Issues in Developmental Disabilities Epilepsy in the Intellectually and Developmentally Disabled Lecture Presenter: Christopher M. Inglese, M.D. Regional.
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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 Appearance
Convulsive 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
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