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Visual evoked potentials (VEP) showed no response in the left eye, and normal P100 latency and amplitude in the right eye
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Spontaneous intracranial hypotension is characterized by postural headache that is generally associated with neck and/or back pain, radicular symptoms, nausea, and vomiting, and can sometimes be accompanied by cranial nerve symptoms. Although ocular manifestations are common, visual impairment due to optic nerve involvement is not commonly reported...
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... computed tomography scan, performed at another clinic 2 days prior to admission to our clinic, demonstrated normal findings. The complete blood count and biochem- istry analysis were normal. The antinuclear antibody test (ANA), performed to rule out a connective tissue disease, was negative. Results of agglutination tests for Brucella in serum and CSF were negative and the angiotensin con- verting enzyme in the serum was also negative. The brain MRI was normal. The contrast-enhanced orbital MRI revealed increased intensity in fat-saturated T2-weighted images and abnormal contrast enhancement of the left optic nerve (Fig. 1a-c). The cerebral MR venography was nor- mal. A visual evoked potential (VEP) test showed no response in the left eye, and normal P100 latency and amplitude in the right eye (Fig. ...
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Background and purpose:
Spontaneous intracranial hypotension (SIH) is a known cause of headaches and neurologic symptoms, but the frequency of cranial nerve symptoms and abnormalities on magnetic resonance imaging (MRI) has not been well described. The purpose of this study was to document cranial nerve findings in patients with SIH and determine the relationship between imaging findings and clinical symptoms.
Methods:
Patients diagnosed with SIH with pre-treatment brain MRI at a single institution from September 2014 to July 2017 were retrospectively reviewed to determine the frequency of clinically significant visual changes/diplopia (cranial nerves 3 and 6) and hearing changes/vertigo (cranial nerve 8). A blinded review of brain MRIs before and after treatment was conducted to assess for abnormal contrast enhancement of cranial nerves 3, 6, and 8. Imaging results were correlated with clinical symptoms.
Results:
Thirty SIH patients with pre-treatment brain MRI were identified. Sixty-six percent of patients had vision changes, diplopia, hearing changes, and/or vertigo. Cranial nerve 3 and/or 6 enhancement was present in nine patients on MRI, with 7/9 patients experiencing visual changes and/or diplopia (odds ratio [OR] 14.9, 95% confidence interval [CI] 2.2-100.8, p = .006). Cranial nerve 8 enhancement was present in 20 patients on MRI, with 13/20 patients experiencing hearing changes and/or vertigo (OR 16.7, 95% CI 1.7-160.6, p = .015).
Conclusions:
SIH patients with cranial nerve findings on MRI were more likely to have associated neurologic symptoms than those without imaging findings. Cranial nerve abnormalities on brain MRI should be reported in suspected SIH patients as they may support the diagnosis and explain patient symptoms.
Pain occurs with optic neuropathies associated with inflammatory central nervous system diseases (MS and NMO), idiopathic intracranial hypertension and spontaneous hypotension, giant cell arteritis, immunomediated systemic diseases, compressive lesions, or infective disorders. Pain can precede the onset of visual loss in acute optic neuritis, it can be irradiated to the orbital region in giant cell arteritis and parasellar compressive optic neuropathies, or it may be located to the back of the eye with posterior scleritis. History of symptoms together with complete neuro-ophthalmological examination must guide the differential diagnosis and neuroimaging. Painful visual loss due to different pathophysiological mechanisms requires specific treatment and prognosis.
Importance: Acetazolamide is commonly used to treat idiopathic intracranial hypertension (IIH), but there is insufficient information to establish an evidence base for its use. Objective: To determine whether acetazolamide is beneficial in improving vision when added to a low-sodium weight-reduction diet in patients with IIH and mild visual loss. Design, Setting, and Participants: A multicenter, randomized, double-masked, placebo-controlled study of acetazolamide in 165 participants with IIH and mild visual loss who received a low-sodium weight-reduction diet. Participants were enrolled at 38 academic and private practice sites in North America from March 2010 to November 2012 and followed up for 6 months (last visit in June 2013). All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation (PMD) between −2 and −7 dB. The mean age was 29 years, and all but 4 participants were women. Interventions: Low-sodium weight-reduction diet plus the maximally tolerated dosage of acetazolamide (up to 4 g/d) or matching placebo for 6 months. Main Outcomes and Measures: The planned primary outcome variable was the change in PMD from baseline to Month 6 in the most affected eye, as measured using a Humphrey field analyzer. PMD is a measure of global visual field loss (mean deviation from age-corrected normal values), with a range of 2 to −32 dB; larger negative values indicate greater vision loss. Secondary outcome variables included changes in papilledema grade, quality of life (Visual Function Questionnaire 25 [VFQ-25] and 36-Item Short Form Health Survey), headache disability, and weight at Month 6. Results: The mean improvement in PMD was greater with acetazolamide (1.43 dB, from −3.53 at baseline to −2.10 dB at Month 6; n = 86) than with placebo (0.71 dB, from −3.53 to −2.82 dB; n = 79); the difference was 0.71 dB (95% confidence interval [CI]: 0–1.43 dB; P = 0.050). Mean improvements in papilledema grade (acetazolamide: −1.31, from 2.76 to 1.45; placebo: −0.61, from 2.76 to 2.15; treatment effect: −0.70; 95% CI: −0.99 to −0.41; P < 0.001) and vision-related quality of life as measured by the National Eye Institute VFQ-25 (acetazolamide: 8.33, from 82.97 to 91.30; placebo: 1.98, from 82.97 to 84.95; treatment effect: 6.35; 95% CI: 2.22–10.47; P = 0.003) and its 10-item neuro-ophthalmic supplement (acetazolamide: 9.82, from 75.45 to 85.27; placebo: 1.59, from 75.45 to 77.04; treatment effect: 8.23; 95% CI: 3.89–12.56; P < 0.001) were also observed with acetazolamide. Participants assigned to acetazolamide also experienced a reduction in weight (acetazolamide: −7.50 kg, from 107.72 to 100.22 kg; placebo: −3.45 kg, from 107.72 to 104.27 kg; treatment effect: −4.05 kg; 95% CI: −6.27 to −1.83 kg; P < 0.001). Conclusions and Relevance: In patients with IIH and mild visual loss, the use of acetazolamide with a low-sodium weight-reduction diet compared with a diet alone resulted in modest improvement in visual field function. The clinical importance of this improvement remains to be determined.
