Spontaneous intracranial hypotension: a case series and literature review.

Spontaneous intracranial hypotension (SIH) is a condition of negative intracranial pressure resulting from cerebrospinal fluid (CSF) leakage from the dural sac and is a well-known cause of orthostatic headache. Diagnosis and management can be difficult, often requiring coordination between multiple disciplines. Low CSF pressure and diffuse meningeal enhancement on brain MRI are the major instrumental features of the classic syndrome. Neuroimaging plays a key role in diagnosing SIH, particularly in atypical clinical presentations, by recognizing the specific findings of brain sagging on MRI and detecting the level of CSF leak on spinal imaging, thus guiding therapy accordingly. Since SIH could present with such a heterogeneous clinical picture, careful history taking and increased awareness of atypical presentations are of utmost importance. We review the existing SIH literature, illustrate management, clinical and neuroimaging findings of four consecutive patients with atypical SIH, who were recently referred to our hospital for evaluation to simplify and streamline the management of SIH.

Association between Delirium and Cognitive Impairment: Is there a Link?

BACKGROUND: Delirium and dementia are both disorders involving global cognitive impairment that can occur separately or at the same time in the elderly. OBJECTIVE: The aim of this study was to examine the frequency, correlation, and relative risk between delirium and cognitive impairment in a prospective population study starting at the basal line (onset of delirium) over a period of five years. The secondary aim was to determine any possible correlation between the kind of delirium and a specific type of dementia. MATERIALS AND METHODS: We studied 325 patients diagnosed according to the DSM-IV. The neuropsychological, moods and delirium disorders were evaluated with Hamilton Depression Rating Scale, Delirium Rating Scale-Revised-98, MMSE, Rey auditory-verbal learning test, Digit Span, Symbol Digit Modalities Test, Raven Progressive Matrices, ADL, and IADL. RESULTS: The prevalence of delirium in our population was 89 cases (27.4%): 78 patients (48 women and 30 men) showed evolution toward dementia (mean age was 67.9 ± 6.1 years for men and 68.4 ± 9.1 for women), and 11 patients (5 men and 6 women) presented only isolated delirium without evolution toward cognitive impairment (mean age of men was 68.1 ± 5.1 years and of women 66.4 ± 7.1). The neuropsychological study of the patients with delirium with dementia evolution revealed statistically significant differences over time with a statistically significant intergroup difference and predisposition toward depression. CONCLUSION: The association between delirium and cognitive impairment and the possible role of delirium as an early marker of neurodegenerative diseases need to be investigated in the future.

Wearing off: A complex phenomenon often poorly recognized in Parkinson's disease. A study with the WOQ-19 questionnaire.

BACKGROUND: 'Wearing off' refers to the phenomenology of movement disorders in Parkinson's disease (PD) that appears early and is much commoner than generally believed. It may be present in the form of either motor symptoms or non-motor symptoms. AIM: To investigate the utility of wearing-off questionnaire (WOQ-19, Italian version) in the outpatient clinical practice to assess the suitability of different combinations of treatment, in various stages of PD. METHODS: 73 consecutive patients (58% male and 42% female) suffering from PD were recruited through the Santorso Hospital and San Martino Hospital from September 2012 to March 2014. The patients were asked to indicate whether or not they experienced any of the 19 symptoms listed in the questionnaire during the day; if one or more of these referred symptoms relating to PD improved after taking an additional dose of levodopa; and, if these variations were present routinely. Furthermore, we also evaluated the possible correlation between the presence of motor and non-motor symptoms that were listed in the WOQ 19 with the motor impairment assessed by the Hoehn and Yahr stage (HY) and the Unified Parkinson's Disease Rating Scale (UPDRS) part III motor section, in the whole patient cohort as well as in different subgroups undergoing therapy. RESULTS: Among the 73 patients, 22% were receiving levodopa (a mean daily dose of 300 ± 121.3 mg), 38.3% levodopa and dopamine agonists, 12.3% levodopa/rasagiline, 8.2% levodopa/selegiline, and 19.2% a combination of levodopa/dopamine agonists (DA)/monoamine oxidase inhibitors (MAOI). The most prevalent symptoms were the non-motor symptoms included in the WOQ-19. A significant correlation between the scores obtained on the different motor and non-motor items recorded by the WOQ-19 and the UPDRS motor section and HY scores was found. The therapeutic benefit was especially related to the motor symptoms. DISCUSSION: In clinical practice, this simple and easily administered questionnaire may be useful for the early detection of fluctuations in symptomatology in patients with PD. It will, therefore, help to improve the quality of the global care of patients with PD detected in various stages of the disease.

Clinical and Imaging Study of Isolated and Mixed Rest and Action Tremor-Essential Tremor versus Parkinson's Disease.

BACKGROUND: Some patients present an unusual association of both action tremor (AT) and rest tremor (RT) making the differential diagnosis between essential tremor (ET) and Parkinson's disease (PD) difficult. AIM: To investigate this particular clinical picture trying to focus on possible peculiar clinical inferences. PATIENTS AND METHODS: Twenty-three patients with atypical tremor syndrome were selected for the study. They underwent neurological examination, neuroimaging study, and brain DaTSCAN single-photon emission computed tomography. RESULTS: Twenty-three patients were evaluated; 17 presented mixed-tremor syndrome, while six patients showed only isolated AT or RT. DaTSCAN was pathological in 19 patients and normal in 3 patients. The emerging statistical data highlighted a positive correlation between disease duration and DaTSCAN abnormalities; Fisher's exact test showed a marked difference in evolution toward a dysfunction of dopaminergic pathways in patients with both AT and RT phenotype. CONCLUSION: The possible correlation between PD and ET has often been discussed without any clear findings. Are these patients suffering from ET prone to develop PD? Or are they the expression of a specific clinical phenotype? Our clinical survey has not led to absolute considerations; however, it seeks to highlight the clinical markers that might arouse the suspicion of extrapyramidal disease in patients with atypical tremor syndrome.

Mild Encephalitis with a Reversible Splenial Lesion: A Clinical Benign Condition, often Underrecognized - Clinical Case and Literature Review.

Mild encephalitis with reversible lesion in the splenium is a clinicoradiological syndrome characterized by a variegated symptomatology with a solitary mass in the central portion of the splenium of the corpus callosum. Complete spontaneous resolution is the hallmark of this syndrome, though its pathogenesis is still unknown. We describe the clinical picture of a 51-year-old woman who developed a partial sensitive seizure, with MRI evidence of a lesion localized in the posterior portion of the corpus callosum. The patient made a full recovery thanks to the administration of antiepileptic drugs. Acquiring knowledge of this syndrome, in the wide diagnostic panel which includes vertebrobasilar diseases besides the broad range of metabolic and electrolyte disorders, is crucial to a prompt clinical diagnosis and in establishing a reliable prognosis at an early stage.

Parkinson's disease and cerebrovascular disease: is there a link? A neurosonological case-control study.

Cerebrovascular disease (CVD) and idiopathic Parkinson's disease (PD) frequently occur in the elderly; however, CVD is not frequent in the PD population. The possible relationship between PD and CVD was studied with controversial findings. More specifically, it is unclear whether PD can be protective against the development of vascular disease. To assess the neurosonological examination of a group of PD patients matched with a control group of patients not affected by PD along with the potential risk of developing CVD in the PD group to evaluate any differences. The analysis of the left common carotid artery (CCA) revealed a mean intima-media thickness (IMT) of 0.77 ± 0.21 mm in the PD group and 0.83 ± 0.17 mm in the control group, while the right CCA mean IMT was 0.61 ± 0.17 mm in the PD patients and 0.98 ± 0.18 mm in the control group. The difference was statistically significant in both sides. PD patients show a lower IMT value in older age (70-80 years) and a reduced cardiovascular risk.

Pain perception in major depressive disorder: a neurophysiological case-control study.

BACKGROUND: Depression and pain may sometimes be related conditions. Occasionally, depression may be associated with physical symptoms, such as back pain and headache. Moreover, depression may impair the subjective response to pain and is likely to influence the pain feeling. Conversely, chronic pain may represent an emotional condition as well as physical sensation, and can influence both the mood and behaviour. AIM: To better understand the relationship between pain and depression, we therefore assessed the pain threshold and the tolerance pain threshold in patients with depressive disorders. MATERIALS AND METHODS: We conducted a case-control study and selected patients who had recently received a diagnosis of major depression (DSM-IV), before treatment, and without any significant pain complaints. Age- and sex-matched healthy controls were also included. Tactile and pain thresholds were assessed in all subjects through an electrical stimulation test. All results were compared between the groups. RESULTS: 27 patients and 27 age-matched healthy controls were included in the study. Tactile, pain and tolerance thresholds were evaluated in all subjects. The pain threshold and pain tolerance were lower in patients with major depression than controls. All differences were statistically significant (p<0.05). CONCLUSION: These results suggest the abnormal processing of pain stimuli in depressive disorders.

Cognitive profiles in Mild Cognitive Impairment (MCI) patients associated with Parkinson's disease and cognitive disorders.

BACKGROUND: Mild cognitive impairment (MCI) is rapidly becoming one of the most common clinical manifestations affecting the elderly and represents an heterogeneous clinical syndrome that can be ascribed to different etiologies; the construct of MCI in Parkinson's disease (PD) (MCI-PD) is more recent but the range of deficits is still variable. Early recognition and accurate classification of MCI-PD could offer opportunities for novel therapeutic interventions to improve the natural pathologic course. OBJECTIVE: To investigate the clinical phenotype of amnestic mild cognitive impairment (aMCI) and in patients with PD and MCI (MCI-PD). MATERIALS AND METHODS: Seventy-three patients with aMCI and in 38 patients with MCI-PD were enrolled. They all underwent Mini-mental State Examination (MMSE), the Rey auditory-verbal learning test and the immediate visual memory (IVM) item of the Mental Deterioration Battery, the Rey auditory-verbal learning test included the Rey-immediate (Rey-I), and the delayed recall of the word list (Rey test deferred, Rey-D). The Geriatric Depression Scale (GDS) was used for mood assessment. RESULTS: The results of the Rey-I and Rey-D and of the IVM item showed statistically significant differences between the aMCI and the MCI-PD group. The mean Rey-I and Rey-D score was significantly lower as well as the IVM score was higher in patients with aMCI than in those with MCI-PD, aMCI patients showed greater impairment in long-term memory, whereas more aMCI than MCI-PD patients had preserved attention, computation, praxis, and conceptualization. CONCLUSIONS: Our findings demonstrate that the cognitive deficit profile is specific for each of the two disorders: Memory impairment was a typical feature in aMCI patients while MCI-PD patients suffered from executive functions and visuospatial attention deficits.

Cerebrospinal fluid and lumbar puncture: the story of a necessary procedure in the history of medicine.

The aim of our work was to investigate the different historical stages that led gradually to the discovery of the anatomical structures that form and contain cerebrospinal fluid (CSF), until the Quincke idea, to collect the liquid directly at the lumbar level delivering to humanity a diagnostic tool present and absolutely irreplaceable in everyday clinical practice. This is done through consultation of all the historical medical literature, together with the critical examination of the original articles when available in the most rigorous chronological and speculative order, which enabled knowledge advancement.

Corneomandibular reflex: Anatomical basis.

Corneomandibular reflex is a pathological phenomenon evident in cases of severe brainstem damage. It is considered to be a pathological exteroceptive reflex, associated with precentro bulbar tract lesions. The sign is useful in distinguishing central neurological injuries to metabolic disorders in acutely comatose patients, localizing lesions to the upper brainstem area, determining the depth of coma and its evolution, providing evidence of uncal or transtentorial herniation in acute cerebral hemisphere lesions, and it is a marker of supraspinal level impairment in amyotrophic lateral sclerosis and multiple sclerosis. This sign was evident in a patient with severe brain damage. We discuss the literature findings and its relevance in prognosis establishment.

Sleep disturbance and cognitive disorder: epidemiological analysis in a cohort of 263 patients.

The aim of this study was to investigate and describe frequency and characteristics of sleep disorders in a large cohort of community dwelling persons with several degrees and typologies of cognitive disorders. 236 patients (78 men and 158 women) were enrolled with different subtypes of dementia: Alzheimer's disease (AD), vascular dementia (VaD), mixed dementia, mild cognitive impairment (MCI), dementia with Lewy bodies (DLB), parkinson's disease dementia (PDD), and frontotemporal lobar degeneration (FTLD), respectively. The sleep disturbances evaluated were: insomnia, excessive daytime sleepiness (EDS), REM behavior disorder (RBD), restless legs syndrome (RLS), and nightmares. Every type of sleep disorder was present in each type of dementia but with significant differences. Insomnia is found to be more present and specific for AD; EDS was associated with the presence of dementia in the elderly with LBD or PDD; RLS and nightmares that were recognized mainly in FTD, LBD, and PDD patients scores; patients with MCI had a frequency of sleep disturbances of any type equal to that of patients with AD presenting mostly insomnia, nightmares or RLS more frequently; nightmares were more frequent among LBD and PDD patients. Frequency of RDB was more frequent in FTD, AD, and VaD. Our findings demonstrate that sleep disturbance was related to dementia. A careful clinical evaluation of sleep disorders should be performed routinely in the clinical setting of persons with cognitive decline.

Neurological complications of tick borne encephalitis: the experience of 89 patients studied and literature review.

Tick borne encephalitis (TBE) is an acute febrile syndrome that can be complicated with neurological symptoms ranging from mild meningitis to severe encephalomyelitis. The causative agent is a virus belonging to the family of flaviviruses. We have collected a series of 89 patients and compared the clinical course with the main data of the literature of TBE. This review in addition describes the clinical manifestations associated with TBE infections, the main molecular-biological properties of these viruses, and the different factors that define the incidence and severity of disease who are frequently situated in the age group young/adult with a social harm and functional non-negligible. This review also contains diagnostic elements and neuropathological features typical of this infection and a brief summary of vaccination against TBE.