Neuro Flashcards
(168 cards)
1
Q
what is status epilepticus?
management?
A
a single seizure lasting >5 minutes, or >= 2 seizures within a 5-minute period
use IV benzodiazepines such as diazepam or lorazepam - can repeat dose after 10-20 mins
if not responding 2nd line = phenytoin
if still not after 45 mins then general anaesthesia
2
Q
how is a SAH diagnosed?
A
CT head
but need to LP if negative - not all show on CT
3
Q
What are the general causes of loss of consciousness
A
Cardiovascular including Vasovagal syncope (most common)
Neurological
Psychogenic
4
Q
First line medication for seizures
A
Lamotrigine 25mg increased by 25mg every two weeks up to 75 mg
Note most common side effect is a hypersensitivity rash
5
Q
What features indicate syncope?
A
Positional
Provoking factors; dehydration, emotion, heat
Prodrome; dizziness and sweating
6
Q
Types of seizures
A
Generalised tonic clinic
Myoclonic jerks
Partial seizures
Absent seizures
Focal seizures
7
Q
Signs of raised ICP
A
Papilloedema
Peripheral field loss and blond spots
6th nerve palsy (other reasons too)
Hypertension
Vomiting
Headache
8
Q
Investigating headaches
A
CT
MRI
LP - measures CSF pressure
9
Q
Acute migraine treatment
A
NSAIDs
Antiemetics
Triptans
10
Q
Migraine secondary prevention
A
Beta blocker or amitriptyline
if unsuccessful can try antiepileptics or antihypertensives
11
Q
Investigations in meningitis
A
CSF sample via lumbar puncture
Blood cultures
Viral serology
Throat swab
MRI brain if encephalitis likely
12
Q
Meningitis treatment
A
3rd generation cephalosporin: cefotaxime or ceftriaxone
And dexamethasone
If over 60 add amoxicillin
13
Q
Treatment for temporal arteritis
A
Immediate oral prednisolone
14
Q
signs/symptoms of Wernicke’s encephalopathy
A
Confusion
Ataxia
Nystagmus
Ophthalmoplegia
PEripheral
Neuropathy
15
Q
TIA presents to GP within 7 days?
A
300mg aspirin and refer for specialist review
16
Q
focal seizures based on where they occur
A
temporal lobe - hallucinations, epigastric/emotional, automatisms, deja vu/dysphasia
frontal lobe - head/leg movements, jacksonian march
parietal lobe - parasthesia/sensory abnormalities
occipital - visual floaters and flashes
17
Q
cluster headache acute and prophylactic management
A
acute - 100% oxygen and subcut sumatriptan
prophylaxis - verapamil and steroids
18
Q
trigeminal neuralgia
symptoms + treatment
A
severe, unilateral electric shock pain in face/head precipitated by light touch
treat with carbemazapine
19
Q
what symptoms are common in syncope
A
presyncopal symtpoms
motor symptoms; twitching/jerking of limbs, tongue biting and incontinence can occur
20
Q
typical non-epileptic attack disorder history
A
- prolonged episodes
- conscious even with profound movements
- collateral history
- normal EEG/MRI etc
21
Q
typical cardiogenic syncope history
A
- sudden onset
- no warning
- short duraiton
- usualy middle aged or older
- CV history
22
Q
Broca’s aphasia
A
expressive aphasia where the patient’s speech is non-fluent and halting, repetition is impaired.
23
Q
Wernicke’s aphasia
A
fluent speech, impaired comprehension, and impaired ability to repeat back phrases.
24
Q
prophylaxis for contacts in meningitis
A
rifampicin
note meningitis is a notifiable disease
25
intracranial space occupying lesions
tumours
vascular - haemorrhages
infective
hydrocephalus
26
brain tumour presentation
raised ICP
neurological deficits
cortical/meningeal irritation
hormonal effects
27
causes of raised ICP
increased brain mass/volume
increase in CSF
increase in blood
28
what is cerebral perfusion pressure
CPP = ABP - ICP
29
management of acute ICP rise
ensure all routine measures; position, temperature, BP etc under control
- heavy sedation +/-paralysis
- CSF drainage
- osmotic therapy with mannitol
- hyperventilation
- barbiturate therapy
- decompressive craniotomy
30
how do extra dural bleeds usually present
usually following trauma resulting in an arterial bleed - commonly to side of head and involving middle meningeal artery
lucid interval may be present betwen trauma and patient deteriorating
31
how does a subdural bleed usually occur
venous injruy - may not be very traumatic
often in elderly, alcoholics, etc
haematoma usually develops very slowly
32
what are the NICE headache red flags
-Sudden onset high severity headache
-Headache with fever
-New onset neurological deficit
-New onset cognitive dysfunction
-Change in personality
-Impaired level of consciousness
-Recent head trauma (within past three months)
-Headache triggered by cough, sneeze, exercise, or changes in posture.
-Headache associated with halos around lights or headaches that get worse in the dark.
-Headache associated with jaw claudication and scalp tenderness.
33
what does increased tone indicate
what is the difference between rigidity and spasticity
increased tone indicates as UMN lesion
rigidity; increased tone that is not velocity dependent - indicates an extrapyramidal lesion such as in Parkinson's disease
spasticity;velocity dependent increase of tone which indicates a pyramidal lesion
34
what is the nerve root of each movement in the neurological examination
Shoulder abduction C5
Elbow flexion C5/C6
Elbow extension Radial nerve C7
Wrist extension Radial nerve C6
Finger extension Posterior interosseous nerve C7
Finger flexion C8
Finger abduction Ulnar nerve T1
Thumb abduction Median nerve T1
Hip flexion L1/L2
Hip extension L5/S1
Knee flexion Sciatic nerve S1
Knee extension Femoral nerve L3/L4
Foot dorsiflexion Deep peroneal nerve L4
Toe dorsiflexion L5
Foot plantarflexion S1
35
how do reflexes change in pathology
Reflexes are decreased or absent in LMN pathology, and exaggerated in UMN pathology.
36
what are the nerve roots of the reflexes tested
Biceps reflex C5-C6
Triceps reflex C7-C8
Supinator reflex C6-C7
Knee reflex L3-L4
Ankle reflex S1
37
what tracts are impaired in loss of
- vibration sensation
- pinprick sensation
vibration - dorsal columns
pinprick - spinothalamic tracts
38
what is the MRC grading scale for muscle strength
0) No power
1) Twitching but no movement
2) Movement, but cannot overcome gravity
3) Can overcome gravity
4) Movement against gravity and resistance
5) Normal muscle strength
39
what is brown-sequard syndrome
a hemisection of the spinal cord
40
what are the main investigations used in neurology
CT
MRI
LP
EEG
EMG
41
When is global areflexia seen
Sensory (+/- motor) poly neuropathy
If length dependent the lower limbs are lost before the upper limbs
42
What does asymmetric loss of reflexes indicate
Mononeuropathy, mononeuropathy multiplex or radiculopathy
43
What happens to reflexes in
- acute spinal cord lesions “spinal shock”
- Lambert Eaton Myasthenic Syndrome
- loss of reflexes
- lost but can return after voluntary sustained contraction of the responsible muscle
44
What is Pes Cavus
A sign of chronic neurological disease most commonly seen in Charcot Marie tooth disease. Can also be seen in cerebral palsy, nerve rauma and spinal cord tethering
Foot deformity of an elevated arch, forefoot drop and toe clawing, muscle actions
45
What is pseudoathetosis
Loss of sense of joint position causing slow involuntary movements usually of the arms
Caused by sensory ataxia
Can be seen in ataxic neuropathy, myelopathy, thalami and parietal lesions - ie anywhere along the sensory pathway
46
What is high stepping gait a sign of
peripheral sensory neuropathy
can be motor
47
What is myotonia? When is it seen
Prolonged muscle contraction once activated due to abnormal activity of the muscle fibre membrane
Seen mainly in myotonic dystrophy types 1 & 2
Can be elicited voluntarily (make fist) or by percussion of thenar eminence
48
What needs to be asked in a neurological systems review
Headaches
Fits faints funny turns
Memory
Vision
Hearing
Speech
Numbness, tingling
Weakness
Sensation
Incontinence or erectile dysfunction
Balance and coordination
49
Where do motor pathways decussate
What will lesions therefore show
Medulla
Proximal to medulla = contralateral
Medulla = ipsilateral CN signs and contralateral limb signs
After medulla = ipsilateral signs
50
Where do the
- dorsal columns
- spinothalamic tracts
decussate
Dorsal columns = medulla
Spinothalamic tracts = dorsal root ganglion
51
What are the main differentials for weakness in all four limbs
Acute peripheral neuropathy such as Guillan Barre syndrome
Acute myelopathy (acute spinal cord syndrome) eg transverse myelitis
(Both present with weakness and altered sensation
Reflexes most helpful to distinguish)
Myasthenia Gravis
Acute myosotis
52
What is the main treatment for guillian barre syndrome
Intravenous immunoglobulins
53
What information does a lumbar puncture give you and normal ranges
Opening pressure - <20cm
Blood glucose
WBC - <5 WBCs w/ no neutrophils
RBC - none but occasional blood contamination
Protein - <0.45g/dL
CSF glucose - approx 2/3 of plasma glucose
Appearance - clear
can also do gram stain, cytology, cultures, viral/bacterial PCR, oligoclonal bands which indicates IgG synthesis in the CNS, tests for xanthochromia
54
How can GBS affect
- CSF / LP results
- breathing
LP - increased protein due to albuminocytological dissociation in the CSF. Normal cell count, normal cells and all else
Breathing can be affected by lying flat as neuromuscular ventilatory failure occurs due to a weak diaphragm - shown by increased neck weakened, reduced chest expansion, CO2 retention
FVC can become markedly reduced - consider ICU
55
acute cervical cord syndrome
Mixture of 4 limb motor and sensory signs
May have pain
Many have sphincter symptoms e..g bladder urgency and frequency
Examination expected to show increased tone and brisk reflexes in all four limbs but often can have flaccid tone and absent reflexes - spinal shock
If only posterior cord may have no sensory symptoms - otherwise can find a sensory level with pinprick
Sensory ataxia often present - unsteady gait
56
Cauda equina syndrome
Lower limb lower motor neurone symptoms and signs
Normal upper limbs
Combination of motor and sensory problems, bladder disturbance, saddle anaesthesia
Pain may be present if due to compressive pathology
57
Myasthenia gravis symptoms
Muscle weakness is cardinal symptom
No sensory symptoms
Can affect any muscle but typically eyes, mouth and proximal limbs
Respiratory muscles can be involved especially when lying down due to weak diaphragm
Weakness is fatigable - worse the more the muscle is used ie end of the day
58
Acute myositis
Rare condition with weakness predominantly of the proximal limb
Myalgia may be present
Reflexes and sensation normal
59
Characterising features of:
1. Migraine
2. Cluster headache
3. Tension headache
4. Temporal arteritis
1. Unilateral, pounding, headache with multiple triggers. Lasts for hours. Aversion to lights, noises and can be preceded by aura
2. Occur frequently for a couple of weeks. 20 minutes of unilateral, debilitating pain with retro orbital pain, red eye and eye watering
3. Tight band like sensation triggered by stress
4. Scalp tenderness, unilateral, jaw Claudication
60
Characterising features of
1 trigeminal neuralgia
2 acute glaucoma
3 sinusitis
1 brief stabbing pain when brushing teeth or chewing
2 pain around eye, blurred vision with halos around lights
3 facial, tenderness and rhinorrhea
61
Characterising features of
1 meningitis
2 SAH
3 raised ICP
1 photophobia, neck stiffness, fever
2 sudden onset excruciating headache
3 headache triggered by positional change or exertion and changes in vision
62
Characterising features of collapse caused by
1. Hypertrophic cardiomyopathy/ cardiogenic syncope
2. Arrhythmia/ cardiogenic syncope
3. Aortic stenosis
4. Carotid sinus hypersensitivity
1. Triggered by vigorous exercise in a young person
2. Palpitations or chest pain beforehand - FHx of sudden unexplained death
3. Older person, chest pain, breahtlessness and collapse of exertion
4. Collapse on shaving or turning head
63
what are the defined illnesses within Guillan-Barre syndrome
Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) - most common
Acute Motor Axonal Neuropathy (AMAN)
Acute Motor and Sensory Axonal Neuropathy (AMSAN) - severe
Miller-Fisher Syndrome - ophthalmoplegia, arreflexia and ataxia
Pure Sensory Neuropathy
Acute Pandysautomia
all are acute or subacute monophasic illnesses - progress for up to 4 weeks acute or up to 8 weeks subacute
64
what is AIDP
Acute Inflammatory Demyelinating Polyradiculoneuropathy
begins with parasthesia and pains followed by muscle weakness usually beginning with the legs
can start in arms or face rarely
sometimes causes ophthalmaplegia
has autonomic features; labile BP, arrhythmia, constipation, abdominal distention
2/3 have an identifiable trigger
can't progress for longer than 4 weeks by definition
65
differential diagnoses of Guillan Barre
when examining ensure signs fit with LMN disease otherwise not GB!
differentials;
acute myelopathies
botulism
diphtheria
Lyme disease
Porphyria
vasculitis neuropathy
66
suspected GBS but...
significant protein elevation in CSF; >5g/dL
significant cell numbers in CSF
think impairment of CSF flow - spinal block e.g. epidural infection, disc disease or tumours
some cells normal (10-20) but if >50 think HIV seroconversion which can present similarly or alternative diagnosis
67
what is the pathophysiology of GBS?
Peripheral neuropathy
Demyelinating form; immune attack; macrophage mediated demyelinating disease of the peripheral nerves
Axonal disease particularly at the nodes of Ranvier by plasmalemma
both thought to be caused by a combination of macrophage activity via T cell pathway and of antibody mediated attack via plasma B cells
note in AMSAN there is severe Wallerian degeneration which impairs full recovery
68
how does GBS generally present?
LMN signs
- Arreflexia
- Reduced tone
- reduced power if motor involvement
- reduced sensation if sensory involvement
usually ascending
progresses over days -weeks
80% make a full recovery but long process with physio, OT and neurorehab
69
management for GBS
- prophylaxis for DVT/PE; LMWH
- monitor respiratory function using FVC; if declining pt may be peri-arrest; ventilate if severely reducing
- IV immunoglobulins
- plasma exchange (equivalent outcomes for IVIG)
- oral steroids not effective
70
what can trigger GBS?
Viral infections; EBV, CMV, HIV, influenza
Bacterial infections; campylobacter (stands out as a trigger), mycoplasma pneumonia, E coli
Systemic illnesses; Hodgkins lymphoma, CLL, hyperthyroidism
Others; pregnancy, surgery, bone marrow transplant
71
why is campylobacter infection a particular trigger for GBS?
shared epitopes on the bacteria lipo-oligosaccharides with the human gangliosides (abundant cells in neuronal membrane) - target for antigenic attack as body tries to fight infection
some genetic variability in susceptibility
72
what are nerve conduction studies used for
highly sensitive in detecting peripheral nervous system disease, allow the precise anatomical localisation of the lesion, and characterise the pathology
sensory and motor studies measuring conduction speed and amplitude,
73
what presentations are LP useful in
- headache particularly measuring pressure
- neurological infection e..g meningitis
- inflammatory NS conditions e.g. transverse myelitis
- GBS
- Cancer of the CNS
74
CSF analysis results in
- bacterial infection e.g. meningitis
- viral infection
- TB
- fungal infection
bacterial - high opening pressure, high protein >1, v high cell count with neutrophils, low glucose
viral - normal opening pressure, slightly raised protein(0.5-1), raised cell count (lymph), normal glucose
TB - v high opening pressure, slightly raised cell count (lymph), high protein 1-5g/dL, low glucose <1/3
fungal - v high opening pressure, raised cell count (lymph), raised protein (0.5-2), slightly low glucose
75
CSF in
-MS
-cancer
-SAH
MS; usually normal except in extremely active disease can have 10-20 white cells
oligoclonal bands present
cancer; high WBC and protein, low glucose, abnormal cytology
SAH; RBCs (less sensitive) and xanthochromia; starts after a few hours
76
what are CMAPs and SNAPs in nerve conduction studies
CMAP compound muscle action potential
SNAP sensory nerve action potential
77
chronic inflammatory demyelinating polyneuropathy
considered the chronic version of GBS
progressive weakness and impaired sensory function in the legs and arms
treated with IV IG
78
how does a Bells palsy present?
cause?
treatment?
prognosis?
one sided facial weakness of whole side of face ie. no forehead sparing
sensation in tact
may have hyperacusis, taste change, dry eyes (functions of CNVII)
cause unknown; potentially viral
-if treated early steroids are effective; 1 week of prednisolone 60mg o.d
-advise patient to tape eye closed to avoid corneal damage
facial weakness can worsen over the first few days. The majority recover completely: most cases recover within 4-6 months. The time taken to recover relates to the severity of the initial weakness. Recurrence is uncommon
79
what is Ramsay Hunt syndrome
Herpes zoster (“shingles”) of the geniculate ganglion of the facial nerve
typically associated with painful vesicles affecting the external ear and occasionally on the palate
80
common peroneal nerve palsy
background function of the CPN
foot drop with weakness
typically painless, the ankle reflex is preserved & often occurs after a period of unaccustomed activity involving prolonged kneeling
The CPN winds round the fibular head and is susceptible to compression at this site. The CPN innervates tibialis anterior, responsible for ankle dorsiflexion, and the peroneal muscles which evert the foot, but not tibialis posterior (tibial nerve), responsible for inversion. It does not subserve the ankle reflex, and hence this is spared.
81
what is the commonest cause of a root lesion (radiculopathy) leading to foot drop
vertebral disc prolapse leading to compression of the L5 nerve root
The L5 root contributes a significant number of fibres to tibialis anterior, peroneal muscles and tibialis posterior and hence there is weakness of both inversion and eversion of the foot.
usually also involves S1 to some extent so ankle reflex
82
causes of a foot drop
lumbar nerve root lesion
common perineal nerve palsy
83
what will nerve conduction studies show in CPN palsy
severe compression can be associated with axonal loss which will show denervation changes on EMG
most are demyelinating and may show slower conduction but usually fully recover
84
presentation of a radial nerve palsy
compression of the radial nerve against the mid-shaft of the humerus
causes wrist drop
neurophysiology can be helpful, to confirm the diagnosis and add prognostic information, and a wrist drop splint can greatly improve hand function pending recovery.
85
degenerative cervical myelopathy (DCM)
what is it
symptoms
diagnosis
treatment
most common form of spinal cord dysfunction in adults
age-related osteoarthritic changes cause narrowing of the cervical spinal canal, leading to chronic spinal cord compression and neurologic disability.
symptoms
pain
loss of motor function - especially dextrous function
loss of sensory function
loss of autonomic function
MRI gold standard for diagnosis
decompressive surgery
86
what drugs can exacerbate myasthenia gravis
lots of different drugs - be careful prescribing
don't need to learn but examples:
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
87
what cranial nerves are affected by a vestibular schwannomas
what are the classical symptoms
CNs V, VII and VIII
vertigo, sensorineural hearing loss and tinnitus
typically arises from cells of the VIII nerve at the cerebellopontine junction and later involves CNV (absent corneal reflex) and CNVII (facial palsy)
requires urgent ENT referral
88
in what patient group should triptans be avoided as treatment for cluster headaches
people with a history of coronary artery disease
89
idiopathic intracranial hypertension
- presentation
- management
young, overweight females at risk
presents with headache, blurred vision, papiloedema, possible 6th nerve palsy
management:
advise weight loss
diuretics e..g acetazolamide
repeated LP to reduce pressure
90
What do you need to know from a patient after a loss of consciousness collapse
Are they well? Illness can precipitate a seizure
What was she doing at the time? Thinking vasovagal
Any warning or aura
First memory on recovery - after syncope memory is usually retained
Pain, injuries, incontinance, tongue biting - more towards epileptic
Previous attacks?
Other neurological symptoms now or ever
91
What do you need to know from a witness after a LoC collapse
Any warning? Pale sweaty nauseous points to syncope. Twitching, blankness, eye movement points to seizure
Fall stiffly or floppily? Stiff more likely seizure. Floppy more likely faint
Shaking? Brief myoclonus jerks syncope. Rhythmic clinic jerks seizure
Cyanosis? More in seizures
Duration? In syncope usually less than a minute seizure linger
Duration of shaking? Prolonged could be NEAD
Condition on recovery
92
Immediate assessments and investigations after a suspected seizure
Vital signs, oxygenation and BM
ECG - cardiac rhythm disturbance?
Neurological examination
Bloods for acute metabolic disturbances e.g. hyponatraemia, hypocalcaemia
CT head usually done
93
What is a complex partial seizure / focal impaired awareness seizure
Seizure activity confined to one area of the brain
Awareness is impaired without total LoC and may have partial recollection of events
Often staring blankly
94
What is a focal aware seizure
Confined to one area of the brain e.g. twitching of the face and arm but the patient is fully aware
Much less common than focal impaired awareness
95
What can focal seizures progress to
Can spread to whole brain and develop into a generalised tonic clinic seizure
Patient will then totally lose consciousness and begin to display features of a GTCS
96
What symptoms are typical of a partial seizure of the temporal lobes
Auditory symptoms - includes primary auditory cortex
Deja vu - temporal lobe important in memory
Bizarre psychic phenomena e.g. derealisaiton and depersonalisation
Automatism e.g. picking, lip smacking, repetitive mumbling
97
What factors can be important to ask about to identify underlying causes for epilepsy
Birth history - ask about prematurity, delivery
Childhood milestones or developmental delay
Seizures in childhood or infancy
Significant head injury
History of CNS infection
FHx of epilepsy
Medications
Recreational drugs and alcohol
98
What are febrile convulsions?
Common in early childhood - seizure activity that occurs associated with a high fever
Normally brief convulsions however some can be prolonged activity
Typically occur around 18 months
99
How do febrile convulsions relate to later developing epilepsy
Most children with a single and uncomplicated convulsion do not go on to have epilepsy
Some do lead to epilepsy later in life usually with seizures originating in the temporal lobes and associated with scarring in this area - Mesial Temporal Sclerosis
Risk factors for going on to have epilepsy include prolonged and several febrile convulsions, additional features such as transient hemiparesis, FHx epilepsy, convulsions occurring at an atypical age or with only a mild pyrexia
100
When do serum anticonvulsant levels need to be measured
Phenytoin is the only one that needs monitoring with close correlation between serum level and efficacy
Others only need measuring if concern about toxicity or overdose, concordance, absorption and a drug interaction causing a drop in levels
101
What group should sodium valproate be completely avoided in and why
Any women of childbearing agents
Major congenital defects - especially neural tube defects; spina bifida and anencephaly
Syndrome of dysmorphia, developmental delay and cognitive impairment;t known as foetal valproate syndrome
102
Can people with epilepsy ever be medication free?
Some patients who have been seizure free for several years especially those who had childhood epilepsy may be considered to withdraw from anticonvulsants
103
What are the issues with switching anticonvulsant medications
Avoiding breakthrough seizures - gradually reduce one whilst gradually increasing the other I.e. taking two drugs for several weeks at adjusting doses
Tolerability of new drug
Potential interactions between the two during changeover period or interaction with any other drugs taken particularly OCP
implications for driving - breakthrough seizure means stopping driving for at least six months and informing DVLA. Either way DVLA recommends stopping driving for six months whist changeover occurs
104
What are the common side effects of lamotrigine and the serious side effects
Common dose related: sedation, dizziness, nausea, insomnia
Rarer and serious: allergic skin rash, multisystem hyeprsensitivity reaction. Rapid medical attention and discontinuation needed
105
Risk of lamotrigine in pregnancy
No AED can be said to be completely safe in pregnancy
Lamotrigine at 200mg or less a day is said to be minimal risk and out balances risk of seizures to mother and foetus
Take folic acid 5mg daily as soon as contraception stopped and continue throughout first trimester
106
What factors can reduce seizure threshold
Current illness eg chest infection or UTI causing pyrexia and raised inflammatory markers
Missing medication
New medication that reacts with AEDs to lower seizure threshold eg tramadol, amitriptylline
Alcohol excess
Recreational drugs
Metabolic disturbance inc hypoglycaemia and hyponatraemia
Broken sleep, fatigue and jet lag
GI disturbances which may impair absorption
107
Driving implications after a breakthrough seizure
Tell DVLA
License removed
Need to be seizure free for a year for return of license
108
Doctors duty if patient does not want to inform DVLA
Make all efforts to persuade pt to inform DVLA about event seizure - personal, family and public safety
Explain to pt that they will have to break confidentiality and inform the DVLA if not
Record all encounters in medical records
Discuss with medical defence organisation before informing DVLA
109
What is medically refractory epilepsy
How can it be managed
Seizures failed to come under control with at least two different anticonvulsant drugs at adequate tried doses
Each new drug less likely to be successful but some respond with 2 or more AEDs
This disorder increases risk if status epilepticus and of sudden unexplained death in epilepsy
110
Hen is epilepsy surgery considered
Where a well defined structural cause for seizures is apparent
In a part of the brain that resection may be possible
Further investigation required to assess possible benefit from surgery, without undue risk of damage and input of MDT
111
Treatment pathway for status eplipeticus
Benzodiazepine first line (repeated)
IV antipileltic second line eg IV phenytoin or sodium valproate
General anaesthesia third line
112
What is the DVLA advice after a first seizure
Inform DVLA and be seizure free for 6 months before driving
113
Where is a lesion that causes a relative afferent pupillary defect
How is it detected
Optic neuropathy - damage to CN II
Detected by a swinging light test - when moving light quickly between eyes pupil should remain constricted due to direct and consensual response
In a RAPD the dir3ft response is impaired so the pupil dilates when light shone into that eye
114
What is optic neuritis
An area of inflammation that damages the optic nerve
115
What blood tests should be done in optic nerve pathology
Serum B12 - optic neuropathy can mimic optic neuritis
Non specific inflammatory markers - CRP, ESR
Specific tests for autoimmune conditions - ANA, ANCA, dsDNA, ENA, anti cardio lipid antibodies, serum ACE levels - optic neuritis could be the first presentation of a multi system autoimmune disease such as SLE, granulomatosis with polyangitis, sarcoidosis
Serology for possible infective triggers - HIV, syphilis, hep B and C
Rare; aquaporin 4 antibodies which can result in an attack in specific parts of the CNS including optic nerves
116
What is the diagnosis of a single demyelinating episode before it can be MS
How can diagnosis of MS be made
Clinically isolated syndrome of CNS demyelination (shown on MRI)
Has to happen twice to be MS
If LP is done after first episode and shows oligoclonal bands then a diagnosis of MS could be made immediately
Can also consider scheduled MRI to look for demyelination lesions over time
Clinical attacks of inflammation within the CNS at different times and in different places
117
Where can ataxia arise from?
Cerebellar ataxia - cerebellum and it’s connections in the brain
Sensory ataxia - dorsal columns OR peripheral nerves / nerve roots
Vestibular ataxia - vestibular apparatus and its connections
118
How can types of ataxia be distinguished from each other
Vertigo and nausea - main presenting symptom in vestibular and made worse with head movements. Often associated with hearing loss and/or tinnitus. V & N can also be present in cerebellar ataxia so ask about coordination in arms and if one side worse .e.g in cerebellar stroke
Sensory disturbance or loss - numbness, pins and needles in limbs or trunk. May be aware balance worse in the dark or closing eyes in shower (Romberg). May additionally have Lhermittes sign(pain on neck flexion) or tight sensation around trunk
Bladder disturbance - sensory ataxia due to a SC lesion in often associated with bladder urgency and frequency. Rarely present due to sensory ataxia from the peripheral nerves so think dorsal columns/SC lesion if bladder disturbance
119
What are the potential risks of high dose steroids
GI irritation - give PPI cover
Agitation, restlessness and insomnia
Rarely a steroid psychosis can occur
Hyperglycaemia usually with chronic steroid use but can rarely happen acutely
Very rarely avascular necrosis of the hip can occur with short term high dose steroid use
120
How are high dose steroids given for MS relapse
1g methylprednisolone IV in the morning for 3 days in an inpatient setting
As an outpatient can give methylprednisolone 500mg daily for 5 days
121
What tests are needed before giving high dose steroids
Ensure no signs of systemic infection before giving steroids which are immunosuppressant - WCC, CRP
FBC, renal function and random glucose along with dipstick for UTI
122
What is secondary progressive MS?
How is treatment approached?
Begins relapsing remitting but then transforms to progressive disease - atonal degeneration and loss of nerve cells with minimal inflammation
Once progressive then disease modifying drugs no longer use of and treatment switches to symptomatic relief including for spasticity e.g. baclofen, neuropathic pain e.g. gabapentin, depression e..g sertraline, urinary symptoms e.g oxybutynin
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What are the range of treatments used for neuropathic pain
Anticonvulsants are often used
Pregabalin or gabapentin are effective
Tricyclics antidepressants but note anticholinergic side effects
Trigeminal neuralgia can be managed with carbamazepine or oxcarbazepine
SNRI duloxetine can be helpful
Sometimes nerve blocks, epidurals and surgical options considered
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Types of MS
Relapsing remitting - most common. Acute attacks followed by remission
Secondary progressive - relapsing remitting deteriorating to have signs and symptoms between relapses. 65% of RR patients go on to SP within 15 years
Primary progressive - progressive from onset without remission. More common in older people
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when do NICE guideline recommend starting anti-epileptics
normally after a second seizure but some circumstances after a first seizure...
- pt has remaining neurological deficit
- brain imaging shows a structural abnormality
- EEG shows unequivocal epileptic activity
- patient or their family or carers consider the risk of having a further seizure unacceptable
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What are the common presenting symptoms of MS
Optic neuritis - blurred vision, pain on movement, colour desaturation
Cerebral - fatigue, weakness, sensory disturbance
Brain stem symptoms - vertigo, slurred speech, ataxia, in coordination, double vision
Spinal cord symptoms - weakness, sensory disturbance, autonomic dysfunction
127
How can grey matter involvement happen in MS
what is its significance
Neuronal loss and axonal transection can occur - grey matter
Irreversible - may be the primary driver of disability in MS
relevant to cognitive impairment and seizures
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What are the chronic plaques in MS compared to acute plaques
Chronic plaque are an expanding area of microglial cells around an inactive centre- cause of axonal damage
Acute plaques are macrophage mediated inflammation via antigens crossing the blood brain barrier and initiating an autoimmune response
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What are evoked potentials tests
Can be visual or somatosensory
Assess speed of transmission in optic nerve or in posterior columns of the spinal cord, the brain stem and the cerebral cortex
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What effects do disease modifying agents have in MS
Reduce MRI lesion activity
Reduce progressive brain atrophy
Reduce rates of elapses
Reduce sustained disability progression
May increase product of nerve growth factor
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What are the injectable disease modifying therapies for MS
Beta- interferons
- more frequent doses (IM weekly or SC multiple times a week)
Glatiramer acetate (copaxone)
- subcutaneous daily
These first 2 are considered first line therapies. Side effects few and include skin reactions, flu like symptoms and occasionally an acute reaction
Then…
Alemtuzumab (lemtrada)
- IV two courses
Natalizumab (tysabri)
- IV monthly
These two are considered higher efficacy
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What are the oral disease modifying therapies in MS
Dimethyl fumerate (tecfidera)
Teriflunomide (aubagio)
Fingolimod (gilenya)
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What is the link between alemtuzumab and other diseases
Other autoimmune diseases are increased by use of alemtuzumab including thyroid disease, immune thrombocytopenia and goodpasture syndrome which need treating
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What is the link between natazlizumab (tysabri) and the JC virus
JC virus is endemic in the population
But interaction of antibodies from previous infection with natalizumab can cause progressive multifocal leukoencephalopathy which has a 25% mortality rate
Link betwEen duration of treatment and risk
Need to test for antibodies of the JC virus in patients receiving treatment to assess risk and decide whether to change treatments/use a different option
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Where does the spinal cord terminate
L1
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What conditions can mimic MS
Anticardiolipin antibody system
Devics syndrome - neuromyelitis optica
Neurosarcoidosis
Sjögren’s syndrome
For the last two important to ask about systemic symptoms to distinguish from MS e.g rashes, fever, athropathy, weight loss or respiratory difficulties
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how does juvenile myoclonic epilepsy tend to present
unprovoked episodes of myoclonus (jerking) bilaterally in the upper and lower limbs which occur more often early in the morning or near bedtime (i.e. nearing sleep deprivation)
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Management of myasthenia Gravis
First line pyridostigmine - long acting acteylcholinesterase inhibitor
When not controlled start immunosuppressive - prednisolone
Thymectomy if associated thymoma
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Management of myasthenic crisis
Plasmapheresis
IV immunoglobulins
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Cerebellar symptoms
Dysdiodochokinesia
Ataxia
Nystagmus
Intention tremor
Slurred speech
Hypotonia
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where is the lesion in internuclear ophthalmoplegia
what condition is it most common in
in the medial longitudinal fasiculus; the communication between the CNIII and CNVI when looking left or right so causes nystagmus of one eye i.e. if L eye nystagmus when looking to left then INO on R side of MLF
most common in MS where a demyleinating lesion occurs here
142
what are the causes of cervical cord syndromes
inflammatory
- MS, post infective, neuromyelitis optics
- connective tissue diseases
compressive
- discs
- tumours
infective
metabolic
- B12 or copper deficiencies
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what are the vascular causes of a sudden onset hemiparesis
ischameic stroke
primary intracerebral haemorrhage
extra-cerebral haemorrhage (sub-arachnoid, sub dural, extra dural)
venous sinus thrombosis
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what are the vascular causes of a sudden onset hemiparesis
ischameic stroke
primary intracerebral haemorrhage
extra-cerebral haemorrhage (sub-arachnoid, sub dural, extra dural)
venous sinus thrombosis
143
what are the vascular causes of a sudden onset hemiparesis
ischameic stroke
primary intracerebral haemorrhage
extra-cerebral haemorrhage (sub-arachnoid, sub dural, extra dural)
venous sinus thrombosis
144
what are the symptoms of a third nerve palsy
dilated pupil (mydriasis)
ptosis
abdcuted eye
145
what is the treatment for a focal seizure
lamotrigine or levetiracetam
146
what is Charcot Marie tooth disease
an inherited peripheral neuropathy affecting sensory and motor nerves
usually inherited in an autosomal dominant pattern
presents with LMN signs typically in first 2 decades of life
147
A-E causes of peripheral neuropathy
Alcohol
B12 deficiency
Cancer and CKD
Diabetes and Drugs
Every Vasculitis
148
What is Lambert Eaton Myasthenic Syndrome
typically occurs in patients with small-cell lung cancer
similar presentation to myasthenia gravis but symptoms temporarily improve initially with exercise
149
what is neurofibromatosis
inherited condition that causes neuromas to develop throughout the nervous system
type 1 more common than type 2
many neurological symptoms and associations
cafe-au-lait spots on the skin
bony dysplasia
yellow brown sots on the iris
axillary freckling
note type 2 is most associated with acoustic neuromas especially bilateral
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what is tuberous sclerosis
generic condition that causes harmatomas (benign growths) in multiple systems affecting the skin, brain, lungs, heart, kidneys and eyes
associated with epilepsy and developmental delay
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what group of patients with TIA should be admitted immediately for assessment
on warfarin/a DOAC/ or has a bleeding disorder and they are suspected of having a TIA, they should be admitted immediately for imaging to exclude a haemorrhage
152
what are the first line treatments for neuropathic pain
amitriptyline, duloxetine, gabapentin, pregabalin
153
what is first line for trigeminal neuralgia
carbamazepine
154
trochlear nerve palsy
Defective downward gaze and vertical diplopia
patient tilts their head to the opposite side
eye deviates supero-laterally
155
what is syringomyelia
a collection of cerebrospinal fluid within the spinal cord
presents with loss of sensation to temperature but the preservation of light touch, proprioception and vibration - classically "cape" like distribution
UMN signs
diagnose with MRI
treat by treating cause e.g. tumour, trauma, or by draining with a shunt if persistent
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carpal tunnel syndrome
compression of the median nerve in the carpal tunnel
- parasthesia in thumb, index and middle finger
- pt shakes hand for relief esp at night
- wasting of thenar evidence
- weak thumb abduction
- tapping causes parasthesia (Tinel's sign)
- wrist flexion causes symptoma (Phalen's sign)
treat with corticosteroid injections and last resort surgical decompression
157
What is cataplexy
Strongly associated condition
sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened)
Around two-thirds of patients with narcolepsy have cataplexy
158
Link between lambert Eaton syndrome and exercise
Muscle weakness improves with exercise
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which anti epileptics affect the p450 system
carbamazepine - inducer
phenytoin - inducer
sodium valproate - inhibitor
160
causes of bilateral facial nerve palsy
Sarcoidosis
Guillian-Barre
Lyme disease
Bilateral acoustic neuromes - NF type 2
161
subacute combined degeneration of the spinal cord
- due to B12 deficiency
- dorsal columns and lateral corticospinal tracts affected
- proprioception and vibration lost first then parasthesia
- UMN signs develop
162
motor neurone disease presentation
can present with both upper and lower motor neurone signs
rarely presents before aged 40
163
drug used in MND
riluzole
used mainly in ALS - prolongs life by about 3 months
164
respiratory care and nutrition in MND
BIPAP at night
PEG feeding
165
what is spinal stenosis
symptoms/signs
pressure on spine from mass, trauma or spondylolisthesis
altered sensation below affected level
LMN signs at affected level
UMN signs below affected level
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cervical spondylosis signs
altered sensation below affected levele
LMN signs in the upper limbs
UMN signs in the lower limbs
