Clinical Neuroscience

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

USLU Ilgen Ferda1, ELIF Gökçal1, GÜRSOY Esra Azize1, KOLUKISA Mehmet1, YILDIZ Babacan Gulsen1

MAY 30, 2020

Clinical Neuroscience - 2020;73(05-06)

DOI: https://doi.org/10.18071/isz.73.0177

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.

AFFILIATIONS

  1. Bezmialem Vakif University, Department of Neurology, Istanbul, Turkey

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.

Clinical Neuroscience

The etiology and age-related properties of patients with delirium in coronary intensive care unit and its effects on inhospital and follow up prognosis

ALTAY Servet, GÜRDOGAN Muhammet, KAYA Caglar, KARDAS Fatih, ZEYBEY Utku, CAKIR Burcu, EBIK Mustafa, DEMIR Melik

Delirium is a syndrome frequently encountered in intensive care and associated with a poor prognosis. Intensive care delirium is mostly based on general and palliative intensive care data in the literature. In this study, we aimed to investigate the incidence of delirium in coronary intensive care unit (CICU), related factors, its relationship with inhospital and follow up prognosis, incidence of age-related delirium and its effect on outcomes. This study was conducted with patients hospitalized in CICU of a tertiary university hospital between 01 August 2017 and 01 August 2018. Files of all patients were examined in details, and demographic, clinic and laboratory parameters were recorded. Patients confirmed with psychiatry consultation were included in the groups of patients who developed delirium. Patients were divided into groups with and without delirium developed, and baseline features, inhospital and follow up prognoses were investigated. In addition, patients were divided into four groups as <65 years old, 65-75 yo, 75-84 yo and> 85 yo, and the incidence of delirium, related factors and prognoses were compared among these groups. A total of 1108 patients (mean age: 64.4 ± 13.9 years; 66% men) who were followed in the intensive care unit with variable indications were included in the study. Of all patients 11.1% developed delirium in the CICU. Patients who developed delirium were older, comorbidities were more frequent, and these patients showed increased inflammation findings, and significant increase in inhospital mortality compared to those who did not develop delirium (p<0.05). At median 9-month follow up period, rehospitalization, reinfarction, cognitive dysfunction, initiation of psychiatric therapy and mortality were significantly higher in the delirium group (p<0.05). When patients who developed delirium were divided into four groups by age and analyzed, incidence of delirium and mortality rate in delirium group were significantly increased by age (p<0.05). Development of delirium in coronary intensive care unit is associated with increased inhospital and follow up morbidity and mortality. Delirium is more commonly seen in geriatric patients and those with comorbidity, and is associated with a poorer prognosis. High-risk patients should be more carefully monitored for the risk of delirium.

Clinical Neuroscience

Autonomic nervous system may be affected after carpal tunnel syndrome surgery: A possible mechanism for persistence of symptoms after surgery

ONDER Burcu, KELES Yavuz Betul

After carpal tunnel surgery, some patients report complaints such as edema, pain, and numbness. Purpose – The aim of this study was to evaluate autonomic nervous system function in patients with a history of carpal tunnel surgery using sympathetic skin response (SSR). Thirty three patients (55 ±10 years old) with a history of unilateral operation for carpal tunnel syndrome were included in the study. The SSR test was performed for both hands. Both upper extremities median and ulnar nerve conduction results were recorded. A reduced amplitude (p=0.006) and delayed latency (p<0.0001) were detected in the SSR test on the operated side compared to contralateral side. There was no correlation between SSR and carpal tunnel syndrome severity. Although complex regional pain syndrome does not develop in patients after carpal tunnel surgery, some of the complaints may be caused by effects on the autonomic nervous system.

Clinical Neuroscience

Simultaneous subdural, subarachnoideal and intracerebral haemorrhage after rupture of a peripheral middle cerebral artery aneurysm

BÉRES-MOLNÁR Anna Katalin, FOLYOVICH András, SZLOBODA Péter, SZENDREY-KISS Zsolt, BERECZKI Dániel, BAKOS Mária, VÁRALLYAY György, SZABÓ Huba, NYÁRI István

The cause of intracerebral, subarachnoid and subdural haemorrhage is different, and the simultaneous appearance in the same case is extremely rare. We describe the case of a patient with a ruptured aneurysm on the distal segment of the middle cerebral artery, with a concomitant subdural and intracerebral haemorrhage, and a subsequent secondary brainstem (Duret) haemorrhage. The 59-year-old woman had hypertension and diabetes in her medical history. She experienced anomic aphasia and left-sided headache starting one day before admission. She had no trauma. A few minutes after admission she suddenly became comatose, her breathing became superficial. Non-contrast CT revealed left sided fronto-parietal subdural and subarachnoid and intracerebral haemorrhage, and bleeding was also observed in the right pontine region. The patient had leucocytosis and hyperglycemia but normal hemostasis. After the subdural haemorrhage had been evacuated, the patient was transferred to intensive care unit. Sepsis developed. Echocardiography did not detect endocarditis. Neurological status, vigilance gradually improved. The rehabilitation process was interrupted by epileptic status. Control CT and CT angiography proved an aneurysm in the peripheral part of the left middle cerebral artery, which was later clipped. Histolo­gical examination excluded mycotic etiology of the aneu­rysm and “normal aneurysm wall” was described. The brain stem haemorrhage – Duret bleeding – was presumably caused by a sudden increase in intracranial pressure due to the supratentorial space occupying process and consequential trans-tentorial herniation. This case is a rarity, as the patient not only survived, but lives an active life with some residual symptoms.

Clinical Neuroscience

[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

All articles in the issue

Related contents

Clinical Oncology

[Obesity and cancer]

VALTINYI Dorottya

[The role of obesity in the development of cancer is well-known from ages. However, these days we witness the explosion-like increase of obesity, globally, but mainly in the economically advanced population, and, which is even more alarming, among youngsters. The prognosis of the obesity-related cancer is rather poor, therefore, the prevention, including the screening, have outstanding importance. Unfortunately, the participation of the obes persons, especially obes women, in these programs is very low. The diagnostics and therapies should consider the special features of obesity, which are related to the magnitude, distribution, composition of fatty tissue connected to the changes in pharmacokinetics. Moreover, the problems might be complicated with obesity-associated non-tumorous severe diseases (e.g. cardiovascular, diabetes type 2).This review covers different aspects of obesity-cancer relationships, with an emphasis on everyday oncology.]

Clinical Oncology

[Novelties in the management of Hodgkin lymphoma]

ILLÉS Árpád, MOLNÁR Zsuzsa, MILTÉNYI Zsófia

[Hodgkin lymphoma is a lymphoproliferativ disease, it is about 12-18% of all lymphomas. It has typical morphologic, clinical and therapeutic features, which can distinguish from other lymphoma types. Due to risk- and PET/CT adapted treatment Hodgkin lymphoma is a curable lymphoma with an 80-90% long-term survival, however, refracter- and relapsed patients’ therapy is a great challange. Cure rate can increase due to the development of the diagnostic and treatment modalities, but the use of standard recommendation is necessary. The aim of this review is to show new WHO 2016 lymphoma classifi cation, role of the new diagnostic options, especially 18FDG-PET/CT, Lugano classifi cation and fi rst-line and salvage therapeutic possibilities and to introduce the immunotherapy, like brentuximab vedotin and PD1 inhibitors. Certain points of hemopoietic stem cell transplantation will be also covered.]

Clinical Oncology

[Clinical role of multigenic prognostic tests in breast cancer therapy]

GYŐRFFY Balázs

[Current clinical practice for breast cancer originates in “evidence based medicine”. In this, each tumor receives a therapy optimal for a given patient population - which might not be optimal for each individual patient. Multigenic tests determining expression of a set of genes can provide additional support in this decision process. Two such tests (MammaPrint and Prosigna) have already received FDA clearance. A number of additional test are commercially available (IHC4, Oncotype DX, EndoPredict, BCI). A common property of these assays is their utility in estrogen receptor positive early breast cancer. The main clinical problem answered by them is the necessity of adjuvant chemotherapy. To date, no reliable algorithm has been identifi ed capable to pinpoint the most effective chemotherapy combination for a given patient. Furthermore, there is no trustworthy test for triple negative breast cancer. The assays utilize different technologies (immunohistochemistry, gene chips, RT-PCR) and a discrepant list of genes - these result in discordance of the predictions for the individual patient. Despite these shortcomings, multigenic tests quickly gained foothold in breast cancer therapy decision process. Their utility is supported by the cost reduction for the health care providers by lowering the number of patients eligible for chemotherapy.]

Lege Artis Medicinae

[Depression in medical practice - the possibilities of diagnosis and treatment]

OSVÁTH Péter

[Nowadays, there is an increasing incidence of depressive mood disorders, so we have more and more depressed patients in everyday clinical practice. Unfortunately, in many cases, this is not recognized and thus the treatment of depressed patients is not adequately addressed. Untreated depression not only increases the burden of the patient and his environment, but leads to serious and dangerous psychic and somatic complications. However, with modern and complex psychopharmacological, psychotherapeutic and sociotherapeutic methods, depressive mood disorders can be effectively treated. In this paper I review the etiologic background, the characteristics of depressive mood disorders and the most important steps of making diagnosis, as well as the state of the art therapeutic options. Rapid recognition and effective treatment of depressive symptoms has important therapeutic and preventive significance, so today, besides psychiatrists, there is an increasing role for general practitioners and other specialists (internists, neurologists, etc.).]

Lege Artis Medicinae

[Physiological-pathological muscle atrophy in elderly - interventions potencially inhibiting this progressive process ]

SZÉKÁCS Béla, MOLNÁR Andrea, BESENYEI Attila, MARTONY Zsuzsanna

[In old and very old age, one of the most prevalent signs of aged body’s decline is the progressive loss of muscle mass and function. First itself the physiological aging process can be dominant in the complex causative background but later it is usually intertwined with pathological mechanisms. The importance of muscle system is extremely high in the physiological regulation of various vital life processes The paper also points out the far-reaching consequences of sarcopenia syndrome that leads to general weakness, falls, traumas, acceleration of co-morbidities, rapidly declining self independence, ultimately frailty syndrome, and death. The initial body mass index has been recently replaced by a more adequate, more complex diagnostic approachment of sarcopenia that evaluates both muscle mass/strength and physical performance. Prevention or breaking the process of sarcopenia needs complex intervention which includes special fast protein rich diet with leucin and vitamin D combined with frequent physical exercise. ]