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Cureus Jan 2024We present a case of an adult patient experiencing progressive visual loss. An initial presentation was concerning for neuromyelitis optica with optic chiasm...
We present a case of an adult patient experiencing progressive visual loss. An initial presentation was concerning for neuromyelitis optica with optic chiasm involvement. However, persistent contrast enhancement observed in follow-up brain and orbit images raised suspicion for optic tract malignant neoplasm. Histopathological evolution of optic nerve biopsy confirmed the diagnosis of an optic chiasm glioma. The patient was then referred to oncology for chemotherapy.
PubMed: 38313959
DOI: 10.7759/cureus.51614 -
The Journal of Clinical Endocrinology... Jan 2024Outcome of craniopharyngioma is related to its locoregional extension, which impacts resectability and the risk of surgical complications. To maximize resection and...
CONTEXT
Outcome of craniopharyngioma is related to its locoregional extension, which impacts resectability and the risk of surgical complications. To maximize resection and minimize complications, optic tract localization, temporal lobe extension and hypothalamic involvement are essential for surgical management.
OBJECTIVE
To assess the outcome of craniopharyngiomas depending on their relation to the hypothalamus location.
METHODS
We conducted a retrospective analysis of 79 patients with a craniopharyngioma who underwent surgery from 2007 to 2022. Craniopharyngiomas were classified in three groups, depending on the type of hypothalamus involvement assessed by preoperative MRI: infra-hypothalamic (type A, n=33); perforating the hypothalamus (type B, n=40); supra-hypothalamic (type C, n=6). Surgical strategy was guided by the type of hypothalamic involvement, favoring endonasal approaches for type A and type B, and transcranial approaches for type C.
RESULTS
Long-term disease control was achieved in 33/33 (100%), 37/40 (92%) and 5/6 (83%) patients in type A, B and C respectively. In type B, vision was improved in 32/36 (89%) patients, while hypothalamic function was improved, stable or worsened in 6/40 (15%), 32/40 (80%) and 2/40 (5%) patients respectively. Papillary craniopharyngiomas were found in 5/33 (15%), 9/40 (22%) and 3/6 (50%) patients in types A, B and C respectively. In four patients, BRAF/MEK inhibitors were used, with significant tumor shrinkage in all cases.
CONCLUSION
Craniopharyngiomas located below the hypothalamus or perforating it can be safely treated by transsphenoidal surgery. For supra-hypothalamic craniopharyngiomas, postoperative results are less favorable, and documenting a BRAF-mutation may improve outcome, if targeted therapy was efficient enough to replace surgical debulking.
PubMed: 38287910
DOI: 10.1210/clinem/dgae049 -
Journal of Neuroscience Research Jan 2024Primary cilia are microtubule-based sensory organelles that project from the apical surface of most mammalian cells, including oligodendrocytes, which are myelinating...
Primary cilia are microtubule-based sensory organelles that project from the apical surface of most mammalian cells, including oligodendrocytes, which are myelinating cells of the central nervous system (CNS) that support critical axonal function. Dysfunction of CNS glia is associated with aging-related white matter diseases and neurodegeneration, and ciliopathies are known to affect CNS white matter. To investigate age-related changes in ciliary profile, we examined ciliary length and frequency in the retinogeniculate pathway, a white matter tract commonly affected by diseases of aging but in which expression of cilia has not been characterized. We found expression of Arl13b, a marker of primary cilia, in a small group of Olig2-positive oligodendrocytes in the optic nerve, optic chiasm, and optic tract in young and aged C57BL/6 wild-type mice. While the ciliary length and ciliated oligodendrocyte cells were constant in young mice in the retinogeniculate pathway, there was a significant increase in ciliary length in the anterior optic nerve as compared to the aged animals. Morphometric analysis confirmed a specific increase in the ciliation rate of CC1 /Olig2 oligodendrocytes in aged mice compared with young mice. Thus, the prevalence of primary cilia in oligodendrocytes in the visual pathway and the age-related changes in ciliation suggest that they may play important roles in white matter and age-associated optic neuropathies.
Topics: Animals; Mice; Mice, Inbred C57BL; Optic Nerve; Oligodendroglia; Neuroglia; White Matter; Mammals
PubMed: 38284846
DOI: 10.1002/jnr.25273 -
European Radiology Jan 2024To evaluate the combined performance of orbital MRI and intracranial visual pathway diffusion kurtosis imaging (DKI) in diagnosing dysthyroid optic neuropathy (DON).
OBJECTIVES
To evaluate the combined performance of orbital MRI and intracranial visual pathway diffusion kurtosis imaging (DKI) in diagnosing dysthyroid optic neuropathy (DON).
METHODS
We retrospectively enrolled 61 thyroid-associated ophthalmopathy (TAO) patients, including 25 with DON (40 eyes) and 36 without DON (72 eyes). Orbital MRI-based apical muscle index (MI), diameter index (DI) of the optic nerve (ON), area index (AI) of the ON, apparent diffusion coefficient (ADC) and signal intensity ratio (SIR) of the ON, DKI-based kurtosis fractional anisotropy (KFA) and mean kurtosis (MK) of the optic tract (OT), optic radiation (OR), and Brodmann areas (BAs) 17, 18, and 19 were measured and compared between groups. The diagnostic performances of models were evaluated using receiver operating characteristic curve analyses and compared using the DeLong test.
RESULTS
TAO patients with DON had significantly higher apical MI, apical AI, and SIR of the ON, but significantly lower ADC of the ON than those without DON (p < 0.05). Meanwhile, the DON group exhibited significantly lower KFA across the OT, OR, BA17, BA18, and BA19 and lower MK at the OT and OR than the non-DON group (p < 0.05). The model integrating orbital MRI and intracranial visual pathway DKI parameters performed the best in diagnosing DON (AUC = 0.926), with optimal diagnostic sensitivity (80%) and specificity (94.4%), followed by orbital MRI combination (AUC = 0.890), and then intracranial visual pathway DKI combination (AUC = 0.832).
CONCLUSION
Orbital MRI and intracranial visual pathway DKI can both assist in diagnosing DON. Combining orbital and intracranial imaging parameters could further optimize diagnostic efficiency.
CLINICAL RELEVANCE STATEMENT
The novel finding could bring novel insights into the precise diagnosis and treatment of dysthyroid optic neuropathy, accordingly, contributing to the improvement of the patients' prognosis and quality of life in the future.
KEY POINTS
• Orbital MRI and intracranial visual pathway diffusion kurtosis imaging can both assist in diagnosing dysthyroid optic neuropathy. • Combining orbital MRI and intracranial visual pathway diffusion kurtosis imaging optimized the diagnostic efficiency of dysthyroid optic neuropathy.
PubMed: 38276980
DOI: 10.1007/s00330-024-10615-9 -
Child's Nervous System : ChNS :... Apr 2024Intracranial teratoma represents a rare neoplasm, occurring predominantly during childhood. Characteristic symptoms depend on the location but are mainly hydrocephalus,... (Review)
Review
BACKGROUND
Intracranial teratoma represents a rare neoplasm, occurring predominantly during childhood. Characteristic symptoms depend on the location but are mainly hydrocephalus, visual disturbances, hypopituitarism, and diabetes insipidus. Initial diagnosis can be challenging due to similar radiological features in both teratomas and other lesions such as craniopharyngiomas. Gross total resection is recommended if feasible and associated with a good prognosis.
CASE DESCRIPTION
A 10-year-old girl presented with newly diagnosed growth retardation, fatigue, cephalgia and bilateral hemianopia. Further laboratory analysis confirmed central hypothyroidism and hypercortisolism. Cranial magnetic resonance imaging showed a cystic space-occupying lesion in the sellar and suprasellar compartment with compression of the optic chiasm without hydrocephalus present, suspicious of craniopharyngioma. Subsequently, an endonasal endoscopic transsphenoidal near-total tumor resection with decompression of the optic chiasm was performed. During postoperative recovery the patient developed transient diabetes insipidus, the bilateral hemianopia remained unchanged. The patient could be discharged in a stable condition, while hormone replacement for multiple pituitary hormone deficiency was required. Surprisingly, histopathology revealed conspicuous areas of skin with formation of hairs and squamous epithelia, compatible with a mature teratoma.
CONCLUSIONS
We present an extremely rare case of pediatric sellar teratoma originating from the pituitary gland and a review of literature focusing on the variation in presentation and treatment. Sellar teratomas are often mistaken for craniopharyngioma due to their similar radiographic appearances. However, the primary goal of treatment for both pathologies is to decompress eloquent surrounding structures such as the optic tract, and if applicable, resolution of hydrocephalus while avoiding damage to the pituitary stalk and especially the hypothalamic structures. If feasible, the aim of surgery should be gross total resection.
Topics: Female; Humans; Child; Craniopharyngioma; Hemianopsia; Pituitary Neoplasms; Hypopituitarism; Diabetes Insipidus; Central Nervous System Neoplasms; Teratoma; Hydrocephalus
PubMed: 38276973
DOI: 10.1007/s00381-024-06296-w -
World Neurosurgery Apr 2024Craniopharyngiomas are histologically benign tumors that originate from squamous rests along the pituitary stalk. They make up approximately 1.2% to 4.6% of all...
Craniopharyngiomas are histologically benign tumors that originate from squamous rests along the pituitary stalk. They make up approximately 1.2% to 4.6% of all intracranial tumors and do not show significant differences in occurrence based on sex. Adamantinomatous craniopharyngiomas have 2 peaks of incidence, commonly observed in patients from ages 5 to 15 years and again from 45 to 60 years. In contrast, papillary craniopharyngiomas mainly affect adults in their fifth and sixth decades of life. The "malignancy" of craniopharyngiomas is attributed to their location and the challenges associated with achieving complete removal because they can manifest in the sellar, parachiasmatic, and intraventricular regions or a combination of these. Various approaches have been used to resect these tumors. Radical resection offers the most promising option for disease control, potential cure, and the ability to transform the disease from lethal to survivable in children, allowing for a functional adult life. Meticulous evaluation is crucial to determine the appropriate approach and side, with particular emphasis on closely examining the relationship between the tumor and optic pathways (nerve, chiasm, tract), which are frequently involved. This assessment should also include the tumor's relationship with other crucial structures, such as the hypothalamus and adjacent arteries, to ensure that the strategy is adjusted accordingly to further minimize the risk of postoperative morbidity. Video 1 demonstrates a left-sided pterional transsylvian approach to remove a parachiasmatic craniopharyngioma involving the left optic chiasm and tract.
Topics: Adult; Child; Humans; Craniopharyngioma; Pituitary Neoplasms; Pituitary Gland; Hypothalamus; Optic Chiasm
PubMed: 38266994
DOI: 10.1016/j.wneu.2024.01.083 -
Medicina (Kaunas, Lithuania) Dec 2023is a self-propagating microorganism that commonly causes respiratory tract infections. It can also cause a variety of extrapulmonary symptoms with or independently of...
is a self-propagating microorganism that commonly causes respiratory tract infections. It can also cause a variety of extrapulmonary symptoms with or independently of respiratory symptoms, such as skin lesions, arthralgia, myalgia, hemolysis, cardiac lesions, gastrointestinal symptoms, and central nervous system lesions, which are rare manifestations reported in approximately 0.1% of cases. In this study, we present a unique case of Mycoplasma-related abducens nerve palsy, polyarthritis, and erythema multiforme without respiratory disease. The patient was a 69-year-old woman who presented to our hospital with a skin rash, fever, arthralgia, and diplopia without respiratory symptoms. Brain magnetic resonance imaging showed optic neuritis on the right side, suggesting the diplopia was caused by right abducens nerve palsy. However, the etiologies of abducens nerve palsy were not revealed by the physical examination, blood biochemistry tests, or bacteriological examinations, including the cerebrospinal fluid examination obtained at admission. Mycoplasma infection was suspected from erythema multiforme revealed by a skin biopsy and polyarthralgia, and it was finally diagnosed according to elevated Mycoplasma particle agglutination (PA) antibodies in paired serum. Though minocycline did not improve her diplopia, the daily administration of 30 mg of prednisolone gradually improved her symptoms, and the Mycoplasma PA antibody titer, which was regularly measured in the clinical course, also decreased, suggesting a relationship between Mycoplasma infection and abducens nerve palsy. This is the first case of isolated abducens nerve palsy, which was reported as the only central neurological symptom in an adult patient with Mycoplasma infection. The mechanism or pathogenesis of CNS manifestations caused by remains to be elucidated, and further investigation is needed. Hence, Mycoplasma infection is a common disease. Clinicians should be aware of the diverse manifestations, including abducens nerve palsy, of Mycoplasma infection and should consider Mycoplasma infection even in the absence of typical respiratory symptoms.
Topics: Humans; Adult; Female; Aged; Diplopia; Erythema Multiforme; Mycoplasma Infections; Abducens Nerve Diseases; Arthritis; Arthralgia
PubMed: 38256298
DOI: 10.3390/medicina60010036 -
Neurosurgical Review Jan 2024Cavernomas are histologically benign vascular malformations found at different sites in the brain. A rare site for such cavernomas, however, is the anterior optic... (Review)
Review
Cavernomas are histologically benign vascular malformations found at different sites in the brain. A rare site for such cavernomas, however, is the anterior optic pathway, comprising the optic nerve, chiasma, and optic tract-called optochiasmatic cavernomas (OCC). These lesions usually present with sudden onset or progressive vision loss, headache, and features mimicking pituitary apoplexy. In this paper, we describe a case of OCC operated at our center. We carry out an updated review of literature depicting cases of OCC, their clinical presentation, management, and postoperative complications. We also propose a novel classification system based on lesion location and further analyze these cavernoma types with respect to the surgical approach used and visual outcome. A 30-year-old lady had presented with a 3-week history of progressive bilateral vision loss and headache. Based on imaging, she was suspected to have a cavernous angioma of the chiasma and left optic tract. Due to progressive vision deterioration, the lesion was surgically excised using pterional craniotomy. Postoperatively, her visual symptoms improved, but she developed diabetes insipidus. Clinical and radiological follow-up has been done for 18 months after surgery. A total of 81 cases have been described in the literature, including the present case. Chiasmal apoplexy is the most common presentation. Surgical excision is the standard of care. Our analysis based on lesion location shows the most appropriate surgical approach to be used for each cavernoma type. Visual outcome correlates with the preoperative visual status. Visual outcome is good in patients presenting with acute chiasmal apoplexy, and when complete surgical excision is performed. The endonasal endoscopic approach was found to provide the best visual outcome. In addition to preoperative visual status, complete surgical excision predicts favorable visual outcomes in OCC. Our proposed classification system guides the appropriate surgical approach required for a particular location of the cavernoma.
Topics: Adult; Female; Humans; Headache; Hemangioma, Cavernous; Optic Chiasm; Optic Nerve; Stroke; Vision Disorders
PubMed: 38238497
DOI: 10.1007/s10143-024-02288-1 -
The British Journal of Ophthalmology Jan 2024To describe the pattern of MRI changes in the pregeniculate visual pathway in Leber hereditary optic neuropathy (LHON).
PURPOSE
To describe the pattern of MRI changes in the pregeniculate visual pathway in Leber hereditary optic neuropathy (LHON).
METHOD
This retrospective observational study enrolled 60 patients with LHON between January 2015 and December 2021. The abnormal MRI features seen in the pregeniculate visual pathway were investigated, and then correlated with the causative mitochondrial DNA (mtDNA) mutation, the distribution of the MRI lesions and the duration of vision loss.
RESULT
The cohort included 48 (80%) males and 53 (88%) had bilateral vision loss. The median age of onset was 17.0 years (range 4.0-58.0). 28 (47%) patients had the m.11778G>A mutation. 34 (57%) patients had T2 hyperintensity (HS) in the pregeniculate visual pathway and 13 (22%) patients with chiasmal enlargement. 20 patients (71%) carrying the m.11778G>A mutation had T2 HS, significantly more than the 14 patients (44%) with T2 HS in the other LHON mutation groups (p=0.039). Furthermore, significantly more patients in the m.11778G>A group (16 patients (57%)) had T2 HS in optic chiasm (OCh)/optic tract (OTr) than the other LHON mutation groups (7 patients (22%), p=0.005). Optic chiasmal enlargement was more common in patients with vision loss duration <3 months compared with those ≥3 months (p=0.028).
CONCLUSION
T2 HS in the pregeniculate visual pathway is a frequent finding in LHON. Signal changes in the OCh/OTr and chiasmal enlargement, in particular within the first 3 months of visual loss, were more commonly seen in patients carrying the m.11778G>A mtDNA mutation, which may be of diagnostic significance.
PubMed: 38237954
DOI: 10.1136/bjo-2023-324628 -
Cureus Dec 2023This is a case of an orbital abscess evidenced radiologically in a 41-year-old female with no comorbidities. She was healthy and had no history of trauma or infection of...
This is a case of an orbital abscess evidenced radiologically in a 41-year-old female with no comorbidities. She was healthy and had no history of trauma or infection of the adjacent structures. She denied having symptoms of upper or lower respiratory and urinary tract infections. The decision for surgical drainage was made following a slow response to antimicrobial agents after 24 hours, a progressive painful erythematous eyelid swelling, and further deterioration of vision. Her clinical condition and visual acuity improved following cutaneous incision and drainage. Culture and sensitivity results for urine and orbital abscess were positive for Staphylococcus (S.) aureus. The patient regained full visual recovery without any sequelae. In conclusion, an orbital abscess is a blinding and life-threatening condition that rarely occurs in immunocompetent individuals and uncommonly arises from distant sources. A high index of suspicion, early institution of appropriate diagnostic imaging, and aggressive medical and surgical treatment are necessary for a favorable visual outcome in orbital abscess cases.
PubMed: 38229775
DOI: 10.7759/cureus.50693