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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Case of the Day
Case of the Day
An eight-month-old boy was brought with fever since three days, two episodes of focal convulsions and weakness over the left side of the body. On examination there was ‘port wine’ stain on the right side of face with hypomelanotic patches all over the body.
An eight-month-old boy was brought with fever since three days, two episodes of focal convulsions and weakness over the left side of the body. On examination there was ‘port wine’ stain on the right side of face with hypomelanotic patches all over the body.
Port wine color
On contrast enhanced MRI of the brain, there is intense leptomeningeal enhancement with pachymeningeal enhancement along the right cerebral convexity. There are multiple tortuous pial vessels and enlarged subarachnoid spaces. There is a subdural hygroma on the right side, The frontoparietal calvarium is thickened. There is mild atrophy of the right cerebral hemisphere.
On contrast enhanced MRI of the brain, there is intense leptomeningeal enhancement with pachymeningeal enhancement along the right cerebral convexity. There are multiple tortuous pial vessels and enlarged subarachnoid spaces. There is a subdural hygroma on the right side, The frontoparietal calvarium is thickened. There is mild atrophy of the right cerebral hemisphere.
Sturge-Weber syndrome is named after William Allen Sturge English physician English physician (1850-1919) and Frederick Parkes Weber , English dermatologist (1863-1962), who documented and described the various clinical and radiological features of the disorder in the late 19th and early 20th centuries. William Sturge: described the cutaneous, ocular, and neural features of the disorder in 1879. Frederick Parkes Weber: later documented the radiological changes, such as intracranial calcifications, in 1922. The syndrome was also initially described by Rudolf Schirmer in 1860, but it is named after the detailed contributions of Sturge and Weber.
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