Fulminant Subacute Sclerosing Panencephalitis Presenting with Acute Ataxia and Hemiparesis in a 15-Year-Old Boy

Abstract

BackgroundSubacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading.Case ReportWe report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG). Magnetic resonance imaging disclosed multiple white-matter hyperintensities, suggesting a diagnosis of acute disseminated encephalomyelitis. He received intravenous steroids, and within 4 days of hospital admission he developed unilateral slow myoclonic jerks. Repeat EEG revealed Rademecker complexes, pathognomonic of SSPE, and an elevated titer of IgG antimeasles antibodies was detected in his cerebrospinal fluid. The disease progressed rapidly and the patient succumbed within 15 days of hospitalization. The diagnosis of SSPE was confirmed by autopsy.ConclusionsThis case illustrates the difficulty of recognizing fulminant SSPE when it manifests with asymmetric clinical and EEG abnormalities.

Clinical conundrum: status epilepticus culminating into acute dystonia myoclonus

“…SSPE characteristically presents as a chronic condition (over 6–10 years), but it can occur acutely (over 1–6 months) with various atypical presentations, and diagnosis could be an enigma. These include seizures, vision loss, hemiparesis and ataxia dystonia 2 19–22. It can present with status epilepticus also 23.…”

“…It could present with clinical features of blindness, ataxia, focal motor seizures, hemiparesis and acute dystonia. Most of the fulminant cases are clinically atypical, with rare patients showing characteristic clinical and EEG findings of SSPE 2. This case was even more challenging, as after a prolonged episode of status epilepticus, the patient continued to be in altered sensorium and developed dystonia, which initially was thought to be due to hypoxic injury.…”

Encéphalite post-rougeoleuse

“…Certaines bactéries sont aussi impliquées (Campylobacter, Chlamydia, Legionella). Des étiologies parasitaires (Plasmodium, Toxoplasma) sont également connues [4].…”

Advances in Antiviral Therapy for Subacute Sclerosing Panencephalitis

“…The neurological disability index [ 6 ] is used to determine the therapeutic effects of various treatments on SSPE. The progression of SSPE may take different courses, some of which involve a fulminant form of rapid progression accompanied by a severe prognosis [ 7 ]. On the contrary, there are also cases with good prognosis, such as patients who exhibit slow progression over 10 years or more, patients who show repeated chronic recurrence and remission, and a small number of patients that display spontaneous improvement in symptoms and are able to walk again after being bedridden [ 8 ].…”