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.

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.

Modeling the interaction among three cerebellar disorders of eye movements: periodic alternating, gaze-evoked and rebound nystagmus.

A woman, age 44, with a positive anti-YO paraneoplastic cerebellar syndrome and normal imaging developed an ocular motor disorder including periodic alternating nystagmus (PAN), gaze-evoked nystagmus (GEN) and rebound nystagmus (RN). During fixation there was typical PAN but changes in gaze position evoked complex, time-varying oscillations of GEN and RN. To unravel the pathophysiology of this unusual pattern of nystagmus, we developed a mathematical model of normal function of the circuits mediating the vestibular-ocular reflex and gaze-holding including their adaptive mechanisms. Simulations showed that all the findings of our patient could be explained by two, small, isolated changes in cerebellar circuits: reducing the time constant of the gaze-holding integrator, producing GEN and RN, and increasing the gain of the vestibular velocity-storage positive feedback loop, producing PAN. We conclude that the gaze- and time-varying pattern of nystagmus in our patient can be accounted for by superposition of one model that produces typical PAN and another model that produces typical GEN and RN, without requiring a new oscillator in the gaze-holding system or a more complex, nonlinear interaction between the two models. This analysis suggest a strategy for uncovering gaze-evoked and rebound nystagmus in the setting of a time-varying nystagmus such as PAN. Our results are also consistent with current ideas of compartmentalization of cerebellar functions for the control of the vestibular velocity-storage mechanism (nodulus and ventral uvula) and for holding horizontal gaze steady (the flocculus and tonsil).

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.

Search for SCA2 blood RNA biomarkers highlights Ataxin-2 as strong modifier of the mitochondrial factor PINK1 levels.

Ataxin-2 (ATXN2) polyglutamine domain expansions of large size result in an autosomal dominantly inherited multi-system-atrophy of the nervous system named spinocerebellar ataxia type 2 (SCA2), while expansions of intermediate size act as polygenic risk factors for motor neuron disease (ALS and FTLD) and perhaps also for Levodopa-responsive Parkinson's disease (PD). In view of the established role of ATXN2 for RNA processing in periods of cell stress and the expression of ATXN2 in blood cells such as platelets, we investigated whether global deep RNA sequencing of whole blood from SCA2 patients identifies a molecular profile which might serve as diagnostic biomarker. The bioinformatic analysis of SCA2 blood global transcriptomics revealed various significant effects on RNA processing pathways, as well as the pathways of Huntington's disease and PD where mitochondrial dysfunction is crucial. Notably, an induction of PINK1 and PARK7 expression was observed. Conversely, expression of Pink1 was severely decreased upon global transcriptome profiling of Atxn2-knockout mouse cerebellum and liver, in parallel to strong effects on Opa1 and Ghitm, which encode known mitochondrial dynamics regulators. These results were validated by quantitative PCR and immunoblots. Starvation stress of human SH-SY5Y neuroblastoma cells led to a transcriptional phasic induction of ATXN2 in parallel to PINK1, and the knockdown of one enhanced the expression of the other during stress response. These findings suggest that ATXN2 may modify the known PINK1 roles for mitochondrial quality control and autophagy during cell stress. Given that PINK1 is responsible for autosomal recessive juvenile PD, this genetic interaction provides a concept how the degeneration of nigrostriatal dopaminergic neurons and the Parkinson phenotype may be triggered by ATXN2 mutations.

Neuro-ophthalmology of invasive fungal sinusitis: 14 consecutive patients and a review of the literature.

BACKGROUND: Invasive fungal sinusitis is a rare condition that usually occurs in immunocompromised patients and often presents as an orbital apex syndrome. It is frequently misdiagnosed on presentation and is almost always lethal without early treatment. DESIGN: Retrospective case series of 14 consecutive patients with biopsy-proven invasive fungal sinusitis from four tertiary hospitals. PARTICIPANTS: Fourteen patients (10 men and 4 women; age range 46-82 years). METHODS: Retrospective chart review of all patients presenting with invasive fungal sinusitis between 1994 and 2010 at each hospital, with a close analysis of the tempo of the disease to identify any potential window of opportunity for treatment. MAIN OUTCOME MEASURES: Demographic data, background medical history (including predisposing factors), symptoms, signs, radiological findings, histopathological findings, treatment approach and subsequent clinical course were recorded and analysed. RESULTS: Only one patient was correctly diagnosed at presentation. Only two patients were not diabetic or immunocompromised. The tempo was acute in two patients, subacute in nine patients and chronic in three patients. In the subacute and chronic cases, there was about 1 week of opportunity for treatment, from the time there was a complete orbital apex syndrome, and still a chance for saving the patient, to the time there was central nervous system invasion, which was invariably fatal. Only two patients survived - both had orbital exenteration, as well as antifungal drug treatment. CONCLUSIONS: Invasive fungal sinusitis can, rarely, occur in healthy individuals and should be suspected as a possible cause of a progressive orbital apex syndrome.

Steroid-responsive recurrent limbic encephalitis associated with small cell lung cancer and neuropil antibodies.

Paraneoplastic limbic encephalitis (PLE) associated with small cell lung cancer (SCLC) often presents with antibodies to intracellular antigens and a poor outcome even after tumor resection and immunotherapy. We report a PLE patient presenting with generalized seizures, shortterm memory impairment and medial temporal lobe hyperintensity in MRI. Initial screening revealed significantly elevated thyroid antibody levels suggesting Hashimoto's encephalopathy. Following methylprednisolone treatment, her seizures ceased, MRI findings disappeared and memory impairment showed a partial resolution in 5 months. Two months later, she developed further generalized seizures. Chest X-ray showed a mass lesion, which was demonstrated by needle biopsy to be a small cell lung carcinoma (SCLC). The panel of onconeural antibodies including cell-membrane antigens was negative. However, the patient's serum and cerebrospinal fluid IgG, obtained during both exacerbations, immunolabeled cytoplasm and dendrites of Purkinje cells, cerebellar and hippocampal molecular layers, basal ganglia, thalamus, and the surface of cultured hippocampal neurons, in a manner distinct from previously identified neuropil antibodies associated with SCLC. These neuropil antibodies appear to be associated with a favorable response to treatment. Further studies are required for determination of the target antigen.

Immunologically mediated dementias.

Although most dementias are due to neurodegenerative or vascular disease, it is important to diagnose immunologically mediated dementias quickly because they can be both rapidly progressive and readily treatable. They usually affect function of limbic and cortical structures, but subcortical involvement can also occur. Because of the variety of symptoms and the rapid course, these dementias present a particular challenge to the clinician and may require evaluation and intervention in the inpatient setting. Diagnostic workup typically reveals evidence of an autoimmune process and, in some cases, cancer. In contrast to the neurodegenerative processes, many of the immunologically mediated dementias respond to immunomodulatory therapy.