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Year : 2013  |  Volume : 1  |  Issue : 2  |  Page : 199-200

An unusual association of headache

Department of Medicine, Command Hospital, Lucknow, Uttar Pradesh, India

Date of Web Publication13-Dec-2013

Correspondence Address:
K. V. S. Hari Kumar
Department of Medicine, Command Hospital, Lucknow - 226 002, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2321-4848.123055

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How to cite this article:
Kumar KH, Gupta AK. An unusual association of headache. Arch Med Health Sci 2013;1:199-200

How to cite this URL:
Kumar KH, Gupta AK. An unusual association of headache. Arch Med Health Sci [serial online] 2013 [cited 2022 May 18];1:199-200. Available from: https://www.amhsjournal.org/text.asp?2013/1/2/199/123055


A 43-year-old man presented with recurrent headache of two years duration. The patient initially presented to a general practitioner in 2010 and was diagnosed as a case of tension headache. He was prescribed intermittent analgesics and had no significant relief. He denied other neurological complaints and was referred to us for evaluation as migraine. The patient gave a history of unilateral headache involving right side of the head and associated with intermittent episodes of nausea and vomiting. The headaches used to occur at a frequency of 3-4 episodes per month, and each attack used to last for approximately 8 hours and gets relieved with analgesics. He denied seizures, photophobia, motor or sensory complaints, and any other symptoms suggestive of raised intracranial pressure. The patient noticed a slight asymmetry in the face with thickening of skin over the right side for past two years. He denied a similar history in family members.

Examination revealed normal vital parameters and no evidence of vitiligo or other autoimmune disorders. He had mild atrophy of right half of the face involving the cheek with sparing of lips, eyelids, forehead, and scalp [Figure 1]. There is the presence of a big linear scar over the left side of the face (coup de sabre) [Figure 2]. The rest of the systemic examination was normal. He was clinically diagnosed as a case of Parry Romberg syndrome associated with migraine without aura.
Figure 1: Clinical photograph showing hemiatrophy of face

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Figure 2: Clinical photograph showing coup de sabre

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His investigations showed normal hematological and biochemical parameters. Immunological profile including rheumatoid factor and antinuclear antibody were negative. CT scan of the brain was normal. He was managed with Sumatriptan and Flunarazine prophylaxis for migraine. He was advised about the cosmetic surgery for adipose tissue and muscle reconstruction, but the patient denied consent for the same. Parry-Romberg syndrome is a neuro-cutaneous degenerative condition characterized by atrophy of facial tissues. [1] The condition is mostly unilateral (90-95%), and rare cases of bilateral involvement are reported. The disease usually occurs in the second decade and is self-limiting in most of the cases. The disease involves all the tissues of the affected area including skin, fat, muscle, and bone. PRS is shown to have varied systemic associations including neurologic (epilepsy, migraine, aneurysm, imaging abnormalities), ocular (enopthalmos, uveitis, heterochromia), cutaneous (vitiligo, alopecia), oral (decayed tooth, tongue atrophy), skeletal (muscular dystrophy, hypoplastic mandible, and maxilla), and miscellaneous (lipodystrophy, dextrocardia, retinitis etc.) disorders. The neurologic disorders are the commonest with migraine or facial pain seen in 45% of patients, epilepsy in 10% and white matter hyperintensities in 5% of PRS patients. Our case showed involvement of only skin and fat. The etiopathogenesis is unclear, and the theories put forward are autoimmune, viral infections, endocrine disturbances, and alteration in fat metabolism. [2] A sharp demarcated line between normal and abnormal skin called coup de sabre develops.

The diagnosis is clinical and based on characteristic cutaneous and soft tissue findings. The differential diagnoses include partial lipodystrophy, hemifacial microsomia, and post-traumatic atrophy. Parry-Romberg syndrome is considered a form of localized scleroderma by a few authors, and they may represent a spectrum of the same disease. [3] The hemifacial atrophy of the face is below forehead in Parry Romberg syndrome and is located in the fronto-parietal scalp region in cases of scleroderma. It is an auto-limitable condition, and there is no cure. Cosmetic surgical therapy is planned after arrest of disease progression. The treatment is usually based on reposition of adipose tissue. The complications include neurological ailments like epilepsy, migraine, and ophthalmic disorders like Horner's syndrome etc. [4] Our case highlights the rare presentation of Parry-Romberg syndrome with migraine. The above case emphasizes the need for multidisciplinary consultations apart from symptomatic management of neurological complications.

  References Top

1.Stone J. Parry-Romberg syndrome: A global survey of 205 patients using the Internet. Neurology 2003;61:674-6.  Back to cited text no. 1
2.Deshmukh SP, Sharma YK, Dogra BB, Chaudhari NC. A case of Parry-Romberg syndrome with congenital heart disease. Indian J Dermatol Venereol Leprol 2012;98:105-6.  Back to cited text no. 2
3.Orozco-Covarrubias L, Guzman-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R. Scleroderma 'en coup de sabre' and progressive facial hemiatrophy. Is it possible to differentiate them? J Eur Acad Dermatol Venereol 2002;16:361-6.  Back to cited text no. 3
4.Balchandra C, Ravikumar BC. Parry-Romberg syndrome with epilepsy. Indian J Dermatol Venereol Leprol 2000;66:145-6.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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