Superior oblique palsy as the initial manifestation of anti-contactin-1 IgG4 autoimmune nodopathy: A case report.

Autoimmune nodopathy (AN) is a group of peripheral neuropathies caused by antibodies targeting the nodes of Ranvier or paranodes. It typically presents with sensory ataxia, distal limb weakness, and tremor, and often has a subacute onset, with limited response to immunoglobulin or corticosteroids. We report a case of anti-contactin-1 neuropathy initially manifesting as isolated superior oblique palsy, aiming to broaden the clinical spectrum of the disease. A 68-year-old male with well-controlled diabetes, hypertension, and hyperlipidemia developed acute binocular vertical diplopia, progressing over two months to include distal paresthesia, sensory ataxia, ageusia, and dysarthria. Concurrent nephrotic syndrome was identified. Nerve conduction studies supported demyelination. Despite treatment with intravenous methylprednisolone followed by long-term immunosuppression, some disability persisted. Serum archived during his admission tested positive for anti-contactin-1 IgG, with IgG4 as the predominant subclass, in the flow cytometry assay for AN. This case extends the clinical spectrum of AN. Some cases of isolated cranial nerve palsies, especially in the relevant context like nephrotic syndrome, may be attributed to AN. Prompt initiation of more effective therapies, such as rituximab, could significantly improve outcomes.

Torsion and clinical features in patients with acquired fourth cranial nerve palsy.

This retrospective study aimed to compare objective/subjective torsion and other clinical characteristics of patients with acquired trochlear nerve palsy. This study included 82 consecutive patients who were diagnosed with acquired fourth cranial nerve palsy between 2014 and 2021 and who were followed up for ≥ 6 months. The etiologies, ocular deviation, objective and subjective torsions were reviewed. The etiologies were classified as ischemic, traumatic, brain lesion, idiopathic, or other. The patients were classified into two groups according to the recovery state: full recovery and partial/no-recovery. We compared the torsion and clinical features based on the etiology and recovery state. The average age was 59.1 ± 11.1 years, and 58 (71.0%) of the patients were male. The most common cause was ischemic (n = 49, 59.7%) and other common causes included traumatic (n = 16, 19.5%), brain lesion (n = 8, 9.8%), idiopathic (n = 5, 6.1%) and others (n = 4, 4.9%). Of the 82 patients, 56 (68.3%) were assigned to the full recovery group, and 26 (31.7%) were assigned to the partial/no-recovery group. The average age and number of patients with ischemic causes of palsy were greater in the full recovery group (p = 0.026 and p < 0.000, respectively). The vertical deviation angle, tilted angle on the Lancaster red-green test (LRGT), proportion of patients who experienced subjective torsion on the LRGT, and head tilt were smaller in the full recovery group (p = 0.037, 0.042, 0.045, and 0.006, respectively). Ischemic trochlear nerve palsy, advanced age, a small deviation angle at the primary position, and few cases of excyclotorsion on LRGT were characteristic of the full recovery group of acquired unilateral trochlear nerve palsy patients.

Case report: isolated trochlear nerve palsy associated with posterior cerebral artery aneurysm.

We describe a case of isolated trochlear nerve palsy caused by an unruptured posterior cerebral artery (PCA) aneurysm in an 82-year-old male who consulted an ophthalmologist after developing diplopia. Magnetic resonance angiography showed a left PCA aneurysm in the ambient cistern, and T2WI showed an aneurysm compressing the left trochlear nerve to the cerebellar tentorium. Digital subtraction angiography revealed that the lesion was located between the left P2a segment. We attributed this isolated trochlear palsy to left PCA unruptured aneurysm pressure. Thus, we performed stent-assisted coil embolization. The aneurysm was obliterated, and trochlear nerve palsy improved completely.

Abducens and Trochlear Nerve Palsies After COVID-19 Vaccination: Report of Two Cases.

The authors report two cases of an abducens palsy and a trochlear nerve palsy, respectively, in two patients who received a coronavirus disease 2019 (COVID-19) vaccine 2 weeks previously. Given the lack of other symptoms, normal test results, and spontaneous resolution of the diplopia, a likely association with the COVID-19 vaccine was suggested. [J Pediatr Ophthalmol Strabismus. 2022;59(5):e50-e53.].

The etiologies of isolated fourth cranial nerve palsy: a 10-year review of 158 cases.

PURPOSE: To identify the etiologies of isolated fourth cranial nerve palsy in Ramathibodi hospital, Thailand. METHODS: Patients diagnosed with isolated fourth nerve palsy from January 1, 2009, through July 31, 2020 in Ramathibodi Hospital, were included in this retrospective, observational case series. The demographic data of patients, age at presentation, the etiologies of isolated fourth nerve palsy and neuroimaging results (if indicated) were recorded. RESULTS: We identified 154 unilateral and 4 bilateral cases of isolated fourth nerve palsy. Mean age at presentation was 38.89 ± 25.71 years old. Most of the unilateral cases were congenital (57.79%), with microvasculopathy (27.92%), intracranial neoplasm (8.44%) and other etiologies. Trauma with closed head injury was the most common etiology of bilateral cases (75%), followed by ruptured arteriovenous malformation (25%). Twenty-one of the 43 (48.84%) patients with microvasculopathy fourth nerve palsy underwent neuroimaging, with normal findings, and all patients' symptoms resolved within 6 months of symptom onset. CONCLUSIONS: In our series, most of the isolated fourth nerve palsy cases were congenital, followed in frequency by microvasculopathy and intracranial tumor, as in many studies. In cases of microvasculopathy, the clinical signs and symptoms resolved within 6 months in all cases: observation was sufficient, with no necessity for neuroimaging. However, neuroimaging should be considered in cases with atypical presentations, such as headache, periorbital pain, or if there is rapid progression or no recovery.

Unilateral Isolated Trochlear Nerve Palsy due to Ipsilateral Midbrain Infarction.

Ischemic stroke is a very rare etiology in cases of isolated trochlear nerve palsy, and no reports of ipsilateral trochlear nerve palsy caused by unilateral stroke have so far been published. However, we now report a case of isolated trochlear nerve palsy due to ipsilateral dorsal small midbrain infarction in a 70-year-old woman who presented with acute onset of diplopia. There were no other clinical manifestations, but brain magnetic resonance imaging revealed a small ischemic lesion in the right dorsal midbrain, showing that isolated trochlear nerve palsy can be caused by stroke.

The incidence and presumed aetiologies of fourth cranial nerve palsy in Korea: a 10-year nationwide cohort study.

AIMS: To investigate the incidence and presumed aetiologies of fourth cranial nerve (CN4) palsy in Korea METHODS: Using the nationally representative dataset of the Korea National Health Insurance Service-National Sample Cohort from 2006 to 2015, newly developed CN4 palsy cases confirmed by a preceding disease-free period of ≥4 years were identified. The presumed aetiology of CN4 palsy was evaluated based on comorbidities around the CN4 palsy diagnosis. RESULTS: Among the 1,108,292 cohort subjects, CN4 palsy newly developed in 390 patients during 10-year follow-up, and the overall incidence of CN4 palsy was 3.74 per 100,000 person-years (95% confidence interval, 3.38-4.12). The incidence of CN4 palsy showed a male preponderance in nearly all age groups, and the overall male-to-female ratio was 2.30. A bimodality by age-group was observed, with two peaks at 0-4 years and at 75-79 years. The most common presumed aetiologies were vascular (51.3%), congenital (20.0%), and idiopathic (18.5%). The incidence rate of a first peak for 0-4 years of age was 6.17 per 100,000 person-years, and cases in this group were congenital. The second peak incidence rate for 75-79 years of age was 11.81 per 100,000 person-years, and the main cause was vascular disease. Strabismus surgery was performed in 48 (12.3%) patients, most of whom (72.9%) were younger than 20 years. CONCLUSION: The incidence of CN4 palsy has a male predominance in Koreans and shows bimodal peaks by age. The aetiology of CN4 palsy varies according to age-groups.

Unilateral Fourth Nerve Palsy due to Presumed Metastatic Melanoma.

ABSTRACT: An 81-year-old man with a history of metastatic melanoma presented with sudden onset of painless, binocular vertical diplopia. The clinical examination was consistent with a right fourth nerve palsy. An MRI of the head revealed a mass dorsal to the right tectum at the level of the inferior colliculus. An MRI just 4 months prior did not show a lesion in that location. An MRA of the head did not show an aneurysm. This is a rare case of an isolated fourth nerve palsy believed to be due to metastatic melanoma compressing the nerve along the dorsal midbrain.

Unusual manifestation of heat stroke: Isolated trochlear nerve palsy.

Heat stroke is a life-threatening disease characterized by hyperthermia and neurological dysfunction. The central nervous system is highly sensitive to hyperthermia, which causes neurological complications due to the involvement of the cerebellum, basal ganglia, anterior horn cells, and peripheral nerves. Several studies reported about clinical symptoms and brain image findings of heat stroke. Isolated cranial nerve dysfunction caused by lacunar infarction is an extremely rare condition in patient with heat stroke. We experienced a rare case of trochlear nerve palsy due to midbrain infarction caused by heat stroke.

Fourth Nerve Palsy Due to Neurocysticercosis.

Neurocysticercosis is the most common disease of the nervous system caused by a parasite. Ophthalmological manifestations of cysticercosis typically are due to direct intraorbital or intraocular involvement, parenchymal brainstem involvement, or secondary to hydrocephalus or increased intracranial pressure. We describe a patient with a unilateral fourth nerve palsy, a rare presentation of neurocyticercosis.

Pituitary Apoplexy Accompanying Temporal Lobe Seizure as a Complication.

BACKGROUND: Pituitary apoplexy is an acute clinical syndrome caused by infarction and/or hemorrhage of pituitary adenoma, which typically presents with severe headache, visual deterioration, and endocrine abnormalities. However, temporal lobe seizure (and temporal lobe epilepsy) has not been viewed as a symptom of pituitary apoplexy in the literature. CASE DESCRIPTION: To elucidate further such a rare complication of temporal lobe seizure, we describe here the rare clinical manifestations of a 55-year-old previously healthy man with pituitary apoplexy harboring headache, combined palsies involving cranial nerves III to VI, endocrinologic disturbances, and temporal lobe seizure. In addition, we discuss the temporal lobe seizure (and temporal lobe epilepsy) associated with pituitary adenoma based on the literature. CONCLUSIONS: Although further accumulation of clinical data is needed, we would like to emphasize the importance of recognition of temporal lobe seizure caused by pituitary apoplexy, and to suggest that early surgery could be considered as an option in patients displaying such a rare complication.

A case of bilateral fourth nerve palsy associated with pseudotumor cerebri syndrome / Caso de paralisia bilateral de quarto nervo associada com síndrome do pseudotumor cerebral

ABSTRACT Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome.

RESUMO A sindrome do pseudotumor cerebral é uma síndrome enigmática caracterizada por aumento da pressão intracraniana sem causa definida. Na síndrome do pseudotumor cerebral, as paralisias de nervo oculomotor além da paralisia unilateral ou bilateral do sexto nervo craniano são raramente vistas. Nós reportamos o caso de uma paciente feminina de 25 anos com paralisia bilateral de quarto nervo craniano como resultado da síndrome do pseudotumor cerebral. Após tratamento médico para síndrome do pseudotumor cerebral, os sinais e sintomas oculares e sistêmicos da paralisia nervosa foram resolvidos. Em conclusão, a paralisia de quarto nervo craniano pode estar associada com síndrome de pseudotumor cerebral, portanto médicos devem considerar paralisias raras de nervo oculomotor, mesmo bilateralmente, em pacientes com síndrome do pseudotumor cerebral.

Isolated Trochlear Nerve Palsy in a Patient with Superior Cerebellar Rete Mirabile.

BACKGROUND: Rete mirabile is a very rare vascular malformation and superior cerebellar artery (SCA) rete mirabile is not reported previously. We report a new case of rete mirabile of SCA initially detected by magnetic resonance imaging and transfemoral cerebral angiography. CASE DESCRIPTION: This report illustrates the case of a 58-year-old man who presented with vertical diplopia. Brain magnetic resonance angiography and transfemoral cerebral angiography revealed a rete mirabile of SCA and 3-dimensional volume isotropic turbo spin echo acquisition, brain magnetic resonance imaging sequence, demonstrated that the cisternal segment of the ipsilateral trochlear nerve was compressed by this vascular malformation. We assumed that his cranial nerve palsy was caused by the rete mirabile of the right SCA. During the 8 weeks presence of diplopia, the patient was observed and the symptom was relieved spontaneously. CONCLUSIONS: We provide a first report in the literature of rete mirabile involving the SCA and suggest a descriptive knowledge of rete mirabile for clinicians during decision-making of treatment.

Trochlear nerve palsy associated with Moyamoya disease.

A 48-year-old woman with intracranial hemorrhage at the right hemisphere and the right midbrain was diagnosed as Moyamoya disease (MMD). While restoring consciousness, she complained of diplopia. The left hypertropia with a compensatory right head tilt was noted. The follow up brain image showed the evidence of past hemorrhage at the level of the right inferior colliculus. In this case, the hemorrhage directly damaged the right trochlear nucleus or intra-axial trochlear nerve before decussation and caused left (contralateral) hypertropia. To our knowledge, no report has been described of the trochlear nerve palsy in hemorrhagic Moyamoya disease. Here, we present a case of the patient with trochlear nerve palsy after hemorrhagic MMD and summarize the characteristics of trochlear nerve palsy according to its lesion sites.

A case of bilateral fourth nerve palsy associated with pseudotumor cerebri syndrome.

Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome.