Management Strategy of Intracranial Complications of Sinusitis: Our Experience and Review of the Literature.

Objective: Sinusitis or rhinosinusitis is a very common disease worldwide, and in some cases, it leads to intracranial complications (ICS). These are more common in immunocompromised patients or with underlying comorbidities, but even healthy individuals, can be affected. Nowadays, ICS have become less common thanks to improved antibiotic therapies, radiological diagnostic methods, surgical techniques and skills. Nonetheless, they can still cause significant morbidity and mortality. For this reason, management of these complications requires a multidisciplinary approach to plan and customize treatment options. This paper presents our strategy in the management of a series of intracranial complications induced by acute sinusitis and compares our experience and outcomes with the literature. Study design: Single institute experience, retrospective analysis of cases series and literature review. Methods: Adult and child patients who were treated for ICS in the Department of Otorhinolaryngology at Sion Hospital, in Switzerland from 2016 to 2020 were included. Their symptoms, medical history, clinical and radiological findings, treatment, and outcome were documented. Results: Eight patients (6 males- 2 females) aged from 14 to 88 y.o., were enrolled. None had any previous history of chronic, or recurrent sinusitis. Moreover, very few presented specific rhinological symptoms, but with neurological or other symptoms. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) were used to confirm the diagnosis of all ICS. All types of known intracranial complications were observed in our cohort with a wide range of extension and severity of sinusitis. A multidisciplinary approach with individual treatments was tailored to each patient. Outcomes were favorable in almost all patients with neither recurrence, nor neurological sequels being observed in the follow-up. Only one patient was lost due to fatal complications of advanced lung cancer. Conclusion: ICS remain a challenging clinical problem due to substantial associated morbidity and mortality. The incidence of these complications is relatively low. Therapeutical management guidelines are lacking. Early detection and multidisciplinary approach are key to successful treatment.

Case report: Pallidal deep brain stimulation for treatment of tardive dystonia/dyskinesia secondary to chronic metoclopramide medication.

Objectives: Tardive dystonia/dyskinesia (TDD) occurs as a side effect of anti-dopaminergic drugs, including metoclopramide, and is often refractory to medication. While pallidal deep brain stimulation (DBS) has become an accepted treatment for TDD secondary to neuroleptic medication, there is much less knowledge about its effects on metoclopramide-induced TDD. Methods: We present the case of a woman with metoclopramide-induced TDD, whose symptoms were initially misjudged as "functional." After 8 years of ineffective medical treatments, she received bilateral implantation of quadripolar electrodes into the posteroventral lateral globus pallidus internus (GPi). Results: GPi DBS led to significant symptom reduction [Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor score 24/44 at admission and 7/44 at discharge]. Chronic stimulation led to full recovery from TDD symptoms 9 years after surgery. The BFMDRS motor score decreased to 0.5 (98% improvement). Discussion: Pallidal DBS may result in sustained improvement of TDD secondary to chronic metoclopramide intake in the long term.

Robot-induced hallucinations in Parkinson's disease depend on altered sensorimotor processing in fronto-temporal network.

Hallucinations in Parkinson's disease (PD) are disturbing and frequent non-motor symptoms and constitute a major risk factor for psychosis and dementia. We report a robotics-based approach applying conflicting sensorimotor stimulation, enabling the induction of presence hallucinations (PHs) and the characterization of a subgroup of patients with PD with enhanced sensitivity for conflicting sensorimotor stimulation and robot-induced PH. We next identify the fronto-temporal network of PH by combining MR-compatible robotics (and sensorimotor stimulation in healthy participants) and lesion network mapping (neurological patients without PD). This PH-network was selectively disrupted in an additional and independent cohort of patients with PD, predicted the presence of symptomatic PH, and associated with cognitive decline. These robotics-neuroimaging findings extend existing sensorimotor hallucination models to PD and reveal the pathological cortical sensorimotor processes of PH in PD, potentially indicating a more severe form of PD that has been associated with psychosis and cognitive decline.

How non-drug interventions affect the quality of life of patients suffering from progressive cognitive decline and their main caregiver.

BACKGROUND: In the absence of cure for age-related neurodegenerative diseases, non-drug interventions (NDIs) represent useful options. Quality of life (QOL) is a multidimensional concept progressively affected by cognitive decline. How single or multiple NDIs impact QOL is unknown. RESULTS: We found no significant effect of multiple over single NDI on QOL. Socio-demographic variables influenced patients' (age, gender, caregivers' occupational status, management of patients' financial affairs) and caregivers' (gender, occupational status, patients' severity of cognitive decline) QOL. When dyads interrupted interventions after 6 months, their QOL was lower and caregivers' anxiety, depression and physical symptoms were higher at the end of the study. CONCLUSIONS: While the type and number of interventions do not appear to be critical, the continuity of adapted interventions in the long-term might be important for maintaining QOL of patients and caregivers. METHODS: This is a multicenter (7 Swiss Memory Clinics), quasi-experimental, one-year follow-up study including 148 subjects (mild cognitive impairment or mild dementia patients and their caregivers). Primary outcome was the effect of multiple vs single NDIs on QOL. Secondary outcome included NDIs effect on patients' cognitive impairment and functional autonomy, caregivers' burden, severity of patients' neuropsychiatric symptoms and dyads' anxiety and depression.

[Brain function in complex regional pain syndrome†: implications for pain management]. / Syndrome douloureux régional complexe†: rôle du système nerveux central et implications pour la prise en charge.

The International Association for the Study of Pain (IASP) proposed the current diagnostic description of complex regional pain syndrome (CRPS) for the distinct and complex chronic pain condition in 1994. Since this classification, studies on the syndrome have led to a better understanding of the underlying pathophysiological mechanisms, epidemiology and therapeutic approaches. F. Luthi of SUVA Care reviewed CRPS in detail in 2014 and 2019 issues of the Revue médicale suisse. The purpose of this article is to provide an update of results on the neural mechanisms involved in this syndrome and how this helps management of CRPS, in particular bringing awareness to physicians of all specialties of the first symptoms with practical advice for investigations and treatment.

Le syndrome douloureux régional complexe (SDRC) a été défini en 1994 par l'International Association for the Study of Pain (IASP). Depuis cette définition, les études concernant ce syndrome ont permis une meilleure compréhension quant aux mécanismes physiopathologiques sous-jacents, à l'épidémiologie et aux approches thérapeutiques. Le SDRC a déjà été bien décrit dans deux numéros de la Revue médicale suisse de 2014 et 2019 par F. Luthi de la Clinique romande de réadaptation (SUVACare). Le but de cet article est de rapporter les connaissances récentes sur les mécanismes neuronaux impliqués dans ce syndrome et impactant la prise en charge. Nous souhaitons sensibiliser les médecins de toutes spécialités à la reconnaissance des premiers symptômes et diffuser des conseils pratiques quant aux investigations et aux traitements.

Peripheral neuropathy and cognitive impairment associated with a novel monoallelic HARS variant.

BACKGROUND: A 49-year-old male presented with late-onset demyelinating peripheral neuropathy, cerebellar atrophy, and cognitive deficit. Nerve biopsy revealed intra-axonal inclusions suggestive of polyglucosan bodies, raising the suspicion of adult polyglucosan bodies disease (OMIM 263570). METHODS AND RESULTS: While known genes associated with polyglucosan bodies storage were negative, whole-exome sequencing identified an unreported monoallelic variant, c.397G>T (p.Val133Phe), in the histidyl-tRNA synthetase (HARS) gene. While we did not identify mutations in genes known to be associated with polygucosan body disease, whole-exome sequencing revealed an unreported monoallelic variant, c.397G>T in the histidyl-tRNA synthetase (HARS) gene, encoding a substitution (Val133Phe) in the catalytic domain. Expression of this variant in patient cells resulted in reduced aminoacylation activity in extracts obtained from dermal fibroblasts, without compromising overall protein synthesis. INTERPRETATION: Genetic variants in the genes coding for the different aminoacyl-tRNA synthases are associated with various clinical conditions. To date, a number of HARS variant have been associated with peripheral neuropathy, but not cognitive deficits. Further studies are needed to explore why HARS mutations confer a neuronal-specific phenotype.

[Capgras delusion and Alzheimer disease]. / Syndrome de Capgras et maladie d'Alzheimer.

Capgras delusion is classified with the misidentification syndromes. In dementia it associates cognitive deficiency of memory and facial recognition (prosopoagnosia) with delirious idea of substitution by a double. The first reported case in the paper describes the important affective and comportmental reactions due to the identification of a double perceived as an imposter, affecting both the suffering person and his family. Rarely, as reported in the second case, misrecognition concerns the person itself (autoprosopagnosia) who can have the illusion to be in front of a twin brother (« auto-Capgras ¼). We discuss data from the literature concerning prevalence, results of cerebral imaging and functional prognosis associated with this curious syndrome.

Le syndrome de Capgras fait partie des troubles de l'identification des personnes. Dans la démence, il associe des déficits cognitifs de la mémoire et de la reconnaissance des visages (prosopagnosie) et des idées délirantes de substitution par des sosies. La première situation rapportée dans l'article décrit les importantes réactions affectives et comportementales engendrées par l'identification d'un sosie qui est perçu comme un imposteur, affectant à la fois le malade et son entourage. Rarement, comme rapporté dans la deuxième situation, la fausse reconnaissance concerne la personne elle-même (autoprosopagnosie) qui peut avoir l'illusion d'avoir en face d'elle un frère jumeau (« auto-Capgras ¼). Nous discutons de données issues de la littérature concernant la prévalence, les résultats de l'imagerie cérébrale ainsi que le pronostic fonctionnel liés à ce curieux syndrome.

[Abnormal movements and internal medicine pathologies]. / Mouvements anormauxet médecine interne.

Hyperkinesias can be revelaed by an internal medicine pathology. An acute chorea can be found in association with non ketotic hypergycemia, lupus, antiphospholipid syndrome, endocrinopathies, pregnancy or oral contraceptive initiation, or psychostimulant medication. Acute dystonic syndromes are found in association with the initiation of an old generation neuroleptic, metoclopramide, oral contraceptive and pregnancy. It should be differentiated from hyper- or hypo-calcémie tetany. Tremors are found in association with many drugs and hormones. Akathisisc syndromes, classically found in association with chronic neuroleptic use, include restless legs, found in association with iron deficiency anemia, pregnancy, many drugs and polyneuropathies, as well as in the withdrawal syndrome of benzodiazepines and opiates.

Des hypercinésies (ou dyskinésies) peuvent évoquer une maladie de médecine interne. Une chorée aiguë peut être causée par une hyperglycémie non cétosique, un lupus, un syndrome antiphospholipide, une endocrinopathie, une grossesse, l'initiation d'un contraceptif, une polyglobulie, ou la prise de psychostimulants. Les syndromes dystoniques aigus se rencontrent avec l'initiation d'un neuroleptique, de métoclopramide, d'un contraceptif ou d'une grossesse et doivent se distinguer de la tétanie hyper- ou hypo-calcémique. Les tremblements orientent vers les médicaments ou les hormones. Les myoclonies se voient classiquement dans les encéphalopathies métaboliques (hépatiques, rénales) ou médicamenteuses. Les syndromes akathisiques, associés à la prise chronique de neuroleptiques, incluent le syndrome des jambes sans repos, lié aux anémies ferriprives, à la grossesse, à certains médicaments, aux neuropathies et au sevrage des benzodiazépines ou d'opiacés.

Combined thalamic and subthalamic deep brain stimulation for tremor-dominant Parkinson's disease.

Deep brain stimulation (DBS) in the thalamic ventral intermediate (Vim) or the subthalamic nucleus (STN) reportedly improves medication-refractory Parkinson's disease (PD) tremor. However, little is known about the potential synergic effects of combined Vim and STN DBS. We describe a 79-year-old man with medication-refractory tremor-dominant PD. Bilateral Vim DBS electrode implantation produced insufficient improvement. Therefore, the patient underwent additional unilateral left-sided STN DBS. Whereas Vim or STN stimulation alone led to partial improvement, persisting tremor resolution occurred after simultaneous stimulation. The combination of both targets may have a synergic effect and is an alternative option in suitable cases.

Definition and management of varicella zoster virus-associated meningoradiculitis: a case report.

BACKGROUND: The varicella zoster virus affects the central or peripheral nervous systems upon reactivation, especially when cell-mediated immunity is impaired. Among varicella zoster virus-related neurological syndromes, meningoradiculitis is an ill-defined condition for which clear management guidelines are still lacking. Zoster paresis is usually considered to be a varicella zoster virus-peripheral nervous system complication and treated with oral antiviral therapy. Yet in the literature, the few reported cases of herpes zoster with mild cerebral spinal fluid inflammation were all considered meningoradiculitis and treated using intravenous antiviral drugs, despite absence of systemic signs of meningitis. Nevertheless, these two clinical pictures are very similar. CASE PRESENTATION: We report the case of an alcohol-dependent elderly Caucasian man presenting with left lower limb zoster paresis and mild cerebral spinal fluid inflammation, with favorable outcome upon IV antiviral treatment. We discuss interpretation of liquor inflammation in the absence of clinical meningitis and implications for the antiviral treatment route. CONCLUSION: From this case report we suggest that varicella zoster virus-associated meningoradiculitis should necessarily include meningitis symptoms with the peripheral neurological deficits and cerebral spinal fluid inflammation, requiring intravenous antiviral treatment. In the absence of (cell-mediated) immunosuppression, isolated zoster paresis does not necessitate spinal tap or intravenous antiviral therapy.

[Dementia with motor and language disorders]. / Démences avec troubles moteurs et du langage.

Memory is not the only core diagnostic criteria in Alzheimer's disease and many dementias are characterized by other cognitive deficits. Moreover dementias are often associated with multiple and complex motor signs. The first part of this reviewcovers parkinsonism in diffuse Lewy Body Disease and other neurodegenerative diseases, corticobasal syndrome, or motor deficit in the motoneurone disease-frontotemporal dementia spectrum. In the second part, primary progressive aphasia and its three variants including basic clinical evaluation are described. These complex clinical syndromes involving motor and language systems are important for the clinical practice since they are part of diagnostic criteria of several neurodegenerative diseases and can be considered as phenotypical markers of neurodegeneration.

Visual signs and symptoms in patients with the visual variant of Alzheimer disease.

BACKGROUND: Prominent visual symptoms can present in the visual variant of Alzheimer's disease (VVAD). Ophthalmologists have a significant role to play in the early diagnosis of VVAD. METHODS: We retrospectively reviewed the files of ten consecutive patients diagnosed with VVAD. All patients had a full neuro-ophthalmologic examination, a formal neurological and neuro-psychological testing, and cerebral MRI to confirm diagnosis. In addition, functional neuroimaging was obtained in seven patients. RESULTS: The common primary symptom at presentation with all patients was difficulty with near vision (reading difficulty n = 8, "visual blur" in near vision n = 2), and difficulty writing (n = 3). Following assessment, impaired reading and writing skills were evident in 9/10 and 8/10 patients respectively. Median distance visual acuity was 20/25 and at near the median visual acuity was J6. Partial homonymous visual field defect was detected in 80 % (8/10) of the patients. Color vision was impaired in all patients when tested with Ishihara pseudoisochromatic plates, but simple color naming was normal in 8/9 tested patients. Simultanagnosia was present in 8/10 patients. Vision dysfunction corresponded with cerebral MRI findings where parieto-occipital cortical atrophy was observed in all patients. PET scan (5 patients) or SPECT (2 patients) revealed parieto-occipital dysfunction (hypometabolism or hypoperfusion) in all 7 tested patients CONCLUSIONS: Visual difficulties are prominent in VVAD. Dyslexia, incomplete homonymous hemianopia, preserved color identification with abnormal color vision on Ishihara, and simultanagnosia were all symptoms observed frequently in this patient series. Ophthalmologists should be aware of the possibility of neurodegenerative disorders such as VVAD in patients with unexplained visual complaints, in particular reading difficulties.

Demyelination in mild cognitive impairment suggests progression path to Alzheimer's disease.

The preclinical Alzheimer's disease (AD) - amnestic mild cognitive impairment (MCI) - is manifested by phenotypes classified into exclusively memory (single-domain) MCI (sMCI) and multiple-domain MCI (mMCI). We suggest that typical MCI-to-AD progression occurs through the sMCI-to-mMCI sequence as a result of the extension of initial pathological processes. To support this hypothesis, we assess myelin content with a Magnetization Transfer Ratio (MTR) in 21 sMCI and 21 mMCI patients and in 42 age-, sex-, and education-matched controls. A conjunction analysis revealed MTR reduction shared by sMCI and mMCI groups in the medial temporal lobe and posterior structures including white matter (WM: splenium, posterior corona radiata) and gray matter (GM: hippocampus; parahippocampal and lingual gyri). A disjunction analysis showed the spread of demyelination to prefrontal WM and insula GM in executive mMCI. Our findings suggest that demyelination starts in the structures affected by neurofibrillary pathology; its presence correlates with the clinical picture and indicates the method of MCI-to-AD progression. In vivo staging of preclinical AD can be developed in terms of WM/GM demyelination.

Evolution of source EEG synchronization in early Alzheimer's disease.

Alzheimer's disease (AD) disrupts functional connectivity in distributed cortical networks. We analyzed changes in the S-estimator, a measure of multivariate intraregional synchronization, in electroencephalogram (EEG) source space in 15 mild AD patients versus 15 age-matched controls to evaluate its potential as a marker of AD progression. All participants underwent 2 clinical evaluations and 2 EEG recording sessions on diagnosis and after a year. The main effect of AD was hyposynchronization in the medial temporal and frontal regions and relative hypersynchronization in posterior cingulate, precuneus, cuneus, and parietotemporal cortices. However, the S-estimator did not change over time in either group. This result motivated an analysis of rapidly progressing AD versus slow-progressing patients. Rapidly progressing AD patients showed a significant reduction in synchronization with time, manifest in left frontotemporal cortex. Thus, the evolution of source EEG synchronization over time is correlated with the rate of disease progression and should be considered as a cost-effective AD biomarker.

[Mirror behaviors in dementia: the many mirror signs]. / Conduites spéculaires dans les démences: les multiples signes du miroir.

Mirror behaviors in advanced dementia are: the mirror sign of Abely and Delmas, where the patient stares at his face (environment-driven behavior of Lhermitte); non recognition of the self in the mirror (autoprosopagnosia and/or delirious auto-Capgras); mirror agnosia of Ramachandran and Binkofski where the patient do not understand the concept of mirror and its use; the psychovisual reflex, or reflex pursuit of the eyes when passively moving a minrror in front of a patient (intact vision); mirror writing (procedural learning). We describe four demented patients with mirror behaviors assessing brain mechanisms of self recognition, social brain and mental and visuo-spatial manipulation of images and objects.

[Acute amnestic syndrome: left thalamo-polar infarct]. / Syndrome amnésique aigu: AVC thalamo-polaire gauche.

An 80-year old American patient was found wandering in a mountain village of Switzerland, with an anterograde, prospective, retrograde, dyschronologic amnesic syndrome without confabulation, paramnesia or false recognitions, disoriented, slightly confused, with no focal sensory, motor, ataxic or visual field deficit, with a mild dysexecutive syndrome. The MR imaging showed an acute thalamo-polar artery infarct. A dysconnection of the mamillo-othalamic and thalamo-temporal pathways is felt at the origin of the amnesic syndrome. A brief review of the other presentation of this chamelon syndrome is presented, together the main etiologies at its origin.