Primary angiitis of the central nervous system in a patient with transient episodes of headache and aphasia: A case report.

Primary angiitis of the central nervous system (PACNS) refers to a rare form of vasculitis of unknown cause, with a challenging diagnostic work-up. We report the case of a 57-year-old patient who presented with transient episodes of headache and global aphasia. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis with moderate elevated protein and normal glucose. CSF and serum tests for infections and autoimmune/paraneoplastic antibodies were negative, except CSF polymerase chain reaction testing that detected Epstein-Barr virus (EBV). Magnetic resonance imaging of the brain with intravenous gadolinium showed meningeal enhancement and pachymeningitis. Due to continuous relapsing episodes of aphasia, a leptomeningeal and brain tissue biopsy was performed and revealed lesions of granulomatous necrotising vasculitis of medium-sized leptomeningeal and intracranial vessels, as well as negative in situ hybridism for EBV. A diagnosis of primary granulomatous necrotising angiitis of the central nervous system was made, and the patient was treated with intravenous methylprednisolone and oral cyclophosphamide, showing excellent response to treatment. Diversity in clinical and laboratory features makes it difficult for PACNS to be distinguished by other systemic vasculitides. Laboratory tests and neuroimaging can provide guidance in evaluation of the patients and exclude other possible causes, but tissue biopsy remains the gold standard for a definite diagnosis.

Predictors of outcomes after surgery for medically intractable insular epilepsy: A systematic review and individual participant data meta-analysis.

Insular epilepsy (IE) is an increasingly recognized cause of drug-resistant epilepsy amenable to surgery. However, concerns of suboptimal seizure control and permanent neurological morbidity hamper widespread adoption of surgery for IE. We performed a systematic review and individual participant data meta-analysis to determine the efficacy and safety profile of surgery for IE and identify predictors of outcomes. Of 2483 unique citations, 24 retrospective studies reporting on 312 participants were eligible for inclusion. The median follow-up duration was 2.58 years (range, 0-17 years), and 206 (66.7%) patients were seizure-free at last follow-up. Younger age at surgery (≤18 years; HR = 1.70, 95% CI = 1.09-2.66, P = .022) and invasive EEG monitoring (HR = 1.97, 95% CI = 1.04-3.74, P = .039) were significantly associated with shorter time to seizure recurrence. Performing MR-guided laser ablation or radiofrequency ablation instead of open resection (OR = 2.05, 95% CI = 1.08-3.89, P = .028) was independently associated with suboptimal or poor seizure outcome (Engel II-IV) at last follow-up. Postoperative neurological complications occurred in 42.5% of patients, most commonly motor deficits (29.9%). Permanent neurological complications occurred in 7.8% of surgeries, including 5% and 1.4% rate of permanent motor deficits and dysphasia, respectively. Resection of the frontal operculum was independently associated with greater odds of motor deficits (OR = 2.75, 95% CI = 1.46-5.15, P = .002). Dominant-hemisphere resections were independently associated with dysphasia (OR = 13.09, 95% CI = 2.22-77.14, P = .005) albeit none of the observed language deficits were permanent. Surgery for IE is associated with a good efficacy/safety profile. Most patients experience seizure freedom, and neurological deficits are predominantly transient. Pediatric patients and those requiring invasive monitoring or undergoing stereotactic ablation procedures experience lower rates of seizure freedom. Transgression of the frontal operculum should be avoided if it is not deemed part of the epileptogenic zone. Well-selected candidates undergoing dominant-hemisphere resection are more likely to exhibit transient language deficits; however, the risk of permanent deficit is very low.

Case Report: Possible Serotonin Syndrome in a Patient Taking Kratom and Multiple Serotonergic Agents.

INTRODUCTION: Kratom, an unregulated herbal supplement, has emerged as self-treatment for anxiety/depression. Kratom exhibits inhibition at multiple cytochrome P450 isozymes involved in metabolism of prescription medications, including serotonergic agents. We report a case of possible serotonin syndrome induced by kratom use in combination with prescription psychotropic medications. CASE: A 63-year-old male presented with diaphoresis, flushing, aphasia, confusion, dysarthria, right facial droop, and oral temperature of 39.6oC (103.2oF), lactate 2.7 mmol/L, and creatine phosphokinase of 1507 IU/L. Initial differential diagnoses included acute ischemic stroke and bacterial meningitis. Despite partial treatment with alteplase and broad-spectrum antibiotics, symptoms persisted, and subsequent physical exam noted hyperreflexia, clonus, tremors, and temperature of 41.1oC (106oF). Home medications included a chronic regimen for anxiety/depression with bupropion, buspirone, desvenlafaxine, trazodone, and ziprasidone, in addition to kratom. Clinical suspicion for serotonin syndrome led to initiation of cyproheptadine, lorazepam, and cooling blankets. Aphasia, facial droop, and confusion improved after administration of cyproheptadine. Bupropion was restarted during hospitalization; remaining medications restarted at the discretion of the primary care provider. DISCUSSION: Risk of serotonin syndrome with multiple serotonergic agents is well-known. Kratom is metabolized by cytochrome P40 isozymes 3A4, 2C9, and 2D6, and exhibits inhibition at those enzymes, in addition to 1A2. Pharmacokinetic interactions of kratom with prescription serotonergic agents metabolized through these isozymes has the potential to increase systemic exposure of serotonin, potentially leading to serotonin syndrome. CONCLUSION: Because substances contained in kratom can inhibit metabolism of prescription serotonergic medications, clinicians must be aware of potential development of serotonin syndrome.

Head Acupuncture Plus Schuell's Language Rehabilitation for Post-Stroke Aphasia: A Systematic Review and Meta-Analysis of 32 Randomized Controlled Trials.

OBJECTIVE: To evaluate the existing randomized controlled trials (RCTs) for evidence of the efficacy and safety of head acupuncture (HA) plus Schuell's language rehabilitation (SLR) in post-stroke aphasia. METHODS: Seven databases including Embase, PubMed, Cochrane Library, Technology Periodical Database, the China National Knowledge Infrastructure, SinoMed and Wanfang Data Information Site were searched for RCTs published from database inception until November 14, 2021. RCTs that compared HA plus SLR with sham (or blank) control, acupuncture therapy alone, certain language rehabilitation therapy alone or other therapies for post-stroke aphasia were included. Data were extracted and assessed, and the quality of RCTs was evaluated. Fixed-effects model was used, with meta-inflfluence analysis, meta-regression, and regression-based sub-group analyses applied for exploration of heterogeneity. Publication bias was estimated by funnel plots and Egger's tests. RESULTS: A total of 32 RCTs with 1,968 patients were included and 51 comparisons were conducted classified as types of strokes and aphasia. (1) For patients with aphasia after ischemic stroke, HA plus PSA showed significantly higher accumulative markedly effective rate [relative risk (RR)=1.55, 95% confidence interval (CI): 1.19-2.02, I2=0%] and accumulative effective rate (RR=1.22, 95% CI: 1.09-1.36, I2=0%). (2) For patients with comprehensive types of stroke, HA plus PSA was more effective in increasing recovery rate (RR=1.89, 95% CI: 1.39-2.56, I2=0%), accumulative markedly effective rate (RR=1.53, 95% CI: 1.36-1.72, I2=9%) and accumulative effective rate (RR=1.14, 95% CI: 1.09-1.19, I2=34%). (3) For patients with aphasia after stroke, HA plus PSA was superior to PSA alone with statistical significance in increasing recovery rate (RR=2.08, 95% CI: 1.24-3.46, I2=0%), accumulative markedly effective rate (RR=1.49, 95% CI: 1.24-1.78, I2=0%) and accumulative effective rate (RR=1.15, 95% CI: 1.06-1.24, I2=39%). (4) For patients with multiple types of aphasia, HA plus PSA also demonstrated significantly higher recovery rate (RR=1.86, 95% CI: 1.28-2.72, I2=0%), accumulative markedly effective rate (RR=1.55, 95% CI: 1.35-1.78, I2=22%), and accumulative effective rate (RR=1.17, 95% CI: 1.11-1.23, I2=41%). (5) For patients with motor aphasia after ischemic stroke, compared with PSA alone, HA plus PSA showed significantly higher accumulative markedly effective rate (RR=1.38, 95% CI: 1.06-1.79, I2=0%) and accumulative effective rate (RR=1.20, 95% CI: 1.05-1.37, I2=0%). Meta-regression analyses were performed without significant difference, and publication bias was found in some comparisons. CONCLUSION: HA plus SLR was significantly associated with better language ability and higher effective rate for patients with post-stroke aphasia, and HA should be operated cautiously especially during acupuncture at eye and neck. (Registration No. CRD42020154475).

Clinical Reasoning: A 55-Year-Old Woman With Recurrent Episodes of Aphasia and Vision Changes.

A 55-year-old woman presented with recurrent episodes of headache, vision changes, and language disturbances. Brain MRI showed multifocal white matter lesions, microhemorrhages, and enlarged perivascular spaces. After an extensive and unrevealing workup, she underwent a biopsy of brain and meninges that revealed thick and hyalinized leptomeningeal and cortical vessel walls that were strongly positive for ß-amyloid by immunohistochemical staining, suggestive of cerebral amyloid angiopathy (CAA). CAA can present as a spectrum of inflammatory responses to the deposition of amyloid-ß in the vessel walls. Her clinical presentation, radiologic, and histopathologic findings supported a diagnosis of probable CAA-related inflammation (CAA-ri). Although an uncommon entity, it is important to recognize it because most patients respond to immunosuppressive therapy.

Associação entre o número de deglutições, resíduo faríngeo e broncoaspiração na esclerose múltipla / Association between the number of swallowing, pharyngeal residue and bronchopulmonary aspiration in multiple sclerosis

RESUMO Objetivo verificar a associação entre o número de deglutições e presença de resíduo faríngeo e broncoaspiração em pessoas com esclerose múltipla. Métodos estudo transversal observacional de exames de videofluoroscopia de 231 deglutições de indivíduos com esclerose múltipla. Três fonoaudiólogas avaliaram as deglutições de IDDSI 1 (International Dysphagia Diet Standardisation Initiative) (5 ml e 10 ml) e IDDSI 4 (8 ml) quanto à presença de resíduo faríngeo e de penetração/aspiração. Deglutições que não apresentaram resíduo faríngeo foram classificadas como deglutições sem resíduos faríngeos (DSR) e as que apresentaram, como deglutições com resíduos faríngeos (DCR), sendo estas últimas subdivididas em resíduos faríngeos em todas as ofertas ou eventuais (DCR1 e DCR2). O número de deglutições foi analisado por um avaliador cego e comparado com os dados demográficos e clínicos. Resultados das 231 deglutições, 73 (31,6%) apresentaram resíduos faríngeos. O número médio de deglutições foi semelhante nas deglutições sem e com resíduos faríngeos em cada consistência e volume e nas variáveis idade, gênero, tipo de esclerose múltipla e incapacidade funcional. Houve associação entre a média do número de deglutições e a ausência de penetração/aspiração, quando comparada às deglutições sem e com resíduos faríngeos, nas DCR2 e em indivíduos acima de 50 anos. Ao analisar intragrupo, observou-se associação nas DCR, sendo maior na ausência de penetração/aspiração e nas DCR2. Conclusão não houve correlação entre o número de deglutições e a presença de resíduos em recessos faríngeos na esclerose múltipla. Todavia, o número de deglutições foi maior quando houve resíduo e ausência de disfagia e de penetração/aspiração, em indivíduos mais velhos.

ABSTRACT Purpose To verify the association between the number of swallows and the presence of pharyngeal residue and bronchoaspiration in people with Multiple Sclerosis. Methods An observational cross-sectional study of videofluoroscopic examinations of 231 swallows from individuals with Multiple Sclerosis. Three speech therapists evaluated IDDSI 1 (International Dysphagia Diet Standardisation Initiative) (5ml and 10ml) and IDDSI 4 (8ml) deglutitions for pharyngeal residue and penetration/ aspiration. Swallows with no pharyngeal residue were classified as swallows without pharyngeal residue (SWTR) and those with pharyngeal residue (SWR), the latter subdivided into pharyngeal residue in all or occasional offerings (SWR1 e SWR2). The number of swallows was analyzed by a blind evaluator and compared with demographic and clinical data. Results Of the 231 swallows, 73 (31.6%) showed pharyngeal residues. The mean number of swallows was similar in the deglutitions with and without pharyngeal residues in each consistency and volume and in the variables age, gender, type of Multiple Sclerosis and functional disability. There was an association between the mean number of swallows and the absence of penetration/aspiration when comparing deglutitions with and without pharyngeal residues, in SWR2 and in individuals over 50 years of age. When analyzing intragroup, an association was observed in SWR, being higher in the absence of penetration/aspiration and in SWR2. Conclusion There was no correlation between the number of swallows and the presence of residues in pharyngeal recesses in multiple sclerosis. However, the number of swallows was higher when there was residue and absence of dysphagia and penetration/aspiration, and in older individuals.

Aphasic status epilepticus in a tertiary referral center in Turkey: Clinical features, etiology, and outcome.

OBJECTIVE: Aphasic status epilepticus (ASE), although rare, may often remain underdiagnosed. We aimed to report the patients diagnosed with ASE and describe their clinical presentation, etiology, electrophysiological findings, neuroimaging, treatment options, and outcome. METHODS: We retrospectively analyzed a series of 28 patients diagnosed with ASE between January 2010 and August 2019 in our tertiary referral center. We reviewed medical files, patient charts, and short- and long-term intermittent electroencephalogram (EEG) recordings. Demographical, historical, clinical, neuroimaging, electrophysiological parameters, administered antiseizure medications, and prognosis of all patients were recorded. Furthermore, EEGs were re-evaluated according to Salzburg criteria. RESULTS: Most patients presented with tumors (n = 11) and cerebrovascular disease (CVD) (n = 11), while the rest were diagnosed with hyperglycemia (n = 2), autoimmune encephalitis (n = 1), remote intracranial abscess (n = 1), mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) attack (n = 1), or unknown cause (n = 1). Seventy-one percent of patients had prior history of epilepsy. The most common EEG findings were rhythmic delta activity with fluctuation and epileptiform discharges ≤ 2.5 Hz with spatiotemporal evolution (66 %). Magnetic resonance imaging (MRI) and EEG findings indicated dominant hemisphere involvement in all patients. No casualties occurred; 71 % patients exhibited total recovery, while 29 % had mild-moderate sequela aphasia. None of the patients received aggressive treatment for ASE. SIGNIFICANCE: A relatively higher number of ASE patients could be contributed to the literature through this study. ASE should be considered in patients with prolonged unexplained aphasia, especially with pre-existing risk factors, such as prior history of epilepsy, structural lesion, and metabolic disorders accompanied by concordant EEG findings. Although the underlying cause is a determining prognostic factor, this study shows a significant number of patients with complete remission; thus, the prognosis of ASE may be considered more favorable than other types of nonconvulsive status epilepticus.

Recovery of Severe Aphasia After Cranioplasty: Considerations on a Case Study.

Cranioplasty is the main surgical intervention for repairing cranial defects performed in about 80% of the patients following cancer surgery or decompressive craniectomy. Although some works have shown recovery of motor and cognitive function, including memory, attention, and executive functions, until today no studies have focused on language recovery after cranioplasty. A 68-year-old woman came to the Neurorehabilitation Unit of the IRCCS Neurolesi (Messina, Italy) because of a fluent aphasia due to a severe left nucleocapsular hemorrhage and greatly improved her motor and neuropsychological status after cranioplasty. Results confirmed that cranioplasty might significantly improve motor and neuropsychological function, besides aphasia. Healthcare professionals involved in rehabilitation should be aware of the potential role of cranioplasty in improving rehabilitative outcomes to better plan a more personalized rehabilitative program. Moreover, rehabilitation nurses can play a pivotal role within the rehabilitation process, as they are educated to interact and communicate with the patient suffering from aphasia.

Anti-NMDA receptor encephalitis presenting as isolated aphasia in an adult.

Anti-NMDA receptor (NMDA-r) encephalitis is a relatively rare cause of autoimmune encephalitis with divergent clinical presentations. We report a case of an adult patient with anti-NMDA-r encephalitis presenting with isolated, abrupt-onset aphasia. Her condition remained unaltered over a period of 6 months. The patients' electroencephalogram findings were typical for NMDA-r encephalitis; however, her magnetic resonance imaging and cerebrospinal fluid analysis were normal. She responded well to immunotherapy, and aphasia eventually resolved. The natural course of the present case contradicts the rapidly progressive nature of typical NMDA-r encephalitis. Furthermore, it broadens the clinical spectrum of anti-NMDA-r encephalitis, to incorporate isolated, nonprogressive aphasia.

Dislocated Dental Prothesis Sitting at the Larynx in an Aphasic Patient: Potentially Fatal Situation.

INTRODUCTION: Foreign body in respiration tract is a life-threatening emergency and requires urgent treatment. The diagnosis and treatment requires awareness and suspicion of signs and symptoms of foreign body aspiration. CASE: A unique case of total dental prothesis aspiration of a 44-year-old aphasic patient is presented. The prothesis is completely removed from the larynx. DISCUSSION: Foreign-body aspiration is frequently suspected in children, it is rarely thought about in adults with subacute or chronic respiratory symptoms unless an evident history of an aspiration event is obtained. Facial trauma, dental procedures, central nervous system dysfunction due to stroke, mental retardation, metabolic encephalopathy, seizures, and alcoholism are precipitating factors in adults. Careful clinical history and physical examination is mandatory. Foreign bodies located in the laryngeal region are almost always treated with surgery. CONCLUSION: Loose dentures must be avoided especially in mental retarded, aphasic patients, and patients with central nervous system dysfunction.

Screening for Language Disorders in Stroke: German Validation of the Language Screening Test (LAST).

BACKGROUND: Screening of aphasia in acute stroke is crucial for directing patients to early language therapy. The Language Screening Test (LAST), originally developed in French, is a validated language screening test that allows detection of a language deficit within a few minutes. The aim of the present study was to develop and validate two parallel German versions of the LAST. METHODS: The LAST includes subtests for naming, repetition, automatic speech, and comprehension. For the translation into German, task constructs and psycholinguistic criteria for item selection were identical to the French LAST. A cohort of 101 stroke patients were tested, all of whom were native German speakers. Validation of the LAST was based on (1) analysis of equivalence of the German versions, which was established by administering both versions successively in a subset of patients, (2) internal validity by means of internal consistency analysis, and (3) external validity by comparison with the short version of the Token Test in another subset of patients. RESULTS: The two German versions were equivalent as demonstrated by a high intraclass correlation coefficient of 0.91. Furthermore, an acceptable internal structure of the LAST was found (Cronbach's α = 0.74). A highly significant correlation (r = 0.74, p < 0.0001) between the LAST and the short version of the Token Test indicated good external validity of the scale. CONCLUSION: The German version of the LAST, available in two parallel versions, is a new and valid language screening test in stroke.

Aplicação do teste M1-Alpha em sujeitos normais com baixa escolaridade: estudo piloto / M1-Alpha test in normal subjects with low educational Level: a pilot study

OBJETIVO: Verificar o desempenho de sujeitos normais com baixa escolaridade no teste M1-Alpha e obter parâmetros que possam ser utilizados na avaliação clínica de pacientes afásicos com baixa escolaridade, expostos a este teste. MÉTODOS: Foram selecionados 30 sujeitos normais de baixa escolaridade (um a quatro anos de estudo), com idade superior a 18 anos e inferior a 60 anos, sendo 15 do gênero masculino e 15 do gênero feminino. Todos foram submetidos à aplicação do teste M1-Alpha, que comporta entrevista semidirigida e provas controladas. Todas as respostas corretas receberam um ponto. Os dados foram submetidos a tratamento estatístico. RESULTADOS: Foi verificado um maior número de erros, bem como uma maior variabilidade nas respostas, em tarefas de escrita copiada, ditado, leitura em voz alta e compreensão escrita. CONCLUSÃO: A baixa escolaridade influencia o desempenho dos indivíduos nas tarefas de escrita copiada, ditado, leitura em voz alta e compreensão escrita. Foi possível obter dados de referência, que poderão ser utilizados na aplicação clínica do teste M1-Alpha em pacientes com baixa escolaridade.

PURPOSE: To determine the performance of normal subjects with low educational level on the M1-Alpha test, and to obtain parameters for potential use in the clinical evaluation of aphasic patients with low educational level. METHODS: Participants were 30 normal subjects with low educational level (one to four years of schooling), 15 male and 15 female, with ages over 18 years and below 60 years. All subjects were submitted to the M1-Alpha test, which comprehends semi-directed interview and controlled tasks. One point was given for every correct answer. Data were statistically analyzed. RESULTS: It was verified a higher number of errors, as well as greater variability of responses, in the following tasks: copying, writing to dictation, reading comprehension, and reading aloud. CONCLUSION: Low educational level influences the performance of subjects on the tasks copying, writing to dictation, reading aloud, and reading comprehension. It was possible to obtain reference data for potential clinical application of the M1-Alpha test in patients with low educational level.

The effects of cortical ischemic stroke on auditory processing in humans as indexed by transient brain responses.

OBJECTIVE: To investigate the effects of cortical ischemic stroke and aphasic symptoms on auditory processing abilities in humans as indicated by the transient brain response, a recently documented cortical deflection which has been shown to accurately predict behavioral sound detection. METHODS: Using speech and sinusoidal stimuli in the active (attend) and the passive (ignore) recording condition, cortical activity of ten aphasic stroke patients and ten control subjects was recorded with whole-head MEG and behavioral measurements. RESULTS: Stroke patients exhibited significantly diminished neuromagnetic transient responses for both sinusoidal and speech stimulation when compared to the control subjects. The attention-related increase of response amplitude was slightly more pronounced in the control subjects than in the stroke patients but this difference did not reach statistical significance. CONCLUSIONS: Left-hemispheric ischemic stroke impairs the processing of sinusoidal and speech sounds. This deficit seems to depend on the severity and location of stroke. SIGNIFICANCE: Directly observable, non-invasive brain measures can be used in assessing the effects of stroke which are related to the behavioral symptoms patients manifest.