neurology Flashcards
1
Q
spastic gait
A
Pace shortens and narrow base is maintained
more pronounced in extensor muscles causing stiff and slow walking.
2
Q
parkinsonian gait
A
posture is stooped and arm swing reduced
gait becomes hesitant with shorter rapid steps which slows down to a shuffle in advanced disease
3
Q
Describe the presentation of cerebellar ataxia
A
In lateral cerebellar disease - stance becomes broad-based and uncoordinated
In midline (vermis) cerebellar disease - truncal ataxia leads to a tendency to fall backwards or sideways
4
Q
Outline the effect of gait occurring in sensory ataxia?
A
Loss of proprioception - leading to a broad-based, high-stepping gait
5
Q
syncope
A
a short duration (usually 20-30s) of loss of consciousness caused by a global reduction in cerebral blood flow
6
Q
components of motor axis
A
motor cortex
corticospinal tracts
anterior horn cells
spinal nerve roots
peripheral nerves
neuromuscular junctions
muscle fibres
7
Q
components of pyramidal system
A
corticospinal tract
corticobulbar tract
8
Q
upper motor neurone lesion features
A
signs on opposite side to lesion
no muscle wasting
spasticity (+/- clonus)
hyperreflexia
extensor plantar response (Babinski signs)
9
Q
lower motor neurone lesion features
A
signs on same side as the lesion
fasciculations
wasting
hypotonia
hyporeflexia
10
Q
two patterns of clinical features which occur in an upper motor neurone lesion?
A
hemiparesis and paraparesis
11
Q
hemiparesis
A
weakness of limbs on one side (usually caused by a lesion within the brain or brainstem e.g. a stroke)
12
Q
paraparesis
A
weak legs.
indicative of bilateral damage to the corticospinal tracts (most commonly due to lesions below the T1 spinal level
13
Q
lower motor neurone
A
motor pathway from anterior horn cell or cranial nerve via a peripheral nerve to the motor endplate
14
Q
most common causes of a lower motor neurone lesion
A
anterior horn cells lesions e.g. motor neurone disease
spinal root lesion e.g. cervical and lumbar disc lesions
peripheral nerve lesions e.g. trauma, compression etc.
15
Q
most common disease of the neuromuscular junction?
A
Myaesthenia Gravis
16
Q
elevation of what plasma muscle enzyme indicates muscle disease?
A
creatine kinase
17
Q
sensory peripheral nerves carry information from the dorsal root ganglion to the brain via three main pathways, what are they?
A
posterior columns
spinothalamic tracts
spinocerebellar tract
18
Q
sensory modalities transmitted via the posterior columns
A
vibration
two-point discrimination
light touch
19
Q
sensory modalities transmitted via the spinothalamic tract
A
pain
temperature
20
Q
route of the posterior column
A
fibres ascend uncrossed to the gracile and cuneate nuclei in the medulla.
axons from the second-order neurones cross the midline to form the medial lemniscus and pass to the thalamus
21
Q
route of the spinothalamic tract
A
fibres synapse in dorsal horn of the cord, cross the cord at that spinal level and ascend to the thalamus
22
Q
sensory modalities are transmitted via the spinocerebellar tract?
A
proprioception
23
Q
route of the spinocerebellar tract
A
does not decussate i.e. innervates the ipsilateral side of the body
24
Q
The quality and distribution of the symptoms depend on the site of the lesion. Outline the potential sites of nerve lesions and their corresponding characteristics?
A
Peripheral nerve lesions - symptoms in the distribution of the affected nerve
Spinal root lesions - symptoms referred to affected dermatome
Spinal cord lesions - Symptoms are evident below the level of the lesion
Pontine lesions - Loss of all possible sensation on the opposite side of the lesion
Thalamic lesions - A rare cause of complete contralateral sensory loss
Cortical lesions - sensory loss, neglect of one side of the body and subtle disorders of sensation
25
What root value does the ankle jerk reflex correspond to?
S1
26
What root value does the knee jerk reflex correspond to?
L2, 3, 4
27
What root value does the biceps jerk reflex correspond to?
C5
28
What root value does the supinator jerk reflex correspond to?
C6
29
What root value does the triceps jerk reflex correspond to?
C7
30
What root value does the abdominals reflex correspond to?
T8-11
31
corticobulbar tract
component of the pyramidal system.
responsible for transmitting neurones from cerebrum to the cranial nerves.
- control muscles of the face and neck - facial expression
- mastication, swallowing
32
extrapyramidal system
component of the motor system which do not travel through the pyramids.
- complex movements
- postural control
33
components of extrapyramidal system
| Hint = RPMLT
rubrospinal tract
pontine reticulospinal tract
medullary reticulospinal tract
lateral vestibulospinal tract
tectospinal tract
34
cranial nerve nuclei that are located in midbrain
CN 1, 2, 3, 4
35
cranial nerve nuclei that are located in pons
CN 5, 6, 7, 8
36
cranial nerve nuclei that are located in medulla
CN 5, 7, 8, 9, 10, 11, 12
37
causes of anosmia (first cranial nerve pathology)
nasal congestion (most common)
anterior fossa tumours
head injury
38
what structure do the optic nerves enter the cranial cavity through?
optic foramina
39
Outline the course of the optic nerve
Optic nerve transmitted into the cranial cavity
Forms optic chiasm
Continued as the optic tracts
Fibres of the optic tract project to the visual cortex of the occipital lobe through the lateral geniculate body and third cranial nerve nucleus for pupillary light reflex.
40
Specific visual field losses can indicate a specific pathology. What does the presence of paracentral scotoma indicate?
Retinal lesion
41
Specific visual field losses can indicate a specific pathology. What does the presence of monocular field loss indicate?
Optic nerve lesion
42
Specific visual field losses can indicate a specific pathology. What does the presence of bitemporal hemianopia indicate?
Chiasmal lesion
43
Specific visual field losses can indicate a specific pathology. What does the presence of homonymous hemianopia indicate?
Optic tract/radiation/cortex/pole lesion (usually due to posterior cerebral artery infraction)
44
Specific visual field losses can indicate a specific pathology. A lesion in what lobe(s) of the brain may present as a homonymous quadrantanopia indicate?
Temporal/parietal lesion
45
Name some causes for persistent pupillary dilatation
A third cranial nerve palsy
Antimuscarinic eye drops
Myotonic pupil (no pathological indication - no constriction on light or convergence)
46
Name some causes of persistent pupillary constriction
- Parasympathomimetic eye drops (used in treatment of glaucoma)
- Horner's Syndrome
- Argyll Robinson pupil (abnormality seen in neurosyphilis - absent reaction to light but convergence causes constriction)
- Opiate addiction
47
A palsy of the seventh cranial nerve, cerebellar signs and involvement of the fifth, sixth and eighth cranial nerves indicates a lesion at what anatomical position?
Cerebellopontine angle (most commonly meningioma or acoustic neuroma)
48
What is the motor function of the trochlear nerve (CN IV)?
Superior oblique muscle
49
What is the motor function of the abducens nerve (CN VI)?
Lateral rectus muscle
50
What is the motor function of the oculomotor nerve (CN III)?
Superior, inferior and medial rectus muscles
Sphincter pupilae
Levator palpebrae superioris
51
The brainstem, cortex and cerebellum coordinate the third, fourth and sixth cranial nerve to 'conjugate' eye movements. What is the clinical presentation of a defect in this coordination (called supranuclear palsy)?
Lead to paralysis of conjugate movements of the individual muscles innervated by the third, fourth and sixth cranial nerves
52
Describe the clinical picture of an infranuclear palsy of the third cranial nerve
Unilateral complete ptosis with 'down and out' appearance
Total paralysis leads to a dilated, fixed pupil
Partial paralysis paralysed pupil but still reacted to reactive to light
53
Describe the clinical picture of an infranuclear palsy of the fourth cranial nerve
Torsional diplopia (two objects at an angle) when attempting to look down
Head is tilted away from the side of the lesion
54
Describe the clinical picture of an infranuclear palsy of the sixth cranial nerve
The eye cannot be abducted beyond the midline
Eye turns in medially with concurrent diplopia
55
What is the most common cause of a total and partial third cranial nerve palsy respectively?
Total palsy - berry aneurysm of the posterior communicating artery
Partial palsy - diabetes mellitus
56
What is often the earliest sign of a fifth cranial nerve lesion?
Diminution of the corneal reflex
57
What is the clinical presentation of a total fifth cranial nerve palsy?
Unilateral sensory loss on the face, tongue and buccal mucosa with jaw deviation to the side of the lesion
58
What is the cardinal feature of a supranuclear palsy of the fifth cranial nerve?
Brisk jaw jerk reflex
59
What is trigeminal neuralgia?
Severe paroxysms of knife-like pain occur in one or more sensory divisions of the trigeminal nerve brought on by a specific trigger
No objective clinical signs
60
How is trigeminal neuralgia treated?
Anticonvulsant carbamazepine suppresses attacks
Thermocoagulation of the tigeminal ganglion may be necessary
61
What are the two major branches of the seventh cranial nerve unrelated to facial muscle innervation?
Chorda tympani - providing taste to the anterior two third of the tongue
Nerve to the stapedius - dampening effect on sound
62
What are the potential features of a facial nerve palsy in addition to the paralysis of facial muscles?
Hyperacusis (undue sensitivity to sounds)
| Loss of taste to the anterior two thirds of the tongue
63
Outline the features of a lower motor neurone lesion of the facial nerve
Ipselateral weakness of the facial muscles
Hyperacusis
Loss of taste of the anterior two thirds of the tongue
64
What is Bell's Palsy?
Common, acute isolated unilateral facial nerve palsy probably due to a viral herpes simplex infection causing swelling within the petrous part of the temporal bone
65
How is a lower motor neurone facial palsy managed?
```
Protect cornea from ulceration
Oral prednisolone (60mg/day for 10 days)
```
Antivirals used in severe cases
66
What is the differential diagnosis of paralysed facial muscles?
Bell's Palsy
Ramsey-Hunt Syndrome
Parotid Tumour
67
What is Ramsey-Hunt Syndrome?
Herpes zoster (shingles) of the geniculate ganglion causing facial muscles palsy and herpetic vesicles of the external auditory meatus/soft palate
68
Outline the features of an upper motor neurone lesion of the facial nerve
Weakness of the lower part of the face on the side opposite to the lesion
Wrinkling of the forehead and eye closure are normal
69
What are the features of an eighth cranial nerve palsy?
Cochlear nerve branch - sensorineural deafness and tinnitus
Vestibular nerve branch - vertigo +/- nausea/vomiting
70
List some causes of eighth nerve palsy
Brainstem (tumour, mutiple sclerosis)
Cerebellopontine angle (acoustic neuroma)
Petrous temporal bone (trauma, middle ear infection)
71
What is nystagmus?
Rhythmic oscillation of the eyes
72
What are the two types of nystagmus?
Pendular - no rapid phase (due to longstanding visual impairment)
Jerk - rapid and slow phases (two subtypes: horizontal or vertical)
73
The lower four cranial nerves lie in the medulla (the "bulb") are usually affected together. Outline the features of a lower motor neurone type palsy of the bulbar cranial nerves (bulbar palsy)
Dysarthria (slow speech)
Dysphagia
Nasal regurgitation
Wasting and fasciculation of the tongue
74
What are the most common causes of bulbar palsy?
Motor neurone disease
| Syringobulbia (fluid-filled cavity in the brainstem)
75
Outline the features of an upper motor neurone type palsy of the bulbar cranial nerves (pseudobulbar palsy)
Weakness of the muscles of speech and swallowing with small, spastic tongue (no wasting or fasciculation)
76
What are the most common causes of pseudobulbar palsy?
Motor neurone disease
| Multiple sclerosis
77
What is normal CSF pressure?
80-120 mmH2O
78
What is the purpose of nerve conduction studies?
Differentiates between axonal and demyelination neuropathy
79
What are some known complications of lumbar punctures?
Post-procedure headache, infection, herniation (coning)
80
What are the cardinal investigations of suspected muscle disease? (4)
```
Serum creatine kinase and aldose
EMG
Muscle biopsy (histology and immunohistochemical staining)
```
81
What area of the brain is chiefly responsible for states of arousal?
Reticular formation (extending from the brainstem to the thalamus)
82
What is coma?
A state of unconsciousness from which the patient cannot be roused
83
What is a stuporous state?
When a patient is sleepy but can be roused with vigorous stimulation
84
A patient unresponsive to anything except pain is broadly corresponsive to what GCS level?
8
85
What must coma be differentiated from?
Persistent vegetative state
Brain death
Locked-in syndrome
86
What is a persistent vegetative state?
A state of wakefulness in which sleep-wake cycle persists but without detectable awareness
87
What is the cardinal sign of brain death?
Bilaterally fixed and dilated pupils
88
What are dysconjugate eyes a sign of?
Brain stem lesion
89
Conjugate lateral deviation of the eyes indicates what two potential pathologies?
Ipselateral cerebral haemorrhage/infarction
| Contralateral pontine lesion
90
What is brain death?
Irreversible loss of the capacity for consciousness, combined with the irreversible loss of the capacity to breathe
91
What are the three main criteria of the diagnosis of brain death?
1. Irremediable structural brain damage
2. Absent motor responses to any stimulus
3. Absent brainstem function (lack of pupillary, corneal, gag, cough, doll's head or caloric reflexes, lack of spontaneous breathing)
92
What is the definition of a stroke?
Rapid onset of neurological deficit lasting greater than 24-hours, which is the result of a vascular origin.
93
What is the definition of stroke in evolution?
When symptoms and signs are getting worse (usually within 24-hours of onset)
94
What is the definition of a minor stroke?
When the patient recovers without significant neurological deficit, usually within one week
95
What is the definition of a transient ischaemic attack?
Transient episode of neurological dysfunction caused by focal brain, spinal cord or retinal ischaemia without acute infarction.
96
Arterial thrombosis causing stroke can arise from what locations?
Atheromatous plaques
Cardiac mural thrombi (post-MI)
Left atrium (due to atrial fibrillation)
97
What is the differential diagnosis of stroke?
Intracranial venous thrombosis
Multiple sclerosis
Space-occupying lesion e.g. tumour/abscess
98
In young adults, 20% of are caused by carotid/vertebral artery dissection. What clinical picture might be suggestive of this pathophysiology?
Recent neck pain, trauma or neck manipulation
99
What are the major risk factors for thromboembolic stroke?
Hypertension, diabetes mellitus, cigarette smoking, hyperlipidaemia, obesity, excessive alcohol consumption
100
What are the differential diagnoses for transient ischaemic attack?
Demyelination, tumour
101
How is the risk of stroke measured following a confirmed TIA?
ABCD2 risk
102
Hemiplegia is often caused by infarction of what area of the brain during a cerebral haemorrhage stroke?
Infarction of the internal capsule
103
What is the internal capsule?
Narrow zone of motor and sensory fibres that converges on the brainstem from the cerebral cortex
104
Brainstem infarction can manifest in a number of different clinical presentations. Name a few. (4)
Lateral medullary syndrome
Coma
Locked-in syndrome
Pseudobulbar palsy
105
What is lateral medullary syndrome?
Most common of the brainstem vascular syndromes caused by occlusion of the posteiror inferior cerebellar artery
Presents as sudden vomiting, vertigo, facial numbness, Horner's syndrome, diminished gag reflex
106
Coma as a result of stroke is caused by infarction of what site in the brain?
Reticular activating syndrome
107
Upper brainstem stroke causes what pathology?
Locked-in syndrome
108
Lower brainstem stroke causes what pathology?
Pseudobulbar palsy
109
What is multi-infarct dementia?
Also called vascular dementia
Multiple small cortical infarcts resulting in generalised intellectual loss with step-wise progression
Clinical picture of dementia, pseudobulbar palsy and Parkinsonian 'shuffling' gait
110
What is the indicated acute treatment of stroke?
Aspirin (300mg ASAP)
Thrombolysis (IV tPA) given within 4.5hrs in acute ischaemic stroke
111
What secondary interventions are put in place following acute stroke?
Hypertension management
Carotid endarterectomy (in the case of stenosed carotid artery to prevent recurrence of stroke)
Address other modifiable risk factors including smoking, obesity etc.
112
What are the major risk factors for intracerebral haemorrhage?
Hypertension, excess alcohol consumption, increasing age and smoking
113
What is the presentation of intracerebral haemorrhage?
Sudden loss of consciousness and stroke often accompanied by severe headache
114
What is a subarachnoid haemorrhage?
Refers to spontaneous arterial bleeding into the subarachnoid space
115
What is the radiological appearance of a subarachnoid haemorrhage?
Hyperdense appearance typically around the branching points of arteries of the Circle of Willis
116
What are the most common aetiologies of subarachnoid haemorrhage?
Rupture of saccular 'berry aneurysms'
| Congenital arteriovenous malformation
117
What are the symptoms of a berry aneurysm before it ruptures?
Often asymptomatic before they rupture
Any symptoms are due to mass effect, most commonly a painful third cranial nerve palsy
118
What are the symptoms of a berry aneurysm when it ruptures?
Sudden onset of severe headache (often occipital), accompanied by nausea and vomiting
Potential loss of consciousness and meningeal irritation (neck stiffness and positive Kernig's sign)
119
Lumbar punctures must be delayed until at least 12 hours after onset of symptoms in order to confirm diagnosis. Why is this?
To allow sufficient time for haemoglobin to degrade into oxyhaemoglobin and bilirubin (detectable for up to two weeks following SAH)
120
How is subarachnoid haemorrhage immediately managed?
Best rest and supportive measures with control of hypertension and IV nimodipine (CCB) to reduced cerebral artery spasm.
Discussion with neurosurgeon for obliteration of aneurysm to prevent re-bleeding
121
What is a subdural haematoma?
Accumulation of blood in the subdural space following a venous bleed.
122
What is the aetiology of subdural haematoma?
Almost always trauma. Latency between trauma and onset of symptoms may be weeks or months
123
What are the main clinical features of a subdural haematoma?
Headache, drowsiness and fluctuant confusion
124
What is an extradural haematoma?
Bleeding outside of the dura mater most commonly due to temporal bone fracture and subsequent rupture of the underlying middle meningeal artery
125
Clinically how does extradural haematoma present?
Head injury with a brief period of unconsciousness followed by a lucid interval of recovery.
Then followed by rapid neurological deterioration if drainage is not immediately carried out.
126
seizure
transient occurrence of signs and symptoms due to abnormally excessive or asynchronous neuronal activity within the brain.
127
epilepsy
a continuing tendency to have seizures
128
epilepsy classification
generalised seizure type
partial seizure type
129
tonic seizures
intense body stiffening
130
tonic-clonic seizures (grand mal)
sudden onset
rigid tonic phase followed by a convulsion (clonic) phase
eyes remain open; lasts seconds to minutes
may be associated with tongue biting or urinary incontinence
131
what period typically follows a grand mal seizure?
post-ictal phase
132
post-ictal phase
flaccid unresponsiveness followed by confusion or drowsiness lasting several hours.
headache is common
133
absence seizures (petit mal)
disorder of childhood in which the child ceases all activity, stares and pales for a few seconds only.
tendency to develop generalised tonic-clonic seizures in adulthood
134
myotonic seizures
isolated muscle jerking
135
akinetic seizures
cessation of movement, falling and loss of consciousness
136
partial seizures
epileptic activity involving part of the brain only
1. simple (not affecting consciousness or memory)
2. complex (affecting awareness and memory)
May become generalised (secondarily generalised)
137
stimuli that may induce seizures in an epileptic patient?
flashing lights or a flickering television screen may provoke an attack
138
how is a seizure medically aborted?
rectal or IV diazepam or lorazepam
139
repeated seizures with minimal recovery periods between may lead to what medical emergency?
status epilepticus
140
status epilepticus
continuous seizures for greater than thirty minutes
141
risk to life in status epilepticus
death from cardiorespiratory failure
142
What may precipitate status epilepticus?
Sudden withdrawal of anti-epileptic drugs, intercurrent illness or alcohol abuse
143
How is status epilepticus managed outside of a hospital setting?
Rectal diazepam or buccal midazolam
144
How is status epilepticus managed in a hospital setting?
Administer oxygen and obtain IV access
IV lorazepam/rectal diazepam (first line)
If seizures continue - given phenytoin (second line) then phenobarbital (third line)
145
For refractory status epilepticus, what management is required?
Intubation, ventilation and anaesthesia in intensive care
146
What is the principle of treatment for epilepsy?
Monotherapy is the goal with dose titrated up until seizures are controlled
147
Describe the common syndrome of intoxication of all anti-convulsants
Ataxia, nystagmus and dysarthria
148
What is the pathophysiology of idiopathic Parkinson's disease?
Progressive depletion of dopamine-secreting cells in the substantia nigra due to abnormal accumulation of cytoplasmic inclusions called Lewy bodies
149
Outline the clinical features of Parkinson's disease
Combination of rest tremor, rigidity and bradykinesia (slow movements)
Poverty of movement and speech
Postural changes (stoop)
Non-motor features (depression, hallucinations, dementia, insomnia, fatigue, weight loss etc.)
150
How is Parkinson's disease managed?
Levodopa (combined with peripheral decarboxylase inhibitor)
Dopamine agonists
Monoamine oxidase B inhibitors (inhibit dopamine catabolism)
Physiotherapy
151
What is benign essential tremor?
Familial (autosomal dominant) tremor of the arms and head. Not usually present at rest and made more obvious when adopting a posture and anxiety.
Improved by alcohol, propranolol and mirtazepine
152
What is chorea?
Continuous flow of jerky, quasi-purposive movements interfering with voluntary movements but absent during sleep
153
What is Huntington's Disease?
Autosomal dominant (full penetrance) relentlessly progressive course with chorea, personality change preceding dementia and death
154
What is the pathophysiology of Huntingdon's Disease?
CAG repeats in the Huntingdon's disease gene on chromosome 4 leads to the production of mutant huntingtin protein leading to a depletion of GABA and acetylcholine in the basal ganglia
155
What is hemiballismus?
Violent swinging of one side of the body usually caused by infarction or haemorrhage in the contralateral subthalamic nucleus
156
What is myoclonus?
Sudden, involuntary jerking of a single muscle or group of muscles. Most commonly benign essential myoclonus which is a sudden body jerk just before falling asleep.
157
What is multiple sclerosis
A chronic debilitating autoimmune disorder of the CNS in which multiple plaques of demyelination occur disseminated in time and space.
158
Multiple sclerosis has a predilection for distinct CNS sites; name five.
1. Optic nerves
2. Periventricular white matter
3. Brainstem
4. Cerebellum
5. Cervical spinal cord
159
The clinical features of multiple sclerosis depend on the site of demyelination. Outline the clinical presentation of optic neuropathy caused by multiple sclerosis
Blurred vision and unilateral eye pain
Inflammation at the optic head causes pallor and disc swelling (optic neuritis)
Retrobulbar (proximal to the fundus) neuritis may occur in which case fundus examination is normal
160
The clinical features of multiple sclerosis depend on the site of demyelination. Outline the clinical presentation of brainstem demyelination caused by multiple sclerosis
Diplopia, vertigo, dysphagia, dysarthria, facial muscle weakness
161
The clinical features of multiple sclerosis depend on the site of demyelination. Outline the clinical presentation of spinal cord lesions caused by multiple sclerosis
Numbness/paraesthesia are common in MS and reflect involvement of the spinothalamic tract and posterior column lesions.
162
There are four main clinical patterns of multiple sclerosis; what are they?
1. Relapsing-remitting MS (85-90%)
2. Secondary progressive MS
3. Primary progressive MS (10-15%)
4. Relapsing-progressive MS (<5%)
163
Describe the relapsing-remitting variant of multiple sclerosis
Symptoms occur in attacks with onset over days which then recover over a period of weeks. Disability is accumulated over time due to suboptimal recovery of relapses.
164
Describe the secondary progressive variant of multiple sclerosis
Late-stage of relapsing-remitting MS with a progressive disability over years.
165
Describe the primary progressive variant of multiple sclerosis
Characterised by progressive worsening disability with no evidence of relapsing or remitting.
166
What is the differential diagnosis of multiple sclerosis?
Early MS, without the diagnostic necessity of time, may be difficult to distinguish from other inflammatory conditions including:
- SLE
- Sarcoidosis
- Mass or vascular lesion
167
What investigations are indicated for the diagnosis and monitoring of multiple sclerosis?
MRI of brain and spinal cord
Electrophysiological tests
CSF examination (usually unnecessary - useful for exclusion of other pathology)
168
Outline some of the treatment options for multiple sclerosis
| 6
1. Short courses of steroids - reduces severity of relapses
2. Subcutaneous beta-interferon - reduces relapse rate
3. Glatiramer acetate - similar effect to interferon
4. Natalizumab - monoclonal antibody (second line option)
5. Newer agents e.g. fingolimod - reduce relase rate significantly
6. Physiotherapy nand occupational therpay
169
What is Kernig's sign?
Inability to allow full extension of the knee when the hip if flexed to 90 degrees
170
Outline the clinical picture of infective meningitis
Headache, neck stiffness and fever develop over minutes to hours
Photophobia, vomiting and papilloedema
171
List three major complications of infective meningitis
Venous sinus thrombosis
Severe cerebral oedema
Cerebral abscess
172
How may a major complication present with ongoing meningitis?
Progressive drowsiness
Lateralising signs
Cranial nerve lesions
173
What is the cardinal sign of meningococcal septicaemia?
Non-blanching, petechial or purpuric rash (with shock)
174
Outline the course of viral meningitis
Usually benign and self-limiting condition lasting about 4-10 days with no serious sequelae
175
Outline the presentation of chronic meningitis
Long history of vague symptoms including headache, lassitude, anorexia, vomiting etc.
176
List some potential diagnosis of meningitis
Subarachnoid haemorrhage
Migraine
Viral encephaliits
Cerebral malaria (can mimic meningitis)
177
Give four examples of bacteria which can cause meningitis
Neisseria meningitidis
Streptococcus pneumoniae
Staphylococcus aureus
Listeria monocytogenes
178
Give four examples of viruses which can cause meningitis
Enterovirus (ECHO, Coxsackie)
Poliomyelitis
Mumps
HIV
179
Give two examples of fungi which can cause meningitis
Cryptococcus neoformans
| Candida albicans
180
It is important to immunise close contacts of those diagnosed with meningococcal meningitis (i.e. close family). What prophylaxis is used?
Oral rifampicin/ciprofloxacin to erradicate nasal carriage of causative organism
181
How is tuberculous meningitis diagnosed?
Auramine stain
182
How is cryptococcal meningitis diagnosed?
India ink stain
183
What investigation should not be performed if meningococcal sepsis is suspected?
Lumbar puncture (due to risk of cerebellar coning)
184
How is acute suspected bacterial meningitis treated?
Immediate third-generation cephalosporin (e.g. ceftriaxone) and amoxicillin (covering Listeria) and dexamethasone
Specific antibiotics following results of Gram staining, culture and sensitivities
185
What is encephalitis?
Inflammation fo the brain parenchyma
186
How does encephalitis differ from meningitis?
Unlike meningitis, there is usually cerebral dysfunction with altered mental status, motor and sensory deficits
187
What are the clinical features of acute viral encephalitis?
Mild, self-limiting illness with fever, headache and drowsiness
Severe disease is rare but can cause seizures and coma
188
In the case of suspected HSV or VZV encephalitis, what is the treatment?
IV aciclovir (10mg/kg every 8 hrs for 14-21 days)
189
Cerebral abscess follows infection spread from parameningeal infective focuses or from a distal site e.g. bloodstream. Give examples of parameningeal infective focuses.
Paranasal air sinuses
Middle ear
Skull fracture
190
Outline the clinical features of a cerebral abscess
Headache, focal neurological signs, seizures
Potential raised intracranial pressure
191
What investigation is contraindicated in the case of suspected cerebral abscess?
Lumbar puncture (due to risk of cerebellar coning)
192
How is cerebral abscess treated?
IV antibiotics and sometimes surgical decompression
193
How doe spinal epidural abscess present?
Back pain and fever accompanied by paresis/root lesions
194
What four clinical syndromes may arise from untreated neurosyphilis?
1. Asymptomatic neurosyphilis
2. Meningovascular syphilis
3. General paralysis of the insane
4. Tabes dorsalis
195
How is neurosyphilis treated? What is the outcome of the treatment
Benzylpenicillin 1g IM daily for 10 day
May arrest but not reverse any neurological damage
196
Outline the presentation and treatment of Creutzfeldt-Jakob disease
Pathologically spongiform change in the brain due to infective prion causing progressive dementia and death (incurable)
197
The HIV virus is not directly neuroinvasive or neurovirulent but can cause meningitis in a variety of ways. How?
HIV seroconversion
AIDS-demnetia complex
Progressive multifocal leukoencephalopathy (PML)
198
What is progressive multifocal leukoencephalopathy?
A fatal demyelinating disease of the central nervous system (CNS), caused by the lytic infection of oligodendrocytes by a human polyomavirus, JC virus (JCV)
199
How is progressive multifocal leukoencephalopathy related to HIV?
Patients become susceptible to JCV infection at very low CD4 counts, as in HIV infection
200
What are the clinical features of a cerebral tumour?
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Progressive focal neurological deficits
Raised intracranial pressure
Focal/generalised epilepsy
201
List three of the most common primary malignant brain tumours
Glioma
Embryonal tumours (e.g. medulloblastoma)
Lymphoma
202
List two of the most common primary benign brain tumours
Meningioma
| Neurofibroma
203
What primary cancers commonly metastasise to the brain?
Bronchus, breast, prostate
204
What neurological defects may arise from frontal lobe tumours?
Personality change, aparthy, intellectual deterioration
Frontal speech area (Broca's area) - expressive dysphasia
Motor cortex (pre-central gyrus) - hemiparesis
205
What neurological defects may arise from parietal lobe tumours?
Homonymous field defect
Cortical sensory loss
Hemiparesis
Seizures
206
What are the cardinal signs/symptoms of raised intracranial pressure?
Headache (changes with posture, worse on coughing, sneezing, bending or straining)
Vomiting
Papilloedema
207
As tumours grow, there may be compression of the brainstem causing what symptoms?
Drowsiness respiratory depression, bradycardia, coma and death
208
What are the potential differential diagnoses of brain tumour?
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Other masses (cerebral abscess, tuberculoma, subdural/intracranial haematoma)
Stroke
Benign idiopathic intracranial hypertension
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209
What is benign idiopathic intracranial hypertension?
Benign condition common in young obese women presenting with headache and papilloedema causing raised intracranial pressure
210
What is hydrocephalus?
Excessive amounts of CSF within the cranium
211
Where is CSF produced in the brain?
In the cerebral ventricles
212
What are the two potential mechanisms behind the cause of hydrocephalus?
1. Increased production of CSF (rare)
| 2. Blockage to outflow
213
What is a congenital cause of hydrocephalus?
Arnold-Chiari malformation
214
What is Arnold-Chiari malformation?
Cerebellar tonsils descend into the cervical canal - blocking drainage of CSF
215
List some causes of adult hydrocephalus
Posterior fossa/brainstem tumours
Subarachnoid haemorrhage/head injury/trauma
Third ventricle colloid cyst (intermittent hydrocephalus)
Normal-pressure hydrocephalus
216
What is normal pressure hydrocephalus?
Dilation of the cerebral ventricles without signs of raised ICP.
217
How does normal pressure hydrocephalus present?
Common in the elderly
Dementia, urinary incontinence and ataxia
218
How is hydrocephalus treated?
Surgical insertion of a shunt to allow drainage of CSF (ventriculoperitoneal/ventriculoatrial)
219
What are the key features of a headache history?
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Location, severity and character
Associated symptoms e.g. nausea, photophobia, fever
Presence of autonomic symptoms e.g. ptosis or tearing
Triggers - the effect of posture?
Is the headache episodic or part of a pattern?
Duration
Analgesic use
Family history
Red flag symptoms
Sudden onset?
Features of raised ICP?
Jaw claudications/scalp tenderness?
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220
What is a tension headache? What is the cause?
Very common bilateral headache
Neurovascular irritation and referred tenderness to scalp
221
What does a tension headache feel like?
Feeling of pressure/tightness all around the scalp
No associated symptoms common in migraine such as aura or photophobia
222
How is tension headache treated?
Explanation and reassurance
Analgesic withdrawal (to avoid overuse headache)
Tricyclic antidepressants in frequently recurrent cases
223
Migraine is a very common, debilitating headache disorder characterised into three types. Name them
1. Migraine with aura (classic migraine)
2. Migraine without aura (common migraine)
3. Migraine variants (unilateral motor or sensory symptoms often mistaken as a stroke)
224
What is the typical presentation of migraine?
Unilateral, throbbing associated with nausea, vomiting, photophobia
225
What cause visual auras?
Related to depression of visual cortex/retinal function and may preceed a migraine by minutes or hours
226
What are some of the potential symptoms of aura?
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Scotoma
Unilateral blindness
Flashes
Aphasia
Numbness
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227
Give a few differential diagnoses of migraine
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Meningitis
Subarachnoid haemorrhage
TIAs/stroke
228
How are mild migraines treated in the acute setting?
Simple analgesics and high-dose soluble aspirin/NSAIDs
Antiemetic if nauseous (e.g. metoclopramide)
229
How are moderate/severe migraines treated in the acute setting?
Triptans (e.g. sumatriptan)
230
What prophylaxis may be prescribed to prevent migraines?
Anticonvulsants e.g. valproate
beta-blockers - propranolol
Amytriptyline
231
What are trigeminal autonomic cephalgias?
Headaches characterised by unilateral trigeminal distribution of pain with ipsilateral autonomic features e.g. tearing
232
Name the types of trigeminal autonomic cephalgias
Cluster headache
Paroxysmal hemicrania
Hemicrania continua
SUNCT syndrome
233
What are cluster headaches?
Rapid onset, short-lived unilateral headaches with clustering of multiple attacks over a period of a few months before a period of remission
234
Describe the clinical presentation of cluster headaches
Pain often begins around the eyes and only lasts 1-2 hours
Autonomic features include tearing, rhinorrhoea and Horner's sydnrome
235
What vertebral levels does the spinal cord extend from and to?
C1 to L1
236
What structure continues from the spinal cord at L1?
Cauda equina
237
Paraplegias is almost always caused by a lesion in what anatomical location?
Spinal cord
238
Hemiparesis is almost always caused by a lesion in what anatomical location?
The brain
239
Outline the presentation of spinal cord compression
Progressive weakness of the legs with upper motor neurone pattern
240
What are the two patterns of onset for spinal cord compression?
Acute (hours to days)
| Chronic (weeks to months)
241
At what level are sensory symptoms felt in spinal cord compression?
Sensation diminishing one/two spinal levels below the level of the lesion
242
What symptoms are felt in the later stages of spinal cord compression?
Painless urinary retention and constipation
243
What is the most common cause of acute spinal cord compression?
Vertebral tumour (metastasis from brain, breast, lung etc.)
244
What is a frequent infective cause of spinal compression in some parts of the world?
Tuberculosis
245
Chronic compression of the spinal cord due to cervical spondylotic myelopathy is the most common cause of what disability in the elderly?
Spastic paraparesis
246
What is the differential diagnosis of spinal cord compression?
Intrinsic lesions of the spinal cord:
Transverse myelitis (inflammation due to viral infection)
Anterior spinal artery occlusion
Multiple sclerosis
247
What is cauda equina syndrome?
Spinal damage at the L1 level or below
248
Cauda equina syndrome can present acutely or chronically. What are the typical symptoms?
Back pain, weakness and numbness of the legs
Reduced reflexes
Saddle paraesthesia
Loss of sphincter control
Reduced desire to void urine progressing to painless urinary retention
249
What is Freidrich's ataxia?
An autosomal recessive disorder is the most common hereditary spinocerebellar degeneration causing ataxia, dysarthria and nystagmus
250
What is motor neurone disease?
Destruction of motor neurones and anterior horn cells
251
What is the prognosis of motor neurone disease?
Most patients die within three years from respiratory failure as a result of bulbar palsy and pneumonia
252
Motor neurone disease comprises of a mixture of four clinical patterns. What are they?
1. Progressive muscular atrophy
2. Amyotrophic lateral sclerosis
3. Progressive bulbar and pseudobulbar palsy
4. Primary lateral sclerosis
253
What are the features of progressive muscular atrophy?
Muscle wasting and fasciculations
254
What are the features of amyotrophic lateral sclerosis?
Progressive spastic tetra/paraparesis with lower motor neurone signs e.g. fasciculations
255
What are the features of progressive bulbar/pseudobulbar palsy?
Dysarthria, dysphagia with wasting and fasciculation of the tongue
256
What are the features of primary lateral sclerosis?
Progressive tetraparesis
257
What are the differential diagnoses of the motor neurone disease?
Cervical spine lesion (distinguished by the presence of sensory symptoms)
Idiopathic multifocal motor neuropathy
258
What is the medical treatment for motor neurone disease?
Riluzole (slows progression slightly)
259
What is dementia?
A clinical syndrome with multiple causes defined by a progressive acquired loss of higher mental function of sufficient severity to cause significant disability
260
What are the four different clinical entities of dementia?
Alzheimers disease (most common)
Vascular (multi-infarct) dementia
Dementia with Lewy bodies
Frontotemproal dementia
261
What is the presentation of Alzheimer's disease?
Insidious onset with slow disintegration fo personality and intellect.
Decline in language and visuospatial skills are common
262
How is Alzheimer's disease treated?
Acetylcholinesterase inhibitors (donezepril, rivastigmine and galantaime)
Memantine (NMDA receptor antagonist)
263
What is the typical pattern of progression of vascular dementia?
Stepwise deterioration
264
How is dementia with Lewy bodies characterised clinically?
Fluctuating cognition and Parkinsonism
265
What is the most prominent feature of frontotemporal dementia?
Early personality changes
266
What treatment must be avoided in dementia with Lewy bodies?
Neuroleptics
267
What is a mononeuropathy?
A process affecting a single nerve
268
What is mononeuritis multiplex?
A process affecting several or multiple nerves
269
What is carpal tunnel syndrome?
A mononeuropathy of the median nerve due to compression under the flexor retinaculum.
270
What are the clinical features of carpal tunnel syndrome?
Pain and paraesthesia in the affected hand, typically worse at night
271
How is carpal tunnel syndrome?
Nocturnal splints or local injections of steroids
Surgical decompression is definitive treatment (flexor retinaculum fasciotomy)
272
What is polyneuropathy?
Acute or chronic diffuse disease process involving motor, sensory and autonomic nerves.
273
What are the common potential sensory symptoms in polyneuropathy?
Numbness, tingling, pain in extremities, unsteadiness
274
What are the common potential motor symptoms in polyneuropathy?
Muscular weakness
275
What are the common potential autonomic symptoms in polyneuropathy?
Postural hypotension, urinary retention, erectile dysfunction, diarrhoea/constipation, diminished sweating, impaired pupillary response, arrhythmias
276
What are some common causes of mono neuritis multiplex?
Diabetes mellitus, malignancy, sarcoidosis
277
What is Guillain-Barre Syndrome?
Post-infective acute neuropathy causing inflammatory demyelination
278
What are the clinical features of Guillain-Barre Syndrome?
Progressive onset of distal limb weakness/numbness (usually symmetrical) that reaches its nadir in approx. 4 weeks
279
What are the clinical features of Guillain-Barre Syndrome?
Sensory symptoms - paraesthesia
Motor symptoms - progressive onset of distal limb weakness/numbness (usually symmetrical)
Autonomic symptoms - postural hypotension, cardiac arrhythmias, ileus and bladder atony
280
What is Miller Fisher Syndrome?
A rare variant of Guillain-Barre Syndrome that affects cranial nerves to the eyes causing ophthalmoplegia and ataxia
281
How is Guillain-Barre Syndrome?
Diagnosis is made clinically and can be confirmed after a number of weeks with nerve conduction studies
282
What is the differential diagnosis of Guillain-Barre Syndrome?
Other causes of neuromuscular paralysis:
Hypokalaemia, polymyositis, myasthenia gravis
283
Outline the management of Guillain-Barre Syndrome?
Vital capacity monitored 4-hourly to recognise deteriorating respiratory failure
Intravenous immunoglobulin (+/- plasma exchange)
Supportive treatment (heparin, physiotherapy, NG/PEG tube)
284
What is the most common cause of thiamin (vitamin B1) deficiency?
Chronic alcohol dependency
285
What pathology arises from pyridoxine (vitamin B6) deficiency?
Sensory neuropathy
286
What pathology arises from vitamin B12 deficiency?
Syndrome of subacute combined degeneration of the cord causing distal sensory loss
287
How does the presentation of muscular disease differ from the weakness caused by neuropathy?
In muscular disease reflexes and sensation are normal
288
What is myasthenia gravis?
An autoimmune disorder of the neuromuscular junction transmission characterised by weakness and fatiguability of ocular, bulbar and proximal limb muscle
289
What is the pathophysiology of myasthenia gravis?
Autoantibodies to acetylcholine receptors causing receptor loss
290
What is the presentation fo myasthenia gravis?
Fatigue muscle on sustained or repeated activity that improves after rest.
Ocular muscles are the first to be involved causing ptosis
Difficulty talking, swallowing and breathing follows
291
How is myasthenia gravis treated?
Anticholinesterases
Immunosuppression
Plasmapheresis and intravenous immunoglobulin
Thymectomy
292
What are the muscular dystrophies?
Inherited group of progressive myopathic disorders
293
What is the inheritance patterns of both Duchenne's and Becker's muscular dystrophies?
X-linked recessive
294
What is the presentation of Duchenne's muscular dystrophy?
Present in early adulthood with weakness in proximal muscles and grossly elevated creatine kinase (100-200x normal limit)
295
What is the presentation of Becker's muscular dystrophy?
Present later in life with a milder degree of clinical involvement than Duchenne's muscular dystrophy
296
What is myotonia?
Delayed muscle relaxation after contraction
297
What are the two most common myotonia disorders?
Dystrophia myotonica and myotonia congenita
298
What is dystrophia myotonica?
Autosomal dominant condition causing progressive distal muscle weakness, myotonia, facial muscles weakness and ptosis
299
What is myotonia congenita?
Autosome dominant condition causing myotonia exacerbated by rest and cold
Becomes evident in childhood
300
What is delirium?
Acute or subacute condition in which impairment of consciousness accompanied by changes in personality and mood
Severity can vary and often fluctuates
301
How is delirium managed/treated?
| 4
Investigation and treatment of the underlying condition
Withdrawing all possible drugs responsible (TCAs, opiates and benzodiazepines etc.)
Rehydration
Adequate pain relief/sedation
302
What are the most commonly prescribed hypnotics?
Benzodiazepines
303
What are the four most common side effects of a neuroleptic such as haloperidol?
Drowsiness, hypotension, tachycardia and convulsions
