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.

Current territorial organization for access to revascularization therapies for acute ischemic stroke in the Veneto region (Italy) from 2017 to 2021.

INTRODUCTION: To evaluate the access to treatments with intravenous thrombolysis (IVT) and/or mechanical thrombectomy (MT) in acute ischemic stroke patients admitted to stroke units (SUs) of Veneto region (Italy) according to current "hub-and-spoke" model from 2017 to 2021. PATIENTS AND METHODS: We retrospectively analyzed data on treatments with IVT and/or MT for stroke patients admitted to the 23 SUs (6 Hubs and 17 Spokes) of the 6 macro-areas including 9 local sanitary units (LSUs) and 2 hospitals. RESULTS: We reported 6093 treatments with IVT alone, 1114 with IVT plus MT, and 921 with MT alone. Number of stroke unit (SU) beds/100,000 inhabitants ranges from 2.3 to 2.8, and no difference was found among different macro-areas. Number of treatments/100,000 inhabitants/year ranges from 19 to 34 for IVT alone, from 2 to 7 for IVT plus MT, and from 2 to 5 for MT alone. Number of IVT alone/SU bed/year ranges from 9 to 21 in the Hub and from 6 to 12 in the Spokes. Rate of IVT plus MT in patients directly arrived in the same LSU's Hub ranges from 50 to 81%, likewise the one of MT alone ranges from 49 to 84%. CONCLUSIONS: Treatment target rates of IVT and MT set by Action Plan for Stroke in Europe 2018-2030 has been globally exceeded in the Veneto region. However, the target rate of MT and access revascularization treatments is heterogeneous among different macro-areas. Further efforts should be made to homogenize the current territorial organization.

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.

Relevance of sleep quality on caregiver burden in Parkinson's disease.

Parkinson's disease (PD) is a neurodegenerative disorder which affects the quality of life of patient and their family. Sleep disorders appear in 80-90% of PD patients and have a great impact on the PD well-being. We examined the relationship of patients' sleep quality and depression on burden, mood, quality of life, and quality of sleep of their caregivers. A multicenter, regional (Veneto), observational, cross-sectional study that included 55 patient-caregiver pairs was conducted. Patients were assessed using Parkinson's Disease Sleep Scale (PDSS) and Epworth Sleepiness Scale (ESS) for sleep disorders, Beck Depression Inventory (BDI) as a measure of depression, and Parkinson's Disease Questionnaire (PDQ-39) as a measure of quality of life. Caregivers were evaluated by the Caregiver Burden Inventory (CBI) a measure of burden, BDI, SF-36 Health Survey as measures of HRQoL, and Medical Outcomes Study-Sleep Scale (MOS-SS) for quality of sleep. CBI, HRQoL, MOS-SS, and BDI scores displayed no association with patients' age, cognition (Mini Mental State Examination (MMSE) and Frontal Assessment Battery (FAB)), disease duration, and Hoehn and Yahr (H&Y), and UPDRS III scales whereas were significantly correlated with patients' quality of sleep, depression, and quality life. CBI and HRQoL were also associated respectively with patients' ESS and L-dopa daily dose. This study underscores the presence of a significant relationship between patient and caregiver quality of life. Interestingly, sleep quality and depression rather than motor disability best predicted caregivers' well-being.

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.

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.

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.

A systematic review of catechol-0-methyltransferase inhibitors: efficacy and safety in clinical practice.

UNLABELLED: Catechol-O-methyltransferase (COMT) inhibitors are drugs commonly used in the management of patients with Parkinson disease complicated by motor fluctuations. Among them, entacapone is the most commonly used. Tolcapone has been reintroduced in patients where entacapone has proved to be ineffective after being withdrawn from the market because of sporadic cases of hepatotoxicity. The last COMT inhibitor is nebicapone, which use in clinical practice is still under study. OBJECTIVES: The objectives of this study were to analyze the clinical efficacy in reducing motor complications and to evaluate their use in clinical practice and the adverse events reported in the literature. METHODS: Scientific articles of the main previously mentioned drugs have been reviewed. RESULTS: All these 3 drugs have proved to be effective in improving wearing-off and significantly reduce the daily dose of levodopa at the number of daily intakes. Tolcapone is undoubtedly the most effective drug, although in clinical practice sporadic cases of hepatotoxicity have limited its use in patients unresponsive to entacapone. Nebicapone is effective, and its safety is still under evaluation. Entacapone is generally well tolerated, and no significant adverse events are reported. CONCLUSIONS: To manage motor fluctuations, the use of COMT inhibitors is now consolidated in the common clinical practice. Tolcapone is used as a second choice in patients with severe motor fluctuations not responsive to entacapone.

Myelin protein zero Val102fs mutation manifesting with isolated spinal root hypertrophy.

The Val102fs mutation of the myelin protein zero gene (MPZ) has been associated with Charcot-Marie-Tooth disease type 1B (CMT1B). Here we describe an unusual presentation of the Val102fs mutation characterized by symptoms of spinal root hypertrophy with no overt peroneal muscular atrophy. Two sisters aged 41 and 35 years complained of neck pain and presented only pes cavus or deep-tendon hyporeflexia. In both of them magnetic resonance imaging revealed non-enhancing hypertrophy of spinal roots misdiagnosed as neurofibromatosis; neurophysiology disclosed a demyelinating neuropathy and addressed the correct molecular diagnosis. This report adds new data concerning the clinical presentations of MPZ mutations.

Peroneal nerve orthodromic sensory conduction technique: normative data.

In some definite patients, a standard neurophysiological tool may not solve a complete differential diagnosis in common nerve peroneal neuropathy. In this study we have assessed a new simple procedure to study the orthodromic sensory conduction of both the superficial peroneal nerves (SPN) and deep peroneal nerves (DPN) in a heterogeneous group of 55 normal subjects. The mean sensory orthodromic conduction velocity of the SPN was 58.35 m/s. The mean sensory orthodromic conduction velocity of the mixed nerve action potential (MNAP) of the DPN was 55.27 m/s. The sensory conduction velocity, the amplitude of sensory-evoked potentials of SPN and DPN across the fibular head and the normative values are discussed. Our results confirm that these recording methods are easy to repeat and reliable in identifying peroneal neuropathy.

Neuropsychological changes after subthalamic nucleus stimulation: a 12 month follow-up in nine patients with Parkinson's disease.

Deep brain stimulation of the subthalamic nucleus has been recognized as one of the most promising techniques to decrease 'off' motor symptoms and motor fluctuations, allowing a reduction of drug therapy and limiting side effects of drug therapy. However, there is still open debate on the possible consequences of chronic subthalamic stimulation on general cognitive performance. A general amelioration of cognitive performance, in particular of executive functions has been reported but results are not homogeneous. We studied nine patients with Parkinson's Disease for 12 months following surgery for deep stimulation, studying their cognitive performances, paying particular attention to linguistic tests and selective alternating words production. Our results may be consistent with a slowing of cognitive activity, with a reduction of quantitative production, but with an increase in control of linguistic production, which is more precise and definite. We discuss the possible significance of these results, fully aware that only nine patients were involved, and that the potential for generalization is seriously limited, with a particular overview on the frontal-subthalamic pathway, which in our opinion is responsible for the results we observed.

Cognitive changes following subthalamic nucleus stimulation in two patients with Parkinson disease.

The limits of a drug therapy in severe forms of Parkinson disease have led to refining neurosurgery on the basal ganglia. Deep brain stimulation of the subthalamic nucleus has been recognized as one of the most promising techniques to decrease "off" motor symptoms and motor fluctuations, allowing a reduction of drug therapy and limiting side effects of the drugs. There is still open debate on the possible consequences of chronic subthalamic stimulation in other ways, apart from motor symptoms, of general cognitive performance. We examined and followed two patients with Parkinson disease for 9 mo. after surgery for deep stimulation, studying their cognitive performances. There is a general amelioration of cognitive performances, in particular as far as linguistic capabilities is concerned. We discuss the possible significance of these results, reminding strenuously that only two patients were involved, so the potential for generalization is seriously limited.