The Romberg's Connection 2009 Survey Results

Comments for Rombergs Symptoms History

Page 12. II. Rombergs Symptoms History
6. Comments for Rombergs Symptoms History:
Symptoms started as "shortening" of my right jaw/chin at age 24- around age 37-38 tissue started to go away and one indentation started on my cheek bone.
I did not know anything was wrong until I lost vision in one eye at age 25. They tested for everything and it wasn't until I noticed atrophy on my forehead (above affected eye) that I got a diagnosis. Since then, it appears that my jawline and nose are also affected. I am not sure if I didn't notice, or if it reactivated in my twenties/thirties.
PRS affected my left arm/hand & left leg/foot.
In the past 10 or 15 years I have had marked atrophy of the chin area where fat grafts were done 30+ years ago. I am uncertain as to whether this is active PRS or simply the loss of the fat grafts over time. I have not observed any new areas of atrophy.
The left side of my face is affected. Whole Left skull bones in CAT scan. Left jaw, lips and chin in soft tissue, (muscle and fat) and connective tissue have atrophied. Nose is turned slightly to the left. Eye socket sits lower then the right. Left ear sits lower then the left ear canal is small and narrow. Occiput hole in back of skull is smaller on left then right. Base of skull is turned to the left on axis. Brown discoloration on left neck, and jaw with some blood vessels that are superficial. Brown discoloration on right side of back with indentions also has blood vessels that are superficial.
Some time in the late 1990's, I had two minor traffic accidents about two or three years apart. My car was rear ended on both of those occasions at very slow speeds (I was stopped both times, and the car(s) behind me hit my car). I had some right neck pain and have wondered if that was what might have triggered my Rombergs.

I have also noticed that I have a lot of itching (off and on) on the right side of my forehead and my right ear. I feel the itching has something to do with the Romberg's as there is no itching on the left side of my face.

I also noticed about 8 or 9 years ago that my right eyelid drooped and eventually, I noticed that my left cheek was fuller than my right cheek and mistakenly thought that I had a sinus infection in my left (good) cheek). My General Practitoner told me that I didn't have a sinus infection, but referred me to an ENT doctor. My ENT doctor ordered a CAT scan and then diagnosed me with Parry-Rombergs syndrome. He stated that he had heard of this syndrome, but had never seen a case of it.
White spot on lower left chin area . Slower eruption of teeth on left side. Smaller teeth, atropthy of tongue.
I had a cousin who also had it, but I didn't get to meet her, I wanted to, but she passed away before I could.
Symptoms began at age 8 with a nickel-sized lesion on the top, lefthand side of the scalp which was itchy and then became bald and indented.

Next at age 11 came another itchy lesion above the left eyebrow. The skin darkened slightly and formed a vertical indentation.

Then came around age 15 a slightly noticeable wasting on the left side, forming a perfect line from the outer edge of left eye down to just above the chin. This wasting was not truly noticeable until about age 20, as I was fully mature and had thinned out from "baby fat." I had not linked all these events until a "Eureeka!" moment at age 20.

Tingling and pain on the affected side. Pain in the jaw, ears, head, pins and needles in hands, numbness arms and legs, near blindness in the affected eye, eye developed a squint, double vision, went smaller then the other eye. Sharp pains in the eye like a needle sticking in to it. Mouth affected, deep hole in the upper pallet. So many things over the years I have forgotton about, until someone on the group mentions them.
Started with a stain on the belly.
Looking at photos, I believe PRS started when I was a teen. However, I didn't notice anything until I had my wisdom teeth pulled at 24. I felt the "hole" in my cheek was related to missing my wisdom teeth on my left side. In my mid 30s I questioned my new dentist about it and he told me that the wisdom teeth had nothing to do with it and I should talk to my doctor about it. That started me down the path of discovery that I had PRS.
It started like a rash under my eye then i had it under my chin and at the corner of my right eye near the hair. during it's activities i had headaches problems eating, it was colored a bruise and very hard until the end of the atrophy.
I am in the beginning of menopause and I suspect that I may have symptoms starting again, but it's so subtle, I'm not sure yet.
First symptom en coupe de sabre, which goes from crown to chin, loss of eye brow and lash hair, later fat loss.
A small white mark appeared on the left side of my temple next to my eye. This grew and over a few years turned medium brown & the skin was shiny. Areas affected (all on the left) are two marks on upper lip, large mark covering most of my left cheek. A long paler mark just in front of my left ear. A large mark on my back. All the areas lost the tissue/fat under the skin. Facial bone development was stunted and my eye sockets are not level with each other. Teeth are extremely misaligned.
In June 2008 son was diagnosed; in Dec 2008 he underwent microsurgery with Dr Siebert at New York University. So in reference to Question 2 & 3 above, it is impossible at this time to determine if symptoms are still active. A second surgery in scheduled for 4/09.
Two good hits to the head (one in front and one in back) approx age of 7. Shortly after had fainting spells and the start of visual problems.
I was born with a brown sign on my left cheek. I can't really remember but the atrophy started at age 5-7?
First symptoms were just discoloration. After 2 years of that, the atrophy started.
Gray hair age 5
Pigment changes, migraines age 7-8
Subtle facial atrophy age 9-11
Progressive wasting of soft tissue and fat, tongue, lips
Age 17 moved jaws surgically to realign
The first manifestation of the disease was that I had grand mal seizures at the age of 9. My facial deformities weren't apparent until a year or so later.
It is hard to say when the symptoms started but looking back at pictures, I believe it was in my mid 30's. That is also the same time that I lost several teeth on my affected side. At the time, we had no idea how my roots could have atrophied so quickly. I had just seen the dentist 6 months before.
No pain symtoms.
Lots of headaches in childhood, with 'sinus' headaches increasing until adulthood. Sensitivity to heat..profuse sweating...sick to stomach. En Coup de Sabre is not noticable, but can be felt through skin, extending to flat spot on crown. Gray streak in hair appeared at 7 y/o. SX's at birth included a bruising on lower back, just above hips--noticed by paternal gm when changing diaper. "Wasting of face" became prominent in early 20's with burn continuing through 30's.
The first thing that my barber noticed was the indent in my skull. He told me that he saw other patrons with similar indents and it was caused by using forceps during delivery. About a month or 2 later, I started getting headaches and went to see a neurologist. Without doing any tests, he told my parents that I had the classic "coupe de sabre" and was sure it was Rhomberg's Syndrome. He was the doctor who performed the biopsies.
Unsure if the symptoms are getting worse or not.
19 - 22yrs gradual onset of 7th cranial nerve palsy on right side of face
25-26 yrs gradual onset of moderate left leg weakness and loss of balance, occasional vertigo. TWO slightly pigmented, indented lines running down forehead appeared
27 yrs mild weakness in left arm
28 yrs ONE mildly pigmented, indented line appeared runing down nose from eyebrow level to tip of nose.
29 yrs gradual loss of pain sensation in left leg
She has markings which look like bruises on her forehead, left eye, dark rings around both eyes, down her nose, and under left side of nose, on upper and lower lip on left side, and brown markings from her left side of face all the way in one line to her chin along her jaw.
Left side of face, especially forehead and orbit. Significant progression following each pregnancy, primarily causing the eye to not close properly when sleeping, leading to corneal dryness. Right ear and right side of neck occurred much later - probably late thirties. The skin is extremely thin on the neck. The right earlobe is gone and skin very thin over the firmer cartilage.
Left side affected most notably the face, eye. Also left breast is much larger than the right, indentation on my back between shoulder blades that is very sensitive. Also having episodes of what feels like electricity going through my left temple. Doesn't happen constantly, however I would have maybe one episode every few months and now it seems to happen every couple of weeks.
Started on the lip in middle of face first specialist thought it was frostbite way back then. (it doesnt even snow here). Dentist was the one who picked it up as area appeared to be "growing" referred to an oral specialist who decided to do investigative surgery (lost two front teeth) as it affected gum, bone and lip. By the time of the surgery, it had advanced quickly and affected nose. Following surgery there was no advancement. Suffered severe migraines but that was put down as related to something else. One side of face slightly lower than other but all else ok.
Hair loss, then indention(s), largest one is down her forehead to between her eyes mostly to the left.
Parry-Romberg has effected the skin, tissue, muscle and bone on the left side of my face. Also effected has been been my eye muscles, eye lashes, cornea, and sclera. I had a spontaneous filtering bleb in my sclera which required a graft to cover.
Symptoms seemed "dormant" or very slow in progression from age 10 -16. Then ages 16-18 visible progression in short amounts of time.
En coup de sabre at age 8 progressing through to age 21.
Chin and jaw midteens through age 24
Right cheek age 20
Symptoms appeared on forehead approximately 2 months after trauma to mid forehead.
My right ear is smaller than my left ear.
Had an impact injury 2 cm round at the top of my forehead. Rombergs started from there. Just before the incident my four children all had chicken pox and I had been suffereing from a virus which was similar to glandular fever or Ross River fever. It took months to get over the virus and in those first six months the progression was rapid but has slowed over the past ten years. Doctor thinks my body was fighting the virus and then got confused by the injury which trigger my immune system to attack the injury site.
Symptoms started with white patchs around jaw line which then progressed with loss of soft tissue, a dent at the top of the head on right side and a small indent on forehead, hairline.
Seizures, starting at the age of two and a half and en coup de sabre at the same time. Gradually the en coup de sabre became an atrohy of the left side of the face. Till today he suffers from epileptic attacks. Only in the last year he suffers from over 200 seizures.
Question 2 "Are the symptoms stll active" should have maybe option. I think the symptoms are still active but have slowed down.
Before age 5: White spot on neck, gray hair, missing eyelashes, asymmetrical nose
Additional facial changes apparent at about age 9. Disfigurement quickly became apparent from age 9-17.

Surgical intervention began age 16 with good results.
The whole left side of my body is noticably smaller than the right. My brain is the same size on both sides, however the electrical signals from the RIGHT side of my brain are lower (tested by "Transcranial Magnetic Stimulation"). It has not been determined if this is linked to my PRS or not, as this result is apparently somewhat unusual.
I was born with left side facial paralysis (just could not independently move the left side of my face) or it may have happened in early childhood - my first memory is about 6 when I could not wink with my left eye - this was diagnosed at age 54/55 as possible mild cerebal palsy due to premature birth, but having later been diagnosed with PR, I wonder if this was the start of it but on the other hand it could be two different conditions.
The right side of my face is definitely affected. My plastic surgeon also sees signs on the left side of my face. The right side of my tongue is also affected.
Gradual hemi-facial atrophy was noticed first; small spots of vitiligo on the head (which has made the nearby hair lose pigment); migraines assumed linked to Rombergs; more recently a persistent case of episcleritis (inflammation of one part of the eye), assumed because of the fact that my eyelid on the affected side of my face does not always close properly when sleeping, causing the eye to become dry and susceptible to irritations.
It first presented on the face as a violaceous rash. In all the places where the redness or rash was present are now the areas of atrophy.
Have forehead line, line under chin and another brown area on other side of chin. Right side has had nerve (trigeminal) sensations on and off--that's what originally made me sit up and take notice (that and a photograph)

Left thigh has an indentation in it.

Changes evolved as follows: pigment blotches, face pain, forehead line and small indent, left thigh pain and indent, line on chin (right side), line under chin indents a bit, now brown spot on left side under chin. Cheek changes as well, but doc says does not know if that's age or condition. Have had body-wide fasciculations for past couple of years, don't know if that's attributable to medication or what?
Had lyme in 2004.
Suffered jaw spasms and migraine - not for last 10 years or so though.
My symptoms first started out as being a darkening of skin near the lower corner of the left side of my mouth- people used to always comment that my face was dirty. My nostril openings were always different shaped too, and I've always had a "dent" in the top right side of my head as well as a flat and bald spot on the back of my head as well. Although this disease has started and stopped over the years, I've noticed lately that it has seemed to have ramped up again in my face on the left side as I can feel something odd there as well as pain around my eye. I've also suffered a Grand Mal seizure for the first time in Oct. '08 which has been attributed to 3 lesions on my brain. I believe these are on the left side- opposite from the en coup de sabre- weird! The en coup de sabre has run down into my right eye brow which is pretty much gone now too.
Luckily, none of the rest of my body seems to be affected at this point though!! : )
At age 5, a rash appeared on the left side of his face. (cheek) For the next 2 years, it changed colors from red, to pink, blue, white and then a mocha-brown color. In that time doctors said it was an allergy, another doctor said it was Atopic Dermatitis. A few months after his 7th birthday, we first noticed the atrophy although we didn't know what we were seeing, he just looked kind of "puny". About 6 weeks later the atrophy was obvious and we rushed him to the doctor with 3 years worth of photos and got the diagnosis. He has atrophy on cheek and a little atrophy on his tongue.
Face affected (above right eye, nose little bit) from age 5 until 7, after that stopped and stayed stable. Headaches started at age 4 and still suffering from headaches (some periods often for 4 days a week, some periods without headaches for several weeks).
Hearing loss on the affected side, as well as facial hair growth.
Tightness in the neck and back muscles of the left side.
Affects my face and my foot on the right side. Have lost soft tissue from the base of my foot - sole and heel.
PRS is contained to the right side of my face. Was extremely active after pregnancies. (age 18-21)
We are not sure if it is still active. I had trouble answering question #2 above.
I am not sure that she has problems externally, but has many problems believed related to Rombergs.
At the age of 12, the skin around the right side of my mouth started to droop.
When I was approximately 9 years old, I had facial trauma that left a bruise on my right jawline. The bruise never went away, and the tissue on that spot thinned. At 14, the hemi-facial atrophy happened seemingly overnight. Dr. Murray injected 11 cc. liquid silicone in my cheek and jaw at 21. In my late 20's, I lost tissue around my right eye. Atrophy began in optic nerve of affected eye at 30. Grand Mal seizures in 40's controlled well by medication.
The questions about whether its still active or not..........I'm not sure.
I did not know some of my sytoms until i read your web site and a lot made sense. Loss of eye lashes and moustache, jaw spasms, eye ache, dent in scalp, then over years loss of fat in eye, face, nose and chin.
The only area affected aside from the face is a small section on her back (same side) where soft tissue/muscle seems to be missing.
I feel that the major progress in symptoms happened between 18 and 28. Since then the progression is very very slow but has not stopped altogether.
Started with a dark line and progressed to the left side. Also affected her teeth and gums on that side. Some pain and weakness on left side later down the road. Did not answer #2. Patient is now deceased.
Number responding = 65 / percent responding = 45% of total respondents (143).

Disclaimer: Please be advised that everyone's experiences may be different and appropriate treatments may vary. Any medical information that you find on The Romberg's Connection website must NOT serve as a substitute for a consultation with one's personal physician. Our visitors should discuss any specific questions or concerns they may have about Rombergs with health care professionals who are familiar with the specifics of their special case.
As a support group, The Romberg's Connection is unable to offer medical advice to anyone.

Throughout this survey, when we use the term "Rombergs" or "Romberg" we are referring to: "Parry Romberg", "Parry Rombergs", "Parry Romberg Syndrome" and "PRS".

This is an unscientific survey designed to gather information from those who must deal with Rombergs on a daily basis.