Neurology Flashcards
What are the clinical features of Multiple System Atrophy?
MSA characteristically presents with:
- Parkinsonism
- atuonomic disturbance (atonic bladder, postural hypotension, erectile dysfunction)
- Cerebellar Signs (ataxia, tremor, dysarthria)
What are the Parkinson’s Plus Syndromes and their main features?
Parkinson-plus syndromes (aka. atypical parkinsonism) are a group of neurodegenerative diseasese that present with parkinsonism and a vareity of additional features.
Multiple System Atrophy:
- Atuonomic dysfunction with urogenital problems
- Lewy bodies
Progressive Supranucelar Palsy:
- Vertical gaze palsy
- Frontal lobe disturbances
Corticobasal degeneration:
- Alien limb phenomenon
- Asymmetric motor symptoms
Dementia with Lewy Bodies:
- Visual hallucinations
- Lewy bodies
What are the diagnostic criteria for migraine without aura?
A: At least 5 attack fulfulling B-D
B: Headaches lasting 4-72 hours
C: Headache has at least 2 of the following characteristics:
- Unilateral
- Pulsating quality
- Moderate or severe pain intensity
- Aggrevation by, or causing avoidance of routine physical activity
D: During headache at least 1 of the following:
- Nausea ± vomiting
- Phtophobia and phonophobia
E: Not attributed to another disorder.
What % of migraines present with auras
Around 25% of migraines have an aura. These tend to be easier to diagnose than those without aura.
NICE details on auras:
- Fully reversible
- Develop over at least 5 minutes
- last 5-60 minutes
What type of drug is ondansetron?
What is a common side effect of this drug?
Ondansetron is an antiemetic that acts as a 5-HT3 antagonist.
Common side effects include constipation.
What type of drug is metoclopramide?
What are common side effects?
Metoclopramide is an antiemetic that is a dopamine antagonist.
Side effects include:
- Parkinsonism/movement disorders
- Diarrhoea
- Hypotension
- Asthenia (abnormal physical weakness)
What type of drug is domperidone?
What are common side effects?
Domperidone is an antiemetic that is a dopamine antagonist.
Side effects include:
- Asthenia (physical weakness)
- lactation disorders
- Loss of libido
- Diarrhoea
What are common sequalae of bacterial meningitis?
- Deafness (most common)
- Other neurological: epilepsy, paralysis
- Infectious: Sepsis, intracerebral abscess
- Pressure: brain herniation, hydrocephalus
How must patients abstain from driving following a first TIA?
How long if multiple TIAs?
Current guidelines state that patients must not drive for 1 month following a TIA. (no need to inform DVLA)
If there are multiple TIAs in a short period of time: 3 months.
The driver is legally responsible for infroming the DVLA about this; if they drive nonetheless, the doctor should contact the DVLA and inform the patient about such action.
How must patients abstain from driving following a first unprovoked seizure?
How long after a diagnosis of epilepsy?
6 months if there are no relevant structural abnormalities on brain imaging, and no definite epileptiform activity on EEG. If these conditions are not met, then it increases to 12 months.
If diagnosed with epilepsy, the person needs to be seizure free for 12 months.
Once withdrawing from epilepsy medication the patient should stop driving, and continue this 6 months after last dose.
How must patients abstain from driving following an episode of syncope?
Simple faint: no restriction
Singe episode, explained and treated: 4 weeks off
Single episode, unexplained: 6 months off.
≥ episodes: 12 months off.
How does migraine in children differ to adults?
In children, attacks may be shorter lasting, headache is more commonly bilateral, and GI disturbances such as nausea and vomiting are more prominent.
What is the commonest cause of bitemporal hemianopia that:
a) predominantly affects the lower quadrants
b) predominantly affects the upper quadrants
Upper quadrant defect predominant: inferior chiasmal compression. Commonly a pituitary tumour.
Lower quadrant defect predominant: superior chiasmal compressions. Commonly a craniopharyngioma.
What are common side effects of sodium valproate?
- P450 inhibitor
- GI: nausea
- Increased appetitite and weight gain
- Alopecia (regorwth may be curley)
- Hepatotoxicity
- Teratogenicity
- Tremor
- Thrombocytopenia
- Hyponatraemia
- Hyperammonemic encepalopathy
Describe the MRC scale of muscle power used in neurological examinations.
How can you divide peripheral neuropathies?
Give examples for each category.
Peripheral neuropathies can be subdivided into those with predominant motor loss, and those with predominantly sensory loss.
Motor loss:
- Guillain-Barré
- Charcot-Marie-Tooth (Hereditary sensorimotor neuropathies)
- Chronic inflammatory demyelinating polyneuropahty (bit like long-lasting GBS)
- Porphyria, lead poisoning, diphtheria
Sensory loss:
- Diabetes
- Alcoholism (both directly due to toxic effects and due to viatmin deficiency)
- B12 deficiency (subacute combined degeneration of spinal cord. Dorsal column usually affected first (proprioception/vibration) prior to distal paraesthesia.
- Leprosy
- Uraemia, Amyloidosis
Define neuropathic pain.
Neuropathic pain may be defined as pain which arises following damage or disruption of the nervous system.
Summarise the treatment algorithm for the management of neuropathic pain.
For specific conditions management may vary.
1st line one of:
- Amitryptiline
- Duloxetine
- Gabapentin or pregabalin
If 1st line doesnt work, try one of the other three.
Tramodol may be used as rescue therapy for exacerbations of neruopathic pain.
Topical capsaicin may be used for localised neuropathic pain (e.g. postherpetic neuroalgia).
Referral to pain managment clinic may be indicated if probelms are difficult to treat and persist.
What is Brown-Séquard Syndrome?
Describe the clinical features.
Brown-Séquard Syndrome is a rare spinal disorder that resluts from an injury to one side of the spinal cord, resulting in hemisection.
This results in:
- ipsilateral upper motor neurone signs
- ipsilateral dorsal column signs (loss of light touch and vibration and proprioception)
- contralateral spinothalamic signs (loss of pain and temperature sensation)
Describe the main patters on motor and sensroy damage observed with a radial nerve palsy.
Motor: Wrist drop (as yupplies all the muscles in the forearm). Aka. saturday night palsy.
Sensory: Sensory loss to small area between the dorsal aspect of 1st and 2nd metacarpals.
What criteria are used to diagnose multiple sclerosis?
The McDonald criteria.
Basically a sophisticated way of showing “two lesions seperated by time and space”.
Describe the acute managment of migraines.
First-line: Offer compination therapy with an oral triptan plues either NSAID or paracetamol.
For young people (12-17) consider nasal spray triptan instead of oral.
If these measures are not effective, offer non-oral metoclopramide (a D2 antagonist) and non-oral NSIAD or Triptan.
Summarise the prophylactic managment of migraines.
Prophylaxis should be given if experiencing ≥2 attacks/month.
1st line: topiramate or propanolol. (propanoloy preferred in women of childbearing age).
If this fails, offer 10 sessions of accupunture, or gabapentin.
Adivse that 400mg OD of riboflavin may help with migraines.
What are the symptoms of cerebellar disease?
D - Dysdiedokinesia
A - Ataxia (limb and truncal)
N - Nystagmus (horizontal; ipsilateral hemisphere)
I - Intention tremor
S - Slurred staccato speech; Scanning dysarthria
H - Hypotonia
(P - Past pointing)
What are causes of cerebellar disease?
- Fridreich’s Ataxia
- Neoplastic (e.g. cerebellar haemiangioma)
- Stroke
- Alcohol
- Multiple sclerosis
- Hypothyroidism
- Drugs: Phenytoin, lead poisoning
Roughly describe which area the PCA, MCA and ACA supply.
What is the name of the tool used to assess patients with suspected strokes?
ROSIER (Recognition of stroke in the emergency room) - recommended by NICE to assess stroke symptoms in the acute setting
NOTE: FAST is a tool only used by the general public to allow them to seek quick help.
What is autonomic dysreflexia?
Autonomic dysrelfexia is a sudden and very exaggerated sympathetic nervous system activation, presenting with etreme hypertension, flushing and sweating above the level of the cord lesion.
It is triggered by afferent signals, usually faecal impaction or urinary retention which cause a sympathetic spinal reflex via the thoracocolumbar outflow.
It occurs in patients with a lesion above T6 in the spinal cord.
What are the characterisitc feautures of a common perineal leasion?
The most characteristic is foot drop. Others include:
- Weekness of foot dorsiflexion
- Weakness of foot eversion
- Weakness of extensor hallucis longus
- Sensory loss over dorsum of foot and lower part of leg
- Wasting of anterior tibial and peroneal muscles
What are the features that point towards a diagnosis of motor neurone disease?
Characteristic features:
- Fasciculations
- Absence of sensory signs/symptoms
- Mixture of upper and motor neurone signs
- Wasting of small muscles in hand/tibialis anterior is common
Other features:
- Doesn’s affect occular muscles
- No cerebellar signs
- Abdominal reflexes and sphincter dysfunction if present is a late feature.
What is Hoffman’s Sign?
In which conditions can this be observed?
Hoffman’s sign is elicited by flicking the distal phalynx of the middle finger, causing momentary flexion. A positive result is exaggerated flexion of the terminal phalynx of the thumb.
This is an upper motor neurone sign, as seen in e.g.:
- Multiple sclerosis
- Degenerative cervical myelopathy
What is Kernig’s Sign and when can this be observed?
Kernig’s sign is pain on knee extension from a position of hip flexion and knee flexion.
It suggests meningeal irritation, e.g. meningitis or subarachnoid haemorrhage.
What is Tinel’s test, and when can this be observed?
What is another test that is positive in this condition?
Tinel’s test is a way to test for an irritated nerve. It is elicited by lightly tapping the nerve repeatedly. If positive, it will cause a tingling sensation.
It is most commonly used to diagnose Carpal tunnes syndrome; tap a the anterior wrist, and a tingling should be felt in thumb, index and middle finger.
Another test is Phalen’s Sign (aka. reverse prayer sign). When this is held for 30-60 seconds, a positive sign would again elicit tingling/stinging/pain.
What is a complication of sinusitis that you need to suspect if someone develops focal neurological signs and fever?
Cerebral abscess.
Other than from sinusitis, this can also be a complication of middle ear infections.
What is cavernous sinus thrombosis?
How does it present?
A blood clot within the cavernous sinus can be septic or aseptic.
Septic is more common, as a result of infection spreading from nose/sinuses/ears. Other causes include: neoplasia, trauma.
Clinical features include:
- Opthalmoplegia (6th nerve typically before 3rd and 4th)
- Trigeminal nerve involvment may occur. -> Hyperaesthesia of upper face and eye pain
- Central retinal vein thrombosis
With regards to hearing, what is acommon complaint of facial nerve palsy?
The facial nerve also supplies the corda timpani, which supplies the stapedius muscle. This muscle reduces loudness, and therefore paralysis is associated with hyperacusis. Other features of a facial nerve palsy include:
- Facial muscles: paralysis. Can’t close eyelids, but it’s droopy
- Tasta: loss of anterior 2/3rds of tongue
- Parasympathetic: lacrimal and salivary glands.
What are the clinical features of tuberous sclerosis?
Clinical features
Cutaneous:
- Depigmented “ash-leaf” spots with fluoresce under UV light
- Roughened patches of skin over lumbar spine (Shagreen patches)
- Adenoma sebaceum (aka. Angiofibromas): butterfly distribution over nose
- Fibromata beneath nails (subungal fibromata)
- Café-au-lait spots may be seen (though more associated with neurofibromatosis)
Neurological feautres:
- Developmental delay
- Epilepsy
- Intellectual impairment
Others:
- Increased lifetime risk of cancer
- Retinal hamartomas (dense white areas on retina)
- Rhabdomyomas of the heart
- Gliomatous changes that occur in brain lesions
- Polycystic kidneys,
- Lung cysts (lymphangioleiomyomatosis)
Distinguish between Erb’s and Klumpke’s Paralysis.
Erb’s:
- Damage to C5 and C6 nerve roots
- Leads to winged scapula
- Arm in “waiter’s tip position”
Klumpke’s:
- Damage to C8 and T1 nerve roots
- leads to claw hand by affecting intrinsic muscles of the hand
Compare and contrast the skin lesions seen in tuberous sclerosis and neurofibromatosis.
Tuberous sclerosis:
- Ash-leaf (depigmented) spots (depigmented)
- Angiofibroma (facial rash)
- Shagreen pathches
- Subungal fibromata
Neurofibromatosis:
- Café-au-lait spots
- Neurofibromas
- Frackling in skin folds
What is the first-line medication used after a TIA or stroke (after 14 days)?
First line: clopidogrel 75mg + statin
Second Line: aspirin 75mg + dipyridamole + statin
Third line: dipyridamole + statin
Summarise the antiplatelet regimens for a patient with an ischaemic stroke.
Aspirin 300mg OD for 2 weeks, then clopidogrel 75mg long-term.
Plus a statin.
Describe the muscles of the eye and their inntervation.
Third nerve:
- Medial rectus
- Inferior Oblique
- Inferior rectus
- Superior recus
4th nerve:
- Superior oblique
6th nerve:
- Lateral rectus
What are the contraindications to prescribing triptans?
Triptans should not be prescribed in patients with a history of, or significant risk for:
- Ischaemic Heart Disease
- Cerebrovascular Disease
How should triptans be taken?
- Should be taken as soon as possible after the onset of headache (rather than onset of aura)
- There are several modes of administration:
- Oral, nasal spray, SC injections
What are the adverse effects of triptans?
Tingling, head, heaviness, pressure, tighness in throat and chest.
Describe when AED can be considered to be stopped.
Can be considered to be stopped if seizure free for > 2 years, with AEDs being stopped over 2-3 months.
What is degenerative cervical myelopathy?
DCM involves cord dysfunction from compression in the neck.
Due to its mobility, the vertebral column of the neck is particularly prone to degenerative changes such as disc herniation, ligament hypertrophy or ossification, and osteophyte formation.
DCM is more common with age.
What are the common symptoms of degenerative cervical myelopathy?
A common presentation is paraesthesia, which make it easily confused with carpal tunnel syndrome . Other symptoms include:
- Neck pain/stiffness
- Unilateral limb weakness, numbness, loss of dexterity
- Unilateral body pain
- Autonomic dysfunction (bowel/bladder incontinence, ED)
- Imbalance/falls
What are the common signs of degenerative cervical myelopathy?
- Motor signs:
- Pyramidal weakness
- Limb hyperreflexia
- Spasticity
- Hoffman’s sign
- Babinski’s sign
- Lhermitte’s sign (electric shock sensation down the spine on eck felxion)
Summarise the management of Parkinson’s disease.
Fist-line treatment:
- If motor symptoms are affecting the patients QoL: levodopa (often in conjunction with a decarboxylase inhibitor such as carbidopa)
- If motor symptoms are not affecting the patient’s QoL:
- Dopamine agonist (e.g. ropinirole, apomorphine)
- Levodopa
- Monoamine oxidase B (MAO-B) inhibitors (e.g.slelgiline)
If a patient continues to have smptoms despite optimal levodopa treatment, add dopamine agonist, MAO-B inhibitor or catechol-O-methyltransferase inhibitor (e.g. entecapone, opicapone) as an adjunct.
(NB that bromocriptine and cabergoline have been associated with pulmonary/cardiac fibrosis and are therefore not recommended first line)
What medication can be used to control drug induced parkinsonism?
Antimuscarinics, such as procyclidine, can be used to control drug induced parkinsonism (block cholinergic receptors).
What are the symptoms of normal pressure hydrocephalus?
They can be remembered as wet, wobbly and whacky.
- Urinary incontinence
- Gait abnormality (may be similar to Parkinson’s disease)
- Dementia and bradyphrenia
~60% of patients will have all 3 at diagnosis; symptoms develop over a few months.
What are the investigations of normal pressure hydrocephalus?
-
CT head (non-contrast)/MRI
- Would show snlarged ventricles with cortical atrophy
-
Levodopa challenge
- Parkinson’s is an important differential, so patient is given therapeutic trial of levodopa. If symptomsa re responsive, NPH can be ruled out.
Summarise the management of normal pressure hydrocephalus.
If patient is suitable for surgery:
- Ventriculoperitoneal shunt
- Control of cardiovascular risk factors (HTN, diabetes, statins etc)
If not suitable for surgery:
- Control of cardiovascular risk factors
- Large-volume CSF taps
What is the mode of inheritance of Charcot-marie-Tooth disease?
CMT disease is autosomal dominant.
What is Charcot-Marie-Tooth disease?
CMT, a hereditary sensorimotor neuropathy (HSMN), is an autosomal dominant disease of the peripheral nerves. It is due to a defect in the PMP-22 gene which codes for myelin. As the name suggests, it affects both motor and sensory preipheral nerves.
This leads to progressive peripheral nerve damage:
- Distal muscle wasting (pes cavus, hammer toe)
- Foot drop
- Leag weakness
Symptoms often start in puberty.
What is Multiple System Atrophy?
MSA is a rare neurodegenerative disorder characterised by:
- Parkinsonian features
- Autonomic dysfunction
- Impotence
- Urinary incontinence
- Urinary retention
- Postural instability
- Orthostatic hypotension
It is a Parkinsons Plus disease, and responds poorly to dopaminergic medication.
There are 2 types, MSA-P and MSA-C; the former presents with predominantly Parkinsonian features, the latter with Cerebellar features.
Summarise the Oxford Stroke Classification.
The Oxford Stroke Classification (Bamford Classification) classifies strokes based on the initial symptoms. Summary as follows:
- Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg.
- Homonymous Hemianopia
- Higher cognitive dysfunction (e.g. dysphasia)
Total anterior circulation infarcts:
- Involves MCA and ACA
- All 3 of above criteria present
Partial circulation infarcts:
- Involves smaller arteries of the anterior circulation, e.g. upper or lower division of MCA
- 2 of the above symptoms present
Lacunar infarcts:
- Involves perforating arteries of the internal capsule
- Presents with one of the following:
- Unilateral weakness (± sensory deficit) of face and arm, arm and leg or all three
- Pure sensory stroke
- Ataxic hemiparesis
Posterior Circulation infarcts:
- Involves vertebrobasilar arteries
- Presents with one of the following
- Cerebellar of brainstem syndromes
- LoC
- Isolated homonymous hemianopia
What is the most effective treatment for degenerative cervical myelopathy?
Decompressive surgery is the most effective treatment currently - has been shown to prevent disease progression.
What is an internuclear ophthalmoplegia?
INO occurs due to a lesion of the medial longitudinal fasciculus, a tract that allows conjugate eye movement. This results in impariment of adduction of the ipsilateral eye. The contralateral eye aboducts - with nystagmus. (see image)
A common cause of this condition in younger patients is MS.
What is cavernous sinus syndrome?
Cavernous sinus syndrome is a syndrome characterised by neuropahties of the cranial nerves passing through the cavernous sinus. These include:
- Occulomotor
- Trochlear
- Ophthalmic
- Maxillary (V2)
- Abducens
Clinical features:
- Palsies of the nerves above
What are potential causes of cavernous sinus syndrome?
- Infection
- Vascular:
- Aneurysm
- Thrombosis
- Inflammation (SLE, amyloidosis)
- Neoplasia
Summarise the aetiology and risk factors for Bell’s Palsy.
The aetiology is not fully known. Potentially viral-associated (HSV) or Lyme disease.
Risk factors include pregnancy: 3x more common.
How is Bell’s Palsy managed?
- Prednisolone 1 mg/kg for 10 days within 72 hours of onset (aciclovir has no benefit)
- Eye care: artificial tears/eye lubricants