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A 20-year-old woman with abnormal eye movements
Digital Journal of Ophthalmology 2021
Volume 27, Number 1
January 4, 2021
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Vivian Paraskevi Douglas, MD, DVM, MBA | Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts Homer H. Chiang, MD | Department of Ophthalmology, UT Health San Antonio, San Antonio, Texas Konstantinos A. A. Douglas, MD, DVM, MBA | Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts Tavé Van Zyl, MD | Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts Nurhan Torun, MD | Division of Ophthalmology, Department of Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts
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| Diagnosis and Discussion | Eight-and-a-half syndrome was first described in 1998 by Eggenberger.(2) It is a rare pontine neuro-ophthalmologic syndrome characterized by a combination of one-and-a-half syndrome and ipsilateral facial nerve palsy and is most often caused by infarction. This syndrome has also been described in demyelinating disorders involving the pons level as well as space-occupying lesions such as tuberculomas or cavernomas.(5)
Cavernous malformations (CMs) are well-defined, tightly packed masses of thin-walled, dilated, sinusoidal capillaries with endothelial lining and fibrous adventitia. Although CMs may occur anywhere in the central nervous system, most are found in the cerebrum, accounting for up to 25% of all vascular malformations. In a meta-analysis of 11 natural history studies, brainstem CMs comprised 18 % of lesions.(6)
Because of the high density of cranial nerve nuclei and associated nerve tracts, patients with brainstem CMs commonly present with multiple cranial neuropathies and variable ophthalmic manifestations. Progressive neurological deficits are not uncommon and were observed in 39 % of patients in one case series.(1)
Characteristic T1- and T2-weighted MRI findings include a “popcorn” pattern of variable intensity due to evolving blood products. Remote bleeds may appear as a hemosiderin ring in the periphery of the lesion.
In conclusion, brainstem CMs can have variable ophthalmic manifestations owing to the close proximity of cranial nerve nuclei in the brainstem. Common ophthalmic manifestations include extraocular motility deficits that result in diplopia and long-term ocular surface sequelae due to lagophthalmos, with decreased or absent blink. Early recognition is of key importance for determining proper treatment of brainstem CMs, which should include the input of neurologists, neuro-ophthalmologists, and neurosurgeons for optimal results. | |
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