Caregiver burden and its related factors in advanced Parkinson’s disease: data from the PREDICT study

Introduction: Caring for a person with Parkinson’s disease (PD) is associated with an increased risk of psychiatric morbidity and persistent distress. The objective of this study was to describe the burden and the related factors of caregivers of advanced PD (APD) patients either treated with continuous dopaminergic delivery systems or standard therapy. Methods: This cross-sectional, epidemiologic study conducted in 13 Italian sites enrolled PD patients treated with continuous dopaminergic delivering systems [either levodopa/carbidopa intestinal gel (LCIG) infusion or continuous subcutaneous apomorphine infusion (CSAI)] or continuation of standard of care (SOC) with a caregiver. Patient quality of life (QoL) and caregiver burden were assessed using the Parkinson’s Disease Questionnaire (PDQ-8) and Zarit Burden Inventory (ZBI), respectively. Results: 126 patients (mean age 69.3 ± 8 years) and their caregivers (mean age 57.9 ± 12.9) were enrolled. Most caregivers were spouses. Fifty-three patients were treated with LCIG, 19 with CSAI, and 54 with SOC. Mean ZBI scores were 29.6 ± 14.4 for LCIG, 35.8 ± 20.2 for CSAI, and 31.4 ± 16.0 for SOC. Caregivers of LCIG, CSAI, and SOC patients showed no burden or mild/moderate burden in 74, 53, and 63% of the cases, respectively. Mean PDQ-8 scores were 11.25 ± 5.67, 11.26 ± 5.55, and 14.22 ± 6.51 in LCIG, CSAI, and SOC patients. Neurologists considered patients “very much or much improved” in 89, 58, and 13% of the LCIG, CSAI, and SOC groups using the Clinical Global Impression–Global Improvement Scale. Predictors significantly associated with caregiver burden were patients and caregivers’ judgment of QoL and caregivers’ need to change work. Conclusions: Caregiver burden showed a tendency to be lower when patients are treated with LCIG than with CSAI or SOC

How future surgery will benefit from SARS-COV-2-related measures: a SPIGC survey conveying the perspective of Italian surgeons

COVID-19 negatively affected surgical activity, but the potential benefits resulting from adopted measures remain unclear. The aim of this study was to evaluate the change in surgical activity and potential benefit from COVID-19 measures in perspective of Italian surgeons on behalf of SPIGC. A nationwide online survey on surgical practice before, during, and after COVID-19 pandemic was conducted in March-April 2022 (NCT:05323851). Effects of COVID-19 hospital-related measures on surgical patients' management and personal professional development across surgical specialties were explored. Data on demographics, pre-operative/peri-operative/post-operative management, and professional development were collected. Outcomes were matched with the corresponding volume. Four hundred and seventy-three respondents were included in final analysis across 14 surgical specialties. Since SARS-CoV-2 pandemic, application of telematic consultations (4.1% vs. 21.6%; p < 0.0001) and diagnostic evaluations (16.4% vs. 42.2%; p < 0.0001) increased. Elective surgical activities significantly reduced and surgeons opted more frequently for conservative management with a possible indication for elective (26.3% vs. 35.7%; p < 0.0001) or urgent (20.4% vs. 38.5%; p < 0.0001) surgery. All new COVID-related measures are perceived to be maintained in the future. Surgeons' personal education online increased from 12.6% (pre-COVID) to 86.6% (post-COVID; p < 0.0001). Online educational activities are considered a beneficial effect from COVID pandemic (56.4%). COVID-19 had a great impact on surgical specialties, with significant reduction of operation volume. However, some forced changes turned out to be benefits. Isolation measures pushed the use of telemedicine and telemetric devices for outpatient practice and favored communication for educational purposes and surgeon-patient/family communication. From the Italian surgeons' perspective, COVID-related measures will continue to influence future surgical clinical practice

How to avoid trivial mistakes during IPG replacement in patients treated with DBS for movement disorders: Technical note from 13-years experience

Purpose: Recent data suggest how adverse events occur more frequently after Implantable Pulse Generator (IPG) replacement than during the deep electrode positioning in patients treated with Deep Brain Stimulation (DBS). For instance, erroneous extension adjustment to change in laterality and inaccurate lead connection represent problems, which strongly affect patients outcome. We analyzed our data after 13 years of IPG replacement. Materials and Methods: We treated 107 patients (83 PD and 24 Dystonia) with DBS in 13 years. The Dual Channel IPGs replaced during this period were 91. 25 patients needed more than one replacement, especially among the dystonic population. During surgery, we temporarily marked in all the cases the right extension lead before the disconnection from the exhausted IPG. Good impedances were intraoperatively checked in all the cases. Results: Our surgical technique allowed us to avoid any erroneous change in laterality or abnormal impedances due to a suboptimal connection to the IPG. The mean duration of the operation was 25 min and a quick postoperative restart of DBS was possible in all the cases. Stability of symptoms after IPG replacement was achieved in all the patients, with an accurate clinical management within the first 48-72 postoperative hours. Conclusion: Our surgical and postoperative management demonstrates how to avoid some important adverse events with some easy steps, without any discomfort for the patients in terms of duration of surgery or longer hospitalization. Thus, stability of symptoms after the IPG replacement may be easily guaranteed during the first postoperative period

Huntington's disease-like presentation in Spinocerebellar ataxia type 12

No abstract is available for this article

Subtotal arytenoidectomy for the treatment of laryngeal stridor in multiple system atrophy: phonatory and swallowing results

Multiple system atrophy (MSA), according to second consensus on MSA, is a neurological disorder characterized by a combination of autonomic failure and parkinsonism, or cerebellar ataxia, or both.1 Among MSA manifestations, diurnal and nocturnal inspiratory stridor associated with sleep apnoea may help in clinical diagnosis, and its most accepted explanation is vocal folds abductor dysfunction. A possible complication of this situation is sudden nocturnal death. As described in the literature, the use of C-PAP and tracheotomy are the most common treatment proposed for the therapy of the laryngeal disfunction.2 Alternatively laterofixation of the vocal fold according to Ejnell3 or laser arytenoidectomy have been proposed. Here we present the case of nocturnal inspiratory stridor in a MSA patient treated with CO2 laser subtotal aritenoidectomy, with particular attention on phonatory and swallowing outcome

Effect of Globus Pallidus internus stimulation on gait in multifocal dystonia: A case study

A study was conducted to investigate the influence of deep brain stimulation (DBS) of the Globus Pallidus internus (GPi) on multifocal dystonia, called as clinical, spatio-temporal, and kinematic parameters of lower limbs during plain gait. The patient, 52 years old female without skeletal deformities, was recruited from the Center of Movement Disorder at the Department of Neuroscience, in Ferrara, Italy to conduct the investigations. Joint kinematics and spatio-temporal gait parameters were measured by means of 6 infrared cameras at 100 samples per second. Sixteen markers were attached to the subject's lower limbs and pelvis according to the Plug-in Gait protocol (PIG). The median values and confidence intervals of spatio-temporal parameters for the two sides and parameters for right and left side were reported

Idiopathic cervical dystonia and non-motor symptoms: a pilot case-control study on autonomic nervous system

Purpose: Non-motor symptoms, such as sleep disturbances, fatigue, neuropsychiatric manifestations, cognitive impairment, and sensory abnormalities, have been widely reported in patients with idiopathic cervical dystonia (ICD). This study aimed to clarify the autonomic nervous system (ANS) involvement in ICD patients, which is still unclear in the literature. Methods: We conducted a pilot case-control study to investigate ANS in twenty ICD patients and twenty age-sex-matched controls. The Composite Autonomic System Scale 31 was used for ANS clinical assessment. The laser Doppler flowmetry quantitative spectral analysis, applied to the skin and recorded from indices, was used to measure at rest, after a parasympathetic activation (six deep breathing) and two sympathetic stimuli (isometric handgrip and mental calculation), the power of high-frequency and low-frequency oscillations, and the low-frequency/high-frequency ratio. Results: ICD patients manifested higher clinical dysautonomic symptoms than controls (p < 0.05). At rest, a lower high-frequency power band was detected among ICD patients than controls, reaching a statistically significant difference in the age group of ≥ 57-year-olds (p < 0.05). In the latter age group, ICD patients showed a lower low-frequency/high-frequency ratio than controls at rest (p < 0.05) and after mental calculation (p < 0.05). Regardless of age, during handgrip, ICD patients showed (i) lower low-frequency/high-frequency ratio (p < 0.05), (ii) similar increase of the low-frequency oscillatory component compared to controls, and (iii) stable high-frequency oscillatory component, which conversely decreased in controls. No differences between the two groups were detected during deep breathing. Conclusion: ICD patients showed ANS dysfunction at clinical and neurophysiological levels, reflecting an abnormal parasympathetic-sympathetic interaction likely related to abnormal neck posture and neurotransmitter alterations

Acute Painful Neuropathy in a Heroin Body Packer

A broad range of peripheral neurological complications have been recognized in heroin addicts. Peripheral Nervous System (PNS) involvement, usually secondary to trauma during loss of consciousness, commonly manifests as compressive neuropathy or rhabdomyolysis. Other, rare, non traumatic PNS lesions include poliradiculopathy, plexopathy, Guillan-Barrè syndrome and mononeuropathy. Acute heroin related non compressive neuropathy is rare, and the aetiology is still unclear. In heroin abusers painful acute neuropathy has rarely been described. We report the case of a 33-year-old male with a history of heroin abuse. He was found in his home in coma, maintaining the same sitting position for several hours. He was admitted to the intensive care unit, where rhabdomyolysis complicated by acute renal failure was diagnosed, requiring treatment with haemodialysis. A Computed Tomography (CT) scan of the abdomen revealed the presence of several foreign bodies in sigma and the urinary exams showed high concentration of opiates, revealing that the patient was a body packer. Further laboratory exams showed Hepatitis C Virus (HCV) infection with normal liver function. Upon awakening, five days after admission, the patient complained of severe burning pain, mechanical allodynia, itch, tingling and pinprick sensation in both feet and legs. The Douleur Neuropathique en 4 Questions (DN4) scale was 8/10, Numeric Rating Scale (NRS) scale score was 9/10 and Neuropathic Pain Symptom Inventory (NPSI) score was 37. Neurologic examination showed reduced sensitivity to temperature and pinprick in both legs and feet. Neurophysiological evaluation, performed 4 weeks after admission, showed normal nerve conduction studies. Thermal Quantitative Sensory Testing (t-QST) showed a patchy distribution of cold and warm hypoesthesia in the lower limbs. The patient also described positive symptoms (hyperalgesia and aftersensation following hot stimuli, mechanical static and dynamic allodynia) in the same areas. Sympathetic Skin Response (SSR) performed in both arms and feet was normal. Laser Doppler Flowmetry showed reduced local vasodilation after heat stimulus (C-mediated local axonal reflex) and normal vasoconstriction reflex (mediated by adrenergic sympathetic fibres) distally in the legs. The patient declined undergoing a skin biopsy for diagnostic purposes. The clinical and neurophysiological findings indicate the presence of small fibre neuropathy, with main involvement of C fibres, following a length-dependent distribution. To relieve the severe painful symptoms the patient was treated with gabapentin, fentanyl, duloxetina and pregabalin, with a gradual resolution of pain. The follow up examination, performed after 12 months, showed an improvement of the small fibre related symptoms with resolution of spontaneous pain and attenuation of itch, tingling and pinprick sensation, confirmed by the t-QST evaluation. A broad range of peripheral neurological complications have been recognized in heroin abusers. Peripheral nerve lesions in drug addicts may be caused by injection of substances in the vicinity of a nerve, local infection or nerve compression during coma. Combined nerve or plexus lesions and rhabdomyolysis have been reported in relation to possible trauma but they may occur also without apparent trauma to muscles or nerves. In these latter cases toxic or allergic reaction to heroin or adulterants are probably more important causes of rhabdomyolysis and nerve lesions than limb compression. Reports on acute heroin-related non compressive neuropathy are scarce. Dabby et al. described six patients who developed acute PNS injury following intravenous or intranasal heroin self administration with no evidence of compression injury or inflammation. Four patients had plexopathy and two had symmetric distal axonal sensorimotor neuropathy; five had concomitant rhabdomyolysis. They proposed that a toxic mechanism could be responsible for acute neuropathy following heroin abuse. In heroin abusers painful complications have been reported, but the pain was related to myelopathy, plexopathy, sciatic neuropathy and rhabdomyolysis4; none of these cases had length-dependent symptoms or clinical signs of small fibre damage. Numerous studies in humans and animals report that opioids can elicit abnormal pain, like thermal hyperalgesia and/or mechanical allodynia, following both acute and chronic administration of morphine, heroin, methadone, fentanyl and remifentanil. The mechanism responsible for the tactile and thermal hypersensitivity observed following opioid exposure is still unclear. Many hypotheses have been put forward, including sensitization of peripheral nerve endings or second-order neurons, enhanced descending facilitation of nociceptive pathways, increased production, release and decreased re-uptake of neurotransmitters involved in nociception, activation of glial cells and opioid associated epigenetic changes in Deoxyribonucleic Acid (DNA) methylation. Prolonged exposure to opioids results in long-lasting neuroadaptive changes that promote a state of hypersensitivity to normal non-noxious tactile and to noxious thermal stimulation that may promote activation of the nociceptive system. The aetiology of acute PNS complication in heroin addicts can be related to different mechanisms. The impurities and additives present in illicit drugs may cause allergies and have their own toxic effects; mechanical trauma is a potential mechanism of focal nerve injury and rhabdomyolysis. Evidence of an immunologic cause has been found in three patients who developed acute brachial plexopathy and rhabdomyolysis after heroin administration and in one heroin addict who developed a lumbosacral plexus neuropathy and responded to steroid therapy. We describe the case of a body packer who developed acute painful neuropathy with involvement of small nerve fibre with concomitant rhabdomyolysis and acute renal failure. The patient was HCV positive; all the other causes of Small Fibre Neuropathy (SFN) were excluded. In our patient there was no evidence of an inflammatory or immunologic process and mechanical factors probably didn’t play a major role in the pathogenesis. The patient had a history of prolonged lying before arriving to the emergency room but the neuropathy was symmetric and distal, being inconsistent with focal trauma or compression. Acute renal failure and haemodialysis can be possible causes of SFN but in our patient there was not a correlation of the symptoms with the haemodialytic sessions and there was not an improvement of symptoms after achieving normal renal function. HCV infection can play a role in SFN, but the acute presentation of the symptoms in temporal relation with heroin intoxication and their gradual improvement suggest that a toxic effect of heroin or added substances can be responsible for neural and muscle toxicity in our patient. In conclusion a careful neurophysiological evaluation should be considered in heroin abusers presenting with acute pain to rule out SFN

Parkinson's disease-dementia in trans LRP10 and GBA variants: Response to deep brain stimulation

We report on a patient with Parkinson's disease and dementia who underwent DBS with excellent response in motor features; the genotype is heterozygous for a novel LRP10 variant in trans with a GBA variant. He had a more severe phenotype compared to the father who only carries the LRP10 variant