Table 1.

Studies characterizing rates and causes of diagnostic error in neuro-ophthalmic conditions

Authors, Publication Year	Design	Population	Condition(s)	Outcome(s) of interest	Misdiagnosis rate	Summary
Dillon et al., 1994 [21]	Retrospective review	588 patients	Optic neuropathy, diplopia, ptosis, proptosis	Appropriateness of diagnostic testing prior to neuro-ophthalmic consultation	N/A	For optic neuropathy, diplopia, and ptosis, rate of unnecessary testing ranged from 16–26%, resulting in excessive costs. For proptosis, testing was appropriate, but 56% of neuroimaging studies were sub-optimal to answer clinical question
Elmalem et al., 2010 [16]	Retrospective review	17 patients	3rd nerve palsy due to compression by Pcomm aneurysm	Missed diagnoses on noninvasive imaging	47%	Neuroradiology training and providing specific clinical information to radiologists was associated with diagnostic accuracy
McClelland et al., 2012 [14]	Prospective cohort study	84 patients	Multiple	Appropriate diagnostic imaging prior to neuro-ophthalmic consultation	69%	38% had suboptimal neuroimaging prior to neuro-ophthalmic consultation, and 29% required additional neuroimaging
Fisayo et al., 2016 [17]	Retrospective review	165 patients	IIH	Misdiagnosis rate and causes	40%	24% had delayed alternative diagnosis. Unnecessary procedures were common, including LP in 80% of misdiagnosed patients. Most errors were due to inaccurate examination or inaccurate weighing of differential
Stunkel et al., 2018 [13]	Retrospective cross-sectional study	122 patients	Optic neuritis	Misdiagnosis rate and causes	60%	16% had unnecessary LP and 11% received unnecessary IV steroids. Most errors were due to inaccurate weighing of differential or inaccurate examination
Blanch et al., 2019 [26]	Prospective cohort study	198 patients	Papilledema	Misdiagnosis rate for papilledema using fundus photographs	21%	Sensitivity was close to 100% across all specialties (neuro-ophthalmology, neurology, ophthalmology and emergency medicine). Specificity ranged from 43–100% depending on specialty
Hartmann et al., 2019 [25]	Retrospective review	16 patients	Intracranial hypertension due to compression of dural venous sinus by meningioma	Delayed diagnosis	63%	40% had a poor visual outcome in the setting of delayed diagnosis. Most errors were due to misinterpretation of or failure to obtain sufficient imaging
Kahraman-Koytak et al., 2019 [18]	Retrospective review	35 patients	Optic nerve sheath meningioma	Misdiagnosis rate and causes	71%	64% had a poor visual outcome in the setting of delayed diagnosis. 24% received unnecessary treatment with steroids, 20% receiving unnecessary lumbar puncture. Most errors were due to weighing of differential or inaccurate imaging interpretation

N/A not applicable. Pcomm posterior communicating artery. IIH idiopathic intracranial hypertension. LP lumbar puncture. IV intravenous