diagnosis of epilepsy
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Practical Approach
To An Epileptic
Patient
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Amr Hasan, M.D. Associate professor of Neurology - Cairo University
Diagnosis of Epilepsy
3
History taking..
• Physical examination in most
cases is relatively unrevealing
• History – taking from the patient
and observer is the most
important step for reaching the
diagnosis.
Diagnosis of Epilepsy
4
Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy
Axis 2: • Identify type of seizure (seizure semiology)
International classification of epileptic seizures.
Axis 3: • Investigations aiming at confirmation of the
diagnosis & searching for an aetiology
Axis 4: • Identify epileptic syndrome International
classification of epilepsy & epileptic syndromes
AXIS 1: D.D. OF EPILEPSY
6
A) Episodic impairment of consciousness
1. Impaired cerebral perfusion e.g. TIAs
2. Syncope.
3. Metabolic disturbances (e.g.,hypoglycemia).
4. Sudden increase in intracranial pressure.
5. Sleep disorders.
6. Movement disorders.
7. Psychologically – related phenomena.
Red flags:
8
-Young adult female, low socioeconomic and educational state. -Nontraditional triggers
- Events in the waiting room…audience - Longer duration >2min - Historionic behavior, comorbid psych depression - Rapid cognitive recovery - Symptomatizing lengthy dis: fatigue, fibromyalgia - Refractory to AED - High frequency not affected by AED - History of abuse - Pt recalls events during period he was unresponsive
AXIS 1: D.D. OF EPILEPSY
9
B) Drop Attacks
• Falling spells without warning signs, without loss of
consciousness or post-ictal symptoms.
• Identifiable mechanisms OTHER THAN epilepsy include;
1. Brainstem ischemia, or rarely if both ACA arise from the
same stenosed ICA.
2. Hydrocephalus.
AXIS 1: D.D. OF EPILEPSY
10
B) Drop Attacks
3. Otolithic catastrophe” of Meniere disease.
4. Cataplexy.
5. orthostatic hypotension.
6. EPS.
AXIS 1: D.D. OF EPILEPSY
11
C) Transient Focal Neurologic Manifestions
1. Migraine with aura.
2. Transient global amnesia.
3. Transient ischemic attacks.
4. Structural intracranial lesions (AVM, chronic subdural
hematoma).
AXIS 1: D.D. OF EPILEPSY
12
C) Transient Focal Neurologic Manifestions
5. Metabolic disorders (hyperglycemia “NKHO”,
hypoglycemia, hypercalcemia, hyponatremia).
6. Labyrinthine disorders (e.g. Meniere’s and BPV).
7. Paroxysmal abnormal involuntary movements e.g.
Paroxysmal dystonia & tics.
8. Psychological.
AXIS 1: D.D. OF EPILEPSY
13
D) Non-Epileptic Paroxysmal Attacks in Pediatric Age Group
• Breath-holding attacks: • Cyanotic spells
• Pallid spells “reflex anoxic seizures”
• Movement disorders:
• Benign non-epileptic myoclonus of early infancy
Paroxysmal choreoathetosis
• Shuddering attacks,
• hyperekplexia, paroxysmal torticollis and tics
Diagnosis of Epilepsy
14
Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy
Axis 2: • Identify type of seizure (seizure semiology)
International classification of epileptic seizures.
Axis 3: • Investigations aiming at confirmation of the
diagnosis & searching for an aetiology
Axis 4: • Identify epileptic syndrome International
classification of epilepsy & epileptic syndromes
Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy
International Classification of Epilepsies
16
I. Partial (focal, local) seizures
A. Simple partial seizures (consciousness not impaired)
1. With motor signs
2. With sensory symptoms
3. With autonomic symptoms or signs
4. With psychic symptoms
International Classification of Epilepsies
17
I. Partial (focal, local) seizures B. Complex partial seizures (temporal lobe or psychomotor
seizures; consciousness impaired)
1. Simple partial onset, followed by impairment of
consciousness
a. With simple partial features (A.1-A.4), followed by impaired
consciousness
b. With automatisms
2. With impairment of consciousness at onset
a. With impairment of consciousness only
b. With automatisms
International Classification of Epilepsies
18
I. Partial (focal, local) seizures
C. Partial seizures evolving to secondarily generalized seizures
(tonic-clonic, tonic or clonic)
1. Simple partial seizures (A) evolving to generalized seizures
2. Complex partial seizures (B) evolving to generalized seizures
3. Simple partial seizures evolving to complex partial seizures,
evolving to generalized seizures
International Classification of Epilepsies
19
II. Generalized seizures (convulsive or nonconvulsive)
1. Simple partial seizures (A) evolving to generalized seizures
2. Complex partial seizures (B) evolving to generalized seizures
3. Simple partial seizures evolving to complex partial seizures,
evolving to generalized seizures
III. Unclassified epileptic seizures (caused by incomplete
data)
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
20
I. Partial (focal, local) seizures
A. Simple partial seizures
• Are caused by focal cortical discharge that results in seizure
phenomenon appropriate to the function of the discharging
area of the brain without impairment of consciousness
• SPS may consist of motor , sensory, autonomic or psychic
symptoms and signs
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
21
I. Partial (focal, local) seizures
• The symptoms are, at least initially, always strictly contralateral to the
hemispheric focus and may represent the expression of excitatory
(positive—irritative) phenomena, inhibitory (negative—suppressive or
paralytic) phenomena, or a combination of the two.
A. Simple partial seizures
1. Simple partial seizures with motor signs:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
22
I. Partial (focal, local) seizures
• The simplest form of simple partial seizure with motor signs is
clonus, which consists of rhythmic alternating contraction and
relaxation of muscle groups controlled by the precentral gyrus
A. Simple partial seizures
1. Simple partial seizures with motor signs:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
23
I. Partial (focal, local) seizures
• The episodes may be self-limited (clonic focal seizure), recurrent (focal
motor status epilepticus), or continuous (epilepsia partialis continua).
• Spread of the discharge along contiguous areas of the precentral gyrus
gives rise to the characteristic march of spreading involvement of muscle
groups in jacksonian seizures.
A. Simple partial seizures
1. Simple partial seizures with motor signs:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
24
I. Partial (focal, local) seizures
• Somatosensory seizures are usually described as"numbness,"
"tingling," "pins and needles," or "like a weak electric shock" and
may arise from the postcentral (most often) or precentral areas.
A. Simple partial seizures
2. Simple partial seizures with sensory symptoms:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
25
I. Partial (focal, local) seizures
• The focal discharge may spread to the adjacent sensory cortex, producing
a jacksonian march of sensory phenomena.
• The focal discharge also may spread to the adjacent motor cortex,
producing motor symptoms.
A. Simple partial seizures
2. Simple partial seizures with sensory symptoms:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
26
I. Partial (focal, local) seizures
• The focal discharge may spread to the adjacent sensory cortex, producing
a jacksonian march of sensory phenomena.
• The focal discharge also may spread to the adjacent motor cortex,
producing motor symptoms.
A. Simple partial seizures
3. Simple partial seizures with autonomic symptoms or signs:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
27
I. Partial (focal, local) seizures
• Autonomic symptoms accompanying simple partial seizures may consist of
epigastric sensations, flushing or pallor, sweating, pupil dilation,
diaphoresis, piloerection, nausea, vomiting, borborygmi, or incontinence.
A. Simple partial seizures
3. Simple partial seizures with autonomic symptoms or signs:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
28
I. Partial (focal, local) seizures
• Psychic symptoms of simple partial seizures may include dysphasia,
dysmnesia, cognitive symptoms, affective symptoms, illusions, or
structured hallucinations.
• Dysphasic symptoms may take the form of speech arrest, vocalization, or
palilalia (involuntary repetition of a syllable or phrase).
A. Simple partial seizures
4. Simple partial seizures with psychic symptoms.
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
29
I. Partial (focal, local) seizures
• Dysmnesic symptoms, distortions of memory, may take the form of a temporal
disorientation, a dreamy state, a flashback, the sensation that an experience has
occurred before (deja vu, if, visual; deja entendu, if auditory), or the sensation
that a familiar sensation is new (jamais vu, if visual; jamais entendu, if auditory).
• Occasionally, a patient may experience a rapid recollection of episodes from the
past (panoramic vision).
A. Simple partial seizures
4. Simple partial seizures with psychic symptoms.
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
30
I. Partial (focal, local) seizures
• The central feature of CPSs is impairment of consciousness,
which is defined as the inability to respond normally to exogenous
stimuli by virtue of altered awareness or responsiveness.
B. Complex Partial Seizures (Psychomotor or Temporal
Lobe Seizures)
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
31
I. Partial (focal, local) seizures
• Responsiveness refers to the ability of the patient to carry out
simple commands or willed movement.
• Awareness refers to the patient's contact with events during the
period in question and its recall.
B. Complex Partial Seizures (Psychomotor or Temporal
Lobe Seizures)
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
32
I. Partial (focal, local) seizures
• The period of impairment of consciousness may or may not be
preceded by symptoms or signs of a simple partial seizure.
B. Complex Partial Seizures (Psychomotor or Temporal
Lobe Seizures)
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
33
I. Partial (focal, local) seizures
• Other manifestations may appear during the period of impaired
consciousness, or automatisms (i.e., nonreflex actions performed
"automatically," without conscious volition, and for which the patient
has no recollection.
B. Complex Partial Seizures (Psychomotor or Temporal
Lobe Seizures)
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
34
II. Generalized seizures
• Absence seizures consist of sudden onset and cessation of
impaired responsiveness, accompanied by a unique 3-Hz spike and
wave EEG pattern.
1. Absence Seizures:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
35
II. Generalized seizures
•No aura is present, and little or no postictal symptomatology occurs.
• The majority of absence seizures last 10 seconds or less and may
be accompanied by mild clonic components, atonic or tonic
components, automatisms, or autonomic components.
1. Absence (Petit Mal) Seizures:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
36
II. Generalized seizures
• Absence seizures usually first manifest between the ages of 5 and
12 years and often stop spontaneously in the teens.
1. Absence Seizures:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
37
II. Generalized seizures
•Myoclonic seizures consist of brief, sudden muscle contractions that may be generalized or localized, symmetric or asymmetric, synchronous or asynchronous.
• No loss of consciousness is usually detectable
2. Myoclonic Seizures:
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
38
II. Generalized seizures
•Tonic seizures consist of a sudden increase in muscle tone in the axial or extremity muscles, or both, producing a number of characteristic postures.
•Consciousness is usually partially or completely lost
3. Tonic Seizures
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
39
II. Generalized seizures
•Prominent autonomic phenomena occur.
•Postictal alteration of consciousness is usually brief, but it may last several minutes.
•Tonic seizures are relatively rare and usually begin between 1 and 7
years of age
3. Tonic Seizures
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
40
II. Generalized seizures
•Atonic seizures consist of sudden loss of muscle tone.
•The loss of muscle tone may be confined to a group of muscles, such as the neck, resulting in a head drop.
•Alternatively, atonic seizures may involve all trunk muscles, leading
to a fall to the ground .
4. Atonic Seizures
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
41
II. Generalized seizures
•Clonic seizures occur almost exclusively in early childhood.
•The attack begins with loss or impairment of consciousness associated with sudden hypotonia or a brief, generalized tonic spasm.
•This is followed by 1 minute to several minutes of bilateral jerks,
which are often asymmetric and may appear predominately in one limb
5. Clonic Seizures
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
42
II. Generalized seizures
•During the attack, the amplitude, frequency, and spatial distribution of these jerks may vary greatly from moment to moment.
•In other children, particularly those aged 1 to 3 years, the jerks
remain bilateral and synchronous throughout the attack.
•Postictally, recovery may be rapid, or a prolonged period of confusion or coma may ensue.
5. Clonic Seizures
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
43
II. Generalized seizures
• Before the tonic phase of a tonic-clonic seizure, bilateral jerks of the
extremities or focal seizure activity may occur.
• The onset of the seizure is marked by loss of consciousness and increased
muscle tone (tonic phase), which usually results in a rigid, flexed posture at
first, and then a rigid, extended posture.
6. Tonic-Clonic (Grand Mal) Seizures
AXIS 2: IDENTIFY TYPE OF SEIZURE
TYPES OF SEIZURES
44
II. Generalized seizures
• This is followed by bilateral rhythmic jerks that become further apart (clonic
phase).
• Prominent autonomic phenomena are observable during the tonic and
clonic phases. Incontinence may occur.
• The patient awakens by passing through stages of coma, confusional state
and drowsiness
6. Tonic-Clonic (Grand Mal) Seizures
Diagnosis of Epilepsy
45
Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy
Axis 2: • Identify type of seizure (seizure semiology)
International classification of epileptic seizures.
Axis 3: • Investigations aiming at confirmation of the
diagnosis & searching for an aetiology
Axis 4: • Identify epileptic syndrome International
classification of epilepsy & epileptic syndromes
Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy
Axis 2: • Identify type of seizure (seizure semiology)
International classification of epileptic seizures.
AXIS 3: INVESTIGATIONS
Labs
47
Should be tailored according to the case;
• Drug screen
• Electrolytes
• KFT
• LFT
• CSF examination
• Lactic acid
AXIS 3: INVESTIGATIONS
EEG
48
Conventional Electroencephalography
• Used in establishing the diagnosis and focality of
an epileptic disorder
• Normal EEG doesnot exclude presence of
epilepsy thus the yield of EEG can be increased
by Sleep deprivation(< 4 h sleep )
• Invasive techniques are used in some cases
which include: • Sphenoidal leads,
• Subdural
• Epidural electrodes
• Depth electrodes.
AXIS 3: INVESTIGATIONS
EEG
49
Multiple Electroencephalography (EEG) exams
• Records interictal and ictal events, sometimes with sleep
deprivation or other activation techniques may help in
bringing out the abnormality.
EEG monitoring with video (video-EEG)
• Used widely in the evaluation of epileptic fits , exclusion
of non-epileptic attacks and to define the electro-clinical
characteristics of the seizures.
AXIS 3: INVESTIGATIONS
NEUROIMAGING
50
A) MRI
• MRI Has become necessary in
delineating anatomic abnormalities
that may be the etiology of the
seizure focus such as:
• Focal cerebral pathologies e.g.
neoplastic lesions ,infarction,
vascular malformations.
AXIS 3: INVESTIGATIONS
NEUROIMAGING
57
A) MRI
• Diffuse cerebral
pathologies e.g.
cytoarchitectural lesions,
such as neuronal
heterotopias, gyral
anomalies, and other
abnormalities.
AXIS 3: INVESTIGATIONS
NEUROIMAGING
58
B) Functional Neuroimaging
• When anatomical imaging fails to identify a lesion,
positron emission tomography (PET) and single photon
emission tomography (SPECT) are used.
• PET measures regional cerebral metabolism and blood
flow. It has been quite successful in identifying the focus
as an area of hypometabolism between attacks.
AXIS 3: INVESTIGATIONS
NEUROIMAGING
59
B) Functional Neuroimaging
• SPECT demonstrates regional cerebral blood flow, which
is linked to cerebral metabolism and can therefore be
used to identify the epileptic focus and can be used to
study both ictal and inter-ictal states.
AXIS 3: INVESTIGATIONS
NEUROIMAGING
60
C) Others
1. Proton MRS
2. Magnetoencephalograpy/magnetic source imaging
• However, PET ,SPECT ,Proton MRS &
Magnetoencephalograpy/magnetic source imaging
should be only done when surgical resection is planned
Diagnosis of Epilepsy
61
Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy
Axis 2: • Identify type of seizure (seizure semiology)
International classification of epileptic seizures.
Axis 3: • Investigations aiming at confirmation of the
diagnosis & searching for an aetiology
Axis 4: • Identify epileptic syndrome International
classification of epilepsy & epileptic syndromes
Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy
Axis 2: • Identify type of seizure (seizure semiology)
International classification of epileptic seizures.
Axis 3: • Investigations aiming at confirmation of the
diagnosis & searching for an aetiology
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
64
EPILEPTIC SYNDROME IS DEFINED BY
1. Type or types of seizure e.g. Absence ,GTC.
2. Age of seizure onset
3. Aetiology
4. Degree of neurologic and intellectual deficit
5. Clinical evolution of the epilepsy
6. EEG pattern
7. Neuroimaging abnormality
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
66
I. LOCALIZATION RELATED (IDIOPATHIC)
Benign epilepsy of childhood with centrotemporal spikes
• The syndrome is termed "rolandic" epilepsy
because of the characteristic features of partial
seizures involving the region around the lower
portion of the central gyrus of Rolando.
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
67
I. LOCALIZATION RELATED (IDIOPATHIC)
Benign epilepsy of childhood with centrotemporal spikes
• Common characteristic features includes :Unilateral
somatosensory involvement, mostly of the tongue
(occasionally of the inner cheeks, lips, gums, or even a
single tooth) ,Speech arrest ,Preservation of
consciousness in most cases ,Pooling of saliva &Tonic
or tonic-clonic spread to face.
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
68
I. LOCALIZATION RELATED (IDIOPATHIC)
Benign epilepsy of childhood with centrotemporal spikes
• The typical interictal EEG shows centrotemporal spikes
or SW, which are either unifocal or bifocal.
• Carbamazepine is often the first medication to be tried,
and seizures usually are well controlled.
• Excellent prognosis.
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
71
I. LOCALIZATION RELATED (IDIOPATHIC)
Childhood epilepsy with occipital paroxysms:
• Early onset type :
• Young childern 5 ys
• Ictal vomiting , deviation of the eye ,impairment of the
consciousness sometimes progress into GTCs
• Two thirds of seizures occur during sleep
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
72
I. LOCALIZATION RELATED (IDIOPATHIC)
Childhood epilepsy with occipital paroxysms:
• Late onset (Gastaut) type :
• Older childern 9 ys
• Formed visual hallucinations or amaurosis followed
by hemiclonic convulsions with post-ictal migrain
• EEG: occipital spikes
• Excellent prognosis
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
73
II. GENERALIZED(IDIOPATHIC):
Childhood absence epilepsy
• Seizure semiology:
• Absence:Typical absence
• High frequency hundreds / day
• + Myoclonus
• + GTCS
• 3-12 years
• EEG changes: 3-Hz spike and wave
• Treatment : VPA, EXM
• Favorable outcome( long term remission)
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
77
II. GENERALIZED(IDIOPATHIC):
Juvenile myoclonic epilepsy
• Genetic disorder
• Females>Males
• Age 8-26 Y, but 79% mostly12-18 Y
• Symmetric myoclonus (upper limbs)
• GTCs: common
• Absence (associated or independently)
• Photosensitivity
• EEG : polyspike and spike wave(rapid)
• Respond to medical ttt: VPA,LEV
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
85
II. GENERALIZED (CRYPTOGENIC):
West syndrome (infantile spasms):
• Triad of :
1. Seizures
2. Psychomotor retardation or regression
3. Specific EEG changes : hypsarrythmia.
• 1st year of life
• Related to prenatal ,natal or postnatal insult
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
86
II. GENERALIZED (CRYPTOGENIC):
West syndrome (infantile spasms):
• Seizures
1. Flexor spasms
2. Extensor spasms
3. Mixed flexor & extensor spasms
• Resistant to treatment
• Poor prognosis
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES
87
II. GENERALIZED (CRYPTOGENIC):
Lennox Gastaut syndrome
• Mixed seizure disorder:
• (Tonic, TC, Myoclonic, Atypical absence & drop attacks )
• Mental retradation
• EEG:
Slow spike & wave
Sharp & slow wave complex
Slow abnormal background
• Treatment : commonly need Polytherapy
• Poor prognosis & cure rarely achieved