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Medicine year 4 CDM > Case 7: Epilepsy, Hyperkinetic, GBS > Flashcards

Case 7: Epilepsy, Hyperkinetic, GBS Flashcards

1
Q

Syncope features

A
  • Aura: Feeling light headed
  • Flops to floor
  • Appears: Pallor or ashen faced
  • Eyes closed
  • Couple of brief jerks possible
  • Floppy position
  • May have rapid breathing
  • Rapid recovery, orientated
  • Amnesic for a brief period, possibly seconds
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2
Q

Seizure features

A
  • Aura: deja vu, focal SM symptoms possible
  • Stiffens ‘tonic phase’
  • Appears: red/blue
  • Often eyes open
  • Sustained convulsion
  • Classically UL flexed, LL extended
  • Tonic cry, grunting then snore post ictal
  • Prolonged recovery, amnesic phase
  • May have no recollection for hours after events
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3
Q

Non-epileptic attack

A
  • May be emotionally upset, environment may trigger
  • Normal colour unless breath holding
  • May resist eye opening
  • Waxing and waning arrhythmic movement
  • May have thrashing or irregular movement
  • Variable, may be tearful or breath holding
  • Variable recollection
  • Often full awareness including during event but disassociated from surroundings so cant respond
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4
Q

Types of generalised seizures and Todd’s paresis

A

Generalised seizures: tonic clonic, absence, myoclonic, atonic
Todd’s paresis: post ictal weakness especially after focal seizure

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5
Q

What happens in a tonic-clonic seizure

A
  • Limb stiffens then rhythmically jerks
  • May be accompanied by loss of consciousness, biting of side of tongue, incontinence, post ictal confusion
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6
Q

What happens in different types of seizures

A
  • Absence: behavioural pause, usually less than 10s
  • Atonic: sudden loss of tone (limpness) and loss of consciousness
  • Myoclonic: sudden jerk of bilateral limbs, face or trunk
  • Simple focal seizure: jerkiness in distal limb which spreads
  • Complex focal: odd behaviour (wandering), hallucinations, delusions
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7
Q

The different childhood epileptic syndromes

A
  • benign rolandic
  • infantile spams/ West syndrome
  • juvenile myoclonic epilepsy
  • Lennox gastaut
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8
Q

Benign rolandic epilepsy: childhood seizure

A
  • focal motor seizures involving one side of face or muscles involved in speech and swallowing
  • presents at night
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9
Q

West syndrome: childhood epilepsy

A
  • repeated flexion followed by extension of arms
  • presents age 4-8 months
  • usually affects those who have suffered brain damage or have genetic abnormalities
  • poor prognosis
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10
Q

Juvenile myoclonic epilepsy presents: childhood epilepsy

A
  • upper body mycoclonic seizure or tonic clonic seizure
  • Presents when child wakes up
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11
Q

Investigations for epilepsy

A
  • rule out non-epileptic causes of seizures: glucose, U+Es, ECG
  • EEG
  • consider MRI
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12
Q

Side effects of anti-epileptics

A
  • General: sedation, dizziness, suicidal thoughts, psychosis, weight changes, osteoporosis
  • may cause elevated LFTs
  • carbamazepine may cause mild leukopenia
  • sodium valproate may cause thrombocytopenia
  • Carbamzepine reduces effectiveness of contraception except UID/IUS
  • Lamotrigine- use progesterone only contraception
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13
Q

How long after first seizure can patients not drive for

A
  • 6 months if investigations normal
  • 12 months if structural abnormality or abnormal EEG
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14
Q

Vasovagal syncope: causes and management

A
  • Causes- pain, unpleasant sensation, orthostatic, prolonged recumbency
  • Mangement- Lie flat, reassurance
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15
Q

Epilepsy investigations

A
  • EEG- doesnt exclude epilepsy, helps assess risk of reoccurence
  • ECG- in case its an arrhyhtmia (check QTc)
  • Observation
  • Basic bloods including glucose
  • Neurological and cardiovascular exam
  • Consider brain imaging i.e. head injury, neurological abnormality, Todds paresis

After 2 or more seizures you scan and treat

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16
Q

Risk factors for recurrent seizures

A
  • Remote symptomatic seizure
  • Neurological defecit
  • Seizure whilst asleep
  • Abnormal EEG
  • Abnormal MRI
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17
Q

Management of status epilepticus

A
  • Early onset 5-10 mins- Lorazepam (Benzo diazepam)
  • Established status 10-30mins- Valproate/ Phenytoin/ Levetiracetam
  • Refractory status >30 mins- Propofol/ Midazolam/ Thiopentone
  • Lots of epipilepsy medication is teratogenic (valproate) and shouldnt be given to anyone of child bearing age
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18
Q

SUDEP

A
  • Sudden unexplained death in epilepsy with no clear cause
  • Risk factors- male, poor compliance, generalised seizures, nocturnal seizures, LD, pregnancy
    Management mainly around managing epilepsy and education/awareness
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19
Q

Causes of seizures

A
  • Generalised seizures are due to global problems with the brain i.e. probably genetic
  • Focal seizures are due to local problems i.e. stroke, tumour, encephalomalacia secondary to trauma
  • Provoked seizures i.e. metabolic (glucose, sodium), head injuries, drugs, alcohol withdrawal
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20
Q

Causes of recurrent seizures in epilepsy

A
  • Drug resistant epilepsy (1/3 all patients)
  • Non compliance
  • Alcohol/drugs
  • Sleep deprivation
  • Changes in AED levels e.g. pregnancy, enzyme inducing meds
  • Infection
  • Wrong diagnosis
  • Development of another diagnosis e.g. syncope
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21
Q

Classical features of GBS

A
  • A preceding respiratory or gastrointestinal infection 2-3 weeks before the presentation
  • Initial presentation with paraesthesia in the lower limbs but no objective sensory loss
  • Symmetrical LMN pattern of ascending weakness that is affecting the lower limbs more than the upper limbs and areflexia
  • Progression is rapid and acute (over a few days) but not hyper-acute (which would suggest a vascular cause).
22
Q

GBS pathophysiology

A
  • Immune process that attacks the myelin sheath
  • Autoimmune process due to molecular mimicry
  • Can have cranial nerve involvement (diplopia), autonomic features: urinary retention, diarrhoea
  • Most common trigger: Campylobacter jejuni
  • Most common varient: acute inflammatory demyelinating polyneuropathy (AIDP)
23
Q

Timeline of GBS

A
  • Present with mild paraestheia in lower limbs: no sensory deficit on examination
  • Progressive weakness starts in lower limbs and progresses to upper
  • Flaccid paralysis with areflexia
  • Back or leg pain
  • Later on: bowel and bladder symptoms
  • Peaks at 2 weeks then plateaus then dramatic recovery at least 28 days after onset
  • Return to daily function but come residual deficit i.e. fatigue, weakness and pain
  • Mortality is due to respiratory muscle involvement and neuromuscular respiratory failure: have regular FVC- causes T2RF
24
Q

Investigations for GBS

A
  • Bloods: FBC, U&E, LFT (abnormal), CK (mildly raised), CRP, Calcium, anti-ganglioside antibodies (anti-GM1)
  • Lumbar puncture: shows albuminocytologic dissociation (elevated proteins in CSF with little or no white cells)
  • Nerve conduction studies (NCS)/electromyography (EMG): localises pathology to peripheral nerves and shows patchy demyelination
  • Findings on lumbar puncture and NCS/EMG are present after 7 days
25
Q

Treatment for GBS

A
  • Plasma exchange (PLEX) or intravenous immunoglobulins, dont use in combination
  • Catheterisation if urinary retention
  • Laxatives for constipation (due to immobilisation or autonomic issues)
  • Prophylactic anticoagulants if immobilised
  • Nutritional support by NG feeding
  • IF FVC <20 ml/kg tend to progress to respiratory failure- consider ICU admission or NIV
  • Long term: neurorehabilitation including physiotherapy and occupational therapy
26
Q

Poor prognostic features for GBS

A
  • age > 40 years
  • poor upper extremity muscle strength
  • previous history of a diarrhoeal illness (specificallyCampylobacterjejuni)
  • high anti-GM1 antibody titre
  • need for ventilatory support
  • Around 5% of patient die and 15% have severe motor problems
27
Q

Bedside tests for GBS

A
  • stool culture to look for recent C jejuni
  • ECG to look for arrhythmiaregular
  • spirometry to assess FVC
  • breath count (deep breath in then count out loud when breathing out)
28
Q

Hyperkinetic movement spectrum

A

Athetosis → Dystonia → Chorea → Ballismus → Tic → Myoclonus

29
Q

Athetosis and dystonia

A

Athetosis: writhing movement, seen in Huntington

Dystonia: fixed abnormal posture, can be jerky. Slow, sustained co-contraction of agonist and antagonist muscles to produce a twisted postureof a body part or region.It repeatedly involves thesamegroupofmuscles

30
Q

Chorea

A

Dance like flitting movement. Huntington’s. Restless, agitated. Often try to disguise with normal movement. Involuntary,irregular,purposeless,nonrhythmic, rapid andunsustained movements which seem to flow randomly from one body part to another; they are unpredictable.

31
Q

Ballismus and TICS

A

Ballismus: flinging movement, normally hemiballismus (occurs on one side). Normally due to lesion in basal ganglia or stroke, hypoglycaemia

Tics: movement or speech. Can be mixed with psychological issues like OCD i.e. doing something 3 times. Caused by tourettes. Abnormal, stereotypic repetitive movement (motor tics) or abnormal sounds (phonic tics) which can be suppressed temporarily but may need to be released at some point

32
Q

Myoclonus

A

Rapid, sudden movement. Causes myoclonic epilepsy, ischaemic brain injury (Lambs-abert syndrome- treat with anti-epileptic drugs), drug effect- opiates, pregabalin, amitriptyline. Sudden, brief, shock like jerks are caused by muscular contraction (positive myoclonus) or inhibition (negative myoclonus, such as asterixis).

33
Q

Tremor and functional movement disorders

A

Tremor: An oscillatory usually rhythmic (to and from) movement of one or more body part such as the neck, tongue, chin or vocal cords or a limb

Functional movement disorders: can be any presentation. Can be any speed but variable and inconsistent, tend to be distractable. However, some non functional disorders can have several presentations like myoclonus and dystonia

34
Q

Describing a tremor

A
  • Body distribution: What limb(s) body regions are affected? Is it symmetrical?
  • Frequency: How fast is it? Does it appear fine (rapid) or coarse (slow)? Hertz (Hz) is numbers of cycles of a tremor in 1 second
  • Amplitude: how big is it?
  • Chronicity: How long has the tremor been there? How has it evolved?
  • Activation conditions: What makes the tremor worse? What brings it on? Postural (Body or limb held in position against gravtiy). Kinetic (with a particular movement)
  • Any further neurological symptoms? Such as bradykinesia (parkinsonian) etc.
  • Genetic/Drug-Induced clues: Is there a family history of fine tremor? (Essential Tremor), Does it get worse with salbutamol? (Physiological Tremor)
35
Q

Types of tremor

A
  • Physiological tremor:Fine, rest, low amplitude tremor worsened by caffeine, anxiety, salbutamol etc.
  • Essential Tremor:Inherited postural tremor, fine-coarse, moderate amplitude bilateral upper limb tremor, can affect head and voice, most noticeable on daily activities such as drinking from a cup
  • Psychogenic (Functional) Tremor:Variable features, abrupt onset, spontaneous remission, distractable
  • Cerebellar Tremor:Coarse, Intention tremor that increases with amplitude on reach, can be unilateral
36
Q

Huntingtons genetics

A
  • Autosomal Dominant inheritance
  • Trinucloetide (CAG) repeat mutation in the Huntingtin gene on the short arm of Chromosome 4: codes for glutamine
  • Generally high penetrance
  • Demonstrates Anticipation (younger age of onset with successive generations, increase in repeat number): more profound in males
  • Mutation leads to Huntingtin protein clumping which leads to caudate and putamen degeneration
37
Q

Causes of chorea

A

Drug induced in idiopathic Parkinsonism (dyskinesia), Basal ganglia stroke, meningitic infections, post-infectious autoimmune issues (Sydenham’s Chorea- post rheumatic fever) and electrolyte imbalances

38
Q

Presentation of Huntingtons

A
  • Chorea(involuntary, random, irregular and abnormal body movements)
  • Dystonia(abnormal muscle tone, leading to abnormal postures)
  • Rigidity(increased resistance to the passive movement of a joint)
  • Eye movement disorders
  • Dysarthria(speech difficulties)
  • Dysphagia(swallowing difficulties)
  • Motor impersistince: patient can sustain simple voluntary act
  • Saccadic eye movement
39
Q

Early phase of Huntingtons

A
  • Motor: involuntary distal movements/twitches, reduced co-ordination
  • Psychological: apathy, trouble with complex tasks
  • Behavioural: personality change, irritability, disinhibition, mood disorders
40
Q

Middle phase of Huntington’s

A
  • Motor: chorea, impaired voluntary movement, impaired gait and balance, eye movement disorders
  • Psychological: impaired executive function, memory loss
  • Behavioural: increasing severity, can manage some ADL’s
41
Q

Late phase of Huntington’s

A
  • Motor: Severe motor impairement, Bradykinesia/rigidity, falls, dysphagia/dysphasia, bladder/bowel issues
  • Psychological: cognitive impairement, can experience psychosis
  • Behavioural: reduced severity, cannot manage ADL’s
42
Q

Management of Huntington’s

A
  • Tetrabenazine for chorea
  • Antipsychotics for psychiatric symptoms
  • Antidepressants i.e. SSRI for depression
  • Physiotherapy to maintain joint therapy
  • Advanced directives and end of life care
  • Genomic counselling: decide whether to undergo genetic testing
43
Q

Effects of genomic counselling: Huntington’s

A
  • Emotional wellbeing
  • Genetic implications for family members
  • Decisions about having children
  • Career opportunities
  • Life insurance
44
Q

Huntington’s prognosis

A

Huntington’s chorea is a progressive condition. Life expectance is around 10-20 years after the onset of symptoms. As the disease progresses, patients become more frail and susceptible to illness (e.g., infections, weight loss, falls and pressure ulcers). Death is often due toaspiration pneumonia.Suicideis also a common cause of death.

45
Q

Tardive dyskinesia

A
  • Due to long term use of anti-dopaminergic agents, due to increased dopamine sensitivity
  • Typically involves facial movements: grimacing, lip smacking and pursing, tongue movements, choreaform movement
46
Q

Dyskinesia in Parkinson’s

A

On long term dopamine replacement. Essentially patients are given too much dopamine and cause chorea at peak concentrations.

47
Q

Neuroepileptic Malignant Syndrome (NMS) and Serotonin Syndrome

A
  • Severe autonomic dysfunction (Hyperthemia, Hypertension, Tachycardia)
  • Neurological abnormalities (includcing reduced conscious level)
  • NLMS classically has longer onset and is associated with hyporeflexia and lead pipe rigidity
  • Patients with DLB (dementia with lewy bodies) are very sensitiveto antipsychotics and are predisposed to NMS. Initiation of antipsychotic medication in DLB patients should be discussed with their neurologist/physician.
  • Serotonin Syndrome has a more abrupt onset and is associated with hyperreflexia, increased tone and clonus, as well as dilated pupils and hyperactive bowel sounds
48
Q

Serotonin syndrome

A
  • Causative drugs: Serotonergic drugs i.e. SSRI, SNRI, tramadol, cocaine
  • Speed of onset: Rapid (<1 day)
  • Course: resolves rapidly
  • Tone: increased tone, tremors + clonus
  • Increased reflexes
49
Q

Neuroepileptic malignant syndrome

A
  • Causative drugs: Dopamine antagonists i.e. anti psychotics, metoclopramide
  • Speed on onset: gradual (1-7 days)
  • Gradual course
  • Tone: Diffuse lead pipe rigidity
  • Decreased reflexes
50
Q

Management of NMS and SS

A
  • Stop offending agent
  • Supportive (IV fluids, cooling, addressing any further metabolic or electrolyte abnormalities)
  • In severe NMS give Bromocriptine (dopamine agonist) and dantrolene (muscle relaxant)
  • In severe SS give Benzodiazepines

Medicine year 4 CDM (44 decks)

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