sex,activity ,learning ---all lead to?

WHAT IS SUBARACHNOID CHORISTOMA?

Q.All are true regarding intracranial lipoma except

a. subarachnoid choristomas, congenital malformations

b. due to maldifferentiation of the meninx primitive

c. most common location ---36% pericallosal, 25% quadrigeminal/superior cerebellar cisterns

d. intracranial vessels  and cranial nerves often course around  lipomas

e. interhemispheric lipomas are nearly always associated with hypogenesis of the corpus callosum

ANS .—d

 Intracranial lipomas may be defined as subarachnoid choristomas. They are abnormal collections of fat, neither hamartomatous nor neoplastic, believed derive from maldifferentiation of the meninx primitiva as a disorder of development of the subarachnoid space

Intracranial vessels (e.g., pericallosal arteries) and cranial nerves often course through, rather than around, lipomas .

What is LISI mutations ?

Q. The above image finding is associated with all the following genes except

 a.LIS1 mutations.

b.DCX (doublecortin) mutations.

c. PROKR2

d.ARX mutations.

e.RELN mutations

ANS .---c

PROKR2 gene is associated with Kallman syndrome.

Kallmann syndrome may be of X-linked, autosomal dominant, or autosomal recessive inheritance . It can be divided by genotype into four different types: KAL1 (X-linked) and the autosomal types KAL2, KAL3, and KAL4.

VLDLR mutations is associated with the lissencephaly band heterotopia spectrum.

LIS1 mutations may result in the phenotypes of the Miller-Dieker syndrome, isolated lissencephaly sequence (ILS), or subcortical band heterotopia (SBH)

The DCX gene, or XLIS located on Xq22.3-q23, encodes a protein named doublecortin, which, similar to LIS1, depending on the type of mutation, may result in the phenotype of ILS, SBH or central pachygyria

Dorsalizing gradient genes --Septooptic dysplasia (SOD)

552.All are true regarding  septooptic dysplasia (SOD) except

a. overrexpression of dorsalizing gradient genes 

b. optic nerve hypoplasia and deficiency of the septum pellucidum

c. HESX1 gene implicated

d. concurrent schizencephaly  in 50% of patients

e. hypoplasia of the hypothalamus

ANS ---a

Neural Tube Dorsalizing Gradient Mutations are divided into those that involve overexpression of dorsalizing gradient genes, such as duplication of the dorsal horns of the spinal cord, duplication of dorsal brainstem structures, and the dorsal interhemispheric variant of HPE, and those that involve underexpression of dorsalizing gradient genes, such as septooptic dysplasia (SOD).

Who does not want to remember "her "?

Diagnostic Criteria For Neurofibromatosis Type 1

Two or more must be present: 1.Café-au-lait spots ≥6 (5 mm child, 15 mm adult) 2.Neurofibromas: ≥2 3.Plexiform neurofibroma: 1 4.Axillary (intertriginous) freckling 5.Optic nerve glioma 6.Lisch nodules (iris hamartomas): ≥2 7. “Distinctive bone lesions”: sphenoid wing dysplasia or long bone dysplasia 8.First-degree relative with neurofibromatosis 1

 

Diagnostic Criteria for Neurofibromatosis Type 2

1.Bilateral cerebellopontine angle (CPA) masses (histologic proof not required) 2.A first-degree relative with neurofibromatosis 2 and either        a. A unilateral CPA mass         b.Any two of the following: schwannoma                                                      ; neurofibroma                                                       ;meningioma                                                      ;  glioma                                                    ; juvenile posterior subcapsular lens                                                       opacity (cataract)

Gave me more pleasure than any thunderous and blazing crackers and the sweetest sweets of this world.-------

Yesterday on the day of Diwali ,door bell ranged quite early.My son anmol opened the door.He immediately recognized the cobbler’s son and gave him shoes to polish. I saw a 6-7yrs old child standing in front of me holding the shoes which my son had just given.I got appalled ,he was a tender boy Something flashed in my mind .The boy was holding the shoes to polish and thereby earn some money on the day of LAXMIPUJA.

Many questions rushed to mind and become bewildered.That child was not old enough to understand what I wanted to say .A sense of guilt prevailed in my mind but I was puzzled –today being the day of LAXMIPUJA.What to do?

Suddenly ,I took the shoes from his hand and gave him some rupees to cleberate the Diwali.In the evening I worshipped Laxmi but my worship was done early in the morning---and that gave me more pleasure than any thunderous and blazing crackers and the sweetest sweets of this world.

HAPPY DIPAWALI TO ALL

PANDA IN BRAIN

514.All are true regarding neuroimaging of Wilson disease except

a. “face of the giant panda” sign in medulla

b. the “bright claustral” sign

c. Diffusion-weighted MR shows variable findings,

d. significant decrease in the NAA/Cr ratio in the parietal-occipital cortex, frontal white matter, and basal ganglia

e. may improve and/or resolve after effective treatment

514.---a

www.doctorshangout.com

Two specific MRI signs have been described in WD. The better known is the “face of the giant panda” sign , which describes hyperintense signal on long-TR sequences throughout the midbrain with sparing of the red nucleus, the lateral portion of the pars reticulata of the substantia nigra, and a portion of the superior colliculus.

www.japi.org

The other sign, the “bright claustral” sign, correlates with focal hyperintense signal of the claustrum on long-TR sequences . In a recent investigation, the “face of the giant panda” sign was found in 12% and the “bright claustral” sign in 4% of patients with WD.

WHAT IS AMYLOID IMAGING

• The major pathologic features that characterize AD are senile plaques, neurofibrillary tangles, decreased synaptic density, neuron loss, and cerebral atrophy
•  Neurofibrillary tangles consist of pathologic aggregates of tau protein (115). These pathologic aggregates form in the presence of hyperphosphorylation of the tau protein
• The second pathologic lesion associated with AD is the senile (neuritic) plaque. A neuritic plaque consists of a dense central β-amyloid core with inflammatory cells and dystrophic neurites in its periphery. The β-amyloid core is made up of fragments of the amyloid precursor protein that have aggregated into β-pleated sheets in the extracellular space.
•  The ability to identify deposits of abnormal protein (amyloid plaque), which is a cardinal pathologic feature of the disease in living patients, has the ability to revolutionize patient diagnosis and management of AD
• Amyloid imaging is related to imaging of amyloid plaque.
• A large number of  ligands have been synthesized and evaluated as candidates for amyloid imaging agents. 
• These can be classified into six categories of derivatives: Congo-red, Thioflavine T, stilbene, vinylbenzoxazole, DDNP, and miscellaneous.
•  The compound that has been most extensively studied is Pittsburgh Compound B, commonly referred to as PIB .
• PET imaging tracers approved by FDA for amyloid imaging are , florbetaben F18 injection (Neuraceq, Piramal Imaging).,florbetapir (Amyvid, Eli Lilly and Company)  and flutemetamol (Vizamyl, GE Healthcare),

AD POSITIVE CASE

NORMAL CASE

 

REF ---http://www.diagnosticimaging.com/articles/amyloid-imaging-next-frontier-alzheimer%E2%80%99s-care

WHAT IS STATUS CRIBOSUS

Q.All are true regarding space of Virchow-Robin (VRS) except

a. isointense to CSF on all pulse sequences

b. type 2 lacunae

c. lack mass effect

d. round, oval, or curvilinear with well-defined, smooth margins

e.common in relation to anterior commissure

ANS---b

www.ajnr.org

archneur.jamanetwork.com

• Perivascular space of Virchow-Robin (VRS) is an extension of the subarachnoid space that accompanies penetrating vessels into the brain to the level of the capillaries. 
• The VRS at the base of the brain follow the lenticulostriate arteries as they enter the basal ganglia through the anterior perforated substance. On axial images they are typically adjacent to the anterior or posterior surface of the lateral portion of the anterior commissure . In the coronal or sagittal plane they are adjacent to the superior surface of the commissure or just lateral to the putamen.
• Those in the high convexity  follow the course of the penetrating cortical arteries and arterioles from the high-convexity gray matter into the centrum semiovale.
• High signal intensity (i.e., higher intensity than CSF, most notably on proton density–weighted or FLAIR images) foci in the midbrain  can be seen from enlarged perivascular spaces(along  branches of the collicular and accessory collicular arteries)
• Small VR spaces (less than 2 mm) are found in all age groups and probably represent a normal anatomic finding . With advancing age, VR spaces are found with increasing frequency and larger apparent size .
• In one report, lenticulostriate VR spaces had a mild correlation with age, whereas high-convexity VR spaces, although more rare, had a much stronger correlation with age .
• Age, hypertension, dementia, and incidental subcortical white matter lesions were significantly associated with large (greater than 2 mm) VR spaces.
• Migraine patients  have been reported to show prominent perivascular spaces.
• Distinction between dilated perivascular spaces and lacunar infarction is a common problem on clinical MR images. Three criteria must be assessed by the radiologist in these cases: location, morphology, and signal intensity.
• Generally useful guidelines are that lacunar infarctions often are larger than 5 mm, are not symmetric, are located in the upper two thirds of the putamen, and are not isointense to CSF on all imaging sequences
• . Conversely, dilated perivascular spaces usually are isointense to CSF on all pulse sequences, bilaterally symmetric, less than 5 mm in diameter, and located in the inferior one third of the putamen .Size is certainly the weakest discriminator of those mentioned.

New pathologic classification of cerebral lacunae

Type Pathologic criteria 1 Old, small, deep cerebral infarcts with irregular cavities containing macrophages and parenchymal fragments surrounded by gliosis 2 Old, small hemorrhages with hemosiderin-laden macrophages and iron pigmentation of their walls 3 Dilated perivascular spaces; they are round, very regular cavities that always contain one or two sections of an artery with a patent lumen and usually normal walls: the cavity is lined by a single layer of epithelial cells that correspond to the leptomeningeal cells forming the normal lining of the perivacular spaces; according to their number and size, four varieties of types 3 exist. 3a Numerous small, round perivascular spaces (état criblé or status cribosus) 3b Perivascular dilation destroying the adjacent brain (lacunes de désintégration, vaginalite destructive) 3c Solitary subputaminal cavities surrounding the lenticulostriate arteries at their entrance into the lentiform nucleus 3d Expanding perivascular spaces that cause mass effect with possible reactive lesions such as gliosis, spongiosis, swollen oligodendroglia, and myelin loss with edema REF Magnetic Resonance Imaging of the Brain and Spine, 4th Edition,Atlas, Scott W.