Bevacizumab-induced isolated oculomotor nerve palsy in glioblastoma multiforme

Introduction: Bevacizumab, a monoclonal antibody against the vascular endothelial growth factor receptor, is the standard treatment of recurrent glioblastoma multiforme. In addition to common systemic side effects of bevacizumab, there are rare cases of cranial nerve palsy. Case report: We report a case of transient oculomotor nerve palsy after systemic administration of bevacizumab. Twenty-four hours after the systemic infusion of bevacizumab, transient oculomotor nerve palsy developed in a 49-year-old male patient. In the cranial MRI, there was no malignancy-related progression. Management and outcome: Bevacizumab treatment was discontinued. Methylprednisolone was started considering that bevacizumab increased the inflammatory response. Oculomotor nerve palsy resolved in 14 days. Discussion: There are many side effects of bevacizumab whose mechanisms of action have not been fully explained. Cranial nerve involvement is rarely reported. Our case is the first reported case of bevacizumab-induced oculomotor nerve palsy.

A taxonomy for brainstem cavernous malformations: subtypes of midbrain lesions

OBJECTIVE Anatomical taxonomy is a practical tool that has successfully guided clinical decision-making for patients with brain arteriovenous malformations. Brainstem cavernous malformations (BSCMs) are similarly complex lesions that are difficult to access and highly variable in size, shape, and position. The authors propose a novel taxonomy for midbrain cavernous malformations based on clinical presentation (syndromes) and anatomical location (identified with MRI). METHODS The taxonomy system was developed and applied to an extensive 2-surgeon experience over a 30-year period (1990–2019). Of 551 patients with appropriate data who underwent microsurgical resection of BSCMs, 151 (27.4%) had midbrain lesions. These lesions were further subtyped on the basis of predominant surface presentation identified on preoperative MRI. Five distinct subtypes of midbrain BSCMs were defined: interpeduncular (7 lesions [4.6%]), peduncular (37 [24.5%]), tegmental (73 [48.3%]), quadrigeminal (27 [17.9%]), and periaqueductal (7 [4.6%]). Neurological outcomes were assessed using modified Rankin Scale (mRS) scores. A postoperative score ≤ 2 was defined as a favorable outcome; a score > 2 was defined as a poor outcome. Clinical and surgical characteristics and neurological outcomes were compared among subtypes. RESULTS Each midbrain BSCM subtype was associated with a recognizable constellation of neurological symptoms. Patients with interpeduncular lesions commonly presented with ipsilateral oculomotor nerve palsy and contralateral cerebellar ataxia or dyscoordination. Peduncular lesions were associated with contralateral hemiparesis and ipsilateral oculomotor nerve palsy. Patients with tegmental lesions were the most likely to present with contralateral sensory deficits, whereas those with quadrigeminal lesions commonly presented with the features of Parinaud syndrome. Periaqueductal lesions were the most likely to cause obstructive hydrocephalus. A single surgical approach was preferred (> 90% of cases) for each midbrain subtype: interpeduncular (transsylvian-interpeduncular approach [7/7 lesions]), peduncular (transsylvian-transpeduncular [24/37]), tegmental (lateral supracerebellar-infratentorial [73/73]), quadrigeminal (midline or paramedian supracerebellar-infratentorial [27/27]), and periaqueductal (transcallosal-transchoroidal fissure [6/7]). Favorable outcomes (mRS score ≤ 2) were observed in most patients (110/136 [80.9%]) with follow-up data. No significant differences in outcomes were observed between subtypes (p = 0.92). CONCLUSIONS The study confirmed the authors’ hypothesis that taxonomy for midbrain BSCMs can meaningfully guide the selection of surgical approach and resection strategy. The proposed taxonomy can increase diagnostic acumen at the patient bedside, help identify optimal surgical approaches, enhance the consistency of clinical communications and publications, and improve patient outcomes.

Case Report: Isolated, unilateral oculomotor palsy with anti-GQ1b antibody following COVID-19 vaccination

Neurological complications following vaccinations are extremely rare, but cannot be eliminated. Here, we report the first case of unilateral oculomotor nerve palsy (ONP) with anti-GQ1b antibody after receiving the Pfizer-BioNTech COVID-19 (BNT162b2) mRNA vaccine. A 65-year-old man developed diplopia and ptosis in the right eye 17 days after vaccination, without preceding infection. Neurological examination revealed mild blepharoptosis, limitation of adduction, and vertical gaze on the right side. Increased levels of anti-GQ1b ganglioside antibody in the serum and albuminocytologic dissociation in the cerebrospinal fluid were detected. Cranial magnetic resonance imaging showed swelling and enhancement of the right oculomotor nerve. The patient was diagnosed with right ONP accompanied with anti-GQ1b antibody, and intravenous immunoglobulin (IVIG) therapy for 5 days was administered. The limitation of adduction and vertical gaze improved, and ptosis markedly resolved after IVIG treatment. Given the temporal sequence of disease progression, laboratory findings, and a favorable response to IVIG, a causal relationship cannot be ruled out between the occurrence of ONP and COVID-19 immunization. Since immunomodulatory treatments significantly hasten the recovery and minimize the residual symptoms in anti-GQ1b antibody syndrome, clinicians should be aware of this clinical condition following COVID-19 vaccination.

Tolosa-Hunt Syndrome Presenting as Cluster-Like Headache

A 48-year-old man came with a left-sided headache that was compatible with diagnostic criteria of cluster headache. Left oculomotor nerve palsy developed 2 weeks after headache onset. Magnetic resonance imaging showed wall thickening and enhancement by contrast material in the lateral aspect of the left cavernous sinus, consistent with a possible inflammatory lesion. The patient reported the almost complete remission of the pain and diplopia after steroid therapy. We speculate that Tolosa-Hunt syndrome should be included as a cause of cluster-like headaches.

Recurrent painful ophthalmoplegic neuropathy: a cause for recurrent third nerve palsy in a child

Recurrent painful ophthalmoplegic neuropathy (RPON), previously called ophthalmoplegic migraine, is a rare condition characterised by recurrent episodes of headache and ophthalmoplegia. We report a case of 11-year-old girl with recurrent painful ophthalmoplegia due to isolated right oculomotor nerve involvement. MR brain imaging showed enhancing lesion of cisternal segment of right oculomotor nerve. A possibility of Tolosa Hunt syndrome was considered and she was treated with glucocorticoids, followed by azathioprine due to recurrence. In the fourth episode, she developed migraine headache followed by right third nerve palsy, after which the diagnosis was revised to RPON. She was started on flunarizine along with short-term glucocorticoids. At 1-year follow-up, she remained asymptomatic. RPON should be considered in patients with recurrent third nerve palsy to avoid inadvertent long-term exposure to immunosuppressive agents.

Unilateral Oculomotor Nerve Palsy Associated with Leptomeningeal Carcinomatosis and Intracranial Hypertension

Meningeal carcinomatosis is caused by cancer cells invading the meninges and can cause cranial nerve palsies or intracranial hypertension. Intracranial hypertension can present various symptoms such as headache, visual loss, diplopia and may rarely include unilateral cranial nerve palsy. We report a 57-year-old female with leptomeningeal carcinomatosis and intracranial hypertension who presented as unilateral oculomotor nerve palsy.

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.