Subacute Sclerosing Panencephalitis Presenting as Choreoathetosis and Basal Ganglia Hyperintensities

Abstract

Subacute sclerosing panencephalitis is a devastating neurodegenerative disorder characterized by cognitive and memory deterioration, personality changes, behavior abnormalities, and a steady motor decline. Extrapyramidal manifestations are commonly seen in the advanced stage of the disease. We discuss a 10-year-old boy with subacute sclerosing panencephalitis presenting with generalized choreoathetosis, myoclonus, and cognitive decline. He had not been immunized for measles. His elder sister had a viral exanthematous illness 4 years ago. Magnetic resonance imaging revealed bilateral, asymmetric putaminal hyperintensities. A diagnosis of subacute sclerosing panencephalitis was confirmed by periodic complexes in the electroencephalograph and elevated antimeasles antibody titers in the blood and cerebrospinal fluid (1:625). Our case highlights that atypical clinical and radiological features of subacute sclerosing panencephalitis should always be borne in mind in children from endemic areas. Generalized choreoathetosis and bilateral putaminal involvement in the index case add to the spectrum of atypical presentations of subacute sclerosing panencephalitis in children.

Spectrum of Movement Disorders Among Children With Subacute Sclerosing Panencephalitis: A Cross-Sectional Study

“…29 It is also possible that imaging indicated the diagnosis, introducing a selection bias in cases of subacute sclerosing panencephalitis with movement disorders reported so far. Correlation with basal ganglia signal change has also been reported among children with subacute sclerosing panencephalitis with dystonia 11,12,15 and chorea-athetosis 20 (Supplementary Table S1). However, presence of radiologic abnormalities does not seem to correlate with worse prognosis or earlier disease progression in subacute sclerosing panencephalitis.…”

“…Movement disorders other than myoclonus, which are reported among a small proportion (1.6%-5.3%) of patients with subacute sclerosing panencephalitis, have been mainly described among adult patients with advanced disease. [10][11][12][13][14][15][16][17][18][19][20][21][22][23] Moreover, these descriptions chiefly emanate from individual case reports and small case series, which have observed chorea-athetosis, dystonia, parkinsonism, tics, stereotypies, and tremors (Supplementary Table S1). Movement disorders offer an opportunity for symptomatic relief, and an avenue to offer improvement in quality of life among patients with subacute sclerosing panencephalitis, especially considering that therapeutic efforts largely unsuccessful.…”

The spectrum of psychiatric manifestations in subacute sclerosing panencephalitis: A systematic review of published case reports and case series

“…In addition to direct damage to brain tissue, alterations in neurotransmitter and immune system function are possible mechanisms involved in the pathogenesis of depression in SSPE. [45][46][47] In its initial phases, SSPE is often mistakenly identified as malingering. "Malingering" refers to the intentional feigning or exaggeration of symptoms for external gain, such as to avoid school work, or to gain attention or sympathy.…”

“…Damage to the limbic system may lead to dysfunction of the neural circuits involved in mood regulation, leading to depression. In addition to direct damage to brain tissue, alterations in neurotransmitter and immune system function are possible mechanisms involved in the pathogenesis of depression in SSPE 45 47 …”

Diffuse White Matter Involvement in Subacute Sclerosing Panencephalitis

“…3 Rarely, brainstem and basal ganglia involvement may also be involved in SSPE. 4,5 To conclude, in any child with rapidly progressive dementia with white matter abnormalities, prior history of measles should be meticulously probed, and SSPE should be evaluated.…”