Parry-Romberg syndrome

Description

Parry-Romberg syndrome: rare disorder of progressive facial hemiatrophy. Slowly progressive atrophy of the soft tissues of one side of the face, associated with trigeminal neuralgia and involvement of the eyes and hair. Muscles, cartilage and the underlying bony structures may also be involved.

The prevalence is estimated to be at least 1/700,000 individuals, women being slightly more affected than men.

PHA usually presents during the first 20 years of life, characterized by slowly progressive unilateral atrophy of various tissues (skin, connective tissue, fat, muscle and rarely, underlying bony structures) in the territory of the 5th cranial nerve.

Seizures and headaches are the most common neurologic symptoms. Ophthalmologic anomalies (enophthalmos, globe retraction, ptosis, iris heterochromia, uveitis, retinal vasculitis, glaucoma and eyelid atrophy) may result in visual impairment or blindness


History

1825Caleb Hillier Parry included an account of the condition now known as progressive hemifacial atrophy. This description appeared in Collections from Unpublished Papers in 1825, 3 years after his death, published by his eldest son, the physician, Charles Henry Parry.

Miss F., aged twenty-eight, tall, thin, and with a somewhat florid complexion, thirteen or fourteen years ago, when at school, was rather suddenly seized with some degree of hemiplegia of the left side, accompanied with transient confusion of intellects. The exact state of the original symptoms it is now impossible to ascertain. It is certain, however, that she has at various times been affected with headaches, and that from the period of the attack the left side of the face began to grow more thin than the right, and the eye to become less prominent, and therefore to appearance smaller.,,when she protrudes her tongue, it turns to the left ; but there is no dropping of the eyelids, or corner of the mouth, nor any weakness or numbness of the limbs on the affected side.

Parry 1825: 478-480

1846Romberg gave a complete description of the disorder Trophoneurosen in Klinische Ergebnisse 1846: 75-81

1871 – Albert Eulenburg (1840–1917), a German neurologist, was the first to use the descriptive term ‘Hemiatrophia facialis progressiva‘ [Progressive Hemifacial Atrophy (PHA)]

1983Lewkonia and Lowry definitive review.


Associated Persons


Alternative names

  • Progressive hemifacial atrophy (PHA)
  • Hemiatrophia facialis progressiva
  • Romberg syndrome

References


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Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and informatics. Asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | vocortex |

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