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 Multiply-
Handicapped
 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
 Cognitive
 Motoric
 Sensory
 Attentional
 Behavioral
 Affective

Cognitive Mental Retardation
 SMR
 MMR
 Learning Disabilities
 Apraxias/Dyspraxias

Motoric Cerebral Palsy
 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
 Impulsivity
 Hyperkinesis
 Affective Storms
 Episodic Dyscontrol
 Self Injurious Behavior
 Aggression

Affective
Mood Disorders
 Anxiety
 Depression
 Bipolar, Cyclic mood disturbances
 Thought Disorders

Autistic Spectrum Disorders
 Aspergers
 Hellers
 Retts
 Kanners (classical autism)
 PDD NOS

Common Medical Comorbidities
 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

The outward manifestations of the epilepsies can be purely subjective, experiential, imposed emotions.

A predisposition for unprovoked, recurrent seizures by a proximate identifiable cause.

Collections of signs, symptoms from a common cause which define recognizable patterns of disease.

There are many ways to classify the epilepsies or seizures

Classifications cont.
 By Cause or Etiology
 Idiopathic
 Cryptogenic
 Symptomatic

By Clinical Appearance
Convulsive Non Convulsive
Grand Mal
Major Motor
Petit Mal
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
 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

International Classification of
Epileptic Seizures-Generalized
 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 Statistics-
Prevalence
 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

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
 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
 Tremor
 Dyskenesias associated =BPD
 Benign neonatal myoclonus
 Sleep myoclonus
 Apnea

Salient Nonepileptic Disorders at
Different Ages: Age 2-18 months
 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.
 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
 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
 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
 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

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