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AKH (Kisumu) CME 10.02.15
Dr. Dilraj Singh Sokhi BMedSci(Hons) MBChB(Hons) MRCP(UK)(Neurology)
Honorary Teacher in Adult Clinical Neurology, University of Sheffield (UK)
Visiting Trainee Neurologist and ILAE Epilepsy Teacher, Aga Khan University Hospital
(Nairobi)
Outline
 Introduction
 Causes and risk factors
 Classification of seizures
 Diagnosis and investigation
 Management
 Social aspect
 Conclude
TLOC (“Blackouts”)
Blackouts
Problem with blood circulation
(Syncope)
Primary disturbance
of brain function
Epilepsy Non-epileptic
attacks
Idiopathic generalised
epilepsy
Focal epilepsyUnclassifiable
epilepsy
Non-cardiacCardiac
WHO Report 2005; de Boer et al. 2008; Mathers et al. 2008
Africa: 2x  incidence + prevalence
3-5x  mortality
Kenya:Similar figures (Ngugi 2011, 2012, 2013)
ILAE GCAE “Bridging the Gap: Epilepsy in WHO Africa Region”
Epilepsy – Global Burden
 Seizure: transient signs and/or symptoms due to abnormal excessive synchronous neuronal activity in the brain.
 Epilepsy: enduring predisposition to repeated and unprovoked seizures occurring more than twice in a year.
 Kenya:
1-1.2% of 40 million people
National Epilepsy Guidelines
www.epilepsykenya.org
Epilepsy
Video
Causes
 Infections
 Metabolic (acquired and genetic)
 Head trauma
 Perinatal injury
 Toxic
 SOL
 Vascular
 Congenital
 Degenerative
• Ti
Ba-Diop et al, 2014
Causes of Epilepsy in Africa
Cysticercosis
Most significant food-born parasite (WHO, 2014)
Neurocysticercosis
 ~1/3 of epilepsy in T.solium-endemic countries
White et al. 1997, 2000; Del Brutto et al. 2005;
Montano et al. 2005; Burneo et al. 2009; Singh et al. 2012
Neurocysticercosis Diagnosis
 Absolute criteria
 CNS Biopsy +ve
 Cystic lesions with scolex on neuroimaging
 Subretinal parasites by fundoscopic
 Major criteria
 Highly suggestive lesions on neuroimaging
 EITB +ve
 Resolution of brain cystic lesions after anti-helminthics
 Spontaneous resolution of small single enhancing lesions
 Minor criteria
 Suggestive lesions on neuroimaging
 Suggestive clinical manifestations
 Positive CSF Ab or Ag ELISA
 Extraneural cysticercosis
 Epidemiologic criteria
 Individuals in endemic area
 Frequent travel to endemic areas
 Household contact with T. solium infection
Definitive:
- one absolute OR
- two major + one minor OR
- one epidemiologic
Probable
- one major + two minor OR
- one major + one minor + one
epidemiologic OR
- three minor + one epidemiologic
Del-Brutto et al 2001
Trigger Factors
 Non-adherence to treatment / stopping treatment
 Sleep deprivation / exhaustion
 Acute infections and fever
 Flickering lights e.g. televisions, computers, disco
 Alcohol intake/withdrawal
 Substance abuse/withdrawal
 Hormonal imbalances (catamenial-seizures)
 Dehydration
 Emotional Stress
 Hyperventilation
Classification + Semiology
Focal Epilepsy
Classification of Epilepsies
It’s all in the HISTORY!
Always always always get a collateral history (?video)
Onset
 age of first seizure
 Association with a particular event, accident, illness, fever?
 Is there always fever with the seizures?
Pre-ictal phase
 Any precipitating factors?
 Are there any prodromal symptoms?
History (cont…)
 lctal phase – semiology (description of seizure itself)
 Is there an aura? What does it consist of?
 Does the patient scream?
 Where in the body? How does the event start (e.g. turning face)
 Does the patient jerk? If so, both arms and legs, or one side?
 Are they unconscious? Does the patient fall down?
 Does the patient have incontinence of urine or stool?
 Does the patient bite the tongue?
 Does the patient make irrational or abnormal movements?
 Breathing: stertorous/snoring, shallow/deep, hyperventilating?
 How long is the ictal phase?
History (cont…)
History (cont…)
 Post-ictal
 How long does the convulsion last? (incl. post-ictal phase)
 How is the patient's behaviour after the seizure?
 Is there any focal sign?
 How long is the recovery phase?
 Other important details
 Time: At what time of the day or night do the seizures occur
(daytime, when sleeping or awakening)?
 Frequency: when was the first / last / worst seizure?
How frequent have the seizures been?
Has there been a change in the frequency?
What is the interval between seizures?
History (cont…)
 Family history
 Pregnancy and perinatal history
 Developmental history (milestones)
 Past Medical History
 Medicines or alcohol used?
 Social History
Examination and Investigations
 IT’S ALL IN THE HISTORY!
 Examination (BP, temp, neuro)
 Video EEG is gold standard
 EEG and brain imaging reasonable
 ECG is mandatory
 Not much room for other investigations except:
 FBC, U&E, Mg, Ca, glucose, inflammatory markers
ECGs Quiz!
Differential Diagnosis of Seizures
 Syncope
 Psychogenic seizures
 Cardiac arrhythmia
 Hyperventilation and panic attacks
 Night terrors in children
 Breath holding spells in children
Video
Syncope vs. Seizures
Video
PNES in the Clinic
Conversational Analysis for PNES
Feature Epilepsy PNES
Seizure symptoms Volunteered
Detailed
Negation explained
Interviewer-initiated
No detail
“Focussing resistanee“
Formulation work Extensive Little; Head-turn sign
Gaps in consciousness Exact description ‘I don‘t know‘
Metaphors Consistent image of
independently acting
external opponent
Seizure as place or space
person goes to
Catastrophising
 Always directly ask about symptoms of hyperventilation
Reuber et al, 2009
Abversek et al, 2011
PNES vs Epilepsy – Features?
 Prevent injury
 Prevent death
 when in water, SUDEP
 Reduce interruption of daily life (seizure + post-ictal)
 Driving regulations in UK
 Prolonged seizures (>30 mins) = permanent brain damage
 ?cure in the longer term
Why Control Epilepsy?
Treatment – First Aid
 Move patient away from fire, traffic or water
 Take away any objects that could harm the patient
 Loosen tight clothes, remove glasses
 Put wooden stick into the mouth to prevent injury
 Put something soft under the head
 Turn patient on his or her left side, so that saliva and
mucus can run out of the mouth
 Try to stop the jerking, or restrain the movements.
 Remain with patient until regains consciousness
 Give them something to drink during the seizure
 Put them in the recovery position at the end
Treatment – First Aid
 Move patient away from fire, traffic or water
 Take away any objects that could harm the patient
 Loosen tight clothes, remove glasses
Put wooden stick into the mouth to prevent injury
 Put something soft under the head
 Turn patient on his or her left side, so that saliva and
mucus can run out of the mouth
Try to stop the jerking, or restrain the movements.
 Remain with patient until regains consciousness
Give them something to drink during the seizure
 Put them in the recovery position at the end
Treatment - Considerations
 Confirmed diagnosis of active epilepsy:
 ≥ 2 unprovoked seizures > 24 hours apart in a year
 Rarely can start after single seizure. Evidence needed:
relevant neurological deficit
abnormal EEG: epileptiform activity or focal slowing
patient, after adequate counselling, desires treatment
 Counsel patients – precipitating factors, adherence,
social impact, safety, side effects etc
 Also consider: - gender and age
- Other meds esp cART
- Other PMH
Treatment (1)
 Initiation of treatment
 Start with one drug and small dose
 Gradually adjust dosage at two weeks intervals until:
- complete seizure control
- maximum pharmacologically tolerated dose is reached
 If no seizure control, add second drug and consider
gradually reducing or maintaining the initial drug
 The aim of treatment is to achieve the lowest maintenance
dose which provides complete seizure control.
 Gradual introduction of AED can produce therapeutic
effects but with fewer side-effects.
 Severe "intoxication" side-effects at the beginning of the
treatment indicate too rapid or too large dose increases.
Treatment (2)
 Maintenance
 Ideally, only one drug should be used.
 If the first drug has only produced a partial response, then a
second drug can be added gradually taking into
consideration drug interactions.
 The aim should be to have a maximum of two drugs.
 If the two drugs fail, then consult the next level.
Partnership between patient and provider is
important to ensure that the patient
understands the importance of adhering to
treatment.
Treatment (3)
 Follow up and monitoring
 Holistic approach with partnership of patient, family and
care providers enhances patient's insight and compliance.
 Drug monitoring should be done by measuring serum levels
in cases where there is difficulty in management.
 Compliance is the key to successful seizure control, and
counselling the patient is the most critical factor.
Treatment (3)
 When to withdraw drugs
 If the patient has been seizure-free for 2-3 years (depends)
 Prior to drug withdrawal, consider:
- Focal seizures are often very difficult to control
especially hippocampus and other temporal
lobe areas. Relapse rate is high. ? Carry on
indefinitely
- IGE generalised seizures have best remission rates
- Perisistently abnormal EEG vs. seizures controlled
- Patient views: may opt to remain on medications
despite achieving prolonged remission.
5-10% chance getting another seizure anyway.
 Counselling is very important to alert them of the chance of
Treatment (4)
 How to withdraw treatment
Done in a very gradual manner
at the lowest dosages
over three to six months.
In case of poly-therapy, each drug should be withdrawn
separately one after the other.
Treatment Choices
First Line
Phenobarbitone
Phenytoin
Carbamezapine
Sodium Valproate
Rescue medication
Second Line
Clonazepam, Clobazam, Lorazepam, Lamotrigine, Gabapentin, Pregabalin,
Ethosuximide, Methsuximide, Esclimezapine, Oxcarbazapine, Topiramate,
Levetiracetam, Peramapanel, Tiagabine, Vigabatrin, etc etc
REFER TO GUIDELINES
FOR RATIONALE OF
CHOICES, DOSES,
REGIMES ETC
Status Epilepticus
Other (Social) Aspects
 Drugs have to be taken for many years, possibly a life-time.
 Sudden discontinuation of the drugs may result in recurrence of the
seizures or in life-threatening status epilepticus.
 It may take days  few weeks before drugs have any effect.
 Combination with herbal treatment might be dangerous as interaction
between the drugs and the herbs unpredictable.
 Not contagious and anyone can touch the person while they are having
a seizure (e.g. to remove them from the danger of fire or water) or in
between the seizures.
 Child of normal intelligence should be placed in normal school.
 Over-protection not helpful in a child's upbringing, but reasonable
precautions should be taken
 Epilepsy should be talked about with family, school, work etc
 Epilepsy is NOT a reason for not marrying or have a family.
Summary
 3 main causes of TLOC; important to differentiate
 Clinical features of these 3 main types
 Its all in the history! (and the video…)
 (some) Role of investigations: ECG always, EEG, CT/MRI
 Treatment options; status epilepticus
 Counselling patients and families/caretakers is key on all
aspects of their disease.
Acknowledgements
 Professor Markus Reuber
 Dr. Richard Grünewald
 Dr. Stephen Howell
 AKH (Kisumu)
ILAE PNES TF Global Survey
 We will collect Pan-African, including Kenyan, data this year
 Has been approved by local ethics board!
www.TinyURL.com/pneskenya
Questions?
 References available on request

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Epilepsy CME Kisumu 10th February 2015

  • 1. AKH (Kisumu) CME 10.02.15 Dr. Dilraj Singh Sokhi BMedSci(Hons) MBChB(Hons) MRCP(UK)(Neurology) Honorary Teacher in Adult Clinical Neurology, University of Sheffield (UK) Visiting Trainee Neurologist and ILAE Epilepsy Teacher, Aga Khan University Hospital (Nairobi)
  • 2. Outline  Introduction  Causes and risk factors  Classification of seizures  Diagnosis and investigation  Management  Social aspect  Conclude
  • 3. TLOC (“Blackouts”) Blackouts Problem with blood circulation (Syncope) Primary disturbance of brain function Epilepsy Non-epileptic attacks Idiopathic generalised epilepsy Focal epilepsyUnclassifiable epilepsy Non-cardiacCardiac
  • 4. WHO Report 2005; de Boer et al. 2008; Mathers et al. 2008 Africa: 2x  incidence + prevalence 3-5x  mortality Kenya:Similar figures (Ngugi 2011, 2012, 2013) ILAE GCAE “Bridging the Gap: Epilepsy in WHO Africa Region” Epilepsy – Global Burden
  • 5.  Seizure: transient signs and/or symptoms due to abnormal excessive synchronous neuronal activity in the brain.  Epilepsy: enduring predisposition to repeated and unprovoked seizures occurring more than twice in a year.  Kenya: 1-1.2% of 40 million people National Epilepsy Guidelines www.epilepsykenya.org Epilepsy
  • 7. Causes  Infections  Metabolic (acquired and genetic)  Head trauma  Perinatal injury  Toxic  SOL  Vascular  Congenital  Degenerative
  • 8. • Ti Ba-Diop et al, 2014 Causes of Epilepsy in Africa
  • 10. Neurocysticercosis  ~1/3 of epilepsy in T.solium-endemic countries White et al. 1997, 2000; Del Brutto et al. 2005; Montano et al. 2005; Burneo et al. 2009; Singh et al. 2012
  • 11. Neurocysticercosis Diagnosis  Absolute criteria  CNS Biopsy +ve  Cystic lesions with scolex on neuroimaging  Subretinal parasites by fundoscopic  Major criteria  Highly suggestive lesions on neuroimaging  EITB +ve  Resolution of brain cystic lesions after anti-helminthics  Spontaneous resolution of small single enhancing lesions  Minor criteria  Suggestive lesions on neuroimaging  Suggestive clinical manifestations  Positive CSF Ab or Ag ELISA  Extraneural cysticercosis  Epidemiologic criteria  Individuals in endemic area  Frequent travel to endemic areas  Household contact with T. solium infection Definitive: - one absolute OR - two major + one minor OR - one epidemiologic Probable - one major + two minor OR - one major + one minor + one epidemiologic OR - three minor + one epidemiologic Del-Brutto et al 2001
  • 12. Trigger Factors  Non-adherence to treatment / stopping treatment  Sleep deprivation / exhaustion  Acute infections and fever  Flickering lights e.g. televisions, computers, disco  Alcohol intake/withdrawal  Substance abuse/withdrawal  Hormonal imbalances (catamenial-seizures)  Dehydration  Emotional Stress  Hyperventilation
  • 16. It’s all in the HISTORY! Always always always get a collateral history (?video) Onset  age of first seizure  Association with a particular event, accident, illness, fever?  Is there always fever with the seizures? Pre-ictal phase  Any precipitating factors?  Are there any prodromal symptoms?
  • 17. History (cont…)  lctal phase – semiology (description of seizure itself)  Is there an aura? What does it consist of?  Does the patient scream?  Where in the body? How does the event start (e.g. turning face)  Does the patient jerk? If so, both arms and legs, or one side?  Are they unconscious? Does the patient fall down?  Does the patient have incontinence of urine or stool?  Does the patient bite the tongue?  Does the patient make irrational or abnormal movements?  Breathing: stertorous/snoring, shallow/deep, hyperventilating?  How long is the ictal phase?
  • 19. History (cont…)  Post-ictal  How long does the convulsion last? (incl. post-ictal phase)  How is the patient's behaviour after the seizure?  Is there any focal sign?  How long is the recovery phase?  Other important details  Time: At what time of the day or night do the seizures occur (daytime, when sleeping or awakening)?  Frequency: when was the first / last / worst seizure? How frequent have the seizures been? Has there been a change in the frequency? What is the interval between seizures?
  • 20. History (cont…)  Family history  Pregnancy and perinatal history  Developmental history (milestones)  Past Medical History  Medicines or alcohol used?  Social History
  • 21. Examination and Investigations  IT’S ALL IN THE HISTORY!  Examination (BP, temp, neuro)  Video EEG is gold standard  EEG and brain imaging reasonable  ECG is mandatory  Not much room for other investigations except:  FBC, U&E, Mg, Ca, glucose, inflammatory markers
  • 23. Differential Diagnosis of Seizures  Syncope  Psychogenic seizures  Cardiac arrhythmia  Hyperventilation and panic attacks  Night terrors in children  Breath holding spells in children
  • 24. Video
  • 26. Video
  • 27. PNES in the Clinic
  • 28. Conversational Analysis for PNES Feature Epilepsy PNES Seizure symptoms Volunteered Detailed Negation explained Interviewer-initiated No detail “Focussing resistanee“ Formulation work Extensive Little; Head-turn sign Gaps in consciousness Exact description ‘I don‘t know‘ Metaphors Consistent image of independently acting external opponent Seizure as place or space person goes to Catastrophising  Always directly ask about symptoms of hyperventilation Reuber et al, 2009
  • 29. Abversek et al, 2011 PNES vs Epilepsy – Features?
  • 30.  Prevent injury  Prevent death  when in water, SUDEP  Reduce interruption of daily life (seizure + post-ictal)  Driving regulations in UK  Prolonged seizures (>30 mins) = permanent brain damage  ?cure in the longer term Why Control Epilepsy?
  • 31. Treatment – First Aid  Move patient away from fire, traffic or water  Take away any objects that could harm the patient  Loosen tight clothes, remove glasses  Put wooden stick into the mouth to prevent injury  Put something soft under the head  Turn patient on his or her left side, so that saliva and mucus can run out of the mouth  Try to stop the jerking, or restrain the movements.  Remain with patient until regains consciousness  Give them something to drink during the seizure  Put them in the recovery position at the end
  • 32. Treatment – First Aid  Move patient away from fire, traffic or water  Take away any objects that could harm the patient  Loosen tight clothes, remove glasses Put wooden stick into the mouth to prevent injury  Put something soft under the head  Turn patient on his or her left side, so that saliva and mucus can run out of the mouth Try to stop the jerking, or restrain the movements.  Remain with patient until regains consciousness Give them something to drink during the seizure  Put them in the recovery position at the end
  • 33. Treatment - Considerations  Confirmed diagnosis of active epilepsy:  ≥ 2 unprovoked seizures > 24 hours apart in a year  Rarely can start after single seizure. Evidence needed: relevant neurological deficit abnormal EEG: epileptiform activity or focal slowing patient, after adequate counselling, desires treatment  Counsel patients – precipitating factors, adherence, social impact, safety, side effects etc  Also consider: - gender and age - Other meds esp cART - Other PMH
  • 34. Treatment (1)  Initiation of treatment  Start with one drug and small dose  Gradually adjust dosage at two weeks intervals until: - complete seizure control - maximum pharmacologically tolerated dose is reached  If no seizure control, add second drug and consider gradually reducing or maintaining the initial drug  The aim of treatment is to achieve the lowest maintenance dose which provides complete seizure control.  Gradual introduction of AED can produce therapeutic effects but with fewer side-effects.  Severe "intoxication" side-effects at the beginning of the treatment indicate too rapid or too large dose increases.
  • 35. Treatment (2)  Maintenance  Ideally, only one drug should be used.  If the first drug has only produced a partial response, then a second drug can be added gradually taking into consideration drug interactions.  The aim should be to have a maximum of two drugs.  If the two drugs fail, then consult the next level. Partnership between patient and provider is important to ensure that the patient understands the importance of adhering to treatment.
  • 36. Treatment (3)  Follow up and monitoring  Holistic approach with partnership of patient, family and care providers enhances patient's insight and compliance.  Drug monitoring should be done by measuring serum levels in cases where there is difficulty in management.  Compliance is the key to successful seizure control, and counselling the patient is the most critical factor.
  • 37. Treatment (3)  When to withdraw drugs  If the patient has been seizure-free for 2-3 years (depends)  Prior to drug withdrawal, consider: - Focal seizures are often very difficult to control especially hippocampus and other temporal lobe areas. Relapse rate is high. ? Carry on indefinitely - IGE generalised seizures have best remission rates - Perisistently abnormal EEG vs. seizures controlled - Patient views: may opt to remain on medications despite achieving prolonged remission. 5-10% chance getting another seizure anyway.  Counselling is very important to alert them of the chance of
  • 38. Treatment (4)  How to withdraw treatment Done in a very gradual manner at the lowest dosages over three to six months. In case of poly-therapy, each drug should be withdrawn separately one after the other.
  • 39. Treatment Choices First Line Phenobarbitone Phenytoin Carbamezapine Sodium Valproate Rescue medication Second Line Clonazepam, Clobazam, Lorazepam, Lamotrigine, Gabapentin, Pregabalin, Ethosuximide, Methsuximide, Esclimezapine, Oxcarbazapine, Topiramate, Levetiracetam, Peramapanel, Tiagabine, Vigabatrin, etc etc REFER TO GUIDELINES FOR RATIONALE OF CHOICES, DOSES, REGIMES ETC
  • 41. Other (Social) Aspects  Drugs have to be taken for many years, possibly a life-time.  Sudden discontinuation of the drugs may result in recurrence of the seizures or in life-threatening status epilepticus.  It may take days  few weeks before drugs have any effect.  Combination with herbal treatment might be dangerous as interaction between the drugs and the herbs unpredictable.  Not contagious and anyone can touch the person while they are having a seizure (e.g. to remove them from the danger of fire or water) or in between the seizures.  Child of normal intelligence should be placed in normal school.  Over-protection not helpful in a child's upbringing, but reasonable precautions should be taken  Epilepsy should be talked about with family, school, work etc  Epilepsy is NOT a reason for not marrying or have a family.
  • 42. Summary  3 main causes of TLOC; important to differentiate  Clinical features of these 3 main types  Its all in the history! (and the video…)  (some) Role of investigations: ECG always, EEG, CT/MRI  Treatment options; status epilepticus  Counselling patients and families/caretakers is key on all aspects of their disease.
  • 43. Acknowledgements  Professor Markus Reuber  Dr. Richard Grünewald  Dr. Stephen Howell  AKH (Kisumu)
  • 44. ILAE PNES TF Global Survey  We will collect Pan-African, including Kenyan, data this year  Has been approved by local ethics board! www.TinyURL.com/pneskenya

Notas do Editor

  1. Half population will have TLOC Half will be seizures, ¼ syncope, 18% NEAD