case 1. a-what are the finding ? b-what is the expected history ? c-what is the diagnosis?

Case 1

• A-What are the finding ?

• B-What is the expected history ?

• C-What is the diagnosis?

• FINDING : 5- MARKES • 1- there are of confluent patchy area of large

scattered abnormal T2 signal developing in multiple areas of subcortical white matter bilaterally.

• 2- the lesion is bright on diffusion WI(active disease)

• B- The expected history is post vaccination or post viral infection. 2- MARKES

• Dx:ADEM 3- MARKES

adem

• Findings:  Since the prior normal study, there has been interval development of scattered abnormal T2 signal developing in multiple areas of white matter bilaterally. Specific areas involved include the periventricular white matter, the centrum semiovale, the middle cerebellar peduncles, and the subcortical white matter of the frontal and temporal cortices bilaterally. These abnormal white matter foci do not enhance and do not demonstrate restricted diffusion. MRA images are normal. The visualized intracranial vessels are free of aneurysmal dilatation or stenosis. Vessel morphology and distribution is normal. There is no evidence of vasculitis.

adem• ADEM represents an abnormal immune response to viral illness or

vaccination. The disease is most commonly seen in children, often with an abrupt onset following a viral infection or recent vaccination. Common viral infections causing ADEM include measles, mumps, rubella, and chicken pox. Less commonly, ADEM develops following a disseminated bacterial infection (usually mycoplasma).

• ADEM typically presents with seizure activity or focal neurological signs 4-7 days following the clinical onset of a viral infection. Other signs and symptoms include headache, fever, irritability, dizziness, vomiting, and nuchal rigidity. Symptoms often spontaneously resolve spontaneously over several weeks, though 10-20% of patients experience permanent neurological deficits.

• Grossly, ADEM appears as patchy perivenous inflammation and demyelination throughout the brain, most commonly within the deep nuclei and deep white matter. The etiology of ADEM is unclear, but it is believed to result from an antigen-antibody response against CNS proteins, mainly myelin. Gross specimens have shown marked similarities to experimentally induced cases of allergic encephalitis mediated by anti-myelin.

• Radiologic Overview of the Diagnosis:

• Radiologic Overview of the Diagnosis: • CT images may demonstrate large patchy foci of decreased attenuation within

the sub-cortical white matter, and to a lesser extent, the deep brain nuclei, corresponding to area of demyelination. More commonly, however, CT will fail to demonstrate any evidence of demyelination.

• MR again demonstrates areas of demyelination within the subcortical white matter, as well as the deep nuclei, which are affected in approximately 50% of cases. One or both hemispheres may be involved, though distribution is commonly asymmetric. The brainstem, cerebellar white matter, and spinal cord may be involved, as well. Foci of demyelination are seen as irregular areas of T1 and T2 prolongation, with various patterns of enhancement in the acute phase. Foci of acute and subacute demyelination also demonstrate restricted diffusion.

• Key points: • Scattered demyelination which develops several days after the onset of a viral

prodrome or vaccination. • Typically involves sub-cortical white matter and deep brain nuclei. • May demonstrate enhancement and restricted diffusion in the acute phase.

Case 2

• A-What are the finding ?

• B-What is the expected history ?

• C-What is the diagnosis or differentials Dx?

• Finding: 5- MARKES • 1-there is oval shape small complex mass at

medial aspect of left temporal lobe .• 2- there is small tiny internal cystic changes. Daignosis :DNET 3- MARKES

• DNET ,a low-grade astrocytoma or a ganglioglioma, which are the more common supratentorial tumors seen in young patients.

• They occur in young patients (age range one week to thirty years) who usually present with intractable partial complex seizures.  2- MARKES

• Because DNET does not recur after resection, postoperative radiation or chemotherapy is not needed.

• Pearls:• DNET is a pathologically benign tumor occurring in

young patients who usually present with partial complex seizures.

• The tumors are usually in the temporal lobe and are of low density on CT. On MR, lesions are Tl hypointense and T2 hyperintense and may display cystic features.

• Imaging alone cannot distinguish DNET from a low-grade astrocytoma or a ganglioglioma, which are the more common supratentorial tumors seen in young patients.

• Selected References:

• Dysembryoplastic neuroepithelial tumor (DNET) is a recently described, pathologically benign tumor arising within the supratentorial cortex.  Tumors are occasionally cystic and demonstrate at least one of the following three characteristics:

• a specific glioneuronal component • multinodular lesion consisting of glial nodules • association with focal cortical dysplasia. • They occur in young patients (age range one week to thirty years) who usually

present with intractable partial complex seizures.  Because DNET does not recur after resection, postoperative radiation or chemotherapy is not needed.

• Diagnosis:  CT and MRI for characterization. Diagnosis requires biopsy.• Treatment: Curable by excision.• Radiology: The majority of tumors occur in the temporal lobe with the frontal

lobe being the second most common location.  On CT, they appear as a low-density, well-demarcated lesion which may appear cystic.  The margins of the tumor are well -circumscribed and may remodel the adjacent calvarium.  On MR, they demonstrate low signal on T1-weighted and high signal on T2-weighted images.  Little peritumoral edema is seen.  Differentiation from ganglioglioma or low-grade astrocytoma is not possible by imaging.

• CT:   Hypo-attenuating lesion in the left temporal lobe demonstrating peripheral enhancement and thin corona of surrounding edema.

• MR:   Tl-hypointense, T2-hyperintense 3 cm temporal lobe mass with little surrounding edema.   The mass demonstrates circumferential enhancement and appears cystic centrally.

• CT:   Hypo-attenuating lesion in the left temporal lobe demonstrating peripheral enhancement and thin corona of surrounding edema.

• MR:   Tl-hypointense, T2-hyperintense 3 cm temporal lobe mass with little surrounding edema.   The mass demonstrates circumferential enhancement and appears cystic centrally.

Case 3

• What are the finding?

• What is the diagnosis?

• Finding:(6 mrks)• 1-There is extensive Sino nasal disease

filling and causing of all the Para nasal sinuses especially the maxillary, ethmoid as well as sphenoid air sinus :(2 mrks)

• It appear moderately hyper intense on T1WI and signal void( fungal ball )on T2WI with peripheral enhancement in post contrast study of the fungal ball :(2 mrks)

• There is bulging of the lamina papyrasia bilaterally abutting the medial rectus bilaterally more marked on the right side. :(2 mrks)

• No Intracranial extension .

• Diagnosis fungal sinusitis :(4mrks)

Case 4

• What are the finding?

• What is the diagnosis?

• Finding: :(6 mrks)• 1- left MCA hyper dense sign• 2- blurring of the left basal ganglia• 3- effacement of left cortical sulci (MCA territory.• 4-Mild mass effect on left frontal horn

• Diagnosis : :(4 mrks) acute infarction of

MCA territory .

Case 3

Case 5

• What are the finding?

• What is the diagnosis?

• Mention three clinical or radiological signs?

• 1- fusiform enlargement of the optic chiasma

• 2- there are altered signal intensity

• Of the white matter around the fourth ventricle. (HAMARTOMA)

• Diagnosis NF 1

• Intracranial manifestations of NF1 include development of optic pathway gliomas, cerebral gliomas, hydrocephalus, schwannomas of the cranial nerves, vascular dysplasias, hamartomas, craniofacial plexiform neurofibromas, and spongiotic myelinopathy. +

• NF1 can involve the spine, musculoskeletal system, and the gastrointestinal (GI) tract, and

• Criteria for diagnosis

• Six or more café au lait macules larger than 5 mm in the greatest diameter in prepubertal children and larger than 1.5 cm in postpubertal individuals

• Two or more neurofibromas of any type or one plexiform neurofibroma

• Multiple freckles (Crowe sign) in the axillary or inguinal region • A distinctive osseous lesion, such as sphenoid dysplasia or thinning

of long bone cortex, with or without pseudoarthrosis • Optic glioma • Two or more iris hamartomas (Lisch nodules) seen on slitlamp or

biomicroscopy examination • A first-degree relative (parent, sibling, offspring) with NF1, as

diagnosed by using the criteria above

• Skull • Many skull abnormalities are well demonstrated on plain

radiographs. These include macrocephaly, absence of the greater and lesser wings of the sphenoid, absence of the orbital floor, hypoplasia of the lesser wings of the sphenoid, enlarged orbits, enlargement of cranial foramina, enlargement of orbital margins, sclerosis in the vicinity of the optic foramen (optic nerve sheath meningioma), facial asymmetry, hypoplasia of the paranasal sinuses, mandibular abnormalities, mandibular hypoplasia with flattening of the external contour, thinning of the ramus, coronoid hyperplasia, widening of the lateral and medial coronoid spaces, and calvarial defects adjacent the left lambdoid suture.

• Multiple frontobasal osseomeningeal defects causing cerebrospinal fluid (CSF) rhinorrhea and meningoencephalocele can occur, and the osseous defects may be depicted on plain radiographs.

• Chest • Plain radiograph findings include inferior rib notching, twisted and

ribbonlike ribs in the upper thoracic cage, posterior mediastinal masses secondary to intrathoracic meningoceles, mediastinal and lung masses secondary to neurofibromas, and dumbbell neurofibromas.

• Reported changes of lung parenchyma include progressive pulmonary interstitial fibrosis leading to formation of bullae and honeycomb lung.

• The incidence of spontaneous pneumothorax and hemothorax is increased.

• Changes of interstitial lung disease and pulmonary hypertension may be seen, such as dilatation/enlargement of the central pulmonary arteries and peripheral pruning of vessels.

• Appendicular skeleton • Bowing or an S-shaped deformity of long bones,

hyperplasia or hypoplasia of long and short bones, pseudoarthrosis, erosions, periosteal dysplasia, intramedullary longitudinal osteosclerotic streaks, single or multiple cystic bone lesions, and focal gigantism may be depicted.

• Joint abnormalities reported include protrusio acetabuli, dislocation of the hip, dislocation of the radius and ulna, absence of a patella, and neuropathic arthropathy of the knee.

• Spine • A sharply angled kyphoscoliosis centered at the thoracolumbar

junction is seen in 50% of patients. The kyphosis is more pronounced than the scoliosis, and the incidence increases with advancing age.

• Enlargement of the intervertebral foramina, scalloping of the vertebral bodies (anterior, posterior, lateral), hypoplasia of the vertebral pedicles, wedged-shaped vertebrae, spondylolisthesis, spinal clefts, osteolysis, and spindling of the transverse processes may be depicted.

• Spinal fusion may be complicated by pseudoarthrosis and curve progression.

• Spinal segments may be unstable, leading to subluxation or dislocation.

Case 6

What is the study?

What are the finding?

What is the diagnosis?

• 1- sagittal TIWI

• 2-sagittal T2WI FAT SAT.

• 3-Post contrast T1WI FAT SAT

• 4- MRV

• Finding:

• 1-MR images nicely show this vascular channel in the right occipital region traversing the calvarium

• 2-this venous channel communication with superior sagittal sinus to the dilated diploic veins of the skull

• diagnosis : Sinus pericranii

• Sinus pericranii is a rare disorder characterized by a congenital or acquired epicranial blood-filled nodule of the scalp that is in communication with an intracranial dural sinus through dilated diploic veins of the skull. Simply put, it connects the intracranial and extracranial venous systems. Sinus pericranii usually present in the pediatric age group as a focal swelling on the scalp. They are felt to be predominantly congenital in origin. Sinus pericranii are mostly located near the midline. The frontal region is most commonly involved, followed by the parietal and the occipital region (as in our case). It is soft and mobile and may be mistaken for a lipoma or subcutaneous cyst. Treatment is usually surgical.

• Radiologic Overview of the Diagnosis • The plain film was ordered to assess for osseous involvement. The ultrasound

was indicated as a vascular malformation was in the differential given the soft nature of the nodule. It was fortunate that this was done prior to biopsy as the ultrasound demonstrated communication with the transverse sinus.

• Key points: • Vascular malformation that connects the intracranial and extracranial venous

systems. • Ultrasound helpful for pre-biopsy characterization of soft tissue abnormalities.

• These findings are compatible with a sinus pericranii. MR images nicely show this vascular channel in the right occipital region traversing the calvarium.

•  Head CT demonstrates left frontal vascular anomaly with thinning of the calvarium. MRI demonstrates communication of the intracranial and extracranial circulations.

• There is a small, oval-shaped flow-void within the mid-anterior parietal scalp. This lesion communicates with the superior sagittal sinus and an adjacent dilated scalp vein, thus creating an abnormal communication between the intracranial and extracranial venous systems. Within this midline lesion, Differential Diagnosis:  Lytic lesion in the skull of a child

• Congenital anomaly (e.g., Sinus pericranii) • Eosinophilic granuloma  • Fibrous Dysplasia • Hemangioma • Infection • Metastases

CASE 7

• 1-WHAT ARE THE FINDINGS ?

2-MENTION THE IMPORTANT CLINCAL FINDING?

3-what is the diagnosis?

• 1- The cerebral hemispheres demonstrate asymmetric atrophy, marked on the right, with enlargement of the subjacent subarachnoid spaces

• 2- Thin, gyriform calcification is present in the distribution of the right occipital lobe.

• Clinical features of Sturge-Weber include facial port-wine stain nevi ipsilateral to the cerebral findings, mental retardation, hemianopsia and hemiplegia

• The causative etiology is unknown, but the disease is characterized by angiomata in the distribution of the fifth cranial nerve and in the leptomeninges, most commonly in the parietal and occipital lobes.

Sturge weber

• Findings:  The cerebral hemispheres demonstrate asymmetric atrophy, marked on the left, with enlargement of the subjacent subarachnoid spaces. Bulky, gyriform calcification is present in the distribution of the left posterior temporal, parietal and occipital lobes. A smaller focus of calcification is seen in the left frontal lobe as well. There is no evidence of acute hemorrhage or infarct.

• Sturge-Weber syndrome is sporadic disease known descriptively as encephalotrigeminal angiomatosis.  The causative etiology is unknown, but the disease is characterized by angiomata in the distribution of the fifth cranial nerve and in the leptomeninges, most commonly in the parietal and occipital lobes.  This focal abnormal development of venous drainage results in vascular congestion, ischemia, atrophy and eventually calcification.  "Port-wine" vascular nevus flammeus in the trigeminal nerve distribution (most commonly in V1) is a commonly encountered cutaneous finding.  Patients frequently present with seizure in the first year of life.  These are usually focal and involve the side of the body contralateral to the nevus.  Focal cerebral ischemia may result in dystrophic calcification, seizure (80%), mental retardation (>50%), hemianopsia, and hemiplegia.  About one third of patients will have ocular involvement, with buphthalmos and glaucoma.

• Radiology: • Calcification may be seen on plain film of the skull, and on the CT scanogram. 

Significant calcium deposition before the age of 2 is uncommon.  Tram-tracking is the characteristic finding, resulting from calcification of apposing gyri surrounding a dilated sulcus.  CT demonstrates gyriform, curvilinear calcification, most prominent in the parietal and occipital lobes ipsilateral to the facial nevus.  Secondary changes in the skull are sometimes present, such as enlargement of the paranasal sinuses and mastoid air cells.  Strong post-contrast enhancement may be seen in both the angiomata, as well as the ipsilateral choroid plexus.  Prominent collateral drainage through subependymal and medullary veins may be seen, particularly with angiography or MR venography.

• Pearls:• Clinical features of Sturge-Weber include facial port-wine stain nevi ipsilateral

to the cerebral findings, mental retardation, hemianopsia and hemiplegia. • Atrophy and dystrophic calcification is secondary to venous congestion. • Parieto-occipital distribution is most common. • Calcifications are gyriform.

Case 4 زايدة حالة