A Rare Case of Steroid-Resistant Neurosarcoidosis of the Cavernous Sinus With Optic Neuropathy: A Case Report

2021 ◽  
pp. 194187442199364
Author(s):  
Shiva Barforoshi ◽  
Nikhil A. Patel ◽  
Alvin P. Singh ◽  
Terrence Li ◽  
Benjamin Mba

Sarcoidosis is an inflammatory disease that presents with nervous system involvement in 5-10% of cases, commonly known as neurosarcoidosis.1 While there are no randomized controlled trials for the treatment of neurosarcoidosis, expert opinion supports initial treatment with corticosteroids and the use of steroid-sparing or anti-TNF agents in refractory or severe cases. We report a case of a 48-year-old African American male with a past medical history of biopsy-proven hepatic and renal sarcoidosis and progressive headache, dizziness, and blurry vision for 5 months, presenting with an acute exacerbation of right-sided vision loss over one day. MRI of the brain revealed a dural-based mass extending into the right cavernous sinus and compressing the right optic nerve. Given the pathological confirmation of systemic granulomatous disease consistent with sarcoidosis, clinical manifestations, bilateral hilar and mediastinal lymphadenopathy, MRI findings typical of central nervous system inflammation, and exclusion of other possible etiologies, the patient was diagnosed with probable neurosarcoidosis. Corticosteroids initially resolved his symptoms; however, he suffered an acute relapse. Combination therapy with corticosteroids plus mycophenolate mofetil (MMF) led to the eventual resolution of his symptoms. Only 8 cases of neurosarcoidosis involving the cavernous sinus have been reported. Our patient represents the only reported case of probable neurosarcoidosis of the cavernous sinus with optic neuropathy successfully treated with corticosteroid plus MMF combination therapy. We highlight the need to consider early, aggressive treatment in cases of neurosarcoidosis with optic neuropathy and to develop criteria to guide treatment strategy based on neurolocalization and the degree of neurological disability.

2017 ◽  
Vol 27 (6) ◽  
pp. e184-e186 ◽  
Author(s):  
Giorgia C. Albertini ◽  
Eleonora Corbelli ◽  
Maurizio Battaglia Parodi ◽  
Francesco Bandello

Purpose To describe a case of bilateral choroidal neovascularization (CNV) in multifocal choroiditis (MFC) associated with dabrafenib and trametinib chemotherapy for metastatic melanoma. Case We present a case of a 57-year-old man with MFC who underwent combination therapy with dabrafenib plus trametinib for metastatic melanoma. The patient presented to our ophthalmology department complaining of bilateral vision loss of 2 days’ duration. He underwent multimodal imaging showing a MFC reactivation complicated by bilateral CNV. The patient underwent 3 ranibizumab injections in the right eye and 7 ranibizumab injections in the left eye. Anatomical and functional improvement has been observed. Conclusions This report emphasizes the importance of strict ophthalmologic follow-up in patients with metastatic melanoma treated with dabrafenib plus trametinib since rare severe ocular toxicities can occur, especially in patients with a history of uveitis.


Author(s):  
Aluisio Rosa Gameiro Filho ◽  
Guilherme Sturzeneker ◽  
Ever Ernesto Caso Rodriguez ◽  
André Maia ◽  
Melina Correia Morales ◽  
...  

Abstract Background To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. Case presentation A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. Conclusions We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.


2021 ◽  
Vol 9 ◽  
pp. 2050313X2110030
Author(s):  
Asfandyar Mufti ◽  
Muskaan Sachdeva ◽  
Khalad Maliyar ◽  
Marissa Joseph

Background: Hereditary haemorrhagic telangiectasia is an autosomal dominant genetic disorder characterized by abnormalities in blood vessel formation. The clinical manifestations of patients affected with hereditary haemorrhagic telangiectasia include mucocutaneous telangiectasias and visceral arteriovenous malformations. Case Summary: We report the case of a 30-year-old female diagnosed with hereditary haemorrhagic telangiectasia presenting with the classic triad of recurrent epistaxis, mucocutaneous telangiectasias and family history of hereditary haemorrhagic telangiectasia with activin receptor-like kinase 1 mutation. Upon skin examination, she was noted to have telangiectasias under left naris, inner lower lip and surface of the tongue, and a vascular malformation on the right forearm. Conclusion: Although the skin involvement and epistaxis may be mild symptoms and signs of hereditary haemorrhagic telangiectasia, timely recognition of these can ensure vigilant monitoring of potential severe complications from cerebral and pulmonary visceral arteriovenous malformations.


2021 ◽  
pp. 160-162
Author(s):  
John R. Mills

A 62-year-old man with a history of migraine came to the emergency department with sudden onset of horizontal diplopia and, subsequently, bilateral ptosis. He noted feeling unsteady when walking. He reported that the diplopia worsened throughout the day. He had a history of hepatitis C infection. He had some vision loss in his left eye, which was thought to relate to a retinopathy. He disclosed that he had a history of cold feet and had notably high arches. He had a pacemaker because of syncope attributed to sick sinus syndrome. Computed tomography angiography of the head and neck were ruled negative for intracranial stenosis, occlusions, or aneurysms. Computed tomography of the head indicated a tiny lacunar infarct in the right caudate head. Magnetic resonance imaging of the brain identified a tiny, periaqueductal, enhancing abnormality in the right midbrain that was thought to be likely ischemic, but there was some concern for a demyelinating or inflammatory lesion. Cerebrospinal fluid evaluation indicated an increased protein concentration. Serologic evaluation for myasthenia gravis striational antibodies were positive at a titer of 1:240. Serum protein studies indicated the presence of polyclonal hypergammaglobulinemia. Myasthenia gravis was effectively ruled out. Given the hyperacute time course, the patient’s clinical disorder was most probably explained by an ischemic stroke that affected the oculomotor nuclei regions causing ptosis and ophthalmoparesis. On follow-up, the patient was discovered to have a patent foramen ovale. Whether the patent foramen ovale was a contributing factor to the stroke is uncertain. The recurrence rate in this setting is thought to be low relative to other causes of stroke. Ultimately it was decided to not close the patent foramen ovale and to maintain the patient on clopidogrel and adult low-dose aspirin. The onset of diplopia is typically sudden, but this occurs exclusively with vascular pathologic processes. Diplopia that appears intermittently with diurnal variation suggests the possibility of a neuromuscular junction disease such as myasthenia gravis.


2017 ◽  
Vol 8 (1) ◽  
pp. 61-66 ◽  
Author(s):  
María Carmen Guixeres Esteve ◽  
Augusto Octavio Pardo Saiz ◽  
Lucía Martínez-Costa ◽  
Samuel González-Ocampo Dorta ◽  
Pedro Sanz Solana

The early development of lens opacities and lens subluxation are the most common causes of vision loss in patients with anterior megalophthalmos (AM). Cataract surgery in such patients is challenging, however, because of anatomical abnormalities. Intraocular lens dislocation is the most common postoperative complication. Patients with AM also seem to be affected by a type of vitreoretinopathy that predisposes them to retinal detachment. We here present the case of a 36-year-old man with bilateral AM misdiagnosed as simple megalocornea. He had a history of amaurosis in the right eye due to retinal detachment. He presented with vision loss in the left eye due to lens subluxation. Following the removal of the subluxated lens, it was deemed necessary to perform a vitrectomy in order to prevent retinal detachment. Seven months after surgery, an Artisan® Aphakia iris-claw lens was implanted in the anterior chamber. Fifteen months of follow-up data are provided.


2001 ◽  
Vol 94 (2) ◽  
pp. 322-326 ◽  
Author(s):  
Kazumichi Yamada ◽  
Masaki Miura ◽  
Haruhiko Miyayama ◽  
Naohiko Furuyoshi ◽  
Jun Matsumoto ◽  
...  

✓ The authors report the first case of a solitary xanthogranuloma of the cavernous sinus without systemic manifestation. A 43-year-old woman experienced decreased visual acuity on the right side in association with papilledema and a visual field defect. She was alert and no other neurological sign was observed. She had no family history of hyperlipoproteinemia and no cutaneous and/or systemic xanthomatous lesions. Skull x-ray films showed an enlarged right superior orbital fissure. Computerized tomography revealed a low-density oval mass 2 × 3 cm in diameter in the right cavernous region that was compressing the right optic nerve in the lateral to medial direction. This mass exhibited isointensity on T1-weighted and mixed-pattern hyperintensity on T2-weighted magnetic resonance images with diffuse contrast enhancement. Cerebral angiography demonstrated fine staining in the margin, originating from the right middle meningeal artery. These neuroimaging findings closely mimicked those associated with trigeminal neuroma. The lesion was partially removed via a right-sided zygomatic approach, and her symptoms improved. Clinical characteristics and histopathological features of this rare case are discussed.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
G. Manenti ◽  
F. Di Giuliano ◽  
A. Bindi ◽  
V. Liberto ◽  
V. Funel ◽  
...  

Primary central nervous system lymphomas (PCNSLs) are mainly B-cells lymphomas. A risk factor for the development of PCNSL is immunodeficiency, which includes congenital disorders, iatrogenic immunosuppression, and HIV. The clinical course is rapidly fatal; these patients usually present signs of increased intracranial pressure, nausea, papilledema, vomiting, and neurological and neuropsychiatric symptoms. PCNSL may have a characteristic appearance on CT and MR imaging. DWI sequences and MR spectroscopy may help to differentiate CNS lymphomas from other brain lesions. In this paper, we report a case of a 23-year-old man with T-primary central nervous system lymphoma presenting with a mass in the right frontotemporal lobe. We describe clinical, CT, and MRI findings. Diagnosis was confirmed by stereotactic biopsy of the lesion.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 15546-15546
Author(s):  
E. Troncoso ◽  
S. Bonicatto ◽  
A. Mainella ◽  
A. Barbero ◽  
M. Lavezzaro ◽  
...  

15546 Background: ENB is a rare embrionary tumor derived from neuroblasts of the olfactory sensory system. Polypoid mass with epistaxis or nasal obstruction are the most common clinical manifestations of this tumor. It’s invasive and it frequently causes regional and distant metastasis. ENB requires a multimodality therapy. The objective of this study is to describe the form of presentation, diagnosis, treatment and evolution of this tumor on a female patient (pt), as a casuistic contribution. Methods and Case Report: A 61 year old woman with a four month history of epistaxis, nasal pain and anosmia. CT shows nasal mass invading the entire nasal cavity and upper maxillary. Pt underwent nasal resection and reconstruction with frontal flaps. Anatomopathology: ENB invading the bone. Cromogranin (+), sinaptofisin (+), CK (−), NSE (−). Three months later: lesion on the right wing nasal and a mass in the soft palate. MRI: mass on the floor of the nasal fossa that involve the left maxillary sinus and the bone palate. Kadish stage C. We treated her with three cycles of chemotherapy using cisplatin 30 mg/sqM d 1–3 iv and etoposide 100 mg/sqM d 1–3 iv. After that, remission was observed in the wing nasal lesion but the the soft palate mass shows progression. MRI: mass in nasal fossa that destroys the left maxillary sinus and causes lysis of the upper maxillary and orbital floor. Pt was treated with radiotherapy (6000 cGy) showing complete remission of the nasal lession and partial response on the soft palate, verified by physical examination and RMI. Four months after the end of radiotherapy, she continues under control and maintains the response to therapy without evidence of progression. Conclusion: ENB is an unfrequent tumor without any standard treatment. In our case, the combination of surgery, CH and RT has been effective for local control of the disease with good tolerance and acceptable quality of life. No significant financial relationships to disclose.


Author(s):  
Jamsheed A. Desai ◽  
Jessica Dobson ◽  
Michel Melanson ◽  
Giovanna Pari ◽  
Albert Yongwon Jin

A 74-year-old man presented with a four week history of behavioural disturbances, upper and lower extremity numbness and impaired balance. He had been treated with metronidazole for six months for osteomyelitis of the right hallux. Examination revealed encephalopathy, and glove-and-stocking sensory loss to pinprick with reduced vibration threshold at the toe. The gait was wide based and ataxic. Nerve conduction studies showed a large fibre sensory-motor axonal polyneuropathy. Magnetic resonance imaging (MRI) revealed a solitary restricted diffusion lesion in the splenium of the corpus callosum (Figure A, B) with subtle prolongation of T2 (Figure C). The radiographic differential diagnosis included hypoglycaemia, viral encephalitis, antiepileptic drug toxicity/withdrawal and metronidazole toxicity. The combination of the imaging finding with the history of prolonged metronidazole use suggested metronidazole induced encephalopathy.


2018 ◽  
Vol 97 (6) ◽  
pp. 578-580
Author(s):  
Vivian Nunes Arruda ◽  
Melissa De Andrade ◽  
Rhafir Gonçalves

Hepatic Angiosarcoma is a rare malignancy of endothelial cell origin that is generally idiopathic and presents non-specific clinical manifestations. Hemoperitoneum can occur in 17-27% of cases and is a result of tumor rupture, which has a devastating prognosis. We present a 79-year-old female patient with history of diffuse right upper quadrant abdominal pain associated with syncope episodes and unintentional weight loss. Physical examination was unremarkable except for painful palpation of the right upper quadrant. Laboratory exams indicated chronic disease anemia, a 2.7 INR, decreased albumin, increased C-Reactive Protein and GGT, and normal AST, ALT, Alkaline Phosphatase and Bilirubin. Viral hepatitis serologies were negative. Abdominal ultrasonography revealed hepatomegaly and solid liver lesions of heterogeneous echogenicity and imprecise limits. Three-phase abdominal CT showed multiple liver masses of heterogeneous pattern of enhancement suggestive of atypical hemangioma associated with ascites. Chest CT revealed bilateral pulmonary nodules suggestive of metastasis. During hospital stay, the patient developed a massive hemoperitoneum that required emergency laparotomy. In this circumstance, liver and omentum biopsies were performed and the pathology reports ultimately indicated the possibility of hepatic angiosarcoma. The patient developed refractory hemoperitoneum and hemorrhagic shock and ultimately passed away, 48 days after hospital admission. Definitive diagnosis was only available posteriorly, with immunohistochemistry positivity for ERG, CD34 and Factor VIII-related antigen on both omentum and lung samples. This case study provides valuable clinical discussion and emphasizes how a transdisciplinary approach is essential to correctly diagnose and manage such complex cases.


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