Introduction: Jarcho-Levin syndrome is a genetic skeletal disorder characterized by short-trunk dwarfism with vertebral and rib anomalies. The association with other organs such as the spinal, brain, and the urinary system has been reported. The paper reports a rare case of Jarcho-Levin syndrome associated with neural tube defects and other congenital malformations.

Case presentation: The patient came with neglected myelomeningocele and severe hydrocephalus. Type 3 growing skull fracture complicated the management of this syndrome. This paper describes the management of JLS associated with neural tube defects as well as the severe hydrocephalus and the growing skull fracture.

Conclusion: The paper reports a rare case of Jarcho-Levin syndrome associated with neural tube defects and other congenital malformations. The treatment should be carefully tailored.

 Keywords: Growing skull fracture, hydrocephalus, Jarcho-Levin syndrome, myelomeningocele, spondylocostal dysostosis

Introduction: Phaeohyphomycosis is an infection caused by dematiaceous or pigmented filamentous fungi, which contain melanin in their cell walls. These fungal organisms are neurotropic and are reported to cause cerebral abscesses regardless of the immune status of the patient. The objective of this case report is to emphasize the importance of distinguishing cerebral phaeohyphomycosis from high grade glioma through sufficient microbiological and histopathological studies in order to provide early diagnosis and adequate antifungal therapy if and when cerebral phaeohyphomycosis is diagnosed.

Case presentation: This case report describes a 23-year-old male from South East Asia presenting with memory disturbance and cognitive decline with significant improvement in presenting features post-operatively. We illustrate the radiological and clinical similarities between high-grade glioma and fungal brain abscess caused by Cladophialophora bantiana. We also discuss the microbiological and histopathological features.

Conclusion: The importance of performing microbiological and histopathological testing of the specimen prior to starting the patient on antifungals is emphasized.

Keywords: brain abscess, cerebral phaeohyphomycosis, cladophialophora bantiana, fungal infection

Introduction: Spontaneous spinal epidural hematoma (SSEH) is a relatively uncommon pathology of the spine. The incidence of SSEH is approximately 0.1 – 1 per 100,000 individuals. SSEH can cause acute spinal cord compression and rapid onset of neurologic deficits. Although rare, SSEH must be considered in the differential diagnosis of patients presenting with sudden onset of neurologic symptoms without a history of trauma.

Case presentation: We report a case of a 19-year-old female patient who presented with sudden onset of weakness of all four limbs with pain in the upper cervical region. She has no history of trauma. Magnetic Resonance Imaging (MRI) of the spine was done, which showed an epidural mass in the cervical region, causing severe cord compression. Emergency cervical laminectomy and evacuation of epidural hematoma has was done with excellent post-operative recovery.

Conclusion: Spontaneous spinal epidural hematoma is a relatively rare entity of the spine. The signs and symptoms of this rare condition are non-specific. In patients presenting with a sudden onset of neurologic deficits, the condition should be one of the differential diagnoses. MRI is the investigation of choice in patients suspected to have SSEH. Early surgical intervention results in a better outcome. The level of preoperative neurologic deficit, the severity of the condition, and operative interval are important factors affecting the postoperative outcome.

Keywords: cervical spine, laminectomy, spontaneous spinal epidural hematoma

Introduction: Inflammatory myofibroblastic tumor (IMT), a subtype of inflammatory pseudotumor, is a rare cause of non-neoplastic sellar mass. IMTs have been reported in various anatomical locations of the body, but their presence in the Central Nervous System (CNS), especially in the pituitary gland, is rare. The objective of our case report is to analyze the basis of their mimicry, discuss their differential diagnosis, and review the literature related to inflammatory pseudotumors of the pituitary.

Case presentation: A 45-year-old male presented in our hospital with chief complaints of severe headache since two months ago and blurring of vision since two weeks ago. On investigation, Magnetic Resonance Imaging (MRI) revealed a pituitary macroadenoma, which was compressing the optic chiasma and encasing the left internal carotid artery. The patient underwent endonasal endoscopic transsphenoidal excision of the pituitary lesion and became asymptomatic in the postoperative period. Histopathology reported the presence of spindle-shaped cells, inflammatory cells, respiratory epithelium lined tissue, and areas of necrosis suggestive of an inflammatory myofibroblastic tumor.

Conclusion: In the following communication, we discuss this esoteric pseudotumor of the pituitary and its peculiarities in diagnosis and management as it can easily disguise itself as a pituitary macroadenoma on radiological investigations, thus hoodwinking the physician of its actual pathology. We also review the literature to perceive the best management protocols and prognostic factors by drawing parallels with IMT in other parts of the CNS. The differential diagnosis of inflammatory myofibroblastic tumors of the CNS is difficult and relies mainly on histological analysis.

Keywords: Inflammatory myofibroblastic tumor, pituitary neoplasm, plasma cell granuloma, pseudotumor

Background: When two or more adjacent intracranial aneurysms arises from same or different arteries with separate origins and partially adherent walls is referred as “Kissing aneurysm”. These are rare aneurysms¹. Among different locations of kissing aneurysms reported in the literature, kissing aneurysms of bilateral distal anterior cerebral arteries (DACA) - two different aneurysms situated symmetrically on opposite DACA are very rare.

Case Description: 69 years old lady with multiple comorbidities was admitted with anterior inter-hemispheric bleed, Magnetic Resonance Angiography (MRA) and Digital Subtraction Angiography (DSA) showed ruptured bilateral DACA aneurysms in mirror position to each other forming a Mickey Mouse head appearance. Patient was managed surgically by craniotomy and clipping of both aneurysms.

Conclusion: Kissing aneurysms of bilateral DACA aneurysms are very rare. DSA is essential to be useful for preoperative diagnosis and planning. Basic principle of securing the parent artery proximally and clipping the neck after meticulous dissection should be followed.

Key words: Kissing aneurysm, distal anterior cerebral artery aneurysm, Digital Subtraction Angiography.