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| LETTER TO EDITOR |
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| Year : 2009 | Volume
: 57
| Issue : 2 | Page : 225-226 |
Parenchymal brain cysts in Schimmelpenning-Feuerstein-Mims syndrome
Mahesh Kamate1, Arun Dumale1, Virupaxi Hattiholi2
1 Department of Pediatrics, KLE University's J N Medical College, Belgaum, Karnataka State, India
2 Department of Radiology, KLE University's J N Medical College, Belgaum, Karnataka State, India
| Date of Acceptance | 25-Jan-2009 |
Correspondence Address:
Mahesh Kamate
Department of Pediatrics, KLE University's J N Medical College, Belgaum, Karnataka State
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.51310
How to cite this article:
Kamate M, Dumale A, Hattiholi V. Parenchymal brain cysts in Schimmelpenning-Feuerstein-Mims syndrome. Neurol India 2009;57:225-6 |
Sir,
A 10-year-old girl presented with verrucous epidermal nevus over right half of the face and neck [Figure 1], recurrent complex-partial seizures from six years of age, and poor scholastic performance. The skin lesions evolved with age from pink to yellowish-brown color. She also had limbal dermoid and the rest of the examination including skeletal system was normal. Magnetic resonance imaging (MRI) of the brain revealed right-sided hemiatrophy with parenchymal cysts in the deep parietal white matter [Figure 2]. The combination of characteristic verrucous skin lesions, ocular findings and the hemiatrophy on MRI are diagnostic of Schimmelpenning- Feuerstein-Mims syndrome More Details (SFM) syndrome.
SFM syndrome is a rare neurocutaneous disorder with clinical heterogeneity. The disorder consists of a triad of linear sebaceous nevus, seizures and mental retardation. Central nervous system (CNS) involvement occurs in the form of hemimegalencephaly with ipsilateral gyral malformations, seizures, hemiparesis, mental retardation, cranial nerve palsies and hydrocephalus. [1] Neuroimaging findings in SFM syndrome include the primary lesions: Malformations of cortical development like hemimegalencephaly, polymicrogyria; gliomatosis, hemiatrophy with or without parenchymal cysts, vascular malformations and intracranial/intraspinal lipomas and secondary features: Porencephaly, infarcts and atrophy. [2] Other rare findings include enlarged ventricles, cortical hypoplasia, intracerebral calcification, arachnoid cysts, intracranial tumors and skull asymmetry. [3] CNS complications are more likely to be associated with epidermal nevi on the head and the CNS abnormalities are most often ipsilateral to the skin lesions. [4] Though hemiatrophy has been reported before in SFM, parenchymal cysts in brain have rarely been reported in SFM syndrome. There is mention of porencephalic cysts in the literature. [4] but no report of parenchymal cysts like the ones seen in our case.
| » References | |  |
| 1. | Menascu S, Donner EJ. Linear nevus sebaceous syndrome: Case reports and review of the literature. Pediatr Neurol 2008;38:207-10.  [PUBMED] [FULLTEXT] |
| 2. | Barkovich AJ. Pediatric neuroimaging. 4 th ed. Philadelphia: Lippincott Williams and Wilkins; 2005. |
| 3. | Zhang W, Simos PG, Ishibashi H, Wheless JW, Castillo EM, Breier JI, et al. Neuroimaging features of epidermal nevus syndrome. AJNR 2003;24:1468-70. |
| 4. | Baker RS, Ross PA, Baumann RJ. Neurological complications of the epidermal nervous syndrome. Arch Neurol 1987;44:227-32. |
[Figure 1], [Figure 2]
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