Presentation
The present article reports such a case of an 8-year-old girl who presented with mainly hard tissue changes, without neurological or intraoral soft tissue changes. [doi.org]
Entire Body System
- Epilepsy
The cranial CT and MRI appearances of a 14-year-old girl with Parry-Romberg syndrome and epilepsy are described. The findings are compared with the two published descriptions of MRI and CT in such patients. [ncbi.nlm.nih.gov]
Carica un file multimediale Wikipedia Istanza di malattia Sottoclasse di facial nerve disease, brain inflammatory disease, inflammatory and autoimmune disease with epilepsy, genetic peripheral neuropathy, rare genetic epilepsy Prende il nome da Moritz [commons.wikimedia.org]
Neurology (1997) [ Pubmed ] Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome. [wikigenes.org]
- Surgical Procedure
The best time for the surgical procedure usually depends on the preference of the physician. Some physician would prefer early surgical intervention and others would prefer to wait awhile prior to doing the surgical procedure. [syndromespedia.com]
I have had over fifty operations and surgical procedures for Romberg's… Mary B: Scleroderma En Coup de Sabre I feel like it is tearing my head open. [sclero.org]
Surgical procedures for facial deformity, once major growth completes, are being considered, keeping a close look on disease progression. [doi.org]
procedures, depending on the specific problem. [2] [5] Progressive hemifacial atrophy stops progressing on its own within 2 to 20 years, which makes it hard to decide if a therapy is successful. [rarediseases.info.nih.gov]
- Severe Pain
It is accompanied by abnormalities by the neurological area which includes episodes of severe pain in the facial region and seizures. It can be classified with the syndromes of the neurocutaneous system. [syndromespedia.com]
Roughly 45% of people with Parry–Romberg syndrome also have trigeminal neuralgia (severe pain in the tissues supplied by the ipsilateral trigeminal nerve, including the forehead, eye, cheek, nose, mouth and jaw) and/or migraine (severe headaches that [en.wikipedia.org]
Additional neurological symptoms that may occur include abnormal sensations (e.g., prickling or burning sensations called paresthesia) in the facial area and/or episodes of severe pain in the facial areas supplied by the fifth cranial nerve (trigeminal [rarediseases.org]
Jaw & Teeth
- Hemiatrophy of the Tongue
A patient who had linear scleroderma associated with ipsilateral hemiatrophy of the tongue and subsequent facial hemiatrophy was studied. [ncbi.nlm.nih.gov]
In addition to facial wasting that may include the ipsilateral salivary glands and hemiatrophy of the tongue, unilateral involvement of the ear, larynx, oesophagus, diaphragm, kidney and brain have been reported 11, 14. [ispub.com]
Skin
- Alopecia
There was a patch of alopecia and atropic areas of skin, recognised as scleroderma "en coup de sabre". Ophthalmic examination showed miosis. Horner's syndrome and ocular motility disturbance. [ncbi.nlm.nih.gov]
It may be associated with a localized patch of scleroderma in the frontal region of the scalp, causing a patch of cicatrical alopecia (called a 'coup de sabre' because it resembles the scar of a wound made by a sabre). [whonamedit.com]
[…] side A sunken-in appearance of both the eye, as well as the cheek, on the affected side of the face Changes in skin color (pigmentation), including darkening (hyperpigmentation) or lightening (hypopigmentation) Facial hair may turn white and fall out (alopecia [childrenshospital.org]
PHA may start with alopecia, hair hypopigmentation, and atrophy may be preceded by cutaneous induration and hypo- or hyperpigmentation. [orpha.net]
Face, Head & Neck
- Facial Hemihypertrophy
Keywords: Facial asymmetry, mandibular hemiatrophy, mandibular hemihypertrophy How to cite this article: Indurkar A, Degwekar S, Gondivkar S. Facial hemihypertrophy and facial hemiatrophy: Report of 2 cases. [jiaomr.in]
Neurologic
- Seizure
Intractable complex partial seizures developed in a 3-year-old female with normal intracranial findings on computed tomography. [ncbi.nlm.nih.gov]
Seizures, ophthalmoparesis, and pupillary abnormalities are the most common. The aetiology and pathogenesis of the disease are unknown. The possibility that this is a `slow virus' disease is suggested. [doi.org]
- Trigeminal Neuralgia
Keywords Trigeminal Neuralgia Dizygotic Twin Central Nervous System Lesion Pediatr Ophthalmol Strabismus Excerpta Medica Foundation These keywords were added by machine and not by the authors. [link.springer.com]
We describe 2 patients with PRS and neurological symptoms (one with trigeminal neuralgia associated with deafness, and the second with hemifacial pain associated with migraine without aura) in conjunction with abnormal cerebral MRI including white matter [ncbi.nlm.nih.gov]
- Headache
Sometimes I get headaches, and I believe it is because of this illness… Vivi: Facial Scleroderma (Linear/Morphea or Parry Romberg's) I feel really bad, I want to get better, I want to be pretty, I am hurt when I see my cousins healthy and pretty and I [sclero.org]
The most common complications that appear in association to this health disorder are: trigeminal neuritis, facial paresthesia, severe headache and epilepsy, being this last one the most frequent complication of the Central Nervous System. [ncbi.nlm.nih.gov]
Migraine headache Migraine headaches [ more ] 0002076 Onset Age symptoms begin 0003674 Poliosis Patch of white hair White patch [ more ] 0002290 Short mandibular rami 0003778 Sporadic No previous family history 0003745 Tongue atrophy Wasting of the tongue [rarediseases.info.nih.gov]
- Agitation
The one with the facial hair, agitated breathing, sweating, dilated pupils. Aquele com barba, respiração agitada, a suar, pupilas dilatadas. There's someone else, North Carolina, that has facial hair. Barba grande é inaceitável. [tr-ex.me]
Workup
These guidelines provide an overview of the definition, epidemiology, classification, pathogenesis, laboratory workup, histopathology, clinical scoring systems, as well as imaging and device-based workup of localized scleroderma. [therombergsconnection.com]
In the absence of trauma or infection, a complete workup and evaluation is indicated and perhaps an etiology can be identified. Best wishes. [realself.com]
Treatment
Treatments for Parry Rombergs Syndrome A 26–Year Experience with Microsurgical Reconstruction of Hemifacial Atrophy and Linear Scleroderma. [sclero.org]
It is a suitable technique of choice in the treatment of bilateral progressive facial hemiatrophy. [ncbi.nlm.nih.gov]
Prognosis
The prognosis for vision was good. Fluid silicone injection was the major modality in treatment and the results were generally excellent. [ncbi.nlm.nih.gov]
What is the prognosis for Parry-Romberg syndrome? The prognosis for individuals with Parry-Romberg syndrome varies. In some cases, the atrophy ends before the entire face is affected. [medicinenet.com]
Back to top What is the prognosis? The prognosis for individuals with Parry-Romberg syndrome varies. In some cases, the atrophy ends before the entire face is affected. [faces-cranio.org]
Prognosis The prognosis for persons with Parry Romberg Syndrome varies from one person to another. In some persons, the atrophy will end before the person’s entire face gets affected. [syndromespedia.com]
Etiology
Etiological investigations showed the presence of antinuclear antibodies and a positive Rose-Waaler latex test without an inflammatory syndrome. Corticotherapy was proposed (1 mg/kg/day) with good progression. [ncbi.nlm.nih.gov]
The etiology of this rare syndrome still remains unclear. [medigoo.com]
Etiology The exact etiology of PHA is unknown. [orpha.net]
Epidemiology
These guidelines provide an overview of the definition, epidemiology, classification, pathogenesis, laboratory workup, histopathology, clinical scoring systems, as well as imaging and device-based workup of localized scleroderma. [therombergsconnection.com]
Record : found Abstract : found Article : not found Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. [scienceopen.com]
Summary Epidemiology The prevalence is estimated to be at least 1/700,000 individuals, women being slightly more affected than men. [orpha.net]
Epidemiology of childhood cancer in India. Indian J Cancer 2009;46:264-73. [ PUBMED ] 5. Mishra SK, Ramesh C. Reproduction of custom-made eye prosthesis manoeuvre: A case report. J Dent Oral Hyg 2009;1:59-63. 6. Lubkin V, Sloan S. [j-ips.org]
[…] cartilage grafts, bone grafts, orthognathic surgery, and bone distraction.[14] The timing of surgical intervention is controversial; some surgeons prefer to wait until the disease has run its course[3] while others recommend early intervention.[15] Epidemiology [en.wikipedia.org]
Pathophysiology
The cause and pathophysiology are unknown. Ophthalmic involvement is common, with progressive enophthalmos a frequent finding. [ncbi.nlm.nih.gov]
The cause and pathophysiology are unknown. [indjos.com]
Trauma Heredity Peripheral trigeminal neuritis FACIAL HEMIATROPHY PATHOPHYSIOLOGY: It has been suggested that Facial Hemiatrophy is primarily caused by arrest of development during growing period or toward the end of it. [medifitbiologicals.com]
Prevention
Management of PFH comprises a long term follow-up of somatic disorders, and prevention of psychological problems. Treatment of PFH is symptomatic and consists of plastic surgery after the disease activity has stopped. [ncbi.nlm.nih.gov]
Because the length of the surgery is long, electrolytes and glucose often are needed to be monitored to prevent arrhythmias and hypoglycemia. [omicsonline.org]
The NINDS supports research on neurological disorders such as Parry-Romberg syndrome with the goal of finding ways to prevent, treat, and cure them. [medicinenet.com]
Some Parry-Romberg experts are skeptical that the procedure can actually reverse the course of the disease and prevent damage to the underlying bone and muscle. [cnn.com]