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Journal of Visualized Experiments : JoVE Nov 2019Oculomotor neurons (CN3s) and trochlear neurons (CN4s) exhibit remarkable resistance to degenerative motor neuron diseases such as amyotrophic lateral sclerosis (ALS)...
Oculomotor neurons (CN3s) and trochlear neurons (CN4s) exhibit remarkable resistance to degenerative motor neuron diseases such as amyotrophic lateral sclerosis (ALS) when compared to spinal motor neurons (SMNs). The ability to isolate and culture primary mouse CN3s, CN4s, and SMNs would provide an approach to study mechanisms underlying this selective vulnerability. To date, most protocols use heterogeneous cell cultures, which can confound the interpretation of experimental outcomes. To minimize the problems associated with mixed-cell populations, pure cultures are indispensable. Here, the first protocol describes in detail how to efficiently purify and cultivate CN3s/CN4s alongside SMNs counterparts from the same embryos using embryonic day 11.5 (E11.5) Isl:GFP transgenic mouse embryos. The protocol provides details on the tissue dissection and dissociation, FACS-based cell isolation, and in vitro cultivation of cells from CN3/CN4 and SMN nuclei. This protocol adds a novel in vitro CN3/CN4 culture system to existing protocols and simultaneously provides a pure species- and age-matched SMN culture for comparison. Analyses focusing on the morphological, cellular, molecular, and electrophysiological characteristics of motor neurons are feasible in this culture system. This protocol will enable research into the mechanisms that define motor neuron development, selective vulnerability, and disease.
Topics: Animals; Cell Culture Techniques; Cell Nucleus; Embryo, Mammalian; Green Fluorescent Proteins; LIM-Homeodomain Proteins; Mice; Mice, Transgenic; Motor Neurons; Oculomotor Nerve; Spinal Cord; Transcription Factors; Trochlear Nerve
PubMed: 31789317
DOI: 10.3791/60440 -
La Tunisie MedicalePainful ophthalmoplegia is a common presenting symptom in neuro-ophthalmology emergencies. We report an unusual case of a recurrent painful ophthalmoplegia due to a...
Painful ophthalmoplegia is a common presenting symptom in neuro-ophthalmology emergencies. We report an unusual case of a recurrent painful ophthalmoplegia due to a third nerve schwannoma mimicking « ophthalmoplegic migraine ». A 18 year-old girl had presented 4 episodes of left eye painful ophthalmoplegia respectively in 8, 13, 16 and 17 years old. One year after the last episode, neurological examination was normal. Brain MRI focused on the oculomotor nerve showed an enhancing nodular lesion suggesting a third nerve schwannoma. Thus, recurrent painful ophthalmoplegia revealing oculomotor nerve schwannoma, as described in our case, is exceptional. To our knowledge, only thirteen cases have been reported in the literature. Third nerve schwannoma is a rare cranial nerve tumor, typically revealed by progressive palsy of the oculomotor nerve. Recurrent painful ophthalmoplegia with persistent headache and enhancement in brain imaging should suggest tumoral lesions.
Topics: Adolescent; Female; Humans; Magnetic Resonance Imaging; Neurilemmoma; Oculomotor Nerve; Ophthalmoplegia; Ophthalmoplegic Migraine; Tolosa-Hunt Syndrome
PubMed: 35261021
DOI: No ID Found -
Surgical Neurology International 2020Although it is well known that internal carotid-posterior communicating artery (ICA-PcomA) aneurysms compress the oculomotor nerve and cause nerve palsy, cases of...
BACKGROUND
Although it is well known that internal carotid-posterior communicating artery (ICA-PcomA) aneurysms compress the oculomotor nerve and cause nerve palsy, cases of ICA-PcomA aneurysms splitting the oculomotor nerve are extremely rare.
CASE DESCRIPTION
We present the rare case of an asymptomatic, growing, left-sided ICA-PcomA aneurysm that was confirmed to split the oculomotor nerve. We report the clinical course and discuss the underlying mechanism. The oculomotor nerve, which is an aggregate of multiple fibers, exhibits age-related loss of compactness in the arrangement of its nerve fibers.
CONCLUSION
We speculate that injury to the nerve fibers by aneurysmal compression was avoided because of the rare phenomenon of splitting of the oculomotor nerve.
PubMed: 33194286
DOI: 10.25259/SNI_612_2020 -
Indian Journal of Ophthalmology 2013To derive a reliable estimate of the frequency of pupillary involvement and to study the patterns and course of anisocoria in conjunction with ophthalmoplegia in... (Comparative Study)
Comparative Study
AIMS
To derive a reliable estimate of the frequency of pupillary involvement and to study the patterns and course of anisocoria in conjunction with ophthalmoplegia in diabetes-associated oculomotor nerve palsy.
MATERIALS AND METHODS
In this prospective analytical study, standardized enrolment criteria were employed to identify 35 consecutive patients with diabetes-associated oculomotor nerve palsy who were subjected to a comprehensive ocular examination. Standardized methods were used to evaluate pupil size, shape, and reflexes. The degree of anisocoria, if present and the degree of ophthalmoplegia was recorded at each visit.
RESULTS
Pupillary involvement was found to be present in 25.7% of the total number of subjects with diabetic oculomotor nerve palsy. The measure of anisocoria was < 2 mm, and pupil was variably reactive at least to some extent in all cases with pupillary involvement. Majority of patients in both the pupil-involved and pupil-spared group showed a regressive pattern of ophthalmoplegia. Ophthalmoplegia reversed much earlier and more significantly when compared to anisocoria.
CONCLUSIONS
Pupillary involvement in diabetes-associated oculomotor nerve palsy occurs in about 1/4 th of all cases. Certain characteristics of the pupil help us to differentiate an ischemic insult from an aneurysmal injury to the 3 rd nerve. Ophthalmoplegia resolves much earlier than anisocoria in diabetic oculomotor nerve palsies.
Topics: Adult; Aged; Aged, 80 and over; Anisocoria; Diabetes Complications; Female; Humans; Incidence; India; Male; Middle Aged; Oculomotor Nerve Diseases; Ophthalmoplegia; Prospective Studies; Pupil
PubMed: 23275215
DOI: 10.4103/0301-4738.99999 -
Investigative Ophthalmology & Visual... Apr 2007High-resolution magnetic resonance imaging (MRI) can now directly demonstrate innervation to extraocular muscles and quantify optic nerve size. A quantitative MRI...
PURPOSE
High-resolution magnetic resonance imaging (MRI) can now directly demonstrate innervation to extraocular muscles and quantify optic nerve size. A quantitative MRI technique was developed to study the oculomotor nerve (CN3) and applied to congenital fibrosis of extraocular muscles (CFEOM) and congenital oculomotor palsy.
METHODS
The subarachnoid portions of the CN3s were imaged with a 1.5-T MRI scanner and conventional head coils, acquiring heavily T(2)-weighted oblique axial planes 1-mm thick and parallel to the optic chiasm. Thirteen normal subjects, 14 with CFEOM, and 3 with congenital CN3 palsy were included. Digital image analysis was used to measure CN3 diameter, which was correlated with motility findings.
RESULTS
In CFEOM, CN3 diameter was bilaterally subnormal in eight subjects, unilaterally subnormal in three subjects, and normal in three subjects. Mean +/- SD CN3 diameter in CFEOM was 1.14 +/- 0.61 mm, significantly smaller than the diameter in normal subjects, which measured 2.01 +/- 0.36 mm (P < 0.001). CN3 diameter variably correlated with clinical function. One subject with congenital CN3 palsy showed bilateral CN3 hypoplasia, but CN3 diameter was normal in two other subjects with congenital CN3 palsy.
CONCLUSIONS
Unilateral or bilateral hypoplasia of CN3 is quantitatively demonstrable using MRI in many cases of CFEOM and occasionally in congenital CN3 palsy. Variations in CN3 diameter in CFEOM and congenital CN3 palsy suggest mechanistic heterogeneity of these disorders that may be clarified by further imaging and genetic studies.
Topics: Adolescent; Adult; Aged; Brain Stem; Female; Fibrosis; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Oculomotor Muscles; Oculomotor Nerve; Oculomotor Nerve Diseases; Strabismus
PubMed: 17389489
DOI: 10.1167/iovs.06-0691 -
Neural Regeneration Research Oct 2016Functional recovery after oculomotor nerve injury is very poor. Electrical stimulation has been shown to promote regeneration of injured nerves. We hypothesized that...
Functional recovery after oculomotor nerve injury is very poor. Electrical stimulation has been shown to promote regeneration of injured nerves. We hypothesized that electrical stimulation would improve the functional recovery of injured oculomotor nerves. Oculomotor nerve injury models were created by crushing the right oculomotor nerves of adult dogs. Stimulating electrodes were positioned in both proximal and distal locations of the lesion, and non-continuous rectangular, biphasic current pulses (0.7 V, 5 Hz) were administered 1 hour daily for 2 consecutive weeks. Analysis of the results showed that electrophysiological and morphological recovery of the injured oculomotor nerve was enhanced, indicating that electrical stimulation improved neural regeneration. Thus, this therapy has the potential to promote the recovery of oculomotor nerve dysfunction.
PubMed: 27904500
DOI: 10.4103/1673-5374.193248 -
Pain Research & Management 2019Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is an uncommon disorder with repeated episodes of ocular cranial... (Review)
Review
BACKGROUND
Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. The age of presentation is most often during childhood or adolescence. MRI has a central role in the assessment of the RPON, especially to distinguish orbital, parasellar, or posterior fossa lesions that mimic symptoms of RPON. Actually, oculomotor nerve tumors may be masquerade as RPON so that MRI follow-ups are required to detect the possibility of tumor etiology.
CASE PRESENTATION
We report a 16-year-old boy with a 7-year follow-up and multiple brain MRI data, previously diagnosed as OM. The last brain MRI, performed during an acute phase of oculomotor paresis with ipsilateral headache, showed a nodular lesion described as schwannoma of III cranial nerve. Then, we reviewed the literature on OM and RPON in pediatric age with a focus on brain MRI findings.
CONCLUSIONS
This review highlights the important role of serial brain MRIs in the long-term follow-up of RPON, especially in the cases with childhood onset, in order to not delay the diagnosis of a possible oculomotor nerve schwannoma.
Topics: Adolescent; Cranial Nerve Neoplasms; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Neurilemmoma; Oculomotor Nerve Diseases; Ophthalmoplegic Migraine
PubMed: 31662812
DOI: 10.1155/2019/5392945 -
AJNR. American Journal of Neuroradiology Sep 1995To assess the utility of MR in third cranial nerve palsy.
PURPOSE
To assess the utility of MR in third cranial nerve palsy.
METHODS
We reviewed precontrast and postcontrast MR of 50 patients with third cranial nerve palsy.
RESULTS
MR demonstrated an appropriate lesion in 32 cases. Of these patients, 6 had brain stem lesions and 15 had involvement of the nerve in the cavernous sinus; lesions of the cisternal segment of the nerve were present in 11 patients, with enhancement of this segment observed in 9 patients. An inflammatory or infiltrative source of the palsy was indicated in 19 of these 32 cases. Of 7 patients with pupillary involvement suggestive clinically of a compressive lesion, 4 demonstrated thickening and enhancement consistent with an infiltrative lesion of the nerve. Eighteen patients with pupil-sparing third cranial nerve palsies and a history of diabetes or vascular disease had normal MR findings, with no enhancement of the third cranial nerve observed.
CONCLUSIONS
Patients who do not have a history of diabetes or hypertension and in whom a complete or incomplete third cranial nerve palsy develops with or without pupil sparing should undergo MR imaging initially (unless there are clear symptoms or signs of subarachnoid hemorrhage) to exclude the presence of an infiltrative lesion or intraparenchymal process. Patients who have a history of vascular disease and a clinical presentation that is suggestive of an ischemic event may be observed initially, but should undergo imaging if improvement does not occur within 3 months.
Topics: Brain; Brain Diseases; Brain Neoplasms; Brain Stem; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Male; Nerve Compression Syndromes; Oculomotor Nerve; Ophthalmoplegia; Pupil Disorders; Retrospective Studies
PubMed: 7502972
DOI: No ID Found -
Surgical Neurology International 2023Isolated oculomotor nerve palsy is a relatively uncommon symptom of pituitary adenoma that usually occurs in association with pituitary apoplexy or cavernous sinus (CS)...
BACKGROUND
Isolated oculomotor nerve palsy is a relatively uncommon symptom of pituitary adenoma that usually occurs in association with pituitary apoplexy or cavernous sinus (CS) invasion.
CASE DESCRIPTION
We report two cases of relatively small pituitary adenomas with neither apoplexy nor CS invasion presenting as isolated oculomotor nerve palsy. Both patients presented with gradually worsening diplopia, without headache or visual field defects. Magnetic resonance imaging (MRI) showed a pituitary tumor with no evidence of intratumoral hemorrhage. Computed tomography revealed a lateroposterior extension of the tumor with the erosion of the posterior clinoid process. Constructive interference in steady-state MRI revealed compression of the oculomotor nerve by the tumor at the oculomotor triangle. The patients underwent endoscopic transsphenoidal surgery, and the intraoperative findings showed that the tumors did not invade the CS. The tumors were completely resected, and the oculomotor palsies resolved fully.
CONCLUSION
These cases illustrate the need to consider isolated oculomotor nerve palsy as an initial manifestation of a relatively small pituitary adenoma with neither apoplexy nor CS invasion. Based on the characteristic radiological findings, early surgical treatment is recommended to preserve oculomotor function.
PubMed: 37810282
DOI: 10.25259/SNI_421_2023 -
Case Reports in Neurology 2021Primary Epstein-Barr virus (EBV) infection is the main cause of infectious mononucleosis (IM), which typically presents with a triad of fever, lymphadenopathy, and...
Primary Epstein-Barr virus (EBV) infection is the main cause of infectious mononucleosis (IM), which typically presents with a triad of fever, lymphadenopathy, and tonsillar pharyngitis in young adults. In contrast, neurological manifestations of IM are rare. We report on a 23-year-old man with subacute oculomotor nerve palsy followed by symptoms of IM 6 days later. Primary EBV infection was confirmed by PCR detection of EBV DNA in blood as well as by subsequent serology. High-resolution magnetic resonance imaging revealed an edematous change at the root exit zone and gadolinium enhancement of the right oculomotor nerve as well as pial enhancement adjacent to the right ventral mesencephalon. A review of the literature identified 5 further patients with isolated oculomotor nerve palsy as the presenting symptom of unfolding primary EBV infection. MRIs performed in 3 of those 5 patients revealed a pattern of contrast enhancement similar to that of the present case. This case report and literature review highlight that, although rare, IM should be considered in the differential diagnosis of oculomotor nerve palsy in young adults.
PubMed: 35082645
DOI: 10.1159/000520437