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

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

Tariq A Gojwari, Omair Ashraf Shah, Arshad Hussain, Ajaz Mohiuddin, G Hassan, Mohammad Masood, Imran Hamid 
 Department of Radiology, SKIIMS, Soura, Jammu and Kashmir, India

Correspondence Address:
Omair Ashraf Shah
167 Nursingh Garh, Karanagar, Srinagar, Jammu and Kashmir - 190 010
India




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;69:1135-1136


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 Dec 5 ];69:1135-1136
Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1135/325331


Full Text



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}{Figure 2}{Figure 3}

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References

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3Russell JD, Wise PH, Rischbieth HG. Vascular malformations of the hypothalamus: A cause of isolated growth hormone deficiency. Pediatrics 1980;66;306-9.
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