Pearls & Oy-sters: A Challenging Optic Neuropathy-What Not to Miss in Optic Nerve Sheath Enhancement.

Optic neuropathies include a wide range of disorders from ischemic, toxic, demyelinating, or inflammatory processes with acute/subacute onset to more gradual compressive or genetic etiologies. Accurate clinical history and multimodality optic nerve imaging including MRI and optical coherence tomography have greatly improved the diagnosis of patients with optic neuropathies. We report a case of a woman with severe monocular visual acuity deficit. Optic nerve sheath enhancement seen on MRI led to a broad differential diagnosis including demyelinating causes, optic nerve sheath meningioma (ONSM), tuberculosis, and sarcoid optic neuropathy. Lack of response to treatment with steroids or plasmapheresis led to biopsy, which confirmed the diagnosis of ONSM.

Long-term follow-up of subthalamic nucleus deep brain stimulation in patients with Parkinson's disease: An analysis of survival and disability milestones.

BACKGROUND: Data on the long-term survival and incidence of disability milestones after subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD) is limited. OBJECTIVES: To estimate mortality and assess the frequency/time-to-development of disability milestones (falls, freezing, hallucinations, dementia, and institutionalization) among PD patients post STN-DBS. METHODS: A longitudinal retrospective study of patients undergoing STN-DBS. For mortality, Cox proportional hazards regression analysis was performed. For disease milestones, competing risk analyses were performed and cumulative incidence functions reported. The strength of association between baselines features and event occurrence was calculated based on adjusted hazard ratios. RESULTS: The overall mortality for the 109 patients was 16 % (62.1 ± 21.3 months after surgery). Falls (73 %) and freezing (47 %) were both the earliest (40.4 ± 25.4 and 39.6 ± 28.4 months, respectively) and most frequent milestones. Dementia (34 %) and hallucinations (32 %) soon followed (56.2 ± 21.2 and mean 60.0 ± 20.7 months after surgery, respectively). Higher ADL scores in the OFF state and higher age at surgery were associated with falls, freezing, dementia and institutionalization. CONCLUSIONS: Long-term mortality rate is low after STN-DBS. Disease milestones occur later during the disease course, with motor milestones appearing first and at a higher frequency than cognitive ones.

Dental Caries, Tooth Loss and Quality of Life of Individuals Exposed to Social Risk Factors in Northeast Brazil.

This study aimed to investigate the impact of dental caries and tooth loss on oral health-related quality of life (OHRQoL) in socioeconomically disadvantaged people. A population-based, cross-sectional survey was conducted in 28 cities at social risk in Northeast Brazil. All permanent residents aged 12, 15-19, 35-44, and 65-74 years were eligible, and 3063 were included. Participants answered a questionnaire on socioeconomic status, beliefs, and behaviors. Trained local dentists performed oral clinical examinations during home visits. Caries and tooth loss were evaluated using the decayed, missing, and filled teeth (DMFT) index and OHRQoL was evaluated using the Oral Health Impact Profile-14 (OHIP-14). Poisson regression analysis was performed to assess the relationship between individual domains, OHIP-14 scores, dental caries, tooth loss, and socioeconomic/demographic characteristics. Mean DMFT (standard deviation) scores were 2.68 (4.01), 4.84 (4.30), 15.35 (7.26), and 26.72 (8.03) for groups aged 12, 15-19, 35-44, and 65-74 years, respectively. Most participants (70%) were partially edentulous and 13% were completely edentulous. Caries and tooth loss significantly increased with age and impacted OHRQoL. Physical pain (5.8%) and psychological discomfort (5.8%) were the most commonly reported on the OHIP-14. Untreated caries (prevalence ratio (PR), 1.54; 95% confidence interval (CI), 1.37-1.72) and edentulism (PR, 1.29; 95% CI, 1.08-1.53) had a significant negative impact on OHRQoL. Income, level of education, sex, age, and oral hygiene habits were also related to OHRQoL. There was a high prevalence of dental caries and edentulism in all age groups except 12-year-olds. OHRQoL was negatively impacted by these oral conditions across the lifespan, with a trend towards more negative scores and higher impact in older adults.

Impact of brain biopsy on management of nonneoplastic brain disease.

Introduction: Diagnostic yield of brain biopsy in neoplastic brain disease is high and its clinical impact is well established. In nonneoplastic brain disease with negative conventional investigation, decision to undergo invasive procedures is difficult due to its inherent risk and known lower diagnostic yield. Research question: What is the clinical impact of brain biopsy results on management of nonneoplastic brain disease ? Material and methods: A multidisciplinary team retrospectively reviewed and included all nonneoplastic brain disease cases submitted to biopsy between 2009 and 2019, in a tertiary hospital in Lisbon. Baseline characteristics were registered, including immunosuppression status, diagnostic workup, and treatment prior to biopsy. Diagnostic yield, clinical impact and in-hospital complication rates were assessed. Results: Sixty-four patients were included, 20 (31.3%) of them immunosuppressed (15 HIV â€‹+ â€‹patients). Thirty-five (67.7%) were previously treated with steroids or antiinfectious agents, with higher percentage (93.3%) in the immunosuppressed group. Biopsy results were diagnostic in 46 (71.9%) cases. More frequent diagnosis was infectious in 20 (31.2%), neoplastic in 12 (18.8%) and inflammatory diseases in 8 (12.5%). Brain biopsy resulted on impact on patient's clinical management in 56 (87.5%), of which 37(57.8%) were submitted to treatment change. In-hospital complications were registered in 4 (6.6%) patients. Discussion and conclusion: Brain biopsy had clinical impact, including a change in treatment, in most patients studied, and may be considered a useful diagnostic option in nonneoplastic brain disease. However, associated complication rate is not negligible, and previous thorough workup, patient selection and risk-benefit assessment are important.

Longitudinally extensive transverse myelitis with anti-myelin oligodendrocyte glycoprotein antibodies following SARS-CoV-2 infection.

We report the case of a patient with symptoms of myelopathy following acute SARS-CoV-2 infection. MRI documented a longitudinally extensive transverse myelitis and further investigation was unremarkable with the exception of positivity for MOG-IgG in serum. This report extends the spectrum of post-COVID-19 neurological syndromes, and documents a very significant improvement to long-term oral corticosteroid therapy in this setting. Further prospective studies are needed to establish the risk of recurrence in this subset of patients.

Chemotherapy and diffuse low-grade gliomas: a survey within the European Low-Grade Glioma Network.

BACKGROUND: Diffuse low-grade gliomas (DLGGs) are rare and incurable tumors. Whereas maximal safe, functional-based surgical resection is the first-line treatment, the timing and choice of further treatments (chemotherapy, radiation therapy, or combined treatments) remain controversial. METHODS: An online survey on the management of DLGG patients was sent to 28 expert centers from the European Low-Grade Glioma Network (ELGGN) in May 2015. It contained 40 specific questions addressing the modalities of use of chemotherapy in these patients. RESULTS: The survey demonstrated a significant heterogeneity in practice regarding the initial management of DLGG patients and the use of chemotherapy. Interestingly, radiation therapy combined with the procarbazine, CCNU (lomustine), and vincristine regimen has not imposed itself as the gold-standard treatment after surgery, despite the results of the Radiation Therapy Oncology Group 9802 study. Temozolomide is largely used as first-line treatment after surgical resection for high-risk DLGG patients, or at progression. CONCLUSIONS: The heterogeneity in the management of patients with DLGG demonstrates that many questions regarding the postoperative strategy and the use of chemotherapy remain unanswered. Our survey reveals a high recruitment potential within the ELGGN for retrospective or prospective studies to generate new data regarding these issues.

Vernet syndrome resulting from varicella zoster virus infection-a very rare clinical presentation of a common viral infection.

Vernet syndrome is a unilateral palsy of glossopharyngeal, vagus, and accessory nerves. Varicella zoster virus (VZV) infection has rarely been described as a possible cause. A 76-year-old man presented with 1-week-long symptoms of dysphonia, dysphagia, and weakness of the right shoulder elevation, accompanied by a mild right temporal parietal headache with radiation to the ipsilateral ear. Physical examination showed signs compatible with a right XI, X, and XI cranial nerves involvement and also several vesicular lesions in the right ear's concha. He had a personal history of poliomyelitis and chickenpox. Laringoscopy demonstrated right vocal cord palsy. Brain MRI showed thickening and enhancement of right lower cranial nerves and an enhancing nodular lesion in the ipsilateral jugular foramen, in T1 weighted images with gadolinium. Cerebrospinal fluid (CSF) analysis disclosed a mild lymphocytic pleocytosis and absence of VZV-DNA by PCR analysis. Serum VZV IgM and IgG antibodies were positive. The patient had a noticeable clinical improvement after initiation of acyclovir and prednisolone therapy. The presentation of a VZV infection with isolated IX, X, and XI cranial nerves palsy is extremely rare. In our case, the diagnosis of Vernet syndrome as a result of VZV infection was made essentially from clinical findings and supported by analytical and imaging data.

A randomized triple-blind crossover trial of a hydrocolloid-containing dentifrice as a controlled-release system for fluoride.

OBJECTIVE: To evaluate retention of intraoral fluoride in biofilm and saliva, an experimental dentifrice containing hydrocolloid (tara gum) was used as a controlled-release system for fluoride (F). MATERIALS AND METHODS: In a triple-blind randomized crossover trial with washout, 18 individuals used the following different dentifrices for a week: 100-TGF (sodium fluoride NaF associated with tara gum, 1100 mg/L), 50-TGF (50% NaF associated with tara gum + 50% free NaF, 1100 mg/L), PC (free NaF, 1100 mg/L), TG (with tara gum and without F), and placebo (without F or tara gum). On the seventh day of dentifrice use, biofilm was collected at 1 and 12 h, and saliva was collected up to 60 min and 12 h after the last toothbrushing. F concentrations were determined by physico-chemical analysis of fluoride using the hexamethyldisiloxane-facilitated diffusion technique. Data were subjected to two-way analysis of variance (repeated measures) and Spearman's correlation coefficient (p < 0.05) testing. RESULTS: No significant difference was observed with the same dentifrice regarding F retention in biofilm at 1 and 12 h after toothbrushing for the 100-TGF, placebo, and TG groups (p > 0.05). The highest area under the curve values in saliva were found for the 50-TGF, 100-TGF, and PC groups. CONCLUSION: The dentifrice containing hydrocolloid as a controlled-release system for F promoted F retention in the oral cavity, even at 12 h after brushing. CLINICAL RELEVANCE: Hydrocolloid added to dentifrices as a controlled-release system for F might contribute to a higher anti-caries effect. TRIAL REGISTRATION: NCT02809014.

Disability in Activities of Daily Living and Severity of Dyskinesias Determine the Handicap of Parkinson's Disease Patients in Advanced Stage Selected to DBS.

BACKGROUND: There is scarce data on the level of handicap in Parkinson's disease (PD) and none in advanced stage PD. OBJECTIVE: To assess the handicap in advanced stage PD patients with disabling levodopa-induced motor complications selected to deep brain stimulation (DBS). METHODS: Data was prospectively recorded during routine evaluation for DBS. Handicap was measured using London Handicap Scale (LHS) (0 = maximal handicap; 1 = no handicap). Disease severity was evaluated using the Hoehn & Yahr scale and the UPDRS/MDS-UPDRS, during off and on after a supra-maximal dose of levodopa. Schwab and England Scale (S&E) was scored in off and on. Dyskinesias were scored using the modified Abnormal Involuntary Movement Scale (mAIMS). Results concern cross-sectional assessment before DBS. RESULTS: 100 PD patients (mean age 61 (±7.6); mean disease duration 12.20 (±4.6) years) were included. Median score of motor MDS-UPDRS was 54 in off and 25 in on. Mean total LHS score was 0.56 (±0.14). Patients were handicapped in several domains with a wide range of severity. Physical Independence and Social Integration were the most affected domains. Determinants of total LHS score were MDS-UPDRS part II off (ß= -0.271; p = 0.020), S&E on (ß= 0.264; p = 0.005) and off (ß= 0.226; p = 0.020), and mAIMS on (ß= -0.183; p = 0.042) scores (R2  = 29.6%). CONCLUSIONS: We were able to use handicap to measure overall health condition in advanced stage PD. Patients were moderately to highly handicapped and this was strongly determined by disability in ADL and dyskinesias. Change in handicap may be a good patient-centred outcome to assess efficiency of DBS.

Advanced Parkinson disease patients have impairment in prosody processing.

BACKGROUND: The ability to recognize and interpret emotions in others is a crucial prerequisite of adequate social behavior. Impairments in emotion processing have been reported from the early stages of Parkinson's disease (PD). This study aims to characterize emotion recognition in advanced Parkinson's disease (APD) candidates for deep-brain stimulation and to compare emotion recognition abilities in visual and auditory domains. METHOD: APD patients, defined as those with levodopa-induced motor complications (N = 42), and healthy controls (N = 43) matched by gender, age, and educational level, undertook the Comprehensive Affect Testing System (CATS), a battery that evaluates recognition of seven basic emotions (happiness, sadness, anger, fear, surprise, disgust, and neutral) on facial expressions and four emotions on prosody (happiness, sadness, anger, and fear). APD patients were assessed during the "ON" state. Group performance was compared with independent-samples t tests. RESULTS: Compared to controls, APD had significantly lower scores on the discrimination and naming of emotions in prosody, and visual discrimination of neutral faces, but no significant differences in visual emotional tasks. CONCLUSION: The contrasting performance in emotional processing between visual and auditory stimuli suggests that APD candidates for surgery have either a selective difficulty in recognizing emotions in prosody or a general defect in prosody processing. Studies investigating early-stage PD, and the effect of subcortical lesions in prosody processing, favor the latter interpretation. Further research is needed to understand these deficits in emotional prosody recognition and their possible contribution to later behavioral or neuropsychiatric manifestations of PD.

Cerebral Amyloid Angiopathy Associated with Inflammation: Report of 3 Cases and Systematic Review.

INTRODUCTION: Cerebral amyloid angiopathy associated with inflammatory process (CAA-I) is a rare potentially treatable encephalopathy, characterized by an inflammatory response to vascular deposits of ß-amyloid. We aimed to describe 3 clinical cases and perform a systematic review of all neuropathologically proved CAA-I case reports to describe its clinical and pathologic features and outcome under different treatments. METHODS: We searched PubMed and Cochrane Library and screened references of included studies and review articles for additional citations. Outcome was classified at the last available follow-up by the modified Rankin Scale (mRS). RESULTS: A total of 67 publications, reporting on 155 patients, were included. Mean age was 66.9 years, and 53.5% were men. The most common clinical presentation was cognitive dysfunction (48.0%) followed by headaches (38.7%), seizures (36.7%), and pyramidal signs (20.0%). Perivascular and vasculitic inflammation with granuloma was the most common pathologic pattern (27.5%). Eighty-six percent were treated with corticosteroids and 33.9% with cyclophosphamide. Forty-two percent regained independence (mRS score 0-2), whereas 20.5% were left with a severe handicap (mRS score 3-5) and 37.5% died. There were no statistically significant differences in outcome between patients treated with therapy with corticosteroids alone comparing with those treated with combination corticosteroids with cytostatic agents. CONCLUSIONS: The most common clinical manifestation of CAA-I was cognitive dysfunction. The functional outcome was unfavorable in the majority of the patients, with death or severe disability in almost two third of the cases, despite treatment. No differences in outcome could be detected between patients treated with corticosteroids versus patients treated with cytostatics, combined with corticosteroids.

Delayed leukoencephalopathy after acute carbon monoxide intoxication.

Delayed leukoencephalopathy is an uncommon complication of hypoxic-ischemic events of different etiologies, including carbon monoxide intoxication. We present a case of a 40-year-old male patient who was admitted with rapidly progressive neurocognitive and behavioral deficits. There was a history of accidental carbon monoxide intoxication one month before, presenting with loss of consciousness and short hospitalization, followed by a complete clinical recovery. The imaging studies in the delayed phase depicted confluent, symmetric supra-tentorial white matter lesions in keeping with diffuse demyelinization. Restricted diffusion and metabolite abnormalities in magnetic resonance proton spectroscopy were also seen. The diagnosis of CO-mediated delayed post-hypoxic leukoencephalopathy was assumed after exclusion of other mimickers. Hyperbaric oxygen therapy was tentatively performed and the patient had a favorable clinical and radiological evolution.

Biopsy-negative, varicella zoster virus (VZV)-positive giant cell arteritis, zoster, VZV encephalitis and ischemic optic neuropathy, all in one.

A 72-year-old man developed clinical features of giant cell arteritis (GCA) and ipsilateral ophthalmic-distribution zoster, followed within 2 weeks by VZV encephalitis and 2 months later by ischemic optic neuropathy. Temporal artery biopsy was histopathologically negative for GCA, but contained VZV antigen and VZV DNA in multiple non-contiguous (skip) areas. The collective clinical and laboratory findings revealed a remarkably close temporal association of zoster, multifocal VZV vasculopathy with temporal artery infection, biopsy-negative VZV-positive GCA and VZV encephalitis.