Clinical Neuroscience

Watershed infarction in hypereosinophilic syndrome: a diagnostic dilemma in FIP1L1-PDGFR alpha-associated myeloid neoplasm

IMELDA Marton1, PÓSFAI Éva1, ANNUS János Kristóf2, BORBÉNYI Zita1, NEMES Attila1, VÉCSEI László2,3, VÖRÖS Erika4,5

MAY 30, 2015

Clinical Neuroscience - 2015;68(05-06)

DOI: https://doi.org/10.18071/isz.68.0212

Introduction - The FIP1L1-PDGFR alpha-positive, hypereosinophilic syndrome (HES) is a new category of hematological entities. Various clinical symptoms may occur, with no specific characteristics in either the clinical picture or the neuroimaging findings, and this may give rise to a diagnostic dilemma. A report on a long follow-up period (10 years) in a case of HES that presented with neuropsychiatric symptoms appears to be unique. Besides the complexity of the diagnostic process, the successful treatment is discussed. Case report - The HES was diagnosed in a male patient at the age of 33 years, with involvement of the central nervous system and the myocardium. After the onset of the clinical signs, the MRI indicated bilateral cerebral and cerebellar cortico-subcortical lesions involving the watershed areas, mainly in the parieto-occipital regions. High-dose intravenous steroid (methylprednisolone 500 mg/day) alleviated the neurological symptoms within a few weeks, and the administration of imatinib (200 mg/day) resulted in an impressive regression of the hypereosinophilia and splenomegaly within 6 weeks. During the follow-up, the patient has continued to receive imatinib. The molecular remission has persisted, no new complaints have developed and the condition of the patient has remained stable. Conclusion - The timely recognition of the HES and identification of the disease subtype which led to the administration of imatinib may be the key to successful treatment. The long stable follow-up period gives rise to a new dilemma in the treatment of the HES in these special cases: for how long should a patient receive a tyrosine kinase inhibitor, and may the treatment be suspended?

AFFILIATIONS

  1. 2nd Department of Medicine and Cardiology Centre, Albert Szent-Györgyi Clinical Centre, Medical Faculty, University of Szeged
  2. Department of Neurology, Albert Szent-Györgyi Clinical Centre, Medical Faculty, University of Szeged
  3. Neuroscience Research Group of the Hungarian Academy of Sciences and University of Szeged
  4. Department of Radiology, Albert Szent-Györgyi Clinical Centre, Medical Faculty, University of Szeged
  5. Euromedic Diagnostics Szeged

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Clinical Neuroscience

[Zonisamide: one of the first-line antiepileptic drugs in focal epilepsy ]

JANSZKY József, HORVÁTH Réka, KOMOLY Sámuel

[Chronic administration of antiepileptic drugs without history of unprovoked epileptic seizures are not recommended for epilepsy prophylaxis. Conversely, if the patient suffered the first unprovoked seizure, then the presence of epileptiform discharges on the EEG, focal neurological signs, and the presence of epileptogenic lesion on the MRI are risk factors for a second seizure (such as for the development of epilepsy). Without these risk factors, the chance of a second seizure is about 25-30%, while the presence of these risk factors (for example signs of previous stroke, neurotrauma, or encephalitis on the MRI) can predict >70% seizure recurrence. Thus the International League Against Epilepsy (ILAE) re-defined the term ’epilepsy’ which can be diagnosed even after the first seizure, if the risk of seizure recurrence is high. According to this definition, we can start antiepileptic drug therapy after a single unprovoked seizure. There are four antiepileptic drugs which has the highest evidence (level „A”) as first-line initial monotherapy for treating newly diagnosed epilepsy. These are: carbamazepine, phenytoin, levetiracetam, and zonisamide (ZNS). The present review focuses on the ZNS. Beacuse ZNS can be administrated once a day, it is an optimal drug for maintaining patient’s compliance and for those patients who have a high risk for developing a non-compliance (for example teenagers and young adults). Due to the low interaction potential, ZNS treatment is safe and effective in treating epilepsy of elderly people. ZNS is an ideal drug in epilepsy accompanied by obesity, because ZNS has a weight loss effect, especially in obese patients.]

Clinical Neuroscience

Chronic cerebrospinal venous insufficiency - disease or misdiagnosis?

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Background and purpose - Former studies reported internal jugular vein stenosis in patients with multiple sclerosis. We aimed to evaluate if these venous stenoses were real and cerebral venous outflow of patients with multiple sclerosis differed from that of normal controls. Methods - 20 controls were prospectively investigated by angiography and duplex ultrasound. Seven patients with multiple sclerosis underwent angiography in other centers; we reviewed these registrations and performed venous ultrasound examinations. Results - Angiography displayed >50% stenosis of internal jugular vein in 19 controls (69±17% on the right and 73±13% on the left side) and <50% stenosis in 1 control (43.5% and 44.6%). All 7 patients had at least one-sided stenosis. The mean degree of stenosis was 63±16% on the right and 67±13% on the left side. There was no significant difference in the degree of stenosis between patients and controls. However, these “stenoses” disappeared if the contrast agent was injected at a catheter position below the orifice of the subclavian vein during venography. The venous flow volume was also similar between groups: 479.7±214.1 and 509.8±212.0 ml/min (right and left side) in the patients and 461.3±224.3 and 513.6±352.2 ml/min in the control group; p=0.85 and 0.98 (right and left). Color and power duplex imaging also revealed normal blood flow of the internal jugular vein in all patients and controls. Conclusion - The cerebral venous status of patients with multiple sclerosis and controls were similar. The angiographic “stenoses” were virtual, caused by the contrast dilution effect of the non-contrast blood stream of the subclavian vein.

Clinical Neuroscience

[Acoustic CR®-neuromodulation - first experiencies in Hungary with novel patented method in therapy of chronic subjectiv tinnitus]

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[Objective - Acoustic CR®-neuromodulation is a novel patented method for the therapy of chronic subjective tinnitus and has been tested in Hungary, as one of the first European countries introducing this procedure. It can be used for the treatment of monaural or binaural tonal tinnitus. Suitability of patients for this therapy was assessed by the help of an appropriate set of criteria. Aim of our study was to analyze 6-month therapy and related measurement data of patients first treated with this method in Hungary and evaluate the results. Method - 27 outpatients (20 males, seven females) with a minimum of 6-month long history of subjective tinnitus were assessed (four detected on the right side, six on the left side, 17 on both sides) who were treated for six months by Acoustic CR®-neuromodulation. On 44 treated ears (21 right, 23 left), changes of tinnitus frequency and loudness were measured and analysed, using Visual Analogue Scale (VAS) loudness/annoyance/pitch scores and Tinnitus Handicap Inventory tests, which were performed at defined intervals during the treatment period. Results - During this 6-month treatment period, significant decrease was detected in tinnitus frequency and loudness by tinnitometry (irrespective of the affected side), and an improvement in VAS annoyance/pitch scores and THI test results. VAS loudness did not show any significant changes. Conclusion - Acoustic CR®-neuromodulation therapy may be a useful treatment of subjective chronic tinnitus, but its efficacy should be proved in controlled clinical trials.]

Clinical Neuroscience

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[Wide variety of the movements (from the physiologic body position changes to different pathologic events) can be seen during the sleep period. The most important types of these movements are the sleep related events (from the parasomnias to the restless leg related movements), the movement disorders and the epilepsy related events. To differentiate between these events is required special skill, which is based on appropriate characterisation of the events (for example timing, repetition, pattern), the polysomnographic and video-EEG examination and validated questionnaires (FLEP scale). The appropriate differential diagnostics and therapy must be based on the knowledge of the relationship of the sleep architecture and movement events. This review would like to provide guideline for the understanding and recognizing the nature of the sleep related movements.]

Clinical Neuroscience

[Validation of the Hungarian Unified Dyskinesia Rating Scale]

HORVÁTH Krisztina, ASCHERMANN Zsuzsanna, ÁCS Péter, BOSNYÁK Edit, DELI Gabriella, PÁL Endre, KÉSMÁRKI Ildikó, HORVÁTH Réka, TAKÁCS Katalin, BALÁZS Éva, KOMOLY Sámuel, BOKOR Magdolna, RIGÓ Eszter, LAJTOS Júl

[Background - The Unified Dyskinesia Rating Scale (UDysRS) was published in 2008. It was designed to be simultaneous valid, reliable and sensitive to therapeutic changes. The Movement Disorder Society organizing team developed guidelines for the development of official non- English translations consisting of four steps: translation/back-translation, cognitive pretesting, large field testing, and clinimetric analysis. The aim of this paper was to introduce the new UDysRS and its validation process into Hungarian. Methods - After the translation of UDysRS into Hungarian and back-translated into English, it was reviewed by the UDysRS translation administration team. Subsequent cognitive pretesting was conducted with ten patients. For the large field testing phase, the Hungarian official working draft version of UDysRS was tested with 256 patients with Parkinson’s disease having dyskinesia. Confirmatory factor analyses (CFA) determined whether the factor structure for the valid Spanish UDysRS could be confirmed in data collected using the Hungarian Official Draft Version. To become an official translation, the Comparative Fit Index (CFI) had to be ≥0.90 compared to the Spanish-language version. Results - For the Hungarian UDysRS the CFI was 0.98. Conclusion - The overall factor structure of the Hungarian version was consistent with that of the Spanish version based on the high CFIs for the UDysRS in the CFA; therefore, this version was designated as the Official Hungarian Version Of The UDysRS.]

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Hyperhomocysteinemia in female migraineurs of childbearing ages

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Clinical Neuroscience

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Clinical Neuroscience

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[Evidence based medicine requires objective methods for the assessment of status of the patients. The method described by the authors makes it possible to assess motoric impairment of patients in an objective way. It is based on three-dimensional motion analysis. Authors present the case history of two patients with spastic hemiparesis due to central nervous system damage. Changes in motoric impairment were followed by three-dimensional motion analysis. This method can be adapted for the assessment of motor impairment arising from other reasons as well.]

Clinical Neuroscience

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[Background and purpose - The classical risk factors did not explain all the possible ethiology of cerebral stroke. Genetic polymorphisms responsible for thrombophilia were implicated recently as risk factors of stroke. In this geneticoepidemiological study the author’s aim was to analyse the tendency of genetic polymorphisms to cluster in a cohort of young and elderly stroke patients and in healthy subjects in Hungary. Methods - 253 patients with stroke were compared with 173 healthy blood donors on the basis of genetic polymorphisms of platelet GP IIb/IIIa receptor (33 LeuPro), prothrombin gene G20210A, Factor V Leiden mutation, ACE I/D, methylenetetrahydrofolate reductase (MTHFR) and β fibrinogen gene G455A. These data were acquired using PCR. Questionnaires were used to investigate the family history and to determine the risk factor profile. The subtypes of stroke were analysed in a stroke cohort grouped according to different polymorphisms. Results - An increased frequency of GP IIIa heterozygousity was found as compared to a West-European stroke cohort (31% versus 19%). The prothrombin gene variant (2.9% European and 4.8% in Hungary) was also found to increase in frequency. In young stroke patients (age <50) compared with control subjects the odds ratios were higher: in prothrombin gene (OR: 4.9), in Leiden mutation (OR: 1.67), in fibrinogen gene (OR: 1.64) and in MTHFR(+/+) (OR: 1.58). Clustering of two polymorphisms could only be detected in young patients. These clustering polymorphisms were GP IIb/IIIa with prothrombin G20210A variant (OR: 6.74, 95% CI 1.1-18.2) and prothrombin gene variant with MTHFR (OR: 5.3, CI95 1.2-8.3). Conclusion - Selected and clustered genetic polymorphisms of haemostatic factors could be responsible for the high stroke morbidity in Central Europe. The presence and clustering tendency of these factors have been described in young stroke victims.]

Hypertension and nephrology

[Blood pressure management for stroke prevention and in the acute stroke. The new guideline of European Society of Hypertension (ESH, 2018), European Society of Cardiology and Hungarian Society of Hypertension (HSH, 2018)]

JENEI Zoltán

[Hypertension is the leading modifiable risk factor for stroke. Its prevalence amongst stroke patient is about 60-70% and the benefit of blood pressure (BP) lowering therapy on stroke risk reduction is well established. However the optimal BP targets for preventing stroke and reducing stroke consequences have been controversial. The new European (ESC/ESH) and Hungarian (HSH) hypertension guideline published in 2018 highlighted the primary and secondary prevention of stroke and the BP management in the acute stroke care as well. According results from ACCORD, SPRINT, HOPE-3, and other metaanalysis the systolic blood pressure (SBP) lowering < 120 mmHg has not favourable effect, thus in hypertensive patients < 65 years the SBP should be lowered to a BP range of 120-129 mmHg. In older patients ≥ 65 years the SBP should be targeted to a BP range of 130-139 mmHg (IA). In patients with acute intracerebral haemorrhage careful acute BP lowering with iv. therapy, to <180 mmHg should be considered only in case of SBP ≥ 220 mmHg (IIaB). In patients with acute ischaemic stroke who are eligible for iv. thrombolysis, BP should be carefully lowered and maintained to < 180/105 mmHg for at least the first 24 h after thrombolysis (IIaB). If the patient is not eli gible for lysis and BP ≤ 220/110 mmHg, routine BP lowering drug therapy is not recommended inside 48-72 h (IA). In patients with markedly elevated BP > 220/110 mmHg who do not receive fibrinolysis, drug therapy may be considered, based on clinical judgement, to reduce BP by 15% during the first 24 h after the stroke onset (IIbC). After 72 h of acute stroke in case of hypertensive patients < 65 years the SBP should be lowered to a BP range of 120-129 mmHg (IIaB). In older patients ≥ 65 years the SBP should be targeted to a BP range of 130-139 mmHg (IA). If BP < 140/90 mmHg after stroke, the BP lowering should be considered (IIbA). It is recommended to initiate an antihypertensive treatment with combination, preferably single pill combination of renin-angiotensin system blockers plus a calcium channel blocker and/or a thiazide like diuretics (IA). Lowering SBP < 120 mmHg is not recommended due to advers events regardless of age and type of stroke either in primary or secondary stroke prevention.]