Monoclonal Paraproteinemia with subacute encephalopathy, seizures, and scleromyxedema

Abstract

For 3 months, a 37-year-old man developed memory loss and personality changes, followed by fever, seizures, bilateral upper motor neuron signs, and increased CSF protein with skin induration that was compatible with scleromyxedema. An IgG-type paraproteinemia was identified. He improved with plasmapheresis, but died in acute respiratory and renal failure. Autopsy revealed right temporal demyelination and meningeal inflammation.

Scleromyxedema and the dermato‐neuro syndrome: case report and review of the literature

“…In three cases the brain parenchyma was unremarkable, 5,8,30 in one, strong staining of brain astrocytes and endothelial cells for IL-6 was shown in the absence of other brain abnormalities, 28 in two there was acute cerebral edema, 26,30 and in the final case punctate foci of acute rarefaction and demyelination, focal meningeal inflammation and focal loss of Purkinje cells were noted. 27,32 Brain biopsies performed on two patients while they were acutely ill revealed no significant abnormalities in one instance 8 and showed gliosis and mild demyelinization in the other. 25 While some evidence 24,27,32 suggests that demyelination may be a feature of the dermatoneuro syndrome, the exact pathophysiology remains unclear.…”

“…Brain biopsies performed on two patients while they were acutely ill revealed no significant abnormalities in one instance 8 and showed gliosis and mild demyelinization in the other 25 . While some evidence 24,27,32 suggests that demyelination may be a feature of the dermato‐neuro syndrome, the exact pathophysiology remains unclear.…”

“…2,3,6 In patients with dermato-neuro syndrome, although many patients appeared to spontaneously recover, a number who were given supportive care only died of their disease. 5,26,34 To date, plasmapheresis 10,27,32,39 and IVIG 33,37 appear to have resulted in the most dramatic recovery of neurological status. Our patient did not respond to plasmapheresis and steroids, but subsequently had a good response to melphalan and IVIG.…”

“…In addition to our case, review of the literature in the English language identified 18 cases of dermato‐neuro syndrome showing the classic presentation of acute onset seizure and coma, usually accompanied by fever and preceded by a flu‐like illness 5,8,10,14,25–28,30–40 (Table 1). There were seven additional cases in which patients with scleromyxedema had acute onset of severe neurologic symptoms, however the combination of both seizure and coma did not occur 8,9,13,26,29,41,42 (Table 2).…”

Scleromyxedema with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis?

“…Neurological impairment is an infrequent but progressive and sometimes fatal complication, without a widely accepted pathological mechanism. Hyperviscosity due to hypergammaglobulinemia may be responsible by the neurological features seen in some patients 2 . The favorable clinical response to plasmapheresis is probably based on removal of disease‐related plasma contents.…”