The Spectrum of Vestibular Migraine: Clinical Features, Triggers, and Examination Findings.

OBJECTIVE: To assess the ictal symptoms, interictal symptoms, psychiatric comorbidities, and interictal neuro-otologic examination findings in vestibular migraine (VM). METHODS: Retrospective chart review of 491 patients seen from August 2014 until March 2018 at a tertiary neurology referral center for vestibular disorders to identify patients fulfilling the 2012 VM criteria. RESULTS: One hundred and thirty-one patients (105 women) were identified. Mean age of VM onset was 44.3 (±13.7) years. Preceding the onset of vestibular symptoms, most had migraine (57.3%) and motion sickness (61.1%). It was common to have a family history of migraine (50.8%) and episodic vestibular symptoms (28.1%). Common ictal symptoms were triggered (visually induced and head-motion) and spontaneous vertigo, accompanied by photophobia and phonophobia (118/131 [90.1%] patients), nausea (105/131 [80.2%] patients), aural symptoms (79/131 [60.3%] patients), and headache (65/131 [49.6%] patients). Interictally, many experienced visually induced (116/131 [88.6%] patients), head-motion (86/131 [65.6%] patients), and persistent (67/131 [51.1%] patients) dizziness. Psychiatric comorbidities include anxiety (92/131 [70.2%] patients), depression (53/131 [40.5%] patients), insomnia (38/131 [29.0%] patients), phobic disorders (15/131 [11.5%] patients), and psychogenic disorders (11/131 [8.4%] patients). Common triggers were stress (52/131 [39.7%] patients), bright lights (35/131 [26.7%] patients), weather changes (34/131 [26.0%] patients), and sleep deprivation (34/131 [26.0%] patients). Interictal neuro-otologic examination was abnormal in 56/131 (42.7%), usually hyperventilation-induced, head-shaking-induced, vibration-induced, and positional nystagmus. The most common balance-test finding was impaired sharpened Romberg's test (22/130 [16.9%] patients). CONCLUSIONS: In this single center study, we found that VM typically affects women in their 40s, with a personal and family history of migraine. Typical ictal symptoms were triggered and spontaneous vertigo, associated with photophobia and phonophobia, nausea, aural symptoms, and headache. Interictal vestibular symptoms, comorbid psychiatric disorders, and non-specific interictal neuro-otologic findings were common.

Neuro-Ophthalmic Symptoms of Primary Headache Disorders: Why the Patient With Headache May Present to Neuro-Ophthalmology.

BACKGROUND: Primary headache disorders can cause many ophthalmic symptoms that lead many patients to present for neuro-ophthalmic evaluation. Neuro-ophthalmologists frequently encounter these patients in clinical practice. EVIDENCE ACQUISITION: A literature review was completed in PubMed using the following terms paired with "migraine" and "headache:" dry eye, eye pain, monocular diplopia, binocular diplopia, photophobia, visual field defect, tunnel vision, floaters, amaurosis fugax, transient visual obscuration, autonomic symptoms, anisocoria, visual snow, Alice in Wonderland syndrome, and palinopsia. RESULTS: Patients with migraine experience a wide range of visual disturbances including aura and more complex perceptual abnormalities such as Alice in Wonderland syndrome and visual snow. Visual disturbances may consist of positive and/or negative phenomena and may be binocular or monocular. Migraine and other primary headache disorders can be associated with photophobia, eye pain, dry eye, autonomic features, and anisocoria. CONCLUSIONS: Patients with primary headache disorders may experience a wide range of visual and ophthalmic symptoms. An understanding of the typical features of these disorders allows providers to help patients find appropriate treatment without unnecessary testing and to recognize when atypical presentations require additional evaluation.

Relapsing Painful Ophthalmoplegic Neuropathy: No longer a "Migraine," but Still a Headache.

PURPOSE OF REVIEW: Recurrent painful ophthalmoplegic neuropathy (RPON), formerly known as ophthalmoplegic migraine, is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. This review discusses the clinical presentation, current understanding of the pathophysiology, key differential diagnoses, and evaluation and treatment of RPON. RECENT FINDINGS: The literature is limited due to the rarity of the disorder. Recent case reports and series continue to suggest the age of first attack is most often during childhood or adolescence as well as a female predominance. Multiple recent case reports and series demonstrate focal enhancement of the affected cranial nerve, as the nerve root exits the brainstem. This finding contributed to the current classification of the disorder as a neuropathy, with the present understanding that it is due to a relapsing-remitting inflammatory or demyelinating process. The link to migraine remains a cause of disagreement in the literature. RPON is a complex disorder with features of inflammatory neuropathy and an unclear association with migraine. Regardless, the overall prognosis is good for individual episodes, but permanent nerve damage may accumulate with repeated attacks. A better understanding of the pathogenesis is needed to clarify whether it truly represents a single disorder and to guide its treatment. Until that time, a combined approach with acute and preventive therapies can mitigate acute symptoms as well as attempt to limit recurrence of this disabling syndrome.

Perineural spread of basosquamous carcinoma to the orbit, cavernous sinus, and infratemporal fossa.

Basosquamous carcinoma is a rare, highly aggressive variant of basal cell carcinoma with elevated rates of recurrence, perineural invasion, and metastasis. We describe a patient who presented with unilateral complete ophthalmoplegia, ptosis, optic neuropathy, and trigeminal neuropathy due to perineural intracranial invasion of nasal basosquamous carcinoma via the sphenopalatine fossa to the cavernous sinus, orbit, and infratemporal fossa. To our knowledge, this is the first reported case of basosquamous carcinoma with perineural invasion involving the cavernous sinus in the English language ophthalmic literature. Physicians should be aware of the diagnostic features, clinical challenges and aggressive nature of basosquamous carcinoma, a rare diagnosis that can lead to significant morbidity/mortality when left unrecognized.

Metastatic Melanoma of the Optic Nerve Sheath.

A 23-year-old man with a history of metastatic melanoma developed painful vision loss to counting fingers with enhancement of optic nerve on contrast-enhanced magnetic resonance imaging (MRI) and received a diagnosis of optic neuritis from an outside hospital. Despite empiric corticosteroid therapy, the patient worsened and developed secondary central retinal vein occlusion with further deterioration of vision. Repeat MRI demonstrated optic nerve sheath (ONS) involvement suggestive of optic perineuritis (OPN) and an ONS biopsy confirmed a rare case of isolated metastatic melanoma. Our case highlights the clinical and radiographic features that can mimic OPN and delay diagnosis and treatment.

The Idiopathic Intracranial Hypertension Treatment Trial: A Review of the Outcomes.

OBJECTIVE: The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) was the first large, randomized study on the use of acetazolamide and weight loss for treatment of idiopathic intracranial hypertension-associated vision loss. The multicenter trial also examined a number of secondary outcomes. This review summarizes all available results of the study published in the literature since 2014. BACKGROUND: Prior to the IIHTT, clinicians managed idiopathic intracranial hypertension based on data from small, unmasked trials, expert opinion, and clinical experience. Due to the lack of empiric evidence, there were no official treatment protocols to guide treatment of the disorder. METHODS: We performed a PubMed literature search for all articles with data from the IIHTT Study Group. After review of each article and any relevant supporting literature, the results were compiled into a summary of the literature. RESULTS: The PubMed search identified 14 articles with primary and/or secondary outcome data from the IIHTT. We summarized the findings for the primary outcome of visual field outcomes in the acetazolamide treatment group compared to the placebo group, as well as secondary outcomes for the safety and tolerability of acetazolamide, cerebrospinal fluid opening pressure, quality of life, fundus photography, and optical coherence tomography. While both groups demonstrated improvement on most outcomes, acetazolamide had a greater effect even when controlling for its effect on weight loss. CONCLUSIONS: As the first large, randomized, prospective trial, the IIHTT extensively expanded the available data on idiopathic intracranial hypertension treatment. Most importantly, it provided support for the safe use of acetazolamide up to 4 g daily with weight loss for effective treatment of mild vision loss in IIH, with associated improvements in papilledema, increased intracranial pressure, and quality of life.

The QuantiFERON-TB Gold In-Tube Assay in Neuro-Ophthalmology.

BACKGROUND: Although QuantiFERON-TB Gold In-Tube (QFT-GIT) testing is regularly used to detect infection with Mycobacterium tuberculosis, its utility in a patient population with a low risk for tuberculosis (TB) has been questioned. The following is a cohort study analyzing the efficacy of QFT-GIT testing as a method for detection of active TB disease in low-risk individuals in a neuro-ophthalmologic setting. METHODS: Ninety-nine patients from 2 neuro-ophthalmology centers were identified as having undergone QFT-GIT testing between January 2012 and February 2016. Patients were divided into groups of negative, indeterminate, and positive QFT-GIT results. Records of patients with positive QFT-GIT results were reviewed for development of latent or active TB, as determined by clinical, bacteriologic, and/or radiographic evidence. RESULTS: Of the 99 cases reviewed, 18 patients had positive QFT-GIT tests. Of these 18 cases, 12 had documentation of chest radiographs or computed tomography which showed no evidence for either active TB or pulmonary latent TB infection (LTBI). Four had chest imaging which was indicative of possible LTBI. None of these 18 patients had symptoms of active TB and none developed active TB within the follow-up period. CONCLUSIONS: Based on our results, we conclude that routine testing with QFT-GIT in a low-risk cohort did not diagnose active TB infection. We do not recommend routine QFT-GIT testing for TB low-risk individuals, as discerned through patient and exposure history, ocular examination, and clinical judgment, in neuro-ophthalmology practice.

Idiopathic Orbital Inflammation Associated With Relapsing Polychondritis.

The authors describe a patient with recurrent idiopathic orbital inflammatory disease as an unusual presentation of relapsing polychondritis. There are very few reported cases in the literature of relapsing polychondritis associated with idiopathic orbital inflammation. Clinicians should be aware of the orbital and ophthalmic presentations of relapsing polychondritis.

Clinical characteristics of Alice in Wonderland syndrome in a cohort with vestibular migraine.

BACKGROUND: Alice in Wonderland syndrome (AIWS) is a rare sensory perception disorder, most often caused by migraine in adults. We aimed to characterize the clinical characteristics of AIWS in a cohort of vestibular migraine (VM) patients. METHODS: Retrospective chart review of patients diagnosed with VM seen between August 2014 and January 2018. RESULTS: Seventeen patients were identified (10 women) with a median age at onset of 45 years (range 15-61 years), and median age at presentation of 49 years (range 17-63 years). Eighty-two percent reported 1 AIWS symptom, 12% reported 3 symptoms, and 6% described 2 symptoms. The most common symptom was visual distortions (47%), followed by extrapersonal misperceptions (41%) and somesthetic distortions (29%). Most AIWS occurred during VM episodes (77%). Eleven patients were seen in follow-up; 10 described complete or partial resolution of both AIWS and VM with migraine preventive therapy, while 1 experienced complete resolution of VM but continued to have AIWS. Neuro-otologic abnormalities improved in 2 patients. CONCLUSIONS: This study characterizes the clinical features of AIWS in patients with VM. We observed several rare and highly unusual AIWS misperceptions (frosted-glass vision, underwater vision, dolly zoom effect, sensation of the brain coming out of the head, closed-eye visual hallucinations, and headlight glare-induced marco/microsomatognosia), and resolution or improvement in AIWS and VM with migraine preventive treatment.

Pathologic Markers Determining Prognosis in Patients With Treated or Healing Giant Cell Arteritis.

PURPOSE: To provide quantitative evidence linking the CD68 (cluster of differentiation 68)+ macrophage marker found on temporal artery biopsies (TABs) with disease prognosis. DESIGN: Retrospective, cross-sectional study. METHODS: We examined 42 consecutive patients who had undergone unilateral TABs at a single hospital in 2015. Clinical data, laboratory data, and histopathologic features of TABs were recorded. Inclusion criteria were clinical diagnosis of giant cell arteritis (GCA) with TAB performed at the same center. CD68 immunohistochemistry was used to label macrophages in the TABs. Primary outcome was multiple logistic regression and bivariate comparisons to measure the association between CD68+ cells per histologic section with placement on immunomodulatory therapy (IMT). RESULTS: Twenty seven patients were female (64%), with a mean age of 72 (standard deviation [SD.] ±7.7). Eleven patients (26%) were placed on IMT, 17 (40%) had disease recurrence during steroid taper, and 25 (60%) were referred to rheumatology. Of 42 biopsies, 35 underwent staining with CD68 to confirm active inflammation in suspicious, but not diagnostic, specimens. Patients eventually placed on IMT had increased CD68+ cells per slice compared to those not on IMT (median 5.00 [25th-75th quartile 2.00-7.15] vs 1.21 [0.38-2.57], P = .031, respectively). A receiver operating characteristic (ROC) curve demonstrates that 2.17 CD68+ cells/slice predicts placement on IMT with an odds ratio of 1.54 (95% confidence interval 1.02-2.33, P = .038). CONCLUSIONS: Patients refractory to initial steroid tapers and those eventually placed on IMT had increased CD68 cells per section. CD68+ macrophages and their location on the internal elastic lamina may predict disease severity in patients with presumed GCA. Our results suggest that this marker may expedite patient triaging to alternate treatment to the usual steroid therapy.

Update on Ocular Myasthenia Gravis.

Ocular myasthenia gravis (OMG) is a localized form of myasthenia gravis in which autoantibodies directed against acetylcholine receptors block or destroy these receptors at the postsynaptic neuromuscular junction. The hallmark of OMG is a history of painless weakness or fatigability of the extraocular muscles and ptosis with normal pupillary function and visual acuity. Clinical, laboratory, electrophysiologic, and pharmacologic tests are available for diagnosis. Treatment can begin with symptom management; there is no cure. Prognosis is improved by use of immunomodulators. Despite advances in treatment, research is needed, especially in the areas of surgical intervention and medical therapy based on risk stratification.

Evaluation and management of the swollen optic disk in cryptococcal meningitis.

Cryptococcal meningitis is the most common and severe form of cryptococcal infection. In addition to infiltrative and inflammatory mechanisms, intracranial hypertension commonly complicates cryptococcal meningitis and may cause significant visual and neurological morbidity and mortality. The mainstays of treatment for cryptococcal meningitis include standard antifungal therapy, management of intracranial hypertension, and treatment of underlying immunosuppressive conditions. Early and aggressive management of intracranial hypertension in accordance with established guidelines reduces the risk of long-term visual and neurological complications and death. Traditional recommendations for treating elevated intracranial pressure in idiopathic intracranial hypertension including acetazolamide, weight loss, and avoiding serial lumbar punctures-are not helpful in cryptococcal meningitis and may be harmful.

Neuro-Ophthalmology Cases for the Neurologist.

Neurologists should be aware of specific urgent and emergent neuro-ophthalmic conditions, including giant cell arteritis, arterial dissection, intracranial aneurysm, pituitary apoplexy, and invasive sino-orbital fungal infection (eg, mucormycosis). Early recognition and treatment can greatly impact patient morbidity and mortality, including the preservation of vision and life. Neurologists should be cognizant of the key and differentiating clinical and radiographic features for these presentations.