Corner for Images

Case 1 A 5-year-old boy was brought in with complaints of right upper limb weakness for 3 years associated with abnormal positioning of the head noticed by the parents and deviation of the neck toward the left since 2 min after hearing loud noises, followed by unresponsiveness for 2 min. On motor examination, the child had contractures in the right ankle, hypertonia in the right upper limb, and right hemiplegic gait. Magnetic resonance imaging (MRI) brain in Figure 1a shows gliotic and cystic encephalomalacic changes in the left fronto-parieto-temporal lobe with white matter volume loss, Figure 1b shows ipsilateral calvarial thickening and asymmetric pneumatization of left frontal sinus, and Figure 1c shows hemiatrophy of the left cerebral peduncle with mild flattening of the left hemipons, suggestive of Wallerian degeneration.Figure 1: (a) Axial magnetic resonance imaging (MRI) image of the brain on T2 sequrence. The orange arrow indicates cystic encephalomalacic changes with left cerebral hemiatrophy. (b) Axial MRI image of the brain on T2 sequrence. The orange arrow indicates ipsilateral calvarial thickening. (c) Axial MRI image of the brain on T2 sequrence. The orange arrow indicates ipsilateral enlarged frontal sinus and hemiatrophy of the left cerebral peduncle suggesting Wallerian degenerationFinal diagnosis – Dyke-Davidoff-Masson syndrome with sequelae of prior vascular insult with resultant Wallerian degeneration. Dyke-Davidoff-Masson syndrome is a condition marked by hemicerebral atrophy, typically resulting from a brain injury during the fetal stage or early childhood.[1] Typical radiological features of the condition include cerebral hemiatrophy along with compensatory enlargement of the skull and paranasal sinuses on the affected side. While it is most frequently observed in adolescents and adults, cases have also been reported in children.[2-4] The clinical manifestations include seizures, intellectual disability, facial asymmetry, and contralateral hemiparesis. Cerebral hemiatrophy can be of two types, infantile (congenital) and acquired.[5] The infantile variety results from various etiologies such as infections, neonatal or gestational vascular occlusion involving the middle cerebral artery, unilateral cerebral arterial circulation anomalies, and coarctation of the midaortic arch. The patient becomes symptomatic in the perinatal period or infancy. The main causes of acquired type are trauma, tumor, infection, ischemia, hemorrhage, and prolonged febrile seizure.[5,6] Age of presentation depends on the time of insult, and characteristic changes may be seen only in adolescence or adult. Findings may include Wallerian degeneration of the mesencephalon and middle fossa hypoplasia, atrophy in the basal ganglia and in the brainstem, capillary malformations may be detected in some situations, calvarial thickening (affected side), hyperpneumatization of frontal sinus (also ethmoid sinuses and mastoid air cells). Case 2 A 46-year-old female presented with lower back ache and weakness in bilateral lower limbs for 6 months. MRI spine showed a well-defined T1 isointense/T2 hyperintense mass lesion involving D11, D12, and L1 vertebral bodies and IV disc levels in Figure 2a. In Figure 2b, the lesion shows heterogeneous contrast enhancement on postcontrast study with a thin T2 hypointense rim within the periphery of the lobulated margins, with few foci of swan blooming within the lesion. Figure 2c shows that the lesion is seen causing anterior scalloping into the vertebral body and in the pre and paravertebral regions.Figure 2: (a) Sagittal MRI spine on T2 sequence. The orange arrow indicates a T2 hyperintense mass lesion involving D11,D12 and L1 vertebral bodies. (b) Sagittal MRI spine on postcontrast sequence. The orange arrow indicates heterogenous contrast enhancement on post contrast study with a thin T2 hypointense rim within the periphery. (c) Axial MRI spine. The orange arrow anterior scalloping into the vertebral body in the pre and paravertebral regionsOn histopathological correlation, the findings were consistent with schwannoma. Final diagnosis – spinal schwannoma Schwannomas, less commonly called neurinomas or neurilemmomas, are benign tumors of Schwann cell origin and are the most common tumor of peripheral nerves, including cranial nerves. The vast majority of schwannomas are sporadic, with a peak presentation in the 5th to 6th decades. There is no sex predilection. When they occur in patients with neurofibromatosis type 2, schwannomas usually present by the 3rd decade. Schwannomas typically present as well-circumscribed masses that displace adjacent structures without evidence of direct invasion. Cystic and fatty degeneration are common findings. As the size of the tumor increases, the likelihood of internal heterogeneity also rises, often due to cystic degeneration or intratumoral hemorrhage.[7,8] Hemorrhagic changes are observed in approximately 5% of cases. Calcification, however, is a rare feature. The typical MR appearance shows iso- to hyperintensity relative to muscle on T1-weighted images, appears hyperintense on fluid-sensitive sequences, and frequently demonstrates diffuse enhancement on contrast-enhanced images.[9] Case 3 A 54-year-old-female presented with intermittent headache for 5 months. MRI brain shows an ill-defined area of T2 signal intensity with no diffusion restriction in the right frontal and parietal regions in Figure 3a and bilateral temporal lobes in Figure 3b.Figure 3: (a) Axial magnetic resonance imaging (MRI) image of the brain on T2 sequence. The orange arrow indicates an ill-defined area of T2 signal intensity in the right frontal and parietal lobes. (b) Axial MRI image of the brain on T2 sequrence. The orange arrow indicates ill defined area of T2 signal intensity in the bilateral temporal lobesOn histopathological and Immunohistochemistry (IHC) correlation , Isocitrate Dehydrogenase-1 (IDH-1) was negative in tumor cells and hence, the diagnosis was Glioblastoma IDH wild type. Final diagnosis – gliomatosis cerebri Gliomatosis cerebri is defined as a glioma with extensive infiltration affecting three or more cerebral lobes, often extending into infratentorial areas.[10] Although gliomatosis cerebri was removed from the 2016 WHO classification and reclassified under various diffuse glioma subtypes, the term remains widely used in clinical practice today.[11] Gliomatosis cerebri can be divided into two types. Type 1in which there is no discrete mass which is usually IDH wild-type and type 2 in which there is a discrete mass with further diffuse CNS involvement and IDH mutation is more common in this subtype.[12] Gliomatosis cerebri growth pattern can be seen in all diffuse gliomas of any grade. Given the challenges in detecting and managing gliomatosis, multiple imaging modalities are often employed to achieve an accurate diagnosis, guide treatment planning, and monitor disease progression. Advances in MRI – including MR spectroscopy, fluid-attenuated inversion recovery sequences, and diffusion-weighted imaging – have enhanced the detection of infiltrative gliomas that do not exhibit contrast enhancement. In addition, positron emission tomography (PET) using 18F-fluorodeoxyglucose-PET serves as a complementary imaging tool.[13] Because of the extensive involvement of the brain, complete surgical resection of these tumors is extremely challenging or not feasible. As a result, treatment typically depends on a combination of chemotherapy and radiotherapy, with the standard approach being radiotherapy administered alongside temozolomide.[14] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.