Late progression of neurological symptoms and MRI T2 hyperintensities in Parry-Romberg syndrome

Dibaj, Payam; Herrendorf, Gregor; Bahn, Erik; Obermann, Mark

doi:10.1136/jnnp-2016-313091

Cited by 6 publications

(5 citation statements)

References 4 publications

(7 reference statements)

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“… 4 Brain histological studies in PRS/ECDS have been few and may be non-specific, consistent with changes of vasculitis. 4 , 10 Whilst the periventricular white matter changes in Figure 3B in our case is radiologically similar to leucoariaosis described in other vascular disease, leucoariaosis is not a term used in descriptions of this illness and may be another observation for further examination. Definitive postmortem studies of the brain were not found and this is also an area of further research.…”

Section: Discussionsupporting

confidence: 73%

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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

et al. 2016

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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.

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Section: Discussionsupporting

confidence: 73%

“…Despite the numerous neurological manifestations as summarized by Vix and colleagues in their recent review and late deterioration with a hemiparesis as noted by Dibaj and colleagues, in our patient apart from seizures, the illness has run a benign course. 4 , 10 Her care has been centered around seizure control and psychosocial support.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2016

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“…The trigeminal neuralgia ( 32 , 33 , 70 , 71 ), facial paresthesia, severe headache, and epilepsy are the most common complications of PHA ( 31 – 33 ). Variable neurological pathology was described in PHA patients.…”

Section: Resultsmentioning

confidence: 99%

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

Хамаганова

2018

Front. Med.

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Similar clinical and histhopathological features in progressive hemifacial atrophy and linear scleroderma en coup de sabre are well known. Trauma may predispose to the development of both diseases. The lack of association with anti-Borrelia antibodies was shown in both cases as well. The otolaryngological and endocrine disorders may be associated findings in both diseases. However, there are certain differences in neurological and ophthalmological changes in the diseases.

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“…Magnetic resonance imaging abnormalities observed in ParryÀRomberg syndrome, a neurocutaneous disorder characterized by progressive hemifacial atrophy and other systemic changes including CNS involvement (including epilepsy and headache), incorporate T2/FLAIR hyperintensities, brain atrophy, CMBs, leptomeningeal enhancement and microcalcifications [45].…”

Section: Parryàromberg Syndromementioning

confidence: 99%

Cerebral microbleeds: a magnetic resonance imaging review of common and less common causes

Renard

2018

Euro J of Neurology

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Cerebral microbleeds (CMBs) are small foci of (acute, subacute or chronic) blood products, best seen using magnetic resonance imaging (MRI) techniques sensitive to iron deposits (i.e. gradient-echo T2*-weighted and susceptibility-weighted imaging), frequently encountered in small vessel disease (SVD) (with hypertensive vasculopathy and cerebral amyloid angiopathy as the most frequent conditions) and also in other disorders. In this review, the MRI characteristics of CMBs and the associated MRI abnormalities encountered in common and less common SVD and non-SVD conditions are the main focus. Identification of the origin of CMBs depends on their localization, the presence of other associated MRI abnormalities, and the patient's history and clinical state.

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Late progression of neurological symptoms and MRI T2 hyperintensities in Parry-Romberg syndrome

Cited by 6 publications

References 4 publications

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

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

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

Cerebral microbleeds: a magnetic resonance imaging review of common and less common causes

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