Sturge-Weber syndrome (encephalotrigeminal angiomatosis)

Findings

Figure 1 and 2: Enlargement of left frontal sinus; generalized left hemishperic cerebral atrophy with significant white matter involvement; enlargement of ipsilateral choroid plexus of this noncontrast enhanced study; low intensity gyral signal corresponding to gyral calcifications. If CT had been performed in this case, classic "tram-track" calcifications would be seen.
Figure 3: Hyperintense signal within remaining white matter on FLAIR consistent with gliosis.
Figure 4 and Figure 5: Hyperpneumatization of left frontal sinus (Figure 4); generalized left cerebral hemispheric atrophy; skull thickening (Figure 4); enlargement of ipsilateral choroid plexus.


Diagnosis: Sturge-Weber syndrome(encephalotrigeminal angiomatosis)


Sturge Weber syndrome (encephalotrigeminal angiomatosis) is a rare neurocutaneous syndrome in which patient has port wine nevus in V1 (ophthalmic branch) distribution of trigeminal nerve, seizures and occasionally hemiplegia. It is a predominantly sporadic disease. The basic pathophysiology is likely due to faulty development of cortical venous drainage resulting in leptomeningeal angioma formation, venous stasis, vascular congestion, and hypoxia of the affected cortex. Slowly progressive atrophy of the brain underlying the angioma occurs, along with the characteristic cortical calcifications.
On imaging, the classic findings include hemiatrophy with gliosis, ipsilateral choroid plexus hypertrophy due to collateral deep venous flow, and leptomeningeal enhancement (early) or “tram-track” cortical calcifications (late). Enlargement of deep medullary white matter veins can be seen on enhanced MR images and MR venography may show a paucity of cortical veins.
Although the cortical calcifications are best seen on CT imaging, they may also be identified as low-signal intensity cortical signal along cortical gyri. Cortical calcifications are usually progressive, posterior to anterior.
In addition, imaging findings include Dyke-Davidoff-Masson syndrome, characterized by elevated petrous ridge/sphenoid wing, and hemihypertrophy of the skull. There is also hyperpneumatization of the ipsilateral paranasal sinuses, usually frontal sinuses.
Treatment includes longstanding seizure therapy. If seizures are not controlled by medication, surgical resection of the affected lobe may be performed.

Practice Panning on a Rainy Day

After months of living without natural rain, Luzon finally had two typhoons in a span of three days. Well, the dry spell may be over for now, but traffic and floods take over.

For me, though, it's time to practice my panning shots. I still haven't perfected this technique. And this is especially difficult on a rainy day.

I have to boost up my camera's ISO to make up for the relative darkness caused by the typhoon (a little shake can easily ruin the shot, especially as I don't have an expensive lens with a VR or IS).

I also had to protect my camera from the rain. It is very difficult to be holding the camera steady and clutch the umbrella under my arm. Some people looked at me thinking maybe I'm a media man trying to shoot photographs for tomorrow's newspapers.

So here I just want to share two almost "successful" shots I made this afternoon.... Okay, I know I have to practice more.
(By the way I used here my D40x camera with the 18-55 kit lens)

Spinal arteriovenous malformation

Here is a classical MR picture of Spinal AVM, again submitted by Dr MGK Murthy,

Sr Consultant, Teleradiology Providers, http://www.teleradproviders.com/

Choroid plexus papilloma

Findings

There is a frond-like mass in the trigone of the right lateral ventricle with mostly isointense signal on T1 and T2, demonstrating intense contrast enhancement. There is no local parenchyma invasion. There is severe hydrocephalus.

Differential diagnosis:
- Choroid plexus papilloma
- Choroid plexus carcinoma
- Choroid plexus meningioma
- Villous hypertrophy
- Intraventricular metastasis
- Ependymoma


Diagnosis: Choroid plexus papilloma


Key points

General
- Tumor that arise from the choroid plexus epithelium
- WHO Grade I tumor – benign slow growing tumor
- Four to eight times more common than choroid plexus carcinoma

Epidemiology
- Most common brain tumor in those under 1 year of age
- 75% of cases are in those under 10 years old
- Approximately to 2 to 4% of pediatric brain tumors
- Approximately 0.5% of adult brain tumors

Clinical Presentation
- Signs and symptoms of increased intracranial pressure from CSF overproduction and impaired CSF resorption
- May present with focal neurologic deficit

Treatment
- Surgical resection
- Almost 100% 5 year survival rate for choroid plexus papilloma
- Often require post resection shunting


Radiology

Lobulated, frond, or "cauliflower-like" intraventricular mass
Often arises in the lateral ventricle trigone in children
Often arises in the 4th ventricle and cerebellopontine angle in adults
May have drop metastasis to the spinal canal
Associated with hydrocephalus because of CSF overproduction or obstruction
Only limited local parenchymal invasion (compared to choroid plexus carcinoma)
Often difficult to differentiate from choroid plexus carcinoma

CT
- Iso or hyper dense ventricular mass
- 25% contain punctuate calcification
- Intense enhancement
- May have cysts or hemorrhage

MRI
- T1 Iso or hypo intense
- T2 variable hyper intense, with flow voids
- Intense enhancement

CSF Rhinorrhea-MRI appearance

Here is a MRI T2 weighted coronal image of a patient with CSF Leak through the cribiform plate. Case By Dr MGK Murthy. Sr Consultant Teleradiology Providers

Clinicians get the critical imaging results late!!

"Referring clinicians failed to electronically acknowledge over one-third of abnormal imaging results in an outpatient setting, even when a computerized test result notification system designed to alert referring physicians was used, according to a paper from Texas. Providers were unaware of critical imaging results in 4% of cases as long as four weeks after reporting."

Source-Diagnostic Imaging

Ventriculitis

Findings

There is a left parieto-occipital ventriculostomy catheter is present entering the left occipital horn. There is diffuse dilation of the lateral ventricles (left greater than right), third ventricle, and fourth ventricle. There is focal, marked enhancement of the occipital horns of the ventricles bilaterally, with debris noted dependently in the lateral ventricles. This debris demonstrates restricted diffusion. There is T2 prolongation in the white matter surrounding the occipital horns bilaterally.

Differential diagnosis:
- Bilateral lateral ventriculitis
- Lymphoma
- Intraventricular hemorrhage
- Ependymal tumor spread (primary or metastatic)
- Prominent ependymal veins


Diagnosis: Ventriculitis


Key points


Ventriculitis is infection of the ependyma lining the ventricles of varying etiologies.
May be caused by rupture of brain abscess into the ventricle, as a complication of meningitis (30% of cases), or as a complication of neurosurgical devices (most common ventriculostomy).
Can be caused by viral, fungal, bacterial, or parasitic organisms. Most common – bacterial (Staphylococcus, Streptococcus, Enterobacter). Viral or fungal – immuno-compromised patients.
High mortality – 40-80%
Treatment – surgical irrigation and drainage, treatment of infectious etiology (antibiotics)

Imaging findings:
- CT: Ventriculomegaly with diffuse enhancement of the ventricular walls. Usually layering debris within the ventricles. Subtle low density surrounding the ventricles (edema).
- MR: Ventriculomegaly with layering debris in the ventricles (hyperintense on T1WI and FLAIR). Debris will demonstrate restricted diffusion on DWI (pus). Bright enhancement of ventricular walls. May have associated findings of choroid plexitis (uncommon).