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Year : 2007  |  Volume : 55  |  Issue : 2  |  Page : 160-162

Parry Romberg's disease with intractable partial epilepsy

Department of Neurology, Vivekananda Institute of Medical Sciences and Ramkrishna Mission Seva Pratisthan, Sarat Bose Road, Kolkata, India

Date of Acceptance10-Oct-2006

Correspondence Address:
Amit Haldar
CD-296, Salt Lake, Kolkata - 700 064
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.32791

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 » Abstract 

Parry Romberg's syndrome is an uncommon disorder characterized by atrophy of skin and subcutaneous tissue of one side of face. It has neurologic sequel. The commonest of which is epilepsy. Here, we present a 17-year old girl with features of Parry Romberg's disease with intractable epilepsy. Her seizures have stopped with systemic corticosteroids. This treatment response, together with previous reports is suggestive of an autoimmune basis to this disorder. Thus the epilepsy in some such cases may be steroid responsive.

Keywords: Autoimmune, Parry Romberg′s syndrome, partial epilepsy, steroid responsiveness

How to cite this article:
Haldar A, Mukherjee A. Parry Romberg's disease with intractable partial epilepsy. Neurol India 2007;55:160-2

How to cite this URL:
Haldar A, Mukherjee A. Parry Romberg's disease with intractable partial epilepsy. Neurol India [serial online] 2007 [cited 2020 Jan 29];55:160-2. Available from:

 » Introduction Top

Progressive facial hemiatrophy, otherwise known as  Parry-Romberg syndrome More Details (PRS) is an uncommon disorder characterized by progressive atrophy of skin, soft tissue, muscle, and underlying bone of one side of face. Rarely, ipsilateral or contralateral limbs may be affected. The disease usually starts in the first two decade of life with skin changes resembling localized scleroderma.[1] Various neurological complications including migraine, facial pain, seizure and radiological changes have been described in PRS. Of these, partial seizure appears to be the commonest.[2] In a world wide survey about 11% of Parry Romberg's disease patients had coexistent epilepsy.[3]

The basis for this epilepsy has varied from structural lesions, to chronic encephalitis.[4],[5] We report here a girl with PRS, who developed intractable epilepsy. The management of this girl provides newer insights into the management and pathogenesis of this disorder.

 » Case Report Top

A 17 year old right handed girl presented with partial onset seizure involving left side since last 1 year. The seizures were mainly tonic with occasional clonic components and involved left face, hand and leg. She had a few episodes of generalized tonic clonic seizures. Born at full term out of a non-consanguineous marriage she had normal developmental milestones. At the age of three years the parents noted a linear discoloration of skin in the right supraorbital region. This was diagnosed as localized scleroderma and the girl was prescribed topical steroid. Soon afterwards, she started developing atrophy of right side of face. This gradually progressed to involve right temple, right cheek, and right jaw. The atrophy in linear scleroderma usually does not spread below the forehead. So at the age of 7 years the diagnosis was changed to PRS. No skin biopsy was done as histology cannot always reliably differentiate between the two conditions.[6],[7]

She had her first episode of generalized tonic-clonic seizure at the age of 16 years. EEG and CT scan of brain were normal. Carbamazepine was started but she continued to have episodes of left focal motor seizures involving face and upper limb. The seizure frequency increased progressively and was uncontrolled in spite of adequate dosage of carbamazepine, clobazam, sodium valproate, phenobarbitone, and lamotrigine. At the time of examination, she was having prolonged episodes of tonic and clonic seizures involving left arm, leg, and face, which recurred 6-7 times daily. Physical examination showed atrophy and hyper pigmentation of right side of face. There was no atrophy of limbs. Systemic examination was normal. Neurological examination showed normal intelligence, cognition and speech. There was no evidence of any developmental regression. Cranial nerves were normal. There was left hemiparesis (power 4/5). Deep tendon reflexes were brisk on left side and plantar response was extensor. She had impaired fine movements in left hand. However, she was able to walk without aid. Routine hemogram and biochemical tests were normal. Cerebrospinal fluid study was within normal limits. There were only 5 lymphocytes. No oligoclonal bands were found. EEG, done on two occasions was normal. MRI scan, performed using T1, T2, and FLAIR sequence revealed focal atrophy in right fronto-parietal cortex [Figure - 1]. Considering the intractable nature of her focal seizures, progressive neurological deterioration and focal atrophy of right fronto-parietal lobe, the differentials of Parry Romberg's disease with epilepsy and Rasmussen's encephalitis were considered. Patient was advised to have evaluation for surgical resection. The parents denied consent. Autoimmune markers were negative.

The girl was started on corticosteroids (oral prednisolone at 1 mg/kg/day) after which her seizures stopped within one month. No other anti-epileptic drugs were modified during this period. After 3 months steroids were gradually tapered. Last 6 months, she has had only one episode of convulsion. That too occurred when we tried to stop the steroids completely. She is now on her eighth month of oral prednisolone. Presently she is on 10 mg/day Her anti-epileptics are also being tapered . We could not repeat MRI imaging as the girl has undergone a plastic surgery with a MRI incompatible facial prosthesis.

 » Discussion Top

PRS is a rare disorder of unknown etiology. The similarity between PRS and scleroderma has raised the speculation that PRS may be an autoimmune disease. The presence of autoantibody in serum of patients of PRS and occasional co-existence of this condition with other established autoimmune disorders like SLE and rheumatoid arthritis further supports the autoimmune hypothesis.[8],[9],[10] Resolution or improvement of the lesion after immunosuppressive treatment also supports the autoimmune mechanism. However, despite all these points, there are only a handful of cases of PRS where systemic steroids have been used for neurological management.[11] Ours is in fact one of the first reports to document stabilization of epilepsy after use of steroids in such cases. We had considered the option of using intravenous immunoglobulin. But keeping the prohibitive cost in mind, we kept it as a second choice. Moreover, as the patient responded dramatically to steroids we have not used it till date.

The mechanism by which epilepsy develops in PRS is not clear. Traditionally, the epilepsy has been ascribed to cortical dysgenesis and other such structural abnormalities. Two recent reports have highlighted the co- existence of PRS and Rasmussen syndrome.[12],[13] The clinical description of Rasmussen syndrome consists of intractable seizures, progressive hemiparesis and focal atrophy of brain. Brain biopsy shows feature of chronic encephalitis. Evidence suggests that Rasmussen's encephalitis (RE) is an autoimmune disorder. Chughani et al . recently reported a case of PRS in a boy of 7 years who developed intractable focal seizures. Based on clinical, radiological, PET scan, and brain biopsy findings they concluded that their patient had RE. Our patient had resistant epilepsy, progressive hemiparesis, neurocognitve decline and MRI evidence of focal brain atrophy. All these features closely resemble the clinical features of RE. But in addition she had facial hemiatrophy. Thus, the condition cannot be explained only by RE.

Despite the limitations, our case could be explained by two hypotheses. Firstly, there may be an association of two rare disorders, namely PRS and RE. At present, neither PRS nor RE has a known underlying cause. The possibility that they share a common autoimmune mechanism should be explored. The second possibility is that this girl had Parry Romberg's disease with intractable epilepsy. But the condition responded to steroids. Either way, steroid responsiveness may suggest that chronic autoimmune encephalitis is responsible for the epilepsy in some such cases rather than dysgenesis.[11],[13],[14] The possibility of a coincidental remission cannot be ruled out completely. However, this seems unlikely considering the fact that the seizures recurred immediately on stoppage of steroids. West syndrome, Lennaux Gastaut syndrome, Landau Kleffner's syndrome, and CSWS are also responsive to steroids though the exact basis of action is not known. The epilepsy of PRS may be an addition to this expanding list.

 » References Top

1.Mendonca J, Viana SL, Freitas F, Lima G. Late-onset progressive facial hemiatrophy (Parry-Romberg syndrome). J Postgrad Med 2005;51:135-6.   Back to cited text no. 1    
2.Asher SW, Berg BO. Progressive hemifacial atrophy: Report of three cases, including one observed over 43 years, and computed tomographic findings. Arch Neurol 1982;39:44-6.  Back to cited text no. 2  [PUBMED]  
3.Stone J. Parry-Romberg syndrome: A global survey of 205 patients using the Internet. Neurology 2003;61:674-6.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Dupont S, Catala M, Hasboun D, Semah F, Baulac M. Progressive facial hemiatrophy and epilepsy: A common underlying dysgenetic mechanism. Neurology 1997;48:1013-8.  Back to cited text no. 4  [PUBMED]  
5.Wartenberg R. Progressive facial hemiatrophy. Arch Neurol Psychiatry 1945;54:75-96.  Back to cited text no. 5    
6.Gambichler T, Kreuter A, Hoffmann K, Bechara FG, Altmeyer P, Jansen T. Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications. BMC Dermatol 2001;1:9.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Blaszczyk M, Janninger CK, Jablonska S. Childhood scleroderma and its pecularities. Cutis 1996;58:141-52.  Back to cited text no. 7    
8.Garcia-de-la Torre I, Castello-Sendra J, Esgleyes-Ribot T, Martinez-Bonilla G, Guerrerosantos J, Fritzler MJ. Autoantibodies in Parry Romberg syndrome: A serologic study of14 patients. J Rheumatol 1995;22:73-7.  Back to cited text no. 8    
9.Kleiner-Baumgarten A, Sukenik S, Howitz J. Linear scleroderma, hemiatrophy and systemic lupus erythrematosus. J Rheumatol 1989;16:1141-3.  Back to cited text no. 9    
10.Littman B. Linear scleroderma: A response to neurological injury? Report and literature review. J Rheumatol 1989;16:1135-40.  Back to cited text no. 10    
11.Korkmaz C, Adapinar B, Uysal S. Beneficial effect of immunosuppressive drugs on Parry-Romberg syndrome: A case report and review of the literature. South Med J 2005;98:940-2.  Back to cited text no. 11  [PUBMED]  
12.Straube A, Padovan CS, Seelos K. Parry-Romberg syndrome and Rasmussen syndrome: Only an incidental similarity? Nervenarzt 2001;72:641-6.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Shah R, Juhαsz C, Kupsky WJ, Asano E, Sood S, Fain D, et al . Rasmussen encephalitis associated with Parry-Romberg syndrome. Neurology 2003;61:395-7.  Back to cited text no. 13    
14.Pupillo G, Andermann F, Dubeau F. Linear Scleroderma and intractable epilepsy: Neuropathologic evidence for a chronic inflammatory process. Ann Neurol 1996;39:277-8.  Back to cited text no. 14  [PUBMED]  


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