Neurology Flashcards
1
Q
What is an acoustic neuroma?
A
benign tumours of the Schwann cells surrounding the auditory nerve (vestibulocochlear nerve) that innervates the inner ear.
2
Q
Symptoms of acoustic neuroma
A
o Patient usually 40-60 yo o Vertigo (CNVIII) o Hearing loss – unilateral sensorineural hearing loss (CNVIII) o Tinnitus – unilateral (CNVIII) o Absent corneal reflex (CNV) o Facial palsy (CNVII) – if the tumour grows large enough to compress the facial nerve (NO FOREHEAD SPARING = LMN lesion) o Dizziness / imbalance o Sense of fullness in the ear
3
Q
Are acoustic neuromas unilateral or bilateral?
A
- Usually, unilateral
- Bilateral vestibular schwannomas = neurofibromatosis type 2
4
Q
What CNs are affected by acoustic neuromas?
A
cranial nerves V, VII and VIII
trigeminal, facial and vestibulocochlear
5
Q
IX for acoustic neuroma
A
- Audiometry: to assess hearing loss (sensorineural pattern)
- Auditory brainstem reflexes: results will be abnormal
- Brain CT/MRI: diagnosis of tumour
6
Q
Mx for acoustic neuroma and risks with the treatment
A
- URGENT ENT REFERRAL
- ENT management:
o Conservative – monitoring if there are no symptoms
o Surgery – to remove tumour
o Radiotherapy – to reduce tumour growth - Risks with treatment:
o Vestibulocochlear nerve injury – permanent hearing loss or dizziness
o Facial nerve injury – facial weakness
7
Q
Other names for acoustic neuroma
A
Vestibular schwannoma
Cerebellopontine angle tumours
8
Q
What is Bell’s palsy?
A
an acute unilateral facial nerve weakness or paralysis of rapid onset (less than 72 hours) and unknown cause.
The history and examination otherwise are unremarkable. Deficits in all facial zones equally that fully evolve within 72 hours.
9
Q
Cause of Bell’s palsy
A
Idiopathic
10
Q
Symptoms of bells palsy
A
- Single episode
- Unilateral lower motor neurone facial nerve palsy – involves upper and lower parts of the face
o Reduction in movement on the affected side
o Drooping of eyebrow and corner of mouth
o Loss of nasolabial fold - Rapid onset (less than 72 hours)
- Ear and postauricular region pain
- Difficulty chewing, dry mouth and change in taste
- Incomplete eye closure, dry eye, eye pain or excessive tearing
- Numbness / tingling in cheek + mouth
- Speech articulation problems, drooling
- Hyperacusis
- 3-6 months later – synkinesis (abnormal & involuntary synchronous movement of a facial region with reflex or voluntary movement in another facial region)
11
Q
Diagnosis of bells palsy
A
clinical diagnosis of exclusion
12
Q
Mx for bells palsy
A
• If patient presents within 72 hours of symptom onset = PREDNISOLONE:
o 60mg OD for 5 days, then reduce by 10mg daily
• Eye protection
o Lubricating eye drops
o Tape eye shut at night
o Sunglasses outdoors
• Antivirals (with prednisolone) may offer small benefit, discuss with specialist
13
Q
Complications of bells palsy
A
- Exposure keratopathy – eye pain, requires ophthalmology review
- Eye injury, corneal ulceration, vision loss
- Synkinesis – abnormal facial muscle contraction during voluntary movements
14
Q
Recovery from bells palsy
A
- Most patients fully recover over several weeks, can take 12 months to recover
- 1/3rd are left with some residual weakness
- Refer to a facial nerve specialist if there is no improvement after 3 weeks of treatment, there’s incomplete recovery 5 months after initial onset or there are any atypical features.
15
Q
What is a brain abscess?
A
a suppurative collection of microbes (most often bacterial, fungal or parasitic) within a gliotic capsule occurring within the brain parenchyma. Lesions can be single or multi-focal.
It is potentially life threatening
16
Q
Causes of brain abscesses
A
- Bacterial: strep pyogenes, strep milleri, staph aureus
- Fungal: aspergillus fumigates, candida albicans, cryptococcus neoformans
- Parasitic: toxoplasma gondii
17
Q
How do brain abscesses happen?
A
- Haematogenous spread through bloodstream
- From an infected adjacent area e.g., ears or sinuses
18
Q
Risk factors for brain abscess
A
- Sinusitis
- Otitis media
- Recent dental procedure or infection
- Recent neurosurgery
- Skull fracture
- Meningitis
- Congenital heart disease
- Endocarditis
- DM
- HIV or immunocompromised
- IVDU
19
Q
Symptoms of brain abscess
A
- Seizures
- Fever
- Localising signs
- Signs of increased ICP
- Signs of infection elsewhere
20
Q
Ix for brain abscess
A
- CT/MRI = ring enhancing lesion
- Raised WCC
- Raised ESR
21
Q
Mx for brain abscess
A
- Neurosurgery referral
- Antibiotics: CEFTRIAXONE
- Treat raised ICP
22
Q
Common cancers that metastasise to the brain
A
Lung
Breast
Renal cell carcinoma
Melanoma
23
Q
Symptoms of brain mets
A
- Increased ICP o Headache worse on waking, lying down, bending forwards, coughing and straining o Vomiting o Papilloedema o Reduced GCS - Seizures - Evolving focal - Subtle personality change
24
Q
Ix for brain mets
A
- CT/MRI brain
- Consider biopsy
25
What is cerebral palsy?
umbrella term referring to a non-progressive disease of the brain originating during the antenatal, neonatal or early post-natal period while brain neuronal connections are still evolving, that results in disorders of movement and posture development.
26
Prevalence of cerebral palsy
2 in 1000
27
Impairments seen in cerebral palsy
- Disorders of movement
- Disorders of posture
- Learning impairment
- Visual impairment and squint
- Hearing loss
- Speech and language difficulties
- Behavioural problems
- Epilepsy
28
Presentation of cerebral palsy
- Delayed motor milestones
- Abnormal tone in infancy
- Persistence of primitive reflexes
- Abnormal gait
- Feeding difficulties
- Other developmental delays e.g., language and social
29
What are the primitive reflexes
```
Moro's (startle) reflex
Rooting reflex
Sucking reflex
Tonic neck reflex
Grasping reflex
Stepping reflex
```
30
Causes of cerebral palsy
• Antenatal (80%)
o Cerebral malformation
o Cerebral dysgenesis
o Congenital infection
• Intrapartum (10%)
o Hypoxic-ischaemic encephalopathy (birth asphyxia)
```
• Post-natal (10%)
o Cerebral ischaemia
o Intraventricular haemorrhage
o Head trauma
o Hydrocephalus
o Non-accidental head injury
o Hyperbilirubinaemia (severe neonatal)
```
31
What are the 3 types of cerebral palsy?
Spastic
Ataxic hypotonic
Dyskinetic
32
Ix for cerebral palsy
1. Brain imaging – USS in neonates, CT or MRI
2. Congenital infection screen
3. Metabolic screen
33
Mx of cerebral palsy
MDT
34
What is hypoxic-ischaemic encephalopathy?
- Seen in neonates.
- Ischaemic damage to the brain causes a disturbance of neurological behaviour
- Areas most affected are the ‘watershed zones’ between the major arteries as they’re most susceptible to hypoperfusion.
35
How is hypoxic-ischaemic encephalopathy classified
mild (resolves over a few days)
moderate
severe (50% of mortality and 80% risk of cerebral palsy)
36
Symptoms of HIE
floppy after birth, seizures, irregular breathing, hypertonic
37
Ix for HIE
```
APGAR score
fetal umbilical artery pH <7
arterial base deficit >12
MRI brain
EEG
presence of multisystemic organ failure
```
38
Tx of HIE
therapeutic hypothermia (33-35°C for 72 hrs within first 6hrs of life) and supportive measures
39
What is chronic fatigue syndrome?
4 months + of disabling fatigue affecting mental and physical function more than 50% of the time in the absence of other disease which may explain the symptoms.
40
risk factors for chronic fatigue syndrome
Female
| Past psychiatric history
41
Symptoms of chronic fatigue syndrome
- Fatigue
- Sleep problems
- Muscle/joint pain
- Headaches
- Painful lymph nodes without enlargement
- Sore throat
- Cognitive dysfunction
- Physical or mental exertion makes symptoms worse
- General malaise
- Dizziness
- Nausea
- Palpitations
42
Ix for chronic fatigue syndrome
```
• Screening blood tests to exclude other pathology
o FBC
o U&E
o LFT
o Glucose
o TFT
o ESR
o CRP
o Calcium
o CK
o Ferritin
o Coeliac screen
o Urinalysis
```
43
Mx for chronic fatigue syndrome
1. CBT
2. Graded exercise therapy
3. Pacing
4. Low dose amitriptyline
5. Referral to pain management clinic
44
What is CNI, its function and foramen?
Olfactory
smell
Cribriform plate
45
What is CNII, its function and foramen?
Optic
Sight
OPtic canal
46
What is CNIII and its function
Oculomotor
Eye movements = superior, inferior and medial rectus & inferior oblique
Pupil constriction
Accommodation
Eyelid opening
47
What happens in CNIII palsy?
Palsy results in
- Ptosis
- Down and out eye
- Dilated fixed pupil
48
CNIII foramen
Superior orbital fissure
49
Causes of CNIII palsy
o DM
o Vasculitis e.g., temporal arteritis, SLE
o False localising sign due to uncal herniation through tentorium if raised ICP
o Posterior communicating artery aneurysm: pupil dilated + pain
o Cavernous sinus thrombus
o Weber’s syndrome – ipsilateral third nerve palsy with contralateral hemiplegia, caused by midbrain strokes
o Amyloid
o MS
50
CNIV name, function, palsy symptoms and foramen
Eye movement (Superior oblique)
Palsy = defective downward gaze = vertical diplopia
Superior orbital fissure
51
What is CNV, its function and 3 foramen?
Trigeminal
facial sensation and mastication
```
V1 = superior orbital fissure
V2 = foramen rotundum
V3 = foramen ovale
```
52
What happens in CNV lesions?
Lesions =
- Trigeminal neuralgia
- Loss of corneal reflex (afferent)
- Loss of facial sensation
- Paralysis of mastication
- Deviation of jaw to weak side
53
What is CNVI, its function, symptoms of palsy and foramen?
VI = abducens
Eye movement
- Lateral rectus
Palsy = defective abduction = horizontal diplopia
Superior orbital fissure
54
What is CNVII, its function, symptoms of palsy and foramen?
VII = facial
Facial movement
Taste anterior 2/3rds
Lacrimation
Salivation
- Flaccid paralysis of the upper and lower face
- Loss of corneal reflex (efferent)
- Loss of taste
- Hyperacusis
Internal auditory meatus
55
What is CNVIII, its function, symptoms of palsy and foramen?
VIII = vestibulocochlear
Hearing
Balance
Hearing loss
Vertigo
Nystagmus
Acoustic neuroma
Internal auditory meatus
56
What is CNIX, its function, symptoms of palsy and foramen?
IX = glossopharyngeal
Taste (posterior 1/3rd)
Salivation
Swallowing
Mediated input from carotid body & sinus
Hypersensitive carotid sinus reflex
Loss of gag reflex (afferent)
Jugular foramen
57
What is CNX, its function, symptoms of palsy and foramen?
X = vagus
Phonation
Swallowing
Innervates viscera
Uvula deviates away from site of lesion
Loss of gag reflex (efferent)
Jugular foramen
58
What is CNXI, its function, symptoms of palsy and foramen?
XI – accessory
Head and shoulder movement
Weakness turning head to contralateral side
Jugular foramen
59
What is CNXII, its function, symptoms of palsy and foramen?
XII – hypoglossal
Tongue movement
Tongue deviates towards side of lesion
Hypoglossal canal
60
Afferent and efferent limb of corneal reflex
```
Ophthalmic nerve (V1)
Facial nerve
```
61
Afferent and efferent limb of jaw jerk
Mandibular nerve
| Mandibular nerve
62
Afferent and efferent limb of gag reflex
glossopharyngeal nerve
| Vagal nerve
63
Afferent and efferent limb of carotid sinus reflex
Glossopharyngeal nerve
| Vagal nerve
64
Afferent and efferent limb of pupillary light reflex
Optic nerve
| Oculomotor nerve
65
Afferent and efferent limb of lacrimation reflex
```
Ophthalmic nerve (v1)
Facial nerve
```
66
What is dementia
a syndrome characterised by deterioration in cognition resulting in impairment in the activities of daily living. Cognitive decline often affects multiple domains.
67
Memory screening tools
```
o 10 point cognitive screener
o 6 item cognitive impairment test
o Abbreviated mental test score
o GP assessment of cognition
o Mini mental state examination (MMSE score <24 = dementia)
```
68
Bloods for impaired memory
```
o FBC
o U&E
o LFT
o Calcium
o Glucose
o ESR/CRP
o TFTs
o Vitamin B12
o Folate
```
69
causes of dementia
- Alzheimer’s disease
- CVS – multi infarct dementia
- Lewy bpdy dementia
- Huntington’s
- CJD
- Pick’s disease – atrophy of frontal and temporal lobes
- HIV
70
Symptoms of Alzheimer's disease
Progressive persistent global cognitive impairment. Short term memory impairment.
Irritable, behaviour/mood change. Apathy.
71
causes of Alzheimer's disease
Unknown
72
Risk factors for Alzheimer's disease
amyloid precursor protein gene mutations and old age
73
Ix for Alzheimer's disease
MMSE
| CT head
74
Pathology of Alzheimer's disease
Accumulation of B-amyloid peptide that results in progressive neuronal damage, neurofibrillary tangles, amyloid plaques and loss of ACh
75
Tx for Alzheimer's disease
Acetylcholinesterase inhibitors e.g. donepezil, rivastigmine, galantamine.
NMDA antagonist = memantine
76
Symptoms of vascular dementia
Sudden onset and stepwise deterioration
Cognitive impairment, motor disorders and changes in behaviour.
77
Cause of vascular dementia
Cumulative effect of many small strokes.
78
RFs for vascular dementia
HTN, DM, hyperlipidaemia, old age, TIA, AF, smoking, obesity, CAD
79
Ix for vascular dementia
MMSE
US of carotids
Cranial MRI – infarcts and extensive white matter changes
80
Mx for vascular dementia
There’s damage to grey and white matter from vascular causes
81
Tx for vascular dementia
Treat risk factors.
Antiplatelet therapy e.g. aspirin to prevent strokes/TIA
There is often co-morbid Alzheimer’s so then give a cholinesterase inhibitor
82
What are the 3 main subtypes of vascular dementia?
3 main subtypes:
- Stroke related VD
- Subcortical VD (small vessel disease)
- Mixed dementia – VD + alzheimer’s
83
Symptoms of frontotemporal dementia
Onset in middle age, before 65 yo.
Slow onset
Inappropriate social behaviour and motor deficits/akinetic Parkinsonism in later stages.
Relatively preserved memory.
84
types of frontotemporal dementia
- Pick's disease
- Chronic progressive aphasia (CPA) – non fluent speech
- Semantic dementia – fluent progressive aphasia
85
cause of frontotemporal dementia
Genetic component
86
ix for frontotemporal dementia
MMSE
| CT/MRI
87
pathology of frontotemporal dementia
```
Atrophy of frontal and temporal lobes.
Pick bodies (cytoplasmic inclusions of aggregated tau proteins – silver staining). Associated w Pick’s disease
```
88
tx for frontotemporal dementia
Cholinesterase inhibitors and memantine are usually not effective & can worsen symptoms.
Give SSRIs for depression
Give olanzapine for agitation, hallucinations, insomnia
89
symptoms of lewy body dementia
Dementia with extrapyramidal / Parkinson’s motor symptoms, visual hallucinations, falls, rapid eye movement sleep behaviour disorder
Cognition may be fluctuating – in contrast to other forms of dementia
90
ix for lewy body dementia
SPECT (called a DaTscan)– decreased occipital perfusion / metabolism
Clinical diagnosis
91
pathology of lewy body dementia
Cerebral atrophy.
Lewy bodies = alpha-synuclein-positive cytoplasmic inclusions in neurones which cause neuronal degeneration
92
mx for lewy body dementia
Can treat Parkinsonian symptoms with Parkinson’s drugs
Acetylcholinesterase inhibitors (donepezil) and memantine can be used
Avoid antipsychotics – can develop irreversible parkinson’s
93
important differentials for dementia that are possibly treatable
- Hypothyroidism
- Addison’s
- B12/folate/thiamine deficiency
- Syphilis
- Brain tumour
- Normal pressure hydrocephalus
- Subdural haematoma
- Depression
- Chronic drug use e.g., alcohol, barbiturates
94
how do cholinesterase inhibitors work and what are they used for?
- Reversible cholinesterase inhibition leads to increased Ach concentration = improves dementia symptoms
- 1st line = Alzheimer’s dementia (mild-moderate) & vascular dementia
- Can be used in some cases of Lewy body dementia, frontotemporal dementia and Parkinson’s disease
95
example cholinesterase inhibitors
donepezil, rivastigmine, galantamine
96
side effects of cholinesterase inhibitors
nausea, dizziness, insomnia, cholinergic crisis
97
when is donepezil CI
relatively CI in patients with bradycardia
98
What is memantine and when is it used?
- Used in moderate to advanced Alzheimer’s disease & vascular dementia
- Can be used in combination with cholinesterase inhibitors
- An NMDA antagonist = results in decreased glutamate-induced calcium-mediated excitotoxicity
99
Side effects of memantine
- Side effects = headaches, dizziness, confusion, hallucinations, seizures
100
What features make a patient more likely to have delirium than dementia?
- Impairment of consciousness
- Fluctuation of symptoms e.g., worse at night, periods of normality
- Abnormal perception – illusions and hallucinations
- Agitation, fear
- Delusions
101
What features make a patient more likely to have depression than dementia?
- Short history, rapid onset
- Biological symptoms e.g., weight loss, sleep disturbance
- Patient worried about poor memory
- Reluctant to take tests, disappointed with results
- MMSE – variable
- Global memory loss – dementia typically causes recent memory loss
102
Causes of delirium
```
Pain
Infection
Nutrition
Constipation
Hydration
Medication/Metabolic
Environment
```
103
Symptoms of delirium
```
memory disturbances (loss of short term > long term)
may be very agitated or withdrawn
disorientation
mood change
visual hallucinations
disturbed sleep cycle
poor attention
```
104
Mx of delirium
tx cause
modify environment
1st line sedative = haloperidol 0.5mg
2nd line = olanzapine
105
what neurological changes does diabetes typically cause?
peripheral neuropathy
Sensory loss, not motor loss
glove and stocking distribution
106
mx of diabetic neuropathy
Do the following when diabetic neuropathy is painful:
1st line = amitriptyline, duloxetine, gabapentin or pregablin
2nd line = try one of the other 3 drugs above
Rescue therapy for exacerbations = tramadol
pain management clinics
107
What are the neurological GI affects of diabetes?
Gastrointestinal autonomic neuropathy.
Get gastroparesis, chronic diarrhoea or GORD
108
what are the symptoms and mx of gastroparesis seen in diabetic gastrointestinal autonomic neuropathy?
symptoms = erratic BM control, bloating and vomiting
mx = metoclopramide, domperidone or erythromycin
109
what is encephalitis?
inflammation of the brain
110
causes of encephalitis
autoimmune
Most common = viral
- herpes simplex virus
- varicella zoster virus
- EBV
- enterovirus
- adenovirus
- influenza virus
- polio, mumps, rubella and measles
bacterial & fungal
111
presentation of encephalitis
```
Altered consciousness
Altered cognition
Unusual behaviour
Acute onset of focal neurological symptoms
Acute onset of focal seizures
Fever
```
112
diagnosis of encephalitis
LP - CSF for viral PCR
CT scan if LP is CI (GCS below 9, harm-dynamically unstable, active seizures or post-ictal)
MRI scan after LP
EEG
Swabs - throat and vesicle swabs to find cause
HIV testing
113
mx of encephalitis
IV acyclovir for HSV or VZV
IV ganciclovir for CMV
Repeat LP to ensure successful tx before stopping antivirals
114
complications of encephalitis
```
Lasting fatigue and prolonged recovery
Change in personality or mood
Changes to memory and cognition
Learning disability
Headaches
Chronic pain
Movement disorders
Sensory disturbance
Seizures
Hormonal imbalance
```
115
what is epilepsy and what are seizures?
umbrella term for a condition where there is a tendency to have seizures.
Seizures are transient episodes of abnormal electrical activity in the brain.
116
Ix for epilepsy
EEG
MRI brain - to diagnose structural problems & tumours
ECG
117
What are the types of generalised seizures?
```
tonic-clonic (grand mal)
tonic
clonic
typical absence (petit mal)
myoclonic: brief, rapid muscle jerks
atonic
```
118
What type of seizures involve a complete LOC?
Generalised seizures
119
What happens during a generalised tonic clonic seizure?
```
LOC almost immediately
Tonic - muscle tensing
clonic - muscle jerking
tongue biting
incontinence
groaning
irregular breathing
post-ictal period
```
120
Mx for generalised seizures
1st line = sodium valproate
121
mx for tonic clonic seizures
1st line = sodium valproate
| 2nd line = lamotrigine or carbamazepine
122
symptoms of absence seizure and what type of seizure is it?
generalised seizure
patient becomes blank, stares into space and abruptly returns to normal
unaware of surroundings
won't respond
last 10-20 seconds
usually in children and they grow out of it
123
mx of absence seizures
First line: sodium valproate or ethosuximide
124
What are focal seizures?
previously termed partial seizures
these start in a specific area, on one side of the brain
usually start in the temporal lobes
125
what level of consciousness is expected in focal seizures?
the level of awareness can vary in focal seizures:
1) Focal aware (previously termed 'simple partial')
2) focal impaired awareness (previously termed 'complex partial')
3) Awareness unknown
126
What are the types of focal seizures?
motor (e.g. Jacksonian march)
non-motor (e.g. déjà vu, jamais vu; )
other features such as aura
127
mx for focal seizures
First line: carbamazepine or lamotrigine
| Second line: sodium valproate or levetiracetam
128
What are atonic seizures?
Generalised seizures
Also known as drop attacks
characterised by brief lapses in muscle tone.
<3 minutes
Typically begin in childhood
can indicate lennox-gastaut syndrome
129
mx for atonic seizures
First line: sodium valproate
| Second line: lamotrigine
130
What are myoclonic seizures?
Generalised seizures
Sudden brief muscle contractions, like a sudden jump
Usually patient is awake
Can happen in infancy as part of juvenile myoclonic epilepsy = Janz syndrome
131
mx of myoclonic seizures
First line: sodium valproate
| Other options: lamotrigine, levetiracetam or topiramate
132
Who gets juvenile myoclonic epilepsy (Janz syndrome)?
teens, more common in girls
133
Features of juvenile myoclonic epilepsy (Janz syndrome)
1. Infrequent generalized seizures, often in morning
2. Daytime absences
3. Sudden, shock like myoclonic seizure
usually good response to sodium valproate
134
When do patients get alcohol withdrawal seizures?
occur in patients with a history of alcohol excess who suddenly stop drinking
the peak incidence of seizures is at around 36 hours following cessation of drinking
135
why do patients get alcohol withdrawal seizures
chronic alcohol consumption enhances GABA mediated inhibition in the CNS (similar to benzodiazepines) and inhibits NMDA-type glutamate receptors.Alcohol withdrawal is thought to be lead to the opposite (decreased inhibitory GABA and increased NMDA glutamate transmission)
136
what are psychogenic non-epileptic seizures?
previously termed pseudoseizures, this term describes patients who present with epileptic-like seizures but do not have characteristic electrical discharges
patients may have a history of mental health problems or a personality disorder
137
What are the key facts about infantile spasms (West syndrome)?
Brief spasms beginning in first few months of life
1. Flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times
2. Progressive mental handicap
3. EEG: hypsarrhythmia
usually 2nd to serious neurological abnormality (e.g. TS, encephalitis, birth asphyxia) or may be cryptogenic
poor prognosis
138
What happens in benign rolandic epilepsy?
paraesthesia (e.g. unilateral face), usually on waking up
139
Important considerations for prescribing anti-epileptics
Prescribe by brand
antiepileptics can induce/inhibit the P450 system resulting in varied metabolism of other medications, for example warfarin
anti epileptics are generally teratogenic - advice women to take advice from a neurologist prior to conception
Breastfeeding is generally considered safe for mothers taking antiepileptics with the possible exception of the barbiturates
140
Driving rules following a seizure
generally patients cannot drive for 6 months following a seizure.
For patients with established epilepsy they must be fit free for 12 months before being able to drive
141
How does sodium valproate work and what is it used for?
Increases GABA activity
| 1st line for generalised seizures
142
Side effects of sodium valproate
```
ncreased appetite and weight gain
alopecia: regrowth may be curly
P450 enzyme inhibitor
ataxia
tremor
hepatitis
pancreatitis
thrombocytopaenia
teratogenic (neural tube defects)
```
143
How does carbamazepine work and what is it used for?
Binds to sodium channels increasing their refractory period
| First line for focal seizures
144
Side effects of carbamazepine
```
P450 enzyme inducer
dizziness and ataxia
drowsiness
leucopenia and agranulocytosis
SiADH
visual disturbances (especially diplopia)
```
145
How does lamotrigine work and what is it used for?
Sodium channel blocker
| 2nd line for generalised and focal seizures
146
Side effect of lamotrigine
Stevens-Johnson syndrome
147
How does phenytoin work and what is it used for?
Binds to sodium channels increasing their refractory period
| Used as an IV infusion in refractory status
148
Side effects of phenytoin
```
P450 enzyme inducer
dizziness and ataxia
drowsiness
gingival hyperplasia, hirsutism, coarsening of facial features
megaloblastic anaemia
peripheral neuropathy
enhanced vitamin D metabolism causing osteomalacia
lymphadenopathy
```
149
What is status epilepticus
defined as seizures lasting more than 5 minutes or more than 3 seizures in one hour or or 2 or more seizures in a 5 minutes period without the person returning to normal between the,
150
Mx of status epilepticus
```
ABC
Airway protection
O2
Assess cardiac function
Check BMs
```
IV lorazepam 4mg, repeat after 10 mins
In community - buccal midazolam or rectal diazepam
If seizures persist = IV phenytoin or phenobarbital
No response within 45 minutes = induction of GA
151
What is essential tremor?
an autosomal dominant condition which usually affects both upper limbs
152
What are the symptoms of essential tremor?
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor)
153
Mx of essential tremor
propranolol is first-line
| primidone is sometimes used
154
What are the types of tremors?
1 Rest tremor
2 Action tremor
- Postural tremor (when in a specific position)
- Kinetic tremor = simple (uniformly throughout a voluntary movement), task specific or intention (worse as the body part approaches the target, associated with the cerebellum)
- Isometric tremor
155
What is an extra dural haemorrhage and where is the bleed coming from?
Bleed between the skull and the dura mater.
usually caused by rupture of the middle meningeal artery in the temporo-parietal region
156
What causes extra dural haemorrhages?
Fracture of the temporal bone e.g. from traumatic brain injury
Rarely:
- Atriovenous abnormalities
- bleeding disorders
157
what does an extra dural haemorrhage look like on CT?
Bi-convex lemon shaped mass limited by the cranial sutures
Secondary features - midline shift and brainstem herniation
158
symptoms of an extra dural haemorrhage
ongoing headache following traumatic head injury
LOC then a period of improved neurological symptoms and consciousness (lucidity) followed by a rapid decline over hours as the haematoma gets larger
159
mx of extra dural haemorrhage
ABCDE
Coagulation bloods and reverse any anticoagulation
Prophylactic abx if open skull fracture
Antiepileptics to reduce seizure risk
Mannitol/barbiturates to reduce ICP
Definitive management = Burr hole craniotomy to remove the haematoma, may need hemicraniotomy if there's lots of blood/oedema to prevent brain stem herniation
160
what is the anatomical landmark of the middle meningeal artery?
MMA lies underneath the pterion
| Its where the parietal, frontal, sphenoid and temporal bones fuse
161
Ix for extradural haemorrhage
BMs to rule out hypoglycaemic cause of reduced GCS
ECG to rule out heart block causing reduced GCS
Coagulation
Group and save
CT head
Skull xray
Cerebral angiography - to look for atriovenous malformation
162
complications of extra dural haemorrhage
Infection: due to skull fracture or as a result of operative intervention
Cerebral ischaemia: typically occurs adjacent to the haematoma
Seizures
Cognitive impairment
Hemiparesis
Hydrocephalus due to obstruction of the ventricles
Brainstem injury: due to significantly raised ICP
163
Signs of extradural haemorrhage
```
Reduced GCS
Confusion
Cranial nerve deficits - oculomotor nerve palsy = fixed and dilated ipsilateral pupil
Motor/sensory deficits in limbs
Hyperreflexia
Spasticity
Upping plantar reflex
Cushing's triad = bradycardia, HTN and deep/irregular breathing
```
164
What is cushings triad?
A physiological response to raised ICP to attempt to improve perfusion
HTN
Bradycardia
Irregular breathing pattern
165
what causes malaria?
caused by Plasmodium protozoa which is spread by the female Anopheles mosquito.
There are four different species which cause disease in man:
Plasmodium falciparum (causes nearly all episodes of severe malaria)
Plasmodium vivax
Plasmodium ovale
Plasmodium malariae
166
Protective factors against malaria
Sickle cell trait
G6PD deficiency
HLA-B53
absence of Duffy antigens
167
Features of severe malaria caused by plasmodium falciparum
```
schizonts on a blood film
parasitaemia > 2%
hypoglycaemia
acidosis
temperature > 39 °C
severe anaemia
complications as below
```
168
Complications of severe malaria caused by plasmodium falciparum
cerebral malaria: seizures, coma
acute renal failure: blackwater fever, secondary to intravascular haemolysis, mechanism unknown
acute respiratory distress syndrome (ARDS)
hypoglycaemia
DIC
169
symptoms of non-falciparum malaria
general features of malaria: fever, headache, splenomegaly
Plasmodium vivax/ovale: cyclical fever every 48 hours. Plasmodium malariae: cyclical fever every 72 hours
Plasmodium malariae: is associated with nephrotic syndrome.
170
General symptoms and signs of malaria
Suspect malaria in someone who lives or has travelled to an area of malaria.
The incubation period is 1-4 weeks after infection with malaria although it can lie dormant for years.
```
Fever, sweats and rigors
Malaise
Myalgia
Headache
Vomiting
```
Pallor due to the haemolytic anaemia
Hepatosplenomegaly
Jaundice as bilirubin is released during the rupture of red blood cells
171
Diagnosis of malaria
Malaria blood film - in EDTA bottle. Send 3 samples over 3 consecutive days (48 hour cycle of malaria released into blood from blood cells)
172
Mx of malaria
Oral options in uncomplicated malaria:
- Artemether with lumefantrine (Riamet)
- Proguanil and atovaquone (Malarone)
- Quinine sulphate
- Doxycycline
Intravenous options in severe or complicated malaria:
- Artesunate. This is the most effective treatment but is not licensed.
- Quinine dihydrochloride
173
Advice for malaria prophylaxis
Be aware of locations that are high risk
No method is 100% effective alone
Use mosquito spray (e.g. 50% DEET spray) in mosquito exposed areas
Use mosquito nets and barriers in sleeping areas
Seek medical advice if symptoms develop
Take antimalarial medication as recommended
174
What are options for antimalarial medications?
Proguanil and atovaquone (Malarone)
Taken daily 2 days before, during and 1 week after being in endemic area
Most expensive (around £1 per tablet)
Best side effect profile
Mefloquine
Taken once weekly 2 weeks before, during and 4 weeks after being in endemic area
Can cause bad dreams and rarely psychotic disorders or seizures
Doxycycline
Taken daily 2 days before, during and 4 weeks after being in endemic area
Broad-spectrum antibiotic therefore it causes side effects like diarrhoea and thrush
Makes patients sensitive to the sun causing a rash and sunburn
175
What causes meningitis in adults?
bacterial causes:
- neisseria meningitides (meningococcus)
- Streptococcus pneumoniae (pneumococcus)
If over 60/immunosuppressed, consider listeria monocytogenes
viral causes:
- coxsackie virus
- echovirus
- mumps
- HSV
- CMV
- HZV
- HIV
- measles
176
Symptoms of meningitis
```
Fever
Neck stiffness
Vomiting
Headache
Photophobia
Altered consciousness
Seizures
```
If there's meningoccocal septicaemia = non-blanching rash
Kernig's test - lift leg and straighten knee
Brudzinski's test - lift neck/head
177
CSF findings in bacterial meningitis
cloudy appearance
Low glucose
High protein
10-5000 white cells (polymorphs - neutrophils)
178
CSF findings in viral meningitis
Clear appearance
60-80% of plasma glucose
normal/raised proteins
15-1000 white cells (lymphocytes)
179
CSF findings in TB meningitis
slightly cloudy, fibrin web appearance
Low gluocse
High protein
10-1000 lymphocytes
Use Ziehl-Neelsen stain & PCR
180
Ix for meningitis
```
FBC
CRP
Coagulation screen
Blood culture
Whole blood PCR
Blood glucose
Blood gas
LP - if no signs of raised ICP
```
181
Mx of meningitis
IM benzylpenicillin in the community if meningococcal disease is suspected
Initial empirical therapy for adults <50 = IV cefotaxime
Add on amoxicillin if aged >50
Meningococcal meningitis = IV benzylpenicillin or cefotaxime
Pneumococcal meningitis = IV cefotaxime
Meningitis caused by haemophilus influenzae = IV cefotaxime
Meningitis caused by listeria = IV amoxicillin + gentamicin
Penicillin allergic = chloramphenicol
AND GIVE IV DEXAMETHASONE
- To reduce risk of neurological sequelae
- don't give if: septic shock, meningococcal septicaemia, immunocompromised or its following surgery
182
Post exposure prophylaxis for meningococcal meningitis contacts
prophylaxis needs to be offered to household and close contacts of patients affected with meningococcal meningitis & people who been exposed to respiratory secretion, regardless of the closeness of contact - within 7 days of symptom onset
Give oral ciprofloxacin or rifampicin
183
What causes brain mets?
```
Lung cancer (most common)
Breast ca
Malignant melanoma
Renal cell carcinoma
Colorectal ca
Pancreatic ca
Testicular ca
```
184
symptoms of brain mets
Seizures
Focal neurological deficits
cognitive deficits
Headaches
185
Ix for brain mets
CT +/- PET to find primary
186
Mx of brain mets
Surgical resection
radiotherapy
glucocorticoids to reduce tumor oedema
Palliative care
187
Symptoms of migraines
a severe, unilateral, throbbing headache
associated with nausea, photophobia & phonophobia
attacks may last up to 72 hours
patients characteristically go to a darkened, quiet room during an attack
'classic' migraine attacks precipitated by an aura (1/3 of patients)
typical aura are visual, progressive, last 5-60 minutes and are characterised by transient hemianopic disturbance or a spreading scintillating scotoma
188
Common triggers for migraines
```
tiredness, stress
alcohol
combined oral contraceptive pill
lack of food or dehydration
cheese, chocolate, red wines, citrus fruits
menstruation
bright lights
```
189
What is the diagnostic criteria for migraines?
International Headache Society
Must have at least 5 attacks that fulfill the following criteria:
1) Lasts 4-72 hours
2) headache with 2 of the following: unilateral, pulsatile, moderate/severe pain, aggravation by physical activity
3) during headache 1 of the following: nausea/vomiting, photophobia/phonophobia
4) not attributed to another disorder
190
Mx for migraines
ACUTE TX:
1) oral triptan (sumatriptan) + NSAID/paracetamol
2) metoclopramide/prochlorperazine + NSAID/triptan
PROPHYLAXIS:
1) topiramate / propranolol (give propranolol to women of child bearing age - topiramate is teratogenic & reduces effectiveness of hormonal contraception)
2) acupuncture
3) riboflavin
4) menstrual migraine = frovatriptan / zolmitriptan
Pizotifen is no longer recommended - weight gain and drowsiness
191
types of migraine
migraine without aura
migraine with aura
silent migraine - aura but no headache
hemiplegic migraine
192
Symptoms of hemiplegic migraine
```
Typical migraine symptoms
Sudden or gradual onset
Hemiplegia (unilateral weakness of the limbs)
Ataxia
Changes in consciousness
```
193
what are triptans and when are they used?
5HT receptor agonists - serotonin receptor agonists
used to abort migraines when they develop
194
mx of migraines in pregnancy
1st line = paracetamol
2nd line = NSAIDs in 1st and 2nd trimester
Avoid aspirin and codeine during pregnancy
195
what is contraindicated in migraines with aura?
The COCP
196
what is motor neurone disease?
an umbrella term that encompasses a variety of specific diagnoses. Motor neurone disease is a progressive, ultimately fatal condition where the motor neurones stop functioning. There is no effect on the sensory neurones and patients should not experience any sensory symptoms.
197
What are some types of MND?
Amyotrophic lateral sclerosis (ALS) - most common type
Progressive bulbar palsy (affects talking and swallowing)
progressive muscular atrophy
primary lateral sclerosis
198
pathophysiology of MND
progressive degeneration of both upper and lower motor neurones. The sensory neurones are spared.
199
risk factors for MND
family hx
smoking
heavy metals
certain pesticides
200
presentation of MND
Late middle aged man
insidious progressive weakness of muscles throughout body affecting limbs, trunk, face and speech
increasing fatigue
clumsiness
dysarthria (slurred speech)
Wasting of small hand muscles/tibialis anterior
LMN signs -
- muscle wasting
- reduced tone
- fasciculations
- reduced reflexes
UMN signs -
- increased tone/spasticity
- brisk reflexes
- pogoing plantar responses
Doesn't affect
- external ocular muscles
- no cerebellar signs
- abdominal reflexes preserved
- loss of sphincter function = late feature
201
diagnosis of MND
by specialist
diagnosis of exclusion
nerve conduction studies = normal motor conduction
electromyography = reduced action potentials with increased amplitude
MRI to exclude cervical cord compression and myelopathy
202
management of MND
Riluzole - slows disease progression, extends survival by a few months
Edaravone - used in US not UK, slows disease progression
NIV - especially at night
MDT
Advanced directives
End of life care
203
Main causes of death in MND
Respiratory failure
| Pneumonia
204
Characteristics of amyotrophic lateral sclerosis (ALS)
typically LMN signs in arms and UMN signs in legs
in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase
205
Characteristics of primary lateral sclerosis
UMN signs only
206
Characteristics of progressive muscular atrophy
LMN signs only
affects distal muscles before proximal
carries best prognosis
207
characteristics of progressive bulbar palsy
palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
carries worst prognosis
208
Prognosis of MND
50% of patients die within 3 years
209
What is multiple sclerosis?
chronic progressive cell-mediated autoimmune disorder characterised by demyelination in the central nervous system
210
who gets MS?
3 times more common in women
most commonly diagnosed in people aged 20-40 years
much more common at higher latitudes (5 times more common than in tropics)
Symptoms improve in pregnancy and the post part period
211
types of MS
Clinically isolated syndrome
Relapsing remitting disease
Secondary progressive disease
Primary progressive disease
212
pathology of MS
oligodendrocytes around neurones that produce myelin sheath in the CNS are affected
there is activation of immune cells against the myelin
in early disease, re-myelination can occur and symptoms can resolve
MS lesions are disseminated in time and space
213
Causes of MS
```
Multiple genes
Epstein–Barr virus (EBV)
Low vitamin D
Smoking
Obesity
```
214
Symptoms of MS
Symptoms usually progress over more than 24 hours.
At the first presentation, symptoms tend to last days to weeks and then improve.
OPTIC NEURITIS:
- Demyelination of optic nerve
- Loss of vision in one eye
EYE MOVEMENT ABNORMALITIES:
- 6th CN lesion
- Internuclear ophthalmoplegia
- Conjugate lateral gase disorder
FOCAL WEAKNESS:
- Bells palsy
- Horners syndrome
- Limb paralysis
- Incontinence
FOCAL SENSORY SYMPTOMS:
- Trigeminal neuralgia
- Numbness
- Paraesthesia
- Lhermitte's sign: electric shock sensation down the spine when flexing the neck
ATAXIA
- sensory ataxia (Romberg's test)
- cerebellar ataxia
215
what happens in a clinically isolated syndrome of MS
the first episode of demyelination and neurological signs and symptoms
MS cannot be diagnosed on one episode as the lesions have not been “disseminated in time and space”.
May never have another episode or develop MS
Lesions on MRI = more likely to progress to MS
216
What happens in relapsing-remitting MS?
It is characterised by episodes of disease and neurological symptoms followed by recovery.
217
What happens in secondary progressive MS?
there was relapsing-remitting disease at first, but now there is a progressive worsening of symptoms with incomplete remissions
Symptoms become more and more permanent
218
what is primary progressive MS?
a worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions
Symptoms have to be progressive over a period of 1 year to diagnose primary progressive MS.
219
Diagnosis of MS
Neurologist - clinical diagnosis
MRI scans - to show demyelinating lesions
LP = oligocloncal bands in CSF
220
What do oligoclonal bands in CSF demonstrate?
Multiple sclerosis
221
Symptoms of optic neuritis
Central scotoma. This is an enlarged blind spot.
Pain on eye movement
Impaired colour vision
Relative afferent pupillary defect: pupil dilates on swinging a light from the unaffected to affected eye
222
Causes of optic neuritis
```
MS
Sarcoidosis
Systemic lupus erythematosus
Diabetes
Syphilis
Measles
Mumps
Lyme disease
```
223
Mx for optic neuritis
Urgent ophthalmology review
Steroids
50% with single episode of optic neuritis go on to develop MS in next 15 years
224
Mx of MS
MDT
Disease modifying drugs and biologic therapies
Methylprednisolone in relapses
Exercise
Neuropathic pain = amitriptyline or gabapentin
Urge incontinence = oxybutynin
Spasticity = baclofen, gabapentin and physiotherapy
225
What is myasthenia Gravis?
an autoimmune condition that causes muscle weakness that gets progressively worse with activity and improves with rest.
226
who gets myasthenia Gravis?
women <40 yo
| men >60 yo
227
What is associated with myasthenia Gravis?
thymoma (tumour of the thymus gland)
228
what antibodies are seen in myasthenia Gravis?
acetylcholine receptor antibodies
Rarer antibodies:
- muscle specific kinase antibodies
- low density lipoprotein receptor related protein 4 (LRP4) antibodies
229
Symptoms of myasthenia Gravis
weakness that gets worse with muscle use and improves with rest
symptoms better in the morning and worse at the end of the day
symptoms affect the proximal muscles and small muscles of the head and neck
diplopia - due to extra ocular muscle weakness
ptosis - eyelid weakness causing drooping of the eyelids
weakness in facial movements
difficulty with swallowing
fatigue in the jaw when chewing
slurred speech
progressive weakness with repetitive movements
230
how to elicit muscle fatiguability seen in myasthenia Gravis on examination
Repeated blinking will exacerbate ptosis
Prolonged upward gazing will exacerbate diplopia on further eye movement testing
Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides
Check for a thymectomy scar.
Forced vital capacity
231
Diagnosis of myasthenia Gravis
Test for the antibodies:
1) Acetylcholine receptor (ACh-R) antibodies (85% of patients)
2) Muscle-specific kinase (MuSK) antibodies (10% of patients)
3) LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%)
CT/MRI thymus for thymoma
Edrophonium test - give IV edrophonium chloride (neostigmine) - in MG will give a brief and temporary relief of muscle weakness
232
Mx of myasthenia Gravis
Reversible acetylcholinesterase inhibitors (pyridostigmine or neostigmine)
Immunosuppression with prednisolone or azathioprine
Thymectomy
Monoclonal antibodies: rituximab / eculizumab
233
What is a myasthenic crisis?
a severe complication of myasthenia gravis
life threatening
acute worsening of symptoms
can lead to respiratory failure as a result of weakness in the respiratory muscles
234
Mx of myasthenic crisis
BiPAP / intubation and ventilation
IV immunoglobulins
plasma exchange
235
What is Parkinson's disease?
a condition where there is a progressive reduction of dopamine in the basal ganglia (specifically the substantia nigra) of the brain, leading to disorders of movement.
The symptoms are characteristically asymmetrical, with one side affected more than the other.
236
What is the classic triad of symptoms seen in Parkinson's disease?
Resting tremor
Rigidity
Bradykinesia
237
symptoms of parkinsons disease
unilateral pill rolling tremor (worse on rest/when distracted, improves with movement)
cogwheel rigidity
handwriting gets smaller and smaller
shuffling gait
difficulty initiating movements e.g. standing to walking
difficulty in turning when standing
hypomimia - reduced facial movements and expressions
```
Depression
sleep disturbance/insomnia
anosmia
postural instability
cognitive impairment/memory problems
```
238
name some Parkinson's-plus syndromes
Multiple system atrophy
dementia with leeway bodies
progressive supra nuclear palsy
corticobasal degeneration
239
What is multi system atrophy?
a rare condition where the neurones of multiple systems in the brain degenerate.
affects the basal ganglia & multiple other areas
degeneration of the basal ganglia = Parkinson’s presentation.
degeneration in other areas = autonomic dysfunction (causing postural hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (causing ataxia).
240
What is dementia with Lewy bodies?
a type of dementia associated with features of Parkinsonism
causes a progressive cognitive decline
associated symptoms: visual hallucinations, delusions, disorders of REM sleep and fluctuating consciousness.
241
How is parkinsons disease diagnosed?
clinical diagnosis by specialist
242
Mx of parkinsons disease
Levodopa:
- co-benyldopa (levodopa + benserazide)
- co-careldopa (levodopa + carbidopa)
+ COMT inhibitors - entacapone (to slow the breakdown of levodopa)
Dopamine agonists - bromocrytopine/carbergoline
MOA-B inhibitors - selegiline/rasagiline
243
How is levodopa used to treat Parkinson's disease?
levodopa = synthetic dopamine
must be combined with a drug that stops levodopa being broken down by the body = carbidopa or benserazide
levodopa is the most effective parkinsons drug but it becomes less effective over time - reserved for when other treatments aren't controlling symptoms
244
Side effects of levodopa
when doses are too high patients get dyskinesias:
- dystonia
- chorea
- athetosis
245
How do dopamine agonists work in parkinsons, name some and what are the side effects?
Mimic dopamine in the brain
bromocryptine, pergolide, carbergoline
SE: pulmonary fibrosis
246
How do MAO-B inhibitors work in parkinsons and name some?
they stop the break down neurotransmitters like dopamine, serotonin and adrenalin
Selegiline, rasagiline
247
Test to see if fluid draining from ears/nose is CSF
Test glucose - would be positive in CSF but not in mucus
Beta-2-transferrin is gold standard
