Movement Disorder Early in the Presentation of Two Children with Subacute Sclerosing Pan-Encephalitis
Hanin Al-Gethami *
Neuro-Science Center, King Fahd Medical City, Riyadh, Saudi Arabia.
Mohammad Talal Alrifai
Pediatric Neurology Consultant, Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
Ahmed Al-Rumayyan
Pediatric Neurology Consultant, Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
Waleed Al-Tuwaijri
Pediatric Neurology Consultant, Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
Duaa Ba-Armah
Pediatric Neurology Consultant, Pediatric Neurology Division, Department of Pediatrics, King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
*Author to whom correspondence should be addressed.
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.
Keywords: Subacute sclerosing panencephalitis, prognosis, myoclonic spasms, quadriplegia movement disorder, central precocious puberty