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)Graduate Research Fellow in Epilepsy, International Livestock Research Institute (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 + prevalence3-5x 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 peopleNational Epilepsy Guidelines

www.epilepsykenya.org

Epilepsy

Video

Causes

Infections Metabolic (acquired and genetic) Head trauma Perinatal injury Toxic SOL Vascular Congenital Degenerative

• TiBa-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 / two major + one minor / one epidemiologic

Probable one major + two minor / one major + one minor + one epidemiologic / three minor +

one epidemiologic

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 PNESSeizure symptoms Volunteered

DetailedNegation explained

Interviewer-initiatedNo detail“Focussing resistanee“

Formulation work Extensive Little; Head-turn signGaps in consciousness Exact description ‘I don‘t know‘Metaphors Consistent image of

independently acting external opponent

Seizure as place or space person goes toCatastrophising

Always directly ask about symptoms of hyperventilationReuber 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

The recovery position

Unconscious patients should be placed in this semi-prone/recovery position to minimize the risk of obstruction or inhalation of vomitus

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 deficitabnormal EEG: epileptiform activity or focal slowingpatient, 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 recurrence as a permanent cure cannot be assured.

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 LinePhenobarbitonePhenytoinCarbamezapineSodium ValproateRescue medication

Second LineClonazepam, 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