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An epileptic syndrome in which the primary clinical manifestation is

LANGUAGE REGRESSION

ACQUIRED AUDITORY AGNOSIA

  • ONSET, subacute, steady or stuttering & initially consists of loss of understanding of spoken language
  • EVENTUALLY, speech output is disrupted & paraphasias & phonologic errors appear
  • SEVERE, child becomes entirely mute & doesn’t respond to nonverbal sounds (acknowledge phone ringing-knocking on the door)
  • 200 cases described between 1968 & 1992
  • Review of 1,497 overnight video EEG performed over 5-yr interval, Van Hirtum-Das et al found 102 records showed significant sleep activation defined as a spike-wave index of 25% or higher
  • 20% --> LKS & 32% --> autistic
  • Unknown
  • Structural abnormalities
  • Preceding neurologic condition (meningitis-neonatal encephalopathy)
  • Immune disorder (45% IgG, 9% IgM anti-brain autoantibodies)
  • Genetic disorder

AGE OF ONSET

3 – 8 yrs

Peak 4 – 5 yrs

  • The apparently “generalized” epileptiform discharges --> caused by single primary focus with secondary bilateral synchrony
  • Activation of reticulo-thalamic-cortical system with secondary bilateral synchronization through corpus callosum

OTHER SYMPTOMS

Before onset, language & development --> unremarkable

Irritability

Hyperactivity

Poor reasoning

Aggressiveness

Attention-deficit disorder

Autistic-like behavior

Sz 70-80%

Typically infrequent

Easily treated

Types (generalized tonic-partial clonic-atypical absence+atonic)

Most common sz, eye blinking or ocular deviation, head drops, minor automatisms + 2ry generalization

AWAKE:

  • Variable
  • Focal (posterior temporal)
  • Multifocal
  • Generalized epileptic abnormalities
  • Normal

SLEEP:

  • Marked activation
  • Occupied 40-90% of the first slow-wave cycle
  • Long clusters of spike-wave activity, maximal over centrotemporal head regions but spread diffusely
  • Massa et al
  • Less activation of epileptiform discharges in stages 1 & 2

The intrasylvian cortex is the likely pacemaker of epileptic discharges (4 children)

Perisylvian spikes (13/19 pts)

  • 10 bilateral & 3 unilateral
  • 4 --> spikes outside perisylvian region
  • 2 --> no spikes

STRUCTURAL NEUROIMAGING

FUNCTIONAL NEUROIMAGING

Gaggero et al (10 children):

  • 6 --> Reduced blood flow during drowsiness
  • 4 --> Correlated with the site of most prevalent EEG discharges
  • Longer duration of CSWS --> more likely abnormal study

De Tiege et al (18 children):

  • 10 --> hypermetabolism, maximally in parietotemporal junction
  • Furthermore, significant association existed between absence of hypermetabolic cerebral region & corticosteroid treatment before study

Focal hypermetabolism --> active phase

Evolution to focal hypometabolism or normal --> recovery

All children with regional hypermetabolism --> hypometabolism in frontal regions --> attention deficit

  • No polysomnographic studies
  • However, Beaumanoir, notes 10% --> absence of sleep spindles

Goal:

  • Control sz
  • Improve neuropsychological function
  • Contraindicated --> phenytoin, CBZ & barbiturates --> worsen neuropsychological outcome & EEG discharges
  • Avoid polypharmacy
  • Valproic acid
  • Ethosuximide
  • Benzodiazepines
  • Keppra
  • Sulthiame

Diazepam:

3-4 weeks

0.5-0.75 mg/kg/d

Side effects: hypotonia, hyperexcitability, problematic behavioral disinhibition

  • Prednisone 2-5 mg/kg/d
  • Methylprednisolone 20 mg/kg/d X 3 days
  • ACTH 80 IU/d with 3-month taper
  • Earlier steroids --> shorter duration --> better outcome
  • ?dose vs ?duration
  • Tapering --> relapse
  • 2 g/kg over 4 days
  • ?more studies
  • Multiple subpial transections
  • Placing parallel slices through the cortex to disrupt horizontal cortical synchronizing networks yet leaving subcortical-cortical input intact
  • More studies
  • Ketogenic diet
  • VNS

Sz --> resolve or markedly decrease by puberty

EEG --> resolve by puberty

Neuropsychological --> problematic (10-44% normal language & intelligence)

  • Uncommon (underrecognized)
  • If clinically suspected, must obtian adequate sleep EEG + slow-wave sleep
  • Treatment must extend beyond just controlling sz
  • Early treatment with effective therapy is essential for improvement in neuropsyhological outcome
  • Not good evidence to guide treatment decisions at this time
  • AED have minimal role
  • Multicenter studies to compare therapeutic efficacy & tolerability of the various regimens should be considered

THANK YOU

SUMMARY

Nickels et al, 2008

PROGNOSIS

Nickels et al, 2008

OTHER THERAPIES

IN THE NAME OF ALLAH,

MOST GRACIOUS, MOST MERCIFUL

Journal of clinical neurophysiology, 2003

LANDAU-KLEFFNER SYNDROME

SURGERY

BY TAHANI JAMALY

INCIDENCE

IVIG

Mikati et al, Nickels et al

Nickels et al, 2008

STEROIDS & ADRENOCORTICOTROPIC HORMONE

ETIOLOGY

Connolly, et al, 1999

HIGH-DOSE BENZODIAZEPINES

AED

TREATMENT

PATHOPHYSIOLOGY

EFFECT ON SLEEP PATTERNS

EEG

Nickels et al, 2008

Journal of clinical neurophysiology, 2003

EEG

Normal

Nickels et al, 2008

MAGNETOENCEPHALOGRAPHY

Paetau et al, Nickels et al, 2008

Sobel et al, Nickels et al, 2008

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