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Clinical Neuroscience

JULY 30, 2020

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report


Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.

Clinical Neuroscience

MAY 30, 2020

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

Clinical Neuroscience

NOVEMBER 30, 2016

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

JULY 30, 2016

Syndrome of trephined-underestimated and poorly understood complication after decompressive craniectomy


Decompressive craniectomy (DC) is still a matter of debate, with a numerous complications as expansion of haemorrhagic contusions, external cerebral herniation, subdural hygromas, post-traumatic hydrocephalus (HC). The often overlooked “syndrome of the trephined” (ST) as a delayed complication of DC also known as sinking skin flap sy initially described in 1939.ST is characterised by the neurological changes associated with alteration of the pressure/volume relationship between intracranial pressure (ICP), volume of cerebrospinal fluid (CSF), blood, and brain tissue in patients with large bone defects. This review aims at elucidating the mechanisms responsible for the development of ST, and providing useful tips and red-flag signs for healthcare professionals involved with care of post DC patients. Symptoms identified on time could help to develop appropriate treatment strategies for this suddenly deteriorating, but possible reversible condition. Although the treatment strategy is straightforward, calling for a prompt cranioplasty, the correction of HC through CSF diversion devices might require a lengthy optimisation period. Continuous changes in the setting of the shunting systems or spinal tap might lead to dangerous swinging of the midline structures causing further neurological deterioration. Thus, finding the right balance in terms of clinical management often represents a significant challenge.

Clinical Neuroscience

JULY 30, 2013

[Shunt insufficiency due to knot formation in the peritoneal catheter]

FEKETE Gábor, NAGY Andrea, PATAKI István, BOGNÁR László, NOVÁK László

[The authors report a rare case of the peripheral obstruction of a ventriculoperitoneal shunt. Premature baby was operated on hydrocephalus due to germinal matrix bleeding. After two months of implantation of venticuloperitoneal shunt peripheral insufficiency of the system was emerged. During the shunt revision extensive knot formation became visible. We simply cut the catheter above the knot and the working shunt was replaced into the abdominal cavity. The postoperative course was uneventful and the baby was free of complaints for more than one year. The pathomechanism of knot formation is not clear thus the discovery of the problem during the operation is an unexpected event. In our opinion tight knot cannot be spontaneously formed intraabdominally. Loose knots can be developed and can reduce the capacity of liquor flow. We think that the knot tightens during pulling out. Longer peritoneal catheters can precipitate multiple looping and/or axial torquations and increase the peripheral resistance of the shunt. In such cases when the pulling out is challenged conversion to laparotomy is suggested.]

Clinical Neuroscience

NOVEMBER 30, 2011

[Vascular or “lower body parkinsonism”. Rise and fall of one diagnosis]


[The “arteriosclerotic parkinsonism”, which is called vascular parkinsonism (VP), was first described by Critchley1. The broad based slow gait, reduced stride lenght, start hesitation, freezing and paratonia was mentioned as “lower body parkinsonism” (LBP) which can be associated by slow speech, dysexecutive syndrome, and hand tremor of predominantly postural character. In VP the DAT-scan proved normal dopamine content of the striatum in contrast with Parkinson’s disease (PD). Additionally, Lewy bodies of brainstem type were not found in VP. Probability of VP increases if central type pathologic gait is prominent; the hands are slightly involved, the MRI indicates transparent periventricular white substance and/or brain atrophy. In some cases differentiation of gait apraxia and parkinsonism could be challenging. There is no rigor of the lower limbs at rest in neither of them, the disturbance of movement is evoked by the gait itself. Three subtypes of “gait ignition failure” has been recently described: (1) ignition apraxia, (2) equilibrium apraxia and (3) mixed gait apraxia. The primary progressive freesing gait was considered as a Parkinson-plus syndrome. Freesing occurs more frequently in diseases with pakinsonism than in PD. The grade of ventricle dilatation and the frontal leukoaraiosis was similar in LBP and gait apraxia. In cases of normal pressure hydrocephalus the impaired gait may mimic PD. Pathologic gait in VP can be explained by the lesions of the senso-motor association pathways in dorsal paramedian white substance within the vulnerable borderzone region. These may be colocalized with the representation of the lower extremities in the posterior third of the supplementer motor area. Rektor2 proposed to change the name of LBP to “cerebrovascular gait disorder”. Notwithstandig central type gait disorder develops also in many degenerative diseases other than cerebro-vascular origin. The neuronal net controling the regulation of movement is widespread, therefore several cortical and subcortical lesions could elicit large variations of pathologic gait, ie.: ataxia, apraxia, ignition failure, akinesis etc. In conclusion: most of the central gait disorders regarding the pathology and their appearance can not be called “parkinsonism”; these are much closer related to the localization of lesions rather than to the diagnostic categories.]

Clinical Neuroscience

DECEMBER 20, 2008

[Pediatric intraventricular tumors]

MARKIA Balázs, GYORSOK Zsuzsanna, KORDÁS Mariann, BOGNÁR László

[Pediatric intraventricular tumors present a well circumscribed group from surgical point of view. These tumors growing in the ventricular system cause hydrocephalus in most of the cases, the presenting symptoms are the signs of raised intracranial pressure. The mass lesion may remain silent for a long period, especially in infancy due to compensatory mechanisms, and the tumor might reach extreme size making the surgery a real challenge. This group has very specific postoperative problems resulting from the disturbance of CSF circulation. In this study we present the retrospective analysis of 55 patient operated for intraventricular tumor in the National Institute of Neurosurgery between 1991 and 2006. Data were analysed regarding histological type, presenting symptoms, type of surgical approach, radicalitiy of the resection and postoperative complications. In addition to our own results brief presentation of the specific histological groups is given based on the available literature.]

Clinical Neuroscience

JANUARY 22, 2008

[Occlusive hydrocephalus caused by a fourth ventricle arachnoid cyst (in English language)]

SZŰCS Anna, VÁRADY Péter, PESTALITY Péter, FABÓ Dániel, LALIT Narula, KENÉZ József

[The case history of a woman with occlusive hydrocephalus caused by a fourth ventricle cyst is presented. She had slowly progressive complaints and symptoms - concentration and memory disturbances, low-tempered mood, then slight dizziness, loss of appetite and progressive headache - transitorily misinterpreted for signs of depression. She had been treated by psychotherapy and antidepressants for months. Since she did not improve she was referred to a psychiatric hospital. The rapidly progressing neurological syndrome with worsening headache, gait disturbance and vomiting was finally identified and it turned out to be caused by a fourth ventricle CSF blockage of unknown aetiology. An acute neurosurgical intervention was indicated. It revealed a huge fourth ventricle cyst, undetectable on MRI, occupying the whole ventricle. The resection of its walls resulted in complete recovery. We conclude that since unspecific mental complaints and symptoms suggesting depression may be misleading, their organic origin has to be excluded.]

Clinical Neuroscience

APRIL 20, 2002

[New methods in stroke intensive therapy: hemicraniectomy in patients with complete middle cerebral artery infarction and treatment of intracerebral and intraventricular hemorrhage with urokinase]

KAKUK Ilona, MAJOR Ottó, GUBUCZ István, NYÁRY István, NAGY Zoltán

[Life-threatening, complete middle cerebral artery infarction occurs in up to 10% of all stroke patients. The “malignant media occlusion” is an infarction occupying more than 50% of middle cerebral artery territory. The malignant, space-occupying supratentorial ischemic stroke is characterised by a mortality rate of up to 80%. Several reports indicate, that hemicraniectomy in this situation can be life-saving. Hemicraniectomy increases cerebral perfusion pressure and optimises retrograde perfusion via the leptomeningeal collateral vessels. A case of a patient is presented, having progressive neurological deterioration due to massive cerebral infarctions. The patient rehabilitation was successful. Decompressive surgery is life saving and can also give acceptable functional recovery. Hemorrhagic stroke is due to stroke in 15% of cases and in 10%, it is “spontaneous” intracerebral hematoma. The intracerebral and intraventricular hemorrhage represents one of the most devastating types of stroke associated with high morbidity and mortality. The 30-day mortality rate is 35% to 50% and most survivors are left with a neurological disability. The value of surgical therapy is debatable. The aspiration and urokinase therapy of the hematoma of intracerebral hemorrhage could improve final neurological outcome. Spontaneous, nontraumatic intraventricular hemorrhage frequently carries a grave prognosis. A large part of morbidity after intraventricular hemorrhage is related to intracranial hypertension from hydrocephalus. One patient presented had intracerebral hemorrhage and another had intraventricular hemorrhage treated with urokinase. Rapid and extensive reduction in the amount of intracerebral and intraventricular blood occurred. Urokinase lysis is safe and can be a potentially beneficial intervention in intracerebral and intraventricular hemorrhage. By performing decompressive craniectomy, the neurologists of stroke departments and intensive care units with the neurosurgeons will have to play major role in the management of stroke patients.]

Lege Artis Medicinae

JULY 20, 2006


KAZY Zoltán, PUHÓ Erzsébet, CZEIZEL Endre

[INTRODUCTION - The primary aim of the study was to investigate the possibility of a teratogenic effect of vaginal metronidazole treatment during pregnancy. MATERIALS AND METHODS - Various congenital abnormalities and all (1-3) their matched controls were analysed in the population-based database of the Hungarian Case-Control Surveillance of Congenital Abnormalities between 1980 and 1996. The study group comprised 38 151 pregnant women who had newborns without any congenital abnormalities (control group) and 22 843 pregnant women who had newborns or fetuses with congenital abnormalities. Main outcome measures were analysed in 22 congenital abnormalities groups. The use of metronidazole during the second and third trimesters being common, the secondary objective of the study was to analyse the effect of vaginal metronidazole treatment (500 mg of Klion) on gestational age and birth weight in the control group without congenital abnormalities. RESULTS - The prevalence of vaginal metronidazole treatment during pregnancy was 1.7% in the case group (n=388) and 1.5% in the control group (n=570) (odds ratio (OR), 1.1, 95% confidence interval (CI): 1.0-1.3). Comparisons of cases and their matched controls showed an association between vaginal metronidazole treatment during the second and third months of gestation and congenital hydrocephaly (adjusted OR with 95% CI, 10.7, 1.1-104.5), but this was only based on five cases. The analysis of metronidazole treatment reported by the physicians did not confirm this association. After metronidazole use the mean gestational age was slightly shorter (by 0.1 week) and the mean birth weight was also slightly lower (by 40g) in the treated group. CONCLUSION - This finding should only be regarded as an indication for a possible association between vaginal treatment with metronidazole during pregnancy and congenital hydrocephalus. The results showed that vaginal metronidazole treatment alone was not able to prevent a possible vaginal infection that is associated with preterm birth, and that the prevalence of low birth weight was higher among treated women. The protective effect of metronidazole on preterm birth and low birth weight was not confirmed.]