CASE PRESENTATION: A substance abusing G2P1 mother spontaneously delivered at term an appropriate for gestational age @entity1 . Neonatal @entity410 appeared at 21 hours and empiric anticonvulsive and antimicrobial treatment was started. At 25 hours, first vesicles appeared. While routine evaluations remained normal, a head CT revealed multifocal @entity63 , and a later MRI showed multifocal @entity4125 and diffusion abnormalities in the corticospinal tracts. The clinical diagnosis of incontinentia @entity7173 (stage 1) was secured by histopathology. Follow-up at 13 months showed @entity245 . DISCUSSION: We discuss the unusually early bilateral, fronto-occipital @entity63 (CT day 3). On the MR imaging (day 7) extensive symmetric cerebral corticomedullar destruction and diffusion sequences with corticospinal tracts abnormalities are seen, which then evolve (day 26) to extensive symmetric cerebral destruction. We review the literature, genetics, suspected pathophysiology and possible neonatal manifestation. CONCLUSION: Incontinentia @entity7173 is rare and, therefore, diagnosis is frequently delayed. Nevertheless, in the setting of therapy refractory @entity410 , excluded @entity281 , and @entity1910 , a high index of suspicion is needed. This is the first report of simultaneous corticomedullar involvement as early as the third day of life.

Therapy resistant neonatal @entity410 , XXXX , and unusually early neuroradiological changes: incontinentia @entity7173 : a case report, literature review and insight into pathogenesis.

@entity1 :: ('9606', 'Species') :: ['girl'] @entity410 :: ('MESH:D012640', 'Disease') :: ['seizures'] @entity1910 :: ('MESH:D005076', 'Disease') :: ['linear vesicular rash'] @entity63 :: ('MESH:D007511', 'Disease') :: ['ischemic injuries', 'corticomedullar ischemias'] @entity245 :: ('MESH:D002658', 'Disease') :: ['global developmental delay'] @entity4125 :: ('MESH:D011693', 'Disease') :: ['petechiae'] @entity281 :: ('MESH:D007239', 'Disease') :: ['infections'] @entity7173 :: ('MESH:D007184', 'Disease') :: ['pigmenti']
@entity1910 :: (MESH:D005076,Disease) :: ['linear vesicular rash']
