Glioblastoma multiforme with oculomotor nerve involvement: case report and literature review.

Gliomas involving the cranial nerves III-XIII are rare. Even rarer are glioblastomas multiforme (GBMs) with only 10 cases previously reported. Oculomotor nerve involvement was described in only 2 patients. The mechanisms proposed so far include an origin from the nerve itself or an extension within the nerve of a midbrain tumor. We report the case of a 69-year-old man who presented with an isolated left oculomotor nerve palsy. He was found to have a left temporal GBM extended to the frontal lobe. Diagnostics and intraoperative and pathological findings clearly demonstrated a massive infiltration of the cisternal portion of the left oculomotor nerve. We suppose this could be the first case of direct oculomotor nerve invasion by exophytic spread of a supratentorial GBM or by subarachnoid seeding from a temporal tumor. Less probably, it could be the first case of an oculomotor nerve GBM with a temporal lobe invasion.

Meningioma originating from the oculomotor nerve without dural attachment in a child.

Meningioma originating from the oculomotor nerve without dural attachment in children has been rarely reported. A 6-year-old patient presented ptosis of the right eye for 5 years. MRI indicated an occupying lesion in the right cavernous sinus. A tumor originating from the oculomotor nerve without dural attachment was found during subsequent surgery and confirmed as meningioma by pathology. Subsequently, the tumor was removed completely, and the oculomotor nerve was reconstructed using the sural nerve. The patient's symptoms were relieved partially after 3 months. The findings of this case suggested that the mechanisms underlying meningioma involve ectopic arachnoid cap cells within the nerve sheath. Thus, the tumor should be removed completely; also, nerve reconstruction is suggested.

Post-traumatic Oculomotor Nerve Palsy due to Intraneural Hemorrhage: A Case Report.

Oculomotor nerve palsy following traumatic brain injury is a rare entity. A head injury can result in diffuse neuronal axonal injury with subsequent microbleed within the nerve tissue. We report an unusual case of a patient who presented with complete isolated right third nerve palsy following a road traffic accident. In this patient, magnetic resonance imaging (MRI) revealed swelling and edema of the right oculomotor nerve along its cisternal segment with contrast enhancement. The acquisition of susceptibility-weighted images on MRI helped to clinch the diagnosis of intraneural hemorrhage as a cause of post-traumatic oculomotor nerve palsy.

Malignant nerve sheath tumor of oculomotor nerve in a pediatric patient.

Malignant nerve sheath tumors are extremely rare pathologies. They tend to occur within peripheral nerves and have close association of neurofibromatosis disease. Here, we present the second case of MNST of oculomotor nerve in literature. The patient was a 2-year-old girl with left sided oculomotor nerve palsy. After resection, the patient immediately had chemotherapy and radiotherapy. One year after surgery disease progressed with extensive intracranial seedings, and she passed away.

Oculomotor nerve schwannoma: case series and literature review.

Oculomotor nerve schwannomas are rare benign cranial nerve tumors. There are only a limited number of reports on this pathology in the literature, and there are currently no established management guidelines that aid providers in deciding on surgical versus nonsurgical management. We assess the published literature on the topic to identify indications for treatment as well as outcome measures (e.g., local control rates, survival rates, and complication rates) that have been reported as associated with the various treatment modalities. We attempt to develop an algorithm for evaluation and treatment of oculomotor nerve schwannomas in order to establish consensus on how these tumors should be treated.

Complete oculomotor nerve palsy - first manifestation of gastric adenocarcinoma: clinical experience and literature review.

The diagnosis and management of the alteration of the normal function of the oculomotor nerve (third cranial nerve) varies depending on the characteristics of the paralysis, the age of the patient, and the associated symptoms and signs. Oculomotor nerve palsy may be caused by lesions located anywhere from the oculomotor nucleus to the termination of the third nerve in the extraocular muscles. Although there have been significant advances in neuroimaging to facilitate early diagnosis, the management of a patient presenting with isolated oculomotor palsy is still challenging. This review tackles the case of a 52-year-old patient, with a history of pulmonary tuberculosis (at the age of five), referred to the Department of Ophthalmology, St. Spiridon Emergency Clinical Hospital, Iasi, Romania. The patient had diplopia accompanied by right eyelid ptosis, symptoms that began suddenly 10 days before hospitalization. The clinical examination showed right eye grade II palpebral ptosis, exotropia with limitation of eyeball movements in adduction, supra-∕infraduction. Biomicroscopic examination of the anterior pole revealed the presence of anisocoria and light-near dissociation on the affected side. Numerous investigations were performed to identify the cause, starting with tumoral markers, which were within normal limits. Magnetic resonance angiography (MRA) was performed, and posterior communicating artery aneurysm was ruled out. The endocrinology examination and hormonal laboratory tests were also within normal parameters. Due to suspicions of generalized tuberculosis raised by the infectious disease doctor or presence of secondary lesions, thoraco-abdomino-pelvic computed tomography (CT) scan with contrast agent was done and its findings required gastroenterological exploration. After various explorations, the certainty diagnosis was set by histopathological examination, which revealed gastric adenocarcinoma.

The trajectory of the medial longitudinal fasciculus in the human brain: A diffusion imaging-based tractography study.

The aim of this study is to investigate the trajectory of medial longitudinal fasciculus (MLF) and explore its anatomical relationship with the oculomotor nerve using tractography technique. The MLF and oculomotor nerve were reconstructed at the same time with preset three region of interests (ROIs): one set at the area of rostral midbrain, one placed on the MLF area at the upper pons, and one placed at the cisternal part of the oculomotor nerve. This mapping protocol was tested in an HCP-1065 template, 35 health subjects from Massachusetts General Hospital (MGH), 20 healthy adults and 6 brainstem cavernous malformation (BCM) patients with generalized q-sampling imaging (GQI)-based tractography. Finally, the 200 µm brainstem template from Center for In Vivo Microscopy, Duke University (Duke CIVM), was used to validate the trajectory of reconstructed MLF. The MLF and oculomotor nerve were reconstructed in the HCP-1065 template, 35 MGH health subjects, 20 healthy adults and 6 BCM patients. The MLF was in conjunction with the ipsilateral mesencephalic part of the oculomotor nerve. The displacement of MLF was identified in all BCM patients. Decreased QA, RDI and FA were found in the MLF of lesion side, indicating axonal loss and/or edema of displaced MLF. The reconstructed MLF in Duke CIVM brainstem 200 µm template corresponded well with histological anatomy. The MLF and oculomotor nerve were visualized accurately with our protocol using GQI-based fiber tracking. This GQI-based tractography is an important tool in the reconstruction and evaluation of MLF.

Iron accumulation in the oculomotor nerve of the progressive supranuclear palsy brain.

Abnormal iron accumulation around the substantia nigra (SN) is a diagnostic indicator of Parkinsonism. This study aimed to identify iron-related microarchitectural changes around the SN of brains with progressive supranuclear palsy (PSP) via postmortem validations and in vivo magnetic resonance imaging (MRI). 7 T high-resolution MRI was applied to two postmortem brain tissues, from one normal brain and one PSP brain. Histopathological examinations were performed to demonstrate the molecular origin of the high-resolution postmortem MRI findings, by using ferric iron staining, myelin staining, and two-dimensional laser ablation-inductively coupled plasma-mass spectrometry (LA-ICP-MS) imaging. In vivo iron-related MRI was performed on five healthy controls, five patients with Parkinson's disease (PD), and five patients with PSP. In the postmortem examination, excessive iron deposition along the myelinated fiber at the anterior SN and third cranial nerve (oculomotor nerve) fascicles of the PSP brain was verified by LA-ICP-MS. This region corresponded to those with high R2* values and positive susceptibility from quantitative susceptibility mapping (QSM), but was less sensitive in Perls' Prussian blue staining. In in vivo susceptibility-weighted imaging, hypointense pixels were observed in the region between the SN and red nucleus (RN) in patients with PSP, but not in healthy controls and patients with PD. R2* and QSM values of such region were significantly higher in patients with PSP compared to those in healthy controls and patients with PD as well (vs. healthy control: p = 0.008; vs. PD: p = 0.008). Thus, excessive iron accumulation along the myelinated fibers at the anterior SN and oculomotor nerve fascicles may be a pathological characteristic and crucial MR biomarker in a brain with PSP.

Oculomotor Nerve Palsy Due to Unusual Causes.

BACKGROUND: An isolated oculomotor nerve (CN III) palsy is a diagnostic concern because of the potential for serious morbidity or life-threatening causes. We present 5 unusual causes of oculomotor nerve palsy that escaped initial diagnosis in order to raise awareness of their associated features that will facilitate correct diagnosis. METHODS: This study consisted of a retrospective analysis of clinical features and imaging of 5 patients who were referred for neuro-ophthalmologic evaluation with presumed diagnosis of oculomotor nerve palsy of unknown reasons. RESULTS: A complete CN III palsy and an inferior division CN III palsy were diagnosed with a schwannoma in the cavernous sinus and orbital apex portion, respectively; a middle-aged woman with aberrant regeneration was found to have a small meningioma; an adult man with ptosis was diagnosed with cyclic oculomotor paresis with spasms; and a patient after radiation was diagnosed with neuromyotonia. CONCLUSIONS: Localizing the lesion of oculomotor nerve palsy and careful examination of the imaging is crucial. Aberrant regeneration, cyclic pupil changes, and past medical history of amblyopia, strabismus, or radiation are also very helpful for diagnosis.

A Rare Intracranial Collision Tumor of Meningioma and Metastatic Uterine Adenocarcinoma: Case Report and Literature Review.

BACKGROUND: A collision tumor is a rare entity consisting of 2 histologically distinct tumor types (benign or malignant) in the same anatomic location. This can occur from a tumor-to-tumor metastasis or as a result of 2 adjacent intracranial tumors colliding and growing together. To our knowledge, this is the first reported case of collision tumor with confirmed meningioma and uterine adenocarcinoma. Multiple mechanisms have been proposed for the facilitative growth of collision tumors, including local epigenetic signaling. Clinically, it is important to consider collision tumors in the differential diagnosis of a rapidly growing intracranial lesion in the setting of systemic cancer to provide optimal surgical and postoperative management. CASE DESCRIPTION: A 78-year-old, right-handed woman with a known 10-year history of stable meningioma presented for evaluation of a right sphenoid wing lesion. She had recently completed treatment of uterine papillary serous carcinoma with no evidence of disease on follow-up imaging. On presentation, there was significant progression of the meningioma resulting in brain compression and right third nerve palsy. The patient underwent urgent resection of the lesion. Pathology demonstrated a collision tumor with a combination of metastatic uterine papillary serous carcinoma and meningioma. CONCLUSIONS: It is important to consider a collision tumor when a patient with a benign intracranial lesion presents with rapid progression, even in the context of a systemic cancer that rarely metastasizes to the brain. Appropriate histopathologic assessment is crucial in these cases and can have a significant impact on treatment plan and prognosis.

Pituitary adenoma presenting as acute onset isolated complete third cranial nerve palsy without vision changes.

We report a case of isolated unilateral complete pupil involving third cranial nerve palsy due to pituitary adenoma with parasellar extension into the right cavernous sinus. The patient was referred to us from neurosurgery with sudden onset binocular vertical diplopia with complete ptosis, and mild right-sided headache of 5-day duration. Ocular examination revealed pupil involving third cranial nerve palsy in right eye while rest of the examination including automated perimetry was normal. MRI brain with contrast revealed a mass lesion with heterogenous enhancement in the sella suggestive of a pituitary macroadenoma with possible internal haemorrhage (apoplexy). In addition, the MRI showed lateral spread to the right cavernous sinus which was causing compression of the right third cranial nerve. The patient was systemically stable. This report highlights a unique case as the lesion showed a lateral spread of pituitary adenoma without compression of the optic chiasm or other cranial nerves.

A Study of Gene Expression Changes in Human Spinal and Oculomotor Neurons; Identifying Potential Links to Sporadic ALS.

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that causes compromised function of motor neurons and neuronal death. However, oculomotor neurons are largely spared from disease symptoms. The underlying causes for sporadic ALS as well as for the resistance of oculomotor neurons to disease symptoms remain poorly understood. In this bioinformatic-analysis, we compared the gene expression profiles of spinal and oculomotor tissue samples from control individuals and sporadic ALS patients. We show that the genes GAD2 and GABRE (involved in GABA signaling), and CALB1 (involved in intracellular Ca2+ ion buffering) are downregulated in the spinal tissues of ALS patients, but their endogenous levels are higher in oculomotor tissues relative to the spinal tissues. Our results suggest that the downregulation of these genes and processes in spinal tissues are related to sporadic ALS disease progression and their upregulation in oculomotor neurons confer upon them resistance to ALS symptoms. These results build upon prevailing models of excitotoxicity that are relevant to sporadic ALS disease progression and point out unique opportunities for better understanding the progression of neurodegenerative properties associated with sporadic ALS.

A clinical study of ocular motor nerve functions after petroclival meningioma resection.

BACKGROUND: Ocular motor dysfunction is one of the most common postoperative complications of petroclival meningioma. However, its incidence, recovery rate, and independent risk factors remain poorly explored. METHODS: A prospective analysis of 31 petroclival meningiomas was performed. Operative approaches were selected by utilizing a new 6-region classification of petroclival meningiomas we proposed. Two scores were used to evaluate the functions of the oculomotor and abducens nerves. Pearson correlation analysis and binary logistic regression analysis were used to identify independent risk factors for intraoperative oculomotor and abducens nerve injury. RESULTS: Postoperative new-onset dysfunctions in the pupillary light reflex and eye/eyelid movements as well as abducens paralysis were detected in eight (25.8%), ten (32.3%) and twelve (38.7%) cases, respectively. Their corresponding recovery rates after 6 months of follow-up were 75% (6/8), 80% (8/10), and 83.3% (10/12), respectively, and their mean times to start recovery were 4.03, 2.43, and 2.5 months, respectively. Tumor invasion into the suprasellar region/sphenoid sinus was the only risk factor for dysfunctions in both the pupillary light reflex (p = 0.001) and eye/eyelid movements (p = 0.002). Intraoperative utilization of the infratrigeminal interspace was the only risk factor for dysfunction in eyeball abduction movement (p = 0.004). CONCLUSIONS: Dysfunctions of the oculomotor and abducens nerves recovered within 6 months postoperatively. Tumor extension into the suprasellar region/sphenoid sinus was the only risk factor for oculomotor nerve paralysis. Eye/eyelid movements were more sensitive than the pupillary light reflex in reflecting nerve dysfunctions. Intraoperative utilization of the infratrigeminal interspace was the only risk factor for abducens nerve paralysis.

Apoptotic Markers in the Midbrain of the Human Neonate After Perinatal Hypoxic/Ischemic Injury.

Our previous postmortem studies on neonates with neuropathological injury of perinatal hypoxia/ischemia (PHI) showed a dramatic reduction of tyrosine hydroxylase expression (dopamine synthesis enzyme) in substantia nigra (SN) neurons, with reduction of their cellular size. In order to investigate if the above observations represent an early stage of SN degeneration, we immunohistochemically studied the expression of cleaved caspase-3 (CCP3), apoptosis inducing factor (AIF), and DNA fragmentation by using terminal deoxynucleotidyltransferase-mediated dUTP-biotin 3'-end-labeling (TUNEL) technique in the SN of 22 autopsied neonates (corrected age ranging from 34 to 46.5 gestational weeks), in relation to the severity/duration of PHI injury, as estimated by neuropathological criteria. No CCP3-immunoreactive neurons and a limited number of apoptotic TUNEL-positive neurons with pyknotic characteristics were found in the SN. Nuclear AIF staining was revealed only in few SN neurons, indicating the presence of early signs of AIF-mediated degeneration. By contrast, motor neurons of the oculomotor nucleus showed higher cytoplasmic AIF expression and nuclear translocation, possibly attributed to the combined effect of developmental processes and increased oxidative stress induced by antemortem and postmortem factors. Our study indicates the activation of AIF, but not CCP3, in the SN and oculomotor nucleus of the human neonate in the developmentally critical perinatal period.

Oculo-zygomatic nerve transfer for facial synkinesis: An anatomical feasibility study.

BACKGROUND: Patients with severe oro-ocular synkinesis often present with concomitant inefficient smile excursion on the affected site. In theory, oculo-zygomatic nerve transfer may decrease synkinesis and improve smile by redirecting nerve fibers to their target muscle. The aim of this study was to explore the feasibility of nerve transfer in human cadavers between a caudal branch innervating the orbicularis oculi to a cephalad branch innervating the zygomaticus major muscles. METHODS: Eighteen hemi-faces were dissected. Reach for direct coaptation of a caudal nerve branch innervating the orbicularis oculi muscle to a cephalad nerve branch innervating the zygomaticus major muscle was assessed. Measurements included total number of nerve branches as well as maximum dissection length. Nerve samples were taken from both branches at the site of coaptation and histomorphometric analysis for axonal count was performed. RESULTS: The number of sub-branches to the orbicularis oculi muscle was 3.1 ± 1.0 and to the zygomaticus major muscle 4.7 ± 1.2. The maximal length of dissection of the caudal nerve branch to the orbicularis oculi muscle was 28.3 ± 7.3 mm and for the cranial nerve branch to the zygomaticus major muscle 23.8 ± 6.5 mm. Transection and tension-free coaptation was possible in all cases but one. The average myelinated fiber counts per mm2 was of 5,173 ± 2,293 for the caudal orbicularis oculi branch and 5,256 ± 1,774 for the cephalad zygomaticus major branch. CONCLUSION: Oculo-zygomatic nerve transfer is an anatomically feasible procedure. The clinical value of this procedure, however, remains to be proven.

Congenital Multiple Ocular Motor Nerve Palsy Complicated by Splitting of the Lateral Rectus Muscle.

We report a case of congenital multiple ocular motor nerve palsy combined with splitting of the lateral rectus muscle (LR). A 59-year-old Japanese female was investigated for worsening esotropia after corrective surgery. She presented with left hypertropia (35Δ) and esotropia (45-50Δ). Orbital magnetic resonance imaging (MRI) showed reduced belly sizes in the superior rectus, inferior rectus, and superior oblique muscles and splitting of the LR, extending from the origin to the belly, in the left eye. Splitting of the LR belly was detected on MRI in a case of congenital multiple ocular motor nerve palsy.