| Identifier |
walsh_2022_s3_c5 |
| Title |
An Apple a Day |
| Creator |
Joseph Fong, James O'Brien, Anil Patel |
| Subject |
trigeminal neuralgia, 3rd nerve palsy, amyloidosis, skull base tumors, cranial nerve palsies |
| History |
A 73-year-old female presented with a 4-month-history of gradually worsening binocular oblique diplopia, right upper; eyelid ptosis, right-sided facial weakness, and facial pain and paresthesia in the right V1 and V2 distribution. She presented; with a prior diagnosis of trigeminal neuralgia as a result of these symptoms. She reported no systemic symptoms.; Examination showed best-corrected visual acuity of 20/40 right eye and 20/25 left eye with no evidence of optic; neuropathy. Decreased acuity was attributed to mild keratopathy and cataracts. She had an efferent pupillary defect in the; right eye ; there was no relative afferent pupillary defect. She had 50% right upper eyelid ptosis. Ocular motility was notable; for -4 adduction, -2 supraduction, and -2 infraduction deficits of the right eye; motility of left eye was normal. Facial; sensation was diminished in the right V1, V2, and V3 dermatomes. Anterior segment examination was notable for; neurotrophic keratopathy in the right eye as well as visually-significant cataracts bilaterally. Fundus exam was normal; bilaterally. Magnetic resonance imaging of the brain and orbits (representative radiology images) revealed an ill-defined,; intensely enhancing cavernous sinus lesion which was isointense in both T1 and T2-weighted images. The lesion extended; anteriorly into the superior orbital fissure and posteriorly to obliterate the right Meckel's cave. There was enhancement of; the V2 division of the trigeminal nerve extending through the right foramen rotundum and right pterygopalatine fossa.; There was also enhancement of the V3 division extending through the right foramen ovale. She was given gabapentin to; alleviate her facial pain. |
| Disease/Diagnosis |
Pathologic analysis of the biopsy specimen (representative pathology images) revealed islands of pink, amorphous; depositions that stained positively with Congo Red and demonstrated apple green birefringence with polarized light,; consistent with amyloidosis. Following this diagnosis, hematology/oncology was consulted, and she underwent a systemic; work-up, including bone marrow biopsy which was remarkable for a population of 10-12% monoclonal plasma cells.; Additionally, positron emission tomography computed tomography revealed numerous bony lytic lesions. She was; diagnosed with smoldering plasma cell myeloma with secondary amyloidosis of the cavernous sinus and trigeminal nerve.; She was started on combination therapy with lenalidomide, bortezomib, and dexamethasone. We describe an elderly; female with amyloidosis involving the cavernous sinus, Meckel's cave, and the superior orbital fissure, manifesting with; oculomotor and trigeminal nerve dysfunction. Amyloidosis is defined as extracellular deposition of amorphous,; heterogeneous protein in body tissues and organs that can occur as an isolated, localized disease or secondary to; underlying chronic inflammation, occult neoplastic disorders, chronic renal failure or rheumatoid arthritis(1). There are 17; previously reported cases of trigeminal amyloidosis since the first case was reported in 1957. These reports describe; localized amyloidosis of the trigeminal ganglion as slow-growing masses capable of a perineural pattern of spread capable; of causing trigeminal neuropathy similar to our patient(2-4). Of the cases previously reported in the literature, most; patients were successfully treated with surgical resection of the amyloidoma(2). Once the diagnosis is established, an; extensive systemic workup should be performed to rule out the presence of systemic amyloidosis, chronic inflammation, or; occult neoplastic disorder(2). In our patient's case, systemic workup revealed the presence of smoldering plasma cell; myeloma. |
| Date |
2022-02 |
| References |
1. Amyloidosis: a convoluted story. Kyle RA. Br J Haematol. 2001;114:529-538.; 2. Hashmi H, Dhanoa J, Manapuram S. Trigeminal Amyloidoma: Case Report and Review of Literature. Cureus. 2018; Dec 28;10(12):e3795. doi: 10.7759/cureus.3795. PMID: 30868009; PMCID: PMC6402733.; 3. Bilateral Meckel's cave amyloidoma: a case report. Gultasli N, van den Hauwe L, Bruneau M, D'Haene N, Delpierre; I, Balériauxa D. J Neuroradiol. 2012;39:119-122.; 4. Intracavernous trigeminal ganglion amyloidoma: case report. Bookland MJ, Bagley C, Schwarz J, Burger PC, Brem H.; Neurosurgery. 2007;60:0. |
| Format |
application/pdf |
| Type |
Text |
| Source |
54th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting 2022: Walsh Session III |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2022. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6rfj5jj |
| Contributor Primary |
Joseph W. Fong, MD |
| Contributor Secondary |
James O'Brien, Anil Patel |
| Setname |
ehsl_novel_fbw |
| ID |
2100243 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6rfj5jj |
