Neuroradiology nuggets: Fulminant cerebral edema in SLE associated autoimmune encephalopathy

Shriram Varadharajan1, Meena Nedunchelian2, Kavitha3, Madhu Bashini4

1Senior Consultant, Neuroradiology, Kauvery Hospital, Radial Road, Chennai

2Junior Consultant, Radiology, Kauvery Hospital, Radial Road, Chennai

3Consultant, Rheumatology, Kauvery Hospital, Radial Road, Chennai

4Senior Consultant, Internal Medicine, Kauvery Hospital, Radial Road, Chennai

Clinical profile

Adult female with SLE presented with myositis and neuropsychiatric manifestations, had worsening with deranged biochemistry (CPK and LDH), developed vocal cord palsy and dysphagia with altered sensorium.

Images

Fig (1): Initial CT brain scan shows confluent white matter hypodensities

Fig (2): MRI coronal and axial T2 images show confluent WM signal changes

Fig (3): Coronal inversion recovery images at level of pelvis showing diffuse intramuscular changes, suggestive of myositis

Fig (4): Interval MRI after progressive worsening of symptoms T2 Sag and axial images after 2 weeks show confluent WM signal changes with diffuse sulcal effacement, transtentorial herniation and brainstem compression.

Fig (5): DWI images show areas of restricted diffusivity in basal ganglia and supratentorial WM

Clinical outcome

Patient had worsening of encephalopathy and raised intracranial pressure in spite of aggressive medical management and underwent bilateral surgical decompression as salvage / rescue attempt, however she eventually succumbed to the fulminal cerebral oedema.

Teaching points

Diffuse cerebral oedema can occur in setting of autoimmune conditions such as SLE and patients can present with non-specific neuropsychiatric manifestations.

CT can help detect white matter changes and diffuse cerebral oedema causing sulcal effacement, however findings are subtle in the early phase while MRI changes are more prominent and often more specific.

Rapid worsening of clinical status can occur in such settings of autoimmune leukoencephalopathy and high index of suspicion is needed to escalate treatment strategies early.

References

  • Zouari R, Saeid MZ, Marzouk M, Rachdi A, Ben Sassi S. Diffuse cerebral edema with leukoencephalopathy revealing systemic lupus erythematosus: A case report and review of literature. Lupus. 2023;32(13):1561-1571.
  • Theisen A, Bose P, Knight C, Oliver M. Leukoencephalopathy and cerebral edema as the presenting manifestations of SLE in an ANA-negative adolescent female: a case report and review of literature. Pediatr Rheumatol Online J. 2020 Jul 13;18(1):58.

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