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Optic atrophy as the first symptom in Hallervorden-Spatz syndrome.

A 16-year-old boy with the classic or postinfantile type of Hallervorden-Spatz syndrome is described. Bilateral optic atrophy with visual loss but without retinal changes was the only presenting symptom. Mild cognitive impairment, behavioural disturbances and insidious extrapyramidal involvement appeared later. MRI showed marked symmetrical hypointensity of the globi pallidi and substantia nigra. This new observation suggests that the occurrence of optic atrophy in a patient with Hallervorden-Spatz syndrome should be regarded as noncoincidental and stresses the importance of an accurate neurological work-up in all adolescents with any unusual form of progressive optic atrophy.

Adolescent↗

"Pusher syndrome" following cortical lesions that spare the thalamus.

Stroke patients with "pusher syndrome" show severe misperception of their own upright body orientation although visual-vestibular processing is almost intact. This dissociation argues for a second graviceptive system in humans for the perception of body orientation. Recent studies revealed that the posterior thalamus is an important part of this system. The present investigation aimed to study the cortical representation of this system beyond the thalamus. We evaluated 45 acute patients with and without contraversive pushing following left-or right-sided cortical lesions sparing the thalamus. In both hemispheres, the simple lesion overlap associated with contraversive pushing typically centered on the insular cortex and parts of the postcentral gyrus. The comparison between pusher patients and controls who were matched with respect to age, lesion size, and the frequency of spatial neglect, aphasia and visual field defects revealed only very small regions that were specific for the pusher patients with cortical damage sparing the thalamus. Obviously, the cortical structures representing our control of upright body orientation are in close anatomical proximity to those areas that induce aphasia in the left hemisphere and spatial neglect in the right hemisphere when lesioned. We conclude that in addition to the subcortical area previously identified in the posterior thalamus, parts of the insula and postcentral gyrus appear to contribute at cortical level to the processing of the afferent signals mediating the graviceptive information about upright body orientation.

Aged↗

Crossed aphasia with left spatial neglect and visual imperception: a case report.

A 64-year-old right-handed woman with no left-handers in the family developed aphasia associated with moderate left hemiparesis and dense left homonymous hemianopia following rupture of a right middle cerebral artery aneurysm and subsequent selective surgery confined to the right hemisphere. Severe left spatial neglect and constructional apraxia were also present. The patient was an achondroplasic dwarf whose previous medical and neurological history was otherwise unremarkable. Computed tomography of the brain showed a large right temporo-insulofrontoparietal lesion. Language and nonverbal cognitive functions were assessed after 2 and 6 months, and then four years later. A reportedly overall language disruption in the acute period evolved into Wernicke's aphasia and then into a mild form of conduction aphasia. The associated left spatial neglect eventually shrank to a minimum. The patient never had clinically detectable visual agnosia, but on specific tests of visual recognition and perception some impairment was found four years after onset. The left hemiparesis disappeared in time while the left hemianopia persisted. This case is a convincing example of an entirely righthanded person in whom both linguistic and visuospatial functions are represented in the right hemisphere.

Achondroplasia↗

Frameless image-guided neuronavigation in orbital surgery: practical applications.

Frameless neuronavigation has been increasingly used in intracranial and spinal neurosurgery. However, the application of neuronavigation in orbital surgery has not yet been reported. The purpose of this study was to evaluate whether frameless navigation in the orbit provides sufficient clinical application accuracy and thus a useful tool in orbital surgery. A frameless infrared-based neuronavigation system (VectorVision, BrainLAB, Heimstetten, Germany) was used in the microsurgical removal of 11 orbital tumors (five cavernomas, three lymphomas, two dermoid tumors, and one rhabdomyosarcoma). The transconjunctival approach was performed in seven cases, lateral orbitotomy in three cases, and the extradural pterional approach was performed in one case. The surgery was successful and without any complications in all cases. The registration accuracy of the neuronavigation was between 1.8 and 2.2 mm, with a mean of 1.9 mm. Subtotal tumor debulking was performed in one case with lymphoma. One patient was only biopsied due to suspected systemic lymphoma disease. Total removal of the tumor could be accomplished in the other nine patients. Image guidance during orbital surgery offered excellent three-dimensional guidance on the surface of the intraorbital lesions, allowing a safer, more controlled surgery. The surgical targets in the orbit are fixed structures, thus no shifting occurs and continuous high intraoperative navigation accuracy can be achieved. The use of the navigation clearly reduces the operative risk and increases the effectiveness of microsurgical orbital procedures.

Adult↗

Clinical manifestations and management of orbital mucoceles: the role of ophthalmologists.

PURPOSE: To report the clinical features of orbital mucoceles and discuss the role of ophthalmologists in the management of patients with orbital mucoceles. METHODS: A retrospective chart review was performed of all patients with orbital mucoceles treated at the National Taiwan University Hospital from 1990 through 2002. The basic profiles, clinical features, and the management of the patients with orbital mucoceles were recorded and analyzed. RESULTS: The records of a total of 15 patients (aged 22 to 76, mean 45.5 years) with orbital mucoceles were selected for this study. The initial presentations included proptosis in ten patients (66.7%), diplopia in five (33.3%), ocular movement limitation in four (26.7%), periorbital pain in four (26.7%), palpable mass lesion in four (26.7%), ptosis in three (20.0%), decreased visual acuity in three (20.0%), headache in two (13.3%), and pupil abnormality with relative afferent papillary defect in one (6.67%). Of the 15 patients, the origins of the orbital mucocele were in the frontoethmoidal sinus in six (40.0%), the ethmoidal sinus in three (20.0%), the frontal sinus in three (20.0%), the maxillary sinus in one (6.7%), and the sphenoidal sinus in two (13.3%). The transcaruncular approach for the management of orbital mucoceles was performed in eight cases, the Lynch approach in three cases, the transforniceal approach in two cases, and functional endoscopic sinus surgery in one case. After surgery, no recurrence was noted. CONCLUSIONS: The frontoethmoidal sinus was the commonest origin of an orbital mucocele, and proptosis was the most frequent sign in patients with an orbital mucocele. All cases showed a favorable response to surgical intervention, and the transcaruncular approach may offer a good surgical option for the management of orbital mucoceles, especially for ophthalmologists.

Adult↗

Glioblastoma multiforme of the pineal region.

Glioblastoma multiforme (GBMs) tumors are exceedingly rare tumors in the pineal region. We present three cases in which patients presented with a pineal/posterior third ventricular region mass and review all the previously reported cases in the literature. Pineal region GBM seems to be a very aggressive tumor with a high rate of leptomeningeal and ependymal metastatic disease. Patients usually present with signs and symptoms of hydrocephalus and Parinaud's syndrome. The clinical and radiological characteristics of pineal GBM do not differentiate it from other malignancies of this region, thus surgical biopsy is generally required for definitive diagnosis. Glioblastoma should be considered in the differential diagnosis of the pineal region tumors, especially when evidence of leptomeningeal or ependymal metastatic disease is present.

Adult↗

Applications of artificial intelligence in robot-assisted surgery: a systematic review.

To characterize applications of artificial intelligence (AI) in robot-assisted surgery, summarize technical and clinical performance, and assess the quality of the available evidence. PubMed, Web of Science Core Collection, and Scopus were searched for English-language journal articles published from 1 January 2020 through 31 October 2025. Randomized, observational, model-development, validation, and feasibility studies evaluating AI in robot-assisted surgery or closely related image-guided minimally invasive workflows were eligible. Two reviewers independently performed study selection, data extraction, and risk-of-bias assessment. Owing to heterogeneity in surgical procedures, AI tasks, analytical units, validation strategies, and outcomes, findings were synthesized descriptively without statistical pooling. The review was registered in the International Prospective Register of Systematic Reviews (CRD420251175699). Seventeen studies were included: seven clinical prediction or decision-support studies, eight intraoperative recognition, segmentation, or image-guided studies, and two training or workflow studies. Five prediction studies reported area-under-the-curve values of 0.74-0.95. Technical studies reported F1 or Dice scores of 0.525-0.995 and task-specific accuracies of 0.840-0.998. Two randomized studies suggested benefits for personalized suturing feedback and automated camera control, but neither established improved patient outcomes. Only one study had low overall risk of bias; the remaining studies were at high or unclear risk or raised some concerns. AI applications in robot-assisted surgery show promise for prediction, intraoperative perception, training, and workflow support. Evidence primarily demonstrates technical feasibility rather than established clinical effectiveness. Independent multicenter validation and prospective evaluation of patient, educational, and workflow outcomes are required before widespread implementation.

Robotic Surgical Procedures↗

A practical approach for the evaluation of women with abnormal polytomography or elevated prolactin levels.

Based upon the experience gained in the evaluation of 60 patients with abnormal polytomography and/or elevated prolactin levels, the following observations can be made: Patients with amenorrhea, amenorrhea and galactorrhea, galactorrhea alone, or anovulatory cycles and infertility may or may not have pituitary tumors. Clinical symptoms do not always correlate with the prolactin level, and patients with normal prolactins may have pituitary tumors. The incidence of empty sella is significant (15.8% in this series). Visual field examination is not a useful screening procedure, but evaluation of thyroid function is important to detect the occasional patient with hypothyroidism (3.5% in this series). The insulin tolerance test is not helpful in detecting the presence of pituitary tumors or in guiding management decisions, and the CT scan contributes little and should be omitted from the evaluation process. A straightforward, economical, and efficient approach to this clinical problem is presented.

Adenoma↗

Medulloblastoma causing a corticosteroid-responsive optic neuropathy.

A 25-year-old man had signs of optic nerve dysfunction. A diagnosis of optic neuritis was made and was supported by rapid resolution of signs and symptoms with use of corticosteroids. After initial negative radiologic investigations, his corticosteroid dosage was tapered, and the recurrence of his symptoms and signs prompted repeat computed tomographic scans. A suprasellar mass and a posterior-fossa mass were observed, and frontal craniotomy disclosed typical medulloblastoma, confirmed histologically. Although visual signs and symptoms in medulloblastoma have been reported secondary to optochiasmatic arachnoiditis and to papilledema, to the best of our knowledge this is the first reported case in which direct compression of the optic nerve has occurred as a result of extension of a posterior-fossa medulloblastoma. The favorable response of this compressive tumor to corticosteroids, stimulating an inflammatory optic neuritis, emphasizes the importance of reassessing any optic neuritis that follows an atypical course.

Adolescent↗

Clinical manifestations and radiologic findings in craniopharyngiomas in adults.

The clinical and histopathologic findings in two adults with craniopharyngioma emphasized the wide age range and diverse initial manifestations of this tumor. A retrospective clinical review of 49 additional patients substantiated these points and disclosed that over 40% were initially examined by ophthalmologists. The initial manifestations fell into four broad categories: (1) visual system abnormalities (77% of the cases), (2) headache (67%), (3) endocrinologic abnormalities (57%), and (4) mental status abnormalities (19%). The frequency of these manifestations varied slightly among different age groups. The radiologic features in the two patients described (a 75-year-old man and a 38-year-old woman) included a distinct appearance on computed tomographic scans. These craniopharyngioma were not calcified and were isodense without contrast but enhanced densely and uniformly with contrast, simulating an aneurysm of the circle of Willis.

Adolescent↗

Clinical and computed tomographic findings in the Foster Kennedy syndrome.

A 52-year-old man had progressive loss of visual acuity in his left eye associated with anosmia of five years duration. Clinical findings included papilledema in the right eye and optic atrophy in the left eye. A diagnosis of Foster Kennedy syndrome was made. Careful attention to the ophthalmoscopic appearance of the left eye disclosed optic disk swelling in regions without significant atrophy as well as dilated retinal veins. Both of these signs suggested increased intracranial pressure, rather than a primary anterior ischemic optic neuropathy. High resolution computed tomographic scanning confirmed the presence of a large subfrontal meningioma and an expanded right optic nerve sheath, consistent with the papilledema noted clinically.

Frontal Lobe↗

Magnetic resonance imaging of craniopharyngioma.

Craniopharyngiomas are common tumors located in the suprasellar region. Contrast enhancement, cyst formation, and calcification are the three characteristic features of craniopharyngiomas on computed tomographic scan. More than 90% of suprasellar craniopharyngiomas exhibit at least two of these three features, thus providing easy radiologic detection. We treated a 41-year-old man in whom a large suprasellar craniopharyngioma producing severe visual loss was not detected by computed tomography but was easily identified with magnetic resonance imaging. Thus, despite high-resolution computed tomographic scan, large suprasellar craniopharyngiomas can be missed. Magnetic resonance imaging may be superior to computed tomography in detecting these tumors.

Adult↗