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

Abstract

We describe a case of Parry Romberg syndrome/en coupe de sabre 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.

Headache in progressive facial hemiatrophy (Parry-Romberg syndrome): A paradigmatic case and systematic review of the literature

“…Gastrointestinal symptoms (nausea and/or emesis) are described in 8/52 (15.4%) cases, including our patient. Blurred vision (21,50), diplopia (21,35), scotoma (37), visual auras (45) or intermittent visual loss (50) were noted in some cases. In one case, the individual had a progressive loss of vision with impaired ocular movements due to a thrombosed giant aneurysm of the cavernous carotid artery of the same side (35).…”

Clinical case of Parry–Romberg syndrome

“…Обсуждается взаимосвязь между морфеей (редким заболеванием, характеризующимся отвердением, утолщением кожи с изменением ее цвета и формированием вытянутых бляшек) и СПР, так как симптом «удара саблей» наблюдается при обоих этих состояниях [19][20][21][22]. Симптом может наблюдаться вне структуры СПР, как самостоятельная клиническая находка [23]. Также известны случаи СПР, при которых «удар саблей» отмечается на шее, а не на лице [24].…”