A case study of acute oropharyngeal palsy concomitant with diabetic ketoacidosis.

Acute oropharyngeal palsy is a rare variant of Guillain-Barré syndrome. In our study we present the case of a 63-year-old man with general symptoms who was diagnosed with diabetic ketoacidosis and prescribed insulin therapy. Two weeks later, the patient complained of paraesthesia of the perioral region and the tip of the tongue, dysphagia, and dysarthria. These symptoms were initially thought to be complications of the patient's type-1 diabetes. Due to rapidly developing paraparesis, the patient became bedridden. Clinical symptoms, cerebrospinal fluid analysis and a nerve conduction study resulted in a diagnosis of acute oropharyngeal palsy, a variant of Guillain-Barré syndrome. After five consecutive days of intravenous immunoglobulin treatment, neurological symptoms improved and the need for insulin ceased. One year later, the patient's only remaining neurological symptom was loss of tendon reflexes in the lower extremities. Furthermore, the patient's blood glucose level was normal without the use of medications or a special diet. Here, we report that oropharyngeal palsy can co-occur with diabetic ketoacidosis, and that immuntherapy is effective in treating both oropharyngeal palsy and type-1 diabetes. To our knowledge, this is the first description of a patient presenting with acut oropharyngeal palsy concomitant with diabetic ketoacidosis.

Visually guided associative learning in pediatric and adult migraine without aura.

INTRODUCTION: The Rutgers Acquired Equivalence Test is a visually guided equivalence learning paradigm that involves rule acquisition and generalization. Earlier we found impaired performance in this paradigm among adult migraine patients without aura. The aim of the study was to investigate if similar impairments can be found already in the pediatric form of the disease and to compare the performance of the pediatric study population with that of an adult study population. We hypothesized that the deficits observed in adults would be observable already in the pediatric population. METHODS: Twenty-seven children and adolescents newly diagnosed with migraine without aura and 27 age- and sex-matched healthy controls were tested with the Rutgers Acquired Equivalence Test. Their performance data were compared to each other and those of an earlier adult study population involving 22 patients and 22 age- and sex-matched healthy controls. Four parameters characterizing performance in the two main phases of the paradigm were calculated for each of the four groups. Performance parameters were compared with Mann-Whitney U test. RESULTS: In contrast to the decreased performance of the adult patients in the Rutgers Acquired Equivalence Test, no significant difference was found between pediatric patients and controls in any phase of the paradigm. CONCLUSION: Children living with migraine without aura do not exhibit the same cognitive deficits in the Rutgers Acquired Equivalence Test as their adult counterparts. It can be hypothesized that the deficit of equivalence learning is not an inherent feature of the migrainous cognitive profile, rather the result of the interference of the disease with normal development.

Acquired equivalence and related memory processes in migraine without aura.

Introduction Interictal deficits of elementary visuo-cognitive functions are well documented in patients with migraine and are mostly explained in terms of neocortical hyperexcitability. It has been suggested that the basal ganglia and the hippocampi might also be affected in migraine. If so, a deterioration of learning and memory processes related to these structures is expected. Methods A visual learning paradigm thought to be capable of dissociating learning/memory processes mediated by the basal ganglia from processes mediated by the hippocampus (the Rutgers Acquired Equivalence Test) was applied to a group of patients with migraine without aura and to age- and sex-matched controls. Results Patients with migraine showed a significantly poorer performance in both main phases of the test and the deficit in the phase considered to be dependent on the hippocampi was especially marked. Conclusions These results can be interpreted as behavioural support for findings that have suggested the involvement of the basal ganglia and the hippocampi in migraine, but further research is needed to clarify these findings.

[Thrombolysis in case of ischemic stroke caused by aortic dissection]. / Thrombolysis agyi infarktust okozó aortadissectio esetén.

Seldom, an acute aortic dissection can be the etiology of an acute ischemic stroke. The aortic dissection typically presents with severe chest pain, but in pain-free dissection, which ranges between 5-15% of the case, the neurological symptoms can obscure the sypmtos of the dissection. By the statistical data, there are 15-20 similar cases in Hungary in a year. In this study we present the case history of an acute ischemic stroke caused by aortic dissection, which is the first hungarian publication in this topic. A 59-year-old man was addmitted with right-gaze-deviation, acute left-sided weakness, left central facial palsy and dysarthric speech. An acute right side ischemic stroke was diagnosed by physical examination without syptoms of acute aortic dissection. Because, according to the protocol it was not contraindicated, a systemic intravenous thrombolysis was performed. The neurological sypmtoms disappeared and there were no complication or hypodensity on the brain computed tomography (CT). 36 hours after the thrombolysis, the patient become restlessness and hypoxic with back pain, without neurological abnormality. A chest CT was performed because of the suspition of the aortic dissection, and a Stanford-A type dissection was verified. After the acute aortic arch reconstruction the patient died, but there was no bleeding complication at the dissection site caused by the thrombolysis. This case report draws attention to the fact that aortic dissection can cause acute ischemic stroke. Although it is difficult to prove it retrospectively, we think the aortic dissection, without causing any symptoms or complain, had already been present before the stroke. In our opinion both the history of our patient and literature reviews confirms that in acute stroke the thrombolysis had no complication effect on the aortic dissection but ceased the neurological symptoms. If the dissection had been diagnosed before the thrombolysis, the aortic arch reconstruction would have been the first step of the treatment, without thrombolysis.

Efficacy and safety of abobotulinumtoxinA liquid formulation in cervical dystonia: A randomized-controlled trial.

BACKGROUND: Approved botulinum toxin A products require reconstitution. AbobotulinumtoxinA solution for injection is a ready-to-use liquid formulation of abobotulinumtoxinA. OBJECTIVES: The objective of this study was to demonstrate the superior efficacy of abobotulinumtoxinA solution for injection to placebo and to test the noninferior efficacy of abobotulinumtoxinA solution for injection versus abobotulinumtoxinA (dry formulation) in cervical dystonia. METHODS: This was a phase-3, multicenter, prospective, double-blind, randomized, active, and placebo-controlled study (N = 369). Patients with cervical dystonia were randomized (3:3:1) to abobotulinumtoxinA solution for injection 500 U, abobotulinumtoxinA 500 U, or placebo. Following the double-blind phase, patients received abobotulinumtoxinA solution for injection, open-label, for up to 4 cycles. The primary outcome was change from baseline at week 4 of the Toronto Western Spasmodic Torticollis Rating Scale total score. Secondary measures included change from baseline or cycle baseline in Toronto Western Spasmodic Torticollis Rating Scale scores. RESULTS: At week 4, both products were superior to placebo (Toronto Western Spasmodic Torticollis Rating Scale total score least square mean decrease from baseline, abobotulinumtoxinA solution for injection 500 U -12.5, abobotulinumtoxinA 500 U -14.0, placebo -3.9; P < .0001 vs placebo). The noninferiority limit of 3 points in the Toronto Western Spasmodic Torticollis Rating Scale total score at week 4 was not met for abobotulinumtoxinA solution for injection versus abobotulinumtoxinA. Toronto Western Spasmodic Torticollis Rating Scale total score reductions were maintained for up to 4 cycles of abobotulinumtoxinA solution for injection open-label follow-up treatment. Safety profiles of abobotulinumtoxinA solution for injection and abobotulinumtoxinA were similar, with dysphagia and injection-site pain the most frequent drug-related adverse events. CONCLUSIONS: Although the predefined noninferiority criterion was not met, abobotulinumtoxinA solution for injection was similarly effective to freeze-dried abobotulinumtoxinA in reducing Toronto Western Spasmodic Torticollis Rating Scale total scores with a similar safety profile. AbobotulinumtoxinA solution for injection efficacy was maintained with chronic open-label treatment, and this novel formulation may add convenience as well as dosing accuracy to treatment with abobotulinumtoxinA. © 2016 International Parkinson and Movement Disorder Society.

Quantitative assessment of brain iron by R2* relaxometry in patients with cervical dystonia.

BACKGROUND: The pathophysiology of cervical dystonia is poorly understood. Increased brain iron deposition has been described in different movement disorders. Our aim was to investigate brain iron content in patients with cervical dystonia, using R2* relaxation rate, a validated MRI marker of brain iron level. METHODS: Twelve female patients with primary focal cervical dystonia (mean age: 45.4 ± 8.0 years) and 12 age-matched healthy female subjects (mean age: 45.0 ± 8.0 years) underwent 3T MRI to obtain regional R2* relaxation rates of the thalamus, caudate nucleus, putamen, and globus pallidus (GP). Regions of interest were delineated automatically on T1-weighted MRIs. RESULTS: R2* values in the putamen were positively correlated with age. Patients with cervical dystonia showed elevated R2* values in the GP. CONCLUSIONS: This pilot study provides the first quantitative support for increased brain iron deposition in cervical dystonia. Further studies are needed to explore the implications of this finding.

[Changes of the T cell composition in the thymus during the COVID-19 pandemic]. / A csecsemomirigy T-sejtjeinek összetételében létrejövo változások a COVID­19-pandémia alatt.

INTRODUCTION: In our study, we aimed to investigate whether the COVID-19 infection itself or the vaccination against it affect the differentiation of T cells in the thymus, and whether the reduction in T cell counts observed in the blood of COVID-19-infected individuals is also observed at the tissue level in the thymus. METHOD: Data from a total of 55 thymectomy patients were processed to create three groups: 1) the pre-COVID-19 (PC) group included 22 patients, 12 women and 10 men, who underwent thymectomy between 2008 and 2013; 2) in the no-COVID-19 (NC) group (patients without verified infection or vaccination), 20 patients, 11 women and 9 men, underwent thymectomy in 2020-2021; 3) the vaccinated or infected COVID-19 (VIC) group included 13 patients, 4 women and 9 men, who underwent thymectomy also in 2020-2021. The pathological samples were immunohistochemically tested for CD4, CD8, CD25 and FOXP3 to verify the helper, cytotoxic and regulatory T cells. RESULTS: The VIC group had significantly lower values for CD4, compared to the PC and NC groups. The FOXP3 value was significantly lower in the VIC and NC groups compared to the PC group. No significant differences were found for CD8 and CD25 between the groups studied. DISCUSSION: The COVID-19 infection or vaccination affects the T cell composition of the thymus. Decreased expression of CD4 has been demonstrated in the VIC group, which confirms a decrease in the T cell counts that also occurs in the thymus. The low FOXP3 levels observed in the NC group during the COVID-19 era, compared to the PC group, may be indicative of a high rate of asymptomatic coronavirus infections and a worsening of immunetolerance. CONCLUSION: First in the world, we have verified that the helper T cell composition of the thymus in COVID-19 infection era is reduced, and in the asymptomatic patients the immune function is decreased as well. Orv Hetil. 2022; 163(52): 2062-2066.

Safety, effectiveness, and duration of effect of BOTOX after switching from Dysport for blepharospasm, cervical dystonia, and hemifacial spasm dystonia, and hemifacial spasm.

BACKGROUND: Local injection of botulinum toxin type A is first-line treatment of blepharospasm, cervical dystonia, and hemifacial spasm; however, there is uncertainty about the optimal dose of toxin for each indication as well as dose-conversion ratios that should be used when switching products in clinical practice. OBJECTIVE: The goal of this study was to compare the safety, effectiveness, and duration of clinical effect of BOTOX and Dysport and Dysportdagger after drug switching (Dysport to BOTOX) among patients with movement disorders. METHODS: A total of 48 patients diagnosed with blepharospasm (n = 27), cervical dystonia (n = 12), or hemifacial spasm (n = 9) were evaluated during a single-arm, crossover-design study in which each patient was his/her own historical control using a 5:1 or 4:1 conversion ratio of Dysport to BOTOX units. Patients were assessed (using Jankovic, Visual Analog Scale, general pain scale, Toronto Western Spasmodic Torticollis Rating Scale [TWSTRS] scores) after the last injection of Dysport and the first injection of BOTOX. Moreover, each patient kept a diary during treatment to track onset and duration of therapeutic effect. RESULTS: Although BOTOX and Dysport were both clinically effective, this effect was more significant with BOTOX compared to Dysport based on TWSTRS for cervical dystonia (p = 0.012), and Jankovic scores for blepharospasm (p = 0.006). Longer duration of effect also was noted with BOTOX than with Dysport (blepharospasm, 62.2 days vs 47.4 days (p = 0.001); cervical dystonia, 64.3 days vs 44.6 days (p = 0.014); hemifacial spasm, 65.1 days vs 41.8 days (p < 0.014), respectively). Of the 48 patients, 19 experienced at least one adverse drug reaction (ADR) during Dysport treatment, with the most commonly reported ADRs being ptosis for blepharospasm and hemifacial spasm and neck weakness for cervical dystonia. No patient reported an ADR during BOTOX treatment. CONCLUSIONS: Results suggest therapeutic effectiveness is enhanced with BOTOX compared to Dysport at a dosing ratio between 5:1 and 4:1 (Dysport:BOTOX). Safety and duration of therapeutic effect also are enhanced with BOTOX. Further research is needed.

[Multidisciplinary therapy of Tourette syndrome]. / Tourette-szindróma multidiszciplináris kezelése.

The marked fluctuation in symptoms with a spectrum of behavioral problems contribute to misdiagnosis of Tourette syndrome. The authors review the recent progress in diagnosis and management with an emphasis on multidisciplinary approach. Possible associations with various genes have been found in etiology of Tourette syndrome. Development of the disease comes of dopaminerg neurotransmission disorder resulting in cortico-striato-thalamic system dysfunction. Tics are brief movements or sounds that occur intermittently and unpredictably mimicking fragments of normal behavior. Diagnostic criteria are based on the motor and vocal phenomena and their dynamics. The key concept in management are the tic severity scaling correlating with quality of life measurements. Therapeutic interventions indicated at severe alteration in patient's quality of life. Treatment plan combines various drug protocols, psychotherapy and behavioral therapy which should be optimalized for most disabling symptom. Social isolation and self injurious behavior complicates the treatment resistant, severe cases. In these subgroup of patient, an adequate selection of stereotactic intervention could provide an effective control of tic severity or behavioral disorder. Tourette syndrome, as a typical neuropsychiatric disorder, is a striking example for improved efficacy of multidisciplinary approach.