Regional Cerebral Blood-Flow with 99mTc-ECD Brain Perfusion SPECT in Landau-Kleffner Syndrome: Report of Two Cases

Nemati, Reza; Nabipour, Iraj; Javadi, Hamid; Chabi, Negar; Assadi, Majid

doi:10.1155/2014/617343

Cited by 4 publications

(3 citation statements)

References 12 publications

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“…Using 99mTc-ECD SPECT imaging, asymmetrical temporo-parietal perfusion seems to be a common finding in LKS [184]. Pullens et al, through the fMRI of a recovered adult LKS female, studied audiovisual multi-sensory processing, because LKS individuals are often skilled in reading, but fail in speech perception.…”

Section: Physiopathologymentioning

confidence: 99%

Continuous Spike–Waves during Slow Sleep Today: An Update

Posar,

Visconti

2024

Children

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In the context of childhood epilepsy, the concept of continuous spike–waves during slow sleep (CSWS) includes several childhood-onset heterogeneous conditions that share electroencephalograms (EEGs) characterized by a high frequency of paroxysmal abnormalities during sleep, which have negative effects on the cognitive development and behavior of the child. These negative effects may have the characteristics of a clear regression or of a slowdown in development. Seizures are very often present, but not constantly. The above makes it clear why CSWS have been included in epileptic encephalopathies, in which, by definition, frequent EEG paroxysmal abnormalities have an unfavorable impact on cognitive functions, including socio-communicative skills, causing autistic features, even regardless of the presence of clinically overt seizures. Although several decades have passed since the original descriptions of the electroclinical condition of CSWS, there are still many areas that are little-known and deserve to be further studied, including the EEG diagnostic criteria, the most effective electrophysiological parameter for monitoring the role of the thalamus in CSWS pathogenesis, its long-term evolution, the nosographic location of Landau–Kleffner syndrome, standardized neuropsychological and behavioral assessments, and pharmacological and non-pharmacological therapies.

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Section: Physiopathologymentioning

confidence: 99%

Continuous Spike–Waves during Slow Sleep Today: An Update

Posar,

Visconti

2024

Children

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“…MRI scans are normal in most LKS cases, but PET and SPECT studies have showed focal or multifocal changes or perfusion asymmetries predominantly affecting, but not restricted to the temporal regions [26]. Functional MRI study [27] suggested intact temporal lobe processing but an altered temporal to frontal connectivity.…”

Section: Lks and Etiologymentioning

confidence: 99%

Landau Kleffner Syndrome: An Unusual Case With Progressive Ataxia Prior To Language Regression And Autistic-Like Behaviors

Chen¹,

Yang²,

Ning³

et al. 2019

Neuropsychiatry

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To report a case with Landau-Kleffner syndrome (LKS) presenting with unusual clinical features. Methods The clinical features of the case including clinical manifestations, serial electroencephalography (EEG) outcomes, neuroimaging findings, neuropsychological outcomes, interventional approaches and long-term follow-up outcomes, were analyzed. Results This previously normal girl (3y10m), presented with progressive ataxia as an onset symptom, ensued with cognitive impairments and language regressions (characterized by verbal auditory agnosia and expressive aphasia), and autistic-like behaviors. She had no evidence of clinical seizures, with normal brain and spine MRI scans, intact auditory exams and normal brain auditory evoked potential, although the sleep-activated inter-ictal epileptiform discharges of EEG were revealed. The patient showed gradual resolution of symptoms with long-term high-dose steroids plus intravenous immunoglobulin (IVIG) regimens, in consistence with the gradual improvement of the outcomes of serial EEGs. She recovered and returned to normal kindergarten 1y1m after the onset of the disease, consistent with the normal EEG results. Her full-scale intelligence quotient (IQ) was 71 score, with verbal IQ 66 and performance IQ 81 respectively, tested at the time of 1y3m after the onset. The long-term prognosis of the case was good as she had good academic performance and was a monitor of the class at the time of her long-term follow-up of 5y8m after the onset. Conclusion LKS is heterogeneity entity, especially for those with the atypical onset such as ataxia, as well as absence of clinical seizures. The combination of long-term high-dose steroid plus IVIG regimens may be especially effective in LKS.

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“…The EEG is a basic component in LKS diagnosis, which typically shows abnormal epileptic discharges in children, while clinical seizures are observed in about 70% of the cases. EEG shows spike and wave discharges that are widespread, multifocal or shifting, mostly in the temporal region, sometimes unilateral and are particularly exacerbated Open Access Case during sleep [4].…”

Section: Introductionmentioning

confidence: 99%