Neurology Flashcards
Define epilepsy
neurological disorder that can cause someone to experience recurrent seizures (sudden, uncontrolled, disorganised electrical activity in the brain)
Define seizure
(sudden, uncontrolled, disorganised electrical activity in the brain)
What’s the difference between focal and generalised seizures?
Focal = in a specific area, on one side of the brain
Generalised = involves networks on both sides of the brain
Name four types of focal seizures
Temporal - aura (hallucinations, experiential phenomena, rising epigastric sensation), automatisms
Frontal - head/ leg movements, posturing, post-ictal weakness, Jacksonian march
Parietal - paraesthesia
Occipital - floaters/ flashes
Presentation of temporal seizure
aura (hallucinations, experiential phenomena, rising epigastric sensation)
automatisms (lip-smacking)
Presentation of frontal lobe seizure
head/ leg movements
Posturing
Post-ictal weakness
Jacksonian march - seizure begins with tingling/ twitching in finger/ toe/ corner of mouth, spreads to larger area of body (licking limbs, head turning, eye movements)
Presentation of parietal seizure
Paraesthesia
Presentation of occiptal seizure
floaters/ flashes
Types of generalised seizure
Tonic - tensing, clenching, tongue biting, incontinence
Atonic - complete loss of tone, collapse
Myoclonic - jerking limb, eye rolling, convulsions
Grand mal - aura + tonic (10-60s) + clonic (seconds - minutes)
Absence (petit mal) - patient unresponsive but conscious, stares, many attacks in one day
Features of a tonic seizure
Tensing
Clenching
Tongue biting
Incontinence
Features of atonic seizure
Complete loss of tone, collapse
Features of myoclonic seizure
Jerking limbs
Eye rolling
COnvulsions
Features of grand mal seizure
Aura + tonic seizure (10-60s) + clonic seizure (seconds- minutes)
Features of petit mal seizure
patient unresponsive but conscious, stares, many attacks in one day
Preventative management of focal seizures
Levetiracetam/ lamotrigine (men + women)
Management of generalised tonic-clonic
Men - sodium valproate
Women - Levetiracetam/ lamotrigine
Management of myoclonic seizures
Men - sodium valproate
Women - Levetiracetam
Management of tonic-clonic seizures
Men - sodium valproate
Women - Lamotrigine
Management of absence seizures
Ethosuximide (men + women)
What medication would you definitely not use for women of childbearing age who have epilepsy?
maternal use of sodium valproate is associated with a significant risk of neurodevelopmental delay in children. Guidance is now clear that sodium valproate should not be used during pregnancy and in women of childbearing age unless clearly necessary.
DVLA + epilepsy
Patient must inform the DVLA (or Doctor if the patient refuses). Can’t drive - seizure in the last 12 months/ changed medication in the last 6 months.
Define status epilepticus
Single seizure > 5 mins, 2 + seizures within a 5 minute period
Mx:
A to E
PR dizaepam/ buccal midazolam (pre-hospital), IV lorazepam (in hospital)
2nd dose of BDZs if unresolved after 5-10 minutes
IV phenytoin (or levetiracetam, sodium valproate)
Refractory status - induce general anaesthesia
Management of status epilepticus
Single seizure > 5 mins, 2 + seizures within a 5 minute period
Mx:
A to E
PR dizaepam/ buccal midazolam (pre-hospital), IV lorazepam (in hospital)
2nd dose of BDZs if unresolved after 5-10 minutes
IV phenytoin (or levetiracetam, sodium valproate)
Refractory status - induce general anaesthesia
What is Non-epileptic attack disorder?
type of seizure which looks similar to an epileptic seizure but it is not caused by abnormal electrical discharges or blood pressure
Occur when brain can’t handle particular emotion/ thoughts/ memories/ sensations
Patients may have a Hx of mental health problems or personality disorder
What is narcolepsy?
brain unable to regulate sleep-wake cycle, characterised by excessive daytime sleepiness (EDS) + REM abnormalities for > 3 months
Clinical presentation of narcolepsy
Onset in teenage years: EDS, hypnagogic (falling asleep)/ hypnopompic (waking) hallucinations, sleep paralysis, sleep attacks, catoplexy (conscious collapse)
Define catoplexy
sudden loss of muscle tone while patient is conscious (conscious collapse), triggered by loud noise or emotion e.g. laughter/ surprise.
Management of narcolepsy
Sleep hygiene + lifestyle modifications (e.g. forced naps)
Stimulants e.g. Modafinil
Antidepressants e.g. SSRIs
What is shingles?
acute unilateral pain blistering caused by reactivation of VSV (lie dormant in dorsal root/ cranial nerve ganglia), most commonly affects dermatomes T1-L2
Most common dermatomes affected by shingles
T1-L2
Clinical presentation of shingles
Prodrome (burning pain over dermatome for 2-3 days, fever, headache, lethargy) →
Erythematous macular rash (does not cross midline)
Diagnosis of shingles
Clinical
Management of shingles
Advice (avoid high-risk groups e.g. pregnant women/ immunocompromised)
Analgesia (paracetamol + NSAIDs → amitriptyline → oral corticosteroids if immunocompetent)
Antivirals - acyclovir, famciclovir, valacyclovir
Avoid if <50YO w/ mild rash + mild pain + no underlying RFs
Where does VSV lie dormant before reactivating and causing shingles?
Dorsal root/ cranial nerve ganglia
Most common complication of shingles
post-herpetic neuralgia (lasting pain in area of shingles, most resolve within 6 months)
Complications of shingles
Most common = post-herpetic neuralgia (lasting pain in area of shingles, most resolve within 6 months)
Others - herpes zoster ophthalmicus (ocular complications), herpes zoster oticus (ear lesions/ facial paralysis)
What is Wernicke-Korsakoff syndrome?
Thiamine (Vitamin B1) deficiency most commonly caused by excess alcohol consumption.
If Wernicke’s encephalopathy is left untreated, Korsakoff syndrome will develop
Triad of Wernicke’s encephalopathy
Encephalopathy: confusion, disorientation, indifference, inattentiveness
Occulomotor disturbance (nystagmus, opthalmoplegia)
Ataxia
Clinical features of Korsakoff’s syndrome
Memory impairment (antero + retrograde)
Confabulation
Behavioural change
Management of Wernicke-Korsakoff syndrome
Requires urgent thiamine supplementation + abstinence from alcohol
((Wernicke’s encephalopathy = medical emergency with high mortality if untreated, Korsakoff syndrome = generally irreversible))
What is Huntington’s?
inherited (autosomal dominant) neurodegenerative disorder that causes progressive neurological dysfunction, starts from 30-50YO
Pathophysiology of Huntington’s
Trinucleotide repeat disorder (repeat expansion of CAG) causes defect in huntington gene on chromosome 4 → degeneration of cholinergic + GABAnergic neurons in the striation of the basal ganglia
Exhibits genetic anticipation - successive generations have more repeats in gene → earlier age of onset + increased severity
Inheritance pattern of Huntington’s disease
Autosomal dominant
What is genetic anticipation? which disease is it associated with?
Trinucleotide repeat disorder –> successive generations have more repeats in gene → earlier age of onset + increased severity
Huntington’s
Genetic defect that causes Huntington’s disease
Trinucleotide repeat disorder (repeat expansion of CAG) causes defect in huntington gene on chromosome 4 → degeneration of cholinergic + GABAnergic neurons in the striation of the basal ganglia
Clinical presentation of Huntington’s
30-50YO.
Cognitive/ psychiatric/ mood problems → movement disorder (chorea, dystonia, rigidity, eye movement disorders, dysarthria, dysphagia)
Management of Huntington’s
Supportive (no options to slow/ stop progression)
Tetrabenazine - chorea
SSRIs - depression
Physiotherapy
Speech and language therapy
Genetic counselling for relatives/ children
Medical management of Chorea in Huntington’s disease
Tetrabenazine
What is Parkinson’s disease?
progressive neurodegenerative disorder caused by degeneration of dopaminergic neurons in the substantia nigra → classic triad (bradykinesia, tremor, rigidity) of features that present asymmetrically
Clinical presentation of Parkinson’s disease
Asymmetrical
Triad: bradykinesia (short, shuffling gait w/ reduced arm swing), tremor (pin-rolling), rigidity (cogwheel)
Other: mask-like face, psychiatric symptoms (depression, dementia, psychosis, sleep disturbance), impaired olfaction
Parkinson’s vs. benign essential tremor
BET = symmetrical, 6-12Hz, improves at rest/ worse on voluntary movement
Parkinson’s = asymmetrical, 4-8Hz, worse at rest/ improves on voluntary movement
Management of Parkinson’s
Motor symptoms affecting QOL = levodopa
Usually combined with carbidopa to reduce side effects (dry mouth, anorexia, palpitations, postural hypotension, psychosis)
Motor symptoms not affecting QOL = dopamine agonist, MAO-B inhibitor (e.g. selegiline) or levodopa
((Ease symptoms but don’t slow progression))
Management of Parkinson’s - motor symptoms affecting QOL
levodopa
Usually combined with carbidopa to reduce side effects (dry mouth, anorexia, palpitations, postural hypotension, psychosis)
Management of Parkinson’s - motor symptoms not affecting QOL
dopamine agonist, MAO-B inhibitor (e.g. selegiline) or levodopa
Why is levodopa usually combined with carbidopa?
To reduce side effects: dry mouth, anorexia, postural hypotension, psychosis, palpitations
What is dementia?
Syndrome characterised by progressive, irreversible global cognitive deficits. Memory impairment is often the first symptom with progression to other symptoms.
What is the most common cause of Dementia in the UK?
Alzheimer’s disease
Pathophysiology of Alzheimer’s Disease
amyloid plaques + neurofibrillary tangles (tau protein) accumulate → decreased information transmission → widespread cerebral atrophy involving the cortex + hippocampus
Clinical presentation of Alzheimer’s Disease
60yrs +, progressive memory loss over time
Management of Alzheimer’s disease
acetylcholinesterase inhibitor (e.g. donepezil, rivastigmine, galantamine) + memantine
What is vascular dementia?
Second most common cause of dementia in the UK. Cognitive impairment caused by vascular damage + impaired blood supply to the brain.
Clinical presentation of Vascular Dementia
several months/ years of a sudden/ stepwise deterioration of cognitive function. Difficulty w/ attention/ concentration, seizures, memory/ gait/ speech/ emotional disturbance.
RFs for Vascular dementia
Hx of CVA (or family Hx), AF, HTN, DM, hyperlipidaemia, smoking, obesity
What type of dementia is associated with a stepwise progression?
Vascular dementia
What is Lewy Body dementia?
Type of dementia associated with Parkinson’s.
Charcaterised by the presence of Lewy bodies in the substantia nigra/ paralimbic + neocortical areas.
Where can Lewy bodies be found in Lewy body dementia?
the substantia nigra/ paralimbic + neocortical areas.
What type of dementia can be characterised by fluctuation in cognition + parkinsonism + psychiatric features?
Lewy body dementia
Clinical presentation of Lewy Body dementia
progressive cognitive impairment (FLUCTUATES) affecting executive functioning + attention > memory. Parkinsonism. Psychiatric features (hallucinations + delusions)
Management of Lewy Body Dementia
Acetylcholinesterase + memantine.
AVOID NEUROLEPTICS - high percentage of DLB patients exhibit worsening parkinsonism, sedation, immobility, or even neuroleptic malignant syndrome (NMS) after exposure to antipsychotics
Why should antipsychotics be avoided in patients with Lewy Body dementia?
high percentage of DLB patients exhibit worsening parkinsonism, sedation, immobility, or even neuroleptic malignant syndrome (NMS) after exposure to antipsychotics
What is fronto-temporal dementia?
Degeneration of frontal + temporal lobes of brain –> personality change + impaired social conduct. Starts at a younger age
Behavioural presentation of fronto-temporal dementia
altered emotional responsiveness, decreased interpersonal skis, change in preferences
What type of dementia more commonly affects 40-60YOs?
Frontal-temporal dementia
Semantic presentation of Fronto-temporal dementia
reduced understanding of words, name retrieval difficult, inability to recognise objects/ family faces
Non-fluent presentation of fronto-temporal dementia
speech takes effort, breakdown in output of language, speech apraxia
The types of presentation seen in patients with fronto-temporal dementia
Behavioural - altered emotional responsiveness, decreased interpersonal skis, change in preferences
Semantic - reduced understanding of words, name retrieval difficult, inability to recognise objects/ family faces
Non-fluent - speech takes effort, breakdown in output of language, speech apraxia
Methods to assess dementia
AMTS - 6-8/10 = dementia
MMSE - 24/30 = dementia
MoCA - 26/30 = dementia
Addenbrookes - 82-88/100 = dementia
What are the five domains tested in the Addenbrookes assessment of Dementia?
Attention
Memory
Language
Visuospatial functioning
Verbal functioning
What is normal pressure hydrocephalus?
Abnormal build up of CSF in brain’s ventricles –> dilatation of centricles + pressure on the brain. Typically caused by head injury/ SAH/ meningitis.
Pathophysiology of normal pressure hydrocephalus
Normal flow of CSF blocked (decreased CSF absorption at arachnoid villi) → build up of fluid in ventricles → ventricles enlarge and put pressure on the brain
Clinical presentation of normal pressure hydrocephalus
Triad: urinary incontinence + dementia/ bradyphrenia + gait abnormality
Symptoms develop over a few months
What condition typically presents with: urinary incontinence + dementia/ bradyphrenia + gait abnormality?
Normal pressure hydrocephaus
Investigation of normal pressure hydrocephalus?
Non-contrast CT - visualise hydrocephalus + dilatation of ventricles
Management of normal pressure hydrocephalus
Ventriculoperitoneal shunting (complication = seizures, infections, intracerebral haemorrhage)
