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|Year : 2021 | Volume
| 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
|Date of Submission||25-Feb-2020|
|Date of Decision||28-Apr-2020|
|Date of Acceptance||09-Jul-2020|
|Date of Web Publication||2-Sep-2021|
Omair Ashraf Shah
167 Nursingh Garh, Karanagar, Srinagar, Jammu and Kashmir - 190 010
Source of Support: None, Conflict of Interest: None
|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 2022 Jan 23];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. 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. In addition to the neurological features, endocrine presentation can be seen in the form of hypopituatrism.,
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|| |
Bebin EM, Gomez MR. Prognosis in Sturge-Weber disease: Comparison of unihemispheric and bihemispheric involvement. J Child Neurol 1988;3:181-4.
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.
Russell JD, Wise PH, Rischbieth HG. Vascular malformations of the hypothalamus: A cause of isolated growth hormone deficiency. Pediatrics 1980;66;306-9.
Billson VR, Gillam GL. An unusual case of Sturge-Weber syndrome. Pathology 1984;16:462-5.
[Figure 1], [Figure 2], [Figure 3]