P1: OXT/OZN/JDO P2: PSB
0521779407-E-01 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:10
522 Ehlers-Danlos Syndrome Encephalitis
■Life-threatening spontaneous arterial rupture, bowel rupture, or
uterine rupture in type IV
Prognosis
■Normal life expectancy, except type IV (few live past age 40)
Encephalitis........................................
CAROL A. GLASER, MD
history & physical
History
■Definition: inflammation of the brain parenchyma
■Many individuals also with meningeal involvement (“meningoen-
cephalitis”)
■Significant morbidity and mortality
■2 entities:
➣acute/infections – often gray matter
➣postinfections: often white matter involvement, primarily demy-
elinating process
➣overlap in clinical manifestations of infectious vs. postinfectious
Signs & Symptoms
■Altered mental status is hallmark of encephalitis.
■Fever and headache
■Often with lethargy and confusion that may progress to stupor and
coma
■Seizures and focal neurologic findings also seen
➣Bizarre behavior, hallucination, aphasia is characteristic of her-
pes but can be seen with many other agents
Causative Agent
■Most cases presumed viral, though many bacteria and fungi have
been associated
➣Viral agents include but not limited to:
➣Herpesviruses (HSV1, HSV2, VZ*, CMV, EBV*, HHV6)
■Enteroviruses
■Arboviruses (e.g. SLE, WEE, EEE, VEE, West Nile)
■Measles*
■Mumps*
■Respiratory viruses; Influenza A/B*, Parainfluenza*, adenovirus