Fulminant Demyelinating Diseases

Abstract

Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.

Malignant multiple sclerosis: clinical and demographic prognostic factors

“…The different criteria adopted always include the early disability provoked by frequent attacks and/or progression, and intense inflammatory activity detected by magnetic resonance imaging 6 . The term fulminant MS, although adopted previously, is currently associated with Marburg' s variant in which progression is monophasic with inexorable deterioration and rapid death 7 . Malignant/aggressive forms occur in 4-14% of cases 3,4 ; and this Brazilian cohort, in which 8.5% of patients developed severe disability within five years, confirmed these data.…”

Tumefactive demyelination of the spinal cord: a case report

“…We excluded it because of lack of typical symptoms (fever and whole-body inflammation, headache, seizures or aphasia and confusion). 5 CSF results and radiographic features did not support that diagnosis. The variant, acute haemorrhagic leukoencephalitis (Hurst disease), could also be excluded because of the absence of haemorrhage.…”

“…Our results interfere with a recently published review article that describes a fulminant demyelinating disease as a range of inflammatory demyelinating disorders associated with rapid progression to disability within several days to weeks, culminating in the need for hospital admission and aggressive therapy for an acute attack. 5 Among fulminant demyelinating diseases, acute disseminated myelitis can be one of the possible diagnoses because of pleocytosis in CSF and the tumefactive pattern of lesions. However, acute disseminated myelitis is only rarely located in the spinal cord.…”

A Case of Acute Disseminated Encephalomyelitis in a MIddle-Aged Adult

“…Given both the therapeutic and prognostic implications of considering this a case as the first demyelinating event of MS, it important to make clear that this most likely does not represent MS given the initial subacute presentation of encephalopathy, the presence of multifocal lesions with illdefined margins, and pathology showing widespread inflammation. In contrast, MS often presents with distinct episodes of focal neurological deficits with concordant confluent demyelinating lesions of varying ages appearing ovoid in shape, often affecting the white matter more than gray matter, the latter of which is seen more commonly in ADEM 4 . CSF studies reveal the persistent presence of oligoclonal bands more frequently in MS in comparison to ADEM 5 .…”