Neuro fifth yr Flashcards
Summary of Huntington’s?
inherited neurodegenerative condition. It is progressive and incurable
Typically results in death 20 years after initial symptoms develop. Death often due to respiratory disease. Suicide common.
AD
trinucleotide repeat disorder: repeat expansion of CAG
HTT gene on chromosome 4
Anticipation - where the disease is presents at an earlier age in successive generations, and increased severity of disease
results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia
Features of Huntington’s?
typical develop after 35 years of age
cognitive, psychiatric or mood problems
chorea (involuntary, abnormal movements)
personality changes (e.g. irritability, apathy, depression) and intellectual impairment
dystonia
saccadic eye movements
Speech difficulties (dysarthria)
Swallowing difficulties (dysphagia)
Diagnosis and management of Huntington’s?
Dx
made in a specialist genetic centre using a genetic test for the faulty gene
pre-test and post-test counselling regarding the implications of the results
Tx
no treatment options for slowing or stopping the progression of the disease
supporting the person and their family
MDT - OT, PT, psychology, SALT, genetic counselling, advanced directives, EoL care planning
Symptomatic relief:
Medications to suppress disordered movement - antipsychotics (e.g., olanzapine), BDZs, Dopamine-depleting agents (e.g., tetrabenazine)
Depression - antidepressants
Types of migraines?
Migraine without aura
Migraine with aura (visual changes - blurring, lines, loss of visual fields)
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine (hemiplegia, ataxia, change in consciousness - mimic stroke)
What are migraines?
Neurological condition that is a cause of primary headache.
Occur in attacks following typical pattern
more common in women
Features of migraines?
a severe, unilateral, throbbing headache
associated with nausea, photophobia and phonophobia
attacks may last up to 72 hours
patients characteristically go to a darkened, quiet room during an attack
‘classic’ migraine attacks are precipitated by an aura. These occur in around one-third of migraine patients
typical aura are visual, progressive, last 5-60 minutes and are characterised by transient hemianopic disturbance or a spreading scintillating scotoma
formal diagnostic criteria are produced by the International Headache Society
5 stages - prodromal, aura, headache, resolution, recovery
Triggers for migraines?
Tired/stress
Alcohol
COCP
Lack of food or dehydration
Food - cheese, chocolate, red wine, citrus fruits
Menstruation
Bright lights
Acute treatment of migraine?
5-HT receptor agonists acutely
first-line: offer combination therapy with an oral triptan and an NSAID, or an oral triptan and paracetamol
for young people aged 12-17 years consider a nasal triptan in preference to an oral triptan
if the above measures are not effective or not tolerated offer a non-oral preparation of metoclopramide* or prochlorperazine and consider adding a non-oral NSAID or triptan
Prophylaxis of migraines?
5-HT receptor antagonist in prophylaxis
prophylaxis should be given if patients are experiencing 2 or more attacks per month.
topiramate or propranolol - Propranolol should be used in preference to topiramate in women of childbearing age as it may be teratogenic and it can reduce the effectiveness of hormonal contraceptives
if these measures fail NICE recommends ‘a course of up to 10 sessions of acupuncture over 5-8 weeks’
migraines triggered by menstruation - prophylaxis with NSAIDs (mefanamic acid) or triptans
What is Brown-Sequard syndrome?
Caused by lateral hemisection of the spinal cord
Features - ipsilateral weakness below lesion, ipsilateral loss of proprioception and vibration sensation, contralateral loss of pain and temperature sensation
What is Parkinson’s disease?
a condition where there is a progressive reduction of dopamine in the basal ganglia of the brain, leading to disorders of movement. Characteristicially asymmetrical, with one side more affected than the other.
Basal ganglia is responsible for coordinating habitual movements such as walking or looking around, controlling voluntary movements and learning specific movement patterns. Part of the basal ganglia called the substantia nigra produces a neurotransmitter called dopamine.
Features of Parkinsons disease?
typical patient is an older aged man around the age of 70. Twice as common in men. Mean age of diagnosis is 65 yrs.
Triad - resting tremor, rigidity, bradykinesia
Unilateral tremor - 4-6Hz, pill rolling, pronounced when resting, improves on voluntary movement, worsens if distracted
Cogwheel rigidity
Bradykinesia - movements slower and smaller - handwriting, shuffling gait, difficulty initiating movement, difficulty turning, hypomimia
Depression
Sleep disturbance and insomnia
Anosmia
Postural instability
Cognitive impairment and memory problems
Difference between Parkinsons tremor and benign essential tremor?
Parkinsons - asymmetrical, 4-6Hz, worse at rest, improves with intentional movement, other Parkinson features, no changes with alcohol
Benign essential tremor - symmetrical, 5-8Hz, improves at rest, worse with intentional movement, no other Parkinsons features, improves with alcohol
Diagnosis of Parkinsons?
Clinical diagnosis - should be made by a specialist
NICE recommend using UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria.
Can use single photon emission computed tomography (SPECT) to differentiate between Parkinsons and benign essential tremor
Management of Parkinsons?
Treatment is initiated and guided by a specialist, and is tailored to each individual patient and their response to different medications.
No cure - focus on controlling Sx and minimising SE
Levodopa - boost dopamine levels, usually combined with drug that stops levodopa being broken down in the body (peripheral decarboxylase inhibitors - carbidopa, benserazide) - so combination drugs include: co-benyldopa, co-careldopa. Levodopa is most effective but becomes less effective over time - so is saved for when other treatments aren’t managing Sx. Too much dopamine is SE - cause dyskinesias (e.g., dystonia, chorea, athetosis)
COMT inhibitors - catechol-o-methytransferase inhibitors, e.g., entacapone. Taken with levodopa to slow breakdown of levodopa in the brain.
Dopamine agonists - mimic dopamine in BG and stimulate dopamine receptors. Less effective than levodopa, and used to delay use of levodopa. Eg. bromocryptine, pergolide, Cabergoline. SE - pulmonary fibrosis.
Monoamine oxidase-B inhibitors - Monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin and adrenaline. The monoamine oxidase-B enzyme is more specific to dopamine and does not act on serotonin or adrenalin. Hence increases circulating dopamine. Delays use of levodopa. Eg. selegiline, rasagiline
Examples of Parkinson-plus syndromes?
MSA
Dementia with Lewy bodies
Progressive Supranuclear Palsy
Corticobasal degeneration
Features of drug-induced Parkinsonism?
motor symptoms are generally rapid onset and bilateral
rigidity and rest tremor are uncommon
Summary of multi system atrophy?
2 types - MSA-P (predominant Parkinsonism features) and MSA-C (predominant cerebellar features)
Shy-Drager syndrome is a type of MSA
Features - Parkinsonism, autonomic disturbance (ED, postural hypotension, atonic bladder, constipation, sweating), cerebellar signs
Summary of progressive supranuclear palsy?
Features - postural instability and falls, impairment of vertical gaze (pt may complain of difficulty reading or descending stairs as down gaze is worse than up gaze), Parkinsonism, cognitive impairment (primary frontal lobe dysfunction)
Management - poor response to L-dopa
Summary of Lewy body dementia?
Accounts for 20% of cases
Alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas.
Features - progressive cognitive impairment, typically before Parkinsonism. Fluctuating cognition. Early impairments in attention and and executive function rather than just memory loss. Visual hallucinations.
Diagnosis - clinical. SPECT.
Management - acetylcholinesterase inhibitors (e.g. donepezil, rivastigmine) and memantine. Neuroleptics should be avoided in Lewy body dementia as patients are extremely sensitive and may develop irreversible parkinsonism.
Summary of corticobasal degeneration?
Between 50 - 70
Increasing numbers of brain cells becoming damaged or dying - due to build up of tau
Features - ‘useless hand’, muscle stiffness, tremors, dystonia, cerebellar Sx, slow and slurred speech, Sx of dementia, difficulty swallowing - usually one limb and then spreads to the rest of the body
Conditions causing tremor?
Parkinsonism - resting, pill rolling
Essential tremor - postural tremor (worse if arms outstretched, improves with alcohol and rest, titubation (head tremor), strong FHx)
Anxiety
Thyrotoxicosis
Hepatic encephalopathy - Hx of CLD
CO2 retention - Hx of COPD
Cerebellar disease - intention tremor, past-pointing, nystagmus
Drug withdrawal - alcohol, opiates
Management of essential tremor?
Propranolol first line
Primidone
Pathway of facial nerve?
Exits brain stem at cerebellopontine angle. Passes through temporal bone and parotid gland/
Divides into 5 branches - temporal, zygomatic, buccal, marginal mandibular and cervical
Function of facial nerve?
Motor - supples muscles of facial expression, stapedius in inner ear, and posterior digastric, stylohyoid and platysma muscles in the neck.
Sensory - carries taste from anterior 2/3 of tongue
Parasympathetic - supply to the submandibular and sublingual salivary glands and the lacrimal gland (stimulating tear production).
Summary of Bell’s palsy?
acute, unilateral, idiopathic, facial nerve paralysis
aetiology unknown - role of HSV has been investigated
peak incidence is 20-40 years. common in pregnant women
Features of Bells palsy?
LMNL facial nerve palsy - forehead affected, no sparing
post-auricular pain - may precede paralysis
altered taste
dry eyes
hyperacusis
Management of Bells palsy?
oral prednisolone within 72 hours of onset of Bell’s palsy - 50mg for 10 days
debate about use of anti-virals
eye care - artificial tears, eye lubricants, tape closed at night
f the paralysis shows no sign of improvement after 3 weeks, refer urgently to ENT
a referral to plastic surgery may be appropriate for patients with more long-standing weakness e.g. several months
Summary of Ramsay-Hunt syndrome?
caused by the varicella zoster virus (VZV)
unilateral lower motor neurone facial nerve palsy
painful and tender vesicular rash in the ear canal, pinna and around the ear on the affected side. This rash can extend to the anterior 2/3 of the tongue and hard palate.
Treatment should ideally be initiated within 72 hours. Treatment is with:
Prednisolone
Aciclovir
Also - lubricating eye drops
Causes of LMN facial nerve palsy?
Bells
Ramsay Hunt
Infection: Otitis media, Malignant otitis externa, HIV, Lyme’s disease
Systemic disease: Diabetes, Sarcoidosis, Leukaemia, Multiple sclerosis, Guillain–Barré syndrome
Tumours: Acoustic neuroma, Parotid tumours, Cholesteatomas
Trauma: Direct nerve trauma, Damage during surgery, Base of skull fractures
Summary of trigeminal neuralgia?
trigeminal nerve - 3 branches - ophthalmic, maxillary, mandibular
syndrome characterised by severe unilateral pain. Majority idiopathic, but can be due to compression of trigeminal roots by tumours or vascular problems
Features
unilateral disorder - brief electric shock like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve
pain evoked by light touch - washing, shaving, smoking, talking and brushing teeth, and frequently spontaenously
trigger areas - nasolabial fold or chin
pains usually remit for variable periods
Red flags - sensory changes, deafness or other ear problems, history of skin or oral lesions that could spread perineurally, pain only in ophthalmic division of trigeminal nerve or bilaterally, optic neuritis, FHx of MS, age of onset before 40
Tx - carbamazepine, failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology, surgery to decompress or intentionally damage the trigeminal nerve is an option.
What is Guillain-Barre syndrome?
acute paralytic polyneuropathy that affects peripheral nervous system
immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni, also CMV, EBV)
cross-reaction of antibodies with gangliosides in the peripheral nervous system - anti-ganglioside antibody. Due to molecular mimicry
What is Miller-Fisher syndrome?
variant of Guillain-Barre syndrome
associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first
usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
anti-GQ1b antibodies are present in 90% of cases
Features of Guillain-Barre syndrome?
Symptoms usually start within 4 weeks of the preceding infection. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.
65% of pt’s have back/leg pain in the initial stages of the illness
progressive, symmetrical weakness of all limbs - classically ascending (legs first)
reflexes are reduced or absent
sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
Hx of gastroenteritis
respiratory muscle weakness
CN involvement - diplopia, bilateral facial nerve palsy, oropharyngeal weakness is common
autonomic involvement - urinary retention, diarrhoea
less common - papilloedema (though to be secondary to reduced CSF resorption
Ix of Guillain Barre syndrome?
lumbar puncture - rise in protein with normal WBC count
nerve conduction studies - decreased motor nerve conduction velocity (due to demyelination)
Management of Guillain Barre syndrome?
IV immunoglobulins
Plasma exchange (alternative to IV IG)
Supportive care
VTE prophylaxis (pulmonary embolism is a leading cause of death)
In severe cases with respiratory failure patients may need intubation, ventilation and admission to the intensive care unit.
Prognosis of Guillain Barre syndrome?
80% will fully recover
15% will be left with some neurological disability
5% will die
What is motor neurone disease?
is a progressive, ultimately fatal condition where the motor neurones stop functioning.
There is no effect on the sensory neurones and patients should not experience any sensory symptoms.
Pathophysiology of motor neurone disease?
progressive degeneration of both upper and lower motor neurones. The sensory neurones are spared.
There is a genetic component and many genes have been linked with an increased risk of developing the condition.
increased risk with smoking, exposure to heavy metals and certain pesticides.
Symptoms of MND?
typical patient is a late middle aged (e.g. 60) man, possibly with an affected relative
insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech.
often first noticed in the upper limbs
increased fatigue when exercising
may complain of clumsiness, dropping things more often or tripping over.
They can develop slurred speech (dysarthria).
Doesn’t affect external ocular muscles
No cerebellar signs
Abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature
Signs of motor neurone disorder?
Signs of LMNL - muscle wasting, reduced tone, fasciculations, reduced reflexes
Signs of UMNL - increased tone or spasticity, brisk reflexes, upping plantar responses
Diagnosis of MND?
Based on clinical presentation and excluding other conditions
Should be made by specialist
Unfortunately, the diagnosis is often delayed, which causes considerable anxiety and stress.
Normal motor conduction - show normal motor conduction and can help exclude a neuropathy. EMG - shows a reduced number of action potentials with increased amplitude.
MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy
Management of MND?
No effect treatments for heating or reversing the progression of the disease
MDT approach
Riluzole slow progression of the disease and extend survival by a few months in ALS - initiated by a specialist
Edaravone - used in USA
NIV used at home to support breathing at night improves survival
PEG for nutrition
End of life care planning
Types of MND?
Amyotrophic lateral sclerosis (50% of patients)
typically LMN signs in arms and UMN signs in legs
in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase
Primary lateral sclerosis
UMN signs only
Progressive muscular atrophy
LMN signs only
affects distal muscles before proximal
carries best prognosis
Progressive bulbar palsy
palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
carries worst prognosis
What is multiple sclerosis?
chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system
Epidemiology of multiple sclerosis?
F>M 3:1
aged 20-40
more common at higher latitudes
Subtypes of multiple sclerosis?
Relapsing-remitting disease
most common form, accounts for around 85% of patients
acute attacks (e.g. last 1-2 months) followed by periods of remission
Secondary progressive disease
describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses
around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis
gait and bladder disorders are generally seen
Primary progressive disease
accounts for 10% of patients
progressive deterioration from onset
more common in older people
Features of multiple sclerosis?
non-specific features - lethargy
visual - optic neuritis, optic atrophy, Uhthoff’s phenomenon (worsening of vision following rise in body temperature), internuclear ophthalmoplegia
Sensory - pins/needles, numbness, trigemintal neuralgia, Lhermitte’s syndrome (paraesthesiae in limbs on neck flexion)
Motor - spastic weakness (commonly seen in legs)
Cerebellar - ataxia (seen in acute relapse), tremor
Others
urinary incontinence
sexual dysfunction
intellectual deterioration
Diagnosis of multiple sclerosis?
made by a neurologist based on the clinical picture and symptoms suggesting lesions that change location over time
determination of “lesions disseminated in space and time” - multiple parts of the CNS and over course of time
Ix of multiple sclerosis?
MRI
high signal T2 lesions
periventricular plaques
Dawson fingers: often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum
CSF
oligoclonal bands (and not in serum)
increased intrathecal synthesis of IgG
Visual evoked potentials
delayed, but well preserved waveform
Management of acute relapse of multiple sclerosis?
High-dose steroids (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten the length of an acute relapse.
It should be noted that steroids shorten the duration of a relapse and do not alter the degree of recovery (i.e. whether a patient returns to baseline function)
Management of multiple sclerosis?
MDT - neurologist, specialist nurses, PT, OT, pt education
DMARDs - reduce risk of relapses
indication -
relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
options -
natalizumab
ocrelizumab
fingolimod
beta-interferon
glatiramer acetate
fatigue - exclude (anaemia, DM, depression) - trial of amantadine, mindfulness, CBT
spasticity - baclofen and gabapentin are first line. PT, cannabis, botox
bladder dysfunction - may take the form of urgency, incontinence, overflow etc, US to assess bladder emptying, if significant residual volume → intermittent self-catheterisation
if no significant residual volume → anticholinergics may improve urinary frequency
oscillopscia - visual fields oscillate - gabapentin
What is Erb-Duchenne paralysis?
Damage to C5/6 roots
Winged scapula
May be caused by breech presentation
What is Klumpke’s paralysis?
Damage to T1
Loss of intrinsic hand muscles
Due to traction
Criteria for brain stem death testing?
Deep coma of known aetiology
Reversible causes excluded
No sedation
Normal electrolytes
How to test for brain stem death?
Fixed pupils which do not respond to sharp changes in the intensity of incident light
No corneal reflex
Absent oculo-vestibular reflexes - no eye movements following the slow injection of at least 50ml of ice-cold water into each ear in turn (the caloric test)
No response to supraorbital pressure
No cough reflex to bronchial stimulation or gagging response to pharyngeal stimulation
No observed respiratory effort in response to disconnection of the ventilator for long enough (typically 5 minutes) to ensure elevation of the arterial partial pressure of carbon dioxide to at least 6.0 kPa (6.5 kPa in patients with chronic carbon dioxide retention).
Adequate oxygenation is ensured by pre-oxygenation and diffusion oxygenation during the disconnection (so the brain stem respiratory centre is not challenged by the ultimate, anoxic, drive stimulus)
Done by 2 appropriately experienced Drs on 2 separate occasions - both experienced in brain stem death + 5 years post-grad experience + one must be consultant
Predictive factors in SAH?
conscious level on admission
age
amount of blood on CT head
Complications of SAH?
re-bleeding, hydrocephalus, vasospasm, hyponatraemia, seizures
Tx of SAH?
supportive (bed rest, analgesia, VTE prophylaxis, discontinuation of antithrombotics)
nimodipine for vasospasm
intracranial aneurysms - risk of rebleeding so treated with coil or clipping
Tx of hydrocephalus?
external ventricular drain - right lateral ventricle
Ventriculoperitoneal shunt - long-term CSF diversion technique drains from ventricles to peritoneum
Obstructive - remove pathology
Ix for hydrocephalus?
CT, MRI, LP
Causes of non-obstructive hydrocephalus?
- imbalance of CSF production absorption:
increased CSF production (choroid plexus tumour) or more commonly a failure of reabsorption at the arachnoid granulations (e.g. meningitis or post-haemorrhagic).
Causes of obstructive hydrocephalus?
tumour, acute haemorrhage, developmental abnormalities
Types of head injuries?
diffuse axonal injury occurs as a result of mechanical shearing following deceleration, causing disruption and tearing of axons
intra-cranial haematomas can be extradural, subdural or intracerebral, while contusions may occur adjacent to (coup) or contralateral (contre-coup) to the side of impact
Subdural bleed blood vessel?
convex - middle meningeal artery
Extra dural bleed blood vessel?
bridging veins
concave
Bells palsy - ipsilateral or contralateral?
ipsilateral
How to differentiate between AICA and PICA strokes?
AICA strokes can be differentiated from PICA strokes as AICA causes ipsilateral facial paralysis and deafness, but PICA does not.
Summary of T1 nerve root lesion?
wasting of intrinsic muscles of hand
weakness of finger abduction and adduction and thumb adduction
finger flexion normal
altered touch along ulnar border of forearm
reflex normal
What to give for SCC?
dexamethasone
CSF findings of bacterial meningitis?
High pressure, raised protein, excess neutrophils
When to start anti-epileptic Tx?
anti-epileptic treatment should not be started after the first episode of seizure, unless in the following circumstances:
* the presence of a neurological deficit
* structural abnormality on brain imaging
* unequivocal epileptic activity on the EEG
* the patient, family or carers consider the risk of having a further seizure is unacceptable
Ankle reflex?
S1-S2
Knee reflex?
L3-L4
Biceps reflex
C5-C6
Triceps reflex?
C7-C8
How does tetanus present?
Trismus - lock jaw
Facial spasms - grimace expression, forced grinning
Back pain
Increased tone
Dysphagia
Spasms
Temperatures
Injecting drug habits is a RF for tetanus infection
How does botulism present?
Flaccid paralysis (not spasms)
Usually symmetrical and descending - muscles of neck, shoulders, upper extremities first
CN6 palsy?
Defective abduction
Horizontal diplopia (could be first sign of brain mets)
Lateral rectus
