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NEUROIMAGE
Year : 2021  |  Volume : 69  |  Issue : 4  |  Page : 1135-1136

Sturge Weber Syndrome with Pituitary Macroadenoma in an Adult: An Unusual Association


Department of Radiology, SKIIMS, Soura, Jammu and Kashmir, India

Date of Submission25-Feb-2020
Date of Decision28-Apr-2020
Date of Acceptance09-Jul-2020
Date of Web Publication2-Sep-2021

Correspondence Address:
Omair Ashraf Shah
167 Nursingh Garh, Karanagar, Srinagar, Jammu and Kashmir - 190 010
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.325331

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How to cite this article:
Gojwari TA, Shah OA, Hussain A, Mohiuddin A, Hassan G, Masood M, Hamid I. Sturge Weber Syndrome with Pituitary Macroadenoma in an Adult: An Unusual Association. Neurol India 2021;69:1135-6

How to cite this URL:
Gojwari TA, Shah OA, Hussain A, Mohiuddin A, Hassan G, Masood M, Hamid I. Sturge Weber Syndrome with Pituitary Macroadenoma in an Adult: An Unusual Association. Neurol India [serial online] 2021 [cited 2021 Sep 25];69:1135-6. Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1135/325331




Sturge Weber Syndrome (SWS) is a congenital anomaly characterized by a facial port wine stain in trigeminal distribution, retinal choroidal angioma and leptomeningeal angioma.[1] SWS usually presents in the form of seizures, headache or ocular features and seizures usually present in the first year of life in 75% of the patients.[2] In addition to the neurological features, endocrine presentation can be seen in the form of hypopituatrism.[3],[4]

A 30-year-old male patient was admitted to our center with a history of doubtful seizure episode. On inquiring from family members, a history of recurrent seizures from the age of 6 yrs was obtained. On examining the patient, reddish discoloration of left half of the face was observed [Figure 1]. A CT scan of the head was done, which showed atrophy of the left cerebral hemisphere with subtle gyral calcifications and hyperostosis of overlying bone especially along frontal lobe [Figure 2]. MRI was done for further evaluation which showed morphological features similar to CT, but on CEMRI images, characteristic leptomeningeal enhancement was seen suggestive of a leptomeningeal angioma. A suprasellar mass showing features characteristic of a macroadenoma was also seen and endocrine evaluation was ordered [Figure 3]a and [Figure 3]b. Taking into account the characteristic facial port wine angioma and leptomeningeal angioma on CEMRI in addition to the history of epilepsy, a diagnosis of Sturge Weber Syndrome was made. The presence of a non-functional pituitary macroadenoma was also documented.
Figure 1: Image of the patient showing the typical left sided port wine angioma

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Figure 2: Non contrast CT image showing atrophy of left cerebral hemisphere, subtle calcifications in the occipital lobe and thickened bone

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Figure 3: (a) T1 post contrast sagittal image showing enhancing sellar suprasellar lesion suggestive of a pituitary macroadenoma. (b) T1 sagittal post contrast image showing characteristic enhancing leptomeningeal angioma. Thickening of overlying calvaria is also noted

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  References Top

1.
Bebin EM, Gomez MR. Prognosis in Sturge-Weber disease: Comparison of unihemispheric and bihemispheric involvement. J Child Neurol 1988;3:181-4.  Back to cited text no. 1
    
2.
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.  Back to cited text no. 2
    
3.
Russell JD, Wise PH, Rischbieth HG. Vascular malformations of the hypothalamus: A cause of isolated growth hormone deficiency. Pediatrics 1980;66;306-9.  Back to cited text no. 3
    
4.
Billson VR, Gillam GL. An unusual case of Sturge-Weber syndrome. Pathology 1984;16:462-5.  Back to cited text no. 4
    


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