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Clinical Neuroscience

JULY 30, 2020

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

OTTÓFFY Gábor, KOMÁROMY Hedvig

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

JULY 30, 2019

[Treatment of complex regional pain syndrome with amitriptyline]

KOMOLY Sámuel

[Introduction - Complex regional pain syndrome is a di­stressing neuropathic pain condition without known etiology and evidence based treatment. Case presentation - Here a posttraumatic severe case of complex regional pain syndrome is presented, successfully treated by amitriptyline monotherapy. Amitriptyline is one of the most effective evidence based treatments of peri­pheral diabetic neuropathic pain and other neuropathic pain syndromes. Discussion - Amitriptyline seems to be effective to decrease pain, autonomic and motor symptoms in chronic regional pain syndrome. Conclusion - Controlled trials may be warranted to test the effectiveness of amitriptyline in complex regional pain syndrome.]

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

LÁNG József, GANAU Mario, PRISCO Lara, BOZSIK Krisztina, BANCZEROWSKI Péter

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.

Lege Artis Medicinae

JUNE 20, 2014

[Posttraumatic growth among cancer patients]

TANYI Zsuzsanna

[In addition to studies of the negative effects of psychological trauma, recognition of benefit of highly distressing life experiences is increasingly becoming the focus in psycho-traumatology, usually under the name of posttraumatic growth (PTG). The recent review first demonstrates the domains of PTG and factors in the development of PTG. After this, the most frequent domains and the prevalence of PTG in the case of cancer patients are discussed. Finally, the paper tries to answer the question whether there is any relationship between PTG and physical health. ]

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

JANUARY 25, 2013

[The significance of high-resolution ultrasonography in the diagnosis of peripheral nerve disorders]

SCHEIDL Erika, JOSEF Böhm, FARBAKY Zsófia, DEBRECZENI Róbert, BERECZKI Dániel, ARÁNYI Zsuzsanna

[High resolution ultrasonography is an emerging technique for the investigation of peripheral nerves and is increasingly used worldwide in the diagnosis of peripheral nerve disorders, however, until now it is not widespread in Hungary. According to the literature this method is especially useful in entrapment neuropathies, traumatic peripheral nerve injuries, tumors of the peripheral nerves and sonographically guided interventions. Ultrasonography allows precise morphological analysis and quantitative measurements of the nerves providing useful complementary information to electrodiagnostic data. In entrapment neuropathies ultrasound shows nerve swelling mainly proximal to the sites of compression and a focal change of echotexture. On longitudinal scan, an abrupt caliber change and spindle-like swelling of the compressed nerve segment can be seen. Evaluation of the anatomical background and visualisation of the postoperative and posttraumatic changes provide useful information for planning of the therapy. Ultrasound may be of significant help in localizing the pathological nerve segment when it is at an electrophysiologically inaccessible site or when substantial secondary axonal loss precludes precise electrophysiological localization and it might even show pathological changes when nerve conduction studies are normal. Contrary to electrophysiological investigation ultrasonography might discover neurotmesis in the akute phase of traumatic nerve injuries indicating the necessity of surgical intervention. We provide a summary of the main indications and further application areas of this method.]

Clinical Neuroscience

NOVEMBER 30, 2011

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

SZIRMAI Imre

[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

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.]