Parry Romberg Syndrome

Research

A compilation of research done on Parry Romberg Syndrome.

A doctor's review of 205 Parry Romberg patients via the Internet

Basically, just a list of people that he could find that have the disease and their extremity. No findings or research on cure.

Parry Romberg Syndrome Global Survey

Parry Romberg Syndrome Global Survey

Jon Stone, MB, ChB, MRCP From the Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK.

Affects of Parry Romberg Syndrome on the eyes

Transactions of the American Opthalmological Society

Conclusion: Ocular manifestations of progressive hemifacial atrophy are varied, but can progress from mild visual impairment to blindness.

Progressive hemifacial atrophy. A natural history study.

M T Miller, MD and M A Spencer, MD Department of Ophthalmology and Visual Sciences Eye and Ear Infirmary University of Illinois at Chicago, USA.

Parry Romberg Syndrome thought as NOT hereditary

Conclusion: The occurrence of PFH in one of a monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosis of PFH and accurate follow-up is essential to disclose the occurrence of complications.

Progressive facial hemiatrophy: a complex disorder not only affecting the face. A report in a monozygotic male twin pair

CV Hulzebos, TW de Vries, W Armbrust, PJJ Sauer, WS Kerstjens-Frederikse (2004)

Acta Paediatrica 93 (12) , 1665–1669 doi:10.1111/j.1651-2227.2004.tb00861.x

Parry Romberg Origin

Parry-Romberg syndrome or progressive facial hemiatrophy (PFH) is a rare disease of unknown etiology characterized by atrophy of the skin and subcutaneous tissue on one side of the face. The authors present the case of a 32-year-old woman with PFH who had migraine and an intracranial aneurysm. The findings support the hypothesis that the disease could be related to a neural crest migration disorder, from which both fronto-nasal mass and cranial vessels take origin.

Parry-Romberg syndrome with migraine and intracranial aneurysm.

Pichiecchio A, Uggetti C, Grazia Egitto M, Zappoli F.

2002 Aug 27;59(4):606-8; discussion 481.

Epilepsy and Parry Romberg Syndrome

RESULTS: After surgery, the patient was seizure free (follow-up period of 4 years and 7 months). The resected temporal lobe showed a variety of dramatic microanatomic alterations (small groups of ectopic cells, neuronal loss, gliosis, and activated Microglial cells) in mesial structures, including the entorhinal cortex, subiculum, and dentate gyrus. At the electron-microscopic level, we found that in the dentate gyrus, the number of synapses in the cell-sparse region adjacent to the ectopic mass of neurons was almost twice that found in the molecular and polymorph cell layers, indicating the intrusion of neuritic processes and synapse formation. In addition, the symmetrical axosomatic synapses characteristically found on granule cells, which are likely derived from gamma-aminobutyric acid (GABA)ergic inhibitory basket cells, were not observed.

CONCLUSION: The complete seizure relief after surgery suggests that the pacemaker region(s) of seizure activity were within the resected tissue. However, we do not know which of the multiple neuropathologic findings reported here were the primary cause of seizure activity. Nevertheless, the changes found in the dentate gyrus circuitry appear to be among the most important alterations that would lead to epilepsy.

DeFelipe J, Segura T, Arellano JI, Merchan A, DeFelipe-Oroquieta J, Martin P, Maestu F, Cajal S, Sanchez A, Sola RG (2001)

Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome.

Epilepsia 42:1198-1203

Possible cause of Parry Romberg Syndrome

Conclusion: ...Instead, we favor the hypothesis of hyperactivity of the sympathetic nervous system, specifically, inflammation of the superior cervical ganglion in a young patient, as the cause of progressive facial hemiatrophy.

Richard C. Cory, David A. Clayman, Walter J. Faillace, Shaun W. McKee, and Carlos H. Gama

Clinical and Radiologic Findings in Progressive Facial Hemiatrophy (Parry-Romberg Syndrome)

From the Departments of Radiology (R.C.C., D.A.C., S.W.McK.), Neurosurgery (W.J.F.), and Neurology (C.H.G.), University of Florida Health Science Center/Jacksonville.

AJNR 18:751–757, Apr 1997 0195-6108/97/1804–0751
American Journal of Neuroradiology

Parry Romberg Syndrome and the Teeth

The Internet Journal of Dental Science

Temitope Esan, FMCDS (Nig)
Adeyemi Oluniyi Olusile, MSc (Lond)

From the Department of Restorative Denistry, Obafemi Awolowo University