SavedAdd your journal

Journals / Epilepsi / 2018 / Cilt: 24 - Sayı: 2

Sturge Weber Syndrome Diagnosed at Late Age, Associated with Psychiatric Complaints and Status: A Case Report

Geç Yaşta Tanı Konan, Psikiyatrik Yakınmaların ve Statusun Eşlik Ettiği Sturge Weber Sendromu: Olgu Sunumu

Hilal TAŞTEKİN TOZ
EREN GÖZKE
Boran Can SARAÇOĞLU
Journal
Epilepsi
Pages
78–82
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

The patient was discharged after 10 days with a normal neurological examination and follow-up has not revealed any seizure or sequel findings with the ASA and valproic acid therapy.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome characterized by a usually unilateral facial port-wine stain, ipsilateral leptomeningeal angiomatosis, glaucoma, epileptic seizures that are difficult to control, and progressive cerebral dysfunction.
📊
Key Findings & Data:A 41-year-old woman presented with headache, nausea, vomiting, and a speech disorder.
💡
Conclusion & Impact:The patient was discharged after 10 days with a normal neurological examination and follow-up has not revealed any seizure or sequel findings with the ASA and valproic acid therapy.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome characterized by a usually unilateral facial port-wine stain, ipsilateral leptomeningeal angiomatosis, glaucoma, epileptic seizures that are difficult to control, and progressive cerebral dysfunction. A 41-year-old woman presented with headache, nausea, vomiting, and a speech disorder. On examination a lesion on the right side of her face consistent with hemangioma was noted, as well as a high fever, hyperglycemia, and tonsillitis. Electroencephalography revealed a paroxysmal abnormality in the right frontotemporal region, and contrast-enhanced cranial magnetic resonance imaging revealed vascularization and dural contrast enhancement in the right cerebral hemisphere. The patient was diagnosed with SWS and discharged with acetylsalicylic acid (ASA) and valproic acid therapy, but returned 2 weeks later with tonic-clonic status epilepticus. The seizures were controlled with an infusion of diazepam in the intensive care unit and the dosage of valproic acid was increased. Aripiprazole was initiated for visual and auditory hallucinations. The patient was discharged after 10 days with a normal neurological examination and follow-up has not revealed any seizure or sequel findings with the ASA and valproic acid therapy.

Özet

Sturge-Weber sendromu (SWS), yüzde porto şarabı renginde çoğunlukla tek taraflı fasiyal anjiom, aynı tarafta leptomeningeal anjiomatozis, glokom, kontrolü zor epilepsi nöbetleri ve ilerleyici serebral fonksiyon bozukluğu ile karakterize nadir görülen sporadik bir nörokutanöz sendromdur. Baş ağrısı, bulantı, kusma, konuşma bozukluğu yakınmaları ile başvuran 41 yaşında kadın hastada sağ yüz yarımında hemanjiomla uyumlu lezyon, yüksek ateş, hiperglisemi ve tonsillit saptandı. Elektroensefalografide (EEG) sağ frontotemporal bölgede paroksismal bozukluk ile uyumlu bulgular ve kontrastlı beyin manyetik rezonans görüntülemede (MRG) sağ serebral hemisferde artmış vaskülarizasyon ile dural kontrastlanma görüldü. Sturge-Weber sendromu tanısı konulan hasta asetil salisilik asit (ASA) ve valproik asit tedavisi ile taburcu edildi, ancak çıkışından iki hafta sonra status epileptikus tablosu ile getirildi. Nöbetleri yoğun bakım şartlarında diazepam infüzyonu ile kontrol altına alındı. Valproik asit dozu yükseltildi. Daha sonra ortaya çıkan görsel ve işitsel halüsinasyonlar nedeni ile aripiprazol başlandı. On gün sonra nörolojik muayenesi normal olarak taburcu edildi. Valproik asit ve ASA tedavisi ile nöbetsiz ve sekelsiz olarak takip edilmektedir

🔖

Cite This Article

TOZ, H. T., GÖZKE, E., SARAÇOĞLU, B. C. (2018). Sturge Weber Syndrome Diagnosed at Late Age, Associated with Psychiatric Complaints and Status: A Case Report. Epilepsi, Cilt: 24 - Sayı: 2, 78–82. https://ajindex.com/articles/152908

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (11)

  1. Sturge WA. A case of partial epilepsy, apparently due to a lesion of one of the vasomotor centers of the brain. Trans Clin Soc London 1879;12:162–7. Weber FP. A Note on the Association of Extensive Hæmangiomatous Nævus of the Skin with Cerebral (Meningeal) Hæmangioma, especially Cases of Facial Vascular Nævus with Contralateral Hemiplegia. Proc R Soc Med 1929;22(4):431–42.
  2. Lopez J, Yeom KW, Comi A, Van Haren K. Case report of subdural hematoma in a patient with Sturge-Weber syndrome and literature review: questions and implications for therapy. J Child Neurol 2013;28(5):672–5.
  3. Ünalp A, Uran N, Ersahin Y. A case of Sturge-Weber syndrome who benefit from early surgical intervention [Article in Turkish]. Ege Tıp Derg 2008;47(2):151–3.
  4. Demir Ö, Yazıcı T. Sturge-Weber Syndrome with Different Intracranial Pathologies: Case Report [Article in Turkish]. Turkiye Klinikleri J Med Sci 2012;32(3):882–6. Haslam RHA. The nervous system. In: Behrman RE, Kliegman RM, Nelson WE, Vaughan III VC, editors. Nelson Textbook of Pediatrics. 14th ed. Philadelphia: W. B. Saunders Company; 1992. p. 1473–538. Comi AM. Pathophysiology of Sturge-Weber syndrome. J Child Neurol 2003;18(8):509–16.
  5. Kossoff EH, Hatfield LA, Ball KL, Comi AM. Comorbidity of epilepsy and headache in patients with Sturge-Weber syndrome. J Child Neurol 2005;20(8):678–82.
  6. Canpolat M, Per H, Yıkılmaz A, Gümüş H, Özcan A, Poyrazoğlu HG, et al. Sturge-Weber syndrome: clinical and radiological evaluation [Article in Turkish]. Gaziantep Tıp Derg 2013;19(1):30–4.
  7. Turin E, Grados MA, Tierney E, Ferenc LM, Zabel A, Comi AM. Behavioral and psychiatric features of Sturge-Weber syndrome. J Nerv Ment Dis 2010;198(12):905–13.
  8. Utsunomiya T, Shimizu H, Sunaga S, Sugano H, Arai N. A case of Sturge-Weber syndrome with severe temper tantrum [Article in Japanese]. No Shinkei Geka 2006;34(8):819–24.
  9. Planche V, Chassin O, Leduc L, Regnier W, Kelly A, Colamarino R. Sturge-Weber syndrome with late onset hemiplegic migrainelike attacks and progressive unilateral cerebral atrophy. Cephalalgia 2014;34(1):73–7.
  10. Cambon H, Truelle JL, Baron JC, Chiras J, Tran Dinh S, Chatel M. Focal chronic ischemia and concomitant migraine: an atypical form of Sturge-Weber angiomatosis? [Article in French]. Rev Neurol (Paris) 1987;143(8-9):588–94.
  11. Suskauer SJ, Trovato MK, Zabel TA, Comi AM. Physiatric Findings in Individuals with Sturge-Weber Syndrome. Am J Phys Med Rehabil 2010;89(4):323–30. Sachdev P. Schizophrenia-like psychosis and epilepsy: the status