Case Review: Lupus Patient with Acute Disseminating Encephalomyelitis

The diagnosis of neuropsychiatric lupus often proves difficult and requires a multimodal approach due to overlapping characteristics with other factors, such as infections or metabolic abnormalities. Doctors must exclude alternative explanations for the signs and symptoms.

CSF analysis, imaging data and autoimmune workup help. In up to one-third of SLE cases, CSF analysis shows increased level of protein, decreased glucose and/or lymphocytic pleocytosis. These findings, however, are neither sensitive nor specific.^4,5 Roughly 20 different anti­bodies have been detected in neuropsychiatric lupus, and this includes anti-ribosomal P antibodies and anti-N-methyl-D-aspartate receptor.^4,5 Imaging, particularly MRI, proves especially useful, but many patients with SLE have some abnormalities detected on imaging.⁵

ADEM Discussion

ADEM, a fulminant demyelinating disease, is a monophasic disorder of the central nervous system. It’s rarely reported in adults. It’s most common in children because of immature myelin, the relative immaturity of the immune system and the increased frequency of infections seen in this age group.6 The incidence ranges from 0.4–0.8/100,000 per year.^6,7,8,9 In approximately 30% of cases, ADEM progresses to multiple sclerosis (MS).¹⁰ Child mortality rates range from 10–30%, but most patients recover with minimal to no neurological disability.^6,11,9,12

A 2015 study suggests infections trigger an immune response that causes disseminated multifocal inflammation and patchy demyelination.¹³ Neurologic symptoms such as fever and encephalopathy typically occur in ADEM from one to three weeks after the inciting event (which often is misleading because it resembles encephalitis) and progress rapidly.^6,14 Other symptoms include weakness, ataxia, headache, meningismus, paresis, lethargy and seizures leading to coma.

Doctors can diagnose ADEM via clinical characteristics, laboratory findings and imaging features. CSF findings are abnormal in two thirds of cases and include pleocytosis and elevated protein.11 Oligoclonal bands are typically absent. Pathologic findings show prominent perivenous demyelination with infiltrates of lymphocytes and macrophages.¹¹ CT scans are usually abnormal but nonspecific, and may show hypodense areas within cerebral white matter and juxtacortical areas.⁸

MRI remains the most useful tool for diagnosing ADEM. Typical MRI findings include widespread, bilateral, asymmetric increased signal intensity on T2 weighted imaging within the white and gray matter of the brain and spinal cord.^6,8

Differential diagnoses include MS, encephalitis, optic neuritis and clinical isolated syndrome.¹¹ The International Pediatric MS Study Group has proposed diagnostic criteria to discriminate ADEM from MS, which have been validated and used widely.^6,8,14

There is no standard treatment for ADEM, but once the diagnosis is made, supportive care and high-dose IV methylprednisolone (10–30 mg/kg/day, up to 1 g/day) are imperative.¹¹ Aggressive physical and occupational therapy are also essential to successful recovery. Alternative treatments include intravenous immunoglobulin and plasma exchange.