Rehabilitation Management of A Child with Atypical Case of Sturge Weber Syndrome

Authors

  • Selly Christina Anggoro Rsup Persahabatan

DOI:

https://doi.org/10.25170/djm.v21i2.3378

Keywords:

Epilepsy; Leptomeningeal angioma; Port-wine birthmark; Stroke-like episodes; Sturge–Weber syndrome.

Abstract

Sturge-Weber syndrome (SWS) is a capillary-venous malformation affecting the brain, the eye, and the adjacent trigeminal dermatomes of the skin or a facial port wine stain or nevus. Type 3 SWS is a form of disease that comes without the physical facial angioma that made the disease undiagnosed and untreated optimally. We report a case of a 6-year-old girl with history of seizures without fever since she was 13 months old. The seizure was repeated couple times within a month and a year afterwards, but relatively controlled with routine oral medications, with last seizure attack was when she was 4 years 8 months old.  Unluckily, by the age of 6 and 3 months old, she was brought to the emergency department due to sudden focal seizure and weakness on her right extemities. The enhanced MSCT angio and brain MRI examination revealed the abnormality of the cerebral angiography, and raised the diagnostic of Sturge-Weber syndrome. After several medical treatment and rehabilitation programs, her gross motor ability and balance was getting much improved with some dysfunction on fine motor ability on her right hands and little difficulties on her basic speech. There was also some disturbance of her cognitive skill since she seemed to have a slower memory recall and speed processing after the latest seizure attack.  

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References

1.Pascual-Castroviejo I, Diaz-Gonsales C, Garcia-Melian RM, Gonzales-Casado I, Munoz- Hiraldo E. Sturge-Weber syndrome. Study of 40 patients. Pediatr Neurol 1993;9:283-8.
2.(rare)Sturge Weber Syndrome. Downloaded from https://rarediseases.org/rare-diseases/sturge-weber-syndrome/.
3.Rochkind S, Hoffman HJ, Hendrick EB. Sturge-Weber syndrome: Natural history and prognosis. J Epilepsy 1990;3:293-304.
4.Zanzmera P, Patel T, Shah V. Diagnostic dilemma: Sturge-Weber syndrome, without facial nevus. J Neurosci Rural Pract. 2015 Jan-Mar; 6(1): 105–107. doi: 10.4103/0976-3147.143215. Download from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4244768/
5.Sudarsanam A, Ardern-Homes SL. Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol. 2014 May;18(3):257-66. doi: 10.1016/j.ejpn.2013.10.003. Epub 2013 Nov 7.
6.Comi A. Current Therapeutic Options in Sturge-Weber Syndrome. Semin Pediatr Neurol. 2015;22(4):295-301. doi:10.1016/j.spen.2015.10.005
7.Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg. 2004;23:87–98. [PubMed] [Google Scholar]
8.Sujansky E, Conradi S. Sturge-Weber syndrome: Age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol. 1995;10:49–58. [PubMed] [Google Scholar]
9.Roach ES. Neurocutaneous syndromes. Pediatr Clin North Am. 1992;39:591–620. [PubMed] [Google Scholar]
10.Physical Medicine and Rehabilitation. Downloaded from: https://www.sciencedirect.com/topics/neuroscience/physical-medicine-and-rehabilitation
11.What is Physical Medicine and Rehabilitation? Downloaded from: https://www.aapmr.org/about-physiatry/about-physical-medicine-rehabilitation

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Published

2022-08-28

How to Cite

1.
Anggoro SC. Rehabilitation Management of A Child with Atypical Case of Sturge Weber Syndrome. DJM [Internet]. 2022 Aug. 28 [cited 2024 Nov. 5];21(2):184-93. Available from: https://ejournal.atmajaya.ac.id/index.php/damianus/article/view/3378
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