Background
The aim of the study was to correlate cognitive decline and cerebral vasoreactivity in 150 asymptomatic right‐handed patients with severe ≥70 % unilateral internal carotid artery (ICA) ...stenosis and to evaluate the role of intracranial collateral circulation during cognitive testing.
Methods
Cognitive assessment was performed by means of Montreal Cognitive Assessment (MoCA) and Mini Mental State Exam (MMSE) scales. Cerebrovascular reactivity (CVR) and intracranial collateral circulation were evaluated by means of breath holding index (BHI) and transcranial color Doppler (TCD) sonography.
Results
The results were compared with 150 right‐handed controls matched for demographic variables and vascular risk factors. Patients with severe unilateral ICA stenosis had MMSE scores within a normal range, but MoCA scores were lower than normal. By examining the side of the observed stenosis, it has been noted that patients with left‐sided ICA stenosis had lower MoCA scores in categories of language and episodic memory performance, while patients with right‐sided ICA stenosis had lower MoCA scores in a category of visual–spatial skills. All patients had BHI values lower than normal. Subjects with a single intracranial collateral artery recruited had slightly better cognitive results than the patients with two or more collateral arteries activated.
Conclusions
Results of the study showed that altered cerebrovascular reactivity and cerebral hypoperfusion might be responsible for the reduction of specific cognitive functions ipsilateral to the ICA stenosis, therefore BHI and MoCA might be useful tools when screening for cognitive decline in asymptomatic patients with severe ICA stenosis.
Abstract
Background
The aim of the study was to correlate cognitive decline and cerebral vasoreactivity in 150 asymptomatic right‐handed patients with severe ≥70 % unilateral internal carotid artery ...(ICA) stenosis and to evaluate the role of intracranial collateral circulation during cognitive testing.
Methods
Cognitive assessment was performed by means of Montreal Cognitive Assessment (MoCA) and Mini Mental State Exam (MMSE) scales. Cerebrovascular reactivity (CVR) and intracranial collateral circulation were evaluated by means of breath holding index (BHI) and transcranial color Doppler (TCD) sonography.
Results
The results were compared with 150 right‐handed controls matched for demographic variables and vascular risk factors. Patients with severe unilateral ICA stenosis had MMSE scores within a normal range, but MoCA scores were lower than normal. By examining the side of the observed stenosis, it has been noted that patients with left‐sided ICA stenosis had lower MoCA scores in categories of language and episodic memory performance, while patients with right‐sided ICA stenosis had lower MoCA scores in a category of visual–spatial skills. All patients had BHI values lower than normal. Subjects with a single intracranial collateral artery recruited had slightly better cognitive results than the patients with two or more collateral arteries activated.
Conclusions
Results of the study showed that altered cerebrovascular reactivity and cerebral hypoperfusion might be responsible for the reduction of specific cognitive functions ipsilateral to the ICA stenosis, therefore BHI and MoCA might be useful tools when screening for cognitive decline in asymptomatic patients with severe ICA stenosis.
- Fabry disease is a rare X-linked inherited lysosomal storage disease affecting multiple organ systems, presenting in the central nervous system (CNS) as white matter lesions with underlying ...cerebral vasculopathy and autoinflammatory changes of the choroid plexus and leptomeninges. We present a young female patient (age 36 years) admitted to our department due to visual loss on the left eye. Magnetic resonance imaging (MRI) showed demyelinating lesions in the frontal and parietal lobe, periventricularly, in mesencephalon and right cerebellar hemisphere, and left optic neuritis; MR angiography was normal. Her medical history revealed renal dysfunction, hypothyroidism, and miscarriage in the 6
month of pregnancy due to eclampsia and Fabry disease in the family (mother). Cerebrospinal fluid analysis showed mild pleocytosis, normal blood brain barrier function and oligoclonal bands type 3. Visual evoked potentials showed prechiasmal dysfunction of the left optic nerve. Genetical testing for Fabry disease was positive (two heterozygous mutations), with decreased alpha galactosidase activity values and increased Lyso GB3 values. The patient received corticosteroid therapy (methylprednisolone) 1 g for 5 days, which led to regression of visual disturbances on the left eye. After this acute treatment, there was a question of definitive diagnosis and further treatment of the underlying cause. Considering renal dysfunction, miscarriage, arterial hypertension, positive genetic and biochemical testing for Fabry disease, as well as MRI findings showing lesions in posterior circulation, we concluded that the patient probably had Fabry disease with autoinflammatory changes in the CNS and should be treated with enzyme replacement therapy. Still, there was a question of optic neuritis on the left eye and positive oligoclonal bands favoring the diagnosis of multiple sclerosis. Therefore, further clinical and neuroradiological follow up was needed to distinguish multiple sclerosis and Fabry disease in this patient.
Stroke is the second leading cause of death and the most important cause of adult disability worldwide and in Croatia. In the past, stroke was almost exclusively considered to be a disease of the ...elderly; however, today the age limit has considerably lowered towards younger age. The aim of this study was to determine age and gender impact on stroke patients in a Croatian urban area during one-year survey. The study included all acute stroke patients admitted to our Department in 2004. A compiled stroke questionnaire was fulfilled during hospitalization by medical personnel on the following items: stroke risk factors including lifestyle habits (smoking and alcohol), pre-stroke physical ability evaluation, stroke evolution data, laboratory and computed tomography findings, outcome data and post-stroke disability assessment. Appropriate statistical analysis of numerical and categorical data was performed at the level of p < 0.05. Analysis was performed on 396 patients, 24 of them from the younger adult stroke group. Older stroke patients had worse disability at hospital discharge and women had worse disabilities at both stroke onset and hospital discharge, probably due to older age at stroke onset. Younger patients recovered better, while older patients had to seek secondary medical facilities more often, as expected. The most important in-hospital laboratory findings in young stroke patients were elevated lipid levels, while older patients had elevated serum glucose and C-reactive protein. Stroke onset in younger patients most often presented with sudden onset headache; additionally, onset seizure was observed more frequently than expected. Stroke risk factor analysis showed that women were more prone to hypertension, chronic heart failure and atrial fibrillation, whereas men had carotid disease more frequently, were more often smokers and had higher alcohol intake. Additionally, age analysis showed that heart conditions and smoking were more prevalent among older stroke patients. In conclusion, considerable differences were established between age and gender stroke patient groups, confirming the need of permanent national stroke registry and subsequent targeted action in secondary care, and prevention with education on risk factors, preferably personally tailored.
This case report presents a patient diagnosed with Tolosa-Hunt syndrome (THS) after an extensive neuro-diagnostic and neuroimaging evaluation. Diagnostic work-up included thorough physical and ...neurological examination, complete laboratory serum assessments, neuroendocrine and immunohistochemistry analysis, cerebrospinal fluid analysis, neurophysiology assessment, ophthalmologist examination and neuroimaging. Th e most important diagnostic tool in deriving the diagnosis of THS was neuroimaging evaluation that included baseline and follow-up cranial contrast enhanced magnetic resonance imaging (MRI). Baseline cranial contrast enhanced MRI detected a nonspecific inflammatory granulomatous lesion in the right cavernous sinus extending basally towards the right trigeminal cave (Meckel's cave) and anteriorly towards the apex of the right orbit. Systemic intravenous high-dose corticosteroid therapy was administered for 3 consecutive days and then tapered down to lower oral steroid doses. Following therapy, the patient experienced complete regression of symptoms. Follow-up cranial contrast enhanced MRI showed significant regression of inflammatory lesion in the area of right cavernous sinus, thus verifying the efficacy of the treatment applied. This paper shows that an extensive diagnostic schedule for THS must be conducted prior to therapeutic treatment, for the possibility of alternative diagnosis. Patients suspected of having THS require careful evaluation, appropriate treatment, and follow-up.
The first hospital-based registry of patients with multiple sclerosis (MS) was established at the University Department of Neurology, Sestre milosrdnice University Hospital Centre, Zagreb, Croatia, ...in 2014. The aim of the registry was to continuously provide data on the number of hospital-managed MS patients, patterns of disease progression, predictors of disability progression, changes in lifespan and long-term outcomes. Relevant medical data included age and gender of MS patients, family history of MS, data on previous immunization, disease course, Expanded Disability Status Scale (EDSS) score, cerebral magnetic resonance imaging (MRI) lesion load quantification, and cerebrospinal fluid analysis. Lifestyle habits in MS patients including smoking and alcohol consumption were also analyzed. All data were obtained from primary medical records between January 1, 2014 and January 1, 2015, and entered into the database. Data were evaluated retrospectively according to age and gender differences. Results showed that the majority of patients enrolled in the registry had the remitting relapsing course of disease, with low EDSS score indicating no disability or minimal disability. Cerebrospinal fluid analysis showed that oligoclonal bands were present in the majority of MS patients, with affected blood-brain-barrier permeability. According to the remitting relapsing course of the disease, cerebral MRI quantitative analysis demonstrated a significant lesion load in the majority of patients. When stratified by lifestyle habits, smokers and alcohol consumers were more prevalent among male patients. Our hospital-based registry might be considered as a prototype for the national MS registry and should be improved for reliable statistical analysis.
Autoimmune Encephalitis Lisak, Marijana; Špiljak, Bruno; Pašić, Hanna
Archives of psychiatry research,
02/2023, Letnik:
59, Številka:
1
Journal Article
Recenzirano
Odprti dostop
The purpose of this paper is to provide a comprehensive review of recent literature data for autoimmune encephalitis (AIE). AIE refers to inflammatory, non-infectious, immune-mediated encephalitis ...characterised by neuroinflammation, synthesis of neuronal autoantibodies (NAAs), directed against surface, synaptic and intracellular antigens, with subsequent neuronal dysfunction. It is characterised by heterogeneous anatomic-clinical syndromes and prominent neuropsychiatric symptoms. Due to overlapping of different clinical and diagnostic biomarkers, AIE is often considered diagnosis of exclusion and requires an extensive work-up. Systematic search of the term «autoimmune encephalitis» in the PubMed database was performed, with limitation set for systematic review in papers English, published from 2004- 2022. Further analysis was performed by the search of the author’s reference list and Autoimmune Encephalitis Alliance (AEA) website. The analysis was conducted according to PRISMA (The Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Outcomes that were sought included: AIE classification and presentation; diagnostic processing; treatment. Preset search of published systematic reviews in PubMed database, derived eighty six papers. Further screening of derived data, author’s reference list and AEA website was performed, according to previously defined out- comes. Finally, sixteen papers were independently selected and thoroughly analysed, with relevant conclusions present- ed in this paper. AIE is a severe inflammatory central nervous system (CNS) disorder with a complex differential diagnosis that often remains unrecognised. AIE research has established a wide range of new autoimmune antibodies syndromes, clinical and diagnostic biomarkers, which have improved diagnostic approach and treatment. Initial application of immunotherapy improves the outcome of disease.
Patofi ziologija multiple skleroze tradicionalno uključuje imunološki posredovanu upalnu reakciju središnjeg živčanog sustava, tzv. neuroinfl amaciju, koja je posredovana CD4+ pomoćnim T stanicama ...tipa 1. Dostupna imunomodulacijska terapija koja je razvijena na temelju ove teorije smanjuje stopu relapsa za otprilike jednu trećinu, ali ne može u potpunosti spriječiti ni relapse ni akumulaciju invaliditeta, te uglavnom nije učinkovita u primarno progresivnom obliku multiple skleroze. B stanice imaju temeljnu ulogu u patogenezi autoimunih neuroloških poremećaja i važni su regulatori procesa aktivacije T stanica, proizvodnje citokina i stvaranja ektopičnih germinalnih centara u središnjem živčanom sustavu. Smatra se da su memorijske B stanice, koje prelaze krvno-moždanu barijeru, podložne ponovljenoj stimulaciji, klonalnoj ekspanziji i diferencijaciji u plazma stanice koje izlučuju protutijela. Posljedično, središnji živčani sustav bolesnika nije samo ciljna meta imunopatološkog procesa, već postaje i mjesto lokalne proizvodnje protutijela. B stanice mogu ili povećati ili ublažiti neuroinfl amaciju, ali u većini bolesnika prevladava proupalni učinak stanica. Deplecija B stanica pomoću humaniziranih monoklonskih antitijela koja prepoznaju specifi čne ciljne antigene, npr. CD20, CD25 ili CD52, je obećavajući terapijski koncept u bolesnika s multiplom sklerozom. Primjena monoklonskih protutijela smatra se gotovo revolucionarnom terapijom i potentna je mogućnost liječenja multiple skleroze, sa snažnim učinkom na progresiju bolesti i dokazanom učinkovitosti u primarno progresivnom obliku bolesti koji se liječi na nezadovoljavajući način.
Cognitive dysfunction appears all through the course of multiple sclerosis (MS). Mild and moderate cognitive impairment is present in up to 40% of MS patients and severe cognitive decline affects ...more than 50% of patients in progressive course of the disease. The most common cognitive disorders in MS include diminished information processing speed, compromised word fluency, complex attention deficit and executive dysfunction.
In this mini review, we present the reader with the most common neuropsychological assessments for the evaluation of cognition in MS, addressing the question of cognitive relapse. Source of data presented in this review is PubMed search of the recently published literature on cognitive decline in MS.
Patients with cognitive relapse often fail to meet diagnostic criteria for classical relapse in MS. Although, cognitive decline relates poorly to functional disability in MS, it correlates well with neuropsychological testing and with neuroimaging parameters of the disease.
Cognitive decline might be considered as additional indicator of MS activity, and therefore evaluated routinely, irrespective of clinical presentation. Brief cognitive assessment, with confirmed psychometric qualities, might be useful in detection of cognitive relapse in MS patient.
Parkinsonova bolest (PB) općenito se smatra primarnim poremećajem pokreta, ali većina bolesnika pati i od ne-motornih
oralnih salivarnih simptoma. Najčešći salivarni simptomi, sijaloreja i ...kserostomija, imaju značajan negativan utjecaj na
kvalitetu života bolesnika. Iako su navedeni simptomi potpuno suprotni, oboje značajno narušavaju oralno zdravlje bolesnika.
Sijaloreja se definira kao povećana količina zadržavajuće sline, a povezana je s prekomjernim lučenjem sline ili nedostatnim
odstranjivanjem sline. Za razliku od sijaloreje, kserostomija se subjektivno definira kao suhoća usta i povezana je s nedovoljnim
lučenjem sline. Kserostomija potiče neravnotežu oralne mikroflore i oralnu patologiju koja često dovodi do pothranjenosti
kod bolesnika s PB. Povezana je s autonomnom disfunkcijom ili se može smatrati nuspojavom dopaminergičkih ili
antikolinergičkih lijekova. U PB se za procjenu sijaloreje i kserostomije primjenjuju različite metode procjene, uključujući
standardizirane ljestvice za procjenu ne-motoričkih simptoma i upitnike oralnog zdravlja. Slijedom toga, liječenje salivarnih
simptoma uključuje farmakološki i ne-farmakološki pristup te kirurške intervencije. Multidisciplinarni pristup u kliničkoj
neurologiji i dentalnoj medicini, koji obuhvaća preciznu procjenu salivarnih simptoma te učinkovito liječenje, ukazuje na
uspješno liječenje bolesnika s PB.
PDF
