| Identifier |
walsh_2018_s3_c4 |
| Title |
A Can of Worms - Video |
| Creator |
Ali Hamedani; Madhura Tamhankar |
| Affiliation |
(AH) University of Pennsylvania, Philadelphia, Pennsylvania |
| Subject |
Miller Fisher variant, Diplopia, Guillain-Barré, CIDP |
| History |
A 67 year-old man presented to the ophthalmology clinic with one day of binocular horizontal diplopia. He also complained of one week of bilateral periorbital pain which was worse on the right than the left. Four days prior, he had undergone partial right nephrectomy for grade II papillary renal cell carcinoma. He denied vision loss; other neurologic symptoms including weakness, numbness, or imbalance; or constitutional symptoms such as fever, chills, or weight loss. Visual acuity was 20/20 in each eye with normal color vision and full visual fields to confrontation. Pupils were equally reactive without afferent pupillary defect. External examination was unremarkable without proptosis, ptosis, or eyelid retraction. Motility was mildly limited in abduction bilaterally, and he had a comitant 16 prism diopter esotropia. MRI brain, acetylcholine receptor antibodies, and TSH were ordered, and the patient was referred to neuro-ophthalmology. Two weeks later, he presented to neuro-ophthalmology clinic with worsening diplopia and was found to have complete bilateral abduction deficits and 50 PD esotropia. He was admitted to the neurology service and during his hospital stay developed right upper and lower facial weakness, distal vibratory loss, and mild sensory ataxia. MRI brain revealed a questionable fullness of the right cavernous sinus, but on further review this was reported to be normal. Lumbar puncture revealed an opening pressure of 21cm, WBC 1/mL, RBC 1/mL, glucose 64 mg/dL, protein 98 mg/dL, and normal cytology. Serum GQ1b antibodies were negative, and EMG/NCS revealed evidence of axonal polyneuropathy without demyelinating features. He was treated empirically for Miller-Fisher syndrome with IVIG 2g/kg. Despite treatment, his diplopia continued to worsen over the next three months and he developed a new left supraduction deficit in addition to his bilateral abduction deficits (Figure 1). Further diagnostic testing was then performed. |
| Disease/Diagnosis |
CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, and disialosyl antibodies) |
| Presenting Symptom |
A 67 year-old man presented to the ophthalmology clinic with one day of binocular horizontal diplopia. He also complained of one week of bilateral periorbital pain which was worse on the right than the left. Four days prior, he had undergone partial right nephrectomy for grade II papillary renal cell carcinoma. He denied vision loss; other neurologic symptoms including weakness, numbness, or imbalance; or constitutional symptoms such as fever, chills, or weight loss. Visual acuity was 20/20 in each eye with normal color vision and full visual fields to confrontation. Pupils were equally reactive without afferent pupillary defect. External examination was unremarkable without proptosis, ptosis, or eyelid retraction. Motility was mildly limited in abduction bilaterally, and he had a comitant 16 prism diopter esotropia. MRI brain, acetylcholine receptor antibodies, and TSH were ordered, and the patient was referred to neuro-ophthalmology. Two weeks later, he presented to neuro-ophthalmology clinic with worsening diplopia and was found to have complete bilateral abduction deficits and 50 PD esotropia. He was admitted to the neurology service and during his hospital stay developed right upper and lower facial weakness, distal vibratory loss, and mild sensory ataxia. MRI brain revealed a questionable fullness of the right cavernous sinus, but on further review this was reported to be normal. Lumbar puncture revealed an opening pressure of 21cm, WBC 1/mL, RBC 1/mL, glucose 64 mg/dL, protein 98 mg/dL, and normal cytology. Serum GQ1b antibodies were negative, and EMG/NCS revealed evidence of axonal polyneuropathy without demyelinating features. He was treated empirically for Miller-Fisher syndrome with IVIG 2g/kg. Despite treatment, his diplopia continued to worsen over the next three months and he developed a new left supraduction deficit in addition to his bilateral abduction deficits (Figure 1). Further diagnostic testing was then performed. |
| Date |
2018-03 |
| References |
1. Ito M, Kuwabara S, Odaka M, Misawa M, Koga M et al. Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical analysis of 581 cases. J Neurol 2008; 255(5): 674-82. 2. Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T. Clinical features and prognosis of Miller Fisher syndrome. Neurology 2001; 56(8): 1104-6. 3. Willison HJ, O'Leary CP, Veitch J, Blumhardt LD, Busby M et al. The cliical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies. Brain 2001; 124: 1968-77. 4. Sanvito L, Rajabally YA. Optic neuropathy associated with CANOMAD: description of 2 cases. Muscle Nerve 2011; 44(3): 451-5. 5. Attarian S, Boucraut J, Hubert AM, Uzenot D, Delmont E et al. Chronic ataxic neuropathies associated with anti-GD1b IgM antibodies: response to IVIg therapy. JNNP 2010; 81(1): 61-4. 6. Delmont E, Jeandel PY, Hubert AM, Marcq L, Boucraut J et al. Successful treatment with rituximab of one patient with CANOMAD neuropathy. J Neurol 2010; 257(4): 655-7. |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
2018 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2018 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2018. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s63z297q |
| Setname |
ehsl_novel_fbw |
| ID |
1320257 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s63z297q |
