Once subacute sclerosing panencephalitis (SSPE) sets in, it progresses through various stages, beginning with mood swings, personality shifts, depression, lethargy, and sometimes fever and headaches. This initial phase can last as long as six months. The second stage is marked by jerky movements, spasms, vision loss, dementia, and seizures. In the third stage, those jerky movements evolve into writhing and stiffness. Ultimately, the final stage involves autonomic failure, where heart rate, blood pressure, and breathing become chaotic, leading to coma and, eventually, death. Tragically, around 95 percent of SSPE cases end fatally.
Tragic ending
In the case of the young boy, his parents are unsure about when he contracted measles. When medical professionals examined him, they learned that in the six months leading up to the visit, he had begun experiencing jerky movements, falling, and a steady decline in cognitive abilities. Previously, he had been a healthy baby, meeting all his developmental milestones without issue.
His decline was clearly indicative of SSPE. Imaging revealed lesions in his brain, and tests showed elevated anti-measles antibodies in his cerebrospinal fluid. An electroencephalogram (EEG) confirmed brain waves associated with SSPE. Additionally, he exhibited the jerky movements and cognitive decline typical of this condition.
What was particularly striking were his rolling and swirling eyes. Vision issues are common with SSPE as the condition can harm the retina or optic nerve, with some patients even facing total vision loss. However, in this boy’s situation, he developed rapid, erratic, and multidirectional eye movements known as opsoclonus. While this is often seen in brain cancer patients, it can also result from brain inflammation due to infections. Experts suggest that the movements stem from the loss of certain specialized neurons related to coordinated movement, specifically Purkinje cells and omnipause cells.
The boy’s neurologists believe this might be the first instance of opsoclonus linked to SSPE documented on video. They administered antiviral medication and drugs to manage his seizures, but unfortunately, his condition continued to deteriorate.
