Transcript Epilepsy In the Intellectually and Developmentally Disabled

Issues in Developmental Disabilities
Epilepsy in the Intellectually and
Developmentally Disabled
Lecture Presenter:
Christopher M. Inglese, M.D.
Regional Epilepsy Center
St. Luke's Medical Center
Milwaukee,Wisconsin
Video of Inglese
Epilepsy In The MultiplyHandicapped
 Worldwide movement to deinstitutionalize patients with MR
 Improved seizure control, fewer side
effects and less complicated regimens
allow more successful placement in
community
Intellectual and Developmental
Disabilities Associated with Epilepsy
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Cognitive
Motoric
Sensory
Attentional
Behavioral
Affective
Cognitive Mental Retardation
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SMR
MMR
Learning Disabilities
Apraxias/Dyspraxias
Motoric Cerebral Palsy
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Spastic
Extrapyramidal
Developmental Dyspraxias
Hypotonia
Weakness
Sensory
 Hearing Loss
 Visual Impairment
 Sensory Integration Dysfunction
Attentional
 ADHD -Combined Type, Inattentive
Subtype
 Primary Disorders of Vigilance
 Secondary Disorders of Vigilance
Behavioral
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Impulsivity
Hyperkinesis
Affective Storms
Episodic Dyscontrol
Self Injurious Behavior
Aggression
Affective
Mood Disorders
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Anxiety
Depression
Bipolar, Cyclic mood disturbances
Thought Disorders
Autistic Spectrum Disorders
 Aspergers
 Hellers
 Retts
 Kanners (classical autism)
 PDD NOS
Common Medical Comorbidities
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Congenital malformations
Chromosomal Abnormalities
Genetic Disorders
Metabolic Disorders
Static Enephalopathis
Terminology & Definitions
Diagnostic Criteria for Mental Retardation
 IQ < 70
 Impairment in interpersonal relations,
self-care, maturation
 Onset before age 18
 DSM IV 37.90
Seizures
The outward manifestations of
the epilepsies can be purely
subjective, experiential, imposed
emotions.
Epilepsy
A predisposition for unprovoked,
recurrent seizures by a
proximate identifiable cause.
Epileptic Syndromes
Collections of signs, symptoms
from a common cause which
define recognizable patterns of
disease.
The Classification of the
Epilepsies
There are many ways to classify
the epilepsies or seizures
Classifications cont.
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By Cause or Etiology
Idiopathic
Cryptogenic
Symptomatic
By Clinical Appearance
Convulsive
Non Convulsive
Grand Mal
Petit Mal
Major Motor
Minor Motor
By Electro-Clinical Characteristics*
*Determined by the Anatomic Substrate of
the Seizure Generator
Partial Onset
Generalized Onset
Diagnostic Evaluation
 Complete History
 Detailed
physical/neuro
exam
 Family History
 Routine blood
work, toxic and
metabolic
screening, serum
levels
 EEG (often requires
sedation)
 Neuro-imaging
(MRI preferred)
 Video-EEG
monitoring
 Video-recording of
events
Why is Classification Important?
 Basic Science and Clinical Scientists
must have uniformity of definitions in
heterogeneous conditions
 “Apples to apples, oranges to
oranges”
Classification Facilitates Research
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Causal Mechanisms
Treatments
Outcomes
Predispositions
International Classification of
Epileptic Seizures
Partial Seizures
Simple Partial
Complex Partial
Simple or Complex Partial which
generalize
 Sensory
 Motor
 Autonomic
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International Classification of
Epileptic Seizures-Generalized
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Absence (typical and atypical)
Myoclonic
Tonic
Clonic
Atonic-astatic
International Classification of
Epileptic Seizures-Unclassified
 Febrile Seizures
 Reflex Epilepsies
 Status Epilepticus
Classification of Epilepsy
Syndromes
 Idiopathic focal epilepsies
 Familial focal epilepsies
 Symptomatic and Cryptogenic focal
epilepsies
Idiopathic Generalized Epilepsies
 Reflex Epilepsies
 Epileptic Encephalopathies
 Progressive myoclonus epilepsies
Epidemiology and StatisticsPrevalence
 Numerator-old and new cases
 Denominator-population at risk
Epidedemiology (continued)
 Prevalence of MMR IQ < 70 3.7-7.6
per 1000
 Prevalence of SMR IQ < 50 2.8-4.6
per 1000
 Prevalence of epilepsy 4.0-8.8 per
1000
 Prevalence of MR in childhood
epilepsy 31-41%
Epidedemiology (continued)
 MMR and epilepsy 8-18%
 SMR and Epilepsy 30-36%
 Prevalence of Epilepsy in Swedish
study of 6-13 year olds – 2 per 1000
(98 of 48,873)
The risk of Epilepsy increases
30 fold when associated with:
TBI
CP
MR
The risk is 5-15% higher with
previous meningitis or encephalitis
 Hauser and Nelson CP or MR 11% w/
epilepsy-Both CP/MR 48% with
Epilepsy
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Epilepsy can be a disabling
condition in and of itself
Disease stigma
Autonomy
Driving restrictions
Impact of seizures
on memory
 Impact of
treatment on
mood, memory
motivation to learn
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 Occupational
restrictions
 Discrimination
 Impact on learning
of ictus, interictal
state, postical state
Epilepsy
Can tremendously potentiate the impact
of a disability when added to co-existing
challenges, comorbidities
 Cognitive
 Neuromotor
 Sensory
 Attentional
 Behavioral self
regulatory
 Affect and mood
General Principles of
Management-Diagnostic
 Is it Epilepsy?
 Both epileptic and
non-epileptic
seizures?
 Are seizures
caused exclusively
by controllable
medical conditions?
 Cardiac?
 Hemodynamicvascular?
 Iatrogenic?
 Endocrenologic?
 Metabolic?
General Principles of Treatment:
Is Treatment Necessary?
 Febrile Fits
 BRE
 Select appropriate drug for seizure
type or syndrome
 Avoid seizure exacerbating drugs
 Select drug that may target other
issues of importance to patient
 Migraine, mood, sleep, weight, sex
Generalized Principals of
Treatment (continued)
 Discontinue meds whenever possible
 Consensus with client regarding
treatment or discontinuation
Salient Nonepileptic Disorders at
Different Ages: Age 0-2 months
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Tremor
Dyskenesias associated =BPD
Benign neonatal myoclonus
Sleep myoclonus
Apnea
Salient Nonepileptic Disorders at
Different Ages: Age 2-18 months
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Paroxysmal torticollis
Opsoclonus-myoclonus syndrome
Sandiffers syndrome
Jactatio capitis
Masturbation
Paroxysmal choreo-athetosis
GERD
Salient Nonepileptic Disorders at
Different Ages: Age 18 months - 5 yrs.
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Disorder
Pavor nocturnus
Benign positional vertigo
Nodding puppet syndrome
Enuresis nocturnus
Familial dystonia-chorea
Athetosis
Salient Nonepileptic Disorders at
Different Ages: 5-12 yrs. & beyond
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Tics
Complicated migraine
ADHD inattentive type
Parasomnias
Vertebro basilar migraine
Syncope
Hyperventilation syndrome
Panic attacks
Affective storms-rage
Obstructive apnea
General Principles of Treatment
 Avoid polytherapy whenever possible
 Why?
 Efficacy-studies have shown that 60%
of people with IDD and Epilepsy can
be controlled with one drug
Tolerability
 Sedation increases with burden of
superfluous drugs
 Phamacodynamic effects, can't be
measured
 Avoid drugs that may worsen
comorbid diseases
 VPA, CBZ, Wt. Gain, obesity,
diabetes, joint disease
Newer Drugs?
 There is no evidence that newer
drugs are significantly more effective
 Distinguished by
 Less significant AE's
 Ease of administration
 Reduced need for surveillance labs, level
monitoring
 Potential to be useful for comorbidities.
Refractory Epilepsy
 There is no consensus regarding the
definition of Intractable Seizures.
Seizures which persist despite
appropriate therapy.
 Persistent seizures in spite of
adequate trials of 2 or more first and
second line drugs dosed to maximally
tolerated levels within an acceptable
therapeutic range.
Types of Intractable Seizures
 True intractable epilepsy
 Pseudo intractable
Medically and Surgically
Intractable Epilepsy
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Not accessible for resective surgery
Failure of resection surgery
Palliative surgery not applicable
Failure of palliative surgery
Favorable Factors for Seizure
Remission-Clinical
 Normal intellectual development
 Normal neurological exam
 Absence of any clinical or
imaging evidence of brain
damage
Favorable Factors for Seizure
Remission-Seizure related
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Age of onset of Epilepsy > 2
Only one type of seizure
Low frequency of seizures
No tonic-atonic-astatic seizures
Rapid remission with first drug
Brief period of poor control
No episodes of SE
A benign syndromic diagnosis
Favorable Factors for
Seizure Remission-EEG related
 Normal EEG at onset of RX
 Rapid improvement, normalization of
EEG
 Normal background features on EEG
 No slowing or slow spike waves
Approach to the Person with
Intractable Seizures
 Is it Epilepsy?
 Have appropriate drugs been
prescribed?
 Have drugs been taken as prescribed?
 Does person uniquely metabolize
drug?
 Have seizure precipitants been
controlled for?
Intractable Epilepsy (continued)
 Every PWE deserves a careful
evaluation if intractable
Intractable Epilepsy (continued)
Presurgical evaulation
Record habitual seizures
Appropriate imaging
Not all MRI's of equal quality
Functional Imaging to better define
Epileptogenic Zone: SPECT, PET,
FMRI, MEG
 Neuropsychology
 WADA
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Intractability (continued)
 Nociferous Cortex (NC) seizure
causing
 Eloquent Cortex (EC) Functionally
important
 If all data supports hypothesis that
NC can be removed sparing EC,
patient is a surgical candidate
Goals of Epilepsy Surgery
 Surgery freedom or significant
reduction of seizure burden to improve
quality of life without compromise of:
 1. Memory 2. Cognition 3. Language
4. Mood stability
 If risks exceed benefits, offer:
 1.VNS 2. Ketogenic Diet 3. Palliative
procedures 4. Participation in clinical
trials
Issues of Importance in Managing
Epilepsy in People with IDD-Seizure
Precipitants
 Fever-may be hard to
document
 Infections-may be
hard to identify
 Hypoglycemia-delay in
recognition
 Stress-may not be
articulated
 Etoh withdrawal-may
not be suspected
 Hyperventilation-may
be syndrome related
 Medicationsantidepressents, mood
stabilizers, and mania
drugs that cause
seizures
 Abrupt discontinuation
of meds-benzo's/barbs
used for behavior
intermittently and
withdrawal seizures
Conditions Often Misdiagnosed
as Epilepsy in the IDD
 Sudden
aggression,mood
shifts
 Self abuse
 Bizarre behavior
 Movement
disorders
 Staring
 Eye blinking
 Nystagmus
 Exaggerated startle
 Lethargy
Issues and Challenges in Diagnosing
and Caring for Individuals with Epilepsy
and IDD
 It can be difficult to
extract a history from
the client, due to
language problems
and cognitive
limitations
 Lack of caretakers
knowledge base,
willingness to be part
of the care delivery
team- "I'm just the
driver doc!"
 Poor documentation of
relevant features of
event (due to our
inaccessibility for
teaching)
 Diagnostic tests may
require cooperation,
sedation, can limit
diagnostic yield of:
EEG, neuropsych,
WADA, some
functional imaging
Issues and Challenges in Diagnosing
and Caring for Individuals with Epilepsy
and IDD-continued
 Individuals with IDD
have increased
sensitivity to
neuropsychiatric drug
Adverse Effects
 Limited detection of
AE's that may be
subjective
 Paradoxical
sensitivities to AE
(opposite effects)
 Increased risk of
seizure exacerbation
(DPH)
 Increased prevalence
of psychiatric, medical
comorbidities
 Political-economic
trends, limited access
 Indifference,
prejudice born of
ignorance and greed
 Social Darwinian life
boat ethics
Issues and Challenges in Diagnosing
and Caring for Individuals with Epilepsy
and IDD-continued
 Prejudicial and
Discriminatory
resource
allocation-The IDD
with Epilepsy will
never drive, work,
and pay taxes, why
commit limited
resources?
 Limited access to
quality social
services,
counseling,
vocational
rehabilitation,
Psychiatric services
Abbreviations
 IDD-Individual with
Developmental
Disabilities
 AE-Adverse Effects
 QOL-Quality of Life
 VNS-Vagus Nerve
Stimulation
 NC-Nociferous Cortex
 EQ-Eloquent Cortex
 PWE-Persons with
Epilepsy
 MMR-mild mental
retardation
 SMR-Severe mental
retardation
 PDD-Pervasive
Development Disorder
 TBI-Traumatic Brain
Injury
 CP-Cerebral Palsey