Movement Disorder Early in the Presentation of Two Children with Subacute Sclerosing Pan-Encephalitis

Hanin Al-Gethami; Mohammad Talal Alrifai; Ahmed Al-Rumayyan; Waleed Al-Tuwaijri; Duaa Ba-Armah

Hanin Al-Gethami Neuro-Science Center, King Fahd Medical City, Riyadh, Saudi Arabia
Mohammad Talal Alrifai Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
Ahmed Al-Rumayyan Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
Waleed Al-Tuwaijri Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
Duaa Ba-Armah Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.

Keywords: Subacute sclerosing panencephalitis, prognosis, myoclonic spasms, quadriplegia movement disorder, central precocious puberty

Abstract

Subacute sclerosing panencephalitis (SSPE) is a progressive degenerative disease caused by measles infection with overall poor prognosis in spite multiple modalities of treatment. The course of the disease is characterized by progressive neurological decline in the form of behavioral and personality changes followed by a stage of characteristic periodic myoclonic spasms followed by a stage of quadriplegia movement disorder, vegetative state and frequently early death. Here we report two cases with atypical presentation of early rather than late movement disorder during illness and the unusual association of central precocious puberty preceding the course of illness in one of the cases.