The Importance of Long-term Partner Observation in Cognitive Evaluation: A Very Early Creutzfeldt-Jakob Disease in a Patient with Mild Cognitive Impairment.

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a fatal degenerative brain disease characterized by rapidly progressive dementia. Sporadic CJD (sCJD) is the best-known and most common subtype. Because the disease is uncommon and has highly diverse presenting symptoms, early diagnosis is challenging. We herein report a case of probable sCJD diagnosed at a very early stage. CASE PRESENTATION: A 61-year-old female patient had mild attention and memory problems for a few months that were noticed by her husband but did not bother her and did not affect her daily life activities. The first brain magnetic resonance imaging (MRI) at another hospital was normal, lacking diffusion-weighted imaging (DWI). Although the newly taken brain MRI without DWI was normal, the patient's husband brought his patient to our outpatient clinic because he continued to think that there was a difference in his wife's attention and memory. A neurological examination of the patient revealed almost normal findings. The neuropsychiatric evaluation of the patient was consistent with mild cognitive impairment. The patient's electroencephalography taken upon admission had no characteristic findings for CJD but showed generalized epileptiform activity. Therefore, the patient was hospitalized, and a second brain MRI, including DWI sequences, was performed. DWI displayed bilateral asymmetrical typical patterns of restricted diffusion. Cerebrospinal fluid 14-3-3 was positive, and total-tau was highly elevated. She had a diagnosis of probable sCJD at an early stage. Later, the patient developed progressive dementia, ataxia, seizures, and extrapyramidal symptoms, followed by mutism, and died. CONCLUSION: Although there is no cure for CJD today, early diagnosis is essential, mainly because of its potential infectivity and for future planning. Diagnosing sCJD in its early stages is difficult. However, taking into account the observations of not only the patient's history but also their longterm partners in cognitive evaluations will be helpful in making an early and accurate diagnosis.

Optical coherence tomography and neurodegeneration in epilepsy.

PURPOSE: To compare optical coherence tomography measurements; central macular thickness, ganglion cell complex, and retinal nerve fiber layer thickness in patients with epilepsy versus healthy controls. METHODS: We evaluated 28 eyes of 28 patients with epilepsy and 34 eyes of 34 healthy subjects. Central macular thickness, ganglion cell complex, and retinal nerve fiber layer thickness measurements were performed by spectral-domain optical coherence tomography. RESULTS: Superior and superotemporal quadrant ganglion cell complex, average, and superior quadrant retinal nerve fiber layer thickness measurements were significantly lower in epilepsy group compared to healthy control subjects. Central macular thickness was significantly lower in polytherapy group compared to monotherapy group. Ganglion cell complex and retinal nerve fiber layer thickness measurements were not significantly different between polytherapy and monotherapy groups. CONCLUSION: The present study shows that epileptic patients taking antiepileptic drugs have reduced ganglion cell complex and retinal nerve fiber layer thickness compared to healthy controls. This can be related to the epileptic process in the brain. Optical coherence tomography may be a useful tool for showing the neurodegeneration in patients with epilepsy.

Evaluation of serum thiol-disulphide homeostasis parameters as oxidative stress markers in epilepsy patients.

The present study focuses on the investigation of the dynamic thiol-disulphide homeostasis in patients with epilepsy and understanding the effects of antiepileptic drugs on thiol levels. A total of 148 participants, 75 of whom had epilepsy and 73 were healthy volunteers, were included in the study. Total thiol and native thiol levels of all epilepsy patients and healthy volunteers were measured. Disulphide level, disulphide/native thiol, disulphide/total thiol and native/total thiol ratios were calculated from these values. The results were compared between epilepsy patients and healthy volunteers. A statistically significant difference was found between native thiol level, total thiol level, disulphide level, disulfide/total thiol, disulphide/native thiol and native/total thiol ratios between patients with epilepsy and healthy volunteers (p = 0.002, p = 0.035, p < 0.001, p < 0.001, p < 0.001, p < 0.001, respectively). The drugs used had a significant effect on disulphide, disulphide/total thiol, native/total thiol levels (p values 0.004, 0.009, 0.009, respectively). Decreased levels of serum native, total thiol and increased disulfide levels as parameters of oxidative stress may be considered as parameters to explain the pathogenesis or consequences of epilepsy.

Vestibular evoked myogenic potential responses in Parkinson's disease.

BACKGROUND AND PURPOSE: - Our objectives were to determine the differences in the vestibular evoked myogenic potential (VEMP) responses in patients diagnosed with early staged idiopathic Parkinson's disease (PD) compared to the normal population and evaluate the vestibular system disorder causing balance-posture disorders. Second aim of this study was to investigate caloric test responses particularly in early staged PD compared to normal popu-lation. METHODS: Thirty patients (14 females and 16 males; mean age, 60.6 ± 13.1 years) diagnosed with idiopathic PD and 28 healthy subjects (20 males and 8 females; mean age, 59.1 ± 6.4 years) were included. The patient and control groups were subdivided according to their age, gender and the patient group was subdivided according to onset time of the Parkinson symptoms, Hoehn-Yahr staging. The subgroups were compared for VEMP and caloric test responses. RESULTS: There were no significant differences between the study and control groups for right and left VEMP measurements. Patients over 60 years and under 60 years did not show significant differences in terms of right and left mean VEMP measurements. However, P1 amplitude was significantly lower in patients over 60 years old (P = .004). Gender, disease duration, BERG balance scale and Hoehn-Yahr stage had no effect on the VEMP amplitudes. There was no significant correlation with the side of Parkinsonian symptoms to the side of canal paresis (P = .566) and the side on which no VEMP response was obtained in caloric test. CONCLUSION: VEMP responses were not different between PD and healthy subjects. VEMP P1 amplitude was decreased with age in PD group. Canal paresis and symptoms side were not statistically correlated in caloric test.

Serum PGE2, 15d-PGJ, PPARγ and CRP levels in patients with schizophrenia.

Many hypotheses have been proposed for the development of schizophrenia, including the one proposing that exogenous and endogenous factors are linked to inflammatory processes. There is strong evidence about the immunological and inflammatory dysfunction in schizophrenia. In this study, we aimed to measure serum 15-deoxy-delta(12,14)-prostaglandin J (15d-PGJ), peroxisome proliferator-activated receptor gamma(PPARγ), prostaglandin E2 (PGE2) and C-reactive protein (CRP) levels. Forty-four patients and 39 healthy volunteers were included in the study. Serum PGE2, 15d-PGJ, PPARγ and CRP levels were measured in both the groups. Demographic data forms were filled out for the patient group, and the Positive and Negative Syndrome Scale, Clinical Global Impression-Severity scale and Calgary Depression scale were used to assess patients' clinical status. Serum PGE2, 15d-PGJ and PPARγ levels were found to be significantly lower in patients with schizophrenia than in healthy controls. There was no significant relationship between the serum PGE2, 15d-PGJ and PPARγ levels and CRP levels.In this study, the evidence of systemic inflammatory conditions in patients with schizophrenia was found. The duration of the disease has been found to be the only variable that independently affects all three biomarker levels in the patients with schizophrenia.

Red Cell Distribution Width in Diagnosis of Brain Death.

INTRODUCTION: Red blood cell distribution (RDW) is a hematologic index automatically calculated by blood cell counters. Research about RDW in traumatic brain injury showed positive correlation between high RDW values and mortality, which inspired us to investigate whether RDW could be used as a supportive diagnostic biomarker for diagnosis of brain death. Our hypothesis is that RDW may be useful as a biomarker that supports the diagnosis of brain death. METHODS: After approval of the ethics committee, 209 patients who had been diagnosed with brain death between January 2012 and July 2018 were retrospectively reviewed. The RDW values of patients on the days of admission, brain death, and cardiac arrest were recorded. Data were collected from hospital database and patient charts. RESULTS: Statistical analysis revealed that the RDW values on the days of brain death and cardiac arrest were significantly higher than on the day of admission. In addition, the RDW values for the cardiac arrest day were significantly higher than on the day of brain death (P < .001). CONCLUSIONS: We can say that the increase in RDW, which is reported to be an indicator of mortality for many diseases, can be a supporting biomarker for brain death diagnosis when evaluated concomitantly with clinical diagnostic criteria.

Brain Deaths and Donors in an Education and Research Hospital.

BACKGROUND: We aimed to define the causes of brain death (BD), criteria, and tests used for diagnosis, rates of family consent, and rates of organ donation in intensive care units (ICUs) of an education and research hospital. METHODS: The data of patients with BD diagnosis in 7 years in our hospital was collected from an electronic database and archives retrospectively consisting of the demographic data, the causes of BD, criteria, and the tests used for diagnosis, family consent, and organ donation rates. RESULTS: A total of 210 patients with BD diagnosis were enrolled in the study. There was a decline in number of patients with BD diagnosis between 2012 (54.76%) and 2018 (17.64%) in the neurology and neurosurgery ICU, while it increased from 35.71% in 2012 to 70.6% in 2018 in the general ICU. The most common cause of hospitalization for BD was spontaneous intracranial hemorrhage (43.8%). A total of 47.6% of brain-dead patients who did not qualify for organ donation were resuscitated unnecessarily after cardiac death. In 2012, diagnosis was always supported by ancillary tests, while in 2018, a total of 35.29% of the patients were diagnosed solely by clinical examination; 23.8% of patients' families had given consent for organ donation, and 19.53% of 210 patients became donors. CONCLUSION: Physicians should be aware that patients with poor neurologic outcome can be candidates of BD donation, and careful examination and rapid diagnosis is crucial. All segments of society and the health care professionals should be informed and updated about organ donation and BD regularly to raise the numbers of organ donation.

Forced normalization: An overlooked entity in epileptic patients.

The "forced normalization" phenomenon is characterized by acute/subacute onset of psychotic symptoms in the early post-ictal period with dramatic improvement of electrophysiological epileptiform activity. A 56 years old female with going on personality changes, maladaptive behaviours and a mild cognitive impairment since last seizure which was forty-five days ago has been admitted. An evident increase was observed in her maladaptive behaviours with the use of levetiracetam. She began describing visual hallucinations and déjàvu. Control EEG performed 24h after the seizure was completely normal. Levetiracetam therapy was replaced with phenytoin. Quetiapine therapy was also administered. Psychotic symptoms disappeared.

Unexplained neuropsychiatric symptoms in intensive care: A Fahr Syndrome case.

Fahr Syndrome is a rare disease where calcium and other minerals are stored bilaterally and symmetrically in the basal ganglia, cerebellar dentate nucleus and white matter. Fahr Syndrome is associated with various metabolic disorders, mainly parathyroid disorders. The presented case discusses a 64-year old male patient admitted to the intensive care unit of our hospital diagnosed with aspiration pneumonia and urosepsis. The cranial tomography examination to explain his nonspecific neurological symptoms showed bilateral calcifications in the temporal, parietal, frontal, occipital lobes, basal ganglia, cerebellar hemisphere and medulla oblongata posteriorly. His biochemical test results also indicated parathormone-calcium metabolic abnormalities. Fahr Syndrome must be considered for a definitive diagnosis in patients with nonspecific neuropsychiatric symptoms and accompanying calcium metabolism disorders in order to control serious morbidity and complications because of neurological damage.

Elevated interictal serum HSP-70 levels as an indicator of neurodegeneration for chronic migraine.

OBJECTIVE: To investigate whether there is a relationship between chronic migraine and heat shock protein-70. METHODS: The case-control progressive study was conducted at Ankara Numune Teaching and Research Hospital, Ankara, Turkey, from January to June 2013, and comprised patients over 18 years of age who were diagnosed with chronic migraine and did not have any other known neurological illness. Age and gender-matched volunteers with no history of headache or neurological illness were included as controls. In order to exclude other central nervous system diseases, computed tomography and/or magnetic resonance imaging was carried out. Blood samples to evaluate serum heat shock protein-70 levels were obtained from the patients during headache-free periods and from the controls following 8 hours of fasting. The samples were interpreted using the enzyme-linked immunosorbent assay reader. RESULTS: There were 40 controls and an equal number of cases in the study. Mean heat shock protein-70 levels were higher in the cases 2.37±1.91ng/dl compared to thecontrols1.81±1.30 ng/dl, but the difference was not statistically significant (p=0.12). Serum heat shock protein-70 levels were also compared in terms of the duration of migraine disease, frequency of migraine attacks, Visual Analogue Scale score, migraine attack duration and the presence of aura, but no statistically significant difference was found (p=0.13, p=0.17, p=0.90, p=0.68, p=0.95 respectively). CONCLUSIONS: Heat shock protein-70 was not a reliable chronic migraine biomarker.

Comparison of Cognitive Functions Between Obstructive Sleep Apnea Syndrome and Simple Snoring Patients: OSAS May Be a Modifiable Risk Factor for Cognitive Decline.

By comparing neurocognitive test results from patients with obstructive sleep apnea syndrome (OSAS) and those from patients with simple snoring, we aimed to establish whether OSAS negatively influences cognition. Patients with mild-to-severe OSAS (n = 29) and nonhypoxic simple-snoring patients (n = 30) were admitted to the study. All participants in both groups were evaluated with polysomnography and neurocognitive tests, including the Stroop Test, Rey Auditory Verbal Learning Test, Judgment of Line Orientation, Trail-Making Test, and Symbol Digit Modalities Test (SDMT). Significant differences were identified between the groups for test scores on the Rey 1, SDMT, and Stroop tests. We propose that accurate OSAS diagnosis and treatment might help to prevent cognitive decline.