Epilepsy

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Epilepsy

„Sacred Disease“
Definition
A chronic neurologic disorder manifesting by
repeated epileptic seizures (attacks or fits)
which result from paroxysmal uncontrolled
discharges of neurons within the central
nervous system (grey matter disease).
 The clinical manifestations range from a
major motor convulsion to a brief period of
lack of awareness. The stereotyped and
uncontrollable nature of the attacks is
characteristic of epilepsy.
Pathogenesis
 The 19th century neurologist Hughlings
Jackson suggested “a sudden excessive
disorderly discharge of cerebral neurons“ as
the causation of epileptic seizures.
 Recent studies in animal models of focal

epilepsy suggest a central role for the


excitatory neurotransmiter glutamate
(increased in epi) and inhibitory gamma
amino butyric acid (GABA) (decreased)
Epidemiology and course
 Epilepsyusually presents in childhood or
adolescence but may occur for the first time
at any age.
Epidemiology and course
 5% of the population suffer a single sz at
some time
 0.5-1% of the population have recurrent sz =

EPILEPSY
 70% = well controlled with drugs (prolonged

remissions); 30% epilepsy at least partially


resistant to drug treatments = INTRACTABLE
EPILEPSY.
Epilepsy
is a symptom of numerous disorders, but in the majority of
sufferers the cause remains unclear despite careful history
taking,examination and investigation!
Epilepsy - Classification
 The modern classification of the epilepsies is
based upon the nature of the seizures rather
than the presence or absence of an
underlying cause.
 Seizures which begin focally from a single
location within one hemisphere are thus
distinguished from those of a generalised
nature which probably commence in a
deeper structures (brainstem? thalami) and
project to both hemispheres simultaneously.
Epilepsy - Classification
 Focal seizures – account for
80% of adult epilepsies
- Simple partial seizures
- Complex partial seizures
- Partial seizures secondarilly
generalised

 Generalised seizures

 Unclassified seizures
Focal (partial) seizures
 Simple partial seizures
Motor, sensory, vegetative or psychic
symptomatology
Typically consciousness is preserved
Focal (partial) seizures
 Simple partial seizures
Motor, sensory, vegetative or psychic
symptomatology
Typically consciousness is preserved
Focal (partial) seizures
 Simple partial seizures
Motor, sensory, vegetative or psychic
symptomatology
Typically consciousness is preserved
Focal (partial) seizures
 Complex partial seizures (= psychomotor seizures)
Initial subjective feeling (aura), Impaired level of
consciousness, abnormal behavior (perioral and
hand automatisms)
Usually originates in TL
Focal (partial) seizures
 Partial seizures evolving to tonic/clonic convulsions
– secondary generalised tonic/clonic seizures
(sGTCS)
Generalized seizures
(convulsive or non-convulsive)

 Absences
 Myoclonic seizures
 Clonic seizures
 Tonic seizures
 Atonic seizures
Generalized seizures

 Absences
 Myoclonic seizures
 Clonic seizures
 Tonic seizures
 Atonic seizures
Epilepsy
Differential Diagnosis
The following should be considered in the diff. dg. of epilepsy:
 Syncope attacks (when pt. is standing; results from global reduction
of cerebral blood flow; prodromal pallor, nausea, sweating; jerks!)
 Cardiac arrythmias (e.g. Adams-Stokes attacks). Prolonged arrest of
cardiac rate will progressively lead to loss of consciousness – jerks!
 Migraine (the slow evolution of focal hemisensory or hemimotor
symptomas in complicated migraine contrasts with more rapid
“spread“ of such manifestation in SPS. Basilar migraine may lead to
loss of consciousness!
 Hypoglycemia – seizures or intermittent behavioral disturbances may
occur.
 Narcolepsy – inappropriate sudden sleep episodes
 Panic attacks
 PSEUDOSEIZURES – psychosomatic and personality disorders
Epilepsy – Investigation
 The concern of the clinician is that epilepsy may be symptomatic of
a treatable cerebral lesion.
 Routine investigation: Haematology, biochemistry (electrolytes,
urea and calcium), chest X-ray, electroencephalogram (EEG).
Neuroimaging (CT/MRI) should be performed in all persons aged 25
or more presenting with first seizure and in those pts. with focal
epilepsy irrespective of age.
 Specialised neurophysiological investigations: Sleep deprived
EEG, video-EEG monitoring.
 Advanced investigations (in pts. with intractable focal epilepsy
where surgery is considered): Neuropsychology, Semiinvasive or
invasive EEG recordings, MR Spectroscopy, Positron emission
tomography (PET) and ictal Single photon emission computed
tomography (SPECT)
Epilepsy - Treatment
 The majority of pts respond to drug therapy
(anticonvulsants). In intractable cases surgery may
be necessary. The treatment target is seizure-
freedom and improvement in quality of life!
 Basic rules for drug treatment: Drug treatment
should be simple, preferably using one
anticonvulsant (monotherapy). “Start low, increase slow“.
Polytherapy is to be avoided especially as drug
interactions occur between major anticonvulsants.
 The commonest drugs used in clinical practice are:
Carbamazepine, Sodium valproate, Phenytoin (first line drugs)
Lamotrigine, Topiramate, Levetiracetam, Pregabaline (new AEDs)
Epilepsy – Treatment (cont.)
 If pt is seizure-free for three years, withdrawal of
pharmacotherapy should be considered. Withdrawal
should be carried out only if pt is satisfied that a further
attack would not ruin employment etc. (e.g. driving
licence). It should be performed very carefully and
slowly! 20% of pts will suffer a further sz within 2 yrs.
 The risk of teratogenicity is well known (~5%), especially
with valproates, but withdrawing drug therapy in
pregnancy is more risky than continuation. Epileptic
females must be aware of this problem and thorough
family planning should be recommended. Over 90% of
pregnant women with epilepsy will deliver a normal child.
Epilepsy – Surgical Treatment
 A proportion of the pts with intractable epilepsy will
benefit from surgery.
 Epilepsy surgery procedures: Curative (removal of
epileptic focus) and palliative (seizure-related risk
decrease and improvement of the QOL)
 Curative (resective) procedures: Anteromesial
temporal resection, selective
amygdalohippocampectomy, extensive lesionectomy,
cortical resection, hemispherectomy.
 Palliative procedures: Corpus callosotomy and Vagal
nerve stimulation (VNS).
Status Epilepticus
 A condition when consciousness does not return
between seizures for more than 30 min. This state
may be life-threatening with the development of
pyrexia, deepening coma and circullatory collapse.
Death occurs in 5-10%.
 Status epilepticus may occur with frontal lobe
lesions (incl. strokes), following head injury, on
reducing drug therapy, with alcohol withdrawal, drug
intoxication, metabolic disturbances or pregnancy.
 Treatment: AEDs intravenously ASAP, event.
general anesthesia with propofol or thipentone
should be commenced immediately.

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