Sporadic Creutzfeldt Jakob disease is a rare, fast-progressing neurodegenerative disease with a fatal outcome. Even though its treatment options are scarce, and there is no cure for the disease, adequate diagnosis can help patients and their families come to terms with the disease on time, and give them valuable time to plan accordingly. We report a case of a patient presenting to our emergency department with a 2-week history of ataxia, and oscillopsia. Her initial neurological examination revealed subtle dysarthria, diplopia with left gaze, wide-based ataxic gait with occasional small steps, sinistropulsion in the Romberg position, and ataxia of the limbs, predominantly of the left arm. The patient at that time did not exhibit cognitive impairment, movement disorders, or other neurological signs. Her initial brain MSCT was without lesions or other pathomorphological supstrate. During hospitalization, treatable causes were firstly excluded with blood and CSF lab tests excluding metabolic, toxic, infectious, autoimmune, and paraneoplastic causes. Detailed medical history revealed subtle personality changes, while cognitive testing revealed moderate cognitive impairment. Brain MRI and EEG 4 days after hospitalization reported typical changes seen with advanced prion disease surprisingly being the fact that the patient had a mild to moderate clinical picture. RtQuIC analysis of the CSF was performed to prove probable sCJD and was positive. The patient's family were given instructions, while the wishes of the patient, and family members were fulfilled concerning planning future care. Afterward, the patient's state deteriorated rapidly as per the tragic prognosis of sCJD resulting in akinetic mutism, and death. Ataxia without cognitive impairment, rigor, or movement disorders is an uncommon clinical presentation for a disease with a 1:1 000 000 incidence rate. Modern diagnostic methods in way of more advanced brain MRI capabilities, and RT-QuIC obviate the need for complicated, and potentially infectious brain biopsy in diagnosing sCJD. Alongside the case report, we present a short but comprehensive literature review of modern data regarding the sCJD. This case report and literature review serve to educate clinicians about this rare but devastating disease.
After headache, vertigo is the most common symptom, not only in neurology or otorhinolaryngology but also one of the most common symptoms in medicine in general. Due to the different aetiology, acute and chronic manifestations, combination with other diseases, drugs, or other substances such as narcotics, alcohol or synthetic drugs, and unclear description of vertiginous symptoms on admission, vertigo as a disease or only a symptom can easily mislead. Due to the illusiveness of the symptoms as perceived and reported by patients and doctors alike, vertiginous symptoms warrant cooperation between patients and doctors, and amongst doctors of different specialities as well. In the Republic of Croatia, as well as in the rest of Europe 1 , the waiting lists for an appointment in the hospital are getting longer and longer. Moreover, it can take several months to a year to get an appointment with a specialist. Waiting listsThe waiting list for hospitals for vertigo can vary depending on several factors, such as the healthcare system in a particular country or region, the availability of specialized healthcare providers, and the severity of the condition. In general, the waiting times for treatment of vertigo can range from a few weeks to several months. This is because there may be a limited number of healthcare professionals who specialize in treating vertigo, such as otolaryngologists or neurologists. Additionally, diagnostic tests, such as vestibular function tests or imaging studies, may also contribute to the waiting time. There can be several issues associated with waiting lists for vertigo treatment. Some common challenges include: a) Limited resources: Hospitals and healthcare facilities may have a limited number of specialists, equipment, and resources dedicated to diagnosing and treating vertigo. This can result in longer waiting times for patients seeking treatment. b) High demand: Vertigo is a common symptom, and many individuals may seek medical attention for its treatment. The high demand for services can contribute to longer waiting lists. c) Prioritization of cases: Hospitals often prioritize patients based on the severity of their condition.Patients with more severe or urgent cases may be given priority over those with less urgent cases. This can lead to longer waiting times for individuals with milder or less urgent symptoms. d) Geographic location: The availability of specialized healthcare providers can vary depending on the geographic location. Individuals residing in rural or remote areas may have limited access to healthcare services, resulting in longer waiting times.
Spectacular Shrinking Deficit (SSD) is a term attributed by J.P. Mohr to a rare cerebrovascular event defined by a rapid and dramatic improvement of major hemispheric stroke syndrome. It is presumed to be caused by the migration of initially embolic occlusion of an internal carotid artery or middle cerebral artery to its distal branches. It is only reported in several case reports, and case series with differing criteria of what defies an SSD meaning that its presumed prevalence rate of 7-14% of major hemispheric stroke syndromes could be an overestimation. It is usually associated with the cardioembolic cause of stroke, and it has a higher prevalence rate in younger patients, males, and nondiabetics. Our case is a 58 -year old male who presented to our ER with a major hemispheric stroke syndrome (deviation of head and eyes on the right side, central facioparesis on the left, left hemiplegia, and left hemineglect, NIHSS 16) 30 minutes after symptom onset. He was aggressive, insisting nothing was wrong with him. His initial brain CT showed acute ischemic changes in the right temporooccipital region with an inadequate filling of distal branches of the right MCA shown on CT brain angiography. His symptoms spectacularly improved in our ICU (NIHSS 0) 51 minutes from symptom onset negating the need for thrombolysis. Except for one positive Beta -2 GPI test his detailed laboratory tests, 24h Holter ECG, TTE, carotid, and vertebral artery ultrasound were noneventful. He had no cognitive or neurological deficit. He denied the possibility of performing TEE and prolonged cardiac monitoring. Control brain MRI 4 days and 4 months later confirmed moderate ischemic changes of the right insular, temporal, and occipital cortex. Studies report that spontaneous recanalization usually happens in 17% of patients but does not correlate with TIA. This is the first report of an SSD with moderate ischemic stroke and no leftover neurological deficit. Other case series report moderate ischemic stroke SSD with a small residual neurological deficit (NIHSS 2-4). Perhaps mood changes can be associated with selective neuronal loss found in animals and patients suffering transient occlusion of the brain artery. The cardioembolic cause can in certainty be excluded with TEE and prolonged cardiac monitoring.
Introduction: This study aimed to review the prevalence of NMS in patients with Parkinson's disease in Split-Dalmatia County and to investigate the effects of other variables on the number and structure of NMS. Materials and Methods: This observational cross-sectional pilot study was conducted at the Department of Neurology, University Hospital of Split. This study lasted from November 2017. to April 2018. and it included 31 patients, hospitalized or treated in the infirmary at the Department of Neurology. By examining medical documentation we acquired necessary demographic and medical information about our subjects. Subjects were then examined and subjected to filling out Parkinson's disease-specific questionnaires: NMSQuest, Hoehn&Yahr scale, UPDRS, and Schwab&England scale. To analyze given variables we used the Mann-Whitney U test, Kruskal-Wallis test, Spearman's coefficient of correlation, and ꭓ2 test. Results: On average 12 dwifferent NMS were reported per patient. Most prevalent NMS were reported to be nocturia (67.74%), urinary urgency (61.29%), constipation (61.29%), intense dreams (58.06%), and forgetfulness (58.06%). The least prevalent NMS reported were delusions (6.45%), hallucinations (16.12%), and daytime sleepiness (25.80%). There were neither statistical differences in the total number of NMS nor in demographic variables between men and women. The increased burden of NMS correlated with the disease duration. Prevalence of depression, anxiety, and miscellaneous symptoms rose with the length of disease duration. We established a connection between the number of NMS and the stage of disease according to Hoehn and Yahr. Patients in higher stages of disease (moderate and severe) reported more severe NMS burden than patients in the mild stage of the disease, while patients in the moderate stage had the largest NMS score of all groups. Patients in the mild stage reported urinary, also digestive, and cognitive symptoms, in the moderate stage urinary symptoms, sexual dysfunction, and cardiovascular symptoms were most reported, while in the severe stage of the disease, patients reported cardiovascular symptoms, depression, and anxiety alongside urinary symptoms as most prevalent. Conclusions: Patients are most affected by the autonomic spectrum of NMS, especially urinary symptoms. Duration of the disease and stage of the disease are proven to affect the total number of NMS, while age at onset, current age, and sex have been disproven to have any effect on the total NMS score. Patients in the moderate stage reported the most NMS.
In this letter, we present our opinion, subserved to sociological data, on an underwhelming state of affairs concerning the public, and medical community opinion of functional neurological disorders in Croatia.Functional neurological disorders abbreviated "FND" (ICD -10 F44, F45) are defined as various neurological symptoms such as limb weakness, unexplained sensory symptoms, cognitive disorders, gait difficulty, movement disorders, or paroxysmal episodes which are inconsistent and incompatible with current knowledge of known organic neurological diseases (1). The prevalence of FND is estimated to be 50 per 100 000 population per year with an incidence of 4 to 12 per 100 000 population per year (2). Total costs of ED visits and inpatient care of adult FND in the USA are above $1.2 billion annually (1) comparable to hardest-to-treat neurological diseases. Current understanding of FND has advanced with neurobiological data of altered neuronal networks, and the exclusion of necessary stressor agents advancing its understanding from previous definitions such as psychogenic, conversive, or medically unexplained neurological disorders. Combining detailed medical history, and clearly defined positive signs in neurological examination demonstrating inconsistency allows this to be a safe diagnosis with the frequency of misdiagnosis being 4%, and with a rate of reversal of diagnosis being only 0.4% (2). Neurologists› role is paramount in treating FND starting with an adequate explanation of diagnosis, arranging a multidisciplinary treatment plan (physiotherapy, cognitive behavioral therapy by psychologists, and non-pharmacological methods), and follow-up of patients. Early diagnosis, honest follow-up, and discourse show promising results in treating this disorder (3). FND as a diagnosis in Croatia is still underrecognized by primary physicians, neurologists, patients, and the general public. No adequate scientific research on it is represented in the Croatian scientific bibliography search engine (CROSBY) with keywords such as "dissociative disorder" entered in search engine resulting in 28 papers, "conversive disorder" presenting 10 papers, "psychogenic" presenting 8 papers, and "functional neurological disorder" presenting 0 papers (4). FND is often mentioned to be second to headaches with regards to outpatient neurological visits, and the CROSBY keyword "headache" produces 276 results showing the disparity in coverage of these diagnoses in Croatia. According to the Croatian Public Health Department Mental disease Bilten (5) morbidity percentage
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