Issues in Developmental Disabilities Epilepsy in the Intellectually and Developmentally Disabled

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Issues in Developmental DisabilitiesEpilepsy in
the Intellectually and Developmentally Disabled

• Lecture Presenter
• Christopher M. Inglese, M.D.
• Regional Epilepsy Center
• St. Luke's Medical Center
• Milwaukee,Wisconsin

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Video of Inglese
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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

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Intellectual and Developmental Disabilities
Associated with Epilepsy

• Cognitive
• Motoric
• Sensory
• Attentional
• Behavioral
• Affective

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Cognitive Mental Retardation

• SMR
• MMR
• Learning Disabilities
• Apraxias/Dyspraxias

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Motoric Cerebral Palsy

• Spastic
• Extrapyramidal
• Developmental Dyspraxias
• Hypotonia
• Weakness

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Sensory

• Hearing Loss
• Visual Impairment
• Sensory Integration Dysfunction

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Attentional

• ADHD -Combined Type, Inattentive Subtype
• Primary Disorders of Vigilance
• Secondary Disorders of Vigilance

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Behavioral

• Impulsivity
• Hyperkinesis
• Affective Storms
• Episodic Dyscontrol
• Self Injurious Behavior
• Aggression

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AffectiveMood Disorders

• Anxiety
• Depression
• Bipolar, Cyclic mood disturbances
• Thought Disorders

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Autistic Spectrum Disorders

• Aspergers
• Hellers
• Retts
• Kanners (classical autism)
• PDD NOS

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Common Medical Comorbidities

• Congenital malformations
• Chromosomal Abnormalities
• Genetic Disorders
• Metabolic Disorders
• Static Enephalopathis

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Terminology DefinitionsDiagnostic Criteria for
Mental Retardation

• IQ lt 70
• Impairment in interpersonal relations, self-care,
  maturation
• Onset before age 18
• DSM IV 37.90

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Seizures

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

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Epilepsy

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

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Epileptic Syndromes

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

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The Classification of the Epilepsies

• There are many ways to classify the epilepsies or
  seizures

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Classifications cont.

• By Cause or Etiology
• Idiopathic
• Cryptogenic
• Symptomatic

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By Clinical Appearance
Convulsive Non Convulsive
Grand Mal Petit Mal
Major Motor Minor Motor
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By Electro-Clinical Characteristics
Determined by the Anatomic Substrate of the
Seizure Generator

• Partial Onset

• Generalized Onset

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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

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Why is Classification Important?

• Basic Science and Clinical Scientists must have
  uniformity of definitions in heterogeneous
  conditions
• Apples to apples, oranges to oranges

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Classification Facilitates Research

• Causal Mechanisms
• Treatments
• Outcomes
• Predispositions

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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

• Absence (typical and atypical)
• Myoclonic
• Tonic
• Clonic
• Atonic-astatic

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International Classification of Epileptic
Seizures-Unclassified

• Febrile Seizures
• Reflex Epilepsies
• Status Epilepticus

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Classification of Epilepsy Syndromes

• Idiopathic focal epilepsies
• Familial focal epilepsies
• Symptomatic and Cryptogenic focal epilepsies

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Idiopathic Generalized Epilepsies

• Reflex Epilepsies
• Epileptic Encephalopathies
• Progressive myoclonus epilepsies

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Epidemiology and Statistics-Prevalence

• Numerator-old and new cases
• Denominator-population at risk

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Epidedemiology (continued)

• Prevalence of MMR IQ lt 70 3.7-7.6 per 1000
• Prevalence of SMR IQ lt 50 2.8-4.6 per 1000
• Prevalence of epilepsy 4.0-8.8 per 1000
• Prevalence of MR in childhood epilepsy 31-41

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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)

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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

• Occupational restrictions
• Discrimination
• Impact on learning of ictus, interictal state,
  postical state

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Epilepsy
Can tremendously potentiate the impact of a
disability when added to co-existing challenges,
comorbidities

• Attentional
• Behavioral self regulatory
• Affect and mood

• Cognitive
• Neuromotor
• Sensory

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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?

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General Principles of TreatmentIs 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

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Generalized Principals of Treatment (continued)

• Discontinue meds whenever possible
• Consensus with client regarding treatment or
  discontinuation

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Salient Nonepileptic Disorders at Different Ages
Age 0-2 months

• Tremor
• Dyskenesias associated BPD
• Benign neonatal myoclonus
• Sleep myoclonus
• Apnea

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Salient Nonepileptic Disorders at Different Ages
Age 2-18 months

• Paroxysmal torticollis
• Opsoclonus-myoclonus syndrome
• Sandiffers syndrome
• Jactatio capitis
• Masturbation
• Paroxysmal choreo-athetosis
• GERD

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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

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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

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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

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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

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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.

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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.

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Types of Intractable Seizures

• True intractable epilepsy
• Pseudo intractable

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Medically and Surgically Intractable Epilepsy

• Not accessible for resective surgery
• Failure of resection surgery
• Palliative surgery not applicable
• Failure of palliative surgery

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Favorable Factors for Seizure Remission-Clinical

• Normal intellectual development
• Normal neurological exam
• Absence of any clinical or imaging evidence of
  brain damage

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Favorable Factors for Seizure Remission-Seizure
related

• Age of onset of Epilepsy gt 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

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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

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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?

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Intractable Epilepsy (continued)

• Every PWE deserves a careful evaluation if
  intractable

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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

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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

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Issues of Importance in Managing Epilepsy in
People with IDD-Seizure Precipitants

• Medications-antidepressents, mood stabilizers,
  and mania drugs that cause seizures
• Abrupt discontinuation of meds-benzo's/barbs used
  for behavior intermittently and withdrawal
  seizures

• 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

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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

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Issues and Challenges in Diagnosing and Caring
for Individuals with Epilepsy and IDD

• 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

• 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!"

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Issues and Challenges in Diagnosing and Caring
for Individuals with Epilepsy and IDD-continued

• Increased prevalence of psychiatric, medical
  comorbidities
• Political-economic trends, limited access
• Indifference, prejudice born of ignorance and
  greed
• Social Darwinian life boat ethics

• 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)

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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

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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