Neurology Flashcards
1
Q
xWhat is the treatment for Trigeminal neuralgia?
A
Carbamazepine
If no improvement - refer to neurology
2
Q
What are the branches of the Trigeminal nerve?
A
- Ophthalmic
- Maxillary
- Mandibular
3
Q
What is a marcus gunn pupil?
A
- found in MS
- a.k.a relative afferent pupillary defect (RAPD)
- Constriction of pupils of both eyes when the light stimulus is applied to the normal eye
2. Dilatation of pupils of both eyes when the light stimulus is rapidly transferred from the normal eye to the affected eye.
4
Q
Foot drop is caused by compression of which nerve?
A
common peroneal nerve
5
Q
Loss of sensation of big toe is caused by which nerve root being affected?
A
L5
6
Q
What nerve is mainly responsible for tongue movements?
A
Hypoglossal nerve
7
Q
Where is Broca’s area located and what is its function?
A
Frontal lobe - usually left
Motor function of speech
8
Q
How does a lesion in Broca’s area present?
A
BROKEN speech of Broca aphasia (speaks in short meaningful phrases)
9
Q
Where is Wernicke’s area located?
A
Temporal lobe - usually left side
(95% of people have a left dominant hemisphere)
10
Q
How does a lesion in Wernicke’s area present?
A
Long WORDY speech of Wernicke’s with no meaning
11
Q
A lesion in the cerebellum leads to symptoms on which sides of the body?
A
Lesions of the cerebellum affect the ipsilateral side of the body.
12
Q
What are the clinical features of idiopathic intracranial hypertension?
A
- young obese females
- headache - worse in the morning and at night - improved by standing
- nausea and vomiting.
- may also complain of pulsatile tinnitus (described as whooshing or buzzing)
- diplopia and deteriorating vision, eventually leading to blindness if not treated.
- On funduscopy- papilledema.
- Computed tomography (CT) scans will reveal no mass, but sometimes small slitlike ventricles and an empty sella sign can be seen.
- Diagnosis is usually made by measuring the opening lumbar puncture (LP) pressure, which is high
13
Q
What is the treatment for idiopathic intracranial hypertension?
A
LP to drain excess CSF
Acetazolamide may help to reduce CSF production
Weight loss
14
Q
What is the defect that leads to neurofibromatosis type 1?
A
Autosomal dominant mutation of neurofibromin (NF1 gene) on chromosome 17, which is a tumor suppressor gene (that inhibits p21 ras oncoprotein). This mutation leads to uncontrolled cell pro- liferation.
NF1 is more common (90% of all neurofibromatosis is type 1)
15
Q
What are the main clinical features of NF1?
A
- Neurofibromas - skin lumps - can be small or large
- axillary and groin freckling
- café au lait spots - flight, light brown patches usually seen from birth
- Lisch nodules - iris hamartomas - lumps on the iris that don’t affect vision
- optic gliomas - can affect vision
- epilepsy
- learning difficulties
16
Q
What is the defect that causes neurofibromatosis type 2?
A
Autosomal dominant mutation of merlin on chromosome 22, which is also a tumor suppressor gene.
17
Q
What are the main clinical features of neurofibromatosis type 2?
A
Bilateral acoustic neuromas (schwannomas)
Think NF type 2, on chromosome 22, leads to 2 acoustic neuromas
18
Q
What is the cause of an extradural haematoma?
A
Associated with temporal bone skull fracture
typically due to direct trauma to the pterion - the weakest part of the skull located on the temporal bone - resulting in rupture of the middle meningeal artery
This could happen for example in an RTA
19
Q
What are the classic features of extradural haematoma?
A
TALK AND DIE
- lucid period followed by rapid deterioration as the bleeding expands and can lead to transtentorial herniation
- CT: “Biconvex” lens that does not cross suture lines
20
Q
What is a subdural haematoma?
A
Venous bleeding between the dura and arachnoid membrane due rupture of small bridging veins
21
Q
Subdural haematoma is seen more commonly in which groups of people?
A
babies (shaken baby syndrome)
Alcoholics
Elderly following a fall
22
Q
What are the clinical features of subdural haematoma?
A
due to it being a slow venous bleed the onset of symptoms can be delayed and go on for longer before they present
- headache
- confusion
- drowsiness
- limb weakness or speech disturbance
CT head shows crescent shaped appearance of the bleeding, DOES cross suture lines
23
Q
What is the cause of a subarachnoid haemorrhage?
A
- rupture of cerebral aneurysms, especially berry aneurysms, causing bleeding into subarachnoid space
- can be due to aneurysm that has spontaneously developed in the person’s life) or associated with underlying conditions such as
polycystic kidney disease or connective tissue diseases like Marfan’s syndrome or Ehler Danlos syndrome - Increased risk in smokers and hypertension
24
Q
How does SAH present?
A
- sudden onset severe headache - thunderclap headache
- typically occipital
- worst headache I’ve ever had
- there may be a history of recent headache “sentinel headache”- warning leak
- vomiting, collapse
- seizures, coma
- CT head: blood in the cisterns and filling of blood along the sulci and fissures.
- If CT is negative you need to do LP - positive for xanthochromia (bilirubin breakdown from RBCs)
25
What are upper motor neurone signs
- hypertonia
- spastic paralysis
- hyperreflexia
- Babinski positive
(reflexes are uninhibited by UMNs)
26
what are lower motor neurone signs?
- hypotonia
- flaccid paralysis
- hyporeflexia
- muscle atrophy
- fasciculations
27
What is the most common type of motor neurone disease (MND)?
ALS amyotrophic lateral sclerosis
28
What are the clinical features of MND?
- rapidly progressive, fatal disease
- mixture of upper motor AND lower motor signs
- autonomic nervous system and cognition are spared
- muscle weakness
- muscle wasting/atrophy - thenar eminence wasting, tongue wasting
- fasciculations
- spasticity (hyperreflexia)
- dysphagia
- dysarthria
- most common cause of death is respiratory depression
29
what is the cause of subacute combined degeneration of the cord
B12 deficiency causing demyelination
Affects the dorsal column and lateral corticospinal tracts
(dietary, pernicious anaemia)
30
What are the clinical features of subacute combined degeneration of the cord?
mixture of UMN + LMN + sensory signs
- symmetrical
- Romberg's positive (loss of proprioception and position sense)
- sensory ataxia (gait)
- brisk knee reflexes
- absent ankle reflexes
- babinski positive (upgoing planters)
-
31
What is a positive Babinski sign?
positive = abnormal
Big toe bends towards the face (dorsiflexion) and other toes fan out
also called "extensor plantar response"
Normal response: big toe should flex down (plantar flexion)
32
What is Romberg's test?
Patient closes their eyes for at leats 60 seconds
Romberg's test is positive if they are unable to maintain their balance
Indicates a problem with proprioception and usually a problem with dorsal columns
Positive in
- tabes dorsalis
- subacute combined degeneration of the cord
- brown-sequard syndrome
33
what is the pathophysiology of Huntington's disease
- Autosomal dominant
- Mutation of Huntingtin gene on chromosome 4
- trinucleotide repeat disorder - CAG is repeated an abnormal number of times
- exhibits anticipation
34
What is the triad of Huntington's disease?
1. early onset dementia
2. behaviour changes - depression, aggression, hypersexuality
3. choreiform movements
35
What are the differences between Huntington's and ALS?
Huntington's affects cognition whereas ALS usually doesn't
Hungtinton's gives you chorea which is characteristic
Huntington's is inherited while ALS is usually not
36
What is the pathophysiology of Parkinson's disease
Degeneration of dopaminergic neurones in the substantial nigra (in the basal ganglia) - not sure why these neurones die.
Dopamine needed in these nerves for control of motor movements
presence of Lewy bodies
Parkinson's = low dopamine in the brain
37
What are the tetrad of symptoms in Parkinson's?
1. Bradykinesia - slowness of initiating and executing movements
2. resting tremor - unilateral (pill-rolling tremor at rest)
3. rigidity - cog wheel
4. postural instability - poor balance and frequent falls
Other features
- Shuffling gait
- loss of facial expression - mask like face (hypomimia)
- hypophonia (weak, soft voice)
- dysphagia
- difficulty with fine motor skills - doing up buttons, writing (micrographia)
- difficulty turning over In beed
- sleep disturbance
Can develop dementia in the advanced stages
38
Parkinson's plus syndrome - multi system atrophy - what are the key features
- parkinsonism
- ataxia
- autonomic dysfunction
- difficult to distinguish from PD in the early stages but it progresses more rapidly
- unlike PD - does not respond to levodopa/carbodopa
39
Parkinson's plus syndrome - progressive supranuclear palsy (PSP) - what are the key features
- parkinsonism
- EYE PROBLEMS - cannot move the eyes vertically
- recurrent falls - they typically fall backwards due to "axial rigidity" (where in PD they fall forwards)
- less likely to have a tremor
- speech and swallowing are affected more commonly than PD
- progresses more rapidly than PD
40
What are the extra-pyramidal symptoms that can be caused by anti-psychotics?
These extrapyramidal symptoms (EPS) include
- acute dystonic reac- tions (muscle spasms, especially those of the neck [torticollis] and eyes [oculogyric crisis])
- akathisia (restless legs)- pseudoparkinsonism (bradykinesia, resting tremor etc)
- tardive dyskinesia (involuntary movements such as tongue dart- ing out of the corner of the mouth or lip smacking).
41
What is tardive dyskinesia?
extra-pyramidal symptoms typically caused by dopamine receptor antagonist drugs such as: metoclopramide, anti-psychotics - symptoms persist despite stopping medications for at least 1 month
Involuntary movements of tongue, lips, neck, trunk and limbs e.g. tongue smacking, tongue protrusion
42
What are the features of Lewy body dementia and how can you distinguish it from Parkinson's disease?
- visual hallucinations
- parkinsonism
- sleep disturbance
- dementia and extrapyramidal symptoms come on within 12 months of each other (unlike in PD when cognitive decline happens at least 12 months after and can happen many years later)
43
what is Friedreich's ataxia?
A trinucleotide repeat disorder
autosomal recessive mutation of frataxin gene on chromosome 9 - impairs the mitochondria
Affects the spinal columns and also the cerebellum
44
What are the clinical features of FA?
- muscle weakness
- staggering gait
- frequent falls
- loss of proprioception and vibration
- loss of deep tendon reflexes
- nystagmus
- dysarthria
- kyphoscoliosis
- pes cavus (high arch of the foot that does not flatten with weight bearing)
- cardiomyopathy
- diabetes
45
What is Lhermitte's sign?
Bending the head forward triggers an electric shock sensation down the neck and spine
46
What is Uhthoff phenomenon?
Worsening of neurological symptoms in MS in a warmer environement such as
- warm bath
- warm weather
- exercise - increases body temperature
47
Which part of the nervous system does MS affect?
Autoimmune neurological disorder affecting the CNS - demyelination + inflammation
Thus you get ONLY UMN symptoms
Also autonomic + sensory symptoms
48
What are the symptoms of optic neuritis
- sudden onset
- unilateral
- painful eye movements
- loss of vision - partial or complete - can be blurry vision
- loss of colour vision
49
What is Charcot's triad in MS?
1. nystagmus
2. intention tremor
3. stacatto speech
not pathogonomic for MS
50
What is internuclear ophthalmoplegia?
Seen in MS
lesion of the medial longitudinal fasciculus
you get inability to adduct one eye (the affected eye) with nystagmus in the other eye
51
What are the key features of facioscapulohumeral muscular dystrophy?
- presents age 10-20 years old
- muscle weakness starts in the face and progresses downwards - to shoulder, humeral muscles, abdominal muscles and then anterolateral compartment of the face
- progressive difficulty whistling or sucking through a straw or blowing up a balloon
- winging of the scapula
52
What is another term for Guillain Barre syndrome?
acute inflammatory demyelinating polyneuropathy
53
What is the pathophysiology behind GBS?
- autoimmune
- following an infection (usually by C.jejuni or CMV) - there is an autoimmune reaction on myelin of peripheral sensory and motor nerves
Cause LMN signs
54
How does GBS present?
- symmetrical
- ASCENDING muscle weakness starting in hands and feet, spreading towards the trunk
- bulbar CNs can be affected which can lead to dysarthria dysphagia, drooling
- serious risk of respiratory failure
- Hx of GI or respiratory infection in the past 30 days
*affects peripheral nerves so you get LMN Sx*
Sensory
- numbness and tingling spreading from hands and feet upwards
- loss of proprioception
- loss of reflexes
Autonomic
- bladder dysfunction
55
What is the most common trigger for GBS?
infection with campylobacter jejune
56
How do you diagnose GBS?
LP
57
What is the pathophysiology behind Myasthenia gravis
- autoimmune
- type 2 hypersensivity reaction
- autoantibodies against the post-synaptic acetylcholine receptors on muscle
58
What is the clinical presentation of myasthenia gravis?
- mostly facial muscles are affected
- fatiguebaility of facial muscles - symptoms worse with use/throughout the day
- bilateral ptosis (drooping of upper eyelid)
- diplopia due to weakness of eye muscles
-dysarthria
- dysphagia
- dysphonia
- myasthenia snarl
CAN affect muscles of limbs - especially shoulder girdle - may c/o difficulty lifting arms above their head
NO autonomic symptoms (unlike Lambert Eaton syndrome)
59
What malignancy is associated with myasthenia gravis?
Thymoma
60
how do you diagnose myasthenia gravis?
anti–acetylcholine receptor antibodies.
nerve stimulation tests
CT of the chest to rule out thymoma
61
what is the treatment for myasthenia gravis?
acetylcholinesterase inhibitors e.g. pyridostigmine - reduce acetylcholine breakdown in the synapse so there is more available
62
What is Lambert eaton syndrome
- autoimmune
- antibodies attack PRE-SYNAPTIC VOLTAGE GATED CALCIUM CHANNELS in the NMJ - thus reducing release of acetylcholine
- is a paraneoplastic syndrome highly associated with small cell lung cancer
63
What is the clinical presentation of Lambert eaton syndrome?
- unlike MG, less likely to involve facial muscles
- proximal muscle weakness - difficulty climbing stairs, difficulty getting out of a chair
- Unlike MG - symptoms improve with use
64
What is the treatment for lambert eaton syndrome?
Treatment with pyridostigmine and immunosuppressants is similar to MG. Treatment should also be aimed at any underlying malignancy.
65
What is Progressive multifocal leukoencephalopathy (PML)?
demyelinating disease caused by infection by the JC virus (John Cunningham virus)
Typically occurs in immunocompromised people - HIV/AIDs
66
Symptoms of depression
1) Low mood and 2)
Anhedonia are the 2 core symptoms
then you have:
Fatigue or loss of energy
Worthlessness/ excessive or inappropriate guilt
Recurrent thoughts of death, suicidal thoughts or actual suicide attempts
Difficulty concentrating
Psychomotor agitation ot retardation
Weight loss
Decreased libido
Insomnia or hypersomnia- early morning waking
67
What are the classic symptoms of wernicke's encephalopathy?
1. acute confusion
2. ataxia
3. opthlamoplegia / nystagmus
68
what are the typical features of Korsakoff syndrome?
- anterograde and retrograde amnesia
- confabulation
- hallucinations.
69
What is the cause of Wernicke's encephalopathy?
chronic alcoholism leading to thiamine deficiency (B1)
70
Before giving glucose to a malnourished patient with chronic alcoholism, what should you do?
You must replace thiamine before giving IV glucose as otherwise you can trigger wernicke's encephalopathy
71
What is beriberi syndrome?
Occurs due having a diet made up mostly of polished white rice - leading to thiamine deficiency
there are 2 types: wet and dry
wet - affects cardiovascular system
dry - affects nerves
72
What is pellagra?
A disorder caused by niacin (B3) deficiency
1. dermaitits
2. diarrhoea
3. dementia
4. if untreated can lead to DEATH
73
Pituitary adenoma commonly results in which visual defect?
Bitemporal hemianopia
74
What are features of antiphospholipid syndrome?
- recurrent PE/sDVTs
- stroke
- venous sinus thrombosis
- recurrent miscarriages
- presence of anti-phospholipid antibodies (such as lupus anticoagulant)
75
What is an important adverse effect of carbamazepine?
agranulocytosis and aplastic anaemia - monitor FBC
76
What are the CSF findings in bacterial meningitis?
- protein raised
- glucose low
- polymorphonuclear cells high (e.g. neutrophils)
77
What are the CSF findings in viral meningitis?
- protein is normal or midly rased
- glucose is normal
- high lymphocytes (mononuclear cells)
78
What is Kerning sign?
patient in supine position, hip and knee are flexed to a right angle, and then knee is slowly extended by the examiner. The appearance of resistance or pain during extension of the patient's knees beyond 135 degrees
79
What is Brudzinski sign
Positive if passive flexion of the neck results in reflex flexion of the hips and knees,
80
What are the symptoms of cluster headache?
- severe, excruciating unilateral orbital-supraorbital eye pain
- associated with autonomic symptoms, such as ptosis, miosis, conjunctival injection, rhinorrhea, lacrimation
- lasting 15 minutes to 4 hours or longer.
- A distinguishing feature is the regularity of the attacks (occurring at the same time each day), speculating a connection with circadian rhythm.
- It is often referred as “alarm clock HA” because of the ability to wake a person from sleep with regular timing.
- Present as very agitated during an attack e.g. pacing
- Generally, many patients respond to inhalation of 100% oxygen.
- IV sumatriptan
- It is also more commonly seen in men.
81
What is the first line anti-epileptic for tonic-clonic seizures?
sodium valproate - except for women of child bearing age
For females who are able to have children, offer lamotrigine or levetiracetam [unlicensed use] as first-line monotherapy.
82
What is conversion disorder?
Also called functional neurological disorder
neurological symptoms - motor or sensory - inconsistent with patterns of known neurologic diseases or other medical conditions
83
What is somatisation?
typically multiple symptoms that persist for many years
"medically unexplained symptoms" - no organic pathology
Patient preoccupied and stressed by their symptoms
not reassured by negative tests
84
What is pick's disease?
- fronto-temporal dementia
- presents as change in personality/ inappropriate behaviour
- difficulty with speech (can lead to primary progressive aphasia)
85
What is Creutzfeldt-Jakob disease (CJD)
Prion disease
Rapidly progressive - fatal <6 months
Dementia + myoclonus
Ataxia
86
What can you use to treat post-herpetic neuralgia?
Can use neuropathic pain agents such as amitriptyline
87
What is the genetic defect that causes Duchenne Muscular dystrophy?
X-linked recessive mutation of dystrophin gene (DMD)
Dystrophin needed for proper muscle structure - without it you get myocyte damage and cell death
88
What are the clinical features of DMD?
- affects mostly boys
- onset early childhood
- progressive muscle wasting - which occurs first in hips/legs
- unsteady gait - frequent falls
- waddling gait
- abnormal gait when walking (shoulder pushed back, belly forward to maintain balance)
- Pseudohypertrophy of calf muscles (calf enlargement due to being replaced by fat + fibrous tissue)
- Gower sign - using hands to push on their legs to stand
- wheelchair bound by around age 12
- high CK levels
89
How is Duchenne MD diagnosed?
muscle biopsy - absent dystrophin
90
What is the genetic defect behind myotonic dystrophy?
autosomal dominant defect of DMPK gene on chromosome 19
Trinucleotide repeat disorder
exhibits anticipation
91
How does myotonic dystrophy present
- presents in adulthood unlike DMD and BMD
- Unlike DMD and BMD it is a multi system disorder
- myotonia
- progressive muscle wasting
- facial muscles wasting - ptosis, temporal hollowing
- dysarthria hyper nasal speech
- cataracts
- testicular atrophy - infertility
- frontal balding
- diabetes, cardiac arrhythmia
92
What is the cause of Horner's syndrome?
Compression of the cervical sympathetic chain - typically by an apical lung cancer such as Pancoast tumour (but has other causes)
93
What are the symptoms of Horner syndrome?
symptoms occur IPSILATERAL to the tumour due to direct compession
RAMP:
- facial flushing
- anhidrosis (absence of facial sweating)
- miosis (pupil constriction)
- ptosis
94
What is tabes dorsalis?
- tertiary syphilis - occurs decades after initial infection
- degeneration of nerves in the dorsal columns
- particularly affects sensation + proprioception and vibration
95
What are the features of tabes dorsalis:?
- sensory ataxia - high stepping gait with the foot "stomping" the ground
- paraesthesia - lacinting pain (shooting/stabbing pains)
- argyll robertson pupil
- can develop Charcot Joint due to neuropathic osteoarhropathy
- Romberg positive
- absent deep tendon reflexes
- tabetic ocular crises
96
What is an argyll Robertson pupil?
Pupil does not constrict to light
but there is normal accommodation - constricts when looking at a near object
97
Symptoms of ocular motor nerve palsy? (CN III)
- Eye is "down and out"
- difficult adducting the eye
- difficulty elevating the eye
- ptosis of upper eyelid
- diplopia as eyes no aligned
Parasympathetic innervation of the pupil is not in the oculomotor nerve but runs alongside it
if pupil is normal - may be ischaemic in cause
If pupil is BLOWN (fixed, dilated) - this could be due to compression by a tumour or posterior communicating artery aneurysm compressing the parasympathetic fibres
98
Symptoms of Trochlear nerve palsy (CN IV)
supplies the SUPERIOR OBLIQUE muscle of the eye which introits and depresses the eye (looking down)
- vertical diplopia (double vision)
- at rest eye deviates upwards
- diplopia on downward vision - walking down stairs, reading a book
- they tilt their head to the unaffected eye to compensate
99
Symptoms of abducens nerve palsy? (CN VI)
Supplies the LATERAL rectus muscle which is responsible to eye abduction
- They can't abduct the eye
- Horizontal diplopia
- at rest eye is more medially position (unopposed medial rectus action)
100
What are the symptoms of Bell's palsy
(facial nerve inflammation is a LMN)
- unilateral facial paralysis - ipsilateral to the facial nerve that is affected
- INCLUDING THE FOREHEAD
- difficulty blinking on one side
- difficulty smiling on one side
- may need an eye patch to prevent the eye from drying out
101
What is the treatment for Bell's Palsy
- Oral prednisolone if they present within 72 hours
antivirals no longer recommended
102
What are examples of focal seizures?
- simple partial - consciousness intact
- complex partial - impaired consciousness
Note they can develop into SECONDARY GENERALISED seizures
103
What are examples of generalised seizures?
- tonic-clonic - most common type
- absence seizures
- myoclonic
- atonic seizures
104
How do atonic seizures present?
Drop attack - they collapse to the ground - loss of tone + loss of consciousness
105
What is hemiballismus?
Sudden wild/violent flailing of one arm due to contralateral sub thalamic nucleus lesion e.g. infarct/haemorrhage
106
What are the cerebellar signs?
DANISH
Dysdiadochokinesis
Ataxia
Nystagmus
Intention tremor
Staccato speech
Hypotonia
107
What is contained in the dorsal columns?
- SENSORY TRACT (ascending)
- contains sensory fibres carrying information about VIBRATION, PROPRIOCEPTION and DISCRIMINATORY TOUCH
- Does not cross over at the segmental level, it ascends upwards and decussates in the MEDULLA
108
What is contained in the spinothalamic tract?
- SENSORY TRACT
- ascending pain and temperature and crude touch sensory information.
- The axons ascend for just a few spinal cord segments before crossing in the spinal cord
109
What is contained in the corticospinal tracts?
MOTOR FIBRES - DESCENDING fibres supplying the contralateral side of the body
Lateral corticospinal tract 90% of the fibres - like the dorsal column - decussates in the medulla
Anterior corticospinal tract
110
Syringomyelia
A cystic cavity forms within the central canal of the spinal card - it expands segmentally but also longitudinally
it mainly affects the spinothalamic tract on both sides of the spinal cord - symmetrical loss of pain, temperature and pressure sensation
Dorsal column is usually spared because it does not cross the spinal cord at the segmental level -it ascends upwards and crosses over the brain stem - so vibration, proprioception and discriminatory touch are preserved.
As It expands it CAN compress the cell bodies of LMNs leaving the spinal cord and cause some motor symptoms at the level of the syrinx
111
What is the classic presentation of syrinogomyelia?
loss of pain and sensation in cape like distribution - affecting upper limbs and shoulders in particular
Can present with burns in fingers
112
What is the first line treatment for absence seizures?
Ethosuximide
113
What is the typical EEG finding in absence seizures?
EEG shows 3-Hz generalized spike and slow wave discharges.
114
What are the drug treatments for Alzheimer's disease?
1. anti cholinesterase inhibitors (more ACh available at the synapse)
- donepezil
- Rivastigmine
- Galantamine
used for mild to moderate AD
2. Non competitive NMDA receptor antagonists
- memantine
Used in severe AD or if they cannot take the above
These two class of drugs are not used together
115
