Neurology Flashcards
Types of stroke
Ischemic- clots
Haemorrhagic- bleeds
TIA (transient ischemic attack
Which is more common ischaemic or haemorrhagic stroke
Ischaemic stroke-85%
Haemorrhagic- 15%
Ischaemic Stroke
Interruption of cerebral blood supply:
Embolism
Thrombosis
Systemic hypoperfusion
Stroke- FAST
FACE
ARMS
SPEECH
TIME TO CALL 999
Anterior cerebral artery supplies
midline portions of the frontal lobe and parietal lobe
Middle cerebral artery supplies
majority of the lateral surface of the hemisphere
Posterior cerebral artery supplies
inferior portion of the temporal lobe and occipital lobe
Wernicke area
Controls the ability to understand the meaning of words
Broca’s area
premotor area for speech sounds
Wernicke’s area location
Usually found on left superior temporal gyrus
Broca’s area location
Left posterior inferior frontal gyrus
Wernicke’s area blood supply
Inferior division of the MCA
Broca’s area blood supply
Superior division of the MCA
Oxford Community Stroke Project (OCSP) Classification
Clinical classification of patterns of neurological deficit in acute ischaemic stroke
Oxford Community Stroke Project (OCSP) Classification- different classifications
Anterior Circulation Infarction- Partial (PACI) or Total (TACI)
Posterior Circulation Infarction (POCI)
Lacunar Infarction (LACI)
Anterior Circulation Infarction- arteries affected
Anterior and middle cerebral arteries
Anterior Circulation Infarction- signs and symptoms
Contralateral weakness
Contralateral sensory loss/sensory inattention
Dysarthria
Dysphasia (receptive, expressive)
Homonymous Hemianopia/visual inattention
Higher cortical dysfunction
Posterior Circulation Infarction- arteries affected
2 vertebral arteries, basilar artery, 2 posterior cerebral arteries
Posterior Circulation Infarction- signs and symptoms
Cranial nerve palsy and a contralateral motor/sensory deficit (‘crossed signs’)
Conjugate eye movement disorder (e.g. horizontal gaze palsy)
Cerebellar dysfunction (e.g. vertigo, nystagmus, ataxia, dysarthria)
Isolated homonymous hemianopia
Bilateral events can cause reduced GCS
Lacunar Infarction
Occlusion of deep penetrating arteries
Affects a small volume of subcortical white matter
Underlying process is often referred to as small vessel disease
Lacunar Infarction- symptoms
Do not present with cortical features as subcortical white matter affected e.g. dysphasia, apraxia, neglect, visual field loss
Lacunar syndromes
Pure motor hemiparesis
Ataxic hemiparesis
‘Clumsy hand’ and dysarthria
Pure hemisensory
Mixed sensorimotor
Total anterior circulation stroke (TACS)- criteria
All 3: Unilateral weakness, homonymous hemiopia, higher cerebral dysfunction
Partial anterior circulation stroke (PACS)- criteria
2 of: Unilateral weakness, homonymous hemiopia, higher cerebral dysfunction
Lacunar syndrome (LACS)
1 of: Pure sensory stroke, pure motor stroke, sensori-motor stroke, ataxic hemiparesis
Posterior circulation syndrome (POCS)
1 of: Cranial nerve palsy + contralateral motor/ sensory deficit, bilateral motor/sensory deficit, conjugate eye movement disorder, cerebellar dysfunction, Isolated homonymous hemiopia or cortical blindness
NIHSS- NIH Stroke Scale
Grade and track the severity
Monitor response to acute treatments
Stroke- 1st line investigation
Urgent CT Head (+/- CT angiography)
Stroke- Head CT purpose
Differentiae between ischemic and haemorrhagic stroke
Utility of CT in acute stroke- pros
Quick
Readily available 24/7
Sensitive for haemorrhage
May see a ‘hyperdense vessel’
Utility of CT in acute stroke- cons
Cannot usually diagnose an infarct in the acute phase
Less sensitive than MRI for picking up other abnormalities, LACS + PCS
ASPECTS- Alberta Stroke Program Early CT Score
Segmental estimation of early infarction on CT head in MCA stroke
RAPID software uses a machine learning algorithm to calculate this score.
Ischemic Stroke- treatment-Thrombolysis
Breaks down acute clot- potentially life saving
Within 4,5 hrs of symptoms on set
Risk- haemorrhage, allergic reaction
Ischemic Stroke- treatment-Thrombolysis- Contraindications
Symptoms only minor/ rapidly improving
Haemorrhage on CT/MRI
Active bleeding from any site
Recent GI/UT haemorrhage
Recent treatment with heparin/warfarin
Recent surgery/trauma
Plus many more
Post-thrombolysis care
More aggressive blood pressure monitoring
Vigilance for complications (bleeding)
24 hour CT head (haemorrhagic transformation)
Mechanical Thrombectomy
Mechanical recanalisation of the culprit vessel (catheter aspiration and/or stent retrievers)
6 hour time-window
Mechanical Thrombectomy- risk
femoral haematoma/ pseudoaneurysm, retroperitoneal bleeding, vessel rupture, arterial dissection
Ischaemic Penumbra
Reversibly injured brain tissue around ischemic core (irreversibly damaged brain tissue)
Pathological subtypes of ischaemic stroke (TOAST classification)
Large vessel disease (50%)
Small vessel disease (25%)
Cardioembolic (20%)
Unknown (cryptogenic) (3%)
Rare causes (2%) e.g. dissection, CVST, vasculitis
Stroke- treatment- lifestyle
Smoking cessation
Drug and alcohol cessation
Dietary modifications
Exercise
Driving advice
Stroke- treatment- medical
Thrombolysis
Antiplatelet
Anticoagulation
Statin therapy
Stroke- treatment
Thrombolysis +/- Mechanical Thrombectomy if indicated
or Aspirin 300mg
Neuroepithelial cells
stem cells that differentiate into neurons and glial cells
Glia (gilal cells or neuroglia)
non-neuronal cells in the CNS (brain and spinal cord) and the peripheral nervous system that do not produce electrical impulses.
They maintain homeostasis, form myelin, and provide support and protection for neurons.
Types of Glial cells
Astrocytes, ependymal cells, oligodendrocytes, microglial
Astrocytes
perform metabolic, structural, homeostatic, and neuroprotective tasks
Oligodendrocytes
produce the myelin sheath insulating neuronal axons
Gliomas
a common type of brain tumour, include astrocytoma, ependymoma, oligodendrocyte
WHO grade 1 and 2 – “low”
WHO grade 3 and 4 – “high”
Germ cell tumours
rare paediatric usually cancerous tumours in the pituitary/pineal region
Tumour of the sellar region
Craniopharyngiomas a usually benign, cystic tumours
Brain tumours- cranial nerves
Schwannoma e.g. eighth nerve - acoustic neuroma
Brain tumour- Haematopoietic
Lymph cells - primary CNS lymphoma
Brain tumour- Secondary /metastatic tumours
Lung
Breast
Colorectal
Testicular
Renal cell
Malignant melanoma
Brain tumour- WHO classification
Graded according to how fast they grow and how likely they are togrow back after treatment using both histology and genetics into four grades of malignancy
-1 most benign, 4 most malignant
Brain Tumour Grades 1
Slow growing, non-malignant, and associated with long-term survival
Brain Tumour Grades 2
Have cytological atypia. These tumours are slow growing but recur as higher-grade tumours.
Brain Tumour Grades 3
Have anaplasia and mitotic activity. These tumours are malignant
Brain Tumour Grades 4
Anaplasia, mitotic activity with microvascular proliferation, and/or necrosis. These tumours reproduce rapidly and are very aggressive malignant tumours
Low grade gliomas – grade 2
Slow growing but will undergo anaplastic transformation
Astrocytomas – 3-5 years
Oligodendroglioma – 7-10 years
Average Survival 10 years
Median age 35 years
High Grade Gliomas – 3 and 4
Most common type - 85% of all new cases of malignant primary brain tumour
Either as primary tumour or from pre-existing low grade
High Grade Gliomas – 3 and 4- natural history
Median age onset 45 for 3, 60 for 4
Survival times 3 – 3-5 years 4 – 12 months
Brain tumour- known causes
Majority no cause found
Ionising radiation
5% family history
Immunosuppression (CNS lymphoma)
Brain tumour- symptoms
Varied- dependent on tumour type, grade and site
-Headache
-Seizures
-Focal neurological symptoms
-Other non-focal symptoms
Brain tumour- headache
Woken by headache, worse in the morning, worse lying down, associated with N&V, exacerbated by coughing, sneezing, drowsiness
Typically 1st symptom and seen at presentation
Headache- brain tumour vs norm pop
70% patients with brain tumour will have headache (same as normal population)
Brain tumour- headache red flags
Headache PLUS
Headache and age (>50)
New/changed headache
Previous history of cancer
Frontal lobe function
Prefrontal area – Personality, Inhibition
Frontal - Motor function, Language production (Broca’s area)
Parietal lobe function
Sensory processing
Spatial orientation
Visual field pathway
Occipital lobe function
Visual processing
Cerebellum function
Balance, coordination
Temporal lobe function
Language comprehension
Auditory processing
Visual field pathway
Memory
Brainstem function
cranial nerve nuclei
control subconscious body functions
Focal symptoms
(progressive over days – weeks)
Weakness
Sensory loss
Visual/speech disturbance
Ataxia
Non-focal symptoms
Personality change/behaviour
Memory disturbance
Confusion
Brain tumour- seizures
21% presenting symptom
>80% patients with a brain tumour
First fit 2-6% = brain tumour
Frontal lobe- seizure
Limb jerking, head or eye deviation
Parietal lobe- seizure
Sensory disturbance – spreading tingling
Occipital lobe- seizure
Positive visual disturbance - coloured balls
Temporal lobe- seizure
Déjà vu
Jamais vu
Memories
Feeling of dread
Rising feeling
Seizure- signs
Papilloedema-
Focal neurological deficit- Hemiparesis, Hemisensory loss, Visual field defect, Dysphasia
Papilloedema
Swelling of the optic disc due to elevated intracranial pressure
Low grade brain tumour- presentation
typically present with seizures (can be incidental finding)
High grade brain tumour- presentation
rapidly progressive neurological deficit. Symptoms of raised intracranial pressure
Brain tumour- headache red flags
- With features of raised intracranial pressure (including papilloedema and VIth nerve palsy)
-With focal neurology. Check for field defect
Brain tumour- urgent referrals
New onset focal seizure
Rapidly progressive focal neurology (without headache)
Past history of other cancer
Brain tumour- 1st line investigations
Imaging- CT (with contrast), MRI
Functional MRI
Brain tumour- 2nd line investigation
Brain biopsy/surgery- Histology, molecular markers and genetics
Brain tumour- treatment
Depends on tumour type, grade and site
Treatment is non-curative (except for grade I)
Brain tumour prognosis
Brain cancer 5 year survival rate is 12%
Compare to breast cancer – 76% over 10 years
Brain tumour- treatment high grade glioma
Steroids, surgery, chemo
Radiotherapy is mainstay of treatment
Brain tumour- treatment low grade glioma
Surgery – early resection
Radiotherapy and early chemotherapy
Traumatic brain injury
Evidence of neurological dysfunction caused by external force
Traumatic brain injury- aetiology
RTC, falls (elderly), assaults, sports and recreation
Contrecoup injury
Counterblow, brain lies in CSF, collision on head causes “free floating” brain to head back of head
Primary vs secondary Traumatic brain injury
primary brain injury results from mechanical injury at the time of the trauma
secondary brain injury is caused by the physiologic responses to the initial injury
Contusions
Bruising of the brain, close to bony providences
Diffuse axonal injury
White matter lesions, high rotation or deceleration
Little haemorrhage- need MRI to see
Types of brain haemorrhage
epidural haemorrhage, subdural haemorrhage, subarachnoid haemorrhage, and intraparenchymal haemorrhage
Epidural haemorrhage
Most dangerous but if drained, good recovery
bleeding between the inside of the skull and dura mater
Subdural haemorrhage
Presents typically with milder trauma
Collection of blood under the dura mater
Subarachnoid haemorrhage
Can cause finger like extensions of blood that fill the sulci and bathe the brain as seen on CT
Accumulation of blood between the arachnoid and pia mater
Intraparenchymal haemorrhage
bleeding into the brain parenchyma proper
Secondary traumatic brain injury- treatment
Maintenance of homeostasis- (iv fluids, glucose ect)
Management of seizures- diazepam
Surgery- rarer
When to CT- presents with a head injury
Any form of clinical concern- not returned to pretty much near normal when under observations
Glasgow coma scale
Composed of three parameters: best eye response (E), best verbal response (V), and best motor response (M), 3-15, 3 being the worst and 15 the best
Chronic traumatic encephalopathy
Only found at autopsy
Thought to be linked to repeated head injuries and blows to the head
Basal ganglia disease
Hardware problem and software problems (no cell dead, problem with brain circuit)
Basal ganglia disease- hardware problems
Parkinson’s disease
Huntington’s disease
Basal ganglia disease- software problems
Essential tremor
Dystonia
Tourette
Parkinson’s disease- three cardinal features
Brady/Akinesia- (problems with buttons, writing smaller, walking deteriorated)
Tremor- (at rest, may be unilateral)
Rigidity
Spasticity vs rigidity
Spasticity- more resistance in one direction, more tone initial part of movement, velocity dependent
Rigidity- Same resistance in all directions, not velocity dependent
Spasticity vs rigidity- pathophysiology
spasticity arises as a result of damage to the corticoreticulospinal (pyramidal) tracts, rigidity is caused by dysfunction of extrapyramidal pathways
Bradykinesia
slowness of movement and speed (or progressive hesitations/halts) as movements are continued
Akinesia
inability to perform a clinically perceivable movement
Parkinson’s disease- aetiology
Cell loss in substantia nigra- Inherited factors, oxidative stress, mitochondrial dysfunction, environmental factors (risk factors, toxin induced)
Parkinson’s disease- pathophysiology
Loss of dopaminergic neurons in the substantia nigra
Parkinson’s disease- treatment overview
Non curative, to replace lost dopamine to reduce symptoms
Parkinson’s disease- treatment- L dopa
L-dopa > dopamine> dopamine receptor
Parkinson’s disease- treatment- dopamine agonist
Activate dopamine receptor like dopamine
Parkinson’s disease- treatment- COMT/MAO-B inhibitors
Inhibits Catechol-O-Methyl-Transferase + Monoamino-
oxidase- reponisble for removing the neurotransmitters norepinephrine, serotonin and dopamine from the brain
Parkinson’s and tremor on action
Unlikely to be Parkinson’s, more likely to be essential tremor
Essential tremor- signs
Postural/action tremor
No/little rest tremor
No increased tone
No problems with fine finger movements
Essential tremor- treatment
BBs, primidone
Deep brain stimulation useful
Essential tremor-pathophysiology
Software issue- the result of an abnormally functioning central oscillator
Meningitis
Inflammation of the meninges
Meninges
Outermost- dura mater
Arachnoid
Pia Mater
Causes of Meningitis- infective
Bacterial
Viral
Fungal
Parasitic
Causes of Meningitis- non infective
Paraneoplastic
Drug side effects
Autoimmune(e.g. vasculitis/SLE
Meningitis- infective routes
Via blood stream
Extracranial infection
Neurosurgical complications
Meningitis- infective- pathophysiology
Bacteria enter CSF, either transcellularly (through endothelial cells) or paracellularly (next to endothelial cells)
Infected WBCs can act as trojan horse
Meningitis- infective barriers
Blood-CSF and blood-brain barrier ordinarily protect against meningitis and encephalitis respectively
Infective meningitis- classic triad
fever, headache, neck stiffness
Infective meningitis- first line antibiotic in community
IM benzylpenicillin
Infective meningitis- 1st line investigations
Assess GCS (Glasgow Coma Score)
Blood cultures
Infective meningitis- 1st line treatment
Broad spectrum antibiotics
Steroids (IV dexamethasone)
Infective meningitis- first line antibiotic in hospital
ceftriaxone or cefotaxime
Infective meningitis- first line antibiotic- special considerations
Penicillin allergic
Immunocompromised 🡪 risk of listeria
Recent travel 🡪 risk of penicillin resistance
Infective meningitis- diagnostic investigation
Lumbar puncture and lab tests
Infective meningitis- gram Negative cocci
Neisseria
Infective meningitis- gram positive cocci
Pneumococcus (strep.pneumoniae)
Infective meningitis- bacterial clinical signs
May appear septic
Focal neurology
?purpuric rash
Infective meningitis- viral clinical signs
Recent viral illness
Less severe
Infective meningitis- TB clinical signs
Weight loss
Night sweats
Insidious onset
Infective meningitis- cryptococcal clinical signs
HIGH OPENING PRESSURE!!!- when doing lumbar puncture
Infective meningitis- bacterial RFs
Students
Travel
Immunosuppressed
Infective meningitis- Viral RFs
Small children
Immunosuppressed
Infective meningitis- TB RFs
TB contact
Immunosuppressed
Infective meningitis- cryptococcal RFs
HIV
Immunosuppressed
Infective meningitis- bacteria species- neonate
Strep B
Infective meningitis- bacteria species- child
N. meningitidis, S. pneumoniae,
H. Influence
Infective meningitis- bacteria species- adult
Neisseria meningitidis, S. pneumoniae
Infective meningitis- bacteria species- elderly
N. meningitidis, S. pneumoniae
Infective meningitis- bacteria species- immunocompromised
Listeria
Encephalitis- causes
Usually viral
-Herpes Simplex
-Varicella Zoster
Tropical
Non-infective- autoimmune, paraneoplastic
Encephalitis
Inflammation of the brain
Encephalitis- signs
Preceding “flu-like” illness
Altered GCS: confusion, drowsiness, coma
Fever
Seizures
Memory loss
(+/- meningism)
Encephalitis- investigations
MRI head
Lumbar puncture (Lymphocytic CSF, Viral PCR)
Encephalitis- treatment
Mostly supportive
Aciclovir if HSV or VZV
Tetanus
Inoculation through skin with Clostridium tetani spores (found globally in soil)
e.g. stepping on a nail, dirty wounds
Bacteria produce toxins!
Tetanospasmin
travel retrogradely along axons
Interferes with neurotransmitter release 🡪 increased neuron firing 🡪 unopposed muscle contraction and spasm
Incubation around 8 days
Tetanus- management
Vaccinate if risk injury
Supportive- Muscle relaxants
Paracetamol/cooling
Immunoglobulin to mop up toxin
Metronidazole to clear any residual bacteria
Rabies
Virus- Inoculation through skin with saliva of rabid animal (dogs, cats, foxes etc)
e.g. lick, bite, splash
Travels retrogradely along nerves
Stroke
a clinical syndrome, caused by cerebral infarction or
haemorrhage, typified by rapidly developing signs of focal and global disturbance of
cerebral functions lasting more than 24 hours or leading to death
Transient ischemic attack (TIA)
acute loss of cerebral or ocular
function with symptoms lasting less than 24 hours caused by an inadequate
cerebral or ocular blood supply as a result of low blood flow, ischemia, or embolism
associated with disease of the blood vessels, heart or blood
Ischemic stroke- aetiology
Blood vessel in the brain is blocked
Usually, an atherosclerotic plaque or a clot in a larger artery ruptures, travels
downstream, gets trapped in a narrower artery in the brain.
Embolic strokes are common complications of AF and atherosclerosis of
the carotid arteries
Haemorrhagic stroke- aetiology
Bleeding from a blood vessel within the brain.
HTN is the main cause of intracerebral haemorrhagic stroke.
Strokes- Differential Diagnosis
Hypoglycaemia Labyrinthine disorders
Migrainous aura
Mass lesions
Postictal weakness
Simple partial seizures
Functional hemiparesis
ABCD2 Score
estimates risk of stroke (CVA) after a transient ischemic attack (TIA)
ABCD2 Score- stands for
Age: >60yrs (1 point)
BP >140/90 (1 point)
Clinical features
-Unilateral weakness, 2 points
-Speech disturbance without weakness, 1 point.
Duration (60 mins< = 2 points, 10–59 mins =1 point)
Diabetes (1 point)
High risk of TIA becoming stroke
ABCD2 >3
Or AF, 1< TIA in a week, TIA on anticoagulants
TIA treatment
Low risk < 7 days, high risk <1 day
Statin, antiplatelet (clopidogrel or aspirin), treat BP, no driving until seen by a specialist
Active Rehabilitation
Facilitating recovery through modification of the neural networks
Active Rehabilitation techniques- Priming
Makes NS more receptive to rehabilitation interventions: Imagery, Touch, Transcranial direct current stimulation, Transcranial magnetic stimulation
Active Rehabilitation techniques-Augmenting
Augment the effects of rehabilitation interventions- Robotics, Biofeedback
Active Rehabilitation techniques- Specific interventions
Neurophysiological
Task Specific practice
Adaptation
Incomplete Recovery- strategies facilitate recovery of function through training, use of aids and appliances or modification of environment
Preventative rehabilitation
Reduce immobilisation: Passive range of motion exercises
Gait in neurological disorders
Weakness
Balance
Stiffness
Slowness
Walking
Heelstroke, footflat, midstance, pushoff, acceleration, midswing, deceleration
Dropped foot
Inability to activate ankle dorsiflexors in swing phase of gait
Dropped foot- pathophysiology
Lesion of CNS- corticospinal tract
Balance treatment
Vestibular exercises
Physiotherapy
Increase the base
Improve vision
Posture and slow treatment
Dopaminergic drugs
Metronomes
Spasticity
Disordered sensori-motor control resulting from an UMN lesion, presenting as intermittent or sustained involuntary activation of muscles
Headache classification- Primary
Migraine, Cluster, Tension Type
Headache classification- Secondary
Meningitis, Subarachnoid Haemorrhage, GCA, Idiopathic Intracranial Hypertension, Medication overuse headache
Brain tumour- headache red flags
New headache with Hx cancer, Cluster headache, Seizure, Significantly altered conciousness, memory, confusion, coordination, Papilloedema
Kernig’s sign
Pts is kept in supine position, hip and knee are flexed to a right angle, and then knee is slowly extended- resistance or pain >135 degree= +ive kernig sign
Positive Kernig’s sign
Meningitis
Migraine signs
Attacks last 4-72 hours
Two of the following: Unilateral , Pulsing, Moderate/severe, Aggravation by routine physical activity
Nausea and /or vomiting
Photophobia and phonophobia
Photophobia
abnormal sensitivity to light, especially of the eyes
Phonophobia
persistent, abnormal, and unwarranted fear of sound
Migraine pain
Unilateral, pulsing, Moderate/severe, Aggravation by routine physical activity
Tension type headache signs
30 mins to 7 days
Bilateral
Pressing/tightening (non pulsating) quality
Mild or moderate intensity
Not aggravated by routine physical activity
No nausea or vomiting (anorexia may occur)
No more than one of photophobia and phonophobia
Tension type headache- characteristic
Bilateral
Pressing/tightening (non pulsating) quality
Mild or moderate intensity
Not aggravated by routine physical activity
Cluster headache
Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180
minutes if untreated
Accompanied by ipsilateral cranial autonomic features +/ a sense of
restlessness or agitation
Attacks have a frequency from 1 every other day to 8 per day
Cluster headache- pain
Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 mins
Trigeminal Neuralgia- definition
intense facial pain in the distribution of the trigeminal nerve
Classical Trigeminal Neuralgia
distributions of the trigeminal nerve
Electric shock like, shooting, stabbing or sharp
Precipitated by innoculous stimuli to the affected side of the face
Migraine- acute treatment
Oral triptan + NSAID/ paracetamol
DO NOT USE OPIOIDS
Migraine- preventive treatment 1st line
topiramate or propranolol
Migraine- preventive treatment 2nd line
Amitriptyline
Meningitis signs
Drowsy, Pyrexial, Neck stiffness
Subarachnoid Haemorrhage signs
Thunderclap headache – max severity within seconds
Subarachnoid Haemorrhage- 1st line investigations
CT head
Subarachnoid Haemorrhage- management
Surgery for aneurysms
Nimodipine- CCB to prevent vasospasm
Raised Intracranial Pressure signs
Worse on waking, coughing, sneezing, straining, lying down (postural)
Nausea, vomiting
Papilloedema
Papilloedema
swelling of the optic disc due to elevated intracranial pressure
Idiopathic Intracranial Hypertension signs
Headache of raised ICP
Visual disturbance – acuity, fields
Papilloedema
Idiopathic Intracranial Hypertension RFs
Obesity
Drugs (tetracycline)
Female
Idiopathic Intracranial Hypertension investigations
CSF normal but pressure high
Imaging to exclude secondary cause and cerebral venous sinus thrombosis
Idiopathic Intracranial Hypertension management
Management of RFs (ie wgt loss)
consider pharmacotherapy
Idiopathic Intracranial Hypertension- pharmacotherapy
Acetazolamide / Topiramate/ Diuretics
Giant Cell Arteritis (GCA)
> 50 yrs
Associated with PMR
Jaw claudication
Visual symptoms
Tender temporal arteries
Raised inflammatory markers
Giant Cell Arteritis (GCA) diagnostic investigation
ultrasonography +/ temporal artery biopsy
Giant Cell Arteritis (GCA) 1st line treatment
high dose steroid
Chronic Daily Headache
Headache on ≥ 15 days per month
Chronic headache- common primary causes
Chronic Migraine, Tension-type headache, cluster headache, paroxysmal hemicranias
Hemicrania continua
New daily persistent headache
Chronic headache- common secondary causes
Medication overuse headache
Chronic post-traumatic headache
Raised intracranial pressure
Low CSF pressure headache
Chronic meningitis
Medication overuse headache- common causes
Ergotamine, Triptans, Opioids or Combination analgesic medications
Medication overuse headache
Regular use for >3 months of one or more symptomatic treatment
Headache has developed or markedly worsened during drug use
Dystonia
Prolonged muscle contraction causing abnormal posture or related movements
Idiopathic generalized dystonia
Childhood-onset dystonia often starting in 1 leg with ipsilateral progression
Genetic cause is common
Focal dystonia
Dystonia confined to one part of the body
Focal dystonia- examples
Spasmodic torticollis, blepharospasm, writer cramp
Spasmodic torticollis
head pulled to one side
Blepharospasm
involuntary contraction of orbicularis oculi (responsible for closing eyelids)
Acute dystonia
Medication induced dystonia
Acute dystonia- examples
Torticollis (head pulled back), trismus (oromandibular spasm) +/ oculogyric crisis (eyes drawn back)
Acute dystonia- treatment
diphenhydramine or benzatropine- antidopaminergic agents
Generalised dystonia- treatment
Levodopa + physio
Focal dystonia- treatment
botulinum toxin + physio
Tourette syndrome
complex neurodevelopmental disorder characterised by motor and vocal tics beginning in childhood
Tourette syndrome- RFs
Male, 3-8 yrs, PMH/FH of OCD or ADHD, FM of tourette’s
Tourette syndrome- pathophysiology
Unknown, multiple genetic loci implicated and neuroanatomical differences on MRI
Tourette syndrome- tic paradox
Tics are voluntary, but often unwanted- the desire to tic comes from the relief of the odd sensation that builds up prior
Tourette syndrome- non medical treatment
Cognitive behavioural approaches- 1st line
Tourette syndrome- medical treatment
Alpha-2 agonist, antipsychotic, botox
Epilepsy- differential diagnosis
Postural syncope, hypoglycaemia, migraine, Benign paroxysmal Positional vertigo, TIA, cardiogenic syndrome
Epileptic Seizure
Paroxysmal event in which changes of behaviour, sensation and cognition are caused by excessive, hypersynchronous neuronal discharges in the brain
Epileptic Seizures- characteristics
30-120 seconds
May occur from sleep
Stereotypical seizures / syndromal seizure types
May be associated with other brain dysfunction
Epileptic Seizures- temporal lobe
Focal impaired awareness seizure
Epileptic Seizures- Frontal lobe
Focal aware seizure
Focal seizure
Originating within networks linked to one hemisphere + often seen with underlying structural disease
Focal seizure without impairment of consciousness
Awareness is unimpaired with focal, motor, sensory, automimic or psychic symptoms
No post-ictal symptoms
Focal seizure with impairment of consciousness
Awareness is impaired- either at seizure onset or following a simple partial aura
Commonly temporal lobe- post ictal symptoms is a feature
Focal seizure evolving into a bilateral, convulsive seizure
2/3 pts with partial seizures, electrical disturbances start focally, spreads widely, causing generalized seizure, typically convulsive
Generalized seizures
Originating at some point within an rapidly engaging bilaterally distributed networks leading to simultaneous onset of widespread electrical discharge with no localizing features (to one hemisphere)
Absence seizures
Brief pauses eg suddenly stops talking mid sentence, then carries off where they left off, presents in childhood
Tonic-clonic seizures
Loss of conscience, limbs stiffen (tonic) then jerk (clonic)- may have one without the other
Post-ictal confusion + drowsiness
Myoclonic seizures
sudden jerk of a limb, face or trunk
Pts may be thrown to ground or have a violently disobedient limb- ‘flying saucer epilepsy’
Atonic (akinetic) seizures
Sudden loss of muscle tone causing a fall, no LOC
Syncope
Paroxysmal event in which changes in behaviour, sensation and cognition are caused by an insufficient blood or oxygen supply to the brain.
Syncope- characteristics
Situational
Typically from sitting/ standing- rarely sleep
Duration 5-30 seconds- recovery in 30s
Presyncopal symptoms
Cardiogenic syncope- characteristics
less warning, history of heart disease
Functional / dissociative (nonepileptic) seizure
Paroxysmal event in which changes in behaviour, sensation and cognition are caused by mental processes triggered by internal or external aversive stimuli.
Functional / dissociative (nonepileptic) seizure- characteristics
Situational
Duration 1-20 minutes
Dramatic motor phenomena or prolonged atonia, Eyes closed, Ictal crying and speaking
Rapid or slow postictal recovery
History of psychiatric illness, other somatoform disorders
Epilepsy versus syncope: Factors suggestive of epilepsy
Tongue biting, head turning, muscle pain, cyanosis (blue/grey lips), postictal confusion
Epilepsy versus syncope: Factors suggestive of syncope
Prolonged upright position, sweating prior to LOC, nausea, presyncopal symptoms (dizzy, nausea, sweating, palpitations), pallor
Epilepsy vs. Functional / Dissociative Seizures- suggestive of FDS
Very frequent, prolonged attacks
Those in which the body movements come and go
Attacks where the person is emotionally upset afterwards
Pre-ictal anxiety
Structural / metabolic (focal) epilepsy
Associated with focal brain abnormality, may start at any age
Structural / metabolic (focal) epilepsy- types of seizures
Partial seizures +/- with impairment of consciousness, secondary generalised seizures
Structural / metabolic (focal) epilepsy- first line treatment
Lamotrigine (anticonvulsant)/ carbamazepine (anticonvulsants)/ levetiracetam (anticonvulsants)
Structural / metabolic (focal) epilepsy- first line investigation
MRI- anatomical temporal lobe abnormalities
EEG can be diagnostic but a normal EEG does not exclude epilepsy
Genetic (idiopathic) generalised epilepsy
No associated brain abnormality, manifestation usually <30 years
Genetic (idiopathic) generalised epilepsy- seizure types
Absence, myoclonic, primary generalised tonic clonic seizures
Genetic (idiopathic) generalised epilepsy- first line treatment
Lamotrigine (anticonvulsant) / levetiracetam (anticonvulsant)/ valproate (anticonvulsant)
Genetic (idiopathic) generalised epilepsy- diagnostic test
EEC- electroencephalogram
Modes of action of Anti-seizure medications- presynaptic neurone
Inhibit voltage-gated Na+/ Ca2+
Increase activity of voltage-gated K+
Inhibit SV2A
Overall decrease in pre-synaptic excitability and neurotransmitter release
Modes of action of Anti-seizure medications- inhibitory neurotransmitters
Increase activity of GABA receptor
Inhibit GABA transaminase/ transporter
Overall more GABA in synapse
Modes of action of Anti-seizure medications- presynaptic neurone- Voltage gated Na+ channels
Na+ influx increases excitability and drives APs
Inhibited by carbamazepine, lamotrigine, oxcarbazepine
Modes of action of Anti-seizure medications- presynaptic neurone- Voltage gated K+ channels
K+ efflux reduces neuronal excitability, channel activity increased
Modes of action of Anti-seizure medications- presynaptic neurone- Voltage gated Ca2+ channels
Ca2+ influx drives neurotransmitter release, channel inhibited
Modes of action of Anti-seizure medications- presynaptic neurone- SV2A
Requires for release of neurotransmitter from vesicles
Inhibited by levetiracetam
Modes of action of Anti-seizure medications- inhibitory neurotransmitters- target the GABA receptor
Reduces neuronal excitability
GABA receptor activity increased by benzodiazepines, barbiturates, felbamate, topiramate
Modes of action of Anti-seizure medications- inhibitory neurotransmitters- target the GABA transaminase
Degrades GABA, inhibited to elevate GABA levels
Modes of action of Anti-seizure medications- inhibitory neurotransmitters- target the GABA transporter
Removes GABA from the synapse, inhibited to elevate GABA levels
Other epilepsy therapies
Surgery
Vagus nerve simulation
Cerebellum anatomy
largest part of the hindbrain, located in posterior cranial fossa
comprises of two hemispheres joined by the vermis- sub-divided into three lobes – anterior, posterior, and flocculonodular separated by two transverse fissures
Role of the cerebellum- main
accuracy and coordination
Role of the cerebellum
-accuracy and coordination
-motor control and learning
-contributes to timing and sensory acquisition
-involved in the prediction of the sensor consequences of action
-eye movements, speech, limb movements, fine motor skills, gait, posture,
balance, cognition
Ataxia
Sign, not a disease + results from cerebellar dysfunction (problems with balance and co-ordination) can be caused by structural damage to the cerebellum or can be inherited or acquired
Ataxia- aetiology
Inherited- autosomal dominant, autosomal recessive (Friedreich’s ataxia), X linked, mitochondrial
Acquired- toxic/metabolic (alcohol, vit def, drugs), immune mediated (gluten related) infective, degenerative, trauma, neoplastic
Symptoms of cerebellar dysfunction
-dizzy – unsteady / wobbly / clumsy
-falls, stumbles
-difficulty focusing / double vision / ‘oscillopsia’
-slurred speech
-problems with swallowing
-tremor
-problems with dexterity / fine motor skill
Nystagmus
rhythmical, repetitive and involuntary movement of the eyes
Ataxia- clinical signs
Gait is unsteady with a tendency to falls
Hand coordination is impaired
Dysarthria or dysphagia may be present
Ocular symptoms- nystagmus
Intentional tremor
Classification of ataxia
Scale for the Assessment and Rating of Ataxia (SARA)
Mild- mobilising independently or with one walking aid
Moderate- Mobilising with 2 walking aids or walking frame
Severe- predominantly wheelchair dependent
Ataxia-first line investigation
MRI brain demonstrates cerebellar atrophy and / or dysfunction
Ataxia- common blood test
For genetic testing, autoimmune screen (esp gluten related serology)
Motor neurone disease (MND)
Cluster of neurodegenerative disease characterised by selective loss of neurone in motor cortex, cranial nerve nuclei, and anterior horn cells
Motor neurone disease (MND) vs MS and polyneuropathies
Never sensory loss or sphincter disturbance in MND, unlike MS and polyneuropathies
Motor neurone disease (MND) vs myasthenia
MND never affects eye movement, distinguishing from myasthenia
Motor neurone disease (MND)- 4 types
ALS (amyotrophic lateral sclerosis or Lou Gehrig’s disease)- most common
Progressive bulbar palsy- 10-20%
Progressive muscular atrophy <10%
Primary lateral sclerosis- rare
ALS (amyotrophic lateral sclerosis)
Loss of motor neurones in motor cortex + anterior horn of the cord, so combined UMN + LMN signs
UMN symptoms
Weakness (pyramidal)
Spasticity
Hyperreflexia
LMN symptoms
Wasting
Weakness
Fasiculation
Hypotonia/flaccidity
Reflexes are reduced
Progressive bulbar palsy
disease of the nuclei of CN IX-XII
Progressive bulbar palsy- signs
Progressive, relentless
Oropharyngeal muscles
Dysarthria
Dysphasia
Jaw spasms/bruxism
Progressive muscular atrophy
Anterior horn cell lesion, so LMN signs only
Affects distal muscles groups before proximal
Better prognosis than ALS
Primary lateral sclerosis
Loss of betz cells in motor cortex
Mainly UMN, marked leg spastic leg weakness and pseudobulbar palsy, no cognitive sign
Motor neurone disease- investigations
Clinical diagnosis
Motor neurone disease- treatment overview
Improve survival and QoL
Motor neurone disease- treatment
Riluzole
Non-invasive ventilation- for Nocturnal respiratory insufficiency
Multi-disciplinary specialist care
Motor neurone disease- treatment- riluzole
inhibitor of glutamate release and NMDA receptor antagonist
Only medication to improve survival
Dementia
A neurodegenerative disease with progressive decline in several cognitive domains
20% of pop > 80 yrs
Dementia subtypes
Alzheimer’s disease, vascular dementia, Lewy body dementia, frontotemporal dementia
Alzheimer’s disease
Most common type of dementia, cognitive impairment is progressive, non-cognitive symptoms may come and go, but pts become sedentary
Alzheimer’s disease risk factors
1st degree relative, Down’s syndrome, genetics, low physical/ cognitive activity, depression, loneliness, smoking
Alzheimer’s disease pathophysiology
Atrophy in the temporal, frontal and parietal area
Senile plaques (beta-amyloid) and neurofibrillary tangles are the characteristic histopathological features postmortem
