Download 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      

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

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* *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 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    

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