| Literature DB >> 31903771 |
Priyanka Madaan1, Prashant Jauhari1, Zulfiqar M Luhar1, Biswaroop Chakrabarty1, Sheffali Gulati1.
Abstract
Autistic epileptiform regression is an uncommon but extensively described malady in children. The clinico-etiological spectrum of this entity ranges from electrical status epilepticus in sleep to various neurogenetic and neurodegenerative disorders. Identification of these disorders is crucial considering their therapeutic and prognostic implications. Simple investigations such as neuroimaging and electroencephalography with activation procedures can provide valuable diagnostic clues in resource-limited settings; facilitating targeted genetic/metabolic testing. Here we report a 3.5-year-old girl with autistic regression and epilepsy. Neuronal ceroid lipofuscinosis was suspected as her electroencephalogram showed photoparoxysmal response on low-frequency (1-3 Hz) intermittent photic stimulation. A deficient leukocyte tripeptidyl peptidase 1 enzyme confirmed the diagnosis of late infantile neuronal ceroid lipofuscinosis.Entities:
Keywords: autistic regression; electroencephalography; epilepsy; neuronal ceroid lipofuscinosis; photosensitivity
Mesh:
Year: 2020 PMID: 31903771 DOI: 10.1177/1550059419899327
Source DB: PubMed Journal: Clin EEG Neurosci ISSN: 1550-0594 Impact factor: 1.843