Core: Neurology Flashcards
MND Aetiology
- Often unknown
- Mutations in SOD-1, TDP43, FUS
- Familial - C9orf72 w/ hexanucleotide repeat GGGGCC on chromosome 9
MND pathophysiology
- Oxidative damage to neurones
- Damaged motor neurones then die.
- Progressive weakness UMN and LMN
MND Fasciculation causes
- Abnormally large motor units due to MN death, fewer nerve fibres to innervate large units.
MND classic presentation
- Weakness + wasting + fasciculation
- UMN and LMN signs = wasted muscle + brisk reflexes.
MND other presentations
- Progressive muscular atrophy - purely LMN. Starting in one limb and progressively involving others.
- Progressive bulbar and pseudobulbar palsy - lower CN nuclei involvement. Dysarthria, dysphagia, nasal regurgitation and choking. Tongue fasciculations and emotional incontinence.
- Primary lateral sclerosis - Slow progressive tetraparesis and pseudobulbar palsy.
Patient - Over 40, stumbling spastic gait, foot drop, proximal myopathy, weak grip, fasciculations
MND
MND Ix
- Often clinical
2. EMG
MND Rx
- Riluzole - antiglutaminergic
- Symptom management; Drooling = propantheine, amitriptyline
Dysphagia = blend foods
Pain = analgesic ladder
Respiratory distress = NIV
Patient - Any age, acute onset headache, neck stiffness and fever. ?purpuric rash
Meningitis
Meningitis Aetiology
- Bacteria - Meningococcus, S.pneumoniae, S.aureus, GBS, TB, listeria, E.coli
- Viral - Enterovirus, mumps, HSV, HIV, EBV
- Fungal - Crytococcus (HIV), candida.
- IT drugs
Meningitis Pathology
- Inflammation of the meninges
- Transmission often via direct extension from ear, nose, throat, blood or direct trauma.
- Pia-arachnoid space becomes congested w/ neutrophils and a layer of pus forms.
- Adhesions can be formed which can cause CN palsies.
Meningitis Presentation
- Headache
- Neck stiffness
- Fever
- Photophobia
- N&V
- Rash
- SHOCK
- Kernig’s sign.
- Bulging fontanelle
Meningitis Ix
- Clinically suggested
- Sepsis 6
- FBC
- LP if not contraindicated
Meningitis Rx
- In the community = 1.2g benpen stat or 1g cefotaxime IM
- Sepsis 6
- Cefotaxime IV 2g/6hr + amoxicillin (if listeria suspected)
- Rx empirically according to MC&S
- Notify PHE
Meningitis Contact prophylaxis
- Rifampicin 600mg/12hr 2 days or ciprofloxacin 500mg PO 1 dose
Patient - Any age, meningism, no rash, behavioural changes, seizures and focal neurology
Encephalitis
Encephalitis Aetiology
- Viral - HSV, VZV, Enterovirus, adenovirus. SSPE following measles.
Encephalitis Pathology
- Virus replicates in the bloodstream.
- Enters neural cells and causes congestion and disruption of function within the brain.
Encephalitis Presentation
- Meningism (often less severe than meningitis)
- Personality and behavioural change
- Viral prodrome
- lethargy
- Seizure
- CN issues
Encephalitis Ix
- MRI brain - parenchymal inflammation and swelling.
- EEG - Periodic sharp and slow wave complexes
- CSF - raised lymphocytes
- Viral PCR.
Encephalitis Rx
- Immediate IV acyclovir (10mg/kg 3xdaily for 14-21 days)
2. Symptom control –> Seizure
Patient - Headaches (worse on coughing, leaning forward), vomiting, new onset seizure, progressive defect.
Mass lesion in the brain
Mass lesion aetiology
- Commonly metastasis from the bronchus, breast, stomach, prostate, thyroid and kidney.
- Primary = astrocytoma, oligodendroglioma, cerebral lymphoma
- Benign = meningioma, neurofibroma.
Mass lesion pathology
- Mass effect.
- As the lesion grows, its shifts structures within the brain creating pressure against the cranium.
- Direct infiltration of brain tissue.
Mass lesion presentation
- Raised ICP
- New onset seizure
- Focal neurology curtailing to a certain part of the brain
- Personality changes etc.
Mass lesion Ix
1) CT/MRI
2) Biopsy
3) XR and PET
Mass lesion Rx
1) Surgical resection if possible
2) Cerebral oedema - dexamethasone
3) Chemo/radio (gamma knife)
Patient - Old, alcoholic, post traumatic head injury … reducing GCS ?
Subdural haematoma
Subdural haematoma Aetiology
- Head trauma
- Spontaneous
- Coagulopathy or blood thinners
- Intracranial HTN
- Child abuse … shaken baby y’all
Subdural haematoma pathology
- Venous bleed.
- Atrophic brains - greater stretch of a bridging vein which connects the brain to a dural sinus.
- Low pressure bleed dissects the arachnoid from the dura and blood pools in the cranium.
- Herniation can occur.
Subdural haematoma Presentation
- Elderly
- Alcoholic
- Headache
- Reducing GCS or drowsiness, confusion
- Focal neurology; paresis or sensory losses
- Seizure
- Coma
- Coning !!!!! Arghhhhh
Subdural haematoma Ix
1) CT - hyperdense white crescent moon shaped mass at skull edge. midline shift.
2) Bloods - coag screen.
Subdural haematoma Rx
1) ABCDE
2) Neurosurgery
3) Reduce cerebral pressure and oedema w/ mannitol if present risk of herniation
Patient - 40, sudden onset occipital headache, ‘worst i’ve ever had darling’, neck stiffness.
Fuckkkkkk - SAH
SAH Aetiology
- Idiopathic
- Berry aneurysm
- AVM
- Association w/ PKD, meningitis, coagulopathy
SAH pathology
- Spontaneous arterial bleed
- Circle of willis berry aneurysm
- Downstream ischaemia
SAH presentation
- Sudden onset occipital headache
- Worst ever doc
- Or presents with low GCS at ED oops.
- Vomiting
- Coma
- Raised ICP signs
- Hx of other smaller similar headaches (sentinel bleeds)
SAH Ix
1) CT ASAP - Subarachnoid blood, intraventricular blood, star sign.
2) LP - xanthochromia
3) CT angiography to find aneurysm
SAH Rx
1) ABCDE
2) bed rest with best supportive care
3) Manage BP - nimodipine (CCB) for 3 weeks
4) Decompressive craniotomy
5) Coil/clip the aneurysm
Patient - Young rugby player, been to the pub, punched in the side of the head. Brief LOC at the time and has felt fine since.
- ?Extradural
Extradural haemorrhage aetiology
- Traumatic injury to the temporal bone
- Following LP
Extradural haemorrhage Pathology
- Temporal bone fracture
- Torn middle meningeal artery whose foramen passes through the bone.
- Pooling of blood between the bone and brain.
Extradural haemorrhage presentation
- Brief LOC at time of injury followed by lucid injury.
- Developing stupor, ipsilateral dilated pupil and contralateral hemiparesis.
- Coning
- Raised ICP
Extradural haemorrhage Ix
1) CT head - hyper dense lenticular mass
2) Skull XR for fracture
3) bloods - FBC and coagulation
Extradural haemorrhage Rx
1) ABCDE
2) Neurosurgery - decompressive craniotomy
Patient - Old man, pill rolling tremor, narrow suffering gait and slow like a tortoise. Keeps kicking his wife in bed and can’t smell how bad his feet smell.
Parkinson’s disease
PD Aetiology
- Unknown
- Mixed genetics and environmental
- Oxidation hypotheses
- PARK gene
PD pathology
- loss of pigmented dopaminergic neurones in the substantia nigra and the presence of lewy bodies.
- Loss of dopaminergic neurones lead to decreased movement .
PD Presentation
- Tremor
- Bradykinesia
- Rigidity
- Anosmia
- Depression
- Lack of expression
- Autonomic issues; urinary urgency and hypotension
- Micrographia
Parkinson Plus
1) Progressive supranuclear palsy = parkinsonism + postural instability + falls + pseudo bulbar palsy + dementia
2) Multiple system atrophy = Autonomic Sx + ataxia + Parkinsonism
PD Ix
1) Clinical
2) DAT scan
PD Rx
1) Urgent neurology referral
2) Education
3) Dopamine replacement w/ levodopa combined w/ a dopa decarboxylase inhibitor (co-beneldopa or co-careldopa)
4) Deep brain stimulation
Epilepsy Aetiology
- Idiopathic
- Mass lesions
- Vascular changes
- Neurodegeneration
- Drugs and alcohol
Epilepsy Pathology
- Sudden synchronous discharge of cerebral neurones causing signs or symptoms apparent to the patient or/and observer
- Abnormalities in ion channels which influence neurotransmitters and cause aberrant firing.
- Triggers = sleep deprivation, alcohol, drugs, strobe lights
Epilepsy presentation
- Prodrome
- Aura
- Seizure (focal, simple, complex, absence, GTCS, myoclonic, atonic)
- Post-ictal periods of confusion
Partial seizure - simple
- Patient is conscious
- One limb jerking (jacksonian)
Partial seizure - complex
- Loss of patient consciousness
- one limb jerking
Generalised absence seizure
- often start in childhood
- LOC and vacant expression
- Patient doesn’t realise that any time has passed.
Generalised tonic clonic seizure
- Tonic phase (stiffness)
- Clonic phase (limbs jerking)
- Often w/ tongue biting and incontinence
Generalised Myoclonic
- Loss of consciousness
- Jerking or twitching only
Generalised Atonic seizure
- Drops to the ground w/ a complete loss of tone.
Epilepsy Ix
1) Bloods - including calcium
2) ECG
3) MRI brain for mass lesion
4) EEG
5) Drug screen and hx
Epilepsy Rx
1) ABCDE
2) Anti-epileptic drugs;
GTCS = Valproate, lamotrigine, carbamazepine, topiramate Focal = carbamazepine, lamotrigine.
Myoclonic = Valproate. Absence = Valproate
Status epilepticus
Early <30min - O2 + ECG/BP + Bloods + Lorazepam IV 4mg and repeat if needed.
Established status 30-90min - phenytoin 15mg/kg IV
Persisting status - Phenobarbital 10mg/kg IV or valproate 25mg/kg
Still persisting - RSI and intubation.
Patient - Sudden loss of neurological function (dropping face + arm weakness) only lasts a few mins and fully resolves
TIA
Patient - sudden loss of neurological function (drooping face, weak arm and slurred speech) persisting over 24hrs
Stroke
CVA aetiology
- Atherosclerotic disease
- AF
- RF’s = HTN, smoking, alcohol, high cholesterol, AF, obesity
CVA Pathology
- Either brief or persistent downstream ischemia following embolus or haemorrhage.
- Neuronal death and loss of function.
- Transient (TIA) or persistent (stroke)
CVA Presentation
- SUDDEN loss of function.
- Carotid system = amaurosis fugax, aphasia, hemiparesis, sensory loss and hemianopic visual loss.
- Vertebrobasilar system = Diplopia, vertigo, chocking, ataxia.
CVA Ix;
1) Clinical judgement
2) CT or MRI to assess damage
3) ECG
4) Bloods including; FBC, COAG, culture
5) Carotid artery Doppler
CVA Rx
1) ABCDE
2) Thrombolysis (window 4.5hrs) - alteplase
3) 300mg aspirin if not apt for thrombolysis.
4) Haemorrhage - control BP and refer to neurosurgery.
5) stroke rehab
6) TIA = Clopidegrel and statins for life.
Patient - Middle aged woman, increasing tiredness throughout the day, evening double vision and slurring of the speech. This is worse w/ repeated movements.
Myasthenia Gravis
Patient - receiving therapy for small cell bronchial carcinoma, foot weakness which improves w/ exercise and use.
Labert eaton
MG Aetiology
- Acquired
- Causes unknown
- Associated w/ thymoma or thymic hyperplasia
- Associated w/ other autoimmune disease.
MG pathology
- Antibodies to ACH receptor proteins are found.
- Immune complexes on the post-synaptic membrane prevent ACH binding at the NMJ.
MG presentation
- Fatigability of actions and reflexes.
- Proximal limbs, extraocular muscles, facial muscles.
- Fluctuating and fatiguable weakness.
- Can effect resp muscles.
MG Ix
1) Serum anti-ACHr antibodies and anti-MUSK
2) Repetitive nerve stimulation leading to weakness.
3) Tensilon test - immediate improvement in weakness.
4) CXR for thymoma
MG Rx
1) Pyridostigmine 60mg. 3-4hr duration. Inhibits ACH breakdown
2) Immunosuppression - AZA, MM
3) Thymectomy
4) Plasmapheresis + IVIG for exacerbations.
Patient - Middle life, fidgety progressing to uncontrollable limb movements, family history, depression
Huntingtons disease
HD Aetiology
- CAG nucleotide repeat expansion - huntingtin
- Toxic gain of function.
- Anticipation phenomenon
- AD inherited
HD Pathology
- selective neuronal loss in the neostratum, caudate nucleus and putamen.
HD Presentation
- middle life
- Fidgetiness progressing to chorea
- Psychiatric issues such as depression and mania.
- Chorea is eventually replaced by parkinsonism.
- Dysarthria and dysphagia
- Tics and myoclonus.
HD Ix
1) Clinical
2) Genetic testing for CAG
3) FHx
4) Genetic counselling
HD Rx
1) Benzo, valproate etc for chorea
2) Rx depression and psych empirically
3) Nil
Patient - Elderly, impaired memory function over a number of months/years, autobiographical memory, language, word finding w/ lack of self insight
AD dementia
Patient - Elderly, visual hallucinations often small animals and people, fluctuating cognition, parkinsonism
LB dementia
Patient - Step-wise cognitive decline following overt or covert CV events. Apraxic gait, pyramidal signs.
Vascular dementia
Patient - Middle aged, personality change, affect blunting, inappropriate behaviour, impaired language and fluent speech. Sx of ALS
FTD
Dementia Aetiology
- Degenerative = AD, LBD, FTD, HD, PD, CJD
- Vascular
- Metabolic = Uraemia, Thiamine
- Toxins = alcohol, drugs
Dementia Pathology
- AD = UK, amyloid plaques, neurofibrillary tangles.
- LBD = presence of lewy bodies, associated with PD.
- FTD = familial, TAU mutations or MND associated.
Dementia Presentation
- Memory loss
- Cognition loss
- Permanent not transient
- See patient summaries for more in detail explanation of diseases.
Dementia Ix
1) MMSE, MOCA, AMT
2) Bloods; FBC, TFT, B12 etc. rule out organics.
3) Psych evaluation - depression can cause pseudo-dementia
4) Brain imaging - mass lesion or brain atrophy, evidence of vascular insults.
5) Memory clinic evaluation
Dementia Rx
1) General = lifestyle and continued cognitive work
2) Cholinesterase inhibitors - rivastigmine, donepezil, galantamine
2) NMDA receptor antagonist = memantine
Patient - Young female, presents with eye pain and blurred vision, few weeks later w/ limb weakness. other Sx on questioning = vertigo, and dysphagia
MS
MS aetiology
- Genetics - multi-gene and complex
- Environment - vitamin D association due to increased prevalence further from equator
- Autoimmune
- Viral trigger - EBV etc
MS Pathology
- Demyelination of the CNS
- Often white matter
- Common in optic nerves, corpus callosum, brainstem.
MS Presentation
- Varied and vague signs and symptoms
- Visual changes (optic neuritis)
- Sensory symptoms
- Clumsiness
- Urinary symptoms
- Can be relapse and remitting, secondary progressive or primary progressive.
MS Ix
1) 2 episodes disseminated in space and time
2) MRI w/ gadolinium enhancement
3) LP - CSF show oligoclonal bands IgG
MS Rx
1) No cure
2) General; education, MDT
3) Rx Sx
4) Relapses - IV pred 1g daily for 3 days
5) DMD’s - Beta-interferon
6) Aggressive = Natalizumab or fingolimod (oral)
Patient - Episodic headache. Aura (lights, sounds, feelings). Photophobia. N&V.
Migraine
Migraine Aetiology
- Genetics - multiple. Neuronal excitability
- Environmental triggers;
C - Chocolate
H - hangovers
O - orgasm
C - cheese
O - OCP
L - lie-ins
A - alcohol
T - tumult
E - exercise
Migraine pathology
- Neurogenic spreading cortical depolarisation.
- wave of neuronal activity followed by a period of depression
- Activation of CN5 nerves leads to headache.
Migraine Presentation
- starts around puberty w/ increasing prevalence to the 4th decade of life.
- Headache + photophobia + N&V + aura (zig zag lines, image fragmentation, shimmering)
- Dx criteria =
Headache lasting 4hrs 3x monthly.
+ 2 of the following
1) unilateral pain
2) Throbbing pain
3) mod to severe in intensity
4) motion sensitivity.
+ 1 of the following
1) N&V
2) photophobia
Migraine Ix
1) Clinical
2) Rule out other causes if suspicious
Migraine Rx
1) Explain
2) Educate around triggers
3) Acute attacks = analgesia, metoclopramide (N&V), triptans
4) Suppression = propanolol, amitryptaline, valproate, topiramate
Patient - Progressing weakness in the distal limb muscles and/or distal numbness. Following viral infection or food poisoning. Absent reflexes and the weakness in progressing proximally.
GBS
GBS aetiology
- Molecular mimicry post infection - CMV or
campylobacter. - Monophasic - doesn’t reoccur.
GBS Pathology
- Molecular mimicry - immune system attacks the myelin as the pathogen mimics these.
- Demyelinating.
GBS Presentation
- 1-3 weeks post infection
- Progressive weakness and numbness in the distal muscles of a limb spreading proximally.
- autonomic nerves involvement.
- Resp muscles may be involved.
GBS Ix
1) clinical
2) Confirmed w/ nerve conduction studies.
3) Raised CSF protein
GBS Rx
1) Monitor vital capacity for rest difficulties
2) LMWH and teds
3) IVIG in the first 2 weeks can reduce severity
4) Self limiting
Stroke thrombolysis time window
4.5hrs
PPS - Progressive supra nuclear palsy
- Features
- Management
- Impairment of vertical gaze (going down stairs - CN exam of ocular muscles)
- Parkinsonism
- Falls
- Slurred speech
- Cognitive impairment
- Poor response to L-dopa
- Sx control
Degenerating cervical myelopathy
- Causes
- Presentation
- Ix
- Rx
- RF = smoking due to its effects on the iV discs and high axial loading.
- Pain
- Loss of motor function (dexterity, stiffness and odd gait)
- Loss of sensory function
- Loss of autonomic function
- Hoffman’s sign - Gold standard = MRI c spine - disc degeneration and ligament hypertrophy and cord signal change.
- Urgent spinal surgery assessment.
- Damage can be permanent if not sorted ASAP.
- Decompressive surgery is definitive.
Valproate
- MOA
- SE
- Increases GABA activity
- P450 Inhibitor
- Nausea
- Weight gain
- Ataxia
- Tremor
- Hepatotoxic
- TTP
- Teratogenic
Syringomyelia
- Causes
- Features
- Dilatation of the CSF space in the spinal cord.
- Occurs in thoracic segments and causes compression of spinothalamic tracts decussating in the anterior white commissure.
- Arnold chiari malformation (herniation of cerebellar tonsils through the foramen magnum, congenital or acquired.) - Loss of sensation of pain. temp and crude touch.
- Cape like.
Intracranial venous thrombosis
- Causes
- Presentation
- Ix
- Rx
- Cerebral infarction due to tissue congestion and obstruction.
- Infections
- Trauma
- Pregnancy
- COCP
- Hypercoaguable states.
- SLE - Lateral sinus (headache)
- Cavernous sinus (compressive Sx and nerve palsies)
- Stroke type syndrome.
- Headache, severe, similar to SAH.
- Seizure
- Impaired consciousness
- Focal neurology. - MR venogram
- CT
- Bloods for clotting. - Similar to stroke pts.
- Anticoagulation or thrombolysis.
