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Case Report

Status epilepticus in scleromyxedema

Sofia Markoula , Sofia Zouroudi , Sotirios Giannopoulos , Kimon Tsoukanelis , Ananstasia Zikou ,
2
1
1
1
1
Athanassios P. Kyritsis 1
1 Department of Neurology, University Hospital of Ioannina, 45110 Ioannina, Greece.
2 Department of Radiology, University Hospital of Ioannina, 45110 Ioannina, Greece.
A B S T R AC T
Scleromyxedema is a rare dermatologic disorder, characterized by erythematous or yellowish lichenoid waxy papules. Neurological
manifestations are rare but well-recognized. A 51-year-old woman, diagnosed with scleromyxedema, was admitted to the hospital with
status epilepticus, caused by brain lesions, as disclosed in a brain magnetic resonance imaging (MRI). The patient was treated with
anticonvulsants and corticosteroids and gradually recovered fully. A complete remission of the lesions was shown in a follow-up brain
MRI. In cases with scleromyxedema and the presence of neurological manifestations, we need to pay attention to central nervous system
involvement, especially when combined with brain MRI lesions, and treat the patient appropriately.

Key words: Scleromyxedema; epilepsy; status epilepsticus; dermatologic disorder

INTRODUCTION legs with impaired joint mobility of her fingers, perhaps
as a consequence of the skin thickening associated with
Scleromyxedema is a rare dermatologic disorder, her scleromyxedema. With regard to the neurological
characterized by erythematous or yellowish lichenoid manifestations, aphasia and bilateral pyramidal
waxy papules. Neurological manifestations are rare weakness were found.
but well-recognized in patients with scleromyxedema,
among other extracutaneous complications. [1-4] The patient became rapidly disorientated and developed
generalized tonic-clonic seizures leading to convulsive
We report a patient with scleromyxedema presenting status epilepticus. Intravenous diazepam and phenytoin
with refractory status epilepticus, caused by brain lesions, (according to status epilepticus treatment protocol) was
probably related to the disease. administered and status epilepticus was not resolved and
general anesthesia and intubation in the intensive care
CASE REPORT unit was required to achieve seizures control.

A 51-year-old right handed Caucasian woman was Using magnetic resonance imaging (MRI), low signal
admitted to the hospital with subacute onset of confusion, intensity on T1-weighted images and high signal
slurred speech and expressive aphasia that had developed intensity on T2-weighted and fluid-attenuated
in a period of 3 days. The patient had been diagnosed inversion recovery images of the brain revealed
with scleromyxedema for 15 years and had been under bilateral fronto-parietal and left fronto-occipital
regular follow up and monthly administration of cortical lesions [Figures 1 and 2]. Furthermore,
immunoglobulin. Her general physical examination at “hyperintense vein sign” was observed in right frontal
admission showed changes in the cutaneous presentation area, compatible with slow flow in isolated cortical
of her scleromyxedema. This patient presented with veins [Figure 1]. After intravenous gadolinium
diffuse confluent papulosquamous eruption and administration, no abnormal enhancement was
thickening of the skin on the face, body, arms and the revealed [Figure 2].

Corresponding Author: Dr. Sofia Markoula, Department of Following a lumbar puncture, mildly elevated protein
Neurology, University Hospital of Ioannina, 45110 Ioannina,
Greece. E-mail: smarkoula@grads.uoi.gr This is an open access article distributed under the terms of the Creative
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Cite this article as: Markoula S, Zouroudi S, Giannopoulos S, Tsoukanelis
K, Zikou A, Kytritsis AP. Status epilepticus in scleromyxedema.
Neuroimmunol Neuroinflammation 2016;3:124-6.
DOI: 10.20517/2347-8659.2015.39
Received: 29-09-2015; Accepted: 04-03-2016

124 © 2016 Neuroimmunology and Neuroinflammation | Published by OAE Publishing Inc.