Seizure And Epilepsy

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NE URO LO GY

17

SEIZURES AND EPILEPSY DR DAYRIT AND DRA DE GUZMAN

DEFINITION  OF  TERMS   • SEIZURE   ü Transient   &   reversible   alteration   of   behavior   caused   by   a   paroxysmal,   abnormal   &   excessive   neuronal   discharge   ü Symptom  or  sign   • Seizure  à  Actual  attack   • CEREBRAL  PROBLEM  à  Not  spinal  cord  or  muscle   problem   • IF  YOU  HAVE  RECURRENT  SEIZURES  –  YOU  HAVE   EPILEPSY   • SEIZURE   DISORDERS   –   SYNONYMOUS   WITH   EPILEPSY   [the   term   epilepsy   is   usually   not   used   because  it  brings  stigma]     • EPILEPSY   ü 2   or   more   seizures   not   directly   provoked   by   intracranial   infection,   drug   withdrawal,   acute   metabolic  changes  or  fever   ü OLD   DEFINITION   –   “Due   to   an   excessive   and   disorderly   discharge   of     cerebral   nervous   tissue   on   muscles”   ü Diagnosis     • Epilepsy  à  Disorder/Disease   • When  you  write  the  diagnosis,  you  put  EPILEPSY   • MANIFESTED  BY  SEIZURES     • CONVULSION   ü Intense   paroxysm   of   involuntary   repetitive   muscular   contractions   ü Motor  component  of  seizure   • TAKE  NOTE:  Not  all  seizures  are  convulsions!     • • • •

INCIDENCE  OF  EPILEPSY   Prevalence:  5-­‐10  per  1000   44  cases  per  100,000  persons  each  year  (US  data)   10%  will  experience  a  seizure  by  age  80   Bimodal  Distribution:   ü 1st  year  of  life   ü Over  60  years  old   • Pediatric  and  geriatric  population  

• Most  common  etiology  à  IDIOPATHIC   • 2/3  of  all  epileptic  seizures  begin  in  childhood   • 75%  unclear  etiology  à  Idiopathic     CLASSIFICATION  OF  SEIZURES   SEIZURES   HAVE   BEEN   GROUPED   IN   SEVERAL   WAYS:   • International  League  Against  Epilepsy  (ILAE)   • Philippine   Version:   Philippine   League   Against   Epilepsy  (PLAE)   ACCORDING  TO…   • Presumed  Etiology   ü Idiopathic  (Primary)   • Usually  childhood  onset  or  with  family  history   ü Symptomatic  (Secondary)   • Secondary   to   a   tumor,   an   old   stroke,   previous   infection  (meningitis)   • Site  of  Origin   • Example:   Coming   from   the   Temporal   Lobe   à   Temporal  Lobe  Epilepsy   • Clinical  Form   ü Generalized   ü Focal   • Frequency   ü Isolated   ü Cyclic   ü Repetitive   ü Closely  spaced  sequence  (Status  Epilepticus)   • Special  Electrophysiologic  Correlates   • Based  on  Electroencephalogram  (EEG)     DISTINCTION  IS  MADE  BETWEEN:   • Classification   of   Seizures   (clinical   manifestations   of   epilepsy;  grand  mal,  petit  mal,  etc.)  AND     • Classification   of   Epilepsies   or   Epileptic   Syndromes   which  are  disease  constellations              

LEA THERESE R. PACIS + STEPHEN GABASAN J

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  CLASSIFICATION   BASED   ON   THE   INTERNATIONAL   CLASSIFICATION  OF  EPILEPTIC  SEIZURES:   • PARTIAL/FOCAL:   occur   within   discrete   regions   of   the   brain  [one  side  of  the  brain]   ü Simple  Partial  –  MOST  COMMON   • Intact  level  consciousness   - Begin   with   MOTOR,   SENSORY   or   AUTONOMIC   phenomena   depending   on   the   cortical   region   affected   o MOTOR   –   MOST   COMMON   (Jacksnonian   March)   • Can  occur  when  the  patient  is  awake  or  asleep   • As   compared   to   tremors  à   Only   seen   when   the   patient  is  awake   • (+)   Todd’s   Paralysis   –   transient   hemiparesis   after  an  attack   o

SOMATOSENSORY  or  Special  Sensory  

• Patient  sometimes  feel  numb  or  “goosebumps”   o AUTONOMIC   o p   • Vomiting     • Diaphoretic   • Cold  clammy  skin     o PSYCHIC  –  COGNITIVE   COMPLEX  PARTIAL  -­‐  with  impaired  consciousness   o AURA  followed  by  impaired  consciousness   o Patient  may  appear  awake  but  lost  contact  with   the   environemtn   and   do   not   respond   to   instructions  or  questions  for  few  minutes   o Usually   stare   or   remain   motionless   or   engage   in   repetitive   semi-­‐purposeful   motor   behaviours   called   AUTOMATISMS   –   chewing,   grimacing,  gesturing,  lip  smacking,  snapping  of   fingers   o May  become  hostile  or  aggressive  if  restrained   o Seizure   discharge   arise   form   temporal   lobe   or   medial  frontal  lobe   o Symptoms   take   many   forms   but   usually   sterotyped   o Epigastric  symptoms  are  common   o Affective   (fear),   Cognitive   (déjà   vu),   sensory   (olfactory  hallucinations)     • GENERALIZED   ü Rise  from  both  cerebral  hemispheres  simultaneously   ü  Bilaterally  symmetrical  and  without  local  onset     ü Types:   - Absence  –  common  in  chilren   ü

• Also  called  Petit  Mal  Seizure  

- Myoclonic  –  seen  in  patients  in  the  ICU   • “Sleep  jerks”   - Tonic   - Clonic   - Atonic  –  common  in  children   • Suddenly  falls  down  due  to  loss  of  muscle  tone   - Tonic-­‐Clonic       • MOST   COMMON:   Generalized   Tonic-­‐Clonic     Seizures   (GTC)   à   Also   called   Grand   Mal     Seizures  J     • TONIC   PHASE   –   initial   manifestations   are   unconsiousness   and   tonic   contractions   of   limb   muscles  (10-­‐30  seconds)   o Expiration  induces  vocalizations  (cry  or   moan)   o Cyanosis   o Contractions  of  masticatory  muscles   o Patient  falls  to  the  ground   • CLONIC   PHASE   –   alternating   muscle   contraction     and   relaxation   of   symmetric   limb   jerking  (30-­‐60  secs  or  longer)   o Ventilatory   efforts   return   immediately   after   cessation   of   tonic   phase   and   cyanosis  clears   o Mouth  may  froth  with  saliva   o Muscles  may  become  flaccid   o Sphincter   relaxation   may   produce   urinary  incontinence     o May   remain   unconscious   for   variable   period  of  time   o If  >2  mins  –  STATUS  EPILEPTICUS   • RECOVERY   PHASE   –   regaining   consciousness   maybe   followed   by   post-­‐ictal   confusion   and   or   headache   o Full   orienation   in   10-­‐30   minutes   (longer   with   status   or   pre-­‐existing   structural  or  metabolic  brain  disorders)   MECHANISM   OF   SEIZURE   INITIATION   AND   PROPAGATION:   1. high  frequency  of  action  potentials   2. hypersynchronization   EPILIPTOGENESIS   –   transformation   of   a   normal   neuronal   netweok   that   becomes   chronically   hyperexcitable,     “KINDLING   PHENOMENON”   –   a   result   of   repeated   stimulation   of   subconvulsive   electrical   pulses   form   an   established   focus   elsewhere;   controversial   in   humans  

LEA THERESE R. PACIS + STEPHEN GABASAN J

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Epilepsy   may   evolve   with   increase   in   frequency,   duration  or  spread  of  the  seizures   o Interictal  EEG  background  is  abnormally  slow   o Spontaneous  resolution  of  epilepsy  is  unusual   o Prognosis   depends   on   the   underlying   neurologic   condition   II. Generalized  Epilepsies  and  Syndromes   ü Idiopathic,   with   age-­‐related   onset   or   Primary   Generalized  (BNFC,  absence,  JME,  etc.)   ü Symptomatic   or   Secondary   Generalized   (West   syndrome,  Lennox-­‐Gastaut  syndrome)     ü IDIOPATHIC  EPILEPSY   o

• NATURE   OF   THE   DISCHARGING   LESION   IN   EPILEPSY:   -­‐ SEIZURES   GENERATION   REQUIRE   THREE   CONDITION   o Population   of   pathologically   excitable  neurons   o An   increase   in   excitatory   glutaminergic   activity   through   recurrent   connetions   to   spread   the   discharge  -­‐  ↑  GLUTAMATE   o A   reduction   in   the   activity   of   the   normally   inhibitory   gabanergic   projections  -­‐  ↓  GABA   •

 

SPECIAL   EPILEPTIC   DISORDERS   –   seen   in   children   o Myoclonus  and  myoclonic  seizures   o Reflex   e pilepsy   o Acquired  aphasia  with  convulsive  disorder   o Febrile   and   other   seizures   of   infancy   and   childhood   o Hysterical  seizures  –  “psychogenic”  

INTERNATIONAL   CLASSIFICATION   OF   EPILEPSIES   AND  EPILEPTIC  SYNDROMES   I. Localization-­‐related   ü Idiopathicà      IDIOPATHIC  EPILEPSY  or  “PRIMAY   EPILEPSY”     o There   is   no   underlying   cause   indetifies   other   than  an  heriditary  predisposition   o Presumed  to  be  GENETIC  of  origin   o Ofthen  with  a  (+)  family  history   o As   a   rule,   begins   early   in   life   –   20   years   old   and   below   o Not   associated   with   evidence   of   structural   or   nervous  or  mental  disorders   o Normal  interictal  EEG  background  and  MRI   • Inter-­‐ictal   à   Period   in   between   episodes   of   seizures   (Time   wherein   the   patient   is   not   undergoing  seizure)   Favorable  response  to  antiepileptic  therapy   Benign   prognosis   with   spontaneous   resolution   in   time   ü Symptomatic  à  Secondary  Epilepsy   o Seizures   have   an   identifiable   and   acquired   structural  cause   o o

• Difficult  to  control,  treat,  and  manage   o o

There   is   evidence   for   focal   or   generalized   neurological  disease   Mental  retardation  or  deterioration  may  occur  

IDIOPATHIC  EPILEPSY:   o as  a  rule,  begin  early  in  life   o not  associated  with  evidence  of  structural,   nervous,  or  mental  disorders   o normal  interictal  EEG  background   o favorable  response  to  anti-­‐epileptic   therapy   o benign  prognosis  with  spontaneous   resolution  in  time  

•

  ü ü ü

ü

ü ü ü ü

ü

ABSENCE  or  PETIT  MAL  SEIZURES   Pyknoepilepsy  –  “Pykno”  –  compact  or  dense”   Features.  brevity,  frequency,  paucity  of  motor  activity   "A  moment  of  absentmindedness  or  day  dreaming"   o Without   a   warning....   sudden   interruption   of   consciousness....   stares   and   briefly   stops   talking   or   ceases  to  respond   o 10   percent   are   completely   motionless;   the   rest   have  fine  clonic  (myoclonic)  movements  of  eyelids,   facial  muscles  or  fingers,  at  a  rate  of  3  per  second   EEG   pattern   of   generalized   3-­‐per-­‐second   spike-­‐ and-­‐wave  pattern   After   2   to   10   seconds,   or   longer,   patient   reestablishes   full   contact   with   the   environment   and   resumes   pre-­‐seizure   activity.   Hyperventilation  may  induce  an  attack   As  many  as  several  hundred  may  occure  in  a  day   Rarely  begins  before  4  years  or  after  puberty   Attacks   tend   to   diminish   during   adolescence   and   then   disappear,   to   be   raplaced   by   other   forms   of   generalized  seizure   "SECONDARY"  epilepsy   o Seizures   have   an   identifiable   and   acquired   structural  cause   o There   is   evidence   for   focal   or   generalized   neurological  disease   o mental  retardation  or  deterioration  may  occur   o Rarely  begins  before  4  years,  or  after  puberty   o As  many  as  several  hundreds  may  occur  in  a  day  

LEA THERESE R. PACIS + STEPHEN GABASAN J

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Attacks  tend  to  diminish  during  adolescence  and  then   disappear,   to   be   replaced   by   other   forms   of   generalized  seizures     MESIAL  TEMPORAL  LOBE  EPILEPSY   Hippocampal  sclerosis:   o there   is   selective   loss   of   neurons   in   the   dentate   hilus   and  the  hippocampal  pyramidal-­‐cell  layer   o relative   preservation   of   dentate   granule   cells   and   a   small   zone   of   pyramidal   cells   (in   the   cornu   ammonis,   field  2,  of  the  hippocampus)   the   dense   gliosis   that   accompanies   the   loss   of   neurons   causes  shrinkage  and  hardening  of  tissue   the  term  "mesial  temporal  sclerosis"  has  also  been  used  for   this   lesion,   because   often   there   is   neuronal   loss   in   the   neighboring   entorhinal   cortex   and   amygdaladebate   about  whether  hippocampal  sclerosis  is  a  cause  or  an   effect  of  seizures   it   has   been   seen   in   a   wide   variety   of   epileptic   conditions,   including   cryptogenic   temporal-­‐lobe   epilepsy   and   epilepsy   that   follows   febrile   seizures   or   other   brain   insults  early  in  life,  as  well  as  in  animal  models  of  head   injury  and  seizures  induced  by  chemicals   Hypotheses  about  the  mechanism  of  epileptogenesis   § structural   r eorganization   § selective   n euronal   l oss   § neurogenesis   § molecular   alterations,   such   as   changes   in   neurotransmitter  receptors   Some   investigators   have   suggested   that   the   selective   vulnerability  of  certain  neurons  may  be  a  mechanism  of   epileptogenesis  in  hippocampal  sclerosis   In  animal  models,  excitatory  interneurons  located  within   the   dentate   gyrus,   which   normally   activate   inhibitory   interneurons,  appear  to  be  selectively  lost    Loss   of   these   excitatory   cells   would   be   expected   to   impair   the   inhibitory   feedback   and   feed-­‐forward   mechanisms   that   act   on   dentate   granule   cells,   resulting   in  hyperexcitability   An   intriguing   hypothesis   lies   in   the   phenomenon   of   neurogenesis   § Almost   all   neurons   in   the   brain   are   postmitotic   and  do  not  divide  in  adults,  but  progenitor  cells  in   the   dentate   gyrus   of   the   hippocampus   are   known   to  divide   § Postnatal   neurogenesis   in   the   hippocampus   can  occur  throughout  life   § The   potential   clearly   exists   for   an   imbalance   between  excitation  and  inhibition  as  new  neurons   differentiate  and  form  synaptic  connections  

§

o

ü

o o

o

o

o

o

o

o

§

ü ü

ü

ü

changes   at   the   molecular   level   -­‐   the   most   prominent   of   these   are   alterations   in   the   composition  and  expression  of  GABAA  receptors  on   the  surface  of  hippocampal  dentate  granule  cells   normally,  GABAA  receptors  in  adults,  which  consist   of  five  subunits,  serve  as  inhibitors,  hyperpolarizing   the   neuron   by   allowing   passage   of   chloride   ions  when  activated  

PATHOLOGY  OF  EPILEPSY   Primary  generalized  epilepsies:  majority  are  grossly   and  microscopically  normal   Symptomatic  epilepsies:  neuronal  loss  and  gliosis,   porencephaly,  hamartoma,  heterotopia,  dysgenetic  cortex,   vascular  malformations,  and  tumor   Focal  epilepsies:  gliosis,  fibrosis,  vascularization,   meningocerebral  cicatrix,  hippocampal  sclerosis   SEIZURE  IN  ADULTS   Secondary  to  medical  diseases   o Withdrawal  seizures  —  AED  withdrawal   o Infections  —  CNS,  or  systemic  infections   o Metabolic  encephalopathies  —  hypoglycemia,   hyponatremia,  uremia,  hepatic  encephalopathy   o Medications  as  a  cause  of  seizures  —  antibiotics   (carbapenem),  tricyclic  antidepressants   o Global  arrest  of  circulation  and  cerebrovascular   diseases  —  hypoxic  encephalopathy   o Acute  head  injury     SEIZURE  IN  ADULTS  

Sodium  Channels   o Familial  generalized  seizures   o Benign  Familial  neonatal  convulsions   ü Potassium  Channels   o Benign   i nfantile   e pilepsy   o Episodic  ataxia  type  1   ü Ligand-­‐gated  Channels   o Autosomal  dominant  nocturnal  frontal  seizures   o Familial  generalized  and  febrile  seizures   o Juvenile  myoclonic  epilepsy     ü Calcium   C hannel   o Episodic  ataxia  type  2     DIAGNOSTICS   ü

ü

EEG   o Indications:   § To  confirm  the  diagnosis  of  epilepsy   § An  adequate  EEG  should  include  a  sleep  and   awake  recording  

LEA THERESE R. PACIS + STEPHEN GABASAN J

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To  classify  the  seizure  type   To  make  a  diagnosis  of  non-­‐convulsive  status   epilepticus   Role  of  Interictal  EEG  in  Epilepsy   § Confirms  clinical  diagnosis  of  epilepsy   § Classification  of  seizure  types   § Definition  of  Epileptic  syndromes   • Monitoring  of  response  to  AED  treatment   • Evaluation   of   patients   with   single   seizures   § Guide   in   the   decision to discontinue AED § §

o

treatment

ü

BRAIN  IMAGING  (MRI  or  CT)     o Indications:   § Partial  onset  seizures  at  any  age   § Adult  onset  seizure  of  any  type   § Presence   of   focal   neurologic   deficit   CT  and   MRI  allow  identification  of  structural  lesions     • MRI   higher   specificity   and   sensitivity   in   diagnosing   congenital   brain   anomalies,   hippocampal   sclerosis,   AV   malformations,   tumors   • CT  scan  :  if  MRI  is  not  available  or  in  those  with   pacemakers.   aneurysm   clips,   severe   claustrophobia   ü DIFFERENTIAL  DIAGNOSES   o Syncope/Faints   o TIA   o Drop  attacks   o Complicated  migraine   o Hypertensive  emergency   o Psychiatric  disorders  

    TREATMENT   ü

ü

CRITERIA   FOR   STARTING   ANTIEPILEPTIC   DRUG   (AED)   o The  diagnosis  of  epilepsy  must  be  firm  and  definite   o Risk  of  seizure  recurrence  must  be  sufficient   o Seizure   type   or   epilepsy   syndrome   The   AIM   of   Treatment   with   AEDs   is   to   prevent   seizures   for   the   following  reasons:   §  Prevent  injury   § Avoid  disruption  to  employment  or  education   § Minimize   the   social   consequences   of   the   condition   § Try  to  prevent  status  epilepticus   WHEN  T O  R EFER  T O  A  SPECIALIST  

o o o o o

Need   t o   c onfirm   d iagnosis   Poor  seizure  control   Severe/toxic   s ide   e ffects   Patients  planning  a  pregnancy   Seizure  free  patient  considering  drug  withdrawal  

CAUSES  OF  RECURRENT  SEIZURES  IN  DIFFERENT  AGE   GROUPS  (Adams,  19th  Edition)   • Neonates   ü Congenital  Maldevelopment   ü Birth  Injury   ü Anoxia   ü Metabolic  Disorder   - Hypocalcemia   - Hypoglycemia   - Vitamin  B6  Deficiency   - Biotinidase  Deficiency   - Phenylketonuria   - Others     • Infancy  (1-­‐6  months)   ü As  above   ü Infantile  Spasms  (West  Syndrome)     • Early  Childhood  (6  months  –  3  years)   ü Infantile  Spasms   ü Febrile  Convulsions   ü Birth  Injury  and  Anoxia   ü Infections   ü Trauma   ü Metabolic  Disorders   ü Cortical  Dysgenesis   ü Accidental  Drug  Poisoning     • Childhood  (3—10  years)   ü Perinatal  Anoxia   ü Injury  at  birth  or  later   ü Infections   ü Thrombosis  of  Cerebral  Arteries  or  Veins   ü Metabolic  Disorders   ü Cortical  Malformations   ü Lennox-­‐Gastaut  Syndrome   ü “Idiopathic”  (Probably  Inherited)   ü Rolandic  Epilepsy     • Adolescence  (10-­‐18  years)   ü Idiopathic   Epilepsy   (Including   genetically   transmitted   types)   ü Juvenile  Myoclonic  Epilepsy   ü Trauma   ü Drugs  

LEA THERESE R. PACIS + STEPHEN GABASAN J

5

  • Early  Adulthood  (18-­‐25  years)   ü Idiopathic  Epilepsy   ü Trauma   ü Neoplasm   ü Withdrawal  from  alcohol  or  other  sedative  drugs     • Middle  Age  (35-­‐60  years)   ü Trauma   ü Neoplasm   ü Vascular  Disease   ü Alcohol  or  other  drug  withdrawal     • Late  Life  (Older  than  60)   ü Vascular  Disease  (usually  postinfarction)   ü Tumor   ü Abscess   ü Degenerative  Disease   ü Trauma      

WHAT  DRUG  TO  CHOOSE:  

Lamotrigine   MYOCLONIC  

Valproate  

Levetiracetam   Zonisamide   Partial  

Carbamazepine  

Valproate  

Phenytoin  

Lamotriging   Oxcarbazepine   Levetiracetam  

 

Drug  

Partia l  

Generaliz ed   secondary  

Toni c   Clon ic  

Absenc e  

Myoclon us  

Mixe d  

Carbamazep ine  

+  

+  

+  

x  

x  

o  

Clonazepam  

+  

+  

+  

?  

 

?  +  

Phenobarbit al  

+  

+  

+  

o  

?  +  

?  

Phenytoin  

+  

+  

+  

x  

x  

o  

Valproate  

+  

+  

+  

+  

+  

+  

MEDICATION  

SIDE  EFFECTS  

Phenobarbital  

Sedation,  sleepiness,   hyperactivity,  weakness  

Dilantin  –   Phenyhydantoin,   Phenytoin  

Dizziness,  poor  balance,   weakness,  thick  gums,   excessive  hair  growth,   allergic  rash,  SJS  

Tegretol  –   Carbamazepine  

Allergic  rash,  dizziness,   sleepiness,  weakness,   headache,  gastric   discomfort,  SJS,  leukopenia,   hyponatremia  

Epival/Depakene  

Transient  loss  of  appetite,   alopecia,  nausea,  vomiting,   weight  gain  

Rivotril  –  Clonazepam  

Sleepiness,  weakness,  in   chidren  –  increase  bronchial   secretions  

 Trileptal  –   Oxcarbazepine  

Headache,  dizziness,   sleepiness,  nausea  and   hyponatremia  

EFFICACY   ESTABLISHED   AED   AGAINST   COMMON   SEIZURE  D RUGS:   SEIZURE  TYPE  

FIRST  LINE    

SECOND  LINE  

TONIC  CLONIC  

Valproate  

Lamotrigine  

Carbamazepine   Phenytoin   ABSENCE  

Valproate  

Topiramate  

Ethosuximide  

LEA THERESE R. PACIS + STEPHEN GABASAN J

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Neurontin  –  Gabapentin  

Sleepiness,  fatigue,   dizziness,  weakness  and   rashes  

Lamictal  –  Lamotrigine  

Allergic  rash,  drowsiness  

Topamax  

Weight  loss,  mood  changes,   sleepiness,  dizziness  and   kidney  stones  

  -­‐

start   at   the   lowest   computed   appropriate   dose   and   increase   slowly   until   seizure   control   is   achieved   or   side  effects  develop   Titrate  slowly  to  allow  tolerance  to  CNS  side  effects   Keep   the   regimen   simple   wth   OD-­‐   BID   dosing,   if   possible    

-­‐ -­‐

SURGICAL  TREATMENT   -­‐ -­‐ -­‐

temporal   lobectomy-­‐   50%   improvement   in   5   years   in  complex  partial  seizures   Corpus   callostomy   –   recommended   for   cotnrol   of   intractable   partial   and   secondary   generalized   seizures  –  especially  atonic  drop  attacks   Hemispherectomy   –   recommended   for   severe   and   extensive   unilateral   cerebral   disease   with   intractable  m otor  seizures  and  hemiplegia   o Rasmussens   encephalitis,   Sturge-­‐Weber   syndrome  and  Large  porencephalic  Cysts  

6-­‐9  

ABCs,   Establish   Fosphenytoin   IV,   Blood   work,   (20  m g/kg  PE)   Give   Thiamine   and  glucose  

10-­‐20    

Lorazepam   (0.1     mg/kg)   or   Diazepam   (0.2   mg/kg)  

21-­‐40  

Phenytoin   (15-­‐ 20  m g/kg)  

Add   phenytoind   (5   gm/kg)   /     fosphenytoin   (5   mg/kg)   followed   by  Phenobarbital   (20   m g/kg)   or   go   to  anesthesia  

41-­‐60  

 

Add   phenobarbital   (5-­‐10  m g/kg)  

Greater  than  60  

Add   phenytoin   Anesthesia   with   (5   mg.kg)   x   2   Midazolam   or   followed   by   Propofol   phenobarbital   (20   mg/kg)   followed   by   pentobarbital  

ROLE  OF  KETOGENIC  AND  MEDIUM  CHAIN   TRIGLYCERIDE  DIET:  

FACTORS  RELATED  TO  SUCCESSFUL  WIDTHRAWAL  OF   AEDs   -­‐ -­‐ -­‐ -­‐

-­‐ -­‐

Single  type  of  lesion   Normal  neurologic  examination   Normal  IQ   Normal  E EG  following  treatment  

-­‐ -­‐

COMPLICATIONS  OF  THE  DISEASE:   -­‐

-­‐ -­‐

STATUS  EPILEPTICUS   o Recurrent   generalized   convulsions   at   a   frequency   that   prevents   regaining   of   conciousness  in  the  interval  between  seizures   o Prolonged   convulsive   status   (longer   than   30   mins.)   carries   high   risk   for   serious   neurologic   sequelae  (“epileptic  encephalopathy”   o MANAGEMENT  

Time  in  M inutes  

Standard   Treatment  

0-­‐5  

Diagnosis   and   Lorazepam   (1.0   assessment   mg/kg)  

-­‐

Proposed   Treatment  

exact  m echanism  –  U NKNOWN     chronic  ketosis  induced  by  a  diet  high  in  fat  results   in   improvement   of   cerebral   energetics   and   augmentation  of  G ABA  effects  (?)   used  m ainly  in  children  (1-­‐10  years  old)   2/3   reduction   in   seizure   frequency   and   reduction   of  A ED  usage   Effective  in  refractory  epilepsy   CHARACTERISTICS:   o Consists  of  daily  regimen  of  1g.kg  p rotein   o Enough   fat   to   make   up   desired   caloric   requirements   o Very  small  amount  of  carbs   o Ketogenic  :  antiketogenic  potential  ratio  –  3:1   Caloric  D istribution:   o Ketogenic  –  87%  fat,  6  %  CHO  and  7%  CHON   o MCT   –   60%   MCT,   11%   fat,   19%   CHO   and   10%   CHON     o Thus  m ore  palatable  and  no  increase  in  plasma   cholesterol    

     

LEA THERESE R. PACIS + STEPHEN GABASAN J

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