The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalities

We describe a case of Parry Romberg syndrome/ <em>en coupe de sabre</em> in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainf...

Full description

Bibliographic Details
Main Authors: Karan Seegobin, Kamille Abdool, Kanterpersad Ramcharan, Haramnauth Dyaanand, Fidel Rampersad
Format: Article
Language:English
Published: MDPI AG 2016-09-01
Series:Neurology International
Subjects:
Online Access:http://www.pagepress.org/journals/index.php/ni/article/view/6661
_version_ 1797934960945922048
author Karan Seegobin
Kamille Abdool
Kanterpersad Ramcharan
Haramnauth Dyaanand
Fidel Rampersad
author_facet Karan Seegobin
Kamille Abdool
Kanterpersad Ramcharan
Haramnauth Dyaanand
Fidel Rampersad
author_sort Karan Seegobin
collection DOAJ
description We describe a case of Parry Romberg syndrome/ <em>en coupe de sabre</em> in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy. Clinical, immunologic and neuroradiological abnormalities are discussed. In some cases, this illness can run a benign and stable course.
first_indexed 2024-04-10T18:08:08Z
format Article
id doaj.art-5a2ec6c9b13045c595513e0bcb5e08bf
institution Directory Open Access Journal
issn 2035-8385
2035-8377
language English
last_indexed 2024-04-10T18:08:08Z
publishDate 2016-09-01
publisher MDPI AG
record_format Article
series Neurology International
spelling doaj.art-5a2ec6c9b13045c595513e0bcb5e08bf2023-02-02T12:24:35ZengMDPI AGNeurology International2035-83852035-83772016-09-018310.4081/ni.2016.66613526The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalitiesKaran Seegobin0Kamille Abdool1Kanterpersad Ramcharan2Haramnauth Dyaanand3Fidel Rampersad4Neurology Unit, San Fernando Teaching HospitalNeurology Unit, San Fernando Teaching Hospital; University of the West IndiesNeurology Unit, San Fernando Teaching Hospital; University of the West IndiesUniversity of the West Indies; Rheumatology Unit, San Fernando Teaching HospitalUniversity of the West Indies; Radiology Department, San Fernando Teaching HospitalWe describe a case of Parry Romberg syndrome/ <em>en coupe de sabre</em> in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy. Clinical, immunologic and neuroradiological abnormalities are discussed. In some cases, this illness can run a benign and stable course.http://www.pagepress.org/journals/index.php/ni/article/view/6661Parry Romberg SyndromeRheumatologyNeuroradiologyEncephalopathyWest Indies
spellingShingle Karan Seegobin
Kamille Abdool
Kanterpersad Ramcharan
Haramnauth Dyaanand
Fidel Rampersad
The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalities
Neurology International
Parry Romberg Syndrome
Rheumatology
Neuroradiology
Encephalopathy
West Indies
title The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalities
title_full The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalities
title_fullStr The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalities
title_full_unstemmed The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalities
title_short The chronic encephalopathy of Parry Romberg Syndrome and en coupe de sabre with a 31-year-history in a West Indian woman: clinical, immunologic and neuroimaging abnormalities
title_sort chronic encephalopathy of parry romberg syndrome and en coupe de sabre with a 31 year history in a west indian woman clinical immunologic and neuroimaging abnormalities
topic Parry Romberg Syndrome
Rheumatology
Neuroradiology
Encephalopathy
West Indies
url http://www.pagepress.org/journals/index.php/ni/article/view/6661
work_keys_str_mv AT karanseegobin thechronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT kamilleabdool thechronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT kanterpersadramcharan thechronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT haramnauthdyaanand thechronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT fidelrampersad thechronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT karanseegobin chronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT kamilleabdool chronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT kanterpersadramcharan chronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT haramnauthdyaanand chronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities
AT fidelrampersad chronicencephalopathyofparryrombergsyndromeandencoupedesabrewitha31yearhistoryinawestindianwomanclinicalimmunologicandneuroimagingabnormalities