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]
Aktekin B, Oguz Y, Aydin H, Senol U (2005) Cortical silent period in a patient with focal epilepsy and Parry-Romberg syndrome. Epilepsy Behav 6: 270-273. [omicsonline.org]
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 Scar
There were several facial scars. Maxilla and mandible were edentulous with right-sided arch hypotrophy ( Figure 3 ). Her neck was supple with limited range of motion. [omicsonline.org]
Psychiatrical
- Distractibility
Figueroa, Combined maxillary and mandibular distraction osteogenesis in patients with hemifacial microsomia, American Journal of Orthodontics and Dentofacial Orthopedics, 147, 5, (566), (2015). [doi.org]
Severe deformities may require additional procedures, such as pedicled temporal fascia flaps, cartilage grafts, bone grafts, orthognathic surgery, and bone distraction. [medigoo.com]
Meanwhile, persons who have the severe form of the syndrome which may need additional surgical procedures like cartilage grafts, othognathic surgery, bone grafts, bone distraction, and pedicled temporal fascia flaps. [syndromespedia.com]
Myocutaneous or "TRAM" flap) or latissimus dorsi muscle to the face. [19], [20], [21], [22], [23] Severe deformities may require additional procedures, such as pedicled temporal fascia flaps, cartilage grafts, bone grafts, orthognathic surgery, and bone distraction [indjos.com]
Severe deformities may require additional procedures, such as pedicled temporal fascia flaps, cartilage grafts, bone grafts, orthognathic surgery, and bone distraction.[14] The timing of surgical intervention is controversial; some surgeons prefer to [en.wikipedia.org]
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]
For those who report neurological symptoms such as migraine or seizures, MRI scan of the brain is the imaging modality of choice. [medigoo.com]
- Trigeminal Neuralgia
Parry-Romberg syndrome is also associated with neurological abnormalities such as episodes of severe facial pain (trigeminal neuralgia) and seizures. [pharmainfo.net]
Associations The condition is often accompanied by: seizures migraines hemiplegia trigeminal neuralgia linear scleroderma (en coup de saber) Other possible associations include: Rasmussen encephalitis 8 CT/MRI brain may show atrophy on one side of the [radiopaedia.org]
- Headache
On follow up after 6 months, her frequency of headache was significantly decreased. [njms.in]
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]
- Focal Neurological Deficit
[…] that include epilepsy, migraine headache, cranial nerve deficits, hemiplegia, cognitive abnormalities, and fixed focal neurologic deficits. 1,2 Cranial CT and MRI findings in Parry-Romberg syndrome are usually ipsilateral to the facial hemiatrophy, and [appliedradiology.com]
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
It is a suitable technique of choice in the treatment of bilateral progressive facial hemiatrophy. [ncbi.nlm.nih.gov]
Moreover, there is no universally accepted treatment or permanent cure for this condition. [indjos.com]
Parry Romberg Syndrome-A Review of Treatment Options. [biomedpharmajournal.org]
The physicians usual treat Parry Romberg syndrome with the following therapies or treatment: Pharmacological treatment The physicians usually prescribe immunosuppressive drugs such as corticosteroids, azathioprine, methotrexate, and cyclosphosphamide [syndromespedia.com]
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]
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]
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]
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]
Although the etiology of Parry-Romberg syndrome is unknown, several causes have been proposed. It has been postulated that it actually falls within the spectrum of localized, linear scleroderma. [appliedradiology.com]
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]
[…] 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]
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]
Pathophysiology
The cause and pathophysiology are unknown. [indjos.com]
We reviewed the literature on published cases of progressive facial hemiatrophy (Parry-Romberg syndrome) to identify possible pathophysiological mechanisms of the syndrome. [ncbi.nlm.nih.gov]
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
Because the length of the surgery is long, electrolytes and glucose often are needed to be monitored to prevent arrhythmias and hypoglycemia. [omicsonline.org]
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]
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]