19/11/2014

Referrals

Seizures, Syncope and Strange Episodes

Collapse  Loss of consciousness  Loss of awareness ‘vacant’  Loss of interaction  Limb/body shaking  Involuntary movements  Talking ‘rubbish’ 

Dr Russell Hewett Consultant Neurologist

Diagnosis

Differential Preliminary Diagnosis



Epileptic seizure

Seizure/Epilepsy

– generalised or focal (+/- secondary generalisation) 

Syncope (vasovagal, postural, cardiogenic)



Other physiological:

Syncope ?Seizure Other 0

– Metabolic (hypoglycamia) – Sleep disorder – Transient Ischaemic attack

5

10

15

20

Preliminary diagnosis

6

9

GTC seizure

14

35

4

Syncope

Psychological

?seizure

– Pseudo-seizures or psychogenic non-epileptic attacks – Hyperventilation, panic attacks Other

35

%

Focal seizure Alcohol related



30

n

Epilepsy Seizure

25

5 14

22

?seizure

12

26

seizure vs syncope

4

seizure vs NEAD

1 5

8

Diagnosis Preliminary Diagnosis

History

Diagnosis after 1 year Seizure/Epilepsy

Seizure/Epilepsy

Syncope

Syncope

?seizure ?Seizure

Death

Other

Other 0

5

10

15

20

Preliminary diagnosis GTC seizure Focal seizure Alcohol related Syncope ?seizure

Other

30

n

Epilepsy Seizure

25

35

%

6

9

14

35

4 5 14

22

?seizure

12

26

seizure vs syncope

4

seizure vs NEAD

1 5

8

0

10

20

30

After 1 year Epilepsy (started meds) Single seizure and abnormal EEG Single seizure Alcohol-related Syncope ?seizure ?NEAD ?seizure vs syncope Death Other/Not Epilepsy

40

n 13 3 12 7 15 4 2 3 1 5

% 20 5 29 24 13

Examination

Investigations

1 8

1

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History 

History from patient



History from eye witness - VITAL



Need detailed history of: 1. 2. 3. 4.

Epileptic seizures

Background and provoking factors Preceding symptoms Description of attack What happened after event

Semiology

Definitions Seizure 

the clinical manifestation of an abnormal, excessive excitation and synchronization of a population of cortical neurons

Epileptic seizures Epilepsy



two or more recurrent seizures unprovoked by systemic or acute neurologic insults



Generalised – Tonic – Clonic



Focal – Depends on origin of

seizure

– Tonic-Clonic – Atonic – Myoclonic

+/- impaired consciousness +/- becoming generalised

– Absence

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1. Background/Provoking Factors   

   

Previous history of seizures Family history of epilepsy Structural abnormalities: – Birth injury; head injury; stroke; meningitis; encephalitis; neurosurgery Febrile convulsions in childhood Myoclonic jerks in / since teens Change in alcohol, drugs, sleeping tablets Sleep deprivation

2. Preceding symptoms 

Generalised: – Usually no warning



Focal – ‘Aura’ – Strange taste, smell – Déjà-vu, jamais-vu – Fear – Ascending unusual feeling – Sensory changes

Focal - localisation

3. Description of attack - Witness Focal 

Automatisms – Oro-facial (lip smacking) – Manual (picking, stroking)



Head and eye deviation Limb dystonia/jerking Reduced interaction or responsiveness



Duration

 

– Temporal lobe seizures 2-4 minutes – Frontal lobe seizures 10-20 seconds

3. Description – Tonic-clonic seizure:

4. After event 



Body stiff, rigid, crashing to ground Often letting out loud scream From rigid to rhythmical limb jerking (not tremor) Colour purple/ blue (as if dead) Eyes often open (rolled back)



Lasts 1-2 minutes, then jerking slows down



Often heavy breathing, snoring Possibly incontinence

   



Witness: – Non-responsive for some minutes – Often post-ictal confusion – Dysphasia, Todd‘s palsy (if focal onset)



Patient: – – – – –

May not recall events or extremely delayed Headache Achey all over (myalgia) Goes to sleep it off Tongue biting (?)

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Detailed History

Syncope

1. Background and provoking factors 2. Preceding symptoms 3. Description of attack (from witness) 4. What happened after event

Syncope – Background and provoking factors 

Background

Syncope – Prodrome 

Different symptoms – Blackening / darkening of vision

– BP- lowering medication – Teenagers – Previous hx or family history?

– Seeing spots – Ringing in ears – Hot flush



– Feeling ‘dizzy’ or lightheaded

Provoking factors – Medical procedures/ pain – Hot surroundings – Getting up too quickly/ prolonged standing 



Key question: – Similar to having got up too quickly?

e.g. gone into kitchen or bathroom, standing in queue

Syncope – Description and After 

Patient – – – –

Fade to black or sudden LOC Rapid recovery Slightly confused Tongue biting and incontinence

The patient on the plane 

Beware of convulsive syncope: – If kept in upright position for too long, may be more

severe (e.g. plane, car)

– Can go rigid



Witness – – – –

Flop to ground Pale, sweaty, ashen Short duration Rapid recovery

– May have rhythmic jerks (< 30s) – May be incontinent – May be briefly confused, feel rotten for rest of day

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Cardiac syncope 

History of cardiac disease



Prodrome: – Perhaps palpitations – May be very sudden – Beware: collapse during exercise

Non-epileptic attack

Hysteria

Psychogenic non-epileptic seizures NEAD  Hysterical seizures  Hystero-epilepsy  Pseudoseizures  Dissociative seizures  Conversion disorder  

Non-epileptic attack - Background Can be difficult to differentiate Social problems  Sexual and physical trauma/abuse  Functional co-morbidities  Particular associated disorders:  

– Irritable bowel syndrome – CFS/ME – Hysterectomy – Anxiety, depression – Asthma

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Psychogenic non-epileptic attack Prodrome  

Non-epileptic attack - Description 

Depersonalisation/derealisation

Many different types – Lie still – Prolonged events, waxing and waning

Non-specific symptoms:

– Thrashing

– Inability to move

– Shaking/tremor all over (rarely jerking)

– Fear

– Back arching (classical hysterical attack)

– Breathlessness – “Dizzy”

Resisting eye opening Injuries can occur during an attack  Incontinence/Tongue biting 

– Pressure on head / chest



Non-epileptic attack - After

Arc de Cercle 

May appear confused or recover quickly



Often emotional, tearful



May describe awareness of surrounding during event

Conversational Analysis

Conversational Analysis

Feature

Epilepsy

Non-Epileptic Attack

Feature

Epilepsy

Non-Epileptic Attack

Subjective seizure symptoms

Volunteered Discussed in detail

Avoided Discussed sparingly

Reference to seizure suppression

Often

Rare

Formulation attempts

Extensive, much detail

Absent, little detail

Description by negation

Rare

Common

Seizures as a topic

Self-initiated

Initiated by interviewer

Description of period of LOC

Attempts to fill gap Difficulty in describing sensations

Inability to remember anything

Focus on seizure description

Easy

Difficult (focusing resistance) Conceptualisation of seizures

External, threatening entity Metaphors of fighting

Less active struggle

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Examination

Hyperventilation / Panic attack 

Breathlessness, tight chest  Bilateral parasthesia  Tetany, hands cramping  Lightheaded 



Often not aware of overbreathing

Determined by history: – Focused neurological examination – Fundoscopy – Erect/Supine BP, heart rate – Auscultation of heart



Ask them to try to reproduce – Involvuntary movements – hyperventilation

Investigations

•

Status epilepticus

ECG

Status epilepticus 5 min or more of (i) continuous clinical and/or electrographic seizure activity or (ii) recurrent seizure activity without recovery (returning to baseline) between seizures

Status epilepticus 5 min or more of (i) continuous clinical and/or electrographic seizure activity or (ii) recurrent seizure activity without recovery (returning to baseline) between seizures Generalised Convulsive Status Defined as convulsions that are associated with rhythmic jerking of the extremities.

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Status epilepticus

Status epilepticus

5 min or more of (i) continuous clinical and/or electrographic seizure activity or (ii) recurrent seizure activity without recovery (returning to baseline) between seizures

5 min or more of (i) continuous clinical and/or electrographic seizure activity or (ii) recurrent seizure activity without recovery (returning to baseline) between seizures

Generalised Convulsive Status Defined as convulsions that are associated with rhythmic jerking of the extremities.

Generalised Convulsive Status Defined as convulsions that are associated with rhythmic jerking of the extremities.

Non-convulsive Status Seizure activity seen on EEG without clinical findings associated with GCSE.

Non-convulsive Status Seizure activity seen on EEG without clinical findings associated with GCSE.

Refractory Status Seizure activity persists after receiving adequate doses of an initial benzodiazepine followed by second AED

Emergent intial therapy benzodiazepines

Management Emergent Initial Therapy

Urgent Control Therapy

Drug

Evidence

Considerations

Lorazepam (IV)

I, Level A

Respiratory depression (RD), small volume of distribution

Midazolam (IV, IM, buccal)

I, Level A

RD, short acting

Diazepam (IV, rectal)

IIa, Level A

RD, short acting

RCT: BZD vs placebo Respiratory depression was seen less frequently in those treated with benzodiazepines for GCSE than for those who received placebo (Alldredge et al 2011)

Refractory Status Therapy

Urgent control therapy

Phenytoin

• Unless cause recognised and definitively corrected • Maintenance or further control

Advantages

Drug

Evidence

Considerations



Long experience

Valproate

IIa, Level A

Hepatotoxicity, hyperammonaemia, pancreatitis, TBI. Used with GBM



Single daily dose regimen for good compliance

Phenytoin

IIa, Level B

Arrhythmias, hypotension, purple glove syndrome



No need for a taper-up schedule

Midazolam

IIb, Level B

Phenobarbital

IIb, Level C



Levetiracetam

IIb, Level C

Relatively quick disappearance of the medication after stopping.

Diasadvantages 

Neurotoxicity – Cerebellar ataxia, nystagmus,

tremor, dysarthria, drowsiness



Cosmetic: – thickening of skin, acne,

undesired hair growth, and gum swelling.

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19/11/2014

Rash

Steven Johnson Syndrome (0.1%) 5% Mortality

Toxic Epidermal Necrolysis 30-35% Mortality

Urgent control therapy

Pharmacokinetics of Phenytoin

• Unless cause recognised and definitively corrected • Maintenance or further control Drug

Evidence

Considerations

Valproate

IIa, Level A

Hepatotoxicity, hyperammonaemia, pancreatitis, TBI. Used with GBM

Phenytoin

IIa, Level B

Arrhythmias, hypotension, purple glove syndrome

Midazolam (cont infusion)

IIb, Level B

RD, hypotension, renal elimination

Phenobarbital

IIb, Level C

RD, hypotension

Levetiracetam

IIb, Level C

Not-hepatically metabolised, minimal drug interactions

Refractory SE therapy

Refractory SE therapy

• Determined by cEEG and clinical exam • Immediate alternative therapy + boluses for breakthrough SE

• Determined by cEEG and clinical exam • Immediate alternative therapy + boluses for breakthrough SE

Drug

Evidence

Considerations (RD, hypotension)

Drug

Evidence

Considerations (RD, hypotension)

Midazolam

Insuff data

Tachyphylaxis with prolonged use

Midazolam

Insuff data

Tachyphylaxis with prolonged use

Propofol

Insuff data

Cardiac failure, rhabdomyolisis, metabolic acidosis, PRIS

Propofol

Insuff data

Cardiac failure, rhabdomyolisis, metabolic acidosis, PRIS

Thiopental

Insuff data

Cardiac depression, metabolised to pentobarbital (paralytic ileus, withdrawal seizures)

Thiopental

Insuff data

Cardiac depression, metabolised to pentobarbital (paralytic ileus, withdrawal seizures)

• Intensity: Dictated by cEEG to attain cessation or burst suppression • Duration: 24-48h, but no defined duration or ‘number of trials to be considered futile

9

19/11/2014

Refractory SE therapy - alternatives Ketamine IVIg/PE  Steroids

CASES

Hypothermia Ketogenic diet  Vagal Nerve Stimulator









Case 1 

20 year old RH female

 

5 year history of “turns” Twice in supermarket, once shopping, once standing in tanning parlour, one at home All whilst standing

      

Feels hot Hearing loss Vision goes white LOC (seconds) Feels normal immediately following turn

Case 2 

50 yr old LH male

 

Sitting in living room Detached feeling – ‘in a strange place’ for seconds, ‘before blacking out’ Came around with wife looking at him



    

Wife description Hand searching around the chair Head turned to the right, right arm stiffened and then whole body shook for 2 minutes Unresponsive for 2 mins Confused for 15 mins

Managed to abort the last episode by sitting with her head between her knees

Case 3 

22 year old RH male student



In street handing out night club flyers



Sudden severe sharp abdominal pain – Felt unwell and nauseated – Leans against wall to vomit – Dizzy (light headed and his vision becomes blurry) – No memory until he comes around sitting against wall – Felt slightly disoriented – Friends told him that his arms were shaking



Friends account – Legs buckle – Went to aid, supporting him upright, then slowly lowering him to a sitting position – Some jerking movements of his arms as they lowered him to ground – Seemed to come in and out of consciousness for a few minutes before came round – Slightly disoriented, but rapidly back to normal – Pale and clammy

10

19/11/2014

Case 4

Case 4 

21 year old RH man

 

At home in kitchen, standing Loss of consciousness without warning Came around on floor, confused, drowsy and tremulous



Girlfriend account





   

Heavy alcohol of several years (100+units/week) No alcohol for ~24 hours 5 year history of increasingly frequent sudden solitary jerks / jolts Had become family joke No previous generalised seizures

– Fell rigid to floor – Jerking movements of all 4 limbs for 5 minutes – Unresponsive for 10 minutes, confused for 20 minutes

11