Week 12 Flashcards
What is horner’s syndrome?
Results from ipsilateral disruption of cervical/thoracic sympathetic chain
Consists of:
- miosis
- ptosis
- apparent enopthalmos
- anhidrosis
What is the clinical presentation of radial neuropathy?
Most common cause: entrapment at spiral groove
Presenting symptoms:
- wrist and finger drop (wrist and finger extension weakness)
- usually painless
Sensory change over prone surface of thumb and lateral side of the hand as well as lower half of finger 2 and lateral half of finger 3
What is the clinical presentation of ulnar neuropathy?
Most common cause: entrapment at ulnar groove (medial epicondyle of humerus)
Presenting symptoms:
- may be history of trauma at elbow
- sensory disturbance and weak grip
- usually painless
Sensory change over medial aspect of the hand; half of finger 3,4 and 5
What is the clinical presentation of medial neuropathy?
Most common cause: entrapment within carpal tunnel at wrist
Presenting symptoms:
- history of intermittent nocturnal pain, numbness and tingling, often relieved by shaking hand
- patient may complain of weak grip
- +ve Tinel’s sign/Phalen’s test
Sensory change over finger tips of thumb, 2,3 and 4 and supine surface of hand from half of finger 4 to thumb
What is the clinical presentation of common peroneal neuropathy?
Most common cause: entrapment at fibular head
Presenting symptoms:
- may be history of trauma, surgery or external compression
- acute onset foot drop + sensory disturbance
- usually painless
Motor weakness:
- Tibialis anterior resulting in ankle dorsiflexion weakness
- Extensor hallicus longus resulting in great toe extension weakness
What is the clinical presentation of femoral neuropathy?
Most common cause: haemorrhage/trauma
Presenting symptoms:
- weakness of quadriceps
- weakness of hip flexion
- numbness in medial shin
Sensory change over medial aspect of lower leg
What are the causes of length dependent axonal neuropathy?
Diabetes Alcohol Nutritional deficiency (folate/B12) Immune mediated (RA, SLE, vasculitis) Metabolic/endocrine (renal failure, hypothyroidism) Infectious (HIV, Hep B&C) Genetic Neoplastic (myeloma) Paraneoplastic Critical illness
Drugs: isoniazid, cisplatin, amiodarone, gold
Describe the clinical presentation of neuropathy:
Depends on the type and distribution of affected fibres
Motor: weakness/muscle atrophy
Sensory:
large (myelinated) fibres -> sensory ataxia, loss of vibration sense +/- tingling
small (thin/unmeylinated fibres) -> impaired pin prick, temperature, painful, burning, numbness and tingling
Autonomic:
-> postural hypotension, erectile dysfunction, GI disturbance, abnormal sweating
Describe Gullain-Barre syndrome:
- Acute inflammatory demyelinating neuropathy
- Post-infectious autoimmune aetiology (e.g. campylobacter, CMV, EBV)
- Progressive (ascending) weakness over days
- Flaccid, quadraparesis with areflexia
- +/- respiratory/bulbar/autonomic involvement
- treated with IVIG or apheresis
- CIDP: chronic form (steroid and IVIG responsive)
- Demyelinating pattern on nerve conduction is supportive in diagnosis of GB, as well as high protein levels in CSF
Describe myasthenia gravis:
Pathogenesis and investigation:
- Autoimmune disorder; antibodies to acetycholine receptor at post-synaptic NMJ
- May be associated with thymic hyperplasia or thymoma (residual tumour of thymus gland)
- Affects young women in 20s and older men in 70s
- Fatiguable weakness of ocular. bulbar, neck, respiratory and/or limb muscles
Investigation and management
- Antibodies to AChR present in 85% of cases
- Single fibre EMG and repetitive nerve stimulation also abnormal
- Managed with pyridostigmine (anti-acetylcholineesterase) and immunosuppressive therapies (steroids and IVIG)
What is the difference between primary and secondary headache?
Primary headache = headache and its associated is the disorder (eg migraine, tension-type headache, clutster headache)
Secondary headache = secondary to underlying cause (eg subarachnoid haemorrhage, space-occupying lesion, meningitis, temporal arteritis, high/low intracranial pressure, drug-induced)
What are the important features in headache history?
- Onset
- time to maximal symptoms
- circumstances at onset
- Severity and quality of pain
- pain scale 1-10
- throbbing/pulsatile/’vice-like’/stabbing)
- Location/radiation of pain
- Presence of aura/prodrome
- Periodicity
- duration and frequency
- Associated features
- Age at onset of headache
- Triggers/exacerbating/relieving factors
- Family history
- Social/employment history
- Medication history
- Co-morbid depression/sleep disturbance
What clinical signs are important when examining a patient with headache?
General/systemic:
Reduced conscious level, BP/pulse, pyrexia, meningism, skin rash, temporal artery tenderness
Cranial nerve:
Pupillary responses. visual fields +/- blind spot, eye movements, fundoscopy
What are red flags in pt with headache?
- Age >50 years
- Thunderclap headache
- Focal/non-focal neurological deficit
- Worsening of symptoms with posture (high/low CSF pressure headache), valsalva (coughing/straining), or physical exertion
- Early morning headaches
- Systemic symptoms: fever, weight loss
- Seizures, meningism
- Termporal artery tenderness/jaw claudication
- Specific situations- cancer, pregnancy, post-partum, HIV/immunosuppression
SNOOPT
Systemic symptoms, neurological signs or symptoms, older age at onset, onset is acute (under 5 minutes), previous headache history is different/absent, triggered headache (valsalva or posture)
Describe migraine:
- Female preponderance
- Prevalence highest in 25-55yr
- Positive FHx
- Triggers; hormonal, weather, stress, hunger, sleep disturbance, exertion, alcohol, foods
- Can be with (30%) or without aura
Management:
- lifestyle: avoid triggers, reduce caffeine/alcohol intake, encourage regular meals and sleep patterns
- acute management: simple analgesia (paracetamol, aspirin, NSAIDs), triptans (sumatriptan), +/- antiemetic
- prophylaxis: beta-blockers, tricyclic antidepressants, anti-epilepsy drugs
Describe features of high and low pressure headaches:
High:
- optic disc swelling (papilloedema)
- enlarged blind spot
- impaired visual acuity/colour vision
- 3 and 6 nerve palsy
Low:
- worse on sitting/standing up and relieved by lying down
- results from CSF leakage
- causes: post-lumbar puncture, spontaneous intracranial hypotension)
Describe assessment and investigation of thunderclap headache:
Causes: subarachnoid haemorrhage, intracerebral haemorrhage, arterial dissection
Investigations: primary aim is to identify SAH
- bloods
- 12-lead ECG
- urgent CT brains
- lumbar puncture
Describe the epidemiology of stroke:
Modifiable RFs
- BP
- Smoking
- DM
- Heart disease (valvular, ischaemic, AF)
- PVD
- past TIA
- Increased PCV
- Carotid bruit
- The pill
- Hyperlipidaemia
- Alcohol use
Describe the pathological processes that can result in stroke:
Haemorrhagic: rapidly developing clinical signs of neurological dysfunction attributable to a focal collection of blood within the brain parenchyma or ventricular system that is not caused by trauma
Intracranical bleed causes:
- small vessel disease (deep perforating vasculopathy)
- amyloid angiopathy
- abnormalities in blood vessels
- blood clotting deficiencies
- haemorrhagic transformation of an infarct
- tumours
- drug useage: cocaine, amphetamine
Ischaemic: an episode of neurological dysfunction caused by focal cerebral, spinal or retinal infarction
Describe the clinical presentation of stroke:
TACS: total anterior circulation syndrome
- hemiparesis + higher cortical dysfunction + hemianopia
- usually proximal MCA or ICA occlusion
PACS: partial anterior circulation syndrome
- isolated higher cortical dysfunction OR
- any 2 of hemiparesis, higher cortical dysfunction, hemianopia
- usually branch MCA occlusion
POCS: posterior circulation syndrome
- isolated hemianopia OR
- brainstem syndrome
- can include perforating arteries, PCA or cerebellar arteries
LACS: lacunar syndrome
- pure motor stroke
- OR pure sensory stroke
- OR sensorimotor stroke
- OR ataxic hemiparesis
- OR clumsy hand-dysarthria
- perforating artery/small vessel disease
Describe the management of stroke:
3 treatments proven to be effective:
- thrombolysis
- within 4.5 hrs of symptom onset
- thombectomy
- within 6-8hrs of symptom onset
- admission to a stroke unit
- coordinated care
- short-long term therapy
- prevent aspiration
The earlier treatment is given the better
Brain imaging is important for diagnosis and management of an acute stroke
Describe the pathogenesis and clinical presentation of SAH:
Aneurysm formation; localised dilatation of artery
RFs: smoking, female sex, hypertension, +ve FHx, ADPCK, Ehlers Danlos, coarctation of the aorta
Exam looking for:
- photophobia
- meningism
- subhyaloid haemorrhages
- vitreous haemorrhages
- speech and limb disturbances
- pulmonary oedema
History:
- sudden onset headache
- LOC, seizures, visual, speech and limb disturbances
- sentinel headache
Describe the investigation and management of SAH:
CT:
- confirms diagnosis
- clues to aetiology
- identifies complications
- prognostic: fisher grade
Other investigations:
- LP (xanthochromia)
- CTA/MRA
- Digital subtraction angiogram
Management:
- surgical clipping
- endovascular (coils, stents and glue)
- conservative
What are the complication of SAH?
- Re-haemorrhage
- Delayed ischaemia
- Hydrocephalus
- Hyponatraemia
- ECG changes (CVS symptoms due to output of catecholamines)
- LRTI, PE, UTI
- Seizures
- Might also require sequential DVT prophylaxis
Outline GCS:
Eye opening:
- Spontaneously
- To speech
- To pain
- No response
Verbal response:
- Oriented to time, person and place
- Confused
- Inappropriate words
- Incomprehensible sounds
- No response
Motor response
- Obeys command
- Moves to localised pain
- Flex to withdraw from pain
- Abnormal flexion
- Abnormal extension
- No response
Describe prevalence of MS in Scotland:
Scotland has highest incidence and prevalence in world
Approx 1 in 600 people in Scotland have MS
Highest prevalence in Orkney
Describe pathology of MS:
Acute episodes of inflammation are associated with focal neurological deficits
Demyelinating results in loss of neurological function:
- weak leg
- visual loss
- urinary incontinence
Optic neuritis, clinically isolated syndromes, transverse myelitis and radiologically isolated syndromes may all develop into MS
Describe presentation and diagnosis of MS:
MS is diagnosed where there is evidence of 2 or more episodes of demyelination disseminated in space and time
Usually presents:
- neuro symptoms that develop over a few days
- history of transient neuro symptoms that have lasted for more than 24 hours and spontaneously resolved
- ‘hidden’ relapses should always cause suspiscion of MS
- optic neuritis/visual disruption
- Bell’s palsy
- labyrinthitis
- sensory sypmtoms
- bladder symptoms in young man/woman without children
SEE SUMMARIES FOR SYMPTOM DIAGRAM
Outline role of MRI, LP and VERS in MS diagnosis:
MRI:
- image brain and cervical spine with gadolinium contrast
- possible to make diagnosis of MS with one scan:
- evidence of dymyelination in 2 regions can indicate dissemination in space
- if enhancing and non-enhancing areas of dymyelination are seen this can indicate dissemination in time
LP: look for presence of oligoclonal bands (need matched blood sample)
VERS:
- measure conduction of nerve signals in optic nerve to look fir subclinical optic neuritis
- conduction will be slower if a patient has had optic neuritis in the past
Describe treatment for MS:
Relapse:
- steroid regime
RRMS: DMARDs
- alemtuzumab
- natalizumab
Describe treatment for MS:
Relapse:
- steroid regime (+ PPI for gastric protection)
RRMS: DMARDs - alemtuzumab - natalizumab oral treatments - fingolimod - dimethyl fumarate
Describe the pathological changes seen in Parkinson’s disease:
- Basal ganglia have role in maintaining posture and initiating voluntary movement
- Loss of dopaminergic neurones within substantia nigra
- Surviving neurones contain Lewy bodies
- PD manifests clinically after loss of approximately 50% of dopaminergic neurones
Describe the CFs of PD:
Bradykinesia: slowness in initiation of voluntary movement with progressive reduction in speed and amplitude of repetitive actions
Also with muscular rigidity, 4-6 Hz rest tremor (pill-rolling), posutral instability
Non-motor symptoms:
- neuropsychiatric;
- dementia, depression, anxiety
- autonomic;
- constipation, urinary urgency/nocturia, erectile dysfunction, excessive salivation/sweating, postural hypotension
- sleep;
- REM sleep behaviour disorder, restless leg syndrome, daytime somnolence
- other;
- reduced olfactory function, fatigue, pain and sensory symptoms
Describe investigations of PD:
Bloods:
- if tremor present: TFTs, copper/caeruloplasmin
Structural imaging:
- CT/MRI brain normal in PD
- Abnormal in vascular parkinsonism, Parkinson plus disorders
Functional imaging:
- Imaging of presynaptic dopaminergic function using DAT SPECT is abnormal in degenerative parkinsonism
Outline the principles of drug management of PD:
Pharmacological aim: restore dopamine levels
Clinical aim: improve motor symptoms / improve QOL
Drug classes: • L-dopa • Dopamine agonists • MAO-B inhibitors • COMT- inhibitors
L-dopa:
- taken up by dopaminergic neurones and decarboxylated to dopamine within presynaptic terminals
- prescribed with dopa-decarboxylase inhibitor
Longer term: Approx. 50% of patients develop motor complications after 5 years L-dopa
– Fluctuation in motor response
– Dyskinesia – most commonly
chorieform movements at peak dose
Describe the role of MDT in PD:
Multi-disciplinary team • GP • Neurologist / care of the elderly physician • Parkinson’s disease nurse specialist • Physiotherapist • Speech and language therapist • Psychiatrist • Psychologist • Occupational therapist • (Palliative care team) • (Neurosurgeon)
What is in differential diagnosis of PD?
- Diagnosis of PD is largely clinical diagnosis
- Diagnostic certainty improves with follow-up
- Disorders commonly mistaken for PD;
- benign tremor disorders (e.g. essential tremor)
- dementia with Lewy bodies
- vascular parkinsonism
- Parkinson plus disorders (e.g. progressive supranuclear palsy, multiple system atrophy)
- Drug-induced parkinsonism/tremor
Outline causes for LOC:
- Syncope
- Migraine
- Narcolepsy/cataplexy
- Transient global amnesia
- Panic attacks
Define seizures and epilepsy:
Seizure: episode of neuronal hyperactivity causing signs and symptoms
Seizures can be focal or generalised
Focal: area of abnormality in otherwise normal brain, which can spread and cause generalised seizure
Generalised: thought to be genetic, arise from generalised tendency to repeated discharges and action potentials
- potentially disorder of metabolism of NTs or ion channels
- affects whole brain at once
Epilepsy: at least two unprovoked episodes of seizure
Briefly outline types of epilepsy:
Focal epilepsy:
- history trauma/birth injury
- focal aura/sequelae (including gustatory/sensory/motor features)
- post-attack confusion/drowsiness
- automatisms
- noctural events
GGE features:
- photo-sensitivity
- age of onset= 8-26
- alcohol or sleep deprivation
- myoclonus (especially in AM)
- lack of aura
- seizures within 2 hours of awakeneing
- FHx of IGE
- EEG abnormal
Discuss investigations of LOC;
History: from patient and eye witness
Brain imaging; MRI in younger people
EEG;
- observe activity on EEG with lying at rest, then hyperventilation, with photic stimulation and watch with drowsiness
- can also do ambulatory/video telemetry EEG
Systemic provocations
Outline long term management of epilepsy:
Focal Epilepsy
Lamotrigine, Carbamazepine, Levetiracetam
Generalised Epilepsy
Valproate, Levetiracetam, Lamotrigine
Describe status epilepticus:
Status Epilepticus • repetitive or prolonged epileptic seizures • a medical emergency • may cause profound systemic / neuronal damage • recognised mortality
Definition >2 seizures without full recovery of neurologic function between seizures or continuous seizure activity >30 mins
What are radiological and lab tests for CNS infection:
CT in suspected meningitis: - CT to exclude mass lesion / mass effect, gross cerebral oedema - Doesn’t exclude raised ICP - CT before LP if: - GCS ≤ 12 - CNS Signs - Papilloedema - Immunocompromised - Seizure ANTIBIOTICS PRE-CT SCAN
LAB TESTS:
- history and exam
- blood cultures (blood PCR)
- throat culture, viral gargle
- FBC, U&Es, LFTs, CRP
- LP
- cell count, gram stain, culture and PCR
- protein and glucose
- viral PCR
How are CNS infections managed?
Possible BM: IV ceftriaxone
Definitive antibiotic therapy for BM:
- Meningococcal
- IV ceftriaxone or ben pen
- 5-7 days
- Pneumococcal
- IV ceftriaxone or ben pen
- 14 days
- Listeria
- IV amox
- 21 days
Describe common pathological processes in common dementias:
AD: environmental and genetic factors play a role in accumulation of beta-amyloid peptide resulting in progressive neuronal damage, neurofibrillary tangles and loss of neurotransmitter acetylcholine
VD: cumulative effects of many small strokes, thus sudden onset and stepwise deterioration is characteristic
Lewy Body Dementia: typically with fluctiating cognitive impairment, detailed visual hallucinations and later, parkinsonism. Histology is characterised by Lewy Bodies in brainstem and neocortex
Pick’s: frontal and temporal atrophy without AD histology, genes on on chromosome 9 are important
Other causes are alcohol/drug abuse, repeated head trauma, pellagra, Whipple’s disease, Huntington’s, Parkinson’s
Describe clinical presentation of common dementias:
Suspect AD in adults with enduring, progressive and global cognitive impairment: visuo-spatial skill (gets lost), memory, verbal abilities and executive function are all affected Also anosognosia (lack of insight into the problems engendered by the disease) Later they may be irritability, mood disturbance, behavioural changes, agnosia (may not recognise self in the mirror)
No standard natural history
Discuss investigation of common dementia syndromes:
FBC, ESR, U&Es, Ca2+. LFT, TFTs, autoantibodies, B12/folate, syphillis serology
CT/MRI (for vascular damage, haemorrhage or structural pathology)
Consider also EEG, CSF, functional imaging (FDG, PET, SPECT)
Metabolic, genetic and HIV tests if indicated
