Ocular vs neurosyphilis. are they the same? A guide to investigation and management.

This article reviews key concepts in the epidemiology, clinical features, diagnosis and management of ocular syphilis. It is not a systematic review or meta-analysis, but highlights the critical clinical features and investigations in patients with ocular syphilis. It reviews the overlap and interplay between ocular and neuro syphilis and provides practical guidance to diagnose and manage patients with ocular syphilis.

One simple question detects motion sickness susceptibility in migraine patients.

OBJECTIVE: To find out if motion sickness susceptibility (MSS) of vestibular migraine (VM) patients and migraine only (MO) patients can be reliably detected with a single simple question: "Can you read while travelling in a car without getting motion sick?". METHOD: Ninety-two definite VM and 58 MO patients and 74 healthy control (HC) subjects were asked about their MSS and about being able to read while riding in a car without becoming motion sick. A Motion Sickness Susceptibility Questionnaire (MSSQ-Short) including childhood (MSA), adulthood (MSB) and total (MST) parts was also administered to all participants. ROC curves of MSSQ-Short were prepared for "not being able to read in a car" as the gold standard. RESULTS: Mean MSA scores were significantly higher in both VM and MO patients than in HCs (p < 0.001), but their scores were not significantly different (p = 0.171). Mean MSB and MST scores were significantly higher in VM than in MO patients (p < 0.001) and both VM and MO patients had significantly higher scores than HCs (p < 0.001). MSA scores were significantly higher than MSB scores in MO patients (p < 0.001). All sections of the questionnaire were associated with high area-under-curve values for MSS detected by the question about being able to read in a car without becoming motion sick. CONCLUSION: We propose that all migraine patients could have the same level of MSS in childhood but MO patients are able to compensate over years, but VM patients are not. A quick way to determine MSS is to ask about the ability to read without becoming motion sickness while riding a car.

One simple question detects motion sickness susceptibility in migraine patients

Abstract Objective To find out if motion sickness susceptibility (MSS) of vestibular migraine (VM) patients and migraine only (MO) patients can be reliably detected with a single simple question: "Can you read while travelling in a car without getting motion sick?". Method Ninety-two definite VM and 58 MO patients and 74 healthy control (HC) subjects were asked about their MSS and about being able to read while riding in a car without becoming motion sick. A Motion Sickness Susceptibility Questionnaire (MSSQ-Short) including childhood (MSA), adulthood (MSB) and total (MST) parts was also administered to all participants. ROC curves of MSSQ-Short were prepared for "not being able to read in a car" as the gold standard. Results Mean MSA scores were significantly higher in both VM and MO patients than in HCs (p< 0.001), but their scores were not significantly different (p= 0.171). Mean MSB and MST scores were significantly higher in VM than in MO patients (p< 0.001) and both VM and MO patients had significantly higher scores than HCs (p< 0.001). MSA scores were significantly higher than MSB scores in MO patients (p< 0.001). All sections of the questionnaire were associated with high area-under-curve values for MSS detected by the question about being able to read in a car without becoming motion sick. Conclusion We propose that all migraine patients could have the same level of MSS in childhood but MO patients are able to compensate over years, but VM patients are not. A quick way to determine MSS is to ask about the ability to read without becoming motion sickness while riding a car.

The Relationship between the Subjective Visual Horizontal and Ocular Vestibular Evoked Myogenic Potentials in Acute Vestibular Neuritis.

OBJECT: Vestibular evoked myogenic potentials (VEMPs) and the subjective visual horizontal (SVH) (or vertical [SVV]) have both been considered tests of otolith function: ocular-VEMPs (oVEMPs) utricular function, cervical VEMPs (cVEMPs) saccular function. Some studies have reported association between decreased oVEMPs and SVH, whereas others have not. DESIGN: A retrospective study of test results. SETTING: A tertiary, neuro-otology clinic, Royal Prince Alfred Hospital, Sydney, Australia. METHOD: We analyzed results in 130 patients with acute vestibular neuritis tested within 5 days of onset. We sought correlations between the SVH, oVEMPs, and cVEMPs to air-conducted (AC) and bone-conducted (BC) stimulation. RESULTS: The SVH deviated to the side of lesion, in 123 of the 130 AVN patients, by 2.5 to 26.7 degrees. Ninety of the AVN patients (70%) had abnormal oVEMPs to AC, BC or both stimuli, on the AVN side (mean asymmetry ratio ± SD [SE]): (64 ± 45.0% [3.9]). Forty-three of the patients (35%) had impaired cVEMPs to AC, BC or both stimuli, on the AVN side, [22 ± 41.6% (4.1)]. The 90 patients with abnormal oVEMP values also had abnormal SVH. Correlations revealed a significant relationship between SVH offset and oVEMP asymmetry (r = 0.80, p < 0.001) and a weaker relationship between SVH offset and cVEMP asymmetry (r = 0.56, p < 0.001). CONCLUSIONS: These results indicate that after an acute unilateral vestibular lesion, before there has been a chance for vestibular compensation to occur, there is a significant correlation between the SVH, and oVEMP results. The relationship between SVH offset and oVEMP amplitude suggests that both tests measure utricular function.

Adult Spinal Primary Leptomeningeal Medulloblastoma Presenting as Pseudotumour Cerebri Syndrome.

A previously well 34-year-old man presented with severe pseudotumour cerebri. Imaging showed that he had a cauda equina tumour which proved to be a medulloblastoma. There was no tumour mass in the posterior fossa so we assume that this was a primary leptomeningeal medulloblastoma. In patients with somewhat atypical pseudotumour, spinal imaging should always be considered.

Selective Bilateral Vestibular Neuropathy in a Turkish CMT1B Family With a Novel MPZ Mutation.

PURPOSE OF REVIEW: To report the findings in 12 members over 3 generations of a family with dominantly inherited Charcot-Marie-Tooth disease (CMT1B) due to a novel MPZ mutation, who all had moderately severe selective impairment of vestibular function with normal hearing. Methods used were video head impulse testing of the function of all 6 semicircular canals, Romberg test on foam, nerve conduction studies, and whole exome and Sanger sequencing. RECENT FINDINGS: All affected patients had a demyelinating neuropathy and a novel MPZ mutation: c.362A>G (chr1: 161276584, p.D121G). All also had normal hearing for age but a moderately severe impairment of semicircular canal function and a positive Romberg test on foam. SUMMARY: Some CMT mutations can impair vestibular function, presumably because of a vestibular nerve involvement but spare hearing. In such patients, impairment of vestibular function and impairment of proprioception contribute to imbalance.

The Pathology of the Vestibular System in CANVAS.

OBJECTIVE: To describe the site of lesion responsible for the severe, bilateral, symmetrical, selective loss of vestibular function in Cerebellar Ataxia with Neuronopathy and Vestibular Areflexia Syndrome (CANVAS), an adult-onset recessively-inherited ataxia, characterized by progressive imbalance due to a combination of cerebellar, somatosensory, and selective vestibular impairment with normal hearing. METHODS: Histologic examination of five temporal bones and the brainstems from four CANVAS patients and the brainstem only from one more, each diagnosed and followed from diagnosis to death by one of the clinician authors. RESULTS: All five temporal bones showed severe loss of vestibular ganglion cells (cell counts 3-16% of normal), and atrophy of the vestibular nerves, whereas vestibular receptor hair cells and the vestibular nuclei were preserved. In contrast, auditory receptor hair cells, the auditory ganglia (cell counts 51-100% of normal), and the auditory nerves were relatively preserved. In addition, the cranial sensory ganglia (geniculate and trigeminal), present in two temporal bones, also showed severe degeneration. CONCLUSIONS: In CANVAS there is a severe cranial sensory ganglionopathy neuronopathy (ganglionopathy) involving the vestibular, facial, and trigeminal ganglia but sparing the auditory ganglia. These observations, when coupled with the known spinal dorsal root ganglionopathy in CANVAS, indicate a shared pathogenesis of its somatosensory and cranial nerve manifestations. This is the first published account of both the otopathology and neuropathology of CANVAS, a disease that involves the central as well as the peripheral nervous system.

Optic nerve sheath decompression saves sight in severe papilloedema: results from 81 eyes in 56 patients with pseudotumor cerebri.

PURPOSE: To report the outcome of optic nerve sheath decompression (ONSD) for papilloedema in a teaching hospital in western Turkey. METHODS: The charts of 56 patients who had ONSD surgery between April 2007 and September 2019 were collated; and a total of 81 operated and 31 fellow eyes were included. Pre- and postoperative ophthalmologic examination including best-corrected visual acuity (BCVA), colour vision (CV), visual field (VF) analysis, fundoscopic examination and demographic and medical characteristics of the patients were noted and outcomes after surgery were investigated. RESULTS: Of all study eyes, 49 (43.7%) eyes had BCVA 0.2 or less and 62 (55.3%) eyes had mean deviation (MD) below - 20.0 dB. 62 (55.3%) eyes had Frisen grade 4 or 5 papilloedema. Almost half of the eyes had severe vision loss. After ONSD, BCVA, CV and MD in both operated and fellow non-operated eyes improved significantly (p < 0.001, p = 0.009 and p < 0.001 for operated, p < 0.001, p = 0.007 and p < 0.001 for fellow eyes, respectively). Earlier surgery and higher cerebrospinal fluid opening pressure were related to better outcomes. None of the patients had major operative complications. CONCLUSION: Optic nerve sheath decompression can safely improve vision not only of the operated but also of the non-operated eye, even in cases with severe vision loss from severe bilateral papilloedema. Regardless of initial VA and VF, patients may benefit from ONSD; the earlier it is done the more likely the better outcome.

Spontaneous Recovery of the Vestibulo-Ocular Reflex After Vestibular Neuritis; Long-Term Monitoring With the Video Head Impulse Test in a Single Patient.

Vestibular rehabilitation of patients in whom the level of vestibular function is continuously changing requires different strategies than in those where vestibular function rapidly becomes stable: where it recovers or where it does not and compensation is by catch-up saccades. In order to determine which of these situations apply to a particular patient, it is necessary to monitor the vestibulo-ocular reflex (VOR) gains, rather than just make a single measurement at a given time. The video Head Impulse Test (vHIT) is a simple and practical way to monitor precisely the time course and final level of VOR recovery and is useful when a patient has ongoing vestibular symptoms, such as after acute vestibular neuritis. In this study, we try to show the value of ongoing monitoring of vestibular function in a patient recovering from vestibular neuritis. Acute vestibular neuritis can impair function of any single semicircular canal (SCC). The level of impairment of each SCC, initially anywhere between 0 and 100%, can be accurately measured by the vHIT. In superior vestibular neuritis the anterior and lateral SCCs are the most affected. Unlike after surgical unilateral vestibular deafferentation, SCC function as measured by the VOR can recover spontaneously after acute vestibular neuritis. Here we report monitoring the VOR from all 6 SCCs for 500 days after the second attack in a patient with bilateral sequential vestibular neuritis. Spontaneous recovery of the VOR in response to anterior and lateral SCC impulses showed an exponential recovery with a time to reach stable levels being longer than previously considered or reported. VOR gain in response to low-velocity lateral SCC impulses recovered with a time constant of around 100 days and reached a stable level at about 200 days. However, in response to high-velocity lateral SCC and anterior SCC impulses, VOR gain recovered with a time constant of about 150 days and only reached a stable level toward the end of the 500 days monitoring period.

Invasive Fungal Sinusitis Presenting as Acute Posterior Ischemic Optic Neuropathy.

Invasive fungal sinusitis causes painful orbital apex syndrome with ophthalmoplegia and visual loss; the mechanism is unclear. We report an immunocompromised patient with invasive fungal sinusitis in whom the visual loss was due to posterior ischaemic optic neuropathy, shown on diffusion-weighted MRI, presumably from fungal invasion of small meningeal-based arteries at the orbital apex. After intensive antifungal drugs, orbital exenteration and immune reconstitution, the patient survived, but we were uncertain if the exenteration helped. We suggest that evidence of acute posterior ischaemic optic neuropathy should be a contra-indication to the need for orbital exenteration in invasive fungal sinusitis.

Delayed relapse in pseudotumor cerebri due to new stenosis after transverse sinus stenting.

A patient presented with recurrent severe pseudotumor cerebri (PTC). Transverse sinus stenting is a very effective treatment option, however stenosis and intracranial hypertension can recur. In our patient, stenting initially resulted in resolution of papilloedema. However, after 5 years, a new stenosis developed which required further stenting. This case highlights the fact that, in patients with PTC who undergo transverse sinus stenting, a small proportion require repeat treatment due to formation of a new stenosis, usually adjacent to the existing stent. Patients with severe disease, such as ours, may be at higher risk of recurrence. Regardless of the severity, all patients who undergo stenting should have regular ocular follow-up.

A 43-year-old woman presenting with subacute, bilateral, sequential facial nerve palsies, then developing pseudotumour cerebri.

A patient presented elsewhere with what appeared to be a simple, unilateral, chronic suppurative otitis media and then developed an ipsilateral facial palsy. She soon developed the same problem on the other side. At the time, a brain MRI had been ordered but the clinician did not review it with a radiologist. The surgical specimens were not sent for histopathology. When transferred to our institution 3 months later, the patient had severe bilateral papilloedema due to intracranial hypertension due to missed cerebral venous sinus thrombosis. Further surgery revealed that the pathology in the temporal bone was B-cell lymphoma, which, fortunately, responded to chemoradiotherapy. There was good resolution of the facial palsies, but the patient has severe permanent visual loss due to optic atrophy.