LandauKleffner Syndrome An Enigma

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Landau-Kleffner SyndromeAn Enigma?

• Dr Jayaprakash A Gosalakkal
• Consultant Paediatric Neurologist
• University Hospitals of Leicester

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Major problems

• Acquired speech problems(Aphasia)
• Epilepsy or epileptiform discharges
• Neuropsychological and behavioural problems
• More common in boys 12
• All suffer from speech problems about 75have
  epilepsy

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Signs

• Parents often notice a gradual inability of the
  child to respond to their calls
• This may extend to other sounds like non
  recognition of telephone rings
• They may almost appear autistic
• All types of speech problems have been described
• There may sometimes be jargon speech

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Behavioural Problems

• Cognitive problems are common
• Hyperactivity and attention deficit may be
  present
• Rarely progression to sever disinhibition and
  psychosis have been reported

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Seizures

• Seizures occur in 75of patients
• In one third they may may be single status
  occurring around 5-10 years
• Seizures are often nocturnal
• They respond well to treatment and often remit by
  13-15 years

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Seizure Types

• They may be heterogeneous
• Generalized tonic clonic seizures and focal motor
  seizures have been emphasized
• Many other types like atypical absences,atonic
  seizures with head drop,minor automatism and
  secondary Tonic clonic seizures have been
  described
• Pure tonic seizures are rare and probably
  incompatible with the diagnosis

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EEG Changes

• Although EEG abnormalities are present in this
  syndrome by definition, no consensus exists about
  what constitutes typical abnormalities
• On awake EEG, the background is usually normal
  initially. Focal theta slowing over the area of
  the discharges or even generalized slowing
  (probably secondary to medications) may be seen.
  In the awake state, some epileptiform
  abnormalities may be seen. The discharges are
  either focal or bilateral with temporal or
  parietal predominance.
• Many well-documented cases of AEA developing
  after well-established language have shown
  exclusively or predominantly right-sided
  discharges

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ECSWS(CSWS)and LKS

• Continuous spike and slow waves occur bur are not
  a prerequisite for diagnosis
• Epilepsy with continuous spike and slow waves
  during sleep is an age related,partially
  reversible syndrome
• The main difference with LKS is the aphasia in
  the latter,interictal EEG changes are more
  temporal in LKS and frontal in ECSWS
• The diagnosis of LKS is clinical.LKS cannot exist
  without language problems ECSWS cannot be
  diagnosed without EEG changes

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Treatment of LKS

• 1)Seizures may be infrequent ,age limited and
  often easily controlled with antiepileptic drugs.
• 2)Attempts to control epileptiform
  discharges,linguistic,behavioural and other
  abnormalities with antiepileptic drugs have been
  unsuccessful
• 3)Some children respond well to high dose
  steroids or ACTH

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Preferred drugs

• Sodium Valproate
• Ethosuxamide
• Clonazepam
• Clobazam
• ACTH or Steroids may be treatment of choice

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Other drugs Tried in LKS

• Limited studies on lamotrigine, sultiame,
  felbamate, nicardipine, vigabatrin,
  levetiracetam,
• Studies on VNS and Ketogenic diet are few.
• Some reports suggest carbamazepine and
  phenobarbitone may exacerbate symptoms.
• Use of IVIG has been associated with initial
  dramatic response in some patients

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Surgery in LKS

• In medically intractable cases subpial
  intracortical transections have been used with
  relatively good success
• The idea is to eliminate the capacity of cortical
  tissue to generate seizures while preserving
  normal physiological function.
• This is based on the theory that most neuronal
  connections are vertical while epilepsy spreads
  horizontally

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Results of Surgery

• In One study speech recovery occurred in
  11/14/patients (morrel F et al Epilepsia 1993)
• In a UK study it was concluded Behaviour and
  seizure frequency improved dramatically after
  surgery in all children. Improvement in language
  also occurred in all children, although none
  improved to an age-appropriate level.

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Prognosis

• Studies have confirmed epilepsy associated with
  LKS has a good prognosis
• The intellectual functions of patients with
  Landau-Kleffner syndrome in some studies were
  normal.
• Their everyday life were sometimes disrupted
  because of persisting language disorder.
• In some cases, remission and relapse may occur.
  The prognosis is improved when the onset of the
  disorder is after age 6 and when speech therapy
  is started early

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Research

• Connolly et al reported a higher incidence of
  autoantibodies against the brain in children with
  LKS
• The Role of HSV infections in some cases of LKS
  has been raised.
• The response of some patients to IVIG raises the
  question of autoimmune process

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Support

• Educational and other support to patients and
  family is paramount
• Parents are often bewildered at the changes in a
  otherwise normal child
• Speech therapy and Learning support is important

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Sub Pial Transections
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Treatment of LKS
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Conclusion

• LKS is an unusual age related childhood epileptic
  syndrome.
• The striking abnormality are linguistic and
  neurobehavioral
• There appears to be a spectrum of severity
• More research is required