Proposed modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy: Five case reports and literature review

Liu, Yinglu; Wang, Miao; Bian, Xiaowei; Qiu, Enchao; Han, Xun; Dong, Zhao; Yu, Shengyuan

doi:10.1177/0333102420944872

Cited by 20 publications

(69 citation statements)

References 65 publications

(119 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Although two attacks are necessary, it strongly suggests to consider RPON even at the rst attack, in presence of described characteristics. Thus, as mentioned by Wong(11) and Yinglu (14), we suggest to add into the diagnostic criteria the MRI ndings, including enhancement and thickening of the nerve involved. We analyzed the relationship between RPON and schwannoma.…”

Section: Discussionmentioning

confidence: 88%

“…In cases where MRI was negative, it is important to understand whether the imaging was really negative or the timing was wrong. In fact, in some patients there is no evidence of MRI abnormalities neither during the interictal phase nor during the rst attack and it could only be found after attacks (14). Therapy in acute phase had been administered in 70% of patients using corticosteroids.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Recurrent Painful Ophthalmoplegic Neuropathy: Diagnostic Controversies. Single Case Report with a Systematic Review

Falsaperla

Presti

Bianco

et al. 2021

Preprint

View full text Add to dashboard Cite

Background: Ophthalmoplegic migraine, renamed “Recurrent Painful Ophthalmoplegic Neuropathy (RPON) in 2013 by the International Headache Society is a rare neurologic disorder characterized by recurrent attacks of ophthalmoplegia associated to ipsilateral headache. The etiology is still unknown. Typical magnetic resonance imaging findings show a focal nerve thickening and contrast enhancement. In the majority of cases, there is a full recovery within days or weeks. There is no evidence supporting a specific treatment. The review defines the characteristics of the recurrent painful ophthalmoplegic neuropathy in patients within 2 years of age underlying the importance of the role of magnetic resonance imaging even in presence of the first attack. Thus, an emblematic case report is presented.Case presentation: The authors present a case of third cranial nerve paresis in a 17-month-old male child, presenting a neuroradiological pattern highly suggestive of schwannoma, aneurism or recurrent painful ophthalmoplegic neuropathy. Thus, a review of the literature with the pediatric casuistry of recurrent painful ophthalmoplegic neuropathy occurred within 2 years of age focusing on diagnostic considerations is presented. The authors highlight the importance to consider recurrent painful ophthalmoplegic neuropathy in presence of magnetic resonance imaging findings and clinical symptoms referable to aneurysm or schwannoma. Thus, the review defines the characteristics and the neuroradiological findings at the first RPON attack occurred under 2 years of age.Conclusion: Although two attacks are necessary, the review strongly suggests to consider recurrent painful ophthalmoplegic neuropathy even at the first attack, in presence of described characteristics and the aforementioned magnetic resonance imaging findings.

show abstract

Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Recurrent Painful Ophthalmoplegic Neuropathy: Diagnostic Controversies. Single Case Report with a Systematic Review

Falsaperla

Presti

Bianco

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…Oculomotor nerve enhancement has previously been confirmed to be an indication of a variety of underlying etiologies [6, 12-15], including Tolosa-Hunt syndrome, recurrent painful ophthalmoplegic neuropathy (RPON), and oculomotor nerve tumors, and our patients should be especially distinguished from these nonischemic causes. However, pain and ophthalmoplegia were not resolved within 72 h of starting corticosteroid therapy, and it had never been reported that the oculomotor nerve was solely thickened and enhanced without enlargement of the cavernous sinus in Tolosa-Hunt syndrome [13]. Several patients with RPON had also revealed unilateral thickening and enhancement of the oculomotor nerve, but the cisternal segment was almost always involved [13, 16].…”

Section: Discussionmentioning

confidence: 99%

“…However, pain and ophthalmoplegia were not resolved within 72 h of starting corticosteroid therapy, and it had never been reported that the oculomotor nerve was solely thickened and enhanced without enlargement of the cavernous sinus in Tolosa-Hunt syndrome [13]. Several patients with RPON had also revealed unilateral thickening and enhancement of the oculomotor nerve, but the cisternal segment was almost always involved [13, 16]. Solid tumors frequently demonstrate nodular enhancement of the cisternal segment of the oculomotor nerve, and some may show the extension of mass lesions into the cavernous sinus or even intraorbital extension through the superior orbital fissure.…”

Section: Discussionmentioning

confidence: 99%

“…Follow-up MRI revealed a slight change in the degree of enhancement of the oculomotor nerve in 5 patients. This was indeed different from RPON, in which the abnormal enhancement was located in the root exit zone of the oculomotor nerve and resolved quickly with corticosteroids [13, 16]. Enhancement of the cisternal segment of the oculomotor nerve was identified in a patient with susceptible diabetic ONP [6].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Diabetic Oculomotor Nerve Palsy Displaying Enhancement of the Oculomotor Nerve in the Orbit and Cavernous Sinus on MRI

Zhao¹,

Li²,

Chang³

et al. 2021

Eur Neurol

View full text Add to dashboard Cite

Introduction: Imaging data were scarce on diabetic oculomotor nerve palsy (ONP). Our study explored the MRI features and their clinical implications for diabetic ONP. Methods: Fifty-nine patients with a clinical diagnosis of diabetic ONP were recruited from our department between January 2015 and December 2019. Orbital MRI was retrospectively analyzed, and follow-up scans were obtained for 5 patients. Based on the ocular motor nerve palsy scale, the difference in the scores on the first and last hospital days was defined as the improvement score and was used to assess the treatment effects in all. Results: Thirty-eight (64.41%) patients presented thickening and enhancement of the cavernous segment and inferior division of the intraorbital segment of the ipsilateral oculomotor nerve, with the cisternal segment spared in all. After complete resolution of symptoms, follow-up MRI in 5 patients revealed that the enhancement was less obvious compared with the previous images. 6 patients in the enhancement group and 4 patients in the nonenhancement group were treated with 80 mg of methylprednisolone. Significant differences were not detected in the median improvement scores between patients with and those without corticosteroid use (p = 0.240). Conclusion: Thickening and enhancement of the unilateral oculomotor nerve were common imaging findings in diabetic ONP, and they persisted after complete resolution of symptoms in some patients. The cavernous segment and the inferior division of the intraorbital segment were simultaneously involved, and the cisternal segment was often spared. Refraining from corticosteroids was recommended even with nerve enhancement.

show abstract

Clinical and imaging clues to the diagnosis and follow‐up of ptosis and ophthalmoparesis

Keene

Kan

Meeren

et al. 2022

J cachexia sarcopenia muscle

View full text Add to dashboard Cite

Ophthalmoparesis and ptosis can be caused by a wide range of rare or more prevalent diseases, several of which can be successfully treated. In this review, we provide clues to aid in the diagnosis of these diseases, based on the clinical symptoms, the involvement pattern and imaging features of extra‐ocular muscles (EOM). Dysfunction of EOM including the levator palpebrae can be due to muscle weakness, anatomical restrictions or pathology affecting the innervation. A comprehensive literature review was performed to find clinical and imaging clues for the diagnosis and follow‐up of ptosis and ophthalmoparesis. We used five patterns as a framework for differential diagnostic reasoning and for pattern recognition in symptomatology, EOM involvement and imaging results of individual patients. The five patterns were characterized by the presence of combination of ptosis, ophthalmoparesis, diplopia, pain, proptosis, nystagmus, extra‐orbital symptoms, symmetry or fluctuations in symptoms. Each pattern was linked to anatomical locations and either hereditary or acquired diseases. Hereditary muscle diseases often lead to ophthalmoparesis without diplopia as a predominant feature, while in acquired eye muscle diseases ophthalmoparesis is often asymmetrical and can be accompanied by proptosis and pain. Fluctuation is a hallmark of an acquired synaptic disease like myasthenia gravis. Nystagmus is indicative of a central nervous system lesion. Second, specific EOM involvement patterns can also provide valuable diagnostic clues. In hereditary muscle diseases like chronic progressive external ophthalmoplegia (CPEO) and oculo‐pharyngeal muscular dystrophy (OPMD) the superior rectus is often involved. In neuropathic disease, the pattern of involvement of the EOM can be linked to specific cranial nerves. In myasthenia gravis this pattern is variable within patients over time. Lastly, orbital imaging can aid in the diagnosis. Fat replacement of the EOM is commonly observed in hereditary myopathic diseases, such as CPEO. In contrast, inflammation and volume increases are often observed in acquired muscle diseases such as Graves' orbitopathy. In diseases with ophthalmoparesis and ptosis specific patterns of clinical symptoms, the EOM involvement pattern and orbital imaging provide valuable information for diagnosis and could prove valuable in the follow‐up of disease progression and the understanding of disease pathophysiology.

show abstract

Proposed modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy: Five case reports and literature review

Cited by 20 publications

References 65 publications

Recurrent Painful Ophthalmoplegic Neuropathy: Diagnostic Controversies. Single Case Report with a Systematic Review

Recurrent Painful Ophthalmoplegic Neuropathy: Diagnostic Controversies. Single Case Report with a Systematic Review

Diabetic Oculomotor Nerve Palsy Displaying Enhancement of the Oculomotor Nerve in the Orbit and Cavernous Sinus on MRI

Clinical and imaging clues to the diagnosis and follow‐up of ptosis and ophthalmoparesis

Contact Info

Product

Resources

About