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Lege Artis Medicinae

JULY 20, 2019

[Epidemiology, comorbidity, etiology of depression and cardiovascular diseases]

PÉTER László, RIHMER Zoltán

[Psychiatric problems are a common comorbid condition in patients with cardiovascular disease. Depression is a well-known risk factor for the development of cardiovascular disease and mortality too. Psychiatric and cardiovascular diseases are prevalent public health problems in the western world. Depression is associated with endo­thelial dysfunction and increased platelet aggregation, which may explain the consequent cardiovascular complications. Specific affective temperaments may be associated with special cardiovascular diseases. Antidepressant therapy does not only reduce depression but also cardiac mortality.]

Lege Artis Medicinae

JULY 20, 2019

[Severe polymyositis associated with multiplex pulmonary abscesses]

SZABÓ Katalin, VINCZE Anett, NAGY-VINCZE Melinda, DANKÓ Katalin, GRIGER Zoltán

[INTRODUCTION – Idiopathic inflammatory myopathies are heterogeneous autoimmune diseases characterized by immune mediated inflammation of the skeletal muscles. CASE REPORT – A case of a 62-year-old male patient with severe proximal muscle weakness, elevated creatine kinase and swallowing difficulity is presented. Electromyography showed myogenic pattern, thus probable polymyositis was diagnosed. Radiological examination has confirmed bilateral multiplex lung lesions, which were caused by the possibility of tumor, tuberculosis, vasculitis and abscess as well. The condition of the patient deteriorated, nasogastric feeding, high dose steroid treatment was initiated, which reduced the patient's creatinine kinase values, but muscle strength was not changed. Based on the results of various investigations, the condition of the patient was finally confirmed by the development of myositis, resulting dysphagia, chronic aspiration, and multiplex lung abscess. Antibiotic therapy, steroid treatment was continued and finally intravenous immunoglobulin treatment was administered. The condition of the patient gradually improved, the swallowing dysfunction disappeared, and the lung abscesses were resolved. As a result of physiotherapy and rehabilitation treatment, the patient could walk again. CONCLUSIONS – Nasogastric feeding is recommended to prevent aspiration in the case of myositis-associated dysphagia. In case of steroid refractory therapy, the use of intravenous immunoglobulin may be effective. ]

Clinical Neuroscience

JULY 30, 2019

Relationship between Status Epilepticus Severity Score and etiology in adult NCSE patients

GENC Fatma, ERDAL Abidin, AKCA Gizem, KARACAY Ertan, GÖKSU Özaydın Eylem, KUTLU Gülnihal, GÖMCELI Bicer Yasemin

Purpose - Nonconvulsive status epilepticus (NCSE) is a heterogeneous, severe neurological disorder of different etiologies. In this study, the outcomes of NCSE episodes was assessed in a large series of adult patients. Our objective was to evaluate relationship between Status Epilepticus Severity Score (STESS) and etiology and the role of etiological factors on predicting the outcomes. Method - In this retrospective study, the medical records of 95 patients over 18 years of age who were diagnosed with NCSE between June 2011 and December 2015 were reviewed. Their treatment and follow-up for NCSE was performed at the Epilepsy Unit in Department of Neurology, Antalya Research and Training Hospital. Etiological factors thought to be responsible for NCSE episodes as well as the prognostic data were retrieved. The etiological factors were classified into three groups as those with a known history of epilepsy (Group 1), primary neurological disorder (Group 2), or systemic/unknown etiology (Group 3). STESS was retrospectively applied to patients. Results - There were 95 participants, 59 of whom were female. Group 1, Group 2, and Group 3 consisted of 11 (7 female), 54 (33 female), and 30 (19 female) patients, respectively. Of the 18 total deaths, 12 occurred in Group 2, and 6 in Group 3. The negative predictive value for a STESS score of ≤ 2 was 93.88% (+LR 2.05 95% CI: 1.44-2.9 and -LR 0.3 95% CI 0.10-0.84 ) in the overall study group. While the corresponding values for Group 1 (patients with epilepsy), Group 2 (patients with primary neurological disorder), and group 3 (patients with systemic or unknown etiology) were 100%, 92.59% (+LR 2.06 95%CI: 1.32-3.21 and -LR 0.28 95% CI 0.08-1.02 ) 83.33% (+LR 1.14 95%CI: 0.59-2.9 and -LR 0.80 95% CI 0.23-2.73). Conclusions - This study included the one of the largest patients series ever reported in whom STESS, a clinical scoring system proposed for use in patients with status epilepticus, has been implemented. Although STESS appeared to be quite useful for predicting a favorable outcome in NCSE patients with epilepsy and primary neurological disorders, its predictive value in patients with systemic or unknown etiology was lower. Further prospective studies including larger NCSE samples are warranted.

Clinical Neuroscience

JULY 30, 2019

Cerebral cavernous malformation type 1 with retinal blood vessel tortuosity and KRIT1 gene mutation

KALMÁR Tibor, MARÓTI Zoltán, VADVÁRI Árpád, HALMOSI Ágnes, KÁLOVITS Ferenc, KÁLMÁN Bernadette

Cerebral cavernous malformations (CCMs) represent a relatively rare and heterogeneous clinical entity with mutations identified in three genes. Both sporadic and familial forms have been reported. We present a young female patient with episodic paresthesia and headaches, but without acute neurological deficits. Her mother had a hemorrhaged cavernoma surgically removed 21 years ago. Cranial magnetic resonance imaging revealed multiple cavernous malformations in the size of a few millimeters and the ophthalmologic exam detected retinal blood vessel tortuosity in the proband. Targeted exome sequencing analysis identified a nonsense mutation in exon 16 of the KRIT1 gene, which resulted in a premature stop codon and a truncated protein underlying the abnormal development of cerebral and retinal blood vessels. This mutation with pathogenic significance has been reported before. Our case points to the importance of a thorough clinical and molecular work up despite the uncertain neurological complaints, since life style recommendations, imaging monitoring and genetic counseling may have major significance in the long term health of the patient.

Clinical Neuroscience

MAY 30, 2019

Population-based stroke screening days in the 12th district of Budapest in 2011 and 2016 - What have and what have not changed?

FOLYOVICH András, BOTOS Nóra, BALOGH Erzsébet, BAKOS Mária, HERTELENDY Anna, BÉRES-MOLNÁR Anna Katalin

Introduction - Population-based screening is an option to identify persons at high risk for stroke. However it is associated with rather high expenses, necessitating the selection of effective methods that take local characteristics into account. The 12th district of Budapest has a long tradition of population-based screening for frequent and preventable diseases. The Szent János Hospital hosts an annual stroke screening day. In the present study, previously published data from the 2011 screening were compared with those obtained in 2016, looking for changes and tendencies throughout the examined period. Subjects and methods - The screening day was conducted in a generally similar way in 2011 and 2016. Similarly to the previous event, the program was organized on a Saturday, the call for the event was spread by the local newspaper. The crew composition was the same. As regards the components of the screening (currently including general history taking, risk status assessment, blood pressure measurement, BMI assessment, cholesterol and blood glucose tests, carotid duplex ultrasonography, and ophthalmological examination), the only difference was the absence of cardiologic examination (it was conducted on an independent day). The anonymous data sheet was the same. Results - The number of participants in the 2016 event was 33, to provide more comfortable conditions. The female predominance was slightly less pronounced but was still present in 2016 (60.6% vs. 72.9%). The mean age became substantially higher (71.2 y vs. 62.9 y). The ratios of participants with higher level of education (97% vs. 94%) and those who are married were still remarkable. The most frequent risk factors were the same; however the ratio of participants with hypertension, ‘other heart disease’, and diabetes increased, whereas that of with hyperlipidemia and obesity decreased. The incidence of atrial fibrillation was unaltered. None of the participants in 2016 admitted smoking (previously this ratio was 20.8%) or drinking heavily. The findings of the carotid ultrasonography revealed a more favorable vascular status. Ophthalmological assessments (predominantly hypertensive alterations on fundoscopy) revealed that the pathological vs. physiological ratio switched to 1:2 from 2:1. The final evaluation of the screening program likewise demonstrated an improved overall state of health of the population. Conclusions - We observed a more favorable stroke risk status of the population in 2016. Whether it is indeed a tendency unknown at present. The role of the local media in calling for screening is still decisive, and the cohesive power of the family is important.

Clinical Neuroscience

MAY 30, 2019

Hyperhomocysteinemia in female migraineurs of childbearing ages

ALEMDAR Murat, SELEKLER Macit Hamit

Background and purpose - Migraine is a risk factor for ischemic stroke in women of childbearing ages. Previous researches revealed a higher prevalence of hyperhomocysteinemia in migraineurs. Possible differences on the frequencies of hyperhomocysteinemia between migraine with aura and migraine without aura could contribute the established variances in stroke risk between these migraine types. Therefore, we aimed to search if the frequency of hyperhomocysteinemia was different between these subtypes of migraine or not. Methods - We analyzed the findings of serum homocysteine levels in female migraineurs of 16-49 years old who admitted to our outpatient clinic. Results - Homocysteine level was elevated in 13.3% of study population. There were not any significant differences on median serum homocysteine levels between migraine with aura (8.0 mikromol/L) and without aura (8.5 mikromol/L). (p=0.426) The frequencies of hyperhomocysteinemia were also similar (9.1% versus 16.7%, respectively; p=0.373). Correlation analyses did not reveal any linear correlation between ages and homocysteine levels either in group of migraine with aura or in group of migraine without aura (p=0.417 and p=0.647, respectively). Similarly, any linear correlation between disease ages and homocysteine levels either in group of migraine with aura or in group of migraine without aura was not detected (p=0.359 and p=0.849, respectively). Conclusion - The median serum homocysteine levels and the frequencies of hyperhomocysteinemia are similar between migraine with aura and without aura in women of childbearing ages. Therefore, the variances on stroke risk ratios between these types of migraine are probably not originated from the differences of serum homocysteine status.

Clinical Neuroscience

MARCH 30, 2019

[Symptomatic trigeminal autonomic cephalalgia without headache]

RÓZSA Anikó, KOVÁCS Krisztina, GUBA Katalin, GÁCS Gyula

[We report the case of a 60-year-old man who exhibited trigeminal autonomic symptoms on his right side (numbness of the face, reddening of the eye, nasal congestion) occurring several times a day, for a maximum of 60 se­conds, without any pain. The complaints were similar to trigeminal autonomic cephalalgia, just without any headache. Our 60-year-old male patient underwent a craniocervical MRI as part of his neurological workup, which revealed lesions indicative of demyelination. Further testing was guided (ophthalmological examination, VEP, CSF test) by the presumptive diagnosis of multiple sclerosis. It is likely that in his case the cause of these trigeminal and autonomic paroxysms is MS. Here we present an overview of the few cases we found in the literature, although we did not find any similar case reports. Perhaps the most interesting among these is one in which the author describes a family: a 54-year-old female exhibiting the autonomic characteristics of an episodic cluster headache, only without actual headache, her son, who had typical episodic cluster headaches with autonomic symptoms, and the woman’s father, whose short-term periorbital headaches were present without autonomic symptoms. We had not previously encountered a case of trigeminal autonomic cephalalgia without headache in our practice, nor have we had an MS patient exhibiting similar neurologic symptoms. The significance of our case lies in its uniqueness. ]

Clinical Neuroscience

MARCH 30, 2019

Secretory meningioma with bone infiltration and orbital spreading

KÁLOVITS Ferenc, TAKÁTS Lajos, SOMOGYI Katalin, GARZULY Ferenc, TOMPA Márton, KÁLMÁN Bernadette

Secretory meningioma is a rare form of meningiomas which differentiates from the meningothelial subtype. It is characterized by significant peritumor edema and distinct immunohistochemical and molecular genetic profiles. We present a middle aged female patient with secretory meningioma infiltrating the orbital bone from the primary cranial base location and causing exophthalmos, features rarely described with this tumor. Surgical resection was challenging because of the associated brain swelling and rich vascularization of the tumor. Imaging and immunohistochemical studies revealed characteristic hallmarks of secretory meningioma. While histologically it was a benign tumor, due to the orbital bone and soft tissue infiltration, postoperative management of neurological sequelae was challenging. This case highlights distinctive clinical, imaging and histological features along with individual characteristics of a rare form of meningiomas.

Clinical Neuroscience

MARCH 30, 2019

Incidental intracranial lipomas: Assessment of 163 patients

TÜRK Okan, YALDIZ Can

Intracranial lipomas (ILs) are rare congenital lesions which consist 0.1-0.5% of intracranial lesions. They are usually asymptomatic. Our serial of 163 patients is the largest IL serial in literature. Files of the patients who were diagnosed with IL at outpatient clinic of neurosurgery between 2009 and 2018 were screened retrospectively. A total of 163 patients were detected to have been diagnosed with IL according to radiologic findings between 2009 and 2018. Of the patients, 96 were female and 67 were male. Intracranial lipomas are self-limited, slowly growing benign lesions which do not cause a mass effect. We believe that the present study would be a main source due to the currently available insufficient number of studies in literature.

Clinical Neuroscience

MARCH 30, 2019

[Sturge Weber type 3 presenting with occipital epileptic seizure: case report ]

SERİNDAĞ Cansu Helin, EREN Fulya, KARAHAN Gökçen Muazzez, GUL Gunay, SELCUK Hakan, KARA Batuhan, SOYSAL Aysun

[Sturge Weber syndrome is the third most common neurocutaneous syndrome after neurofibromatosis and tuberous sclerosis. Three distinct types were identified. Type 3 with leptomeningeal involvement alone is the rarest among other types. The reported case is a 21-years-old female patient without any known chronic disease. She admitted to the emergency department after visual symptoms and headache, followed by generalized tonic clonic seizure. EEG of the patient showed left occipital seizure activity. The contrast enhanced magnetic resonance imaging (MRI) showed left occipital leptomeningeal angioma. Digital substraction angiography (DSA) revealed minimal blushed contrast enhancement on late venous phase and lack of superficial cortical veins. Her focal seizures were under control with levatiracetam and lacosamide treatment. The reported case is unique because of the late onset presentation with focal seizure without mental retardation.]