ABSTRACT
BACKGROUND: Prion diseases are rapidly progressive fatal conditions caused by abnormally shaped proteins. Sporadic Creutzfeldt - Jakob disease (sCJD) is the most common human prion disorder accounting for 85-90 % of cases. Clinical manifestations include rapidly evolving dementia in conjunction with neurological symptoms such as ataxia, myoclonus, pyramidal and extrapyramidal signs. However, the early symptoms of the disease are often non-specific and mental disorder is delayed, making the diagnostic process difficult and challenging. PATIENTS AND METHODS: We present 3 cases with atypical early symptoms and late onset of cognitive decline. The first case presented with isolated visual symptoms (Heidenhain variant), the second patient had isolated anomic aphasia and the third one non-convulsive status epilepticus. A review of the past literature concerning the atypical and rare early clinical features of the sCJD was conducted. RESULTS: The following manifestations were found: psychiatric and visual symptoms, which are relatively common, epileptic seizures, otologic symptoms and presentation of sCJD as an acute vascular event. Moreover, language, communication and writing impairments, movement disorders, symptoms from the peripheral nervous system and bulbar signs were reported as well. CONCLUSION: Increased clinical suspicion, along with the aid of existing diagnostic methods and the development of novel techniques could contribute to a better understanding of the disease's pathophysiology, early and accurate diagnosis and improvement of patient management.
Subject(s)
Creutzfeldt-Jakob Syndrome/diagnosis , Aged , Creutzfeldt-Jakob Syndrome/pathology , Creutzfeldt-Jakob Syndrome/physiopathology , Female , Humans , Male , Middle AgedABSTRACT
INTRODUCTION: Intracerebral haemorrhage in patients suffering from cerebral venous thrombosis (CVT) is relatively uncommon. CVT typically occurs in hypercoagulable state of various causes. Some drugs play a causative role in CVT and thrombopoietin receptor agonists are among them. CASE SUMMARY: We present a female patient with refractory immune thrombocytopenic purpura (ITP) treated with romiplostim, suffering from severe thrombosis of jugular vein expanding intracranially. Despite being treated with adequate anticoagulation, she developed spontaneous bilateral epidural and subdural hematomas with devastating outcome. DISCUSSION: To our knowledge, this is the first reported case of spontaneous atraumatic epidural hematomas due to CVT in adult patient. We support that in our patient, blood stasis leading to the dissection of dura mater, platelet dysfunction, and anticoagulation treatment contributed to the formation of the intracranial, extracerebral haemorrhages.
Subject(s)
Cerebral Hemorrhage/etiology , Hematoma, Epidural, Cranial/etiology , Intracranial Thrombosis/etiology , Purpura, Thrombocytopenic, Idiopathic/complications , Venous Thrombosis/etiology , Adult , Anticoagulants/adverse effects , Cerebral Hemorrhage/diagnostic imaging , Fatal Outcome , Female , Hematoma, Epidural, Cranial/diagnostic imaging , Humans , Intracranial Thrombosis/diagnostic imaging , Intracranial Thrombosis/drug therapy , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Risk Factors , Treatment Outcome , Venous Thrombosis/diagnostic imaging , Venous Thrombosis/drug therapyABSTRACT
OBJECTIVE: Fatigue is one of the most frequent and important nonmotor symptoms of patients with Parkinson disease (PD), affecting quality of life. Although, in some cases, it may be a severe and debilitating complaint, it remains relatively unexplored. The PFS-16 is a fatigue measure, specifically designed for PD patients. The aim of this study was to investigate the psychometric properties of Parkinson fatigue scale (PFS-16) in Greek PD patients. METHODS: In total, 99 patients with PD were assessed. The following psychometric properties were tested: data quality, floor/ceiling effects, reliability (internal consistency, test-retest reliability), and construct validity. Construct validity was evaluated by examining correlations with other variables including other fatigue measures such as Fatigue Severity Scale (FSS) and the vitality scale (SF-VT) of SF-36. Moreover, assumptions were explored about "known" groups concerning fatigue. RESULTS: The mean score for the PFS-16 was 2.95 (± 0.91); acceptability was good with negligible floor and ceiling effects. Results showed high internal consistency (Cronbach's alpha, 0.96) and test-retest reliability (ICC, 0.93). Strong correlations were observed between the PFS-16 and other fatigue (FFS and SF-VT) measures (rs = 0.77 and - 0.70, p < 0.001), revealing appropriate validity. Furthermore, predictions for "known" groups validity were verified. CONCLUSION: The Greek version of the PFS-16 showed satisfactory reliability and validity and thus can be regarded as a useful tool in assessing fatigue in PD.
