Skip to main content
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1974 Sep;37(9):997–1004.

Neurological complications of progressive facial hemiatrophy

Sheldon Mark Wolf 1,2,3,1, M Anthony Verity 1,2,3
PMCID: PMC494830

Abstract

Progressive left facial hemiatrophy began in a boy at the age of 5 years. Over the next 20 years, he developed wasting of the left shoulder and arm muscles, ptosis of the eyelids, ophthalmoplegia, convergence nystagmus, pupillary dilatation, hemiparesis, seizures, and dysarthria. A pneumoencephalogram showed cerebellar hemiatrophy. Biopsy of the cerebellum revealed loss of and degenerative changes in Purkinje cells. A deltoid muscle biopsy studied by histochemical and enzymatic procedures was normal. There are many neurological and ophthalmological complications of progressive facial hemiatrophy. 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.

Full text

PDF
997

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ASHLEY F. L., REES T. D., BALLANTYNE D. L., Jr, GALLOWAY D., MACHIDA R., GRAZER F., MCCONNELL D. V., EDGINGTON T., KISKADDEN W. AN INJECTION TECHNIQUE FOR THE TREATMENT OF FACIAL HEMIATROPHY. Plast Reconstr Surg. 1965 Jun;35:640–648. doi: 10.1097/00006534-196506000-00009. [DOI] [PubMed] [Google Scholar]
  2. BANKS T. L., SUGAR H. S. OCULAR MANIFESTATIONS OF FACIAL HEMIATROPHY. Bull Sinai Hosp Detroit. 1963;11:83–88. [PubMed] [Google Scholar]
  3. CRIKELAIR G. F., MOSS M. L., KHURI A. Facial hemiatrophy. Plast Reconstr Surg Transplant Bull. 1962 Jan;29:5–13. doi: 10.1097/00006534-196201000-00002. [DOI] [PubMed] [Google Scholar]
  4. Dawson T. A. Facial hemiatrophy (Parry-Romberg syndrome). Br J Dermatol. 1966 Oct;78(10):545–546. [PubMed] [Google Scholar]
  5. HICKMAN J. W., SHEILS W. S. PROGRESSIVE FACIAL HEMIATROPHY. REPORT OF A CASE WITH MARKED HOMOLATERAL INVOLVEMENT. Arch Intern Med. 1964 May;113:716–720. doi: 10.1001/archinte.1964.00280110096019. [DOI] [PubMed] [Google Scholar]
  6. HOFMANN W. W. Cerebellar lesions after parenteral dilantin administration. Neurology. 1958 Mar;8(3):210–214. doi: 10.1212/wnl.8.3.210. [DOI] [PubMed] [Google Scholar]
  7. Johnson R. V., Kennedy W. R. Progressive facial hemiatrophy (Parry-Romberg syndrome). Contralateral extraocular muscle impairment. Am J Ophthalmol. 1969 Apr;67(4):561–564. doi: 10.1016/0002-9394(69)94265-2. [DOI] [PubMed] [Google Scholar]
  8. KNIGHT J. S., MOORE J. R. Hemiatrophy of the face; report of a case. Oral Surg Oral Med Oral Pathol. 1959 May;12(5):585–590. doi: 10.1016/0030-4220(59)90160-4. [DOI] [PubMed] [Google Scholar]
  9. KOKENGE R., KUTT H., MCDOWELL F. NEUROLOGICAL SEQUELAE FOLLOWING DILANTIN OVERDOSE IN A PATIENT AND IN EXPERIMENTAL ANIMALS. Neurology. 1965 Sep;15:823–829. doi: 10.1212/wnl.15.9.823. [DOI] [PubMed] [Google Scholar]
  10. Kumar P., Agrawal B. V., Singh N. P., Mukerji M., Edoliya T. N. Progressive right hemifacial atrophy with contralateral cerebral hemiatrophy. J Assoc Physicians India. 1971 Aug;19(8):595–597. [PubMed] [Google Scholar]
  11. MOSS M. L., CRIKELAIR G. F. Progressive facial hemiatrophy following cervical sympathectomy in the rat. Arch Oral Biol. 1960 Jan;1:254–258. doi: 10.1016/0003-9969(60)90052-2. [DOI] [PubMed] [Google Scholar]
  12. MOURA R. A. Progressive facial hemiatrophia. Report of a case showing ocular and neuro-ophthalmologic changes. Am J Ophthalmol. 1963 Mar;55:635–639. [PubMed] [Google Scholar]
  13. Nelhaus G. Acquired unilateral vitiligo and poliosis of the head and subacute encephalitis with partial recovery. Neurology. 1970 Oct;20(10):965–974. doi: 10.1212/wnl.20.10.965. [DOI] [PubMed] [Google Scholar]
  14. SUGAR H. S., BANKS T. L. FUCHS' HETEROCHROMIC CYCLITIS ASSOCIATED WITH FACIAL HEMIATROPHY (SCLERODERMA EN COUP DE SABRE). Am J Ophthalmol. 1964 Apr;57:627–632. [PubMed] [Google Scholar]
  15. Selhorst J. B., Kaufman B., Horwitz S. J. Diphenylhydantoin-induced cerebellar degeneration. Arch Neurol. 1972 Nov;27(5):453–455. doi: 10.1001/archneur.1972.00490170085012. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES