Clinical Neuroscience

[Multiple sclerosis coexisting with spinal cord ependymoma: a case report]

EYLEM Degirmenci

SEPTEMBER 30, 2012

Clinical Neuroscience - 2012;65(09-10)

[Background - In this paper, we report a coexistence of multiple sclerosis and an intradural spinal cord tumor. Case report - A 34-year-old woman who had a history of relapsing-remitting multiple sclerosis for the last 15-years presented with acute sensory loss and spasticity in her left lower limb and her spinal magnetic resonance imaging study revealed an intradural spinal cord tumor in the lumbar spine, further diagnosed as ependymoma. Conclusion - We call attention to this rare association of MS and a spinal cord tumor, emphasizing the need for investigation of new symptoms during the evolution of MS.]

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Clinical Neuroscience

[Health status and costs of ambulatory patients with multiple sclerosis in Hungary]

PÉNTEK Márta, GULÁCSI László, RÓZSA Csilla, SIMÓ Magdolna, ILJICSOV Anna, KOMOLY Sámuel, BRODSZKY Valentin

[Background and purpose - Data on disease burden of multiple sclerosis from Eastern-Central Europe are very limited. Our aim was to explore the quality of life, resource utilisation and costs of ambulating patients with multiple sclerosis in Hungary. Methods - Cross-sectional questionnaire survey was performed in two outpatient neurology centres in 2009. Clinical history, health care utilisation in the past 12 months were surveyed, the Expanded Disability Status Scale and the EQ-5D questionnaires were applied. Cost calculation was conducted from the societal perspective. Results - Sixty-eight patients (female 70.6%) aged 38.0 (SD 9.1) with disease duration of 7.8 (SD 6.7) years were involved. Fifty-five (80.9%) had relapsing-remitting form and 52 (76.5%) were taking immunomodulatory drug. The average scores were: Expanded Disability Status Scale 1.9 (SD 1.7), EQ-5D 0.67 (SD 0.28). Mean total cost amounted to 10 902 Euros/patient/year (direct medical 67%, direct nonmedical 13%, indirect costs 20%). Drugs, disability pension and informal care were the highest cost items. Costs of mild (Expanded Disability Status Scale 0-3.5) and moderate (Expanded Disability Status Scale 4.0-6.5) disease were 9 218 and 17 634 Euros/patient/year respectively (p<0.01), that is lower than results from Western European countries. Conclusion - Our study provides current inputs for policy making and contributes to understanding variation of costof- illness of multiple sclerosis in Europe.]

Clinical Neuroscience

[Burning sensation in oral cavity - burning mouth syndrome in everyday medical practice]

GERLINGER Imre

[Burning mouth syndrome (BMS) refers to chronic orofacial pain, unaccompanied by mucosal lesions or other evident clinical signs. It is observed principally in middle-aged patients and postmenopausal women. BMS is characterized by an intense burning or stinging sensation, preferably on the tongue or in other areas of the oral mucosa. It can be accompanied by other sensory disorders such as dry mouth or taste alterations. Probably of multifactorial origin, and often idiopathic, with a still unknown etiopathogenesis in which local, systemic and psychological factors are implicated. Currently there is no consensus on the diagnosis and classification of BMS. This study reviews the literature on this syndrome, with special reference to the etiological factors that may be involved and the clinical aspects they present. The diagnostic criteria that should be followed and the therapeutic management are discussed with reference to the most recent studies.]

Clinical Neuroscience

[Administration of preemptive analgesia by diclofenac to prevent acute postcraniotomy headache]

SIMON Éva, BÁNK Judit, GÁL Judit, SIRÓ Péter, NOVÁK László, FÜLESDI Béla, MOLNÁR Csilla

[Purpose - Postcraniotomy headache (PCH) is a frequent perioperative complication in neuroanesthesia. The aim of the present work was to assess the incidence of PCH and to test the efficacy and safety of preoperatively administered diclofenac. Methods - Patients undergoing craniotomies for intracranial tumor resections were enrolled. In the case group 100 mg diclofenac p.o. one hour prior to surgery was used as a preemptive analgesic along with infiltration of the surgical site with a combination of lidocaine and epinephrine. In controls only surgical site infiltration was used. VAS scores were assessed preoperatively, on the day of surgery (DoS), on the 1st and 5th postoperative days. Results - We have found that PCH of any severity is between 50-90% during the first five days after surgery. The number of cases characterized as “no pain” significantly decreased in the early postoperative period, but remained in both groups still higher on the 5th postoperative day than observed preoperatively. In both groups, the number of headaches characterized as mild pain remained relatively stable and substantial increases in case numbers were observed in moderate and severe headaches, showing a declining tendency over time in the postoperative period. A significant effect of diclofenac pretreatment was observed compared to controls on DoS (χ2: 10.429, p<0.015), on the 1st (χ2: 8.75, p<0.032) and 5th postoperative days (χ2: 14.3, p<0.002). Conclusions - The relatively low incidence of severe PCH on day five in the diclofenac group may indicate that preoperatively administered diclofenac effectively reduces postcraniotomy headache. A randomized study is encouraged to test this hypothesis.]

Clinical Neuroscience

[A prospective study evaluating the clinical characteristics of cluster headache]

ERTSEY Csaba, VESZA Zsófia, BANGÓ Márta, VARGA Tímea, NAGYIDEI Diána, MANHALTER Nóra, BOZSIK György

[Introduction - Although cluster headache (CH) is one of the most severe human pain syndromes, its symptoms and therapeutic possibilities may be suboptimally recognised in current medical practice in Hungary. Aim - To present the clinical characteristics of CH based on a prospective study of patients attending the Headache Service of the Department of Neurology, Semmelweis University. Methods - We collected information about the symptoms, diagnosis and previous treatment of CH patients by filling in a 108-item questionnaire during outpatient visits. Results - In the 5-year period between 2004 and 2008 we obtained data from 78 CH patients (57 males and 21 females; mean age: 44.6±14.6 years). The male:female ratio did not change in subgroups based on disease onset (calendar years). Ninety-three percent considered CH the most severe pain state of their life. The pain was strictly unilateral, affecting the territory of the 1st trigeminal division in all patients. The attacks were accompanied by signs of ipsilateral cranial parasympathetic activation (lactimation 83%, conjunctival injection 67%, rhinorrhea 56%, nasal congestion 43%); less frequently, signs of sympathetic dysfunction (ptosis 48%, miosis 7%) were also present. Two patients had attacks showing the typical localisation, severity and time course of CH attacks, but not accompanied by autonomic phenomena. A considerable part of the patients also observed symptoms that are usually ascribed to migraine (nausea 41%, vomiting 18%, photophobia 68%, phonophobia 58%). This may have been instrumental in the fact that, regardless of the characteristic clinical symptoms, the diagnosis of CH took 10 years on average. At the time of their examination 63% of patients were not using adequate abortive medications and 59% did not have an adequate prophylactic measure. Discussion - Cluster headache is characterised by attacks of devastating pain that warrant an early diagnosis and adequate treatment. Our study underlines that information about the diagnosis and therapy of CH should be emphasized on occasions of neurology specialty training and continuing medical education.]

Clinical Neuroscience

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Clinical Neuroscience

[Health status and costs of ambulatory patients with multiple sclerosis in Hungary]

PÉNTEK Márta, GULÁCSI László, RÓZSA Csilla, SIMÓ Magdolna, ILJICSOV Anna, KOMOLY Sámuel, BRODSZKY Valentin

[Background and purpose - Data on disease burden of multiple sclerosis from Eastern-Central Europe are very limited. Our aim was to explore the quality of life, resource utilisation and costs of ambulating patients with multiple sclerosis in Hungary. Methods - Cross-sectional questionnaire survey was performed in two outpatient neurology centres in 2009. Clinical history, health care utilisation in the past 12 months were surveyed, the Expanded Disability Status Scale and the EQ-5D questionnaires were applied. Cost calculation was conducted from the societal perspective. Results - Sixty-eight patients (female 70.6%) aged 38.0 (SD 9.1) with disease duration of 7.8 (SD 6.7) years were involved. Fifty-five (80.9%) had relapsing-remitting form and 52 (76.5%) were taking immunomodulatory drug. The average scores were: Expanded Disability Status Scale 1.9 (SD 1.7), EQ-5D 0.67 (SD 0.28). Mean total cost amounted to 10 902 Euros/patient/year (direct medical 67%, direct nonmedical 13%, indirect costs 20%). Drugs, disability pension and informal care were the highest cost items. Costs of mild (Expanded Disability Status Scale 0-3.5) and moderate (Expanded Disability Status Scale 4.0-6.5) disease were 9 218 and 17 634 Euros/patient/year respectively (p<0.01), that is lower than results from Western European countries. Conclusion - Our study provides current inputs for policy making and contributes to understanding variation of costof- illness of multiple sclerosis in Europe.]

Clinical Neuroscience

[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

Clinical Neuroscience

Matrix metalloproteinases and their tissue inhibitors in relapsing remitting multiple sclerosis: Possible markers and treatment agents

SANLI Arzu, OZTURK Musa, SOYSAL Aysun, DOVENTAS Yasemin, BASOGLU Fulya, GOZUBATIK-CELIK R. Gokcen, BAYBAS Sevim

Matrix metalloproteinases (MMPs), which are synthesized by many cell groups and responsible for the destruction of matrix proteins, and endogen tissue inhibitors of MMPs (TIMPs) have a role in the pathogenesis of Multiple Sclerosis (MS) by affecting the blood-brain barrier. We aimed to investigate the role of MMPs and TIMPs in the immunopathogenesis and in the course of multiple sclerosis (MS). We enrolled 25 relapsing remitting MS patients, who had a definite MS diagnosis according to McDonald criteria and 25 healthy subjects similar for age and gender as control group. MMP-9- and TIMP-1 levels were measured twice in patient group (one time during an attack and one in remission) and once in healthy subjects. MMP-9- and TIMP-levels of patients during attack and remission period and MMP-9/TIMP-1 ratio were found significantly higher than in the control subjects. In patient group MMP-9 and TIMP-1 levels and MMP-9/TIMP-1 ratio during attacks were not significantly different than during remission period. However, when subdivided according to their number of attacks, patients with 2 attacks had significantly higher levels during attack period comparing to remission period (p<0.05); in case of patients with more than 2 attacks did not have a statistically significant difference in attack and remission periods. Matrix metalloproteinases are important actors in MS immunopathogenesis, particularly in the early period and inhibitor agents for these enzymes can be used as a treatment option.

Clinical Neuroscience

[MR imaging of acute disseminated encephalomyelitis and multiple sclerosis in children. A review (in English language)]

PATAY Zoltán

[Inflammatory diseases of the central nervous system (CNS) are relatively rare in children, but their relevance to public health is considerable due to frequent and significant long term morbidity and even mortality. As in adults, acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) and their variants are the most common entities in this group of pathologies in the pediatric patient population. Recent efforts have focused on establishing standardized diagnostic criteria schemes to facilitate the diagnosis and differential diagnosis of these diseases, however especially with multiple sclerosis those have not been fully validated yet for disease occurring in children. In recent decades the role of MRI has been constantly increasing in the diagnostic work-up of suspected inflammatory diseases of the CNS as well as in the follow-up of patients with confirmed disease. Currently, MRI is the first-line diagnostic imaging modality in ADEM and MS and is fully integrated in the most widely used diagnostic criteria schemes, but it has a key role in clinical therapeutic research trials as well. This paper provides an update on the current concepts and strategies of MRI in inflammatory diseases of the CNS, as well as a review of the imaging semiology of the various disease entities and variants with emphasis on clinical and imaging particularities relevant to the pediatric patient population.]

Clinical Neuroscience

[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. ]