Neuro 2 Flashcards
What are the risk factors of MS?
- white
- female:male 2:1 at least
- EBV
- late 20s (mean age of onset 30)
- FH (2% first degree relative, 30% both parents)
- lives in north
- smoking
What are the types of MS?
Increasing in disability:
- Relapsing and remitting (de and remyelination)
- Primary progressive MS (no remission)
- Secondary progressive MS (after stepwise)
- Progressive relapsing and remitting
When progressive you have axonal damage
What are the features of optic neuritis?
- Pain on eye movement
- rapid decrease of central vision
- decrease in colour vision
- centrocaecal scotoma
- normal disc / mild swelling
- Improves w/in 3 wks of onset
What are poor prognostic signs of MS?
- older males
- motor signs at onset
- many relapses early on
- many MRI lesions
- axonal loss
If someone has a clinical isolated syndrome what’s the chance of progressing to MS?
- MRI scan clean then 20% chance of progression to MS in next 10-15 years
- 2-3 lesions on MRI then 60-80% chance of progression to MS in next 10-15 years
If MRI -; OCB + = 24% vs. 4% (OCB-)
OCB= oligoclonal bands
How do you diagnose MS?
It requires lesions disseminated in time and space:
- clinical history
- contrast MRI (IV gadolinium leaks out if <30 days) so you would want to see contrast enhancing and non contrast enhancing lesions
- oligoclonal IgG bands in CSF
What are the signs and symptoms of MS?
Sensory
- dysaesthesia
- pins and needles
- vibration sense decreased
- trigeminal neuralgia
Motor
- spastic weakness
- myelitis
Sexual/GU
- erectile dysfunction
- anorgasmia
- urine retention
- incontinence
GI
- swallowing disorders
- constipation
Eye
- diplopia
- hemianopia
- optic neuritis
- uhthoffs phenomenon (dec vision with heat, and exercise)
- bilateral inter-nuclear ophthlamoplegia
- pupil defects
Cerebellum
- ataxia
- intention tremor
- scanning (monotonous) speech
- falls
Cognitive
- decline
- amnesia
- mood changes
DDx for MS
ADEM (Acute disseminated encephalomyelitis)
- confusion
- seizures
- lesions resolve and don’t recur
Neuromyelitis opticia- autoimmune disease against aquaporin 4- (MOG, AQP4 assay)
How do you treat MS?
In relapse:
- high dose methylprednisolone 500mg oral, 2 x daily for 5 days with gastro protection such as omeprazole
- if severe give methylpred 1g IV daily for 3-5 days within 14 days of onset
- consider Plasma Exchange
Long term:
- encourage a stress free life
- vitamin D
RRMS: Attack Prevention 1 st Line: Injectables • Β-IFN (decreases relapses by 30%) • Glatiramer Acetate (copaxone) Orals Teriflunomide (Aubagio) Fumarate (Tecfidera)
Attack Prevention 2nd Line • Fingolimod (Gilenya) • Natalizumab (Tysabri) • Alemtuzumab (Lemtrada)** •Cladribine - monoclonal antibodies against T cells (alemtuzumab, natalizumab), decreases relapses by 68% - azothioprine
Secondary progressive MS:
- glatiramer, mitoxantrone (doxorubicin analogue)
How do you define an MS relapse?
Rapidly progressive neurological dysfunction
days (hours, days)
• >24 h <1month
• First event – CIS Clinically isolated syndrome (monofocal/multifocal)
- more than 30 days after previous
What are the typical features of transverse myelitis?
- bilateral sensory/motor symptoms (asymmetric -> incomplete cord) - defined sensory level - sphincter disturbance - Lhermitte’s / paroxysmal tonic spasms - progression over 4h to 21 days
What are the atypical features of transverse myelitis?
- complete transverse myelitis
- cauda equina syndrome
- areflexia
- progressive symmetric spastic paraparesis
What are the features of MS affecting the cerebellum or brain stem?
Typical - internuclear ophthalmoplegia - ataxia and nystagmus - multifocal signs (facial sensory loss + vertigo) - paroxysmal phenomena (> 24 hrs)
Atypical
- complete external ophthalmoplegia
- Isolated trigeminal neuralgia
- fluctuating ocular/bulbar symptoms
- parkinsonism / movement d/o
What blood tests would you do if you suspect MS?
- full blood count
- inflammatory markers for example erythrocyte sedimentation rate, C-reactive protein
- liver function tests
- renal function tests
- calcium
- glucose
- thyroid function tests
- vitamin B12
- HIV serology.
How do you treat a first presentation of optic neuritis?
Do MRI brain
- normal -> check vision: worse than 20/40 consider MP and follow up with MRI at 2-4 wks, not normal consider MP and follow up at 3-6 months
- not normal -> treat with MP
What signs can be seen if you get a patient to open and close their fingers rapidly?
Decrease in amplitude: basal ganglia pathology
Decrease in rate: UMN/pyramidal
Decrease in rhythm: cerebellum
What’s the function of the rubrospinal tract?
Flexor movement of upper limbs
What’s the function of the lateral vestibulospinal tract
Extensors and posture
What’s the function of the reticulospinal tract?
Muscle tone and gait (dampens monosynaptic reflex arch so UMN lesions increase reflexes)
What’s the differential for a black out?
Syncope
Seizure
Non-epileptic attack
What are the features of syncope?
- Prolonged upright position
- Sweating prior to LOC
- Pre-syncopal symptoms
- Pallor
- Nausea
What are the features of a seizure?
- Cyanosis
- Tongue bite
- Post-ictal confusion
- LOC for >5 minutes
- Muscle pain
What are the types of syncope and how would you distinguish between them?
Postural
Reflex
Cardiac
With postural and reflex syncope the cause is autonomic (prodromal nausea and sweating). Patients have problems with BP regulation. No change in Life expectancy.
Cardiac No trigger/abrupt > 10%/yr risk of death ?FHx sudden death <35 yrs ?RF for IHD ?PMH of IHD
ECG:
>35yrs: LBBB, Q-waves
<35yrs: Delta, PR, QT, LVH
What are the rules about epilepsy and driving?
You must be fit free for 12 months before driving.
When coming off AEDs you must be fit free for six months after stopping
Can you drive after a black out?
If unprovoked seizure:
- you must be seizure free for 6 months
If syncope suspected (provocative, prodromal, postural):
- you can drive
If reflex vasovagal when sitting:
- you must be seizure free for 6 months
If likely cardiovascular syncope:
- syncope free for 6 months or 1 week if treated
What’s useful when considering if a seizure is epileptic or not?
NEA
- long duration
- asynchronous movements
- pelvic thrusting
- side to side body/head movements
- closed eyes
- ictal crying
- memory recall ok
Epileptic
- side tongue biting
- stertorous breathing
- postictal confusion
- tense face
- loss of bowel control
What are the comorbidities of epilepsy?
Substance misuse
Anxiety
Depression
What’s the prognosis of treated epilepsy?
Seizure control: 50-60% with first AED
Intractable: 10-20%
What’s the risk of seizure recurrence after AED withdrawal?
Risk of seizure recurrence:
2 yrs Sz free -> 20-40%
5 yrs Sz free -> 10-20%
What’s the risk of SUDEP?
1% if frequent GTCS
0.5% overall
What’s the risk of SUDEP?
- GTCS frequency
- Nocturnal seizures
What is status epilepticus and how do you treat it?
If seizure lasts more than 5 minutes.
Diazepam 10−20 mg rectally, repeated once 15 minutes
or Midazolam 10 mg buccally
Early status
- Lorazepam (i.v.) 2- 4 mg bolus, repeated once after 10−20 minutes
- Give usual AED medication!!!
Established status
- Phenytoin 15-20 mg/kg
- and/or Phenobarbitone 10-15 mg/kg
Refractory status
General anaesthesia:
- Propofol (1-2mg/kg bolus, then 2-10mg/kg/hour)
- Midazolam (0.1-0.2mg/kg bolus, then 0.05-0.5mg/kg/hour)
- Thiopentone (3-5mg/kg bolus, then 3-5mg/kg/hour) fat stores saturation
What’s the diagnosis?
A patient with progressive, painless weakness and wasting with mixed upper and lower motor neuron signs
motor neuron disease (amyotrophic lateral sclerosis)
What are the features of spinal onset ALS?
- 6 months of progressive weakness of upper limb
- Fasciculations of muscle in affected limb
- Cramps and spasms
- Weight loss +
- Extensor plantars
What are the differentials for weakness and wasting of one upper limb?
- ALS
- branchial neuritis
- conduction block neuropathy
- structural cervical spine disease
What are the features of a conduction block neuropathy?
- Rare
- Usually onset before 60 years
- median 6 years (1-20) to diagnosis
- CB not always demonstrated
- Weakness in non-wasted muscles
What are the features of bulbar onset ALS?
- 9 months of progressive difficulty in speaking and swallowing
- Weight loss +++
- Inappropriate laughing and crying
- Breathless when lying flat
Which sites are the first affected in ALS?
- Upper limb 30%
- Lower limb 35%
- Bulbar 30%
- Respiratory 1-2%
- Trunk 1-2%
What are the features of ALS?
- LMN: weakness, wasting and fasciculation
- UMN: stiffness (spasticity), brisk reflexes
What’s the prognosis of MND?
50% dead within 30 months
80% dead within 5 years
What’s seen in MND biopsies?
Ubiquinated inclusions (e,g TDP-43)
Skein-like inclusion
Bunina Bodies
Dystrophic neurites
What’s the inheritance of MND?
90% not inherited
5-10% autosomal dominant
Mutations in C9orf72 explain 40% of cases of familial ALS-FTD (frontotemporal dementia)
What are the treatments for MND?
- Emotionality (amytriptiline)
- Cramps (baclofen, physio)
- Secretions (hyoscine, BoTox)
- Sleep disturbance (NIV)
- Depression and anxiety (supportive care, antidepressants)
What’s the name for a head tremor?
titubation
What are the features of an essential tremor?
- postural tremor: worse if arms outstretched
- improved by alcohol and rest
- most common cause of titubation (head tremor)
What’s the management of an essential tremor?
- propranolol is first-line
- primidone is sometimes used
Which drugs can cause ataxia?
- carbamazepine
- phenytoin
- sodium valproate
What are the features of NF1?
- Café-au-lait spots (>= 6, 15 mm in diameter)
- Axillary/groin freckles
- Peripheral neurofibromas
- Iris hamartomas (Lisch nodules) in > 90%
- Scoliosis
- Pheochromocytomas
What are the features of NF2?
- Bilateral vestibular schwannomas
- Multiple intracranial schwannomas, mengiomas and ependymomas
What is NF1 also known as? Which chromosome is the gene on and how many are affected?
Recklinghausen’s syndrome
chromosome 17
1 in 4,000
Which chromosome is affected by NF2? What’s the incidence?
Chromosome 22 and affects around 1 in 100,000
What are the features of tuberous sclerosis?
Cutaneous features
- 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 but more common in NF1
Neurological features
- developmental delay
- epilepsy (infantile spasms or partial)
- intellectual impairment
Also
- 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
What type of drug is memantine?
NMDA receptor antagonist
What’s the mechanism of phenytoin?
binds to sodium channels increasing their refractory period
What is the treatment of choice for restless legs syndrome?
- simple measures: walking, stretching, massaging affected limbs
- treat any iron deficiency
- dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
- benzodiazepines
- gabapentin
What are the 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
What is the treatment of choice for neuropathic pain (not diabetic)?
Amitriptyline or duloxetine or gabapentin or pregabalin
What’s Todd’s paresis and which patients are most likely to experience it?
Patients with focal seizures may experience post-ictal weakness (Todd’s paresis)
What are the features of temporal lobe epilepsy?
Hallucinations (auditory/gustatory/olfactory)
Epigastric rising/Emotional
Automatisms (lip smacking/grabbing)
Deja vu/Dysphasia post-ictal
What are the features of frontal lobe epilepsy?
Head/leg movements , posturing, post-ictal weakness
What are the features of parietal lobe epilepsy?
Paraesthesia
What are the features of occipital lobe epilepsy?
Floaters/flashes
How do you treat cluster headaches?
- acute: 100% oxygen (80% response rate within 15 minutes), subcutaneous triptan (75% response rate within 15 minutes)
- prophylaxis: verapamil is the drug of choice. There is also some evidence to support a tapering dose of prednisolone
- NICE recommend seeking specialist advice from a neurologist if a patient develops cluster headaches with respect to neuroimaging
What drugs are used to treat lewy body dementia?
Both acetylcholinesterase inhibitors (e.g. donepezil, rivastigmine) and memantine can be used as they are in Alzheimer’s.
In LEMS what auto antibodies might you see?
Voltage-gated calcium-channel antibodies
What is the grading scale for muscle strength?
Grade 0 No muscle movement
Grade 1 Trace of contraction
Grade 2 Movement at the joint with gravity eliminated
Grade 3 Movement against gravity, but not against added resistance
Grade 4 Movement against an external resistance with reduced strength
Grade 5 Normal strength
What are the conditions that make up peripheral neuropathy?
Predominately motor loss
- Guillain-Barre syndrome
- porphyria
- lead poisoning
- hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- diphtheria
Predominately sensory loss
- diabetes
- uraemia
- leprosy
- alcoholism
- vitamin B12 deficiency
- amyloidosis
What’s the pathogenesis of Guillain Barre syndrome?
- cross reaction of antibodies with gangliosides in the peripheral nervous system
- correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated
- anti-GM1 antibodies in 25% of patients
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
How do you manage normal pressure hydrocephalus?
- ventriculoperitoneal shunting
- around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages
What is the most common long-term complication of meningitis?
Sensorineural hearing loss
- other neurological: epilepsy, paralysis
- infective: sepsis, intracerebral abscess
- pressure: brain herniation, hydrocephalus
Most common adult cause (mortality)
- meningococcus (10%)
- pneumococcus (25%)
What are the risk factors for IIH?
- obesity
- female sex
- pregnancy
- drugs*: oral contraceptive pill, steroids, tetracycline, vitamin A, lithium
*if intracranial hypertension is thought to occur secondary to a known causes (e.g. Medication) then it is of course not idiopathic
How do you manage IIH?
- weight loss
- diuretics e.g. acetazolamide
- topiramate is also used, and has the added benefit of causing weight loss in most patients
- repeated lumbar puncture
- surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
Loss of corneal reflex with with dizziness and right-sided hearing loss. Diagnosis and investigation of choice?
Acoustic neuroma, MRI of the cerebellopontine angle
What is the medical management in an acute myasthenic crisis?
Plasmapheresis and intravenous immunoglobulin
What is the treatment for neuroleptic malignant syndrome?
Dantrolene and lorazepam
What investigations are done for 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 anymore due to the risk of cardiac arrhythmia
How do you manage MG?
- long-acting anticholinesterase inhibitors e.g. pyridostigmine
- immunosuppression: prednisolone initially
- thymectomy
Is a CT or MRI scan more sensitive for posterior fossa pathology?
MRI
Which of the following conditions should be included in your differential diagnosis of vertigo?
- Labyrinthitis
- Vestibular neuronitis
- BPPV
- lateral medullary syndrome
In what proportion of cases is CT negative for SAH?
7%
Once you have a diagnosis of SAH what do you do?
- CT intracranial angiogram (to identify a vascular lesion e.g. aneurysm or AVM)
- +/- digital subtraction angiogram (catheter angiogram)
How do you treat a SAH?
- The treatment in spontaneous SAH is in accordance with the causative pathology
- Most intracranial aneurysms are now treated with a coil by interventional neuroradiologists, but a minority require a craniotomy and clipping by a neurosurgeon
- Until the aneurysm is treated, the patient should be kept on strict bed rest, well controlled blood pressure and should avoid straining in order to prevent a re-bleed of the aneurysm
- Vasospasm is prevented using a 21-day course of nimodipine (a calcium channel inhibitor targeting the brain vasculature) and treated with hypervolaemia, induced-hypertension and haemodilution**
- Hydrocephalus is temporarily treated with an external ventricular drain (CSF diverted into a bag at the bedside) or, if required, a long-term ventriculo-peritoneal shunt
What are the complicstions of aneurysmal SAH?
- Re-bleeding (in around 30%)
- Vasospasm (also termed delayed cerebral ischaemia), typically 7-14 days after onset
- Hyponatraemia (most typically due to syndrome inappropriate anti-diuretic hormone (SIADH))
- Seizures
- Hydrocephalus
- Death
What are the predictive factors in SAH?
- conscious level on admission
- age
- amount of blood visible on CT head
What’s the cushings reflex?
- hypertension
- bradycardia
What’s the typical finding of an infant with hydrocephalus?
- increasing head circumference
- bulging fontanelle
- sunsetting of eyes caused by pressure on the tectal plate (impaired upward gaze)
- dilated scalp veins
- bradycardia
- seizuers
- coma
How would you investigate hydrocephalus?
- CT head is used as a first line imaging investigation since it is fast and shows adequate resolution of the brain and ventricles
- MRI may be used to investigate hydrocephalus in more detail, particularly if there is a suspected underlying lesion
- Lumbar puncture* is both diagnostic and therapeutic since it allows you to sample CSF, measure the opening pressure, but also to drain CSF to reduce the pressure
*Lumbar puncture must not be used in obstructive hydrocephalus since the difference of cranial and spinal pressures induced by the drainage of CSF will cause brain herniation.
How do you treat hydrocephalus?
- An external ventricular drain (EVD) is used in acute, severe hydrocephalus and is typically inserted into the right lateral ventricle and drains into a bag at the bedside
- A ventriculoperitoneal shunt (VPS) is a long-term CSF diversion technique that drains CSF from the ventricles to the peritoneum
- In obstructive hydrocephalus, the treatment may involve surgically treating the obstructing pathology
What does EVD stand for?
External ventricular drain
What does VPS stand for?
Ventriculoperitoneal shunt
What’s a subfalcine herniation?
When the cingulate gyrus herniates under the falx cerebri
What’s the term to describe the brain herniating through an open skull fracture?
Transcalvarial herniation
How would sheehan’s syndrome look on CT?
Acute: CT scan findings in case of acute presentation of Sheehan’s syndrome may show non-hemorrhagic pituitary gland enlargement
Chronic: may show an empty sella or decreased sellar volume.
Give examples of neuropathic pain
- diabetic neuropathy
- post-herpetic neuralgia
- trigeminal neuralgia
- prolapsed intervertebral disc
What are the features of multiple system atrophy?
- atonic bladder
- postural hypotension
- erectile dysfunction
- ataxia
- slurred speech
- parkinsons symptoms
Which things would indicate MSA rather than Parkinson’s disease?
- rapid progression of symptoms
- they’ve experienced falls early on
- don’t respond to levodopa
- speech severely affected
- breath nosily
Give examples of parkinson plus syndromes
Multiple system atrophy (MSA)
Progressive supranuclear palsy (PSP)
Corticobasal degeneration (CBD)
Which features would suggest Parkinson-plus syndromes rather than idiopathic PD?
- a lack of or irregular resting tremor
- a reduced response to dopaminergic drugs
- early-onset postural instability
- increased rigidity in axial muscles
- dysautonomia
- alien limb syndrome
- supranuclear gaze palsy
- apraxia
- involvement of the cerebellum
What are the features of PSP?
- Supranuclear ophthalmoplegia
- Neck dystonia
- Parkinsonism
- Pseudobulbar palsy
- Behavioral and cognitive impairment
- Imbalance and walking difficulty
- Frequent falls
What are the causes of a bilateral facial nerve palsy?
- sarcoidosis
- Guillain-Barre syndrome
- Lyme disease
- bilateral acoustic neuromas (as in neurofibromatosis type 2)
- as Bell’s palsy is relatively common it accounts for up to 25% of cases f bilateral palsy, but this represents only 1% of total Bell’s palsy cases
What are the causes of a unilateral facial nerve palsy?
In addition to all the things that cause bilateral palsy:
- Bell’s palsy
- Ramsay-Hunt syndrome (due to herpes zoster)
- acoustic neuroma
- parotid tumours
- HIV
- multiple sclerosis*
- diabetes mellitus
- stroke (upper motor neuron)
What investigations would you do after a stroke?
- CT head
- carotid dopplers
- lipid profile
- fasting glucose
What artery supplies the thalamus?
Posterior cerebral, thalamogeniculate
Middle cerebral, lenticulostriate
Anterior cerebral, penetrating arteries
Anterior choroidal
Which scale is used to assess the degree of dependence following a stroke?
Modified Rankin scale
Which scale determines if you need thrombolysis?
National Institute of health stroke scale
What does the basilar artery supply?
Pons
Inferior and superior aspect of the cerebellum
