open access

Vol 50, No 3 (2016)
Case reports
Submitted: 2015-10-05
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Mild Cognitive Impairment as a single sign of brain hemiatrophy in patient with Localized Scleroderma and Parry–Romberg Syndrome

Elzbieta Klimiec12, Aleksandra Klimkowicz-Mrowiec12
DOI: 10.1016/j.pjnns.2016.02.002
·
Neurol Neurochir Pol 2016;50(3):215-218.
Affiliations
  1. Department of Neurology, University Hospital no. 2, Bydgoszcz, Poland
  2. Department of Neurology, Jagiellonian University, School of Medicine, Krakow, Poland

open access

Vol 50, No 3 (2016)
Case reports
Submitted: 2015-10-05

Abstract

Neurologic involvement is well recognized in Systemic Scleroderma and increasingly reported in Localized Scleroderma. MRI brain abnormalities are often associated with symptoms such as seizures or headaches. In some cases they may be clinically silent. We describe a 23 years old female with head, trunk and limbs scleroderma who developed Parry–Romberg Syndrome. Brain MRI showed ipsilateral temporal lobe atrophy without any prominent neurologic symptoms. Neuropsychological examination revealed Mild Cognitive Impairment. During the 7 years of follow up we have noticed progression of face atrophy but no progression of brain atrophy. Cognitive functions have been stable. This case highlight that major MRI brain abnormalities in LS may occur with only subtle clinical manifestation such as Mild Cognitive Impairment.

Abstract

Neurologic involvement is well recognized in Systemic Scleroderma and increasingly reported in Localized Scleroderma. MRI brain abnormalities are often associated with symptoms such as seizures or headaches. In some cases they may be clinically silent. We describe a 23 years old female with head, trunk and limbs scleroderma who developed Parry–Romberg Syndrome. Brain MRI showed ipsilateral temporal lobe atrophy without any prominent neurologic symptoms. Neuropsychological examination revealed Mild Cognitive Impairment. During the 7 years of follow up we have noticed progression of face atrophy but no progression of brain atrophy. Cognitive functions have been stable. This case highlight that major MRI brain abnormalities in LS may occur with only subtle clinical manifestation such as Mild Cognitive Impairment.

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Keywords

Localized Scleroderma, Facial hemiatrophy, Parry–Romberg Syndrome, Neurocutaneous Syndrome, Mild Cognitive Impairment, Magnetic Resonance Imaging

About this article
Title

Mild Cognitive Impairment as a single sign of brain hemiatrophy in patient with Localized Scleroderma and Parry–Romberg Syndrome

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 50, No 3 (2016)

Pages

215-218

Page views

316

Article views/downloads

1132

DOI

10.1016/j.pjnns.2016.02.002

Bibliographic record

Neurol Neurochir Pol 2016;50(3):215-218.

Keywords

Localized Scleroderma
Facial hemiatrophy
Parry–Romberg Syndrome
Neurocutaneous Syndrome
Mild Cognitive Impairment
Magnetic Resonance Imaging

Authors

Elzbieta Klimiec
Aleksandra Klimkowicz-Mrowiec

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