Neurology conditions Flashcards
1
Q
What is Bell’s palsy?
A
Acute unilateral peripheral facial nerve palsy, physical exam and history unremarkable.
Fully evolves within 72 hours
2
Q
What are the LMN causes of Bell’s palsy?
A
Idiopathic Pregnancy Diabetes Mellitus Iatrogenic Infective (Herpes) Ramsay hunt syndrome (Herpes zoster) Trauma Neurological Neoplastic Hypertension Sarcoidosis Sjorgen's Melkersson-Rosenthal syndrome
3
Q
What are the causes of UMN Bell’s palsy?
A
Cerebrovascular disease
Intracranial tumours
Multiple slcerosis
Syphillis
HIV
Vasculitides
IF B/L immunosuppression, GBS or lyme disease
Recurrent: Lymphoma, sarcoidosis, lyme disease
Children - Lyme disease and middle ear disease
4
Q
How can you tell the difference between LMN or UMN causes of Bell’s palsy?
A
A UMN bell’s palsy is forehead-sparing
Can still lift their eyebrows
5
Q
What are the risk factors for Bell’s palsy?
A
Intracranial influenza vaccinaiton Pregnancy Upper respiratory tract infection Black or Hispanic ancestry Arid/cold climate Hypertension Family history of Bell's palsy Diabetes
6
Q
What are the symptoms for Bell’s palsy?
A
Onset and progression of palsy Presence of fever Prior episodes of facial palsy Otological symptoms Presence of other cranial neuropathies Collagen vascular Use of neurotoxic medicaitons Known, prior or current malignancy Pregnancy Immunosuppression
7
Q
What are the signs of Bell’s palsy?
A
Head and neck examination
Cranial nerve exam
Eyebrow sparing
8
Q
What investigations are done for Bell’s palsy?
A
Serology (Lyme, Herpes and zoster)
Check BP in children
9
Q
Why do you check BP in children with Bell’s palsy?
A
Children present with facial palsy in Aortic coarctation
10
Q
What is the management for Bell’s palsy?
A
Steroids (prednisolone to people >16 within 72 hours)
Antivirals (moderate benefit)
Surgery (if no reduced paralysis)
11
Q
What are the complications for Bell’s palsy?
A
Keratoconjunctiva sicca
Ectropion (Sagging eyelid)
Contracture and synkinesis
Gustatory hyperlacrimation
12
Q
What are CNS tumours?
A
Tumours of the CNS
Cannot truly differentiate into benign and malignant as cause severe damage either way
13
Q
How do you differentiate CNS tumours?
A
High-grade
Low-grade
Metastases
14
Q
What makes a high-grade tumour?
A
Glioma and glioblastoma multiforme
Primary central lymphoma
Medulloblastoma
15
Q
What is a low-grade tumour?
A
Meningioma Acoustic neuroma Neurofibroma Pituitary tumour Craniopharyngioma Pineal tumour
16
Q
What cancers cause brain metastases?
A
Lung Breast Stomach Prostate Thyroid Colorectal
17
Q
What causes CNS tumours?
A
Arise from any cells in the CNS
e.g. glial cells, ependymal cells, oligodendrocytes
18
Q
What are the risk factors for CNS tumours?
A
Ionising radiation
Immunosuppression (e.g. HIV)
Inherited syndromes (e.g. Neurofibromatosis, tuberous sclerosis)
19
Q
What are the signs and symptoms of CNS tumours?
A
Depends on size and location of tumour Headache (Worse in morning and laying down) Nausea and vomiting Seizures Progressive focal neurological deficits Cognitive and behavioural symptoms Papilloedema
20
Q
What clinical features localise a tumour to the temporal lobe?
A
Dysphasia
Contralateral homonymous hemianopia
Amnesia
Odd/inexplicable phenomena
21
Q
Which clinical features localise a tumour to the frontal lobe?
A
Hemiparesis Personality change Broca's dysphasia Unilateral anosmia Concrete thinking Executive dysfunction
22
Q
What clinical features localise a tumour to the parietal lobe?
A
Hemisensory loss
Reduced 2 point discrimination
sensory inattention
Dysphasia
23
Q
What clinical features localise a tumour to the occipital lobe?
A
Contralateral visual field defects
24
Q
What are the investigations for CNS tumours?
A
CRP/ESR (eliminate causes)
CT/MRI/PET (visualise tumour)
MRI angiography (see blood supply to tumour)
Biopsy/Tumour removal
Distant mets are rare with primary CNS tumours
25
What are cluster headaches?
Neurological disorder characterised by recurrent, severe headaches on one side of the head typically around the eye, tending to recur over a period of weeks
26
What are the 2 types of cluster headaches?
Episodic
| Chronic
27
What are episodic cluster headaches?
Occur in periods lasting 7 days - 1 year
| Separated by pain-free periods lasting a month or longer
28
What are chronic cluster headaches?
Occurring for 1 year without remission or with short-lived remissions of less than a month.
Can arise de novo or from episodic cluster headaches
29
What causes cluster headaches?
Unknown
May be surperficial temporal artery smooth muscle hyperreactivity to 5HT
Genetic factor implicated (Autosomal Dominant gene has a role)
30
What are the risk factors for cluster headaches?
```
Male
Family history
Head injury
Smoking
Drinking
```
31
What are the signs and symptoms of cluster headaches?
Occur in clusters lasting 4-12 weeks
| Occur at night, 1-2hrs after sleeping`
32
What are the symptoms of cluster headaches?
```
Occurs rapidly around 10 mins
Intense, sharp and penetrating pain
Centred around eye, temple or forehead
Unilateral affects same side
Lasts 30-45 mins
Find it difficult to stay still and will pace around and bang their heads on things
```
33
What are the associated symptoms of cluster headaches?
Ipsilateral features:
- Ipsilateral lacrimation
- Rhinorrhoea
- Nasal congestion
- Eye lid swelling
- Facial swelling and flushing
- Conjunctival infection
- Partial Horner's syndrome
34
What triggers cluster headaches?
Alcohol
Exercise and solvents
Sleep disruption
35
What are the investigations of cluster headaches?
Clinical diagnosis based on history
| Neurological examination may be useful
36
What is Epilepsy?
Recurrent tendency to spontaneous, intermittent, abnormal electrical activity in part of the brain, manifesting as seizures (paroxysmal synchronised cortical electrical discharges)
37
What are the types of seizures?
```
Partial
- Complex
- Simple
Generalised
- Tonic-clonic
- Abscence
- Myoclonic
- Atonic
```
38
What are the structural causes of epilepsy?
```
Cortical scarring
Developmental
Space-occupying lesion
Stroke
Hippocampal sclerosis
Vascular malformations
```
39
What are the non-epileptic causes of seizures?
```
Trauma
Stroke
Haemorrhage
Raised ICP
Alcohol or Benzodiazepin withdrawal
Liver disease
Infection
High temperature
Drugs (Cocaine, tramadol, theophylline, tricyclics)
Metabolic disturbance
```
40
What are the 'other' causes of epilepsy?
Tuberculous sclerosis
Sarcoidosis
SLE
PAN
41
What are common things that look like seizures?
Syncope
Migraine
Non-epileptiform seizure disorder (e.g. dissociated disorder)
42
What are the types of frontal/partial seizure epilepsy?
Frontal lobe focal motor seizure
Temporal lobe seizure
Frontal lobe complex partial seizure
43
What are the signs and symptoms of frontal lobe focal motor seizure?
Motor convulsions
Jacksonian march
Post-ictal flaccid weakness (Todd's paralysis)
44
What is a jacksonian march?
Muscular spasm spreads from distal limb to ipsilateral face
45
What are the signs and symptoms of temporal lobe seizure?
Aura (Visceral or psychic symptoms)
| Hallucinations (Usually olfactory or affecting taste)
46
What are the signs and symptoms of frontal lobe complex partial seizure?
Loss of consciousness
Involuntary actions/disinhbition
Rapid recovery
47
What are the types of generalised seizures?
Tonic-clonic (Grand mal)
Abscence (Petit mal)
Non convulsive status epilepticus
48
What are the signs and symptoms of grand mal seizures?
Vague symptoms before attack (irritability)
Tonic phase (generalised muscle spasm)
Clonic phase (Repetitive synchronous jerks)
Faecal/urinary incontinence
Tongue bitings
Post-ictal phase: Impaired consciousness, lethargy, confusion, headache, back pain, stiffness
49
What are the signs and symptoms of petit mal seizures?
Onset in childhood
Loss of consciousness but maintained postures
The patient will appear to stop talking and stare
NO post-ictal phase
50
What are the signs and symptoms of non-convulsive status epilepticus?
Acute confusional state
Often fluctuating
Difficult to distinguish from dementia
51
What are the investigations for Epilepsy?
```
FBC
U&E
LFTs
Glucose
Calcium
Magnesium
ABG
Toxicology screen
Prolactin
EEG
CT/MRI
Other
```
52
How do you manage newly diagnosed epilepsy?
Start anti-convulsant treatment after >2 unprovoked seizures
Drugs change depending on type of epilepsy
- Generalised: Sodium valproate or Lamotrigine
- Abscence: Sodium valproate, lamotrigine or ethosuximide
- Partial: Carbamezapine
53
What is status epilepticus?
A seizure lasting >30 mins or repeated seizures without recovery and regain of consciousness in between
54
How do you manage status epilepticus?
```
ABC approach
Check glucose
IV lorazepam or IV/PR Diazepam
If they recur, use IV phenytoin - need ECG monitor
If fails consider general anaesthesia
Treat cause
Check plasma levels of anticonvulsants
```
55
What are the complications of epilepsy?
```
Fractures from tonic-clonic seizures
Behavioural problems
Sudden death
Complications of drugs:
- Gingival hypertrophy (Phenytoin)
- Neutropenia and osteoporosis (Carbamezapine)
- Stevens-Johnson syndrome (Lamotrigine)
```
56
What is an extradural haemorrhage?
Bleeding and accumulation of blood in the extradural space -between dura mater and skull
57
What causes an extradural haemorrhage?
Trauma
| - Usually due to fracture of the pterion area - rupturing the middle meningeal artery.
58
What are the risk factors for extradural haemorrhage?
Age
Bleeding tednency (E.g. haemophilia)
Drugs (warfarin)
59
What are the symptoms of extradural haemorrhage?
Temporary loss of consciousness /drowsiness
Then Lucid interval (resolved consciousness levels)
Then progressive deterioration in GCS as ICP rises
Increasing severe headache/vomiting/confusion/ fits +/- hemiparesis with brisk reflexes and upgoing plantars
Ipsilateral pupil dilation, coma deepens, b/l limb weakness
60
What are the signs of extradural haemorrhage?
```
Scalp traumas or fracture
Signs of raised ICP (dilated, unresponsive pupils)
Cushing's response
- Hypertension
- Bradycardia
- Irregular breathing
```
61
What are the investigations for extradural haemorrhage?
Urgent CT scan
- Check for haematoma, may get a contracoup injury on opposite side
- Look for features of raised ICP e.g. midline shift
62
What is Guillain-Barre syndrome?
GBS is a type of acute inflammatory neuropathy.
Clinically defined syndrome associated with motor difficulty, absence of deep tendon reflexes, paraesthesias without sensory losses and an increase in CSF albumin without cellular reaction
63
What are the subtypes of GBS?
Acute inflammatory demyelinating polyardiculoneuropathy
Acute motor and sensroy axonal neuropathy
Acute motor axonal neuropathy
Acute sensory neuropathy
Acute pandysautomnia
64
Which Antibodies are the types of GBS associated with?
AIDP - none
AMSAN - GM1, GM1b & GD1a
AMAN - GM1, GM1b, GD1a, GalNac-GD1a
Acute sensory neuropathy - GD1b
65
What are the risk factors for GBS?
History of GI or respiratory infections (1-3 weeks after)
Association with zika virus
Vaccinaitons
Malignancies (lymphomas)
Pregnancy (Decreases during pregnancy and increases after delivery)
66
What are the symptoms of GBS?
```
Weakness
Pain
Reflexes
Sensory symptoms
Autonomic symptoms
```
67
What are the signs of GBS?
```
Hypotonia
Demonstrate altered sensation or numbness
Reduced or absent refelxes
Fasciculation may occasionally be absent
Facial weakness (asymmetrical)
Autonomic dysfunction
Respiratory muscle paralysis
```
68
What investigations are done for GBS?
```
Electrolytes
Lumbar puncture (Raised CSF protein, no raised CSF cells, not until weakness)
Antibody screen
Spirometry
Nerve conduction studies
ECG
```
69
What is the management of GBS?
```
Plasma exchange
IV immunoglobulin
DVT prophylaxis
Admission to ICU
Pain relief
```
70
What are the complications of GBS?
Persistent paralysis
Respiratory failure requiring ventilation
Hypotension or Hypertension
Thromboembolism, pneumonia, skin breakdown
Cardiac arrhythmia
Ileus
Aspiration pneumonia
Urinary retention
Psychiatric problems e.g. depression, anxiety
71
What is Horner's syndrome?
Disruption of sympathetic nerves supplying the eye
72
What are the three things of Horner's syndrome?
```
Partial ptosis (upper eyelid drooping)
Miosis (Pupillary constriction leading to anisocoria)
Hemifacial anhidrosis (abscence of sweating)
```
73
What causes horner's syndrome?
Cranial nerve lesions
Preganglionic nerve lesions
Postganglionic nerve lesions
74
What causes cranial nerve lesions?
```
MS
Cerebrovascular accidents
Pituitary or basal skull tumours
Basal meningitis
Neck trauma
Syringiomyelia
Anrold-chiari malformation
Spinal cord tumours
```
75
What causes pre-ganglionic nerve lesions?
```
Apical lung tumours (Pancoast tumour)
Lympadenopathy
Lower brachial plexus trauma or rib
Aortic aneurysm
Trauma or surgical injury
Neuroblastoma
Mandibular dental abscess
```
76
What causes post-ganglionic nerve lesions?
```
Cluster headaches or migraines
Herpes zoster infectoin
Internal carotid artery dissection
Raeder's syndrome
Carotid-cavernous fistula
Temporal arteritis
```
77
What investigations should be done in Horner's syndrome?
CXR
CT/MRI
CT angiography/Carotid ultrasound
78
What is the pharmacological testing for Horner's?
- Cocaine eye drops normally cause dilatation of eyes but wont in horners
- Apraclonidine is an alternative to cocaine - dilates a horner's pupil but not a normal one
- Hydroxyamfetamine causes dilatation in first and second order lesions but not third
79
What is the management of horner's
Treat underlying cause
80
What is Huntington's disease?
Autosomal dominant trinucleotide repeat disease (CAG repeat on chromosome 4)
81
What characterises huntington's disease?
Progressive chorea and dementia
| Typically starts in middle age
82
What causes huntington's disease?
The huntingtin gene codes for huntingtin
In the huntingtin gene there is a trinucleotide repeat expansion that results in toxic gain of function
Causes atrophy and neuronal loss of striatum and cortex
Autosomal dominant
Earlier age of onset with each generation
83
What are the symptoms of huntington's disease?
```
Family history
Insidious onset in middle age
Progressive
Early symptoms
Late symptoms
Drug history (cocaine, antipsychotics)
```
84
What are the early symptoms of huntington's disease?
```
Lability
Dysphoria
Irritability
Incoordination
Fidgeting
Clumsiness
Mental inflexibility
Anxiety
Develops in dementia
```
85
What are the late symptoms of huntington's disease?
```
Rigid
Involuntary, jerky, duskinetic movement soften accompanied by grunting and dysarthria (chorea)
Dementia
Fits
Akinetic
Bed-bound
Death
```
86
What are the signs of huntington's disease?
```
Chorea
Dysarthria
Slow voluntary sacades
Supranuclear gaze restriction
Parkinsonism
Dystonia
MMSE shows cognitive and emotional deficits
```
87
What are the investigations for huntington's disease?
Genetic analysis
- Diagnosed if >39 CAG repeats
- Reduced penetrance leads to intermediate number of CAG repeats
Imaging
- Brain MRI/CT show symmetrical atrophy of striatum and butterfly dilation of the lateral ventricles
Bloods
88
What is Hydrocephalus?
Enlargement of the cerebral ventricular system due to accumulation of CSF.
Too much CSF produced, blocked CSF flow or insufficient CSF reabsorbing
89
What are the types of CSF?
Non-obstructive / communicating
Hydrocephalus ex vacuo
Obstructive / non-communicating hydrocephalus
90
What is a communicating hydrocephalus?
Impaired CSF reabsorption into the subarachnoid villi
91
What is a hydrocephalus ex vacuo?
Apparent enlargement of the ventricles as a compensatory change due to brain supply
92
What is a non-communicating hydrocephalus?
Blockage of CSF within the ventricles
93
What are risk factors for hydrocephalus?
```
Congenital:
- Premature birth causing bleeding in ventricles
- Uterus infection
- CNS abnormal development
Acquired:
- CNS infeciton
- Bleeding in brain from trauma/stroke
- Age >65 years
- Vascular disease
```
94
What are the symptoms of obstructive/communicating hydrocephalus?
Acute drop in GCS
| 6th nerve palsy causing diplopia
95
What are the symptoms of normal pressure hydrocephalus?
Dementia
Gait disturbance
Urinary incontinence
96
What are the signs of obstructive hydrocephalus?
Low GCS
Papilloedema
6th nerve palsy
97
Why is the 6th cranial nerve most susceptible to raised ICP?
It has the largest intracranial path
98
What are the signs of obstructive jaundice in neonates?
```
Increased head circumference
Sunset sign (downward cojugate deviation of the eyes)
```
99
What investigations are done for Hydrocephalus?
CT head (First line)
CSF
- Ventricular drain or LP, indicate pathology
LP (Contraindicated in raised ICP, therapeutic in normal pressure hydrocephalus)
100
What is a migraine?
Chronic, episodic, neurological disorder that presents in early-to-mid life
101
What causes migraines?
Brains are hyperexcitable to a variety of stimuli
102
What are the risk factors for migraines?
```
Family history of migraine
High caffeine intake
Exposure to barometric pressure
Female sex
Obesity
Habitual snoring
Stressful life events
Overuse of headache movements
Lack of sleep
Low socio-economic status
Allergies or asthma
Hypertension
Hypothyroidism
Diet
```
103
What are the symptoms for migraine?
Visual or other aura
Aura without headache
Episodic severe headaches without aura, often premenstrual, U/L, with allodynia
104
What are the signs for migraine?
None
105
What are the criteria for migraines without aura?
```
>/=5 headaches lasting 4-72 hours
Nausea / vomiting
Photo/phonophobia
With 2 of:
- Unilateral
- Pulsating
- Abnormalities suggesting another cause
```
106
What are the investigations for Migraines?
```
ESR
LP
CSF culture
CT head
MRI brain
```
107
What are red flags seen on examination for migraines?
Papilloedema
New seizure
Abnormal neurological signs
New-onset cluster headaches
108
What are the orange flags seen on examination for migraines?
New headache and no other clear diagnosis
Headache aggravated by exertion or valsalva
Headache with vomiting
New headache in 50+
Changed pattern of headaches
109
What are the yellow flags for migraines?
Migraine
| Memory loss
110
What is the management for migraines?
Relieve symptoms (NSAIDs > Triptans) - if not, paracetamol
Anti-emetics for N&V
High flow O2 via non-rebreather mask
Reduce frequency and severity of migraine attacks
Identify trigger factors to avoid them
Prophylaxis if occurs >2/month
111
What can be done as prophylaxis for migraines?
Start low-dose and re-evaluate after trial
Non-pharmaceutical help: CBT, Biofeedback- assisted relaxation
Treat for 4-6 months
Menstrual migraine > hormonal therapy to suppress menses
Main drugs: Anticonvulsants (topiramate), tCAs and Beta blockers
CCBs for hemiplegic migraine and migraine with brainstem aura
112
What are the complications for migraines?
```
Status migrainosus
Migrainous infarction
Migraine-triggered seizures
Depression
Chronic migraine
Persistent aura without infarciton
```
113
What is motor neuron disease?
A progressive neurodegenrative disorder of cortial, brainstem and spinal motor neurons (LMN and UMN) - this is selective loss of neurons in more cortex, cranial nerve nuclei and anterior horn cells!
- No sesnory loss
114
What are the subtypes of MND?
Amyotrophic lateral sclerosis (ALS)
Progressive muscular atrophy variant
Progressive bulbar palsy variant
Primary lateral sclerosis variant
115
What is ALS?
Lou gherig's disease
| Combined degeneration of upper and lower motor neruoens resulting in a mix of LMN and UMN signs
116
What is progressive muscular atrophy variant MND?
Only LMN signs
| Better prognosis
117
What is progressive bulbar palsy variant MND?
```
Only affects CN IX-XII
Dysarthria
Dysphagia
Wasted fasciculating tongue
Brisk jaw jerk reflex
```
118
What is primary lateral sclerosis variant MND?
```
Loss of Betz cells in motor cortex
UMN pattern of weakness
Brisk reflexes
Extensor plantar responses
No LMN signs
```
119
What causes motor neuron disease?
Unknown
Free radical damage and gluatamate excitotoxicity have been implicated
Associated with frontotemporal lobar dementia
120
What are the risk factors for MND?
Genetic predisposition/family history
Age > 40
Smoking
Athleticism/ family history
121
What are the symptoms of MND?
Weakness of limbs (proximal myopathy)
Speech disturbance
Swallowing disturbances (choking on food)
Behavioural changes
122
What are the signs of MND?
```
Combo of UMN and LMN signs
LMN
- Muscle wasting
- Fasciculations
- Flaccid weakness
- Hyporeflexia
UMN
- Spastic weakness
- Extensory plantar response
- Hyperreflexia
Sensory exam is normal
Frontotemporal dementia occurs in 25%
```
123
What are the investigations for MND?
Clinical diagnosis
EMG: Diffused/ongoing/chronic denervation seen
Nerve-conduction studies
MRI to exclude cord compression/brainstem lesions
Bloods
- Mild elevation of CK
- ESR (Raised)
- Anti GM1-ganglioside antiboides (Negative)
124
What is multiple sclerosis?
Inflammatory demyelinating disease of the CNS - discrete plaques of demyelination occur at multiple CNS sites, from T-cell mediated immune response
125
What are the types of MS?
Relapsing-remitting MS
Clinically isolated syndrome
Primary progressive MS
Marburg variant
126
What is relapsing-remitting MS?
Commonest form
| Clinical attacks of demyelination with poor healing in between attacks
127
What is Clinically isolated syndrome?
Single clinical attack of demyelination
The attack itself doesn't count as MS
10-50% progress to MS
128
What is Primary progressive MS?
Steady accumulation of disability with NO relapsing-remitting MS
129
What is Marburg variant MS?
Severe fulminant variant of MS leading to advanced disability or death within weeks
130
What causes MS?
Unknown
AI bases with potential environmental trigger in genetically susceptible individuals
Immune-mediated damage to myelin sheaths results in impaired axonal conduction
131
What are the risk factors of MS?
EBV exposure
| Prenatal vitamin D levels
132
What are the symptoms of MS?
```
Varies on site
Usually monosymptomatic
Optic neuritis (most common)
Sensory
Motor
Autonomic
Psychological/cognitive
Sexual
GI
Cerebellum
Early on, relapses then remits to full recovery
Uhthoff's sign
Lhermitte's sign
```
133
What are the optic neuritis symptoms?
Unilateral deterioration of visual acuity and colour perception
Pain on eye movement
Rapid loss of centrla vision
134
What are the sensory symptoms of MS?
Pins and needles
Numbness
Burning
135
What are the motor symptoms of MS?
Limb weakness
Spasms
Stiffness
Heaviness
136
What are the autonomic symptoms of MS?
Urinary urgency
Hesitancy
Incontinence
Impotence
137
What are the psychological/cognitive symptoms of MS?
Depression
Psychosis
Amnesia
Reduced executive fucntioning
138
What are the sexual symptoms of MS?
Erectile dysfunction
| Anorgasmia
139
What are the GI symptoms of MS?
Swallowing disorders
| Constipation
140
What are the cerebellar symptoms of MS?
Trunk and limb ataxia
Intention tremor
Scanning speech falls
141
What is Uhthoff's sign?
Worsening of neurological symptoms as body gets overheated from hot weather, saunas, hot tubs etc
142
What is Lhermitte's sign?
Electrical sensation that runs down the back and into the limbs when neck is flexed
143
What are the signs of MS?
```
Optic neuritis
Visual field testing
RAPD
Internuclear ophthalmoplegia
Paraesthesia
UMN signs
Limb ataxia
Dysdiadochokinesia
Ataxic wide-based gait
Scanning speech
Central scotoma
Loss of coloured visions
Impaired visual acuity
```
144
What are the investigations for MS?
Diagnosis based on finding of 2 or more CNS lesions with corresponding symptoms, seperated in time and space - Mcdonald criteria!
Lumbar puncture
- Microscopy (exclude infection/inflammatory causes)
- CSF electrophoresis shows unmatched oligoclonal bands
MRI brain
- Plaques identifies
- Gadolinium enhancement shows active lesions
Evoked potentials
- Visual, auditory and somatosensory evoked potentials may show delayed conduction velocity
145
What is Myasthenia gravis?
An AI disease affecting the neuromuscular junction producing weakness in skeletal muscles
146
What causes myasthenia gravis?
Antibodies against the nicotininc acetylcholine receptor which interferes with the neuromuscular transmission via depletion os post-synaptic receptor sites
Can be paraneoplastic
147
What is Lambert-Eaton syndrome?
Paraneoplastic subtype of MG caused by auto antibodies against pre-synaptic calcium channels, leading to impairment of acetylcholine release
148
What are the risk factors for MG?
AI conditions
149
What are the symptoms of MG?
```
Muscle weakness that worsens with repetitive use or towards the end of the day - fatiguability
Ocular symptoms
- Drooping eyelids
- Diplopia
Bulbar symptoms
- Facial weakness (myasthenic snarl)
- Disturbed hypernasal speech
- Difficulty smiling, chewing or swallowing
```
150
What is the order of muscles that become weakened?
Extraocular > Bulbar > Face > Neck > Limb girdle > Trunk
In lambert-eaton syndrome fatiguability improves with use
151
What are the signs of MG?
```
May be generalised
May be bulbar (relating to medulla oblongatea)
May be ocular
Eye signs
- Ptosis
- complex opthalmoplegia
- ocular fatigue
Ice on eyes test (putting ice packs on eyelids for 2 mins improves transmission and reduces ptosis)
Bulbar signs (reading aloud causes dysarthria or nasal speech)
Limbs
- Test power
- tendon reflexes are normal
```
152
What are the investigations for MG?
```
CK (Exclude myopathies)
Serum acetylcholine receptor antibody
If seronegative
- TFTs
- Anti-voltage gated calcium channel antibodye
Tensilon test (Not used)
Nerve conduction study
EMG
CT thorax/CXR
- visualise thymoma or lung malignancies
```
153
What is Neurofibromatosis?
An Autosomal dominant genetic disorder affecting cells of neural crest origin, resulting in the development of multiple neuroctaneous tumours
There are 2 types
154
What is type 1 neurofibromatosis?
Von Recklinghausen's disease
- Peripheral and spinal neurofibromatosis
- Multiple cafe au lait spots
- Freckling (axillary/inguinal)
- Optic nerve glioma
- Lisch nodules (on iris)
- Skeletal deformities
- Phaeochromocytomas
- Renal artery stenosis
155
What is Neurofibromatosis type 2?
A condition characterised by:
- Schwannomas
- Meningiomas
- Gliomas
- Cataracts
156
What causes neurofibromatosis?
Associated with mutations in tumour suprression genes NF1 and NF2
157
What are the risk factors for neurofibromatosis?
Severe crush trauma
| Parent with neurofibromatosis
158
What are the symptoms of type 1 neurofibromatosis?
```
Family history
Skin lesions
Learning difficulties (40%)
Headaches
Disturbed vision (optic gliomas)
Precoccious puberty (lesions of pit. gland from an optic glioma)
```
159
What are the symptoms of type 2 neurofibromatosis?
```
Family history
Hearing loss
Tinnitus
Balance problems
Headache
Facial pain
Facial numbness
```
160
What are the signs of type 1 neurofibromatosis?
5+ cafe au lait macules of >5mm (prepubertal)
5+ cafe au lait macuels of >15mm (Post-pubertal)
Freckling in armpit or groin
Lisch nodules (hamartomas on the iris - slit lamp)
Spinal scoliosis
Short stature
161
What are the signs of type 2 neurofibromatosis?
Few or no skin lesions
| Sensorineural deafness with facial nerve palsy or cerebellar signs (if schwannoma is large)
162
What are the investigations for neurofibromatosis?
```
Full body exam for skin lesions
Ophthalmological assessment
Audiometry
MRI brain and spinal cord
Skull X-ray (sphenoid dysplasia in NF1)
Genetic testing
```
163
What is Parkinsons's disease?
A neurodegenerative disorder
164
What is the triad of parkinsonism?
Tremor
Rigidity/increased tone
Bradykinesia/hypokinesia
165
What causes primary parkinson's disease?
Genetic component, subsequent environmental factors/exposure contributing to evolution of clinical disease
166
What causes secondary parkinson's disease?
Neuroleptic therapy (e.g. schizophrenia)
Vascular insults (e.g. in basal ganglia)
MPTP toxin from illicit drug contamination
Post-encephalitis
Repeated head injury
Manganese of copper toxicity (wilson's diseae)
HIV
167
What are the risk factors for parkinson's disease?
Increasing age
History of familial PD in younger-onset disease
Mutation in gene encoding glucocerebrosidase
Metal exposure
Male
Head truama
Toxin exposure
Occupation (Teacher, HCP, construction worker, carpenter or cleaner)
168
What are the key diagnostic features of signs and symptosm?
```
Presence of risk factors
Bradykinesia
Resting tremor
Rigidity
Postural instability
```
169
What are the other diagnostic features of parkinson's disease?
```
Masked facies
Hypophonia
Hypokinetic
Micrographia
Stooped posture
Shuffling gait
Conjugate gaze disorders
Fatigue
Constipaiton
Depression
Anxiety
Dementia
```
170
What are the uncommon features of parkinson's disease?
Exposure to Neuroleptics or antiemetics
| Featrues of atypical parkinsonism (Acute onset, rapid progression, cognitive impairment)
171
What are the investigations for Parkinson's disease?
Dopaminergic agent trial (improved symptoms)
MRI brain (normal)
Functional neuroimaging
Olfactory testing
Brain pathology
Genetic testing
Lewy bodies and positive reactivity to synuclein with immunohistochemical staining
172
What is Radiculoapthy?
Spinal nerve root compression
173
What are the most common types of radiculopathy?
Lumbar and cervical radiculopathy
174
What are the causes of radiculopathy?
```
Herniated disc
Spinal stenosis
Degenerative disc disease
Osteoarthritis
Facet joint degeneration/hypertrophy
LIgamentous hypertrophy
Spondylolisthesis (vertebra moves and rests on vertebrae below)
Rarer: Radiation, DM, Neoplastic disease, lyme meningitis
```
175
What are the risk factors for radiculopathy?
```
Age
Overweight
Poor posture
Improper lifting technique
Family history of degenerative bone conditions
```
176
What are the signs and symptoms of radiculopathy?
Lumbar or sacral radiculopathy (primary sciatica)
Pain/electrical sensations in arms or fingers or legs at level of compression
Dull reflexes
Dynatomal sensory disturbance (numbness, tingling, reduced pain and temperature sensation)
LMN weakness and eventual muscle wasting
UMN signs below affected level (Spasticity, weakness, brisk reflexes and upgoing plantars)
Position and vibration sense may be lost
177
What are the investigations for Radiculopathy?
```
Full Neurological exam
MRI
X-ray and MRI
EMG to test nerve function
Nerve conduction studies
```
178
What is Raised intracranial pressure?
The volume inside the cranium is fixed so any increase in the contents can lead to raised ICP/ Can be mass efect, oedema or obstruction to fluid outflow
Normal ICP in adults is <15mmHg
179
What causes raised intracranial pressure?
```
Primary or metastatic tumours
Head injury
Haemorrhage
Infection (Meningitis, encephalitis, brain abscesses)
Hydrocephalus
Cerebral oedema
Status epilepticus
Aneurysms
```
180
What are the symptoms of a raised ICP?
```
Headache (worse on coughing and leaning forward, worse in morning)
Vomiting
Altered GCS
History of trauma
Poor vision
```
181
What are the signs of a raised ICP?
Altered GCS
Falling pulse and rising BP (Cushing's response)
Cheyne-Stokes respiration
Pupil changes
Reduced visual acuity
peripheral visual field loss
Papilloedema (unreliable sign but venous pulsaiton of disc may be absent)
182
What is Cheyne-stokes respiration?
Progressively deeper and sometimes faster breathing followed by a gradual decrease that results in a temporary stop in breathing
183
What are the investigations for Raised ICP?
```
U&Es
FBC
LFT
Glucose
Serum osmolality
Clotting
Blood culture
Consider toxicology screen
CXR
CT head
Consider LP if safe
```
184
What is spinal cord compression?
Pressure injury to the spinal cord with Neurological symptoms dependent on site and extent of the injury
185
What is Cauda equina syndrome?
Symptoms due to damage to the bundle of nerves below the end of the spinal cord (L2 down)
186
What is conus medullaris syndrome?
Tapered, lower end of the spinal cord i the conus medullaris.
Conus medullaris syndrome is compression at L1/L2
187
What causes spinal cord compression?
```
Most causes: Trauma and tumours
Tumours are more frequently metastases
Spinal abscess
TB (Pott's disease)
Haematoma (warfarin)
Intrinsic cord tumour
Atlanto-axial sublulxation
Myeloma
Congenital lumbar disc disease
```
188
What are the risk factors for spinal cord compression?
```
Trauma
Malignancy
Osteoporosis
Metabolic bone disease
Vertebral disc disease
```
189
What are the symptoms of spinal cord compression?
```
History of trauma or malignancy
Pain
Weakness
Sensory loss
Disturbance of bowel and bladder function
```
190
What are the signs of spinal cord compression?
```
Normal above lesions
Diaphragmatic breathing
Reduced anal tone
Hyporeflexia
Priapism
Spinal shock
Sensory loss (level of lesion)
Motor
- Weakness or paralysis
- Downward plantars (acute)
- UMN signs below lesions
- LMN signs at level of lesion
```
191
What is Brown-Sequard syndrome?
Seen with hemisection of spinal cord
192
What are the investigations for spinal cord compression?
Radiology
- Lateral radiographs of spine to look for loss of alignment, fractures etc
- MRI
Bloods
- FBC, U&E, Calcium, ESR, Immunoglobulin electrophoresis (Multiple Myeloma), syphilis serol, LFTs, PSA
- CXR for lung malignancy, TB
- Urine (Bence Jones proteins - multiple myeloma)
193
What is a stroke?
Acute neurological deficit of two different types
194
What are the types of stroke?
Ischaemic or Haemorrhagic
195
What is an ischaemic stroke?
Acute neurological deficit lasting more than 24 hours and caused by cerebrovascular aetiology caused by vascular occlusion or stenosis.
Resulting in intraparenchumal and/or subarachnoid haemorrhage
196
What is a Haemorrhagic stroke?
Acute neurological deficit caused by cerebrovascular aetiology. Due to a rupture of a cerebrospinal artery, resulting in intraparenchymal subarachnoid and intraventricular haemorrhage
197
What causes an ischaemic stroke?
Transient of permanent critical reduction in cerebral blood flow due to arterial occlusion, Hypotension, vasculitis or cocaine
198
How does thrombosis cause ischaemic strokes?
Lacunar infarcts (Small vessels)
Can occur in larger vessels (middle cerebral artery)
Can arise in prothrombotic states (e.g. dehydration, thrombophilia)
199
How does an embolus cause ischaemic stroke?
Carotid dissection
Carotid atherosclerosis
AF
200
What causes a haemorrhagic stroke?
```
Hypertension
Charcot-Bouchard microaneurysm rupture
Amyloid angiopathy
Arteriovenous malformaitons
Anticoagulant therapy
Trauma
Tumours
Vasculitis
```
201
What are the risk factors for ischaemic stroke?
```
Older age
Family history
History of stroke
Hypertension
Smoking
Diabetes mellitus
Atrial fibrillaiton
Comorbid cardiac conditions
Carotid artery stenosis
Sickle cell disease
Dyslipidaemia
People with lower levels of education
Obesity
Elevated CRP
Aortic arch plaques
```
202
What are the risk factors for haemorrhagic stroke?
```
Hypertension
Advanced age
Male sex
Asian, black and/or hispanic
Family history of heamorrhagic stroke
Asian, black or hispanic
Haemophilia
Smoking
Illicit sympathomimetic drugs
Leukaemia
Thrombocytopeina
AD mutaitons
Hereditary haemorrhagic telangiectasia
Leukaemia
Moyamoya disease
NSAID
```
203
What are the symptoms of ischaemic stroke?
```
Sudden onset
Vision loss or visual field deficit
Impaired co-ordination
Loss of function
Diplopia
Sensory loss
Vertigo/dizziness
Nausea and / or vomiting
Confusion
altered sensation
Headahce
Neck/facial pain
```
204
What are the signs of ischaemic stroke in the anterior cerebral artery?
Hemiparesis
Confusion
Disturbance of intellect, executive function, judgement and social behaviour
205
What are the signs of ischaemic stroke in the middle cerebral artery?
```
Facial weakness
Hemiparesis
Hemisensory loss
Apraxia
Hemineglect
Aphasia if left sided
Hemineglect
Quadrantopia
```
206
What are the signs of ischaemic stroke in the posterior cerebral artery?
Homonymous hemianopia, visual agnosia
207
What are the symptoms of haemorrhagic stroke?
```
Neck stiffness
History of AF
History of liver disease
Altered sensation
weakness
Headache
Sensory loss
Aphasia
Dysarthria
Ataxia
Vertigo
Nausea/vomiting
Confusion
Gaze paresis
Altered consciousness
```
208
What are the signs of haemorrhagic stroke?
```
Meningism
Severe headache
Coma within hrs
Carotid bruit
AF
previous TIA
IHF
```
209
What investigations are done for ischaemic stroke?
```
FBC
Serum glucose (normal)
Serum electrolytes (normal)
Serum U&Es (Normal)
Cardiac enzymes (normal)
PTT/aPTT (Normal/coagulopahty)
Toxicology screen
ESR
Carotid doppler US
CT head MRI brain ECG
CXR
CT cerebral angiogram
```
210
What are the investigations for haemorrhagic stroke?
```
Chemistry panel (normal)
CBC (exclude thrombocytopenia)
Clotting test
Platelet function test
LFT
Non-infused CT
ECG
CT angiography and venography
MR angiography and venography
MRI brain with diffusion-weighted imaging adn gradient echo sequence
```
211
What is the management for stroke?
<4.5 hrs since onset:
- CT Head to exclude haemorrhage
- IV alteplase
- Aspirin 24 hrs later
>4.5hrs since onset;
- 300mg Aspirin (heparin if high emboli recurrence)
- Swallow assessment
- Thromboprophylaxis
Secondary:
- clopidogrel / aspirin 75mg
- warfarin if paroxysmal AF
- control risk factors
Surgical:
Carotd endarterectomy
212
What are the complications of stroke?
```
Aspiration pneumonia
Cerebral oedema
Immobility
Infections
DVT
Cardiovascular events
Death
```
213
What is a subarachnoid haemorrhage?
Arterial haemorrhage into the subarachnoid space
214
What causes a subarachnoid haemorrhage?
Rupture of a saccular aneurysms at base of brain (berry aneurysm) - 85%
Perimesencephalic haemorrhage - 10%
Arteriovenous malformations, bleeding diathesis, vertebral artery dissection - 5%
215
What are the risk factors for a subarachnoid haemorrhage?
```
Hypertension
Smoking
Excessive alcohol intake
Bleeding disorders
Family history of SAH
Saccular aneurysms
```
216
What are saccular aneursysms associated with?
```
PKD
Coarctation of he aorta
Marfan's syndrome
Ehlers-Danlos syndrome
SLE
```
217
What are the symptoms of SAH?
```
Sudden-onset worst headache ever
Nausea/vomiting
Collapse
Seizures
Neck stiffness
Photophobia
Reduced level of consciousness
```
218
What are the sign's of SAH?
Meningism (Neck stiffness, kernig's sign, pyrexia)
GCS - Check for deterioration
Signs of raised ICP
- Papilloedema, CN IV/III palsies, HTN, bradycardia, focal neurological signs
219
What investigations are done for SAH?
```
FBC (Leucocytosis)
INR (Raised)
PTT (Raised)
Troponin (High)
CT scan asap
if inconclusive - LP (increased pressure, incerased red cells, blood then xanthochromia)
ECG: 50% have an abnormal ECG
```
220
What is a Subdural haemorrhage?
A collection of clotting blood that forms in the subdural space
221
What are the 3 classifications of subdural haemorrhage?
Acute
Subacute (3-7 days after)
Chronic (2-3 weeks after)
222
Who are the at risk groups of SDH?
Infants
Elderly
Alcoholics
People on anticoagulation treatment
223
What causes SDH?
Trauma
| Less commonly: Rupture of aneurysm or vascular malformations
224
What are the risk factors for SDH?
Recent trauma
Coagulopathy and anticoagulant use
Advanced age (>65)
225
What are the symptoms of acute SDH?
History of trauma
| Reduced consciousness level
226
What are the symptoms of subacute SDH?
Worsening headache 7-14 days after injury
| Altered mental state
227
What are the symptoms of chronic SDH?
```
Headache
Confusion
Cognitive impairment
Gait deterioration
Focal weakness
Seizures
Sleepiness
```
228
What are the signs of acute SDH?
```
Reduced GCS
Ipsilateral fixed dilated pupil
Pressure on brainstem (reduced consciousness + bradycardia)
Evidence of trauma
Papilloedema
```
229
What are the signs of chronic SDH?
Neurological examination may be normal
Focal neurological signs (3rd nerve palsy)
Evidence of trauma
Papilloedema
230
What investigations are done for SDH?
```
FBC
U&Es
LFTs
Thrombocytopenia
Coagulation screen
Cross-match/Group and save
CT head
C-spine imaging
```
231
What is the management of acute SDH?
```
ALS protocol
Watch out for C-spine injury
Raised ICP consider osmotic diuresis
Conservative if small
Surgical (for irrigation/evacuation)
- Burr twist drill and burr hole craniotomy
- Craniotomy
```
232
What is the management for chronic SDH?
Burr hole or craniotomy and drainage
Assess any causes of trauma
Children - treat with percutaneous aspiration via open fotanelle
233
What are complications of SDH?
```
Neurological deficits
Come
Stroke
Surgical-site infection
Epilepsu
Recurrence of SDH postoperatively
```
234
What is a tension headache?
Generalised headache throughout the head with a predilection for involving the frontal and occipital regions.
Pain expressed as being a 'tight-band' around the head
TTH can be episodic or chronic
235
What causes tension headache?
Muscle contraction considered cause of pain
Stress most common trigger
Disturbed sleep patterns can trigger episodic headache
Insomnia and other sleep disorders are associated with chronic headache
236
What are the risk factors for headaches?
```
Mental tension
Stress
Missing meals
Fatigue
Somatisation
Female sex
20-39 years old
Lower socioeconomic status
Analgesic overuse
```
237
What are the signs and symptoms of tension headache?
Pressure or tightness
Related to neck pain
Non-pulsatile head pain
Often photophobia and exacerbation by movement are common
Mild nausea may occur
Head pain, frontal or occipital head pain
238
What are the investigations for tension headache?
Not needed but can do CT sinus, MRI brain and LP which would all be normal
239
What is the management for tension headaches?
Reassure positive diagnosis
Attention to stress, anxiety or depression
Physiotherapy for mobilisation
Advice on opioids/medications
Simple analgesia
- Ibuprofen fine, possibly paracetamol or naproxen, aspirin
TCA
- Amitriptyline
240
What is a TIA?
Episode where someone has signs or symptoms of a stroke but they only last a short amount of time.
Usually last a few minutes - few hours
241
What are the risk factors for TIA?
```
Diabetes
Age >40
Severely clogged or narrowed arteries
Obesity
Heart disease
Illegal drug use or heavy alcohol use
Recent childbirth
Sedentary lifestyle/lack of exercse
Current or past history of blood clots
High blood cholesterol
```
242
What are the signs and symptoms of TIA?
Single or many attacks
Similar in the arterial area to a stroke there
Amaurosis fugax if goes to retinal artery
May have limb-shaking
Syncope, dizziness not typical in TIAs
243
What are the investigations for TIA?
```
Blood glucose (<3.3)
U&Es (Low Na+, K+ or High Ca2+)
FBC (possibly raised, polycythaemia, thrombocytopenia)
Brain MRI [First line]
CXR
```
244
How do you manage TIA?
ABCD2 assessment
- If = 3: Aspirin or clopidogrel adn a statin
- If >/= 4: Aspirin or clopidogrel, measure for secondary CVS prevention
Those with AF should be anticoagulated
Cardiac emboli / Af need warfarin
Carotid endarterectomy if >/= 70% stenosis on internal carotid
245
How does a TIA affect their ability to drive?
```
Group 1 (Car/motorcycle):
- Not drive for a month, no need to tell DVLA for 1 TIA, multiple needs 3 months off
```
```
Group 2 (lorry or bus):
- Licence refused/revoked for a year
```
246
What are the complications for TIAs?
Stroke
| Myocardial infarction
247
What is trigeminal Neuralgia?
Neuralgia involving one or more of the brances of the trigeminal nerves, often causing severe pain
248
What triggers trigeminal Neuralgia?
```
Vibration
Skin contact
Eating
Talking
Dental protheses
Brushing teeth
Exposure to wind
```
249
What causes trigeminal neuralgia?
Thought to be due to compression of the trigeminal nerve by a loop of artery or vein
Secondary causes (5-10%)
Can be MS or Brainstem lesions but thats rare
Compression of the trigeminal root by anomalous or aneurysmal intracranial vessels or a tumour
250
What are risk factors for trigeminal neuralgia?
Increased age
| MS
251
What are the signs and symptoms of trigeminal neuralgia?
Sudden u/l stabbing pain in distribution of one or more of the branches of the trigeminal nerve
Recurrent
Lasts from a few seconds to a couple of mins
Periods of reission may vary
Pain is decribed a being shock-like (Face screws with pain)
252
What are the investigations for Trigeminal neuralgia?
Diagnosis is clinical
| MRI needed to exclude secondary diagnosis
253
What is Wernicke's encephalopathy?
Presence of Neurological symptoms caused by biochemical lesions of the CNS following exhaustion of Vitamin B reserves
254
What causes wernicke's encephalopathy?
```
Main cause is alcohol consumption which results in thiamine deficiency by causing:
- Inadequate nutritional thiamine intake
- Decreased thiamine absorption
- Impaired thiamine utilisation by cells
Other causes:
- Eating disorders
- Malnutrition
- Prolonged vomiting
- GI malignancy
- Chronic subdural haematoma
- AIDS
- Hyperemesis gavidarum
- Thyrotoxicosis
```
255
What is Hyperemesis gravidarum?
Severe nausea, vomiting, weight loss in pregnancy
256
What are the risk factors for Wernicke's encephalopathy?
```
Alcohol dependence
AIDS
Cancer treatment
Malnutrition/eating disorder
History of GI surgery
```
257
What are the symptoms of wernicke's encephalopathy?
Vision changes
Loss of muscle coordination
memory loss/change
Hallucinations
258
What are the signs of Wernicke's encephalopathy?
```
Triad
- Confusion
- Ophthalmoplegia
- Ataxia
Patients are mentally alert with vocabulary, comprehension, motor skills, social habits and naming ability maintained
You will see:
- Decreased reflexes/temperature
- Rapid pulse
Korsakoff's psychosis:
- Amnesia
- Confabulation (made-up stories fill any gaps in memory)
```
259
What investigations are done for wernicke's encephalopathy?
Clinical diagnosis
- Parenternal thiamine (improvement)
- Renal function
- FBC
- Electrolytes
- Glucose
- Ammonia
- LFTs
- Magnesium (Low)
