A Rare Case of Acute Necrotizing Encephalopathy of Childhood: A Case Report.

Acute necrotizing encephalopathy of childhood (ANEC) is a severe neurological disorder characterized by rapid-onset encephalopathy, often associated with viral infections. Acute necrotizing encephalopathy of childhood is associated with a very high mortality rate, and survivors may face long-term neurological sequelae. Acute necrotizing encephalopathy of childhood needs to be differentiated from its closest differential diagnosis, acute disseminated encephalomyelitis (ADEM). Most of the patients with ADEM recover, with a few of them having residual neurological deficits. We present a case of an eight-year-old boy with an acute history of fever, febrile seizures, and drowsiness. Magnetic resonance imaging revealed a symmetric tricolor appearance of bilateral thalamic lesions, characteristic of ANEC.

An Unusual Case of Ehlers-Danlos Syndrome Presenting as Proptosis.

Ehlers-Danlos syndrome (EDS) is a rare, heterogeneous group of genetic connective tissue disorders that affect collagen proteins. Currently, they are classified into 13 subtypes, many of which share general characteristics such as thin, hyperextensible skin and joint hypermobility. Vascular Ehlers-Danlos syndrome (vEDS) is characterized by tissue fragility, which predisposes individuals to premature arterial, uterine, or intestinal rupture. In this case, a young female presented with proptosis, skin hyperelasticity, and multiple joint dislocations. On computed tomography angiography (CTA), a direct caroticocavernous fistula, along with multiple segments of narrowing and ectasia in the internal carotid arteries and vertebral arteries, were detected, leading to a diagnosis of vEDS. This case report highlights the importance of clinical evaluation and the role of imaging in detecting this rare condition.

Cervical Spine Magnetic Resonance Imaging Findings in Hirayama Disease.

Background Hirayama disease is an uncommon type of cervical cord myelopathy seen typically in young males due to trauma from flexion movements. This study aims to assess the clinical presentations and classify the extent of various cervical spine MRI findings for the local population. Methodology A retrospective study of 13 patients diagnosed with Hirayama disease on cervical MRI was performed from January 2017 to December 2022 at Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune. Results Of the 13 patients, 12 (92%) were male, and one (8%) was female. Nine (69%) patients were in the 16-25-year age group, two (15%) were in the 26-35-year age group, and one (8%) each was in the 6-15-year and 66-75-year age groups. Upper limb weakness was the most common clinical symptom seen in 12 (92%) patients, followed by distal muscle atrophy in seven (54%) patients. Tremors in the hand were a rare symptom seen in two patients. Claw hand was an atypical symptom seen in one patient. On cervical MRI, all patients showed excessive forward shifting of the posterior dura on flexion, with resultant cord compression due to tightness of the dural sac. One (8%) patient had no signs of myelopathy, while 12 (92%) patients had developed chronic myelomalacia and showed abnormal cord hyperintensity and atrophy in the lower cervical cord. All 13 (100%) patients showed increased laminodural space on flexion; the mean thickness was 4.08 mm, with the minimum and maximum thickness being 2.4 mm and 6.7 mm, respectively. Classifying by length of the anterior bulging dura, one (8%) patient showed involvement of less than two vertebral body segments, eight (62%) patients showed involvement of two to four vertebral body segments, and four (30%) patients showed involvement of more than four vertebral body segments. Crescent-shaped post-contrast enhancement on flexion was seen in all eight (100%) patients who underwent a contrast study. Prominent epidural flow voids on flexion were seen in six (46%) patients. Conclusions Hirayama disease is an uncommon type of cervical myelopathy seen typically in juvenile males. The occult onset of distal upper limb weakness and atrophy during puberty, typical MRI features of lower cervical cord atrophy, and the presence of a crescent-shaped enhancing mass in the posterior epidural space are pathognomonic of the condition. A few atypical cases can also occur. Early diagnosis and treatment are crucial to avoiding serious dysfunction.

Rare Occurrence of Double Outlet Right Ventricle and Cardiomyopathy on Dynamic Contrast-Enhanced Cardiac MRI: A Case Report.

We present a case of a middle-aged man with recent onset of vague chest pain. On dynamic contrast-enhanced cardiac magnetic resonance (CMR) imaging, he was found to have a double outlet right ventricle (DORV) and restrictive/infiltrative cardiomyopathy. These two conditions are not known associations and could be two entirely different entities.

Neuroimaging Spectrum of Intracranial Lipomas.

BACKGROUND: Intracranial lipomas are rare congenital malformations at characteristic sites. Though largely asymptomatic, some cause significant morbidity. We are studying this for the local population.  Objectives: The purpose of this article is to analyze the spectrum of intracranial lipomas, determine their distribution at characteristic locations, and assess their associations.  Method: A retrospective study of 21 patients diagnosed with intracranial lipomas detected on CT and MRI performed between September 2017 and May 2022 at Dr. D.Y. Patil Medical College, Hospital & Research Center, Pune. RESULTS: Amongst the 21 patients with intracranial lipomas, the most common location was the pericallosal region seen in 11 patients (n = 11, 52.3%), of which nine were curvilinear type (n = 9), more common than tubular nodular type (n = 2). Other locations (n = 10, 47.7%) were found to be; quadrigeminal cistern in six patients (28.5%), cranial diploic space in two patients (9.5%), one each in interhemispheric falx (4.8%), and cribriform plate (4.8%). Associated anomalies were observed in three patients, which were fronto-ethmoidal encephalocele (n = 1), partial agenesis of the corpus callosum (n = 2), extension with a frontal subcutaneous lipoma (n = 1), and bilateral intraventricular extension (n = 1). Presenting symptoms were headache and dizziness (38.1%), seizures (19%), swelling on the forehead (9.5%), and other non-specific clinical symptoms (33.3%). CONCLUSION: Intracranial lipomas are rare congenital lesions that are usually asymptomatic and discovered incidentally. However, lipomas at interhemispheric locations can cause seizure disorders and some present with headaches and dizziness. Few have associations like communication with extracranial swellings and corpus callosum agenesis.