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1.
Croat Med J ; 62(2): 154-164, 2021 Apr 30.
Article in English | MEDLINE | ID: mdl-33938655

ABSTRACT

AIM: To assess the correlations of B regulatory cells (Bregs) and monocyte subsets in peripheral blood with the National Institutes of Health (NIH)-consensus-defined clinical manifestations of chronic graft-vs-host disease (cGvHD), in an attempt to establish their role as cellular biomarkers. METHODS: This multidisciplinary prospective study enrolled adult cGVHD patients treated in the University Hospital Center Zagreb and University of Zagreb School of Medicine. Immunophenotypic subpopulations of CD24highCD38high Bregs (CD27-, CD27+, and total) and monocyte (classical, intermediate, and non-classical) counts were correlated with demographic, transplant, and cGVHD-related data. Bivariate correlation analysis was performed to evaluate the correlations between Bregs and monocytes subsets and cGVHD organ involvement, as well as cGVHD severity and immunosuppression intensity. RESULTS: Twenty-two adult patients (54.5% female) with cGVHD were enrolled. The median (range) age was 44.5 years (24-65). All patients were transplanted for hematologic malignancies and 40.9% had severe NIH cGVHD global score. The median time from cGVHD diagnosis to the analysis was 16.6 months (0-176). The organ most frequently affected with cGVHD were the eyes (68.2%), skin (45.5%), lungs (45.5%), and liver (40.9%). Lower total and CD27-Bregs counts were correlated with worse cGVHD severity, higher immunosuppression intensity, and lung cGVHD, in terms of cell count, but also with skin cGVHD, in terms of percentages. Patients with liver and joint/fascia cGVHD had a lower percentage of non-classical monocytes and patients with more severe global NIH score had a higher classical monocytes count. CONCLUSION: Different organs affected by cGVHD are differently associated with different subpopulations of Bregs and monocytes.


Subject(s)
Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Adult , Aged , Chronic Disease , Female , Humans , Male , Middle Aged , Monocytes , Prospective Studies , United States , Young Adult
2.
Psychiatr Danub ; 33(Suppl 4): 1267-1273, 2021.
Article in English | MEDLINE | ID: mdl-35503939

ABSTRACT

The aim of this review is to emphasize the importance of mental activity and aerobic physical exercise as one of the most important health-related activities which may delay the onset or slow down the progression of Alzheimer's dementia. Studies have shown that the elderly who regularly engage in mental and physical activities have a lower risk of dementia development. Performing mental and physical activities regularly has a synergistic effect on the improvement of cognitive functions. Complex mental activity during life is associated with a reduction in the hippocampal atrophy rate, which is a sensitive early-stage biomarker of dementia while regular physical exercise can slow down the progressive reduction of the cortical brain volume which occurs during aging. Mental activity increases a person's "cognitive reserve" and promotes the formation of new communications between brain cells. Since it is not possible to influence genetic components of Alzheimer's dementia, preventative interventions such as encou¬raging regular engagement in mental and physical activities are extremely important. Activities need to be safe, moderate, comfortable, and adapted as to type, duration, and especially the health and functional status of the patient. In the near future, it is expected that genome analysis in personalized medicine will guide us in the right direction on certain types of physical and mental exercise.


Subject(s)
Alzheimer Disease , Aged , Aging , Alzheimer Disease/therapy , Atrophy , Cognition , Exercise , Humans
3.
Psychiatr Danub ; 33(Suppl 4): 1298-1302, 2021.
Article in English | MEDLINE | ID: mdl-35503945

ABSTRACT

BACKGROUND: Patients with obesity may have symptoms of sexual dysfunction (SD). Little is known about these symptoms in obese patients in Croatia and the aim of this study was to explore them. SUBJECTS AND METHODS: This was a cross-sectional study carried out in tertiary healthcare centre at the Croatian Obesity Treatment Referral Center in University Hospital Center of Zagreb. 103 patients (72 female, 31 male, mean age 48.7±11.87 years, mean BMI 40.42) were included. SD symptoms were assessed using the internationally acclaimed questionnaire Arizona Sexual Experience Scale (ASEX), that was recently validated for Croatian language. Patients were also administered a questionnaire, prepared for this purpose, that enquired about their previous known chronic disorders. Statistical analyses included t-test, chi-squared test and bivariate Pearson's correlations. RESULTS: Average total response on the questionnaire was 12.4 (women 13.2, range 3-30; men 10.6, range from 5-19). A total score of 19 or more was present in 5 (4.8 %, range from 19-30, average 22.4; 1 man, 4 women), at least one question with a score 5 or greater on any item was found in 36 (34.9 %, 5 men, 31 women), while a score of 4 or more on three items was found in 20 patients (19.4 %, 2 men, 18 women). Overall median response was 3 (range 1-6). Women were found to have more pronounced symptoms of SD (p<0.05). The overall results on ASEX were found to be in significant correlation with regard to depression (r=0.22, p=0.03), as well as anxiety (r=0.2, p=0.04). Significant correlations were also found with regard to age (r=0.31), mobility (r=0.25), and pain/uneasiness (r=0.22) (p<0.05). CONCLUSIONS: This study brings valuable observations on the presence of SD symptoms in obese patients in Croatia. SD symptoms were found to be present in up to one-third of our patients, more pronounced in women, and in significant correlation with depression and anxiety. However, median response on ASEX suggests that overall SD symptoms in our group of patients are not that expressed.


Subject(s)
Language , Sexual Dysfunction, Physiological , Adult , Croatia/epidemiology , Cross-Sectional Studies , Female , Humans , Male , Middle Aged , Obesity/epidemiology , Sexual Dysfunction, Physiological/epidemiology , Sexual Dysfunction, Physiological/etiology
4.
Psychiatr Danub ; 33(Suppl 4): 490-495, 2021.
Article in English | MEDLINE | ID: mdl-34718271

ABSTRACT

BACKGROUND: Patients with epilepsy commonly report sexual dysfunction (SD) and reproductive difficulties. This study aimed to evaluate the relationship between epilepsy, antiepileptic drugs (AEDs) and SD, and its association with the quality of life and depressive symptoms. SUBJECTS AND METHODS: This was a prospective study carried out in a tertiary healthcare centre. SD was evaluated using the internationally acclaimed questionnaire Arizona Sexual Experiences Scale (ASEX) that was successfully translated into Croatian and validated for this purpose. Depressive symptoms and quality of life were evaluated using the Hamilton Rating Scale for Depression (HAM-D17) and Quality of life in epilepsy-31 inventory (QOLIE-31). RESULTS: Of 108 patients (68 (63 %) women, 40 (37 %) men, mean age 39.54±15.91 (range18-80) years) with epilepsy, 16 (14.8%) had focal, 38 (35.2%) generalized and 44 (40.7%) both types of epilepsy. Mean overall total score on the ASEX questionnaire was 11.94±5.61 (mean total score women 12.85±6.00, mean total score men 10.4±4.55), with 48 reporting that they had sexual activity in the past week. Nine (8.33%) patients (7 (6.48%) women, 2 (1.85%) men, mean age 47.66±19.33 (range 25-80) years) had a score 19 and above, 38 (35.18%) patients (27 (25%) women, 9 (8.33%) men, mean age 46.82±17.78 (range 19-80) years) individual score 5 and above on any one item, and 33 (30.55%) patients (26 (24.07%) women, 7 (6.48%) men, mean age 48.87±17.8 (range 19-80) years) had an individual score 4 and above on any three items. Significant correlations were found between SD and older age (p=0.001) and between more pronounced symptoms regarding SD on ASEX and female gender (p=0.000). There were no significant correlations between the type of epilepsy and SD, nor between the AEDs (old generation vs. modern) and SD. Significant correlations were found between the SD and more pronounced depressive symptoms (p=0.003) and between the SD and a lower quality of life (p=0.001). CONCLUSIONS: Results of our study suggest SD is experienced by around one-third of patients in our group, which is similar to the previous percentage of SD reported in the community sample. Women were found to experience more pronounced symptoms of SD on ASEX. Symptoms of SD were found to be significantly correlated with older age, female gender, lower quality of life and depressive symptoms, while no significant correlations were found with the type of epilepsy and the AEDs.


Subject(s)
Epilepsy , Sexual Dysfunction, Physiological , Adult , Aged , Aged, 80 and over , Anticonvulsants/adverse effects , Epilepsy/drug therapy , Epilepsy/epidemiology , Female , Humans , Male , Middle Aged , Prospective Studies , Quality of Life , Sexual Dysfunction, Physiological/epidemiology , Young Adult
5.
Acta Clin Croat ; 60(4): 758-764, 2021 Dec.
Article in English | MEDLINE | ID: mdl-35734498

ABSTRACT

Intracranial aneurysms have a prevalence of about 2% of the population. They are a common incidental finding of noninvasive neuroimaging methods, raising the question of the necessity of treatment of patients with an asymptomatic intracranial aneurysm. For long, the only treatment option was surgical clipping of aneurysm neck. In the last 25 years, endovascular techniques have been developed as an alternative solution for patients who are not eligible for neurosurgical procedures. Research has shown better results of embolization procedures with lower rates of complications, but a higher rate of recanalization is still a major drawback of endovascular coiling. There are no strict protocols and the treatment of choice for intracranial aneurysms should be agreed upon by both the physician and the patient. This review aims to provide an insight into the management of intracerebral aneurysms with emphasis on the decision making problems faced by clinicians.


Subject(s)
Aneurysm, Ruptured , Embolization, Therapeutic , Endovascular Procedures , Intracranial Aneurysm , Aneurysm, Ruptured/surgery , Embolization, Therapeutic/adverse effects , Embolization, Therapeutic/methods , Endovascular Procedures/adverse effects , Endovascular Procedures/methods , Humans , Intracranial Aneurysm/diagnosis , Intracranial Aneurysm/etiology , Intracranial Aneurysm/therapy , Neurosurgical Procedures/adverse effects , Treatment Outcome
6.
Br J Neurosurg ; 34(3): 316-320, 2020 Jun.
Article in English | MEDLINE | ID: mdl-32046512

ABSTRACT

Background: Normal pressure hydrocephalus (NPH) is communicating hydrocephalus characterised by normal intraventricular pressures. It presents with the triad of gait impairment, cognitive decline, and urinary incontinence. The term idiopathic normal pressure hydrocephalus (iNPH) is used in cases where the etiology is unknown. The aim of this study was to assess the prevalence and management of iNPH in our institution.Method: This was a retrospective study carried out at a tertiary health care center. Retrospective case series analysis was conducted using the existing electronic medical record data (2009-2017) on patients with hydrocephalus.Results: Forty-two (6.7%) patients with iNPH were identified, mean age 71.5 ± 8.8 years, 21 male (mean age 71.5 ± 9.3 years) and 21 female (mean age 71.5 ± 8.5 years). Ataxia was recorded in 39, symptoms of dementia in 31, and urinary incontinence in 29 patients. Forty patients were treated surgically by placing a ventriculoperitoneal (VP) shunt. One of the two patients treated by endoscopic third ventriculostomy (ETV) was subsequently treated by placing a VP shunt due to clinical deterioration. Significant improvements were noticed in cognitive and urinary symptoms, in the triad symptom sum score on the Japanese NPH scale, as well as in Evans' index and callosal angle (CA) on brain MRI (p < 0.05). Significant positive correlation was found between age and gait disturbance (Spearman's rho = 49.86% p = 0.0017), age and incontinence (Spearman's rho = 35.22%, p = 0.0351), age and triad symptom sum score (Spearman's rho = 44.67%, p = 0.0056), female gender and dementia (Spearman's rho = 34.94%, p = 0.0367), and among all three variables on the Japanese NPH scale (p < 0.0001).Conclusions: Treatment of iNPH with VP shunt showed significant improvement. A properly designed study is required to address the efficacy of ETV in the treatment of iNPH.


Subject(s)
Hydrocephalus, Normal Pressure , Hydrocephalus , Aged , Aged, 80 and over , Female , Humans , Hydrocephalus, Normal Pressure/epidemiology , Hydrocephalus, Normal Pressure/surgery , Male , Middle Aged , Retrospective Studies , Treatment Outcome , Ventriculoperitoneal Shunt , Ventriculostomy
7.
Croat Med J ; 61(1): 62-65, 2020 Feb 29.
Article in English | MEDLINE | ID: mdl-32118380

ABSTRACT

Hereditary factor XI (FXI) deficiency is a mild bleeding disorder, rare in the general population but relatively common among Ashkenazi Jews. The human F11 gene comprises 15 exons, spanning over 23 kb of the long arm of chromosome 4 (4q35). Homozygotes or compound heterozygotes typically show severe FXI deficiency, whereas heterozygotes show partial or mild deficiency. However, the genotype-phenotype relationship is difficult to establish, even among individuals within the same family. We report on a female patient with a heterozygous variant in F11 and FXI deficiency (49 IU/dL), who suffers from menorrhagia since menarche and easy bruising. She experienced excessive bleeding during thyroidectomy and after a cesarean section. Her younger sister, who carries the same heterozygous variant in F11 and has mild FXI deficiency (47 IU/dL), has menorrhagia without other bleeding difficulties although she has undergone several surgeries. Their father, who carries the same missense variant, has not experienced any bleeding difficulties (but he has not undergone any surgeries either). The family study revealed that the A428C mutation was inherited from the father. This variant has not previously been described in the literature and is the first F11 variant described in the Croatian population. Our study showed that even when family members have the same germline F11 variant, they still may experience phenotypic variability, making disease prognosis more complex.


Subject(s)
Factor XI Deficiency/genetics , Factor XI/genetics , Menorrhagia/genetics , Mutation, Missense/genetics , Adult , Cesarean Section , Exons , Female , Heterozygote , Humans , Male , Pedigree , Pregnancy , Thyroidectomy
8.
Psychiatr Danub ; 32(Suppl 4): 562-567, 2020 Nov.
Article in English | MEDLINE | ID: mdl-33212464

ABSTRACT

BACKGROUND: Patients with obesity may experience lower urinary tract symptoms (LUTS). Little is known about these symptoms in obese patients in Croatia. The aim of this study was to asses LUTS in this group of patients. SUBJECTS AND METHODS: This cross-sectional study was carried out in a tertiary healthcare centre. 111 participants were included (81 women and 30 men, age 23-78 years), with BMI>30 kg/m2. LUTS were evaluated using International consultation on incontinence questionnaires (ICIQ) investigating symptoms of overactive bladder (OAB) and urinary incontinence (UI): ICIQ-OAB and ICIQ-UI Short Form (SF). We evaluated also some of the questions on the EQ-5D-5L questionnaire. RESULTS: On ICIQ-OAB patients most often reported:UI (46.85% (N=52)), nocturia (42.34% (N=47)) and increased frequency of urination (34.23% (N=38)), and on ICIQ-UI SF: UI when coughing and sneezing (44.44% (N=32)), urgency UI (43.06% (N=31)) and UI during exercise/physical activity (22.22% (N=16)). Women were found to be more significantly affected by OAB symptoms (p<0.05). Significant correlations were found between the overall results on ICIQ-OAB and hypertension (r=0.32). CONCLUSIONS: The results of this study confirm that obese patients in Croatia experience LUTS as well. A higher incidence of LUTS was found among women and gender-independent among hypertensive obese patients.


Subject(s)
Lower Urinary Tract Symptoms/epidemiology , Obesity/epidemiology , Adult , Aged , Cough , Croatia/epidemiology , Cross-Sectional Studies , Exercise , Female , Humans , Hypertension/epidemiology , Male , Middle Aged , Sex Characteristics , Sneezing , Surveys and Questionnaires , Urinary Bladder, Overactive/epidemiology , Urinary Incontinence/epidemiology , Young Adult
9.
Neurol Sci ; 40(6): 1275-1278, 2019 Jun.
Article in English | MEDLINE | ID: mdl-30746559

ABSTRACT

ALS is the most frequent motor neuron disorder in adults with suggested complex relationship regarding gender. Studies investigating ALS and hormones have provided varying results. ALS onset during pregnancy is uncommon and pregnancy after the ALS symptom onset is even rarer. We present three patients with the onset of ALS symptoms before or during pregnancy and propose a putative disease modifying mechanism leading to attenuation of disease progression that we observed during the pregnancies.


Subject(s)
Amyotrophic Lateral Sclerosis/complications , Amyotrophic Lateral Sclerosis/pathology , Disease Progression , Pregnancy Complications , Female , Humans , Pregnancy
10.
Psychiatr Danub ; 31(1): 111-115, 2019 Mar.
Article in English | MEDLINE | ID: mdl-30948698

ABSTRACT

BACKGROUND: Alzheimer's disease (AD) and vascular dementia (VaD) represent a leading public-health problem given the rising age of the population. Early diagnosis of dementia, especially at the stage of mild cognitive impairment (MCI) has become an important goal of the modern patient work-up. Brain perfusion single-photon emission computed tomography (SPECT) has become a mainstay of diagnostic algorithms in patients with dementia showing specific patterns of hypoperfusion in temporal and parietal lobes. Clinical electroencephalography (EEG) is a relatively simple and inexpensive diagnostic tool showing potential in assessing cortical thinning and lower perfusion in temporoparoetal regions. SUBJECTS AND METHODS: Our study was a cross sectional and included retrospective analysis of the group of patients diagnosed with AD, VaD and MCI. The study group consisted of 50 patients - 29 females and 21 males. All patients underwent EEG and SPECT analysis as part of regular work-up. RESULT: Patients with AD exhibit EEG changes mostly in the form of theta waves, focal abnormalities and spike-and-wave complexes in frontotemporal regions with the reduction in the amplitude of alpha waves. SPECT in most patients exhibited hypoperfusion in temporoparietal regions with occasional unilateral abnormalities in frontotemporal region. EEG changes in patients with VaD are predominantly in the form of theta waves while SPECT findings show mostly "patchy" abnormalities. EEG readings are normal or exhibit minimal changes in the group of patients with MCI, while SPECT imaging exhibits mostly normal CBF. CONCLUSION: EEG and SPECT are diagnostic methods which show specific changes, especially in AD. EEG can be used to monitor the therapeutic effect and progression of AD as well as the possible transition from MCI to early stage AD. SPECT on the other hand, being a more expensive and less available method, can be utilized as an add-on method to increase the specificity and sensitivity of the diagnostic algorithm.


Subject(s)
Alzheimer Disease , Cognitive Dysfunction , Dementia, Vascular , Electroencephalography , Alzheimer Disease/diagnostic imaging , Cognitive Dysfunction/diagnostic imaging , Cross-Sectional Studies , Dementia, Vascular/diagnostic imaging , Female , Humans , Male , Retrospective Studies , Tomography, Emission-Computed, Single-Photon
11.
Psychiatr Danub ; 31(Suppl 5): 774-780, 2019 Dec.
Article in English | MEDLINE | ID: mdl-32160171

ABSTRACT

BACKGROUND: Electromyography (EMG) and nerve conduction studies (NCS) are an unpleasant and sometimes painful examinations. Pain can reduce patient's compliance and have a negative effect on the examination results. Different studies report that music affects pain perception by acting as a distractor, by inducing positive emotional valence or through the concept of convergence of different sensory modalities. The aim of this study was to explore the effect of music and different environmental and sociodemographic factors on pain perception during EMG and NCS. SUBJECTS AND METHODS: Sixty patients with suspected neuromuscular disease were randomized into music and control group. Specific questionnaire assessed sociodemographic characteristics, medical history, examination waiting time, examination extent and biometeorological forecast. The numerical rating scale was used for the evaluation of pain. The examiner evaluated patient's compliance after the examination. RESULTS: NCS was less painful for patients in the music group (p=0.03), as well as for more cooperative patients (p=0.011). For patients who previously underwent EMG/NCS, present NCS was more painful (p=0.001), regardless of the music intervention (p=0.019). EMG was more painful for older patients (p=0.041). Patients with lower level of education reported lower pain during NCS (p=0.026). Gender, financial satisfaction, biometeorological forecast, diabetes, depression or malignant disease, use and dosing of analgesics or antidepressants, symptoms, examination waiting time and the examination extent had no effect on pain perception. CONCLUSIONS: Music significantly decreased the perception of pain associated with NCS, but not the EMG portion of the examination. During EMG pain level was not significantly reduced, but the median of pain was still lower. Generally, the pain level during NCS, unlike the one during EMG, was affected by patients' compliance, level of education and painful predetermination. We propose using music during EMG/NCS because it can make the examination more comfortable for the patient and thus contribute to better quality of this examination.


Subject(s)
Electromyography , Music , Neural Conduction , Pain Perception/physiology , Pain/prevention & control , Pain/physiopathology , Female , Humans , Male , Middle Aged , Music Therapy
12.
Croat Med J ; 57(3): 229-38, 2016 Jun 30.
Article in English | MEDLINE | ID: mdl-27374824

ABSTRACT

AIM: To determine the frequency and the characteristics of cutaneous manifestations, especially vitiligo and alopecia areata, in patients with chronic graft-vs-host disease (cGVHD). METHODS: 50 patients with cGVHD were prospectively enrolled in the observational study protocol and evaluated by an experienced dermatologist. The evaluation was focused on the clinical spectrum of skin and adnexal involvement, and the cutaneous GVHD score was determined according to National Institutes of Health (NIH) Consensus criteria. The presence of vitiligo, alopecia, xerosis, nail changes, and dyspigmentation was also assessed. RESULTS: Out of 50 cGVHD patients, 28 (56%) had skin involvement, and 27 of them (96%) had hypo and/or hyperpigmentations. 11 patients (39%) had a mild cutaneous NIH cGVHD score, 22% moderate, and 39% severe. 15 (30%) patients had nail changes and 10 (20%) had vitiligo or alopecia areata. Univariate analysis showed that patients with vitiligo/alopecia areata received more lines of prior systemic immunosuppressive therapy (P=0.043), had lower Karnofsky performance status (P=0.028), and had a higher B-cell number (P=0.005), platelet count (P=0.022), and total protein (P=0.024). Vitiligo and alopecia areata were associated with higher NIH skin score (P=0.001), higher intensity of immunosuppressive treatment (P=0.020), and total body irradiation conditioning (P=0.040). Multivariate regression model showed that patients with higher NIH skin scoring were 3.67 times more likely to have alopecia and/or vitiligo (odds ratio 3.67; 95% confidence interval 1.26-10.73), controlled for all other factors in the model (age at study entry, number of B-cells, platelet count, and global NIH score). CONCLUSION: These data indicate that vitiligo and alopecia areata occur more frequently in cGVHD than previously reported.


Subject(s)
Alopecia Areata/complications , Graft vs Host Disease , Vitiligo/complications , Adolescent , Adult , Aged , Alopecia Areata/chemically induced , Child , Cohort Studies , Female , Hematopoietic Stem Cell Transplantation , Humans , Immunosuppressive Agents/adverse effects , Male , Middle Aged , Prospective Studies , Transplantation, Homologous , Vitiligo/chemically induced , Young Adult
13.
Lijec Vjesn ; 137(9-10): 297-306, 2015.
Article in Croatian | MEDLINE | ID: mdl-26749953

ABSTRACT

Complex regional pain syndrome (CRPS) represents a state of constant and often disabling pain, affecting one region (usually hand) and often occurs after a trauma whose severity does not correlate with the level of pain. The older term for this condition of chronic pain associated with motor and autonomic symptoms is reflex sympathetic dystrophy or causalgia. The aim of this review, based on contemporary literature, is to show the epidemiology and etiology, proposed pathophysiological mechanisms, method of diagnosis and treatment options, prevention and mitigation of this under-recognized disease. CRPS I occurs without known neurological damage, unlike CRPS II, where the history of trauma is present and in some cases damage to the peripheral nervous system can be objectively assessed using electromyoneurography. New diagnostic methods, such as quantitative sensory testing (CST), challenge this division because the CST findings in patients with CRPS I can suggest damage to Adelta peripheral nerve fibers. Except for distinguishing type I and type II disease, it is important to bear in mind the diversity of clinical presentation of CRPS in acute and chronic phase of the disease. This regional pain syndrome typically includes the autonomic and motor signs and thus differs from other peripheral neuropathic pain syndromes. The complexity of the clinical presentation indicates the likely presence of different pathophysiological mechanisms underlying this disease. Previous studies have demonstrated the autonomic dysfunction, neurogenic inflammation and neuroplastic changes. The diagnosis of CRPS is based on anamnesis and clinical examination on the basis of which the disease can be graded according to the Budapest Criteria. A valuable aid in differentiating subtypes of the disease is electromyoneurography. The treatment of CRPS is as complex as the clinical picture and the pathophysiology of the disease and requires interdisciplinary cooperation and individual approach. The pharmacological approach is mainly symptomatic, including analgesics, glucocorticoids, baclofen, bisphosphonates and prophylactic administration of vitamin C. Physical therapy besides preventing atrophy and contractures reduces the use of analgesic therapy. Invasive approach includes stimulation of the spinal cord, peripheral nerve catheters with anesthetic and amputation that patients in severe condition gladly accept. Further research is needed to better understand the disease and more effective therapies.


Subject(s)
Complex Regional Pain Syndromes , Pain Management/methods , Pain Measurement/methods , Complex Regional Pain Syndromes/diagnosis , Complex Regional Pain Syndromes/epidemiology , Complex Regional Pain Syndromes/therapy , Global Health , Humans , Incidence
14.
Clin Neurol Neurosurg ; 237: 108122, 2024 02.
Article in English | MEDLINE | ID: mdl-38262155

ABSTRACT

Even though the classical clinical concept supports the clear difference between diseases affecting the central and peripheral nervous systems, this difference is becoming less rigid. Here, we report the case of a 50-year-old male patient who presented with acroparaesthesia, headache, and flaccid tetraparesis after febrile diarrhea. Nerve conduction studies indicated action potentials with low amplitudes, which are typical in acute motor and sensory axonal neuropathy. Magnetic resonance revealed a round lesion in the splenium consistent with a diagnosis of reversible splenial lesion syndrome. A polyclonal antiganglioside antibody response was detected. The patient was successfully treated with intravenous immunoglobulins. The coexistence of reversible splenial lesion syndrome and acute motor and sensory axonal neuropathy has not been described in the literature so far. We discuss our diagnostic dilemmas and the possible role of antiganglioside antibodies in the occurrence of simultaneous lesions of the central and peripheral nervous systems.


Subject(s)
Guillain-Barre Syndrome , Peripheral Nervous System Diseases , Male , Humans , Middle Aged , Guillain-Barre Syndrome/complications , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/drug therapy , Peripheral Nervous System Diseases/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Magnetic Resonance Imaging
15.
J Neuromuscul Dis ; 11(1): 75-84, 2024.
Article in English | MEDLINE | ID: mdl-38073396

ABSTRACT

BACKGROUND: Risdiplam is an orally administered treatment for spinal muscular atrophy which leads to an improvement in motor function as measured by functional motor scales compared with placebo. Although risdiplam has been registered since 2020, real-world data in adults is still scarce. There have been no new safety signals so far, with some results pointing that risdiplam may be effectiveObjective:The objective was to present real-world data of 31 adult patients with spinal muscular atrophy type 2 and type 3 treated with risdiplam in the Republic of CroatiaMethods:Treatment effects were assessed with motor function tests and patient reported outcome measures, including Individualized Neuromuscular Quality of Life questionnaire, and Jaw Functional Limitation Scale. Side effects, as well as subjective improvements and symptoms, were noted. RESULTS: Majority of patients did not report any side effects. During treatment, we have observed clinically meaningful improvements in some patients, with stabilization of motor functions in the remaining patients. The majority of patients with bulbar function impairment experienced bulbar function improvement, all patients reported an increased quality of life with treatment. An unexpected observed treatment effect was weight gain in a third of all patients with some patients reporting an increase in appetite and subjective improvement in digestion. CONCLUSIONS: Risdiplam treatment was well tolerated with subjective and objective positive outcomes registered as measured by functional motor scales and patient-reported outcomes. Since risdiplam is administered orally and acts as a systemic therapy for a multisystemic disorder, effects in systems other than neuromuscular can be expected and should be monitored. Due to systemic nature of the disease patients need multidisciplinary monitoring.


Subject(s)
Muscular Atrophy, Spinal , Spinal Muscular Atrophies of Childhood , Adult , Humans , Quality of Life , Muscular Atrophy, Spinal/drug therapy , Motor Neurons , Spinal Muscular Atrophies of Childhood/drug therapy
17.
World J Surg Oncol ; 11: 224, 2013 Sep 12.
Article in English | MEDLINE | ID: mdl-24028681

ABSTRACT

OBJECTIVES: To refresh clinical diagnostic dilemmas in patients presenting with symptoms resembling to those of parkinsonism, to report rare association of colon cancer and paraneoplastic stiff person syndrome (SPS), and to draw attention on the possible correlation of capecitabine therapy with worsening of paraneoplastic SPS. METHODS: Case report of the patient with paraneoplastic SPS due to colon cancer that was misdiagnosed as idiopathic Parkinson's disease (iPD), whose symptoms worsened after beginning adjuvant capecitabine chemotherapy. RESULTS: We describe a 55-year-old woman with subacute onset of symmetrical stiffness and rigidity of the truncal and proximal lower limb muscles that caused lower body bradykinesia, gait difficulties, and postural instability. Diagnose of iPD was made and levodopa treatment was initiated but failed to provide beneficial effect. Six months later, colon cancer was discovered and the patient underwent surgical procedure and chemotherapy with capecitabine thereafter. Aggravation of stiffness, rigidity, and low back pain was observed after the first chemotherapy cycle and capecitabine was discontinued. Furthermore, levodopa was slowly discontinued and low dose of diazepam was administered which resulted in partial resolution of the patient's symptoms. CONCLUSION: Paraneoplastic SPS is rare disorder with clinical features resembling those of parkinsonian syndrome and making the correct diagnosis remains a challenge. The diagnosis of parkinsonian syndrome should be re-examined if subsequent examinations discover an associated malignant process. Although it remains unclear whether the patients with history of SPS are at the greater risk for symptoms deterioration after administration of capecitabine, clinicians should be aware of capecitabine side effects because recognition and appropriate management can prevent serious adverse outcomes.


Subject(s)
Antimetabolites, Antineoplastic/adverse effects , Colonic Neoplasms/diagnosis , Deoxycytidine/analogs & derivatives , Diagnostic Errors , Drug-Related Side Effects and Adverse Reactions/etiology , Fluorouracil/analogs & derivatives , Parkinson Disease/diagnosis , Stiff-Person Syndrome/diagnosis , Capecitabine , Colonic Neoplasms/drug therapy , Deoxycytidine/adverse effects , Female , Fluorouracil/adverse effects , Humans , Middle Aged , Parkinson Disease/physiopathology , Prognosis , Stiff-Person Syndrome/drug therapy
18.
Coll Antropol ; 37(3): 1015-8, 2013 Sep.
Article in English | MEDLINE | ID: mdl-24308253

ABSTRACT

Complex regional pain syndrome type I (CRPS I) is a disorder of one or more extremities characterized by pain, abnormal sensitivity (allodynia), swelling, limited range of motion, vasomotor instability, fatigue and emotional distress. The symptoms may be aggravated by even minor activity or weather change. It is usually provoked by injury, surgery or injection but in a small proportion of patients CRPS I develops without a clear causative event. There are several literature reports on CRPS after rubella and hepatitis B vaccination. We present a case of CRPS I affecting the left arm after diphtheria and tetanus (Di-Te) vaccination in the left deltoid muscle in a young girl having experienced profound emotional stress before the vaccination procedure. History data on previous minor trauma at the site of vaccination or emotional stress may necessitate temporary vaccination delay due to their proneness to impaired local or systemic immune response and CRPS as a complication of vaccination. If a child or an adult has prominent swelling and severe pain after vaccination, the diagnosis of CRPS I should be considered and if confirmed, the multidisciplinary treatment should start as soon as possible.


Subject(s)
Arm , Diphtheria-Tetanus Vaccine/adverse effects , Edema/etiology , Reflex Sympathetic Dystrophy/etiology , Adolescent , Edema/immunology , Female , Humans , Reflex Sympathetic Dystrophy/immunology
19.
Cureus ; 14(7): e27213, 2022 Jul.
Article in English | MEDLINE | ID: mdl-36035038

ABSTRACT

Coronavirus disease 2019 (COVID-19) vaccines have been reported as possible triggers of the production of antibodies pathogenic to the peripheral nerve and neuromuscular junction. We report on a patient who experienced vertical diplopia three weeks after the booster dose of the Pfizer-BioNTech vaccine (Comirnaty®). The diagnosis of myasthenia gravis (MG) was established based on highly positive antibodies to the nicotinic acetylcholine receptor (nAChR). Treatment with pyridostigmine and prednisone was started with gradually raising doses. On a follow-up exam two months after treatment initiation, clinical improvement was noted with an almost normal bulbomotor examination. The occurrence of diplopia following COVID-19 vaccination should raise suspicion of new-onset ocular MG and testing for anti-nAChR antibodies is advised.

20.
Vaccines (Basel) ; 10(1)2022 Jan 13.
Article in English | MEDLINE | ID: mdl-35062778

ABSTRACT

The BNT162b2 (Pfizer BioNTech) mRNA vaccine is an effective vaccine against COVID-19 infection. Here, we report an adverse event following immunization (AEFI) in a 48-year-old female patient who presented with fasciculations, migraine auras without headaches and in an increased discomfort of previously present palpitations, as well as excitation and insomnia. Her fasciculations were intermittently present until the time this paper was written, starting from the 6th day post-vaccination; they changed localization and frequency, but most commonly they were generalized, affecting almost all muscle groups. The patient also suffered from two incidents of migraine auras with visual kaleidoscope-like phenomena without headaches a few months after the vaccination. These symptoms were considered to be AEFI and no causal relation with the vaccine could be proven.

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