CM Clincopathologic Conference (CPC) April 15 th 2016 Neurology Resident: Kiruba Dharaneeswaran Pathologist: Ronald Hamilton

History HPI: CM was a 19 year old young man with past medical history of shaken baby syndrome at age of 2 complicated by partial complex epilepsy and quadriparesis. He had severe developmental delay and mental retardation. He also has history of microcephaly. He was trach and g-tube dependant and had scoliosis with posterior spinal fusion.

Examination Mental Status: unable to follow or track but opens eyes. CN: bilateral mild facial weakness. MOTOR: spasticity in both upper and lower extremities. LE>UE. SENS: intact to light touch REFLEX: brisk

Spastic Quadriparesis Spastic quadriplegia is generally caused by brain damage or disruptions in normal brain development preceding birth. Four types of brain damage that can cause spastic quadriplegia. – damage to the white matter (periventricular leukomalacia), – abnormal brain development (cerebral dysgenesis), – bleeding in the brain (intracranial hemorrhage), – brain damage due to lack of oxygen (hypoxic-ischemic encephalopathy or intrapartum asphyxia)

Diagnosis of Shaken Baby Syndrome Retinal Hemorrhages – bilateral, although asymmetric and unilateral are well recognized. – too numerous to count and extend to the ora serrata. – Two thirds of retinal hemorrhages associated with abusive head trauma occur in multiple layers Subdural Hematoma – Can extend to SAH and intraparencyhmal – increased intracranial pressure( not always) – cerebral edema – Interhemispheric bleeding is an early and specific finding in intracranial bleeds caused by shaking.

Radiology T1WI shows bilateral fluid collections as a result of chronic bilateral subdural hematomas and new subdural hematomas in the right frontal and posterior interhemispheric region.

Expected Neurpathology Shaken baby syndrome. Retinal and optic nerve hemorrhages. (probably caused by shaking of the vitreous body which pulls the retina and tears its delicate vessels.) SBS. Severe brain atrophy secondary to HIE.

PHS Brain Conf

Virtual Microscopy H&E Slide A H&E Slide B H&E Slide C H&E Slide D H&E Slide E H&E Slide F H&E Slide G H&E Slide H H&E Slide I H&E Slide J

References 1.Kempe CH, Silverman FN, Steele BF, et al. The battered-child syndrome. JAMA Jul :17-24.[Medline].[Medline] 2.Gilkes MJ, Mann TP. Fundi of battered babies. Lancet : Caffey J. On the theory and practice of shaking infants. Its potential residual effects of permanent brain damage and mental retardation. Am J Dis Child Aug. 124(2): [Medline].[Medline] 4.Geddes JF, Hackshaw AK, Vowles GH. Neuropathology of inflicted head injury in children. I. Patterns of brain damage. Brain Jul. 124(Pt 7): [Medline].[Medline] 5.Geddes JF, Vowles GH, Hackshaw AK, et al. Neuropathology of inflicted head injury in children. II. Microscopic brain injury in infants. Brain Jul. 124(Pt 7): [Medline].[Medline] 6.HHS. U.S. Department of Health and Human Services: Administration for Children and Families. Child Maltreatment UpToDate 8.Radiology Assistant.