 So, hi everyone, I am Ishwar Kumar, final year resident, Divya Patil Hospital, Kolhapur. And I would like to thank IRIA for giving me this opportunity to present at 20th MRA conference. And I would like to thank my mentors, Dr. Nitin Badwani sir, who is a professor at HOD and Mahesh sir, who is senior resident for great teachers and who are my guides for this paper making. So my topic is Fecomatosis and I will be presenting some typical, atypical, common and not so common cases which presented to our department. So Fecomatosis are neurocatenase syndromes, are heterogeneous group of disorders and have variable inheritance pattern and as of now more than 30 entities are included. So we will be discussing some of the uncommon associations and the typical findings of most common neurocatenase syndromes through some cases. And also we will be discussing a rare case of incontinentia pigmenti. So all these cases are done on 3,000 MRA machine in our department. And the most common neurocatenase syndromes described in literature are tuberosulatosis NF1 and NF2. So if we consider NF1, the plexiform neurofibromas and bilateral optic gliomas are almost pathognomic for the diagnosis of NF1. And similarly, bilateral acoustic schwannomas are pathognomic for NF2 and are cortical tuber subepandemol hematomas, cutaneous angiofibromas and segas for tuberosulatosis. And there are several rare Fecomatosis with CNS manifestations like incontinentia pigmenti which require exclusively clinical or histological diagnosis. So to start with cases, this is the first case 20-year-old female patient presented with long-term back pain, upper and lower limb pain. So this is a typical but not so common case of NF1 with extensive subcutaneous and deep plexiform neurofibromas with symmetrical whole body involvement, which is pathognomic for NF1. So these are transverse images and here we are seeing the lobilated T2 hyperintense lesion along the supraorbital nerve and inside the lesion we are seeing areas of hypotenesity. So these are plexiform neurofibromas along supraorbital nerve and also we are seeing diffusely involved musculoskeletal planes at the level of skull base with neurofibromas. This is the coronal image we are seeing plexiform neurofibromas along the brachial plexus and also in the subcutaneous plane of the neck and also plexiform neurofibromas involving the lumbosacral plexus and also in the abdominal wall and also the upper limbs. And this is another case, this is a long-year-old boy with seizures, vomiting, headache, burying of his young clinically, he had axillary KFLA spots. So this is an uncommon diffusely infiltrative brainstem astrocytoma causing abstract to heterocephalitis and a typical bilateral staphelomas in a patient with axillary KFLA spots and neurofibromas type 1. And in this image we are seeing, these are flare images, we are seeing dilated ventricles with periventricular linear hyperintense signal, what we call it as periventricular interstitial edema or CSFC page and here we are seeing hyperintense signal within the telamy and the telamy are swollen as well. In the posterior part of the cerebral epiaduct of the midbrain we are seeing hyperintense signal on flare images and this is flare image at the level of pons, posterior pons is involved and also the middle cerebellar peduncle is involved bilaterally, more on the left side and the cerebellar white matter is also involved. The left inferior cerebellar peduncle is involved and also the dermis is involved and superior cerebellar peduncle and the cerebellum is showing facilitated diffusion on ADC map so similar restriction characteristics are seen even on the other legions as well. So here in this image, this is T2-way-to-image, we are seeing posterior bulging of the globe off-center to the optic disc, so this is stapheloma which are bilateral and this is in a typical association with only few reported cases with NF1 and the tachyl plate which is biconvex and swollen which is causing obstruction to the epiaduct with resultant obstructive heterocephalus and we are seeing the enlarged lateral ventricle and also dilated third ventricle with normal fourth ventricle. So this is in diffusely infiltrative brainstem astrocytoma which is an uncommon association of NF1. This is another case, 27-year-old male patient presented with imbalance, slur speech, progressive hearing loss, facial palsy, mouth deviation to the left side and giddiness. So this is a case of bilateral schwannomas with a typical enhancement and infiltrative characteristics in a young patient with NF2. So in this image, T2-way-to-image which is demonstrating predominately hyperintensilations in the bilateral CP angles at the level of MCP without distinct margins. So the lesion on the right side is more indistinct and the lesion on the left side is little bit distinct. We can make out the margins of this lesion better than the margins of this lesion. At a more higher level, again we are seeing mass effect of this lesion which is on the left side, mass effect on pons. This is an ADC map which is showing no restriction to the right-sided lesion, equivocal peripheral areas of low ADC values to the left-sided lesion. Again drive sequence at the level of pons. This right-sided lesion is more infiltrative and the left-sided lesion is more distinct and the margins are little bit clear than the right-sided lesion and also we can see the extension of this lesion into the IAC here we are seeing the hypotenuse extension into the IAC and also the extension into the IAC on the right side as well and this lesion in the craniocaudal plane or the coronal plane is centered at the pontometallary junction. So post-contrast images on T1, pre-contrast image here we are seeing laterally, so to high-point tense lesions on the left side a little bit of hemorrhagic area, post-contrast image on the right lesion which is not showing enhancement with only a enhancing foci in the center of the lesion and also the IAC component is not showing any enhancement counter to that on the left side the lesion is showing peripheral areas of enhancement and also the component of in the IAC is enhancing so this is an atypical case of NF2, so this is a 30-year-old female patient presented with seizures, so typical but subtle case of tuberous sclerosis. Here we are seeing on flare images and T2-V2D images, high-point tense triangular shaped signal in the cortex and the subcortical area of the right parietal lobe and this is suggestive, this can be suggestive of a cortical tuber or it can be a focal cortical dysplasia unless we have an associated finding of tuberous sclerosis we cannot for sure call this as cortical tuber because it is isolated and and also in this case if we see carefully we are seeing these subparmenomal hematomas and together now these two are diagnostic of tuberous sclerosis. If we miss this subtle subparmenomal hematomas we cannot make the diagnosis of tuberous sclerosis in this case. So this is another case to a rolled shale presented with seizures and this is a typical and obvious case of tuberous sclerosis and this is T2-V2D coronal image, multiple cortical and subcortical areas of hyperintensities, cortical tubers and linear hyperintense radiating bands. Here we are seeing the radiating bands from periventricular to subcortical white matter and the transverse T2-V2D image showing the subparmenomal nodules and these nodules are hyperintense on T2 which is owing to their calcifications and which is evident on ADC map as well. ADC is a sherry based map and hyperintensity in the ADC can also be due to calcification other than restriction. So another case 50 year old patient presented with progressive headache on clinical examination patient had nasolabial angiofibromas. So this is in a typical case of tuberous sclerosis in a patient with adenoma, sebaceous and an isolated brain finding of SEGA. So transverse image, coronal image, T2-V2D images and we are seeing this heterogeneous intraventricular mass which is centered in the left lateral ventricle which is abutting the septum pellucidum and also the appendema. So this lesion is extending directly into the left forearm of Munro and compressing the outlet of right forearm of Munro which is resulting in obstructive heterocephalus and that is also this periventricular interstitial edema more on the frontal side. In this coronal image we are seeing the third ventricle which is collapsed and the dilated temporal horns of the lateral ventricle. In the GRE image we are seeing this normal appendimal vein and the lesion does not show any blooming and on T1 weighted pre-contrast image. The lesion is iso-to-hypoimptons which demonstrated intense almost homogenous enhancement on Gerlindem enhanced post-contrast T1 based image and maybe a little bit of non-enhancing areas otherwise it is homogeneously enhancing. And as we know the clinical history so the diagnosis of tuberous sclerosis is made and this is a SEGA sub appendimal gene cell astroceatoma. So if you don't know the clinical finding of adenoma sebaceous for this patient the diagnosis would have been a centerneroceatoma than a SEGA. So this is another case this is a rare case of incontinence of pigment with vasculitis associated brain infarction in a full-term neonate. This is a three-day old full-term female baby presented with multiple erythematous papules and plaques on bilateral upper limbs, lower limbs and axilla. The baby white was 3 kg at birth, active in ephembrale. She had an episode of generalized tonic-clonic seizures and echocardiography was reported as normal. So here we are seeing Q2 weighted transverse image, heterogeneous signal in the bilateral corona radiata and posterior limb of the internal capsule and also there are halter signal densities in the subcortical white matter as well. So this is an TWI image and ADC maps and here we are seeing multiple foci of diffusion restriction which are diffusely involving the bilateral cerebral hemispheres which is more prominent on the right side and these does not follow any vascular territory and the corpus callosum is also involved. The cerebellum is also involved and also there is complete involvement of the right thalamus and focal areas of involvement of the left thalamus. And here we images areas of blooming involving bilateral cerebral hemispheres and also in the right cerebellar hemisphere and all these findings together find favor CNS manifestation of vasculitis associated hemorrhagic infarct in a newborn with incontinence apigmenti. And concluding my paper the MRM plays an important role leading in the diagnosis or diagnosing this heterogeneous group of neurogradient disorders especially with advent of higher thalamus magnets and newer sequences. So if we see in the case of C3 although the site bilaterality and spread strongly suggested chifanomas, infiltrative nature and a typical enhancement pattern raise diagnostic challenge. In C4 differentiating the isolated cortical tubal from FCD would have been difficult if not for the subtle subabendamel nodules and in C5 where there is an isolated brain finding of SEGA the diagnosis of central neurocytoma may have been favored if the patient had not been clinically examined for nasolabial and G5 bromas. In the case of C7 multiple random hemorrhagic infarcts in brain favored vasculitis as a cause of encephalitis. In several previous studies the histologic and neuro radiologic observations have speculated that vasculatiology causing ischemia or an abnormal protein triggering and encephalitic process may be responsible for neurologic complications of incontinence apigmenti and in C2 infiltrative astrocytoma, bilateral cephaloma surround common associations of NF1. So the radiology should be aware of clinical future, satirical appearances and subtle findings to be able to make accurate diagnosis. So these are my references and thank you very much for your patience.