Neurology -- Correspondence for Carreño et al., 68 (16) 1308-1310

Correspondence published:

Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis: Bernard Echenne, Guillaume Sébire (3 August 2007)

Reply from the authors: Mar Carreño, Antonio Donaire, Iratxe Maestro (3 August 2007)

Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis 3 August 2007

Bernard Echenne,
Service de Neuropédiatrie, Université de Sherbrooke
3001 12eme avenue Nord J1H5N4 Sherbrooke, Canada,
Guillaume Sébire
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Re: Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis

bernard.echenne{at}usherbrooke.ca Bernard Echenne, et al.

We read with interest the report by Carreno et al on the similarity of anatomical findings between Rasmussen Encephalitis (RE) and Parry Romberg syndrome (PRS). [1]
The inflammatory lesions in their patients, as in other published cases, are close to the characteristic anatomical lesions of RE. However, the anatomical abnormalities were different in other studies including: thickness and hyalinosis of meningeal vessels with marked neovascularization; meningeal fibrosis with joined gyri; cortical calcifications; and slight gliosis without inflammatory lesions or lymphocytic proliferation. [2]
These findings may suggest another physiopathological mechanism that could be a neural crest cells gene promoter dysfunction leading to vascular dysplasia and meningeal fibrosis and/or inflammation rather than a primary auto-immune condition. [3]
Given these conflicting data, the indication of immunosuppressive treatment suggested by Carreno et al should be carefully considered based on anatomical and immunological investigations.
References
1. Carreno M, Donaire A, Barcelò A, et al. Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis. Neurology 2007;68:1308-1310.
2. Chbicheb M, Gelot A, Rivier F et al. Syndrome de Parry Romberg et épilepsie. Rev Neurol 2005;161:92-97.
3. Creuzet S, Couly G, Le Douarin N. Patterning the neural crest derivatives during development of the vertebrate head: insights from avian studies. J Anat 2005;207:447-459.
Disclosure: The authors report no conflicts of interest.

Reply from the authors 3 August 2007

Mar Carreño,
Hospital Clínic
Barcelona, Spain,
Antonio Donaire, Iratxe Maestro
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Re: Reply from the authors

MCARRENO{at}clinic.ub.es Mar Carreño, et al.

We thank Dr. Echenne and Sébire for their comments. As they point out, neuropathological findings in patients with linear scleroderma/Parry Romberg syndrome with neurological symptoms differ and may include changes suggestive of a dysgenetic rather than a primary inflammatory process. [4]
However, clear inflammatory changes identical to those described in Rasmussen encephalitis have been reported. [5] An immune-mediated response has also been confirmed in other patients by the findings of positive CSF oligoclonal bands and response to immunosuppressive treatment. [6,7]
Considering the difficulty in identifying the pathogenic mechanism in an individual case, immunosuppressive treatment could be considered in those patients with progressive and severe disease and no MRI evidence of obvious dysgenetic features.
References
4. Dupont S, Catala M, Hasboun D, Semah F, Baulac M. Progressive facial hemiatrophy and epilepsy: a common underlying dysgenetic mechanism. Neurology 1997; 48:1013-1018.
5. Shah JR, Juhasz C, Kupsky WJ, et al. Rasmussen encephalitis associated with Parry-Romberg syndrome. Neurology 2003; 61:395-397.
6. Stone J, Franks AJ, Guthrie JA, Johnson MH. Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry 2001; 70:382-385.
7. Goldberg-Stern H, deGrauw T, Passo M, Ball WS, Jr. Parry-Romberg syndrome: follow-up imaging during suppressive therapy. Neuroradiology 1997; 39:873-876.
Disclosure: The authors report no conflicts of interest.