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====== Landau-Kleffner syndrome ====== | ====== Landau-Kleffner syndrome ====== | ||
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- | [{{ : | + | Landau-Kleffner syndrome (LKS), or acquired epileptiform aphasia, is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephalograph (EEG) activity. |
- | children. Neurology 1957; | + | ===== History ===== |
+ | [{{ : | ||
+ | William Landau & Frank Kleffner in 1957[(: | ||
+ | children. Neurology 1957; | ||
+ | |||
+ | EEGs in five cases showed “generalized spikes” in three patients, spikes on the right hemisphere in one patient, and, for the fifth patient, only slow waves in the left temporal area that later became generalized. One patient did not have an EEG. The “generalized spikes” described in this original report were not labeled ESES or CSWS, because these two EEG patterns had not yet been described in 1957. Also, this early report did not designate if the EEG abnormalities were found in the sleep or waking record. | ||
+ | ===== Clinical features ===== | ||
+ | * among patients with LKS, 40–50% show ESES, and focal discharges were seen, especially in the frontal and temporal areas, in 70–100% of patients with behavior disorders | ||
+ | * LKS may evolve into ESES/CSWS patterns with the prognosis for LKS better than the prognoses for these two EEG waveforms. | ||
+ | ===== EEG ===== | ||
+ | * regional spikes ar observed especially in the fronto-, centro-, or posterior-temporal areas[(: | ||
+ | |||
+ | ===== Treatment ===== | ||
+ | * steroids have been effective, and some AEDs, like valproate, were helpful in reducing seizures, but less often improved speech[(: | ||
+ | * Multiple subpial transection (MST) has been found to be useful in LKS[(: | ||
+ | * However there is a wide variability in reported outcomes in literature and some authors have argued that there is insufficient evidence to suggest that multiple subpial transection provides additional benefits over and above the mixed recovery often seen in LKS and related regressive epilepsies[(: | ||
+ | ====== References ====== | ||
+ | ~~REFNOTES~~ |