Master Sturge Weber Syndrome with Picmonic for Medicine

With Picmonic, facts become pictures. We've taken what the science shows - image mnemonics work - but we've boosted the effectiveness by building and associating memorable characters, interesting audio stories, and built-in quizzing.

Sturge Weber Syndrome

Sturgeon fish with webs
Picmonic
Sturge-Weber syndrome, sometimes called encephalotrigeminal angiomatosis, is a rare congenital neurocutaneous disorder characterized by proliferation of arteries in the brain, resulting in multiple angiomas. Specifically, patients demonstrate leptomeningeal angiomas, along with arteriovenous malformations (AVMs). This syndrome is often associated with port-wine stains on the face, ipsilateral to the leptomeningeal angioma, along with neurologic consequences, like seizures, varying degrees of mental retardation, and glaucoma. Normally, all manifestations of this disease are on the same side of the head. There is also an increased incidence of pheochromocytomas. On CT, patients with Sturge-Weber syndrome display tram-track calcifications, which indicate cortical and subcortical calcifications.
9 KEY FACTS
PATHOPHYSIOLOGY
GNAQ Gene
G-neck Genie

Sturge-Weber syndrome is not inherited, but rather occurs sporadically as a result of a somatic activating mutation occurring in the GNAQ gene.

SIGNS AND SYMPTOMS
Port Wine Stain
Port red Wine

Port-wine stains, also called nevus flammeus, or firemarks, are birthmarks that occur as a result of capillary malformations in the skin. Port-wine stains tend to grow proportional to the growth of a child, rather than fading. They may present as a red color, maturing to a dark red, or purple color with age. These can present anywhere on the body, but are most often seen on the face. Lesions in the trigeminal nerve distribution are sometimes associated with Sturge-Weber syndrome.

Arterial Venous Malformation
Web of Arteries and Veins

In Sturge-Weber syndrome patients are subject to various permutations of arteriovenous malformations (AVM). These include capillary malformations, leading to facial port-wine stains, along with malformations in the retinal vessels, pia mater and choroid in the area of the trigeminal nerve.

Leptomeningeal Angiomas
Laptop in meninges

Patients with Sturge-Weber syndrome may develop abnormal proliferation of blood vessels in the leptomeninges, which refer to the pia mater and arachnoid mater of the brain and spinal cord. Commonly, patients with Sturge-Weber syndrome have leptomeningeal angiomas on the ipsilateral side as their port-wine stain.

Intellectual Disability (Mental Retardation)
Read-tarred-book

In Sturge-Weber syndrome, neurological concerns relate to the extent of angioma growth on brain tissue. This may result in neurologic consequences, such as developmental delay, or mental retardation.

Seizures
Caesar

In this syndrome, angiomas (occurring as a result of arteriovenous malformations) create abnormal conditions for brain function. As a result, patients with Sturge-Weber syndrome may display seizure activity, and this is the most common early neurologic manifestation, often starting by one year of age. Patients may have initial focal seizures progressing to frequent, secondary generalized seizures, along with increasing seizure frequency and duration, despite the use of antiepileptic drugs. Therapies include surgical intervention, vagal nerve stimulation and anticonvulsant drug therapy.

Pheochromocytoma
Phiat-chrome

Pheochromocytomas are adrenal gland tumors that secrete catecholamines. Patients with Sturge-Weber syndrome have a higher predisposition towards developing pheochromocytomas than the normal population.

Glaucoma
Glock-eye

Those with Sturge-Weber syndrome often have ocular involvement, typically in the form of glaucoma, a disorder of increased fluid pressure in the eye. These most commonly present ipsilaterally to the site of the patient’s port-wine stain, and patients can display vision loss, strabismus, myopia, visual field defect, optic nerve cupping and corneal edema.

Tram-Track Calcifications
Tram-Track

On CT scan, patients with Sturge-Weber syndrome display tram-track calcifications, which indicate cortical and subcortical calcifications.

Unlock all 9 facts & definitions with Picmonic Free!

JOIN FREE

Take the Sturge Weber Syndrome Quiz

Picmonic's rapid review multiple-choice quiz allows you to assess your knowledge.

START QUIZ NOW

It's worth every penny

Our Story Mnemonics Increase Mastery and Retention

Memorize facts with phonetic mnemonics

Unforgettable characters with concise but impactful videos (2-4 min each)

Ace Your Medical (MD/DO) Classes & Exams with Picmonic:

Over 900,000 students use Picmonic’s picture mnemonics to improve knowledge, retention, and exam performance.

Choose the #1 Medical (MD/DO) student study app.

Picmonic for Medicine (MD/DO) covers information that is relevant to your entire Medical (MD/DO) education. Whether you’re studying for your classes or getting ready to conquer the USMLE Step 1, USMLE Step 2 CK, COMLEX Level 1, or COMLEX Level 2, we’re here to help.

Works better than traditional Medical (MD/DO) flashcards.

Research shows that students who use Picmonic see a 331% improvement in memory retention and a 50% improvement in test scores.