Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough
Purpose: Midline structural defects in the neural axis can give rise to neuro-ophthalmic symptoms. We report a rare case of keyhole aqueduct syndrome presenting after two years of severe cough due to gastroesophageal reflux disease. Observations: A 58-year-old woman with a 2-year history of daily, s...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2018-06-01
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| Series: | American Journal of Ophthalmology Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2451993617303158 |
| _version_ | 1819036630427631616 |
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| author | Angela Jinsook Oh Bryan Alexander Lanzman Yaping Joyce Liao |
| author_facet | Angela Jinsook Oh Bryan Alexander Lanzman Yaping Joyce Liao |
| author_sort | Angela Jinsook Oh |
| collection | DOAJ |
| description | Purpose: Midline structural defects in the neural axis can give rise to neuro-ophthalmic symptoms. We report a rare case of keyhole aqueduct syndrome presenting after two years of severe cough due to gastroesophageal reflux disease. Observations: A 58-year-old woman with a 2-year history of daily, severe cough presented to the neuro-ophthalmology clinic with progressive diplopia and oscillopsia. Examination revealed a 1–2 Hz down-beating nystagmus in primary gaze that worsened with left, right, and down gazes. Gaze evoked nystagmus and mild paresis were also seen with up gaze. There was an incomitant left hypertropia due to skew deviation that worsened with right and up gazes and improved with down gaze. She also had a right-sided ptosis and a 3 mm anisocoria not due to cranial nerve 3 paresis or Horner's syndrome. Brain magnetic resonance imaging showed a 1.5 mm × 11.7 mm × 6 mm midline cleft in the ventral midbrain communicating with the cerebral aqueduct, consistent with keyhole aqueduct syndrome. Her nystagmus and diplopia improved with oral acetazolamide treatment, at high doses of 2500–3000 mg per day. Conclusions and importance: We report the first case of midbrain keyhole aqueduct syndrome with ocular motor and other neuro-ophthalmic manifestations associated with severe cough. Although her cough was effectively treated and intracranial pressure measurement was normal, her ophthalmic symptoms continued to progress, which is common in previous cases reported. Treatment with acetazolamide led to significant improvement, supporting the use of acetazolamide in this rare condition. Keywords: Keyhole aqueduct syndrome, Midbrain cleft, Mesencephalic cleft, Syrinx, Syringobulbia, Down-beating nystagmus, Cerebrospinal fluid |
| first_indexed | 2024-12-21T08:08:35Z |
| format | Article |
| id | doaj.art-e83ccf82eda04f58b841b827598f791a |
| institution | Directory Open Access Journal |
| issn | 2451-9936 |
| language | English |
| last_indexed | 2024-12-21T08:08:35Z |
| publishDate | 2018-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | American Journal of Ophthalmology Case Reports |
| spelling | doaj.art-e83ccf82eda04f58b841b827598f791a2022-12-21T19:10:45ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362018-06-0110128131Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe coughAngela Jinsook Oh0Bryan Alexander Lanzman1Yaping Joyce Liao2Department of Ophthalmology, Stanford School of Medicine, 2452 Watson Court, Palo Alto, CA 94303-5353, USADepartment of Radiology, Neuroradiology Division, Stanford University Medical Center, 300 Pasteur Dr. S031, Stanford, CA 94305, USADepartment of Ophthalmology, Stanford School of Medicine, 2452 Watson Court, Palo Alto, CA 94303-5353, USA; Department of Neurology, Stanford School of Medicine, 291 Campus Drive, Stanford, CA 94305, USA; Corresponding author. Department of Ophthalmology, Stanford University School of Medicine, 2452 Watson Court, Palo Alto, CA 94303-5353, USA.Purpose: Midline structural defects in the neural axis can give rise to neuro-ophthalmic symptoms. We report a rare case of keyhole aqueduct syndrome presenting after two years of severe cough due to gastroesophageal reflux disease. Observations: A 58-year-old woman with a 2-year history of daily, severe cough presented to the neuro-ophthalmology clinic with progressive diplopia and oscillopsia. Examination revealed a 1–2 Hz down-beating nystagmus in primary gaze that worsened with left, right, and down gazes. Gaze evoked nystagmus and mild paresis were also seen with up gaze. There was an incomitant left hypertropia due to skew deviation that worsened with right and up gazes and improved with down gaze. She also had a right-sided ptosis and a 3 mm anisocoria not due to cranial nerve 3 paresis or Horner's syndrome. Brain magnetic resonance imaging showed a 1.5 mm × 11.7 mm × 6 mm midline cleft in the ventral midbrain communicating with the cerebral aqueduct, consistent with keyhole aqueduct syndrome. Her nystagmus and diplopia improved with oral acetazolamide treatment, at high doses of 2500–3000 mg per day. Conclusions and importance: We report the first case of midbrain keyhole aqueduct syndrome with ocular motor and other neuro-ophthalmic manifestations associated with severe cough. Although her cough was effectively treated and intracranial pressure measurement was normal, her ophthalmic symptoms continued to progress, which is common in previous cases reported. Treatment with acetazolamide led to significant improvement, supporting the use of acetazolamide in this rare condition. Keywords: Keyhole aqueduct syndrome, Midbrain cleft, Mesencephalic cleft, Syrinx, Syringobulbia, Down-beating nystagmus, Cerebrospinal fluidhttp://www.sciencedirect.com/science/article/pii/S2451993617303158 |
| spellingShingle | Angela Jinsook Oh Bryan Alexander Lanzman Yaping Joyce Liao Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough American Journal of Ophthalmology Case Reports |
| title | Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough |
| title_full | Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough |
| title_fullStr | Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough |
| title_full_unstemmed | Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough |
| title_short | Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough |
| title_sort | vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough |
| url | http://www.sciencedirect.com/science/article/pii/S2451993617303158 |
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