Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre

Abstract Background The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. Methods This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher’s exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities. Results Fourteen patients were studied: 6 males and 8 females; median age 14 years (range 3–20). We observed neuroimaging abnormalities in 2/6 ECDS and 5/8 ECDS/PRS patients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (p < 0.05). Conclusions In summary, we observed several distinct radiographic patterns associated with ECDS/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS/PRS in the future.

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P25 Magnetic resonance imaging findings in children with Parry Romberg syndrome and en coup de sabre

Rheumatology ◽

10.1093/rheumatology/kez414.006 ◽

2019 ◽

Vol 58 (Supplement_4) ◽

Author(s):

Harry Knights ◽

Elizabeth Minas ◽

Faraan Khan ◽

Kshitij Mankad ◽

Lindsay Shaw ◽

...

Keyword(s):

White Matter ◽

Magnetic Resonance ◽

Conflicts Of Interest ◽

Case Series ◽

Cutaneous Manifestations ◽

Street Hospital ◽

Hemifacial Atrophy ◽

Cranial Nerve Dysfunction ◽

Parry Romberg Syndrome ◽

Mr Brain

Poster presentation Tuesday 8 October Background En coup de sabre (ECDS) is a form of linear scleroderma characterised by skin induration followed by unilateral pigmentation dysregulation over the frontoparietal region with associated scarring. Parry Romberg syndrome (PRS) is a sporadic neurocutaneous disease characterised by slow and progressive hemifacial atrophy of the skin, muscles, and bony structures. Both conditions are associated with neurological symptoms such as headache and seizures. A wide variety of magnetic resonance findings associated with these conditions have been described. However, to date, attempts to identify predictors of MRI abnormalities have remained unsuccessful. The aim of this study was to: (i) describe the abnormalities seen on MRI in a large group of children with PRS/ECDS; and (ii) identify demographic, clinical, and laboratory predictors of MRI abnormalities. Methods This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with PRS/ECDS seen from 2001-2018. We identified patients with cutaneous symptoms consistent with the clinical descriptions of PRS/ECDS: lateral forehead scleroderma with/without hemifacial atrophy. Presenting clinical, immunological, and MR brain findings are reported. Results are expressed as medians and ranges or frequencies and percentages. Fisher’s exact test was used to identify clinical associations with MRI abnormalities. Results A total of 17 patients were identified: 7 males and 10 females; median age 13 years (range 3 – 23). Non-cutaneous manifestations included seizures, headaches, stroke, cranial nerve dysfunction, anxiety, hypertension, hypermobility, Raynaud’s, myositis, and gastro-oesophageal reflux. The positive MR findings included ipsilateral enhanced white matter signalling, mineral deposition, lateral ventricle dilation, leptomeningeal enhancement, cavernomas, generalised atrophy, and loss of cortical gyration. The presence of seizures and the PRS phenotype were significantly associated with an abnormal MRI (p < 0.05) and showed trends towards significance with ipsilateral enhanced white matter signalling (p = 0.09). Conclusion The development of seizures and the PRS phenotype predict abnormalities in MR brain imaging in childhood PRS/ECDS. Baseline and regular monitoring MR brain scans should be carried out in all patients with PRS/ECDS to identify abnormalities associated with the development of seizures. Conflicts of Interest The authors declare no conflicts of interest.

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ASPREE-NEURO study protocol: A randomized controlled trial to determine the effect of low-dose aspirin on cerebral microbleeds, white matter hyperintensities, cognition, and stroke in the healthy elderly

International Journal of Stroke ◽

10.1177/1747493016669848 ◽

2016 ◽

Vol 12 (1) ◽

pp. 108-113 ◽

Cited By ~ 18

Author(s):

Stephanie A Ward ◽

Parnesh Raniga ◽

Nicholas J Ferris ◽

Robyn L Woods ◽

Elsdon Storey ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

White Matter ◽

Primary Prevention ◽

Magnetic Resonance ◽

Controlled Trial ◽

Brain Magnetic Resonance Imaging ◽

Cerebral Microbleeds ◽

Resonance Imaging ◽

Healthy Elderly ◽

Cerebral Microbleed

Rationale Cerebral microbleeds seen on brain magnetic resonance imaging are markers of small vessel disease, linked to cognitive dysfunction and increased ischemic and hemorrhagic stroke risk. Observational studies suggest that aspirin use may induce cerebral microbleeds, and associated overt intracranial hemorrhage, but this has not been definitively resolved. Aims ASPREE-NEURO will determine the effect of aspirin on cerebral microbleed development over three years in healthy adults aged 70 years and over, participating in the larger ‘ASPirin in Reducing Events in the Elderly (ASPREE)’ primary prevention study of aspirin. Sample size Five hundred and fifty-nine participants provide 75% power (two-sided p value of 0.05) to determine an average difference of 0.5 cerebral microbleed per person after three years. Methods and design A multi-center, randomized placebo-controlled trial of 100 mg daily aspirin in participants who have brain magnetic resonance imaging at study entry, one and three years after randomization and who undergo cognitive testing at the same time points. Study outcomes The primary outcome is the number of new cerebral microbleeds on magnetic resonance imaging after three years. Secondary outcomes are the number of new cerebral microbleeds after one year, change in volume of white matter hyperintensity, cognitive function, and stroke. Discussion ASPREE-NEURO will resolve whether aspirin affects the presence and number of cerebral microbleeds, their relationship with cognitive performance, and indicate whether consideration of cerebral microbleeds alters the risk-benefit profile of aspirin in primary prevention for older people. Trial registration Australian New Zealand Clinical Trials Registry ACTRN12613001313729.

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Magnetic resonance imaging cinecisternography in patients with white matter lesions

Journal of Stroke and Cerebrovascular Diseases ◽

10.1016/s1052-3057(10)80048-5 ◽

1992 ◽

Vol 2 (4) ◽

pp. 213-215

Author(s):

Yasuo Fukuuchi ◽

Takahiro Amano ◽

Hayao Shiga ◽

Makoto Ichijo ◽

Yoshiaki Itoh ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

White Matter ◽

Magnetic Resonance ◽

White Matter Lesions ◽

Resonance Imaging

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Does imaging of the olfactory tract change the clinical management of patients with olfactory disturbance? A case series of 100 consecutive patients

The Journal of Laryngology & Otology ◽

10.1017/s0022215114001625 ◽

2014 ◽

Vol 128 (9) ◽

pp. 810-813 ◽

Cited By ~ 2

Author(s):

J Powell ◽

M Reda Elbadawey ◽

I Zammit-Maempel

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Clinical Management ◽

Case Series ◽

Olfactory Dysfunction ◽

Case Review ◽

Resonance Imaging ◽

Cross Sectional ◽

Olfactory Tract ◽

Cross Sectional Imaging

AbstractBackground:Isolated olfactory dysfunction is a common complaint; the vast majority of cases are benign and untreatable. A common dilemma is whether to image the olfactory tract of affected patients.Methods:A case review of 100 consecutive patients who underwent magnetic resonance imaging for the primary complaint of olfactory dysfunction was performed. Patients with a diagnosis of chronic rhinosinusitis, with or without nasal polyps, were excluded.Results:Magnetic resonance imaging abnormalities that were considered clinically relevant to the presentation of olfactory dysfunction were found in only seven patients (7 per cent). Of these, only one patient (1 per cent) had an abnormality found that altered their clinical management. A comparison of the findings for children (less than 16 years old,n = 5) with those for adults (equal to or more than 16 years old,n = 95) revealed that 4 per cent of adults scanned had olfactory-related pathology diagnosed, as opposed to 60 per cent of children.Conclusion:Cross-sectional imaging may not be necessary in most patients with olfactory dysfunction. Imaging adds little to the patient history and clinical examination findings.

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Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging

Cardiology in the Young ◽

10.1017/s1047951114000031 ◽

2014 ◽

Vol 25 (2) ◽

pp. 338-347 ◽

Cited By ~ 52

Author(s):

David C. Bellinger ◽

Michael J. Rivkin ◽

David DeMaso ◽

Richard L. Robertson ◽

Christian Stopp ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Brain Imaging ◽

Tetralogy Of Fallot ◽

Resonance Imaging ◽

Neurodevelopmental Outcomes ◽

Visual Spatial ◽

Referent Group ◽

Structural Brain ◽

Structural Brain Imaging

AbstractBackground: Few data are available on the neuropsychological, behavioural, or structural brain imaging outcomes in adolescents who underwent corrective surgery in infancy for tetralogy of Fallot. Methods: In this single-centre cross-sectional study, we enrolled 91 adolescents (13–16 years old) with tetralogy of Fallot and 87 referent subjects. Assessments included tests of academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition, as well as brain magnetic resonance imaging. Results: Genetic abnormalities or syndromes were present in 25% of tetralogy of Fallot patients, who had markedly greater neuropsychological morbidities than did patients without a syndrome. However, even patients without a syndrome performed significantly worse than the referent group or population norms in all of the neuropsychological domains assessed. In multivariable regression in those without a genetic/phenotypic syndrome, the strongest predictors of adverse late neurodevelopmental outcomes included a greater number of complications at the first operation, more total surgical complications across all operations, and occurrence of post-operative seizures. The presence of at least one abnormality on structural magnetic resonance imaging was more frequent in tetralogy of Fallot patients than the referent group (42% versus 8%). Conclusions: Adolescents with tetralogy of Fallot are at increased neurodevelopmental risk and would benefit from ongoing surveillance and educational supports even after childhood.

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White matter hyperintensity on magnetic resonance imaging: clinical and neuroanatomic correlates in the depressed elderly

Journal of Neuropsychiatry ◽

10.1176/jnp.1.2.135 ◽

1989 ◽

Vol 1 (2) ◽

pp. 135-144 ◽

Cited By ~ 127

Keyword(s):

Magnetic Resonance Imaging ◽

White Matter ◽

Magnetic Resonance ◽

White Matter Hyperintensity ◽

Resonance Imaging

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Longitudinal Diffusion Tensor Magnetic Resonance Imaging Study of Radiation-Induced White Matter Damage in a Rat Model

Cancer Research ◽

10.1158/0008-5472.can-08-2661 ◽

2009 ◽

Vol 69 (3) ◽

pp. 1190-1198 ◽

Cited By ~ 83

Author(s):

Silun Wang ◽

Ed X. Wu ◽

Deqiang Qiu ◽

Lucullus H.T. Leung ◽

Ho-Fai Lau ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

White Matter ◽

Magnetic Resonance ◽

Diffusion Tensor ◽

Imaging Study ◽

Magnetic Resonance Imaging Study ◽

Resonance Imaging ◽

Longitudinal Diffusion ◽

White Matter Damage ◽

Radiation Induced

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Volumetric Evaluation of Dead Space in Ischial Pressure Injuries Using Magnetic Resonance Imaging: A Case Series

Advances in Skin & Wound Care ◽

10.1097/01.asw.0000797960.52759.75 ◽

2021 ◽

Vol 34 (12) ◽

pp. 668-673

Author(s):

Dong Gyu Kim ◽

Eun Soo Park ◽

Seung Min Nam ◽

Han Gyu Cha ◽

Chang Yong Choi

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Dead Space ◽

Case Series ◽

Resonance Imaging

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Incidental mastoiditis on magnetic resonance imaging scans: clinical relevance and cost implications

The Journal of Laryngology & Otology ◽

10.1017/s0022215118001949 ◽

2018 ◽

Vol 132 (11) ◽

pp. 1010-1012 ◽

Cited By ~ 1

Author(s):

Y Abbas ◽

H S Yuen ◽

A Trinidade ◽

G Watters

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Incidental Finding ◽

Case Series ◽

Resonance Imaging ◽

Retrospective Case ◽

Reporting Practice ◽

Significant Pathology ◽

Cost Implications ◽

Rule Out

AbstractObjectivesTo determine: (1) the incidence of incidental ‘mastoiditis’ reported on magnetic resonance imaging scans performed in patients with asymmetrical sensorineural hearing loss and/or unilateral tinnitus; (2) how many of those patients have actual otological pathology and/or require treatment; and (3) the financial implications of such a reporting practice.MethodRetrospective case series.ResultsBetween October 2015 and November 2016, 500 patients underwent magnetic resonance imaging of the internal auditory meatus to rule out cerebellopontine angle lesions. There was an incidental finding of increased mastoid signalling in 5.8 per cent (n= 29), of which 20.7 per cent (6 of 29) were reported as bilateral cases. The diagnosis of ‘mastoiditis’ was found in 39.7 per cent (29 of 73). None of these patients had any pathology identified clinically. Other significant pathology was identified in a further 8.8 per cent (n= 44).ConclusionThe diagnosis of mastoiditis is primarily clinical. An incidental finding of high signalling in the mastoid region on magnetic resonance imaging is highly unlikely to represent actual clinical disease. In patients who are scanned for other reasons and who do not complain of otological symptoms, such findings are unlikely to require otolaryngology input.

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