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Rasmussen syndrome
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Rasmussen syndrome

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Contributors: Jennifer Vermilion MD, Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Rasmussen syndrome (or Rasmussen encephalitis) is a rare neurodegenerative disease that leads to brain inflammation and atrophy, usually affecting 1 cerebral hemisphere. It is characterized by intractable epilepsy, progressive hemiplegia, and cognitive decline. The cause of inflammation is unknown, making targeted treatments difficult.

The age of onset ranges from infancy to adulthood, but onset is most commonly during childhood. Prior to the acute phase of disease, there may be a prodromal phase of mild hemiparesis or well-controlled focal epilepsy for up to several years. The acute phase is characterized by rapidly progressive seizures, hemiparesis, hemianopia, aphasia (if the dominant hemisphere is involved), and cognitive decline over the first year. The final stage consists of permanent neurological deficits and relapsing epilepsy. Adolescent- or young adulthood-onset occurs in about 10% of patients.

Adolescent-onset disease has a slower rate of progression and less severe fixed neurological deficits compared to the childhood-onset disease. These patients may present with unilateral movement disorders such as hemiathetosis and hemidystonia. Although early seizures are common in Rasmussen syndrome, cases with delayed seizure onset or even absence of seizures have been reported.

Codes

ICD10CM:
G04.81 – Other encephalitis and encephalomyelitis

SNOMEDCT:
230191005 – Rasmussen syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Sturge-Weber syndrome – look for characteristic facial port-wine stain
  • Dyke-Davidoff-Mason syndrome
  • Hemimegalencephaly
  • Unihemispheric cerebral vasculitis
  • Mitochondrial encephalopathy, lactic acidosis, and stroke (MELAS syndrome)
  • Parry-Romberg syndrome (progressive facial hemiatrophy)
  • Landau-Kleffner syndrome – may present similarly but does not typically have associated hemiparesis or imaging findings of unilateral cerebral atrophy
  • Anti-NMDA receptor encephalitis – may present similarly but does not typically have associated hemiparesis or imaging findings of unilateral cerebral atrophy
  • Viral encephalitis – may present similarly but does not typically have associated hemiparesis or imaging findings of unilateral cerebral atrophy
  • Stroke
  • Cerebral vasculitis

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 10/29/2018
Last Updated: 11/09/2018
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Rasmussen syndrome
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Rasmussen syndrome : Aphasia, Pleocytosis in CSF, Focal neurologic deficit, Hemiparesis, Cognitive decline, Continuous partial seizures
Copyright © 2019 VisualDx®. All rights reserved.