title: Mild Cognitive Impairment as a single sign of brain hemiatrophy in patient with Localized Scleroderma and Parry-Romberg Syndrome.
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.
authors.affiliation: Department of Neurology, University Hospital, Krakow, Poland; Department of Neurology, Jagiellonian University, School of Medicine, Krakow, Poland. Electronic address: eklimiec@gmail.com.
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Klimiec
authors.affiliation: Department of Neurology, University Hospital, Krakow, Poland; Department of Neurology, Jagiellonian University, School of Medicine, Krakow, Poland.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Klimkowicz-Mrowiec
mesh_terms: D000328:Adult; D001284:Atrophy / Q000209:etiology / Q000473:pathology; D001927:Brain Diseases / Q000209:etiology* / Q000473:pathology; D060825:Cognitive Dysfunction / Q000209:etiology*; D005150:Facial Hemiatrophy / Q000150:complications*; D005260:Female; D006801:Humans; D012594:Scleroderma, Localized / Q000150:complications*; D013702:Temporal Lobe / Q000473:pathology; D055815:Young Adult
keywords: Facial hemiatrophy; Localized Scleroderma; Magnetic Resonance Imaging; Mild Cognitive Impairment; Neurocutaneous Syndrome; Parry–Romberg Syndrome