Symptomatic Oculomotor Nerve Cyst in a 3-Year-Old Child: Case Report With Emphasis on Surgical Management

BACKGROUND AND IMPORTANCE Third nerve palsies in the pediatric population are most commonly caused by trauma, tumors, or vascular abnormalities. Cystic oculomotor nerve neuropathies, however, are rare. We report the case of a symptomatic cyst along and within the oculomotor nerve, which has not been described previously. CLINICAL PRESENTATION Here, we report a case of a 3-yr-old girl presenting with a progressive painless oculomotor nerve palsy. A magnetic resonance imaging revealed a cystic formation along the cisternal and cavernous course of the nerve. Due to lack of alternative treatment options, surgery was offered. Intraoperative direct nerve stimulation allowed for identification of a non-functional part of the cyst wall and open fenestration and biopsy were executed. Histopathology revealed neuritis. Serology was negative for various pathogens. The oculomotor palsy rapidly resolved. At a follow-up 5 yr after surgery, the girl is asymptomatic and the cisternal part of the cyst remains collapsed. CONCLUSION This is the first report of a symptomatic cyst along and within the oculomotor nerve treated effectively with open fenestration and decompression highlighting the importance of intraoperative neuromonitoring in cranial nerve surgery. Uncertainty remains regarding the etiology of this disease.

Case Report: Oculomotor Palsy With Cyclic Spasms in a Patient With Charcot-Marie-Tooth Disease Type 1

Oculomotor palsy with cyclic spasms is an extremely rare condition whose exact pathophysiology remains a mystery. We followed a boy from the onset of symptoms at the age of ten months until 15 years and documented the case with video oculography. In addition, he was diagnosed with hereditary motor and sensory neuropathy (Charcot-Marie-Tooth disease type 1). Although a pure coincidence cannot be ruled out, it is conceivable that the underlying demyelinating neuropathy of this patient rendered the oculomotor nerve more susceptible to damage.

Sudden Onset Diplopia Associated With Better Diabetes Control - Too Fast Is Not Too Good

Introduction: Treatment-induced neuropathy of diabetes (TIND) presenting as a new-onset peripheral neuropathy usually associated with autonomic dysfunction has been documented in literature. It can occur when there is a drop of more than 2% in HbA1C in a three month period. Worsening of existing peripheral neuropathy and retinopathy has also been observed suggesting a common pathophysiological mechanism. However, the onset of a cranial neuropathy temporally linked to intensified glycemic control has not been well reported. Case Report: A 32-year-old male patient presented to the emergency department with double vision, preceded by headache, and increased burning in his feet. No other neurologic symptoms were reported. Past medical history was significant for poorly controlled, labile type 1 diabetes mellitus. His home medications included gabapentin for peripheral neuropathy, long-acting and short-acting meal time insulin regimen. Physical examination was unremarkable except for a neurological exam which showed pupil-sparing third cranial nerve palsy. CT angiography of the head and neck revealed no acute ischemia, hemodynamically significant stenosis, aneurysm or dissection. MRI of the brain also showed no abnormality. Review of his prior HbA1c values showed a decrease of 3.6 % (14% to 10.4%) over 3 months. The patient was diagnosed with TIND presenting with simultaneous worsening of peripheral neuropathy and new onset right oculomotor nerve palsy. He was discharged on reduced doses of insulin, targeting a gradual decline in HbA1c after initial stabilization. The patient was also asked to liberalize his diet and was given an eye patch. While his peripheral neuropathy improved, there was not much improvement in his diplopia at a two week follow-up visit. Discussion: Our patient presented with worsening of peripheral neuropathy and pupil sparing oculomotor palsy. Secondary causes including CVA and aneurysm were excluded with brain imaging. Diabetes related microvascular disease presenting with pupil sparing oculomotor palsy is commonly seen in patients older than 50 years of age with uncontrolled diabetes and other vascular risk factors like hypertension and dyslipidemia. Absence of hypertension and hyperlipidemia, sudden onset of presentation and an intense control of DM as evidenced by rapid decline of A1C make TIND the likely cause for his presentation. The paradoxical worsening of his peripheral neuropathy also supports the diagnosis of TIND. Through this case, we highlight the importance of recognizing this entity as management differs significantly. Quality improvement metrics for practitioners in various institutions often include HbA1c target ranges in diabetic patients. Our case highlights the issues associated with rapid corrections of HbA1c and thus we advocate for a gradual decrease of 2 % every 3 months till target goals are reached.

Pretemporal Transcavernous Approach for Resection of an Orbito-Cavernous Oculomotor Schwannoma

The following operative video demonstrates surgical tenets and nuances of the pretemporal transcavernous approach in an unusual case of a 33-year-old patient suffering new onset diplopia and a third nerve palsy due to an orbito-cavernous oculomotor schwannoma. Near total resection was accomplished through an extended pterional craniotomy with pretemporal transcavernous exposure of her lesion, resulting in resolution of her preoperative oculomotor palsy and visual dysfunction. When combined with extended pterional and modified frontotemporal orbitozygomatic approaches, the pretemporal transcavernous approach provides excellent surgical access to the parasellar region including the superior orbital fissure and cavernous sinus. Meticulous dissection and early identification of tissue planes, including cranial nerve and vascular anatomy, allows for safe removal of tumors arising in this region.The link to the video can be found at: https://youtu.be/EuIRTP7wWBQ.

Complete Oculomotor Palsy after Influenza Vaccination in a Young Healthy Adult: A Case Report

Influenza vaccines are known to have a few neurological complications, such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, and acute disseminated encephalomyelitis. However, oculomotor palsy caused by influenza vaccination is extremely rare. We present a case report of a 25-year-old woman without any medical history who developed complete oculomotor palsy 2 weeks after influenza vaccination. Other possible causes of oculomotor nerve palsy, such as stroke, compressive lesions, infections, and autoimmune disorders, were eliminated by blood tests, cerebrospinal fluid examination, and imaging studies. Hence, influenza vaccine was considered as the likely cause.

Acute surgery for a case of superior vermian arteriovenous malformation producing raised venous pressure coexisting with basilar-superior cerebellar artery aneurysm presenting subarachnoid hemorrhage; Case report

Background: Superior vermian subtype of arteriovenous malformation (AVM) coexisting with proximal feeder aneurysm on basilar-superior cerebellar artery (BA-SCA) junction is an extremely rare situation. We experienced a case of this rare entity presenting with subarachnoid hemorrhage (SAH), and herein, introduce the outline and clinical features of this experience together with the actual surgical video. Case Description: A 54-year-old man SAH patient with severe headache, disturbance of consciousness, and left oculomotor palsy was urgently admitted to our hospital. Imaging examination demonstrated superior vermian AVM with BA-SCA aneurysm, and both lesions were treated through two different approaches (left pterional craniotomy in conjunction with zygomectomy, and left posterior interhemispheric occipital transtentorial approach) in acute phase of SAH. Both lesions were completely disappeared postoperatively and the patient’s postoperative course was favorable, without symptomatic cerebral vasospasm. Although slight oculomotor palsy remained, the patient recovered well and was transferred to a rehabilitation hospital for further improvement. Conclusion: In the cases of AVM coexisting with proximal feeder aneurysm, presenting with SAH, disorders of intracranial venous return associated with an AVM can be a vital hindrance to managing cerebral vasospasm; therefore, treating both lesions in the acute phase may lead to good outcomes.

Early Recurrence of an Infantile Endodermal Oculomotor Nerve Cyst following Surgical Fenestration: A Case Report

Introduction: Infantile endodermal oculomotor nerve cyst (EONC) is an extremely rare entity. There are very few pediatric cases reported in the literature, and as expected, oculomotor palsy is the most common presenting symptom. To date however, the risk of recurrence of these lesions following surgical intervention is unclear due to a lack of long-term radiological follow-up. Case Presentation: We present a case of a 13-month-old male patient with an EONC and detail his surgical fenestration and postoperative course. Somewhat surprisingly, re-expansion occurred within 6 months and remained stable 2 years later. Discussion: A surgical approach to fenestration of an EONC in an infant is possible and should be performed by an expert neurosurgeon. Early recurrence is underreported in the current literature, and we encourage longer term radiological surveillance of these lesions after surgery to optimize primary and recurrent management in the future.