Factors affecting resolution of oculomotor nerve palsy following endovascular embolization of posterior communicating artery aneurysms / Factores que influyen en el tratamiento de la parálisis oculomotora con embolización endovascular de aneurismas comunicantes posteriores

Purpose: To investigate the effect of endovascular embolization of posterior communicating artery (Pcom) aneurysms on concomitant oculomotor nerve palsy (OMNP) and factors affecting the effect of treatment. Materials and methods: Patients with the Pcom aneurysms concomitant with OMNP were retrospectively enrolled for endovascular treatment of the aneurysms. All patients had the endovascular management. The clinical effect, degree of OMNP, size of the aneurysm, type of treatment, subarachnoid hemorrhage (SAH), and time from onset to treatment were analyzed on the resolution of OMNP. Results: Ninety-six patients with 99 Pcom aneurysms were enrolled and treated endovascularly, with the success rate of 100%. Immediately after endovascular treatment, 75 aneurysms (75.75%) got complete occlusion, and 24 (24.24%) nearly complete occlusion. Followed up for 3–18 (mean 8.52 ± 0.56) months, complete resolution of the OMNP was achieved in 63 patients (65.63%), partial resolution in 21 (21.88%), and non-recovery in the other 12 (12.50%). The degree of OMNP at onset, SAH, and time from onset to treatment were significantly (P < 0.05) correlated with the resolution of OMNP. Univariate analysis revealed that younger age of the patient, degree of OMNP at onset, presence of subarachnoid hemorrhage, and time from disease onset to treatment were significantly (P < 0.05) associated with the recovery of OMNP. Multivariate analysis revealed that the younger age, degree of OMNP at onset, and time from disease onset to treatment were significantly (P < 0.05) associated with the recovery of OMNP. Conclusion: Endovascular embolization of Pcom aneurysms concomitant with OMNP can effectively improve the OMNP symptoms, especially for patients with moderate and a shorter history of OMNP. Younger age, degree of oculomotor nerve palsy at onset, and time from onset to treatment may significantly affect recovery of oculomotor nerve palsy.(AU)

Objetivo: Investigar la eficacia de la embolización intravascular del aneurisma de comunicación posterior (Pcom) en pacientes con parálisis oculomotora (OMNP) y los factores que influyen en la eficacia. Materiales y métodos: Se analizaron retrospectivamente los datos clínicos de la terapia intravascular en pacientes con aneurismas Pcom con OMNP. Todos los pacientes recibieron tratamiento intravascular. Se analizaron los efectos de la eficacia clínica, el grado de OMNP, el tamaño del aneurisma, el método de tratamiento, la hemorragia subaracnoidea y el tiempo desde el inicio hasta el tratamiento en la regresión de OMNP.Resultados: Un total de 96 pacientes con 99 aneurismas Pcom fueron tratados con éxito. Inmediatamente después del tratamiento intravascular, 75 casos (75,75%) de aneurismas fueron completamente ocluidos y 24 casos (24,24%) casi completamente ocluidos. Durante el seguimiento de 3 a 18 meses (promedio: 8,52 ± 0,56 meses), se logró la resolución completa en 63 casos (65,63%), la resolución parcial en 21 (21,88%) y la no recuperación en los otros 12 (12,50%). El grado de OMNP al inicio, la hemorragia subaracnoidea y el tiempo de inicio a tratamiento se correlacionaron significativamente con la resolución de la OMNP (p < 0,05). El análisis univariado mostró que la menor edad del paciente, el grado de OMNP, la presencia de hemorragia subaracnoidea y el tiempo transcurrido desde el inicio de la enfermedad hasta el tratamiento se correlacionaron significativamente con la recuperación de OMNP (p < 0,05). Conclusión: La embolización intravascular del aneurisma Pcom combinada con OMNP puede mejorar eficazmente los síntomas de OMNP, especialmente en pacientes con OMNP a corto y mediano plazo. La edad temprana, el grado de parálisis del nervio oculomotor al inicio y el tiempo desde el inicio hasta el tratamiento tuvieron un efecto significativo en la recuperación de la parálisis del nervio oculomotor.(AU)

Intravascular Large B-cell Lymphoma Diagnosed by Nasal Biopsy in a Patient Presenting with Bilateral Ptosis and Ophthalmoplegia.

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of lymphoma, involving the lumen of predominantly small blood vessels, especially capillaries. The orbit is an uncommon site of involvement for IVLBCL, and diagnosis before autopsy is even more rare as most cases are established post-mortem. Herein, the authors describe a 73-year-old male who presented with 3 weeks of progressive bilateral ptosis and ophthalmoplegia. Computed tomography (CT) and subsequent magnetic resonance imaging (MRI) revealed diffuse abnormal thickening and enhancement of bilateral orbital apices, superior orbital fissures, and cavernous sinus, along with persistent focal opacification of the left frontal and ethmoid sinuses. Infectious and inflammatory workup of serum and cerebrospinal fluid was negative. Ethmoidal sinus and middle turbinate biopsy confirmed intravascular large B-cell lymphoma and the patient was started on R-CHOP chemotherapy regimen.

Odontogenic Lemierre's syndrome with septic superior ophthalmic vein and cavernous sinus thrombophlebitis complicated by blindness and ophthalmoplegia.

Lemierre's syndrome is a triad consisting of oropharyngeal infection, internal jugular vein thrombophlebitis, and systemic embolisation typically involving lung and brain. Orbital involvement in this life-threatening condition is rare but potentially blinding and may be an indicator of intracranial involvement. We describe a case of odontogenic Lemierre's syndrome complicated by extensive orbital and intracranial septic venous thrombosis, with optic and cranial neuropathy resulting in monocular blindness and ophthalmoplegia. A multidisciplinary approach with abscess drainage, antibiotic and antithrombotic therapy, and close radiological monitoring was critical for preserving contralateral vision and neurological function.

A Case of Orbital Apex Syndrome as a Complication of Herpes Zoster Ophthalmicus.

We present a case of herpes zoster ophthalmicus (HZO) with a rare complication of orbital apex syndrome (OAS) manifesting as optic perineuritis with multiple cranial nerve palsies. A 65-year-old with COPD presented to the hospital with a vesicular rash involving his left eyelid. He was admitted for HZO and a concurrent COPD exacerbation. The HZO was treated with antivirals and the COPD exacerbation was treated with corticosteroids. On hospital day three, he developed left-sided ptosis, ophthalmoplegia, and a mid-dilated fixed pupil. MRI of the brain demonstrated enhancement of the left optic nerve sheath, rectus muscles, and periorbital soft tissues. He was diagnosed with OAS and treated with an increased dose of corticosteroids. After two months, his orbital symptoms resolved. This case is unique because the patient developed HZO in the setting of corticosteroid treatment for a COPD exacerbation, and his HZO progressed to OAS despite proper initiation of antiviral therapy.

Orbital apex syndrome secondary to herpes zoster ophthalmicus: Clinical features and outcomes case report and systematic review.

BACKGROUND: This study aimed to describe the clinical features of patients with orbital apex syndrome (OAS) as a complication of herpes zoster ophthalmicus (HZO) and to identify factors associated with poor visual acuity outcomes. METHODS: We performed a systematic review and retrospective analysis of the clinical characteristics and outcomes of patients with OAS secondary to HZO reported in the literature over 42 years (1978-2020). RESULTS: We analysed 21 cases, 20 of which were identified in the literature, together with our patient. Their median age was 65 years, with equal involvement in both sexes. The median onset of OAS due to HZO was 10 days (range 1-28 days). The median time of treatment initiation was five days (range 1-21 days). All patients presented with reduced visual acuity, complete ophthalmoplegia, and ptosis. Most patients (17/21, 80.95%) were treated with systemic antiviral and corticosteroid therapy. Three (3/21, 14.29%) patients were immunocompromised. Recovery for ophthalmoplegia (19/21, 90.48%) and ptosis (16/21, 76.19%) was good. Half of the patients (9/18, 50%) showed poor vision recovery. Starting treatment more than 72 h after HZO onset (p = 0.045) was more likely to cause poor vision recovery. CONCLUSION: OAS is a rare, serious, and potentially late complication of HZO and continued observation up to and perhaps beyond four weeks is justifiable, if not encouraged. Early initiation of treatment with systemic antiviral and/or corticosteroids within 72 h of onset of HZO appears beneficial for the recovery of visual acuity.

Herpes Zoster Opthalmicus-Related Ophthalmoplegia: Anatomical, Pathogenetic, and Therapeutic Perspectives.

OBJECTIVE: To investigate the anatomical, pathogenetic, and pharmacological characteristics of herpes zoster ophthalmicus (HZO)- related ophthalmoplegia. METHODS: Case report-based systematic review was performed. RESULTS: This study included 96 patients (54 [56.25%] women and 42 [43.75%] men [P = 0.221]). The mean age at presentation was 64.32 ± 17.48 years. All the patients included in the study had HZO- related ophthalmoplegia, with rash presenting as initial symptom in 87 (90.62%) cases, and diplopia in 9 (9.38%) cases. Thirty-seven (38.54%) patients achieved complete recovery, whereas 59 (61.46%) patients had permanent ophthalmoplegia. Females recovered in 26/54 cases and males in 11/42 cases (P = 0.028). Recovery rates after peroral versus intravenous antivirals (15/38 versus 19/46) and > 10 days versus ≤10 days antiviral treatment (22/54 versus 12/30) did not significantly differ ( P = 0.865 and P = 0.947, respectively). immunocompetent patients treated with corticosteroids had significantly better recovery rates compared to immunodeficient counterparts (17/34 [50.00%] and 5/22 [22.73%], respectively [ P = 0.041]). CONCLUSIONS: The outcome of HZO-related ophthalmoplegia is associated with gender, immune status, corticosteroid use, and time of antiviral treatment initiation.

Case Report: Isolated facial and trigeminal nerve palsy without ataxia in anti-GQ1b antibody syndrome secondary to Mycoplasma pneumonia.

The presence of anti-GQ1b antibodies in serum or cerebrospinal fluid is a diagnostic indicator of the Miller-Fisher variant of Guillain-Barré syndrome (GBS), whereas anti-GQ1b antibody syndrome is rarely presented as acute bilateral pain in the cheeks and masticatory muscle fatigue without ophthalmoplegia, ataxia, or limb weakness. Here, we report a case of a female patient diagnosed with GBS characterized only by the involvement of the facial and trigeminal nerves who was positive for serum anti-GQ1b antibodies secondary to Mycoplasma pneumoniae infection. The patient was treated with macrolide antibiotics and neurotrophic drugs, and her symptoms were significantly alleviated after 1 month. This case indicates a new clinical presentation of GBS and anti-GQ1b antibody syndrome with a differential diagnosis of multiple cranial nerve damage of which neurological physicians should be aware. Positive anti-GQ1b antibodies secondary to infection were observed in this case, and antibiotic treatment resulted in a favorable prognosis. The specific underlying mechanism requires further investigation.

Orbital giant cell myositis is an unusual and potentially lethal cause of bilateral ophthalmoplegia - A case report and literature review.

We present a case of orbital giant cell myositis (OGCM), presenting with bilateral subacute progressive ophthalmoplegia and optic nerve dysfunction. An early extraocular muscle biopsy confirmed the diagnosis and guided appropriate management. Comprehensive investigation excluded any underlying systemic disease, including myocarditis. Twenty two months after presentation, the patient remains well on azathioprine with complete resolution of orbital signs.

Rhino-orbital mucormycosis during steroid therapy in COVID-19 patients: A case report.

PURPOSE: To report two cases of COVID-19 under treatment with a corticosteroid; in one case rhino-orbitocerebral mucormycosis and in another one rhino-orbital mucormycosis developed. CASE PRESENTATION: A 40-year old woman and a 54-year old man with severe COVID-19 underwent corticosteroid therapy for immune-related lung injuries. The first case presented with a bilateral visual loss and complete ophthalmoplegia of the right eye. The second case presented with vision loss, proptosis, orbital inflammation, and complete ophthalmoplegia on the left side. Histopathologic, nasal endoscopic examinations, and radiologic findings confirmed mucormycosis in both patients. The patients denied orbital exenteration and were managed with systemic amphotericin B and daily endoscopic sinus debridement and irrigation with diluted amphotericin B. Because of the intracranial space involvement, the first case died. The second case was successfully managed surgically and medically. CONCLUSION: Rhino-orbital/cerebral mucormycosis may be developed in COVID-19 patients under treatment with corticosteroid, and requires prompt diagnosis and management.

A case of Graves' disease presenting with internal ophthalmoplegia during methylmercaptoimidazole treatment.

A 28-year-old Japanese woman positive for TSH receptor antibody and anti-nuclear antibody complained of difficulty seeing nearby objects, severe throbbing retro-orbital pain, diplopia, blepharoptosis and upward gaze palsy when she became hypothyroid during treatment with 30 mg methylmercaptoimidazole for Graves' hyperthyroidism. Brain magnetic resonance imaging revealed slightly swollen bilateral inferior rectus muscles, suggesting the external ophthalmoplegia due to the muscle pathology commonly encountered in Graves' disease. The retro-orbital pain was associated with marked accommodation failure and the pupillary abnormalities. The left and/or right eye showed intermittent, asymmetric and fluctuating mydriasis, being unresponsive to ordinary light but slowly responsive to strong sunlight and slowly responsive in a dark room. During the 5-year period, mydriasis was observed 9 times on both sides, 11 times only on the right side and 4 times only on the left side. Internal ophthalmoplegia with tonic pupils and accommodation failure affecting both the pupillary sphincter muscle and ciliary muscle due to damage to the parasympathetic outflow to these muscles was suggested. Autoimmune mechanism and/or the mechanism underlying channelopathy affecting the ciliary ganglion or short ciliary nerves might be responsible for this fluctuating complication. This very rare panophthalmopathy affecting both external and internal muscles occurred when the patient was suffering from iatrogenic hypothyroidism during the 30 mg methylmercaptimidazole treatment for Graves' disease.

Immune-mediated ophthalmoparesis with anti-GD1a antibodies.

A young woman presented to neurology with a 1 month history of progressive diplopia on lateral gaze and a 1 week history of headaches. On examination she was found to have complex ophthalmoparesis with binocular horizontal diplopia, failure of abduction bilaterally and limited upgaze with convergence-retraction nystagmus. The rest of the neurological examination was normal. She was admitted for investigations: blood, CT brain, MR brain and lumbar puncture results were normal. Anti-GD1a antibodies were strongly positive; anti-GM1, anti-GM2 and anti-GD1b were also positive. On follow-up 3 weeks later, the complex ophthalmoplegia persisted. It was decided to treat with intravenous immunoglobulins (IVIgs) with good response but recurrence at 2 weeks post infusion. She was treated with 4 weekly IVIg courses and remains responsive and controlled over 1 year since presentation but becomes symptomatic in the week running up to each dose; thus, disease modifying treatment is currently being considered.

Transient oculomotor paralysis after cerebral angiography: A case report.

RATIONALE: A special case of transient oculomotor nerve palsy after cerebral angiography. PATIENT CONCERNS: A 55-year-old man developed oculomotor nerve dysfunction after right radial artery puncture angiography. DIAGNOSES: Cerebral angiography-induced oculomotor nerve palsy. INTERVENTIONS: According to the patient's disease state, intravenous drip of dexamethasone 10 mg/d. OUTCOMES: Magnetic resonance imaging (MRI) showed no abnormalities, and the patient recovered completely after 48 hours of hormone therapy. LESSONS: Transient eye palsy caused by contrast agent encephalopathy is a clinically rare neurological dysfunction caused by adverse effects of contrast agents. Early prevention and correct treatment are critical.

A Yeast-Based Screening Unravels Potential Therapeutic Molecules for Mitochondrial Diseases Associated with Dominant ANT1 Mutations.

Mitochondrial diseases result from inherited or spontaneous mutations in mitochondrial or nuclear DNA, leading to an impairment of the oxidative phosphorylation responsible for the synthesis of ATP. To date, there are no effective pharmacological therapies for these pathologies. We performed a yeast-based screening to search for therapeutic drugs to be used for treating mitochondrial diseases associated with dominant mutations in the nuclear ANT1 gene, which encodes for the mitochondrial ADP/ATP carrier. Dominant ANT1 mutations are involved in several degenerative mitochondrial pathologies characterized by the presence of multiple deletions or depletion of mitochondrial DNA in tissues of affected patients. Thanks to the presence in yeast of the AAC2 gene, orthologue of human ANT1, a yeast mutant strain carrying the M114P substitution equivalent to adPEO-associated L98P mutation was created. Five molecules were identified for their ability to suppress the defective respiratory growth phenotype of the haploid aac2M114P. Furthermore, these molecules rescued the mtDNA mutability in the heteroallelic AAC2/aac2M114P strain, which mimics the human heterozygous condition of adPEO patients. The drugs were effective in reducing mtDNA instability also in the heteroallelic strain carrying the R96H mutation equivalent to the more severe de novo dominant missense mutation R80H, suggesting a general therapeutic effect on diseases associated with dominant ANT1 mutations.

Disseminated lymphoblastic lymphoma revealed by an isolated third cranial nerve palsy.

Dysfunction of the third cranial nerve can be provoked by a number of different conditions. An isolated cranial neuropathy as a first clinical sign of a non-Hodgkin lymphoma is very infrequent. We represent here an atypical case of lymphoblastic lymphoma revealed by an isolated third cranial nerve palsy. The patient was managed by alternating cycles of cyclophosphamide, vincristine, and prednisone. She made a full recovery with a complete resolution of the symptomatology.

Resolved External Ophthalmoplegia and Hearing Loss in Wernicke's Encephalopathy With Thiamine Replacement.

BACKGROUND: Wernicke encephalopathy (WE) is classically described by a clinical triad consisting of confusion, ataxia, and ophthalmoplegia, but recent reports emphasize a history of malnutrition along with 2 elements of the WE triad (Caine's criteria) to enhance diagnostic sensitivity. The ophthalmoplegia, vestibular, and auditory expeditious improvement with intravenous thiamine usually confirms the diagnosis; serum levels generally provide additional diagnostic certainty. METHODS: Here, we discuss the case of a woman with a distant history of gastric sleeve, poor nutrition and protracted vomiting, who developed acute confusion, imbalance, near-total external ophthalmoplegia (EO), and hearing loss. The baseline thiamine level was 28 πmol/L (Normal: 70-180 πmol/L). We performed serial neurological, vestibular, and audiological examination to document over 5 days, the effect of intravenous (IV) thiamine, and again at 3 months with continued oral supplementation. We provide serial documentation with photographs and video recording of oculomotor abnormalities, audiometric testing, and a video of horizontal head impulse testing, and imaging findings. RESULTS: Over the course of 5 days of IV thiamine supplementation, we demonstrate our patient's resolution of near complete EO. We assessed vestibular paresis with horizontal head impulse testing, after complete resolution of the EO. The initially positive bilateral h-HIT showed decreased gain and overt corrective saccades, it clinically resolved by day 5, but video h-HIT testing demonstrated persistent decreased horizontal vestibulo-ocular reflex (VOR) gain and covert horizontal saccades, which persisted at the 3-month examination. By contrast, the vertical VOR gain was normal without corrective saccades. Bedside audiometry completed during the acute phase demonstrated severely restricted auditory speech comprehension, which normalized 3 months later. Severe truncal ataxia improved as well. CONCLUSIONS: This case is an example of how awareness of the variations in the clinical presentation of WE can be crucial in achieving an early diagnosis and obtaining better outcomes. A history of the poor nutritional status can be an important clue to aid in this early diagnosis.

The Use of Systemic Steroids in the Treatment of Herpes Zoster Ophthalmicus-Related Ophthalmoplegia: Case Report and Case Meta-analysis.

PURPOSE: The role of systemic steroids in the treatment of ophthalmoplegia in the setting of herpes zoster ophthalmicus (HZO) is controversial. We conducted a case report-based meta-analysis to investigate the role of systemic steroid in the recovery of efferent dysfunctions in HZO. DESIGN: Case-report based meta-analysis. METHOD: We report a case of herpes zoster ophthalmicus-related ophthalmoplegia (HZORO) in which systemic steroid led to complete resolution of external ophthalmoplegia. We further identified subjects from published cases of HZO-related ophthalmoplegia by searching PubMed and Google Scholar, which elicited 42 articles (49 cases) after excluding those younger than 18 years or with incomplete follow-up data. With the present case, a total of 50 cases are included in the analysis. Main outcome measure is the recovery of efferent dysfunction at the last known follow-up, coded as 1 for complete recovery or 0 for noncomplete recovery. We performed multivariable linear regression and Cox proportional hazards analysis to determine the contribution of steroid duration on the status of complete recovery. RESULTS: Multivariable linear regression showed significant association between duration of steroid treatment and status of complete recovery (P < .001). Cox proportional hazards analysis showed a hazard ratio of 1.1 (P = .04), indicating that longer treatment duration increased chance of complete recovery. Age, gender, and initial steroid dose did not contribute significantly to recovery status. CONCLUSION: Our meta-analysis suggests that extended steroid taper may aid the recovery of ophthalmoplegia in the setting of HZO and should be investigated further in the future.

CANOMAD: a neurological monoclonal gammopathy of clinical significance that benefits from B-cell-targeted therapies.

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, immunoglobulin M [IgM] paraprotein, cold agglutinins, and disialosyl antibodies) is a rare syndrome characterized by chronic neuropathy with sensory ataxia, ocular, and/or bulbar motor weakness in the presence of a monoclonal IgM reacting against gangliosides containing disialosyl epitopes. Data regarding associated hematologic malignancies and effective therapies in CANOMAD are scarce. We conducted a French multicenter retrospective study that included 45 patients with serum IgM antibodies reacting against disialosyl epitopes in the context of evocating neurologic symptoms. The main clinical features were sensitive symptoms (ataxia, paresthesia, hypoesthesia; n = 45, 100%), motor weakness (n = 18, 40%), ophthalmoplegia (n = 20, 45%), and bulbar symptoms (n = 6, 13%). Forty-five percent of the cohort had moderate to severe disability (modified Rankin score, 3-5). Cold agglutinins were identified in 15 (34%) patients. Electrophysiologic studies showed a demyelinating or axonal pattern in, respectively, 60% and 27% of cases. All patients had serum monoclonal IgM gammopathy (median, 2.6 g/L; range, 0.1-40 g/L). Overt hematologic malignancies were diagnosed in 16 patients (36%), with the most frequent being Waldenström macroglobulinemia (n = 9, 20%). Forty-one patients (91%) required treatment of CANOMAD. Intravenous immunoglobulins (IVIg) and rituximab-based regimens were the most effective therapies with, respectively, 53% and 52% of partial or better clinical responses. Corticosteroids and immunosuppressive drugs were largely ineffective. Although more studies are warranted to better define the optimal therapeutic sequence, IVIg should be proposed as the standard of care for first-line treatment and rituximab-based regimens for second-line treatment. These compiled data argue for CANOMAD to be included in neurologic monoclonal gammopathy of clinical significance.

The oculomotor neurovascular conflict: Literature review and proposal of management.

BACKGROUND: The Oculomotor nerve (OCN) lies in a close relationship with large arteries inside the basal cisterns. Therefore, it may be compressed by vascular malformations or aneurysms. Nevertheless, the compression is not always related to pathologic conditions. Indeed, some cases of neurovascular conflicts of the OCN have been described in the literature. METHODS: A case of neurovascular conflict of the OCN resolved after steroid treatment is reported. Additionally, a systematic literature review of those cases was performed. RESULTS: OCN palsy due to a neurovascular conflict has been described as acute or chronic persistent palsy, or with an intermittent presentation. Symptoms result from compression, although asymptomatic compression is not uncommon. Surgical treatment, pharmacological treatment, and observation have been reported as options in the literature. Microvascular decompression was employed effectively in some cases of OCN neurovascular conflict. Nevertheless, other cases were treated successfully with steroids (persistent presentation) and carbamazepine (intermittent presentation). A management algorithm based on the results of the literature review is proposed. CONCLUSIONS: Treatment options for OCN neurovascular conflicts and their results are heterogeneous. Based on the literature review, the pharmacological treatment seems to be the most appropriate first-line approach, reserving surgery for refractory cases. Collecting clinical information about new cases will allow defining treatment standards for this rare condition.