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Frontiers in Neuroscience 2023Xanthogranulomas are considered rare tumors, with their sellar and non-sellar frequency ranging from 1.6 to 7% among intracranial lesions, and described as a separate...
Xanthogranulomas are considered rare tumors, with their sellar and non-sellar frequency ranging from 1.6 to 7% among intracranial lesions, and described as a separate entity by the World Health Organization in 2000. The diagnosis of sellar xanthogranulomas is challenging, given their uncertain origin and clinical course. In addition, the limited reporting of sellar xanthogranuloma cases and the absence of characteristic images make these entities difficult to distinguish from other cystic lesions of the sellar region, such as adamantinomatous craniopharyngiomas, Rathke's cleft cysts, pituitary tumors, arachnoid cysts, epidermoid cysts, and dermoid cysts. Here, we describe the clinical presentation, radiological findings, immunohistochemical/histopathological analysis, and the ultrastructural examination by transmission electron microscopy of five sellar xanthogranulomas cases reported in two care centers in Cordoba, Argentina. Two males and three females between 37 and 73 years of age (average 51.8 years) presented with persistent headaches, generalized endocrine defects, and visual problems. MRI revealed cystic formations in the sellar region, which usually projected into adjacent tissues such as the suprasellar region or cavernous sinuses, and compressed other structures such as the optic chiasm, pituitary gland, and cranial nerves. All patients underwent surgical intervention to remove the tumor tissue. The histopathological analysis of the samples showed cellular tissue with a xanthogranulomatous appearance, inflammatory cellular infiltrate (mainly lymphocytes and macrophages), fibroblasts, abundant collagen fibers, and hemorrhages. An ultrastructural analysis helped to identify cellular infiltrates and granules resulting from tumor cell activity. The data support the hypothesis that sellar xanthogranulomas could occur as an inflammatory reaction secondary to the rupture and hemorrhage of a previous cystic process, thereby generating an expansion of the tumor body toward adjacent tissues. The information obtained from these cases contributes to the current knowledge about this disease's origin and clinical and histological evolution. However, the scarcity of patients and the observed phenotypic heterogeneity make its diagnosis still challenging. Undoubtedly, more investigations are needed to provide additional information in order to be able to achieve a more accurate diagnosis and effective treatment of this rare disease.
PubMed: 37811322
DOI: 10.3389/fnins.2023.1227144 -
Surgical Neurology International 2023Neuroglial cysts (NCs) are uncommon benign cysts covered by an epithelial layer, accounting for <1% of all intracranial cysts. The optimal management approach for these...
BACKGROUND
Neuroglial cysts (NCs) are uncommon benign cysts covered by an epithelial layer, accounting for <1% of all intracranial cysts. The optimal management approach for these cysts remains a subject of debate. Given their rarity, management principles used for arachnoid cysts can be applied to NCs.
CASE DESCRIPTION
We present a case of a 35-year-old male without prior medical history, who presented to the neurosurgery clinic with complaints of absence seizures. A neurological examination revealed subtle weakness in the left upper limb. Brain magnetic resonance imaging demonstrated a large cystic lesion in the posterior frontal and anterior parietal lobes of the right hemisphere. The patient underwent an awake craniotomy, during which a cystoventricular fenestration was performed on the motor cortex cyst. Histopathological examination confirmed the diagnosis of NC. At the 4-month follow-up, the patient experienced complete recovery, with normal strength (5/5) in all limbs and absence of seizure remission.
CONCLUSION
This case highlights the successful use of awake craniotomy for the fenestration of an NC in the motor cortex. Given the rarity of NCs, there is no consensus on the optimal treatment strategy. However, in this particular case, the patient achieved complete recovery without any new neurological deficits following the procedure.
PubMed: 37810319
DOI: 10.25259/SNI_477_2023 -
Journal of Neurosurgery. Case Lessons Oct 2023Intracranial epidermoid cysts (ECs) are rare benign lesions of ectodermal origin that can be found in the cerebellopontine angle (CPA). If large enough, they compress...
BACKGROUND
Intracranial epidermoid cysts (ECs) are rare benign lesions of ectodermal origin that can be found in the cerebellopontine angle (CPA). If large enough, they compress surrounding structures, causing cranial neuropathies, cerebellar dysfunction, and hydrocephalus.
OBSERVATIONS
In this case report, the authors present a patient with headaches and diplopia secondary to a CPA EC. The cyst had wrapped itself around and was strangulating the abducens nerve. This choking of the nerve was believed to have been the cause of the patient's double vision. The arachnoid band was released with a myringotomy knife.
LESSONS
Intracranial ECs typically cause symptoms from mass effect on surrounding structures; it is unusual for one to have choked off the nerve in the fashion presented in this case. Knowledge of the potential ways that ECs can affect nervous structures is critical for resecting them without injuring normal tissue.
PubMed: 37782964
DOI: 10.3171/CASE23383 -
Frontiers in Neurology 2023Symptoms induced by arachnoid cysts in the fallopian canal are uncommon, and facial nerve paralysis without cerebrospinal fluid otorrhea is comparatively rarer.
INTRODUCTION
Symptoms induced by arachnoid cysts in the fallopian canal are uncommon, and facial nerve paralysis without cerebrospinal fluid otorrhea is comparatively rarer.
METHODS
Herein, we present two cases of arachnoid cysts in the fallopian canal with acute severe facial nerve paralysis and review the relevant literature.
RESULTS
The symptoms and imaging findings of these two cases resembled those of facial nerve schwannomas. Cerebrospinal fluid otorrhea occurred upon removal of the arachnoid cyst, and the facial nerve was observed to be separated into multiple filaments or compressed and atrophied. Facial-hypoglossal nerve anastomosis and decompression were conducted after packing the dehiscence of cerebrospinal fluid otorrhea for the two cases.
CONCLUSION
Arachnoid cysts of the fallopian canal rarely cause facial nerve paralysis. Enhanced magnetic resonance imaging is vital for differentiating schwannomas. Different treatment strategies should be adopted for patients with different degrees of facial nerve paralysis; however, concurrent repair of cerebrospinal fluid otorrhea and facial nerves during surgery can occasionally be challenging.
PubMed: 37745664
DOI: 10.3389/fneur.2023.1226404 -
Revista Paulista de Pediatria : Orgao... 2023To report a rare case of a patient with a molecular diagnosis of Kleefstra syndrome (KS) who has four other chromosomal alterations involving pathogenic variants.
OBJECTIVE
To report a rare case of a patient with a molecular diagnosis of Kleefstra syndrome (KS) who has four other chromosomal alterations involving pathogenic variants.
CASE DESCRIPTION
Male patient, two years old, with global delay, including in neuropsychomotor development, ocular hypertelorism, broad forehead, brachycephaly, hypotonia, ligament laxity, unilateral single palmar crease and arachnoid cyst. The microarray-based comparative genomic hybridization (a-CGH) identified copy number variations (CNVs) in five regions: 9q34.3, 6p22.1, Yq11.223, Yp11.23, and 2q24.1. The heterozygous microdeletion in 9q34.3 involving the EHMT1 gene confirms the diagnosis of KS.
COMMENTS
The presence of pathogenic CNVs and/or those of uncertain significance, located on chromosomes 2, 6 and Y, may be contributing to a variability in the patient's clinical condition (arachnoid cyst, single palmar fold and ligament laxity), compared to other individuals with only KS genetic alteration, making the dignosis of the disease harder.
Topics: Male; Humans; Child, Preschool; DNA Copy Number Variations; Comparative Genomic Hybridization; Heterozygote; Cysts
PubMed: 37729241
DOI: 10.1590/1984-0462/2024/42/2022230 -
Journal of Surgical Case Reports Sep 2023Spinal extradural arachnoid cysts are rare benign lesions occurring along the cerebrospinal axis. They may be associated with pain or varying degrees of neurological...
Spinal extradural arachnoid cysts are rare benign lesions occurring along the cerebrospinal axis. They may be associated with pain or varying degrees of neurological compressive symptoms. Brown-Sequard syndrome is a rare sequalae, where there is ipsilateral upper motor neuron paralysis with loss of proprioception as well as contralateral loss of pain and temperature sensation below the lesion. We present a 33-year-old female with a 6-month history of worsening right lower limb weakness and a 2-month history of right lower limb pain. Motor examination revealed right lower limb weakness as well as exaggerated knee and ankle jerk reflexes. A magnetic resonance imaging (MRI) was done, which showed an eccentrically located T4-7 cystic extradural mass causing severe cord compression. She had T4-7 laminectomies with total excision of the cyst and disconnection of the fistulous tract between the cyst and the subarachnoid space. She made full neurologic recovery with no complications.
PubMed: 37727223
DOI: 10.1093/jscr/rjad514 -
Cureus Aug 2023The formation of an intradural extramedullary arachnoid cyst is a rare complication of spinal anesthesia. We present a case of a 34-year-old female patient who developed...
The formation of an intradural extramedullary arachnoid cyst is a rare complication of spinal anesthesia. We present a case of a 34-year-old female patient who developed neurological symptoms including a bilateral reduction in sensation and strength in the lower limbs following a C-section under spinal anesthesia. MRI of the thoracic spine revealed a lesion at the level of T11/T12 and the upper limit of the L1 vertebral body, which was pushing the cord to the side, and a diagnosis of the intradural extramedullary arachnoid cyst was established. The prognosis for this condition is good and surgical excision leads to resolution of the symptoms. Proper and prompt diagnosis is crucial to rule out other differentials and prevent permanent neurological damage in these patients.
PubMed: 37692609
DOI: 10.7759/cureus.43199 -
Journal of UOEH 2023Rheumatoid meningitis is a rare neurological manifestation that is difficult to diagnose. Here we report a 77-year-old woman who developed a left hemiparesis because of...
Rheumatoid meningitis is a rare neurological manifestation that is difficult to diagnose. Here we report a 77-year-old woman who developed a left hemiparesis because of an arachnoid cyst with rheumatoid meningitis. The patient had a 13 year history of rheumatoid arthritis, which had been successfully treated. She developed left hemiparesis and visited our hospital. MRI showed a high signal intensity lesion in the right frontal subarachnoid space and an arachnoid cyst on Fluid attenuated inversion recovery (FLAIR). A gadolinium enhanced T1 weighted image revealed associated abnormal enhancement. We removed the arachnoid cyst and diagnosed the patient as having rheumatoid meningitis. Her symptoms and MRI findings improved rapidly by methylprednisolone pulse therapy. Rheumatoid meningitis is rare, but a case of arachnoid cyst associated with rheumatoid meningitis was extremely rare. This is the first report of such a case.
Topics: Humans; Female; Aged; Hospitals; Meningitis; Cysts
PubMed: 37661390
DOI: 10.7888/juoeh.45.185 -
BMC Pediatrics Aug 2023Migraine and tension-type headache are common primary headaches in children. There is a risk of developing secondary headache in children. The current study was aimed to...
PURPOSE
Migraine and tension-type headache are common primary headaches in children. There is a risk of developing secondary headache in children. The current study was aimed to evaluate magnetic resonance imaging findings (MRI) in children with migraine or tension-type headache.
METHODS
The study was planned in children with migraine or tension-type headaches who have been followed up in the pediatric neurology outpatient clinic with regular office visits for at least two years and had neuroimaging in the last year.
RESULTS
280 patients (187 female patients) datas were studied. 91 (61 female patients) were followed up with the diagnosis of migraine and 189 (126 female patients) with the diagnosis of tension-type headaches. The age of patients was found to be 13.1 ± 3.4 years. Brain tumor was found in one child with tension-type headache who had papilledema. Incidental MRI findings found 7.7% and 12.7% in migraine and tension-type headache, respectively. MRI findings in the study were arachnoid cyst (14), pituitary adenoma (6), mega cisterna magna (6), pineal cyst (3), non-specific gliosis (2) and tumor (1).
CONCLUSION
Arachnoid cysts were found incidental as the most common MRI finding in children with migraine or tension-type headache. The rare life-threatening secondary headache may develop in children. The fundus examination as a complement to the neurological examination can be useful for requesting MRI.
Topics: Child; Humans; Female; Adolescent; Tension-Type Headache; Migraine Disorders; Headache; Magnetic Resonance Imaging; Neuroimaging; Brain Neoplasms
PubMed: 37649015
DOI: 10.1186/s12887-023-04264-y