What are MuSK and LRP4?
Proteins which support the acetylcholine receptor complex.
| Can have antibodies against these in myasthenia gravis.
658
What would be seen in neurophysiology tests in myasthenia gravis?
Decremental muscle response to repetitive electrical nerve stimulation.
659
What imaging should be carried out in myasthenia gravis?
CT of thymus
660
What age does ocular myasthenia gravis affect?
Any age
661
Is ocular myasthenia gravis more common in males or females?
Males
662
Describe the prognosis in ocular myasthenia gravis.
Most progress to generalised MG within a year.
663
What percentage of patients with ocular myasthenia gravis have anti-AChR antibody?
50%
664
How do most people with late-onset myasthenia gravis present?
Ocular MG
665
Describe the prognosis in late-onset myasthenia gravis.
Becomes generalised after 2-3yrs
666
Describe the thymus in Anti-MuSK myasthenia gravis.
Normal
667
Describe the tongue, cheek, and orbit muscles in Anti-MuSK myasthenia gravis.
Fatty change occurs
668
Describe the onset of anti-MuSK myasthenia gravis.
- 36% have ocular onset
| - 26% have oculo-bulbar onset
669
What is congenital myasthenic syndrome?
Mutations in ColQ, DOK7, or CHRNE which produce a myasthenia gravis-like picture.
670
What can be used to control symptoms in myasthenia gravis?
Acetylcholineesterase inhibitor (pyridostigmine)
671
Give seven side effects of pyridostigmine.
- Increased salivation
- Lacrimation
- Sweats
- Vomiting
- Miosis (excessive pupil constriction)
- Diarrhoea
- Colic
672
How can relapses of myasthenia gravis be treated?
Prednisolone
673
Give a steroid sparing agent used to treat myasthenia gravis.
Azathioprine
674
Give a type of surgery which can be used to treat myasthenia gravis, and when it would be used.
Thymectomy
| - Consider if onset <50yrs and not easily controlled by acetylcholineesterase inhibitors, and they are sero-positive
675
How is Anti-MuSK myasthenia gravis treated?
- Poor response to pyridostigmine
| - Better response to rituximab
676
Give five lifestyle changes/factors to help myasthenia gravis.
- Avoid exacerbating drugs
- Follow up in clinic
- Exercise is safe
- Encourage normal pregnancy
- Avoid live vaccines
677
Describe the prognosis in myasthenia gravis.
Relapsing, but not progressive symptoms.
678
What is a myasthenic crisis?
Weakness of respiratory muscles during a relapse.
679
How is a myasthenic crisis treated?
Plasmapheresis or IV Ig
680
Give four differential diagnoses of myasthenia gravis.
- Poliomyelitis
- SLE
- Takayasu’s arteritis
- Botulism
681
What is complex ophthalmoplegia?
Eye movements are reduced in all direction, and patient has double vision.
682
Give six differential diagnoses for complex ophthalmoplegia.
- Myasthenia gravis
- Graves disease
- Botulism toxin
- Miller Fisher (variant of Guillan Barre)
- Mitochondrial chronic progressive external ophthalmoplegia (CPEO)
- Brainstem stroke
683
Describe partial ptosis.
Eyelid drooping but still above the pupil.
684
Describe complete ptosis.
Eyelid covers the pupil.
685
Describe the criteria for exophthalmos.
Bottom eyelid below inferior limbus.
686
Describe the criteria for lid retraction.
Top eyelid above superior limbus.
687
Describe how muscle strength changes in Lambert-Eaton Myasthenic Syndrome.
Movements become stronger.
688
Describe the presentation of Lambert-Eaton Myasthenic Syndrome.
- Dry eyes and mouth
- Limb weakness
- Erectile dysfunction
689
Describe the pathology in Lambert-Eaton Myasthenic Syndrome.
Usually associated with antibodies to voltage gated calcium channels in the presynaptic terminal.
690
Give two causes (and their frequencies) of Lambert-Eaton Myasthenic Syndrome.
- Autoimmune (50%)
| - Paraneoplastic (50%)
691
What cancer usually causes Lambert-Eaton Myasthenic Syndrome?
Small cell lung carcinoma
692
What investigation can diagnose Lambert-Eaton Myasthenic Syndrome?
Incremental potential on repetitive stimulation of EMG.
693
How is Lambert-Eaton Myasthenic Syndrome treated?
- 3,4-diaminopyridine
| - Immunosuppression
694
What is the difference between dysarthria and dysphasia?
DYSARTHRIA
- Problem with speech and pronunciation (not language)
DYSPHASIA
- Problem with understanding and producing language
695
What is the main role of the cerebellum?
Control the timing and pattern (coordination) of motor activation during movement.
696
Give six causes of progressive ataxias (in order of prevalence).
- Familial/genetic
- Gluten ataxia (coeliac disease)
- Idiopathic
- Alcohol
- Degenerative (multi-system atrophy)
- Paraneoplastic
697
What is the most common familial/genetic ataxia?
Friedreich’s ataxia
698
Give three features of ataxia which may suggest it is paraneoplastic.
- Rapid onset
- Fast progression
- Associated with oscillopsia
699
What is the most likely cause of sporadic idiopathic ataxia?
Autoimmune
700
Give three features of sporadic idiopathic ataxia which lead to the conclusion that it is likely to be autoimmune.
- 47% have other autoimmune conditions
- 71% have HLA DQ2
- 60% have anti-cerebellar antibodies
701
Give an autoantibody which has been linked to cerebellar ataxia.
Name another autoimmune disease in which this antibody has been implicated.
Anti-GAD
| Also appears in T1DM.
702
Describe the macroscopic changes to the cerebellum in autoimmune ataxia.
Swells at first and then becomes atrophic.
703
Describe the symptoms of cerebellar ataxia.
- Slurring/staccato speech
- Swallowing difficulties
- Oscillopsia
- Clumsiness
- Intention tremor
- Unsteadiness when walking
- Falls
- Cognitive abnormalities
704
Describe oscillopsia.
- Patient experiences blurred vision, and objects in the visual field appear to oscillate
- Other people see involuntary eye movement of both eyes in random directions
705
Describe the signs of cerebellar ataxia.
- Dysarthria
- Nystagmus
- Limb ataxia
- Intention tremor
- Truncal ataxia
- Gait ataxia
- Oscillopsia
706
What is it called when damage to the cerebellum leads to cognitive abnormalities?
Cerebellar cognitive affective syndrome
707
Give four cognitive processes that can be abnormal in cerebellar cognitive affective syndrome.
- Sequencing
- Language
- Executive function
- Visuospatial abilities
708
Give the four classifications of ataxia.
- Congenital
- Diseases where ataxia is one of the many features
- Familial ataxia (divided into autosomal dominant and recessive)
- Sporadic (acquired) ataxias
*Ataxias due to structural damage is not included in the classification
709
What investigation should be carried out in people with ataxia and why?
MRI scan to exclude structural problems.
710
What spinal roots form the median nerve?
C6-T1
711
Give the signs of a median nerve lesion at the wrist.
- Weakness of abductor pollicis brevis
| - Sensory loss over radial 3.5 fingers and palm
712
Give a sign of a median nerve lesion of the anterior interosseous nerve.
Weakness of flexion of distal phalanx of thumb and index finger.
713
Give the causes of carpel tunnel syndrome.
MEDIAN TRAPS
- Myxoedema
- Enforced flexion (eg. Colles’ splint)
- Diabetic neuropathy
- Idiopathic
- Acromegaly
- Neoplasms
- Tumours
- Rheumatoid arthritis
- Amyloidosis
- Pregnancy/pre-menstrual oedema
- Sarcoidosis
714
Give three treatment options for carpel tunnel syndrome.
- Splinting
- Local steroid injection
- Decompression surgery
715
Give a sign of a brachial plexus lesion.
Pain/paraesthesiae and weakness in affected arm in a variable distribution.
716
Give six causes of a brachial plexus lesion.
- Trauma
- Radiotherapy (breast carcinoma)
- Heavy rucksack
- Cervical rib
- Thoracic outlet compression
- Neuralgic amyotrophy
717
What spinal roots make up the ulnar nerve?
C7-T1
718
What is the most common cause of an ulnar nerve lesion.
Elbow trauma
719
What are the signs of an ulnar nerve lesion?
- Weakness/wasting of medial wrist flexors, interossei, and medial lumbricals (claw hand)
- Hypothenar eminence wasting
- Sensory loss over medial 1.5 fingers and ulnar aide of hand
- Weak flexion of 4th and 5th DIPJ
720
Give three treatment options for an ulnar nerve lesion.
- Rest and avoid elbow pressure
- Soft elbow splinting
- Surgery
721
What spinal roots make up the radial nerve?
C5-T1
722
What is the most common cause of a radial nerve lesion?
Compression against the humerus
723
What are the signs of a radial nerve lesion?
- Test for wrist and finger drop with elbow flexed and arm pronated
- Variable sensory loss (anatomical snuff box reliably affected)
724
Give four muscles or groups of muscles involved with a radial nerve lesion.
BEST
- Brachioradialis
- Extensors
- Supinator
- Triceps
725
What spinal roots make up the lateral cutaneous nerve of the thigh?
L2-L3
726
Give a sign of a lesion of the lateral cutaneous nerve of the thigh.
Meralgia paraesthetica is anterolateral burning thigh pain from entrapment under the inguinal ligament.
727
What spinal roots supply the phrenic nerve?
C3-C5
728
Give two signs of a phrenic nerve lesion.
- Orthopnoea
| - Raised hemidiaphragm on CXR
729
Give 13 causes of a phrenic nerve lesion.
- Lung cancer
- Myeloma
- Thymoma
- Cervical spondylosis/trauma
- Thoracic surgery
- Muscular dystrophy
- Phrenic nucleus lesion (eg. MS)
- C3-C5 zoster
- HIV
- Lyme disease
- TB
- Paraneoplastic syndromes
- Big left atrium
730
What spinal roots make up the sciatic nerve?
L4-S3
731
Give two causes of a sciatic nerve lesion.
- Pelvic tumours
| - Pelvic/femur fractures
732
What are the symptoms of a sciatic nerve lesion?
- Lesions affect hamstrings and all muscles below the knee
- Foot drop
- Loss of sensation below knee (laterally)
733
What spinal roots make up the tibial nerve?
L4-S3
734
What are the signs of a tibial nerve lesion?
- Inability to stand on tiptoe (plantar flexion)
- Can’t invert foot
- Can’t flex toes
- Sensory loss over sole
735
What spinal roots make up the common peroneal nerve?
L4-S1
736
Where is the most common place that a common peroneal nerve lesion occurs?
It gets damaged as it winds around the fibular head.
737
Give two causes of a common peroneal nerve lesion.
- Trauma
| - Sitting cross-legged
738
What are the signs of a common peroneal nerve lesion?
- Foot drop
- Weak ankle dorsiflexion/eversion
- Sensory loss over dorsum of foot
739
What is meant by a ‘functional symptom’?
Abnormality with the function of the body, with the structure still in tact.
Nothing will be found on examination or investigation.
740
What is meant by an ‘organic symptom’?
Structural abnormality with the body that can be detected on examination or investigations.
741
What is meant by ‘somatisation’?
The manifestation of psychological distress by the presentation of physical symptoms.
742
What is meant by ‘dissociation’?
Dissociating immediate reality into alternative reality by disconnecting from thoughts and feelings.
743
Give eight things that should be examined in a mental state examination.
- Appearance
- Behaviour
- Speech
- Mood
- Thoughts
- Perception
- Cognition
- Insight
744
What is one set of functions/symptoms that the MMSE doesn’t test for?
Frontal lobe functions
745
How does ECT work?
Induces seizure into the patient.
746
When is ECT used?
For severely depressed patients.
747
Give an advantage and a disadvantage of ECT.
ADVANTAGE
- Works fast
DISADVANTAGE
- Can affect episodic memory
748
How do most antipsychotic medications (eg. Haloperidol) work?
Block D2 (dopamine) receptor
749
What is depression caused by in Parkinson’s disease?
Serotonin, noradrenaline, and dopamine depletion
750
What causes psychosis when treating Parkinson’s disease?
Excess dopamine
751
The depletion of which neurotransmitter causes memory impairment in Alzheimer’s disease?
Acetylcholine
752
Describe the typical pattern of symptoms if a lesion is in the cerebral hemisphere.
Unilateral
753
Describe the typical pattern of symptoms if a lesion is in the spinal cord.
Bilateral spastic paraparesis
754
Describe the typical pattern of symptoms if a lesion is in the cerebellum.
Coordination problems
755
Describe the typical pattern of symptoms if a lesion is in the brainstem.
Unpredictable and symptoms that don’t seem to follow a logical pattern.
756
Describe the typical pattern of symptoms if a lesion is in the peripheral nerves.
Symmetrical glove and stocking LMN distribution.
757
Is flashing lights a positive or negative symptom?
Positive
758
Give two conditions that may cause patients to see flashing lights.
- Migraine
| - Seizures
759
Is visual loss a positive or negative symptom?
Negative
760
What condition would result from a lesion of the right optic nerve?
Right monocular blindness
761
What condition would result from a lesion of the optic chiasm?
Bitemporal hemianopia
762
What condition would result from a lesion of the right optic tract?
Left homonymous hemianopia
763
What condition would result from a lesion of the right Meyer’s loop?
Left homonymous superior quadrantanopia
764
What condition would result from a lesion of the right baum’s loop?
Left homonymous inferior quadrantanopia
765
What condition would result from a lesion of the right primary visual field?
Left homonymous hemianopia with macular sparing
766
What is a ‘100%’ sensitive sign of a III cranial nerve palsy due to a space occupying lesion?
Pupil involvement
767
Why isn’t pupil involvement in a III cranial nerve palsy specific for a space occupying lesion?
It can also occur with a ‘medical’ III nerve palsy (eg. Diabetes).
768
Give the three aspects of Horner’s syndrome.
- Anhydrosis
- Miosis (small pupil)
- Ptosis
769
What is Horner’s syndrome caused by?
Damage to the sympathetic nerve supply of the face
770
What signs are seen on the ipsilateral side in lateral medullary syndrome?
- Horner’s syndrome
- Limb ataxia
- Loss of facial sensation of pain and temperature
- Reduced corneal reflex
- Dysarthria
- Dysphagia
771
What signs are seen on the contralateral side in lateral medullary syndrome?
Loss of pain and temperature sensation
772
What symptoms will be seen at the level of the lesion in Brown Sequard Syndrome?
Ipsilateral loss of pain and temperature
773
Give three types of primary headache.
- Migraine
- Cluster
- Tension type
774
Give five causes of secondary headache.
- Meningitis
- Subarachnoid haemorrhage
- Giant cell arteritis
- Idiopathic intracranial hypertension
- Medication overuse headache
775
Give a ‘miscellaneous’ type of headache.
Trigeminal neuralgia
776
Give four features of the history of headaches which suggest a secondary headache.
- Age >50yrs
- History of HIV/cancer/trauma/risk factors for cerebral venous sinus thrombosis
- Changing personality or cognitive dysfunction
- Vomiting without any other obvious cause
777
Give seven features of the headaches themselves which suggest a secondary cause.
- Jaw claudication/visual disturbance
- Severe eye pain
- Changing in frequency, characteristics, or associated symptoms
- Postural
- Sudden onset/thunderclap
- Exacerbated by exercise or valsalva (eg. Coughing, laughing, straining)
- Focal neurological symptoms
778
Give four features on examination that may suggest a secondary cause for a headache.
- Fever
- Altered conciousness
- Neck stiffness
- Other abnormal neurological examination
779
Give six indications for immediate referral for a headache.
- Thunderclap headache
- Seizure and new headache
- Suspected meningitis
- Suspected encephalitis
- Red eye
- Headache + new focal neurology (including papilloedema)
780
Give six red flags for a brain tumour when a patient presents with a headache.
- New headache with a history of cancer
- Cluster headache
- Seizure
- Significantly altered conciousness, memory, confusion, coordination
- Papilloedema
- Other abnormal neurological exam or symptom
781
Give four treatment approaches to treating headaches.
- Lifestyle modification and trigger management
- Pharmacological treatment
- Psychological and behavioural treatments
- Surgical treatments
782
Give two pharmacological abortive treatments of a migraine.
- Combination therapy with oral triptan + NSAID/paracetamol
| - Anti-emetic
783
What two medications are usually used to prevent migraine.
- Topimarate
| - Propranolol
784
Give two alternative treatments for prevention of migraines.
- Acupuncture
| - Amitriptyline
785
Give a lifestyle ‘supplement’ that may help to prevent migraines.
Riboflavin
786
Give a ‘last resort’ treatment for prevention of migraines if there has been failure to respond to at least three prior pharmacological treatments.
Botulinum toxin type A
787
Give four pharmacological treatments for trigeminal neuralgia.
- Carbamazepine
- Lamotrigine
- Phenytoin
- Gabapentin
788
Give two treatments for acute attacks of cluster headaches.
- 100% oxygen for about 15 minutes via non-rebreathable mask (not in COPD)
- Sumatripan
789
Give five treatments for preventing cluster headaches.
- Suboccipital steroid injections
- Intranasal civamide
- Verapamil
- Lithium
- Melatonin
790
How many attacks does someone have to have to be diagnosed with a migraine without aura?
5 attacks
791
How long does a migraine last?
4-72 hours
792
2 of the following 4 features should be present to diagnose a migraine...
- Unilateral
- Pulsing
- Moderate/severe
- Aggravation by routine physical activity
793
During a migraine headache, at least one of the following 2 features should occur...
- Nausea and/or vomiting
| - Photophobia and phonophobia
794
How many attacks must the patient have had to be diagnosed with a migraine with aura?
2
795
Describe the motor weakness experienced in a migraine aura.
No motor weakness
796
Describe two types of migraine aura symptoms.
- Fully reversible visual symptoms including positive features and/or negative features
- Fully reversible dysphasic speech disturbance
797
In a migraine aura, are the visual symptoms the same or different in each eye?
Homonymous (same in each eye)
798
In a migraine aura, are the sensory symptoms unilateral or bilateral?
Unilateral
799
Describe the timing of symptoms of a migraine aura.
- At least one aura symptom develops gradually over >5mins and/or different aura symptoms occur in succession over >5mins
- Each symptom lasts >5mins and <60mins
800
When does the headache occur in a migraine with aura?
During aura or follows within 60mins
801
Give nine possible triggers for migraines.
CHOCOLATE
- Chocolate
- Hangovers
- Orgasms
- Cheese
- Oral contraceptives
- Lie-ins
- Alcohol
- Tumult
- Exercise
802
Give three ‘types’ of tension type headache.
- Infrequent
- Frequent
- Chronic
803
How many and how often do infrequent tension type headaches occur?
>10 attacks occurring <1day/month
804
How long does a tension type headache last?
30 minutes to 7 days
805
A tension type headache has 2 of the following characteristics...
- Bilateral
- Pressing/tightening (not pulsating) quality
- Mild/moderate intensity
- Not aggravated by routine physical activity
806
Does nausea/vomiting occur in tension type headaches?
No
807
Does photo/phonophobia occur in tension type headaches?
No more than one should occur
808
How common are cluster headaches?
Not very common
809
How many attacks must someone have to be diagnosed with cluster headaches.
5
810
Describe the severity of pain in cluster headaches.
Severe or very severe
811
Where is the pain felt in cluster headaches?
Unilateral orbital, supraorbital, and/or temporal pain
812
How long do cluster headaches last if left untreated?
5-180minutes
813
Give another feature that occurs with cluster headaches.
Ipsilateral cranial autonomic features and/or a sense of restlessness or agitation.
814
Describe the frequency of attacks in cluster headaches.
1 every other day to 8 per day
815
Describe episodic cluster headaches.
>2 cluster periods lasting 7 days to 1 year separated by pain free periods lasting >1 month.
816
Describe chronic cluster headaches.
Attacks occur for more than 1 year without remission or with remission lasting <1month.
817
How many attacks does a patient need to have to be diagnosed with trigeminal neuralgia?
3
818
Is the pain unilateral or bilateral in trigeminal neuralgia?
Unilateral
819
Describe where the pain occurs in trigeminal neuralgia.
One or more distributions of the trigeminal nerve, with no radiation beyond the trigeminal distribution.
820
The pain felt in trigeminal neuralgia has at least 3 of the following 4 characteristics....
- Reoccurring in paroxysmal attacks from a fraction of a second to 2 minutes
- Severe intensity
- Electric shock like, shooting, stabbing, or sharp
- Precipitated by innoculous stimuli to the affected side of the face
821
How long does the pain last in trigeminal neuralgia?
Fraction of a second to 2 minutes
822
Describe chronic daily headaches.
Headache on >15days per month
823
Give six primary causes of chronic daily headaches.
- Chronic migraine
- Chronic tension type headache
- Chronic cluster headache
- Chronic paroxysmal hemicranias
- Hemicrania continua
- New daily persistent headache
824
Give five secondary causes of chronic daily headache.
- Medication overuse headache
- Chronic post-traumatic headache
- Raised intracranial pressure
- Low CSF pressure headache
- Chronic meningitis
825
How often is a medication overuse headache present?
>15days/month
826
Give four medications likely to cause a medication overuse headache.
- Ergotamine
- Triptans
- Opioids
- Common analgesic medications
827
How are medication overuse headaches treated?
Stopping analgesics
828
Give four aetiologies (and examples) of an olfactory nerve lesion.
- Trauma (ethmoid bone fracture)
- Neurodegenerative disease (Alzheimer’s)
- Congenital (Kallmann syndrome)
- Space occupying lesion
829
What are the clinical features of an olfactory nerve lesion?
Anosmia
830
Give six aetiologies (and examples) of an optic nerve lesion.
- Ischaemic optic neuropathy
- Inflammation (MS)
- Trauma
- Tumours
- Impaired nutrition (B12 deficiency)
- Drugs
831
Give two clinical features of an optic nerve lesion.
- Impaired vision
| - Loss of pupillary light reflex
832
Give two aetiologies of an oculomotor nerve lesion.
- Ischaemic
| - Compression
833
Give four clinical features of an oculomotor nerve lesion.
- Eye looks down and out
- Ptosis
- Horizontal diplopia
- Fixed dilated pupil (usually happens in compressive trauma)
834
Give three aetiologies of a trochlear nerve lesion.
- Microvascular damage
- Trauma
- Cavernous sinus thrombosis
835
Give three clinical features of a trochlear nerve lesion.
- Extorsion of eye
- Diplopia (especially when looking down)
- Mild esotropia (eyes turn in)
836
Give five aetiologies of a trigeminal nerve lesion.
- Tumour
- Vascular compression
- Oral surgery
- Inflammation
- Cavernous sinus thrombosis
837
Give two clinical features of a V1 trigeminal nerve lesion.
- Absent corneal reflex
| - Anaesthesia of forehead
838
Give a clinical feature of a V2 trigeminal nerve lesion.
- Anaesthesia of midface
839
Give two clinical features of a V3 trigeminal nerve lesion.
- Anaesthesia of chin, lower lip, and anterior 2/3 of tongue
| - Muscles of mastication paralysed
840
Give a clinical feature of a lesion to the tensor tympani branch of the trigeminal nerve.
Hearing impairment
841
Give two general features of a trigeminal nerve lesion.
- Jaw deviates to affected side
| - Trigeminal neuralgia
842
Give five aetiologies of an abducens nerve lesion.
- Tumour
- Trauma
- Pseudotumour cerebri (benign intracranial hypertension)
- Cavernous sinus thrombosis
- Duane syndrome (congenital palsy of abducens nerve)
843
Give two clinical features of an abducens nerve lesion.
- Horizontal diplopia
| - Esotropia (medial deviation of eyes)
844
Give nine aetiologies of facial nerve lesions.
- Trauma
- Infection (Herpes, Lyme)
- Tumours
- Pregnancy
- Diabetes
- Guillan Barre
- Sarcoidosis
- Amyloidosis
- Stroke
845
Give a clinical feature of a central facial nerve lesion.
Contralateral mouth drooping
846
Give three clinical features of a peripheral facial nerve lesion.
- Ipsilateral inability to frown
- Ipsilateral inability to close eyelids
- Ipsilateral mouth drooping
847
Give two general clinical features of a facial nerve lesion.
- Taste disorders
| - Dry mouth/eyes
848
Give three aetiologies of a vestibulocochlear nerve lesion.
- Bacterial meningitis
- Lyme disease
- Tumour
849
Give four clinical features of a vestibulocochlear nerve lesion.
- Sensorineural hearing loss
- Vertigo
- Horizontal nystagmus
- Motion sickness
850
What is the usual cause of a glossopharyngeal nerve lesion?
Often unknown (may be associated with compression by a blood vessel)
851
Give six clinical features of a glossopharyngeal nerve lesion.
- Loss of gag reflex
- Loss of carotid sinus reflex
- Flaccid paralysis of soft palate
- Sensory loss over soft palate
- Mild dysphagia
- Throat/ear pain (glossopharyngeal neuralgia)
852
Give five aetiologies of a vagus nerve lesion.
- Trauma
- Diabetes
- Inflammation
- Aortic aneurysms
- Tumours
853
Give six clinical features of a vagus nerve lesion.
- Loss of gag reflex
- Flaccid paralysis of soft palate
- Epiglottic paralysis (aspiration)
- Dysphagia
- Vocal cord paralysis
- Gastroparesis (poor gastric emptying)
854
Give an aetiology of an accessory nerve lesion.
- Surgery in lateral cervical region
855
Give two clinical features of an accessory nerve lesion.
- Paresis of sternocleidomastoid
| - Paresis of trapezius
856
Give two aetiologies of a hypoglossal nerve lesion.
- Tumours
| - Trauma
857
Give two clinical features of a hypoglossal nerve lesion.
- Atrophy and fasciculation of tongue
| - Tongue deviates to side of lesion when protruded
858
Give another name for Bell’s Palsy.
Idiopathic facial nerve lesion
859
Give two risk factors for Bell’s Palsy.
- Pregnancy
| - Diabetes
860
Give a form of treatment which may speed up recovery in Bell’s Palsy.
Steroids (Prednisolone)
861
What is a stroke?
A clinical syndrome, caused by cerebral infarction or haemorrhage, typified by rapidly developing signs of focal and global disturbance of cerebral function lasting more than 24hrs or leading to death.
862
Give four pathological causes of stroke.
- Small vessel occlusion/cerebral microangiopathy or thrombosis in situ
- Cardiac emboli
- Atherothromboembolism
- CNS bleeds
863
Give three causes of cardiac Emboli which may cause a stroke.
- Atrial fibrillation
- Endocarditis
- MI
864
Give five potential causes of CNS bleeds.
- Hypertension
- Trauma
- Aneurysm rupture
- Anticoagulation
- Thrombolysis
865
Give two types of CNS bleeds which can cause a stroke.
- Intracerebral haemorrhage
| - Subarachnoid haemorrhage
866
Give many risk factors for a stroke.
- Hypertension
- Smoking
- Diabetes mellitus
- Heart disease (valvular, ischaemic, atrial fibrillation)
- Peripheral vascular disease
- Past TIA
- Increased packed cell volume
- Carotid bruit
- Oral contraceptive pill
- Hyperlipidaemia
- Alcohol overuse
- Increased clotting
- Increased homocysteine
- Syphilis
867
Give many differential diagnoses of a stroke.
- Head injury
- Hypo/hyperglycaemia
- Subdural haemorrhage
- Intracranial tumours
- Hemiplegic migraine
- Epilepsy (Todd’s palsy)
- CNS lymphoma
- Pneumocephalus (air entry via otitis or mastoid air cells)
- Wernicke’s encephalopathy
- Drug overdose
- Hepatic encephalopathy
- Mitochondrial cytopathies
- Herpes encephalitis
- HIV, HTLV-1, toxoplasmosis
- Abscesses (eg. Typhoid)
- Mycotic aneurysm
- Coccidiodes immitis
- Acanthamoeba/naegleria
868
Describe the rate of onset of stroke symptoms.
- Sudden onset
| - May have further progression over hours (rarely days)
869
Give six signs/symptoms of anterior cerebral artery stroke.
- Leg weakness
- Sensory disturbance in legs
- Gait apraxia (truncal ataxia)
- Incontinence
- Drowsiness
- Akinetic mutism (decrease in spontaneous speech, stuporus state)
870
Give six clinical features of a middle cerebral artery stroke.
- Contralateral arm and leg weakness
- Contralateral sensory loss
- Hemianopia
- Aphasia
- Dysphasia
- Facial droop
871
Give six clinical features of a posterior cerebral artery stroke.
- Contralateral homonymous hemianopia
- Cortical blindness with bilateral involvement of occipital lobe branches
- Visual agnosia (can’t interpret visual information, but can see)
- Prospagnosia (inability to recognise faces)
- Dyslexia, anomic aphasia, colour naming, and discrimination problems
- Unilateral headaches
872
Give five clinical features of posterior circulation strokes.
- Motor deficits (hemiparesis, tetraparesis, facial paresis)
- Dysarthria and speech impairment
- Vertigo, nausea, vomiting
- Visual disturbances
- Altered conciousness
873
Give three clinical features of brainstem infarcts.
- Quadriplegia
- Disturbances of gaze and vision
- Locked-in syndrome
874
Give four structures that may be affected in lacunar infarcts.
- Basal ganglia
- Internal capsule
- Thalamus
- Pons
875
Give the five potential syndromes resulting from lacunar infarcts.
- Ataxic hemiparesis
- Pure motor
- Pure sensory
- Sensorimotor
- Dysarthria/clumsy hand
876
Give three (unreliable) signs which may suggest a haemorrhagic stroke.
- Meningism
- Severe headache
- Coma within hours
877
Give four signs which may suggest an ischaemic stroke.
- Carotid bruit
- Atrial fibrillation
- Past TIA
- Ischaemic heart disease
878
Why should pulse/BP/ECG be carried out in a stroke?
Rule out atrial fibrillation
879
When should hypertension be treated if someone is having a stroke?
Treating high blood pressure may cause harm.
880
What blood glucose should be aimed for in a patient having a stroke?
4-11mmol/L
881
Give four indications for an urgent CT/MRI in a stroke patient.
- Thrombolysis considered
- Cerebellar stroke
- Unusual presentation
- High risk of haemorrhage
882
Stroke patients should be imaged ideally within what time frame?
24hrs
883
Which type of imaging is most sensitive for an acute infarct in a stroke?
Diffusion-weighted MRI
884
What type of imaging best rules out a primary haemorrhage in a stroke patient?
CT
885
When can thrombolysis be given for a stroke?
Up to 4.5 hours post onset of symptoms
886
Describe what is used as thrombolysis treatment in a stroke.
Recombinant tissue plasminogen activator (Alteplase)
887
Give eight contraindications to thrombolysis in a stroke patient.
- Recent surgery
- Recent arterial puncture
- History of active malignancy
- Evidence of brain aneurysms
- Patient on anticoagulation
- Severe liver disease
- Acute pancreatitis
- Clotting disorder
888
When should a stroke patient be on ‘nil by mouth’?
If swallowing attempts might lead to choking
889
When can antiplatelet agents be used to treat a stroke?
Only once haemorrhagic stroke is excluded
890
What antiplatelet agent is given in a stroke?
Aspirin
891
What is the mortality after a first stroke by day 56?
12%
892
In what percentage of patients does a full recovery occur after a stroke?
Less than 40%
893
Give six complications of a stroke.
- Aspiration pneumonia
- Pressure sores
- Contractures
- Constipation
- Depression
- Stress in spouse
894
Give eight general methods of primary prevention for a stroke.
- Platelet treatments
- Lower cholesterol
- Treat atrial fibrillation
- Lower blood pressure
- Smoking cessation
- Control diabetes
- Folate supplements may help
- Lifelong anticoagulation if rheumatic or prosthetic heart valves
895
Give three antiplatelet agents that can help to prevent stroke.
- Aspirin
- Dipyridamole
- Clopidogrel
896
Give two treatments that can help to prevent stroke in atrial fibrillation.
- Warfarin
| - DOACs
897
What antiplatelet agent is commonly used for secondary prevention of a stroke?
Clopidogrel
898
Describe anticoagulation treatment regimes after a stroke.
- Start warfarin two weeks after stroke
- Use antiplatelet therapy until anticoagulated
- If already anticoagulated, replace with antiplatelet for one week
899
Give three things to look out for which may indicate hypertension in a stroke assessment.
- Retinopathy
- Nephropathy
- Cardiomegaly on CXR
900
Give three investigations which may be carried out to assess cardiac sources of emboli in a stroke.
- 24hr ECG to look for atrial fibrillation
- CXR might show enlarged left atrium
- Echocardiogram may show thrombus or valvular lesions
901
How would carotid artery stenosis be assess in a stroke?
Carotid doppler ultrasound +/- CT/MRI angiography
902
What is a transient ischaemic attack?
Sudden onset of focal CNS phenomena due to temporary occlusion of part of the cerebral circulation, usually by emboli, lasting <24hrs.
903
What percentage of first strokes are preceded by a TIA?
15%
904
As well as strokes, give another condition that TIAs are a warning sign for.
Myocardial infarction
905
Compare the signs of a TIA and a stroke.
Features of a TIA mimic those of a stroke in the same arterial territory.
906
Do global events (syncope, dizziness) usually occur in a TIA?
No
907
Give a feature of TIAs which suggests critical intracranial stenosis.
Multiple highly stereotyped attacks
908
In a TIA, what might the patient experience if there is an embolus in the retinal artery?
Amaurosis fugax
909
Give five signs that point to specific causes of a TIA.
- Carotid bruit
- Hypertension
- Heart murmur from valve disease
- Atrial fibrillation
- Fundoscopy may show retinal artery emboli
910
Give four causes of a TIA.
- Atherothromboembolism
- Cardioembolism
- Hyperviscosity
- Vasculitis (rare)
911
Give five differential diagnoses of a TIA.
- Hypoglycaemia
- Migraine aura
- Focal epilepsy
- Hyperventilation
- Retinal bleeds
912
Give six rare differential diagnoses of a TIA.
- Malignant hypertension
- MS
- Intracranial tumours
- Peripheral neuropathy
- Phaeochromocytoma
- Somatization
913
Give six investigations to carry out in a TIA.
- Bloods
- CXR
- ECG
- Carotid doppler +/- angiography
- CT or diffusion-weighted MRI
- Echocardiogram
914
Give four general approaches to management of a TIA.
- Control cardiovascular risk factors
- Antiplatelet drugs
- Warfarin
- Carotid endarterectomy
915
Give four ways to control cardiovascular risk factors after a TIA.
- Lower blood pressure
- Control hyperlipidaemia
- Control diabetes
- Smoking cessation
916
Give three antiplatelet drugs that might be started after a TIA.
- Clopidogrel
- Aspirin
- Dipyidamole
917
When is warfarin given after a TIA.
If it was caused by cardiac emboli
918
What is an endarterectomy?
Surgery to remove an atheromatous plaque
919
When is a carotid enderterectomy carried out in a TIA?
If >70% stenosis at origin of internal carotid artery and operative risk is good.
920
What system is used to determine whether urgent referral is required after a TIA?
ABCD2
921
Give the elements of ABCD2.
- Age >60yrs (1)
- Blood pressure >140/90 (1)
- Clinical features (unilateral weakness (2), speech disturbance without weakness (1))
- Duration of symptoms (>1hr (2), 10-59mins (1))
- Diabetes (1)
922
A score of _______ on ABCD2 is strongly predictive that the patient will have a stroke.
6+
923
A score of ______ on ABCD2 indicates that the patient should be seen by a specialist in 24 hrs.
4+
924
All patients with a suspected TIA should be seen by a specialist within ____________.
7 days
925
How long should the patient avoid driving after a TIA?
1 month
926
When should the DVLA be informed about TIAs.
Multiple attacks in a short period, or residual deficit
927
What is the risk of a stroke within 90 days if a patient is treated for a TIA within 72hrs?
2%
928
What is the risk of a stroke within 90 days is a TIA is treated within 3 weeks?
10%
929
What is the combined risk of a stroke and MI per year after a TIA?
9%
930
Compare the rate of onset in delirium and dementia.
DELIRIUM
- Acute (hours to days)
DEMENTIA
- Insidious (months to years)
931
Compare the course of the condition in delirium and dementia.
DELIRIUM
- Fluctuating
DEMENTIA
- Progressive
932
Compare the duration of delirium and dementia.
DELIRIUM
- Hours to weeks
DEMENTIA
- Months to years
933
Compare the consciousness levels in delirium and dementia.
DELIRIUM
- Altered
DEMENTIA
- Normal unless severe
934
Use the pneumonic to give the signs/symptoms of multiple sclerosis.
DEMYELINATION
- Diplopia
- Eye movements painful (optic neuritis)
- Motor weakness
- nYstagmus
- Elevated temperature worsens symptoms (Uhthoff’s phenomenon)
- Lhermitte’s sign
- Intention tremor
- Neuropathic pain
- Ataxia
- Talking slurred
- Impotence
- Overactive bladder
- Numbness
