NEURO Flashcards
What is neurodegeneration
Progressive loss of function of neurone due to cell death or degeneration
Give the definition of dementia
Decline in memory, intellect or personality which is severe enough to interfere with ADL
How is dementia diagnosed
- Predominantly a clinical diagnosis
Investigations in dementia
ACE III- attention, memory, fluency, language, vitamin
Dementia screen- bloods, urinalysis, psychometric testing, MRI brain
Differentials for dementia
Old age, depression, physical health problems eg DM, hypothyroid, vit def
Treatment for dementia
- Acetylcholinesterase inhibitors eg donepezil, rivastigmine
- NMDA antagonist eg memantine
- Rf reduction in vascular dementia is important
What is pseudo dementia
- Cognitive impairments secondary to mental illness eg depression/anxiety
Pseudo will say don’e know where as normal would make something up- confabulation
Clinical features of Alzheimer’s
Episodic memory
Visuospatial problems
Getting lost in familiar places
4 As: aphasia, anomia(deficit of expressive language), acalculia, ADLs are a struggle
What happens in the late stages of Alzheimer’s
- Frank dementia
- Bradykinesia
- Psychosis
- Seizures
- Incontinence
- Mutism
What is the prognosis for Alzheimer’s
Incurable, long progressive course
Pathophysiology of Alzheimer’s
- B-amyloid plaques, accumulation of amyloid in the hippocampus, enterhinal cortex and amygdala
- Neurofibrillary tangles- intercellular accumulation of tau
- Cortical atrophy - mostly in frontal and temporal lobes
Investigations in Alzheimer’s
- Rule out other causes
- Neuropsychological testing: Addenbrookes (differentiates between front-temporal dementia and Alzheimer’s)
- MMSE
- MOCA
- Functional imaging- reduced blood flow and glucose metabolism in tempo-parietal and hippocampus, cortical atrophy and enlarged ventricles
Management of Alzheimer’s
No cure
- AChE Inhibitors eg donepezil, rivastigmine, galantamine- inhibit Ach degradation at the synaptic cleft
- NMDA antagonists- behavioural
- Antidepressants/psychotics
- Cognitive training
- Emotional and social stimulation
- Exercise programmes
- Caregiver training programmes
Clinical features of Lewy body dementia
- Visual hallucinations
- Fluctuation
- Parkinsonism- bradykinesia, tremor, rigidty
- Sleep disorders
- Psychosis
Investigations for Lewy body dementia
Psychometric testing
- deficits in attention
- Executive functional decline
- Fluctuation of cognitive performance
- Episodic memory preserved
Pathophysiology of Lewy body dementia
- Pallor of substantia nigra
- Lewy bodies (alpha- synuclein)
Treatment of Lewy body dementia
- Anti-parkinsons tx can worsen psychosis
- avoid neuroleptics- causes tardive dyskinesia > hypersensitisation to dopamine in the nigrostriatal pathway
Risk factors for Vascular dementia
- Smoking
- Overweight/obese
- Sedentary lifestyle
- Poor diet
Types of Vascular Dementia
- Step wise decline: mini strokes; multi infarct
- Generalised decline: diffuse white matter disease
Clinical features of Vascular Dementia
- Sundowning
- Good and bad days
- Mood plays a bigger role
- Emotional incontinence
- Inappropriate laughter or tearfulness
- Good and bad days
- Focal neurology
Management of Vascular Dementia
- Non-Pharmacological interventions
- AChE and NMDA inhibitors are NOT recommended
- Manage emotional symptoms
- Modifiable and preventable- lifestyle
What is Fronto-temporal linked to?
MND
- tends to affect younger people
Clinical picture of fronto-temporal dementia
- Behaviour variant (apathic, disinhibition, lack of insight, emotional lability)
- Semantic (can speak, receptive aphasia)
- Progressive non-fluent aphasia - expressive aphasia)
What investigations would you do for fronto-temporal dementia and what would you expect to see?
- MRI > fronto-temporal atrophy
- Genetic testing
- Assess for MND
Treatment for fronto-temporal dementia
- SSRIs
- Holistic management
What is the pathophysiology of MND?
- Progressive loss of motor neurones and dismantling of the motor neurone system
- Anterior horn most commonly affected
Clinical features of MND
- Dysfunction/weakness in one part of the body > spreads gradually
- Resp failure > failure of muscles ventilation
- Non sensory S&S or eyes or bladder involvement
UMN signs of MND
- hypertonia
- hyperreflexia
- up going plantars
- weakness
LMN signs of MND
- weakness
- wasting
- reduced tone
- reduced reflexes
- fasciculation’s
Lumbar signs of MND
- foot drop
- fasciculation’s (esp in thigh)
Bulbar signs of MND
- jaw jerk
- dysarthria
- swallowing
Prognosis of MND
- incurable: death av. 3 yrs
- weight loss= poor prognosis (due to hyper metabolic state)
Treatment of MND
consider-
- Nutritional management
- Non-invasive ventilation
- Counselling
Clinical features of Parkinson’s Disease
- Bradykinesia
- Cogwheel rigidity
- Resting tremor
- Postural instability
- Hyposmia
- Excessive saliva production
- Memory impairment
- Autonomic dysfunction
- Depression/anhedonia
- REM sleep disorders
- Non motor symptoms often precede motor signs
- Dementia (lewy body)
- Psychosis
- (can start presentation with constipation and GI symptoms or depression and REM first)
What are the neurological findings in PD
Loss of dopaminergic neurones in the substantia nigra. Decreased production of dopamine > suppressed movement
Presence of Lewy bodies
Clinical diagnosis of PD- history
- Unilateral onset
- Resting tremor
- Progressive
- Anosmia
- Excellent response to l-dopa and a response for 5+ yrs
- 10yr+ clinical course
- Visual hallucinations
Management of PD
- Levodopa (dopamine precursor)
- Carbidopa
- Avoid Tx until onset of disabling Sx
- Dopamine receptor agonists (bromocriptine, ropinirole, cabergoline) - THESE CAUSE IMPULSE BEHAVIOURS SO NEEED TO BE MONITORED
- Levodopa + dopa-decarboxylase inhibitor
- MAO-B i: inhibits dopamine breakdown
- Anti cholinergics: reduce tremor
- COMT inhibitros
Monitoring people with PD
- Always ask about impulsive things (dopamine agonists cause this)
What is Parkinsons plus
- Falls at presentation Multiple system atropy - Symmetrical in onset - Parkinsonism with autonomic(impotence/incontinence, postural hypotension, cerebellar signs) , corticospinal and cerebellar dysfunction - Poor repsosne to l-dopa - Rapid progression - absence of tremor - Eye involvement- progressive supra nuclear palsy
Progressive supranuclear palsy (Parkinsons plus)
- Symmetrical
- Parkinsonism
- Early falls
- Postural instability
- Vertical gaze palsy
- Trunk rigidity
Speech and swallow problems
Supranuclear gaze palsy (Parksinsons plus)
- Pt has difficulty with voluntary down gaze
- Poor response to dopamine
- ?PEG feeding due to pseudo bulbar palsy
- 5yrs to incapacity
Dementia with Lewy Bodies/ Parkinson’s Disease Dementia
- Progressive, degenerative dementia
- Cognitive impairment precede motor signs
- Visual hallucinations
- Fluctuating cogntive funciton excessive daytime somnolence
- Anterograde memory loss
- Delusions
What are some acute causes of headache
- infection: meningitis/encephalitis
- haemorrhage: SAH, extradural
- venous sinus thrombosis
- sinusitis
- low pressure headache: CSF leak
- acute glaucoma
What are some chronic causes of headache?
- tensions headache
- migraine
- cluster headache
- trigeminal headache
- raised ICP
- medication overuse headache
- GCA
Migraine symptoms
- Unilateral
- Pulsatile headaches- last up to 24 hours with nausea and photophobia
Causes of migraine
CHOCOLATE
- chocolate
- OCP
- caffiene
- alcohol
- travel
- exercise
Treatment of migraines
acute- NSAIDs, triptans
chronic- BB, amitriptyline topiramate
IF aura- cant use OCP
topiramate = teratogenic
Symptoms of cluster headache
- Unilateral pain around the eye
- Watery./bloodshot eye
- Clusters of headaches followed by pain free periods for months or years
- 15 mins> 3hrs
Management for cluster headaches
- Oxygen 12L/min 100% for 15 mins?
- Sumatriptan - acute
- verapamil - prevent
Symptoms of Trigeminal neuralgia
- Unilateral face pain in V3 distribution
very severe, electric shock pain for a few seconds
Treatment of Trigeminal Neuralgia
Carbamezapine (lamotrogine, phenytoin, gabapentin)
- microvascular decompression
Symtoms of a tension headache
- 30 mins> 7 days
- Photophobia OR phonophobia
- Bilateral, pressing, mild/moderate
NOT AGGRAVATED BY EXERCISE
Symptoms of GCA
Tired, achy jaw when eating and curtains drop down over eyes- amaurosis fugax
- shaving, change in temp
- Raised CRP
- temporal artery biopsy = neg
GCA management
- steroids > prednisolone 60mg
- PPI
- bisphosphonates
Important things about subarachnoid haemorrhage
- Thunderclap headache (max severity in seconds)
- star shape on CT, rupture of berry aneurysm (associated with PKD, coarctation of the aorta, Ehlers-Danlos)
- 12 hours after- lumbar puncture- xanthchroma
Treatment of SAH
- nimodipine (CCB)- prevent artery spasm
- drainage
- saline
Important things about a subdural haemorrhage
- head injury > vein rupture, latent period - crescent on CT (8-10 wks later clot breaks down and ICP raises)
- headache, drowsiness, confusion
- chronic- dark blood, acute white blood
- at risk- elderly, alcoholics, shaken babies
Tx- surgical removal
Important things about a extradural haemorrhage
trauma to temporal bone > bleed form middle meningeal artery
- egg shape (biconvex) - CT
- needs immediate surgical drainage
- lucid period
- GCS drops suddenly
- accurate symptoms
What to do in intracerebral haemorrhage
- Check warfarin INR, consider reversal with vit K
(too much warfarin INR^, vit K decreases INR) - if platelets are low, do platelet infusion
What percentage of strokes are ischaemic vs haemorrhagic
ischaemic 85%
haemorrhagic 15%
Risk factors for stroke
- HTN, smoking, DM, heart diease:valvular,ischaemic,AF, periperhal vascular disease, prev TIA, COCP, alcohol use, hyperlipidamia, clotting abnormalities
Features of a stroke
- LOC
- seizure activity
- new acute onset of: asymmetric facial, arm, leg weakness, speech disturbance, visual field defect
Treatment of an ischaemic stroke
Stabilise, 300mg aspirin
thrombolysis <4.5hr (alteplase)
Secondary prevention of stroke
- Modify risk factors: HTN, statin, smoking, weight loss
- Clopidogrel 75mg (aspirin or dipyridamole if not tolerated)
- Carotid endarterectomy
Define TIA
- Stroke symptoms lasting <24 hours
What is the risk score for TIA
ABCD2- risk of a stroke following a TIA
- Age >60yrs
- Blood pressure >140/90
- Clinical features of the event( unilateral weakness, speech disturbance without weakness, other)
- Duration of symptoms (over 60 mins, 10-59 mins, less than 10 mins)
- Diabetes
Management of TIA
- Conservative (reduce BP, cholesterol, control diabetes, diet and exercise, DVLA- can’t drive for 1 month)
- Clopidogrel 75mg or aspirin or dipyridamole if not tolerated
- Carotid endarectomy if >70% occlusion
S&S of anterior and middle cerebral artery infarct (total anterior circulation infarct)
- Unilateral and/or hemisensory loss
- Homonymous hemianopia
- Dysphasia/dysarthria
S&S of posterior circulation infarct
- Vestibulbobasilar arteries
- Cerebellar syndromes
- Loss of consciousness
- Homonymous hemianopia
S&S of lacunar infarct
- Perforating arteries (supplying internal capsule and basal ganglia)
- Pure sensory or pure motor or pure sensorimotor
- Ataxic hemiparesis
What is Weber’s syndrome?
- Ipsilateral third nerve palsy with contralateral weakness
What happens when there is a lesion on basilar artery
- Locked in syndrome
- Paralysed but higher thought functioning is normal
What is amaurosis fugax
- Retinal artery > embolism
- Curtain coming down over vision
Causes of seizures
- Epilepsy
- Febrile convulsions
- Alcohol withdrawal seizures
- Psychogenic non-epileptic seizures
- Brain injury
- Infection
- Trauma
- Metabolic disturbance
When to start treatment of seizures
- Anti-seizure treatment usually started after 2nd seizure
- Or after 1st if neurological deficit, structural abnormality, positive EEG, risk of a further seizures is unacceptable
Treatment of generalised seizure
- Sodium valproate (teratogenic)
- 2nd line- Lamotrigine, carbamazepine
Treatment of partial/focal seizures
- Carbamazepine
- 2nd: leveritacetam (keppra)
Treatment of absence seizures
- Sodium valproate or ethosuximide
- CARBAMAZEPINE CAN WORSEN ABSENCE SEIZURES
What medication should pregnant women avoid
- Sodium valproate > can cause neural tube defects
- Phenytoin > can cause cleft palate
- instead use lamotrigine, carbamazepine
Describe a complex partial seizure
- A disturbance of consciousness or awareness
Describe a generalised seizure
- Widespread seizure activity in both hemispheres
- can be absence, tonic clonic, myoclonic, atonic
Describe a focal/partial seizure
- Seizure activity is limited to a part of one hemisphere
- Focus= site in brain where seizures began
Describe an absence seizure
- Brief episode where patient appears blank/staring
Describe a tonic clonic seizure
- Muscle rigidity followed by repeated contraction and relaxation (shaking)
Describe a myoclonic seizure
- Brief, rapid, muscle jerks
Describe an atonic seizure
- Drop attacks
- Lose muscle tone and drop to ground
What causes multiple sclerosis
T cell mediated immune response, causing demyelinated plaques in the CNS
Visual symptoms of MS
- Optic neuritis (unilateral eye pain on movement and loss of central vision)
- Optic atrophy
- Uthoff’s- worsening of visual symptoms when body temp is high
Other symptoms of MS
- Numbness, tingling, trigeminal neuralgia
- Lhermittes syndrome: paraesthsia in limbs on neck flexion
- Spastic weakness
- Cerebellar signs (ataxia, diplopia, intention tremor)
- Urinary incontinence
- Sexual dysfunction
- Intellectual deterioration
Who does MS tend to affect
- Woman
- 20-40
- Temperate climate (low vit D)
What is needed for a diagnosis of MS
- 2 lesions disseminated by time and space - McDonald criteria
- MRI- demyelinated plaques
Types of MS
- Relapsing-remitting (85%)
- Primary progressive (10%)
- Secondary progressive
Management of MS
- Reduce stress
- Vit D
- Methylprednisolone for 5 days (shorten acute relapses)
- DMARDs (beta interferon- reduces number of relapses)(azathioprine)(glatiramer acetate) treats spasticity, bladder dysfunction
What is Huntington’s Disease
- Autosomal dominant (50% inheritance chance)- gene on chromosome 4
- Neurodegenerative
- Progressive
- Incurable
- Degeneration of cholinergic and GABA neurones in striatum of basal ganglia
Features of HD
- Personality changes (irritability, apathy, depression)
- Lack of coordination, unsteady gait
- Chorea (overshooting, unintentional movements)
- Dystonia/hypotonia
- Intention tremor
- Saccadic eye movement
- Fits
- Intellectual impairment
- Progressive dementia
Management of HD
- No curative treatment
- MDT approach (genetic counsellors, SALT, dietician, PT, OT, palliative care, psychologists)
- Symptom control
- Dopamine antagonists eg tetrabenazine for chorea
Prognosis of HD
Usually die within 20yrs after symptoms develop. Usually from pneumonia, heart disease, suicide, choking, malnutrition
What is Motor Neurone Disease
- Degenerative condition that affects motor neurones: motor cortex (UMN), cranial nerve nuclei (UMN/LMN), anterior horn cells (LMN)
- SOD2 gene mutation- rare familiar cause
Subtypes of MND
- ALS (amyotrophic lateral sclerosis)
- Progressive bulbar palsy (rare)
- Progressive muscular atrophy
- Primary lateral sclerosis
What is not affected in MND
- No sensory involvement or cerebellar signs
- Never affects eye movement
MND: ALS S&S
- 40+
- LMN- progressive limb weakness (often upper limn), wasting of thenar/tibialis anterior, fasciculation’s
- UMN- hypertonia, brisk reflexes, upgoing plantars, spasticity
- Loss of motor neurones in motor cortex and anterior horn cells
MND: Progressive bulbar palsy S&S
- Muscles of talking, chewing and swallowing affected
- Absent jaw jerk (LMN)
- ## Tongue paralysed, no fasciculation’s, increased jaw jerk and pharyngeal reflex (UMN)
MND: Progressive muscular atrophy S&S
- Purely LMN
- Wasting, often in small muscles of hand
- Distal muscles affected first
- May be unilateral start > bilateral
MND: Primary lateral sclerosis
- Affects purely UMN
Investigations for MND
- Nerve conduction studies (exclude peripheral neuropathy/myopathy)
- Electromyography
- MRI- exclude cervical cord compression, MS and myelopathy
Management of MND
- Feeding and respiratory support > overnight non-invasive ventilation
- MDT approach> OT, SALT, PT, dieticians, MND specialists nurses
- Riluzole: prolongs survival by a few months
- Hyoscine: for drooling
- Baclofen: for spasticity
- Anti depressants
What causes Guillain-Barre syndrome
- Immune mediated demyelination fo the peripheral nervous system
- Infection a couple weeks prev (classically campylobacter)
Features of Guillain-Barre syndrome
- Ascending weakness (proximal muscles affected first, affecting lower limbs before upper)
- prev infection couple weeks before
- Little sensory disturbance
- Arreflexia
- Cranial nerve dysfunction > diplopia
- Autonomic dysfunction > sweating, tachycardia, arrhythmias, urinary retention
Investigations of Guillain-Barre syndrome
- Nerve conduction studies= slow
- CSF: raised protein, normal WCC
- Anti-GM1 antibodies (in 25% of patients)
Management of Guillain-Barre syndrome
- IV immunoglobulin for 5 days (IVIg)
- Plasma exchange (IVIg could be contraindicated so do this)
- 85% will make full recovery
What is Miller-Fisher syndrome
- A varient of Guillain-Barre
- Opthalmoplegia, ataxia, arreflexia (eyes affected first)
- Descending paralysis
What is Myasthenia Gravis
- Autoimmune disease
- Fatiguability due to fewer available AChRs at NMJ
- You would find Ach-R and MuSK
Features of MG
- Female, 30-50
- Other autoimmune diseases
- Increasing muscular fatigue
On order- extra ocular (ptosis, diplopia), bulbar (swallowing, chewing, dysphonia), face, neck, limbs(proximal), girdle, trunk - Normal tendon reflexes > a brisk, one off muscle contraction is possible in normal and MG
Signs of MG
- Peek sign- after 30 secs of forced eyelid closure, the patient is unable to keep the lids fully closed
- myasthenic snarl- May be observed when patient tries to smile,
Investigations of MG
- Antibodies: anti-AChR, MuSK
- CT thorax > thymus
- Ice test > ice applied to affected lid and ptosis improves
- Tensilon - + result shows improvmentn in muscular strength after administration
Treatment of MG
- Acetylcholinesterase inhibitors > slow down re-uptake of Ach eg pyridostigme, neostigme. (cholinergic s/e- ^parasympathetic activation, ^salivation, lacrimation, sweats, vomiting, miosis)
- Immunosuppressants for relapses eg prednisolone, azathioprine, methotrexate
- Thyectomy
What is a myasthenic crisis
- weakness of resp muscles
- may need intubation and ventilation
- treat with plasmapheresis/ IVIg
- Treat trigger > infections, medications, post op
Causes of depression
- Drugs eg BB, opioids
- metabolic eg anaemia, B12/foalte def, cancer
- Infective eg post viral, UTI
- Infalmmatory eg temporal arteritis
- intracranial eg post stroke, parkinsons, delirium, dementia
Tx of depression
- Antidepressants eg SSRIs- vascular depression often responds poorly to htis
- talking therpies
social inclusion and community support
ECT
Blackout differentials
- vasovagal syncope
- cardiac syncopee
- non epileptic attacks
- intermittent hydrocephalus (rare)
- migraine (if no loss of awareness)
