PARRY ROMBERG SYNDROME THE GOBAL SURVEY OF …



Manuscript Number MS#200202724

|SUPPLEMENTARY DATA for WEB |

Parry-Romberg Syndrome: a global survey of 205 patients using the Internet.

Jon Stone MB ChB MRCP

Email - Jon.Stone@ed.ac.uk

(E) - Figure 1. Geographical Distribution of respondents

[pic]

(E) - Figure 2. Age of Onset

Table 1: Parry-Romberg Syndrome – Clinical Characteristics of 205 respondents – extended data

|Demographics | |

|Sex |165 Female: 40 Male |

|Age (median, range) |32 Years (range 4-64) |

|Country of Origin |USA (132), Australia (19), UK (13) Canada (12), New Zealand (5), South Africa (4),France (2), Ireland |

| |(2), Sweden (2), Argentina, Finland, Germany, Guatemala, Holland, India, Iran, Italy, Japan, Korea, |

| |Singapore, Tunisia, Turkey, Venezuela (1 each). |

|Handedness |90% right handed. |

|Diagnosis | |

|Age of onset (median, range) |10 years (1-50). (Delay to Diagnosis - 4 years (0-41)) |

|Diagnostic label* |45% Parry-Romberg disease/syndrome; 40% Romberg syndrome; 16% (Progressive) Facial Hemiatrophy; 16% |

| |Scleroderma ‘en coup de sabre’ (5% isolated); 6% Linear Scleroderma (1% isolated). |

|Self rated severity |Very Severe (7%), Severe (24%), Moderate (36%), Mild (23%), Very Mild (8%) |

| | |

|Location Affected | |

|Face |100% ( 46% left, 52% right, 2% bilateral) |

|Forehead - involvement |63% |

| – with a line |51% (10% in the corner of the eye, 20% in the middle of the eyebrow, 19% within one centimetre of the |

| |middle, 1% unspecified). |

|Lower Face |75% Cheek, 43% Chin, 55% lips, 50% teeth/gums , 25% tongue |

|Arm, Trunk or Leg |9% Arm( 74% ipsilateral), 14% Trunk ( 81% ipsilateral), |

| |10% Leg ( 75% ipsilateral). 19% any involvement of arm, trunk or leg. |

| | |

|Other Symptoms | |

|Epilepsy |11% (23 patients, nine described partial seizures - 5 left side of body, 4 right)- no correlation with|

| |severity, Scleroderma ‘en coup de sabre’ or distribution of hemiatrophy. |

|Migraine / Facial Pain |52% Migraine (lateralised in 55% - 90% of these pain ipsilateral to hemiatrophy) |

|Facial Pain |46% Facial pain (always ipsilateral to hemiatrophy) - ranging from 'dull ache' to electric shock type |

| |pain. Occurring equally in left and right hemiatrophy |

|Eye / Vision problems |46% (e.g globe retraction, abrasions, uveitis). 17% history of uveitis. |

|'Unusually Cold Hands" |31%. Six patients had a diagnosis of Raynaud's phenomenon.30% had Scleroderma ‘en coup de sabre’ |

|Jaw problems |35% jaw pain or difficulty opening or closing jaw. |

|Depression and Anxiety |46% anxious, 10% depressed (HAD score≥8). |

| | |

|Possible Aetiological Factors |

|Genetic |Six (3%) patients had a relative with facial asymmetry. |

| |None formally diagnosed (orbital asymmetry had been confirmed medically in one). |

|Autoimmune Disorders |Vitiligo (17%), Thyroid problems (10%), Systemic sclerosis (5%), Inflammatory Bowel Disease (5%), |

| |Rheumatoid Arthritis (4%), Ankylosing Spondylitis (2%), Lupus (SLE) (2%), and MS (0%). |

|Factors at onset |12% reported an injury they felt was relevant to their condition. |

|Pregnancy / Childbirth |26% of patients reported acceleration of symptoms (68% of these were women during pregnancy or |

| |childbirth). |

|Lyme Disease |3 patients tested positive for Lyme. None had clinical disease. |

| | |

*More than one diagnostic label allowed

-----------------------

[pic]

................
................

In order to avoid copyright disputes, this page is only a partial summary.

Google Online Preview   Download