Which neurologic consequence is commonly associated with Sturge-Weber syndrome due to leptomeningeal angiomatosis?

Which neurologic consequence is commonly associated with Sturge-Weber syndrome due to leptomeningeal angiomatosis?

• A. Seizures
• B. Hearing loss
• C. Peripheral neuropathy
• D. Masticatory dysfunction

Answer: (A)

Leptomeningeal angiomatosis creates abnormal blood vessels that overlie the cortex, leading to chronic cortical irritation, ischemia, and gliosis with characteristic cortical calcifications. This environment defines an epileptogenic zone, so seizures are the most common neurologic consequence and often appear early in childhood, sometimes progressing with hemiparesis and cognitive impairment as brain function is affected on the affected side. Imaging typically shows pial angiomatosis with tram-track calcifications along the cortex on the involved side, correlating with the seizure focus. Hearing loss, peripheral neuropathy, or masticatory dysfunction do not arise from this leptomeningeal vascular process in the same way; they are not the typical neurologic manifestations of Sturge-Weber syndrome driven by leptomeningeal angiomatosis.

Leptomeningeal angiomatosis creates abnormal blood vessels that overlie the cortex, leading to chronic cortical irritation, ischemia, and gliosis with characteristic cortical calcifications. This environment defines an epileptogenic zone, so seizures are the most common neurologic consequence and often appear early in childhood, sometimes progressing with hemiparesis and cognitive impairment as brain function is affected on the affected side. Imaging typically shows pial angiomatosis with tram-track calcifications along the cortex on the involved side, correlating with the seizure focus.

Hearing loss, peripheral neuropathy, or masticatory dysfunction do not arise from this leptomeningeal vascular process in the same way; they are not the typical neurologic manifestations of Sturge-Weber syndrome driven by leptomeningeal angiomatosis.