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Landau-Kleffner syndrome (acquired epileptiform aphasia)
Epidemiology:
- rare
Pathology:
- affects the parts of the brain that control speech & comprehension of language
Clinical manifestations:
1) children develop normally until ages of 3 & 7
2) development of aphasia (comprehension & speech) is gradual
3) seizures may occur
Special laboratory:
- abnormal electroencephalogram (EEG)
Differential diagnosis:
1) diagnosis may be difficult
2) autism
2) hearing impairment
3) learning disability
4) auditory/verbal processing disorder
5) attention deficit disorder
6) mental retardation
7) childhood schizophrenia
8) emotional/behavioral problems
Management:
1) anticonvulsants
2) corticosteroids
3) speech therapy
4) multiple subpial transection
a) controversial surgical technique
b) severing of pathways of abnormal electrical brain activity
Prognosis:
- prognosis is variable
a) some may have a permanent severe language disorder
b) others may regain much of their language abilities (may take months or years)
c) remissions & relapse may occur
d) prognosis is improved when the onset of the disorder is after age 6 & when speech therapy is started early
e) seizures generally disappear by adulthood
General
syndrome
References
- National Institute on Deafness & Other Conditions (NIDCD)
http://www.nidcd.nih.gov/health/voice/landklfs.asp
- NINDS Landau-Kleffner Syndrome Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Landau-Kleffner-Syndrome-Information-Page