Clinical Neuroscience

NMDA-receptor associated encephalitis in a woman with mature cystic ovarian teratoma

VANYA Melinda1, FÜVESI Judit2, KOVÁCS A. Zoltán3, GORGORAPTIS Nikos4, SALEK-HADDADI Afram4, KOVÁCS LÁSZLÓ5, BÁRTFAI György1

NOVEMBER 30, 2016

Clinical Neuroscience - 2016;69(11-12)

DOI: https://doi.org/10.18071/isz.69.0427

Introduction - N-methyl-D-aspartate receptor (NMDA-R) antibody-associated encephalitis has been reported in the international neurological literature to be associated with mature or immature ovarian teratomas (OTs). However, few cases of encephalitis were diagnosed in Hungary. In 2011 Hollody et al. described the first case of anti-NMDA receptor associated encephalitis in Hungary. Objective - Our aim was to present a case of NMDA-R antibody-mediated encephalitis in a woman with OT thereby providing information facilitating diagnosis of OT in women, who present with symptoms of encephalitis. Case - We report the case of a 25 year-old women, who developed NMDA-R -antibody associated autoimmune encephalitis and who displayed confusion, disorientation, a behavioural disturbance with agitation and features of paranoia and at least one reported generalized tonic clonic seizure and orofacial dyskinesia. Magnetic resonance imaging revealed a functional ovarian cyst measuring 3.3 cm, which was removed surgically and demonstrated histologically to be a mature cystic OT. The serum was positive for antibodies to NMDA receptors. Following intravenous immunoglobulin treatment, oophorectomy and a 5-day course of plasma exchange, followed by corticosteroid and azathioprine immunosuppressive therapy, the patient displayed a significant clinical improvement. Conclusion - Cystic teratomas are common benign ovarian lesions in women of reproductive age. Although the association of OTs and NMDA-R antibody-associated encephalitis has been described in the international neurological literature, this relationship needs to be considered from on the interdisciplinary aspect by the health care providers.

AFFILIATIONS

  1. Department of Obstetrics and Gynaecology, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
  2. Department of Neurology, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
  3. Department of Psychiatry, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
  4. Centre for Neurosciences, Department of Neurology, The Royal London Hospital, London, United Kingdom
  5. Department of Rheumatology, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary

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Clinical Neuroscience

Patient characteristics and the effects of intravenous immunoglobulin in patients with Guillain-Barre syndrome

GUZEY ARAS Yesim, TANIK Osman, DOGAN GÜNGEN Belma, KOTAN Dilcan

Purpose - The purpose of this study is to determine the diagnosis- and treatment-related characteristics in Guillain-Barré syndrome (GBS) and to evaluate the effects of early intravenous immunoglobulin (IVIg) treatment on disability, mortality and prognosis. Materials and methods - Adult patients who were diagnosed with GBS in our clinic between January 2000 and January 2014 were retrospectively scanned. While the patients undergoing IVIg treatment were included in the study, the other neuropathic diseases were excluded. Patients were divided into two groups based on the administration time of the IVIg treatment; Group 1 (<7 days) and Group 2 (≥ 7 days) Group 1 consisted of patients undergoing IVIg treatment within 7 days after presentation of symptoms and Group 2 consisted of patients undergoing IVIg treatment on and after 7th day following presentation of symptoms. The scores from Hughes Functional Grading Scale (HFGS) on admission and one month laterwere recorded in all patients in order to evaluate the disability and prognosis in terms of demographic and clinical laboratory characteristics. Results - In this study, 49 GBS patients were included (31 patients in Group 1 and 18 patients in Group 2). Demyelinating form of GBS was determined in 22 (44.8%) patients.). While there was no difference between both groups (p: 0.288, p: 0.762, p: 0.693 respectively) in terms of intensive care and rehabilitation requirement and progression, only 2 patients in Group 1 died. While HFGS mean score on admission in all the patient groups was 3.27±0.974, their HFGS mean score at month 1 was 2.53±1.226. There was no difference between the groups in terms of HFGS mean scores on admission and at month 1. Within each groups, there was a significant improvement between initial (on admission) HFGS scores and HFGS scores acquired at month 1. Conclusion - In this study, demyelinating form was more frequent than axonal form. A total of 2 g/kg dose of IVIg treatment administered for 5 days as a standard in GBS patients ensured a significant improvement on both disability and early and late administration and early administration of the treatment does not lead to any difference in intensive care unit and rehabilitation requirements.

Clinical Neuroscience

Vitreous humor diffusion measurements from diffusionweighted imaging in idiopathic intracranial hypertension

CAGLI Bekir, TUNCEL Alpaslan Sedat, YILMAZ Erdem, TEKATAS Aslan, ERMIS Veli

Background - Idiopathic intracranial hypertension is a disease with uncertain etiology. It is not caused by an intracranial mass lesion or hydrocephalus and is characterized by abnormal elevation of intracranial pressure and normal composition of the cerebrospinal fluid. The orbita and intracranial area are closely related anatomically. Elevated intracranial pressure can be transmitted to the orbita through the cerebrospinal fluid around the optic nerve sheath changes at the vitreous humor on diffusion-weighted imaging have not been systemically studied in idiopathic intracranial hypertension. Purpose - The purpose of this study was to investigate diffusion changes in the vitreous humor in patients with intracranial hypertension. Methods - In this retrospective study, 25 patients with papilledema and who had been definitively diagnosed with idiopathic intracranial hypertension and 20 control participants were evaluated. Control subjects and patients were scanned with a 1.5 Tesla magnetic resonance imaging. Apparent diffusion coefficient maps were obtained from diffusion-weighted imaging with a b value of 1000 s/mm2 and apparent diffusion coefficient values were automatically calculated. These images were obtained by a radiologist who was blinded to the details of the study for center of each vitreous humor and the body of lateral ventricle. The mean apparent diffusion coefficient values of each vitreous humor and the body of the lateral ventricle were calculated for each group (control group and patients) and quantitative comparisons were performed. Results - There were no statistically significant differences in mean apparent diffusion coefficient values of the right vitreous humor, left vitreous humor and the body of the lateral ventricle between the patients with idiopathic intracranial hypertension and the control group (p=0.766, p=0.864, p=0.576, respectively). Discussion - Vitreous humor is a closed system and has no direct relationship with the cerebrospinal fluid or cerebral tissue and although morphological changes occur in the orbital structures, including the optic disk and optic nerve in idiopathic intracranial hypertension, the indirect effects of these changes on the vitreous humor may be too subtle to measure. Conclusion - We did not find a significant difference in the mean apparent diffusion coefficient value of the vitreous humor between the patients with idiopathic intracranial hypertension and the control group. However, future studies will be necessary to determine if changes in the vitreous humor can be used to diagnose intracranial hypertension.

Clinical Neuroscience

Unilateral thalamic infarction causing downward gaze palsy in a patient with uncorrected tetralogy of fallot: a case report

ANNUS Ádám, BENCSIK Krisztina, JÁRDÁNHÁZY Tamás, VÉCSEI László, KLIVÉNYI Péter

Introduction - Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD). Adults with surgically uncorrected forms of this condition are extremely rare, since operation is recommended in childhood to prevent cyanosis. Cyanotic CHD increases the risk of thromboembolic events. An endothelial dysfunction caused by chronic hypoxia and shear stress due to rheological alterations with a platelet dysfunction appear to be the explanation behind this finding. Paramedian thalamic infarction causing vertical gaze palsy without midbrain involvement is an infrequent finding. We report here a rare case of a patient with untreated TOF, who suffered a left-sided unilateral thalamic infarction presenting as downward gaze palsy and diplopia. Case presentation - A 44-year-old women complained of sudden onset diplopia and vertigo. Neurological examination revealed a downward gaze palsy with other symptoms related to a vertebrobasilar territory circulatory disturbance. The MRI scan revealed an acute infarction, 8 mm in diameter in the left medial thalamic region without midbrain involvement. Discussion - Adults with uncorrected forms of TOF are extremely uncommon, and descriptions of stroke in these patients are therefore rarities. We set out to give a concise survey of the literature regarding TOF patients with stroke. Conclusion - We present a rare case of unilateral thalamic infarction causing downward gaze palsy in an adult patient with uncorrected TOF. Cyanotic CHD is regarded as one of the risk factors of stroke. Besides other pathologic conditions, ischaemic stroke at an early age should raise the suspicion of a cardioembolic origin and, in rare cases, might result from cyanotic CHD.

Clinical Neuroscience

[Recommendation for treatment options in advanced Parkinson's disease]

ASCHERMANN Zsuzsanna, DIBÓ György, KLIVÉNYI Péter, KOVÁCS Norbert, KOVÁCS Tibor, TAKÁTS Annamária, GERTRÚD Tamás, VARANNAI Lajos

[The treatment of advanced Parkinson’s disease is challenging for both physicians and caregivers. The device-aided therapies need expertise and dedicated hospital centers. In this summary we have concluded the available data and recommendation for the treatment options in advanced Parkinson’s disease and adopt them to the daily care in Hungary. ]

Clinical Neuroscience

[The role of zonisamide in the treatment of women with epilepsy]

JUHOS Vera

[The antiepileptic drugs can effect fertility, development of gynecological diseases and occurence of sexual problems. They can cause a number of “cosmetic” problem and also influence the selection of safe contraceptive method. Many antiepileptic drugs can cause congenital malformations or affect the new-born child’s psychomotor and cognitive development, therefore during pregnancy should be treated with extreme caution in women with epilepsy. Most types of epilepsies accompany the patient through their whole life. Women spend almost the third of their lives after menopause and - due to the formation of associated diseases as well - this period is also special. According to the 2013 recommendation of International League Epilepsy (ILAE), zonisamide is one of the first-line antiepileptic drugs in focal epilepsy. In my review I discuss women’s epilepsy in the viewpoint of the application of zonisamid. ]

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Clinical Neuroscience

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Clinical Neuroscience

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Clinical Neuroscience

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