Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

A case of adult-onset Sturge-Weber syndrome type III without intracranial calcification, presenting with transient homonymous hemianopia

Yuto Hayashi, M.D.1), Yuri Sugiura, M.D.1), Rie Nakatani, M.D., Ph.D.1), Katsuya Araki, M.D., Ph.D.1), Masayuki Moriya, M.D., Ph.D.1) and Masaru Yokoe, M.D., Ph.D.1)

1)Department of Neurology, Toyonaka Municipal Hospital

Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder. Almost all cases of SWS are diagnosed in children, but some are diagnosed in adults. We describe a case of isolated leptomeningeal angiomatosis without intracranial calcification. A 33-year-old woman was admitted because of sudden-onset right homonymous hemianopia with headache and nausea. These symptoms disappeared by the next morning. She had no history of seizure or mental retardation. No facial angioma was found on physical examination. Brain CT showed no intracranial calcification or atrophic cortex. The blood and cerebrospinal fluid analyses yielded normal results. The findings in the electroencephalogram were unremarkable. MRI with susceptibility weighting (SWI) revealed dilated transmedullary veins in the left occipital lobe.Contrast-enhanced T1-weighted imaging (CE-T1WI) illustrated abnormal leptomeningeal enhancement in the left occipitoparietal cortex and enhancement and enlargement of the choroid plexus in the left lateral ventricle. Postgadolinium contrast-enhanced f FLAIR imaging demonstrated more extensive enhancement of the leptomeningeal lesions than did CE-T1WI. The symptoms and the findings on these images were suggestive of a diagnosis of SWS type III. Clinicians should keep in mind that some cases of SWS manifest with only minor symptoms, such as migraine. If SWS is suspected, SWI and contrast-enhanced MRI should be performed.
Full Text of this Article in Japanese PDF (1536K) @@@@ Full Text of this Article in English HTML

(CLINICA NEUROL, 61: 132|135, 2021)
key words: Sturge-Weber syndrome, vascular malformation, epilepsy, homonymous hemianopia

(Received: 1-May-20)