EIF2AK1

Clinical Characteristics

The following description is based on the Mao et al., article published in the American Journal of Human Genetics in 2020.

Growth parameters
The single individual currently identified with a pathogenic EIF2AK1 mutation has age-appropriate growth parameters.

Neurological features
The individual identified with LEMSPAD syndrome had developmental delay that primarily manifested as delayed walking (at 21 months) with an abnormal spastic gait and toe-walking. Additional features include speech delay and dysarthria with poor articulation, lower-extremity hypertonia, progressive spasticity, hyperreflexia, and bradykinesia, anxiety and attention deficit-hyperactivity disorder. The individual reported had normal cognition and attended a mainstream school with accommodations. Her nerve conduction studies were found to be normal.

Brain imaging at age 2 years showed nonspecific T2-weighted hyperintensities at the posterior lateral ventricles and periventricular gliosis. Mid-sagittal T1-weighted image showed appropriate size of corpus callosum and cerebellar vermis. There were no myelination defects found.