Neurology Flashcards
Drug side effects:
Peripheral neuropathy
amiodarone phenytoin metronidazole nitrofurantoin isoniazid
Drug side effects:
thrombocytopenia
sodium valproate
heparin
Mechanism of action:
Phenytoin
binds to sodium channels increasing their refractory period
P450 inducer
Phenytoin
Adverse effects
Acute
initially: dizziness, diplopia, nystagmus, slurred speech, ataxia
later: confusion, seizures
Phenytoin
Adverse effects
Chronic
gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness
megaloblastic anaemia (secondary to altered folate metabolism)
peripheral neuropathy
enhanced vitamin D metabolism causing osteomalacia
lymphadenopathy
dyskinesia
Phenytoin
Adverse effects
Idiosyncratic
fever rashes, including severe reactions such as toxic epidermal necrolysis hepatitis Dupuytren's contracture* aplastic anaemia drug-induced lupus
Phenytoin monitoring
Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose should be checked if:
adjustment of phenytoin dose
suspected toxicity
detection of non-adherence to the prescribed medication
Brain lesions
Gross anatomy
Parietal lobe lesions
sensory inattention apraxias astereognosis (tactile agnosia) inferior homonymous quadrantanopia Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
Brain lesions
Gross anatomy
Occipital lobe lesions
homonymous hemianopia (with macula sparing)
cortical blindness
visual agnosia
Brain lesions
Gross anatomy
Temporal lobe lesions
Wernicke’s aphasia: this area ‘forms’ the speech before ‘sending it’ to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent
superior homonymous quadrantanopia
auditory agnosia
prosopagnosia (difficulty recognising faces)
Brain lesions
Gross anatomy
Frontal lobe lesions
expressive (Broca's) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting disinhibition perseveration anosmia inability to generate a list
Brain lesions
Gross anatomy
Cerebellar lesions
midline lesions: gait and truncal ataxia
hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus
Brain lesions
Medial thalamus and mammillary bodies of the hypothalamus
Wernicke’s and Korsakoff’s
Brain lesions
Subthalamic nucleus of the basal ganglia
Hemiballism (coarse, violent, wide amplitude movements, ipsilateral arm and leg).
Brain lesions
Striatum (caudate nucleus) of the basal ganglia
Huntington’s Chorea
Brain Lesions
Substantia nigra of the basal ganglia
Parkinson’s disease
Brain lesions
Amygdala
Kluver-Bucy syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia
What is Friedreich’s ataxia?
Friedreich’s ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich’s ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation.
The typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features.
Neurological features of Friedreich’s ataxia
absent ankle jerks/extensor plantars
cerebellar ataxia
optic atrophy
spinocerebellar tract degeneration
Other features of Friedreich’s ataxia
hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate
uses of carbamazepine
focal seizures - epilepsy
trigeminal neuralgia
bipolar disordre
Mechanism of action - carbamazepine
binds to sodium channels to increase their refractory period
Side effects of carbamazepine
P450 enzyme inducer dizziness and ataxia drowsiness headache visual disturbances (especially diplopia) Steven-Johnson syndrome leucopenia and agranulocytosis hyponatraemia secondary to syndrome of inappropriate ADH secretion
Carbamazepine is known to exhibit autoinduction, hence when patients start carbamazepine they may see a return of seizures after 3-4 weeks of treatment.
Stroke symptoms
anterior cerebral artery
Contralateral hemiparesis and sensory loss, lower extremity > upper
Stroke symptoms
middle cerebral artery
Contralateral hemiparesis and sensory loss, upper extremity > lower
Contralateral homonymous hemianopia
Aphasia
Stroke symptoms
posterior cerebral artery
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
Stroke symptoms
Weber’s syndrome (branches of the posterior cerebral artery that supply the midbrain)
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
Stroke Symptoms
Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
Stroke Symptoms
Anterior inferior cerebellar artery (lateral pontine syndrome)
Symptoms are similar to Wallenberg’s (see above), but:
Ipsilateral: facial paralysis and deafness
Stroke symptoms
retinal/ ophthalmic artery
amaurosis fugax
Stroke symptoms
Basilar artery
Locked in syndrome
Stroke symptoms
Lacunar infarcts
present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
strong association with hypertension
common sites include the basal ganglia, thalamus and internal capsule
Tuberous sclerosis
Autosomal dominant
Like neurofibromatosis, the majority of features seen in TS are neurocutaneous
Cutaneous features of tuberous sclerosis
depigmented ‘ash-leaf’ spots which fluoresce under UV light
roughened patches of skin over lumbar spine (Shagreen patches)
adenoma sebaceum (angiofibromas): butterfly distribution over nose
fibromata beneath nails (subungual fibromata)
café-au-lait spots* may be seen
Neurological features of tuberous sclerosis
developmental delay
epilepsy (infantile spasms or partial)
intellectual impairment
Other features of tuberous sclerosis
retinal hamartomas: dense white areas on retina (phakomata)
rhabdomyomas of the heart
gliomatous changes can occur in the brain lesions
polycystic kidneys, renal angiomyolipomata
lymphangioleiomyomatosis: multiple lung cysts
Sensory lesions of the spinal cord
Neurosyphilis
affects the dorsal columns
loss of vibration and proprioception
Motor lesions of the spinal cord
ALS/MND
affects both upper (corticospinal tracts) and lower motor neurons
results in a combination of upper and lower motor neuron signs
Motor lesions of the spinal cord
Polio
affects anterior horns resulting in lower motor neuron signs
Combined motor and sensory lesions of the spinal cord
Brown-Sequard syndrome (spinal cord hemisection)
Which tracts
- Lateral corticospinal tract
- Dorsal columns
- Lateral spinothalamic tract
Combined motor and sensory lesions of the spinal cord
Brown-Sequard syndrome (spinal cord hemisection)
Clinical notes
- Ipsilateral spastic paresis below lesion
- Ipsilateral loss of proprioception and vibration sensation
- Contralateral loss of pain and temperature sensation
Combined motor and sensory lesions of the spinal cord
Subacute combined degeneration of the spinal cord (vitamin B12 & E deficiency)
Which tracts
- Lateral corticospinal tracts
- Dorsal columns
- Spinocerebellar tracts
Combined motor and sensory lesions of the spinal cord
Subacute combined degeneration of the spinal cord (vitamin B12 & E deficiency)
Clinical notes
- Bilateral spastic paresis
- Bilateral loss of proprioception and vibration sensation
- Bilateral limb ataxia
Combined motor and sensory lesions of the spinal cord
Friedreichs ataxia
Which tracts
- Lateral corticospinal tracts
- Dorsal columns
- Spinocerebellar tracts
Combined motor and sensory lesions of the spinal cord
Friedreichs ataxia
Clinical notes
- Bilateral spastic paresis
- Bilateral loss of proprioception and vibration sensation
- Bilateral limb ataxia
In addition cerebellar ataxia → other features e.g. intention tremor
Combined motor and sensory lesions of the spinal cord
Anterior spinal artery occlusion
which tracts
- Lateral corticospinal tracts
2. Lateral spinothalamic tracts
Combined motor and sensory lesions of the spinal cord
Anterior spinal artery occlusion
Clinical notes
- Bilateral spastic paresis
2. Bilateral loss of pain and temperature sensation
Combined motor and sensory lesions of the spinal cord
Syringomelia
Which tracts
- Ventral horns
2. Lateral spinothalamic tract
Combined motor and sensory lesions of the spinal cord
Syringomelia
Clinical notes
- Flacid paresis (typically affecting the intrinsic hand muscles)
- Loss of pain and temperature sensation
Combined motor and sensory lesions of the spinal tract
MS
Which tracts
Asymmetrical, varying spinal tracts involved
Combined motor and sensory lesions of the spinal tract
MS
Clinical notes
Combination of motor, sensory and ataxia symptoms
Motor and descending tracts
pyrimadal tracts
Lateral corticospinal tracts
anterior corticospinal tracts
Motor and descending tracts
Extrapyrimidal tracts
rubrospinal tracts
reticulospinal tracts
olivospinal tracts
vestibular spinal tracts
Sensory and ascending tracts
Dorsal column medial lemniscus system
Grascile fasciculus
Cuneate fasciculus
Sensory and ascending tracts
Spinocerebellar tracts
posterior spinocerebellar tracts
anterior spinocerebellar tracts
Sensory and ascending tracts
Anterolateral tracts
anterior spinothalamic tracts
lateral spinothalamic tracts
spino-oliviary tract
Bitemporal hemianopia
lesion of optic chiasm
upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour
lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
Homonymous quadrantanopias
superior: lesion of the inferior optic radiations in the temporal lobe (Meyer’s loop)
inferior: lesion of the superior optic radiations in the parietal lobe
mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
homonymous hemianopia
incongruous defects: lesion of optic tract
congruous defects: lesion of optic radiation or occipital cortex
macula sparing: lesion of occipital cortex
what are congruent visual defects
A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric.
incongruous defects = optic tract lesion;
congruous defects = optic radiation lesion or occipital cortex
Key features of myasthenia gravis
The key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest:
extraocular muscle weakness: diplopia
proximal muscle weakness: face, neck, limb girdle
ptosis
dysphagia
Associations with myasthenia gravis
thymomas in 15%
autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE
thymic hyperplasia in 50-70%
Investigations of myasthenia gravis
single fibre electromyography: high sensitivity (92-100%)
CT thorax to exclude thymoma
CK normal
autoantibodies: around 85-90% of patients have antibodies to acetylcholine receptors. In the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies
Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used any more due to the risk of cardiac arrhythmia
Management of myasthenia gravis
long-acting acetylcholinesterase inhibitors
pyridostigmine is first-line
immunosuppression:
prednisolone initially
azathioprine, cyclosporine, mycophenolate mofetil may also be used
thymectomy
Management of myasthenic crisis
plasmapheresis
intravenous immunoglobulins
Pharmacology of triptans
Triptans are specific 5-HT1B and 5-HT1D agonists used in the acute treatment of migraine. They are generally used first-line in combination therapy with an NSAID or paracetamol.
_ take after the onset of headache, not aura
adverse effects of triptans
‘triptan sensations’ - tingling, heat, tightness (e.g. throat and chest), heaviness, pressure
triptan contraindications
patients with a history of, or significant risk factors for, ischaemic heart disease or cerebrovascular disease
What is restless leg syndrome
Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present
Features of restless leg syndrome
uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest
paraesthesias e.g. ‘crawling’ or ‘throbbing’ sensations
movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)
Causes and associations of restless leg syndrome
there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy