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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 20, 2015

[A rare complication of a rare disease; stroke due to relapsing polychondritis]


[Relapsing polychondritis (RP) is an episodic and progressive inflammatory disease of cartilaginous structures. Its diagnosis is based primarily on clinical features such as laboratory parameters, biopsy. Neurological complications occur in 3% of the cases and are classified as an important cause of death. The cranial nerve disorders are most common but hemiplegia, ataxia, myelitis, polyneuritis, seizures, confusion, hallucination and headache can also happen. The aetiology of central nervous system involvement is still unknown. Moreover stroke has rarely reported in these patients. The diagnosis of stroke is challenging because of its rarity among these patients. Perhaps vasculitis is the common underlying mechanism. Also meningitis and encephalitis can occur during the course of RP. A 44 year-old woman was admitted with uncontemplated left hemiparesis, redness, swelling, and tenderness of the metacarpophalangeal and interphalangeal joints of the right hand and the cartilaginous portion. White blood cell count, C-reactive protein and the erythrocyte sedimentation rate were elevated. Vasculitis biomarkers were normal in our patient. Carotid and vertebral artery doppler ultrasonography, cranial and cervical MR Angiography were normal. Echocardiography showed a mild mitral valve prolapse and regurgitation. Our patient had the history of auricular polychondritis but she had not been diagnosed. Hemiparesis was her first neurological manifestation that led her to doctors for diagnosis. Our patient fulfilled the criteria of RP so no biopsy was needed. She was treated with oral prednisolone (80 mg/day) and aspirin (300 mg/day) and now she is on 10 mg prednisolone and 150 mg azathioprine. Two months later her physical and neurological symptoms returned to normal.]

Clinical Neuroscience

MARCH 30, 2020

CANOMAD syndrome with respiratory failure

SALAMON András, DÉZSI Lívia, RADICS Bence, VARGA Tímea Edina, HORTOBÁGYI Tibor, TÖMÖSVÁRI Adrienn, VÉCSEI László, KLIVÉNYI Péter, RAJDA Cecília

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Clinical Neuroscience

SEPTEMBER 30, 2019

[The Expanded Disability Status Scale scoring in patients with multiple sclerosis]


[Gait disturbance is a major symptom in patients with multiple sclerosis. The Expanded Disability Status Scale (EDSS) was first used in clinical trials of multiple sclerosis for the assessment of disability, however it has become more and more widely used in clinical practice as well. Nowadays its use is essential in application of the new diagnostic criteria, the new clinical form classification and in monitoring the efficacy of therapies. EDSS is based on a standardised neurological examination, but focuses on those symptoms that are frequent in multiple sclerosis. Based on the examination it assesses seven functional systems: visual, brainstem, pyramidal, cerebellar, sensory, bowel-bladder and cerebral functions. EDSS scores can be determined based on the scores given in the functional systems and on testing the walking distance. In newer versions the “Ambulation score” has been added. This chapter clarifies the scores based on the maximal walking distance and the need for a walking aid to walk this distance. The Neurostatus/EDSS training method improves the reproducibility of the standardised neurological examination that forms the basis of the EDSS scoring. Of the tests assessing walking, the Timed-25 Foot Walk Test and the self-administered 12-Item Multiple Sclerosis Walking Scale are suitable for routine evaluation of walking performance. An increase of more than 20% in the Timed-25 Foot Walk may be considered a significant change in gait. ]

Clinical Neuroscience

MARCH 30, 2019

[Rehabilitation possibilities and results after neurosurgical intervention of brain tumors ]

DÉNES Zoltán, TARJÁNYI Szilvia, NAGY Helga

[Objectives - Authors examined the rehabilitation possi­bi­lities, necessities, and results of patients after operation with brain tumor, and report their experiences. Method - Retrospective, descriptive study at the Brain Injury Rehabilitation Unit, in National Institute for Medical Rehabilitation. Patients - Patients were admitted consecutively after rehabilitation consultation, from different hospitals, following surgical intervention of brain tumors, between 01 January 2001 and 31 December 2016. Patients participated in a postacute inpatient rehabilitation program, in multidisciplinary team-work, leaded by Physical and Rehabilitation Medicine specialist included the following activities: rehabilitation nursing, physical, occupational, speech, psychological and neuropsychological therapy. Results - At the rehabilitation unit, in the sixteen-year period 84 patients were treated after operation with brain tumor. Patients arrived at the unit after an average of 41 days to the time of the surgical intervention (range: 10-139 days), and the mean length of rehabilitation stay was 49 days (range: 2-193 days). The mean age of patients was 58 years (20-91), who were 34 men and 50 women. The main symptoms were hemiparesis (64), cognitive problems (26), dysphagia (23), aphasia (16), ataxia (15), tetraparesis (5), and paraparesis (1). The mean Barthel Index at the time of admission was 35 points, whereas this value was 75 points at discharge. After the inpatient rehabilitation, 73 patients improved functionally, the status of 9 patients did not show clinically relevant changes, and 2 patients deteriorated. During the rehabilitation 10 patients required urgent interhospital transfer to brain surgery units, 9 patients continued their oncological treatment, two patients continued rehabilitation treatment at another rehabilitation unit, and after rehabilitation 73 patients were discharged to their homes. Conclusions - Inpatient rehabilitation treatment could be necessary after operation of patients with brain tumor especially when functional disorders (disability) are present. Consultation is obligatory among the neurosurgeon, rehabilitation physician and the patient to set realistic rehabilitation goals and determine place and method of rehabilitation treatment, but even at malignancies cooperation with oncological specialist also needed. Authors’ experience shows benefits of multidisciplinary rehabilitation for patients after brain tumor surgery. ]

Clinical Neuroscience

JANUARY 30, 2019

Evaluation of neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio in essential tremor

TAK Zeynal Abidin Ali, SENGUL Yildizhan

Introduction - Although essential tremor (ET) is the most common cause of tremor, the pathology and underlying mechanisms have not fully understood yet. In addition to kinetic tremor, patients may present several types of tremor, gait ataxia, hearing deficits and eye movement abnormalities. Non-motor symptoms and signs have also added to definition of ET. There is significant evidence indicating the neurodegenerative nature of the disease. New studies indicate that inflammation may have a place in the etiology. The neutrophil-to lymphocyte ratio (NLR) and the platelet-to lymphocyte ratio (PLR) have recently begun to be used as a marker of systemic inflammation. Our study aims at finding a clue for systemic inflammation in ET. Methods - 67 patients with ET and 40 healthy controls were recruited for the study. The total white blood cells (WBC), absolute neutrophil count, lymphocyte count and platelet count were retrieved. The NLR was calculated by dividing the neutrophil count by the lymphocyte count and the PLR was calculated by dividing the platelet count by the lymphocyte count. Results - Patient and control groups were similar in terms of age and gender. The mean age of patient group was 25.29 ± 8.24 years and that of control group was 26.77 ± 6.73 years. The NLRs were 1.85 ± 0.58 in the patient group and 1.96 ± 0.53 in the control group. For the patient group and the control group the PLRs were 103.52 ±32.80 and 91.26 ± 31.57 respectively. There were no statistically significant differences between the participants for both NLR and PLR. Conclusion - The pathophysiological mechanism for essential tremor (ET) remains unclear. However, there is an increasing amount of research being conducted on the subject. Discussions about ET’s definition as a neurodegenerative disease are ongoing. Although previous studies showed that neuroinflammation could be a part of etiology of disease, this study has failed to demonstrate systemic inflammation in ET.

Clinical Neuroscience

NOVEMBER 30, 2017

A case with reversible neurotoxicity induced by metronidazole

EREN Fulya, ALDAN Ali Mehmet, DOGAN Burcu Vasfiye, GUL Gunay, SELCUK Hatem Hakan, SOYSAL Aysun

Background - Metronidazole is a synthetic antibiotic, which has been commonly used for protozoal and anaerobic infections. It rarely causes dose - and duration - unrelated reversible neurotoxicity. It can induce hyperintense T2/FLAIR MRI lesions in several areas of the brain. Although the clinical status is catastrophic, it is completely reversible after discontinuation of the medicine. Case report - 36-year-old female patient who had recent brain abscess history was under treatment of metronidazole for 40 days. She admitted to Emergency Department with newly onset myalgia, nausea, vomiting, blurred vision and cerebellar signs. She had nystagmus in all directions of gaze, ataxia and incompetence in tandem walk. Bilateral hyperintense lesions in splenium of corpus callosum, mesencephalon and dentate nuclei were detected in T2/FLAIR MRI. Although lumbar puncture analysis was normal, her lesions were thought to be related to activation of the brain abscess and metronidazole was started to be given by intravenous way instead of oral. As lesions got bigger and clinical status got worse, metronidazole was stopped. After discontinuation of metronidazole, we detected a dramatic improvement in patient’s clinical status and MRI lesions reduced. Conclusion - Although metronidazole induced neurotoxicity is a very rare complication of the treatment, clinicians should be aware of this entity because its adverse effects are completely reversible after discontinuation of the treatment.

Clinical Neuroscience

MAY 30, 2015

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

IMELDA Marton, PÓSFAI Éva, ANNUS János Kristóf, BORBÉNYI Zita, NEMES Attila, VÉCSEI László, VÖRÖS Erika

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?

Clinical Neuroscience

JANUARY 30, 2015

[The first identified Central-Eastern European patient with genetically confirmed dentatorubral-pallidoluysian atrophy]


[Aims - Dentatorubral-pallidoluysian atrophy (DRPLA) is an inherited neurodegenerative disorder characterized by a trinucleotide repeat expansion. The disease mainly occurs amongst the Japanese and is extremely rare in the European population. The characteristic clinical symptoms are cerebellar ataxia, dementia, choreoathetoid movements, epileptic seizures and myoclonus. The aim of this study is to present the first genetically confirmed Hungarian case of DRPLA. Case report - The middle-aged female patient developed the characteristic clinical symptoms except myoclonus over her late thirties with positive family history. The major finding in the skull magnetic resonance imaging was the atrophy of infratentorial brain structures with the consequential dilation of related cerebrospinal fluid spaces. A detailed neuropsychological examination was also performed and it revealed moderate cognitive dysfunctions, mild depression and anxiety. As underlying conditions, Huntington’s disease and common spinocerebellar ataxia forms all came into consideration, but all the result of the respective genetic tests were negative. However, the test for mutation in the ATN1 gene revealed pathological heterozygous CAG repeat expansion. Conclusion - This case study serves as the first description of genetically confirmed DRPLA in the Central-Eastern region of Europe, the clinical features of which seems to be very similar to the previously reported cases.]

Clinical Neuroscience

NOVEMBER 28, 2014

[Symptomatic subependymomas of the ventricles. Review of twenty consecutive cases]


[Background and purpose - Intraventricular subependymomas are rare benign tumors, which are often misdiagnosed as ependymomas. To review the clinicopathological features of subependymomas. Patient selection and methods - Retrospective clinical analysis of intraventricular subependymomas and systematic review of histological slides operated on at our center between 1985 and 2005. Results - Twenty subependymomas presented at the median age of 50 years (range 19-77). Two (10%) were found in the third, three (15%) in the forth, and 15 in the lateral ventricles. There was male preponderance (12 vs. 8). Ataxia (n=13) and papilledema (n=7) were the most common clinical presentations. Fifteen patients underwent gross total resection, and five had subtotal resection. None of the cases showed mitotic figures, vascular endothelial proliferation or necrosis. Cell proliferation marker MIB-1 activity (percentage of positive staining tumor cells) ranged from 0 to 1.4% (mean 0.3). Two cases were treated with preoperative radiation therapy (50 Gy) before the CT era, three other patients received postoperative radiation therapy for tumors originally diagnosed histologically as low grade ependymomas. Three patients (15%) died of surgical complication between one and three months postoperatively, and three patients died of unrelated causes in eight, 26 and 110 months. Fifteen patients were alive without evidence of tumor recurrence at a median follow-up time of 10 years. Conclusion - Subependymomas are low-grade lesions and patients do well without adjuvant radiotherapy. Small samples from more cellular areas may be confused with low grade ependymomas, and unnecessary radiotherapy may follow. Recurrences, rapid growth rates should warrant histological review, as hypocellular areas of ependymomas may also be a source of confusion.]