Neuro: Part 5 Flashcards
Motor effects in primary generalised seizures
BILATERAL SYMMETRICAL and synchronous motor manifestations
What are partial (focal) seizures
These are localised to a part of one hemisphere
What causes partial seizures
Structural diseases
How can a partial seizure progress
Can become secondary generalised tonic-clonic seizures
What are tonic-clonic generalised seizures
Causes bilateral tonic and clonic muscle contractions
What is the Tonic phase of the seizure
- Consciousness lost and skeletal muscles will suddenlyy tense - patient falls
- Upward deviation of the eyes (open)
- Mouth open
How long does he tonic phase of a seizure last
10-20 seconds
What is the clonic phase of a seizure
Muscle relaxation of tense muscle from tonic and re-contraction - convulsions
Bilateral,, rythmic muscle jerking
Clinical presentation of primary generalised tonic-clonic seizure
- Often no AURA stage (sudden progression to post-itically)
- Loss of consciousness
- Eyes open
- Tongue bitten
- Incontinence/faeces
- Drowsiness, coma or confusion post-itically
- Characteristic groan as they fall to ground
What is petit mal
Generalised seizure in which there is brief loss and return of consciousness and NO POSTICTAL state
When is petit mal seen
Children
Clinical presentation of petit mal
- Child ceases activity, stares and pales (stops talking and then carries on)
- Unawaree of attack
Diagnostic of petit mal
EEG - shows 3-Hz spike
How does petit mal progress as a condition as child becomes adult
Develop generalised tonic-clonic seizures in adult life
What are myoclonal seizures
Seizures in myoclonic families - those who have brief, involuntary twitching of a group of muscles
Isolated jerk of limb, face or trunk
Clinical presentation of myoclonic seizure
Patient thrown suddenly to the ground or have a disobedient limb
What are tonic seizures
Sustained increase tone with a groan as they fall to the ground
Intense stiffening of body
What are tonic seizures not followed by jerking
Because there is no clonic phase
What is an atonic seizure
Sudden loss in muscle tone and cessation of movement - fall to th aground
What are simple partial seizures
- Focal serisures that do not effect consciousness or memory
- Asymptomatic and no post-octal symptoms
What are complex partial seizures
- Affect awareness and memory before, during or immediately after seizure
Where do complex partial seizures occur
Usually in the temporal lobe (speech, memory and emotion)
Post-Ictal signs of complex partial seizures
Confusion
In which complex partial seizure is recovery more rapid
Temporal and frontal lobe
What happens to a partial seizure in 2/3 of patients
Becomes secondary generalised seizure - conclusions seen
Clinical presentation of complex partial seizures in the temporal lobe
REMEMBER: effects memory, emotions and speech understanding
1. AUTO: Deja vu, auditory hallucinations, funny smell and fear
Anxiety or out of body experience, automatisms (chewing fiddling etc)
Clinical presentation of complex partial seizures in the frontal lobe
REMEMBER: Motor and though processing
- Posturing or peddling movements of leg
- Jacksonian march (seizure goes up and down motor homunculus)
- Post-ictal todd’s palsy - paralysis of limbs involved in seizure for a few hours
Clinical presentation of parietal lobe focal seizure
- SENSORY DISTURBANCES - tingling/numbness
Clinical presentation of occipital lobe focal seizure
Visual phenomenas (lines, flashes or spots)
How do we distinguish epilepsy over syncope
- Tongue biting
- head turning
- Muscle pain
- Loss of consciousness
- Cyanosis
- Post-ical symptoms
How do we distinguish syncope from epilepsy
- CAUSES: Prolongues standing, sweat prior to unconsciousness and nausea
What causes non-epileptic seizures
Situational dependnat
Signs of epileptic vs non-epileptic seizures
- Non: Longer, close mouth and eyes during tonic-clonic movements and pelvic thrusting - NO INCONTIENCNE OR TONGUE BITING
Sleep is not a factor
PRE-ICTAL ANXIETY symptoms in non
Differential diagnosis of epilepsy
- Postural syncope
- Cardiac arrhythmia
- TIA
- MIGRAINE
- Hyperventilation
- Hypoglycaemia
- Panic attacks
- Non-epileptic seizure
Rule of thumb when diagnosing epilepsy
- Has to be at least 2 or more unprovoked seizures occurring more than 24 hours apart - HISTORY taking
What diagnostics are sued for seizures
- EEG
- MRI
- CT Head
- FBC
- Genetic testing
Why is an EEG done for seizures
NON-diagnostic but supports diagnosis
Seizure type and what epilepsy syndrome seen
3Hz spike
Why is an EEG not diagnostics
Becomes normal between attacks
Role of MRI in seizures
Imaging of hippocampus
Role of hippocampus
Short-term to long-term memory
Spatial memory for navigation
Role of CT in seizures
- SPACE OCCUPYING LESIOSN (tumour caused seizure??)
2. Structural abnormalities
Role of FBA in seizures
- Hypoglycaemia
- RENAL and liver function tests
- Rules out comorbities
When is genetic testing useful for seizures
Myoclonic epilepsy (remember, family dependant)
How is seizures treated when not knowing the actual type of seizure present
- ABCDE
- Glucose monitor
- DIAZEPAM (rectally or IV)
- IV PHENYTOIN (prophylaxis anti-epileptic drug)
——2nd line——-
Anaesthetist involvement and ventilation
When should drugs for seizure not be given
When it is one-off
Treatment of generalised tonic-clonic seizure
ORAL SODIUM VOLPROATE (teratogenic)
ORAL LAMOTRIGINE
ORAL CARBAMAZEPINE
Side-Effect of generalised tonic-clonic seizure
Weight gain
Hair loss
Liver failure
Side effects of oral LAMOTRIGINE
Maculopapular rash
Blurred vision
Vomiting
Side effects of ORAL CARBAMAZEPINE
Diplopia Rashes Leucopenia Impaired balance Drowsiness
What are maculopapular rash
Flat red area on skin with small bumbs
Treatment of petit mal
- ORAL SODIUM VALPROATE
- ORAL LAMOTRIGINE
- ORAL ETHOSUXIMIDE
Side effect of ORAL ETHOSUXIMIDE
Rashes
Night terrors
How are partial/focal seizures treated
- CARBAMEZEPINE
- SODIUM VALPROATE
- LAMOTRIGINE
Surgical procedures for seizures
- Surgical resection of tumour
2. VAGAL NEVRE STIMULATION - reduces seizure frequency
What fatal condition can repeated seizures result in
Status epilepticus (MEDICAL EMERGENCY)
What is status epileptics
Continuous seizures without recovery of consciousness
Risks if we get grand-map one after another
risk of death from CV failure
What causes status epileptics
- Stopping preventative treatment
- ALovhol abuse
- Poor compliance to therapy
Lifestyle changes to patients with epilepsy
MUST INFORM DVLA - can’t drive until a year without seizures
Do not swim alone and avoid dangerous sports (rock-climbing)
What is SUDEP
Sudden unexpected death epilepsy: Nocturnal apnoea or asytole during night
Define dementia
- A syndrome caused by a number of brain disorders which causes memory loss, difficulties with thinking, problem-solving or language as well as difficult ire with activities of daily living
What is the most common form of dementia
Alzheimer’s
What structures are damaged in alzheimer’s
Causes degeneration of cerebral cortex with cortical atrophy
Pathophysiology of alzheimer’s
1, Accumulation of beta-amyloid peptides = progressive neuronal damage, neurofibrillary tangles increase sin number of amyloid plaques causes loss of ACh
What is vascular dementia
Brian damage due to cerebrovascular disease (stroke, multi-infarcts or changes in small vessel structure)
How do we diagnose vascular dementia
Raised BO
Past strokes
Focal CNS signs
What is lewy-body dementia
Deposition of abnormal proteins within neurone in brainstem and neocortex
Clinical presentation of lewy body dementia
Fluctuating cognitive impairment Visual hallucinations Impairment to frontal lobe Parkinsons Loss of inhibition Depression Variation in attention and alertness
What disease is lewy body dementia associated with
PARKINSON’s
What is fronts-temporal (pick’s) dementia
- Degeneration and atrophy of frontal and temporal lobes
Clinical signs of fronts-temporal dementia
- BEHAVIOURAL and personality change
Early preservation of episodic memory and spatial orientation - Emotional unconcern
- Lower inhibition
THIS IS A FORM OF MIXED DEMENTIA as it effect many places
Rarer forms of dementia
- Liver failure caused
- HIV caused
- Prion disease (CREUTZFELDT-JAKOB)
- Vitamin B12/folate deficiency
- HUNTINGON’s
Risk factors for dementia
- AGE
- FAM HISTORY
- DOWN SYNDROME
- Alcohol
- High BP
- Diabetes
- Atherosclerosis
- Depression
- High oestrogen levels
Clinical presentation of alzheimer’s
- Short-term emory loss early symptoms
- Personality deterioration
- Intellect gone
- Decline in speech
- Visuospatial skills
- Apraxia
- Agnosia
Define agnosia
Failure to recognise objects
Define apraxia
Impaired ability to cary out skilled motor tasks
Differential diagnosis of dementia
- Substance abuse (Alcohol)
- Hypothyroidism
- Space-occupying lesions intracranial
- Huntington’s
How is dementia diagnosed
- HISTORY - cognitive function assessment
- MMSE (mini mental state examination
- Exclusion of rare causes of dementia (substance abuse, vit B12)
- FBC Liver biochemistry, B12, thyroid function
- Neuropsychology
- Brain CT in younger patients
- MRI (atrophy)
- Brian functions
How is brain function assessed in dementia patients
PET and SPECT scanning - blood supply
Functional MRI - brain networks
How doe the Mini Mental State Examination work
Score of 25 or above out of 30 normal
18-24 = mild impairment
17 = serious impairment
How is dementia treated
- SUPPORT
- LIFESTYLE CHANGES (preventative)
- MEDICATIONS
What support can we give dementia patients
- Talking to far and friends
- Board games - stimulation
- Specialist memory service
- CARERS
Preventative therapy for dementia
- Helath behaviour
- Smoking cessation, diet and low alcohol
- 6 leisure activities a week = lower risk
- Education, mental activities and occupation decreases risk
- Changes in amyloid-beta are seen 25 years before onset (early monitoring)
What medications can be given for alzheimer patients
- ORAL DONEPEZIL or RIVASTIGMINE (ACh inhibitors - increases ACh)
- RAMUPRUL - vascular dementia (prevents worsening)
Define Parkinson’s Disease
- Degenerative movement disorder caused by reduction in DOPAMINE IN SUBSTANTIA NIGRA
What characterises Parkinsons
- RIGIDITY
- BRADYKINEASIA (slow to execute movement)
- Resting tremor
When is peak onset of parkinson’s
55-65
What gender does Parkinson’s effect
MALES
Risk factors of Parkinson’s
- Male
- Increasing age
- Family History
- Being a non-smoker
- Drug induced
- Environmental factors
What environmental factors can induce parkinson’s
1, Pesticides
MPTP - opiates
Mitochondrial dysfunction + Oxidative stress!!!!
How do alterations in genes cause parkinson’s
- Mutation in parkin and alpha-synuclein gene
What forms the basal ganglia
- STRIATUM
- GLOBUS PALLIDUS
- SUBSTANTIA NIGRA
- SUBTHALAMIC NUCLEUS
Pathophysiology of Parkinson’s
- MITOCHONDRIAL DYSFUNCTION AND OXIDATIVE STRESS causes progressive degeneration of dopaminergic neurone from pars compacts of Substantia nigra that project to striatum
- Reduced striatum dopamine levels
- Thalamus is more inhibited = parkinson’s
How does Parkinson’s worsen
LEWY-BODIES become more widespread from lower brainstem to midbrain and then into cortex
How can we tell the severity of parkinson’s
extent of dopaminergic cell loss correlated with degree of akinesia (muscle rigidity, stiffness and lack of responsiveness)
What are lewy-bodies made of
ALPHA-SYNUCLEIN and UBIQUITIN
Clinical presentation of Parkinson’s
INITIAL:
Impaired dexterity and unilateral foot drop
ASSYMETRICAL
Anosmia (reduced sense of smell) Depression Aches and pain REM sleep disorders Urinary urgency Hypotension and constipation
LATER:
TREMOR
RIGIDITY
BRADYKINESIA
When are tremors in parkinson’s worse
REST
Where are tremors seen
ASYMMETRICALLY in hands
What makes tremors better
Voluntary movement
What makes treours worse
Anxiety
How to distinguish parkinson’s from cerebellar trmeours
4-6 cycles a sec
Repetitive hand movement worsens in rhythm the longer attempted
Why do we get rigidity in Parkinson’s
We get lesions in extrapyramidal tract (increased muscle tones in limbs and trunk)
Clinical presentation of rigidity in Parkinson’s joint
- Limbs resist passive extension throughout movement (does not go away like in clasp-knife phenomenon)
- Pain and problems in bed
Characteristics of Bradykinesia in Parkinson’s
- Slow to initiate movement of repetitive actions:
REDUCED LINK RATE
MONOTONOUS HYPOPHONIC SPEECH
MICROGRAPHIA (writing smaller) - Changes in gait
- Expressionless face (hypo mimesis)
Characteristics in Gait in Bardykinesia
- REDUCED ASYMMETRICAL ARM SWING
- NARROW GAIT
- STOOPED POSTURE and SMALL STEPS
- FESTINANCE
What is Festinance
- Shuffling steps
2. Dragging foot with flexed trunk
Facial characteristics in Parkinson’s
- Drooling of saliva
- Swallowing difficulty (late event)
- Aspiration pneumonia
- Depression
- Constipation
- Urinary frequency increased
Differential diagnosis of Parkinson’s
- Benign essential tremor
- LEWY BODY DEMENTIA
- Drug-induced
- WILSON’s disease
- Trauma
- Dopamine antagonists
How is benign essential tremor treated
- PROPRANOLOL (contraindicated in diabetes)
2. PRIMIDONE (anti-seizure) or GABAPENTIN (anti-epileptic)
How is Parkinson’s diagnosed
- HISTORY
- Response to LEVODOPA
- Dementia, incontiennce, symmetry and early falls
How is Parkinson’s treated
- Compensate for loss of dopamine
- ——PRIMARY——–
1. Balance
2. Speech
3. Gait
Non respond to medication so PHYSIOTHERAPY + Physical activity
- ——-SECONDARY——
1. ORAL LEVODOPA with decarboxylase inhibitor (CO-CARELDOPA)
Why is LEVODOPA given and not dopamine
L-DOPA is a precursor which can cross BBB
Why is decarboxylase inhibitor given alongside LEVODOPA
Prevents peripheral conversion of L-DOPA (maximises dose that can cross BBB and reduces peripheral side-effects)
Side-effects of LEVODOPA
- NAUSEA
- VOMITING
- ARRYTHMIAS
- PSYCHOSIS
- VISUAL HALLUCINATION
Motor complications of LEVODOPA
- Reduced efficacy over time even if dose is increased
- ON-dyskinesias (hyperkinetic, choreiform movement - rapid, repetitive and jerky involuntary movements)
- OFf-dyskinesias (fixed, painful dystonic posturing (twisting and repetitive movements or abnormal fixed postures - sustained muscle contraction)
- FREEZING
How long doe sit take for LEVODOPA to stop working
5-10 years
What can be given instead of LEVODOPA so we can use it as a later treatment
- MAO-B and COMT inhibitors - dopamine antagonists
Does LEVODOPA alter disease progression
NO - symptom relief
Name some DOPAMINE ANTAGONIST
- ORAL ROPINIROLE
2. ORAL PRAMIPREXOLE
Side-Effects of dopamine antagonist
- Drowsiness
- Nausea
- Hallucination
- Compulsive behaviour
What dopamine antagonist should we avoid
Bromocriptine and cabergoline - cardiac valvulopathy
Name an MAO-B inhibitor
ORAL SELEGILINE or ORAL RASAGILINE
How do MAO-B inhibitors work
Inhibit MAO-B which breakdown dopamine = reduction of dopamine breakdown so dopamine remains for longer
Side-Effect of MAO-B inhibitor
Postural hypotension and AF
Name a COMT inhibitor
- ORAL ENTACAPONE or TOLCAPONE
Role of COT inhibitr
Breaks down dopamine
Side-effect of COMT inhibitor
TOLCAPONe causes liver damage
Ho do we treat neuropsychiatric complications
ORAL CITALOPRAM = SSRIs
2. ORAL QUETIAPINE (anti-psychotics)
Surgical treatments of Parkinson’s
- DEEP BRAIN STIMULATION to help those who are partially-dopamine responsive
- SURGICAL ABLATION o overreactive basal ganglia circuits (sub thalamic nuclei)
