Neurology - part 1 Flashcards
What are two methods of investigation problems with the peripheral nervous system (2)?
- EMG
- Nerve conduction studies
What are two findings in a nerve conduction study?
- Decreased amplitude/ smaller waves = axon loss (e.g. MND, peripheral neuropathy)
- Delayed signal = demyelination (e.g. GBS)
What are two findings in electromyography?
- Myopathy = decreased action potential duration and amplitude
- Neuropathy = increased action potential duration and amplitude
What is a myopathy vs neuropathy?
- Myopathy = problem with the muscle (e.g. muscular dystrophy)
- Neuropathy = problem with the nerve (e.g. MND)
What are 3 ways of investigating problems with the CNS?
- EEG (electroencephalogram)
- MRI/ CT
- LP
What is NCCT first line for (3)?
- Stroke
- ICH
- Raised ICP
What is a contrast CT useful for?
Brain abscess
What are some differences in of PNS and CNS lesions in terms of presentation (2)?
- PNS = back pain?, bi-lateral,
- CNS = headache?, uni-lateral, visual changes
What is a lateralising sign?
Lesion affecting a single hemisphere (e.g. pronator drift, homonymous hemianopia, u/l weakness)
What are 3 important tracts to know about and where do they decussate?
- (DCML) dorsal column medial lemniscal (medulla)
- Spinothalamic (1-2 spinal levels above)
- Corticospinal (medulla)
What does the DCML carry (4)?
- Proprioception
- Fine touch
- Vibration
- 2 point discrimination
What does spinothalamic tract carry (3)?
- Lateral = pain + temperature
- Anterior = crude touch
What does the corticospinal tract carry (1)?
Motor signals to muscles
What nerve roots are responsible for the key reflexes?
- Bicep = C5/6
- Tricep = C7
- Knee = L4
- Ankle = S1
What nerve roots are responsible for the key dermatomes (6)?
- C6 = thumb
- C7 = middle + palm
- C8 = little
- T10 = bellybuT-TEN
- L5 = big toe (largest of the five)
- S1 = heel + sole
think of body doing splits with arms out
What are the nerve roots responsible for the key myotomes (5)?
- C5/6 = elbow flexion + shoulder abduction
- C7 = wrist flex, elbow extend
- C8 = hand flexion
- L5 = dorsiflexion
- S1 = plantar flexion
What are two common causes of foot drop?
- Common peroneal nerve lesion = MC
- L5 radiculopathy
How could you differentiate between common peroneal nerve lesion and L5 radiculopathy (2)?
- Common peroneal = ankle jerk absent, weak eversion
- L5 radiculopathy = ankle jerk present, weak inversion
What is a stroke?
A sudden infarct in the blood supply to part of the brain with symptoms lasting more than 24 hours
What are the two categories of stroke?
- Haemorrhagic (15%)
- Ischemic (85%)
What presentation is suggestive of a stroke?
Any sudden onset neurological symptom
What is ischemia vs infarction?
- Ischemia = inadequate blood supply
- Infarction = complete blockage of blood –> tissue death
What are the causes of a stroke (7)?
- Thrombus (+atherosclerosis)
- Embolus + infective endocarditis
- Shock
- Vasculitis
- Trauma
- Aneurysm
- Anticoagulants
What might cause a stroke in young people (6)?
- ADPKD (berry aneurysms)
- Congenital heart malformations
- COCP
- Blood clotting disorders
- Cocaine
- Infective endocarditis
Which congenital heart malformations increase the risk of stroke (3)?
- ASD
- VSD
- Patent foramen ovale
allow embolus from systemic circulation to enter the left ventricle and go to brain
What type of stroke affects the deep brain structures?
Lacunar stroke
What is a TIA?
A temporary neurological dysfunction lasting less than 24 hours caused by ischemia, but without infarction
What are some typical symptoms of a stroke (6)?
- Limb weakness
- Facial weakness
- Dysphasia
- Visual field defect
- Sensory loss
- Ataxia and vertigo
What are some risk factors for an ischemic stroke (13)?
- Smoking
- Hypertension
- AF
- Previous stoke/ TIA
- Diabetes
- Obesity
- Raised cholesterol
- Family history
- Vasculitis
- Thrombophilia
- COCP
- Migraines (with aura)
- Older age
What are the Bamford/ Oxford stroke classifications (4)?
- Total anterior circulation stroke
- Partial anterior circulation strokes
- Posterior circulation stroke
- Lacunar stroke
How does a total anterior circulation stroke present (3)?
- Unilateral sensory/ motor loss
- Homonymous hemianopia
- Higher cortical function loss e.g. dysphasia
Need all 3 for this classification
How does partial anterior circulation stroke present?
2 out of 3 of the criteria for a TACS
How does a posterior circulation stroke present (3)?
- Isolated vision change
- Cerebella Sx
- Contralateral CN palsy
How does a lacunar stroke present (3)?
- Pure motor stroke
- Pure sensory stroke
- Ataxic hemiparesis
What would be the presentation of an ACA stroke (2)?
- Lower limb»_space;» upper limb
- Behavioural change
sensory and motor deficit
What would be the presentation of an MCA stroke (4)?
- Upper limb»_space;» lower limb
- Face droop (forehead spared)
- Brocca/ wernicke aphasia (in dominant hemisphere)
- Ipsilateral gaze deviation
How does PCA stroke present (2)?
- Contralateral homonymous hemianopia (with macular sparing)
- Visual agnosia
What is webers stroke?
Branches of PCA that supply the midbrain blocked
What is the presentation of webners stroke (2)?
- Ipsilateral CN 3 palsy (down and out)
- Contralateral hemiplegia (upper + lower)
What is Wallenberg stroke?
PICA infarct
lateral medullary syndrome
What is the presentation of Wallenberg stroke (4)?
- Ipsilateral spinothalamic face loss (pain + temp)
- Contralateral spinothalamic limb/ torso loss
- Nystagmus
- Ataxia
What is a basilar artery stroke known as?
Locked in syndrome
carries blood to brain stem
How does a retinal artery stroke present?
Amaurosis fugax
transient loss of vision
How does a Benedikt stroke present?
Wallenberg + gait disturbance (red nucleus infarct)
How does an AICA stroke present?
Wallenberg + hearing loss + ipsilateral facial paralysis
no dysphasia
lateral pontine syndrome
What tool can be used to assess the likelihood of a stroke (2)?
- FAST (community)
- ROSIER tool (ED)
How is a patient presenting with a stroke managed (5)?
- BG testing (exclude hypoglycaemia)
- Immediate NCCT head
- Aspirin 300mg (for 2 weeks)
- Thrombolysis < 4.5 h
- Thrombectomy < 6h
- Consider diffusion weighted MRI
- Carotid doppler USS
How is a TIA managed (3)?
- Aspirin 300mg (daily)
- Referral for specialist assessment within 24 hours (or within 7 days if > 7 days since symptoms resolved)
- Diffusion weight MRI scanning
What are the specialist treatments offered to those with an ischemic stroke (2)?
- Thrombolysis (alteplase)
- Thrombectomy
How should blood pressure be managed in those with ischemic strokes?
Only treated in hypertensive emergency or to reduce risks associated with IV thrombolysis
reducing BP can reduce perfusion to brain
What are some complications of those with ischemic stroke (5)?
- Haemorrhagic transformation
- Cerebral oedema
- Seizures
- Aspiration pneumonia
- Long-term complications
What is haemorrhagic transformation in those with ischemic stroke?
Damaged tissue during ischemic stroke allows bleeding from the brain
What underlying causes of stroke are assessed in those who have had a TIA/ stroke (2)?
- AF (using ECG)
- Carotid artery stenosis (using USS)
How can carotid artery stenosis be managed (2)?
- Carotid endarterectomy (when > 70% stenosed)
- Angioplasty (balloon dilation) and stenting
When is thrombolysis offered to those with stroke?
4.5 hours within onset of symptoms (slightly different for those who have woken up with stroke)
What are some contraindications to thrombolysis (4)?
- Previous haemorrhagic stroke
- Recent major surgery
- Active internal bleeding
- Uncontrolled hypertension
When is thrombectomy offered for those with a stroke?
Usually within 6 hours, sometimes up to 24 hours if imaging is favourable
How are strokes/ TIAs managed long term (3)?
- Clopidogrel daily - P2Y12 inhibitor (or aspirin + dipyridamole)
- Atorvastatin
- BP + diabetes control
What are the 4 types of intracranial haemorrhage?
- Extradural haemorrhage
- Subdural haemorrhage
- Subarachnoid haemorrhage
- Intracerebral haemorrhage
Which intracranial haemorrhage constitute a stroke (2)?
- Subarachnoid haemorrhage
- Intracerebral haemorrhage
Where does a subdural haemorrhage bleed between?
Dura mater and arachnoid mater
Where does an extradural haemorrhage bleed between?
Skull and dura mater
What shape is a subdural haemorrhage on CT?
Banana shaped
What shape is a extradural haemorrhage on CT?
Lemon shaped
What percentage of strokes do intracerebral and subarachnoid haemorrhages account for?
10-20%
What are some risk factors for intracerebral haemorrhage (7)?
- Head injuries
- Hypertension
- Aneurysms
- Anticoagulants
- Bleeding disorders/ thrombocytopenia
- Brain tumours
- Alcoholism
What are the signs/ symptoms of intracerebral haemorrhage (5)?
- Headache
- Seizures
- Vomiting
- Reduced GCS
- Focal neurological symptoms
What values is the GCS between?
3-15
What are the scores allocated to eyes in GCS (4)?
- Spontaneous opening
- Speech
- Pain
- No response
What scores are allocated to verbal response in GCS (5)?
- Orientated
- Confused
- Inappropriate words
- Incomprehensible sounds
- No response
What scores are allocated to motor response in GCS (6)?
- Obeys command
- Localises pain
- Withdraws from pain
- Abnormal flexion to pain
- Extension to pain
- No response
What GCS score needs intubation?
8/15 or less
Damage to which blood vessel usually causes an extradural haemorrhage?
Middle meningeal artery
Damage to which region of the head often results in damage to the middle meningeal artery and therefore an extradural haemorrhage?
Temporal bone fracture
What age patient is usually affected by extradural haemorrhage?
Younger patients (20-30)
What is the typical presentation of a patient with extradural haemorrhage?
Head trauma and ongoing headache initially with symptoms improving then rapid worsening
Damage to which blood vessel usually causes an subdural haemorrhage?
Bridging veins
What age is typically affected by a subdural haemorrhage (2)?
- Older patients
- Babies (shaken baby)
What are some risk factors for subdural haemorrhage (3)?
- Head trauma
- Dementia
- Alcoholism
*both cause brain atrophy - causing arachnoid to pull away from dura
What time period do subdural haemorrhages usually come on over?
Longer time period than extradural as usually caused by bleeding from veins
How are subdural and epidural haemorrhages managed (3)?
- Correct clotting abnormalities, stabilise BP
- Craniotomy
- Burr holes
How does an intracerebral haemorrhage present?
Similar to an ischemic stroke but with a headache
What is the presentation of a patient with a SAH?
Sudden onset occipital headache ‘thunderclap headache’
What are some other symptoms of SAH other than headache (3)?
- Meningism (hhotophobia, neck stiffness, vomiting)
- Raised ICP Sx (CN3, 6 palsy, cushings triad)
- Neurological symptoms
What are some risk factors for SAH (12)?
- Hypertension
- Smoking
- Alcohol
- Cocaine
- Family history
- Sickle cell
- Connective tissue disorders
- ADPKD
- Neurofibromatosis
- Aged 45-70
- Women
- Black
How is a SAH investigated?
CT head = hyperlattenuation in SAS
most reliable within 6 hours
What can be performed if CT head is negative for SAH?
Lumbar puncture
How long after symptoms should a lumbar puncture be performed for a SAH?
More than 12 hours
What would a lumbar puncture show for those with SAH (2)?
- Xanthocromia
- Raised red cell count
How can the source of the bleeding in a SAH be located?
CT angiogram
How is a SAH managed (2)?
- Aneurysm coiling or clipping
- Neurosurgery
What are 2 complications of a SAH?
- Vasospasm
- Hydrocephalus
How is vasospasm treated in those with SAH?
Nimodipine (CCB)
How is hydrocephalus treated (in those with SAH) (2)?
- Therapeutic lumbar puncture
- Shunt (ventriculoperitoneal/ external)
How is raised intracranial pressure treated in those with intracranial haemorrhage?
Mannitol
What is the most common cause of a head injury in the UK?
-
RTA = mc
also alcohol + drugs
What criteria might suggest a patient with a head injury would need a scan (6)?
- Persistently reduced GCS (<15)
- Vomited on more than 1 occasion
- Seizure/ focal neurological deficit
- Battle sign (fluid from ear/ nose)
- > 65
- Dangerous mechanism of injury
A fracture in which part of the skull may cause CSF to leak from nose?
Basilar skull fracture (base of skull)
What are some complications of head injuries (4)?
- Epilepsy
- Mood disorders
- Personality changes
- Focal neurological deficits
What is dementia?
Progressive condition that causes irreversible impairment in memory, cognition, communication or personality
What are the main types of dementia (4)?
- Alzheimers (60%)
- Vascular dementia
- Lewy body dementia
- Fronto-temporal dementia
What term is often used to describe symptoms similar to dementia but not as severe?
Mild cognitive impairment
What age would be considered early onset for dementia?
< 65
What are some differential diagnoses for dementia (8)?
- Delirium
- Medication use
- Psychiatric conditions e.g. depression/ bereavement
- Parkinsons
- Brain tumours
- Hyper(para)thyroidism
- Cushings
- Nutritional deficiencies
What medications can cause symptoms of dementia in particular (3)?
- Anticholinergics (urological drugs e.g. Oxybutynin)
- Antihistamines
- TCA
What nutritional deficiencies may cause dementia like symptoms in particular (2)?
- B12 deficiency
- Thiamine (B1) deficiency - Wernicke-Korsakoff syndrome
What are some risk factors for developing dementia (5)?
- Sedentary lifestyle
- Lack of mental stimulation
- Obesity
- High BP
- High BG
What are 2 specific risk factors for the development of Alzheimers?
- Downs syndrome
- Family history
What are the general signs/ symptoms of dementias (4)?
- Forgetful (events/ names)
- Asking same questions
- Difficult remembering words
- Impaired decision making
What is the general progression of symptoms in dementia?
Slow and insidious worsening of symptoms and ability to function
What are the prominent features of Alzheimers?
- Agnosia - can’t recognise
- Apraxia
- Aphasia
- Dysphagia
What is the pattern of memory loss in Alzheimers?
Short term memory worse affected
What is a prominent feature of vascular dementia?
Stepwise decrease in functioning/ symptoms
symptoms vary depending on the area of the brain affected
What type of person is typically affected by vascular dementia?
Stereotypically unhealthy patient - overweight, smokes, drinks
What is a prominent feature of fronto-temporal dementia (2)?
- Frontal = thinking and memory
- Temporal = speech and language
What is a prominent feature of Lewy body dementia (2)?
- Parkinsonism
- Hallucinations
What is the pathophysiology of Alzheimers dementia?
Beta amyloid proteins accumulate as plaques and tau neurofibrillary triangles –> death of brain cells
How is Alzheimers managed with medication (2)?
- Cholinesterase inhibitors
- Memantine
What are 3 examples of cholinesterase inhibitors used for Alzheimers?
- Galantamine
- Donepezil
- Rivastigmine
What causes Lewy body dementia?
Lewy body accumulation in the cortex
What is the main constituent of Lewy bodies?
Alpha synuclein
What is Lewy body dementia an umbrella term for?
- Dementia with Lewy bodies (memory affected first)
- Parkinsons disease dementia (movement affected first)
What is the difference between Parkinson’s disease and dementia with Lewy bodies?
Likely due to the location of accumulation of Lewy bodies
considered a spectrum of disorders
The collection of which protein leads to fronto-temporal dementia?
Tau protein/ pick body
What sometimes causes frontotemporal dementia?
Autosomal dominant mutation in tau protein
What is frontotemporal dementia associated with?
ALS (15-20% of those with ALS develop FTD)
How are those with dementia initially managed in primary care (2)?
- Exclude physical cause (bloods, CXR MSU)
- Referral to memory clinic
How are those with dementia investigated in secondary care (2)?
- ACE (addenbrookes cognitive examination)
- MRI
What would an MRI show in those with dementia (2)?
- Atrophy of brain
- Lack of structural pathology
How are those with dementia managed (5)?
- Lasting power of attorney
- Advanced decisions
- Antidepressants
- Antipsychotics (risperidone)
- Benzos (for crisis management)
What are some associated behavioural and psychological symptoms of dementia (6)?
- Depression
- Anxiety
- Agressions
- Agitation
- Hallucinations
- Sleep disturbances
What is delirium?
Acute fluctuating disturbance in mental function
What are the signs/ symptoms of delirium (6)?
- Agitation
- Confusion
- Drowsiness
- Hallucinations
- Delusions
- Memory problems
What are the causes of delirium?
- Pain
- Infection
- Nutrition
- Constipation
- Hydration status
- Medications
- Environvment/ electrolytes
What medications commonly cause delirium (5)?
- Benzos
- Opioids
- Anticholinergics
- Dopaminergics
- Steroids
What might make a diagnosis of delirium more likely in terms of presentation (3)?
- Hallucinations (esp. visual)
- Altered consciousness (hyperalert/ drowsiness)
- Agitation more common (looking round room, fidgety)
What is the second most common neurodegenerative condition after dementia?
Parkinsons disease
What is the underlying cause of parkisnons disease?
Loss of dopamine producing neurones in the substantial nigra pars compacta
What protein accumulation causes the death of these neurones in PD?
Alpha synuclein aggregating to form Lewy bodies
What is the mechanism by which parkinsons makes it more difficult to initiate movement?
Decrease in nigrostriatal pathway neurones –> less GABA-ergic inhibition (via indirect pathway) –> gross cortex inhibition –> more difficult to initiate movement
What are some risk factors for PD (3)?
- Family history
- Male (2:1)
- Older age
What is protective against PD?
Smoking
What medications can cause parkinonism or exacerbate parkinsons (2)?
- Antipsychotics (especially typical)
- Metoclopramide (antiemetic)
What is a feature of drug induced parkinsonism?
Bilateral resting tremor
What are the 4 typical symptoms of parkinsons disease?
- Bradykinesia
- Resting tremor
- Rigidity
+ Postural instability (not part of the triad, but a key symptom
What is the typical feature/ presentation of symptoms in PD?
Asymmetrical symptoms with one side affected much more than the other
What are some symptoms of PD when walking (3)?
- Stooped posture
- Reduced arm swing
- Shuffling gait
What are two types of rigidity found in parkinsons disease?
- Cogwheel = jerky
- Lead pipe = smooth tremor throughout
What are some other features of PD other than the 4 main features (7)?
- Depression
- Sleep disturbance (REM sleep disorders)
- Postural instability
- Loss of sense of smell
- Cognitive impairment/ memory problems
- Constipation
- Autonomic symptoms (e.g. postural hypotension)
What are the features of a tremor in parkinsons disease (3)?
- 4-6 hertz (pill rolling)
- Asymmetrical (worse on one side)
- Resting
What is a key differential to the tremor seen in PD?
Benign essential tremor
What are the features of benign essential tremor when compared to PD?
- Higher frequency 6-12 Hz
- Intention tremor
- Symmetrical
- Improves with alcohol
How is PD diagnosed?
Clinically by a specialist
How is more mild PD treated (3)?
- Da agonist
- Monoamine oxidase - B inhibitors
- Levodopa
What is an example of a dopamine agonist used for PD?
Bromocriptine
What is an example of a MAOB-i used for parkinsons?
Selegiline
How do MAOB-i work?
Prevent the breakdown of dopamine
What is a complication of long term use of dopamine agonists?
Pulmonary fibrosis
What medication is used in more severe PD?
Levodopa
What medications are levodopa combined with to prevent its breakdown/ metabolism (2)?
- COMT inhibitors
- Peripheral decarboxylase inhibitors - always combined with this
What is an example of a COMT inhibitor?
Entacapone
What is an example of a peripheral decarboxylase inhibitor?
Carbidopa
What treatments are offered to those with PD where initial medications are ineffective (2)?
- Deep brain stimulation
- Apomorphine (dopamine agonist) sub cut infusion
What is a side effect of levodopa (4)?
- Postural hypotension
- Confusion
- Psychosis
- Dyskinesia/ chorea
What can be given for those with PD who develop dyskinaesia due to levodopa?
Amantadine (glutamate antagonist)
What is a phenomena seen in those who take levodopa for a long time?
“On-off phenomena” motor fluctuations throughout the day
Overall what is the typical treatment of parkinsons (3)?
- Levodopa + decarboxylase inhibitor (co-careldopa)
- COMT inhibitors, DA agonists
- DBS
What two conditions are similar to PD but important differentials especially in those not responding to treatments?
- Progressive supranuclear palsy (PSP)
- Corticobasal degeneration
How is essential tremor treated (5)?
- Propanolol = 1st
- Primidone
- Gabapentin
- Botox injections
- Beep brain stimulation
What are 3 primary causes of headaches?
- Migraine
- Tension headache
- Cluster headache
What are some other (secondary) causes of headache (8)?
- Meningitis/ infection
- Sinusitis
- Giant cell arteritis
- Intracranial haemorrhage
- Medication overuse headache
- Glaucoma
- Pre-eclampsia
- Raised ICP
- Trigeminal neuralgia
What is the most common type of primary headache disorder?
Tension headache
What type of pain is felt in tension headaches?
Bilateral tight band around sides of head - temples, occipital region and neck
What are other associated symptoms with a tension headache?
There are not really any
no N+V, motion sickness or visual changes
What are some triggers for tension headaches (5)?
- Stress
- Alcohol
- Dehydration
- Stagnant head/ neck position
- Lack of sleep
How can the symptoms of tension headaches be treated medically?
Analgesics - paracetamol, Ibuprofen
What can be used to treat for chronic/ frequent tension headaches?
Amitriptyline
What is the most common type of recurrent primary headache disorder?
Migraine
Which gender is most commonly affected by migraines?
Females
What age do migraines typically begin?
< 40 year olds
most common in young adults/ adolescents
What are the types of migraine (4)?
- Migraine with aura
- Migraine without aura
- Silent migraine
- Hemiplegic migraine
What is a silent migraine?
Migraine that does not cause a headache only an aura
What are the 5 stages of a migraine?
- Prodrome
- Aura
- Headache
- Resolution
- Postdrome
What does a prodrome refer to in the development of a migraine?
Change in mood/ fatigue up to days before an attack
What does an aura refer to in the development of a migraine?
Neurological changes in the minutes before an attack
What are the most common areas an aura may affect in the development of migraines (4)?
- Visual = MC
- Sensory (parasthaesia)
- Speech (dysphasia)
- Motor (weakness)
What are some common visual changes associated with migraines with aura (3)?
- Sparks in vision
- Blurred vision
- ‘QR code’ vision
What is a migraine that causes motor symptoms known as?
Hemiplegic migraine
What are the symptoms of a hemiplegic migraine (3)?
- Unilateral limb weakness
- Ataxia
- Reduced consciousness
What is an important condition to rule out in those with migraines and associated symptoms?
Stroke/ TIA
What can sometimes cause the abrupt end to symptoms of migraines (2)?
- Vomiting
- Sleeping
What is the types of pain experienced in a migraine?
Unilateral moderate - severe throbbing headache
may come on quickly or gradually
What are some associated symptoms with migraines (other than headache) (5)?
- Nausea/ vomiting
- Photo/phono/osmo phobia
- Aura
- Motion sickness
- Facial weekness
What are some triggers for migraines (8)?
- Stress
- Over/under sleeping
- Caffeine
- Chocolate
- Alcohol
- Dehydration
- Menstruation
- Exercise/ orgasm
How are migraines managed acutely first line?
Triptans (e.g. sumatriptan) + NSAID/ paracetamol
What is the mechanism of triptans?
Serotonin receptor agonist
various effects including cranial vasoconstriction
What are some contraindications for triptans (3)?
- Hypertension
- CAD
- Stroke
things vasoconstriction would negatively affect
How are migraines treated prophylactically (2)?
- 1st = Propanolol
- 2nd = TCAs e.g. amitriptyline
What is given as migraine prophylaxis first line in asthmatics?
Topiramate
What is important to check in females diagnosed with migraines?
Take them off oral contraceptive pill
What type of pain is felt in those with cluster headaches?
Severe unilateral pain, usually around the eye
What is the pattern of attacks seen in cluster headaches?
Periods of pain 15min - 3hours, 3-4 times per day, for 4-12 weeks, followed by a period of pain free lasting weeks, months or years
What are some risk factors for cluster headaches (4)?
- Male
- 30-50
- Genetics/ family history
- Smoking
What are some other features of cluster headaches other than pain (4)?
- Ptosis
- Red, swollen watery eye
- Rinorrhoea
- Miosis
How are cluster headaches treated acutely (2)?
- SC triptans (sumatriptan)
- High flow oxygen 100%
How are cluster headaches treated with prophylaxis?
Verapamil (CCB)
What is the presentation of a patient with trigeminal neuralgia?
Shooting/ stabbing/ electric shock pain usually on ONE side of the face
Where can trigeminal neuralgia affect?
Any combination of the trigeminal nerve branches:
* V1 ophthalmic
* V2 maxillary
* V3 mandibular
What condition is trigeminal neuralgia associated with?
Multiple sclerosis
What are some other risk factors for trigeminal neuralgia other than multiple sclerosis (2)?
- Female
- Older age
What is the treatment for trigeminal neuralgia?
- 1st = Carbamazepine
- Surgical intervention
What is the most common secondary headache?
Medication overuse headache
How many days per months should analgesia be taken for a headache to be termed a medication overuse headache?
15/ 30 days
10/30 for opiates
What often precedes sinusitis?
Viral infection
Where is pain felt in sinusitis?
Areas above the eyebrows and below the eyes
When might sinusitis need to be treated?
After 10 days
How is sinusitis treated (2)?
- Steroid nasal spray
- Penicillin V (phenoxymethylpenicillin)
What is giant cell arteritis?
Large cell vasculitis mainly affecting temporal artery
What are some risk factors for GCA (3)?
- Female
- > 50 years
- Caucasian
What are the signs/ symptoms of GCA (3)?
- Jaw claudication
- Temporal tenderness
- Amaurosis fugax
How is GCA investigated (2)?
- Bloods = raised ESR
- Temporal artery biopsy = granulomas + skip lesions
How is GCA treated (2)?
- IV methylprednisolone (if vision loss)
- PO prednisolone (if no vision loss)
What is an epileptic seizure?
Transient episodes of abnormal electrical activity in the brain
What is another type of seizure (not epileptic)?
Non-epileptic seizure (functional seizure)
What are some causes of non-epileptic seizures (5)?
- Psychological
- Syncope
- Hypoglycaemia
- Arrhythmias
- Functional neurological disorder
What are two ways to differentiate non epileptic seizures from epileptic seizures?
- Epileptic = eyes open
- Non-epileptic = injuries less common
What defines epilepsy (in terms of when the seizures happen)?
2 or more unprovoked seizures that occur more than 24 hours apart
What are some causes of seizures (8)?
- Epilepsy (idiopathic)
- Vascular
- Infections
- Fever
- Metabolic/ electrolyte disturbances
- Trauma
- Eclampsia
- Autoimmune disease e.g. SLE
What are some conditions associated with epilepsy (5)?
- Brain tumours
- NFM1
- Dementia
- Cerebral palsy
- History of trauma/ infection
What are the features of an epileptic seizure (3)?
- Eyes open
- Synchronous movements
- Can happen at night
What is thought may be a cause of epilepsy?
Imbalances between the inhibitory GABA and excitatory glutamate neurotransmitters
How long does an epileptic seizure typically last?
< 2 minutes
What can lower the seizure threshold in those with epilepsy (4)?
- Hypoglycaemia
- Alcohol
- Stress + lack of sleep
- Medications (e.g. ciprofloxacin, lithium, TCAs)
What are the phases of epileptic seizures (4)?
- Prodrome
- Aura
- Ictal event
- Post-ictal period
What are symptoms of a prodrome before a seizure (3)?
- Changes in mood
- Changes in sensation
- Changes in behaviour
What are the symptoms of an aura associated with a seizure (5)?
- Change in smell/ taste
- Lip smacking
- Visual changes
- Sensory disturbances
- Deja vu
- Sudden emotional changes
the beginning of the seizure - a focal seizure that then spreads to become a generalised seizure
How long does a prodrome last before a seizure?
Days before the seizure
How long does an aura last before a seizure?
Minutes/ seconds
What are the symptoms associated with the post-ictal period after a seizure (4)?
- Confusion
- Drowsiness
- Headache/ body ache
- Emotional changes
How long does the post-ictal period last?
Minutes to hours
How are epileptic seizures classified depending on the part of the brain they affect (2)?
- Generalised - bilateral, often lose consciousness
- Focal - features confined to one region
What are the main types of generalised seizures?
- Tonic clonic
- Absence
- Myoclonic
- Tonic
- Atonic
- Clonic
What is the presentation of a patient with a tonic clonic seizure?
Loss of consciousness, muscles tense –> repeated jerking of muscles
What other symptoms may the patient have during a tonic clonic seizure (4)?
- Tongue biting
- Incontinence
- Groaning
- Irregular breathing
What is the presentation of a patient with an absence seizure?
Period of loss of consciousness and awareness for 10-20 seconds
Who is most commonly affected by absence seizures?
Children, most adults have grown out of them
What is the presentation of a myoclonic seizure?
Sudden brief muscle jerks affecting any part of the body, usually remain conscious
What is the presentation of atonic seizures?
Sudden brief loss of muscle tone, consciousness maintained
What is the presentation of tonic seizures?
Sudden stiffening of muscles, resulting in a fall if standing (around 20 seconds) - sometimes lose consciousness
What is the presentation of a clonic seizure?
Repeated rhythmic muscle jerking in the upper body/ face (last a few minutes) - sometimes lose consciousness
What is a feature of absence seizures on an EEG?
3 Hz spike
What are the two types of focal seizures?
- Simple
- Complex
What is the difference between a simple and complex focal seizure?
- Simple = remain aware
- Complex = lose awareness
What are the symptoms of a focal seizure in each hemisphere of the brain?
- Temporal = repeated movements + dysphasia
- Frontal = repeated muscle twitching/ paralysis
- Parietal = paraesthesia
- Occipital = visual changes
What two neurological phenomena sometimes occur with frontal lobe seizures?
- Todds paralysis
- Jacksonian marching (twitching along the motor homunculus)
What extra brain structure is often involved in complex focal seizures?
Basal ganglia
What are some differentials for epileptic-seizures (6)?
- Vasovagal syncope (fainting)
- Hemiplegic migraine
- Psychogenic non epileptic seizures
- Arrhythmias
- Hypoglycaemia
- TIA/ stroke
How is epilepsy investigated (5)?
- EEG
- MRI/ CT
- ECG
- Serum electrolyte
- BG
What blood marker is commonly raised in those with epileptic seizures?
Prolactin
How are generalised seizures treated (2)?
- Sodium valproate (in all except women who may become pregnant)
- Lamotrigine/ levetiracetam (for women of childbearing age)
How are focal seizures treated?
- 1st = Lamotrigine or levetiracetam
- 2nd = carbamazepine
What medication is first line in those with absence seizures?
Ethosuximide
What medication is first line for myoclonic seizures (2)?
- Male = sodium valproate
- Female = levetiracetam
What medication is first line for tonic or atonic (2)?
- Male = sodium valproate
- Female = lamotrigine
What are some other important steps to take in the management of people with epilepsy (2)?
- Cannot drive until seizure free for 1 year (must tell DVLA)
- Advise not to swim/ operate heavy machinery
In what way is sodium valproate teratogenic?
Increases likelihood of neural tube defects
What is a side effect of lamotrigine?
Stephen Johnson syndrome
What is a side effect of ethosuxamide?
Agranulocytosis (like clozapine)
What AED has lots of different side effects and isn’t used very much any more?
Phenytoin
What is important to tell women on AEDs?
Lots of them interact with hormonal contraceptives
the same goes with some other medications
What is a severe complication of those who have seizures?
Status epilepticus
What is status epilepticus?
- Seizure lasting more than 5 minutes
or - Multiple seizures without regaining consciousness
How is status epilepticus treated (5)?
- ABCDE management
- IV lorazepam/ other benzo
- IV lorazepam (after 5 min)
- IV levetiracetam/ phenytoin/ valproate
- Phenobarbital/ general anaesthesia
What is multiple sclerosis?
Autoimmune condition causing demyelination in the CNS
What type hypersensitivity is MS?
Type 4
What cell is attacked in MS?
Oligodendrocytes
Who is typically affected by MS (2)?
- Younger adults especially
- Women (<40)
What phase is often used to describe MS and can be useful in radiological and clinical diagnosis?
Disseminated in space and time
lots of parts of the body affected at different points in time
What are some causes/ risk factors/ precipitating factors of MS (7)?
- Smoking
- EBV/ other infections
- Obesity
- Genetics
- Vitamin D deficiency
- Other autoimmune diseases
- Female
How quickly do symptoms of MS come on at initial presentation?
Over 24 hours
How long do symptoms last at initial MS presentation?
Days to weeks, then improve
What is the most common presentation of MS?
Optic neuritis
What are the symptoms/ signs of optic neuritis (4)?
- Unilateral reduced vision
- Pain (with eye movement)
- Loss of colour vision (especially red)
- Relative afferent pupillary defect
What is a relative afferent pupillary defect?
Pupil of affected eye constricts more when shining light in the contralateral eye than the affected eye
How should optic neuritis be managed?
- Urgent ophthalmology review
- High dose steroids
What are some other symptoms of MS other than optic neuritis (5)?
- Weakness (incontinence, limb weakness, Horners syndrome)
- Sensory symptoms (paraesthesia, trigeminal neuralgia)
- Eye movement abnormalities
- Ataxia
- Hyper-reflexia
- Intention tremor
What is a specific sensory sign seen in MS when flexing the neck?
Lhermittes sign
What other sensory sign is found in MS amongst other neurological conditions?
Transverse myelitis - site of inflammation in the spinal cord
What eye movement disorder sometimes present in those with MS (2)?
- Intranuclear ophthalmoplegia
- Horizontal lateral gaze disorder
What causes intranuclear ophthalmoplegia (INO)?
Lesion in the medial longitudinal fasciculus
What is the clinical feature of INO?
Impaired adduction on the same side of the lesion and nystagmus in the contralateral abducting eye
What is the clinical feature of horizontal lateral gaze disorder?
When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct
What is the group of 3 signs/ symptoms found in MS known as?
Charcots neurological triad
What is in charcots neurological triad?
- Nystagmus
- Dysarthria
- Intention tremor
What are two types of ataxia that may occur in MS?
- Sensory ataxia = due to loss of proprioception (causes +ve Rombergs test)
- Cerebellar ataxia
What are some other causes of optic neuritis other than MS (4)?
- SLE
- Sarcoidosis
- Infections e.g. syphilis, Lyme disease
- Neuromyelitis optica
What are the 4 patterns of disease in those with MS?
- Clinically isolated syndrome
- Relapsing-remitting
- Primary progressive (never has remission)
- Secondary progressive (started as relapsing-remitting
How is MS investigated (3)?
- MRI
- Lumbar puncture
- NCS = decreased velocity (due to demyelination)
What would be suggestive of MS on a lumbar puncture?
Oligoclonal bands
What criteria are sometimes helpful in the diagnosis of MS?
Mc Donald criteria
How are relapses of MS treated (2)?
- Methylprednisolone
- Plasmapheresis + IV Ig (if severe)
What sometimes precipitates a flare of MS?
Infections!!
How is MS managed in the long term?
Disease modifying drugs
What are some disease modifying drugs used to treat MS (3)?
- Natalizumab
- Glatiramer
- Beta interferon
What is a complication of some of the disease modifying therapies used in MS?
Progressive multifocal leukoencephalopathy (reactivation of the JC virus - living in glial cells)
What are some differential diagnoses for MS?
- Migraine with aura
- Stroke
- Hypoglycaemia/ hypothyroidism
- Infections
- Brain tumour
- Neuromyelitis optica
What is neuromyelitis optica?
Sister disease to MS with lots of similar symptoms but WORSE
only affects eyes and cord (not brain)
What are 3 anterior horn diseases?
- MND = mc
- Poliomyelitis
- Spinal muscular atrophy
- Anterior spinal artery infarct
What is the anterior horn of the spinal cord?
The place where motor information exits the spinal cord
What are the symptoms of anterior horn diseases?
Purely motor symptoms
What is usually the cause of an anterior spinal artery infarct (2)?
- AAA repair
- AD repair
What are the signs/ symptoms of an anterior spinal artery infarct (2)?
- Bilateral paralysis (below lesion)
- Bilateral loss of pain + temp
How is polio spread?
Faeco-oral spread
VERY contagious
What is a complication of polio?
Attacks anterior horns –> lower motor neurone signs + paralysis
What is spinal muscular atrophy?
Auto recessive condition causing floppiness that can present at various ages (including in newborns) with progressing muscle weakness
What is motor neurone disease?
A neurodegenerative disorder that selectively targets motor neurones leading to their degeneration through unknown mechanisms
What are some risk factors for motor neurone disease (4)?
- Genetics/ family history
- Exposure to toxic, viral, heavy metals
- Smoking thought to increase risk
- Male
What is the typical patient affected by MND?
60 y/o male
What gene mutation has been associated with MND?
SOD-1 gene mutation
What condition is MND linked to?
Frontotemporal dementia
What is the types of MND (4)?
- Amytrophic lateral sclerosis
- Progressive bulbar palsy
- Progressive muscular atrophy
- Primary lateral sclerosis
Which neurones are affected in ALS?
Upper and lower motor neurones
What are the signs/ symptoms of ALS (4)?
- Muscle weakness + waisting
- Muscle fasciculations
- Dysarthria/phagia
- Generalised fatigue
What might a patient complain of with ALS to a GP in the initial stages (3)?
- More clumsy
- Dropping things
- Tripping over
What are the two initial presentations found in those with ALS?
- Bulbar onset symptoms
- Limb onset symptoms
What is the prognosis for those diagnosed with ALS?
Poor typically 3-5 year survival
What nerves are affected in progressive bulbar palsy?
Lower motor neurones of cranial nerves 9, 10, 12
What are the signs/ symptoms of PBP (3)?
- Dysphagia
- Dysarthria
- Tongue atrophy
worst prognosis
What nerves are affected in progressive muscular atrophy?
Lower motor neurones
What are the signs/ symptoms of progressive muscular atrophy (3)?
- Muscle weakness
- Muscle atrophy
- Fasciculations
What nerves are affected in primary lateral sclerosis?
Upper motor neurones
What are the signs/ symptoms of primary lateral sclerosis (2)?
- Spasticity
- Weakness
How can progressive muscular atrophy be distinguished from ALS and PLS?
Absence of:
* Brisk reflexes
* Spasticity
* Babinski
How can primary lateral sclerosis be distinguished from ALS and PMA?
No muscle atrophy
How is MND diagnosed?
Clinically after excluding other conditions
EMG (can be abnormal), nerve conduction and MRI can help
How is MND managed (2)?
- Riluzole (can slow progression)
- Supportive care
How is spasticity in MND treated?
Baclofen
How is drooling in MND treated?
Hyoscine
What type of ventilation is often required in MND?
BiPAP
What is usually the most suitable way to feed people with MND?
Percutaneous endoscopic gastroscopy tube (PEG)
What symptoms are never found in those with MND (3)?
- Sensory Sx
- Eye Sx
- Sphincters never affected
What are the general signs/ symptoms of UMN lesions (3)?
- Increased reflexes
- Hypertonia / increased spasticity
- Babinski sign +ve (toes upward)
What are the general signs/ symptoms of LMN lesions (5)?
- Atrophy
- Fasciculations
- Reduced tone
- Reduced reflexes
- -ve Babinski
What is Huntington’s chorea?
Progressive neurodegenerative genetic disorder characterised by gradual onset of involuntary movements
What is the inheritance pattern of Huntington’s chorea?
Autosomal dominant
What gene and chromosome are affected in Huntington’s?
HTT (huntingtin) gene on chromosome 4
What is the dysfunction of the DNA in huntingtons chorea?
Trinucleotide repeat disorder of CAG
How many repeats of CAG are required for people to develop huntingtons?
36 or more
What changes in the development of huntingtons disease are seen from generation to generation?
Anticipation - symptoms present earlier and more severely
What are the signs/ symptoms of huntingtons chorea (6)?
- Chorea
- **Dementia **
- Psychiatric issues/ depression
- Dystonia - abnormal muscle tone –> abnormal postures
- Dysphagia/arthria
- Rigidity
- Eye movement disorders
What is usually the first symptoms of those with huntingtons?
Cognitive decline/ depression
What age does huntingtons usually present?
30 - 50
How is huntingtons investigated?
- Clinical suspicion e.g. symptoms + family history
- Genetic testing = diagnostic
How is huntingtons chorea managed (2)?
Symptomatic relief:
* Tetrabenazine for chorea
* Valproic acid for muscle twitching
What are the two most common causes of death in huntingtons?
- Resp arrest
- Suicide
What are two neuromuscular junction disorders?
- Myasthenia gravis
- Lambert eaton myasthenia disorder
What is myasthenia gravis?
Autoimmune condition attacking the neuromuscular junction
What is the pathophysiology of MG?
ACh receptor antibodies bind to postsynaptic ACh receptors, blocking them and preventing stimulation by ACh
As well as AChR antibodies what two other antibodies sometimes cause MG?
- Muscle specific kinase (MuSK)
- Low-density lipoprotein receptor-related protein 4 (LRP4)
What is another mechanism by which ACh receptor antibodies cause symptoms?
They activate the compliment system leading to cell damage at postsynaptic membrane
What are MuSK and LRP4 proteins important for the creation and organisation of?
Acetylcoline receptors
What is the key feature of those with MG?
Fatiguability - weakness that improve with rest/ worsens with activity
Why does MG improve with rest?
AChR are cleared from the postsynaptic ACh receptors when resting
What two groups of people are most commonly affected by MG?
- Men over 60 (with thyomas)
- Women under 40 (autoimmune)
Which two muscle groups are most commonly affected in MG?
- Small muscles of the head and neck
- Proximal muscles of the limbs
What are the signs/ symptoms of MG (5)?
- Ptosis
- Diplopia
- Dysphagia/arthria
- Weakness in limbs e.g. tripping over
- Fatigue in jaw when chewing
What condition is there a link to with MG?
Thymomas (30% develop MG)
How can you exacerbate symptoms of MG on a physical examination (3)?
- Repeated blinking exacerbates ptosis
- Prolonged upward gazing exacerbates diplopia
- Repeated abduction of one arm will result in unilateral weakness
How is MG investigated (3)?
- Antibodies tests (85% have AChR, 10 MuSK, 5 LRP4)
- CT/ MRI thymus (to look for thymoma)
- Edrophonium test if any doubt
How does edrophonium test work in MG?
Edrophonium blocks cholinesterase –> more ACh available at postsynaptic receptor –> weakness of muscle improves
How is MG managed (4)?
- Pyridostigmine (cholinesterase inhibitor)
- Immunosurpression (e.g. azathioprine, prednisolone)
- Thymectomy - can improve symptoms even if no thymoma
- Rituximab
What is a serious complication of MG?
Myasthenia crisis
How can severity of myasthenic crisis be monitored?
Forced vital capacity (easy test involves counting numbers in 1 breath)
How is myasthenic crisis managed (3)?
- NIV/ mechanical ventilation
- IVIg (against AChR)
- Plasmapheresis
How long does MG last?
It is lifelong
What is Lambert eaton myasthenic syndrome?
Autoimmune condition affecting neuromuscular junction
What is the pathophysiology of Lambert eaton syndrome?
Antibodies are formed against voltage gated calcium channels and destroy them –> less ACh released into the synapse
What is the importance of voltage gated calcium channels in the NMJ?
They assist in the release of ACh
What condition is usually associated with cases of Lambert eaton myasthenic syndrome?
SCLC (paraneoplastic syndrome)
can also occur as a primary autoimmune disorder
What are the signs/ symptoms of Lambert eaton myasthenic syndrome?
- Proximal muscle weakness e.g. difficulty climbing stairs
- Autonomic dysfunction e.g. dry mouth, impotence
- Reduced/ absent reflexes
What is a key feature of Lambert eaton myasthenia syndrome?
Muscle strength improves after use
How is Lambert eaton myasthenic syndrome investigated (3)?
- Excluding SCLC (CT)
- Nerve conduction studies/ EMG
- Blood tests for voltage gated calcium antibodies
How is Lambert eaton myasthenic syndrome managed (3)?
- Diaminopyridine (amifampridine)
- Immunosurpression
- IVIg/ plamapheresis
How does diaminopyradine work?
Blocks voltage gated potassium channels in presynaptic membrane –> prolongs depolarisation –> assists calcium channels in carrying out their function