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Issues in Developmental Disabilities Epilepsy in the Intellectually and Developmentally Disabled
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Transcript of Issues in Developmental Disabilities Epilepsy in the Intellectually and Developmentally Disabled
Issues in Developmental DisabilitiesEpilepsy in the Intellectually and
Developmentally Disabled
Lecture Presenter:
Christopher M. Inglese, M.D.Regional Epilepsy CenterSt. Luke's Medical Center
Milwaukee,Wisconsin
Video of Inglese
Epilepsy In The Multiply-Handicapped Worldwide movement to de-
institutionalize 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
AffectiveMood 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 & DefinitionsDiagnostic 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. By Cause or Etiology Idiopathic Cryptogenic Symptomatic
By Clinical AppearanceConvulsive Non Convulsive
Grand Mal Petit Mal
Major Motor Minor Motor
By Electro-Clinical Characteristics*
Partial Onset Generalized Onset
*Determined by the Anatomic Substrate of the Seizure Generator
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
Cognitive Neuromotor Sensory
Attentional Behavioral self
regulatory Affect and mood
Can tremendously potentiate the impact of a disability when added to co-existing challenges, comorbidities
General Principles of Management-Diagnostic Is it Epilepsy? Both epileptic and
non-epileptic seizures?
Are seizures caused exclusively by controllable medical conditions?
Cardiac?
Hemodynamic-vascular?
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
Medications-antidepressents, 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