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.
History
Frank R Kleffner [1925-2015William Landau & Frank Kleffner in 1957 [Landau W & Kleffner F, 1957] described six patients who were diagnosed with “acquired largely receptive aphasia, sometimes recurrent with some association with a convulsive disorder.” These attacks began as partial, myoclonic, or absence seizures, which they called “petit mal,” but were not closely correlated with the aphasic symptoms.
In cases with an epileptiform pattern, “a severe paroxysmal EEG abnormality” was seen, usually diffuse. Any EEG improvement paralleled the speech improvement, and the general prognosis was good with appropriate treatment.
William M Landau 1924-2017EEGs 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 [Robinson RO et al., 2001].
Treatment
- Steroids have been effective, and some AEDs, like valproate, were helpful in reducing seizures, but less often improved speech [Paquier PF et al., 1992; Wang S et al., 2006].
- Multiple subpial transection (MST) has been found to be useful in LKS [Irwin K et al., 2001; Grote CL et al., 1999]
- 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 [Downes M et al., 2015]
References
. Language outcome following multiple subpial transection for Landau-Kleffner syndrome. Brain. 1999 Mar;122 ( Pt 3):561-6. doi: 10.1093/brain/122.3.561.
Irwin K, Birch V, Lees J, Polkey C, Alarcon G, Binnie C, et al. Multiple subpial transection in Landau-Kleffner syndrome. Dev Med Child Neurol. 2001 Apr;43(4):248-52. doi: 10.1017/s0012162201000470.
LANDAU WM, KLEFFNER FR (1957) Syndrome of acquired aphasia with convulsive disorder in children. Neurology 7 (8):523-30. DOI: 10.1212/wnl.7.8.523 PMID: 13451887.
Paquier PF, Van Dongen HR, Loonen CB (1992) The Landau-Kleffner syndrome or 'acquired aphasia with convulsive disorder'. Long-term follow-up of six children and a review of the recent literature. Arch Neurol 49 (4):354-9. DOI: 10.1001/archneur.1992.00530280034019 PMID: 1558514.
Robinson RO, Baird G, Robinson G, Simonoff E (2001) Landau-Kleffner syndrome: course and correlates with outcome. Dev Med Child Neurol 43 (4):243-7. DOI: 10.1017/s0012162201000469 PMID: 11305401.
Wang S, Zhang YH, Bao XH, Wu Y, Jiang YW, Liu XY | display-authors=etal (2006) [Clinical characteristics and long-term prognosis of Landau-Kleffner syndrome]. Zhonghua Er Ke Za Zhi 44 (2):105-9. PMID: 16624025.