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ARTICLES: S. Appenzeller, M. A. Montenegro, S. San Juan Dertkigil, P. D. Sampaio-Barros, J. F. Marques-Neto, A. M. Samara, F. Andermann, and F. Cendes Neuroimaging findings in scleroderma en coup de sabre Neurology 2004; 62: 1585-1589 [Abstract] [Full text] [PDF]

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Reply to Chang

Fernando Cendes, Simone Appenzeller and Frederick Andermann   (22 June 2004)

Neuroimaging findings in scleroderma en coup de sabre

Gregory Y Chang, Soo-Hyun Park, Young-Chul Youn, and Oh-Sang Kwon   (22 June 2004)

Reply to Chang 22 June 2004
Fernando Cendes, Department of Neurology, University of Campinas - UNICAMP Campinas, SP, Brazil - 13084-970, Simone Appenzeller and Frederick Andermann Send Correspondence to journal: Re: Reply to Chang fcendes{at}unicamp.br Fernando Cendes, et al.	We thank Dr. Chang for his letter describing a patient with bilateral facial atrophy related to hypertrophic pachymeningitis and chronic pulmonary fibrosis, and for referring to our publication. [1] Hypertrophic pachymeningitis is a localized or diffuse disorder due to thickening of the dura mater, with or without associated inflammation. [3] Causes include rheumatoid arthritis, syphilis, Wegener’s granulomatosis, sarcoidosis, tuberculosis, and cancer. Hypertrophic pachymeningitis may also relate to other fibrosclerotic diseases. [4,5] Pulmonary nodules and granulomatous skin infiltration may coexist and other organs may be involved. [4] When no specific cause is found, it is referred to as idiopathic hypertrophic pachymeningitis. [3,4,6] The inflammatory process may cause destruction of subcutaneous tissue and cartilage. [6] Scleroderma en coup de sabre and Parry-Romberg syndrome (PRS) have not been associated with pachymeningitis. In our series, the exclusion of systemic sclerosis and systemic organ involvement was part of the diagnostic criteria as described in the 'Patients and Methods' section. [1] Pulmonary fibrosis or pachymeningitis were not seen in our patients. To our knowledge, this has not been reported in PRS or in scleroderma en coup de sabre. Based on the data presented by Dr. Chang, his patient most likely does not have PRS. She probably has a systemic inflammatory disorder involving meninges, cranial nerves, lungs, and connective tissue. The clinical response to prednisone is consistent with this. It would be interesting to see the results of her immunologic investigation, including antinuclear antibodies, and ophthalmologic evaluation. Perhaps it would be more appropriate to describe this patient as having facial hemiatrophy related to pachymeningitis. References 3. Masson C, Henin D, Hauw JJ, Rey A, Raverdy P, and Masson M. Cranial pachymeningitis of unknown origin: a study of seven cases. Neurology 1993; 43(7): 1329-1334 4. Kupersmith MJ, Martin V, Heller G, Shah A, and Mitnick HJ. Idiopathic hypertrophic pachymeningitis. Neurology 2004; 62(5): 686-694. 5. Levine MR, Kaye L, Mair S, Bates J. Multifocal fibrosclerosis: report of a case of bilateral idiopathic sclerosing pseudotumor and retroperitoneal fibrosis. Arch Ophthalmol 1993; 111: 841–843. 6. de Deus-Silva L, Queiroz LS, Zanardi VA et al. Hypertrophic pachymeningitis: case report. Arq Neuropsiquiatr 2003; 61(1):107-11.
Neuroimaging findings in scleroderma en coup de sabre 22 June 2004
Gregory Y Chang, 121st US Army Gen Hosp HHC 121st US Army Gen Hosp, Box #277, Soo-Hyun Park, Young-Chul Youn, and Oh-Sang Kwon Send Correspondence to journal: Re: Neuroimaging findings in scleroderma en coup de sabre gychang{at}operamail.com Gregory Y Chang, et al.	We read with interest the article by Appenzeller et al [1] in which they presented nine cases of unilateral en coup de sabre - Parry-Romberg syndrome (PRS) with adjacent skull and underlying cortical abnormalities. Despite the contiguous inflammatory nature of these lesions, dural involvement was not noted and systemic manifestations were not mentioned. We recently saw an older woman with bilateral PRS, bifrontal hypertrophic pachymeningitis, and chronic pulmonary fibrosis. A 69-year-old woman with a chronic headache and a recent seizure onset was noted by her family to have a progressive atrophy involving not only the right cheek but also bitemporal areas since a right Bell's palsy seventeen years ago. Examination showed marked loss of subcutaneous fat over the fronto-temporal scalp bilaterally with sparing of fatty tissue around the bridge of the nose resulting in prominent vertical and horizontal frown wrinkles. (Panel A) Additionally, the right cheek was retracted from loss of subcutaneous fat. (arrow, Panel A) CSF examination revealed acellular fluid but with markedly elevated IgG level of 17.53 mg/dL (0.6-3.3). Oral prednisone was started with complete resolution of her chronic headaches. Bilateral PRS may be difficult to recognize. In our case, retraction of the right cheek in presence of bilateral scalp atrophy suggested the diagnosis. Sparing of tissue over the nasal bridge has been previously emphasized as a characteristic feature in unilateral cases. [2] Although skull and intraparenchymal lesions are typical, our case suggests adjacent inflammatory dural involvement may also be prominent. Furthermore, this inflammatory response may extend out of the facial-cranial region. Recognition of this acquired neurocutaneous complex is important, since underlying cerebral lesions are amenable to medical treatment. References 1. Appenzeller S, Montenegro MA, San Juan Dertkigil S et al. Neuroimaging findings in scleroderma en coup de sabre. Neurology 2004;62:1585-1589. 2. Lehman TJ. The Parry Romberg syndrome of progressive facial hemiatrophy and linear scleroderma en coup de sabre. Mistaken diagnosis or overlapping conditions? J Rheumatol 1992;19:844-845. Figure Figure Legend A) Black arrow points to the focal loss of subcutaneous fat with retraction of overlying cheek consistent with right hemifacial atrophy of Parry-Romberg syndrome. Absence of en coup de sabre deformity and bilateral frontalis and temporalis atrophy is prominent. B) Gadolinium- enhanced T1-weighted coronal image of frontal lobe shows thick enhancing dura and falx (arrowheads). C) Chest x-ray shows right interstitial fibrosis unchanged from 2 years prior. Note: The opinions or assertions contained herein are the private views of the author (GYC) and are not to be construded as representing the views of the Department of Defense, or the Department of the Army.