Name : Maryam
Family : Noori
Gender : Female
Age : 5
A 5-year-old female presented with progressive psychomotor regression, seizure, ophthalmoplegia, swallowing and respiratory difficulties.
Imaging Procedures and Findings
A 5-year-old female presented with progressive psychomotor regression, seizure, ophthalmoplegia, swallowing and respiratory difficulties. Performed MRI revealed abnormal signal intensity (T2 & FLAIR) in both thalami (especially dorsomedial nucli), dorsal part of pons, periaquiductal gray-matter, cerebellum & centrum semiovale.
Profound perinatal asphyxia shows hypersignal intensity in dorsolateral putamina, lateral thalami, dorsal BS& peri-Rolandic cortex with past history of perinatal asphyxia. In MELAS, stroke like signal abnormality without vascular distribution also noted. Wilson disease shows T2 changes in older children (teens). Leigh syndrome presents in children less than 6 yrs.
Diagnostic Imaging Brain, Osborn et al. Fundamental of diagnostic radiology, William E. Brant, Clyde A. Helms.