Neurology Flashcards
1
Q
What is a migraine characterised by?
A
- a severe, unilateral, throbbing headache
- associated with nausea, photophobia and phonophobia
- attacks may last up to 72 hours
- patients characteristically go to a quiet, dark room during an attack.
classic migraine attacks are precipitated by an aura. - typical aura are visual, progressive and last 5-60 minutes, transient hemianopia disturbances
2
Q
what are the risk factors for migraines and triggers for migraines?
A
Risk Factors
- Family history
- high caffeine intake
- exposure to change in barometric pressure
- females
Triggers
- tiredness, stress
- alcohol
- COCP
- lack of food/dehydration
- cheese, chocolate, red wines, citrus fruits
- menstruation
- bright lights
3
Q
what are the diagnostic features for migraine?
A
- presence of risk factors
- prolonged headache
- nausea
decreased ability to function - if the headache has the following characteristis - unilateral location, pulsating quality, moderate to severe pain intensity, aggravation by or causing avoidance of routine physical activity
- if it is not attributed to anything else
4
Q
what investigations would you consider for migraines?
A
it is a clinical diagnosis
consider
ESR, LP, CSF, CT head
5
Q
how do you treat migraines?
A
Rescue therapy - sumatriptan and high flow oxygen.
to treat mild to moderate symptoms
- give NSAIDs or aspirin
you can add anti-emetics
2nd line = paracetamol mono-therapy
to treat severe symptoms
1st line -triptan (almotriptan)
you can add anti-emetic, hydration and NSAIDs
2nd line - ergot alkaloids (ergotamine/caffine)
Prophylaxsis
- propranolol is 1st line
- topiromate can be given if BB is not suitable
6
Q
what is a tension headache?
A
a form of primary headache
they can episodic or chronic
they are rarely disabling
7
Q
what are the characteristic features of a tension headache?
A
often described s a tight band around the head or a pressure sensation - symptoms tend to be bilateral (migraine tend to be unilateral)
lower intensity than a migraine
not associated with aura, nausea/vomiting o aggravated by routine physical activity
may be related to stress
8
Q
what is a chronic tension headache defined as?
A
tension headache lasting more than 15 days per month
9
Q
how would you treat acute and chronic tension headaches?
A
acute - simple analgesics (aspirin, paracetamol, ibuprofen, naproxen)
Chronic symptoms - antidepressants (amitriptyline or doxepin)
10
Q
what are the features of a cluster headache?
A
attacks of severe pain localised to the unilateral orbital, supra-orbital, and/or temporal areas; they last 15 minutes to 3 hours.
the attacks usually occur at the same time period for several weeks
they can occur from once every other day to 8 times per day
may be accompanied by redness, lacrimation, lid swelling.
more common in men and smokers
11
Q
what investigations would you order for cluster headaches?>
A
Brain CT or MRT - to eliminate secondary causes
ESR - exclude giant cell arteritis in patients over 50
pituitary function tests - to exclude secondary causes
12
Q
how do you treat an acute attack of cluster headache?
A
1st line
- subcutaneous sumatriptan with high flow oxygen
2nd line
- intranasal zolmitriptan (nasal)
- if they have CVD of uncontrolled hypertension then the first line should be just oxygen
13
Q
how do you treat ongoing episodic/chronic cluster headache?
A
1st line - verapamil
2nd line - lithium, topiramate, gabapentin or melatonin
14
Q
what are the tiggers for cluster headaches?
A
alcohol
volatile smells
warm temps
sleep
15
Q
what is temporal arteritis?
A
a large vessel vasculitis
It primarily affects branches of the external carotid artery and is the most common form of vasculitis in adults.
Typically occurs in woman older than 50.
16
Q
what are the clinical features of temporal arteritis?
A
typically older than 50
usually rapid onset (less than one month)
headache
jaw claudication
visual disturbances secondary to anterior ishcemic optic neuropathy - can be transient or visual loss
tender, palpable temporal artery
around 50% will have features of polymyalgia rheumatica (aching, morning stiffness in proximal limb muscles)
also there may be lethary, depression, low grade fever, anorexia, night sweats.
17
Q
what are the investigations for temporal arteritis?
A
ESR
CRP
FBC
LFTs
consider temporal artery biopsy (will show skip lesions)
aortic arch angiography
18
Q
how do you treat suspected and confirmed temporal arteritis?
A
suspected with no visual/neurological symptoms - prednisolone
suspected with neurological symptoms or signs - methylprednisolone plus IV pulse therapy
confirmed temporal arteritis
1st line prednisolone and aspirin can be added plus osteoporosis prevention (calcium carbonate and ergocalciferol and alendronic acid)
19
Q
what are some differential diagnosis for acute single episode headaches?
A
meningitis encephalitis subarachnoid haemorrhage head injury sinusitis glaucoma tropical illness
20
Q
what is an ischaemic stroke?
A
when blood supply in a cerebral vascular territory is critically reduced due to occlusion or stenosis of a cerebral artery.
Symptoms must last for more than 24 hours
21
Q
what is a transient ischaemic attack?
A
it has typical symptoms of rapidly resolving unilateral weakness or numbness
22
Q
what is a haemorrhagic stroke?
A
vascular rupture with bleeding into the brain parenchyma
23
Q
what are the types of ischaemic strokes?
A
thrombotic (thrombosis from large vessels e.g. the carotid) Embolic stroke (usually a blood clot but fat, air or clumps of bacteria can also act as an embolus. Also AF is an important cause of emboli forming in the heart)
24
Q
what are the risk factors for an ischaemic stroke?
A
general RF for CV disease - age, hypertension, smoking, hyperlipidaemia, DM)
Fam history
AF
25
what are the key diagnostic features of a stroke?
```
vision lost or visual field deficit (homonymous hemianopia)
weakness
aphasia
impaired co-ordination
swallowing problems
```
26
what are the first line investigations to order for a stroke?
1st line - CT head,
MRI brain,
serum glucose and serum electrolytes (hypoglycaemia and electrolyte imbalances may cause focal neurological symptoms),
serum urea and creatinine (renal failure may be a potential contraindication for some stoke treatment),
ECG to exclude cardiac arrhythmia or ischaemia,
FBC to detect conditions that may be potential contraindications for some acute stoke treatment and interventions,
prothrombin time and PTT
27
what would you see on CT and MRI of a person who has had an ischaemic stroke?
in many cases - the CT is normal within the first few hours of an ischaemic stroke
You would see hypo-attenuation (darkness) or the brain parenchyma; loss of grey matter- white matter differentiation and sulcal effacement; hyper-attenuation (brightness) in an artery indicated a clot)
MRI - contraindicated in patients with certain metal implants such as pacemakers
acute ischaemic infarct appears bright on diffusion-weighted imaging
28
how do you treat an ischaemic stroke?
presentation within 4.5 and no contraindication to thrombolysis
1st line = alteplase
aspirin can be added, or endovascular intervention
Plus supportive care and swallowing assessment
for non-ambulatory patients give VTE propyhlaxis (heparin)
presentation after four hours or contraindication to thrombolysis
aspirin and supportive care
swallowing assessment
add VTE prophylaxis (heparin)
29
what are the clinical features of a TIA?
symptoms last less than 24 hours but typically resolve within one hour
focal neurological deficit - unilateral weakness or sensory loss, amaurosis fugax or hemianopsia, aphasia, cranial nerve defects, vertigo, lack of coordination, ataxi, syncope.
30
risk factors for TIA
```
AF
valvular disease
carotid stenosis
congestive heart failure
hypertension
DM
smoking
alcohol abuse
advanced age
```
31
what is ABCD2
```
for TIA - risk of stroke after a TIA
age over 60
BP over 140/90
clinical features (unilateral weakness or speech impairment)
duration of symptoms
diabetes
```
32
how is a TIA treated?
atherosclerotic or small-vessel TIA - 1st line - platelet therapy (aspirin or aspirin plus clopidogrel) plus a lipid lowering agent (atorvastatin)
if there is greater than 50% carotid stenosis - stent
If there is a cardioembolic TIA without ischaemic heart disease give anticoagulation (warfarin or rivaroxaban) as first line
If there is TIA with ischaemic heart disease - anticoagulation plus lipid lowering agent
33
what investigations would you order for a TIA?
blood glucose - hypoglycaemic events can mimic TIA
chemistry profile - sever hyponatraemia can trigger seizures or induce generalised weakness
extremely low potassium or very high calcium can also cause generalised weakness
FBC
prothrombin time, INR and activated PTT
ECG
brain MRI with diffusion
fasting lipid profile
34
what are the different types of haemorrhagic strokes?
intraparenchymal/intracerebral bleeding within the brain)
subarachnoid (bleeding on the surface of the brain)
intraventricular
35
what are the risk factors for haemorrhagic stoke?
```
age
hypertension
arteriovenous malformation
anticoagulation therapy
male sex
asian, black and/or hispanic
```
36
what are the key features of a haemorrhagic stroke?
```
altered sensation
headache
weakness
sensory loss
aphasia
dysarthria
ataxia
```
```
Headache
Altered mental status
Nausea & Vomiting
Hypertension
Seizures
Focal neurological deficits
```
37
what are some features of headaches which suggest subarachnoid or intracerebral?
intracerebral - usually insidious onset and gradually increasing intensity
Sudden onset with gradual moderation or the most severe headache of my life - suggests subarachnoid
thunderclap headache is characteristic88 of subarachnoid headache.
38
what investigations would you perform for a suspected haemorrhagic stroke?
None infused CT - will show hyperdense lesion
chemistry panal
FBC - to exclude thrombocytopenia asa cause of haemorrhage, also a low platelet count suggests a secondary cause of haemorrhage
clotting factors to rule out coagulopathy
ECG
platelet function test
urine drug screen
LFT
ICH score
39
how do you treat a haemorhagic stroke?
1st line -neurosurgical evaluation
airway protection and aspiration precautions
support
40
what is cerebellar syndrome?
a disorder when the cerebellum becomes inflamed or damaged.
| the cerebellum is the area of the brain responsible for controlling gait and muscle coordination.
41
what are the symptoms of cerebellar disease?
```
DANISH
Dysdiadochokinesia, Dymetria (past pointing)
A ataxia
N nystamus
I intention tremour
S- slurred staccato speach
H- Hypotonia
```
42
what are the causes of cerebellar syndrome?
```
stoke
alchohol
MS
hypothyroidism
drugs - phenytoin, lead poisoning,
paraneoplastic e.g. secondary to lung cancer
```
43
what is status epilepticus?
5 or more minutes of either continuous seizure activity or repetitive seizures without regaining consciousness
44
what are generalised seizures
generalised tonic-colonic seizures classically involve loss of consciousness and a phasic tonic stiffening of the limbs, followed by repetitive clonic jerking.
45
what are absence seizures?
characterised by abrupt cessation of activity and responsiveness, minimal associated movements, staring episodes, and no aura/postical state
46
what is a focal seizure?
previously termed partial seizure
clinical manifestations of seizures that arise from one portion of the brain.
the level of awareness can vary
can be motor or non-motor
47
what investigations would order for generalised seizures?
EEG
blood glucose
FBC - look for infection
electrolyte panel
toxicology screen - illicit substances may cause seizures
head CT - may reveal a structural lesion
serum prolactin - greater than twice the baseline indicated GTCS
serum creatine kinase
48
how are acute generalised seizures managed?
1st line - IV or rectal benzodiazepine (lorazepam) plus supportive care
49
what is the ongoing treatment for generalised seizures?
1st line sodium valproate
other primary options include lamotrigine, topiramte, oxcarbazepine, carbamazepin, phenytoin.
secondary options include - zonisamide, lacosamide, clobazam, gabapentin
if monotherapy does not work anticonvulsant duel therapy can be used
lamotrigine and topiramate
lamotrigine and valproic acid
topiramate and carbamezapine
50
what are the features of a focal seizure?
movement of one side of the body or one specific body part
a premonitory sensation or experience (fear, epigastric sensation, deja vu)
automatisms (picking at clothes, smacking of lips)
temporary aphasia
staring and loss of contact with the environment
51
what are the investigations for focal seizures?
```
blood glucose
FBC
electrolyte panal
toxicology screen
LP and CSF analysis
CT head
MRI brain
EEG
```
52
what is the management of multiple focal seizures in a short interval?
1st line - lorazepam/diazepam plus airway maintenance
53
what is the first line treatment for ongoing focal seizures?
carbamazepine
other anti-epileptics can also be used
54
what investigations should be ordered for absence seizures?
EEG
55
how are absence seizures managed?
if no history of GTCS
1st line - ethosuximide or valproic acid or lamotrigine
2nd line - topiramate or zonisamide or levetiracetam
If there is a history of GTCS
valproic acid or lamotrigine
56
what is non-epileptic attack disorder?
epileptic seizures that are not caused by electrical activity in the brain
57
what are the clinical features of non-epileptic attack disorder?
most common they will mimic tonic colonic seizure
the person does not usually loose consciousness but they may be unable to respond or react during the attack
some may have an aura
58
what causes non-epileptic attack disorder?
it is currently believed that it is the brain's response to overwhelming stress or when the brain has overload
often people with this disorder have other health problems such as chronic illness, anxiety or depression
59
how is non-epileptic attack disorder diagnosed?
EEG - measures electrical activity in the brain
a raised prolactin suggests epileptic seizure
60
what is narcolepsy?
a chronic condition characterised by a disruption of the sleep-wake cycle and rapid eye movement (REM) sleep intrusion.
They can not regulate wake-sleep cycles
61
what are the symptoms of narcolepsy?
excessive day time sleepiness
cataplexy (sudden and transient loss of muscle tone caused by strong emotion)
hypnagogic/hypnopompic hallucinations as(as waking or as falling to sleep)
sleep paralysis
only 10-15% of people will have all 4 symptoms
62
what investigations would you perform for narcolepsy?
actigraphy and sleep diary
overnight polysomnography
mulstiple sleep latency test
63
how do you treat narcolepsy?
sleep hygiene and lifestyle changes
- patients should be advised to sleep at regular times, to avoid shift work, have 15-30 min naps at lunchtime and/or afternoon
patients should avoid heavy meals and afternoon caffeinated beverages and should abstain from alcohol
adequate psychosocial support should be given
plus - Modafinil
64
how do you treat narcolepsy with cataplexy?
1st line - avoidance of trigger plus sodium oxybate and/or antidepressants (fluoxetine, venlafaxine)
2nd line - monoamine oxidase inhibitors (MAOIs)
65
what is shingles?
shingles (herpes zoster) is an acute, unilateral, painful blistering caused by reactivation of the varicella zoster virus
66
what are the symptoms of shingles?
localised pain to a dermatome, the pain is burning, stinging, itching or tingling and ranges from mild to severe) - the most commonly involved ganglia are the thoracic and trigeminal nerves
pruritus in the affected dermatome
rash - erythematous maculopapular rash, which is followed by the appearance of clear vesicles. The vesicle eventually pustulate and form crusts
if the trigeminal nerve is affected the rash may cause corneal ulceration.
67
how is shingles managed?
oral antiviral therapy (famciclovir, secondary option is aciclovir)
plus simple analgesics plus calamine lotion
if severe pain give opioid analgesics and topical analgesics
if there is eye involvement - prompt referral to the ophthalmologist
68
what are the complications of shingles?
herpes zoster opthalmicus - if it occurs in the trigeminal nerve - the infection may cause conjunctivitis, keratitis, corneal ulceration, glaucoma and blindness.
superinfection skin lesions (staph or strept)
encephalitis
transverse myelitis
varicella zoster retinitis
disseminated zoster - seen in severely immunocompromised patients, the vesicular rash involves several dermatomes and visceral involvement may also occur (IV aciclovir should be used)
69
who is the shingles vaccine given to?
offered to all patients aged 70-79
| is is contraindicated in immunocompromised patients
70
what causes wernicke-korsakoff syndrome?
caused by deficiency of thiamine
71
what is wernicke's encephalopathy?
a neurological emergency resulting from thiamine deficiency with varied neurocognitive manifestation
72
why does thiamine deficiency cause problems?
because thiamine deficiency causes impaired glucose metabolism and decreases cellular energy
the brain is vulnerable to impaired glucose metabolism
this leads to neuronal death in certain neuronal populations with high metabolic requirements and high thiamine turnover.
73
what are the causes of wernicke's encephalopathy?
main cause is alcohol abuse
rarer causes: persistent vomiting, stomach cancer, dietary insufficiency
74
what are the symptoms of wernicke's encephalopathy?
```
mental slowing, impaired concentration, and apathy
confusion, altered GCS
ataxia
ophthalmoplegia
nystagmus (the most common ocular sign)
```
75
what investigations should your order for wernicke's encephalopathy?
```
therapeutic trial of parenteral thiamine
finger prick glucose
FBC
serum electrolytes
renal function
LFT
urinary and serum drug screen
serum ammonia
blood alcohol level
blood thiamine and metabolites
```
76
how is wernicke's encephalopathy treated?
stabilisation and IV thiamine and IV magnesium sulfate and multivitamins
if there is ongoing risk of wernicke's encephalopathy - give dietary supplementation with thiamine
77
what is korsakoff's syndrome?
anterograde amnestic syndrome with disproportionate impairment of recent memory relative to other cognitive domains, accompanied sometimes with confabulation as a long term consequence of thiamine deficiency
degeneration of the mammillary bodies
often no complete recovery of memory deficit
78
what is Huntington's disease?
an autosomal dominant neurogenerative disorder.
it is a progressive and incurable condition that typically results in death 20 years after the initial symptoms develop
it is a trinucleotide repeat disorder repeat expansion of CAG which results in degeneration of cholinergic and GABAergic neurones in the striatum of the basal ganglia
79
what are the typical features of huntington's disease?
chorea (jerky involuntary movement)
personality changes (irritability, apathy, depression) and intellectual impairment
dystonia
saccadic eye movement
loss of coordination
deficit in fine motor coordination (finger tapping)
impaired tandem walking
80
what investigations would you perform for huntington's?
diagnosis is clinical
consider CAG repeat testing
PCR
Capillary or gel electrophoresis
MRI or CT scan
81
how is huntington's managed?
counselling for patients and family
if there is chorea - antipsychotics or benzodiazepines
if there is depression - antidepressants - citalopram or fluoxetine
behaviour problems and mood instability - carbamazepine or valproic acid
behavioural problems where anxiety or insomnia is prominent (SSRI or benzodiazepines)
anxiety without prominent behavioural problems - SSRI
OCD behaviours - SSRI or antipsychotics or clomipramine or CBT
If there is bradykinesia and rigidity predominant - dopamine agonists (levodopa)
82
what is parkinson's disease?
chronic progressive neurological disorder characterised by motor symptoms of resting tremor, rigidity, bradykinesia and postural instability.
83
what is the pathophysiology of parkinson's?
there is loss of dopaminergic neurones in the substantia nigra with findings of lewy bodies in the affected neurones.
loss of these neurones within the basal ganglia accounts of the constellation of motor symptoms.
84
what are the clinical features of parkinson's?
```
bradykinesia (slow movements)
resting tremor - typically pill rolling
rigidity - cogwheel - due to superimposed tremor
postural instability - develops later
shuffling gait
stooped posture
drooling
psychiatric features
impaired olfaction (smelling)
```
85
what investigations would you perform for parkinson's?
dopaminergic agent trial
also consider MRI brain, functional neuroimaging, olfactory testing, genetic testing, 24 hour urine copper (wilsons disease should be excluded)
86
How is parkinson's treated?
** treatment does not stop the progression of disease just helps symptoms.
if the motor symptoms are affecting the patients quality of life give levodopa
other options are dopamine agonists (pramipexole or ropinirole) or monoamine oxidase B
these can also be added to
second line carbidopa or domperidone
physical activity - improves functional performance
Deep brain stimulation (only if symptoms not controlled with other drugs) this may include intermittent apomorphine injection and/or continuous SC apomorphine infusion
*levodopa is usually combined with a decarboxylase inhibitor
87
what can cause parkinsonism?
```
parkinson's disease
drug-induced (anti-psychotics, metoclopramide)
wilson's disease
post-encephalitis
toxins
```
88
what is the role of the frontal lobe?
```
movement
personality
counting
spelling
decision making
```
89
what is the role of the parietal lobe ?
sensory information
| allows us to locate physical location and guides movement in 3d space
90
what is the role of the temporal lobe?
hearing
smell
memory
visual recognition of faces
91
what is the role of the occipital lobe?
processing visual information
92
what is vascular dementia?
a chronic progressive disease of the brain bringing about cognitive impairment.
There is loss of brain parenchyma is predominately from cerebrovascular causes such as infarction and small-vessel changes.
93
what are the common diagnostic factors for vascular dementia?
```
history of stoke(s)
difficulty solving problems
apathy
disinhibition
slowed processing of info
poor attention
retrieval memory deficit
frontal release reflexes
```
94
what are risk factors for vascular dementia?
age >60
obesity
hypertension
smoking
95
what investigations would you order for vascular dementia?
```
FBC
ESR (to rule out inflam or vasculitis)
blood glucose level
Renal and LFT
syphilis serology
vitamin B12
folate
thyroid function
CT or MRI
ECG
```
96
how would you treat vascular dementia?
atherosclerotic ischaemic disease - antiplatelet therapy (aspirin) and lifestyle modification
if there is severe carotid stenosis - carotid angioplasty and stenting
cardioembolic disease
anticoag therapy - warfarin or rivaroxaban a
97
what is alzheimer's dementia?
chronic, progressive neurodegenerative disorder characterised by a global, non-reversible impairment in cerebral functioning
deterioration occurs over 8-10 years.
insidious onset and a progressive slow decline.
it often co-exists with other forms of dementia
98
what are the features of alzheimer's disease?
memory decline with loss of recent memory first
disorientation to time and place
misplacing items/getting lost
nominal dysphagia - difficulty naming objects/people
apathy
decline in ADL
personality change unremarkable initial physical examination
99
what investigations should you order for Alzheimer disease?
```
cognitive testing (MMSE)
FBC
```
rule out other causes of dementia:
metabolic panel
serum TSH
serum vitamin B12 deficiency
rule out recreational drug use - urine drug screen
CT - may exclude space-occupying lesion
MRI - generalized atrophy
100
what are the differentials of Alzheimer's disease?
```
delirium
depression
vascular dementia
dementia with lewy bodies
frontotemporal dementia
Parkinson's disease dementia
Creutzfeld-jacob disease
```
101
how is Alzheimer's treated
supportive treatment
environmental control measures (home safety assessment)
cholinesterase inhibitors (donepezil) - the goal of this is to slow symptoms of the disease progression by preserving memory and functional abilities
antidepressants and antpsychotics (risperidone) can be added
102
what is lewy body dementia?
a neurodegenerative disorder with Parkinsonism, progressive cognitive decline, prominent executive dysfunction, and visuospatial impairment
the characteristic pathalogical feature is lewy-bodies in the substantia nigra, paralimbic and neocortical areas
103
what are the diagnostic features of lewy body dementia ?
cognitive fluctuations are a core feature and may include fluctuations in cognition, attention and arousal.
visual hallucinations (typically present early in the disease)
motor symptoms - some parkinsonian features
REM sleep behavioral disturbance - vivid dreams are accompanied by the loss of associated atonia of REM sleep - patients tend to act out their dreams.
104
how is dementia with lewy bodies treated?
acute - 1st line - short acting benzodiazepines
ongoing
1st line - cholinesterase inhibitors - donepezil
for psychotic symptoms add atypical anti psychotic (risperidone)
if depression add SSRI or benzodiazepine
if sleep disturbances add clonazepam or melatonin
if there are motor symptoms add carbidopa or levodopa
105
what is frontotemporal lobar degeneration?
it is the third most common type of cortical dementia
106
what are the three types of frontotemporal lobar degeneration?
Picks disease
progressive non fluent aphasia
semantic demential
107
what are the common feature of frontotemporal lobar degeneration?
onset before 65
insidious onset
relatively preserved memory and visuospatial skills
personality change and social conduct problems
108
what are the features of picks disease?
personality change and impaired social conduct
hyperorality, disinhibition, increased appetite and perseveration behaviors
focal gyral atrophy with a knife blade appearance is characteristic of Picks disease
109
what are the features of CPA (progressive non-fluent aphasia/chronic progressive aphasia)
the major factor is non fluent speech
they make short utterances that are agrammatic
comprehension is rarely preserved
110
what is semantic dementia?
Here the patient has a fluent progressive aphasia. The speech is fluent but empty and conveys little meaning. Unlike in Alzheimer's memory is better for recent rather than remote events.
111
what is creutzfeldt-jakob disease and what are the features?
rapidly progressive neurological condition caused by prion proteins
the features are rapid onset dementia and myoclonus
112
what is hydrocephalus?
a condition where there is an accumulation of CSF within the ventricular system of the brain and is caused by an imbalance between CSF production and absorption
113
what is normal pressure hydrocephalus?
characterized by the clinical features of hydrocephalus (levodopa-unresponsive gait apraxia with or without cognitive impairment or urinary symptoms) but without significantly raised CSF pressure as measured by LP
114
what are the features of normal pressure hydrocephalus?
it is a reversible cause of dementia seen in elderly patients
the classical triad of symptoms is urinary incontinence , dementia and bradyphrenia and gait abnormality (may be similar to Parkinson disease)
they may develop over time
they may have been diagnosed with Parkinson's - but levodopa will not improve symptoms ** this is a key to diagnosis
115
what investigations would you order for normal pressure hydrocephalus?
CT head
MRI head
levodopa challenge
LP can also be considered- should reveal normal CSF pressure
116
what is the treatment form normal pressure hydrocephalus?
ventriculoperitoeal shunting
| plus control of CV risk factors
117
what is the presentation of hydrocephalus?
usually patients present with symptoms due to raised ICP which include:
headache - typically worse in the morning, when lying down and during valsalva)
nausea and vomiting
papiloedema
coma (in severe cases)
118
what two categories can hydrocephalus be broadly divided into?
Obstructive (non-communicating) - due to structural pathology blocking the flow of CSF - causes can be tumours, acute haemorrhage (SAH or IVH) and developmental abnormalities
non-obstructive - due to imbalance of CSF production and absorption - either caused by increased CSF production (e.g. choroid plexus tumour) or failure to reabsorb at the arachnoid granulations (meningitis or post-haemorrhagic)
119
what investigations would you perform for hydrocephalus?
CT head
MRI
LP - diagnostic and therapeutic
120
how is hydrocephalus treated?
an external ventricular drain is used in acute, severe hydrocephalus
a ventriculoperitoneal shunt is a long term CSF diversion technique
if obstuctive - the treatment may involve surgically treating the obstruction
**LP should not be used in obstructive as it will cause herniation
121
what is a meningioma?
a primary tumour of the cranial and spinal meninges
| they are predominantly benign
122
what are the features of a meningioma?
headache
neurological deficit
seizure
123
what investigations would you perform for a meningioma?
MRI head or spine without or with contrast
| CT head or spine
124
how is a meningioma treated?
if asymptomatic - observation
symptomatic at any size
- surgical resection
if they are older than 65 and the tumour is less than 3cm then just observation
125
what is an acoustic neuroma?
vestibular schwannoma
| a benign tumour that grows from the superior vestibular component of the vestibulocochlear nerve.
126
how does an acoustic neuroma present?
asymmetrical hearing loss
facial numbness
progressive episodes of numbness
They may also have
tinnitus
difficulty localising sounds
127
what investigations would you order for acoustic neuroma?
audiogram
gadolinium-enhanced MRI head
CT head
auditory brainstem reflexes
128
how do your treat acoustic neuroma?
if the tumour is not growing then just observe
if the tumour is growing - focused radiation or surgery
129
what is a medulloblastoma?
a malignant, invasive brain tumour arising from the cerebellar vermis.
the vast majority arise sporadically in the first two decades of life
130
what is an astrocytoma?
it is a type of glioma (giant cell in origin)
131
what are the different types of astrocytomas?
pilocytic astrocytoma - good prognosis, completely benign, mainly seen in children
diffuse astrocytoma - premalignant tumour
anaplastic astrocytoma - malignant glioblastoma multiforme (GMB) - most common phenotype - very malignant
132
what are the clinical features of space occupying lesions in the brain?
progressive headache - worse on waking from sleep in the morning
the pain is increased by coughing, straining and bending forwards
it is sometimes relieved by vomiting
```
drowsiness
vomiting
cardinal sign is papilloedema
progressive neurological deficit (depends on the area of the brain is affected
seizures
```
133
what are the investigations for brain tumours?
CT and MRI
Bloods - FBC, U&Es, LFTs, B12 etc
biopsy - skull burr-hole
*** LP is contraindicated when there is any possibility of a mass lesion as it can prevent coning
134
what is multiple sclerosis?
it is a chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system
135
what are the different types of multiple sclerosis?
Relapse-remitting disease - the commonest form, acute attacks followed by periods of remission.
Secondary progressive disease - describes relapse and remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses. Generally 65% of patients with relapse and remitting disease go on to develop secondary progressive. Gait and bladder disorders are generally seen
Primary progressive - progressive deterioration from the start.
136
what are the symptoms of MS?
Visual - optic neuritis: common presenting feature, optic atrophy, Uhthoff's phenomenon: worsening of vision following rise in body temperature, internuclear ophthalmoplegia
Sensory - pins/needles, numbness, trigeminal neuralgia, Lhermitte's syndrome: paresthesia in limbs on neck flexion
motor: spastic weakness:most commonly seen in the legs
Cerebellar: ataxia: more often seen during an acute relapse than as a presenting symptoms, tremor
Others - urinary incontinence, sexual dysfunction, intellectual deterioration
137
what investigations would you order for MS?
| and what would the tests show?
MRI brain - hyperintensities in the periventricular white matter
MRI spinal - demyelinating lesions in the spinal cord, particularly the cervical spinal cord
FBC - should be normal
Metabolic panel - should be normal
TSH - should be normal
Vitamins B12 - should be normal
138
How would you treat an acute relapse of MS?
1st line - methylprednisolone
| this will only shorted the length of acute relapse but will not alter the length of recovery
139
how is ongoing relapse and remitting MS treated?
beta-interferon (it has been shown to reduce relapse rate by 30%)
Life style - regular exercise programmes and good sleep hygiene
if they are still complaining of fatigue - modafinil
sensory symptoms - gabapentin or pregabalin
Urine frequency - oxybutynin
antispasticity medication - baclofen
for tremor - propranolol
140
how is secondary progressive MS treated?
1st line - siponimod or methylprednisolone
141
what is myasthenia gravis?
Autoimmune disorder of the postsynaptic membrane at the neuromuscular junction in skeletal muscle resulting in insufficient functioning acetylcholine receptors and therefore impair neuromuscular transmission .
* type two hypersensitivity
Is often associated with thymic hyperplasia
common in woman aged 20-30
and common in men aged 60-70
often associated with RA and SLE
142
what are the clinical features of myasthenia gravis?
the key feature is muscle fatigability which become progressively worse on sustain or repeated activity and improve after rest.
Extraocular muscles
- ptosis and double vision(drooping eyelids and double vision) * pupils are spared.
Dysphagia (difficulting chewing or swallowing)
Dysarthria - changes to speech
facial paresis- changes in expression - flattened or transverse smile
proximal limb weakness - difficulty climbing upstairs.
there will be no evidence of muscle waisting.
143
what investigations would you perform fo MG?
serum acetylcholine receptor antibody analysis also MuSK and LRP4 antibodies
Muscle specific tyrosine fucntion tests
serial pulmonary function tests: MG crisis - low FVC and NIF
CT/MRI - to look for thymoma
single fibre electromyography
144
what is the management of MG?
long acting anticholinesterase inhibitors - pyridostigmine
Add prednisolone if needed
thymectomy
Immunosuppressants: Azathioprine usually offered first-line. Inhibits purine synthesis
145
what is the management of severe MG or acute crisis MG?
intubation and mechanical ventilation
plasmapheresis
prednisolone can be added.
146
what are some exacerbating factors of MG?
```
the most common factor is exertion
drugs which may exacerbate are:
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracycline
```
147
what is guillain-Barre syndrome?
immune mediated demyelination of the peripheral nervous system often triggered by an infection
148
what are the common infections that can lead to GBS?
```
cytomegalovirus
Epstein barr virus
hepB, C or HIV
campylobacter jejuni
Mycoplasma species
mycoplasma pneumomiae
```
149
what are the clinical features of GBS ?
progressive onset of limb weakness - usually symmetrical
it affects the lower extremities before upper and proximal before distal muscle
usually at its worst by 4 weeks
reflexes lost early on
often sensory symptoms (paraesthesia in the hands and feet)
Mild to severe involvement of respiratory and facial muscles
back and leg pain
bulbar dysfunction causing oropharyngeal weakness
extraocular muscle weakness
there may be autonomic involvement - urinary retention, diarrhoea
150
if proximal muscle weakness evolves for >4 to 8 weeks was would you suspect rather than GBS?
chronic inflammatory demyelinating polyradiculoneuropathy
151
what investigations would you perform if you suspected GBS?
nerve conduction studies - will show slowing of nerve conduction
Lumbar puncture - will show increased CSF protein, and usually normal WBC
LFTs - elevated AST and ALT
Spirometry
antiganglioside antibody
152
what is Miller Fisher syndrome ?
variant of Guillain-Barre syndrome
associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first
usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
anti-GQ1b antibodies are present in 90% of cases
153
how do you treat GBS?
IVIG
plasmapheresis
if decreased VC - then ventilation
154
what is motor neurone disease?
cluster of major degenerative diseases - causes loss of motor neurones
UMN and LMN are affected - but no sensory or autonomic disturbances.
155
what are the four different types of MND?
amyotrophic lateral sclerosis (50% of patients)
Primary lateral sclerosis
Progressive muscular atrophy
progressive bulbar palsy
156
what are the features of amyotrophic lateral sclerosis?
combination of UMN and LMN findings.
typical UMN findings: loss of coordination, spasticity, muscle spasms and hyper-reflexia.
spastic unsteady gait
LMN findings: weakness with atrophy and fasciculations
foot drop
increased lumbar lordosis
dyspnoea or orthopnoea from progressive diaphragmatic weakness
*genetic
Limb onset ALS
Bulbar onset ALS
respiratory onset ALS
eventually death usually due to resp failure
survival is usually 3-5 years but can be up to 10 years.
157
what investigations would you perform for MND?
mainly clinical diagnosis
```
but rule out other diseases
EMG
repetitive nerve stimulation
MRI brain and spine
vitamin b12
HIV test
genetic testing
```
158
what is the criteria for ALS?
El Escorial criteria
Clinically definite ALS - evidence for UMN and LMN in 3 segments of neuroaxis
Clinically probable ALS: evidence of UMN and LMN signs in at least 2 segments of neuroaxis
159
what are the features of primary lateral sclerosis?
it is an isolated UMN disorder, characterised by progressive weakness with associated spasticity
median survival 56 months
160
what are the features of progressive muscular atrophy?
LMN signs only characterised by progressive weakness, atrophy and fasciculations
161
what are the features 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
162
how is MND managed?
```
Riluzole
Ventilation support
Baclofen or tizanidine for spasticity
antidepressants
amitriptyline for drooling
```
163
what is Cerebral palsy?
an umbrella term referring to a non-progressive disease of the brain originating during antenatal, neonatal or early postnatal period when brain neuronal connections are still evolving
all patients present with motor impairment
164
what are some causes of cerebral palsy?
antenatal (80%) - cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)
intrapartum - (10%) birth asphyxia/trauma
postnatal (10%) - intraventricular haemorrhage, meningitis, head trauma
165
what are the features and diagnostic factors of cerebral palsy?
```
Delay in motor development - delayed motor milestones
Delay in speech development
delay in cognitive/intellectual development
Retention of primitive reflexes
lack of age appropriate reflexes
spasticity/clonus (80% )
toe walking
scissor gait
knee hyperextension
crouched gain
contratures
```
166
what are the different classifications of cerebral palsy?
Spastic: 70% - lesion in UMN
Dyskinetic - damage to basal ganglia (dystonia and chorea
Ataxic - damage to cerebellum - shaky and uncoordinated
Mixed
167
what investigations would you perform for cerebral palsy?
MRI brain
168
how is cerebral palsy managed?
MDT approach
OT/physio/speech therapy
Orthoses - bracing or splinting
treatment for spasticity - botulinum toxin type a injection
Bracing or casting or if needed orthopaedic surgery if required.
If dystonia - levodopa
169
what conditions is cerebral palsy often associated with?
learning difficulties
epilepsy
squints
hearing impairment
170
what is neurofibromatosis?
a genetic disorder that causes tumours to form on nerve tissue.
171
what is neurofibromatosis type 1?
An AD genetic disorder, chromosome 17 which encodes neurofibromin.
172
what are the clinical features of neurofibromatosis type 1?
cafe au lait spots
multiple neurofibromas
iris Lisch nodules
axillary freckling
unilateral diffuse plexiform neurofibroma divisions of the trigeminal nerve (3-5% of patients)
they often have pain
neurological deficit
compromised vision
173
what investigations would you perform for neurofibromatosis type 1?
MRI and or CT
PET scan
biopsy
genetic testing
174
how is neurofibromatosis treated?
surveillance and surgical removal
175
what are the clinical features of neurofibromatosis type 2?
Gene mutation on chromosome 22
bilateral vestibular schwannomas (acoustic neuroma)
multiple intracranial schwannomas
meningiomas and ependymomas
176
what are the different types of meningitis and the common causes of them?
```
Viral (common but self limiting)- enterovirus, mumps HSV
bacterial (rare but serious) - streptococcus pneumoniae, haemophilus influenzae type B, and neisseria meningitidis
Fungal meningitis (progressive and life threatening) - cryptococcus species.
```
177
what are the symptoms of viral meningitis?
```
headache
nausea and vomiting
photophobia
neck stiffness
fever
```
178
how is confirmed viral meningitis treated?
HSV or varicella zoster - antiviral - aciclovir
If CMV confirmed - ganciclovir
any other causes - supportive care
179
what are the features of fungal meningitis?
```
progressive headache
severe headache - due to raised ICP
nuchal rigidity
photophobia
symptoms of hydrocephalus
reduced visual acuity and papilloedema - signs of raised ICP
```
180
what are the common features of bacterial meningitis?
```
headache
neck stiffness
fever
altered mental status
confusion
photophobia
vomiting
seizures
```
in infants:
- hypothermia
- irritability
- poor feeding
- apnoea
181
how is bacterial meningitis treated?
less than one month - cefotaxime and ampicillin
1 month to 50 years - cefotaxime OR vancomycin and ceftriaxone
also add dexamethasone
older than 50 -ampicillin and vancomycin and cefotaxime
and add dexamethasone
confirmed meningococcal meningitis - benzylpenicillin or cefotaxime
Pneumococcal meningitis - cefotaxime
haemophilus influenzae meningitis - cefotaxime
Listeria meningitis - amoxicillin and gentamicin
IV dexamethasone should be given to reduce risk of neurological sequelae
182
who is prophylaxis for meningitis? and what prophylaxis is offered?
household and close contacts of patients with meningococcal meningitis.
given if they have had contact within 7 days before the onset
oral ciprofloxacin or rifampicin
183
what are the investigations for meningitis
```
FBC
CRP
CSF - protein, glucose, culture, gram stain
coagulation screen
blood culture
whole blood PCR
blood glucose
blood gas
CT head
```
184
what would the CSF show for different types of meningitis?
Bacterial
Cloudy appearance, low glucose, high protein, high polymorphs (neutrophils)
Viral
clear/clowdy appearance, glucose level may be low, protein is normal or elevated
TB
slightly cloudy, fibrin web , low glucose, high protein, high WCC
185
what is encephalitis?
inflammation of the brain parenchyma, cerebral function is usually abnormal with altered mental status and motor and sensory deficit
186
what is encephalopathy?
defined by a disruption of brain function without inflammation process in the brain (metabolic disturbances, hypoxia, drug)
187
what are the clinical features of encephalitis?
fever
headache
seizures
focal features - aphasia, hemianopia, hemiparesis, brisk tendon reflexes
altered mental state
some patients will have evidence of meningeal inflam with headache, photophobia and neck stiffness.
188
what are the causes of encephalitis ?
HSV-1 responsible for 95% of cases - typically affects temporal and inferior frontal lobes
others include:
enteroviruses, coxsackie virus
neisseria meningitides, TB, syphilis, listeria, measles, toga virus
189
what investigations would you perform for encephalitis?
```
FBC
peripheral blood smear
serum electrolytes
LFTs
throat swab
blood cultures
sputum culture
CT brain
MRI brain
EG
CSF analysis, culture, serology and PCR for HSV
```
190
how do you treat encephalitis?
viral aetiology
- aciclovir
may add ganciclovir
191
what is a squint?
Squint (strabismus) is characterised by misalignment of the visual axes. Squints may be divided into concomitant (common) and paralytic (rare)
192
what are the two types of squint?
concomitant - due to imbalance in extraocular muscle. Convergent is more common than divergent.
Paralytic - due to paralysis of extraocular muscles
193
what are the causes of squints?
concominat squints seems to be a complex genetic trait with the involvement of more than one gene
paralytic - related to malfunction of one or more of the 3 cranial nerves - oculomotor, trochlear, and abducens.
restrictive strabismus - graves disease and orbital fractured
sensory strabismus - reduced visual acuity in one eye
194
what are the different directions of squints?
Horizontal - esotropia (inwards, if nerve related would be abducens), exotropia (outward, if related to nerve would be oculomotor)
Vertical - hypertropia (upwards cranial nerve 4 - trochlear) , hypotropia (downwards, oculomotor)
195
how would you diagnose a squint?
```
cover test
ask the child to focus on a object
cover one eye
observe movement of uncovered eye
cover other eye and repeat test
```
196
how is a squint managed?
correct visual acuity
eye patches to prevent amblyopia
extraocular muscle surgery
197
what are the features of horner's syndrome?
miosis (small pupil)
ptosis
enophthalmos (sunken eye)
anhidrosis (loss of sweating one one side)
198
what is seen in congenital horners syndrome?
differences in iris colour
199
what are the different lesions and where would they cause anhidrosis in horner's syndrome?
central lesions (stoke, MS, tumour, encephalitis) - anhidrosis of the face, arm and trunk
preganglionic lesions (pancoast's tumour, thyroidectomy, trauma) - Anhidrosis of the face
Post ganglionic lesions - no anhidrosis (carotid artery dissection, carotid aneurysm, cavernous sinus thrombosis, cluster headache)
200
what is bells palsy?
acute, unilateral, idiopathic facial nerve paralysis.
| probable viral aetiology - HSV
201
what are the features of Bell's palsy?
LMN facial nerve palsy - forehead will be affected
usually a single episode
almost always unilateral - bilateral would suggest lyme disease, GBS, sarcoidosis etc...
absence of other symptoms such as fever, malaise, myalgia, headache, rash etc
dry eyes
post auricular pain
they may have altered taste
202
what is the management of Bells' palsy?
prednisolone
eye care is important - artificial tears
for severe presentation antiviral therapy can be given
203
what is trigeminal neuralgia?
chronic, debilitating condition resulting in
intense and extreme episodes of pain
Compression of the trigeminal nerve results in demyelination and excitation
of the nerve resulting in erratic pain signalling
204
what are the sensory and motor functions of the trigeminal nerve?
the sensory portion of the nerve supplies sensation to the face and scalp through 3 divisions (ophthalmic, maxillary and mandibular)
the motor roots travel with the mandibular division and supply the muscle of mastication
205
when is it common for trigeminal neuralgia to present?
peak incidence is between 50-60 years
more common in females than males
almost always unilateral
206
what causes trigeminal neuralgia?
in most cases it is due to compression of the trigeminal nerve by a loop of vein or artery
can also be due to local pathology such as aneurysms, meningeal inflammation, tumours (vestibular schwannoma) - local pathology is more common in younger people as a cause of compression
may also be due to fifth nerve lesion due to pathology in the brainstem (tumour, MS, infarction), in the cerebellopontine angle (acoustic neuroma, other tumour), within the petrous bone (spreading middle ear infection), within the cavernous sinus)
207
what are the risk factors for trigeminal neuralgia?
hypertension is the main risk factor
triggers: washing affected area, shaving, eating, talking and dental prostheses
208
what is the presentation of trigeminal neuralgia?
it is almost always unilateral
at least 3 attacks of unilateral facial pain
facial pain occurs in one or more distributions of the trigeminal nerve with no radiation beyond the trigeminal distribution
the pain has at least 3 of the following:
- reoccurring in paroxysmal attacks from a fraction of a second to 2 minutes
- severe intensity
- electric shock like, shooting, stabbing or knife like
- precipitated by innocuous (non-harmful) stimuli by the affected side - washing or shaving
209
what are the DD for trigeminal neuralgia?
giant cell arteritis/ temporal arteritis should be excluded
| dental pathology, temporomandibular joint dysfunction, migraine
210
how is trigeminal neuralgia diagnosed?
mainly a clinical diagnosis
- at least 3 attacks with unilateral facial pain
MRI to exclude other causes or pathologies
211
how is trigeminal neuralgia managed?
Typical analgesics and opioids DO NOT WORK
Anticonvulsants e.g. oral carbamazepine supress attacks in most patients
other less effective options include oral phenytoin, gabapentin and lamotrigine
may spontaneously remit after 6-12 month
if drugs fail then surgery may be needed
- microvascular decompensation
- gamma knife surgery
212
parkinson plus syndromes
Multi-system atrophy (MSA)
MSA is an adult-onset, rapidly progressive disease that is characterised by profound autonomic dysfunction leading to severe postural hypotension, urogenital dysfunction and a plethora of other features including cerebellar and corticospinal features. There is a poor response to treatment.
Progressive supranuclear palsy (PSP)
PSP is a neurodegenerative disorder that typically begins at age 50-60 years and is characterised by vertical gaze dysfunction, dysarthria and cognitive decline. Tremor is rare in this condition.
Dementia with Lewy-body (DLB)
DLB is characterised by early onset dementia (< 1 year) with features of parkinsonism. Dementia is usually the proceeding feature prior to motor symptoms and it is characterised by visual hallucination and fluctuating consciousness
```
Corticobasal degeneration (CBD)
CBD is a neurodegenerative disorder that is characterised by a progressive dementia, parkinsonism and limb apraxia. Apraxia refers to problems with motor planning (i.e. unable to wave hello).
```
In severe cases CBD may be associated with alien limb syndrome when there is a feeling that the limb is acting on its own.
